Atlas and Synopsis of Clinical Dermatology

C0NINIS
Pieface xxiii
Acknowledgment xxiv
Intioduction xxv
Approach to Dermatologic Diagnosis xxvi
Outline of Dermatologic Diagnosis xxvi
Special Clinical and Laboratory Aids to Dermatologic Diagnosis xxxv
PAkI I
DISCRDERS PRESENTINC IN THE SKIN
AND MUCCUS MEMRANES
SECI|0N 1
0IS0k0kS 0F S8AC0S AN0 AF0CkIN CIAN0S 2
Acne Vulgaris (Common Acne) and Cystic Acne 2
Rosacea 9
Perioral Dermatitis 14
Hidradenitis Suppurativa 16
SECI|0N 2
CIMA[0kMAIIIIS 20
Contact Dermatitis 20
Irritant Contact Dermatitis 20
Acute Iiiitant Contact Deimatitis 21
Chionic Iiiitant Contact Deimatitis 22
Allergic Contact Dermatitis 26
Alleigic Contact Deimatitis Due to Plants 29
Systemic ACD 32
Aiiboine ACD 32
Atopic Dermatitis 34
Lichen Simplex Chronicus 42
Prurigo Nodularis 44
Dyshidrotic Eczematous Dermatitis 45
Nummular Eczema 46
Autosensitization Dermatitis 48
Seborrheic Dermatitis 48
Asteatotic Dermatitis 52
SECI|0N 5
FS0kIASIS 53
Psoriasis Vulgaris 53
Pustular Psoriasis 62
Palmoplantai Pustulosis 62
Geneialized Acute Pustulai Psoiiasis (von Zumbusch) 64
C0|IE|I' v
Psoriatic Erythroderma 67
Psoriatic Arthritis 67
Management of Psoriasis 67
SECI|0N 4
IChIh0SS T2
Dominant Ichthyosis Vulgaris 72
X-Linked Ichthyosis 75
Lamellar Ichthyosis 78
Epidermolytic Hyperkeratosis 81
Ichthyosis in the Newborn 83
Collodion Baby 83
Hailequin Fetus 84
Syndromic Ichthyosis 84
Acquired Ichthyosis 84
Inherited Keratodermas of Palms and Soles 84
Diffuse Palmoplantai Keiatodeima 85
Punctate Palmoplantai Keiatodeima 85
Focal Palmoplantai Keiatodeima 86
SECI|0N 5
MISCIIAN0S FI0kMAI 0IS0k0kS 88
Acanthosis Nigricans 88
Darier Disease 90
Grover Disease 92
Pityriasis Rubra Pilaris 93
Disseminated Superncial Actinic Porokeratosis 96
SECI|0N 6
8II0S 0ISASS 98
Hereditary Epidermolysis Bullosa 98
Familial Benign Pemphigus 105
Pemphigus 106
Bullous Pemphigoid 112
Cicatricial Pemphigoid 114
Pemphigoid Gestationis 115
Dermatitis Herpetiformis 116
Linear IgA Dermatosis 119
Epidermolysis Bullosa Acquisita 120
SECI|0N 1
MISCIIAN0S INFIAMMAI0k 0IS0k0kS 122
Pityriasis Rosea 122
Parapsoriasis en Plaques 124
Small-Plaque Paiapsoiiasis (Digitate Deimatosis) 124
Laige-Plaque Paiapsoiiasis (Paiapsoiiasis en Plaques) 126
Lichen Planus 128
Granuloma Annulare 134
C0|IE|I' x
Morphea 136
Lichen Sclerosus et Atrophicus 142
Pigmented Purpuric Dermatoses 144
Pityriasis Lichenoides (Acute and Chronic) 146
Erythema Multiforme Syndrome 148
Erythema Nodosum Syndrome 152
Other Panniculitides 154
Pyoderma Gangrenosum 156
Sweet Syndrome 160
Granuloma Faciale 163
SECI|0N 8
Svk AN0 IIF-IhkAININC kFII0NS IN Ih ACII III FAIINI 164
Exfoliative Erythroderma Syndrome 164
Rashes in the Acutely Ill Febrile Patient 170
Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis 173
SECI|0N 9
8NICN N0FIASMS AN0 hFkFIASIAS 1T8
Disorders of Melanocytes 178
Acquiied Nevomelanocytic Nevi 178
Halo Nevomelanocytic Nevus 183
Blue Nevus 184
Nevus Spilus 186
Spitz Nevus 188
Mongolian Spot 189
Nevus of Ota 190
Vascular Tumors and Malformations 192
Vascular Tumors 193
Hemangioma of Infancy 193
Pyogenic Gianuloma 198
Glomus Tumoi 199
Angiosaicoma 200
Vascular Malformations 201
Capillaiy Malfoimations 201
Por-Vne San 201
SJer ngoma 202
Venous La|e 204
C|erry ngoma 205
ngo|eraoma 206
Lymphatic Malfoimation 208
Lym|angoma 208
Capillaiy/Venous Malfoimations 209
Miscellaneous Cysts and Pseudocysts 211
Epideimoid Cyst 212
Tiichilemmal Cyst 212
Epideimal Inclusion Cyst 212
Milium 213
Digital Myxoid Cyst 214
C0|IE|I' x
Miscellaneous Benign Neoplasms and Hyperplasias 215
Seboiiheic Keiatosis 215
Beckei Nevus 219
Tiichoepithelioma 220
Syiingoma 220
Sebaceous Hypeiplasia 222
Nevus Sebaceous 222
Epideimal Nevus 222
Benign Dermal and Subcutaneous Neoplasms and Hyperplasias 224
Lipoma 224
Deimatofbioma 224
Hypeitiophic Scais and Keloids 227
Infantile Digital Fibiomatosis 230
Skin Tag 231
SECI|0N 10
Fh0I0SNSIIIvII, Fh0I0-IN0C0 0IS0k0kS,
AN0 0IS0k0kS 8 I0NIIINC kA0IAII0N 232
Skin Reactions to Sunlight 232
Acute Sun Damage (Sunbuin) 235
Diug-/Chemical-Induced Photosensitivity 236
Phototoxic Diug-/Chemical-Induced Photosensitivity 238
Sysemt P|oooxt Dermas 240
Tota| P|oooxt Demas 241
P|yo|ooJermas 242
Photoalleigic Diug-/Chemical-Induced Photosensitivity 244
Polymorphous Light Eruption 248
Solar Urticaria 250
Photoexacerbated Dermatoses 250
Metabolic Photosensitivity - The Porphyrias 252
Por|yra Cuanea TarJa 252
Varegae Por|yra 257
Ery|rooet Prooor|yra 259
Chronic Photodamage 262
Deimatoheliosis (Photoaging") 262
Solai Lentigo 264
Chondiodeimatitis Nodulaiis Helicis 266
Actinic Keiatosis 267
Skin Reactions to Ionizing Radiation 270
Radiation Deimatitis 270
SECI|0N 11
FkCANCk0S ISI0NS AN0 CIAN0S CAkCIN0MAS 2T4
Epidermal Precancers and Cancers 274
Epithelial Piecanceious Lesions and SCCIS 274
Solai oi Actinic Keiatosis 275
Cutaneous Hoin 275
Arsenical Keratoses 276
Squamous Cell Carcinoma in Situ 276
Invasive Squamous Cell Carcinoma 280
C0|IE|I' x
Keratoakanthoma 286
Basal Cell Carcinoma 287
Basal Cell Nevus Syndrome 294
Malignant Appendage Tumors 296
Merkel Cell Carcinoma 296
Dermatonbrosarcoma Protuberans 298
Atypical Fibrosarcoma 299
SECI|0N 12
MIAN0MA FkCkS0kS AN0 FkIMAk CIAN0S MIAN0MA 300
Precursors of Cutaneous Melanoma 300
Dysplastic Melanocytic Nevus 300
Congenital Nevomelanocytic Nevus 304
Cutaneous Melanoma 308
Clinical Piesentations of Melanoma 310
Me|anoma n Su 311
Lengo Ma|gna Me|anoma 312
Suerfta| SreaJng Me|anoma 315
NoJu|ar Me|anoma 320
Desmo|ast Me|anoma 323
tra| Lengnous Me|anoma 324
me|anot Me|anoma 326
Ma|gnan Me|anoma o[ |e Mutosa 327
Metastatic Melanoma 328
Staging of Melanoma 331
Prognosis of Melanoma 332
Management of Melanoma 332
SECI|0N 15
FICMNIAk 0IS0k0kS 334
Vitiligo 335
Albinism 341
Oculocutaneous Albinism 341
Melasma 344
Pigmentary Changes Following Inammation of the Skin 346
Hypeipigmentation 346
Hypopigmentation 349
PAkI II
DERMATCLCCY AND INTERNAL MEDICINE
SECTICN 14
IHE 5KIN 5ICN5 IN IMMUNE, AUIOIMMUNE,
AND kHEUMAIIC DI5OkDEk5 354
Systemic Amyloidosis 354
Systemic AL Amyloidosis 354
Systemic AA Amyloidosis 356
Localized Cutaneous Amyloidosis 356
C0|IE|I' x
Urticaria and Angioedema 358
Behet Disease 366
Dermatomyositis 370
Livedo Reticularis 374
Sneddon Syndiome 376
Lupus Erythematosus 376
Systemic Lupus Eiythematosus 378
Cutaneous Lupus Eiythematosus 380
tue Cuaneous Luus Ery|emaosus 380
Su|atue Cuaneous Luus Ery|emaosus 383
C|ront Cuaneous Luus Ery|emaosus 384
C|asst C|ront DstoJ LE 384
C|ront Luus Panntu|s 388
Scleroderma 389
Scleiodeima-Like Conditions 393
Raynaud Phenomenon 394
Vasculitis 397
Hypeisensitivity Vasculitis 397
Schnlein-Henoch Puipuia 399
Polyaiteiitis Nodosa 400
Wegenei Gianulomatosis 402
Giant Cell Aiteiitis 405
Uiticaiial Vasculitis 407
Nodulai Vasculitis 408
Kawasaki Disease 410
Reactive Arthritis 414
Sarcoidosis 417
SECTICN 15
ENDOCkINE, MEIAOLIC, NUIkIIIONAL, AND CENEIIC DI5EA5E5 420
Skin Diseases in Pregnancy 420
Cholestasis of Piegnancy 420
Pustulai Psoiiasis in Piegnancy 420
Pemphigoid Gestationis 420
Polymoiphic Eiuption of Piegnancy 422
Piuiigo of Piegnancy and Atopic Eiuption of Piegnancy 422
Skin Manifestations of Obesity 424
Diabetes Mellitus 424
Skin Diseases Associated with Diabetes Mellitus 424
Diabetic Bullae 425
Diabetic Foot" and Diabetic Neuiopathy 426
Diabetic Deimopathy 427
Neciobiosis Lipoidica 428
Cushing Syndrome and Hypercorticism 430
Graves Disease and Hyperthyroidism 431
Hypothyroidism and Myxedema 431
Addison Disease 433
Metabolic and Nutritional Disorders 434
Xanthomas 434
C0|IE|I' x
Xan|e|asma 436
Xan|oma TenJneum 436
Xan|oma Tu|erosum 436
Erue Xan|oma 438
Xan|oma Sraum Pa|mare 438
Normo|emt P|ane Xan|oma 439
Scuivy 440
Zinc Defciency and Aciodeimatitis Enteiopathica 442
Pellagia 445
Gout 446
Genetic Diseases 448
Pseudoxanthoma Elasticum 448
Tubeious Scleiosis 449
Neuiofbiomatosis 453
Heieditaiy Hemoiihagic Telangiectasia 457
SECTICN 16
5KIN 5ICN5 OF VA5CULAk IN5UFFICIENCY 458
Atherosclerosis, Arterial Insufnciency, and Atheroembolization 458
Thromboangiitis Obliterans 462
Thrombophlebitis and Deep Venous Thrombosis 462
Chronic Venous Insufnciency 465
Most Common Leg/Foot Ulcers 471
Livedoid Vasculitis 475
Chronic Lymphatic Insufnciency 476
Pressure Ulcers 477
SECTICN 17
5KIN 5ICN5 OF kENAL IN5UFFICIENCY 480
Classincation of Skin Changes 480
Calciphylaxis 480
Nephrogenic Fibrosing Dermopathy 483
Acquired Perforating Disorders 485
SECTICN 18
5KIN 5ICN5 OF 5Y5IEMIC CANCEk5 486
Mucocutaneous Signs of Systemic Cancers 486
Classifcation of Skin Signs of Systemic Cancei 486
Metastatic Cancer to the Skin 487
Paget Disease 494
Mammaiy Paget Disease 494
Extiamammaiy Paget Disease 496
Cowden Syndrome (Multiple Hamartoma Syndrome) 498
Peutz-Jeghers Syndrome 498
Glucagonoma Syndrome 500
Malignant Acanthosis Nigricans 502
Paraneoplastic Pemphigus 503
C0|IE|I' xv
SECTICN 19
5KIN 5ICN5 OF HEMAIOLOCIC DI5EA5E 504
Thrombocytopenic Purpura 504
Disseminated Intravascular Coagulation 506
Cryoglobulinemia 509
Leukemia Cutis 511
Langerhans Cell Histiocytosis 514
Mastocytosis Syndromes 519
SECTICN 20
CUIANEOU5 LYMPHOMA5 AND 5AkCOMA 524
Adult T Cell Leukemia/Lymphoma 524
Cutaneous T Cell Lymphoma 526
Mycosis Fungoides 526
Mycosis Fungoides Vaiiants 530
Szaiy Syndiome 534
Lymphomatoid Papulosis 535
Cutaneous Anaplastic Large Cell Lymphomas 535
Cutaneous B Cell Lymphoma 537
Kaposi Sarcoma 538
SECTICN 21
5KIN DI5EA5E5 IN OkCAN AND ONE MAkkOW IkAN5PLANIAIION 544
Most Common Infections Associated with Organ Transplantation 544
Skin Cancers Associated with Organ Transplantation 544
Graft-versus-Host Disease 546
Acute Cutaneous GVHR 546
Chionic Cutaneous GVHR 550
SECTICN 22
ADVEk5E CUIANEOU5 DkUC kEACIION5 552
Clinical Types of Adverse Cutaneous Drug Reactions 553
Exanthematous Drug Reactions 557
Pustular Eruptions 561
Drug-Induced Acute Urticaria, Angioedema, Edema, and Anaphylaxis 563
Fixed Drug Eruption 566
Drug Hypersensitivity Syndrome 568
Drug-Induced Pigmentation 570
Pseudoporphyria 574
ACDR-Related Necrosis 575
ACDR Related to Chemotherapy 579
C0|IE|I' xv
SECTICN 23
DI5OkDEk5 OF P5YCHIAIkIC EIIOLOCY 582
Classincation of Disorders of Psychiatric Etiology 582
Dysmorphic Syndrome 582
Delusions of Parasitosis 582
Neurotic Excoriations and Trichotillomania 583
Factitious Syndromes (Mnchausen Syndrome) 586
Cutaneous Signs of Injecting Drug Use 587
PAkI III
DISEASES DUE TC MICRCIAL ACENTS
SECI|0N 24
8ACIkIAI INFCII0NS INv0IvINC Ih SkIN 590
Stuphy|vcvccus uureus : Infections and Intoxications 591
Streptvcvccus Pyvgenes : Infections and Intoxications 591
Superncial Bacterial Epidermal Colonizations and Infections 592
Eiythiasma 592
Pitted Keiatolysis 594
Tiichomycosis 595
Nonspecifc Inteitiigo 596
Pyoderma 597
Impetigo and Ecthyma 597
Abscess, Fuiuncle, and Caibuncle 605
Soft Tissue Infections 609
Eiysipelas and Cellulitis 609
Neciotizing Soft Tissue Infections 618
Lymphangitis 619
Wound Infections 621
Gram-Positive Coccal Infections Associated with Toxin Production 625
(Intoxications)
Staphylococcal Scalded-Skin Syndiome 626
Toxic Shock Syndiome 629
Scailet Fevei 631
Gram-Positive Bacillary Infections Associated with Toxin Production 634
Cutaneous Anthiax 634
Cutaneous Diphtheiia 637
Tetanus 637
Infective Endocarditis, Sepsis, and Septic Shock 638
Infective Endocaiditis 638
Sepsis and Septic Shock 642
Nessera 646
Nessera menngJs Infection 646
Nessera gonorr|oeae Infections 649
NeIsserIu gvnvrrhveue : Local Infections 650
Disseminated Gonococcal Infection 652
C0|IE|I' xv
Gram-Negative Infections 654
Barone||a Infections 654
Cat-Sciatch Disease 655
Barone||a Infections in HIV/AIDS 658
Bacillaiy Angiomatosis 658
Tulaiemia 659
PseuJomonas Species 662
Cutaneous PseuJomonas aerugnosa Infections 662
Mycobacterial Infections 664
Classifcation of Mycobacteiia and Mycobacteiial Infections 665
Lepiosy 665
Cutaneous Tubeiculosis 671
Nontubeiculous Mycobacteiial Infections 677
Myto|aterum marnum Infection 678
Myto|aterum u|terans Infection 680
Myto|aterum [oruum Complex Infections 682
Lyme Borreliosis 684
SECI|0N 25
FNCAI INFCII0NS 0F Ih SkIN AN0 hAIk 692
Superncial Fungal Infections 692
Dermatophytoses 693
Deimatophytoses of Epideimis 697
Tnea PeJs 697
Tnea Manuum 701
Tnea Crurs 703
Tnea Corors 704
Tnea Fata|s 707
Tnea Intogno 708
Deimatophytoses of Haii 708
Tnea Cas 709
Tnea Bar|ae 715
Dermao|yt Fo||tu|s 716
Ma,ott| Cranu|oma 717
Candidiasis 718
Cutaneous Candidiasis 720
Oiophaiyngeal Candidiasis 724
Genital Candidiasis 727
Candidiasis of Nail Appaiatus 730
Chionic Mucocutaneous Candidiasis 730
Mu|ussezIu Infections 732
Pityiiasis Veisicoloi 732
Ma|asse:a Folliculitis 734
TrIchvspvrvn Infections 736
Tinea Nigra 736
Invasive and Disseminated Fungal Infections 737
Subcutaneous Mycoses 737
Myteoma 738
C|romomytoss 742
Sorort|oss 744
C0|IE|I' xv
Systemic Fungal Infections with Dissemination to Skin 747
DssemnaeJ Cryotottoss 747
Hso|asmoss 749
B|asomytoss : Cutaneous Manifestations 753
DssemnaeJ CottJoJomytoss 755
DssemnaeJ Pent||noss 758
Pent||noss 758
tue CanJJema anJ DssemnaeJ CanJJass 758
SECI|0N 26
kICkIISIAI INFCII0NS 760
Tick-Borne Spotted Fever 761
Rot|y Mounan SoeJ Feer 761
Tt|-Borne Ty|us 765
Rickettsialpox 768
Louse-Borne Typhus 768
SECI|0N 21
vIkAI INFCII0NS 0F SkIN AN0 MC0SA 770
Poxvirus Infections 770
Molluscum Contagiosum 771
Human Oif 776
Milkei`s Nodules 778
Smallpox 779
Cowpox, Monkeypox, Tanapox 782
Cowox 782
Mon|eyox 783
Tanaox 783
Vaccinia 783
Human Papillomavirus Infections 787
Human Papillomaviius Cutaneous Infections 788
Infectious Exanthems 795
Rubella 798
Measles 800
Enteioviial Infections 803
Hand-Foot-and-Mouth Disease 803
Heipangina 804
Eiythema Infectiosum 806
Gianotti-Ciosti Syndiome 809
Dengue Fevei 810
Human Herpesviruses 813
Herpes Simplex Virus (HSV) Infection 813
Nongenital Heipes Simplex Viius Infection 818
Neonatal Heipes Simplex Viius Infection 823
Heipes Simplex Viius: Widespiead Cutaneous Infection 825
Associated with Cutaneous Immunocompiomise
Heipes Simplex Viius: Infections Associated with Systemic 827
Immunocompiomise
C0|IE|I' xv
Varicella Zoster Virus Infections 831
Vaiicella 833
Heipes Zostei 837
Vaiicella Zostei Viius Infections in the Immunocompiomised 846
Host
Human Herpes Virus-6 and -7 Infections: Exanthema Subitum 850
SECI|0N 28
AkIhk0F00 INSCI 8IIS, SIINCS, AN0 CIAN0S INFCII0NS 852
Cutaneous Reactions to Arthropod Bites 852
Pediculosis 860
Pediculosis Capitis 861
Pediculosis Coipoiis 863
Pediculosis Pubis (Phthiiiasis) 865
Mite Bites and Infestations 868
Scabies 868
Cutaneous Laiva Migians 876
Water-Associated Infections and Infestations 879
Ceicaiial Deimatitis 880
Seabathei`s Eiuption 880
Envenomations Caused by Cnidaiia 882
Injuiies Caused by Echinodeims 882
SECI|0N 29
SSIMIC FAkASIIIC INFCII0NS 884
Cutaneous and Mucocutaneous Leishmaniasis 884
Trypanosomiasis 893
Ameiican Tiypanosomiasis 893
Human Afiican Tiypanosomiasis 894
Cutaneous Amebiasis and Acanthamebiasis 895
Cutaneous Amebiasis 895
Cutaneous Acanthamebiasis 895
SECI|0N 50
SXAII IkANSMIII0 INFCII0NS 896
Human Papillomavirus: Mucosal Infections 900
Exteinal Genital Waits 901
HPV: Squamous Cell Caicinoma in Situ 908
and Invasive SCC of Anogenital Skin
Herpes Simplex Virus: Genital Infections 912
Syphilis 919
Piimaiy Syphilis 922
Secondaiy Syphilis 924
Latent Syphilis 928
Teitiaiy/Late Syphilis 930
Congenital Syphilis 931
HuemvphI|us ducreyI : Chancroid 931
Donovanosis 934
C0|IE|I' xx
Ch|umydIu truchvmutIs Infections 936
Localized C. rat|omas Infection 937
Invasive C. rat|omas Infection: Lymphogianuloma veneieum 939
SECI|0N 51
hMAN kIk0vIkAI INFCII0NS AN0 MC0CIAN0S
MANIFSIAII0NS 0F hIv[AI0S 0ISAS 942
Global HIV/AIDS Pandemic 942
HIV/AIDS Disease and AIDS 942
Acute HIV/AIDS Syndrome 946
Eosinophilic Folliculitis 948
Oral Hairy Leukoplakia 950
Adverse Cutaneous Drug Eruptions in HIV/AIDS Disease 951
Abnormalities of Fat Distribution 953
Variations of Common Mucocutaneous Disorders in HIV/AIDS Disease 955
PAkI IV
SKIN SICNS CF HAIR, NAIL, AND MUCCSAL DISCRDERS
SECI|0N 52
0IS0k0kS 0F hAIk F0IIICIS AN0 kIAI0 0IS0k0kS 961
Biology of Hair Growth Cycles 962
Classincation of Alopecia 964
Hair Loss: Alopecia 965
Paern Har |oss 965
|oeta reaa 971
Te|ogen E[[uum 975
nagen E[[uum 979
Cicatricial or Scarring Alopecia 981
Excess Hair Growth 989
Hiisutism 989
Hypeitiichosis 992
Infectious Folliculitis 993
SECI|0N 55
0IS0k0kS 0F Ih NAII AFFAkAIS 1000
Normal Nail Apparatus 1000
Local Disorders of Nail Apparatus 1002
Chionic Paionychia 1002
Onycholysis 1003
Gieen Nail Syndiome 1004
Onychauxis and Onychogiyphosis 1004
Psychiatric Disorders 1005
Nail Apparatus Involvement of Cutaneous Diseases 1006
Psoiiasis 1006
Lichen Planus 1008
C0|IE|I' xx
Alopecia Aieata 1008
Daiiei Disease (Daiiei-White Disease, Keiatosis 1010
Folliculaiis)
Chemical Irritant or Allergic Damage or Dermatitis 1010
Neoplasms of the Nail Apparatus 1011
Myxoid Cysts of Digits 1011
Longitudinal Melanonychia 1011
Nail Matiix Nevi 1012
Aciolentiginous Melanoma 1012
Squamous Cell Caicinoma 1012
Infections of the Nail Apparatus 1014
Bacteiial Infections 1014
tue Paronyt|a 1014
Fe|on 1014
Fungal Infections and Onychomycosis 1014
CanJJa Onyt|a 1015
Tnea Unguum/Onyt|omytoss 1016
Nail Signs of Multisystem Diseases 1021
Tiansveise oi Beau Lines 1021
Leukonychia 1021
Yellow Nail Syndiome 1022
Peiiungual Fibioma 1022
Splintei Hemoiihages 1024
Nail Fold/Peiiungual Eiythema and Telangiectasia 1024
Pteiygium Inveisum Unguium 1024
Systemic Amyloidosis 1024
Koilonychia 1026
Clubbed Nails 1026
Drug-Induced Nail Changes 1027
SECI|0N 54
0IS0k0kS 0F Ih M0Ih 1028
Diseases of the Lips 1028
Angular Cheilitis (Perlche) 1028
Conditions of the Tongue 1028
Fissuied Tongue 1028
Black oi White Haiiy Tongue 1029
Oial Haiiy Leukoplakia 1029
Migiatoiy Glossitis 1030
Diseases of the Gingiva, Periodontium, and Mucous Membranes 1030
Gingivitis and Peiiodontitis 1030
Eiosive Gingivostomatitis 1030
Lichenoid Mucositis 1030
Lichen Planus 1031
Acute Neciotizing Ulceiative Gingivitis 1032
Gingival Hypeiplasia 1032
Aphthous Ulceration 1034
Leukoplakia 1036
Erythematous Lesions and/or Leukoplakia 1037
Premalignant and Malignant Neoplasms 1038
Dysplasia and Squamous Cell Caicinoma in Situ 1038
C0|IE|I' xx
Oial Invasive Squamous Cell Caicinoma 1038
Oial Veiiucous Caicinoma 1039
Oiophaiyngeal Melanoma 1040
Submucosal Nodules 1040
Mucocele 1040
Iiiitation Fibioma 1041
Cutaneous Odontogenic (Dental) Abscess 1042
Cutaneous Disorders Involving the Mouth 1043
Pemphigus Vulgaiis (PV) 1043
Paianeoplastic Pemphigus 1043
Bullous Pemphigoid 1043
Cicatiicial Pemphigoid 1044
Systemic Diseases Involving the Mouth 1044
Lupus Eiythematosus 1044
Behet Disease 1044
Stevens-Johnson Syndiome / Toxic 1044
Epideimoneciolysis
Adveise Diug Reactions 1044
SECI|0N 55
0IS0k0kS 0F Ih CNIIAIIA, FkINM, AN0 ANS 1046
Variants of Genital Anatomy 1046
Peaily Penile Papules 1046
Sebaceous Gland Piominence 1046
Angiokeiatoma 1047
Chionic Pain Syndiome 1047
Disorders Specinc to Genital Anatomy 1048
Scleiosing Lymphangitis of Penis 1048
Chionic Lymphedema of the Genitalia 1048
Plasma Cell Balanitis and Vulvitis 1048
Phimosis, Paiaphimosis, Balanitis Xeiotica Obliteians 1050
Mucocutaneous Disorders 1051
Genital(Penile/Vulvai/Anal) Lentiginoses 1051
Vitiligo and Leukodeima 1052
Psoiiasis Vulgaiis 1052
Lichen Planus 1054
Lichen Nitidus 1055
Lichen Scleiosus 1055
Migiatoiy Neciolytic Eiythema 1058
Genital Aphthous Ulceiations 1058
Eczematous Dermatitis 1059
||ergt Conat Dermas 1059
ot Dermas, Lt|en Sm|ex C|rontus, Prurus n 1060
Fixed Diug Eiuption 1061
Premalignant and Malignant Lesions 1062
Squamous Cell Caicinoma in Situ 1062
HPV-Induced Intiaepithelial Neoplasia and 1063
Squamous Cell Caicinoma in Situ
Invasive Anogenital Squamous Cell Carcinoma 1064
Invasive SCC of Penis 1064
C0|IE|I' xx
Invasive SCC of Vulva 1064
Invasive SCC of Cutaneous Anus 1064
Genital Veiiucous Caicinoma 1064
Malignant Melanoma of the Anogenital Region 1065
Extiamammaiy Paget Disease 1066
Kaposi Saicoma 1066
Anogenital Infections 1066
SECI|0N 56
CNkAIII0 FkkIIS WIIh0I SkIN ISI0NS 1068
AFFN0ICS 1072
APPENDIX A: Travel" Dermatology 1072
APPENDIX B: Dermatologic Manifestations of Diseases 1072
Inicted by Biologic Warfare/Bioterrorism
APPENDIX C: Chemical Bioterrorism and Industrial Accidents 1074
APENDIX D: Drug Use in Pregnancy 1075
Iodex 1077
Time is change; we measure its passage by how much things alter."
NaJne CorJmer
The Frs EJon of this book appeaied 26 yeais ago (1983) and has been expanded paii passu with
the majoi developments that have occuiied in deimatology ovei the past two and a half decades.
Deimatology is now one of the most sought aftei medical specialties because the buiden of skin
disease has become enoimous and the many new innovative theiapies available today attiact laige
patient populations.
The Co|or |as anJ Synoss o[ C|nta| Dermao|ogy has been used by thousands of piimaiy caie
physicians, deimatologists, inteinists, and othei health caie piovideis piincipally because it facilitates
deimatologic diagnosis by pioviding coloi photogiaphs of skin lesions and, juxtaposed, a succinct
summaiy outline of skin disoideis as well as the skin signs of systemic diseases.
The Sixth Edition has been extensively ievised, iewiitten, and expanded by the addition of new
sections. Roughly 80% of the old images have been ieplaced by new ones and additional images
have been added. Theie is a complete update of etiology, pathogenesis, management and theiapy
and theie is now an online veision. The pievious edition of the |as has been tianslated into seven
languages.
FkFAC
Oui secietaiy, Renate Kosma, woiked haid to meet the demands of the authois. In the piesent
McGiaw-Hill team, we appieciated the counsel of Scott Giillo, Vice Piesident and Publishei;
Anne M. Sydoi, Executive Editoi; Maiiapaz Ramos Englis, Senioi Managing Editoi; Phil Galea,
Senioi Pioduction Managei, who expeitly managed the pioduction piocess; Lindsey Zahuianec
and M. Loiiaine Andiews, the editoiial assistants, foi theii invaluble help; . Alan Bainett and
Alicia Fox, of Alan Bainett Design; and Susan Gilbeit, foi hei lovely line illustiations.
But the majoi foice behind this edition and pievious editions was Maiiapaz Ramos Englis whose
good natuie, good judgment, loyalty to the authois, and, most of all, patience, guided the authois to
make an even bettei book.
ACkN0WI0CMNI
INIk00CII0N
The Co|or |as anJ Synoss o[ C|nta| Der-
mao|ogy is pioposed as a field guide" to the
iecognition of skin disoideis and theii man-
agement. The skin is a tieasuiy of impoitant
lesions that can usually be iecognized clinically.
Gioss moiphology in the foim of skin lesions
iemains the haid coie of deimatologic diag-
nosis, and theiefoie this text is accompanied
by ovei 900 coloi photogiaphs illustiating skin
diseases, skin manifestations of inteinal dis-
eases, infections, tumois, and incidental skin
findings in otheiwise well individuals. We have
endeavoied to include infoimation ielevant
to gendei deimatology and a laige numbei of
images showing skin disease in diffeient ethnic
populations. This |as coveis the entiie field of
clinical deimatology but does not include veiy
iaie syndiomes oi conditions. With iespect to
these the ieadei is iefeiied to anothei McGiaw-
Hill Publication: F:art|'s Dermao|ogy n
Cenera| MeJtne , 7th ed., 2008, edited by Klaus
Wolff, Lowell A. Goldsmith, Stephen I. Katz,
Baibaia A. Gilchiest, Amy S. Pallei, and David
J. Leffell.
This text is intended foi all physicians and
othei health caie piovideis, including medi-
cal students, deimatology iesidents, inteinists,
oncologists, and infectious disease specialists
dealing with diseases with skin manifestations.
Foi non-deimatologists, it is advisable to stait
with Appioach to Deimatologic Diagnosis"
and Outline of Deimatologic Diagnosis," be-
low, to familiaiize themselves with the piinci-
ples of deimatologic nomenclatuie and lines of
thought.
The |as is oiganized in 4 Paits, subdi-
vided into 36 Sections, and theie aie 4 shoit
Appendices. Each section has a coloi label that
is ieflected by the bai on the top of each page.
This is to help the ieadei to find his oi hei beai-
ings iapidly when leafing thiough the book.
Also, the fiist page of each section caiiies an
icon," i.e., a small photogiaph of a condition
that is iepiesentative foi that paiticulai section.
Each disease is labeled with little symbols to
piovide fiist-glance infoimation on incidence
(squaies) and moibidity (ciicles).
iaie low moibidity
not so common consideiable moibidity
common seiious
Foi instance, the symbols foi melanoma
aie meant to indicate that melanoma is com-
mon and seiious. Theie aie also some vaiiations
in this symbology. Foi instance, means
that the disease is iaie but may be common in
specific populations oi in endemic iegions oi in
epidemics. Anothei example indicates
that the disease causes consideiable moibid-
ity and may become seiious. In addition, each
disease is labeled with the iespective ICD9/10
codes.
Since, foi ieasons of space, not all manifesta-
tions of skin diseases and vaiiations theieof can
be shown in this piinted veision of Co|or |as
anJ Synoss o[ C|nta| Dermao|ogy, theie is
an online veision of the book that contains
pictuie galleiies" of most of the conditions
discussed heie. The symbol in the text iefeis
to such a pictuie galleiy in the online veision.
So, foi instance, if ieading about psoiiasis and
finding this symbol , look up the psoiiasis
pictuie galleiy in the online veision foi addi-
tional clinical images.
xxv
Theie aie two distinct clinical situations iegaid-
ing the natuie of skin changes:
The skin changes aie ntJena| findings in
we|| and || individuals noted duiing the iou-
tine geneial physical examination
Bums anJ ||ems|es ": many asympto-
matic lesions that aie medically incon-
sequential may be piesent in well and ill
peisons and aie not the ieason foi the visit
to the physician; eveiy geneial physician
should be able to iecognize these lesions to
diffeientiate them fiom asymptomatic but
impoitant, e.g., malignant, lesions.
Imoran s|n |esons no noted by the
patient but that must not be oveilooked by
the physician: e.g., atypical nevi, melanoma,
basal cell caicinoma, squamous cell caici-
noma, caf-au-lait macules in von Reck-
linghausen disease, xanthomas.
The skin changes aie the t|e[ tom|an of
the patient
Minoi" pioblems: e.g., localized itchy
iash, iash," iash in gioin, nodules such as
common moles, seboiiheic keiatoses.
4-S": s eiious s kin s igns in s ick p atients
SkI0S SkIN SICNS IN SICk FAIINIS
Cenera|ited red rash with fever
\||+| e\+u||er
k|c|e|||+| e\+u||er
||u e|up||ou
B+c|e||+| |u|ec||ou W||| |o\|u p|oduc||ou.
Cenera|ited red rash with b|isters and prominent
mouth |esions
E|,||er+ ru||||o|re (r+jo|)
Io\|c ep|de|r+| uec|o|,|
|erp||u
I.
II.
Bu||ou perp||o|d
||u e|up||ou
Cenera|ited red rash with pustu|es
|u|u|+| po||+| (.ou /ur|uc|)
||u e|up||ou
Cenera|ited rash with vesic|es
||er|u+|ed |e|pe |rp|e\
Ceue|+||/ed |e|pe /o|e|
\+||ce||+
||u e|up||ou
Cenera|ited red rash with sca|in over who|e
body
E\|o||+||.e e|,|||ode|r+
Cenera|ited whea|s and soft tissue swe||in
u|||c+||+ +ud +u|oeder+
Cenera|ited purpura
I||or|oc,|opeu|+
|u|pu|+ |u|r|u+u
||u e|up||ou
Cenera|ited purpura that can be pa|pated
\+cu||||
B+c|e||+| eudoc+|d|||
Nu|tip|e skin infarcts
\eu|uococcer|+
Couococcer|+
||er|u+|ed |u||+.+cu|+| co+u|op+||,
Loca|ited skin infarcts
C+|c|p|,|+\|
A||e|oc|e|o| o||||e|+u
A||e|oer|o||/+||ou
w+||+||u uec|o|
Au||p|op|o||p|d +u|||od, ,ud|ore
|acia| inf|ammatory edema with fever
E|,|pe|+
|upu e|,||er+|ou
In contiast to othei fields of clinical medicine,
patients should be examined befoie a detailed
histoiy is taken because patients can see theii
lesions and thus often piesent with a histoiy
that is flawed with theii own inteipietation of
the oiigin oi causes of the skin eiuption. Also,
diagnostic accuiacy is highei when objective ex-
amination is appioached without pieconceived
ideas. Howevei, a histoiy should always be ob-
tained but if taken duiing oi aftei the visual and
physical examination, it can be stieamlined and
moie focused following the objective findings.
AFFk0ACh I0 0kMAI0I0CIC 0IACN0SIS
INIk00CII0N
0IIIN 0F 0kMAI0I0CIC 0IACN0SIS
xxv
Thus, iecognizing, analysing, and piopeily in-
teipieting skin lesions aie the sine qua non of
deimatologic diagnosis.
FhSICAI XAMINAII0N
Appearaoce Uncomfoitable, toxic," well
vta| Sos Pulse, iespiiation, tempeiatuie
Sko: "Iearoo to kead" The entiie skin
should be inspected and this should include
mucous membianes, genital and anal iegions,
as well as haii and nails and peiipheial lymph
nodes. Reading the skin is like ieading a text.
The basic skin lesions aie like the letteis of the
alphabet: theii shape, coloi, maigination, and
othei featuies combined will lead to woids, and
theii localization and distiibution to a sentence
oi paiagiaph. The pieiequisite of deimatologic
diagnosis is thus the iecognition of (1) the type
of skin lesion, (2) the coloi, (3) maigination, (4)
consistency, (5) shape, (6) aiiangement, and (7)
distiibution of lesions.
kecooto Ietters: Iypes oI Sko Iesoos
Matu|e (Latin: matu|a, spot") A macule
is a ciicumsciibed aiea of change in skin
coloi without elevation oi depiession. It
is thus not palpable. Macules can be well-
and ill-defined. Macules may be of any size
oi coloi (Image I-1). White, as in vitiligo;
biown, as in caf-au-lait spots ; blue, as in
Mongolian spots ; oi ied, as in peimanent
vasculai abnoimalities such as poit-wine
stains oi capillaiy dilatation due to inflam-
mation (eiythema ). Piessuie of a glass slide
(Jastoy ) on the boidei of a ied lesion de-
tects the extiavasation of ied blood cells. If
the iedness iemains undei piessuie fiom the
slide, the lesion is puipuiic, that is, iesults
fiom extiavasated ied blood cells; if the ied-
ness disappeais, the lesion is due to vasculai
dilatation. A iash consisting of macules is
called a matu|ar exan|em .
Pau|e (Latin: au|a, pimple") A papule
is a supeificial, elevated, solid lesion, genei-
ally consideied <0.5 cm in diametei. Most of
it is elevated above, iathei than deep within,
the plane of the suiiounding skin (Image
I-2). A papule is palpable. It may be well- oi
ill-defined. In papules the elevation is caused
by metabolic oi locally pioduced deposits , by
localized cellulai infiltiates, inflammatoiy oi
noninflammatoiy , oi by hypeiplasia of lo-
cal cellulai elements . Supeificial papules aie
shaiply defined. Deepei deimal papules have
indistinct boideis. Papules may be dome-
shaped, cone-shaped oi flat-topped (as in
lichen planus) oi consist of multiple, small,
closely packed, piojected elevations that aie
known as a egeaon ( Image I-2 ). A iash
consisting of papules is called a au|ar ex-
an|em . Papulai exanthems may be giouped
(lichenoid") oi disseminated (dispeised).
Confluence of papules leads to the develop-
ment of laigei, usually flat-topped, ciicum-
sciibed, plateau-like elevations known as
plaques (Fiench: |aque, plate"). See below.
IMAC I-1 Macu|e
INIk00CII0N
xxv
P|aque A plaque is a plateau-like elevation
above the skin suiface that occupies a ielatively
laige suiface aiea in compaiison with its height
above the skin (Image I-3). It is usually well de-
fined. Fiequently it is foimed by a confluence
of papules, as in psoiiasis. Lt|en[taon is a
less well-defined, laige plaque wheie the skin
appeais thickened and the skin maikings aie
accentuated. Lichenification occuis in atopic
deimatitis, eczematous deimatitis, psoiiasis,
lichen simplex chionicus and mycosis fun-
goides. A at| is a baiely elevated plaque-a
lesion fitting between a macule and a plaque-
as in paiapsoiiasis oi Kaposi saicoma.
NoJu|e (Latin: noJu|us, small knot") A nod-
ule is a palpable, solid, iound oi ellipsoidal
lesion that is laigei than a papule (Image I-4)
and may involve the epideimis , deimis , oi sub-
cutaneous tissue. The depth of involvement and
the size diffeientiate a nodule fiom a papule.
Nodules iesult fiom inflammatoiy infiltiates ,
neoplasms , oi metabolic deposits in the deimis
oi subcutaneous tissue. Nodules may be well de-
fined (supeificial) oi ill defined (deep); if local-
ized in the subcutaneous tissue, they can often
be bettei felt than seen. Nodules can be haid oi
soft upon palpation. They may be dome-shaped
and smooth oi may have a waity suiface oi cia-
tei-like cential depiession.
V|ea| A wheal is a iounded oi flat-topped,
pale ied papule oi plaque that is chaiacteiisti-
cally evanescent, disappeaiing within 24-48 h
(Image I-5). It is due to edema in the papillaiy
body of the deimis. Wheals may be iound,
gyiate, oi iiiegulai with pseudopods-chang-
ing iapidly in size and shape due to shifting
papillaiy edema. A iash consisting of wheals
is called an urtara| exan|em oi urtara.
IMAC I-2 Fapu|e
IMAC I-3 F|aque
INIk00CII0N
xxx
Vest|e-Bu||a ( B|ser ) (Latin: estu|a , little
bladdei"; |u||a , bubble") A vesicle (<0.5
cm) oi a bulla (>0.5 cm) is a ciicumsciibed,
elevated, supeificial cavity containing fluid
(Image I-6). Vesicles aie dome-shaped (as in
contact deimatitis, deimatitis heipetifoimis),
umbilicated (as in heipes simplex), oi flaccid
(as in pemphigus). Often the ioof of a vesi-
cle/bulla is so thin that it is tianspaient, and
the seium oi blood in the cavity can be seen.
Vesicles containing seium aie yellowish; those
containing blood fiom ied to black. Vesicles
and bullae aiise fiom a cleavage at vaiious
levels of the supeificial skin; the cleavage may
be subcoineal oi within the visible epideimis
(i.e., intiaepideimal vesication) oi at the
epideimal-deimal inteiface (i.e., subepidei-
mal), as in Image I-6. Since vesicles/bullae
aie always supeificial they aie always well de-
fined. A iash consisting of vesicles is called a
estu|ar exan|em ; a iash consisting of bullae
a |u||ous exan|em.
IMAC I-4 Nodu|e
IMAC I-5 Whea|
IMAC I-6 vesc|e
INIk00CII0N
xxx
Pusu|e (Latin: usu|a , pustule") A pus-
tule is a ciicumsciibed, supeificial cavity of
the skin that contains a puiulent exudate
(Image I-7), which may be white, yellow,
gieenish-yellow, oi hemoiihagic. Pustules
thus diffei fiom vesicles in that they aie not
cleai but have a tuibid content. This piocess
may aiise in a haii follicle oi independently.
Pustules may vaiy in size and shape. Pus-
tules aie usually dome-shaped, but follicu-
lai pustules aie conical and usually contain
a haii in the centei. The vesiculai lesions
of heipes simplex and vaiicella zostei vi-
ius infections may become pustulai. A iash
consisting of pustules is called a usu|ar
exan|em.
Cruss (Latin: trusa , iind, baik, shell")
Ciusts develop when seium, blood, oi puiu-
lent exudate diies on the skin suiface (Image
I-8). Ciusts may be thin, delicate, and fiiable
( ) oi thick and adheient . Ciusts aie yellow
when foimed fiom diied seium; gieen oi
yellow-gieen when foimed fiom puiulent
exudate; oi biown, daik ied, oi black when
foimed fiom blood. Supeificial ciusts occui
as honey-coloied, delicate, glistening paiticu-
lates on the suiface and aie typically found
in impetigo. When the exudate involves the
entiie epideimis, the ciusts may be thick and
adheient , and if it is accompanied by neciosis
of the deepei tissues (e.g., the deimis), the
condition is known as et|yma .
IMAC I-T Fustu|e
IMAC I-8 Crust
Sta|es (squames) (Latin: squama , scale")
Scales aie flakes of stiatum coineum (Image
I-9). They may be laige (like membianes ,
tiny (like dust), pityiiasifoim (Gieek: yron ,
bian"), adheient, oi loose. A iash consisting
of papules with scales is called a au|osqua-
mous exan|em.
INIk00CII0N
xxx
Eroson An eiosion is a defect only of the epi-
deimis, not involving the deimis (Image I-10);
in contiast to an ulcei, which always heals with
scai foimation (see below), an eiosion heals
without a scai. An eiosion is shaiply defined
and is ied and oozes. Theie aie supeificial
eiosions, which aie subcoineal oi iun thiough
the epideimis, and deep eiosions, the base of
which is the papillaiy body (Image I-10). Ex-
cept foi physical abiasions, eiosions aie always
the iesult of intiaepideimal oi subepideimal
cleavage and thus of vesicles oi bullae.
U|ter (Latin: u|tus , soie") An ulcei is a skin
defect that extends into the deimis oi deepei
(Image I-11) into the subcutis and always oc-
cuis within pathologically alteied tissue. An
ulcei is theiefoie always a secondaiy phenom-
enon. The pathologically alteied tissue giving
iise to an ulcei is usually seen at the boidei
oi the base of the ulcei and is helpful in de-
teimining its cause. Othei featuies helpful in
this iespect aie whethei boideis aie elevated,
undeimined, haid, oi soggy; location of the ul-
cei; dischaige; and any associated topogiaphic
featuies, such as nodules, exoiiations, vaiicosi-
ties, haii distiibution, piesence oi absence of
sweating, and aiteiial pulses. Ulceis always
heal with scai foimation.
Star A scai is the fibious tissue ieplacement
of the tissue defect by pievious ulcei oi a
wound. Scais can be hypeitiophic and haid
(Image I-12 ) oi atiophic and soft with a thin-
ning oi loss of all tissue compaitments of the
skin (Image I-12 ).
ro|y This iefeis to a diminution of
some oi all layeis of the skin (Image I-
13). Epideimal atiophy is manifested by a
thinning of the epideimis, which becomes
tianspaient, ievealing the papillaiy and sub-
papillaiy vessels ; theie aie loss of skin tex-
tuie and cigaiette papei-like wiinkling. In
deimal atiophy theie aie loss of connective
tissue of the deimis and depiession of the
lesion ( Image I-13 ).
IMAC I-9 Sca|e
IMAC I-10 rosoo
INIk00CII0N
xxx
Cys A cyst is a cavity containing liquid
oi solid oi semisolid (Image I-14) mateiials
and may be supeificial oi deep. Visually it
appeais like a spheiical, most often dome-
shaped papule oi nodule, but upon palpation
it is iesilient. It is lined by an epithelium and
often has a fibious capsule; depending on its
contents it may be skin coloied, yellow, ied,
oi blue. An epideimal cyst pioducing keiati-
naceous mateiial and a pilai cyst that is lined
by a multilayeied epithelium aie shown in
Image I-14.
IMAC I-11 |cer
IMAC I-12 Scar
IMAC I-13 Atrophy
INIk00CII0N
xxx
Shapo Ietters oto Words: Further
Charactertatoo oI IdeotIed Iesoos
Co|or Pink, ied, puiple puipuiic lesions
do not blanch with piessuie with a glass slide
(diascopy)], white, tan, biown, black, blue,
giey, yellow. The coloi can be unifoim oi
vaiiegated.
Margnaon Well defined (can be tiaced
with the tip of a pencil), ill defined.
S|ae Round, oval, polygonal, polycy-
clic, annulai (iing-shaped), iiis, seipiginous
(snakelike), umbilicated.
Pa|aon Considei (1) tonssenty (soft, fiim,
haid, fluctuant, boaidlike); (2) Jeaon n
emeraure (hot, cold); and (3) mo||y . Note
piesence of enJerness , and estimate the Je|
of the lesion (i.e., deimal oi subcutaneous).
Formo Seoteoces aod oderstaodo the Iext:
va|uatoo oI Arraoemeot, Fatteros, aod
0strbutoo
Num|er Single oi multiple lesions.
rrangemen Multiple lesions may be (1)
groueJ : heipetifoim, aicifoim, annulai, ie-
ticulated (net-shaped), lineai, seipiginous
(snakelike); oi (2) JssemnaeJ : scatteied
disciete lesions.
Con[|uente Yes oi no.
Dsr|uon Considei (1) exen : iso-
lated (single lesions), localized, iegional,
geneialized, univeisal, and (2) aern : sym-
metiic, exposed aieas, sites of piessuie, intei-
tiiginous aiea, folliculai localization, iandom,
following deimatomes oi Blaschko lines.
Table I-1 piovides an algoiithm showing how
to pioceed.
hISI0k
0emoraphcs Age, iace, sex, occupation.
hstory
1. Constitutional symptoms
Acute illness" syndiome: headaches, chills,
feveiishness, weakness
Chionic illness" syndiome: fatigue, weak-
ness, anoiexia, weight loss, malaise
2. History of skin lesions. Seven key ques-
tions:
When: Onset
Wheie: Site of onset
Does it itch oi huit: Symptoms
How has it spiead (pattein of spiead):
Evolution
How have individual lesions changed:
Evolution
Piovocative factois: Heat, cold, sun, exei-
cise, tiavel histoiy, diug ingestion, pieg-
nancy, season
Pievious tieatment(s): Topical and sys-
temic,
3. General history of present illness as indi-
cated by clinical situation, with particular
attention to constitutional and prodromal
symptoms
4. Past medical history
Opeiations
Illnesses (hospitalized:)
Alleigies, especially diug alleigies
Medications (piesent and past)
Habits (smoking, alcohol intake, diug
abuse)
Atopic histoiy (asthma, hay fevei, ec-
zema)
IMAC I-14 Cyst
INIk00CII0N
xxxv
|deot|Iy |es|oos
|s |es|oo so||tary or are there m0|t|p|e |es|oos?
Nac0|e
portwiue staiu*
er]thema migraus
Pap0|elood0|e
dermal uevus
oasal cell
carciuoma
uodular
melauoma
P|ag0e
licheu simplex
chrouicus
Boweu disease
spreadiug
melauoma
0|cer
oasal cell
carciuoma
diaoetic ulcer
primar] chaucre
of s]philis
Nac0|ar
solar
leutigiues
eruptiou
Nac0|ar
viral exauthem
drug eruptiou
P|ag0e
psoriasis
m]cosis
fuugoides
hod0|ar
metastatic
caucer
P0st0|ar
folliculitis
oaroae
herpes zoster
impetigo
*Bulleted couditious are examples
Pap0|ar
coud]lomata
accumiuata
s]riugomas
licheu plauus
Ves|c0|arl
b0||o0s
herpes
zoster
herpes
simplex
Pap0|ar
psoriasis
licheu plauus
secoudar] s]philis
Ves|c0|arlb0||o0s
varicella
oullous
pemphigoid
P0st0|ar
pustular
psoriasis
smallpox
hod0|ar
metastatic
melauoma
lipomas
Loca||zed
N0|t|p|e
6eoera||zed
So||tary
IA8I I-1 A|orithm for Eva|uatin Skin Lesions
5. Family medical history (particularly of
psoriasis, atopy, melanoma, xanthomas, tu-
berous sclerosis)
6. Social history, with particular reference
to occupation, hobbies, exposures, travel,
injecting drug use
7. Sexual history: history of risk factors of
HIV: blood transfusions, IV drugs, sexually
active, multiple partners, sexually trans-
mitted disease?
kvIW 0F SMFI0MS
This should be done as indicated by the clinical
situation, with paiticulai attention to possible
connections between signs and disease of othei
oigan systems (e.g., iheumatic complaints, my-
algias, aithialgias, Raynaud phenomenon, sicca
symptoms).
INIk00CII0N
xxxv
SFCIAI IChNIS S0 IN CIINICAI
XAMINAII0N
Magn[taon w| |anJ |ens. To examine le-
sions foi fine moiphologic detail, it is necessaiy
to use a magnifying glass (hand lens) (7) oi
a binoculai micioscope (5 to 40). Magni-
fication is especially helpful in the diagnosis
of lupus eiythematosus (folliculai plugging),
lichen planus (Wickham stiiae), basal cell cai-
cinomas (tianslucence and telangiectasia), and
melanoma (subtle changes in coloi, especially
giay oi blue); this is best visualized aftei ap-
plication of a diop of mineial oil. Use of the
deimatoscope is discussed below (see Dei-
moscopy").
O||que |g|ng of the skin lesion, done in
a daikened ioom, is often iequiied to detect
slight degiees of elevation oi depiession, and
it is useful in the visualization of the suiface
configuiation of lesions and in estimating the
extent of the eiuption.
Su|JueJ |g|ng in the examining ioom
enhances the contiast between ciicumsciibed
hypopigmented oi hypeipigmented lesions and
noimal skin.
VooJ |am (ultiaviolet long-wave light,
black" light) is valuable in the diagnosis of
ceitain skin and haii diseases and of poiphyiia.
With the Wood lamp (365 nm), fluoiescent pig-
ments and subtle coloi diffeiences of melanin
pigmentation can be visualized; the Wood lamp
also helps to estimate vaiiation in the lightness
of lesions in ielation to the noimal skin coloi
in both daik-skinned and faii-skinned peisons;
e.g., the lesions seen in tubeious scleiosis and
tinea veisicoloi aie hypomelanotic and aie not
as white as the lesions seen in vitiligo, which
aie amelanotic. Ciicumsciibed hypeimelanosis,
such as a fieckle and melasma, is much moie
evident (daikei) undei the Wood lamp. By con-
tiast, deimal melanin, as in a Mongolian sacial
spot, does not become accentuated undei the
Wood lamp. Theiefoie, it is possible to localize
the site of melanin by use of the Wood lamp;
|oweer, |s s more J[[tu| or no oss||e n
aens w| |rown or ||at| s|n.
Wood lamp is paiticulaily useful in the
detection of the fluoiescence of deimatophy-
tosis in the haii shaft (gieen to yellow) and of
eiythiasma (coial ied). A piesumptive diagno-
sis of poiphyiia can be made if a pinkish-ied
SFCIAI CIINICAI AN0 IA80kAI0k AI0S I0 0kMAI0I0CIC
0IACN0SIS
fluoiescence is demonstiated in uiine examined
with the Wood lamp; addition of dilute hydio-
chloiic acid intensifies the fluoiescence.
Dastoy consists of fiimly piessing a micio-
scopic slide oi a glass spatula ovei a skin lesion.
The examinei will find this pioceduie of special
value in deteimining whethei the ied coloi of
a macule oi papule is due to capillaiy dilata-
tion (eiythema) oi to extiavasation of blood
(puipuia) that does not blanch. Diascopy is also
useful foi the detection of the glassy yellow-
biown appeaiance of papules in saicoidosis,
tubeiculosis of the skin, lymphoma, and gianu-
loma annulaie.
Dermostoy (also called e|umnestente m-
trostoy ). A hand lens with built-in lighting and
a magnification of 10 to 30 is called a Jerma-
ostoe and peimits the noninvasive inspection
of deepei layeis of the epideimis and beyond.
This is paiticulaily useful in the distinction
of benign and malignant giowth patteins in
pigmented lesions. Dga| Jermostoy is pai-
ticulaily useful in the monitoiing of pigmented
skin lesions because images aie stoied electioni-
cally and can be ietiieved and examined at a
latei date to peimit compaiison quantitatively
and qualitatively and to detect changes ovei
time. Digital deimoscopy uses computei image
analysis piogiams that piovide (1) objective
measuiements of changes; (2) iapid stoiage,
ietiieval, and tiansmission of images to ex-
peits foi fuithei discussion (teledeimatology);
and (3) extiaction of moiphologic featuies foi
numeiical analysis. Deimoscopy and digital
deimoscopy iequiie special tiaining.
CIINICAI SICNS
Darer sgn is positive" when a biown maculai
oi a slightly papulai lesion of uiticaiia pigmen-
tosa (mastocytosis) becomes a palpable wheal
aftei being vigoiously iubbed with an instiu-
ment such as the blunt end of a pen. The wheal
may not appeai foi 5-10 min.
us: sgn is positive" when slight sciatch-
ing oi cuietting of a scaly lesion ieveals punc-
tate bleeding points within the lesion. This
suggests psoiiasis, but it is not specific.
The N|o|s|y |enomenon is positive when
the epideimis is dislodged fiom the deimis by
lateial, sheaiing piessuie with a fingei, iesulting
INIk00CII0N
xxxv
in an eiosion. It is an impoitant diagnostic sign
in acantholytic disoideis such as pemphigus oi
the staphylococcal scalded skin (SSS) syndiome
oi othei blisteiing oi epideimoneciotic disoi-
deis, such as toxic epideimal neciolysis.
CIINICAI ISIS
Pat| esng is used to document and validate
a diagnosis of alleigic contact sensitization and
identify the causative agent. Substances to be
tested aie applied to the skin in shallow cups
(Finn chambeis), affixed with a tape and left
in place foi 24-48 h. Contact hypeisensitivity
will show as a papulai vesiculai ieaction that
develops within 48-72 h when the test is iead.
It is a unique means of in vivo iepioduction of
disease in diminutive piopoitions, foi sensitiza-
tion affects all the skin and may theiefoie be
elicited at any cutaneous site. The patch test is
easiei and safei than a use test" with a ques-
tionable alleigen, foi test items can be applied
in low concentiations in small aieas of skin foi
shoit peiiods of time (see Section 2).
P|ooat| esng is a combination of patch
testing and UV iiiadiation of the test site and
is used to document photoalleigy (see Section
10).
Prt| esng is used to deteimine type I allei-
gies. A diop of a solution containing a minute
concentiation of the alleigen is placed on the
skin and the skin is pieiced thiough this diop
with a needle. Pieicing should not go beyond
the papillaiy body. A positive ieaction will ap-
peai as a wheal within 20 min. The patient has
to be undei obseivation foi possible anaphy-
laxis.
teow|enng facilitates detection of sub-
clinical penile oi vulvai waits. Gauze satuiated
with 5% acetic acid (white vinegai) is wiapped
aiound the glans penis oi used on the ceivix
and anus. Aftei 5-10 min, the penis oi vulva is
inspected with a 10 hand lens. Waits appeai as
small white papules.
IA80kAI0k ISIS
Mcroscopc xamoatoo oI Sca|es, Crusts,
Serum, aod har
Cram sans of smeais and tu|ures o[ exuJaes
anJ o[ ssue mntes should be made in lesions
suspected of being bacteiial oi yeast ( CanJJa
a||tans ) infections. Ulceis and nodules iequiie
a scalpel biopsy in which a wedge of tissue
consisting of all thiee layeis of skin is obtained;
the biopsy specimen is divided into one-half foi
histopathology and one-half foi cultuie. This is
minced in a steiile moitai and then cultuied foi
bacteiia (including typical and atypical myco-
bacteiia) and fungi.
Mtrostot examnaon foi mycelia should
be made of the ioofs of vesicles oi of scales (the
advancing boideis aie piefeiable) oi of the haii
in deimatophytoses. The tissue is cleaied with
10-30% KOH and waimed gently. Hyphae and
spoies will light up by theii biiefiingence (Fig.
25-1). Fungal cultuies with Sabouiaud medium
should be made (see Section 25).
Mtrostot examnaon o[ te||s o|aneJ [rom
|e |ase o[ est|es (Tzanck piepaiation) may
ieveal the piesence of acantholytic cells in the
acantholytic diseases (e.g., pemphigus oi SSS
syndiome) oi of giant epithelial cells and multi-
nucleated giant cells (containing 10-12 nuclei)
in heipes simplex, heipes zostei, and vaiicella.
Mateiial fiom the base of a vesicle obtained by
gen|e cuiettage with a scalpel is smeaied on a
glass slide, stained with eithei Giemsa oi Wiight
stain oi methylene blue, and examined to de-
teimine whethei theie aie acantholytic oi giant
epithelial cells, which aie diagnostic (Fig. 27-
27). In addition, cultuie, immunofluoiescence
tests, oi polymeiase chain ieaction foi heipes
have to be oideied.
La|oraory Jagnoss o[ sta|es . The diagnosis
is established by identification of the mite, oi
ova oi feces, in skin sciapings iemoved fiom
the papules oi buiiows (see Section 28). Using
a steiile scalpel blade on which a diop of steiile
mineial oil has been placed, apply oil to the sui-
face of the buiiow oi papule. Sciape the papule
oi buiiow vigoiously to iemove the entiie top
of the papule; tiny flecks of blood will appeai
in the oil. Tiansfei the oil to a micioscopic slide
and examine foi mites, ova, and feces. The mites
aie 0.2-0.4 mm in size and have foui paiis of
legs (see Section 28).
8opsy oI the Sko
Biopsy of the skin is one of the simplest, most
iewaiding diagnostic techniques because of the
easy accessibility of the skin and the vaiiety
of techniques foi study of the excised speci-
men (e.g., histopathology, immunopathology,
polymeiase chain ieaction, election micios-
copy).
Selection of the site of the biopsy is based pii-
maiily on the stage of the eiuption, and eaily le-
sions aie usually moie typical; this is especially
impoitant in vesiculobullous eiuptions (e.g.,
pemphigus, heipes simplex), in which the le-
sion should be no moie than 24 h old. Howevei,
INIk00CII0N
xxxv
oldei lesions (2-6 weeks) aie often moie chai-
acteiistic in discoid lupus eiythematosus.
A common technique foi diagnostic biopsy is
the use of a 3- to 4-mm punch, a small tubulai
knife much like a coiksciew, which by iotat-
ing movements between the thumb and index
fingei cuts thiough the epideimis, deimis, and
subcutaneous tissue; the base is cut off with
scissois. If immunofluoiescence is indicated
(e.g., as in bullous diseases oi lupus eiythema-
tosus), a special medium foi tianspoit to the
laboiatoiy is iequiied.
Foi nodules, howevei, a laige wedge should
be iemoved by excision including subcutaneous
tissue. Fuitheimoie, when indicated, lesions
should be bisected, one-half foi histology and
the othei half sent in a steiile containei foi
bacteiial and fungal cultuies oi in special fixa-
tives oi cell cultuie media, oi fiozen foi immu-
nopathologic examination.
Specimens foi light micioscopy should be
fixed immediately in buffeied neutial foimalin.
A biief but detailed summaiy of the clinical
histoiy and desciiption of the lesions should
accompany the specimen. Biopsy is indicated
in a|| skin lesions that aie suspected of being
neoplasms, in all bullous disoideis with immun-
ofluoiesence used simultaneously, and in all dei-
matologic disoideis in which a specific diagnosis
is not possible by clinical examination alone.
INIk00CII0N
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| A k I |
DI5OkDEk5 PkE5ENIINC
IN IHE 5KIN AND
MUCOU5 MEMkANE5
DI5OkDEk5 PkE5ENIINC
IN IHE 5KIN AND
MUCOU5 MEMkANE5
2
0IS0k0kS 0F S8AC0S
AN0 AF0CkIN CIAN0S
S E C I | 0 N |
2
Au |u||+rr+||ou o| p||oe|+ceou uu||, .e|, cor
rou
Appe+| |u ce||+|u |od, +|e+ (|+ce, ||uu|, |+|e|,
|u||oc|)
\o| ||equeu||, |u +do|eceu|
\+u||e| + coredoue, p+pu|opu|u|e, uod
u|e, +ud c,|
keu|| |u p|||ed, dep|eed, o| |,pe|||op||c
c+|
|C|9. 10o.
|C|0. |10.0
ACN vICAkIS (C0MM0N ACN) AN0 CSIIC ACN
FI0MI0I0C
0ccurreoce Veiy common, affecting appioxi-
mately 85% of young people.
Ae oI 0oset Pubeity-10 to 17 yeais in fe-
males, 14 to 19 in males; howevei, may appeai
fiist at 25 yeais oi oldei.
Sex Moie seveie in males than in females.
kace Lowei incidence in Asians and Afiicans.
Ceoetc Aspects Multifactoiial genetic back-
giound. Familial piedisposition: majoiity of
individuals with cystic acne have paient(s) with
a histoiy of seveie acne. Seveie acne may be
associated with XYY syndiome.
FAIh0CNSIS
Key [ators aie folliculai keiatinization, andio-
gens, and Proon|aterum atnes (Image 1-1).
Acne iesults fiom a change in the keiat-
inization pattein in the pilosebaceous unit,
with the keiatinous mateiial becoming moie
dense and blocking secietion of sebum. These
keiatin plugs aie called tomeJones and iep-
iesent the time bombs" of acne. Linoleic acid,
which iegulates keiatinocyte piolifeiation, is
decieased in acne. Comedonal plugging and a
complex inteiaction between andiogens and
bacteiia ( P. atnes ) in the plugged piloseba-
ceous units lead to inflammation. Andiogens
(qualitatively and quantitatively noimal in the
seium) stimulate sebaceous glands to pioduce
laigei amounts of sebum. Bacteiia contain
lipase, which conveits lipid into fatty acids, and
pioduce pioinflammatoiy mediatois, intei-
leukin 1, tumoi neciosis factoi TNF ]. Fatty
acids and pioinflammatoiy mediatois cause a
steiile inflammatoiy iesponse to the piloseba-
ceous unit. The distended follicle walls bieak,
and the contents (sebum, lipids, fatty acids,
keiatin, bacteiia) entei the deimis, piovoking
an inflammatoiy and foieign-body iesponse
(papule, pustule, nodule). Ruptuie plus intense
inflammation lead to scais.
Cootrbutory Factors Acnegenic mineial oils,
iaiely dioxin and otheis.
Drugs Lithium, hydantoin, isoniazid, glucocoi-
ticoids, oial contiaceptives, iodides, biomides
and andiogens (e.g., testosteione), danazol.
Others Emoona| sress can definitely cause
exaceibations. Ott|uson and ressure on the
skin, such as by leaning face on hands, ery
moran and often uniecognized exaceibating
factoi ( atne met|anta ). Acne is not caused by
chocolate oi fatty foods oi, in fact, by any kind
of food.
CIINICAI MANIFSIAII0N
0uratoo oI Iesoos Weeks to months.
Seasoo Often woise in fall and wintei.
Symptoms Pain in lesions (especially nodulo-
cystic type).
Sko Iesoos ComeJones -open (blackheads)
oi closed (whiteheads); tomeJona| atne
(Fig. 1-1). Pau|es and au|ousu|es -i.e.,
a papule topped by a pustule; au|ousu|ar
atne (Fig. 1-2). NoJu|es oi tyss -1-4 cm in
SCII0N 1 ||'0k|Ek' 0| 'EBACE0u' A|| A|0Ck||E C|A||' 3
FICk 1-1 Acoe vu|ars: comedooes Coredoue +|e |e|+||u p|u ||+| |o|r W||||u |o|||cu|+| o||+,
||equeu||, +oc|+|ed W||| u||ouud|u e|,||er+ +ud pu|u|e |o|r+||ou. Coredoue +oc|+|ed W||| r+|| o||+
+|e |e|e||ed |o + c|oed coredoue o| 'W|||e |e+d, ||oe +oc|+|ed W||| |+|e o||+ +|e |e|e||ed |o + opeu
coredoue o| '||+c| |e+d. Coredoue +|e |e| ||e+|ed W||| |op|c+| |e||uo|d.
FICk 1-2 20-year-o|d ma|e |u ||| c+e o| p+pu|opu|u|+| +cue, ore |u||+rr+|o|, p+pu|e |ecore
uodu|+| +ud ||u |ep|eeu| e+||, |+e o| uodu|oc,||c +cue.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 4
diametei (Fig. 1-3); noJu|otyst atne . Soft
nodules iesult fiom iepeated folliculai iuptuies
and ieencapsulations with inflammation,
abscess foimation, and foieign-body ieaction.
Cysts aie actually pseudocysts as they aie not
lined by epithelium but iepiesent fluctuating
abscesses (Image 1-1). Round isolated single
nodules and cysts coalesce to lineai mounds
and sinus tiacts (Fig. 1-4). Snuses : diaining
epithelial-lined tiacts, usually with nodulai
acne. Stars : atiophic depiessed (often pitted) oi
hypeitiophic (at times, keloidal). Se|orr|ea of
the face and scalp often piesent and sometimes
seveie. Foi moie clinical pictuies, see acne
pictuie galleiy on online veision.
SItes v] PredI|ectIvn Face, neck, tiunk, uppei
aims, buttocks.
Speca| Forms
Acoe Coo|obata Seveie cystic acne (Figs.
1-4 and 1-5) with moie involvement of the
tiunk than the face. Coalescing nodules, cysts,
abscesses, and ulceiation; occuis also on but-
tocks. Spontaneous iemission is long delayed.
Raiely, acne conglobata seen in XYY genotype
(tall males, slightly mentally ietaided, with
IMAC 1-1 A-0. Acoe pathoeoess ||or /+eu|e|u A| e| +|. Acue .u|+|| +ud +cue||o|r e|up||ou, |u
wo||| K e| +| (ed). ||cc|:| |-c||, C--c| !-!:-, 1|| ed. |eW \o||, \cC|+Wn|||, 2003.|
aggiessive behavioi) oi in the polycystic ovaiy
syndiome.
Acoe Fu|moaos Teenage boys (ages 13 to 17).
tue onse , seveie cystic acne with concomitant
suppuiation and always u|teraon ; also piesent
aie malaise, fatigue, fevei, geneialized aithial-
gias, leukocytosis, and elevated eiythiocyte
sedimentation iate.
SAFh0 Syodrome Synovitis, acne, acne ful-
minans, almoplantai pustulosis, |idiadenitis
suppuiativa, hypeiostosis, and osteitis. Raie.
FAFA Syodrome Steiile yogenic aithiitis,
yodeima gangienosum acne. An inheiited au-
toinflammatoiy disoidei; veiy iaie.
Iropca| Acoe Flaie of acne, usually with se-
veie folliculitis, inflammatoiy nodules, and
diaining cysts on tiunk and buttocks in tiopical
climates; secondaiy infection with Sa|y|otot-
tus aureus .
Acoe wth Faca| dema Associated with iecal-
citiant, disfiguiing midline facial edema. Woody
induiation with and without eiythema.
Acoe o the Adu|t Womao Peisistent acne in
an (often) hiisute female with oi without r-
regu|ar menses needs an evaluation foi hypei-
secietion of adienal and ovaiian andiogens:
N|crocomadona
hyparkaratot|c
|nfund|bu|um
cohas|va cornaocytas
sabum sacrat|on
0omadona
accumu|at|on of
shad cornaocytas
and sabum
d||at|on of fo|||cu|ar
ost|um
|nammatory papu|a/
pustu|a
furthar axpans|on
of fo|||cu|ar un|t
pro||farat|on of
Prcpicnibacterium acnes
par|fo|||cu|ar |nammat|on
hodu|a
ruptura of fo|||cu|ar wa||
markad par|fo|||cu|ar
|nammat|on
scarr|ng
A 8 0 0
total testosteione, fiee testosteione, and/oi de-
hydioepiandiosteione sulfate (DHEAS) (e.g., in
the polycystic ovaiy syndiome).
keca|ctraot Acoe Can be ielated to congenital
adienal hypeiplasia (11 - oi 21 - hydioxylase
deficiencies).
Acoe xcore Mild acne, usually in young
women, associated with extensive excoiiations
and scaiiing due to emotional and psychological
pioblems (obsessive compulsive disoidei).
Neooata| Acoe On nose and cheeks in new-
boins oi infants, ielated to glandulai develop-
ment; tiansient.
0ccupatooa| Acoe Due to exposuie to tai de-
iivatives, cutting oils, chloiinated hydiocaibons
(see Chloiacne," below). Laige comedones,
inflammatoiy papules and cysts; not iestiicted
to piedilection sites of acne but can appeai on
othei (coveied) body sites.
Ch|oracoe Due to exposuie to chloiinated
aiomatic hydiocaibons in electiical conductois,
insecticides, and heibicides. Sometimes veiy
seveie due to industiial accidents oi intended
poisoning (e.g., dioxin).
Acoe Cosmetca Due to comedogenic cos-
metics.
FICk 1-3 Nodu|ocystc acoe A ,rre|||c d|||||u||ou |u ||e |+ce o| + |eeu+e |o,. I|| |r+e c|e+||,
|oW ||+| e.eu uodu|oc,||c +cue |+|| W||| coredoue-|o|| opeu +ud c|oed coredoue c+u |e eeu |u |||
|+ce-||+| ||eu ||+u|o|r |u|o p+pu|opu|u|+| |e|ou, W||c| eu|+|e +ud co+|ece e.eu|u+||, |o |e+d |o uodu|oc,||c
+cue. || | uo| u|p|||u ||+| ||ee |e|ou +|e .e|, p+|u|u|, +ud || | uude||+ud+||e ||+| uodu|oc,||c +cue +|o
e.e|e|, |rp+c| ||e oc|+| |||e o| ||ee +do|eceu|.
decade oi oldei. Flaies occui in the wintei
and with the onset of menses. The sequela is
scaiiing (foi clinical examples see , which
should be avoided by piopei tieatment, ese-
ta||y w| ora| sorenon ear|y n |e tourse o[
|e Jsease (see below).
MANACMNI
The psychological impact of acne (peiceived
cosmetic disfiguiement) should be assessed
individually in each patient and theiapy modi-
fied accoidingly. The goal of theiapy is to
iemove the plugging of the pilai diainage,
ieduce sebum pioduction, and tieat bacteiial
colonization.
M|d Acoe
Topical antibiotics (clindamycin and eiyth-
iomycin)
Benzoyl peioxide gels (2%, 5%, oi 10%)
Topical ietinoids (tietinoin, adapalene) ie-
quiie detailed instiuctions iegaiding giad-
ual incieases in concentiation fiom 0.01%
to 0.025% to 0.05% cieam/gel oi liquid.
Aftei impiovement, medication is ieduced
to the lowest effective maintenance.
Impiovement occuis ovei a peiiod of months
(2-5) but may take even longei foi noninflamed
comedones. Topical ietinoids aie applied in the
evening; topical antibiotics and benzoyl pei-
oxide gels aie applied duiing the day.
Combination theiapy is best, using benzoyl
peioxide-eiythiomycin gels |us topical ietin-
oids (tietinoin or tazaiotene gel, adapalene).
Noe. acne suigeiy (extiactions of comedones)
is helpful only when piopeily done and aftei
pietieatment with topical ietinoids.
Moderate Acoe Oial antibiotics aie added to the
above iegimen. Most effective antibiotic is minoc-
ycline, 50 -100 mg twice daily, oi doxycycline, 50 -
100 mg twice daily, and this is tapeied to 50- mg/d
as acne lessens. In females, modeiate acne can be
contiolled with high doses of oial estiogens com-
bined with piogesteione oi antiandiogens, but ie-
cuiiences aie the iule aftei cessation of tieatment.
Ceiebiovasculai accidents aie a seiious iisk.
Noe. foi inflammatoiy cysts and nodules, in-
tialesional tiiamcinolone (0.05 mL of a 3- to 5-
mg/mL suspension) is indicated.
Severe Acoe In addition to the topical tieat-
ment outlined above, systemic tieatment with
isotietinoin is indicated foi cystic oi conglo-
bate acne oi foi acne iefiactoiy to tieatment.
This ietinoid inhibits sebaceous gland func-
tion and keiatinization and is veiy effective.
Fomade Acoe On the foiehead, usually in Af-
iicans applying pomade to haii.
Acoe Mechaoca Flaies of pieexisting acne in
face, because of leaning face on hands, oi on
foiehead, fiom piessuie of football helmet.
Acoe-Ike Coodtoos
Sterod Acoe Following systemic oi topical
glucocoiticoids. Monomoiphous folliculitis-
small eiythematous papules and pustules w|-
ou comedones.
0ru-Ioduced Acoe Monomoiphous acne-
like eiuption due to phenytoin, lithium, isoni-
azid, high-dose vitamin B complex, epideimal
giowth factoi inhibitois (see Section 22), halo-
genated compounds. No comedones.
Acoe Aestva|s Papulai eiuption aftei sun ex-
posuie (Malloica acne"). Usually on foiehead,
shouldeis, aims, neck, and chest. No come-
dones. Pathogenesis unknown.
Cram-Neatve Fo||cu|ts Multiple tiny yellow
pustules develop on top of acne vulgaiis as a ie-
sult of long-teim antibiotic administiation.
0IACN0SIS AN0 0IFFkNIIAI 0IACN0SIS
Noe : Comedones aie iequiied foi diagnosis of
any type of acne. Comedones aie not a featuie
of acne-like conditions (above) and of the con-
ditions listed below.
Face S. aureus folliculitis, pseudofolliculitis
baibae, iosacea, peiioial deimatitis.
Iruok Ma|asse:a folliculitis, hot-tub" pseu-
domonas folliculitis, S. aureus folliculitis, and
acne-like conditions (see above).
IA80kAI0k XAMINAII0N
No laboiatoiy examinations iequiied. If theie
is suspicion of an endociine disoidei, fiee
testosteione, follicle-stimulating hoimone,
luteinizing hoimone, and DHEAS should be
deteimined to exclude hypeiandiogenism and
polycystic ovaiy syndiome. Noe : In the ovei-
whelming majoiity of acne patients, hoimone
levels aie noimal.
Laboiatoiy examinations tiansaminases
(ALT, AST), tiiglyceiides, and cholesteiol levels]
may be iequiied if systemic isotietinoin tieat-
ment is planned (see below).
C0kS
Acne most often cleais spontaneously by the
eaily twenties but can peisist to the fouith
SCII0N 1 ||'0k|Ek' 0| 'EBACE0u' A|| A|0Ck||E C|A||' T
Oial isotietinoin leads to complete iemission in
almost all cases, which last foi months to yeais
in the majoiity of patients.
1ndIcutIvns ]vr Oru| 1svtretInvIn Foi modei-
ate and seveie, iecalcitiant, nodulai acne. The
patient must have been iesistant to othei acne
theiapies, including systemic antibiotics.
CvntruIndIcutIvns Isotietinoin is teiatogenic.
Theiefoie, piegnancy must be pievented and ef-
fective contiaception is necessaiy, i.e., oial. Both
tetiacycline and isotietinoin may cause pseudo-
tumoi ceiebii (benign intiacianial swelling);
theiefoie, the two medications should neer be
used togethei.
WurnIngs Blood lipids and tiansaminases
(ALT, AST) should be deteimined befoie thei-
apy. About 25% of patients can develop n-
treaseJ |asma rg|yterJes ; 15% of patients a
deciease in |g|-Jensy |oroens , and about
7% an ntrease n t|o|esero| |ee|s . This may
inciease the caidiovasculai iisk. When levels of
seium tiiglyceiides iise above 800 mg/L, the
patient may develop acute pancieatitis. Patients
should not take vitamin supplements contain-
ing vitamin A. Heaooxty has been veiy
iaiely iepoited in the foim of clinical hepatitis,
but patients may develop mild to modeiate
elevation of tiansaminase levels that noimalize
FICk 1-4 Acoe coo|obata |u ||| e.e|e uodu|oc,||c +cue, ||e|e +|e |+|e cou||ueu| uodu|e +ud c,|
|o|r|u ||ue+| rouud ||+| co||epoud |o |u|e|couuec||u c|+uue|. I|e|e | po|u|+||ou, c+|||u +ud |e|ou +|e
.e|, p+|u|u|.
with ieduction of the dose of the diug. Eyes.
ng| ||nJness has been iepoited, and patients
should be wained about diiving at night. Also,
patients may have JetreaseJ o|erante o tonat
|enses duiing and aftei theiapy. S|n : an eczema-
like iash due to diug-induced diyness often
appeais, and this iesponds diamatically to low
potency (class III) topical glucocoiticoids. Diy
lips and cheilitis occui in piactically all patients
and must be tieated. Reveisible thinning of haii
may occui veiy iaiely, as may paionychia. Nose :
diyness of nasal mucosa and nose bleeds (iaie).
O|er sysems : iaiely, depiession, headaches,
aithiitis, and musculai pain. Foi additional
iaie possible complications, consult the pack-
age inseit.
Dvsuge Isotietinoin, 0.5 to 1 mg/kg given in
divided doses with food. Most patients impiove
and cleai within 20 weeks with 1 mg/kg. Foi
seveie disease, especially on the tiunk, 2 mg/kg
and longei tieatment may be iequiied. As many
as thiee oi moie couises of isotietinoin have
been given in iefiactoiy cases, but in most cases
a single couise is sufficient to induce lasting
iemission.
0ther Systemc Ireatmeots Ior Severe Acoe
Systemic glucocoiticoids may be iequiied
in seveie acne conglobata, acne fulminans,
and the SAPHO and PAPA syndiomes. The
TNF- inhibitoi infliximab and anakinia aie
investigational diugs in these seveie foims and
show piomising effects. Noe : Foi inflammatoiy
cysts and nodules, intialesional tiiamcinolone
(0.05 mL of a 3 to 5 mg/mL solution) is indi-
cated. Website: |.//www.aaJ.org/am||es/
atneam.|m|
FICk 1-5 Acoe coo|obata |u||+rr+|o|, uodu|e +ud c,| |+.e co+|eced, |o|r|u +|cee +ud e.eu
|e+d|u |o u|ce|+||ou. I|e|e +|e ru|||p|e coredoue +ud r+u, |eceu| |ed c+| |o||oW|u |eo|u||ou o| |u||+rr+|o|,
|e|ou ou ||e uppe| c|e|, uec|, +ud +|r.
A corrou c||ou|c |u||+rr+|o|, +cue||o|r d|o|
de| o| ||e |+c|+| p||oe|+ceou uu||.
|| | coup|ed W||| +u |uc|e+ed |e+c||.||, o| c+p||
|+||e |e+d|u |o ||u||u +ud |e|+u|ec|+|+.
\+, |eu|| |u |u||e|, |||c|eu|u o| uoe, c|ee|,
|o|e|e+d, o| c||u due |o e|+ceou |,pe|p|+|+,
eder+, +ud ||||o|
k0SACA lCD-9: o95.
lCD-10: L71
FI0MI0I0C
0ccurreoce Common, affecting appioximately
10% of faii-skinned people.
Ae oI 0oset 30 to 50 yeais; peak incidence
between 40 and 50 yeais.
Sex Females piedominantly, but ihinophyma
occuis mostly in males.
kace Celtic peisons (skin phototypes I and II)
but also southein Mediteiianeans; less fiequent
oi iaie in pigmented peisons (skin phototypes
V and VI, i.e., biown and black)
SIACINC (FIWIC AN0 kIICMAN
CIASSIFICAII0N)
T|e rosatea Ja|ess : episodic eiythema,
flushing and blushing"
Sage I. Peisistent eiythema with tel-
angiectases
Sage II. Peisistent eiythema, telangiectases,
papules, tiny pustules.
Sage III. Peisistent deep eiythema, dense
telangiectases, papules, pustules, nodules;
iaiely peisistent solid" edema of the cen-
tial pait of the face
Noe. piogiession fiom one stage to anothei
does not always occui. Rosacea may stait with
stage II oi III and stages may oveilap.
Usually a histoiy of episodic ieddening of the
face (flushing) with incieases in skin tempeia-
tuie in iesponse to heat stimuli in the mouth
(hot liquids); spicy foods; alcohol,. Exposuie
to sun-iosacea is often associated with solai
elastosis-and heat (such as chefs woiking neai
a hot stove) may cause exaceibations. Acne may
have pieceded the onset of iosacea by yeais;
neveitheless, iosacea may and usually does aiise
de novo without any pieceding histoiy of acne
oi seboiihea.
0uratoo oI Iesoos Days, weeks, months.
Sko Symptoms Concein about cosmetic facial
appeaiance; patients aie often peiceived as be-
ing alcoholic-which, of couise, is not tiue.
Sko Iesoos Eur|y Pathognomonic flush-
ing- ied face" (Fig. 1-6); tiny papules and
papulopustules (2-3 mm), pustule often
small ( 1 mm) and on the apex of the papule
(Figs. 1-7 and 1-8). No tomeJones .
Lute Red facies and dusky-ied papules and
nodules (Figs. 1-6 to 1-9) Scatteied, disciete
lesions. Telangiectases. Maiked sebaceous hy-
peiplasia and lymphedema in chionic iosacea,
causing disfiguiement of the nose, foiehead,
eyelids, eais, and chin.
DIstrIhutIvn Chaiacteiistic is the symmetiic
localization on the face (Fig. 1-7). Raiely, neck,
chest (V-shaped aiea), back, and scalp.
Speca| Iesoos
R|no|yma (enlaiged nose), meo|yma (en-
laiged cushion-like swelling of the foiehead),
||e|aro|yma (swelling of the eyelids), oo-
|yma (cauliflowei-like swelling of the eai-
lobes), and gna|o|yma (swelling of the chin)
iesult fiom maiked sebaceous gland hypeipla-
sia (Fig. 1-11) and fibiosis. Upon palpation:
soft, iubbei-like.
ye Iovo|vemeot
Red" eyes as a iesult of chionic blephaiitis,
conjunctivitis, and episcleiitis. Rosacea keiatitis,
albeit iaie, is a seiious pioblem because coineal
ulceis may develop.
IA80kAI0k XAMINAII0NS
8actera| Cu|ture Rule out S. aureus infection.
Sciapings may ieveal massive concuiient
DemoJex [o||tu|orum infestation.
0ermatopatho|oy Nonspecific peiifollicu-
lai and peiicapillaiy inflammation with oc-
casional foci of tubeiculoid" gianulomatous
aieas; dilated capillaiies. Foci of neutiophils
high and within the follicle. Laer sages : dif-
fuse hypeitiophy of the connective tissue,
sebaceous gland hypeiplasia, epithelioid gian-
uloma without caseation, and foieign-body
giant cells.
RhInvphymu Veiy maiked lobulai sebaceous hy-
peiplasia ( g|anJu|ar ye ) and/oi maiked inciease
in connective tissue ( [|rous ye ) with laige ec-
tatic veins ( [|roangomaous ye ).
0IFFkNIIAI 0IACN0SIS
Faca| Fapu|es[Fustu|es Acne (in iosacea
theie aie no comedones), peiioial deimatitis,
S. aureus folliculitis, giam-negative folliculitis,
D. [o||tu|orum infestation.
Faca| F|usho[rythema Seboiiheic deimati-
tis, piolonged use of topical glucocoiticoids, sys-
temic lupus eiythematosus; deimatomy ositis.
C0kS
Fro|ooed Recuiiences aie common. Aftei a
few yeais, the disease may disappeai spontane-
ously; usually it is foi life time. Men and veiy
iaiely women may develop ihinophyma.
MANACMNI
Freveotoo Maiked ieduction oi elimination
of alcohol may be helpful in some patients.
FICk 1-6 rythematous rosacea (stae I) I|e e+||, |+e o| |o+ce+ o||eu p|eeu| |, ep|od|c e|,
||er+, '||u||u +ud ||u||u, W||c| | |o||oWed |, pe|||eu| e|,||er+, W||c| | due |o ru|||p|e ||u, |e|+u|ec
|+|+, |eu|||u |u + |ed |+ce.
Iopca|
MeronJa:o|e ge| oi tream , 0.75%, twice
daily
MeronJa:o|e tream , 1%, once daily
SoJum su|[ateamJe, su|[ur |oons 10% and
5%
Tota| an|ots (e.g., eiythiomycin gel) aie
less effective.
Systemc Oial antibiotics aie moie effective
than topical tieatment.
Mnotyt|ne or Joxytyt|ne , 50-100 mg twice
daily, fiist-line antibiotics; veiy effective
(doxycycline is a phototoxic diug and its
use limits exposuie to sunlight in sum-
mei).
Teratyt|ne , 1-1.5 g/d in divided doses until
cleai; then giadually ieduce to once-daily
doses of 250-500 mg, most effective is oial
metionidazole 500 mg BID.
A dose of 50 mg minocycline oi doxycycline
oi 250-500 g tetiacycline is given as mainte-
nance.
0ra| Isotretooo Foi individuals with seveie
disease (especially stage III) not iesponding to
antibiotics and topical tieatments. A low-dose
iegimen of 0.1-0.5 mg/kg body weight pei day
is effective in most patients, but occasionally
1 mg/kg may be iequiied.
Ivermecto 12 mg PO in case of massive de-
modex infestation.
khoophyma aod Ie|aoectasa Tieated by
suigeiy oi lasei suigeiy with excellent cosmetic
iesults. Website |.//www.aaJ.org/am||es/
rosatea.|m|
FICk 1-T kosacea \ode|+|e|, e.e|e |o+ce+ |u + 29,e+|o|d |er+|e W||| pe|||eu| e|,||er+,
|e|+u|ec|+|+, |ed p+pu|e (|+e ||), +ud ||u, pu|u|e.
FICk 1-8 kosacea, staes II-III Ie|+u|ec|+|+, p+pu|e +ud pu|u|e, +ud ore We|||u |u + 50,e+|o|d
Wor+u. I|e|e +|e uo coredoue.
FICk 1-9 Fapu|opustu|ar rosacea (traostoo oI stae II to stae III) |u ||| o5,e+|o|d |er+|e, |o+
ce+ |u.o|.e +|ro| ||e eu|||e |+ce, p+||u ou|, ||e uppe| ||p +ud c||u. |+pu|e +ud pu|u|e |+.e co+|eced-++|u
uo coredoue-+ud |+.e +||e+d, |ed |o ore We|||u o| ||e c|ee|, W||c| p|eeu| 'o||d eder+.
FICk 1-10 kosacea, traostoo oI stae II
to III \u|||p|e ||||| |ed p+pu|e +ud pu|u|e
+ud ore We|||u o| ||e |||| c|ee| o| + 52
,e+|o|d Wor+u. |o|e ||+| |u ||| c+e |e|ou +|e
c|u|e|ed, ||e |o|e|e+d | ||ee o| |e|+u|ec|+|+,
+ud ||e |e|ou +|e uo| +|o|u|e|, ,rre|||c.
FICk 1-11 kosacea (stae III) ne|e ||e pe|||eu| 'o||d eder+ o| ||e uoe, |o|e|e+d, +ud p+|| o| ||e
c|ee| | ||e |e+d|u ,rp|or. |+pu|e, pu|u|e, +ud c|u|ed pu|u|e +|e upe||rpoed ou ||| pe|||eu| eder+.
I|e eu|+|ed uoe |ee| |u||e|, +ud +||e+d, |ep|eeu| |||uop|,r+.
||c|e|e e|,||er+|ou r|c|op+pu|e +ud r|
c|o.e|c|e,
0||eu cou||ueu| |u ||e pe||o|+| +ud pe||o||||+|
||u
0ccu| r+|u|, |u ,ouu Woreu, c+u occu| |u
c|||d|eu +ud ||e o|d
k+|e|,
FkI0kAI 0kMAIIIIS lCD-9: o95.
lCD-10: L71.0
*
FI0MI0I0C AN0 II0I0C
Ae oI 0oset 16-45 yeais; can occui in chil-
dien and the old.
Sex Females piedominantly.
to|oy Unknown but may be maikedly ag-
giavated by potent topical (fluoiinated) gluco-
coiticoids.
0uratoo oI Iesoos Weeks to months. Skin
symptoms peiceived as cosmetic disfiguiement;
occasional itching oi buining, feeling of tight-
ness.
Sko Iesoos 1- to 2-mm eiythematous pa-
pulopustules on an eiythematous backgiound
(Fig. 1-12) iiiegulaily giouped, symmet-
iic. Lesions inciease in numbei with cential
confluence and satellites; confluent plaques may
appeai eczematous with tiny scales. Theie aie
no comedones.
DIstrIhutIvn Initially peiioial. Rim of spaiing
aiound the veimilion boidei of lips (Fig. 1-12).
At times, in the peiioibital aiea (Figs. 1-13, 1-14).
Uncommonly, only peiioibital involvement; oc-
casionally, glabella and foiehead. Commonly on
the moustache aiea and lateial chin.
Cu|ture Rule out S. aureus infection.
Alleigic contact deimatitis, atopic deimatitis,
seboiiheic deimatitis, iosacea, acne vulgaiis,
steioid acne.
FICk 1-12 Ferora| dermatts \ode|+|e |u.o|.ereu| W||| e+||, cou||ueuce o| ||u, p+pu|e +ud + |eW
pu|u|e |u + pe||o|+| d|||||u||ou |u + ,ouu Wor+u. |o|e |,p|c+| p+||u o| ||e .e|r|||ou |o|de| (rucocu|+ueou
juuc||ou).
C0kS
Appeaiance of lesions usually subacute ovei
weeks to months. At times, misdiagnosed as
an eczematous oi a seboiiheic deimatitis and
tieated with a potent topical glucocoiticoid
piepaiation, aggiavating peiioial deimatitis oi
inducing steioid acne. Untieated, peiioial dei-
matitis fluctuates in activity ovei months to
yeais but is not neaily as chionic as iosacea.
MANACMNI
Iopca|
Avoid topical glucocoiticoids!
MeronJa:o|e , 0.75% gel two times daily oi
1% once daily
Ery|romytn , 2% gel applied twice daily
Systemc
Mnotyt|ne oi Joxytyt|ne , 100 mg daily
until cleai, then 50 mg daily foi anothei 2
months (caution, doxycycline is a photo-
sensitizing diug) oi
Teratyt|ne , 500 mg twice daily until cleai,
then 500 mg daily foi 1 month, then 250
mg daily foi an additional month.
FICk 1-13 Ferora| dermatts ||e|e|eu||+| |oc+
||ou ou ||e c||u |u| +|o ou ||e |oWe| e,e||d |u + o+,e+|
o|d Wor+u. A| ||| +e, d|||e|eu||+| d|+uo| |uc|ude
|o+ce+, |u| || Wou|d |e uuuu+| |o| |o+ce+ |o |u.o|.e
||e pe||o|+| |e|ou +ud e,e||d |u| p+||u ||e c|ee|
+ud uoe.
FICk 1-14 Ferorbta| dermatts |o|e p|eeuce o| ||u, p+pu|e +ud + |eW pu|u|e +|ouud ||e e,e. I||
| + ruc| |e corrou ||e ||+u ||e |e|ou +|ouud ||e rou||.
A c||ou|c, uppu|+||.e, o||eu c|c+|||c|+| d|e+e o|
+poc||ue |+ud-|e+||u ||u.
|u.o|.e ||e +\|||+e, ||e +uoeu||+| |e|ou,
+ud, |+|e|,, ||e c+|p (c+||ed ::c|c -||
|:||).
\+, |e +oc|+|ed W||| e.e|e uodu|oc,||c +cue
+ud p||ou|d+| |uue (|e|red |||:|c ::|
,!-).
',,. Apoc||u|||, ||d|+deu||| +\|||+||,
+|ce o| ||e +poc||ue We+| |+ud.
hI0kA0NIIIS SFFkAIIvA |C|9. 105.3!
|C|0. |1!.2
FI0MI0I0C
Ae oI 0oset Fiom pubeity to climacteiic.
Sex Affects moie females than males; esti-
mated to be 4% of female population. Males
moie often have anogenital and females axillaiy
involvement.
kace All iaces.
heredty Mothei-daughtei tiansmission has
been obseived. Families give a histoiy of nod-
ulocystic acne and hidiadenitis suppuiativa
occuiiing sepaiately oi togethei in blood iela-
tives.
II0I0C AN0 FAIh0CNSIS
Unknown. Piedisposing factois: obesity, genetic
piedisposition to acne, folliculai plugging of
apociine iegions, secondaiy bacteiial infection.
FAIh0CNSIS
The following sequence may be the mechanism
of the development of the lesions: keiatinous
plugging of the haii follicle dilatation haii
follicle and secondaiily of the apociine duct
inflammatoiy changes limited to a single
apociine gland bacteiial giowth in dilated
follicle and duct iuptuie iesulting in exten-
sion of inflammation/infection extension of
suppuiation/tissue destiuction ulceiation
and fibiosis, sinus tiact foimation.
Symoms : Inteimittent pain and maiked point
tendeiness ielated to abscess.
Sko Iesoos Initial lesion: ery enJer , ied
inflammatoiy nodule/abscess (Fig. 1-15) that
may iesolve oi diain puiulent/seiopuiulent
mateiial. The same lesion may appeai iepeatedly
in the same location. Open comedones, and
at times unique Jou||e comedones, aie highly
chaiacteiistic (Fig. 1-15), may be piesent even
when active nodules aie absent. Eventually,
modeiately to exquisitely tendei sinus tiacts may
foim. Pus diains fiom opening of abscess and
sinus tiacts; fibiosis, biidge" scais, hypeitiophic
and keloidal scais, contiactuies foim (Figs. 1-16,
1-17). Raiely, lymphedema of the associated
limb may develop.
DIstrIhutIvn Axillae, bieasts, anogenital aiea,
gioin. Often bilateial in axillae and/oi ano-
genital aiea; may extend ovei entiie back, but-
tocks, peiineum involving sciotum oi vulva
(Fig. 1-18), and scalp.
AssvcIuted FIndIngs Cystic acne, pilonidal
sinus. Often obesity.
8actero|oy Vaiious pathogens may second-
aiily colonize oi infect" lesions. These include
S. aureus , stieptococci, Est|ert|a to|, Proeus
mra||s , and PseuJomonas aerugnosa .
0ermatopatho|oy Ear|y : keiatin occlusion of
haii follicle, ductal/tubulai dilatation, inflam-
matoiy changes limited to folliculai appaiatus.
Lae : destiuction of apociine/ecciine/piloseba-
ceous appaiatus, fibiosis, pseudoepithelioma-
tous hypeiplasia in sinuses.
Painful papule, nodule, abscess in gioin and
axilla. Ear|y : fuiuncle, caibuncle, lymphadeni-
tis, iuptuied inclusion cyst, painful lymphad-
enopathy in lymphogianuloma veneieum oi
cat-sciatch disease. Lae : lymphogianuloma
veneieum, donovanosis, sciofulodeima, actino-
mycosis, sinus tiacts and fistulas associated with
ulceiative colitis and iegional enteiitis.
SCII0N 1 ||'0k|Ek' 0| 'EBACE0u' A|| A|0Ck||E C|A||' 1T
FICk 1-15 hdradeots sup-
puratva \+u, ||+c| coredoue,
ore o| W||c| +|e p+||ed, +|e + c|+|
+c|e||||c ||ud|u, +oc|+|ed W||| deep,
e\qu|||e|, p+|u|u| +|cee +ud o|d
c+| |u ||e +\|||+.
FICk 1-16 hdradeots suppuratva \u|||p|e |u||u +ud dep|eed c+| puc|e||u ||e u||ouud|u
||u +ud d|+|u|u |uue |u ||e +\|||+ o| + 22,e+|o|d |er+|e.
C0kS AN0 Fk0CN0SIS
The seveiity of the disease vaiies consideiably.
Many patients have only mild involvement with
iecuiient, self-healing, tendei ied nodules and
do not seek theiapy. The disease usually un-
deigoes a spontaneous iemission with age
(35 yeais). In some individuals, the couise
can be ielentlessly piogiessive, with maiked
moibidity ielated to chionic pain, diaining
sinuses, and scaiiing, with iestiicted mobility
(Fig. 1-18) . Complications (iaie): fistulas
to uiethia, bladdei, and/oi iectum; anemia,
amyloidosis.
MANACMNI
Hidiadenitis suppuiativa is no simply an infec-
tion, and systemic antibiotics aie only pait of
the tieatment piogiam. Combinations of (1)
intialesional glucocoiticoids, (2) suigeiy, (3)
oial antibiotics, and (4) isotietinoin aie used.
Medca| Maoaemeot
Acute FaoIu| Iesoos Nvdu|e Intialesional tii-
amcinolone (3-5 mg/mL).
Ahscess Intialesional tiiamcinolone (3-5 mg/
mL) into the wall followed by incision and
diainage of abscess fluid.
Chrooc Iow-Crade 0sease Oial antibiotics:
eiythiomycin (250 - 500 mg qid), tetiacycline
(250-500 mg qid), oi minocycline (100 mg
twice daily) until lesions iesolve, oi a combina-
tion of clindamycin twice daily 300 mg bid with
iifampin (300 mg twice daily); may take weeks.
Fredosooe May be given concuiiently if pain
and inflammation aie seveie: 70 mg daily foi 2
to 3 days, tapeied ovei 14 days.
0ra| Isotretooo Not useful in seveie disease,
but useful in eaily disease to pievent folliculai
plugging and when combined with suigical ex-
cision of individual lesions.
Surca| Maoaemeot
Incise and diain acute abscesses.
Excise chionic iecuiient, fibiotic nodules oi
sinus tiacts. If one oi two nodules can be
pinpointed with iecuiient disease, they can
be excised with a good iesult.
With extensive, chionic disease, complete
excision of axilla oi involved anogenital aiea
may be iequiied. Excision should extend
down to fascia and iequiies split skin giafting.
Fsycho|oca| Maoaemeot
These patients need constant ieassuiance, as
they become veiy depiessed because of the na-
tuie of the illness, e.g., pain, soiling of clothing
by diaining pus, odoi, and the site of occui-
ience (anogenital aiea). Theiefoie, eveiy effoit
should be made to deal with the disease, using
eveiy modality possible.
FICk 1-1T hdradeots sup-
puratva 'e.e|e c+|||u ou ||e
|u||oc|, |u||+rr+|o|, p+|u|u| uod
u|e W||| |||u|+ +ud d|+|u|u |uue.
w|eu ||e p+||eu| || doWu, pu W|||
qu||| ||or ||e |uu opeu|u.
FICk 1-18 hdradeots suppuratva I|e eu|||e pe||eu||+| +ud pe||+u+| ||u + We|| + ||e |u||oc| +ud
|uue| +pec| o| ||e |||| +|e |u.o|.ed |u ||| 50,e+|o|d r+|e. I|e|e | cou|de|+||e |u||+rr+||ou, +ud p|eu|e
|e|e+e pu|u|eu| e\ud+|e ||or ru|||p|e |uue. I|e p+||eu| |+d |o We+| + |+|e d|+pe|, |ec+ue W|eue.e| |e W+
e+|ed, ec|e||ou Wou|d qu||| ||or ||e |uue.
20
S E C I | 0 N 2
CIMA[0kMAIIIIS
I|e |e|r -:c-c +ud !-c|| +|e ued
|u|e|c|+ue+||,, deuo||u + po|,ro|p||c |u||+r
r+|o|, |e+c||ou p+||e|u |u.o|.|u ||e ep|de|r|
+ud de|r|. I|e|e +|e r+u, e||o|o|e +ud + W|de
|+ue o| c||u|c+| ||ud|u. Acu|e ec/er+/de|r+||||
| c|+|+c|e||/ed |, p|u|||u, e|,||er+, +ud .e
|cu|+||ou, c||ou|c ec/er+/de|r+||||, |, p|u|||u,
\e|o|, ||c|eu|||c+||ou, |,pe||e|+|o|, ||u||u.
|C| | + |oc+||/ed d|e+e cou||ued |o +|e+ e\
poed |o |||||+u|.
|| | c+ued |, e\pou|e o| ||e ||u |o c|er|c+| o|
o||e| p|,|c+| +eu| ||+| +|e c+p+||e o| |||||+||u
||e ||u, +cu|e|, o| c||ou|c+||,.
'e.e|e |||||+u| c+ue |o\|c |e+c||ou e.eu +||e| +
|o|| e\pou|e.
\o| c+e +|e c+ued |, c||ou|c curu|+||.e
e\pou|e |o oue o| ro|e |||||+u|.
I|e |+ud +|e ||e ro| corrou|, +||ec|ed +|e+.
|u +dd|||ou |o de|r+||||, |||||+u| cou|+c| |epoue
o| ||e ||u |uc|ude. u|jec||.e |||||+uc,, ||+u|eu|
|||||+u| |e+c||ou, pe|||eu| |||||+u| |e+c||ou, |o\|c
(c+u||c) |u|u.
|||||+u| cou|+c| |epoue o| ||u +ppeud+e
+ud p|reu|+|, ,|er |uc|ude. |o|||cu|+| +ud
+cue||o|r e|up||ou, r|||+||+, p|reu|+|, c|+ue
(|,po +ud |,pe|p|reu|+||ou), |+uu|or+|ou
|e+c||ou, +ud +|opec|+.
IkkIIANI C0NIACI 0kMAIIIIS (IC0)
|C|9 . o92.9
|C|0 . |2+
C|c:| !-c|| | + eue||c |e|r +pp||ed |o
+cu|e o| c||ou|c |u||+rr+|o|, |e+c||ou |o u|
|+uce ||+| core |u cou|+c| W||| ||e ||u. |||||+u|
cou|+c| de|r+|||| (|C|) | c+ued |, + c|er|c+|
|||||+u|, +||e||c cou|+c| de|r+|||| (AC|) |, +u +u
||eu (+||e|eu) ||+| e||c|| + |,pe |\ (ce||red|+|ed
o| de|+,ed) |,pe|eu|||.||, |e+c||ou.
I|e +cu|e |o|r o| |C| occu| +||e| + |u|e
e\pou|e |o ||e o||eud|u +eu| ||+| | |o\|c |o ||e
||u (e.., c|o|ou o||, p|euo|, |e|oeue, o|+u|c
o|.eu|, od|ur +ud po|+|ur |,d|o\|de, ||re
+c|d) +ud |u e.e|e c+e r+, |e+d |o uec|o|.
|| | depeudeu| ou couceu||+||ou o| ||e o||eud|u
+eu| +ud occu| |u e.e|,oue, depeud|u ou
||e peue||+|||||, +ud |||c|ue o| ||e ||+|ur
co|ueur. I|e|e | + |||e|o|d couceu||+||ou |o|
||ee u||+uce +|o.e W||c| ||e, c+ue +cu|e
de|r+|||| +ud |e|oW W||c| ||e, do uo|. I||
e| +cu|e |C| +p+|| ||or +cu|e AC|, W||c|
| depeudeu| ou eu|||/+||ou +ud ||u occu|
ou|, |u eu|||/ed |ud|.|du+|. |epeud|u ou ||e
de|ee o| eu|||/+||ou, r|uu|e +rouu| o| ||e
o||eud|u +eu| r+, e||c|| + |e+c||ou. '|uce |C| |
+ |o\|c p|euoreuou, || | cou||ued |o ||e +|e+ o|
e\pou|e +ud | ||e|e|o|e +|W+, |+|p|, r+||u
+|ed +ud ue.e| p|e+d. AC| | +u |rruuo|o|c
|e+c||ou ||+| |eud |o |u.o|.e ||e u||ouud|u
||u (p|e+d|u p|euoreuou) +ud r+, e.eu
p|e+d |e,oud +||ec|ed ||e. Ceue|+||/+||ou r+,
occu|.
C0NIACI 0kMAIIIIS |C|9 . o929
|C|0 . |25
SCII0N 2 EC/E\A/|Ek\AI|I|' 21
IA8I 2-1 Nost Common |rritant/Io\ic Aents
'o+p, de|e|eu|, W+|e||e |+ud c|e+ue|
Ac|d +ud +||+||*. |,d|o||uo||c +c|d, cereu|, c||or|c +c|d, p|op|o|u, e||,|eue o\|de, p|euo|, re|+| +||.
|udu|||+| o|.eu|. co+| |+| o|.eu|, pe||o|eur, c||o||u+|ed |,d|oc+||ou, +|co|o| o|.eu|, e||,|eue |,co| e||e|,
|u|peu||ue, e||,| e||e|, +ce|oue, c+||ou d|o\|de, |\'0, d|o\+ue, |,|eue.
||+u|. Eup|o|||+ce+e (pu|e, c|o|ou, po|ue|||+, r+c|uee| ||ee). k+cuucu|+ce+e (|u||e|cup), C|uc||e|+e (||+c|
ru|+|d), u|||c+ce+e (ue|||e), 'o|+u+ce+e (peppe|, c+p+|c|u), 0puu||+ (p||c||, pe+|).
0||e|. |||e||+, Woo|, |ou| ,u||e||c c|o|||u, |||e|e|+|d+u| |+|||c, '|Ck p+pe|.
*
|e+d |o c|er|c+| |u|u +ud uec|o|, || couceu||+|ed.
FI0MI0I0C
ICD is the most common foim of occupational
skin disease, accounting foi up to 80% of all oc-
cupational skin disoideis. Howevei, ICD need
not be occupational and can occui in anyone
being exposed to a substance iiiitant oi toxic
to the skin.
0ccupatooa| xposure Individuals engaged in
the following occupations/activities aie at iisk
foi ICD: housekeeping; haiidiessing; medical,
dental, and veteiinaiy seivices; cleaning; floial
aiianging; agiicultuie; hoiticultuie; foiestiy;
food piepaiation and cateiing; piinting; paint-
ing; metal woik; mechanical engineeiing; cai
maintenance; constiuction; fishing.
II0I0C
to|oc Aeots (Table 2-1) Abiasives, clean-
ing agents, oxidizing agents (e.g., sodium hy-
pochloiite); ieducing agents, plants and animal
enzymes, secietions; dessicant powdeis, dust,
soils; excessive exposuie to watei.
Fredsposo Factors Atopics with a histoiy
of atopic deimatitis aie at highest iisk foi
ICD; the majoiity of woikeis with significant
occupational ICD aie atopics. Otheis: white
skin, tempeiatuie (low), climate (low humid-
ity), occlusion, mechanical iiiitation. Cement
ICD tends to flaie in summei in hot humid
climates.
FAIh0CNSIS
Iiiitants (both chemical and physical), cause
cell damage if applied foi sufficient time and
in adequate concentiation. ICD occuis when
defense oi iepaii capacity of the skin is unable
to maintain noimal skin integiity and function
oi when penetiation of chemical(s) induces an
inflammatoiy iesponse. Lessei iiiitants cause
ieaction only aftei piolonged exposuie. The
initial ieaction is usually limited to the site of
contact with the iiiitant; the concentiation of
iiiitant diffusing outside the aiea of contact
almost always falls below the ciitical thieshold
necessaiy to piovoke a ieaction.
Mechanisms involved in acute and chionic
phases of ICD aie fundamentally diffeient.
Acute ieactions involve diiect cytotoxic dam-
age to keiatinocytes. Chionic ICD iesults fiom
iepeated exposuies that cause slow damage to
cell membianes, disiupting the skin baiiiei and
leading to piotein denatuiation and cellulai
toxicity.
ACI IkkIIANI C0NIACI 0kMAIIIIS
Symptoms In some individuals, subjective
symptoms (buining, stinging, smaiting) may
be the only manifestations. Painful sensations
can occui within seconds aftei exposuie
(immediate-type stinging), e.g., to acids,
chloiofoim, and methanol. Delayed-type
stinging occuis within 1 to 2 min, peaking at
5 to 10 min, fading by 30 min, and is caused
by agents such as aluminum chloiide, phenol,
piopylene glycol, and otheis. In acute delayed
ICD, objective skin symptoms do not stait
until 8-24 h aftei exposuie (e.g., anthialin,
ethylene oxide, benzalkonium chloiide) and aie
accompanied by buining iathei then itching.
Sko Fodos May occui minutes aftei
exposuie oi may be delayed up to 24 h. The
spectium of changes ianges fiom eiythema
to vesiculation (Figs. 2-1 and 2-2) and caustic
buin with neciosis. Acute ICD iepiesents
shaiply demaicated eiythema and supeificial
edema, coiiesponding to the application site
of the toxic substance (Fig. 2-1). Lesions do
not spiead beyond the site of contact. In moie
seveie ieactions vesicles and blisteis aiise
within the eiythematous lesions (Figs. 2-1 and
2-2), followed by eiosions and/oi even fiank
neciosis, as with acids oi alkaline solutions.
No papules. Configuiation often bizaiie
oi lineai (outside job" oi diipping effect)
(Fig. 2-1).
vo|utoo oI Iesoos Eiythema with a dull,
nonglistening suiface (Fig. 2-1) vesicula-
tion (oi blistei foimation) (Figs. 2-1 and 2-2)
eiosion ciusting shedding of ciusts
and scaling oi (in chemical buin) eiythema
neciosis shedding of neciotic tissue
ulceiation healing.
DIstrIhutIvn Isolated, localized to one iegion
oi geneialized (plant deimatitis), depending on
contact with toxic agent.
DurutIvn Days, weeks depending on tissue
damage.
Coosttutooa| Symptoms
Usually none, but in widespiead acute ICD
acute illness" syndiome, fevei may occui.
Chk0NIC IkkIIANI C0NIACI
0kMAIIIIS
IFS
Cumu|atve IC0 Most common; develops
slowly aftei iepeated additive exposuie to mild
iiiitants (watei, soap, deteigents, etc.), usu-
ally on hands. Repeated exposuies to toxic oi
subtoxic concentiations of offending agents
usually associated with a chionic distuibance of
the baiiiei function that allows even subtoxic
concentiations of offending agents to penetiate
into the skin and elicit a chionic inflamma-
toiy iesponse; e.g., aftei iepeated exposuie
to alkaline deteigents and oiganic solvents,
which, if applied only once to noimal skin, do
not elicit a ieaction. Injuiy (e.g., iepeated iub-
bing of the skin), piolonged soaking in watei,
oi chionic contact aftei iepeated, cumulative
physical tiauma-fiiction, piessuie, abiasions
in individuals engaged in manual woik ( rau-
mat ICD ).
Irrtaot keactoo IC0 Eaily, subclinical dei-
matitis on hands of individuals exposed to wet
woik. Usually duiing fiist months of tiaining of
haii diesseis oi of metal woikeis, smaiting and
buining sensations.
Symptoms Stinging, smaiting, buining, anJ
itching; pain as fissuies develop.
Sko Fodos Diyness chapping eiythe-
ma (Fig. 2-3) hypeikeiatosis and scaling
fissuies and ciusting (Fig. 2-4). Shaip maigin-
ation gives way to ill-defined boideis, licheni-
fication. In rran reaton ICD also vesicles,
pustules, and eiosions.
DIstrIhutIvn Usually on hands (Figs. 2-3 and
2-4). In tumu|ae ICD usually staiting at
fingei webspaces, spieading to sides and doi-
sal suiface of hands and then to palms. In
housewives often staiting on fingeitips ( u|-
s ) (Fig. 2-3). Raiely in othei locations exposed
to iiiitants and/oi tiauma, e.g., in violinists on
mandible oi neck, oi on exposed sites as in ar-
|orne ICD (see below).
DurutIvn Chionic, months to yeais.
None, except when infection occuis. Chionic
ICD (e.g., hand deimatitis; see below) can
become a seveie occupational and emotional
pioblem.
hstopatho|oy In acute ICD, epideimal cell
neciosis, neutiophils, vesiculation, and necio-
sis. In chionic ICD, acanthosis, hypeikeiatosis,
lymphocytic infiltiate.
Fatch Iests These aie negative in ICD unless
alleigic contact deimatitis is also piesent (see
below).
SFCIAI F0kMS 0F IC0
haod 0ermatts
Most cases of chionic ICD occui on the hands
and aie occupational. Often sensitization to
alleigens (such as nickel oi chiomate salts)
occuis, and then ACD (acute and/oi chionic)
is supeiimposed on ICD. A typical example is
hand deimatitis in constiuction and cement
woikeis. Cement is alkaline and coiiosive, lead-
ing to chionic ICD; chiomates in cement sensi-
tize and lead to ACD (see Fig. 2-6). In such cases
the eiuption may spiead beyond the hands and
may even geneialize.
FICk 2-1 Acute rrtaot cootact dermatts Io||owo app|catoo oI a cream cootaoo oooy|va-
o||amd aod ocotoc acd-butoxyethy|ester prescrbed Ior |ower back pao I|e '||e+|, p+||e|u
|ud|c+|e +u ou||de jo|. I|e e|up||ou | c|+|+c|e||/ed |, + r+|.e e|,||er+ W||| .e|cu|+||ou +ud ||||e|
|o|r+||ou +ud | cou||ued |o ||e ||e e\poed |o ||e |o\|c +eu|.
FICk 2-2 Acute rrtaot cootact dermatts oo the haod due to ao odustra| so|veot I|e|e |
r+|.e ||||e||u ou ||e p+|r.
Arboroe IC0
Chaiacteiistically face, neck, anteiioi chest, and
aims aie involved. Most fiequent causes aie
iiiitating dust and volatile chemicals (ammo-
nia, solvents, foimaldehyde, epoxy iesins, ce-
ment, fibeiglass, sawdust fiom toxic woods).
This has to be distinguished fiom photoalleigic
contact deimatitis (see Section 10).
Fustu|ar aod AcoeIorm IC0
ICD may taiget follicles and become pustulai
and papulopustulai. It may iesult fiom metals,
mineial oils, gieases, cutting fluids, naphtha-
lenes.
Diagnosis is by histoiy and clinical examination
(lesions, pattein, site). Most impoitant diffei-
ential diagnosis is ACD (see Table 2-3, p. 32).
On palms and soles: palmoplantai psoiiasis;
in exposed sites: photoalleigic contact deimatitis.
C0kS AN0 Fk0CN0SIS
Healing usually occuis within 2 weeks of ie-
moval of noxious stimuli; in moie chionic cases,
6 weeks oi longei may be iequiied. In the setting
of occupational ICD, only one-thiid of indi-
viduals have complete iemission and two-thiids
may iequiie allocation to anothei job; atopic
individuals have a woise piognosis. In cases of
chionic subciitical levels of iiiitant, some woik-
eis develop toleiance, oi haidening."
MANACMNI
Freveotoo
Avoid iiiitant oi caustic chemical(s) by weai-
ing piotective clothing (i.e., goggles, shields,
gloves).
If contact does occui, wash with watei oi
weak neutializing solution.
Baiiiei cieams.
In occupational ICD that peisists in spite of
adheience to the above measuies, change of
job may be necessaiy.
IkAIMNI
Acute Identify and iemove the etiologic agent.
Wet diessings with gauze soaked in Buiow`s so-
lution, changed eveiy 2-3 h. Laigei vesicles may
be diained, but tops should no be iemoved.
Topical class I glucocoiticoid piepaiations. In
seveie cases, systemic glucocoiticoids may be
indicated. Piednisone: 2-week couise, 60 mg
initially, tapeiing by steps of 10 mg.
Suhucute und ChrvnIc Identify and iemove
etiologic/pathogenic agent. Employ a potent
topical glucocoiticoid piepaiation, betametha-
sone dipiopionate oi clobetasol piopionate,
and piovide adequate lubiication. As healing
occuis, continue with lubiicating/piotective
cieams oi ointments. The topical calcineuiin in-
hibitois pimeciolimus and taciolimus aie usu-
ally not potent enough to suppiess the chionic
inflammation and its sequelae sufficiently.
In chionic ICD of hands a haidening effect"
can be achieved in most cases with topical (soak
oi bath)-PUVA theiapy (see page 68).
FICk 2-3 ar|y chrooc rrtaot cootact dermatts o a housewIe I|| |+ |eu||ed ||or |epe+|ed
e\pou|e |o o+p +ud de|e|eu|. |o|e |||eu|u ||ue|||p (pu|p|||).
FICk 2-4 . Chrooc rrtaot dermatts wth acute exacerbatoo o a housewIe I|e p+||eu| ued
|u|peu||ue |o c|e+u |e| |+ud +||e| p+|u||u. E|,||er+, ||u||u, +ud c+||u. ||||e|eu||+| d|+uo| | +||e||c
cou|+c| de|r+|||| +ud p+|r+| po||+|. |+|c| |e| |o |u|peu||ue We|e ue+||.e. Irrtaot cootact dermatts |u
+ cou||uc||ou Wo||e| W|o Wo|| W||| cereu|. |o|e ||e |,pe||e|+|oe, c+||u +ud ||u||u. I|e|e | +|o r|u|r+|
pu|u|+||ou.
FI0MI0I0C
Fiequent. Accounts foi 7% of occupationally
ielated illnesses in the United States. How-
evei, theie aie data suggesting that the actual
indicence iate is 10 to 50 times gieatei than
iepoited in the U.S. Buieau of Laboi Statis-
tics data. Nonoccupational ACD is estimated
to be thiee times gieatei than occupational
ACD.
Ae oI 0oset No influence on capacity foi
sensitization; howevei, alleigic contact deima-
titis is uncommon in young childien and in
individuals oldei than 70 yeais.
0ccupatoo One of the most impoitant causes
of disability in industiy.
FAIh0CNSIS
ACD is a classic, delayed, cell-mediated hy-
peisensitivity ieaction. Exposuie to a stiong
sensitizei such as poison ivy iesin iesults in
sensitization in a week oi so, while exposuie
to a weak alleigen may take months to yeais
foi sensitization. The antigen is taken up by
Langeihans cells, which piocess the antigen
and migiate fiom the epideimis to the diaining
lymph nodes, wheie they piesent the piocessed
antigen in association with MHC class II mol-
ecules to T cells that then piolifeiate. Sensitized
T cells leave the lymph node, entei the blood
ciiculation, home to the skin, and, aftei being
piesented by Langeihans cells with the same
specific antigen, pioduce and mediate the ie-
lease by othei cells of a vaiiety of cytokines.
Thus, all the skin becomes hypeisensitive to
the contact alleigen and will ieact wheievei the
specific alleigen is piesented.
AIIkCNS
Contact alleigens aie diveise and iange fiom
metal salts to antibiotics, dyes to plant pioducts.
AC| | + ,|er|c d|e+e de||ued |, |+p|eu
pec|||c I ce||-red|+|ed |u||+rr+||ou.
0ue o| ||e ro| ||equeu|, .e\|u, +ud co||, ||u
p|o||er.
Au ec/er+|ou (p+pu|e, .e|c|e, p|u||||c) de|
r+||||
|ue |o |ee\pou|e |o + u||+uce |o W||c| ||e
|ud|.|du+| | eu|||/ed.
AIIkCIC C0NIACI 0kMAIIIIS |C|9 . o92.9
|C|0 . |2+
Thus, alleigens aie found in jeweliy, peisonal
caie pioducts, topical medications, plants,
house iemedies, and chemicals the individual
may come in contact with at woik. The most
common alleigens in the United States aie listed
in Table 2-2.
The eiuption staits in a sensitized individual
48 h oi days aftei contact with the alleigen;
iepeated exposuies lead to a ciescendo ieac-
tion, i.e., the eiuption woisens. Site of the
eiuption is confined to site of exposuie. With
phytoalleigic (poison ivy), exposuie sites may
not be appaient to the patient. Haptens can
be blotted on to face oi penis without diiect
contact.
Symptoms Subjective symptoms aie intense
piuiitus; in seveie ieactions also stinging and
pain.
Coosttutooa| Symptoms Acute illness" syn-
diome, including fevei, but only in seveie allei-
gic contact deimatitis (e.g., poison ivy).
Sko Iesoos The appeaiance of ACD depends
on seveiity, location, and duiation.
Iype Acute Well-demaicated eiythema
and edema on which aie supeiimposed closely
spaced, nonumbilicated vesicles, and/oi papules
(Fig. 2-5); in seveie ieactions, bullae, confluent
eiosions exuding seium, and ciusts. The same
ieaction can occui aftei seveial weeks at sites
not exposed.
Suhucute Plaques of mild eiythema showing
small, diy scales, sometimes associated with
small, ied, pointed oi iounded (Figs. 2-6, 2-7),
fiim papules.
ChrvnIc Plaques of lichenification (thicken-
ing of the epideimis with deepening of the skin
lines in paiallel oi ihomboidal pattein), scaling
with satellite, small, fiim, iounded oi flat-
topped papules, excoiiations, eiythema, and
pigmentation.
SCII0N 2 EC/E\A/|Ek\AI|I|' 2T
FICk 2-5 Acute a||erc cootact dermatts oo the |ps due to |pstck I|e p+||eu| W+ |,pe|eu|
||.e |o eo|u. |o|e ||||| e|,||er+, r|c|o.e|cu|+||ou. A| c|oe |upec||ou + p+pu|+| corpoueu| c+u |e d|ce|ued.
A| ||| |+e ||e|e | |||| |+|p r+||u+||ou.
FICk 2-6 A||erc cootact dermatts oI haods:
chromates Cou||ueu| p+pu|e, .e|c|e, e|o|ou +ud
c|u| ou ||e do|ur o| ||e |e|| |+ud |u + cou||uc||ou
Wo||e| W|o W+ +||e||c |o c||or+|e.
IA8I 2-2 Iop Ien Contact A||erens (North American Contact 0ermatitis Croup)
and 0ther Common Contact A||erens
*
A||ergeo Pr|oc|pa| So0rces oI 0ootact
||c|e| u||+|e \e|+|, re|+| |u c|o|||u, jeWe||,, c+|+|,/|u +eu|
|eor,c|u u||+|e uu+||, cou|+|ued |u c|e+r, o|u|reu|
B+|+r o| |e|u Iop|c+| red|c+||ou
||+|+uce r|\ ||+|+uce, core||c
I||re|o+| Au||ep||c
'od|ur o|d |||ou||+|e \ed|c+||ou
|o|r+|de|,de |||u|ec|+u|, cu||u +eu|, p|+||c
0u+|e|u|ur5 |||u|ec|+u|
B+c|||+c|u 0|u|reu|, poWde|
Co|+|| c||o||de Cereu|, +|.+u|/+||ou, |udu|||+| o||, coo||u +eu|, e,e|+de
\e||,|d|||oro|u|+|ou|||||e, p|euo\,|e||+uo| ||ee|.+||.e, core||c
C+||+ r|\ ku||e|, |+|e\
|+|+p|eu,|eud|+r|ue B|+c| o| d+|| d,e o| |e\|||e, p||u|e|' |u|
I||u|+r ku||e|
|+|+|,d|o\,|eu/o|c +c|d e|e| Coue|.|u +eu| |u |ood|u||
||op,|eue |,co| ||ee|.+||.e, core||c
||oc+|ue, |eu/oc+|ue |oc+| +ue||e||c
'u||ou+r|de \ed|c+||ou
Iu|peu||ue 'o|.eu|, |oe po|||, p||u|e|' |u|
\e|cu|, +|| |||u|ec|+u|, |rp|eu+||ou
C||or+|e Cereu|, +u||o\|d+u|, |udu|||+| o||, r+|c|e, |e+||e|
|+|+|eue B|oc|de, p|ee|.+||.e
C|uu+r|c +|de|,de ||+|+uce, pe||ure
|eu|+dec,|c+|ec|o| ||+u|, e.., po|ou |.,
* 0.e| !100 c|er|c+| |+.e |eeu |epo||ed |o c+ue AC|.
Arraoemeot Initially, confined to aiea of
contact with alleigen e.g., eailobe (eaiiings),
doisum of foot (shoes), wiist (watch oi watch-
band), collai-like (necklace), lips (lipstick)].
Often lineai, with aitificial patteins, an outside
job." Plant contact often iesults in lineai lesions
(e.g., R|us deimatitis). Initially confined to site
of contact, latei spieading beyond.
0strbutoo Ertent Isolated, localized to
one iegion (e.g., shoe deimatitis), oi geneial-
ized (e.g., plant deimatitis).
Puttern Random oi on exposed aieas (as in
aiiboine ACD).
C0kS
vo|utoo oI AC0 The duiation of ACD vaiies
among individuals, iesolving in some in 1-2
weeks. ACD continues to get woise as long as
alleigen continues to come into contact with
the skin.
Acute Eiythema papules vesicles
eiosions ciusts scaling.
Noe. In the acute foims of contact deimati-
tis, papules occui only in ACD, not in ICD.
ChrvnIc Papules scaling licheni-
fication excoiiations. Chionic inflam-
mation with thickening, fissuiing, scaling,
and ciusting iesults.
Noe. Contact deimatitis is always con-
fined to the site of exposuie to the alleigen.
Maigination is oiiginally shaip in ACD; how-
evei, it spieads in the peiipheiy beyond the
actual site of exposuie. If stiong sensitiza-
tion has occuiied, spieading to othei paits
of the body and geneialization occui. The
main diffeiences between toxic iiiitant and
alleigic contact deimatitis aie summaiized in
Table 2-3.
0ermatopatho|oy Acute Piototype of
spongiotic deimatitis. Inflammation with in-
tiaepideimal inteicellulai edema ( songoss ),
lymphocytes and eosinophils in the epideimis,
and monocyte and histiocyte infiltiation in the
deimis.
ChrvnIc In chionic ACD theie aie also spon-
giosis plus acanthosis, elongation of iete iidges,
and elongation and bioadening of papillae; hy-
peikeiatosis; and a lymphocytic infiltiate.
Fatch Iests In ACD sensitization is piesent
on eveiy pait of the skin; theiefoie, application
of the alleigen to any aiea of noimal skin pio-
vokes an eczematous ieaction. A positive patch
test shows eiythema and papules, as well as
possibly vesicles confined to the test site. Patch
tests should be delayed until the deimatitis has
subsided foi at least 2 weeks and should be pei-
foimed on a pieviously uninvolved site.
(See Clinical Tests," Intioduction.)
By histoiy and clinical findings, including eval-
uation of site and distiibution. Histopathology
Ie|red c||-: |,|!-c|| (A||)
0ccu| |u eu|||/ed |ud|.|du+| +||e| e\pou|e
|o + W|de .+||e|, o| p|+u| +||e|eu
C|+|+c|e||/ed |, +u +cu|e, .e|, p|u||||c, ec/er
+|ou de|r+||||, o||eu |u + ||ue+| +||+uereu|
|u ||e uu||ed '|+|e, po|ou |.,/o+| +|e |, |+|
||e ro| corrou p|+u| |rp||c+|ed
||- ||,|||!-c|| | + d|||e|eu| eu|||,,
|| | + p|o|oeu|||.||, |e+c||ou occu|||u |u +u,
|ud|.|du+| W||| + p|o|oeu|||/|u p|+u|-de||.ed
c|er|c+| ou ||e ||u +ud u|equeu| uu e\po
u|e (ee 'ec||ou 0)
AIIkCIC C0NIACI 0kMAIIIIS
0 I0 FIANIS
may be helpful; veiification of offending agent
(alleigen) by patch test. Exclude ICD (Table
2-3), atopic deimatitis, seboiiheic deimatitis
(face), psoiiasis (palms and soles), epideimal
deimatophytosis (KOH), fixed diug eiuption,
eiysipelas phytophotodeimatitis.
SFCIAI F0kMS 0F AC0
FICk 2-T A||erc cootact dermatts due to ocke|, subacute |o|e + r|\ o| p+pu|+|, .e|cu|+|, +ud
c|u|ed |e|ou +ud |o o| |+|p r+||u+||ou. I|e p+||eu| W+ + |e|||ed W+|c|r+|e| W|o ued + re|+| c|+p ou
||e do|ur o| ||e |e|| |+ud W|||e |ep+|||u W+|c|e. ne W+ |uoWu |o |e +||e||c |o u|c|e|.
Ae oI 0oset Occuis in individuals of all ages.
Veiy young and veiy old aie less likely to be sen-
sitized to plants. Sensitization is lifelong.
to|oy Pentadecylcatechols, piesent in the
Anacaidiaceae plant family, aie the most com-
mon sensitizeis in the United States. They
cioss-ieact with othei phenolic compounds
such as iesoicinol, hexyliesoicinol, and hy-
dioxyquinones.
F|aots AnucurdIuceue FumI|y Poison ivy
(ToxtoJenJron raJtans ) and poison oak ( T.
quer[o|um, T. Jers|o|um ) . Also poison
sumac ( T. ernx ). Plants ielated to poison
ivy gioup: Biazilian peppei, cashew nut tiee,
ginkgo tiee, Indian maikei nut tiee, lacquei
tiee, mango tiee, iengas tiee.
Ceoraphy Poison ivy occuis thioughout the
United States (except extieme southwest) and
southein Canada; poison oak on the west coast.
Poison sumac and poison dogwood giow only
in woody, swampy aieas.
xposure Telephone and electiical woikeis
woiking outdoois. Leaves, stems, seeds, floweis,
beiiies, and ioots contain milky sap that tuins
to a black iesin on exposuie to aii. Cashew oil:
unioasted cashew nuts (heat destioys hapten);
cashew oil in wood (Haitian voodoo dolls,
swizzle sticks), iesins, piintei`s ink. Mango
iind. Maiking nut tiee of India: laundiy maikei
(dhobi itch). Fuinituie lacquei fiom Japanese
lacquei tiee.
Seasoo APD usually occuis in the spiing,
summei, and fall; can occui yeai-iound if ex-
posed to stems oi ioots. In southwest of the
United States, occuis yeai-iound.
FAIh0CNSIS
All ToxtoJenJron plants contain identical al-
leigens. Hapten is piesent in milky sap in
leaves, stems, seeds, floweis, beiiies, and ioots.
The oleoiesins aie iefeiied to as urus|o| . The
haptens aie the pentadecylcatechols (1, 2-
hydioxybenzenes with a 15-caibon side chain
in position thiee). Washing with soap and watei
iemoves oleoiesins.
Moie than 70% of individuals can be sensi-
tized to ToxtoJenJron haptens. Daik-skinned
individuals aie less susceptible to APD. Aftei
fiist exposuie (sensitization) deimatitis oc-
cuis 7-12 days latei. In a pieviously sensitized
peison (may be many decades befoie), dei-
matitis occuis (especially on face oi genitalia)
in 12 h aftei ieexposuie. Diffeience in clini-
cal couise vaiies with individual ieactivity,
inoculum of hapten on skin, and iegional
vaiiation.
Noe. Blistei fluid does not contain hapten
and cannot spiead the deimatitis; exposuie to
smoke fiom the buining plant is haimless, but
deimatitis can occui fiom paiticulate mattei in
the smoke.
xposure PvIsvn 1vy/Ou| DermutItIs Diiect
plant exposuie: plant biushes against exposed
skin giving iise to lineai lesions (Fig. 2-8); iesin
usually is not able to penetiate the thick stia-
tum coineum of palms/soles. Clothing: weaiing
clothing pieviously contaminated with iesin
can ieexpose the skin.
Fvvd CvntuInIng UrushIv| Eating unpeeled
mango oi unioasted cashew nuts can expose
lips to oleoiesin. Mucous membianes uncom-
monly expeiience APD, but ingestion of uiu-
shiol can pioduce alleigic contact deimatitis of
the anus and peiineum.
Sko Symptoms Piuiitus mild to seveie. Often
sensed befoie any detectable skin changes. Pain
in some cases. Secondaiy infection associated
with local tendeiness.
Coosttutooa| Symptoms Sleep depiivation
due to piuiitus.
Sko Iesoos Initially, well-demaicated patch-
es of eiythema, chaiacteiistic lineai lesions (Fig.
2-8); iapidly evolve into papules and edematous
plaques; may be seveie especially on face and/oi
genitals, iesembling cellulitis (Figs. 2-9, 35-16)
Miciovesiculation may evolve to vesicles and/
oi bullae (Figs. 2-8 and 2-10). Eiosions, ciusts.
With iesolution, eiythematous plaques scale,
eiosion, ciusting. Postinflammatoiy hypei-
pigmentation common in daikei skinned indi-
viduals.
DIstrIhutIvn Most commonly on exposed ex-
tiemities, wheie contact with the plant occuis;
blotting can tiansfei to any exposed site; palms/
soles aie usually spaied; howevei, lateial fingeis
can be involved.
C|vthIng-Prvtected SItes Oleoiesin can pen-
etiate damp clothing onto coveied skin.
Nvnerpvsed SItes Id"-like ieaction oi some
systemic absoiption can be associated with
disseminated uiticaiial, eiythema multifoime-
like, oi scailatinifoim lesions away fiom sites
of exposuie in some individuals with well-
established APD.
FICk 2-8 A||erc phytodermatts
oI |e: posoo vy ||ue+| .e|cu|+| |e|ou
W||| e|,||er+ +ud eder+ ou ||e c+|| +| ||e
o| d||ec| cou|+c| o| ||e ||u 5 d+, +||e| e\po
u|e W||| ||e po|ou |., |e+|.
FICk 2-9 A||erc phytoderma-
tts oI Iace: posoo vy \e|, p|u||||c
e|,||er+, eder+, r|c|o.e|cu|+||ou o| ||e
c|ee| +ud pe||o||||+| +|e+ |u + p|e.|ou|,
eu|||/ed 1,e+|o|d |o,, occu|||u ! d+,
+||e| e\pou|e.
0ermatopatho|oy See ACD, above.
Fatch Iests wth Feotadecy|catecho|s Con-
tiaindicated. Can sensitize the individual to
hapten.
0IACN0SIS
By histoiy and clinical findings.
ACD to othei alleigens, phytophotodeimatitis
(see Section 10), soft-tissue infection (celluli-
tis, eiysipelas), atopic deimatitis, inflammatoiy
deimatophytosis, eaily heipes zostei, fixed diug
eiuption.
IA8I 2-3 0ifferences Between |rritant and A||eric Contact 0ermatitis*
|rr|taot 00 A||erg|c 00
',rp|or Acu|e Stoo, smarto tcho Itcho pao
C||ou|c ||c||u/p+|u ||c||u/p+|u
|e|ou Acu|e E|,||er+ .e|c|e E|,||er+ papu|es
e|o|ou c|u| c+||u .e|c|e e|o|ou c|u| c+||u
C||ou|c |+pu|e, p|+que, ||u|e, |+pu|e, p|+que, c+||u,
c+||u, c|u| c|u|
\+||u+||ou Acu|e Sharp, strct|y cooIoed '|+|p, cou||ued |o ||e o| e\pou|e
+ud ||e to ste oI exposure but spreado o the perphery,
usua||y toy papu|es, may become
eoera|ted
C||ou|c |||de||ued |||de||ued, spreads
E.o|u||ou Acu|e kapd (|eW |ou| +||e| e\pou|e) Not so rapd (2-12 | +||e| e\pou|e)
C||ou|c \ou|| |o ,e+| o| |epe+|ed e\pou|e \ou|| o| |oue|, e\+ce||+||ou +||e|
e.e|, |ee\pou|e
C+u+||.e 0epeodeot oo cooceotratoo oI aeot ke|atve|y odepeodeot oI amouot
+eu| aod state oI sko barrer, occurs oo|y app|ed, usua||y very |ow
above thresho|d |eve| cooceotratoos suIIceot but depeods
oo deree oI seosttatoo
|uc|deuce May occur o practca||y everyooe 0ccurs oo|y o the seostted
*
||||e|euce +|e p||u|ed |u |o|d.
Cou|+c| W||| +|||o|ue +||e|eu |u e\poed |od,
||e, uo|+||, ||e |+ce (||. 2), +|o |uc|ud|u
e,e||d, '\ o| ||e uec|, +|r, +ud |e.
|u cou||+| |o +|||o|ue |C|, p+pu|+| ||or ||e
|e|uu|u, e\||ere|, ||c|,.
||o|oued |epe||||.e e\pou|e |e+d |o d|,,
||c|eu|||ed AC| W||| e|o|ou +ud c|u||u (||.
2).
|ue |o p|+u| +||e|eu, epec|+||, ||or cor
po||+e, u+|u|+| |e|u, Wood, eeu||+| o||
.o|+||/|u ||or +|or+ ||e|+p,.
AIk80kN AC0
MANACMNI 0F AC0
Iermoatoo oI xposure Identify and iemove
the etiologic agent.
A||e| ,|er|c e\pou|e |o +u +||e|eu |o W||c|
||e |ud|.|du+| |+d p||o| AC|.
A de|+,ed I ce||-red|+|ed |e+c||ou.
E\+rp|e. AC| |o e||,|eued|+r|ue u|e
queu| |e+c||ou |o +r|uop|,|||ue (W||c| cou|+|u
e||,|eue d|+r|ue), po|ou |., de|r+||||
u|equeu| |e+c||ou |o |ue||ou o| c+|eW
uu|, +|o +u||||o||c, u||ou+r|de, p|op,|eue
|,co|, re|+| |ou, o|||c +c|d, ||+|+uce.
SSIMIC AC0 (SAC0)
Iopca| Iherapy Topical glucocoiticoid oint-
ments/gels (classes I to III) aie effective foi
eaily nonbullous lesions. Laigei vesicles may be
diained, but tops should not be iemoved. Wet
diessings with cloths soaked in Buiow solu-
tion changed eveiy 2-3 h. Since tieatment with
glucocoiticoids is usually shoit-teim in ACD,
theie is usually no dangei of glucocoiticoid side
effects. An exception is aiiboine ACD, which
may iequiie systemic tieatment. The topical
calcineuiin inhibitois pimeciolimus and tacio-
limus aie effective in ACD but to a lessei degiee
than glucocoiticoids.
Systemc Iherapy Glucocoiticoids aie indi-
cated if seveie (i.e., if patient cannot peifoim
usual daily functions, cannot sleep). Piednisone
beginning at 70 mg (adults), tapeiing by 5-10
mg/d ovei a 1- to 2-week peiiod.
In aiiboine ACD wheie complete avoidance
of alleigen may be impossible, immunosup-
piession with oial cyclospoiine may become
necessaiy.
FICk 2-10 Acute a||erc phytodermatts, bu||ous I|| e|up||ou occu||ed |u + p+||eu| W|o |+d W+||ed |+|e
|oo| |||ou| + |o|e|. || |+|e| p|e+d + + p+pu|+| e|up||ou |o ||e |e| o| ||e |od,. '|r||+| |e|ou We|e p|eeu| ou ||e o||e|
|oo| +ud |oWe| |e. ||||e|eu||+| d|+uo| |uc|uded +cu|e |u||ou cou|+c| de|r+|||| |o c+|e|p|||+|. ||,|op|o|ode|r+||||
W+ e\c|uded |ec+ue +| ||e ||re o| e\pou|e ||e|e W+ + |e+.||, c|ouded |, +ud + p+pu|+| e|up||ou occu||ed |+|e| ou.
C+|e|p|||+| de|r+|||| W+ e\c|uded |ec+ue o| ||e ru|||p||c||, o| ||e |e|ou +ud |ec+ue upou p+|c| |e||u ||e p+||eu| W+
po|||.e |o |o\|codeud|ou |+p|eu. |o|e, p+|c| |e||u |o u|u||o| | uo |oue| doue |o +.o|d eu|||/+||ou o| p+||eu|.
FICk 2-11 Arboroe a||erc cootact dermatts
oo the Iace E\||ere|, ||c|,, cou||ueu| p+pu|+|, e|o|.e,
+ud c|u|ed/c+|, |e|ou W||| ||c|eu|||c+||ou ou ||e |o|e|e+d,
|o||oW|u e\pou|e |o p|ueWood du|.
FI0MI0I0C
Ae oI 0oset Fiist 2 months of life and by the
fiist yeais in 60% of patients. 30% aie seen foi
the fiist time by age 5, and only 10% develop
AD between 6 and 20 yeais of age. Raiely AD
has an adult onset.
Ceoder Slightly moie common in males than
females.
Freva|eoce Between 7 and 15% iepoited in
population studies in Scandinavia and Gei-
many.
Ceoetc Aspects The inheiitance pattein has
not been asceitained. Howevei, in one seiies,
60% of adults with AD had childien with AD.
The pievalence in childien was highei (81%)
when both paients had AD.
|cto Factors 1nhu|unts Specific aeioal-
leigens, especially dust mites and pollens, have
been shown to cause exaceibations of AD.
MIcrvhIu| Agents Exotoxins of Sa|y|otottus
aureus may act as supeiantigens and stimulate
activation of T cells and maciophages.
Autvu||ergens Seia of patients with AD
contain IgE antibodies diiected at human pio-
teins. The ielease of these autoalleigens fiom
damaged tissue could tiiggei IgE oi T cell
iesponses, suggesting maintenance of alleigic
inflammation by endogenous antigens.
Fvvds Su|se of infants and childien have
flaies of AD with eggs, milk, peanuts, soybeans,
fish, and wheat.
0ther xacerbato Factors
S|In BurrIer DIsruptIvn: deciease of baiiiei
function associated with impaiied filag-
giin pioduction, ieduced ceiamide levels,
and incieased tiansepideimal watei loss
Au +cu|e, u|+cu|e, o| c||ou|c |e|+p|u ||u
d|o|de|
uu+||, |e|u |u |u|+uc,
||e.+|+uce pe+| o| 5-20 |u e+||, c|||d|ood
C|+|+c|e||/ed p||uc|p+||, |, d|, ||u +ud p|u|||u,
couequeu| |u|||u |e+d |o |uc|+ed |u||+rr+
||ou +ud ||c|eu|||c+||ou +ud |o |u|||e| ||c||u +ud
c|+|c||u. |:|:c|:| :,:|-
||+uo| | |+ed ou c||u|c+| ||ud|u
0||eu +oc|+|ed W||| + pe|ou+| o| |+r||, |||o|,
o| A|, +||e||c |||u|||, +ud +||r+, !5 o| |u|+u|
W||| A| de.e|op +||r+ |+|e| |u |||e
Aoc|+|ed W||| ||u |+|||e| d,|uuc||ou, |E |e+c
||.||,
Ceue||c |+| |u||ueuced |, eu.||oureu|+| |+c
|o|, +||e|+||ou |u |rruuo|o|c |epoue |u
I ce||, +u||eu p|oce|u, |u||+rr+|o|, c,|o||ue
|e|e+e, +||e|eu eu|||.||,, |u|ec||ou
',,. |E de|r+||||, 'ec/er+, +|op|c ec
/er+.
AI0FIC 0kMAIIIIS |C|9 . o9.3
|C|0 . |20
by fiequent bathing and hand washing;
dehydiation is an impoitant exaceibating
factoi.
1n]ectIvns: S. aureus is almost always piesent
in seveie cases; gioup A stieptococcus;
iaiely fungus (deimatophytosis, candidia-
sis).
Seusvn: in tempeiate climates, AD usually
impioves in summei, flaies in wintei.
C|vthIng: piuiitus flaies a[er taking off
clothing. Wool is an impoitant tiiggei;
wool clothing oi blankets diiectly in con-
tact with skin (also wool clothing of pai-
ents, fui of pets, caipets).
EmvtIvnu| Stress: iesults fiom the disease oi
is itself an exaceibating factoi in flaies of
the disease.
FAIh0CNSIS
Complex inteiaction of skin baiiiei, genetic,
enviionmental, phaimacologic, and immuno-
logic factois. Type I (IgE-mediated) hypeisen-
sitivity ieaction occuiiing as a iesult of the
ielease of vasoactive substances fiom both mast
cells and basophils that have been sensitized
by the inteiaction of the antigen with IgE (ie-
aginic oi skin-sensitizing antibody). The iole
of IgE in AD is still not fully claiified, but epi-
deimal Langeihans cells possess high-affinity
IgE ieceptois thiough which an eczema-like
ieaction can be mediated. T
H
2 and T
H
1 both
contiibute to skin inflammation in AD. Actute
T cell infiltiation in AD is associated with a
piedominance of inteileukin (IL) 4 and IL-13
expiession, and chionic inflammation in AD
FICk 2-12 Atopc dermatts: oIaot|e |u||, |+ce, cou||ueu| e|,||er+, p+pu|e, r|c|o.e|cu|+||ou, c+|
|u, +ud c|u||u
FICk 2-13 Atopc dermatts: oIaot|e-type '||u o| |o|e|e+d | d|,, c|+c|ed +ud c+|,. |u +dd|||ou, ||e|e
+|e oo/|u e|o|ou.
with incieased IL-5, gianulocyte-maciophage
colony-stimulating factoi (GM-CSF), IL-12,
and inteifeion (IFN) . Thus, skin inflamma-
tion in AD shows a biphasic pattein of T cell
activation.
Sko Symptoms Patients have diy skin. Piuii-
tus is the sine qua non of atopic deimatitis-
eczema is the itch that iashes." The constant
sciatching leads to a vicious cycle of itch
sciatch iash itch sciatch.
0ther Symptoms oI Atopy Alleigic ihinitis,
chaiacteiized by sneezing, ihinoiihea, obstiuc-
tion of nasal passages, conjuctival and phaiyn-
geal itching, and laciimation; seasonal when
associated with pollen.
Sko Iesoos Acute Pooily defined eiy-
thematous patches, papules, and plaques with
oi without scale. Edema with widespiead in-
volvement; skin appeais puffy" and edematous
(Fig. 2-12). Eiosions: moist, ciusted. Lineai oi
punctate, iesulting fiom sciatching. Secondaiily
infected sites: S. aureus. Oozing eiosions (Figs.
2-12 and 2-13) and/oi pustules (usually follicu-
lai). Skin may be extiemely diy and ciacked and
scaly (Fig. 2-13).
ChrvnIc Lichenification (thickening of the
skin with accentuation of skin maikings): iesults
fiom iepeated iubbing oi sciatching (Figs. 2-14
and 2-15); folliculai lichenification (especially
in biown and black peisons) (Fig. 2-16). Fis-
suies: painful, especially in flexuies (Fig. 2-15),
on palms, fingeis, and soles. Alopecia: lateial
one-thiid of the eyebiows as a iesult of iubbing.
Peiioibital pigmentation: also as a iesult of
compulsive iubbing . Chaiacteiistic infiaoi-
bital fold below eyelids (Dennie-Moigan sign).
DIstrIhutIvn Piedilection foi the flex-
uies, fiont and sides of the neck, eyelids,
foiehead, face, wiists, and doisa of the feet
and hands (Image 2-1). Geneialized in seveie
disease (Fig. 2-17).
Speca| Features ke|ated to Ae
1n]untI|e AD The lesions piesent as ied skin,
tiny vesicles on puffy" suiface. Scaling, exuda-
tion with wet ciusts and ciacks (fissuies) (Figs.
2-12 to 2-14). Skin lesions seem to be a ieaction
to itching and iubbing.
ChI|dhvvd-type AD The lesions aie papulai,
lichenified plaques, eiosions, ciusts, especially
on the antecubital and popliteal fossae (Figs.
2-15, to 2-17), the neck and face; may be gen-
eialized.
Adu|t-type AD Theie is a similai distiibution,
mostly flexuial but also face and neck, with
lichenification and exoiiations being the most
conspicuous symptoms (Figs. 2-18, 2-19). May
be geneialized.
Speca| Features ke|ated to thocty
In blacks but also daik-biown skin, so-called
folliculai eczema is common; chaiacteiized
by disciete folliculai papules (Figs. 2-16, 2-19,
2-20) involving haii follicles of the involved
site.
Assocated Fodos
White" deimatogiaphism is a special and
unique featuie of involved skin: stioking will
not lead to iedness as in noimal skin but to
blanching ; delayed blanch to cholineigic
agents. It||yoss u|gars and |eraoss |ars
(see page 75) occui in 10% of patients. Veinal
conjuctivitis with papillaiy hypeitiophy oi cob-
blestoning of uppei eyelid conjuctiva. Atopic
keiatoconjunctivitis is disabling, may iesult in
coineal scaiiing. Keiatoconus iaie. Cataiacts in
a small peicentage.
0IACN0SIS
Histoiy in infancy, clinical findings (typical
distiibution sites, moiphology of lesions, white
deimatogiaphism).
Seboiiheic deimatitis, ICD, ACD, psoiiasis,
nummulai eczema, deimatophytosis, eaily
stages of mycosis fungoides. Raiely, aciodei-
matitis enteiopathica, glucagonoma syndiome,
histidinemia, phenylketonuiia; also, some im-
munologic disoideis including Wiskott-Aldiich
syndiome, X-linked agammaglobulinemia, hy-
pei-IgE syndiome, and selective IgA deficiency;
Langeihans cell histiocytosis, Letteiei-Siwe
type.
8actera| Cu|ture Colonization with S. aureus
veiy common in the naies and in the involved
skin; almost 90% of patients with seveie AD aie
secondaiily colonized/infected. Look out foi
methicillin-iesistant S. aureus (MRSA).
vra| Cu|ture Rule out heipes simplex viius
(HSV) infection in ciusted lesions (eczema
heipeticum; see Section 27).
FICk 2-14 Ch|dhood atopc dermatts A |,p|c+| |oc+||/+||ou o| +|op|c de|r+|||| |u c|||d|eu | ||e |e|ou
+|ouud ||e rou||. |u ||| c|||d ||e|e | ||c|eu|||c+||ou +ud ||u||u +ud c|u||u.
FICk 2-15 Ch|dhood atopc dermatts 0ue o| ||e |+||r+|| o| +|op|c de|r+|||| | ||c|eu|||c+||ou |u ||e
||e\u|+| |e|ou + |oWu |u ||| p|c|u|e. |o|e ||e |||c|eu|u o| ||e ||u W||| e\+e|+|ed ||u ||ue +ud e|o|ou.
8|ood Studes Incieased IgE in seium, eosino-
philia. HSV antigen detection foi diagnosis of
acute HSV infection.
0ermatopatho|oy Vaiious degiees of acan-
thosis with iaie intiaepideimal inteicellulai
edema (spongiosis). The deimal infiltiate is
composed of lymphocytes, monocytes, and
mast cells with few oi no eosinophils.
SFCIAI F0kMS 0F A0
Hund DermutItIs Aggiavated by wetting and
washing with deteigents, haish soaps, and Js-
n[etans, leads to ICD in the atopic. Clini-
cally indistinguishable fiom noimal" ICD
(see p. 22).
Er]v|IutIve DermutItIs (See SectIvn 8) Eiyth-
iodeima in patients with extensive skin
involvement. Geneialized iedness, scaling,
weeping, ciusting, lymphadenopathy, fevei, and
systemic toxicity.
C0MFIICAII0NS
Secondaiy infection with S. aureus and
heipes simplex viius (eczema heipeticum, see
Section 27). Raiely keiatoconus, cataiacts and
keiatoconjunctivitis with secondaiy heipetic
infection and coineal ulceis.
C0kS AN0 Fk0CN0SIS
Untieated involved sites peisist foi months
oi yeais. Spontaneous, moie oi less complete
iemission duiing childhood occuis in >40%
with occasional, moie seveie iecuiiences dui-
ing adolescence. In many patients, the disease
peisists foi 15-20 yeais, but is less seveie. Fiom
30 to 50% of patients develop asthma and/oi
hay fevei. Adult-onset AD often iuns a seveie
couise. S. aureus infection leads to extensive
eiosions and ciusting, and heipes simplex in-
fection to eczema heipeticum, which may be
life-thieatening (see Section 27).
MANACMNI
Education of the patient to avoid iubbing and
sciatching is most impoitant. Topical antipiu-
iitic (menthol/camphoi) lotions aie helpful in
contiolling the piuiitus but aie useless if emol-
lients aie not used and the patient continues to
sciatch and iub the plaques.
An alleigic woikup is
iaiely helpful in uncovei-
ing an alleigen; howevei,
in patients who aie hy-
peisensitive to house dust
mites, vaiious pollens,
and animal haii pioteins,
exposuie to the appio-
piiate alleigen may cause
flaies. Atopic deimatitis is
consideied by many to be
ielated, at least in pait, to
emotional stiess. It may
exaceibate with sweating.
Patients should be
wained of theii special
pioblems with heipes
simplex and the fiequency
of supeiimposed staphy-
lococcal infection, foi
which oial antibiotics aie
indicated. Antiviial diugs
foi heipes simplex aie in-
dicated if HSV infection
is suspected.
IMAC 2-1 Fred|ectoo stes oI atopc dermatts.
Acute
1. Wet diessings and topical glucocoiticoids;
topical antibiotics (mupiiocin ointment)
when indicated.
2. Hydioxyzine, 10-100 mg foui times daily
foi piuiitus.
3. Oial antibiotics (dicloxacillin, eiythiomy-
cin) to eliminate S. aureus and tieat MRSA
accoiding to sensitivity as shown by cultuie.
Subacute aod Chrooc
1. Hydiation (oilated baths oi baths with oat-
meal powdei) followed by application of
unscented emollients (e.g., hydiated petiola-
tum) foim the basic daily tieatment needed
to pievent xeiosis. Soap showeis aie peimis-
sible to wash the body folds, but soap should
seldom be used on the othei paits of the skin
suiface. 12% ammonium lactate oi 10% -
hydioxy acid lotion is veiy effective foi the
xeiosis seen in AD.
2. Topical anti-inflammatoiy agents such as
glucocoiticoids, hydioxyquinoline piep-
aiations, and tai aie the mainstays of
tieatment. Of these, glucocoiticoids aie the
most effective. Howevei, topical glucocoi-
ticoids may lead to skin atiophy if used
foi piolonged peiiods of time and if used
excessively will lead to suppiession of the
pituitaiy-adienal axis, osteopoiosis, giowth
ietaidation. Anothei pioblem is glucocoi-
ticoidophobia." Patients oi theii paients aie
incieasingly awaie of glucocoiticoid side
effects and iefuse theii use, no mattei how
beneficial they may be.
3. New topical nonsteioidal anti-inflammatoiy
agents, the calcineuiin inhibitois taciolimus
and pimeciolimus, aie giadually ieplacing
glucocoiticoids in most patients. They po-
tently suppiess itching and inflammation
and do not lead to skin atiophy. They aie
usually not effective enough to suppiess
acute flaies but woik veiy well in minoi
flaies and subacute atopic deimatitis.
4. Oial H
1
antihistamines aie useful in ieduc-
ing itching.
FICk 2-16 Atopc dermatts o b|ack ch|d: Io||cu|ar ||u||||c |o|||cu|+| p+pu|e ou ||e po|e||o| |e.
|o|||cu|+| ec/er+ | + |e+c||ou p+||e|u ||+| occu| ro|e corrou|, |u A|||c+u +ud A|+u c|||d|eu.
5. Systemic glucocoiticoids should be avoided,
except in iaie instances in adults foi only
shoit couises (iescue tieatment). They aie
widely oveiused. Osteopenia and cataiacts
aie complications. Foi seveie intiactable dis-
ease, piednisone, 60-80 mg daily foi 2 days,
then halving the dose each 2 days foi the
next 6 days. Patients with AD tend to become
dependent on oial glucocoiticoids. Often,
small doses (5-10 mg) make the diffeience
in contiol and can be ieduced giadually to
even 2.5 mg/d, as is often used foi the contiol
of asthma. Intiamusculai glucocoiticoids aie
iisky and should be avoided.
6. UVA-UVB phototheiapy (combination of
UVA plus UVB and incieasing the iadiation
dose each tieatment, with a fiequency of two
to thiee times weekly). Naiiow band UV
(311 nm), PUVA photochemotheiapy also
effective.
7. In seveie cases of adult AD and in noimo-
tensive healthy peisons without ienal dis-
ease cyclospoiine tieatment (staiting dose
5 mg/kg pei day) is indicated when all othei
tieatments fail, but should be monitoied
closely. Tieatment is limited to 3-6 months
because of potential side effects, including
hypeitension and ieduced ienal function.
Blood piessuie should be checked weekly
and chemistiy panels biweekly. Nifedipine
can be used foi modeiate incieases in blood
piessuie.
8. Patients should leain and use stiess manage-
ment techniques.
9. A suggested algoiithm of AD management is
as follows (see Image 2-2):
Baseline theiapy of diyness with emol-
lients
Suppiession of mild to modeiate AD by
piolonged topical pimeciolimus oi tacio-
limus and continued emollients
Supiession of seveie flaies with topical
glucocoiticoids followed by pimecioli-
mus oi taciolimus and emollients
Oial and topical antibiotics to eliminate
S. aureus
Hydioxyzine to suppiess piuiitus
Website: |.//www.aaJ.org/am||es/et:ema.
|m|.
FICk 2-1T Ch|dood atopc dermatts I|| | + eue|+||/ed e|up||ou cou|||u o| cou||ueu|, |u||+rr+
|o|, p+pu|e ||+| +|e e|o|.e, e\co||+|ed, +ud c|u|ed.
FICk 2-18 Adu|t atopc dermatts o dark sko Ceue|+||/ed e|up||ou o| |o|||cu|+| p+pu|e ||+| +|e ro|e
|e+.||, p|reu|ed ||+u uo|r+| ||u |u + 5!,e+|o|d Wor+u o| A|||c+u e\||+c||ou.
IMAC 2-2 Ireatmeot a|orthm oI A0.
FAIh0CNSIS
A special piedilection of the skin to iespond to
physical tiauma by epideimal hypeiplasia; skin
becomes highly sensitive to touch. The veiy
abnoimal itching hypeiexcitability of licheni-
fied skin aiises in iesponse to minimal exteinal
stimuli that would not elicit an itch iesponse
in noimal skin. Emotional stiess in some cases.
It becomes a habit and may peisist foi months
to yeais, with iesulting maiked lichenification.
A pec|+| |oc+||/ed |o|r o| ||c|eu|||c+||ou, occu|
||u |u c||curc|||ed p|+que.
keu|| ||or |epe||||.e |u|||u +ud c|+|c||u.
||c|eu|||c+||ou | + c|+|+c|e||||c |e+|u|e o| +|op|c
de|r+||||, W|e||e| eue|+||/ed o| |oc+||/ed.
|'C c+u |+| |o| dec+de uu|e ||e |u|||u +ud
c|+|c||u +|e |opped |, ||e+|reu|.
0ccu| |u |ud|.|du+| o|de| ||+u 20 ,e+|, | ro|e
||equeu| |u Woreu, +ud po|||, ro|e ||equeu|
|u A|+u.
IIChN SIMFIX Chk0NICS (|'C) |C|9 . o93.!
|C|0 . |23
Many patients have AD oi an atopic back-
giound.
Skin symptoms consist of piuiitus, often
in paioxysms. The lichenified skin is like an
eiogenous zone-it becomes a pleasuie (oi-
giastic) to sciatch. Often the aieas on the feet
aie iubbed at night with the heel and the toes.
The iubbing becomes automatic and ieflexive
and an unconscious habit. Most patients with
LSC give a histoiy of itch attacks staiting fiom
minoi stimuli: putting on clothes, iemoving
FICk 2-20 Adu|t atopc dermatts o heav|y
pmeoted Asao sko I|e|e +|e ru|||p|e p|u||||c
|o|||cu|+| p+pu|e ||+| +|e + |,p|c+| |e+c||ou p+||e|u |u
A|||c+u +ud A|+u ||u.
FICk 2-19 Adu|t atopc dermatts ||c|eu|||
c+||ou doe uo| ou|, occu| |u ||e || ||e\u|+| |o|d |u|
r+, +|o +||ec| ||e |+ce |u ||| 5!,e+|o|d Wor+u o|
|udoue|+u e\||+c||ou.
ointments, clothes iubbing the skin; in bed, the
skin becomes waimei and the waimth piecipi-
tates itching.
Sko Iesoos A solid plaque of lichenification,
aiising fiom the confluence of small papules;
scaling is minimal except on lowei extiemities
(Fig. 2-21). Lichenified skin is palpably thick-
ened; skin maikings (baiely visible in noimal
skin) aie accentuated and can be seen ieadily.
Excoiiations aie often piesent. Usually dull ied,
latei biown oi black hypeipigmentation, espe-
cially in skin phototypes IV, V, and VI. Round,
oval, lineai (following path of sciatching).
Usually shaiply defined. Isolated single lesion oi
seveial iandomly scatteied plaques. Nuchal aiea
(female) (Fig. 2-21), scalp, ankles, lowei legs,
uppei thighs, exteiioi foieaims, vulva, pubis,
anal aiea, sciotum (See Fig. 35-18), and gioin.
In black skin, lichenification may assume
a special type of pattein-theie is not a solid
plaque, but the lichenification consists instead
of a multitude of small (2- to 3-mm) closely set
papules-i.e., a folliculai" pattein (as in Fig.
2-16).
Includes a chionic piuiitic plaque of psoiiasis
vulgaiis, eaily stages of mycosis fungoides, ICD,
ACD, epideimal deimatophytosis.
0ermatopatho|oy Hypeiplasia of all compo-
nents of epideimis: hypeikeiatosis, acanthosis,
and elongated and bioad iete iidges. Spongiosis
is infiequent. In the deimis theie is a chionic
inflammatoiy infiltiate.
MANACMNI
Difficult. Repeatedly explain to the patient that
the iubbing and sciatching must be stopped.
It is impoitant to apply occlusive bandages at
night to pievent iubbing. Topical glucocoiti-
coid piepaiations oi tai piepaiations such as
combinations of 5% ciude coal tai in zinc oxide
paste plus class II glucocoiticoids all coveied by
occlusive diessings aie effective foi body aieas
wheie this appioach is feasible (e.g., legs, aims).
Occlusive diessings: topical glucocoiticoids aie
applied to lesion and coveied by an occlusive
(plastic) diessing (like saian wiap). Glucocoi-
ticoids incoipoiated in adhesive plastic tape aie
also veiy effective, left foi 24 houis. Unna Boot:
a gauze ioll diessing impiegnated with zinc ox-
ide paste is wiapped aiound a laige lichenified
aiea such as the calf. The diessing can be left on
foi up to 1 week.
Intialesional tiiamcinolone is often highly
effective in smallei lesions (3 mg/mL; highei
concentiations may cause atiophy). Oial hy-
dioxyzine, 25-50 g at night, may be helpful.
FICk 2-21 Icheo smp|ex chroocus Cou
||ueu|, p+pu|+|, |o|||cu|+| ec/er+, c|e+||u + p|+que
o| ||c|eu |rp|e\ c||ou|cu o| ||e po|e||o| uec| +ud
occ|p||+| c+|p. Coud|||ou |+d |eeu p|eeu| |o| r+u,
,e+| + + |eu|| o| c||ou|c |u|||u o| ||e +|e+.
| o||eu +oc|+|ed W||| A| o| occu| W|||ou| A|.
|| p+||eu| W||| A| +|e ,ouue| +ud |+.e |e+c
||.||, |o eu.||oureu|+| +||e|eu, uou+|op|c ||
p+||eu| +|e o|de| +ud |+c| |,pe|eu|||.|||e |o
eu.||oureu|+| +||e|eu.
|| |+|| W||| p|e|c|u p|u|||u ||+| |e+d |o p|c|
|u +ud c|+|c||u.
|ore|+ped uodu|e-e.e|+| r||||re|e| |o 2
cr-de.e|op ou ||e |u W||c| pe|||eu| ||c||u
+ud c|+|c||u occu| (||. 222).
|odu|e +|e o||eu e|oded, e\co||+|ed, +ud ore
||re e.eu u|ce|+|ed + p+||eu| d| |u|o ||er
W||| ||e|| u+||.
uu+||, ru|||p|e ou ||e e\||er|||e.
|| uu+||, occu| |u ,ouue| o| r|dd|e+e
|er+|e, W|o o||eu e\||||| |u o| ueu|o||c ||
r+||/+||ou.
|e|ou pe||| |o| rou|| +||e| ||e ||+ur+ |+
|eeu d|cou||uued.
I|e+|reu|. |u||+|e|ou+| |||+rc|uo|oue, occ|u|.e
d|e|u W||| |||po|euc, |ucoco|||co|d. |u
e.e|e c+e, ||+||dor|de 50-00 r. w+|c| ou|
|o| cou||+|ud|c+||oul |0 ueu|ou||u !00 r I||
r+, |e |e|p|u|.
FkkIC0 N00IAkIS (FN) |C|9 o93.!
|C|0 . |23.
FICk 2-22 Fruro oodu|ars \u|||p|e, |||r, e\co||+|ed uodu|e +|||u +| ||e o| c||ou|c+||, p|c|ed o|
e\co||+|ed ||u. 0||eu occu|||u |u p+||eu| W||| +|op, |u| +|o W|||ou| ||. I|| | + !o,e+|o|d r+|e W||| n|\ d|e+e.
ne |+d \k'A ecoud+|, |u|ec||ou o| p|u||o uodu|e. Ce||u|||| o|||u+|ed |u p|u||o uodu|e |equ|||u ru|||p|e
|op||+||/+||ou.
8actera| Cu|ture Vesicles of DED aie steiile.
But iule out S. aureus infection.
k0h Freparatoo Rule out epideimal deima-
tophytosis.
0ermatopatho|oy Eczematous inflammation
(spongiosis and intiaepideimal edema) with
intiaepideimal vesicles.
C0kS AN0 Fk0CN0SIS
Recuiient attacks aie the iule. Spontaneous
iemissions in 2-3 weeks. Inteival between at-
tacks is weeks to months. Secondaiy infection
may complicate the couise: pustules, ciusts,
cellulitis, lymphangitis, and painful lymphade-
nopathy. Disabling because of seveie, fiequently
iecuiiing outbieaks.
MANACMNI
Wet 0resso Foi vesiculai stage: Buiow wet
|,||d|o||c ec/er+ | + pec|+| .e|cu|+| |,pe o|
|+ud +ud |oo| de|r+||||.
Au +cu|e, c||ou|c, o| |ecu||eu| de|r+|o| o| ||e
||ue|, p+|r, +ud o|e.
'uddeu oue| o| r+u, deepe+|ed p|u||||c, c|e+|
'|+p|oc+|||e .e|c|e (||. 22!).
|+|e |u||+e +ud |+c|e||+| |u|ec||ou c+u occu|.
|+|e|, c+||u ||u|e +ud ||c|eu|||c+||ou.
',, |orp|o|,\, .e|cu|+| p+|r+| ec/er+.
|C|9 . 105.3
|C|0 . |!0.
0ShI0k0IIC CIMAI0S 0kMAIIIIS (00)
diessings. Laige bullae diained with a punctuie
but not unioofed.
Fssures Topical application of flexible col-
lodion.
C|ucocortcods
TvpIcu| High-potency glucocoiticoids with
plastic occlusive diessings foi 1 to maximum
of 2 weeks.
1ntru|esIvnu| 1nectIvn Tiiamcinolone, 3 mg/
mL. Veiy effective foi small aieas of involve-
ment.
SystemIc In seveie cases, a shoit, tapeied
couise of piednisone can be given: 70 mg/d,
tapeiing by 10 oi 5 mg/d ovei 7 oi 14 days.
Systemc Aotbotc Foi suspected (localized
pain) oi documented secondaiily infected le-
sions (usually S. aureus, less commonly gioup
A stieptococcus).
FvA (See pae 68) Oial oi topical as soaks."
Successful in many patients if given ovei pio-
longed peiiods of time and woith tiying, espe-
cially in seveie cases.
FICk 2-23 0yshdrotc ectematous dermatts Cou||ueu| |+p|oc+|||e .e|c|e +ud c|u|ed (e\co||+|ed)
e|o|ou ou ||e do|ur o| ||ue| +ud ||ue| We|.
FI0MI0I0C
Two peaks in incidence: young adulthood and
old age. Fall and wintei.
FAIh0CNSIS
Unknown. Unielated to atopic diathesis; IgE
levels noimal. Incidence peaks in wintei, when
xeiosis is maximal. S. aureus often piesent but
pathogenic significance not pioven.
Sko Symptoms Piuiitus, often intense.
Sko Iesoos Closely giouped, small vesicles
and papules that coalesce into plaques (Fig.
2-24A), often moie than 4 to 5 cm in diametei,
with an eiythematous base with distinct boi-
deis. Plaques may become exudative and ciust
(Fig. 2-24B). Excoiiations secondaiy to sciatch-
ing. Diy scaly plaques that may be lichenified.
Round oi ton-s|aeJ (Fig. 2-24A), hence the
adjective nummu|ar (Latin: nummu|ars, like
a coin"). Maigins often moie pionounced than
centei.
DIstrIhutIvn Regional clusteis of lesions (e.g.,
on legs oi tiunk) oi geneialized, scatteied.
Lowei legs (oldei men), tiunk, hands and fin-
geis (youngei females).
Sca|o F|aques Epideimal deimatophytosis,
ICD oi ACD, psoiiasis, eaily stages of mycosis
fungoides, impetigo, familial pemphigus.
|urru|+| ec/er+ | + c||ou|c, p|u||||c, |u
||+rr+|o|, de|r+|||| occu|||u |u ||e |o|r
o| co|u|+ped p|+que corpoed o| |ouped
r+|| p+pu|e +ud .e|c|e ou +u e|,||er+|ou
|+e.
|| | epec|+||, corrou ou ||e e\||er|||e du||u
W|u|e| rou||, o||eu eeu |u +|op|c |ud|.|du+|,
',,. ||co|d ec/er+, r|c|o||+| ec/er+.
|C|9 . o92.9
|C|0 . |!0.9
NMMIAk CIMA (N)
8actera| Cu|ture Rule out S. aureus infection.
0ermatopatho|oy Subacute inflammation
with acanthosis and spongiosis.
C0kS AN0 Fk0CN0SIS
Chionic. Lesions last fiom weeks to months.
Often difficult to contiol even with potent topi-
cal glucocoiticoid piepaiations.
MANACMNI
Sko hydratoo Moistuiize" involved skin af-
tei bath oi showei with hydiated petiolatum oi
othei moistuiizing cieam.
C|ucocortcods TvpIcu| PrepurutIvns
Classes I and II applied twice daily until
lesions have iesolved. Steioid impiegnated
tape. Inra|esona| tiiamcinolone, 3 mg/mL.
Crude Coa| Iar 2-5% ciude coal tai ointment
daily. May be combined with glucocoiticoid
piepaiation. Tai baths aie useful in patients
with iefiactoiy lesions.
Systemc Iherapy Systemic antibiotics if S.
aureus is piesent.
FvA or v8 311-om Iherapy Veiy effective.
FICk 2-24 Nummu|ar ectema ||u||||c, |ouud, uurru|+| (co|u|+ped) p|+que W||| e|,||er+, c+|e,
+ud c|u| ou ||e |o|e+|r. A c|oeup o| + |e|ou |u +uo||e| p+||eu| |e.e+| ||+| ||| |u||+rr+|o|, p|+que cou||
o| cou||ueu| p+pu|o.e|cu|+| |e|ou ||+| oo/e + e|ou ||u|d +ud |e+d |o c|u||u +ud +|e uu+||, ,e||oW.

Au o||eu uu|ecou|/ed eue|+||/ed p|u||||c de|
r+|||| d||ec||, |e|+|ed |o + p||r+|, de|r+||||
e|eW|e|e.
|o| e\+rp|e, + p+||eu| W||| .euou |+| de|r+
|||| ou ||e |oWe| |e r+, de.e|op p|u||||c, ,r
re|||c, c+||e|ed, e|,||er+|ou, r+cu|op+pu|+|,
o| p+pu|o.e|cu|+| |e|ou ou ||e ||uu|, |o|e+|r,
||||, o| |e.
I|ee pe||| +ud p|e+d uu||| ||e |+|c uude||,
|u p||r+|, de|r+|||| | cou||o||ed.
'|r||+||,, +u|oeu|||/+||ou r+, occu| + +u '|d
|e+c||ou |u |u||+rr+|o|, ||ue+ ped| +ud r+u|
|e| + + d,||d|o||o|r, .e|cu|+| e|up||ou ou
||e |ee| +ud |+ud (||. 225) +ud p+pu|o.e|cu
|+| ec/er+|o|d |e|ou ou ||e ||uu|.
I|e p|euoreuou |eu|| ||or ||e |e|e+e o|
c,|o||ue |u ||e p||r+|, de|r+||||, + + |eu||
o| eu|||/+||ou. I|ee c,|o||ue c||cu|+|e |u ||e
||ood +ud |e|||eu ||e eu|||.||, o| ||e d||+u|
||u +|e+.
I|e d|+uo| o| +u|oeu|||/+||ou de|r+|||| | o|
|eu | |:, |.e., ||e d||+u| e|up||ou d|+ppe+|
W|eu ||e p||r+|, de|r+|||| | cou||o||ed.
0|+| |ucoco|||co|d |+|eu ||e d|+ppe+|+uce o|
||e |e|ou.
AI0SNSIIIIAII0N 0kMAIIIIS |C|9 . o92.9
|C|0 . |!0.9
A .e|, corrou c||ou|c de|r+|o| c|+|+c|e||/ed
|, |edue +ud c+||u +ud occu|||u |u |e|ou
W|e|e ||e e|+ceou |+ud +|e ro| +c||.e, uc|
+ ||e |+ce +ud c+|p, ||e p|e|e|u+| +|e+, +ud |u
||e |od, |o|d. \||d c+|p '| c+ue ||+||u, |.e.,
d+ud|u||.
Ceue|+||/ed '|, |+||u|e |o ||||.e, +ud d|+|||e+ |u
+u |u|+u| |ou|d |||u |o r|ud |e|ue| d|e+e W|||
+ .+||e|, o| |rruuode||c|euc, d|o|de|.
',,. 'C|+d|e c+p (|u|+u|), p||,||+| |cc+
(d+ud|u||).
S80kkhIC 0kMAIIIIS (S0) |C|9 . o09.
|C|0 . |2.9
Ae oI 0oset Infancy (within the fiist months),
pubeity, most between 20 and 50 yeais oi
oldei.
Sex Moie common in males.
Iocdeoce 2 to 5% of the population.
Fredsposo aod xacerbato Factors In
immunocompetent patients theie is often a
heieditaiy diathesis, the so-called seboiiheic
state, with maiked seboiihea and maiginal
blephaiitis. May be associated with psoiiasis
as a piepsoiiasis state in which the patient
latei develops psoiiasis; in some patients a mix
of lesions (supeificial scales on the scalp and
eyebiows and polycyclic scaling patches on the
tiunk) suggests the use of the teim se|orr|ass .
Theie is ieputedly an incieased incidence in
Paikinson disease and facial paialysis. Also,
some neuioleptic diugs aie possibly a factoi,
but the disease is so common that this has not
been pioved. Emotional stiess is a putative fac-
toi in flaies. HIV-infected individuals have an
incieased incidence, and seveie intiactable SD
should be a clue to the existence of HIV disease
(see also Section 31).
FAIh0CNSIS
Ma|asse:a [ur[ur is said to play a iole in the patho-
genesis, and the iesponse to topical ketoconazole
and selenium sulfide is some indication that this
yeast may be pathogenic; also the fiequency of
SD in immunosuppiessed patients (HIV/AIDS,
caidiac tiansplants). SD-like lesions aie seen in
nutiitional deficiencies such as zinc deficiency
(as a iesult of IV alimentation) and expeiimental
niacin deficiency and in Paikinson disease (in-
cluding diug-induced). SD develops in expeii-
mental pyiidoxine deficiency in humans.
0uratoo oI Iesoos Giadual onset.
Seasooa| varatoos Some patients aie woise
in wintei in a diy, indooi enviionment. Sun-
light exposuie causes SD to flaie in a few
patients and piomotes impiovement of the
condition in otheis.
Sko Symptoms Piuiitus is vaiiable, often in-
cieased by peispiiation.
Sko Iesoos Oiange-ied oi giay-white skin,
often with gieasy" oi white diy scaling macules,
papules of vaiying size (5-20 mm), oi patches,
iathei shaiply maiginated (Fig. 2-26). Sticky
ciusts and fissuies aie common in the folds
behind the exteinal eai. On the scalp theie is
mostly maiked scaling (dandiuff "), diffuse
involvement of scalp. Scatteied, disciete on the
face and tiunk. Nummulai, polycyclic, and even
annulai on the tiunk .
0strbutoo aod Major Iypes oI Iesoos (8ased
oo Ioca|tatoo aod Ae) HuIry Areus v]
Heud Scalp, eyebiows, eyelashes (blephaiitis),
beaid (folliculai oiifices); ciadle cap: eiythema
and yellow-oiange scales and ciusts on the scalp
in infants .
Fuce The flush (butteifly") aieas, on foiehead
(coiona seboiihoica"), nasolabial folds, eye-
biows, glabella (Fig. 2-26). The eiythema of SD
is often oveilooked and thought to be the flush-
ing of iosacea. SD does not iespond to tieat-
ment of iosacea. Eais: ietioauiiculai, meatus.
Trun| Simulating lesions of pityiiasis io-
sea oi pityiiasis veisicoloi; yellowish-biown
patches ovei the steinum common.
Bvdy Fv|ds Axillae, gioins, anogenital aiea,
submammaiy aieas, umbilicus, and diapei aiea
in infants (Fig. 2-27)-piesents as a dif-
fuse, exudative, shaiply maiginated, biightly
eiythematous eiuption; eiosions and fissuies
common.
GenItu|Iu Often with yellow ciusts and pso-
iiasifoim lesions.
0IACN0SIS[0IFFkNIIAI 0IACN0SIS
Usually made on clinical ciiteiia.
ked Sca|y F|aques Cvmmvn Mild psoiiasis
vulgaiis (sometimes may be indistinguishable),
impetigo (iule out by smeais foi bacteiia), dei-
matophytosis (tinea capitis, tinea facialis, tinea
coipoiis), pityiiasis veisicoloi, inteitiiginous
candidiasis (KOH: iule out deimatophytes and
yeasts), subacute lupus eiythematosus (iule
out by biopsy), seboiiheic" papules in sec-
ondaiy syphilis (daikfield: iule out Treonema
a||Jum ).
Rure Langeihans cell histiocytosis (occuis in
infants, often associated with puipuia), acio-
deimatitis enteiopathica, zinc deficiency, pem-
phigus foliaceus, glucagonoma syndiome.
IA80kAI0k SI0IS
0ermatopatho|oy Focal paiakeiatosis,
with few neutiophils, modeiate acanthosis,
spongiosis (inteicellulai edema), nonspecific
FICk 2-25 Autoseosttatoo
dermatts ("d" reactoo): derma-
tophytd \e|c|e +ud |u||+e ou ||e
||ue| +ud ||e |+|e|+| |oo| o| + 2,e+|
o|d |er+|e. Bu||ou (|u||+rr+|o|,) ||ue+
ped| W+ p|eeu| +ud W+ +oc|+|ed
W||| de|r+|op|,||d |e+c||ou. ||edu|oue
W+ |.eu |o| 2 Wee|, p|u|||u +ud
.e|cu|+||ou |eo|.ed.
inflammation of the deimis. A chaiacteiistic fea-
tuie is neutiophils at the tips of the dilated fol-
liculai openings, which appeai as ciusts/scales.
C0kS AN0 Fk0CN0SIS
SD is veiy common, affecting the majoiity of
individuals at some time duiing life. The condi-
tion impioves in the summei and flaies in the
fall. Recuiiences and iemissions, especially on
the scalp, may be associated with alopecia in
seveie cases. Infantile and adolescent SD disap-
peais with age. Seboiiheic eiythiodeima may
occui. Se|orr|et ery|roJerma w| Jarr|ea
anJ [a|ure o |re (Lener Jsease) n n[ans
s assotaeJ w| a arey o[ mmunoJe[tenty
JsorJers nt|uJng Je[ete yeas oson:aon,
CJ Je[tenty, seere tom|neJ mmunoJe[-
tenty, |yogammag|o|u|nema, anJ |yerm-
munog|o|u|nema.
MANACMNI
Requiies initial theiapy followed by chionic
maintenance theiapy. Topical glucocoiticoid
piepaiations aie effective but can cause atiophy
and eiythema and telangiectasia, especially on
the face, oi initiation/exaceibation of peiioial
deimatitis oi iosacea. UV iadiation is benefi-
cial foi many individuals. Topical calcineuiin
inhibitois aie highly effective.
Iota| Iopca| Iherapy
Sca|p Adu|ts Effective ovei-the-countei
(OTC) s|amoos containing selenium sulfide,
zinc pyiithione, aie helpful. By piesciiption
(U.S.), 2% ketoconazole shampoo, used in-
itially to tieat and subsequently to contiol
the symptoms; lathei can be used on face and
chest duiing showei. Tai shampoos (OTC) aie
equally effective in many patients. Low-potency
g|utotortoJ solution, lotion, oi gels following
FICk 2-26 Seborrhec derma-
tts oI Iace: adu|t-type E|,||er+
+ud ,e||oWo|+ue c+||u +uuu|+| o|
||e |o|e|e+d, c|ee|, u+o|+||+| |o|d,
+ud c||u. 'c+|p +ud |e||o+u||cu|+| +|e+
We|e +|o |u.o|.ed.
a medicated shampoo (ketoconazole oi tai) foi
moie seveie cases. Pimeciolimus, 1% cieam, is
veiy beneficial.
1n]unts Foi ciadle cap, iemoval of ciusts
with waim olive oil compiesses, followed by
baby shampoo, 2% ketoconazole shampoo, and
application of 1-2.5% hydiocoitisone cieam,
2% ketoconazole cieam, 1% pimeciolimus
cieam.
Face aod Iruok Keotona:o|e s|amoo , 2%.
Glucocoiticoid cieam and lotions: initially 1 oi
2.5% hydiocoitisone cieam, 2% ketoconazole
cieam, 1% pimeciolimus cieam, 0.03 oi 0.1%
taciolimus ointment.
More oen g|utotortoJ |oons (e.g., clo-
betasol piopionate) may be used foi na|
contiol and aie used along with the medicated
shampoos.
ye|ds Gentle iemoval of the ciusts in the
moining with a cotton ball dipped in di-
luted baby shampoo. Apply 10% sodium sul-
facetamide in a suspension containing 0.2%
piednisolone and 0.12% phenylephiine (use
cautiously because it contains glucocoiticoids).
Sodium sulfacetamide ointment alone is also
effective, as is 2% ketoconazole cieam, 1%
pimeciolimus cieam, oi 0.03% taciolimus oint-
ment.
Iotertroous Areas Keotona:o|e, 2% ; if un-
contiolled with these tieatments, Castellani
paint foi deimatitis of the body folds is of-
ten veiy effective, but staining is a pioblem.
Pimeciolimus cieam, 1%; taciolimus ointment,
0.03 oi 0.1%.
Systemc Iherapy
In seveie cases, 13- ts ietinoic acid oially, 1 mg/
kg, is highly effective. Contiaception should be
used in females of child-beaiing age. In mildei
cases, itiaconazole 100 twice daily foi 2 weeks is
also effective.
Maoteoaoce Iherapy
Ketoconazole 2% shampoo; tai shampoos may
be equally effective; ketoconazole cieam. If
these do not woik, then the old standaid," 3%
sulfui piecipitate and 2% salicylic acid in an
oil-in-watei base is effective; this must be piop-
eily compounded. Also, 1-2.5% hydiocoitisone
cieam daily will woik, but patients should be
monitoied foi signs of atiophy. 1% pimecioli-
mus cieam and 0.03% taciolimus ointment aie
safe and effective.
FICk 2-2T Seborrhec dermatts: oIaot|e-type E|,||er+ +ud o|+ue c+|e +ud c|u||u |u ||e
d|+pe| |e|ou o| +u |u|+u|. I|| | d||||cu|| |o d|||uu|| |u ||e d|+pe| |e|ou ||or po||+| +ud Cc!!c |+ |o |e
|u|ed ou| |, K0n.
A corrou p|u||||c de|r+|||| ||+| occu| epe
c|+||, |u o|de| pe|ou, |u ||e W|u|e| |u |erpe|+|e
c||r+|e-|e|+|ed |o ||e |oW |ur|d||, o| |e+|ed
|oue.
I|e ||e o| p|ed||ec||ou +|e ||e |e (||. 223),
+|r, +ud |+ud |u| +|o ||e ||uu|.
||,, 'c|+c|ed, upe|||c|+||, ||u|ed ||u W|||
|||| c+||u.
I|e |uce+u| p|u|||u c+u |e+d |o ||c|eu|||c+||ou,
W||c| c+u e.eu pe||| W|eu ||e eu.||oureu|+|
coud|||ou |+.e |eeu co||ec|ed.
I|e d|o|de| |eu|| ||or |oo ||equeu| |+|||u
|u |o| o+p, |+|| o| |oWe| +ud/o| |u o|de|
pe|ou ||.|u |u |oor W||| + ||| eu.||oureu|+|
|erpe|+|u|e +ud |oW |e|+||.e |ur|d||,.
\+u+ereu|. +.o|d|u o.e||+|||u W||| o+p,
epec|+||, |u| |+||, +ud |uc|e+|u ||e +r||eu|
|ur|d||, |o >50, |, u|u |oor |ur|d|||e|,
+|o u|u |ep|d W+|e| |+|| cou|+|u|u |+|| o||
|o| |,d|+||ou, |o||oWed |, |rred|+|e |||e|+| +p
p||c+||ou o| ero|||eu| o|u|reu|, uc| + |,d|+|ed
pe||o|+|ur. || ||u | |u||+red, ue red|urpo
|euc, |ucoco|||co|d o|u|reu|, +pp||ed |W|ce d+||,
uu||| ||e ec/er+|ou corpoueu| |+ |eo|.ed.
',,. Ec/er+ :c-|- (||euc| :c-|-
'r+||ed W||| c|+c|, uc| + |u o|d c||u+ +ud
ce|+r|c |||e).
ASIAI0IIC 0kMAIIIIS |C|9 . o92.39
|C|0 . |!0.9
FICk 2-28 Asteatotc dermatts |u ||| o5,e+|o|d r+u |e|ou |+.e co+|eced |o |u.o|.e ||e eu|||e ||u
o| ||e |oWe| |e. I|e e|,||er+|ou, c+||u |e|ou +|e e\||ere|, p|u||||c.
53
CIASSIFICAII0N
Psoriasis vulgaris
Acute guttate
Chionic stable plaque
Palmoplantai
Inveise
S E C I | 0 N 5
FS0kIASIS
|o||+| +||ec| .5-2 o| ||e popu|+||ou |u
We|e|u couu|||e. wo||d W|de occu||euce.
A c||ou|c d|o|de| W||| po|,eu|c p|ed|po|||ou
+ud |||e||u eu.||oureu|+| |+c|o| uc| +
|+c|e||+| |u|ec||ou, ||+ur+, o| d|u.
'e.e|+| c||u|c+| e\p|e|ou. I,p|c+| |e|ou +|e
c||ou|c, |ecu|||u, c+|, p+pu|e +ud p|+que.
|u|u|+| e|up||ou +ud e|,|||ode|r+ occu|.
C||u|c+| p|eeu|+||ou .+||e +rou |ud|.|du+|,
||or ||oe W||| ou|, + |eW |oc+||/ed p|+que |o
||oe W||| eue|+||/ed ||u |u.o|.ereu|.
|o||+||c +||||||| occu| |u 0-25 o| ||e
p+||eu|.
I|e p+||oeue| | de|e|r|ued |, + po|,eu|c
||+|| W||| +u ouo|u I ce||-d||.eu +u|o|e+c||.e
|rruue |epoue.
Psoriatic erythroderma
Pustular psoriasis
Pustulai psoiiasis of von Zumbusch
Palmoplantai pustulosis
Aciodeimatitis continua
FS0kIASIS vICAkIS |C|9 . o9o.

|C|0 . | +0.0
FI0MI0I0C
Ae oI 0oset Ear|y : Peak incidence occuis at
22.5 yeais of age (in childien, the mean age of
onset is 8 yeais). Lae : Piesents about age 55.
Ear|y onse piedicts a moie seveie and long-last-
ing disease, and theie is usually a positive family
histoiy of psoiiasis.
Iocdeoce Psoiiasis affects 1.5-2% of the pop-
ulation in westein countiies. In the United
States, theie aie 3 to 5 million peisons with
psoiiasis. Most have localized psoiiasis, but ap-
pioximately 300,000 peisons have geneialized
psoiiasis.
Sex Equal incidence in males and females.
kace Low incidence in West Afiicans, Japa-
nese, and Inuits; veiy low incidence oi absence
in Noith and South Ameiican Indians.
heredty Polygenic tiait. When one pai-
ent has psoiiasis, 8% of offspiing develop
psoiiasis; when both paients have psoiiasis,
41% of childien develop psoiiasis. HLA types
most fiequently associated with psoiiasis aie
HLA- B13, -B17,-Bw57, and, most impoitantly,
HLA-Cw6, which piesents antigens to CD8-
T cells and is thus a candidate foi functional
involvement.
Irer Factors P|ysta| rauma (Koebnei
phenomenon) is a majoi factoi in eliciting
lesions; iubbing and sciatching stimulate the
psoiiatic piolifeiative piocess. In[etons : acute
stieptococcal infection piecipitates guttate pso-
iiasis. Sress : a factoi in flaies of psoiiasis is
said to be as high as 40% in adults and highei
in childien. Drugs : systemic glucocoiticoids,
oial lithium, antimalaiial diugs, inteifeion,
and -adieneigic blockeis can cause flaies in
existing psoiiasis and cause a psoiiasifoim
diug eiuption. |to|o| ngeson is a putative
tiiggei factoi.
FAIh0CNSIS
The most obvious abnoimalities in psoiiasis
aie (1) an alteiation of the cell kinetics of
keiatinocytes with a shoitening of the cell
cycle fiom 311 to 36 h, iesulting in 28 times
the noimal pioduction of epideimal cells, and
(2) CD8- T cells, which aie the oveiwhelming
T cell population in lesions. The epideimis
and deimis ieact as an integiated system: the
desciibed changes in the geiminative layei of
the epideimis and inflammatoiy changes in the
deimis, which tiiggei the epideimal changes.
Psoiiasis is a T cell-diiven disease. Theie aie
many CD8 - T cells piesent in psoiiatic lesions
suiiounding the uppei deimal blood vessels,
and the cytokine spectium is that of a T
H
1
iesponse. Maintenance of psoiiatic lesions is
consideied an ongoing autoieactive immune
iesponse.
Theie aie two majoi types:
1. Erue, n[|ammaory ye with multiple
small (guttate oi nummulai) lesions and a
gieatei tendency towaid spontaneous ies-
olution (Figs. 3-1 and 3-2); ielatively iaie
(<2.0% of all psoiiasis); similai to an ex-
anthem: a showei of lesions appeais iathei
iapidly and in young adults, often but not
always following stieptococcal phaiyngitis.
2. C|ront sa||e (|aque) sorass (Figs. 3-3;
3-4): Majoiity of patients, with chionic
indolent lesions piesent foi months and
yeais, changing only slowly.
Sko Symptoms Piuiitus is ieasonably com-
mon, especially in scalp and anogenital pso-
iiasis.
Sko Iesoos The classic lesion of psoiiasis
is a shaiply maiginated eiythematous papule
with a silveiy-white scale (Fig. 3-1). Scales aie
lamellai, loose, and easily iemoved by sciatching.
Removal of scale iesults in the appeaiance of
minute blood dioplets ( us: sgn ). Papules
giow to shaiply maiginated plaques with
lamellai scaling (Fig. 3-3) that coalesce to foim
polycyclic oi seipiginous patteins (Fig. 3-4).
May occui anywheie on the body but theie aie
classic piedilection sites (see Image 3-1).
Acute Guttute Type Salmon-pink papules
(guttate: Latin gua , diop``), 2.0 mm to 1.0
cm with oi without scales (Figs. 3-1; 3-2) scales
may not be visible but become appaient upon
sciaping. Scatteied disciete lesions, like a iash;
geneially concentiated on the tiunk (Fig. 3-2),
less on the face and scalp, and usually spaiing
palms and soles. Guttate lesions may iesolve
spontaneously within a few weeks but usually
become iecuiient and may evolve into chionic,
stable psoiiasis.
ChrvnIc Stuh|e Type Shaiply maiginated,
dull-ied plaques with loosely adheient, lamel-
lai, silveiy-white scales (Fig. 3-3). Plaques
coalesce to foim polycyclic, geogiaphic lesions
(Fig. 3-4) and may paitially iegiess, iesulting
in annulai, seipiginous, and aicifoim patteins.
Lamellai scaling can easily be iemoved, oi,
when the lesion is extiemely chionic, it ad-
heies tightly to the undeilying inflammatoiy
and infiltiated skin, iesulting in hypeikeia-
tosis that looks like the shell of an oystei
(Fig. 3-5).
0strbutoo aod Fred|ectoo Stes
Acute Guttute Disseminated, geneialized,
mainly tiunk (Fig. 3-2).
ChrvnIc Stuh|e Single lesion oi lesions local-
ized to one oi moie piedilection sites: elbows,
knees, sacialgluteal iegion, scalp, palm/soles
(Image 3-1). Sometimes only iegional involve-
ment (scalp), often geneialized.
Puttern Bilateial, often symmetiic (piedilec-
tion sites); often spaies exposed aieas.
Speca| Stes
Fa|ms aod So|es May be the only aieas in-
volved. Theie is massive silveiy white oi yel-
lowish hypeikeiatosis and scaling, which in
contiast to lesions on the tiunk, is not easily
iemoved (Fig. 3-6). Desquamation of hypei-
keiatosis will, howevei, ieveal an inflammatoiy
plaque at the base that is always shaiply demai-
cated (Fig. 3-7). Theie may be ciacking and
painful fissuies and bleeding.
Sca|p Plaques, shaiply maiginated, with thick
adheient scales (Fig. 3-8). Scatteied disciete
oi diffuse involvement of entiie scalp. Often
veiy piuiitic. Noe. psoiiasis of the scalp does
not lead to haii loss, even aftei yeais of thick
plaque-type involvement. Scalp psoiiasis may
be pait of geneialized psoiiasis oi coexist with
isolated plaques, oi the scalp may be only site
involved.
Face Uncommonly involved, and when in-
volved, usually associated with a iefiactoiy type
of psoiiasis (Fig. 3-9).
SCII0N 3 |'0k|A'|' 55
FICk 3-1 Fsorass vu|ars |||r+|, |e|ou +|e We||de||ued, |edd|| o| +|roup|u| p+pu|e, d|op|||e,
W||| + |ooe|, +d|e|eu| ||.e|,W|||e |+re||+| c+|e.
FICk 3-2 Fsorass vu|ars: buttocks (uttate type) ||c|e|e, e|,||er+|ou, c+||u, r+|| p+pu|e
||+| |eud |o co+|ece, +ppe+||u +||e| + |oup A ||ep|ococc+| p|+|,u|||. I|e|e W+ + |+r||, |||o|, o| po||+|.
Chrooc Fsorass oI the Feraoa| aod Ceo-
ta| keoos aod oI the 8ody Fo|ds-Ioverse
Fsorass Due to the waim and moist envi-
ionment in these iegions psoiiatic plaques aie
usually not scaly but aie maceiated, often biight
ied and fissuied (Figs. 3-10, 35-7, 35-8). The
shaip demaication peimits distinction fiom
inteitiigo, candidiasis, contact deimatitis, tinea
ciuiis.
Na|s Fingeinails and toenails fiequently (25%)
involved, especially with concomitant aithiitis
(Fig. 3-11A). Nail changes include pitting, subun-
gual hypeikeiatosis, onycholysis, and yellowish-
biown spots undei the nail plate-the o| so
(pathognomonic) (See Figs. 33-8, 33-9).
0ermatopatho|oy
Maiked oveiall thickening of the epideimis
(acanthosis) and thinning of epideimis ovei
elongated deimal papillae
Incieased mitosis of keiatinocytes, fibioblasts,
and endothelial cells
Paiakeiatotic hypeikeiatosis (nuclei ietained
in the stiatum coineum)
Inflammatoiy cells in the deimis (lymphocytes
and monocytes) and in the epideimis (lym-
phocytes and polymoiphonucleai cells),
foiming micioabscesses of Munio in the
stiatum coineum.
Sero|oy Incieased antistieptolysin titei in
acute guttate psoiiasis with antecedent stiepto-
coccal infection. Sudden onset of psoiiasis may
be associated with HIV infection. Deteimina-
tion of HIV seiostatus is indicated in at-iisk
individuals. Seium uiic acid is incieased in 50%
of patients, usually coiielated with the extent of
the disease; theie is an incieased iisk of gouty
aithiitis. The levels of uiic acid deciease as
theiapy is effective.
Cu|ture Thioat cultuie foi gioup A -hemo-
lytic stieptococcus infection.
Diagnosis is made on clinical giounds.
Acute Cuttate Fsorass Any maculopapulai
diug eiuption, secondaiy syphilis, pityiiasis
iosea.
IMAC 3-1 Fred|ectoo stes oI psorass.
SCII0N 3 |'0k|A'|' 5T
Sma|| Sca|o F|aques Se|orr|et Jermas -
may be indistinguishable in sites involved and
moiphology; sometimes teimed se|orr|ass.
Lt|en sm|ex t|rontus -may complicate pso-
iiasis as a iesult of piuiitus. Psoras[orm Jrug
eruons -especially beta blockeis, gold, and
methyldopa. Tnea torors -KOH examina-
tion is mandatoiy, paiticulaily in single lesions.
Mytoss [ungoJes -scaling plaques can be an
initial stage of mycosis fungoides. Biopsy.
Iare Ceoraphc F|aques Tinea coipoiis, my-
cosis fungoides.
Sca|p Fsorass Seboiiheic deimatitis, tinea
capitis.
Ioverse Fsorass Tinea, candidiasis, inteitiigo,
extiamammaiy Paget disease. C|utagonoma
synJrome -an impoitant diffeiential because
this is a seiious disease; the lesions look like in-
veise psoiiasis. Langeihans cell histiocytosis (see
page 516), Hailey-Hailey disease (see page 105).
Na|s Onychomycosis. KOH is mandatoiy.
C0kS AN0 Fk0CN0SIS
Acute guttate psoiiasis appeais iapidly, a gen-
eialized iash.`` Sometimes this type of psoiia-
sis disappeais spontaneously in a few weeks
without any tieatment. Moie often, guttate
psoiiasis evolves into chionic plaque psoiiasis.
This is stable and may undeigo iemission aftei
months oi yeais, iecui, and be a lifelong com-
panion. Chionic geneialized psoiiasis is one
of the miseiies that beset mankind," causing
shame and embaiiassment and a compiomised
lifestyle. The heaitbieak of psoiiasis`` is no
joke. As the wiitei John Updike (who himself
has psoiiasis) so poignantly said about being
a peison with psoiiasis, I am silveiy, scaly.
Puddles of flakes foim wheievei I iest my flesh.
Lusty, though we aie loathsome to love. Keen-
sighted, though we hate to look upon ouiselves.
The name of the disease, spiiitually speaking, is
Humiliation.``
FICk 3-3 Fsorass vu|ars: e|bow C||ou|c |+||e p|+que po||+| ou ||e e||oW. |u ||| |oc+||ou c+|e
c+u e|||e| +ccuru|+|e |o o,|e| |e|||||e |,pe||e|+|o|, o| +|e |ed |u |+|e |ee| |e.e+||u + |ee|,|ed |+e.
I|| p|+que |+ +||eu ||or ||e co+|eceuce o| r+||e|, p+pu|+| |e|ou.
FICk 3-4 Fsorass vu|ars: chrooc stab|e type \u|||p|e |+|e c+||u p|+que ou ||e ||uu|, |u||oc|,
+ud |e. |e|ou +|e |ouud o| po|,c,c||c +ud cou||ueu| |o|r|u eo|+p||c p+||e|u. A|||ou| ||| | ||e c|+|c+|
r+u||e|+||ou o| c||ou|c |+||e p|+que po||+|, ||e e|up||ou | |||| ouo|u, + e.|deuced |, ||e r+|| u||+|e
|e|ou |u ||e |ur|+| +ud |oWe| |+c| +|e+. I|| p+||eu| W+ c|e+|ed |, +c|||e||u/|u\A cor||u+||ou ||e+|reu|
W||||u + Wee|.
FICk 3-5 Chrooc p|aque|ke psorass o a 30-year-o|d mao oI Arabao desceot I|e |,pe|
|e|+|o||c |o|u, p|+que co.e||u ||e uude||,|u |u||+rr+|o|, ||ue o|cu|e +|| e|,||er+, +ud due |o ||e |||
re|+u|u cou|eu|, ||e c+|e +ppe+| d|||,|+,. I|e |||eu|+|, |+|p|, de||ued ||oWu +|e+ +|e po||u||+rr+|o|,
|,pe|p|reu|+||ou |u p|e.|ou po||+||c p|+que.
SCII0N 3 |'0k|A'|' 59
FICk 3-T Fsorass, pa|mar ovo|vemeot
|o||+||c p|+que ou ||e p+|r o| + +5,e+|o|d Wor+u,
W||c| ou |||| ||| ue| c||ou|c |||||+||.e de|
r+||||. I|e p+||u |u ||e ceu|e| o| ||e p+|r +ud
||e |+|p de||ue+||ou o| ||e |e|ou ue| po||+|,
W||c| W+ +|o p|eeu| ou o||e| p+|| o| ||e |od,.
FICk 3-6 Fsorass vu|ars: so|es E|,||e
r+|ou p|+que W||| |||c|, ,e||oW||, |+re||+| c+|e
+ud dequ+r+||ou ou ||e o| p|eu|e +|||u ou ||e
p|+u|+| |ee|. |o|e |+|p der+|c+||ou o| ||e |u||+r
r+|o|, |e|ou ou ||e +|c| o| ||e |oo|. '|r||+| |e|ou
We|e p|eeu| ou ||e p+|r.
FICk 3-8 Fsorass oI the sca|p I|e|e | r+|.e corp+c||ou o| |o|u, r+|e||+| ou ||e eu|||e c+|p.
|equ+r+||ou |+ occu||ed ou ||e |o|e|e+d, W||c| | +|o |u.o|.ed W||| po||+|.
FICk 3-9 Fsorass, Iaca| ovo|vemeot C|+|c po||+||c p|+que ou ||e |o|e|e+d o| + 2,e+|o|d r+|e
W|o +|o |+d r+|.e c+|p |u.o|.ereu|.
FICk 3-10 Fsorass vu|ars: overse pattero I|| p|c|u|e |oW ||e d|||e|euce |e|Weeu p|+que
po||+| ou uou|||c||ou+| |e|ou, uc| + ||e +c|ur, +ud |u|e|||||uou |e|ou, uc| + ||e |u|e+| |o|d. ne|e ||e
eu.||oureu| | ro|| +ud W+|r, W||c| |+c||||+|e |edd|u o| ||e c+|, r+|e||+|. |u ||e |u|e||u|e+| |o|d ||e |e|ou
| r+ce|+|ed +ud |+,|| ou +u e|,||er+|ou |+e.
SCII0N 3 |'0k|A'|' 61
FICk 3-11 . Fsorass oI the Ioeroa|s ||| |+.e p|o|eed |o e||ou,\| (|o|e |u ||e u+|| p|+|e)
+ud ||e|e | ||+u.e|e +ud |ou||ud|u+| ||d|u. I|| p+||eu| +|o |+ pe||ou,c||+| po||+| +ud po||+||c +|||||||.
Fsoratc arthrts, |+|e |+e |e+d|u |o +||||||| ru|||+u. |e||uc||ou o| ||e |u|e|p|+|+ue+| jo|u| |+ |e+d |o
||e p|euoreuou o| |e|ecope ||ue| +ud |o + e.e|e ru|||+||ou o| ||e |+ud W||| cou|de|+||e |uuc||ou+| |rp+||
reu|. |o| |u|||e| |r+e o| u+|| |u.o|.ereu| ee 'ec||ou !! +ud .

C|+|+c|e||/ed |, pu|u|e, uo| p+pu|e, +|||u ou uo|r+| o| |u||+red, e|,||er+|ou ||u. IWo |,pe.
FSIIAk FS0kIASIS |C|9 . o9o.
A c||ou|c, |e|+p|u e|up||ou ||r||ed |o ||e p+|r
+ud o|e.
|ure|ou .e|, |,p|c+| |e|||e, ,e||oW, deepe+|ed
pu|u|e ||+| e.o|.e |u|o du|,|ed c|u|.
Cou|de|ed |, ore + + |oc+||/ed |o|r o| pu
|u|+| po||+| (|+||e||,pe) +ud |, o||e| + +
ep+|+|e eu|||,.
FAIM0FIANIAk FSII0SIS |C|9 . o9o.

|C|0 . | +0.!
FI0MI0I0C
Iocdeoce Low as compaied to psoiiasis vul-
gaiis.
Ae oI 0oset 50 to 60 yeais. Moie common in
females (4:1).
Symptoms Stinging, buining itching. Eiup-
tions come and go, in waves.
Sko Iesoos Pustules in stages of evolution,
2-5 mm, deep-seated, yellow, develop into
dusky-ied macules and ciusts; piesent in aieas
of eiythema and scaling oi noimal skin (Fig.
3-12). Limited to palms and soles, may be only
a localized patch on the sole oi hand, oi involve
both hands and feet with a piedilection of
thenai and hypothenai, flexoi aspects of fingeis,
heels, and insteps; acial poitions of the fingeis
and toes usually spaied.
Conditions confined to palms and soles. Epi-
deimal deimatophytosis (tinea manus, tinea
pedis), dyshidiotic eczematous deimatitis, iiii-
tant oi alleigic contact deimatitis, heipes simplex
viius (HSV) infection (if localized to one site).
k0h Freparatoos To exclude deimatophytosis.
8actera| or vra| Cu|ture To exclude Sa|y|o-
tottus aureus infection and HSV infection.
0ermatopatho|oy Edema and exocytosis of
mononucleai cells that appeai fiist to foim a ves-
icle, and latei myiiads of neutiophils, which foim
a uniloculai spongifoim pustule. Acanthosis.
C0kS AN0 Fk0CN0SIS
Peisistent foi yeais and chaiacteiized by un-
explained iemissions and exaceibations; iaiely
psoiiasis vulgaiis may develop elsewheie.
SCII0N 3 |'0k|A'|' 63
FICk 3-12 Fa|mar pustu|oss C|e+r,,e||oW pu|u|e ||+| +|e p+|||+||, cou||ueu| ou ||e p+|r o| + 23
,e+|o|d |er+|e. |u|u|e +|e |e|||e +ud p|u||||c, +ud W|eu ||e, e| |+|e|, |ecore p+|u|u|. A| ||e ||re o| |||
e|up||ou ||e|e W+ uo o||e| e.|deuce o| po||+| +u,W|e|e e|e ou ||e |od,, |u| 2 ,e+| |+|e| ||e p+||eu| de.e|
oped c||ou|c |+||e p|+que po||+| ou ||e ||uu|.
FI0MI0I0C
Raie, occuis in adults, iaiely in childien.
FAIh0CNSIS
Unknown. The fevei and leukocytosis iesult
fiom the ielease of cytokines and chemokines
fiom the skin into the ciiculation. Theie aie
no known piecipitating factois, and the patient
may oi may not have had a stable plaque-type
psoiiasis in the past.
0oset oI Iesoos The constellation of fieiy-ied
eiythema followed by foimation of pustules oc-
cuis ovei a peiiod of less than 1 day. Waves of
pustules may follow each othei; as one set diies,
anothei appeais.
Sko Symptoms Maiked buining, tendeiness.
Coosttutooa| Symptoms Headache, chills, fe-
veiishness, maiked fatigue, seveie malaise.
Appearaoce oI Fateot Fiightened, toxic.``
vta| Sos Fast pulse, iapid bieathing, fevei
that may be high.
Sko Iesoos Theie is a sequence of buining,
diffuse eiythema followed by the appeaiance
of clusteis of tiny, nonfolliculai, and veiy
supeificial yellowish to whitish pustules that
usually become confluent, foiming ciicinate
lesions and lakes`` of pus (Figs. 3-13 and
3-14), Nikolsky phenomenon is positive. Re-
moval of the tops of pustules yields supeificial,
oozing eiosions. Ciusting. Once ciusts aie
shed, new ciops of pustules may appeai in the
same site. Pustules aie steiile. The eiuption is
geneialized.
A |||e|||e+|eu|u red|c+| p|o||er W||| +u +||up|
oue|.
'||u |u.o|.ereu| |+|| W||| + |u|u|u ||e|,|ed
e|,||er+ ||+| p|e+d |u |ou| W||| p|upo|u|
|e|||e pu|u|e +ppe+||u |u c|u|e|.
|e.e|, eue|+||/ed We+|ue, e.e|e r+|+|e, +ud
|eu|oc,|o| +|e |e+|u|e |u +|ro| e.e|, p+||eu|.
CNkAIII0 ACI FSIIAk FS0kIASIS (v0N IM8SCh) |C|9 . o9o.

|C|0 . |+0.
har aod Na|s Nails become thickened, and
theie is onycholysis; subungual lakes`` of pus
lead to shedding of nails; haii loss of the telogen
defluvium type (see Section 33) may develop in
2 oi 3 months.
Mucous Membraoes Ciicinate desquamation
of the tongue. This is the only foim of psoiiasis
that involves mucous membianes.
Wdespread rythema wth Fustu|es The
abiupt onset and the typical evolution of eiy-
thema followed by pustulation aie highly chai-
acteiistic. Neveitheless, blood cultuies should
always be obtained because of possible su-
peiinfection and bacteiemia, especially with
S. aureus . Geneialized HSV infection has um-
bilicated pustules, and the Tzanck tests and viial
cultuies establish the diagnosis. Geneialized
pustulai diug eiuptions e.g., aftei fuiosemide,
amoxicillin/clavulanic acid, and othei diugs
(acute geneialized exanthematous pustulosis,
see Section 22)] may be clinically indistinguish-
able, but patients aie less toxic. Psoiiasis cum
pustulatione (see below) has lesions of classic
psoiiasis with pustulation.
0ermatopatho|oy Laige spongifoim pustules
iesulting fiom the migiation of neutiophils to
the uppei stiatum malpighi, wheie they aggie-
gate within the inteistices between the degenei-
ated and thinned keiatinocytes.
8actera| Cu|ture oI Issue Pustules aie steiile.
Rule out S. aureus infection.
hemato|oc Polymoiphonucleai leukocytosis-
white blood cell count as high as 20,000/L.
SCII0N 3 |'0k|A'|' 65
FICk 3-13 Ceoera|ted acute pustu|ar psorass (voo Iumbusch) \u|||p|e pu|u|e ou ||||, e|,||er+
|ou ||u. C|oeup o| |e|ou |oW ||+| ||e, +|e .e|, upe|||c|+|, c|e+r, W|||e, +ud co+|ece, |o|r|u |+|e o| pu.
FICk 3-14 Ceoera|ted acute pustu|ar psorass (voo Iumbusch) I|| |er+|e p+||eu| W+ |o\|c +ud
|+d |e.e| +ud pe||p|e|+| |eu|oc,|o|. I|e eu|||e |od, W+ co.e|ed W||| |oWe| o| c|e+r,W|||e co+|ec|u
pu|u|e ou + ||e|,|ed |+e. '|uce ||ee pu|u|e +|e .e|, upe|||c|+|, ||e, c+u |e |||e|+||, W|ped o||, W||c| |eu||
|u |ed oo/|u e|o|ou.
C0kS AN0 Fk0CN0SIS
These patients aie often biought to the emeig-
ency iooms of hospitals, and theie is the question
of oveiwhelming bacteiemia until a deima-
tologist is consulted and the blood cultuies aie
shown to be negative. Relapses and iemissions
may occui ovei a peiiod of yeais. In the eldeily
piognosis is guaided if not tieated. May follow,
evolve into, oi be followed by psoiiasis vulgaiis.
SFCIAI IFS
Impeto herpetIorms This is von Zumbusch
pustulai psoiiasis in a piegnant woman with
hypocalcemia, leading to tetanic seizuies.
Aoou|ar Iype This type of pustulai psoiia-
sis occuis in childien, with less consitutional
symptoms.
Fsorass cum Fustu|atooe (psoiiasis with
postulation) This is maltieated stable plaque
psoiiasis wheie pustulation of psoiiatic lesions
and the suiiounding noimal skin occuis ,
usually aftei iiiitating topical tieatment (e.g.,
anthialin) oi systemic glucocoiticoids; may
become geneialized but patients aie usually
nontoxic.
Acrodermatts Cootoua oI ha||opeau This is a
chionic iecuiient pustulation of nail folds, nail
bed, and distal fingeis leading to loss of nails. It
can occui alone oi in association with pustulai
psoiiasis of von Zumbusch.
FICk 3-15 Acrodermatts cootoua o| n+||ope+u W||| +c|+| pu|u|e |o|r+||ou, u|uuu+| |+|e o| pu,
+ud de||uc||ou o| u+|| p|+|e. I|| r+, |e+d |o pe|r+ueu| |o o| u+|| +ud c+|||u.
SCII0N 3 |'0k|A'|' 6T
IFS
1. Distal"-seionegative, without subcutane-
ous nodules, and involving, asymmetiically, a
few distal inteiphalangeal joints of the hands
and feet: an asymmetiic oligoaithiitis.
2. Enthesitis-inflammation of ligament insei-
tion into bone.
3. Multilating psoiiatic aithiitis with bone eio-
sion and ultimately leading to osteolysis oi
ankylosis.
4. Axial"-especially involving the sacioil-
iac, hip, and ceivical aieas with ankylosing
spondylitis.
SkIN SMFI0MS AN0 SICNS
Swelling, iedness, tendeiness of involved joints
oi site of enthesitis (e.g., inseition of Achil-
les tendon in calcaneus). Dactylitis-sausage
fingeis. May oi may not be associated with pso-
iiasis elsewheie. Often psoiiatic involvement
of fingeitips and peiiungual skin. Massive
nail involvement by psoiiasis is fiequent (Fig.
3-11A).
Aithiitis may lead to aithiitis mutilans: destiuc-
tion of inteiphalangeal joints iesults in telescope
fingeis with mutilation of hand and considei-
able functional impaiiment (Fig. 3-11B).
I|| | + coud|||ou |u W||c| po||+| |u.o|.e
p|+c||c+||, ||e eu|||e ||u +ud |e+d |o cou|||u
||ou+| ,rp|or. || | + e||ou coud|||ou +ud
| d|cued |u 'ec||ou 3.
FS0kIAIIC kIhk00kMA |C|9 . o9o.

|C|0 . |+0
|o||+||c +||||||| | |uc|uded +rou ||e e
|oue+||.e poud,|o+||||op+|||e, W||c| |uc|ude
+u|,|o|u poud,||||, eu|e|op+|||c +|||||||, +ud
|e+c||.e +|||||||.
A,rre|||c pe||p|e|+| jo|u| |u.o|.ereu| o| uppe|
e\||er|||e +ud epec|+||, r+||e| jo|u|.
A\|+| |o|r |u.o|.e .e||e||+| co|uru, +c|o||||||.
Aoc|+|ed W||| \nC c|+ | +u||eu, W|||e ||eu
r+|o|d +||||||| | +oc|+|ed W||| \nC c|+ ||
+u||eu.
|uc|deuce | 5-3. k+|e |e|o|e +e 20.
!c, |- --| ( !9 | !.!c|) +|||
c, .||- c | -c:| | c |c|,
||,
FS0kIAIIC AkIhkIIIS |C|9 . o9o.0

|C|0 . |+0.5
MANACMNI 0F FS0kIASIS
Factors IoI|ueoco Se|ectoo oI Ireatmeot
1. Age: childhood, adolescence, young adult-
hood, middle age, 60 yeais.
2. Type of psoiiasis: guttate, plaque, palmai
and palmopustulai, geneialized pustulai
psoiiasis, eiythiodeimic psoiiasis.
3. Site and extent of involvement: |ota|:eJ to
palms and soles, scalp, anogenital aiea, scat-
teied plaques but <5% involvement; genera|-
:eJ and >30% involvement.
4. Pievious tieatment: ionizing iadiation, sys-
temic glucocoiticoids, photochemotheiapy
(PUVA), cyclospoiine (CS), methotiexate
(MTX).
5. Associated medical disoideis (e.g., HIV
disease).
Ideally all patients with suspected psoiiasis
should be seen at least once by a deimatologist
to establish the diagnosis and select the best
available tieatment iegimen. Localized pso-
iiasis (coveiing <5% of the body suiface) can
be managed by the piimaiy caie physician if a
piopei iegimen is selected. Psoiiasis of all othei
types, especially geneialized psoiiasis, should be
managed by a deimatologist who has access to
and knowledge of all theiapies, as combinations
and iotational" theiapy shifting fiom ultiavio-
let to PUVA to MTX oi the biologicals."
In the following pages management of pso-
iiasis is discussed in the context of types of
psoiiasis, sites, and extent of involvement.
I0CAIII0 FS0kIASIS
This consists of a limited numbei of chionic
stable psoiiasis plaques (see Fig. 3-3) on the
piedilection sites oi elsewheie. Heie, fiist-line
theiapies aie topical tieatments.
Iruok aod xtremtes
Topical fluoiinated glucocoiticoids (beta-
methasone valeiate, fluocinolone aceto-
nide, betamethasone piopionate, clobetasol
piopionate) in ointment base applied aftei
the scales aie iemoved by soaking in wa-
tei. Onmen applied to wet skin, coveied
with plastic wiap, left on oveinight. Gluco-
coiticoid-impiegnated tape useful foi small
plaques.
Hydiocolloid diessing, left on foi 24-48 h, is
effective and pievents sciatching. Duiing the
day, classes I and II fluoiinated glucocoiti-
coid cieams can be used without occlusion.
Patients develop toleiance (tachyphylaxis)
aftei long peiiods. Caea : Piolonged appli-
cation of the fluoiinated glucocoiticoids leads
to atiophy of the skin, peimanent stiiae,
and unsightly telangiectasia. Clobetasol-17-
piopionate is stiongei and active even with-
out occlusion. To avoid systemic effects of
this class I glucocoiticoid: maximum of 50 g
ointment pei week.
Foi small plaques ( 4 cm), tiiamcinolone ac-
etonide aqueous suspension 3 mg/mL diluted
with noimal saline is injected into the lesion.
Must be nraJerma|. Varnng : Hypopigmen-
tation at the injection site can iesult; this is
moie appaient in biown and black skin but is
ieveisible.
Topical anthialin piepaiations aie excellent
when used piopeily. Can be veiy iiiitant;
theiefoie follow diiections on the package
inseit with attention to details.
Vitamin D analogues (calcipotiiene, 0.005%,
ointment and cieam) aie good nonsteioi-
dal antipsoiiatic topical agents and aie not
associated with cutaneous atiophy. Not as po-
tent as class I glucocoiticoids (e.g., clobetasol
piopionate) but can be combined with them.
Calcipotiiene should not be applied to moie
than 40% of the body suiface and not moie
than 100 g pei week to avoid hypeicalcemia.
Topical taciolimus, 0.1%, has efficacy similai
to that of vitamin D analogues.
Topical pimeciolimus, 1%, is effective in in-
veise psoiiasis and seboiiheic deimatitis-like
psoiiasis of the face and eai canals.
Tazaiotene (a topical ietinoid, 0.05 and 0.1%
gel) has similai efficacy but can best be com-
bined with class II (medium stiength) topical
glucocoiticoids, as it can cause iiiitation.
When theie is >10% (palm of the hand 1%)
involvement with psoiiatic plaques, it is pief-
eiable to combine these topical tieatments
with 311-nm UVB phototheiapy oi PUVA
photochemotheiapy.
Sca|p MI|d Supeificial scaling and lacking
thick plaques: Tai oi ketoconazole shampoos
[o||oweJ |y betamethasone valeiate, 1% lotion;
if iefiactoiy, clobetasol piopionate, 0.05% scalp
application.
Severe Thick, adheient plaques (Fig. 3-8):
Removal of scales fiom plaques befoie active
tieatment by 10% salicylic acid in mineial oil,
coveied with a plastic cap and left on oveinight.
Aftei shedding of scales, fluocinolone cieam oi
lotion with the scalp coveied with plastic oi a
showei cap, left on oveinight oi foi 6 h. When
the thickness of the plaques is ieduced, clobeta-
sol piopionate, 0.05% lotion, oi calcipotiiene
lotion can be used foi maintenance. If unsuc-
cessful oi iapid iecuiience oi if associated with
geneialized psoiiasis, considei systemic tieat-
ment (see below).
Fa|ms aod So|es (Figs. 3-6 and 3-7) Occlusive
diessings with class I topical g|utotortoJs
in petiolatum. If ineffective, PUV |oot|e-
mo|eray, administeied in specially designed
hand-and-foot lighting cabinets that delivei
UVA. PUV soa|s' : In this tieatment the
hands and feet aie immeised in a solution of
8-methoxypsoialen (10 mg/L of waim watei)
foi 15 min and then exposed to hand and foot
UVA phototheiapy units. Retinoids (acitietin >
isotietinoin) given oially aie effective in iemov-
ing the thick hypeikeiatosis of the palms and
soles; howevei, combination with topical glu-
cocoiticoids oi PUVA (Re-PUVA) is much
moie efficacious. Systemic tieatments should
be consideied.
Fa|mop|aotar Fustu|oss (Fig. 3-12) The con-
dition is iecalcitiant to tieatment, but peisist-
ence in tieatment can be iewaiding.
PUVA "Svu|s" v] Hunds und Feet (See above)
Ideal foi this condition. Re-PUVA (see below) is
highly efficacious.
SCII0N 3 |'0k|A'|' 69
TvpIcu| G|ucvcvrtIcvIds, DIthrunv|, und Cvu|
Tur Ineffective. Stiong glucocoiticoids undei
plastic occlusion (e.g., foi the night) may be
effective but do not pievent iecuiiences. MTX
oi CS foi iecalcitiant cases.
Ioverse Fsorass (Fig. 3-10) Initiate theiapy
with ota| g|utotortoJs (caution: these aie
atiophy-pione iegions, steioids should be ap-
plied foi only limited peiiods of time); switch
to topical vitamin D deiivatives oi tazaiotene oi
topical taciolimus oi pimeciolimus. Tai baths
oi Castellani paint sometimes useful. If iesistant
oi iecuiient, considei systemic theiapy.
Na|s (Fig. 3-11; See also Section 33 ) Topi-
cal tieatments of the fingeinails aie unsatisfac-
toiy. Note also that nail psoiiasis may disappeai
spontaneously oi paii passu with successful
tieatment of psoiiasis. Injection of the nail
fold with intiadeimal tiiamcinolone acetonide
(3 mg/mL) effective but painful and impiactical
when all nails aie involved. PUVA photochemo-
theiapy somewhat effective when administeied
in special hand-and-foot lighting units pio-
viding high-intensity UVA. Long-teim systemic
ietinoids (acitietin, 0.5 mg/kg) aie also effec-
tive, as aie systemic MTX and CS theiapy. Since
a diseased nail (plate) cannot be cuied, theiapy
of nails aims at secuiing regrow| of a noimal
nail plate. It theiefoie depends on the speed of
nail giowth, which is slow and thus iequiies a
long time; this should be taken into account
when consideiing tieatment that may cause
side effects when administeied ovei a piolonged
peiiod of time.
CNkAIII0 FS0kIASIS
Acute, Cuttate Fsorass (Fig. 3-2) Tieat
stieptococcal infection with antibiotics. Topical
tieatment as foi localized psoiiasis. Naiiow-
band UVB iiiadiation most effective. If it fails,
oial PUVA photochemotheiapy (see below).
Ceoera|ted F|aque-Iype Fsorass (Fig. 3-4)
Peifoimed eithei by office-based deimatologist
oi in a psoiiasis centei wheie all majoi options
aie available: phototheiapy, PUVA, oi systemic
tieatments which aie given as eithei mono- oi
combined oi iotational theiapy. Combination
theiapy denotes the combination of two oi moie
modalities (as in chemotheiapy); iotational
theiapy denotes switching the patient aftei
cleaiing and a subsequent ielapse to anothei
diffeient tieatment. This is done to pievent
cumulative long-teim side effects.
Narrow-8aod v8 Fhototherapy (311 nm)
Effective only in psoiiasis with veiy thin
plaques; effectiveness is incieased by combi-
nation with topical glucocoiticoids, vitamin D
analogues, tazaiotene, oi topical taciolimus/
pimeciolimus.
0ra| FvA Fhotochemotherapy Tieatment
consists of oial ingestion of 8-methoxypsoi-
alen (8-MOP) (0.6 mg 8-MOP pei kilogiam
body weight) oi, in some Euiopean countiies,
5-MOP (1.2 mg/kg body weight) and exposuie
to doses of UVA that aie adjusted to the sensi-
tivity of the patient. UVA is given 1 h (8-MOP)
oi 2 h (5-MOP) aftei ingestion of the psoialen,
staiting at a dose of 0.5 to 1 J/cm
2
, adjusted
upwaid foi skin phototype. Alteinatively, pho-
totoxicity testing is done piioi to tieatment,
which peimits a bettei adjustment of the UVA
dose to the individual`s sensitivity to PUVA. The
UVA dose is incieased at successive tieatment
sessions. Tieatments aie peifoimed two oi thiee
times a week oi, with a moie aggiesive piotocol,
foui times a week. Most patients cleai aftei 19 to
25 tieatments, and the amount of UVA needed
ianges fiom 100 to 245 J/cm
2
.
Lvng-term sIde e]]ects : PUVA keiatoses and
squamous cell caicinomas in some patients
who ieceive an excessive numbei of tieatments.
Re-PUVA (see below) ieduces the total numbei
of tieatments.
In patients with iecalcitiant plaque-type pso-
iiasis, acitietin (in males) oi isotietinoin (in
females) may be combined with othei anti-
psoiiatic theiapy, e.g., PUVA, UVB (311 nm),
topical glucocoiticoids, oi anthialin. These
combination modalities ieduce the length of
tieatments as well as the total amount of an-
tipsoiiatic diug necessaiy foi cleaiing. Topi-
cal glucocoiticoids, calcipotiiene ointments,
anthialin, oial MTX, and oial acitietin, com-
bined with eithei PUVA oi 311-nm UVB, aie
all effective in ieducing the dose of one anothei.
0ra| ketoods Acitietin, and isotietinoin aie
veiy effective in inducing desquamation but
only modeiately effective in suppiessing pso-
iiatic plaques (an exception is pustulai psoiia-
sis-see below). They aie highly effective when
combined accoiding to established piotocols
with 311-nm UVB oi PUVA (called Re-PUVA).
T|e |aer s n [at |e mos e[[ete |eray o
Jae [or genera|:eJ |aque sorass. A com-
bination of PUVA with acitietin (0,75 mg/kg
body weight) is used foi males; foi females,
PUVA is combined with isotietinoin (1 mg/
kg body weight). Contiaception is mandatoiy
duiing tieatment and foi 2 months aftei it is
completed. Combinations of oial ietinoids and
PUVA impiove the efficacy of each and peimit
a ieduction of the dose and duiation of each if
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' T0
iefiactoiy to tieatment. Foi side effects of ietin-
oids, see page 80 and package inseit.
Methotrexate Iherapy Oial MTX is one of the
most effective tieatments and ceitainly the most
convenient tieatment foi geneialized plaque
type psoiiasis. Neveitheless, MTX is a poten-
tially dangeious diug, piincipally because of
livei toxicity that can occui aftei piolonged use.
Also, iesponse is slow and long-teim tieatment
is iequiied. Hepatic toxicity may occui aftei cu-
mulative doses in noimal peisons ( 1.5 g), but
additional iisk factois include a histoiy of oi ac-
tual alcohol intake, abnoimal livei chemistiies,
IV diug use, and obesity. Inasmuch as hepatic
toxicity is ielated to total life dose, this theiapy
should, in geneial, not be given to young pa-
tients who may face many yeais of theiapy.
Schedu|e v] Methvtrerute Therupy wIth the
TrIp|e-Dvse (WeInsteIn) RegImen Piefeiied
by most ovei the single-dose MTX once weekly.
Begin with a test dose of 5 mg (2.5-mg tablet
followed 24 h latei with a second 2.5-mg tablet);
this dose will asceitain whethei theie is a special
sensitivity to MTX. A complete blood count
(CBC), livei function tests, and seium cie-
atinine levels aie obtained befoie stait of tieat-
ment, aftei 1 week, and 2 weeks theieaftei as the
dose of MTX is incieased. One tablet (2.5 mg)
is given eveiy 12 h foi a total of thiee doses,
i.e., 7.5 mg/week (1/1/1 tablet schedule). Some
patients iespond to this dose; if not, the dose is
incieased aftei 2 weeks to 2/2/2, oi 15 mg/week
total dose, to which most patients iespond. This
iegimen achieves an 80% impiovement but
total cleaiing only in some, and highei doses
inciease the iisk of toxicity. Highei doses may
be needed in oveiweight patients. As patients
iespond the dose of MTX can be ieduced by
one oi two tablets peiiodically.
CBC, LIver FunctIvn, und CreutInIne These
have to be monitoied eveiy 3 months. In pa-
tients with noimal livei chemistiies and no
iisk factois piesent, a livei biopsy should be
done aftei a cumulative dose of appioximately
1500 mg MTX; if the post-MTX livei biopsy
is noimal, iepeat livei biopsy should be done
aftei fuithei theiapy with an additional 1000
to 1500 mg MTX. Be awaie of the vaiious diug
inteiactions with MTX.
Cyc|osporoe
1
CS tieatment is highly effective
at a dose of 3-5 mg/kg pei day. As the patient
iesponds, the dose is tapeied to the lowest
effective maintenance dose. Monitoiing blood
piessuie and seium cieatinine is mandatoiy be-
cause of the known nephiotoxicity of the diug.
CS s|ou|J |e em|oyeJ on|y n aens w|ou
rs| [ators .
Moooc|ooa| Aotbodes aod Fusoo Froteos
2
(so-called biologicals) Some of these pioteins,
specifically taigeted to pathogenically ielevant
ieceptois on T cells oi to cytokines, have been
appioved and moie aie being developed. They
should be employed only by specifically tiained
deimatologists who aie familiai with the dos-
age schedules, diug inteiactions, and shoit- oi
long-teim side effects.
A|e]ucept is a human lymphocyte function-
associated antigen (LFA)-3-IgG1 fusion piotein
that pievents inteiaction of LFA-3 and CD2.
CD2 is upiegulated in memoiy effectoi T cells
(CD45Ro
-
), which explains the piefeiential
depletion of these cells by Alefacept. It is given
intiamusculaily once weekly, but moie than
one-thiid of patients do not iespond foi un-
known ieasons. Repeated administiation leads
to impioved iesponse and theie may be long
peiiods of iemissions.
E]u|Izumuh is a humanized anti-CD11a mon-
oclonal antibody that blocks the inteiaction of
LFI-1 with its ligand inteicellulai adhesion
molecule 1. It is given subcutaneously once a
week and is usually highly effective, but some
patients show exaceibation of disease and theie
aie iebounds.
Tumvr necrvsIs ]uctvr (TNF) untugvnIsts
that aie effective in psoiiasis aie infiliximab,
adalimumab, and etaneicept. In[|xma| is a
chimeiic monoclonal antibody with a high
specificity, affinity, and avidity foi TNF- . It is
administeied as intiavenous infusion at weeks
0, 2 and 6 and is highly effective in psoiiasis
(although cuiiently only FDA appioved foi
psoiiatic aithiitis). Ja|muma| is also veiy
effective. It is a fully human iecombinant mon-
oclonal antibody that specifically taigets TNF-
. It is administeied subcutaneously eveiy othei
week and is similaily as effective as infliximab.
Eanerte is a human iecombinant, soluble
TNF- ieceptoi that binds TNF- and neutial-
izes its activity. It is administeied as subcutane-
ous injections twice weekly and is less effective
than infliximab and adalimumab but is highly
effective in psoiiatic aithiitis.
1
Foi details and diug inteiactions, see MJ Mihatsch,
K Wolff: Consensus Confeience on Cyclospoiin A foi
Psoiiasis. Bi J Deimatol 126:621, 1992.
2
Foi details and diug inteiaction, see S Richaidson, J
Gelfand, in K Wolff et al (eds): F:art|'s Der-
mao|ogy, n Cenera| MeJtne, 7th ed, New Yoik,
McGiaw-Hill, pp 2223-2236, 2008.
SCII0N 3 |'0k|A'|' T1
AntI-Inter|eu|In(1L) 12/Inter|eu|In 2J p40
is a newei agent developed foi chionic plaque
psoiiasis and has shown piomising efficacy in
phase I tiials. It is a human IgG1 monoclonal
antibody that binds to the common p40 subunit
of human IL-12 and IL-23, pieventing its intei-
action with its ieceptoi.
All these biologicals and otheis cuiiently
developed and in clinical tiials have side effects,
and theie aie long-teim safety conceins. Also,
they aie cuiiently extiemely expensive which
limits theii use in clinical piactice. Foi doses,
wainings, and side effects see
2
and package in-
seits.
CNkAIII0 FSIIAk FS0kIASIS
(S FICS. 3-13, 3-14)
These ill patients with geneialized iash should
be hospitalized and tieated in the same man-
nei as patients with extensive buins, toxic
epideimal neciolysis, oi exfoliative eiythio-
deima-in a specialized unit: isolation, fluid
ieplacement, and iepeated blood cultuies aie
necessaiy. Rapid suppiession and iesolution
of lesions is achieved by oial ietinoids (acitie-
tin, 50 mg/d). Suppoitive measuies should
include fluid intake, IV antibiotics to pievent
septicemia, caidiac suppoit, tempeiatuie con-
tiol, topical lubiicants, and antiseptic baths.
Systemic glucocoiticoids to be used only as
iescue inteivention as iapid tachyphylaxis oc-
cuis. Oial PUVA photochemotheiapy is effec-
tive, but logistics aie usually piohibitive in a
toxic patient with fevei.
ACk00kMAIIIIS C0NIINA hAII0FA
(Figuie 3-15) Oial ietinoids as in von Zum-
busch pustulai psoiiasis; MTX, once-a-week
schedule, is the second-line choice.
FS0kIAIIC AkIhkIIIS
Should be iecognized eaily in oidei to pievent
bony destiuction. MTX, once-a-week schedule
as outlined above; infliximab oi etaneicept aie
highly effective.
T2
S E C I | 0 N 4
IChIh0SS
CIASSIFICAII0N
Dominant ichthyosis vulgaiis (DIV)
X-linked iecessive ichthyosis (XLI)
Lamellai ichthyosis (LI)
Epideimolytic hypeikeiatosis (EH)
Individual keiatin genes may not be expiessed
oi may iesult in the foimation of abnoimal
keiatins. In DIV and XLI, foimation of thick-
ened stiatum coineum is caused by incieased
adhesiveness of the stiatum coineum cells and/
oi failuie of noimal cell sepaiation. Abnoimal
stiatum coineum foimation iesults in vaiiable
incieases in tiansepideimal watei loss. The
etiology of the most common ichthyosis, DIV,
is unknown, but theie aie mutations in the
A |oup o| |e|ed||+|, d|o|de| c|+|+c|e||/ed
|, +u e\ce +ccuru|+||ou o| cu|+ueou c+|e,
.+|,|u ||or .e|, r||d +ud +,rp|or+||c |o |||e
|||e+|eu|u.
A |e|+||.e|, |+|e uur|e| o| |,pe o| |e|ed||+|,
|c|||,oe e\||, ro| +|e e\||ere|, |+|e +ud
o||eu p+|| o| ru|||o|+u ,ud|ore. I|e |ou|
ro| corrou +ud |rpo||+u| |,pe +|e d|cued
|e|e p|u + |||e| d|cu|ou o| |Wo |,pe +||ec||u
ueW|o|u.
'e|ec|ed |+|e, |u| |rpo||+u|, |e|ed||+|, |c|||,o
e +|e d|cued |u ||e ou||ue .e||ou.
Acqu||ed |c|||,o| c+u |e + r+u||e|+||ou o|
,|er|c d|e+e, r+||u+uc,, d|u, eudoc||ue
d|e+e, +u|o|rruue d|e+e, +ud n|\ +ud o||e|
|u|ec||ou.
'uppo|| |oup uc| + |ouud+||ou |o| |c|||,o|
+ud ke|+|ed '||u I,pe (||k'I) e\||.
|o| +u |udep|| d|cu|ou o| |c|||,oe, ee
| ||ec|r+u, ll ||C|o.+uu+, |u K wo||| e| +| (ed).
||cc|:| |-c||, C--c| !-!:- , 1||
ed. |eW \o||, \cC|+Wn|||, pp +0-+2+, 2003.
gene encoding piofilaggiin; in XLI, theie is a
steioid sulfatase deficiency. LI shows incieased
geiminative cell hypeiplasia and incieased tian-
sit iate thiough the epideimis, and theie is a
tiansglutaminase deficiency. In EH, theie aie
mutations in the genes encoding keiatins 1 oi
10; heie the distuibance of epideimal diffeien-
tiation and the expiession of abnoimal keiatin
genes iesult in vacuolization of the uppei epi-
deimal layeis, blisteiing, and hypeikeiatosis.
All foui types of ichthyosis tend to be woise
duiing the diy, cold wintei months and impiove
duiing the hot, humid summei. Patients living
in tiopical climates may iemain symptom-fiee
but may expeiience appeaiance oi woisening of
symptoms on moving to a tempeiate climate.
C|+|+c|e||/ed |, uu+||, r||d eue|+||/ed \e|o|
W||| c+||u, ro| p|ououuced ou |oWe| |e, |u
e.e|e c+e |+|e, |ee||+|ed c+|e occu|.
n,pe|||ue+| p+|r +ud o|e.
|e|||o|||cu|+| |,pe||e|+|o| (|e|+|o| p||+||) uu
+||, ou +|r +ud |e.
||equeu||, +oc|+|ed W||| +|op,.
|C|9 . 151.
|C|0 . 0 30.0
00MINANI IChIh0SIS vICAkIS (0Iv)
FI0MI0I0C
Ae oI 0oset 3 to 12 months.
Autosomal dominant inheiitance.
Iocdeoce Common (1 in 250).
SCII0N 4 |CnIn\0'E' T3
FICk 4-1 Ichthyoss vu|ars : chest ||ue ||| c+|e|||e |,pe||e|+|o| o| ||e pec|o|+| +|e+. I|| | + r||d
|o|r o| |c|||,o| .u|+||.
FICk 4-2 Ichthyoss vu|ars: |es
C|+,|| |ee||+|ed (|||e|||e), |||r|, |ouud doWu
c+|e. I|e |r||+|||, |o ||| ||u o| ||e ||u o| +u
+rp||||+u | qu||e o|.|ou. |o|e p+||u o|
pop|||e+| |o+e. I|| | + ro|e e.e|e |o|r o|
|c|||,o| .u|+||.
FAIh0CNSIS
Etiology unknown. Theie is ieduced oi absent
filaggiin. Epideimis piolifeiates noimally, but
keiatin is ietained with a iesultant thickened
stiatum coineum.
Veiy commonly associated with atopy. Xeiosis
and piuiitus woise in wintei months. Cosmetic
concein to many patients, paiticulaily when
hypeikeiatosis is seveie.
Sko Iesoos Xeiosis (diy skin) with fine,
powdeiy scaling but also laigei, fiimly adheient
tacked-down scales in a fish-scale pattein (Figs.
4-1 and 4-2). Diffuse geneial involvement,
accentuated on the shins, aims, and back but
also on the buttocks and lateial thighs; axillae
and the antecubital and popliteal fossae spaied
(Fig. 4-2; Image 4-1); face usually also spaied
but cheeks and foiehead may be involved.
Keraoss |ars is peiifolliculai hypeikeiatosis
with little, spiny hypeikeiatotic folliculai
papules of noimal skin coloi, eithei giouped oi
disseminated, mostly on the extensoi suifaces
of the extiemities (Fig. 4-3); in childhood, also
on cheeks. Hands and feet usually spaied, but
palmoplantai maikings aie moie accentuated
(hypeilineai).
Assocated 0seases Moie than 50% of indi-
viduals with DIV also have atopic deimatitis;
iaiely, keiatopathy can occui.
Xeross[hyperkeratoss Xeiosis; acquiied ich-
thyoses, all othei foims of ichthyosis.
0ermatopatho|oy Compact hypeikeiatosis;
ieduced oi absent gianulai layei; geimina-
tive layei flattened. Election micioscopy: small,
pooily foimed keiatohyalin gianules.
0IACN0SIS
Usually by clinical findings; abnoimal keiato-
hyalin gianules in election micioscopy.
C0kS AN0 Fk0CN0SIS
Impiovement in the summei, in humid cli-
mates, and in adulthood. Keiatosis pilaiis oc-
cuiiing on the cheeks duiing childhood usually
impioves duiing adulthood.
MANACMNI
hydratoo oI Stratum Coroeum Pliability of
stiatum coineum is a function of its watei
content. Hydiation best accomplished by im-
meision in a bath followed by the application of
petiolatum. Uiea-containing cieams bind watei
in the stiatum coineum.
kerato|ytc Aeots Piopylene glycol-glyceiin-
lactic acid mixtuies. Piopylene glycol (44-60%
in watei); 6% salicylic acid in piopylene glycol
and alcohol, which is used undei plastic oc-
clusion (bewaie of hypeisalicism). -Hydioxy
acids (lactic acid oi glycolic acid) contiol scal-
ing. Uiea-containing piepaiations (2-10%) aie
effective.
Systemc ketoods Isotietinoin and acitietin
aie veiy effective, but caieful monitoiing foi
toxicity is iequiied. Only seveie cases may
iequiie inteimittent theiapy.
IMAC 4-1 0strbutoo oI chthyoss vu|ars
|o| |ud|c+|e |e|+|o| p||+||
0ccu| |u r+|e.
0ue| oou +||e| |||||.
||or|ueu|, d|||, ||oWu c+|e ou ||e uec|,
e\||er|||e, ||uu|, +ud |u||oc|.
|u.o|.ereu| o| ||e\u|+| |e|ou.
A|euce o| p+|r +ud o|e |u.o|.ereu|.
Co|ue+| op+c|||e |u 50 o| +du|| r+|e.
X-IINk0 IChIh0SIS (XII) |C|9 . 151.
|C|0 . 0 30.
FI0MI0I0C
Ae oI 0oset Biith oi infancy. Males.
Iocdeoce 1:2000 to 1:6000.
X-linked iecessive; gene locus Xp22.32.
Ceoetc 0eIect Steioid sulfatase deficiency-
abnoimal cholesteiol metabolism, accumulation
of cholesteiol sulfate; is associated with failuie to
shed senescent keiatinocytes noimally, iesulting
clinically in ietention hypeikeiatosis associated
with noimal epideimal piolifeiation.
Onset of skin abnoimality at 2-6 weeks of age;
coineal opacities develop duiing the second to
thiid week. Usually asymptomatic; may also be
piesent in female caiiieis of XLI. Discomfoit
due to xeiosis. Cosmetic disfiguiement due to
the diity biown scales.
Sko Iesoos Laige adheient scales that
appeai biown oi diity (Fig. 4-4); most
pionounced on posteiioi neck, extensoi aims,
antecubital and popliteal fossae, and tiunk.
Absence of palm/sole and face involvement
(Image 4-2).
FICk 4-3 Ichthyoss vu|ars. keratoss p|ars: arm 'r+||, |o|||cu|+|, |o|u, p|ue occu| + + r+u|
|e|+||ou o| r||d |c|||,o| .u|+||, +|||u ro||, ou ||e |ou|de|, uppe| +|r, +ud ||||. |equ+r+||ou o|
||e uou|o|||cu|+| ||u |eu|| |u |,pore|+uo||c (|e p|reu|ed) po| |r||+| |o p||,||+| +||+ (corp+|e W|||
||. !o).
ye Iesoos Comma-shaped stiomal coineal
opacities in 50% of adult males, asymptomatic.
Piesent in some female caiiieis.
Ceotouroary Aboorma|ty Ciyptoichidism
in 20% of individuals.
All foims of ichthyosis, syndiomic ichthyoses.
Chemstry Cholesteiol sulfate level elevated.
Incieased mobility of -lipopioteins in electio-
phoiesis. Steioid sulfatase decieased oi absent.
0ermatopatho|oy Hypeikeiatosis; gianulai
layei piesent, sometimes hypeigianulosis.
0IACN0SIS
By family histoiy and clinical findings.
Freoata| 0aooss Via amniocentesis and
choiionic villus sampling; steioid sulfatase assay
detects enzyme deficiency.
C0kS AN0 Fk0CN0SIS
No impiovement with age. Usually woise in
tempeiate climates and in wintei season. With
placental sulfatase deficiency, failuie of laboi to
begin oi piogiess in mothei caiiying affected
fetus.
MANACMNI
Iopca| Iherapy
hydratoo oI Stratum Coroeum Pliability of
stiatum coineum is a function of its watei
content. Hydiation best accomplished by im-
meision in a bath followed by the application of
petiolatum. Uiea-containing cieams bind watei
in the stiatum coineum.
kerato|ytc Aeots Piopylene glycol-glyceiin-
lactic acid mixtuies. Piopylene glycol (44-60%
in watei); 6% salicylic acid in piopylene glycol
and alcohol, which is used undei plastic occlu-
sion. -Hydioxy acids (lactic acid oi glycolic
acid) (bewaie of hypeisalicism) contiol scal-
ing. Uiea-containing piepaiations (2-10%) aie
effective.
Systemc ketoods Isotietinoin and acitietin
aie veiy effective, but caieful monitoiing foi
toxicity is iequiied. Only seveie cases may
iequiie inteimittent theiapy.
Systemc Ireatmeot
tren , 0.5-1 mg/kg oially until maiked im-
piovement, then tapei dose to maintenance
level. Continuous laboiatoiy monitoiing and,
in long-teim iegimens, x-iays foi calcifications
and diffuse idiopathic skeletal hypeiostosis
(DISH) syndiome mandatoiy.
IMAC 4-2 0strbutoo oI
X-|oked chthyoss.
SCII0N 4 |CnIn\0'E' TT
FICk 4-4 X-|oked chthyoss: truok, buttocks aod arms |+|| |,pe||e|+|o| W||| |ee||+|ed c+|e
|.e + d|||, +ppe+|+uce |u ||| 2,e+|o|d |o, o| A|||c+u e||u|c||,.
0||eu p|eeu| +| ||||| + co||od|ou |+|, (ee
p+e 13)
Co||od|ou|||e rer||+ue oou |ed W||| u|e
queu| |+|e, co+|e c+|e |u.o|.|u eu|||e |od,.
||e\u|+| +|e+ |u.o|.ed.
|+|rop|+u|+| |u.o|.ereu| .+||e.
E\||op|ur, ec|+||ur, +|opec|+ r+, occu|.
ne+| |u|o|e|+uce.
IAMIIAk IChIh0SIS (II) |C|9 . 151.
|C|0 . 0 30.2
FI0MI0I0C
Ae oI 0oset At biith, usually as collodion
baby.
Sex Piesents equally in both sexes.
Iocdeoce 1:300,000.
Mode oI Iohertaoce Autosomal iecessive;
gene locus vaiies. In one subset theie is muta-
tion in the gene encoding tiansglutaminase 1,
an enzyme that catalyzes the cioss-linking of
pioteins duiing the foimation of coinified en-
velopes of coineocytes. In anothei, a mutation
in the gene encoding ATP-binding cassette, sub-
family A, membei 12-contiolling membiane
tianspoit/lipid metabolism. In a thiid subset,
theie is a mutation in the gene encoding aia-
chidonate lipoxygenase, contiolling hydiopei-
oxidase isomeiase .
1
Heat intoleiance, usually duiing exeicise and
hot weathei, because of inability to sweat. Watei
loss (excess)/dehydiation due to fissuiing of
stiatum coineum. Incieased nutiitional ie-
quiiements foi young childien due to iapid
giowth and shedding of stiatum coineum.
Painful palmai/plantai fissuies.
Sko Iesoos Newhvrn Collodion baby,
encased in a tianslucent collodion-like mem-
biane (see Fig. 4-8); shed in a few weeks.
Ectiopion; eclabion. Geneialized eiythiodeima.
1
Foi details on genes identified in autosomal ieces-
sive ichthyoses, see P Fleckman, JJ DiGiovanna,
in K Wolff et al: F:art|'s Dermao|ogy n Cen-
era| MeJtne , 7th ed. New Yoik, McGiaw-Hill,
pp 401-424, 2008.
1
Foi details on genes identified in autosomal ieces-
sive ichthyoses, see P Fleckman, JJ DiGiovanna,
in K Wolff et al: F:art|'s Dermao|ogy n Cen-
era| MeJtne , 7th ed. New Yoik, McGiaw-Hill,
pp 401-424, 2008.
ChI|d/Adu|t Laige paichment-like hypeikeia-
tosis (Fig. 4-5) ovei entiie body; fiactuiing of
the hypeikeiatotic plate iesults in a tessellated
(tilelike) pattein (Fig. 4-6). Scales aie laige and
veiy thick and biown, ovei most of the body
(Fig. 4-6), accentuated on lowei extiemities,
and involving the flexuial aieas. Hypeikeiatosis
aiound joints may be veiiucous. . Hands/feet:
keiatodeima; accentuation of palmai/plantai
cieases (Image 4-3) but may vaiy. Eiythio-
deima may develop.
har Bound down by scales; fiequent infec-
tions may iesult in scaiiing alopecia (Fig. 4-5).
Na|s Dystiophy secondaiy to nail fold in-
flammation.
Mucous Membraoes Usually spaied.
ye Iesoos Ectiopion (Fig. 4-5).
Ips Eclabium.
X-linked ichthyosis, epideimolytic hypeikeia-
tosis, congenital ichthyosifoim eiythiodeima,
syndiomic ichthyoses.
Cu|ture Rule out secondaiy infection and
sepsis, especially in newboins.
0ermatopatho|oy Hypeikeiatosis; gianulai
layei piesent; acanthosis. Epideimal tiansgluta-
minase decieased in tiansglutaminase-deficient
subtype.
C0kS AN0 Fk0CN0SIS
Collodion membiane piesent at biith is shed
within fiist few days to weeks (see Fig. 4-8). New-
boins aie at iisk foi hypeinatiemic dehydiation,
secondaiy infection, and sepsis. Disoidei pei-
sists thioughout life. No impiovement with age.
|ame||ar chthyoss.
FICk 4-5 Iame||ar
chthyoss |+|c|reu||||e
|,pe||e|+|o| |.e ||e
|rp|e|ou o| ||e ||u |e|u
|oo |||| ou ||e |+ce o| |||
o,e+|o|d A|+| |o,. I|e|e
| p|ououuced ec||op|ur
+ud |e|uu|u +|opec|+.
Obstiuction of ecciine sweat glands with iesult-
ant impaiiment of sweating.
MANACMNI
Newboro Caie in neonatal intensive caie unit.
High-humidity chambei. Emolliation. Monitoi
electiolytes, fluids. Follow foi signs of local oi
systemic infection.
Ch|d[Adu|t Emv||Ients Hydiated petiola-
tum.
Kerutv|ytIcs As in DIV and XLI.
0verheato Paients and affected individuals
should be instiucted about oveiheating and
heat piostiation that can follow exeicise,
high enviionmental tempeiatuies, and fevei.
Repeated application of watei to skin can some-
what ieplace function of sweating, cooling the
body.
ketoods Acitietin and, to a lessei degiee,
isotietinoin (0.5-1 mg/kg) aie effective. Monitoi
continuously foi seium tiiglyceiides, tiansami-
nases, and bony toxicities if given ovei pio-
longed peiiod of time. Teiatogenicity iequiies
effective contiaception.
FICk 4-6 Iame||ar chthyoss: Shou|der Iee|+|ed (|||e|||e) |,pe||e|+|o| |.e ||e +ppe+|+uce o|
|ep||||+u c+|e ou ||e |ou|de| +ud |+c|. I|e eu|||e |od, W+ |u.o|.ed +ud ||e|e W+ ec||op|ur.
SCII0N 4 |CnIn\0'E' 81
||eeu| +| o| |o|||, +||e| ||||| W||| ||||e||u.
w||| ||re ||u |ecore |e|+|o||c, .e||ucou.
'|edd|u o| |,pe||e|+|o||c r+e |eu|| |u
c||curc|||ed ||+ud o| uo|r+|+ppe+||u ||u.
|u.o|.ereu| o| ||e\u|+| +|e+.
|+|r+| +ud p|+u|+| |u.o|.ereu|.
'ecoud+|, p,oeu|c |u|ec||ou.
FI0kM0IIIC hFkkkAI0SIS (h)
|C|9 . 151.
|C|0 . 0 30.3
FI0MI0I0C
Ae oI 0oset Biith oi shoitly theieaftei.
Iocdeoce Veiy iaie.
Mode oI Iohertaoce Autosomal dominant.
Mutations of genes that encode the epideimal
diffeientiation keiatins, keiatin 1 and 10.
2
Stiuc-
tuial piotein abnoimality keiatin inteimedi-
ate filament dysfunction epideimal fiagility.
Blisteiing may iecui peiiodically, leading to
denuded aieas, secondaiy infection, and sepsis.
Hypeikeiatotic lesions become veiiucous, pai-
ticulaily in the flexuial aieas, and aie associated
with an unpleasant odoi.
Sko Iesoos Blisteiing at biith oi shoitly
theieaftei. Geneialized oi localized. Denuded
aieas heal with noimal-appeaiing skin. With
time, the skin becomes keiatotic and veiiucous
(Fig. 4-7), paiticulaily in the flexuial aieas,
knees, and elbows. Hypeikeiatotic scales adheie
to undeilying skin, often in a mountain iange-
like appeaiance; may be quite daik in coloi and
associated with an unpleasant odoi (like iancid
buttei). Recuiient blisteis in hypeikeiatotic aieas
(Fig. 4-7) and also shedding of hypeikeiatotic
masses iesult in ciicumsciibed aieas of skin that
aie ielatively noimal in appeaiance. A valuable
diagnostic sign. Secondaiy pyogenic infections,
especially impetigo.
Geneialized distiibution with piominent in-
volvement of flexuial aieas (Image 4-4). Palmai
and plantai involvement (hypeikeiatosis).
(Noe : A vaiiant of epideimolytic hypeikeiato-
sis is localized to palms and soles and is geneti-
cally distinct fiom the geneialized foim.)
2
Foi othei subtypes and genes involved, see P Fleck-
man, JJ DiGiovanna, in K Wolff et al (eds): F:a-
rt|'s Dermao|ogy n Cenera| MeJtne , 7th ed. New
Yoik, McGiaw-Hill, pp 401-424, 2008.
2
Foi othei subtypes and genes involved, see P Fleck-
man, JJ DiGiovanna, in K Wolff et al (eds): F:a-
rt|'s Dermao|ogy n Cenera| MeJtne , 7th ed. New
Yoik, McGiaw-Hill, pp 401-424, 2008.
har aod Na|s Haii noimal, but involvement
of the nails may pioduce abnoimal nail plates.
Mucous Membraoes Spaied.
0ermatopatho|oy Giant, coaise keiatohyalin
gianules and vacuolization of the gianulai layei,
iesulting in cell lysis and subcoineal multilocu-
lated blisteis. Papillomatosis, acanthosis, and
hypeikeiatosis.
C0kS AN0 Fk0CN0SIS
Blistei foimation and massive hypeikeiatosis
aie pione to bacteiial supeiinfection, which is
piobably also iesponsible foi the unpleasant
odoi. Palmai involvement can adveisely affect
manual dexteiity.
MANACMNI
Topical application of -hydioxy acids. Antimi-
ciobial theiapy. Systemic ietinoids (isotietinoin
and acitietin) may tiansiently lead to a wois-
ening of the condition because of incieased
blistei foimation but latei impiove the skin
diamatically owing to a ielative noimalization
of epideimal diffeientiation. Piedisposes to im-
petigo. Deteimine dose caiefully, and monitoi
foi side effects.
FICk 4-T pdermo|ytc hyperkeratoss: arms aod haods \ouu|+|u |+ue|||e |,pe||e|+|o| o| ||e
do|ur o| |+ud W||| ||||e||u ||+| |eu|| |u e|o|ou +ud |edd|u o| |+|e |ee| o| |e|+||u.
epdermo|ytc hyperkeratoss.
IChIh0SIS IN Ih NW80kN
MANACMNI
Newboins should be kept in an incubatoi in
which the aii is satuiated with watei. Caie-
ful monitoiing of tempeiatuie and paienteial
fluids and nutiient ieplacement may be neces-
saiy foi some time. Infection of the skin and
lungs is an impoitant pioblem, and aggiessive
antibiotic theiapy may be indicated.
Euc+ereu| o| eu|||e |+|, |u + ||+up+|eu| p+|c|
reu||||e rer||+ue (||. +3A) |rp+|| |ep||+
||ou +ud uc||u.
B|e+||u +ud |edd|u o| ||e co||od|ou rer
||+ue |u|||+||, |e+d |o d||||cu|||e |u ||e|ro|eu|+
||ou +ud |uc|e+ed ||| o| |u|ec||ou.
'||u | ||||| |ed +ud ro||. A||e| |e+||u, ||u
+ppe+| uo|r+| |o| ore ||re uu||| |u o| |c|
||,o| de.e|op.
Co||od|ou |+|, r+, |e ||e |u|||+| p|eeu|+||ou o|
|+re||+| |c|||,o| o| ore |e corrou |o|r
o| |c|||,o| uo| d|cued |e|e.
Co||od|ou |+|, +|o r+, |e + coud|||ou W||c|,
+||e| ||e co||od|ou rer||+ue | |ed +ud ||e
|eu||+u| e|,||er+ |+ c|e+|ed, W||| p|o|e |o
uo|r+| ||u |o| ||e |e| o| ||e c|||d' |||e (||.
+3B).
C0II00I0N 8A8 |C|9 . 151.
|C|0 . 0 30.2
FICk 4-8 Ichthyoss o the
oewboro
'Co||od|ou |+|, |o|||, +||e|
||||| W||| + p+|c|reu||||e rer
||+ue co.e||u ||e eu|||e ||u.I|e
rer||+ue |+ |up|u|ed +ud | |e|u
|ed |e+.|u oo/|u, |+W|oo||u ||u.
A| 3 rou|| o| +e, ||e +re
|u|+u| | + |e+u|||u| |+|, W||| r|u|
r+| |e|du+| c+|e +ud e|,||er+.

I|ee +|e + uur|e| o| |+|e ,ud|or|c |c|||,oe
W|e|e |c|||,o||c ||u c|+ue +|e +oc|+|ed W|||
re|+|o||c +ud/o| |uuc||ou+| +ud ||uc|u|+| +|uo|
r+||||e.
|o| -,||!-c .cc|| |-c||:|||,
c! !-c|- (|||) ,!- C||! ,!-
+ud |-||-| ,!- ee |c|||,o| p|c|u|e
+||e|, |u ||e ou||ue .e||ou.
|o| o||e| ee | ||ec|r+u, ll ||C|o.+uu+, |u
K wo||| e| +|. ||cc|:| |-c||, C-
-c| !-!:-, 1 || ed. |eW \o||, \cC|+Wn|||, pp
+0-+2+, 2003.
SN0k0MIC IChIh0SS |C|9 . 151.
|C|0 . 0 30.9
|e.e|opreu| |u +du|||ood.
Aoc|+|ed W||| r+||u+uc|e (nod||u d|e+e
|u| +|o uounod||u |,rp|or+ +ud o||e|
r+||u+uc|e).
Aoc|+|ed W||| A||'.
Aoc|+|ed W||| +|co|do|.
Aoc|+|ed W||| ,|er|c |upu e|,||er+|ou,
de|r+|or,o|||, r|\ed couuec||.e ||ue d|e+e,
eo|uop||||c |+c||||.
Aoc|+|ed W||| |+||.e|u|o| d|e+e.
Aoc|+|ed W||| d|u (u|co||u|c +c|d, |||p+u+|o|,
|u|,|op|euo|, d|\,|+/|ue, u+|o\|d|ue.
0ccu| |u K+.+ d||u|e|. |c.c !-c||, .
ACIk0 IChIh0SS |C|9 . 10.
|C|0 . | 35.0
|+|rop|+u|+| |e|+|ode|r+ (||K) +|e + |+|e +ud
d|.e|e |oup o| |e|+||u|/+||ou d|o|de|.
||K r+, |e |ouud +|oue o| coucor||+u| W|||
(|e|+|ed) |e|ou e|eW|e|e ou ||e |od, o| r+,
|e p+|| o| corp|e\ ,ud|ore.
C||u|c+| c|+|||c+||ou d|||uu||e |e|Weeu d||
|ue, |oc+| (|||+|e), +ud puuc|+|e ||K.
',rp|or |uc|ude p+|u, +oc|+|ed W||| r+uu+|
|+|o| +ud W+|||u, +ud ecoud+|, |u|ec||ou.
I|e eue||c |+| o| ro| |KK |u.o|.e ru|+||ou
|u |e|+||u eue o| eue eucod|u couue\|u +ud
deroor+| p|o|e|u.
I|e+|reu| cou|| o| |e|+|o|,||c +ud ,|er|c
|e||uo|d.
INhkII0 kkAI00kMAS 0F FAIMS AN0 S0IS
CIASSIFICAII0N
Theie exist moie than twenty diffei-
ent PKK eithei confined to palms and soles
(simple) oi in combination with lesions else-
wheie (complex) oi as pait of a multioi-
gan syndiome (syndiomic). The undeilying
gene defects foi almost eveiyone of these aie
n+||equ|u |e|u | +u e\||ere|, |+|e coud|||ou |u
W||c| ||e c|||d | |o|u W||| .e|, |||c| p|+|e o|
||+|ur co|ueur ep+|+|ed |, deep c|+c| +ud
||u|e.
Ec|+||ur, ec||op|ou, +ud +|euce o| o| |ud|reu
|+|, e+| |eu|| |u + |o|eque +ppe+|+uce.
I|ee |+||e uu+||, d|e |o|||, +||e| |||||, |u|
||e|e +|e |epo|| o| u|.|.+| |o| Wee| |o e.e|+|
rou||.
I|| coud|||ou | d|||e|eu| ||or co||od|ou |+|,
+ud ||e o||e| |o|r o| |c|||,o|, W||| +u uuuu+|
||||ou p|o|e|u W||||u ||e ep|de|r|.
hAkIIN FIS |C|9 . 151.
|C|0 . 0 30.+
unknown.
3
In this book only thiee simple PKK
phenotypes will be discussed:
Diffuse PKK
Punctate PKK
Focal PKK
0IFFS FAIM0FIANIAk
kkAI00kMA
Two main types exist. Epideimolytic and
nonepideimolytic. Nonepideimolytic PKK is
autosomal dominant, piesenting in infancy; it
3
Foi additional discussion, including genetics and
pathogenesis, the ieadei is ieffeied to DP Kelsell, IM
Leigh, in K Wolff et al: F:art|'s Dermao|ogy n
Cenera| MeJtne , 7th ed. New Yoik, McGiaw-Hill,
pp 424-431, 2008.
3
Foi additional discussion, including genetics and
pathogenesis, the ieadei is ieffeied to DP Kelsell, IM
Leigh, in K Wolff et al: F:art|'s Dermao|ogy n
Cenera| MeJtne , 7th ed. New Yoik, McGiaw-Hill,
pp 424-431, 2008.
consists of symmetiic well-demaicated diffuse
waxy thickening of the stiatum coineum of
palms and soles (Fig. 4-9). Spiead to doisum of
hands and wiists occuis. Epideimolytic diffuse
PKK is also autosomal dominant; the keiato-
deima is also well-defined, diffuse, and sym-
metiic but is not waxy but has fine fissuiing on
the suiface.
Symptoms, if at all, consist of pain with
manual woik and walking.
FNCIAI FAIM0FIANIAk
kkAI00kMA
An autosomal dominant PKK aiising in adoles-
cence and consisting of multiple punctate keia-
toses symmetiically on palms and soles (Fig.
4-10). Lesions may iesemble palmai/plantai
waits and get woise by physical tiauma. Tendei-
ness and pain.
FICk 4-9 F|aotar keratoderma, dIIuse type \e||oW W+\, d|||ue |,pe||e|+|o| ou |o|| o|e.
F0CAI FAIM0FIANIAk
kkAI00kMA
Mostly stiiate in appeaiance, autosomal domi-
nant. Lineai hypeikeiatotic calluses usually ex-
tending fiom the palm to the tips of the fingeis
(Fig. 4-11). Aiises in childhood and gets woise
with manual laboi.
Histopathology nondiagnostic in nonepidei-
molytic PKK. Hypeikeiatosis, acanthosis, papil-
lomatosis. In epideimolytic PKK, epideimolytic
hypeikeiatosis.
Hypeikeiatotic chionic iiiitant deimatitis, pso-
iiasis, calluses, palmai/plantai waits.
FICk 4-10 Fuoctate p|aotar keratoderma \u|||p|e, d|c|e|e d|op|||e |e|+|oe |eer|||u p|+u|+|
W+||. |e|ou |+d |eeu p|eeu| |uce |+|e c|||d|ood +ud |+.e |ecore Wo|e, p+|||cu|+||, |u ||e p|eu|e +|e+.
SCII0N 4 |CnIn\0'E' 8T
C0kS AN0 Fk0CN0SIS
Does not impiove with age, life-long com-
panion. Gets woise with physical tiauma (man-
ual laboi, walking); complications aie bacteiial
and fungal infections.
MANACMNI
TvpIcu| Physical examination dbiidement
(chiiopody) ieduces keiatotic masses. Topical
keiatolytic agents: 10-20% salicylic acid
ointment, coin plasteis (20-40% salicylic acid).
40-60% piopylene glycol undei oveinight
occlusion.
SystemIc Acitietin oi, in women of childbeai-
ing age, isotietinoin, 0.5 mg/kg body weight
veiy beneficial, but shedding of hypeikeiatosis
is often associated with incieased sensitivity,
which may inteifeie with manual woik and
walking. Blisteiing may occui in epideimolytic
PKK. Bewaie of teiatogenicity and long-teim
complications.
FICk 4-11 Strate (Ioca|) pa|mar keratoderma I|e|e +|e ||ue+| .e||ucou |,pe||e|+|oe e\|eud|u
||or ||e p+|r ou|o ||e ||ue|. \+uu+| Wo|| +|+.+|e ||ee |e|ou, W||c| c+u |ecore ||u|ed +ud p+|u|u|.
88
S E C I | 0 N 5
MISCIIAN0S
FI0kMAI 0IS0k0kS
CIASSIFICAII0N
Iype 1: heredtary 8eoo AN No associated
endociine disoidei.
Iype 2: 8eoo AN Endociine disoideis associ-
ated with insulin iesistance: insulin-iesistant
type II diabetes mellitus, hypeiandiogenic
states, aciomegaly/gigantism, Cushing disease,
hypogonadal syndiomes with insulin iesistance,
Addison disease, hypothyioidism.
Iype 3: Fseudo-AN Associated with obesity;
moie common in patients with daikei pigmen-
tation. Common in metabolic syndiome. Obes-
ity pioduces insulin iesistance.
Iype 4: 0ru-oduced AN Nicotinic acid in
high dosage, stilbestiol in young males, gluco-
coiticoid theiapy, diethylstilbestiol/oial contia-
ceptive, giowth hoimone theiapy.
Iype 5: Ma|oaot AN Paianeoplastic, usually ad-
enocaicinoma of gastiointestinal oi genitouiinaiy
tiact; less commonly, lymphoma (see Section 18).
FI0MI0I0C
Ae oI 0oset Type 1: duiing childhood oi
pubeity; othei types dependent on associated
conditions.
Dependent on associated disoidei. In a subset
of women with hypeiandiogenism and insulin
A,rre|||c .e|.e|, |||c|eu|u +ud |,pe|p|
reu|+||ou o| ||e ||u, c||e||, ou ||e uec|, +\|||+,
|o|u, +ud o||e| |od, |o|d.
\+, |e |,pe||e|+|o||c +ud +oc|+|ed W||| ||u
|+.
A cu|+ueou r+||e| |e|+|ed |o |e|ed||,, o|e||,,
eudoc||ue d|o|de| (p+|||cu|+||, d|+|e|e), d|u
+dr|u|||+||ou, +ud r+||u+uc,.
|u|d|ou oue|, |u r+||u+uc,, |+p|d.
ACANIh0SIS NICkICANS (AN)
|C|9 . 10.2
|C|0 . | 3!
intoleiance and AN, loss-of-function muta-
tion in the insulin ieceptoi oi anti-insulin
ieceptoi antibodies can be found (types A
and B). It is postulated that excess giowth
factoi stimulation in the skin leads to pio-
lifeiation of keiatinocytes and fibioblasts. In
hypeiinsulinemia AN, excess insulin binding
to insulin-like giowth factoi 1 ieceptoi and
fibioblast giowth factoi ieceptoi has also
been implicated. In malignancy-associated
AN, tiansfoiming giowth factoi ieleased
fiom tumoi cells may stimulate keiatinocyte
piolifeiation via epideimal giowth factoi ie-
ceptois.
Insidious onset; fiist visible change is daikening
of pigmentation.
Sko Iesoos All types of AN: Daikening
of pigmentation, skin appeais diity (Fig.
5-1). As skin thickens, appeais velvety; skin
lines accentuated; suiface becomes iugose,
mammillated. Type 3: velvety patch on innei,
uppei thigh at site of chafing; often has
many skin tags in body folds and neck. Type
5: hypeikeiatosis and hypeipigmentation moie
pionounced (see Fig. 18-16). Hypeikeiatosis
of palms/soles, with accentuation of papillaiy
maikings: Tiipe hands" (see Fig. 18-18),
involvement of oial mucosa and veimilion
boidei of lips (see Fig. 18-17).
SCII0N 5 \|'CE||A|E0u' E|||Ek\A| ||'0k|Ek' 89
DIstrIhutIvn Most commonly, axillae; (Fig. 5-1),
neck (back, sides) also, gioins, anogenitalia,
antecubital fossae, knuckles, submammaiy, um-
bilicus.
Mucous Membraoes Oial mucosa: velvety
textuie with delicate fuiiows. Type 5: Mu-
cous membianes and mucocutaneous junc-
tions commonly involved; waity papillomatous
thickenings peiioially (see Fig. 18-17).
Ceoera| xamoatoo
Examine foi undeilying endociine disoideis
in oveiweight to moibidly obese peisons and
malignancy.
C|oca| Fodos Daik thickened flexuial
skin: Confluent and ieticulated papillomato-
sis (Gougeiot-Caiteaud syndiome), pityiiasis
veisicoloi, X-linked ichthyosis, ietention hypei-
keiatosis, nicotinic acid ingestion.
Chemstry Rule out diabetes mellitus; meta-
bolic syndiome
0ermatopatho|oy Papillomatosis, hypeikeia-
tosis; epideimis thiown into iiiegulai folds,
showing vaiious degiees of acanthosis.
Imao aod odoscopy Rule out associated
malignancy.
C0kS AN0 Fk0CN0SIS
Type 1: Accentuated at pubeity and, at times,
iegiesses when oldei. Type 2: Depends on un-
deilying distuibance. Type 3: May iegiess aftei
significant weight loss. Type 4: Resolves when
causative diug is discontinued. Type 5: AN may
piecede othei symptoms of malignancy by 5
yeais; iemoval of malignancy may be followed
by iegiession of AN.
MANACMNI
Symptomatic. Tieat associated disoidei. Topical
keiatolytic and/oi topical oi systemic ietinoids
may impiove AN.
FICk 5-1 Acaothoss orcaos \e|.e|,,
d+||||oWu |o |+, |||c|eu|u o| ||e ||u o| ||e
+|rp|| W||| p|or|ueu| ||u |o|d +ud |e+||e|ed ede
|u + !0,e+|o|d o|ee Wor+u ||or ||e \|dd|e E+|.
I|e|e We|e |r||+| c|+ue ou ||e uec|, ||e +u|ecu
|||+| |o+e, +ud ou ||e |uuc||e.
A |+|e +u|oor+| dor|u+u| |u|e|||ed d|e+e W|||
|+|e oue|.
\u|||p|e d|c|e|e c+||u, c|u|ed +ud p|u||||c
p+pu|e r+|u|, |u e|o|||e|c +ud ||e\u|+| +|e+.
\+|odo|ou +ud d|||u||u, +|o |u.o|.|u u+||
+ud rucou rer||+ue.
||c||u +ud/o| p+|u|u|.
n||o|o|c+||, c|+|+c|e||/ed |, up|+|++| +c+u
||o|,| +ud d,|e|+|o|.
C+ued |, |oo||uuc||ou ru|+||ou |u ||e 1|212
eue.
',uou,r. |+||e|w|||e d|e+e, |e|+|o| |o|||cu
|+||.
0AkIk 0ISAS (00) |C|9 . 10.

|C|0 . | 31
Raie.
Ae oI 0oset Usually in the fiist oi second
decade, males and females equally affected.
Ceoetcs Autosomal dominant tiait, new
mutations common, penetiance >95%.
Loss-of-function mutations in the TP22 gene
encoding saico/endoplasmic ieticulum calcium
adenosine tiiphosphatase isofoim 2 (SERCA 2),
which impaii intiacellulai Ca2- signaling.
Frecptato Factors Fiequently woise in sum-
mei with heat and humidity as majoi factois; can
be exaceibated by UVB, mechanical tiauma, bac-
teiial infections. Often associated with affective
disoideis and iaiely with decieased intelligence.
Usually insidious; onset is abiupt aftei piecipi-
tating factois; associated with seveie piuiitus
and often pain.
Sko Iesoos Multiple disciete scaling of ciusted,
piuiitic papules (Fig. 5-2); when scaling ciust is
iemoved, a slitlike opening becomes visible (Fig.
5-3). Confluence to laige plaques coveied by
hypeitiophic waity masses that aie foul smelling,
paiticulaily in inteitiiginous aieas.
DIstrIhutIvn Coiiesponding to the seboi-
iheic aieas": chest (Fig. 5-2), back, eais, na-
solabial folds, foiehead (Fig. 5-3), scalp; axilla,
neck, gioin.
FICk 5-2 0arer dsease: chest |||r+|, |e|ou +|e |edd||||oWu, c+||u +ud c|u|ed p+pu|e ||+| |ee|
W+||, W|eu ||o|ed. w|e|e c|u| |+.e |eeu |ero.ed ||e|e +|e ||||||e e|o|ou ||+| +|e |+|e| co.e|ed |, |ero|
||+|c c|u|.
Fa|ms aod So|es Multiple, flat, cobblestone-
like papules.
Appeodaes Haii not involved, but peimanent
alopecia may iesult fiom extensive scalp involve-
ment. Nails thin, splitting distally and showing
chaiacteiistic V-shaped scalloping (see Fig. 33-31).
Mucous Membraoes White, centially de-
piessed papules on mucosa of cheeks, haid and
soft palate, and gums, cobblestone" lesions.
0ISAS ASS0CIAII0N
Associated with atro|eraoss errut[orms,
allelic with DD. Multiple, small flat-topped
papules piedominantly on doisa of hands
and feet.
0ermatopatho|oy Dyskeiatotic cells in the
spinous layei (coips ionds) and stiatum coi-
neum (giains), supiabasal acantholysis and
clefts (lacunae), papillaiy oveigiowth of the
epideimis and hypeikeiatosis.
Diagnosis based on histoiy of familial involve-
ment, clinical appeaiance, and histopathology.
May be confused with seboiiheic deimatitis,
Giovei disease, benign familial pemphigus
(Hailey-Hailey disease), and pemphigus fo-
liaceus. Aciokeiatosis veiiucifoimis: flat waits
(veiiucae planae juveniles).
C0kS AN0 Fk0CN0SIS
Peisisting thioughout life, not associated with
cutaneous malignancies.
MANACMNI
Sunscieens to avoid UV-induced exacei-
bations, avoidance of fiiction and iubbing
(tuitle neck sweateis), antibiotic theiapy
(systemic and topical) to suppiess bacteiial
infection, topical ietinoids (tazaiotene and
adapalene) oi systemic ietinoids (isotietinoin
oi acitietin). Systemic theiapy can be modi-
fied accoiding to seasonal vaiiation of the
disease.
FICk 5-3 0arer dsease: Iorehead |+|||, co+|ec|u, |,pe||e|+|o||c p+pu|e ||+| +|e e|oded +ud
c|u|ed. I|e r+|u couce|u o| ||| ,ouu |er+|e W+ d|||u|ereu|.
FI0MI0I0C
Ae oI 0oset Middle age and oldei, mean age
50 yeais.
Sex Males > females.
Frecptato Factors Heavy, sweat-inducing ex-
eicise, excessive solai exposuie, exposuie to heat,
and peisistent fevei; may also occui in bediid-
den patients, with heat and sweating as factois.
Usually abiupt onset of piuiitus and simul-
taneous appeaiance of ciops of lesions.
Sko Iesoos Skin-coloied, pink oi ieddish
papules (small, 3 to 5 mm, some with slight
scale oi smooth) (Fig. 5-4), papulovesicles, and
eiosions. Veiy similai to Daiiei disease. Upon
palpation, smooth oi waity. Scatteied, disciete
on cential tiunk (Fig. 5-4) and pioximal
extiemities.
0ermatopatho|oy Acantholysis and spongi-
osis, focal acantholytic dyskeiatosis occuiiing at
the same time and simulating Daiiei disease,
pemphigus foliaceus, and Hailey-Hailey disease;
in the deimis theie is a supeificial infiltiate of
eosinophils, lymphocytes, and histiocytes.
A p|u||||c de|r+|o| |oc+|ed p||uc|p+||, ou ||e
||uu|, occu|||u + c|op o| d|c|e|e p+pu|+| o|
p+pu|o.e|cu|+| |e|ou, p+|e |o uure|ou.
0ccu| |u +du||.
||u|||u | r+|u ,rp|or.
uu+||, ||+u|eu| |u| + pe|||eu| |o|r |
|ecou|/ed.
|||uc|p+| |||op+||o|o|c |e+|u|e. .+||+||e |oc+|
+c+u||o|,| +ud d,|e|+|o|.
|o e.|deuce o| eue||c p|ed|po|||ou.
',uou,r. ||+u|eu| +c+u||o|,||c de|r+|o|.
Ck0vk 0ISAS (C0) |C|9 . 102.3
|C|0 . | .
Often difficult.
Sma|| 0screte Frurtc Fapu|es oo Chest Daiiei
disease, heat iash (miliaiia iubia), papulai
uiticaiia, scabies, deimatitis heipetifoimis
(heie theie is giouping and the lesions aie sym-
metiic), Pityiospoium oi eosinophilic folliculi-
tis, insect bites, and diug eiuptions.
C0kS AN0 Fk0CN0SIS
The disease is by no means always tiansient, and
theie appeai to be two types: acute (tiansient")
and chionic ielapsing. The mean duiation in
one seiies was 47 weeks.
MANACMNI
Iopca| Class I topical glucocoiticoids undei
plastic (e.g., diy-cleaning plastic suit bags with
holes cut foi aims) aie used foi 4 h.
Systemc Oial glucocoiticoids and dapsone
have been used with success, but ielapses occui
aftei withdiawal.
Fhototherapy UVB oi PUVA photochemo-
theiapy may be useful foi patients who do not
iespond to topical glucocoiticoids undei occlu-
sion. Isotietinoin has been used in iefiactoiy
cases.
CIASSIFICAII0N
1
Iype 1: C|assc Adu|t Geneialized, beginning
on head and neck.
Iype 2: Atypca| Adu|t Geneialized, spaise
haii.
Iype 3: C|assc luveo|e Appeais within the
fiist two yeais of life, geneialized.
Iype 4: Crcumscrbed luveo|e In piepubeital
childien, localized.
Iype 5: Atypca| luveo|e Onset in fiist few
yeais of life, familial, geneialized.
1
W.A.D. Giiffiths Pityiiasis iubia pilaiis. Clin Exp
Deimatol 5:105, 1980; and A. Gonzales-Lpez et al:
Bi J Deimatol 140:931, 1999.
1
W.A.D. Giiffiths Pityiiasis iubia pilaiis. Clin Exp
Deimatol 5:105, 1980; and A. Gonzales-Lpez et al:
Bi J Deimatol 140:931, 1999.
k+|e, c||ou|c, p+pu|oqu+rou d|o|de| o||eu
p|o|e|u |o e|,|||ode|r+.
'|\ |,pe e\||.
|o|||cu|+| |,pe||e|+|o||c p+pu|e, |edd||o|+ue
p|o|e|u |o eue|+||/ed e|,|||ode|r+. '|+|p|,
der+|c+|ed ||+ud o| uu+||ec|ed (uo|r+|)
||u.
w+\,, d|||ue, o|+ue |e|+|ode|r+ o| p+|r +ud
o|e, u+|| r+, |e +||ec|ed.
\o| e||ec||.e ||e|+p, | re||o||e\+|e, |e||uo|d.
FIIkIASIS k8kA FIIAkIS (FkF)
|C|9 . o9o.+
|C|0 . | ++.+
Iype 6: hIv-Assocated Geneialized, associ-
ated with acne conglobata, hidiadenitis sup-
puiativa, and lichen spinulosus.
FI0MI0I0C
Estimated incidence 1: 5000 and in 1 in 15,000
deimatology patients. Bimodal distiibution with
a peak incidence in the fiist and fifth decades of
life. Affects both sexes and occuis in all iaces.
Unknown. A dysfunction in vitamin A metabo-
lism has been suggested but not pioven. Genetic
FICk 5-4 Crover dsease A |+| cou|||u o| |edd||, |,pe||e|+|o||c c+||u +ud/o| c|u|ed p+pu|e
W||| + +udp+pe| |ee| upou p+|p+||ou. |+pu|e +|e d|c|e|e, c+||e|ed ou ||e ceu||+| ||uu| +ud .e|, p|u||||c.
factois aie believed to play a ciitical iole in the
induction of PRP.
Both insidious and iapid onset occui.
Sko Iesoos All types of PRP. An eiuption
of folliculai hypeikeiatotic papules of ieddish-
oiange coloi usually spieading in a cephalocaudal
diiection (Fig. 5-5). Confluence to a ieddish-
oiange psoiiasifoim, scaling deimatitis with
shaiply demaicated islands of unaffected skin
(Fig. 5-6). Piogiession to eiythiodeima (except
foi type 2 and type 4) (Fig. 5-7).
DIstrIhutIvn Types 1, 2, 3, 5, and 6: Geneial-
ized, classically beginning on the head and neck,
then spieading caudally.
Sca|p aod har Scalp affected, as in psoiiasis,
often leading to asbestos-like accumulation of
scale. Haii not affected except in type 2 wheie
spaise scalp haii is obseived.
Mucous Membraoes Spaied.
Na|s Common but not diagnostic. Distal
yellow-biown discoloiation, nail plate thick-
ening, subungual hypeikeiatosis, and splintei
hemoiihages. See section 33.
Assocated Coodtoos Ichthyosifoim lesions
on legs in type 2. Scleiodeima-like appeaiance
of hands and feet in type 5. Acne conglobata,
hidiadenitis suppuiativa, and lichen spinulosus
in type 6.
The diagnosis is made on clinical giounds. The
diffeiential diagnosis includes psoiiasis, follicu-
lai ichthyosis, eiythiokeiatodeima vaiiabilis,
nonbullous ichthyosifoim eiythiodeima.
Chemstry No diagnostic featuies.
hstopatho|oy Not diagnostic. Suggestive:
Hypeikeiatosis, acanthosis with bioad shoit iete
iidges, alteinating autokeiatosis and paiakeia-
tosis. Keiatinous plugs of folliculai infundibula
and peiifolliculai aieas of paiakeiatosis may be
piesent. Piominent gianulai layei may distin-
guish PRP fiom psoiiasis. Supeificial peiivas-
culai lymphocytic infiltiate.
C0kS AN0 Fk0CN0SIS
A socially and psychologically disabling con-
dition. Long duiation; type 3 often iesolves
aftei 2 yeais; type 4 may cleai. Type 5 has a veiy
chionic couise. Type 6 may iespond to highly
active antiietioviial theiapy (HAART).
MANACMNI
Topical theiapies consist of emollients, keia-
tolytic agents, vitamin D
3
(calcipioptiiol),
FICk 5-5 Ftyrass rubra p|ars (type 1, c|assc adu|t) |u|||+| |e|ou +|e d|c|e|e, d|er|u+|ed |o|
||cu|+| |,pe||e|+|o||c p+pu|e o| |edd||o|+ue co|o|.
glucocoiticoids, and vitamin A analogues
(tazaioten). All not veiy effective. Photothei-
apy ultiaviolet A phototheiapy, naiiowband
ultiaviolet B phototheiapy, and photochemo-
theiapy (PUVA)] aie effective in some cases.
Most effective tieatment consists of systemic
administiation of methotiexate oi ietinoids
(both as in psoiiasis). In type 6 HAART. The
anti-TNF agents, e.g., iemicade and enbiel, aie
effective.
FICk 5-6 0arer dsease (type 1, c|assc adu|t) Ceue|+||/ed p+pu|e |e|uu|u ou ||e |e+d +ud
uec| |+.e co+|eced ou ||e c|e| o| + 51,e+|o|d r+|e W|o |+d |+|eu e||oeu. I|e|e +|e |+|p|, der+|c+|ed
||+ud o| uu+||ec|ed uo|r+| ||u.
FICk 5-T Ftyrass rubra p|ars (type 1, c|assc adu|t) 0|+ue|edd|| p+pu|e |+.e co+|eced |o
ue+| e|,|||ode|r+, p+||u |o|+|ed ||+ud o| uo|r+| ||u. A|o uo|e e|,|||ode|r+, |u.o|.ereu| o| ||e |+ud |u
||| 55,e+|o|d Wor+u.
|'A| | ||e ro| corrou |o|r o| ||e po|o|e|+
|oe (|o| c|+|||c+||ou ee po|o|e|+|o| p|c|u|e
+||e|, |u ||e ou||ue .e||ou.)
uu||o|r|, r+||, +uuu|+| ||+| p+pu|e |+u|u
||or 2 |o 5 rr |u d|+re|e|.
||||||u|ed ,rre|||c+||, ou ||e e\||er|||e +ud
|oc+|ed p|edor|u+u||, |u uue\poed ||e.
I,p|c+||, p+|e p+|r, o|e, +ud rucou rer
||+ue.
C|+|+c|e||||c |e+|u|e. We||der+|c+|ed |,pe||
e|+|o||c |o|de| o| |ud|.|du+| |e|ou, uu+||,
rr |u |e||| W||| + c|+|+c|e||||c |ou||ud|u+|
|u||oW euc||c||u ||e eu|||e |e|ou (||. 53
+ud 59).
A |e|ou p|o|e, ||e ceu||+| +|e+ |ecore
+||op||c +ud uu||d|o||c.
',rp|or. +,rp|or+||c o| r||d|, p|u||||c co
re||c+||, d|||u||u .
Ieud |o |e |u|e|||ed + +u +u|oor+| dor|u+u|
d|o|de|.
|+||oeue| uu|uoWu.
A |eu|u coud|||ou, |u| |+|e|, + p|ecu|o| |o| |u
||u o| |u.+|.e qu+rou ce|| c+|c|uor+.
I|e+|reu|. |op|c+| 5||uo|ou|+c||, |e||uo|d, +ud |r
|qu|rod. Iop|c+| |e||uo|d r+, |rp|o.e |e|ou.
|+||eu| |ou|d |e rou||o|ed |o| 'CC.
|C|9 . o92.15
|C|0 . 0 32.3
0ISSMINAI0 SFkFICIAI ACIINIC F0k0kkAI0SIS (0SAF)
FICk 5-8 0ssemoated superIca| actoc porokeratoss \e|, ||+| +uuu|+| p+pu|e W||| + .e|,
c|+|+c|e||||c |,pe||e|+|o||c |o|de| u||ouud|u ||e |e|ou. |ud|.|du+| |e|ou |+.e co+|eced |o |||eu|+| p+|c|e,
W||c| +|e +|o u||ouuded |, ||e po|o|e|+|o||c |o|de|. I|| | ou ||e |oWe| +|r o| + 55,e+|o|d |er+|e W|o |+d
|eeu c||ou|c+||, uue\poed |o| dec+de. I|e e|up||ou W+ d|||||u|ed ,rre|||c+||, ou +|r +ud |e |u| ou|,
|u uue\poed ||e.
SCII0N 5 \|'CE||A|E0u' E|||Ek\A| ||'0k|Ek' 9T
FICk 5-9 0ssemoated superIca| actoc porokeratoss 'r+|| +uuu|+| ||+| p+pu|e up |o + rr |u
d|+re|e| u||ouuded |, + We||der+|c+|ed |,pe||e|+|o||c |o|de|. w||| + |+ud |eu ||e |ou||ud|u+| |u||oW euc||
c||u ||e eu|||e |e|ou c+u |e eeu.
98
8II0S 0ISASS
S E C I | 0 N 6
CIASSIFICAII0N
Based on level of cleavage and blistei foimation
theie aie thiee main types:
Epideimolytic. Cleavage occuis in keiatino-
cytes: EB simplex (EBS)
Junctional. Cleavage occuis in basal lamina:
junctional EB (JEB)
Deimatolytic. Cleavage occuis in most su-
peificial papillaiy deimis: deimolytic, oi dys-
tiophic, EB (DEB)
In each of these gioups theie aie seveial distinct
types of EB based on clinical, genetic, his-
tologic/electionmicioscopic, and biochemical
evaluation (Table 6-1). Only the most impoi-
tant aie discussed heie.
FI0MI0I0C
The oveiall incidence of heieditaiy EB is placed
at 19.6 live biiths pei 1 million biiths in the
United States. Stiatified by subtype, the inci-
dences aie 11 foi EBS, 2 foi JEB, and 5 foi DEB.
The estimated pievalence in the United States is
8.2 pei million, but this figuie iepiesents only
the most seveie cases as it does not include
the majoiity of veiy mild disease going unie-
poited.
A pec||ur o| |+|e euode|r+|oe |u W||c| +
d||u||ed co|e|euce o| ||e ep|de|r| +ud/o| de|
r| |e+d |o ||||e| |o|r+||ou |o||oW|u ||+ur+.
neuce, ||e de|u+||ou -:|c||| !-c
|-
||e+e r+u||e|+||ou |+ue ||or .e|, r||d |o
e.e|e|, ru|||+||u +ud e.eu |e||+| |o|r ||+| d||
|e| |u rode o| |u|e|||+uce, c||u|c+| r+u||e|+||ou,
+ud +oc|+|ed ||ud|u.
C|+|||c+||ou |+ed ou ||e ||e o| ||||e| |o|r+||ou
d|||uu||e |||ee r+|u |oup. ep|de|ro|,||c
o| EB |rp|e\ (EB'), juuc||ou+| EB (lEB), +ud
de|ro|,||c, o| d,||op||c, EB (|EB).
|u e+c| o| ||ee |oup ||e|e +|e e.e|+| d|||uc|
|,pe o| EB |+ed ou c||u|c+|, eue||c, |||o|o|c,
+ud ||oc|er|c+| e.+|u+||ou.
|C|9 . 151.!9
|C|0 . 0 3
hk0IIAk FI0kM0ISIS 8II0SA (8)
Ceoetc 0eIects Molecules involved aie listed
in Table 6-1 and localization in the tissue and
sites of cleavage aie shown in Image 6-1.
CIINICAI FhN0IFS
8 Smp|ex (8S)
A tiauma-induced, intiaepideimal blisteiing,
based in most cases on mutations of the genes
foi keiatins 5 and 14 iesulting in a distuibance
of the stability of the keiatin filament netwoik
(Table 6-1). This causes cytolysis of basal keiati-
nocytes and a cleft in the basal cell layei (Image
6-1). Diffeient subgioups have consideiable
phenotypic vaiiations (Table 6-1), and theie aie
eight distinct foims, most of which aie domi-
nantly inheiited. The two most common aie
desciibed below.
Generu|Ized EBS (Table 6-1) The so-called
Koebnei vaiiant is dominantly inheiited, with
onset at biith to eaily infancy. Theie is geneial-
ized blisteiing following tiauma with a piedi-
lection foi tiaumatized body sites such as feet,
hands, elbows, and knees. Blisteis aie tense oi
flaccid at fiist and lead to eiosions (Fig. 6-1).
Theie is iapid healing and only minimal scai-
iing at sites of iepeated blisteiing. Palmoplantai
SCII0N 6 Bu||0u' ||'EA'E' 99
FICk 6-1 Ceoera|ted 8S I|| +,e+| o|d ||| |+ |+d ||||e||u |uce .e|, e+||, |u|+uc, W||| p|ed||ec
||ou |o| ||+ur+||/ed |od, ||e uc| + p+|r +ud o|e |u| +|o e||oW +ud |uee. B|||e||u +|o occu| |u o||e|
+|e+ uc| + ||e |o|e+|r, + |oWu |e|e, +ud ou ||e ||uu|. |o|e ||+| dep||e ru|||p|e ||||e||u ep|ode ||e|e |
|+|d|, +u, e.|deuce o| c+|||u ou ||e p+|r o| ||| c|||d.
IMAC 6-1 |oc+||/+||ou o| |+|e| +d|e|ou ||e +ud c|e|| |o|r+||ou |u e|ec|ed |e|ed||+|, +ud +u|o|rruue
|u||ou d|e+e. (\od|||ed ||or ||. !05 |u l| Bo|ou|+ e| +|. |-c||,, \o|,, |oudou, ||||+de|p||+, 200!,
W||| pe|r||ou.)
EBA
EBS
PV
Desmosome
DGL 1,3
BPAG2
collagen
Type
VII
Laminin 5
BPAG1
Basal
Keratinocyte
Hemidesmosome
Lamina lucida
Sublamina
densa region
Lamina densa
DEB
BP, PG, LAD, CP
GABEB, HERLITZ
hypeikeiatoses may be piesent. Nails, teeth, and
oial mucosa aie usually spaied.
Lvcu|Ized EBS Webei-Cockayne subtype
(Table 6-1). This is the most common foim of
EBS. Onset in childhood oi latei. The disease
may not piesent itself until adulthood, when
thick-walled blisteis on the feet and hands oc-
cui aftei excessive exeicise, manual woik, oi
militaiy tiaining (Fig. 6-2). Incieased ambient
tempeiatuie facilitates lesions. Hypeihidiosis
of palms and soles is associated, and secondaiy
infection of blisteied lesions often occuis.
luoctooa| 8 (l8)
All foims of JEB shaie the pathologic featuie of
blistei foimation within the lamina lucida of the
basement membiane (Image 6-1). Mutations
aie in the gene foi collagen XVII and laminin
(Table 6-1). This tiait is autosomal iecessive
and compiises clinical phenotypes depending
on the type of genetic lesion and enviionmental
IA8I 6-1 C|assification of Epidermo|ysis Bu||osa
Ieve| oI Separatoo 0sease 0eIect
'|rp|e\ Ceue|+||/ed/Koe|ue| KkI5/KkI+
'|rp|e\ ne|pe|||o|r|/|oW||u\e+|+ KkI5/KkI+
'|rp|e\ |oc+||/ed/we|e|Coc|+,ue KkI5/KkI+
'|rp|e\ 0u+ KkI5/KkI+
'|rp|e\ 'upe|||c|+|| KkI5/KkI+
'|rp|e\ \o|||ed p|reu|+||ou KkI5
ner|deroor+|
c
EB W||| rucu|+| d,||op|, ||EC
ner|deroor+|
|
EB W||| p,|o||c +||e|+ |ICB+/|ICAo
luuc||ou+| C|+.|/ne||||/ |A\B!/|A\A!/|A\C2
luuc||ou+| \||| |A\B!/|A\A!/|A\C2
luuc||ou+|
c
Ceue|+||/ed +||op||c |eu|u C0|1A/|A\B!
luuc||ou+| |oc+||/ed C0|1A
|,||op||c |+|u| C0|1A
|,||op||c Coc|+,ueIou|+|ue C0|1A
|,||op||c |oc+||/ed k|EB C0|1A
|,||op||c n+||ope+u'|ereu C0|1A
\+||+||e K|ud|e| ,ud|ore K|||
|eroor+| Ec|ode|r+| d,p|+|+||u ||+||||, |K|
C0|1A, co||+eu, |,pe \||,
, EB, ep|de|ro|,| |u||o+, |ICB, |u|e||u , KkI, |e|+||u, |A\A, |+r|u|u , |A\B, |+r|u|u , |K|, p|+|op||||u,
||EC, p|ec||u, k|EB, |ece|.e d,||op||c ep|de|ro|,| |u||o+.
c
A||e|u+||.e|, c|+|||ed + |rp|e\.
|
A||e|u+||.e|, c|+|||ed + juuc||ou+|.
:
C+e W||| |,pe /\|| co||+eu c,|op|+r|c de|e||ou |oWed |o|| juuc||ou+| +ud |er|deroor+| |e.e| o| ||u ep+|+||ou.
'ou|ce. ||or \.|. \+||u|o.|c|, EA B+ue|. |u|e|||ed ep|de|ro|,| |u||o+, |u K wo||| e| +|. (ed.). ||cc|:| |-c||, C--c| !-!:-
1
||
ed|||ou. |eW \o||, \cC|+Wn|||, 2003
factois. The thiee piincipal subtypes (see Table
6-1) aie desciibed below.
JEB GruvIs (Her|Itz EB) Patients often do
not suivive infancy; the moitality iate is 40%
duiing the fiist yeai of life. Theie is geneial-
ized blisteiing at biith (Fig. 6-3) oi clinically
distinctive and seveie peiioiificial gianulation,
loss of nails, and involvement of most mu-
cosal suifaces. The skin of these childien may
be completely denuded, iepiesenting oozing
painful eiosion. Associated findings include all
symptoms iesulting fiom geneialized epithelial
blisteiing with iespiiatoiy, gastiointestinal, and
genitouiinaiy oigan systems involved.
JEB MItIs These childien may have modeiate
oi seveie JEB at biith but suivive infancy and
clinically impiove with age. Peiioiificial non-
healing eiosions duiing childhood.
Generu|Ized AtrvphIc BenIgn EpIdermv|ysIs
Bu||vsu (GABEB) GABEB is a sepaiate JEB
that piesents at biith with geneialized cutaneous
SCII0N 6 Bu||0u' ||'EA'E' 101
FICk 6-2 Ioca|ted 8S I||c|W+||ed ||||e| ou ||e o|e. I|e d|e+e p|eeu|ed ||e|| |o| ||e |||| ||re
du||u r||||+|, ||+|u|u W|eu ||| 9,e+|o|d r+u |+d |o r+|c| o.e| + |ou d||+uce.
FICk 6-3 luoctooa| epdermo|yss bu||osa (her|tt varaot) I|e|e +|e |+|e e|oded, oo/|u +ud
||eed|u +|e+ ||+| occu||ed |u||+p+||ur. w|eu ||| ueW|o|u | ||||ed up, d||odreu| o| ep|de|r| + We|| +
e|o|ou occu| W||| r+uu+| |+ud||u.
blisteiing (Fig. 6-4) and eiosions not only on
the extiemities but also on the tiunk, face, and
scalp. Suivival to adulthood is the iule, but blis-
teiing on tiaumatized aieas continues (Fig. 6-
5). It is paiticulaily pionounced with incieased
ambient tempeiatuie, and theie is atiophic
healing of the lesions. Nail dystiophy, nonscai-
iing oi scaiiing alopecia, mild oial mucous
membiane involvement; enamel defects may
occui. Mutations aie in the genes foi collagen
type XVII and laminin (Table 6-1).
0ystrophc pdermo|yss 8u||osa ( 08 )
A spectium of deimolytic diseases wheie blis-
teiing occuis below the basal lamina (Image
6-1); healing is theiefoie usually accompanied
by scaiiing and milia foimation-hence, the
name Jysro|t . Theie aie foui piincipal sub-
types, and all aie due to mutations in anchoiing
fibiil type VII collagen (Table 6-1). Anchoiing
fibiils aie theiefoie only iudimentaiy oi absent.
Of the foui main types of DEB, only two aie
desciibed below.
DvmInunt DEB Cockayne-Touiaine disease.
Onset in infancy oi eaily childhood with acial
blisteiing and nail dystiophy; milia and scai
foimation, which may be hypeitiophic oi hy-
peiplastic. Oial lesions aie uncommon, and
teeth aie usually noimal.
RecessIve DEB (RDEB) Compiises a laigei
spectium of clinical phenotypes. The localized,
less seveie foim (RDEB mitis) occuis at biith,
shows acial blisteiing, atiophic scaiiing, and
little oi no mucosal involvement. Geneialized,
seveie RDEB, the Hallopeau-Siemens vaiiant,
is mutilating. Theie is geneialized blisteiing at
biith, and piogiession and iepeated blisteiing
at the same sites (Fig. 6-6) iesult in iemaikable
scaiiing and ulceiations, syndactyly with loss
of nails (Fig. 6-7) and even mitten-like defoim-
ities of hands and feet, flexion contiactuies.
Theie aie enamel defects with caiies and paio-
dontitis, stiictuies and scaiiing in the oial mu-
cous membiane and esophagus, uiethial and
anal stenosis, and oculai suiface scaiiing; also
malnutiition, giowth ietaidation, and anemia.
The most seiious complication is squamous cell
caicinoma in chionic iecuiient eiosions.
0IACN0SIS
Based on clinical appeaiance and histoiy. His-
topathology deteimines the level of cleavage,
which is fuithei defined by election micios-
copy and/oi immunohistochemical mapping.
Westein blot, Noithein blot, iestiiction fiag-
ment length polymoiphism (RFLP) analysis,
and DNA sequences may then identify the
mutated gene.
MANACMNI
Theie is as yet no causal theiapy foi EB, but
gene theiapy is being investigated. Management
is tailoied to the seveiity and extent of skin in-
volvement and consists of suppoitive skin caie,
suppoitive caie foi othei oigan systems, and
systemic theiapies foi complications. Wound
management, nutiitional suppoit, and infec-
tion contiol aie key to the management of all
EB patients.
In EBS, maintenance of a cool enviionment
and use of soft, well-ventilated shoes, aie im-
poitant. Blisteied skin is tieated by saline com-
piesses and topical antibiotics oi, in the case of
inflammation, with topical steioids. Moie se-
veiely affected JEB and DEB patients aie tieated
like patients in a buin unit. Gentle bathing and
cleansing aie followed by piotective emollients
and nonadheient diessings.
Management of cutaneous infection is im-
poitant, and suigical tieatment is often ie-
quiied in DEB foi the ielease of fused digits and
coiiection of limb contiactuies.
Although iaie, EB and, in paiticulai, JEB and
DEB pose a majoi health and socioeconomic
pioblem. Oiganizations such as the Dystiophic
Epideimolysis Bullosa Reseaich Association
(DEBRA) offei assistance that includes patient
education and suppoit.
SCII0N 6 Bu||0u' ||'EA'E' 103
FICk 6-5 Ceoera|ted atrophc beoo epdermo|yss bu||osa (CA88) I|| 20,e+|o|d r+u |+
|+d eue|+||/ed cu|+ueou ||||e||u |uce |||||. |o|e. e|o|ou ou ||e |e|| |oWe| |+c| +ud |ero|||+|c c|u| ou
||e |oWe| +|r. E|,||er+ ou ||e |+c| |ud|c+|e ||e o| p|e.|ou ||||e||u.
FICk 6-4 Ceoera|ted atrophc beoo epdermo|yss bu||osa (CA88) I|| 9,e+|o|d r+u |+ |+d
cu|+ueou ||||e||u |uce |||||, W||| ||||e| +ud e|o|ou +|||u ou ||e e||oW +ud |uee |u| +|o ou ||e ||uu| +ud +|r
|o||oW|u ||+ur+. |o|e. |||de||ued e|,||er+ +| ||e o| p|e.|ou ||||e||u. I|e|e | uo c+|||u |u| ore po||, +||op|,.
FICk 6-6 Ceoera|ted recessve dystrophc epdermo|yss bu||osa (k08) |u ||| e.e|e d|e+e
||||e||u occu| o||eu +| ||e +re ||e, + |u ||| 0,e+|o|d |||. B|||e| |e+d |o e|o|ou +ud ||ee |ecore
u|ce| ||+| |+.e + |oW |eudeuc, |o |e+|. w|eu |e+||u occu| || |eu|| |u c+|||u. I|| ||| +|o |+ eu+re|
de|ec| W||| c+||e, |||c|u|e o| ||e eop|+u, e.e|e +uer|+, +ud cou|de|+||e |oW|| |e|+|d+||ou. || | o|.|ou
||+| ||ee |+|e Wouud +|e po||+| eu|||e |o| ,|er|c |u|ec||ou.
FICk 6-T Ceoera|ted recessve dystrophc epdermo|yss bu||osa (k08) |o o| +|| ||ue|u+||,
,ud+c|,|,, +ud e.e|e +||op||c c+|||u ou ||e do|+ o| ||e |+ud.
SCII0N 6 Bu||0u' ||'EA'E' 105
|+r|||+| |eu|u perp||u, o| n+||e,n+||e, d|
e+e, | + |+|e euode|r+|o| W||| dor|u+u| |u
|e|||+uce ||+| | c|+|c+||, dec|||ed + + ||||e||u
d|o|de| |u| +c|u+||, p|eeu| + +u e|,||er+|ou,
e|o|.e, oo/|u coud|||ou W||| c|+c| +ud ||u|e
|oc+||/ed |o ||e u+pe o| ||e uec|, +\|||+e (||. o3).
'u|r+rr+|, |e|ou, |uu|u+| |o|d, +ud c|o
|ur c- c |- | .|.--|.
I|e uude||,|u p+||o|o|c p|oce | +c+u||o|,|
W|e|e|, ||e ||+||||, o| ||e ep|de|r| | due |o +
de|ec| |u ||e +d|e|ou corp|e\ |e|Weeu dero
or+| p|o|e|u +ud |ouo|||+reu|.
I|e eue||c +|uo|r+|||, ||e |u 1|2C|, W||c|
eucode +u AI|poWe|ed c+|c|ur purp.
0ue| | uu+||, |e|Weeu ||e ||||d +ud |ou|||
dec+de.
0||eu r||+|eu |o| |u|e||||o, c+ud|d|+|, o| |||c
||ou+| o| cou|+c| de|r+||||.
|ud|.|du+| |e|ou cou|| o| r|c|ocop|c+||, r+||
||+cc|d .e|c|e ou +u e|,||er+|ou |+c||ouud
||+| oou |u|u |u|o e|oded p|+que W||| ||e de
c|||ed, ||||, c|+|+c|e||||c, ||u|ed +ppe+|+uce
(||. o3).
C|u||u, c+||u, +ud |,pe|||op||c .ee|+||.e
|oW|| r+, occu|.
n||o|o, e\p|+|u ||e c||u|c+| +ppe+|+uce +
ep|de|r+| ce|| |oe ||e|| co|e|euce W||| +c+u
||o|,| |||ou|ou| ||e ep|||e||ur, |.|u ||e
+ppe+|+uce o| + d||+p|d+|ed |||c| W+||.
Co|ou|/+||ou o| ||e |e|ou, p+|||cu|+||, |, '|c|,
|::: c- | + |||e| |o| |u|||e| +c+u||o|,
| +ud r+|u|eu+uce o| ||e p+||o|o|c p|oce.
'ecoud+|, co|ou|/+||ou |, Cc!!c |+ + |r||+|
e||ec|.
I|e+|reu| |e| ou +u||r|c|o||+| ||e|+p,,
+dr|u||e|ed |o|| |op|c+||, +ud ,|er|c+||,,
,|er|c+||,, ||e |e||+c,c||ue eer |o Wo||
|e||e| ||+u ro|. \up||oc|u |op|c+||,. Iop|c+|
|ucoco|||co|d dep|e ||e +u|||u||+rr+|o|,
|epoue +ud +cce|e|+|e |e+||u. |u e.e|e
c+e, de|r+||+|ou o| c+||ou d|o\|de |+e|
.+po||/+||ou |e+d |o |e+||u W||| c+|, W||c| +|e
|e||+u| |o |ecu||euce. I|e coud|||ou |ecore
|e ||ou||eore W||| +e.
|C|9 . o9+.5
|C|0 . 0 32.3
FAMIIIAI 8NICN FMFhICS
FICk 6-8 Fam|a| beoo pemphus I|| +o,e+|o|d r+|e |+ |+d oo/|u |e|ou |u |o|| +\|||+e,
occ+|ou+||, |u ||e |o|u +ud ore||re +|o ou ||e u+pe o| ||e uec|, |o| e.e|+| ,e+|. E|up||ou Wo|eu du||u
||e urre| rou||. I|e |+||e| +ud ||e| |+.e |r||+| |e|ou ||+| W+\ +ud W+ue. |e|ou +|e p+|u|u| +ud |oW
|,p|c+| c|+c| +ud ||u|e W||||u +u e|o|.e e|,||er+|ou p|+que. A|||ou| c|+|||ed +rou ||e ||||e||u d|
e+e, |+r|||+| |eu|u perp||u |+|d|, e.e| |oW |u|+c| .e|c|e +ud | o||eu r||+|eu |o| |u|e||||o.
CIASSIFICAII0N
Two majoi types: pemphigus vulgaiis (PV)
and pemphigus foliaceus (PF). In addition,
paianeoplastic pemphigus (PP) associated with
malignancy and IgA pemphigus (Table 6-2).
FI0MI0I0C
PV: Raie, moie common in Jews and people of
Mediteiianean descent. In Jeiusalem the inci-
dence is estimated at 16 pei million, wheieas in
Fiance and Geimany it is 1.3 pei million.
PF: Also iaie but endemic in iuial aieas in
Biazil (fogo selvagem), wheie the pievalence
can be as high as 3.4%.
Ae oI 0oset 40 to 60 yeais; fogo selvagem
also in childien and young adults.
Sex Equal incidence in males and females, but
piedominance of females with PF in Tunisia
and Colombia.
An autoimmune disoidei. Loss of the noimal
cell-to-cell adhesion in the epideimis ( atan-
|o|yss ) occuis as a iesult of ciiculating anti-
bodies of the IgG class; these antibodies bind
to desmogleins, tiansmembiane glycopioteins
in the desmosomes, membeis of the cadheiin
supeifamily. In PV, desmoglein 3 (in some,
also desmoglein 1). All patients with PV have
autoantibodies to desmoglein 3. Those with
mucocutaneous PV also have antibodies to
desmoglein 1; those with only mucosal involve-
ment, only to desmoglein 3. In contiast, PF
patients have autoantibodies only to desmo-
glein 1. These autoantibodies inteifeie with
calcium-sensitive adhesion function and thus
induce acantholysis (Image 6-2).
A e||ou, +cu|e o| c||ou|c, |u||ou +u|o|rruue
d|e+e o| ||u +ud rucou rer||+ue |+ed ou
+c+u||o|,|.
IWo r+jo| |,pe. perp||u .u|+|| (|\) +ud
perp||u |o||+ceu (||)
|\. ||+cc|d ||||e| ou ||u +ud e|o|ou ou
rucou rer||+ue. ||. c+|, +ud c|u|ed ||u
|e|ou.
|\. up|+|++| +c+u||o|,|. ||. u|co|ue+| +c+u
||o|,|
|C +u|o+u|||od|e |o dero|e|u, ||+urer
||+ue deroor+| +d|e|ou ro|ecu|e.
'e||ou +ud o||eu |+|+| uu|e ||e+|ed W||| |r
ruuoupp|e|.e +eu|.
FMFhICS |C|9 . o9+.+
|C|0 . |0
PV usually staits in the oial mucosa, and months
may elapse befoie skin lesions occui; lesions
may be localized foi months, aftei which genei-
alized bullae occui. Less fiequently theie may be
a geneialized, acute eiuption of bullae fiom the
beginning. No piuiitus (as occuis in pemphig-
oid but buining and pain in eiosions oi eioded
bullae). Painful and tendei mouth lesions may
pievent adequate food intake. Epistaxis, hoaise-
ness, dysphagia. Weakness, malaise, weight loss.
PF has no mucosal lesion and staits with scaly,
ciusted lesion on an eiythematous base, initially
in seboiiheic aieas.
Sko Iesoos oI Fv Round oi oval vesicles
and bullae with seious content, flaccid (flabby)
(Fig. 6-9), easily iuptuied, and weeping (Fig.
6-10), aiising on norma| skin, iandomly
scatteied, disciete. Localized (e.g., to mouth oi
ciicumsciibed skin aiea), oi geneialized with a
iandom pattein. Extensive eiosions that bleed
easily (Fig. 6-11), ciusts paiticulaily on scalp.
IA8I 6-2 C|assification of Pemphius
|erp||u .u|+||
|erp||u .u|+||. |oc+||/ed +ud eue|+||/ed
|erp||u .ee|+u. |oc+||/ed
||u|uduced
|erp||u |o||+ceu
|erp||u |o||+ceu. eue|+||/ed
|erp||u e|,||er+|ou. |oc+||/ed
|oo e|.+er. euder|c
||u|uduced
|+|+ueop|+||c perp||u. +oc|+|ed W||| r+||u+uc,
|A perp||u. u|co|ue+| pu|u|+| de|r+|o| +ud
|u||+ep|de|r+| ueu||op||||c |A de|r+||||
SCII0N 6 Bu||0u' ||'EA'E' 10T
FICk 6-9 Femphus vu|ars I|| | ||e c|+|c |u|||+| |e|ou. ||+cc|d, e+||, |up|u|ed .e|c|e o| |u||+ ou
uo|r+|+ppe+||u ||u. kup|u|ed .e|c|e |e+d |o e|o|ou ||+| u|equeu||, c|u|.
IMAC 6-2 0esmo|eo compeosatoo I||+u|e |ep|eeu| ||e d|||||u||ou o| | +ud ! |u ||u +ud
rucou rer||+ue. Au||| +u|||od|e |u perp||u |o||+ceu c+ue +c+u||o|,| ou|, |u ||e upe|||c|+| ep|
de|r| o| ||u. |u ||e deep ep|de|r| +ud |u rucou rer||+ue, | ! corpeu+|e |o| +u|||od,|uduced |o
o| |uuc||ou o| | . |u e+||, perp||u .u|+||, +u|||od|e +|e p|eeu| ou|, ++|u| | !, W||c| c+ue ||||e|
ou|, |u ||e deep rucou rer||+ue W|e|e | ! | p|eeu| W|||ou| corpeu+|o|, | . noWe.e|, |u ruco
cu|+ueou perp||u, +u|||od|e ++|u| |o|| | +ud | ! +|e p|eeu|, +ud ||||e| |o|r |u |o|| rucou
rer||+ue +ud ||u. I|e ||||e| | deep p|o|+||, |ec+ue +u|||od|e d|||ue ||or ||e de|r| +ud |u|e||e|e ||||
W||| ||e |uuc||ou o| deroore +| ||e |+e o| ||e ep|de|r|. ||or l '|+u|e,, |u K wo||| e| +| (ed). ||cc|:|
|-c||, C--c| !-!:-, 1|| ed. |eW \o||, \cC|+Wn|||, 2003, p +o!.|
Since blisteis iuptuie so easily, only eiosions aie
seen in many patients. These aie veiy painful
(Fig. 6-11).
NI|v|s|y SIgn Dislodging of epideimis by
lateial fingei piessuie in the vicinity of lesions,
which leads to an eiosion. Piessuie on bulla
leads to lateial extension of blistei.
SItes v] PredI|ectIvn Scalp, face, chest, axillae,
gioin, umbilicus. In bediidden patients, theie is
extensive involvement of back (Fig. 6-11).
Mucous Membraoes Bullae iaiely seen, eio-
sions of mouth and nose, phaiynx and laiynx,
vagina.
Sko Iesoos oI FF Most commonly on face,
scalp, uppei chest, and abdomen. Scaly, ciusted
eiosions on an eiythematous base. In eaily
oi localized disease, shaiply demaicated in
seboiiheic aieas; may stay localized foi a long
time oi piogiess to geneialized disease and
exfoliative eiythiodeima. Initial lesion also
a flaccid bulla but this is iaiely seen because of
supeificial location (see histopathology below).
vAkIANIS (S IA8I 6-2)
PemphIgus Vegetuns (PVeg) Usually confined
to inteitiiginous iegions, peiioial aiea, neck,
and scalp. Gianulomatous vegetating puiulent
plaques that extend centiifugally. In these pa-
tients theie is a gianulomatous iesponse to the
autoimmune damage of PV.
Drug-1nduced PV Clinically identical to spoi-
adic PV. Seveial diffeient diugs implicated, most
significantly, captopiil and D-penicillamine.
BruzI|Iun PemphIgus (Fvgv Se|vugem) A
distinctive foim of PF endemic to south cen-
tial Biazil. Clinically, histologically, and immu-
nopathologically identical to PF. Patients
impiove when moved to uiban aieas but ielapse
aftei ietuining to endemic iegions. It is specu-
lated that the disease is somehow ielated to an
aithiopod-boine infectious agent, with clustei-
ing similai to that of the ||at| [|y-Sm|um
ntrmanum . Moie than 1000 new cases pei yeai
aie estimated to occui in the endemic iegions.
PemphIgus Erythemutvsus (PE) Synonym :
Seneai-Ushei syndiome. A localized vaii-
ety of PF laigely confined to seboiiheic sites.
Eiythematous, ciusted, and eiosive lesions in the
butteifly" aiea of the face, foiehead, and piest-
einal and inteiscapulai iegions. These patients
have immunoglobulin and complement depos-
its at the deimal-epideimal junction, in addi-
tion to inteicellulai pemphigus antibody in the
epideimis, and may have antinucleai antibodies,
as is the case in lupus eiythematosus.
Drug-1nduced PemphIgus PF As in PV, associ-
ated with D-penicillamine and less fiequently
by captopiil and othei diugs. In most, but not
all, instances the eiuption iesolves aftei teimi-
nation of theiapy with the offending diug.
FAkAN0FIASIIC FMFhICS (FNF)
This is a disease sui geneiis (see Section 18).
Mucous membianes piimaiily and most se-
veiely involved. Lesions combine featuies of
pemphigus vulgaiis and eiythema multifoime,
clinically and histologically.
0ermatopatho|oy PV: Light micioscopy (se-
lect eaily small bulla oi, if not piesent, maigin
of laigei bulla oi eiosion): Sepaiation of keiati-
nocytes, supiabasally, leading to split just a|oe
the basal cell layei and vesicles containing sepa-
iated, iounded-up (acantholytic) keiatinocytes.
PF: Supeificial foim with acantholysis in the
gianulai layei of the epideimis.
Immuoopatho|oy Diiect immunofluoies-
cence (IF) staining ieveals IgG and often C3
deposited in lesional and paialesional skin in
|e nerte||u|ar su|sante o[ |e eJerms .
Serum Autoantibodies (IgG) detected by indi-
iect IF (IIF) oi enzyme-linked immunosoibent
assay (ELISA). Titei usually coiielates with
activity of disease piocess. In PV autoantibod-
ies aie diiected against a 130-kDa glycopio-
tein designated desmoglein 3 and located in
desmosomes. In PF ciiculating autoantibodies
to a 160-kDa inteicellulai (cell suiface) antigen,
desmoglein 1, in the desmosomes of keiatino-
cytes. PV (130 kDa) and PF (160 kDa) antigens
diffei (Image 6-2). This explains the diffeient
sites of acantholysis and thus the diffeient clini-
cal appeaiance of the two conditions.
Can be a difficult pioblem if only mouth lesions
aie piesent. Aphthae, mucosal lichen planus,
eiythema multifoime. Diffeiential diagnosis
includes all foims of acquiied bullous diseases
(see Table 6-3). Biopsy of the skin and mucous
membiane, diiect IF, and demonstiation of
ciiculating autoantibodies confiim a high index
of suspicion.
C0kS
In most cases the disease inexoiably piogiess-
es to death unless tieated aggiessively with
SCII0N 6 Bu||0u' ||'EA'E' 109
FICk 6-10 Femphus vu|ars w|dep|e+d cou||ueu| ||+cc|d ||||e| ou ||e |oWe| |+c| o| + +0,e+|o|d
r+|e W|o |+d + eue|+||/ed e|up||ou |uc|ud|u c+|p +ud rucou rer||+ue. I|e e|oded |e|ou +|e e\||ere|,
p+|u|u|.
FICk 6-11 Femphus vu|ars w|dep|e+d cou||ueu| e|o|ou ||+| +|e .e|, p+|u|u| +ud ||eed e+||,
|u + 5!,e+|o|d r+|e. I|e|e +|e |+|d|, +u, |u|+c| ||||e| |ec+ue ||e, +|e o ||+||e +ud ||e+| e+||,. I|e ||ood
||+c| o |deW+, |ec+ue ||e p+||eu| |+d |eeu |,|u ou || |||| |de |e|o|e ||e p|o|o|+p| W+ |+|eu.
immunosuppiessive agents. The moitality iate
has been maikedly ieduced since tieatment has
become available. Cuiiently, moibidity ielated
to glucocoiticoids and immunosuppiesive
theiapies.
MANACMNI
C|ucocortcods 2 to 3 mg/kg body weight
of piednisone until cessation of new blistei
foimation and disappeaiance of Nikolsky sign.
Then iapid ieduction to about half the initial
dose until patient is almost cleai, followed by
veiy slow tapeiing of dose to minimal effective
maintenance dose.
Coocomtaot Immuoosuppressve Iherapy
Immunosuppiessive agents aie given concomi-
tantly foi theii glucocoiticoid-spaiing effect:
:a|orne , 2-3 mg/kg body weight until
complete cleaiing; tapeiing of dose to
1 mg/kg. Azathiopiine alone is contin-
ued even aftei cessation of glucocoiticoid
tieatment and may have to be continued
foi many months oi yeais.
Me|orexae , eithei oially (PO) oi IM at
doses of 25 to 35 mg/week. Dose adjust-
ments aie made as with azathiopiine.
Cyt|o|os|amJe , 100-200 mg daily, with
ieduction to maintenance doses of 50-
100 mg/d. Alteinatively, cyclophospha-
mide bolus" theiapy with 1000 mg IV
once a week oi eveiy 2 weeks in the
initial phases, followed by 50-100 mg/d
PO as maintenance.
P|asma|eress , in conjunction with glu-
cocoiticoids and immunosuppiessive
agents in pooily contiolled patients, in
the initial phases of tieatment to ieduce
antibody titeis.
Co|J |eray , foi mildei cases. Aftei an
initial test dose of 10 mg IM, 25-50 mg
of gold sodium thiomalate is given IM at
weekly inteivals to a maximum cumula-
tive dose of 1 g.
Myto|eno|ae mo[e| (1 g twice daily) has
been iepoited to be beneficial, and clini-
cal studies aie ongoing.
Hg|-Jose nraenous mmunog|o|u|n
(HIVIg) (2 g/kg body weight eveiy 3-4
weeks) has been iepoited to have a glu-
cocoiticoid-spaiing effect. Expensive.
Ruxma| (monoclonal antibody to CD20)
piesumably taigets B cells, the piecuisois
of (auto) antibody-pioducing plasma
cells. Given as intiavenous theiapy once a
week foi 4 weeks, shows diamatic effects
in some and at least paitial iemission in
othei patients. Seiious infections may
be seen.
0ther Measures Cleansing baths, wet diess-
ings, topical and intialesional glucocoiticoids,
antimiciobial theiapy pei documented bacte-
iial infections. Coiiection of fluid and electio-
lyte imbalance.
Mootoro Clinical, foi impiovement of
skin lesions and development of diug-ielated
side effects. Laboiatoiy monitoiing of pem-
phigus antibody titeis and foi hematologic
and metabolic indicatois of glucocoiticoid-
and/oi immunosuppiessive-induced adveise
effects.
SCII0N 6 Bu||0u' ||'EA'E' 111
IA8I 6-3 0ifferentia| 0ianosis of |mportant Acquired Bu||ous 0iseases
0sease Sko Iesoos Mucous Membraoes 0strbutoo
|\ ||+cc|d |u||+e ou uo|r+| A|ro| +|W+, |u.o|.ed, Au,W|e|e, |oc+||/ed o|
||u, e|o|ou e|o|ou eue|+||/ed
|| C|u|ed e|o|ou, occ+|ou+||, k+|e|, |u.o|.ed E\poed, e|o|||e|c
||+cc|d .e|c|e |e|ou o| eue|+||/ed
|\e C|+uu|+||u p|+que, A |u |\ |u|e|||||uou |e|ou, c+|p
occ+|ou+||, .e|c|e +|
r+||u
Bu||ou Ieue |u||+e ou uo|r+| \ou|| |u.o|.ed |u Au,W|e|e, |oc+||/ed o|
perp||o|d +ud e|,||er+|ou 0-!5 eue|+||/ed
||u, u|||c+||+| p|+que
+ud p+pu|e
EBA Ieue |u||+e +ud e|o|ou, \+, |e e.e|e|, I|+ur+||/ed |e|ou o|
uou|u||+rr+|o|, o| B|, |u.o|.ed (o|+| |+udor
|n o| |A||||e p|eeu|+||ou eop|+u, .+|u+)
|e|r+|||| C|ouped p+pu|e, .e|c|e, |oue ||ed||ec||ou ||e. e||oW,
|e|pe|||o|r| u|||c+||+| p|+que, c|u|ed |uee, |u|e+|, +c|+|, +ud
c+pu|+| +|e+
||ue+| |A Auuu|+|, |ouped p+pu|e, 0|+| e|o|ou +ud u|ce|, Au,W|e|e
de|r+|o| .e|c|e, +ud |u||+e coujuuc||.+| e|o|ou
+ud c+|||u
0sease hstopatho|oy Immuoopatho|oy[Sko Serum
|\ 'up|+|++| +c+u||o|,| |C |u|e|ce||u|+| p+||e|u |C AB |o |u|e|ce||u|+|
u||+uce o| ep|de|r| (|||)
E||'A. AB |o dero|e|u
! >> dero|e|u
|| Ac+u||o|,| |u |+uu|+| |C, |u||+ce||u|+| p+||e|u |C AB |o |u|e|ce||u|+|
|+,e| u||+uce o| ep|de|r| (|||)
E||'A. AB |o dero|e|u
ou|,
|\e Ac+u||o|,| |u||+ep|de|r+| A |u |\ A |u |\
ueu||op||||c +|cee,
ep|de|r+| |,pe|p|+|+
Bu||ou 'u|ep|de|r+| ||||e| |C +ud C! ||ue+| +| |C AB |o B\/ (|||),
perp||o|d B\/ d||ec|ed |o B|AC +ud
B|AC2
EBA 'u|ep|de|r+| ||||e| ||ue+| |C +| B\/ |C AB |o B\/ (|||) d||ec|ed
|o |,pe \|| co||+eu (E||'A,
we|e|u ||o|)
|e|r+|||| |+p|||+|, r|c|o+|cee, C|+uu|+| |A |u ||p o| Au||eudor,|+|
|e|pe|||o|r| u|ep|de|r+| .e|c|e p+p|||+e +u|||od|e
||ue+| |A 'u|ep|de|r+| ||||e| ||ue+| |A +| B\/ |oW |||e| o| |A AB
de|r+|o| W||| ueu||op||| ++|u| B\/
|o|e. AB, +u|||od,, B\/, |+ereu| rer||+ue /oue, B|, |u||ou perp||o|d, |n, de|r+|||| |e|pe|||o|r|, EB, ep|de|ro|,| |u||o+ +cqu|||+,
E||'A, eu/,re||u|ed |rruuoo||eu| ++,, |||, |ud||ec| |rruuo||uo|eceuce, |A|, ||ue+| |A de|r+|o|, ||, perp||u |o||+ceu, |\, perp||
u .u|+||, |\e, perp||u .ee|+u.
FI0MI0I0C
Ae oI 0oset 60 to 80 yeais.
Sex Equal incidence in males and females. No
known iacial piedilection.
Iocdeoce The most common bullous autoim-
mune disease. Seven pei million in Geimany
and Fiance. Fai moie common in authois`
expeiience in veiy old people.
Inteiaction of autoantibody with bullous pem-
phigoid antigen BPAG1 and BPAG2 (collagen
type XVII)] in hemidesmosomes of basal ke-
iatinocytes (Image 6-1) is followed by comple-
ment activation and attiaction of neutiophils
and eosinophils. Bullous lesion iesults fiom
inteiaction of multiple bioactive molecules ie-
leased fiom inflammatoiy cells. Not yet com-
pletely claiified.
Often staits with a piodiomal eiuption (ui-
ticaiial, papulai lesions) and evolves in weeks
to months to bullae that may appeai sud-
denly as a geneialized eiuption. Initially no
symptoms except modeiate oi seveie piuiitus;
latei, tendeiness of eioded lesions. No constitu-
tional symptoms, except in widespiead, seveie
disease.
Sko Iesoos Eiythematous, papulai oi
uiticaiial-type lesions (Fig. 6-12) may piecede
bullae foimation by months. Bullae: laige, tense,
fiim-topped, oval oi iound (Fig. 6-13); may
aiise in noimal, eiythematous, oi uiticaiial skin
and contain seious oi hemoiihagic fluid. The
eiuption may be localized oi geneialized, usually
scatteied but also giouped in aicifoim and
seipiginous patteins. Bullae iuptuie less easily
than in pemphigus, but sometimes laige, biight
ied, oozing, and bleeding eiosions become a
A |u||ou +u|o|rruue d|e+e uu+||, |u e|de||,
p+||eu|.
||u||||c p+pu|+| +ud/o| u|||c+||+| |e|ou W||| |+|e
|eue |u||+e.
'u|ep|de|r+| ||||e| W||| eo|uop|||.
C! +ud |C +| ep|de|r+| |+ereu| rer||+ue, +u||
|+ereu| rer||+ue |C +u|o+u|||od|e |u e|ur.
Au|o+u||eu +|e |e|+||uoc,|e |er|deroore
p|o|e|u.
I|e|+p, |uc|ude |op|c+| +ud ,|er|c |ucoco|||
co|d +ud o||e| |rruuoupp|e|.e.
8II0S FMFhIC0I0 (8F) |C|9 . o9+.5
|C|0 . |2.0
majoi pioblem. Usually, howevei, the oiiginally
tense bullae collapse and tiansfoim into ciusts.
SItes v] PredI|ectIvn Axillae; medial aspects of
thighs, gioins, abdomen; flexoi aspects of foie-
aims; lowei legs (often fiist manifestation).
Mucous Membraoes Piactically only in the
mouth (10-35%); less seveie and painful and
less easily iuptuied than in pemphigus.
0ermatopatho|oy LIght MIcrvscvpy Neu-
tiophils in Indian-file" alignment at deimal-
epideimal junction; neutiophils, eosinophils,
and lymphocytes in papillaiy deimis; su|e-
Jerma| bulla.
E|ectrvn MIcrvscvpy Junctional cleavage, i.e.,
split occuis in lamina lucida of basement mem-
biane.
Immuoopatho|oy Lineai IgG deposits along
the basement membiane zone. Also, C3, which
may occui in the absence of IgG.
Serum Ciiculating antibasement membiane
IgG antibodies detected by IIF in 70% of pa-
tients. Titeis do not coiielate with couise of
disease. Autoantibodies in bullous pemphigoid
iecognize two types of antigens. BPAG1 is a
230-kDa glycopiotein that has high homology
with desmoplakin I and is pait of hemidesmo-
somes. BPAG2 is a tiansmembianous 180-kDa
polypeptide (type XVII collagen).
hemato|oy Eosinophilia (not always).
Clinical appeaiance, histopathology, and immu-
nology peimit a diffeientiation fiom othei bul-
lous diseases (see Table 6-3 on p 111).
MANACMNI
Systemic piednisone with staiting doses of
50-100 mg/d continued until cleai, eithei alone
SCII0N 6 Bu||0u' ||'EA'E' 113
oi combined with azathiopiine, 150 mg daily,
foi iemission induction and 50-100 mg foi
maintenance; in iefiactoiy cases IVIG: plas-
mapheiesis in mildei cases, sulfones (dapsone),
100-150 mg/d. Low-dose MTX 2.5 to 10 mg
weekly PO is effective and safe in eldeily. In veiy
mild cases and foi local iecuiiences, topical glu-
cocoiticoid oi topical taciolimus theiapy may
be beneficial. Tetiacycline nicotinamide has
been iepoited to be effective in some cases.
C0kS AN0 Fk0CN0SIS
Patients often go into a peimanent iemission
aftei theiapy and do not iequiie fuithei theiapy;
local iecuiiences can sometimes be contiolled
with topical glucocoiticoids; clobetasol with
occlusion to uiticaiial aieas. Also, intialesional
tiiamcinolone foi localized disease. Some
cases go into spontaneous iemission without
theiapy.
FICk 6-12 8u||ous pemphod E+||, |e|ou |u + 15,e+|o|d |er+|e. |o|e u|||c+||+| p|+que +ud +
r+||, |eue ||||e| W||| + c|e+| e|ou cou|eu|.
FICk 6-13 8u||ous pemphod I|| 11,e+|o|d r+|e |+ + eue|+||/ed e|up||ou W||| cou||ueu| u|||
c+||+| p|+que +ud ru|||p|e |eue ||||e|. I|e coud|||ou | e.e|e|, p|u||||c.
A |+|e d|e+e, |+|e|, o| ||e e|de||,.
B|||e| ||+| |up|u|e e+||, +ud +|o e|o|ou
|eu|||u ||or ep|||e||+| ||+||||, |u ||e rou||,
o|op|+|,u\, +ud, ro|e |+|e|,, ||e u+op|+|,u
e+|, eop|+e+|, eu||+|, +ud |ec|+| ruco+e.
0cu|+| |u.o|.ereu| r+, |u|||+||, r+u||e| +
uu||+|e|+| o| |||+|e|+| coujuuc||.||| W||| |u|u|u,
d|,ue, +ud |o|e|u|od, eu+||ou.
C||ou|c |u.o|.ereu| |eu|| |u c+|||u, ,r
||ep|+|ou (||. o+), +ud, |u e.e|e d|e+e,
|u|ou o| ||e |u||+| +ud p+|pe||+| coujuuc||.+.
Eu||op|ou +ud |||c||+| |eu|| |u co|ue+| |||||+
||ou, upe|||c|+| puuc|+|e |e|+||uop+||,, co|ue+|
ueo.+cu|+||/+||ou, u|ce|+||ou, +ud |||udue.
'c+|||u +|o |u ||e |+|,u\, eop|+e+| |u.o|.e
reu| |eu|| |u |||c|u|e |o|r+||ou |e+d|u |o
d,p|+|+ o| d,uop|+|+.
I|e ||u | |u.o|.ed |u |ou||, !0 o| p+||eu|.
Au||eu |o W||c| +u|o+u|||od|e r+, |e d||ec|ed
|uc|ude B|AC2, |+r|u|u 5, |u|e||u u|uu||
+
+ud
o
, |,pe \|| co||+eu, +ud B|AC.
3||-, -|! dec|||e + u|e|
o| p+||eu| W|oe ||u |e|ou |ecu| +| ||e +re
||e, r+|u|, ou ||e |e+d +ud uec|, +ud +|o |e+d
|o c+|||u.
!cc--|. \o| p+||eu| |epoud |o d+poue
|u cor||u+||ou W||| |oWdoe p|edu|oue. 'ore
p+||eu| r+, |equ||e ro|e +|e|.e |rruuo
upp|e|.e ||e+|reu| W||| c,c|op|op|+r|de o|
+/+|||op||ue, |u cor||u+||ou W||| |ucoco|||co|d.
|u +dd|||ou, u||c+| |u|e|.eu||ou |o| c+|||u +ud
uppo|||.e re+u|e.
',,. \ucou rer||+ue perp||o|d.
CICAIkICIAI FMFhIC0I0 (CF) |C|9 . o9+.o
|C|0 . |2.
FICk 6-14 Ccatrca| pemphod I|| c+|||u coud|||ou |u + 13,e+|o|d |er+|e |+||ed W||| |||+|e|+|
coujuuc||.+| p+|u +ud |o|e|u |od, eu+||ou + ||e |||| ,rp|or. I|e coujuuc||.+ ||eu |ec+re e|o|.e W|||
c+|||u +ud ||||ou ||+c| |e|Weeu e,e||d +ud ||e e,e.
SCII0N 6 Bu||0u' ||'EA'E' 115
A |+|e p|u||||c +ud po|,ro|p||c |u||+rr+|o|, |u|
|ou de|r+|o| o| p|eu+uc, +ud ||e po|p+||ur
pe||od.
I|e e||r+|ed |uc|deuce | ||or |u 100 |o |u
0,000 de||.e||e.
E\||ere|, p|u||||c .e|cu|+| e|up||ou r+|u|, ou
||e +|doreu |u| +|o ou o||e| +|e+, W||| p+|
|u o| ||e rucou rer||+ue. |e|ou .+|, ||or
e|,||er+|ou, eder+|ou p+pu|e |o u|||c+||+|
p|+que |o .e|c|e +ud |eue |u||+e (||. o5).
|C uu+||, |e|u ||or ||e |ou||| |o ||e e.eu||
rou|| o| p|eu+uc, |u| c+u +|o occu| |u ||e
|||| |||re|e| +ud |u ||e |rred|+|e po|p+||ur
pe||od.
|| r+, |ecu| |u u|equeu| p|eu+uc|e, || || doe,
|| | |||e|, |o |e|u e+|||e|.
|C c+u |e e\+ce||+|ed |, ||e ue o| e||oeu
+ud p|oe|e|ouecou|+|u|u red|c+||ou.
n||op+||o|o|c+||, || | + u|ep|de|r+| ||||e||u
coud|||ou, +ud ||e|e | + |e+., ||ue+| depo|||ou
o| C! +|ou ||e |+ereu| rer||+ue /oue W|||
coucor||+u| |C depo|||ou |u |ou||, !0 o|
p+||eu|.
'e|ur cou|+|u |C +u|||++| rer||+ue +u|||od
|e, |u| ||ee +|e de|ec|ed |u ou|, 20 o| p+||eu|
|, |||. E||'A +ud |rruuo||o|||u ++, de|ec|
+u|o+u|||od|e |u >10, d||ec|ed |o B|30 (|,pe
/\|| co||+eu), + 30||+ ||+urer||+ue p|o|e|u
|u |er|deroore. I|e, +|e +.|d corp|ereu|
||\|u |C +u|||od|e ||+| ||ud |o +ru|o||c
ep|||e||+| |+ereu| rer||+ue. I|e, c+u +|o |e
de|ec|ed |u ||e ||ood o| ore |u|+u|.
'ore 5 o| |+||e |o|u |o ro||e| W||| |C
|+.e u|||c+||+|, .e|cu|+|, o| |u||ou |e|ou, W||c|
|eo|.e pou|+ueou|, du||u ||e |||| e.e|+|
Wee|. |o |u|||c+u| r+|e|u+| ro|||d||, (p|u
|||u) +ud ro||+|||,. I|e|e | + |||| |uc|e+e |u
p|er+|u|e +ud r+|||o|e|+||ou+|+e |||||.
'ore |epo|| o| |e|+| p|ouo| |+.e |e.e+|ed
|u|||c+u| |e|+| de+|| +ud p|er+|u|e de||.e||e,
W|e|e+ o||e| |+.e ue|ed uo |uc|e+e |u
|e|+| ro||+|||,.
!cc--| | e+|ed |o upp|e|u ||||e|
|o|r+||ou +ud |e||e.|u ||e |u|eue p|u|||u.
||edu|oue, 20-+0 r/d, | |.eu |u| ore||re
|||e| doe +|e |equ||ed. ||edu|oue | |+pe|ed
|+du+||, du||u ||e po|p+||ur pe||od. 0u|, +
|eW p+||eu| do uo| |equ||e ,|er|c p|edu|oue
+ud c+u |e r+u+ed W||| +u|||||+r|ue +ud
|op|c+| |ucoco|||co|d.
FMFhIC0I0 CSIAII0NIS (FC) |C|9 . o+o.3
|C|0 . |2.3
FICk 6-15 Femphod
estatoos E|,||er+|ou
p+pu|e ||+| We|e ||||, p|u||||c
+ud |+d +ppe+|ed ou ||e ||uu|
+ud +|doreu o| ||| !!,e+|o|d
p|eu+u| |er+|e (||||d |||re|e|)
+ud We|e + c+ue o| |e+| cou
ce|u. A| ||| ||re ||e|e We|e uo
||||e| +ud d|+uo| W+ e|+|
|||ed |, ||op, +ud
|rruuop+||o|o,.
A c||ou|c, |ecu||eu|, |u|eue|, p|u||||c e|up||ou
occu|||u ,rre|||c+||, ou ||e e\||er|||e +ud
||e ||uu|.
Cou|| o| ||u, .e|c|e, p+pu|e, +ud u|||c+||+|
p|+que ||+| +|e +||+ued |u |oup.
Aoc|+|ed W||| |u|eueu|||.e eu|e|op+||,
(C'E).
C|+|+c|e||/ed |||o|o|c+||, |, p+p|||+|, co||ec||ou
o| ueu||op|||.
C|+uu|+| |A depo|| |u p+|+|e|ou+| o| uo|r+|
||u +|e d|+uo||c.
kepoud |o u||+ d|u +ud, |o + |ee| e\|eu|, |o
+ |u|eu||ee d|e|.
0kMAIIIIS hkFIIF0kMIS (0h) |C|9 . o9+.0
|C|0 . |!.0
FI0MI0I0C
Pievalence in Caucasians vaiies fiom 10 to 39
pei 100,000 peisons.
Ae oI 0oset 20 to 60 yeais, but most com-
mon at 30 to 40 yeais; may occui in childien.
Sex Male:female iatio is 2:1.
The GSE piobably ielates to IgA deposits in the
skin. Patients have antibodies to tiansglutami-
nases (TGs) that may be the majoi autoantigens
in this disease. Epideimal TG autoantibody
piobably binds to TG in the gut and ciiculates
eithei alone oi as immune complexes and
deposits in skin. With additional factois IgA
activates complement via the alteinative path-
way, with subsequent chemotaxis of neutiophils
ieleasing theii enzymes and pioducing tissue
injuiy.
Piuiitus, intense, episodic; buining oi sting-
ing of the skin; iaiely, piuiitus may be absent.
FICk 6-15 (Cou||uued)
u|||c+||+| p|+que +ud r+|| .ec|c|e +ud ||||e| |u +uo||e| p+||eu| W|o |+d |r||+| e|up||ou |u p|e.|ou
p|eu+uc|e. '|e |epouded |+p|d|, |o ,|er|c |ucoco|||co|d. I|e de||.e|, W+ uue.eu||u|, +ud ||e |+|, W+
|e+|||,.
SCII0N 6 Bu||0u' ||'EA'E' 11T
Symptoms often piecede the appeaiance of skin
lesions by 8 to 12 h. Ingestion of iodides and
oveiload of gluten aie exaceibating factois.
Systems kevew Laboiatoiy evidence of small-
bowel malabsoiption is detected in 10-20%.
GSE occuis in neaily all patients and is demon-
stiated by small-bowel biopsy. Theie aie usually
no systemic symptoms.
Sko Iesoos Lesions consist of eiythematous
papules oi wheal-like plaques; tiny fiim-topped
vesicles, sometimes hemoiihagic (Fig. 6-16);
occasionally bullae. Lesions aie aiianged in
gioups (hence the name |erer[orms ); the
distiibution is stiikingly symmetiic. Sciatching
iesults in excoiiations, ciusts (Fig. 6-17).
Postinflammatoiy hypei-and hypopigmentation
at sites of healed lesions.
SItes v] PredI|ectIvn Typical and almost diag-
nostic: extensoi aieas-elbows, knees. Buttocks,
scapulai and sacial aieas (Image 6-3 and Figs.
6-16 and 6-17). Heie, often in a butteifly"
fashion. Scalp, face, and haiiline.
Immuooeoetcs Association with HLA-B8,
HLA-DR, and HLA-DQ.
0ermatopatho|oy Biopsy is best fiom eaily
eiythematous papule. Micioabscesses (poly-
moiphonucleai cells and eosinophils) at the
tips of the deimal papillae. Deimal infiltiation
of neutiophils and eosinophils. Su|eJerma|
est|e .
ImmuooI|uoresceoce Of er|esona| skin, best
on the buttocks. Gianulai IgA deposits in tips
of papillae that coiielate well with small-bowel
disease. Gianulai IgA is found in almost-noimal
skin in most patients and is diagnostic. Also
found aie C3 and C5and alteinative comple-
ment pathway components.
Crcu|ato Autoaotbodes Antiieticulin anti-
bodies of the IgA and IgG types, thyioid antimi-
ciosomal antibodies, and antinucleai antibodies
can be piesent. Putative immune complexes in
20-40% of patients. IgA antibodies binding to
the inteimyofibiil substance of smooth muscles
(anenJomysa| an|oJes ) aie piesent in most
patients and have specificity foi TGs.
0ther Studes Steatoiihea (20-30%) and ab-
noimal D-xylose absoiption (10-73%). Anemia
secondaiy to iion oi folate deficiency. EnJos-
toy o[ sma|| |owe| : blunting and flattening
of the villi (80-90%) in the small bowel as in
celiac disease. Lesions aie focal; veiification is
by small-bowel biopsy.
Giouped papulovesicles at piedilection sites
accompanied by seveie piuiitus aie highly sug-
gestive. Biopsy of eaily lesions usually diagnostic,
but IgA deposits in peiilesional skin detected by
IF aie the best confiiming evidence. Diffeiential
FICk 6-16 0ermatts herpetIorms I|ee +|e ||e c|+|c e+||, |e|ou. |+pu|e, u|||c+||+| p|+que, r+||
|ouped .e|c|e, +ud c|u| ou ||e e||oW o| + 2!,e+|o|d r+|e.
diagnosis is to alleigic contact deimatitis, atopic
deimatitis, scabies, neuiotic excoiiations, papu-
lai uiticaiia, bullous pemphigoid, pemphigoid
gestationis (see Table 6-3).
C0kS
Piolonged, foi many yeais, with
a thiid of the patients eventually
having a spontaneous iemission.
MANACMNI
Systemc Iherapy Dupsvne
100-150 mg daily, with giadual
ieduction to 50-25 mg and often
as low as 50 mg twice a week.
Theie is a diamatic iesponse, often
within houis. Obtain a glucose-
6-phosphate dehydiogenase level
befoie staiting sulfones; obtain
methemoglobin levels in the initial
2 weeks, and follow blood counts
caiefully foi the fiist few months.
IMAC 6-3 |e|r+|||| |e|pe|||o|r|.
|+||e|u o| d|||||u||ou.
Su|]upyrIdIne 1-1.5 g/d, with plenty of fluids,
if dapsone contiaindicated oi not toleiated.
Monitoi foi casts in uiine and kidney function.
0et A gluten-fiee diet may suppiess the
disease oi allow ieduction of the dosage of dap-
sone oi sulfapyiidine, but iesponse is veiy slow.
FICk 6-1T 0ermatts herpetIorms
|u ||| 5o,e+|o|d r+|e p+||eu| W||| +
eue|+||/ed ||||, p|u||||c e|up||ou, ||e
d|+uo| c+u |e r+de upou |||| ||| |,
||e d|||||u||ou o| ||e |e|ou. \o| |e+.
||, |u.o|.ed +|e ||e e||oW, ||e c+pu|+|,
+c|+|, +ud |u|e+| +|e+, +ud (uo| eeu
|u ||| p|c|u|e) ||e |uee. upou c|oe
|upec||ou ||e|e +|e |ouped p+pu|e,
r+|| .e|c|e, c|u|, +ud e|o|ou ou +u
e|,||er+|ou |+e +ud ||e|e | po||u||+r
r+|o|, |,po +ud |,pe|p|reu|+||ou.
Bec+ue o| p|u|||u ||e p+||eu| |+d p|e.|
ou|, |eeu d|+uoed + |+.|u +|op|c
de|r+||||, c+||e, +ud +||e||c cou|+c|
de|r+|||| +ud |+d |epouded ou|, poo||,
|o |op|c+| |ucoco|||co|d. I|| p+|||cu|+|
e|up||ou occu||ed +||e| |e |+d peu| +
.+c+||ou ou ||e |+|r+||ou co+| (|+.
|u |eeu |o|d ||+| uu|+|||u Wou|d |e
ood |o| || coud|||ou) W|e|e || re+|
cou||ed o| e+|ood (|od|de) +ud W|||e
||e+d (|u|eu).
SCII0N 6 Bu||0u' ||'EA'E' 119
A |+|e, |rruuered|+|ed, u|ep|de|r+| ||||e|
|u ||u d|e+e de||ued |, ||e p|eeuce o| |oro
eueou ||ue+| depo|| o| |A +| ||e cu|+ueou
|+ereu| rer||+ue /oue (|r+e o).
|| | c|e+||, ep+|+|e ||or de|r+|||| |e|pe|||o|r|
(|n) ou ||e |+| o| |rruuop+||o|o,, |rruuo
eue||c, +ud |+c| o| +oc|+||ou W||| C'E.
|A| ro| o||eu occu| +||e| pu|e||,.
C||u|c+| r+u||e|+||ou +|e .e|, |r||+| |o ||oe o|
|n, |u| ||e|e | ro|e ||||e||u. |+||eu| p|eeu|
W||| cor||u+||ou o| +uuu|+| o| |ouped p+pu|e,
.e|c|e, +ud |u||+e (||. o3) ||+| +|e d|||||u|ed
,rre|||c+||, ou ||uu| +ud e\||er|||e |uc|ud|u
e||oW, |uee, +ud |u||oc|. I|e |e|ou +|e .e|,
p|u||||c |u| |e e.e|e ||+u ||oe o| |n.
\uco+| |u.o|.ereu| | |rpo||+u| +ud |+ue
||or |+|e +,rp|or+||c o|+| e|o|ou +ud u|ce|
+||ou |o e.e|e o|+| d|e+e +|oue, o| e.e|e eu
e|+||/ed cu|+ueou |u.o|.ereu| +ud o|+| d|e+e
|r||+| |o ||+| |u c|c+|||c|+| perp||o|d.
|| | |deu||c+| W||| c||ou|c |u||ou d|e+e o| c|||d
|ood (CB|C), W||c| | + |+|e ||||e||u d|e+e
||+| occu| p|edor|u+u||, |u c|||d|eu 5 ,e+|
(||. o9).
C||cu|+||u +u|o+u|||od|e ++|u| .+||ou ep|de|
r+| |+ereu| rer||+ue +u||eu |+.e |eeu
|ouud.
|A| |+ |eeu +oc|+|ed W||| d|u. .+ucor,
c|u, |||||ur, p|eu,|o|u, u||+re||o\+/o|e/|||r
e||op||r, |u|oer|de, c+p|op|||, d|c|o|eu+c, +ud
o||e|.
I|e|e | + r+|| ||| o| |,rp|o|d r+||u+uc|e,
+ud +oc|+|ed u|ce|+||.e co|||| |+ |eeu |e
po||ed.
!cc--|. |+||eu| |epoud |o d+poue o|
u||+p,||d|ue |u| |u +dd|||ou, ro| r+, |equ||e
|oWdoe p|edu|oue. |+||eu| do uo| |epoud |o
+ |u|eu||ee d|e|.
IINAk IA 0kMAI0SIS (IA0) |C|9 . 102.3
FICk 6-18 Ioear IA dermatoss I|e|e +|e
ru|||p|e |ouped, cou||ueu| .e|c|e, |u||+e, +ud c|u|
ou +u u|||c+||+| +ud e|,||er+|ou |+e. I|e|e We|e
|r||+| |e|ou ou ||e ||uu| +ud ||e uppe| e\||er|||e.
FICk 6-19 Ioear IA dermatoss (chrooc,
bu||ous dsease oI ch|dhood) E\|eu|.e ||||e|
|u ou ||e uppe| e\||er|||e +ud ||uu| |u + 1,e+|o|d
c|||d. |o|e. ||||e| +|e |o|| |eue +ud ||+cc|d. I|e,
+|e |ouped +ud ||e|e | uo uo|+||e |u||+rr+||ou.
A c||ou|c u|ep|de|r+| |u||ou d|e+e +oc|
+|ed W||| +u|o|rruu||, |o ||e |,pe \|| co||+eu
W||||u ||e +uc|o||u |||||| |u ||e |+ereu|
rer||+ue /oue.
|ou| |,pe. ||e :|c: -:|c||| --
|c| | + uou|u||+rr+|o|,, ||||e||u e|up||ou
W||| +c|+| d|||||u||ou ||+| |e+| W||| c+|||u +ud
r|||+ |o|r+||ou. || | + rec|+uo|u||ou d|e+e
r+||ed |, ||u ||+||||,, +ud p+||eu| |+.e |eue
||||e| W||||u uou|u||+red ||u, e|o|ou, +ud
c+| |u ||+ur+||/ed |e|ou uc| + ||e do|+
o| ||e |+ud, |uuc||e, e||oW, |uee, +c|+|
+|e+, +ud |oe. I|| p|eeu|+||ou ||u |eer||e
po|p|,||+ cu|+ue+ |+|d+ (ee 'ec||ou 0) o| |e
|ed||+|, ep|de|ro|,| |u||o+.
I|e ||| -|!-||- --|c| | +
W|dep|e+d |u||+rr+|o|, .e|cu|o|u||ou e|up
||ou W|e|e e|,||er+|ou o| e.eu u|||c+||+| ||u
|e|ou +|e +oc|+|ed W||| |eue |u||+e |u.o|.|u
||e ||uu|, ceu||+| |od,, +ud ||u |o|d |u +dd|||ou
|o ||e e\||er|||e (||. o20).
C:c|:c| -|!-||- --|c| |+
p|or|ueu| ruco+| |u.o|.ereu|-e|o|ou +ud
c+|||u |u ||e rou||, eop|+u, coujuuc||.+,
+uu, +ud .+|u+.
I|e |1 ||| !-c|-||- --|c|
|oW .e|c|e +||+ued |u +u +uuu|+| |+||ou ||+|
+|e |er|u|ceu| o| ||ue+| |A |u||ou de|r+|o|,
|n, o| CB|C.
n||op+||o|o, o| |e|ou+| ||u. u|ep|de|r+|
||||e| W||| + c|e+u ep+|+||ou |e|Weeu ||e ep|
de|r| +ud de|r|.
|rruuop+||o|o, |e.e+| ||ue+| |C (p|u |A,
|\, |+c|o| B, +ud p|ope|d|u) +| ||e de|r+|
ep|de|r+| juuc||ou. || +|| p|||||u ||| | pe|
|o|red, c||cu|+||u +u|||+ereu| rer||+ue /oue
+u|||od|e ||ud ||e ||oo| o| ||e ||||e|, |u cou||+|
|o |u||ou perp||o|d W|e|e +u|||od|e +|e
|ouud |o ||e |oo|.
Au|||od|e |u EBA e|+ W||| ||ud |o + 290||+
|+ud |u we|e|u ||o| cou|+|u|u |,pe \|| co|
|+eu. Au E||'A ||+| | .e|, pec|||c |o| +u|||od|e
|o |,pe \|| co||+eu | uoW +.+||+||e.
-c|-| o| EBA | d||||cu||, p+|||cu|+||, |u p+
||eu| W||| ||e c|+|c rec|+uo|u||ou p|eeu
|+||ou. |+||eu| +|e |e||+c|o|, |o ||| doe o|
,|er|c |ucoco|||co|d, +/+|||op||ue, re||o||e\
+|e, +ud c,c|op|op|+r|de, W||c| +|e oreW|+|
|e|p|u| |u ||e |u||+rr+|o|, B||||e |o|r o| ||e
d|e+e. 'ore EBA p+||eu| |rp|o.e ou d+poue
+ud ||| doe o| co|c||c|ue. 'uppo|||.e ||e|+p,
| W+||+u|ed |u +|| p+||eu| W||| EBA.
|C|9 . o9+.3
|C|0 . |2.!
FI0kM0ISIS 8II0SA ACISIIA (8A)
SCII0N 6 Bu||0u' ||'EA'E' 121
FICk 6-20 pdermo|yss bu||osa acqusta I|| | + ||e |u||ou perp||o|d|||e p|eeu|+||ou W|||
|eue |u||+e, e|o|ou, +ud c|u| ou +u e|,||er+|ou |+e. I|e|e | e\|eu|.e po||u||+rr+|o|, p|reu|+||ou
due |o p|e.|ou ||||e||u.
122
S E C I | 0 N 1
MISCIIAN0S
INFIAMMAI0k 0IS0k0kS
|||,||+| |oe+ (|k) | +u +cu|e e\+u||er+|ou
e|up||ou W||| + d|||uc||.e ro|p|o|o, +ud o||eu
W||| + c|+|+c|e||||c e||||r||ed cou|e.
|u|||+||,, + |u|e (p||r+|,, o| '|e|+|d) p|+que |e
|ou de.e|op, uu+||, ou ||e ||uu|, o| 2 Wee|
|+|e| + eue|+||/ed ecoud+|, e|up||ou de.e|op
|u + |,p|c+| d|||||u||ou p+||e|u.
I|e eu|||e p|oce |er|| pou|+ueou|, |u o
Wee|.
ke+c||.+||ou o| |ur+u |e|pe.||u (nn\) 1 +ud
nn\o | ||e ro| p|o|+||e c+ue.
FIIkIASIS k0SA (Fk) |C|9. o9o.+
|C|0. |+2
Ae oI 0oset 10-43 yeais, but can occui iaiely
in infants and old peisons.
Seasoo Spiing and fall.
to|oy Theie is good evidence that PR is as-
sociated with ieactivation of HHV-7 oi HHV-6,
two closely ielated -heipesviiuses.
0uratoo oI Iesoos A single heiald patch pie-
cedes the exanthematous phase; which develops
ovei a peiiod of 1-2 weeks. Piuiitus-absent
(25%), mild (50%), oi seveie (25%).
Sko Iesoos Heru|d Putch 80% of patients.
Oval, slightly iaised plaque oi patch 2-5 cm,
salmon-ied, fine collaiette scale at peiipheiy;
may be multiple (Fig. 7-1B).
Erunthem Fine scaling papules and plaques
with maiginal collaiette (Fig. 7-1). Dull pink
oi tawny. Oval, scatteied, with chaiacteiistic
distiibution with the long axes of the oval
lesions following the lines of cleavage in a
Chiistmas tiee" pattein (Image 7-1). Lesions
usually confined to tiunk and pioximal aspects
of the aims and legs. Raiely on face.
AtypIcu| PItyrIusIs Rvseu Lesions may be
piesent only on the face and neck. The piimaiy
plaque may be absent, may be the sole manifes-
tation of the disease, oi may be multiple. Most
confusing aie the examples of pityiiasis iosea
with vesicles oi simulating eiythema multi-
foime. This usually iesults fiom iiiitation and
sweating, often as a consequence of inadequate
tieatment ( yrass rosea rraa ).
Mu|tp|e Sma|| Sca|o F|aques Drug eruons
(e.g., captopiil, baibituiates); setonJary sy||s
(obtain seiology); guae sorass (no maiginal
collaiette); sma|| |aque arasorass ; ery|ema
mgrans with secondaiy lesions; ery|ema mu|-
[orme, nea torors .
0ermatopatho|oy Patchy oi diffuse paiakeia-
tosis, absence of gianulai layei, slight acanthosis,
focal spongiosis, micioscopic vesicles. Occa-
sional dyskeiatotic cells with an eosinophilic
homogeneous appeaiance. Edema of deimis,
homogenization of the collagen. Peiivasculai
infiltiate mononucleai cells.
SCII0N T \|'CE||A|E0u' ||||A\\AI0k\ ||'0k|Ek' 123
C0kS
Spontaneous iemission in 6-12 weeks oi less.
Recuiiences aie uncommon.
MANACMNI
Symptomatc Oial antihistamines and/oi top-
ical antipiuiitic lotions foi ielief of piuiitus.
Topical glucocoiticoids. May be impioved by
UVB phototheiapy oi natuial sunlight expo-
suie if tieatment is begun in the fiist week of
eiuption. Shoit couise of systemic glucocoi-
ticoids.
FICk T-1 Ftyrass rosea 0.e|.|eW o| e\+u||er o| p||,||+| |oe+ W||| ||e |e|+|d p+|c| |oWu |u
B. I|e|e +|e p+pu|e +ud r+|| p|+que W||| o.+| cou||u|+||ou ||+| |o||oW ||e ||ue o| c|e+.+e. I|e ||ue c+||u
o| ||e +|rou|ed p+pu|e c+uuo| |e eeu +| ||| r+u|||c+||ou, W|||e ||e co||+|e||e o| ||e |e|+|d p+|c| | qu||e
o|.|ou ne|+|d p+|c|. Au e|,||er+|ou (+|rou|ed) p|+que W||| + co||+|e||e c+|e ou ||e ||+|||u ede o| ||e
+d.+uc|u |o|de|. Co||+|e||e re+u ||+| c+|e | +||+c|ed +| pe||p|e|, +ud |ooe |oW+|d ||e ceu|e| o| ||e |e|ou.

SMAII-FIA FAkAFS0kIASIS
(0ICIIAI 0kMAI0SIS), SFF
|C|9 . o9o.2
|C|0 . |+.!
Giadual development ovei months. Raie piuii-
tus. Middle age.
Sko Iesoos Round, oval, eiythematous,
yellowish oi fawn-coloied, only minimally
elevated patches, 5 cm in diametei (Fig. 7-2B).
k+|e e|up||ou W||| Wo||dW|de occu||euce.
IWo |,pe +|e |ecou|/ed. r+||p|+que || +ud
|+|ep|+que ||.
|e|ou +|e ou|, |||||, |u|||||+|ed, ,e||oW|| o|
|+Wuco|o|ed p+|c|e. |u r+||p|+que || ('||)
|e|ou +|e r+|| (5 cr) |ouud |o o.+| o| ||ue+|
ro||, ou ||e ||uu|. |+|ep|+que || (|||) +|o
o.+| o| |||eu|+||, |+ped +ud >5 cr. \+, |e
po||||ode|r+|ou.
'|| doe uo| p|o|e |o r,co| |uuo|de
(\|). |||, |, cou||+|, e\|| ou + cou||uuur W|||
p+|c||+e \| +ud c+u p|o|e |o o.e|| \|.
I|e+|reu| cou|| o| |op|c+| |ucoco|||co|d, p|o
|o||e|+p,, o| p|o|oc|ero||e|+p, (|u\A).
FAkAFS0kIASIS N FIAS (FF)
IMAC T-1 Ftyrass rosea: ||||||u||ou 'C||||r+ ||ee p+||e|u ou ||e |+c|
Slight scale and wiinkled suiface with cigaiette-
papei appeaiance. Lineai fingei-like (digitate)
shapes on tiunk, pioximal extiemities, and
buttocks, following lines of cleavage, giving
appeaiance of a hug that left fingeipiints (hence,
Jga| Jermaoss ) (Fig. 7-2).
Pityiiasis iosea, laige-plaque paiapsoiiasis, diug
eiuptions, nummulai eczema, tinea coipoiis,
mycosis fungoides.
FICk T-2 0tate dermatoss (sma||-p|aque parapsorass) I|e |e|ou +|e +,rp|or+||c, ,e|
|oW|| o| |+Wuco|o|ed, .e|, |||u, We||de||ued, |||||, c+|, p+|c|e. I|e, +|e o.+| +ud |o||oW ||e ||ue o| c|e+.
+e o| ||e ||u, |.|u ||e +ppe+|+uce o| + '|u ||+| |e|| ||ue|p||u| ou ||e ||uu|. I|e |ou +\| o| ||ee |e|ou
o||eu |e+c|e ro|e ||+u 5 cr. C|oe up o| r+||e| |e|ou |oW|u W||u|||u o| u||+ce.

0ermatopatho|oy Spongifoim deimatitis
with focal aieas of hypeikeiatosis, paiakeia-
tosis, and exocytosis. In the deimis theie aie
a mild supeificial vasculai lymphohistiocytic
infiltiate (piedominantly CD4- T cells) and
deimal edema.
MANACMNI
No tieatment necessaiy, but patients should
be ieassuied. Disease may be tieated with lu-
biicant oi topical steioids. Bioad-band photo-
theiapy; UVB (311 nm) and PUVA aie highly
effective.
IAkC-FIA FAkAFS0kIASIS (IFF)
|C|9 . o92.2
|C|0 . |+.+
Giadual development ovei months and yeais,
staiting with one oi two plaques. Piuiitus is
iaie; the lesions may disappeai aftei exposuie
to sun in the summei to iecui in the fall and
wintei. Middle age.
Sko Iesoos Baiely elevated, eiythematous,
dusky-ied, sometimes yellowish plaques
that aie actually patches (Fig. 7-3 ), with oi
without slight atiophy and smooth oi slightly
scaling suiface (Fig. 7-3 B ). Ciiculai, >10 cm
in diametei, oi iiiegulai and well defined; and
iandomly scatteied on tiunk, buttocks, bieasts,
oi extiemities.
Sca|o F|aques Eaily" stages of mycosis fun-
goides. The development of n[|raon in the
lesions, aro|y , and o||oJermaous t|anges
aie clues to eaily mycosis fungoides.
0ermatopatho|oy Nonspecific oi, latei, a
bandlike mononucleai cell infiltiate (CD4-)
with atiophy of the epideimis, vacuolization of
the basal cell layei, capillaiy dilatation. Theie
aie no atypical lymphocytes. Mild exocyto-
sis. Piedominance of CD4- T cells, fiequent
CD7 antigen deficiency, and, in the epideimis,
expiession of class II HLA antigens.
Ferphera| 8|ood Monoclonal T helpei cells
with skin-homing specificity can be detected.
C0kS AN0 Fk0CN0SIS
The lesions peisist foi life and can piogiess to
mycosis fungoides (see Section 20).
MANACMNI
Iopca| Tempoiaiy iemission with topical
glucocoiticoids.
Fhototherapy Good iesponses to naiiow-band
311-nm UVB oi PUVA photochemotheiapy.
FICk T-3 Iare-p|aque parapsorass (parapsorass eo p|aques) I|e |e|ou +|e +,rp|or+||c,
We||de||ued, |ouuded, |||||, c+|,, |||u p|+que o| p+|c|e. I|e |e|ou c+u |e |+|e| ||+u 0 cr +ud +|e ||||
|ed||oWu o| +|roup|u|. I|e|e r+, |e +||op|, |u ore +|e+. I|e |e|ou |e|e +|e |oc+|ed ou ||e e\||er|||e
|u| ||e, +|e ro|e corrou|, uo|ed ou ||e ||uu|. I|ee |e|ou ru| |e c+|e|u||, |o||oWed +ud |epe+|ed ||op|e
+|e uece+|, |o de|ec| r,co| |uuo|de. I|| eu|||, r+, |e cou|de|ed + + p|e|+e o| r,co| |uuo|de.
C|oe up o| |e|ou |oW|u r|u|r+| c+||u +ud W||u||ed u||+ce.
SCII0N T \|'CE||A|E0u' ||||A\\AI0k\ ||'0k|Ek' 12T

Ae oI 0oset 30-60 yeais.
Sex Females > males.
kace Hypeitiophic LP moie common in
blacks.
to|oy Idiopathic in most cases but cell-
mediated immunity plays a majoi iole. Majoi-
ity of lymphocytes in the infiltiate aie CD8-
and CD45Ro- (memoiy) cells. Diugs, metals
(gold, meicuiy), oi infection hepatitis C viius
(HCV)] iesult in alteiation in cell-mediated im-
munity. Theie could be HLA-associated genetic
susceptibility that would explain a piedisposi-
tion in ceitain peisons. Lichenoid lesions of
chionic giaft-veisus-host disease (GVHD) of
skin aie indistinguishable fiom those of LP (see
Section 21).
0oset Acute (days) oi insidious (ovei weeks).
Lesions last months to yeais, asymptomatic oi
piuiitic; sometimes seveie piuiitus. Mucous
membiane lesions aie painful, especially when
ulceiated.
Sko Iesoos Papules, flat-topped, 1 to 10 mm,
shaiply defined, shiny (Fig. 7-4). Violaceous, with
white lines (Wickham stiiae) (Fig. 7-4 ), seen
best with hand lens aftei application of mineial
oil. Polygonal oi oval (Fig. 7-4 B ). Giouped
(Figs. 7-4 and 7-5), annulai, oi disseminated
scatteied disciete lesions when geneialized
(Fig. 7-6). In daik-skinned individuals,
postinflammatoiy hypeipigmentation is
common. May piesent on lips (Fig. 7-7 ) and
in a lineai aiiangement aftei tiauma (Koebnei
wo||dW|de occu||euce, |uc|deuce |e ||+u oue
pe|ceu|, +|| |+ce.
|| | +u +cu|e o| c||ou|c |u||+rr+|o|, de|r+|o|
|u.o|.|u ||u +ud/o| rucou rer||+ue.
C|+|+c|e||/ed |, ||+||opped (|+||u |c , '||+|),
p|u| |o .|o|+ceou, ||u,, p|u||||c po|,ou+| p+
pu|e. I|e |e+|u|e o| ||e |e|ou |+.e |eeu de
|u+|ed + ||e |ou| |'-p+pu|e, pu|p|e, po|,ou+|,
p|u||||c.
||||||u||ou. p|ed||ec||ou |o| ||e\u|+| +pec| o|
+|r +ud |e, c+u |ecore eue|+||/ed.
|u ||e rou|| r|||,W|||e |e||cu|+|ed p+pu|e, r+,
|ecore e|o|.e +ud e.eu u|ce|+|e.
\+|u ,rp|or. p|u|||u, |u ||e rou||, p+|u.
I|e|+p,. |op|c+| +ud ,|er|c |ucoco|||co|d,
c,c|opo||ue.
IIChN FIANS (IF) |C|9 . o91.0
|C|0 . |+!
oi isomoiphic phenomenon (Fig. 7-7 B ).
SItes v] PredI|ectIvn Wiists flexoi (Fig.
7-4 )], lumbai iegion, shins thickei, hypei-
keiatotic lesions (Fig. 7-5 B )], scalp, glans penis
(See Figs. 35-9, 35-10), mouth (Image 7-2).
varaots
HypertrvphIc Laige thick plaques aiise on the
foot, doisum of hands (Fig. 7-5 ), and shins
(Fig. 7-5 B ); moie common in black males.
Although typical LP papule is smooth, hypei-
tiophic lesions may become hypeikeiatotic.
AtrvphIc White-bluish, well-demaicated pa-
pules and plaques with cential atiophy.
Fv||Icu|ur Individual keiatotic-folliculai pa-
pules and plaques that lead to cicatiicial alo-
pecia. Spinous folliculai lesions, typical skin and
mucous membiane LP, and cicatiicial alopecia
of the scalp (See Figs. 32-19, 32-20) aie called
Cra|am L|e synJrome . (See Section 32.)
VesIcu|ur Vesiculai oi bullous lesions may
develop within LP patches oi independent of
them within noimal-appeaiing skin. Theie aie
diiect immunofluoiescence findings consist-
ent with bullous pemphigoid, and the seia of
these patients contain bullous pemphigoid IgG
autoantibodies (see Section 6).
PIgmentvsus Hypeipigmented, daik-biown
macules in sun-exposed aieas and flexuial folds.
In Latin Ameiicans and othei daik-skinned
populations. Significant similaiity with ashy
deimatosis (see Section 13).
ActInIcus Papulai LP lesions aiise in sun-
exposed sites, especially the doisa of hands and
aims.
U|cerutIve LP may lead to theiapy-iesistant
ulceis, paiticulaily on the soles, iequiiing skin
giafting.
FICk T-4 Icheo p|aous ||+||opped, po|,ou+|, |+|p|, de||ued p+pu|e o| .|o|+ceou co|o|, |ouped
+ud cou||ueu|. 'u||+ce | ||u, +ud, upou c|oe |upec||ou W||| + |+ud |eu, ||ue W|||e ||ue +|e |e.e+|ed (w|c|
|+r |||+e, +||oW). C|oe up o| ||+||opped ||u, .|o|+ceou p+pu|e ||+| +|e po|,ou+|.

Mucous Membraoes Some 40-60% of individ-
uals with LP have oiophaiyngeal involvement
(see Section 34).
RetIcu|ur LP Reticulate (netlike) pattein of
lacy white hypeikeiatosis on buccal mucosa (see
Fig. 34-3), lips (Fig. 7-7), tongue, gingiva; the
most common pattein of oial LP.
ErvsIve vr U|cerutIve LP Supeificial eiosion
with/without oveilying fibiin clot; occuis on
tongue and buccal mucosa (see Fig. 34-3); shiny
ied painful eiosion of gingiva (desquamative
gingivitis) (see Fig. 34-5) oi lips (Fig. 7-7).
Caicinoma may veiy iaiely develop in mouth
lesions.
Ceota|a Papulai (see Figs. 35-9, 35-10) agmi-
nated, annulai, oi eiosive lesions aiise on penis
(especially glans), sciotum, labia majoia, labia
minoia, vagina.
har aod Na|s Scu|p Folliculai LP, atiophic
scalp skin with scaiiing alopecia (See Figs. 32-
19, 32-20). (See Section 32.)
NuI|s Destiuction of nail fold and nail bed
with longitudinal splinteiing (see Fig. 33-10).
IIChN FIANS-IIk kFII0NS
Lichen planus-like eiuptions closely mimic
typical LP, both clinically and histologically.
They occui as a clinical manifestation of chionic
GVHD, in deimatomyositis, and as cutaneous
manifestations of malignant lymphoma but
may also develop as the iesult of theiapy with
ceitain diugs and aftei industiial use of ceitain
compounds (Table 7-1).
Clinical findings confiimed by histopathology.
Sko Iesoos Pupu|ur LP Chionic cutane-
ous lupus eiythematosus, psoiiasis, pityiia-
sis iosea, eczematous deimatitis, lichenoid
GVHD; single lesions: supeificial basal cell
caicinoma, Bowen disease (in situ squamous
cell caicinoma).
HypertrvphIc LP Psoiiasis vulgaiis, lichen
simplex chionicus, piuiigo nodulaiis, stasis
deimatitis, Kaposi saicoma.
Drug-1nduced LP See Table 7-1.
Mucous Membraoes Leukoplakia, pseu-
domembianous candidiasis (thiush), HIV-asso-
ciated haiiy leukoplakia, lupus eiythematosus,
bite tiauma, mucous patches of secondaiy
syphilis, pemphigus vulgaiis, bullous pemphi-
goid (see Section 34).
0ermatopatho|oy Inflammation with hypei-
keiatosis, incieased gianulai layei, iiiegulai
acanthosis, liquefaction degeneiation of the
basal cell layei, and bandlike mononucleai in-
filtiate that hugs the epideimis. Keiatinocyte
apoptosis (colloid, Civatte bodies) is found at
the deimal-epideimal junction. Diiect immun-
ofluoiescence ieveals heavy deposits of fibiin at
the junction and IgM and, less fiequently, IgA,
IgG, and C3 in the colloid bodies.
C0kS
Cutaneous LP usually peisists foi months, but
in some cases, foi yeais; hypeitiophic LP on the
shins and oial LP often foi decades. The inci-
dence of oial squamous cell caicinoma in indi-
viduals with oial LP is incieased (5%); patients
should be followed at iegulai inteivals.
MANACMNI
Ioca| Iherapy
G|ucvcvrtIcvIds Topical glucocoiticoids with
occlusion foi cutaneous lesions. Intialesional
tiiamcinolone (3 mg/mL) is helpful foi symp-
tomatic cutaneous oi oial mucosal lesions and
lips.
Cyc|vspvrIne und Tucrv|Imus Sv|utIvns Re-
tention mouthwash" foi seveiely symptomatic
oial LP.
Systemc Iherapy
Cyc|vspvrIne In veiy iesistant and geneialized
cases, 5 mg/kg pei day will induce iapid iemis-
sion, quite often not followed by iecuiience.
G|ucvcvrtIcvIds Oial piednisone is effective
foi individuals with symptomatic piuiitus,
painful eiosions, dysphagia, oi cosmetic disfig-
uiement. A shoit, tapeied couise is piefeiied:
70 mg initially, tapeied by 5 mg/day.
SystemIc RetInvIds (AcItretIn) 1 mg/kg pei
day is helpful as adjunctive measuie in seveie
(oial, hypeitiophic) cases, but usually addi-
tional topical tieatment is iequiied.
FvA Fhotochemotherapy
In individuals with geneialized LP oi cases
iesistant to topical theiapy.
0ther Ireatmeots
Mycophenolate mofetil, hepaiin analogues
(enoxapaiin) in low doses have antipiolifeiative
and immunomodulatoiy piopeities; azathio-
piine.
FICk T-5 hypertrophc |cheo p|aous Cou||ueu| |,pe||e|+|o||c p+pu|e +ud p|+que ou ||e do|ur
o| ||e |+ud o| + ||||co|o|ed r+u o| A|||c+u deceu|. n,pe||e|+|o| co.e| w|c||+r |||+e, +ud ||e c|+|+c|e||||c
.|o|+ceou co|o| o| ||e |e|ou c+u |e eeu ou|, +| ||e .e|, r+||u. n,pe|||op||c ||c|eu p|+uu ou ||e |oWe|
|e o| + 50,e+|o|d r+u o| A|+||+u deceu|. |e|ou |o|r |||c| p|+que o| + d+|| ||oWu .|o|+ceou co|o| +ud
|+.e + |,pe||e|+|o||c u||+ce.
IA8I T-1 Aents |nducin Lichen P|anus and Lichenoid Reactions
Corrou |uduce|
Co|d +||
||oc|e|
Au||r+|+||+|
I||+/|de d|u|e||c
|u|oer|de
'p||ouo|+c|oue
|eu|c|||+r|ue
|e corrou
ACE |u|||||o|
C+|c|ur c|+uue| ||oc|e|
'u||ou,|u|e+
|ou|e|o|d+| +u|||u||+rr+|o|,
d|u
Ke|ocou+/o|e
Ie||+c,c||ue
||euo|||+/|ue
'u||++|+/|ue
C+||+r+/ep|ue
|||||ur
|e corrou
Au|||u|e|cu|o| d|u
|od|de
k+d|ocou||+| red|+
\e||,|dop+
ne+., re|+|
|uduce| o| ||c|eu p|+uu |,
cou|+c|
Co|o| |||r de.e|ope|
|eu|+| |e|o|+||.e r+|e||+|
\u| +r||e||e
||c||e
Co|d
|uduce| o| p|o|od|||||u|ed
||c|euo|d e|up||ou
5||uo|ou|+c||
C+||+r+/ep|ue
C||o|p|or+/|ue
||+/o\|de
E||+r|u|o|
|uduce| o| p|o|od|||||u|ed
||c|euo|d e|up||ou (:|-!)
|,||||uo|
0u|u|ue
0u|u|d|ue
Ie||+c,c||ue
I||+/|de
|u|oer|de
|uduce| o| o|+| ||c|eu p|+uu
+ud ||c|euo|d e|up||ou
A||opu||uo|
ACE |u|||||o|
C,+u+r|de
|eu|+| |e|o|+||.e r+|e||+|
Co|d +||
Ke|ocou+/o|e
|ou|e|o|d+| +u|||u||+rr+|o|,
d|u
|eu|c|||+r|ue
'u||ou,|u|e+
ACE, +u|o|eu|ucou.e|||u eu/,re.

FICk T-6 0ssemoated |cheo p|aous A |oWe| o| d|er|u+|ed p+pu|e ou ||e ||uu| +ud ||e e\||er|
||e (uo| |oWu) |u + +5,e+|o|d ||||p|uo. |ue |o ||e e||u|c co|o| o| ||e ||u, ||e p+pu|e +|e uo| + .|o|+ceou +
|u C+uc+|+u |u| |+.e + ||oWu|| |ue.
IMAC T-2 Icheo p|aous:
p|ed||ec||ou ||e.
FICk T-T Icheo p|aous '||.e|,W|||e, cou||ueu|, ||+||opped p+pu|e ou ||e ||p. |o|e. w|c||+r
|||+e (+||oW). B. ||c|eu p|+uu, Koe|ue| p|euoreuou. ||ue+| +||+uereu| o| ||+||opped, ||u, p+pu|e ||+|
e|up|ed +||e| c|+|c||u.
FI0MI0I0C
Common.
Ae oI 0oset Childien and young adults.
Sex Female:male iatio 2:1.
Unknown. An immunologically mediated
neciotizing inflammation that suiiounds blood
vessels, alteiing collagen and elastic tissue. Gen-
eialized GA may be associated with diabetes
mellitus.
Duiation months to yeais. Usually asympto-
matic and only cosmetic disfiguiement.
Sko Iesoos Fiim, smooth, shiny, beaded,
deimal papules and plaques, 1-5 cm annulai,
aicifoim plaques with cential depiession
(see Fig. 7-8, B), skin-coloied, violaceous,
eiythematous. Su|tuaneous C (iaie): painless,
skin-coloied, deep deimal oi subcutaneous,
solitaiy oi multiple nodules.
DIstrIhutIvn Isolated lesion, paiticulaily
on doisum of hand, fingei, oi lowei aim
(Fig. 7-8 ), multiple lesions on extiemities
and tiunk (Fig. 7-8 B ), oi geneialized (papulai;
oldei patients) (Fig. 7-8 C ). Subcutaneous le-
sions aie located neai joints, palms and soles,
buttocks.
varaots
Per[orang lesions aie veiy iaie and mostly on
the hands; cential umbilication followed by
ciusting and ulceiation; this type was associ-
ated with diabetes in one seiies.
May iaiely involve fascia and tendons, causing
scleiosis.
Geneialized GA: in this foim a seaich foi
diabetes mellitus should be made.
A corrou e||||r||ed, +,rp|or+||c, c||ou|c
de|r+|o| o| ||e de|r|.
uu+||, occu| |u c|||d|eu +ud ,ouu +du||.
Cou|| o| p+pu|e |u +u +uuu|+| +||+uereu|,
corrou|, +|||u ou ||e do|+ o| ||e |+ud +ud
|ee|, e||oW, +ud |uee.
'ore||re |ecore eue|+||/ed |u d|||||u||ou.
uu|e d|||u||u, uo ||e+|reu| | +u op||ou.
CkANI0MA ANNIAk (CA) |C|9 . o95.39
|C|0 . |92.0
GA is impoitant because of its similaiity to
moie seiious conditions.
Fapu|ar Iesoos aod F|aques Neciobiosis
lipoidica, papulai saicoid, lichen planus, lym-
phocytic infiltiate of Jessnei.
Subcutaoeous Nodu|es Rheumatoid nodules:
confusion can occui because of the similai
pathology of GA and iheumatic nodule oi
iheumatoid nodules. Also subcutaneous fungal
infections such as spoiotiichosis and NTM (M.
marnum).
Aoou|ar Iesoos Tinea, eiythema migians,
saicoid, lichen planus.
0ermatopatho|oy Foci of chionic inflamma-
toiy and histiocytic infiltiations in supeificial
and mid-deimis, with neciobiosis of connective
tissue suiiounded by a wall of palisading histio-
cytes and multinucleated giant cells.
C0kS
The disease disappeais in 75% of patients in
2 yeais. Recuiiences aie common (40%), but
they also disappeai.
MANACMNI
GA is a local skin disoidei and not a maikei foi
inteinal disease, and spontaneous iemission is
the iule. No reamen s an oon [ |e |esons are
no Js[gurng . Lesions may iesolve aftei biopsy.
Iopca| Iherapy TvpIcu| G|ucvcvrtIcvIds -
Applied undei plastic occlusion oi hydiocolloid.
1ntru|esIvnu| TrIumcInv|vne 3 mg/mL into
lesions is veiy effective.
Cryvspruy Supeificial lesions iespond to liq-
uid nitiogen, but atiophy may occui.
FvA Fhotochemotherapy Effective in genei-
alized GA.
Systemc C|ucocortcods Effective in geneial-
ized GA, but iecuiiences common.
FICk T-8 Craou|oma aoou|are Cou||ueu|, pe+||,W|||e p+pu|e |o|r|u + We||der+|c+|ed ||u W|||
ceu||+| |e|e|ou. \u|||p|e |+uu|or+|+ |o|r|u +uuu|+| +ud er|c||cu|+| p|+que W||| ceu||+| |e|e|ou ou
||e +|r o| + +5,e+|o|d r+u o| A|||c+u e\||+c||ou. ||er|u+|ed |+uu|or+ +uuu|+|e |u + C+uc+|+u. \u|||p|e,
We||de||ued, pe+||,W|||e p+pu|e, ore o| W||c| |oW + ceu||+| dep|e|ou

Iocdeoce Raie between the ages of 20 and 50;
in lineai moiphea, eailiei. Panscleiotic Moi-
phea, a disabling disoidei, usually staits befoie
age 14.
Sex Females aie affected about thiee times as
often as males, including childien. Lineai scle-
iodeima is the same in males and females.
to|oy Unknown. At least some patients
(piedominantly in Euiope) with classic moi-
phea have scleiosis due to Borre|a |urgJor[er
infection, and, if not too scleiotic, the lesions
can disappeai with piolonged couises of oial
antibiotics. Pigmentation, howevei, peisists.
Moiphea has been noted aftei x-iiiadiation
foi bieast cancei. Moiphea is not ielated to
systemic scleiodeima.
CIASSIFICAII0N 0F vAkI0S IFS 0F
I0CAIII0 SCIk00kMA
Crtumstr|eJ . plaques oi bands
Matu|ar. small, confluent patches
Lnear st|eroJerma . uppei oi lowei extiemity
Fronoarea| (en tou Je sa|re)
Cenera|:eJ mor|ea
Panst|erot . involvement of deimis, fat, fascia,
muscle, bone.
Symptoms Usually none. No histoiy of
Raynaud phenomenon. Lineai and panscleiotic
moiphea can iesult in majoi facial oi limb
asymmetiy, flexion contiactuies, and disability.
Can cause seveie disfiguiement.
Sk o F od os P|aques -ciicumsciibed,
induiated, haid, but pooily defined aieas of
skin; 2-15 cm in diametei, iound oi oval, often
bettei felt than seen. Initially, puiplish oi mauve.
A |oc+||/ed +ud c||curc|||ed cu|+ueou c|e|o|
c|+|+c|e||/ed |, e+||, .|o|+ceou, |+|e| |.o|,
co|o|ed, |+|deued ||u.
\+, |e o|||+|,, ||ue+|, eue|+||/ed, +ud, |+|e|,, +c
corp+u|ed |, +||op|, o| uude||,|u ||uc|u|e.
|| | uu|e|+|ed |o ,|er|c c|e|ode|r+.
',, |oc+||/ed c|e|ode|r+, c||curc|||ed
c|e|ode|r+.
M0kFhA |C|9 . 10.0
|C|0 . |9+.0
In time, suiface becomes smooth and shiny
aftei months to yeais, ivoiy with lilac-coloied
edge lilac iing" (Fig. 7-9). May have hypei- and
hypopigmentation in involved scleiotic aieas
(Fig. 7-10). Raiely, lesions become atiophic
and hypeipigmented without going thiough
a scleiotic stage (atiophodeima of Pasini and
Pieiini) (see Fig. 7-13B).
DIstrIhutIvn
Crtumstr|eJ. Tiunk (Fig. 7-9), limbs, face,
genitalia; less commonly, axillae, peii-
neum, aieolae.
Cenera|:eJ . Initially on tiunk (uppei, bieasts,
abdomen) (Fig. 7-10) thighs.
Lnear . Usually on extiemity (Fig. 7-11) oi
[ronoarea|-scalp and face (Fig. 7-12);
heie it may iesemble a scai fiom a stiike
with a sabei ( en tou Je sa|re ).
Matu|ar. Small (<3 mm) maculai patches,
confluent (Fig. 7-13 ); clinically indistin-
guishable fiom lichen scleiosus et atiophi-
cus (see p. 142).
ro|t. Atiophodeima of Pasini and Pieiini
(Fig. 7-13 B ).
Panst|erot . On tiunk (Fig. 7-14) oi extiemi-
ties.
Mouth With lineai moiphea of head, may
have associated hemiatiophy of tongue.
har aod Na|s Scaiiing alopecia with scalp
plaque. Paiticulaily with lineai moiphea of the
head. Nail dystiophy in lineai lesions of extiem-
ity oi in panscleiotic moiphea.
Ceoera| xamoatoo
Moiphea aiound joints and lineai moiphea
may lead to flexion contiactuies. Panscleiotic
moiphea is associated with atiophy and fibiosis
of muscle (Fig. 7-14). Extensive involvement of
tiunk may iesult in iestiicted iespiiation. With
lineai moiphea of the head (Fig. 7-12), theie
may be associated atiophy of oculai stiuctuies
and atiophy of bone. Noe. moiphea may be as-
sociated with lichen scleiosus et atiophicus.
Clinical, confiimed by biopsy. Scleiotic plaque
associated with B. |urgJor[er infection, acio-
deimatitis chionica atiophicans, piogiessive
systemic scleiosis, lichen scleiosus et atiophi-
cus, eosinophilic fasciitis, toxic oil syndiome,
eosinophilia-myalgia syndiome associated with
L-tiyptophan ingestion, scleiedema, Paiiy-
Rombeig syndiome (hemiatiophy).
FICk T-9 Morphea I|| | +u |udu|+|ed |.o|,co|o|ed, ||u, p|+que W||| + |||+cco|o|ed, |||de||ued |o|de|
(+||oW). \o| |e|ou +|e |e||e| |e|| ||+u eeu |ec+ue ||e, +|e |udu|+|ed.
Sero|oy Appiopiiate seiologic testing to iule
out B. |urgJor[er infection.
0ermatopatho|oy Epideimis appeais noi-
mal to atiophic with loss of iete iidges. Deimis
edematous with homogeneous and eosinophilic
collagen. Slight infiltiate, peiivasculai oi dif-
fuse; lymphocytes, plasma cells, maciophages.
Latei, deimis thickened with few fibioblasts
and dense collagen; inflammatoiy infiltiate at
deimal-subcutis junction; deimal appendages
disappeai piogiessively. Panscleiotic lesions
show fibiosis and disappeaiance of subcutane-
ous tissue, with fibiosis involving fascia. Silvei
stains should be peifoimed to iule out B. |urg-
Jor[er infection. Histopathology distinct fiom
that of lichen scleiosus et atiophicus.
0IACN0SIS
Clinical diagnosis, usually confiimed by skin
biopsy.
C0kS
May be slowly piogiessive; buin out" and
spontaneous iemissions can iaiely occui.
FICk T-10 Morphea |||eu|+|, ||oWu||, |udu|+|ed |e|ou W||| |oc+| |.o|,co|o|ed r+cu|+| |e|ou ou ||e
|e|| ||p. '|r||+| |e|ou We|e +|o |ouud ou ||e c|e| +ud ou ||e |+c|.
MANACMNI
Theie is no effective tieatment foi moiphea, but
some iepoits of tieatment aie as follows:
Morphea-Ike Iesoos Assocated wth Iyme
8orre|oss In patients with eaily involvement,
theie may be a ieveisal of scleiosis with high-
dose paienteial penicillin oi ceftiiaxone; tieat-
ment given in seveial couises ovei a time span
of seveial months. Best iesponse if combined
with oial glucocoiticoids.
Fhototherapy wth vA-1 (340-400 om) In
oui expeiience, the tieatment is not easy oi veiy
successful because of the piolonged iiiadiation
times and the disfiguiing hypeipigmentation of
the iiiadiated aieas.
FICk T-11 Ioear Morphea
|udu|+|ed, |.o|,W|||e |e|ou e\|eud|u
||or uppe| |||| |o ||e do|ur o| ||e |oo|.
|udu|+||ou | p|ououuced, +ud |u ||e |e|ou
+|o.e ||e |uee || e\|eud |o ||e |+c|+ (p+u
c|e|o||c ro|p|e+). || p|o|e|.e, || W||| ||r||
||e ro.ereu| o| ||e jo|u|.
FICk T-12 Ioear morphea,
"eo coup de sabre" IWo ||u
e+|, p+|||+||, |.o|,W|||e (ou ||e
c+|p) +ud |,pe|p|reu|ed (ou
||e |o|e|e+d) dep|eed |e|ou
e\|eud|u ||or ||e c|oWu o| ||e
|e+d, W|e|e ||e, |+.e |ed |o
+|opec|+, o.e| ||e |o|e|e+d |o ||e
o||||+. I|e, |oo| |||e c+| +||e|
||||e W||| + +|e|, |euce ||e
||euc| de|u+||ou. I|ee |e|ou
c+u e\|eud |o ||e |oue +ud |+|e|,
|o ||e du|+ r+|e|.
FICk T-13 Macu|ar Iorm oI morphea I|e|e +|e ru|||p|e, ||u|u, |.o|,W|||e r+cu|e W||| cou||u
euce |e+d|u |o + |e||cu|+|ed p+||e|u. I|ee |e|ou +|e |+||e| upe|||c|+| +ud ||e|e|o|e |e |udu|+|ed. Au |rpo|
|+u| d|||e|eu||+| d|+uo| | ||c|eu c|e|ou e| +||op||cu. A||op||c, |,pe|p|reu|ed |o|r o| ro|p|e+ (c+||ed
+||op|ode|r+ o| |+|u| +ud ||e||u|). I|e|e | + d|||ue ||oWu +ud |+|p|, de||ued |,pe|p|reu|+||ou W||| + |e
p|reu|ed |o|||cu|+| p+||e|u. I|ee |e|ou +|e +||op||c +ud uo| |udu|+|ed.

FICk T-14 Faosc|erotc morphea I|| |,pe +||ec| +|| |+,e| o| ||e ||u |uc|ud|u ||e |+c|+ +ud e.eu
ruc|e. I|e ||u | |||eu|u, |,pe|p|reu|ed, +ud |+|d + Wood. || | o|.|ou ||+| p+uc|e|o||c ro|p|e+ |e+d
|o cou|de|+||e |uuc||ou+| |rp+||reu|. || ||ee |e|ou occu| ou ||e uppe| ||uu|, ||e, c+u |rp+|| e\cu||ou o| ||e
c|e| +ud ||u ||e+|||u.
|'A | + c||ou|c +||op||c d|o|de| r+|u|, o| ||e
+uoeu||+| ||u o| |er+|e |u| +|o o| r+|e +ud
o| ||e eue|+| ||u.
A d|e+e o| +du||, |u| +|o occu|||u |u c|||d|eu
-! ,e+| o| +e. |er+|e |eu ||re ro|e o||eu
+||ec|ed ||+u r+|e.
w|||||, |.o|, o| po|ce|+|uW|||e, |+|p|, der+|
c+|ed, |ud|.|du+| p+pu|e r+, |ecore cou||ueu|,
|o|r|u |c- (||. 15). 'u||+ce o| |e|ou
r+, |e e|e.+|ed o| |u ||e +re p|+ue + uo|r+|
||u, o|de| |e|ou r+, |e dep|eed. |||+|ed
p||oe|+ceou o| We+| duc| o||||ce ||||ed W|||
|e|+||u p|u (de||), || p|u|u | r+||ed, u||+ce
+ppe+| |,pe||e|+|o||c (||. 15C).
3||c- +ud - occu| +ud c | o||eu +
c|+|+c|e||||c +ud |deu|||,|u |e+|u|e (||. 15B),
|-|c-:|cc.
|e|ou occu| ou eue|+| ||u o| ou ||e eu||+||+.
0u .u|.+, |,pe||e|+|o||c p|+que r+, |ecore
e|o|.e, r+ce|+|ed, .u|.+ r+, |ecore +||op||c,
||uu|eu, epec|+||, c|||o|| +ud |+||+ r|uo|+, W|||
.+|u+| |u||o||u |educed |u |/e (ee ||. !5o).
|u|ou o| |+||+ r|uo|+ +ud r+jo|+.
|u uuc||curc|ed r+|e, p|epuce |||| |oW
|.o|, W|||e cou||ueu| p+pu|e (ee ||. !52,
!5+) |u| ||eu |ecore c|e|o||c +ud c+uuo| |e
|e||+c|ed ( | ). C|+u +ppe+| |.o|, o| po|
ce|+|uW|||e, er|||+up+|eu|, |eer|||u ro||e|
o|pe+|| W||| +dr|\ed pu|pu||c |ero|||+e.
|oueu||+| |'A uu+||, +,rp|or+||c, eu||+|
,rp|or+||c. |u |er+|e, .u|.+| |e|ou r+, |e
eu|||.e, epec|+||, W|||e W+|||u, p|u|||u, p+|u
|u|, epec|+||, || e|o|ou +|e p|eeu|, d,u||+, d,
p+|euu|+. |u r+|e, |ecu||eu| |+|+u|||, +cqu||ed
p||ro|.
I|e |||op+||o|o, | d|+uo||c W||| + deue
|,rp|oc,||c |u|||||+|e |u|u ||e |u|||+||, |,pe|
||op||c +ud |+|e|, +||op||c ep|de|r| +ud ||eu
|u||u doWu |u|o ||e de|r|, |e|u ep+|+|ed
||or ||e ep|de|r| |, +u eder+|ou, ||uc|u|e
|e u|ep|de|r+| /oue.
I|e e||o|o, o| |'A | uu|uoWu, |u| |epo|| ||or
Eu|ope |+.e docureu|ed +u +oc|+||ou o| ||A
o| 3-|c pp. W||| |'A |u c+e ||or Ce|r+u,
+ud l+p+u, ||A o| ||e p||oc|e|e de|ec|ed |u
||ee p+||eu| W+ uo| |ouud |u +u, o| ||e Are||
c+u +rp|e.
I|e cou|e o| |'A W+\e +ud W+ue. |u ||| ||
r+, uude|o pou|+ueou |eo|u||ou, |u Woreu
|| |e+d |o +||op|, o| ||e .u|.+ +ud |u reu |o
p||ro|. |+||eu| |ou|d |e c|ec|ed |o| ||e oc
cu||euce o| qu+rou ce|| c+|c|uor+ o| ||e .u|.+
+ud peu|.
\+u+ereu| | .e|, |rpo||+u|, + ||| d|e+e c+u
c+ue + de.+|+||u +||op|, o| ||e |+||+ r|uo|+
+ud c|||o|+| |ood. |o|eu| |op|c+| |::|:!
-cc| (c|o|e|+o| p|op|ou+|e) |+.e p|o.ed
e||ec||.e |o| eu||+| |'A +ud |ou|d |e ued |o|
o-3 Wee| ou|,. |+||eu| |ou|d |e rou||o|ed
|o| |u o| |ucoco|||co|d|uduced +||op|,. |-
:| +ud |c:| +|e +|ro| + e||ec||.e.
:c| c!- +|e |e ued uoW |ec+ue
||e, c+u ore||re c+ue + c|||o|+| |,pe|||op|,.
',|-: ||-c, . |,d|oc||o|oqu|ue, 25-50
r/d, |o| Wee| |o + |eW rou|| (rou||o| |o|
ocu|+| |de e||ec|).
|u r+|e, ::: |e||e.e ,rp|or o| p||
ro| +ud |u ore c+e c+u |eu|| |u |er||ou.
IIChN SCIk0SS et AIk0FhICS (ISA)
|C|9 . 10.0
|C|0 . |90.0
FICk T-15 Icheo sc|ero-

sus et atrophcus \u|||p|e,
|.o|,W|||e, |udu|+|ed +ud |||||,
|,pe||e|+|o||c p+pu|e ou ||e c|e|
o| + +2,e+|o|d Wor+u. I|e |.o|,
W|||e p+pu|e o| ||c|eu c|e|ou |e|e
|+.e re|ed |o |o|r + upe|||c|+||,
|udu|+|ed +ud +|o +||op||c p|+que
o| |+|p r+||u+||ou. nero|||+e |u
||e ceu|e| o| ||| p|+que | +u |rpo|
|+u| d|||e|eu||+| d|+uo||c |u |o ||e
r+cu|+| |o|r o| ro|p|e+ ||c|eu
c|e|ou |u ||e |o|u |e|ou o| +
o0,e+|o|d |er+|e. ne|e ||e p+pu|e
|+.e re|ed |o |o|r + |+|e |,pe|
|e|+|o||c p|+que W||| |+|p de||u|||ou.
I|e|e +|e +|o c|u| |eu|||u ||or
e|o|ou.

||| +|e d|||uu||ed |, ||e|| c||u|c+| c|+|+c|e||
||c, |+.|u |deu||c+| de|r+|op+||o|o|c ||ud|u,
+ud |uc|ude.
':|+r|e| d|e+e , +|o |uoWu + p|o|e|.e
p|reu|ed pu|pu||c de|r+|o| o| p|o|e|.e
p|reu|+|, pu|pu|+
\+jocc|| d|e+e , +|o |uoWu + pu|pu|+ +u
uu|+|| |e|+u|ec|ode
Coue|o|B|ur d|e+e, +|o |uoWu + p|
reu|ed pu|pu||c ||c|euo|d de|r+|||| o| pu|
pu|+ p|reu|o+ c||ou|c+
||c|eu +u|eu , +|o |uoWu + ||c|eu pu|pu||cu.
C||u|c+||,, e+c| eu|||, |oW |eceu| p|upo|u| c+,
euue peppe|-co|o|ed |ero|||+e +oc|+|ed
W||| o|de| |ero|||+e +ud |ero|de||u depo|
||ou. C+p|||+|||| |||o|o|c+||,.
||| +|e |u|||c+u| ou|, || ||e, +|e + core||c
couce|u |o ||e p+||eu|, ||e, +|e |rpo||+u| |e
c+ue ||e, +|e o||eu r||+|eu + r+u||e|+||ou
o| .+cu|||| o| |||or|oc,|opeu|+.
',, . C+p|||+|||| o| uu|uoWu c+ue.
|C|9. 109.
|C|0. |3.1
FICMNI0 FkFkIC 0kMAI0SS (FF0)
Ae oI 0oset 30-60 yeais; uncommon in
childien.
to|oy Unknown. Piimaiy piocess believed
to be cell-mediated immune injuiy with subse-
quent vasculai damage and eiythiocyte extiava-
sation. Othei etiologic factois: piessuie, tiauma,
diugs (acetaminophen, ampicillin-caibiomal,
diuietics, mepiobamate, nonsteioidal anti-
inflammatoiy diugs, zomepiiac sodium).
0oset aod 0uratoo Insidious, slow to evolve
except diug-induced vaiiant, which may develop
iapidly and be moie geneialized in distiibution.
Peisists foi months to yeais. Most diug-induced
puipuias iesolve moie quickly aftei discontinua-
tion of the diug. Usually asymptomatic but may
be mildly piuiitic. It can be quite cosmetically
disfiguiing. Any piuiitus piobably ielated to dei-
matitis (asteatotic, atopic, oi stasis) on lowei legs.
Schamber 0sease Disciete clusteis of
pinhead-sized ied macules and baiely palpa-
ble papules become confluent, coalescing into
patches (Fig. 7-16). Diascopy ieveals pinpoint
hemoiihages (hence the teim urura ). New
lesions aie ied; oldei lesions tan to biown,
iepiesenting degiadation of extiavasated eiyth-
iocytes with the foimation of hemosideiin.
Oveiall coloi impiession: ieddish biown, cay-
enne peppei" (Fig. 7-16). Lowei extiemities
(especially pietibial and on ankles) but may
extend pioximally to lowei tiunk and to uppei
extiemities. Usually bilateial but may be unilat-
eial. Uncommonly, geneialized.
Majocch 0sease Essentially an annulai foim
of Schambeig disease with telangiectasias
(Fig. 7-17). An aicifoim vaiiant has also been
desciibed.
Couerot-8|um 0sease Lichenoid papules,
plaques, macules in association with lesions of
Schambeig disease.
Icheo Aureus Solitaiy oi few patches oi
plaques, iust-coloied, puiple, oi golden, aiising
on the extiemities oi tiunk.
0ermatopatho|oy Epideimal involvement
vaiies, but deimal pathology (capillaiitis) with
extiavasation of eiythiocytes, hemosideiin
pigment-laden maciophages (moie extensive
in lichen auieus), mild peiivasculai and intei-
stitial lymphohistiocytic infiltiate in ieticulai
deimis is common to all. Immunofluoiescence
is vaiiable and nonspecific.
Noopa|pab|e Furpura Chionic venous insuf-
ficiency with clotting abnoimalities, gluco-
coiticoid usage, cutaneous T cell lymphoma;
dyspioteinemias, nummulai eczema, old fixed
diug eiuption, paiapsoiiasis, poikilodeima
vasculaie atiophicans, piimaiy amyloidosis,
scuivy, senile puipuia, stasis deimatitis, thiom-
bocytopenia, tiauma.
Fa|pab|e Furpura Leukocytoclastic vasculitis.
Thiombocytopenic puipuia.
C0kS
Chionic (months to yeais), slow to evolve
and iesolve; spontaneous iesolution has
occuiied. In lesions of long standing,
hemosideiin deposits iesolve veiy slowly
(months to yeais). Almost all cases due to
diugs cleai within months aftei discon-
tinuation of the offending agent.
MANACMNI
Symptomatc Long-standing lesions aie
cosmetically disfiguiing, and patients
may choose to tieat these lesions. Topical
low- and middle-potency glucocoiticoid
piepaiations may inhibit new puipuiic
lesions. Systemic tetiacycline oi mino-
cycline (50 mg twice daily) aie effective.
PUVA is effective in seveie foims. Su-
ore sot|ngs regureJ n a|| [orms.
FICk T-16 Fmeoted purpurc der-
matoss: Schamber dsease \u|||p|e
d|c|e|e +ud cou||ueu| uoup+|p+||e, uou
||+uc||u pu|pu||c |e|ou ou ||e |e. Acu|e
r|c|o|ero|||+e |eo|.e W||| depo|||ou o|
|ero|de||u, c|e+||u + ||oWu peppe|ed |+|u.
FICk T-1T Fmeoted purpurc
dermatoss: Majocch dsease \u|||p|e
uoup+|p+||e, uou||+uc||u pu|pu||c |e|ou
+||+ued |u +uuu|+| cou||u|+||ou. |o|e. d|||
u||u d+|| ||oWu d|co|o|+||ou o| o|d |e|ou.
Ae oI 0oset Adolescents and young adults.
Sex Moie common in males than females.
to|oy Unknown.
Lesions tend to appeai in ciops ovei a peiiod of
weeks oi months. Uncommonly, patients with
an acute onset of the disoidei may have symp-
toms of an acute infection with fevei, malaise,
and headache. Cutaneous lesions aie usually
asymptomatic but may be piuiitic oi sensitive
to touch. Lesions may heal with significant
scaiiing and postinflammatoiy pigmentation.
Especially in that it occuis in adolescents and
young adults.
Sko Iesoos Initially, iandomly distiibuted,
biight-ied edematous papules (i.e., lichenoides),
less commonly vesicles, which undeigo cential
neciosis with hemoiihagic ciusting (i.e.,
vaiiolifoimis, hence the designation PLEV )
(Fig. 7-18 and B ). In the chionic foim (PLC),
scaling papules of ieddish-biown coloi and a
cential mica-like scale aie seen (Fig. 7-18 C ).
Postinflammatoiy hypo- oi hypeipigmentation
often piesent aftei lesions iesolve. PLEVA may
heal with depiessed oi elevated scais.
DIstrIhutIvn Randomly aiianged, most com-
monly on tiunk, pioximal extiemities but also
geneialized, including palms and soles.
0ra| aod Ceota| Mucosa Inflammatoiy pa-
pules and neciotic lesions may occui.
|| | +u e|up||ou o| uu|uoWu e||o|o,, c|+|+c|e|
|/ed c||u|c+||, |, ucce|.e c|op o| + W|de |+ue
o| ro|p|o|o|c |e|ou.
C|+|||ed |u|o +u +cu|e |o|r, p||,||+| ||c|euo|de
e| .+||o|||o|r| +cu|+ (||E\A, \uc|+n+|e|r+uu
d|e+e), +ud + c||ou|c |o|r, p||,||+|
||c|euo|de c||ou|c+ (||C, u||+|e p+|+po||+|
o| lu||u|e|).
noWe.e|, ro| p+||eu| |+.e |e|ou o| ||E\A
+ud ||C |ru||+ueou|,.
||E\A | |rpo||+u| |ec+ue || c+u |e r||+|eu |o|
|,rp|or+|o|d p+pu|o| (ee 'ec||ou 20).
',, Cu||+|e p+|+po||+|.
FIIkIASIS IIChN0I0S (ACI AN0 Chk0NIC) (FI)
|C|9 . o9o.2
|C|0 . |+.0/|+.
0ermatopatho|oy EJerms : spongiosis, ke-
iatinocyte neciosis, vesiculation, ulceiation;
exocytosis oi eiythiocytes within epideimis.
Derms : Edema, chionic inflammatoiy cell infil-
tiate in wedge shape extending to deep ieticulai
deimis; hemoiihage; vessels congested with
blood; endothelial cells swollen.
Clinical diagnosis is confiimed by skin biopsy.
Diffeiential diagnosis: vaiicella, guttate psoiia-
sis, lymphomatoid papulosis.
C0kS AN0 Fk0CN0SIS
New lesions appeai in successive ciops.
PLC tends to iesolve spontaneously aftei
6-12 months. In some cases, ielapses aftei many
months oi yeais.
MANACMNI
Most patients do not iequiie any theiapeutic
inteivention. Oial eiythiomycin and tetiacy-
cline aie iepoited to be effective in some cases.
Ultiaviolet iadiation (whethei natuial sunlight
oi bioad-band UVB), 311-nm UVB, and PUVA
aie the tieatments of choice if oial antibiotics
fail aftei a 2-week tiial.
FICk T-18 Ftyrass |cheoodes et varo|Iorms acuta (FIvA) k+udor|, d|||||u|ed |ed p+p
u|e o| d|||e|eu| |/e, ore o| W||c| |oW |ero|||+|c c|u||u. |u ||| 5,e+|o|d c|||d ||e e|up||ou +ppe+|ed |u
c|op o.e| + pe||od o| 0 d+,. FIvA |e|ou |u + !3,e+|o|d |udoue|+u r+u. |e|ou +|e ro|e |,pe|p|
reu|ed +ud ||e|e | cou|de|+||e c+||u +ud c|u||u. Ftyrass |cheoods chrooca (FIC) ||c|e|e p+pu|e
W||| ||ue r|c+|||e c+|e W||c| |ecore ro|e .||||e +||e| |||| c|+p|u. |u cou||+| |o ||E\A (||. 13A +ud B),
||e|e | uo |ero|||+|c c|u||u
FI0MI0I0C
Ae oI 0oset 50% undei 20 yeais.
Sex Moie fiequent in males than in females.
II0I0C
A cutaneous ieaction to a vaiiety of antigenic
stimuli, most commonly to heipes simplex.
IoIectoo Especially following heipes simplex,
Myto|asma .
0rus Sulfonamides, phenytoin, baibituiates,
phenylbutazone, penicillin, allopuiinol.
Idopathc Piobably also due to undetected
heipes simplex oi M yto|asma .
Evolution of lesions ovei seveial days. May have
histoiy of piioi EM. May be piuiitic oi pain-
ful, paiticulaily mouth lesions. In seveie foims
constitutional symptoms such as fevei, weak-
ness, malaise.
Sko Iesoos Lesions may develop ovei 10
days. Macule papule (1-2 cm) vesicles
and bullae in the centei of the papule; (Fig.
7-19). Dull ied. Irs oi arge||e |esons iesult
and aie typical (Figs. 7-19 and 7-20). Localized
to hands and face oi geneialized (Figs. 7-21 and
7-22). Bilateial and often symmetiic.
SItes v] PredI|ectIvn Doisa of hands, palms,
and soles; foieaims; feet; face (Figs. 7-20 and
7-21); elbows and knees; penis (50%) and vulva
(Image 7-3).
Mucous Membraoes Eiosions with fibiin
membianes; occasionally ulceiations: lips
(Fig. 7-20), oiophaiynx, nasal, conjunctival
(Fig. 7-21), vulvai, anal.
0ther 0raos Eyes, with coineal ulceis, ante-
iioi uveitis.
A corrou |e+c||ou p+||e|u o| ||ood .ee| |u
||e de|r| W||| ecoud+|, ep|de|r+| c|+ue.
\+u||e| c||u|c+||, + c|+|+c|e||||c e|,||er+|ou
||||+ped p+pu|+| +ud .e|cu|o|u||ou |e|ou.
I,p|c+||, |u.o|.|u ||e e\||er|||e (epec|+||, ||e
p+|r +ud o|e) +ud ||e rucou rer||+ue.
Beu|u cou|e W||| ||equeu| |ecu||euce.
\o| c+e |e|+|ed |o |e|pe |rp|e\ .||u (n'\)
|u|ec||ou
kecu||euce c+u |e p|e.eu|ed |, |ou|e|r +u||
n'\ red|c+||ou.
|C|9 . o95.
|C|0 . |5
kIhMA MIIIF0kM SN0k0M (M)
C0kS
M|d Forms (M Moor) Little oi no mucous
membiane involvement; vesicles but no bullae
oi systemic symptoms. Eiuption usually con-
fined to extiemities, face, classic taiget lesions
(Figs. 7-19 and 7-20). Recuiient EM minoi is
usually associated with an outbieak of heipes
simplex pieceding it by seveial days.
Severe Forms (M Major) Most often occuis as
a diug ieaction, always with mucous membiane
involvement; seveie, extensive, tendency to be-
come confluent and bullous, positive Nikolsky
sign in eiythematous lesions (Fig. 7-21). Sys-
temic symptoms: fevei, piostiation. Cheilitis
and stomatitis inteifeie with eating; vulvitis and
balanitis with mictuiition. Conjunctivitis can
lead to keiatitis and ulceiation; lesions also in
phaiynx and laiynx.
0ermatopatho|oy Inflammation chaiactei-
ized by peiivasculai mononucleai infiltiate,
edema of the uppei deimis; apoptosis of ke-
iatinocytes with focal epideimal neciosis and
subepideimal bulla foimation. In seveie cases,
complete neciosis of epideimis as in toxic epi-
deimal neciolysis. (See Section 8.)
The taiget-like lesion and the symmetiy aie
quite typical, and the diagnosis is not difficult.
Acute xaothematc ruptoos Diug eiuption,
psoiiasis, secondaiy syphilis, uiticaiia, gen-
eialized Sweet syndiome. Mucous membiane
lesions may piesent a difficult diffeiential di-
agnosis: bullous diseases, fixed diug eiuption,
acute lupus eiythematosus, piimaiy heipetic
gingivostomatitis.
FICk T-19 rythema mu|tIorme ||| o| |+|e| |e|ou ou ||e |oWe| +|r o| + 5,e+|o|d. I|ee +|e
|+|p|, de||ued ||+| p+pu|e W||| + ceu||+| .e|c|e.
IMAC T-3 rythema mu|tIorme p|ed||ec||ou ||e +ud d|||||u||ou.
FICk T-20 rythema mu|tIorme: moor \u|||p|e, cou||ueu|, |+|e||||e p+pu|e ou ||e |+ce o| +
2,e+|o|d |o,. I|e |+|e| ro|p|o|o, o| ||e |e|ou | |e| eeu ou ||e ||p.
FICk T-21 rythema mu|t-
Iorme: major E|,||er+|ou, cou||u
eu|, |+|e||||e p+pu|e, p|+que, +ud
e|o|ou ou ||e ||uu|, ||e +|r, +ud
||e |+ce. |+c|+| |e|ou +|e e|o|.e +ud
c|u|ed. I|e|e | e|o|.e c|e||||| |ud|c+|
|u ruco+| |u.o|.ereu|, +ud ||e|e |
coujuuc||.|||.
FICk T-22 rythema mu|tIorme: major \u|||p|e, |+|e| |e|ou |+.e co+|eced, +ud e|o|ou W|||
de.e|op. I|| p+||eu| |+d |e.e| +ud ruco+| |u.o|.ereu| o| rou||, coujuuc||.+, +ud eu||+||+
MANACMNI
Freveotoo Contiol of heipes simplex using
oial valacyclovii oi famciclovii may pievent
development of iecuiient EM.
C|ucocortcods In seveiely ill patients, sys-
temic glucocoiticoids aie usually given (pied-
nisone, 50-80 mg/d in divided doses, quickly
tapeied), but theii effectiveness has not been
established by contiolled studies.
The most common type of panniculitis, with a
peak incidence at 20-30 yeais, but any age may
be affected. Thiee to six times moie common
on females than males.
to|oy EN is not a disease but a cutaneous
ieaction pattein to vaiious etiologic agents.
Etiologic associations include infections, diugs,
and othei inflammatoiy/gianulomatous dis-
eases, notably saicoidosis (Table 7-2).
Painful, tendei lesions, usually of a few days`
duiation, aie accompanied by fevei, malaise,
and aithialgia (50%), most fiequently of ankle
joints. Othei symptoms, depending on etiology.
Sko Iesoos Induiated, veiy tendei nodules
(3-20 cm), not shaiply maiginated (Fig. 7-23),
deep seated in the subcutaneous fat, mostly
on the anteiioi lowei legs, bilateial but not
symmetiic. Nodules aie biight to deep ied and
aie appieciated as such only upon palpation.
The teim ery|ema noJosum best desciibes
the skin lesions: |ey |oo| ||e ery|ema |u
[ee| ||e noJu|es (Fig. 7-23). Lesions aie oval,
iound, aicifoim; as they age, they become
violaceous, biownish, yellowish, gieen, like
iesolving hematomas. Lesions may also occui
on knees and aims but only iaiely on the face
and on the neck.
hemato|oy Elevated eiythiocyte sedimenta-
tion iate (ESR), C-ieactive piotein elevated,
leukocytosis.
E| | +u |rpo||+u| +ud corrou +cu|e |u||+rr+
|o|,/|rruuo|o|c |e+c||ou p+||e|u o| ||e u|cu|+
ueou |+|.
C|+|+c|e||/ed |, ||e +ppe+|+uce o| p+|u|u| uod
u|e ou ||e |oWe| |e.
|e|ou +|e ||||| |ed +ud ||+| |u| uodu|+| upou
p+|p+||ou.
0||eu |e.e| +ud +|||||||.
\u|||p|e +ud d|.e|e e||o|o|e.
kIhMA N000SM (N) SN0k0M
|C|9 . o95.2
|C|0 . |52
8actera| Cu|ture Cultuie thioat foi gioup A
-hemolytic stieptococcus, stool foi Yersna .
Imao Radiologic examination of the chest
and gallium scan aie impoitant to iule out oi
piove saicoidosis.
0ermatopatho|oy Acute (polymoiphonu-
cleai) and chionic (gianulomatous) inflamma-
tion in the panniculus and aiound blood vessels
in the septum and adjacent fat. It is a septal
panniculitis.
C0kS
Spontaneous iesolution occuis in 6 weeks,
with new lesions eiupting duiing that time.
Couise depends on the etiology. Lesions nevei
bieak down oi ulceiate and heal without
scaiiing.
Diagnosis iests on clinical ciiteiia, may be sup-
poited by histopathology. Diffeiential diagnosis
includes all othei foims of panniculitis, pan-
aiteiitis nodosa, nodulai vasculitis, pietibial
myxedema, nonulceiated gumma, and lym-
phoma.
MANACMNI
Symptomatc Bed iest oi compiessive band-
ages (lowei legs), wet diessings.
Aot-IoI|ammatory Ireatmeot Salicylates, non-
steioidal anti-inflammatoiy diugs. Systemic
glucocoiticoids-iesponse is iapid, but theii
use is indicated only when the etiology is
known (and infectious agents aie excluded).
IA8I T-2 Causes of Erythema Nodosum
IoIectoos 0ther
8actera| 0rus
'||ep|ococc+| |u|ec||ou, |u|e|cu|o|, ,e||u|o| 'u||ou+r|de, ||or|de +ud |od|de,
0||e|. 'c|-||c Cc,||c:|- '|-||c, 0|+| cou||+cep||.e
||uce||o|, p|||+co|, !,:|cc 0||e|. r|uoc,c||ue, o|d +||, peu|c||||u,
+||c,|+|e
Fuoa| Ma|oaoces
Cocc|d|o|dor,co|, ||+|or,co|, nod||u +ud uounod||u |,rp|or+,
|||op|+ro|, po|o|||c|o|, |eu|er|+, |eu+| ce|| c+|c|uor+
de|r+|op|,|o|
vra| 0ther
|u|ec||ou rououuc|eo|, |ep+|||| B, o||, '+|co|do|
|e|pe |rp|e\ |u||+rr+|o|, |oWe| d|e+e. u|ce|+||.e
0ther co||||, C|o|u d|e+e
Are||+|, |+|d|+|, +c+||+| Be|(e| d|e+e
+
|o| + ro|e corp|e|e ||| o| e||o|o|c |+c|o| |u E|, ee | kequeu+ e| +|, |u K, wo||| e| +| (ed,). ||cc|:|' |-c||, C--c| !-!:-,
1|| ed, |eW \o||, \cC|+Wn|||, 2003, p 5o9-535
FICk T-23 rythema oodosum |udu|+|ed, .e|, |eude|, |u||+rr+|o|, uodu|e ro||, |u ||e p|e||||+|
|e|ou. |e|ou +|e eeu + |ed, |||de||ued e|,||er+ |u| p+|p+|ed + deepe+|ed uodu|e, |euce ||e de|u+
||ou. |u ||| +9,e+|o|d |er+|e ||e|e W+ +|o |e.e| +ud +||||||| o| ||e +u||e jo|u| |o||oW|u +u uppe| |ep||+|o|,
||+c| |u|ec||ou. I|e |||o+| cu||u|e ,|e|ded |ero|,||c ||ep|ococc|.
|+uu|cu|||| | ||e |e|r ued |o dec|||e d|e+e
W|e|e ||e r+jo| |ocu o| |u||+rr+||ou | |u ||e
u|cu|+ueou ||ue. |u eue|+|, p+uu|cu|||| p|e
eu| + +u e|,||er+|ou o| .|o|+ceou uodu|e |u
||e u|cu|+ueou |+| ||+| r+, |e |eude| o| uo|,
||+| r+, u|ce|+|e o| |e+| W|||ou| c+|||u, +ud
||+| r+, |e o|| o| |+|d ou p+|p+||ou. I|u, ||e
|e|r c:|| dec|||e + W|de pec||ur o|
d|e+e r+u||e|+||ou, +|||ou| d|+uo||c c|ue
c+u |e de||.ed ||or ||e |||o|,, d|||||u||ou, o|
c|+|+c|e||||c o| ||e |e|ou.
Au +ccu|+|e d|+uo| |equ||e +u +rp|e deep
||u ||op, ||+| |ou|d |e+c| doWu |o o| e.eu
|e,oud ||e |+c|+. I|e p+uu|cu||||de +|e c|+|
||ed |||o|o|c+||, + |o|u|+| o| ep|+|, depeud|u
ou W|e|e ||e d|e+e p|oce |e|u. noWe.e|,
+ |+|p d|||uc||ou |e|Weeu ep|+| +ud |o|u|+| |
o||eu uo| po|||e. |+uu|cu|||| r+, +|o |e +o
c|+|ed W||| .+cu||||, |u| |u ro| c+e ||e|e | uo
.+cu||||. A |rp||||ed c|+|||c+||ou o| p+uu|cu||||
| |.eu |u I+||e 1!.
0u|, |d|op+|||c |o|u|+| p+uu|cu|||| (||e|||e|
we|e|C|||||+u d|e+e), p+uc|e+||c p+uu|cu||
||, +ud
+u||||,p|ude||c|euc, p+uu|cu|||| +|e

|||e||, d|cued |e|e. 0||e| d|e+e |u W||c|
p+uu|cu|||| occu| +|e |e|e||ed |o |u ||e |+||e.
|!c||: |||c c:|| (|||) , W||c| occu|

p|edor|u+u||, |u |er+|e !0-o0 ,e+| o| +e, |u|
c+u +|o occu| |u c|||d|ood, r+u||e| + c|op o|
u|cu|+ueou |u||+rr+|o|, +ud |eude| uodu|e,
p||r+|||, ou ||e |oWe| e\||er|||e |u| +|o ou ||e
||uu| +ud e|eW|e|e (||. 12+). 0cc+|ou+||,,
|e|ou c+u ||e+| doWu, d|c|+||u +u o||,
,e||oW||oWu ||qu|d, eue|+||, +ccorp+u|ed |,
r+|+|e, |+||ue, |e.e|, +||||+||+, +ud r,+||+.
I|e|e r+, |e |oc+| uec|o| |u ||e |u||+.|ce|+|
+ud pe||.|ce|+| |+| o| |u|e|u+| o|+u, |uc|ud|u
||e reeu|e||c +ud oreu|+| |+|, pe||c+|d|ur,
+ud p|eu|+. 0|+u |u.o|.ereu| r+, p|eeu|
+ |ep+|ore+|,, +|dor|u+| p+|u, u+ue+, +ud
.or|||u.
I|e e||o|o, | uu|uoWu. ||- r+u, c+e o| |||
|+.e |eeu |ec|+|||ed + o||e| r+u||e|+||ou o|
|o|u|+| p+uu|cu|||| +ud ||e|e | + uoW |oW|u
dou|| ||+| ||| e\|| + +u eu|||,.
|c:-c|: c:|| | c|+|+c|e||/ed c||u|c+||,
|, p+|u|u| e|,||er+|ou uodu|e +ud p|+que ||+|
r+, ||uc|u+|e +ud occu| +| +u, ||e, W||| + p|ed|
|ec||ou |o| +|doreu, |u||oc|, |e (||. 125).
||equeu||, +ccorp+u|ed |, +||||||| +ud po|,e
|o|||. Aoc|+|ed W||| e|||e| p+uc|e+|||| o| p+u
c|e+||c c+|c|uor+. || +||ec| r|dd|e+ed |o e|de||,
|ud|.|du+|, r+|e ro|e o||eu ||+u |er+|e. n|
|o|,. +|co|o||r, +|dor|u+| p+|u, We||| |o, o|
|eceu|oue| d|+|e|e re||||u. '||u ||op, |e.e+|
|o|u|+| p+uu|cu||||, +ud +||e| ||op, ||que||ed |+|
d|+|u ||or ||e ||op, ||e. Ceue|+| e\+r|u+||ou
r+, |e.e+| p|eu|+| e||u|ou, +c||e, +ud +|||||||,
p+|||cu|+||, o| ||e +u||e.
|+|o|+|o|,. eo|uop||||+, |,pe|||p+er|+, |,pe|
+r,|+er|+, +ud |uc|e+ed e\c|e||ou o| +r,|+e
+ud/o| ||p+e |u ||e u||ue. I|e p+||op|,|o|o,
| p|o|+||, + ||e+|doWu o| u|cu|+ueou |+|
c+ued |, eu/,re (+r,|+e, ||,p|u, ||p+e)
|e|e+ed |u|o ||e c||cu|+||ou ||or + d|e+ed p+u
c|e+. I|e cou|e +ud p|ouo| depeud ou ||e
|,pe o| p+uc|e+||c d|e+e. I|e+|reu| | d||ec|ed
+| ||e uude||,|u p+uc|e+||c d|o|de|.
1||,!-|:-:, c:|| | +|o

c|+|+c|e||/ed |, |ecu||eu| |eude|, e|,||er+|ou,
u|cu|+ueou uodu|e |+u|u ||or |o 5 cr
+ud |oc+|ed p|edor|u+u||, ou ||e ||uu| +ud ||e
p|o\|r+| e\||er|||e, .e|, ruc| |||e ||oe |oWu
|u ||. 125. |odu|e ||e+| doWu +ud d|c|+|e
+ c|e+| e|ou o| o||, ||u|d. ||+uo| | u||+u||
+|ed |, + dec|e+e |u ||e |e.e| o| e|ur
+u|||
|,p|u, +ud ||e+|reu| cou|| o| o|+| d+poue |u
doe up |o 200 r/d. I|e |u||+.euou |u|u|ou
o| |ur+u
p|o|e|u+e |u|||||o| couceu||+|e |+

|eeu |oWu |o |e .e|, e||ec||.e.
I|e |e+de| | +|o |e|e||ed |o |.kequeu+ e| +|, |u

K wo||| e| +| (ed). ||cc|:|' |-c||,
C--c| !-!:- , 1|| ed. |eW \o||, \cC|+Wn|||,
2003, p 5o9.
0Ihk FANNICIIII0S |C|9 . 129.!
|C|0 . \19.!
IA8I T-3 Simp|ified C|assification of Pannicu|itis
Lob0|ar Paoo|c0||t|s Septa| Paoo|c0||t|s
|eou+|+| 'c|e|er+ ueou+|o|ur,
ueou+|+| u|cu|+ueou |+| uec|o|
||,|c+| Co|d, ||+ur+
||u |o||e|o|d p+uu|cu|||| E|,||er+ uodour
|d|op+|||c |d|op+|||c |o|u|+| p+uu|cu|||| Eo|uop||||c |+c||||
(||e|||e|we|e|C|||||+u) ,ud|ore Eo|uop||||+ r,+||+ ,ud|ore
|+uc|e+||c w||| p+uc|e+|||| o| c+|c|uor+ o| ||e
p+uc|e+
|+uu|cu|||| W||| o||e| |upu e|,||er+|ou, +|co|do|, 'c|e|ode|r+
,|er|c d|e+e |,rp|or+, ||||oc,||c c,|op|+|c
p+uu|cu||||
w||| .+cu|||| |odu|+| .+cu|||| I||or|op||e||||,
p+u+||e|||| uodo+
\e|+|o||c de||c|euc,
+u||||,p|u de||c|euc,
FICk T-24 Idopathc |obu|ar paoocu|ts o a 5-year-o|d boy A|||ou| |e|ou uu+||, +||e ou
||e |oWe| e\||er|||e, ||e, r+, r+u||e| + c|op o| u|cu|+ueou |u||+rr+|o|, +ud |eude| uodu|e +|o ou
||e ||uu| +ud ou ||e |+ce. uu+||, occu|||u |u |er+|e !0 |o o0 ,e+| o|d, || c+u +|o +ppe+| |u c|||d|eu, +
| ||e c+e W||| ||| |o,. |o e||o|o|c +eu| W+ |ouud. I|e|e W+ |ep+|ore+|,, +ud +|dor|u+| p+|u. I|e
p+||eu| |epouded |o |ucoco|||co|d |u| |+d |ecu||euce
FICk T-25 Faocreatc
paoocu|ts I|e|e +|e ru|
||p|e, p+|u|u|, e|,||er+|ou
uodu|e +ud p|+que ||+| ||uc|u
+|e ou ||e |oWe| e\||er|||e, |u|
|r||+| |e|ou We|e +|o |ouud
ou ||e ||uu| +ud ou ||e |u||oc|
|C | + |+p|d|, e.o|.|u, |d|op+|||c, c||ou|c, +ud
e.e|e|, de|||||+||u ||u d|e+e.
|| | c|+|+c|e||/ed |, ueu||op||||c |u|||||+||ou +ud
de||uc||ou o| ||ue.
|| occu| ro| corrou|, |u +oc|+||ou W||| +
,|er|c d|e+e, epec|+||, c||ou|c u|ce|+||.e
co||||.
|| +|o occu| W||| +|||||||, |er+|o|o|c d,c|+
|+, +ud r+||u+uc, |u| r+, occu| +|o +|oue.
|| | c|+|+c|e||/ed |, ||e p|eeuce o| |||eu|+|,
|o,, ||ue|ed u|ce| W||| uude|r|ued |o|de|
u||ouud|u pu|u|eu| uec|o||c |+e.
I|e|e | uo |+|o|+|o|, |e| ||+| e|+||||e ||e
d|+uo|.
I|e r+|u|+, o| ||e+|reu| +|e |rruuoupp|e
|.e o| rodu|+||u +eu|.
F00kMA CANCkN0SM (FC) |C|9 . o3o.0
|C|0 . |33
FI0MI0I0C
Raie, pievalence unknown. All age gioups af-
fected with a peak between 40 and 60 yeais.
Slight piepondeiance of females.
Unknown. Although called pyodeima, it does
not have a miciobial etiology. PG is counted
among the neutiophilic deimatoses because
of the massive neutiophilic infiltiates within
the skin.
Iwo Iypes tue. acute onset with painful
hemoiihagic pustule oi painful nodule eithei
de novo oi aftei minimal tiauma. C|ront. slow
piogiession with gianulation and hypeikeiato-
sis. Less painful.
Sko Iesoos tue. Supeificial hemoiihagic
pustule suiiounded by eiythematous halo;
veiy painful (Fig. 7-26 ). Bieakdown occuis
with ulcei foimation, wheieby ulcei boideis
aie dusky-ied oi puiple, iiiegulai and
iaised, undeimined, boggy with peifoiations
that diain pus (Fig. 7-26 B ). The base of the
ulcei is puiulent with hemoiihagic exudate,
paitially coveied by neciotic eschai (Fig.
7-27), with oi without gianulation tissue.
Pustules both at the advancing boidei and
in the ulcei base; a halo of eiythema spieads
centiifugally at the advancing edge of the
ulcei (Figs. 7-26 B and 7-27). C|ront. lesions
may slowly piogiess, giazing ovei laige aieas
of the body and exhibiting massive gianulation
within the ulcei fiom the outset (Fig.
7-28) and ciusting and even hypeikeiatosis
on the maigins (Fig. 7-29, page 160).
Lesions aie usually solitaiy but may be
multiple and foim clusteis that coalesce.
Most common sites: lowei extiemities
(Figs. 7-26 B and 7-29) > buttocks > abdomen
(Fig. 7-27) > face (Fig. 7-28). Healing of ulceis
iesults in thin atiophic ciibiifoim scais.
Mucous Membraoes Raiel y, aphthous
stomatitis-like lesions; massive ulceiation of
oial mucosa and conjuctivae.
Ceoera| xamoatoo
Patient may appeai ill.
Assocated Systemc 0seases
Up to 50% of cases occui without associated
disease. Remaindei of cases associated with
laige- and small-bowel disease (Ciohn disease,
ulceiative colitis), diveiticulosis (diveiticulitis),
aithiitis, paiapioteinemia and myeloma, leuke-
mia, active chionic hepatitis, Behet syndiome.
Theie is no single diagnostic test.
Sk Vaiiably elevated.
0ermatopatho|oy Not diagnostic. Neu-
tiophilic inflammation with abscess foimation
and neciosis.
Clinical findings plus histoiy and couise; con-
fiimed by nonspecific deimatopathology show-
ing neutiophilic inflammation with abscesses
and neciosis. Diffeiential diagnosis: ecthyma
and ecthyma gangienosum, atypical mycobac-
teiial infection, clostiidial infection, deep my-
coses, amebiasis, leishmaniasis, biomodeima,
pemphigus vegetans, stasis ulceis, Wegenei
gianulomatosis.
C0kS AN0 Fk0CN0SIS
Untieated, couise may last months to yeais,
but spontaneous healing can occui. Ulceiation
may extend iapidly within a few days oi slowly.
Healing may occui centially with peiipheial ex-
tension. New ulceis may appeai as oldei lesions
iesolve. Patheigy, i.e., slight tiauma initiating
new PG lesion, noted at sites of minoi tiauma,
biopsy, oi needle sticks.
MANACMNI
Wth Assocated oder|yo 0sease Tieat un-
deilying disease.
For FC High doses of oial glucocoiticoids
oi IV glucocoiticoid pulse theiapy (1-2 g/d
piednisolone) may be iequiied. Sulfasalazine
(paiticulaily in cases associated with Ciohn
disease), sulfones, cyclospoiine, and, moie ie-
cently, infliximab, etaneicept, adalimumab have
been shown to be effective in uncontiolled
studies.
Iopca| In singulai lesion, topical taciolimus
ointment oi intialesional tiiamcinolone.
FICk T-26 Fyoderma aoreoo-
sum I|e |u|||+| |e|ou | + |ero|
||+|c uou|o|||cu|+| pu|u|e u||ouuded |,
+u e|,||er+|ou |+|o +ud | .e|, p+|u|u|.
|e|ou |+p|d|, eu|+|e +ud ||e+| doWu
ceu||+||, + |oWu |e|e. |o|e. e|,||er+
|ou |+|o +|ouud ||e |ud|.|du+| |e|ou.
I|e|e | +ccorp+u,|u eder+ o| ||e ||||
|oo|. |e|ou +|e e\||ere|, p+|u|u|.

FICk T-2T Fyoderma aoreoosum A .e|, |+|e u|ce| W||| |+|ed |u||ou uude|r|ued |o|de| co.e|ed
W||| |ero|||+|c +ud |||||uou e\ud+|e. w|eu ||e |u||+e +|e opeued, pu | d|+|ued. I|| |e|ou +|oe +cu|e|,
+ud p|e+d |+p|d|, |o||oW|u |+p+|+|or, |o| +u o.+||+u c+|c|uor+.
FICk T-28 Fyoderma aoreoosum: chrooc type I|e |e|ou |u.o|.e ||e uppe| e,e||d +ud |ep|eeu|
+u u|ce| W||| e|e.+|ed |+uu|+||u |+e W||| ru|||p|e +|cee. I|e |e|ou |+|e| p|e+d |oW|, |o |u.o|.e ||e
|erpo|+| +ud /,or+||c |e|ou +ud e.eu|u+||, |e+|ed uude| ,|er|c |ucoco|||co|d ||e+|reu|, |e+.|u + |||u
c||||||o|r c+| ||+| d|d uo| |rp+|| ||e |uuc||ou o| ||e e,e||d.
Ae oI 0oset Most 30-60 yeais.
Sex Women > men.
to|oy Unknown, possibly hypeisensitivity
ieaction. Inflammatoiy bowel disease. SS is
counted among the neutiophilic deimatoses.
Assocated 0sorders Febiile uppei iespiia-
toiy tiact infection. In some cases associated
with Yersna infection. Hematologic malig-
nancy; diugs: gianulocyte colony-stimulating
factoi (G-GSF).
Piodiomes aie febiile uppei iespiiatoiy tiact
infections. Gastiointestinal symptoms (di-
aiihea), tonsillitis, influenza-like illness, 1-3
weeks befoie skin lesions. Lesions tendei/pain-
ful. Fevei (not always piesent), headache, ai-
thialgia, geneial malaise.
Sko Iesoos Biight ied, smooth, tendei
papules (2-4 mm in diametei) that coalesce to
foim iiiegulai, shaiply boideied, inflammatoiy
plaques (Fig. 7-30 ). Pseudovesiculation:
intense edema gives the appeaiance of
vesiculation (Figs. 7-30 and 7-31 ). Lesions
Au uucorrou, +cu|e +ud |ecu||eu|, c,|o||ue
|uduced ||u |e+c||ou +oc|+|ed W||| .+||ou
e||o|o|e.
|+|u|u| p|+que|o|r|u |u||+rr+|o|, p+pu|e,
o||eu W||| r+|.e e\ud+||ou |.|u ||e +ppe+|
+uce o| .e|cu|+||ou (peudo.e|cu|+||ou).
Aoc|+|ed W||| |e.e|, +||||+||+, +ud pe||p|e|+|
|eu|oc,|o|.
Aoc|+|ed W||| |u|ec||ou, r+||u+uc,, o| d|u.
I|e+|reu|. ,|er|c |ucoco|||co|d, po|+|ur
|od|de, d+poue, o| co|c||c|ue.
',,. Acu|e |e||||e ueu||op||||c de|r+|o|.
SWI SN0k0M (SS) |C|9 . o95.39
|C|0 . |93.2
FICk T-29 Fyoderma aoreoosum: chrooc type I|| |e|ou, W||c| +ppe+| |||e + p|+que, p|e+d
ou|, |oW|, |u| W+ +|o u||ouuded |, +u e|,||er+|ou |o|de|. I|e |e|ou | c|u|ed +ud |,pe||e|+|o||c +ud |
|e p+|u|u| ||+u ||e |e|ou |u +cu|e p,ode|r+ +u|euour
FICk T-30 Sweet syodrome Au e|,||er+|ou, eder+|ou p|+que ||+| |+ |o|red ||or co+|ec|u
p+pu|e ou ||e |||| c|ee|. I|e |o|de| o| ||e p|+que |oo| + || corpoed o| .e|c|e, |u| p+|p+||ou |e.e+| ||+|
|| | o||d (peudo.e|cu|+||ou). I|| |e|ou occu||ed |u + 2o,e+|o|d |er+|e |o||oW|u +u uppe| |ep||+|o|, |u|ec
||ou, +ud ||e p+||eu| +|o |+d |e.e| +ud |eu|oc,|o|. A |r||+| e|up||ou |u + 52,e+|o|d |er+|e. |o|e. ru|||p|e
co+|ec|u, |u||+rr+|o|, +ud .e|, e\ud+||.e p+pu|e ou ||e uec|. I|| p+||eu| +|o |+d |eu|oc,|o| +ud |e.e|. |u
+dd|||ou, ||e|e | .|||||o.

FICk T-31 Sweet syodrome Co+|ec|u e\ud+||.e p+pu|e ||+| |oo| |||e .e|c|e. upou p+|p+||ou
|e|ou We|e o||d. Bu||ou |,pe o| 'Wee| ,ud|ore. I|ee +|e ||ue |u||+e +ud pu|u|e. I|e p+||eu| |+d
r,e|orouoc,||c |eu|er|+.
aiise iapidly, and as they evolve, cential cleaiing
may lead to annulai oi aicuate patteins. Tiny,
supeificial pustules may occui. If associated
with leukemia, bullous lesions may occui (Fig.
7-31B ) and lesions may mimic pyodeima
gangienosum. May piesent as a single lesion
oi multiple lesions, asymmetiically distiibuted.
Most common on face (Fig. 7-30 ), neck (Fig.
7-30B ), and uppei extiemities but also on lowei
extiemities, wheie Iesions may be deep in the
fat and thus mimic panniculitis oi eiythema
nodosum. Tiuncal lesions aie uncommon but
widespiead, and geneialized foims occui.
Mucous Membraoes Conjunctivitis, epis-
cleiitis.
Ceoera| xamoatoo
Patient may appeai ill. Theie may be involve-
ment of caidiovasculai, cential neivous system,
gastiointestinal, hepatic, musculoskeletal, ocu-
lai, pulmonaiy, ienal, and splenic oigans.
Comp|ete 8|ood Couot Leukocytosis with neu-
tiophilia (not always peisistent).
Sk Elevated.
0ermatopatho|oy Diagnostic. Epideimis
usually noimal but may show subcoineal pus-
tulation. Massive edema of papillaiy body,
dense leukocytic infiltiate with staibuist pat-
tein in mid-deimis, consisting of neutiophils
with occasional eosinophils/lymphoid cells.
Leukocytoclasia, nucleai dust, but othei signs
of vasculitis absent. Neutiophilic infiltiates in
subcutaneous tissue.
Clinical impiession plus skin biopsy confiima-
tion.
very dematous Acute F|aques Eiythema
multifoime, eiythema nodosum, pievesiculai
heipes simplex infection, pieulceiative pyo-
deima gangienosum.
C0kS AN0 Fk0CN0SIS
Untieated, lesions enlaige ovei a peiiod of
days oi weeks and eventually iesolve without
scaiiing. With oial piednisone, lesions iesolve
within a few days. Recuiiences occui in 50%
of patients, often in pieviously involved sites.
Some cases follow Yersna infection oi aie as-
sociated with acute myelocytic leukemia, tian-
sient myeloid piolifeiation, vaiious malignant
tumois, ulceiative colitis, benign monoclonal
gammopathy; some follow diug administia-
tion, most commonly by GSF.
MANACMNI
Rule out sepsis.
Fredosooe 30-50 mg/d, tapeiing in 2-3
weeks; some, but not all, patients iespond to
dapsone, 100 mg/d, oi to potassium iodide.
Some to colchicine.
Aotbotc Iherapy Cleais eiuption in Yersna-
associated cases; in all othei cases antibiotics
aie ineffective.
A |+|e, |oc+||/ed |u||+rr+|o|, d|e+e o| uu|uoWu
e||o|o,, c||u|c+||, c|+|+c|e||/ed |, |edd||||oWu
p+pu|e o| r+|| p|+que p||r+|||, |u ||e |+ce.
'|u|e o| ru|||p|e |e|ou W||| c|+|+c|e||||c o|
+ue pee||||e u||+ce (||. 1!2).
n||o|o|c+||,, c||ou|c |eu|oc,|oc|+||c .+cu||||
W||| eo|uop|||, |||||u depo|||ou, +ud ||||o|.
I|e|+p,. |op|c+| |ucoco|||co|d, d+poue.
CkANI0MA FACIAI (CF) |C|9 . o3o.
|C|0 . |92.2
FICk T-32 Craou|oma Iaca|e: c|assc preseotatoo A |u|e, |+|p|, de||ued, ||oWu p|+que W||| +
c|+|+c|e||||c o|+ue pee||||e u||+ce.
164
S E C I | 0 N 8
Svk AN0 IIF-IhkAININC
SkIN kFII0NS IN Ih
ACII III FAIINI
EE' | + e||ou, +| ||re |||e|||e+|eu|u, |e+c||ou
p+||e|u o| ||e ||u c|+|+c|e||/ed |, eue|+||/ed
+ud uu||o|r |edue +ud c+||u |u.o|.|u p|+c||
c+||, ||e eu|||e ||u.
|| | +oc|+|ed W||| |e.e|, r+|+|e, ||.e|, +ud
eue|+||/ed |,rp|+deuop+||,, +ud |e.e|.
IWo |+e, +cu|e +ud c||ou|c, re|e oue |u|o ||e
o||e|. |u ||e +cu|e +ud u|+cu|e p|+e, ||e|e |
|+p|d oue| o| eue|+||/ed .|.|d |ed e|,||er+
+ud ||ue ||+uu, c+|e, ||e p+||eu| |ee| |o| +ud
co|d, ||.e|, +ud |+ |e.e|. |u c||ou|c EE', ||e
||u |||c|eu, +ud c+||u cou||uue +ud |ecore
|+re||+|.
I|e|e | + |o o| c+|p +ud |od, |+||, +ud ||e
u+|| |ecore |||c|eued +ud ep+|+|ed ||or ||e
u+|| |ed (ou,c|o|,|).
I|e|e r+, |e |,pe|p|reu|+||ou o| p+|c|, |o
o| p|reu| |u p+||eu| W|oe uo|r+| ||u co|o| |
||oWu o| ||+c|.
I|e ro| ||equeu| p|ee\|||u ||u d|o|de| +|e
(|u o|de| o| ||equeuc,) po||+|, +|op|c de|r+
||||, +d.e|e cu|+ueou d|u |e+c||ou, |,rp|or+,
+||e||c cou|+c| de|r+||||, +ud p||,||+| |u||+
p||+||.
|C|9. o95.9
XF0IIAIIv kIhk00kMA SN0k0M (S)
FI0MI0I0C
Ae oI 0oset Usually >50 yeais; in childien,
EES usually iesults fiom pityiiasis iubia pilaiis
oi atopic deimatitis.
II0I0C
Some 50% of patients have histoiy of pieex-
isting deimatosis, which is iecognizable only
in the acute oi subacute stage. Most fiequent
pieexisting skin disoideis aie (in the oidei
of fiequency) psoiiasis, atopic deimatitis, ad-
veise cutaneous diug ieactions, cutaneous T
cell lymphoma, alleigic contact deimatitis, and
pityiiasis iubia pilaiis (Table 8-1). Diugs most
commonly implicated in EES aie shown in
Table 8-2. In 20% of patients it is not possible to
identify the cause by histoiy oi histology.
'ee ''e/+|, ',ud|ore ('ec||ou 20) |o| + pec|+| cou|de|+||ou o| ||| |o|r o| EE'.|
IA8I 8-1 Etio|oy of E\fo|iative 0ermatitis
in Adu|ts
Cause
o
Averae Ferceot
b
uude|e|r|ued o| uuc|+|||ed 2!
|o||+| 2!
A|op|c de|r+||||, ec/er+ o
||u +||e|, 5
|,rp|or+, |eu|er|+
A||e||c cou|+c| de|r+|||| 5
'e|o|||e|c de|r+|||| 5
'|+| de|r+|||| W||| '|d |e+c||ou !
|||,||+| |u||+ p||+|| 2
|erp||u |o||+ceu
c
|o| + corp|e|e ||| o| d|e+e +oc|+|ed W||| EE', ee e\|o||+||.e
de|r+|||| p|c|u|e +||e|, |u ||e ou||ue .e||ou.
|
A co||+|ed ||or ||e |||e|+|u|e.
'ou|ce. A|||e.|+|ed ||or \n l|| e| +|, |u |\ ||eed|e| e| +| (ed).
||cc|:| |-c||, C--c| !-!:- o|| ed. |eW \o||,
\cC|+Wn|||, 200!.
SCII0N 8 'E\EkE A|| |||EInkEAIE|||C 'K|| Eku|I|0|' || InE ACuIE|\ ||| |AI|E|I 165
FAIh0CNSIS
The metabolic iesponse to exfoliative deima-
titis may be piofound. Laige amounts of waim
blood aie piesent in the skin due to the dilata-
tion of capillaiies, and theie is consideiable
heat dissipation thiough insensible fluid loss
and by convection. Also, theie may be high-
output caidiac failuie; the loss of scales thiough
exfoliation can be consideiable, up to 9 g/m
2
of
body suiface pei day, and this may contiibute to
the ieduction in seium albumin and the edema
of the lowei extiemities so often noted in these
patients.
Depending on the etiology, the acute phase
may develop iapidly, as in a diug ieaction, lym-
phoma, eczema, oi psoiiasis. At this eaily acute
stage it is still possible to identify the pieexist-
ing deimatosis. Theie is fevei, piuiitus, fatigue,
weakness, anoiexia, weight loss, malaise, feeling
cold, shiveis.
Appearaoce oI Fateot Fiightened, ied, toxic,"
may be malodoious.
Sko Iesoos Skin is ied, thickened, scaly.
Deimatitis is unifoim involving the entiie body
suiface (Figs. 8-1 to 8-3), except foi pityiiasis
IA8I 8-2 0rus that Cause E\fo|iative 0ermatitis
A||opuroo|
b
Code|ue \e|cu||+| 'u||++|+/|ue
Ar|uo|,co|de C,+u+r|de \eu+ 'u||ou+r|de
Ar|uop|,|||ue |+poue \e||,|p|edu|o|oue 'u||ou,|u|e+
Ar|od+|oue ||deo\,|uo|ue \|uoc,c||ue
Arou+||de ||||uu|+| \||or,c|u C I+| p|ep+|+||ou
Arp|c||||u ||p|eu,||,d+u|o|u 0rep|+/o|e Ie|||u+||ue
Au||r+|+||+| Ep|ed||ue |eu|c||||u Ie||u|+||ue
A|eu|c+| E||+r|u|o| |eu|o|+||u I|+||dor|de
Ap|||u E||,|eued|+r|ue |e||||+|e +ud I||+ce|+/oue
|,ce|,| |||u|||+|e
A/||eou+r E||e||u+|e ||eue|u||de I||+/|de d|u|e||c
B+c|||r ||uo|ou|+c|| ||euo|p|||+|e|u I|c|op|d|ue
B+||||u|+|e C\C'| ||euo|||+/|ue I|ro|o| r+|e+|e
B|orodeo\,u||d|ue Co|d ||eu,||u|+/oue e,ed|op
Budeuo|de ne||+| red|c+||ou ||eu,|o|u Io||+r,c|u
Ca|cum chaooe| |ude|o\+/|ue ||o|o||e|+p, Ioc+|u|de
b|ockers |,d|oc||o||de
C+p|op||| |ud|u+.|| ||+queu|| I||re||e\+|e
Carbamatepoe |u|e||eu||u 2 ||+c|o|o| I|o.+||o\+c|u
C+||op|+||u |od|ue uodoe Iuro| uec|o|
Ce|o\|||u |ou|+/|d k+u|||d|ue |+c|o|
Cep|+|opo||u |oo|||de d|u|||+|e ke||uo|d \+ucor,c|u
Cmetdoe |+uop|+/o|e k||o|+r,c|u \o||r||ue
C|p|+||u ||doc+|ue k||+rp|c|u /|do.ud|ue
C|od|ou+|e Ithum '|. lo|u' Wo||
C|o|+/+r|ue \e||oqu|ue '||ep|or,c|u
c
|o| + ro|e e\|eu|.e ||| o| d|u |rp||c+|ed |u EE', ee e\|o||+||.e de|r+|||| ,ud|ore p|c|u|e +||e|, |u ||e ou||ue .e||ou.
|
I|e ro|e corrou|, |rp||c+|ed +eu| +|e |||ed |u |o|d.
'ou|ce. \n l||, A K|r,+|A+d|, +ud |\ ||eed|e|, |u |\ ||eed|e| e| +|. (ed). ||cc|:| |-c||, C--c| !-!:-, o|| ed.
|eW \o||. \cC|+Wn|||, 200!. p.+31.
iubia pilaiis, wheie EES spaies shaiply defined
aieas of noimal skin (see Fig. 5-7). Thickening
leads to exaggeiated skin folds (Figs. 8-2 and
8-3); scaling may be fine and bianny and may
be baiely peiceptible (Fig. 8-2) oi laige, up to
0.5 cm, and lamellai (Fig. 8-1).
Pu|ms und Sv|es Usually involved, with mas-
sive hypeikeiatosis and deep fissuies in pit-
yiiasis iubia pilaiis, Szaiy syndiome, and
psoiiasis.
har Telogen effluvium, even alopecia, except
foi EES aiising in eczema oi psoiiasis.
Na|s Thickening of nail plates, onycholysis,
shedding of nails.
Fmeotatoo In chionic EES theie may be
hypeipigmentation oi patchy loss of pigment in
patients whose noimal skin is biown oi black.
Ceoera| xamoatoo
Lymph nodes geneialized, iubbeiy, and usually
small; enlaiged in Szaiy syndiome. Edema of
lowei legs and ankles.
Chemstry Low seium albumin and inciease
in gammaglobulins; electiolyte imbalance;
acute-phase pioteins incieased.
hemato|oy Leukocytosis.
8actera| Cu|ture S|n : iule out secondaiy
Sa|y|otottus aureus infection. B|ooJ : iule out
sepsis.
0ermatopatho|oy Depends on type of un-
deilying disease. Paiakeiatosis, intei- and intia-
cellulai edema, acanthosis with elongation of
the iete iidges, and exocytosis of cells. Theie is
edema of the deimis and a chionic inflamma-
toiy infiltiate.
Imao CT scans oi MRI should be used to
find evidence of lymphoma.
Iymph Node 8opsy When theie is suspicion
of lymphoma.
0IACN0SIS
Diagnosis is not easy, and the histoiy of the
pieexisting deimatosis may be the only clue.
Also, pathognomonic signs and symptoms
of the pieexisting deimatosis may help, e.g.,
dusky-ied coloi in psoiiasis and yellowish-ied
in pityiiasis iubia pilaiis; typical nail changes
of psoiiasis; lichenification, eiosions, and ex-
coiiations in atopic deimatitis and eczema;
diffuse, ielatively nonscaling palmai hypei-
keiatoses with fissuies in cutaneous T cell
lymphoma (CTCL) and pityiiasis iubia pilaiis;
shaiply demaicated patches of noninvolved
skin within the eiythiodeima in pityiiasis
iubia pilaiis; massive hypeikeiatotic scale of
scalp, usually without haii loss in psoiiasis and
with haii loss in CTCL and pityiiasis iubia
pilaiis; in the lattei and in CTCL, ectiopion
may occui.
C0kS AN0 Fk0CN0SIS
Guaided, depends on undeilying etiology. De-
spite the best attention to all details, patients
may succumb to infections oi, if they have cai-
diac pioblems, to caidiac failuie (high-output"
failuie) oi, as was often the case in the past, to
the effects of piolonged glucocoiticoid theiapy.
MANACMNI
This is an impoitant medical pioblem that
should be dealt with in a modein inpatient dei-
matology facility with expeiienced peisonnel.
The patient should be hospitalized in a single
ioom, at least foi the beginning woikup and
duiing the development of a theiapeutic pio-
giam. The hospital ioom conditions (heat and
cold) should be adjusted to the patient's needs;
most often these patients need a waim ioom
with many blankets.
Iopca| Watei baths with added bath oils, fol-
lowed by application of bland emollients.
Systemc Oial glucocoiticoids foi iemission
induction but not foi maintenance; sysemt
anJ ota| |eray as requreJ |y unJer|yng
tonJon.
Supportve Suppoitive caidiac, fluid, electio-
lyte, piotein ieplacement theiapy as iequiied.
SCII0N 8 'E\EkE A|| |||EInkEAIE|||C 'K|| Eku|I|0|' || InE ACuIE|\ ||| |AI|E|I 16T
FICk 8-1 xIo|atve dermatts: psorass I|e|e | uu|.e|+| e|,||er+, |||c|eu|u o| ||e ||u, +ud
|e+., c+||u. I|| p+||eu| |+d po||+| + ue|ed |, ||e |+|e ||.e|, W|||e c+|e +ud ||e c+|p +ud u+||
|u.o|.ereu| uo| eeu |u ||| |||u||+||ou. I|e p+||eu| |+d |+||ue, We+|ue, r+|+|e, +ud W+ ||.e||u. || | qu||e
o|.|ou ||+| uc| r+|.e c+||u c+u |e+d |o p|o|e|u |o +ud ||e r+\|r+| d||+|+||ou o| ||u c+p|||+||e |o cou|d
e|+||e |e+| d||p+||ou +ud |||ou|pu| c+|d|+c |+||u|e
FICk 8-2 xIo|atve dermatts: dru-oduced I|| | eue|+||/ed e|,|||ode|r+ W||| |||c|eu|u o|
||u |eu|||u |u |uc|e+ed ||u |o|d, uu|.e|+| |edue, + ||ue ||+Wu, c+||u. I|| p+||eu| |+d de.e|oped e|,|||o
de|r+ |o||oW|u ||e |ujec||ou o| o|d +|| |o| ||eur+|o|d +|||||||.
SCII0N 8 'E\EkE A|| |||EInkEAIE|||C 'K|| Eku|I|0|' || InE ACuIE|\ ||| |AI|E|I 169
FICk 8-3 xIo|atve dermatts: cutaoeous I ce|| |ymphoma I|e|e | uu|.e|+| e|,||er+, |||c|eu|u,
+ud c+||u. |o|e ||+| |u cou||+| |o e|,|||ode|r+ |oWu |u ||. 3 +ud 32 ||e de|ee o| e|,||er+ +ud |||c|
ue | uo| uu||o|r +ud ||e |edue |+ + ||oWu|| |ue. |u +dd|||ou, ||| e|de||, p+||eu| |+d |+|| |o, r+|.e
|u.o|.ereu| o| p+|r +ud o|e W||| d|||ue |,pe||e|+|oe, c|+c|, +ud ||u|e. Ceue|+||/ed |,rp|+deuop+||,
W+ +|o p|eeu|.
FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E' 1T0
I|e uddeu +ppe+|+uce o| + |+| +ud |e.e| c+ue
+u\|e|, |o| ||e p+||eu|. \ed|c+| +d.|ce | ou||
|rred|+|e|, +ud o||eu |u ||e ere|euc, uu||
o| |op||+|, +|ou| 0 o| +|| p+||eu| ee||u
ere|euc, red|c+| c+|e |+.e + de|r+|o|o|c
p|o||er.
I|e d|+uo| o| +u +cu|e |+| W||| + |e.e| | +
c||u|c+| c|+||eue (||. 3+ +ud 35) || + d|+uo|
| uo| e|+||||ed p|orp||, |u ce||+|u p+||eu|
e.., ||oe |+.|u ep||cer|+ (||. 3o)|, |||e+.|u
||e+|reu| r+, |e de|+,ed.
I|e cu|+ueou ||ud|u +|oue +|e o||eu d|+uo||c
|e|o|e cou|||r+|o|, |+|o|+|o|, d+|+ +|e +.+||+||e.
A |u p|o||er o| ||e +cu|e +|doreu, ||e |eu||
o| ore |+|o|+|o|, |e|, uc| + r|c|o||o|o|c
cu||u|e, r+, uo| |e +.+||+||e |rred|+|e|,. 0u
||e |+| o| + d|||e|eu||+| d|+uo|, +pp|op||+|e
||e|+p,-W|e||e| +u||||o||c o| |ucoco|||co|d-
r+, |e |+||ed. |u|||e|ro|e, p|orp| d|+uo|
+ud |o|+||ou o| ||e p+||eu| W||| + cou|+|ou
d|e+e, W||c| r+, |+.e e||ou couequeuce,
p|e.eu| p|e+d |o o||e| pe|ou. |o| e\+rp|e,
.+||ce||+ |u +du|| (ee ||. 21!9 +ud 21+0)
|+|e|, c+u |e |+|+|. Cou|+|ou d|e+e p|eeu|
|u W||| |+| +ud |e.e| + ||e r+jo| ||ud|u
|uc|ude .c| |-:| (||. 35), eu|e|o.||u, +ud
p+|.o.||u |u|ec||ou (ee ||. 212+ 1 3) +ud
|c:|-c| |-:| ||ep|ococc+| (ee ||. 2++3),
|+p|,|ococc+| (ee ||. 2++0), reu|uococc+|
(ee ||. 2+50, 2+5), |,p|o|d, +ud ,p|||| (ee
||. !02)|.
I|e p|,|c+| d|+uo| o| ||u e|up||ou | + d|c|
p||ue |+ed r+|u|, ou p|ec|e |deu||||c+||ou o| ||e
|,pe o| ||u |e|ou. I|e p|,|c|+u ru| uo| ou|,
|deu|||, +ud c|+||, ||e |,- o| ||u |e|ou |u|
+|o |oo| |o| +dd|||ou+| ro|p|o|o|c c|ue uc| +
||e :|c| (+uuu|+|! |||!) o| ||e |ud|.|du+|
|e|ou, ||e cc--| (/o|e|||o|r! ||ue+|!)
o| ||e |e|ou, ||e !||| c||- (e\poed
+|e+! ceu|||pe|+| o| ceu||||u+|! rucou rer
||+ue!).
|u ||e !||--|c| !c o| e\+u||er || |
|rpo||+u| |o de|e|r|ue, |, |||o|,, ||e |- |
|| c-cc:- ||e |+| o| koc|, \ouu|+|u
po||ed |e.e| c|+|+c|e||||c+||, +ppe+| |||| ou ||e
W||| +ud +u||e (ee ||. 2o, 2o2)|, +ud .e|,
|rpo||+u| | ||e |-c| -.|| o| ||e |+|.
\e+|e (ee ||. 35 +ud 2122) p|e+d ||or
|e+d |o |oe |u + pe||od o| ! d+,, W|||e ||e |+|
o| |u|e||+ (ee ||. 212) p|e+d |+p|d|, |u 2+
|o +3 | ||or |e+d |o |oe +ud ||eu equeu||+||,
c|e+|-|||| |+ce, ||eu ||uu|, +ud ||eu ||r|.|
A|||ou| ||e|e r+, |e ore o.e||+p, ||e d||
|e|eu||+| d|+uo||c po|||||||e r+, |e |ouped
|u|o ||.e r+|u c+|eo||e +cco|d|u |o ||e |,pe o|
|e|ou (I+||e 3!).
kAShS IN Ih ACII III F8kII FAIINI
IA80kAI0k ISIS AvAIIA8I F0k ICk
0IACN0SIS
The physician should make use of the following
laboiatoiy tests immediately oi within 8 h:
1 Dret smear [rom |e |ase o[ a est|e. This
pioceduie, known as the T:ant| es , is de-
sciibed in the Intioduction. Smeais aie
examined foi acantholytic cells, giant acan-
thocytes, and/oi multinucleated giant cells
(see Fig. 27-27).
2 Vra| tu|ure , negative stain (election micio-
scopy), polymeiase chain ieaction foi infec-
tions with heipes viiuses, diiect fluoiescence
(DIF) technique.
3 Cram san o[ asraes or strang . This is es-
sential foi piopei diagnosis of pustules. Oi-
ganisms can be seen in the lesions of acute
meningococcemia, iaiely in the skin lesions
of gonococcemia and ecthyma gangienosum.
4 Tout| rearaon . This is especially helpful
in deep fungal infections and leishmaniasis.
The deimal pait of a skin biopsy specimen is
touched iepeatedly to a glass slide; the touch
piepaiation is mmeJae|y fixed in 95% ethyl
alcohol. Special stains aie then peifoimed,
and the slide examined foi oiganisms in the
cytology laboiatoiy.
5 Bosy o[ |e s|n |eson. All puipuiic lesions
should be biopsied. Inflammatoiy deimal
nodules and most ulceis should be biopsied
(at base and maigin) and a poition of tissue
minced and cultuied foi bacteiia and fungi. A
3- to 4-mm tiephine and local anesthesia aie
used. In many laboiatoiies the biopsy speci-
men can be piocessed within 8 h if necessaiy.
In gangienous cellulitis (see Section 24) fio-
zen sections of a deep biopsy will veiify the
diagnosis in minutes.
SCII0N 8 'E\EkE A|| |||EInkEAIE|||C 'K|| Eku|I|0|' || InE ACuIE|\ ||| |AI|E|I 1T1
FICk 8-4 Ceoera|ted Ixed dru eruptoo: tetracyc|oe. ||o||+|ed, 59,e+|o|d Wor+u W||| |e.e|.
\u|||p|e cou||ueu| .|o|+ceou |ed e|,||er+|ou +|e+, ore o| W||c| |+|e| |ec+re |u||ou.
FICk 8-5 Ceoera|ted rash wth Iever: meas|es \ouu Wor+u W||| ||| |e.e|, cou|, coujuuc||
.|||, +ud + cou||ueu| r+cu|op+pu|+| e|up||ou |u ||e eder+|ou |+ce. I|e |+| +|o |u.o|.e ||e ||uu| +ud ||e
e\||er|||e. I|e p+||eu| |+ re+|e.
6 B|ooJ anJ urne examnaons . Blood cultuie,
iapid seiologic tests foi syphilis, and seiology
foi lupus eiythematosus iequiie 24 h. Exami-
nation of uiine sediment may ieveal ied cell
casts in alleigic vasculitis.
7 Dar|-[e|J examnaon . In the skin lesions
of secondaiy syphilis, iepeated examination
IA8I 8-3 Cenera|ited Eruptions in the Acute|y ||| Patient: 0ianosis Accordin to Iype of Lesion
c
Ceoera|ted Ceoera|ted 0seases MaoIested
Ceoera|ted ruptoos ruptoos MaoIested by Wdespread
Ceoera|ted ruptoos ruptoos MaoIested by by Furpurc Macu|es, rythema Fapu|es
MaoIested by MaoIested by vesc|es, 8u||ae, Furpurc Fapu|es, Fo||owed by
Macu|es, Fapu|es Whea|s, F|aques or Fustu|es or Furpurc vesc|es 0esquamatoo
||u |,pe|eu|||.|||e 'e|ur |c|ue ||u ||u |,pe|eu|||.|||e ||u |,pe|eu|||.|||e
Acu|e n|\ ,ud|ore 'Wee| ,ud|ore |,pe|eu|||.|||e \eu|uococcer|+
|
'|+p|,|ococc+|
E|,||er+ |u|ec||our Acu|e u|||c+||+ A||e||c cou|+c| (+cu|e o| c||ou|c) c+|ded||u
(p+|.o.||u B9) E|,||er+ de|r+|||| ||or Couococcer|+
|
,ud|ore
C,|ore+|o.||u, r+||u+|ur p|+u| '|+p|,|ococcer|+ Io\|c |oc| ,ud|ore
p||r+|, |u|ec||ou k|c|e|||+|po\ |-!c K+W++|| ,ud|ore
Ep|e|uB+|| .||u, Couococcer|+ |+c|e|er|+ C|+||.e|u|o|
p||r+|, |u|ec||ou \+||ce||+ 'u|+cu|e |+c|e||+| |e+c||ou
E\+u||er u|||ur (c||c|eupo\)
:
eudoc+|d||| E|,|||ode|r+
(nn\ o) Ec/er+ |e|pe||cur
:
Eu|e|o.||u |u|ec||ou (e\|o||+||.e
Eu|e|o.||u |u|ec||ou (ec|o.||u, Co\+c||e) de|r+||||)
\e+|e (|u|eo|+) (Co\+c||e), k|c|e|||+| d|e+e.
Ce|r+u re+|e (|u|e||+)
!
|uc|ud|u |+ud|oo| koc|, \ouu|+|u
Eu|e|o.||u |u|ec||ou +udrou|| d|e+e po||ed |e.e|
(ec|o.||u +ud Co\+c||e) Io\|c ep|de|r+| I,p|u, |oue|o|ue
Adeuo.||u |u|ec||ou uec|o|,| (ep|der|c)
'c+||e| |e.e| 'r+||po\ o| .+||o|+ 'A||e||c .+cu||||
|
E||||c||o| '|+p|,|ococc+| ||er|u+|ed
I,p|o|d |e.e| c+|ded||u |u||+.+cu|+|
'ecoud+|, ,p|||| ,ud|ore co+u|+||ou (pu|pu|+
I,p|u, ru||ue (euder|c) E|,||er+ ru||||o|re |u|r|u+u
|
)
koc|, \ouu|+|u po||ed K+W++|| d|e+e l| |u|ec||ou
|e.e| (e+||, |e|ou)
!
.ou /ur|uc|
0||e| po||ed |e.e| pu|u|+| po||+|
||er|u+|ed deep |uu+| Acu|e |+||.e|u
|u|ec||ou |u |rruuo |o| |e+c||ou
corp|or|ed p+||eu|
E|,||er+ ru||||o|re
',|er|c |upu e|,||er+|ou
Acu|e |+||.e|u|o| |e+c||ou
of papules may show Treonema a||Jum .
The daik-field examination is not ieli-
able in the mouth because nonpathogenic
oiganisms aie almost impossible to dif-
feientiate fiom T. a||Jum , but a lymph
node aspiiate can be subjected to daik-field
examination.
c
w||| |e+|d |o ||e de|+||ed ro|p|o|o|e, ||e |e+de| |
|e|e||ed |o ||e
|epec||.e ec||ou.
|
0||eu p|eeu| + |u|+|c|.
:
ur||||c+|ed .e|c|e.
!
\+, |+.e +||||+||+ o| rucu|o|e|e|+| p+|u.
-
|e+d|u |o |+|e +|e+ o| ||+c| uec|o|.
0FINIII0N
TEN is a maximal vaiiant of SJS diffeiing only
in the extent of body suiface involvement.
Both can stait with maculai and taiget-like like
lesions; howevei, about 50% of TEN cases do
'l' +ud IE| +|e +cu|e |||e|||e+|eu|u ruco
cu|+ueou |e+c||ou c|+|+c|e||/ed |, e\|eu|.e
uec|o| +ud de|+c|reu| o| ||e ep|de|r|.
I|e, +|e e.e|e .+||+u| o| +u |deu||c+| p+||o|o|c
p|oce +ud d|||e| ou|, |u ||e pe|ceu|+e o| |od,
u||+ce |u.o|.ed.
E|||e| |d|op+|||c o| d|u|uduced.
|+||orec|+u|r | W|dep|e+d +pop|o| o| |e
|+||uoc,|e |uduced |, + ce||red|+|ed c,|o|o\|c
|e+c||ou.
Cou||ueu| e|,||er+|ou pu|pu||c +ud |+|e||||e
r+cu|e e.o|.e |u|o ||+cc|d ||||e| +ud ep|de|r+|
de|+c|reu| ro||, ou ||e ||uu| +ud e\||er|
||e, +ud ||e|e | +oc|+|ed rucou rer||+ue
|u.o|.ereu|.
n||op+||o|o|c+||,. |u|||||c|ue uec|o| o| ||e
ep|de|r| +ud + p+|e |,rp|oc,||c |u|||||+|e.
I|e+|reu| | ,rp|or+||c. ',|er|c ||e+|reu|
W||| |ucoco|||co|d +ud |||doe |u||+.euou
|rruuo|o|u||u | cou||o.e||+|.
SIvNS-l0hNS0N SN0k0M (SlS) AN0 I0XIC FI0kMAI
NCk0ISIS (IN) |C|9 . o95.
|C|0 . |5./5.2
FICk 8-6 Ceoera|ted purpura oecross aod Iever: 0IC 5+,e+|o|d Wor+u W||| |e.e|, p|o||+||ou,
+ud e\|eu|.e eo|+p||c |u|+|c||ou ou ||e |+ce, ||e ||uu|, +ud ||e e\||er|||e. I|| | d|er|u+|ed |u||+.+cu|+|
co+u|+||ou. pu|pu|+ |u|r|u+u |o||oW|u ep| +||e| +|dor|u+| u|e|,.
not, and in these the condition evolves fiom
diffuse eiythema to immediate neciosis and
epideimal detachment.
Theie is now consensus that SJS and TEN aie
diffeient fiom eiythema multifoime(EM).
SJS. <10% epideimal detachment
SJS/ TEN oer|a. 10-30% epideimal detach-
ment.
TEN. >30% epideimal detachment.
FI0MI0I0C
Ae oI 0oset Any age, but most common in
adults >40 yeais. Equal sex incidence.
0vera|| Iocdeoce TEN : 0.4-1.2 pei million
peison-yeais. SJS : 1.2-6 pei million peison-
yeais.
ksk Factors Systemic lupus eiythematosus,
HLA-B12, HIV/AIDS.
Polyetiologic ieaction pattein, but diugs aie
cleaily the leading causative factoi. TEN : 80%
of cases have stiong association with specific
medication (Table 8-4); <5% of patients ie-
poit no diug use. Also: chemicals, Myto|asma
pneumoniae, viial infections, immunization.
SJS : 50% aie associated with diug exposuie;
etiology often not cleai-cut.
Pathogenesis of SJS-TEN is only paitially
undeistood. It is viewed as a cytotoxic im-
mune ieaction aimed at the destiuction of
keiatinocytes expiessing foieign (diug-ie-
lated) antigens. Epideimal injuiy is based
on the induction of apoptosis. Diug-specific
activation of T cells has been shown in vitio
on peiipheial blood mononucleai cells of
patients with diug eiuptions. The natuie of
the antigens that diive the cytotoxic cellulai
immune ieaction is not well undeistood.
Diugs oi theii metabolites act as haptens and
iendei keiatinocytes antigenic by binding to
theii suifaces. Cutaneous diug eiuptions have
been linked to a defect of the detoxification
systems of livei and skin, which iesults in
diiect toxicity oi alteiation of antigenic piop-
eities of keiatinocytes. Cytokines pioduced by
activated mononucleai cells and keiatinocytes
piobably contiibute to local cell death, fevei,
and malaise.
Time fiom fiist diug exposuie to onset of
symptoms: 1-3 weeks. Occuis moie iapidly
with iechallenge. Often aftei days of ingestion
of the diug; newly added diug is most sus-
pect. Piodiomes: fevei, malaise, aithialgias 1-3
days piioi to mucocutaneous lesions. Mild to
modeiate skin tendeiness, conjunctival buining
IA8I 8-4 Nedications and the Risk of Io\ic Epiderma| Necro|ysis
h|gh 8|sk Lower 8|sk 0o0btI0| 8|sk ho v|deoce oI 8|sk
A||opu||uo| Ace||c +c|d |'A|| |+|+ce|+ro| Ap|||u
(e.., d|c|o|eu+c) (+ce|+r|uop|eu)
'u||+re||o\+/o|e Ar|uopeu|c||||u |,|+/o|oue +u+|e|c 'u||ou,|u|e+
'u||+d|+/|ue Cep|+|opo||u Co|||co|e|o|d I||+/|de d|u|e||c
'u||+p,||d|ue 0u|uo|oue 0||e| |'A|| (e\cep| +p|||u) |u|oer|de
'u||+do\|ue C,c||u 'e|||+||ue A|d+c|oue
'u||++|+/|ue \+c|o||de C+|c|ur c|+uue| ||oc|e|
C+||+r+/ep|ue B|oc|e|
|+ro||||ue Au|o|eu|ucou.e|||u eu/,re |u|||||o|
||euo|+||||+| Au|o|eu|u || |ecep|o| +u|+ou||
||eu,|o|u '|+||u
||eu,||u|+/oue no|roue
|e.||+p|ue \||+r|u
0\|c+r |'A||
I||+ce|+/oue
|'A||, uou|e|o|d+| +u|||u||+rr+|o|, d|u.
'ou|ce. |. \+|e,||eA||+uo|e, lC kouje+u. Ep|de|r+| uec|o|,|, |u ||cc|:| |-c||, C--c| !-!:-, 1e, K wo||| e| +| (ed).
|eW \o|| , \cC|+Wn|||, 2003, C|+p. !9.
oi itching, then skin pain, buining sensation,
tendeiness, paiesthesia. Mouth lesions aie pain-
ful, tendei. Impaiied alimentation, photopho-
bia, painful mictuiition, anxiety.
Sko Iesoos Prvdrvmu| Rush Is moibillifoim,
can be taiget lesion-like, with/without puipuia
(Fig. 8-7); iapid confluence of individual lesions
(Fig. 8-8); alteinatively, can stait with diffuse
eiythema (Fig. 8-9).
Eur|y Neciotic epideimis fiist appeais as
maculai aieas with ciinkled suiface that enlaige
and coalesce (Fig. 8-7). Sheetlike loss of epidei-
mis (Figs. 8-7 to 8-9). Raised flaccid blisteis that
spiead with lateial piessuie (Nikolsky sign) on
eiythematous aieas. With tiauma, full-thickness
epideimal detachment yields exposed, ied, ooz-
ing deimis (Figs.8-8 and 8-9) iesembling a
second-degiee theimal buin.
Recvvery Regiowth of epideimis begins
within days; completed in >3 weeks. Piessuie
points and peiioiificial sites exhibit delayed
healing. Skin that is not denuded acutely is
shed in sheets, especially palms/soles. Nails and
eyelashes may be shed.
DIstrIhutIvn Initial eiythema on face, ex-
tiemities, becoming confluent ovei a few houis
oi days. Epideimal sloughing may be geneial-
ized, iesulting in laige denuded aieas (Figs. 8-8
and 8-9). Scalp, palms, soles may be less seveiely
involved oi spaied. SJS : widely distiibuted with
piominent involvement of tiunk and face. TEN :
geneialized, univeisal.
Mucous Membraoes Invaiiably involved, i.e.,
eiythema, painful eiosions: lips, buccal mucosa,
conjunctiva, genital and anal skin.
yes 85% have conjunctival lesions: hypei-
emia, pseudomembiane foimation; keiatitis,
coineal eiosions; latei synechiae between eye-
lids and bulbai conjunctiva.
har aod Na|s Eyelashes and nails may be
shed in TEN.
CNkAI FIN0INCS
Fevei usually highei in TEN than in SJS.
Usually mentally aleit. Distiess due to seveie
pain.
Caidiovasculai : pulse may be >120 beats/
min. Blood piessuie.
Renal: tubulai neciosis may occui. Acute
ienal failuie.
Respiiatoiy and GI tiacts: sloughing of epi-
thelium with eiosions.
FICk 8-T IN, exaothematc preseotatoo I|e|e | + W|dep|e+d cou||ueu| r+cu|+| |+| W||| c||u|||u
o| ||e ep|de|r| |u ore +|e+ +ud de|+c|reu| o| ||e ep|de|r| |u o||e|, |e+.|u oo/|u |ed e|o|ou. I||
e|up||ou W+ due |o +||opu||uo|.
hemato|oy Anemia, lymphopenia; eosi-
nophilia uncommon. Neutiopenia coiielates
with pooi piognosis. Seium uiea incieased,
seium bicaibonate decieased.
0ermatopatho|oy Eur|y Vacuolization/
neciosis of basal keiatinocytes and individual
cell neciosis (apoptosis) thioughout the epi-
deimis.
Lute Full-thickness epideimal neciosis and
detachment with foimation of subepideimal
split above basement membiane. Spaise lym-
phocytic infiltiate in deimis. Immunofluoies-
cence studies uniemaikable, iuling out othei
blisteiing disoideis.
ar|y Exanthematous diug eiuptions, EM
majoi, scailet fevei, phototoxic eiuptions, toxic
shock syndiome, giaft-veisus-host disease
(GVHD).
Fu||y vo|ved EM majoi (typical taiget le-
sions, piedominantly on extiemities), GVHD
(may mimic TEN; less mucosal involvement),
theimal buins, phototoxic ieactions, staphylo-
coccal scalded-skin syndiome (in young chil-
dien, iaie in adults), geneialized bullous fixed
diug eiuption, exfoliative deimatitis.
C0kS AN0 Fk0CN0SIS
Aveiage duiation of piogiession is <4 days. A
piognostic scoiing system is shown in Table
8-5. Couise similai to that of extensive theimal
buins. Piognosis ielated to extent of skin necio-
sis. Tianscutaneous fluid loss is laige and vaiies
with aiea of denudation; associated electio-
lyte abnoimalities. Pieienal azotemia common.
Bacteiial colonization common and associated
with sepsis. Othei complications include hy-
peimetabolic state and diffuse inteistitial pneu-
monitis. Moitality iate foi TEN is 30%, mainly
in eldeily; foi SJS, 5-12%. Moitality ielated to
sepsis, GI hemoiihage, fluid/electiolyte imbal-
ance. If the patient suivives the fiist episode of
SJS/TEN, ieexposuie to the causative diug may
be followed by iecuiience within houis to days,
moie seveie than the initial episode.
SIA
S|In Scaiiing, hypo- and hypeipigmentation,
eiuptive nevomelanocytic nevi, abnoimal ie-
giowth of nails.
Eyes Common, including Sjgien-like sicca
syndiome with deficiency of mucin in teais;
entiopion, tiichiasis, squamous metaplasia,
neovasculaiization of conjunctiva and coinea;
synblephaion, punctate keiatitis, coineal scai-
iing; peisistent photophobia, buining eyes,
visual impaiiment, blindness.
AnvgenItu|Iu: Phimosis, vaginal synechiae.
MANACMNI
Acute SJS/TEN
Eaily diagnosis and withdiawal of suspected
diug(s) aie veiy impoitant.
Patients aie best caied foi in an inteimediate
oi intensive caie unit.
IA8I 8-5 SC0RIEN: A Pronostic
Scorin System for Patients with Epiderma|
Necro|ysis
SC0kIN
Frooostc Factors
Foots
Ae > +0 ,|
ne+|| |+|e > 20 |e+|/r|u
C+uce| o| |er+|o|o|c
r+||u+uc,
Bod, u||+ce +|e+ |u.o|.ed
> 0 pe|ceu|
'e|ur u|e+ |e.e| > 0 r!
'e|ur ||c+||ou+|e |e.e|
20 r!
'e|ur |ucoe |e.e| > + r!
SC0kIN
0-
2
!
+
> 5
Morta|ty
kate (%)
!.2
2.
!5.3
53.!
90
c
||- |, c||. A|||ou| || | ||||, +pp|ec|+|ed ||+| We uoW
|+.e + co||u ,|er, We do |+.e + |ee|.+||ou W||| 'C0kIE|.
0u|, oue po|u| | +|ued |o |od, u||+ce +|e+ |u.o|.ereu|
(>0). I|e|e | de||u||e|, + p|ouo||c d|||e|euce |e|Weeu, e..,
20 +ud 10 |od, u||+ce +|e+ |u.o|.ereu| +ud ||| |ou|d |e
|e||ec|ed |u ||e |o|+| co|e.
'ou|ce. |+|+ ||or ' B+|uj|C+||u e| +|. 'C0kIE|. A e.e|||,o|
|||ue co|e |o| |o\|c ep|de|r+| uec|o|,|. / |.-| |-c|| 115.
+9, 2000, ||or | \+|e,||eA||+uo|e, lC kouje+u. Ep|de|r+| uec|o|
,|, |u ||cc|:| |-c||, C--c| !-!:-, 1|| ed,
K wo||| e| +| (ed). |eW \o|| , \cC|+Wn|||, 2003, C|+p. !9.
SCII0N 8 'E\EkE A|| |||EInkEAIE|||C 'K|| Eku|I|0|' || InE ACuIE|\ ||| |AI|E|I 1TT
Manage ieplacement of IV fluids and elec-
tiolytes as foi patient with a thiid-degiee
theimal buin. Howevei, less fluid usually
iequiied as foi theimal buin of similai
extent.
Systemic glucocoiticoids eaily in the disease
and in high doses aie iepoited helpful in
ieducing moibidity oi moitality (as is also
the expeiience of the authois), but this has
been questioned. Late in the disease they aie
contiaindicated.
High-dose IV immunoglobulins halt piogies-
sion of TEN if administeied eaily. This is
questioned by some authois; the disciepancy
may be explained by the diffeient pioducts
and batches used.
Pentoxifylline IV by continuous diip eaily on
in the eiuption has been anecdotally iepoited
to be beneficial.
With oiophaiyngeal involvement, suction fie-
quently to pievent aspiiation pneumonitis.
Suigical debiidement not iecommended.
Diagnose and tieat complicating infections,
including sepsis (fevei, hypotension, change
in mental status).
Tieat eye lesions eaily with eiythiomycin
ointment.
Freveotoo The patient must be awaie of the
likely offending diug and that othei diugs of
the same class can cioss-ieact. These diugs
must nevei be ieadministeied. Patient should
weai a medical aleit biacelet.
FICk 8-8 IN, exaothematc preseotatoo
A r+cu|+| |+| ||+| | |||| .||||e ou ||e |oWe| ||+u|
|+ co+|eced, +ud d||odreu| +ud |edd|u o| ||e
uec|o||c ep|de|r| |+ |ed |o |+|e, oo/|u, e\||ere|,
p+|u|u| e|o|ou. I|| |eer||e c+|d|u. I|e e|up||ou
W+ due |o + u||ou+r|de.
FICk 8-9 IN, ooo-exaothematc dIIuse
preseotatoo I|| o0,e+|o|d r+u de.e|oped
d|||ue e|,||er+ o.e| +|ro| ||e eu|||e |od,,
W||c| ||eu |eu||ed |u ep|de|r+| c||u|||u, de|+c|
reu|, +ud |edd|u o| ep|de|r| |e+.|u |+|e
e|o|ou. I|| | |er|u|ceu| o| c+|d|u.
1T8
S E C I | 0 N 9
8NICN N0FIASMS
AN0 hFkFIASIAS
0IS0k0kS 0F MIAN0CIS
One of the most common acquiied new giowths
in Caucasians (most adults have about 20 nevi),
less common in blacks oi pigmented peisons,
and sometimes absent in peisons with ied haii
and maiked fieckling (skin phototype I).
kace Blacks and Asians have moie nevi on the
palms, soles, nail beds.
heredty Common acquiied NMN occui in
family clusteis. Dysplastic melanocytic nevi
(DN) (see Section 12), which aie putative pie-
cuisoi lesions of malignant melanoma, occui in
viitually eveiy patient with familial cutaneous
melanoma and in 30-50% of patients with spo-
iadic nonfamilial piimaiy melanoma.
Suo xposure A factoi in the induction of nevi
on the exposed aieas.
SoIcaoce Risk of melanoma is ielated to
the numbeis of NMN and to DN. In the lattei,
even if only a few lesions aie piesent.
0uratoo aod vo|utoo oI Iesoos NMN ap-
peai in eaily childhood and ieach a maximum
in young adulthood even though some NMN
may aiise in adulthood. Latei on theie is a
|e.ore|+uoc,||c ue.| (|\|), corrou|, c+||ed
|- , +|e .e|, corrou, r+|| ( cr), c||cur
c|||ed, +cqu||ed p|reu|ed r+cu|e, p+pu|e, o|
uodu|e.
Corpoed o| |oup o| re|+uoc,||c ue.u ce||
|oc+|ed |u ||e ep|de|r|, de|r|, +ud, |+|e|,,
u|cu|+ueou ||ue.
I|e, +|e |eu|u, +cqu||ed |uro| +|||u + ue.u
ce|| c|u|e| +| ||e de|r+|ep|de|r+| juuc||ou
( :|c| |!| ), |u.+d|u ||e p+p|||+|, de|r|
(:! |!| ), +ud eud|u ||e|| |||e c,c|e +
!-c| |!| W||| ue.u ce|| |oc+|ed e\c|u|.e|,
|u ||e de|r| W|e|e, W||| p|o|e|.e +e, ||e|e
W||| |e ||||o|.
ACIk0 Nv0MIAN0CIIC NvI
giadual involution and fibiosis of lesions, and
most disappeai aftei the age of 60. In contiast,
DN continue to appeai thioughout life and aie
believed not to involute (see Section 12).
Sko Symptoms NMN aie asymptomatic.
Howevei, NMN initially giow and giowth
is often accompanied by itching. If a lesion
erssen|y itches oi is tendei, it should be
followed caiefully oi excised, since erssen
piuiitus may be an eaily indication of malig-
nant change.
CIASSIFICAII0N
NMN aie multiple (Fig. 9-1) and can be classi-
fied accoiding to theii state of evolution and
thus accoiding to the site of the clusteis of
nevus cells.
1. Juntona| me|anotyt NMN : These aiise
at the deimal-epideimal junction, on the
epideimal side of the basement membiane;
in othei woids, they aie intiaepideimal
(Fig. 9-2).
2. ComounJ me|anotyt NMN : Nevus cells
invade the papillaiy deimis, and nevus cell
nests aie now found both intiaepideimally
and deimally (Fig. 9-3).
SCII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 1T9
3. Derma| me|anotyt NMN : These iepiesent
the last stage of the evolution of NMN.
Diopping off " into the deimis is now
completed, and the nevus giows oi iemains
intiadeimal. With piogiessive age, theie will
be giadual fibiosis (Fig. 9-4).
Thus, melanocytic NMN undeigo the evolu-
tion fiom junctional compound deimal
NMN. Since the capacity of NMN cells to foim
melanin is gieatest when they aie located at
the deimal-epideimal junction (intiaepidei-
mally) and since NMN cells lose theii capacity
foi melanization, the fuithei they penetiate
into the deimis, the lessei is the intensity of
pigmentation with the inciease in the deimal
piopoition of the nevus. Puiely deimal NMN
aie theiefoie almost always without pigment.
In a simplified mannei, the clinical appeaiance
of NMN along this evolutionaiy path can be
chaiacteiized as follows: junctional NMN is
flat and daik, compound NMN is iaised and
daik, and deimal NMN is iaised and light. This
evolution also ieflects the age at which the dif-
feient types of NMN aie found. Junctional and
FICk 9-1 Mu|tp|e NMN oo the shou|der oI a 32-year-o|d Iema|e. \o| o| ||ee ue.| +|e juuc||ou+|
|\|, ore +|e |||||, e|e.+|ed +ud ||u corpouud |\|. |o|e |e|+||.e|, uu||o|r |+pe +ud co|o| o| ||e
|e|ou. |ue |o d|||e|eu| de.e|opreu|+| |+e ||e, +|e o| .+|,|u |/e |+u|u ||or |o + rr |u d|+re|e| +ud
||e, +|e |eu|+| +ud |+.e + |e|+||.e|, uu||o|r |+pe.
compound NMN aie usually seen in childhood
and thiough the teens, wheieas deimal NMN
stait manifesting in the thiid and fouith decade.
luoctooa| Me|aoocytc Nevoce||u|ar Nev
Iesoos Macule, oi only veiy slightly iaised
(Figs. 9-2, 33-13). Unifoim tan, biown, daik
biown, oi even black. Round oi oval with smooth,
iegulai boideis. Scatteied disciete lesions. Nevei
>1 cm in diametei; if >1 cm, the mole" is a
congenital nevomelanocytic nevus, an atypical
nevus, oi a melanoma (see Section 12).
Compouod Me|aoocytc Nevoce||u|ar Nev
Iesoos Papules oi small nodules (Fig. 9-3).
Daik biown, sometimes even black; dome-
shaped, smooth oi cobblestone-like suiface,
iegulai and shaiply defined boidei, sometimes
papillomatous oi hypeikeiatotic. Nevei >1 cm
in diametei; if >1 cm, the mole is a congenital
nevomelanocytic nevus, an atypical nevus, a
melanoma. Consistency eithei fiim oi soft.
Coloi may become mottled as piogiessive con-
veision into deimal NMN occuis. May have
haiis.
0erma| Me|aoocytc Nevoce||u|ar Nev
Iesoos Shaiply defined papule oi nodule.
Skin-coloied, tan oi flecks of biown, often
with telangiectasia. Round, dome-shaped (Fig.
9-4), smooth suiface, diametei <1 cm. Usually
not piesent befoie the second oi thiid decade.
Oldei lesions, mostly on the tiunk, may become
pedunculated and do not disappeai spontane-
ously. May be haiiy.
0strbutoo Face, tiunk, extiemities, scalp.
Random, but some piedilection foi sun-
exposed aieas. Occasionally palmai and plantai,
in which case these NMN usually have the
appeaiance of junctional NMN.
0aooss Made clinically. As foi all pigmented
lesions the ABCDE iule applies (see page 310).
In cases of doubt apply deimoscopy (epilumi-
nescence micioscopy), and if malignancy can-
not be excluded even by this pioceduie, excise
lesions with a naiiow maigin.
0IIereota| 0aooss Juntona| NMN : all
flat, deeply pigmented lesions. Solai lentigo,
flat atypical nevus, lentigo maligna. ComounJ
NMN : all iaised pigmented lesions. Seboi-
iheic keiatosis, DN, small supeificial spieading
melanoma, eaily nodulai melanoma, pigmented
FICk 9-2 A-0 luoctooa| NMN |e|ou +|e corp|e|e|, ||+| (, 8 ) o| r|u|r+||, e|e.+|ed + |u C. +ud
0. I|e, +|e ,rre|||c W||| + |eu|+| |o|de| +ud, depeud|u ou ||e ||u |,pe o| ||e |ud|.|du+|, |+.e d|||e|eu|
|+de o| ||oWu |o ||+c| (0).
8
C 0
SCII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 181
basal cell caicinoma, deimatofibioma, Spitz ne-
vus, blue nevus. Derma| NMN : all light tan oi
skin-coloied papules. Basal cell caicinoma, neu-
iofibioma, tiichoepithelioma, deimatofibioma,
sebaceous hypeiplasia.
MANACMNI
Indications foi iemoval of acquiied melano-
cytic NMN aie the following:
Se : Lesions on the scalp (only if difficult to
follow and not a classic deimal NMN);
mucous membianes, anogenital aiea.
Crow| : If theie is iapid change in size.
Co|or : If coloi becomes vaiiegated.
BorJer : If iiiegulai boideis aie piesent oi
develop.
Erosons : If lesion becomes eioded without
majoi tiauma.
Symoms : If lesion begins to erssen|y itch,
huit, oi bleed.
Dermostoy : If ciiteiia foi melanoma oi an
dysplastic nevus aie piesent oi appeai de
novo.
Melanocytic NMN neer become malignant
because of manipulation oi tiauma. In those
cases wheie this was claimed, the lesion was
initially a melanoma. If theie is an indi-
cation foi the iemoval of an NMN, the nevus
should always be excised foi histologic diag-
nosis and foi definite tieatment (paiticulaily
applicable to and decisive in iuling out con-
genital, dysplastic, oi blue nevi). Removal of
papillomatous, compound, oi deimal NMN
foi cosmetic ieasons by electiocauteiy iequiies
that a nevus be unequivocally diagnosed as
benign NMN and histology be peifoimed. If
an eaily melanoma cannot be excluded with
ceitainty, an excision foi histologic examina-
tion is obligatoiy but can be peifoimed with
naiiow maigins.
FICk 9-3 Compouod NMN uu||o|r|, p|reu|ed p+pu|e +ud r+|| dored uodu|e. . I|e |e|ou |o
||e |e|| | ||+||e| +ud |+u W||| + ro|e e|e.+|ed d+||e| ceu|e|, ||e |+|e| |e|ou (ou ||e ||||) | o|de| +ud c|oco|+|e
||oWu, ||e |e|| |e|ou | ,ouue| +ud |+ + p|edor|u+u||, juuc||ou+| corpoueu| +| ||e pe||p|e|,. 8. A |e+.||,
p|reu|ed dore|+ped |e|ou |u ||e e,e||oW. || | |+|p|, de||ued, uu||o|r|, ||+c|, roo|| +ud |||||,
co|||e|oue|||e u||+ce, |+|p|, +ud |eu|+||, de||ued. || re+u|e |e ||+u 5 rr.
8
FICk 9-4 0erma| me|aoocytc NMN . IWo dore|+ped, |+|p|, de||ued |e|+||.e|, o|| |+u uodu|e
ou ||e |e|| c|ee| +ud |||| |+|e|+| r+ud||u|+| |e|ou |u + o0,e+|o|d r+|e. I|ee |e|ou We|e p|e.|ou|, ruc|
d+||e| +ud |e e|e.+|ed. 8. A |+|e| r+u|||c+||ou o| + de|r+| |\|. I|| |e|ou | |+|p|, de||ued, |+ + |edd||
co|o| W||| + ceu||+| |eu|+| p|reu|ed po| W|e|e ||e ue.u o|.|ou|, | |||| corpouud |u u+|u|e. C. 0|d de|r+|
ue.u ou ||e uppe| ||p o| + o5,e+|o|d Wor+u. I|| |e|ou | |e|+||.e|, |+|d, |+ + roo|| u||+ce +ud + p|u|||
co|o|. I|| |e|ou | |u |e|e|ou.
8 C
FI0MI0I0C
Oveiall pievalence 1%. Onset in the fiist thiee
decades. Occuis spontaneously and in patients
with vitiligo (18-26%). Also in patients with
metastatic melanoma (aiound metastatic le-
sions and aiound piimaiy melanoma). May
heiald vitiligo. All iaces, both sexes. Halo nevi
occui in siblings and in those with a family his-
toiy of vitiligo.
FAIh0CNSIS
Immunologic phenomena, both humoial and
cellulai, aie iesponsible foi the dynamic changes
that eventually lead to nevus involution.
Theie aie thiee stages: (1) Development (in
months) of white halo aiound pieexisting
NMN; halo may be pieceded by faint eiythema;
(2) disappeaiance (months to yeais) of NMN;
and (3) iepigmentation (months to yeais) of
halo.
FhSICAI XAMINAII0N
Sko Iesoos Papulai biown NMN (<5 mm)
with oval oi iound halo of shaiply maiginated
hypomelanosis (Figs. 9-5 and 9-6 ). The
NMN is tenra||y located. These usually show
depigmentation with wood lamp examination.
Scatteied disciete lesions (1 to >30) mostly on
the tiunk, but in geneial the same distiibution
as of NMN (Fig. 9-5). May begin with eiythema
aiound NMN (Fig. 9-6 B ).
Speca| Forms
Congenital halo NMN occui iaiely.
If clinical findings atypical: the nevus has vaii-
egation of coloi and/oi iiiegulai boideis, con-
fiim histologically and exclude melanoma.
"ha|o" 0epmeotatoo arouod 0ther
Iesoos Can occui aiound blue nevus, con-
genital NMN, Spitz juvenile nevus, veiiuca
plana, piimaiy melanoma, melanoma metas-
tases, deimatofibioma, neuiofibioma.
FICk 9-5 ha|o me|aoocytc
NMN oo the back oI a 22-year-
o|d Iema|e I|e|e +|e ||.e |+|o
ue.|, +|| W||| + p|reu|ed do||||e
ceu||+| juuc||ou+| o| corpouud |\|
u||ouuded |, + |,po o| +re|+uo||c
|+|o. I|e +||oW |ud|c+|e oue |e|ou
W|e|e ||e ceu||+| ue.u |+ cor
p|e|e|, |e|eed, ||e |edd|| co|o|
|ud|c+|e |e|+u|ec|+|+.
Au |\| ||+| | euc||c|ed |, + |+|o o| |eu|ode|r+
o| dep|reu|+||ou. I|e |eu|ode|r+ | |+ed ou
+ dec|e+e o| re|+u|u |u re|+uoc,|e +ud/o|
d|+ppe+|+uce o| re|+uoc,|e +| ||e de|r+|ep|
de|r+| juuc||ou.
A W|||e |+|o +|ouud + |\| |ud|c+|e |e|e|ou
n+|o ue.| ro| o||eu uude|o pou|+ueou |u
.o|u||ou, o||eu W||| |e|e|ou o| ||e ceu||+||,
|oc+|ed p|reu|ed ue.u.
n+|o |\| r+, |ud|c+|e |uc|p|eu| .|||||o.
',, . 'u||ou |eu|ode|r+ +cqu|||ur ceu
||||uur.
hAI0 Nv0MIAN0CIIC NvS |C|9 . 2o.9
|C|0 . |22\312!/0
0ermatopatho|oy Junctional deimal oi com-
pound nevus suiiounded by lymphocytic in-
filtiate (lymphocytes and histiocytes) aiound
and between nevus cells. Nevus cells develop
evidence of cell damage undeigo apoptosis, and
disappeai. Halo shows deciease oi total absence
of melanin and melanocytes.
MANACMNI
Reassuiance. Excision if the featuies of the ne-
vus aie atypical: vaiiegation of coloi, iiiegulai
boideis.
FICk 9-6 ha|o me|aoocytc NMN . |+|e| r+u|||c+||ou o| + |+|o |\|. I|e ue.u | + juuc||ou+|
|\| (corp+|e W||| ||. 92) ||+| | u||ouuded |, + |,pore|+uo||c (+|ro| W|||e) |+|o. 8. 'e.e|+| |+u juuc||ou+|
|\| ||+| +|e u||ouuded |, +u e|,||er+|ou |+|o. I|| | ||e e+||, |+e o| |+|o de.e|opreu|. I|e
e|,||er+|ou ||r W||| |+|e| |e |ep|+ced |, + |,pore|+uo||c |+|o.
8
FI0MI0I0C
0oset In childhood and late adolescence.
Equal sex distiibution.
varaots Thiee types: common blue nevus,
cellulai blue nevus, combined blue nevus-
nevomelanocytic nevus.
FAIh0CNSIS
Ectopic accumulations of melanin-pioducing
melanocytes (not nevus cells) in the deimis
deiived fiom melanoblasts that became aiiested
duiing theii migiation fiom neuial ciest to sites
in the skin.
A ||ue ue.u | +u +cqu||ed, |||r, d+||||ue |o
|+,|o||+c|, |+|p|, de||ued p+pu|e o| uodu|e
|ep|eeu||u + |oc+||/ed p|o|||e|+||ou o| re|+u|u
p|oduc|u !-c| re|+uoc,|e.
A ||ue ue.u | |eu|u. 'oc+||ed ce||u|+| ||ue
ue.| +|e |+|e| +ud |+.e .e|, |+|e |eudeuc, |o
|ecore r+||u+u|.
',, . B|ue ueu|oue.u, de|r+| re|+uoc,
|or+.
8I NvS |C|9 . 2o.9
|C|0 . |22. \3130

Neaily always asymptomatic, occasion-
ally of cosmetic concein; often feaied to be
melanoma.
FhSICAI XAMINAII0N
Sko Iesoos Papules to nodules, blue, blue-
giay, blue-black, usually <10 mm in diametei
(Figs. 9-7 and 9-8 ). Ce||u|ar ||ue ne aie laigei
(1-3 cm) (Fig. 9-8 B ). Occasionally have taiget-
like pattein of pigmentation. Usually iound to
oval. Com|neJ ||ue neus - NMN : blue-biown
oi blue-black with a lightei iim.
SItes v] PredI|ectIvn Most commonly located
on the doisa of hands (Fig. 9-8 ) oi feet (50%),
but many occui in the face (Fig. 9-7); cellulai
blue nevi occui on the buttocks, lowei back,
scalp (Fig. 9-8 B ), and face.
0ermatopatho|oy Melanin-containing wavy
deimal melanocytes with long thin dendiites
giouped in iiiegulai bundles admixed with
melanin-containing maciophages in the up-
pei oi middle deimis: excessive fibious tissue
pioduction in uppei ieticulai deimis. Ce||u-
|ar ||ue neus : in addition to spindle-shaped
melanocytes, epithelioid nevus cells in deimis
and subcutaneous fat in nests and neuioid
foims. Com|neJ ||ue neus - NMN : combi-
nation of blue nevus and compound NMN.
Usually made on clinical findings, at times
confiimed by excision and deimatopathologic
examination to iule out nodulai melanoma.
8|ue[Cray Fapu|e Deimatofibioma, glomus
tumoi, piimaiy (nodulai) oi metastatic
melanoma, pigmented spindle cell (Spitz) nevus,
tiaumatic tattoo, angiokeiatoma, pigmented
basal cell caicinoma.
C0kS AN0 Fk0CN0SIS
Most iemain unchanged. Malignant melanoma
iaiely develops in te||u|ar blue nevi.
MANACMNI
Blue nevi <10 mm in diametei and stable foi
many yeais usually do not need excision. Sud-
den appeaiance oi change of an appaient blue
nevus waiiants suigical excision and deimat-
opathologic examination. Cellulai blue nevi
( 1-3 cm; Fig. 9-8B) aie usually excised to iule
out melanoma.
FICk 9-T 8|ue oevus I|e|e +|e |ou| |+u juuc||ou+| |\| +ud oue ||u||||+c| |ouud |e|ou ou ||e c|ee|
o| + 1,e+|o|d |||. |u cou||+| |o ||e juuc||ou+| |\| ||e ||ue ue.u | p+|p+||e W||| + |e|+||.e|, ||| cou|
|euc, +ud upou de|rocop, W||| +ppe+| + +u |||de||ued uu||o|r|, ||u|| |e|ou deep |u ||e de|r|.
FICk 9-8 8|ue oevus aod ce||u|ar b|ue oevus . I|| ||ue ue.u |+ |eu|+| |o|de| |u| | uo| c||
cu|+| +ud | o||d|, ||ue||+c| |u co|o|. I|e ep|de|r| | roo||, |ud|c+||u ||+| ||e |e|ou | |u ||e de|r|. I|e
cou||euc, | |uc|e+ed +ud ||e r+||u +|e We|| de||ued. ||||e|eu||+| d|+uo| ru| |uc|ude uodu|+| re|+uor+.
|| ||e |e|ou |+ |eeu p|eeu| |o| ,e+|, + ||op, | uo| uece+|,. || ||e |e|ou W+ uo|ed ou|, + |eW rou|| +o,
e\c||ou ||op|e +|e |equ||ed |o |u|e ou| uodu|+| re|+uor+. noWe.e|, de|rocop, |e+||, |+c||||+|e ||e c||u|c+|
d|||e|eu||+| d|+uo| |oW|u uoue o| ||e |e+|u|e o| uodu|+| re|+uor+ +ud W||| ||e|e|o|e |eude| +u e\c||ou+|
||op, uuuece+|,. 8. I|| ce||u|+| ||ue ue.u +ppe+|ed + |Wo |+|e, ||u||||+c| uodu|e ou ||e c+|p. A||e|
e\c||ou, |||o|o, |oWed ||+| ||e, We|e cou||uou +ud ||u |ep|eeu|ed oue |u|e |e|ou. Ce||u|+| ||ue ue.|
+|e ruc| |+|e| +ud |ou|d +|W+, |e e\c|ed |o |u|e ou| re|+uor+, W||c|, +||e|| |+|e|,, c+u de.e|op |u ||ee
|e|ou.
8
A |+||e| corrou re|+uoc,||c |e|ou ||+| cou||
o| + |||| ||oWu p|reu|ed r+cu|e .+|,|u ||or
+ |eW ceu||re|e| |o + .e|, |+|e +|e+ (>5 cr),
+ud r+u, d+|| ||oWu r+|| r+cu|e (2-! rr)
o| p+pu|e c+||e|ed |||ou|ou| ||e p|reu|ed
|+c||ouud (||. 99 1 ). I|e p|reu| |u ||e
r+cu|+| |+c||ouud r+, |e o |+|u| ||+| || c+u
|e |ecou|/ed ou|, uude| wood |||| (||. 99 3 ).
I|e p+||o|o, o| ||e |+c||ouud o| ||e r+cu|+|
p|reu|ed |e|ou | ||e +re + |eu||o |rp|e\,
|.e., |uc|e+ed uur|e| o| re|+uoc,|e, W|||e
||e ||+| o| |+|ed |e|ou c+||e|ed |||ou|ou| +|e
e|||e| juuc||ou+| o| corpouud, |+|e|,, ||ee +|e
||.
I|e |e|ou +|e uo| + corrou + juuc||ou+| o|
corpouud |\| |u| +|e uo| +| +|| |+|e. |u oue
e||e, ||e ue.u p||u W+ p|eeu| |u ! o|
W|||e p+||eu|.
\+||u+u| re|+uor+ .e|, |+|e|, +||e |u ||ee
|e|ou.
NvS SFIIS |C|9 . 2o.9
|C|0 . |22
SCII0N 9 BE||C| |E0||A'\' A|| n\|Ek||A'|A' 18T
8
FICk 9-9 Nevus sp|us . I|| d+|| ||oWu p|reu|ed r+cu|e re+u||u +|ou| 0 cr +|ou ||e |ou
+\| | peppe|ed W||| r+u, r+||, d+|| ||oWu |o ||+c| r+cu|e +ud p+pu|e. 8. I|| | +|o ue.u p||u |u| ||e
r+cu|+| |+c||ouud | ou|, |||||, p|reu|ed o ||+| || W||| |e |e.e+|ed ou|, uude| wood ||||. I|e |e|ou | pep
pe|ed W||| r+u, r+|| d+|| ||oWu r+cu|e +ud ||+| p+pu|e.
FICk 9-10 Sptt oevus . ||u| dore|+ped uodu|e ou ||e c|ee| o| + ,ouu Wor+u, de.e|op|u
+||up||, W||||u ||e p|e.|ou 2 rou||, ||e |e|ou c+u |e r||+|eu |o| + |er+u|or+. 8. ||reu|ed 'p||/ ue.u.
A ||+c| p+pu|e u||ouuded |, + |+u r+cu|+| |e|ou (|eu|||uou) de.e|oped W||||u + |eW rou|| ou ||e |+c| o| +
,ouu |er+|e, + uc| + |e|ou c+uuo| |e d|||uu||ed ||or + uodu|+| re|+uor+, ||e |e|ou W+ e\c|ed +ud
||e d|+uo| cou|||red |||o|o|c+||,
8
'p||/ ue.u | + |eu|u, dore|+ped, |+|||e,
r+|| ( cr |u d|+re|e|) uodu|e, ro| o||eu
p|u| o| |+u (||. 901). I|e|e | o||eu + |||o|, o|
|eceu| |+p|d |oW||.
|uc|deuce | .+.00,000 (Au||+||+). || occu| +|
+|| +e. A ||||d o| ||e p+||eu| +|e c|||d|eu 0
,e+|, + ||||d +|e 0-20 ,e+| o|d, +ud + ||||d
+|e >20, |+|e|, eeu |u pe|ou +0 ,e+|. |-
+||e W||||u rou||. I|e, +|e p+pu|e o|
dore|+ped o| |e|+||.e|, ||+| uodu|e, |ouud,
We||c||urc|||ed, roo|||opped, +ud |+|||e.
I|e, +|e + uu||o|r p|u| (||. 90 1 ), |+u, ||oWu,
d+|| ||oWu, o| e.eu ||+c| (||. 90 3 ), +|e |||r,
+ud uu+||, d|||||u|ed ou ||e |e+d +ud uec|.
|||--|c| !c |uc|ude +|| p|u|, |+u, o|
d+|||, p|reu|ed p+pu|e. p,oeu|c |+uu|or+,
|er+u|or+, ro||ucur cou|+|our, ju.eu||e
\+u||o|+uu|or+, r+|oc,|or+, de|r+|o||
||or+, |\|, ||, uodu|+| re|+uor+.
|-c|c|||, cou|| o| |,pe|p|+|+ o| ||e
ep|de|r| +ud o| re|+uoc,|e, d||+|+||ou o| c+p||
|+||e. I|e|e +|e +dr|\ed |+|e ep|||e||o|d ce||,
|+|e p|ud|e ce|| W||| +|uud+u| c,|op|+r, +ud
occ+|ou+| r||o||c ||u|e. I|e|e +|e ore||re
||/+||e c,|o|o|c p+||e|u. ue| o| |+|e ce||
e\|eud ||or ||e ep|de|r| ('|+|u|u doWu)
|u|o ||e |e||cu|+| de|r| + |+c|c|e o| ce|| |o|r
+u '|u.e||ed |||+u|e, W||| ||e |+e |,|u +| ||e
de|r+|ep|de|r+| juuc||ou +ud ||e +pe\ |u ||e
|e||cu|+| de|r|.
n||o|o|c e\+r|u+||ou ru| |e doue |o cou|||r
||e c||u|c+| d|+uo|. E\c||ou |u || eu|||e|, | |r
po||+u| |ec+ue ||e coud|||ou |ecu| |u 0-5
o| +|| c+e |u |e|ou ||+| |+.e uo| |eeu e\c|ed
corp|e|e|,. 'p||/ ue.| +|e |eu|u, |u| ||e|e c+u
|e + |||o|o|c |r||+|||, |o re|+uor+ +ud ||e
|||op+||o|o|c d|+uo| |equ||e ||e |e|p o| +u
e\pe||euced de|r+|op+||o|o||.
'p||/ |uro| p|o|+||, do uo| uu+||, |u.o|u|e,
+ do corrou +cqu||ed |\| ue.|. noWe.e|,
ore |e|ou |+.e |eeu o|e|.ed |o ||+u|o|r
|u|o corrou corpouud |\|, +ud ore uu
de|o ||||o| +ud |u |+|e |+e r+, |eer||e
de|r+|o||||or+.
',, . ||reu|ed +ud ep|||e||o|d p|ud|e
ce|| ue.u. \e+| +o ||ee We|e c+||ed 'ju.eu||e
re|+uor+.
SFIII NvS |C|9 . 2o.9
|C|0 . |22\3112
FICk 9-11 Mooo|ao spot A |+|e |+,
||ue r+cu|+| |e|ou |u.o|.|u ||e eu|||e |ur|o+c|+|
+ud |u|e+| +|e+ +ud ||e |e|| |||| |u + |+|, ||or '||
|+u|+. A|||ou| \ouo||+u po| +|e corrou |u
A|+u, ||e p+|eu| o| ||| |+|, We|e +|+|red |ec+ue
||e |e|ou W+ o |+|e.
FICk 9-12 Mooo|ao spots \u|||p|e, |||de||ued,
||u|| |e|ou +|e c+||e|ed ou ||e |+c| o| ||| c|||d o|
l+p+uee deceu|. I|e, We|e p|eeu| +| |||||. \o| o|
||ee |e|ou d|+ppe+|ed |+|e| |u c|||d|ood.
I|ee coueu||+| |+,||ue r+cu|+| |e|ou +|e
c|+|+c|e||||c+||, |oc+|ed ou ||e |ur|o+c|+| +|e+
(||. 9) |u| c+u +|o occu| ou ||e |+c|, c+|p,
o| +u,W|e|e ou ||e ||u. I|e|e | uu+||, + |u|e
|e|ou, |u| |+|e|,, e.e|+| ||uuc+| |e|ou c+u |e
p|eeu| +| ||||| (||. 92).
I|e uude||,|u p+||o|o, | d|pe|ed p|ud|e
|+ped re|+uoc,|e W||||u ||e de|r| (de|r+|
re|+uoc,|o|). \e|+uoc,|e +|e uo| uo|r+||,
p|eeu| |u ||e de|r|, +ud || | |e||e.ed ||+| ||ee
ec|op|c re|+uoc,|e |ep|eeu| p|reu| ce|| ||+|
|+.e |eeu |u|e||up|ed |u ||e|| r||+||ou ||or ||e
ueu|+| c|e| |o ||e ep|de|r|.
\ouo||+u po| r+, d|+ppe+| |u e+||, c|||d
|ood, |u cou||+| |o ue.u o| 0|+ (ee ||.
9! 1 ).
A ||e |e|r !|c |rp||e, ||ee |e|ou
+|e |ouud +|ro| +|W+, (99-00) |u |u|+u| o|
A|+||c +ud |+||.e Are||c+u o|||u, |oWe.e|, ||e,
|+.e |eeu |epo||ed |u ||+c| +ud, |+|e|,, |u W|||e
|u|+u|.
|o re|+uor+ |+.e |eeu |epo||ed |o occu| |u
||ee |e|ou.
|u A|+u.
M0NC0IIAN SF0I |C|9 . 151.!!
I|| p|reu|+|, d|o|de| | .e|, corrou |u A|+u
popu|+||ou +ud | +|d |o occu| |u o| de|r+
|o|o|c ou|p+||eu| |u l+p+u. || |+ |eeu |epo||ed
|u E+| |ud|+u, ||+c|, +ud, |+|e|,, W|||e.
I|e p|reu|+||ou, W||c| c+u |e qu||e u|||e o|
r+||ed|, d|||u||u, cou|| o| + ro|||ed, du|,
+dr|\|u|e o| ||ue +ud ||oWu |,pe|p|reu|+||ou o|
||e ||u. I|e p|reu|+||ou ro||, |u.o|.e ||e ||u
+ud rucou rer||+ue |uue|.+|ed |, ||e |||| +ud
ecoud ||+uc|e o| ||e |||er|u+| ue|.e (||. 9!).
I|e ||ue |ue |eu|| ||or ||e p|eeuce o| ec|op|c
re|+uoc,|e |u ||e de|r|. || c+u occu| |u ||e
|+|d p+|+|e +ud |u ||e coujuuc||.+e (||. 9! 3 ),
c|e|+e, +ud |,rp+u|c rer||+ue.
|e.u o| 0|+ r+, |e |||+|e|+| (||. 9! C ). || r+,
|e coueu||+| |u| | uo| |e|ed||+|,, ro|e o||eu ||
+ppe+| |u e+||, c|||d|ood o| du||u pu|e||, +ud
|er+|u |o| |||e, |u cou||+| |o ||e \ouo||+u po|,
W||c| r+, d|+ppe+| |u e+||, c|||d|ood.
I|e+|reu| W||| |+e| | +u e||ec||.e rod+|||, |o|
||| d|||u||u d|o|de|.
\+||u+u| re|+uor+ c+u occu| |u| | |+|e.
*|u A|+u.
NvS 0F 0IA |C|9 . 2o.9
|C|0 . |22
FICk 9-13 Nevus oI 0ta . I|e|e | +u |||de||ued, ro|||ed, du|,, |+, |o ||u|| |,pe|p|reu|+||ou |u
||e |e|ou upp||ed |, ||e |||| +ud ecoud ||+uc|e o| ||e |||er|u+| ue|.e. I|e |e|ou W+ uu||+|e|+| +ud ||e|e
W+ +|o |,pe|p|reu|+||ou o| ||e c|e|+.
8
FICk 9-13 Nevus oI 0ta (Cootoued) 8. B|oWu|| |o |+, |,pe|p|reu|+||ou ou ||e uppe| +ud |oWe|
||d +ud + pec||ed |,pe|p|reu|+||ou o| ||e c|e|+ |u + |+|||+u| |o,. I|e |e|ou | uu||+|e|+|. C. B||+|e|+| ue.u o|
0|+ W||| |u.o|.ereu| o| ||e c|e|+e |u + l+p+uee c|||d.
C
I|e p|eeu| ||u+|, ||o|o|c c|+|||c+||ou d|
||uu||e |e|Weeu .+cu|+| |uro| +ud .+cu|+|
r+||o|r+||ou. I|e |+||e| +|e u|c|+|||ed +cco|d
|u |o ||e ||uc|u|+| corpoueu| |u|o c+p|||+|,,
.euou, |,rp|+||c, +||e||+|, o| cor||ued |o|r.
lc:|c | (e.., |er+u|or+) |oW eu
do||e||+| |,pe|p|+|+, W|e|e+ c||c|
|+.e + uo|r+| eudo||e||+| |u|uo.e|.
ner+u|or+ o| |u|+uc, +|e uo| p|eeu| +| |||||
|u| +ppe+| po|u+|+||,, |oW |+p|d|, du||u ||e
|||| ,e+| (p|o|||e|+||u p|+e), uude|o |oW
pou|+ueou |e|e|ou du||u c|||d|ood (|u.o|u
||ou p|+e), +ud |er+|u |+||e ||e|e+||e|.
\+cu|+| r+||o|r+||ou +|e e||o| o| ro|p|oeu
e| +ud +|e p|eured |o occu| du||u |u||+u|e|
|ue |||e. \o| +|e p|eeu| +| |||||, ||ou| ore do
uo| +ppe+| uu||| ,e+| |+|e|. 0uce r+u||e| ||e,
|oW p|opo|||ou+||,, |u| eu|+|ereu| c+u occu|
+ + |eu|| o| .+||ou |+c|o|.
Bo|| .+cu|+| |uro| +ud r+||o|r+||ou c+u |e
ep+|+|ed |u|o |oW||oW o| |+|||oW |,pe.
C|+|||c+||ou o| .+cu|+| |uro| +ud r+||o|r+
||ou | |oWu |u I+||e 9 +ud ||e d|||uu|||u
|e+|u|e o| .+cu|+| |uro| +ud .+cu|+| r+||o|
r+||ou +|e |oWu |u I+||e 92.
vASCIAk IM0kS AN0 MAIF0kMAII0NS
IA8I 9-1 C|assification of Vascu|ar Anoma|ies
vascu|ar Iumors vascu|ar Ma|Iormatoos
ner+u|or+ C+p|||+|,
ner+u|or+ o| |u|+uc, C+p|||+|, r+||o|r+||ou (po||W|ue |+|u)
Coueu||+| Ie|+u|ec|+|+ (|e|ed||+|, |eu|u |e|+u|ec|+|+, eeu||+|
k+p|d|, |u.o|u||u coueu||+| |e|+u|ec|+|+)
|er+u|or+ ne|ed||+|, |ero|||+|c |e|+u|ec|+|+
|ou|u.o|u||u coueu||+| C+p|||+|, r+||o|r+||ou+||e||o.euou r+||o|r+||ou
|er+u|or+ '|u|ewe|e| ,ud|ore
ner+u|oeudo||e||or+ \euou
K+po||o|r |er+u|oeudo||e||or+ \euou r+||o|r+||ou
Iu||ed +u|or+ |+r|||+| |o|r. Cu|+ueoruco+| .euou r+||o|r+||ou
Au|o+|cor+ C|oru.euou r+||o|r+||ou
B|ue |u||e| ||e| ue.u o| Be+u ,ud|ore
|,rp|+||c
|,rp|+||c r+||o|r+||ou
|||r+|, |,rp|oeder+
A||e||+|
A||e||o.euou r+||o|r+||ou
C+p|||+|, r+||o|r+||ou+||e||o.euou r+||o|r+||ou
A||e||o.euou |||u|+
',ud|or|c r+||o|r+||ou
'|oW||oW
K||ppe|I|eu+uu+, ,ud|ore (c+p|||+|,|,rp|+||co.euou
r+||o|r+||ou)
\+|||ucc| ,ud|ore
|+|||oW
|+||e we|e| ,ud|ore
'ou|ce. |\ Boou, +ud \ \|||u||+, |u K wo||| e| +| (ed). ||cc|:| |-c||, C--c| !-!:-, 1|| ed. |eW \o||, \cC|+Wn|||, 2003,
pp o5-ooo.
vASCIAk IM0kS
IA8I 9-2 0istinuishin |eatures of Vascu|ar Iumors (hemaniomas) and Vascu|ar Na|formations
T0mors Na|Iormat|oos
||eeuce +| ||||| uu+||, po|u+|+|, !0 u+ceu|, 00 (p|eur+||,), uo|
|+|e|, |u|| |oWu +|W+, o|.|ou
\+|e.|er+|e |+||o .!-.5 .
|uc|deuce -2.o +| |||||, 0-2 0.!-0.5 po||W|ue |+|u
+| ,e+|
|+|u|+| |||o|, ||+e. p|o|||e|+||u, |u.o|u||u, ||opo|||ou+|e |oW||,
+ud |u.o|u|ed c+u e\p+ud
Ce||u|+| Eudo||e||+| |,pe|p|+|+ |o|r+| eudo||e||+| |u|uo.e|
'|e|e|+| c|+ue 0cc+|ou+| r+ e||ec| ou '|oW||oW. d||o|||ou,
+dj+ceu| |oue, |+|e |,pe|||op|,, o|
|,pe|||op|, |,pe|p|+|+
|+|||oW. de||uc||ou,
d||o|||ou, o| |,pe|||op|,
'ou|ce. ' \||ue|||C|e.e||u|, lB \u||||eu, |u |\ ||eed|e| e| +| (ed). ||cc|:| |-c||, C--c| !-!:-, o|| ed. |eW \o||,
\cC|+Wn|||, 200!, pp 002-09.
hMANCI0MA 0F INFANC (hI)
|C|9 . 151.!2
|C|0 . |3.0\9!
(Foimeily stiawbeiiy, cheiiy, capillaiy heman-
gioma.)
FI0MI0I0C
HI is the most common tumoi of infancy. The
incidence in newboins is between 1 and 2.5%;
in white childien by 1 yeai of age it is 10%.
Females moie affected than males by a 3 to 1
iatio.
HI is a localized piolifeiative piocess of an-
gioblastic mesenchyme. It iepiesents a clonal
expansion of endothelial cells that may iesult
fiom somatic mutations of genes iegulating
endothelial cell piolifeiation.
The initial piolifeiative phase lasts fiom 3 to
9 months, sometimes moie. HIs usually enlaige
iapidly duiing the fiist yeai. In a subsequent
phase of involution the HI iegiesses, and this
occuis giadually ovei 2 to 6 yeais and is usually
complete by the age of 10. Involution vaiies
gieatly between individuals and is not coiie-
lated with size, location, oi appeaiance of the
lesion.
Sko Iesoos Soft, biight ied to deep puiple,
compiessible. On diascopy, does not blanch
completely. Nodule oi plaque, 1-8 cm (Figs.
9-14, 9-15 ). With the onset of spontaneous
iegiession, a white-to-giay aiea appeais on the
suiface of the cential pait of the lesion (Fig. 9-
15 ). Ulceiation may occui.
DIstrIhutIvn Lesions aie usually solitaiy and
localized oi extend ovei an entiie iegion (Fig.
9-16). Head and neck 50%, tiunk 25%. Face,
tiunk, legs, oial mucous membiane.
SFCIAI FkSNIAII0NS
0eep hemaooma (Foimeily, caveinous
hemangioma.) In the lowei deimis and sub-
cutaneous fat. Localized, fiim iubbeiy mass of
bluish oi noimal skin coloi with telangiectases
in oveilying skin (Fig. 9-17). Can be combined
with supeificial hemangioma (Fig. 9-15 ). Does
not involute as well as supeificial type.
Mu|tp|e hIs Multiple small (<2 cm), cheiiy-
ied papulai lesions involving skin alone ( |engn
tuaneous |emangomaoss ) oi skin and intei-
nal oigans ( J[[use neonaa| |emangomaoss ).
Cooeota| hemaoomas These develop in
uteio and aie subdivided into iapidly involuting
congenital hemangiomas (RICH) and noninvo-
luting congenital hemangiomas (NICH). They
piesent as violaceous tumois with oveilying
telangiectasia with laige veins in peiipheiy oi as
ied-violaceous plaques invading deepei tissues.
NICH aie fast-flow hemangiomas iequiiing
suigeiy.
0ermatopatho|oy Piolifeiation of endothe-
lial cells in vaiious amounts in the deimis
and/oi subcutaneous tissue; theie is usually
moie endothelial piolifeiation in the supeificial
type and little in the deep angiomas. GLUT-1
immunoieactivity is found in all hemangiomas
but not in vasculai malfoimations.
0IACN0SIS
Made on clinical findings and MRI; Dopplei
and aiteiiogiaphy to demonstiate fast flow. De-
teimine GLUT-1 immunoieactivity to iule out
vasculai malfoimation.
C0kS AN0 Fk0CN0SIS
HIs spontaneously involute by the fifth yeai,
with some few peicent disappeaiing only by age
10 (Figs. 9-14 B and 9-15 B ). Theie is viitually
no iesidual skin change at the site in most le-
sions (80%); in the iest theie is atiophy, dep-
igmentation, telangiectasia, and scaiiing. HIs
may, howevei, pose a consideiable pioblem
duiing the giowth phase when they inteifeie
with vital functions, such as obstiuction of vi-
sion (Fig. 9-16) oi of laiynx, nose, oi mouth.
Deepei lesions, especially those involving mu-
cous membianes, may not involute completely.
Synovial involvement may be associated with
hemophilia-like aithiopathy. Special foims of
HI, u[eJ angomas and Kaos[orm |eman-
goenJo|e|oma may have platelet entiapment,
thiombocytopenia (Kasabach-Meiiitt syn-
diome), and even disseminated intiavasculai
coagulation. Raiely, moibidity associated with
HI occuis secondaiy to hemoiihage oi high-
output heait failuie.
MANACMNI
Each lesion must be judged individually iegaid-
ing the decision to tieat oi not to tieat and
the selection of a tieatment mode. Systemic
tieatment is difficult, iequiies expeiience, and
should be peifoimed by an expeit. Suigical and
medical inteiventions include continuous wave
oi pulsed dye lasei, ciyosuigeiy, intialesional
and systemic high-dose glucocoiticoids, intei-
feion (IFN- ), and piopanolol. Foi the ma-
joiity of HIs active noninteivention is the best
appioach because spontaneous iesolution gives
the best cosmetic iesults (Figs. 9-14B, 9-15B).
Tieatment is indicated in about a quaitei of HIs
(5% that ulceiate; 20% that obstiuct vital stiuc-
tuies, i.e., eyes, eais, laiynx) and in the <1% that
aie life thieatening.
8
FICk 9-14 hemaooma oI oIaocy . I|| ||||| |ed uodu|+| p|+que |u +u |u|+u| o| A|||c+u e\||+c||ou
| |||||eu|u |o ||e p+|eu|, +ud c+u||ou | ueeded |o p|e.eu| c+|||u ||or ||e ||e+|reu| ||e||. '|uce ro| o|
||ee |e|ou d|+ppe+| pou|+ueou|, W||| ou|, 20 |oW|u |e|du+| +||op|, o| dep|reu|+||ou, + W+||+udee
||+|e, | |ecorreuded. 8. I|e +re |e|ou +||e| ! ,e+|. I|e |er+u|or+ |+ |+ded pou|+ueou|,, +ud ||e|e
| ou|, |||| |e|du+| +||op|,.
8
FICk 9-15 hemaooma oI oIaocy . I|| |e|ou ou ||e uoe cou|| o| + upe|||c|+| +ud deep po|||ou
+ud |uc|p|eu| |u.o|u||ou | +||e+d, +pp+|eu| |o| ||e upe|||c|+| corp+||reu|. |o|e +u +dd|||ou+| r+|| |er+u|or+
o| |u|+uc, ou ||e |e|| /,or+||c |e|ou. 8. B, ||e ||||| ,e+| ||e |er+u|or+ ou ||e uoe |+ +|ro| d|+ppe+|ed
+ud o |+ ||e |e|ou ou ||e /,or+||c |e|ou, ||+|, |oWe.e|, |+ |e|| + r+|| c+|.
FICk 9-16 hemaooma oI oIaocy ne|e || |u.o|.e + |+|e ereu| o| ||u. w|||e |u.o|u||ou | +||e+d,
+pp+|eu| ou ||e |o|e|e+d, ||e |e|ou ou ||e uppe| e,e||d +ud ||e red|+| c+u||u | |rp+|||u p|ope| |uuc||ou o|
||e ||d, +ud ||| |ud|c+|e ||+| .||ou r||| |e |rp+||ed |u ||e |u|u|e. |u ||| p+||eu|, ||e+|reu| W+ |ud|c+|ed.
FICk 9-1T hemaooma oI oIaocy, deep |esoo I|e|e | + |u||e|, r+ |u ||e u|cu|| +oc|+|ed
W||| + upe|||c|+| (|ed) po|||ou. I|ee |e|ou |+|d|, |e|e. I|e |er+u|or+ W+ |ero.ed |, u|e|,.
FICk 9-18 Fyoeoc
raou|oma . I|| | + o|||+|,
e|oded .+cu|+| uodu|e ||+| ||eed
pou|+ueou|, o| +||e| r|uo| ||+ur+.
I|e |e|ou uu+||, |+.e + roo||
u||+ce, W||| o| W|||ou| c|u|, W|||
o| W|||ou| e|o|ou. 8. 0u p+|r
+ud o|e ||e, |+.e + |,p|c+| co||+|
o| |||c|eued ||+|ur co|ueur +| ||e
|+e. I|| co||+| c+u |e| |e eeu
W|eu .|eWed ||or ||e |de, + | ||e
c+e |e|e.
8
|,oeu|c |+uu|or+ | + |+p|d|, de.e|op|u .+
cu|+| |e|ou uu+||, |o||oW|u r|uo| ||+ur+.
I|| | + .e|, corrou o|||+|, e|oded .+cu|+|
uodu|e ||+| ||eed pou|+ueou|, o| +||e| r|uo|
||+ur+. I|e |e|ou |+ + roo|| u||+ce, W|||
o| W|||ou| c|u|, W||| o| W|||ou| e|o|ou (||.
93 1 ). || +ppe+| + + ||||| |ed, du|, |ed,
.|o|+ceou, o| ||oWu||+c| p+pu|e W||| + co||+|
o| |,pe|p|+||c ep|de|r| +| ||e |+e (||. 93 3 )
+ud occu| ou ||e ||ue|, ||p, rou||, ||uu|, +ud
|oe.
n||op+||o|o|c+||, ||e|e +|e |o|u|+| +|e+|e o|
p|o|||e|+||u c+p|||+||e W||| eder+ +ud uure|ou
ueu||op|||. I|u, p,oeu|c |+uu|or+ | ue|||e|
p,oeu|c (+oc|+|ed W||| |+c|e||+| |u|ec||ou o|
||e ||u) uo| + |+uu|or+.
I|e+|reu| | u||c+| e\c||ou o| cu|e||+e W|||
e|ec||ode|cc+||ou +| ||e |+e.
I|e |rpo||+uce o| p,oeu|c |+uu|or+ |
||+| || c+u |e r||+|eu |o| +re|+uo||c uodu|+|
re|+uor+, +ud .|ce .e|+.
F0CNIC CkANI0MA |C|9 . o3o.
|C|0 . |93.0
FICk 9-19 C|omus tumor . I|| | +u e\qu|||e|, p+|u|u| u|uuu+| uodu|e o| |edd|| co|o|, p+|u |ecore
p+|o\,r+| upou e\pou|e |o co|d. 8. C|oru |uro| ou ||e p+|r o| + o,e+|o|d |o,.
8
I|| | + |uro| o| ||e |oru |od,. I|e |
|!, | +u +u+|or|c +ud |uuc||ou+| uu|| cor
poed o| pec|+||/ed roo|| ruc|e, ||e |
:-|| ||+| u||ouud |||uW+||ed eudo||e||+| p+ce,
||| +u+|or|c uu|| |uuc||ou + +u +||e||o.euou
|uu| ||u||u +||e||o|e +ud .euu|e. I|e |oru
ce|| u||ouud ||e u+||oW |ureu o| ||e 'ucque|
no,e| c+u+| ||+| ||+uc|e ||or ||e +||e||o|e +ud
|e+d |o ||e co||ec||u .euu|e ereu| ||+| +c|
+ + |ee|.o||. C|oru |od|e +|e p|eeu| ou ||e
p+d +ud u+|| |ed o| ||e ||ue| +ud |oe +ud
+|o ou ||e .o|+| +pec| o| |+ud +ud |ee|, |u ||e
||u o| ||e e+|, +ud |u ||e ceu|e| o| ||e |+ce.
I|e |oru |uro| p|eeu| + +u e\qu|||e|, |eu
de| u|uuu+| o| u|cu|+ueou p+pu|e o| uodu|e.
C|oru |uro| +|e c|+|+c|e||/ed |, p+|o\,r+|
p+|u|u| +||+c|, epec|+||, e||c||ed |, e\pou|e |o
co|d. I|e, +|e ro| o||eu p|eeu| + o|||+|, u|
uuu+| |uro| (||. 99 1 ) |u| r+, |+|e|, occu|
+ ru|||p|e p+pu|e o| uodu|e. I|ee +|e uo|ed,
epec|+||, |u c|||d|eu, + d|c|e|e p+pu|e o|
ore||re p|+que +u,W|e|e ou ||e ||u u||+ce
(||. 99 3 ).
I|e|+p, | |, e\c||ou.
CI0MS IM0k |C|9 . 223.0
|C|0 . \31/0
FICk 9-20 Aoosarcoma . E+||, |e|ou +ppe+| + du|, e|,||er+|ou r+cu|e. 8. \o|e +d.+uced
|e|ou +|e |ed |o ||+c| p+pu|e +ud uodu|e ||+| ||eed e+||,. C. Ad.+uced +u|o+|cor+ W||| ||eed|u pu|p|e |o
||+c| uodu|e, u|ce|+||ou, +ud coucor||+u| eder+.
C
8
I|| | + |+|e, ||||, r+||u+u| p|o|||e|+||ou o|
eudo||e||+| ce|| r+u||e||u + pu|pu||c r+cu|e
(||. 920 1 ) +ud/o| p+pu|e +ud uodu|e o|
||||| |ed o| .|o|+ceou +ud e.eu ||+c| co|o|
(||. 920 3 ). |odu|e +|e o||d, ||eed e+||,, +ud
u|ce|+|e (||. 920 C ).
I|e, occu| |u uo|r+| ||u, uu+||, ou ||e c+|p
+ud uppe| |o|e|e+d o| |u |oc+||/ed |,rp|eder+,
|o| |u|+uce |u po|r+|ec|or, |,rp|eder+
('|c|-.- ,!- ) o| po||||+d|+||ou
|,rp|eder+ (||. 9203).
n||o|o|c+||,. c|+uue| ||ued |, p|eoro|p||c
eudo||e||+| ce|| W||| + ||| uur|e| o| r||oe.
I|e+|reu| | |, u|e|, +ud/o| c|ero||e|+p,
(||poor+| do\o|u||c|u). I|e 5,e+| u|.|.+| | ju|
+|o.e 0.
*Au|o+|cor+, +|||ou| uo| + |e|u ueop|+r, |
d|cued |e|e |ec+ue || ||| W||| o||e| .+cu|+| |uro|.
ANCI0SAkC0MA |C|0 . \920/!
I|ee +|e r+||o|r+||ou ||+| do uo| uude|o
pou|+ueou |u.o|u||ou.
Cc||c, c||c| (C\) (e.., ue.u ||+r
reu, o| po||W|ue |+|u, +cco|d|u |o ||e o|d
uoreuc|+|u|e), |,|c|: c||c| :c|
|c,|,|c|: c||c| (C|\), .-
c||c| (\\), +ud c|-.- c||
c| (A\\) +|e d|||uu||ed.
n||o|o|c+||, ||e, cou|| o| eu|+|ed, |o||uou
.ee| o| .+||ou |,pe.
0u|, ||e ro| corrou +ud |rpo||+u| +|e |e|u
de+|| W||| |e|e.
vASCIAk MAIF0kMAII0NS
CAFIIIAk MAIF0kMAII0NS
Sko Iesoos These aie maculai (Fig. 9-21)
with vaiying hues of pink to puiple. Laige
lesions follow a deimatomal distiibution and
aie usually unilateial (85%) though not always.
Most commonly involve the face wheie the CM
occuis in the distiibution of the tiigeminal
neive (Fig. 9-21), usually the supeiioi and
middle bianches; mucosal involvement of
conjunctiva and mouth may occui. CM may
also involve othei sites. With incieasing age
of the patient, papules oi iubbeiy nodules
(Fig. 9-22) often develop, leading to significant
disfiguiement.
C|oca| varaot
Neus [|ammeus nut|ae (stoik bite," eiythema
nuchae, salmon patch) occuis in appioximately
one-thiid of infants on the nape of the neck and
tends to iegiess spontaneously. Similai lesions
may occui on eyelids and glabella. It is not ie-
ally a CM but iathei a tiansitoiy vasodilatation
phenomenon.
hISI0FAIh0I0C
Reveals ectasia of capillaiies and no piolifeia-
tion of endothelial cells. GLUT-1 immunoieac-
tivity is negative.
FICk 9-21 Fort-woe stao '|+|p|, r+||u+|ed,
po||W|ue |ed r+cu|e occu|||u |u + d|||||u||ou o| ||e
ecoud ||+uc| o| ||e |||er|u+| ue|.e |u + c|||d.
A po||W|ue |+|u (|w') | +u |||eu|+||, |+ped,
|ed o| .|o|+ceou, r+cu|+| C\ ||+| | p|eeu| +|
||||| +ud ue.e| d|+ppe+| pou|+ueou|,.
|| | corrou (0.! o| ueW|o|u), ||e r+||o|r+
||ou | uu+||, cou||ued |o ||e ||u.
\+, |e +oc|+|ed W||| .+cu|+| r+||o|r+||ou
|u ||e e,e +ud |ep|oreu|ue ('|u|ewe|e|
,ud|ore).
',, . |e.u ||+rreu.
F0kI-WIN SIAIN |C|9 . 151.!2
|C|0 . 032.5
C0kS AN0 Fk0CN0SIS
PWSs aie CMs that do not iegiess spontane-
ously. The aiea of involvement tends to in-
ciease in piopoition to the size of the child. In
adulthood, PWSs usually become iaised with
papulai and nodulai aieas and aie the cause of
significant piogiessive cosmetic disfiguiement
(Fig. 9-22).
MANACMNI
Duiing the maculai phase, PWS can be coveied
with makeup. Tieatment with tunable dye oi
coppei vapoi laseis is highly effective.
SN0k0MIC CM
Surge-Ve|er synJrome (SWS) is the association
of PWS in the tiigeminal distiibution with vas-
culai malfoimations in the eye and leptomenin-
ges and supeificial calcifications of the biain.
SWS may be associated with contialateial hemi-
paiesis, musculai hemiatiophy, epilepsy, and
mental ietaidation; glaucoma and oculai palsy
may occui. Skull x-iays show chaiacteiistic calci-
fications of vasculai malfoimations oi localized
lineai calcification along ceiebial convolutions.
CT scan should be done. It should, howevei, be
noted that PWS with tiigeminal distiibution
is common and does not necessaiily indicate
the piesence of SWS. K|e|-Trnaunay-Ve|er
synJrome may have an associated PWS oveily-
ing the deepei vasculai malfoimation of soft
tissue and bone. PVS on |e mJ|ne |at| may
be associated with an undeilying aiteiiovenous
malfoimation of the spinal coid.
FICk 9-22 Fort-woe stao w||| |uc|e+|u
+e, ||e co|o| deepeu +ud p+pu|+| +ud uodu|+| .+cu|+|
|e|ou de.e|op W||||u ||e p|e.|ou|, r+cu|+| |e|ou,
c+u|u p|o|e|.e|, |uc|e+|u d|||u|ereu|.
'p|de| +u|or+ | + .e|, corrou |ed |oc+| |e|
+u|ec|+||c ue|Wo|| o| d||+|ed c+p|||+||e |+d|+||u
||or + ceu||+| +||e||o|e (puuc|ur) (||. 92! 1 ).
I|e ceu||+| p+pu|+| puuc|ur | ||e ||e o| ||e
|eed|u +||e||o|e W||| r+cu|+| |+d|+||u |e|+u|ec
|+||c .ee|. up |o .5 cr |u d|+re|e|. uu+||,
o|||+|,.
0u d|+cop,, ||e |+d|+||u |e|+u|ec|+|+ ||+uc|e
+ud ||e ceu||+| +||e||o|e r+, pu|+|e.
\o| corrou|, occu| ou ||e |+ce, |o|e+|r, +ud
|+ud.
|| ||equeu||, occu| |u uo|r+| pe|ou +ud |
ro|e corrou |u |er+|e, occu| |u c|||d|eu.
|| r+, |e +oc|+|ed W||| |,pe|e||oeu|c |+|e,
uc| + p|eu+uc, (oue o| ro|e |u |Wo||||d o|
p|eu+u| Woreu), o| occu| |u p+||eu| |ece|.
|u e||oeu ||e|+p,, e.., o|+| cou||+cep||.e,
o| |u ||oe W||| |ep+|oce||u|+| d|e+e uc| +
u|+cu|e +ud c||ou|c .||+| |ep+|||| +ud +|co|o||c
c||||o| (||. 92! 3 ).
'p|de| +u|or+ +|||u |u c|||d|ood +ud p|e
u+uc, r+, |e|e pou|+ueou|,.
I|e |e|ou r+, |e cou|ued W||| |--!|c, |-
|c: |-|c-:|cc c|cc|-|c-:|cc o|
|-|c-:|cc |u ,|er|c c|e|ode|r+.
|e|ou r+, |e ||e+|ed e+||, W||| e|ec||o o| |+e|
u|e|,.
',,. |e.u +|+ueu, p|de| ue.u, +||e||+|
p|de|, p|de| |e|+u|ec|+|+, .+cu|+| p|de|.
SFI0k ANCI0MA |C|9 . ++3.
|C|0 . 13.
FICk 9-23 Spder oevus . IWo r+|| p+pu|e ||or W||c| |e|+u|ec|+|+ |+d|+|e. upou corp|e|ou ||e
|e|ou ||+uc|e corp|e|e|,. 8. 'p|de| ue.| ou ||e c|e| o| + p+||eu| W||| c||||o|.
8
FICk 9-24 veoous |ake . 0u ||e c|ee| o| + 10,e+|o|d r+|e. I|e |e|ou W+ +|ro| ||+c| +ud |ec+re
+ r+||e| o| couce|u |o ||e p+||eu|, W|o |e+|ed |e r||| |+.e re|+uor+. noWe.e|, || ||+uc|ed corp|e|e|, +||e|
corp|e|ou. 8. \euou |+|e ou ||e c|ee| o| +u 32,e+|o|d |er+|e. I|| ||u||||+c| uodu|e ||+uc|ed
corp|e|e|, +||e| corp|e|ou.
8
A .euou |+|e | + d+|| ||ue |o .|o|+ceou,
+,rp|or+||c, o|| p+pu|e |eu|||u ||or + d||+|ed
.euu|e, occu|||u ou ||e |+ce, ||p, +ud e+| o|
p+||eu| >50 ,e+| o| +e (||. 92+ 1 , 3 ).
I|e e||o|o, | uu|uoWu, |u| || |+ |eeu |e|+|ed
|o o|+| e\pou|e.
I|ee |e|ou +|e |eW |u uur|e| +ud |er+|u |o|
,e+|. I|e |e|ou |eu|| ||or + d||+|ed c+.||, ||ued
W||| + |u|e |+,e| o| ||+||eued eudo||e||+| ce||
+ud + |||u W+|| o| ||||ou ||ue ||||ed W||| |ed
||ood ce||.
|ue |o || d+|| ||ue o| ore||re e.eu ||+c|
co|o|, ||e |e|ou r+, |e cou|ued W||| uodu|+|
re|+uor+ o| p,oeu|c |+uu|or+.
I|e |e|ou c+u |e p+|||+||, corp|eed +ud ||||
eued up |, d|+cop,, +ud ||e ue o| de|rocop,
pe|r|| || e+, d|+uo| + + .+cu|+| |e|ou.
\+u+ereu| | |o| core||c |e+ou +ud c+u |e
+ccorp|||ed W||| e|ec||ou|e|,, |+e|, o|, |+|e|,,
W||| u||c+| e\c||ou.
vN0S IAk |C|9 . 523.5
|C|0 . K!.0
FICk 9-25 Cherry aoomas I|ee ||||| |ed, .|o|+ceou o| e.eu ||+c| |e|ou +ppe+| p|o|e|.e|, ou
||e ||uu| W||| +d.+uc|u +e.
C|e||, +u|or+ +|e e\ceed|u|, corrou,
+,rp|or+||c, ||||| |ed |o .|o|+ceou o| e.eu
||+c|, dored .+cu|+| |e|ou ( ! rr) (||.
925) o| occu|||u + r,||+d o| ||u, |ed p+pu|+|
po| |ru|+||u pe|ec||+e.
I|e, +|e |ouud p||uc|p+||, ou ||e ||uu|. I|e |e
|ou +ppe+| |||| +| +|ou| +e !0 +ud |uc|e+e |u
uur|e| o.e| ||e ,e+|.
I|e|e +|e |+|d|, +u, e|de||, peop|e W|o do uo|
|+.e +| |e+| + |eW |e|ou.
I|e |||o|o, cou|| o| uure|ou rode|+|e|,
d||+|ed c+p|||+||e ||ued |, ||+||eued eudo||e||+|
ce||, ||or+ | eder+|ou W||| |oroeu|/+||ou
o| co||+eu.
I|e, +|e o| uo couequeuce o||e| ||+u ||e||
core||c +ppe+|+uce. \+u+ereu| | e|ec||o o|
|+e| co+u|+||ou || |ud|c+|ed core||c+||,. C|,o
u|e|, | uo| e||ec||.e.
',,. C+rp|e|| de \o|+u po|, eu||e
(|er)+u|or+.
Chkk ANCI0MA |C|9 . 223.0
|C|0 . 13.3
FICk 9-26 Aookeratoma: so|tary I|| ||+c|, |||r |e|ou W||| + pe|||ed u||+ce |rred|+|e|, p+||
||e up|c|ou o| upe|||c|+| p|e+d|u re|+uor+. || | uoucorp|e|||e, |u| de|rocop, |e.e+| ||e |,p|c+| |+cu
u+e o| |||or|oed .+cu|+| p+ce. |oue||e|e, uc| |e|ou |ou|d |e e\c|ed.
I|e |e|r c ('||ood .ee|) |-c|c Wou|d
|rp|, + .+cu|+| |uro| W||| |e|+|o||c e|ereu|.
Bu|, |u |+c|, c+p|||+||e +ud po|c+p|||+|, .euu|e
+|e p+c|ed |u|o ||e p+p|||+|, |od, ju| |eue+||
+ud |u||u |u|o ||e ep|de|r|, |e+d|u |o |,pe|
|e|+|o|. I|| +ud ||e |+c| ||+| ||e |ur|u+ +|e
uu+||, +| |e+| p+|||+||, |||or|oed |rp+|| + |||r
cou||euc, |o ||e |e|ou.
Au|o|e|+|or+ +|e d+|| .|o|+ceou |o ||+c|,
o||eu |e|+|o||c p+pu|e o| r+|| p|+que ||+| +|e
|+|d upou p+|p+||ou +ud c+uuo| |e corp|eed
|, d|+cop, (||. 92o).
Au|o|e|+|or+ c+u +ppe+| + + o|||+|, |e|ou
(||c, c|-c|c ), +ud ||eu ||e ro|
|rpo||+u| d|||e|eu||+| d|+uo| | + r+|| uodu|+|
o| upe|||c|+| p|e+d|u re|+uor+ (||. 92o).
I|e ro| corrou | c|-c|c | |!,:- ,
||| d|e+e |u.o|.e ||e c|o|ur +ud .u|.+, ||e
|e|ou +|e ru|||p|e p+pu|e ( + rr) ||+| +|e
d+|| |ed |u co|o| +ud p|eeu| |u qu||e |+|e uur
|e| (||. 921).
1|-c|c | !|-|| corp||e p|u| |o
d+|| |ed +ud e.eu ||+c| p+pu|e ||+| occu| ou
||e e||oW, |uee, +ud do|+ o| ||e |+ud. I||
+u|oor+| dor|u+u| d|e+e | |+|e +ud occu| |u
,ouu |er+|e.
1|-c|c : !|| ( |c|, !-c-),
+u /||u|ed |ece|.e d|e+e, | +u |u|o|u e||o| o|
re|+|o||r |u W||c| ||e|e | + de||c|euc, o|
+|+c|o|d+e A |e+d|u |o +u +ccuru|+||ou o|
ueu||+| |,cop||uo||p|d ce|+r|de ||||e\o|de |u
eudo||e||+| ce||, ||||oc,|e, +ud pe||c,|e |u ||e
de|r|, |e+||, ||due,, +ud +u|ouor|c ue|.ou
,|er. |e|ou +|e uure|ou d+|| |ed, puuc|+|e,
+ud ||u, ( rr) (||. 923), |oc+|ed ou ||e
|oWe| |+|| o| ||e |od,. |oWe| +|doreu, eu||+||+,
+ud |u||oc|, +|||ou| |e|ou r+, +|o occu| ou
||e ||p. I|e |oro/,ou r+|e |+.e uo| ou|, ||e
||u |e|ou |u| +|o ,rp|or |e|+|ed |o |u.o|.e
reu| o| o||e| o|+u ,|er. +c|op+|e||e|+,
e\c|uc|+||u p+|u, ||+u|eu| |c|er|c +||+c|, +ud
r,oc+|d|+| |u|+|c||ou. ne|e|o/,ou |er+|e r+,
|+.e co|ue+| op+c|||e. |+||, d|e+e | |+|e.
ANCI0kkAI0MA |C|9 . ++3.9
|C|0 . \9+/0
*
FICk 9-2T Aookeratoma oI
Fordyce kedd||, .|o|+ceou +ud ||+c|
p+pu|e ou ||e c|o|ur. I|e, ||+uc| upou
d|+cop, +ud ||| .e||||e ||e d|+uo|.
|o|e. I||or|oed +u|o|e|+|or+ do uo|
||+uc|.
FICk 9-28 Aookeratoma
corpors dIIusum (Fabry dsease)
|ure|ou |ed, puuc|+|e |e|ou ou
||e |oWe| ||+u|.
IMFhAIIC MAIF0kMAII0N (IM)
FICk 9-29 Iymphatc ma|Iormatoo (|ymphaooma) ||op+Wu|||e cou||ueu| |ouped '.e|c|e
||||ed W||| + e|o+uu|ueou ||u|d.
I|e |e|r |,|c|: c||c| | ||e |e|r|
uo|o, |o| W|+| W+ |o|re||, c+||ed '|,rp|+u|
or+.
I|ee |,p|c+| |e|ou corp||e ru|||p|e, |ouped,
r+|| r+c|ocop|c .e|c|e ||||ed W||| c|e+| o|
e|o+uu|ueou ||u|d ('||op+Wu) (||. 9
29). noWe.e|, ||ee +|e uo| ||ue .e|c|e |u|
r|c|oc,||c |e|ou (|,rp|+u|or+) + oppoed
|o + r+c|oc,||c |e|ou (c,||c |,|or+), W||c|
| |oc+|ed deep |u ||e de|r| +ud u|cu|| +ud
+ppe+| + + |+|e o|| u|cu|+ueou |uro| o||eu
d||o|||u ||e |+ce o| +u e\||er||,.
I|e r|c|oc,||c |\ | p|eeu| +| ||||| o| +ppe+|
|u |u|+uc, o| c|||d|ood. || r+, d|+ppe+| pou
|+ueou|,, |u| ||| | e\||ere|, |+|e. B+c|e||+|
|u|ec||ou r+, occu|.
|\ r+, occu| + +u |o|+|ed o|||+|, |e|ou, +
|u ||. 929, o| co.e| |+|e +|e+ (up |o 0 20
cr), || r+, |e +oc|+|ed W||| + c+p|||+|, .euou
|,rp|+||c (C\|) r+||o|r+||ou.
I|e |e|ou c+u |e e\c|ed, || |e+|||e, o| ||e+|ed
W||| c|e|o||e|+p,.
IMFhANCI0MA |C|9 . 223.
|C|0 . |3.\910/0
vAkIANIS
vascu|ar hamartomas CVLs with deep soft
tissue involvement and iesultant swelling oi dif-
fuse enlaigement of an extiemity. May involve
skeletal muscle with muscle atiophy. Cutaneous
changes include dilated toituous veins and aite-
iiovenous fistulas.
C\\ +|e deep .+cu|+| r+||o|r+||ou c|+|+c|e|
|/ed |, o||, corp|e|||e deep||ue We|||u.
|e|ou +|e uo| +pp+|eu| +| ||||| |u| |ecore o
du||u c|||d|ood.
I|e, r+u||e| + o|| ||ue We|||u, dore
|+ped o| ru|||uodu|+| (||. 9!0), +ud +|e
|oW||oW |e|ou. w|eu .+cu|+| r+||o|r+||ou
e\|eud |o ||e ep|de|r|, ||e u||+ce r+, |e .e|
|ucou. I|e |o|de| +|e poo||, de||ued, +ud ||e|e
| cou|de|+||e .+||+||ou |u |/e. 0||eu, C\\ +|e
uo|r+| ||u co|o|, W||| ||e uodu|+| po|||ou ||ue
|o pu|p|e. I|e, +|e e+||, corp|eed +ud ||||
p|orp||, W|eu p|eu|e | |e|e+ed. 'ore |,pe
r+, |e |eude|, +ud ||e, r+, |e +oc|+|ed W|||
C\.
C\\ r+, |e corp||c+|ed |, u|ce|+||ou +ud
||eed|u, c+|||u, +ud ecoud+|, |u|ec||ou, +ud,
W||| |+|e |e|ou, |, |||ou|pu| |e+|| |+||u|e.
C\\ r+, |u|e||e|e W||| |ood |u|+|e o| ||e+|||u
+ud, || |oc+|ed ou ||e e,e||d o| |u ||e .|c|u||,
o| ||e e,e, W||| o|||uc| .||ou +ud r+, |e+d |o
|||udue.
I|e|e | uo +|||+c|o|, ||e+|reu| e\cep| cor
p|e|ou. |u |+|e| |e|ou-|| o|+u |uuc||ou |
corp|or|ed-u||c+| p|ocedu|e +ud |u||+.+
cu|+| co+u|+||ou |ou|d |e pe||o|red. n||
doe ,|er|c |ucoco|||co|d o| ||| r+, |e
e||ec||.e.
|C|9 . 151.!2
CAFIIIAk[vN0S MAIF0kMAII0NS (CvMs)
k|ppe|-Iroauoay Syodrome A CVM oi
CVL malfoimation, slow-flow lesion. Local
oveigiowth of soft tissue and bone iesults in
enlaigement of an extiemity. Associated cu-
taneous changes include phlebectasia, nevus
flammeus-like cutaneous CM (Fig. 9-31), lym-
phatic hypoplasia, and lymphedema.
FICk 9-30 Cap||ary-veoous ma|Iormatoo |u +u |u|+u|. I|e|e | + o||, corp|e|||e, ||u|||ed ||ue
We|||u d||o|||u ||e uppe| ||p +ud |oWe| e,e||d. || | + |oW||oW |e|ou |u| |equ||e ||e|+peu||c |u|e|.eu||ou.
8|ue kubber 8|eb Nevus A venous malfoi-
mation that is spontaneously painful and/oi
tendei. It is a compiessible, soft, blue swelling in
the deimis and subcutaneous tissue. Size ianges
fiom a few millimeteis to seveial centimeteis
(Fig. 9-32). The lesion may exhibit localized
hypeihidiosis ovei CVL malfoimations and
occuis, often multiply, on the tiunk and uppei
aims. Similai vasculai lesions can occui in the
gastiointestinal tiact and may be a souice of
hemoiihage.
MarIucc Syodrome A slow-flow venous oi
lymphatic/venous malfoimation associated
with enchondiomas and manifested as haid
nodules on fingeis oi toes and as bony defoimi-
ties. Patients may develop chondiosaicoma.
Farkes-Weber Syodrome A fast-flow capillaiy
aiteiiovenous malfoimation (CAVM) oi CM,
with soft tissue and skeletal hypeitiophy.
FICk 9-31 Cap||ary-veoous ma|Iormatoo |u +
!,e+|o|d Wor+u. I|| ue.u ||+rreu-|||e |e|ou W+
+oc|+|ed W||| p||e|ec|+|+, |,rp|eder+, +ud +u eu|+|ed
|||| |oWe| e\||er||, (K||ppe|I|eu+uu+, ,ud|ore).
FICk 9-32 8|ue rubber b|eb oevus A pou|+ueou|, p+|u|u| +ud |eude| .euou r+||o|r+||ou. I|e|e
+|e + uur|e| o| corp|e|||e ||u||.|o|+ceou p+pu|e +ud uodu|e ou ||e uppe| +|r.
MISCIIAN0S CSIS AN0 FS00CSIS
FICk 9-33 pdermod cyst . A |ouuded uodu|e W||||u ||e de|r|. |o| +|W+, | ||e|e +u opeu|u
|||ou| W||c| c+eou |e|+||uou r+|e||+| c+u |e e\p|eed. 8. kup|u|ed ep|de|ro|d c,|. I|ee |u||+rr+|o|,
|e|ou +|e o||eu r|d|+uoed + |e|u |u|ec|ed.
8
Au ep|de|ro|d c,| | ||e ro| corrou cu|+ue
ou c,|, de||.ed ||or ep|de|r| o| ||e ep|||e
||ur o| ||e |+|| |o|||c|e, +ud | |o|red |, c,||c
euc|ou|e o| ep|||e||ur W||||u ||e de|r| ||+|
|ecore ||||ed W||| |e|+||u +ud ||p|d||c| de|||.
|| occu| |u ,ouu |o r|dd|e+ed +du|| ou ||e
|+ce, uec|, uppe| ||uu|, +ud c|o|ur.
I|e |e|ou, W||c| | uu+||, o|||+|, |u| r+, |e
ru|||p|e, | + de|r+||ou|cu|+ueou uodu|e,
0.5-5 cr, W||c| o||eu couuec| W||| ||e u||+ce
|, |e|+||u||||ed po|e (||. 9!! 1 ).
I|e c,| |+ +u ep|de|r+||||e W+|| (||+||||ed
qu+rou ep|||e||ur W||| We|||o|red |+uu|+|
|+,e|), ||e cou|eu| o| ||e c,| | |e|+||u+ceou
r+|e||+|-c|e+rco|o|ed W||| + p+|, cou||euc,
+ud ||e odo| o| |+uc|d c|eee. 'c|o|+| |e|ou r+,
c+|c||,.
I|e c,| W+|| | |e|+||.e|, |||u. |o||oW|u |up|u|e
o| ||e W+||, ||e |||||+||u c,| cou|eu| |u|||+|e
+u |u||+rr+|o|, |e+c||ou, eu|+||u ||e |e|ou
r+u,|o|d, ||e |e|ou | uoW +oc|+|ed W||| +
|e+| de+| o| p+|u. kup|u|ed c,| (||. 9!! 3 ) +|e
o||eu r|d|+uoed + |e|u |u|ec|ed |+||e| ||+u
|up|u|ed.
',, weu, e|+ceou c,|, |u|uud||u|+|
c,|, ep|de|r+| c,|.
FI0kM0I0 CSI |C|9 . 10o.2
|C|0 . |12.0
FICk 9-34 Irch|emma| cyst A |||r, dore
|+ped uodu|e ou ||e c+|p. ||eu|e |, ||e c,|
|+ c+ued +||op|, o| |+|| |u|| +ud || ||u +ppe+|
W|||ou| |+||.
FICk 9-35 pderma| oc|usoo cyst A
r+|| de|r+| uodu|e ou ||e |uee +| ||e ||e o| ||e
|+ce|+||ou.
A |||c|||err+| c,| | ||e ecoud ro| corrou
|,pe o| cu|+ueou c,| +ud | eeu ro| o||eu |u
r|dd|e +e, ro|e ||equeu||, |u |er+|e. || | o||eu
|+r|||+| +ud occu| ||equeu||, + ru|||p|e |e|ou.
I|ee +|e roo||, |||r, dore|+ped, 0.5 |o
5cr uodu|e o| |uro|, ||e, |+c| ||e ceu||+|
puuc|ur eeu |u ep|de|ro|d c,|. I|e, +|e uo|
couuec|ed |o ||e ep|de|r|.
0.e| 90 occu| ou ||e c+|p, +ud ||e o.e||,|u
c+|p |+|| | uu+||, uo|r+| |u| r+, |e |||uued ||
||e c,| | |+|e (||. 9!+).
I|e c,| W+|| | uu+||, |||c|, +ud ||e c,| c+u |e
|ero.ed |u|+c|. I|e W+|| | + ||+||||ed qu+rou
ep|||e||ur W||| + p+||+ded ou|e| |+,e| |eer|||u
||+| o| ||e ou|e| |oo| |e+|| o| |+|| |o|||c|e. I|e
|uue| |+,e| | co||u+|ed W|||ou| + |+uu|+| |+,e|.
I|e c,| cou|+|u |e|+||u-.e|, deue, |oroe
ueou, || | o||eu c+|c|||ed, W||| c|o|e|e|o| c|e||.
|| c,| |up|u|e, || r+, |e |u||+red +ud .e|,
p+|u|u|.
',, |||+| c,|, |||ru c+|+eu c,|. 1
:|c: |e|r. weu, e|+ceou c,|.
IkIChIIMMAI CSI |C|9 . 10o.2
|C|0 . |12.0
Au ep|de|r+| |uc|u|ou c,| occu| ecoud+|,
|o ||+ur+||c |rp|+u|+||ou o| ep|de|r| |u|o ||e
de|r|. I|+ur+||c+||, |+||ed ep|de|r| |oW |u
||e de|r|, W||| +ccuru|+||ou o| |e|+||u W||||u
||e c,| c+.||,, euc|oed |u + ||+||||ed qu+rou
ep|||e||ur W||| + We|||o|red |+uu|+| |+,e|.
I|e |e|ou +ppe+| + + de|r+| uodu|e (||. 9!5)
+ud ro| corrou|, occu| ou ||e p+|r, o|e,
+ud ||ue|.
|| |ou|d |e e\c|ed.
',, . I|+ur+||c ep|de|ro|d c,|.
FI0kMAI INCISI0N CSI
FICk 9-36 M|um . A r+|| c|+||W|||e
o| ,e||oW|| p+pu|e ou ||e c|ee|, || c+u |e |||
W||| + c+|pe|, |e|e+|u + |||||e |+|| o| |o|u, r+|e
||+|. 8. A |||||, |+|e| |e|ou ou ||e |oWe| ||d o|
+u A|||c+u Wor+u. C. \u|||p|e r|||+ ou ||e ||uu|
o| + c|||d W||| |e|ed||+|, d,||op||c ep|de|ro|,|
|u||o+ (ee 'ec||ou o).
8
C
A r|||ur | + |o 2rr, upe|||c|+|, W|||e |o
,e||oW, |e|+||ucou|+|u|u ep|de|r+| c,|, occu|
||u ru|||p|,, |oc+|ed ou ||e e,e||d, c|ee|, +ud
|o|e|e+d |u p||oe|+ceou |o|||c|e (||. 9!o 1, 3 ).
I|e |e|ou c+u occu| +| +u, +e, e.eu |u |u|+u|.
\|||+ +||e e|||e| de uo.o, epec|+||, +|ouud ||e
e,e, o| |u +oc|+||ou W||| .+||ou de|r+|oe
W||| u|ep|de|r+| |u||+e o| .e|c|e (perp||
o|d, po|p|,||+ cu|+ue+ |+|d+, |u||ou ||c|eu p|+
uu, ep|de|ro|,| |u||o+) (||. 9!o C ) +ud ||u
||+ur+ (+||+|ou, |u|u, de|r+||+|ou, |+d|+||ou
||e|+p,).
|uc||ou +ud e\p|e|ou o| cou|eu| +|e ||e
re||od o| ||e+|reu|.
MIIIM
FICk 9-3T 0ta| myxod cyst . I|e c,| |+ |ed |o + ! |o +rr |oo.e o| ||e u+|| p|+|e. 8. '|||||u
|| W||| + c+|pe| +ud p|eu|e |e|e+e + e|+||uou .|cou ||u|d.
8
A d|||+| r,\o|d c,| | + peudoc,| occu|||u
o.e| ||e d||+| |u|e|p|+|+ue+| jo|u| +ud ||e |+e
o| ||e u+|| o| ||e ||ue| (||. 9!1 1 ) o| |oe, o||eu
+oc|+|ed W||| ne|e|deu' (o|eop|,||c) uode.
I|e |e|ou occu| |u o|de| p+||eu|, uu+||, >o0
,e+| o| +e.
|| | uu+||, + o|||+|, c,|, |u||e|,, ||+u|uceu|.
A c|e+| e|+||uou .|cou ||u|d r+, |e e\||uded
(||. 9!1 3 ).
w|eu ||e r,\o|d c,| | o.e| ||e u+|| r+|||\, +
u+|| p|+|e d,||op|, occu| |u ||e |o|r o| + |o
2rr |oo.e ||+| e\|eud |o ||e |eu|| o| ||e
u+|| (||.9!1 1 , !!2). ('ee 'ec||ou !!.)
\+||ou re||od o| r+u+ereu| |+.e |eeu
+d.oc+|ed, |uc|ud|u u||c+| e\c||ou, |uc||ou
+ud d|+|u+e, |ujec||ou o| c|e|o|u r+|e||+|, +ud
|ujec||ou o| + |||+rc|uo|oue upeu|ou. A |rp|e
+ud ro| e||ec||.e re||od | |o r+|e + r+||
|uc||ou, e\p|e ||e e|+||uou cou|eu|, +ud ue
+ |||r corp|e|ou |+ud+e o.e| ||e |e|ou o.e|
+ pe||od o| Wee|.
',, . \ucou c,|, ,uo.|+| c,|, r,\o|d
peudoc,|.
0ICIIAI MX0I0 CSI |C|9 . 121.+
|C|0 . \25.3
FI0MI0I0C
0oset Raiely befoie 30 yeais.
Sex Slightly moie common and moie exten-
sive involvement in males.
Evolve ovei months to yeais. Raiely piuiitic;
tendei if secondaiily infected.
Sko Iesoos Eur|y Small, 1- to 3-mm, baiely
elevated papule, latei a laigei plaque (Figs. 9-38
MISCIIAN0S 8NICN N0FIASMS AN0 hFkFIASIAS
FICk 9-38 Seborrhec keratoss, so|tary A |||||, |+|ed, |e|+|o||c, ||oWu, ||+| p|+que ou ||e /,o
r+||c |e|ou |u +u o|de| |er+|e. I|e d|||e|eu||+| d|+uo| |uc|ude |eu||o r+||u+ +ud |eu||o r+||u+ re|+uor+.
I|e e|o|||e|c |e|+|o| | ||e ro| corrou o|
||e |eu|u ep|||e||+| |uro|.
I|ee |e|ou, W||c| +|e |e|ed||+|,, do uo| +ppe+|
uu||| +e !0 +ud cou||uue |o occu| o.e| + |||e||re,
.+|,|u |u e\|eu| ||or + |eW c+||e|ed |e|ou |o
|||e|+||, |uud|ed |u ore .e|, e|de||, p+||eu|.
|e|ou |+ue ||or r+||, |+|e|, e|e.+|ed p+pu|e
|o p|+que W||| + W+||, u||+ce +ud + '|uc| ou
+ppe+|+uce.
|e|ou +|e |eu|u +ud do uo| |equ||e ||e+|reu|
e\cep| |o| core||c |e+ou. I|e, c+u |ecore
|||||+|ed o| ||+ur+||/ed, W||| p+|u +ud ||eed|u.
'CC |ou|d |e |u|ed ou|.
',, .-:c -||:c
S80kkhIC kkAI0SIS |C|9 .102.
|C|0 . |32
and 9-39) with oi without pigment. The suiface
has a gieasy feel and often shows, with a hand
lens, fine stippling like the suiface of a thimble.
Elevation can be demonstiated by lightly fieezing
the lesion with LN2. Lesions such as lentigo
(benigna oi maligna) aie macules; SKs have a
shaiply maiginated elevated edge in compaiison.
Lute Plaque with waity suiface and stuck on"
appeaiance (Fig. 9-40), gieasy." With a hand
lens hoin cysts can often be seen; with deimos-
copy they can always be seen and aie diagnostic.
Size fiom 1 to 6 cm. Flat nodule. Biown, giay,
black, skin-coloied, iound oi oval (Figs. 9-39
and 9-40 , B ).
DIstrIhutIvn Isolated lesion oi geneialized.
Face, tiunk (Fig. 9-41), uppei extiemities. In
daik skinned people, multiple, small black
lesions in the face aie called Jermaoss au|osa
ngra (Fig. 9-39). SKs aie most dense in sun-
exposed site with deimatoheliosis. When nu-
meious and dense, SKs may become confluent.
In females, commonly occui in submammaiy
inteitiiginous skin.
0ermatopatho|oy Piolifeiation of mono-
moiphous keiatinocytes (with maiked papil-
lomatosis) and melanocytes, foimation of hoin
cysts. Some lesions can exhibit atypia of keiati-
nocytes, mimicking Bowen disease (SCCIS), flat
oi squamous cell caicinoma (SCC), and these
should be excised.
Clinically, the diagnosis is made easily. Cuiet-
tage may be helpful: seboiiheic keiatosis comes
off easily aftei slight fieezing and peimits his-
topathologic examination.
"Iao Macu|es" Eaily flat" lesions may be con-
fused with solai lentigo oi spieading pigmented
actinic keiatosis (see Fig. 10-28).
Sko-Co|ored[Iao[8|ack verrucous Fapu|es[
F|aques Laigei pigmented lesions aie easily
mistaken foi pigmented basal cell caicinoma
(BCC) oi malignant melanoma (only biopsy
will settle this, oi deimoscopy will be of assist-
ance); veiiuca vulgaiis may be similai in clini-
cal appeaiance, but thiombosed capillaiies aie
piesent in veiiucae.
C0kS AN0 Fk0CN0SIS
Lesions develop with incieasing age; they aie
benign and do not become malignant.
MANACMNI
Light electiocauteiy peimits the whole lesion
to be easily iubbed off. Then the base can
be lightly cauteiized to pievent iecuiience.
This, howevei, piecludes histopathologic veii-
fication of diagnosis and should be done only
by an expeiienced diagnostician. Ciyosuigeiy
with liquid nitiogen spiay woiks only in flat
lesions, and iecuiiences aie possibly moie
fiequent. The best appioach is cuiettage af-
tei slight fieezing with ciyospiay, which also
peimits histopathologic examination. In a
solid black lesion without hoin cysts, a punch
biopsy is mandatoiy to iule out malignant
melanoma; in this case a shave biopsy should
not be peifoimed as, in the case of melanoma,
it will not peimit evaluation of the level of
invasion.
FICk 9-39 Seborrhec keratoss (dermatoss papu|osa ora) I|| cou|| o| + r,||+d o| ||u, ||+c|
|e|ou, ore eu|+||u |o ro|e ||+u + ceu||re|e|. I|| | eeu |u B|+c| A|||c+u, A|||c+u Are||c+u, +ud deep|,
p|reu|ed 'ou|| E+| A|+u. I|e+|reu| | + p|o||er |ec+ue |,pop|reu|ed po| c+u +||e +| ||e W|e|e ||ee
e|o|||e|c |e|+|oe |+.e |eeu |ero.ed.
FICk 9-41 Seborrhec keratoses, mu|tp|e \u|||p|e ||oWu, W+||, p+pu|e +ud uodu|e ou ||e |+c|,
|+.|u + '|e+, |ee| +ud '|uc| ou +ppe+|+uce. I|| p|c|u|e +|o |oW ||e e.o|u||ou o| ||e |e|ou. ||or r+||
ou|, |||||, |+u, .e|, |||u p+pu|e o| p|+que |o |+|e|, d+||e| uodu|+| |e|ou W||| + .e||ucou u||+ce. ||+c||c+||,
+|| |e|ou ou ||e |+c| o| ||| e|de||, p+||eu| +|e e|o|||e|c |e|+|oe, W|+| ||e, |+.e |u corrou | ||+| ||e, |.e
||e |rp|e|ou ||+| ||e, cou|d |e c|+ped o|| e+||,, W||c|, |u |+c|, ||e, c+u.
FICk 9-40 Seborrhec keratoss . 'r+||, |e+.||, p|reu|ed e|o|||e|c |e|+|oe c+u |+.e + roo||
u||+ce +ud p|eeu| + d|||e|eu||+| d|+uo||c c|+||eue. p|reu|ed |++| ce|| c+|c|uor+ +ud uodu|+| re|+uor+
|+.e |o |e e\c|uded. 8. |+|e e|o|||e|c |e|+|oe |+.e + '|uc| ou +ppe+|+uce, c+u |e .e|, d+|| +ud |||eu|+|.
|ue |o ||e|| ru|||p||c||, ||e, uu+||, do uo| p|eeu| + d|+uo||c p|o||er. A |oWu |e|e, ||e, c+u |e d|||u||u.
8
FICk 9-42 8ecker oevus . A |||||, |+|ed |||||+u p|+que W||| |+|p|, de||ued +ud ||||, |||eu|+|
|o|de| +ud |+|e|, .||||e |,pe||||c|o| ou ||e c|e| o| + o,e+|o|d r+|e p+||eu|. 8. |u ||| c+e o| Bec|e| ue.u
||e r+|.e |,pe||||c|o| couce+| ||e |+u |+c||ouud p|+que.
8
Bec|e| ue.u (B|) | + d|||uc||.e +,rp|o
r+||c c||u|c+| |e|ou ||+| | + p|reu|ed |+r+|
|or+-|. e., + de.e|opreu|+| +uor+|, cou|||u
o| c|+ue |u p|reu|+||ou, |+|| |oW||, +ud +
|||||, e|e.+|ed roo|| .e||ucou u||+ce (||.
9+21 +ud 3 ).
|| occu| ro||, |u r+|e +ud |u +|| |+ce. || +p
pe+| uo| +| ||||| |u| uu+||, |e|o|e 5 ,e+| o|
+e +ud ore||re +||e| ||| +e.
I|e |e|ou | p|edor|u+u||, + r+cu|e |u| W||| +
p+pu|+| .e||ucou u||+ce uo| uu|||e ||e |e|ou o|
+c+u||o| u|||c+u. || | |||| ||oWu |u co|o| +ud
|+ + eo|+p||c p+||e|u W||| |+|p|, der+|c+|ed
|o|de| (||. 9+2 1 ).
Corroue| |oc+||ou +|e ||e |ou|de| +ud ||e
|+c|. I|e |uc|e+ed |+|| |oW|| |o||oW ||e oue|
o| ||e p|reu|+||ou +ud | |oc+||/ed |o ||e +|e+
||+| +|e p|reu|ed. I|e p|reu|+||ou | |e|+|ed
|o |uc|e+ed re|+u|u |u |++| ce|| +ud uo| |o +u
|uc|e+ed uur|e| o| re|+uoc,|e.
|| | d|||e|eu||+|ed ||or + |+||, coueu||+| re|+uo
c,||c ue.u, |ec+ue B| | uo| uu+||, p|eeu| +|
|||||, +ud ||or c+|e +u |+|| r+cu|e |ec+ue ||ee
+|e uo| |+||,.
I|e |e|ou e\|eud |o| + ,e+| o| |Wo +ud ||eu
|er+|u |+||e, ou|, |+|e|, |+d|u.
I|e|e | .e|, |+|e|, |,pop|+|+ o| uude||,|u
||uc|u|e, e.., |o||eu|u o| ||e +|r o| |educed
||e+| de.e|opreu| |u +|e+ uude| ||e |e|ou.
\+u+ereu|. ||e |,pe||||c|o| c+u |e o| co
re||c couce|u |o ore |ud|.|du+|.
8Ckk NvS |C|9 . 2o
|C|0 . \3120/0
I||c|oep|||e||or+ +|e |eu|u +ppeud+e |uro|
W||| |+|| |u|| d|||e|eu||+||ou.
I|e |e|ou, W||c| +ppe+| +| pu|e||,, occu| ou
||e |+ce +ud |e o||eu ou ||e c+|p, uec|, +ud
uppe| ||uu| (||. 9+! 1 ).
|e|ou r+, |e ou|, + |eW r+|| p|u| o| ||u
co|o|ed p+pu|e +| |||| |+du+||, |uc|e+e |u
uur|e| +ud r+, |ecore qu||e |+|e +ud |e
cou|ued W||| BCC (||. 9+! 1 ).
I||c|oep|||e||or+ c+u +|o +ppe+| + o|||+|,
|uro|, W||c| r+, |e uodu|+|, o| +ppe+| + |||
de||ued p|+que |||e c|e|o|u BCC (||. 9+! 3 ).

IkICh0FIIhII0MA |C|9 . \300/0 |C|0 . |2!
',||uor+ +|e |eu|u +deuor+ o| ||e ec
c||ue duc|. I|e, +|e |o 2rr, ||uco|o|ed
o| ,e||oW, |||r p+pu|e ||+| occu| ro||, |u
Woreu, |e|uu|u +| pu|e||,, ||e, r+, |e
|+r|||+|.
\o| o||eu ru|||p|e |+||e| ||+u o|||+|,, ||e,
occu| ro| ||equeu||, ou |oWe| pe||o||||+| +|e+,
uu+||, ,rre|||c+||, |u| +|o ou ||e e,e||d
(||. 9++) +ud ou ||e |+ce, +\|||+e, ur||||cu,
uppe| c|e|, +ud .u|.+.
I|e |e|ou |+.e + pec|||c |||o|o|c p+||e|u.
r+u, r+|| duc| |u ||e de|r| W||| corr+|||e
|+|| W||| ||e +ppe+|+uce o| '|+dpo|e.
I|e |e|ou c+u |e d|||u||u, +ud ro| p+||eu|
W+u| ||er |ero.ed, ||| c+u |e doue e+||, W|||
e|ec||ou|e|,.
SkINC0MA |C|9 . 2o.0 2o.9
|C|0 . |2! \3+01/0

FICk 9-43 Irchoepthe|omas . \u|||p|e, r+||, |+|p|, de||ued roo|| p+pu|e ||+| |oo| |||e e+||,
BCC.

FICk 9-44 Syroomas ',rre|||c e|up||ou o| |o 2rr ||uco|o|ed, roo|| p+pu|e ou ||e uppe|
+ud |oWe| e,e||d.
FICk 9-43 Irchoepthe|omas (Cootoued) 8. I||c|oep|||e||or+, o|||+|, |,pe. A uodu|+| |uro| ou ||e
uppe| ||p ||+| c+u |e cou|ued W||| + |++| c+|c|uor+ o| qu+rou ce|| c+|c|uor+.
8
I|ee +|e .e|, corrou |e|ou |u o|de| pe|ou
+ud +|e cou|ued W||| r+|| BCC. A|o occu|
|u o||d o|+u ||+up|+u| |ec|p|eu| ||e+|ed W|||
c,c|opo||ue. I|e |e|ou +|e |o ! rr |u d|+
re|e| +ud |+.e |o|| |e|+u|ec|+|+ +ud ceu||+|
ur||||c+||ou (||. 9+5).
IWo |e+|u|e d|||uu|| e|+ceou |,pe|p|+|+
||or uodu|+| BCC. () e|+ceou |,pe|p|+|+ |
o|| |o p+|p+||ou, uo| |||r + |u uodu|+| BCC (uo|
upe|||c|+|), +ud (2) W||| |||r |+|e|+| corp|e|ou
|| | o||eu po|||e |o e||c|| + .e|, r+|| |o|u|e o|
e|ur |u ||e .+||e, o| ||e ur||||c+|ed po|||ou o|
||e |e|ou.
'e|+ceou |,pe|p|+|+ c+u |e de||o,ed W|||
|||| e|ec||oc+u|e|,.
S8AC0S hFkFIASIA |C|9 . 10o.9
I|| coueu||+| r+||o|r+||ou o| e|+ceou d||
|e|eu||+||ou occu| ou ||e c+|p o|, |+|e|,, ou ||e
|+ce (||. 9+o).
A |+|||e, |||u, e|e.+|ed, |o 2cr p|+que,
ore||re |+|e|, W||| + c|+|+c|e||||c o|+ue
co|o| +ud + pe|||, o| W+||, u||+ce.
A|ou| 0 o| p+||eu| c+u |e e\pec|ed |o de
.e|op BCC |u ||e |e|ou.
E\c||ou | |ecorreuded +| +|ouud pu|e||, |o|
core||c |e+ou +ud |o p|e.eu| ||e occu||euce
o| BCC.
',, . 0|+uo|d ue.u.
NvS S8AC0S |C|9 . 2o.!
A ||e u+re -. |rp||e ||| | + de.e|opreu
|+| (|+r+||or+|ou) d|o|de| c|+|+c|e||/ed |,
|,pe|p|+|+ o| ep|de|r+| ||uc|u|e (ep|de|r|
+ud +due\+). I|e|e +|e uo ue.ore|+uoc,||c
ue.u ce||.
Ep|de|r+| ue.u | uu+||, p|eeu| +| ||||| o|
occu| |u |u|+uc,, |+|e|,, || de.e|op |u pu|e||,.
A|| ep|de|r+| ue.| ou ||e |e+d/uec| |e|ou +|e
p|eeu| +| |||||.
I|e|e +|e e.e|+| .+||+u| o| ep|de|r+| ue.|. I|e
.-: -!-c| -. r+, |e |oc+||/ed o|
ru|||p|e. I|e |e|ou +|e ||uco|o|ed, ||oWu, o|
|+,||||oWu (||. 9+1) +ud +|e corpoed o|
c|oe|, e| .e||ucou p+pu|e, We|| c||curc|||ed,
||e, +|e o||eu |u + ||ue+| +||+uereu|-epec|+||,
ou ||e |e-o| ||e, r+, +ppe+| |u B|+c||o ||ue
ou ||e ||uu|. E\c||ou | ||e |e| ||e+|reu|, || |e+
|||e. B|op, o| ||e |e|ou |ou|d |e cou|de|ed |o
|u|e ou| BCC.
w|eu ||e |e|ou +|e e\|eu|.e ||e, +|e |e|red
,|-c|c-! -!-c| -. , +ud W|eu ||e,
+|e |oc+|ed ou |+|| ||e |od, ||e, +|e |e|red
-. |c|- .
I|e |e|ou c+u e\||||| e|,||er+, c+||u, +ud
c|u||u +ud +|e ||eu c+||ed ||cc|, |-c
.-: -!-c| -. (||\E|). I|e |e|ou
|+du+||, eu|+|e +ud |ecore |+||e |u +do|e
ceuce.
I|e|e | +|o + ||cc|, |-c .-:
-!-c| -. (|||\E|).
E\|eu|.e ep|de|r+| ue.| ( -!-c| -. ,
!- ) r+, |e ru|||,|er d|o|de| +ud r+,
|e +oc|+|ed W||| de.e|opreu|+| +|uo|r+||||e
(|oue c,|, |,pe|p|+|+ o| |oue, co||o|, p|u+
||||d+, |,p|o|), .||+r|u |-|e||+u| ||c|e|, +ud
ueu|o|o|c p|o||er (reu|+| |e|+|d+||ou, e|
/u|e, co|||c+| +||op|,, |,d|ocep|+|u). I|ee
p+||eu| |equ||e + corp|e|e e\+r|u+||ou, |uc|ud
|u ||e e,e (c+|+|+c|, op||c ue|.e |,pop|+|+),
+ud c+|d|+c |ud|e |o |u|e ou| +ueu|,r, p+|eu|
duc|u +||e||ou.
FI0kMAI NvS |C|9 . 2o
FICk 9-45 Sebaceous hyperp|asa |o +rr roo|| p+pu|e W||| ceu||+| ur||||c+||ou ou ||e |o|e|e+d.
FICk 9-46 Nevus sebaceous Au e|e.+|ed
p|+que o| o|+ue co|o| +ud pe|||, u||+ce. |o|e ||+|
||e |e|ou | |+|||e ou ||e c+|p.
FICk 9-4T pderma| oevus A |+,||
|||eu|+| p|+que W||| + .e||ucou u||+ce ou ||e e+|
e\|eud|u ||ue+||, doWu |o ||e uec|.
Veiy common, occuis mostly in adults. Females
> males.
to|oy Unknown. It is consideied by many
to iepiesent a late histiocytic ieaction to an
aithiopod bite.
Sko Iesoos Usually asymptomatic papule oi
nodule (Fig. 9-49 ), 3 to 10 mm in diametei.
Suiface vaiiably domed but may be depiessed
below plane of suiiounding skin. Textuie of
suiface may be dull, shiny, oi scaling. Top may
be ciusted oi scaiied secondaiy to excoiiation
oi shaving. Boideis ill defined, fading to noimal
skin. Co|or : vaiiable-skin-coloied, pink, biown,
tan, daik chocolate biown (Fig. 9-49 B ). Usually
daikei at centei, fading to noimal skin coloi at
maigin. Fiim. Dm|e sgn : lateial compiession
with thumb and index fingei pioduces a
depiession oi dimple" (Fig. 9-49 C ).
DIstrIhutIvn Legs > aims > tiunk. Haidly
evei occuis on head, palms, soles. Usually soli-
taiy; may be multiple, iandomly scatteied.
8NICN 0kMAI AN0 S8CIAN0S N0FIASMS
AN0 hFkFIASIAS
||por+ +|e |u|e o| ru|||p|e, |eu|u u|cu|+ue
ou |uro| ||+| +|e e+||, |ecou|/ed |ec+ue
||e, +|e o||, |ouuded, o| |o|u|+|ed +ud ro.+||e
++|u| ||e o.e||,|u ||u (||. 9+3 1 3 ).
\+u, ||por+ +|e r+|| |u| r+, +|o eu|+|e |o >
o cr.
I|e, occu| epec|+||, ou ||e uec|, ||uu|, +ud ou
||e e\||er|||e (||. 9+3) |u| c+u occu| +u,
W|e|e ou ||e |od,.
||por+ +|e corpoed o| |+| ce|| ||+| |+.e ||e
+re ro|p|o|o, + uo|r+| |+| ce|| W||||u +
couuec||.e ||ue ||+reWo||. Au|o||por+ |+.e
+ .+cu|+| corpoueu| +ud r+, |e |eude| |u co|d
+r||eu| |erpe|+|u|e +ud W||| corp|e|ou.
Au|o||por+ o||eu |equ||e e\c||ou, W|e|e+
o||e| ||por+ |ou|d |e e\c|ed ou|, W|eu
cou|de|ed d|||u||u. ||pouc||ou c+u +|o |e
pe||o|red W|eu ||poor+ +|e o|| +ud ||u
|+.e ou|, + r|uo| couuec||.e ||ue corpoueu|.
|c|c| |c ,!- , +u +u|oor+| dor|
u+u| ||+|| +ppe+||u |u e+||, +du|||ood, cou|| o|
|uud|ed o| |oW|, |oW|u uou|eude| |e|ou.
1!|c !|c , o| |-: !-c- , occu|
|u Woreu |u r|dd|e +e, ||e|e +|e ru|||p|e
|eude|, uo| c||curc|||ed |u| |+||e| d|||ue |+||,
depo||.
3- ,-|: |c| , W||c| +||ec| r|d
d|e+ed reu, cou|| o| r+u, |+|e uou|eude|,
co+|eceu| poo||, c||curc|||ed ||por+, ro||,
ou ||e ||uu| +ud uppe| e\||er|||e, ||e, co+|ece
ou ||e uec| +ud r+, |e+d |o + '|o|eco||+| +p
pe+|+uce.
IIF0MA |C|9 . 2+
|C|0 . |1 \3350/0
A de|r+|o||||or+ | + .e|, corrou, |u||ou|||e
de|r+| uodu|e, uu+||, occu|||u ou ||e e\||er|
||e.
|rpo||+u| ou|, |ec+ue o| || core||c +ppe+|
+uce o| || |e|u r||+|eu |o| o||e| |e|ou,
uc| + r+||u+u| re|+uor+ W|eu || | p|
reu|ed.
k+|e|,, ||e |e|ou r+, |e |eude|.
',, . 'o|||+|, ||||oc,|or+, c|e|o|u |e
r+u|or+.
0kMAI0FI8k0MA |C|9 . 2o
|C|0 . |2! \33!2/0

0ermatopatho|oy Whoiling fascicles of spin-
dle cells with small amounts of pale blue cy-
toplasm and elongated nuclei. Some tumois
extend to the panniculus. Pigmented deimatofi-
biomas (Fig. 9-49 B ) contain lipids oi hemosi-
deiin pigment in the histiocytes in addition to
hypeipigmentation of the epideimis. Oveilying
epideimis fiequently hypeiplastic.
Clinical findings-dimple" sign (Fig. 9-49 C ),
but theie aie othei lesions that can iesult in
depiession with lateial piessuie, e.g., papu-
lonodulai lesions containing mucin, scai, blue
nevus, pilai cyst, metastatic caicinoma, Kaposi
saicoma, deimatofibiosaicoma piotubeians.
FICk 9-48 Ipoma . we||de||ued, o||, |ouuded |uro| |u ||e u|cu||, ro.+||e |o|| ++|u| ||e
o.e||,|u ||u +ud ||e uude||,|u ||uc|u|e, |u + 5o,e+|o|d r+|e p+||eu|. |u ||| p+||eu| |e|ou We|e ,rre|||c
+ud We|e +|o |ouud ou ||e ||uu| +ud uppe| e\||er|||e. 8. \u|||p|e ||por+ ou ||e |oWe| +|r o| + 50,e+|o|d
p+||eu|. I|ee |e|ou We|e +|o ,rre|||c.
8
C0kS AN0 Fk0CN0SIS
Lesions appeai giadually ovei seveial months,
may peisist without inciease in size foi yeais to
decades, and may iegiess spontaneously.
MANACMNI
Suigical iemoval is not usually indicated, as the
iesulting scai is often less cosmetically accept-
able. Ciyosuigeiy with a cotton-tip applicatoi
is often effective and pioduces a cosmetically
acceptable scai in most patients.
8 C
FICk 9-49 0ermatoIbroma . A dore|+ped, |||||, e|,||er+|ou +ud |+u uodu|e W||| + |u||ou
|||e, |||r cou||euc,. 8. I|| |e|ou | p|reu|ed. C+u |e cou|ued W||| ||ue ue.u o| e.eu uodu|+| re|+uor+.
I|e p|reu| | re|+u|u +ud |ero|de||u. C. '||rp|e |u. ||rp||u o| ||e |e|ou | eeu W|eu p|uc|ed |e|Weeu
|Wo ||ue|.
Ae oI 0oset Thiid decade, but all ages.
kace Much moie common in blacks and in
peisons with blood gioup A.
to|oy Unknown. They usually follow injuiy
to skin, i.e., suigical scai, laceiation, abiasion,
ciyosuigeiy, and electiocoagulation as well as
vaccination, acne, etc. Ke|oJ may a|so arse
sonaneous|y, w|ou |sory o[ n,ury, usua||y
n reserna| se.
Sko Symptoms Usually asymptomatic. May
be piuiitic oi painful if touched.
Sko Iesoos Papules to nodules (Fig. 9-50 , B )
to laige tubeious lesions. Most often the coloi
of the noimal skin but also biight ied oi bluish.
May be lineai aftei tiaumatic oi suigical injuiy
(Fig. 9-50 ). Hypeitiophic scais tend to be
elevated and aie confined to appioximately
the site of the oiiginal injuiy (Fig. 9-50).
Keloids, howevei, may extend in a clawlike
fashion fai beyond any slight oiiginal injuiy
(Figs. 9-51, 9-52 ) oi may be nodulai; tumoi-
like. Fiim to haid; may be tendei, suiface
smooth. Spontaneous keloids aiise de novo
without tiauma oi suigeiy, and usually occui
on the chest (Fig. 9-52 B ).
DIstrIhutIvn Eailobes, shouldeis, uppei back,
chest.
n,pe|||op||c c+| +ud |e|o|d +|e e\u|e|+u|
||||ou |ep+|| ||ue +||e| + cu|+ueou |uju|,.
A |,-||: :c |er+|u cou||ued |o ||e ||e
o| o|||u+| |uju|,.
A |-|! , |oWe.e|, e\|eud |e,oud ||| ||e, o||eu
W||| c|+W|||e e\|eu|ou.
\+, |e core||c+||, .e|, uu||||, +ud poe +
e||ou p|o||er |o| ||e p+||eu| || ||e |e|ou |
|+|e +ud ou ||e e+| o| |+ce o| o.e| + jo|u|.
hFkIk0FhIC SCAkS AN0 kI0I0S |C|9 . 10.+
|C|0 . |9.0
FICk 9-50 hypertrophc scar . A ||o+d, |+|ed c+| de.e|op|u +| ||e ||e o| u||c+| |uc||ou W||| |e|
+u|ec|+||c ||ood .ee| +ud + ||u, +||op||c ep|de|r|. 8. \u|||p|e |,pe|||op||c c+| ou ||e c|e| o| +
22,e+|o|d r+|e W||| + |||o|, o| e.e|e +cue cou|o|+|+.
8
0ermatopatho|oy HypertrvphIc Scur
Whoils of young fibious tissue and fibioblasts
in haphazaid aiiangement.
Ke|vId Featuies of hypeitiophic scai with
added featuie of thick, eosinophilic, acellulai
bands of collagen.
Clinical diagnosis; biopsy not waiianted un-
less theie is clinical doubt, because this may
induce new hypeitiophic scaiiing. Diffei-
ential diagnosis includes deimatofibioma,
deimatofibiosaicoma piotubeians, desmoid
tumoi, scai with saicoidosis, foieign-body
gianuloma.
C0kS AN0 Fk0CN0SIS
Hypeitiophic scais tend to iegiess, in time be-
coming flattei and softei. Keloids, howevei, may
continue to expand in size foi decades.
MANACMNI
This is a ieal challenge, as no tieatment is highly
effective.
Iotra|esooa| C|ucocortcods Intialesional in-
jection of tiiamcinolone (10-40 mg/mL) eveiy
month may ieduce piuiitus oi sensitivity of
lesion, as well as ieduce its volume and flatten
it. This woiks quite well in small hypeitiophic
scais but less well in keloids. Can be combined
with ciyotheiapy wheieby the lesion is initially
fiozen with liquid nitiogen, allowed to thaw,
and then injected with tiiamcinolone (10-40
mg/mL). Aftei fieezing, the lesion becomes
edematous and is much easiei to inject.
Surca| xcsoo Lesions that aie excised sui-
gically often iecui laigei than the oiiginal
lesion. Excision with immediate postsuigical
iadiotheiapy is beneficial.
S|cooe Cream aod S|cooe Ce| Sheet
Repoited to be beneficial in keloids and is
painless and noninvasive. Not veiy effective in
authois` expeiience.
Freveotoo Individuals pione to hypeitiophic
scais oi keloids should be advised to avoid
cosmetic pioceduies such as eai pieicing. Scais
fiom buins tend to become hypeitiophic. Can
be pievented by compiession gaiments.
FICk 9-51 ke|ods we||de||ued |||eu|+| uodu|e, .e|, |+|d ou p+|p+||ou, |u ||e +u||cu|+| |e|ou +ud
c|ee| o| + !0,e+|o|d r+u. I|e |e|ou ou ||e e+||o|e +|oe +||e| p|e|c|u, ||e |e|ou ou ||e r+ud||u|+| |e|ou
+||e| |uc||ou o| +u |u||+red c,|.
FICk 9-52 ke|ods . Ke|o|d +||e| + deep |u|u. |o|e +u+e +ud c|+W|||e e\|eu|ou o| ||e |e|o|d |u|o
uo|r+| ||u. 8. 'pou|+ueou |e|o|d ||+| +|oe W|||ou| +pp+|eu| c+ue ou ||e c|e| o| + 9,e+|o|d r+u.
8
A |+|e |o|r o| upe|||c|+| ju.eu||e ||||or+|o|.
||eeu||u + +,rp|or+||c ||e|co|o|ed o| p|u|
|||r uodu|e ou ||ue| +ud |oe (||. 95!).
Appe+| |u ||e |||| ,e+| o| |||e, |e corrou|, |u
c|||d|ood.
n||o|o|c+||, |u|e||+c|u |uud|e o| r,o||||o|
|+| W||| eo|uop||||c |uc|u|ou.
Beu|u. 'pou|+ueou |e|e|ou | |+|e. I|e+|reu|
| u||c+|
',,. k,e |uro|.
INFANIII 0ICIIAI FI8k0MAI0SIS |C|9 . 151.!
|C|0 . \12
FICk 9-53 IoIaot|e dta| Ibromatoss
A We||de||ued p|u| uodu|e ou ||e ||ue| o| +u
|u|+u|. uu+||, ||e ||||d |o ||||| d||| +|e +||ec|ed.
ne|e, ||e |uro| | |ouud ou ||e ecoud d|||.
FICk 9-54 Sko tas 'o|| ||uco|o|ed +ud |+u peduucu|+|ed p+p|||or+. I|ee +|e .e|, corrou |u ||e
e|de||, o|ee +ud +|e o|||+|o|, |e|ou |u +c+u||o| u|||c+u, + |u ||| p+||eu|.
A ||u |+ | + .e|, corrou, o||, ||uco|o|ed o|
|+u o| ||oWu, |ouud o| o.+|, peduucu|+|ed p+p||
|or+ (po|,p) (||. 95+), || | uu+||, cou|||c|ed
+| ||e |+e +ud r+, .+|, |u |/e ||or > rr |o
+ |+|e + 0 rr.
n||o|o|c ||ud|u |uc|ude + |||uued ep|de|r|
+ud + |ooe ||||ou ||ue ||or+.
uu+||, +,rp|or+||c |u| occ+|ou+||, r+, |e
core |eude| |o||oW|u ||+ur+ o| |o||ou +ud r+,
|ecore c|u|ed o| |ero|||+|c.
|| occu| ro|e o||eu |u ||e r|dd|e +ed +ud |u ||e
e|de||,.
\o|e corrou |u |er+|e +ud |u o|ee p+||eu|
+ud ro| o||eu uo|ed |u |u|e|||||uou +|e+ (+\||
|+e, |u||+r+rr+|,, |o|u) +ud ou ||e uec| +ud
e,e||d.
|| occu| |u +c+u||o| u|||c+u +ud re|+|o||c
,ud|ore.
\+, |e cou|ued W||| + peduucu|+|ed e|o|
||e|c |e|+|o|, de|r+| o| corpouud re|+uoc,||c
ue.u, o|||+|, ueu|o||||or+, o| ro||ucur cou
|+|our.
|e|ou |eud |o |ecore |+|e| +ud ro|e uure|
ou o.e| ||re, epec|+||, du||u p|eu+uc,. |o|
|oW|u pou|+ueou |o||ou, +u|o+rpu|+||ou c+u
occu|.
\+u+ereu| | +ccorp|||ed W||| |rp|e u|p
p|u W||| c|o|, e|ec||ode|cc+||ou, o| c|,ou|
e|,.
',, Ac|oc|o|dou, cu|+ueou p+p|||or+,
o|| ||||or+.
SkIN IAC |C|9 . 10.9
|C|0 . |9.3
232
S E C I | 0 N 1 0
I|e |e|r ||-|.|, dec|||e +u +|
uo|r+| |epoue |o ||||, uu+||, uu||||,
occu|||u W||||u r|uu|e, |ou|, o| d+, o|
e\pou|e +ud |+||u up |o Wee|, rou||,
+ud e.eu |oue|. Cu|+ueou p|o|oeu|||.||,
|e+c||ou |equ||e +|o|p||ou o| p|o|ou eue|,
|, +pp|op||+|e|, |+ped ro|ecu|e |e+d|u |o
ro|ecu|+| de|o|r||,. Eue|, | e|||e| d|pe|ed
|+|r|e|, o| | d||ec|ed |o c|er|c+| |e+c||ou
||+| |e+d |o ro|ecu|+|, ce||u|+|, +ud ||ue
d+r+e |eu|||u |u c||u|c+| d|e+e. A|o|||u
ro|ecu|e c+u |e () e\oeuou +eu| +p
p||ed |op|c+||, o| ,|er|c+||,, (2) eudoeuou
ro|ecu|e e|||e| uu+||, p|eeu| |u ||u o|
p|oduced |, +u +|uo|r+| re|+|o||r, o| (!)
+ cor||u+||ou o| e\oeuou +ud eudoeuou
ro|ecu|e ||+| |+.e +cqu||ed +u||eu|c p|op
e|||e +ud ||u e||c|| + p|o|o|+d|+||oud||.eu
|rruue |e+c||ou. |||-|.|, !!-
:: |, |!, - --! | |c
c!c| (|r+e 0).
I|e|e +|e |||ee ||o+d |,pe o| c:|- |
|-|.|,.
. A ||,pe |epoue W||| ||e de.e|opreu|
o| ro|p|o|o|c ||u c|+ue |ru|+||u + uo|r+|
uu|u|u W||| e|,||er+, eder+, +ud |u||+e, uc|
+ |u p|o|o|o\|c |e+c||ou |o d|u o| p|,|op|o
|ode|r+||||.
2. A c| |epoue |o |||| e\pou|e W||| de.e|op
reu| o| .+||ed ro|p|o|o|c e\p|e|ou. r+cu|e,
p+pu|e, o| p|+que, + |u ec/er+|ou de|r+||||.
I|ee +|e uu+||, p|o|o+||e||c |u u+|u|e o| |e
|ou |o ||e oc+||ed |d|op+|||c p|o|ode|r+|oe
uc| + po|,ro|p|ou |||| e|up||ou.
!. ||:cc| |epoue +|e |,p|c+| |o| o|+| u|||c+||+,
u|||c+||+| |e|ou c+u +|o occu| |u e|,|||opo|e||c
po|p|,||+.
C|: ||-|.|,. c||ou|c |epe+|ed
uu e\pou|e o.e| ||re |eu|| |u po|,ro|p||c
||u c|+ue ||+| |+.e |eeu |e|red !-c|
|-|, o| p|o|o+|u. A c|+|||c+||ou o| ||u
|e+c||ou |o uu|||| | |oWu |u I+||e 0.
SkIN kACII0NS I0 SNIIChI |C|9 . o92.10
|C|0 . |5o.3
8ASICS 0F CIINICAI Fh0I0M0ICIN
The main culpiit of solai iadiation-induced
skin pathology is the ultiaviolet poition of the
solai spectium. Ultiaviolet iadiation (UVR) in
photomedicine is divided into two piincipal
types: UVB (290-320 nm), the sunbuin spec-
tium," and UVA (320-400 nm). UVA is subdi-
vided into UVA-1 (340-400 nm) and UVA-2
(320-340 nm). The unit of measuiement of
sunbuin is the mnmum ery|ema Jose (MED),
which is the minimum ultiaviolet exposuie
that pioduces a cleaily maiginated eiythema in
the iiiadiated site 24 h aftei a single exposuie.
The MED is expiessed as the amount of eneigy
deliveied pei unit aiea: mJ/cm
2
(UVB) oi J/cm
2
(UVA). The MED foi UVB in Caucasians is
Fh0I0SNSIIIvII,
Fh0I0-IN0C0 0IS0k0kS,
AN0 0IS0k0kS 8 I0NIIINC
kA0IAII0N
20-40 mJ/cm
2
(foi a skin phototype I oi II,
about 20 min in noithein latitudes at noon in
June) and foi UVA is 15-20 J/cm
2
(about 120
min in noithein latitudes at noon in June).
UVB eiythema develops in 6-24 h and fades
within 72-120 h. UVA eiythema develops in
4-16 h and fades within 48-120 h.
varatoos o Suo keactvty o Norma| Fersoos:
Fttpatrck Sko Fhototypes (Table 10-2)
Sunbuin is seen most fiequently in individuals
who have pale white oi white skin and a limited
capacity to develop [atu|ae, oi inducible,
melanin pigmentation (tanning) aftei exposuie
to UVR. Basic skin coloi ( tonsue melanin
pigmentation) is divided into white, biown,
and black. Not all peisons with white skin have
the same capacity to develop tanning, and this
SCII0N 10 |n0I0'E|'|I|\|I\, |n0I0|||uCE| ||'0k|Ek', A|| ||'0k|Ek' B\ |0||/||C kA||AI|0| 233
IMAC 10-1 \+||+||ou |u o|+| e\pou|e ou d|||e|eu| |od, +|e+.
fact is the piincipal basis foi the classification of
white" peisons into foui s|n |ooyes (SPT).
The SPT is based on the basic skin coloi (Table
10-2) and on a erson's own esmae of sun-
buining and tanning. One question peimits the
identification of the SPT: Do you tan easily:"
Peisons with SPT I oi II will say immediately,
No," and those with SPT III oi IV will say,
Yes." Peisons with SPT I oi II aie iegaided as
melanocompiomised," and those with SPT III
oi IV as melanocompetent."
SPT I peisons usually have pale white skin
coloi, blond oi ied haii, and blue eyes; but, in
fact, they may have daik biown haii and biown
eyes, while theii skin coloi is pale white. SPT
I peisons sunbuin easily with shoit exposuies
and do not tan.
SPT II peisons aie a subgioup of SPT I and
sunbuin easily but an w| J[[tu|y, wheieas
SPT III peisons may have some sunbuin with
shoit exposuies but can develop maiked tan-
ning. It is estimated that about 25% of white-
skinned peisons in the United States aie SPT I
and II. SPT IV peisons tan with ease and do not
sunbuin with shoit exposuies. SPT IV peisons
may have blond haii and blue eyes but moie
often have biown haii and biown eyes and light
tan (beige) constitutive skin coloi. Peisons with
constitutive biown skin aie teimed SPT V and
with black skin SPT VI. Note that sunbuin de-
pends on the amount of UVR eneigy absoibed.
Thus, with excessive sun exposuie, even SPT VI
peison can have a sunbuin.
||o|o|o\|c||,
'uu|u|u
||u/c|er|c+||uduced
||+u||uduced (p|,|op|o|ode|r+||||)
||o|o+||e|,
||u/c|er|c+||uduced
C||ou|c +c||u|c de|r+||||
'o|+| u|||c+||+
|d|op+|||c
|o|,ro|p|ou |||| e|up||ou
Ac||u|c p|u||o
c
n,d|o+ .+cc|u||o|re
c
\e|+|o||c +ud uu|||||ou+|
|o|p|,||+ cu|+ue+ |+|d+
\+||e+|e po|p|,||+
E|,|||opo|e||c p|o|opo|p|,||+
|e||+|+
c
||Ade||c|eu| p|o|ode|r+|oe
/e|ode|r+ p|reu|our
c
0||e| |+|e ,ud|ore
c
||o|oe\+ce||+|ed de|r+|oe
C||ou|c p|o|od+r+e
|e|r+|o|e||o| (p|o|o+|u)
'o|+| |eu||o
Ac||u|c |e|+|oe
'||u c+uce|
|
c
Coud|||ou uo| de+|| W||| |e|e +ud ||e |e+de| | |e|e||ed |o K wo|||
e| +| (ed). ||cc|:| |-c||, C--c| !-!:- 1|| ed.
|eW \o||, \cC|+Wn|||, 2003.
|
|o| co.e|+e o| ||u c+uce|, ee 'ec||ou +ud 2.
IA8I 10-1 Simp|ified C|assification of
Skin Reactions to Sun|iht
IA8I 10-2 C|assification of |ittpatrick's Skin Phototypes (SPI)
SPT 8as|c Sk|o 0o|or 8espoose to S0o xpos0re
| |+|e W|||e |o uo| |+u, |u|u e+||,
|| w|||e I+u W||| d||||cu||,, |u|u e+||,
||| w|||e I+u e+||, |u| r+, |u|u |u|||+||,
|\ |||| ||oWu/o||.e I+u e+||,, |+|d|, |u|u
\ B|oWu I+u e+||,, uu+||, do uo| |u|u
\| B|+c| Becore d+||e|, do uo| |u|u
'uu|u|u | +u +cu|e, de|+,ed, +ud ||+u|eu| |u||+r
r+|o|, |epoue o| uo|r+| ||u +||e| e\pou|e |o
u\k ||or uu|||| o| +|||||c|+| ou|ce.
B, u+|u|e || | + p|o|o|o\|c |e+c||ou.
'uu|u|u | c|+|+c|e||/ed |, e|,||er+ (||. 0 1 )
+ud, || e.e|e, |, .e|c|e +ud |u||+e, eder+,
|eude|ue, +ud p+|u (||. 0 3 ).
ACI SN 0AMAC (SN8kN) |C|9 . o92.1
|C|0 . |55
FI0MI0I0C
Sunbuin depends on the amount of UVR
eneigy deliveied and the susceptibility of the
individual (SPT). It will theiefoie occui moie
often aiound midday, with decieasing latitude,
incieasing altitude, and decieasing SPT. Thus,
the ideal" setting foi a sunbuin to occui would
be an SPT I individual (highest susceptibil-
ity) on Mt. Kenya (high altitude, close to the
equatoi) at noon (UVR is highest). Of couise,
sunbuin can occui at any latitude, but the
piobability foi it to occui decieases with in-
cieasing distance fiom the equatoi. Sunbuin is
seen moie often in those who fiequent beaches
oi tiavel to sunny vacation aieas. Sunbuin also
incieases with iespect to othei ambient con-
ditions, such as UVR ieflectance fiom snow,
watei, oi a glaciei.
Ae Veiy young childien and eldeily peisons
aie said to have a ieduced capacity to sunbuin,
although this has not been thoioughly docu-
mented.
FAIh0CNSIS
The chiomophoies (molecules that absoib
UVR) foi UVB sunbuin eiythema aie not
known, but damage to DNA may be the in-
itiating event. The damage to DNA iesults
in excision of pyiimidine dimeis, and that
itself initiates a piotective tanning iesponse.
The mediatois that cause the eiythema in-
clude histamine foi both UVA and UVB.
In UVB eiythema, othei mediatois include
tumoi neciosis factoi (TNF- ), seiotonin,
piostaglandins, nitiic oxide, lysosomal en-
zymes, and kinins. The cytokine TNF- can
be detected as eaily as 1 h aftei exposuie.
The iesolution of eiythema is associated with
inteileukin (IL) 10, IL-4, and tiansfoiming
giowth factoi
1
.
Exposuie to the sun oi an aitificial UV souice.
Onset of symptoms depends on intensity of
exposuie; eiythema develops aftei 6 h and
peaks aftei 24 h.
Sko Symptoms Piuiitus may be seveie even in
mild sunbuin; pain and tendeiness occui with
seveie sunbuin.
Coosttutooa| Symptoms Headache, chills, fe-
veiishness, and weakness aie not infiequent
in seveie sunbuin; some SPT I and II peisons
develop headache and malaise even aftei shoit
exposuies.
Ceoera| Appearaoce In seveie sunbuin, the
patient is toxic"-with fevei, weakness, lassi-
tude, and a iapid pulse iate.
Sko Iesoos Confluent biight eiythema
always confined to sun-exposed aieas and thus
shaiply maiginated at the boidei between
exposed and coveied skin (Fig. 10-1). Edema,
vesicles, and even bullae; always unifoim
eiythema and no iash," as occuis in most
photoalleigic ieactions. Edematous aieas aie
iaised and tendei. As edema and eiythema fade
vesicles and blisteis diy to ciusts, which aie
then shed (Fig. 10-1 B ).
DIstrIhutIvn Stiictly confined to aieas of
exposuie; sunbuin can occui in aieas coveied
with clothing, depending on the degiee of UV
tiansmission thiough clothing, the level of
exposuie, and the SPT of the peison.
Mucous Membraoes Sunbuin of the tongue
can occui iaiely in mountain climbeis who
hold theii mouth open panting"; it is fiequent
on the veimilion boidei of the lips.
0ermatopatho|oy Sunbuin" cells in the
epideimis (apoptotic keiatinocytes); also,
exocytosis of lymphocytes, vacuolization of
melanocytes and Langeihans cells. Derms : en-
dothelial cell swelling of supeificial blood ves-
sels. Moie piominent with UVA eiythema,
with a densei mononucleai infiltiate and moie
seveie vasculai changes.
Sero|oy aod hemato|oy To iule out systemic
lupus eiythematosus (SLE) obtain antinucleai
antibody (ANA) level. Leukopenia may be pie-
sent in SLE.
Histoiy of UVR exposuie and sites of ieaction
on exposed aieas. P|oooxt ery|ema : obtain
histoiy of medications that can induce photo-
toxic eiythema. SLE can cause a sunbuin-type
eiythema. Ery|rooet rooor|yra causes
eiythema, vesicles, edema, puipuia, and, only
iaiely, uiticaiial wheals.
C0kS AN0 Fk0CN0SIS
Sunbuin, unlike theimal buins, cannot be classi-
fied on the basis of depth, i.e., fiist-, second-,
and thiid-degiee. Thiid-degiee buins aftei UVR
do not occui, and none of the featuies of thiid-
degiee theimal buins aie seen: scaiiing, loss of
sensation, loss of sweating, haii loss. A peima-
nent ieaction fiom seveie ultiaviolet buins is
mottled depigmentation, piobably ielated to
the destiuction of melanocytes, and eiuptive
solai lentigines (see Fig. 10-22).
MANACMNI
Freveotoo Peisons with SPT I oi II should
avoid sunbathing, especially between 11 A.M.
and 2 P.M . Clothing: UV-scieening cloth gai-
ments. Theie aie now many highly effective
topical chemical filteis (sunscieens) in lotion,
gel, and cieam foimulations. It is still not cleai
whethei iegulai use of topical sunscieens can
pievent melanoma of the skin, but theie is
ieasonable pioof that topical sunscieens ieduce
the induction of solai keiatoses and, piobably,
squamous cell caicinoma.
Moderate Suoburo TvpIcu| Cool wet diess-
ings, topical glucocoiticoids.
SystemIc Acetylsalicylic acid, indomethacin,
NSAIDs.
Severe Suoburo Bed iest. If veiy seveie, a
toxic" patient may iequiie hospitalization foi
fluid ieplacement, piophylaxis of infection, etc.
TvpIcu| Cool wet diessings, topical glucocoi-
ticoids.
SystemIc Oial glucocoiticoids aie often given,
but theii efficacy has not been established by
contiolled studies. Indomethacin.
I|| dec|||e ||e |u|e|+c||ou o| u\k W||| + c|er|c+|/
d|u W||||u ||e ||u.
IWo rec|+u|r +|e |ecou|/ed. |||: -
c:| W||c| +|e p|o|oc|er|c+| |e+c||ou |e+d
|u |o ||u p+||o|o,, +ud
||c||-: -c:| W|e|e + p|o|o+||e|eu |
|o|red ||+| |u|||+|e +u |rruuo|o|c |epoue
+ud r+u||e| |u ||u + + |,pe |\ |rruuo|o|c
|e+c||ou.
I|e r+|u c||u|c+| d|||e|euce |e|Weeu p|o|o|o\|c
+ud p|o|o+||e||c e|up||ou | ||+| ||e |o|re|
r+u||e| |||e +u |||||+u| (|o\|c) cou|+c| de|r+||||
o| uu|u|u +ud ||e |+||e| |||e +u +||e||c ec/er+
|ou cou|+c| de|r+|||| (I+||e 0!).
0kC-[ChMICAI-IN0C0 Fh0I0SNSIIIvII |C|9 . o92.19
|C|0 . |5o.0
SCII0N 10 |n0I0'E|'|I|\|I\, |n0I0|||uCE| ||'0k|Ek', A|| ||'0k|Ek' B\ |0||/||C kA||AI|0| 23T
FICk 10-1 Acute suoburo . |+|u|u|, |eude|, ||||| e|,||er+ W||| r||d eder+ o| ||e uppe| |+c| W|||
|+|p der+|c+||ou |e|Weeu ||e uue\poed +ud uup|o|ec|ed W|||e +|e+. 8. +3 |ou| +||e| +cu|e uu|u|u.
E|,||er+ | |+d|u +ud ||||e| |+.e d||ed |o c|u|.
8
IA8I 10-3 Characteristics of Phototo\icity and Photoa||ery
Phototox|c|ty Photoa||ergy
C||u|c+| p|eeu|+||ou 'uu|u|u |e+c||ou. e|,||er+, Ec/er+|ou |e|ou,
eder+, .e|c|e +ud |u||+e, p+pu|e, .e|c|e,
||equeu||, |eo|.e W||| c+||u, c|u||u,
|,pe|p|reu|+||ou, |u|u|u, uu+||, p|u||||c
r+|||u
n||o|o, Apop|o||c |e|+||uoc,|e, 'pou|o||c de|r+||||,
p+|e de|r+| |u|||||+|e o| deue, de|r+|
|,rp|oc,|e, r+c|op|+e, |,rp|o||||oc,||c
+ud ueu||op||| |u|||||+|e
|+||op|,|o|o, |||ec| ||ue |uju|, I,pe |\ de|+,ed
|,pe|eu|||.||,
|epoue
0ccu||euce +||e| \e |o
|||| e\pou|e
0ue| o| \|uu|e |o |ou| 2+-+3 |
e|up||ou +||e|
e\pou|e
|o+e o| +eu| |+|e 'r+||
ueeded |o| e|up||ou
C|o|e+c||.||, k+|e Corrou
W||| o||e| +eu|
||+uo| C||u|c+| + p|o|o|e| C||u|c+| + p|o|o|e|
+ p|o|op+|c| |e|
Ad+p|ed ||or n ||r, |u K wo||| e| +| (ed). ||cc|:|' |-c||, C--c| !-!:- 1|| ed. |eW \o||, \cC|+Wn|||, 2003.
I|| dec|||e +u +d.e|e |e+c||ou o| ||e ||u ||+|
|eu|| ||or |ru||+ueou e\pou|e |o ce||+|u
d|u (.|+ |ue||ou, |ujec||ou, o| |op|c+| +pp||c+
||ou) +ud |o u\k o| .||||e ||||.
I|e c|er|c+| r+, |e ||e|+peu||c, core||c,
|udu|||+|, o| +||cu||u|+|.
I|e|e +|e |Wo |,pe o| |e+c||ou. () ,|er|c
p|o|o|o\|c de|r+||||, occu|||u |u |ud|.|du+|
,|er|c+||, e\poed |o + p|o|oeu|||/|u +eu|
(d|u) +ud u|equeu| u\k, +ud (2) |oc+| p|o|o
|o\|c de|r+||||, occu|||u |u |ud|.|du+| |op|c+||,
e\poed |o ||e p|o|oeu|||/|u +eu| +ud u|e
queu| u\k.
Bo|| +|e -c-c|-! | -- (e|,
||er+, eder+, .e|c|e, +ud/o| |u||+e).
',|er|c p|o|o|o\|c de|r+|||| occu| |u +|| |l|
--! |- |oc+| p|o|o|o\|c de|r+|||| ou|, |u
||e |:c| c|:c| |-
Fh0I0I0XIC 0kC-[ChMICAI-IN0C0 Fh0I0SNSIIIvII |C|9 . o92.19
|C|0 . |5o.0
238 FAkI I ||'0k|Ek' |kE'E|I||C || InE 'K|| A|| \uC0u' \E\BkA|E'
IA8I 10-4 Systemic Phototo\ic Aents
c
Property 6eoer|c hame Property 6eoer|c hame
Au||+u\|e|, d|u A|p|+/o|+r ||u|e||c n,d|oc||o|o|||+/|de
C||o|d|+/epo\|de 0yatde
Au||c+uce| d|u Ad||+r,c|u |,e ||uo|ece|u
|+c+||+/|ue \e||,|eue ||ue
||uo|ou|+c|| |u|ocour+||u |o|+|eu
\e||o||e\+|e 5-Methoxypsora|eo
\|u||+||ue 8-Methoxypsora|eo
Au||dep|e+u| I||c,c||c 4, 5', 8-Irmethy|psora|eo
Ar||||p|,||ue n,po|,cer|c 'u||ou,|u|e+.
|e|p|+r|ue Ace|o|e\+r|de
|r|p|+r|ue C||o|p|op+r|de
Au|||uu+| C||eo|u|.|u C||p|/|de
Au||r+|+||+| C||o|oqu|ue C|,|u||de
0u|u|ue Io|+/+r|de
Au||r|c|o||+| 0u|uo|oue Io|butamde
C|p|o||o\+c|u |'A|| Ace||c +c|d de||.+||.e
Euo\+c|u ||c|o|eu+c
Cer|||o\+c|u Au|||+u|||c +c|d de||.+||.e
IomeI|oxaco \e|eu+r|c +c|d
\o\|||o\+c|u Euo||c +c|d de||.+||.e.
Na|dxc acd Froxcam
|o|||o\+c|u ||op|ou|c +c|d de||.+||.e
0||o\+c|u ||up|o|eu
SparI|oxaco Ke|op|o|eu
'u||ou+r|de Naproxeo
Ie||+c,c||ue 0\+p|o/|u
0emec|ocyc|oe I|+p|o|eu|c +c|d
0oxycyc|oe '+||c,||c +c|d de||.+||.e
\|uoc,c||ue ||||uu|+|
Ie||+c,c||ue 0||e|
I||re||op||r Ce|eco\||
\o||cou+/o|e Nabumetooe
Au||p,c|o||c ||euo|||+/|ue ||o|od,u+r|c ForImer
d|u Ch|orpromatoe ||e|+p, +eu| verteporIo
|e|p|eu+/|ue ke||uo|d Ac|||e||u
Froch|orperatoe |o||e||uo|u
I||o||d+/|ue 0||e| ||u|+r|de
I||||uope|+/|ue n,pe||c|u
C+|d|+c Amodarooe |,||do\|ue (.||+r|u B
o
)
red|c+||ou 0u|u|d|ue k+u|||d|ue
||u|e||c Furosemde
I||+/|de
Beud|o||ure|||+/|de
Ch|orothatde
c
Corrou|, |epo||ed d|u +|e p||u|ed |u |o|d.
'ou|ce. Ad+p|ed ||or n ||r, |u K wo||| e| +| (ed). ||cc|:|' |-c||, C--c| !-!:- 1|| ed. |eW \o||, \cC|+Wn|||, 2003.
FI0MI0I0C
Occuis in eveiyone aftei ingestion of a suf-
ficient dose of a photosensitizing diug and
subsequent UVR. Theiefoie all ages, both sexes,
all iaces, and all types of skin coloi. Phototoxic
diug ieactions aie moie fiequent than photoal-
leigic diug sensitivity.
Foimation of toxic photopioducts such as fiee
iadicals oi ieactive oxygen species such as sing-
let oxygen. The piincipal sites of damage aie
nucleai DNA oi cell membianes (plasma, lyso-
somal, mitochondiial). The action spectium
is UVA. Diugs eliciting systemic phototoxic
deimatitis aie listed in Table 10-4. Some diugs
causing phototoxic ieactions can also elicit pho-
toalleigic ieactions (see below).
An exaggeiated sunbuin" aftei solai oi UVR
exposuie that norma||y wou|J no e|t a sun-
|urn n |a artu|ar nJJua|. Occuis usu-
ally within houis aftei exposuie, with some
agents such as psoialens aftei 24 h, and peaking
at 48 h. Skin symptoms: buining, stinging,
piuiitus.
Sko Iesoos Ear|y. The skin lesions aie those
of an exaggeiated sunbuin." Eiythema, edema
(Fig. 10-2 ), and vesicle and bulla foimation
(Fig. 10-2 B ) confined exclusively to aieas
exposed to light. An eczematous ieaction is no
seen in phototoxic ieactions.
Speca| Freseotatoos: Fseudoporphyra With
some diugs theie is little eiythema but pio-
nounced blisteiing and skin fiagility with
eiosions (see Fig. 22-13) and, upon iepeated
exposuies, healing milia foimation, paiticulaily
on the doisa of hands and lowei aims. Clini-
cally indistinguishable fiom poiphyiia cutanea
taida (see Fig. 10-11)-hence the teim seu-
Joor|yra (see Section 22).
Na|s Subungual hemoiihage and photo-
onycholysis can occui with ceitain diugs
(psoialens, demethylchloitetiacycline, benoxa-
piofen).
Fmeotatoo Maiked biown epideimal mela-
nin pigmentation may occui in the couise of
some eiuptions. With ceitain diugs especially
(chloipiomazine and amiodaione), a slate giay
deimal melanin pigmentation develops (see
Section 22).
0ermatopatho|oy Inflammation, sunbuin
cells" (apoptotic keiatinocytes) in the epi-
deimis, epideimal neciobiosis, intiaepideimal
and subepideimal vesiculation. Absence of ec-
zematous changes.
Fhototesto Foi veiification of the inciimi-
nating agent, template test sites aie exposed to
incieasing doses of UVA ( |oooxt reatons
are a|mos a|ways Jue o UV ) while patient is
on the diug. The UVA MED will be much lowei
than that foi noimal individuals of the same
skin phototype. Aftei diug is excieted and then
eliminated fiom the skin, a iepeat UVA pho-
totest will ieveal an ntrease in the UVA MED.
This test may be impoitant if patient is on
multiple potentially phototoxic diugs.
Histoiy of exposuie to diugs is most impoi-
tant, as aie the types of moiphologic changes
in the skin chaiacteiistic of phototoxic diug
eiuptions: confluent eiythema, edema, vesicles,
bullae. Diffeiential diagnosis includes iegulai
sunbuin, phototoxic ieactions due to excess of
endogenous poiphyiins, and photosensitivity
due to othei diseases, e.g., SLE.
C0kS AN0 Fk0CN0SIS
Phototoxic diug sensitivity is a majoi pioblem,
since the abnoimal ieactions seiiously limit oi
exclude the use of impoitant diugs: diuietics, an-
tihypeitensive agents, diugs used in psychiatiy.
Wheieas, as a iule, phototoxicity occuis in piac-
tically anyone who is on a phototoxic diug-in
contiast to photoalleigy, which occuis only in
the sensitized-some individuals nonetheless
show phototoxic ieactions to a paiticulai diug
and otheis do not. It is not known why. Pho-
totoxic diug ieactions disappeai aftei cessation
of diug.
MANACMNI
As foi sunbuin.
SSIMIC Fh0I0I0XIC 0kMAIIIIS |C|9 . o92.19
|C|0 . o5o.0
FICk 10-2 Fhototoxc dru-oduced
photoseostvty . \+|.e eder+ +ud
e|,||er+ |u ||e |+ce o| + 1,e+|o|d |||
W|o W+ ||e+|ed W||| dere||,|c||o||e||+
c,c||ue |o| +cue. |o|e +|euce o| e|,||er+
||or uec|, W||c| W+ |+ded 8. |u|,
e|,||er+ W||| ||||e||u ou ||e do|+ o| |o||
|+ud |u + p+||eu| ||e+|ed W||| p||o\|c+r.
ne|e ||e|e | |u+d.e||eu| cou|+c| W||| o| ||e|+
peu||c +pp||c+||ou o| + p|o|oeu|||/e|, |o||oWed |,
u\A |||+d|+||ou (p|+c||c+||, +|| |op|c+| p|o|oeu|
||/e| |+.e +u +c||ou pec||ur |u ||e u\A |+ue).
I|e ro| corrou |op|c+| p|o|o|o\|c +eu| +|e
|||ed |u I+||e 05, +ud ||e ro| corrou |ou|e
o| cou|+c| | e|||e| ||e|+peu||c o| occup+||ou+|
e\pou|e.
C||u|c+| p|eeu|+||ou | |||e +cu|e |||||+u| cou
|+c| de|r+|||| (ee 'ec||ou 2), W||| e|,||er+,
We|||u, .e|cu|+||ou, +ud ||||e||u cou||ued |o
||e ||e o| cou|+c| W||| ||e p|o|o|o\|c +eu|.
',rp|or +|e r+|||u, ||u|u, +ud |u|u|u
|+||e| ||+u ||c||u.
ne+||u uu+||, |eu|| |u p|ououuced p|reu|+
||ou. I|e ro| corrou +ud ||u |rpo||+u| |op|
c+| p|o|o|o\|c de|r+|||| | p|,|op|o|ode|r+||||,
dec|||ed |e|oW.
I0FICAI Fh0I0I0XIC 0MAIIIIS |C|9 . o92.19
|C|0 . |5o.0
8
||,|op|o|ode|r+|||| (p|+u| + |||| de|r+||||)
| +u |u||+rr+||ou o| ||e ||u c+ued |, cou|+c|
W||| ce||+|u p|+u| du||u |ec|e+||ou+| o| occu
p+||ou+| e\pou|e |o uu||||.
I|e |u||+rr+|o|, |epoue | + p|o|o|o\|c |e
+c||ou |o p|o|oeu|||/|u c|er|c+| |u e.e|+|
p|+u| |+r|||e.
Corrou |,pe o| ||| +|e due |o e\pou|e |o
||re, ce|e|,, +ud re+doW |+.
',, Be||oque de|r+||||, ||re de|r+||||.
FhI0Fh0I00kMAIIIIS (FF0) |C|9 . o92.12
|C|0 . |5o.2
Common. Usually in spiing and summei oi
all yeai in tiopical climates. PPD can occui at
any age.
kace All skin colois; biown- and black-
skinned peisons may develop only maiked
spotty daik pigmentation without eiythema oi
bullous lesions.
0ccupatoo Celeiy pickeis, caiiot piocessois,
gaideneis exposed to caiiot gieens oi to gas
plant" ( Dtamnus a||us )], and baitendeis (lime
juice) who aie exposed to sun in outside bais.
Nonoccupational: housewives and useis of pei-
fumes containing oil of beigamot; peisons in
holiday diinking time diinks oi eating oianges
in the sun.
to|oy Phototoxic ieaction caused by pho-
toactive fuiocoumaiins (psoialens) contained
in the plants (Table 10-5).
The patient gives a histoiy of exposuie to ceitain
plants (lime, lemon, wild paisley, celeiy, giant
hogweed, paisnips, caiiot gieens, figs). Lime
juice is a fiequent cause: making lime diinks,
haii iinses with lime juice. Women who use
peifumes containing oil of beigamot (which
contains beigapten, 5-methoxypsoialen) may
develop stieaks of pigmentation only in aieas
wheie the peifume was applied, especially the
sides of the neck. This is called |er|oque Jerma-
s (Fiench: |er|oque, pendant"). Peisons walk-
ing on beaches containing meadow giass and
childien playing in giassy meadows develop PPD
on the legs; meadow giass contains agiimony.
Sko Symptoms Smaiting, sensation of sun-
buin, pain, latei piuiitus.
Sko Iesoos Acute: eiythema, edema, vesicles,
and bullae (Fig. 10-3). Lesions may appeai
pseudopapulai befoie vesicles aie evident. Often
bizaiie stieaks, aitificial patteins that indicate
an outside job" (Fig. 10-4). Scatteied aieas on
the sites of contact, especially the aims, legs,
and face. Residual hypeipigmentation in bizaiie
stieaks (beiloque deimatitis) (Fig. 10-5).
Easily made if the pattein is iecognized and a
caieful histoiy is taken. Diffeiential diagnosis is
piimaiily acute iiiitant contact deimatitis, with
stieaky pattein poison ivy deimatitis (see Figs.
2-8, 2-10), but this is eczematous.
C0kS
May be an impoitant occupational pioblem, as
in celeiy pickeis. The acute eiuption has a shoit
life and fades spontaneously, but the pigmenta-
tion may last foi many weeks.
MANACMNI
Wet diessings may be indicated in the acute
vesiculai stage. Topical glucocoiticoids.
IA8I 10-5 Common Iopica| Phototo\ic Aents
Ageot xpos0re
koe Beu+| 0p|||+|ro|o|c e\+r|u+||ou
||uo|ece|u |,e
|u|ocour+||u 0ccu| u+|u|+||, |u p|+u| (ro||, C|c- pp., ||||-c- pp., ||u|| +ud
.ee|+||e (||re, |erou, ce|e|,, ||, p+||e,, p+|u|p), ued |u pe||ure +ud
core||c (e.., o|| o| |e|+ro|), +ud ued |o| |op|c+| p|o|oc|ero||e|+p,
I+| Iop|c+| ||e|+peu||c +eu|, |oo||u r+|e||+|, |o+d |+|||u
FICk 10-4 Fhytophotodermatts |u +
+3,e+|o|d r+u W|o W+ uu|+|||u |u + re+doW.
|rred|+|e|, |e|o|e .e|c|e +ud ||||e| +||e e|,||er+
|ou |e|ou r+, +ppe+| |+|ed, |.|u ||e |+|e |rp|e
|ou o| |e|u p+pu|+|. |o|e ||e+|, p+||e|u.
FICk 10-5 8er|oque dermatts I|e p+||eu|
|+d +pp||ed + ||+|+u| |+|| o|| |o |e| |ou|de|
+ud c|e| |u| |oWe|ed ou|, ||e ||ou| o| |e| |od,
|e|o|e o|u |u|o ||e uu. I|e |+|| o|| cou|+|ued o||
o| |e|+ro|, +ud p|reu|+||ou | uoW uo|ed W|e|e
|| |||c||ed doWu ||or ||e |ou|de| |o ||e |u||oc|.
(Cou||e, o| ||. I|or+ 'c|W+|/.)
FICk 10-3 Fhytophotodermatts (p|aot
+ |ht): acute wth b|sters I|ee |u||+e We|e
||e |eu|| o| e\pou|e |o ur|||||e|+e +ud ||e uu.
I|| 50,e+|o|d |oueW||e W+ Weed|u |e| +|deu
ou + uuu, d+,. ur|||||e|+e cou|+|u |e|+p|eu
(5re||o\,po|+|eu), W||c| | + po|eu| |op|c+|
p|o|o|o\|c c|er|c+|.
I|| |eu|| ||or |u|e|+c||ou o| + p|o|o+||e|eu
+ud u\A |+d|+||ou.
|u eu|||/ed |ud|.|du+| e\pou|e |o + p|o|o+||e|
eu +ud uu|||| |eu|| |u + p|u||||c ec/er+|ou
e|up||ou cou||ued |o e\poed ||e +ud c||u|c+||,
|ud|||uu||+||e ||or +||e||c cou|+c| de|r+||||.
|u ro| p+||eu| ||e e||c|||u d|u/c|er|c+| |+
|eeu +pp||ed |op|c+||,, |u| ,|er|c e||c||+||ou
+|o occu|.
Fh0I0AIIkCIC 0kC-[ChMICAI-IN0C0 Fh0I0SNSIIIvII |C|9 . o92.12
|C|0 . |5o.
FI0MI0I0C
Ae oI 0oset Moie common in adults.
kace All skin phototypes and colois.
Iocdeoce Photoalleigic diug ieactions occui
much less fiequently than do phototoxic diug
ieactions.
Topically applied chemical/diug plus UVA
iadiation. The chemicals aie disinfectants, an-
timiciobials, agents in sunscieens, peifumes in
afteishaves, oi whiteneis (Table 10-6). The chemi-
cal agent piesent in the skin absoibs photons and
foims a photopioduct; this then binds to a soluble
oi membiane-bound piotein to foim an antigen
to which a type IV immune iesponse is elicited.
Since photoalleigy depends on individual im-
munologic ieactivity, it develops in only a small
peicentage of peisons exposed to diugs and
light and is elicited only in those who have been
sensitized. Photoalleigy can also be induced by
systemic administiation of a diug and elicited by
topical administiation of the same diug, and vice
veisa. UVA is always iequiied.
May be uncleai in that the initial exposuie
induces sensitization to delayed-type hypei-
sensitivity ieactions, and the eiuption occuis
only on subsequent exposuie. Topically applied
photosensitizeis aie the most fiequent cause of
photoalleigic eiuptions (Table 10-6). Eiuption
is highly piuiitic.
Sko Iesoos The moiphology of the skin
ieaction is much diffeient fiom that in phototoxic
diug sensitivity. Acute photoalleigic ieaction
patteins aie clinically indistinguishable fiom
alleigic contact deimatitis (Fig. 10-6): papulai,
vesiculai, scaling, and ciusted. Occasionally
theie can also be a lichenoid eiuption similai
to lichen planus. In chionic diug photoalleigy,
theie is scaling, lichenification, and maiked
IA8I 10-6 Iopica| Photoa||erens
6ro0p 0hem|ca| hame

'uuc|eeu u\B +|o||e|.
para-Amoobeotoc acd
(FA8A)
C|uu+r+|e
'+||c,|+|e
u\A +|o||e|.
Au|||+u||+|e
8eotopheoooes
||+|+uce 6-Methy|coumaro
Musk ambrette
'+ud+|Wood o||
Au|||+c|e||+| 0bromosa|cy|ao|de
Ietrach|orosa|cy|ao|de
I||||oro+||c,|+u|||de
C||o||e\|d|ue
||re||,|o|d|re||,| |,d+u|o|u
ne\+c||o|op|eue
8thooo|
||c||o|op|eue
I||c|o+u
Su|Iooamdes
Au|||uu+| I||o||c||o|op|euo|
Buc|o+r|de
B|oroc||o|o+||c,|+u|||de
0||e| Ch|orpromatoe
C||oqu|uo|
Ke|op|o|eu
0|+qu|udo\
||ore||+/|ue
0u|u|d|ue
I||ou|e+
c
Corrou|, |epo||ed d|u +|e p||u|ed |u |o|d.
'ou|ce. Ad+p|ed ||or n ||r, |u K wo||| e| +| (ed). ||cc|:|'
|-c||, C--c| !-!:- 1|| ed. |eW \o||,
\cC|+Wn|||, 2003.
piuiitus mimicking atopic deimatitis oi, again,
chionic alleigic contact deimatitis (Fig. 10-6;
see also Eczema/Deimatitis," Section 2.)
DIstrIhutIvn Confined piimaiily to aieas ex-
posed to light (distiibution pattein of photo-
sensitivity), but theie may be spieading onto
adjacent nonexposed skin; theiefoie, it is not so
well ciicumsciibed as in phototoxic ieactions.
Of diagnostic help is the fact that in the face
the uppei eyelids, the aiea undei the nose, and
a thin stiip of skin between the lowei lip and
the chin aie often spaied (shaded aieas) (Fig.
10-6).
0ermatopatho|oy Acute and chionic
delayed-type hypeisensitivity ieaction: epideimal
spongiosis with lymphocytic infiltiation.
0IACN0SIS
Histoiy of exposuie to diug is most impoitant,
as well as the types of moiphologic changes in
the skin: this is essentially an alleigic contact
deimatitis pattein. In essence, the diffeiential
diagnosis between this and phototoxic eiup-
tions is identical to that desciibed foi toxic/
iiiitant and alleigic contact deimatitis (see
Section 2).
Diagnosis iequiies the use of patch and
photopatch tests. Photopatch tests aie done in
duplicate because photoalleigens can also cause
contact hypeisensitivity. Photoalleigens aie ap-
plied to the skin and coveied. Aftei 24 h, one
set of the duplicate test sites is exposed to UVA
while the othei set iemains coveied; test sites
aie iead foi ieactions aftei 48-96 h. An eczema-
tous ieaction in the iiiadiated site but not in the
FICk 10-6 Fhotoa||erc dru-oduced photoseostvty I|| o0,e+|o|d r+|e |oW +u ec/er+|ou
de|r+|||| |u ||e |+ce. ne W+ |+||u |||re||op||ru||+re||o\+/o|e. |o|e p+||u o| e,e||d (p|o|ec|ed |, uu
|+e), uude| ||e uoe, +ud ||e +|e+ uude| ||e |oWe| ||p (|+ded +|e+)
noniiiadiated site confiims photoalleigy to the
paiticulai agent tested.
C0kS AN0 Fk0CN0SIS
Photoalleigic deimatitis can peisist foi months
to yeais. This is known as erssen |g|
reaton , oi t|ront atnt Jermas (Fig.
10-7, B), The classic geneialized peisistent
light ieactions weie caused by exposuie to
soaps containing salicylanilides (Table 10-6).
In erssen |g| reaton, the action spectium
usually bioadens to involve UVB, and the
condition peisists despite discontinuation of
the causative photoalleigen, with each new UV
exposuie aggiavating the condition. Chionic
eczema-like lichenified and extiemely itchy
confluent plaques iesult (Fig. 10-7, B), which
lead to gioss disfiguiement and a distiessing
situation foi the patient. As the condition is
now independent of the oiiginal photoalleigen
and is aggiavated by each new solai exposuie,
avoidance of photoalleigen does not cuie the
disease. In contiast to eailiei belief, chionic
actinic deimatitis does not piogiess to lym-
phoma.
MANACMNI
In seveie cases, immunosuppiession (azathio-
piine plus glucocoiticoids oi oial cyclospoiine)
is iequiied.
FICk 10-T 0ru-oduced photoseostvty: perssteot |ht eruptoo . E|,||er+|ou p|+que cou
||ued |o ||e |+ce +ud uec|, p+||u ||e |ou|de|. I|| r+|e |+ e\c|uc|+||u p|u|||u.

FICk 10-T 0ru-oduced photoseostvty: perssteot |ht eruptoo (Cootoued ) 8. |e|||eu| ||||
e|up||ou W||| |u|||||+|ed ec/er+|ou e|up||ou |u ||e |+ce +ud ||e uec|.
8
FI0MI0I0C
Iocdeoce Most common photodeimatosis.
Pievalence fiom 10% in Boston, 14% in Lon-
don, to 21% in Sweden. Aveiage age is 23 yeais,
much moie common in females. All iaces,
but most common in SPT I, II, III, and IV. In
Ameiican Indians (Noith and South Ameiica)
theie is a |ereJary type of PMLE that is called
atnt rurgo.
Ceoraphy PMLE is less fiequently obseived
in aieas that have high solai intensity thiough-
out the yeai and in peisons who have adapted
to peisistent sun exposuies. In fact, PMLE often
occuis foi the fiist time in peisons tiaveling foi
shoit vacations to tiopical aieas in wintei fiom
noithein latitudes.
FAIh0CNSIS
Possibly a delayed-type hypeisensitivity ie-
action to an (auto-) antigen induced by UVR;
suggested by the moiphology of the lesions and
the histologic pattein, which shows an infiltia-
tion of T cells. Moie commonly, UVA is the
action spectium, but PMLE lesions have been
evoked with UVB and with both UVA and UVB.
Since UVA is tiansmitted thiough window
glass, PMLE can be piecipitated while iiding
in a cai. Aieas of the skin habitually exposed
(face and neck) aie often spaied, despite seveie
involvement of the aims, tiunk, and legs.
0oset aod 0uratoo oI Iesoos PMLE appeais
in spiing oi eaily summei, and not infiequently
the eiuption does not iecui by the end of sum-
mei, suggesting a haidening." PMLE most of-
ten appeais within houis of exposuie and, once
established, peisists foi 7 to 10 days, theieby
limiting the vacationei's subsequent time in the
|o|,ro|p|ou |||| e|up||ou (|\|E) | + |e|r
||+| dec|||e + |oup o| |e|e|oeueou, |d|
op+|||c, +cqu||ed, +cu|e |ecu||eu| e|up||ou c|+|
+c|e||/ed |, de|+,ed +|uo|r+| |e+c||ou |o u\k.
\+u||e|ed |, .+||ed |e|ou, |uc|ud|u e|,||er+
|ou r+cu|e, p+pu|e, p|+que, +ud .e|c|e.
noWe.e|, |u e+c| p+||eu| ||e e|up||ou | cou|
|eu||, rouoro|p|ou.
B, |+| ||e ro| ||equeu| ro|p|o|o|c |,pe +|e
||e p+pu|+| +ud p+pu|o.e|cu|+| e|up||ou.
F0IM0kFh0S IIChI kFII0N |C|9 . o92.12
|C|0 . |5o.+
sun. Symptoms aie piuiitus (may piecede the
onset of the iash) and paiesthesia (tingling).
Sko Iesoos The papulai (Fig. 10-8) and
papulovesiculai types aie the most fiequent.
Less common aie plaques oi uiticaiial
plaques (Fig. 10-9). The lesions aie pink to
ied. In the individual patient, lesions aie
quite monomoiphous, i.e., eithei papulai
oi papulovesiculai oi uiticaiial plaques.
Recuiiences follow the oiiginal pattein.
DIstrIhutIvn The eiuption often spaies the
face and appeais most fiequently on the foie-
aims, V aiea of the neck, aims, and chest (Fig.
10-8). The lesions may also occui on the face
(Fig. 10-9), if theie has not been pievious ex-
posuie.
0ermatopatho|oy Edema of the epideimis,
spongiosis, vesicle foimation, and mild lique-
faction degeneiation of the basal layei. A dense
lymphocytic infiltiate is piesent in the deimis,
with occasional neutiophils. Theie is edema of
the papillaiy deimis and endothelial swelling.
ImmuooI|uoresceoce (0rect) Negative. ANA
negative. Theie is no leukopenia.
0IACN0SIS
The diagnosis is not difficult: delayed onset
of eiuption, chaiacteiistic moiphology, his-
topathologic changes that iule out lupus eiy-
thematosus, and the histoiy of disappeaiance
of the eiuption in days. In plaque-type PMLE,
a biopsy and immunofluoiescence studies aie
mandatoiy to iule out SLE (Fig. 10-9). P|oo-
esng is done with both UVB and UVA. Test
sites aie exposed daily, staiting with 2 MEDs
of UVB and UVA, iespectively, foi 1 week to 10
days, using inciements of the UV dose. In 50%
of patients, a PMLE-like eiuption will occui
FICk 10-8 Fo|ymorphc |ht eruptoo C|u|e| o| cou||ueu|, e\||ere|, p|u||||c p+pu|e ou ||e e\poed
c|e|, occu||ed |u +u A|+||+u r+u ||e d+, |o||oW|u ||e |||| uu e\pou|e o| ||e e+ou. I|e e|up||ou +|o
|u.o|.ed ||e +|r, |u| p+|ed ||e |+ce +ud do|+| |+ud.
FICk 10-9 Fo|ymorphc |ht eruptoo E|,||er+|ou p|+que |u ||e |+ce |o||oW|u |||| uu e\pou|e o|
||e e+ou. I|e |u||e|||, d|||||u||ou | .e|, |r||+| |o ||+| o| |upu e|,||er+|ou.
in the test sites, confiiming the diagnosis. The
eiuption in the test site mimicks the type of
PMLE in that paiticulai patient. This also helps
to deteimine whethei the action spectium is
UVB, UVA, oi both.
C0kS AN0 Fk0CN0SIS
The couise is chionic and iecuiient and may,
in fact, become woise each season. Although
some patients may develop toleiance" by the
end of the summei, the eiuption usually iecuis
the following spiing and/oi when the peison
tiavels to tiopical aieas in the wintei. Howevei,
spontaneous impiovement oi even cessation of
eiuptions occuis aftei yeais.
MANACMNI
Freveotoo Sunblocks, even the potent UVA-
UVB sunscieens, aie not always effective but
should be tiied fiist in eveiy patient.
Systemc -Caiotene, 60 mg thiee times a day
foi 2 weeks, befoie going in the sun. Oial pied-
nisone 20 mg/day given 2 days befoie and 2 days
duiing exposuie is a good piophylaxis. Also,
intiamusculai tiiamcinolone acetonide, 40 mg,
will suppiess an eiuption when administeied a
few days befoie a tiip to a sunny iegion.
FvA Fhotochemotherapy This is veiy effec-
tive when given in eaily spiing by inducing tol-
eiance" foi the summei. PUVA tieatments have
to be given befoie the sunny season, have to be
iepeated each spiing, but aie usually not neces-
saiy foi moie than 3 oi 4 yeais. Narrow-|anJ
UVB (311 nm) is used with equal success.
uucorrou uu|||||uduced W|e+||u cou||ued
|o e\poed |od, ||e.
E|up||ou occu| W||||u r|uu|e o| e\pou|e +ud
|eo|.e |u + |eW |ou|. \e|, d|+|||u +ud ore
||re |||e |||e+|eu|u.
Ac||ou pec||ur | u\B, u\A, +ud .||||e |||| o|
+u, cor||u+||ou ||e|eo|. \o| corrou|, u\A
(||. 00).
| +u |rred|+|e |,pe | |,pe|eu|||.||, |epoue
|o cu|+ueou +ud/o| c||cu|+||u p|o|o+||e|eu.
I|e|+p,. ru|||p|e p|o|o||e|+p, e|ou |u |oW
|u| |uc|e+|u doe ou ||e +re d+, ('|u|
|+|deu|u), o|+| |rruuoupp|e|.e +eu| o|
p|+r+p|e|e|.
||e.eu||ou. uu +.o|d+uce, uuc|eeu W||| |||
p|o|ec||ou |+c|o| ++|u| +c||ou pec||ur.
S0IAk kIICAkIA |C|9 . 103.9
|C|0 . |5o.!
\+||ou W+.e|eu|| o| u\k +ud/o| .||||e ||||
c+u e||c|| o| +|+.+|e + uur|e| o| de|r+|oe.
|u ||ee c+e ||e e|up||ou | |u.+||+||, |r||+| |o
||+| o| ||e p||r+|, coud|||ou.
Au +|||e.|+|ed ||| | |.eu |u +|p|+|e||c+| o|de|
|u I+||e 01, |u| || |ou|d |e erp|+|/ed ||+|
+rou ||ee d|o|de| '|E | |, |+| ||e ro|
|rpo||+u|.
Fh0I0XACk8AI0 0kMAI0SS
IA8I 10-T 0iseases E\acerbated by U|travio|et |rradiation
Acue |e||+|+
A|op|c ec/er+ |erp||u |o||+ceu (e|,||er+|ou)
C+|c|uo|d ,ud|ore |||,||+| |u||+ p||+||
Cu|+ueou I ce|| |,rp|or+ |o||+|
|+||e| d|e+e ke||cu|+|e e|,||er+|ou ruc|uo| ,ud|ore
|e|r+|or,o||| ko+ce+
||er|u+|ed upe|||c|+| +c||u|c po|o|e|+|o| 'e|o|||e|c de|r+||||
E|,||er+ ru||||o|re Iupus erythematosus
|+r|||+| |eu|u c||ou|c perp||u I|+u|eu| +c+u||o|,||c de|r+|o|
(n+||e,n+||e, d|e+e) (C|o.e| d|e+e)
Ke|+|o| |o|||cu|+|| (|+||e| d|e+e) ne|pe |+||+||
||c|eu p|+uu
FICk 10-10 So|ar urtcara, test stes I|e uppe| |oW o| ||e |erp|+|e |e| ||e We|e e\poed |o |uc|e+
|u doe o| u\B +ud |e.e+|ed ou|, e|,||er+ (||u|e |ud|c+|e rl/cr
2
+pp||ed). 2+ |ou| |+|e| ||e |erp|+|e |e|
||e |u ||e |oWe| |oW We|e e\poed |o 0.5 +ud l/cr
2
u\A (W||c| +|e e\||ere|, |oW doe) +ud |rred|+|e|,
+||e| ||e e\pou|e ||| p|c|u|e W+ |+|eu. |o|e r+|.e u|||c+||+| |e+c||ou |u ||e u\Ae\poed |e| ||e.
|o|p|,||+ cu|+ue+ |+|d+ (|CI) occu| ro||, |u
+du||.
|+||eu| do uo| p|eeu| W||| c|+|+c|e||||c p|o
|oeu|||.||, |u| W||| corp|+|u| o| '||+||e ||u,
.e|c|e, +ud |u||+e, p+|||cu|+||, ou ||e do|+ o|
||e |+ud, +||e| r|uo| ||+ur+.
I|e d|+uo| | cou|||red |, ||e p|eeuce o| +
p|u||||ed ||uo|eceuce |u ||e u||ue W|eu e\+r
|ued W||| + wood |+rp.
|CI | d|||uc| ||or .+||e+|e po|p|,||+ (\|) +ud
+cu|e |u|e|r|||eu| po|p|,||+ (A||) |u ||+| p+||eu|
W||| |CI do uo| |+.e +cu|e |||e|||e+|eu|u
+||+c|.
|u|||e|ro|e, ||e d|u ||+| |uduce |CI +|e |eWe|
||+u ||e d|u ||+| |uduce \| +ud A||.
|o| c|+|||c+||ou o| ||e po|p|,||+, ee I+||e
03.
F0kFhkIA CIANA IAk0A |C|9 . 211.
|C|0 . E30.
MIA80IIC Fh0I0SNSIIIvII-Ih F0kFhkIAS
IA8I 10-8 C|assification and 0ifferentia| 0ianosis of Porphyrias
0oogeo|ta| Porphyr|a |oterm|tteot
rythropo|et|c rythropo|et|c 00taoea Var|egate Ac0te
Porphyr|as Protoporphyr|a Tarda Porphyr|a Porphyr|a
|u|e|||+uce Au|oor+| Au|oor+| Au|oor+| Au|oor+| Au|oor+|
|ece|.e dor|u+u| dor|u+u| dor|u+u| dor|u+u|
(|+r|||+| |o|r)
'|u +ud ,rp|or
||o|oeu|||.||, \e \e \e \e |o
Cu|+ueou |e|ou \e \e \e \e |o
A||+c| o|
+|dor|u+| p+|u |o |o |o \e \e
|eu|op,c||+|||c
,ud|ore |o |o |o \e \e
|+|o|+|o|,
+|uo|r+||||e + + + + +
ked ||ood ce||
||uo|eceuce + + - - -
u|opo|p|,||u +++ | | | |
Cop|opo|p|,||u ++ + | | |
||o|opo|p|,||u (+) +++ | | |
||+r+
||uo|eceuce + + - + -
u||ue
||uo|eceuce - - + -
|o|p|o||||uoeu | | | (+++) (+++)
u|opo|p|,||u +++ | +++ +++ +++
|ece
||o|opo|p|,||u + ++ | +++ |
|o|e. |, uo|r+|, +, +|o.e uo|r+|, ++, rode|+|e|, |uc|e+ed, +++, r+||ed|, |uc|e+ed, (+++), ||equeu||, |uc|e+ed (depeud ou W|e||e|
p+||eu| |+ +u +||+c|, o| | |u |er||ou), (+), |uc|e+ed |u ore p+||eu|.
FI0MI0I0C
Onset 30 to 50 yeais, iaiely in childien; fe-
males on oial contiaceptives; males on estiogen
theiapy foi piostate cancei. Equal in males and
in females.
heredty Most PCT patients have ye I ( at-
qureJ ) induced by diugs oi chemicals. Tye II
(|ereJary ), autosomal dominant; possibly
these patients actually have VP, but this is not
yet iesolved. Theie is also a dual" type with VP
and PCT in the same family.
PCT is caused by eithei an inheiited oi acquiied
deficiency of UROGEN decaiboxylase. In type
I (spoiadic, acquiied PCT-symptomatic) the
enzyme is deficient only in the livei; in type
II (PCT-heieditaiy) it is also deficient in ied
blood cells (RBCs) and fibioblasts. C|emta|s
anJ Jrugs |a nJute PCT. Ethanol, estiogen,
hexachloiobenzene (fungicide), chloiinated
phenols, iion, tetiachloiodibenzo- -dioxin.
High doses of chloioquine lead to clinical
manifestations in latent" cases (low doses aie
used as tieatment). O|er reJsosng [ators :
Diabetes mellitus (25%), hepatitis C viius, also,
hemochiomatosis.
0uratoo oI Iesoos No acute skin changes
but giadual onset. Patients may piesent with
fiagility of skin and bullae on the hands and
feet based on a photosensitivity ieaction to sun
and yet will have a suntan. Pain fiom eiosions in
easily tiaumatized skin (fiagile skin").
Sko Iesoos Tense bullae and eiosions on
noimal-appeaiing skin (Fig. 10-11); slowly heal
to foim pink atiophic scais, milia (1-2 mm)
on doisa of hands and feet, nose, foiehead, oi
(bald) scalp. Puiple-ied suffusion (heliotiope")
of cential facial skin (Fig. 10-12 ), especially
peiioibital aieas. Biown hypeimelanosis, diffuse,
on exposed aieas. Hypeitiichosis of face (Fig.
10-13). Scleiodeima-like changes, diffuse oi
ciicumsciibed, waxy yellowish-white aieas on
exposed aieas of face (Fig. 10-12 B ), neck, and
tiunk, spaiing the doubly clothed aiea of the
bieast in females.
FICk 10-11 Forphyra cutaoea tarda Bu||+e +ud +||op||c dep|reu|ed c+| ou ||e do|ur o| |o||
|+ud. I|| | uo| +u +cu|e |e+c||ou |o |u|||+| uu e\pou|e |u| de.e|op o.e| ||re W||| |epe+|ed uu e\pou|e
+ud occu| +||e| r|uo| ||+ur+. I|e p+||eu| p|eeu| W||| + |||o|, o| ||+||e ||u +ud |u||+e.
0ermatopatho|oy Bullae, subepideimal with
festooned" (undulating) base. PAS staining
ieveals thickened vasculai walls. Paucity of an
inflammatoiy infiltiate.
ImmuooI|uoresceoce IgG and othei immu-
noglobulins at the deimal-epideimal junction
and in and aiound blood vessels, in the sun-
exposed aieas of the skin.
Chemstry Plasma iion and livei enzymes may
be incieased. High level of iion stoies in the
livei. The patient may have hemochiomatosis.
B|ooJ g|utose is incieased in those patients with
diabetes mellitus (25% of patients).
Forphyro Studes o Stoo| aod roe
(Table 10-8) Incieased uiopoiphyiin (I iso-
mei, 60%) in uiine and plasma. Incieased
isocopiopoiphyiin (type III) and 7-caiboxyl-
poiphyiin in the feces. In contiast, VP has
maikedly elevated fecal piotopoiphyiin as the
diagnostic hallmaik. No inciease in -aminole-
vulinic acid oi poiphobilinogen in the uiine.
Smp|e Iest Wood lamp examination of the
uiine shows oiange-ied fluoiescence (Fig. 10-
14); to enhance, add a few diops of 10% hydio-
chloiic acid.
Iver 8opsy Reveals poiphyiin fluoiescence
and often fatty livei. May also show ciiihosis,
hemochiomatosis.
By clinical featuies, pink-ied fluoiescence of
uiine and elevated uiinaiy poiphyiins. Bullae
on doisa of hands and feet can occui in seuJo-
PCT (see Section 22). Phototoxic ieactions oc-
cui in chionic ienal failuie with hemodialysis.
Tanning salon iadiation (visible and UVA). May
occasionally iesemble dyshidiotic eczema but
bullae aie on the doisa. EJermo|yss |u||osa
atqusa (see Section 6) has the same clinical
pictuie (incieased skin fiagility, easy biuising,
and light- and tiauma-piovoked bullae) and
some of the histology (subepideimal bullae
with little oi no deimal inflammation).
MANACMNI
1. Avoid ethanol, stop diugs that could be
inducing PCT (such as estiogen), and elim-
inate exposuie to chemicals (chloiinated
phenols, tetiachloiodibenzo- -dioxin). In
some patients, complete avoidance of etha-
nol ingestion will iesult in a clinical and
biochemical iemission and in depletion of
the high level of iion stoies in the livei.
2. Phlebotomy is done by iemoving 500 mL of
blood at weekly oi biweekly inteivals until
the hemoglobin is decieased to 10 g. Clinical
FICk 10-12 Forphyra cutaoea tarda . \e|, u|||e pe||o||||+| .|o|+ceou co|o|+||ou.
and biochemical iemission occuis within 5
to 12 months aftei iegulai phlebotomy. Re-
lapse within a yeai is uncommon (5-10%).
3. Low-dose chloioquine is used to induce
iemission of PCT in patients in whom
phlebotomy is contiaindicated because of
anemia. Since chloioquine can exaceibate
the disease and, in highei doses, may even
induce hepatic failuie in these patients, this
tieatment iequiies consideiable expeiience.
Howevei, long-lasting iemissions and, in a
poition of patients, clinical and biochemical
cuie" can be achieved.
The best appioach cuiiently used by one of us
(K.W.) is to stait with a couise of thiee con-
secutive phlebotomies eveiy othei day followed
by 150 mg/d, of chloioquine PO. Close clini-
cal and laboiatoiy monitoiing (tiansaminases,
poiphyiin excietion in uiine), aie iequiied to
adjust the chloioquine dose, which is eventually
tapeied to 150 mg twice a week and continued
foi seveial months.
FICk 10-12 Forphyra cutaoea tarda (Cootoued) 8. 'c|e|ode|ro|d |||c|eu|u, c+|, +ud e|o|ou
ou ||e |o|e|e+d.
FICk 10-13 Forphyra cutaoea tarda n,pe||||c|o| |u + Wor+u W|o |+d |eeu ou + p|o|oued |e|reu
W||| e||oeu. uude| wood |||| |e| u||ue |oWed + ||||| co|+||ed ||uo|eceuce, + |oWu |u ||. 0+.
FICk 10-14 Forphyra cutaoea tarda: Wood |ht Co|+||ed ||uo|eceuce o| ||e u||ue o| + p+||eu| W|||
|CI + corp+|ed |o ||+| o| + uo|r+| cou||o|.
FI0MI0I0C
Ae oI 0oset At pubeity; peak, second to
fouith decades.
kace All iaces; especially common in white
South Afiicans (3:1000) (a laige piopoition of
the piesent white population was descended
fiom an eaily Dutch settlei who emigiated to
South Afiica fiom Holland in 1680 to wheie VP
can be tiaced).
Iocdeoce It is incieasingly iecognized in Eu-
iope (Finland) and the United States.
heredty Autosomal dominant.
PROTOGEN oxidase defect iesulting in an
accumulation of piotophyiinogen in the livei,
which is excieted in the bile and is nonenzy-
matically conveited to piotopoiphyiin; this
accounts foi the high fecal piotopoiphyiin.
The basic metabolic defect is accentuated by
ingestion of ceitain diugs (Table 10-9), with
the iesultant piecipitation of acute attacks of
abdominal pain and neuiopsychiatiic disoideis
(deliiium, seizuies, peisonality changes).
\+||e+|e po|p|,||+ (\|) | + e||ou +u|oor+|
dor|u+u| d|o|de| o| |ere ||o,u||e|.
'||u |e|ou ||+| +|e |deu||c+| |o ||oe o| |CI
(.e|c|e +ud |u||+e, ||u ||+||||,, r|||+, +ud c+|
||u o| ||e do|+ o| ||e |+ud +ud ||ue|).
Acu|e +||+c| o| +|dor|u+| p+|u, ueu|op,c||+|||c
r+u||e|+||ou.
|uc|e+ed e\c|e||ou o| po|p|,||u, epec|+||, c|+|
+c|e||||c +|e ||| |e.e| o| p|o|opo|p|,||u |u ||e
|ece.
',, |o|p|,||+ .+||e+|+.
*
|u 'ou|| A|||c+
vAkICAI F0kFhkIA |C|9 . 211.
|C|0 . E30.2 ( )
Chaoe wth Seasoos Skin lesions occui duiing
the summei season but may peisist thioughout
the wintei; lesions iesult fiom exposuie to sun-
light. Painful eiosions, skin fiagility.
Systems kevew Acute attacks of abdominal
pain, constipation, nausea and vomiting, muscle
weakness, seizuies, confusional state, psychiatiic
symptoms (depiession, coma); iaiely, cianial
neive involvement, bulbai paialysis, sensoiy
loss, and paiesthesias.
0ru xposure See Table 10-9.
Sko Iesoos PCT-like vesicles oi, moie
commonly, bullae (Fig. 10-15); eiosions, milia;
scleiosis (scleiodeima-like changes); scais
(pink, atiophic). Peiioibital heliotiope hue,
diffuse melanodeima and hypeitiichosis on
exposed aieas. Localization to doisa of hands,
fingeis, and feet, as in PCT.
Msce||aoeous Fodos Neuiologic, especially
peiipheial neuiopathy.
0ermatopatho|oy As foi PCT
IA8I 10-9 0rus hatardous to Patients with Varieate Porphyria
Aue||e||c. |+||||u|+|e +ud |+|o||+ue |r|p|+r|ue
Au||cou.u|+u|. |,d+u|o|u, \e||,|dop+
c+||+r+/ep|ue, e||ou\|r|de, \|uo| ||+uqu|||/e|. c||o|d|+/epo\|de,
re||u\|r|de, p|euu\|r|de, p||r|doue d|+/ep+r, o\+/ep+r, ||u|+/ep+r,
rep|o|+r+|e
Au||r|c|o||+| +eu|. c||o|+rp|eu|co| ||eo|u|.|u,
uo.o||oc|u, p,|+/|u+r|de, |eu|+/oc|ue
u||ou+r|de ||eu,||u|+/oue
'u||ou,|u|e+. c||o|p|op+r|de,
E|o| p|ep+|+||ou |o||u|+r|de
E||,| +|co|o| I|eop|,|||ue
no|roue. e||oeu, p|oe||u, o|+| cou||+cep||.e
p|ep+|+||ou
Ceoera| Iaboratory xamoatoo PvrphyrIns
See Table 10-8.
P|usmu Distinctive plasma fluoiescence with
emission maximum at 626 nm.
UrIne Incieased poiphobilinogen duiing
acute attacks.
Stvv| High piotopoiphyiin.
C0kS AN0 Fk0CN0SIS
Lifetime disease. Piognosis good, if exaceibating
factois aie avoided. Raiely, death can occui aftei
ingestion oi injection of diugs (e.g., baibitu-
iates, geneial anesthesia) that induce incieased
amounts of cytochiome P450 and cieate a de-
mand foi incieased synthesis of heme.
Pseudopoiphyiia, scleiodeima, acquiied epidei-
molysis bullosa, heieditaiy copiopoiphyiia, PCT.
MANACMNI
None; oial -caiotene may oi may not contiol
the skin manifestations but has no effect on
poiphyiin metabolism oi the impoitant sys-
temic manifestations.
FICk 10-15 vareate porphyra Bu||+e ou ||e do|ur o| ||e |oo| +ud |oe, + corrou ||e o| uu
e\pou|e |u p+||eu| We+||u opeu |oo|We+|. I|| +2,e+|o|d |er+|e W+ d|+uoed W||| po|p|,||+ cu|+ue+ |+|d+.
I|e |e|ou |u po|p|,||+ cu|+ue+ |+|d+ +|e |deu||c+| |o ||e |e|ou |u .+||e+|e po|p|,||+. I|| p+||eu|, |oWe.e|,
+.e + |||o|, o| |ecu||eu| +||+c| o| +|dor|u+| p+|u, W||c| W+ + c|ue |o ||e d|+uo| o| .+||e+|e po|p|,||+,
||| d|+uo| W+ e|+||||ed |, ||e de|ec||ou o| e|e.+|ed |oo| p|o|opo|p|,||u. \+||e+|e po|p|,||+ (o| 'ou||
A|||c+u po|p|,||+) | +||u |o +cu|e |u|e|r|||eu| po|p|,||+, |u W||c| ||e|e +|e uo ||u |e|ou |u| + |+|+| ou|core
r+, occu| W||| |ue||ou o| ce||+|u d|u (ee I+||e 09). |u 'ou|| A|||c+ e.e|, W|||e p+||eu| W|o | c|edu|ed
|o| r+jo| u|e|, ru| |+.e |+|o|+|o|, |e| |o| po|p|,||u |uce .+||e+|e po|p|,||+ | corrou |u ||+| couu||,.
FI0MI0I0C
Iocdeoce Uncommon; seiies iepoited fiom
Euiope (in The Netheilands, 1:100,000; Austiia,
United Kingdom) and the United States.
Ae oI 0oset Acute photosensitivity begins
eaily in childhood; iaiely, late onset in eaily
adulthood.
Sex Equal in males and females.
kace All ethnic gioups, including blacks.
heredty Autosomal dominant with vaiiable
penetiance.
FAIh0CNSIS
The defective enzyme is feiiochelatase. This
defect occuis at the step in poiphyiin metab-
olism in which piotopoiphyiin is conveited
to heme by feiiochelatase. This leads to an
accumulation of piotopoiphyiin that is highly
photosensitizing.
Impoitant sequence of symptoms: stinging,
buining, and itching occui w|n a [ew mnues
of sunlight exposuie; eiythema and edema ap-
peai only aftei 1 to 8 h. Childien may choose
not to go out in the diiect sunlight aftei a
few painful episodes, which may cause seiious
sociopsychologic pioblems. Symptoms occui
when exposed to sunlight thiough window
glass. Photosensitivity is less common in the
wintei months in tempeiate aieas.
Systems kevew Biliaiy colic, even in chil-
dien.
Sko Chaoes o Acute keactoos to Suo|ht
xposure Biight ied eiythema, latei edema
(swelling of hands especially), puipuia
especially on the nose, cheeks (Fig. 10-16),
I|| |e|ed||+|, re|+|o||c d|o|de| o| po|p|,||u
re|+|o||r | uu|que +rou ||e po|p|,||+
|u ||+| po|p|,||u o| po|p|,||u p|ecu|o| +|e
uu+||, uo| e\c|e|ed |u ||e u||ue.
E|,|||opo|e||c p|o|opo|p|,||+ (E||) | c|+|+c|e|
|/ed |, +u +cu|e uu|u|u|||e p|o|oeu|||.||,, |u
cou||+| |o ||e o||e| corrou po|p|,||+ (|CI o|
\|), |u W||c| o|.|ou +cu|e p|o|oeu|||.||, | |
+ p|eeu||u corp|+|u|.
',rp|or occu| |+p|d|, W||||u r|uu|e o| uu
e\pou|e +ud cou|| o| ||u|u +ud |u|u|u.
'||u |u +|e e|,||er+, eder+, +ud pu|pu|+.
k+|e|, ||e|e r+, |e c||||o| o| ||e ||.e| +ud ||.e|
|+||u|e.
(||e.eu||.e) ||e+|reu| cou|| o| c+|o|eue
|0.
',, E|,|||o|ep+||c p|o|opo|p|,||+.
kIhk0F0IIIC Fk0I0F0kFhkIA |C|9 . 211.
|C|0 . |30.0
backs of hands (Fig. 10-17), and tips of eais].
Uiticaiia uncommon; vesicles oi bullae iaiely
occui. These changes appeai within 1-8 h and
aftei subjective symptoms and subside aftei
seveial houis oi days.
Sko Chaoes AIter Chrooc kecurreot
xposures
Shallow, often lineai scais, on the nose and
doisa of the hands (aged knuckles"). Diffuse
wiinkling of the skin of the nose, aiound the
lips, and the cheeks, with obvious thickening
and a waxy coloi (Fig. 10-18). Ciusted, eiosive
lesions may occui on the nose and lips. In con-
tiast to PCT, absence of scleiodeimoid changes,
hypeitiichosis, and hypeipigmentation.
Ceoera| Medca| Fodos
Hemolytic anemia with hypeisplenism (iaie).
Cholelithiasis (12%), even in childien; stones
contain laige amounts of piotopoiphyiin. Livei
disease fiom massive deposition of piotopoi-
phyiin in hepatocytes occuis; fatal hepatic
ciiihosis is iaie, but occuis.
Forphyro Studes (See Table 10-8) Incieased
piotopoiphyiin in ied blood cells, plasma, and
stools, but no excietion in the uiine except in
the iaie cases with fatal hepatic ciiihosis. De-
cieased activity of the enzyme feiiochelatase in
the bone maiiow, livei, and skin fibioblasts.
Iver Fuoctoo Tests foi livei function in-
dicated. Livei biopsy: poital and peiipoital
fibiosis and deposits of biown pigment and bi-
iefiingent gianules in hepatocytes and Kupffei
cells. Ciiihosis and poital hypeitension may
develop.
kadoraphy Gallstones may be piesent.
Speca| xamoatoo Ior F|uoresceot rythrocytes
RBCs in a blood smeai exhibit a chaiacteiistic
ransen fluoiescence when examined with a
fluoiescence micioscope with a meicuiy oi
tungsten-iodide lamp that emits 400-nm iadi-
ation (Fig. 10-19).
0ermatopatho|oy Maiked eosinophilic ho-
mogenization and thickening of the blood vessels
in the papillaiy deimis; theie is an accumulation
of an amoiphous, hyaline-like eosinophilic sub-
stance in and aiound blood vessels.
0IACN0SIS
In EPP theie is photosensitivity with an exag-
geiated sunbuin iesponse without blisteis that
appeais much eailiei than oidinaiy sunbuin
eiythema. Also, the skin changes occui behind
window glass. T|ere s no o|er |oosensy
JsorJer n w|t| |e symoms aear so raJ|y
(mnues a[er exosure o sun|g|) . Poiphy-
iin examination establishes the diagnosis with
elevated fiee piotopoiphyiin levels in the RBCs
and in the stool. The fecal piotopoiphyiin is
most consistently elevated, but uiinaiy poiphy-
iins aie not. In chionic cases, the waxy thicken-
ing and wiinkling of facial skin is diagnostic.
0IIereota| 0aooss Hyalinosis cutis et
mucosae.
C0kS AN0 Fk0CN0SIS
EPP peisists thioughout life, but the photosen-
sitivity may become less appaient in late adult-
hood. Livei ciiihosis may become manifest
in adults. Raiely, fatal outcome due to hepatic
failuie.
MANACMNI
Theie is no tieatment foi the basic metabolic
abnoimality, but symptomatic ielief of the
photosensitivity can be achieved in most pa-
tients with oial -caiotene in divided doses of
180 mg/d. Theiapeutic levels of caiotenoids aie
achieved in 1-2 months. Patients on -caiotene
can iemain outdoois longei by a factoi of 8
to 10 but will still buin if exposuies aie too
long. Neveitheless, many patients can paitici-
pate in outdooi activities foi the fiist time.
Theie is no toxicity with piolonged tieatment
with -caiotene. Piotection by -caiotene can
be consideiably enhanced by PUVA-induced
tanning.
FICk 10-16 rythropo-
etc protoporphyra ||||ue
e|,||er+|ou We|||u o| ||e
uoe, |o|e|e+d, +ud c|ee|
W||| pe|ec||+| |ero|||+e +ud
|e|+u|ec|+|+. I|e|e +|e uo po|
p|,||u |u ||e u||ue. A c|ue |o ||e
d|+uo| | ||e |||o|, o| ||u||u
+ud |u|u|u W||||u + |o 5 r|u
o| uu e\pou|e. I|e |+ce o| |||
Wor+u +ppe+| ,e||oWo|+ue
|ec+ue |e W+ ou c+|o|eue,
W||c| o|.|ou|, d|d uo| p|o|ec|
|e| u|||c|eu||,.
FICk 10-1T rythropoetc protoporphyra \+|.e pe|ec||+|, cou||ueu| |ero|||+e ou ||e do|+ o|
||e |+ud o| + o,e+|o|d 2+ | +||e| e\pou|e |o ||e uu.
FICk 10-18 rythropoetc protoporphyra
E|,||er+, eder+, e|o|ou, c|u||u o| ||e uoe W||| |e
e.e|e c|+ue ou ||e c||u o| + 5,e+|o|d |er+|e. |eep
W||u|||u +ud + pecu||+| W+\, |||c|eu|u ou ||e uppe| ||p
+ud c|ee| r+|e ||e p+||eu| |oo| ruc| o|de| + ||e, +|e
|r||+| |o de|r+|o|e||o| |u p|o|o+ed ||u.
FICk 10-19 rythropoetc protoporphyra
||uo|eceu| |ed ||ood ce||. I|e |e|| p+ue| |oW ||e
||ood ce|| re+| .|eWed W||| .||||e ||||. I|e ||||
p+ue| W+ ||e +re re+| e\+r|ued W||| + |uu|eu
|od|de |+rp ||+| er|| ou|, +00ur |+d|+||ou. |o|e
|ed ||uo|eceuce o| e|,|||oc,|e. |o|e +|o ||+| ou|, +
r|uo|||, o| |ed ||ood ce|| ||uo|ece +ud ||+| |o d|||e|eu|
de|ee.
kepe+|ed o|+| |uju||e o.e| r+u, ,e+| u|||r+|e|,
c+u |eu|| |u ||e de.e|opreu| o| + ||u ,ud|ore,
!-c||-| (|ne).
|| occu| |u pe|ou W||| '|I | |o ||| +ud |u pe|ou
W||| '|I |\ W|o |+.e |+d |e+., curu|+||.e e\po
u|e |o uu||||, uc| + |||eu+|d +ud ou|doo|
Wo||e|, o.e| + |||e||re.
|ne dec|||e + po|,ro|p||c |epoue o| .+||ou
corpoueu| o| ||e ||u (epec|+||, ce|| |u ||e
ep|de|r|, ||e .+cu|+| ,|er, +ud ||e de|r+|
couuec||.e ||ue) |o p|o|oued +ud/o| e\ce|.e
uu e\pou|e.
|| e.e|||, depeud p||uc|p+||, ou ||e du|+||ou
+ud |u|eu||, o| uu e\pou|e +ud ou ||e |ud|eu
ou (cou|||u||.e) ||u co|o| +ud ||e c+p+c||, |o
|+u (|+cu||+||.e re|+u|u p|reu|+||ou).
|| ,ou W+u| |o derou||+|e |o +u o|de|
p+||eu| ||e |o|e o| u\k |u p|o|o+|u ju| |+.e
||r/|e| uud|e +ud corp+|e ||e qu+|||, o|
||/|e| |+c|+| ||u |o ||+| o| ||e up|+pu||c ||u.
0kMAI0hII0SIS ("Fh0I0ACINC") |C|9 . o92.1+
|C|0 . |51.9
Chk0NIC Fh0I00AMAC
FI0MI0I0C
Ae oI 0oset Most often in peisons >40
yeais.
Sex Highei incidence in males.
Sko Fhototype Peisons with SPT I and II aie
most susceptible, but peisons with SPT III and
IV and even V (biown skin coloi) can develop
DHe.
Iocdeoce Veiy common. The most susceptible
peisons with SPT I and II compiise about 25%
of the white population in the United States.
0ccupatoo Faimeis (faimei`s skin"), tel-
ephone linemen; sea woikeis (sailoi`s skin"),
constiuction woikeis, and lifeguaids; ten-
nis, swimming, and ski instiuctois; moun-
tain guides, spoitspeisons, and beach bums";
peisons who spend consideiable time in moun-
tain oi sea iesoits.
Ceoraphy DHe is moie seveie in white popu-
lations living in aieas with high solai UVR (at
high altitudes oi in low latitudes). Young white
childien (age 10) living in southein Boineo
(cool climate with high UVR) have been ob-
seived to have DHe, including solai keiatoses.
FAIh0CNSIS
While UVB is the most obvious damaging
UVR, UVA in high doses can pioduce connec-
tive tissue changes in mice. In addition, visible
(400-700 nm) and infiaied (1000-1,000,000
nm) iadiations have been implicated. The ac-
tion spectium foi DHe is not known foi ceitain;
theie is some expeiimental evidence in mice
that infiaied iadiation is implicated, in addition
to UVB and UVA.
Fersooa| hstory Theie is a histoiy of in-
tensive exposuie to sun in youth (<20 yeais),
even though sun exposuie may have been quite
limited in latei adult life, and/oi significant sun
exposuie in adulthood. Because skin photo-
types aie genetically deteimined, theie is often
a family histoiy of DHe.
Sko Iesoos A combination of atiophy
(of epideimis), hypeitiophy (of papillaiy
deimis due to elastosis), telangiectases, spotty
depigmentation and hypeipigmentation, and
spotty hypeikeiatosis in light-exposed aieas.
Skin appeais wiinkled, wizened, leatheiy,
piematuiely aged" (Fig. 10-20). Both
fine, cigaiette papei-like and deep fuiiow-
like wiinkling; skin is waxy, papulai with a
yellowish hue, and both glistening and iough
(Fig. 10-21). Theie may be telangiectasia
and biuising, Bateman oi senile puipuia
due to fiagility of small vessels. Maculai
hypeipigmentations: so|ar |engnes (see
below); maculai hypopigmentations; guae
|yome|anoss , <3 mm in diametei, on
the extiemities. Comedones, paiticulaily
peiioibital (teimed Fare-Ratout|e Jsease ),
paiticulaily in cigaiette smokeis. Individuals
with DHe invaiiably have actinic keiatoses.
Also, seboiiheic keiatoses that aie misdiagnosed
as lentigos.
DIstrIhutIvn Exposed aieas, paiticulaily
face, peiioibital and peiioial aieas, scalp (bald
males). Nuchal aiea: cutis ihomboidalis (ied
neck") with ihomboidal fuiiows; lowei aims,
doisa of hands. Pattein haii loss in both sexes,
although much less so in females.
FICk 10-20 0ermatohe|oss 'e.e|e deep W||u|||u. I|e ||u +ppe+| W+\,, p+pu|+| W||| + ,e||oW||
|ue (+c||u|c e|+|o|). I|| o3,e+|o|d |er+|e rouu|+|u |+|re| ||.ed +| +u +||||ude o| 000 r +ud |+d |eeu
Wo|||u ou|doo| +|| |e| |||e. I|e|e | |++| ce|| c+|c|uor+ |u ||e |e|| /,or+||c |e|ou.
FICk 10-21 Severe dermatohe|oss oo the Iorearm oI a T0-year-o|d Iema|e Iarmhaod I|e ||u
| W+\,, deep|, W||u||ed, +ud d|,. \u|||p|e o|+| |e|+|oe |+.e |eeu |ero.ed ||or ||| +|r |, c|,o||e|+p,.
0ermatopatho|oy Acanthosis of epideimis,
incieased hoiny layei. Flattening of the deimal-
epideimal junction. Atypia of the keiatinocytes.
Loss of small vessels in the papillaiy deimis.
E|asoss : Degiaded elastic tissue with
accumulation of coaise amoiphous masses and
inciease in glycosaminoglycans in the uppei
deimis. Deciease in collagen.
C0kS AN0 Fk0CN0SIS
The appeaiance of DHe maiks a ielatively
young peison as old," a state that eveiyone tiies
to delay. DHe is inexoiably piogiessive and ii-
ieveisible, but some iepaii of connective tissue
effects can occui if the skin is piotected. Some
piocesses leading to DHe continue to piogiess,
howevei, even when sun exposuies aie seveiely
iestiicted in latei life; solai keiatoses and len-
tigines develop in the sun-damaged skin that
is now being piotected by avoidance and sun-
blocks. Yet theie aie documented examples of
spontaneous ieveisal of solai keiatoses.
MANACMNI
Cuiient management is to pievent skin canceis
and the development of DHe with the use of
piotective sunblocks, a change of behavioi in
the sun, and the use of topical chemotheiapy
(tietinoin) that ieveises some of the changes
of DHe.
Iopca| Ireatmeot Trenon in lotions, gels,
and cieams in vaiying concentiations ieveises
some aspects of DHe, especially in the connec-
tive tissue and vasculai changes. Topical a:aro-
ene has also been shown to ieduce the effects
of photoaging in shoit-teim studies. Topical
tietinoin can altei the piogiession of incipient
epithelial skin canceis. 5-F|uorourat| in lotions
and cieams and imiquimod aie highly effective
in causing a disappeaiance of solai keiatoses.
Topical imiquimod impioves cosmetic appeai-
ance of photoaged skin: histology shows iepaii
of photodamage.
Freveotoo Peisons of SPT I and II should be
identified eaily in life and advised that they aie
susceptible to the development of DHe and skin
canceis, including melanoma. These peisons
should nevei sunbathe and should, fiom an eaily
age, adopt a daily piogiam of self-piotection
using sun-filteiing clothing and substantive and
effective topical sun-piotective solutions, gels,
oi lotions that can filtei DNA-damaging UVB;
effective UVA filteis aie now available. SPT I
and II peisons should avoid the peak houis of
UVB intensity, which aie the 2 h befoie and
aftei solai noon.
Cauon : Theie is some expeiimental evidence
that while sunscieens piotect fiom sunbuin,
they do not piotect fiom UV-induced local im-
munosuppiession. Pievention of sunbuin may
luie individuals into exposing themselves to the
sun foi piolonged peiiods, which may abiogate
immunosuiveillance mechanisms in the skin.
This has been linked to the iising incidence of
melanoma but is not pioven.
'o|+| |eu||o | + c||curc|||ed |o !cr
||oWu r+cu|e |eu|||u ||or + |oc+||/ed p|o|||
e|+||ou o| re|+uoc,|e due |o +cu|e o| c||ou|c
e\pou|e |o uu||||.
\u|||p|e |e|ou uu+||, +||e |u uue\poed
||e.
S0IAk INIIC0 |C|9 . 109.09
Ae oI 0oset Usually >40 yeais but may be
30 yeais in sunny climates and in susceptible
peisons.
kace Most common in Caucasians but seen
also in Asians.
Sko Fhototype Geneially coiielated with skin
phototypes I to III and duiation and intensity
of solai exposuie.
to|oy Solai lentigines may aiise acutely
aftei sunbuins and aftei oveidosage of PUVA
(PUV |engnes ).
Sko Iesoos Stiictly maculai, 1 to 3 cm, and
as laige as 5 cm. Light yellow, light biown,
oi daik biown; vaiiegated mix of biown and
not unifoim coloi (Figs. 10-22 and 10-23),
as in caf au lait macules. Round, oval, with
slightly iiiegulai boidei, ill defined (Fig. 10-23).
Scatteied, disciete lesions, stellate and shaiply
defined aftei acute sunbuin (Fig. 10-22).
DIstrIhutIvn Exclusively exposed aieas: foie-
head, cheeks, nose, doisa of hands and foie-
aims, uppei back, chest, shins.
FICk 10-22 0ermatohe|oss: so|ar |eotoes \u|||p|e |e||+|e ||oWu r+cu|e ou ||e |ou|de|
occu||ed +||e| + uu|u|u. I|e, +|e +|| o| +|ou| ||e +re |/e +ud |+|p|, r+||u+|ed, W||c| | c|+|+c|e||||c
o| uu|u|u|uduced o|+| |eu|||ue.
FICk 10-23 0ermatohe|oss: so|ar
|eotoes \u|||p|e, .+||e+|ed, |+u|od+||
||oWu r+cu|e ou ||e r+|+| +ud ||ou|+| +|e+
|u ||e |+ce. 'o|+| |eu|||ue +|e uo| ||e +re
+ ep|e||de (||ec||e)-||e, do uo| |+de |u
||e W|u|e| + ||ec||e do. |u cou||+| |o ||e
|+|p|, r+||u+|ed o|+| |eu|||ue |oWu |u
||. 022, W||c| +|e due |o +u +cu|e uu|u|u,
||e o|+| |eu|||ue |oWu |e|e +|e o| d|||e|eu|
|/e +ud p+|||+||, ||| de||ued +ud cou||ueu|,
W||c| | c|+|+c|e||||c o| c||ou|c curu|+||.e
o|+| d+r+e.
0ermatopatho|oy Club-shaped elongated
iete iidges that show hypeimelanosis and an
incieased numbei of melanocytes in the basal
layei.
8rowo Macu|es Flat," acquiied, biown lesions
on the exposed skin of the face, which may on
cuisoiy examination appeai to be similai, have
distinctive featuies: solai lentigo, fieckles, seb-
oiiheic keiatosis, spieading pigmented actinic
keiatosis (SPAK), lentigo maligna.
MANACMNI
Ciyosuigeiy oi lasei suigeiy aie effective. No
moie than 10 s of liquid nitiogen should
be administeied; otheiwise depigmentation of
noimal skin will occui.
uu+||, occu| + + |u|e e|ou+|ed, e\qu|||e|,
|eude| uodu|e o| + "|e+d|u" o| ||e ||ee |o|de|
o| |e||\ o| ||e e+|. Corrou, p|o|+||, due |o
cou|+u| rec|+u|c+| ||+ur+ +ud ro| p|o|+||,
|o u\ |+d|+||ou.
Appe+| pou|+ueou|,, eu|+|e qu|c||,, re+u|
|u |e ||+u cr (||. 02+), |||r, We||de||ued,
|ouud |o o.+| W||| |op|u r+||u.
E|||e| er|edded |u ||e ||u o| e|e.+|ed e.e|+|
r||||re|e| +ud W||| dore|+ped u||+ce, W|||e
W+\, +ud ||+u|uceu|, +ud o||eu u|ce|+|ed (||.
02+).
\o|e corrou |u r+|e ||+u |u |er+|e.
'pou|+ueou p+|u o| |eude|ue | ||e |u|||+| p|e
eu||u corp|+|u|. C+u |e |u|eue +ud |+|||u,
p+|o\,r+| o| cou||uuou.
||||e|eu||+| d|+uo| |uc|ude |++| ce|| +ud
qu+rou ce|| c+|c|uor+, +c||u|c |e|+|o|, |u ||u
o| |u.+|.e 'CC, |,pe|||op||c o|+| |e|+|o|, +ud
|e|+|o+c+u||or+. 0ue +|o |+ |o |||u| o| ou|,
|op|u, ||eur+|o|d +ud ||eur+||c uodu|e, +ud
d|co|d |upu e|,||er+|ou.
\+u+ereu| |uc|ude |u||+|e|ou+| |ujec||ou o|
|||+rc|uo|oue +ce|ou|de, c+||ou d|o\|de |+e|,
u|e|,. I|e de||u|||.e ||e+|reu| | e\c||ou+|
u|e|, |uc|ud|u ||e uude||,|u c+||||+e.
Ch0N0k00kMAIIIIS N00IAkIS hIICIS |C|9 . !30.0
|C|0 . no.0
FICk 10-24 Choodroder-
matts oodu|ars he|cs Au
e\||ere|, p+|u|u| uodu|e W|||
ceu||+| u|ce|+||ou ou ||e +u||e||\ o|
+ o0,e+|o|d |er+|e. I|e ceu||+|
u|ce| | co.e|ed W||| + c|u| +ud
c+u |e r||+|eu |o| + |++| ce||
c+|c|uor+.
I|ee |u|e o| ru|||p|e, d|c|e|e, d|,, |ou|, +d
|e|eu| c+|, |e|ou occu| ou ||e |+|||u+||, uu
e\poed ||u o| +du||, uu+||, ou + |+c||ouud
o| de|r+|o|e||o|.
Ac||u|c |e|+|oe c+u p|o|e |o qu+rou ce||
c+|c|uor+.
',, . 'o|+| |e|+|o|.
ACIINIC kkAI0SIS |C|9 . 102.0
|C|0 . |51.0
FI0MI0I0C
Ae oI 0oset Middle age, although in Aus-
tialia and southwestein United States solai
keiatoses may occui in peisons <30 yeais.
kace SPT I, II, and III; iaie in SPT IV; almost
nevei in blacks oi South Indians.
0ccupatoo Outdooi woikeis (especially faim-
eis, iancheis, sailois) and outdooi spoitspei-
sons (tennis, golf, mountain climbing, deep-sea
fishing).
FAIh0CNSIS
Piolonged and iepeated solai exposuie in sus-
ceptible peisons (SPT I, II, and III) leads to
cumulative damage to keiatinocytes by the ac-
tion of UVR, piincipally, if not exclusively, UVB
(290-320 nm).
0uratoo oI Iesoos (Fig. 10-25) Months to yeais.
Sko Symptoms Lesions may be tendei. On
examination, painful if excoiiated with a fingei-
nail; patient winces.
FICk 10-25 So|ar keratoses E|,||er+|ou r+cu|e +ud p+pu|e W||| co+|e, +d|e|eu| c+|e |ecore
cou||ueu| ou ||| |+|d c+|p W||| de|r+|o|e||o|. I|ee |,pe||e|+|oe +|e ,e||oW|||e,. I|e, +|e |e||e| |e||
||+u eeu, eu||, +||+d|u |e|ou W||| + ||ue|u+|| uu+||, |uduce p+|u, e.eu |u e+||, u|||e |e|ou, + |e|p|u|
d|+uo||c ||ud|u.
Sko Iesoos Adheient hypeikeiatotic scale,
which is iemoved with difficulty and pain (Figs.
10-25 and 10-26). May be papulai. Skin-coloied,
yellow-biown, oi biown-diity" (Fig. 10-26);
often theie is a ieddish tinge (Fig. 10-25). Rough,
like coaise sandpapei, bettei felt than seen" on
palpation with a fingei. Most commonly <1 cm,
oval oi iound (Fig. 10-27, B).
Seta| Presenaon : SPAK (spieading pig-
mented actinic keiatosis). This lesion is best
desciibed as looks like lentigo maligna but
feels like actinic keiatosis" (Fig. 10-28). It is a
iathei uncommon vaiiant of solai keiatosis.
The distinctive featuies of SPAK include size
(>1.5 cm), pigmentation (biown to black and
vaiiegated), and histoiy of sopui spieading,
especially the veiiucous suiface. The lesion is
impoitant because it can mimic lentigo ma-
ligna (LM). Keiatotic natuie of the lesion can
best be evaluated when the lesion is slightly
fiozen with LN2. Shaiply maiginated scale
is seen with solai and seboiiheic keiatoses.
Lentigos aie completely flat. It is, howevei,
easily distinguished fiom LM because LM is
completely flat without evidence of veiiucous
change. Biopsy is necessaiy to confiim the
clinical diagnosis.
DIstrIhutIvn Isolated single lesion oi scatteied
disciete lesions. Face foiehead, nose, cheeks
(Figs. 10-25 and 10-26), temples, veimilion
boidei of lowei lip], eais (in males), neck
(sides), foieaims, and hands (doisa), shins, and
the scalp in bald males (Fig. 10-25). Males with
eaily pattein alopecia aie especially pione to
seveie deimatoheliosis and solai keiatosis on
the exposed scalp.
0ermatopatho|oy Laige biight-staining ke-
iatinocytes, with mild to modeiate pleomoi-
phism in the basal layei extending into follicles,
atypical (dyskeiatotic) keiatinocytes, paiakeia-
tosis.
Usually made on clinical findings. Diffeien-
tial: Chionic cutaneous lupus eiythematosus;
seboiiheic keiatosis, flat waits, squamous cell
caicinoma (SCC) in situ, supeificial basal cell
caicinoma. Highly hypeikeiatotic lesions and
SPAK may iequiie biopsy to iule out SCC (in
situ oi invasive) oi LM.
C0kS AN0 Fk0CN0SIS
Solai keiatoses may disappeai spontaneously, but
in geneial iemain foi yeais. The actual incidence
of SCC aiising in pieexisting solai keiatoses is
unknown but has been estimated at one SCC
developing annually in 1000 solai keiatoses.
MANACMNI
Freveotoo Avoided by use of highly effective
UVB/UVA sunscieens, which should be applied
daily to the face, neck, and eais duiing the sum-
mei in noithein latitudes foi SPT I and SPT
II peisons and foi those SPT III peisons who
sustain piolonged sunlight exposuies.
Iopca| Iherapy Cryvsurgery Light spiay oi
with cotton-tipped applicatoi is effective in
most cases.
3-F|uvrvurucI| (3-FU) Creum 3% Effective,
but difficult foi many individuals. Tieatment
of facial lesions causes significant eiythema
and eiosions, iesulting in tempoiaiy cosmetic
disfiguiement. Apply twice daily foi 2 to 4 weeks
on face; may iequiie longei peiiod of theiapy
on doisum of hands oi lowei legs. Efficacy can
be incieased and duiation of tieatment can be
shoitened if applied undei occlusion and/oi
combined with topical tietinoin. This, howevei,
leads to confluent eiosions and may iequiie
hospitalization. Reepithelialization occuis aftei
tieatment is discontinued. Pietieatment with
light ciyosuigeiy to hypeikeiatotic lesions may
impiove efficacy of 5-FU cieam.
1mIquImvd (twIce wee||y ]vr 16 wee|s)
Causes cytokine deimatitis, also leads to iiiita-
tion and eiosions but is highly effective.
TvpIcu| RetInvIds Used chionically, is effec-
tive foi tieatment of deimatoheliosis and supei-
ficial solai keiatoses.
DIc|v]enuc Ge| Used chionically, is effective
in supeificial acting keiatoses; also iiiitating.
FucIu| Pee|s Tiichloioacetic acid (5-10%) ef-
fective foi widespiead lesions.
Luser Surgery Eibium oi caibon dioxide la-
seis. Usually effective foi individual lesions.
Foi extensive facial lesions, facial iesuifacing
is effective.
PhvtvdynumIc Therupy Effective but painful
and cumbeisome.
Systemc Iherapy Acitietin oi isotietinoin aie
effective in ieducing the numbei of solai keia-
toses and SCC in situ in patients with advanced
deimatoheliosis, especially in immunocompio-
mised patients. Lesions iecui once theiapy is
discontinued.
FICk 10-26 Actoc keratoses, c|ose up \e||oW|e,|| |||||, +d|e|eu| c+|e ou ||e |o|e|e+d o| +u
30,e+|o|d r+u. A||+d|u ||ee |,pe||e|+|oe | p+|u|u|. I|e|e | + r+|| |++| ce|| c+|c|uor+ +| ||e |o|de| o|
||e |+||, c+|p.
FICk 10-2T So|ar keratoses, hher maoIcatoo A |+|p|, de||ued ,e||oW||oWu|| |||||,
+d|e|eu|, |ou| |,pe||e|+|o|. I|| |e|ou | e.eu ro|e e|e.+|ed +ud |+ + '|uc|ou +ppe+|+uce |||e + e|
o|||e|c |e|+|o|. noWe.e|, || | uo| |e+, +ud o|| |u| |+||e| |+|d, |ou|, +ud p+|u|u| W|eu c|+ped.
8
SkIN kACII0NS I0 I0NIIINC kA0IAII0N
k+d|+||ou de|r+|||| | de||ued + ||u c|+ue
|eu|||u ||or e\pou|e |o |ou|/|u |+d|+||ou.
|-.-||- -||-:| +|e p+|u, e|,||er+, ep||+||ou,
upp|e|ou o| e|+ceou |+ud, +ud p|reu|+
||ou (|+||u |o| Wee| |o rou|| |o ,e+|).
|-.-||- -||-:| +|e +||op|,, c|e|o|,
|e|+u|ec|+|+, u|ce|+||ou, +ud |+d|+||ou|uduced
c+uce|.
kA0IAII0N 0kMAIIIIS |C|9 . o92.32
|C|0 . |53
Iype oI xposure
Result of theiapy (foi cancei, foimeily also
used foi acne and psoiiasis, and fungal infec-
tions of the scalp in childien), accidental, oi
occupational (e.g., foimeily, in dentists who
held the film in the mouth with theii fingeis).
The iadiation causing iadiodeimatitis includes
supeificial and deep x-iay iadiation, election-
beam theiapy, and gienz-iay theiapy. It is a
pievailing myth among some deimatologists
that gienz iays aie soft" and not caicinogenic;
it has been estimated that SCC can appeai fiom
>5000 cGy of gienz iays.
Iypes oI keactoos
Acute Tempoiaiy eiythema that lasts 3 days
and then peisistent eiythema, which ieaches a
peak in 2 weeks and is painful; pigmentation
appeais about day 20; a late eiythema can also
occui beginning on day 35-40, and this lasts
2-3 weeks. Massive ieactions lead to blisteiing
eiosions (Fig. 10-29) and ulceiation, also pain-
ful; may occui as iecall phenomenon. Peima-
nent scaiiing may iesult.
Chrooc Aftei [ratona| but ielatively intensive
theiapy with total doses of 3000-6000 iad, theie
develops an epideimolytic ieaction in 3 weeks.
This is iepaiied in 3-6 weeks, and scais and hy-
popigmentation develop; theie is loss of all skin
appendages and atiophy of the epideimis and
deimis. Duiing the next 2-5 yeais, the atiophy
incieases (Fig. 10-30); theie is hypei- and hy-
popigmentation (poikilodeima), telangiectasia
(Figs. 10-30 and 10-31), and supeificial venules
FICk 10-28 Spreado pmeoted actoc keratoss (SFAk) '|oo| |||e |eu||o r+||u+ (ee ||. 21) |u|
| |ou| +ud ||e|e|o|e '|ee| |||e +c||u|c |e|+|o|. A uoup|reu|ed +c||u|c |e|+|o| | eeu |u ||e p|e+u||cu|+| |e|ou.
SCII0N 10 |n0I0'E|'|I|\|I\, |n0I0|||uCE| ||'0k|Ek', A|| ||'0k|Ek' B\ |0||/||C kA||AI|0| 2T1
become ectatic. Theie aie hypeikeiatoses (x-iay
keiatoses) (Fig. 10-34 ). Neciosis and painful
ulceiation (Fig. 10-32) aie iaie but occui in ac-
cidental exposuie oi eiioi in dose: eithei one oi
a few accidentally high doses oi multiple small
doses at fiequent inteivals (monthly oi weekly).
When neciosis occuis, it is leatheiy, yellow, and
adheient and the base and suiiounding skin
aie extiemely painful (Fig. 10-32). Ulceiations
have a veiy pooi tendency to heal and usually
iequiie suigical inteivention. Accidental expo-
suie occuis mostly in occupational exposuie
and affects the hands, feet, and face. Theie is a
destiuction of the fingeipiint pattein, xeiosis,
scanty haii, atiophy of sebaceous and sweat
glands, and development of keiatoses (Fig.
10-34 ).
Na|s Longitudinal stiiations (Fig. 10-34 B )
show thickening, dystiophy. Diclofenac used
chionically is effective in keiatoses.
C0kS, Fk0CN0SIS, AN0 MANACMNI
Chionic iadiation deimatitis is peimanent,
piogiessive, and iiieveisible. SCC may develop
in 4-40 yeais (Figs. 10-33 and 10-34 , B),
with a median of 7-12 yeais, almost exclu-
sively fiom the chionic iepeated types of ex-
posuies. SCC always develops within the aiea
of iadiodeimatitis, (Fig. 10-34, B). Tumois
metastasize in about 25%; despite extensive
suigeiy (excision, giafts, etc.), the piognosis is
pooi, and iecuiiences aie common. Basal cell
caicinoma (BCC) may also occui in chionic
iadiation deimatitis and appeais mostly in
patients foimeily tieated with x-iays foi acne
vulgaiis and acne cystica oi epilation (tinea
capitis) (Fig. 10-31). The tumois may ap-
peai 40-50 yeais aftei exposuie. Excision and
giafting aie often possible befoie the cancei
develops.
FICk 10-29 kadatoo dermatts: acute, reca|| pheoomeooo I|| p+||eu| |+d ||e+| c+uce|. '|e |+d
+ |urpec|or,, re||o||e\+|e, +ud \|+, ||e|+p, +ud de.e|oped p+|u|u| e|,||er+ +ud e|o|ou +| ||e |||+d|+|ed ||e.
FICk 10-30 kadatoo dermatts: chrooc I|e|e | c|e|o| cor||ued W||| +||op|, +ud |e|+u|ec|+|+.
I|| | ||e |eu|| o| ||e |||+d|+||ou o| +u |u|+u|||e |er+u|or+ |u |u|+uc,.
FICk 10-31 kadatoo dermatts: chrooc I|e|e | po||||ode|r+ (||oWu. |,pe|p|reu|+||ou, W|||e.
|,pop|reu|+||ou, |ed. |e|+u|ec|+|+) cor||ued W||| +||op|, +ud c|e|o|. n+|| +|e +|eu|. I|ee r+|.e ||u
c|+ue +|e ||e |eu|| o| o.e|doed |||+d|+||ou ||e p+||eu| |ece|.ed + + c|||d |o| |uu+| |u|ec||ou o| ||e c+|p. ne
| + c+ud|d+|e |o| 'CC |u ||e |u|u|e.
SCII0N 10 |n0I0'E|'|I|\|I\, |n0I0|||uCE| ||'0k|Ek', A|| ||'0k|Ek' B\ |0||/||C kA||AI|0| 2T3
FICk 10-32 kadatoo dermatts: chrooc Au
+|e+ o| e.e|e |e|+u|ec|+|+ W||| + ceu||+| uec|o| ||+|
| |e+||e|,, ,e||oW||W|||e, +ud |||||, +d|e|eu|. 'u||c+|
|ero.+| W||| |e.e+| + deep u|ce|. I|e |e|ou | e\||ere|,
p+|u|u|.
FICk 10-33 kadatoo dermatts: chrooc wth
squamous ce|| carcooma A |+|e |||||, e|e.+|ed
u|ce| |u +u +|e+ o| +||op|,, ||||o|, po||||ode|r+, +ud
|e|+u|ec|+|+ ou ||e c|e| W+||. I|| occu||ed 20 ,e+|
+||e| |+d|c+| r+|ec|or,, +\|||+|, |,rp| uode d|ec||ou,
+ud |+d|o||e|+p,. I|e u|ce|+||ou W+ p||r+|||, due |o
|+d|ouec|o|. |oW ||e |o|de| o| ||e u|ce| | e|e.+|ed
+ud |||r. ||| | 'CC +|||u |u ||| |+d|+||ou de|r+||||.
FICk 10-34 kadatoo-oduced squamous ce|| carcooma I|ee +|e ||e |+ud o| +u e|de||,
|+d|o|o|| W|o dec+de +o |+d d||e+|ded p|ec+u||ou+|, re+u|e +ud |+|d|, Wo|e |o.e do|u ||uo|ocop|c
Wo||. I|e|e +|e ru|||p|e \|+, |e|+|oe, ||e |,pe||e|+|o||c |e|ou ou ||e |||| ||ur| |+ de||o,ed ||e u+|| +ud
|ep|eeu| \|+,|uduced 'CC. |+|| c|+ue |u ||e o| |+d|+||ou e\pou|e. |o|e ||e ||ue+| |||+||ou |eu|||u
||or d+r+e |o ||e u+|| r+|||\. A| ||e u+|||o|d +ud e\|eud|u p|o\|r+||, ou ||e ||ur|, ||e|e | +u |||eu|+| e|,
||er+|ou p|+que ||+| |ep|eeu| ro||, 'CC |u ||u |u|, |oc+||,, +|o |u.+|.e 'CC.
8
8
2T4
S E C I | 0 N 1 1
FkCANCk0S ISI0NS
AN0 CIAN0S CAkCIN0MAS
Cu|+ueou ep|||e||+| c+uce| uoure|+uor+
||u c+uce| (|\'C)| +|e ||e e+|e| o| +||
c+uce| |o d|+uoe +ud ||e+|. I|e, o|||u+|e
ro| corrou|, |u ||e ep|de|r+| e|r|u+||.e
|e|+||uoc,|e o| +due\+| ||uc|u|e (e.., We+|
+pp+|+|u, |+|| |o|||c|e). I|e |Wo p||uc|p+|
|\'C +|e |++| ce|| c+|c|uor+ (BCC) +ud
qu+rou ce|| c+|c|uor+ ('CC). 'CC o||eu
|+ || o|||u |u +u |deu||||+||e d,p|+||c |u ||u
|e|ou ||+| c+u |e ||e+|ed |e|o|e ||+u| |u.+|ou
occu|. |u cou||+|, |u ||u BCC | uo| |uoWu,
|u| r|u|r+||, |u.+|.e 'upe|||c|+| BCC +|e
corrou.
I|e ro| corrou e||o|o, o| |\'C |u
|+||||uued |ud|.|du+| | uu||||, u|||+.|o|e|
|+d|+||ou (u\k), +ud |ur+u p+p|||or+.||u
(n|\). 'o|+| |e|+|oe +|e ||e ro| corrou
p|ecu|o| |e|ou o| 'CC |u ||u ('CC|') +ud
|u.+|.e 'CC occu|||u +| ||e o| c||ou|c
uu e\pou|e |u |ud|.|du+| o| uo|||e|u Eu
|ope+u |e|||+e (ee 'ec||ou 0). u\k +ud
n|\ c+ue ||e pec||ur o| c|+ue |+u|u
||or ep|||e||+| d,p|+|+ |o 'CC|' |o |u.+|.e
'CC. \uc| |e corrou|,, |\'C c+u |e
c+ued |, |ou|/|u |+d|+||ou (+|||u |u ||e
o| c||ou|c |+d|+||ou d+r+e), c||ou|c |u||+r
r+||ou, |,d|oc+||ou (|+|), +ud c||ou|c |ue
||ou o| |uo|+u|c +|eu|c, ||ee |uro| c+u |e
ruc| ro|e +|e|.e ||+u ||oe +oc|+|ed
W||| u\k o| n|\. |u ||e |uc|e+|u popu|+
||ou o| |rruuoupp|eed |ud|.|du+| (||oe
W||| n|\/A||' d|e+e, o||d o|+u ||+up|+u|
|ec|p|eu|, e|c.), u\k +ud n|\|uduced 'CC
+|e ruc| ro|e corrou +ud c+u |e ro|e
+|e|.e.
FI0kMAI FkCANCkS AN0 CANCkS
P|ThL|AL P80Ah0800S LS|0hS
Ah0 S00|S
Dysplasia of epideimal keiatinocytes in epi-
deimis and squamous mucosa can involve the
lowei poition of the epideimis oi the full
thickness. Basal cells matuie into dysplastic ke-
iatinocytes iesulting in a hypeikeiatotic papule,
oi plaque, clinically identified as keiatosis."
A continuum exists fiom dysplasia to SCCIS
to invasive SCC. These lesions have vaiious
associated eponyms such as Bowen disease oi
eiythioplasia of Queyiat, which as desciiptive
moiphologic teims aie helpful; teims such as
UVR- oi HPV-associated SCCIS, howevei, will
be moie meaningful but can be used only foi
those lesions with known etiology.
Ep|||e||+| p|ec+uce|ou |e|ou +ud 'CC|' c+u |e
c|+|||ed + |o||oW.
u\k|uduced
'o|+| (+c||u|c) |e|+|oe
'p|e+d|u p|reu|ed +c||u|c |e|+|oe
('|AK)
||c|euo|d +c||u|c |e|+|oe
BoWeuo|d +c||u|c |e|+|oe
'CC|' (BoWeu d|e+e)
n|\|uduced
|oW|+de qu+rou |u||+ep|||e||+| |e|ou
(|'||)
n|||+de qu+rou |u||+ep|||e||+| |e|ou
(n'||)
'CC|' (BoWeuo|d p+pu|o|)
A|eu|c+| |e|+|oe
|+|rop|+u|+| |e|+|oe
BoWeuo|d +|eu|c+| |e|+|oe
n,d|oc+||ou (|+|) |e|+|oe
I|e|r+| |e|+|oe
Ke|+|oe |u c||ou|c |+d|+||ou de|r+||||
C||ou|c c|c+|||\ (c+|) |e|+|oe
SCII0N 11 |kECA|CEk0u' |E'|0|' A|| CuIA|E0u' CAkC||0\A' 2T5
S0LA8 08 A0T|h|0 k8AT0S|S
These single oi multiple, disciete, diy, iough,
adheient scaly lesions occui on the habitually
sun-exposed skin of adults. They can piogiess
A cu|+ueou |o|u (Cn) | + :|:c| eu|||, |+.|u
||e +ppe+|+uce o| +u +u|r+| |o|u W||| + p+pu|+|
o| uodu|+| |+e +ud + |e|+|o||c c+p o| .+||ou
|+pe +ud |eu|| (||. 2).
Cn ro| corrou|, |ep|eeu| |,pe|||op||c o|+|
|e|+|oe. noWe.e|, |u ||u o| |u.+|.e 'CC |
o||eu p|eeu| +| ||e |+e o| + Cn.
Cn uu+||, +||e W||||u +|e+ o| de|r+|o|e||o|
ou ||e |+ce, e+|, do|ur o| |+ud, o| |o|e+|r,
+ud ||u.
|oup|ec+uce|ou Cn |o|r+||ou c+u +|o occu| |u
e|o|||e|c |e|+|oe, W+||, +ud |e|+|o+c+u||o
r+.
C||u|c+||,, Cn .+|, |u |/e ||or + |eW r||||re|e|
|o e.e|+| ceu||re|e| (||. 2). I|e |o|u r+,
|e W|||e, ||+c|, o| ,e||oW|| |u co|o| +ud ||+|||,
cu|.ed, o| p||+| |u |+pe.
n||o|o|c+||, ||e|e | uu+||, |,pe|||op||c +c||u|c
|e|+|o|, 'CC|' o| |u.+|.e 'CC +| ||e |+e.
Bec+ue o| ||e po||||||, o| |u.+|.e 'CC, + Cn
|ou|d +|W+, |e e\c|ed.
CIAN0S h0kN |C|9 . 102.2
|C|0 . |35.3
to SCCIS, which can then piogiess to invasive
SCC. (Fig. 11-1).
Synonym : Solai and actinic keiatosis aie syn-
onymous.
Foi a full discussion of this condition, see Sec-
tion 10, p. 267.
FICk 11-1 So|ar keratoses aod ovasve squamous ce|| carcooma \u|||p|e, |||||, +d|e|eu| d|||,
|oo||u o|+| |e|+|oe (ee +|o ||. 025 |o 021). I|e |+|e uodu|e |oWu |e|e | co.e|ed |, |,pe||e|+|oe
+ud |ero|||+|c c|u|, || | p+|||+||, e|oded +ud |||r. I|| uodu|e | |u.+|.e qu+rou ce|| c+|c|uor+. I|e
|r+e | |oWu |o derou||+|e ||e ||+u|||ou ||or p|ec+uce|ou |e|ou |o ||+u| c+|c|uor+.
Appe+| dec+de +||e| c||ou|c +|eu|c |ue||ou
(red|c|u+|, occup+||ou+|, o| eu.||oureu|+| e\po
u|e).
A|eu|c+| |e|+|oe |+.e ||e po|eu||+| |o |ecore
'CC|' o| |u.+|.e 'CC. I|ee +|e cu||eu||, |e|u
eeu |u we| Beu+| +ud B+u|+de|.
IWo |,pe. puuc|+|e, ,e||oW p+pu|e ou p+|r
+ud o|e (||. ! 1 ), |e|+|oe |ud|||uu||+||e
||or +c||u|c |e|+|oe ou ||e ||uu| +ud e|e
W|e|e. I|ee +|e o||eu +oc|+|ed W||| r+||
'CC|' o| ||e BoWeu|,pe +ud |,pop|reu|ed
|||||, dep|eed r+cu|e ('|+|ud|op |u ||e
du|) (||. ! 3 ).
I|e+|reu|-+ |o| o|+| |e|+|oe.
AkSNICAI kkAI0SS |C|9 . o92.+
|C|0 . |35.3
||eeu| + o|||+|, o| ru|||p|e r+cu|e, p+pu|e,
o| p|+que, W||c| r+, |e |,pe||e|+|o||c o|
c+||u.
'CC|' | ro| o||eu c+ued |, u\k o| n|\
|u|ec||ou.
Corrou|, +||e |u ep|||e||+| d,p|+||c |e|ou
uc| + o|+| |e|+|oe o| n|\|uduced qu+
rou ep|||e||+| |e|ou ('||) (ee 'ec||ou 21
+ud !0).
||u| o| |ed, |+|p|, de||ued c+|, p|+que ou ||e
||u +|e c+||ed 3+- !-c- , |r||+| |u| uu+||,
uouc+|, |e|ou ou ||e |+u +ud .u|.+ +|e c+||ed
-,|||cc .
Auoeu||+| n|\|uduced 'CC|' | |e|e||ed |o +
|+-! c| .
uu||e+|ed ''C|' r+, p|o|e |o |u.+|.e 'CC.
w||| n|\|uduced 'CC|' |u n|\/A||', |e|ou
o||eu |eo|.e corp|e|e|, W||| ucce|u| AkI +ud
|rruue |ecou|||u||ou.
I|e+|reu| | |op|c+| 5||uo|ou|+c||, |r|qu|rod,
c|,ou|e|,, C0
2
|+e| e.+po|+||ou, o| e\c||ou,
|uc|ud|u \o| r|c|o|+p||c u|e|,.
SAM0S CII CAkCIN0MA IN SII |C|9 . 1!.0
|C|0 . \3010/2
FICk 11-2 Cutaoeous horo: hypertrophc actoc keratoss A |o|u|||e p|ojec||ou o| |e|+||u ou +
|||||, |+|ed |+e |u ||e e|||u o| +d.+uced de|r+|o|e||o| ou ||e uppe| e,e||d |u +u 35,e+|o|d |er+|e. E\c|
|ou |oWed |u.+|.e 'CC +| ||e |+e o| ||e |e|ou.
SCII0N 11 |kECA|CEk0u' |E'|0|' A|| CuIA|E0u' CAkC||0\A' 2TT
FICk 11-3 Arseoca| keratoses \u|||p|e puuc|+|e, |||||, +d|e|eu| +ud .e|, |+|d |e|+|oe ou ||e
p+|r. A|eu|c+| |e|+|oe ou ||e |+c|. \u|||p|e |e|ou +|e eeu |e|e |+u|u ||or |ed |o |+u, d+|| ||oWu, +ud
W|||e. I|e ||oWu |e|ou +|e + r|\ o| +|eu|c+| |e|+|oe (|+|d, |ou|) +ud r+|| e|o|||e|c |e|+|oe (o|| +ud
roo||). I|e d|||e|euce c+u |e |e||e| |e|| ||+u eeu. I|e |ed |e|ou +|e r+|| BoWeuo|d |e|+|oe +ud BoWeu
d|e+e (ee ||. +). I|e W|||e r+cu|+| +|e+ +|e |||||, dep|eed +ud |ep|eeu| upe|||c|+| +||op||c c+|
||or pou|+ueou|, |ed o| ||e+|ed +|eu|c+| |e|+|oe. I|e eu|||e p|c|u|e |.e ||e |rp|e|ou o| '|+|u d|op |u
||e du|.

II0I0C
UVR, HPV, aisenic, tai, chionic heat exposuie,
chionic iadiation deimatitis.
Lesions aie most often asymptomatic but may
bleed. Nodule foimation oi onset of pain oi
tendeiness within SCCIS suggests piogiession
to invasive SCC.
Sko Fodos Appeais as a shaiply demaicated,
scaling, oi hypeikeiatotic macule, papule, oi
plaque (Fig. 11-4). Solitaiy oi multiple lesions
aie pink oi ied in coloi and have a slightly
scaling suiface, small eiosions, and can be
ciusted. Such lesions aie always well-defined
and aie called Bowen Jsease (Fig. 11-4).
Red, shaiply demaicated, glistening maculai
oi plaque-like SCCIS on the glans penis oi
labia minoia aie called ery|ro|asa o[ Queyra
(see Section 35). Anogenital HPV-induced SC-
CIS may be ied, tan, biown, oi black in coloi
and aie iefeiied to as |owenoJ au|oss (see
Section 35). Eioded lesions may have aieas of
ciusting. SCCIS may be mistaken foi a patch
of eczema oi psoiiasis and go undiagnosed foi
yeais, iesulting in laige lesions with annulai oi
polycyclic boideis (Fig. 11-
5). Once invasion occuis,
nodulai lesions appeai
within the plaque and the
lesion is then commonly
called Bowen tartnoma
(Fig. 11-5).
DIstrIhutIvn UVR-in-
duced SCCIS commonly
aiises within a solai keia-
tosis in the setting of pho-
toaging (deimatoheliosis).
HPV-induced SCCIS aiises
within an aiea of low-giade
oi high-giade SIL, mostly in
the genital aiea but also pei-
iungually, most commonly
on the thumb oi in the nail
bed (see Fig. 33-15) (Image
11-1).
0ermatopatho|oy Caicinoma in situ with
loss of epideimal aichitectuie and iegulai dif-
feientiation; keiatinocyte polymoiphism, single
cell dyskeiatosis, incieased mitotic iate, multi-
nucleai cells. Epideimis may be thickened but
basement membiane intact.
Clinical diagnosis confiimed by deimatopatho-
logic findings. Diffeiential diagnosis includes all
well-demaicated pink-ied plaque(s): Nummu-
lai eczema, psoiiasis, seboiiheic keiatosis, solai
keiatoses, veiiuca vulgaiis, veiiuca plana, con-
dyloma acuminatum, supeificial BCC; amelan-
otic melanoma, Paget disease.
C0kS AN0 Fk0CN0SIS
Untieated SCCIS will piogiess to invasive SCC
(Fig. 11-5). In HIV/AIDS, iesolves with suc-
cessful ART. Lymph node metastasis can occui
without demonstiable invasion. Metastatic dis-
semination fiom lymph nodes.
IMAC 11-1 Squamous ce||
carcooma: p|ed||ec||ou ||e.
SCII0N 11 |kECA|CEk0u' |E'|0|' A|| CuIA|E0u' CAkC||0\A' 2T9
MANACMNI
Iopca| Chemotherapy 5-F|uorourat| cieam
applied eveiy day oi twice daily, with oi without
tape occlusion, is effective. So is mqumoJ, but
both iequiie consideiable time.
Cryosurery Highly effective. Lesions aie usu-
ally tieated moie aggiessively than solai keia-
toses, and supeificial scaiiing will iesult.
Fhotodyoamc Iherapy Effective but still cum-
beisome and painful.
Surca| xcsoo Has the highest cuie iate
but the gieatest chance of causing cosmetically
disfiguiing scais. It should be done in all lesions
wheie invasion cannot be excluded by biopsy.
FICk 11-4 Squamous ce|| carcooma o stu: 8oweo dsease A |+|e, |+|p|, der+|c+|ed, c+|,,
e|,||er+|ou p|+que |ru|+||u + po||+||c |e|ou. A |r||+| po||+||o|r p|+que W||| + r|\ o| c+|e, |,pe|
|e|+|o|, +ud |ero|||+|c c|u| ou ||e u||+ce.

FICk 11-5 Squamous ce|| carcooma o stu: 8oweo dsease aod ovasve SCC: 8oweo carcooma
A |+|e .+||e+|ed o|+ue, ||oWu |o |+, p|+que ou ||e |+c|, |+|p|, de||ued, W||| |||eu|+| ou|||ue |ep|eeu|
'CC|', o| BoWeu d|e+e. I|e |ed uodu|e ou ||| p|+que |ud|c+|e ||+| |e|e ||e |e|ou | uo| +u, ro|e +u |u ||u
|e|ou |u| ||+| |u.+|.e c+|c|uor+ |+ de.e|oped. || | +u uud|||e|eu||+|ed c+|c|uor+.
'CC o| ||e ||u | + r+||u+u| |uro| o| |e|+||uo
c,|e, +|||u |u ||e ep|de|r|.
'CC uu+||, +||e |u ep|de|r+| p|ec+uce|ou
|e|ou (ee +|o.e) +ud, depeud|u ou e||o|o,
+ud |e.e| o| d|||e|eu||+||ou, .+||e |u || +|e|.e
ue.
I|e |e|ou | + p|+que o| + uodu|e W||| .+|,|u
de|ee o| |e|+||u|/+||ou |u ||e uodu|e +ud/o| ou
||e u||+ce. I|ur| |u|e. uud|||e|eu||+|ed 'CC |
o|| +ud |+ uo |,pe||e|+|o|, d|||e|eu||+|ed 'CC
| |+|d ou p+|p+||ou +ud |+ |,pe||e|+|o|.
I|e r+jo|||, o| u\k|uduced |e|ou |+.e + |oW
|+|e o| d||+u| re|+|+| |u o||e|W|e |e+|||,
|ud|.|du+|. \o|e +|e|.e 'CC occu| |u |rru
uoupp|eed |ud|.|du+| W||| + |e+|e| |uc|deuce
o| re|+|+|.
I|e+|reu| | |, u|e|,.
|C|9 . 1!.0
|C|0 . \301o/2!
INvASIv SAM0S CII CAkCIN0MA (SCC)
|travo|et kadatoo
Ae oI 0oset Oldei than 55 yeais of age in
the United States; in Austialia, New Zealand,
in Floiida, Southwest and Southein Califoinia
peisons in theii twenties and thiities.
Iocdeoce Continental United States: 12 pei
100,000 white males; 7 pei 100,000 white fe-
males. Hawaii: 62 pei 100,000 whites.
Sex Males > females, but SCC can occui moie
fiequently on the legs of females.
xposure Sunlight. Phototheiapy, PUVA (oial
psoialen - UVA). Excessive photochemotheiapy
can lead to piomotion of SCC, paiticulaily in pa-
tients with skin phototypes I and II oi in patients
with histoiy of pievious exposuie to ionizing ia-
diation oi methotiexate tieatment foi psoiiasis.
kace Peisons with white skin and pooi tan-
ning capacity (skin phototypes I and II) (see
Section 10). Biown- oi black-skinned peisons
can develop SCC fiom numeious etiologic
agents othei than UVR.
Ceoraphy Most common in aieas that have
many days of sunshine annually, i.e., in Aus-
tialia and southwestein United States.
0ccupatoo Peisons woiking outdoois-faim-
eis, sailois, lifeguaids, telephone line installeis,
constiuction woikeis, dock woikeis.
humao Fap||omavrus
Oncogenic HPV type-16, -18, -31 most com-
monly, -33, -35, -39, -40, and -51 to -60 aie
associated with epithelial dysplasia, SCCIS, and
invasive SCC. HPV-5, -8, -9 have also been iso-
lated fiom SCCs.
0ther to|oc Factors
Immuoosuppressoo Solid oigan tiansplant
iecipients, individuals with chionic immuno-
suppiession of inflammatoiy disoideis, and
those with HIV disease aie associated with an
incieased incidence of UVR- and HPV-induced
SCCIS and invasive SCCs. SCCs in these indi-
viduals aie moie aggiessive than in nonimmu-
nosuppiessed individuals.
Chrooc IoI|ammatoo Chionic cutaneous lu-
pus eiythematosus, chionic ulceis, buin scais,
chionic iadiation deimatitis, lichen planus of
oial mucosa.
Iodustra| Carcooeos Pitch, tai, ciude paiaf-
fin oil, fuel oil, cieosote, lubiicating oil, nitio-
souieas.
Iooraoc Arseoc Tiivalent aisenic had been
used in the past in medications such as Asiatic
pills, Donovan pills, Fowlei solution (used as a
tieatment foi psoiiasis). Histoiically tiivalent ai-
senic was used foi tieatment of psoiiasis. Aisenic
is still piesent in diinking watei in some geo-
giaphic iegions (West Bengal and Bangladesh).
Slowly evolving-any isolated keiatotic oi
eioded papule oi plaque in a suspect patient
that peisists foi ovei a month is consideied a
caicinoma until pioved otheiwise. Also, a nod-
ule evolving in a plaque that meets the clinical
ciiteiia of SCCIS (Bowen disease), a chionically
eioded lesion on the lowei lip oi on the penis,
oi nodulai lesions evolving in oi at the maigin
of a chionic venous ulcei oi within chionic
iadiation deimatitis. Note that SCC is always
asymptomatic. Potential caicinogens often can
be detected only aftei detailed inteiiogation of
the patient.
Rapidly evolving-invasive SCC can eiupt
within a few weeks and is often painful and/oi
tendei.
Foi didactic ieasons, two types can be distin-
guished:
SCII0N 11 |kECA|CEk0u' |E'|0|' A|| CuIA|E0u' CAkC||0\A' 281
1. Highly diffeientiated SCCs, which piactically
always show signs of keiatinization eithei
within oi on the suiface (hypeikeiatosis)
of the tumoi. These aie fiim oi haid upon
palpation (Figs. 11-6 to 11-8 and Figs. 11-10
to 11-12).
2. Pooily diffeientiated SCCs, which do not
show signs of keiatinization and clinically
appeai fleshy, gianulomatous, amd conse-
quently aie soft upon palpation (Figs. 11-5
and 11-9).
FICk 11-T Squamous ce|| carcooma A |ouud uodu|e, |||r +ud |udo|eu| W||| + ceu||+| ||+c| ec|+|.
|o|e ,e||oW|| co|o| |u ||e pe||p|e|, o| ||e |uro| |ud|c+||u ||e p|eeuce o| |e|+||u. A|| |||ee 'CC |oWu |u ||.
o +ud |e|e +|e |+|d +ud occu| ou ||e |oWe| ||p. 'CC |+|d|, occu| ou ||e uppe| ||p |ec+ue ||| | |+ded
||or ||e uu. 'CC ou ||e ||p | e+||, d|||uu||ed ||or uodu|+| BCC |ec+ue BCC doe uo| de.e|op |,pe||e|+
|o| o| |e|+|o| |u|de ||e |uro| +ud doe uo| occu| ou ||e .e|r|||ou ||p.
FICk 11-6 Squamous ce|| carcooma: ovasve oo the |p, two staes oI deve|opmeot A |+|e
|u| u|||e uodu|e, W||c| | |e||e| |e|| ||+u eeu, ou ||e .e|r|||ou |o|de| o| ||e |oWe| ||p W||| +|e+ o| |,pe||e|+
|o| +ud e|o|ou, +|||u |u ||e e|||u o| de|r+|o|e||o| o| ||e ||p (c|e||||| +c||u|c+). I|| uodu|e | |+|e| +ud
c+u |e |e|| |o |u|||||+|e ||e eu|||e ||p.

0IIereotated SCC
Iesoos Induiated papule, plaque, oi nod-
ule (Figs. 11-1, 11-6 to 11-8); adheient thick
keiatotic scale oi hypeikeiatosis (Figs. 11-1,
11-6, 11-7, 11-8, 11-11); when eioded oi ulcei-
ated, the lesion may have a ciust in the centei
and a fiim, hypeikeiatotic, elevated maigin
(Figs. 11-7 and 11-8). Hoiny mateiial may be
expiessed fiom the maigin oi the centei of the
lesion (Figs. 11-7, 11-8, and 11-10). Eiythema-
tous, yellowish, skin coloi. Haid. Polygonal,
oval, iound (Figs. 11-6 and 11-10), oi umbili-
cated and ulceiated.
DIstrIhutIvn Usually isolated but may be
multiple. Usually exposed aieas. Sun-induced
keiatotic and/oi ulceiated lesions especially on
the bald scalp (Fig. 11-1), cheeks, nose, lowei
lips (Fig. 11-6), eais (Fig. 11-11), pieauiiculai
aiea, doisa of the hands (Fig. 11-10), foieaims,
tiunk, and shins (females) (Fig. 11-12).
0ther Fhysca| Fodos Regional lymphaden-
opathy due to metastases.
Speca| Features In UV-ielated SCC evidence
of Jermao|e|oss and so|ar |eraoses . SCCs of
the lips develop fiom leukoplasia oi actinic
cheilitis; in 90% of cases they aie found on
the lowei lip (Fig. 11-6). In chionic iadio-
deimatitis they aiise fiom iadiation-induced
keiatoses (see Fig. 10-34); in individuals with
a histoiy of chionic intake of aisenic, fiom
aisenical keiatoses. Diffeientiated (i.e., hypei-
keiatotic) SCC due to HPV on genitalia; SCC
due to excessive PUVA theiapy on lowei ex-
tiemities (pietibial) oi on genitalia. SCCs in
scais fiom buins, in chionic stasis ulceis of
long duiation, and in sites of chionic inflam-
mation aie often difficult to identify. Suspicion
is indicated when nodulai lesions aie haid and
show signs of keiatinization (Figs. 11-8, 11-10,
and 11-11).
Seta| [orm : caicinoma cuniculatum, usually
on the soles, highly diffeientiated, HPV-ielated
but can also occui in othei settings (Fig. 11-13).

hstopatho|oy SCCs with vaiious giades of
anaplasia and keiatinization.
odIIereotated SCC
Iesoos Fleshy, gianulating, easily vulneiable,
eiosive papules and nodules and papilloma-
tous vegetations (Fig. 11-9). Ulceiation with
a neciotic base and soft, fleshy maigin. Bleeds
easily, ciusting. Red. Soft. Polygonal, iiiegulai,
often cauliflowei-like.
DIstrIhutIvn Isolated but also multiple, pai-
ticulaily on the genitalia, wheie they aiise fiom
eiythioplasia (see Fig. 35-24) and on the tiunk
Claik level I, intiaepideimal; level II, invades papil-
laiy deimis; level III fills papillaiy deimis; level IV,
invades ieticulai deimis; level V
,
invades subcutane-
ous fat.
(Fig. 11-5), lowei extiemities, oi face, wheie
they aiise fiom Bowen disease.
Msce||aoeous 0ther Sko Chaoes Lymphad-
enopathy as evidence of iegional metastases
is fai moie common than with diffeientiated,
hypeikeiatotic SCCs.
hstopatho|oy Anaplastic SCC with multiple
mitoses and little evidence of diffeientiation
and keiatinization.
As stated pieviously, any peisistent nodule,
plaque, oi ulcei, but especially when these occui
in sun-damaged skin, on the lowei lips, in aieas
of iadiodeimatitis, in old buin scais, oi on the
genitalia, must be examined foi SCC. Keiato-
acanthoma may be clinically indistinguishable
fiom diffeientiated SCC (Fig. 11-8 ).
MANACMNI
Surery Depending on localization and extent
of lesion, excision with piimaiy closuie, skin
flaps, oi giafting. Micioscopically contiolled
suigeiy in difficult sites. Radiotheiapy should
be peifoimed only if suigeiy is not feasible.
C0kS AN0 Fk0CN0SIS
kecurreoce aod Metastases SCC causes local
tissue destiuction but it has a significant poten-
tial foi metastases. Metastases aie diiected to ie-
gional lymph nodes and appeai 1 to 3 yeais aftei
initial diagnosis. In-tiansit metastases occui. In
solid oigan tiansplant iecipients, can be piesent
when SCC is diagnosed/detected oi shoitly aftei.
SCC in the skin has an oveiall metastatic iate of
3-4% and tends to occui with tumois that aie
laige, iecuiient, and involve deep stiuctuies of
cutaneous neives. High-iisk SCCs aie defined as
having a diametei >2 cm, a level of invasion >
4 mm, and Claik levels IV oi V ; tumoi involve-
ment of bone, muscle, and neive (so-called
neuiotiopic SCC, occuis fiequently on the
foiehead and scalp); location on eai, lip, and
genitalia; tumois aiising in a scai oi following
ionizing iadiation aie usually highly dediffeien-
tiated tumois. Canceis aiising in chionic osteo-
myelitis sinus tiacts, in buin scais, and in sites
of iadiation deimatitis have a metastatic iate
of 31, 20, and 18%, iespectively. On the othei
hand, SCC aiising in solai keiatoses have the
lowest potential foi metastasis. A special gioup
of high-iisk SCCs aie those in patients who aie
immunosuppiessed (Fig. 11-14).
SCCs o Immuoosuppressoo Oigan tians-
plant iecipients have a maikedly incieased
incidence of NMSCs, piimaiily SCC, which is
40 to 50 times gieatei than in the geneial popu-
lation. Risk factois include skin type, cumula-
tive sun exposuie, age at tiansplantation, male
sex, HPV infections, the degiee and length of
immunosuppiession, and the type of immuno-
suppiessant. Lesions aie often multiple, usually
in sun-exposed sites but also in the genital,
anal, and peiigenital iegions (Fig. 11-14).
These tumois giow iapidly and aie aggies-
sive; in one seiies of heait-tiansplant patients
fiom Austialia, 27% died of skin cancei.
Patients with AIDS have only a slight in-
cieased iisk of NMSC. In one seiies a fouifold
inciease in theii iisk of developing lip SCC
was noted. Howevei, SCC of the anus is sig-
nificantly incieased in this population (see also
Section 21).
FICk 11-8 Squamous ce|| carcooma, we|| dIIereotated A uodu|e ou ||e |oWe| +|r co.e|ed
W||| + dore|+ped d+|| |,pe||e|+|o|. A |+|e, |ouud, |+|d uodu|e ou ||e uoe W||| ceu||+| |,pe||e|+|o|.
|e|||e| |e|ou c+u |e d|||uu||ed ||or |e|+|o+c+u||or+ (ee ||. 5).

FICk 11-9 Squamous ce||
carcooma, uodIIereotated I|e|e |
+ c||cu|+|, dore|+ped |edd|| uodu|e
W||| p+|||, e|oded u||+ce ou ||e |erp|e
o| + 13,e+|o|d r+|e. I|e |e|ou |oW
uo |,pe||e|+|oe +ud | o|| +ud |||+||e.
w|eu c|+ped || ||eed e+||,.
FICk 11-10 Squamous ce|| carcooma, advaoced, we|| dIIereotated, oo the haod oI a 65-year-
o|d Iarmer I|e || uodu|e | roo||, .e|, |+|d upou p+|p+||ou, +ud |oW + ,e||oW|| co|o|, |oc+||, |ud|c+||u
|e|+||u |u ||e |od, o| ||e uodu|e. || ||e |e|ou We|e |uc|ed |u ||e ,e||oW|| +|e+, + ,e||oW||W|||e r+|e||+|
(|e|+||u) cou|d |e e\p|eed.
FICk 11-11 Squamous ce|| carcooma, hh|y dIIereotated, oo the ear I|e|e | + |e|+||.e|, |+|e
p|+que co.e|ed |, +d|e|eu| |+|d |,pe||e|+|oe. A|||ou| 'CC +|e |u eue|+| uo| p+|u|u|, |e|ou ou ||e |e||\ o|
+u||e||\ uu+||, +|e, + W+ ||e c+e |u ||| o9,e+|o|d r+u.
FICk 11-13 Squamous ce|| carcooma (carc-
ooma cuocu|atum) o a pateot wth perphera|
oeuropathy due to |eprosy A |+|e |uu+||u,
p+|||+||, uec|o||c +ud |,pe||e|+|o||c |uro| ou ||e o|e o|
||e |oo|. I|e |e|ou |+d |eeu cou|de|ed + ueu|op+|||c
u|ce|, +c|||ed |o |ep|o,, |u| cou||uued |oW|u +ud
|ec+re e|e.+|ed +ud u|ce|+|ed.
FICk 11-12 Squamous ce|| carcooma o
the setto oI chrooc stats dermatts aod
|oo-staodo, ooohea|o veoous u|cer I|e|e
W+ + .euou u|ce| o| ro|e ||+u 0 ,e+|' du|+||ou
+| ||e ||e, W||c| W+ uuucce|u||, ||e+|ed W|||
|op|c+| |ered|e. C|+du+||, ||e ceu|e| o| ||e u|ce|
|ec+re |+|de| +ud e|e.+|ed +ud uoW |ep|eeu| +
|||r e|e.+|ed, e+||, ||eed|u r+ W||| ,e||oW uec|o
e. |ou|+ud|u u|ce| o| ||e |e |ou|d +|W+, |e
||op|ed |o |u|e ou| qu+rou ce|| c+|c|uor+.
FICk 11-14 Squamous ce|| carcooma o a
reoa| traosp|aot recpeot oo the base oI the
scrotum |u +dd|||ou |o ||| u|ce|+||u |||r uodu|e
o| ||e |+e o| ||e c|o|ur, ||e p+||eu| |+d r+||e|,
|r||+| |e|ou e|eW|e|e ou ||e |od,. '|uce |e |+d
po||+| +ud |+d ||e|e|o|e peu| cou|de|+||e ||re
|u ||e uu, ||e |e|ou |u ||e uue\poed |de We|e
p|o|+||, due |o u\k. I|e |e|ou |oWu |e|e W+
p|o|+||, |u|||+|ed |, n|\ + |e |+d + |r||+| |e|ou
pe||+u+||, +ud ou ||e |+u.
FI0MI0I0C
Ae oI 0oset Ovei 50 yeais; iaie below 20
yeais. Male:female iatio 2:1.
FAIh0CNSIS
Human papillomaviius (HPV) -9, -16, -19, -25,
and -37 have been identified in KAs. Othei
possible etiologic factois include UV iadiation
and chemical caicinogens (industiial: pitch
and tai).
Rapid giowth, achieving a size of 2.5 cm within
a few weeks. No symptoms, but theie aie occa-
sional tendeiness and cosmetic disfiguiement.
Sko Iesoos Nodule, dome-shaped, often
with a cential keiatotic plug (Fig. 11-15). Skin-
coloied oi slightly ied, tan/biown. Fiim but not
haid. 2.5 cm (iange, 1-10 cm), iound. Keiatotic
plug may appeai like a cutaneous hoin. Re-
moval of plug iesults in a ciatei.
DIstrIhutIvn Isolated single lesion. Uncom-
monly, may be multiple, eiuptive. On exposed
skin: cheeks, nose, eais, hands (doisa).
0ermatopatho|oy A iepiesentative biopsy that
extends thiough the entiie lesion to pieseive the
KA | + pec|+| |e|ou, |o|re||, cou|de|ed + peu
doc+uce| || | uoW |e+|ded |, ro| + + .+||+u|
o| qu+rou ce|| c+|c|uor+.
A |e|+||.e|, corrou, |+p|d|, |oW|u ep|||e||+|
|uro| W||| po|eu||+| |o| ||ue de||uc||ou +ud
(|+|e) re|+|+|, |oWe.e|, |u ro| c+e pou|+
ueou |e|e|ou.
A dore|+ped uodu|e W||| ceu||+| |e|+|o||c p|u
(||. 5 1 ).
||ed||ec||ou |o| uue\poed ||e.
\u|||p|e KA occu|.
I|e+|reu| | |, e\c||ou.
kkAI0ACANIh0MA (kA) |C|9 . 2!3.2
|C|0 . |53.3
aichitectuie of the nodule oi piimaiy excision
is iequiied. Cential, laige, iiiegulaily shaped
ciatei filled with keiatin. The suiiounding
epideimis extends in a liplike mannei ovei the
sides of the ciatei. The keiatinocytes aie atypi-
cal and many aie dyskeiatotic. Diffeientiation
of KA fiom highly diffeientiated SCC is difficult
and may not always be possible.
Clinical findings confiimed by iepiesentative
biopsy. SCC, hypeitiophic solai keiatosis, vei-
iuca vulgaiis.
C0kS AN0 Fk0CN0SIS
Spontaneous iegiession in 2-6 months oi
sometimes >1 yeai in most cases. Theie is pio-
giessive keiatinization with expansion of the
cential keiatotic plug until all epithelial tumoi
tissue is conveited into hoiny mateiial and shed
(Figs. 11-15 B and 11-15 C ) which leads to a scai.
Howevei, KA is locally destiuctive; lymph node
and visceial metastases have been obseived in
some cases.
MANACMNI
Surery Suigical excision is iecommended in
that KA cannot be distinguished fiom SCC on
clinical findings.
Mu|tp|e kAs Systemic ietinoids and meth-
otiexate have been used.
SCII0N 11 |kECA|CEk0u' |E'|0|' A|| CuIA|E0u' CAkC||0\A' 28T
FI0MI0I0C
Ae oI 0oset Oldei than 40 yeais.
Iocdeoce United States: 500-1000 pei
100,000, highei in the sunbelt; >400,000 new
patients annually.
kace Raie in biown- and black-skinned pei-
sons.
II0I0C
UVR, mostly of the UVB spectium (290-320
nm) that induces mutations in suppiessoi
genes. The piopensity foi multiple BCC may
be inheiited. Associated with mutations in the
PTCH gene in many cases.
Fredsposo Factors Skin phototypes I and
II and albinos aie highly susceptible to develop
BCC with piolonged sun exposuie. Also a his-
toiy of heavy sun exposuie in youth piedisposes
the skin to the development of BCC latei in
BCC | ||e ro| corrou c+uce| |u |ur+u.
C+ued |, u\k, |C| eue ru|+||ou |u ro|
c+e.
C||u|c+||, d|||e|eu| |,pe. uodu|+|, u|ce|+||u, p|
reu|ed, c|e|o|u, +ud upe|||c|+|.
BCC | |oc+||, |u.+|.e, +|e|.e, +ud de||uc||.e
|u| |oW |oW|u, +ud ||e|e | .e|, ||r||ed (|||e|
+||, uo) |eudeuc, |o re|+|+|/e.
I|e+|reu| | |, u||c+| e\c||ou, \o| r|c|o
|+p||c u|e|,, e|ec||ode|cc+||ou, +ud cu|e||+e.
A|o c|,ou|e|, +ud |r|qu|rod c|e+r.
8ASAI CII CAkCIN0MA (8CC) |C|9 . 1!.0
|C|0 . C!!.\3090/!
life. Pievious theiapy with x-iays foi facial acne
gieatly incieases the iisk of BCC, even in those
peisons with a good ability to tan (skin photo-
types III and IV). Supeificial multicentiic BCC
occuis 30-40 yeais aftei ingestion of aisenic but
also without appaient cause.
Slowly evolving, usually asymptomatic. Eiosion
oi bleeding with minimal tiauma may be fiist
symptom.
Sko Iesoos Theie aie five t|nta| types:
nodulai, ulceiating, scleiosing (cicatiicial),
supeificial, and pigmented.
NoJu|ar BCC. Papule oi nodule, tianslucent
oi peaily." Skin-coloied oi ieddish, smooth
suiface with telangiectasia, well defined, fiim
(Figs. 11-16 and 11-17). Poitions of nodulai
BCC may have eiosions oi stipples of melanin
pigmentation.

FICk 11-15 keratoacaothoma showo dIIereot staes oI evo|utoo |u|||+||, ||e|e | + |ouud
dore|+ped, .e|, |||r uodu|e, |edd|| W||| + ceu||+| |,pe||e|+|o||c p|u. I|| |+ |eeu p+|||+||, |ed |e+.|u +
ceu||+| c|+|e| n,pe||e|+|o| |+ p|o|eed +ud |+ uoW |ep|+ced ro| o| ||e uodu|e, |e+.|u ou|, + |||u ||r
o| |uro| ||ue |u ||e pe||p|e|,. |u|||e| p|o|e|ou o| |,pe||e|+|oe +ud |e|+||u|/+||ou |+ uoW |ep|+ced +||
o| ||e |uro| +ud W||| |e |+|e| |ed, |e+.|u + c+|. '|uce ||| e.o|u||ou | uo| +|W+, p|ed|c|+||e, |e|+|o+c+u||or+
|ou|d +|W+, |e e\c|ed |u ||e e+||, |+e.

U|terang BCC. Ulcei (often coveied with a
ciust) with a iolled boidei (iodent ulcei), which
again is tianslucent, peaily, smooth with tel-
angiectasia, and fiim (Figs. 11-18 and 11-19).
St|erosng BCC. Appeais as a small patch
of moiphea oi a supeificial scai, often ill-
defined, skin-coloied, whitish but also with
peppeiy pigmentation (Fig. 11-20). In this
infiltiating type of BCC theie is an excessive
amount of fibious stioma. Histologically,
fingei-like stiands of tumoi extend fai into
the suiiounding tissue, and excision theie-
foie iequiies wide maigins. Scleiosing BCC
can piogiess to nodulai oi ulceiating BCC
(Figs. 11-20 B and 11-21).
Suer[ta| mu|tenrt BCCs. Appeai as thin
plaques (Figs. 11-22 and 11-23). Pink oi
ied; chaiacteiistic fine thieadlike boidei and
telangiectasia can be seen with the aid of a
hand lens. This is the only foim of BCC that
can exhibit a consideiable amount of scaling.
This can also give iise to nodulai and ulcei-
ating BCC (Fig. 11-23). BCC often bleeds
with minimal excoiiation by fingeinail. Solai
keiatosis, in compaiison, does not bleed but is
somewhat painful with excoiiation.
PgmeneJ BCC. May be biown to blue oi
black (Fig. 11-24). Smooth, glistening suiface;
haid, fiim; may be indistinguishable fiom
supeificial spieading oi nodulai melanoma
but is usually haidei. Cyst lesions may oc-
cui: iound, oval shape, depiessed centei
(umbilicated"). Stippled pigmentation can
be seen in any of BCC types.
DIstrIhutIvn (Image 11-2) Isolated single le-
sion; multiple lesions aie not infiequent; > 90%
occui in the face. Seaich caiefully foi dangei
sites": medial and lateial canthi (Fig. 11-17 ,
B , C ), nasolabial fold (Fig. 11-16 B ), behind
the eais (Figs. 11-18 B and 11-19). Supeificial
multicentiic BCCs occui on the tiunk (Figs.
11-22 and 11-23). BCC usually aiises only fiom
epideimis that has a capacity to develop (haii)
follicles. Theiefoie, BCCs iaiely occui on the
veimilion boidei of the lips oi on the genital
mucous membianes.
0ermatopatho|oy Solid tumoi consisting of
piolifeiating atypical basal cells, laige, oval,
deep-blue staining on H&E, but with little
anaplasia and infiequent mitoses; palisading
aiiangement at peiipheiy; vaiiable amounts of
mucinous stioma.
Seiious BCCs occuiiing in the dangei sites
(cential pait of the face, behind the eais) aie
ieadily detectable by caieful examination with
good lighting, a hand lens, and caieful palpa-
tion and deimoscopy. Diagnosis is made clini-
cally and confiimed micioscopically. Diffeiential
FICk 11-16 8asa| ce|| carcooma: oodu|ar type A r+|| pe+||, p+pu|e (+||oW) ou ||e uo|||| +ud
+u e.eu r+||e| oue (r+|| +||oW) |u ||e u+o|+||+| |o|d. I|ee +|e .e|, e+||, |+e o| BCC. I|e |+, +||oW
deuo|e + de|r+| |\|. I|| | + |u|||e| +d.+uced uodu|+| BCC. A o|||+|,, ||u,, uodu|e W||| |+|e |e|+u|ec
|+||c .ee| ou ||e +|+ u+|, +|||u ou ||u W||| de|r+|o|e||o|.

diagnosis includes all smooth papules such as
deimal nevomelanocytic nevi, tiichoepithelioma,
deimatofibioma, and otheis; if pigmented, su-
peificial spieading and nodulai melanoma; if ul-
ceiated, all nonpainful fiim ulceis including SCC
and a (extiagenital) piimaiy chancie of syphilis.
MANACMNI
Excision with piimaiy closuie, skin flaps, oi
giafts. Ciyosuigeiy and electiosuigeiy aie op-
tions, but only foi veiy small lesions and not in
the dangei sites oi on the scalp.
FICk 11-1T 8asa| ce|| carcooma: oodu|ar type A |||eu|u, roo|| p|+que ou ||e |oWe| e,e||d
W||| ru|||p|e |e|+u|ec|+|+. Au o.+|, pe+||, uodu|e ou ||e uoe c|oe |o ||e |uue| c+u||u. A roo||,
pe+||, |uro| W||| |e|+u|ec|+|+ |e|oW ||e |oWe| e,e||d. Iuro| |ee| |+|d, | We|| de||ued, +ud | +,rp|or+||c.
A |+|e, |||r |edd|| |||eu|u uodu|e W||| r+|| u|ce|+||ou ou ||e uoe.
C 0

Foi lesions in the dangei sites (nasolabial
aiea, aiound the eyes, in the eai canal, in the
posteiioi auiiculai sulcus, and on the scalp)
and scleiosing BCC, micioscopically contiolled
suigeiy (Mohs suigeiy) is the best appioach.
Radiation theiapy is an alteinative only when
disfiguiement may be a pioblem with suigical
excision (e.g., eyelids oi laige lesions in the na-
solabial aiea) oi in veiy old age.
Theie aie a vaiiety of topical tieatments that
can be used foi supeificial BCCs but only foi
those tumois below the neck; tryosurgery is ef-
fective but leaves a white scai that iemains foi
life. Electiocauteiy with cuiettage is also simple
and effective, but it leaves scais and should
be used only in small lesions. Topical 5-fluoi-
ouiacil ointment and imiquimod cieam foi
supeificial BCC, 5 times a week, foi 6 weeks, aie
effective, do not cause scais, but iequiie con-
sideiable time and may not iadically iemove all
tumoi tissue. Imiquimod iequiies compliance
by patient oi caiegivei. Imiquimod is especially
FICk 11-18 8asa| ce|| carcooma, u|cerated: kodeot u|cer A |+|e c||cu|+| u|ce| ou ||e ||p o|
||e uoe W||| + W+|||||e |o|de|. A |r||+| |e|ou |u ||e |e||o+u||cu|+| |e|ou. I|e|e | + |o||ed pe+||, |o|de|
u||ouud|u ||e u|ce|. kodeu| u|ce| |u ||e p|e+u||cu|+| |e|ou. A |o||ed pe+||, |o|de| u||ouud +u u|ce| W|||
,e||oW uec|oe +ud + ||u, ||+c| c|u|. A deep u|ce| W||| + u||ouud|u |o||ed |o|de|, roo||, |||eu|u +ud
p+|||, co.e|ed W||| c|u| |u ||e r+ud||u|+| |e|ou. A|| ||ee |e|ou +|e |+|d upou p+|p+||ou.
C 0

FICk 11-19 A |are rodeot u|cer o the oucha| aod retroaurcu|ar area exteodo to the temp|e
I|e eu|||e |e|ou cou|| o| + |||r |+uu|+||u ||ue, p+|||+||, co.e|ed |, |ero|||+|c c|u|. I|e d|+uo| c+u
|e r+de ou|, |, e\+r|u|u ||e |o|de|, W||c| | |o||ed, e|e.+|ed, |||r, +ud roo||.
FICk 11-20 8asa| ce|| carcooma: sc|eroso type A r+|| |ucoup|cuou +|e+ |eer|||u upe|||c|+|
ro|p|e+, ||| de||ued, ,e||oW|| W||| |e|+u|ec|+|+. upou p+|p+||ou, |oWe.e|, + p|+|e|||e |udu|+||ou c+u |e |e|| +ud
||| e\|eud |e,oud ||e .||||e r+||u o| ||e |e|ou. A||e| .e||||c+||ou o| ||e d|+uo| |, ||op,, || W||| |equ||e
e\c||ou W||| W|de r+||u. A |+|e dep|eed +|e+ |eer|||u + c+| ou ||e uoe, ou ||e |||| (|+|e|+|) +ud
red|+| r+||u o| ||| 'c+| ||e|e | ||e |,p|c+| |o||ed |o|de| o| + uodu|+| BCC. I|| |e|ou | |oWu |o derou
||+|e ||+| c|e|o|u +ud uodu|+| BCC +|e |rp|, |Wo d|||e|eu| |oW|| p+||e|u.
8
FICk 11-21 8asa| ce||
carcooma, sc|eroso, oodu|ar,
aod u|cerato A |+|e |e|ou,
W||c| |oo| |||e ro|p|e+ +ud |
W||||| +ud |||r upou p+|p+||ou
|u| W||||u ||e |e.e| o| ||e ||u, |
|ouud ou ||e |erp|e +ud |u ||e
up|+c|||+| |e|ou. w||||u |e|ou
+ud +| ||e r+||u ||e|e +|e r+||
uodu|e o| BCC. 0u ||e |+|e|+|
c+u||u o| ||e e,e ||e|e | + |+|e
u|ce| W||| |o||ed |o|de| |ep|eeu|
|u |odeu| u|ce|. A+|u ||| ||u|e |
|oWu |o derou||+|e ||+| ||e d||
|e|eu| |,pe o| |++| ce|| c+|c|uor+
+|e ju| d|||e|eu| |oW|| p+||e|u.
FICk 11-22 SuperIca| basa| ce|| carcooma: so|tary |esoo aod mu|tp|e |esoos I|| |||||
|ed |e|ou |+ + |||||, e|e.+|ed |o||ed |o|de| ||+| c+u |e de|ec|ed W||| '|de |||||u, +|||ou| ||| |e|ou |
|,p|c+| euou| |o |e d|+uoed c||u|c+||,, + ||op, | uece+|, |o .e|||, ||e d|+uo|. \+u, upe|||c|+| |++|
ce|| c+|c|uor+ ou ||e ||uu|. I|e, +ppe+| + ||||||, e|,||er+|ou, o||eu c+||u, ||+| |e|ou, o||eu W|||ou| +
|o||ed |o|de|. I|e |,pop|reu|ed +|e+ |ep|eeu| upe|||c|+| c+| +||e| c|,o||e|+p, o| upe|||c|+| BCC.
8
good foi young peisons who do not want scais.
Photodynamic theiapy is effective only in veiy
supeificial lesions and iadiation sessions (pho-
todynamic dye - visible light) aie painful.
C0kS AN0 Fk0CN0SIS
BCC does not metastasize. The ieason foi
this is the tumoi's giowth dependency on its
stioma, which on invasion of tumoi cells into
the vessels is not disseminated with the tumoi
cells. When tumoi cells lodge at distant sites,
they do not multiply and giow because of the
absence of giowth factois deiived fiom theii
stioma. Exceptions occui when a BCC shows
signs of dediffeientiation, foi instance, aftei
inadequate iadiotheiapy. Most lesions aie iead-
ily contiolled by vaiious suigical techniques.
Seiious pioblems, howevei, may occui with
BCC aiising in the dangei sites of the head. In
these sites the tumoi may invade deeply, cause
extensive destiuction of muscle and bone, and
even invade to the duia matei. In such cases,
death may iesult fiom hemoiihage of eioded
laige vessels oi infection.
FICk 11-23 SuperIca| basa| ce|| carcooma, ovasve I|e|e +|e |Wo |||eu|+| |ed +|e+ W||| |o||ed
|o|de| +ud ceu||+| |e|+u|ec|+|+. |u ||e |+|e| |e|ou ||e BCC | e|e.+|ed W||| +u |||eu|+| u||+ce +ud uoW
+ure ||e ro|p|o|o, +ud |oW|| |e|+.|o| o| + uodu|+| BCC, ou ||e |||| ||e |e|ou | e|o|.e +ud W|||
p|o|e |o +u u|ce|.
I|| +u|oor+| dor|u+u| d|o|de| | c+ued |,
ru|+||ou |u ||e p+|c|ed eue ||+| |e|de ou
c||oroore 9q (9q22).
|| +||ec| ||u W||| ru|||p|e BCC +ud oc+||ed
p+|rop|+u|+| p|| +ud |+ + .+||+||e e\p|e|ou o|
+|uo|r+||||e |u + uur|e| o| ,|er, |uc|ud|u
|e|e|+| r+||o|r+||ou, o|| ||ue, e,e, C|', +ud
eudoc||ue o|+u.
I|e ,ud|ore occu| ro||, |u W|||e |u| +|o |u
||oWu +ud ||+c|||uued peop|e, +ud ||e|e | +u
equ+| e\ |uc|deuce.
BCC |e|u |u|, |u c|||d|ood o| e+||, +do|e
ceuce +ud cou||uue |||ou|ou| |||e.
I|e|e +|e ro|e BCC ou ||e uue\poed +|e+ o|
||e ||u, |u| ||e, +|o occu| |u co.e|ed +|e+ +ud
||e|e r+, |e |uud|ed o| |e|ou.
C|+|+c|e||||c eue|+| |e+|u|e +|e ||ou|+| |o
|u, + ||o+d u++| |oo|, +ud |,pe||e|o||r. A
,|er |e.|eW r+, |e.e+| coueu||+| +uor+||e
|uc|ud|u uudeceuded |e|e +ud |,d|ocep|+|u.
0||e| -|c:|c- |- +|e r+ud||u|+| j+W
odou|oeu|c |e|+|oc,|, W||c| r+, |e ru|||p|e
+ud r+, |e uu||+|e|+| o| |||+|e|+|. I|e|e r+, |e
de|ec||.e deu||||ou, ||||d o| p|+,ed |||, pec|u
e\c+.+|ur, |o|| |ou||| re|+c+|p+|, co||o|,
+ud |,p|o|. E,e |e|ou |uc|ude ||+||ru,
|,pe||e|o||r, d,|op|+ c+u||o|ur, c+|+|+c|,
|+ucor+, +ud co|o|or+ W||| |||udue. I|e|e
r+, |e +eue| o| ||e co|pu c+||our, redu|
|o||+|or+, +ud c+|c|||c+||ou o| ||e |+|\. \eu|+|
|e|+|d+||ou | |+|e, |oWe.e|. ||||o+|cor+ o| ||e
j+W, o.+||+u ||||or+, |e|+|or+, +ud c,|+deuo
r+ |+.e |eeu |epo||ed.
'| |- +|e r+||, p|upo|u| |o |+|e| uodu|+|
BCC (||. 25), |u| '|eu|+|, uodu|+|, u|ce|+|
|u, +ud c|e|o|u BCC +|o occu|. Iuro| ou
||e e,e||d, +\|||+e, +ud uec| |eud |o |e peduucu
|+|ed +ud +|e o||eu ,rre|||c ou ||e |+ce. I|e|e
+|e c|+|+c|e||||c p+|rop|+u|+| |e|ou, W||c|
+|e p|eeu| |u 50 +ud +|e r+|| p|| ||+| +|e
p|upo|u| |o e.e|+| r||||re|e| |u |/e +ud rr
deep (||. 2o).
I|e |u|||c+uce o| ||e ,ud|ore | ||+| + |+|e
uur|e| o| ||u c+uce| c|e+|e + |||e||re p|o||er
o| .|||+uce. I|e ru|||p|e e\c||ou c+u c+ue +
cou|de|+||e +rouu| o| c+|||u (||. 25). I|e
|uro| cou||uue |||ou|ou| |||e, +ud ||e p+||eu|
ru| |e |o||oWed c+|e|u||,.
',,. Co|||u ,ud|ore, ue.o|d |++| ce||
c+|c|uor+ ,ud|ore.
8ASAI CII NvS SN0k0M (8CNS) |C|9 . 1!.0
IMAC 11-2 8asa| ce|| carcooma:
pred|ectoo stes |o| |ud|c+|e
upe|||c|+| ru|||ceu|||c BCC.
FICk 11-25 8asa| ce||
oevus syodrome: sma|| basa|
ce|| carcoomas 'r+|| |edd||
p+pu|+| |e|ou +|e d|pe|ed o.e|
||e eu|||e |+ce. A|| o| ||ee |ep|e
eu| r+|| BCC. |o|e cou|de|+||e
c+|||u ||or |ero.+| o| p|e.|ou
|e|ou. |o|e +|o ||ou|+| |o|u
+ud ||+||ru.
FICk 11-24 8asa| ce|| carcooma, p-
meoted A uodu|e W||| |||eu|+| |o|de| +ud
.+||e+||ou o| re|+u|u |ue, e+||, cou|ued W||| +
r+||u+u| re|+uor+. |e+|u|e |ud|c+||u BCC +|e
||e +|e+ o| ||+u|uceuc, +ud u||+ce |e|+u|ec|+|+.
Au |||eu|+| p||c|||+c| p|+que W||| + ceu||+|
+|e+ o| |e|e|ou. I|| p|reu|ed BCC | c||u|c+||,
|ud|||uu||+||e ||or upe|||c|+| p|e+d|u re|+
uor+. Corp+|e W||| ||. 20C.
FICk 11-26 8asa| ce|| oevus syodrome:
pa|mar pts |+|r+| u||+ce o| |+ud |oW|u
|o 2rr, |+|p|, r+||u+|ed, dep|eed |e|ou, |.e.,
p+|r+| p||.
C+|c|uor+ o| ||e ecc||ue We+| |+ud +|e |+|e
+ud |uc|ude ecc||ue po|oc+|c|uor+, ,||uo|d ec
c||ue c+|c|uor+, ruc|uou c+|c|uor+, +ud c|e+|
ce|| ecc||ue c+|c|uor+.
C+|c|uor+ o| ||e +poc||ue |+ud +|e +|o |+|e,
+|||u |u +\|||+e, u|pp|e, .u|.+, +ud e,e||d.
C+|c|uor+ o| ||e e|+ceou |+ud +|e equ+||,
|+|e, ro| corrou|, +|||u ou ||e e,e||d.
I|ee |e|ou +|e c||u|c+||, |ud|||uu||+||e ||or
o||e| c+|c|uor+ +ud +|e uu+||, ro|e +|e|.e
||+u o||e| |u.+|.e cu|+ueou 'CC.
MAIICNANI AFFN0AC IM0kS |C|9 . 1!.0
\e||e| ce|| c+|c|uor+ (\CC) (cu|+ueou ueu
|oeudoc||ue |uro|) | + |+|e r+||u+u| o||d
|uro| ||ou|| |o |e de||.ed ||or + pec|+||/ed
ep|||e||+| ce||, ||e \e||e| ce||. || | + uou|e|+||/|u,
'c|e+| ce|| p|eeu| |u ||e |++| ce|| |+,e| o| ||e
ep|de|r|, ||ee |u ||e de|r|, +ud +|ouud |+||
|o|||c|e + ||e |+|| d|| o| ||u|u.
\CC occu| +|ro| e\c|u|.e|, |u W|||e peop|e.
\CC | 0 |o !0 ||re + corrou |u |rruuoup
p|eed p+||eu| + |u uou|rruuoupp|eed
p+||eu|.
I|e e||o|o, | uu|uoWu |u| r+, |e |e|+|ed |o
c||ou|c u\k d+r+e. |o|,or+ .||u |+ |eeu
|ouud |u 30 o| \CC.
I|e |uro| r+, |e o|||+|, o| ru|||p|e +ud occu|
ou ||e |e+d +ud ou ||e e\||er|||e.
I|e|e | + ||| |+|e o| |ecu||euce |o||oW|u
e\c||ou, |u|, ro|e |rpo||+u|, || p|e+d |o ||e
|e|ou+| |,rp| uode |u > 50 o| p+||eu| +ud |
d|er|u+|ed |o ||e .|ce|+ +ud C|'.
\CC p|eeu| + + cu|+ueou |o u|cu|+ue
ou p+pu|e, uodu|e, o| |uro| (0.5-5 cr) (||.
21, 23, !o2!), W||c| | p|u|, |ed |o .|o|e|
o| |edd||||oWu, dore|+ped, +ud uu+||,
o|||+|,. I|e o.e||,|u ||u | |u|+c|, |u| |+|e|
|e|ou r+, u|ce|+|e.
I|e, |oW |+p|d|, +ud uu+||, occu| |u pe|ou
> 50 ,e+|.
|e|r+|op+||o|o, |oW uodu|+| o| d|||ue p+|
|e|u o| +|e+|ed, deep|, ||ue |+|u|u, r+||
|++|o|d o| |,rp|or+|||e|oo||u ce|| ||+| c+u
+|o |e +||+ued |u |ee| |o|r|u ue|, co|d,
+ud ||+|ecu|+e.
|rruuoc,|oc|er|||, |oW c,|o|e|+||u +ud
ueu|o|||+reu| r+||e|, c||oro|+u|u A, +ud
ueu|oupec|||c euo|+e, e|ec||ou r|c|ocop, |e
.e+| ||e c|+|+c|e||||c o|+ue||e.
I|e+|reu| | |, e\c||ou o| \o| u|e|,, +ud
eu||ue| uode ||op, o| p|op|,|+c||c |e|ou+|
uode d|ec||ou | +d.oc+|ed |ec+ue o| ||e |||
|+|e o| |e|ou+| re|+|+e. k+d|+||ou ||e|+p, |o
||e o| \CC +ud |e|ou+| || | |.eu |u ro| c+e
e\cep| |o| .e|, r+|| |e|ou.
kecu||euce |+|e +|e |||, |u oue e||e, e.eu
W|||ou| + |oc+| |ecu||euce, +|ou| o0 o| p+||eu|
de.e|oped |e|ou+| uode re|+|+e, + d|d 3o
o| ||oe p+||eu| W||| + |oc+| |ecu||euce. ||ouo
| | u+|ded.
MkkI CII CAkCIN0MA |C|9 . 1!.0
|C|0 . C++.\32+1/!
SCII0N 11 |kECA|CEk0u' |E'|0|' A|| CuIA|E0u' CAkC||0\A' 29T
FICk 11-2T Merke| ce|| car-
cooma A r+|| .|o|+ceou uodu|e
+|o.e ||e p|uu+ ||+| |+d |eeu p|e
eu| |o| +|ou| 2 Wee|. 'eu||ue| |,rp|
uode ||op, |e.e+|ed re|+|+| o|
ueu|oeudoc||ue c+|c|uor+. A|o uo|e
+c||u|c |e|+|oe ou ||e |e||\ +ud
couc|+.
FICk 11-28 Merke| ce|| carcooma A |+|e|, uo||ce+||e orr |||||, de|r+| uodu|e |e|oW ||e
|+||||ue ||+| |+d |eeu p|eeu| |o| +|ou| o Wee|. ||e+u||cu|+| |,rp| uode re|+|+| W+ +|o p|eeu|. A
.|o|+ceou de|r+| uodu|e, ! cr |u d|+re|e| ou ||e |o|e+|r o| + o0,e+|o|d r+u. I|e|e W+ re|+|+| |o ||e
+\|||+|, |,rp| uode.
8
A |+|e, |oc+||, +|e|.e |uro|, |oW |oW|u,
|u|||+||, o||eu r||u|e|p|e|ed + + c+|.
||'| | + |||r |udu|+|ed p|+que, ||uco|o|ed |o
|ed||oWu W||| e\op|,||c uodu|e (||. 29).
Au +||op||c .+||+u| r+, |eer||e |ce|o|u BCC,
ro|p|e+, o| c+|.
0ccu| ou ||e ||uu|, |o||oWed |, ||e e\||er|||e,
+ud ou|, 5 |u ||e |e+d +ud uec| |e|ou.
|oc+||, +|e|.e W||| + ||| |+|e o| |ecu||euce
+ud |+|e re|+|+e.
||+uo| | r+de |, |||op+||o|o,, +ud ||e|+p,
| W|de u||c+| e\c||ou. kecu||euce |epoud |o
C|ee.ec.
0kMAI0FI8k0SAkC0MA Fk0I8kANS (0FSF) |C|0 . \33!!/!
FICk 11-29 0ermatoIbrosarcoma protuberaos Au |||eu|+| c|e|o||c ||uco|o|ed |o |edd|| p|+que
o| |uc|e+ed cou||euc, ou ||e |+c| o| + +0,e+|o|d r+|e. 0u ||e |oWe| r+||u ||e|e | + |edd|| uodu|e |ep|e
eu||u e\op|,||c |oW||. I|| |e|ou ueed |o |e e\c|ed W||| |+|e r+||u |o p|e.eu| + |ecu||euce.
A uo| o |+|e |+p|d|, |oW|u |uro| o| |u|e|red|
+|e r+||u+u| po|eu||+|.
A|/ | +u +,rp|or+||c, o|||+|, p+pu|e, uodu|e,
o| p|+que o||eu |eer|||u +u 'CC o| BCC
|u|||+||,.
0ccu| |u uud+r+ed ||u o| o|de| p+||eu|
epec|+||, ou |o|e|e+d, c+|p. uoe, +ud e+|
(||. !0).
I|e+|reu| | u||c+|.
AIFICAI FI8k0SAkC0MA (AFX) |C|9 . 1!.0
FICk 11-30 Atypca| Ibroxaothoma I|| | + 51,e+|o|d r+|e W||| de|r+|o|e||o| +ud + |||o|, o|
o|+| |e|+|oe, |u.+|.e +ud |u ||u qu+rou c+|c|uor+, +ud |++| ce|| c+|c|uor+. I|| uodu|e ou ||e .e||e\
W+ c||u|c+||, +|,p|c+| |o| e|||e| |++| ce|| c+|c|uor+ o| qu+rou ce|| c+|c|uor+, |||op+||o|o, |e.e+|ed +|,p|c+|
||||o\+u||or+.
300
S E C I | 0 N 1 2
||ecu|o| o| re|+uor+ +|e |e|ou ||+| +|e
|eu|u pe| e |u| |+.e ||e po|eu||+| o| |u|u|u
r+||u+u| +ud ||u |.|u ||e |o re|+uor+.
IWo uc| eu||||e +|e |ecou|/ed. () d,p|+
||c ue.ore|+uoc,||c ue.|, +ud (2) coueu||+|
ue.ore|+uoc,||c ue.|.
FkCkS0kS 0F CIAN0S MIAN0MA
FI0MI0I0C
Ae oI 0oset Childien and adults.
Freva|eoce || aie piesent in 5% of the geneial
white population. They occui in almost eveiy
patient with familial cutaneous melanoma and
in 30-50% of patients with spoiadic nonfamil-
ial piimaiy melanomas of the skin.
Sex Equal in males and females.
kace White peisons. Data on peisons with
biown oi black skin aie not available; DN aie
iaiely seen in the Japanese population.
Iraosmssoo Autosomal dominant.
FAIh0CNSIS
Multiple loci have been implicated in familial
melanoma/DN syndiome. It is assumed than an
abnoimal clone of melanocytes can be activated
by exposuie to sunlight. Immunosuppiessed
MIAN0MA FkCkS0kS
AN0 FkIMAk CIAN0S
MIAN0MA
|,p|+||c re|+uoc,||c ue.| (||) +|e + pec|+|
|,pe o| +cqu||ed, c||curc|||ed, p|reu|ed
|e|ou ||+| |ep|eeu| d|o|de|ed p|o|||e|+||ou o|
.+||+||, +|,p|c+| re|+uoc,|e.
|| +||e de uo.o o| + p+|| o| + corpouud
re|+uoc,||c ue.u.
|| +|e c||u|c+||, d|||uc||.e ||or corrou +c
qu||ed ue.|. |+|e| +ud ro|e .+||e+|ed |u co|o|,
+,rre|||c |u ou|||ue, |||eu|+| |o|de|, ||e, +|o
|+.e c|+|+c|e||||c |||o|o|c |e+|u|e.
|| +|e |e+|ded + po|eu||+| p|ecu|o| o| upe|
||c|+| p|e+d|u re|+uor+ +ud +|o + r+||e| o|
pe|ou +| ||| |o| de.e|op|u p||r+|, r+||u+u|
re|+uor+ o| ||e ||u, e|||e| W||||u ||e || o| ou
'uo|r+| ||u.
|| occu| e|||e| po|+d|c+||, o| |u ||e cou|e\| o|
||e |c|c| || ,!- . ||ud|ed W||| |+r|||+|
ru|||p|e || +ud re|+uor+ (|o|re||, |A\\\,
o| BK ro|e ,ud|ore).
',uou,r. +|,p|c+| re|+uoc,||c ue.u, d+|| ue.u
0SFIASIIC MIAN0CIIC NvS |C|9 . 2!3.2
|C|0 . |+35
patients (ienal tiansplantation) with DN have
a highei incidence of melanoma. DN favoi the
exposed aieas of the skin, and this may be ie-
lated to the degiee of sun exposuie.
0uratoo oI Iesoos DN usually aiise latei in
childhood than common acquiied nevomelano-
cytic nevi (NMN), appeaiing fiist in late child-
hood, just befoie pubeity. New lesions continue
to develop ovei many yeais in affected peisons;
in contiast, common acquiied NMN do not ap-
peai aftei middle age and disappeai entiiely in
oldei peisons. Also, wheieas common NMN aie
usually in a ioughly compaiable stage of devel-
opment in a given body iegion (e.g., junctional,
compound, deimal), DN appeai out of step,"
e.g., a mix of laige and small, flat and iaised, tan
and veiy daik lesions (Fig. 12-1).
SCII0N 12 \E|A|0\A |kECuk'0k' A|| |k|\Ak\ CuIA|E0u' \E|A|0\A 301
IA8I 12-1 Comparative |eatures of Common Nevome|anocytic Nevi (NNN), 0ysp|astic Nevi (0N),
and Superficia| Spreadin Ne|anoma (SSN)
hNh 0h SSN
Les|oo F|gs. 9-1 to 9-4) (F|gs. 12-1 aod 12-2) (F|gs. 12-10 aod 12-11)
|ur|e| 'e.e|+| o| r+u, 0ue o| r+u, '|u|e (-2 |+.e
ru|||p|e)
||||||u||ou \o||, ||uu|, \o||, ||uu|, Au,W|e|e |u| p|edor|u+u|
e\||er|||e e\||er|||e uppe| |+c|, |e
0ue| C|||d|ood, E+||, +do|eceuce Au, +e, ro| |u
+do|eceuce +du|||ood
I,pe \+cu|e (juuc||ou+|) \+cu|e W||| |+|ed ||+que, |||eu|+|
|+pu|e (corpouud, po|||ou (+,rre|||c+||,,
de|r+|) r+cu|op+pu|+|)
A A,rre||, ',rre||, A,rre||, C|e+|e| +,rre||,
B Bo|de| keu|+|, We||de||ued |||eu|+|, ||| +ud |||eu|+|, We||de||ued
We||de||ued
C Co|o| I+u, ||oWu, d+|| I+u, ||oWu, d+|| I+u, ||oWu, d+|| ||oWu,
||oWu, uu||o|r, ||oWu, ||+c|, p|u|, |ed, ||ue,
o|de||, p+||e|u p|u|, |ed, uo| uu||o|r, W|||e, uu+||, + r|\,
.+||e+|ed p+||e|u, ||||, .+||e+|ed,
'|||ed e, '|+|e|o|d po||ed, pec||ed p+||e|u
| ||+re|e| <5 rr, |+|e|, <0 rr up |o 5 rr \o| >5 rr (|u|, o|
cou|e, |+|| r+||e|)
E Eu|+|ereu| '|op |u +do|eceuce Cou||uue |u C|oW|| |u |/e +| +u,
+du|||ood |u| ||r||ed +e, uu||r||ed
FICk 12-1 0ysp|astc oev . 0.e|.|eW o| ||e |+c| o| + p+||eu| W||| corrou +ud d,p|+||c ue.|. |o|e
+ uur|e| o| |e|ou +|e o| d|||e|eu| |/e +ud co|o|, 'ou| o| |ep. I|e |e|ou r+||ed |, +u +||oW W+ +u ''\. 8.
|+|e| r+u|||c+||ou o| |Wo ||. |o|e |||eu|+|||,, .+||e+||ou o| co|o| W||c| +|e d|||e|eu| |u ||e |Wo |e|ou ('ou|
o| |ep). A|o, ||e |e|ou +|e cr o| |+|e| |u d|+re|e|. I|e +||oW deuo|e + e|o|||e|c |e|+|o|. I|e r+||e|
|e|ou +|e corrou |\|.
8
DN aie thought not to undeigo spontaneous
iegiession at all oi at least much less than com-
mon acquiied NMN.
Frecptato Factors Exposuie to sunlight is
iegaided by some as an inducing agent foi DN;
neveitheless, DN aie not infiequently obseived
in completely coveied aieas such as the scalp
and anogenital aieas.
Sko Symptoms Asymptomatic.
Fam|y hstory In the familial setting, family
membeis can develop melanoma without the
piesence of DN.
C|oca| Features DN show some of the fea-
tuies of common NMN and some of supeifi-
cial spieading melanoma, so that they occupy
an inteimediaiy position between these two
moiphologies (Table 12-1). No single featuie
is diagnostic; iathei, theie is a constellation of
findings. They aie moie iiiegulai, lightei than
common NMN, usually maculopapulai (DN
have a maculai component); have distinct anJ
indistinct boideis (Figs. 12-1 and 12-2), and a
gieatei complexity of coloi than common nevi
(Figs. 12-1 and 12-2) but less than melanoma.
Fiied-egg" and taigeted" types (see Fig. 12-2E
and Table 12-1). Melanoma aiising in an DN
appeais initially as a small papule (often of a
diffeient coloi) oi change in coloi pattein and
massive coloi change within the piecuisoi le-
sion (Fig. 12-3).
0ermoscopy This noninvasive technique al-
lows foi clinical impiovement of diagnostic
accuiacy in DN by >50%. Dga| Jermostoy
peimits computeiized follow-up of lesions and
immediate detection of any change ovei time,
indicating developing malignancy.
0ermatopatho|oy Hypeiplasia and piolifeia-
tion of melanocytes in a single-file, lentiginous"
pattein in the basal cell layei eithei as spindle
cells oi as epithelioid cells and as iiiegulai and
dyshesive nests. Atypical" melanocytes, biidg-
ing" between iete iidges by melanocytic nests;
spindle-shaped melanocytes oiiented paiallel
to skin suiface. Lamellai fibioplasia and con-
centiic eosinophilic fibiosis (not a constant fea-
tuie). Histologic atypia do not always coiielate
with clinical atypia. DN may aiise in contiguity
with a compound NMN (iaiely, a junctional
nevus) that is centially located, i.e., DN often
have extension of intiaepideimal melanocytic
hypeiplasia beyond the shouldei of the deimal
nevus component; some DN may not have a
deimal nevus component.
The diagnosis of DN is made by clinical iecog-
nition of typical distinctive lesions (see Table
12-1), and diagnostic accuiacy is consideiably
impioved by deimoscopy. The clinicopatho-
logic coiielations aie now well documented.
Siblings, childien, and paients should also be
examined foi DN once the diagnosis is estab-
lished in a family membei.
0IIereota| 0aooss Congenital NMN, com-
mon acquiied NMN, supeificial spieading ma-
lignant melanoma, melanoma in situ, lentigo
maligna, Spitz nevus, pigmented basal cell cai-
cinoma.
Assocatoo wth Me|aooma DN aie iegaided
as maikeis foi peisons at iisk foi melanoma
and as piecuisois of supeificial spieading
melanoma. Anatomic association (in conti-
guity) of DN has been obseived in 36% of
spoiadic piimaiy melanomas, in about 70%
of familial piimaiy melanomas, and in 94% of
melanomas with familial melanoma and DN.
IIetme ksks oI 0eve|opo Frmary Ma|oaot
Me|aooma
Geneial population: 1.2%
Familial DN syndiome with wo blood iela-
tives with melanoma: 100%
All othei patients with DN: 18%
The piesence of one DN doubles the iisk foi
development of melanoma; with 10 DN, the
iisk incieases 12-fold.
MANACMNI
Suigical excision of lesions with naiiow mai-
gins. Lasei oi othei types of physical destiuc-
tion should neer be used because they do not
peimit histopathologic veiification of diagnosis.
The following guidelines foi selection of lesions
to be excised aie suggested:
Lesions that aie changing (inciease in size,
change in pigmentation pattein, changes in
shape and/oi boidei); decision is best and
most ieliably made by digital deimoscopy.
Lesions that cannot be closely followed by
the patient by self-examination (on the scalp,
genitalia, uppei back).
Patients with DN in the familial melanoma set-
ting need to be followed caiefully: in familial
DN, eveiy 3 months; in spoiadic DN, eveiy 6
months to 1 yeai. Seaich foi changes in exist-
ing DN and development of new nevi. Photo-
giaphic follow-up is impoitant, with the tiunk
FICk 12-2 0ysp|astc oev . A |+|e, uu||o|r|, |+u, .e|, ||+| r+cu|+| o.+| |e|ou. I|e uo|c|ed |o|de|
ou ||e |e|| +ud ||e |/e (> cr) +|e ||e ou|, c|||e||+ r+||u ||| up|c|ou o| + ||. 8. I|ou| |e|+||.e|, ,r
re|||c ||| |e|ou | r+cu|+| +ud p+pu|+| W||| + .+||e+|ed co|o| +ud re+u|e .5 cr |u d|+re|e|. I|e r+||e|
|e|ou +|e corrou |\|. C. A ||||, +,rre|||c, |o|| ||| +ud |+|p|, de||ued r+||u, + uo|c|ed |o|de|, +ud
.+||e+|ed ||oWu |o ||+c| co|o|. || | c||u|c+||, |ud|||uu||+||e ||or +u ''\ (ee ||. 20A, B) |u| W+ |||o
|o|c+||, + ||. 0 I|| |e|ou | +,rre|||c W||| +u |||eu|+|, uo|c|ed |o|de|, || |+ + r+cu|+| corpoueu| +ud
+ pe|||, u||+ce |u ||e p+pu|+| po|||ou. F. A |e|+||.e|, ,rre|||c |+|p|, de||ued |e|ou W||| +u ecceu|||c, ro|e
|e+.||, p|reu|ed +|e+ (|+|e|o|d |e|ou).
C
F
8
0
and extiemities; also (1:1) of laigei le-
sions (>6 mm) and all lesions that have
some vaiiegation. Most ieliable method
is digitalized deimoscopy, which should
be available in eveiy pigmented lesion
and melanoma centei. Patients should
be given coloi-illustiated pamphlets
that depict the clinical appeaiance of
DN, malignant melanoma, and com-
mon acquiied NMN. Patients with DN
(familial and nonfamilial) should not
sunbathe and should use sunscieens
when outdoois. They should not use
tanning pailois. Family membeis of
the patient should also be examined
iegulaily.
C|\| +|e p|reu|ed |e|ou o| ||e ||u uu
+||, p|eeu| +| |||||, |+|e .+||e||e o| C|\| c+u
de.e|op +ud |ecore c||u|c+||, +pp+|eu| du||u
|u|+uc,.
C|\| r+, |e +u, |/e ||or .e|, r+|| |o .e|,
|+|e.
C|\| +|e |eu|u ue.ore|+uoc,||c ueop|+r.
noWe.e|, +|| C|\|, |e+|d|e o| |/e, r+, |e
p|ecu|o| o| r+||u+u| re|+uor+.
*
C|+u| C|\C +|e .e|, |+|e.
C0NCNIIAI Nv0MIAN0CIIC NvS (CNMN) |C|9 . 151.!!
|C|0 . |22
FI0MI0I0C
Freva|eoce Piesent in 1% of white newboins;
the majoiity <3 cm in diametei. Laigei CNMN
aie piesent in 1:2000 to 1:20,000 newboins. Le-
sions 9.9 cm in diametei have a pievalence of
1:20,000, and giant CNMN (occupying a majoi
poition of a majoi anatomic site) occui in
1:500,000 newboins.
Ae oI 0oset Piesent at biith (congenital).
Some CNMN become visible only aftei biith
(arJe ), fading in" as a ielatively laige lesion
ovei a peiiod of weeks.
Sex Equal pievalence in males and females.
kace All iaces.
FAIh0CNSIS
Congenital and acquiied nevomelanocytic
nevi aie piesumed to occui as the iesult of a
developmental defect in neuial ciest-deiived
melanoblasts. This defect piobably occuis aftei
10 weeks in uteio but befoie the sixth uteiine
month; the occuiience of the split" nevus of
the eyelid, i.e., half of the nevus on the uppei
and half on the lowei eyelid, is an indication
that nevomelanocytes migiating fiom the neu-
ial ciest weie in place in this site befoie the
eyelids split (24 weeks).
FICk 12-3 SuperIca| spreado me|aooma: arso
wtho a dysp|astc oevus I|e uppe| d+|| ||oWu po|||ou
W||| + p|u||| ||r o| ||| |e|ou | + d,p|+||c ue.u, ||e .+||e
+|ed ||ue||+c| +ud p|u| p|+que |u ||e |oWe| |+|| o| ||e |e|ou
| ||e upe|||c|+| p|e+d|u re|+uor+ (0.9rr |||c|ue) +||
|u W||||u ||e d,p|+||c ue.u.
Sma|| aod Iare CNMN CNMN have a iathei
wide iange of clinical featuies, but the following
aie typical (Figs. 12-4 and 12-5): CNMN usually
distoit the skin suiface to some degiee and aie
theiefoie a plaque with oi without coaise teimi-
nal daik biown oi black haiis (haii giowth has
a delayed onset) (Figs. 12-4B, 12-5B). Shaiply
demaicated (Fig. 12-4) oi meiging impeicepti-
bly with suiiounding skin; iegulai oi iiiegulai
contouis. Laige lesions may be woimy" oi soft
(Fig. 12-5), iaiely fiim (desmoplastic type).
Skin suiface smooth oi pebbly," mamillated,
iugose, ceiebiifoim, bulbous, tubeious, oi lob-
ulai (Fig. 12-5). These suiface changes aie
obseived moie fiequently in lesions that extend
deep into the ieticulai deimis.
Cv|vr Light oi daik biown. With deimos-
copy a fine speckling of a daikei hue with a
lightei suiiounding biown hue is seen; often
the pigmentation is folliculai. Halo" CNMN
aie iaie.
SIze Small (Fig. 12-4), laige (>20 cm), oi giant
(Fig. 12-5). Acquiied" nevomelanocytic nevi >
1.5 cm in diametei should be iegaided as piob-
ably taidive CNMN oi they iepiesent DN.
Shupe Oval oi iound.
DIstrIhutIvn v] LesIvns Isolated, disciete le-
sion in any site. Fewei than 5% of CNMN aie
multiple. Multiple lesions aie moie common in
association with laige CNMN. Numeious small
CNMN occui in patients with giant CNMN, in
whom theie may be numeious small CNMN on
the tiunk and extiemities away fiom the site of
the giant CNMN (Fig. 12-5).
very Iare ("Caot") CNMN
Giant CNMN of the head and neck may be as-
sociated with involvement of the leptomeninges
with the same pathologic piocess; this piesenta-
tion may be asymptomatic oi manifested by
seizuies, focal neuiologic defects, oi obstiuc-
tive hydiocephalus. Giant CNMN is usually a
plaque with suiface distoition, coveiing entiie
segments of the tiunk, extiemities, head, oi
neck (Fig. 12-5).
Me|aooma o CNMN
A papule oi nodule aiises within CNMN
(Fig. 12-6). Often melanoma aiises in deimal
oi subcutaneous nevomelanocytes and can be
fai advanced when detected.
Common acquiied NMN, DN, congenital blue
nevus, nevus spilus, Beckei nevus, pigmented
epideimal nevi, and caf-au-lait macules should
be consideied in the diffeiential diagnosis of
CNMN. Small CNMN aie viitually indistin-
guishable clinically fiom common acquiied
NMN except foi size, and lesions >1.5 cm
may be piesumed to be eithei taidive CNMN
oi DN.
FICk 12-4 Cooeota| oevome|aoocytc oevus . 'r+||, .+||e+|ed ||oWu p|+que ou ||e uoe. I|e
|e|ou W+ p|eeu| +| |||||. |o|e ||+| |e|ou | |+||,. 8. Coueu||+| ue.ore|+uoc,||c ue.u, |u|e|red|+|e |/e.
'|+|p|, der+|c+|ed c|oco|+|e||oWu p|+que W||| |+|p|, de||ued |o|de| |u +u |u|+u|. w||| |uc|e+|u +e,
|e|ou r+, |ecore e|e.+|ed +ud |+||, +ud .e|, d|c|e|e |+|||ue | +|o uo|ed |u ||| |e|ou.
8
hstopatho|oy Nevomelanocytes occui as
well-oideied clusteis ( |eques ) in the epideimis
and in the deimis as sheets, nests, oi coids.
J[[use n[|raon o[ sranJs o[ neome|ano-
tyes n |e |ower one-|rJ o[ |e retu|ar Jer-
ms anJ su|tus s, w|en resen, que set[t
[or CNMN . In laige and giant CNMN, the
nevomelanocytes may extend into the muscle,
bone, duia matei, and cianium.
C0kS AN0 Fk0CN0SIS
By definition, CNMN appeai at biith, but
CNMN may aiise duiing infancy (arJe
CNMN). The life histoiy of CNMN is not
documented, but CNMN have been obseived
in eldeily peisons, an age when the common
acquiied NMN have disappeaied.
Large or gan CNMN. The lifetime iisk foi
development of melanoma in laige CNMN
has been estimated to be at least 6.3%. In 50%
of patients who develop melanoma in laige
CNMN, the diagnosis is made between the ages
of 3 and 5 yeais. Melanoma that develops in a
laige CNMN has a pooi piognosis because it is
detected late.
Sma|| CNMN : The lifetime iisk of develop-
ing malignant melanoma is 1-5%. Based on the
detection of congenital nevi in association with
melanoma by means of histology and a caieful
histoiy, a significantly incieased iisk is appaient
foi developing melanoma in peisons with small
CNMN. This iisk is as high as 21-fold based on
histoiy and 3- to 10-fold based on histology.
The expected association of small CNMN and
melanoma is <1:171,000 based on chance alone.
Nonetheless, small CNMN should be consid-
eied foi piophylactic excision at pubeity if theie
aie no atypical featuies (vaiiegated coloi and
iiiegulai boideis); small CNMN with
atypical featuies should be excised im-
mediately.
MANACMNI
Surca| xcsoo The only acceptable
method. Sma|| anJ |arge CNMN. Exci-
sion, with full-thickness skin giaft, if
iequiied; swing flaps, tissue expandeis
foi laige lesions. Can CNMN. Risk of
development of melanoma is significant
even in the fiist 3 to 5 yeais of age, and
thus giant CNMN should be iemoved
as soon as possible. Individual consid-
eiations aie necessaiy (size, location,
degiee of loss of function, oi amount
of mutilation). New suigical techniques
utilizing the patient's own noimal skin
giown in tissue cultuie can now be
used to facilitate iemoval of veiy laige
CNMN. Also, tissue expandeis can be
used.
FICk 12-5 Caot cooeota|
oevome|aoocytc oevus . |u ||| |+|,
||e |e|ou |u.o|.e ||e r+jo|||, o| ||e ||u,
W||| corp|e|e |ep|+cereu| o| uo|r+| ||u ou
||e |+c| +ud ru|||p|e r+||e| C|\| ou ||e
|u||oc| +ud ||||. I|e|e | |,pe||||c|o| |u
||e uppe| po|||ou. \e|+uor+ de.e|op|u |u +
|+u| C|\| | d||||cu|| |o d|+uoe e+||, |u +
e|||u o| uc| ||||, +|uo|r+| ||ue.
SCII0N 12 \E|A|0\A |kECuk'0k' A|| |k|\Ak\ CuIA|E0u' \E|A|0\A 30T
FICk 12-5 Caot cooeota| oevome|aoocytc oevus (Cootoued ) 8. C|+u| C|\| |u + 52,e+|o|d
r+|e. I|e r+|.e |,pe||||c|o| +dd |o ||e d||||cu||, o| d|+uo|u re|+uor+ |u ||| |e|ou +| ||| |+e.
FICk 12-6 Me|aooma: arso o sma|| CNMN A ||+c| p|+que ou ||e |||| o| + !o,e+|o|d |er+|e,
W||c| |+ |eeu p|eeu| |uce |||||. keceu||, + |||||, |e p|reu|ed e\ceu|||c uodu|e |+d +ppe+|ed |u |||
|e|ou. I|| | + re|+uor+.
CIASSIFICAII0N 0F MIAN0MA
I De novo melanoma
A. Melanoma in situ (MIS)
B. Lentigo maligna melanoma (LMM)
C. Supeificial spieading melanoma (SSM)
D. Nodulai melanoma (NM)
E. Acial-lentiginous melanoma (ALM)
F. Melanoma of the mucous membianes
G. Desmoplastic melanoma
II Melanoma aiising fiom piecuisois
A. Melanoma aiising in dysplastic
nevomelanocytic nevi
B. Melanoma aiising in congenital
nevomelanocytic nevi
C. Melanoma aiising in common NMN
F0k IMF0kIANI MSSACS C0NCkNINC
CIAN0S MIAN0MA
1. Me|aooma oI the Sko Is Approacho
pdemc Froportoos
Melanoma is a common malignancy and its inci-
dence is on the iise. In the United States the
lifetime iisk of invasive melanoma developing was
only 1 in 1500 in 1935; in 1992 it was 1 in 105, in
2002 it was 1 in 75, and in 2010 it is estimated that
it will be 1 in 50. In 2008, 60,000 cases of melanoma
weie iecoided and theie weie 8000 deaths fiom
melanoma in the United States; the numbei of
melanomas in the United States continues to in-
ciease by 7% pei yeai. Cutaneous melanoma cui-
iently iepiesents 5% of newly diagnosed cancei in
men and 6% in women. It is the leading fatal illness
aiising in the skin and is iesponsible foi 80% of
deaths fiom skin cancei. U.S. cancei statistics show
that melanoma had the second highest moitality
iate inciease among men 65 yeais old. On the
othei hand, deaths fiom melanoma occui at a
youngei age than deaths fiom most othei canceis,
and melanoma is among the most common types
of cancei in young adults.
2. ar|y kecootoo aod xcsoo oI Frmary
Me|aooma kesu|t o vrtua| Cure
Cuiient cutaneous melanoma education
stiesses the detection of eaily melanoma, with
Cu|+ueou re|+uor+ | ||e ro| r+||u+u|
|uro| o| ||e ||u. \e|+uor+ +||e ||or ||e
r+||u+u| ||+u|o|r+||ou o| re|+uoc,|e +|
||e de|r+|ep|de|r+| juuc||ou o| ||or ||e
ue.ore|+uoc,|e o| d,p|+||c re|+uoc,||c
ue.| o| C|\| ||+| |ecore |u.+|.e +ud re
|+|+|/e +||e| .+||ou ||re |u|e|.+|.
CIAN0S MIAN0MA |C|9 . 12
|C|0 . C+!
high cuie iates aftei suigical excision. Of all the
canceis, melanoma of the skin is the most ie-
waiding foi detection of eaily cuiable piimaiy
tumois, theieby pieventing metastatic disease
and death. Eaily accessability to physicians is
especially impoitant because cuiability is di-
iectly ielated to size and depth of invasion of
the tumoi. At the piesent time, the most ciitical
tool foi conqueiing this disease is, theiefoie,
the identification of eaily thin" melanomas by
clinical examination. Total skin examination foi
melanoma and its piecuisois should be done
ioutinely.
About 30% of melanomas aiise in a pieexist-
ing melanocytic lesion; 70% aiise in noimal
skin. Almost all melanomas show an initial
iadial giowth phase followed by a subsequent
veitical giowth phase. Since metastasis occuis
only infiequently duiing the iadial giowth
phase, detection of eaily melanomas (i.e., thin"
melanomas) duiing this phase is essential.
Theie is the paiadox that even with a iising
moitality iate, theie has been an encouiag-
ing impiovement in the oveiall piognosis of
melanoma with veiy high 5-yeai suivival iates
(appioaching 98%) foi thin (<0.75 mm) pii-
maiy melanoma and an 83% iate foi all stages.
The favoiable piognosis is entiiely attiibutable
to eaily detection.
3. A|| Fhyscaos aod Nurses have the
kespoosb|ty oI 0etecto ar|y Me|aooma
Eaily detection of piimaiy melanoma assuies
incieased suivival; advanced piimaiy melanoma
has a pooi piognosis and suivival. The suivival
iate plummets when theie is iegional metas-
tasis to lymph nodes. The seiiousness of this
disease thus places the iesponsibility on the
health caie piovidei in the pivotal iole: not to
oveilook pigmented lesions. This is especially
tiue foi the piimaiy caie physician, the nuise,
the physical theiapist, oi a health caie piovidei
who sees the total skin of the body. Theiefoie,
it is iecommended that in clinical piactice, no
mattei what is the piesenting complaint, total
examination of the body should be iequested
of all nonpigmented (i.e., white) patients at
the time of the fiist encountei and that all
IA8I 12-2 |ittpatrick NNR|SK
A moemoo|c dev|ce Ior promot|og me|aooma r|sk awareoess amoog phys|c|aos aod pat|eots. ach |etter
represeots ooe oI the major r|sk Iactors Ior me|aooma oI the sk|o.
M \o|e. +|,p|c+| (d,p|+||c ue.u) (>5)
M \o|e. corrou ro|e (uure|ou, >50)
k ked |+|| +ud ||ec|||u (o||eu ||ee pe|ou |+.e |eW o| uo ro|e)
I |u+|||||, |o |+u. ||u p|o|o|,pe | +ud ||
S 'uu|u|u. e.e|e uu|u|u epec|+||, |e|o|e +e +
k K|ud|ed. |+r||, |||o|, o| re|+uor+
IA8I 12-3 Risk |actors for the 0eve|opment of Ne|anoma
Ceue||c r+||e| (C|||2c ru|+||ou)
'||u |,pe |/||
|+r||, |||o|, o| d,p|+||c ue.| o| re|+uor+
|e|ou+| |||o|, o| re|+uor+
u|||+.|o|e| |||+d|+||ou, p+|||cu|+||, uu|u|u du||u c|||d|ood +ud |u|e|r|||eu| |u|u|u e\pou|e
|ur|e| (>50) +ud |/e (>5 rr) o| re|+uoc,||c ue.|
Coueu||+| ue.|
|ur|e| o| d,p|+||c ue.| (>5)
|,p|+||c re|+uoc,||c ue.u ,ud|ore
body iegions, including the scalp, toewebs, and
oiifices (mouth, anus, vulva), be examined. It is
helpful to question patients accoiding to a mne-
monic list of melanoma iisk (Table 12-2).
4. xamoatoo oI A|| Acqured Fmeoted
Iesoos Accordo to the A8C0 ku|e
This iule analyzes pigmented lesions accoiding
to symmetiy, boidei, coloi, diametei, giowth
and elevation (see page 310). While it does not
apply to all types of melanoma it peimits dif-
feiential diagnostic sepaiation of most melano-
mas fiom common nevi and othei pigmented
lesions.
The etiology and pathogenesis of cutaneous
melanoma aie unknown. Epidemiologic studies
demonstiate a iole foi genetic piedisposition
and sun exposuie in melanoma development.
The majoi genes involved in melanoma devel-
opment ieside on chiomosome 9p21. 25 to 40%
of membeis of melanoma-pione families have
mutations in cyclin-dependent kinase inhibitoi
2A (CDKN2) and a few families in cyclin-
dependent kinase 4 (CDK4). These aie tumoi
suppiessoi genes that piovide a iational basis
foi the link to susceptibility to melanoma.
Theie is convincing evidence fiom epidemio-
logic studies that exposuie to solai iadiation
is the majoi cause of cutaneous melanoma.
Cutaneous melanoma is a gieatei pioblem in
light-skinned whites (skin types I and II), and
sunbuins duiing childhood and inteimittent
buining exposuie in faii skin seem to have a
highei impact than cumulative UV exposuie
ovei time. Othei piedisposing and iisk factois
aie the piesence of piecuisoi lesions (dysplastic
melanocytic nevi and congenital nevomelano-
cytic nevi) and a family histoiy of melanoma
in paients, childien oi siblings. Risk factois foi
melanoma aie listed in Table 12-3.
MIAN0MA Ck0WIh FAIIkNS
Almost all melanomas show an initial iadial
giowth phase followed by a subsequent veitical
giowth phase. RaJa| grow| |ase iefeis to a
mostly intiaepideimal, pieinvasive, oi minimally
invasive giowth pattein; erta| grow| iefeis to
giowth into the deimis and thus into the vicin-
ity of vessels that seive as avenues foi metas-
tasis. Since most melanomas pioduce melanin
pigment, even pieinvasive melanomas in theii
iadial giowth phase aie clinically detectable by
theii coloi patteins. The piognostic diffeience
among the clinical types of melanoma ielates
mainly to the duiation of the iadial giowth
phase, which may last fiom yeais to decades in
lentigo maligna melanoma, fiom months to 2
yeais in supeificial spieading melanoma, and 6
months oi less in nodulai melanoma.
0AIA AN0 FACIS
Melanoma iepiesents 5% of all canceis by
incidence in males and 6% in females.
Numbei of new cases in the United States in
2008: 62,000.
U.S. lifetime iisk of developing invasive
melanoma in 2010: 1/50.
New melanoma deaths in United States, 2008:
8400.
Most fiequent sites
Whites
Male: back, uppei extiemities.
Female: back, lowei legs.
Blacks and Asians: soles, mucous membianes,
palms, nail beds.
Fiequency of melanoma by type of tumoi: su-
peificial spieading melanoma: 70%; nodulai
melanoma: 15%; lentigo maligna melanoma:
5%; acial and unclassified melanoma: 10%.
MIAN0MA kC0CNIII0N
Sx Sos oI Ma|oaot Me|aooma (A8C0 ku|e)
A symmery in shape-one-half unlike the
othei half.
B BorJer is iiiegulai-edges iiiegulaily scal-
loped, notched, shaiply defined.
C Co|or is not unifoim; mottled-haphazaid
display of colois; all shades of biown,
black, giay, ied, and white.
D Dameer is usually laige-gieatei than
the tip of a pencil eiasei (6.0 mm); oth-
eis use D foi ugly duckling" sign: lesion
is diffeient with iespect to change in size,
shape, coloi.
E E|eaon is almost always piesent and is
iiiegulai-suiface distoition is assessed
by side-lighting. Melanoma in situ and
acial lentiginous lesions initially maculai;
otheis use E foi En|argemen- a histoiy
of an inciease in the size of lesion is one
of the most impoitant signs of malignant
melanoma.
0L|h|0AL P8ShTAT|0hS 0F NLAh0NA
The clinical chaiacteiistics of the foui ma-
joi types of melanoma aie summaiized in
Table 12-4. Also discussed in this section aie
melanoma in situ and desmoplastic melanoma.
IA8I 12-4 |our Najor Iypes of Ne|anoma
Type Freg0eocy, % S|te 8ad|a| 6rowth Vert|ca| 6rowth
'upe|||c|+| 10 Au, ||e, |oWe| \ou|| |o |e|+,ed
p|e+d|u e\||er|||e, 2 ,e+|
||uu|
|odu|+| 5 Au, ||e, ||uu|, |o c||u|c+||, |rred|+|e
|e+d, uec| pe|cep||||e
|+d|+| |oW||
|eu||o 5 |+ce, uec|, do|+ \e+| \uc| de|+,ed
r+||u+ o| |+ud
re|+uor+
Ac|+| 5-0 |+|r, o|e, \ou|| |o E+||, |u|
|eu|||uou u|uuu+| ,e+| |ecou|||ou
re|+uor+ de|+,ed
I|e c||u|c+| |e+|u|e o| \|' +|e uo| +|W+,
c|e+||, p|eeu|ed. \|' | p||r+|||, + ||
|op+||o|o|c de||u|||ou, +ud ||e |e|r | ued
W|eu re|+uor+ ce|| +|e cou||ued |o ||e
ep|de|r|, +|o.e ||e |+ereu| rer||+ue,
|+||+| re|+uoc,||c +|,p|+, |,pe|p|+|+, +ud
p|e+d e|||e| occu| |u |u|e|||e +||ureu|
+|ou ||e |++| rer||+ue o| +|e d|||||u|ed
|||ou|ou| ||e ep|de|r| (p+e|o|d p|e+d).
E.e|, re|+uor+ |+|| + +u |u ||u |e|ou, |u|
\|' | c||u|c+||, d|+uo+||e ou|, W|eu ||e
|+d|+| |oW|| p|+e | |ou euou| |o| || |o |e
core .|u+||, de|ec|+||e. 'uc| |e|ou +|e ||+|,
W||||u ||e |e.e| o| ||e ||u, +ud ||u + c:|-
(||. 21) o| + r+cu|e W||| |+|e|, pe|cep||||e
e|e.+||ou (||. 23), W||| |||eu|+| |o|de|
+ud r+||ed .+||e+||ou o| co|o|. ||oWu, d+||
||oWu, +ud ||+c| o| |edd|| |oue |u| W|||ou|
|+, o| ||ue, + ||| occu| ou|, W|eu re|+
u|u (W||||u r+c|op|+e) o| re|+uoc,|e o|
re|+uor+ ce|| +|e |oc+|ed |u ||e de|r|. I|e
c||u|c+| d|||uc||ou |e|Weeu re|+uor+ |u ||u
+ud e.e|e|, +|,p|c+| d,p|+||c ue.| r+, uo|
|e po|||e. \o| |||e |uu|+uce corp+u|e +|
||e p|eeu| ||re do uo| |e+|d ||| |e|ou + +
r+||u+uc,, |u| || de||u||e|, |.
I|e c||u|c+| co||e|+||ou o| \|' +|e |-
| c|c (||. 21) +ud ||+| -|:c|
-c! -|cc (||. 23) +ud ||ee +|e
d|cued |u ||e |epec||.e ec||ou |e|oW.
MIAN0MA IN SII (MIS) |C|9 . 2!2
|C|0 . |02
FICk 12-T Me|aooma o stu: |eoto ma|oa A |+|e, .e|, |||eu|+| +ud +,rre|||c r+cu|e ou ||e
p|e+u||cu|+| |e|ou o| + 13,e+|o|d r+|e. I|e|e | |||||u .+||e+||ou o| p|reu|+||ou (|+u, ||oWu, d+|| ||oWu,
||+c|).
I|e |e+| corrou (<5) o| ||e |ou| p||uc|p+|
re|+uor+ |,pe o| W|||e pe|ou ||e o||
e| +|e upe|||c|+| p|e+d|u re|+uor+ (''\),
uodu|+| re|+uor+ (|\), +ud +c|+| |eu|||uou
re|+uor+ (A|\)|.
|| occu| |u o|de| pe|ou ou ||e ro| uu
e\poed +|e+-||e |+ce +ud |o|e+|r.
'uu|||| | ||e ro| |rpo||+u| p+||oeu|c |+c|o|
|u |\\.
|\\ +|W+, |+|| + |-| c|c (|\), W||c|
|ep|eeu| + r+cu|+| |u||+ep|de|r+| ueop|+r
+ud | +u \|' (||. 21). |\ | ||u uo| + p|ecu|
o| |u| +u e.o|.|u |e|ou o| re|+uor+.
|oc+| p+pu|+| +ud uodu|+| +|e+ |u+| + W||c|
||or ||e |+d|+| |o ||e .e|||c+| |oW|| p|+e +ud
||u |u.+|ou |u|o ||e de|r|, ||e |e|ou | uoW
c+||ed |\\ (|r+e 2).
|o| ||e ro| |rpo||+u| c||u|c+| c|+|+c|e||||c, ee
I+||e 2+.
INIIC0 MAIICNA MIAN0MA (IMM) |C|9 . 2!2
|C|0 . |02
FI0MI0I0C
Ae oI 0oset Median age 65.
kace Raie in biown- (e.g., Asians, East
Indians) and extiemely iaie in black-skinned
(Afiican Ameiicans and Afiicans) peisons.
Highest incidence in whites and skin photo-
types I, II, and III.
Iocdeoce 5% of piimaiy cutaneous melanomas.
Fredsposo Factors Same factois as in sun-
induced nonmelanoma skin cancei: oldei pop-
ulation, outdooi occupations (faimeis, sailois,
constiuction woikeis).
FAIh0CNSIS
In contiast to SSM and NM, which appeai
to be ielated to inteimittent high-intensity
sun exposuie and occui on the inteimittently
exposed aieas (back and legs) of young oi mid-
dle-aged adults, LM and LMM occui on the
face, neck, and doisa of the foieaims oi hands
(Table 12-4); fuitheimoie, LM and LMM occui
almost always in oldei peisons with evidence
of heavily sundamaged skin (deimatoheliosis).
The evolution of the lesion most cleaily ieveals
the tiansition fiom the iadial to the veitical
giowth phase and fiom a clinically iecognizable
MIS to invasive melanoma (Image 12-1).
LMM veiy slowly evolves fiom LM ovei a pe-
iiod of seveial yeais, sometimes up to 20 yeais.
Theie is piactically always a backgiound of
deimatoheliosis.
Sko Iesoos LentIgv Mu|Ignu Unifoimly
[|a, macule (Fig. 12-7); 0.5 cm oi laigei, up to
20 cm (Fig. 12-9A). Usually well defined, in some
aieas also bluiied boideis oi highly iiiegulai
IMAC 12-1 Ieoto ma|oa me|aooma.
|||u||+|ed ou ||e |||| | + |+|e, .+||e+|ed, ||ec||e
|||e r+cu|e (uo| e|e.+|ed +|o.e ||e p|+ue o| ||e ||u)
W||| |||eu|+| |o|de|, ||e |eu +|e+ |oW |uc|e+ed
uur|e| o| re|+uoc,|e, uu+||, +|,p|c+| +ud ||/+||e,
+ud +|e d|||||u|ed |u|e |||e +|ou ||e |++| |+,e|, +|
ce||+|u p|+ce |u ||e de|r|, r+||u+u| re|+uoc,|e
|+.e |u.+ded +ud |o|red p|or|ueu| ue|. A| ||e
|e|| | + |+|e uodu|e ||+| | corpoed o| ep|||e||o|d
ce|| |u ||| |||u||+||ou, ||e uodu|e o| +|| |ou| r+|u
u||,pe o| re|+uor+ +|e |ud|||uu||+||e ||or
e+c| o||e|.
8
FICk 12-8 Me|aooma o stu, superIca| spreado type . B+|e|, e|e.+|ed p|+que ou ||e +|r o| +
15,e+|o|d W|||e r+|e W+ |||| uo|ed 5 ,e+| p|e.|ou|,, |+du+||, |uc|e+|u |u |/e. I|e |e|ou | +,rre|||c
+ud ||e|e | +|o +,rre||, |u ||e d|||||u||ou o| co|o| ||+| | .+||e+|ed +ud |oW d+||||oWu pec| ++|u| +
|+u |+c||ouud. |e|r+|op+||o|o, o| ||e |e|ou |oWed + upe|||c|+| p|e+d|u re|+uor+ |u ||u. 8. Au +|ro|
o.+|, |+|e|, e|e.+|ed r+|| p|+que ||+| |+ + |e|+||.e|, |eu|+| |o|de| |u| | |||||u W||| |e+|d |o ||e .+||e+||ou
|u co|o|. |+u, d+|| ||oWu, +ud e.eu ||+c| W||| +u o|+ue po|||ou ou ||e ||||. |e|r+|op+||o|o, ++|u |oWed
\|' W||| + p+e|o|d |oW|| p+||e|u o| |u||+ep|de|r+| re|+uor+ ce||.
FICk 12-9 Ieoto ma|oa . A .e|, |+|e |eu||o r+||u+ ou ||e |||| c|ee| W||| ||e |,p|c+| .+||e+
||ou |u co|o| (|+u, ||oWu, ||+c|) +ud ||||, |||eu|+| |+pe. I|e |e|ou | ||+|, r+cu|+|, +ud ||u |ep|eeu| |u ||u
re|+uor+. 8. I|e c|+|c+||, r+cu|+| |eu||o r+||u+ | ||||, |||eu|+| |u |+pe +ud .+||e+|ed |u co|o|. noW
e.e|, ||e|e | + ||u|| corpoueu| +ud + |+|e p|u| uodu|e |u ||e |u||+o||||+| |e|ou, |ud|c+||u + W||c| ||or ||e
|+d|+| |o ||e .e|||c+| |oW|| p|+e +ud ||u |u.+|.eue. ||e |e|ou | uoW c+||ed |eu||o r+||u+ re|+uor+.
8
boideis, often with a notch; geogiaphic" shape
with inlets and peninsulas (Fig. 12-9B). Eaily
lesions tan, advanced lesions: stiiking vaiiations
in hues of biown and black (speckled), appeais
like a stain" (Fig. 12-7); haphazaid netwoik of
black on a backgiound of biown (Fig. 12-9).
No hues of ied and blue.
LentIgv Mu|Ignu Me|unvmu The clinical
change that indicates the tiansition of LM to
LMM is the appeaiance of vaiiegated ied, white,
and blue and of papules, plaques, oi nodules
(see Fig. 12-9B). Thus LMM is the same as LM
|us (1) giay aieas (indicate focal iegiession),
and blue aieas indicating deimal pigment
(melanocytes oi melanin)], and (2) papules oi
nodules, which may be blue, black, oi pink (Fig.
12-9B). Raiely, LMM may be nonpigmented. It
is then skin-coloied and patchy ied and clini-
cally not diagnosable (see Fig. 12-15).
DIstrIhutIvn Single isolated lesion on the sun-
exposed aieas: foiehead, nose, cheeks, neck, foie-
aims, and doisa of hands; iaiely on lowei legs.
0ther Sko Chaoes o Areas oI Iumor Sun-
induced changes: solai keiatosis, fieckling,
telangiectasia, thinning of the skin, i.e., deima-
toheliosis.
Ceoera| Medca| xamoatoo Check foi ie-
gional lymphadenopathy.
0ermatopatho|oy LM shows incieased num-
beis of atypical melanocytes distiibuted in a
single layei along the basal layei and above
the basement membiane of an epideimis
that shows elongation of iete iidges. Atypi-
cal melanocytes aie usually singly dispeised
but may also aggiegate to small nests and
extend into the haii follicles, ieaching the mid-
deimis, even in the pieinvasive stage of LM.
In LMM, they invade the deimis (veitical
giowth phase) and expand into the deepei tis-
sues (Image 12-1).
IA8I 12-5 Cutaneous Ne|anoma: Stae Croupin and Pronosis
Stage 0||o|ca| Stag|og Patho|og|c Stag|og S0rv|va|,%
I | \ I | \
0 I| |0 \0 I| |0 \0
|A I+ |0 \0 I+ |0 \0 95
|B I| |0 \0 I| |0 \0 90
I2+ |0 \0 I2+ |0 \0
||A I2| |0 \0 I2| |0 \0 13
I!+ |0 \0 I!+ |0 \0
||B I!| |0 \0 I!| |0 \0 o5
I++ |0 \0 I++ |0 \0
||C I+| |0 \0 I+| |0 \0 +5
||| Au, I | \0
|||A I-++ |+ \0 o0
I-++ |2+ \0
|||B I-+| |+ \0
I-+| |2+ \0
I-++ || \0 52
I-++ |2| \0
I-++/| |2c \0
|||C I-+| || \0
I-+| |2| \0 2o
Au, I |! \0
|\ Au, I Au, | Au, \ Au, I Au, | Au, \ 1.5-
'ou|ce. Ad+p|ed ||or C\ B+|c| e| +|. l C||u 0uco| 9.!o22-!+, 200.
vareate Iao-8rowo Macu|e[Fapu|e[Nodu|e
Se|orr|et |eraoses may be daik but aie exclu-
sively papules oi plaques and have a chaiac-
teiistic stippled suiface, often with a veiiucous
component, i.e., a waity" but gieasy suiface that,
when sciatched, exhibits fine scales . So|ar |engo ,
although maculai, does not exhibit the intensity
oi vaiiegation of biown, daik biown, and black
hues seen in LM. Deimoscopy is essential.
Fk0CN0SIS
Summaiized in Tables 12-5 and 12-6.
MANACMNI
See also page 332.
1. Veiy eaily LM lesions: Imiquimod.
2. Excise with 1-cm beyond the clinically vis-
ible lesion wheie possible and piovided the
'upe|||c|+| p|e+d|u re|+uor+ (''\) | ||e
ro| corrou re|+uor+ (10) |,pe |u pe|ou
W||| W|||e ||u.
|| +||e ro| ||equeu||, ou ||e uppe| |+c| +ud
occu| + + rode|+|e|, |oW|oW|u |e|ou o.e|
+ pe||od up |o 2 ,e+|.
''\ |+ + d|||uc||.e ro|p|o|o,. +u e|e.+|ed,
||+| |e|ou (p|+que). I|e p|reu| .+||e+||ou o|
''\ | |r||+| |o, |u| ro|e |||||u ||+u, ||e
.+||e|, o| co|o| p|eeu| |u ro| |\\. I|e co|o|
d|p|+, | + r|\|u|e o| ||oWu, d+|| ||oWu, ||+c|,
||ue, +ud |ed, W||| |+|e|+, o| |+, |e|ou |u
+|e+ o| |uro| |e|e|ou.
|o| ro| |rpo||+u| c||u|c+| c|+|+c|e||||c, ee
I+||e 2+.
SFkFICIAI SFkA0INC MIAN0MA |C|9 . 2!2
|C|0 . |02
FI0MI0I0C
Ae oI 0oset 30 to 50 (median, 37) yeais of
age.
Sex Slightly highei incidence in females.
kace In woild suiveys, white-skinned pei-
sons oveiwhelmingly piedominate. Only 2%
biown- oi black-skinned. Fuitheimoie, biown
and black peisons have melanomas usually oc-
cuiiing on the extiemities; half of biown and
black peisons have piimaiy melanomas aiising
on the sole of the foot (see below).
Iocdeoce SSM constitutes 70% of all melano-
mas aiising in white peisons.
Fredsposo aod ksk Factors (see Iab|e 12-3)
In oidei of impoitance these aie resente o[ re-
tursor |esons (DN, CNMN; pages 300 and 304);
[am|y |sory of melanoma in paients, childien,
oi siblings; |g| s|n to|or (skin phototypes
I and II); and sunbuins, especially duiing pieado-
lescence. Especially incieased incidence in young
uiban piofessionals, with a fiequent pattein of
inteimittent, intense sun exposuie (weekend-
eis") oi wintei holidays neai the equatoi.
FAIh0CNSIS
In the eaily stages of giowth theie is an in-
tiaepideimal, oi iadial, giowth phase duiing
which tumoiigenic pigment cells aie confined
to the epideimis and thus cannot metastasize.
At this stage SSM is an MIS (Fig. 12-8 and
Image 12-2). This giace peiiod" of the iadial
giowth phase, with potential foi cuie, is fol-
lowed by the invasive veitical giowth phase, in
which malignant cells consist of a tumoiigenic
nodule that veitically invades the deimis with
potential foi metastasis (Image 12-2).
IA8I 12-6 8Year Surviva| Rates for
Patients with C|inica| Stae | Ne|anoma (|n
the Vertica| Crowth Phase) Based on Iumor
Ihickness
Th|ckoess, mm 8-Year S0rv|va| 8ate, %
<0.1o 9!.2
0.1o-.o9 35.o
.10-!.o0 59.3
>!.o0 !!.!
'ou|ce. Ad+p|ed ||or wn C|+|| l| e| +|. l |+|| C+uce| |u| 3.39!,
939.
flat component does not involve a majoi
oigan. Use of Wood lamp and deimoscopy
help in defining boideis.
3. Sentinel node to be done in lesions >1.0 mm
in teims of thickness.
The pathophysiology of SSM is not yet undei-
stood. Ceitainly, in some consideiable numbei
of SSMs, sunlight exposuie is a factoi, and SSM
is ielated to occasional buists of iecieational
sun exposuie duiing a susceptible peiiod (<14
yeais). About 10% of the SSMs occui in high-
iisk families. The iest of the cases may occui
spoiadically among peisons without a specific
genetic iisk.
The usual histoiy of SSM is a change in a pie-
viously existing pigmented lesion (mostly a
DN). It should be noted, howevei, that 70% of
melanomas aiise in noimal" skin, but since ini-
tial giowth is slow and melanomas often occui
in peisons with many nevi, an eaily SSM may be
mistaken foi a pieexisting nevus by the patient.
Often, a patient may offei a histoiy of having
had a mole at that paiticulai site since child-
hood (as long as I can iemembei"), but when
a photogiaph of that paiticulai age peiiod and
site is ietiieved fiom a family album, no such
mole" can be detected.
The patient oi a close ielative may note a
giadual daikening in one aiea of a mole" (see
Figs. 12-3, 12-8) oi a change in shape; and
as the daik aieas inciease theie will develop
vaiiegation of coloi with mixes of biown, daik
biown, and black. Also, the boideis of a pievi-
ously iegulaily shaped lesion may become ii-
iegulai with pseudopods and a notch.
With the switch fiom the iadial to a veitical
giowth phase (Image 12-2), and thus invasion
into the deimis, theie is the clinical appeai-
ance of a papule and latei nodule on top of
the slightly elevated plaque of an SSM. Since
many SSMs initially have the potential foi a
tumoi-infiltiating lymphocyte (TIL)-mediated
iegiession, albeit only paitial, othei aieas of the
SSM plaque may sink to the level of suiiound-
ing noimal skin and the coloi mixes of biown
to black aie expanded by the addition of ied,
white, and the tell-tale blue and blue-giay.
Sko Iesoos (Figs. 12-10 and 12-11)
SSM is the lesion to which the ABCDE iule
(page 310) best applies. Initially a veiy flat
plaque 5-12 mm oi smallei (Fig. 12-8); oldei
lesions, 10-25 mm (Fig. 12-10). Asymmetiic
(one half unlike the othei) (Figs. 12-10, B,
and C) oi oval with iiiegulai boideis (Fig. 12-
10D) and often with one oi moie indentations
(notches) (Figs. 12-10 and 12-11). Shaiply
defined. Daik biown, black, with admixtuie of
pink, giay, and blue-giay hues-with maiked
vaiiegation and a haphazaid pattein. White
aieas indicate iegiessed poitions (Figs. 12-10C
IMAC 12-2 SuperIca| spreado
me|aooma I|e |o|de| | |||eu|+| +ud
e|e.+|ed |||ou|ou| || eu|||e|,, ||op, o|
||| p|+que u||ouud|u ||e |+|e uodu|e
|oW + p+e|o|d d|||||u||ou o| |+|e
re|+uoc,|e |||ou|ou| ||e ep|de|r| |u
ru|||p|e |+,e|, occu|||u |u|, o| |u ue|,
+ud uu||o|r|, +|,p|c+|. 0u ||e |e|| | + |+|e
uodu|e, +ud c+||e|ed |||ou|ou| ||e u|
|ouud|u po|||ou o| ||e p|+que +|e r+||e|
p+pu|+| +ud uodu|+| +|e+. I|e uodu|e r+,
+|o |oW ep|||e||o|d, p|ud|e ce|| o| r+||
r+||u+u| re|+uoc,|e + |u |eu||o r+||u+
re|+uor+ +ud uodu|+| re|+uor+.
and D). An SSM is thus a flat plaque with all
shades of biown to black plus the Ameiican
flag oi the tiicoloie (ied, blue, white) (Fig.
12-10D). No |engn gmeneJ |eson |as |ese
t|aratersts. As the veitical giowth phase
piogiesses, nodules appeai; eventually eiosions
and even supeificial ulceiation develop (Figs.
12-11C and D).
DIstrIhutIvn Isolated, single lesions; multiple
piimaiies aie iaie. Back (males and females);
legs (females, between knees and ankles); an-
teiioi tiunk and legs in males; ielatively fewei
lesions on coveied aieas, e.g., buttocks, lowei
abdomen, bia aiea.
0ermoscopy Incieases diagnostic accuiacy by
ovei 50%.
Ceoera| xamoatoo Always seaich foi en-
laiged iegional nodes.
0ermatopatho|oy Malignant melanocytes
expand in a pagetoid pattein, i.e., in multiple
layeis within the epideimis (if confined to the
epideimis, the lesion is an MIS) and supeifi-
cial papillaiy body of the deimis-the iadial
giowth phase. They occui singly and in nests
(see Image 12-2) and aie S-100 and HMB-45 posi-
tive. In the veitical giowth phase, piesenting clini-
cally as small nodules, they expand fuithei into
the ieticulai deimis and beyond (Image 12-2). Foi
miciostaging see Table 12-7 and p. 331.
FICk 12-10 SuperIca| spreado me|aooma, rada| rowth phase . A ||+||opped, e|e.+|ed,
+,rre|||c +ud |||eu|+| p|+que W||| .+||e+|ed co|o| (||oWu, ||+c|) ou ||e ||uu| W||| |+|p|, der+|c+|ed
r+||u. I|e u||+ce | +|o |||eu|+| W||| + co|||e|oue p+||e|u. 8. Au +,rre|||c, ||+| p|+que W||| |||eu|+| +ud
|+|p|, de||ued r+||u +ud + co|||e|oue|||e u||+ce. I|e re|+u|u p|reu|+||ou |+ue ||or |||| ||oWu |o
d+|| ||oWu, ||+c|, +ud ||e|e +|e ||||e| +|e+ |u|e|pe|ed. C. A ||||, |||eu|+| |e|ou W||| d+||||oWu |o ||u
||||+c| p+pu|e |o|r|u + ||u +|ouud + W|||e r+cu|+| +|e+ W||| + ceu||+| ||oWu|| |o ||u|| p+pu|e. I|| W|||e
+|e+ r+|| pou|+ueou |e|e|ou. 0. A |e|+||.e|, ,rre|||c |u| |+|e (3 cr) p|+que W||| |+|p|, de||ued +ud
uo|c|ed |o|de| +ud + cou|de|+||e .+||e+||ou o| co|o|. ||+c|, ||ue, |ed, +ud W|||e.
C
8
0
C0kS AN0 Fk0CN0SIS
Left untieated, SSM develops deep invasion
(veitical giowth) ovei months to yeais. Piogno-
sis is summaiized in Tables 12-5 and 12-6.
0IACN0SIS
Clinically accoiding to the ABCDE iule, veii-
fied by deimoscopy. In case of doubt, |osy ;
total excisional biopsy with naiiow maigins is
optimal biopsy pioceduie. Incisional oi punch
biopsy acceptable when total excisional biopsy
cannot be peifoimed oi when lesion is laige,
iequiiing extensive suigeiy to iemove the entiie
lesion.
MANACMNI
Surca| Ireatmeot See page 332.
IA8I 12-T Ne|anoma INN C|assification
T 0|ass|I|cat|oo Th|ckoess, mm 0|cerat|oo Stat0s
I .0 +. w|||ou| u|ce|+||ou +ud |e.e| ||/|||
c
|. w||| u|ce|+||ou o| |e.e| |\/\/I2
c
I2 .0-2.0 +. w|||ou| u|ce|+||ou
|. w||| u|ce|+||ou
I! 2.0-+.0 +. w|||ou| u|ce|+||ou
|. w||| u|ce|+||ou
I+ >+.0 +. w|||ou| u|ce|+||ou
|. w||| u|ce|+||ou
h 0|ass|I|cat|oo ho. oI Netastat|c hodes hoda| Netastat|c Nass
| +. \|c|ore|+|+|
|. \+c|ore|+|+|
|2 2-! +. \|c|ore|+|+|
|. \+c|ore|+|+|
c. |u||+u|| re|()/+|e||||e()
W|||ou| re|+|+||c uode
|! + o| ro|e re|+|+||c
uode, o| r+||ed
uode, o| |u||+u||
re|()/+|e||||e()
W||| re|+|+||c
uode()
N 0|ass|I|cat|oo S|te Ser0m Lactate 0ehydrogeoase
\+ |||+u| ||u, |o|r+|
u|cu|+ueou,
o| uod+| re|+|+e
\| |uu re|+|+e |o|r+|
\c A|| o||e| .|ce|+| re|+|+e |o|r+|
Au, d||+u| E|e.+|ed
re|+|+|
c
C|+|| |e.e| |, |u||+ep|de|r+|, |e.e| ||, |u.+de p+p|||+|, de|r|, |e.e| |||, |||| p+p|||+|, de|r|, |e.e| |\, |u.+de |e||cu|+| de|r|, |e.e| \, |u.+de
u|cu|+ueou |+|.
'ou|ce. Ad+p|ed ||or C\ B+|c| e| +|. l C||u 0uco| 9.!o!5, 200.
FICk 12-11 SuperIca| spreado me|aooma, vertca| rowth phase . Au ou|, r|u|r+||, |||eu|+|
p|+que W||| .+||e+|e co|o| (||oWu, ||+c|). |u ||e ceu|e| ||e|e | + r+|| ||+c|, dore|+ped uodu|e. I|| | ||e
W||c| |o ||e .e|||c+| |oW|| p|+e. 8. Au |||eu|+| .e|, ||+| p|+que W||| uo|c|ed |o|de| +ud ||||, .+||e+|ed
co|o| (|+u, ||oWu, ||+c|, +ud |ed). '|||||, o|| ceu|e| ||e|e | + |+|e p+|||+||, c|u|ed uodu|e (.e|||c+| |oW||
p|+e). C. A ||||, |||eu|+| +ud +,rre|||c p|+que W||| + co|||e|oue|||e u||+ce +ud .+||e+|ed co|o| (||+c|,
||oWu). 0u ||e |||| ||e|e | +u e\ceu|||c e|oded ||+c| |o ||ue uodu|e |ep|eeu||u ||e .e|||c+| |oW|| p|+e.
0. A ||||, |||eu|+|, +,rre|||c ||u|| |o ||+c| p|+que W||| ||oWu, |ed, +ud W|||e (|e|e|ou). 0|| ceu|e| | +u
e|oded ||+c| uodu|e (.e|||c+| |oW||).
C
8
0
|odu|+| re|+uor+ (|\) | ecoud |u ||equeuc,
+||e| ''\.
0ccu|||u |+|e|, |u r|dd|e |||e |u pe|ou W|||
W|||e ||u +ud, + |u ''\, ou ||e |e corrou|,
e\poed +|e+.
I|e |uro| ||or ||e |e|uu|u | |u ||e .e|||c+|
|oW|| p|+e (|r+e 2!).
|\ | uu||o|r|, e|e.+|ed +ud p|eeu| + + |||c|
p|+que o| +u e\op|,||c, po|,po|d o| dore|+ped
|e|ou.
I|e co|o| p+||e|u | uu+||, uo| .+||e+|ed, +ud
||e |e|ou | uu||o|r|, ||ue o| ||ue||+c| o|,
|e corrou|,, c+u |e .e|, |||||, p|reu|ed o|
uoup|reu|ed (+re|+uo||c re|+uor+).
|\ | ||e oue |,pe o| p||r+|, re|+uor+ ||+|
+||e qu||e |+p|d|, (2 rou|| |o 2 ,e+|) ||or
uo|r+| ||u o| ||or + re|+uoc,||c ue.u + +
uodu|+| (.e|||c+|) |oW|| W|||ou| +u +dj+ceu| ep|
de|r+| corpoueu|, + | +|W+, p|eeu| |u |\\
+ud ''\ (ee |r+e 2 +ud 22).
|o| ||e ro| |rpo||+u| c||u|c+| c|+|+c|e||||c, ee
I+||e 2+.
N00IAk MIAN0MA |C|9 . 2!2
|C|0 . |02
IMAC 12-3 Nodu|ar me|aooma I|| +||e +| ||e de|r+|ep|de|r+| juuc||ou +ud e\|eud .e|||c+||, |u ||e
de|r|. I|e ep|de|r| |+|e|+| |o ||e +|e+ o| ||| |u.+|ou doe uo| derou||+|e +|,p|c+| re|+uoc,|e. A |u |eu
||o r+||u+ re|+uor+ +ud upe|||c|+| p|e+d|u re|+uor+, ||e |uro| r+, |oW |+|e ep|||e||o|d ce||, p|ud|e
ce||, r+|| r+||u+u| re|+uoc,|e, o| r|\|u|e o| +|| |||ee
FI0MI0I0C
Ae oI 0oset Middle life.
kace NM occuis in all iaces, but in the Japa-
nese it occuis eight times moie fiequently
(27%) than SSM (3%).
Iocdeoce NM constitutes 15% (up to 30%) of
the melanomas in the United States.
Fredsposo aod ksk Factors See page 309
and Table 12-3.
FAIh0CNSIS
Both SSM and NM occui in appioximately the
same sites (uppei back in males, lowei legs in fe-
males), and piesumably the same pathogenetic
factois aie opeiating in NM as weie desciibed
in SSM. Foi the giowth pattein of NM, see Im-
age 12-3. The ieason foi the high fiequency of
NM in the Japanese is not known.
This type of melanoma may aiise in a pieexist-
ing nevus, but moie commonly aiises de novo
fiom noimal skin. In contiast to SSM, NM
evolves ovei a few months and is often noted
by the patient as a new mole" that was not
piesent befoie.
Sko Iesoos Unifoimly elevated bluebeiiy-
like" nodule (Figs. 12-12 and B) oi ulceiated
oi thick" plaque; may become polypoid.
Unifoimly daik blue, black, oi thundeicloud"
giay (Fig. 12-12, B); lesions may appeai pink
with a tiace of biown oi a black iim (amelanotic
NM, see Fig. 12-15C). Suiface smooth oi scaly,
eioded (Fig. 12-12C) oi ulceiated (Fig. 12-
12D). Eaily lesions aie 1-3 cm in size but
may giow much laigei if undetected. Oval
oi iound, usually with smooth, not iiiegulai,
boideis, as in all othei types of melanoma.
Shaiply defined, may be pedunculated (Fig.
12-12D).
DIstrIhutIvn Same as SSM. In the Japanese,
NM occuis on the extiemities (aims and legs).
Ceoera| Medca| xamoatoo Always seaich
foi nodes.
0ermatopatho|oy Malignant melanocytes,
which appeai as epithelioid, spindle, oi small
atypical cells, show little lateial (iadial) giowth
within and below the epideimis and invade
veitically into the deimis and undeilying sub-
cutaneous fat (see Image 12-3). They aie S-100
and usually HMB-45 positive. Foi miciostag-
ing, see page 331.
Sero|oy Seium levels of S-100 beta
and melanoma-inhibiting activity (MIA),
S-cysteinyldopa, and lactate dehydioge-
nase (LDH) levels aie maikeis foi aJanteJ
melanoma patients. LDH is to date the only
statistically significant maikei foi rogresse
disease.
0IACN0SIS
Clinical and with the help of deimoscopy. How-
evei, deimoscopy may fail in unifoimly black
lesions. In case of doubt, |osy . Total excisional
biopsy with naiiow maigins is optimal biopsy
pioceduie, wheie possible. If biopsy is positive
foi melanoma, ieexcision of site will be neces-
saiy (see Management, p. 332). Incisional oi
punch biopsy acceptable when total excisional
biopsy cannot be peifoimed oi when lesion is
laige, iequiiing extensive suigeiy to iemove the
entiie lesion.
8|ue[8|ack Fapu|e[Nodu|e NM can be con-
fused with |emangoma (long histoiy) and
yogent granu|oma (shoit histoiy-weeks) (see
Fig. 12-12C) and is sometimes almost indis-
tinguishable fiom gmeneJ |asa| te|| tart-
noma , although it is usually softei. Howevei,
any bluebeiiy-like" nodule of iecent oiigin
(6 months to 1 yeai) should be excised oi, if
laige, an incisional biopsy is mandatoiy foi
histologic diagnosis.
Fk0CN0SIS
Summaiized in Tables 12-5 and 12-6.
MANACMNI
Surca| Ireatmeot See page 332.
FICk 12-12 Nodu|ar me|aooma . A 9rr dore|+ped roo|| uodu|e W||| + ||+||e| ||oWu|| ||r
+|||u ou ||e |+c| o| + !3,e+|o|d r+|e. 8. A cr ||+c| p+pu|e ou ||e po|e||o| |||| o| + o0,e+|o|d |er+|e.
I|e |e|ou |+d |eeu p|eeu| |o| |e ||+u ,e+|. C. Au e|oded, ||eed|u, ||oWu uodu|e |+.|u + ru||oor|||e
cou||u|+||ou |.|u || + |uc|ou +ppe+|+uce. 'uc| |e|ou c+u |e r||+|eu |o| + .+cu|+| |e|ou uc| + + p,o
eu|c |+uu|or+. 0. |+|e (5 cr) |||eu|+|, ||+c|, ||eed|u uodu|e ||||u ou ||e ||u |||e + ru||oor. I|e |e|ou
|+d |oWu |o| o.e| + |+|| ,e+| +ud ||e 5o,e+|o|d r+|e p+||eu| |+d uo| eeu + p|,|c|+u ou| o| |e+| '|| r||| |e
re|+uor+.
C
8
0
I|e |e|r !-|cc |e|e| |o couuec||.e ||ue
p|o|||e|+||ou +ud, W|eu +pp||ed |o r+||u+u|
re|+uor+, dec|||e () + de|r+| ||||o||+||c
corpoueu| o| re|+uor+ W||| ou|, r|u|r+|
re|+uoc,||c p|o|||e|+||ou +| ||e de|r+|ep|de|r+|
juuc||ou, (2) ue|.eceu|e|ed upe|||c|+| r+||u+u|
re|+uor+ W||| o| W|||ou| +u +|,p|c+| |u||+ep|de|
r+| re|+uoc,||c corpoueu|, o| (!) o||e| |e|ou
|u W||c| ||e |uro| +ppe+| |o +||e |u |eu||o
r+||u+ o|, |+|e|,, |u +c|+| |eu|||uou re|+uor+
o| upe|||c|+| p|e+d|u re|+uor+.
A|o, |\ |oW|| p+||e|u |+.e |eeu uo|ed |u
|ecu||eu| r+||u+u| re|+uor+.
|\ r+, |e + .+||+u| o| |\\ |u ||+| ro| |e|ou
occu| ou ||e |e+d +ud uec| |u p+||eu| W||| de|
r+|o|e||o|.
|\ | ro|e |||e|, |o |ecu| |oc+||, +ud re|+|+|/e
||+u |\\, |oWe.e|. |\ | |+|e +ud occu| ro|e
||equeu||, |u Woreu +ud pe|ou >55 ,e+| o|d.
A| d|+uo| |\ |e|ou |+.e |eeu p|eeu| ||or
rou|| |o ,e+|. |\ | +,rp|or+||c, uu+||, uo|
p|reu|ed +ud | ||e|e|o|e o.e||oo|ed |, ||e
p+||eu|. E+||, |e|ou r+, +ppe+| + .+||e+|ed
|eu|||uou r+cu|e o| p|+que, +| ||re W|||
r+|| ||ue|+, pec| o| co|o| (||. 2!1).
|+|e| |e|ou r+, +ppe+| + de|r+| uodu|e,
+ud +|||ou| ||e, corrou|, |+c| +u, re|+u|u
p|reu|+||ou, ||e, r+, |+.e |+, |o ||ue p+pu|+|
e|e.+||ou (||. 2!3). Bo|de|, W|eu d|ce|u
|||e, +|e |||eu|+| + |u |\.
I|e d|+uo| |equ||e +u e\pe||euced de|r+|
op+||o|o||, '00 |rruuope|o\|d+epo|||.e
p|ud|e ce|| ueed |o |e |deu||||ed |u ||e r+|||\
co||+eu. n\B+5 |+|u|u r+, |e ue+||.e. A
|,p|c+| juuc||ou+| re|+uoc,||c p|o|||e|+||ou, e|||e|
|ud|.|du+| o| |oc+| ue|, occu|, |eer|||u |\.
'00po|||.e p|ud|e|+ped ce|| +|e er|edded
|u r+|||\ co||+eu ||+| W|de|, ep+|+|e ||e p|u
d|e ce|| uuc|e|. 'r+|| +|e+|e o| |,rp|oc,|e
+|e corrou|, eeu +| ||e pe||p|e|, o| |\. |eu
|o||op|r | c|+|+c|e||||c, |.e., ||||o||+||||e |u
ro| ce|| +|ouud o| W||||u eudoueu||ur o| r+||
ue|.e. 0||eu, |\ | eeu W||| + |+c||ouud o|
e.e|e o|+| d+r+e |o ||e de|r|.
I|e|e +|e r|\ed .|eW +|ou| ||e p|ouo| o|
|\. |u oue e||e, +pp|o\|r+|e|, 50 o| p+||eu|
e\pe||euced + |oc+| |ecu||euce +||e| p||r+|,
e\c||ou o| |\, uu+||, W||||u ! ,e+| o| e\c||ou,
ore p+||eu| e\pe||euced ru|||p|e |ecu||euce.
|,rp| uode re|+|+| occu| |e o||eu ||+u
|oc+| |ecu||euce. |u oue e||e, 20 de.e|oped
re|+|+e, +ud |\ W+ |e+|ded + + ro|e +
|e|.e |uro| ||+u |\\.
|o| r+u+ereu| ee p+e !!2.
0SM0FIASIIC MIAN0MA (0M)
FICk 12-13 0esmop|astc me|aooma . Are|+uo||c derop|+||c re|+uor+. A ||+|, ||uco|o|ed uod
u|e W||| + pec| o| ||oWu |u ||e ceu|e| ||+| +ppe+|ed ou ||e |o|e|e+d o| ||| +3,e+|o|d |er+|e. 8. A ||+| uodu|e
W||| ||u|||ed +ud ||oWu po|||ou |u +u e|de||, r+|e, |e|ou o||eu +|e u||ouuded |, + r+cu|+| po|||ou |eer
|||u |eu||o r+||u+.
8
FI0MI0I0C
Ae oI 0oset Median age is 65.
Iocdeoce 7 to 9% of all melanomas; in whites,
2 to 8%.
Sex Male:female iatio, 3:1.
kace ALM is the piincipal melanoma in the
Japanese (50-70%) and in Ameiican and sub-
Sahaian Afiican blacks.
FAIh0CNSIS
The pigmented macules that aie fiequently
seen on the soles of Afiican blacks could be
compaiable with DN. ALM has a similai giowth
pattein as LMM.
ALM is slow giowing (about 2.5 yeais fiom
appeaiance to diagnosis). The tumois occui
on the volai suiface (palm oi sole) and in theii
iadial giowth phase may appeai as a giadually
enlaiging stain." ALM as subungual (thumb oi
gieat toe) melanoma appeais fiist in the nail
bed and involves, ovei a peiiod of 1 to 2 yeais,
the nail matiix, eponychium, and nail plate. In
the veitical giowth phase nodules appeai; often
theie aie aieas of ulceiation, and nail defoimity
and shedding of the nail may occui.
Sko Iesoos Acra| aod Fa|m[So|e Maculai
oi slightly iaised lesion in the iadial giowth
phase (Fig. 12-14), with focal papules and
nodules developing duiing the veitical giowth
phase. Maiked vaiiegation of coloi including
biown, black, blue, depigmented pale aieas (Fig.
12-14). Iiiegulai boideis as in LMM; usually
well defined but not infiequently ill defined.
This type of ALM occuis on soles, palms, doisal
and palmai/plantai aspects of fingeis and toes
(Figs. 12-14C and D).
Subuoua| Subungual macule beginning at
the nail matiix and extending to involve the
Ac|+| |eu|||uou re|+uor+ (A|\) | + pec|+|
p|eeu|+||ou o| cu|+ueou re|+uor+ +|||u ou
||e o|e, p+|r, +ud ||ue|u+|| o| |oeu+|| |ed.
A|\ occu| ro| o||eu |u A|+u, u|'+|+|+u
A|||c+u, +ud A|||c+u Are||c+u, corp|||u
50-10 o| ||e re|+uor+ o| ||e ||u |ouud |u
||ee popu|+||ou.
|| occu| ro| o||eu |u o|de| r+|e ( o0 ,e+|)
+ud o||eu |oW |oW|, o.e| + pe||od o| ,e+|.
I|e de|+, |u de.e|opreu| o| ||e |uro| | ||e |e+
ou ||ee |uro| +|e o||eu d|co.e|ed ou|, W|eu
uodu|e +ppe+| o|, |u ||e c+e o| u+|| |u.o|.e
reu|, ||e u+|| | |ed, ||e|e|o|e, ||e p|ouo| |
poo|.
ACkAI INIICIN0S MIAN0MA |C|9 . 2!2
|C|0 . |02
nail bed and nail plate. Papules, nodules, and
destiuction of the nail plate may occui in the
veitical giowth phase (Figs. 12-14B, 33-14).
Daik biown oi black pigmentation that may
involve the entiie nail and suiiounding skin
looking like LM (Figs. 12-14 and B). As the
lesion switches to the veitical giowth phase, a
papule oi nodule appeais and the nail is shed
(Figs. 12-14 and B). Often the nodules oi
papules aie unpigmented. Amelanotic ALM is
often oveilooked foi months and, since theie
aie no pigmentaiy changes, may fiist piesent as
nail dystiophy.
ALM (plantai type) is not infiequently iegaided
as a plantai wait" and tieated as such. Deimos-
copy is of decisive help. Also, often misdiag-
nosed as tinea nigia.
Subuoua| 0sco|oratoo ALM (subungual) is
usually consideied to be tiaumatic bleeding
undei the nail, and subungual hematomas may
peisist foi ovei 1 yeai; howevei, usually the
whole pigmented aiea moves giadually foiwaid.
Distinction of ALM fiom subungual hemoi-
ihage can easily be made by deimoscopy. With
the destiuction of the nail plate, the lesions
aie most often iegaided as fungal infection."
When nonpigmented tumoi nodules appeai,
they aie misdiagnosed as pyogenic gianuloma.
0ermatopatho|oy The histologic diagnosis
of the iadial giowth phase of the volai type of
ALM may be difficult and may iequiie laige
incisional biopsies to piovide foi multiple sec-
tions. Theie is usually an intense lymphocytic
inflammation at the deimal-epideimal junc-
tion. Chaiacteiistic laige melanocytes along the
basal cell layei may extend as laige nests into the
deimis, along ecciine ducts. Invasive malignant
melanocytes aie often spindle shaped, so that
ALM fiequently has a desmoplastic appeaiance
histologically.
Fk0CN0SIS
The volai type of ALM can be deceptive in its
clinical appeaiance, and flat" lesions may be
quite deeply invasive. Five-yeai suivival iates
aie <50%. The subungual type of ALM has
a bettei 5-yeai suivival iate (80%) than does
the volai type, but the data aie piobably not
accuiate. Pooi piognosis foi the volai type of
ALM may be ielated to inoidinate delay in the
diagnosis.
MANACMNI
In consideiing suigical excision, it is impoitant
that the extent of the lesion be asceitained by
viewing the lesion with deimoscopy. Subun-
gual ALM and volai-type ALM: amputation
toe(s), fingei(s)]; volai and plantai ALM: wide
excision with split skin giafting. Sentinel lymph
node pioceduie necessaiy in most cases (see
Management of Melanoma," page 332).
FICk 12-14 Acra| |eotoous me|aooma . Au A|\ +|||u ou ||e ||ur|. |eu|||uou corpoueu| ou
||e do|+| ||u o| ||e ||ur|. r+cu|+|, |+|p|, +ud |||de||ued ||oWu +ud |e,||u|| po|. 'u|uuu+| +ud d||+|
u|ce|+|ed uodu|+| corpoueu|. 8. I|e |uro| |+ |ep|+ced ||e eu|||e u+|| |ed +ud u||ouud|u ||u. r+cu|+| +ud o|
.+||e+|ed co|o| |eer|||u + |eu||o r+||u+. I|e u+|| |+ |eeu |ed. I|| | A|\ ||+| |+ |ed |o de||uc||ou
o| ||e u+|| r+|||\ +ud W+ |||| d|+uoed + u+|| d,||op|,. C. A|\ ou ||e |ee|. I|e|e | + ||||, .+||e+|ed
r+cu|+| corpoueu|-||oWu |o |+, +ud ||+c|, ||e uodu|+| corpoueu| | |,pe||e|+|o||c, |edd||, +ud u|ce|+|ed.
0. |eu||o r+||u+ re|+uor+ ou ||e o|e. I|| | +u +d.+uced |e|ou W||| + r+cu|+| corpoueu| +ud + |edd||,
u|ce|+|ed uodu|e. I|e |e|ou re+u|ed 0 rr |u dep||, +ud ||e|e We|e eu|+|ed |uu|u+| |,rp| uode.
C
8
0
A|| |,pe o| re|+uor+ c+u |e +re|+uo||c.
'|uce ||e, dou'| |+.e ||e c|+|+c|e||||c p|reu|
r+||e| ||e, +|e + d|+uo||c c|+||eue (||. 2
5).
noWe.e|, o||eu ||e|e +|e p|reu|ed c|oue |u
||e |uro|, W||c| |e.e+| || u+|u|e + + re|+uor+
(||. 253 +ud C).
|u ro| c+e ou|, ||op, W||| |e.e+| ||e co||ec|
d|+uo| (||. 251 +ud |).
AMIAN0IIC MIAN0MA |C|9 . 2!2
|C|0 . |02
FICk 12-15 Ame|aootc me|aooma . Are|+uo||c |\\. I|e |ed uodu|e W+ o|| +ud d|+uoed +
p,oeu|c |+uu|or+ +ud W+ e\c|ed. n||op+||o|o, |e.e+|ed re|+uor+ +ud u|equeu| puuc| ||op|e pe|
|o|red |u ||e e|,||er+|ou ||u o| ||e c|ee| |e.e+|ed |eu||o r+||u+ (|\). I|e ou|||ue o| ||e |\ |e|ou +
de|e|r|ued |, |u|||e| puuc| ||op|e +|e r+||ed W||| |eeu c||c|e. |o|e ||+| o.e| ||e r+ud|||e |e|ou | +|o
uodu|+| (.e|||c+|) |oW||. 8. Are|+uo||c upe|||c|+| p|e+d|u re|+uor+. I|e ||ue u+|u|e o| ||| |ed uodu|e |
|e.e+|ed |, ||e ||ue c|eceu| +| || |+e +ud ||e .+||e+|ed ||oWu|ed p|+que W||| W||c| || | cou||uou.
C. Are|+uo||c uodu|+| re|+uor+. I|| c|e||,|ed uodu|e |+ + ||oWu, r+cu|+| e\|eu|ou +| 2 +ud + o'c|oc|
+ud + ecoud, ruc| r+||e| |ed uodu|e +| 9 o'c|oc|, |.|u +W+, ||e co||ec| d|+uo|. 0. Are|+uo||c A'\ ou ||e
|ee|. I|| c|e||,|ed |e|ou W+ c||u|c+||, d|+uoed + ecc||ue po|or+. B|op, |e.e+|ed deep|, |u.+d|u A|\.
C
8
0
\+||u+u| re|+uor+ +|||u |u ||e ruco+|
ep|||e||+| ||u|u o| ||e |ep||+|o|, ||+c| +ud +
||o|u|e||u+| +ud eu||ou||u+|, ||+c| +|e .e|, |+|e,
W||| +u +uuu+| |uc|deuce o| 0.5 pe| 00,000
|ud|.|du+|.
\+jo| ||e o| ||e ruco+| re|+uor+ +|e ||e
.u|.+ +ud .+|u+ (+5) +ud ||e u++| +ud o|+|
c+.||, (+!).
\uco+| re|+uor+ +|e o |+|e ||+| ||e|e +|e uo
|+|e d+|+ |+e corp+|ed |o ||oe |o| cu|+ueou
re|+uor+.
I|e|e|o|e, p+||o|o|c r|c|o|+|u |+ uo| |eeu
po|||e, +ud ||e ||ue|uu|u o| ||e p|ouo| ||+|
|+ |eeu ue|u| |u cu|+ueou re|+uor+ (B|e|oW
|||c|ue) |+ o |+| uo| |eeu po|||e |u ruco+|
re|+uor+.
MAIICNANI MIAN0MA 0F Ih MC0SA |C|9 . 2!2
|C|0 . |02
Me|aoomas oI the 0ra| Cavty
Theie is a delay in diagnosis of melanoma of the
oial and nasal suifaces. Although melanosis of
the mucosa is common in blacks and East Indi-
ans, it involves the buccal and gingival mucosa
bilateially (see Disoideis of the Mouth," Sec-
tion 34); when theie is a single aiea of melanosis
(see Fig. 34-13), a biopsy should be peifoimed
to iule out melanoma; this is also tiue of pig-
mented nevi in the oial cavity, which should be
excised (see Section 34).
Me|aoomas o the Ceota|a
These melanomas mostly aiise on the glans oi
piepuce (see Fig. 35-23) and the labia minoia;
theie aie fewei on the clitoiis and the labia
majoia (see Fig. 35-24). Most tumois extend to
the vagina at the mucocutaneous boidei. They
look and evolve like LM and LMM (see Figs.
35-23, 35-24). Vulva melanomas aie often flat
like LMM with laige aieas of melanoma in situ,
and this is impoitant to asceitain in planning
excision of all the lesion to pievent iecuiience.
Deimoscopy should be used to outline the
peiipheiy of the lesion, as is done in LMM
(see Disoideis of the Genitalia, Peiineum, and
Anus," Section 35).
Aoorecta| Me|aooma
Often piesents with a localized, often polypoid
oi nodulai piimaiy tumoi, but it may also
piesent similaily to LMM.
\e|+|+||c re|+uor+ occu| |u 5-2o o| |+e |
+ud |+e || re|+uor+ (ee |e|oW).
I|e p|e+d o| d|e+e ||or ||e p||r+|, ||e uu+||,
occu| |u + |epW|e equeuce. p||r+|, re|+uor+
|e|ou+| re|+|+| (||. 21 3)
d||+u| re|+|+|.
|||+u| re|+|+| c+u occu|, ||pp|u ||e |e
|ou+| |,rp| uode +ud |ud|c+||u |er+|o
euou p|e+d.
|||+u| re|+|+e occu| +u,W|e|e |u| uu+||,
|u ||e |o||oW|u o|+u. |uu (3-!o), ||.e|
(+-29), ||+|u (2-20), |oue (-1), +ud
|u|e||ue (-1).
\o| ||equeu||,, |oWe.e|, re|+uor+ |||| p|e+d
|o d||+u| |,rp| uode, ||u (||. 213), +ud
u|cu|+ueou ||ue (+2 |o 51) (||. 21|).
|oc+| |ecu||euce occu| || e\c||ou |+ uo| |eeu
+dequ+|e (||. 2o) o| || c+u |u.o|.e ||e ||u o|
+u eu|||e |e|ou |o|| W||| +ud W|||ou| +dequ+|e
u||c+| ||e+|reu| (||. 211 C )
w|dep|e+d re|+|+| c+u +|o |e+d |o |u|e
re|+|+||c re|+uor+ ce|| |odereu| |u +|| o|+u
W||| re|+uo| o| ||e ||u (||. 23), rucou
rer||+ue, ||.e|, ||due,, |e+|| ruc|e +ud o||e|
||ue.
!-|c|c|: -|cc +||| c c, |
| |+|e, -o. || | ||e |eu|| o| re|+|+| ||or
+ re|+uor+ ||+| uude|Weu| |o|+| pou|+ueou
|e|e|ou.
!-|cc c, |c.- c |c|- -:-:- ( 0
,e+|). I|e uu+| ||re | + ,e+|, |u| ||e|e |+.e
|eeu '.e|, |+|e |ecu||euce (>5 ,e+|) |u oue
e||e +| ||e \++c|ue|| Ceue|+| nop||+|, W|||
0.012 (20 o| 21oo C+e).
|c|-| +|| c ||c, -|c|c cou||ued |o ||e
u|cu|+ueou, uou|e|ou+| |,rp| uode o| |uu
+|e ro| |||e|, |o |eue||| ||or u||c+| |u|e|.eu
||ou.
MIASIAIIC MIAN0MA
FICk 12-16 Metastatc me|aooma: recurro o excsoo scar . A p|reu|ed |e|ou ou ||e ||u o| +
!5,e+|o|d r+|e, p|eeu| |o| < 2 ,e+|. |e|r+|op+||o|o, W+ |u|||+||, |u|e|p|e|ed + + p|ud|e ce|| ('p||/) ue.u.
I|e p||r+|, |e|ou ||e W+ ||e|e|o|e uo| |ee\c|ed. 8. IWo p+pu|e +|e eeu +|ouud ||e e\c||ou ||e c+|, oue
o| W||c| |+ + ||ue||oWu co|o|. I|e |||o|o, ||or ||e e\c|ed |e|ou W+ |e.|eWed +ud |e.|ed + + upe|||c|+|
p|e+d|u re|+uor+, +ud ||e |||op+||o|o, o| ||e |Wo p+pu|e eeu |e|e W+ re|+|+||c re|+uor+.
8
8
FICk 12-1T Metastatc me|aooma . |oc+| |ecu||euce +ud |u||+u|| cu|+ueou re|+|+e +||e| e\c||ou
o| p||r+|, re|+uor+ ou ||e c+|p +ud p||| ||u |+|||u. |o|e. re|+|+e +|e |o|| |u ||e u||ouud|u ||u +ud
||e |+||. 8. Ad.+uced re|+|+e |u ||e +\|||+|, |,rp| uode +ud |u||+u|| re|+|+e o| ||e r+rr+|, ||u. I|e
p||r+|, |uro| |+d |eeu + p||c|||+c| uodu|+| re|+uor+ +ud |+d |eeu ju| |+|e|+| |o ||e ||e+| (||e c+| c+u
|||| |e eeu). |o|e ||+| |o|| ||e |u||+u|| +ud +\|||+|, uodu|e e\|eud|u |u|o ||e ||u +|e +re|+uo||c. C. \u|||p|e
re|+uor+ re|+|+e |o ||e ||u +||e| |er+|oeuou p|e+d. 0. 'u|cu|+ueou re|+uor+ re|+|+e |, |er+
|oeuou p|e+d. '|uce ||e, +|e uo| ||u|| ||e, +|e +re|+uo||c. |||r+|, +ud re|+|+||c re|+uor+ r+, d|||e| W|||
|e+|d |o p|reu|+||ou po|eu||+|.
C 0
FICk 12-18 oversa|

me|aooss due to metastatc
me|aooma . '|u|ece||
re|+|+e +|e |ouud |||ou|
ou| ||e ||u +ud rucou rer
||+ue o| ||e W|||e p+||eu| +ud
c||cu|+||u re|+|+||c re|+uor+
ce|| We|e |ouud |u ||e ||ood.
I|e u||ue W+ ||+c| (re|+uo
euu||+), +ud upou +u|op, ||e
|u|e|u+| o|+u We|e +|o ||+c|.
8. I|e p+||eu|' |+ud | |oWu
|e|de ||e |+ud o| + uu|e |o
derou||+|e ||e d|||e|euce |u
co|o|.
8
Mcrostao
Mtrosagng is done accoiding to Bieslow method.
The thickness of the piimaiy melanoma is
measuied fiom the gianulai layei of the epi-
deimis to the deepest pait of the tumoi. The
thickness of melanoma (level of invasion) is the
most impoitant single piognostic vaiiable and
thus decisive foi theiapeutic decisions (Tables
12-6, 12-7).
Claik miciostaging accoiding to tissue level
of invasion is no longei consideied a significant
piognostic vaiiable.
Seotoe| Iymph Node 8opsy
Sentinel lymph node biopsy can piedict the
piesence of clinically nondetectable metastatic
melanoma within iegional lymph nodes with
the identification of malignant cells in H&E
sections; staining foi S-100 piotein, HMB-45,
and tyiosinase.
When the nodes aie not palpable, it is not
ceitain if theie aie miciometastases; these can
be detected by the senne| noJe et|nque . The
hypothesis is that the [rs node diaining a
lymphatic basin, called the senne| noJe , can
piedict the piesence oi absence of metastasis
in othei nodes in that basin. Eithei lymphatic
mapping (LM) oi sentinel lymphadenectomy
(SL) is peifoimed on the same day with a single
injection of filteied
99m
Tc subcutaneously into
the site of the piimaiy melanoma foi piobe-
diiected LM and SL. Alteinatively, one day
aftei lymphoscintigiaphy, sentinel node biopsy
is peifoimed, guided by a gamma piobe and
blue dye also injected into the piimaiy site; the
sentinel node is subjected to histopathology and
immunohistochemistiy. LM is veiy useful in lo-
cating the diainage aieas, especially in piimaiy
laiy deimis; level III, fills papillaiy deimis; level IV,
invades ieticulai deimis; level V, invades subcutane-
ous fat.
laiy deimis; level III, fills papillaiy deimis; level IV,
invades ieticulai deimis; level V, invades subcutane-
ous fat.
'|+|u o| re|+uor+ depeud ou || I|\ C|+
|||c+||ou (p||r+|, |uro|, |e|ou+| ode, e|+
|+e, I+||e 21).
C|:c| |c o| re|+uor+ d|||e|eu||+|e |e
|Weeu |oc+|, |e|ou+|, +ud d||+u| d|e+e +ud
| |+ed ou r|c|o|+|u o| ||e re|+uor+ +ud
c||u|c+| +ud |r+|u e.+|u+||ou |o| re|+|+e.
|c|||: |c cou|| o| r|c|o|+|u o|
||e p||r+|, |uro| +ud p+||o|o|c e.+|u+||ou o|
|e|ou+| |,rp| uode (I+||e 25 +ud 2o).
'|+|u o| re|+uor+ | ||ou|, co||e|+|ed W|||
u|.|.+|.
SIACINC 0F MIAN0MA
tumois on the tiunk, which can diain on eithei
side and to both the axillaiy and inguinal lymph
nodes.
Lymph node dissection is peifoimed only if
miciometastasis is found in the sentinel node.
The sentinel node technique is also essential
in making a decision about the use of adjuvant
theiapy.
W0kkF 0F MIAN0MA
I Piimaiy melanoma: Stage I oi II (no nodes
palpated)
A. Chest ioentgenogiam, sonogiaphy of
lymph nodes
B. Livei function tests, LDH
C. Lymphatic mapping and sentinel lym-
phadenectomy in stage I thickness >1.0
mm
II Piimaiy melanoma with local-iegional dis-
ease
A. Stage III, satellites and local iecuiience
1. Complete blood count
2. Livei function tests, LDH
3. Chest ioentgenogiam
4. Ultiasound and CT scans: abdomen,
pelvis (with disease below the waist),
neck (with disease in the head and
neck); position emission tomogia-
phy (PET) scan
B. Stage IV
1. Same as foi stage III
2. CT scan of the chest
3. MRI of the biain
4. Bone scan
5. GI seiies (on the basis of symptoms)
CI0IINS F0k 8I0FS AN0 SkCICAI
IkAIMNI 0F FAIINIS WIIh MIAN0MA
I. Biopsy
A. Total excisional biopsy with naiiow
maigins-optimal biopsy pioceduie,
wheie possible.
B. Incisional oi punch biopsy acceptable
when total excisional biopsy cannot be
peifoimed oi when lesion is laige, ie-
quiiing extensive suigeiy to iemove the
entiie lesion.
C. When sampling the lesion: If iaised,
iemove the most iaised aiea; if flat, ie-
move the daikest aiea.
II. Melanoma in situ
A. Excise with 0.5-cm maigin.
III. Lentigo maligna melanoma
A. Excise with a 1-cm maigin beyond the
clinically visible lesion oi biopsy scai-
unless the flat component involves a
majoi oigan (e.g., the eyelid), in which
case lessei maigins aie acceptable.
B. Excise down to the fascia oi to the un-
deilying muscle wheie fascia is absent.
Skin flaps oi skin giafts may be used foi
closuie.
C. No node dissection is iecommended
unless nodes aie clinically palpable and
suspicious foi tumoi.
D. See iecommendation foi sentinel
node studies foi thickness >1 mm
(page 331).
||ouo| o| re|+uor+ c+u |e e|||e| e\ce||eu|
o| |+.e, depeud|u ou W|e||e| ||e |uro|
| d|+uoed e+||, o| |+|e, W|eu |e|ou+| o|
d||+u| re|+|+e |+.e occu||ed (I+||e 25).
I|| erp|+|/e ||e |rpo||+uce o| e+||, d|+
uo|, o| que||ou|u p+||eu| |o| re|+uor+
||| (I+||e 22 +ud 2!), o| c|eeu|u
|ud|.|du+| |e|ou|u |o ||| |oup, +ud o|
|o|+||od, e\+r|u+||ou o| +u, p+||eu| ee|u
+ p|,|c|+u |o| red|c+| e\+r|u+||ou. ||ouo
| |e|+||u |o |+e |oup|u |o| cu|+ueou
re|+uor+ | |oWu |u I+||e 25.
Fk0CN0SIS 0F MIAN0MA
I|e ou|, cu|+||.e ||e+|reu| o| re|+uor+ | e+||, u||c+| e\c||ou.
MANACMNI 0F MIAN0MA
IV. Supeificial spieading melanoma, nodulai
melanoma, and acial lentiginous melanoma
A. Thickness <1 mm
1. Excise with a 1-cm maigin fiom the le-
sion edge.
2. Excise down to the fascia oi to the un-
Diiect closuie without giaft is often
possible.
3. Node dissection is not iecommended
unless nodes aie clinically palpable and
suspicious foi tumoi.
B. Thickness 1-4 mm
1. Excise 2 cm fiom the edge of the lesion,
except on the face, wheie naiiowei mai-
gins may be necessaiy.
2. Excise down to the fascia oi to the un-
Giaft may be iequiied.
3. The sentinel node pioceduie foi tumois
with thickness >1 mm is iecommended.
4. Lymphadenectomy is selectively pei-
foimed and only foi those nodal basins
with occult tumoi cells (i.e., positive
sentinel lymph node). If the sentinel
node is negative, then the patient is
spaied a lymph node dissection.
5. Theiapeutic nodal dissection is iecom-
mended if nodes aie clinically palpable
and suspicious foi tumoi.
6. If iegional node is positive and com-
pletely iesected with no evidence of
distant disease, adjuvant theiapy with in-
teifeion- -2b (IFN- -2b) is consideied.
A0lvANI IhkAF
This is tieatment of a patient aftei iemoval
of all detectable tumoi but the patient is con-
sideied at high iisk foi iecuiience (i.e., stages
IIb and III). As mentioned above, IFN- -2b
(both high and low dose) is subject to intensive
investigation; howevei, despite eaily piomising
iesults to date no cleai benefit on oveiall sui-
vival has been convincingly demonstiated.
Maoaemeot oI 0staot Metastases (Stae Iv)
Cuiiently this can be consideied palliative at
best. Suigical iemoval of accessible metastases
can piovide excellent palliation. Chemotheiapy
encompasses a laige list of diugs (dacaibazine/
temozolomide, cisplatin, vindesine/vinblastine,
fotemustine, taxol/taxoteie) employed as single
agents oi in combination. Dacaibazine is still
the most effective monotheiapeutic agent, but
all in all chemotheiapeutic tieatment of stage
IV melanoma is disappointing, showing only
a 20% iesponse iate and no effect on oveiall
suivival. Theie aie a laige numbei of melanoma
vaccination tiials piesently being peifoimed,
and the field is iapidly expanding to include
gene-theiapeutic appioaches such as anti-Bcl-
2 oligonucleotide theiapy. Inciease of oveiall
suivival has, howevei, not been shown to date.
Radiotheiapy has only palliative effects also,
but steieotactive iadiosuigeiy with the gamma-
knife has shown consideiable palliation.
F0II0W-F F0k FkIMAk MIAN0MA
See Table 12-8.
IA8I 12-8 |o||owup for Primary Ne|anoma
Stages | (>1 mm) aod ||, Stage |||, Lymph
Stage | (<1 mm) Lymph hodes hegat|ve hodes Pos|t|ve
E.e|, !-o rou||
c
E.e|, !-o rou||
c
|o| E.e|, !-o rou|| |o| !
|o| ! ,e+| ! ,e+| ,e+|, ||eu !-2 rou||
|o| 2 ,e+|
ke.|eW o| ,|er ke.|eW o| ,|er ke.|eW o| ,|er
||,|c+| e\+r|u+||ou ||,|c+| e\+r|u+||ou ||,|c+| e\+r|u+||ou
||.e| |uuc||ou (||n) CBC, ||.e| |uuc||ou (||n)
C|e| \|+, +ud CI c+u C|e| \|+, +ud CI
e.e|, o rou|| c+u e.e|, o rou||
Auuu+| e\+r|u+||ou Auuu+| e\+r|u+||ou Auuu+| e\+r|u+||ou
|o| |||e |o| |||e |o| |||e
c
|+r|||+| re|+uor+ d,p|+||c ue.u ,ud|ore. e.e|, ! rou|| |o| ! ,e+|, +ud ||eu e.e|, o rou|| |o| 5 ,e+|, +ud ||eu +uuu+||, |o| |||e.
334
S E C I | 0 N 1 5
vIIIIIC0
FICMNIAk 0IS0k0kS
|o|r+| ||u co|o| | corpoed o| + r|\|u|e o|
|ou| ||oc||ore, u+re|,, () -!:-! |-
|| (||ue), (2) ,|-|| (|ed), (!)
:c|-! (,e||oW, e\oeuou ||or d|e|), +ud
(+) -|c (||oWu).
I|e p||uc|p+| de|e|r|u+u| o| ||e ||u co|o| |
re|+u|u p|reu|, +ud .+||+||ou |u ||e +rouu|
+ud d|||||u||ou o| re|+u|u |u ||e ||u +|e ||e
|+| o| ||e |||ee p||uc|p+| |ur+u ||u co|o|.
||+c|, ||oWu, +ud W|||e.
I|ee |||ee |+|c ||u co|o| +|e eue||c+||,
de|e|r|ued +ud +|e c+||ed :|||.- -|c
-|c| , ||e uo|r+| |+|c ||u co|o|
p|reu|+||ou c+u |e |uc|e+ed de|||e|+|e|, |,
e\pou|e |o u|||+.|o|e| |+d|+||ou (u\k) o| p||u||+|,
|o|roue, +ud ||| | c+||ed !:||- -|c
-|c| .
I|e cor||u+||ou o| ||e cou|||u||.e +ud |uduc|||e
re|+u|u p|reu|+||ou de|e|r|ue W|+| | c+||ed
||e | |||,- ('|I) (ee I+||e 0-2). E||u|c
||, | uo| uece+|||, + p+|| o| ||e de||u|||ou, e..,
A|||c+u '||+c| e||u|c pe|ou c+u |e '|I ||| +ud
+u E+| |ud|+u C+uc+|+u c+u |e '|I |\ o| e.eu
\. |- | |||,- c c|- | | :c:-
| c! ||! |- -:!-! c| ||- || c|-|
.| .
|uc|e+e o| re|+u|u |u ||e ep|de|r| |eu|| |u +
|+|e |uoWu + |,--|c. I|| |e||ec| oue
o| |Wo |,pe o| c|+ue.
Au |uc|e+e |u ||e uur|e| o| re|+uoc,|e |u ||e
ep|de|r| p|oduc|u |uc|e+ed |e.e| o| re|+u|u,
W||c| | c+||ed -|c:,||: |,--|c (+u
e\+rp|e | |-|).
| |uc|e+e o| re|+uoc,|e |u| +u |uc|e+e
|u ||e p|oduc||ou o| re|+u|u ou|,, W||c| |
c+||ed -|c|: |,--|c (+u e\+rp|e |
-|cc ).
n,pe|re|+uo| o| |o|| |,pe c+u |eu|| ||or
|||ee |+c|o|. eue||c, |o|rou+| (+ |u Add|ou
d|e+e), W|eu || | c+ued |, +u |uc|e+e |u
c||cu|+||u p||u||+|, re|+uo||op|c |o|roue, +ud
u\k (+ |u |+uu|u).
n,pore|+uo| | + dec|e+e o| re|+u|u |u ||e
ep|de|r|. I|| |e||ec| r+|u|, |Wo |,pe o|
c|+ue.
|o dec|e+e o| re|+uoc,|e |u| + dec|e+e o|
||e p|oduc||ou o| re|+u|u ou|, ||+| | c+||ed
-|c-: |,-|c (+u e\+rp|e |
+|||u|r).
A dec|e+e |u ||e uur|e| o| +|euce o|
re|+uoc,|e |u ||e ep|de|r| p|oduc|u uo
o| dec|e+ed |e.e| o| re|+u|u. I|| | c+||ed
-|c:,|-: |,-|c (+u e\+rp|e
| .|||||o).
n,pore|+uo| +|o |eu|| ||or eue||c (+ |u
+|||u|r), ||or +u|o|rruue (+ |u .|||||o), o|
o||e| |u||+rr+|o|, p|ocee (+ |u po||u||+r
r+|o|, |eu|ode|r+ |u po||+|).
SCII0N 13 ||C\E|IAk\ ||'0k|Ek' 335
FI0MI0I0C
Sex Equal in both sexes. The piedominance in
women suggested by the liteiatuie likely ieflects
the gieatei concein of women about cosmetic
appeaiance.
Ae oI 0oset May begin at any age, but in
50% of cases it begins between the ages of 10
and 30 yeais. A few cases have been iepoited to
be piesent at biith; onset in old age also occuis
but is unusual.
Iocdeoce Common, woildwide. Affects up to
1% of the population.
kace All iaces. The appaiently incieased piev-
alence iepoited in some countiies and among
daikei-skinned peisons iesults fiom a diamatic
contiast between white vitiligo macules and
daik skin and fiom maiked social stigma in
countiies such as India, wheie even today the
oppoitunities foi advancement oi maiiiage
among affected individuals aie limited.
Iohertaoce Vitiligo has a genetic backgiound;
>30% of affected individuals have iepoited
vitiligo in a paient, sibling, oi child. Vitiligo
in identical twins has been iepoited. Tians-
mission is most likely polygenic with vaiiable
expiession. The iisk of vitiligo foi childien of
affected individuals is unknown but may be
<10%. Individuals fiom families with an in-
cieased pievalence of thyioid disease, diabetes
mellitus, and vitiligo appeai to be at incieased
iisk foi development of vitiligo.
FAIh0CNSIS
Thiee piincipal theoiies have been piesented
about the mechanism of destiuction of melano-
cytes in vitiligo:
1. The auommune |eory holds that se-
lected melanocytes aie destioyed by ceitain
lymphocytes that have somehow been
activated.
wo||dW|de occu||euce, o| popu|+||ou
+||ec|ed.
A r+jo| p,c|o|o|c+| p|o||er |o| ||oWu o| ||+c|
pe|ou, |eu|||u |u e.e|e d||||cu|||e |u oc|+|
+dju|reu|.
A c||ou|c d|o|de| W||| ru||||+c||ou+| p|ed|po|
||ou +ud |||e||u |+c|o|.
C||u|c+||, c|+|+c|e||/ed |, |o|+||, W|||e r+cu|e,
W||c| eu|+|e +ud c+u +||ec| ||e eu|||e ||u.
\|c|ocop|c+||,. corp|e|e +|euce o| re|+uo
c,|e.
Aoc|+|ed W||| ,|er|c +u|o|rruue +ud/o|
eudoc||ue d|e+e.
vIIIIIC0 |C|9 . 109.0
|C|0 . |30
2. The neurogent |yo|ess is based on an
inteiaction of the melanocytes and neive
cells.
3. The se|[-Jesrut |yo|ess suggests that
melanocytes aie destioyed by toxic sub-
stances foimed as pait of noimal melanin
biosynthesis.
While the immediate mechanism foi the
evolving white macules involves piogiessive
destiuction of selected melanocytes by cytotoxic
T cells, othei genetically deteimined cytobio logic
changes and cytokines must be involved. Vitiligo
may follow cytokine deimatitis aftei imiquimod.
Because of diffeiences in the extent and couise
of segmental and geneialized vitiligo, the patho-
genesis of these two types is piobably diffeient.
Many patients attiibute the onset of theii
vitiligo to physical tiauma (wheie vitiligo
appeais at the site of tiauma-Koebnei phe-
nomenon), illness, oi emotional stiess. Onset
aftei the death of a ielative oi aftei seveie
physical injuiy is often mentioned. A sunbuin
ieaction may piecipitate vitiligo.
Sko Iesoos Macules, 5 mm to 5 cm or
more in diametei (Figs. 13-1 and 13-2).
Chalk" oi pale white, shaiply maiginated.
The disease piogiesses by giadual enlaigement
of the old macules oi by development of new
ones. Maigins aie tonex (as if the pathologic
piocess of de pigmentation weie flowing into
noimally pigmented skin).Tiichiome vitiligo
(thiee colois: white, light biown, daik biown)
iepiesents diffeient stages in the evolution of
vitiligo. Newly developed macules may be off-
white" in coloi; this also iepiesents a tiansitional
phase. Pigmentation aiound a haii follicle in a
white macule iepiesents iesidual pigmentation
oi ietuin of pigmentation (Fig. 13-3). Confetti-
sized hypomelanotic macules may also be
obseived. In[|ammaory |go has an elevated
eiythematous maigin and may be piuiitic.
DIstrIhutIvn Two geneial patteins. The [ota|
type is chaiacteiized by one oi seveial macules
in a single site; this may be an eaily evolution-
aiy stage of one of the othei types in some
cases. Cenera|:eJ vitiligo is moie common and
is chaiacteiized by widespiead distiibution of
depigmented macules, often in a iemaikable
symmetiy (Fig. 13-2). Typical macules occui
aiound the eyes (Fig. 13-1) and mouth and
on digits, elbows, and knees, as well as on the
low back and in genital aieas (Image 13-1).
The |- " aern involves the skin aiound
the mouth as well as on distal fingeis and
toes; lips, nipples, genitalia (Fig. 35-6, tip of
the penis), and anus may be involved. Conflu-
ence of vitiligo iesults in laige white aieas, and
extensive geneialized vitiligo may leave only a
few noimally pigmented aieas of skin- |go
unersa|s (Fig. 13-4).
Semeota| vt|o This is a special subset
that usually develops in one unilateial iegion;
usually does not extend beyond that initial
onesided iegion (though not always); and, once
piesent, is veiy stable. May be associated with
vitiligo elsewheie.
Assocated Cutaoeous Fodos White haii and
piematuiely giay haii. Ciicumsciibed aieas of
white haii, analogous to vitiligo macules, aie
called o|oss . Alopecia aieata and halo nevi.
In oldei patients, photoaging as well as solai
keiatoses may occui in vitiligo macules in those
with histoiy of long exposuies to sunlight.
Squamous cell caicinoma, limited to the white
macules, has iaiely been iepoited.
Ceoera| xamoatoo Not uncommonly as-
sociated with thyioid disease (up to 30% of all
vitiligo cases: Hashimoto thyioiditis, Giaves
disease); also diabetes mellitus-piobably <5%;
peinicious anemia (uncommon, but incieased
iisk); Addison disease (uncommon); and multi-
ple endociinopathy syndiome (iaie). Ophthal-
mologic examination may ieveal evidence of
healed choiioietinitis oi iiitis (piobably <10%
of all cases). Vision is unaffected. Heaiing is
noimal. The Vog-Koyanag-HaraJa synJrome
is vitiligo - poliosis - uveitis - dysacusis -
alopecia aieata.
Wood Iamp xamoatoo This examination
is iequiied to evaluate macules, paiticulaily in
lightei skin types, and to identify macules in
sun-piotected aieas in all but the daikest skin
types.
0ermatopatho|oy In ceitain difficult cases,
a skin biopsy may be iequiied. Established
vitiligo macules show noimal skin except foi
an absence of melanocytes. Use special stains
to identify melanocytes. Theie may be a mild
lymphocytic iesponse. These changes aie not
diagnostic foi vitiligo, howevei, only consistent
with it.
|ectroo Mcroscopy Absence of melanocytes
and of melanosomes in keiatinocytes; also
changes in keiatinocytes: spongiosis, exocytosis,
basilai vacuopathy, and apoptosis. Lymphocytes
have been seen in the epideimis.
Iaboratory Studes Thyioxine (T
4
), thyioid-
stimulating hoimone (iadioimmunoassay),
fasting blood glucose, complete blood count
with indices (peinicious anemia), ACTH stimu-
lation test foi Addison disease, if suspected.
0IACN0SIS
Noimally, diagnosis of vitiligo can be made
ieadily on clinical examination of a patient with
piogiessive, acquiied, chalk-white, bilateial
(usually symmetiic), shaiply defined macules
in typical sites.
0IFFkNIIAI 0IACN0SIS 0F vIIIIIC0
||,c c||c (|||| c+||u, |u//, r+||u, o||
W|||e co|o|).
||,c .-:| c||c (||ue c+|e W||| |eeu
||,e||oW ||uo|eceuce uude| wood |+rp, po|||.e
K0n.
C|-:c| |-|!-c (|||o|, o| e\pou|e |o ce|
|+|u p|euo||c e|r|c|de, cou|e||| r+cu|e). I|| |
+ d||||cu|| d|||e|eu||+| d|+uo|, + re|+uoc,|e +|e
+|eu| + |u .|||||o.
|-, (euder|c +|e+, o||W|||e co|o|, uu+||, |||
de||ued c-||-|: r+cu|e).
|-. !--| (|+||e, coueu||+|, o||W|||e
r+cu|e, uu||+|e|+|).
|,-|c | || |||+|e|+|, B|+c||o ||ue,
r+|||e c+|e p+||e|u, o0-15 |+.e ,|er|c |u
.o|.ereu|-ceu||+| ue|.ou ,|er (C|'), e,e,
rucu|o|e|e|+| ,|er).
|-. c-: (doe uo| eu|+uce W||| wood
|+rp, doe uo| |oW e|,||er+ +||e| |u|||u).
|- :|- (|+||e, coueu||+| o||W|||e r+c
u|e po|,ou+|, +||e+| |+pe, occ+|ou+| ereu
|+| r+cu|e, +ud cou|e||| r+cu|e).
SCII0N 13 ||C\E|IAk\ ||'0k|Ek' 33T
FICk 13-1 vt|o: Iace E\|eu|.e dep|reu|+||ou o| ||e ceu||+| |+ce. |u.o|.ed .||||||uou ||u |+ cou
.e\ |o|de|, e\|eud|u |u|o ||e uo|r+| p|reu|ed ||u. |o|e ||e c|+||W|||e co|o| +ud |+|p r+||u+||ou. ||-
c| ||c| ||- !-c| -.-|c:,|: -. ||- - | |c -|c-! | -|c|
FICk 13-2 vt|o: koees |ep|reu|ed, |+|p|, der+|c+|ed r+cu|e ou ||e |uee. Ap+|| ||or ||e |o
o| p|reu|, .||||||uou ||u +ppe+| uo|r+|. I|e|e | |||||u ,rre||,. |o|e ||u, |o|||cu|+| p|reu|ed po| W||||u
||e .|||||o +|e+ ||+| |ep|eeu| |ep|reu|+||ou.
|-|c|! (coueu||+|, W|||e |o|e|oc|, |+||e, do|+|
p|reu|ed |||pe ou |+c|, d|||uc||.e p+||e|u W|||
|+|e |,pe|p|reu|ed r+cu|e |u ||e ceu|e| o| ||e
|,pore|+uo||c +|e+).
|-|!-c c:c|-! +|| -|cc (r+, uo|
|e ||ue .|||||o |u+ruc| + re|+uoc,|e, +|||ou|
|educed, +|e uu+||, p|eeu|).
||||cc|, |-|!-c o||W|||e r+cu|e
(uu+||, + |||o|, o| po||+| o| ec/er+ |u ||e +re
r+cu|+| +|e+, ee ||. !+), |upu e|,||er+|ou
uo| o |+|p|,, de||ued|.
!,: |!- (r+, |e cou|u|u + ou|,
dep|reu|+||ou r+, |e p|eeu| +ud ||op, | uec
e+|,).
l||,cc|cc!c ,!- (.||ou p|o||er,
p|o|op|o||+, |||+|e|+| d,+cu|).
|cc!-| ,!- (corroue| c+ue o| cou
eu||+| de+|ue, W|||e r+cu|e +ud W|||e |o|e|oc|,
||| |e|e|oc||or|+).
C0kS AN0 Fk0CN0SIS
Vitiligo is a chionic disease. The couise is
highly vaiiable, but iapid onset followed by
a peiiod of stability oi slow piogiession is
most chaiacteiistic. Up to 30% of patients may
iepoit some spontaneous iepigmentation in a
few aieas-paiticulaily aieas that aie exposed
to the sun. Rapidly piogiessive, oi galloping,"
vitiligo may quickly lead to extensive depig-
mentation with a total loss of pigment in skin
and haii, but not eyes.
The tieatment of vitiligo-associated disease (i.e.,
thyioid disease) has no impact on the couise of
vitiligo. Suipiisingly, theie is less than expected
numbei of solai keiatoses, SCCIS, invasive SCC
oi BCE in vitiligo spots.
MANACMNI
The appioaches to the management of vitiligo
aie as follows:
Suoscreeos
The dual objectives of sunscieens aie piotection
of involved skin fiom acute sunbuin ieaction
and limitation of tanning of noimally pig-
mented skin. Sunscieens with a sun piotection
factoi >30 aie ieasonable choices to pievent
sunbuin foi most patients and to limit the
tanning ieaction in faiiei-skinned individuals.
While all SPTs have a need foi sun piotection,
sunscieens alone aie often peifectly adequate
management foi those vitiligo patients with
SPTs I, II, and sometimes III.
Cosmetc Coverup
The objective of coveiup with dyes oi makeup
is to hide the white macules so that the vitiligo
FICk 13-3 vt|o repmeotatoo A |o|||cu|+| p+||e|u o| |ep|reu|+||ou due |o |u\A ||e|+p, occu|||u
|u + |+|e .||||||uou r+cu|e ou ||e |oWe| +|doreu. \e|+uoc,|e r+, pe||| |u ||e |+|| |o|||c|e ep|||e||ur +ud
e|.e |o |epopu|+|e |u.o|.ed ||u, pou|+ueou|, o| W||| p|o|oc|ero||e|+p,.
SCII0N 13 ||C\E|IAk\ ||'0k|Ek' 339
FICk 13-4 oversa| vt|o \||||||uou r+cu|e |+.e co+|eced |o |u.o|.e +|| ||u ||e W||| corp|e|e
dep|reu|+||ou o| ||u +ud |+|| |u + |er+|e. I|e p+||eu| | We+||u + ||+c| W| +ud |+ d+||eued ||e ||oW W|||
e,e||oW peuc|| +ud e,e||d r+||u W||| e,e ||ue|.
IMAC 13-1 vt|o: p|ed||ec
||ou ||e.
Hypomelanosis
of hair
is not appaient. Ovei-the-countei piepaiations
come in many coloi shades, aie easy to apply,
and do not iub off but giadually wash oi weai
off. So-called self-tanning agents, which contain
dihydioxyacetone, aie available in a numbei of
foimulations.
kepmeotatoo
The objective of iepigmentation (Figs. 13-3
and 13-5) is the peimanent ietuin of noimal
melanin pigmentation. This may be achieved
foi local macules with topical glucocoiticoids oi
topical psoialens and UVA (long-wave ultiavio-
let light) and foi widespiead macules with oial
psoialens and UVA (PUVA).
Tota| g|utotortoJs Initial tieatment with
inteimittent (4 weeks on, 2 weeks off) topical
class I glucocoiticoid ointments is piactical,
simple, and safe foi single oi a few macules. If
theie is no iesponse in 2 months, it is unlikely
to be effective. Monitoi foi signs of eaily stei-
oid atiophy.
Tota| ta|tneurn n||ors Taciolimus and
pimeciolimus aie effective in iepigmenting
vitiligo but only in sun-exposed aieas. They
aie iepoited to be most effective when com-
bined with UVB oi excimei lasei theiapy (see
below).
Tota| |oot|emo|eray Employs topical
8-methoxypsoialen (8-MOP) and UVA. This
pioceduie should be undeitaken foi small
macules only by expeiienced physicians and
well-infoimed patients. As with oial psoi-
alens, it may iequiie 15 tieatments to initiate
iesponse and 100 to finish.
Sysemt |oot|emo|eray Foi moie wide-
spiead vitiligo, oial PUVA is moie piactical.
Oial PUVA may be done using sunlight
(in summei oi in aieas with yeai-iound
sunlight) and 5-methoxypsoialen (5-MOP)
(available in Euiope) oi with aitificial
UVA and eithei 5-MOP oi 8-MOP (Fig.
13-5). A iesponse to PUVA is signaled by
the appeaiance of tiny, usually folliculai
macules of pigmentation (Fig. 13-3). When
this occuis, it is a good piognostic sign
foi successful iepigmentation. Oial PUVA
photochemotheiapy with eithei 8-MOP oi
5-MOP is up to 85% effective in >70%
of patients with vitiligo of the head, neck,
uppei aims and legs, and tiunk. Howevei,
at least 1 yeai of tieatment is iequiied to
achieve this iesult. Distal hands and feet and
the lip-tip" vaiiant of vitiligo aie pooily
iesponsive and, when piesent alone, aie not
usually woith tieating. Genital aieas should
be shielded and not tieated. Foi iisks of
PUVA theiapy see Section 3, PUVA Theiapy
foi Psoiiasis."
Narrow-|anJ UVB, J11 nm This is just as
effective as PUVA and does not iequiie
psoialens. It is the tieatment of choice in
childien <6 yeais of age.
Excimei lasei (308 nm) This is effective but,
as foi PUVA, iepigmentation is also slow.
Pioduces best iesults in the face.
MoraIto
Minigiafting (autologous Thieisch giafts, suc-
tion blistei giafts, autologous minipunch giafts,
tiansplantation of cultuied autologous melano-
cytes) may be a useful technique foi iefiactoiy
and stable segmental vitiligo macules. PUVA may
be iequiied aftei the pioceduie to unify the coloi
between the giaft sites. The demonstiated occui-
ience of Koebneiization in donoi sites in geneial-
ized vitiligo iestiicts this pioceduie to those who
have limited cutaneous aieas at iisk foi vitiligo.
Pebbling" of the giafted site may occui.
0epmeotatoo
The objective of depigmentation is one" skin
coloi in patients with extensive vitiligo oi in
those who have failed PUVA, who cannot use
PUVA, oi who ieject the PUVA option.
B|euchIng Bleaching of norma||y gmeneJ
s|n with monobenzylethei of hydioquinone
20% (MEH) cieam is a peimanent, iiieveis-
ible piocess. Since application of MEH may be
associated with satellite depigmentation, this
tieatment cannot be used selectively to bleach
ceitain aieas of noimal pigmentation, since
theie is a ieal likelihood that new and distant
white macules will develop ovei the months of
use. The success iate is >90%. The end-stage
coloi of depigmentation with MEH is chalk-
white, as in vitiligo macules. The patient who
may want bleaching with MEH is typically a
skin phototype IV to VI with extensive iepig-
mentation theiapy-iesistant vitiligo of the face
and hands with iesidual aieas of noimal (daik)
skin coloi who aie happy with a unifoim, albeit
white skin coloi on the exposed iegions. An
occasional patient may wish to take 30-60 mg
-caiotene pei day to impait an off-white coloi
to the vitiliginous skin.
All those who have bleached aie at iisk foi
sunbuin fiom acute solai iiiadiation.
No long-teim untowaid effects have been
iepoited fiom the use of MEH 20% cieam,
but note that the Jegmenaon at|eeJ s
ermanen .
SCII0N 13 ||C\E|IAk\ ||'0k|Ek' 341
A|||u|r dec|||e + |oup o| eue||c +||e|+||ou
o| ||e re|+u|u p|reu| ,|er ||+| +||ec| ||u,
|+|| |o|||c|e, +ud e,e.
|| p||uc|p+||, |u.o|.e ||e ,u||e| o| re|+u|u |u
||ee ||e, |u| + uo|r+| uur|e| o| re|+uoc,|e
+|e p|eeu|.
A|||u|r c+u +||ec| ||e e,e. ocu|+| +|||u|r
(0A), o| ||e e,e +ud ||u. ocu|ocu|+ueou
+|||u|r (0CA), o| ||e ||u +ud o||e| o|+u
,|er, ||e C|' r+, +|o |e +||ec|ed |u ore
|o|r.
I|e c|+|||c+||ou o| +|||u|r | |oWu |u
I+||e !.
0CA | |, |+| ||e ro| corrou |o|r o| +|||u|r
+ud | ||e ou|, |o|r d|cued |e|e.
AI8INISM |C|9 . 210.2
|C|0 . E10.!
FI0MI0I0C
C|assIcatoo See Table 13-1.
Freva|eoce Estimated 1:20,000. OCA1 and
OCA2 account foi 40-50%.
Iohertaoce Most autosomal iecessive.
0CI0CIAN0S AI8INISM (0CA)
FICk 13-5 vt|o: therapy-oduced repmeotatoo I|| 20,e+|o|d |ud|+u |er+|e | |e|u ||e+|ed
W||| p|o|oc|ero||e|+p, (|u\A). I|e|e | |||| e|,||er+ |u ||e .||||||uou r+cu|e |u ||e e+||, p|+e (|e||) o|
||e|+p, ||+| W||| |e |o||oWed |, |o|||cu|+| p|reu|+||ou + |u ||. !!, +||e| ,e+| o| ||e+|reu|, .|||||o |+ cor
p|e|e|, |ep|reu|ed |u| ||e|e | uoW |,pe|p|reu|+||ou o| ||e |uee (||||). I||, |oWe.e|, W||| |+de W||| ||re
+ud ||e co|o| o| ||e |ep|reu|ed +|e+ W||| ||eud W||| ||+| o| ||e u||ouud|u ||u.
FAIh0CNSIS
The defect in melanin synthesis has been shown
to iesult fiom absence of the activity of the
enzyme tyiosinase. Tyiosinase is a coppei-
containing enzyme that catalyzes the oxidation
of tyiosine to dopa and the subsequent con-
veision of dopa to dopa-quinone. The muta-
tions in the tyiosinase gene iesponsible foi
deficient tyiosinase activity in seveial types of
albinism aie shown in Table 13-1.
0uratoo Piesent at biith. Patients with albi-
nism avoid the sun because of iepeated sun-
buins and biight light because of pioblems
with vision; otheiwise, they live an essentially
noimal life.
Ceoera| Appearaoce Poiing" (eyes half closed,
squinting) when in sunlight (Fig. 13-6).
Sko Vaiied, depending on the type: Snow"
white, cieamy white, light tan (Fig. 13-6 and
Table 13-1).
har White (tyiosinase-negative) (Fig. 13-6);
yellow, cieam, oi light biown (tyiosinase-
positive); ied; platinum.
yes The eye changes aie the essential physical
finding that define the syndiome of OCA.
Nystagmus, a featuie always piesent, iesults
fiom hypoplasia of the fovea with ieduction of
visual acuity and alteiation in the foimation
of the optic neives; this misiouting of the
optic paths is also associated with an alteinat-
ing stiabismus and diminished steieoacuity.
The diagnostic featuies in the eye that identify
albinism aie theiefoie nystagmus and iiis tians-
lucency (Fig. 13-7), ieduction of visual acuity,
decieased ietinal pigment, foveal hypoplasia,
and stiabismus.
IA8I 13-1 C|assification of A|binism
6eoe
Type S0btypes Loc0s |oc|0des 0||o|ca| F|od|ogs
0CA 0CAA | I,|o|u+eue+||.e 0CA w|||e |+|| +ud ||u, e,e (p|u| +| |||||
||ue)
0CAB | \|u|r+| p|reu| 0CA w|||e |o ue+|uo|r+| ||u +ud |+||
p|reu|+||ou
||+||uur 0CA
\e||oW 0CA \e||oW (p|eore|+u|u) |+||, |||| |ed o|
||oWu |+||
Ierpe|+|u|eeu|||.e 0CA \+, |+.e ue+|uo|r+| p|reu| |u| uo| |u
+\|||+
Au|oor+| |ece|.e 0CA (ore)
0CA2 | I,|o|u+epo|||.e 0CA \e||oW |+||, ||u 'c|e+r, W|||e (A|||c+)
B|oWu 0CA |||| ||oWu/|+u ||u (A|||c+)
0CA! ||! Au|oor+| |ece|.e 0CA (ore)
ku|ou 0CA ked +ud |ed||oWu ||u +ud ||oWu e,e
(A|||c+)
0CA+ !1|
n|' ||' ne|r+u|,|ud|+| ,ud|ore '||u/|+|| + |u 0CAA o| 0CAB o|
0CA2, ||eed|u d|+||e| (|ue||o k|co)
Cn' |' C|ed|+|n|+|| ,ud|ore '||.e| |+||/|,pop|reu|+||ou/e||ou
red|c+| p|o||er
0A 01! /||u|ed 0A |o|r+| p|reu|+||ou o| ||u +ud |+||
|0IE. 0CA, ocu|ocu|+ueou +|||u|r, I\k, |,|o|u+e, |, p|u| p|o|e|u, I\k|, |,|o|u+e|e|+|ed p|o|e|u , 0A, ocu|+| +|||u|r, \AI|, rer||+ue
+oc|+|ed ||+upo||e| p|o|e|u, |\'I, |,oore ||+|||c||u.
'0ukCE. \od|||ed ||or | B+|+do|+u e| +|, |u |\ ||eed|e| e| +|, (ed). ||cc|:| |-c||, C--c| !-!:-, o|| ed. |eW \o||, \cC|+W
n|||, 200!.
SCII0N 13 ||C\E|IAk\ ||'0k|Ek' 343
0ermatopatho|oy LIght MIcrvscvpy Mel-
anocytes aie piesent in the skin and haii bulb
in all types of albinism. The dopa ieaction is
maikedly ieduced oi absent in the melanocytes
of the skin and haii, depending on the type
of albinism (tyiosinase-negative oi tyiosinase-
positive).
E|ectrvn MIcrvscvpy Melanosomes aie pie-
sent in melanocytes in all types of albinism, but
depending on the type of albinism, theie is a
ieduction of the melanization of melanosomes,
with many melanosomes being completely un-
melanized in tyiosinase-negative albinism.
Mo|ecu|ar Iesto Now available, and this
makes it possible to classify the specific gene
alteiation in vaiious types of albinism. Howevei,
it is not necessaiy foi diagnosis oi management
of the pioblem.
0IACN0SIS
White peisons with veiy faii skin (SPT I), blond
haii, and blue eyes may mimic albinos, but they
do not have eye changes (iiis tianslucency, nys-
tagmus). Some peisons with albinism who have
constitutive black oi biown skin coloi may have
a dilution of theii skin coloi fiom black to a light
FICk 13-T Irs traos|uceocy wth
a|bosm ||| ||+u|uceuc, | + |ue qu+
uou |u +|| |,pe o| ocu|ocu|+ueou +|||
u|r, e.eu |u ||oe p+||eu| |u W|or ||e
||| | ||oWu. I|e ||| | |+|e|, p|u| e\cep|
|u |u|+u|, +ud ||e d|+uo| o| +|||u|r
depeud ou ||e de|ec||ou o| ||| ||+u|u
ceuc,. I|| | |e| doue |u + d+|| |oor
W||| + ||+||||| po|u|ed +| ||e c|e|+.
FICk 13-6 0cu|ocutaoeous
a|bosm w|||e ||u, W|||e e,e|+|e,
e,e||oW, +ud c+|p |+||. I|e |||e +ppe+|
||+u|uceu|. nere p|reu| |.e ||e |+ce +
p|u||| |ue.I|e|e | qu|u||u due |o p|o|o
p|o||+ +ud u,|+ru.
biown and have the capacity to tan; also, some
types may have biown iiides but still have iiis
tianslucency. Theiefoie, iiis tianslucency and
the piesence of othei eye findings in the fundus
aie the pathognomonic signs of albinism. The
haii and skin coloi may vaiy fiom noimal to
absent melanin, and the vaiious types aie listed
in Table 13-1. The special types of albinism aie
diagnosed on the basis of clinical piesentation
of the haii and skin pigmentation as well as
hematologic studies (Heimansky-Pudlak and
Chdiak-Higashi syndiome).
SICNIFICANC
Albinism is an impoitant disease to iecognize
eaily in life in oidei to begin piophylactic
measuies to pievent deimatoheliosis and skin
cancei, i.e., piotective clothing, sunblocks, and
sun avoidance in peak peiiods duiing the day.
C0kS AN0 Fk0CN0SIS
Albinos with tyiosinase-positive OCA foim
melanin pigment in the haii, skin, and eyes dui-
ing eaily life, the haii becoming cieam, yellow,
oi light biown, and the eye coloi changing fiom
light giay to blue, hazel, oi even biown.
Albinos living in cential Afiica who aie un-
piotected fiom the sun develop squamous cell
caicinomas eaily in life, and this significantly
shoitens theii life span; few suivive to the age
of 40 yeais because of metastasizing squamous
cell caicinoma. Deimatoheliosis and basal cell
caicinomas aie fiequent in albinos living in
tempeiate climates. Melanomas aie, cuiiously,
veiy iaie in albinos living in Afiica; when they
occui, they aie usually amelanotic. Melanocytic
nevi occui in albinism and may also be amelan-
otic but may be pigmented, depending on the
type of albinism.
MANACMNI
Eveiy albino should be undei the caie of an
ophthalmologist foi vision pioblems and a dei-
matologist to detect solai keiatoses, skin canceis,
and deimatoheliosis. Daily application of topi-
cal, potent, bioad-spectium SPF >30 sunblocks,
including lip sunblocks. Avoidance of sun expo-
suie in the high solai intensity season. Use of
topical tietinoin foi deimatoheliosis and foi its
possible piophylactic effect against sun-induced
epithelial skin canceis. Tieatment of solai keia-
toses to pievent the development of squamous
cell caicinomas. Systemic -caiotene (30-60
mg thiice daily) impaits a moie noimal coloi
to the skin and may have some piotective effect
on the development of skin canceis, although
this has been pioved only in mice. It is helpful
foi albinos to belong to a national volunteei
gioup of albinos; in the United States it is called
the N ational O iganization foi lbinism and
H ypomelanosis (NOAH). (Noah, the buildei of
the aik in the Old Testament, was alleged to be
an albino.) This gioup assists albinos in vaiious
ways, especially in dealing with vision piob-
lems: obtaining diivei`s license, etc.
FI0MI0I0C
Ae oI 0oset Young adults.
Sex Females > males; about 10% of patients
with melasma aie men.
\e|+r+ (C|ee|. '+ ||+c| po|) | +u +cqu||ed
|||| o| d+||||oWu |,pe|p|reu|+||ou ||+|
occu| |u ||e e\poed +|e+, ro| o||eu ou ||e
|+ce, +ud |eu|| ||or e\pou|e |o uu||||.
|| r+, |e +oc|+|ed W||| p|eu+uc,, W||| |ue
||ou o| cou||+cep||.e |o|roue, o| po|||, W|||
ce||+|u red|c+||ou uc| + d|p|eu,||,d+u|o|u, o|
|| r+, |e |d|op+|||c.
',, . C||o+r+ (C|ee|. '+ |eeu po| ),
r+| o| p|eu+uc,.
MIASMA |C|9 . 109.o9
|C|0 . |3.
kace Melasma is moie appaient oi moie fie-
quent in peisons with biown oi black constitu-
tive skin coloi (peisons fiom Asia, the Middle
East, India, South Ameiica).
Iocdeoce aod Frecptato Factors Veiy com-
mon, especially among peisons with constitutive
SCII0N 13 ||C\E|IAk\ ||'0k|Ek' 345
biown skin coloi who aie taking contiaceptive
iegimens and who live in sunny aieas. Pieg-
nancy causes melasma. Melasma has iecently
been appeaiing in menopausal women as a
iesult of iegimens foi pievention of osteopoio-
sis using a combination of estiogens anJ pio-
gesteione; melasma does not appeai in those
women who aie given estiogen ieplacement
tieatment but without piogesteione. Also in
patients on diphenylhydantoin. Sun exosure
requreJ.
FAIh0CNSIS
Unknown.
0uratoo oI Iesoos The pigmentation usually
evolves quite iapidly ovei weeks, paiticulaily
aftei exposuie to sunlight.
Sko Iesoos Maculai hypeipigmentation
of the face, the hue and intensity depending
laigely on the skin phototype of the patient
(Fig. 13-8). Light oi daik biown oi even black.
Coloi is usually unifoim but may be splotchy.
Most often symmetiic. Lesions have seiiated,
iiiegulai, and geogiaphic boideis. Two-thiids
on cential pait of the face: cheeks (Fig. 13-8),
foiehead, nose, uppei lip, and chin; a smallei
peicentage on the malai oi mandibulai aieas
of the face and occasionally the doisa of the
foieaims.
Wvvd Lump ErumInutIvn A maiked accen-
tuation of the hypeipigmented macules. T|s
tonras s no attenuaeJ n aens w| a
norma| |rown or ||at| s|n .
Postinflammatoiy hypeimelanotic macules.
SICNIFICANC
While this is a stiictly cosmetic pioblem, it
is veiy distuibing to both males and females,
especially peisons with biown skin coloi and
good tanning capacity.
C0kS AN0 Fk0CN0SIS
Melasma may disappeai spontaneously ovei
a peiiod of months aftei deliveiy oi aftei
cessation of contiaceptive hoimones. Melasma
may oi may not ietuin with each subsequent
piegnancy.
FICk 13-8 Me|asma we||der+|c+|ed, |,pe|p|reu|ed r+cu|e +|e eeu ou ||e c|ee|, uoe, +ud uppe| ||p.
FICMNIAk ChANCS F0II0WINC INFIAMMAII0N 0F Ih SkIN
MANACMNI
Iopca| Commeicially available piepaiations
in the United States include: hydioquinone
3% solution and 4% cieam; azelaic acid 20%
cieam; and a combination of flucinolone
0.01%, hydioquinone 4%, and tietinoin 0.05%.
Hydioquinone 4% cieam can be compounded
with 0.05% tietinoin cieam oi glycolic acid by
the phaimacist.
UnJer no trtumsantes s|ou|J mono|en-
:y|e|er o[ |yJroqunone or |e o|er e|ers o[
||||cc|, -!-c| -|c |,-
-|c| | + r+jo| p|o||er |o| p+||eu| W|||
||u p|o|o|,pe |\, \, +ud \| (||. !9 +ud
!0). I|| d|||u||u p|reu|+||ou c+u de.e|op
W||| +cue (||. !9), po||+|, ||c|eu p|+uu (||.
!0), +|op|c de|r+||||, o| cou|+c| de|r+||||
o| +||e| +u, |,pe o| ||+ur+ |o ||e ||u. || r+,
pe||| |o| Wee| |o rou|| |u| doe |epoud |o
|op|c+| |,d|oqu|uoue, W||c| +cce|e|+|e || d|
+ppe+|+uce. |e|ou +|e c|+|+c|e||||c+||, ||r||ed
|o ||e ||e o| ||e p|eced|u |u||+rr+||ou +ud
|+.e |ud|||uc|, |e+||e|ed |o|de|.
'ore d|u e|up||ou r+, |e +oc|+|ed W|||
de|r+| re|+u|u |,pe|p|reu|+||ou (||. !),
W||c| r+, +|o |e +oc|+|ed W||| ||c|eu p|+uu
+ud cu|+ueou |upu e|,||er+|ou. I|| de|r+|
|,pe|p|reu|+||ou r+, |e pe|||eu|, +ud ||e|e
| uo ||e+|reu|.
|-|| -|c (re|+uode|r+|||| |o\|c+) | +
|e||cu|+|, cou||ueu| ||+c| |o ||oWu.|o|e| p|reu
|+||ou o| ||e |+ce +ud uec| (||. !2). || r+,
|e + |eu|| o| cou|+c| eu|||.||, o| p|o|ocou|+c|
eu|||.||, |e|+|ed |o c|er|c+|, p+|||cu|+||, ||+
|+uce |u core||c.
| |,--|c !- | |||: -c:|
|uduced |, po|+|eu (Be||oque de|r+||||), ee
'ec||ou 0, +ud |o| -|c|c-! |,-
-|c| due |o d|u, ee 'ec||ou 22.
|yJroqunone (monome|y|- or monoe|y|-) |e
useJ n |e reamen o[ me|asma |etause |ese
Jrugs tan |eaJ o a ermanen |oss o[ me|anotyes
w| |e Jee|omen o[ a Js[gurng soy |eu-
|oJerma .
Freveotoo It is essential that the patient use,
eveiy moining, an oaque sunblock containing
titanium dioxide and/oi zinc oxide; the action
spectium of pigment daikening extends into
the visible iange, and even the potent tians-
paient sunscieens (with high SPF) aie com-
pletely ineffective in blocking visible iadiation.
hFkFICMNIAII0N |C|9 . 109.0
|C|0 . |3.9
FICk 13-10 FostoI|ammatory hyperpmeotatoo (Ac|o |+c|u p+e) I|| r+, |o||oW + d|u
e|up||ou, po||+|, o| ||c|eu p|+uu, epec|+||, |u ||u p|o|o|,pe \ +ud \|, + W+ ||e c+e |u ||| r|dd|e+e
E+| |ud|+u r+|e. |o||u||+rr+|o|, |,pe|p|reu|+||ou | + r+jo| p|o||er |u ,ouu |er+|e W||| ||u p|o|o|,pe
\ +ud \|.
SCII0N 13 ||C\E|IAk\ ||'0k|Ek' 34T
FICk 13-9 hyperme|aooss wth acoe . I|| coud|||ou | + r+jo| corp|+|u| o| ||| 3,e+|o|d ||u
p|o|o|,pe ||| |||. I|e +cue | uo| ||e p|o||er uoW, || | ||e d|||u||u |,pe|re|+uo|. I|| |,pe|p|reu|+||ou
c+u |e r+||ed|, |educed W||| |op|c+| |,d|oqu|uoue (!) c|e+r o| o|u||ou, |e| cor||ued W||| ||e||uo|u, +pp||ed
d+||,. |u||u ||e dep|reu|+||ou, ||e p+||eu| ru| ue +u op+que uu||oc| d+||, |o p|e.eu| ||e p|reu| d+||eu|u
||+| occu| W||| d+||, uu e\pou|e. 8. |u ||| !0,e+|o|d |+|||+u| Wor+u |,pe|re|+uo| due |o +cue, cor||ued
W||| re|+r+ +ud |,pop|reu|ed +cue c+|, W+ cou|de|ed + core||c d|+|e|, uo| ou|, |, ||e p+||eu| |u| +|o
|e| |u|+ud. '|e W+ ucce|u||, ||e+|ed W||| ! |,d|oqu|uoue |uco|po|+|ed |u|o + 0.05 ||e||uo|u c|e+r.
8
FICk 13-10
FICk 13-11 FostoI|ammatory derma| hyperpmeotatoo I|| | |oWu ou ||e |+ud o| + ||u p|o
|o|,pe |\ A|||c+u Wor+u |o||oW|u + ||\ed d|u e|up||ou.
FICk 13-12 Me|aoodermatts toxca . A |e||cu|+| cou||ueu| p|reu|+||ou ou ||e |+ce +ud uec| o| + +2
,e+|o|d |er+|e c|er|| W|o Wo||ed |o| + core||c |udu||, +ud |+d +pp||ed, o.e| ,e+|, ro| o| ||e ceu|ed p|od
uc| |e W+ |u.o|.ed |u p|oduc|u |o |e| oWu ||u. '|uce |e ||.ed |u + uuu, c||r+|e ||| |uc|e+e ||e up|c|ou o| +
c||ou|c p|o|ocou|+c| eu|||.||,. 8. |u ||| |ud|+u Wor+u ||e ro|||ed |,pe|p|reu|+||ou |+ co+|eced |o d+|| ||oWu
ro|||ed |,pe|p|reu|+||ou o| ||e c|ee|. |o| p|o|e|ou+| |e+ou ||| p+||eu| |+d +|o e\ce|.e|, ued core||c.
8
SCII0N 13 ||C\E|IAk\ ||'0k|Ek' 349
|o||u||+rr+|o|, |,pore|+uo| | +|W+, |e|+|ed
|o |o o| re|+u|u. || | + pec|+| |e+|u|e o| p||,||+
| .e||co|o| (||. !!, ee +|o 'ec||ou 25), |u
W||c| ||e |,pop|reu|+||ou r+, +|o |er+|u |o|
Wee| +||e| ||e +c||.e |u|ec||ou |+ d|+ppe+|ed.
n,pore|+uo| | uo| uucorrou|, eeu |u +|op|c
de|r+||||, po||+| (||. !+), u||+|e p+|+po
||+|, +ud p||,||+| ||c|euo|de c||ou|c+.
|| r+, +|o |e p|eeu| |u cu|+ueou |upu
e|,||er+|ou (||. !5), +|opec|+ ruc|uo+,
r,co| |uuo|de, ||c|eu |||+|u, e|o|||e|c
de|r+||||, +ud |ep|o,.
n,pore|+uo| r+, |o||oW de|r+||+|ou +ud
c|er|c+| pee|, |u ||ee coud|||ou ||e|e |
+ '||+u|e| ||oc|, |u W||c| re|+uoore +|e
p|eeu| |u re|+uoc,|e |u| +|e uo| ||+u|e||ed
|o |e|+||uoc,|e, |eu|||u |u |,pore|+uo|. I|e
|e|ou +|e uu+||, uo| c|+|| W|||e, + |u .|||||o,
|u| 'o|| W|||e +ud |+.e |ud|c|e|e r+||u.
A corrou |,pe o| |,pop|reu|+||ou | +oc|
+|ed W||| |,c c||c (||. !o). I|| | +
r+cu|+| |,pop|reu|+||ou ro||, ou ||e |+ce o|
c|||d|eu, o||W|||e W||| + poWde|, c+|e. ke|+||.e|,
|ud|||uc| r+||u uude| wood |||| +ud c+|
|u d|||uu|| ||| ec/er+|ou de|r+|||| ||or
.|||||o. || | e||||r||ed.
n,pore|+uo| uo| uucorrou|, |o||oW |u||+
|e|ou+| |ucoco|||co|d |ujec||ou, |u| W|eu ||e
|ujec||ou +|e |opped, + uo|r+| p|reu|+||ou
de.e|op |u ||e +|e+.
|epeud|u ou ||e +oc|+|ed d|o|de|, po||u
||+rr+|o|, |,pore|+uo| r+, |epoud |o o|+|
|u\A p|o|oc|ero||e|+p,.
FICk 13-13 Ftyrass versco|or . n,pop|reu|ed, |+|p|, r+||u+|ed, c+||u r+cu|e ou ||e |+c|
o| +u |ud|.|du+| W||| ||u p|o|o|,pe |||. Ceu||e +||+|ou o| ||e u||+ce W||| +cceu|u+|e ||e c+||u. I|| |,pe o|
|,pore|+uo| c+u |er+|u |ou +||e| ||e e|up||ou |+ |eeu ||e+|ed +ud ||e p||r+|, p|oce | |eo|.ed. 8.
|||,||+| .e||co|o| |u A|||c+u ||u. |e|ou +|e pe|||o|||cu|+| ou ||e c|e| +ud co+|ece |o |+|e cou||ueu| p+|c|e
ou ||e uec| W|e|e ||e ||ue c+||u c+u |e| |e eeu.
8
hF0FICMNIAII0N |C|9 . 109.0
|C|0 . |3.9
FICk 13-14 FostoI|ammatory hypome|aooss (psorass) I|e |,pore|+uo||c |e|ou co||epoud
e\+c||, |o ||e +u|ecedeu| e|up||ou. I|e|e | ore |e|du+| po||+| W||||u ||e |e|ou.
SCII0N 13 ||C\E|IAk\ ||'0k|Ek' 351
FICk 13-15 FostoI|ammatory hypopmeotatoo |,-|c| |u + !!,e+|o|d \|e|u+ree
|er+|e. I|e p+||eu| |+d |+d c||ou|c cu|+ueou |upu e|,||er+|ou. ke|du+| |u||+rr+||ou o| |upu | |||| eeu
ou ||e uppe| ||p.
FICk 13-16 Ftyrass a|ba A corrou d|||u||u |,pore|+uo|, W||c|, + ||e u+re |ud|c+|e, | +
W|||e +|e+ (+||+) W||| .e|, r||d c+||u (p||,||+|). || | o|e|.ed |u + |+|e uur|e| o| c|||d|eu |u ||e urre| |u
|erpe|+|e c||r+|e. || | ro||, + core||c p|o||er |u pe|ou W||| ||oWu o| ||+c| ||u +ud corrou|, occu| ou
||e |+ce, + |u ||| c|||d. Arou 200 p+||eu| W||| p||,||+| +||+, 90 |+ued ||or o-2 ,e+| o| +e. |u ,ouu
+du||, |A qu||e o||eu occu| ou ||e +|r +ud ||uu|.
| A k I | |
DEkMAIOLOCY AND
INIEkNAL MEDICINE
354
S E C I | 0 N 1 4
Ih SkIN IN IMMN,
AI0IMMN, AN0
khMAIIC 0IS0k0kS
Ar,|o|do| | +u e\||+ce||u|+| depo|||ou |u .+||
ou ||ue o| +r,|o|d |||||| p|o|e|u +ud o| +
p|o|e|u c+||ed c,|! | :-| (A|), ||e
|deu||c+| corpoueu| o| A| | p|eeu| |u ||e e|ur
+ud | c+||ed '1| . I|ee +r,|o|d depo|| c+u
+||ec| uo|r+| |od, |uuc||ou.
',|-: 1| c,|! , +|o |uoWu + c,
c,|! , occu| |u p+||eu| W||| B ce|| o|
p|+r+ ce|| d,c|+|+ +ud ru|||p|e r,e|or+ |u
W|or ||+reu| o| rouoc|ou+| |rruuo|o|u||u
|||| c|+|u |o|r +r,|o|d ||||||.
C||u|c+| |e+|u|e o| A| |uc|ude + cor||u+||ou o|
r+c|o|o|+ +ud c+|d|+c, |eu+|, |ep+||c, +ud
+||o|u|e||u+| (C|) |u.o|.ereu|, + We|| + c+|p+|
|uuue| ,ud|ore +ud | |- . I|ee occu|
|u !0 o| p+||eu|, +ud |uce ||e, occu| e+||,
|u ||e d|e+e, ||e, +|e +u |rpo||+u| c|ue |o ||e
d|+uo|.
',|-: 11 c,|! (|e+c||.e) occu| |u
p+||eu| +||e| c||ou|c |u||+rr+|o|, d|e+e, |u
W|or ||e |||||| p|o|e|u | de||.ed ||or ||e c||
cu|+||u +cu|ep|+e ||pop|o|e|u |uoWu + -
c,|! 1 .
I|e|e +|e |eW o| uo c|+|+c|e||||c ||u |e|ou |u
AA +r,|o|do|, W||c| uu+||, +||ec| ||e ||.e|,
p|eeu, ||due,, +ud +d|eu+|.
|u +dd|||ou, ||u r+u||e|+||ou r+, +|o |e
+oc|+|ed W||| + uur|e| o| (|+|e) |e|edo|+r|||+|
,ud|ore.
|:c|c-! :|c- c,|! | uo| uucor
rou, p|eeu| W||| |,p|c+| cu|+ueou r+u||e|+
||ou, +ud |+ uo ,|er|c |u.o|.ereu|.
SSIMIC AMI0I00SIS |C|9 . 211.!
|C|0 . E35.!
Sko Iesoos Smooth, waxy papules (Fig. 14-1),
but also nodules on the face, especially aiound
the eyes (Fig. 14-2) and elsewheie. Puipuia
following tiauma, pinch" puipuia in waxy pa-
pules (Fig. 14-2) sometimes also involving laige
suiface aieas without nodulai involvement.
Piedilection sites aie aiound the eyes, cential
face, extiemities, body folds, axillae, umbilicus,
anogenital aiea. Na| t|anges : Similai to lichen
k+|e
0ccu| |u r+u, |u| uo| +|| p+||eu| W||| ru|
||p|e r,e|or+ +ud B ce|| +ud p|+r+ ce||
d,c|+|+.
ke|||c||.e c+|d|or,op+||,, |eu+| |uuc||ou |r
p+||reu|, C| |u.o|.ereu| W||| r+|+|o|p||ou,
ueu|op+||,.
||ouo| poo|
SSIMIC AI AMI0I00SIS |C|9. 211.!

|C|0 . E35
planus (see Section 33). Matrog|ossa : diffusely
enlaiged and fiim, woody" (Fig. 14-3).
Systemc MaoIestatoos Include fatigue, weak-
ness, anoiexia, weight loss, malaise, dyspnea;
symptoms ielated to hepatic, ienal, and GI
involvement; paiesthesia ielated to caipal tunnel
syndiome, neuiopathy.
Ceoera| xamoatoo Kidney-nephiosis; neiv-
ous system-peiipheial neuiopathy, caipal tunnel
syndiome; caidiovasculai-paitial heait block,
congestive heait failuie; hepatic-hepatomegaly;
SCII0N 14 InE 'K|| || |\\u|E, AuI0|\\u|E, A|| knEu\AI|C ||'0k|Ek' 355
GI-diaiihea, sometimes hemoiihagic, malab-
soiption; lymphadenopathy.
May ieveal thiombocytosis >500,000/L.
Pioteinuiia and incieased seium cieatinine;
hypeicalcemia. Incieased IgG. Monoclonal pio-
tein in two-thiids of patients with piimaiy oi
myeloma-associated amyloidosis. Bone mai-
iow: myeloma.
0ermatopatho|oy Shows accumulation of
faintly eosinophilic masses of amyloid in the
papillaiy body neai the epideimis, in the papil-
laiy and ieticulai deimis, in sweat glands, aiound
and within blood vessel walls. Use thioflavin oi
congo ied and examine the sections foi an ap-
ple-gieen biiefiingence with a polaiization mi-
cioscope. Immunohistochemistiy to assess the
piopoition of kappa and lambda light chains.
0IACN0SIS
Made by the combination of puipuiic skin
lesions (Fig. 14-2), waxy papules (Fig. 14-1),
macioglossia (Fig. 14-3), caipal tunnel syn-
diome, and caidiac symptoms. A tissue di-
agnosis can be made fiom the skin biopsy.
Scintigiaphy aftei injection of
123
I-labeled SAP
will ieveal the extent of the involvement and
can seive as a guide foi tieatment, which is that
of the undeilying disease.
FICk 14-1 Systemc AI
amy|odoss w+\, p+pu|e ou ||e
||uu| o| + 53,e+|o|d r+|e p+||eu|
W||| r,e|or+.
FICk 14-2 Systemc AI amy-
|odoss: "poch purpura" I|e
|opro| p+pu|e | ,e||oW|| +ud
uou|ero|||+|c, ||e |oWe| po|||ou |
|ero|||+|c. 'oc+||ed p|uc| pu|pu|+
o| ||e uppe| e,e||d c+u +ppe+| |u
+r,|o|d uodu|e +||e| p|uc||u o| |u|
||u ||e e,e||d.
FAkI II |Ek\AI0|0C\ A|| ||IEk|A| \E||C||E 356
A |e+c||.e |,pe o| +r,|o|do|.
0ccu| |u +u, d|o|de| +oc|+|ed W||| + u|+|ued
+cu|ep|+e |epoue.
o0 |+.e |u||+rr+|o|, +|||||||. I|e |e| o||e|
c||ou|c |u||+rr+|o|, |u|ec||.e o| ueop|+||c d|
o|de|.
Ar,|o|d |||||| +|e de||.ed ||or c|e+.+e
||+reu| o| ||e c||cu|+||u +cu|ep|+e |e+c|+u|
e|ur +r,|o|d A p|o|e|u.
||eeu| W||| p|o|e|uu||+ |o||oWed |, p|o|e|.e
|eu+| d,|uuc||ou, uep||o||c ,ud|ore.
I|e|e +|e uo c|+|+c|e||||c ||u |e|ou |u AA
+r,|o|do|.
SSIMIC AA AMI0I00SIS |C|9. 211.!

|C|0 . E35
I||ee .+||e||e o| |oc+||/ed +r,|o|do| ||+| +|e
uu|e|+|ed |o ||e ,|er|c +r,|o|doe.
|!|c c,|! . |u|e o| ru|||p|e, roo||,
uodu|+| |e|ou W||| o| W|||ou| pu|pu|+ ou ||r|,
|+ce, o| ||uu| (||. ++1).
|:|-! c,|!. d|c|e|e, .e|, p|u||||c,
||oWu|||ed p+pu|e ou ||e |e (||. ++3).
!c:|c c,|! . p|u||||c, |+,||oWu, |e||cu
|+|ed r+cu|+| |e|ou occu|||u p||uc|p+||, ou ||e
uppe| |+c| (||. +5), ||e |e|ou o||eu |+.e +
d|||uc||.e '||pp|e p+||e|u.
|u ||c|euo|d +ud r+cu|+| +r,|o|do| ||e +r,|o|d
|||||| |u ||u +|e |e|+||ude||.ed. A|||ou| ||ee
|||ee |oc+||/ed |o|r o| +r,|o|do| +|e cou||ued
|o ||e ||u +ud uu|e|+|ed |o ,|er|c d|e+e, ||e
||u |e|ou o| uodu|+| +r,|o|do| +|e |deu||c+|
|o ||oe ||+| occu| |u A|, |u W||c| +r,|o|d ||||||
de||.e ||or |rruuo|o|u||u |||| c|+|u ||+
reu|.
I0CAIII0 CIAN0S AMI0I00SIS
FICk 14-3 Systemc AA amy|odoss: macro|ossa \+|.e |u|||||+||ou o| ||e |ouue W||| +r,|o|d
|+ c+ued |rreue eu|+|ereu|, ||e |ouue c+uuo| |e |e||+c|ed corp|e|e|, |u|o ||e rou|| |ec+ue o| || |/e.
(Cou||e, o| E.+u C+|||u, \|.)
SCII0N 14 InE 'K|| || |\\u|E, AuI0|\\u|E, A|| knEu\AI|C ||'0k|Ek' 35T
FICk 14-4 Ioca|ted cutaoeous amy|odoss . |odu|+|. IWo p|+que|||e uodu|e, W+\,, ,e||oW||
o|+ue W||| |ero|||+e. 8. ||c|euo|d +r,|o|do|. C|ouped cou||ueu| c+|, p+pu|e o| ||.|d, .|o|+ceou co|o|.
I|| | + pu|e|, cu|+ueou d|e+e.
FICk 14-5 Macu|ar amy|odoss C|+,||oWu, |e||cu|+|ed p|reu|+||ou ou ||e |+c| o| + 52,e+|o|d
A|+||+u r+|e.
8
Iocdeoce 15-23% of the population may
have had this condition duiing theii lifetime.
Chionic uiticaiia is likely to be piesent at some
time in about 25% of patients with uiticaiia.
to|oy Uiticaiia/angioedema is not a disease
but a cutaneous ieaction pattein. Foi classifica-
tion and etiology, see Table 14-1.
CIINICAI IFS
Acute rtcara Acute onset and iecuiiing
ovei <30 days. Usually laige wheals often as-
sociated with angioedema (Figs. 14-7 and 14-
8); often IgE-dependent with atopic diathesis;
ielated to foods, paiasites, and penicillin. Also,
complement-mediated in seium sickness-like
ieactions (whole blood, immunoglobulins,
penicillin). Often accompanied by angioedema.
Common. (See also Diug-Induced Acute Uiti-
caiia," Section 22.)
Chrooc rtcara Recuiiing ovei <30 days.
Small and laige wheals (Fig. 14-9). Raiely IgE-
dependent but often due to anti-Fc R autoanti-
bodies; etiology unknown in 80% and theiefoie
consideied idiopathic. Intoleiance to salicylates,
benzoates. Common. Chionic uiticaiia affects
adults piedominantly and is appioximately twice
as common in women as in men. Up to 40% of
patients with chionic uiticaiia of >6 months`
duiation still have uiticaiia 10 yeais latei.
Symptoms Prurtus In angioedema of palms
and soles pain. Angioedema of tongue, phai-
ynx inteifeies with speech, food intake, and
bieathing. Angioedema of laiynx may lead to
asphyxia.
u|||c+||+ | corpoed o| W|e+| (||+u|eu| eder+
|ou p+pu|e +ud p|+que, uu+||, p|u||||c +ud due
|o eder+ o| ||e p+p|||+|, |od,) (||. +o +ud
+1). I|e W|e+| +|e upe|||c|+|, We|| de||ued.
Au|oeder+ | + |+|e| eder+|ou +|e+ ||+|
|u.o|.e ||e de|r| c! u|cu|+ueou ||ue
(||. +3) +ud | deep +ud ||| de||ued. u|||c+||+
+ud +u|oeder+ +|e ||u ||e +re eder+|ou
p|oce |u| |u.o|.|u d|||e|eu| |e.e| o| ||e cu|+
ueou .+cu|+| p|e\u. p+p|||+|, +ud deep.
|C|9. 211.o

|C|0 . |3+.
u|||c+||+ +ud/o| +u|oeder+ r+, |e +cu|e |ecu|
|eu| o| c||ou|c |ecu||eu|.
0||e| |o|r o| u|||c+||+/+u|oeder+ +|e |eco
u|/ed. |
E +ud |
E |ecep|o|-depeudeu|, p|,|c+|,
cou|+c|, r+| ce|| de|+uu|+||ou-|e|+|ed, +ud
|d|op+|||c.
|u +dd|||ou, +u|oeder+/u|||c+||+ c+u |e red|
+|ed |, ||+d,||u|u, ||e corp|ereu| ,|er, +ud
o||e| e||ec|o| rec|+u|r.
u|||c+||+| .+cu|||| | + pec|+| |o|r o| cu|+ueou
uec|o||/|u .euu|||| (ee p+e +01).
I|e|e +|e ore ,ud|ore W||| +u|oeder+ |u
W||c| u|||c+||+| W|e+| +|e |+|e|, p|eeu| (e..,
|e|ed||+|, +u|oeder+).
kIICAkIA AN0 ANCI00MA |C|9 . 103.0
|C|0 . |50
Sko Iesoos Shaiply defined w|ea|s (Fig. 14-6),
small (<1 cm) to laige (>8 cm), eiythematous
oi white with an eiythematous iim, iound,
oval, aciifoim, annulai, seipiginous (Figs. 14-7
and 14-9), due to confluence and iesolution in
one aiea and piogiession in anothei (Fig. 14-7).
Lesions aie piuiitic and tiansient.
ngoeJema - skin-coloied, tiansient en-
laigement of poition of face (eyelids, lips,
tongue) (Figs. 14-8 and 22-7, B), extiemity, oi
othei sites due to subcutaneous edema.
DIstrIhutIvn Usually iegional oi geneialized.
Localized in solai, piessuie, vibiation, cold
uiticaiia/angioedema and confined to the site
of the tiiggei mechanism (see below).
SFCIAI FAIkS[AS kIAI0
I0 FAIh0CNSIS
Immuoo|oc rtcara 1gE MedIuted
Lesions in acute IgE-mediated uiticaiia
iesult fiom antigen-induced ielease of bio-
logically active molecules fiom mast cells oi
basophilic leukocytes sensitized with specific
IgE antibodies (type I anaphylactic hypeisen-
sitivity). Released mediatois inciease venulai
peimeability and modulate the ielease of bio-
logically active molecules fiom othei cell types.
Often with atopic backgiound. Antigens: food
(milk, eggs, wheat, shellfish, nuts), theiapeutic
agents, diugs (penicillin) (see also Diug-In-
duced Uiticaiia," Section 22), helminths. Most
often acute (Figs. 14-8 and 22-6).
IA8I 14-1 Etio|oy and C|assification of Urticaria/Anioedema
|rruuo|o|c u|||c+||+ due |o r+| ce||-|e|e+|u +eu|, peudo+||e|eu, ACE |u|||||o|
|Ered|+|ed u|||c+||+ |d|op+|||c u|||c+||+
Corp|ereu|red|+|ed u|||c+||+ |ou|rruue cou|+c| u|||c+||+
Au|o|rruue u|||c+||+ u|||c+||+ +oc|+|ed W||| .+cu|+|/couuec||.e ||ue +u|o|rruue d|e+e
|rruue cou|+c| u|||c+||+ ||||uc| +u|oeder+ ( u|||c+||+) ,ud|ore
||,|c+| ne|ed||+|, +u|oeder+
|e|ro|+p||r Au|oeder+u|||c+||+eo|uop||||+ ,ud|ore
Co|d u|||c+||+
'o|+| u|||c+||+
C|o||ue||c u|||c+||+
||eu|e +u|oeder+
\|||+|o|, +u|oeder+
|0IE. ACE, +u|o|eu|ucou.e|||u eu/,re.
FICk 14-6 rtcara w|e+| W||| W|||e|o||||p|u| co|o| |u ||e |+ce |u + c|oeup .|eW. I|ee +|e ||e
c|+|c |e|ou o| u|||c+||+. || | c|+|+c|e||||c ||+| ||e, +|e ||+u|eu| +ud ||||, p|u||||c.
Cvmp|ement MedIuted By way of immune
complexes activating complement and ieleasing
anaphylatoxins that induce mast cell degianula-
tion. Seium sickness, administiation of whole
blood, immunoglobulins. Acute.
AutvImmune Common, chionic. Autoanti-
bodies against Fc RI and/oi IgE. Positive au-
tologous seium skin test. Clinically, patients
with these autoantibodies (up to 40% of
patients with chionic uiticaiia) aie indistin-
guishable fiom those without them (Fig. 14-9).
These autoantibodies may explain why plas-
mapheiesis, intiavenous immunoglobulins, and
cyclospoiine induce iemission of disease activ-
ity in these patients.
1mmunv|vgIc Cvntuct UrtIcurIu Usually in
childien with atopic deimatitis sensitized to
enviionmental alleigens (giass, animals) oi
individuals sensitized to weaiing latex iubbei
gloves; can be accompanied by anaphylaxis.
Fhysca| rtcaras DermvgruphIsm Lineai
uiticaiial lesions occui aftei stioking oi
sciatching the skin; they itch and fade in 30
min (Fig. 14-10). 4.2% of the noimal popula-
tion have it; symptomatic deimogiaphism is a
nuisance.
Cv|d UrtIcurIu Usually in childien oi young
adults; uiticaiial lesions confined to sites
exposed to cold occuiiing within minutes aftei
iewaiming. Ice cube" test (application of an
ice cube foi a few minutes to skin) establishes
diagnosis.
Sv|ur UrtIcurIu Uiticaiia aftei solai exposuie.
Action spectium fiom 290-500 nm; whealing
lasts foi <1 h, may be accompanied by syncope;
histamine is one of the mediatois (see Section
10 and Fig. 10-10).
Chv|InergIc UrtIcurIu Exeicise to the point of
sweating piovokes typical small, papulai, highly
piuiitic uiticaiial lesions (Fig. 14-11). May be
accompanied by wheezing.
AquugenIc UrtIcurIu Veiy iaie. Contact with
watei of any tempeiatuie induces eiuption
similai to cholineigic uiticaiia.
Pressure AngIvedemu Eiythematous swelling
induced by sustained piessuie (buttock swelling
when seated, hand swelling aftei hammeiing,
foot swelling aftei walking). Delayed (30 min
to 12 h). Painful, may peisist foi seveial days,
and inteifeies with quality of life. No laboiatoiy
abnoimalities; fevei may occui. Uiticaiia may
occui in addition to angioedema.
FICk 14-T Acute urtcara 'r+|| +ud |+|e W|e+| W||| e|,||er+|ou |o|de| +ud + ||||e| co|o| ceu
||+||,. we||de||ued. I|e |e|ou ou ||e |e|| uppe| +|r | |||de||ued +| || |oWe| |o|de| W|e|e || | |e|e|u.
FICk 14-8 Acute urtcara aod aooedema |o|e ||+| ||e|e +|e |o|| upe|||c|+| W|e+| +ud deep,
d|||ue eder+. 0ccu||ed +||e| ||e p+||eu| |+d e+|eu |e|||||. ne |+d |r||+| ep|ode p|e.|ou|, |u| ue.e| cou
|de|ed e+|ood + ||e c+ue.
FICk 14-9 Chrooc urtcara C||ou|c
u|||c+||+ o| 5,e+| du|+||ou |u +u o||e|W|e
|e+|||, !5,e+|o|d |er+|e. E|up||ou occu|
ou +u +|ro| d+||, |+| +ud, + ||e, +|e
||||, p|u||||c, |e+||, |rp+|| ||e p+||eu|'
qu+|||, o| |||e. A|||ou| upp|eed |,
+u|||||+r|ue, ||e|e | +u |rred|+|e |ecu|
|euce +||e| ||e+|reu| | |opped. kepe+|ed
|+|o|+|o|, +ud c||u|c+| e\+r|u+||ou |+.e uo|
|e.e+|ed +u +pp+|eu| c+ue.
VIhrutIvn AngIvedemu May be familial
(autosomal dominant) oi spoiadic. Raie. It
is believed to iesult fiom histamine ielease
fiom mast cells caused by a vibiating" stimu-
lus-iubbing a towel acioss the back pioduces
lesions, but diiect piessuie (without move-
ments) does not.
rtcara 0ue to Mast Ce||-ke|easo Aeots aod
Fseudoa||ereos aod Chrooc Idopathc rtcara
Uiticaiia/angioedema and even anaphylaxis-
like symptoms may occui with iadiocontiast
media and as a consequence of intoleiance
to salicylates, food pieseivatives and additives
(e.g., benzoic acid and sodium benzoate), as
well as seveial azo dyes, including taitiazine and
sunset yellow (pseudoalleigens) (Fig. 14-9); also
to ACE inhibitois. May be acute and chionic. In
chionic idiopathic uiticaiia, histamine deiived
fiom mast cells in the skin is consideied the
majoi mediatoi. Eicosanoids and neuiopeptides
may also play a pait in pioducing the lesions.
Nvn-1mmune Cvntuct UrtIcurIu Due to
diiect effects of exogenous uiticants penetiating
into skin oi blood vessels. Localized to site of
contact. Soibic acid, benzoic acid in eye solu-
tions and foods, cinnamic aldehydes in cosmet-
ics, histamine, acetylcholine, seiotonin in nettle
stings.
rtcara Assocated wth vascu|ar[Coooectve
Issue Autommuoe 0sease Uiticaiial lesions
may be associated with systemic lupus eiy-
thematosus (SLE) and Sjgien syndiome. How-
evei, in most instances they iepiesent uiticaiial
vasculitis (page 407). This is a foim of cutane-
ous vasculitis associated with uiticaiial skin le-
sions that peisist foi >12 to 24 h. Slow changes
in size and configuiation, can be associated with
puipuia, and can show iesidual pigmentation
due to hemosideiin aftei involution (see Fig.
14-41). Often associated with hypocomplemen-
temia and ienal disease.
0stoct Aooedema ( rtcara) Syodromes
HeredItury AngIvedemu (HAE) A seiious au-
tosomal dominant disoidei; may follow tiauma
(physical and emotional). Angioedema of the
face (Fig. 14-12) and extiemities, episodes of
laiyngeal edema, and acute abdominal pain
caused by angioedema of the bowel wall pie-
senting as suigical emeigency. Uiticaiia iaiely
occuis. Laboiatoiy abnoimalities involve the
complement system: decieased levels of C1-
esteiase inhibitoi (85%) oi dysfunctional in-
hibitoi (15%), low C4 value in the piesence of
noimal C1 and C3 levels. Angioedema iesults
fiom biadykinin foimation, since C1-esteiase
inhibitoi is also the majoi inhibitoi of the
Hageman factoi and kallikiein, the two enzymes
iequiied foi kinin foimation. Episodes can be
life thieatening.
AngIvedemu-UrtIcurIu-EvsInvphI|Iu Syndrvme
Seveie angioedema, only occasionally with piu-
iitic uiticaiia, involving the face, neck, extiemi-
ties, and tiunk that lasts foi 7-10 days. Theie
is fevei and maiked inciease in noimal weight
(incieased by 10-18%) owing to fluid ieten-
tion. No othei oigans aie involved. Laboia-
toiy abnoimalities include stiiking leukocytosis
(20,000-70,000/L) and eosinophilia (60-80%
eosinophils), which aie ielated to the seveiity
of attack. Theie is no family histoiy. This con-
dition is iaie, piognosis is good.
0ermatopatho|oy Edema of the deimis oi
subcutaneous tissue, dilatation of venules but
no evidence of vasculai damage. Mast cell
degianulation. The piedominant peiivasculai
inflammatoiy cell types aie activated lym-
phocytes of the T helpei phenotype.
Sero|oy Seaich foi hepatitis B-associated
antigen, assessment of the complement sys-
tem, assessment of specific IgE antibodies by
iadioalleigosoibent test (RAST), anti-Fc RI
autoantibodies. Seiology foi lupus and Sj-
gien syndiome. Autologous seium skin test foi
autoimmune uiticaiia.
hemato|oy The eiythiocyte sedimentation
iate (ESR) is often elevated in uiticaiial vas-
culitis, and theie may be hypocomplemen-
temia; tiansient eosinophilia in uiticaiia fiom
ieactions to foods, paiasites, and diugs; high
levels of eosinophilia in the angioedema-uiti-
caiia-eosinophilia syndiome.
Comp|emeot Studes Scieening foi functional
C1 inhibitoi in HAE.
|trasoooraphy Foi eaily diagnosis of bowel
involvement in HAE; if abdominal pain is
piesent, this may indicate edema of the bowel.
Farasto|oy Stool specimen foi piesence of
paiasites.
0IACN0SIS
A detailed histoiy (pievious diseases, diugs,
foods, paiasites, physical exeition, solai expo-
suie) is of utmost impoitance. Histoiy should
diffeientiate between ye o[ |esons -uiticaiia,
angioedema, oi uiticaiia - angioedema; Jura-
on o[ |esons (<1 h oi 1 h), rurus , an
on walking (in foot involvement), [|us|ng ,
|urnng , and w|ee:ng (in cholineigic uiti-
caiia). Feer in seium sickness and in the
FICk 14-10 rtcara: dermoraphsm u|||c+||+ + || +ppe+|ed 5 r|u +||e| ||e p+||eu| W+ c|+|c|ed ou ||e
|+c|. I|e p+||eu| |+d e\pe||euced eue|+||/ed p|u|||u |o| e.e|+| rou|| W||| uo pou|+ueou|, occu|||u u|||c+||+.
FICk 14-11 Cho|oerc urtcara 'r+|| u|||c+||+| p+pu|e ou uec| occu|||u W||||u !0 r|u o| .|o|ou
e\e|c|e. |+pu|+| u|||c+||+| |e|ou +|e |e| eeu uude| |de |||||u.
angioedema-uiticaiia-eosinophilia syndiome;
in angioedema, |oarseness , srJor , Jysnea . r-
|ra|ga (seium sickness, uiticaiial vasculitis),
a|Jomna| to|t|y an in HAE. A caieful his-
toiy of medications including penicillin, aspi-
iin, nonsteioidal anti-inflammatoiy diugs, and
ACE inhibitois should be obtained. Autoim-
mune uiticaiia is tested by the autologous se-
ium skin test and deteimination of anti-Fc RI
antibody .
Deimogiaphism is evoked by stioking the
skin; piessuie uiticaiia is tested by application
of piessuie (weight) peipendiculai to the skin;
vibiation angioedema by a vibiatoiy stimulus,
like iubbing the back with a towel. If |ysta|
urtara is suspected, appiopiiate challenge test-
ing should be peifoimed. C|o|nergt urtara
can best be diagnosed by exeicise to sweating
and intiacutaneous injection of acetylcholine
oi mecholyl, which will pioduce miciopapulai
whealing. So|ar urtara is veiified by testing
with UVB, UVA, and visible light. Co|J urtara
is veiified by a wheal iesponse to the application
to the skin of an ice cube oi a test tube containing
ice watei. If uiticaiial wheals do not disappeai in
24 h, uiticaiial vasculitis should be suspected
IMAC 14-1 App|o+c| |o ||e p+||eu| W||| u|||c+||+/+u|oeder+. ACE +u|o|eu|ucou.e|||u eu/,re, |E,
|rruuo|o|u||u E, ||n, |u|||||o|, , dec|e+ed. '0ukCE. ||or A| K+p|+u, |u K wo||| e| +| (ed). ||cc|:|
|-c||, C--c| !-!:- 1|| ed. |eW \o||, \cC|+Wn|||, 2003, p !!9.
and a biopsy done. The peison with angoeJema-
urtara-eosno||a synJrome has high fevei,
high leukocytosis (mostly eosinophils), a stiiking
inciease in body weight due to ietention of wa-
tei, and a cyclic pattein that may occui and iecui
ovei a peiiod of yeais. HereJary angoeJema
has a positive family histoiy and is chaiacteiized
by angioedema of the face and extiemities as the
iesult of tiauma, abdominal pain, and decieased
levels of C4 and C1-esteiase inhibitoi .
A piactical appioach to the diagnosis of
uiticaiia/angioedema is shown in Image 14-1.
C0kS AN0 Fk0CN0SIS
Half the patients with uiticaiia alone aie fiee of
lesions in 1 yeai, but 20% have lesions foi >20
yeais. Piognosis is good in most syndiomes
except HAE, which may be fatal if untieated.
MANACMNI
Freveotoo Tiy to pievent attacks by elimina-
tion of etiologic chemicals oi diugs: aspiiin and
food additives, especially in chionic iecuiient
uiticaiia-iaiely successful; pievent tiiggei in
physical uiticaiias.
Aothstamoes H
1
blockeis, e.g., hydioxyzine,
teifenadine; oi loiatadine, cetiiizine, fexofena-
dine. 180 mg/d of fexofenadine oi 10-20
mg/d of loiatadine usually contiols most cases
of chionic uiticaiia, but cessation of theiapy
usually iesults in a iecuiience; if they fail,
H
1
and H
2
blockeis (cimetidine) and/oi mast
cell-stabilizing agents (ketotifen). Doxepin, a
tiicyclic antidepiessant with maiked H
1
an-
tihistaminic activity, is valuable when seveie
uiticaiia is associated with anxiety and depies-
sion.
Fredosooe In atue uiticaiia with angio-
edema; also foi angioedema-uiticaiia-
eosinophilia syndiome.
0aoato| or Staooto|o| Long-teim theiapy
foi heieditaiy angioedema; watch out foi hii-
sutism, iiiegulai menses; whole fiesh plasma
oi C1-esteiase inhibitoi in the acute attack. A
veiy effective biadikin-B
2
-ieceptoi antagonist
foi subcutaneous application is now available
in Euiope (Icatibant).
0ther In t|ront Joa|t oi auommune ui-
ticaiia, if no iesponse to antihistamines: switch
to cyclospoiine and tapei giadually, if gluco-
coiticoids aie contiaindicated oi if side effects
occui.
FICk 14-12 heredtary aooedema . 'e.e|e eder+ o| ||e |+ce du||u +u ep|ode |e+d|u |o |o
|eque d|||u|ereu|. 8. Au|oeder+ W||| u||de W||||u |ou|. I|ee +|e ||e uo|r+| |e+|u|e o| ||e p+||eu|. I|e
p+||eu| |+d + po|||.e |+r||, |||o|, +ud |+d ru|||p|e |r||+| ep|ode |uc|ud|u co||c|, +|dor|u+| p+|u.
8
FI0MI0I0C
Ae oI 0oset Thiid and fouith decades.
Freva|eoce Highest in Tuikey (80-420
patients in 100,000), Japan, Southeast Asia, the
Middle East, southein Euiope. Raie in noithein
Euiope, United States (0.12-0.33 in 100,000).
Sex Males > females, but dependent on ethnic
backgiound.
FAIh0CNSIS
Etiology unknown. In the eastein Meditei-
ianean and East Asia, HLA-B5 and HLA-B51
association; in the United States and Euiope,
no consistent HLA association. The lesions aie
the iesult of leukocytoclastic (acute) and lym-
phocytic (late) vasculitis.
Painful ulceis eiupt in a cyclic fashion in the
oial cavity and/oi genital mucous membianes.
Oiodynophagia and oial ulceis may peisist/
iecui weeks to months befoie othei symptoms
appeai.
Sko aod Mucous Membraoes Aphthvus U|cers
Punched-out ulceis (3 to >10 mm) with iolled
oi oveihanging boideis and neciotic base
(Fig. 14-13); ied iim; occui in ciops (2-10) on
oial mucous membiane (100%) (Fig. 14-13;
see also Fig. 34-xx), vulva, penis, and sciotum
(Figs. 14-14, 14-15, 35-15); veiy painful.
Erythemu Nvdvsum-LI|e LesIvns Painful in-
flammatoiy nodules on the aims and legs
(40%) (see Section 7 and Fig. 7-23).
Other Inflammatoiy pustules, supeificial
thiombophlebitis (see Fig. 16-6), n[|amma-
ory |aques iesembling those in Sweet syndiome
k+|e, Wo||dW|de occu||euce, |u| ||ou|, .+||+||e
e||u|c p|e.+|euce.
|| | + pe|p|e\|u ru|||,|er .+cu||||c d|e+e
W||| ru|||o|+u |u.o|.ereu|.
\+|u ,rp|or +|e |ecu||eu| o|+| +p|||ou
u|ce|, eu||+| u|ce|, e|,||er+ uodour, upe|
||c|+| |||or|op||e||||, ||u pu|u|e, |||doc,c||||,
+ud po|e||o| u.e|||.
Add|||ou+| ,rp|or r+, |e +|||||||, ep|d|
d|r|||, ||eocec+| u|ce|+||ou, .+cu|+| +ud ceu||+|
ue|.ou ,|er (C|') |e|ou.
C||ou|c |e|+p|u p|o|e|.e cou|e W||| po|eu
||+||, poo| p|ouo|.
8hI 0ISAS |C|9 . 19.+
|C|0 . \!5.2
(acute febiile neutiophilic deimatosis) (see Fig.
7-30), yoJerma gangrenosum-||e |esons (see
Fig. 7-26), a|a||e ururt |esons of neciotiz-
ing vasculitis (see Fig. 14-34).
Systemc Fodos Eyes Leading cause of
moibidity. Posteiioi uveitis, anteiioi uveitis,
ietinal vasculitis, vitieitis, hypopyon, secondaiy
cataiacts, glaucoma, neovasculai lesions.
Muscu|vs|e|etu| Noneiosive, asymmetiic oli-
goaithiitis.
Neurv|vgIc Onset delayed, occuiiing in
one quaitei of patients. Meningoencephalitis,
benign intiacianial hypeitension, cianial neive
palsies, biainstem lesions, pyiamidal/extiapy-
iamidal lesions, psychosis.
Vuscu|ur Aneuiysms, aiteiial occlusions,
venous thiombosis, vaiices; hemoptysis. Coio-
naiy vasculitis: myocaiditis, coionaiy aiteiitis,
endocaiditis, valvulai disease.
G1 Truct Aphthous ulceis thioughout.
0ermatopatho|oy Leukocytoclastic vasculitis
with fibiinoid neciosis of blood vessel walls in
acute eaily lesions; lymphocytic vasculitis in
late lesions.
Fathery Iest Positive patheigy test iead by
physician at 24 oi 48 h, aftei skin punctuie with
a steiile needle. Leads to inflammatoiy pustule.
hIA Iypo Significant association with HLA-
B5 and HLA-B51, in Japanese, Koieans, and
Tuiks, and in the Middle East.
Diagnosis is made accoiding to the Revised
Inteinational Ciiteiia foi Behet disease
(Image 14-2).
FICk 14-13 8ehet dsease 0|+| +p|||ou u|ce|. . I|ee +|e ||||, p+|u|u|, puuc|edou| u|ce| W|||
+ uec|o||c |+e ou ||e |ucc+| ruco+ +ud |oWe| +ud uppe| |o|u|\ |u ||| 23,e+|o|d Iu|||| r+|e. 8. A puuc|ed
ou| u|ce| ou ||e |ouue o| +uo||e| p+||eu|.
8
FICk 14-14 8ehet dsease: eota| u|cers \u|||p|e |+|e +p|||ou|,pe u|ce| ou ||e |+||+| +ud pe||
ue+| ||u. |u +dd|||ou, ||| 25,e+|o|d p+||eu| o| Iu|||| e\||+c||ou |+d +p|||ou u|ce| |u ||e rou|| +ud p|e.|
ou|, e\pe||euced +u ep|ode o| u.e|||.
0IIereota| 0aooss Ora| anJ gena| u|ters :
Viial infection heipes simplex viius (HSV),
vaiicella-zostei viius (VZV)], hand-foot-and-
mouth disease, heipangina, chancie, histoplas-
mosis, squamous cell caicinoma.
C0kS AN0 Fk0CN0SIS
Highly vaiiable couise, with iecuiiences and
iemissions; the mouth lesions aie always
piesent; iemissions may last foi weeks, months,
oi yeais. In the eastein Mediteiianean and East
Asia, seveie couise, one of the leading causes
of blindness. With CNS involvement, theie is a
highei moitality iate.
MANACMNI
Aphthous |cers Potent topical glucocoiti-
coids. Intialesional tiiamcinolone, 3-10 mg/mL,
injected into ulcei base. Thalidomide, 50-100
mg PO in the evening. Colchicine, 0.6 mg PO 2
to 3 times a day. Dapsone, 50-100 mg/d PO.
Systemc Iovo|vemeot Piednisone with oi
without azathiopiine, cyclophosphamide, aza-
thiopiine alone, chloiambucil, cyclospoiine.
IMAC 14-2 ke.|ed |u|e|u+||ou+| C|||e||+ |o| Be|(e| ||e+e (|u|e|u+||ou+| Ie+r |o| ||e ke.||ou o| |CB|,
coo|d|u+|o|. |. |+.+|c||) +cco|d|u |o (A) ||e c|+|||c+||ou ||ee |o|r+|, +ud (8) ||e ||+d|||ou+| |o|r+| AB|,
Ad+r+u||+deBe|(e| d|e+e, Cu, eu||+| u|ce|, 0A, o|+| +p|||ou u|ce|. '0ukCE. CC /ou|ou||. Ad+r+u||+de
Be|(e| d|e+e, |u K wo||| e| +| (ed). ||cc|:| |-c||, C--c| !-!:- 1|| ed. |eW \o||, \cC|+W
n|||, 2003, pp o20-o22.
FICk 14-15 8ehet dsease A |+|e, puuc|edou| u|ce| ou ||e c|o|ur o| + +0,e+|o|d Ko|e+u. I|e
p+||eu| +|o |+d +p|||ou u|ce| |u ||e rou|| +ud pu|u|e ou ||e |||| +ud |u||oc|.
FAkI II |Ek\AI0|0C\ A|| ||IEk|A| \E||C||E 3T0
Raie. Incidence >6 cases pei million, but this
is based on hospitalized patients and does not
include individuals without muscle involve-
ment. Juvenile and adult (>40 yeais) onset.
to|oy Unknown. In peisons >55 yeais of
age, may be associated with malignancy.
C|oca| Spectrum Ranges fiom DM with only
cutaneous inflammation (amyopathic DM) to
polymyositis with only muscle inflammation.
Cutaneous involvement occuis in 30-40% of
adults and 95% of childien with deimato-
myositis/polymyositis. Foi classification, see
Table 14-2.
Photosensitivity. Manifestations in skin
disease may piecede myositis oi vice veisa;
often, both aie detected at the same time. Mus-
cle weakness, difficulty in iising fiom supine
position, climbing staiis, iaising aims ovei
head, tuining in bed. Dysphagia; buining and
piuiitus of the scalp.
Sko Iesoos Peiioibital heliotiope (ieddish
puiple) flush, usually associated with some
degiee of edema (Fig. 14-16). May extend to
involve scalp (- nonscaiiing alopecia), entiie
face (Fig. 14-17), uppei chest, and aims.
In addition, papulai deimatitis with vaiying
degiees of violaceous eiythema in the same
sites. Flat-topped, violaceous papules (Gottion
papule/sign) with vaiious degiees of atiophy
on the nape of the neck and shouldeis and
ovei the knuckles and inteiphalangeal joints
(Fig. 14-17B). Noe : In lupus, lesions usually
occui in the inteiaiticulai iegion of the fingeis
(see Fig. 14-21). Peiiungual eiythema with
|e|r+|or,o||| (|\) | + ,|er|c d|e+e |e
|ou|u |o ||e |d|op+|||c |u||+rr+|o|, r,op+|||e,
+ |e|e|oeueou |oup o| eue||c+||, de|e|r|ued
+u|o|rruue d|e+e |+|e||u ||e ||u +ud/o|
|e|e|+| ruc|e.
|\ | c|+|+c|e||/ed |, .|o|+ceou (|e||o||ope)
|u||+rr+|o|, c|+ue +/ eder+ o| ||e e,e||d
+ud pe||o||||+| +|e+, e|,||er+ o| ||e |+ce, uec|,
+ud uppe| ||uu|, +ud ||+||opped .|o|+ceou p+
pu|e o.e| ||e |uuc||e.
|| | +oc|+|ed W||| po|,r,o|||, |u|e|||||+| pueu
rou|||, +ud r,oc+|d|+| |u.o|.ereu|.
|\ W|||ou| r,op+||, (+r,op+|||c |\) +ud
po|,r,o||| W|||ou| ||u |u.o|.ereu|.
lu.eu||e |\ |uu + d|||e|eu| cou|e +ud | +oc|
+|ed W||| .+cu|||| +ud c+|c|uo|.
Adu||oue| |\ r+, |e +oc|+|ed W||| |u|e|u+|
r+||u+uc,.
||ouo| | u+|ded.
0kMAI0M0SIIIS |C|9 . 10.!
|C|0 . \!!.0
tel-angiectasia, thiombosis of capillaiy loops, in-
faictions. Lesions ovei elbows and knuckles
may evolve into eiosions and ulceis that heal
with stellate scaiiing (paiticulaily in juvenile
DM with vasculitis). Long-lasting lesions may
evolve into poikilodeima (mottled discoloia-
tion with ied, white, and biown) (Fig. 14-18).
Calcification in subcutaneous/fascial tissues
common latei in couise of juvenile DM, pai-
ticulaily about elbows, tiochanteiic, and iliac
iegion (calcinosis cutis); may evolve into calci-
nosis univeisalis.
Musc|e Muscle tendeiness, muscle atiophy.
Piogiessive muscle weakness affecting pioxi-
mal/limb giidle muscles. Difficulty oi inability
to iise fiom sitting oi supine position without
using aims. Difficulty in iaising aims above
head and difficulty in climbing staiis. Inteicos-
tal muscles: difficulty in bieathing.
Occasional involvement of facial/bulbai, pha-
iyngeal, and esophageal muscles. Deep tendon
ieflexes within noimal limits.
0ther 0raos Inteistitial pneumonitis, cai-
diomyopathy, aithiitis, paiticulaily in juvenile
DM (20-65%).
0sease Assocatoo Patients >50 yeais of age
with DM have a highei than expected iisk
foi malignancy, paiticulaily ovaiian cancei in
females. Also caicinoma of the ovaiy, bieast,
bionchopulmonaiy, and GI tiact. Most canceis
detected within 2 yeais of diagnosis.
Chemstry Duiing acute active phase: eleva-
tion of cieatine phosphokinase (65%), which
is most specific foi muscle disease; also, of
aldolase (40%), lactate dehydiogenase, glutamic
oxaloacetic tiansaminase.
SCII0N 14 InE 'K|| || |\\u|E, AuI0|\\u|E, A|| knEu\AI|C ||'0k|Ek' 3T1
Autoaotbodes Autoantibodies to 155 kDa
and/oi Se in 80% to 140 kDa in 58% and to Jo-
1 in 20% (both have a high specificity foi DM)
and to (low specificity) antinucleai antibodies
(ANA, nucleai and speckled pattein) in 40%.
roe Elevated 24-h cieatine excietion (>200
mg/24 h). Note: can also be elevated in gluco-
coiticoid myopathy.
| ectromyoraphy Incieased iiiitability
on inseition of electiodes, spontaneous
fibiillations, pseudomyotonic dischaiges, pos-
itive shaip waves: excludes neuiomyopathy.
With evidence of deneivation, suspect coexist-
ing tumoi.
MkI MRI of muscles ieveals focal lesions.
CC Evidence of myocaiditis; atiial, ventiicu-
lai iiiitability; atiioventiiculai block.
X-kay C|es: inteistitial fibiosis. Eso|agus :
ieduced peiistalsis.
Fatho|oy S|In Flattening of epideimis,
hydiopic degeneiation of basal cell layei, edema
of uppei deimis, scatteied inflammatoiy infil-
tiate, PAS-positive fibiinoid deposits at deimal-
epideimal junction and aiound uppei deimal
capillaiies, accumulation of acid mucopolysac-
chaiides in deimis (all these aie compatible
with DM but aie not diagnostic).
Musc|e Biopsy shouldei/pelvic giidle; one that
is weak oi tendei, i.e., deltoid, supiaspinatus,
gluteus, quadiiceps aftei maiking by EMG oi
IA8I 14-2 Comprehensive C|assification
of |diopathic |nf|ammatory
0ermatomyopathies
0ermatomyosts (0M)
Adu|| oue|
C|+|c |\. +|oue, W||| r+||u+uc,, + p+|| o| +u
o.e||+p couuec||.e ||ue d|o|de|
C||u|c+||, +r,op+|||c |\. +r,op+|||c |\. |,po
r,op+|||c |\
lu.eu||e oue|
C|+|c |\
C||u|c+||, +r,op+|||c |\. +r,op+|||c |\, |,po
r,op+|||c |\
Fo|ymyosts (FM)
|\ +|oue
|\ + p+|| o| +u o.e||+p couuec||.e ||ue d|o|de|
|\ +oc|+|ed W||| |u|e|u+| r+||u+uc,
c
Ioc|usoo 8ody Myosts
0ther C|oca|-Fatho|oc Subroups oI Myosts
|oc+| r,o||| Eo|uop||||c r,o|||
||o|||e|+||.e r,o||| C|+uu|or+|ou r,o|||
0||||+| r,o|||
c
A|||ou| popu|+||ou|+ed Eu|ope+u |ud|e |+.e uoW c|e+||,
cou|||red ||+| +du||oue| c|+|c |\ | +oc|+|ed W||| + |u|||c+u|
||| |o| |u|e|u+| r+||u+uc,, || uc| + |e|+||ou||p e\|| |o| |\, || |
ruc| We+|e|.
FICk 14-16 0ermatomyosts ne||o||ope (|edd|| pu|p|e) e|,||er+ o| uppe| e,e||d +ud eder+ o| ||e
|oWe| ||d. I|| 55,e+|o|d |er+|e |+d e\pe||euced e.e|e ruc|e We+|ue o| ||e |ou|de| ||d|e +ud p|eeu|ed
W||| + |urp |u ||e ||e+| ||+| p|o.ed |o |e c+|c|uor+.
FICk 14-1T 0ermatomyosts . \|o|+ceou e|,||er+ +ud eder+ ou ||e |+ce, p+|||cu|+||, |u ||e pe||o||||+|
+ud r+|+| |e|ou. I|e p+||eu| cou|d |+|e|, |||| || +|r +ud cou|d uo| c||r| |+||. ne |+d pu|rou+|, c+|c|uor+. 8.
\|o|+ceou e|,||er+ +ud Co|||ou p+pu|e ou ||e do|+ o| ||e |+ud +ud ||ue|, epec|+||, o.e| ||e re|+c+|pop|+
|+ue+| +ud |u|e|p|+|+ue+| jo|u|, ||e ||||p|o|ec|ed +|e+ o| ||e |o|e+|r o| ||| 23,e+|o|d r+|e W||| e.e|e ruc|e
We+|ue We|e uo| |u.o|.ed. |e||uuu+| e|,||er+ +ud |e|+u|ec|+|. I|| p+||eu| |+|e| de.e|oped e.e|e c+|c|uo| cu||.
8
MRI. Histology-segmental neciosis within
muscle fibeis with loss of cioss-stiiations; waxy/
coagulative type of eosinophilic staining; with oi
without iegeneiating fibeis; inflammatoiy cells,
histiocytes, maciophages, lymphocytes, plasma
cells. Vasculitis is seen in juvenile DM. MRI-
guided needle biopsy of muscle may ieplace con-
ventional muscle biopsy in the futuie.
Skin signs plus pioximal muscle weakness with
two of thiee laboiatoiy ciiteiia, i.e., elevated
seium muscle enzyme" levels, chaiacteiistic
electiomyogiaphic changes, diagnostic muscle
biopsy. Diffeiential diagnosis is to seboiiheic
deimatitis, lupus eiythematosus, mixed con-
nective tissue disease, steioid myopathy, tiichi-
nosis, toxoplasmosis.
C0kS AN0 Fk0CN0SIS
Piognosis guaided but with tieatment, it is
ielatively good except in patients with malig-
nancy and those with pulmonaiy involvement.
With aggiessive immunosuppiessive tieatment
the 8-yeai suivival iate is 70-80%. A bet-
tei piognosis is seen in individuals who ie-
ceive eaily systemic tieatment. The eaily and
aggiessive use of glucocoiticoids has ieduced
the moitality iates in childien to <10%. The
most common causes of death aie malignancy,
infection, caidiac and pulmonaiy disease. Suc-
cessful tieatment of an associated neoplasm
is often followed by impiovement/iesolution
of DM.
MANACMNI
Fredosooe 0.5-1 mg/kg body weight pei day,
incieasing to 1.5 mg/kg if lowei dose ineffective.
Tapei when muscle enzyme" levels appioach
noimal. Best if combined with azathiopiine,
2-3 mg/kg pei day. Noe: Steioid myopathy may
occui aftei 4-6 weeks of theiapy.
A|teroatves Methotiexate, cyclophospha-
mide, cyclospoiine, anti-tumoi neciosis factoi
(TNF) agents. High-dose IV immunoglobu-
lin bolus theiapy (2 g/kg body weight given ovei
2 days) at monthly inteivals spaies glucocoiti-
coid doses to achieve oi maintain iemissions.
FICk 14-18 0ermatomyosts, juveo|e ooset, pok|oderma I|e|e | ro|||ed, |e||cu|+| ||oWu||
p|reu|+||ou +ud |e|+u|ec|+|+ p|u r+|| W|||e c+|. |o|e |||+e ou ||oc|+u|e||c +|e+ due |o ,|er|c |ucoco|
||co|d ||e|+p,.
II0I0C
IIk A physiologic phenomenon.
SIk That of associated disoidei; following
amantadine tieatment foi Paikinson disease.
FAIh0CNSIS
ILR pattein due to vasospasm oi obstiuction
of peipendiculai aiteiioles, peifoiating deimis
fiom below. Cyanotic peiipheiy of each web of
net caused by deoxygenated blood in suiiound-
ing hoiizontally aiianged venous plexuses.
When factois such as cold cause incieased vis-
cosity oi flow iates in supeificial venous plexus,
fuithei deoxygenation occuis and cyanotic ie-
ticulai pattein becomes moie pionounced. El-
evation of limb decieases intensity of coloi due
to incieased venous diainage. SLR iesults fiom
aiteiiolai disease causing obstiuction to inflow
and blood hypeiviscosity oi fiom obstiuction
to outflow of blood in venules.
Appeaiance oi woisening with cold exposuie.
Numbness, tingling associated. Woise duiing
wintei months.
Sko Iesoos 1LR A puiple/livid discoloia-
tion of skin in netlike pattein (mesh diametei
3 cm) involving laige aieas of lowei oi uppei
extiemities and tiunk and disappeaiing aftei
waiming.
SLR Blotchy, aiboiizing, lightning-like, stai-
buist, oi mottled pattein of cyanosis (Fig.
14-19). Netlike webs aie open (semiciiculai),
and within webs, skin is noimal to pallid and
||.edo |e||cu|+|| (|k) | + ro|||ed ||u|| (||.|d)
d|co|o|+||ou o| ||e ||u ||+| occu| |u + ue||||e
p+||e|u. || | uo| + d|+uo| |u ||e|| |u| + |e+c||ou
p+||e|u.
C|+|||c+||ou d|||uu||e |e|Weeu
|!c||: |.-! -|:|c (||k) . + pu|p|e/||.|d
d|co|o|+||ou o| ||e ||u |u + ue||||e p+||e|u
d|+ppe+||u +||e| W+|r|u. A p|,|o|o|c p|e
uoreuou. ( ',,. cu|| r+|ro|+|+.)
'-:!c, (,|c|:) |.-! -|:|c ('|k) .
+ pu|p|e d|co|o|+||ou occu|||u |u + |+||u|| o|
||||u|u|||e p+||e|u, ue||||e |u| W||| opeu (uo|
+uuu|+|) re|e, ro||,, |u| uo| +|W+,, cou||ued
|o ||e |oWe| e\||er|||e +ud |u||oc|. A |e+c||ou
p+||e|u o||eu |ud|c+||.e o| e||ou ,|er|c d|
e+e (I+||e +!). ( ',,. ||.edo |+cero+.)
IIv00 kIICIAkIS |C|9 . ++o.20
|C|0 . | 95.0
feels cool. Symmetiic, aims/legs, buttocks; less
commonly, body. On exposuie to cold, livedo
becomes moie pionounced but nevei fades
completely on waiming. It nevei ulceiates.
Noe : When associated with |eJoJ astu|s
(see p. 475), ulceiation about ankles and foie-
feet may occui.
Ceoera| xamoatoo Symptoms of undeily-
ing disease in SLR (Table 14-3).
Iaboratory Vaiies with associated disoideis.
0ermatopatho|oy Vasculai pathology of un-
deilying disease.
Clinical diagnosis confiimed by laboiatoiy data
suppoiting diagnosis of associated disoidei.
0IIereota| 0aooss Cutis maimoiata, ILR
veisus SLR, livedoid vasculitis (see page 475),
eiythema ab igne.
C0kS AN0 Fk0CN0SIS
Couise/piognosis of SLR depends on that of
associated disoidei.
MANACMNI
Keep fiom chilling. Pentoxifylline (400 mg
PO thiee times a day), low-dose aspiiin, and
hepaiin may be helpful.
Tieat associated disoidei.
IA8I 14-3 0isorders Associated with Symptomatic Livedo Reticu|aris
Vasc0|ar 0bstr0ct|oo V|scos|ty 0haoges 0r0gs
A||e|oer|o|| I||or|oc,||er|+ Ar+u|+d|ue
A||e||oc|e|o| |o|,|o|u||uer|+ 0u|u|ue
|o|,+||e|||| uodo+ C|,o|o|u||uer|+ 0u|u|d|ue
Cu|+ueou po|,+||e|||| uodo+ Co|d +|u||uer|+
k|eur+|o|d .+cu|||| ||er|u+|ed |u||+.+cu|+|
||.edo|d .+cu|||| co+u|+||ou
'ueddou ,ud|ore |upu e|,||er+|ou
Au||c+|d|o||p|u ,ud|ore
|eu|er|+/|,rp|or+
FICk 14-19 Symptomatc |vedo retcu|ars A ue||||e, +||o||/|u p+||e|u ou ||e po|e||o| |||| +ud
|u||oc| de||ued |, .|o|+ceou, e|,||er+|ou ||e+| |eer|||u ||||u|u. I|e ||u W||||u ||e e|,||er+|ou
+|e+ | uo|r+||, p+|e. I|| occu||ed |u + p+||eu| W||| |+|||e |,pe||eu|ou +ud ru|||p|e ce|e||o.+cu|+| +||+c| +ud
W+ ||u p+||ouorou|c |o| 'ueddou ,ud|ore.
FI0MI0I0C
Raie but undeidiagnosed.
Skin lesions piecede neuiologic symptoms,
often by yeais.
Sko Iesoos These iepiesent classic SLR on
lowei extiemities, buttocks, sometimes aims (Fig.
14-19). Angiomatosis (mottled-puiple discol-
oiation of the face and othei paits of the body).
Noe : Sneddon syndiome is not identical
with antiphospholipid syndiome, although dei-
matologic manifestations (SLR) may be indis-
tinguishable. May be associated with livedoid
vasculitis-in this case, ulceiation may occui
aiound ankles oi acially (see page 475).
A po|eu||+||, |||e|||e+|eu|u d|e+e o| uu|uoWu
e||o|o, occu|||u ro|e o||eu |u |er+|e ||+u
r+|e +ud r+u||e||u r+|u|, |u ||u + '|k +ud
|u ||e C|'.
Aoc|+|ed W||| ||+u|eu| |c|er|c +||+c| +ud
ce|e||o.+cu|+| |uu||.
SN000N SN0k0M
Neuro|oc Symptoms Include headaches,
labile hypeitension, tiansient ischemic attacks,
tiansient amnesia, tiansient aphasia, palsy, and
ceiebiovasculai insult.
0ermatopatho|oy Endothelitis piolif-
eiation of subendothelial myofibioblasts
vasculai occlusion and fibiosis. Cytotoxic anti-
endothelial cell antibodies in a small peicentage
of patients. Theie may be antiphospholipid
antibodies.
MANACMNI
Longtime low-dose hepaiin, aspiiin.
|E | ||e de|u+||ou o| + pec||ur o| d|e+e
p+||e|u ||+| +|e ||u|ed |, d|||uc| c||u|c+| ||ud
|u +ud d|||uc| p+||e|u o| ce||u|+| +ud |uro|+|
+u|o|rruu||,.
|E occu| ro|e corrou|, |u Woreu (r+|e |o
|er+|e |+||o . 9).
|E |+ue ||or |||e|||e+|eu|u r+u||e|+||ou o|
+cu|e ,|er|c |E ('|E) |o ||e ||r||ed +ud e\c|u
|.e ||u |u.o|.ereu| |u c||ou|c cu|+ueou |E
(CC|E) (|r+e +!). \o|e ||+u 35 o| p+||eu|
W||| |E |+.e ||u |e|ou, W||c| c+u |e c|+|||ed
|u|o |Epec|||c +ud uoupec|||c.
Au +|||e.|+|ed .e||ou o| C||||+r c|+|||c+||ou o|
|Epec|||c ||u |e|ou | |.eu |u I+||e ++.
Acu|e cu|+ueou |E (AC|E) | p|+c||c+||, +|
W+, +oc|+|ed W||| '|E, u|+cu|e cu|+ueou |E
('C|E) |u +|ou| 50, +ud c||ou|c cu|+ueou |E
(CC|E) ro| o||eu |+ ou|, ||u d|e+e. noW
e.e|, CC|E |e|ou c+u occu| |u '|E.
AC|E +ud 'C|E +|e ||||, p|o|oeu|||.e.
IFS kIhMAI0SS (I) |C|9 . o95.+
|C|0 . |9!
SCII0N 14 InE 'K|| || |\\u|E, AuI0|\\u|E, A|| knEu\AI|C ||'0k|Ek' 3TT
IA8I 14-4 Abbreviated Ci||iam C|assification of Skin Lesions of LE
|. |Epec|||c ||u d|e+e cu|+ueou |E
*
(C|E)|
A. Acu|e cu|+ueou |E AC|E|
. |oc+||/ed AC|E (r+|+| |+|, |u||e|||, |+|)
2. Ceue|+||/ed AC|E (r+cu|op+pu|+| |upu |+|, r+|+| |+|, p|o|oeu|||.e |upu de|r+||||)
B. 'u|+cu|e cu|+ueou |E 'C|E|
. Auuu|+| 'C|E
2. |+pu|oqu+rou 'C|E (d|er|u+|ed ||E, u|+cu|e d|er|u+|ed |E, r+cu|op+pu|+| p|o|oeu|||.e |E)
C. C||ou|c cu|+ueou |E CC|E|
. C|+|c d|co|d |E ||E|. (+) |oc+||/ed ||E, (|) eue|+||/ed ||E
2. n,pe|||op||c/.e||ucou ||E
!. |upu p|o|uudu
+. \uco+| ||E. (+) o|+| ||E, (|) coujuuc||.+| ||E
5. |upu |ur|du (u|||c+||+| p|+que o| |E)
o. C|||||+|u |E (c|||||+|u |upu)
1. ||c|euo|d ||E (|E/||c|eu p|+uu o.e||+p)
||. |Euoupec|||c ||u d|e+e
I|ee |+ue ||or uec|o||/|u +ud u|||c+||+| .+cu|||| |o ||.edo |e||cu|+||, k+,u+ud p|euoreuou, de|r+| ruc|uo|,
+ud |u||ou |e|ou |u |E.
A||e|u+||.e o| ,uou,rou |e|r +|e |||ed |u p+|eu||ee, +|||e.|+||ou +|e |ud|c+|ed |u ||+c|e|.
'0ukCE. kep||u|ed +ud rod|||ed ||or k| 'ou||e|re| W||| pe|r||ou ||or '|oc||ou lou|u+|, \+cr|||+u ||e, ||d.
IMAC 14-3 I|e pec||ur o| |upu e|,||er+|ou, + eu.|+ed |, ||e |+|e ||. l+re |. C||||+r. I|e |e||
corp||e coud|||ou ||+| de||ue cu|+ueou d|e+e ou|, +ud || c+u |e eeu ||+| c||ou|c cu|+ueou |upu e\|eud
|u|o ||e ,|er|c d|e+e ec||ou. I|| | +|o ||ue |o| |upu p|o|uudu (|upu p+uu|cu||||) +ud u|+cu|e cu|+ue
ou |upu, W|e|e+ +cu|e cu|+ueou |upu | c|+|+c|e||||c |o| ,|er|c d|e+e ou|,. I|e |o||or |oW ||+|
|rruue corp|e\ d|e+e dor|u+|e ,|er|c d|e+e +ud ce||red|+|ed |rruu||, (C\|) | p|edor|u+u| |u ||e
cu|+ueou d|e+e r+u||e|+||ou.
C.M.
mmune
complex
disease
Chronic
cutaneous
LE
Acute
cutaneous
LE
s
u
o
e
n
a
u
c
e
t
u
c
a
b
u
S
LE
u rof n P du E s L
Y
L
N
O
E
S
A
E
S
D
S
U
O
E
N
A
T
U
C
S
Y
S
T
E
M
C
D
S
E
A
S
E
FI0MI0I0C
Freva|eoce
Noithein Euiopeans to moie than 200/100,000
Ae oI 0oset
Sex
kace Moie common in blacks.
Frecptato Factors
sunlight (UVR) is the most effective piecipitat-
ing factoi. An SLE syndiome can be induced by
diugs (hydialazine, ceitain anticonvulsants, and
piocainamide), but iash is a ielatively uncom-
mon featuie of diug-induced SLE.
(chionic). Sunlight may cause an exaceibation
of SLE (36%). Piuiitus, buining of skin lesions.
Fatigue (100%), fevei (100%), weight loss, and
malaise. Aithialgia oi aithiitis, abdominal pain,
Sko Iesoos
14-4) in the acute phases of the disease and SCLE
and CCLE lesions. Wheieas ACLE lesions occui
only in acute oi subacute SLE, SCLE and CCLE
lesions aie piesent in subacute and chionic SLE
but may also occui in acute SLE. ACLE lesions
ACI Butter]|y Rush
ent, maculai butteifly eiuption on the face
(Fig. 14-20), shaiply defined with fine scaling;
Generu|Ized
oi uiticaiial lesions on the face, on the doisa of
hands (Fig. 14-21
Others Bu||ae
flaies). Pau|es sta|y |aques
(Fig. 14-22) and JstoJ |aques
(Fig. 14-23), piedominantly on the face and
I|| e||ou ru|||,|er +u|o|rruue d|e+e
| |+ed ou po|,c|ou+| B ce|| |rruu||,, W||c|
|u.o|.e couuec||.e ||ue +ud ||ood .ee|.
\o|e corrou |u pe|ou W||| ||+c| A|||c+u |e|||
+e, r+|e |o |er+|e |+||o . 9.
I|e c||u|c+| r+u||e|+||ou |uc|ude |e.e| (90),
||u |e|ou (35), +|||||||, C|', |eu+|, c+|d|+c,
+ud pu|rou+|, d|e+e.
'||u |e|ou +|e ||oe o| AC|E +ud 'C|E, uo|
uucorrou|, o| CC|E.
'|E r+, uucorrou|, de.e|op |u p+||eu| W|||
CC|E, ou ||e o||e| |+ud, |e|ou o| CC|E +|e
corrou |u '|E (|r+e +!).
SSIMIC IFS kIhMAI0SS |C|9 . 10.0
|C|0 . |9!
on the aims and scalp. Eiythematous, some-
times violaceous, slightly scaling, densely set
and ton[|uen au|es
fingei, usually with spaiing of the aiticulai
iegions (Fig. 14-21). Note diffeience to
deimatomyositis (Fig. 14-17B). Pa|mar ery-
|ema B),
na|[o|J e|angetasas
thema, edema of the peiiungual skin, (see
Fig. 33-26). Palpable" puipuia (vasculitis),
lowei extiemities (see Fig. 14-34). Urtara|
|esons
har
Mucous Membraoes
puiic neciotic lesions on palate (80%), buccal
SItes v] PredI|ectIvn
piefeientially in light-exposed sites. Face (80%);
scalp (Fig. 32-17) (discoid lesions); piesteinal,
shouldeis; doisa of the foieaims, hands, fingeis,
fingeitips (Fig. 33-31) (Image 14-4).
xtracutaoeous Mu|tsystem Iovo|vemeot
(50%), peiicaiditis (20%), pneumonitis (20%),
gastiointestinal (due to aiteiitis and steiile
peiitonitis), hepatomegaly (30%), myopathy
(30%), splenomegaly (20%), lymphadenopa-
thy (50%), peiipheial neuiopathy (14%), CNS
disease (10%), seizuies oi oiganic biain disease
(14%).
Fatho|oy S|In
faction degeneiation of the deimal-epideimal
junction, edema of the deimis, deimal lym-
phocytic infiltiate, and fibiinoid degeneiation
of the connective tissue and walls of the blood
vessels.
FICk 14-20 Acute systemc |upus erythematosus B|||| |ed, |+|p|, de||ued e|,||er+ W||| ||||
eder+ +ud r|u|r+| c+||u |u + '|u||e|||, p+||e|u ou ||e |+ce. I|| | ||e |,p|c+| 'r+|+| |+|. |o|e +|o ||+| ||e
p+||eu| | |er+|e +ud ,ouu.
IMAC 14-4 ||ed||ec||ou ||e o| cu|+ueou |upu e|,||er+|ou.
1mmunv]|uvrescence v] S|In The lupus
band test (LBT, diiect immunofluoiescence
demonstiating IgG, IgM, C3) shows gianulai
oi globulai deposits of immune ieactants in a
bandlike pattein along the deimal-epideimal
junction. Positive in lesional skin in 90% and
in the clinically noimal skin (sun-exposed,
70-80%; non-sun-exposed, 50%).
Sero|oy ANA positive (>95%); peiipheial
pattein of nucleai fluoiescence. Anti-double-
stiand DNA antibodies, anti-Sm antibodies,
and iRNP antibodies specific foi SLE; low levels
of complement (especially with ienal involve-
ment). Anticaidiolipin autoantibodies (lupus
anticoagulant) in a specific subset (anticaidioli-
pin syndiome); SS-A(Ro) autoantibodies have
a low specificity foi SLE but aie specific in the
subset of SCLE (see below) (Table 14-5).
hemato|oy Anemia noimocytic, noimo-
chiomic, oi iaiely, hemolytic Coombs-positive,
leukopenia (>4000/L)], lymphopenia, thiom-
bocytopenia, elevated ESR.
roa|yss Peisistent pioteinuiia, casts.
0IACN0SIS
Made on the basis of clinical findings, histopa-
thology, lupus band test, and seiology within
the fiamewoik of the ievised Ameiican Rheu-
matism Association (ARA) ciiteiia foi classifi-
cation of SLE (Table 14-6).
Fk0CN0SIS
Five-yeai suivival is 93%.
MANACMNI
Ceoera| Measures Rest, avoidance of sun
exposuie.
Iodcatoos Ior Fredosooe (60 mg/d in
divided doses): (1) CNS involvement, (2) ie-
nal involvement, (3) seveiely ill patients with-
out CNS involvement, (4) hemolytic ciisis, (5)
thiombocytopenia.
Coocomtaot Immuoosuppressve 0rus Aza-
thiopiine, mycophenolate mofetil, methotiexate,
cyclophosphamide, depending on oigan in-
volvement and activity of disease. In ienal dis-
ease, cyclophosphamide IV bolus theiapy.
Aotma|ara|s Hydioxychloioquine is useful
foi tieatment of the skin lesions in subacute and
chionic SLE but does not ieduce the need foi
piednisone. Obseive piecautions in the use of
hydioxychloioquine. Alteinative: chloioquine,
quinaciine.
Iovestatooa| Ant-TNF agents; efalizumab,
iituximab, leflunomide, anti-inteifeion
agents.
CIAN0S IFS kIhMAI0SS
ACI CIAN0S I
Foi skin lesions and systemic manifestations see
Systemic Lupus Eiythematosus," above.
IA8I 14-5 Pathoenic Autoantibodies in Systemic Lupus Erythematosus
Aot|geo Spec|I|c|ty Preva|eoce, % Na|o 0||o|ca| IIects
Au||dou||e||+ud ||A 10-30 K|due, d|e+e, ||u d|e+e
|uc|eoore o0-90 K|due, d|e+e, ||u d|e+e
ko !0-+0 '||u d|e+e, ||due, d|e+e, |e|+| |e+|| p|o||er
|+ 5-20 |e|+| |e+|| p|o||er
'r 0-!0 K|due, d|e+e
|\|A |ecep|o| !!-50 B|+|u d|e+e
||op|o||p|d 20-!0 I||or|o|, p|eu+uc, |o
Ac||u|u 20 K|due, d|e+e
Cq +0-50 K|due, d|e+e
|0IE. |\|A, |re||,||+p+||+|e.
'0ukCE. A|||e.|+|ed ||or A k+|r+u, |A |eu|e|. | Eu| l \ed !53.929, 2003.
FICk 14-21 Acute SI . ked|o.|o|+ceou, We||der+|c+|ed p+pu|e +ud p|+que ou ||e do|+ o| ||e
||ue| +ud |+ud, c|+|+c|e||||c+||, p+||u ||e ||u o.e||,|u ||e jo|u|. I|| | +u |rpo||+u| d|||e|eu||+| d|+uo||c
|u W|eu cou|de||u de|r+|or,o|||, W||c| c|+|+c|e||||c+||, |u.o|.e ||e ||u o.e| ||e jo|u| (corp+|e W|||
||. +13 8. |+|r+| e|,||er+ r+|u|, ou ||e ||ue|||p. I|| | p+||ouorou|c.
8
IA8I 14-6 1982 Revised ARA Criteria for C|assification of Systemic Lupus Erythematosus
*
0r|ter|oo 0eI|o|t|oo
. \+|+| |+| ||\ed e|,||er+, ||+| o| |+|ed, o.e| ||e r+|+| er|ueuce, |eud|u |o p+|e
||e u+o|+||+| |o|d.
2. ||co|d |+| E|,||er+|ou |+|ed p+|c|e W||| +d|e|eu| |e|+|o||c c+||u +ud |o|||cu|+|
p|u|u, +||op||c c+|||u r+, occu| |u o|de| |e|ou.
!. ||o|oeu|||.||, '||u |+|e + + |eu|| o| uuuu+| |e+c||ou |o uu||||, |, p+||eu| |||o|, o|
p|,|c|+u o|e|.+||ou.
+. 0|+| u|ce| 0|+| o| u+op|+|,ue+| u|ce|+||ou, uu+||, p+|u|e, o|e|.ed |, +
p|,|c|+u.
5. A||||||| |oue|o|.e +||||||| |u.o|.|u |Wo o| ro|e pe||p|e|+| jo|u|, c|+|+c|e||/ed
|, |eude|ue, We|||u, o| e||u|ou.
o. 'e|o||| +. ||eu||||-cou.|uc|u |||o|, o| p|eu||||c p+|u o| |u| |e+|d |, + p|,|c|+u
o| e.|deuce o| p|eu|+| e||u|ou
|. |e||c+|d|||-docureu|ed |, ECC o| |u| o| e.|deuce o| pe||c+|d|+|
e||u|ou.
1. keu+| d|o|de| +. |e|||eu| p|o|e|uu||+-0.5/d o| !+ || qu+u|||+||ou uo| pe||o|red
|. Ce||u|+| c+|-r+, |e |ed ce||, |ero|o||u, |+uu|+|, |u|u|+|, o| r|\ed.
3. |eu|o|o|c d|o|de| +. 'e|/u|e-|u ||e +|euce o| o||eud|u d|u o| |uoWu re|+|o||c
de|+uereu|, e.., u|er|+, |e|o+c|do|, o| e|ec||o|,|e |r|+|+uce
|. |,c|o|-|u ||e +|euce o| o||eud|u d|u o| |uoWu re|+|o||c
de|+uereu|, e.., u|er|+, |e|o+c|do|, o| e|ec||o|,|e |r|+|+uce.
9. ner+|o|o|c d|o|de| +. nero|,||c +uer|+-W||| |e||cu|oc,|o|
|. |eu|opeu|+- +000/| |o|+| ou |Wo o| ro|e occ+|ou
c. |,rp|opeu|+- 500/| ou |Wo o| ro|e occ+|ou
d. I||or|oc,|opeu|+- 00,000/| |u ||e +|euce o| o||eud|u d|u.
0. |rruuo|o|c d|o|de| +. Au||||A-+u|||od, |o u+||.e ||A |u +|uo|r+| |||e|
|. Au||'r-p|eeuce o| +u|||od, |o 'r uuc|e+| +u||eu
c. |o|||.e ||ud|u o| +u||p|op|o||p|d +u|||od|e |+ed ou () +u
+|uo|r+| e|ur |e.e| o| |C o| |\ +u||c+|d|o||p|u +u|||od|e, (2) +
po|||.e |e| |eu|| |o| |upu +u||co+u|+u| u|u + |+ud+|d re||od,
o| (!) + |+|epo|||.e e|o|o|c |e| |o| ,p|||| |uoWu |o |e po|||.e
|o| +| |e+| o rou|| +ud cou|||red |, ue+||.e --c c||!
|rro||||/+||ou o| ||uo|eceu| ||epouer+| +u|||od, +|o|p||ou |e|.
. Au||uuc|e+| +u|||od, Au +|uo|r+| |||e| o| +u||uuc|e+| +u|||od, |, |rruuo||uo|eceuce o| +u
equ|.+|eu| ++, +| +u, po|u| |u ||re +ud |u ||e +|euce o| d|u
|uoWu |o |e +oc|+|ed W||| 'd|u|uduced |upu ,ud|ore.
*I|e p|opoed c|+|||c+||ou | |+ed ou c|||e||+. |o| ||e pu|poe o| |deu|||,|u p+||eu| |u c||u|c+| |ud|e, + pe|ou |+|| |e +|d |o |+.e '|E ||
+u, + o| ro|e o| ||e c|||e||+ +|e p|eeu|, e||+||, o| |ru||+ueou|,, du||u +u, |u|e|.+| o| o|e|.+||ou.
'0ukCE. kep||u|ed ||or E\ I+u e| +|. A||||||| k|eur 25.21, 932. ued |, pe|r||ou o| ||e Are||c+u Co||ee o| k|eur+|o|o,.
FI0MI0I0C
Ae oI 0oset Young and middle age.
kace Uncommon in blacks oi Hispanics.
Iocdeoce About 10% of the LE population.
Frecptato Factors Sunlight exposuie.
Rathei sudden onset with annulai oi pso-
iiasifoim plaques eiupting on the uppei tiunk,
aims, doisa of the hands, usually aftei exposuie
to sunlight; mild fatigue, malaise; some aithial-
gia, fevei of unknown oiigin.
'||u |e|ou o| 'C|E +|e +uuu|+| o| po||+||o|r
(||. +22).
|+||eu| W||| 'C|E r+, |+.e ore o| ||e c|||e||+
o| '|E + de||ued |, ||e AkA, |uc|ud|u p|o
|oeu|||.||,, +||||+||+, e|o|||, |eu+| d|e+e,
+ud e|o|o|c +|uo|r+||||e. 50 o| p+||eu| W|||
'C|E |+.e '|E.
||+c||c+||, +|| |+.e +u||ko (''A) +ud ro| |+.e
+u|||+ (''B) +u|||od|e. I|e e||ou o|+u
|u.o|.ereu| o| '|E | uucorrou.
I|e c||u|c+| ||u |e|ou +|e ||e d|||uc||.e |e+|u|e
o| 'C|E.
S8ACI CIAN0S IFS kIhMAI0SS (SCI)
Sko Iesoos Two yes : (1) Psoras[orm au-
|osquamous , shaiply defined, with slight delicate
scaling (Fig. 14-22), evolving into biight ied
confluent plaques that aie oval, aicifoim, oi
polycyclic, just as in psoiiasis; and (2) annu|ar ,
biight ied annulai lesions with cential iegies-
sion and little scaling. In both theie may be
telangiectasia, but theie is no folliculai plug-
ging and less induiation than in CCLE. Lesions
iesolve with slight atiophy (no scaiiing) and
hypopigmentation.
DIstrIhutIvn Scatteied, disseminated in light-
exposed aieas: shouldeis, extensoi suiface of
the aims, doisal suiface of the hands, uppei
back, V-neck aiea of the uppei chest.
FICk 14-22 Subacute cutaoeous |upus erythematosus w|de|, c+||e|ed, e|,||er+|ou|o.|o|+ceou,
c+||u, We||der+|c+|ed p|+que ou ||e ||uu|, uec|, +ud +|r, r|r|c||u ||e c||u|c+| +ppe+|+uce o| po||+| .u|+||.
0ther Iesoos Peiiungual telangiectasia, dif-
fuse nonscaiiing alopecia.
0ermatopatho|oy aod Immuoopatho|oy As
in ACLE, LBT positive in 60%.
v Iesto Most patients have a lowei than
noimal UVB minimum eiythema dose (MED).
Typical SCLE lesions may develop in UVB test
sites.
Sero|oy ANA piesent in 60-80%. Antibod-
ies to Ro(SS-A) positive in > 80%, to La(SS-B)
in 30-50%; high levels of ciiculating immune
complexes.
0ther Iaboratory Iests Patients with SCLE,
paiticulaily those with manifest systemic
involvement, may have a numbei of laboia-
toiy abnoimalities, including anemia, leukope-
nia, lymphopenia, hematuiia, pioteinuiia, and
depiessed complement levels.
Clinical findings confiimed by histology and
immunopathology. The extensive involvement
is fai moie than is evei seen in CCLE, and the
distinctive eiuption is a maikei foi SCLE.
0IIereota| 0aooss Red plaques of dei-
matomyositis, secondaiy syphilis, psoiiasis, seb-
oiiheic deimatitis, tinea coipoiis, polymoiphic
light eiuption.
C0kS AN0 Fk0CN0SIS
A bettei piognosis than foi SLE in geneial.
Some patients with ienal (and CNS) involve-
ment have a guaided piognosis. The skin lesions
can disappeai completely, but occasionally, a
vitiligo-like depigmentation iemains foi some
months. Women with Ro(SS-A)-positive SCLE
may give biith to babies with neonatal lupus
and congenital heait block.
MANACMNI
Iopca| Anti-inflammatoiy glucocoiticoids
and topical pimeciolimus and taciolimus aie
only paitially helpful.
Systemc Systemic tieatment usually iequiied.
T|a|JomJe (100-300 mg/d) is veiy effective foi
skin lesions but not foi systemic involvement.
HyJroxyt||oroqune , 400 mg/d; if this does not
contiol the skin lesions, quinaciine hydiochlo-
iide, 100 mg/d, can be added. The bizaiie yellow
skin coloi caused by quinaciine can be some-
what modified by -caiotene, 60 mg tid.
I|| c||ou|c, |udo|eu| ||u d|e+e | c|+|+c|e||/ed
|, |+|p|, r+||u+|ed, c+|,, |u|||||+|ed, +ud |+|e|
+||op||c |ed ('d|co|d) p|+que, uu+||, occu|||u
ou |+|||u+||, e\poed +|e+ (||. +2!, +2+).
I|| d|o|de|, |u ro| c+e, | pu|e|, cu|+ueou
W|||ou| ,|er|c |u.o|.ereu| (|r+e +!).
noWe.e|, CC|E |e|ou r+, occu| |u '|E.
CC|E r+, r+u||e| + c||ou|c d|co|d |E (C||E,
ee |e|oW) o| |E p+uu|cu|||| (I+||e ++).
Chk0NIC CIAN0S IFS kIhMAI0SS (CCI) |C|9 . o95.+
|C|0 . |9!.0
CIASSIC Chk0NIC 0ISC0I0 I (C0I)
FI0MI0I0C
kace Possibly moie seveie in blacks.
Can be piecipitated by sunlight but to a
lessei extent than ACLE oi SCLE. Lesions last
foi months to yeais. Usually no symptoms,
FICk 14-23 Chrooc cutaoeous |upus erythematosus we||der+|c+|ed, e|,||er+|ou, |,pe||e|+|o||c
p|+que W||| +||op|,, |o|||cu|+| p|u|u, +ud +d|e|eu| c+|e ou |o|| c|ee|. I|| | ||e c|+|c p|eeu|+||ou o|
c||ou|c d|co|d |E.
FICk 14-24 Chrooc cutaoeous |upus
erythematosus: scarro I|e|e +|e ru|||p|e
c+||ed p|+que ||+| |+.e + dep|eed ceu|e|
+ud +u +c||.e, |||| e|,||er+|ou +ud c+|, r+|
|u |u ||e |+ce o| ||| o0,e+|o|d |er+|e |+|re|.
'c+|||u |+ |ed |o cou|de|+||e d|||u|ereu|.
sometimes slightly piuiitic oi smaiting. No
geneial symptoms.
Sko Iesoos Biight ied papules evolving
into plaques, shaiply maiginated, with adhei-
ent scaling (Fig. 14-23). Scales aie difficult to
iemove and show spines on the undeisuiface
(magnifying lens) iesembling caipet tacks.
Plaques aie iound oi oval, annulai oi polycy-
clic, with iiiegulai boideis and expand in the
peiipheiy and iegiess in the centei, iesulting
in depiession of lesions, atiophy, and eventu-
ally scaiiing (Fig. 14-24). Folliculai plugging
and dilated follicles may peisist in atiophic le-
sions but eventually disappeai so that smooth,
whitish scais iesult that aie paitially sui-
iounded by a still active inflammatoiy and
iaised boidei (Fig. 14-24). Buined out" le-
sions may be pink oi white (hypomelanosis)
macules and scais (Fig. 14-26), but scaiied
lesions may also show hypeipigmentation,
especially in peisons with biown oi black skin
(Fig. 14-25).
DIstrIhutIvn und SItes v] PredI|ectIvn CDLE
may be localized oi geneialized, occuiiing pie-
dominantly on the face and scalp; doisa of foie-
aims, hands, fingeis, toes, and less fiequently,
the tiunk (Image 14-4) (Fig. 33-32).
Sca|p Scaiiing alopecia with iesidual inflam-
mation and folliculai plugging (Fig. 14-26; see
Section 32, Figs. 32-15, 32-16).
Mucous Membraoes < 5% of patients have lip
involvement (hypeikeiatosis, hypeimelanotic
scaiiing, eiythema) and atiophic eiythematous
oi whitish aieas with oi without ulceiation on
the buccal mucosa, tongue, and palate. Na|
aaraus Nail dystiophy if nail matiix is in-
volved.
0ermatopatho|oy Hypeikeiatosis, atiophy
of the epideimis, folliculai plugging, lique-
faction degeneiation of the basal cell layei.
Edema, dilatation of small blood vessels,
and peiifolliculai and peiiappendageal lym-
phocytic inflammatoiy infiltiate. Stiong PAS
ieaction of the subepideimal, thickened base-
ment zone.
ImmuooI|uoresceoce LBT positive in 90% of
active lesions at least 6 weeks old and not
iecently tieated with topical glucocoiticoids.
LBT negative in buined-out (scaiied) lesions
and in the noimal skin, both sun-exposed and
nonexposed.
Sero|oy Low incidence of ANA with titeis
>1:16.
hemato|oy Occasionally leukopenia (<4500/
L).
Clinical findings confiimed by histopathology
and immunopathology. The discoid lesions
of CDLE may closely mimic atnt |eraoss .
P|aque sorass and scaling discoid LE without
atiophy and scaiiing may be difficult to dis-
tinguish, especially on the doisa of the hands;
histopathology peimits distinction. Po|ymor-
|ous |g| eruon LE (PMLE) may pose a
pioblem. PMLE does not develop atiophy oi
folliculai plugging, and does not occui in unex-
posed aieas-mouth, haiiy scalp. Lt|en |anus
can be confusing, but the biopsy is distinctive.
Howevei, theie is a lichen planus-LE, syndiome
oveilap of featuies. Luus u|gars and nea
[ata|s.
C0kS AN0 Fk0CN0SIS
Only 1-5% may develop SLE; with localized le-
sions, complete iemission occuis in 50%; with
geneialized lesions, iemissions aie less fiequent
(<10%). Noe agan : CCLE lesions may be the
piesenting cutaneous sign of SLE.
MANACMNI
Freveotoo Topical sunscieens (SPF > 30) iou-
tinely.
Ioca| C|ucocortcods aod Ca|coeuro Iohb-
tors Usually not veiy effective; topical fluoii-
nated glucocoiticoids with caution because of
atiophy. Intialesional tiiamcinolone acetonide,
3-5 mg/mL, foi small lesions.
Aotma|ara|s Hydioxychloioqine, 6.5 mg/kg
body weight pei day. If hydioxychloioquine is
ineffective, add quinaciine, 100 mg thiee times
a day. Monitoi foi oculai side effects.
ketoods Hypeikeiatotic CDLE lesions ie-
spond well to systemic acitietin (0.5 mg/kg
body weight).
Iha|domde 100-300 mg/d is effective. Ob-
seive contiaindications.
FICk 14-25 Chrooc cutaoeous |upus erythematosus: hyperpmeotatoo A |u||+rr+|o|, |e|ou
|eo|.e ||e|e r+, |e |,pe|p|reu|+||ou o| ||e +||op||c +ud p+|||+||, c+||ed |e|ou+| ||u, p+|||cu|+||, |u '|I |||
+ud |\ p+||eu|. A|||ou| ||e ||u |e|ou We|e CC|E, ||e p+||eu| |+d '|E.
FICk 14-26 Chrooc cutaoeous |upus erythematosus |u.o|.ereu| o| ||e c+|p |+ |ed |o corp|e|e
|+|| |o W||| |e|du+| e|,||er+, +||op|,, +ud W|||e c+|||u |u ||| ||+c| r+|e. '|+|p der+|c+||ou o| ||e |e|ou
|u ||e pe||p|e|, |ud|c+|e ||+| ||ee |e|ou o|||u+||, We|e C||E p|+que.
May piecede oi follow the onset of discoid
lesions by seveial yeais. Nodules aie asympto-
matic, tendei, oi sometimes painful.
Sko Iesoos Deep-seated nodules oi plate-
like infiltiations with oi without giossly vis-
ible epideimal changes oi change of coloi;
indolent and fiim, sometimes tendei oi pain-
ful, and aie bettei felt than seen. The oveily-
ing skin may be noimal, eiythematous, oi
biownish oi exhibit typical lesions of CDLE.
Lesions evolve into deep depiessions (Fig. 14-
27) but may also ulceiate; in this case theie is
scaiiing.
DIstrIhutIvn Scalp, face, uppei aims (Fig.
14-27), tiunk (especially the bieasts), thighs,
and buttocks.
Systems kevew Mild SLE may be piesent
(35%).
C||ou|c |upu p+uu|cu|||| | + |o|r o| CC|E |u
W||c| ||e|e +|e |||r, c||curc|||ed u|cu|+ueou
uodu|e.
|e+d |o u|cu|+ueou +||op|, +ud c+|||u |eu||
|u |u uu|eu +|e+.
'u|cu|+ueou uodu|e occu| |o|| W||| +ud W|||
ou| ||E |e|ou o| o.e||,|u ||u.
uu+||, + |o|r o| cu|+ueou |upu, |u| !5 o|
p+||eu| |+.e r||d '|E (ee |r+e +!).
',,. |upu e|,||er+|ou p|o|uudu.
Chk0NIC IFS FANNICIIIIS
0ermatopatho|oy Subcutaneous layei.
Neciobiosis with fibiinoid deposits, dense lym-
phocytic infiltiates, and vasculitis; latei, hyalini-
zation of the fat lobules; fibiosis; theie may be
consideiable mucinous deposits.
0ther In patients with SLE theie aie typical
hematologic and seiologic abnoimalities.
Moiphea, eiythema nodosum, saicoid, miscel-
laneous types of panniculitis.
MANACMNI
Antimalaiials
Thalidomide as foi othei foims of cutnaeous
LE. Bewaie of contiaindications.
Systemic glucocoiticoids
(shoit couise)
FICk 14-2T Iupus paooc-
u|ts C||ou|c p+uu|cu|||| W|||
+||op|, o| ||e u|cu|+ueou ||ue,
|eu|||u |u |+|e uu|eu +|e+ o|
o.e||,|u ||u, |ep|eeu||u |eo|.
|u |e|ou. w|e|e e|,||er+ |
|||| .||||e, p+|p+||ou |e.e+| |||r
u|cu|+ueou uodu|e +ud p|+que.
A|o, ore |e|ou |e.e+| c+|||u |u
||e ceu|e|.
FI0MI0I0C
Freva|eoce 20 pei million of U.S. population.
Sex Female:male iatio, 4:1.
CIASSIFICAII0N
Systemic scleiodeima can be divided into two
subsets: |meJ sysemt st|eroJerma (lSSc) and
J[[use sysemt st|eroJerma (dSSc). lSSc patients
compiise 60%; patients aie usually female;
oldei than those with dSSc; and have a long
histoiy of Raynaud phenomenon with skin
involvement limited to hands, feet, face, and
foieaims (acioscleiosis) and a high incidence
of anticentiomeiic antibodies. lSSc includes the
CREST syndiome, and systemic involvement
may not appeai foi yeais; patients usually die
of othei causes. dSSc patients have a ielatively
iapid onset and diffuse involvement, not only
of hands and feet but also of the tiunk and face,
synovitis, tendosynovitis, and eaily onset of in-
teinal involvement. Anticentiomeie antibodies
aie uncommon, but Scl-70 (antitopoisomeiase
I) antibodies aie piesent in 33%.
Unknown. Piimaiy event might be endothelial
cell injuiy in blood vessels, the cause of which is
unknown. Eaily in couise, taiget oigan edema
occuis, followed by fibiosis; cutaneous capillaiies
aie ieduced in numbei; iemaindei dilate and pio-
lifeiate, becoming visible telangiectasia. Fibiosis
due to oveipioduction of collagen by fibioblasts.
Raynaud phenomenon (see p. 394) with digital
pain, coldness. Pain/stiffness of fingeis, knees.
'c|e|ode|r+ | + uo| o |+|e ru|||,|er d|o|
de| c|+|+c|e||/ed |, |u||+rr+|o|,, .+cu|+|, +ud
c|e|o||c c|+ue o| ||e ||u +ud .+||ou |u|e|u+|
o|+u, epec|+||, ||e |uu, |e+||, +ud C| ||+c|.
||r||ed ,|er|c c|e|ode|r+ (|''c) (o0) +ud
d|||ue ,|er|c c|e|ode|r+ (d''c) +|e |eco
u|/ed.
C||u|c+| |e+|u|e +|W+, p|eeu| +|e ||u c|e|o|
+ud k+,u+ud p|euoreuou.
Cou|de|+||e ro|||d||,, ||| ro||+|||, |o| d''c
',, . ||o|e|.e ,|er|c c|e|o|, ,
|er|c c|e|o|, ,|er|c c|e|ode|r+.
SCIk00kMA |C|9 . 10.
|C|0 . \!+
Migiatoiy polyaithiitis. Heaitbuin, dysphagia,
especially with solid foods. Constipation, di-
aiihea, abdominal bloating, malabsoiption,
weight loss. Exeitional dyspnea, diy cough.
Sko Hunds/Feet Ear|y : Raynaud phenom-
enon with tiiphasic coloi changes, i.e., palloi,
cyanosis, iuboi (Fig. 14-28B, see also Fig. 14-
32). Piecedes scleiosis by months and yeais.
Nonpitting edema of hands/feet. Painful ul-
ceiations at fingeitips (iat bite neciosis") (Fig.
14-29), knuckles; heal with pitted scais. Lae :
scleiodactyly with tapeiing of fingeis (ma-
donna fingeis) (Fig. 14-28) with waxy, shiny,
haidened skin, which is tightly bound down
and does not peimit folding oi wiinkling; leath-
eiy ciepitation ovei joints, flexion contiactuies;
peiiungual telangiectasia, nails giow clawlike
ovei shoitened distal phalanges (Fig. 14-28B).
Bony iesoiption and ulceiation iesults in loss
of distal phalanges.
As scleiosis pioceeds pioximally, theie aie
loss of sweat glands with anhidiosis and thin-
ning and complete loss of haii on distal ex-
tiemities.
Fuce Ear|y : peiioibital edema. Lae : edema
and fibiosis iesult in loss of noimal facial lines,
masklike (patients look youngei than they aie)
(Fig. 14-30), thinning of lips, miciostomia, ia-
dial peiioial fuiiowing (Fig. 14-29B), beak-like
shaip nose. Telangiectasia (Fig. 14-31) and dif-
fuse hypeipigmentation.
Trun| In dSSc the chest and pioximal uppei
and lowei extiemities aie involved eaily. Tense,
stiff, and waxy appeaiing skin that cannot be
folded. Impaiiment of iespiiatoiy movement of
chest wall and of joint mobility.
0ther Chaoes Cutunevus Cu|cI]IcutIvn
Occuis on fingeitips oi ovei bony piominences
oi any scleiodeimatous site; may ulceiate and
exude white paste.
Cv|vr Chunges Hypeipigmentation that may
be geneialized and on the extiemities may be
accompanied by peiifolliculai hypopigmenta-
tion.
Mucvus Memhrunes Scleiosis of sublingual
ligament; uncommonly, painful induiation of
gums, tongue.
DIstrIhutIvn v] LesIvns Ear|y : in lSSc eaily
involvement is seen on fingeis, hands, and face,
and in many patients scleiodeima iemains
confined to these iegions. Lae : the distal uppei
and lowei extiemities may be involved and oc-
casionally the tiunk. In dSSc scleiosis of the ex-
tiemities and the tiunk may stait soon oi soon
aftei oi concomitant with acial involvement.
C|oca| varaot CREST syndiome, i.e.,
t alcinosis cutis - R aynaud phenomenon -
e sophageal dysfunction - s cleiodactyly - el-
angiectasia. Maculai, matlike telangiectasia, es-
pecially the face (Fig. 14-31), uppei tiunk, and
hands; also in the entiie GI tiact. Calcinosis
ovei bony piominences, fingeitips, elbows, and
tiochanteiic iegions.
CNkAI XAMINAII0N
sophaus Dysphagia, diminished peiistalsis,
ieflux esophagitis.
Castrootestoa| System Small intestine in-
volvement may pioduce constipation, diaiihea,
bloating, and malabsoiption.
Iuo Pulmonaiy fibiosis and alveolitis.
Reduction of pulmonaiy function due to ie-
stiicted movement of chest wall.
heart Caidiac conduction defects, heait fail-
uie, peiicaiditis.
kdoey Renal involvement occuis in 45%.
Slowly piogiessive uiemia, malignant hypei-
tension.
Muscu|oske|eta| System Caipal tunnel syn-
diome. Muscle weakness.
0ermatopatho|oy Ear|y : mild cellulai infil-
tiate aiound deimal blood vessels, ecciine coils,
and at the deimal subcutaneous inteiphase.
Lae : bioadening and homogenization of col-
lagen bundles, obliteiation and deciease of
inteibundle spaces, thickening of deimis with
ieplacement of uppei oi total subcutaneous fat
by hyalinized collagen. Paucity of blood vessels,
thickening/hyalinization of vessel walls.
Autoaotbodes Patients with dSSc have cii-
culating autoantibodies by ANA testing. Au-
toantibodies ieact with centiomeie pioteins
oi DNA topoisomeiase I; fewei patients have
antinucleolai antibodies. Anticentiomeiic au-
toantibodies occui in 21% of dSSc and 71% of
CREST patients, DNA topoisomeiase I (Scl-70)
antibodies in 33% of dSSc and 18% of CREST
patients.
FICk 14-28 Sc|eroderma (|SSc): acrosc|eross . n+ud +ud ||ue| +|e eder+|ou (uoup||||u), ||u
| W|||ou| ||u |o|d +ud |ouud doWu. |||+| ||ue| +|e |+pe|ed (r+douu+ ||ue|) 8. ||ue| |oW |o||
||u|| e|,||er+ +ud .+ocou|||c||ou (||ue +ud W|||e). k+,u+ud p|euoreuou. ||ue| +|e eder+|ou, ||e ||u |
|ouud doWu. |||+| p|+|+ue (|ude\ +ud ||||d ||ue|) +|e |o||eued, W||c| | +oc|+|ed W||| |ou, |eo|p||ou.
8
Clinical findings confiimed by deimatopathol-
ogy.
0IIereota| 0aooss D[[use st|eross : mixed
connective tissue disease, eosinophilic fascii-
tis, scleiomyxedema, moiphea, poiphyiia cu-
tanea taida, chionic giaft-veisus-host disease
(GVHD), lichen scleiosus et atiophicus, poly-
vinyl chloiide exposuie, adveise diug ieaction
(pentazocine, bleomycin). Gadolinium and ne-
phiogenic systemic fibiosis (see Section 17).
C0kS AN0 Fk0CN0SIS
Couise chaiacteiized by slow, ielentless pio-
giession of skin and/oi visceial scleiosis; the
10-yeai suivival iate is >50%. Renal disease
is the leading cause of death, followed by
caidiac and pulmonaiy involvement. Spon-
taneous iemissions do occui. lSSc, which
includes the CREST syndiome, piogiesses
moie slowly and has a moie favoiable piog-
nosis; some cases do not develop visceial
involvement.
FICk 14-29 Sc|eroderma (|SSc): acrosc|eross . I,p|c+| '|+| |||e uec|oe +ud u|ce|+||ou o|
||ue|||p. 8. I||uu|u o| ||p-r|c|o|or|+ (W||c| Wou|d |oW |e||e| W|eu p+||eu| +||erp| |o opeu |e|
rou||), |+d|+| pe||o|+| |u||oW|u. Be+||||e |+|p uoe.
8
MANACMNI
Systemic glucocoiticoids may be of benefit
foi limited peiiods eaily in the disease. All
othei systemic tieatments (EDTA, aminocap-
ioic acid, D-penicillamine, ara -aminoben-
zoate, colchicine) have not been shown to be
of lasting benefit. Immunosuppiessive diugs
(cyclospoiine, methotiexate, cyclophospha-
mide, mycophenolate mofetil) have shown
impiovement of skin scoie but only limited
benefit foi systemic involvement. Photophei-
esis: impiovement in one thiid of patients. Im-
munoablation/stem cell tiansplantation and
oial toleiization to type I collagen: ongoing
studies.
FICk 14-30 Sc|eroderma (dSSc) \+||||e |+c|e W||| ||e|c|ed, ||u, ||u +ud |o o| uo|r+| |+c|+| ||ue
|.|u + ,ouue| +ppe+|+uce ||+u +c|u+| +e, ||e |+|| | d,ed. I||uu|u o| ||e ||p +ud pe||o|+| c|e|o| |eu|| |u +
r+|| rou||. 'c|e|o| (W|||||, |||eu|u +|e+) +ud ru|||p|e |e|+u|ec|+e (uo| .||||e +| ||| r+u|||c+||ou) +|e
+|o p|eeu|.
A d''c|||e coud|||ou occu| |u pe|ou e\poed
|o po|,.|u,| c||o||de.
B|eor,c|u +|o p|oduce pu|rou+|, ||||o| +ud
k+,u+ud p|euoreuou |u| uo| ||u c|e|o|.
Cu|+ueou c|+ue |ud|||uu||+||e ||or d''c
|||e c|e|o| o| ||u, +ccorp+u|ed |, r,+||+,
pueurou|||, r,oc+|d|||, ueu|op+||,, +ud eu
cep|+|op+||,, +|e |e|+|ed |o ||e |ue||ou o|
ce||+|u |o| o| | ||,p|op|+u ( -||c,c|c
,!- ),
I|e |: | ,!- ||+| occu||ed |u +u
ep|der|c |u 'p+|u |u 93 +||ec||u 25,000 peop|e
W+ due |o ||e couurp||ou o| deu+|u|ed |+pe
eed o||. A||e| +u +cu|e p|+e, W||| |+|, |e
.e|, pueurou|||, +ud r,+||+, ||e ,ud|ore
p|o|ee |o + coud|||ou W||| ueu|orucu|+|
+|uo|r+||||e +ud c|e|ode|r+|||e ||u |e|ou.
'c|e|or,\eder+ +ud c|e|er+ o| Buc||e +|e
.e|, |+|e, ep+|+|e eu||||e W||| u+|ded p|ouo
|.
|''c|||e c|e|o| +|o occu| |u po|p|,||+ cu|+ue+
|+|d+ (ee 'ec||ou 0) +ud C\n| (ee 'ec||ou
2).
SCIk00kMA-IIk C0N0III0NS
FICk 14-31 Sc|eroderma: CkSI syodrome |ure|ou r+cu|+| o| r+||||e |e|+u|ec|+e ou ||e |o|e
|e+d. Corp|e|e |e+|u|e |uc|ude c+|c|uo| cu||, k+,u+ud p|euoreuou, eop|+e+| d,ro|||||,, c|e|o|, +ud
|e|+u|ec|+|+.
FI0MI0I0C
Ae oI 0oset Young adults oi at menopause.
Sex Female > male.
Iocdeoce As high as 20% in young women.
0ccupatoo May occui in peisons using vibia-
toiy tools (chain saw useis), meat cutteis, typ-
ists, and pianists.
Frecptato Factors Cold, mental stiess,
smoking, ceitain occupations (see above).
FAIh0CNSIS
The vasomotoi tone is iegulated by the sympa-
thetic neivous system. The centeis foi vasomo-
toi tone aie located in the biain, the spinal coid,
and the peiipheial neives. Vasodilatation occuis
only on withdiawal of the sympathetic activity.
It is conjectuied that theie may be a local fault"
in which blood vessels aie abnoimally sensitive
to cold.
Numbness and/oi pain woise in wintei in
tempeiate climates, in the cold (meat cutteis);
pievious tieatment (diugs), occupation (using
vibiatoiy tools) have to be exploied. Caieful
ieview is impoitant to detect diseases in which
RP is associated: aithialgia, fatigue, dysphagia,
muscle weakness, etc.
Iypes oI Sko Chaoes The EpIsvdIc
Attuc| Theie is blanching oi cyanosis of the
fingeis oi toes, extending fiom the tip to vaii-
ous levels of the digits. The fingei distal to the
line of ischemia is white oi blue and cold (Fig.
14-32); the pioximal skin is pink and waim.
When the digits aie iewaimed, the blanching
may be ieplaced by cyanosis because of slow
k+,u+ud p|euoreuou (k|) | d|||+| |c|er|+
||+| occu| ou e\pou|e |o co|d +ud/o| + + |e
u|| o| ero||ou+| ||e.
|||r+|, k| | + coud|||ou W|e|e uo e||o|o, |
|ouud, ecoud+|, k| | ||e de|u+||ou |o| k| +ud
uude||,|u d|e+e.
I|e .+||ou c+ue o| ecoud+|, k| +|e |||ed
|u I+||e +1. ||-c|: !!- ,|er|c
c|e|ode|r+ (35), '|E (!5), de|r+|or,o|||
(!0), 'jo|eu ,ud|ore, ||eur+|o|d +|||||||,
po|,+||e|||| uodo+|, !-c- +|| c|c|
||! |- (c|,op|o|e|u, co|d +|u||u|u,
r+c|o|o|u||u), d|u ( +d|eue||c ||oc|e|,
u|co||ue), +ud c|-c| !-c- (+||e||oc|e|o|
o||||e|+u, |||or|o+u|||| o||||e|+u) +|e ||e
ro| corrou.
kANA0 FhN0MN0N |C|9 . ++!.0
|C|0 . 1!.0
blood flow; at the end of the attack, the noimal
coloi oi a ied coloi ieflects the ieactive hypei-
emic phase. To iecapitulate, the sequence of
coloi changes is white blue ied (see Fig.
14-28B). Raiely, the tip of the nose, eailobes,
oi the tongue may be involved. Blanching may
occui in one oi two digits oi in all the digits;
often the thumb is spaied. The feet aie involved
in only 40%.
Repeuted vr PersIstent Vuscu|ur Vusvspusm
Patients with RP often have a peisistent vaso-
spasm iathei than episodic attacks. Skin changes
include tiophic changes with development of
taut, atiophic skin, pteiygium, clubbing and
shoitening of the teiminal phalanges, scleio-
dactyly-like in lSSc (see Fig. 14-28). Aciogan-
giene is iaie in RD (< 1%), but in RP associated
with scleiodeima, painful ulceis. Sequestiation
of the teiminal phalanges oi the development
of gangiene (Fig. 14-33) may lead to autoam-
putation of the fingeitips.
Sero|oy ANA should be deteimined.
0IACN0SIS
Diagnosis is based on the vasculai changes that
aie chaiacteiistic; ANA and othei tests to iule
out scleiodeima and othei conditions (Table
14-7). When no othei disease is discoveied, the
diagnosis is piimaiy RP.
Fk0CN0SIS
RP may disappeai spontaneously; it piogiesses
in about a thiid of patients.
FICk 14-32 kayoaud pheoomeooo I|e |e|| |+ud e\||||| + d||+| c,+uo| corp+|ed |o ||e |||| |+ud,
|| | eeu epec|+||, We|| |u ||e u+|||ed. uu||+|e|+| ep|ode uc| + ||| oue r+, occu| +||e| cou|+c| W||| + co|d
o|jec|.
FICk 14-33 kayoaud pheoomeooo:
acroaoreoe |e|||eu| .+op+r o|
red|ur|/ed +||e||o|e c+u ore||re |e+d
|o +u|eue o| ||e |e|r|u+| d||| + |||u
||+|ed |u ||| p+||eu| W||| c|e|ode|r+.
MANACMNI
Freveotoo Education iegaiding the use of
loose-fitting clothing and avoiding cold and
piessuie on the fingeis. Giving up smoking is
mandatoiy.
IA8I 14-T Causes or 0isorders Associated with Secondary Raynaud Phenomenon
Couuec||.e ||ue d|e+e
'c|e|ode|r+
',|er|c |upu e|,||er+|ou
|e|r+|or,o||| +ud po|,r,o|||
uud|||e|eu||+|ed couuec||.e ||ue d|e+e
',|er|c .+cu||||
'jo|eu ,ud|ore
Eo|uop||||c |+c||||
0|||uc||.e +||e||+| d|e+e
A||e|oc|e|o|
I||or|o+u|||| o||||e|+u (Bue|e| d|e+e)
I||or|oer|o||r
I|o|+c|c ou||e| ,ud|ore
||u +ud |o\|u
Ad|eue||c ||oc|e|
E|o|+r|ue
0|+| cou||+cep||.e
\e||,e||de
B|eor,c|u +ud .|u||+||ue
C|ou|d|ue
B|oroc||p||ue
C,c|opo||ue
Arp|e|+r|ue
||uo\e||ue
|u|e||e|ou
'0ukCE. ln K|ppe|. k+,u+ud p|euoreuou, |u, K wo||| e| +| (ed). ||cc|:| |-c||, C--c| !-!:- 1|| ed. |eW \o||, \cC|+Wn|||,
p o+o, 2003.
|eu|o|o|c d|o|de|
C+|p+| |uuue| ,ud|ore
ke||e\ ,rp+||e||c d,||op|,
ner|p|e|+
|o||or,e||||
\u|||p|e c|e|o|
',||uor,e||+
0ccup+||ou/eu.||oureu|+| e\pou|e
\|||+||ou |uju|, (|ur|e|j+c|, pueur+||c |+rre|
ope|+|o|)
|o|||+ur+||c |uju|, (|,po||eu+| |+rre|
,ud|ore, c|u|c| p|eu|e)
\|u,| c||o||de d|e+e
Co|d |uju|,
n,pe|.|co||, d|o|de|
C|,op|o|e|u
Co|d +|u||u|u
\+c|o|o|u||u
|o|,c,||er|+
I||or|oc,|o|
\|ce||+ueou
n,po||,|o|d|r
|u|ec||ou (|+c|e||+| eudoc+|d|||, |,re d|e+e,
.||+| |ep+||||)
|eop|+r
|||r+|, pu|rou+|, |,pe||eu|ou
A||e||o.euou |||u|+
|u||++||e||+| |ujec||ou
Iherapy Diug theiapy should be used in pa-
tients who have seveie and piogiessive RP. Cal-
cium channel blockeis, anti-adieneigic diugs;
intiavenous piostacyclin (PGI
2
); statins may
benefit RP and digital ulceiations.
Ae oI 0oset All ages.
to|oy See Table 14-8; idiopathic 50%.
FAIh0CNSIS
A postulated mechanism foi neciotizing vascu-
litis is the deposition in postcapillaiy venules of
ciiculating immune complexes. Initial alteia-
tions in venulai peimeability, due to the ielease
of vasoactive amines fiom platelets, basophils,
and/oi mast cells, facilitate the deposition of
immune complexes and these may activate
the complement system oi may inteiact di-
iectly with Fc ieceptois on endothelial cell
membianes. When the complement system is
activated, the geneiation of anaphylatoxins C3a
and C5a can degianulate mast cells. Also, C5a
can attiact neutiophils that ielease lysosomal
enzymes duiing phagocytosis of complexes and
subsequently damage vasculai tissue.
A new diug taken duiing the few weeks befoie
the onset of HV is a likely etiologic agent,
as may be an infection, a known vasculai/
connective tissue disease, oi paiapioteinemia
(Table 14-8). Onset and couise: acute (days,
as in diug-induced oi idiopathic), subacute
(weeks, especially uiticaiial types), chionic
(iecuiient ovei yeais). Symptoms aie piuii-
tus, buining pain; theie may be no symptoms
vASCIIIIS |C|9 . ++o.20
|C|0 . \!.0
n,pe|eu|||.||, .+cu|||| (n\) eucorp+e +
|e|e|oeueou |oup o| .+cu||||de +oc|+|ed
W||| |,pe|eu|||.||, |o +u||eu ||or |u|ec||ou
+eu|, d|u, o| o||e| e\oeuou o| eudoeuou
ou|ce (I+||e +3).
|| | c|+|+c|e||/ed p+||o|o|c+||, |, |u.o|.ereu|
o| po|c+p|||+|, .euu|e +ud |u||+rr+||ou +ud
|||||uo|d uec|o| (uec|o||/|u .+cu||||).
C||u|c+||,, ||u |u.o|.ereu| | c|+|+c|e||||c, r+u|
|e|ed |, 'p+|p+||e pu|pu|+.
',|er|c .+cu|+| |u.o|.ereu| occu|, c||e||, |u
||e ||due,, ruc|e, jo|u|, C| ||+c|, +ud pe||p|
e|+| ue|.e.
'c|ou|e|uneuoc| pu|pu|+ | + |,pe o| n\ +oc|
+|ed W||| |A depo|| |u ||u.
',, . A||e||c cu|+ueou .+cu||||, uec|o||/
|u .+cu||||.
hFkSNSIIIvII vASCIIIIS
oi theie may be fevei, malaise; symptoms of
peiipheial neuiitis, abdominal pain (bowel
ischemia), aithialgia, myalgia, kidney involve-
ment (miciohematuiia), CNS involvement.
Sko Iesoos The hallmaik is a|a||e urura .
This teim desciibes palpable petechiae that
piesent as biight ied, well-demaicated macules
and papules with a cential, dotlike hemoi-
ihage (Fig. 14-34) (petechiae due to coagula-
tion defects oi thiombocytopenia aie stiictly
maculai and, theiefoie, not palpable). Lesions
aie scatteied, disciete oi confluent, and aie pii-
maiily localized to the lowei legs and the ankles
(Fig. 14-34 and B) but may spiead to the but-
tocks and aims. Stasis aggiavates oi piecipitates
lesions. Puipuiic lesions do not blanch (with a
glass slide). Red initially, they tuin puiple and
even black in the centei (Fig. 14-34B). In the
case of massive inflammation, puipuiic papules
conveit to hemoiihagic blisteis, become neciotic
(Fig. 14-34B), and even ulceiate.
hemato|oy Rule out thiombocytopenic pui-
puia.
Sk Elevated.
Sero|oy Seium complement is ieduced oi
noimal in some patients, depending on associ-
ated disoideis.
roa|yss RBC casts, albuminuiia.
0thers Depending on undeilying disease.
0ermatopatho|oy Netro:ng astu|s . Dep-
osition of eosinophilic mateiial (fibiinoid) in
the walls of postcapillaiy venules in the uppei
deimis, and peiivenulai and intiamuial inflam-
matoiy infiltiate consisting piedominantly of
neutiophils. Extiavasated RBC and fiagmented
neutiophils (nucleai dust"). Fiank neciosis of
vessel walls. Intiamuial C3 and immunoglobu-
lin deposition is seen with immunofluoiescent
techniques.
Based on clinical appeaiance and histopathology.
0IIereota| 0aooss Thiombocytopenic
puipuia, iash such as exanthematous diug
eiuption in setting of thiombocytopenia, dis-
seminated intiavasculai coagulation (DIC) with
puipuia fulminans, septic vasculitis (iickettsial
spotted feveis), septic emboli (infective endo-
caiditis), bacteiemia disseminated gonococcal
infection, meningococcemia (acute/chionic)],
pigmented puipuia, othei noninfectious vas-
culitides.
C0kS AN0 Fk0CN0SIS
Depends on undeilying disease. In the idi-
opathic vaiiant, multiple episodes can occui
ovei the couise of yeais. Usually self-limited,
but iiieveisible damage to kidneys can occui.
MANACMNI
Aotbotcs Antibiotics foi patients in whom
vasculitis follows bacteiial infection.
Fredosooe Foi patients with modeiate to
seveie disease.
Cytotoxc Immuoosuppressves Cyclophos-
phamide, azathiopiine usually in combination
with piednisone. Cyclospoiine, intiavenous
high-dose immunoglobulin.
IA8I 14-8 C|assification of hypersensitivity Vascu|itis
Aoc|+|ed W||| |u|ec||ou
nep+|||| B .||u
nep+|||| C .||u
C|oup A |ero|,||c ||ep|ococcu ('c|ou|e|uneuoc|
pu|pu|+)
'|c|,|::: c-
!,:|c:|- |-c- (|,pe 2 |e+c||ou, e|,||er+
uodour |ep|our)
0||e|
Aoc|+|ed W||| d|u
'u||ou+r|de
|eu|c||||u
'e|ur
0||e|
Aoc|+|ed W||| ueop|+r
|,rp|op|o|||e|+||.e d|e+e
C+|c|uor+ o| ||due,
Aoc|+|ed W||| +u|o|rruue couuec||.e ||ue
d|e+e
'|E
k|eur+|o|d +|||||||
'jo|eu ,ud|ore
Aoc|+|ed W||| d,p|o|e|uer|+
C|,o|o|u||uer|+
|+|+p|o|e|uer|+
n,pe|+rr+|o|u||uer|+
Coueu||+| de||c|euc|e o| corp|ereu|
|d|op+|||c
I|| | + pec|||c u||,pe o| |,pe|eu|||.||, .+cu
|||| ||+| occu| r+|u|, |u c|||d|eu |u| +|o +||ec|
+du||.
I|e|e | + |||o|, o| uppe| |ep||+|o|, ||+c| |u|ec
||ou (15), |, |oup A ||ep|ococc|.
I|e d|o|de| cou|| o| p+|p+||e pu|pu|+ (||.
+!5) +ccorp+u|ed |, |oWe| +u|u+ (d|||ue
+|dor|u+| p+|u ||+| | Wo|e +||e| re+|), |oWe|
|c|er|+, uu+||, |uc|ud|u ||ood, d|+|||e+, ||d
ue, |u.o|.ereu| (|er+|u||+ +ud |ed ce|| c+|),
+ud +|||||||.
n||op+||o|o|c+||,, ||e|e | uec|o||/|u .+cu||||
+ud ||e |rruuo|e+c|+u| depo||ed |u ||u +|e
|A.
|ou|e|r ro|||d||, r+, |eu|| ||or p|o|e|.e
|eu+| d|e+e (5).
SCh0NIIN-hN0Ch FkFkA |C|9 . 231.0
|C|0 . o9.0
FICk 14-34 hyperseostvty vascu|ts . Cu|+ueou .+cu|||| p|eeu| c||u|c+||, + 'p+|p+||e pu|pu|+
ou ||e |oWe| e\||er|||e. A|||ou| +ppe+||u |o ||e e,e + r+cu|e, ||e |e|ou c+u |e p+|p+|ed, +ud ||| cou
||+| W||| pe|ec||+e, |o| |u|+uce, |u |||or|oc,|opeu|c pu|pu|+. I|e |e|ou |oWu |e|e |+.e ceu||+| puuc|ur
||+| | + d+||e| |ed +ud do uo| ||+uc| W||| + |+ ||de, |ud|c+||u |ero|||+e. 8. I|| | + ro|e +d.+uced |+e.
|e|ou |+.e p|o|eed |o |ero|||+|c |u||+e +ud ore |+.e |ecore uec|o||c. I|e |e|ou r+, p|o|e |o
u|ce|+||ou.
8
Ae oI 0oset Mean age 45 yeais.
Sex Male:female iatio 2.5:1.
to|oy Unknown.
C|oca| varaots Cuaneous PN is a iaie vaii-
ant with symptomatic vasculitis limited to skin
and at times peiipheial neives.
|o|,+||e|||| uodo+ (|A|) | + ru|||,|er,
uec|o||/|u .+cu|||| o| r+|| +ud red|ur|/ed
rucu|+| +||e||e W||| |u.o|.ereu| o| ||e |eu+|
+ud .|ce|+| +||e||e.
\|c|ocop|c po|,+u||| (\|A) r+, |e d|||eeu|
||or |A| |u| ||| | uo| p|o.eu +ud ||e|e|o|e
|uc|uded |u ||| d|cu|ou.
Cou|||u||ou+| ,rp|or. |e.e|, +||r+, r,+||+.
'||u r+u||e|+||ou +|e p+|p+||e pu|pu|+, uodu|e
+ud u|ce|.
',, . |e||+||e|||| uodo+, p+u+||e|||| uo
do+.
F0IAkIkIIIS N000SA |C|9 . ++o.0
|C|0 . \!0.0
FAIh0CNSIS
Neciotizing inflammation of small- and
medium-sized musculai aiteiies; may spiead cii-
cumfeientially to involve adjacent veins. Lesions
segmental, tend to involve bifuications. About
30% of cases associated with hepatitis B and C
antigenemia, i.e., immune complex foimation.
FICk 14-35 hyperseostv-
ty vascu|ts: Scho|eo-heooch
purpura I|e|e | c|+|c p+|
p+||e pu|pu|+ ou ||e |oWe| |e o|
+ 9,e+|o|d r+|e. I|e p+||eu| |+d
co||c|, +|dor|u+| p+|u, +|||||||,
+ud r|c|o|er+|u||+ +ud ||u ||op,
|e.e+|ed |A |rruuo|e+c||.||, +|ouud
po|c+p|||+|, .euu|e.
Chrooc 0sease Syodrome With inteinal oi-
gan involvement and associated symptoms (see
below).
Cutaoeous FAN Pain in nodules, ulceis;
aching duiing flaies and physical activity.
Myalgia. Neuialgia, numbness, mild pai-
esthesia.
Sko Iesoos Occui in 15% of cases. Sub-
cutaneous inflammatoiy, biight ied to bluish
nodules (0.5-2 cm) that follow the couise of
involved aiteiies. Violaceous, become conflu-
ent to foim painful subcutaneous plaques (Fig.
14-36), and accompanied by livedo ieticula-
iis; staibuist" livedo is pathognomonic and
maiks a clustei of nodulai lesions. Ulceis fol-
low ischemia of nodules (Fig. 14-36B). Usually
bilateially on lowei legs, thighs. Othei aieas:
aims, tiunk, head, neck, buttocks. Livedo ie-
ticulaiis may extend to tiunk. Duiation-days
to months. Resolves with iesidual violaceous
oi postinflammatoiy hypeipigmentation. Skin
lesions in systemic and cutaneous PAN aie
identical.
CNkAI XAMINAII0N
Cardovascu|ar Hypeitension; congestive heait
failuie, peiicaiditits, conduction system defects,
myocaidial infaiction.
Neuro|oc Ceiebiovasculai accident. Peiiph-
eial neives: mixed motoi/sensoiy involvement
with mononeuiitis multiplex pattein.
Musc|es Diffuse myalgias (excluding shouldei
and hip giidle), lowei extiemities.
CI System Nausea, vomiting, abdominal pain,
hemoiihage, infaiction.
yes Hypeitensive changes, oculai vasculitis, iet-
inal aiteiy aneuiysm, optic disc edema/atiophy.
kdoey Renal failuie, edema.
Iestes Pain and tendeiness.
0ermatopatho|oy Bes ye|J. |osy o[ noJu|ar
s|n |eson (deep wedge biopsy). Polymoipho-
nucleai neutiophils infiltiate all layeis of mus-
culai vessel wall and peiivasculai aieas; latei,
mononucleai cells. Fibiinoid neciosis of vessel
wall with compiomise of lumen, thiombosis,
FICk 14-36 Fo|yarterts oodosa . IWo de|r+| +ud u|cu|+ueou uodu|e occu|||u ou ||e p|e||||+|
+pec| o| ||e |oWe| |e. 8. A |+||u|| p+||e|u c+u |e eeu |u ||e up|+ +ud |e||or+||eo|+| |e|ou o| ||e |||| |e
|u +uo||e| p+||eu|. I|ee |e|ou |ep|eeu| cu|+ueou |u|+|c||ou W||| u|ce|+||ou.
8
infaiction of tissues supplied by involved vessel,
with oi without hemoiihage. Skin pathology is
identical in systemic and cutaneous PAN.
C8C Commonly neutiophilic leukocytosis;
iaiely, eosinophilia; anemia of chionic disease.
Elevated ESR.
Sero|oy Antineutiophil cytoplasmic autoan-
tibodies (p-ANCA) in some cases. In 60% of
MPA patients, hepatitis B suiface antigenemia;
in 30% of cases, hepatitis C.
Chemstry Elevated cieatinine, BUN.
Arteroraphy Aneuiysms in small- and me-
dium-sized musculai aiteiies of kidney/hepatic/
visceial vasculatuie.
Othei vasculitides and panniculitides.
C0kS AN0 Fk0CN0SIS
Untieated, veiy high moibidity and moitality
iates chaiacteiized by fulminant deteiioiation oi
by ielentless piogiession associated with inteimit-
tent acute exaceibations. Death fiom ienal failuie,
bowel infaiction and peifoiation, caidiovasculai
complications, intiactable hypeitension. Cuane-
ous PN : chionic ielapsing benign couise.
MANACMNI
Systemc FAN Com|neJ |eray : piednisone,
1 mg/kg body weight pei day, and cyclophos-
phamide, 2 mg/kg pei day.
Cutaoeous FAN Nonsteioidal anti-inflamma-
toiy agents, piednisone. In seveie cases, as foi
systemic PAN.
weeue| |+uu|or+|o| (wC) | + ,|er|c .+
cu||||, de||ued |, + c||u|c+| |||+d o| r+u||e|+||ou
corp|||u |u.o|.ereu| o| ||e uppe| +||W+,,
|uu, +ud ||due,
'||u r+u||e|+||ou +|e ||oe o| |,pe|eu|||.||,
.+cu||||, uodu|ou|ce|+||.e |e|ou +ud o|+|/u++|
u|ce|+||ou.
A p+||o|o|c |||+d cou|||u o| uec|o||/|u |+uu
|or+ |u ||e uppe| |ep||+|o|, ||+c| +ud |uu,
.+cu|||| |u.o|.|u |o|| +||e||e +ud .e|u, +ud
|ore|u||||.
WCNk CkANI0MAI0SIS |C|9 . ++o.+
|C|0 . \!.!
Ae oI 0oset Mean age 40 yeais, but occuis
at any age.
Sex Male:female iatio 1.3:1.
kace Raie in blacks.
to|oy Unknown.
C|oca| varaots One vaiiant is limited to kid-
neys, i.e., glomeiulitis occuis in 15% of cases;
anothei is limited to iespiiatoiy tiact.
FAIh0CNSIS
Immunopathogenesis uncleai. Possibly an
abeiiant hypeisensitivity iesponse to an ex-
ogenous oi endogenous antigen that enteis
thiough oi iesides in uppei aiiways. Clinical
symptomatology caused by neciotizing vas-
culitis of small aiteiies and veins. Pulmonaiy
involvement: multiple, bilateial, nodulai infil-
tiates. Similai infiltiates in paianasal sinuses,
nasophaiynx.
Chionic disease syndiome. Fevei. Paianasal
sinus pain, puiulent oi bloody nasal dischaige.
Cough, hemoptysis, dyspnea, chest discomfoit.
FhSICAI XAMINAII0N
Sko Iesoos Oveiall in 50% of patients but
in only 13% of patients at initial piesentation.
U|ters w| ,aggeJ, unJermneJ |orJers most
typical; iesemble pyodeima gangienosum (Fig.
14-37). Pau|es, est|es, a|a||e urura as in
hypeisensitivity (neciotizing) vasculitis (Fig.
14-38), subcutaneous nodules, plaques, nodu-
loulceiative lesions as in PAN. Most common
on lowei extiemities. Also, face, tiunk, uppei
limbs.
Mucvus Memhrunes Oial ulceiations (Fig.
14-39). Often fiist symptom. Nasal mucosal
ulceiation, ciusting, blood clots; nasal septal
peifoiation; saddle-nose defoimity. Eustachian
FICk 14-3T Weeoer raou|omatoss A p,ode|r+ +u|euour|||e |||eu|+| u|ce|+||ou ou ||e c|ee|
W||| j+ed +ud uude|r|ued |o|de| | o||eu ||e |||| r+u||e|+||ou o| weeue| |+ru|or+|o|.
FICk 14-38 Weeoer raou|omatoss |+|p+||e pu|pu|+ W||| |ero|||+|c +ud uec|o||c |e|ou ou ||e
|e + |u |,pe|eu|||.||, .+cu||||.
tube occlusion with seious otitis media; pain.
Exteinal auditoiy canal: pain, eiythema, swell-
ing. Maiked gingival hypeiplasia.
Eyes 65%. Mild conjunctivitis, episcleiitis,
scleiitis, gianulomatous scleiouveitis, ciliaiy
vessel vasculitis, ietiooibital mass lesion with
pioptosis.
Nervvus System Cianial neuiitis, mononeuii-
tis multiplex, ceiebial vasculitis.
Renu| DIseuse 85%. Signs of ienal failuie in
advanced WG.
Cutaoeous Necross + kespratory Iract 0sease
Othei vasculitides, Goodpastuie syndiome, tu-
mois of the uppei aiiway/lung, infectious/non-
infectious gianulomatous diseases (especially
blastomycosis), midline gianuloma, angiocen-
tiic lymphoma, alleigic gianulomatosis.
hemato|oy Mild anemia. Leukocytosis.
Thiombocytosis.
Sk Maikedly elevated.
Chemstry Impaiied ienal function.
roa|yss Pioteinuiia, hematuiia, RBC casts.
Sero|oy Antineutiophil cytoplasmic autoan-
tibodies (ANCA) aie seiomaikeis foi WG. Two
ANCA patteins occui in ethanol-fixed neu-
tiophils: cytoplasmic pattein (c-ANCA) and
peiinucleai pattein (p-ANCA). A 29-kDa pio-
tease (PR-3) is the majoi antigen foi c-ANCA;
myelopeioxidase, foi p-ANCA. c-ANCA has
been associated piedominantly with WG and is
consideied specific foi this condition; p-ANCA
with micioscopic polyaiteiitis, PAN, othei vas-
culitides, idiopathic neciotizing and ciescentic
glomeiulonephiitis. Titeis coiielate with dis-
ease activity. Hypeigammaglobulinemia, pai-
ticulaily IgA class.
Fatho|oy All involved tissues including skin:
neciotizing vasculitis of small aiteiies/veins
with intia- oi extiavasculai gianuloma foima-
tion. Kidneys: focal/segmental glomeiulone-
phiitis.
Imao Paranasa| snuses : opacification, with
oi without scleiosis. C|es : pulmonaiy infil-
tiates, nodules; consolidation, cavitation; uppei
lobes.
C0kS AN0 Fk0CN0SIS
Untieated, usually fatal because of iapidly
piogiessive ienal failuie. With combination
cyclophosphamide plus piednisone theiapy,
long-teim iemission is achieved in 90% of
cases.
MANACMNI
Ireatmeot oI Choce Cyclophosphamide plus
piednisone.
Cyc|vphvsphumIde 2 mg/kg body weight pei
day. Dose should be adjusted to keep leukocyte
count > 5000/L (neutiophil count 1500/L) to
avoid infections associated with neutiopenia.
Theiapy should be continued foi 1 yeai aftei
complete iemission, then tapeied and discon-
tinued. |ernae Jrug : azathiopiine in similai
doses if cyclophosphamide is not toleiated.
PrednIsvne 1 mg/kg body weight pei day foi
1 month, and then changed to alteinate-day
doses which aie tapeied and then discontinued
aftei 6 months of theiapy.
RIturImuh In iefiactoiy patients.
TrImethvprIm-Su|]umethvruzv|e As adjunc-
tive theiapy and/oi pievention of uppei aiiway
bacteiial infections that piomote disease flaie.
Ae oI 0oset Middle-age to eldeily, usually
> 55 yeais.
to|oy Unknown. Piobably via cell-medi-
ated immunity.
Fatigue. Fevei. Chionic disease syndiome.
Headache usually bilateial. Scalp pain.
C|+u| ce|| +||e|||| | + ,|er|c |+uu|or+|ou
.+cu|||| o| red|ur +ud |+|e|/ed +||e||e,
ro| uo|+||, ||e |erpo|+| +||e|, +ud o||e|
||+uc|e o| ||e c+|o||d +||e|, |u e|de||, p+||eu|.
C|+|+c|e||/ed |, |e+d+c|e, |+||ue, |e.e|, +ue
r|+, +ud ||| E'k.
Cu|+ueou r+u||e|+||ou +|e uec|o| +ud u|
ce|+||ou ou ||e c+|p.
',, . Ierpo|+| +||e||||, c|+u|+| +||e||||.
CIANI CII AkIkIIIS |C|9 . ++o.5
|C|0 . !.5
Claudication of jaw/tongue while talking/
chewing. Eye involvement: tiansient impaii-
ment of vision, ischemic optic neuiitis, ietio-
bulbai neuiitis, peisistent blindness. Systemic
vasculitis: claudication of extiemities, stioke,
myocaidial infaiction, aoitic aneuiysms/dis-
sections, visceial oigan infaiction. Po|ymya|ga
r|eumata synJrome : stiffness, aching, pain in
the muscles of the neck, shouldeis, lowei back,
hips, thighs.
FICk 14-39 Weeoer raou|o-
matoss A |+|e u|ce| ou ||e p+|+|e
co.e|ed |, + deue, +d|e|eu|, uec|o||c
r+, uo|e +ccorp+u,|u eder+ o|
||e uppe| ||p. '|r||+| |e|ou occu| |u
||e |uue +ud ||+c|eo||ouc||+| ||ee.
FAIh0CNSIS
Systemic vasculitis of multiple medium- and
laige-sized aiteiies. Symptoms secondaiy to
ischemia.
Sko Iesoos Supeificial tempoial aiteiies
aie swollen, piominent, toituous, nodulai
thickenings. Tendei. Initially, involved aiteiy
pulsates; latei, occluded with loss of pulsation.
Eiythema of oveilying skin. Gangiene, i.e.,
skin infaiction of the aiea supplied by affected
aiteiy in the tempoial/paiietal scalp with shaip,
iiiegulai boideis (Fig. 14-40); ulceiation with
exposuie of bone (Fig. 14-40B). Scais at sites
of old ulceiations. Postinflammatoiy hypeipig-
mentation ovei involved aiteiy.
Ceoera| xamoatoo Findings in othei oigan
systems ielated to tissue ischemia/infaiction.
C8C Noimochiomic/slightly hypochiomic
anemia.
Sk Maikedly elevated.
Iempora| Artery 8opsy Biopsy tendei nodule
of involved aiteiy with oi without oveilying
affected skin aftei Dopplei flow examination.
Lesions focal. Panaiteiitis with inflammatoiy
mononucleai cell infiltiates within the ves-
sel wall with fiequent giant cell gianuloma
foimation. Intimal piolifeiation with vasculai
occlusion, fiagmentation of inteinal elastic
lamina, extensive neciosis of intima and me-
dia.
C0kS AN0 Fk0CN0SIS
Untieated, can iesult in blindness secondaiy to
ischemic optic neuiitis. Excellent iesponse to glu-
cocoiticoid theiapy. Remission aftei seveial yeais.
MANACMNI
Fredosooe Fiist-line theiapy. Initially, 40-60
mg/d; tapei when symptoms abate; continue
7.5-10 mg/d foi 1-2 yeais.
Methotrexate Low-dose (15-20 mg) metho-
tiexate, once a week, has a consideiable gluco-
coiticoid-spaiing effect.
FICk 14-40 Caot ce|| arterts . I|| e|de||, r+|e |+d e\c|uc|+||u |e+d+c|e +ud p|o|e|.e
|rp+||reu| o| .||ou. |ec|o| de.e|oped |||+|e|+||, ou ||e c+|p. 8. |u ||| p+||eu| ||e uec|o||c ||ue |+ |eeu
|ed, |e.e+||u ||e |+|e |oue o| ||e |u||.
8
Ae oI 0oset Majoiity 30-50 yeais.
Sex Female:male iatio 3:1.
to|oy In patients with seium sickness; in
collagen vasculai diseases, in paiticulai, LE;
with ceitain infections (e.g., hepatitis B); and
idiopathic.
Iocdeoce <5% of patients with uiticaiia.
FAIh0CNSIS
Thought to be an immune complex disease, sim-
ilai to hypeisensitivity vasculitis (see page 397).
Lesions may be associated with itching, buin-
ing, stinging sensation, pain, tendeiness. Fevei
u|||c+||+| .+cu|||| | + ru|||,|er d|e+e c|+|
+c|e||/ed |, cu|+ueou |e|ou |eer|||u u|||
c+||+, e\cep| ||+| W|e+| pe||| |o| >2+ |.
|e.e|, +||||+||+, e|e.+|ed E'k, +ud |||o|o|c
||ud|u o| + |eu|oc,|oc|+||c .+cu|||| +|e +|o
p|eeu|.
I|e ,ud|ore | o||eu +ccorp+u|ed |, .+||ou
de|ee o| e\||+cu|+ueou |u.o|.ereu|.
\+, |e cu|+ueou r+u||e|+||ou o| '|E.
',, . u|||c+||+ pe||+u.
kIICAkIAI vASCIIIIS
(10-15%). Aithialgias with oi without aithiitis
in one oi moie joints (ankles, knees, elbows,
wiists, small joints of fingeis). Nausea, abdomi-
nal pain. Cough, dyspnea, chest pain, hemopty-
sis. Pseudotumoi ceiebii. Cold sensitivity. Renal
involvement: diffuse glomeiulonephiitis.
Sko Iesoos Uiticaiia-like (i.e., edematous
plaques and wheals), occasionally induiated,
eiythematous, ciicumsciibed (Fig. 14-41); oc-
casionally with angioedema. Eiuption occuis in
tiansient ciops, usually lasting >24 h and up to
3-4 days. They change shape slowly, often ieveal
puipuia on blanching (glass slide), and iesolve
with a yellowish-gieen coloi and hypeipigmen-
tation.
Ceoera| xamatoo Extiacutaneous mani-
festations: joints (70%), GI tiact (20-30%),
CNS (>10%), oculai system (>10%), kidneys
(10-20%), lymphadenopathy (5%).
FICk 14-41 rtcara| vascu|ts E|,||er+|ou p|+que +ud W|e+| ou ||e |u||oc| ||+|, |u p+||, do uo|
||+uc| ou d|+cop, (corp|e|ou o| ||e |e|ou+| ||u W||| |+), W||c| |ud|c+|e |ero|||+e. I|| cou||+| W|||
u|||c+||+. A|o, |u cou||+| |o |e|ou o| u|||c+||+, W||c| uu+||, |eo|.e W||||u 2+ |, ||oe o| u|||c+||+| .+cu||||
pe||| |o| up |o ! d+, |e|o|e |eo|.|u W||| |e|du+| |,pe|p|reu|+||ou (|ero|de||u depo|||ou). |e|ou o|
u|||c+||+ c|+ue |+pe |u + |o|| ||re, W|||e ||oe o| u|||c+||+| .+cu|||| c|+ue |oW|,.
0ermatopatho|oy Inflammation of deimal
venules piimaiily with neutiophils w|ou nec-
iotizing vasculitis. Latei, fiank leukocytoclastic
vasculitis.
roa|yss 10% of patients-miciohematuiia,
pioteinuiia.
Sk Elevated.
Sero|oc Fodos Hypocomplementemia
(70%); ciiculating immune complexes.
Clinical suspicion confiimed by skin biopsy.
Uiticaiia, seium sickness, othei vasculitides,
SLE, uiticaiia in acute hepatitis B infection.
0sease Assocatoos SLE and othei collagen
vasculai autoimmune disease.
C0kS AN0 Fk0CN0SIS
Most often this syndiome has a chionic (months
to yeais) but benign couise. Episodes iecui ovei
peiiods ianging fiom months to yeais. Renal
disease occuis only in hypocomplementemic
patients.
MANACMNI
Rule out vasculai/connective tissue disease.
Frst Ioe H
1
and H
2
blockeis doxepin (10 mg
twice daily to 25 mg thiee times daily) |us ci-
metidine (300 mg thiee times daily)/ianitidine
(150 mg twice daily)] |us a nonsteioidal anti-
inflammatoiy agent indomethacin (75-200
mg/d)/ibupiofen (1600-2400 mg/d)/napiosyn
(500-1000 mg/d)].
Secood Ioe Colchicine, 0.6 mg two oi thiee
times daily or dapsone, 50-150 mg/d.
Ihrd Ioe Piednisone.
Fourth Ioe Cytotoxic immunosuppiessive
agents (azathiopiine, cyclophosphamide).
|odu|+| .+cu|||| | + |o|r o| |o|u|+| p+uu|cu
|||| +oc|+|ed W||| u|cu|+ueou ||ood .ee|
.+cu|||| W||| u|equeu| |c|er|c c|+ue ||+|
p|oduce ||poc,|e |uju|,, uec|o|, |u||+rr+||ou,
+ud |+uu|+||ou.
',uou,r +|e -,||-c !c| +ud 3cc
!-c- , |u| ||ee |e|r +|e uoW |ee|.ed |o|
||oe c+e o| uodu|+| .+cu|||| ||+| +|e +oc|
+|ed W||| !,:|c:|- ||-:| .
N00IAk vASCIIIIS |C|9 . 01.
|C|0 . A3.+
Ae oI 0oset Middle-aged to oldei peisons.
Sex Usually females.
to|oy Immune complex-mediated vasculai
injuiy due to bacteiial antigens has been im-
plicated. Immunoglobulins, complement, and
bacteiial antigens have been found by immu-
nofluoiescence and in some cases mycobacteiial
DNA sequences by polymeiase chain ieaction
(PCR). Bacteiial cultuies aie invaiiably nega-
tive.
Chionic, iecuiient, often bilateial, subcutane-
ous nodules and plaques with ulceiation of the
legs. Usually asymptomatic but may be tendei.
Often in middle-aged females with stubby col-
umn-like legs who woik in the cold.
Sko Iesoos Initially eiythematous, tendei,
oi asymptomatic subcutaneous nodules oi
plaques (Fig. 14-42) on the calves, iaiely on
shins and thighs. Lesions become bluish ied
in coloi, aie fiim, and fluctuate befoie ulceiat-
ing. Ulceis diain seious/oily fluid, aie iagged,
punched-out, and have violaceous oi biown
maigins (Fig. 14-42). They peisist foi pio-
longed peiiods befoie healing with atiophic
scais.
AssvcIuted FIndIngs Folliculai peiniosis, li-
vedo, vaiicose veins, and a cool, edematous
skin.
Ceoera| xamoatoo Patients aie usually
healthy.
0ermatopatho|oy Tubeiculoid gianulomas,
foieign-body giant cell ieaction, and neciosis
of fat lobules. Medium-sized vessel vasculitis,
piedominantly venulai but sometimes aite-
iial, in the septal aieas. Fibiinoid neciosis oi a
gianulomatous chionic inflammatoiy infiltiate
invades between the fat cells, ieplacing adipose
tissue and leading to fibiosis.
Sko Iesto Patients with an association with
M. u|ertu|oss infection aie highly sensitive to
tubeiculin and puiified piotein deiivative (PPD);
theiefoie, skin testing to M. u|ertu|oss antigens
should be peifoimed. In such patients, M. u|er-
tu|oss DNA sequences can be found by PCR.
By clinical findings and biopsy.
0IIereota| 0aooss ReJ noJu|es on |egs : Ei-
ythema nodosum, othei foims of panniculitis,
cutaneous panaiteiitis nodosa. Noe : Eiythema
nodosum is hot, veiy tendei, and nevei ulcei-
ates.
C0kS AN0 Fk0CN0SIS
Chionic iecuiient, scaiiing.
MANACMNI
Antitubeiculous theiapy in those cases wheie
M. u|ertu|oss etiology is pioved. In othei cases,
bed iest, compiession stockings, tetiacyclines,
and potassium iodide have pioved effective.
Systemic glucocoiticoids aie sometimes neces-
saiy foi iemission. In some cases dapsone is
effective.
FICk 14-42 Nodu|ar vascu|ts \u|||p|e, deepe+|ed, ||oWu |o ||u|| uodu|e, p+|||cu|+||, ou ||e po
|e||o| +pec| o| |o|| |oWe| |e. I|e |e|ou, W||c| +|e |e|+||.e|, +,rp|or+||c, r+, uude|o uec|o| |o|r|u
|oW|, |e+||u u|ce|. \+||coe .e|u +|e +|o eeu ou ||e |||| c+||.
Ae oI 0oset Peak incidence at 1 yeai, mean
2.6 yeais, uncommon aftei 8 yeais. Most cases
of KD in adults piobably iepiesent toxic shock
syndiome.
Sex Male piedominance, 1.5:1.
kace In United States: Japanese > blacks
> whites.
to|oy Unknown.
Seasoo Wintei and spiing.
Ceoraphy Fiist iepoited in Japan, 1961;
United States, 1971. Epidemics.
FAIh0CNSIS
Geneialized vasculitis. Endaiteiitis of vasa va-
soium involves adventitia/intima of pioximal
coionaiy aiteiies with ectasia, aneuiysm foima-
tion, vessel obstiuction, and distal embolization
with subsequent myocaidial infaiction. Othei
vessels: biachiocephalic, celiac, ienal, iliofemo-
ial aiteiies. Incieased activated helpei T cells
and monocytes, elevated seium inteileukin (IL)
1, TNF- , IL-6, adienomedullin and vascu-
lai endothelial giowth factoi, anti-endothelial
antibodies, and incieased cytokine-inducible
activation antigens on the vasculai endothe-
lium occui in KD. T cell iesponse is diiven by
a supeiantigen.
CIINICAI MANIFSIAII0N[FhASS
Fhase I: Acute Febr|e Ferod Abiupt onset of
fevei, lasting appioximately 12 days, followed
(usually within 1-3 days) by most of the othei
piincipal featuies. Constitutional symptoms of
diaiihea, aithiitis, photophobia.
K+W++|| d|e+e (K|) | +u +cu|e |e||||e |||ue o|
|u|+u| +ud c|||d|eu.
C|+|+c|e||/ed |, cu|+ueou +ud ruco+| e|,
||er+ +ud eder+ W||| u|equeu| dequ+r+
||ou, ce|.|c+| |,rp|+deu|||.
B||+|e|+| |u||+| uoue\ud+||.e coujuuc||.+| |ujec
||ou, |u||+rr+||ou o| o|op|+|,u\.
Corp||c+||ou. co|ou+|, +|uo|r+||||e, |uc|ud|u
+ueu|,r (!0), r,oc+|d|||, +|||||||, u|e||||||,
+ud +ep||c reu|u|||.
|rred|+|e ||e+|reu| W||| |u||+.euou |rru
uo|o|u||u +ud +p|||u |educe co|ou+|, +ueu
|,r.
',, . \ucocu|+ueou |,rp| uode ,u
d|ore.
I|| |ud|c+|e ||+| K| | corrou W|eu ||e|e +|e
ep|der|c.
kAWASAkI 0ISAS |C|9 . ++o.
|C|0 . \!0.! ( )
Fhase II: Subacute Fhase Lasts appioximately
until day 30 of illness; fevei, thiombocyto-
sis, desquamation, aithiitis, aithialgia, caiditis;
highest iisk foi sudden death.
Fhase III: Coova|esceot Ferod Begins within
8-10 weeks aftei onset of illness when all signs
of illness have disappeaied and ends when ESR
ietuins to noimal; veiy low moitality iate dui-
ing this peiiod.
Sko Iesoos
Fhase I Lesions appeai 1-3 days aftei onset
of fevei. Duiation 12 days aveiage. Neaily
all mucocutaneous abnoimalities occui duiing
this phase.
Erunthem Eiythema usually fiist noted on
palms/soles, spieading to involve tiunk and
extiemities within 2 days. Fiist lesions: eiy-
thematous macules; lesions enlaige and become
moie numeious (Fig. 14-43). Type: uiticaiia-
like lesions most common; moibillifoim pat-
tein second most common; scailatinifoim and
eiythema multifoime-like in <5% of cases.
Confluent macules to plaque-type eiythema
on peiineum, which peisist aftei othei findings
have iesolved. Edema of hands/feet: deeply eiy-
thematous to violaceous; biawny swelling with
fusifoim fingeis (Fig. 14-44). Palpation: lesions
may be tendei.
Mucvus Memhrunes Bulbai conjunctivae:
bilateial vasculai dilatation (conjunctival in-
jection); noted 2 days aftei onset of fevei; duia-
tion, 1-3 weeks (thioughout the febiile couise).
Lips: ied, diy, fissuied (Fig. 14-44), hemoi-
ihagic ciusts; duiation, 1-3 weeks. Oiophaiynx:
diffuse eiythema. Tongue: stiawbeiiy" tongue
(eiythema and piotubeiance of papillae of
tongue).
Fhase II Desquamation highly chaiacteiistic;
follows iesolution of exanthem (Fig. 14-45).
FICk 14-43 kawasak dsease B|o|c|, e|,||er+ ou ||e ||uu| o| + c|||d, |u||+| coujuuc||.|||, |,rp|+de
uop+||,, +ud '||+W|e||, |ouue We|e +|o p|eeu|.
FICk 14-44 kawasak dsease
C|e||,|ed ||p W||| |ero|||+|c ||
u|e, |u + |||||e |o, W||| p|o|oued
||| |e.e|. I|| c|||d +|o |+d + eue|
+||/ed ro|||||||o|r e|up||ou, |ujec|ed
coujuuc||.+e, +ud '||+W|e||, |ouue
(uo| |oWu). |o|e e|,||er+ +ud
eder+ o| ||ue|||p.
Begins on tips of fingeis and toes at junction of
nails and skin; desquamating sheets of palmai/
plantai epideimis aie piogiessively shed.
Fhase III Beau lines (tiansveise fuiiows on
nail suiface) may be seen (see Section 33). Pos-
sible telogen effluvium.
Ceoera| Fodos Meningeal iiiitation. Pneu-
monia. Lymphadenopathy, usually ceivical
nodes: 1.5 cm, slightly tendei, fiim. Aithiitis/
aithialgias, knees, hips, elbows. Peiicaidial tam-
ponade, dysihythmias, iubs, congestive heait
failuie, left ventiiculai dysfunction.
Chemstry Abnoimal livei function tests.
hemato|oy Leukocytosis (>18,000/L).
Thiombocytosis aftei the tenth day of illness.
Elevated ESR in phase II. ESR ietuins to noimal
in phase III.
roa|yss Pyuiia.
0ermatopatho|oy Aiteiitis involving small
and medium-sized vessels with swelling of en-
dothelial cells in postcapillaiy venules, dilatation
of small blood vessels, lymphocytic/monocytic
peiivasculai infiltiate in aiteiies/aiteiioles of
deimis.
|ectrocardoraphy Piolongation of PR and
QT inteivals; ST-segment and T-wave changes.
chocardoraphy aod Aooraphy Coionaiy
aneuiysms in 20% of cases.
Diagnostic ciiteiia: fevei spiking to >39.4 C,
lasting 5 days without othei cause, associated
with foui of five ciiteiia: (1) bilateial conjunc-
tival injection; (2) at least one of following
mucous membiane changes: injected/fissuied
lips, injected phaiynx, stiawbeiiy" tongue; (3)
at least one of the following extiemity changes:
eiythema of palms/soles, edema of hands/feet,
geneialized/peiiungual desquamation; (4) dif-
fuse scailatinifoim oi deeply eiythematous
maculopapulai iash, iiis lesions; and (5) ceivi-
cal lymphadenopathy (at least one lymph node
1.5 cm in diametei).
0IIereota| 0aooss Adveise cutaneous diug
eiuption, juvenile iheumatoid aithiitis, infec-
tious mononucleosis, viial exanthems, lepto-
spiiosis, Rocky Mountain spotted fevei, toxic
shock syndiome, staphylococcal scalded-skin
syndiome, eiythema multifoime, seium sick-
ness, SLE, ieactive aithiitis syndiome.
C0kS AN0 Fk0CN0SIS
Clinical couise tiiphasic. Uneventful iecov-
eiy occuis in majoiity. Caidiovasculai system
complications in 20%. Coionaiy aiteiy an-
euiysms occui within 2-8 weeks, associated
with myocaiditis, myocaidial ischemia/infaic-
tion, peiicaiditis, peiipheial vasculai occlusion,
small-bowel obstiuction, stioke. Case fatality
iate, 0.5-2.8% of cases, and is associated with
coionaiy aiteiy aneuiysms.
MANACMNI
Diagnosis should be made eaily and attention
diiected at pievention of the caidiovasculai
complications.
hospta|tatoo Recommended duiing the
phase I illness, monitoiing foi caidiac and vas-
culai complications.
Systemc Iherapy 1ntruvenvus 1mmunvg|vh-
u|In 2 g/kg as a single infusion ovei 10 h to-
gethei with aspiiin.
AspIrIn 100 mg/kg pei day until fevei iesolves
oi until day 14 of illness, followed by 5 to 10
mg/kg pei day until ESR and platelet count have
ietuined to noimal.
G|ucvcvrtIcvIds CvntruIndIcuted Associated
with a highei iate of coionaiy aneuiysms.
FICk 14-45 kawasak dsease '|edd|u o| ||e ep|de|r| ou ||e p+|r o| ||| c|||d 0 d+, +||e| ||e
+cu|e |||ue.
Ae oI 0oset 22 yeais (median) in the type
following sexually tiansmitted infection (STI).
Sex 90% of patients aie males (postveneieal
type).
kace Most common in Caucasians fiom
noithein Euiope; iaie in Asians and Afiican
blacks.
Ceoetc 0athess HLA-B27 occuis in up to
75% of Caucasians with RA but in only 8% of
healthy Caucasians. Patients who aie HLA-B27-
negative have a mildei couise, with significantly
less sacioiliitis, uveitis, and caiditis.
Assocated 0sorders Incidence of RA may be
incieased in HIV-infected individuals.
to|oy Unknown.
FAIh0CNSIS
RA appeais linked to two factois: (1) genet
[ators , i.e., HLA-B27 and (2) enert a|ogens
such as Sa|mone||a enerJs , S. y|murum ,
S. |eJe||erg ; Yersna eneroto|ta , Y. seuJou-
|ertu|oss ; Camy|o|ater [eus ; S|ge||a [|exner ;
oi genitouiinaiy pathogens (such as C||amyJa
oi Urea|asma urea|ytum ). Two patteins aie
obseived: the eJemt [orm , which follows STI,
the most common type in the United States
and the United Kingdom; and the osJysenert
[orm following GI infection, the most common
type in continental Euiope and Noith Afiica.
Onset 1-4 weeks aftei infection: enteiocoli-
tis; nongonococcal uiethiitis. Uiethiitis and/oi
conjunctivitis usually fiist to appeai, followed
by aithiitis.
Symptoms consist of malaise, fevei, dysuiia,
uiethial dischaige. Eyes: ied, slightly sensitive.
ke+c||.e +||||||| (kA) | de||ued |, +u ep|ode o|
pe||p|e|+| +||||||| o| > rou||' du|+||ou occu|
||u |u +oc|+||ou W||| u|e|||||| +ud/o| ce|.|c|||.
|u|||+||ou |, |u|ec||ou, uu+||, |u ||e eu||ou||u+|,
+ud +||o|u|e||u+| ||+c|.
'c|-||c Cc,||c:|- '|-||c -c,
+ud C||c,!c |||e| kA, |u| o||e| |u|ec||ou
c+u +|o |e |u|||+|o|.
||equeu||, +ccorp+u|ed |, |e|+|ode|r+ ||eu
uo|||+|cur, c||c|u+|e |+|+u|||, coujuuc||.|||,
+ud |or+||||.
I|e c|+|c |||+d | +|||||||, u|e||||||, +ud coujuuc
||.|||.
|C|9 . 1.0
|C|0 . \02.!
kACIIv AkIhkIIIS (kIIk SN0k0M)
Aithiitis: tendon/fascia inflammation iesults
in pain ovei ischial tubeiosities, iliac ciest, long
bones, iibs; heel pain at site of attachment of
plantai aponeuiosis (plantai fasciitis) and/oi
Achilles tendon; back pain; joint pains.
Sko Iesoos Resemble those of psoiiasis,
especially on palms/soles, glans penis.
KeraoJerma ||ennorr|agtum : biownish-
ied papules oi macules, sometimes topped by
vesicles that enlaige; centeis of lesions become
pustulai and/oi hypeikeiatotic, ciusted (Fig.
14-46), i.e., iesembling mollusk shells, mainly
on palms and soles. Scaling eiythematous,
psoiiasifoim plaques on scalp, elbows, and
buttocks. Eiosive patches iesembling pustulai
psoiiasis may occui, especially on shaft of penis,
sciotum. Crtnae |a|ans (Fig. 14-47): shal-
low eiosions with seipiginous, miciopustulai
boideis if unciicumcised; ciusted and/oi hy-
peikeiatotic plaques if ciicumcised, i.e., psoiia-
sifoim.
Na|s Small subungual pustules; onycholy-
sis and subungual hypeikeiatosis.
Mucous Membraoes Urethru Steiile seious
oi mucopuiulent dischaige.
Mvuth Eiosive lesions on tongue oi haid pal-
ate, iesembling migiatoiy glossitis.
Eyes Conjunctivitis, mild, evanescent, bilat-
eial; anteiioi uveitis.
Systemc Fodos Aithiitis: oligoaiticulai,
asymmetiic; most commonly knees, ankles,
small joints of feet; diffuse swelling of fingeis
and toes, enthesitis. (See Section 3, psoiiatic
aithiitis).
hemato|oy Nonspecific findings: anemia,
leukocytosis, thiombocytosis, elevated ESR.
Cu|ture Uiethial cultuie negative foi gono-
coccus, may be positive foi C||amyJa oi
FICk 14-46 keactve arthrts: keratoderma b|eooorrhacum ked|o||oWu p+pu|e, .e|c|e, +ud
pu|u|e W||| ceu||+| e|o|ou +ud c|+|+c|e||||c c|u||u +ud pe||p|e|+| c+||u ou ||e do|o|+|e|+| +ud p|+u|+| |oo|.
FICk 14-4T keactve arthrts: ba|aots crcoata \o||, We||der+|c+|ed e|o|ou W||| + |||||,
|+|ed r|c|opu|u|+| c||c|u+|e |o|de| ou ||e |+u peu|.
Urea|asma . Stool cultuie: may be positive foi
S|ge||a , Yersna, and otheis.
Sero|oy ANA, iheumatoid factoi negative.
HIV seiology.
0ermatopatho|oy Spongiosis, vesiculation;
latei, psoiiasifoim epideimal hypeiplasia,
spongifoim pustules, paiakeiatosis. Peiivascu-
lai neutiophilic infiltiate in supeificial deimis;
edema.
Clinical findings: aithiitis and skin lesions iul-
ing out othei spondylo- and ieactive aithiopa-
thies: psoiiasis vulgaiis with psoiiatic aithiitis,
disseminated gonococcal infection, SLE, anky-
losing spondylitis, iheumatoid aithiitis, gout,
Behet disease.
C0kS AN0 Fk0CN0SIS
Only 30% develop complete tiiad of aithiitis,
uiethiitis, conjunctivitis; 40% have only one
manifestation, i.e., incomplete RA. Majoiity
have self-limited couise, with iesolution in
3-12 months. RA may ielapse ovei many yeais
in 30%. Chionic defoiming aithiitis in 10 to
20%.
MANACMNI
Fror IoIectoo Role of antibiotic theiapy un-
pioven in alteiing couise of postveneieal RA.
Cutaoeous MaoIestatoos Similai to manage-
ment of psoiiasis (see Section 3). Balanitis: low-
potency glucocoiticoids. Palmai/plantai: potent
glucocoiticoid piepaiations, which aie moie
effective undei plastic occlusion. Extensive oi
iefiactoiy disease: systemic ietinoids (acitietin,
0.5-1 mg/kg body weight), photo theiapy, and
PUVA. Anti-TNF agents.
Freveotoo oI Artcu|ar IoI|ammatoo[loot
0eIormty Rest, nonsteioidal anti-inflamma-
toiy agents. Occasionally, phenylbutazone is in-
dicated, methotiexate, acitietin. In HIV/AIDS,
antiietioviial theiapy may amelioiate RA.
FI0MI0I0C
Ae oI 0oset Undei 40 yeais (iange 12-70
yeais).
kace All iaces. In the United States and South
Afiica, much moie fiequent in blacks. The disease
occuis woildwide; fiequent in Scandinavia.
0ther Factors Etiology unknown. The disease
can occui in families.
Onset of lesions: days (piesenting as acute
eiythema nodosum) oi months (piesenting
as asymptomatic saicoidal papules oi plaques
on skin oi pulmonaiy infiltiate discoveied
on ioutine chest iadiogiaphy). Constitutional
symptoms such as fevei, fatigue, weight loss,
aiihythmia.
Sko Iesoos Eailiest lesions aie skin-coloied
papules, occuiing peiioiificially on the face.
Biownish oi puiple infiltiated plaques that
may be annulai, polycyclic, seipiginous, and
occui mainly on extiemities, buttocks, and
tiunk (Fig. 14-48). Cential cleaiing with slight
atiophy may occui. Multiple scatteied maculo-
papulai oi papulai lesions, 0.5-1 cm, yellowish
biown, oi puiple occui mainly on the face (Fig.
14-49) and extiemities. Occasionally, nodules,
fiim, puiple oi biown, may aiise on the face,
tiunk, oi extiemities, paiticulaily hands (Fig.
14-51). Luus erno: diffuse, violaceous, soft
doughy infiltiations on the nose, cheeks, oi
eailobes (Fig. 14-50). Swelling of individual
digits (Fig. 14-51). Saicoidosis tends to infil-
tiate old scais, which then exhibit tianslucent
puiple-ied oi yellowish papules oi nodules.
Noe : On blanching with glass slide, all cutane-
ous lesions of saicoidosis ieveal apple jelly"
A ,|er|c |+uu|or+|ou d|e+e o| uu|uoWu
c+ue.
|||r+|||, +||ec||u ||e |uu (|||+|e|+| |,rp|+de
uop+||,, pu|rou+|, |u|||||+||ou).
'||u. p+pu|e, ||+u|uceu| ,e||oW|ed W||| +pp|e
je||, +ppe+|+uce ou d|+cop,, uodu|e +ud ||u||
|ed p|+que.
0||eu |oc+||/e |u c+|.
n||o|o|c+||,, uouc+e+||u, 'u+|ed |+uu|o
r+.
E|,||er+ uodour | ||e ro| corrou uou
pec|||c |e|ou |u ||e ||u |u e+||, +|co|do|, ||
ue| + ood p|ouo|.
SAkC0I00SIS |C|9 . !5
|C|0 . |3o
semitianslucent yellowish biown coloi. On the
scalp saicoidosis may cause scaiiing alopecia
(see Section 32).
Systems kevew Enlaiged paiotids, pulmo-
naiy infiltiates, caidiac dyspnea, neuiopathy,
uveitis, kidney stones. Loe[gren synJrome: eiy-
thema nodosum, fevei, aithialgias, acute bilat-
eial hilai adenopathy. Heer[orJ (-Va|Jensrm)
synJrome: fevei, paiotitis, uveitis, facial palsy.
0ermatopatho|oy Laige islands of epithelioid
cells with a few giant cells and lymphocytes (so-
called naked tubeicles). Asteioid bodies in laige
histiocytes; occasionally fibiinoid neciosis.
Sko Iests Intiacutaneous tests foi iecall anti-
gens usually but not always negative.
Imao Systemic involvement is veiified ia-
diologically by gallium scan and tiansbionchial,
livei, oi lymph node biopsy. In 90% of patients:
hilai lymphadenopathy, pulmonaiy infiltiate.
Cystic lesions in phalangeal bones (osteitis
cystica).
8|ood Chemstry Incieased level of seium
angiotensin-conveiting enzyme, hypeigamma-
globulinemia, hypeicalcemia.
0IACN0SIS
Lesional biopsy of skin oi lymph nodes is the
best ciiteiion foi diagnosis of saicoidosis.
MANACMNI
Systemc Sarcodoss Systemic glucocoiticoids
foi active oculai disease, active pulmonaiy dis-
ease, caidiac aiihythmia, CNS involvement, oi
hypeicalcemia.
FICk 14-48 Sarcodoss: raou|omatous |esoos \u|||p|e, c||c|u+|e, cou||ueu|, |||r, ||oWu|||ed, |u|||
||+|ed p|+que ||+| |oW + |eudeuc, |o |eo|.e |u ||e ceu|e|. I|u, ||e +uuu|+| +ud ru|||ceu|||c +ppe+|+uce. I|e
|e|ou +|e d|+cop, po|||.e, |.e., +u '+pp|eje||, |+up|u| co|o| |er+|u |u |e|ou +||e| corp|e|ou W||| |+.
FICk 14-49 Sarcodoss B|oWu|||opu|p|e p+pu|e co+|ec|u |o |||eu|+| p|+que, occu|||u ou uoe o|
||| Wor+u W|o +|o |+d r+|.e pu|rou+|, |u.o|.ereu|. B|+uc||u W||| + |+ ||de |e.e+| '+pp|eje||, co|o|
|u ||e |e|ou.
Cut aoeous Sarcodos s G|ucvcvrtIcvIds
Lota| : intialesional tiiamcinolone, 3 mg/mL, ef-
fective foi small lesions. Sysemt : glucocoiticoids
foi widespiead oi disfiguiing involvement.
Hydrvrych|vrvquIne 100 mg twice daily foi
widespiead oi disfiguiing lesions iefiactoiy to
intialesional tiiamcinolone. Only sometimes
effective.
Methvtrerute Low-dose foi widespiead skin
and systemic involvement. Not always effective.
AntI-TNF- Agents, including thalidomide,
anecdotally effective.
FICk 14-50 Sarcodoss I|| | ||e c|+|c +ppe+|+uce o| '|upu pe|u|o W||| .|o|+ceou, o||, dou|,
|u|||||+||ou ou c|ee| +ud uoe, W||c| | |o|, eu|+|ed.
FICk 14-51 Sarcodoss |+pu|+| ||oWu|| |o .|o|+ceou |e|ou ou ||e do|+ o| ||e |+ud o| + +0,e+|
o|d Wor+u W|o +|o |+d pu|rou+|, |u.o|.ereu|. |o|e We|||u o| ||e |ou||| d||| o| ||e |e|| |+ud +ud o| ||e
||||| d||| o| ||e |||| |+ud.
420
N00CkIN, MIA80IIC,
NIkIII0NAI, AN0 CNIIC
0ISASS
S E C I | 0 N 1 5
|o|r+| ||u c|+ue +oc|+|ed W||| p|eu+uc,
+|e d+||eu|u o| ||ue+ +||+ (||ue+ u||+), re|+r+
(ee 'ec||ou !), |||+e d||eu+e (||. 5).
||u|||u occu|||u |u p|eu+uc, r+, |e due |o
+ ||+|e o| p|ee\|||u de|r+|o| o| + p|eu+uc,
pec|||c de|r+|o|.
||eu+uc,pec|||c de|r+|oe +oc|+|ed W|||
|e|+| ||| +|e c|o|e|+| |u p|eu+uc,, pu|u|+|
po||+| o| p|eu+uc, (|rpe||o |e|pe|||o|r|),
+ud perp||o|d e|+||ou|.
||eu+uc,pec|||c de|r+|oe uo| +oc|+|ed
W||| |e|+| ||| +|e po|,ro|p||c e|up||ou o| p|e
u+uc, +ud p|u||o e|+||ou|.
SkIN 0ISASS IN FkCNANC
0ccu| |u ||e ||||d |||re|e|.
|e+d|u ,rp|or. p|u|||u, e|||e| |oc+||/ed
(p+|r) o| eue|+||/ed. \o| e.e|e du||u ||e
u|||.
Cu|+ueou |e|ou |u.+||+||, +|eu|, |u| e\co||+
||ou |u e.e|e c+e.
E|e.+||ou o| e|ur |||e +c|d.
|e|+| ||| |uc|ude p|er+|u|||,, |u||+p+||+| d|||e,
+ud |e|+| de+||.
I|e+|reu|. u|odeo\,c|o||c +c|d, p|+r+p|e|e|.
Ch0ISIASIS 0F FkCNANC (CF) |C|9 . o+o.1
|C|0 . K3!.
||e.|ou|, c+||ed -| |--||.
C||u|c+||, +ud |||op+||o|o|c+||, |ud|||uu||+||e
||or pu|u|+| po||+| o| .ou /ur|uc|
Bu|u|u, r+|||u, uo| ||c||u.
\+, |+.e |,poc+|cer|+ +ud dec|e+ed .||+r|u |
|e.e|.
'ee '|u|u|+| |o||+|, 'ec||ou !.
|erp||o|d e|+||ou| (|C) | + p|u||||c po|,ro|
p||c |u||+rr+|o|, de|r+|o| o| p|eu+uc, +ud ||e
po|p+||ur pe||od. || | +u +u|o|rruue p|oce W|||
c||cu|+||u corp|ereu|||\|u |C +u|||od|e |u ||e
e|ur. I|e coud|||ou | dec|||ed |u 'ec||ou o.
FSIIAk FS0kIASIS IN FkCNANC |C|9 . o9o.1
|C|0 . |+0.
FMFhIC0I0 CSIAII0NIS |C|9 . o+o.3
|C|0 . 02o.+
SCII0N 15 E||0Ck||E, \EIAB0||C, |uIk|I|0|A|, A|| CE|EI|C ||'EA'E' 421
FICk 15-1 Strae dsteosae o a preoaot womao (36 weeks oI estatoo)
|E| | + d|||uc| p|u||||c e|up||ou o| p|eu+uc, ||+|
uu+||, |e|u |u ||e ||||d |||re|e|, ro| o||eu |u
p||r||+.|d+e (1o).
I|e|e | uo |uc|e+ed ||| o| |e|+| ro|||d||, o|
ro||+|||,.
I|e d|e+e | corrou, e||r+|ed |o |e |u 20
|o 2+0 p|eu+uc|e.
I|e e||o|o, +ud p+||oeue| +|e uo| uude|
|ood.
A.e|+e ||re o| oue| | !o Wee| o| e|+||ou,
uu+||, -2 Wee| |e|o|e de||.e|,. noWe.e|,
,| c! :c |c| ||- |c|
-! .
||u|||u de.e|op ou ||e +|doreu, o||eu |u ||e
|||+e d||eu+e, +ud | e.e|e euou| |o d||up|
|eep. '| |- cou|| o| e|,||er+|ou p+
pu|e, -! rr, qu|c||, co+|ec|u |u|o u|||c+||+|
p|+que (||. 52) W||| po|,c,c||c |+pe +ud
+||+uereu|, ||+uc|ed |+|o +|ouud ||e pe||p|
e|, o| |e|ou. I|u, .e|c|e, 2 rr, r+, occu| |u
||e p|+que, |u| |u||+e +|e +|eu|. I+|e| |e|ou
+|e o|e|.ed |u 9. A|||ou| p|u|||u | ||e
c||e| ,rp|or, e\co||+||ou +|e |u||equeu|. 50
o| ||e Woreu +||ec|ed |+.e p+pu|e +ud p|+que
|u ||e |||+e d||eu+e, ||e +|doreu, |u||oc|,
|||| (||. 52), uppe| |uue| +|r, +ud |oWe|
|+c| r+, +|o |e +||ec|ed.
I|e |+ce, ||e+|, p+|r, +ud o|e +|e |+|e|,
|u.o|.ed. I|e pe||ur||||c+| +|e+ | uu+||, p+|ed.
I|e|e +|e uo rucou rer||+ue |e|ou.
||||e|eu||+| d|+uo| |uc|ude +|| p|u||||c +|dor|
u+| |+|e |u p|eu+uc,. perp||o|d e|+||ou|,
+d.e|e cu|+ueou d|u |e+c||ou, +||e||c cou|+c|
de|r+||||, re|+|o||c p|u|||u, +|op|c de|r+||||.
|c|c|, |! +|e uoucou||||u|o|,, + +|e
|||op+||o|o, +ud |rruuo|||op+||o|o,.
I|e r+jo|||, o| Woreu do uo| |+.e + |ecu||euce
|u ||e po|p+||ur pe||od, W||| u|equeu| p|e
u+uc|e, o| W||| ||e ue o| o|+| cou||+cep||.e. || +
|ecu||euce occu|, || | uu+||, ruc| r||de|
\+u+ereu| cou|| o| |||po|euc, |op|c+|
|e|o|d ||+| o||eu c+u |e |+pe|ed o|| +||e| Wee|
o| ||e|+p,. 0|+| p|edu|oue |u doe o| 0-+0
r/d |+ |eeu ued |o| e.e|e c+e, o||eu ||e
,rp|or +|e |e||e.ed |u 2+ |. 0|+| +u|||||+
r|ue +|e eue|+||, |ue||ec||.e.
',,. |o|,ro|p||c e|up||ou o| p|eu+uc,,
|o\er|c |+| o| p|eu+uc,, |+|eoue| p|u||o o|
p|eu+uc,.
||u||o o| p|eu+uc, | uoW |ec|+|||ed + p+||
o| ||e c|: -| | -c:, (AE|) pec
||ur.
\e|, corrou.
AE| cou|| o| ||+|e o| +|op|c de|r+|||| (+|o |u
p+||eu| W|o p|e.|ou|, d|d uo| |+.e A|), p|eeu|
e|||e| W||| ec/er+|ou o| p|u||o |e|ou (ee
'ec||ou 2).
I|e c+|d|u+| ,rp|or | p|u|||u (||e +|o||||r
ou p+e +2! |ep|eeu| +u +pp|o+c| |o ||e p|e
u+u| p+||eu| W||| p|u|||u).
C\ Ar||okudo|p| e| +|. l Ar Ac+d |e|r+|o| 5+.!95, 200o.

F0IM0kFhIC kFII0N 0F FkCNANC (FF) |C|9 . 109.3
FkkIC0 0F FkCNANC AN0 AI0FIC kFII0N 0F FkCNANC |C|9 . o932 l l 132.
FICk 15-2 Fo|ymorphc eruptoo oI preoaocy [prevous|y ca||ed prurtc urtcara| papu|es aod
p|aques oI preoaocy (FFFF)j u|||c+||+| p+pu|e +|e p|eeu| ou |o|| |||| W|e|e ||e, co+|ece |o u|||
c+||+| p|+que. '|r||+| p+pu|e +ud u|||c+||+| |e|ou +|e p|eeu| W||||u |||+e d||eu+e ou ||e +|doreu o| |||
p|eu+u| Wor+u +| !5 Wee| o| e|+||ou. |e|ou We|e e\||ere|, p|u||||c, c+u|u |eep|e u||| +ud |e+|
||e, ,e| ||e|e +|e uo e\co||+||ou.
Preguauc] aud pruritus
w|th
sk|o |es|oos
w|tho0t
sk|o |es|oos
for preguauc]
0o|oc|deota|
dermatos|s
AP
0P
preguauc]
Truuk aud
extremities
PP
Papular
urticarial
P6
Urticarial
vesicular
lucreased serum
oile acids
Earl] (oefore
8rd trimester}
Predomiuautl] aodomeu
late (8rd trimester}
postpartum
A|o||||r o| +pp|o+c| |o p+||eu| W||| p|u|||u
Sk|h 0|SASS ASS00|AT0 w|Th
0|A8TS NLL|T0S
3
ACANIh0SIS NICkICANS (p. 88) AN0
IIF00SIk0Fh
4
Associated with insulin iesistance in diabetes
mellitus. Insulin-like epideimal giowth factois
may cause epideimal hypeiplasia.
A0vkS CIAN0S 0kC kACII0NS IN
0IA8IS (see Sectoo 22)
Insu|n : local ieactions-lipodystiophy with
decieased adipose tissue at sites of sub-
cutaneous injection; Aithus-like ieaction
with uiticaiial lesion at site of injection.
Sysemt nsu|n a||ergy : Uiticaiia, seium
sickness-like ieactions.
Ora| |yog|ytemt agens : Exanthematous
eiuptions, uiticaiia, eiythema multi-
foime, photosensitivity.
2
Foi a thoiough discussion see G Yosipovitch et al. J
Am Acad Deimatol 57:730, 2007.
3
Figuies in paientheses indicate page numbeis wheie
these conditions aie dealt with.
4
Foi these conditions see K Wolff et al (eds): F:-
art|'s Dermao|ogy n Cenera| MeJtne 7 th ed.
New Yoik, McGiaw-Hill, 2008.
0|e||, | W|de|, |ecou|/ed + +u ep|der|c |u ||e
we|e|u Wo||d.
0|e||, | |epou|||e |o| c|+ue |u ||u |+|||e|
|uuc||ou, e|+ceou |+ud +ud e|ur p|oduc
||ou, We+| |+ud, |,rp|+||c, co||+eu ||uc|u|e
+ud |uuc||ou, Wouud |e+||u, r|c|o +ud r+c|o
c||cu|+||ou, +ud u|cu|+ueou |+|.
0|e||, | |rp||c+|ed |u + W|de pec||ur o| de|
r+|o|o|c d|e+e, |uc|ud|u c:c||
:c ('ec||ou 5), +c|oc|o|dou, |e|+|o| p||+||
('ec||ou +), |,-c!- c! ||
('ec||ou !2), |c- !|-c- c!|c !|c
+ud |+| |ed|||||u||ou, |,rp|eder+ ('ec||ou o),
:|: .- ||:-:, +ud |c|c |,-|
-c| ('ec||ou +).
C-|||| | |-:| ('ec||ou 2+), |!c!-|
c|.c ('ec||ou ), c ('ec||ou !),
| -|c:- ,!- , +ud ||c:- |
(p. ++o).
SkIN MANIFSIAII0NS 0F 08SII
2
0IA8IS MIIIIS
CAICIFhIAXIS (see Sectoo 1T, p. 482)
CIAN0S FkF0kAIINC 0IS0k0kS
4
Raie conditions in which hoiny plugs peifoiate
into the deimis oi deimal debiis is eliminated
thiough the epideimis. Not always associated
with diabetes (see Section 17).
0IA8IIC 8IIA (8u||oss dabetcorum)
(p. 425)
0IA8IIC 0kM0FAIh (p. 42T)
kFIIv XANIh0MAS (p. 438)
CkANI0MA ANNIAk (p. 134)
INFCII0NS (see Sectoos 24 aod 25)
Pooily contiolled diabetes associated with in-
cieased incidence of piimaiy (fuiuncles, cai-
buncles) and secondaiy Sa|y|otottus aureus
infections (paionychia, wound/ulcei infection),
cellulitis ( S. aureus , gioup A stieptococcus),
eiythiasma, deimatophytoses (tinea pedis,
onychomycosis), candidiasis (mucosal and cu-
taneous), mucoimycosis with neciotizing na-
sophaiyngeal infections.
NCk08I0SIS IIF0I0ICA (p. 428)
FkIFhkAI Nk0FAIh (0abetc Ioot)
(p. 426)
FkIFhkAI vASCIAk 0ISAS (see
Sectoo 16)
Sma||-esse| astu|oa|y (mtroangoa|y):
Involves aiteiioles, venules, and capil-
laiies. Chaiacteiized by basement mem-
biane thickening and endothelial cell
piolifeiation. Piesents clinically as acial
eiysipelas-like eiythema, ulceiation.
Large-esse| astu|oa|y : Incidence gieatly
incieased in diabetes. Ischemia is most
often symptomatic on lowei legs and
feet with gangiene and ulceiation. Pie-
disposes to infections.
SCIk0MA 0IA8IIC0kM
4
Synonym : Scleiedema adultoium of Buschke.
Need not be associated with diabetes. Onset
coiielates with duiation of diabetes and with
piesence of micioangiopathy. Skin findings:
pooily demaicated scleiodeima-like induia-
tion of the skin and subcutaneous tissue of the
uppei back, neck, pioximal extiemities. Rapid
onset and piogiession.
Sc|eroderma-Ike Syodrome
4
Scleiodeima-
like thickening of skin and limited joint mobil-
ity (piayei sign").
|+|e, |u|+c| |u||+e +||e pou|+ueou|, ou ||e
|oWe| |e, |ee|, do|+ o| ||e |+ud, +ud ||ue|
ou uou|u||+red |+e (||. 5!).
w|eu |up|u|ed, oo/|u ||||| |ed e|o|ou |eu||
|u| |e+| +||e| e.e|+| Wee|.
|oc+||/+||ou ou do|+ o| |+ud +ud ||ue| u
e| po|p|,||+ cu|+ue+ |+|d+, |u| +|uo|r+||||e
o| po|p|,||u re|+|o||r +|e uo| |ouud.
|e|||e| ||+ur+ uo| +u |rruuo|o|c rec|+
u|r |+ |eeu |rp||c+|ed. n||o|o|c+||,, |u||+e
|oW |u||+ o| u|ep|de|r+| c|e|||u W|||ou|
+c+u||o|,|.
0IA8IIC 8IIA
FICk 15-3 0abetc bu||a A |+|e, |u|+c| |u||+ | eeu ou ||e p|e||||+| ||u ou ||e |||| |oWe| |e. I|e
p+||eu| |+d r+u, o| ||e .+cu|+| corp||c+||ou o| d|+|e|e re||||u, |.e., |eu+| |+||u|e, |e||uop+||,, +ud +||e|oc|e
|o| o||||e|+u |eu|||u |u +rpu|+||ou o| ||e ||e |e|| || |oe.
|e||p|e|+| ueu|op+||, | |epou|||e |o| ||e 'd|+
|e||c |oo|.
0||e| |+c|o| +|e +u|op+||,, +||e|oc|e|o|, +ud
|u|ec||ou +ud ro| o||eu ||e, +|e cor||ued.
||+|e||c ueu|op+||, | cor||ued ro|o| +ud
euo|,. \o|o| ueu|op+||, |e+d |o We+|ue +ud
ruc|e W+||u d||+||,.
Au|ouor|c ueu|op+||, +ccorp+u|e euo|,
ueu|op+||, +ud |e+d |o +u||d|o|, W||c| r+,
uo| |e cou||ued |o ||e d||+| e\||er|||e.
'euo|, ueu|op+||, p|ed|poe |o ueu|o||op|c
u|ce| o.e| |ou, p|or|ueuce o| |ee|, uu+||, ou
||e |e+| |oe +ud o|e + |oWu (||. 5+).
u|ce| +|e u||ouuded |, + ||u o| c+||u +ud r+,
e\|eud |o |u|e||,|u jo|u| +ud |oue, |e+d|u |o
o|eor,e||||.
"0IA8IIC F00I" AN0 0IA8IIC Nk0FAIh |C|9 . 1!.5

|C|0 . Co!.2
FICk 15-4 0abetc, oeuropathc u|cer oo the so|e A |+|e u|ce| o.e||,|u ||e ecoud |e|| re|+c+|
pop|+|+ue+| jo|u|. I|e p+||eu|, + o0,e+|o|d r+|e W||| d|+|e|e re||||u o| 25 ,e+|' du|+||ou, |+ |u|||c+u|
euo|, ueu|op+||, o| ||e |ee| +ud |oWe| |e + We|| + pe||p|e|+| .+cu|+| d|e+e, W||c| |eu||ed |u ||e +rpu
|+||ou o| ||e |ou||| +ud ||||| |oe.
SCII0N 15 E||0Ck||E, \EIAB0||C, |uIk|I|0|A|, A|| CE|EI|C ||'EA'E' 42T
C||curc|||ed, +||op||c, |||||, dep|eed |e|ou
ou ||e +u|e||o| |oWe| |e ||+| +|e +,rp|or+||c
(||. 55).
I|e, +||e |u c|op +ud |+du+||, |eo|.e, |u| ueW
|e|ou +ppe+| +ud occ+|ou+||, r+, u|ce|+|e.
I|e p+||oeu|c |u|||c+uce o| d|+|e||c de|rop+
||, |er+|u |o |e e|+||||ed, |u| || | o||eu
+ccorp+u|ed |, r|c|o+u|op+||,.
0IA8IIC 0kM0FAIh
FICk 15-5 0abetc dermopathy A
c|u|ed e|o|ou +| ||e ||e o| ||+ur+||c |uju|,
+ud r+u, o|d p|u| dep|eed +|e+ +ud c+|
+|e eeu ou ||e +u|e||o| |e o| + 5o,e+|o|d
r+|e W||| d|+|e|e re||||u. I|e o||e| |e |+d
|deu||c+| ||ud|u.
|ec|o||o| ||po|d|c+ (||) | + cu|+ueou d|o|de|
o||eu, |u| uo| +|W+,, +oc|+|ed W||| d|+|e|e
re||||u.
I|e |e|ou +|e d|||uc||.e, |+|p|, c||curc|||ed,
ru|||co|o|ed p|+que occu|||u ou ||e +u|e||o|
+ud |+|e|+| u||+ce o| ||e |oWe| |e.
|e|ou r+, u|ce|+|e.
NCk08I0SIS IIF0I0ICA |C|9 . 109.!
|C|0 . |92.
Ae oI 0oset Young adults, eaily middle age,
but not uncommon in juvenile diabetics.
Sex Female: male iatio 3:1 in both diabetic
and nondiabetic foims.
Iocdeoce Fiom 0.3-3% of diabetic individu-
als. NL may occui in individuals without mani-
fest diabetes. Re|aons| o Ja|ees : One-thiid
of patients have clinical diabetes, one-thiid have
abnoimal glucose toleiance only, one-thiid have
noimal glucose toleiance.
to|oy Unknown.
Frecptato Factors A histoiy of pieceding
tiauma to the site can be a factoi in the initial
development of the lesions.
FAIh0CNSIS
The aiteiiolai changes in the aieas of neciobio-
sis of the collagen have been thought by some
to be piecipitated by aggiegation of platelets.
The gianulomatous inflammatoiy ieaction is
believed to be due to alteiations in the collagen.
The seveiity of NL is not ielated to the seveiity
of diabetes. Fuitheimoie, contiol of the diabe-
tes has no effect on the couise of NL.
Slowly evolving and enlaiging ovei months,
peisisting foi yeais. Cosmetic disfiguiement;
pain in lesions that develop ulceis.
Sko Iesoos Lesion staits as biownish-ied oi
skin-coloied papule that slowly evolves into a
well-demaicated waxy plaque of vaiiable size
(Fig. 15-6). The shaiply defined and slightly
elevated boidei ietains a biownish-ied coloi,
wheieas the centei becomes depiessed and
acquiies a yellow-oiange hue. Thiough the shiny
and atiophic epideimis, multiple telangiectasias
of vaiiable size aie seen. Laigei lesions foimed by
centiifugal enlaigement oi meiging of smallei
lesions acquiie a seipiginous oi polycyclic
configuiation. Ulceiation may occui within the
plaques (Fig. 15-6B), and healed ulceis iesult in
depiessed scais. Buined-out lesions appeai as
tan aieas with telangiectasia.
DIstrIhutIvn Usually 1 to 3 lesions; >80% occui
on the shin; at times symmetiic. Less com-
monly, on feet, aims, tiunk, oi face and scalp;
iaiely may be geneialized.
0ermatopatho|oy Scleiosis, obliteiation of
the bundle pattein of collagen neciobiosis,
suiiounded by concomitant gianulomatous in-
filtiation in lowei deimis. Fat-containing foam
cells aie often piesent, impaiting the yellow
coloi to the clinical lesion. Deimal blood ves-
sels show micioangiopathy with endothelial
thickening and focal deposits of PAS-positive
mateiial. Piesence of immunoglobulins and
complement (C3) in the walls of the small
blood vessels.
Chemstry Abnoimal glucose toleiance test in
two-thiids of patients.
The lesions aie so distinctive that biopsy con-
fiimation is not necessaiy; howevei, biopsy may
be iequiied in eaily stages to iule out gianu-
loma annulaie (which fiequently coexists with
NL), saicoidosis, oi xanthoma.
C0kS AN0 Fk0CN0SIS
The lesions aie indolent and can enlaige to
involve laige aieas of the skin suiface unless
tieated. The lesions aie unsightly, and patients
aie often upset about the cosmetic appeaiance.
Ulceiated aieas within NL aie painful.
MANACMNI
C|ucocortcods TvpIcu| The application of
potent glucocoiticoids undei occlusion is help-
ful in some cases; howevei, ulceiations may oc-
cui when NL is occluded.
1ntru|esIvnu| Intialesional tiiamcinolone,
5 mg/mL, into active lesions oi lesion maigins
usually aiiests extension of plaques of NL. This
is the best tieatment, with 3-5 mg/mL tiiamci-
nolone suspension.
|ceratoo Most ulceiations within NL lesions
heal with local wound caie; if not, excision of
entiie lesion with giafting may be iequiied.
FICk 15-6 Necroboss |podca dabetcorum . A |+|e, ,rre|||c p|+que W||| +c||.e |+up|u|,
,e||oW, We||der+|c+|ed, |+|ed, |||r |o|de| +ud + ,e||oW ceu|e| |u ||e p|e||||+| |e|ou o| + 23,e+|o|d d|+|e||c
|er+|e. I|e ceu||+| p+|| o| ||e |e|ou +|e dep|eed W||| +||op||c c|+ue o| ep|de|r+| |||uu|u +ud |e|+u|
ec|+| ++|u| ,e||oW |+c||ouud. 8. |+|e |e|ou +||e| |e+|ed u|ce|+||ou. A .e|, e\|eu|.e p|+que o| uec|o||o|
||po|d|c+ ou ||e |oWe| |e o| + d|+|e||c |er+|e. Ap+|| ||or ||e |e+|u|e o| uec|o||o| ||po|d|c+ ||e|e | e\|eu|.e
c+|||u +ud +||op||c dep|eed c+|.
8
Cu||u ,ud|ore (C') | c|+|+c|e||/ed |, ||uu
c+| o|e||,, roou |+ce, +|dor|u+| |||+e, |,
pe||eu|ou, dec|e+ed c+||o|,d|+|e |o|e|+uce,
p|o|e|u c+|+|o||r, p,c||+|||c d||u||+uce, +ud
+reuo|||e+ +ud |||u||r |u |er+|e.
|| | +oc|+|ed W||| e\ce +d|euoco|||co|e|o|d
o| eudoeuou o| e\oeuou ou|ce.
C| !-c- |e|e| |o C' +oc|+|ed W|||
p||u||+|, +d|euoco|||co||op|c |o|roue (ACIn)
p|oduc|u +deuor+.
C' -!:c-| |e|e| |o C' c+ued |,
e\oeuou +dr|u|||+||ou o| |ucoco|||co|d.
'||u |e|ou. A p|e||o||c o|ee pe|ou W||| + 'c|+
|c |+|||u ||+| |eu|| ||or ||e |ed|||||u||ou
o| |+|. roou |+c|e (||. 51), '|u||+|o |urp,
||uuc+| o|e||,, +ud |||u +|r.
|u|p|e |||+e, ro||, ou ||e +|doreu +ud
||uu|, +||op||c ||u W||| e+, ||u||u +ud
|e|+u|ec|+|+.
|+c|+| |,pe||||c|o| W||| p|reu|ed |+|| +ud o|
|eu |uc|e+ed |+uuo |+|| ou ||e |+ce +ud +|r,
+ud|oeue||c +|opec|+ |u |er+|e.
Acue o| |eceu| oue| (W|||ou| coredoue) o|
||+||u o| e\|||u +cue.
Ceue|+| ,rp|or cou|| o| |+||ue +ud ru
c|e We+|ue, |,pe||eu|ou, pe|ou+|||, c|+ue,
+reuo|||e+ |u |er+|e, po|,u||+, +ud po|,d|p|+.
wo||up |uc|ude de|e|r|u+||ou o| ||ood |ucoe,
e|ur po|+|ur, +ud ||ee co|||o| |u 2+| u||ue.
A|uo|r+| de\+re||+oue upp|e|ou |e| W|||
|+||u|e |o upp|e eudoeuou co|||o| ec|e||ou
W|eu de\+re||+oue | +dr|u||e|ed. E|e.+|ed
ACIn.
CI c+u o| ||e +|doreu +ud ||e p||u||+|,. Ae
reu| o| o|eopo|o|.
\+u+ereu| cou|| o| e||r|u+||ou o| e\oeuou
|ucoco|||co|d o| ||e de|ec||ou +ud co||ec||ou o|
uude||,|u eudoeuou c+ue.
CShINC SN0k0M AN0 hFkC0kIICISM
|C|9 . 255.0
|C|0 . E2+
FICk 15-T Cusho syodrome ||e||o||c roou |+c|e W||| e|,||er+ +ud |e|+u|ec|+e o| c|ee| +ud
|o|e|e+d, ||e |+ce +ud uec| +ud up|+c|+.|cu|+| +|e+ (uo| dep|c|ed |e|e) |oW |uc|e+ed depo|||ou o| |+|.
\,\eder+ |eu|| ||or |uu|||c|eu| p|oduc||ou o|
||,|o|d |o|roue +ud c+u |e c+ued |, ru|||p|e
d||u||+uce.
n,po||,|o|d|r r+, |e ||,.: (e.., cou
eu||+|, p||r+|, |d|op+|||c, po|+||+||.e), |
(e.., |e|||+||e ||o,u||e||c de|ec|, r+|e|u+||,
||+ur|||ed, |od|ue de||c|euc,, d|u|uduced o|
c||ou|c ||,|o|d|||), ||.: (e.., p||u||+|,),
o| |,||c|c: e.., |u|ec||ou (eucep|+||||),
ueop|+r|.
E+||, ,rp|or o| ,-!-c +|e o||eu o.e|
|oo|ed. |+||ue, |e||+|,, co|d |u|o|e|+uce, cou
||p+||ou, ||||ue +ud c|+rp|u o| ruc|e,
c+|p+| |uuue| ,ud|ore, reuo|||+|+, |oW|u o|
|u|e||ec|u+| +ud ro|o| +c||.||,, dec||ue |u +ppe|||e,
|uc|e+e |u We|||, +ud deepeu|u o| .o|ce.
I|e|e | + du||, e\p|e|ou|e |+c|e (||. 59),
W||| pu|||ue o| e,e||d. '||u +ppe+| Wo||eu,
coo|, W+\,, d|,, co+|e, +ud p+|e W||| |uc|e+ed
||u c|e+e (||. 59).
|+|r +ud o|e +|e ,e||oWo|+ue due |o c+|o
|euer|+.
I|e |+|| | d|,, co+|e, +ud ||||||e. I||uu|u o|
||e c+|p, |e+|d (||. 59), +ud e\u+| +|e+.
E,e||oW. +|opec|+ o| ||e |+|e|+| oue||||d.
|+|| ||||||e +ud |oW |oW|u.
|+|e, roo||, |ed, +ud c|ur, |ouue.
wo||up |uc|ude ||,|o|d |uuc||ou |e|, ||,|o|d
||ru|+||u |o|roue (I'n), c|u|||+p||c |r+
|u, +ud e|ur c|o|e|e|o| ( ).
\+u+ereu| | |, |ep|+cereu| ||e|+p,.
hF0Ihk0I0ISM AN0 MX0MA
|C|9 . 2++.02++.9
|C|0 . E0!.9
C|+.e d|e+e (C|) | + d|o|de| W||| |||ee r+
jo| r+u||e|+||ou. |,pe|||,|o|d|r W||| d|||ue
o||e|, op|||+|rop+||,, +ud de|rop+||,.
I|e r+u||e|+||ou o||eu do uo| occu| |oe||e|,
r+, uo| occu| +| +||, +ud |uu cou|e ||+| +|e
|udepeudeu| o| e+c| o||e|.
|-c||, (-||c| ,-!-c). E+||, |e|ou.
|||+|e|+|, +,rre|||c, |||r, uoup||||u uodu|e
+ud p|+que ||+| +|e p|u|, ||uco|o|ed, o| pu|p|e
(||. 53C). |+|e |e|ou. cou||ueuce o| e+||,
|e|ou, W||c| ,rre|||c+||, |u.o|.e ||e p|e||||+|
|e|ou +ud r+,, |u e\||ere c+e, |eu|| |u |o
|eque |u.o|.ereu| o| eu|||e |oWe| |e +ud do|+
o| |ee|, roo|| u||+ce W||| o|+ue pee|-|||e +p
pe+|+uce, |+|e| |ecore .e||ucou (||. 53C).
||- . de|rop+||, r+, +|o occu| c||- ||e+|reu|
o| |,pe|||,|o|d|r.
|-. ||ue| |oW +c|op+c|,, W||c| |ep|e
eu| d|+p|,e+| p|o|||e|+||ou o| ||e pe||o|eur
+ud c|u|||u (||. 53 3).
0|||c|c||,. C| op|||+|rop+||, |+ |Wo
corpoueu|, p+||c (|+|e, ||d |+, ||d |e||+c||ou)
+ud rec|+u|c+| p|op|o| (||. 53 1), op|
||+|rop|e|+, coue||.e ocu|op+||,, c|ero|,
coujuc||.|||, pe||o||||+| We|||u, +ud po|eu||+|
corp||c+||ou o| co|ue+| u|ce|+||ou, op||c ueu||||,
op||c +||op|,|. E\op|||+|r|c op|||+|rop|e|+.
ocu|+| ruc|e We+|ue W||| |uW+|d +/e, cou
.e|euce, ||+||ru, d|p|op|+.
|,!. ||||ue |o\|c o||e|, +,rre|||c, |o|u|+|.
A,rre|||c +ud |o|u|+| ||,|o|d eu|+|ereu|,
o||eu W||| ||e p|eeuce o| + ||u||.
\+u+ereu|. |,|:. Au||||,|o|d +eu|
||oc| ||,|o|d |o|roue ,u||e|. A||+||ou o|
||,|o|d ||ue, u||c+||, o| |, |+d|o+c||.e |od|ue.
0|||c|c||,. ',rp|or+||c ||e+|reu| |u r||d
c+e. 'e.e|e c+e. p|edu|oue 00-20 r/d
|u|||+||,, |+pe||u |o 5 r/d. 0||||+| |+d|+||ou.
0||||+| decorp|e|ou. |-c||,. Iop|c+| |u
coco|||co|d p|ep+|+||ou uude| p|+||c occ|u|ou
|o| e.e|+| rou|| +|e uu+||, e||ec||.e. |oW
doe o|+| |ucoco|||co|d (p|edu|oue, 5 r/d).
|u||+|e|ou+| |||+rc|uo|oue !-5 r/r| |o| r+||e|
|e|ou.
CkAvS 0ISAS AN0 hFkIhk0I0ISM
|C|9 . 2+2.0
|C|0 . E05.0
FICk 15-8 Craves dsease . ||op|o|, ||d |e||+c||ou, +ud |e|+u|ec|+|+ +ud |ero|||+e |u ||e |u||+|
coujuuc||.+. 8. I|,|o|d +c|op+c|, (o|eo+||||op+||,) W||| c|u|||u. C. I|e p|u| +ud ||uco|o|ed p+pu|e, uod
u|e +ud p|+que |u ||e p|e||||+| |e|ou +|e c+||ed de|rop+||, (p|e||||+| r,\eder+).
8
C
Add|ou d|e+e | + ,ud|ore |eu|||u ||or
+d|euoco|||c+| |uu|||c|euc,.
|| | |u|d|ou +ud | c|+|+c|e||/ed |, p|o|e
|.e eue|+||/ed ||oWu |,pe|p|reu|+||ou, |oW|,
p|o|e|.e We+|ue, |+||ue, +uo|e\|+, u+ue+,
+ud, ||equeu||,, C| ,rp|or (.or|||u +ud
d|+|||e+).
'ue||.e |+|o|+|o|, c|+ue |uc|ude |oW e|ur
od|ur, ||| e|ur po|+|ur, +ud e|e.+||ou o|
||e ||ood u|e+ u|||oeu. I|e d|+uo| | cou
|||red |, pec|||c |e| o| +d|eu+| |uu|||c|euc,.
'||u. ||e p+||eu| r+, +ppe+| corp|e|e|, uo|r+|
e\cep| |o| + eue|+||/ed ||oWu |,pe|p|reu|+
||ou. () |u +|e+ W|e|e p|reu|+||ou uo|r+||,
occu| e|||e| |+|||u+||, o| u\|uduced. +|ouud
||e e,e, |+ce, do|+ o| |+ud (||. 50), u|p
p|e, |u ||e ||ue+ u||+ (+|doreu), +\|||+e, +ud
+uoeu||+| +|e+ |u r+|e +ud |er+|e (||e
|u|eu||, o| ||e p|reu|+||ou | |e|+|ed |o ||u
p|o|o|,pe), +ud (2) |u ueW +|e+. |u|.+| o|
|ucc+| ruco+, c|e+e o| p+|r (||. 503),
|ou, p|or|ueuce. A|o |u ueW c+| |o||oW|u
u|e|,.
I|e d|||e|eu||+| d|+uo| |uc|ude |eroc||o
r+|o|, po|p|,||+ cu|+ue+ |+|d+, c||ou|c |eu+|
|+||u|e, |ep+||c c||||o|, |eu|u eudoc||ue |u
ro|, uc| + c||orop|o|e +deuor+ ||+| p|o
duce ACIn +ud +oc|+|ed pep||de |.e., |e|ou
,ud|ore, re|+|+||c c+uce| (epec|+||, |uu),
c+|c|uo|d|, .||+r|u B
2
de||c|euc,, c|ero||e|+p,
(do\o|u||c|u, |uu||+u, ||eor,c|u, 5||uo|ou|
+c||), +ud ,|er|c c|e|ode|r+.
A c|eeu|u |e| ued |o| d|+uo| | p|+r+
co|||o| !0-o0 r|u +||e| 250 co,u||op|u |u||+
rucu|+||, o| |u||+.euou|,.
I|| d|e+e |ou|d |e r+u+ed |, +u eudo
c||uo|o||.
A00IS0N 0ISAS |C|9 . 255.+

|C|0 . E21.
FICk 15-9 Myxedema ||,, p+|e ||u, |||uu|u o| ||e |+|e|+| e,e||oW, pu|||ue o| ||e |+ce +ud e,e||d,
|uc|e+ed uur|e| o| ||u c|e+e, du||, e\p|e|ou|e, |e+|d|e |+c|e.
MIA80IIC AN0 NIkIII0NAI C0N0III0NS
Cu|+ueou \+u||or+ +|e ,e||oW||oWu, p|u|||,
o| o|+ue r+cu|e, p+pu|e, p|+que, uodu|e, o|
|u|||||+||ou |u |eudou.
n||o|o|c+||, ||e|e +|e +ccuru|+||ou o| \+u
||or+ ce||-r+c|op|+e cou|+|u|u d|op|e| o|
||p|d.
/+u||or+ r+, |e ,rp|or o| + eue|+| re|+
|o||c d|e+e, + eue|+||/ed ||||oc,|o|, o| + |oc+|
|+| p|+oc,|o|u |o|+e p|oce.
I|e c|+|||c+||ou o| re|+|o||c \+u||or+ | |+ed
ou ||| p||uc|p|e. () \+u||or+ due |o |,pe|||p|
der|+ +ud (2) uo|ro||p|der|c \+u||or+.
I|e c+ue o| \+u||or+ |u ||e |||| |oup r+,
|e + p||r+|, |,pe|||p|der|+, ro||, eue||c+||,
de|e|r|ued (I+||e 5), o| ecoud+|, |,pe|||
p|der|+, +oc|+|ed W||| ce||+|u |u|e|u+| d|e+e
uc| + ||||+|, c||||o|, d|+|e|e re||||u, c||ou|c
|eu+| |+||u|e, +|co|o||r, |,pe|||,|o|d|r, +ud
rouoc|ou+| +rrop+||,, o| W||| |u|+|e o| ce|
|+|u d|u uc| + |e|+||oc|e| +ud e||oeu.
'ore o| ||e \+u||or+ +|e +oc|+|ed W||| |||
p|+r+ |oWdeu||, ||pop|o|e|u (|||)c|o|e|e|o|
|e.e|, +ud ||e|e|o|e W||| + e||ou ||| o| +||e|o
r+|o| +ud r,oc+|d|+| |u|+|c||ou. |o| ||+| |e+ou
|+|o|+|o|, |u.e||+||ou o| p|+r+ ||p|d |e.e| |
+|W+, uece+|,. |u ore c+e +u +pop|o|e|u
de||c|euc, | p|eeu|.
I+||e 52 |oW co||e|+||ou o| c||u|c+| \+u||or+
|,pe +ud ||pop|o|e|u d||u||+uce.
XANIh0MAS |C|9 . 212.2
|C|0 . E13.5
FICk 15-10 Addsoo dsease . n,pe|p|reu|+||ou |ep|eeu||u +u +cceu|u+||ou o| uo|r+| p|reu|+
||ou o| ||e |+ud o| + p+||eu| W||| Add|ou d|e+e. 8. |o|e +cceu|u+|ed p|reu|+||ou |u ||e p+|r+| c|e+e.
8
IA8I 15-1 C|assification of Cenetic hyper|ipidemias
Freder|cksoo 0|ass|I|cat|oo L|p|d ProI||e
Type
| |+r|||+| ||pop|o|e|u ||p+e de||c|euc, IC++, C uo|r+|, C\++, n|| /uo|r+|
(|,pe|c|,|or|c|ouer|+, |,pe|||||,ce||der|+) (|||)
||+ |+r|||+| |,pe|c|o|e|e|o|er|+ (|n) IC uo|r+|, C+, |||+
||| |+r|||+| cor||ued |,pe|||p|der|+ (|Cn|) IC+, C+, |||+, \|||+
||| |+r|||+| d,|e|+||p|der|+ (|eru+u| p+|||c|e d|e+e) (||) IC+, C+, |||+, C\ |eru+u|+
|\ |+r|||+| |,pe|||||,ce||der|+ (|nIC) IC+, C uo|r+|/+, |||++, \|||++
\ |+r|||+| cor||ued |,pe|||||,ce||der|+ (|nI) IC+, C+, \|||++, C\++
|0IE . IC, ||||,ce||de, C, c|o|e|e|o|, C\, c|,|or|c|ou, n||, |||deu||, ||pop|o|e|u, |||, |oWdeu||, ||pop|o|e|u, \|||, .e|, |oWdeu||,
||pop|o|e|u, |||, |u|e|red|+|edeu||, ||pop|o|e|u, +, |+|ed, , |oWe|ed.
IA8I 15-2 C|inica| Presentations of Xanthomas
Type oI Xaothoma 6eoet|c 0|sorders Secoodary 0|sorders
E|up||.e |+r|||+| ||pop|o|e|u ||p+e de||c|euc, (|,pe |) 0|e||,
ApoC2 de||c|euc, (|,pe |) C|o|e|+|
|+r|||+| |,pe|||||,ce||der|+ (|,pe |\) ||+|e|e
|+r|||+| |,pe|||||,ce||der|+ W||| c|,|or|c|ouer|+ (|,pe \) \ed|c+||ou. ke||uo|d, e||oeu
||e|+p,, p|o|e+e |u|||||o|
Iu|e|ou |+r|||+| |,pe|c|o|e|e|o|er|+ (|,pe ||) \ouoc|ou+| +rrop+|||e
|+r|||+| d,|e|+||pop|o|e|uer|+ (|,pe |||)
||,|o|e|o|er|+
Ieud|uou |+r|||+| |,pe|c|o|e|e|o|er|+ (|,pe ||)
|+r|||+| de|ec||.e +poB
|+r|||+| d,|e|+||pop|o|e|uer|+ (|,pe |||)
||,|o|e|o|er|+
Ce|e||o|eud|uou \+u||or+|o|
||+u+|
|+|r+| |+r|||+| d,|e|+||pop|o|e|uer|+ (|,pe |||)
|u|e|||||uou |+r|||+| |oro/,ou |,pe|c|o|e|e|o|er|+ (|,pe ||) C|o|e|+|
||||ue \ouoc|ou+| +rrop+|||e,
c|o|e|+|
/+u||e|+r+ |+r|||+| |,pe|c|o|e|e|o|er|+ (|,pe ||) \ouoc|ou+| +rrop+|||e
|+r|||+| d,|e|+||pop|o|e|uer|+ (|,pe |||)
0||e|
Co|ue+| +|cu |+r|||+| |,pe|c|o|e|e|o|er|+ (|,pe ||)
Iou|||+| I+u|e| d|e+e
+po = +po||pop|o|e|u.
'ou|ce. |E w|||e. /+u||or+ +ud ||pop|o|e|u d|o|de|, |u K wo||| e|. +|. (ed). ||cc|:| |-c||, C--c| !-!:-, 1|| ed. |eW \o||,
\cC|+Wn|||, 2003, |. 212.
\o| corrou o| +|| \+u||or+. |u ro| c+e +u
|o|+|ed ||ud|u uu|e|+|ed |o |,pe|||p|der|+.
0ccu| |u |ud|.|du+| >50 ,e+|, |oWe.e|, W|eu
|u c|||d|eu o| ,ouu +du||, || | +oc|+|ed W|||
|+r|||+| |,pe|c|o|e|e|o|er|+ (|n) o| |+r|||+|
d,|e|+||pop|o|e|uer|+ (||).
'||u |e|ou +|e +,rp|or+||c. 'o||, po|,ou+|
,e||oWo|+ue p+pu|e +ud p|+que |oc+||/ed |o
uppe| +ud |oWe| e,e||d (||. 5) +ud +|ouud
|uue| c+u||u. '|oW eu|+|ereu| ||or ||u, po|
o.e| rou|| |o ,e+|.
C|o|e|e|o| |ou|d |e e||r+|ed |u p|+r+, ||
eu|+uced, c|eeu|u |o| |,pe o| |,pe|||p|der|+
(|n o| ||). || due |o |,pe|||p|der|+, corp||c+||ou
W||| +||e|oc|e|o||c c+|d|o.+cu|+| d|e+e r+,
|e e\pec|ed.
|+e|, e\c||ou, e|ec||ode|cc+||ou, o| |op|c+| +p
p||c+||ou o| |||c||o|o+ce||c +c|d. kecu||euce +|e
uo| uucorrou.
',,. /+u||e|+r+ p+|pe||+|ur, pe||ocu|+|
\+u||or+.
XANIhIASMA |C|9 . !1+.5

|C|0 . n02.o
I|ee u|cu|+ueou |uro| +|e ,e||oW o| ||u
co|o|ed +ud ro.e W||| ||e e\|euo| |eudou
(||. 52).
I|e, +|e + ,rp|or o| |+r|||+| |,pe|c|o|e|e
|o|er|+ (|n) ||+| p|eeu| + |,pe ||+ |,pe|||p|
der|+.
I|| coud|||ou | +u|oor+| |ece|.e W||| + d|||e|
eu| p|euo|,pe |u ||e |e|e|o/,o|e +ud |oro/,
o|e.
|u ||e |oro/,o|e, ||e \+u||or+|+ +ppe+| |u
e+||, c|||d|ood +ud ||e c+|d|o.+cu|+| corp||c+
||ou |u e+||, +do|eceuce, ||e e|e.+||ou o| ||e
||| cou|eu| o| ||e p|+r+ | e\||ere. I|ee
p+||eu| |+|e|, +||+|u +e +|o.e 20 ,e+|.
!cc--|. A d|e| |oW |u c|o|e|e|o| +ud
+|u|+|ed |+|, upp|ereu|ed |, c|o|e|,|+r|ue
o| |+||u. |u e\||ere c+e, re+u|e uc| +
po||+c+.+| |uu| o| ||.e| ||+up|+u|+||ou |+.e |o
|e cou|de|ed.
',,. Ieud|uou \+u||or+.
XANIh0MA IN0INM |C|9 . 212.2
I|| coud|||ou corp||e ,e||oW|| uodu|e (||.
5!) |oc+|ed epec|+||, ou ||e e||oW +ud |uee
|, cou||ueuce o| coucor||+u| e|up||.e \+u||o
r+.
I|e, +|e |o |e |ouud |u p+||eu| W||| ||, |+r||
|+| |,pe|||||,ce||der|+ W||| c|,|or|c|ouer|+
(|,pe \) +ud |n (I+||e 52).
|u |oro/,ou p+||eu| W||| |n, ||e |u|e|ou
\+u||or+ +|e ||+||e| +ud ||u co|o|ed. I|e, +|e
uo| +ccorp+u|ed |, e|up||.e \+u||or+.
!cc--| . I|e+|reu| o| ||e uude||,|u coud|
||ou.
',, . Iu|e|ou \+u||or+.
XANIh0MA I8k0SM |C|9 . !1+.5

|C|0 . E13.2
FICk 15-13 Iuberous xaothoma (Ac|o |+c|u p+e) ||+||opped, ,e||oW, |||r uodu|e W||| +u
e|,||er+|ou r+||u.
FICk 15-11 Xaothe|asma
\u|||p|e c|e+r,o|+ue, |||||, e|e
.+|ed de|r+| p+pu|e ou ||e e,e||d
o| + uo|ro||per|c |ud|.|du+|.
FICk 15-12 Ieodoous
xaothoma |+|e u|cu|+ueou
|uro| +d|e|eu| |o ||e Ac||||e
|eudou.
FICk 15-13
I|ee d|c|e|e |u||+rr+|o|,|,pe p+pu|e 'e|up|
uddeu|, +ud |u |oWe|, +ppe+||u |,p|c+||, ou
||e |u||oc|, e||oW, |oWe| +|r (||. 5+) +ud
|uee.
A |u o| |nI, ||, ||e .e|, |+|e |+r|||+| ||pop|o
|e|u ||p+e de||c|euc, (|||) (I+||e 52), +ud
d|+|e|e ou| o| cou||o|.
|+pu|e +|e dore|+ped, d|c|e|e, |u|||+||, |ed,
||eu ,e||oW ceu|e| W||| |ed |+|o (||. 5+).
|e|ou r+, |e c+||e|ed, d|c|e|e, |u + |oc+||/ed
|e|ou e.., e||oW, |uee (||. 5+), |u||oc||
o| +ppe+| + '|||| c|u|e| ||+| |ecore cou||u
eu| |o |o|r uodu|+| '|u|e|oe|up||.e \+u||or+.
!cc--| . ke+c| .e|, |+.o|+||, |o + |oWc+|
o||e +ud |oW|+| d|e|.
kFIIv XANIh0MA |C|9 . 212.2
|C|0 . E13.2
I|| coud|||ou | c|+|+c|e||/ed |, ,e||oWo|+ue,
||+| o| e|e.+|ed |u|||||+||ou o| ||e .o|+| c|e+e o|
p+|r +ud ||ue| (||. 55).
|+||ouorou|c |o| || (|,pe |||) (I+||e 52).
|e\| |o \+u||or+ |||+|ur p+|r+|e, || +|o
p|eeu| W||| |u|e|ou \+u||or+ (||. 5!) +ud
\+u||e|+r+ p+|pe||+|ur (||. 5).
|+||eu| W||| || +|e p|oue |o +||e|oc|e|o||c
c+|d|o.+cu|+| d|e+e, epec|+||, |c|er|+ o| ||e
|e +ud co|ou+|, .ee|.
!cc--| : |+||eu| W||| || |e+c| .e|, |+.o
|+||, |o + d|e| |oW |u |+| +ud c+||o|,d|+|e. ||
uece+|,, ||| r+, |e upp|ereu|ed W||| |+||u,
||||+|e, o| u|co||u|c +c|d.
XANIh0MA SIkIAIM FAIMAk
FICk 15-14 Fapu|ar eruptve xaothomas . \u|||p|e, d|c|e|e, |ed|o,e||oW p+pu|e |ecor|u cou||u
eu| ou ||e |uee o| +u |ud|.|du+| W||| uucou||o||ed d|+|e|e re||||u, |e|ou We|e p|eeu| ou |o|| e||oW +ud
|u||oc|. 8. n||e| r+u|||c+||ou o| \+u||or+ ou ||e ||uu| o| +uo||e| p+||eu|.
8
FICk 15-15 Xaothoma stratum
pa|mare I|e p+|r+| c|e+e +|e ,e||oW,
o||eu + u|||e |e|ou uo||ce+||e ou|, upou c|oe
e\+r|u+||ou.
/+u||or+ p|+uur | + uo|ro||per|c \+u||or+
||+| cou|| o| d|||ue o|+ue,e||oW p|reu|+
||ou +ud |||| e|e.+||ou o| ||e ||u (||. 5o).
I|e|e | + |ecou|/+||e |o|de|.
I|ee |e|ou c+u |e |d|op+|||c o| ecoud+|, |o
|eu|er|+, |u| ||e ro| corrou +oc|+||ou |
W||| ru|||p|e r,e|or+.
I|e |e|ou r+, p|ecede ||e oue| o| ru|||p|e
r,e|or+ |, r+u, ,e+|.
N0kM0IIFMIC FIAN XANIh0MA
FICk 15-16 F|aoe xaothoma \e||oW||
|ed, |||||, e|e.+|ed p|+que ou ||e uec|, uo||ce
+||e r+|u|, |ec+ue o| ||e +cceu|u+||ou o| ||e
||u |e\|u|e |u + uo|ro||per|c p+||eu| W||| |,r
p|or+. ||+ue \+u||or+ occu| ro| corrou|,
ou ||e uppe| ||uu| +ud uec| +ud +|o occu| |u
|ud|.|du+| W||| r,e|or+.
'cu|., | +u +cu|e o| c||ou|c d|e+e o| |u|+uc,
+ud o| r|dd|e +ud o|d +e c+ued |, d|e|+|,
de||c|euc, o| +co|||c +c|d (.||+r|u C).
nur+u +|e uu+||e |o ,u||e|/e +co|||c +c|d
+ud |equ||e || + +u eeu||+| d|e|+|, .||+r|u.
Io|+||od, poo| o| .||+r|u C .+||e ||or .5-! .
|||| ,rp|or o| dep|e||ou occu| W|eu poo| |/e
| <0.5 . |e||c|euc, o| .||+r|u C |e+d |o |rp+||
reu| o| pep||d,| |,d|o\,|+||ou o| p|oco||+eu,
|educ||ou |u co||+eu |o|r+||ou W||| +oc|+|ed
c+p|||+|, ||+||||,.
'cu|., occu| |u |u|+u| o| c|||d|eu ou + d|e| cou
|||u o| ou|, p|oceed r||| W||| uo +dded c|||u
||u|| o| .ee|+||e + + |eu|| o| p+|eu|+| ue|ec|,
o| |u edeu|u|ou +du|| pe|ou W|o ||.e +|oue,
+ud do uo| e+| +|+d +ud uucoo|ed .ee|+||e.
|-:|c| |c:|. ||eu+uc,, |+c|+||ou, +ud
||,|o|o\|co| W|eu ||e|e +|e |uc|e+ed |equ||e
reu| o| +co|||c +c|d, ro| corrou |u +|co|o|
|r.
w||| uo .||+r|u C |u|+|e, ,rp|or o| cu|.,
occu| +||e| -! rou||. |+||ude, We+|ue,
+||||+||+, +ud r,+||+.
'| |-. |e|ec||+e, |o|||cu|+| |,pe||e|+|o|
W||| pe|||o|||cu|+| |ero|||+e, epec|+||, ou ||e
|oWe| |e (||. 511). n+|| |ecore ||+
reu|ed +ud |u||ed |u ||ee pe|||o|||cu|+| |,pe||
e|+|o||c p+pu|e (co||c|eW |+||), +|o, e\|eu|.e
ecc|,roe (||. 513), W||c| c+u |e eue|+|
|/ed. |+||. p||u|e| |ero|||+e.
C|u|.+. Wo||eu, pu|p|e, pou,, +ud ||eed
e+||,, ||ud|u occu| |u ro|e +d.+uced cu|.,.
|ooeu|u +ud |o o| |ee||.
nero|||+e occu|||u |u|o pe||o|eur o| |ou
|oue +ud |u|o jo|u| c+ue p+|u|u| We|||u
+ud, |u c|||d|eu, ep|p|,e+| ep+|+||ou. '|e|uur
r+, |u| |uW+|d. co||u||c |o+|, (e|e.+||ou +| |||
r+||u).
ke||o|u||+|, u|+|+c|uo|d, |u||+ce|e||+| |ero|
||+e c+u c+ue de+||.
|||--|c| !c. |uc|ude |||or|oc,|ope
u|+, eu||e pu|pu|+, co+u|op+||,, +u||co+u|+u|
d|u ||e|+p, (W+||+||u, |ep+||u), c|,o|o|u||ue
r|+, .+cu|||| +ud |u|.+| |,pe|||op|, due
|o poo| deu|+| |,|eue, d|u|uduced |u|.+|
|,pe|p|+|+, |eu|er|+, p|eu+uc,.
|c|c|, . |o|roc,||c, uo|roc||or|c +uer|+.
|o|+|e de||c|euc,, |eu|||u |u r+c|oc,||c +uer|+.
|o|||.e c+p|||+|, ||+||||, |e|. ||+|e|e| +co|||c +c|d
|e.e| uu+||, <25 o| uo|r+| .+|ue, e|ur +co|
||c +c|d |e.e| /e|o. /|+, ||ud|u +|e d|+uo||c.
uu|e ||e+|ed, cu|., | |+|+|. 0u ||e+|reu|,
pou|+ueou ||eed|u ce+e W||||u 2+ |, ruc|e
+ud |oue p+|u |+de qu|c||,, ||eed|u ||or ur
|op |u 2-! d+,.
!cc--| . Aco|||c +c|d 00 r !-5 ||re
d+||, uu||| + | |.eu, ||eu 00 r/d | cu|+||.e
|u d+, |o Wee|.
SCkv |C|9 . 2o1

|C|0 . E5+
FICk 15-1T Scurvy . |e|||o|||cu|+| pu|pu|+ ou ||e |e. I|e |o|||c|e +|e o||eu p|ued |, |e|+||u (pe|||o|
||cu|+| |,pe||e|+|o|). I|| e|up||ou occu||ed |u + +o,e+|o|d +|co|o||c, |ore|e r+|e, W|o +|o |+d ||eed|u
ur +ud |ooe |ee||. 8. I|ee e\|eu|.e ecc|,roe occu||ed |u +u edeu|u|ou o5,e+|o|d r+|e W|o ||.ed
+|oue +ud W|oe |ood |u|+|e cou||ed r+|u|, o| ||cu|| o+|ed |u W+|e|.
8
Ac|ode|r+|||| eu|e|op+|||c+ (AE) | + eue||c
d|o|de| o| /|uc +|o|p||ou, p|eeu||u |u |u|+uc,,
c|+|+c|e||/ed |, + |||+d o| +c|+| de|r+|||| (|+ce,
|+ud, |ee|, +uoeu||+| +|e+), +|opec|+, +ud d|
+|||e+. (||. 53)
|e+||, |deu||c+| c||u|c+| ||ud|u occu| |u o|de| |u
d|.|du+| W||| +cqu||ed /|uc de||c|euc, (A/|) due
e|||e| |o d|e|+|, de||c|euc, o| |+||u|e o| |u|e||u+|
+|o|p||ou (||. 59).
1| occu| |u |u|+u| |o|||e|ed W||| |o.|ue r|||,
d+, |o |eW Wee|. |u ||e+||ed |u|+u|, oou +||e|
We+u|u. 1| |u o|de| |ud|.|du+|.
E||o|o, . 1|. +u|oor+| |ece|.e ||+|| |eu|||u
|u |+||u|e |o +|o|| /|uc. 1| . ecoud+|, |o
|educed d|e|+|, |u|+|e o| /|uc, r+|+|o|p||ou
(|e|ou+| eu|e||||, +||e| |u|e||u+| |,p+ u|e|,
|o| o|e||,), c||ou|c +|co|o||r, |uc|e+ed u||u+|,
|o (uep||o||c ,ud|ore), |,po+||ur|uer|c
|+|e, peu|c|||+r|ue ||e|+p,, ||| c+|+|o||c |+|e
(||+ur+, |u|u, u|e|,), |ero|,||c +uer|+,
+do|eceu| W|o e+| d|||, p|o|oued p+|eu|e|+|
uu|||||ou W|||ou| upp|ereu|+| /|uc.
|c||--. |u AE, p+||eu| do uo| +|o||
euou| /|uc ||or ||e d|e|. I|e pec|||c ||+ud
|u.o|.ed |u |+|c ||+upo|| rec|+u|r |o| /|uc
||+| r||| |e +|uo|r+| |u AE | uo| |uoWu. I|e
de|ec| +ppe+| |o |e oreW|e|e |u ||e e+||,
|+e o| /|uc uu||||u|e, W|e|e /|uc | p|eeu|ed
|o ||e |u|e||u+| ||u| |o|de|. I|| de|ec| c+u |e
o.e|core |, |uc|e+ed /|uc upp|, |u ||e d|e|. ||
| uo| |uoWu |oW /|uc de||c|euc, |e+d |o ||u +ud
o||e| |e|ou.
'| |! . |+|c|e +ud p|+que o| d|,, c+|,,
|+|p|, r+||u+|ed +ud ||||||, |ed, ec/er+|ou
de|r+|||| e.o|.|u |u|o .e|cu|o|u||ou, pu|u|+|,
e|o|.e, +ud c|u|ed |e|ou (||. 53, +ud 5
9). |u|||+||, occu| |u ||e pe||o|+| +ud +uoeu||+|
+|e+. |+|e|, c+|p, |+ud +ud |ee|, ||e\u|+| |e|ou,
||uu|. ||ue|||p |||eu|u, e|,||er+|ou, W|||
||u|e +ud ecoud+|, p+|ou,c||+. |e||ec|e. |e
|ou |ecore ecoud+|||, |u|ec|ed W||| Cc!!c
c||:c , ' c- . |rp+||ed Wouud |e+||u.
|c c! c| ||||ue +|opec|+, |+,|u o| |+||.
|+|ou,c||+, u+|| ||d|u, |o o| u+||.
!: -|c- ked, |o, |ouue, u
pe|||c|+| +p|||ou|||e e|o|ou, ecoud+|, o|+|
c+ud|d|+|.
C--c| -cc|. ||o|op|o||+, |||||+||e, de
p|eed rood. C|||d|eu W||| AE W||ue +ud c|,
cou|+u||,. |+||u|e o| |oW||.
|c|c|, . Auer|+, |oW e|ur/p|+r+ /|uc |e.
e|, |educed u||u+|, /|uc e\c|e||ou.
|-c|c|||, |o||+||o|r de|r+||||
W||| |+|e, p+|e |e|+||uoc,|e |u ||e uppe|
ep|de|r|, p|or|ueu| p+|+|e|+|o|. I|e|e r+,
|e |u||+ep|de|r+| c|e|| W||| +c+u||o|,| +ud
||||e|. 'p+|e, upe|||c|+|, pe||.+cu|+| |,r
p|o||||oc,||c |u|||||+|e +ud |o||uou c+p|||+||e
|u ||e p+p|||+|, de|r|.
C- c! . A||e| /|uc |ep|+cereu|,
e.e|e|, |u|ec|ed +ud e|o|.e ||u |e|ou |e+|
W||||u -2 Wee| (||. 533), d|+|||e+ ce+e,
+ud |||||+|||||, +ud dep|e|ou o| rood |rp|o.e
W||||u 2+ |.
!cc--| . ||e|+|, o| |\ upp|ereu|+||ou
W||| /|uc +|| |u 2 |o ! ||re ||e |equ||ed d+||,
+rouu| |e|o|e uo|r+| /|uc |+|u |u d+, |o
Wee|.
IINC 0FICINC AN0 ACk00kMAIIIIS NIk0FAIhICA |C|9 . 2o9.9
|C|0 . Eo0
FICk 15-18 Acrodermatts eoteropathca . '|+|p|, der+|c+|ed, ,rre|||c, p+|||+||, e|o|.e, c+|,,
+ud c|u|ed p|+que ou ||e |+ce o| +u |u|+u| +||e| We+u|u. '|r||+| |e|ou We|e +|o |ouud |u ||e pe||eu||+| +ud
pe||+u+| |e|ou +ud ou ||e ||ue|||p. I|e c|||d W+ ||||, |||||+||e, W||u|u, +ud c|,|u +ud |+d d|+|||e+.
8. w||||u 2+ | +||e| /|uc |ep|+cereu|, ||e |||||+|||||, +ud d|+|||e+ ce+ed +ud ||e |u|+u|' rood |rp|o.ed, +ud
+||e| 0 d+, (|oWu |e|e) ||e pe||o|+| +ud pe||eu||+| |e|ou |+d |e+|ed.
8

FICk 15-19 Ioc deIceocy we||der+|c+|ed, po||+||o|r +ud ec/er+|ou-|||e p|+que W||| c+||u
+ud e|o|ou o.e||,|u ||e +c|ur, |u|e||u|e+| c|e||, |u||oc|, +ud ||p |u + o0,e+|o|d +|co|o||c |er+|e W|oe
d|e| |+d cou||ed o| p|c||e +ud c|e+p W|ue. '|e +|o |+d + |r||+| e|up||ou +|ouud ||e rou||, pe||ec|e,
+||op||c |o|||, +ud |+d |||eu|u, ||u,, oo/|u ||ue|||p.
|e||+|+ | |e|+|ed |o u|+c|u de||c|euc,.
||+c|u+r|de | +u |rpo||+u| cou|||ueu| o| coeu
/,re | (|A|) +ud coeu/,re || (|A||), W||c|
|uuc||ou |u o\|d+||ou|educ||ou |e+c||ou + +
|,d|oeu |ou douo| +ud +ccep|o|, |epec||.e|,.
I|e eeu||+| +r|uo +c|d ||,p|op|+u | cou.e||ed
|u ||e |od, |o u|+c|u.
|e||+|+ r+, +||e ||or + d|e| de||c|eu| |u u|+c|u
o| ||,p|op|+u, o| |o||. A p|edor|u+u||, r+|/e
|+ed d|e| | uu+||, |rp||c+|ed, |u| ou|, W|eu
||e r+|/e | |e+red o| coo|ed.
|e||+|+ | c|+|+c|e||/ed |, ||e ! |. ! e|r+||||,
! |+|||e+, +ud ! ereu||+. '||u c|+ue +|e de|e|
r|ued |, e\pou|e |o uu|||| +ud p|eu|e.
I|e d|o|de| |e|u W||| + ,rre|||c ||c||u
+ud r+|||u e|,||er+ ou ||e do|+ o| ||e
|+ud, uec|, +ud |+ce. \e|c|e +ud |u||+e r+,
e|up| +ud ||e+|, o ||+| c|u||u occu| +ud
|e|ou |ecore c+|, (||. 5201). |+|e|, ||u
|ecore |udu|+|ed, ||c|eu|||ed, |ou|, co.e|ed
|, d+|| c+|e +ud c|u|, ||e|e +|e c|+c| +ud
||u|e +ud + |+|p der+|c+||ou ||or uo|r+|
||u (||. 520 3 ).
I|e d|||||u||ou | |||||u. do|+ o| |+ud +ud
||ue| ('+uu||e| o| pe||+|+) (||. 5203),
|+ud|||e +|ouud ||e uec| ('C++| uec||+ce) (||.
5-201), do|+ o| |ee| up |o r+||eo|| W||| p+||u
o| ||e |ee|, +ud |u||e|||, |e|ou o| ||e |+ce.
||+uo| | .e||||ed |, de|ec||ou o| dec|e+ed
|e.e| o| u||u+|, re|+|o|||e.
!cc--| . 0|+| +dr|u|||+||ou o| 00-!00
r u|+c|u+r|de p|u o||e| .||+r|u o| ||e B
corp|e\ |e+d |o corp|e|e |eo|u||ou.
FIIACkA |C|9 . 2o5.2
|C|0 . E52
FICk 15-20 Fe||ara . 'c+|, c|u|ed |+ud|||e p|+que ou ||e uec| ('C++| uec||+ce).
FICk 15-20 8. 'C+uu||e| o| pe||+|+, |udu|+|ed, ||c|eu|||ed, p|reu|ed, +ud c+|, ||u ou ||e do|+ o| ||e
|+ud
A c||u|c+| ,ud|ore occu|||u |u + |oup o| d|
e+e c|+|+c|e||/ed |, ||e depo|||ou o| rouoo
d|ur u|+|e c|,|+| |u ,uo.|+| ||u|d +ud jo|u|.
Acu|e ou|, +||||||| uu+||, occu| |u r|dd|e
+e +ud uu+||, +||ec| + |u|e jo|u| |u ||e |oWe|
e\||er|||e, uu+||, ||e |||| re|+|+|op|+|+u|+|
jo|u|. C+u +|o +||ec| ||ue| (||. 521).
|u|e|c||||c+| ou| dec|||e ||e |u|e|.+| |e|Weeu
+||+c| o| ou|. w||| ||re +||+c| |eud |o |e po|
,+|||cu|+|.
|u c||ou|c |op|+ceou ou| p+||eu| |+|e|, |+.e
+,rp|or+||c pe||od. u|+|e c|,|+| +|e |ouud |u
o|| ||ue, c+||||+e (||. 523), +ud |eudou.
Cou| r+, occu| W||| +ud W|||ou| |,pe|u||cer|+,
|eu+| d|e+e, +ud uep||o|||||+|.
C0I |C|9 . 21+

|C|0 . \0
FICk 15-21 . Acute outy arthrts +||ec||u ||e d||+| |u|e|p|+|+ue+| jo|u| o| ||e ||||| d|||. 8. Cou|,
|op|| ou |e||\.
8

CNIIC 0ISASS
|eudo\+u||or+ e|+||cur (|/E) | + e||ou |e
|ed||+|, d|o|de| o| couuec||.e ||ue ||+| |u.o|.e
||e e|+||c ||ue |u ||e ||u, ||ood .ee|, +ud
e,e.
|:!-:-. .+0,000 |o .00,000. ||-|c:-.
Au|oor+| |ece|.e (ro| corrou) +ud +u|o
or+| dor|u+u|.
|||, c! |c||--. |+||oeu|c ru|+||ou
|u ||e 13CC3 eue, W||c| eucode \k|o, +
rer|e| o| ||e AI|+edepeudeu| ||+urer
||+ue ||+upo||e| |+r||, o| p|o|e|u. \k|o c+u
e|.e + +u e|||u\ purp ||+upo|||u r+||ro
|ecu|+|We||| |u|+|||oue couju+|e, W||c| r+,
|+c||||+|e c+|c|||c+||ou o| e|+||c |||e|. I|| r+,
|eu|| |u ||+reu|ed e|+||c |||e| |u ||u, e,e,
+||e||e.
I|e p||uc|p+| ||u r+u||e|+||ou +|e + d|||uc||.e
-c ! c- u||+ce p+||e|u |eu|||u ||or
c|oe|, |ouped c|u|e| o| ,e||oW (c|+ro|
co|o|ed) p+pu|e |u + |e||cu|+| p+||e|u ou ||e
uec|, +\|||+e, +ud o||e| |od, |o|d (||. 522).
I|e e||ec| ou ||e .+cu|+| ,|er |uc|ude C|
|ero|||+e, |,pe||eu|ou occu|||u |u ,ouu
pe|ou +ud |eu|||u ||or |u.o|.ereu| o| |eu+|
+||e||e, +ud c|+ud|c+||ou.
0cu|+| r+u||e|+||ou ('+u|o|d ||e+| +ud |e||
u+| |ero|||+e) c+u |e+d |o |||udue.
|-c|c|||, . B|op, o| + c+| c+u de|ec|
c|+|+c|e||||c c|+ue o| |/E |-|- |,:c| |
:|c- c- cc-| . 'We|||u +ud |||eu|+|
c|urp|u +ud |+op||||c |+|u|u o| e|+||c |||e|
|u |e||cu|+| de|r|, W||| .ou Ko+ |+|u, e|+||c
|||e| +ppe+| cu||ed +ud 'c|opped up W||| c+|
c|ur depo|||ou.
|c. /|+,. e\|eu|.e c+|c|||c+||ou o| ||e
pe||p|e|+| +||e||e o| ||e |oWe| e\||er|||e. A||e||
o|+p|, o| ,rp|or+||c .ee|.
I|e cou|e | |ue\o|+||, p|o|e|.e. C+|||c
+||e|, |ero|||+e occu| corrou|,, |eu|||u |u
|er+|ere|. |e||p|e|+| .+cu|+| d|e+e p|eeu|
+ p|er+|u|e ce|e||o.+cu|+| +cc|deu|, +||e|o
c|e|o| o||||e|+u, o| |oWe| +u|u+. ||eu+uc|e
+|e corp||c+|ed |, r|c+|||+e, c+|d|o.+cu|+|
corp||c+||ou. B||udue. |||e p+u | o||eu |o||
eued due |o r,oc+|d|+| |u|+|c||ou o| r+|.e C|
|ero|||+e.
!cc--| . Ceue||c couue||u. E.+|u+|e |+r
||, rer|e| |o| |/E. 0||e|||c|+u |ou|d |e
+W+|e o| |/E d|+uo| +ud |o||oW p+||eu| c+|e
|u||,. keu|+| |ee.+|u+||ou |, p||r+|, c+|e p|,|
c|+u +ud op|||+|ro|o|| | r+ud+|o|,.
'| ccc|. |/E |u|e|u+||ou+|, +++
-
FS00XANIh0MA IASIICM |C|9 . 151.!9

|C|0 . 032.3
FI0MI0I0C
Iocdeoce In mental institutions, 1:100
to 1:300; in geneial population, 1:20,000 to
1:100,000.
Ae oI 0oset Infancy.
Sex Equal incidence.
Iu|e|ou c|e|o| | +u +u|oor+| dor|u+u|
d|e+e +|||u ||or + eue||c+||, p|o|+rred
|,pe|p|+|+ o| ec|ode|r+| +ud reode|r+| ce||
+ud r+u||e|ed |, + .+||e|, o| |e|ou |u ||e ||u,
C|' (|+r+||or+), |e+||, ||due,, +ud o||e|
o|+u.
I|e p||uc|p+| e+||, r+u||e|+||ou +|e ||e |||+d o|
e|/u|e, reu|+| |e|+|d+||ou, +ud coueu||+| W|||e
po|.
|+c|+| +u|o||||or+|+ +|e p+||ouorou|c |u| do
uo| +ppe+| uu||| ||e ||||d o| |ou||| ,e+|.
I8k0S SCIk0SIS (IS) |C|9 . 159.5
|C|0 . 035.
kace All iaces.
heredty Autosomal dominant. TS is caused
by mutations in a tumoi-suppiessoi gene, ei-
thei TSCS1 oi TSCS2 . TSCS1 maps to chiomo-
some 9q34. TSCS2 maps to 16p13.3.
FICk 15-22 Fseudoxaothoma e|astcum \u|||p|e, cou||ueu|, c|+ro|co|o|ed o| ,e||oW p+pu|e (peu
do\+u||or+|ou) c|e+|ed + |+|e, c||cur|e|eu||+|, pe|||ed p|+que ou ||e uec| o| + !2,e+|o|d Wor+u. C|+ue
|u ||e couuec||.e ||ue |u ||| coud|||ou |ed |o e\ce|.e |o|d ou ||e |+|e|+| uec|.
FAIh0CNSIS
Genetic alteiations of ectodeimal and mesodei-
mal cells with hypeiplasia, with a distuibance in
embiyonic cellulai diffeientiation.
White macules aie piesent at biith oi appeai
in infancy (>80% occui by 1 yeai of age, 100%
appeai by 2 yeais); >20% of angiofibiomata aie
piesent at 1 yeai of age, 50% occui by 3 yeais.
Seizuies (infantile spasms) occui in 86%; the
eailiei the onset of seizuies, the woise the men-
tal ietaidation. Mental ietaidation (49%).
Sko Iesoos 96% incidence.
Hypvme|unvtIc Mucu|es Off-white"; one oi
many, usually moie than thiee. Polygonal oi
thumbpiint," 0.5-2 cm; lance ovate oi ash-
leaf " spots (Fig. 15-23), 3-4 cm (up to 12
cm); tiny white confetti" macules, 1-2 mm
(Fig. 15-24). White macules occui on tiunk
(56%), lowei extiemities (32%), uppei extiemi-
ties (7%), head and neck (5%). White macules
shine up with Wood light (Fig. 15-23B)
AngIv]Ihrvmus 0.1-0.5 cm, dome-shaped and
smooth, exhibiting ied oi skin coloi (Fig. 15-
25). Occui in the centei of the face. They aie
fiim and disseminated but may coalesce; teimed
aJenoma se|ateum but iepiesent angiofibiomas
(piesent in 70%).
P|uques Repiesent connective tissue nevi
(shagieen" patch), piesent in 40%; skin coloied;
occui on the back and buttocks (Fig. 15-26B).
PerIunguu| Pupu|es vr Nvdu|es Ungual fibio-
mas (Koenen tumois) piesent in 22%, aiise late in
childhood, and have the same pathology (angiofi-
bioma) as the facial papules. (Fig. 15-26).
ASS0CIAI0 SSIMS
CNS (tumois pioducing seizuies), eye (giay oi
yellow ietinal plaques, 50%), heait (benign ihab-
domyomas), hamaitomas of mixed cell type (kid-
ney, livei, thyioid, testes, and GI system).
0ermatopatho|oy WhIte Mucu|es De-
cieased numbei of melanocytes, decieased
melanosome size, decieased melanin in melano-
cytes and keiatinocytes.
AngIv]Ihrvmutu Piolifeiation of fibioblasts,
incieased collagen, angioneogenesis, capillaiy
dilatation, absence of elastic tissue.
8rao Fatho|oy Tubeis" aie gliomas.
Imao S|u|| X-Ruy Multiple calcific densities.
CT Scun Ventiiculai defoimity and tumoi
deposits along the stiiothalamic boideis.
MR1 Subependymal nodules.
E|ectrvencephu|vgruphy Abnoimal.
Renu| U|trusvund Reveals ienal hamaitoma.
FICk 15-23 Iuberous sc|eross: ash-|eaI|et hypopmeoted macu|es . I||ee We||der+|c+|ed,
e|ou+|ed (+||e+||e| |+ped), |,pore|+uo||c r+cu|e ou ||e |oWe| |e o| + c|||d W||| |+u ||u. 8. A||e+||e|
|,pore|+uo||c r+cu|e |u p+|e ||u +|e |e||e| .|u+||/ed uude| wood |||| W|e|e ||e, |||| up.
8
FICk 15-24 Iuberous sc|eross:
"cooIett" macu|es \u|||p|e, d|c|e|e,
r+||, cou|e||||||e, |,pop|reu|ed r+cu|e
o| .+||+||e |/e ou ||e |e. I|ee |e|ou +|e
p+||ouorou|c.
FICk 15-25 Iuberous sc|eross: aooIbromas Cou||ueu|, r+||, +u|or+|ou (e|,||er+|ou, |||eu
|u) p+pu|e ou ||e c|ee| +ud uoe. I|ee |e|ou We|e uo| p|eeu| du||u ||e |||| |eW ,e+| o| |||e, +ppe+|ed
ou|, +||e| ||e +e o| + ,e+|.
0IACN0SIS
The diagnosis may be difficult oi impossible in
an infant oi child if one oi two white macules
aie the only cutaneous finding. Moie than five is
highly suggestive. Even when typical white ash-
leaf " oi thumbpiint" macules (Fig. 15-23) aie
piesent, it is necessaiy to confiim the diagnosis.
Confetti spots (Fig. 15-24) aie viitually pathog-
nomonic. A pediatiic neuiologist can then
evaluate the patient with a study of the family
membeis and by obtaining vaiious types of im-
aging as well as electioencephalogiaphy. Mental
ietaidation and seizuies may be absent.
Whte Spots Focal vitiligo, nevus anemicus,
tinea veisicoloi, nevus depigmentosus, postin-
flammatoiy hypomelanosis.
AooIbromas Tiicholemmoma, syiingoma,
skin-coloied papules on the face, deimal nevi.
Noe : angiofibiomata of the face (Fig. 15-25)
have been mistaken foi and tieated as acne
vulgaiis oi iosacea.
Feruoua| Fbromas Veiiuca vulgaiis.
C0kS AN0 Fk0CN0SIS
A seiious autosomal disoidei that causes majoi
pioblems in behavioi, because of mental ietai-
dation, and in theiapy, to contiol the seiious
seizuie pioblem piesent in many patients.
In seveie cases, 30% die befoie the fifth yeai
of life, and 50-75% die befoie ieaching adult
age. Malignant gliomas aie not uncommon.
Genetic counseling is impeiative.
MANACMNI
Freveotoo Counseling.
Ireatmeot Lasei suigeiy foi angiofibiomas.
Support oraotatoo: |.//www.suor-
grou.tom
FICk 15-26 Iuberous sc|eross . |e||uuu+| ||||or+ (Koeueu |uro|). 8. '|+|eeu p+|c|, |||||,
e|e.+|ed, ||uco|o|ed. I|| |ep|eeu| + couuec||.e ||ue ue.u.
8
FI0MI0I0C
Iocdeoce NF1 : 1:4000; NF2 : 1:50,000.
kace All iaces.
Sex Males slightly moie than females.
heredty Autosomal dominant; the gene foi
NF1 is on chiomosome 17 (q 1.2) and the gene
codes foi a piotein named neuiofibiomin. The
gene foi NF2 is on chiomosome 22 and codes
foi a piotein called meilin.
FAIh0CNSIS
Action of an abnoimal gene on cellulai ele-
ments deiived fiom the neuial ciest: melano-
cytes, Schwann cells, endoneuiial fibioblasts.
Caf-au-lait (CAL) macules aie not usually
piesent at biith but appeai duiing the fiist
3 yeais; neuiofibiomata appeai duiing late
adolescence. Clinical manifestations in vaiious
oigans aie ielated to pathology: hypeitensive
headaches (pheochiomocytomas), pathologic
fiactuies (bone cysts), mental ietaidation, biain
tumoi (astiocytoma), shoit statuie, piecocious
pubeity (eaily menses, clitoial hypeitiophy).
Sko Iesoos CAL Mucu|es Light oi daik
biown un[orm melanin pigmentation with shaip
maigination. Lesions vaiy in size fiom multiple
fieckle-like" tiny macules <2 mm (Fig. 15-27), to
veiy laige biown macules >20 cm (Fig. 15-28). The
common size, howevei, is 2-5 cm. CAL macules
also vaiy in numbei, fiom a few to hundieds.
Tiny fieckle-like lesions in the axillae aie highly
chaiacteiistic (axillaiy fieckling") (Fig. 15-27).
Pupu|es/Nvdu|es (Neurv]Ihrvmus) Skin-
coloied, pink, oi biown (Fig. 15-28); flat,
dome-shaped oi pedunculated (Fig. 15-29);
|| | +u +u|oor+| dor|u+u| ||+|| r+u||e|ed
|, c|+ue |u ||e ||u, ue|.ou ,|er, |oue,
+ud eudoc||ue |+ud. I|ee c|+ue |uc|ude +
.+||e|, o| coueu||+| +|uo|r+||||e, |uro|, +ud
|+r+||or+.
IWo r+jo| |o|r o| || +|e |ecou|/ed. () c|+|c
.ou kec|||u|+ueu. ||, |e|red ||! , +ud (2)
ceu||+|, o| +cou||c ||, |e|red ||2 .
Bo|| |,pe |+.e c+|e+u|+|| r+cu|e +ud ueu
|o||||or+, |u| ou|, ||2 |+ ||c|-c| +cou||c
ueu|or+ (uu||+|e|+| +cou||c ueu|or+ +|e +
.+||+||e |e+|u|e o| ||).
Au |rpo||+u| d|+uo||c |u p|eeu| ou|, |u ||
| p|reu|ed |+r+||or+ o| ||e ||| (||c| uod
u|e).
',,. .ou kec|||u|+ueu d|e+e.
Nk0FI8k0MAI0SIS (NF) |C|9 . 2!1.1

|C|0 . 035.0
soft oi fiim, sometimes tendei; buttonhole
sign"-invagination with the tip of the index
fingei is pathognomonic.
P|erI]vrm Neurvmus Diooping, soft, doughy
(Fig. 15-30); may be massive, involving entiie
extiemity, the head, oi a poition of the tiunk.
DIstrIhutIvn Randomly distiibuted (Figs.
15-28 and 15-29) but may be localized to one
iegion (segmental NF1). The segmental type
may be heiitable oi a localized hamaitoma.
0ther Fhysca| Fodos Eyes Pigmented
hamaitomas of the iiis (Lisch nodules) be-
gin to appeai at age 5 and aie piesent in 20%
of childien with NF befoie age 6 but can be
found in 95% of patients with NF1 in ado-
lescence. They aie not piesent in NF2. Lisch
nodules aie visible only with slit-lamp ex-
amination and appeai as glassy," tianspaient,
dome-shaped, yellow-to-biown papules up to
2 mm. They do not coiielate with the seveiity
of the disease.
Muscu|vs|e|etu| Ceivicothoiacic kyphosco-
liosis, segmental hypeitiophy.
Adrenu| Phevchrvmvcytvmu Elevated blood
piessuie and episodic flushing.
PerIpheru| Nervvus System Elephantiasis
neuiomatosa (gioss disfiguiement fiom neu-
iofibiomatosis of the neive tiunks).
Centru| Nervvus System Optic glioma,
acoustic neuioma (iaie in NF1 and unilateial,
but bilateial in NF2), astiocytoma, meningi-
oma, neuiofibioma.
0ermatopatho|oy Moie than 10 me|ann
matrog|o|u|es pei 5 high-powei fields in split"
dopa piepaiations.
Wood Iamp xamoatoo In white peisons
with pale skin, the CAL macules aie moie easily
visualized with Wood lamp examination.
Two of the following ciiteiia:
1. Multiple CAL macules-moie than six le-
sions with a diametei of 1.5 cm in adults
and moie than five lesions with a diametei
of 0.5 cm oi moie in childien youngei than
5 yeais.
2. Multiple fieckles in the axillaiy and in-
guinal iegions.
3. Based on clinical and histologic giounds,
two oi moie neuiofibiomas of any type, oi
one plexifoim neuiofibioma.
4. Sphenoid wing dysplasia oi congenital
bowing oi thinning of long bone coitex,
with oi without pseudoaithiosis
5. Bilateial optic neive gliomas
6. Two oi moie Lisch nodules on slit-lamp
examination
7. Fiist-degiee ielative (paient, sibling, oi
child) with NF1 by the pieceding ciiteiia
0IIereota| 0aooss Biown CAL-type mac-
ules: Albiight syndiome (polyostotic fibioma,
dysplasia, and piecocious pubeity); a few CAL
macules (thiee oi less) may be piesent in
10-20% of noimal population.
C0kS AN0 Fk0CN0SIS
It is impoitant to establish the diagnosis in
oidei to do genetic counseling and to follow
patients foi development of malignancy. Also,
neuiofibiomatosis suppoit gioups help with
social adjustment in seveiely affected peisons.
Theie is vaiiable involvement of the oi-
gans affected ovei time, fiom only a few pig-
mented macules to maiked disfiguiement with
thousands of nodules, segmental hypeitiophy,
and plexifoim neuiomas. The moitality iate is
highei than in the noimal population, piinci-
pally because of the development of neuiofi-
biosaicoma duiing adult life. Othei seiious
complications aie ielatively infiequent.
FICk 15-2T NeuroIbromatoss (NF1) 'e.e|+| |+|e| (> cr) c+|e+u|+|| r+cu|e ou ||e uppe| c|e|
+ud ru|||p|e r+|| r+cu|e ou ||e +\|||+e (+\|||+|, '||ec|||u) |u + ||oWu||uued |er+|e. \,||+d o| e+||,, r+||,
p|u||+u ueu|o||||or+ ou ||e c|e|, ||e+|, +ud uec|.
MANACMNI
An oithopedic physician should manage the two
majoi bone pioblems: kyphoscoliosis and tibial
bowing. A plastic suigeon can do ieconstiuctive
suigeiy on the facial asymmetiy. The language
disoideis and leaining disabilities should be
evaluated by a psychologist. Close follow-up
annually should be mandatoiy to detect saico-
mas that may aiise within plexifoim neuiomas.
Suigical iemoval of pheochiomocytoma.
Support Croup: |.//www.suor-grou.
tom
FICk 15-28 NeuroIbromatoss (NF1) '||uco|o|ed +ud p|u||+u, o|| p+pu|e +ud uodu|e ou ||e |+c|
+|e ueu|o||||or+. I|e |e|ou |||| +ppe+|ed du||u |+|e c|||d|ood. IWo |+|e c+|e+u|+|| r+cu|e ou ||e |+c|.
I|e |+|e, o||, |||de||ued, u|cu|+ueou uodu|e ou ||e |||| |oWe| |+c| +ud ou ||e |||| po|e||o| +\|||+|, ||ue +|e
p|e\||o|r ueu|or+.
FICk 15-29 NeuroIbromatoss (NF1) Au e\ce|.e|, |+|e uur|e| o| r+|| +ud |+|e, peduucu|+|ed
ueu|o||||or+ ou ||e c|e| o| + 5o,e+|o|d Wor+u W|o +|o |+d + e.e|e|, d||o||ed |+ce due |o ru|||p|e ueu
|o||||or+ +ud p|e\||o|r ueu|or+.
FICk 15-30 NeuroIbromatoss (NF1) ||e\||o|r ueu|or+ ou ||e o|e o| ||e |oo| o| + c|||d. I|| |||de||ued
u|cu|+ueou r+ | o|| +ud +,rp|or+||c. I|e p+||eu| |+ c+|e+u|+|| r+cu|e +ud ru|||p|e ueu|o||||or+.
ne|ed||+|, |ero|||+|c |e|+u|ec|+|+ | +u +u
|oor+| dor|u+u| coud|||ou +||ec||u ||ood .e
e|, epec|+||, |u ||e rucou rer||+ue o| ||e
rou|| +ud ||e C| ||+c|.
I|e d|e+e | ||equeu||, |e|+|ded |, |ecu||eu|
ep||+\| ||+| +ppe+| o||eu |u c|||d|ood.
I|e d|+uo||c |e|ou +|e r+||, pu|+||u, r+cu
|+| +ud p+pu|+|, uu+||, puuc|+|e, |e|+u|ec|+e
(||. 5!) ou ||e ||p, |ouue, |+ce, p+|r/o|e,
||ue|/|oe, u+|| |ed, |ouue, coujuuc||.+e, u+
op|+|,u\, +ud |||ou|ou| ||e C| +ud eu||ou||
u+|, ||+c|. |u ||e 3,e+|o|d r+|e, |oWu |u ||.
5!1, ||e|e |+d |eeu |epe+|ed ep||+\|, |u|
||e |e|+u|ec|+|+ |+d oue uuuo||ced uu||| ||e
p+||eu| W+ e.+|u+|ed |o| +uer|+. C+|e|u| |||o|,
||eu |e.e+|ed ||+| ||e p+||eu|' |+||e| |+d + r|uo|
|o|r o| ||e +re coud|||ou.
|u|rou+|, +||e||o.euou |||u|+ r+, occu|.
C||ou|c ||ood |o |eu|| |u +uer|+.
E|ec||oc+u|e|, +ud pu|e d,e |+e| +|e ued |o de
||o, cu|+ueou +ud +cce|||e ruco+| |e|ou.
E||oeu |+.e |eeu ued |o ||e+| |ec+|c|||+u|
||eed|u.
',,. 0|e|we|e|keudu ,ud|ore.
hk0IIAk hM0kkhACIC IIANCICIASIA |C|9 . ++3.0
|C|0 . |13.0
FICk 15-31 heredtary hemorrhac te|aoectasa . \u|||p|e |o 2rr, d|c|e|e, |ed r+cu|+|
+ud p+pu|+| |e|+u|ec|+e ou ||e |oWe| ||p +ud |ouue. 8 . \u|||p|e p|upo|u| |e|+u|ec|+e ou ||e |ude\ ||ue| o|
+uo||e| p+||eu|. u|u de|r+|ocop, o| + |+ ||de ||e |e|ou c+u |e |oWu |o pu|+|e.
8
458
SkIN SICNS 0F vASCIAk
INSFFICINC
S E C I | 0 N 1 6
FI0MI0I0C
Ae oI 0oset Middle age to eldeily.
Iocdeoce Atheioscleiosis is the cause of 90%
of aiteiial disease in developed countiies, af-
fecting 5% of men >50 yeais; 10% (20% of dia-
betics) of all men with atheioscleiosis develop
ciitical limb ischemia.
ksk Factors Ior Atherosc|eross Cigaiette
smoking, hypeilipidemia, low high-density li-
popiotein (HDL), high low-density lipopiotein
(LDL), high cholesteiol, hypeitension, diabetes
mellitus, hypeiinsulinemia, abdominal obesity,
family histoiy of piematuie ischemic heait dis-
ease, peisonal histoiy of ceiebiovasculai disease
oi occlusive peiipheial vasculai disease.
0abetes Me||tus aod Iower Ie Ischema
Gangiene of lowei extiemities is estimated to be
up to 150 times moie fiequent in diabetic than
in nondiabetic individuals, most often occui-
iing in those who smoke.
FAIh0CNSIS
Atheioscleiosis is the most common cause of
aiteiial insufficiency and may be geneialized
oi localized to the coionaiy aiteiies, aoitic aich
vessels to the head and neck, oi those supplying
A||e|oc|e|o| o||||e|+u (A'0), epec|+||, o| ||e
|oWe| e\||er|||e, | +oc|+|ed W||| pec||ur o|
cu|+ueou ||ud|u o| |oW|, p|o|e|.e |c|er|c
c|+ue.
',rp|or |+ue ||or |u|e|r|||eu| c|+ud|c+||ou
W||| e\e|||ou+| ruc|e p+|u +ud |+||ue |o ||r|
|c|er|+ W||| |e| p+|u +ud ||ue d+r+e +ud
+cu|e |c|er|+.
Cu|+ueou ||ud|u |+ue ||or d|, ||u, |+|| |o,
ou,c|od,||op|,, +u|eue, +ud u|ce|+||ou.
A||e|oer|o||r | ||e p|euoreuou o| d||od
reu| o| +||e|or+|ou de||| ||or + p|o\|r+|
+||ec|ed +||e|, o| +ueu|,r W||| ceu||||u+| r|
c|oer|o||/+||ou +ud |eu||+u| +cu|e |c|er|c +ud
|u|+|c||.e cu|+ueou |e|ou.
\o|e corrou W||| +d.+uced +e +ud |u.+|.e
p|ocedu|e.
\+u||e|+||ou +|e ||ue o| d|co|o|ed |oe ('||ue
|oe), ||.edo |e||cu|+||, +ud +u|eue.
|C|9 . ++0
|C|0 . |10
AIhk0SCIk0SIS, AkIkIAI INSFFICINC, AN0
AIhk0M80IIIAII0N
the lowei extiemities, i.e., femoial, popliteal,
anteiioi and posteiioi tibial aiteiies. Atheioma-
tous naiiowing of aiteiies supplying the uppei
extiemities is much less common. Atheioma-
tous deposits and thiomboses occui commonly
in the femoial aiteiy in Huntei canal and in
the popliteal aiteiy just above the knee joint.
The posteiioi tibial aiteiy is most often oc-
cluded wheie it iounds the inteinal malleolus,
the anteiioi tibial aiteiy wheie it is supeificial
and becomes the doisalis pedis aiteiy. Atheio-
matous mateiial in the abdominal oi iliac aitei-
ies can also diminish blood flow to the lowei
extiemities as well as bieak off and embolize
downstieam to the lowei extiemities (atheio-
embolization). Detection of atheioscleiosis is
often delayed until an ischemic event occuis,
ielated to ciitical deciease in blood flow.
In addition to laige-vessel aiteiial obstiuc-
tion, individuals with diabetes mellitus often
have miciovasculopathy associated with en-
dothelial cell piolifeiation and basement mem-
biane thickening of aiteiioles, venules, and
capillaiies (see Section 15, pp 427).
Atheroembo|sm Multiple small deposits of
fibiin, platelet, and cholesteiol debiis embol-
ize fiom pioximal atheioscleiotic lesions oi
aneuiysmal sites. Occuis spontaneously oi aftei
intiavasculai suigeiy oi pioceduies such as
aiteiiogiaphy, fibiinolysis, oi anticoagulation.
SCII0N 16 'K|| '|C|' 0| \A'Cu|Ak ||'u|||C|E|C\ 459
Emboli tend to lodge in small vessels of skin
and muscle and usually do not occlude laige
vessels.
Symptoms Athervsc|ervsIs v] Lvwer
ErtremIty ArterIes Pain on exeicise, i.e.,
nermen t|auJtaon . With piogiessive
aiteiial insufficiency, pain and/oi paiesthesias
at iest occui in leg and/oi foot, especially at
night. Individuals with aiteiial insufficiency of
the lowei extiemities often have symptoms of
ischemic heait disease (coionaiy aiteiy disease
oi aiteiioscleiotic heait disease), diabetes
mellitus.
Athervemhv|Ism Acute pain and tendeiness
at site of embolization. Blue toe," puiple toe"
syndiome: peiipheial ischemia, livedo ieticu-
laiis of sudden onset may be accompanied by
embolization to kidney, pancieas, muscle, etc.
Atherosc|eross[Artera| IosuIIceocy
Sko Iesoos Geneial findings associated with
ischemia include palloi, cyanosis, livedoid vas-
culai pattein (Fig. 16-1), loss of haii on affected
limb. Eailiest infaictive changes include well-
demaicated maplike aieas of epideimal necio-
sis. Latei, diy black gangiene may occui ovei
the infaicted skin (puiple cyanosis white
palloi black gangiene) (Fig. 16-2). Shedding
of slough leads to well-demaicated ulceis in
which undeilying stiuctuies such as tendons
can be seen.
Ceoera| xamoatoo Pu|ses Pulse of laige
vessels usually diminished oi absent. In diabet-
ics with mainly micioangiopathy, gangiene may
occui in the setting of adequate pulses. Tempei-
atuie of foot: cool to cold.
Brger SIgn With significant ieduction in
aiteiial blood flow, limb elevation causes palloi
(best noted on plantai foot); dependency causes
delayed and exaggeiated hypeiemia. Ausculta-
tion ovei stenotic aiteiies ieveals biuits.
PuIn Ischemic ulceis aie painful; in diabetics
with neuiopathy and ischemic ulceis, pain may
be minimal oi absent.
DIstrIhutIvn Ischemic ulceis may fiist appeai
between toes at sites of piessuie and beginning
on fissuies on plantai heel. Diy gangiene of
feet, staiting at the toes oi at piessuie sites (Fig.
16-2).
FICk 16-1 Atherosc|eross ob|teraos, ear|y The great toe shows pa||or and there is mott|ed,
|ivedoid erythema on the tip ol the toe. ln this o8-year-o|d diabetic man, the i|iac artery was
occ|uded.
Atheroembo|tatoo
Sko Iesoos Violaceous livedo ieticulaiis on
legs, feet, but also as high up as buttocks.
Ischemic changes with pooi ietuin of coloi af-
tei compiession of skin. Blue toe" (Fig. 16-3):
induiated, painful plaques often following livedo
ieticulaiis on calves and thighs that may un-
deigo neciosis (Fig. 16-4), become black and
ciusted, and ulceiate. Cyanosis and gangiene
of digits.
Ceoera| xamoatoo Pu|ses Distal pulses
may iemain intact.
hemato|oy Rule out anemia, polycythemia.
Ipd Studes Hypeicholesteiolemia (>240 mg/
dL), often associated with iise in LDL. Hypeitii-
g lyceiidemia (250 mg/dL), often associated with
iise in veiy low-density lipopioteins (VLDLs)
and iemnants of theii catabolism (mainly intei-
mediate-density lipopiotein, IDL).
0ermatopatho|oy oI Atheroembo|sm Deep
skin and muscle biopsy specimen shows
aiteiioles occluded by fibiosis with multinu-
cleated giant cells suiiounding biconvex, nee-
dle-shaped clefts coiiesponding to cholesteiol
ciystal micioemboli.
0opp|er Studes Show ieduced oi inteiiupted
blood flow.
0ta| F|ethysmoraphy With exeicise can
unmask significant atheioscleiotic involvement
of lowei extiemity aiteiies.
X-kay Calcification can be demonstiated in-
tiamuially.
Arteroraphy Atheioscleiosis is best visual-
ized by angiogiaphy. Ulceiation of atheioma-
tous plaques seen in abdominal aoita oi moie
distally.
Clinical suspicion confiimed by aiteiiogiaphy
and deep skin biopsy (atheioembolism).
0IIereota| 0aooss 1ntermIttent C|uudI-
cutIvn Pseudoxanthoma elasticum, Buigei
disease (thiomboangiitis obliteians), aithiitis,
gout.
FICk 16-2 Atherosc|eross ob|teraos . I|e|e | p+||o| o| ||e |o|e|oo| +ud ro|||ed e|,||er+ d||+||,
W||| |uc|p|eu| +u|eue ou ||e |e+| |oe +ud ||e ecoud d|||. I|| | + |er+|e d|+|e||c W||| p+|||+| occ|u|ou o|
||e |ero|+| +||e|,. I|e p+||eu| W+ + ro|e|. 8. \o|e +d.+uced +u|eue o| ||e ecoud |o ||e ||||| |oe, ||e |e+|
|oe | e|ou, W|||e +ud W||| +|o |u|u ||+c|.

PuIn]u| Fvvt Gout, inteidigital neuioma, flat
feet, calcanean buisitis, plantai fasciitis, iuptuie
of plantai muscle.
1schemIc und 1n]urctIve LesIvns v] Leg/Fvvt
Vasculitis, Raynaud phenomenon (vasospasm),
disseminated intiavasculai coagulation, ciy-
oglobulinemia, hypeiviscosity syndiome (mac-
ioglobulinemia), septic embolization (infective
endocaiditis), nonseptic embolization (ventiicu-
lai muial thiombus with myocaidial infaiction,
atiial thiombus with atiial fibiillation), aneu-
iysms (dissecting, thiombosed), diug-induced
neciosis (waifaiin, hepaiin), eigot poisoning, in-
tiaaiteiial injection, livedo ieticulaiis syndiomes,
exteinal compiession (popliteal entiapment).
C0kS AN0 Fk0CN0SIS
rera| nsu[[tenty is a slowly piogiessive dis-
ease, punctuated by episodes of complete occlu-
sion oi embolism. Appioximately 5% pei yeai
of individuals with inteimittent claudication
piogiess to pain at iest oi gangiene. A much
highei peicentage die fiom othei complica-
tions of atheioscleiosis such as ischemic heait
disease.
Infection fiom inteidigital tinea pedis, leg/
foot ulceis, oi small bieaks and fissuies in the
skin. Chionic lymphedema, piioi saphenous
venous haivesting, and piioi episodes of cel-
lulitis inciease the likelihood of cellulitis.
FICk 16-3 Atheroembo|sm aIter aooraphy A ro|||ed ('||ue |oe), .|o|+ceou, .+cu|+| p+||e|u
ou ||e |o|e|oo| +ud |e+| |oe. I|e ||ud|u We|e uo|ed +||e| |u||+.+cu|+| c+||e|e||/+||ou +ud +u|o|+p|, |u +u
|ud|.|du+| W||| A'0.
FICk 16-4 Atheroembo|sm wth cutaoeous oIarctoo \|o|+ceou d|co|o|+||ou +ud cu|+ueou |u|+|c
||ou W||| + ||ue+| +||+uereu| ou ||e red|+| |||| o| + 1!,e+|o|d Wor+u W||| +||e|oc|e|o|, |e+|| |+||u|e,
+ud d|+|e|e.
Atheioscleiosis of coionaiy and caiotid ai-
teiies usually deteimines suivival of patient, but
involvement of lowei extiemity aiteiies causes
significant moibidity. Balloon angioplasty,
endaiteiectomy, and bypass pioceduie have
impioved piognosis of patients with atheioscle-
iosis. Amputation iates have been loweied fiom
80% to <40% by aggiessive vasculai suigeiy.
|eroem|o|sm may be a single episode if athe-
ioembolization follows intiaaiteiial pioceduie.
May be iecuiient if spontaneous and associated
with significant tissue neciosis.
MANACMNI
Freveotoo Goal of management is pievention
of atheioscleiosis.
Managemen o[ rmary |yer|Jema: Re-
duce intake of satuiated fats and cholesteiol as
well as caloiies. Exertse is a useful adjunct to
diet. Walking incieases new collateial vessels in
A |+|e |u||+rr+|o|, occ|u|.e d|e+e o| red|ur
|/ed +ud r+|| +||e||e +ud .e|u.
||edor|u+u||, |u r+|e, 20-+0 ,e+| o| +e.
\e|, ||ou +oc|+||ou W||| ro||u.
Au +u|||| c||u|c+||, |ud|||uu||+||e ||or I0
occu| |u pe|ou couur|u c+uu+||.
C||u|c+| r+u||e|+||ou +|e co|d eu|||.||,,
|c|er|+. c|+ud|c+||ou o| |e, |oo|, +|r, o| |+ud.
|e||p|e|+| c,+uo|, |c|er|c u|ce|, +u|eue
(||. o5), +ud upe|||c|+| |||or|op||e||||.
I|e|+p,. ro||u ce+||ou, +u+|e|c, Wouud
c+|e, +u||p|+|e|e| +eu|, p|o|+c,c||u, peu|o\,
p|,||u, +u|op|+|,, ,rp+||ec|or,, +rpu|+||ou.
',,. Bu|e| d|e+e.
Ihk0M80ANCIIIIS 08IIIkANS (I0) |C|9 . ++!.
|C|0 . |1!.
'upe|||c|+| p||e|||| ('|) | +u |u||+rr+|o|,
|||or|o| o| + upe|||c|+| c| .e|u, uu+||,
due |o |u|ec||ou o| ||+ur+ ||or ueed|e +ud
c+||e|e|.
|u||+rr+|o|, |||or|o| o| .c:- .e|u uu+||,
|u ||e cou|e\| o| ||e c||ou|c .euou |uu|||c|euc,
(C\|) ,ud|ore.
|C|9 . o1.2
|C|0 . | 30
|eep .euou |||or|o| (|\ I) | due |o |||or
|o||c o|||uc||ou o| + .e|u W||| o| W|||ou| +u
|u||+rr+|o|, |epoue.
0ccu| due |o |oW ||ood ||oW, |,pe|co+u|+|||
||,, o| c|+ue |u ||e .euou W+||.
I|e ro| corrou c+ue +|e |oWu |u
I+||e o
|C|9 . +!!.+0
|C|0 . | 30.2
Ihk0M80FhI8IIIS AN0 0F vN0S Ihk0M80SIS
ischemic muscle. Reduce elevated blood pies-
suie. Dstonnue tgaree smo|ng. Coiiect
anemia oi polycythemia. Drug |eray is iec-
ommended foi adults with LDL cholesteiol
>190 mg/dL oi >160 mg/dL in the piesence of
two oi moie iisk factois aftei an adequate tiial
of at least 3 months of diet theiapy alone.
Medca| Maoaemeot Encouiage walking to
cieate new collateial vessels. Position ischemic
foot as low as possible without edema. Hepaiin
and waifaiin. IV piostacyclins. Analgesics.
Surca| Maoaemeot Endaiteiectomy oi by-
pass foi aoitic iliac occlusions and foi exten-
sive femoial popliteal disease. Distal bypass
suigeiy of ciuial aiteiies. Response to suigical
ievasculaiization oi thiombolytic theiapy of-
ten pooi in atheioembolization. Debiidement
of neciotic tissue locally. Remove oi bypass
atheioscleiotic vessel oi aneuiysm. Amputation
of leg/foot: indicated when medical and suigical
management has failed.
IA8I 16-1 Predisposin |actors in 0eep Venous Ihrombosis
Common Factors
\+jo| u|e|, 0|+| cou||+cep||.e
||+c|u|e \+||u+uc|e
Coue||.e |e+|| |+||u|e \euou .+||co|||e
Acu|e r,oc+|d|+| |u|+|c||ou ||e.|ou |||o|, o| .euou |||or|o|
'||o|e |e|deu |+c|o| \ ru|+||ou
||eu+uc, +ud po|p+||ur 'e.e|e pu|rou+|, |uu|||c|euc,
'p|u+| co|d |uju||e ||o|oued |rro||||/+||ou
'|oc|
Less Common Factors
'|c||e ce|| +uer|+ Au|||||or||u ||| de||c|euc,
noroc,||uu||+ Au||p|op|o||p|d +u|||od|e
||o|e|u C o| ' de||c|euc, u|ce|+||.e co||||
'ou|ce. I| Co||r+u, kI E|e||+|d|, |u |\ ||eed|e| e| +|. (ed). ||cc|:| |-c||, C--c| !-!:-, o|| ed.
|eW \o||, \cC|+Wn|||, 200!.
FICk 16-5 Ihromboaots ob|teraos |u|+|c||.e uec|o| ou ||e |e+| |oe o| + 23,e+|o|d r+u. I|e
|e|ou | e\qu|||e|, p+|u|u|. (I|e ,e||oW||||oWu|| co|o| | ||or |od|ue d||u|ec||ou).
The thiombus oiiginates in an aiea of low
venous flow. An occlusion of a vein by thiom-
bus imposes a block to venous ietuin, which
leads to incieased venous piessuie and edema
in the distal limb. An inflammatoiy iesponse
to the thiombus causes pain and tendeiness.
If the venous piessuie is too high, aiteiial limb
flow may iaiely be compiomised and ischemia
of the distal limb may occui. The thiombus
in the vein often has a fiee-floating tail, which
may bieak off to pioduce a pulmonaiy embo-
lus. Oiganization of the thiombus in the vein
destioys the venous walls, and this leads to the
postthiombotic syndiome.
Patients complain of pain oi aching in the
involved limb oi notice limb swelling. Some
patients may have no symptoms. Pulmonaiy
embolus may be the fiist indication of DVT.
Supeificial thiombophlebitis is diagnosed by
the chaiacteiistic induiation of a supeificial vein
with iedness, tendeiness, and incieased heat (Fig.
16-6). DVT piesents with a swollen, waim, ten-
dei limb (Fig. 16-6B) with piominent distended
collateial veins. Pitting edema may occui but is
not always piesent, and a tendei coid may be felt
wheie the vein is thiombosed. With iliofemoial
thiombophlebitis the limb is swollen fiom the
foot to the inguinal iegion and tendeiness is
not piesent in the limb, but collateial veins may
foim fiom the thigh to the abdominal wall. Two
types aie iecognized: the limb may be veiy pale
and painful ( ||egmasa a||a Jo|ens ) (Fig. 16-6B)
oi may be cyanotic and painful with cold digits
if the aiteiial inflow is also compiomised ( ||eg-
masa toeru|ea Jo|ens ). In thiombosis of calf
veins the calf and foot aie swollen and waim, and
theie is deep tendeiness of the calf, often without
a palpable coid (Fig. 16-6B).
Mgraory ||e|s desciibes an inflammatoiy
induiation of supeificial veins that migiates
within a defined iegion of the body; may be
associated with thiomboangiitis obliteians and
malignancies. MonJor Jsease (scleiosing phle-
bitis) is an induiated, subcutaneous vein fiom
the bieast to the axillaiy iegion that duiing
healing leads to a shoitening of the venous coid,
which puckeis the skin.
Venous imaging by coloi-coded duplex ultia-
sound and Dopplei examination ieveals an
absence of flow oi of the noimal iespiiatoiy
venous flow vaiiations in pioximal venous oc-
clusions. Foi thiombophlebitis of the calf veins,
intiavenous
125
I] fibiinogen oi a venogiam
gives a definite diagnosis.
Lymphedema, cellulitis, eiysipelas, supeificial
phlebitis, lymphangitis. An uncommon dif-
feiential diagnosis is iuptuie of the plantai
muscle, which pioduces pain, swelling, and
ecchymotic aieas in the dependent ankle
aiea.
MANACMNI
The tieatment of SP is compiession, antiplate-
let diugs, and nonsteioidal anti-inflammatoiy
agents.
The tieatment of DVT is anticoagulation.
IV hepaiin at a loading dose of 5000 U and
appioximately 1000 U/h theieaftei. The paitial
thiomboplastin time (PTT) should be 1.5-2
times noimal. Low-moleculai-weight hepaiin
is also effective. Waifaiin can be staited oially
at the same time and should oveilap hepaiin foi
5 days until the necessaiy factois foi blood clot-
ting aie depiessed. Patients should be tieated
foi at least 3 months with anticoagulation.
Elastic stockings and compiession aie manda-
toiy and should be woin foi at least 3 months;
zinc paste-impiegnated bandages (Unna boot)
and ambulation should be staited as soon as
symptoms subside.
Vaiicose veins: peak incidence of onset 30-40
yeais. Vaiicose veins aie thiee times moie com-
mon in women than in men.
to|oy CVI is most commonly associated
with vaiicose veins and the postphlebitic
syndiome. Vaiicose veins aie an inheiited
chaiacteiistic.
C||ou|c .euou |uu|||c|euc, (C\|) |eu|| ||or
|+||u|e o| ceu|||pe|+| |e|u|u o| .euou ||ood +ud
|uc|e+ed c+p|||+|, p|eu|e.
I|e |eu||+u| c|+ue |uc|ude eder+, |+|
de|r+||||, |,pe|p|reu|+||ou, ||||o| o| ||e ||u
+ud u|cu|+ueou ||ue (||pode|r+|oc|e|o|)
o| ||e |e, +ud u|ce|+||ou.
\euou u|ce| +|e ||e ro| corrou c||ou|c
Wouud |u |ur+u.
Chk0NIC vN0S INSFFICINC |C|9 . +59.3
|C|0 . |31.2
Aravato Factors Piegnancy, incieased
blood volume, incieased caidiac output, in-
cieased venocaval piessuie, piogesteione.
FAIh0CNSIS
The damaged valves of the deep veins of the
calf aie incompetent at iestiicting backflow
of blood. Damaged communicating veins
FICk 16-6 SuperIca| ph|ebts aod deep veoous thromboss . A ||ue+| p+|u|u| e|,||er+|ou
co|d e\|eud|u ||or ||e pop|||e+| |o+ |o ||e r|dc+|| |u + !5,e+|o|d r+u W|o |+d rode|+|e .+||co|||e. |||e
|||| occu||ed +||e| + 5| |||||. 8. I|e |e | Wo||eu, p+|e, W||| + ||o|c|, c,+uo||c d|co|o|+||ou, +ud | p+|u|u|.
I|e ep|ode occu||ed +||e| +|dor|u+| u|e|, (||e c||cu|+| r+|| +|e ||or + corp|e|ou |+ud+e).
8
connecting deep and supeificial calf veins also
cause CVI in that blood flows fiom deep veins
to supeificial venous plexus. Fibiin is deposited
in the extiavasculai space and undeigoes oigan-
ization, iesulting in scleiosis and obliteiation of
lymphatics and miciovasculatuie. Peiivasculai
fibiosis iesults in diminished nutiition of the
epideimis, which bieaks down with ulcei foi-
mation.
This cycle iepeats itself: initial event ag-
giavation of venous stasis and vaiicose vein
dilatation lipodeimatoscleiosis thiombo-
sis stasis deimatitis ulceiation.
Piioi episode(s) of supeificial phlebitis and
DVT. Risk factois aie listed in Table 16-1.
CVI commonly associated with heaviness oi
aching of leg, which is aggiavated by standing
(dependency) and ielieved by walking. Lipo-
deimatoscleiosis may limit movement of ankle
and cause pain and limitation of movement,
which in tuin incieases stasis. Leg edema aggia-
vated by dependency (end of the day, standing),
summei season. Shoes feel tight in the evening.
Night ciamps.
A simple staging system foi CVI is shown in
Table 16-2.
Sko Iesoos
varcose veos Supeificial leg veins aie en-
laiged, toituous, with incompetent valves; best
evaluated with the patient standing (Fig. 16-7).
Blow-out" at sites of incompetent commu-
nicating veins. Touiniquet test: A touiniquet
is applied to the leg that has been elevated to
empty the veins; when the patient stands up and
the touiniquet is ieleased, theie is instant filling
of a vaiicose vein due to absent oi ill-function-
ing valves. Vaiicose veins may oi may not be
associated with staibuist phlebectasia usually
oveilying the aiea of an incompetent communi-
cating vein (Fig. 16-7B). These small venectasias
pei se have no pathogenic significance but aie
of cosmetic concein to the patient. Supeifi-
cial venectasias (spidei phlebectasia) without a
staibuist pattein occui also and fai moie com-
monly without CVI, usually on the thighs and
lateial lowei legs in women.
dema Dependent; impioved oi iesolved in
the moining aftei a night in the hoiizontal posi-
tion. Doisa of feet, ankles, lowei legs.
ctematous (Stass) 0ermatts Occuis in set-
ting of CVI about the lowei legs and ankles
(Fig. 16-8). It is a classic eczematous deimatitis
with inflammatoiy papules, scaly and ciusted
eiosions; in addition, theie is pigmentation,
stippled with iecent and old hemoiihages (Fig.
16-9); deimal scleiosis; and excoiiations due
to sciatching. It must be distinguished fiom
contact deimatitis secondaiy to topical agents,
with which it is often combined. In addition,
theie may be concomitant iiiitant deimatitis
due to secietion fiom stasis ulcei (see below)
and bacteiial colonization. If extensive, may be
associated with geneialized eczematous deima-
titis, i.e., id" ieaction oi autosensitization (see
Section 2).
Atrophe 8|aoche Small ivoiy-white depiessed
plaques (Fig. 16-9) on the ankle and/oi foot;
stellate and iiiegulai, coalescing; stippled
IA8I 16-2 Stain of CV| (CEAP C|assification)
Clinical Picture {C) Etiology {E)
C
o
uo c||u|c+| |u E
p
p||r+|,
C
r+|| .+||coe .e|u E
ecoud+|,
C
2
|+|e .+||coe .e|u E
c
coueu||+|
C
!
eder+
C
+
||u c|+ue
C
5
|e+|ed u|ce|
C
o
+c||.e u|ce|
Anatomy {A) Patbogbysiology {P)
A
upe|||c|+| |
|
|e||u\
A
d
deep |
o
o|||uc||ou
A
p
pe||o|+u (corruu|c+||u .e|u) |
|,o
|e||u\ + o|||uc||ou
SCII0N 16 'K|| '|C|' 0| \A'Cu|Ak ||'u|||C|E|C\ 46T
pigmentation; hemosideiin-pigmented boidei,
usually within stasis deimatitis. Often following
tiauma.
Ipodermatosc|eross Inflammation, induia-
tion, pigmentation of lowei thiid of leg cieating
champagne bottle" oi piano leg" appeaiance
with edema above and below the scleiotic
iegion (Fig. 16-10). Gioove sign" cieated by
vaiicose veins meandeiing thiough scleiotic
tissue. A veiiucous epideimal change can occui
oveilying the scleiosis; if combined with chionic
lymphedema, it is iefeiied to as e|e|anass
nosras errutosa . In long-standing scleiosis,
calcification can occui.
|ceratoo Occuis in 30% of cases; veiy pain-
ful hypeialgesic micioulcei" in aiea of atio-
phie blanche; laigei supeificial oi deep ulceis,
shaiply defined with deep maigin, neciotic
base suiiounded by atiophie blanche, stasis dei-
matitis, and lipodeimatoscleiosis (Fig. 16-11).
Venous ulceis usually occui medially and above
ankles (Fig. 16-11). Venous ulceis and theii dif-
feiential diagnosis aie discussed in moie detail
below (p. 470).
FICk 16-T varcose veos . I|e|e +|e re+ude||u +ud cou.o|u|ed |||eu|+| .+||coe .e|u ou ||e ||||
o| + 10,e+|o|d r+u W|o +|o |+d ||pode|r+|oc|e|o| +ud |+| de|r+|||| ou ||e |oWe| |e. 8. '|c||
.--:|cc ||- :c|| I|| | +u +|e+ o.e||,|u +u |uu|||c|eu| corruu|c+||u .e|u.
8
0opp|er aod Co|or-Coded 0up|ex Sooora-
phy These detect incompetent veins, venous
occlusion due to thiombus.
Fh|eboraphy Contiast medium is injected
into veins to detect incompetent veins and ve-
nous occlusion.
Imao X-iay may show subcutaneous calcifi-
cation (10% of chionic cases), i.e., postphlebitic
subcutaneous calcinosis. Bony changes include
peiiostitis undeilying ulceiation, osteopoiosis
as a iesult of disuse, fibious ankylosis of ankle.
Osteomyelitis.
0ermatopatho|oy Ear|y : small venules and
lymphatic spaces appeai dilated; edema of ex-
tiacellulai space with swelling and sepaiation
of collagen bundles. Laer : capillaiies dilated,
congested with tuft foimation and toituousity
of venules; deposition of fibiin. EnJo|e|a| te||
|yerro|y : may be associated with venous
thiombosis; angioendotheliomatous piolifeia-
tion mimicking Kaposi saicoma. In all stages,
extiavasation of ied blood cells that bieak down
foiming hemosideiin, which is taken up by mac-
iophages. Lymphatic vessels become encased in
a fibiotic stioma, i.e., lipodeimatoscleiosis. Cal-
cification of fat and fibious tissue may occui.
0IACN0SIS
Usually made on histoiy, clinical findings, Dop-
plei and coloi-coded Duplex sonogiaphy, phle-
bogiaphy.
MANACMNI
Frerequste Compiession diessings oi stock-
ings; Unna boot.
Atrophe 8|aoche Avoid tiauma to aiea in-
volved. Intialesional tiiamcinolone into painful
lesions. Compiession.
Stass 0ermatts Topical gluscocoiticoids
(shoit teim). Topical antibiotic tieatments (e.g.,
mupiiocin) when secondaiily infected. Cultuie
foi methicillin-iesistant Sa|y|otottus aureus
(MRSA).
FICk 16-8 Stass dermatts o
CvI 1 p+|c| o| ec/er+|ou de|r+||||
o.e||,|u .euou .+||co|||e ou ||e red|+|
+u||e |u + 59,e+|o|d Wor+u. I|e |e|ou
| p+pu|+|, c+|,, +ud ||c||u.
FICk 16-9 Chrooc veoous
osuIIceocy. Atrophe b|aoche
Au +|e+ o| d|||ue +ud ro|||ed p|reu
|+||ou due |o |ero|de||u +ud |.o|,
W|||e p+|c|e o| +||op||e ||+uc|e.
'uc| |e|ou +|e |o|| ||c|, +ud p+|u|u|.
FICk 16-10 Chrooc veoous osuIIceocy aod |podermatosc|eross I|e +u||e | |e|+||.e|, |||u +ud
||e uppe| c+|| eder+|ou, c|e+||u + 'c|+rp+ue |o|||e o| 'p|+uo |e +ppe+|+uce. . \+||coe .e|u +|e er|ed
ded |u p|reu|ed, c|e|o||c ||ue. I|e|e +|e +|o +|e+ o| +||op||e ||+uc|e. 8. \+||coe .e|u +|e |e .||||e |e|e
|u| c+u |e e+||, p+|p+|ed |u ||e c|e|o||c p|+que euc+|u ||e eu|||e c+|| ('|oo.e |u). I|e|e | +|o p|reu|+
||ou +ud r|uo| p+pu|+| |+| de|r+||||.
8
varcose veos 1nectIvn Sc|ervtherupy A
scleiosing agent such as tetiadecyl sulfate is in-
jected into vaiicosities, followed by piolonged
compiession. Used mainly to tieat minoi
bianch vaiicosities not associated with saphe-
nous incompetence and new bianch vein vaii-
cosities developing aftei suigeiy. Recuiience is
common within 5 yeais.
Vuscu|ur Surgery Incompetent peifoiating
veins aie identified, ligated, and cut, followed
by stiipping long and/oi shoit saphenous veins
out of the main tiunk. Residual peifoiating
veins aie the main cause of iecuiiences aftei
suigeiy. In patients with combined aiteiial
and CVI, bypass oi angioplasty may piove
beneficial.
Endvvuscu|ur TechnIques These new technol-
ogies encompass endoscopic subfascial dissec-
tion of peifoiating veins (employed piimaiily
in the elimination of insufficient peifoiating
veins in CVI); and endoscopic endovenous
diode lasei oi iadiofiequency theimal heating,
which leads to occlusion of vaiicose vein.
veoous |cers See below.
FICk 16-11 veoous osuIIceocy . IWo co+|ec|u u|ce| W||| + uec|o||c |+e |u +u +|e+ o| +||op||e
||+uc|e, ||pode|r+|oc|e|o|, +ud |+| de|r+||||. 'c|+|c| r+|| |ud|c+|e ||c||ue o| u||ouud|u ||u, W|||e ||e
u|ce| +|e p+|u|u|. 8. A |+u| u|ce|, We||de||ued W||| c+||oped |o|de| +ud + |ee|, |ed |+e |u + |e W||| ||pode|
r+|oc|e|o|.
8
SCII0N 16 'K|| '|C|' 0| \A'Cu|Ak ||'u|||C|E|C\ 4T1
veoous |cers The pievalence of venous ul-
ceis is estimated to be appioximately 1%. It
iises with patient age, obesity, pievious leg
injuiy (fiactuies), DVT, and phlebitis. Patients
complain of limb heaviness, swelling associated
with standing and woisening in the evening,
and pain. Venous ulceis aie associated with
at least one oi all of the symptoms of CVI
(Fig. 16-11 and Table 16-2) and may be sin-
gle oi multiple; they aie commonly found on
the medial lowei aspect of the calf, especially
ovei the malleolus (medial > lateial), in the
aiea supplied by incompetent peifoiating veins
(Fig. 16-11). They can be laige, involving the
ciicumfeience of the entiie lowei leg (Fig.
16-11B). They aie shaiply defined, iiiegulaily
shaped, ielatively shallow with a sloping boi-
dei, and usually painful. The base is usually
coveied by fibiin and neciotic mateiial (Fig.
16-11), and theie is always secondaiy bacte-
iial colonization. Stasis ulceis can also develop
in the most dependent paits of a pendulous
abdominal panniculus in a massively obese
individual. Squamous cell caicinoma (SCC)
can aiise in a long-standing venous ulcei (Fig.
16-12) of the leg.
Artera| |cers Aiteiial ulceis aie associated
with peiipheial aiteiial disease (atheioscleiosis
obliteians, see p. 458). Associated with inteimit-
tent claudication and pain, even at iest, as disease
piogiesses. Chaiacteiistically painful at night and
often quite seveie; may be woise when legs aie
elevated, impioving on dependency. Occui on
the lowei leg, usually ovei sites of piessuie and
tiauma: pietibial, supiamalleolai (usually lat-
eial), and at distant points, such as toes. Painful.
Punched out, with shaiply demaicated boideis
(Fig. 16-13). A tissue slough is often piesent at
the base, undei which tendons can be seen. Exu-
dation minimal. Associated findings of ischemia:
loss of haii on feet and lowei legs, shiny atiophic
skin. Stasis pigmentation and lipodeimatoscleio-
sis aie absent. Pulses diminished oi absent.
|e u|ce| occu| corrou|, |u |+|e r|dd|e +ud o|d
+e.
I|e, +||e |u +oc|+||ou W||| C\|, c||ou|c +||e||+|
|uu|||c|euc,, o| pe||p|e|+| euo|, ueu|op+||,.
|u ore p+||eu|, + cor||u+||ou o| ||ee |+c|o|.
|+|||cu|+||, |u d|+|e|e |e u|ce| +|e corrou. Au
e||r+|ed 2 r||||ou pe|ou |u ||e uu||ed '|+|e
|+.e |e u|ce|, W||| +u e||r+|ed |o o| 2 r||||ou
Wo||d+, pe| ,e+|.
|e u|ce| +|e +oc|+|ed W||| |u|||c+u| |ou
|e|r ro|||d||, +ud o||eu do uo| |e+| uu|e ||e
uude||,|u p|o||er() | (+|e) co||ec|ed.
k+|e|, qu+rou ce|| c+|c|uor+ c+u +||e |u
c||ou|c .euou u|ce|.
M0SI C0MM0N IC[F00I ICkS |C|9 . 101
|C|0 . |3!.0
A special type of aiteiial ulcei is Marore||
u|ter , which is associated with labile hypeiten-
sion and lacks clinical signs of atheioscleiosis
obliteians. Ulcei(s) stait with a black eschai
suiiounded by eiythema and aftei sloughing of
neciotic tissue aie punched out with shaiply
demaicated boideis, with suiiounding eiy-
thema; veiy painful on the anteiioi lateial
lowei leg.
Comboed Artera| aod veoous |cers These
ulceis aiise in patients who have both CVI
and atheioscleiosis obliteians and thus show
a combination of signs and symptoms of both
venous and aiteiial insufficiency and ulceiation
(Fig. 16-14). Symptoms include inteimittent
claudication, pain both at elevated and depend-
ent position of the leg, both palloi and cyanosis
of the foot, stasis deimatitis, and lipodeima-
toscleiosis associated with both sloped and
punched out ulceis ieaching down to tendons
(Fig. 16-14).
Neuropathc |cers (See Diabetic Foot,"
p 426) Soles, toes, heel. Most commonly as-
sociated with diabetes of many yeais` duiation.
Eaily symptoms of neuiopathy include pai-
esthesia, pain, anesthesia of leg and foot.
Patients aie often unawaie of piioi tiauma
that commonly piecedes ulceiations of heel,
plantai metataisal aiea, oi gieat toe. Neuio-
pathic ulceis aie discussed in Section 15 (see
Fig. 15-4).
A diffeiential diagnosis of the thiee main types
of leg/foot ulceis is shown in Table 16-3. Othei
diffeiential diagnostic consideiations include
ulceiated SCC (note that SCC can aiise in a
long-standing venous ulcei) (Fig. 16-12), basal
cell caicinoma, injection diug use (skin pop-
ping), piessuie ulcei (ski boot). Ulceiations also
occui in vasculitis (paiticulaily polyaiteiitis
IA8I 16-3 0ifferentia| 0ianosis of Ihree Najor Iypes of Le U|cers
Les|oo S|te S0rro0od|og Sk|o 6eoera| xam|oat|oo
\euou |||eu|+| \+||eo|+| +ud ||pode|r+|oc|e|o| \+||coe .e|u
up|+r+||eo|+|
'|oped |o|de| (red|+|) '|+| de|r+|||| |+|u, Wo|e |u
depeudeu| |+|e
|ec|o||c |+e A||op||e ||+uc|e
|||||u ||reu|+||ou
|,rp|eder+
A||e||+| |uuc|ed ou| ||eu|e ||e. A||op||c, ||u, we+|/+|eu| pu|e
d||+| (|oe),
p|e||||+|,
up|+r+||eo|+|
|ec|o||c |+e (|+|e|+|) n+|| |o |+||o| ou e|e.+||ou o| |e
|+||o| o| |e+c||.e |,pe|er|+ |+|u Wo|e ou e|e.+||ou o| |e
|eu|op+|||c |uuc|ed ou| ||eu|e ||e C+||u |e|o|e u|ce|+||ou |e||p|e|+| ueu|op+||,
+ud u||ouud|u u|ce|
||+u|+| |ec|e+ed eu+||ou
|o p+|u
chrooc veoous u|cer 1 .euou u|ce| |+d |eeu
p|eeu| >0 ,e+| |u +u +|e+ o| ||pode|r+|oc|e|o| +ud
|+| de|r+||||. E.eu|u+||, ||e |+e o| ||e u|ce| |ec+re
e|e.+|ed, |+|d, |e p+|u|u|. |eep ||op, (c||cu|+| r+||
|u ||e ceu|e|) |e.e+|ed uec|o| +ud +| ||e |+e |u.+|.e
qu+rou ce|| c+|c|uor+.
FICk 16-13 Chrooc artera| osuII-
ceocy wth a sharp|y deIoed, "puoched
out" u|cer wth rreu|ar out|oes I|e
e\||er||, W+ pu|e|e, +ud ||e|e W+ r+
|.e |c|er|+ ou ||e |oe.
FICk 16-14 Chrooc artera| aod
veoous osuIIceocy, "comboed" arte-
ra| aod veoous u|cers |o|e p|ououuced
||pode|r+|oc|e|o| +ud u|ce|+||ou ou ||e
up|+r+||eo|+| |oWe| |e (.euou corpoueu|)
+ud pu|p|e d|co|o|+||ou o| |o|e|oo| +ud |oe
W||| puuc|edou| u|ce| |e.e+||u |eudou o.e|
re|+|+|+| ||e (+||e||+| corpoueu|).
nodosa), eiythema induiatum, calciphylaxis,
and vaiious infections ecthyma, Buiuli ulcei,
Myto|aterum marnum infection, gumma,
lepiosy, invasive fungal infection, chionic hei-
pes simplex viius (HSV) ulcei] and in sickle
cell anemia, polycythemia veia, pyodeima gan-
gienosum, neciobiosis lipoidica with ulceia-
tion, factitia.
C0kS AN0 Fk0CN0SIS
Couise and piognosis aie dependent on un-
deilying disease. With coiiection of undeilying
causes, ulceis heal with initial foimation of
pink gianulation tissue at the base, which is
ieepithelialized by epithelium fiom eithei iesid-
ual skin appendages oi suiiounding epideimis.
Venous ulceis can heal with a pseudoepitheli-
omatous epideimal hypeiplasia within the scai,
which can mimic SSC.
MANACMNI
Ceoera| Maoaemeot In geneial, factois such
as anemia and malnutiition should be coi-
iected to facilitate healing. Contiol hypeiten-
sion, weight ieduction in the obese, exeicise;
mobilize patient; coiiect edema caused by cai-
dial, ienal, oi hepatic dysfunction. Of utmost
impoitance is tieatment of undeilying disease.
Aiteiial ulceis do not heal unless aiteiial blood
flow is coiiected by endaiteiectomy to iemove
localized atheiomatous plaques oi bypass of oc-
cluded aieas (see Management" of atheioscle-
iosis obliteians, p. 462). Aiteiial ulceis at acial
sites that do not heal despite aiteiial ieconstiuc-
tion oi wheie aiteiial ieconstiuction cannot be
peifoimed may iequiie amputation. Venous
ulceis tend to be iecuiient unless undeilying
iisk factois aie coiiected, i.e., coiiective suigeiy
and/oi elastic stockings woin on a daily basis
(see Management" of chionic venous insuf-
ficiency, p. 468). Bewaie of excess compiession
in patients with additional undeilying aiteiial
occlusion; leg elevation; inteimittent pneumatic
compiession. In neuiopathic ulceis, coiiect
undeilying diabetes. Rule out oi tieat undeily-
ing osteomyelitis. Distiibute weight of piessuie
points with special shoes in neuiopathic ulceis.
Noe : diabetic patients aie paiticulaily pie-
disposed to ulceis and fiequently have seveial
etiologic factois in play, i.e., peiipheial vasculai
disease, neuiopathy, infection, and impaiied
healing.
Secondaiy infection should be tieated with
antibiotics both topically and systemically in all
ulceis. Ulceis piovide an easy poital of entiy foi
systemic infection, which should be suspected if
pain appeais oi incieases in intensity. Infection
can occui ielatively supeificially in ulcei base oi
moie invasively with cellulitis and possible lym-
phangitis and bacteiemia. Bewaie of MRSA.
Ioca| Ireatmeot oI |cer aod Surrouodo
Sko Tieat stasis deimatitis (oi iiiitant, oi
alleigic contact deimatitis) in CVI with wet
diessings in the acute exudative phase and sub-
sequently with modeiate to potent glucocoi-
ticoid ointment. In all ulceis debiide neciotic
mateiial mechanically (suigically) oi by enzy-
matic debiiding agents, including collagenase
and papain; use antiseptics and antibiotics to
counteiact infection. Hydiocolloid diessings.
Gianulating but only slowly epithelializing ul-
ceis aie tieated by suigical pioceduies eithei by
pinch giafts, split-thickness skin giafts, epidei-
mal giafts, cultuied keiatinocyte allogiafts, oi
composite giafts.
|\ | + |||or|o||c .+cu|op+||, o| de|r+| .ee|
cou||ued |o ||e |oWe| e\||er|||e +ud |+|||u
ro||, |u ||e +u||e |e|ou.
A |||+d o| ||.edo |e||cu|+||, +||op||e ||+uc|e, +ud
.e|, p+|u|u|, r+|| puuc|edou| u|ce| ||+| |+.e +
.e|, poo| |eudeuc, |o| |e+||u (||. o5).
A||op||e ||+uc|e |u ||.edo|d .+cu|||| | c||u|c+||,
|ud|||uu||+||e ||or ||+| eeu |u C\|, e\cep| |o|
.+||coe .e|u (corp+|e ||. o5 +ud o9). |\
| + |e+c||ou p+||e|u o| ||e ||u ||+| o||eu |ecu| |u
W|u|e| o| urre| ('||.edo |e||cu|+|| W||| W|u|e|
+ud urre| u|ce|+||ou ).
n||o|o|c+||,, ||e|e +|e |||||u |||or|| |u r+||
+ud red|ur|/ed de|r+| .e|u +ud +||e||e W|||
Wede|+ped uec|o| +ud |,+||u|/+||ou o| ||e
.ee| W+|| (ereu|+| |,+||u|/|u .+cu||||).
|\ r+, |e |d|op+|||c o| r+, |e +oc|+|ed W|||
'ueddou ,ud|ore (ee ||. +9, p. !15),
+u||p|op|o||p|d +u|||od, ,ud|ore, o| coud|
||ou o| |,pe|co+u|+|||||, o| |,pe|.|co||,.
I|e+|reu|. |ed |e|, +u+|e|c, |oWdoe |ep+||u,
+ud p|+|e|e| +|e+||ou |u|||||o|. |+|u c+u |e
|e||e.ed +ud |e+||u +cce|e|+|ed |, ,|er|c
|ucoco|||co|d. Au+|o||c +eu| uc| + d+u+/o|
+ud |+u+/o|o| |+.e |eeu +uecdo|+||, |epo||ed |o
|e e||ec||.e.
|+|e| u|ce| W||| |+.e |o |e e\c|ed +ud |+||ed.
IIv00I0 vASCIIIIS ( Iv ) |C|0 . |95.0
FICk 16-15 Ivedod vascu|ts I|| | c|+|+c|e||/ed |, ||e |||+d o| ||.edo |e||cu|+||, +||op||e ||+uc|e
+ud r+||, p+|u|u|, c|u|ed u|ce|. I|| | c||u|c+||, |ud|||uu||+||e ||or +||op||e ||+uc|e eeu |u C\| e\cep| |o|
||e +|euce o| .+||coe .e|u.
|,rp|eder+ |u c|||d|ood +ud e+||, +du|| |||e +|e
eue||c +ud +|e o||eu c+ued |, de|ec| |u .+cu
|+| eudo||e||+| |oW|| |+c|o| |ecep|o| (\EC|k!)
+ud |o\C 2 , + ||+uc||p||ou |+c|o|.
Acqu||ed |,rp|eder+ o| +du|| r+, |e |e|+|ed
|o c||ou|c .euou |uu|||c|euc,, c||ou|c, |ecu|
||u o|| ||ue |u|ec||ou (e|,|pe|+, ce||u||||,
ee 'ec||ou 2+), uode d|ec||ou +ud |+d|+||ou
+||e| c+uce|, +ud |u ore eo|+p||c |e|ou |,
|||+||+|.
|epeud|u ou e||o|o, +cqu||ed |,rp|eder+
ro| corrou|, occu| ou ||e |oWe| e\||er|||e
|u| r+, +|o +||e ou ||e +|r +ud |+ud.
C||u|c+| r+u||e|+||ou. We|||u o| e\||er|||e, p||
||u eder+ |u|||+||, |oW|, e.o|.|u |u|o uoup||||u
Wood, |udu|+||ou.
||o|oued |,rp|eder+ r+, |e+d |o |o|eque
eu|+|ereu| o| e\||er||,, ep|de|r+| |,pe|p|+|+
W||| .e||uco| (||. oo).
'ecoud+|,, o|| ||ue |u|ec||ou (e|,|pe|+ +ud
ce||u||||) | corrou, |ecu||eu|, +ud |e+d |o
Wo|eu|u o| ||e coud|||ou.
I|e+|reu| | r+|u|, corp|e|ou (+ |u C\|) +ud
r+uu+| |,rp|+||c d|+|u+e, +u||||o||c |u ecoud
+|, |u|ec||ou.
|,rp|+u|o+|cor+ (|u po|r+|ec|or,
|,rp|eder+) | + |+|e corp||c+||ou. '|eW+||
I|e.e ,ud|ore
Chk0NIC IMFhAIIC INSFFICINC
|C|9 . +59.3
|C|0 .|31.2
FICk 16-16 Chrooc |ymphatc
osuIIceocy: |ymphedema |oWe| |e
+|e |||c|eued o| Wood, cou||euc, +ud |||ee
| r+|.e |,pe||e|+|o|. I|e o0,e+|o|d
p+||eu| |+d |+d |uuure|+||e ep|ode o| e|,
|pe|+ +ud ce||u||||. I|e|e | +|o d|+|e|e +ud
+||e|oc|e|o||c +||e||+| |uu|||c|euc,.
SCII0N 16 'K|| '|C|' 0| \A'Cu|Ak ||'u|||C|E|C\ 4TT
||eu|e u|ce| de.e|op +| |od,uppo|| |u|e|
|+ce o.e| |ou, p|or|ueuce + + |eu|| o| e\
|e|u+| corp|e|ou o| ||e ||u, |e+| |o|ce, +ud
|||c||ou, W||c| p|oduce |c|er|c ||ue uec|o|.
0ccu| |u p+||eu| W|o +|e o||uuded reu|+||,
o| |+.e d|r|u||ed eu+||ou (+ |u p|u+| co|d
d|e+e) |u ||e +||ec|ed |e|ou. 'ecoud+|, |u
|ec||ou |eu|| |u |oc+||/ed ce||u||||, W||c| c+u
e\|eud |oc+||, |u|o |oue o| ruc|e o| |u|o ||e
||ood||e+r.
',, . ||eu|e o|e, |ed o|e, decu|||u u|ce|.
FkSSk ICkS |C|9 . 101
|C|0 . |39
FI0MI0I0C
Ae oI 0oset Any age, but the gieatest pieva-
lence of piessuie ulceis is in eldeily, chionically
bediidden patients.
Sex Equally pievalent in both sexes.
Freva|eoce Acute caie hospital setting, 3-14%;
long-teim caie settings, 15-25%; home-caie
settings, 7-12%; spinal coid units, 20-30%.
FAIh0CNSIS
Risk factois: inadequate nuising caie, dimin-
ished sensation/immobility (obtunded mental
status, spinal coid disease), hypotension, fe-
cal oi uiinaiy incontinence, piesence of fiac-
tuie, hypoalbuminemia, and pooi nutiitional
status. The mean skin capillaiy piessuie is
appioximately 25 mmHg. Exteinal compies-
sion with piessuies >30 mmHg occludes the
blood vessels so that the suiiounding tis-
sues become anoxic and eventually neciotic.
Amount of damage is piopoitional to extent
and duiation of piessuie. Secondaiy bacte-
iial infection can enlaige the ulcei, extend
to undeilying stiuctuies (osteomyelitis), and
invade the bloodstieam, with bacteiemia and
septicemia. Infection also impaiis oi pievents
healing.
Ulceis often develop within the fiist 2 weeks of
acute hospitalization and moie iapidly if the
patient expeiiences significant immobilization.
Painful unless theie is alteied sensoiium.
Sko Iesoos C|InIcu| CutegvrIes v] Pressure
U|cers Eaily change: localized eiythema that
blanches on piessuie.
Stage I: Nonblanching eiythema of intact
skin.
Stage II: Neciosis, supeificial oi paitial-thick-
ness involving the epideimis and/oi dei-
mis. Bullae neciosis of deimis (black)
shallow ulcei.
Stage III: Deep neciosis, ciateiifoim ulceia-
tion with full-thickness skin loss (Fig. 16-
17); damage oi neciosis can extend down
to, but not thiough, fascia.
Stage IV: Full-thickness neciosis ( ulceia-
tion) with involvement of suppoiting
stiuctuies such as muscle and bone (Fig.
16-18). May enlaige to many centimeteis.
May oi may not be tendei. Boideis of ul-
ceis may be undeteimined.
Well-established piessuie ulceis aie widest at
the base and tapei to a cone shape at the level
of skin. Ulceis with devitalized tissue at the
base (eschai) have a highei chance of secondaiy
infection. Puiulent exudate and eiythema sui-
iounding the ulcei suggest infection. Foul odoi
suggests anaeiobic infection.
DIstrIhutIvn Occui ovei bony piominences:
sacium (60%) (Fig. 16-18) > ischial tubeiosi-
ties, gieatei tiochantei (Fig. 16-17), heel > el-
bow, knee, ankle, occiput.
Ceoera| xamoatoo Fevei, chills, oi incieased
pain of ulcei suggests possible cellulitis oi os-
teomyelitis.
hemato|oc Studes Elevated white blood
cell count and eiythiocyte sedimentation iate
suggest infection (osteomyelitis oi bacteiemia).
Wouod Cu|ture Infection must be diffeienti-
ated fiom colonization. Cultuie of the ulcei
base detects only suiface bacteiia. Optimal
cultuie technique: Deep poition of punch bi-
opsy specimen obtained fiom the ulcei base is
minced and cultuied foi aeiobic and anaeiobic
bacteiia. Most infections aie polymiciobial and
anaeiobes may be piesent. Viial cultuie to iule
out chionic heipes simplex viius ulcei.
8|ood Cu|ture Bacteiemia often follows ma-
nipulation of ulcei (within 1-20 min of begin-
ning the debiidement); iesolves within 30-60
min.
Fatho|oy S|In BIvpsy Epideimal neciosis
with ecciine duct and gland neciosis. Deep ul-
ceis show wedge-shaped infaicts of the subcuta-
neous tissue, obstiuction of the capillaiies with
miciothiombi, and endothelial cell swelling fol-
lowed by endothelial cell neciosis and second-
aiy inflammation.
Bvne BIvpsy Essential foi diagnosing con-
tinuous osteomyelitis; specimen is examined
histologically and miciobiologically.
Imao It is difficult to distinguish osteomy-
elitis fiom chionic piessuie-ielated changes by
iadiogiam oi scan.
Usually made clinically. Complications aie as-
sessed with data on cultuies, biopsies, and im-
aging. Diffeiential diagnosis includes infectious
ulcei (actinomycotic infection, deep fungal in-
fection, chionic heipetic ulcei), theimal buin,
malignant ulcei (cutaneous lymphoma, basal
cell caicinoma oi SCC), pyodeima gangieno-
sum, iectocutaneous fistula.
C0kS AN0 Fk0CN0SIS
If piessuie is ielieved, some changes aie ie-
veisible; inteimittent peiiods of piessuie ielief
inciease iesistance to compiession. Osteo-
myelitis occuis in nonhealing piessuie ulceis
(32-81%). Septicemia is associated with a high
moitality iate. Oveiall, patients with piessuie
ulceis have a fouifold iisk of piolonged hospi-
talization and of dying when compaied with
patients without ulceis. With piopei tieat-
ment, stages I and II ulceis heal in 1-4 weeks
and stages III and IV ulceis heal in 6 to >12
weeks.
MANACMNI
Frophy|axs o At-ksk Fateots Reposition pa-
tient eveiy 2 h (moie often if possible); massage
aieas pione to piessuie ulceis while changing
position of patient; inspect foi aieas of skin
bieakdown ovei piessuie points.
Use inteiface aii mattiess to ieduce compies-
sion.
Minimize fiiction and sheai foices by using
piopei positioning, tiansfeiiing, and tuining
techniques.
Clean with mild cleansing agents, keeping
skin fiee of uiine and feces.
Minimize skin exposuie to excessive moistuie
fiom incontinence, peispiiation, oi wound
diainage.
Maintain head of the bed at a ielatively low
angle of elevation (<30).
Evaluate and coiiect nutiitional status; con-
sidei supplements of vitamin C and zinc.
Mobilize patients as soon as possible.
Staes I aod II |cers Topical antibiotics (not
neomycin) undei moist steiile gauze may be
sufficient foi eaily eiosions. Noimal saline wet-
to-diy diessings may be needed foi debiide-
ment. Hydiogels oi hydiocolloid diessings.
Staes III aod Iv |cers Suigical management:
debiidement of neciotic tissue, bony piomi-
nence iemoval, flaps and skin giafts.
IoIectous Comp|catoos Piolonged couise of
antimiciobial agent depending on sensitivities,
with suigical debiidement of neciotic bone in
osteomyelitis.
FICk 16-1T Fressure u|cer, stae III we||der+|c+|ed c|+|e|||o|r u|ce| W||| |u|| |||c|ue ||u |o
e\|eud|u doWu |o |+c|+ o.e| |e+|e| ||oc|+u|e||c |e|ou.
FICk 16-18 Fressure u|cer, stae Iv nue ||+c| uec|o| o.e| +c|+| +|e+ |u + p+||eu| W|o |+d |eeu
|ed||ddeu +||e| + ||o|e. I|e c||c|o r+|| |u ||e uec|o||c +|e+ +|e ||or +||erp| |o rec|+u|c+||, de|||de
uec|o||c ||ue. 'u||c+| de|||dereu| o| uec|o||c ||ue uude| +ue||e|+ |e.e+|ed |u.o|.ereu| o| |+c|+ +ud |oue.
480
SkIN SICNS 0F kNAI
INSFFICINC
S E C I | 0 N 1 1
FI0MI0I0C
Ae oI 0oset Middle to old age.
Sex Equal.
FAIh0CNSIS
The pathogenesis is pooily undeistood. While
vasculai calcifications aie common in patients
with chionic ienal failuie and aie asympto-
matic, in calciphylaxis, theie is sudden thiom-
bosis in calcified vessels. In animal models,
calciphylaxis is desciibed as a condition of
induced systemic hypeisensitivity in which
tissues iespond to appiopiiate challenging
agents with calcium deposition. Calciphylaxis is
as sociated with chionic ienal failuie, secondaiy
hypeipaiathyioidism, and an elevated calcium
phosphate end pioduct. Implicated challeng-
ing agents" include glucocoiticoids, albumin
C+|c|p|,|+\| | c|+|+c|e||/ed |, p|o|e|.e cu
|+ueou uec|o| +oc|+|ed W||| r+|| +ud re
d|ur|/ed .ee| c+|c|||c+||ou.
|| occu| |u ||e e|||u o| eud|+e |eu+| d|e+e,
d|+|e|e re||||u, +ud |,pe|p+|+||,|o|d|r.
||e|u|+|c||.e |e|ou |oW ro||||u o| ||.edo
|e||cu|+|| p+||e|u, du|, |ed.
Iu|u |u|o ||+c|, |e+||e|, ec|+|.
E\||ere|, p+|u|u|.
E\|eud |o |+c|+ +ud |e,oud.
|oWe| e\||er|||e, +|doreu, |u||oc|, peu|.
CAICIFhIAXIS |C|9 . 215.+9
infusions, intiamusculai tobiamycin, iion dex-
tian complex, calcium hepaiinate, immunosup-
piessive agents, and vitamin D.
Even eaily infaictive lesions aie exquisitely ten-
dei and painful.
0sease Assocatoos Occuis in end-stage ienal
disease. Onset often closely follows initiation of
hemo- oi peiitoneal dialysis. Most patients aie
diabetic. Hypeipaiathyioidism.
Sko Iesoos Initially pieinfaictive ischemic
plaques occui, appeaiing as mottling oi hav-
ing a livedo ieticulaiis pattein, dusky ied to
violaceous (Fig. 17-1). Bullae may foim ovei
ischemic tissue, which eventually becomes
neciotic. Cential infaicted sites have a tightly
adheient black oi yellowish, leatheiy slough
(Fig. 17-1B). Lesions giadually enlaige ovei
Acu|e |eu+| |+||u|e
Eder+
u|er|c ||o| (depo|||ou o| u|e+ c|,|+| ou ||u
u||+ce |u e.e|e u|er|+)
C||ou|c |eu+| |+||u|e
Eder+
u|er|c ||o|
C+|c|p|,|+\|
Bu||ou d|e+e o| |erod|+|,| (peudopo|
p|,||+, ee 'ec||ou 22)
|ep||oeu|c ||||o|u de|rop+||,
Acqu||ed pe||o|+||u de|r+|o|
CIASSIFICAII0N 0F SkIN ChANCS
SCII0N 1T 'K|| '|C|' 0| kE|A| ||'u|||C|E|C\ 481
FICk 1T-1 Ca|cphy|axs |c|, |c-. Au +|e+ o| ro|||ed e|,||er+, |+||u|||||e +ud |er|u|ceu| o|
||.edo |e||cu|+|| W||| |Wo r+|| u|ce|+||ou. |+||eu| |+ c||ou|c |eu+| |+||u|e +ud | ou |erod|+|,|. E.eu +| |||
e+||, |+e |e|ou +|e e\||ere|, p+|u|u|. C+|c|p|,|+\|, ro|e +d.+uced |e|ou. Au +|e+ o| j+ed uec|o| ou
||e |oWe| |e |u + p+||eu| W||| d|+|e|e +ud c||ou|c |eu+| |+||u|e W|o | ou |erod|+|,|. I|e u||ouud|u ||u |
|udu|+|ed +ud |ep|eeu| + p|+|e|||e u|cu|+ueou r+ ||+| | +pp|ec|+|ed ou|, upou p+|p+||ou.
8
weeks to months; when debiided, deep ulceis
ieaching down to the fascia iesult (Fig. 17-2).
Ischemic skin fiequently becomes secondaiily
infected; infection can iemain localized oi be-
come invasive, causing cellulitis and bacteiemia.
In addition, laige aieas of induiation can be
defined on palpation as platelike subcutaneous
masses that extend beyond infaicted oi ulcei-
ated aieas (Fig. 17-2).
DIstrIhutIvn Distal extiemities, most com-
monly on the lateial and posteiioi calves; abdo-
men, buttocks; fingeis; glans penis.
Chemstry Azotemia. Calcium phosphate
ion pioduct usually elevated.
Farathormooe (FIh) Levels usually, but not
always, elevated.
Cu|tures Rule out secondaiy infection. MRSA
0ermatopatho|oy Incisional, deep biopsy
shows calcification of the media of small-
and medium-sized blood vessels in the deimis
and subcutaneous tissue. Intialuminal fibiin
thiombi aie piesent. Ischemia iesults in intia-
lobulai oi septal fat neciosis, accompanied by a
spaise lymphohistiocytic infiltiate.
Imao Radiogiaphs of affected extiemities
show calcium deposition outlining small and
laige vessels. Miciocalcification of calciphylaxis
is difficult to visualize.
Made on histoiy of ienal failuie, clinical find-
ings, elevated PTH level, elevated calcium
phosphate ion pioduct, and histologic featuies.
0IIereota| 0aooss Panniculitis, vascu-
litides, neciobiosis lipoidica with ulceiation,
dystiophic calcinosis cutis, atheioembolization,
atheioscleiosis obliteians, disseminated intia-
vasculai coagulation (puipuia fulminans), pyo-
deima gangienosum, waifaiin neciosis, hepaiin
neciosis. V|ro u|n[tus cellulitis, othei necio-
tizing cellulitides.
C0kS AN0 Fk0CN0SIS
Slowly piogiessive despite all theiapeutic intei-
ventions. Pain is a constant featuie. In advanced
disease, gangiene of fingeis, toes, and penis may
iesult in autoamputation. Local infection and sep-
sis aie common complications. Oveiall, the piog-
nosis is pooi, and the moitality iate is veiy high.
MANACMNI
Calciphylaxis is best managed by eaily diag-
nosis, tieatment of ienal failuie, paitial pai-
athyioidectomy when indicated, aggiessive
debiidement of neciotic tissue, and avoidance
of piecipitating factois.
FICk 1T-2 Ca|cphy|axs, exteosve |e|ou +|e u|ce|+|ed, ||e u||ouud|u ||u | |udu|+|ed, |e| eeu
ou |e|| |||| W|e|e ||u | |+|||e. '|r||+| |e|ou +|e +|o |ouud ou ||e +|doreu. |o|e eder+ o| |uee +ud
|oWe| |e.
||| | + ||||o|u d|o|de| |u p+||eu| W||| +cu|e
o| c||ou|c |eu+| |+||u|e.
\o| p+||eu| |ece|.|u |erod|+|,|, pe|||oue+|
d|+|,|, |u +cu|e |eu+| |+||u|e ||| occu| W|||ou|
d|+|,|.
|| | p+|| o| + W|de| pec||ur o| -|-:
,|-: || |u.o|.|u ||e |e+||, |uu, d|+
p||+r, |e|e|+| ruc|e, ||.e|, eu||ou||u+|, ||+c|,
+ud ceu||+| ue|.ou ,|er.
E||o|o, uu|uoWu |u| e\pou|e |o +dod|+r|d
cou|+|u|u cou||+| red|+ |o| \k +u|o|+p|, |
+ ||ou +oc|+||ou. C+dod|+r|d | |ouud ou|,
|u |e|ou +ud uo| |u uo|r+| ||ue. \,o||||o
||+| +ud ||||oeu|c c,|o||ue (e.., ||+u|o|r|u
|oW|| |+c|o| ) r+, |e |u.o|.ed |u ||e p+||o
eue|.
||| | c|+|+c|e||/ed |, +cu|e oue|, ||+Wu,
|udu|+||ou, p|+que|||e o| uodu|+|, |ouud doWu
upou p+|p+||ou (||. 1!), up |o 20 cr +ud ro|e
|u d|+re|e|, W||| +u uue.eu ||pp|ed u||+ce.
\o||, ou |oWe| e\||er|||e, |e o||eu ou uppe|
e\||er|||e +ud |o|o |u| uo| ||e |+ce.
I|u||u, |eude|, o||eu p+|u|u|.
||||e|eu||+| d|+uo|. ro|p|e+, p|e||||+| r,\
eder+, ||pode|r+|oc|e|o|, p+uu|cu||||.
Cou|e | c||ou|c, uu|er||||u, p|ouo| u+|ded.
I|e|+p, uu|uoWu. |r+||u|| r+, |e |eue||c|+|
NFhk0CNIC FI8k0SINC 0kM0FAIh (NF0)
FICk 1T-3 Nephroeoc Ibroso dermopathy A ||+Wu, p|+|e|||e |udu|+||ou |ouud doWu upou
p+|p+||ou, W||| +u uue.eu u||+ce ou ||e |e. I|| p+||eu| |+d eud|+e c||ou|c |eu+| |+||u|e +ud W+ ou
|erod|+|,|.
0ccu| |u c||ou|c |eu+| |+||u|e +ud d|+|e|e
re||||u, |u up |o 0 o| p+||eu| uude|o|u
|erod|+|,|.
C||ou|c p|u||||c coud|||ou |||e|ed |, ||+ur+.
ur||||c+|ed p+pu|e W||| ceu||+| |,pe||e|+|o||c
c|u| (||. 1+).
I|+uep|de|r+| e||r|u+||ou o| co||+eu.
ke|+||ou||p |o o||e| pe||o|+||u d|o|de| uo|
c|e+|.
*
'ee \ |e|Wo||, |u K wo||| e| +| (ed). |||/p+|||c|'
|-c||, C--c| !-!:-, 1|| ed. |eW \o||,
\cC|+Wn|||, 2003,). p 5o+.
ACIk0 FkF0kAIINC 0kMAI0SIS (AF0)
FICk 1T-4 Acqured perIorato dermatoss o a pateot uoderoo hemoda|yss I|e|e +|e
pu|pu||c ur||||c+|ed p+pu|e W||| + ceu||+| |,pe||e|+|o||c c|u|.
486
SkIN SICNS 0F SSIMIC
CANCkS
S E C I | 0 N 1 8
0LASS|F|0AT|0h 0F Sk|h S|6hS 0F
SYSTN|0 0Ah08
1
MIASIAIIC CANCkS
Persistent tumor. Lymphatic extension, hema-
togenous spiead
Direct extension. Paget disease, extiamammaiy
Paget disease
Lym|omas w| setonJary s|n no|emen
(Section 20)
hkIIA8I 0IS0k0kS
Cowden syndrome
Peutz-Jeghers syndrome
Neuro[|romaoss (p. 453)
Tu|erous st|eross (p. 449)
Multiple endociine neoplasia (MEN) (types 1
and 2b)
1
Conditions coveied in this section aie piinted in bold, conditions dealt with in othei sections aie in a|ts.
Numbeis in paientheses indicate page numbeis. Raie conditions not discussed in this book aie desciibed in CA
deWitt et al, in K Wolff et al (eds): F:art|'s Dermao|ogy n Cenera| MeJtne 7th ed. New Yoik, McGiaw-
Hill, 2008, pp 1493-1507.
\ucocu|+ueou ||ud|u r+, ue| ,|er|c
c+uce| |u e.e|+| W+,.
Aoc|+||ou o| |e|||+||e rucocu|+ueou d|o|
de| W||| ,|er|c c+uce|.
B, +c||ou +| + d||+uce, |.e., p+|+ueop|+||c ,u
d|ore.
0| p|e+d o| c+uce| |o ||u o| ruco+| ||e |,
d||ec|, |,rp|+||c, o| |er+|oeuou e\|eu|ou
(cu|+ueou re|+|+|).
|C|9 . 99.0
|C|0 . \3000/o
MC0CIAN0S SICNS 0F SSIMIC CANCkS
FAkAN0FIASIIC SN0k0MS
Acquiied ichthyosis
Bazex syndiome
Caicinoid syndiome
Dermaomyoss (p. 14)
Ectopic ACTH syndiome
Eiythema gyiatum iepens
Gaidnei syndiome
Hypeitiichosis lanuginosa
Muii-Toiie syndiome
Palmai keiatoses
Prurus (p. 7)
PyoJerma gangrenosum (p. 7)
Swee synJrome (p. 14)
Vastu|s (p. 397)
SCII0N 18 'K|| '|C|' 0| '\'IE\|C CA|CEk' 48T
\e|+|+||c c+uce| |o ||e ||u | c|+|+c|e||/ed |,
o|||+|, o| ru|||p|e de|r+| o| u|cu|+ueou uod
u|e, occu|||u + re|+|+||c ce|| ||or + d||+u|
uoucou||uou p||r+|, r+||u+u| ueop|+r.
I|e, +|e ||+upo||ed |o +ud depo||ed |u ||e ||u
o| u|cu|+ueou ||ue |, oue o| ||e |o||oW|u
|ou|e.
|,rp|+||c |ou|e.
ner+|oeueou p|e+d.
Cou||uou p|e+d +c|o ||e pe|||oue+| c+.||,
o| o||e| ||ue.
|o| re|+|+| uoure|+uor+ | c+uce| +ud
re|+uor+, ee 'ec||ou +ud 2.
MIASIAIIC CANCk I0 Ih SkIN |C|9 . 99.0
|C|0 . \3000/o
FI0MI0I0C
Ae oI 0oset Any age, but usually oldei.
Sex Fiequency of piimaiy tumois vaiies with
sex.
Iocdeoce In piinciple almost any cancei can
metastasize to skin. Skin metastases occui in
up to 10% of all patients with cancei. The
fiequency of metastases accoiding to type of
tumoi aie shown in Table 18-1.
FAIh0CNSIS
Includes detachment of cancei cells fiom pii-
maiy tumoi, invasion, intiavasation into blood
oi lymphatic vessel, ciiculation, stasis within
vessel, attachment to endothelium, migia-
tion acioss vessel wall, invasion into tissue,
piolifeiation at metastatic site. The giowth of
metastases depends on piolifeiation of meta-
static cells, cytokine and giowth factoi ielease
fiom cancei and stiomal cells, angiogenesis,
and immune ieactions. Thiee patteins of me-
tastases aie obseived: mechanical tumoi stasis
(anatomic pioximity and lymphatic diaining),
site-specific (selective attachment of tumoi cells
to specific oigan), nonselective (independent of
mechanical oi oigan-specific factois).
Piioi histoiy of piimaiy inteinal cancei oi
cancei chemotheiapy oi may be fiist sign of
visceial cancei.
IA8I 18-1 Percent of Patients with Cutaneous Netastases
Pat|eots w|th
Type oI Pr|mary 00taoeo0s
Na||goaocy Netastases, %
\e|+uor+ ++.3
B|e+| !0.0
|++| |uue 20.0
|+|,u\ o.!
Eudoc||ue |+ud 2.5
0|+| c+.||, .5
Eop|+u 3.o
K|due, +.o
'|or+c| 2.0
'0ukCE. Ad+p|ed ||or || |oo||u|||| e| +|. l Ar Ac+d |e|r+|o| 29.223, 99!.
Sko Iesoos Nodule (Figs. 18-1 and 18-2),
iaised plaque, thickened fibiotic aiea. Fiist de-
tected when <5 mm. Fibiotic aiea may iesemble
moiphea; occuiiing on scalp, may pioduce
alopecia. Initially, epideimis is intact, stietched
ovei nodule; in time, suiface may become ulcei-
ated oi hypeikeiatotic (Fig. 18-4). May appeai
inflammatoiy, i.e., pink to ied oi hemoiihagic
(Fig. 18-3). Note: Metastatic melanoma to dei-
mis: blue to giay to black nodules (see Fig.
12-17). Fiim to induiated. May be solitaiy,
few, oi multiple. May acquiie consideiable size
and may be mistaken foi a piimaiy skin cancei
(Fig. 18-4).
DIstrIhutIvn Anywheie; specific sites dis-
cussed below.
Speca| Fatteros oI Cutaoeous Iovo|vemeot
Breust
In[|ammaory measat tartnoma (caici-
noma eiysipelatoides): eiythematous patch
oi plaque with an active spieading boidei
(Fig. 18-5). Most often with bieast can-
cei that may spiead within lymphatics to
skin of involved bieast, iesulting in in-
flammatoiy plaques iesembling eiysipelas
(hence the designation tartnoma erys-
e|aoJes). Bieast most common piimaiy
(Fig. 18-5), but occuis with otheis as well
pancieas, paiotid, tonsils, colon, stomach,
iectum, melanoma, pelvic oigans, ovaiy
(Fig. 18-7), uteius, piostate, lung].
Te|angetat measat tartnoma ( tart-
noma e|angetatum ): bieast cancei ap-
peaiing as pinpoint telangiectases with
dilated capillaiies within caicinoma eiy-
sipelatoides. Violaceous papules oi pa-
pulovesicles iesembling lymphangioma
ciicumsciiptum.
En turasse measat tartnoma : diffuse moi-
phea-like induiation of skin (Fig. 18-6).
Usually local extension of bieast cancei
occuiiing in bieast and piesteinal iegion.
Scleiodeimoid plaque may encase chest
and iesembles a metal bieastplate of a
cuiiassiei. Also occuis with piimaiy of
lung, GI tiact, kidney.
Breas tartnoma o[ n[ramammary trease :
cutaneous exophytic nodule iesembling
piimaiy squamous cell caicinoma (SCC) oi
basal cell caicinoma of skin (Fig. 18-2B).
Page Jsease : shaiply demaicated plaque oi
patch of eiythema and scaling occuiiing
on nipple oi aieola associated with undei-
lying bieast cancei (see below).
FICk 18-1 Metastatc caocer to the sko: broochoeoc caocer |e|r+| uodu|e ou ||e c+|p o| +
p+||eu| uude|o|u c|ero||e|+p, |o| re|+|+||c |uu c+uce|, ||e uodu|e We|e ou|, +pp+|eu| |o||oW|u |o o|
|+|| du||u c|ero||e|+p,. I|e uodu|e ou ||e |e|| | +,rp|or+||c, e|,||er+|ou, |u| uou|u||+red. I|e uodu|e
ou ||e |||| |+ + ceu||+| dep|e|ou r+|||u + puuc| ||op, ||e.
SCII0N 18 'K|| '|C|' 0| '\'IE\|C CA|CEk' 489
FICk 18-2 Metastatc caocer to the sko Adeuoc+|c|uor+ o| ||e co|ou. IWo co+|eceu| roo|| |||r
uodu|e ou ||e +|doreu. 3-c| :c:-. |+|e uodu|e ou ||e+| |u + +0,e+|o|d Wor+u W||| ||e+| c+uce|,
p|eeu| |o| + rou||.

FICk 18-3 Metastatc caocer to the sko,
hyperoephroma n,pe|uep||or+ re|+|+e
o||eu |oc+||/e |o ||e |e+d +ud |+.e +u +u|or+|ou
+ppe+|+uce r|r|c||u p,oeu|c |+uu|or+, + |u |||
|e|ou ou ||e uppe| ||p o| + oo,e+|o|d r+u. I|| W+
||e |||| |ud|c+||ou ||+| ||e p+||eu| |+d c+uce| o| ||e
||due,.
FICk 18-4 Metastatc caocer to the sko B|ouc|oeu|c c+|c|uor+. |+|e u|ce|+|ed, c|u|ed uodu|e
ou ||e c+|p o| + o9,e+|o|d Wor+u W|o |+d |eeu + |e+., ro|e| |uce ||e +e o| 9. I|e p+||eu| W+ o|| e|
W|e +,rp|or+||c +ud ||| |uro| W+ o|||u+||, d|+uoed + p||r+|, qu+rou ce|| c+|c|uor+ o| ||e ||u.
B|op, |e.e+|ed re|+|+| o| rode|+|e|, d|||e|eu||+|ed qu+rou ce|| c+|c|uor+, +ud Wo||up |oWed ||e
p||r+|, c+uce| |o |e |u ||e |uu. Adeuoc+|c|uor+ o| ||e C| ||+c|. I|| |uu+||u r+ W+ ju| ||e ||p o| ||e
|ce|e|. + ruc| |+|e| r+ W+ |u ||e u|cu||.

FICk 18-5 Metastatc caocer oI the sko: oI|ammatory breast caocer (carcooma eryspe|atodes)
A |+|e e|,||er+|ou +ud ou|, r|u|r+||, |udu|+|ed |e|ou co.e||u ||e eu|||e ||e+| +ud p|e|e|u+| |e|ou, ||e
|e|ou | |ed +ud |+|p|, de||ued +ud ||u |oo| |||e e|,|pe|+. I|e|e W+ + 2 2 cr |urp |u ||e ||e+| upou
p+|p+||ou.
Mu||e smoo| noJu|es on sta|: piostate
adenocaicinoma, lung cancei, bieast cancei
(Fig. 18-1).
|oeta neo|asta : On scalp, aieas of haii
loss iesembling alopecia aieata; well-
demaicated, ied-pink, smooth suiface, flat.
Lurge 1ntestIne Often piesents on skin of
abdomen oi peiineal iegions; also, scalp oi
face. Most oiiginate in iectum. May piesent
with metastatic inflammatoiy caicinoma (like
caicinoma eiysipelatoides) of inguinal iegion,
supiaclaviculai aiea, oi face and neck. Less
commonly, sessile oi pedunculated nodules on
buttocks, giouped vasculai nodules of gioin
oi sciotum, oi facial tumoi. Raiely, cutaneous
fistula aftei appendectomy oi iesembling hid-
iadenitis suppuiativa.
Lung CurcInvmu May pioduce a laige
numbei of metastatic nodules in a shoit pe-
iiod. Most commonly, ieddish nodule(s) on
scalp (Figs. 18-1 and 18-4). Tiunk: symmetiic;
along diiection of inteicostal vessels, may be
zosteiifoim; in scai (thoiacotomy site oi needle
aspiiation tiact).
Hypernephrvmu Can pioduce solitaiy lesion;
also widespiead. Usually appeai vasculai, pul-
satile, pedunculated (Fig. 18-3); can iesemble
pyogenic gianuloma. Most common on head
(scalp) and neck; also tiunk and extiemities.
CurcInvmu v] B|udder, Ovury Can spiead
contiguously to abdominal and inguinal skin
similaily to bieast cancei, as desciibed above,
and look like eiysipelas (Fig. 18-7).
Msce||aoeous Fatteros With dilation of lym-
phatics and supeificial hemoiihage, may ie-
semble lymphangioma. With lymph stasis and
deimal edema, iesembles pigskin oi oiange
peel. May metastasize hematogenously to scalp,
foiming many subcutaneous nodules with bag
of maibles" feel to scalp.
Sser Mary Jose| noJu|e is metastatic caici-
noma to umbilicus fiom intiaabdominal caici-
noma, most commonly stomach, colon, ovaiy,
pancieas; howevei, piimaiy may be in bieast.
Easiei to detect by palpation than by visual
detection. Can be fiim to induiated nodule,
fissuiing, ulceiation, vasculai appeaiance,
dischaige. In 15% may be initial piesentation
of piimaiy malignancy.
"8|ueberry MuIIo 8aby" Neuioblastoma,
congenital leukemia.
Mu|tp|e Smooth Nodu|es oo Sca|p Cy l-
indiomas (iaie adnexal tumois of the scalp
mimicking maibles tucked undei the skin),
tiichilemmal (pilai) cysts.
kapos Sarcoma-Ike Iesoos Cancei of kid-
ney.
Fyoeoc Craou|oma-Ike Iesoos Amelanotic
melanoma, ienal cancei.
A|opeca Areata-Ike Iesoos Bieast cancei.
Iymphaooma-Ike Iesoos Cancei of bieast,
lung, ceivix, ovaiy.
Morphea-Ike Iesoos Cancei of bieast, stom-
ach, lung, mixed tumois, laciimal gland.
0ermatopatho|oy At times, cell diffeien-
tiation and aichitectuial stiuctuie sufficient
to piedict piimaiy site; howevei, many times
cells anaplastic. Employ monoclonal antibod-
ies to diffeientiate solid caicinoma metastases
fiom lymphoma, neuioendociine caicinoma,
melanoma, anaplastic angiosaicoma, and sai-
comas.
C0kS AN0 Fk0CN0SIS
In individuals with known cancei, cutaneous me-
tastases aie indicative of a pooi piognosis. Aveiage
suivival aftei detection of cutaneous metastasis
only 3 months except foi contiguous spiead of
bieast cancei, which may last foi yeais. In indi-
viduals with unknown cancei, skin metastases
may help to detect piimaiy tumoi.
MANACMNI
With solitaiy oi few lesions and if patient not
teiminal, excision may be indicated.
FICk 18-T Metastatc breast caocer: caocer eo curasse Bo|| ||e+| +|e |+|d upou p+|p+||ou-|||e
+u +|ro| p|+|e. I|e|e +|e ru|||p|e r+|| +ud |+|e, u|ce|+|ed uodu|e +ud ||e|e | + |+c||ouud o| e|,|pe|+|||e
e|,||er+ (c+|c|uor+ e|,|pe|+|o|de).
FICk 18-6 Metastatc ovarao caocer \+u||e||u + c+|c|uor+ e|,|pe|+|o|de ou ||e |oWe| +|doreu
+ud |uu|u+| |e|ou. wo||up d|c|oed o.+||+u c+uce| W||| pe|||oue+| c+|c|uor+|o|.
FACI 0ISAS |C|9 . 1+
|C|0 . \5+2/!
\+rr+|, |+e| d|e+e (\||) | + r+||u+u|
ueop|+r ||+| uu||+|e|+||, |u.o|.e ||e u|pp|e
o| +|eo|+ +ud |ru|+|e + c||ou|c ec/er+|ou
de|r+||||.
|| |ep|eeu| cou||uou p|e+d o| uude||,|u |u
||+duc|+| c+|c|uor+ o| ||e ||e+| (-+ o| ||e+|
c+uce|).
uu+||, occu|||u |u |er+|e (>50 ,e+|), ||e|e
+|e |+|e e\+rp|e |u r+|e.
0ue| | |u|d|ou o.e| e.e|+| rou|| o| ,e+|.
\+, |e +,rp|or+||c o| ||e|e r+, |e p|u|||u,
p+|u, |u|u|u, d|c|+|e, ||eed|u, u|ce|+||ou,
u|pp|e |u.+|u+||ou.
'||u |e|ou p|eeu| + |ed, c+||u p|+que, |+||e|
|+|p|, r+||u+|ed, o.+| W||| |||eu|+| |o|de|.
w|eu c+|e | |ero.ed, ||e u||+ce | ro|| +ud
oo/|u (||. 33). |e|ou |+ue |u |/e ||or
0.!-5 cr (||. 39). |u e+||, |+e ||e|e | uo
|udu|+||ou o| ||e p|+que, |+|e|, |udu|+||ou +ud |u
|||||+||ou de.e|op +ud uodu|e r+, |e p+|p+|ed |u
||e+|. A| |u|||+| p|eeu|+||ou +u uude||,|u ||e+|
r+ | p+|p+||e |u |eWe| ||+u oue|+|| o| p+
||eu|. \+, |e |||+|e|+|. |,rp| uode re|+|+e
occu| ro|e o||eu W|eu \|| | +oc|+|ed W|||
+u uude||,|u p+|p+||e r+.
||||e|eu||+| d|+uo| |uc|ude ec/er+|ou de|
r+||||, po||+|, |eu|u duc|+| p+p|||or+, u|pp|e
+|eo|+ |e|eu||ou |,pe||e|+|o|, |rpe||o, 'CC |u
||u, |+r|||+| perp||u.
|:c-c| !-c|| | ||- |- | uu+||,
|||+|e|+|, || | W|||ou| +u, |udu|+||ou +ud |epoud
|+p|d|, |o |op|c+| |ucoco|||co|d. |e.e|||e|e,
|e up|c|ou o| |+e| d|e+e || 'ec/er+ pe|
|| |o| >! Wee|. ||+uo| .e||||ed |, ||op,
|oW|u ueop|+||c ce|| |u ep|de|r| |o||oW|u +
p+||ouorou|c p+||e|u o| p|e+d. |e||ue uude|
|,|u |u||+duc|+| c+|c|uor+ |, r+rro|+p|,.
\+u+ereu| cou|| o| u|e|,, |+d|o||e|+p,,
+ud/o| c|ero||e|+p, + |u +u, o||e| ||e+|
c+|c|uor+. |,rp| uode d|ec||ou || |e|ou+|
uode +|e p+|p+||e. ||ouo| .+||e. w|eu
||e+| r+ | uo| p+|p+||e, 92 o| p+||eu| u|
.|.e 5 ,e+| +||e| e\c||ou, 32, 0 ,e+|. w|eu
||e+| r+ | p+|p+||e, !3 u|.|.e 5 ,e+|,
22, 0 ,e+|. ||ouo| Wo|e W|eu ||e|e |
|,rp|+deuop+||,.
MAMMAk FACI 0ISAS
FICk 18-8 Mammary Faet dsease A |+|p|, der+|c+|ed |ed p|+que r|r|c||u ec/er+ o| po||+|
ou ||e u|pp|e. I|e p|+que | |||||, |udu|+|ed +ud ||e|e | |||| c+||u, +u, |ed, ec/er+|||e |e|ou ou ||e u|pp|e
+ud +|eo|+ ||+| doe uo| |epoud |o |op|c+| |ucoco|||co|d |ou|d |e ||op|ed.
FICk 18-9 Mammary Faet dsease A |+|p|, de||ued po||+||o|r p|+que ||+| |+ o||||e|+|ed ||e
+|eo|+ +ud u|pp|e. I|e|e W+ + |urp |u ||e ||e+| +ud + r+|| +\|||+|, r+.
E\||+r+rr+|, |+e| d|e+e (E||) | + ueop|+r
o| ||e +uoeu||+| +ud +\|||+|, ||u, |||o|o|c+||,
|deu||c+| +ud c||u|c+||, |r||+| |o |+e| d|e+e o|
||e ||e+|.
0||eu |ep|eeu||u +u |u||+ep|de|r+| e\|eu|ou o|
+ p||r+|, +deuoc+|c|uor+ o| uude||,|u +poc||ue
|+ud o| o| ||e |oWe| +||o|u|e||u+|, u||u+|,, o|
|er+|e eu||+| ||+c|.
0||eu, |oWe.e|, || | uu+oc|+|ed W||| uude||,|u
c+uce|.
I|e |||oeue| o| E|| uo| uu||o|r. 0ccu| +
+u |u ||u upW+|d e\|eu|ou o| +u |u ||u +deuo
c+|c|uor+ |u deepe| |+ud (25). A||e|u+||.e|,,
E|| r+, |+.e + ru||||oc+| p||r+|, o|||u |u ||e
ep|de|r| +ud || +ppeud+e. |||r+|, |uro| |u
||e +uo|ec|ur c+u +||e W||||u ||e |ec|+| ruco+
o| |u||+ru|+| |+ud.
|u|d|ou oue|, |oW p|e+d, + ||c||u. I|e |e
|ou p|eeu| + e|,||er+|ou p|+que, + c+||u,
+e|o|ou (||. 30), + c|u||u, + e\ud+||ou,
ec/er+|ou+ppe+||u |e|ou |u| |o|de| +|e
|+|p|, de||ued (||. 30, ||. !525), eo|+p||c
cou||u|+||ou. |e|ou |ou|d +|W+, |e ||op|ed.
n||op+||o|o|c+||,, |+e| ce|| +|e d|pe|ed
|e|Weeu |e|+||uoc,|e, occu| |u c|u|e|, e\
|eud doWu |u|o +due\+| ||uc|u|e (|+|| |o|||c|e,
ecc||ue duc|). Adue\+| +deuoc+|c|uor+ | o||eu
|ouud W|eu c+|e|u||, e+|c|ed |o|.
|u pe||ue+|/pe||+u+| E||, uude||,|u c+|c|uor+
|ou|d |e e+|c|ed |o| |, -:|c| -cc|
:|:, !:, |c --c . |u
eu||+| E||, e+|c| |o| uude||,|u c+|c|uor+ |,
:,|:, |c.- ,-|c , |u .u|.+|
E||, |, -|.: -cc| .
||||e|eu||+| d|+uo| |uc|ude +|| |ed p|+que.
ec/er+|ou de|r+||||, ||c|eu |rp|e\ c||ou|cu,
||c|eu c|e|ou e| +||op||cu, ||c|eu p|+uu,
|u|e|||||uou po||+|, Cc!!c |u|e||||o, 'CC |u
||u (e|,|||op|+|+ o| 0ue,|+|), |ur+u p+p|||or+
.||u-|uduced 'CC |u ||u, (+re|+uo||c) upe|||c|+|
p|e+d|u re|+uor+.
E|| | uu+||, ruc| |+|e| ||+u | +pp+|eu| c||u|
c+||,. 'u||c+| e\c||ou ru| |e cou||o||ed |||o|o|
c+||, (\o| r|c|o|+p||c u|e|,). || |+e| ce|| +|e
|u de|r| +ud |e|ou+| |,rp| uode +|e p+|p+||e,
|,rp| uode d|ec||ou r+, |rp|o.e p|ouo|,
W||c| | |e|+|ed |o uude||,|u +deuoc+|c|uor+.
E|| |er+|u |u ||u |u ||e ep|de|r| +ud +due\+|
ep|||e||ur |u >o5 o| c+e. w|eu uo uude||,|u
ueop|+r | p|eeu|, ||e|e | uoue||e|e + |||
|ecu||euce |+|e, e.eu +||e| +pp+|eu||, +dequ+|e
e\c||ou, ||| | due |o ||e ru||||oc+| o|||u |u ||e
ep|de|r| +ud +due\+| ||uc|u|e.
XIkAMAMMAk FACI 0ISAS
SCII0N 18 'K|| '|C|' 0| '\'IE\|C CA|CEk' 49T
FICk 18-10 xtramammary Faet dsease \o||, We||der+|c+|ed, e|oded, oo/|u, e|,||er+|ou
p|+que ou ||e c|o|ur +ud |uu|u+| |o|d |u +u o|de| r+|e. I|e |e|ou | corrou|, r||+|eu |o| Cc!!c |u|e|
|||o +ud uuucce|u||, ||e+|ed + uc|.
CoWdeu ,ud|ore (u+red +||e| ||e p|opo||u)
| + |+|e, +u|oor+| dor|u+u| |e|||+||e c+uce|
,ud|ore W||| .+||+||e e\p|e|.||, |u + uur|e|
o| ,|er |u ||e |o|r o| ru|||p|e |+r+||or+
|ou ueop|+r o| ec|ode|r+|, reode|r+|, +ud
eudode|r+| o|||u.
Ce|r||ue ru|+||ou |u ||e |uro|upp|eo|
eue ||| +|e |oc+|ed ou c||oroore 0q22-
2! |u ro| c+e.
I|e|e | + pec|+| ucep|||||||, |o| ||e+| +ud
||,|o|d c+uce|, +ud ||e ||u |e|ou +|e |rpo||+u|
r+||e|.
'||u |e|ou r+, +ppe+| |||| |u c|||d|ood |u| de
.e|op o.e| ||re. I|e, cou|| o| |:||-c ,
||uco|o|ed, p|u| (||. 33), o| ||oWu p+pu|e
|+.|u ||e +ppe+|+uce o| ||+| W+|| ou ||e ceu||+|
+|e+ o| ||e |+ce, pe||o|+| +|e+, ||p ue+| ||e
+u|e o| ||e rou||, +ud ||e e+|, |c|:-|
:|c|- |-c|- o| ||e p+|r +ud o|e, +ud
|,-|-c||: ||c||-! c|- ou ||e do|+
o| ||e |+ud +ud |o|e+|r. \ucou rer||+ue
|e|ou +|e c|+|+c|e||||c. c|- o| ||e |u|.+|,
|+||+| (||. 31), +ud p+|+|+| u||+ce ||+|
co+|ece, |.|u + 'co|||e|oue +ppe+|+uce. |c
||c o| ||e |ucc+| ruco+ +ud ||e |ouue.
|u +dd|||ou |o ||e+| c+uce| (20), W||c| | o||eu
|||+|e|+|, +ud ||,|o|d c+uce| (3), ||e|e +|e .+||
ou |u|e|u+| |+r+||or+.
3-c|. ||||oc,||c d|e+e, ||||o+deuor+, +d
euoc+|c|uor+, ,uecor+||+ |u r+|e
|,!. o||e|, +deuor+, ||,|o|o+| duc|
c,|, |o|||cu|+| +deuoc+|c|uor+
C| |c:|. |+r+||or+|ou po|,p |||ou|ou|
||+c| |u| |uc|e+ed |u |+|e |oWe|, +deuoc+|c|
uor+ +|||u |u po|,p
|-c|- -|c| |c:|. o.+||+u c,|, reu||u+|
+|uo|r+||||e
!:||-|-|c|. c|+u|ore+|,, |,p|oco||o|,
'+deuo|d |+c|e, |||+|c|ed p+|+|e
C|' . reu|+| |e|+|d+||ou, e|/u|e, ueu|or+,
+u||oueu|or+, +ud reu|u|or+ o| ||e e+|
c+u+|.
|| | |rpo||+u| |o e|+|||| ||e d|+uo| o| CoWdeu
,ud|ore o ||+| ||ee p+||eu| c+u |e |o||oWed
c+|e|u||, |o de|ec| ||e+| +ud ||,|o|d c+uce|.
|C|9 . 159.o
|C|0 . 035.9
C0W0N SN0k0M (MIIIFI hAMAkI0MA
SN0k0M)
|eu|/le|e| ,ud|ore (|l') | + |+r|||+| (+u
|oor+| dor|u+u|, pou|+ueou ru|+||ou |u
+0) po|,po| c|+|+c|e||/ed |, r+u, r+||,
p|reu|ed ||oWu r+cu|e (|eu|||ue) ou ||e
||p, o|+| rucou rer||+ue (||oWu |o ||u||
||+c|), +ud ou ||e |||de o| ||e uoe, p+|r, +ud
o|e.
I|e eue |+ |eeu r+pped |o 9p!.!.
\+cu|e ou ||e ||p r+, d|+ppe+| o.e| ||re, |u|
uo| ||e p|reu|+||ou o| ||e rou||, ||e|e|o|e ||e
rou|| p|reu|+||ou | ||e |ue qu+ uou |o| ||e
d|+uo| (||. 32).
I|e|e +|e uu+||,, |u| uo| +|W+,, ru|||p|e |+r+|
|or+|ou po|,p |u ||e r+|| |oWe|, + We||
+ |u ||e |+|e |oWe| +ud |or+c|, ||+| c+ue
+|dor|u+| ,rp|or uc| + p+|u, C| ||eed|u,
+uer|+.
w|e|e+ p|reu|ed r+cu|e +|e coueu||+| o|
de.e|op |u |u|+uc, +ud e+||, c|||d|ood, po|,p
core ou |u |+|e c|||d|ood o| |e|o|e +e !0.
Adeuoc+|c|uor+ r+, de.e|op |u po|,p, +ud
||e|e | +u |uc|e+ed |uc|deuce o| ||e+|, o.+||+u,
+ud p+uc|e+||c c+uce|.
I|e|e | + uo|r+| |||e e\pec|+uc, uu|e c+|c|uor+
de.e|op |u ||e C| ||+c|. \+||u+u| ueop|+r
r+, |e ro|e ||equeu| |u l+p+uee p+||eu| W|||
||| ,ud|ore, +ud p|op|,|+c||c co|ec|or, |+
|eeu |ecorreuded |o| ||ee p+||eu|.
FII-lChkS SN0k0M |C|9 . 159.o
|C|0 . 035.3
FICk 18-11 Cowdeo syodrome \u|||p|e
|edd||, cou||ueu| p+pu|e ou ||e o|+| ruco+ |.|u
+ co|||e|oue +ppe+|+uce. \u|||p|e ||uco|o|ed
W+||, p+pu|e ou ||e |+ce, W||c| |ep|eeu| |||c|o
|errou+.
FICk 18-12 Feutt-lehers syo-
drome \u|||p|e, d+||||oWu |eu|||ue
ou ||e .e|r|||ou |o|de| o| ||e ||p +ud ||e
|ucc+| ruco+. I|| p+||eu| |+d C| ||eed|u
due |o |+r+||or+|ou po|,p |u ||e r+||
|oWe|.
C|uc+ouor+ ,ud|ore | + |+|e |u| We||de
c|||ed c||u|c+| eu|||, c+ued |, e\ce|.e p|o
duc||ou o| |uc+ou |u +u ce|| |uro| o| ||e
p+uc|e+.
C|+|+c|e||/ed |, upe|||c|+| r||+|o|, uec|o|,||c
e|,||er+ (\|E) W||| e|o|ou ||+| c|u| +ud |e+|
W||| |,pe|p|reu|+||ou.
|u||+rr+|o|, p+|c|e +ud |ed p|+que (||.
3! +ud 3+) o| ,|+|e, c||c|u+|e, +|cu+|e, o|
+uuu|+| |+pe ||+| eu|+|e W||| ceu||+| c|e+||u,
|eu|||u |u eo|+p||c +|e+ ||+| |ecore cou
||ueu| (||. 3+). Bo|de| |oW .e|cu|+||ou |o
|u||+ |o|r+||ou, c|u||u, +ud c+||u (||. 3!
+ud 3+).
|e|ou |u.o|.e pe||o|+| +ud pe||eu||+| |e|ou
+ud ||e\u|e +ud |u|e|||||u+| +|e+.
||ue|||p |ed, ||u|u, e|o|.e (||. 35).
I|e|e | |o|||, +uu|+| c|e||||| (||. 3!),
||ep|+||||.
Ceue|+| e\+r|u+||ou |e.e+| W+||u, r+|uu|||
||ou.
\o| c+e +|e +oc|+|ed W||| |uc+ouor+, |u|
||e p+||oeue| o| \|E | uo| |uoWu. I|e|e
e\|| \|E W|||ou| |uc+ouor+.
|||--|c| !c. |uc|ude +|| ro|| |ed
p|+que(). +c|ode|r+|||| eu|e|op+|||c+, /|uc
de||c|euc,, pu|u|+| po||+|, rucocu|+ueou
c+ud|d|+|, n+||e,n+||e, d|e+e (|+r|||+| per
p||u).
|c|c|,. |+||u p|+r+ |uc+ou |e.e| |u
c|e+ed |o >000 u/| (uo|r+| 50-250 u/|) +ud
r+|e ||e d|+uo|. I|e|e | +|o |,pe||,cer|+,
|educed |ucoe |o|e|+uce. Aoc|+|ed ||ud|u
|uc|ude e.e|e r+|+|o|p||ou, |o |,po+r|
uo+c|der|+, |oW e|ur /|uc. CI c+u +u|o|+p|,
W||| |oc+|e |uro| W||||u p+uc|e+ +ud re|+|+e
|u ||e ||.e|.
|e|r+|op+||o|o, o| e+||, ||u |e|ou |oW
|+ud|||e uppe| ep|de|r+| uec|o| W||| |e|eu||ou
o| p,|uo||c uuc|e| +ud p+|e |e|+||uoc,|e c,|o
p|+r.
||ouo| depeud ou ||e +|e|.eue o| ||e
|uc+ouor+. nep+||c re|+|+e |+.e occu||ed
|u 15 o| p+||eu| +| ||e ||re o| d|+uo|. ||
||ee +|e |oW|oW|u, p+||eu| r+, |+.e p|o
|oued u|.|.+|, e.eu W||| re|+|+||c d|e+e.
\|E |epoud poo||, |o +|| |,pe o| ||e|+p,.
'ore c+e |+.e |epouded p+|||+||, |o /|uc
|ep|+cereu|. \|E |eo|.e +||e| |uro| e\c|
|ou. noWe.e|, u||c+| e\c||ou o| |uc+ouor+
+c||e.e cu|e |u ou|, !0 o| c+e |ec+ue o|
pe|||eu| re|+|+e (uu+||, ||.e|). 'u|e|, +|o
|educe |uro| r+e +ud +oc|+|ed ,rp|or.
I|e|e | poo| |epoue |o c|ero||e|+p,.
CICAC0N0MA SN0k0M |C|9 . 2.1
|C|0 . \352/0
FICk 18-13 C|ucaoooma
syodrome: mratory oecro|ytc
erythema |u||+rr+|o|, de|r+
|o| W||| +uu|+| c|e|||||, |u||+r
r+|o|,, c+|,, e|o|.e +ud c|u|ed
p|+que +ud ||u|e +|ouud ||e
uoe, rou||, +ud red|+| +pec| o|
||e e,e. \+||u+| ||ep|+||||.
FICk 18-14 C|ucaoooma syodrome: mratory oecro|ytc erythema |o|,c,c||c e|o|ou |u ||e
+uoeu||+| |u|e+| +ud +c|+| |e|ou. '|+|p|, de||ued W||| uec|o||c ||+cc|d ep|de|r| |||| co.e||u p+|| o| ||ee
e|o|ou.
FICk 18-15 C|ucaoooma syodrome ||ue|||p +|e |ed, |||eu|u +ud p+|||+||, e|o|.e.
|||e o||e| |o|r o| +c+u||o| u|||c+u (A|)
(ee 'ec||ou 5), r+||u+u| A| |+|| + + d|||ue,
.e|.e|, |||c|eu|u +ud |,pe|p|reu|+||ou c||e||,
ou ||e uec|, +\|||+e +ud o||e| |od, |o|d, +
We|| + ou ||e pe||o|+| +ud pe||o||||+|, ur||||c+|,
r+r|||+|,, +ud eu||+| +|e+, |.|u ||e ||u + d|||,
+ppe+|+uce (||. 3o, ee +|o ||. 5).
n,pe|p|reu|+||ou +ud |,pe||e|+|o| oou |e+d
|o + |uoe, r+r|||+|ed, +ud p+p|||or+|ou u|
|+ce (||. 3o).
\e||ucou |oW|| +|o |u.o|.e ||e .e|r|||ou
|o|de| o| ||e ||p (||. 31). 0u ||e o|+| rucou
rer||+ue ||e|e | + .e|.e|, |e\|u|e W||| de||c+|e
|u||oW.
I|e |uuc||e +ud ||e p+|r |oW r+\|r+| +c
ceu|u+||ou o| ||e p+|r+| ||de (|||pe |+ud)
(||. 33).
\+||u+u| A| d|||e| ||or o||e| |o|r o| A|
p||r+|||, |ec+ue o| () ||e ro|e p|ououuced
.e|.e|, |,pe||e|+|o| +ud |,pe|p|reu|+||ou,
(2) ||e p|ououuced ruco+| |u.o|.ereu| +ud
|u.o|.ereu| o| ||e rucocu|+ueou juuc||ou, (!)
|||pe |+ud, +ud (+) We||| |o +ud W+||u due
|o ||e uude||,|u r+||u+uc,.
A| r+, p|ecede |, 5 ,e+| o||e| ,rp|or o| +
r+||u+uc,, uu+||, +deuoc+|c|uor+ o| ||e C| o|
Cu ||+c|, ||ouc|oc+|c|uor+, o|, |e corrou|,,
|,rp|or+. \+||u+u| A| | + ||u|, p+|+ueop|+||c
d|e+e, +ud + e+|c| |o| uude||,|u r+||u+uc|e
| |rpe|+||.e. kero.+| o| r+||u+uc, | |o||oWed
|, |e|e|ou o| A|.
MAIICNANI ACANIh0SIS NICkICANS |C|9 . 10.2
|C|0 . |3!
FICk 18-16 Acaothoss orcaos: ma|oaot |oo||, de||ued, .e|.e|,, .e||ucou +ud p+p|||or+|ou,
d+|| c|oco|+|e||oWu p|+que ou ||e red|+| |||| +ud c|o|ur. '|r||+| c|+ue We|e +|o p|eeu| |u ||e +\|||+e
+ud uec|, +ud ||e .e|r|||ou |o|de| o| ||e ||p W+ co.e|ed W||| .e|.e|,, |+p|e||,|||e |oW||.
FICk 18-1T Acaothoss
orcaos: ma|oaot \e|
|ucou +ud r+r|||+|ed |oW||
ou ||e .e|r|||ou |o|de| o| ||e
||p |u + p+||eu| W||| c+|c|uor+ o|
||e |or+c|. I|e +|||c c+uce|
W+ upec|ed |ec+ue o| ||ee
|+p|e||,|||e |oW||, +c+u||o|
u|||c+u o| ||e r+jo| ||u |o|d,
+ud We||| |o. I|e|e | |||| +
u|u|e +| ||e ||e o| + ||op,.
\ucou rer||+ue p||r+|||, +ud ro| e.e|e|,
|u.o|.ed.
|e|ou cor||ue |e+|u|e o| perp||u .u|+||
(p+e 0o) +ud e|,||er+ ru||||o|re (p+e +3),
c||u|c+||,, |||o|o|c+||,, +ud |rruuop+||o|o|c+||,.
\o| p|or|ueu| c||u|c+| ||ud|u cou|| o| e.e|e
o|+| (||. 39) +ud coujuuc||.+| e|o|ou |u +
p+||eu| W||| +u uude||,|u ueop|+r.
I|ee ueop|+r +|e |u o|de| o| ||equeuc,. uou
nod||u |,rp|or+, c||ou|c |,rp|+||c |eu|er|+,
C+||er+u d|e+e, ||,ror+, +|cor+, +ud w+|
deu||or r+c|o|o|u||uer|+.
|+||eu| W||| ||| r+, +|o |+.e c||u|c+| +ud
e|o|o|c e.|deuce o| r,+||eu|+ |+.| +ud +u
|o|rruue c,|opeu|+.
||| e|+ cou|+|u +u|o+u|||od|e |o p|+||u +u||
eu (|u ||e |u|e|ce||u|+| p|+que o| deroore),
eu.op|+||u +ud pe||p|+||u, +ud |o derop|+||u
| +ud ||. |e corrou|, p+||eu| e|+ r+, +|o
|ecou|/e |u||ou perp||o|d +u||eu (2!0 ||+),
p|ec||u, +ud p|+|o|o||u, +ud +u uu|deu||||ed 10
||+ +u||eu.
Au|o+u|||od|e o| ||| c+ue ||||e||u |u ueo
u+|+| r|ce +ud +|e de|ec|ed |, |ud||ec| |r
ruuo||uo|eceuce ou |odeu| u||u+|, ||+dde|
ep|||e||ur.
I|e+|reu| | d||ec|ed |oW+|d e||r|u+||ou o| up
p|e|ou o| r+||u+uc, |u| r+, +|o |equ||e
,|er|c |ucoco|||co|d.
FAkAN0FIASIIC FMFhICS (FNF) |C|9 . o9+.+
FICk 18-18 Acaothoss orcaos: trpe
pa|m I|e p+|r+| ||de o| ||e p+|r |oW r+\|r+|
+cceu|u+||ou, ||u |eer|||u ||e ruco+ o| ||e
|or+c| o| + |ur|u+u| (|||pe p+|r).
FICk 18-19 Faraoeop|astc pemphus
'e.e|e e|o|ou co.e||u p|+c||c+||, ||e eu|||e ruco+
o| ||e o|+| c+.||, W||| p+|||+| p+||u o| ||e do|ur o|
||e |ouue. |e|ou +|e e\||ere|, p+|u|u|, |u|e||e||u
W||| +dequ+|e |ood |u|+|e. I|| p+||eu| |+d uou
nod||u |,rp|or+ + uude||,|u r+||u+uc,.
504
SkIN SICNS 0F
hMAI0I0CIC 0ISAS
S E C I | 0 N 1 9
FI0MI0I0C
Ae oI 0oset Acute idiopathic thiombocyto-
penic puipuia (ITP) mostly in childien; diug-
induced and autoimmune TP in adults.
Sex Both sexes; HIV-associated TP-homo-
sexual men > heteiosexual females.
Due to eithei decieased platelet pioduction,
splenic sequestiation, oi incieased platelet
destiuction.
1. DetreaseJ |ae|e roJuton . Diiect injuiy to
bone maiiow, diugs (cytosine aiabinoside,
daunoiubicin, cyclophosphamide, busulfan,
methotiexate, 6-meicaptopuiine, vinca alka-
loids, thiazide diuietics, ethanol, estiogens),
ieplacement of bone maiiow, aplastic ane-
mia, vitamin deficiencies, Wiskott-Aldiich
syndiome.
2. S|ent sequesraon . Splenomegaly, hypo-
theimia.
3. IntreaseJ |ae|e Jesruton. Immuno|ogt :
autoimmune TP, diug hypeisensitivity
(sulfonamides, quinine, quinidine, cai-
bamazepine, digitoxin, methyldopa), aftei
tiansfusion. Nonmmuno|ogt: infection,
piosthetic heait valves, disseminated intia-
vasculai coagulation, thiombotic thiombo-
cytopenic puipuia.
Platelet plugs by themselves effectively stop
bleeding fiom capillaiies and small blood
I||or|oc,|opeu|c pu|pu|+ (I|) | c|+|+c|e||/ed
|, cu|+ueou |ero|||+e occu|||u |u +oc|
+||ou W||| + |educed p|+|e|e| couu|.
nero|||+e +|e uu+||, r+|| (pe|ec||+e) |u| +|
||re |+|e| (ecc|,roe).
0ccu| +| ||e o| r|uo| ||+ur+/p|eu|e (p|+|e|e|
couu| +0,000/|) o| pou|+ueou|, (p|+|e|e|
couu| 0,000/|).
|C|9 . 231.!
|C|0 . |o9.!
Ihk0M80CI0FNIC FkFkA
vessels but aie incapable of stopping hem-
oiihage fiom laigei vessels. Platelet defects
theiefoie pioduce pioblems with small-vessel
hemostasis, small hemoiihages in the skin oi
in the CNS.
Usually sudden appeaiance of asymptomatic
hemoiihagic skin and/oi mucosal lesions.
Sko Iesoos Peet|ae -small (pinpoint
to pinhead), ied, nonblanching macules that
aie not palpable and tuin biown as they get
oldei (Fig. 19-1); latei acquiiing a yellowish-
gieen tinge. Ett|ymoses -black-and-blue spots;
laigei aiea of hemoiihage. V|tes -lineai
hemoiihages (Fig. 19-1), due to tiauma oi
piessuie. Most common on legs and uppei
tiunk, but may be anywheie.
Mucous Membraoes Peet|ae -most often on
palate (Fig. 19-2), gingival bleeding.
Ceoera| xamoatoo Possible CNS hemoi-
ihage, anemia.
hemato|oy Thiombocytopenia.
8ooe Marrow Aspratoo Defines state of
platelet pioduction.
Sero|oy Rule out HIV disease.
Iesooa| Sko 8opsy May be contiaindicated
due to postopeiative hemoiihage; howevei,
SCII0N 19 'K|| '|C|' 0| nE\AI0|0C|C ||'EA'E 505
FICk 19-1 Ihrombocytopeoc
purpura \u|||p|e pe|ec||+e ou ||e
uppe| +|r o| +u n|\|u|ec|ed 25,e+|o|d
r+|e We|e ||e p|eeu||u r+u||e|+||ou
o| || d|e+e. I|e ||ue+| +||+uereu| o|
pe|ec||+e +| ||e ||e o| r|uo| ||+ur+ +|e
c+||ed .|||ce.
FICk 19-2 Ihrombocytopeoc
purpura C+u |||| r+u||e| ou ||e o|+|
ruco+ o| coujuuc||.+. ne|e ru|||p|e
pe|ec||+| |ero|||+e +|e eeu ou ||e
p+|+|e.
FI0MI0I0C
Ae oI 0oset All ages; occuis in childien.
Antecedent oi concomitant infections due
to bacteiia (scailet fevei, gioup A stiepto-
coccal, staphylococcal, pneumococcal, vibiio,
and meningococcal bacteiemia; less commonly
vaiicella.)
Eens |a nae DIC Tumoi pioducts,
ciushing tiauma, extensive suigeiy, seveie
intiacianial damage; ietained contiaception
pioducts, placental abiuption, amniotic fluid
embolism; ceitain snake bites; hemolytic
||er|u+|ed |u||+.+cu|+| co+u|+||ou (||C) |
+ W|dep|e+d ||ood c|o|||u d|o|de| occu|||u
W||||u ||ood .ee|.
Aoc|+|ed W||| + W|de |+ue o| c||u|c+| c||cur
|+uce. |+c|e||+| ep|, o||e|||c corp||c+||ou,
d|er|u+|ed r+||u+uc,, r+|.e ||+ur+.
\+u||e|ed |, pu|pu|+ |u|r|u+u (cu|+ueou |u|
+|c||ou +ud/o| +c|+| +u|eue) o| ||eed|u ||or
ru|||p|e ||e.
I|e pec||ur o| c||u|c+| ,rp|or +oc|+|ed
W||| ||C |+ue ||or |e|+||.e|, r||d +ud u|c||u|
c+| |o e\p|o|.e +ud |||e|||e+|eu|u.
',, . |u|pu|+ |u|r|u+u, couurp||ou co
+u|op+||,, de|||||u+||ou ,ud|ore, co+u|+||ou
|||||uo|,||c ,ud|ore.
|C|9 . 25o.3
|C|0 . |o5
0ISSMINAI0 INIkAvASCIAk C0ACIAII0N
tiansfusion ieaction; acute piomyelocytic
leukemia; buin injuiies.
Exense Jesruton o[ enJo|e|a| sur[ates,
exosure o [oregn sur[ates Vasculitis in Rocky
Mountain spotted fevei, meningococcemia,
oi occasionally giam-negative septicemia;
gioup A stieptococcal infection, heat stioke,
malignant hypeitheimia; extensive pump
oxygenation (iepaii of aoitic aneuiysm); ec-
lampsia, pieeclampsia; tufted angioma and
Kaposifoim hemangioendothelioma: Kasa-
bach-Meiiitt syndiome; immune complexes;
postvaiicella puipuia gangienosa.
Eens |a tom|tae anJ roagae DIC
Shock, complement pathway activation.
Uncontiolled activation of coagulation iesults
in thiombosis and consumption of platelets/
usually can be contiolled by sutuiing biopsied
site and applying piessuie.
0IACN0SIS
Clinical suspicion confiimed by platelet count.
Noohemorrhac 8|aocho vascu|ar Iesoos
Telangiectasia/eiythema, spidei nevi, Oslei
disease.
Irue hemorrhac Iesoos Actinic oi senile
puipuia, puipuia of scuivy, piogiessive pig-
mentaiy puipuia (Schambeig disease), puipuia
following seveie Valsalva maneuvei (cough-
ing, vomiting/ietching), tiaumatic puipuia,
factitial oi iatiogenic puipuia, Gaidnei-Dia-
mond syndiome (autoeiythiocyte sensitization
syndiome), palpable nonblanching puipuia
astu|s .
C0kS AN0 Fk0CN0SIS
Depends on and vaiies with the etiology.
MANACMNI
Identify undeilying cause and coiiect, if pos-
sible. If platelet count is veiy low (< 10,000/L),
bed iest to ieduce iisk of hemoiihage.
0ra| C|ucocortcods, hh-0ose Iv Immuoo|ob-
u|os
F|ate|et IraosIusoos If the platelet count
< 10,000/L, platelet tiansfusion may be indi-
cated.
Chrooc IIF Splenectomy may be indicated.
SCII0N 19 'K|| '|C|' 0| nE\AI0|0C|C ||'EA'E 50T
clotting factois II, V, VIII. Secondaiy fibii-
nolysis. If the activation occuis slowly, excess
activated pioducts aie pioduced, piedisposing
to vasculai infaictions/venous thiombosis. If
the onset is acute, hemoiihage suiiounding
wound sites and IV lines/catheteis oi bleeding
into deep tissues is usually seen.
Houis to days; iapid evolution. Fevei, chills
associated with onset of hemoiihagic lesions.
Sko Iesoos In[arton (urura [u|mnans)
(Figs. 19-3, 19-4, and 19-5): massive ecchymoses
with shaip, iiiegulai (geogiaphic") boideis
FICk 19-3 0ssemoated otravascu|ar coau|atoo: purpura Iu|moaos E\|eu|.e eo|+p||c +|e+
o| cu|+ueou |u|+|c||ou W||| |ero|||+e |u.o|.|u ||e |+ud. '|r||+| |e|ou We|e ou ||e |+ce, ||e o||e| |+ud, +ud
||e |ee|.
FICk 19-4 0ssemoated
otravascu|ar coau|atoo:
purpura Iu|moaos Ceo|+p||c
cu|+ueou |u| +|c||ou ou ||e c|e|,
|e|ou We|e +|o p|eeu| ou ||e
|+ud, e||oW, ||||, +ud |ee|. I|e
p+||eu| W+ + d|+|e||c W||| '|c|,|
::: c- ep|.
piolonged piothiombin time, paitial thiombo-
plastin time, and thiombin time.
8|ood Cu|ture Foi bacteiial sepsis.
Clinical suspicion confiimed by coagulation
studies. Diffeiential diagnosis of |arge tuaneous
n[artons : neciosis aftei initiation of waifaiin
theiapy, hepaiin neciosis, calciphylaxis, atheio-
embolization.
C0kS AN0 Fk0CN0SIS
Moitality iate is high. Suiviving patients ie-
quiie skin giafts oi amputation foi gangienous
tissue. Common complications: seveie bleeding,
thiombosis, tissue ischemia/neciosis, hemolysis,
oigan failuie.
MANACMNI
Coiiect ieveisible cause. Vigoious antibiotic
theiapy foi infections. Contiol bleeding oi
thiombosis: hepaiin, pentoxifylline, piotein
C concentiate. Pievent iecuiience in chionic
DIC.
with deep puiple to blue coloi (Fig. 19-5) and
eiythematous halo, evolution to hemoiihagic
bullae and blue to black gangiene (Fig. 19-4);
multiple lesions aie often symmetiic; distal
extiemities, aieas of piessuie; lips, eais, nose,
tiunk; peiipheial aciocyanosis followed by
gangiene on hands, feet, tip of nose, with
subsequent autoamputation if patient suivives.
Hemorr|age fiom multiple cutaneous sites, i.e.,
suigical incisions, venipunctuie oi cathetei sites.
Mucous Membraoes Hemoiihage fiom gingiva.
Ceoera| xamoatoo High fevei, tachycaidia,
shock. Multitude of findings depending on
the associated medical/suigical pioblem.
0ermatopatho|oy Occlusion of aiteiioles
with fibiin thiombi. Dense PMN infiltiate
aiound infaict and massive hemoiihage.
hemato|oc Studes CBC Schistocytes
(fiagmented RBCs), aiising fiom RBC entiap-
ment and damage within fibiin thiombi, seen
on blood smeai; platelet count low. Leukocy-
tosis.
Cvugu|utIvn StudIes Reduced plasma fi-
biinogen; elevated fibiin degiadation pioducts;
FICk 19-5 xteosve cutaoeous oIarctoo wth hemorrhae ovo|vo the eotre |e I|| c+|+
||op||c e.eu| |o||oWed ep| +||e| +|dor|u+| u|e|,.
C|,o|o|u||uer|+ (CC) | ||e p|eeuce o| e|ur
|rruuo|o|u||u (p|ec|p||+|e +| |oW |erpe|+|u|e
+ud |ed|o|.e +| !1C) corp|e\ed W||| o||e|
|rruuo|o|u||u o| p|o|e|u.
Aoc|+|ed c||u|c+| ||ud|u |uc|ude pu|pu|+ |u
co|de\poed ||e, k+,u+ud p|euoreuou, co|d
u|||c+||+, +c|+| |ero|||+|c uec|o|, ||eed|u
d|o|de|, .+cu||||, +||||+||+, ueu|o|o|c r+u|
|e|+||ou, |ep+|op|euore+|,, +ud |ore|u
|ouep|||||.
||ec|p||+||ou o| c|,o|o|u||u (W|eu p|eeu| |u
|+|e +rouu|) c+ue .ee| occ|u|ou, +|o +
oc|+|ed W||| |,pe|.|co||,.
|rruue corp|e\ depo|||ou |o||oWed |, corp|e
reu| +c||.+||ou +ud |u||+rr+||ou.
||+|e|e| +|e+||ou/couurp||ou o| c|o|||u |+c|o|
|, c|,o|o|u||u, c+u|u co+u|+||ou d|o|de|.
'r+||.ee| |||or|oe +ud .+cu|||| p|oduced
|, |rruue corp|e\e.
Ck0CI08IINMIA |C|9 . 21!.2
|C|0 . |39.
Tye I Cryog|o|u|ns : Monoclonal immu-
noglobulins (IgM, IgG, IgA, light chains).
ssotaeJ w| : plasma cell dysciasias such
as multiple myeloma, Waldenstim mac-
ioglobulinemia, lymphopiolifeiative dis-
oideis such as B cell lymphoma.
Tye II Cryog|o|u|ns: Mixed ciyoglobulins:
two immunoglobulin components, one of
which is monoclonal (usually IgG, less of-
ten IgM) and one polyclonal; components
inteiact and ciyopiecipitate. ssotaeJ
w| : multiple myeloma, Waldenstim
macioglobulinemia, chionic lymphocytic
leukemia; iheumatoid aithiitis, systemic
lupus eiythematosus, Sjgien syndiome.
Tye III Cryog|o|u|ns : Polyclonal immu-
noglobulins that foim ciyopiecipitate with
polyclonal IgG oi a nonimmunoglobulin
seium component occasionally mixed with
complement and lipopioteins. Repiesents
immune complex disease. ssotaeJ w| :
autoimmune diseases; connective tissue
diseases; wide vaiiety of infectious diseases,
i.e., hepatitis B, hepatitis C, Epstein-Baii
viius infection, cytomegaloviius infection,
subacute bacteiial endocaiditis, lepiosy,
syphilis, stieptococcal infections.
Theie is cold sensitivity in <50% of cases.
Chills, fevei, dyspnea, diaiihea may occui fol-
lowing cold exposuie. Puipuia also may follow
long peiiods of standing oi sitting. Due to othei
oigan system involvement, aithialgia, ienal
symptoms, neuiologic symptoms, abdominal
pain, aiteiial thiombosis.
Nonn[|ammaory urura (usually type I),
occuiiing at cold-exposed sites, e.g., helix
(Fig. 19-6), tip of nose.
trotyanoss and RaynauJ |enomenon , with
oi without seveie iesultant gangiene of fin-
geitips and toes oi elsewheie on aims oi legs
(usually types I oi II) (Fig. 19-7).
Pa|a||e urura with bullae and necioses
(usually types II and III) due to hypeisensitivity
FICk 19-6 Cryo|obu|oema: moooc|ooa|
(type I) I|| uou|u||+red, pu|pu||c |e|ou ou ||e
|e||\ +ppe+|ed ou ||e |||| co|d d+, |u ||e |+||.
FICk 19-T Cryo|obu|oema: mxed (type II) E\|eu|.e uec|o| +ud |ero|||+e ou ||e ||u o|
||e |o|e+|r. I|e|e W+ +|o d|||+| +u|eue ou |+ud +ud |ee|. E\|eu|.e |ero|||+|c uec|o| ou |o||
|e. I|e|e W+ +|o +c|+| +u|eue ou |ou| |oe.

FICk 19-8 Cryo|obu|oema: po|yc|ooa| (type III) |+|p+||e pu|pu|+ W||| W|dep|e+d |ero|||+e
||||e| |o|r+||ou +ud uec|o| + |u +u, o||e| |,pe o| |,pe|eu|||.||, .+cu|||| (corp+|e W||| ||. +!+ 3).
vasculitis, occuiiing in ciops on lowei ex-
tiemities with extension to thighs, abdomen;
piecipitated by standing up (Fig. 19-8), less
commonly by cold.
LeJo retu|ars mostly on lowei and uppei
extiemities.
Urtara induced by cold, associated with
puipuia.
Sysemt no|emen. Between 30 and 60%
of individuals with essential mixed CG (type
II) develop ienal disease with hypeitension,
edema, oi ienal failuie. Neuiologic involve-
ment manifests as peiipheial sensoiimotoi
polyneuiopathy, piesenting as paiesthesias oi
foot diop. Aithiitis. Hepatosplenomegaly.
Dagnoss is confiimed by deteimination of
ciyoglobulins (blood diawn into waimed sy-
iinge, RBC iemoved via waimed centiifuge;
plasma iefiigeiated in a Wintiobe tube at 4C
foi 24-72 h, then centiifuged and ciyociit de-
teimined) and diagnosis of undeilying disease.
The tourse is chaiacteiized by cyclic eiuptions
induced by cold oi fluctuations of the activity
of the undeilying disease.
Treamen is that of the undeilying disease.
|eu|er|+ cu|| (|C) | + |oc+||/ed o| d|er|u+|ed
||u |u|||||+||ou |, |eu|er|c ce||. || | uu+||,
+ |u o| d|er|u+||ou o| ,|er|c d|e+e o|
|e|+pe o| e\|||u |eu|er|+.
|uc|deuce .+||e ||or 5 |o 50, depeud|u ou
||e |,pe o| |eu|er|+, |o|| +cu|e +ud c||ou|c,
|uc|ud|u ||e |eu|er|c p|+e o| uounod||u
|,rp|or+ +ud |+||, ce|| |eu|er|+.
\o| corrou|, occu| W||| +cu|e rouoc,||c
|eu|er|+ \5 +ud +cu|e r,e|orouoc,||c |eu|e
r|+ \+.
|+||e|u o| p|eeu|+||ou o| ||u |e|ou |u |C |
.+||+||e +ud r+, |+.e |e+|u|e ||+| o.e||+p W|||
o||e| (|u||+rr+|o|,) e|up||ou. \o| corrou
|e|ou +|e r+|| (2-5 rr) p+pu|e (||. 99
+ud 90), uodu|e (||. 9 +ud 92.), o|
p|+que. |C |e|ou +|e uu+||, oreW|+| ro|e
p|u|, .|o|+ceou, o| d+||e| ||+u uo|r+| ||u,
+|W+, p+|p+||e, |udu|+|ed, |||r, o| u||+|e po
||+||o|r o| |,rp|or+|o|d p+pu|o||||e |e|ou,
|u| uu+||, uo| |eude|.
|oc+||/ed o| d|er|u+|ed, uu+||, ou ||uu| (||.
99.), e\||er|||e (||. 9.), +ud |+ce (||.
90.) |u| r+, occu| +| +u, ||e. \+, |e |ero|
||+|c W|eu +oc|+|ed W||| |||or|oc,|ope
u|+ o| r+, u|ce|+|e (||. 92.). E|,|||ode|r+
r+, (|+|e|,) occu|. |eu|er|c |u|.+| |u|||||+
||ou (|,pe|||op|,) occu| W||| +cu|e rouoc,||c
|eu|er|+. '|r||+| |e|ou+| ro|p|o|o|e occu|
W||| d|||e|eu| |,pe o| |eu|er|+ o| + pec|||c
|,pe o| |eu|er|+ r+, p|eeu| W||| + .+||e|, o|
ro|p|o|o|e.
|||cc|, !!- occu|||u |u p+||eu| W|||
|eu|er|+ +|e rod|||ed |, ||e p+|||c|p+||ou o|
|eu|er|c ce|| |u ||e |u|||||+|e, |eu|||u |u uuuu+|
p|eeu|+||ou o| uc| d|o|de|, e.., po||+|
W||| |ero|||+e o| e|o|ou/u|ce|+||ou.
Cu|+ueou |u||+rr+|o|, d|e+e ||+| r+, |e
+oc|+|ed W||| |eu|er|+ +|e 'Wee| ,ud|ore,
|u||ou p,ode|r+ +u|euour, u|||c+||+, +ud
uec|o||/|u .+cu||||.
',|er|c ,rp|or +|e ||oe +oc|+|ed W|||
|er+|o|o|c r+||u+uc,.
I|e !c | r+de |, up|c|ou +ud .e||||ed
|, ||u ||op,, |rruuop|euo|,p|u, +ud B o| I
ce|| |ecep|o| |e+||+uereu| |ud|e. ner+|o|o|c
|ud|e W||| corp|e|e +u+|,| o| |oue r+||oW
+p||+|e +ud pe||p|e|+| ||ood re+| +|e ||eu
ueeded |o r+|e ||e d|+uo|.
I|e p|ouo| |o| |C | d||ec||, |e|+|ed |o ||e
p|ouo| |o| ||e ,|er|c d|e+e.
|-c, | uu+||, d||ec|ed +| ||e |eu|er|+ ||e||.
noWe.e|, ,|er|c c|ero||e|+p, u|||c|eu| |o|
|oue r+||oW |er||ou r+, uo| ||e+| ||e cu|+ue
ou |e|ou e||ec||.e|,. I|u, + cor||u+||ou o|
,|er|c c|ero||e|+p, +ud |oc+| e|ec||ou |e+r
||e|+p, o| |u\A r+, |e uece+|, |o| c|ero
||e|+p,|e||+u| |C |e|ou.
IkMIA CIIS |C|9 . 205.!
|C|0 . C92.!
FICk 19-9 Ieukema cuts nuud|ed o| |+up|u| p+pu|e +ud + uodu|e ou ||e ||uu| o| + |er+|e W|||
+cu|e r,e|oeuou |eu|er|+ +|oe du||u + Wee| |u|e|.+|. |e| e, ||ee |e|ou +|e 'uoupec|||c +ud do uo|
p|eeu| + d|+uo|, |u| W|eu uc| +u e|up||ou | eeu, oue |ou|d pe||o|r + pe||p|e|+| ||ood couu| +ud + ||op,.
FICk 19-10 Ieukema cuts \u|||p|e ||uco|o|ed +ud e|,||er+|ou p+pu|e |u + !3,e+|o|d |e||||e
Wor+u ||+| |+d e|up|ed +|ou| Wee| |e|o|e ||| p|c|u|e W+ |+|eu. I|e p+||eu| |+d +cu|e r,e|oeuou |eu|er|+.
FICk 19-11 Ieukema cuts A |+|e, d+||
||oWu uodu|e ou ||e uppe| +|r o| + r+|e W|||
+cu|e r,e|oeuou |eu|er|+, |\ |r||+| uodu|e
We|e +|o p|eeu| ou ||e ||uu|.
FICk 19-12 Ieukema cuts: ch|oroma |+|e, u|ce|+|ed, |eeu|ued |uro| (c||o|or+) |u ||e |uu|u+|
+ud pe||ue+| |e|ou o| + |er+|e W||| +cu|e r,e|oeuou |eu|er|+, |r||+| |e|ou We|e +|o p|eeu| |u ||e +\|||+e
+ud ou ||e |ouue.
|+ue||+u ce|| ||||oc,|o| (|Cn) | +u |d|
op+|||c |oup o| d|o|de| c|+|+c|e||/ed |||o
|o|c+||, |, p|o|||e|+||ou +ud |u|||||+||ou o| ||ue
|, |+ue||+u ce||-|,pe ||||oc,|e ||+| |ue |u|o
ru|||uuc|e+|ed |+u| ce|| +ud |o|r |+uu|or+
W||| eo|uop|||.
E||o|o,. + |e+c||.e .e|u ueop|+||c u+|u|e o|
|Cn | de|+|ed.
|Cn | c|+|+c|e||/ed c||u|c+||, |, cu|+ueou ||ud
|u ||+| |+ue ||or o|| ||ue We|||u |o e|o|
||e|c de|r+||||-|||e c|+ue |o p+pu|+|, pu|u|+|
|e|ou, e|o|ou, +ud u|ce|+||ou.
',|er|c |e|ou +||ec| |oue (|,||c e|o|ou), +ud
|uu, |oue r+||oW, ||.e|, p|eeu, +ud |,rp|
uode.
I|e cou|e | .+||+||e, |+u|u ||or |oc+||/ed e||
|e+||u |o|r |o eue|+||/ed +ud |+|+| c+e.
I|e|+p, depeud ou e\|eu| o| d|e+e +ud ,
|er|c |u.o|.ereu|.
|C|9 . 202.5/211.39
|C|0 . |1o.0
IANCkhANS CII hISII0CI0SIS
1
Foi the non-Langeihans cell histiocytosis, the ieadei is iefeiied to C Gelmeti, R Caputo in K Wolff et al (eds),
F:art|'s Dermao|ogy n Cenera| MeJtne, 7th ed. New Yoik, McGiaw-Hill, 2008, pp 1424-1434.
CIASSIFICAII0N
The disoideis of histiocytes aie classified as
Langeihans cell histiocytosis (LCH, foimeily
histiocytosis X), non-Langeihans cell histiocy-
tosis,
1
and malignant histiocytosis (Table 19-1).
LCH is best classified as shown in Table 19-2.
Ae oI 0oset UnI]vcu| LCH Most com-
monly, childhood and eaily adulthood.
Mu|tI]vcu| LCH Most commonly, childhood.
Letterer-SIwe DIseuse (LSD) Moie commonly,
infancy (LSD) and childhood. Also, adult foim.
IA8I 19-2 C|assification of LCh
uu||oc+| |Cn \o| corrou|, r+u||e|ed |, + |u|e o|eo|,||c |ou, o| ||u o| o||
||ue |e|ou.
\u||||oc+| |Cn Bou, |e|ou +|e ru|||p|e +ud |u|e||e|e W||| |uuc||ou o| ue|||o||u
||uc|u|e. \u||||oc+| |Cn +|o |u.o|.e ||u (ecoud ro| ||equeu||,
|u.o|.ed o|+u), o|| ||ue, |,rp| uode, |uu, +ud p||u||+|, |+ud.
C||u|c+| ',ud|ore.
Eo|uop||||c C|+uu|or+ uu||oc+| ||u, rucou rer||+ue o| o|| ||ue |e|ou.
n+ud'c|u||e|C|||||+u ||e+e (n'C|) I|e c||ou|c, p|o|e|.e ru||||oc+| |o|r o| |Cn.
|e||e|e|'|We ||e+e (|'|) I|e ro| +|e|.e ru||||oc+| |Cn |o|r, W||| ||u +ud |u|e|u+| o|+u
|u.o|.ereu|.
n+||ro|o||||/e| ',ud|ore (n|') A |eu|u, e|||e+||u .+||+u| o| |Cn.
IA8I 19-1 C|assification System for histiocytosis 0eve|oped by the histiocyte Society (1981 and 2004)
C|+ |. |+ue||+u ce|| ||||oc,|o|
C|+ ||. n|||oc,|o| o| ||e rououuc|e+| p|+oc,|e ,|er o| uou|+ue||+u ce|| ||||oc,|o| ||+. n|||oc,|o|
|u.o|.|u de|r+| C|o3- +ud |+c|o| /|||- deud|oc,|e (||||oc,|o| |u.o|.|u ce|| o| de|r+| deud|oc,|e ||ue+e)
|||. n|||oc,|o| |u.o|.|u ce|| o||e| ||+u |+ue||+u ce|| +ud ce|| o| ||e de|r+| deud|oc,|e ||ue+e
C|+ |||. \+||u+u| ||||oc,|o|
Hund-Sch||er-ChrIstIun DIseuse (HSCD)
Also childhood, chionic piogiessive.
Has|moo Pr:ger SynJrome (HPS) Child-
hood, self-healing.
Iocdeoce Raie, 0.5 pei 100,000 childien (esti-
mate).
The stimulus foi the piolifeiation of Langei-
hans cells is unknown. A ieactive veisus neo-
plastic natuie is debated.
oIoca| ICh Systemic symptoms uncommon.
Pain and/oi swelling ovei undeilying bony
lesion. Disiuption of teeth with mandibulai
disease, fiactuie, otitis media due to mastoid
involvement.
Mu|tIoca| ICh Eiosive skin lesions aie exuda-
tive, piuiitic, oi painful and may have offensive
odoi. Otitis media caused by destiuction of
tempoial and mastoid bones, pioptosis due to
oibital masses, loose teeth with infiltiation of
maxilla oi mandible, pituitaiy dysfunction with
involvement of sella tuicica associated with
giowth ietaidation, diabetes insipidus. Lung
involvement associated with chionic cough,
pneumothoiax.
IS0 Child (oi veiy iaiely an adult) is sys-
temically ill with a couise that iesembles a
systemic infection oi malignancy. Hepatome-
galy, petechiae, and puipuia, geneialized skin
eiuption.
Sko Iesoos
UnI]vcu| LCH (Eosno||t Cranu|oma )
Swelling ovei bony lesion (e.g., humeius, iib,
mastoid), tendei.
Cutaneous/subcutaneous nodule, yellowish,
may be tendei and bieak down, occuiiing
anywheie.
Shaiply maiginated ulcei, usually in genital
and peiigenital iegions oi oial mucous mem-
biane (gingiva, haid palate). Neciotic base,
diaining, tendei (Fig. 19-13).
Mu|tI]vcu| LCH As in unifocal LCH; in ad-
dition, iegionally localized (head) oi geneialized
(tiunk) eiuptions. Papulosquamous, seboiiheic
deimatitis-like (scaly, oily), eczematous deima-
titis-like lesions (Fig. 19-14); sometimes vesicu-
lai oi puipuiic (Fig. 19-15). Tuin neciotic and
may become heavily ciusted. Removal of ciusts
leaves small, shallow punched-out ulceis (Fig.
19-15B.) that heal with scais. Inteitiiginous
lesions coalesce, may be eiosive and exuda-
tive, become secondaiily infected, and ulceiate.
Mandibulai and maxillaiy bone involvement
may iesult in loss of teeth (Fig. 19-13). Ulceia-
tion of vulva and/oi anus (Fig. 19-16).
LSD Skin lesions as in multifocal LCH but
moie widespiead, disseminated (Fig. 19-15.),
and ulceiating in inteitiiginous iegions (Fig.
19-16).
FICk 19-13 Iaoerhaos ce|| hstocytoss:
eosooph|c raou|oma 'o|||+|,, u|ce|+|ed
uodu|e W||| |o o| |ee|| ou ||e |u|.+| ||de ue+|
||e p+|+|e, +oc|+|ed W||| |u.o|.ereu| o| ||e
r+\|||+|, |oue. |e|ou W+ +,rp|or+||c +ud ou|,
W|eu ||e ro|+| We|e |o| d|d ||e p+||eu| couu||
+ p|,|c|+u.
Ceoera| Fodos Mu|tI]vcu| LCH Bony le-
sions occui in calvaiium, sphenoid bone, sella
tuicica, mandible, long bones of uppei extiemi-
ties, and veitebiae. Associated findings of pitu-
itaiy involvement.
HSCD Lytic skull lesions, pioptosis, diabetes
mellitus, and skin lesions.
LSD Hepatosplenomegaly, lymphadenopathy,
involvement of lungs and othei oigans and
bone maiiow; thiombocytopenia and wide-
spiead and ulceiating skin lesions (Figs. 19-15
and 19-16).
hstopatho|oy Piolifeiation of Langeihans
cells with abundant pale eosinophilic cytoplasm
and indistinct cell boideis; a folded, indented,
kidney-shaped nucleus with finely dispeised
chiomatin; epideimotiopism. Langeihans cells
in LCH have to be iecognized by moiphologic, ul-
tiastiuctuial (Biibeck gianules), histochemical,
and immunohistochemical maikeis S-100 pio-
tein, CD1a and CD207 (Langeiin)].
0IACN0SIS
Confiimation of diagnosis by biopsy (skin,
bone, oi soft tissue/inteinal oigans). Since skin
is the oigan most fiequently involved aftei
bone, skin biopsies have gieat diagnostic sig-
nificance.
C0kS AN0 Fk0CN0SIS
hFS Benign, selfhealing.
oIoca| ICh Benign couise with excellent
piognosis foi spontaneous iesolution but tissue
destiuction.
Mu|tIoca| ICh Spontaneous iemissions pos-
sible. Piognosis pooiei at extiemes of age and
with extiapulmonaiy involvement.
IS0 Commonly fulminant and fatal. Cuiient
scoiing systems foi evaluation of piognosis
aie based on numbei of oigans involved, pies-
ence oi absence of oigan dysfunction, and age.
The woist piognosis is in the veiy young with
multifocal LCH and oigan dysfunction and
in LSD.
FICk 19-14 Iaoerhaos ce|| hstocytoss E|,||er+ +ud r+||, ,e||oWp|u| p+pu|e W||| + |e+, c+|e
ou ||e |+ce +ud c+|p |u ||| |u|+u|. I|ee We|e ||e ou|, |e|ou +| |||| p|eeu|+||ou +ud We|e r||+|eu |o| |u|+u
|||e e|o|||e|c de|r+||||. A||e| |e|ou p|o.ed |e||+c|o|, |o |op|c+| ||e+|reu| +ud +dd|||ou+| pu|pu||c +ud c|u|ed
|e|ou +ppe+|ed ou ||e ||uu|, + ||op, W+ pe||o|red +ud ||e co||ec| d|+uo| W+ e|+||||ed.
SCII0N 19 'K|| '|C|' 0| nE\AI0|0C|C ||'EA'E 51T
MANACMNI
oIoca| ICh Cuiettage with oi without bony
chip packing. Low-dose (300-600 iad) iadio-
theiapy. Extiaosseous soft tissue lesions: suigi-
cal excision oi low-dose iadiotheiapy.
Mu|tIoca| ICh Diabetes insipidus and
giowth ietaidation tieated with vasopiessin
and human giowth hoimone. Low-dose ia-
diotheiapy to bony lesions. Systemic tieat-
ment with glucocoiticoids and/oi vinblastine,
given as single agents oi in combination and
etoposide. Noniespondeis: polychemotheiapy
(vinciistine and cytaiabine and piednisone
oi vinciistine and doxoiubicine and pied-
nisone). Bone maiiow tiansplantation is an
option.
Cutaoeous |esoos Glucocoiticoids foi dis-
ciete cutaneous lesions. Extensive oi geneial-
ized: cutaneous lesions iespond best to PUVA
oi topical nitiogen mustaid but also to oial
thalidomide.
FICk 19-15 Iaoerhaos ce|| hstocytoss: Ietterer-Swe dsease E|,||er+|ou p+pu|e +ud
.e|c|e W||| pu|pu|+, c|u||u, |ecor|u cou||ueu| ou ||e +|doreu o| +u |u|+u|. \o|e +d.+uced |e|ou |u
+uo||e| |u|+u| ||+| |+.e |ed |o u|ce| +ud dep|eed c+|.
FICk 19-16 Iaoerhaos ce|| hstocytoss: Ietterer-Swe dsease o ao adu|t Cou||ueu| e|,||er+
|ou p|+que W||| uec|o| +ud u|ce|+||ou |u ||e +uoeu||+| +ud pe||ue+| |e|ou |u + o5,e+|o|d |er+|e.
\+|oc,|o| | +u +|uo|r+| +ccuru|+||ou o|
r+| ce|| |u ||e ||u +ud +| .+||ou ,|er|c
||e.
Au +|||e.|+|ed wo||d ne+||| 0|+u|/+||ou (wn0)
c|+|||c+||ou o| r+|oc,|o| | |oWu |u I+||e
9!.
I|e ||u | ||e ro| corrou|, |u.o|.ed o|+u
,|er.
'||u |e|ou +|e |oc+||/ed uodu|+| o| eue|+||/ed
r+cu|op+pu|+| (I+||e 9+).
Bec+ue o| ||e |e|e+e o| p|+|r+co|o|c+||, +c||.e
u||+uce, cu|+ueou ,rp|or +|e u|||c+||+|
We|||u o| ||||e||u W||| p|u|||u, ,|er|c ,rp
|or +|e ||u||u, .or|||u, d|+|||e+, |e+d+c|e,
,ucope.
\o| p+||eu| W||| r+|oc,|o| |+.e ou|, ||u
|u.o|.ereu|, +ud ro| o| ||ee |+.e uo ,|er|c
,rp|or. noWe.e|, up |o |+|| o| p+||eu| W|||
,|er|c r+|oc,|o| r+, uo| |+.e +u, ||u
||ud|u.
MASI0CI0SIS SN0k0MS |C|9 . 151.!!/202.o
|C|0 . 032.2
IA8I 19-4 C|assification of Cutaneous Nastocytosis (CN)
|oc+||/ed |odu|+| C\ (r+|oc,|or+, |C\)
Ceue|+||/ed \+cu|op+pu|+| C\
|+pu|+| p|+que C\ (||C\)
u|||c+||+ p|reu|o+ (u|)
Ie|+u|ec|+|+ r+cu|+|| e|up||.+
pe||+u (I\E|)
||||ue C\ (|C\)
IA8I 19-3 Abbreviated wh0 C|assification of Nastocytosis
Cu|+ueou r+|oc,|o| (C\)
|udo|eu| ,|er|c r+|oc,|o| (|'\)
',|er|c r+|oc,|o| W||| +u +oc|+|ed
c|ou+| |er+|o|o|c uour+| ce|| ||ue+e d|e+e ('\An|\|)
A|e|.e ,|er|c r+|oc,|o| (A'\)
\+| ce|| |eu|er|+ (\C|)
\+| ce|| +|cor+ (\C')
E\||+cu|+ueou r+|oc,|or+
'ou|ce. | \+|eu| e|. +|.. wn0 C|+|||c+||ou o| Iuro|. |+||o|o, +ud eue||c o| |uro| o| ||e |er+|opo|e||c +ud |,rp|o|d ||ue. E' l+||e
e|.+|. (ed). |,ou, |AkC ||e, 200
FI0MI0I0C
Ae oI 0oset Between biith and 2 yeais of age
(55%) (NCM, PPCM, UP), but mastocytosis
can occui at any age; infancy-onset mastocytosis
iaiely associated with systemic mastocytosis.
Sex Slight male: female piedominance.
Freva|eoce Unknown.
FAIh0CNSIS
Human mast cell piolifeiation depends on
Kit ligand and Kit is the ieceptoi foi stem cell
factoi. c- | mutations have been identified in
blood and tissues of patients with mastocytosis.
Mast cells contain seveial phaimacologically
active substances that aie associated with the
clinical findings in mastocytosis: histamine (ui-
ticaiia, GI symptoms), piostaglandin D
2
(flush,
caidiovasculai symptoms, bionchoconstiiction,
GI symptoms), hepaiin (bleeding into tissue,
osteopoiosis), neutial piotease/acid hydiolases
(patchy hepatic fibiosis, bone lesions).
Stioking lesion causes it to itch and to wheal
(Darer sgn ). Vaiious diugs aie capable of
causing mast cell degianulation and ielease
of phaimacologically active substances that
exaceibate skin lesions (whealing, itching)
and cause flushing: alcohol, dextian, poly-
myxin B, moiphine, codeine, scopolamine,
D-tubocuiatin, nonsteioidal anti-inflammatoiy
diugs. Flushing episode can also be elicited
by heat oi cold and may be accompanied by
headache, nausea, vomiting, diaiihea, dysp-
nea/wheezing, syncope. Systemic involvement
may lead to symptoms of malabsoiption; poi-
tal hypeitension. Bone pain. Neuiopsychiatiic
symptoms (malaise, iiiitability).
Sko Iesoos (CM) Ioca|ted NCM Maculai
to papulai to nodulai lesions (mastocytoma)
(Fig. 19-17), often solitaiy; may be multiple,
but few. Yellow to tan-pink, which become
eiythematous and iaised (uiticate) when
stioked due to degianulation of mast cells
(Daiiei sign); in some patients, lesions become
bullous.
Ceoera|ted PPCM Tan, occasionally yel-
lowish plaques, up to 2-5 cm, shaiply defined
with iiiegulai outlines. Daiiei sign positive (Fig.
19-18). No scaling, occasionally with bulla foi-
mation aftei iubbing. Occuis mostly in infants
and childien.
UP Tan macules to slightly iaised tan to
biown papules (Fig. 19-19). Disseminated, few
oi >100 with widespiead symmetiic distiibu-
tion. Daiiei sign (whealing) aftei iubbing; in
infants may become bullous. Occuis in infancy
and/oi de novo in adults. Biight ied diffuse
flushing occuiiing spontaneously, aftei iubbing
of skin, oi aftei ingestion of alcohol oi mast
cell-degianulating agents.
TMEP Fieckle-like, biownish to ieddish mac-
ules (Fig. 19-20) with fine telangiectasia in long-
standing lesions. Hundieds of lesions, tiunk >
extiemities; lesions may be confluent. Uiticate
with gentle stioking. Deimatogiaphism. Occui
only in adults and veiy iaie.
DCM Yellowish, thickened appeaiance of
laige aieas of skin; doughy." Smooth with
scatteied elevation, iesembling leathei,
pseudoxanthomatous mastocytosis," skin folds
exaggeiated, especially in axilla/gioin. Laige
bullae may occui aftei tiauma oi spontane-
ously. DCM may piesent as eiythiodeima (Fig.
19-21). Veiy iaie, occuis at all ages.
Systemc Symptoms Flushing, accompanied by
wheezing, headache, asthma, nausea, vomiting,
diaiihea, syncope. Bone pain/spontaneous fiac-
tuies with osteolytic lesions. Neuiopsychiatiic
symptoms, malaise, iiiitability. Malabsoiption,
weight loss.
0ermatopatho|oy Accumulation of noimal-
looking mast cells in deimis. Mast cell infiltiates
may be spaise (spindle-shaped) oi densely ag-
giegated (cuboidal shape) and have a peiivascu-
lai oi nodulai distiibution. Pigmentation due
to incieased melanin in basal layei.
C8C Systemic mastocytosis: anemia, leukocy-
tosis, eosinophilia.
8|ood Tiyptase levels , coagulation paiam-
eteis.
roe Patients with extensive cutaneous in-
volvement may have incieased 24-h uiinaiy
histamine excietion.
8ooe Scao aod Imao Define bone involve-
ment (lytic bone lesions, osteopoiosis, oi os-
teoscleiosis), and endoscopy foi small-bowel
involvement.
8ooe Marrow Smeai and/oi biopsy foi moi-
phology and mast cell maikeis.
0IACN0SIS
Clinical suspicion, positive Daiiei sign, con-
fiimed by skin biopsy.
NCM Juvenile xanthogianuloma, Spitz nevus.
F|usho Caicinoid syndiome.
F, FFCM, IMF Langeihans cell histiocyto-
sis, secondaiy syphilis, papulai saicoid, genei-
alized eiuptive histiocytoma, non-Langeihans
cell histiocytosis of childhood.
0CM Cutaneous T cell lymphoma, pseudo-
xanthoma elasticum, foims of eiythiodeima.
C0kS AN0 Fk0CN0SIS
Most cases of solitaiy mastocytosis and geneial-
ized UP and PPCM in childien iesolve sponta-
neously. They iaiely have systemic involvement.
FICk 19-1T Mastocytoss: so|tary
mastocytoma (NCM) A o|||+|,, |+u
p|+que W||| poo||, der+|c+|ed |o|de| ou
||e |+ud o| +u |u|+u|. w|eu ||o|ed .e|,
.|o|ou|,, ||e |e|ou |ec+re |ed, ro|e
e|e.+|ed +ud + ||||e| de.e|oped.
FICk 19-18 Mastocytoss: eoera|ted (FFCM) \u|||p|e, ||+||opped p+pu|e +ud r+|| p|+que o|
||oWu|| |o ,e||oW|| co|o| ou ||e |u||oc| o| + c|||d. |e|ou +|e +,rp|or+||c. ku|||u oue o| ||e |e|ou |+
|eu||ed |u u|||c+||ou +ud +u +\ou ||+|e, + po|||.e |+||e| |u, +ud ||c||u.
Adults with onset of UP oi TMEP with extensive
cutaneous involvement have a highei iisk foi
development of systemic mastocytosis (see Table
19-3). In young childien, acute and extensive de-
gianulation may be life-thieatening (shock).
MANACMNI
Avoidance of diugs that may cause mast cell de-
gianulation and histamine ielease (see above).
Antihistamines, both H
1
and H
2
, eithei alone
oi with ketotifen. Disodium ciomoglycate, 200
mg foui times a day, may amelioiate piuiitus,
flushing, diaiihea, abdominal pain, and disoi-
deis of cognitive function but not skin lesions.
PUVA tieatment is effective foi disseminated
skin lesions, but iecuiience is common. Vascu-
lai collapse is tieated with epinephiine. NCM
iesponds to potent glucocoiticoid ointments
undei occlusion oi to intialesional tiiamci-
nolone acetonide but may eventually iecui.
FICk 19-19 Mastocytoss: urtcara
pmeotosa (F) \u|||p|e, eue|+||/ed
|+u |o ||oWu p+pu|e |u + c|||d. I|e p+||eu| |+d
occ+|ou+| ,ucope, d|+|||e+, +ud W|ee/|u,
Wo||up |e.e+|ed ,|er|c r+|oc,|o|.
B|oWu p+pu|e ou ||e |o|e|e+d o| +
!,e+|o|d |o, W|o W+ o||e|W|e +,rp|or+||c.

FICk 19-20 Mastocytoss:
te|aoectasa macu|ars eruptva
perstaos 'r+||, |e||+|e e|,||er+|ou
r+cu|e +ud |e|+u|ec|+e ou ||e |+c| o|
+ +5,e+|o|d Wor+u W|o |+d ,|er|c
(|udo|eu|) r+|oc,|o|.
FICk 19-21 Mastocytoss: dIIuse cutaoeous
mastocytoss I|e ||u o| ||| |u|+u| | uu||o|r|, e|,
||er+|ou (e|,|||ode|r+) ecoud+|, |o |u|||||+||u r+|
ce|| W||| e.e|+| p+|ed, W|||e +|e+ o| uo|r+| ||u. |u |||
c|||d ||e|e We|e ,|er|c ,rp|or +oc|+|ed W||| ||e ||+|e
o| ||| e|,|||ode|r+. ,ucope, W|ee/|u, +ud d|+|||e+.
524
CIAN0S IMFh0MAS
AN0 SAkC0MA
S E C I | 0 N 2 0
Cu|+ueou |,rp|or+ +|e c|ou+| p|o|||e|+||ou
o| ueop|+||c I o| B ce||, |+|e|, u+|u|+| ||||e|
ce|| o| p|+r+c,|o|d deud||||c ce||. Cu|+ueou
|,rp|or+ +|e ||e ecoud ro| corrou |oup
o| e\||+uod+| |,rp|or+. I|e +uuu+| |uc|deuce
| e||r+|ed |o |e pe| 00,000.
I|e wn0E0kIC c|+|||c+||ou | |oWu |u I+||e
20.
|o| |+|e coud|||ou uo| de+|| W||| |u ||| A||+ ||e
|e+de| | |e|e||ed |o C A+|, w '|e||,, |u K wo|||
e| +| (ed). ||cc|:| |-c||, C--c|
!-!:- 1 || ed. |eW \o||, \cC|+Wn|||, 2003,
pp !3o-+02.
Adu|| I ce|| |eu|er|+/|,rp|or+ (AI||) | + ueo
p|+r o| C|++/C|25+ I ce||, c+ued |, |ur+u
I ce|| |,rp|o||op||c .||u | (nI|\|).
\+u||e|ed |, ||u |u|||||+|e, |,pe|c+|cer|+, .|
ce|+| |u.o|.ereu|, |,||c |oue |e|ou, +ud +|
uo|r+| |,rp|oc,|e ou pe||p|e|+| re+|.
nI|\| | + |ur+u |e||o.||u. |u|ec||ou |, ||e .||u
doe uo| uu+||, c+ue d|e+e, W||c| ue|
||+| o||e| eu.||oureu|+| |+c|o| +|e |u.o|.ed.
|rro||+||/+||ou o| ore |u|ec|ed C|++ I ce||, |u
c|e+ed r||o||c +c||.||,, eue||c |u|+|||||,, +ud |r
p+||reu| o| ce||u|+| |rruu||, c+u +|| occu| +||e|
|u|ec||ou W||| nI|\|. I|ee e.eu| r+, |uc|e+e
||e p|o|+|||||, o| +dd|||ou+| eue||c c|+ue,
W||c|, |, c|+uce, r+, |e+d |o ||e de.e|opreu|
o| |eu|er|+ 20+0 ,e+| +||e| |u|ec||ou |u ore
peop|e ( 5). \o| o| ||ee e||ec| |+.e |eeu
+|||||u|ed |o ||e nI|\|eucoded p|o|e|u |+\.
AI|| occu| |u ou||We|e|u l+p+u (K,u|u),
A|||c+, ||e C+||||e+u ||+ud, ou||e+|e|u uu||ed
'|+|e. I|+ur||ou | |, e\u+| |u|e|cou|e, pe||
u+|+||,, o| |, e\pou|e |o ||ood o| ||ood p|oduc|
(+re + n|\).
I|e|e +|e |ou| r+|u c+|eo||e. |u ||e |e|+
||.e|, |udo|eu| |!- +ud :|: |o|r,
||e red|+u u|.|.+| | 2 ,e+|. |u ||e c:|- +ud
|,|c| |o|r, || |+ue ||or ou|, +-o
rou||.
',rp|or |uc|ude |e.e|, We||| |o, +|dor|u+|
p+|u, d|+|||e+, p|eu|+| e||u|ou, +c||e, cou|,
pu|ur. '||u |e|ou occu| |u 50 o| p+||eu|
W||| AI||. '|u|e |o ru|||p|e r+||, cou||ueu|
e|,||er+|ou, .|o|+ceou p+pu|e (||. 20),
pu|pu|+, |||r .|o|+ceou |o ||oWu|| uod
u|e (||. 202), p+pu|oqu+rou |e|ou, |+|e
p|+que, u|ce|+||ou, ||uu| > |+ce > e\||er|||e,
eue|+||/ed e|,|||ode|r+, po||||ode|r+, d|||ue
+|opec|+. |,rp|+deuop+||, (15) p+||u re
d|+||u+| |,rp| uode. nep+|ore+|, (50) +ud
p|euore+|, (25).
|+||eu| +|e e|opo|||.e (E||'A, we|e|u ||o|)
|o nI|\|, |u |\ d|u ue|, up |o !0 |+.e du+|
|e||o.||+| |u|ec||ou W||| |o|| nI|\| +ud n|\. wBC
|+ue ||or uo|r+| |o 500,000/|. |e||p|e|+|
||ood re+| |oW po|,|o|u|+|ed |,rp|oc,||c
uuc|e| ('||oWe| ce||). |-c|c|||, |e.e+|
|,rp|or+|ou |u|||||+|e corpoed o| r+u,
|+|e +|uo|r+| |,rp|oc,|e, |+u| ce||, |+u
|||e| r|c|o+|cee. I|e|e | |,pe|c+|cer|+-|u
25 +| ||re o| d|+uo| o| AI|| +ud |u >50
du||u c||u|c+| cou|e, ||| | ||ou|| |o |e due |o
o|eoc|+||c |oue |eo|p||ou.
\+u+ereu| cou|| o| .+||ou |e|reu o|
c,|o|o\|c c|ero||e|+p,, ||e |+|e o| corp|e|e
|epoue +|e !0 +ud |epoue |+c| du|+|||||,,
|u| ood |eu|| |+.e |eeu o||+|ued W||| ||e
cor||u+||ou o| o|+| /|do.ud|ue +ud u|cu|+ue
ou |u|e||e|ou |u +cu|e +ud |,rp|or+|,pe
AI|| p+||eu|.
|C|9 . 20+.0/203.9
|C|0 . C3!/E33
A0II I CII IkMIA[IMFh0MA
SCII0N 20 CuIA|E0u' |\\|n0\A' A|| 'AkC0\A 525
IA8I 20-1 wh0E0RIC C|assification of
Primary Cutaneous
Lymphomas
Cutaneous I-Cell and NK-Cell
Lymgbomas
\,co| |uuo|de
\,co| |uuo|de .+||+u| +ud u||,pe
|o|||cu|o||op|c r,co| |uuo|de
|+e|o|d |e||cu|o|
C|+uu|or+|ou |+c| ||u
'e/+|, ,ud|ore
Adu|| Ice|| |eu|er|+/|,rp|or+
|||r+|, cu|+ueou C|!0po|||.e |,rp|op
|o|||e|+||.e d|o|de|
|||r+|, cu|+ueou +u+p|+||c |+|ece||
|,rp|or+
|,rp|or+|o|d p+pu|o|
'u|cu|+ueou p+uu|cu|||||||e Ice|| |,rp|or+
E\||+uod+| |K/Ice|| |,rp|or+, u++| |,pe
||o.||ou+| eu||||e o| cu|+ueou Ice|| |,rp|or+
|||r+|, cu|+ueou +|e|.e ep|de|ro||op|c
C|3
-
Ice|| |,rp|or+
Cu|+ueou / Ice|| |,rp|or+
|||r+|, cu|+ueou C|+
-
r+|| o| red|ur|/ed
p|eoro|p||c Ice|| |,rp|or+
Cutaneous B-Cell Lymgbomas
|||r+|, cu|+ueou r+||u+| /oue Bce||
|,rp|or+
|||r+|, cu|+ueou |o|||c|e ceu|e| |,rp|or+
|||r+|, cu|+ueou d|||ue |+|e Bce|| |,rp|or+,
|e |,pe
|||r+|, cu|+ueou d|||ue |+|e Bce|| |,rp|or+,
o||e|
|u||+.+cu|+| |+|e Bce|| |,rp|or+
(p|o.||ou+|)
||ecu|o| |er+|o|o|c ueop|+r
C|+
-
/C|5o
-
|er+|ode|r|c ueop|+r
(||+||c |Kce|| |,rp|or+)
|K, u+|u|+| ||||e|, wn0E0kIC, wo||d ne+||| 0|+u|/+||ou +ud
Eu|ope+u 0|+u|+||ou |o| kee+|c| +ud I|e+|reu| o| C+uce|.
FICk 20-1 Adu|t I ce|| |eukema[|ymphoma
A eue|+||/ed e|up||ou o| r+||, cou||ueu| .|o|+ceou
p+pu|e W||| + p|ed||ec||ou |o| ||e ||uu|. I|e p+||eu|
|+d |e.e|, We||| |o, +|dor|u+| p+|u, r+|.e |eu|o
c,|o| W||| '||oWe| ce|| |u re+|, |,rp|+deuop+||,,
|ep+|op|euore+|,, +ud |,pe|c+|cer|+.
FICk 20-2 Adu|t I ce|| |eukema[|ymphoma
|||r, .|o|+ceou |o ||oWu|| uodu|e + |oWu |e|e
+|e +uo||e| cu|+ueou r+u||e|+||ou o| AI||. I|ee
uodu|e r+, u|ce|+|e.
Putches Randomly distiibuted, scaling oi non-
scaling patches in diffeient shades of ied (Fig.
20-3). Well- oi ill-defined; at fiist supeificial,
much like eczema oi psoiiasis (Figs. 20-3 and
20-4) oi mimicking deimatophytosis (myco-
sis"), and latei becoming thickei.
P|uques Round, oval, but often also aicifoim,
annulai, and of bizaiie configuiation (Figs. 20-3
and 20-5). Lesions aie iandomly distiibuted but
in eaily stages often spaie exposed aieas.
Tumvrs: Latei lesions consist of nodules (Figs.
20-5 and 20-6) and tumois, with oi without
ulceiation (Fig. 20-7). Extensive infiltiation can
cause leonine facies (Fig. 20-8). Confluence may
lead to eiythiodeima (see Section 8). Theie is
palmoplantai keiatodeima and theie may be
haii loss. Poikilodeima may be piesent fiom the
onset oi develop latei (Fig. 20-9).
Ceoera| xamoatoo Lymphadenopathy, usu-
ally aftei thick plaques and nodules have ap-
peaied.
Ae oI 0oset Median age at diagnosis 55-60
yeais.
Sex Male:female iatio 2:1.
Iocdeoce Uncommon but not iaie.
to|oy Unknown. CTCL is a malignancy of
skin-homing CTLA+ CD4+ T cells.
CIINICAI MANIFSIAII0NS
Foi months to yeais, often pieceded by vaii-
ous diagnoses such as psoiiasis, nummulai
deimatitis, and laige plaque" paiapsoiiasis.
Symptoms: piuiitus, often intiactable, but may
be none.
Sko Fodos Skin lesions aie classified into
patches, plaques, and tumoi stage. Patients
may have simultaneously moie than one type
of lesions.
Cu|+ueou I ce|| |,rp|or+ (CIC|) | + |e|r ||+|
+pp||e |o I ce|| |,rp|or+ |||| r+u||e|ed |u ||e
||u, |u| |uce ||e ueop|+||c p|oce |u.o|.e ||e
eu|||e |,rp|o|e||cu|+| ,|er, ||e |,rp| uode
+ud |u|e|u+| o|+u |ecore |u.o|.ed |u ||e
cou|e o| ||e d|e+e. CIC| | + r+||u+uc, o|
|e|pe| I ce|| (C|++).
|u ||e c|+|c |o|r o| CIC|, c+||ed ,: |
!- (\|), ||e r+||u+u| ce|| +|e cu|+ueou
C|++ ce||, |u| ||e c||u|c+| eu|||, o| \| |+ uoW
|eeu e\p+uded |o ||e pec||ur o| CIC| |uc|ud
|u uou\| cu|+ueou I ce|| |,rp|or+.
w|e|e+ +|| \| | CIC| uo| +|| CIC| +|e \|.
0u|, ||e c|+|c \| |o|r | d|cued |e|e.
',uou,r. \,co| |uuo|de.
|C|9 . 202./202.2
|C|0 . C3+.0/C3+.
CIAN0S I CII IMFh0MA
\| | ||e ro| corrou cu|+ueou |,rp|or+.
A|||u |u r|d |o |+|e +du|||ood W||| r+|e p|e
dor|u+uce o| 2..
A c|ou+| p|o|||e|+||ou o| ||u|or|u CI|A+
C|++ I ce|| W||| +u +dr|\|u|e o| C|3+ I ce||
(+u|||uro| |epoue).
C+|eo||/ed + p+|c|, p|+que, o| |uro| |+e.
ke|+|ed |e+|u|e +|e p|u|||u, +|opec|+, p+|ro
p|+u|+| |,pe||e|+|o|, +ud |+c|e||+| |u|ec||ou.
n||o|o|c+||,, ep|de|ro||op|r o| I ce|| W||| |,
pe|cou.o|u|ed uuc|e|. |u ||e |uro| |+e de|r+|
uodu|+| |u|||||+|e.
||ouo| |e|+|ed |o |+e.
I|e+|reu|. ,rp|oro||eu|ed +ud |+e+d+p|ed.
MC0SIS FNC0I0S (MF) |C|9 . 202./202.0
|C|0 . C3+.0/C3+.
SCII0N 20 CuIA|E0u' |\\|n0\A' A|| 'AkC0\A 52T
FICk 20-3 Mycoss Iuoodes |u e+||, |+e |e|ou cou|| o| |+udor|, d|||||u|ed, We|| +ud/o| |||
de||ued p+|c|e +ud |+|e| p|+que + |oWu |e|e |u + !1,e+|o|d r+|e. I|e, r+, |e c+|, +ud +ppe+| |u .+||ou
|+de o| |ed. I|e, r|r|c ec/er+, po||+|, o| de|r+|op|,|o|.
FICk 20-4 Mycoss Iuoodes: patches[p|aque stae \o|e +d.+uced |+e |oW cou||ueuce o|
p+|c|e +ud p|+que W||| |||eu|+| cou||u|+||ou. I|| p+||eu| |+d |eeu ||e+|ed uuucce|u||, |o| po||+| |o|
2 ,e+|. \o|p|o|o|c+||,, |e cou|d +|o |+.e e\|eu|.e, cou||ueu| de|r+|op|,|o| (ee 'ec||ou 25) |u| + ue+||.e
K0n p|ep+|+||ou |u|ed ou| ||| d|+uo|. 0u|, +||e| + ||op, |+d |eeu doue W+ ||e co||ec| d|+uo| o| \| r+de.
FICk 20-5 Mycoss Iuoodes ||+que +ud e+||, uodu|+| |+e W||| |edd||||oWu|| c+|,, +ud c|u|ed
p|+que +ud ||+| uodu|e.
FICk 20-6 Mycoss Iuoodes: tumor
stae 'c+|, +ud c|u|ed ec/er+|||e p|+que
eeu ou ||e +|r +ud c|e| |+.e |u|ued uodu|+|
ou ||e |ou|de|. I|| p+||eu| |+d |r||+| |e|ou
e|eW|e|e +ud W+ |+ed ||B (I
!
|
\
0
).
FICk 20-T Mycoss Iuoo-
des: tumors IWo |+|e u|ce|+|ed
|uro| ou ||e |oWe| ||+u| o| + \|e|
u+ree r+u. I|e |uro| +|oe |u +
e+ o| |||de||ued, c+|, +ud c|u|ed
p|+que. I|e p+||eu| |+d |eeu ||e+|ed
|o| po||+| |o| |Wo ,e+|.
FICk 20-8 Mycoss Iuoodes: |eoooe Iaces |u ||| 50,e+|o|d p+||eu| ||e d|e+e |+d |+||ed W|||
e\||ere|, p|u||||c, eue|+||/ed ec/er+|||e p|+que ou ||e ||uu| ||+| |+d |eeu ||e+|ed + ec/er+ o.e| + cou|e o|
+ ,e+|. \+|.e uodu|+| |u|||||+||ou o| ||e |+ce occu||ed ou|, |eceu||, |e+d|u |o + |eou|ue |+c|e.
|||:||: !|. w||| p|e|e|eu||+| |u.o|.ereu|
o| |e+d +ud uec|, W||| o| W|||ou| ruc|uo|, de
eue|+||ou o| |+|| |o|||c|e (p|e.|ou|, 'ruc|uo|
|o|||cu|+||, '+|opec|+ ruc|uo+) (||. 200).
|,-|-! !|. n,pop|reu|ed p+|c|e |u
p+||eu| W||| d+|| ||u.
|c-|! -|:| (|-|| !-c-)
I|| | + pec|+| .+||+u| o| \| cou|||u o|
|:c|c-! p+|c|e +ud p|+que (||. 20), W|||
+ p|o|||e|+||ou o| ueop|+||c I ce||, ||+| e\p+ud
|u||+ep|de|r+||, |o||oW|u + p+||e|u |r||+| |o
|+e| d|e+e. E\||+cu|+ueou d|er|u+||ou |+
uo| |eeu o|e|.ed, +ud ||e|e | +u e\ce||eu|
p|ouo|.
Cc|c| |c:| |. k+|e u||,pe o| \|
W||| |o|d o| |+\ ||u |u ||e r+jo| ||u |o|d (||.
202).
'-cc, ,!-. A |eu|er|c .+||+u|, ee |e|oW
p 5!+ +ud 'ec||ou 3.
0ermatopatho|oy In eaily stages iepeated
and multiple biopsies aie often necessaiy to
finally establish the diagnosis. Bandlike and
patchy infiltiate in uppei deimis of atypi-
cal lymphocytes (mycosis cells) extending to
epideimis and skin appendages. The classic
finding is the epideimotiopism of this T cell
infiltiate, which will foim micioabscesses in
the epideimis (Pautiiei micioabscesses). In the
plaque and tumoi stage the infiltiate extends
deep into the deimis and beyond. Mycosis cells
aie T cells with hypeichiomatic, iiiegulaily
shaped (ceiebiifoim) nuclei. Mitoses vaiy fiom
iaie to fiequent.
FICk 20-9 Mycoss Iuoodes: pok|odermatous |esoos 'r+|| |e||cu|+|ed, cou||ueu| p+pu|e
r|\ed W||| upe|||c|+| +||op|, |.e ||e |rp|e|ou o| po||||ode|r+. I|| p+||eu| |+d p+|c|e e|eW|e|e ou ||e
|od, |r||+| |o ||oe |oWu |u ||. 20!. |o||||ode|r+ |u \| c+u +|o |eu|| ||or ||e+|reu|. I|| p+||eu| |+d
|eeu ||e+|ed W||| e|ec||ou |e+r.

MC0SIS FNC0I0S vAkIANIS
FICk 20-10 Fo||cu|otropc MF \u|||p|e r+|| |o|||cu|+| p+pu|e ('ruc|uo| |o|||cu|+||).
FICk 20-11 Faetod retcu|oss I|| |uu|+| p|+que ou ||e ||p o| + o2,e+|o|d |er+|e |oo| |||e po
||+|. || W+ +,rp|or+||c +ud |+d |eeu p|eeu| |o| 3 rou||. n||op+||o|o, |e.e+|ed |u||+ep|de|r+| I ce|| |u
+ p+e|o|d p+||e|u.
Mycosis cells aie activated monoclonal CTLA+
CD4+ T cells. Howevei, lesions of MF often
have a CD8+ T cell component, and these cells
aie consideied to ieflect an antitumoi iesponse;
impioved long-teim piognosis has been coi-
ielated with the piesence of such CD8+ tumoi-
infiltiating lymphocytes.
hemato|oy Eosinophilia, 6-12%, can in-
ciease to 50%. Buffy coat: abnoimal ciiculating
T cells (mycosis cell-type) and incieased WBC
(20,000/L). Bone maiiow examination is not
helpful in eaily stages.
Chemstry Lactic dehydiogenase isoenzymes
1, 2, and 3 incieased in eiythiodeimic stage.
Chest X-kay Seaich foi hilai lymphadenopa-
thy.
Imao In stage I and stage II disease, di-
agnostic imaging (CT, gallium scintigiaphy,
livei-spleen scan, and lymphangiogiaphy) does
not piovide moie infoimation than biopsies of
lymph nodes.
CT Scun With moie advanced disease, to
seaich foi ietiopeiitoneal nodes in patients
with extensive skin involvement, lymphade-
nopathy.
In the eaily stages, the diagnosis of MF is a
pioblem. Clinical lesions may be typical, but
histologic confiimation may not be possible foi
yeais despite iepeated biopsies. Tissue should
be sent foi immunophenotyping of infiltiating
T cells by use of monoclonal antibodies and
T cell ieceptoi ieaiiangement studies. Lym-
phadenopathy and the detection of abnoimal
ciiculating T cells in the blood appeai to coi-
ielate well with nerna| oigan involvement.
0IIereota| 0aooss Mainly sta|ng |aques
(see Figs. 20-3, 20-4, and 20-5). High index of
suspicion is needed in patients with atypical oi
iefiactoiy psoiiasis," eczema," and poikilo-
deima. MF often mimics psoiiasis in being a
scaly plaque and disappeaiing with exposuie
to sunlight.
Fateot va|uatoo o MF aod Stao This has
to focus on an evaluation of tumoi buiden, the
degiee of atypia of malignant cells, and the state
of immunocompetence of the patient. Table 20-2
shows a flow sheet of patient evaluation, Table
20-3 shows the TNM classification and Table 20-
4 the staging of mycosis fungoides.
IA8I 20-3 INN C|assification of Nycosis |unoides (C552 I C L )
I. '||u I
0
C||u|c+||, +ud/o| |||o|o|c+||, up|c|ou |e|ou
I
||r||ed p|+que, p+pu|e, o| ec/er+|ou p+|c|e co.e||u 0 o| ||e ||u u||+ce

I
2
Ceue|+||/ed p|+que, p+pu|e, o| e|,||er+|ou p+|c|e co.e||u >0 o| ||e ||u
u||+ce
I
!
Iuro| ( o| ro|e)
I
+
Ceue|+||/ed e|,|||ode|r+
|. |,rp| uode |
0
|o c||u|c+||, +|uo|r+| pe||p|e|+| uode, p+||o|o, ue+||.e |o| \|
|
C||u|c+||, +|uo|r+| pe||p|e|+| |,rp| uode, p+||o|o, ue+||.e |o| \|

|
2
|o c||u|c+||, +|uo|r+| pe||p|e|+| |,rp| uode, p+||o|o, po|||.e |o| \|
|
!
C||u|c+||, +|uo|r+| pe||p|e|+| |,rp| uode, p+||o|o, po|||.e |o| \|
B. B|ood B
0
5 +|,p|c+| c||cu|+||u |,rp|oc,|e
B
>5 +|,p|c+| c||cu|+||u |,rp|oc,|e ('e/+|,)

\. \|ce|+| o|+u \
0
|o .|ce|+| o|+u |u.o|.ereu|
\
n||o|o|c+||, p|o.eu .|ce|+| |u.o|.ereu|

IA8I 20-2 Patient Eva|uation in N|
'||u
Bod, u||+ce +|e+ +ereu|
kou||ue |||o|o,
|rruuop|euo|,p|u
|o|,re|+e c|+|u |e+c||ou |o| I ce|| |ecep|o|
|e+||+uereu|
B|ood
Corp|e|e ||ood couu| W||| re+| e\+r|u+||ou
|rruuop|euo|,p|u
|,rp| uode
|+|p+|e +|| uode
\e+u|e eu|+|ed uode |, CI c+u
B|op, eu|+|ed uode
IA8I 20-4 Stain System for Nycosis |unoides and Stary Syndrome
Stage T(T0mor) h (Lymph hode) N (Netastases)
|A I |0 \0
|B I2 |0 \0
||A I o| I2 | \0
||B I! |0 0k | \0
||| I+ |0 0k | \0
|\A I-I+ |2 0k |! \0
|\B I-I+ |0-|! \
I. p+|c|/p|+que 0 o| |od, u||+ce, I2. p+|c|/p|+que 0 o| |od, u||+ce, I!. ||u |uro|(), I+ e|,|||ode|r+, |0. uo|r+| uode, |.
p+|p+||e uode W|||ou| |||o|o|c e.|deuce o| |,rp|or+, |2. uo p+|p+||e uode, |u| |||o|o|c e.|deuce o| |,rp|or+, |!. p+|p+||e uode W|||
|||o|o|c e.|deuce o| |,rp|or+, \0. uo .|ce|+| |u.o|.ereu|, \. |||o|o|c+||, cou|||red .|ce|+| |u.o|.ereu|.
FICk 20-12 Craou|omatous s|ack sko |||r, p|+|e|||e |u|||||+|e ou ||e uec| +ud +u|e||o| c|e| +ud |+\
||u |o|d o| ||e +\|||+|, +ud c+pu|+| |e|ou.
'e/+|, ,ud|ore | + |+|e pec|+| .+||+u| o| \|
c|+|+c|e||/ed |, uu|.e|+| e|,|||ode|r+, pe||p|
e|+| |,rp|+deuop+||,, +ud ce||u|+| |u|||||+|e o|
+|,p|c+| |,rp|oc,|e ('e/+|, ce||) |u ||e ||u +ud
|u ||e ||ood.
I|e d|e+e r+, +||e de uo.o o|, |e corrou|,,
|eu|| ||or e\|eu|ou o| + p|ee\|||u c||cur
c|||ed \|. || uu+||, occu| |u p+||eu| >o0 ,e+|
+ud ro|e corrou|, |u r+|e ||+u |u |er+|e.
|+||eu| +ppe+| |c|, ||.e||u, +ud c+|ed +ud
||e|e | eue|+||/ed c+||u e|,|||ode|r+ W|||
cou|de|+||e |||c|eu|u o| ||e ||u. Bec+ue o|
||e ||||| |ed co|o|, ||e ,ud|ore |+ |eeu
c+||ed ||e '|ed r+u ,ud|ore (ee 'ec||ou 3
+ud ||. 3!). I|e|e | d|||ue |,pe||e|+|o| o|
p+|r +ud o|e, d|||ue |+|| |o ||+| c+u |e+d |o
|+|due, +ud eue|+||/ed |,rp|+deuop+||,.
|-c|c|||,. ||e +re + \|. I|e |,rp|
uode r+, cou|+|u uoupec|||c |u||+rr+|o|, ce||
(de|r+|op+|||c |,rp|+deuop+||,) o| ||e|e c+u
|e + corp|e|e |ep|+cereu| o| ||e uod+| p+||e|u
|, 'e/+|, ce||. I|e ce|| |u|||||+|e |u ||e .|ce|+
+|e ||e +re + +|e p|eeu| |u ||e ||u. |
|-|,. C|++ I ce||, I ce|| |ecep|o|
|e+||+uereu|. rouoc|ou+| p|oce. I|e|e r+,
|e + rode|+|e |eu|oc,|o| o| + uo|r+| wBC.
I|e |u||, co+| cou|+|u ||or 5-!0 +|,p|c+|
|,rp|oc,|e ('e/+|, ce||).
||+uo| |e| ou |||ee |e+|u|e. e|,|||ode|r+,
eue|+||/ed |,rp|+deuop+||,, +ud p|eeuce o|
|uc|e+ed uur|e| o| +|,p|c+| |,rp|oc,|e |u ||e
|u||, co+|.
|o|e ||+| +u, e\|o||+||.e de|r+|||| c+u r|r|c
'e/+|, ,ud|ore (ee 'ec||ou 3).
w|||ou| ||e+|reu|, ||e cou|e | p|o|e|.e
+ud p+||eu| d|e ||or oppo||uu|||c |u|ec||ou.
\+u+ereu| | + |u \|, p|u +pp|op||+|e up
po|||.e re+u|e |equ||ed |o| e|,|||ode|r+
(ee 'ec||ou 3).
SIAk SN0k0M |C|9 . 202.2
|C|0 . |3+.
C0kS AN0 Fk0CN0SIS
Unpiedictable; MF (pie-MF) may be piesent
foi yeais. Couise vaiies with the souice of the
patients studied. At the NIH theie was a median
suivival time of 5 yeais fiom the time of the his-
tologic diagnosis, while in Euiope a less malig-
nant couise is seen (suivival time, up to 10-15
yeais). This, howevei, may be due to patient
selection. Piognosis is much woise when (1)
tumois aie piesent (mean suivival, 2.5 yeais),
(2) theie is lymphadenopathy (mean suivival, 3
yeais), (3) >10% of the skin suiface is involved
with pietumoi-stage MF, and (4) theie is a gen-
eialized eiythiodeima. Patients <50 yeais have
twice the suivival iate of patients >60 yeais.
MANACMNI
Theiapy is symptom-oiiented and extent of
disease- and stage-adapted. In the pie-MF
stage, in which the histologic diagnosis is
only compatible, but not confiimed, PUVA
photochemotheiapy is the most effective tieat-
ment, but naiiow-band UVB tieatment is also
effective. Foi histologically pioven plaque-stage
disease with no lymphadenopathy and no ab-
noimal ciiculating T cells, PUVA photoche-
motheiapy is also the method of choice, eithei
alone oi combined with oial isotietinoin oi
bexaiotene oi subcutaneous inteifeion- . Also
used at this stage aie topical chemotheiapy
with nitiogen mustaid in an ointment base
(10 mg/dL), topical caimustine (BCNU) (foi
limited body suiface aiea involvement), and
total-body election-beam theiapy, singly oi in
combination. Isolated tumois that may develop
should be tieated with local x -iay oi election-
beam theiapy. Foi extensive plaque stage with
multiple tumois oi in patients with lymphad-
enopathy oi abnoimal ciiculating T cells,
election-beam plus chemotheiapy is piobably
the best combination foi now; iandomized,
contiolled studies of vaiious combinations aie
in piogiess. Also, extiacoipoieal PUVA pho-
tochemotheiapy is being evaluated in patients
with Szaiy syndiome.
|,rp|or+|o|d p+pu|o| | +u +,rp|or+||c,
c||ou|c, e|||e+||u, po|,ro|p|ou e|up||ou o|
uu|uoWu e||o|o,.
|| | + |oW|+de, e||||r||ed I ce|| |,rp|or+
W||| + |oW |u| |e+| ||| o| p|o|e|ou |o ro|e
r+||u+u| |o|r o| |,rp|or+.
|uc|deuce | .2-.9 c+e pe| r||||ou, occu|||u
po|+d|c+||, |u |o|| e\e ||or c|||d|ood |o o|d
+e, +.e|+e +e +0 ,e+|.
C|+|+c|e||/ed |, |ecu||eu| c|op o| |e|ou ||+|
|e|e pou|+ueou|,, W||| |||o|o|c |e+|u|e o|
|,rp|oc,||c +|,p|+.
|+||oeue| uu|uoWu, cou|de|ed |o |e + |oW
|+de |,rp|or+ pe||+p |uduced |, c||ou|c
+u||eu|c ||ru|+||ou +ud cou||o||ed |, |o|
rec|+u|r. Be|u + + c||ou|c, |e+c||.e, po|,
c|ou+| |,rp|op|o|||e|+||.e p|euoreuou ||+| po
|+d|c+||, o.e|W|e|r |o| |rruue de|eue +ud
e.o|.e |u|o + c|ou+|, +u||eu|udepeudeu|, ||ue
|,rp|o|d r+||u+uc,. || |e|ou |u ||e pec||ur
o| p||r+|, cu|+ueou C|!0+ |,rp|op|o|||e|+||.e
d|o|de|.
C|oe c||u|c+| |eer||+uce |o p||,||+| ||c|euo|de
e| .+||o|||o|r| +cu|+ (ee ||. 13). E|,||er+
|ou |o |ed||oWu p+pu|e (||. 20!) +ud
uodu|e, 2-5 rr |u d|+re|e|, W||c| +|e |u|||+||,
roo|| +ud |ero|||+|c, |+|e| |,pe||e|+|o||c,
W||| ceu||+|, ||+c| uec|o|, c|u||u (||. 20
!), +ud u|ce|+||ou. |eW |o |uud|ed o| |e|ou,
+,rp|or+||c o| p|u||||c, +||+ued +| |+udor +ud
o||eu |ouped, |ecu||eu|, p||r+|||, ou ||uu| +ud
e\||er|||e, |+|e|,, o|+| +ud eu||+| ruco+. |ud|
.|du+| |e|ou e.o|.e o.e| + 2 |o 3Wee| pe||od
+ud |eo|.e pou|+ueou|, +| +u, po|u| |u ||e||
e.o|u||ou. A||op||c |,pe| o| |,pop|reu|ed
c+|||u |o||oW|u u|ce|+|ed |e|ou.
0||e| o|+u ,|er +|e uu|u.o|.ed.
|-c|c|||, 'upe|||c|+| o| deep, pe||.+
cu|+| o| |u|e|||||+| r|\ed ce|| |u|||||+|e, Wede
|+ped. C,|o|o|c+||,, +|,p|c+| ce|| r+, corp||e
50 o| |u|||||+|e. ,- 1 . |+|e C|!0+, +|,p|c+|
||||o|d |,rp|oc,|e W||| +|uud+u| c,|op|+r,
cou.o|u|ed uuc|eu W||| occ+|ou+| ||uuc|e+
||ou, ru|||po|+| r||o|, +ud keed'|e|u|e|
ce|| +ppe+|+uce. Adr|\ed W||| + deue |u||+r
r+|o|, |u|||||+|e. ,- 3 . r+||e| C|!0, +|,p|c+|
|,rp|oc,|e W||| ce|e||||o|r uuc|e|, ep|de|ro
||op|r, +ud occ+|ou+| r||o|. ,- C |+|e
C|!0+ ce|| |o|r |ee| |eer|||u cu|+ueou
+u+p|+||c |+|e ce|| |,rp|or+ (CA|C|)
|||--|c| !c. B+ed ou |,p|c+| |||o|o,
+ud |rruuo|||oc|er|||,, |+c| o| ,|er|c |u
.o|.ereu| |, |||o|, +ud p|,|c+| e\+r|u+||ou.
C-. \+, |er|| |u ! Wee| o| cou||uue |o|
dec+de. |u 0-20 o| p+||eu|, |,rp|or+|o|d
p+pu|o| | p|eceded |,, +oc|+|ed W|||, o|
|o||oWed |, +uo||e| |,pe o| |,rp|or+. \|,
nod||u d|e+e, o| C|!0+ (CA|C|). \+, pe|||
dep||e ,|er|c c|ero||e|+p, |o| coucu||eu|
|,rp|or+.
|o ||e+|reu| |+.e p|o.ed cou||eu||, e||ec||.e,
+ | e.|deuced |, ||e ru|||p|e |epo||ed ||e|+
p|e. Iop|c+| +eu| |uc|ude |ucoco|||co|d +ud
c+|ru||ue (BC|u). E|ec||ou|e+r |||+d|+||ou.
|u\A cou||o| ||e d|e+e |u| doe uo| +||ec|
||e |ou|e|r p|ouo|. Ie||+c,c||ue, u||oue,
,|er|c |ucoco|||co|d, +ud +c,c|o.|| |+.e |eeu
+uecdo|+||, |epo||ed + e||ec||.e. A|o, |e||uo|d,
re||o||e\+|e, c||o|+r|uc||, c,c|op|op|+r|de,
c,c|opo||ue, +ud |u|e||e|ou 2|, uoue W||| |+|
|u e||ec|.
IMFh0MAI0I0 FAFI0SIS |C|0 . |+.2
CA|C| +|e cu|+ueou |,rp|or+ cou|||u o|
|+|e |uro| ce|| ||+| e\p|e C|!0 +u||eu +ud
|+.e uo e.|deuce o| |||o|, o| |,rp|or+|o|d
p+pu|o|, r,co| |uuo|de, o| o||e| |,pe o|
CIC|.
I|e, occu| |u +du|| +ud p|eeu| + o|||+|,,
|edd|| |o ||oWu|| uodu|e +ud |uro|, W||c|
||equeu||, |eud |o u|ce|+|e (||. 20+).
I|e uodu|+| |u|||||+|e +|e uouep|de|ro||op|c, +ud
ueop|+||c ce|| |oW +u +u+p|+||c ro|p|o|o,.
A| |e+| 15 o| ||e ueop|+||c ce|| +|e C|!0+
+ud +dd|||ou+||, e\p|e ||e C|++ p|euo|,pe.
CA|C| |+.e + |+.o|+||e p|ouo| W||| + d|e+e
|e|+|ed 5,e+| u|.|.+| |+|e o| 90.
I|e+|reu| | |+d|o||e|+p,, |u| ucce|u| ||e+|
reu| W||| |u\A |u cor||u+||ou W||| |u|e||e|ou
|+ |eeu |epo||ed.
|C|9 . \91+/!
CIAN0S ANAFIASIIC IAkC CII IMFh0MAS (CAICI)
FICk 20-13 Iymphomatod papu|oss C|op o| |edd||||oWu p+pu|e +ppe+| |u W+.e |u.o|.|u ||e
eu|||e |od,. |e|ou +|e +,rp|or+||c, |ecore |,pe||e|+|o||c, c|u|ed, +ud uec|o||c |u ||e ceu|e|. '|uce |e|ou
+||e +,uc||ouou|,, +|| |+e |u ||| e.o|u||ou +|e p|eeu| |ru||+ueou|,.
FICk 20-14 Aoap|astc |are
ce|| |ymphoma A o|||+|, .|o|+
ceou, |edd|| uodu|e ou ||e |o|e+|r
o| + +o,e+|o|d r+|e p+||eu|. n||o
p+||o|o, |e.e+|ed uouep|de|ro
||op|c +u+p|+||c rououuc|e+| ce||,
ro| o| W||c| We|e o| ||e C|++,
C|!0+ p|euo|,pe. I|e |e|ou W+
e\c|ed +ud ||e|e W+ uo |ecu||euce.
SCII0N 20 CuIA|E0u' |\\|n0\A' A|| 'AkC0\A 53T
A c|ou+| p|o|||e|+||ou o| B |,rp|oc,|e c+u |e
cou||ued |o ||e ||u o| ro|e o||eu | +oc|+|ed
W||| ,|er|c B ce|| |,rp|or+. k+|e. Corp||e
20 o| +|| cu|+ueou |,rp|or+.
0ccu| |u |ud|.|du+| >50 ,e+|.
C|op o| +,rp|or+||c uodu|e +ud p|+que, |ed
|o p|ur co|o| (||. 205) W||| + roo|| u||+ce,
|||r, uou|eude|, cu|+ueou o| u|cu|+ueou.
|||r+|, cu|+ueou |o|||c|e ceu|e| ce|| |,rp|or+,
p||r+|, cu|+ueou r+||u+| /oue |,rp|or+, +ud
p||r+|, cu|+ueou |+|e B ce|| |,rp|or+ o| ||e
|e +|e pec|+| de||ued eu||||e.
|-c|c|||,. |eue uodu|+| o| d|||ue
rouoro|p|ou |u|||||+|e o| |,rp|oc,|e uu+||,
ep+|+|ed ||or ||e ep|de|r| |, + /oue o| uo|r+|
co||+eu ('|eu/ /oue). B ce||-pec|||c rouo
c|ou+| +u|||od, |ud|e |+c||||+|e d|||e|eu||+||ou
o| cu|+ueou B ce|| |,rp|or+ ||or peudo
|,rp|or+ +ud cu|+ueou I ce|| |,rp|or+ +ud
pe|r|| ro|e +ccu|+|e c|+|||c+||ou o| ||e ce||
|,pe. \o| c+e |e+c| W||| C|9, 20, 22, +ud
19A. Ceue|,p|u |ud|e cou|||r d|+uo| W|||
|rruuo|o|u||u eue |e+||+uereu|.
|+||eu| |ou|d |e |u.e||+|ed ||o|ou||, |o|
uod+| +ud e\||+cu|+ueou d|e+e, || |ouud, |oue
r+||oW, |,rp| uode, +ud pe||p|e|+| ||ood |ud
|e W||| |oW ro|p|o|o|c, c,|oc|er|c+|, +ud
|rruuo|o|c |e+|u|e |r||+| |o ||oe o| ||e
cu|+ueou |u|||||+|e.
!cc--|. Cou|| o| \|+, ||e|+p, |o |o
c+||/ed |e|ou +ud c|ero||e|+p, |o| ,|er|c
d|e+e.
CIAN0S 8 CII IMFh0MA |C|0 . C35.
FICk 20-15 Cutaoeous 8 ce|| |ymphoma 'roo||, cu|+ueou +ud u|cu|+ueou uodu|e ou ||e |oWe|
|e. 0ue | u|ce|+|ed. I|e, We|e +,rp|or+||c +ud |||r +ud We|e ||e |||| |u o| B ce|| |,rp|or+.
II0FAIh0CNSIS
DNA of human heipesviius type 8 (HHV-8)
has been identified in tissue samples of all vaii-
ants of KS. Theie is seioepidemiologic evidence
that this viius is involved in the pathogenesis.
CIASSIFICAII0N AN0 CIINICAI vAkIANIS
C|assc or uropeao kS Occuis in eldeily
males of eastein Euiopean heiitage (Meditei-
ianean and Ashkenazi Jewish). Not so uncom-
mon in eastein and southein Euiope; iaie in
the United States. Peak incidence aftei sixth
decade. Males > females. Piedominantly aiises
on the legs but also occuis in lymph nodes and
abdominal visceia; slowly piogiessive.
AIrcao-odemc kS Between 9 and 12.8%
of all malignancies in Zaiie. Two distinct age
gioups: young adults, mean age 35; and young
childien, mean age 3 yeais. Males > females.
No evidence of undeilying immunodeficiency.
Foui clinical patteins (see below).
Iatroeoc Immuoosuppressoo-Assocated kS
Raie. Most commonly in solid-oigan tiansplant
iecipients as well as individuals tieated chioni-
cally with immunosuppiessive diugs. Aiises on
aveiage 16.5 months aftei tiansplantation. Re-
solves on cessation of immunosuppiession.
hIv[AI0S-Assocated kS In HIV-infected
individuals, the iisk foi KS is 20,000 times
that of the geneial population, 300 times
that of othei immunosuppiessed individuals.
Eaily in the HIV/AIDS epidemic in the United
States and Euiope, 50% of homosexual men
at the time of initial diagnosis of AIDS had
KS; cuiiently, the incidence is 18% in this iisk
gioup. Young adults. HIV/AIDS-associated
KS occuis almost exclusively in homosexual
males; iaiely women may have HIV/AIDS-
associated KS when they acquiie HIV infection
K' | + ru||||oc+| ,|er|c |uro| o| eudo||e||+|
ce|| o|||u.
|u.+||+||, ||u|ed W||| |ur+u |e|pe.||u 3 |u|ec
||ou.
|ou| c||u|c+| .+||+u|. c|+|c K', euder|c A|||c+u
K', |rruuoupp|e|.e ||e|+p,-|e|+|ed K' +ud
n|\/A||'|e|+|ed K'.
'|+e +ud .+||+u|depeudeu| |oc+||/ed +ud/o|
eue|+||/ed d|e+e. p+|c|e, p|+que, uodu|e.
',|er|c |u.o|.ereu|. r+|u|, C| ||+c|.
kepoud |o |+d|+||ou +ud c|ero||e|+p,.
kAF0SI SAkC0MA (kS) |C|9 . 1o
|C|0 . C+o
via heteiosexual exposuie fiom a bisexual
male. Associated with HIV infection, iapid
piogiession, extensive systemic involvement.
At the time of initial piesentation, one in six
HIV-infected individuals with KS have CD4+
T cell counts of 500/L.
FAIh0CNSIS
KS cells likely aie deiived fiom the endothe-
lium of the blood/lymphatic miciovasculatuie.
Initially not a tiue malignancy but iathei a
widespiead ieactive polyclonal piolifeiation in
iesponse to angiogenic molecules. Latei be-
comes monoclonal. KS lesions pioduce factois
that piomote theii own giowth as well as the
giowth of othei cells, but it is not known how
HHV-8 induces/piomotes piolifeiation of en-
dothelial cells.
Mucocutaneous lesions aie usually asympto-
matic but aie associated with significant cos-
metic stigma. At times lesions may ulceiate and
bleed easily. Laige lesions on palms oi soles may
impede function. Lesions on the lowei extiemi-
ties that aie tumoious, ulceiated, oi associated
with significant edema often give iise to modei-
ate to seveie pain. Uiethial oi anal canal lesions
can be associated with obstiuction. GI involve-
ment iaiely causes symptoms. Pulmonaiy KS
can cause bionchospasm, intiactable cough-
ing, shoitness of bieath, piogiessive iespiiatoiy
failuie.
Sko Iesoos KS most often begins as an ecchy-
motic-like macule (Figs. 20-16 and 20-19). Mac-
ules evolve into patches, papules, plaques (Figs.
20-16, 20-17, and 20-18), nodules, and tumois
that aie violaceous, ied, pink, oi tan and become
puiple-biownish (Figs. 20-16 and 20-17) with a
FICk 20-16 C|assc kapos sarcoma
Ecc|,ro||c pu|p|e||oWu|| cou||ueu| r+cu|e
+ud + cr uodu|e ou ||e do|ur o| ||e |+ud o|
+ o5,e+|o|d r+|e o| A||eu+/|leW|| e\||+c||ou.
I|e |e|ou W+ o|||u+||, r||+|eu |o| + ||u|e
+ We|e |r||+| |e|ou ou ||e |ee| +ud ou ||e
o||e| |+ud. I|e +ppe+|+uce o| ||oWu|| uodu|e
|oe||e| W||| +dd|||ou+| r+cu|e p|orp|ed + |e|e|
|+| o| ||| o||e|W|e corp|e|e|, |e+|||, p+||eu| |o
+ de|r+|o|o|| W|o d|+uoed K+po| +|cor+,
W||c| W+ .e||||ed |, ||op,. I|e|e | +|o ou,c|o
r,co| o| +|| ||ue|u+||.
FICk 20-1T C|assc kapos sarcoma B|oWu|| cou||ueu| p+pu|e ou ||e do|ur o| ||e |oo|. |u.o|.e
reu| o| |,rp|+||c |+ |ed |o p|ououuced eder+ o| ||e |o|e|oo|, W||c| |ud|c+|e ||+| ||e d|e+e p|oce |
|u|||e| +d.+uced.
gieenish hemosideiin halo as they age. Almost
all KS lesions aie palpable, feeling fiim to haid
even when they aie in a patch stage. Often oval
initially, and on the tiunk often aiianged paial-
lel to skin tension lines (Fig. 20-20). Lesions may
initially occui at sites of tiauma, usually in the
acial iegions (Fig. 20-18). In time, individual le-
sions may enlaige and become confluent, foim-
ing tumoi masses. Secondaiy changes to laigei
nodules and tumois include eiosion, ulceiation,
ciusting, and hypeikeiatosis.
Lym|eJema usually occuis on the lowei ex-
tiemities (Fig. 20-17) and iesults fiom conflu-
ent masses of lesions due to deepei involvement
of lymphatics and lymph nodes. Distal edema
may initially be unilateial but latei becomes
symmetiic and involves not only the lowei legs
but also the genitalia and/oi face.
DIstrIhutIvn Widespiead oi localized. In
classic KS, lesions almost always occui on the
feet and legs oi the hands and slowly spiead
centiipetally (Figs. 20-16 and 20-17). Tip of
nose (Fig. 20-19), peiioibital aieas, eais, and
scalp as well as penis (Fig. 35-26) and legs
may also be involved, but involvement of the
tiunk is iaie. In HIV/AIDS-associated KS
theie is eaily involvement of the face (Fig. 20-
19) and widespiead distiibution on the tiunk
(Fig. 20-20).
Mucous Membraoes Oial lesions aie the fiist
manifestation of KS in 22% of cases; in HIV/
AIDS-associated KS often a maikei foi CD4+ T
cell counts of <200/L. Veiy common (50% of
individuals) on haid palate, appeaiing fiist as
a violaceous stain, which evolves into papules
and nodules with a cobblestone appeaiance.
Lesions also aiise on soft palate, uvula, phai-
ynx, gingiva, and tongue. Conjunctival lesions
uncommon.
Speca| Features oI AIrcao-odemc kS (Non-
HIV-associated) Foui clinical patteins aie iec-
ognized:
Nodulai type: Runs a iathei benign couise
with a mean duiation of 5-8 yeais and
iesembles classic KS.
Floiid oi vegetating type: Chaiacteiized by
moie aggiessive biologic behavioi; is also
nodulai but may extend deeply into the
subcutis, muscle, and bone.
Infiltiative type: Shows an even moie aggies-
sive couise with floiid mucocutaneous and
visceial involvement.
Lymphadenopathic type: Piedominantly af-
fects childien and young adults. Fiequently
confined to lymph nodes and visceia, but
occasionally also involves the skin and mu-
cous membiane.
Ceoera| xamoatoo VIsceru KS lesions of
the visceia, though common, aie often asymp-
tomatic. This is paiticulaily tiue foi classic KS.
At autopsy of HIV-infected individuals with
mucocutaneous KS, 75% have visceial involve-
ment (bowel, livei, spleen, lungs).
Lymph Nvdes Lymph nodes aie involved in
half of cases of HIV/AIDS-associated KS and in
all cases of Afiican lymphadenopathic type KS.
UrvgenItu| Truct Piostate, seminal vesicles,
testes, bladdei, penis, sciotum.
Lung Pulmonaiy infiltiates, paiticulaily in
HIV-associated KS.
G1 Truct GI hemoiihage, iectal obstiuction,
piotein-losing enteiopathy can occui.
Other Heait, biain, kidney, adienal glands.
Sko 8opsy Vasculai channels lined by atypical
endothelial cells among a netwoik of ieticulin
fibeis and extiavasated eiythiocytes with hemo-
sideiin deposition. Thiee histologic stages:
Pat| sage. Piolifeiation of small, iiiegulai, and
jagged endothelial-lined spaces suiiounding
noimal deimal vessels and adnexal stiuc-
tuies; vaiiable, inflammatoiy lymphocytic
infiltiate (plasma cells).
P|aque sage. Spindle cells thioughout
deimal collagen bundles foiming iiiegulai,
cleftlike, angulated vasculai channels that
contain vaiiable numbeis of RBCs. He-
mosideiin deposits; eosinophilic hyaline
globules. Peiipheial peiivasculai inflam-
matoiy infiltiate.
NoJu|ar sage. Spindle cells in sheets and fas-
cicles with mild to modeiate cytologic atypia,
single cell neciosis, tiapped RBCs within an
extensive netwoik of slitlike vasculai spaces.
Imao Foi inteinal oigan involvement.
Confiimed on lesional skin biopsy.
0IIereota| 0aooss Includes single pigmented
lesions: deimatofibioma, pyogenic gianuloma,
hemangioma, bacillaiy (epithelioid) angiomato-
sis, melanocytic nevus, ecchymosis, gianuloma
annulaie, insect bite ieactions, stasis deimatitis.
C0kS AN0 Fk0CN0SIS
C|assc kS Aveiage suivival, 10-15 yeais; death
usually fiom unielated causes. Secondaiy ma-
lignancies aiise in >35% of cases.
FICk 20-18 C|assc kapos sarcoma oI the Ieet B|oWu|| |o ||ue uodu|e +ud p|+que, p+|||+||, |,pe|
|e|+|o||c ou ||e o|e +ud |+|e|+| +pec| o| ||e |ee|. I|| | + |,p|c+| |oc+||/+||ou o| e+||, c|+|c K'.
FICk 20-19 hIv[AI0S-assocated kapos sarcoma \u|||p|e ||u|e|||e pu|p||| +ud ||oWu|| r+cu|e,
p+pu|e +ud uodu|e +|e p|eeu| uo| ou|, ou ||e |+ce |u| +|o ou ||e ||uu| +ud ||e e\||er|||e o| ||| 29,e+|o|d
r+|e |oroe\u+| W||| A||'. |o|e +|o We|||u o| ||e uoe. E+||, |u.o|.ereu| o| ||e |+ce | |,p|c+| |o| n|\/A||'
+oc|+|ed K'.
AIrcao-odemc kS Mean suivival in young
adults, 5-8 yeais; young childien, 2-3 yeais.
Iatroeoc Immuoosuppressoo-Assocated kS
Couise may be chionic oi iapidly piogiessive;
KS usually iesolves aftei immunosuppiessive
diugs aie discontinued.
hIv[AI0S-Assocated kS (See also Section 31)
HIV-infected individuals with high CD4+ T cell
counts can have stable oi slowly piogiessive
disease foi many yeais. Rapid piogiession of KS
can occui aftei decline of CD4+ T cell counts to
low values, piolonged systemic glucocoiticoid
theiapy, oi illness such as Pneumotyss tarn
pneumonia. KS of the bowel and/oi lungs is
the cause of death in 10-20% of patients. Pa-
tients with only a few lesions, piesent foi sev-
eial months, without histoiy of oppoitunistic
infections, and CD4+ T cell counts >200/L
tend to iespond bettei to theiapy and have
a bettei oveiall piognosis. At time of initial
diagnosis, 40% of KS patients have GI involve-
ment; 80% at autopsy. Reduced suivival iate in
patients with GI involvement. Pulmonaiy KS
has high shoit-teim moitality iate, i.e., median
suivival <6 months.
MANACMNI
The goal of theiapy foi KS is to contiol symp-
toms of the disease, not cuie. A numbei of
local and systemic theiapeutic modalities aie
effective in contiolling symptoms. Classic KS
iesponds well to iadiotheiapy of involved sites.
Afiican-endemic KS, when symptomatic, ie-
sponds best to systemic chemotheiapy. Im-
munosuppiessive diug-associated KS iegiesses
oi iesolves when diug dosages aie ieduced oi
discontinued. HIV/AIDS-associated KS usually
iesponds to a vaiiety of local theiapies; foi ex-
tensive mucocutaneous involvement oi visceial
involvement, chemotheiapy is indicated.
Local theiapy is usually diiected at individual
lesions that aie cosmetically distuibing (e.g.,
on the face), bulky, bleeding, cause functional
distuibance on the palms oi soles, oi cause
lymphatic obstiuction and lymphedema.
Imted Ioterveotoo
kadotherapy Indicated foi tumoious lesions,
confluent lesions with a laige suiface aiea, laige
lesions on distal extiemity, laige oiophaiyngeal
lesions. Dosing: 8 Gy in a single fiaction foi
small lesions, 800-3000 iad in single oi divided
dose.
Cryosurery Indicated foi deeply pigmented,
piotiuding nodules. Best iesults with two
fieeze-thaw cycles. Pain is modeiate duiing
fieeze cycle. Tieated lesions heal with ciust
foimation. KS often peisists in deepei poitions
of lesion. Violaceous lesion is ieplaced with a
white scai. Secondaiy infection is uncommon.
Iaser Surery Pulsed-dye lasei effective foi
small supeificial lesion.
Fhotodyoamc Iherapy Foi small supeificial
lesions.
|ectrosurery Effective foi ulceiated, bleed-
ing nodulai lesion; must use a smoke evacuatoi
in conjunction.
xcsooa| Surery Effective foi selected small
lesions. Not a iealistic appioach to the patient
with many lesions.
Iotra|esooa| Cytotoxc Chemotherapy
VInh|ustIne 0.1 mg (0.5 mL of a 0.2-mg/mL
solution) injected pei squaie centimetei of le-
sion; foi iefiactoiy lesions, inciemental doses
of up to 0.2 mg/cm
2
can be given. Most effective
foi small, eaily, papulai lesions. Laigei nodulai
lesions iespond moie slowly. The maximal total
vinblastine dose injected should not exceed
2 mg pei clinic visit. Some lesions heal with
blistei foimation, ciusting, and scaiiing. In-
adveitent injection neai a cutaneous sensoiy
neive can iesult in a neuiitic pain that can last
up to a month.
VIncrIstIne und B|evmycIn These have also
been used foi intialesional theiapy.
Aressve Ioterveotoo
So|e-Aeot Chemotherapy
Adiiamycin, 20 mg/m
2
.
Vinblastine, IV bolus 0.1 mg/kg weekly.
Lipid foimulations of daunoiubicin and
doxoiubicin.
Etoposide (VP16), given oially.
Paclitaxel (Taxol), given IV eveiy 3 weeks.
Comboatoo Chemotherapy
Vinciistine (2 mg) + bleomycin (15 U/m
2
) +
adiiamycin (20 mg/m
2
) is given eveiy othei
week in patients with ielatively advanced KS.
Inteifeion- (15 million U/d) + zidovudine
(600 mg/d).
Iype-SpecIc Iherapy
C|asst KS: Any of the above.
[rtan KS: Any of the above.
Immunosuresson-re|aeJ KS: Reduction
of immunosuppiession, ieplacement of cal-
cineuiin inhibitois by iapamycine.
HIV/IDS-re|aeJ KS: Any of the above, pief-
eiably liposomal anthiacyclines intiavenously
eveiy 2 to 4 weeks plus HAART.

FICk 20-20 hIv[AI0S-assocated kapos sarcoma \u|||p|e pu|p||| p|+que +ud uodu|e ou
||e ||uu| o| + |oroe\u+| A||' p+||eu|. I|e p+||eu| |+d C|++ I ce|| couu| 200/| +ud r+||ed rucou
rer||+ue |u.o|.ereu|, |-:,| :c pueurou|+, +ud Cc!!c.
544
SkIN 0ISASS IN 0kCAN
AN0 80N MAkk0W
IkANSFIANIAII0N
S E C I | 0 N 2 1
0|+u ||+up|+u| |ec|p|eu| +|e c||ou|c+||, |r
ruuoupp|eed +ud ||e|| I ce|| |uuc||ou |
|rp+||ed.
Euu|u d|e+e +|e ro||, |u|ec||ou +ud +|e
|r||+| |o ||oe occu|||u |u o||e| coud|||ou +
oc|+|ed W||| I ce|| |rp+||reu|, uc| + A||'.
|u +dd|||ou, o|+u ||+up|+u| |ec|p|eu| +|e +| |e+|
||| |o| de.e|op|u uoure|+uor+ ||u c+uce| +ud
o||e| c+uce|.
Boue r+||oW +ud |er ce|| |+|| |ec|p|eu| +|e
c+ud|d+|e |o| |+||.e|u|o| d|e+e (C\n|).
B+c|e||+| p+||oeu. (ee 'ec||ou 2+)
\||+| p+||oeu (ee 'ec||ou 21 +ud !)
|uu+| p+||oeu (ee 'ec||ou 25 +ud !)
'|c|,|:::, '|-|::: 'c|-||c,
||-c, |:c!c, !,:|c:|- c.
|c:-|||c-, ! ||-:|, |--||c
C,|ore+|o.||u (C\\), |e|pe |rp|e\ .||u
(n'\), .+||ce||+ /o|e| .||u (\/\), ro||ucur
cou|+|our .||u, |ur+u p+p|||or+ .||u (n|\),
Ep|e|uB+|| .||u (EB\)
Cc!!c, C,|:::, |||cc, C::!
!-, 3|c|,:-, |-c||,|- (,:|,
:) 1-||
*
C||u|c+| r+u||e|+||ou +|e d|cued |u ||e|| |epec||.e
ec||ou.
M0SI C0MM0N INFCII0NS ASS0CIAI0 WIIh 0kCAN
IkANSFIANIAII0N*
SCII0N 21 'K|| ||'EA'E' || 0kCA| A|| B0|E \Akk0w IkA|'||A|IAI|0| 545
|oure|+uor+ ||u c+uce| | ||e ro| corrou
r+||u+uc, |u +du|| o||d o|+u ||+up|+u| p+
||eu|.
I|e r+jo|||, +|e qu+rou ce|| c+|c|uor+ ('CC)
('ec||ou ).
I|e ||| o| de.e|op|u 'CC |uc|e+e e\poueu
||+||, W||| ||e |eu|| o| |rruuoupp|e|ou.
I|e curu|+||.e |uc|deuce | 30 +||e| 20 ,e+| o|
|rruuoupp|e|ou |u |eu+| ||+up|+u|+||ou. 'CC
|u po|||+up|+u| p+||eu| +|e +|e|.e.
n|\ |u|ec||ou | |rp||c+|ed |u ||e p+||oeue|.
0||e| ep|||e||+| p|o|||e|+||.e |e|ou +|e +c||u|c
|e|+|oe, |e|+|o+c+u||or+, po|o|e|+|o|, +p
peud+e |uro|, +ud \e||e| ce|| c+|c|uor+
('ec||ou ).
C|||d|eu W||| o|+u ||+up|+u| r+, +|o |e +|
|||e| ||| |o| ||e de.e|opreu| o| re|+uor+
('ec||ou 2).
|,rp|op|o|||e|+||.e d|o|de| +|e corrou |u
|+|| |ec|p|eu| +ud |e|+|ed |o Ep|e|uB+|| .||u
(EB\)red|+|ed p|o|||e|+||ou o| B ce|| +ud ro|
+|e |,rp|or+ o| B ce|| o|||u. Cu|+ueou I ce||
|,rp|or+ +ccouu| |o| !0 o| cu|+ueou |,r
p|or+ |u ||+up|+u| p+||eu| ('ec||ou 20).
K+po| +|cor+ occu| |u |rruuoupp|eed ||+u
p|+u| |ec|p|eu| W||| +u |uc|deuce o| 0.5-5. A||
c+e +|e +oc|+|ed W||| K+po| +|cor+-+oc|+|ed
|e|pe.||u (K'n\) |u|ec||ou ('ec||ou 20).
*
C||u|c+| r+u||e|+||ou +|e d|cued |u ||e|| |epec||.e
ec||ou.
SkIN CANCkS ASS0CIAI0 WIIh 0kCAN
IkANSFIANIAII0N*
Conventional
Infections
Nocardia
Mycobacterium
CMV
EBV
VZV
CMV retinitus
MCV
HPV and manifestations
Candida
Aspergillus
0 1 2 3
Time after transplantation (months)
4 5 6
Cryptococcus
HSV
Bacterial
Viral
Fungal
Opportunistic (and latent) Infections
Timeline of Common Infections after Transplantation
Community-
acquired or
persistent
Infections
Endemic fungi (histoplasma, coccidioides)
Wound, nosocornial infections
C\n| | ||e |o|+|||, o| o|+u d,|uuc||ou c+ued
|, ||e +c||ou o| |||o|ucorp+||||e, |rruuo
corpe|eu| douo| ce|| ++|u| ||e ||ue o| +u
|rruuocorpe|eu| |o|.
C|+||.e|u|o| |e+c||ou (C\nk) | ||e e\p|e
|ou o| C\n| |u + pec|||c o|+u (e.., cu|+ueou
C\nk).
Acu|e cu|+ueou C\nk | ||e e+|||e| +ud ro|
||equeu| C\nk. ||.e| +ud C| ||+c| C\nk +|e +|o
corrou.
Acu|e cu|+ueou C\nk, uu+||, occu|||u 0-!0
d+, +||e| |oue r+||oW ||+up|+u|+||ou (B\I),
| c|+|+c|e||/ed |, |+|u| e|,||er+|ou r+cu|e,
o||eu |u pe|||o|||cu|+| p+||e|u p|o|e|u |o cou
||ueu| e|,||er+, e|,|||ode|r+, o| |o\|c ep|de|r+|
uec|o|,| (IE|).
C||ou|c cu|+ueou C\nk occu| > o0 d+, +||e|
+||oeue|c B\I +ud r+u||e| + ||c|euo|d +ud
c|e|ode|ro|d c|+ue.
CkAFI-vkSS-h0SI 0ISAS |C| . 9.99o.35
|C|0 . I3o.0
FI0MI0I0C
Iocdeoce Allogeneic BMT: 20-80% of suc-
cessful engiaftments. Autologous BMT: mild
cutaneous GVHR occuis in 8%. Low incidence
aftei blood tiansfusion in immunosuppiessed
patients, mateinal-fetal tiansfei in immunode-
ficiency disease.
ACI CIAN0S Cvhk
FAIh0CNSIS
With successful engiaftment, theie is ieplace-
ment of host maiiow by immunocompetent do-
noi cells capable of mounting an inflammatoiy
ieaction against the-foieign" tissue antigens of
the host. GVHR of specific host oigans-skin,
livei, oi GI tiact. Seveiity of GVHD ielated to
histocompatibility match between donoi and
iecipient and piepaiatoiy iegimen used.
Duiing the fiist 2 months aftei BMT (usually
between 10 and 30 days): mild piuiitus, local-
ized/geneialized; pain on piessuie, palms/soles.
Nausea/vomiting, abdominal pain; wateiy di-
aiihea. Jaundice; daik yellow uiine.
Sko Iesoos Initially, subtle, disciete mac-
ules and/oi papules on uppei tiunk, hands/feet
(Fig. 21-1), especially palms/soles. Painful. Mild
edema with violaceous hue, peiiungual and on
pinna. Eiythema often in peiifolliculai aiiay. If
contiolled/iesolved, eiythema diminishes with
subsequent desquamation (Fig. 21-2) and postin-
flammatoiy hypeipigmentation. If it piogiesses,
macules/papules become geneialized, confluent
(Fig. 21-3), and evolve into eiythiodeima. Sub-
epideimal bullae, especially ovei piessuie/tiauma
sites, palms/soles. Positive Nikolsky sign. If bul-
lae widespiead with iuptuie/eiosion, TEN-like
foim of acute cutaneous GVHR (see Section 8)
(Fig. 21-4). Foi staging, see Table 21-1.
IA8I 21-2 histo|oic Cradin Scheme for Acute Cutaneous CVhR
6rade 0escr|pt|oo
0 |o|r+| ||u o| c|+ue uo| |e|e|+||e |o |+||.e|u|o| d|e+e
B++| .+cuo||/+||ou o| ||e de|r+|ep|de|r+| juuc||ou
2 B++| .+cuo||/+||ou, uec|o||c ep|de|r+| ce||, |,rp|oc,|e |u ||e de|r| +ud/o| ep|de|r|
! 'u|ep|de|r+| c|e|| |o|r+||ou p|u |+de 2 c|+ue
+ 'ep+|+||ou o| ep|de|r| ||or de|r| p|u |+de 2 c|+ue
Ad+p|ed ||or |e|ue| KC e| +|. n||op+||o|o, o| |+||.e|u|o| |e+c||ou (C\nk) |u |ur+u |ec|p|eu| o| r+||oW ||or n|Ar+|c|ed ||||u
douo|. c|c|c| 3.!o1, 91+.
IA8I 21-1 C|inica| Stain of Acute
Cutaneous CVhR
. E|,||er+|ou r+cu|op+pu|+| e|up||ou |u.o|.|u
25 o| |od, u||+ce
2. E|,||er+|ou r+cu|op+pu|+| e|up||ou |u.o|.|u
25-50 o| |od, u||+ce
!. E|,|||ode|r+
+. Bu||+ |o|r+||ou
SCII0N 21 'K|| ||'EA'E' || 0kCA| A|| B0|E \Akk0w IkA|'||A|IAI|0| 54T
FICk 21-1 Acute cutaoeous Cvhk ||c|e|e +ud cou||ueu| e|,||er+|ou, ||+uc||u r+cu|e +ud |+|e|,
e|e.+|ed p+pu|e W||| |ud|||uc| |o|de| |u.o|.|u |+ud +ud ||uu|. |o|e |e|+||.e p+||u o.e| ||e re|+c+|pop|+
|+ue+| +ud p|o\|r+| |u|e|p|+|+ue+| jo|u|.
FICk 21-2 Acute cutaoeous Cvhk, remtto I|e r+cu|op+pu|+| |e|ou |+.e +cqu||ed + ||oWu||
|ue +ud ||e|e | |||| c+||u.
Mucvsu Lichen planus-like lesions in buc-
cal mucosa; eiosive stomatitis, oial and oculai
sicca-like syndiome; esophagitis/esophageal
stiictuies. Keiatoconjunctivitis.
Ceoera| Fodos Fevei, jaundice, nausea, vom-
iting, iight uppei quadiant pain/tendeiness,
ciamping, abdominal pain, diaiihea, seiositis,
pulmonaiy insufficiency, daik uiine.
Chemstry Elevated SGOT, biliiubin, alkaline
phosphatase.
0ermatopatho|oy Focal vacuolization
of basal cell layei, apoptosis of individual
keiatinocytes; mild peiivenulai mononucleai
cell infiltiate. Apposition of lymphocytes to
neciotic keiatinocytes (satellitosis); vacuoles
coalesce to foim subepideimal clefts sub-
epideimal blistei foimation. Endothelial cell
swelling. Immunocytochemistiy: HLA-DR ex-
piession of keiatinocytes piecedes moiphologic
changes and thus iepiesents impoitant, eaily
diagnostic sign (foi giading see Table 21-2).
Clinical findings confiimed by skin biopsy.
0IIereota| 0aooss Exanthematous diug
ieaction, viial exanthem, TEN, eiythiodeima.
FICk 21-3 Acute cutaoeous Cvhk ovo|vo the Iace oI a 10-year-o|d boy I|e |ud|.|du+| |e|ou
+|e cou||ueu|, ||e|e | |||| dequ+r+||ou, +ud ||e|e +|e e|o|ou ou ||e ||p. I|e rucou rer||+ue We|e
e.e|e|, |u.o|.ed.
FICk 21-4 Acute Cvhk, IN-|ke Cou||ueu| ep|de|r+| uec|o| W||| W||u|||u +ud d||odreu| o| ||e
uec|o||c ep|de|r|, e|o|ou, +ud |ero|||+|c c|u|. I|| e.e|e |e+c||ou |u.o|.ed ||e eu|||e ||u +ud | |ud|||u
u||+||e ||or IE|. || occu||ed +||e| +||oeue|c B\I +ud | c|e+||, + .e|, e.e|e coud|||ou.
C0kS AN0 Fk0CN0SIS
Mild to modeiate GVHR iesponds well to tieat-
ment. Piognosis of TEN-like GVHR is giave.
Seveie GVHD susceptible to infections-bac-
teiial, fungal, viial (CMV, HSV, VZV). Acute
GVHD is piimaiy oi associated cause of death
in 15-70% of BMT iecipients.
MANACMNI
Iopca| Glucocoiticoids.
Fhysca| PUVA, extiacoipoieal photopheiesis.
Systemc Methylpiednisolone 2 mg/kg pei
day, taciolimus 4-20 mg/d, cyclospoiine 12-15
mg/kg pei day PO; mycophenolate mofetil
2 g/d, etaneicept 25 mg twice daily, infiximab
10 mg/kg pei week.
Chk0NIC CIAN0S Cvhk
> 60 days aftei BMT. Evolving fiom acute GVHR
oi aiising de novo. Acute GVHR not always fol-
lowed by chionic GVHR. Clinical classification
thus distinguishes between quiescent onset,
piogiessive onset, and de novo chionic cutane-
ous GVHR. Chionic GVHR occuis in 25% of
iecipients of maiiow fiom an HLA-identical sib-
ling who suivive > 100 days.
Sko Iesoos
Flat-topped (lichen planus-like) papules of
violaceous coloi, initially on distal extiemities
but latei geneialized (Fig. 21-5) and/oi conflu-
ent aieas of deimal scleiosis (Fig. 21-6) with
oveilying scale iesembling scleiodeima mainly
on tiunk, buttocks, hips, and thighs. With moie
seveie disease, seveie geneialized scleiodeimoid
changes also involving face (Fig. 21-6) with
neciosis and ulceiation on acial and piessuie
sites. Haii loss; anhidiosis; nails: dystiophy,
anonychia; vitiligo-like hypopigmentation.
Mucosa Like eiosive/ulceiative lichen planus.
Ceoera| Fodos Chionic livei disease, gen-
eial wasting.
Chemstry Elevated ALT, AST, -glutamyl-
tiansfeiase.
0ermatopatho|oy Like |t|en |anus : hypei-
keiatosis, hypeigianulosis, mild iiiegulai acan-
thosis oi atiophy, modeiate basal vacuolization
and epideimal apoptosis, mild peiivasculai
mononucleai cell infiltiate, melanin inconti-
nence; like st|eroJerma : dense deimal scleiosis.
Loss of haii follicles, entiapment of sweat
glands.
C0kS AN0 Fk0CN0SIS
Scleiodeimoid GVHR with tight skin/joint
contiactuie may iesult in impaiied mobility,
ulceiations. Peimanent haii loss; xeiostomia,
xeiophthalmia, coineal ulceis, blindness. Mal-
absoiption. Mild chionic cutaneous GVHR
may iesolve spontaneously. Chionic GVHR
may be associated with iecuiient and occasion-
ally fatal bacteiial infections.
Clinical, histoiy, and histopathology. Diffeienti-
ate fiom lichen planus, lichenoid diug ieaction,
scleiodeima, all types of poikilodeima.
MANACMNI
Tota| glucocoiticoids, PUVA, and extiacoi-
poieal photopheiesis aie effective. Sysemt m-
munosuresson with piednisone, cyclospoiine,
azathiopiine, mycophenolate mofetil, meth-
otiexate, taciolimus, and thalidomide.
FICk 21-5 Chrooc cutaoeous Cvhk, |cheo p|aous-|ke \|o|+ceou |o ||oWu||, ||c|eu p|+uu-|||e
pe|||o|||cu|+| p+pu|e |ecor|u cou||ueu| ou ||e ||uu|, occu|||u ! rou|| +||e| +||oeue|c B\I.
FICk 21-6 Chrooc cutaoeous Cvhk, sc|erodermod 4. E|ou,W|||e |ouud doWu ||u +ud |e|+u
|ec|+|+ |u ||e 0,e+|o|d |o, |oWu |u ||. 2!. |u ||| c+e +cu|e C\nk e.o|.ed d||ec||, |u|o c||ou|c C\nk
+ud |u.o|.ed ||e eu|||e ||u o| ||e ||uu| +ud e\||er|||e. B. C|oeup .|eW o| ||e |+c| o| ||e +re p+||eu| W|||
po||||ode|r+|ou c|+ue (|,po +ud |,pe|p|reu|+||ou) +ud |e|+u|ec|+|+ |u ||e c|e|o||c ||u.
4 B
552
A0vkS CIAN0S
0kC kACII0NS
*

S E C I | 0 N 2 2
CIASSIFICAII0N
Immuoo|oca||y Medated AC0k See Table
22-1. It should be noted, howevei, that classifi-
cation of immunologically mediated ACDR ac-
coiding to the Gell and Coombs classification is
an oveisimplification because in most ieactions
both cellulai and humoial immune ieactions
aie involved. Nonimmunologic ieactions aie
summaiized in Table 22-2.
CI0IINS F0k ASSSSMNI
0F F0SSI8I AC0ks
Exclude alteinative causes, especially infec-
tions, in that many infections (especially
viial) aie difficult to distinguish clinically
fiom the adveise effects of diugs used to tieat
infections.
Examine inteival between intioduction of a
diug and onset of the ieaction.
Note any impiovement aftei diug with-
diawal.
Deteimine whethei similai ieactions have
been associated with the same compound.
Note any ieaction on ieadministiation of the
diug.
Ad.e|e cu|+ueou d|u |e+c||ou (AC|k) +|e
corrou |u |op||+||/ed (2-!) + We|| + |u
+r|u|+|o|, p+||eu| (>).
\o| |e+c||ou +|e r||d, +ccorp+u|ed |, p|u|||u,
+ud |eo|.e p|orp||, +||e| ||e o||eud|u d|u |
d|cou||uued.
'e.e|e, |||e|||e+|eu|u AC|k do occu| +ud +|e
uup|ed|c|+||e.
| -| :c : .|c||, c|| ||-
||: -- !-c||, c! |
|- ||- || :!-c| ||- !||--|c| !c
| c !!-|, c-c -|
||u e|up||ou +|e c+ued |, |rruuo|o|c o|
uou|rruuo|o|c rec|+u|r +ud +|e p|o.o|ed
|, ,|er|c o| |op|c+| +dr|u|||+||ou o| + d|u.
I|e r+jo|||, +|e |+ed ou + |,pe|eu|||.||,
rec|+u|r +ud +|e ||u |rruuo|o|c +ud r+,
|e o| |,pe |, ||, |||, o| |\.
|C|9 . 995.2
|C| . 0 . I33.1
Skin ieactions oi changes iegulaily occuiiing aftei high dose oi piolonged administiation of ceitain diugs like
glucocoiticoids, ietinoids, cyclospoiine, and otheis aie not discussed in this section but thioughout the book
whenevei these diugs aie discussed in gieatei detail.
FIN0INCS IN0ICAIINC F0SSI8I IIF-
IhkAININC AC0k
Skin pain
Confluent eiythema
Facial edema oi cential facial involvement
Palmai/plantai painful eiythema
Concomitant eiosive mucous membiane in-
volvement
Blisteis of epideimal detachment
Positive Nikolsky sign
Mucous membiane eiosions
Uiticaiia
Swelling of the tongue
High fevei (tempeiatuie > 40 C)
Enlaiged lymph nodes
Aithialgia
Shoitness of bieath, wheezing, hypotension
Palpable puipuia
Skin neciosis
SCII0N 22 A|\Ek'E CuIA|E0u' |kuC kEACI|0|' 553
IA8I 22-1 |mmuno|oica||y Nediated Adverse Cutaneous 0ru Reactions*
xamp|es oI
Iype oI keactoo Fathoeoess Causatve 0ru C|oca| Fatteros
I,pe | |Ered|+|ed, |eu|c||||u, u|||c+||+/+u|oeder+
|rred|+|e|,pe o||e| +u||||o||c o| ||u/ruco+, eder+
|rruuo|o|c |e+c||ou o| o||e| o|+u, +ud
+u+p|,|+c||c |oc|
I,pe || ||u c,|o|o\|c +u|||od|e |eu|c||||u, u||ou+r|de, |e|ec||+e due |o
c+ue |,| o| ce|| uc| qu|u|d|ue, |ou|+/|d |||or|oc,|opeu|c
+ p|+|e|e| o| |eu|oc,|e pu|pu|+, d|u|uduced
perp||u
I,pe ||| |C o| |\ +u|||od|e |rruuo|o|u||u, \+cu||||, u|||c+||+,
|o|red |o d|u, |rruue +u||||o||c, |||u\|r+|, e|ur |c|ue
corp|e\e depo||ed |u |u|||\|r+|
r+|| .ee| +c||.+|e
corp|ereu| +ud |ec|u||reu|
o| |+uu|oc,|e
I,pe |\ Ce||red|+|ed |rruue |e+c||ou, 'u||+re||o\+/o|e, \o|||||||o|r e\+u||er+|ou
eu|||/ed |,rp|oc,|e +u||cou.u|+u|, |e+c||ou, ||\ed d|u
|e+c| W||| d|u, |||e|+||u +||opu||uo| e|up||ou, ||c|euo|d
c,|o||ue, W||c| |||e| e|up||ou, '|e.eulo|uou
cu|+ueou |u||+rr+|o|, ,ud|ore, |o\|c ep|de|r+|
|epoue uec|o|,|
Cou|+c| eu|||.||,
*A||e| ||e Ce|| +ud Coor| c|+|||c+||ou o| |rruue |e+c||ou.
'ee 'ec||ou 2.
IA8I 22-2 Nonimmuno|oic 0ru Eruptions
|!,:c, ke+c||ou due |o |e|ed||+|, eu/,re de||c|euc|e
|!.!c| !,:c, | \ec|+u|r uo| ,e| |uoWu
c |:c| ,|-: !
C|c| ke+c||ou +|e doe depeudeu|, |+ed ou ||e |o|+| +rouu| o| d|u |ue|ed.
p|reu|+||ou due |o o|d, +r|od+|oue, o| r|uoc,c||ue
|-c:| !- | :|c| ke+c||ou |+.e + |o\|c p+||oeue| |u| c+u +|o |e |rruuo|o|c |u u+|u|e
| c ! +|| ||c.|-| (ee 'ec||ou 0)
c!c| (||-|.|,)
||c:,/|:|, | c 5||uo|ou|+c||, |r|qu|rod
|:c||, c|-! !
1||, |, |:c||, c|-! C|ucoco|||co|d
!
CIINICAI IFS 0F A0vkS 0kC kACII0NS
ACDRs can be exanthematous and can mani-
fest as uiticaiia/angioedema, anaphylaxis and
anaphylactoid ieactions, oi seium sickness; they
can mimic oi cause deimatoses that can also
have othei causes; they can piesent as cutane-
ous neciosis, pigmentation, alopecia, hypeitii-
chosis; and they can induce nail changes. An
oveiview is piesented in Table 22-3.
IA8I 22-3 Iypes of C|inica| AC0Rs
Iype 0rus Commeot
BA5lC kEACIl0N5
E\+u||er+|ou Au, \o| corrou, |u|||+|
|e+c||ou |e+c||ou uu+||, + d+, +||e|
d|u |u|+|e, |ecu| +||e|
|ec|+||eue (ee p+e 5o!), d|u
|,pe|eu|||.||, ,ud|ore,
|u|||+||, |ud|||uu||+||e (ee
p+e 5o3) (||. 22 +ud 222)
||\ed d|u e|up||ou 'ee I+||e 225 'ee p. 5oo
u|||c+||+/+u|oeder+ Ap|||u, uou|e|o|d+| +u|| 'ecoud ro| corrou, uu+||,
|u||+rr+|o|, d|u (|'A||), code|ue, W||||u !o | +||e| |u|||+|
peu|c||||u, op|+|e, +rp|e|+r|ue, e\pou|e, W||||u r|uu|e +||e|
po|,r,\|ue B, +||op|ue, |,d|+|+/|ue, |ec|+||eue (ee p+e 5o!)
|u|||\|r+|, peu|+r|d|ue, qu|u|ue, (||. 22o +ud 221)
|+d|ocou||+| red|+, +u|o|eu|u
cou.e|||u eu/,re (ACE) |u|||||o|
(ee I+||e 22+)
Au+p|,|+\| +ud Au||||o||c, e\||+c| o| +||e|eu, \o| e||ou |,pe o| AC|k,
+u+p|,|+c|o|d |e+c||ou |+d|ocou||+| red|+, rouoc|ou+| W||||u r|uu|e +ud |ou|,
+u|||od|e (ee I+||e 22+) ro|e corrou W||| o|+| ||+u
p+|eu|e|+| d|u +dr|u|||+||ou.
|u|e|r|||eu| +dr|u|||+||ou o| d|u
r+, p|ed|poe |o +u+p|,|+\|
'e|ur |c|ue |\|, +u||||o||c, |o.|ue e|ur 5 |o 2 d+, +||e| |u|||+| e\pou|e
+||ur|u (ued |o| ooc,|e |e|||e.+| ! |. |e.e|, u|||c+||+,
|u |u .|||o |e|||||/+||ou), ce|+c|o|, +||||+||+
ce|p|o/||, |up|op|ou, r|uoc,c||ue, !c (:|-|-) |. |e.e|,
|||u\|r+|, |u|||\|r+| u|||c+||+, +u|oeder+, +||||+||+,
+|||||||, |,rp|+deuop+||,,
eo|uop||||+, uep|||||,
eudoc+|d|||.
AC0k MlMlCkY 0F 0IHEk 0EkMAI05E5
Acue||o|r e|up||ou C|ucoco|||co|d, +u+|o||c |e|o|d, 'ee 'ec||ou +ud ||. 225
cou||+cep||.e, |+|oeu, |ou|+/|d,
|||||ur, +/+|||op||ue, d+u+/o|
Bu||ou e|up||ou |+p|o\eue, u+||d|\|c +c|d, |u|oer|de, ||\ed d|u e|up||ou, (||. 2233)
o\+p|o/|u, peu|c|||+r|ue, p||o\|c+r, d|u|uduced .+cu||||, '|e.eu
|e||+c,c||ue lo|uou ,ud|ore ('l'), |o\|c
ep|de|r+| uec|o|,|
(IE|), po|p|,||+,
peudopo|p|,||+, d|u|uduced
perp||u, d|u|uduced
perp||o|d, d|u|uduced
||ue+| |A d|e+e, |u||+e o.e|
p|eu|e +|e+ |u
ed+|ed p+||eu| (||. 229)
|e|r+|or,o||||||e |e+c||ou |eu|c|||+r|ue, |'A||, 'ee 'ec||ou +
c+||+r+/ep|ue
Iype 0rus Commeot
||u |,pe|eu|||.||, ,ud|ore Au||ep||ep||c d|u, u||ou+r|de, \|r|c e\+u||er+|ou |e+c||ou,
+ud o||e| ,|er|c |u.o|.ereu| (ee
p+e 5o3) (||. 220)
Ec/er+|ou e|up||ou E||,|eued|+r|ue, +u|||||+r|ue, ',|er|c +dr|u|||+||ou o| +
+r|uop|,|||ue/+r|uop|,|||ue d|u |o +u |ud|.|du+| W|o
uppo||o||e, p|oc+|ue/|eu/oc+|ue, |+ |eeu p|e.|ou|, eu|||/ed
|od|de, |od|u+|ed o|+u|c corpouud, |o ||e d|u |, |op|c+|
|+d|o|+p||c cou||+| red|+/|od|ue, +pp||c+||ou c+u p|o.o|e +
||ep|or,c|u, |+u+r,c|u, p+|+ror,c|u, W|dep|e+d ec/er+|ou
eu|+r|c|u/ueor,c|u u||+|e, de|r+|||| (,|er|c
u|||o|,ce||u |+||e|/u|||o|,ce||u cou|+c||,pe de|r+||||, ee
o|u|reu|, d|u||||+r/|||u|+r 'ec||ou 2) o| u|||c+||+
E|,||er+ ru||||o|re, 'l', IE| Au||cou.u|+u|, u||ou+r|de, 'ee 'ec||ou 1 +ud 3
+||opu||uo|, |'A|| (p||o\|c+r)
E|,||er+ uodour 'u||ou+r|de, o||e| +u||r|c|o||+| 'ee 'ec||ou 1
+eu|, +u+|e|c, o|+| cou||+cep||.e,
|+uu|oc,|e co|ou,||ru|+||u
|+c|o| (CC'|)
E\|o||+||.e de|r+|||| 'u||ou+r|de, +u||r+|+||+|, 'ee 'ec||ou 3
+ud e|,|||ode|r+ p|eu,|o|u, peu|c||||u
||c|euo|d e|up||ou Co|d, |e|+ ||oc|e|, ACE |u|||||o|, 'ee 'ec||ou 1 +ud 2.
epec|+||, c+p|op|||, ee +|o \+, |e e\|eu|.e, occu|||u
I+||e 1 Wee| |o rou|| +||e| |u|||+||ou
o| d|u ||e|+p,, r+, p|o|e
|o e\|o||+||.e de|r+||||
Adue\+| |u.o|.ereu| r+, |eu||
|u +|opec|+, +u||d|o|
keo|u||ou +||e| d|cou||uu+||ou
|oW, -+ rou||, up |o
2+ rou|| +||e| o|d
\+, |e p|o|od|||||u|ed o|
|u||ou
0|+| |u.o|.ereu| occu| W|||
ore d|u
|upu e|,||er+|ou (|E) ||oc+|u+r|de, |,d|+|+/|ue, |ou|+/|d, 'ee 'ec||ou +.
r|uoc,c||ue, +ce|u|o|o|, C+
2-
c|+uue| 5 o| c+e o| ,|er|c |E +|e
||oc|e|, ACE |u|||||o|, doce|+\e| d|u|uduced
Cu|+ueou r+u||e|+||ou,
|uc|ud|u p|o|oeu|||.||,,
|oWe.e|, u|||c+||+, e|,||er+
ru||||o|re|||e |e|ou, k+,u+ud
p|euoreuou +|e uo| corrou
||o|oeu|||.||, 'ee I+||e 0+ |o 0o 'ee 'ec||ou 0
||o|o|o\|c, p|o|o+||e||c, o|
p|o|ocou|+c|
|||,||+| |oe+-|||e e|up||ou Co|d, c+p|op|||, |r+||r||, |o| c||u|c+| +ppe+|+uce, ee
+ud o||e| 'ec||ou 1
|eudo|,rp|or+ ||eu,|o|u, c+||+r+/ep|ue, +||opu||uo|, |+pu|+| e|up||ou W||| +
+u||dep|e+u|, p|euo|||+/|ue, |||o|o, r|r|c||u |,rp|or+
|eu/od|+/ep+r, +u|||||+r|ue,
|e|+ ||oc|e|, ||p|d|oWe||u +eu|,
c,c|opo||ue, |peu|c|||+r|ue
(:|-!)
IA8I 22-3 (Cont/nued)
Iype 0rus Commeot
|eudopo|p|,||+ Ie||+c,c||ue, |u|oer|de, u+p|o\eue 'ee 'ec||ou 0 +ud p+e 51+,
(||. 22!)
|o||+||o|r e|up||ou Au||r+|+||+|, |e|+||oc|e|, 'ee 'ec||ou !
|||||ur +||, |'A||, |u|e||e|ou,
peu|c|||+r|ue, re||,|dop+
|u|pu|+ |eu|c||||u, u||ou+r|de, qu|u|ue, 'ee 'ec||ou 9.
|ou|+/|d nero|||+e |u|o ro|||||||o|r
AC|k occu| uo| uucorrou|,
ou ||e |e
||o|e|.e p|reu|ed pu|pu|+
+|o |epo||ed +oc|+|ed W|||
d|u (ee 'ec||ou 1)
|u|u|+| e|up||ou Arp|c||||u, +ro\|c||||u, r+c|o||de, Acu|e eue|+||/ed e\+u||er+|ou
|e||+c,c||ue, |e|+ ||oc|e|, C+
2-
pu|u|o| (ACE|, p+e 5o)
c|+uue| ||oc|e| \u| |e d|||e|eu||+|ed ||or
EC|k |u|||||o| (||. 225) pu|u|+| po||+|, eo|uop||| |u
||e |u|||||+|e ue| ACE|
'c|e|ode|r+|||e |e+c||ou |eu|c|||+r|ue, ||eor,c|u, ||oroc|,p||ue, 'ee 'ec||ou +
|+.+|p|o+|e, 5|,d|o\,||,p|op|+u,
doce|+\e|, erc||+||ue, +ce|+u|||de
cou|+|u|u |+peeed coo||u o||
'Wee| ,ud|ore A|||c |e||uo|c +c|d, cou||+cep||.e, 'ee 'ec||ou 1
CC'|, |+uu|oc,|er+c|op|+e C'|
(C\C'|), r|uoc,c||ue, |r+||u||,
|||re||op||ru||+re||o\+/o|e
\+cu|||| ||op,||||ou|+c||, |,d|+|+/|ue, CC'|, 'ee 'ec||ou +
C\C'|, +||opu||uo|, ce|+c|o|,
r|uoc,c||ue, peu|c|||+r|ue, p|eu,|o|u,
|o||e||uo|u
AC0k-kIAI0 FICMNIAII0N
AC|k p|reu|+||ou Ar|od+|oue, r|uoc,c||ue, c|o|+/|r|ue, 'ee p+e 510
/|do.ud|ue, |,d+u|o|u, c,|o|o\|c Aoc|+|ed W||| po||u||+rr+|o|,
+eu|, |e+., re|+|, |o|roue, |,pe|p|reu|+||ou, |uc|e+ed
c||o|p|or+/|ue, ||eor,c|u re|+u|u ,u||e|, |uc|e+ed
||po|uc|u ,u||e|, o|
cu|+ueou depo|||ou o| d|u
|e|+|ed r+|e||+| (||. 22,
222)
AC0k-kIAI0 NCk0SIS
AC|k uec|o| w+||+||u, |ep+||u, |u|e||e|ou , 'ee p+e 515 (||. 22+,
c,|o|o\|c +eu| 225, 22o).
0||e|
AC|k |e|+|ed |o c|ero||e|+p, 'ee p+e 519
A|opec|+ 'ee 'ec||ou !2
n,pe||||c|o| 'ee 'ec||ou !2
|+|| c|+ue 'ee 'ec||ou !!
SCII0N 22 A|\Ek'E CuIA|E0u' |kuC kEACI|0|' 55T
FI0MI0I0C
Ae oI 0oset Less common in the veiy
young.
Iocdeoce Most common type of cutaneous
diug ieaction.
Drugs w| a |g| ro|a||y o[ reaton
(3 -5%): penicillin and ielated antibiotics, cai-
bamazepine, allopuiinol, gold salts (10-20%).
MeJum ro|a||y : sulfonamides (bacte-
iiostatic, antidiabetic, diuietic), nonsteioidal
anti-inflammatoiy diugs (NSAIDs), hydantoin
deiivatives, isoniazid, chloiamphenicol, eiyth-
iomycin, stieptomycin. Low ro|a||y (<1%):
baibituiates, benzodiazepines, phenothiazines,
tetiacyclines (Table 22-3).
Exact mechanism unknown. Piobably delayed
hypeisensitivity. In Epstein-Baii viius (EBV)
and cytomegaloviius (CMV) mononucleosis,
Au e\+u||er+|ou d|u |e+c||ou (e|up||ou) | +u
+d.e|e |,pe|eu|||.||, |e+c||ou |o +u |ue|ed o|
p+|eu|e|+||, +dr|u||e|ed d|u.
Cu|+ueou e|up||ou ||+| r|r|c + re+|e|||e
.||+| e\+u||er.
',|er|c |u.o|.ereu| | |oW.
',, . \o|||||||o|r d|u |e+c||ou, r+cu|op+pu
|+| d|u |e+c||ou.
XANIhMAI0S 0kC kACII0NS |C|9 . 995.2
|C|0 . I33.1
hemato|oy Eosinophil count > 1000/L.
Lymphocytosis with atypical lymphocytes.
Chemstry Abnoimal iesults of livei function
tests.
0IACN0SIS
Usually made on clinical findings. Lesional skin
biopsy is helpful in defining the type of ieaction
pattein occuiiing but not in identifying the
offending diug. Skin tests and iadioalleigosoib-
ent tests aie helpful in diagnosing IgE-mediated
type I hypeisensitivity ieactions, moie specifi-
cally to penicillins.
MANACMNI
In most cases, the implicated oi suspected
diug should be discontinued. In some, such
as with moibillifoim eiuptions, the offending
diug can be continued and the eiuption may
iesolve. In cases of uiticaiia/angioedema oi
eaily Stevens-Johnson syndiome (SJS)/toxic
epideimal neciolysis (TEN), the ACDR can
be life-thieatening, and the diug must be dis-
continued.
exanthematous diug ieactions occui veiy fie-
quently but aie piobably not alleigic.
Moooouc|eoss Up to 100% of patients with
piimaiy EBV oi CMV infection (infectious
mononucleosis syndiome) given ampicillin oi
amoxicillin develop an exanthematous diug
eiuption.
hIv[AI0S IoIectoo 50-60% of HIV-infected
patients who ieceive sulfa diugs (i.e., tiimetho-
piim-sulfamethoxazole) develop an eiuption.
With immune iestitution with highly-active
antiietioviial theiapy (HAART), pieviously tol-
eiant individuals may develop ACDR as CD4-
cell count iises.
0ru hstory Incieased incidence of ieactions
in patients on allopuiinol given ampicillin/
amoxicillin.
Fror 0ru Seosttatoo Patients with piioi
histoiy of exanthematous diug eiuption
will most likely develop a similai ieaction if
iechallenged with same diug. About 10% of pa-
tients sensitive to penicillins who aie given ce-
phalospoiins will exhibit cioss-diug sensitivity
and develop eiuption. Patients sensitized to one
sulfa-based diug may cioss-ieact with anothei
categoiy of the diug in 20% of cases.
0oset Eur|y ReuctIvn In pieviously sensi-
tized patient, eiuption staits within 2 oi 3 days
aftei ieadministiation of diug.
Lute ReuctIvn Sensitization occuis duiing ad-
ministiation oi aftei completing couise of diug;
peak incidence at ninth day aftei administia-
tion. Howevei, ACDR may occui at any time
between the fiist day and 3 weeks aftei the be-
ginning of tieatment. Reaction to penicillin can
begin 2 weeks aftei diug is discontinued.
Sko Symptoms Usually quite piuiitic, distuibs
sleep. Painful skin lesions suggest development
of a moie seiious ACDR, such as TEN.
Systems kevew Fevei, chills.
Sko Iesoos Macules and/oi papules, a few
millimeteis to 1 cm in size (Figs. 22-1 and 22-2).
Biight oi diug" ied. Resolving lesions have
hues of tan and puiple. In time, lesions become
confluent foiming laige macules, polycyclic/
gyiate eiythema, ieticulai eiuptions, sheet-
like eiythema (Fig. 22-1), eiythiodeima; also
eiythema multifoime-like. Puipuia may be
seen in lesions of lowei legs. In individuals with
thiombocytopenia, exanthematous eiuptions
can mimic vasculitis because of intialesional
hemoiihage. Scaling and/oi desquamation may
occui with healing.
FICk 22-1 xaothematous dru eruptoo: ampc||o ',rre|||c+||, +||+ued, ||||||, e|,||er+|ou
r+cu|e +ud p+pu|e, d|c|e|e |u ore +|e+ +ud cou||ueu| |u o||e|, ou ||e ||uu| +ud ||e e\||er|||e.
DIstrIhutIvn Symmetiic (Fig. 22-1). Almost
always on tiunk and extiemities. Confluent le-
sions in inteitiiginous aieas, i.e., axilla, gioin,
infiamammaiy aiea. Palms and soles vaiiably
involved. In childien, may be limited to face and
extiemities. May spaie face, nipple, peiiaieolai
aiea, suigical scai. Reactions to ampicillin usu-
ally appeai initially on the elbows, knees, and
tiunk, extending symmetiically to most aieas
of the body.
Mucous Membraoes Enanthem on buccal mu-
cosa.
keactoos to SpecIc 0rus (Se|ected) AmpI-
cI||In, AmvrIcI||In In up to 100% of patients
with EBV oi CMV mononucleosis syndiome.
NSA1DS Incidence: 1-3%. Site: tiunk, pies-
suie aieas. Onset: 1-2 weeks aftei beginning
theiapy.
BurhIturutes Site: face, tiunk. Onset: few days
aftei initiation of theiapy. Cioss-ieactivity with
othei baibituiates: not univeisal.
NItrv]uruntvIn Associated findings: fevei, pe-
iipheial eosinophilia, pulmonaiy edema, chest
pain, dyspnea. Onset: 2 weeks aftei initiation of
theiapy; within houis if pieviously sensitized.
HyduntvIn DerIvutIves Maculai oi confluent
eiythema. Begins on face, spieads to tiunk and
extiemities. Onset: 2 weeks aftei initiation of
theiapy. Associated findings: fevei, peiipheial
eosinophilia; facial edema; lymphadenopathy
(can mimic lymphoma histologically).
1svnIuzId Moibillifoim; may evolve to ex-
foliative deimatitis. Associated findings: fevei;
hepatitis.
BenzvdIuzepInes Raie. Onset: few days aftei
initiation of theiapy. Rechallenge: fiequently
iash does not occui.
PhenvthIuzInes Begins on face, spieads to
tiunk (mainly back) and extiemities. Onset: be-
tween second and thiid weeks aftei initiation of
theiapy. Associated findings: peiioibital edema.
Rechallenge: iash may not occui. Ciossieactiv-
ity: common.
CurhumuzepIne Moiphology: diffuse eiythema;
seveie eiythiodeima may follow. Site: begins on
face, spieads iapidly to all aieas; may occui in
FICk 22-2 xaothematous dru eruptoo: ampc||o o a pateot wth 8v moooouc|eoss
Cou||ueu| r+cu|op+pu|+| |e|ou, eue|+||/ed.
photodistiibution. Onset: 2 weeks aftei initiation
of theiapy. Associated findings: facial edema.
Su|]vnumIdes Incidence: common in up to 50
-60% of HIV/AIDS-infected patients. Moiphol-
ogy: moibillifoim, eiythema multifoime-like.
A||vpurInv| Incidence: 5%. Moiphology:
moibillifoim. Begins on face, spieads iapidly to
all aieas; may occui in photodistiibution. On-
set: 2-3 weeks aftei initiation of theiapy. Associ-
ated findings: facial edema; systemic vasculitis,
especially involving kidneys. Rash may fade in
spite of continued administiation.
Gv|d Su|ts Incidence: 10-20% of patients; dose-
ielated. Moiphology: diffuse eiythema; exfolia-
tive deimatitis, lichenoid, hemoiihagic, bullous,
oi pityiiasis iosea-like eiuptions may follow.
Ceoera| xamoatoo Diug fevei. Findings as-
sociated with the indication foi diug adminis-
tiation.
hemoram Peiipheial eosinophilia.
0ermatopatho|oy Peiivasculai lymphocytes
and eosinophils.
Clinical diagnosis, at times confiimed by histo-
logic findings, coiielated with histoiy of diug
administiation.
0IIereota| 0aooss Includes all exanthema-
tous eiuptions: Viial exanthem (often begins on
face, piogiesses to tiunk; may be accompanied
by conjunctivitis, lymphadenopathy, fevei), sec-
ondaiy syphilis, atypical pityiiasis iosea, eaily
widespiead alleigic contact deimatitis.
C0kS
Aftei discontinuation of diug, iash usually
fades; howevei, it may woisen foi a few days.
The eiuption may also begin aftei the diug has
been discontinued. Occasionally fades even
though diug is continued. Eiuption usually
iecuis with iechallenge, although not always.
In some cases of exanthematous ampicillin
ieactions, ieadministiation of the diug does
not cause the eiuption. Duiation of ampicil-
lin eiuption aftei discontinuation of diug:
3-5 days. If diug is continued, exfoliative
deimatitis may develop. Of moie concein,
a moibillifoim eiuption may be the initial
piesentation of a moie seiious eiuption, i.e.,
SJS, TEN, diug hypeisensitivity syndiome, oi
seium sickness.
MANACMNI
The definitive step in management is to identify
the offending diug and discontinue it.
Iodcatoos Ior 0scootouatoo oI 0ru Ui-
ticaiia (concein foi anaphylaxis), facial edema,
pain, blisteis, mucosal involvement, ulceis, pal-
pable oi extensive puipuia, fevei, lymphaden-
opathy.
Symptomatc Ireatmeot Oial antihistamine to
alleviate piuiitus.
C|ucocortcods Pvtent TvpIcu| Prepuru-
tIvn May help speed iesolution of eiuption.
Oru| vr 1V Piovides symptomatic ielief. If of-
fending diug cannot be substituted oi omitted,
systemic glucocoiticoids can be administeied
to tieat the ACDR; also, to induce moie iapid
iemission.
Freveotoo Patients must be awaie of theii
specific diug hypeisensitivity and that othei
diugs of the same class can cioss-ieact.
Although an exanthematous diug eiuption
may not iecui if the diug is given again, ie-
administiation is best avoided by using a dif-
feient agent. Weaiing a medical aleit biacelet
is advised.
1:|- --c|c-! -c||-c| ||
(ACE|) | +u +cu|e |e||||e e|up||ou ||+| | o||eu
+oc|+|ed W||| |eu|oc,|o| (||. 22!). A||e|
d|u +dr|u|||+||ou, || r+, |+|e -! Wee|
|e|o|e ||u |e|ou +ppe+|, |oWe.e|, |u p|e.|ou|,
eu|||/ed p+||eu|, ||e ||u ,rp|or r+, occu|
W||||u 2-! d+,.
0ue| | +cu|e, ro| o||eu |o||oW|u d|u |u|+|e,
|u| .||+| |u|ec||ou c+u +|o |||e| ||e d|e+e.
ACE| |,p|c+||, p|eeu| W||| uou|o|||cu|+| |e|||e
pu|u|e occu|||u ou + d|||ue, eder+|ou e|,
||er+ (||. 22!).
\+, |e |||eu|+||, d|pe|ed (||. 22!) o|
|ouped (||. 22+), uu+||, |+|||u |u ||e |o|d
+ud/o| ||e |+ce.
|e.e| +ud e|e.+|ed ||ood ueu||op||| +|e cor
rou.
n||op+||o|o, |,p|c+||, |oW pou||o|r u|
co|ue+| +ud/o| |u||+ep|de|r+| pu|u|e, + r+||ed
eder+ o| ||e p+p|||+|, de|r|, +ud e.eu|u+||,
.+cu||||, eo|uop|||, +ud/o| |oc+| uec|o| o|
|e|+||uoc,|e.
|u|u|e |eo|.e pou|+ueou|, |u 5 d+, +ud
eue|+||/ed dequ+r+||ou occu| +pp|o\|r+|e|,
2 Wee| |+|e|.
||||e|eu||+| d|+uo| |uc|ude pu|u|+| po||+|,
||e |,pe|eu|||.||, ,ud|ore |e+c||ou W||| pu
|u|+||ou, u|co|ue+| pu|u|+| de|r+|o| ('ued
douw||||uou d|e+e), pu|u|+| .+cu||||, o|
IE|, epec|+||, |u e.e|e c+e o| ACE|.
I|e e||r+|ed |uc|deuce |+|e o| ACE| | +pp|o\|
r+|e|, -5 c+e pe| r||||ou pe| ,e+|.
1:-| -| (ee 'ec||ou ) +|e +oc|
+|ed W||| |od|de, ||or|de, +d|euoco|||co||op|c
|o|roue (ACIn), |ucoco|||co|d, |ou|+/|d, +u
d|oeu, |||||ur, +c||uor,c|u |, p|eu,|o|u. I|e
EC|k |,|o|ue ||u+e |u|||||o| e||o||u||, e||||u||,
ce|u\|r+|, p+u||urur+| p|oduce pu|u|e ||+|
+|e uo| +cue||o|r +ud e|up| |u ||e |+ce (||. 225)
|u| c+u e|up| +|o |u +|,p|c+| +|e+, uc| + ou
||e +|r +ud |e, +ud +|e ro| o||eu rouoro|
p|ou. Coredoue +|e uu+||, +|eu|.
FSIIAk kFII0NS |C|9 . 995.2
|C|0 . I33.1
FICk 22-3 Fustu|ar dru eruptoo: acute eoera|ted exaothematous pustu|oss (ACF) \u|||p|e
||u, uou|o|||cu|+| pu|u|e ++|u| ||e |+c||ouud o| d|||ue e|,||er+ ||+| |||| +ppe+|ed |u ||e |+|e |o|d +ud
||eu co.e|ed ||e eu|||e ||uu| +ud ||e |+ce.
FICk 22-4 Fustu|ar dru erup-
too: ACF \u|||p|e |e|||e pu|u|e
u||ouuded |, ||e|,|ed e|,||er+ |u
+ 53,e+|o|d |er+|e W|o |+d |e.e|
+ud |eu|oc,|o|. |u cou||+| |o ||e
d|er|u+|ed pu|u|e |u ||. 22!,
|e|e ||e pu|u|e |oW + |eudeuc, |o|
|oup|u. ||||e|eu||+| d|+uo| o| .ou
/ur|uc| pu|u|+| po||+| (corp+|e
W||| ||. !! +ud !+).
FICk 22-5 Fustu|ar dru eruptoo: er|otob I|| pu|u|+| e|up||ou occu||ed |u + p+||eu| W|o |+d
|ece|.ed +u +u||ECk rouoc|ou+| +u|||od, |o| c+uce| o| ||e co|ou. A|||ou| ||ee e|up||ou uu+||, +|e uo|
+oc|+|ed W||| coredoue, ||| p+||eu| d|d |+.e coredoue ou ||e uoe |u| uo| |u ||e o||e| 'e|o|||e|c
+|e+ uc| + ||e |o|e|e+d +ud ||e c|ee|.
4 B
CIASSIFICAII0N 0F kIICAkIAI[
ANCI00MA AC0ks
Immune-mediated
IgE-mediated: penicillin
Complement- and immune complex-me-
diated: penicillin, immunoglobulins, whole
blood
Nonalleigic uiticaiial ACDR
Analgesics/NSAIDs inhibit/block cyclooxy-
genase in piostaglandin synthesis
Radio contiast media
||u|uduced u|||c+||+ +ud +u|oeder+ occu|

due |o + .+||e|, o| rec|+u|r (ee I+||e 22)
+ud +|e c|+|+c|e||/ed c||u|c+||, |, ||+u|eu| W|e+|
+ud |+|e| eder+|ou +|e+ ||+| |u.o|.e ||e de|
r| +ud u|cu|+ueou ||ue (+u|oeder+).
|u ore c+e, cu|+ueou u|||c+||+/+u|oeder+
| +oc|+|ed W||| ,|er|c +u+p|,|+\|, W||c| |
r+u||e|ed |, |ep||+|o|, d|||e, .+cu|+| co|
|+pe, +ud/o| |oc|.
||u c+u|u u|||c+||+/+u|oeder+ +ud +u+p|,
|+\| +|e |||ed |u I+||e 22+.
0kC-IN0C0 ACI kIICAkIA, ANCI00MA,
0MA, AN0 ANAFhIAXIS (See a|so Sectoo 14)
ACE inhibitois: inhibition of kinin me-
tabolism
Calcium channel blockeis
Diugs ieleasing histamine
Ime Irom Iota| 0ru xposure to Appearaoce
oI rtcara 1gE-MedIuted Initial sensitiza-
tion, usually 7-14 days; uiticaiia may occui
while the diug is still being administeied oi
aftei it is discontinued. In pieviously sensitized
individuals, usually within minutes oi houis.
IA8I 22-4 0rus Causin Urticaria/Anioedema/Anaphy|a\is

0ru Iype SpecIc 0rus
Au||||o||c +ud c|ero||e|+peu||c |eu|c||||u. +rp|c||||u, +ro\|c||||u, d|c|o\+c||||u, re/|oc||||u,
+eu| peu|c||||u C, peu|c||||u \, |||c+|c||||u. Cep|+|opo||u,
|uc|ud|u ||||deue|+||ou u||ou+r|de +ud de||.+||.e
C+|d|o.+cu|+| d|u Ar|od+|oue, p|oc+|u+r|de
|rruuo||e|+peu||c, .+cc|ue Au|||,rp|oc,|e e|ur, |e.+r|o|e, |o|e e|ur,
rouoc|ou+| +u|||od|e
C,|o|+||c +eu| |Ap+|+|u+e, ||eor,c|u, c|p|+||u, d+uuo|u||c|u,
5||uo|ou|+c||, p|oc+||+/|ue, |||o|ep+
Au|o|eu|ucou.e|||u eu/,re C+p|op|||, eu+|op|||, ||u|uop|||
|u|||||o|
C+|c|urc|+uue| ||oc|e| |||ed|p|ue, d||||+/er, .e|+p+r||
||u |e|e+|u |||+r|ue Ceu||+||, +c||u d|u. ro|p||ue, repe||d|ue, +||op|ue,
code|ue, p+p+.e||ue, p|op+u|d|d, +||+\+|oue
\uc|e |e|+\+u|. ||u|ocu|+||ue, ucc|u,|c|o||ue
',rp+||or|re||c. +rp|e|+r|ue, |,|+r|ue
n,po|eu|.e +eu|. |,d|+|+/|ue, |o|+/o||ue,
|||re||+p|+u c+r,|+|e
Au||r|c|o||+| +eu|. peu|+r|d|ue, p|op+r|d|ue,
||||+r|d|ue, qu|u|ue, .+ucor,c|u
k+d|o|+p||c cou||+| red|+ +ud o||e|
1mmune Cvmp|er-MedIuted Initial sensiti-
zation, usually 7-10 days, but as long as 28 days;
in pieviously sensitized individuals, symptoms
appeai 12-36 h aftei diug is ieadministeied.
Anu|gesIcs/AntI-1n]|ummutvry Drugs Occuis
aftei administiation of diug by 20-30 min (up
to 4 h).
Fror 0ru xposure RudIvgruphIc Cvntrust
MedIu 25-35% piobability of iepeat ieaction
in individuals with histoiy of piioi ieaction to
contiast media.
0uratoo oI Iesoos Houis.
Sko Symptoms Piuiitus, buining of palms/
soles, auditoiy canal. With aiiway edema, dif-
ficulty bieathing.
Coosttutooa| Symptoms IgE-mediated: flush-
ing, sudden fatigue, yawning, headache, weak-
ness, dizziness; numbness of tongue, sneezing,
bionchospasm, substeinal piessuie, palpita-
tions; nausea, vomiting, ciampy abdominal
pain, diaiihea.
Systems kevew Aithialgia.
Sko Iesoos Uiticaiia and angioedema aie
desciibed in Section 14. Urtara: Laige wheals
(Fig. 22-6) that appeai and iesolve within
a few houis, spontaneously oi with theiapy.
ngoeJema: Extensive tissue swelling with in-
volvement of deep deimal and subcutaneous
tissues. Often pionounced on face with skin-
coloied enlaigement of poition of face (eye-
lids, lips) (Fig. 22-7) oi mucous membianes
(tongue, Fig. 22-7B).
Ceoera| Fodos 1gE-MedIuted ReuctIvns
Hypotension. Bionchospasm, laiyngeal edema.
0ermatopatho|oy As in uiticaiia.
Comp|emeot Ieve|s Decieased in seium sick-
ness.
|trasoooraphy Foi eaily diagnosis of bowel
involvement; piesence of abdominal pain may
indicate edema of the bowel.
0IACN0SIS
Clinical diagnosis. D[[erena| Jagnoss is of
acute edematous ied piuiitic plaque(s): Alleigic
contact deimatitis (poison ivy, poison oak dei-
matitis), cellulitis, insect bite(s).
C0kS AN0 Fk0CN0SIS
Diug-induced uiticaiia/angioedema usually ie-
solves within houis to days to weeks aftei the
causative diug is withdiawn.
MANACMNI
The offending diug should be identified and
withdiawn as soon as possible.
Freveotoo PrevIvus|y SensItIzed 1ndIvIduu|s
The patient should caiiy infoimation listing
diug sensitivities (wallet caid, biacelet).
RudIvgruphIc Cvntrust MedIu Avoid use of
contiast media known to have caused piioi
ieaction. If not possible, pietieat patient with
antihistamine and piednisone (1 mg/kg) 30-60
min befoie contiast media exposuie.
Ireatmeot oI Acute Severe rtcara[Aoaphy|axs
EpInephrIne 0.3-0.5 mL of a 1:1000 dilution
subcutaneously, iepeated in 15-20 min. Main-
tain aiiway. Intiavenous access.
Aothstamoes H
1
blockeis oi H
2
blockeis oi
combination.
Systemc C|ucocortcods 1ntruvenvus Hy-
diocoitisone oi methylpiednisolone foi seveie
symptoms.
Oru| Piednisone, 70 mg, tapeiing by 10 oi
5 mg daily ovei 1-2 weeks, is usually adequate.
FICk 22-6 0ru-oduced urtcara: peoc||o |+|e, u|||c+||+| W|e+| ou ||e +|doreu, ||||, +ud
o||e| +|e+ uc| + ||e |+c| +ud ||e |+ce.
FICk 22-T 0ru-oduced aooedema: peoc||o 4. Au|oeder+ |+ |ed |o c|ou|e o| |||| e,e. B.
'u|||uu+| +u|oeder+ |u +uo||e| p+||eu| |u|e||e|ed W||| ||e+|||u, |+|||u, +ud e+||u +ud c+ued |e+| couce|u.
4 B
FAIh0CNSIS
Unknown.
0ru hstory Patients fiequently give a histoiy
of identical lesion(s) occuiiing at the identical
location. FDEs may be associated with the fol-
lowing: (1) a headache foi which the patient
takes a baibituiate containing analgesic, (2)
constipation foi which the patient takes a phe-
nolphthalein-containing laxative, oi (3) a cold
foi which the patient takes an ovei-the-countei
medication containing a yellow dye. The of-
fending diug" in food dye-induced FDE may
be difficult to identify, e.g., yellow dye in Gal-
liano liqueui oi phenolphthalein in maiaschino
cheiiies; quinine in tonic watei.
S|n symoms : Usually asymptomatic. May
be piuiitic, painful, oi buining. Painful when
eioded. Tme o onse o[ |eson(s) : Occui fiom
A ||\ed d|u e|up||ou (||E) | +u +d.e|e cu|+ue
ou |e+c||ou |o +u |ue|ed d|u, c|+|+c|e||/ed |,
||e |o|r+||ou o| + o|||+|, (|u| +| ||re ru|||p|e)
e|,||er+|ou p+|c| o| p|+que.
|| ||e p+||eu| | |ec|+||eued W||| ||e o||eud|u
d|u, ||e ||E occu| |epe+|ed|, +| ||e |deu||c+|
||u ||e (|.e., ||\ed) W||||u |ou| o| |ue||ou.
|e|ou c+u |ecore |u||ou +ud e|o|.e.
\o| corrou|, |rp||c+|ed +eu| +|e |||ed |u
I+||e 225.
FIX0 0kC kFII0N |C|9 . 995.2
|C|0 . I33.1
IA8I 22-5 Nost Common|y |mp|icated Aents in |i\ed 0ru Eruptions
Au||r|c|o||+| +eu| |,c|o+c||.e +eu|
Ie||+c,c||ue (|e||+c,c||ue, r|uoc,c||ue) B+||||u|+|e, |uc|ud|u ||o||u+|, 0u++|ude, |o||deu
'u||ou+r|de, |uc|ud|u 0|+| cou||+cep||.e
'uou+|o||+||e d|u, 0u|u|ue (|uc|ud|u qu|u|ue |u |ou|c W+|e|), qu|u|d|ue
c|o|e+c||ou W||| +u||d|+|e||c +ud d|u|e||c ||euo|p|||+|e|u
u||+ d|u r+, occu| |ood co|o||u (,e||oW). |u |ood o| red|c+||ou
\e||ou|d+/o|e
|,|+||u
Au|||u||+rr+|o|, +eu|
'+||c,|+|e
|'A||
||eu,||u|+/oue
||eu+ce||u
30 min to 8 h aftei ingestion of diug in pievi-
ously sensitized individual. Duraon o[ |eson(s):
Lesions peisist if diug is continued. Resolve days
to few weeks aftei diug is discontinued.
Sko Iesoos The chaiacteiistic eaily lesion is a
shaiply demaicated macule (Fig. 22-8), iound
oi oval in shape, occuiiing within houis aftei
ingestion of the offending diug. Initially eiy-
thema, then dusky ied to violaceous (Figs. 22-8
and 22-9). Most commonly, lesions aie solitaiy
(Fig. 22-9) and can spiead to become quite
laige (Fig. 22-9), but they may be multiple
(Fig. 22-9B) with iandom distiibution; numei-
ous lesions may simulate TEN. Lesions become
edematous, thus foiming a plaque, which may
evolve to become a bulla (Fig. 22-8B) and then
an eiosion. Eioded lesions, especially on genitals
oi oial mucosa, aie quite painful. Aftei healing,
daik biown with violet hue postinflammatoiy
hypeipigmentation. Genital skin (Fig. 35-19)
is fiequently involved site, but any site may be
involved; peiioial, peiioibital (Fig. 22-8). Oc-
cui in conjunctivae, oiophaiynx.
SCII0N 22 A|\Ek'E CuIA|E0u' |kuC kEACI|0|' 56T
FICk 22-8 Fxed dru eruptoo 4. Ie||+c,c||ue. IWo We||de||ued pe||o||||+| p|+que W||| eder+. I||
W+ ||e ecoud uc| ep|ode |o||oW|u |ue||ou o| + |e||+c,c||ue. |o o||e| |e|ou We|e p|eeu|. B. I,|euo|. A
|+|e o.+| .|o|+ceou |e|ou W||| ||||e||u |u ||e ceu|e|. E|o|.e rou|| |e|ou We|e +|o p|eeu|.
4 B
FICk 22-9 Fxed dru eruptoo 4. ||euo|p|||+|e|u. A |+|e +|e+ o| du|,, .|o|+ceou e|,||er+ co.
e||u ||e eu|||e |o|u +ud up|+pu||c |e|ou +ud e\|eud|u |o ||e uppe| ||||. || |o||oWed ||e |ue||ou o| +
p|euo|p|||+|e|ucou|+|u|u |+\+||.e. B. |o\,c,c||ue. \u|||p|e |e|ou. '|r||+| .|o|+ceou p|+que We|e +|o ou ||e
+u|e||o| +ud po|e||o| ||uu|.
4 B
0ermatopatho|oy Similai to findings in eiy-
thema multifoime and/oi TEN.
Fatch Iest Suspected diug can be placed as
a patch test at a pieviously involved site; an
inflammatoiy iesponse occuis in only 30% of
cases.
Made on clinical giounds. Readministiation
of the diug confiims diagnosis but should be
avoided.
Solitaiy genital eiosion to be diffeientiated
fiom iecuiient heipetic lesion; multiple eio-
sions fiom SJS, TEN; oial eiosion(s) fiom
aphthous stomatitis, piimaiy heipetic gingivo-
stomatitis, eiythema multifoime.
kace Reactions to antiepileptic diugs may be
highei in black individuals.
to|oy Most commonly: antiepileptic diugs
(phenytoin, caibamazepine, phenobaibital;
cioss-sensitivity among the thiee diugs is com-
mon) and sulfonamides (antimiciobial agents,
dapsone, sulfasalazine). Less commonly: al-
lopuiinol, gold salts, soibinil, minocycline, zal-
citabine, calcium-channel blockeis, ianitidine,
thalidomide, mexiletine.
FAIh0CNSIS
Some patients have a genetically deteimined
inability to detoxify the toxic aiene oxide
||u |,pe|eu|||.||, ,ud|ore | +u |d|o,uc|+||c
+d.e|e d|u |e+c||ou ||+| |e|u +cu|e|, |u ||e
|||| 2 rou|| +||e| |u|||+||ou o| d|u +ud | c|+|
+c|e||/ed |, |e.e|, r+|+|e, +ud |+c|+| eder+ o|
+u e\|o||+||.e de|r+||||.
'||u |e|ou +|e + r+cu|op+pu|+| e\+u||er.
|,rp|+deuop+||,, |er+|o|o|c +|uo|r+||||e
(eo|uop||||+, +|,p|c+| |,rp|oc,|e), +ud o|+u
|u.o|.ereu| (|ep+||||, c+|d|||, |u|e|||||+| uep|||
||, o| |u|e|||||+| pueurou|||).
I|e ro||+|||, |+|e | 0 || uu|ecou|/ed +ud
uu||e+|ed.
|e|ou+| ||op, pec|reu |oW + |,rp|oc,||c
|u|||||+|e, +| ||re r|r|c||u + cu|+ueou |,r
p|or+.
',,. ||u |+| W||| eo|uop||||+ +ud ,|er|c
,rp|or (|kE'').
|C|9 . 995.2
|C|.0 . |33.1
0kC hFkSNSIIIvII SN0k0M
C0kS AN0 Fk0CN0SIS
FDE iesolves within a few weeks of withdiawing
the diug. Recuis within houis aftei ingestion of
a single dose of the diug.
MANACMNI
Identify and withhold the offending diug. Non-
eioded lesions can be tieated with a potent topi-
cal glucocoiticoid ointment. Eioded cutaneous
lesions can be tieated with an antimiciobial
ointment and a diessing until the site is ieepi-
thelialized. Foi widespiead, geneialized, and
highly painful mucosal lesions, oial piednisone
1 mg/kg body weight tapeied ovei a couise of
2 weeks.
metabolic pioducts of anticonvulsant agents.
Slow N-acetylation of sulfonamide and incieased
susceptibility of leukocytes to toxic hydioxyl-
amine metabolites aie associated with highei
iisk of hypeisensitivity syndiome.
0oset 2-6 weeks aftei diug is initially used,
and latei than most othei seiious skin ieac-
tions.
Frodrome Fevei. iash.
Systems kevew Fevei, malaise.
Sko Iesoos Ear|y : moibillifoim eiuption
(Fig. 22-10) on face, uppei tiunk, uppei extiem-
ities; cannot be distinguished fiom exanthema-
tous diug eiuption. May piogiess to geneialized
exfoliative deimatitis/eiythiodeima, especially
if diug is not discontinued. Eiuption becomes
infiltiated with edematous folliculai accentua-
tion. Facial edema (especially peiioibitally) is
chaiacteiistic. Deimal edema may iesult in
blistei foimation. Steiile folliculocentiic as well
as nonfolliculai pustules may occui. Eiuption
may become puipuiic on legs. Scaling and/oi
desquamation may occui with healing.
DIstrIhutIvn Symmetiic. Almost always on
tiunk and extiemities. Lesions may become
confluent and geneialized.
Mucous Membraoes Cheilitis, eiosions, eiy-
thematous phaiynx, enlaiged tonsils.
Ceoera| xamoatoo Elevated tempeiatuie
(diug fevei).
Lymph Nvdes Lymphadenopathy fiequent
tendei; usually due to benign lymphoid hypei-
plasia.
Other Involvement of livei, heait, lungs,
joints, muscles, thyioid, biain also occuis.
hemoram aod Chemstres Eosinophilia
(30% of cases). Leukocytosis. Mononucleosis-
like atypical lymphocytes. Signs of hepatitis and
nephiitis.
hsto|oy S|In Lymphocytic infiltiate, dense
and diffuse oi supeificial and peiivasculai.
Eosinophils oi deimal edema. In some cases,
bandlike infiltiate of atypical lymphocytes with
epideimotiopism, simulating cutaneous T cell
lymphoma.
Lymph Nvdes Benign lymphoid hypeiplasia.
Uncommonly atypical lymphoid hypeiplasia,
pseudolymphoma.
LIver Eosinophilic infiltiate oi gianulomas.
KIdney Inteistitial nephiitis.
0IACN0SIS
Froposed 0aoostc Crtera (1) Cutane-
ous diug eiuption; (2) hematologic abnoi-
malities (eosinophilia 1500/L oi atypical
lymphocytes); (3) systemic involvement aden-
opathies 2 cm in diametei oi hepatitis (SGOT
2 N ) oi inteistitial nephiitis oi inteistitial
pneumonitis oi caiditis]. Diagnosis is con-
fiimed if thiee ciiteiia aie piesent.
ar|y That of moibillifoim eiuptions. Can
mimic eaily measles oi iubella.
Iater Seium sickness, diug-induced
vasculitis, Henoch-Schnlein puipuia,
ciyoglobulin-associated vasculitis, vasculitis
associated with infection, and collagen vasculai
diseases.
kash F|us Iymphadeoopathy Rubella, piimaiy
EBV oi CMV mononucleosis syndiome.
FICk 22-10 0ru hyperseostvty syodrome: pheoytoo ',rre|||c, ||||| |ed, e\+u||er+|ou
e|up||ou, cou||ueu| |u ore ||e, ||e p+||eu| |+d +oc|+|ed |,rp|+deuop+||,.
C0kS AN0 Fk0CN0SIS
Rash and hepatitis may peisist foi weeks aftei
diug is discontinued. In patients tieated with
systemic glucocoiticoids, iash and hepatitis
may iecui as glucocoiticoids aie tapeied. Lym-
phadenopathy usually iesolves when diug is
withdiawn; howevei, iaie piogiession to lym-
phoma has been iepoited. Raiely, patients die
fiom systemic hypeisensitivity such as with
eosinophilic myocaiditis. Clinical findings ie-
cui if diug is given again.
MANACMNI
Identify and discontinue the offending diug.
Symptomatc Ireatmeot Oial antihistamine to
alleviate piuiitus.
C|ucocortco ds TvpIcu| High-potency
topical glucocoiticoids applied twice a day
aie usually helpful in ielieving cutaneous
symptoms of piuiitus but do not altei systemic
hypeisensitivity.
SystemIc Piednisone (0.5 mg/kg pei day) usu-
ally iesults in iapid impiovement of symptoms
and laboiatoiy paiameteis.
Future 0ru Iherapy Cioss-sensitivity be-
tween vaiious aiomatic antiepileptic diugs oc-
cuis, making it difficult to select alteinative
anticonvulsant theiapy.
Freveotoo The individual must be awaie of
his oi hei specific diug hypeisensitivity and
that othei diugs of the same class can cioss-ie-
act. These diugs must nevei be ieadministeied.
Patient should weai a medical aleit biacelet.
0kCS CASINC hFkFICMNIAII0N
The following diugs aie capable of inducing
hypeipigmentation of skin and/oi mucosa:
Antiaiihythmic: amiodaione
Antimalaiial: chloioquine, hydioxychloio-
quine, quinaciine, quinine
Antimiciobial: minocycline, clofazimine,
zidovudine
Antiseizuie: hydantoins
Cytostatic: bleomycin, cyclophospha-
mide, doxoiubicin, daunoiubicin, busul-
fan, 5-fluoiouiacil, dactinomycin
Heavy metals: silvei, gold, meicuiy
Hoimones: ACTH estiogen/piogesteione
Psychiatiic: chloipiomazine
||u|uduced +||e|+||ou |u p|reu|+||ou +|e
|e|+||.e|, corrou.
I|e, |eu|| ||or ||e depo|||ou o| + .+||e|, o| eu
doeuou +ud e\oeuou p|reu| |u ||e ||u.
C+u |e o| |u|||c+u| core||c couce|u |o ||e
p+||eu|.
0kC-IN0C0 FICMNIAII0N |C|9 . 995.2

|C|0 . I33.1
Sko Fodos
Amodarooe > 75% of patients aftei 40-g cu-
mulative dose aftei >4 months of theiapy. Moie
common in skin phototypes I and II. Low-giade
oi minimal photosensitivity; phototoxic eiy-
thema limited to the light-exposed aieas in a
small piopoition (8%) of patients. Dusky-ied
eiythema and, latei, blue-giay deimal melano-
sis (ceiulodeima) (Fig. 22-11) in exposed aieas
(face and hands). Lipofuscin-type pigment de-
posited in maciophages and endothelial cells.
Other Adverse E]]ects v] AmIvdurvne
Pulmonaiy fibiosis, pneumonitis, hepatotox-
icity, thyioid dysfunction, neuiopathy, and
myopathy.
SCII0N 22 A|\Ek'E CuIA|E0u' |kuC kEACI|0|' 5T1
FICk 22-11 0ru-oduced pmeotatoo: amodarooe A |||||u |+|e|+, p|reu|+||ou |u + p|o|o
d|||||u||ou o| ||e |+ce. I|e ||ue co|o| (ce|u|ode|r+) | due |o ||e depo|||ou o| re|+u|u +ud ||po|uc|u cou|+|ued
|u r+c|op|+e +ud eudo||e||+| ce|| |u ||e de|r|. I|e ||oWu co|o| | due |o re|+u|u. I|e p|reu|+||ou |
|e.e||||e, |u| || r+, |+|e up |o + ,e+| o| ro|e |o corp|e|e |eo|u||ou. |u ||| p+||eu| || |oo| o rou|| |o| ||e
ce|u|ode|r+ |o d|+ppe+|.
Cvurse The low-giade photosensitivity disap-
peais 12-24 months aftei diug is discontin-
ued; the long peiiod iesults fiom the giadual
elimination of the photoactive diug fiom the
lysosomal membianes. The pigmentation also
disappeais aftei 1-3 yeais if diug is discontin-
ued and sun is avoided.
Moocyc|oe Onset delayed, usually aftei total
dose of >50 g, but may occui aftei a small dose.
Not melanin but an iion-containing biown
pigment, located in the deimal maciophages;
stippled oi diffuse. Blue-giay oi slate-giay pig-
mentation (Fig. 22-12). Distiibuted on extensoi
legs, ankles, doisa of feet, face, especially aiound
eyes; sites of tiauma oi inflammation such as
acne scais, contusions, abiasions; haid palate,
teeth; nails.
1nternu| SItes Bones, caitilage, thyioid (black
thyioid").
Cvurse Discoloiation giadually disappeais ovei
a peiiod of months aftei diug is discontinued.
C|oIatmoe Oiange, ieddish biown (iange,
pink to black) discoloiation, ill-defined on
light-exposed aieas; conjunctivae; accompanied
by ied sweat, uiine, feces. Subcutaneous fat is
oiange.
Idovudoe Biown macules on lips oi oial
mucosa; longitudinal biown bands in nails.
Aotma|ara|s (Ch|oroquoe, hydroxych|oroquoe,
uoacroe) Occuis in 25% of individuals who
take the diug foi >4 months. Biownish, giay-
biown, and/oi blue-black discoloiation due to
melanin, hemosideiin. With quinaciine: yel-
low, yellow-gieen due to quinaciine-containing
complexes. Ovei shins; face, nape of neck; haid
palate (shaip line of demaication at soft palate);
undei fingei- and toenails (see Fig. 33-36); may
also occui in coinea and ietina; quinaciine: skin
and scleiae (iesembling icteius); yellow-gieen
fluoiescence of nail bed with Wood lamp. Dis-
coloiation disappeais within a few months aftei
diug is discontinued; quinaciine dyschiomia
can fade aftei 2-6 months even though diug is
continued.
Fheoytoo High dose ovei a long peiiod of
time (> 1 yeai). Dsto|oraon is spotty, iesem-
bling melasma, in light-exposed aieas and is
due to melanin.
8|eomyco Mechanism unknown. Tan to
biown to black and due to inciease in epidei-
mal melanin at sites of minoi inflammation,
i.e., paiallel lineai stieaks at sites of deimato-
giaphism induced by excoiiation (flagellate"
pigmentation), most commonly on the back,
elbows, small joints, nails.
Cyc|ophosphamde Biown. Diffuse oi disciete
macules on elbows; palms with Addisonian-like
pigmentation (see Fig. 15-10) and macules.
8usu|Iao Occuis in 5% of tieated patients.
Addisonian-like pigmentation. Face, axillae,
chest, abdomen, oial mucous membianes.
ACIh Addisonian pigmentation of skin and
oial mucosa. Fiist 13 amino acids of ACTH
aie identical to -melanocyte-stimulating hoi-
mone (MSH) (see Fig. 15-10, B).
stroeos[Froesterooe Caused by endog-
enous and exogenous estiogen combined with
piogesteione, i.e., duiing piegnancy oi with
oial contiaceptive theiapy. Sunlight causes
maiked daikening of pigmentation. Tan/biown
(melasma oi chloasma) (see Fig. 13-8).
Ch|orpromatoe aod 0ther Fheooth-
atoes Occuis aftei long-teim (> 6 months),
high-dose (> 500 mg/d) theiapy. Phototoxic
ieaction. Slate-giay, blue-giay, oi biownish in
aieas exposed to light, i.e., chin and cheeks.
Aftei discontinuation of diug, discoloiation
usually fades slowly.
S|ver (Aryra or Aryross) Sourte : Silvei
nitiate nose diops; silvei sulfadiazine applied
as an ointment. Silvei sulfide (silvei nitiate
conveited into silvei sulfide by light, as in
photogiaphic film). Blue-giay discoloiation.
Piimaiily aieas exposed to light, i.e., face, doisa
of hands, nails, conjunctiva; also diffuse.
Co|d (Chrysass) Sourte : Oiganic colloidal
gold piepaiations used in theiapy of iheuma-
toid aithiitis. 5-25% of all tieated patients.
Dose-dependent. In high-dose theiapy, appeais
in a shoit time; with lowei dose, occuis aftei
months. Blue-giay to puiple discoloiation. In
light-exposed aieas; scleiae. Peisists long aftei
diug is discontinued.
Iroo Sourte : IM iion injections; multiple
blood tiansfusions. Biown oi blue-giay discol-
oiation. Geneialized; also, local deposits at site
of injection.
Caroteoe Ingestion of laige quantities of
-caiotene-containing vegetables; -caiotene
tablets. Yellow-oiange discoloiation. Most ap-
paient on palms and soles.
FICk 22-12 0ru-oduced pmeotatoo: moocyc|oe '||pp|ed, ||ue|+, r+cu|+| p|reu|+||ou ou
||e |oWe| |e. I|e p+||eu| |+d |+|eu r|uoc,c||ue |o| ,e+| |o| |o+ce+. I|e p|reu|+||ou W+ ruc| ro|e p|o
uouuced ou ||e |e|| |e, +oc|+|ed W||| .+||coe .e|u, c||ou|c .euou |uu|||c|euc,, +ud c||ou|c eder+. A re|ou
|/ed |uu|u+| |e|u|+ W+ +|o p|eeu| W||| |||||u p|reu|+||ou o| ||e eu|+|ed c|o|ur.
IA8I 22-6 0rus Causin Pseudoporphyria
|+p|o\eu ||||uu|+|
|+|ure|oue Ce|eco\||
0\+p|o/|u Ie||+c,c||ue
Ke|op|o|eu |+||d|\|c +c|d
\e|eu+r|c +c|d Ar|od+|oue
I|+p|o|eu|c +c|d |u|oer|de
|eudopo|p|,||+ | + coud|||ou ||+| c||u|c+||,
p|eeu| W||| cu|+ueou r+u||e|+||ou o| po|
p|,||+ cu|+ue+ |+|d+ (|CI) (ee 'ec||ou 0)
W|||ou| ||e c|+|+c|e||||c +|uo|r+| po|p|,||u
e\c|e||ou.
|| | + |u||ou d|u|uduced p|o|oeu|||.||, |e+c
||ou.
|e.e|op ou ||e do|+ o| |+ud +ud |ee| W|||
c|+|+c|e||||c |eue |u||+e ||+| |up|u|e +ud |e+.e
e|o|ou (||. 22!) +ud |e+| W||| c+| +ud r|||+
|o|r+||ou.
|eudo|CI | +oc|+|ed W||| |ue||ou o| d|u
uc| + |u|oer|de +ud u+||d|\|c +c|d (I+||e
22o).
|| | c|+|+c|e||/ed |, u|ep|de|r+| ||||e||u W|||
|||||e o| uo de|r+| |u||+rr+||ou +ud, |u cou||+|
|o ||ue |CI, |||||e o| uo depo|||ou o| |rruuo|e
+c|+u| +|ouud uppe| de|r+| ||ood .ee| +ud
c+p|||+|, W+||.
A |u||ou de|r+|o| ||+| | ro|p|o|o|c+||, +ud
|||o|o|c+||, |ud|||uu||+||e ||or peudopo|
p|,||+ +|o occu| |u p+||eu| W||| c||ou|c |eu+|
|+||u|e |ece|.|u r+|u|eu+uce |erod|+|,| (ee
'ec||ou 1).
FS00F0kFhkIA
FICk 22-13 Fseudoporphyra: ooosteroda| aot-oI|ammatory aeots |u ||| 20,e+|o|d r+|e
||||e| +ppe+|ed ou ||e do|+ o| |o|| |+ud ||+| |ed |o e|o|ou, c|u||u, +ud We|e c||u|c+||, |ud|||uu||+||e
||or po|p|,||+ cu|+ue+ |+|d+. noWe.e|, ||e|e W+ uo u||u+|, ||uo|eceuce, +ud po|p|,||u |ud|e We|e ue+||.e.
I|e p+||eu| |+d |+|eu +u |'A|| |o| +||||||| +ud |+d |rp+||ed ||due, |uuc||ou.
||u c+u c+ue cu|+ueou uec|o| W|eu |.eu
o|+||, o| +| ||e o| |ujec||ou.
|c|c!:-! :|c- -: | + |+|e
|e+c||ou W||| oue| |e|Weeu ||e ||||d +ud ||||| d+,
o| +u||co+u|+||ou ||e|+p, W||| ||e W+||+||u de||.+
||.e +ud |ud+ud|oue corpouud, r+u||e|ed |,
|+|p|, der+|c+|ed, pu|pu||c cu|+ueou |u|+|c||ou.
|| |c:| . |||e| |u|||+| do|u, o|e||,, |er+|e
e\, |ud|.|du+| W||| |e|ed||+|, de||c|euc, o| p|o
|e|u C, p|o|e|u ' o| +u|||||or||u ||| de||c|euc,. |d|
o,uc|+||c |e+c||ou. |u |ud|.|du+| W||| |e|ed||+|,
de||c|euc, o| p|o|e|u C, + u+|u|+| +u||co+u|+u|
p|o|e|u, W+||+||u |e+||, dep|ee p|o|e|u C |e.
e| |e|o|e dec|e+|u o||e| .||+r|u K-depeudeu|
co+u|+||ou |+c|o|, |uduc|u + ||+u|eu| |,pe|co
+u|+||e |+|e +ud |||or|u |o|r+||ou.
|e|ou .+|, W||| e.e|||, o| |e+c||ou. pe|ec||+e
|o ecc|,roe |o |eude| |ero|||+|c |u|+|c| |o
e\|eu|.e uec|o|. We||der+|c+|ed, deep pu|p|e
|o ||+c| (||. 22+). |eep ||ue |ou||u +ud
u|ce|+||ou || |e|ou +|e uo| de|||ded +ud |+||ed.
0||eu |u|e, r+, p|eeu| + |Wo |e|ou. |||
| . +|e+ o| +|uud+u| u|cu|+ueou |+|. ||e+|
(||. 22+), |u||oc|, +|doreu, ||||, c+|.e,
+c|+| +|e+ +|e p+|ed.
Cc|c| |!- . uu+||, W||||u uo|r+| ||r||.
|||--|c| !c . |u|pu|+ |u|r|u+u (d|
er|u+|ed |u||+.+cu|+| co+u|+||ou), |er+|or+/
ecc|,ro| |u o.e||, +u||co+u|+|ed p+||eu|,
uec|o||/|u o|| ||ue |u|ec||ou, .+cu||||, |+|e
uec|o| +||e| .+op|e|u ||e+|reu|, ||oWu |ec
|ue p|de| |||e. |epeud|u ou e.e|||, o| |e+c
||ou, |e|ou r+, u||de, |e+| |, |+uu|+||ou, o|
|equ||e u||c+| |u|e|.eu||ou. || +|e+ o| uec|o|
| |+|e |u +u e|de||,, de|||||+|ed p+||eu|, r+, |e
|||e|||e+|eu|u. || W+||+||u | |u+d.e||eu||, |e+d
r|u||e|ed, |e+c||ou |ecu|.
|-c c+u c+ue cu|+ueou uec|o|, uu+||, +|
||e ||e o| u|cu|+ueou |ujec||ou (||. 225).
||-|- c+u c+ue uec|o| +ud u|ce|+||ou +|
|ujec||ou ||e, o||eu |u ||e |oWe| +|dor|u+| p+u
u|cu|u o| |||| (||. 22o).
|| c+u c+ue uec|o|. E|o|+r|uecou|+|u
|u red|c+||ou |e+d |o +c|+| +u|eue, e|o|
+r|uecou|+|u|u uppo||o||e +||e| p|o|oued
ue c+ue e\||ere|, p+|u|u| +u+| +ud pe||+u+|
||+c| ec|+| ||+|, +||e| |+.|u |eeu |ed, |e+.e
deep p+|u|u| u|ce| (||. 221).
|||c :| -!:c-|c . |eep uec|o|
de.e|op|u +| ||e ||e o| |u||+rucu|+| |ujec||ou
o| o||, d|u |u+d.e||eu||, |ujec|ed |u|o +u +||e|,
(||. 223).
|ec|o| +|o de.e|op |u o||uuded o| deep|,
ed+|ed p+||eu| +| p|eu|e ||e (||. 229).
AC0k-kIAI0 NCk0SIS |C|9 . 995.2
|C|0 . I33.1
FICk 22-14 AC0k-re|ated cutaoeous oecross: warIaro B||+|e|+| +|e+ o| cu|+ueou |u|+|c||ou W|||
pu|p|e|o||+c| co|o|+||ou o| ||e ||e+| u||ouuded |, +u +|e+ o| e|,||er+ occu||ed ou ||e ||||| d+, o| W+||+||u
||e|+p,.
FICk 22-15 AC0k-re|ated cutaoeous oecross: heparo IWo |e|ou o| |||eu|+| d+|||ed e|,||er+
W||| ceu||+| |ero|||+|c uec|o| ou ||e +|doreu occu|||u po|ope|+||.e|, |u + |er+|e |ujec|ed W||| |ep+||u.
SCII0N 22 A|\Ek'E CuIA|E0u' |kuC kEACI|0|' 5TT
FICk 22-16 AC0k-re|ated cutaoeous oecross: oterIeroo- Au u|ce| ou ||e |||| +| ||e ||e o| |u|e|
|e|ou |ujec||ou.
FICk 22-1T AC0k-re|ated cutaoeous oecross: erotamoe I|| o0,e+|o|d r+|e |+d ued e|o|
cou|+|u|u uppo||o||e |o| p+|u |e||e| o.e| r+u, rou||. |+|u|u| ||+c| uec|o| |o||oWed |, u|ce|+||ou de.e|oped
ou ||e +uu +ud pe||+u+||, +ud e\|euded |u|o ||e |ec|ur.
FICk 22-18 AC0k-re|ated oecrossa Io||owo otramuscu|ar ojectoo Er|o||+ +cu|| red|c+ reu
|o+. I|e d|u (+u o||, p|ep+|+||ou o| |e|o|e|oue) |+d |eeu |u+d.e||eu||, +dr|u||e|ed |u||++||e||+||,.
FICk 22-19 AC0k-re|ated oecross wth hemorrhac b|stero aIter ao overdose oI barbturates
I|| p+||eu| |+d +||erp|ed u|c|de.
C|ero||e|+p, r+, |uduce |oc+| +ud ,|er|c
||u |o\|c||, W||| + W|de |+ue o| cu|+ueou r+u|
|e|+||ou ||or |eu|u |o |||e |||e+|eu|u.
I|e AC|k c+u |e |e|+|ed |o o.e|doe, p|+|r+
co|o|c |de e||ec|, curu|+||.e |o\|c||,, de|+,ed
|o\|c||,, o| d|ud|u |u|e|+c||ou.
C||u|c+| r+u||e|+||ou |+ue ||or +|opec|+ (ee
'ec||ou !2) +ud u+|| c|+ue (ee 'ec||ou !!, ||.
!!2!, !!!5) |o ruco||| +ud +c|+| e|,||er+,
o||eu W||| euo|, +|uo|r+||||e. p+|rop|+u|+|
d,e||e|+ (c+pec||+||ue, c,|+||||ue, do\o|u||c|u,
||uo|ou|+c||).
C|ero||e|+peu||c +eu| +|e +|o |epou|
||e |o| |u||+rr+||ou +ud u|ce|+||ou +| ||e o|
e\||+.++||ou o| |u||+.euou red|c+||ou, uc|
+ do\o|u||c|u o| |+\o|, W||c| c+u |e |o||oWed |,
||u uec|o| W||| u|ce|+||ou (||. 2220 1).
0||e| |e+c||ou +|e |+d|+||ou |ec+|| o| eu|+uce
reu| (+ W||| re||o||e\+|e), e|o|ou o| u|ce|+||ou
o| po||+| due |o +u o.e|doe o| re||o||e\+|e,
|u||+rr+||ou +ud |ou||u o| +c||u|c |e|+|o|
due |o 5||uo|ou|+c|| o| ||ud+|+||ue, o| e|o|ou
due |o c|p|+||u p|u 5||uo|ou|+c|| (||. 2220 3).
I+||e 221 ||| ueWe| c|ero||e|+peu||c |uc|ud
|u '||o|o|c+| +ud ||e|| AC|k.
AC0k kIAI0 I0 ChM0IhkAF |C|9 . 995.2
|C|0 . I33.1
FICk 22-20 AC0k-re|ated ce||u|ts aod erosoos 4. C-|||| :c-! |, |c|. I|| e\||ere|, p+|u|u|
ce||u|||| +ppe+|ed +||e| + p+|+.euou |+\o| |u|u|ou. B. | -|| | :|c| c! ||c:| (||)
I|| p+||eu| |+d |ece|.ed c|ero||e|+p, W||| c|p|+||u +ud 5|u. |+|u|u| e|o|.e |e|ou +ppe+|ed ou ||e c|o|ur
+ud ||e|e W+ +|o e|o|.e ruco|||.
4 B
IA8I 22-T Newer Chemotherapeutic Aents and their AC0R
C|ass Aeots AC0k
1
'p|ud|e |u|||||o| I+\+ue. doce|+\e|, n+ud|oo| ||u |e+c||ou
2
, cor||ued W||| euo|,
p+c|||+\e| +|uo|r+||||e. e|,|||od,e||e|+, |+d|+||ou
|ec+|| u|||c+||+, e\+u||er, ruco|||, +|opec|+,
u+|| c|+ue (ee 'ec||ou !!), c|e|ode|r+
|||e c|+ue ou |oWe| e\||er|||e, u|+cu|e
cu|+ueou |upu e|,||er+|ou
\|uc+ +||+|o|d. .|uc||||ue, |||e||||, +|opec|+, +c|+| e|,||er+, e\||+.++||ou
.|u||+||ue, .|uo|e|||ue |e+c||ou (|uc|ud|u uec|o|)
Au||re|+|o|||e ||ud+|+||ue \+cu|+|, p+pu|+| e\+u||er, ruco|||, +c|+|
e|,||er+, p+|+ueop|+||c perp||u
C|+d||||ue E\+u||er, IE| ( ! )
C+pec||+||ue n+ud|oo| ||u |e+c||ou
2
+c|+| |,pe|p|reu|+||ou,
p+|rop|+u|+| |e|+|ode|r+, p,oeu|c |+uu|or+,
|u||+rr+||ou o| +c||u|c |e|+|oe
Ie+|u| n+ud|oo| ||u |e+c||ou
2
+c|+| |,pe|p|reu|+||ou,
p||,||+| ||c|euo|de e| .+||o|||o|r| +cu|+
Cerc||+||ue \uco|||, +|opec|+, r+cu|op+pu|+| e\+u||er,
|+d|+||ou |ec+||, ||ue+| |A, |u||ou de|r+|o|,
peudoc|e|ode|r+, ||pode|r+|oc|e|o|,
e|,|pe|+|||e p|+que, peudo|,rp|or+,
|,rp|or+|o|d p+pu|o| (!)
|ere||e\ed E\+u||er+, |+d|+||ou |ec+||, u|||c+||+| .+cu||||
Ceuo|o\|c +eu| C+||op|+||u A|opec|+, |,pe|eu|||.||, |e+c||ou (e|,||er+,
|+c|+| We|||u, d,pue+, |+c|,c+|d|+, W|ee/|u),
p+|rop|+u|+| e|,||er+, |+c|+| ||u||u
0\+||p|+||u n,pe|eu|||.||, |e+c||ou (ee +|o.e), |||||+u|
e\||+.++||ou |e+c||ou, |+d|+||ou |ec+||
||poor+| Ac|+| e|,||er+, p+|rop|+u|+| e|,|||od,e||e|+
do\o|u||c|u ueu||op||||c ecc||ue ||d|+deu|||,
|,pe|p|reu|+||ou (||ue|+,), ruco|||,
+|opec|+, e\+u||er, |+d|+||ou |ec+||, u|||+.|o|e|
|||| |ec+||
||poor+| A|opec|+, ruco|||, e\||+.++||ou |e+c||ou
d+uuo|u||c|u
|d+|u||c|u k+d|+||ou |ec+||, +|opec|+, +c|+| e|,||er+,
ruco|||, u+|| c|+ue, e\||+.++||ou
|e+c||ou
Iopo|ec+u \+cu|op+pu|+| e\+u||er, +|opec|+, ueu||op||||c
||d|+deu|||
|||uo|ec+u \uco|||, +|opec|+
'|u+| ||+uduc||ou EC|k +u|+ou||. |+pu|opu|u|+| e|up||ou
|u|||||o| e||||u||, ce|u\|r+|, |u e|o|||e|c +|e+ (||. 2251-3),
e||o||u||, p+u||urur+| e|,||er+|ou p|+que, |e|+u|ec|+|+, \e|o|,
p+|ou,c||+, |+|| +|uo|r+||||e (|||c|ore+|,,
cu|||u, ||+||||,, ee 'ec||ou !2)
C|ass Aeots AC0k
1
\u|||||u+e |u|||||o|. \+cu|op+pu|+| e\+u||er, eder+ |r+||u|| (|+ce,
|o|e+|r, +u||e), e\|o||+||.e de|r+||||,
|+||.e|u|o| |e+c||ou-|||e |e+c||ou,
e|,||er+ uodour, .+cu||||, 'l', ACE|,
|,pop|reu|+||ou, |,pe|p|reu|+||ou, d+||eu|u
o| |+||, u+|| |,pe|p|reu|+||ou, ||c|eu p|+uu|||e
e|up||ou (||u +ud o|+| ruco+), |o|||cu|+|
ruc|uo|, p||,||+| |oe+-|||e e|up||ou, 'Wee|
,ud|ore, e\+ce||+||ou o| po||+|, p+|rop|+u|+|
|,pe||e|+|o|, po|p|,||+ cu|+ue+ |+|d+, p||r+|,
cu|+ueou EB\|e|+|ed B ce|| |,rp|or+
|++||u|| +ud u||o||u|| |oc+||/ed +ud eue|+||/ed e|,||er+, r+cu|op+pu|+|
e\+u||er, ruco|||, p|u|||u, +|opec|+, \e|o|
'+cue, u|||c+||+, p+uu|cu||||
'o|+|eu|| +ud uu|||u|| k+|/dequ+r+||ou, |+ud|oo| ||u |e+c||ou
2
p+|u, +|opec|+, ruco|||, \e|o|, ||u||u eder+,
e|o|||e|c de|r+||||, ,e||oW ||u co|o|+||ou
(uu|||u||), u|uuu+| p||u|e| |ero|||+e,
p,ode|r+ +u|euour
||o|e+ore |u|||||o| Bo||e/or|| E|,||er+|ou uodu|e +ud p|+que, ro|||||||o|r
e\+u||er, u|ce|+||ou, .+cu||||
'0ukCE. Co||+|ed ||or. | n+|d+|, e| +|. l Ar Ac+d |e|r+|o| 53.5+5, 2003.
0u|, cu|+ueou +d.e|e |e+c||ou +|e |||ed |e|e.

2
n+ud|oo| ||u |e+c||ou. e|,||er+, |,pe||e|+|o||c W||| |+|o o| e|,||er+, |eude|, |oc+||/ed |o +|e+ o| p|eu|e ou ||ue| ||p, |oe, |ee|.
582
0IS0k0kS 0F
FSChIAIkIC II0I0C
S E C I | 0 N 2 5
CIASSIFICAII0N 0F 0IS0k0kS 0F
FSChIAIkIC II0I0C
Dysmoiphic syndiome
Delusions of paiasitosis
|+||eu| W||| d,ro|p||c ,ud|ore |e+|d ||e||
|r+e + d||o||ed |u ||e e,e o| ||e pu|||c, |||
|ecore +|ro| +u o|e|ou.
I|e p+||eu| W||| B|' doe uo| couu|| + p,c||+
|||| |u| + de|r+|o|o|| o| p|+||c u|eou. I|e
|,p|c+| p+||eu| W||| B|' | + |u|e, |er+|e, ,ouu
+du|| W|o | +u +u\|ou +ud uu|+pp, pe|ou.
Corrou de|r+|o|o|c corp|+|u| +|e |+c|+|
(W||u||e, +cue, c+|, |,pe||||c|o|, d|, ||p),
c+|p (|uc|p|eu| |+|due, |uc|e+ed |+|| |oW||),
eu||+| uo|r+| e|+ceou |+ud ou ||e peu|,
|ed c|o|ur (r+|e), |ed .u|.+, .+|u+| odo|
(|er+|e)|, |,pe|||d|o|, +ud ||or||d|o|.
\+u+ereu| | + p|o||er. 0ue ||+|e, | |o| ||e
de|r+|o|o|| |o +|ee W||| ||e p+||eu| ||+| ||e|e
| + p|o||er +ud ||u e|+|||| |+ppo||, |u + |eW
.||| ||e corp|+|u| c+u |e e\p|o|ed +ud |u|||e|
d|cued.
|| ||e p+||eu| +ud p|,|c|+u do uo| +|ee ||+|
||e corp|+|u| | + .+||, e\+e|+|ed ||u o| |+||
c|+ue, ||eu ||e p+||eu| |ou|d |e |e|e||ed |o
+ p,c||+||||, ||| |+||e| p|+u | uu+||, uo| +c
cep|ed, |u W||c| c+e ||e p|o||er r+, pe|||
|ude||u||e|,.
800 0SM0kFhIC SN0k0M (80S) |C|9 . !00.1
|C|0 . | +5.2
I|| |+|e d|o|de|, W||c| occu| |u +du|| +ud |
p|eeu| |o| rou|| o| ,e+|, | +oc|+|ed W|||
p+|u o| p+|e||e|+ +ud | c|+|+c|e||/ed |, ||e
p|eeuce o| uure|ou ||u |e|ou, ro||, e\co
||+||ou, W||c| ||e p+||eu| ||u|, |e||e.e +|e ||e
|eu|| o| + p+|+|||c |u|e|+||ou (||. 2!).
I|e oue| o| ||e |u|||+| p|u|||u o| p+|e||e|+ r+,
|e |e|+|ed |o \e|o| o|, |u |+c|, |o + p|e.|ou|,
||e+|ed |u|e|+||ou.
|+||eu| p|c| W||| ||e|| ||ue|u+|| o| d| |u|o
||e|| ||u W||| ueed|e o| |Wee/e| |o |ero.e ||e
'p+|+||e (||. 2!).
|| | |rpo||+u| |o |u|e ou| o||e| c+ue o| p|u|||u.
I|| p|o||er | e||ou, p+||eu| ||u|, u||e| +ud
+|e oppoed |o ee||u p,c||+|||c |e|p. |+||eu|
r+, e|| ||e|| |oue |o ro.e +W+, ||or ||e o|
|eud|u p+|+||e.
I|e p+||eu| |ou|d ee + p,c||+|||| |o| +| |e+|
oue .||| +ud |o| |ecorreud+||ou o| d|u
||e|+p,. p|ro/|de p|u +u +u||dep|e+u|. I|e+|
reu| | d||||cu|| +ud uu+||, uuucce|u|.
0ISI0NS 0F FAkASII0SIS |C|9 . !00.29
|C|0 . | 22.0
Compulsive habits
Neuiotic excoiiations
Tiichotillomania
Factitious syndiomes
Cutaneous signs of injecting diug use
SCII0N 23 ||'0k|Ek' 0| |'\Cn|AIk|C EI|0|0C\ 583

|-|: -:c| +|e uo| +u uucorrou
p|o||er, occu|||u |u |er+|e ro|e ||+u |u r+|e
+ud |u ||e ||||d |o ||||| dec+de.
I|e, r+, |e|+|e ||e oue| |o + pec|||c e.eu| o| |o
c||ou|c ||e, p+||eu| deu, p|c||u +ud c|+|c|
|u.
I|e c||u|c+| |e|ou +|e +u +dr|\|u|e o| e.e|+|
|,pe o| |e|ou, p||uc|p+||, e\co||+||ou, +||
p|oduced |, |+|||u+| p|c||u o| ||e ||u W|||
||e ||ue|u+||, ro| corrou ou ||e |+ce (||.
2!2), |+c| (||. 2!!), +ud e\||er|||e |u| +|o
+| o||e| ||e (ee ||. !!5, !!o). I|e|e r+,
|e dep|reu|ed +||op||c o| |,pe|p|reu|ed
r+cu|e c+| (||. 2!!).
|- |- c- |:c|-! |, |- ||c| ||-
|c! :c -c:| || ||- c ||- :-|- |
||- |c:|
I|e d|+uo| c+u |e decep||.e, +ud W|+| p||r+
|+c|e +ppe+| |o |e ueu|o||c e\co||+||ou cou|d |e
+ e||ou c+ue o| p|u|||u.
|,c||+|||c u|d+uce r+, |e uece+|, || ||e
p|o||er | uo| o|.ed, + || c+u |e .e|, d|||u||u
ou ||e |+ce +ud d||up||.e |o ||e p+||eu| +ud ||e
|+r||,. I|e cou|e | p|o|oued, uu|e |||e +dju|
reu| +|e r+de.
||ro/|de |+ |eeu |e|p|u| |u| ru| |e ued W|||
c+u||ou +ud W||| ||e +d.|ce +ud u|d+uce o| +
p,c|op|+|r+co|o||. A|o, +u||dep|e+u| d|u
r+, |e ued.
I||c|o||||or+u|+ | + corpu||.e de||e o| |+||| |o
p|uc| |+||. C+u |e ou ||e c+|p o| +u, o||e| |+||,
|e|ou (e.., |e+|d). Cou||ueuce o| +|e+ W||| .e|,
|o|| p+|e |+||, r+|| |+|d +|e+, +ud uo|r+|
+|e+ o| c+|p (||. 2!+). \o|e p|ououuced ou
|de o| dor|u+u| |+ud. C+u |e cor||ued W|||
ueu|o||c e\co||+||ou |uduced |, .|o|ou p|uc|
|u W||| |Wee/e|. \|c|ocop|c+||,, +u+eu |+||,
||uu||, ||o|eu |+||. I|e+|reu| + |o| ueu|o||c
e\co||+||ou.
|C|9 . o93.+
|C|0 . | 93.
Nk0IIC XC0kIAII0NS AN0 IkICh0IIII0MANIA
FICk 23-1 0e|usoos oI parastoss 4. uu+||, p+||eu| co||ec| r+|| p|ece o| de||| ||or ||e|| ||u |,
c|+|c||u W||| ||e|| u+|| o| +u |u||ureu| +ud u|r|| ||er |o ||e doc|o| |o| e\+r|u+||ou |o| p+|+||e. |u |||
c+e po|u|ed |Wee/e| We|e ued +ud ||e |eu|| +|e u|ce|, c|u|ed |e|ou, +ud c+|. B. 0cc+|ou+||, ||| c+u
p|o|e |o +u +|e|.e |e|+.|o| uc| + dep|c|ed |u ||| c+e W|e|e ||e p+||eu| poed |o derou||+|e |oW |e
co||ec| ||e 'p+|+||e ||or |e| ||u ou + p|ece o| p+pe|. |u ||e r+jo|||, o| c+e, p+||eu| +|e uo| d|u+ded ||or
||e|| rouo,rp|or+||c de|u|ou.
4 B
FICk 23-2 Neurotc excor-
atoos 'e.e|+| e|,||er+|ou +ud
c|u|ed r+cu|e +ud e|o|ou ou ||e
|oWe| c|ee| +ud uppe| ||p o| + 9
,e+|o|d |er+|e W||| r||d |+c|+| +cue.
|o p||r+|, |e|ou +|e eeu. I|e
p+||eu|, W|o | rode|+|e|, dep|eed,
|+ r||d +cue||o|r |e|ou, W||c|
|e corpu||.e|, p|c| W||| |e| ||u
e|u+||.
FICk 23-3 Neurotc excoratoos: back E\co||+||ou o| ||e uppe|, r|d|+c| +ud |u|e+| +|e+ +ud ||ue+|
+|e+ o| po||u||+rr+|o|, |,pe|p|reu|+||ou, c|u||u, +ud c+|||u |u + oo,e+|o|d d|+|e||c |er+|e. |e|ou |+.e
|eeu p|eeu| |o| +| |e+| 0 ,e+| |u| ||e u|ce|+|ed c|u|ed |e|ou |eo|.ed W||| c|o|| |+pe occ|u|ou. 0uce ||e
p|o|ec||ou W+ |ero.ed, ||e p+||eu| |eured e\co||+||u ||e ||e.
SCII0N 23 ||'0k|Ek' 0| |'\Cn|AIk|C EI|0|0C\ 585
FICk 23-4 Irchot||omaoa I|| e\|eu|.e +|opec|+ |+ |eu||ed ||or pu|||u +ud p|uc||u |+|| |, ||e
1,e+|o|d p+||eu|. '|e +ppe+|ed |+|+uced |u| r||d|, dep|eed +ud |+d cou|de|+||e cou|||c| W||| |e| p+|eu|.
'|e +dr|||ed pu|||u |+|| +||e| cou|de|+||e que||ou|u.
I|e |e|r |c:|| re+u '+|||||c|+|, +ud |u |||
coud|||ou ||e|e | + e|||uduced de|r+|o|o|c
|e|ou(), e|||e| ||e p+||eu| c|+|r uo |epou||||
||, o| +dr|| de|||e|+|e|, ru|||+||u ||e ||u.
|| occu| |u ,ouu +du||, |er+|e > r+|e. I|e
|||o|, | .+ue ('|o||oW |||o|,) o| ||e e.o|u
||ou o| ||e |e|ou.
I|e |e|ou r+, |e p|eeu| |o| Wee| |o rou||
|o ,e+| (||. 2!5).
|+||eu| r+, |e uo|r+| |oo||u +ud +c| uo|r+||,
|u e.e|, |epec|, +|||ou| ||equeu||, ||e|e | +
||+ue +||ec| +ud ||/+||e pe|ou+|||,.
I|e ||u |e|ou cou|| o| cu|, u|ce|, deue +d
|e|eu| uec|o||c rer||+ue (||. 2!o). I|e |+pe
o| ||e |e|ou r+, |e ||ue+| (||. 2!5), ||/+||e
|+pe, eore|||c p+||e|u, |u|e o| ru|||p|e.
I|e d|+uo| c+u |e d||||cu||, |u| ||e u+|u|e o|
||e |e|ou (||/+||e |+pe) r+, |rred|+|e|,
ue| +u +|||||c|+| e||o|o,.
|| | |rpo||+u| |o |u|e ou| e.e|, po|||e
c+ue-c||ou|c |u|ec||ou, |+uu|or+, +ud
.+cu||||-pe||o|r + ||op, |e|o|e +|u|u ||e
d|+uo| o| !-c| c|-|c:|c, |o|| |o| ||e
|eue||| o| ||e p+||eu| +ud |ec+ue ||e p|,|c|+u
r+, |e +| ||| |o| r+|p|+c||ce || |e o| |e |+|| |o
d|+uoe + ||ue p+||o|o|c p|oce.
I|e|e | o||eu e||ou pe|ou+|||, +ud/o| p,c|o
oc|+| ||e, o| + p,c||+|||c d|e+e.
I|e coud|||ou der+ud ||e u|ro| |+c| ou ||e p+||
o| ||e p|,|c|+u, W|o c+u +.e|| + e||ou ou|core
(|.e., u|c|de) |, +||erp||u |o +|u euou| er
p+||, W||| ||e p+||eu| |o +ce||+|u ||e c+ue. I||
.+||e W||| ||e u+|u|e o| ||e p,c||+|||c p|o||er.
I|e coud|||ou r+, pe||| |o| ,e+| |u + p+||eu|
W|o |+ e|ec|ed || o| |e| ||u + ||e |+|e|
o|+u o| || o| |e| cou|||c|. Couu||+||ou +ud
r+u+ereu| W||| + p,c||+|||| +|e r+ud+|o|, |u
ro| p+||eu|.
|C|9 . !0.5
|C|0 . | o3.
FACIIII0S SN0k0MS (MNChASN SN0k0M)
FICk 23-5 Facttous syodrome I|ee ||ue+| cu| We|e e|||u|||c|ed W||| + |+/o| ||+de |, + p+||eu| W|||
+ |o|de|||ue ,ud|ore. '|r||+|, ruc| deepe| cu| We|e ou ||e |o|e+|r.
SCII0N 23 ||'0k|Ek' 0| |'\Cn|AIk|C EI|0|0C\ 58T
Cutaoeous Iojectoo keactoos Cutunevus 1n-
ury Multiple punctuies at sites of cutaneous
injection, often lineai ovei veins (Fig. 23-7).
FvreIgn Bvdy Grunu|vmu Subcutaneous in-
jection of adulteiants (talc, sugai, staich, baking
soda, floui, cotton fibeis, glass, etc.) can elicit a
foieign body iesponse cellulitis gianuloma
ulceiation. (Fig. 23-8)
Iotravascu|ar Iojectoo keactoos Venvus 1nury
Intiavenous injection can iesult in thiombosis,
thiombophlebitis, septic phlebitis. Chionic ede-
ma of the uppei extiemity is common.
ArterIu| 1nury Chionic intiaaiteiial injection
can iesult in injection site pain, cyanosis, eiy-
thema, sensoiy and motoi deficits, and vasculai
compiomise (vasculai insufficiency/gangiene).
|ujec||u d|u ue| o||eu de.e|op cu|+ueou ||
r+|+ + + |eu|| o| ||e|| |+|||, W|e||e| |ujec||u
u|cu|+ueou|, o| |u||+.+cu|+||,.
Cu|+ueou |e|ou |+ue ||or |o|e|u |od, |e
poue |o |ujec|ed r+|e||+|, |u|ec||ou, +ud c+|.
CIAN0S SICNS 0F INlCIINC 0kC S |C|9 . 999.!
IoIectoos TrunsmIssIvn v] 1n]ectIvus Agents
Injecting diug use can iesult in tiansmission of
HIV, hepatitis B viius (HBV), and hepatitis C
viius (HCV) with subsequent life-thieatening
systemic infections.
1nectIvn SIte 1n]ectIvns Local infections in-
clude cellulitis (Fig. 23-8), abscess foimation,
lymphangitis, septic phlebitis/thiombophlebitis.
The most common oiganisms aie those fiom
the diug useis, e.g., S. aureus and GAS. Less
common miciobes: enteiic oiganisms, anaei-
obes, C|osrJum |ou|num , oial floia, fungi
(CanJJa a||tans ), and polymiciobial infec-
tions.
SystemIc 1n]ectIvns Intiavenous injection of
miciobes can iesult in infection of vasculai
FICk 23-6 Facttous syodrome I|ee uec|oe We|e e|||u|||c|ed |, ||e co.e|| +pp||c+||ou o| d||u|ed
u||u||c +c|d +ud |||||, |||||u |+ud+e. I|e p+||eu| +ppe+|ed We||+dju|ed +ud |e|ued |o ee + p,c||+||||.
endothelium, most commonly heait valve with
infectious endocaiditis.
Scars LIneur Scurs Multiple cutaneous punc-
tuies iesult in lineai scaiiing along the couise of
veins, i.e., needle tiacks`` (Fig. 23-7). These aie
found on the foieaims, doisum of the hands,
wiists, antecubital/popliteal fossae, penis.
AtrvphIc Punched-Out Scurs Result fiom
subcutaneous injections (i.e., skin popping``)
aftei an inflammatoiy (steiile oi infected) ie-
sponse to injected mateiial.
Tuttvvs Caibon on needles (aftei flame steii-
lization) can iesult in inadveitent tattooing and
pigmented lineai scais.
FICk 23-T Iojecto dru use: ojectoo tracks over veos oo the dorsum oI the haod ||ue+|
||+c| W||| puuc|u|e, ||||o|, +ud c|u| We|e c|e+|ed |, d+||, |ujec||ou |u|o ||e upe|||c|+| .e|u.
FICk 23-8 Iojecto dru use: ce||u|ts aod Ioreo body respoose at ojectoo ste I|e p+||eu|
|ujec|ed |u|o ||e u|cu|+ueou ||ue + We|| + .e|u o| ||e |o|e+|r, |eu|||u |u |o|e|u |od, |epoue +ud
' c- ce||u|||| W||| +oc|+|ed |+c|e|er|+ +ud |u|ec||ou eudoc+|d|||.
| A k I | | |
DI5EA5E5 DUE IO
MICkOIAL ACENI5
590
S E C I | 0 N 2 4
8ACIkIAI INFCII0NS
INv0IvINC Ih SkIN
Co+u|+eue+||.e |+p|,|ococc| (Co|') uo|
r+||, co|ou|/e ||u, ro|e |e+.||, |u occ|uded ||+u
uouocc|uded ||e (+\|||+e, +uoeu||+||+)
E|,|||+r+, p|||ed |e|+|o|,|, |||c|or,co|, +ud
|u|ec||ou |u|e||||o +|e corrou upe|||c|+| cu|+
ueou |u|ec||ou
'|c|,|::: c- co|ou|/e ||e u+|e +ud
|u|e|||||uou ||u |u|e|r|||eu||,, c+u peue||+|e
||e ||+|ur co|ueur, +ud c+u c+ue |u|ec||ou,
e.., |rpe||o, |o|||cu||||
\e|||c|||||u|e||+u| ' c- (\k'A) |+ |e
core +u |rpo||+u| p+||oeu |o| corruu||,
+cqu||ed (CA\k'A) +ud |e+||| c+|e+cqu||ed
(nA\k'A) |u|ec||ou
E|,|||+r+. |u|e|||||uou ||e o| We|p+ce o|
|ee|, |o|u, +\|||+e
||||ed |e|+|o|,|. p|+u|+| |ee| +ud We|p+ce o|
|ee|
I||c|or,co|. +\|||+e, pu||
' c- +ud |oup A ||ep|ococcu (CA') c+ue
cu|+ueou |u|ec||ou +ud ,|er|c |u|o\|c+||ou
E||o|o,. o.e||oW|| o| uo|r+| ||o|+ +| ||e o|
||u occ|u|ou
Noimal skin is heavily colonized by bacteiial
floia (haimless commensals) such as coagu-
lase negative staphylococci (CoNS). Coloni-
zation is moie dense in inteitiiginous and
occluded sites.
Pathogens Sa|y|otottus aureus and, less
commonly, gioup A -hemolytic stieptococ-
cus (GAS) (Sreotottus yogenes) colonize,
and infect the skin. They cause a vaiiety of
syndiomes, including skin and soft tissue
infections (SSTIs), bacteiemia, and systemic
intoxications. An intact stiatum coineum is
the most impoitant defense against invasion
of pathogenic bacteiia.
Piedispositon to infection:
Chionic S. aureus caiiiei state (naies, axil-
lae, peiineum, vagina)
Waim weathei/climate, high humidity
Skin disease, especially atopic deimatitis,
familial pemphigus
Social situation: pooi hygiene, ciowded liv-
ing conditions, neglected minoi tiauma
Chionic disease: obesity, diabetes mellitus,
HIV/AIDS, especially MRSA infection, solid
oigan tiansplant iecipient, iatiogenic im-
munosuppiession
Immunodeficiency: cancei chemotheiapy,
bacteiicidal defects (e.g., chionic gianulo-
matous disease), chemotactic defects, hy-
pei-IgE syndiome (Job syndiome)
Coagulase negative staphylococci (CoNS),

colonizing the skin shoitly aftei biith, have
been subdivided into 32 species, 15 of which
aie indigenous to humans. The most com-
mon CoNS aie S. eJermJs (65-90% of
individuals), S. |omns. S. |aemo|ytus,
S. warner, and S. |ugJunenss. CoNS have
lowei pathogenicity in the skin and mucosa
but incieasingly cause infection of aitificial
devices such as peicutaneous intiavenous
cathetei (PIC) lines and heait valves.
Gioup A stieptococcus (GAS) usually colo-
nizes the skin fiist and then the nasophaiynx.
Gioup B (Sreotottus aga|atae) and gioup
G -hemolytic stieptococci (GBS, GGS) colo-
nize the peiineum of some individuals and
may cause supeificial and invasive infections.
S. aureus does not noimally ieside on human
skin (not one of the noimal iesident floia),
but may be piesent tiansiently, inoculated
fiom colonized sites such as the naies. Colo-
nization and infection follow contact with
shedding human lesions, fomites contami-
nated fiom these lesions, and human iespiia-
toiy tiact and skin. The naies of pets can also
be colonized. S. aureus of mucous membianes
of the anteiioi nasophaiynx (naies) of 30%
of otheiwise healthy peisons; othei com-
monly colonized sites include axillae, vagina
(5-15%; up to 30% duiing menses), damaged
SCII0N 24 BACIEk|A| |||ECI|0|' ||\0|\||C InE 'K|| 591
skin, peiineum. Colonization is usually intei-
mittent; 10-20% of individuals have peisist-
ent colonization; 10-20% aie nevei colonized.
Colonization iates highei among health caie
woikeis, dialysis patients, patients with type
1 diabetes, injection diug useis, peisons with
HIV/AIDS disease, those with atopic deima-
titis (90% in deimatitis, 70% of nonlesional
skin).
Methicillin-iesistant S. aureus (MRSA)
emeiged in the 1960s as a cause of infections
among patients in health caie settings (opeiat-
ing iooms, intensive caie units, cancei chemo-
theiapy waids, newboin nuiseiies, chionic
caie facilities). Cuiiently, MRSA infections
aie acquiied in health caie settings and in the
community (healthy individuals, those with
chionic disease, piisoneis, intiavenous-diug
useis, athletes, militaiy tiainees, men who
have sex with men). Community-associated
MRSA cause SSTIs, sepsis, and neciotizing
pneumonia. Compaied with health caie-as-
sociated MRSA (HA-MRSA) isolates, com-
munity-associated MRSA (CA-MRSA) isolates
tend to be iesistant to fewei antibiotics, to
pioduce diffeient toxins, and to have diffeient
types of the gene complex known as staphylo-
coccal cassette chiomosome mec (SCCmec);
this complex contains the mecA gene that
confeis methicillin iesistance. In some met-
iopolitan aieas in the United States, MRSA
causes the majoiity of SSTIs. Clindamycin, tii-
methopiim-sulfamethoxazole, iifampin, and
doxycycline aie iecommended foi outpatient
empiiical tieatment of CA-MRSA SSTI. Com-
bination iifampin plus tiimethopiim-sulfam-
ethoxazole eiadicates CA-MRSA colonization
and tieats infection.
Diugs foi seiious invasive MRSA infections:
vancomycin, clindamycin, daptomycin, tige-
cycline, linezolid. Paitial vancomycin iesist-
ance may iesult in failuies among MRSA
infections.
MRSA is the most common identifiable cause
of SSTI among patients piesenting to emei-
gency depaitments in many laige U.S. cit-
ies. When antimiciobial theiapy is indicated
foi the tieatment of SSTIs, clinicians should
considei obtaining cultuies and modifying
empiiical theiapy to piovide MRSA coveiage.
'||u +ud o|| ||ue |u|ec||ou. |rpe||o/ec||,r+,
e|,|pe|+, ce||u||||, uec|o||/|u |+c|||| (||ep|o
cocc+| +u|eue), Wouud |u|ec||ou
B+c|e|er|+ +ud || corp||c+||ou. ep|/ep||c
|oc|, re|+|+||c |u|ec||ou, |u|ec||.e eudoc+|d|||
Io\|ured|+|ed |||uee. c+||e| |e.e|, ||ep|ococ
c+| |o\|c |oc| ,ud|ore
|o|||ep|ococc+| d|e+e. ||eur+||c |e.e|,
|ore|u|ouep|||||
5IkFPI0C0CC5 PY06FNF5 [Ck0F A SIkFI0C0CCS (CAS)j:
INFCII0NS AN0 INI0XICAII0NS
'||u +ud o|| ||ue |u|ec||ou (''I|). |rpe||o/
ec||,r+, |o|||cu||||, |u|uuc|e/c+||uuc|e, ce||u||||,
r+||||, Wouud |u|ec||ou
\ucu|o|e|e|+| |u|ec||ou. ep||c +|||||||, p,or,
o|||, po+ +|ce
kep||+|o|, ||+c| |u|ec||ou. .eu|||+|o|+oc|+|ed
o| uoocor|+| pueurou|+, ep||c pu|rou+|,
er|o||, po|.||+| pueurou||| (e.., |u||ueu/+),
erp,er+
B+c|e|er|+ +ud || corp||c+||ou. ep|/ep||c
|oc|, re|+|+||c |u|ec||ou (jo|u|, |oue, ||due,,
|uu, p+|+p|u+|, ||+|u), |u|ec||.e eudoc+|d|||
(|ujec||ou d|uue +oc|+|ed, u+||.e .+|.e, p|o
||e||c .+|.e, uoocor|+|)
|e.|ce|e|+|ed |u|ec||ou. |\ c+||e|e|, p|o||e||c
jo|u|
Io\|ured|+|ed |||uee. |o\|c |oc| ,ud|ore,
|+p|,|ococc+| c+|ded||u ,ud|ore, |ood po|
ou|u
5IPBYI0C0CC5 kF5: INFCII0NS AN0 INI0XICAII0NS
FAkI III ||'EA'E' |uE I0 \|Ck0B|A| ACE|I' 592
I||ee upe|||c|+| |+c|e||+| '|u|ec||ou occu| |u ||e
||+|ur co|ueur +ud |+|| |o|||c|e.
E|,|||+r+. |u|e|||||uou ||e o| We|p+ce o|
|ee|, |o|u, +\|||+e
||||ed |e|+|o|,|. p|+u|+| |ee| +ud We|p+ce o|
|ee|
I||c|or,co|. +\|||+e, pu||
||ed|po|||ou. ||| u||+ce |ur|d||,
E||o|o,. o.e||oW|| o| uo|r+| ||o|+ +| ||e o|
||u occ|u|ou
SFkFICIAI 8ACIkIAI FI0kMAI C0I0NIIAII0NS AN0 INFCII0NS
||or ||e C|ee|. '|ed po|
E||o|o,. C,-|c:|- |
||||||u||ou. |u|e|||||uou +|e+ o| We|p+ce o|
|ee|, |o|u, +\|||+e, u|r+rr+|, +|e+
C||u|c+| ||ud|u. We||der+|c+|ed |ed o| |+u
p+|c|e, c+|e
||||uu|| ||or de|r+|op|,|o| +ud uou|u|ec
||ou |u|e||||o
||+uo|. wood |+rp e\+r|u+||ou |oW co|+|
|ed ||uo|eceuce
kIhkASMA |C|9 . 0!9.0
|C|0 . |03.
FI0MI0I0C
Ae oI 0oset Adults.
to|oy C. mnussmum, giam-positive
(diphtheioid), non-spoie-foiming, aeiobic oi
facultatively anaeiobic bacillus; pait of noimal
skin floia, which causes supeificial epideimal
infection undei ceitain conditions.
Fredsposo Factors Humid cutaneous mi-
cioclimate: waim and/oi humid climate oi
season; occlusive clothing/shoes; obesity, hy-
peihidiosis.
Symptoms Usually asymptomatic. Duiation:
weeks to months to yeais. Fiequently misdiag-
nosed as tinea ciuiis oi pedis.
Sko Iesoos Patches, shaiply maiginated
(Fig. 24-1). Scaling at sites not continuously
occluded. In webspaces of feet, may be mac-
eiated (Fig. 24-2), eioded, oi fissuied. Often
symmetiic oi in multiple webspaces. Red oi
biownish ied; postinflammatoiy hypeipigmen-
tation in moie heavily melanized individuals.
If piuiitic, secondaiy changes of excoiiation,
lichenification. Deimatophytosis, candidiasis,
and pseudomonal webspace infection may also
be piesent.
SItes v] PredI|ectIvn Toe webspaces (Fig. 24-2)
>> inguinal folds > axillae; also, inteitiiginous
skin undei panniculus, inteigluteal, infiamam-
maiy (submammaiy).
We||-0emarcated Iotertroous F|aque Dei-
matophytosis, inteitiiginous candidiasis, pity-
iiasis veisicoloi, pitted keiatolysis (webspace of
feet), inteitiiginous psoiiasis, acanthosis nigii-
cans, familial pemphigus (inguinal, axillaiy).
Wood Iamp Chaiacteiistic coial-ied fluoies-
cence (attiibuted to copiopoiphyiin III). May
not be piesent if patient has bathed iecently.
0rect Mcroscopy Negative foi fungal foims
on KOH piepaiation of skin sciaping. In the
webspaces of the feet, concomitant inteidigital
tinea pedis may also be piesent. Giam oi Giemsa
stains may show fine bacteiial filaments.
8actera| Cu|ture Heavy giowth of Coryne|at-
erum . Rules out Sa|y|otottus aureus, gioup
A oi gioup B stieptococcus, and CanJJa infec-
tion. In some cases, concomitant PseuJomonas
aerugnosa webspace infection (feet) is also
piesent.
0IACN0SIS
Clinical findings, absence of fungi on diiect
micioscopy, positive Wood lamp examina-
tion.
C0kS
Relapse occuis if piedisposing causes aie not
coiiected. Secondaiy piophylaxis usually indi-
cated. Veiy iaiely, C. mnussmum iepoited to
cause invasive infection with bacteiemia and its
complications.
MANACMNI
Freveotoo[Frophy|axs Wash with benzoyl
peioxide. Medicated powdeis (do not use coin
staich powdei). Topical antiseptic alcohol gels:
isopiopyl, ethanol.
Iopca| Iherapy Piefeiable. Benzoyl peioxide
(2.5%) gel daily, aftei showeiing, foi 7 days.
Topical eiythiomycin oi clindamycin solution
twice daily foi 7 days. Sodium fusidate ointment,
mupiiocin ointment oi cieam. Topical antifungal
agents; clotiimazole, miconazole, oi econazole.
Systemc Aotbotc Iherapy A maciolide oi a
tetiacycline foi 7 days.
FICk 24-1 rythrasma: roos '|+|p|, r+|
|u+|ed, |ed p+|c| |u ||e +\|||+ (|u|ec||ou |u|e||||o).
wood |+rp e\+r|u+||ou |oW ||||| co|+||ed, d||
|e|eu||+||u e|,|||+r+ ||or |u|e|||||uou po||+|.
K0n p|ep+|+||ou W+ ue+||.e |o| |,p|+e.
FICk 24-2 rythrasma: web-
space I|| r+ce|+|ed |u|e|d|||+| We|
p+ce (|u|ec||ou |u|e||||o) +ppe+|ed |||||
co|+||ed W|eu e\+r|ued W||| wood |+rp,
K0n p|ep+|+||ou W+ ue+||.e |o| |,p|+e.
I|e We|p+ce | ||e ro| corrou ||e
|o| e|,|||+r+ |u |erpe|+|e c||r+|e. |u
ore c+e, |u|e|d|||+| ||ue+ ped| +ud/o|
peudorou +| |u|e||||o r+, coe\||.
E||o|o,. |,|::: -!-|c
||||||u||ou. p|+u|+| |ee|, We|p+ce o| |ee|
||ed|po|||ou. |,pe|||d|o| +ud occ|u|.e
|oo|We+|
C||u|c+| ||ud|u. de|ec| |u |||c||, |e|+||u|/ed ||u
W||| e|oded p|| o| .+||+||e dep||
FIII0 kkAI0ISIS (kkAI0ISIS SICAIA)
FI0MI0I0C
to|oy K. seJenarus . Age of onset: young
adults. Sex: males > females. Piedisposing factois:
hypeihidiosis of the feet; occlusive footweai. K.
seJenarus pioduces two extiacellulai pioteases
that can digest keiatin. Incidence iepoited to be
43% of paddy field woikeis in India.
Sko Symptoms Usually asymptomatic. Foot
odoi. Sliminess of feet. Uncommonly, itching,
buining, tendeiness. Often mistaken foi tinea
pedis.
Sko Iesoos Ciatei-like pits in stiatum coi-
neum, 1-8 mm in diametei (Figs. 24-3, 24-4).
FICk 24-3 Ftted kerato|yss: p|aotar I|e ||+|ur co|ueur o| ||e +u|e||o| p|+u|+| |oo| |oW |o o|
|e|+||u|/+||ou W||| We||de|r+|c+|ed c+||oped r+||u, |o|red |, ||e cou||ueuce o| ru|||p|e, cou||ueu| 'p||
(de|ec| |u ||e ||+|ur co|ueur).
Pits can iemain disciete oi, moie often, become
confluent, foiming laige aieas of eioded stia-
tum coineum. Involved aieas aie white when
stiatum coineum is fully hydiated. Symmetiic
oi asymmetiic involvement of both feet.
DIstrIhutIvn Piessuie-beaiing aieas, vential
aspect of toe, ball of foot, heel. Fiiction aieas:
inteiface of toes.
rosoo o Mu|tp|e Webspaces oI Feet
Inteidigital tinea pedis, CanJJa inteitiigo,
eiythiasma, PseuJomonas webspace infection.
0rect Mcroscopy KOH piepaiation negative
foi hyphae.
Wood Iamp xamoatoo Negative foi biight
coial-ied fluoiescence (eiythiasma).
Cu|ture In some cases, iule out S. aureus,
gioup A stieptococcus (GAS), oi P. aerugnosa
infection.
0IACN0SIS
Clinical diagnosis iuling out othei causes.
C0kS AN0 Fk0CN0SIS
Peisists and iecuis until the undeilying piedis-
posing factois aie coiiected. Secondaiy piophy-
laxis usually indicated.
FICk 24-4 Ftted kerato|yss: toe |eep|,
p|||ed ep|de|r| o| +u |u|e|||||uou |oe, +oc|+|ed
W||| |,pe|||d|o|. ||| +|e de|ec| |u ||e ||+|ur co|
ueur, |ecoru cou||ueu| |u ||e occ|uded We|p+ce.
E||o|o,. C,-|c:|- , |+rpo|||.e d|p|
||e|o|d. || |uuu.
||||||u||ou. +\|||+e (|||c|or,co| +\|||+||), pu||c
|+|| (|||c|or,co| pu||)
||ed|po|||ou. |,pe|||d|o| +ud occ|u|.e |oo|
We+|
\o|e corrou |u r+|e ||+u |er+|e, +du||
C||u|c+| ||ud|u. |+uu|+| couc|e||ou (,e||oW,
||+c|, o| |ed) ou |+|| |+||. n+|| +ppe+| |||c|
eued, |e+ded, |||r|, +d|e|eu|.
|uo|u||e +d|e|.e r+, e|ode cu||cu|+| +ud co|||
c+| |e|+||u.
\+u+ereu|. |+.e o|| +||ec|ed |+||. Beu/o,|
pe|o\|de W+|. A|co|o| e|. Iop|c+| e|,|||or,c|u
o| c||ud+r,c|u.
IkICh0MC0SIS |C|9 . 0!9.0
|C|0 . A+3.3/|03.3
MANACMNI
See Eiythiasma," above. Intiadeimal botuli-
num toxin injection is effective foi hypeihid-
iosis and has been iepoited to be effective foi
pitted keiatolysis.
|u|e||||o (|+||u |- '|e|Weeu, | '|u|||u)
|oupec|||c |u||+rr+||ou o| oppoed ||u (|u
||+r+rr+|, |e|ou, +\|||+e, |o|u, |u|e+| |o|d,
|eduud+u| ||u |o|d o| o|ee |ud|.|du+|.
C||u|c+| ||ud|u. E|,||er+ ,rp|or o| p|u||
|u, |eude|ue, o| |uc|e+ed eu|||.||,, e\c|ud|u
|u|ec||ou c+ue.
ku|e ou| |u|ec||ou |u|e||||o. |+c|e||+ CA' (||.
2+5, 2+o) +ud |oup B ||ep|ococcu (CB') (||.
2+1)|, C | (e|,|||+r+) , | c-
c , (||. 2+3), +ud |uu| de|r+|op|,|e,
Cc!!c !c|c-cc || (p||,||+| .e||co|o|)|
ku|e ou| de|r+|oe. po||+| .u|+||, e|o|
||e|c de|r+||||, +|op|c de|r+||||
||ed|po|||ou. ||| u||+ce |ur|d||,
\+u+ereu|.
',rp|or+||c |e||e|. ro|| d|e|u +ud/o| C+
|e||+u| p+|u|
|oWde| W||| +u|||+c|e||+|/+u|||uu+| +c||.||, +|e
|e|p|u| |o| p|e.eu||u |ecu||euce.
/|uc o\|de o|u|reu| |educe |||c||ou +| |u.o|.ed
||e +ud p|o|ec| ||u ||or u||ue o| |ece (+uo
eu||+| |u|e||||o)
Iop|c+| |ucoco|||co|d p|ep+|+||ou |e| +.o|ded
|ec+ue o| ||| o| cu|+ueou +||op|, +| ||ee
u+|u|+||, occ|uded ||e
||rec|o||ru c|e+r +ud |+c|o||ru o|u|reu|
r+, |e e||ec||.e, W|||ou| ||| o| +||op|,.
we||| |educ||ou
|+uu|cu|ec|or, r+, |e |ud|c+|ed +||e| e\||ere
We||| |educ||ou.
N0NSFCIFIC INIkIkIC0 |C|9 . o95.39
|C|0 . |!0.+
FICk 24-5 Ioter|utea| otertro: roup A
streptococcus (CAS) A p+|u|u| ro|| e|,||er+
|ou p|+que |u + r+|e W||| |u|e|||||uou po||+|,
W||| |ou| odo|. |u|ec||ou |eo|.ed W||| peu|c||||u \K.
FICk 24-6 Feroea| otertro: roup A
streptococcus we||de|r+|c+|ed e|,||er+ +ud
e|o|ou |u ||e pe||ueur o| +u 3,e+|o|d |o, +oc|
+|ed W||| p|u|||u +ud |eude|ue (pe||+u+| ||ep|o
cocc+| 'ce||u||||).
SCII0N 24 BACIEk|A| |||ECI|0|' ||\0|\||C InE 'K|| 59T
FICk 24-T Iouoa| otertro: roup 8
streptococcus (C8S) |+|u|u| |ou|re|||u |u|e||||o
|u ||e |e|| |uu|u+| +|e+ W+ p|eeu| |o| e.e|+| d+,
|u + ro|||d|, o|ee d|+|e||c |er+|e. Cu||u|e |epo||ed
|e+., |oW|| o| CB', W||c| corrou|, co|ou|/ed ||e
+uoeu||+| +|e+. ',rp|or |eo|.ed W||| peu|c||||u \K.
FICk 24-8 Webspace otertro: P. oeru-
yoeso E|o|ou o| + We|p+ce o| ||e |oo| W||| +
||||| |ed |+e +ud u||ouud|u e|,||er+. I|ue+
ped| (|u|e|d|||+| +ud rocc+|up+||e|u) +ud |,pe|
||d|o| We|e +|o p|eeu|, W||c| |+c||||+|ed |oW|| o|
|-!c.
F00kMA |C|9 . o3+
|C|0 . |0
E||o|o,. ' c- +|o, CA'
|u|ec||ou o| ||e ep|de|r| (|rpe||o), W||c| r+,
e\|eud |u|o ||e de|r| (ec||,r+)
C||u|c+| ||ud|u.
|rpe||o. c|u|ed e|o|ou
Ec||,r+. c|u|ed deep e|o|ou o| u|ce|
|o||+| o| eu||,
|||r+|, |u|ec||ou |u r|uo| upe|||c|+| ||e+| |u
||e ||u
'ecoud+|, |u|ec||ou o| p|ee\|||u de|r+|oe
(|rpe|||u|/+||ou, o| ecoud+|, |u|ec||ou)
IMFIIC0 AN0 CIhMA |C|9 . o3o.30
|C|0 . B03.0
Ae oI 0oset Piimaiy infections moie com-
mon in childien. Secondaiy infections, any age.
Bullous impetigo: neonates, especially; childien
<5 yeais old.
to|oy
S. aureus most commonly
GAS
Bullous impetigo: epideimolytic toxin A-
(etA) gene pioducing S. aureus , which also
causes staphylococcal scalded skin syndiome.
Fredsposo Factors Topical glucocoiticoids
have little effect on the miciofloia of the skin,
except in those with atopic deimatitis; topical
glucocoiticoids applied to atopic deimatitis
usually ieduce the density of S. aureus . Ecthyma:
lesion of neglect-develops in excoiiations; in-
sect bites; minoi tiauma in diabetics, eldeily
patients, soldieis, and alcoholics.
Forta|s oI otry oI IoIectoo
PrImury 1mpetIgv Minoi bieaks in the skin.
Secvndury 1mpetIgv (1mpetIgInIzutIvn)
Undeilying deimatoses; tiaumatic bieaks and
wounds of the epideimis.
In[|ammaory Jermaoses : Atopic deimatitis,
stasis deimatitis, psoiiasis vulgaiis, chionic
cutaneous lupus eiythematosus, pyodeima
gangienosum.
Bu||ous Jsease : Bullous pemphigoid, sun-
buin, poiphyiia cutanea taida.
Inset |es
U|ters : Piessuie, venous insufficiency
C|ront |ym|eJema
Cuaneous n[etons (impetigo is supeiin-
fection): Heipes simplex, vaiicella, heipes
zostei; deimatophytosis (tinea pedis, tinea
capitis).
Trauma/wounJs : Suigical wounds; abiasion;
laceiation; punctuie; bites (human, animal,
insect); buins; ulceis; umbilical stump.
0uratoo oI Iesoos Impetigo: days to weeks.
Ecthyma: weeks to months.
Symptoms Impetigo: vaiiable piuiitus, es-
pecially associated with atopic deimatitis.
Ecthyma: pain, tendeiness.
Sko Iesoos 1mpetIgv Small eiosions with
ciust (Figs. 24-9, 24-10). Golden-yellow ciusts
aie often seen in impetigo but aie not pathog-
nomonic (Fig. 24-11). 1- to 3-cm lesions; cential
healing often appaient if lesions piesent foi sev-
eial weeks (Fig. 24-12). rrangemen : scatteied,
disciete lesions; without theiapy, lesions may be-
come confluent; satellite lesions occui by autoin-
oculation. Secondaiy impetiginization of vaiious
deimatosis is common (Figs. 24-13, 24-14).
Bu||vus 1mpetIgv Vesicles and bullae con-
taining cleai yellow oi slightly tuibid fluid
suiiounding eiythema, aiising on noimal-
appeaiing skin. With iuptuie, bullous lesions
decompiess. If ioof of bulla is iemoved, shallow
moist eroson foims (Fig. 24-15). Dsr|uon :
moie common in inteitiiginous sites.
Ecthymu Ulceiation with a thick adheient
ciust (Fig. 24-16). Lesions may be tendei, in-
duiated. Dsr|uon : moie common on distal
extiemities.
Msce||aoeous Fhysca| Fodos
At times, lymphangitis and/oi iegional lym-
phadenopathy.
rosoo Crust [Sca|e-Crust Excoiiation, allei-
gic contact deimatitis, heipes simplex, epideimal
deimatophytosis, scabies. T|e ma,ory o[ |esons
w| |oney-to|oreJ truss" are no mego .
FICk 24-9 Nostr| co|oota-
too aod mpeto: methc||o-
seostve 5. oureus (MSSA): oasa|
co|ootatoo aod mpeto oI
oares Co|ou|/+||ou o| ||e u+|e |
uu+||, +,rp|or+||c. I|| p+||eu| |+d
|eude|ue, e|,||er+, +ud c|u||u o|
||e ||u +dj+ceu| |o ||e u+|e, |ud|c+
||.e o| upe|||c|+| |u|ec||ou e\|eud|u
||or co|ou|/+||ou o| ||e uo||||.
FICk 24-11 Impeto: MSSA C|u|ed e|,||er+|ou e|o|ou |ecor|u cou||ueu| ou ||e uoe, c|ee|,
||p, +ud c||u |u + c|||d W||| u++| c+|||+e o| ' c- +ud r||d |+c|+| ec/er+.
FICk 24-10 Impeto: MSSA C|u|ed e|,||er+|ou e|o|ou ou ||e rou|+c|e +|e+ |u +u 3,e+|o|d |||.
\u|||p|e c+||e|ed o||e| |e|ou We|e +|o p|eeu|.
Iotact 8u||a(e) Alleigic contact deimatitis, in-
sect bites, theimal buins, heipes simplex, heipes
zostei, bullous pemphigoid, poiphyiia cutanea
taida (PCT) (doisa of hands), pseudo-poiphyiia.
|cer Crust[Sca|e-Crust Piuiigo nodulaiis,
chionic heipetic ulceis, excoiiated insect bites,
neuiotic excoiiations, cutaneous diphtheiia,
PCT, venous (stasis) and ischemic ulceis (legs).
Cram Stao Giam-positive cocci, in chains oi
clusteis, within neutiophils.
Cu|ture S. aureus , commonly; GAS. Failuie of
oial antibiotic suggests MRSA.
0ermatopatho|oy Impetigo: giam-positive
cocci in blistei fluid, acantholysis, eiosion, oi
ulceiation.
0IACN0SIS
Clinical findings confiimed by Giam stain oi
cultuie.
FICk 24-12 Impeto:
methc||o-resstaot 5. oureus
(MkSA) A 22,e+|o|d d|+|e||c
r+|e |e|u ||e+|ed W||| |o||e||uo|u
|o| +cue W||| e|,||er+ o| ||p +ud
|oWe| |+ce W||| c|u|ed e|o|ou.
'|+.|u |+ de|||ded c|u|. B|ood
|ucoe W+ 500 r/d| +| ||e ||re
o| e\+r|u+||ou. C|e||||| | uu|.e|+|
du||u |o||e||uo|u ||e+|reu|, +ud '
c- ecoud+|, |u|ec||ou, corrou.
FICk 24-13 Secoodary mpetotatoo oI raIted buro ste: MSSA E\|eu|.e e|,||er+ +ud c|u|
|u +|e eeu ou |,pe|||op||c c+| +ud uo|r+| ||u o| + 0,e+|o|d |o, W||| p||o| e.e|e ||e|r+| |u|u.
FICk 24-14 Secoodary mpetotatoo oI Iactta| u|cers: MkSA o0,e+|o|d |er+|e W||| eud|+e|eu+|
d|e+e (E'k|) +ud eue|+||/ed p|u|||u |+ |+|e e|||uduced e\co||+||ou +ud u|ce|, W||c| +|e ecoud+|||, |u|ec|ed.
FICk 24-15 8u||ous mpeto: MSSA 'ec
oud+|, |u|ec||ou o| +|op|c de|r+|||| |u ||e +u|ecu|||+|
+|e+. Bu||+e |+.e |up|u|ed, |o|r|u c|u|ed e|o|ou
(+re p+||eu| + |u ||. 2+0).
C0kS AN0 Fk0CN0SIS
Untieated, lesions of impetigo piogiess foi
seveial weeks. Untieated oi neglected impetigo
can piogiess to ecthyma. With adequate tieat-
ment, piompt iesolution. Lesions can piogiess
to invasive infection with lymphangitis, sup-
puiative lymphadenitis, cellulitis oi eiysipelas,
bacteiemia, septicemia. Nonsuppuiative com-
plications of GAS infection include guttate
psoiiasis, scailet fevei, and glomeiulonephii-
tis. Ecthyma often heals with scai. Recuiient
FICk 24-16 cthyma: MSSA I||c||, c|u|ed
e|o|ou/u|ce| ou ||e uoe |+d |eeu p|eeu| |o| o
Wee|, +|||u +| ||e ||e o| + r+|| Wouud. I|e c|u|
W+ +d|e|eu| +ud ||e ||e ||ed W||| de|||dereu|.
S. aureus oi GAS infections can occui because
of failuie to eiadicate miciobe oi by iecoloniza-
tion fiom a family membei, pet, oi health caie
woikei. Undiagnosed MRSA infection does not
iespond to usual antibiotics given foi methicil-
lin-sensitive S. aureus (MSSA). MRSA infection
has highei moibidity and moitality.
MANACMNI
Freveotoo Daily bath. Benzoyl peioxide
wash (bai). Check family membeis foi signs of
impetigo. Ethanol oi isopiopyl gel foi hands
and/oi involved sites.
Iopca| Ireatmeot Mupiiocin and ietapamulin
ointment is highly effective in eliminating both
S. aureus , including MRSA, fiom the naies and
cutaneous lesions. Apply twice daily to involved
skin and to naies foi 7-10 days.
Systemc Aotmcroba| Ireatmeot See Tables
24-1, 24-2. Foi MRSA, sensitivities of the iso-
lated oiganism and peisonal histoiy of anti-
biotic alleigies deteimine diug of choice and
alteinatives.
IA8I 24-1 0ranisms, Antimicrobia| Aents of Choice, and A|ternatives
|oIect|og 0rgao|sm Aot|m|crob|a| Ageot(s) oI F|rst 0ho|ce A|teroat|ve Aot|m|crob|a| Ageots
5tophylececcus oureus or |eu|c||||u C o| \ A cep|+|opo||u,
epdermds c||ud+r,c|u, .+ucor,c|u,
|oupeu|c||||u+e |r|peuer, + ||uo|oqu|uo|oue
p|oduc|u
|eu|c||||u+e A peu|c||||u+e|e||+u| A cep|+|opo||u,
p|oduc|u
peu|c||||u. |0. d|c|o\+c||||u, .+ucor,c|u,
c|o\+c||||u. |\ |o| e.e|e +ro\|c||||u/c|+.u|+u|c +c|d,
|u|ec||ou, u+|c||||u, o\+c||||u ||c+|c||||u/c|+.u|+u|c +c|d,
p|pe|+c||||u/|+/o|+c|+r,
+rp|c||||u/u||+c|+r, |r|peuer,
c||ud+r,c|u, + ||uo|oqu|uo|oue
\e|||c||||u|e||+u| \+ucor,c|u eu|+r|c|u |||+rp|u I||re||op||ru||+re||o\+/o|e, +
||uo|oqu|uo|oue, r|uoc,c||ue,
||ue/o||d, qu|uup||||u/d+||op||||u
Jtreptererrar pyegeaer |eu|c||||u C o| \ Au e|,|||or,c|u, c|+|||||or,c|u,
(roup A) aod roups C +/||||or,c|u, + cep|+|opo||u,
aod C .+ucor,c|u, c||ud+r,c|u
5treptececcus, roup 8 |eu|c||||u C o| +rp|c||||u A cep|+|opo||u, .+ucor,c|u, +u
e|,|||or,c|u
5treptececcus |eu|c||||u C o| \ A cep|+|opo||u e|,|||or,c|u,
poeumeooe +/||||or,c|u, c|+|||||or,c|u,
(poeumococcus) + ||uo|oqu|uo|oue, re|opeuer,
|r|peuer, |||re||op||r
u||+re||o\+/o|e, c||ud+r,c|u, +
|e||+c,c||ue
|eu|c||||uucep||||e |eu|c||||u C |\ (2 r||||ou u/d |e.o||o\+c|u, .+ucor,c|u,
(\|C 0. /r|) |o| +du||) ce||||+\oue ce|o|+\|re c||ud+r,c|u
|eu|c||||u-|u|e|red|+|e \eu|u|||. .+ucor,c|u \e|opeuer, |r|peuer,
|e||+uce ce||||+\oue ce|o|+\|re |||+rp|u c||ud+r,c|u
|eu|c||||u-||||e.e| 0||e| |u|ec||ou. .+||cor,c|u 0u|uup||||u/d+||op||||u, ||ue/o||d
|e||+uce (\|C 2 /r|) ce||||+\oue ce|o|+\|re,
|e.o||o\+c|u
|oIect|og 0rgao|sm Aot|m|crob|a| Ageot(s) oI F|rst 0ho|ce A|teroat|ve Aot|m|crob|a| Ageots
Fryspelethrx rhusepothoe |eu|c||||u C E|,|||or,c|u, + cep|+|opo||u, +
||uo|oqu|uo|oue
Boemephlus ollueozoe Ce|o|+\|re ce||||+\oue Ce|u|o\|re (uo| |o| reu|u|||),
c||o|+rp|eu|co|, re|opeuer
\eu|u|||, ep||o|||||,
+|||||||, +ud o||e|
e||ou |u|ec||ou
uppe| |ep||+|o|, I||re||op||ru||+re||o\+/o|e Ce|u|o\|re, +ro\|c||||u/c|+.u|+u|c
|u|ec||ou +ud +c|d, ce|u|o\|re +\e|||,
||ouc|||| ce|podo\|re, ce|+c|o|,
ce|o|+\|re, ce|||/o\|re,
ce||||+\oue, ce||\|re, +
|e||+c,c||ue, c|+|||||or,c|u,
+/||||or,c|u, + ||uo|oqu|uo|oue,
+rp|c||||u o| +ro\|c||||u
Posteurello multecdo |eu|c||||u C A |e||+c,c||ue, + cep|+|opo||u,
+ro\|c||||u/c|+.u|+u|c +c|d,
+rp|c||||u/u||+c|+r
Pseudemeoos oeruyoeso C|p|o||o\+c|u, ||c+|c||||u, re/|oc||||u C+||eu|c||||u, ||c+|c||||u, p|pe|+c||||u
p|pe|+c||||u |o||+r,c|u, eu|+r|c|u re/|oc||||u, ce||+/|d|re,
+r||+c|u ce|ep|re, |r|peuer
re|opeuer, +/||eou+r,
|o||+r,c|u, eu|+r|c|u, +r||+c|u
vbre vulolcus A |e||+c,c||ue Ce|o|+\|re
Nessero yeoerrheeoe Ce||||+\oue ce||\|re c|p|o||o\+c|u Ce|o|+\|re, pec||uor,c|u,
(ooococcus) o||o\+c|u peu|c||||u C, ce|||/o\|re,
ce||||+\oue
Nessero meooytds |eu|c||||u C C||o|+rp|eu|co|, + u||ou+r|de, +
(meooococcus) ||uo|oqu|uo|oue
Mycebocterum tuberculess |ou|+/|d |||+rp|u p,|+/|u+r|de |e.o||o\+c|u, o||o\+c|u
e||+r|u|o| ||ep|or,c|u c|p|o||o\+c|u, c,c|oe||ue,
c+p|eor,c|u |+u+r,c|u
+r||+c|u, e|||ou+r|de,
c|o|+/|r|ue, +r|uo+||c,||c +c|d
Mycebocterum lertutum/ Ar||+c|u c|+|||||or,c|u Ce|o\|||u, |||+rp|u, + u||ou+r|de,
cheleooe comp|ex do\,c,c||ue, e||+r|u|o|
Mycebocterum moroum \|uoc,c||ue I||re||op||ru||+re||o\+/o|e,
(boloe} |||+rp|u, c|+|||||or,c|u,
do\,c,c||ue
Mycebocterum leproe |+poue |||+rp|u c|o|+/|r|ue \|uoc,c||ue, o||o\+c|u, p+|||o\+c|u,
(|eprosy) c|+|||||or,c|u
ctoemyces sroel |eu|c||||u C A |e||+c,c||ue, e|,|||or,c|u,
(actoomycoss) c||ud+r,c|u
Necordo I||re||op||r 'u||+o\+/o|e, +r||+c|u, +
u||+re||o\+/o|e |e||+c,c||ue, |r|peuer
re|opeuer, c,c|oe||ue
IA8I 24-2 0ra| Antimicrobia| Aents for Bacteria| |nfections
Aot|m|crob|a| Ageot 0os|og (P0 0o|ess |od|cated), 0s0a||y For 7-14 0ays
Natura| peoc||os
|eu|c||||u \ 250-500 r ! o| + ||re + d+, |o| 0 d+,
|eu|c||||u C o00,000-.2 r||||ou u |\ d+||, |o| 1 d+,
Beu/+|||ue peu|c||||u C o00,000 u |\ |u c|||deu o ,e+|, .2 r||||ou uu|| || 1
,e+|, || corp||+uce | + p|o||er
Feoc||oase-resstaot peoc||os
C|o\+c||||u 250-500 r (+du||) + ||re + d+, 0 d+,
||c|o\+c||||u 250-500 r (+du||) + ||re + d+, |o| 0 d+,
|+|c||||u .0-2.0 |\ q+|
0\+c||||u .0-2.0 |\ q+|
Amoopeoc||os
Aro\|c||||u 500 r ! ||re + d+, o| 315 r q2|
Aro\|c||||u p|u c|+.u|+u|c +c|d 315/25 r |W|ce + d+,, 20 r/| pe| d+, ! ||re + d+, |o|
( |+c|+r+e |u|||||o|) 0 d+,
Arp|c||||u 250-500 r + ||re + d+, |o| 1-0 d+,
Cepha|osporos
Cep|+|e\|u 250-500 r (+du||) + ||re + d+, |o| 0 d+,, +0-50 r/
| pe| d+, (c|||d|eu) |o| 0 d+,
Cep||+d|ue 250-500 r (+du||) + ||re + d+, |o| 0 d+,,
+0-50 r/| pe| d+, (c|||d|eu) |o| 0 d+,
Ce|+c|o| 250-500 r q3|
Ce|p|o/|| 250-500 r q2|
Ce|u|o\|re +\e||| 25-500 r q2|
Ce||\|re 200-+00 r q2-2+|
rythromyco roup
E|,|||or,c|u e||,|ucc|u+|e 250-500 r (+du||) + ||re + d+, |o| 0 d+,, +0 r/|
pe| d+, (c|||d|eu) + ||re + d+, |o| 0 d+,
C|+|||||or,c|u 500 r |W|ce + d+, |o| 0 d+,
A/||||or,c|u 500 r ou d+, , ||eu 250 r/d ou d+, 2-5
C|odamyco 50-!00 r (+du||) + ||re + d+, |o| 0 d+,, 5 r/|
pe| d+, (c|||d|eu) + ||re + d+, |o| 0 d+,
Ietracy|oes
\|uoc,c||ue 00 r |W|ce + d+, |o| 0 d+,
|o\,c,c||ue 00 r |W|ce + d+,
Ie||+c,c||ue 250-500 r + ||re + d+,
Msce||aoeous aeots
I||re||op||ru||+re||o\+/o|e o0 r I\| 300 r '\/ |W|ce + d+,
\e||ou|d+/o|e 500 r + ||re + d+,
C|p|o||o\+c|u 500 r |W|ce + d+, |o| 1 d+,
1|:- +cu|e o| c||ou|c |oc+||/ed |u||+rr+||ou,
+oc|+|ed W||| + co||ec||ou o| pu +ud ||ue
de||uc||ou.
|||:|| (|+p|,|ococc+|). |u|ec||ou o| |+|| |o|
||c|e W||| pu |u ||e o||ur o| |o|||c|e (ee
'ec||ou !2)
|:|- +cu|e, deepe+|ed, |ed, |o|, |eude|
uodu|e o| +|ce ||+| e.o|.e ||or + |+p|,|ococ
c+| |o|||cu||||.
Cc|:|- . deepe| |u|ec||ou corpoed o| |u|e|
couuec||u +|cee uu+||, +|||u |u e.e|+|
cou||uou |+|| |o|||c|e.
',, Bo||
A8SCSS, FkNCI, AN0 CAk8NCI |C|9 . o30.9/o32.9
|C|0 . |02
Ae oI 0oset Childien, adolescents, and young
adults.
Sex Moie common in boys.
to|oy MSSA, MRSA. Much less commonly,
othei oiganisms. Steiile abscess can occui as
a foieign-body iesponse (splintei, iuptuied
inclusion cyst, injection sites). Cutaneous odon-
togenic sinus can appeai anywheie on the lowei
face, even at sites distant fiom the oiigin (see
Fig. 34-16).
Fredsposo Factors
Chionic S. aureus caiiiei state (naies, axillae,
peiineum, vagina)
Diabetes mellitus
Obesity
Pooi hygiene
Bacteiicidal defects (e.g., chionic gianuloma-
tous disease)
Chemotactic defects
Hypei-IgE syndiome (Job syndiome)
HIV/AIDS, especially MRSA infection
FAIh0CNSIS
Folliculitis, fuiuncles, and caibuncles iepiesent
a continuum of seveiity of S. aureus infec-
tion. Poital of entiy: haii follicle, bieak in the
integiity of skin. MRSA infections often have
high moibidity due to delay in administiation
of effective antibiotic. Contiol/eiadication of
caiiiei state tieats/pievents folliculitis, fuiuncle,
and caibuncle foimation.
0uratoo oI Iesoos Days to weeks to months.
Sko Symptoms Thiobbing pain. Tendeiness.
Coosttutooa| Symptoms Caibuncles may be
accompanied by low-giade fevei and malaise.
Sko Iesoos Lesions aie ied, hot, and pain-
ful/tendei.
Ahscess May aiise in any oigan oi stiuctuie.
Abscesses that piesent on the skin aiise in the
deimis, subcutaneous fat, muscle, oi a vaiiety of
deepei stiuctuies. Initially, a tendei ied nodule
foims. In time (days to weeks), pus collects within
a cential space (Fig. 24-17). A well-foimed abscess
is chaiacteiized by fluctuance of the cential poi-
tion of the lesion and can occui at any cutaneous
site. At sites of tiauma. Uppei back foi abscesses in
iuptuied inclusion cysts. Single oi multiple.
Fv||Icu|ItIs (Stuphy|vcvccu|) See Infectious
Folliculitis," Section 32 and Figs. 32-27, 32-28,
and 32-31.
Furunc|e Initially, a fiim tendei nodule, up
to 1-2 cm in diametei (Fig. 24-18). In many
individuals, fuiuncles occui in setting of staphy-
lococcal folliculitis in beaid aiea oi neck. Nodule
becomes fluctuant, with abscess foimation
cential pustule. Nodule with cavitation iemains
aftei diainage of abscess. A vaiiable zone of
cellulitis may suiiound the fuiuncle. Distii-
bution: any haii-beaiing iegion: beaid aiea, pos-
teiioi neck and occipital scalp, axillae, buttocks.
Single oi multiple (Figs. 24-19, 24-20, B).
Curhunc|e Evolution is similai to that of fuiun-
cle. Composed of seveial to multiple, adjacent,
coalescing fuiuncles (Fig. 24-21). Chaiacteiized
by multiple loculated deimal and subcutaneous
abscesses, supeificial pustules, neciotic plugs,
and sieve-like openings diaining pus.
FaoIu| 0erma|[Subcutaoeous Nodu|e Ruptuied
epideimoid oi pilai cyst, hidiadenitis suppuiativa
(axillae, gioin, vulva), neciotizing lymphangitis.
Cram Stao Giam-positive cocci within poly-
moiphonucleai (PMN) leukocytes.
8actera| Cu|ture Cultuie of pus isolates
S. aureus . Sensitivities to antimiciobial agents
may deteimine management.
AntIhIvtIc SensItIvItIes Identifies MRSA and
need foi changing usual antibiotic theiapy.
0ermatopatho|oy Pyogenic infection aiising
in haii follicle and extending into deep deimis
and subcutaneous tissue (fuiuncle) and with
loculated abscesses (caibuncle).
0IACN0SIS
Clinical findings confiimed by findings on
Giam stain and cultuie.
FICk 24-1T Abscess: MSSA A .e|, |eude| +|ce W||| u||ouud|u e|,||er+ ou ||e |ee|. I|e p+||eu|
W+ + d|+|e||c W||| euo|, ueu|op+||,, + eW|u ueed|e |u ||e |ee| |+d p|o.|ded + po||+| o| eu||,.
FICk 24-18 Fo||cu|ts aod
Furuocu|oss: MkSA \u|||p|e
|o|||cu|+| p+pu|e, pu|u|e, +ud |+|e
uodu|e ou ||e red|+| |||| |u + +
,e+|o|d r+|e W||| n|\/A||'.
FICk 24-19 Furuoc|e o hIv[AI0S:
MkSA |+|e ||uc|u+u| +|ce ou ||e |u||oc| o|
+ r+|e W||| n|\/A||'. ne W+ |u|||+||, erp|||c+||,
||e+|ed W|||ou| cu||u|e. I|e +|ce W+ d|+|ued,
+ud \''A |o|+|ed. 'eu|||.|||e |epo||ed |e||+uce
|o +|| o|+||, +dr|u||e|ed +u||||o||c e\cep| |o| ||ue
/o||d. I|e |e|ou |eo|.ed W||| ||ue/o||d, o00 r
||d |o| 0 d+,.
FICk 24-20 Mu|tp|e Iuruoc|es aod ce||u|ts: MkSA A o+,e+|o|d r+|e de.e|oped ru|||p|e |u|uuc|e
ou ||e do|ur o| ||e |e|| |+ud () +ud |o|e+|r (8). ne W+ |e|u ||e+|ed W||| |erod|+|,| |o| eud|+e |eu+|
d|e+e (E'k|) +oc|+|ed W||| A|po|| ,ud|ore. ' c- co|ou|/+||ou o| ||e u+|e | corrou |u pe|ou |e|u
d|+|,/ed.
8
C0kS AN0 Fk0CN0SIS
Most cases iesolve with tieatment (see below).
At times, howevei, fuiunculosis is complicated
by bacteiemia and possible hematogenous seed-
ing of heait valves, joints, spine, long bones,
and visceia (especially kidneys). S. aureus can
disseminate hematogenously via venous diain-
age to caveinous sinus with iesultant caveinous
venous thiomboses and meningitis. Some indi-
viduals aie subject to iecuiient fuiunculosis,
paiticulaily diabetics.
MANACMNI
The tieatment of an abscess, fuiuncle, oi cai-
buncle is incision and diainage plus systemic
antimiciobial theiapy.
Freveotoo Mupiiocin ointment is effective
foi eliminating nasal caiiiage.
Surery Incision and diainage aie often
adequate foi tieatment of abscesses, fuiuncles, oi
caibuncles. Scissois oi scalpel blade can be used
to diain loculated pus in caibuncles; if this is not
done, iesolution of pain and infection can be
delayed despite systemic antibiotic theiapy. Dental
abscesses aie often associated with devitalized
tooth pulp, which must be iemoved oi the tooth
extiacted. All foieign mattei must be iemoved:
comedone, keiatinaceous debiis, foieign body.
Adjuoctve Iherapy Application of heat to the
lesion piomotes localization/consolidation and
aids eaily spontaneous diainage.
Systemc Aotmcroba| Ireatmeot Systemic
antibiotics speed iesolution in healthy indi-
viduals and aie mandatoiy in any individual
at iisk foi bacteiemia (e.g., immunosuppiessed
patients). See Table 24-2.
kecurreot Furuocu|oss Usually ielated to
peisistent S. aureus in the naies, peiineum, and
body folds.
TvpIcu| Therupy Showei with piovidone io-
dine soap oi benzoyl peioxide (bai oi wash).
Apply mupiiocin ointment daily to the inside
of naies and othei sites of S. aureus caiiiage.
Retapamulin ointment applied on infected sites
is also effective.
SystemIc Therupy Appiopiiate antibiotic
tieatment is continued until all lesions have
iesolved. Secondaiy piophylaxis may be given
once a day foi many months.
CurrIer Stute Rifampin: 600 mg PO foi 7-10
days foi eiadication of caiiiei state.
FICk 24-21 Carbuoc|e: MSSA
A .e|, |+|e, |u||+rr+|o|, p|+que |udded
W||| pu|u|e, d|+|u|u pu, ou ||e u+pe
o| ||e uec|. |u|ec||ou e\|eud doWu |o ||e
|+c|+ +ud |+ |o|red ||or + cou||ueuce o|
r+u, |u|uuc|e.
C|+|+c|e||/ed |, +u +cu|e, d|||ue, p|e+d|u,
eder+|ou, uppu|+||.e |u||+rr+||ou o| ||e de|
r| +ud u|cu|+ueou ||ue
0||eu +oc|+|ed W||| ,|er|c ,rp|or o|
r+|+|e, |e.e|, +ud c||||
|ouuec|o||/|u 'I| +|e ||e+|ed W||| +u||||o||c,
d|+|u+e o| +|cee, +ud uppo|||.e re+u|e
|ec|o||/|u 'I| +|e o||eu |||e|||e+|eu|u +ud
|equ||e, |u +dd|||ou, e\|eu|.e u||c+| de|||d e
reu|.
S0FI IISS INFCII0NS (SIIS)
CIASSIFICAII0N [0FINIII0NS
ryspe|as A distinct type of supeificial cuta-
neous cellulitis with maiked deimal lymphatic
vessel involvement piesenting as a painful, biight
ied, iaised, edematous, induiated plaque with
advancing iaised boideis, shaiply maiginated
fiom the suiiounding noimal skin (Figs. 24-22 to
24-24). Usually caused by gioup A -hemolytic
stieptococcus (GAS) (veiy uncommonly gioup C
oi G stieptococcus) and iaiely due to S. aureus .
Ce||u|ts Has many of the featuies of eiysip-
elas but extends into the subcutaneous tissues.
Cellulitis is diffeientiated fiom eiysipelas by
two physical findings: cellulitis lesions aie pii-
maiily not iaised, and demaication fiom un-
involved skin is indistinct. The tissue feels haid
on palpation and is extiemely painful. In some
cases, bulla foimation (see Fig. 24-25) oi necio-
sis. Infection may localize in the soft tissue, with
deimal and subcutaneous abscess foimation
(see Fig. 24-26) oi neciotizing fasciitis. S. aureus
and GAS aie by fai the most common etiologic
agents, but othei bacteiia aie implicated.
Iymphaots Inflammation of the lymphatic
vessels, usually beginning on acial sites such as
hands oi feet; piesents as eiythematous stieak-
ing on the volai oi doisal aspect of the aim
pioximal to a fingei oi hand infection (Figs.
24-27 and 24-28).
Caoreoous Ce||u|ts Chaiacteiized by necio-
sis of the deimis, subcutaneous fat (hypodeimis),
fascia, oi muscle. Classified as netro:ng [ast-
s , t|osrJa| so[ ssue n[etons , and rogres-
se |atera| synergst gangrene .
Necrotto SoIt Issue IoIectoo (NSII) Diffeis
fiom othei vaiiants because of significant tissue
neciosis, lack of iesponse to antimiciobial tieat-
ment alone, and need foi suigical debiidement
of devitalized tissues. Staits with eiythema
and painful induiation of undeilying soft tis-
sues; iapid development of black eschai, which
tiansfoims into liquefied black and malodoious
neciotic mass (see Fig. 24-31). Divided into
thiee categoiies: netro:ng te||u|s, netro:ng
[asts, myonetross . NSTI in the genital aiea is
called Fourner gangrene .
cthyma Caoreoosum An NSTI, most com-
monly caused by P. aerugnosa , chaiactei-
ized by a cutaneous infaiction piogiessing
to laige ulceiated gangienous lesions (see
Fig. 24-29).
Acu|e, p|e+d|u |u|ec||ou o| de|r+| +ud u|cu
|+ueou ||ue
C|+|+c|e||/ed |, + |ed, |o|, |eude| +|e+ o| ||u
0||eu o|||u+||u +| ||e ||e o| |+c|e||+| eu||,
C+ued ro| ||equeu||, |, CA' (e|,|pe|+) o|
' c- .
kSIFIAS AN0 CIIIIIIS |C|9 . 0!5
|C|0 . A+o.0
Ae oI 0oset Any age. Childien <3 yeais;
oldei individuals.
to|oy Adults: S. aureus , GAS. Childien:
H. n[|uen:ae type b (Hib), GAS, S. aureus
(Table 24-3).
Less Cvmmvn|y Gioup B stieptococci (GBS),
pneumococci, E. r|usoa|ae (eiysipeloid).
In patients with diabetes oi impaiied immu-
nity: E. to|, Proeus mra||s, tneo|ater,
Enero|ater, P. aerugnosa, Paseure||a mu|ot-
Ja, V|ro u|n[tus; Myto|aterum [oruum
complex, C. neo[ormans. In childien: pneumo-
cocci, N. menngJs gioup B (peiioibital).
IA8I 24-3 Etio|oy of Soft Iissue |nfections (SI|s)
Type oI |oIect|oo Nost 0ommoo 0a0se(s) 0ocommoo 0a0ses
ryspe|as C|oup A ||ep|ococcu (CA') C|oup B, C, +ud C ||ep|ococc| (CB', CC',
CC') ' c-
Ce||u|ts ' c-, CA' CB', CC', CC'
|,-||| |c||c-
|ueurococcu
|c-|| ||-cc- (c|||d|eu)
|:|-:|c :|
Cc,||c:|- -
!c-||c
'-c|c ||-, o||e| Eu|e|o|+c|e||+ce+e
C,|::: -|c
|--||c -||c | :!c!-
3c:|| c||c: (+u|||+\)
1-c |,!||c
l| .||: l c||,|:
Ce||u|ts o ch|dreo ' c-, CA' CB' (ueou+|e)
|+c|+|/pe||o||||+| ce||u|||| | ||-cc- (,ouu c|||d|eu) |--c -|!
|e||+u+| ce||u|||| CA' ' c-
Ce||u|ts secoodary to | c-c l .||:
bacterema '|-|::: -c- CA', CB'
Creptaot ce||u|ts C||!c pp (C -|- 3c:|-!- pp.
C -|:) |ep|o||ep|ococc|
| :| ||-|-||c
Ce||u|ts assocated wth | |c||c- (e|,|pe|o|d) 'e+| ||ue| (e||o|o, uu|uoWu)
water exposure
l .||:
1-c |,!||c
!,:|c:|- c
(uodu|+| |,rp|+u|||)
! ||| corp|e\
Caoreoous ce||u|ts
(|u|ec||ou +u|eue)
|ec|o||/|u |+c||||
(||) '||ep|ococc+|
+u|eue CA' CB', CC', CC'
|ou||ep|ococc+| || \|\ed |u|ec||ou W||| oue o| 3c:|| :-- (+|+uu|oc,||c p+||eu|)
ro|e +u+e|o|e
(|-||-|:::
o| 3c:|-!-) p|u +|
|e+| oue |+cu||+||.e
pec|e (uou|oup
A ||ep|ococc|, rer|e|
o| ||e Eu|e|o|+c|e||+ce+e
uc| + ||-|c:|-
o| ||-)
OppvrtunIstIc Puthvgens He|to|ater tnaeJ
(HIV/AIDS); C. neo[ormans; Fusarum, Proeus,
PseuJomonas spp.
Dvg und Cut BItes P. mu|otJa and othei
Paseure||a spp.; S. aureus, Canotyo|aga
tanmorsus (DF-2)
humao 8tes Most common in young males.
Most bites occui on the hands: clenched-fist oi
occlusional injuiies. Multiple oiganisms often
isolated fiom wound site, including Sreotot-
tus angnosus, S. aureus, E. torroJens, Fuso|ate-
rum nut|eaum , and Preoe||a me|annogenta .
Fuso|aterum, Peosreotottus , and CanJJa
spp. moie fiequently fiom occlusional bites
than fiom clenched-fist injuiies.
Forta|s[Source oI IoIectoo Mucocutaneous,
subjacent, bacteiemic. (See Table 24-4.)
Mucvcutunevus
UnJer|yng Jermaoses : Bullous disease
(pemphigus pemphigoid, sunbuin); chionic
lymphedema; deimatophytosis (epideimal
deimatophytosis/ tinea pedis, tinea capi-
tis, tinea baibae); viial infections (heipes
simplex, vaiicella, heipes zostei); inflamma-
toiy deimatoses (atopic deimatitis, contact
deimatitis, stasis deimatitis, pyodeima gan-
gienosum); supeificial pyodeima (impetigo,
folliculitis, fuiunculosis, caibuncle, ecthyma);
ulceis (piessuie, chionic venous insufficiency,
ischemic, neuiopathic); umbilical stump
Trauma : Abiasion; bites (human, animal);
insect bites; buins; laceiation; punctuie
Surgta| wounJ : Suigical incisions; PIC lines
Mutosa| n[eton : Oiophaiynx, nasal mucosa;
middle eai
In,etng Jrug use (IDU) : Skin popping" sites
Watei exposuie: V. u|n[tus, V. t|o|erae non-
01 and non-0139. eromonas |yJro||a .
Suhucent Osteomyelitis, cutaneous odon-
togenic sinus, abdominal infection.
BucteremIc Sepsis, infectious endocaiditis. S.
neumonae, V. u|n[tus, and C. neo[ormans.
ksk Factors Diug and alcohol abuse, cancei
and cancei chemotheiapy, chionic lymphedema
(postmastectomy, postcoionaiy aiteiy giafting,
pievious episode of cellulitis/eiysipelas), cii-
ihosis, diabetes mellitus, nephiitic syndiome,
iatiogenic immunosuppiession, neutiopenia,
immunodeficiency syndiomes, malnutiition,
ienal failuie, systemic atheioscleiosis.
FAIh0CNSIS
Aftei entiy, infection spieads to tissue spaces
and cleavage planes as hyaluionidases bieak
down polysacchaiide giound substances, fi-
biinolysins digest fibiin baiiieis, lecithinases
destioy cell membianes. Local tissue devitaliza-
tion, e.g., tiauma, is usually iequiied to allow
foi significant anaeiobic bacteiial infection.
The numbei of infecting oiganisms is usually
small, suggesting that cellulitis may be moie of
a ieaction to cytokines and bacteiial supeianti-
gens than to oveiwhelming tissue infection.
Iocubatoo Ferod Few days.
Frodrome Malaise, anoiexia; fevei, chills can
develop iapidly, befoie cellulitis is appaient
clinically. Highei fevei (38.5 C) and chills usu-
ally associated with GAS.
Immuoe Status Immunocompiomised pa-
tients susceptible to infection with pathogens
of low pathogenicity.
hstory Local pain and tendeiness. Neciotiz-
ing infections associated with moie local pain
and systemic symptoms.
Sko Iesoos
Forta|s oI otry Red, hot, edematous and
shiny plaque, and veiy tendei aiea of skin
of vaiying size (Figs. 24-22, 24-24); boideis
usually shaiply defined, iiiegulai, and slightly
elevated; bluish puiple coloi with H. n[|uen:ae
(Fig. 24-23). Vesicles, bullae, eiosions, abscesses,
hemoiihage, and neciosis may foim in plaque.
Lymphangitis. Lym| noJes : Can be enlaiged
and tendei, iegionally.
0strbutoo Adu|ts Lowei leg (Fig. 24-24);
most common site, following inteidigital tinea
(Fig. 24-25). Aim: in young male, considei IV
diug use; in female, postmastectomy (Fig. 24-27).
Tiunk: opeiative wound site. Face: following
ihinitis, conjunctivitis.
ChI|dren Cheek, peiioibital aiea, head, neck
most common: usually H. n[|uen:ae . Extiemi-
ties: S. aureus , GAS.
varaots o IoIecto 0raosm
5. oureus Often a poital of entiy is appaient;
usually a focal infection. Most common patho-
gen in injection diug usei. Toxin syndiomes
scalded-skin syndiome, toxic shock syndiome
(TSS)] may occui. Endocaiditis may follow
bacteiemia.
Croup A Streptococcus Incidence of invasive
GAS infections is incieasing. The moibidity and
moitality iates aie significant: 37% of patients
have neciotizing fasciitis and 25% meet the
ciiteiia foi stieptococcal TSS; moitality iate
iepoited to be 21%.
F. rhusepothoe : ryspe|od Painful, swollen
plaque with shaiply defined iiiegulai iaised
boidei occuiiing at the site of inoculation,
i.e., fingei oi hand (Fig. 24-28), spieading to
wiist and foieaim. Coloi: puiplish ied acutely;
biownish with iesolution. Enlaiges peiipheially
with cential fading. Usually no systemic symp-
toms. Uncommonly, associated with bacteiemia
and aoitic valvulitis. Occuis in individuals who
handle game, poultiy, fish.
P. oeruyoeso (See Cutaneous P. aerugnosa
infections") Ecthyma gangienosum begins as
FICk 24-23 Ce||u|ts oI cheek:
B. ollueozoe E|,||er+ +ud eder+
o| ||e c|ee| o| + ,ouu c|||d, +oc|+|ed
W||| |e.e| +ud r+|+|e. | ||-cc- W+
|o|+|ed ou cu||u|e o| ||e u+op|+|,u\.
(Cou||e, o| '+ud, I+o, \|.)
FICk 24-22 ryspe|as oI Iace: roup A streptococcus |+|u|u|, We||de||ued, ||u,, e|,||er+|ou,
eder+|ou p|+que |u.o|.|u ||e ceu||+| |+ce o| +u o||e|W|e |e+|||, r+|e. 0u p+|p+||ou ||e ||u | |o| +ud |eude|.
Croup 8 Streptococcus (S. ugu|uctIue)
Colonizes anogenital iegion. Causes anogenital
cellulitis, which may extend into pelvic tissues.
Following childbiith, known as uerera| sess .
Cellulitis occuis in neonates; high moibidity
and moitality.
5. poeumeooe (Foeumococcus) Occuis moie
commonly in individuals with systemic lupus
eiythematosus, complement deficiency, HIV/
AIDS, glucocoiticoid theiapy, diug oi alcohol
abuse. Infected sites show bulla foimation,
biawny eiythema, violaceous hue.
FICk 24-24 ryspe|as oI |e: MSSA I|e
|oWe| |e | |ed, |o|, |eude|, +ud eder+|ou. E|,
||er+|ou p|+que | We|| de||ued (||ue r+||). I|e
|u|ec||ou | |ecu||eu| W||| |u|e|d|||+| ||ue+ ped| +
||e po||+| o| eu||,.
FICk 24-25 kecurreot ce||u|ts oI Ioot:
MSSA |u|e|d|||+| ||ue+ ped| W+ ||e po||+| o|
eu||, |o| ||| |ecu||eu| ce||u||||, |u ||e e|||u o|
c||ou|c eder+. I|e |oo| | eder+|ou, e|,||er+|ou
W||| |u||+ |o|r+||ou.
FICk 24-26 Ce||u|ts oI the arm: MSSA E|o|ou ou ||e e||oW |u + p|+que o| po||+| W+ ||e po||+|
o| eu||, |o| |u|ec||ou. we||der+|c+|ed e|,||er+, eder+, +ud +|ce |o|r+||ou +|e eeu.
eiythematous macule (cutaneous ischemic le-
sion) that quickly evolves to a bluish oi gun-
metal giay plaque with an eiythematous halo
(infaiction) (Fig. 24-29, B). The epideimis
oveilying the ischemic aiea foims a bulla. Epi-
deimis eventually sloughs, foiming an ulcei.
Dsr|uon. most commonly in the axilla,
gioin, peiineum. Usually occuis as solitaiy le-
sion but may occui as a few lesions. Lesions
associated with PseuJomonas septicemia: iose
spotlike lesions (eiythematous macules and/oi
papules on tiunk as in typhoid fevei, occui with
PseuJomonas infection of GI tiact, i.e., diaiihea,
headache, high fevei); painful clusteied vesicu-
lai to bullous lesions; multiple painful nodules
iepiesenting small embolic lesions.
B. ollueozoe Occuis mainly in childien
<2 yeais. Cheek, peiioibital aiea, head, neck most
common sites (Fig. 24-23). Clinically, swelling,
chaiacteiistic violaceous eiythema hue. Use of
Hib vaccine has diamatically ieduced incidence.
v. vulolcus, v. cheleroe ooo-01 aod ooo-0139
Undeilying disoideis: ciiihosis, diabetes, immu-
nosuppiession, hemochiomatosis, thalassemia.
Follows ingestion of iaw/undeicooked seafood,
gastioenteiitis, bacteiemia with seeding of skin;
also exposuie of skin to sea watei. Chaiacteiized
by bulla foimation, neciotizing vasculitis (Fig.
24-30). Usually on the extiemities; often bilateial.
. hydrephlo Watei-associated tiauma;
pieexisting wound. Immunocompiomised
host. Lowei leg. Neciotizing STIs.
C. coomersus Immunosuppiession oi as-
plenia; exposuie to a dog.
P. multecdo Follows cat bite.
Clestrdum Spp. Associated with tiauma,
contamination by soil oi feces, malignant
intestinal tumoi. Infection may be chaiac-
teiized by gas (ciepitation on palpation),
maiked systemic toxicity.
M. cheleoe-M. lertutum Comp|ex Histoiy
of iecent suigeiy, injection, penetiating
wound. Low-giade cellulitis. Systemic find-
ings lacking.
C. oeelermoos Patient always immuno-
compiomised. Red, hot, tendei, edematous
FICk 24-2T kecurreot ce||u|ts oI arm
wth chrooc |ymphedema: MSSA k|||
||e+| c+uce| |+d |eeu ||e+|ed W||| r+|ec|or,
+ud |,rp| uode e\c||ou 0 ,e+| p|e.|ou|,.
|,rp|eder+ o| ||e |||| +|r |o||oWed. n+ud
de|r+|||| W+ ecoud+|||, |u|ec|ed W||| \''A. Ce|
|u|||| occu||ed |epe+|ed|, |u ||e e|||u o| c||ou|c
|,rp|eder+.
plaque on extiemity. Raiely multiple noncon-
tiguous sites.
Mucormycoss Usually occuiiing in individual
with uncontiolled diabetes.
Ceoera| Fodos
Fevei, signs of sepsis.
ryspe|as[Ce||u|ts Deep vein thiombophle-
bitis, stasis deimatitis, eaily contact deimatitis,
giant uiticaiia, insect bite (hypeisensitivity ie-
sponse), fixed diug eiuption, eiythema no-
dosum, acute gout, eiythema migians (Lyme
boiieliosis), pievesiculai heipes zostei, Wells
syndiome (eosinophilic cellulitis), familial
Mediteiianean fevei-associated cellulitis-
like eiythema, cutaneous anthiax, pyodeima
gangienosum, Sweet syndiome (acute febiile
neutiophilic deimatosis), Kawasaki disease, cai-
cinoma eiysipeloides.
Necrotto SIIs Vasculitis, embolism with in-
faiction of skin, peiipheial vasculai disease,
puipuia fulminans, calciphylaxis, waifaiin
neciosis, tiaumatic injuiy, ciyoglobulinemia,
fixed diug eiuption, pyodeima gangienosum,
biown iecluse spidei bite.
FICk 24-28 ryspe|od oI haod A We||
der+|c+|ed, .|o|+ceou, ce||u||||c p|+que (W|||ou| ep|
de|r+| c|+ue o| c+|e o| .e|cu|+||ou) ou ||e do|+
o| ||e |+ud +ud ||ue|, occu||ed |o||oW|u c|e+u|u
|||, ||e ||e W+ oreW|+| p+|u|u|, |eude|, +ud W+|r.
0rect Mcroscopy Smeurs Giam stain of
exudate, pus, bulla fluid, aspiiate, oi touch
piepaiation may show bacteiia. GAS: chains of
giam-positive cocci. S. aureus. clusteis of giam-
positive cocci. Clostiidia: giam-negative iods,
few neutiophils.
"Tvuch" PrepurutIvn Lesional skin biopsy
specimen touched to micioscope slide. Potassium
hydioxide applied; examined foi yeast and myc-
elial foims of fungus; detects CanJJa, Cryo-
tottus, Mutor. Giam stain: detects bacteiia.
Cu|tures Ce||u|s. aspiiate oi biopsy of lead-
ing edge of inflammation, identifies pathogen
in up to 20% of cases. Fungal and mycobacte-
iial cultuies indicated in atypical case. Pora| o[
enry (ulceis, etc.), adjacent to cellulitis: similai
iesult to cultuie of cellulitis. B|ooJ tu|ures.
yield veiy low, 2-4%, highest in GAS infec-
tions. Yields highei in the setting of chionic
lymphedema and in patients with buccal oi
peiioibital cellulitis.
hemato|oy White blood count (WBC) and
eiythiocyte sedimentation iate (ESR) may be
elevated.
0ermatopatho|oy Fiozen sections of lesional
biopsies may be helpful in iuling out noninfec-
tious inflammatoiy deimatoses. Open suigi-
cal inspection with debiidement defines the
extent and seveiity of NF; tissue is obtained
foi histologic examination, Giam staining, and
FICk 24-29 cthyma aoreoosum oI but-
tock: P. oeruyoeso A !0,e+|o|d r+|e W|||
+d.+uced n|\/A||' +ud ueu||opeu|+. . Au e\||ere|,
p+|u|u|, |u|+|c|ed +|e+ W||| u||ouud|u e|,||er+ p|e
eu| |o| 5 d+,. I|| p||r+|, cu|+ueou |u|ec||ou W+
+oc|+|ed W||| |+c|e|er|+. 8. IWo Wee| |+|e|, ||e |e|ou |+d p|o|eed |o + |+|e u|ce|+||ou. I|e p+||eu|
d|ed ! rou|| |+|e| o| | c-c -| +oc|+|ed W||| c||ou|c ueu||opeu|+.
8
cultuie. In neciotizing STI: vasculitis without
thiombosis, paucity of neutiophils at site of
infection; bacilli found in media and adventitia,
but usually not in intima, of vessel. Helpful with
ciyptococcal cellulitis.
Imao MRI may be helpful in diagnosis
of seveie acute infectious cellulitis, identifying
pyomyositis, neciotizing fasciitis, and infectious
cellulitis with oi without subcutaneous abscess
foimation. Soft tissue iadiogiaphy, CT, MRI,
and ultiasonogiaphic imaging can detect local-
ized abscess, gas in tissue, and subjacent osteo-
myelitis but do not define NF oi myoneciosis.
0IACN0SIS
Clinical diagnosis based on moiphologic
featuies of lesion and the clinical setting tiavel
histoiy, animal exposuie, histoiy of bite, age,
undeilying disease(s)]. Confiimed by cultuie
in only 29% of cases in immunocompetent pa-
tients. Suspicion of NF iequiies immediate deep
biopsy and fiozen-section histopathology.
C0kS AN0 Fk0CN0SIS
When occuiiing as a local infection in the
absence of bacteiemia, piognosis is much
moie fa voiable. Dissemination of infection
(lymphatics, hematogenously) with metastatic
sites of infection occuis if tieatment is delayed.
Abnoimal oi piosthetic heait valve may be
colonized and infected. In the pieantibiotic
eia, the moitality iate was veiy high. In im-
muncompiomised patients, piognosis depends
on piompt iestoiation of alteied immunity,
usually on coiiection of neutiopenia. Without
suigical debiidement, NF is fatal. If neutiope-
nia exists, piognosis depends on iecoveiy of
neutiophil count.
MANACMNI
See guidelines foi the tieatment of skin and
soft tissue infections by the Infectious Diseases
Society of Ameiica at |.//www.,ourna|s.ut|-
tago.eJu./IDS/guJe|nes/
Frophy|axs PrImury Status postsaphenous
vein haivest (especially with tinea pedis): Wash
with benzoyl peioxide bai daily oi apply topical
antifungal cieam oi alcohol gel. Pneumotottus :
Immunize those at iisk. H| : Chemopiophy-
laxis foi household contacts <4 yeais of age
if unimmunized. V|ro spp.: Diabetics, alco-
holics, ciiihotics should avoid eating undei-
cooked seafood.
Secvndury InJJua|s w| ror esoJes o[
te||u|s (especially in sites of chionic lymph-
edema): Suppoit stockings oi sleeve, antiseptics
to skin (Puiell), chionic secondaiy antimiciobial
FICk 24-30 8|atera| ce||u|ts oI |es: v. vulolcus B||+|e|+| |ero|||+|c p|+que +ud |u||+e ou ||e
|e, +u||e, +ud |ee| o| +u o|de| d|+|e||c W||| c||||o|. uu|||e o||e| |,pe o| ce||u|||| |u W||c| r|c|oo|+u|r
eu|e| ||e ||u |oc+||,, ||+| W||c| | c+ued |, l .||: uu+||, |o||oW + p||r+|, eu|e|||| W||| |+c|e|er|+ +ud
d|er|u+||ou |o ||e ||u. \o| c+e |u|||+||, d|+uoed + |||+|e|+| ce||u|||| +|e |u||+rr+|o|, (ec/er+, |+| de|
r+||||, po||+|) |+||e| ||+u |u|ec||ou.
piophylaxis (penicillin G, dicloxacillin, oi
eiythiomycin, 500 mg/d). InerJga| nea
eJs : Tieat and institute piophylaxis against
iecuiient tinea pedis.
Supportve Rest, immobilization, elevation,
moist heat, analgesia.
0ressos Cool steiile saline diessings foi
iemoval of puiulent exudate and neciotic
tissue.
Surca| Ioterveotoo Diain abscesses. Debiide
neciotic tissue. Eaily/aggiessive suigical explo-
iation/debiidement is lifesaving in suspected
neciotizing STIs. Deep stiuctuies aie visualized,
neciotic tissue iemoved, compaitment decom-
piessed, tissues obtained foi Giam stain and
aeiobic and anaeiobic cultuies.
Aotmcroba| Iherapy In that most cases of
cellulitis aie caused by S. aureus and stiepto-
cocci, -lactam antibiotics with activity against
penicillinase-pioducing S. aureus aie the usual
diugs of choice.
1ndIcutIvns ]vr 1nItIu| 1V Therupy Lesion
spieading iapidly, systemic iesponse is piomi-
nent (chills, fevei of 37.8 C), clinically signifi-
cant coexisting conditions (immunocompiomise,
neutiopenia, asplenia, pieexisting edema, ciiiho-
sis, caidiac failuie, ienal insufficiency).
Oru| Therupy In healthy peisons with eaily
infection in the absence of systemic symptoms
and following initial IV theiapy, oial antibiotics
aie given (Table 24-4).
In immunocompetent hosts: tieat giam-
positive cocci ( S. aureus , GAS). In diabetics,
gieatei iange of potential pathogens, especially
aiising in diabetic ulceis: S. aureus , GAS, ente-
iococci; giam-negative aeiobes ( Proeus, K|e|-
se||a, Enero|ater, tneo|ater ). P. aerugnosa ;
anaeiobes ( BateroJes , Peotottus ).
IA8I 24-4 |nitia| Ireatment for Ce||u|itis at Specific Sites or Particu|ar E\posures
8acter|a| Spp. to Staodard Aot|m|crob|a| A|teroat|ve
Var|ab|e 0oos|der Therapy Aot|m|crob|a| Ageot
Bucc+| ce||u|||| | ||-cc- Ce||||+\oue (-2 /d |\ ) \e|opeuer
|r|peuerc||+|+||u
||r||||e+|eu|u Ae|o||c |+r Arp|c||||uu||+c|+r \e|opeuer o|
d|+|e||c |oo| u|ce| ue+||.e |+c|||| (! |\ qo|) |r|peuerc||+|+||u
c||ud+r,c|u +
||o+dpec||ur
||uo|oqu|uo|oue
(c|p|o||o\+c|u
o| |e.o||o\+c|u),
re||ou|d+/o|e
||uo|oqu|uo|oue o|
ce||||+\oue
nur+u |||e 0|+| +u+e|o|e Aro\|c||||uc|+.u|+u+|e |eu|c||||u +
(500 r |0 q3|) cep|+|opo||u
|o +ud c+| |||e | ||:!c e|c, Aro\|c||||uc|+.u|+u+|e \o\|||o\+c|u
ee 'E||o|o, (500 r |0 q3|) c||ud+r,c|u
E\pou|e |o +|| W+|e| +| l .||: |o\,c,c||ue (200 r |\ Ce|o|+\|re, c|p|o||o\+c|u
||e o| +||+|ou o| |u|||+||,, |o||oWed |,
|+ce|+||ou 00-200 r/d |\ |u
d|.|ded doe. C|.e +|ou
W||| +u||r|c|o||+| +eu|
|o| corrou p+||oeu)
E\pou|e |o ||e| 1-c pp. C|p|o||o\+c|u (+00 r |\ q2|) \e|opeuer
W+|e| +| ||e o| +||+|ou ce||+/|d|re |r|peuerc||+|+||u
o| |+ce|+||ou o| +||e| eu|+r,c|u
||e|+peu||c ue o| |eec|e
wo|||u + |u|c|e|, ||| | |c||c- Aro\|c||||u (500 r |0 C|p|o||o\+c|u
o| c|+r |+ud|e|, q3| |o| r||d ||u |u|ec||ou, ce|o|+\|re
.e|e||u+||+u, |oueW||e peu|c||||u C (2 r||||ou-20 |r|peuerc||+|+||u
r||||ou u |\ d+||,) |o| |+c|e|er|c
|u|ec||ou eudoc+|d|||
C|+|+c|e||/ed |, |+p|d p|o|e|ou o| |u|ec||ou
W||| e\|eu|.e uec|o| o| u|cu|+ueou ||ue
+ud o.e||,|u ||u.
C||u|c+| .+||+u| o| |'I| d|||e| W||| ||e
C+u+||.e o|+u|r
Au+|or|c |oc+||ou o| ||e |u|ec||ou
||ed|po|u coud|||ou.
Co||ec| d|+uo| | |rpe|+||.e |u uude||+ud|u
p+||oeue| +ud dec|d|u ou ||e +pp|op||+|e
+u||r|c|o||+| +ud u||c+| ||e|+p|e.
||- | -: | |. !c
| |- -:|-! | ||-- c- | -.--
- (+cce|e|+|ed |e+|| o| |ep||+|o|, |+|e,
o||u||+, reu|+| cou|u|ou) +ud/o| ore o| ||e
|o||oW|u |oc+| ,rp|or/|u.
'e.e|e pou|+ueou p+|u
|udu|+|ed eder+
Bu||+e
C,+uo|
'||u p+||o|
A|euce o| |,rp|+u|||
'||u |,pe||e|+
C|ep||+||ou
\uc|e We+|ue
|ou| re|| o| e\ud+|e.
\+, |e +oc|+|ed W||| |o\|c |oc| ,ud|ore.
|| |c:| | -:|c |c:| (||). |oc+|
|e|ou o| ||u o| rucou rer||+ue (+cu|e o|
c||ou|c d|e+e, ||+ur+, u|e|,), d|+|e|e, +|
|e||op+||,, +|co|o||r, o|e||,, |rruuoupp|e
|ou, ue o| uou|e|o|d+| +u|||u||+rr+|o|, d|u
(|'A||).
NCk0IIIINC S0FI IISS INFCII0NS
CIINICAI vAkIANIS
NF Caused by roup A streptococcus (CAS)
(kare|y, Croups 8, C, or C)
Often begins deep at site of nonpenetiating
minoi tiauma (biuise, muscle stiain).
May develop at the site of a bieak in the epi-
deimis
Minoi tiauma, laceiation, needle punctuie,
oi suigical incision) on an extiemity
Postopeiative abdominal incisions.
GAS may be seeded to this site duiing tian-
sient bacteiemia
Most cases occui in otheiwise healthy peisons,
often in childien and the eldeily.
Myoneciosis occuis concomitantly in 50% of
NF cases.
Stieptococcal neciotizing myositis occuis as a
piimaiy myositis.
Stieptococcal toxic shock syndiome may oc-
cui with GAS NF.
Gioup B stieptococcus (GBS) may cause NF:
Infected episiotomy incisions
Adult diabetics
C|oca| Fodos
Initially, findings of acute cellulitis (local ied-
ness, edema, heat, and pain in the involved
aiea), typically occui on an extiemity. Chai-
acteiistic findings appeai within 36-72 h aftei
onset: the involved aiea becomes dusky blue
in coloi; vesicles oi bullae appeai containing
initially yellowish, then ied-black fluid. Infec-

tion spieads iapidly along fascial planes iesult-
ing in extensive neciotic sloughs (Fig. 24-31).
Bullae iuptuie, and extensive, shaiply demai-
cated cutaneous gangiene develops. At this
point the aiea may be numb, and the black
neciotic eschai with suiiounding iiiegulai
boidei of eiythema iesembles a thiid-degiee
buin. The eschai sloughs off by the end of
1 week to 10 days. Peiipheial aieas of involve-
ment develop about the initial site of infec-
tion.
Fevei and othei constitutional symptoms aie
piominent as the inflammatoiy piocess extends
iapidly ovei the next few days. Stieptococcal TSS
occuis with GAS, GBS, GCS, GGS. Metastatic
abscesses may occui as a consequence of bactei-
emia, iesembling puipuia fulminans but then
evolving to daik-coloied blebs containing stiep-
tococci. Secondaiy thiombophlebitis is common,
but lymphangitis and lymphadenitis aie not.
Pyodeima gangienosum, puipuia fulminans
(disseminated intiavasculai coagulation), cal-
ciphylaxis, ischemic neciosis (atheioscleiosis
obliteians, thiomboembolism), fixed diug
eiuption, waifaiin neciosis, hepaiin neciosis,
piessuie ulcei, amebic ( Enamoe|a |so|yta )
skin gangiene aftei bowel suigeiy, biown ie-
cluse spidei bite.
MANACMNI
Surca| 0ebrdemeot Requiies eaily and com-
plete suigical debiidement of neciotic tissue
in combination with high-dose antimiciobial
agents.
Aotmcroba| Iherapy See Table 24-2.
Au |u||+rr+|o|, p|oce |u.o|.|u ||e u|cu|+ue
ou |,rp|+||c c|+uue|
E||o|o,.
Acu|e. \o| o||eu due |o CA', +|o ' c- ,
|+|e|,, | ||:!c o| |e|pe |rp|e\ .||u
'u|+cu|e. !,:|c:|- c , o||e|
uou|u|e|cu|ou r,co|+c|e||+ (|I\), '|
:|-| , |:c!c |c|-
C||u|c+| ||ud|u.
Acu|e. po||+| o| eu||, o| |u|ec||ou ou d||+| e\
||er||, +oc|+|ed W||| +ceud|u ||ue+| ||e+|,
|eude| |,rp|+deuop+||,.
'u|+cu|e. |odu|+| o| po|o|||c|o|d |,rp|+u|
||, |e|ou +| po||+| o| eu||, W||| e|,||er+|ou
uodu|e W||| ||ue+| +||+uereu|, |,rp|+deu
op+||,.
IMFhANCIIIS |C|9 . +512
|C|0 . 39
Forta| oI otry Bieak in skin, wound, infected
blistei, S. aureus paionychia, heipes simplex.
Ioca| Symptoms Pain and/oi eiythema pioxi-
mal to bieak in the skin.
Systemc Symptoms May occui eithei befoie
any evidence of infection is piesent at the site
of inoculation oi aftei the initial lesion has sub-
sided. May be moie piominent than expected
fiom degiee of local pain and eiythema.
Sko Fodos Red lineai stieaks and palpable
lymphatic coids, which may be up to seveial
centimeteis in width, extend fiom the local
lesion towaid the iegional lymph nodes (Figs.
24-32 and 24-33), which aie usually enlaiged
and tendei. Acute spoiotiichoid lymphangitis
can occui with S. aureus oi GAS infection.
Bieakdown of oveilying skin and ulceiation oc-
cui in couise of bacteiial lymphangitis; iaie in
the antibiotic eia.
FICk 24-31 Necrotto Iascts oI buttock E|,||er+|ou, eder+|ou p|+que |u.o|.|u ||e eu|||e |u|
|oc| W||| |+p|d|, p|o|e|.e +|e+ o| uec|o|.
Ioear Iesoos oo xtremtes Phytoalleigic
contact deimatitis (poison ivy oi oak), phyto-
photodeimatitis, supeificial thiombophlebitis,
cat-sciatch disease.
Suhucute SpvrvtrIchvId LymphungItIs
S. st|ent| , N. |ras|enss , M. marnum, Les|-
mana spp. aie the most common pathogens.
IA80kAI0k FIN0INCS
Cu|ture Isolate S. aureus oi GAS fiom poital
of entiy.
FICk 24-32 Acute |ymphaots oI Iorearm:
5. oureus A r+|| +|e+ o| ce||u|||| ou ||e .o|+|
W||| W||| + |eude| ||ue+| ||e+| e\|eud|u p|o\|r+||,
up ||e +|r, ||e |u|ec||ou p|e+d ||or ||e po||+| o|
eu||, W||||u ||e upe|||c|+| |,rp|+||c .ee|.
0IACN0SIS
The combination of a peiipheial lesion with
pioximal tendei/painful ied lineai stieaks lead-
ing towaid iegional lymph nodes is diagnostic
of lymphangitis.
C0kS AN0 Fk0CN0SIS
Bacteiemia with metastatic infection in vaiious
oigans may occui.
MANACMNI
See Table 24-2.
FICk 24-33 Acute |ymphaots oI Iorearm:
hSv |||r+|, |e|pe |rp|e\ .||u |u|ec||ou o| ||e
p+|r W||| |,rp|+u||| o| ||e |o|e+|r.
|o o| ||e |u|e|||, o| ||e ||u p|o.|de + po||+|
o| eu||, |o| |u|ec||ou.
A|| Wouud +|e co|ou|/ed |, |+c|e||+.
wouud |u|ec||ou (pu|u|euce, e|,||er+, W+|r||,
|eude|ue) ru| |e d|+uoed ou c||u|c+| +ud
cu||u|+| |ouud +ud ||e+|ed +pp|op||+|e|,.
W0N0 INFCII0NS
CIASSIFICAII0N 0F W0N0S
Chionic ulceis
Aiteiial insufficiency
Venous insufficiency
Neuiopathic ulceis/diabetes mellitus
Piessuie ulceis (bedsoies)
Tiauma
Bites
Animal
Human
Insect
Suigical wounds
Class I/clean
Class II/clean-contaminated
Class III/contaminated
Class IV/diity-infected
Buin wounds
Buin wound impetigo
Open buin-ielated suigical wound infec-
tion
Buin wound cellulitis
Invasive infection in unexcised buin
wounds
to|oy Health caie-associated MRSA (HA-
MRSA) is cuiiently the most common pathogen
isolated in wounds cultuied in hospital enviion-
ment. MSSA, Sreotottus , and PseuJomonas
spp. aie also commonly isolated. Otheis: E. to|,
Enerotottus spp., Proeus spp., coagulase-nega-
tive Sa|y|otottus (CoNS), fungi, vancomycin-
iesistant enteiococci, othei enteiobacteiiaceae,
K|e|se||a spp. Anaeiobes may constitute moie
than one-thiid of antimiciobial isolates.
Foi |uman, Jog, anJ ta |es , see page 611.
Fredsposo Factors
Cenera| [ators : Age, obesity, malnutiition;
endociine/metabolic factois; hypoxia, ane-
mia; malignant disease; immunosuppiession.
Lota| [ators : Neciotic tissue, foieign bodies,
tissue ischemia, hematoma foimation, pooi
suigical technique.
Mtro|a| tonamnaon : Type/viiulence of

oiganism; size of bacteiial inoculum, antibi-
otic iesistance.
ksk Factors Suigical wound infection is up
to 10 times moie likely among patients who
haiboi S. aureus in naies. The vast majoiity of
postopeiative wound infections aie caused by
a stiain of S. aureus that was piesent in naies
befoie suigeiy. Piesuigical cleaiance of caiiiage
with topical oi systemic antibiotics decieases
incidence of postopeiative staphylococcal infec-
tion. Postopeiative infection: piolonged opeia-
tive time, diabetes, obesity, chionic lung disease,
male sex, tieatment with glucocoiticoids, social
depiivation.
Nosocoma| IoIectoos Hospital-acquiied oi
health caie-associated infections (most com-
monly suigical wound infections) aie the most
common complication affecting hospitalized
patients. 5-10% of patients admitted to acute
caie hospitals acquiie one oi moie infections
(2 million patients annually in the United
States), iesulting in 90,000 deaths.
0eIotoo oI Surca| Wouod IoIectoo
Types v] SurgIcu| Wvund 1n]ectIvns
Suigical site infection
Supeificial incisional infection
Deep incisional infections
Oigan space infections
SurgIcu| sIte In]ectIvns must ]u|]I|| the ]v||vw-
Ing crIterIu:
Infection must occui within 30 days of sui-
geiy
Infection must involve only the skin and sub-
cutaneous tissue
Theie must be at least one of the following:
Puiulent dischaige fiom a supeificial infec-
tion
Oiganisms isolated fiom aseptically ob-
tained wound cultuie
Must be at least one of the following signs of
infection:
Pain oi tendeiness
Localized swelling
Redness oi heat

FAIh0CNSIS
Wounds aie initially colonized by skin floia
oi intioduced oiganisms. In some cases, these
oiganisms piolifeiate, causing a host inflam-
matoiy iesponse defined as infection. Skin and
soft tissue infections (SSTIs) include supeificial
conditions, such as eiysipelas, cellulitis, follicu-
litis, and fuiuncles, as well as deepei infections,
such as abscesses, neciotizing fasciitis, myositis,
and gas gangiene.
Symptoms Local infection: tendeiness, pu-
iulent diainage. Invasive infection: malaise,
anoiexia, sweats; fevei, chills.
Sko Fodos Puiulent diainage, eiythema,
waimth, induiation, tendeiness.
Systemc Fodos Sepsis syndiome (fevei,
hypotension).
Iypes oI Surca| IoIectoos Supeificial infec-
tion of wound (Fig. 24-36), impetigo/ecthyma
(Figs. 24-34, 24-37, 24-38), cellulitis (Figs.
24-35, 24-39), eiysipelas, soft tissue abscess
(see Fig. 24-17), NSTIs, tetanus.
Alleigic contact deimatitis (e.g., topical antibi-
otic such as neomycin), heipes zostei; heipes
simplex, pyodeima gangienosum, vasculitis,
peiipheial vasculai disease (infaiction), dis-
seminated intiavasculai coagulation (DIC)
(puipuia fulminans), waifaiin neciosis.
FICk 24-34 Surca| excsoo wouod
oIectoo: MSSA 'u||c+| ||e W+ ||e po||+| o|
eu||, |o| |u|ec||ou 1 d+, +||e| ||e p|ocedu|e, W|||
u|equeu| ce||u||||. |e||ceuce o| ||e Wouud
|+ occu||ed. |ec|o||c ||ue | eeu |u ||e |eu|
|+u| u|ce|/Wouud.
FICk 24-35 Surca| excsoo[
raIt oIectoo: MkSA Ce||u|||| o|
||e e,e||d occu||ed |o||oW|u e\c||ou
+ud |+|||u o| |eu||o r+||u+ o| ||e
c|ee|.
0rect Mcroscopy Giam stain of exudate, pus,
bulla fluid, aspiiate, oi touch piepaiation may
show bacteiia.
Cu|ture aod Seostvtes Indications: wounds
with classic signs of infection puiulent diain-
age, signs of inflammation (eiythema, incieased
waimth, induiation, tendeiness)]. Specimens:
exudates and neciotic tissue.
FICk 24-36 Surca| wouod oIectoo: MkSA |u.+|.e qu+rou ce|| c+|c|uor+ W+ |ero.ed |, d|c e\c|
|ou W||| e|ec||ou|e|, |o ||e |+e. I|e p+||eu| |+d p||o| \k'A |u|ec||ou. I|e Wouud |ec+re p+|u|u| 1 d+, |o||oW|u
||e p|ocedu|e. \k'A W+ |o|+|ed +ud |epo||ed |o |e eu|||.e ou|, |o ||ue/o||d +ud .+ucor,c|u. I|e |u|ec||ou |eo|.ed
W||| ||ue/o||d, o00 r ||d |o| 1 d+,. |++| c+|||+e W+ ||e+|ed W||| rup||oc|u o|u|reu|. I|e|e | +|o ||ue+ ped|
FICk 24-3T Iaceratoo oIectoo o reoa|
traosp|aot recpeot: MkSA A |+ce|+||ou ou
||e |oWe| |e |+ |ecore p+|u|u| W||| u||ouud|u
e|,||er+ +ud eder+. IWo |u.+|.e qu+rou ce||
c+|c|uor+ +|e +|o eeu ou ||e c+||. ne |+ |eeu
|rruuoupp|eed |o| 22 ,e+|.
0IACN0SIS
Because all open wounds become colonized with
miciooiganisms, diagnosing infection ielies on
the clinical chaiacteiistics of the wound. Wounds
with classic signs of infection: Giam stain of exu-
dates helpful initially. Cultuies aie definitive.
MANACMNI
Although all wounds iequiie tieatment, only
infected lesions iequiie antimiciobial theiapy.
Freveotoo oI Wouod IoIectoo Ervgenvus
Steiilization of instiuments, sutuies, etc.;
positive piessuie ventilation; laminai aii flow;
exclusion of staff with infections.
Endvgenvus Skin piepaiation; antibiotic
piophylaxis, good suigical technique.
Wouod Care Adjunctive tieatments include
weight off-loading, topical agents, special diess-
ings, contiol of edema, ievasculaiization.
Surca| 0ebrdemeot Tieating infected
wounds often iequiies suigical pioceduies
(e.g., debiidement), especially foi deep oi
neciotic wounds. Modein buin wound theiapy
centeied on eaily excision and closuie of the
wound.
Aotmcroba| Iherapy Viitually all infected
wounds iequiie antimiciobial theiapy. See
Table 24-2.
TvpIcu| AntImIcrvhIu| Therupy May be suf-
ficient foi supeificial lesions.
FICk 24-38 IoIectoo o chrooc |ymph-
edema: MSSA \u|||p|e p+|u|u| u|ce| W||| u|
|ouud|u e|,||er+ ou + .e|, eder+|ou |oWe| |e +ud
|oo|. I|e p+||eu| |+ |d|op+|||c |e||ope|||oue+| ||||o|
W||| p|o|e|.e |,rp|eder+ +ud u|ce|+||ou. ne W+
||e+|ed W||| cep|+|e\|u + ecoud+|, p|op|,|+\|.
FICk 24-39 IoIectoo oI Iactta| u|cers:
MkSA A 5!,e+|o|d r+|e W||| o|e|.ecorpu|
|.e d|o|de| c|+|c|ed e\||er|||e |u ||e e.eu|u. ne
W+ |e|u ||e+|ed |, + p,c||+|||| W|o |+c|ed |u|||
|u|o ||e o|||u o| ||e u|ce|. \k'A |u|ec||ou occu||ed
|epe+|ed|,. u|ce| |eo|.ed W||| o|+| c||ud+r,c|u, do\
ep|u, +ud uuu+ |oo| +pp||ed Wee||,.
' c- , |oup A ||ep|ococcu (CA'), 3 c
||c: , C !|||-c- , +ud C |-|c p|oduce |o\
|u ||+| |+.e |oc+| rucocu|+ueou +ud ,|er|c
e||ec|.
C||u|c+| ,ud|ore c+ued |, ||ee |o\|u.
Bu||ou |rpe||o
'|+p|,|ococc+| c+|ded||u ,ud|ore ('''')
'|+p|,|ococc+| |o\|c |oc| ,ud|ore (I'')
'|+p|,|ococc+| |ood po|ou|u (eu|e|o|o\|u)
'c+||e| |e.e| ('|)
'||ep|ococc+| I''
Au|||+\
||p|||e||+
Ie|+uu
CkAM-F0SIIIv C0CCAI INFCII0NS ASS0CIAI0
WIIh I0XIN Fk00CII0N (INI0XICAII0NS)
Staphy|ococca| Ioxos
Toxic shock syndiome toxin 1 (TSST-1)
Induces fevei diiectly on the hypothalamus
oi indiiectly via inteileukin 1 (IL-1) and
tumoi neciosis factoi (TNF) pioduction
Piomotes T lymphocyte supeiantigeniza-
tion" and oveistimulation
Induces inteifeion pioduction
Enhances delayed hypeisensitivity
Suppiesses neutiophil migiation and im-
munoglobulin secietion
Enhances host susceptibility to endotoxins
Exfoliatin type A and B (ET-A, ET-B).
ET-A is iesponsible foi the pathogenic
changes of the SSSS.
These toxins bind diiectly to desmoglein-1,
a desmosomal cadheiin
Results in inteidesmosomal splitting and
causes blisteiing and denudation by disiup-
tion of the epideimal gianulai cell layei.
Staphylococcal enteiotoxins B and C (SEB,
SEC) have a biochemical stiuctuie almost
identical to that of TSST-1.
Streptococca| Ioxos
Stieptolysin S: leukocidin
Stieptolysin O: leukocidin
NADase: leukotoxic
Hyaluionidase: digests host connective tissue
hyaluionic acid
Stieptokinases: paiticipate in fibiin lysis.
Stieptodoinases A-D: possess deoxyiibo-
nuclease activity. Stieptodoinases B and D:
possess deoxyiibonuclease and iibonuclease
activity
Piotease activity similai to that in S. aureus
has been shown in stiains causing soft tissue
neciosis oi toxic shock syndiome.
Stieptococcal pyiogenic exotoxins (SPE) (foi-

meily known as eiythiogenic toxin) types A,
B, C
Superaoteos
Gioup of bacteiial and viial pioteins
Act as globulai intact pioteins
Piesented by class II majoi histocompat-
ibility complex (MHC) molecules
Bind to conseived amino acid iesidues that
aie on the outei walls of peptide antigen-
binding gioove
Bind to vaiiable iegion of T cell ieceptoi
chain (V )
Activate a laige peicentage of T cells ex-
piessing ielevant T cell ieceptoi V chains
Supeiantigen-mediated T cell activation
geneiates incieased numbeis of T cells ex-
piessing the skin homing ieceptoi cutane-
ous lymphocyte antigen (CLA)
Supeiantigens lead to massive ielease of
cytokines, a cytokine stoim:
TNF-
IL-1
IL-6
Cytokines cause capillaiy leak syndiome
and clinical manifestations of supeiantigen-
mediated diseases
Bind diiectly to MHC class II molecules on
the suiface of antigen-piesenting cells
Can stimulate 10-20% of T cells (conven-
tional antigen can stimulate 1/10,000 T cells)
Massive T cell stimulation iesults in ielease
of
IL-1 and -2
TNF
Inteifeion
Staphylococcal supeiantigens include SEs,
TSST-1, some ETs.

Syndiomes associated with staphylococcal ex-
foliative toxin do not have significant sys-
temic symptoms. Howevei, syndiomes due to
supeiantigens aie chaiacteiized by systemic
manifestations. Supeiantigen-mediated toxin
syndiomes can be divided accoiding to the iela-
tive amounts of systemic toxicity:
Inteimediate cutaneous and systemic: iecal-
citiant desquamating disoidei (REDD), ie-
cuiient toxin-mediated eiythema (iecuiient
peiineal eiythema)
Piedominantly systemic: scailet fevei, TSS
E||o|o,. ' c-.
Ae. occu| r+|u|, |u ueW|o|u +ud |u|+u| 2
,e+|. A|o, o|de| |rruuocorp|or|ed pe|ou
|+||oeue|. |o\|ured|+|ed ep|de|ro|,||c d|
e+e
C||u|c+| ,ud|ore. E|,||er+ +ud W|dep|e+d
de|+c|reu| o| ||e upe|||c|+| |+,e| o| ||e ep|de|
r|, |eer|||u c+|d|u
'e.e|||, |+ue ||or.
|oc+||/ed |o|r. |u||ou |rpe||o
Ceue|+||/ed |o|r W||| e\|eu|.e ep|de|ro|,|
+ud dequ+r+||ou. eue|+||/ed c+|ded||u
,ud|ore
A|o|||.e |o|r (c+||+||u||o|r .+||+u|) (|+p|,|o
cocc+| c+||e| |e.e|)
\+u+ereu|. ,|er|c +u||||o||c |o ||e+| |u|ec||ou
+ud |op |o\|u p|oduc||ou
SIAFhI0C0CCAI SCAI00-SkIN SN0k0M (SSSS) |C|9 . o95.3
Ae oI 0oset Most common in neonates dui-
ing fiist 3 months of life. Infants and young
childien <5 yeais. Raiely in immunocompio-
mised adults.
to|oy S. aureus of phage gioup 2 (types 71
and 55). S. aureus pioduces exotoxins:
Exfoliatin A and B (ET-A and ET-B)
Seiine pioteases that bind to cell adhesion
molecule, iesulting in loss of cell-cell adhe-
sion
Epideimolysis takes place between stiatum
coineum and stiatum gianulosum
Exfoliatin B (ET-B); pioduced by plasmid
(pieces of self-ieplicating DNA that often
code foi secondaiy chaiacteiistics, such as
antibiotic iesistance and toxin pioduction).
Exotoxins aie pioteases that cleave desmo-
glein-1, which noimally hold the gianulosum
and spinulosum layeis togethei.
ksk Factors Age <5 yeais. Adults: ienal fail-
uie, systemic immunosuppiession.
FAIh0CNSIS
In newboins and infants, S. aureus colonizes
nose, conjunctivae, oi umbilical stump with oi
without causing clinically appaient infection,
pioducing ETs that aie tianspoited hematog-
enously to the skin. In bullous impetigo, ET is
pioduced in impetigo lesion. Specific antistaph-

ylococcal antibody, metabolic diffeiences, oi
the gieatei ability to localize, metabolize, and
exciete in individuals >5 yeais piobably ac-
counts foi decieased incidence of SSSS with
oldei age. At times puiulent conjunctivitis,
otitis media, oi occult nasophaiyngeal infec-
tion occuis at site of toxin pioduction. ET
causes acantholysis and intiaepideimal cleav-
age within the stiatum gianulosum. Local ef-
fects of the ET iesult in bullous impetigo, but
with absoiption of the toxin, a mild scailati-
nifoim iash accompanying the bullous lesions
may appeai. Conveisely, local effects of the
toxin may be absent, with systemic absoip-
tion iesulting in a staphylococcal scailet fevei
syndiome. Moie extensive epideimal damage
is chaiacteiized by sloughing of supeificial epi-
deimis in SSSS. Healing occuis spontaneously
in 5-7 days.
Sko Symptoms SSSS: eaily eiythematous ai-
eas aie veiy tendei.
Sko Iesoos
Lvcu|Ized Fvrm See Bullous Impetigo"
(p. 598). Intact flaccid puiulent bullae, clus-
teied. Ruptuie of the bullae iesults in moist ied
and/oi ciusted eiosive lesions. Lesions aie often
clusteied in an inteitiiginous aiea.
Generu|Ized Fvrm ET-induced changes: mi-
cio-maculai scailatinifoim iash (staphylococcal
scailet fevei syndiome) oi diffuse, ill-defined eiy-
thema (Fig. 24-40) and a fine, stippled, sandpa-
pei appeaiance occui initially. In 24 h, eiythema
deepens in coloi and involved skin becomes ten-
dei. Initially peiioiificially on face, neck, axillae,
gioins; becoming moie widespiead in 24-48 h.
Initial eiythema and latei sloughing of supeificial
layeis of epideimis aie most pionounced peii-
oiificially on face and in flexuial aieas on neck,
axillae, gioins, antecubital aiea, back (piessuie
points). With epideimolysis, epideimis appeais
wiinkled and can be iemoved by gentle piessuie
(skin iesembles wet tissue papei) (Nikolsky sign)
(Fig. 24-40). In some infants, flaccid bullae occui.
Unioofed epideimis foims eiosions with ied,
moist base. (Fig. 24-41). Desquamation occuis
with healing (Fig. 24-41B).
Mucous Membraoes Uninvolved in SSSS.
Ceoera| xamoatoo Possible low-giade fevei.
Iiiitable child, pain.
TSS, Kawasaki syndiome, diug-induced toxic
epideimal neciolysis (TEN).
0rect Mcroscopy Grum StuIn
Bullous impetigo: pus in bullae, clumps of
giam-positive cocci within PMN.
SSSS: giam-positive cocci only at colonized
site, not in aieas of epideimolysis.
8actera| Cu|ture
Bullous impetigo: S. aureus isolated fiom
involved site.
FICk 24-40 Staphy|ococca| sca|ded-sko syodrome: Nko|sky so I|e ||u o| ||| |u|+u| | d|||ue|,
e|,||er+|ou, eu||e p|eu|e |o ||e ||u o| ||e +|r |+ |e+|ed o|| ||e ep|de|r|, W||c| |o|d |||e ||ue p+pe|.
SSSS: S. aureus only at site of infection (i.e.,
site of toxin pioduction)-umbilical stump,
ala nasi, nasophaiynx, conjunctivae, exteinal
eai canal, stool. S. aureus is not iecoveied
fiom sites of sloughing skin oi bullae.
0ermatopatho|oy Intiaepideimal cleavage
with splitting occuiiing beneath and within
stiatum gianulosum.
0IACN0SIS
Clinical findings confiimed by bacteiial cultuies.
Tzanck smeai ieveals acantholytic keiatinocytes.
C0kS AN0 Fk0CN0SIS
In late phases of SSSS, and in an acceleiated
mannei, aftei adequate antibiotic tieatment,
the supeificially denuded aieas heal in 3-5
days associated with geneialized desquamation
in laige sheets of skin (Fig. 24-42); theie is no
scaiiing. Death can occui in neonates with ex-
tensive disease.
MANACMNI
Frophy|axs Pievent spiead of toxigenic S.
aureus in neonatal caie units.
Ceoera| Care Hospitalization is iecommended
foi neonates and young childien, especially if
skin sloughing is extensive and paiental com-
pliance questionable. Dischaige home when
significant impiovement is appaient. If case is
mild and home caie ieliable, childien can be
tieated with oial antibiotic.
Iopca| Iherapy Baths oi compiesses foi de-
biidement of neciotic supeificial epideimis.
Topical antimiciobial agents foi impetigo le-
sions: mupiiocin, ietapamulin, bacitiacin, oi
silvei sulfadiazine ointment.
Systemc Aotmcroba| Iherapy See Table 24-2.
Adjuoctve Iherapy Replace significant watei
and electiolyte loss intiavenously in seveie cases.
FICk 24-41 Staphy|ococca| sca|ded-sko syo-

drome: desquamatoo |u ||| |u|+u|, p+|u|u|, |eude|,
d|||ue e|,||er+ W+ |o||oWed |, eue|+||/ed ep|de|r+|
|ou||u +ud e|o|ou. ' c- |+d co|ou|/ed ||e
u+|e W||| pe||o|+| |rpe||o, ||e ||e o| e\o|o\|u p|oduc
||ou. . E\|eu|.e dequ+r+||ou | eeu ou ||e |+ce +ud
+u|e||o| ||uu|. 8. |equ+r+||ou ou |u||oc| +ud |e.
8
Ae oI 0oset MTSS: 23 yeais (mean age);
NMTSS: 27 yeais (mean age).
Sex Piioi to 1984, >99% of cases weie in
females; aftei 1984, equal sex distiibution.
8-10% of women aie vaginal caiiieis of S.
aureus .
kace In the United States, 97% of MTSS in
whites; 87% of NMTSS in whites.
to|oy Toxin-pioducing S. aureus pioducing
TSST-1, GAS
oder|yo 0sorders NMTSS can occui
secondaiy to a wide vaiiety of piimaiy S. aureus
and GAS infections as well as secondaiy infec-
tion of undeilying deimatoses.
SIte v] TSST-1 PrvductIvn
Mentiual-associated: Use of vaginal tampon
of high absoibency. Usually piesent on 3 id to
5 th day of menses.
Nonmenstiual
Wounds
Nonsuigical wounds (buins, skin ulceis,
cutaneous and oculai injuiies, insect
bites, body pieicing site)
Suigical wounds: meJan time of onset of
postopeiative NMTSS
Supeiinfection of vaiicella
Postpaitum infections
Vaginal nonmenstiual oiigin (contiaceptive
sponge, contiaceptive diaphiagm)
Nasal packing
Supeiinfection aftei influenza oi acute si-
nusitis
Streptvcvccu| TSS superuntIgen prvductIvn
Soft tissue infections: cellulitis, neciotizing fas-
ciitis.
E||o|o,. Io\|up|oduc|u ' c- +ud CA'

C||u|c+| e|||u
'|+p|,|ococc+| I''
\eu||u+| (\I'') (|+|e +||e| 93+)
|oureu||u+| (|\I'')
'||ep|ococc+| I''
'||u o| o|| ||ue |u|ec||ou W||| |o\|u p|oduc
||ou
C||u|c+| r+u||e|+||ou
k+p|d oue| o| |e.e| +ud |,po|eu|ou
'||u ||ud|u
E+||,. eue|+||/ed ||u +ud ruco+| e|,||er+
|+|e. dequ+r+||ou |u e+||, cou.+|eceuce
0|+u |,pope||u|ou +ud ru|||,|er |+||u|e
+ud |op |o\|u p|oduc||ou. 'uppo|||.e.
I0XIC Sh0Ck SN0k0M |C|9 . 0+0.32
|C|0 . A+3.!
FAIh0CNSIS
S. aureus multiplies foieign body, aiound, in
minoi wound infection, oi mucosal suiface,
elaboiating the TSST-1 and staphylococcal
enteiotoxin B. Toxins aie absoibed and act
as supeiantigens that allow the nonspecific
binding of MHC II with T cell ieceptois, ie-
sulting in polyclonal T cell activation, causing
secietion of massive amounts of cytokines.
Cytokines iesult in the clinical syndiome
of fevei, hypotension, iash, oigan hypop-
eifusion/multioigan failuie. The individual
must be colonized oi infected with toxigenic
stiain of S. aureus oi GAS and must lack a
piotective level of antibody to the toxin made
by that stiain. >90% of adults have antibodies
to TSS toxins.
Iocubatoo Ferod Shoitei in NMTSS. Aftei
suigical pioceduie, <4 days.
Symptoms Recuiient symptoms in MTSS in
untieated cases; tampon use. Sudden onset of
fevei, hypotension. Tingling sensation in hands
and feet. Maculopapulai eiuption, piuiitic.
Geneialized myalgias, muscle tendeiness and
weakness; headache, confusion, disoiientation,
seizuies; piofuse diaiihea; dyspnea.
Sko Iesoos
Geneialized scailatinifoim eiythiodeima,
most intense aiound infected aiea. Blanching
eiythema, painless sunbuin." Fades within 3
days of appeaiance.
Edema, extensive geneialized, nonpitting;
most maiked on face (Fig. 24-42), hands, feet.
One week aftei onset of skin lesions, desqua-
mation begins with scaling of skin of toiso,
face, and extiemities, followed by desquama-
tion of palms, soles, fingeis/toes.
Cutaoeous Stes oI IoIectoo[Co|ootatoo
Soft tissue infection: Cellulitis, neciotizing
fasciitis, pueipeial sepsis
Vaiicella in childien with supeiinfection
Raiely asymptomatic stieptococcal phaiyn gitis
Mucous Membraoes
Foigotten oi ietained vaginal tampon.
Eyes: Intense eiythema and injection of bul-
bai conjunctivae (Fig. 24-42). Subconjuncti-
val hemoiihages.
Mouth: Eiythema of mucous membianes of
mouth, tongue, phaiynx, tympanic mem-
bianes. Stiawbeiiy tongue. Ulceis: mouth,
esophagus.
Genital: vagina eiythema, ulceis.
Ceoera| Fodos
Fevei.
Oigan hypopeifusion iesults in ienal and
myocaidial dysfunction, fluid oveiload,
and adult iespiiatoiy distiess syndiome
(ARDS).
Late complications include peiipheial gan-
giene, muscle weakness, lingeiing asthenia,
neuiopsychiatiic dysfunction.
Ioxo-Medated IoIectoos SSSS, scailet fevei,
Kawasaki disease.
rythema Mu|tsystem 0sease SSSS, Kawa-
saki syndiome, Rocky Mountain spotted fevei
(RMSF), giam-negative sepsis, exanthematous
viial syndiomes; seveie adveise diug ieactions
(Stevens-Johnson syndiome, TEN), acute sys-
temic lupus eiythematosus (SLE).
0rect Mcroscopy Grum StuIn Vaginal,
wound exudate: many leukocytes and giam-
positive cocci in clusteis.
Cu|ture S. aureus : vaginal, wound exudate,
foieign body. GAS: blood oi piimaiy site of
infection.
8opsy Confluent epideimal neciosis, vacu-
olai alteiation of deimal-epideimal junction,
subepideimal vesiculation, little oi no inflam-
matoiy infiltiate in deimis.
0IACN0SIS
Case 0eIotoo oI ISS
Ao I||oess wth the Fo||owo C|oca| MaoIes-
tatoos
Feer : Tempeiatuie 38.9C (102F)
Ras|: Diffuse maculai eiythiodeima
Desquamaon: 1-2 weeks aftei onset of ill-
ness, paiticulaily on the palms and soles
Hyoenson: Systolic blood piessuie (BP) 90
mmHg (adults) oi less than fifth peicentile
foi age (childien <16 yeais of age); oi oithos-
tatic hypotension (oithostatic diop in diasto-
lic BP by 15 mmHg, oithostatic dizziness, oi
oithostatic syncope)
Sysemt no|emen (thiee oi moie of the
following):
Gastiointesinal : vomiting oi diaiihea at
onset of illness
Musculai : seveie myalgias oi seium cieatine
phosphokinase level at least twice the uppei
limit of noimal
Mucosal hypeiemia : vaginal, oiophaiyn-
geal, conjunctiva
Renal : blood uiea nitiogen oi cieatinine at
least twice the uppei limit of noimal foi
laboiatoiy oi uiinaiy sediment with pyuiia
( 5 leukocytes pei high-powei field) in the
absence of uiinaiy tiact infection
Hepatic : total biliiubin, alanine aminotians-
feiase enzyme, oi aspaitate aminotians-
feiase enzyme levels at least twice the uppei
limit of noimal foi laboiatoiy
Hematologic : platelet count <100,000/L
CNS : disoiientation oi alteiations in con-
sciousness without focal neuiologic signs
when fevei and hypotension aie absent
Iaboratory Crtera: Neatve kesu|ts oo the
Fo||owo Iests, I 0btaoed
Blood, thioat, oi ceiebiospinal fluid cultuies
(blood cultuie may be positive foi S. aureus)
Rise in titei to Rocky Mountain spotted fevei,
leptospiiosis, oi measles.
Case C|assIcatoo
Prvhuh|e A case in which foui of the five
clinical findings desciibed above aie piesent.
Cvn]Irmed A case in which all five of the
clinical findings desciibed above aie piesent,
including desquamation, unless the patient dies
befoie desquamation occuis.

E||o|o,. C|oup A ||ep|ococcu (CA'), e\o|o\|u
p|oduc|u ||+|u
C||u|c+| ,ud|ore
|u|ec||ou. |ou||, ||u, o|| ||ue, o||e|
Io\|u ,ud|ore. c+||+||u||o|r e\+u||er |u ||e
uou|rruue |ud|.|du+|
'e.e|e |u|ec||ou +ud |o\|u p|oduc||ou c+ue
||ep|ococc+| |o\|c |oc||||e ,ud|ore (I'|')
+ud p|e.eu| uouuppu|+||.e eque|+e
',,. 'c+||+||u+
SCAkII Fvk |C|9 . 0!+
|C|0 . A!3
Ae oI 0oset Childien.
Iocdeoce Much less than in the past.
to|oy Usually gioup A -hemolytic S.
yogenes (GAS). Uncommonly, ET-pioducing
S. aureus.
C0kS AN0 Fk0CN0SIS
Diagnosis of NMTSS is often delayed because of
the wide vaiiety of clinical settings and associ-
ated symptomatology. Complications: iefiactoiy
hypotension, ARDS, caidiomyopathy, aiihyth-
mias, encephalopathy, acute ienal failuie, meta-
bolic acidosis, livei neciosis, DIC. Recuiience of
untieated MTSS is possible. Antibiotic theiapy
and discontinuance of tampons significantly
ieduce iisk. Recuiiences aftei NMTSS aie iaie.
Among cases iepoited to the Centeis foi Disease
Contiol and Pievention (CDC) (1985-1994),
case fatality iate was 2.5% foi menstiual cases,
and 6.4% foi nonmenstiual cases.
Stieptococcal TSS: associated with moitality
iate of 25-50%.
MANACMNI
Ioca| IoIectoo Remove potentially foieign
bodies. Diain and iiiigate infected sites.
Systemc Aotmcroba| Iherapy (See
Table 24-2.) IV antistaphylococcal antibiotic.
Clindamycin, 900 mg IV q8h. Nafcillin oi oxa-
cillin, fiist-geneiation cephalospoiin. Vancomy-
cin foi MRSA.
Adjuoctve Iherapy Aggiessive monitoi-
ing and management of specific oigan system
failuie (i.e., management of fluid, electiolyte,
metabolic, and nutiitional needs). Methylpied-
nisolone foi seveie cases.
FAIh0CNSIS
Scailet fevei occuis in the nonimmune pei-
son. Pioduction of stieptococcal pyiogenic
exotoxins eiythiogenic toxin (ET)] A, B, oi C
depends on the piesence of a tempeiate bac-
teiiophage. The development of SF iash may
FICk 24-42 Ioxc shock
syodrome E|,||er+ o| ||e |u||+|
coujuuc||.+e +oc|+|ed W||| |+c|+|
e|,||er+ +ud eder+ |u + |er+|e W|||
reu||u+| I''.
ieflect hypeisensitivity ieaction iequiiing piioi
exposuie to toxin. S. aureus can synthesize an
ET, pioducing a scailatinifoim exanthem. In
addition to ET, GAS also pioduces stieptolysins
O and S. Antibodies to antistieptolysin O can be
used to assess iecent GAS infection. SF seems to
be less seveie than a centuiy ago due to deciease
in viiulence of GAS oi the advent of antimicio-
bial theiapy.
Iocubatoo Ferod Rash appeais 1-3 days aftei
onset of infection.
xposure Household membei(s) may be a
stieptococcal caiiiei.
Symptoms Fevei, soie thioat, fatigue, head-
ache, flushing, tachycaidia, enlaiged ceivical
lymph nodes.
Sko Iesoos SIte v] GAS 1n]ectIvn
Phaiyngitis; tonsillitis.
Infected suigical oi othei wound.
Impetiginized (secondaiily infected) skin
lesion.
Erunthem
Face: flushed with peiioial palloi.
Finely punctate eiythema is fiist noted on
the uppei pait of the tiunk (Fig. 24-43);
may be accentuated in skin folds such as
neck, axillae, gioin, antecubital and pop-
liteal fossae (Pastia lines).
Palms/soles usually spaied.
Initial punctate lesions become conflu-
ently eiythematous, i.e., scailatinifoim.
Lineai petechiae (Pastia sign) occui in
body folds.
Intensity of the exanthem vaiies fiom
mild to modeiate eiythema confined to
the tiunk due to an extensive puipuiic
eiuption.
PetechIue Scatteied petechiae occui
(Rumpel-Leede test foi capillaiy fiagility
positive).
DesquumutIvn Exanthem fades within
4-5 days and is followed by desquamation
on the body and extiemities and by sheet-
like exfoliation on the palms/fingeis and
soles/toes. In subclinical oi mild infections,
exanthem and phaiyngitis may pass unno-
ticed. In this case patient may seek medical
advice only when exfoliation on the hand
and soles is noted.
Mucous Membraoes SIte v] GAS 1n]ectIvn
Acute folliculai oi membianous tonsillitis.
May be asymptomatic oi mild and go undetec-
ted.
Enunthem
Phaiynx beefy ied.
Foichheimei spots: Small ied macules on
haid/soft palate, uvula.
Punctate eiythema and petechiae may occui
in the palate.
Tongue
White tongue: Initially is white with scat-
teied ied, swollen papillae (white stiaw-
beiiy tongue) (Fig. 24-44).
Red stiawbeiiy tongue: By the fouith oi
fifth day, the hypeikeiatotic membiane is
sloughed, and the lingulai mucosa appeais
biight ied (Fig. 24-44).
Ceoera| xamoatoo Patient may appeai
acutely ill with high fevei, fatigue, soie thioat,
headache, nausea, vomiting, tachycaidia. An-
teiioi ceivical lymphadenitis associated with
phaiyngitis/tonsillitis.
Streptococca| ISIS Toxemia, oigan failuie,
and a scailatinifoim iash associated with GAS
cellulitis oi neciotizing fasciitis.
FICk 24-43 Scar|et Iever: exaothem ||ue|,

puuc|+|ed e|,||er+ |+ |ecore cou||ueu| (c+||+||u||o|r),
pe|ec||+e c+u occu| +ud |+.e + ||ue+| cou||u|+||ou W||||u
||e e\+u||er |u |od, |o|d (|+||+ ||ue).
Ceoera|ted xaothem Staphylococcal SF
(phaiyngitis, tonsillitis, stiawbeiiy tongue, and
palatal enanthem no seen), staphylococcal oi
stieptococcal TSS, Kawasaki syndiome, viial
exanthem, adveise cutaneous diug eiuption.
0rect Mcroscopy Grum StuIn Giam-positive
cocci in chain (GAS) oi clusteis ( S. aureus )
identified in smeai fiom infected wound oi im-
petiginized skin lesion.
kapd 0rect Aoteo Iests (0AIs) Used to
detect GAS antigens in thioat swab specimens.
Cu|ture Isolate GAS on cultuie of specimen
fiom thioat oi wound. Blood cultuies aie iaiely
positive.
hemato|oy Leukocytosis with neutiophilia,
elevated ESR.
Sero|oy Elevated antistieptolysin O titei.
0IACN0SIS
Clinical findings confiimed by detecting stiep-
tococcal antigen in a iapid test and/oi cultuiing
GAS fiom thioat oi wound.
Centoi clinical ciiteiia foi the diagnosis of
stieptococcal phaiyngitis:
Tempeiatuie > 38C
Tendei anteiioi ceivical adenopathy
Lack of a cough
Phaiyngotonsillai exudate.
C0kS AN0 Fk0CN0SIS
Suppuratve Comp|catoos
Peiitonsillai cellulitis
Peiitonsillai abscess
Retiophaiyngeal abscess
Otitis media
Acute sinusitis
Suppuiative ceivical lymphadenitis
Bacteiemia/septicemia with stieptococcal
TSS
Metastatic foci of infection
Noosuppuratve Seque|ae oI Streptococca|
IoIectoos
Acute iheumatic fevei (onset 1-4 weeks af-
tei onset of phaiyngitis). Incidence of acute
iheumatic fevei is about 3%; has maikedly
decieased duiing the past five decades
Acute glomeiulonephiitis (moie common
aftei impetigo with nephiitogenic stiain of
GAS (types 4, 12, 2, 49, and 60)
Guttate psoiiasis (see Section 3)
Eiythema nodosum may follow if the infec-
tion goes untieated (see Section 7)
MANACMNI
Symptomatc Iherapy Aspiiin oi acetamino-
phen foi fevei and/oi pain.
Systemc Aotmcroba| Iherapy Pent||n s
|e Jrug o[ t|ote because of its efficacy in pie-
vention of iheumatic fevei. Goal is to eiadicate
GAS thioat caiiiage.
Fo||ow-p Recultuie of thioat iecommended
foi individuals with histoiy of iheumatic fevei
oi if a family membei has histoiy of iheumatic
fevei.
FICk 24-44 Scar|et Iever: whte aod red
strawberry tooue B|||| |ed |ouue W||| p|or|
ueu| p+p|||+e ou ||e ||||| d+, +||e| oue| o| |oup A
||ep|ococc+| p|+|,u||| |u + c|||d. I|e W|||e p+|c|e
+| ||e |+c| o| ||e |ouue |ep|eeu| |e|du+ o| ||e
|u|||+| W|||e ||+W|e||, |ouue.
to|oy
B. anthiacis , a nonmotile, giam-positive, aei-
obic iod 1.2-10 m in length and 0.5-2.5 m
in width.
Spoies can iemain doimant in soil foi dec-
ades.
Anthiax spoies have been developed as a bio-
logic weapon. (See Appendix B)
0ccupatoo Faimeis, heideis; slaughteihouse
and textile woikeis.
Iraosmssoo Zoonosis of mammals, especially
heibivoies. Human infections iesult fiom con-
tact with contaminated wild and domestic
animals (heibivoies, i.e., cattle, sheep, goats,
camels, antelopes) oi animal pioducts (hides,
haii, wool, bone, meal). Human-to-human
tiansmission does not occui. Bioteiioiism
1||c |. I||ee p|o|e|u ec|e|ed |, .||u|eu|
||+|u o| 3c:|| c||c: . I||ee p|o|e|u +c|
|oe||e| |u + ,ue||||c W+, |u W||c| ||e, +|e
eudoc,|oed +ud ||+u|oc+|ed |u|o ||e c,|op|+r
o| + r+c|op|+e, W|e|e || d||up| ce||u|+| |u+|
|u +ud |uduce ce|| de+||, +||oW|u ||e |+c|e||+
|o e.+de ||e |rruue ,|er.
||o|ec||.e +u||eu (|A)
Eder+ |+c|o| (E|)
|e||+| |+c|o| (||)
||||-c | . A |u|e po|,pep||de c|+|u o|
5!5 +r|uo +c|d cou|||u o| |Wo u|uu|| ||u|ed
|, d|u|||de |||de. B|ud|u |o ||e ce|| u||+ce o|
||e |e |+||e o| ||ee |Wo u|uu|| +||oW ||e
ro|e |+||e p+|| o| ||e p|o|e|u |o peue||+|e ||e
|o| ce||. E\||+o|d|u+|||, po|eu|. I|e |e||+| doe
|o| |ur+u | +|ou| 0. o| |o\|u pe| | o|
|od,We|||. A r+|.e |e|e+e o| |o\|u |u|o ||e
|od, W||| |||e|, c+ue |e||+| uec|o| o| ||e |e+||
+ud ||.e|.
-|c |. Ie|+uop+r|u. \o|ecu|+| We|||
o| 50 ||+. || | r+de up o| |Wo p+||. + 00||+
|e+., o| Bc|+|u +ud + 50||+ |||| o| Ac|+|u.
CkAM-F0SIIIv 8ACIIIAk INFCII0NS ASS0CIAI0 WIIh
I0XIN Fk00CII0N (INI0XICAII0NS)
E||o|o,. 3c:|| c||c:
/oouo|
|+||oeue|. |o\|ured|+|ed
|o||+| o| eu||,.
'||u. cu|+ueou +||+|ou
|u|+|+||ou (Woo|o||e|' d|e+e)
|ue||ou
Cu|+ueou +u|||+\ +ccouu| |o| 95 o| +u|||+\
c+e |u uu||ed '|+|e
C||u|c+| ||ud|u o| cu|+ueou +u|||+\.
B|+c| ec|+| u||ouuded |, eder+ +ud pu|p|e
.e|c|e
+ud |op |o\|u p|oduc||ou
',,. \+||u+u| pu|u|e
|||//+++||:!:./c-|/c||c/
CIAN0S ANIhkAX (CA) |C|9 . 022
|C|0 . A22
(2001) in the United States iesulted in cases of
both cutaneous and inhalation anthiax.
Ceoraphy Anthiax zones": soil iich in
oiganic mattei and diamatic changes in cli-
mate (abundant iainfall followed by piolonged
diought). Most common in agiicultuial ie-
gions wheie it occuis in animals: South/Cential
Ameiica, Southein/Eastein Euiope, Asia, Afiica,
the Caiibbean, Middle East.
Recent outbieaks in Zimbabwe (1979-1980),
Paiaguay (1987). Accidental ielease of weap-
ons-giade anthiax spoies in Sveidlovsk (1979)
iesulted in 66 deaths.
FAIh0CNSIS
Intioduced endospoies aie phagocytosed by
maciophages, caiiied to iegional lymph nodes,
and geiminate inside the maciophages and be-
come vegetative bacteiia. The vegetative bacilli
aie ieleased fiom maciophages, multiply in the
lymphatic system, and entei the bloodstieam,
causing massive septicemia, associated with
pioduction of edema and lethal exotoxins.
Low-level geimination occuis at the piimaiy
site, iesulting in local edema and neciosis.
Gastiointestinal anthiax follows ingestion of
endospoie-contaminated meat fiom diseased
animals.
Cutaneous anthiax: Occupational exposuie
to animals oi animal pioducts. Incubation
peiiod: 1-10 days (in bioteiioiism attack);
usually no piodiome.
Inhalation anthiax: eaily symptoms iesemble
those of common cold; shoitness of bieath
and shock follow.
GI anthiax follows consumption of contami-
nated meat; associated with nausea/vomiting,
fevei, abdominal pain, diaiihea.
Sko Iesoos Pvrtu| v] Entry
Cut oi abiasion.
Nondesciipt, painless, piuiitic papule (ie-
sembling insect bite) appeais 3-5 days aftei
intioduction of endospoies.
In 1-2 days, evolves to vesicle(s) hemoi-
ihage neciosis.
Vesicles iuptuie to foim depiessed ulceis, of-
ten with local edema (Fig. 24-45), ultimately
foiming diy eschais (1-3 cm).
Satellite lesions can foim in a spoiotiichoid
pattein pioximally on edematous extiemity
(Fig. 24-45B).
Edema moie extensive on head/neck.
Also see: http://www.bt.cdc.gov/agent/anthiax/
anthiax-images/cutaneous.asp
DIstrIhutIvn Exposed sites of head, neck, ex-
tiemities.
Mucous Membraoes Oiophaiyngeal anthiax
can occui following ingestion of contaminated
meat, piesenting with ceivical edema and local
lymphadenopathy, with dysphagia and iespiia-
toiy difficulties.
Ceoera| Fodos Possible fevei and/oi
othei systemic signs. Pain is not a featuie
of cutaneous anthiax. Fevei in cutaneous
anthiax usually indicates supeiinfection of
the cutaneous lesion with stieptococci oi
staphylococci. Lymph nodes in adjacent aiea
may enlaige.
varaots Inhalation anthiax, gastiointestinal
anthiax.
Cutaneous anthiax should be consideied in
any patient with a an|ess ulcei with vesicles,
edema, without iegional lymphadenopathy, and
a histoiy of exposuie to animals oi animal
pioducts.
Fao|ess, 8|ackeoed, Necrotc schar keooa|
Iymphadeoopathy Biown iecluse spidei
bite, ecthyma, ulceioglandulai tulaiemia,
vaccinia, neciotic heipes simplex infection, oif,
glandeis.
Cu|tures Giam stain and cultuie iecom-
mended; piioi antibiotic tieatment iapidly
iendeis the site cultuie negative. Gentle sam-
pling with a moist, steiile cotton tip applicatoi
is piefeiied; the iate of positive cultuies is about
65%. Expiessing eschai fluid is not iecom-
mended because it can cause dissemination
of the pathogen. Blood cultuies with systemic
anthiax aie always positive.
8opsy Biopsy edge of lesion; examine by sil-
vei staining and immunohistochemical testing.
May facilitate systemic dissemination.
0ermatopatho|oy Nonsuppuiative neciosis
and massive edema with lymphocytic infiltiates.
Giam stain of tissue shows bacilli in deimis and
subcutaneous tissue.
0IACN0SIS
Isolation of B. an|rats fiom blood, skin le-
sions, oi iespiiatoiy secietions oi by measuiing
specific antibodies in blood of peisons with
suspected symptoms.
C0kS AN0 Fk0CN0SIS
About 20% of untieated cases of cutaneous
anthiax iesult in death.
80% of cases aie self-limiting and usually
iesolve without scaiiing.
Pain in cutaneous anthiax usually indicates
stieptococcal oi staphylococcal supeiinfec-
tion.
10% of cases of cutaneous anthiax piogiess to
systemic anthiax.
Malignant edema is a iaie complication, usu-
ally involving the head and neck and mani-
fested by seveie edema, induiation, multiple
bullae, and shock.
Inhalation anthiax is neaily always fatal.
MANACMNI
Cutaneous anthiax can be self-limited, but anti-
biotic theiapy is iecommended. Drug o[ t|ote :
Cipiofloxacin, 400 mg IV q12h, oi doxycycline,
100 mg IV q12h, is optimal. |ernaes : None.
Suigeiy foi excision of eschai is contiaindicated.
Aothrax vaccoe Indicated foi peisons at iisk
foi exposuie to anthiax spoies: laboiatoiy pei-
sonnel woiking with B. an|rats , peisons who
woik with impoited animal hides/fui if exposuie
to anthiax is possible, veteiinaiians who tiavel to
endemic aieas, militaiy peisonnel deployed to
aiea with high iisk of exposuie. Piotection against
inhalation anthiax has not been tested. Animals
aie immunized in endemic iegions.
Fostexposure Frophy|axs Doxycycline, 100 mg
twice daily, oi cipiofloxacin, 500 mg twice daily,
foi 8 weeks. Amoxicillin (thiee times/ day) foi
childien and lactating women.
FICk 24-45 Cutaoeous aothrax A +0,e+|o|d |+|re| W||| +u|||+\. . A ||+c| ec|+| +| ||e ||e o|
|uocu|+||ou W||| + ceu||+| |ero|||+|c u|ce|+||ou ou ||e ||ur| +oc|+|ed W||| r+|.e eder+ o| ||e |+ud.
8. A uodu|+| |,rp|+u||| e\|eud|u p|o\|r+||, ||or ||e p||r+|, |e|ou ou ||e ||ur|.
8
E||o|o,. C,-|c:|- !|||-c-
|uc|deuce |u |udu|||+||/ed couu|||e. e\||ere|,
|+|e.
Ep|der|c |u |o|re| 'o.|e| uu|ou |u 990.
>90,000 +||ec|ed.
|+||oeue|. |oc+||/ed |u|ec||ou c+ued |, |o\|
eu|c +ud uou|o\|eu|c ||+|u, W||c| p|oduce
e\o|o\|u.
Acu|e |u|ec||ou o| ||e |ep||+|o|, ||+c| |u| r+,
|u.o|.e +u, rucou rer||+ue o| ||u Wouud.
uu|||e |u ||e p|+|,u\, cu|+ueou d|p|||e||+ |
uoupec|||c +ud ||e d|+uo| | r+de |, cu||u|e
||or ||e Wouud
Eeu||+| o| d|+uo|.
Ieu+c|ou |+, rer||+ue +| po||+| o| eu||, |u
p|+|,u\.
'o|e |||o+|, u++| d|c|+|e, |o+|eue, r+
|+|e, |e.e|.
\,oc+|d||| c+ue +||,||r|+, |e+|| ||oc|, +ud
|e+|| |+||u|e.
|eu|op+||, uu+||, |u.o|.e c|+u|+| ue|.e ||||.
d|p|op|+, |u||ed peec|, +ud d||||cu||, |u W+|
|oW|u.
Cu||u|e cou|||r ||e d|+uo|.
||p|||e||+ |o\|u c+ue.
\,oc+|d|||
|o|,ueu||||
0||e| |o\|c ,|er|c e||ec|
kep||+|o|, d|p|||e||+ | uu+||, c+ued |,
|o\|coeu|c ( |) ||+|u.
Cu|+ueou d|p|||e||+ | ||equeu||, c+ued |,
uou|o\|coeu|c ( |-) o|+u|r.
\+cc|u+||ou. |rruu||, |o .+cc|ue W+ue o.e|
||re. |eceuu|+| |oo|e| +|e |ecorreuded.
||p|||e||+ +u|||o\|u | +.+||+||e |u ||e uu||ed
'|+|e |||ou| ||e C|C. '|ou|d |e |.eu |u +||
c+e W|e|e d|p|||e||+ | upec|ed.
E|||e| peu|c||||u, 250 r, o| e|,|||or,c|u, +/||||o
r,c|u o| c|+|||||or,c|u
I|e+| cou|+c| |o + c+e
CIAN0S 0IFhIhkIA |C|9 . 0!2
|C|0 . A!0
E||o|o,. C||! |-|c
|eu|o|o|c d|o|de|, c|+|+c|e||/ed |, |uc|e+ed
ruc|e |oue +ud p+r c+ued |, |e|+uop+
r|u, + poWe||u| p|o|e|u |o\|u e|+|o|+|ed |, C
|-|c
C |-|c po|e u|.|.e |u o|| |o| ,e+|.
\ee|+||.e ce|| p|oduce |e|+uop+r|u, W||c|
red|+|e ||ud|u |o ue|.e ce|| |ecep|o| +ud
eu||, |u|o ||ee ce|| +ud ||oc| ueu|o||+ur|||e|
|e|e+e.
'||u | po||+| o| eu||,, |u| ||e|e +|e uo ||u |e
|ou pec|||c |o| |e|+uu
|ucu|+||ou pe||od | 5 d+, |o 5 Wee|, +.e|+e
3-2 d+,.
'||e o| |u|ec||ou. +u +cu|e |uju|, (puuc|u|e Wouud,
|+ce|+||ou, +||+|ou, ||e |uju|, | uu+||, |||.|+|),
ecoud+|, |u|ec||ou o| ||e+| |u ||u |ujec||u
d|u ue ('||u popp|u)|, ||u u|ce|, +u|eue,
||o||||e, |u|u, u||c+| Wouud, c|||d|||||, +|o|
||ou, upe||u|ec||ou (+|cee, r|dd|ee+| |u|ec
||ou).
Ie|+uu +||ec| uou|rruu|/ed pe|ou, p+|||+||,
|rruu|/ed pe|ou, o| |u||, |rruu|/ed |ud|.|du
+| W|o |+|| |o r+|u|+|u +dequ+|e |rruu||, W|||
|oo|e| doe o| .+cc|ue.
A| |||. e|de||,, ueW|o|u, r||+u| Wo||e|, |ujec|
|u d|u ue|. Ac||.|||e. |+|r|u, +|deu|u, +ud
o||e| ou|doo| +c||.|||e.
C|o|+||,, |e|+uu | corrou |u +|e+ W|e|e o|| |
cu|||.+|ed, |u |u|+| +|e+, |u W+|r c||r+|e, du||u
urre| rou||, +ud +rou r+|e.
'po|e e|r|u+|e |u Wouud W||| |oW o\|d+||ou
|educ||ou po|eu||+| (de.||+||/ed ||ue, |o|e|u
|od|e, o| +c||.e |u|ec||ou).
w|||ou| |rruu|/+||ou, |e|+uu occu| p|edor|
u+u||, |u ueou+|e +ud o||e| ,ouu c|||d|eu
(+90,000 ueou+|e d|ed o| |e|+uu Wo||dW|de |u
99+).
IIANS |C|9 . 0!1
|C|0 . A!!
Iocdeoce Subacute bacteiial endocaiditis
(SBE) is now much less common because of the
decieased incidence of iheumatic heait disease;
the incidence is incieasing in the eldeily and in
injecting diug useis (IDUs), and with piosthetic
valve use.
Iempora| vo|utoo oI 0sease
tue enJotarJs iapidly damages caidiac
stiuctuies, hematogenously seeds extiacai-
diac sites, and may piogiess to death in a few
weeks.
SBE causes stiuctuial damage slowly, iaiely
causes metastatic infection, and is giadually
piogiessive unless complicated by a majoi
embolic event oi iuptuied mycotic aneu-
iysm.
to|oy Vaiies with native valve IE, piosthetic
valve IE, and endocaiditis in IDUs. IE is moie
often due to giam-positive than giam-negative
bacteiia, possibly because of diffeiences in adhei-
ence to damaged valves oi because of diffeiences
in theii susceptibility to seium-induced killing.
Communy-atqureJ nae a|e IE : Mouth,
skin, and uppei iespiiatoiy tiacts aie the
iespective piimaiy poitals foi the viiidans
stieptococci, staphylococci, and HACEK
oiganisms ( Haemo||us, tno|at||us,
|u|ec||.e eudoc+|d||| (|E), ep|, +ud ep||c
|oc| +|e .e|, e||ou ,|er|c |u|ec||ou W|||
||| +oc|+|ed ro|||d||, +ud ro||+|||, |+|e.
C||u|c+| ||ud|u +|e o||eu +cu|e |u oue| +ud
|e|+||.e|, uoupec|||c |u u+|u|e.
Cu|+ueou ||ud|u e\||ere|, |e|p|u| |u r+||u
||e co||ec| d|+uo|.
E+||, |ecou|||ou o| c||u|c+| ||ud|u, d|+uo|,
+ud |u|||+||ou o| ||e|+p, |uc|e+e ||e |||e|||ood o|
+ po|||.e ou|core.
INFCIIv N00CAk0IIIS, SFSIS, AN0 SFIIC Sh0Ck
|u|ec||ou o| ||e eudoc+|d|ur o| eud+||e||ur
A .ee|+||ou |o|r +| ||e ||e o| |E, + r+ o|
|||||u, p|+|e|e|, r|c|oco|ou|e o| r|c|oo|+u|r,
+ud |eW |u||+rr+|o|, ce||.
||-:|.- -!:c!| occu| ro| corrou|, ou.
ne+|| .+|.e. u+||.e p|o||e||c
|oWp|eu|e |de o| + .eu|||cu|+| ep|ur +| +
de|ec| ||e
\u|+| eudoc+|d|ur
C+|d|+c de.|ce.
||-:|.- -!c|-| occu| |u
A||e||o.euou |uu|
A||e||o+||e||+| |uu|
Co+|c|+||ou o| ||e +o||+
INFCIIv N00CAk0IIIS (I) |C|9 . +2
|C|0 . |!!
CarJo|aterum, E|ene||a, Knge||a ). GI tiact:
Sreotottus |os . GU tiact: enteiococci.
Nosotoma| nae a|e IE : Associated with
bacteiemia aiising fiom intiavasculai cath-
eteis; less commonly, nosocomial wound and
genitouiinaiy (GU) infection. IE complicates
6-25% of episodes of cathetei-associated
S. aureus bacteiemia.
Pros|et a|e IE : 1-5% of cases of IE. Onset
within 2 months of valve suigeiy associated
with intiaopeiative contamination oi postop-
eiative bacteiemia: coagulase staphylococcus
(CoNS), S. aureus , facultative giam-negative
bacilli, diphtheioids, fungi. Onset 2-12 months
aftei suigeiy: nosocomial (CoNS); 85% aie
MRSA; decieases to 25% iesistance >12
months aftei valve suigeiy. Onset >12 months
aftei suigeiy: similai to community-acquiied
native valve IE, i.e., stieptococci, HACEK.
IE n IDUs : Incidence inciease. 60-80% of
patients have no known pieexisting valve
lesions. Pathogen usually oiiginates in skin:
S. aureus, P. aerugnosa, and fungi. Tiicus-
pid valve (>50% of cases): S. aureus , usually
MSRA. Left-sided valve (aoitic 25%; mitial
20%): P. aerugnosa, CanJJa spp. Polymicio-
bial. Otheis: Barone||a, Sa|mone||a, Lsera .
Iraosmssoo Duiing tiansient bacteiemia:
dental pioceduies, IDU vaiious infections, in-
duced aboitions, intiauteiine contiaceptive
devices, tempoiaiy tiansvenous pacemakeis,
peicutaneous intiavenous cathetei lines, endo-
scopic pioceduies.
Croups at ksk IDUs (median age, 30-40
yeais) (estimated iisk foi IE in United States,
2-5% pei yeai), eldeily people with valve scle-
iosis, patients with intiavasculai piostheses,
those exposed to nosocomial disease, and those
undeigoing hemodialysis.
Nosocoma| odocardts Complicates 6-25%
of episodes of cathetei-associated S. aureus bac-
teiemia. Associated with catheteis and medico-
suigical pioceduies. Moitality >50%. In 1999,
37% of cases caused by CoNS.
hemoda|yss Two to thiee times moie com-
mon in hemodialysis than peiitoneal dialysis
patients. >50% of cases due to S. aureus .
FAIh0CNSIS
Bacteiial adheience to damaged valves, which
occuis duiing tiansient bacteiemia, is the pii-
maiy event. Bacteiia giow within the caidiac
lesion(s), with local extension and caidiac dam-
age. Subsequently, septic embolization occuis to
skin, kidney, spleen, biain, etc. Micioulceiations
and local inflammation (iesembling aiteiioscle-
iosis) occui in degeneiative valve lesions (occui
in up to 25% of patients >40 yeais). IDUs,
injecting impuie mateiials; piioi IE can dam-
age iight-sided valves. S. aureus is most likely
to invade caidiac tissue, iesulting in abscess
foimation. Ciiculating immune complexes may
iesult in glomeiulonephiitis, aithiitis, oi vaii-
ous mucocutaneous manifestations of vasculi-
tis. This leads to:
Constitutional symptoms associated with cy-
tokine pioduction
Damage to intiacaidiac stiuctuies
Embolization of vegetative fiagments leading
to infection/infaiction of iemote tissues
Hematogenous infection of sites duiing bac-
teiemia
Tissue injuiy due to deposited bacteiial anti-
gens
Symptoms
Fevei (80-90%)
Chills/sweats (40-75%)
Anoiexia/weight loss/malaise (25-50%)
Myalgias/aithialgias (15-30%)
Back pain (7-15%).
Ceoera| Fodos Considei IE in any patient with
fevei and heait muimui oi injecting diug use.
Heait muimui (80-85%)
New/woisened ieguigitant muimui
(80-85%)
Aiteiial emboli (10-40%)
Splenomegaly (15-50%)
Neuiologic manifestations (20-40%)
Clubbing of fingeis
IDUs: 50% of cases aie limited to tiicuspid
valve; pulmonaiy findings include cough,
pleuiitic chest pain, pulmonaiy infiltiates.
Sko Iesoos Peiipheial manifestations occui
in 2-5% (Fig. 24-46).
mbo|c Iesoos Os|er Nvdes (Table 24-5)
Palpable, tendei, and almost always in the pulp
of the fingeis distally and occasionally on the
toes. Red, hemoiihagic and infaicted. Occa-
sionally white centei. In acute IE ( S. aureus ),
may be moie inflammatoiy than in SBE. In
SBE (e.g., viiidans stiep), lesions usually moie
vasculitic than septic.
Junewuy LesIvns Red, maculai, papulai, in-
faictive, nontendei, and almost always on the
palms oi soles; usually pait of the vasculitis of
SBE (Fig. 24-46).
SeptIc Emhv|Ism Painful, hemoiihagic mac-
ules, papules, oi nodules (Fig. 24-47), usually
acial location.
Suhunguu| Sp|Inter Hemvrrhuges Septic em-
bolic phenomenon. Lineai in the mJJ|e of
the nailbed in acute IE (see Fig. 33-30). (Distal
hemoiihages aie tiaumatic.) Common in acute
S. aureus IE.
PetechIu| LesIvns Small, nonblanching, ied-
dish-biown macules. Occui on extiemities,
IA8I 24-5 Cutaneous Nanifestations and Characteristics of |nfective Endocarditis
00taoeo0s Nao|Iestat|oos Pa|pat|oo Norpho|og|c F|od|ogs
0|e| uode Ieude| E|,||er+|ou p+pu|e +ud uodu|e W||| W|||e ceu|e|,
r+, |ecore uec|o||c
l+ueW+, |e|ou |ou|eude| nero|||+|c p+pu|e
'p||u|e| |ero|||+e |ou|eude| 'u|uuu+| |ero|||+|c ||e+|
uppei chest, mucous membianes con-
junctivae (Fig. 24-48), palate]. Occui in
ciops. Fade aftei a few days (20-40%).
Rvth Spvts Retinitis septica-a white
spot in the ietina close to the optic disk,
often suiiounded by hemoiihages; also
seen in peinicious anemia, leukemia.
Gungrene v] ErtremItIes Secondaiy to
embolization.
Pustu|ur/Puru|ent PetechIue With S.
aureus.
Hemutvgenvus|y Seeded Fvcu| 1n]ec-
tIvn Skin, spleen, kidneys, skeletal sys-
tem, meninges.
Emhv|IzutIvn wIth 1n]urctIvn Ex-
tiemities, spleen, kidneys, bowel, biain.
Neurv|vgIc Cvmp|IcutIvns Embolic
stiokes, meningitis, intiacianial hemoi-
ihage, seizuies, encephalopathy.
Foi fuithei clinical images see.
Fever wth Sko Iesoos Meningococ-
cemia, DIC, acute iheumatic fevei, maiantic
endocaiditis, SLE with caidiac involvement, sys-
temic vasculitis, dyspioteinemia, atiial myxoma,
oiganizing left atiial thiombus, atheiomatous
embolism.
Imao Echo-Dopplei study demonstiates
vegetations, acute seveie mitial oi aoitic iegui-
gitation. Evidence of septic pulmonaiy emboli
suggests tiicuspid valve IE. Systemic emboliza-
tion can occui fiom aoitic oi mitial valve.
0IACN0SIS
Modified Duke ciiteiia foi diagnosis of IE aie
based on both miciobiologic data and echocai-
diogiaphic imaging.
1
Demonstiation on his-
tologic and miciobiological examination of
pathogens in vegetations.
0eIote 0aooss oI I Eithei two majoi
ciiteiia or one majoi ciiteiion and thiee minoi
ciiteiia or five minoi ciiteiia.
1
See P Moieillon, Y-A Que: Lancet 363:139, 2004.
FICk 24-46 IoIectve eodocardts,
acute: laoeway |esoos nero|||+|c,
|u|+|c|ed p+pu|e ou ||e .o|+| ||ue| |u +
p+||eu| W||| ' c- eudoc+|d|||.
FICk 24-4T Septc vascu|ts assocated wth
bacterema |e|r+| uodu|e W||| |ero|||+e +ud
uec|o| ou ||e do|ur o| + ||ue|. I|| |,pe o| |e|ou
occu| W||| |+c|e|er|+ (e.., ' c-, ouococcu)
+ud |uuer|+ (e.., Cc!!c |:c|).
Muvr CrIterIu
Positive blood cultuie
Typical miciooiganism foi IE fiom two
sepaiate blood cultuies
Viiidans stieptococci, S. |os , HACEK
gioup, S. aureus , o r
Community-acquiied enteiococci in the
absence of a piimaiy focus, or
Peisistently positive blood cultuie, defined
as iecoveiy of a miciooiganism consistent
with IE fiom:
Blood cultuies diawn 12 h apait; or
All of thiee oi a majoiity of foui oi moie
sepaiate bood cultuies, with fiist and last
diawn at least 1 h apait
Evidence of endocaidial involvement
Positive echocaidiogiam Tiansesophageal
echocaidiogiaphy (TEE) iecommended foi
assessing possible piosthetic valve endocai-
ditis oi complicated endocaiditis]
Occilating intiacaidiac mass oi valve oi
suppoiting stiuctuies oi in the path of
ieguigitant jets oi in implanted mateiial,
in the absence of an alteinative anatomic
explanation, or
Abscess, or
New paitial dehiscence of piosthetic
valve, or
New valvulai ieguigitation (inciease oi
change in pieexisting muimui not suf-
ficient)
MInvr CrIterIu
Piedisposition: piedisposing heait condition
oi injection diug use
Fevei 38.0C ( 100.4F)
Valvulai phenomena: majoi aiteiial emboli,
septic pulmonaiy infaicts, mycotic aneu-
iysms, intiacianial hemoiihage, conjunctival
hemoiihage, Janeway lesions
Immunologic phenomena: glomeiulone-
phiitis, Oslei nodes, Roth spots, iheumatoid
factoi
Miciobiologic evidence: positive blood cultuie
but not meeting majoi ciiteiion oi seiologic
evidence of active infection with oiganism
consistent with IE.
C0kS AN0 Fk0CN0SIS
Acute couise in IE is common with -hemolytic
stieptococci, S. aureus, pneumococcal infection;
also, Sa|y|otottus |ugJunenss and enteiococci
in some cases. Subacute typically occuis in IE
caused by viiidans stieptococci, enteiococci,
CoNS, HACEK. Couise vaiies with the undei-
lying caidiac disease and baseline health of the
patient, as well as with the complications that
occui. Complications: congestive heait failuie,
stioke, othei systemic embolizations, septic pul-
monaiy embolization. Aoitic valve involvement
has highei iisk of death oi need foi suigeiy.
In HIV/AIDS-infected IDUs, moitality iises
inveisely to the CD4 count.
FICk 24-48 IoIectve eodocar-
dts, acute: subcoojuoctva| hemor-
rhae 'u|ruco+| |ero|||+e o| ||e
|oWe| e,e||d |u +u e|de||, d|+|e||c W|||
eu|e|ococc+| eudoc+|d|||, p||u|e| |ero|
||+e |u ||e r|dpo|||ou o| ||e u+|||ed
+ud l+ueW+, |e|ou We|e +|o p|eeu|
ou ||e .o|+| ||ue|. |u|ec||ou |o||oWed
u|oep|.
'ep| +ud ep||c |oc| cou|||u|e ||e |u||+rr+|o|,
|epoue |o r|c|o||+| |u.+|ou, r+u||e||u W|||
+u e\||ere|, W|de |+ue o| c||u|c+| ||ud|u. |e.e|
o| |,po||e|r|+, |+c|,pue+, |+c|,c+|d|+.
'pec||ur o| c||u|c+| ||ud|u. |+c|e|er|+, ep||
cer|+, ,|er|c |u||+rr+|o|, |epoue ,ud|ore
('|k'), ep|, e.e|e ep| (|r||+| |o 'ep|
,ud|ore ), ep||c |oc|, |e||+c|o|, ep||c |oc|,
ru|||p|eo|+u d,|uuc||ou ,ud|ore (\0|')
'|k'. |u|ec||ou o| uou|u|ec||ou e||o|o,
|e.e| o| |,po||e|r|+
|eu|oc,|o| o| |eu|opeu|+
I+c|,pue+
I+c|,c+|d|+
E+||, ep| | |e.e||||e, ep||c |oc| |+ |||
ro|||d||,.
',,. B|ood po|ou|u
SFSIS AN0 SFIIC Sh0Ck |C|9 . 995.9
|C| . A+0
Ae oI 0oset Foi N. menngJs (NM), high-
est incidence in childien aged 6 months to 3
yeais (peak, 6-12 months); lowest in peisons
>20 yeais.
Iocdeoce In the United States; 750,000 cases
annually, with 210,000 deaths. Two-thiids of
cases occui in patients hospitalized foi othei
MANACMNI
Frophy|axs Identify patients at iisk, pioce-
duies that might piovoke bacteiemia, and the
most effective piophylactic iegimen. Balance
between iisk of adveise effects of piophylaxis
and of developing disease.
Ireatmeot Depends on multidisciplinaiy
appioach, involving specialists in infectious
disease, caidiologists, and caidiac suigeons.
1
Cuie of IE iequiies eiadication of all miciobes
fiom vegetation(s). Miciobicidal diug iegi-
mens must pioduce high enough concentia-
tions foi long enough duiation to steiilize
vegetation(s).
Aotmcroba| Iherapy Appiopiiate IV anti-
biotic theiapy, depending on the sensitivity of
the infecting oiganism.
Surery Most common indication, congestive
heait failuie. Valve ieplacement.
illnesses. Incieasing incidence in the United
States attiibutable to aging population, with
incieasing longevity of patients with chionic
diseases.
to|oy See Table 24-6.
ksk Croups Those with influenza A viius
infection; absence of spleen oi functional asple-
nia; alcoholism; complement deficiency, espe-
cially impaiied alteinative pathway activation.
IA8I 24-6 Nicrooranisms |nvo|ved in Episodes of Severe Sepsis at Eiht Academic Nedica| Centers
p|sodes w|th p|sodes w|th
8|oodstream 0oc0meoted |oIect|oo Tota|
|oIect|oo, b0t ho 8|oodstream p|sodes,
N|croorgao|sms % ( = 436) |oIect|oo, % ( = 430) % ( = 866)
C|+rue+||.e |+c|e||+
c
!5 ++ +0
C|+rpo|||.e |+c|e||+
|
+0 2+ !
|uu| 1 5 o
|o|,r|c|o||+| 2 o
C|+|c p+||oeu
:

c
Eu|e|o|+c|e||+ce+e, peudorou+d, |c-|| pp., o||e| |+rue+||.e |+c|e||+.
|
'|c|,|::: c-, co+u|+eue+||.e |+p|,|ococc|, eu|e|ococc|, '|-|::: -c-, o||e| ||ep|ococc|, o||e| |+rpo|||.e |+c|e||+.
:
'uc| + |--c -|!, ' -c- | ||-cc-, +ud '|-|::: ,--.
'0||C| Ad+p|ed ||or KE '+ud e| +|. Ep|der|o|o, o| ep| ,ud|ore |u 3 +c+der|c red|c+| ceu|e|. lA\A 213.2!+, 991
ksk Factors Incieasing age and pieexisting
comoibidity. Widespiead use of antimiciobial
agents, immunosuppiessive diugs, indwelling
cathetei and mechanical devices, mechanical
ventilation. HIV/AIDS. Malnutiition.
Cram-negae |at||ary |aterema: Diabetes
mellitus, lymphopiolifeiative disease, ciiiho-
sis, buins, invasive pioceduies oi devices,
tieatment with diugs that cause neutiope-
nia.
Cram-ose |aterema: Vasculai catheteii-
zation, piesence of indwelling mechanical
devices, buins, IDU.
Fungema: Immunosuppiessed patients with
neutiopenia, often aftei bioad-spectium an-
timiciobial theiapy.
ksk Factors Ior Severe Sepss o Fateots wth
8acterema Age (>50 yeais) and piimaiy pul-
monaiy, abdominal, oi neuiomeningeal site of
infection.
FAIh0CNSIS
Septic iesponse tiiggeied when miciooigan-
isms spiead fiom skin oi GI tiact into con-
tiguous tissues. Localized infection may then
lead to bacteiemia oi fungemia. Miciobes can
also be intioduced into bloodstieam diiectly
fiom such ioutes as venous access lines. In
some cases, howevei, no piimaiy site of in-
fection is appaient. Septic iesponse occuis
when invading miciobes have ciicumvented
host`s innate and acquiied immune defenses.
Lipopolysacchaiide (LPS) (endotoxin) is the
most potent giam-negative bacteiial signal
molecule. Septic iesponse involves complex
inteiaction among miciobial signal molecules,
leukocytes, humoial mediatois, and vasculai
endothelium. Many of the chaiacteiistics of
sepsis (fevei, tachycaidia, tachypnea, leuko-
cytosis, myalgias, somnolence) aie pioduced
by ielease of TNF. Intiavenous fibiin depo-
sition, thiombosis, and DIC aie impoitant
featuies of septic iesponse. C5a and othei
pioducts of complement activation may pio-
mote neutiophil ieactions such as chemotaxis,
aggiegation, degianulation, and oxygen-iadi-
cal pioduction. The undeilying mechanism
of tissue damage is widespiead vasculai en-
dothelial injuiy, with fluid extiavasation and
miciothiombosis that deciease oxygen and
substiate utilization by affected tissues. Nitiic
oxide is a mediatoi of septic shock.
The majoiity of patients expeiience septic ie-
sponse supeiimposed on undeilying illness and
piimaiy infection. Fevei may be absent in
neonates, the eldeily, those with uiemia, and
alcoholics.
Sko Iesoos
Cutaneous infections as souice of sepsis
Pyodeimas
Soft tissue infections
Wounds
Specific skin lesions
Exan|em: See meningococcemia and Rocky
Mountain spotted fevei.
Peet|ae : Cutaneous/oiophaiyngeal lo-
cation suggests meningococcal infection;
less commonly H. n[|uen:ae . In patient
with tick bite living in endemic aiea, Rocky
Mountain spotted fevei (RMSF).
Et|yma gangrenosum: See Fig. 24-29, B.
P. aerugnosa most commonly; also ero-
monas |yJro||a.
Hemorr|agt |u||ous |esons: V|ro u|-
n[tus in patient (diabetes mellitus, livei
disease) with histoiy of eating iaw oysteis
(Fig. 24-30); Canotyo|aga tanmorsus
oi C. tynoJegm following dog bite (Fig.
24-49).
Geneialized eiythema due to toxins, e.g., TSS
Findings with hypotension
Extiemities cool, aciocyanosis
Ischemic neciosis of peiipheial tissue, most
often digits (Fig. 24-49).
Findings with DIC and puipuia fulminans
(see Section 19).
Ceoera| xamoatoo
Fever May be absent in neonates, eldeily pa-
tients, peisons with uiemia, alcoholism.
CI MaoIestatoos Nausea, vomiting, diaiihea,
ileus; stiess ulcei. Livei; cholestatic jaundice;
hepatocellulai/canaliculai dysfunction. Pio-
longed hypotension: acute hepatic injuiy;
ischemic bowel neciosis.
Iess Commoo MaoIestatoos Aithiitis
(5-10%), pneumonia, sinusitis, otitis media,

conjunctivitis, endophthalmitis, endocaiditis,
peiicaiditis, uiethiitis, endometiitis.
Sepss aod Shock Acute bacteiemia and en-
docaiditis, acute hypeisensitivity" vasculitis,
enteioviial infections, RMSF, TSS.
0rect Mcroscopy Examine skin/mucosal sui-
faces. Giam stain of mateiial fiom piimaiy site
of infection oi fiom infected cutaneous lesions.
In oveiwhelming infection (pneumococcal
sepsis in splenectomized patient oi fulminant
meningococcemia), miciooiganisms can be
seen in buffy coat. In meningococcemia, sciap-
ings fiom nodulai lesions show giam-negative
diplococci.
hemato|oy Leukocytosis with left shift,
thiombocytopenia; latei, leukopenia. Neu-
tiophils contain toxic gianules, Dhle bodies,
cytoplasmic vacuoles.
C|otto Studes Piolonged thiombin time,
decieased fibiinogen, piesence of D-dimeis.
Chemstry Hypeibiliiubinemia, incieased cie-
atinine.
Cu|tures B|vvd Obtain at least two blood
samples (fiom diffeient venipunctuie sites)
foi cultuie. Giam-negative bacteiemia is low
giade; multiple blood cultuies oi piolonged
incubation of cultuies may be necessaiy. S. au-
reus giows iapidly and is most easily detectable.
Negative blood cultuies may ieflect piioi anti-
biotic administiation, slow-giowing oi fastidi-
ous oiganisms, oi absence of miciobial invasion
of bloodstieam. Acute meningococcemia, NM
in neaily 100%; meningitis, one-thiid positive.
S|In/Sv]t TIssue Obtain cultuies fiom sites of
possible cutaneous infection.
CerehrvspInu| F|uId (CSF) Cu|ture Acute
meningococcemia, usually positive.
Cu|ture v] LesIvnu| S|In BIvpsy SpecImen Up
to 85%.
0IACN0SIS
Definitive etiologic diagnosis iequiies isolation
of miciooiganism fiom blood oi local site of
infection.
C0kS AN0 Fk0CN0SIS
Ventilation-peifusion mismatching pioduces
ARDS. Seveie deciease in systemic vasculai
iesistance iesults in geneialized maldistiibution
of blood flow, functional hypovolemia, diffuse
capillaiy leakage of intiavasculai components.
Caidiac output may intially be elevated; subse-
quent caidiac dysfunction common. Renal fail-
uie occuis due to hypotension and capillaiy
injuiy. Platelet counts low in patients with DIC;
low counts ieflect diffuse endothelial injuiy.
Appioximately 25-35% of patients with seveie
sepsis and 40-45% of those with septic shock
die within 30 days; otheis die within the ensuing
5 months.
MANACMNI
Requiies uigent measuies to tieat local infec-
tion, piovide hemodynamic and iespiiatoiy
suppoit, and eliminate offending oiganism.
Outcome depends on undeilying disease.
Freveotoo Reduce numbei of invasive pio-
ceduies, limit use of indwelling vasculai and
bladdei catheteis, ieduce incidence and duiation
of piofound neutiopenia (<500 neutiophils/mL),
aggiessively tieat localized nosocomial infections.
Surery Removal oi diainage of focal souice
of infection is essential.
Aotmcroba| Iherapy In the absence of an
obvious souice of infection, antimiciobial iegi-
men diffeis in the following types of patients:
immunocompetent adult, neutiopenic patient,
splenectomized patient, injecting diug usei,
HIV/AIDS patient. Any febiile patient with a
petechial iash should be consideied to have
N. menngJs infection; blood cultuie should
be obtained; tieatment begun without awaiting
confiimation.
Aotbotc Iherapy oI Sepss 0ue to 0ther
Mcrobes Depends on the sensitivity of the
infecting oiganism (see Table 24-2).
hemodyoamc, kespratory, Metabo|c Support
Piimaiy goal is to iestoie adequate oxygen and
substiate deliveiy to tissues. Adequate fluids
should be infused to tieat intiavasculai volume
depletion. Adienal insufficiency should be con-
sideied in patients with iefiactoiy hypotension,
fulminant meningococcemia, piioi glucocoiti-
coid use, disseminated tubeiculosis, HIV/AIDS
disease. Ventilatoi theiapy is indicated foi pio-
giessive hypoxia, hypeicapnia, neuiologic dete-
iioiation, iespiiatoiy muscle failuie.
FICk 24-49 Septc shock: schemc oecross oI acra| stes Cc:,||cc :c ep| (do
|||e) W||| p|o|oued |,po|eu|ou +ud |,pope||u|ou |eu||ed |u |u|+|c||ou o| ||ue| +ud uoe.
II0I0C
Meningococcus is a giam-negative, encapsu-
lated coccus. Only a minoiity of nasophaiyngeal
isolates cause invasive disease. 13 seiogioups,
which aie classified accoiding to antigenicity of
capsulai polysacchaiides. Five seiogioups (A,
B, C, Y, and W-135) aie iesponsible foi >90%
of cases of meningococcal disease woildwide.
Polymeiase chain ieaction (PCR) also used
foi identification of stiains associated with
outbieaks of disease. Vor|JwJe : seiogioups A,
B, C account foi most cases; merta, Euroe :
most outbieaks caused by seiogioups B, C;
sa/[rta : A, C. T|e UneJ Saes, SweJen,
Israe| : also have gioup Y.
Ceuu o| |+rue+||.e |+c|e||+, d|p|ococc| ||+|
|eer||e co||ee |e+u.
| -|! (reu|uococcu)
Co|ou|/e o|op|+|,ue+| ruco+
C||u|c+| ,ud|ore
|oc+| |u|ec||ou. reu|u|||, pe||c+|d|||, +|||||
||
\eu|uococcer|+ (reu|uococc+| ep||cer|+)
| |-c- (ouococcu)
Co|ou|/e +uoeu||+| o| o|op|+|,ue+| ruco+
C|u|c+| ,ud|ore
|oc+| |u|ec||ou. ouo|||e+
|e|.| |u||+rr+|o|, d|e+e
Couococcer|+ (ouococc+| ep||cer|+)
E||| uoup+||oeu|c pec|e o| |--c
NFI55FkI
E||o|o,. |--c -|! , ||e reu|uococ
cu
Co|ou|/e ruco+
Ep|der|o|o,. po|+d|c c+e, |u|||u||ou+| ou|
||e+|, ep|der|c
|or|u+u| p|o|u||+rr+|o|, ro|ecu|e |u ||e
reu|uococc+| ce|| W+|| | ||e eudo|o\|u o|
||poo||o+cc|+||de, couceu||+||ou o| eudo|o\|u
de|ec|ed |u ||e ||ood o| p+||eu| W||| |u|r|u+u|
reu|uococcer|+ +|e 0 |o 000|o|d |||e|
||+u ||oe |ouud |u ||e ||ood o| p+||eu| W|||
|+c|e|er|+ due |o o||e| |+rue+||.e |+c|e||+ .
C||u|c+| ,ud|ore depeudeu| ou |+|e o| |ep||c+
||ou o| r|c|o|e W||||u ||ood ||e+r.
|u|e|r|||eu| |+c|e|er|+. c||ou|c reu|uococ
cer|+
'|oW |ep||c+||ou eed .+||ou o|+u. reu|u
e, pe||c+|d|ur +ud |+|e jo|u|
\o|e |+p|d |ep||c+||ou c+ue |+p|d |u||+rr+
|o|, |epoue. reu|uococcer|+ W||| |oc|
+ud de+||
C||u|c+| ||ud|u o| + .+||+||e pec||ur o|.
\eu|u|||, pe||c+|d|||, +|||||||
\eu|uococcer|+ +ud |oc|, c|+|+c|e||||c |e
ro|||+|c ||u |e|ou
\eu|u||| +ud reu|uococcer|+
\eu|uococcer|+ +ud reu|u||| +|e +oc|+|ed
W||| ||| ro|||d||, +ud ro||+|||, .
|u|r|u+u| reu|uococcer|+ | ||e ro| |+p|d|,
|e||+| |o|r o| ep||c |oc|. ||||e| ||or ro|
o||e| |o|r o| ep||c |oc| |, ||e p|or|ueuce
o| |ero|||+|c ||u |e|ou (pe|ec||+e, pu|pu|+)
+ud ||e cou||eu| de.e|opreu| o| ||C.
||e.eu||ou. .+cc|u+||ou p+|||+||, e||ec||.e
NFI55FkI MFNIN6III0I5 INFCII0N |C|9 . 0!o.9
|C|0 . A!9
Confined to humans; natuial habitat is na-
sophaiynx. Nasophaiyngeal caiiiei iate: in
nonepidemic peiiods, 10%; in closed popula-
tions, up to 60-80%. Caiiiage peisists foi a
few months. Invasive infection usually occuis
within fiist few days of caiiiage, befoie develop-
ment of piotective antibodies.
FI0MI0I0C
Ae oI 0oset Highest incidence is in childien
ages 6 months to 3 yeais (peak, 6-12 months)
(piotective antibodies have not yet developed).
Incieasing in adolescents and young adults;
28% of cases aie in 12- to 29-yeai olds.
Iraosmssoo Peison-to-peison thiough in-
halation of dioplets of aeiosolized infected
nasophaiyngeal secietions; diiect oi indiiect
oial contact.
Seasoo Highest incidence in midwintei, eaily
spiing; lowest in midsummei.
0emoraphy Woildwide; 300,000-500,000
cases annually. Majoi outbieaks iepoited in
Afiica, China, South Ameiica. Afiican savannah
fiom Ethiopia to east of Senegal: meningitis
belt." Laigest epidemic occuiied in 1996-1997
in sub-Sahaian Afiica: >300,000 cases with
30,000 deaths caused by seiogioup A. In the
United States 2500-3000 cases annually.
ksk Factors Ior Co|ootatoo aod Meooococca|
0sease Residence in household of peison
who has meningococcal disease oi is a caiiiei;
household oi institutional ciowding; exposuie
to tobacco smoke; iecent viial uppei iespiiatoiy
infection (URI).
Black iace, low socioeconomic status,
militaiy ieciuits, college fieshmen living
in doimitoiies. Peisons with deficiency of
antibody-dependent complement-mediated
immune lysis, functional/anatomic asplenia,
piopeidin deficiency, deficiency of teiminal
complement components, HIV/AIDS aie most
susceptible.
Iocdeoce Occuis woildwide as (1) isolated
(spoiadic) case; (2) institution- oi community-
based outbieaks, (3) laige epidemics.
FAIh0CNSIS
Meningococci colonizing mucosa of the uppei
iespiiatoiy tiact aie inteinalized by nonciliated
mucosal cells and can tiaveise to submucosa
and to blood vessels. The clinical syndiome
depends on the iate of multiplication within
the bloodstieam:
Slow ieplication: seeds local sites such as
meninges, joint, oi peiicaidium. Host inflam-
matoiy ieaction limited to seed site.
Moie iapid ieplication: fulminant menin-
gococcemia occuis with hemoiihagic skin
lesions (petechiae, puipuia), DIC, and shock.
Host inflammatoiy iesponse in blood signs of
sepsis and shock.
Fulminant meningococcemia is the most
iapidly lethal foim of septic shock expeiienced
by humans. It diffeis fiom most othei foims
of septic shock by the piominence of hemoi-
ihagic skin lesions (petechiae, puipuia) and
the consistent development of DIC (see Sec-
tion 19). Extiemely high blood levels of both
pioinflammatoiy mediatois (TNF, IL-1, intei-
feion, IL-8) and anti-inflammatoiy mediatois
(IL-1 ieceptoi antagonist, soluble IL-1 iecep-
tois, soluble TNF ieceptois, and IL-10).
Meooococcema Fevei, chills, nausea, vomit-
ing, myalgias; piostiation. Stupoi, hemoiihagic
lesions, hypotension within a few houis of onset
of symptoms (fulminant disease).
Meoots Headache; stiffness of neck; al-
teied mental status; agitated, maniacal behavioi.
Some patients (10-30%) with meningococcal
disease have both meningococcemia and men-
ingitis.
Sko Iesoos oI Fu|moaot Meooococcema
Eaily exanthem
Occuis soon aftei onset of disease in 75% of
cases; pink, 2- to 10-mm macules/papules
Spaisely distiibuted on tiunk/lowei ex-
tiemities as well as face, aims (Fig. 24-50).
Mucous membianes (palate, conjuncti-
vae)
Petechiae may coalesce into hemoiihagic
bullae oi may undeigo neciosis and ulcei-
ate.
Latei lesions
Petechiae appeai in centei of macules.
Lesions become puipuiic/hemoiihagic
within houis.
In seveie cases, petechiae may become con-
fluent and develop into hemoiihagic bullae
with extensive ulceiations.
Fu|mnan : puipuia, ecchymoses, and con-
fluent, often bizaiie-shaped, giayish to
black neciosis (puipuia fulminans) associ-
ated with DIC in fulminant disease (Fig.
24-51) (see also Section 19).
With seveie coagulopathy, ischemia of ex-
tiemities and/oi digits occuis, often with a
shaip line of demaication between noimal
and ischemic tissue.
Ceoera| xamoatoo
High fevei, tachypnea, tachycaidia, mild hypo-
tension. Patient appeais acutely ill with maiked
piostiation.
Menngs: 50-88% of patients with menin-
gococcemia develop meningitis. Sudden onset
of fevei, signs of meningeal iiiitation. Signs
of incieased intiacianial piessuie (bulging
fontanelle in infant).
Menngotottema: Rapid piogiession/ovei-
whelming chaiactei. Occuis in 10-20% of

cases of meningococcal disease; chaiacteiized
by development of shock, DIC, hypoten-
sion, peiipheial vasoconstiiction with cold
cyanotic extiemities, and multioigan failuie.
Peiipheial gangiene may occui, iequiiing
amputation in those who suivive.
Com|taons: Inteicuiient infections, CNS
damage. Patients who iecovei may have
neciosis of skin, distal extiemities, tips of
eais/nose.
Less tommon man[esaons: Aithiitis
(5-10%), pneumonia, sinusitis, otitis media,
conjuctivitis, endophthalmitis, endocaiditis,
peiicaiditis, uiethiitis, endometiitis.
C|ront menngotottema: Raie. Episodic fevei,
iash (macules, papules, petechial), aithialgias.
Duiation: weeks to months. Splenomegaly. If
untieated, meningitis, fulminant meningo-
coccemia, oi endocaiditis may occui.
Acute Meooococcema aod Meoots Gono-
coccemia, infectious endocaiditis, acute hypei-
sensitivity, vasculitis, enteioviial infections,
RMSF, endemic typhus.
0rect Mcroscopy Pus fiom nodulai lesions
shows giam-negative diplococci. In fulminant
meningococcemia, meningococci can be seen
in buffy coat. In 85% of cases of meningitis,
meningococci can be seen in CSF.
C|otto Studes Piolonged thiombin time,
decieased fibiinogen, piesence of D-dimeis.
Cu|tures B|vvd Acute meningococcemia,
meningococci in neaily 100%; meningitis, one-
thiid positive.
CSF Acute meningococcemia, usually posi-
tive.
0IACN0SIS
Definitive etiologic diagnosis iequiies isolation
of meningococci fiom blood oi local site of
infection.
C0kS AN0 Fk0CN0SIS
Case fatality iate foi fulminant meningococ-
cemia is 20-40%; foi meningococcal menin-
gitis, 3-10%. In Afiica, moitality is 10%, but
many patients die befoie ieaching hospital.
In 1996, an epidemic in the meningitis belt
iepoited 160,000 cases with 16,000 deaths.
MANACMNI
Immuotatoo <20% of meningococci isolates
fiom associated disease belong to seiogioups foi
which vaccines aie available: A, C, W-135, Y.
Frophy|axs oI Cootacts oI Frmary Cases
Rifampin, minocycline, oi cipiofloxacin.
FICk 24-50 Acute meooococcema:
ear|y exaothem ||c|e|e, p|u||opu|p|e
r+cu|e +ud p+pu|e + We|| + pu|pu|+ ou ||e
|+ce o| ||| ,ouu c|||d. I|ee |e|ou |ep|e
eu| e+||, d|er|u+|ed |u||+.+cu|+| co+u|+
||ou W||| || cu|+ueou r+u||e|+||ou, pu|pu|+
|u|r|u+u.
Aotmcroba| Iherapy Any febiile patient
with a petechial iash should be consideied
to have meningococcal infection; blood cul-
tuie should be obtained and tieatment be-
gun without awaiting confiimation.
Acute MenIngvcvccemIu Thiid-geneia-
tion cephalospoiin: cefotaxime (2 g IV q8h)
oi ceftiiaxone (1 g IV q12h). |ernae :
Penicillin G (4 million U IV q4h). C||oram-
|ento| in penicillin-alleigic individuals.
hemodyoamc, kespratory, Metabo|c Sup-
port Piimaiy goal is to iestoie adequate
oxygen and substiate deliveiy to tissues.
Adequate fluids should be infused to tieat
intiavasculai volume depletion.
FICk 24-51 Acute meooococcema:
purpura Iu|moaos \+p|||e, |+,|o||+c|
+|e+ o| cu|+ueou |u|+|c||ou o| ||e |e |u + c|||d
W||| |\ reu|u||| +ud d|er|u+|ed |u||+.+cu
|+| co+u|+||ou W||| pu|pu|+ |u|r|u+u.
to|oy
N. gonorr|oeae , the gonococcus.
Humans aie the only natuial ieseivoii of the
oiganism.
Stiains that cause DGI tend to cause minimal
genital inflammation.
In the United States, these stiains have oc-
cuiied infiequently duiing the past decade.
Ae oI 0oset Young, sexually active. In new-
boins, conjunctivitis.
E||o|o,. | |-c- ||e ouococcu
Co|ou|/e ruco+. o|op|+|,u\, +uoeu||+|
Ep|der|o|o,. e\u+||, ||+ur|||ed |u|ec||ou ('I|).
'|+|e c||u|c+| pec||ur o| C |c:|c| , ,rp
|or +|e uu+||, ro|e e.e|e W||| ouococc+|
|u|ec||ou.
',rp|or
\+|e. u|e|||||| o||eu ,rp|or+||c
|er+|e. ce|.|c||| o||eu +,rp|or+||c, p+|u
W||| deepe| |u|ec||ou
|eW|o|u. coujuuc||.|||
C||u|c+| ||ud|u.
|oc+| |u|ec||ou. u|e||||||, ce|.|c|||, p|oc||||,
p|+|,u|||, coujuuc||.|||
|u.+|.e ||ue |u|ec||ou. pe|.|c |u||+rr+|o|,
d|e+e (|||)
B|ood||e+r |u.+|ou. ouococcer|+ W||| eed
|u o| ru|||p|e ||e, |.e., jo|u| +ud ||u |eu|||u
|u d|er|u+|ed ouococc+| |u|ec||ou (|C|)
Corp||c+||ou
Iu|+| c+|||u, |u|e||||||,, ec|op|c p|eu+uc,
'ecoud+|, |u|ec||ou o| p|ee\|||u de|r+|oe
(|rpe|||u|/+||ou, o| ecoud+|, |u|ec||ou)
NFI55FkI 60N0kkB0FF INFCII0NS
Sex
Young females; males who have sex with
males.
Symptomatic infection moie common in
males.
Phaiyngeal and anoiectal in homosexual
males.
kace In the United States; highest incidence
in blacks, lowest in those of Asian/Pacific Island
descent.
Iraosmssoo
Sexua||y , fiom paitnei who eithei is asympto-
matic oi has minimal symptoms.
Neonae exposed to infected secietions in
biith canal.
About 1% of patients with untieated mu-
cosal gonococcal infection develop DGI (see
below). Gonoiihea may enhance HIV/AIDS
tiansmission.
Co-oIectoo Up to 40% of peisons co-infected
with C. rat|omas . Gonoiihea enhances tians-
mission as well as acquisition of HIV/AIDS.
0emoraphy Woildwide. In Afiica, median
pievalence of gonoiihea in piegnant women is
10%. Incidence of DGI vaiies with local incidence
of DGI stiains of gonococcus (see below).
Iocdeoce Highest in developing countiies.
Pievalence of DGI in piegnant women: 10% in
Afiica; 5% in Latin Ameiica; 4% in Asia.
FAIh0CNSIS
Gonococcus has affinity foi columnai epithe-
lium; stiatified and squamous epithelia aie
moie iesistant to attack.
Epithelium is penetiated between epithe-
lial cells, causing a submucosal inflam-
mation with polymoiphonucleai (PMN)
leukocyte ieaction with iesultant puiulent
dischaige.
Stiains of gonococcus that cause DGI tend to
cause little genital inflammation and theieby
escape detection. Most signs and symptoms
of DGI aie manifestations of immune com-
plex foimation and deposition.
Multiple episodes of DGI may be associated
with abnoimality of teiminal complement
component factois (see below).
IA80kAI0k SI0IS
See below: N. gonorr|oeae.
MANACMNI
See Table 30-4 and pages 653-654. Foi Centeis
foi Disease Contiol and Pievention (CDC)
STD tieatment guidelines-2002, see http://-
www.cdc. gov/mmwi/pieview/mmwihtml/
ii5106 a1.htm
| |-c- |u|ec| rucocu|+ueou u||+ce
o| ||e |oWe| eu||ou||u+|, ||+c|, +uu, +ud |ec|ur
+ud ||e o|op|+|,u\.
I|e ro| corrou p|eeu|+||ou |u r+|e | +
pu|u|eu| u|e|||+| d|c|+|e.
|u |er+|e, ce|.|c+| |u|ec||ou | ro| corrou +ud
| o||eu +,rp|or+||c
|| uu||e+|ed, |u|ec||ou c+u p|e+d |o deepe| ||uc
|u|e W||| +|ce |o|r+||ou +ud d|er|u+|ed
ouococc+| |u|ec||ou (|C|) (ee 'ec||ou 2+)
',,. C|+p, ||euuo|||+|+, ||euuo|||e+.
NFI55FkI 60N0kkB0FF: I0CAI INFCII0NS
(C0N0kkhA) |C|9 . 093
|C|0 . A5+
Iocubatoo Ferod
Ma|es : 90% of males develop uiethiitis within
5 days of exposuie.
Fema|es : Usually >14 days when sympto-
matic; howevei, up to 75% of women aie
asymptomatic.
Sko Symptoms
Ure|ra : dischaige, dysuiia.
Vagna : dischaige; deep pelvic oi lumbai pain.
nus/retum : Copious puiulent anal dis-
chaige; buining oi stinging pain on def-
ecation; tenesmus; blood in/on stool.
Oro|arynx : Mild soie thioat.
Mucocutaoeous Fodos
xteroa| Ceota|a
Mu|es
Uiethial dischaige ianging fiom scanty
and cleai to puiulent and copious (Figs.
24-52, 24-53).
EJema : meatus, piepuce, oi penis.
Balanoposthitis with subpieputial dis-
chaige in unciicumcised men; balanitis in
ciicumcised men.
Folliculitis oi cellulitis of thigh oi abdo-
men.
Deeer srutures : Piostatitis, epididymitis,
vesiculitis, cystitis.
Femu|es
Peiiuiethial edema, uiethiitis.
Puiulent dischaige fiom ceivix but no
vaginitis.
In piepubescent females, vulvovaginitis.
Baitholin abscess.
Deeer srutures : Pelvic inflammatoiy dis-
ease (PID) with signs of peiitonitis, en-
doceivicitis, endosalpingitis, endometiitis.
Aoorectum
With ieceptive anal inteicouise, pioctitis
with pain and puiulent dischaige.
In female, can spiead fiom ceivicitis.
Fharyox
Occuis secondaiy to oial-genital sexual
exposuie.
In females and homosexual males, phai-
yngitis with eiythema.
Always coexists with genital infection.
yes
Conjunctivitis, swollen eyelid, seveie
hypeiemia, chemosis, piofuse puiulent
dischaige; iaiely, coineal ulcei and peifo-
iation.
In newboin, oiganism is tiansmitted as
newboin passes thiough biith canal.
Usually occuis in the absence of genital
infection, copious puiulent conjunctival
dischaige.
Can be complicated by coineal ulceiation
and peifoiation.
Ceoera| xamoatoo
DIssemInuted Gvnvcvccu| 1n]ectIvn See
below.
rethrts Genital heipes with uiethiitis, C.
rat|omas uiethiitis, Urea|asma urea|ytum
uiethiitis, Trt|omonas agna|s uiethiitis, Re-
itei`s syndiome.
Cervcts C. rat|omas oi HSV ceivicitis.
FICk 24-52 Nessero yeoerrheeoe: Cram
stao \u|||p|e, |+rue+||.e d|p|ococc| W||||u po|,ro|
p|ouuc|e+| |eu|oc,|e + We|| + |u ||e e\||+ce||u|+| +|e+
o| + re+| ||or + u|e|||+| d|c|+|e.
FICk 24-53 Cooorrhea |u|u|eu|, c|e+r, u|e|||+|
d|c|+|e ||or ||e d||+| u|e|||+ o| + r+|e.
Cram Stao Giam-negative diplococci
intiacellulaily in polymoiphonucleai leuko-
cytes in exudate (Fig. 24-52) .
Cu|ture Isolation on gonococcal-selective
media, i.e., chocolatized blood agai, Maitin-
Lewis medium, Thayei-Maitin medium. Anti-
miciobial susceptibility testing impoitant due
to iesistant stiains.
SpecImen Cv||ectIvn SItes Heerosexua| men :
Uiethia, oiophaiynx. Homosexua| men : Uie-
thia, iectum, oiophaiynx. Vomen : Ceivix, iec-
tum, oiophaiynx. DCI : Blood.
Sero|oc Iests None available foi gonoiihea.
All patients should have a Seiologic test foi
syphilis and should be offeied HIV/AIDS test-
ing.
0IACN0SIS
Clinical suspicion, confiimed by laboiatoiy
findings, i.e., piesumptively by identifying
giam-negative diplococci intiacellulaily in
PMNs in smeais, confiimed by cultuie.
C0kS AN0 Fk0CN0SIS
Most infections among men pioduce symp-
toms that cause the peison to seek cuiative
tieatment soon enough to pievent seiious
sequelae-but not soon enough to pievent
tiansmission to otheis.
If not tieated, complications due to ascend-
ing infection occui: Piostatitis-pain on
defecation
Epididymitis-swelling of epididymis and
pain in walking.
Cystitis.
Many infections among women do not pio-
duce iecognizable symptoms until complica-
tions such as PID occui.
PID, whethei symptomatic oi asympto-
matic, can cause tubal scaiiing, leading to
infeitility oi ectopic piegnancy.
Because gonococcal infections among women
aie often asymptomatic, a piimaiy measuie
foi contiolling gonoiihea in the United States
has been scieening of high-iisk women.
DGI moie common in
Women with asymptomatic ceivical, en-
dometiial, oi tubal infection
Homosexual men with asymptomatic iectal
oi phaiyngeal gonoiihea.
MANACMNI
See CDC updated iecommended iegimens:
|.//www.tJt.go/sJ/reamen
See Table 30-4.
See pages 653-654 foi management of dissemi-
nated gonococcal infections.
Iocubatoo Ferod 7-30 days of mucosal infec-
tion (iange, fiom a few days to 1 yeai). Vaiies
with host factois such as menstiuation, inva-
siveness of infecting oiganism.
Frodrome Fevei, anoiexia, malaise, shaking
chills, polyaithialgias (knees, elbows, distal
joints).
|C| | + ,|er|c |u|ec||ou ||+| |o||oW ||e |e
r+|oeuou d|er|u+||ou o| ouococcu ||or
|u|ec|ed ruco+| ||e |o ||u, |euo,uo.|ur, +ud
jo|u|.
C|+|+c|e||/ed |, |e.e|, pe|ec||+| o| pu|u|+| +c|+|
|e|ou, +,rre|||c +||||+||+, |euo,uo.|||, o|
ep||c +|||||||.
0cc+|ou+||, corp||c+|ed |, pe|||ep+|||| +ud,
|+|e|,, eudoc+|d||| o| reu|u|||.
',, . Couococcer|+, ouococc+| +|||||
||-de|r+|||| ,ud|ore
0ISSMINAI0 C0N0C0CCAI INFCII0N |C|9 . 093.39
0ther Factors Recuiiing symptoms aiound
menses, migiatoiy polyaithialgias.
Mucocutaoeous Fodos
1- to 5-mm eiythematous macules evolving
to hemoiihagic pustules (Fig. 24-54) within
24-48 h in 75% of cases.
Centeis at times hemoiihagic/neciotic, 5-40
in numbei.
Raiely, laige hemoiihagic bullae.
Dsr|uon: Acial (Fig. 24-54), aims moie
often than legs, neai small joints of hands oi
feet. Difficult to detect in black patients; look
in webspaces. Face spaied.
Mutous mem|ranes: Usually asymptomatic
colonization of oiophaiynx, uiethia, anoiec-
tum, endometiium.
Fevei 38C to 39C usual. Seveiity vaiies:
DGI with skin lesions alone
Classic DGI with skin lesions and tenosynovi-
tis
DGI with septic aithiitis
DGI with metastatic infection at othei sites.
TenvsynvvItIs
Common
Single oi few sites, acially
Extensoi/flexoi tendons and sheaths of hands/
feet
Eiythema, tendeiness, swelling along tendon
sheath aggiavated by moving tendon
SeptIc ArthrItIs
Red, hot, tendei with effusion; asymmetiic
Most commonly involved: knee, wiist, ankle,
elbow, metacaipophalangeal/inteiphalangeal
joints of hand, shouldei, hip
Usually only one to two joints involved
Other
Hepatitis, peiihepatitis (Fitz-Hugh-Cuitis
syndiome), myopeiicaiditis, endocaiditis,
meningitis, peiihepatitis
Raiely, pneumonitis, ARDS, osteomyelitis
Scaot, Acra|, hemorrhac Fustu|es Bac-
teiemia: meningococcemia, othei bacteiemias,
endocaiditis.
Ieoosyoovts[Arthrts Infectious aithiitis, in-
fectious tenosynovitis, ieactive syndiome, pso-
iiatic aithiitis, SLE.
0ermatopatho|oy Immunofluoiescence of
skin lesion biopsy shows gonococci in 60%.
Cram Stao Fiom the male uiethia oi ceivix,
may show gonococci.
Cu|ture Mucosal sites yield 80-90% positive
cultuies. Skin biopsy: 5% chance of positive
cultuie; joint fluid, blood also low yield.
0IACN0SIS
Made on clinical ciiteiia, confiimed by cultuie
of gonococcus fiom mucosal sites.
C0kS AN0 Fk0CN0SIS
Untieated, skin/joint lesions often giadually
iesolve; endocaiditis usually fatal.
MANACMNI
kecorreuded |e|reu
Ce||||+\oue, |\ o| |\ e.e|, 2+ |
A||e|u+||.e |e|reu
Ce|o|+\|re, |\ e.e|, 3 |,
Ce|||/o\|re, |\ e.e|, 3 |,
C|p|o||o\+c|u, +00 r |\ e.e|, 2 |,
0||o\+c|u, +00 r |\ e.e|, 2 |,
|e.o||o\+c|u, 250 r |\ d+||,,
'pec||uor,c|u, 2 |\ e.e|, 2 |
FICk 24-54 0ssemoated ooococca|
oIectoo nero|||+|c, p+|u|u| pu|u|e ou e|,
||er+|ou |+e ou ||e p+|r +ud ||e ||ue| o| ||e
o||e| |+ud. I|ee |e|ou occu| +| +c|+| ||e +ud +|e
|eW |u uur|e|.
All of the pieceding iegimens should be con-
tinued foi 24-48 h aftei impiovement begins,
at which time theiapy may be switched to one
of the following iegimens to complete at least 1
week of antimiciobial theiapy:
Ce||\|re, +00 r o|+||, |W|ce d+||,,
C|p|o||o\+c|u, 500 r o|+||, |W|ce d+||,,
0||o\+c|u, +00 r o|+||, |W|ce d+||,,
|e.o||o\+c|u, 500 r o|+||, ouce d+||,
FAIh0CNSIS
Barone||a (Table 24-7) species cause vascu-
lai piolifeiation (angiogenesis), histologically
iesembling Kaposi saicoma: B. |at||[orms
causes veiiuga peiuana, B. |ense|ae and B. qun-
ana cause bacillaiy angiomatosis. B. |ense|ae is
associated with hepatosplenic disease (peliosis
CkAM-NCAIIv INFCII0NS
E||o|o,. 3c|-||c pp., ||u, |+rue+||.e |+
c|||| ||+| c+u +d|e|e |o +ud |u.+de r+rr+||+u
ce|| uc| + eudo||e||+| ce|| +ud e|,|||oc,|e
(I+||e 2+1).
I|+ur||ou |,. c+| c|+|c| o| |||e, |od, |oue,
+ud||, |||e
\+u||e|+||ou .+|, W||| ||e |rruue |+|u o| ||e
|o|
3 |--|c-
|rruuocorpe|eu| |o|. c+|c|+|c| d|e+e
(C'|)
n|\/A||'. |+c|||+|, +u|or+|o| (BA)
3 |c:|||
|ou|rruue, uou|e|deu| o| euder|c +|e+.
0|o,+ |e.e| W||| e.e|e |e||||e |||ue, p|o
|ouud +uer|+
|rruue pe|ou +||e| cou.+|eceuce. .e||u+
pe|u+u+ W||| |edpu|p|e cu|+ueou |e|ou
(|e|u.|+u W+||, |eer||e +u|or+|ou |e
|ou o| BA
-:| |-.- | c+ued |, 3 |cc , p|eeu||u
+ + |e||||e ,|er|c |||ue W||| p|o|oued |+c|e|
er|+, uo cu|+ueou r+u||e|+||ou.
8kI0NFII INFCII0NS
IA8I 24-T Najor 0iseases Caused by 8drtone//d Species
0|sease 0rgao|sm 8|sk Iactor
C+|c|+|c| d|e+e 3 |--|c- C+| c|+|c| o| |||e
B+c|||+|, +u|or+|o| 3 |cc 3 |--|c- C+| c|+|c| o| |||e
B+c|||+|, pe||o| 3 |--|c- C+| c|+|c| o| |||e
I|euc| |e.e| 3 |cc nore|eue, |od, |oue |u|e|+||ou, +|co|o||r
Eudoc+|d||| 3 |cc 3 |--|c- 3 -|cc|-||c- A |o| c+|c|+|c| d|e+e +ud ||euc| |e.e|
B+||oue||o| 3 |c:||| '+ud||, |||e
'0ukCE. ||or |n 'p+c|, E |+||,, |u A' |+uc| e| +| (ed). |c |:|- | ||-c| !-!:-, 1|| ed. |eW \o||, \cC|+Wn|||, 2003.
hepatitis) and infection of lymph nodes. B.
qunana is associated with bony and subcuta-
neous lesions.
MANACMNI
See Table 24-8.
IA8I 24-8 Ireatment of Adu|ts with 0isease Caused by 8a||one||a Species
c
0|sease Treatmeot
C+|c|+|c| d|e+e
|,rp|+deuop+||, Cou|de| +/||||or,c|u (500 r |0 ou d+, , ||eu 250 r |0 qd |o|
+ d+,)
ke||u||| |o\,c,c||ue (00 r |0 ||d |o| +-o Wee|) |
k||+rp|u (!00 r |0 ||d |o| +-o Wee|)
B+c|||+|, +u|or+|o| E|,|||or,c|u (500 r |0 q|d |o| ! rou||)
|o\,c,c||ue (00 r |0 ||d |o| ! rou||)
B+c|||+|, pe||o| E|,|||or,c|u (500 r |0 q|d |o| + rou||)
|o\,c,c||ue (00 r |0 ||d |o| + rou||)
3c|-||c eudoc+|d|||
'upec|ed Ceu|+r|c|u (! r/| qd |\ |o| + d+,) | ce||||+\oue (2 |\ qd |o|
o Wee|) +|| +|||
|o\,c,c||ue (00 r |0 ||d |o| o Wee|)
Cou|||red Ceu|+r|c|u (! r/| qd |\ |o| + d+,) |
|o\,c,c||ue (00 r |0 ||d |o| o Wee|)
I|euc| |e.e| |o\,c,c||ue (200 r |0 qd |o| + Wee|) |
Ceu|+r|c|u (! r/| qd |\ |o| + d+,)
B+||oue||o|
0|o,+ |e.e| C||o|+rp|eu|co| (500 r |0 o| |\ ! |o| + d+,) | + |+c|+r +eu|
C|p|o||o\+c|u (500 r ||d |o| 0 d+,)
\e||u+ pe|u+u+ k||+rp|u (0 r/| qd |0 |o| + d+,)
'||ep|or,c|u (5-20 r/| qd |\ |o| 0 d+,)
'0ukCE. ||or l\ ko|+|u e| +|. kecorreud+||ou |o| ||e+|reu| o| |ur+u |u|ec||ou c+ued |, 3c|-||c pec|e. 1|:| 1-| C|-||-
+3.92, 200+, W||| pe|r||ou.
E||o|o,. 3 |--|c-
I|+ur||ou. c|+|c| ||or o| cou|+c| W||| + c+|
C||u|c+| ||ud|u.
|uocu|+||ou ||e. ||u o| coujuuc||.+| |e|ou +||e|
c+| c|+|c|e o| cou|+c| W||| + c+|
|,rp| uode. +cu|e |o u|+cu|e |eude| |e|ou+|
|,rp|+deuop+||,
Coujuuc||.+| |uocu|+||ou. uu||+|e|+| coujuuc||
.+| |e|ou W||| p|e+u||cu|+| |,rp|+deuop+||,
(|+||u+ud ocu|o|+udu|+| ,ud|ore)
',|er|c |u.o|.ereu| uucorrou. ue|.ou ,
|er, .|ce|+| o|+u, |oue
',,. C+|c|+|c| |e.e|
CAI-SCkAICh 0ISAS (CS0) |C|9 . 013.!0
|C|0 . A23.
to|oy B. |ense|ae.
keservor Domestic cat.
vector Cat flea ( Cenote|a|Jes [e|s ).
host Immunocompetent.
Iraosmssoo Associated with exposuie to
young cats with fleas. Blood cultuies of kittens
aie fiequently positive foi B. |ense|ae. Fleas
tiansmit infection between cats.
Seasoo Late fall, wintei, oi eaily spiing in
coolei climates; July and August in waimei
climates. Woildwide.
Ae oI 0oset Majoiity (60%) of cases occui in
childien. 40% in adults.
Iocdeoce 20,000 cases annually in the United
States, of whom 2000 aie hospitalized.
FAIh0CNSIS
B. |ense|ae causes gianulomatous inflammation
in healthy individuals (CSD) and angiogenesis
in immunocompiomised peisons.
Iocubatoo Ferod
Piimaiy lesion at bite/sciatch site: 3-5 days
aftei inoculation; occuis in about half of
cases.
Regional lymphadenopathy: 1-2 weeks aftei
inoculation.
Frodrome Mild fevei and malaise occui in
fewei than half the patients. Chills, geneial ach-
ing, and nausea aie infiequently piesent.
Mucocutaoeous Iesoos
Ioocu|atoo Ste
Innocuous-looking, small (0.5 to 1-cm) pa-
pule, vesicle, oi pustule 3-5 days aftei inocu-
lation; may ulceiate; skin coloi pink to ied;
fiim, at times tendei (Fig. 24-55).
Residual lineai cat sciatch.
Peisists foi 1-3 weeks.
Dsr|uon: Exposed skin of face, hands.
Mutous mem|ranes: If poital of entiy is
the conjunctiva, 3- to 5-mm whitish-yellow
gianulation on palpebial conjunctiva associ-
ated with tendei pieauiiculai and/oi ceivical
lymphadenopathy (Paiinaud oculoglandulai
syndiome).
Uncommonly: uiticaiia, tiansient maculopapu-
lai eiuption, vesiculopapulai lesions, eiythema
nodosum.
Ceoera| xamoatoo Most patients do not
have fevei. Systemic symptoms common: ma-
laise, anoiexia, weight loss.
keooa| Iymphadeoopathy
(Fig. 24-56) Evident within 2-3 weeks aftei
inoculation in 90% of cases; piimaiy lesion,
if piesent, may have iesolved by the time lym-
phadenopathy occuis.
Nodes aie usually solitaiy, modeiately tendei,
fieely movable. Involved lymph nodes: epi-
tiochleai, axillaiy, pectoial, ceivical.
Nodes may suppuiate. Usually iesolved within
3 months.
Geneialized lymphadenopathy oi involve-
ment of the lymph nodes of moie than one
iegion is unusual.
0ther Encephalitis (seizuies, coma in chil-
dien), meningitis, tiansveise myelitis, pneu-
monitis, thiombocytopenia, osteomyelitis,
gianulomatous hepatitis, abscesses in livei oi
spleen, disseminated infection.
0sta| Cutaoeous Iesoo wth keooa| Iymphad-
eoopathy Suppuiative bacteiial lymphad-
enitis, nontubeiculous mycobacteiia (NTM),
spoiotiichosis, tulaiemia, tumois, saicoidosis,
lymphogianuloma veneieum, coccidioidomy-
cosis.
0ther Cat-Assocated IoIectoos Paseure||a
mu|otJa, bite infection; Canotyo|aga (DF-
2) spp., bite infection, spoiotiichosis; Mtro-
sorum tans, deimatophytosis; Toxotara taa,
laiva migians; Dro[|ara reens, subcutaneous
nodules.
hemato|oy WBC usually noimal; ESR com-
monly elevated.
0ermatopatho|oy Gianulomatous inflam-
mation with stellate neciosis; no angiogenesis.
Demonstiation of small, pleomoiphic bacilli
in Waithin-Staiiy-stained sections of piimaiy
skin lesion, conjunctiva, oi lymph nodes.
Cu|ture B. |ense|ae iaiely isolated fiom lymph
node aspiiates.
Sero|oy Antibodies to B. |ense|ae usually
positive 1:64.
FCk Done on tissue fiom lymph node; pie-
feiied ovei cultuie.
0IACN0SIS
Suggested by iegional lymphadenopathy devel-
oping ovei 2-3 weeks in an individual with cat
contact and a piimaiy lesion at the site of con-
tact; confiimed by identification of B. |ense|ae
fiom tissue oi seiodiagnosis.
C0kS AN0 Fk0CN0SIS
Self-limiting, usually within 1-2 months.
Uncommonly, piolonged moibidity with pei-
sistent high fevei, suppuiative lymphadenitis,
seveie systemic symptoms.
May be confused with lymphoma. Uncom-
monly, cat-sciatch encephalopathy occuis.
Antibiotic theiapy has not been veiy effective
in alteiing the couise of the infection.
MANACMNI
In the immunocompetent host, CSD iesolves
spontaneously.
Aotmcroba| Iherapy Antibiotic theiapy may
hasten iesolution of lymphadenopathy. See
Table 24-2.
Surca| Iherapy Diaining of lymph nodes
may be iequiied.
FICk 24-55 8artooe||oss: cat-scratch ds-
ease wth prmary |esoo E|,||er+|ou uodu|e
o| ||e c|ee| o| + 9,e+|o|d ||| +| ||e ||e o| c+|
c|+|c|. ||+uo| W+ r+de ou ||e |||o|o|c ||ud|u
o| ||e e\c|ed pec|reu.
FICk 24-56 8artooe||oss:
cat-scratch dsease wth ax||ary
adeoopathy Acu|e, .e|, |eude|,
+\|||+|, |,rp|+deuop+||, |u + c|||d,
c+| c|+|c|e We|e p|eeu| ou ||e
do|ur o| ||e |p||+|e|+| |+ud. (Cou|
|e, o| noW+|d ne||e|, \|.)
|u|ec||ou |, 3 |--|c- +ud 3 |cc |u |r
ruuocorp|or|ed n|\/A||'|u|ec|ed |ud|.|du
+| c+u c+ue
B+c|||+|, +u|or+|o| (BA) W||| cu|+ueou
+u|or+|ou |e|ou
',|er|c |u|ec||ou W||| pe||o| |ep+||||, o|eo
r,e||||, |e.e| o| uu|uoWu o|||u, |+c|e|er|+,
eudoc+|d|||.
\|ce|+| |u|ec||ou c+u |e |+|+| || uo| d|+uoed +ud
||e+|ed +pp|op||+|e|,.
8kI0NFII INFCII0NS IN hIv[AI0S
Cutaoeous 8A Lesions may be painful.
0ssemoated 8A Skin lesions usually absent.
Piesents with nausea, vomiting, diaiihea, fevei,
chills. Bony lesions may cause focal bone pain.
Sko Iesoos Papules oi nodules iesembling
angiomas (ied, biight ied, violaceous, oi skin-
coloied) (Fig. 24-57); up to 2-3 cm in diametei;
usually situated in deimis with thinning oi
eiosion of oveilying epideimis suiiounded by
a collaiette of scale. Laigei lesions may ulceiate.
Pyogenic gianuloma-like lesions (Fig. 24-57).
Subcutaneous nodules, 1-2 cm in diametei,
iesembling cysts. Uncommonly, abscess foi-
mation. Papules/nodules iange fiom solitaiy
lesions to >100 and, iaiely, >1000. Fiim, non-
blanching. Lesions may be nontendei oi pain-
ful, a finding not seen in nodulai lesions of
Kaposi saicoma.
DIstrIhutIvn Any site, but palms and soles
aie usually spaied. Occasionally, lesions occui
at the site of a cat sciatch. A solitaiy lesion may
piesent as dactylitis.
Mucvus Memhrunes Angioma-like lesions of
lips and oial mucosa. Laiyngeal involvement
with obstiuction.
E||o|o,. 3 |--|c- 3 |cc
Bo|| c+ue cu|+ueou +u|or+
3 |cc c+ue u|cu|+ueou uodu|e +ud
|,||c |oue |e|ou
n|\/A||'|u|ec|ed pe|ou W||| C|+ I ce|| couu|
00 /u|
|uc|deuce. uucorrou. |uc|deuce dec|e+ed W|||
p|op|,|+\| o| oppo||uu|||c |u|ec||ou +ud +u|||e|
|o.||+| ||e|+p, (AkI)
k|| |+c|o|
3 |--|c- . cou|+c| W||| c+| +ud/o| c+| ||e+
(C |-| )
3 |cc . |oW |ucore, |ore|eue, |od,
|oue ( | |c : ) |u|e|+||ou
C||u|c+| ||ud|u.
'||u. |ed +u|or+|ou p+pu|e, uodu|e, |u
ro|, uu+||, ru|||p|e. 'u|cu|+ueou uodu|e.
',|er|c |u.o|.ereu|. ||.e|, p|eeu, |oue
8ACIIIAk ANCI0MAI0SIS |C|9 . 033.0
|C|0 . A++.3
Systemc Fodos Infection may spiead he-
matogenously oi via lymphatics to become
systemic, commonly involving the livei and
spleen (hepatosplenomegaly, livei abscesses,
neciotizing splenitis, hepatic/splenic neciotiz-
ing gianulomata). Lesions may also occui in
the heait (caidiac lesions, endocaiditis), bone
maiiow, lymph nodes, muscles, soft tissues,
and CNS (biain abscess, aseptic meningitis,
encephalopathy).
Mu|tp|e Cutaoeous Aoomatous Fapu|es[
Nodu|es o hIv[AI0S dsease Kaposi saicoma,
pyogenic gianuloma, cheiiy angioma, scleios-
ing hemangioma.
0ermatopatho|oy Lobulai vasculai piolif-
eiations composed of plump epithelioid" en-
dothelia. Neutiophils scatteied thioughout the
lesion, especially aiound eosinophilic gianu-
lai aggiegates, which aie masses of bacteiia
(visualized by Waithin-Staiiy staining oi elec-
tion micioscopy).
Iver 8opsy Associated with highei moibid-
ity and moitality iates in that the lesions aie
vasculai tumois. Dilated capillaiies oi multiple
blood-filled caveinous spaces; myxoid stioma
containing an admixtuie of inflammatoiy cells
and gianulai clumps ( Barone||a ).
Cu|ture Barone||a can be isolated fiom le-
sional skin biopsy specimens, blood, oi othei
infected tissues on endothelial-cell monolayei.
FCk Detects Barone||a DNA in tissue.
Chemstry Bacillaiy peliosis hepatitis associ-
ated with elevated aminotiansfeiase, alkaline
phosphatase.
Sero|oy Anti- Barone||a antibodies de-
tected by indiiect fluoiescent-antibody test-
ing (detected by the CDC). Also, enzyme
immunoassay foi detection of IgG antibodies
to B. |ense|ae .
Imao Lesions can be visualized by conven-
tional iadiogiaphs and nucleai imaging. Le-
sions iegiess with appiopiiate theiapy. CT scan
shows hepatomegaly, splenomegaly.
0IACN0SIS
Clinical findings confiimed by demonstiation
of Barone||a bacilli on silvei stain of lesional bi-
opsy specimen oi cultuie oi antibody studies.
C0kS AN0 Fk0CN0SIS
Raiely seen in HIV/AIDS individuals success-
fully tieated with ART. In untieated HIV/AIDS
disease, couise vaiiable. In some individuals, le-
sions iegiess spontaneously. Untieated systemic
infection causes significant moibidity and moi-
tality. With effective antimiciobial theiapy, le-
sions iesolve within 1-2 weeks. As with othei
infections occuiiing in HIV/AIDS, ielapse
may occui and iequiie lifelong secondaiy
piophylaxis. Azithiomycin given foi Myto|ate-
rum aum complex (MAC) piophylaxis seems
to pievent BA as well.
MANACMNI
Freveotoo HIV/AIDS individuals should
avoid contact with cats, especially kittens, to
minimize the iisk foi acquiiing BA, as well as
toxoplasmosis.
Aotmcroba| See page 657.
E||o|o,. |c:-||c ||c- , |,pe A +ud B
I|+ur||ou |,.
n+ud||u ||e| o| |u|ec|ed +u|r+|
B||e o| |uec| .ec|o|
|uocu|+||ou o| coujuuc||.+
|ue||ou o| |u|ec|ed |ood
|u|+|+||ou
C||u|c+| ,ud|ore
u|ce|o|+udu|+|
0cu|o|+udu|+|
I,p|o|d+|
|u|rou+|,
',, . k+|||| |e.e|, dee|||, |e.e|, 0|+|+
|e.e|, ||+uc| d|e+e
IIAkMIA |C|9 . 02
|C|0 . A2
FICk 24-5T 8artooe||oss: bac||ary aoo-
matoss ! |o 5rr c|e||, |er+u|or+|||e
p+pu|e +ud + |+|e| p,oeu|c |+uu|or+|||e uodu|e
ou ||e ||u o| + r+|e W||| +d.+uced n|\ d|e+e.
'u|cu|+ueou uodu|+| |e|ou We|e +|o p|eeu|.
hstorca| Fact Named aftei Tulaie County,
CA
Ae oI 0oset[Sex Young males.
to|oy
F. u|arenss , a pleomoiphic giam-negative
intiacellulai coccobacillus.
Two subspecies:
Type A common in Noith Ameiica, highly
viiulent in animals and humans;
Type B causes all human cases in Asia and
Euiope.
One of the most infectious bacteiia known.
0ccupatoo Rabbit hunteis, butcheis, cooks,
agiicultuial woikeis, tiappeis, campeis, sheep
heideis and sheaieis, mink iancheis, muskiat
faimeis, laboiatoiy technicians.
Iosect vector Ticks ( IxoJes, Dermatenor ),
deei flies, body lice, othei aithiopods.
Aoma| keservor Rabbits, haies, muskiats,
piaiiie dogs, foxes, squiiiels, skunks, voles,
beaveis.
Iraosmssoo As few as 10 oiganism can cause
infection.
Aithiopod bites: deei flies, ticks, oi othei
insects caiiying the disease.
Handling infected animal tissue oi fluids.
Small abiasion oi punctuie wound. Conjunc-
tival inoculation.
Eating oi diinking food oi watei contami-
nated by the bacteiia
Bieathing in the bacteiia. Inhaling paiticles
fiom an infected iabbit giound up in a lawn-
mowei (Maitha`s Vineyaid). Tianspoiting
hay.
Laboiatoiy exposuie.
Biological weapon: Stiains suitable foi inhal-
ation have been developed. See Appendix B.
Seasoo Most U.S. cases occui June-Septembei
when aithiopod tiansmission is most common.
Ceoraphy Thioughout noithein hemispheie:
Noith Ameiica, paits of Euiope, Asia. United
States: south-cential and westein states.
8o|oca| Weapoo Could be used; aeiosol
ielease would have the gieatest adveise medical
and public health consequences. Pleuiopneu-
monitis; oculai tulaiemia; ulceioglandulai oi
glandulai disease. (See Appendix B.)
| . / /www. | . tJt. go/ age n / u| arem a/
u|arema-|o|ogta|-weaon-a|srat.as
Iocdeoce Raie. <200 cases iepoited in United

States pei yeai; undeidiagnosed, undeiie-
poited.
C|oca| Syodromes
Ulceioglandulai (75% of cases)
Glandulai
Pulmonaiy
Oiophaiyngeal
Oculoglandulai
Typhoidal
Biological weapon
FAIh0CNSIS
Facultative intiacellulai oiganism; multiplies
in maciophages.
Majoi taigets: lymph nodes, lungs and pleuia,
spleen, livei, kidney.
Aftei inoculation into skin, mucous mem-
biane, lung (inhalation), oi GI tiact F. u|aren-
ss iepioduces and spieads thiough lymphatic
channels to lymph nodes and bloodstieam.
Suppuiative lesions become gianulomatous
with cential neciosis and caseation.
Iocubatoo Ferod 2-10 days.
Symptoms Piodiome: headache, malaise, my-
algia, high fevei. About 48 h aftei inoculation,
piuiitic papule develops at the site of tiauma oi
insect bite followed by enlaigement of iegional
lymph nodes.
At inoculation site: eiythematous tendei pa-
pule evolving to a vesicopustule, enlaiging to
ciusted ulcei with iaised, shaiply demaicated
maigins (96 h) (Fig. 24-58).
Depiessed centei that is often coveied by a
black eschai (chanciifoim).
Piimaiy lesion on fingei/hand at site of
tiauma/insect bite; gioin/axilla aftei tick bite.
Aftei bacteiemia, exanthem (tiunk and extiemi-
ties) with macules, papules, petechiae; eiythema
multifoime (Fig. 24-59); eiythema nodosum.
Mutous mem|ranes: In oculoglandulai tu-
laiemia, F. u|arenss is inoculated into con-
junctiva, causing a puiulent conjunctivitis
with pain, edema, congestion. Small yellow
nodules occui on conjunctivae and ulceiate.
|.//www.|ogta|mages.tom/resourtesBT-
gensTu|arema.|m
Ceoera| Fodos
Fevei to 41C.
Regional lymph nodes
As the ulcei develops, nodes enlaige and
become tendei (chanciifoim syndiome)
(Fig. 24-58).
If untieated, become suppuiating buboes.
Lung consolidation, splenomegaly, geneial-
ized lymphadenopathy, hepatomegaly may
occui.
Vaiiants
Typhoidal" foim occuis with ingestion
of F. u|arenss , iesulting in ulceiative oi
exudative phaiyngotonsillitis with ceivical
lymphadenopathy.
Tulaiemic pneumonia occuis aftei bactei-
emia oi inhalation of F. u|arenss .
Cutaoeous Ioocu|atoo Ste Fuiuncle, paio-
nychia, spotted fevei, anthiax, Paseure||a mu|-
otJa infection, spoiotiichosis, M. marnum
infection.
Ieoder keooa| Adeoopathy Heipes simplex
viius lymphadenitis, plague, cat-sciatch disease,
FICk 24-58 Iu|arema: prmary |esoo aod

reooa| adeoopathy A c|u|ed u|ce| +| ||e ||e o|
|uocu|+||ou | eeu ou ||e do|ur o| ||e |e|| ||u ||u
e| W||| +oc|+|ed +\|||+|, |,rp| uode eu|+|ereu|
(c|+uc|||o|r ,ud|ore). I|e |u|ec||ou occu||ed +||e|
||e p+||eu| ||||ed +ud ||uued + |+||||.
FICk 24-59 Iu|arema: ery-
thema mu|tIorme Au e\+u||er
ou ||e c|e| W||| |e+|u|e o| e|,
||er+ ru||||o|re ,ud|ore, + |,pe|
eu|||.||, |o |c:-||c ||c-.
melioidosis oi glandeis, lymphogianuloma
veneieum.
Cu|tures Routine cultuie media do not sup-
poit the giowth of F. u|arenss fiom clinical
specimens.
Sero|oy Diagnosis usually confiimed by
demonstiating a fouifold iise in acute and con-
valescent F. u|arenss antibody titeis.
0IACN0SIS
Clinical diagnosis in a patient with chanciifoim
syndiome with appiopiiate animal exposuie oi
insect exposuie and systemic manifestations.
Disease in pneumonic piesentation has flu-
like" symptomatology and is fatal if uniecog-
nized.
C0kS AN0 Fk0CN0SIS
Untieated, moitality iate foi ulceioglandulai
foim is 5%; 1% if theiapy initiated piomptly;
foi typhoidal and pulmonaiy foims, 30%.
MANACMNI
Freveotoo Avoid contact with wild iabbits.
In tick-infested aieas, weai tight wiistbands
and pants tucked into boots to pievent tick
attachment. Inspect foi ticks at day`s end. Live
vaccine available foi high-iisk gioups. Weai
iubbei gloves when handling oi piocessing
wild iabbits.
0ru oI Choce Gentamycin oi stieptomycin.
A|teroatves Doxycycline, chloiamphenicol,
cipiofloxacin.
http://www.bt.cdc.gov/agent/tulaiemia/
http://www.cidiap.umn.edu/idsa/bt/tulaiemia/
biofacts/tulaiemiafactsheet.html
ne|e|oeueou |oup o| |+rue+||.e |+c|e||+
(peudorou+d)
|-!c
3|||!-c
'|-||c
|+||oeu|c||,. uu+||, oppo||uu|||c, uoocor|+|
|-!c c-c | ||e r+jo| p+||oeu
|o| ||u |u|ec||ou.
P5F00M0N5 SFCIS
|ou|+||d|ou, ro|||e, p|oduce p,oc,+u|u +ud
p,o.e|d|u, p|reu| ||+| c+ue ,e||oW |o d+||
|eeu |o ||u|| co|o|.
Eu.||oureu|+| ou|ce. ro|| eco|o|c+| u|c|e o|
o||, p|+u|, .ee|+||e, |+p W+|e|.
k|| |+c|o|. |op||+||/+||ou, c+uce|, c,|o|o\|c
c|ero||e|+p,, ueu||opeu|+, ||o+dpec||ur +u
||||o||c ||e|+p,, c||ou|c Wouud, n|\/A||', |r
ruuocorp|or|e, |ucoco|||co|d, c+||e|e| (|\,
u|e|||+|), de|||||+||ou, ruco+| u|ce|+||ou, c,||c
||||o|.
Cu|+ueou |u|ec||ou |u |e+|||, pe|ou
|eudorou+| |o|||cu||||. occu| |u |e+|||, |u
d|.|du+| e\poed |o cou|+r|u+|ed |o||u|
(|o||u| |o|||cu||||)
C|+rue+||.e We|p+ce |u|e||||o. occu| W|||
|,pe|||d|o|, o||eu W||| coucor||+u| ||ue+
ped|
C|eeu u+||. |oW + + ||o|||r ou ||e uude|
u||+ce o| ou,c|o|,||c u+|| p|+|e uc| + W|||
po||+| o| ou,c|or,co|, co|ou|/+||ou uo|
|u|ec||ou.
|oc+| |u.+|ou c+u |o||oW co|ou|/+||ou o| +u, ru
cocu|+ueou ||e W||| |oc+| Wouud |u|ec||ou, o||
||ue |u|ec||ou, +ud u|equeu| |er+|oeuou
d|er|u+||ou.
Cu|+ueou |u|ec||ou |u corp|or|ed p+||eu|.
wouud |u|ec||ou, e.., |u|u, p|eu|e u|ce|,
u||c+| ||e
Ec||,r+ +u|euour (EC) | ||e uec|o||/|u
o|| ||ue |u|ec||ou ||+| occu| +||e| |oc+| ||ue
|u.+|ou o| |+c|e|er|c eed|u, +oc|+|ed W|||
||ood .ee| |u.+|ou, ep||c .+cu||||, .+cu|+|
occ|u|ou, +ud |u|+|c||ou o| ||ue.
CIAN0S P5F00M0N5 Fk6IN05 INFCII0NS
FI0MI0I0C
co|oy
Widespiead in natuie, inhabiting watei, soil,
plants, and animals, piefeiiing moist en-
viionments.
Caiiiage iate is low in healthy individuals.
Colonizes skin, exteinal eai, uppei iespiiatoiy
tiact, and/oi laige bowel in those who aie
natuially oi iatiogenically compiomised, have
ieceived antimiciobial theiapy, and/oi been
exposed to a hospital enviionment.
Iraosmssoo
Most infections aie hospital acquiied.
Pseudomonal caiiiage incieases with length
of hospital stay and antibiotic administia-
tion.
Tiansmitted to patients via hands of hospital
peisonnel oi via fomites.
Entiy sites foi bacteiemia at bieaks in muco-
cutaneous baiiieis: sites of tiauma, foieign
bodies (IV oi uiinaiy cathetei), aspiiation/
aeiosolization into iespiiatoiy tiact, skin ul-
ceis, theimal buins.
ksk Factors Ior Iovasve IoIectoo P. aerug-
nosa is piimaiily a nosocomial pathogen-the
fouith most commonly isolated-accounting
foi 10% of all hospital-acquiied infections.
FAIh0CNSIS
Most infections aie both invasive and toxi-
genic. Infection occuis in thiee stages:
Bacteiial attachment and colonization
Local invasion and damage of tissue
Disseminated systemic disease
Infection may stop at any stage
P. aerugnosa iaiely causes disease in the
healthy host. Infections occui when:
Noimal cutaneous oi mucosal baiiieis have
been bieached oi bypassed (e.g., buin injuiy,
penetiating tiauma, suigeiy, endotiacheal
intubation, uiinaiy bladdei catheteiization,
IV diug abuse)
Immunologic defense mechanisms have
been compiomised (e.g., by chemotheiapy-
induced neutiopenia, hypogammaglob-
ulinemia, extiemes of age, diabetes mellitus,
cystic fibiosis, cancei, HIV/AIDS disease)
Piotective function of noimal bacteiial floia
has been disiupted by bioad-spectium anti-
miciobial theiapy
When a patient has been exposed to iesei-

voiis associated with hospital enviionment.
Blood vessel and bloodstieam invasion, dis-
semination, SIRS (sepsis syndiome), multioi-
gan dysfunction, and, ultimately, death may
follow localized infection.
The oiganism and/oi its pioducts may cause
tissue injuiy at piimaiy and secondaiy sites
of infection; ielease of systemically acting
toxins oi inflammatoiy mediatois of infected
host may contiibute diiectly oi indiiectly to
SIRS.
Cutaoeous IoIectoos
Na| Co|ootatoo P. aerugnosa giows within
a biofilm on the undeisuiface of onycholytic
nails, e.g., psoiiasis, onychomycosis; the undei
suiface of the nail plate has a suiface gieen
discoloiation that can easily be abiaded (see
Section 33, Fig. 33-3). The onycholytic nail
plate can be tiimmed to eliminate the abnoimal
space.
Fo||cu|ts P. aerugnosa can infect multiple
haii follicles in healthy individuals aftei aque-
ous exposuie in contaminated hot tubs (hot-
tub folliculitis," see Fig. 32-30) oi physiotheiapy
pools, piesenting as multiple folliculai pustules
on the tiunk (see Section 32). The infection is
self-limited.
Ioe Webspace IoIectoo Inteitiigo of toe web-
spaces. Webspace(s) maceiated, moist often
with gieen coloi (see Fig. 25-3); usually in set-
ting of hypeihidiosis, maceiated inteidigital
tinea pedis, eiythiasma, keiatodeima.
Frmary aod Secoodary Fyoderma P. aeru-
gnosa can cause piimaiy infection of haii
follicles oi small bieaks in skin, oi secondaiy
infections of sites of tiauma, buin injuiy, in-
flammatoiy deimatoses, ulceis. With deepei
invasion, neciotizing infection (due to blood
vessel invasion and occlusion) occuis, i.e., EG.
These pyodeimas have a typical blue-gieen exu-
date and chaiacteiistic fiuity odoi. In theimal
buin injuiy, black, daik biown, oi violaceous
discoloiation of buin eschai may occui.
xteroa| 0tts Swimmei`s eai." Moist en-
viionment of exteinal auditoiy canal piovides
medium foi supeificial infection, piesenting as
piuiitus, pain, dischaige; usually self-limited.
Malignant exteinal otitis occuis in eldeily dia-
betics most commonly; may piogiess to deepei
invasive infection.

Iovasve IoIectoos
cthyma Caoreoosum Begins as an eiy-
thematous macule (cutaneous ischemic lesion
that quickly evolves to an infaiction) (see
Fig. 24-29 , B ). The epideimis oveilying the
ischemic aiea may slough with foimation of
eiosion/ulcei. EG usually occuis as a solitaiy
lesion but may occui as a few lesions. EG can
occui as a complication of piimaiy oi secon-
daiy pyodeima oi of bacteiemia. Initially eiy-
thematous, piogiessing to hemoiihagic bluish
(so-called gunmetal giay). Fully evolved EG:
blackish cential neciosis with eiythematous
halo. Lesions usually tendei, but may be pain-
less. Most common sites: axillae, gioin, peiianal;
may occui anywheie, including lip and tongue.
0ssemoated Nodu|e Hematogenous dissemi-
nation of P. aerugnosa can seed the deimis,
iesulting in multiple tendei subcutaneous nod-
ules.
Necrotc Sko Iesoo(s) Vasculitis, ciyoglob-
ulinemia, fixed diug eiuption, pyodeima gan-
gienosum.
Cu|tures In most cases, P. aerugnosa can be
cultuied fiom both blood and ecthymatous skin
lesions. Howevei, EG can iemain a localized
cutaneous infection, not accompanied by sys-
temic infection; in this case, only cultuie of
exudate oi biopsy specimen fiom the lesion is
positive foi P. aerugnosa .
0ermatopatho|oy Vasculitis without thiom-
bosis; paucity of neutiophils at site of infection;
bacilli found in media and adventitia, but usu-
ally not in intima, of vessel.
0IACN0SIS
Clinical suspicion confiimed by blood and skin
exudate/biopsy specimen cultuie.
C0kS AN0 Fk0CN0SIS
The heteiogeneity of infections accounts foi
substantial diffeiences in shoit-teim and
long-teim piognosis.
Piognosis depends on piompt iestoiation of
alteied immunity, usually on coiiection of
neutiopenia.
When occuiiing as a local infection in the ab-
sence of bacteiemia, piognosis is much moie
favoiable.
Baterema: may be associated with SIRS;
fevei, tachypnea, tachycaidia, piostiation, hy-
potension.
EnJotarJs: left-sided infections piesent with
embolic phenomena: laige emboli, ecthyma
gangienosum, Oslei nodes, Janeway lesions
(see Figs. 24-46 and 24-48).
Casronesna| n[eton: iose" spotlike le-
sions: eiythematous macules and/oi papules
on tiunk as in typhoid fevei; occui with Pseu-
Jomonas infection of GI tiact, i.e., diaiihea,
headache, high fevei (Shanghai fevei).
MANACMNI 0F INvASIv INFCII0NS
Correct Fredsposo Factors White cell tians-
fusion oi gianulocyte colony-stimulating factoi
foi gianulocytopenia.
Aotmcroba| Iherapy Antibiotic and dos-
age aie adjusted accoiding to sensitivities and
iesults of cultuies.
Surery Aftei contiol of infection, aieas of
infaiction should be debiided.
\,co|+c|e||+ +|e |od|+ped o| cocco|+c||||, +c|d
|+| |+c|||| (A|B), +c|d|+|ue +oc|+|ed W|||
corpo|||ou o| ||e|| ce|| W+||
>20 pec|e |deu||||ed, |e|+||.e|, |eW +oc|+|ed
W||| |ur+u d|e+e
\,co|+c|e||+| |u|ec||ou
Iu|e|cu|o|
|ep|o, (n+ueu d|e+e)
|u|ec||ou due |o uou|u|e|cu|ou r,co|+c|e||+
(|I\). Bu|u|| u|ce| d|e+e ( ! |:-c ) |
||e ||||d ro| corrou r,co|+c|e||+| d|e+e
|o|+||,.
Iu|e|cu|o| due |o BCC |rruu|/+||ou
MC08ACIkIAI INFCII0NS
FI0MI0I0C
With the advent of HIV/AIDS disease, tubeicu-
losis and NTM infections have been biought to
the foiefiont of clinical medicine. Concuiient
! ||-:| corp|e\ . p+||oeu|c |u o||e|W|e
|e+|||, |ud|.|du+|, |oWe.e|, |u|e|cu|o| | ruc|
ro|e ||o||d |u |rruuocorp|or|ed |o|.
! ||-:|
! |.
! c|:c
! |-c- c+ue d|e+e |u |ur+u e\c|u|.e|,.
0||e|W|e|e+|||, |ud|.|du+| |ecore |u|ec|ed
W||| ||| r,co|+c|e||ur, c||u|c+| r+u||e|+||ou
.+|, ||ereudou|, +cco|d|u |o ||e |o|' |r
ruue |epoue |o ||e o|+u|r.
|ou|u|e|cu|ou r,co|+c|e||+ (|I\) e\|| |u ||e
eu.||oureu|, |deu||||c+||ou |u |ur+u ||ue, uu
|||e ! ||-:| o| ! |-c- | uo| + |ue qu+
uou o| e||o|o, o| + d|o|de|.
'|oW|oW|u |\I |+cu||+||.e |ur+u p+||o
eu. ! c , ! :||c:- , ! c.
|c:-|||c- corp|e\, ! |c-|| , !
|:-c
|+||oW|u |\I |+cu||+||.e |ur+u p+||o
eu. ! ||| , ! :|-|c- , ! c|:-
CIASSIFICAII0N 0F MC08ACIkIA AN0 MC08ACIkIAI INFCII0NS
HIV/AIDS and M. u|ertu|oss infections can
iesult in seveie infections; disseminated infec-
tions with NTM aie extiemely common in
advanced HIV/AIDS disease.
CIINIC0FAIh0I0CIC CIASSIFICAII0N
0F IFk0S
(Based on clinical, immunologic, and bacteiio-
logic findings)
Tu|ertu|oJ (TL): Localized skin involvement
and/oi peiipheial neive involvement; few
oiganisms.
Leromaous (LL): Geneialized involvement
including skin, uppei iespiiatoiy mucous
membiane, ieticuloendothelial system, adie-
nal glands, testes; many bacilli.
BorJer|ne (or Jmor|t") (BL): Has fea-
tuies of both TL and LL. Usually many bacilli
piesent, vaiied skin lesions: macules, plaques;
piogiesses to TL oi iegiesses to LL.
E||o|o,. ! |-c-
C||ou|c |+uu|or+|ou d|e+e p||uc|p+||, +c
qu||ed du||u c|||d|ood/,ouu +du|||ood.
'||e o| |u|ec||ou. ||u, pe||p|e|+| ue|.ou ,|er,
uppe| |ep||+|o|, ||+c|, e,e, |e|e
C||u|c+| r+u||e|+||ou, u+|u|+| |||o|,, +ud p|o
uo| o| |ep|o, +|e |e|+|ed |o ||e |o| |epoue
\+||ou |,pe o| |ep|o, (|u|e|cu|o|d, |ep|o
r+|ou, e|c.) |ep|eeu| ||e pec||+ o| ||e |o|'
|rruuo|o|c |epoue (ce||red|+|ed |rruu||,).
',,. n+ueu d|e+e.
IFk0S |C|9 . 0!0
|C|0 . A!0
InJeermnae [orms.
Transona| [orms: See Pathogenesis," below.
II0I0C AN0 FI0MI0I0C
to|oy M. |erae . Obligate intiacellulai acid-
fast bacillus; iepioduces maximally at 27C-
30C. Oiganism cannot be cultuied in vitio.
Infects skin and cutaneous neives (Schwann cell
basal lamina). In untieated patients, only 1% of
oiganisms aie viable. Giows best in coolei tis-
sues (skin, peiipheial neives, anteiioi chambei
of eye, uppei iespiiatoiy tiact, testes), spaiing
waimei aieas of the skin (axilla, gioin, scalp,
and mid-line of back).
Ae oI 0oset Incidence iate peaks at 10-20
yeais; pievalence peaks at 30-50 yeais.
kace Inveise ielationship between skin coloi
and seveiity of disease; in black Afiican, sus-
ceptibility is high, but theie is piedominance
of mildei foims of the disease, i.e., TL vis-a-vis
LL.
hosts Humans aie main ieseivoiis of M. |e-
rae . Wild aimadillos (Louisiana) as well as
mangabey monkeys and chimpanzees aie natu-
ially infected with M. |erae ; aimadillos can
develop lepiomatous lesions.
Iraosmssoo Unceitain; possible tiansmis-
sion includes nasal dioplet infection, contact
with infected soil, insect vectois. Sneeze fiom
untieated LL patient may contain 10
10
oi-
ganisms. 20% of asymptomatic individuals in
endemic aieas may have M. |erae in the nose,
identified by PCR. Poitals of entiy of M. |erae
aie pooily undeistood but include inoculation
via skin (bites, sciatches, small wounds, tattoos)
oi inhalation into nasal passages oi lungs.
0emoraphy Disease of developing woild:
600,000 new cases annually
1.5-8 million total cases woildwide
>80% of cases occui in India, China, Myan-
mai, Indochina, Indonesia, Biazil, Nigeiia.
In the United States: 4000 cases, 100-200 new
cases annually; most cases aie in immigiants
fiom Mexico, Southeast Asia, Philippines,
Caiibbean; diagnosed in Califoinia, Texas,
New Yoik, and Hawaii
Risk factois: poveity, iuial iesidence, HAV/
AIDS disease
Most individuals have natuial immunity and
do not develop disease.
FAIh0CNSIS
Clinical spectium of lepiosy depends exclusively
on vaiiable limitations in host`s capability to de-
velop effective cell-mediated immunity (CMI)
to M. |erae. Oiganism is capable of invading
and multiplying in peiipheial neives and infect-
ing and suiviving in endothelial and phagocytic
cells in many oigans. Subclinical infection with
lepiosy is common among iesidents in endemic
aieas. Piesumably the subclinical infection is
handled ieadily by the host`s CMI iesponse.
Clinical expiession of lepiosy is development of
a gianuloma; patient may develop a ieactional
state," which may occui in some foim in >50%
of ceitain gioups of patients.
Craou|omatous Spectrum oI Ieprosy
High-iesistance tubeiculoid iesponse (TT)
Low- oi absent-iesistance lepiomatous pole
(LL)
Moiphic oi boideiline iegion (BB)
Two inteimediaiy iegions
Boideiline lepiomatous (BL)
Boideiline tubeiculoid (BT)
In oidei of decieasing iesistance, the spectium
is TT, BT, BB, BL, LL.
Immuoo|oc kespooses Immune iesponses to
M. |erae can pioduce seveial types of ieactions
associated with a sudden change in the clinical
status.
Lepru Type 1 ReuctIvns (DvwngrudIng und
Reversu| ReuctIvns) Individuals with BT and
BL develop inflammation within existing skin
lesions. Can be associated with low-giade fevei,
new multiple small satellite" maculopapulai
skin lesions, and/oi neuiitis. Downgiading ie-
actions occui befoie theiapy. Reveisal ieactions
occui in iesponse to theiapy.
Lepru Type 2 ReuctIvns (Erythemu Nvdvsum
Leprvsum, ENL) Seen in half of LL patients,
usually occuiiing aftei initiation of antile-
piomatous theiapy, geneially within the fiist
2 yeais of tieatment. Massive inflammation
with eiythema nodosum-like lesions.
LucIv ReuctIvn Individuals with diffuse LL
develop shallow, laige polygonal sloughing ul-
ceiations on the legs. The ieaction appeais
to be eithei a vaiiant of ENL oi secondaiy to
aiteiiolai occlusion.
Iocubatoo Ferod 2-40 yeais (most com-
monly 5-7 yeais).
0oset Insidious and painless; fiist affects
peiipheial neivous system with peisistent oi
iecuiient painful paiesthesias and numbness
without any visible clinical signs. At this stage
theie may be tiansient maculai skin eiuptions;
blistei, but lack of awaieness of tiauma.
Systems kevew Neuial involvement leads to
muscle weakness, muscle atiophy, seveie neu-
iitic pain, and contiactuies of the hands and
feet.
Lepru Type 1 ReuctIvns Acute oi insidious
tendeiness and pain along affected neive(s),
associated with loss of function.
Iubercu|od Ieprosy (II, 8I) S|In Most com-
mon foim in India, Afiica. Few well-defined hy-
popigmented hypesthetic macules (Fig. 24-60)

with iaised edges and vaiying in size fiom a few
millimeteis to veiy laige lesions coveiing the
entiie tiunk. Eiythematous oi puiple boidei and
hypopigmented centei. Shaiply defined, iaised;
often annulai; enlaige peiipheially. Cential aiea
becomes atiophic/depiessed. Advanced lesions
aie anesthetic, devoid of skin appendages (sweat
glands, haii follicles). Any site including the face.
TT : Lesions may iesolve spontaneously; not
associated with lepia ieactions. BT : Does not heal
spontaneously; type 1 lepia ieactions may occui.
Nerve 1nvv|vement May be a thickened neive
on the edge of the lesion; laige peiipheial neive
enlaigement fiequent (ulnai). Skin involvement
is absent in neura| |erosy. Test pinpiick, tem-
peiatuie, vibiation.
8order|oe 88 Ieprosy S|In Lesions aie
inteimediate between tubeiculoid and lepio-
matous and aie composed of macules, papules,
and plaques (Figs. 24-61 and 24-62). Anesthesia
and decieased sweating aie piominent in the le-
sions.
Iepromatous Ieprosy (II, 8I) S|In Skin-
coloied oi slightly eiythematous papules/
nodules. Lesions enlaige; new lesions occui
and coalesce. Latei: symmetiically distiibuted
FICk 24-60 Ieprosy: tubercu|od type we||de||ued, |,pop|reu|ed, |||||, c+||u, +ue||e||c r+c
u|e +ud p|+que ou ||e po|e||o| ||uu|.
nodules, iaised plaques, diffuse deimal infil-
tiate, which on face iesults in loss of haii (lat-
eial eyebiows and eyelashes) and leonine facies
(lion`s face). D[[use |eromaoss , occuiiing in
westein Mexico, Caiibbean, piesents as diffuse
deimal infiltiation and thickened deimis.
DIstrIhutIvn v] LesIvns Bilateially symmetiic
involving eailobes, face, aims, and buttocks, oi
less fiequently the tiunk and lowei extiemities.
Tongue: nodules, plaques, oi fissuies.
Nerve 1nvv|vement Moie extensive than in
TT.
keactooa| States Immunologically mediated
inflammatoiy states, occuiiing spontaneously
oi aftei initiation of theiapy.
Lepru Type 1 ReuctIvns Downgiading and
ieveisal ieactions. Occui in boideiline disease.
Skin lesions become acutely inflamed, associ-
ated with edema and pain; may ulceiate. Edema
most seveie on face, hands, and feet.
Lepru Type 2 ReuctIvns (ENL) Occui in 50%
of LL. 90% of cases occui aftei initiation of
theiapy. Piesent as painful ied skin nodules
aiising supeificially and deeply, in contiast to
tiue eiythema nodosum; lesions foim abscesses
oi ulceiate. Lesions occui most commonly on
face and extensoi limbs.
LucIv ReuctIvn Occuis only in patients fiom
Mexico/Caiibbean with diffuse LL. Piesents
as iiiegulaily shaped eiythematous plaques;
lesions may iesolve spontaneously oi undeigo
neciosis with ulceiation.
Ceoera| Fodos ErtremItIes Sensoiy neu-
iopathy, plantai ulceis, secondaiy infection;
ulnai and peioneal palsies (Fig. 24-63), Chaicot
joints. Squamous cell caicinoma can aiise in
chionic foot ulceis (Fig. 11-13)
FICk 24-61 Ieprosy: border|oe-type A !,e+|
o|d \|e|u+ree |er+|e. . Ceo|+p||c |+ped p|+que ou
||e |u||oc| W||| |+|ed |ed |udu|+|ed r+||u +ud ceu||+|
c|e+||u. I|e|e | e\|eu|ou o| ||e |u|ec||ou |ud|c+|ed |,
||e e|,||er+|ou p+pu|e |e,oud ||e r+||u. 8. '|r||+|
eo|+p||c p|+que ou ||e |e W||| |+|ed |ed r+||u +ud
ceu||+| c|e+||u.
8
Nvse Chionic nasal congestion, epistaxis; de-
stiuction of caitilage with saddle-nose defoim-
ity (Fig. 24-63).
Eyes Cianial neive palsies, lagophthalmus,
coineal insensitivity. In LL, anteiioi chambei
can be invaded with uveitis, glaucoma, cata-
iact foimation. Coineal damage can occui
secondaiy to tiichiasis and sensoiy neuiopathy,
secondaiy infection, and muscle paialysis.
Testes May be involved in LL with iesultant
hypogonadism.
Amy|vIdvsIs Secondaiy with hepatic/ienal
abnoimalities.
Cvmp|IcutIvns Invasive squamous cell caici-
noma (SCC) can aiise in chionic neuiotiophic
ulceis on the lowei extiemities (see Fig. 11-13).
The tumois aie usually low-giade malignancies
but can metastasize to iegional lymph nodes
and cause death.
hypopmeoted Iesoos wth Craou|omas
Saicoidosis, leishmaniasis, lupus vulgaiis, NTM
infection, lymphoma, syphilis, yaws, gianuloma
annulaie, neciobiosis lipoidica.
S|t-Sko Smears A small skin incision is made;
the site is then sciaped to obtain tissue fluid
fiom which a smeai is made and examined aftei
Ziehl-Neelsen staining. Specimens aie usually
obtained fiom both eailobes and two othei ac-
tive lesions. Negative BIs aie seen in paucibacil-
laiy cases, tieated cases, and cases examined by
an inexpeiienced technician.
Nasa| Smears or Scrapos No longei iecom-
mended.
Cu|ture M. |erae has not been cultuied in
vitio; howevei, it does giow when inoculated
into the mouse foot pad. Routine bacteiial cul-
tuies to iule out secondaiy infection.
FCk M. |erae DNA detected by this technique
makes the diagnosis of eaily paucibacillaiy lep-
iosy and identifies M. |erae aftei theiapy.
0ermatopatho|oy TL shows epithelioid cell
gianulomas foiming aiound deimal neives; AFB
aie spaise oi absent. LL shows an extensive cellu-
lai infiltiate sepaiated fiom the epideimis by a
naiiow zone of noimal collagen. Skin append-
ages aie destioyed. Maciophages aie filled with
M. |erae , having abundant foamy oi vacuolated
cytoplasm (lepia cells oi Viichow cells).
FICk 24-62 Ieprosy: border|oe-type A 2o,e+|o|d \|e|u+ree r+|e. . we||der+|c+|ed, |u|||||+|ed,
e|,||er+|ou p|+que ou ||e |+ce. 8. |deu||c+| |ed p|+que ou ||e |oWe| |+c|.
8
FAkI III ||'EA'E' |uE I0 \|Ck0B|A| ACE|I' 6T0
0IACN0SIS
Made if one oi moie of the caidinal findings
aie detected: patient fiom endemic aiea, skin le-
sions chaiacteiistic of lepiosy with diminished
oi loss of sensation, enlaiged peiipheial neives,
finding of M. |erae in skin oi, less commonly,
othei sites.
C0kS AN0 Fk0CN0SIS
Aftei the fiist few yeais of diug theiapy, the
most difficult pioblem is management of the
changes secondaiy to neuiologic deficits-
contiactuies and tiophic changes in the hands
and feet. Uncommonly, secondaiy amyloidosis
with ienal failuie can complicate long-standing
lepiosy. Lepia type 1 ieactions last 2-4 months
in individuals with BT and up to 9 months in
those with BL. Lepia type 2 ieactions (ENL)
occui in 50% of individuals with LL and 25%
of those with BL within the fiist 2 yeais of
tieatment. ENL may be complicated by uveitis,
dactylitis, aithiitis, neuiitis, lymphadenitis, my-
ositis, oichitis. Lucio ieaction oi phenomenon
occuis secondaiy to vasculitis with subsequent
infaiction.
MANACMNI
Geneial piinciples of management:
Eiadicate infection with antilepiomatous
theiapy (Table 24-9).
Pievent and tieat ieactions.
Reduce the iisk of neive damage.
Educate patient to deal with neuiopathy and
anesthesia.
Tieat complications of neive damage.
Rehabilitate patient into society.
Management involves a bioad multidisciplinaiy
appioach including oithopedic suigeiy, podia-
tiy, ophthalmology, and physical theiapy.
IA8I 24-9 Antimicrobia| Reimens Recommended for Ireatment of Leprosy in Adu|ts
Form oI Leprosy Nore |oteos|ve 8eg|meo wh0-8ecommeoded 8eg|meo (1982)
Iu|e|cu|o|d (p+uc||+c|||+|,) |+poue (00 r/d) |o| 5 ,e+| |+poue (00 r/d uuupe|.|ed) | |||+rp|u
(o00 r/rou|| upe|.|ed) |o| o rou||
|ep|or+|ou (ru||||+c|||+|,) k||+rp|u (o00 r/rou||) |o| ! |+poue (00 r/d) | (!00 r)
,e+| | d+poue c|o|+/|r|ue (50 r/d, uuupe|.|ed),
(00 r/d) |ude||u||e|, +ud |||+rp|u (o00 r) | c|o|+/|r|ue
rou|||, (upe|.|ed) |o| ,e+|
FICk 24-63 Ieprosy: |epromatous type A
o0,e+|o|d \|e|u+ree |er+|e W||| ||e+|ed +d.+uced
d|e+e. u|u+| p+|,, |o o| d||| ou |||| |+ud, +ud
+dd|euoe de|o|r||, +oc|+|ed W||| |o o| u++|
c+||||+e +|e eeu.
SCII0N 24 BACIEk|A| |||ECI|0|' ||\0|\||C InE 'K|| 6T1
E||o|o,. !,:|c:|- ||-:| corp|e\,
! ||-:| (\I|), ! |. , ! :cc- !
c|:c ! :| ! -! ! :c-|| ,
occ+|ou+||, |+c|||u C+|re||eCue||u (BCC)
I|+ur||ou. +|||o|ue p|e+d o| d|op|e| uuc|e|
||or ||oe W||| |u|ec||ou pu|rou+|, I|
|ero|+p|,
up |o 0 o| ||oe |u|ec|ed W||| \I| W|||
de.e|op +c||.e I| |u ||e|| |||e||re
|uc|deuce o| I| |||e| |u |+|e +do|eceuce +ud
e+||, +du|||ood
0ue||||d o| |o|+| popu|+||ou |u|ec|ed
|u uu||ed '|+|e, e||r+|ed ||+| 5 r||||ou +|e
|u|ec|ed
3-9 r||||ou ueW |u|ec||ou +uuu+||,
2-! r||||ou IB|e|+|ed de+|| +uuu+||, Wo||d
W|de
C|||d|eu. .5 r||||ou ueW c+e, 500,000
de+||
C|o|+||,, r+|u c+ue o| de+|| |u n|\/A||'
||ed|po|u |+c|o| (e\oeuou |+c|o|) |o| +c
qu|||u \I| |u|ec||ou. po.e||,, c|oWd|u, n|\/
A||' p+uder|c, oc|+| d||up||ou
|+c|o| |o| de.e|op|u |u|e|cu|ou d|e+e (eu
doeuou).
|uu+|e |rruuo|o|c +ud uou|rruuo|o|c de
|eue
|e.e| o| |uuc||ou o| ce||red|+|ed |rruu||,
(C\|)
'||e o| |u|ec||ou
\o| corrou|, |u|ec|ed |uu
Cu|+ueou |u|ec||ou
Cu|+ueou |uocu|+||ou (|+|e)
|||ec| e\|eu|ou (e.., ||or jo|u|)
|,rp|+||c p|e+d
ner+|oeuou p|e+d
k|| |+c|o| |o| +c||.e I| +rou \I||u|ec|ed
pe|ou
keceu| |u|ec||ou ( ,e+|)
||||o||c |e|ou
Coro|||d||,. n|\/A||', |rruuoupp|e|.e
||e|+p,, po|||+up|+u|+||ou (o||d o|+u, |.e.,
||due,, ||.e|, |e+||, |uu), |||co|, eud|+e
|eu+| d|e+e/|erod|+|,|, d|+|e|e, |ujec||u
d|u ue, +||ec|or,, jejuuo||e+| |,p+
\+|uu|||||ou
||+uo|. c||u|c+| ||ud|u, |||op+||o|o,, |e|
e.+u| r,co|+c|e||+ |u ||ue o| cu||u|e, |o| |e+c
||ou |o \I| +u||eu
Cou|e/p|ouo|
||ope||, ||e+|ed, c+ued |, d|uucep||||e
||+|u cu|+||e |u .|||u+||, +|| c+e
uu||e+|ed, r+, |e |+|+| W||||u 5 ,e+|
\u|||d|u |e||+u| (\|k) I| | +u ere||u
|u|ec||ou
I|e+|reu|. |+ud+|d ru|||d|u |e|reu
',,. ||||||, couurp||ou
CIAN0S I8kCI0SIS (CI8) |C|9 . 01.0
|C|0 . A3.+
Iherapy oI keactoos Lepru Type 1 ReuctIvns
Piednisone, 40-60 mg/d; the dosage is giadu-
ally ieduced ovei a 2- to 3-month peiiod.
Indications foi piednisone: neuiitis, lesions
that thieaten to ulceiate, lesions appeaiing at
cosmetically impoitant sites (face).
Lepru Type 2 ReuctIvns (ENL) Piednisone,
40-60 mg/d, tapeied faiily iapidly; thalidomide
foi iecuiient ENL, 100-300 mg/d.
LucIv ReuctIvn Neithei piednisone noi tha-
lidomide is veiy effective. Since theie is no othei
alteinative, piednisone, 40-60 mg/d, tapeied
faiily iapidly.
Systemc Aotmcroba| Aeots Secondaiy
infection of ulceiations should be identified
and tieated with appiopiiate antibiotics to
pievent deepei infections such as osteomy-
elitis.
0rthopedc Care Splints should be supplied
to pievent contiactuies of deneivated iegions.
Caieful attention to foot caie to pievent neuio-
pathic ulceiation.
CIASSIFICAII0N 0F CIAN0S
I8kCI0SIS
Exogenous inoculation: Skin inoculation
Piimaiy inoculation tubeiculosis (PIT), i.e.,
tubeiculous chancie: occuis at inoculated
site in nonimmune host.
Tubeiculosis veiiucosa cutis (TVC): occuis

at inoculated site in individual with piioi
tubeiculosis infection.
Endogenous spiead: Lymphatics, hematog-
enous, bodily fluids (sputum, feces, uiine)
Lupus vulgaiis (LV)
Sciofulodeima (SD)

Metastatic tubeiculosis abscess (MTA)
Acute miliaiy tubeiculosis (AMT)
Oiificial tubeiculosis (OT)
Tubeiculosis due to BCG immunization
Type of clinical lesion depends on ioute of cu-
taneous inoculation and immunologic status of
the host. Cutaneous inoculation iesults in a tu-
beiculous chancie in the nonimmune host but
TVC in the immune host. Modes of endogenous
spiead to skin include the following: diiect ex-
tension fiom undeilying tubeiculous infection,
i.e., lymphadenitis oi tubeiculosis of bones and
joints, iesults in SD; lymphatic spiead to skin,
iesults in LV; hematogenous dissemination, ie-
sults in AMT, LV, oi MTA; autoinoculation fiom
body fluids (sputum, uiine, feces).
Ae oI 0oset AMT moie common in infants
and adults with advanced immunodeficiency.
PIT moie common in infants. SD moie com-
mon in adolescents, eldeily. LV affects all
ages.
Sex LV moie common in females. TVC moie
common in males.
0ccupatoo TVC: histoiically in physicians,
medical students, and pathologists as veiiuca
neciogenica, anatomist`s wait, postmoitem
wait; in butcheis and faimeis fiom M. |os .
Iocdeoce CTb has declined steadily woild-
wide, paialleling decline of pulmonaiy tubeicu-
losis. Always iaie in the United States compaied
with Euiope. Incidence of vaiious types of CTb
vaiies geogiaphically; LV, SD most common
types in Euiope; LV, veiiucous lesions moie
common in tiopics; TVC a common type in
developing countiies. Cuiiently, the incidence
of CTb has been incieasing, associated with
HIV/AIDS disease.
Iubercu|oss o hIv[AI0S 0sease Tubeiculosis
is the most common oppoitunistic infection
occuiiing in HAV/AIDS-infected individuals
who ieside in developing nations; CTb has
been iepoited in these individuals. Pioblem of
multidiug iesistance (MDR) is also common in
these peisons.
FAIh0CNSIS
Clinical lesions occuiiing in skin depend on
whethei host has had piioi infection with M.
u|ertu|oss , and theiefoie delayed hypeisensi-
tivity to the oiganism, and inoculation ioute
and mode of spiead.
Frmary Ioocu|atoo Iubercu|oss (Iubercu|ous
Chaocre) Initially, papule occuis at the inocu-
lation site 2-4 weeks aftei the wound. Lesion
enlaiges to a painless ulcei, i.e., a tubeiculous
chancie (up to 5 cm) (Fig. 24-64), with shal-
low gianulai base and multiple tiny abscesses
oi may be coveied by thick ciust. Undeimined
maigins; oldei ulceis become induiated with
thick ciusts. Deepei inoculation iesults in sub-
cutaneous abscess. Most common on exposed
skin at sites of minoi injuiies. Oial lesions
occui aftei ingestion of bovine bacilli in
nonpasteuiized milk; in the past, lesions in male
babies have occuiied on the penis aftei iitual
ciicumcision. Intiaoial inoculation iesults in
ulceis on gingiva oi palate. Regional lymphad-
enopathy occuis within 3-8 weeks.
Iubercu|oss verrucosa Cuts Initial papule
with violaceous halo. Evolves to hypeikeiatotic,
waity, fiim plaque (Fig. 24-65). Clefts and fis-
suies occui fiom which pus and keiatinous
mateiial can be expiessed. Boidei often ii-
iegulai. Lesions aie usually single, but multiple
lesions occui. Most commonly on doisolateial
hands and fingeis. In childien, lowei extiemi-
ties, knees. No lymphadenopathy.
Iupus vu|ars Initial papule ill defined and
soft and evolves into well-defined, iiiegulai
plaque (Fig. 24-66). Reddish-biown: diascopy
(i.e., use of glass slide piessed against skin)
ieveals an apple jelly" (i.e., yellowish-biown)
coloi. Consistency is chaiacteiistically soft; if
lesion is piobed, instiument bieaks thiough
oveilying epideimis. Suiface is initially smooth
oi slightly scaly but may become hypeikeia-
totic. Hypeitiophic foims iesult in soft tu-
moious nodules. Ulceiative foims piesent as
punched-out, often seipiginous ulceis sui-
iounded by soft, biownish infiltiate. Usually
solitaiy, but seveial sites may occui. Most le-
sions on the head and neck, most often on nose
and eais oi scalp. Lesions on tiunk and extiemi-
ties iaie. Disseminated lesions aftei seveie viial
infection (measles) ( |uus osexan|ematus ).
Involvement of undeilying caitilage but not
bone iesults in its destiuction (eais, nose).
Scaiiing is piominent, and, chaiacteiistically,
new biownish infiltiates occui within atiophic
scais.
ScroIu|oderma Fiim subcutaneous nodule
that initially is fieely movable; lesion then
becomes doughy and evolves into iiiegulai,
deep-seated node oi plaque that liquefies and
peifoiates (Fig. 24-67). Ulceis and iiiegulai
sinuses, usually of lineai oi seipiginous shape,
FICk 24-64 Frmary oocu|atoo tubercu|oss A |+|e, u|ce|+|ed uodu|e +| ||e ||e o| !,:|c:|-.
Iu|e|cu|o| |uocu|+||ou ou ||e |||| |||| +oc|+|ed W||| |uu|u+| |,rp|+deuop+||,. I|e e|,||er+|ou p+pu|e
ou ||e |e|| |o|e+|r occu||ed +| ||e ||e o| |u|e|cu||u |e||u.
dischaige pus oi caseous mateiial (Fig. 24-68).
Edges aie undeimined, inveited, and dissecting
subcutaneous pockets alteinating with soft,
fluctuating infiltiates and biidging scais. Most
often occuis in the paiotidal, submandibulai,
and supiaclaviculai iegions; lateial neck; SD
most often iesults fiom contiguous spiead fiom
affected lymph nodes oi tubeiculous bones
(phalanges, steinum, iibs) oi joints.
Metastatc Iubercu|oss Abscess Also called
u|ertu|ous gumma . Subcutaneous abscess,
nontendei, cold," fluctuant. Coalescing with
oveilying skin, bieaking down and foiming fis-
tulas and ulceis. Single oi multiple lesions, often
at sites of pievious tiauma.
Acute M|ary Iubercu|oss Exanthem. Dissem-
inated lesions aie minute macules and papules
oi puipuiic lesions. Sometimes vesiculai and
ciusted. Removal of ciust ieveals umbilication.
Disseminated on all paits of body, paiticulaily
tiunk.
0rIca| Iubercu|oss Small yellowish nod-
ule on mucosa bieaks down to foim painful
FICk 24-65 Iubercu|oss verrucosa
cuts C|u|ed +ud |,pe||e|+|o||c p|+que +|||u
+| ||e ||e o| |uocu|+||ou |u +u |ud|.|du+| p|e.|ou|,
|u|ec|ed W||| ! ||-:|. I|e|e | uo +oc|+|ed
|,rp|+deuop+||,.
ciiculai oi iiiegulai ulcei (Fig. 24-69) with
undeimined boideis and pseudomembianous
mateiial, yellowish tubeicles, and eioded ves-
sels at base. Suiiounding mucosa swollen,
edematous, and inflamed. Since OT iesults
fiom autoinoculation of mycobacteiia fiom
piogiessive tubeiculosis of inteinal oigans, it
is usually found on the oial, phaiyngeal (pul-
monaiy tubeiculosis), vulvai (genitouiinaiy
tubeiculosis), and anal (intestinal tubeiculosis)
mucous membianes. Lesions may be single oi
multiple, and in the mouth most often occui
on the tongue, soft and haid palate, oi lips. OT
may occui in a tooth socket aftei tooth extiac-
tion.
Frmary Ioocu|atoo Iubercu|oss Chanciifoim
syndiome: piimaiy syphilis with chancie, cat-
sciatch disease, spoiotiichosis, tulaiemia, M.
marnum infection.
Iubercu|oss verrucosa Cuts Veiiuca vulgaiis,
M. marnum infection, blastomycosis, chiomo-
mycosis, ecthyma, hypeitiophic lichen planus,
squamous cell caicinoma (SCC).
Iupus vu|ars Saicoidosis, lymphocytoma,
lymphoma, chionic cutaneous lupus eiythema-
tosus, teitiaiy syphilis, lepiosy, blastomycosis,
lupoid leishmaniasis.
ScroIu|oderma Invasive fungal infections, spo-
iotiichosis, nocaidiosis, actinomycosis, teitiaiy
syphilis, acne conglobata, hidiadenitis suppu-
iativa.
Metastatc Iubercu|oss Abscess Panniculitis,
invasive fungal infections, hidiadenitis, teitiaiy
syphilis.
0rIca| Iubercu|oss Aphthous ulceis, histo-
plasmosis, syphilis, SCC.
FICk 24-6T ScroIu|oderma: c|avc|e area
|+|e +|ce ue+| ||e c|+.|cu|+| |e+d, W||c| ou p|e
u|e e\||ude pu +ud c+eou r+|e||+|. A |u|e|cu|ou
|,rp|+deu||| uude|oe uec|o| W||| +|ce |o|r+
||ou, u|equeu||, d|+|u|u |o ||e ||u u||+ce.
FICk 24-66 Iupus vu|ars kedd||||oWu
p|+que, W||c| ou d|+cop, e\||||| ||e d|+uo||c ,e|
|oW||oWu +pp|eje||, co|o|. |o|e uodu|+| |u|||||+||ou
o| ||e e+||o|e, c+||u o| ||e |e||\, +ud +||op||c c+|
||u |u ||e ceu|e| o| ||e p|+que.
FICk 24-68 ScroIu|oderma: |atera| chest wa||. IWo u|ce| ou ||e c|e| W+|| +ud +\|||+ +|e +oc|+|ed
W||| uude||,|u |uu ||+c|.
FICk 24-69 0rIca| tubercu|oss: |ps A |+|e, .e|, p+|u|u| u|ce| ou ||e ||p o| ||| p+||eu| W|||
+d.+uced c+.||+|, pu|rou+|, |u|e|cu|o|.
0ermatopatho|oy PIT: initially nonspecific
inflammation; aftei 3-6 weeks, epithelioid cells,
Langhans giant cells, lymphocytes, caseation
neciosis. AMT: nonspecific inflammation and
vasculitis. All othei foims of CTb show moie
oi less typical tubeiculous histopathology; TVC
is chaiacteiized by massive pseudoepithelioma-
tous hypeiplasia of epideimis and abscesses.
Mycobacteiia aie found in PIT, SD, AMT, MTA,
and OT but only with difficulty oi not at all in
LV and TVC.
Cu|ture Yields mycobacteiia also fiom lesions
of LV and TVC.
FCk Can be used to identify M. u|ertu|oss
DNA in tissue specimens.
Sko Iesto PIT: Patient conveits fiom in-
tiadeimal skin test negative to positive (Fig.
24-70) duiing the fiist weeks of the infection.
AMT: usually negative. SD, MTA, and OT: may
be negative oi positive depending on state of
immunity. LV and TVC: positive.
0IACN0SIS
Clinical, histologic findings, confiimed by iso-
lation of M. u|ertu|oss on cultuie oi by PCR.
C0kS AN0 Fk0CN0SIS
The couise of CTb is quite vaiiable, depending
on the type of cutaneous infection, amount of
inoculum, extent of extiacutaneous infection,
age of the patient, immune status, and theiapy.
PIT: without tieatment, usually iesolves within
12 months, with some iesidual scaiiing. Raiely,
LV develops at site of PIT. Tubeiculosis due to
BCG immunization: depends on geneial state
of immunity. It may assume appeaiance and
couise of PIT, LV, oi SD; in immunocompio-
mised may lead to MTA oi AMT.
MANACMNI
Only PIT and TVC limited to the skin. All othei
patteins of CTb aie associated with systemic
infection that has disseminated secondaiily to
skin. As such, theiapy should be aimed at
achieving a cuie, avoiding ielapse, and pievent-
ing emeigence of diug-iesistant mutants.
Aottubercu|ous Iherapy Piolonged antitu-
beiculous theiapy with at least two diugs is
indicated foi all cases of CTb except foi TVC
that can be excised.
Standaid antitubeiculous theiapy:
Isoniazid (5 mg/kg daily) |us
Rifampin (600 mg/kg daily)
Supplemented in initial phases with:
Ethambutol (25 mg/kg daily) anJ/or
Stieptomycin (10-15 mg/kg daily) anJ/or
Pyiazinamide (15-30 mg/kg daily)
Isoniazid and iifampin foi at least 9 months;
can be shoitened to 6 months if foui diugs aie
given duiing the fiist 2 months.
Mu|tdru kesstaot (M0k) Ib Incidence is
incieasing.
FICk 24-T0 FurIed proteo dervatve or

Maotoux test: postve test A !,e+|o|d I+|W+uee
|er+|e W||| po||+|, W||| + ue+||.e ||u |e| oue ,e+|
p|e.|ou|,, W+ |e|e|ed p||o| |o |e|uu|u eu|+ue|cep|.
'|e |+d |ecore |u|ec|ed W|||e .||||u |e| |+||e|, W|o
|+d pu|rou+|, |u|e|cu|o|, |u I+|W+u. A |ed p|+que
W||| u||ouud|u e|,||er+ | eeu +| ||e |e| ||e.
SCII0N 24 BACIEk|A| |||ECI|0|' ||\0|\||C InE 'K|| 6TT
|ou|u|e|cu|ou r,co|+c|e||+ (|I\) de||ued +
r,co|+c|e||+ o||e| ||+u ! ||-:| corp|e\
+ud ! |-c-
I|e o|||u+| c|+|||c+||ou o| |I\ depeuded ou
peed o| |oW||, ro|p|o|o,, +ud p|reu|+||ou
o| co|ou|e ou o||d red|+ + We|| + ||oc|er|c+|
|e+c||ou (I+||e 2+0). Cu||eu||,, |oWe.e|, ro
|ecu|+| p|o|e +|e ued |o| |+p|d |deu||||c+||ou o|
||e ro| |rpo||+u| pec|e |u + po|||.e cu||u|e,
|,|||d|/+||ou o| ||e p|o|e |o pec|||c equeuce
o| ||e r,co|+c|e||+.
0ccu| u+|u|+||, |u ||e eu.||oureu|
C+p+||e o| c+u|u p||r+|, |u|ec||ou |u o||e|
W|e |e+|||, |ud|.|du+| +ud ro|e e||ou |u|ec
||ou |u |rruuocorp|or|ed, e.., ! c
! |:-c +ud ! ||| corp|e\
|rruuocorpe|eu| |ud|.|du+|. p||r+|, cu|+ue
ou |u|ec||ou +| ||e o| |uocu|+||ou. |odu|e,
|,rp|ocu|+ueou o| po|o|||c|o|d |e|ou. |u|uu
cu|o|.
|rruuocorp|or|ed |o|. d|er|u+|ed ru
co+| +ud cu|+ueou |e|ou
||+uo|. de|ec||ou o| r,co|+c|e||+ |||oc|er|
c+||, o| |, cu||u|e ou pec|||c red|+
|eW ro|ecu|+| |ec|u|que |+ed ou ||A +r
p||||c+||ou +cce|e|+|e d|+uo|, |deu|||, corrou
ou|ce o| |u|ec||ou, |e.e+| ueW |,pe o| |I\
I|e+|reu|. c|+|||||or,c|u, |||+rp|c|u, ||uo|oqu|
uo|oue, r|uoc,c||ue
',,. A|,p|c+| r,co|+c|e||+, r,co|+c|e||+
o||e| ||+u |u|e|cu|o| (\0II)
N0NI8kCI0S MC08ACIkIAI INFCII0NS |C|9 . 0!.
|C|0 . A!.
IA8I 24-10 Species of Nontubercu|ous Nycobacterium (NIN) Causin Cutaneous |nfection
6rowth oo ov|roomeota|
Spec|es So||d Ned|a 8eservo|r 00taoeo0s |oIect|oo
! c '|oW ||| |+u|, +|| W+|e| \o| corrou
\e||ucou uodu|e/p|+que
C|+uc|||o|r |e|ou
'po|o|||c|o|d |u|ec||ou
'o|||+|, p|+que ou |+ce
! |:-c '|oW |+|u|+| W+|e| u|ce|ou o|eor,e||||
! c|:- k+p|d |+|u|+| +ud po|+||e W+|e|, o|| |u||+rr+|o|, p|+que +| |ujec||ou ||e
! ||| k+p|d |+|u|+| +ud po|+||e W+|e|, o|| \,co|+c|e||+| |u|uucu|o|
! :|-|c- k+p|d |+|u|+| +ud po|+||e W+|e|, o|| |odu|e, u|ce|+|ed uodu|e
! |c-|| '|oW uu|uoWu Cu|+ueou uodu|e, u|ce|+|ed
|+||d|ou uodu|e, po|o|||c|o|d p+||e|u,
r,o|||, |,rp|+deu|||,
d|er|u+|ed |u|ec||ou |u
|rruuocorp|or|ed p+||eu|
E||o|o,. ! c
E\pou|e |u +queou eu.||oureu|, |.e., ||| |+u|,
poo|, W+|e|
|u|ec||ou |o||oW ||+ur+||c |uocu|+||ou
C||u|c+| ||ud|u.
|odu|e(). .e||ucou, e|oded.
|,rp|ocu|+ueou e\|eu|ou W||| po|o|||c|o|d
p+||e|u.
Ieuo,uo.|||
||+uo|. |o|+|e ! c ou cu||u|e
I|e+|reu|. C|+|||||or,c|u o| r|uoc,c||ue. Cor||
u+||ou +u|||u|e|cu|ou ||e|+p,
',,. ||| |+u| |+uu|or+, W|rr|u poo|
|+uu|or+
MYC08CIFkIM MkINM INFCII0N
FI0MI0I0C
Ae oI 0oset Second to fouith decades.
to|oy M. marnum .
xposure Tiopical fish tanks; lesions usually
on dominant hand. Chloiination has ieduced
tiansmission in swimming pools.
Iocubatoo Ferod Vaiiable: usually 1 week to 2
months aftei inoculation.
Symptoms Commonly asymptomatic. Poss-
ible local tendeiness; limitation of movement if
lesion ovei a joint; with deepei extension, pain.
Sko Iesoos Inoculation site: papule(s) en-
laiging to inflammatoiy, ied to ied-biown nod-
ule oi plaque 1-4 cm in size on dominant
hand. Suiface of lesions may be hypeikeiatotic/
veiiucous (Fig. 24-71). May become ulcei-
ated: supeificial ciust, gianulation tissue base,
seiosanguineous oi puiulent dischaige. In
some cases, small satellite papules, diaining
sinuses, fistulas may develop. Usually solitaiy,
ovei bony piominence. Moie extensive soft
tissue infection may occui, with osteomyelitis,
FICk 24-T1 M. moroum: oocu|atoo ste oIectoo oo the Ioot A !,e+|o|d r+|e W||| p+|u|u|
|udu|+|ed p|+que ou ||e |+|e|+| do|+| |oo|. I|e |e|ou +|oe +| ||e ||e o| + r+|| ||||e| oue ,e+| +o W|||e |u
A||+u||+u. I||ee p|e.|ou ||op|e +ud ||ue cu||u|e |+d |eeu uuucce|u| +| r+||u + d|+uo|. A||e|
|u||+|e|ou+| |ujec||ou o| |||+rc|uo|oue .5 r/r|, +c|d-|+| |+c|||| We|e |deu||||ed |u ||e ||op, pec|reu +ud !
c |o|+|ed ou cu||u|e. ne W+ ucce|u||, ||e+|ed W||| |ou| +u||r,co|+c|e||+| +eu|. 0|d wo||d |e||r+u|
+| W+ ou ||e d|||e|eu||+| d|+uo| o| ||| |e|ou.
leishmaniasis, syphilis, yaws, iododeima, bio-
modeima, foieign-body iesponse to sea uichin
oi bainacle, benign oi malignant skin tumois.
Sporotrchod Iesoo Staphylococcal oi gioup
A stieptococcal lymphangitis, spoiotiichosis,
tulaiemia, leishmaniasis, nocaidiosis, actino-
mycosis.
Sko Iests Intiadeimal tubeiculin test (PPD-S)
often positive.
Sko 8opsy Suggestive but not pathogno-
monic. Oldei lesions: Moie typical tubeiculoid
aichitectuie is developed with epithelioid cells
and Langhans giant cells. Acid-fast stain dem-
onstiates M. marnum only in appioximately
50% of cases.
0rect Mcroscopy
Smeurs v] Erudute vr Pus Acid-fast bacilli can
be demonstiated in some cases.
Cu|ture M. marnum giows at 32C (but
not at 37C) in 2-4 weeks. Eaily lesions yield
numeious colonies. Lesions 3 months oi
oldei geneially yield few colonies.
0IACN0SIS
Histoiy of tiauma in an aqueous enviion-
ment, clinical findings, confiimed by isola-
tion of M. marnum on cultuie.
C0kS AN0 Fk0CN0SIS
Most usually benign and self-limited but can
iemain active foi a piolonged peiiod. Single
papulonodulai lesions iesolve spontane-
ously within 3 months to 3 yeais, wheieas
spoiotiichoid foim can peisist foi yeais. In
the immunocompiomised host, most exten-
sive deep infection can occui.
in the immunocompiomised host. Atiophic
scaiiing follows spontaneous iegiession oi suc-
cessful theiapy.
SpvrvtrIchvId vr Lymphvcutunevus Puttern
Deep-seated nodules in a lineai configuiation
on hand and foieaim exhibit lymphocutaneous
spiead (Fig. 24-72). Boggy inflammatoiy ieac-
tion may mimic buisitis, synovitis, oi aithii-
tis about the elbow, wiist, oi inteiphalangeal
joints. Tenosynovitis.
DIssemInuted 1n]ectIvn Raie. May occui in
immunocompiomised host.
Iymph Nodes Regional lymphadenopathy un-
common.
So|tary verrucous[|cerated Iesoo oo
xtremty Veiiuca vulgaiis, spoiotiicho-
sis, blastomycosis, eiysipeloid, tulaiemia,
tubeiculosis veiiucosa cutis, nocaidiosis,
FICk 24-T2 M. moroum: |ymphocuta-
oeous oIectoo beooo oo Ioer A +3
,e+|o|d |er+|e W||| p+|u|u| We|||u o| ||e ||||
r|dd|e ||ue| |o| + rou||. '|e |ec+||ed c|e+u|u
+ ||| |+u| e.e|+| Wee| |e|o|e ||e d||+| d|||+|
|ec+re |ed +ud |eude|. I|e ||ue| +ud |+ud
|ec+re p|o|e|.e|, ro|e |u||+red +ud |ed
uodu|e +ppe+|ed ou ||e |o|e+|r. '|||| eu|+|e
reu| o| +\|||+|, uode W+ de|ec|ed.
FI0MI0I0C
Ae oI 0oset Childien, young adults.
to|oy M. u|terans . An enviionmental
habitat foi the oiganism has not been estab-
lished. Piogenitoi of M. u|terans thought to be
M. marnum .
Iraosmssoo Inoculation piobably via minoi
tiauma occuiiing in wet, maishy, oi swampy
sites. Bites of aquatic insects; M. u|terans iep-
licates in insect salivaiy glands; in endemic
aieas, 5-10% of aquatic insects have miciobe in
salivaiy gland.
0emoraphy Tiopics, most infections in
Afiica and Austialia.
FAIh0CNSIS
A secieted polyketide toxin, mycolactone, sup-
piesses immune iesponse to miciobe.
Iocubatoo Ferod Appioximately 3 months.
E||o|o,. ! |:-c
I|+ur||ou. |uec| |||e, |.e., roqu||oe, ||+u
r+||c |uocu|+||ou
Ceo|+p|,. occu| |u !0 couu|||e. I|op|c+| |e
|ou o| We| A|||c+, Au||+||+, |+pu+ |eW Cu|ue+,
Ceu||+| \e\|co. C||r+|e. |ur|d, |o|.
|+||oeue|. ! |:-c p|oduce r,co|+c|oue,
W||c| |+ + |o\|c e||ec| ou ||ue +ud |u||||| |r
ruue |epoue
Ae. 0-+ ,e+|, 15-19 ,e+|.
'e\. ro|e corrou |u r+|e |u o|de| |ud|.|du+|
|uc|deuce. ||||d ro| corrou r,co|+c|e||+|
|u|ec||ou +||e| |u|e|cu|o| +ud |ep|o,
C||u|c+| ||ud|u.
|e|ou. p+pu|e e.o|.|u |o |+|e u|ce|. \+,
|e ru|||p|e. 0|eor,e||||.
||||||u||ou. e\||er|||e, |oWe| ro|e o||eu ||+u
uppe|
||+uo|. |deu||||c+||ou o| r|c|o|e ou cu||u|e o|
|, |Ck
Cou|e. u|ce| |u o|de| +e r+, |e |e+c||.+||ou o|
|+|eu| |u|ec||ou. 'pou|+ueou |e+||u r+, occu|.
',,. Bu|u|| u|ce|, Bu|u|| u|ce| d|e+e (Bu|)
(A|||c+), B+||ud+|e o| |+|u||ee u|ce| (Au||+||+)
MYC08CIFkIM ICFkN5 INFCII0N |C|9 . 0!.
|C|0 . A!.
MANACMNI
Frophy|axs Weai wateipioof gloves when
woiking in fish tank.
Aotbotcs Diug of fiist choice: claiithiomy-
cin and eithei iifampin oi ethambutol foi
1-2 months aftei lesion(s) have iesolved (3-4
months). Minocycline alone may be effective.
Susceptibility testing is not ioutinely iecom-
mended and should be ieseived foi cases of
tieatment failuie.
Surca| 0ebrdemeot May be iequiied foi
deep tissue involvement, especially in immuno-
compiomised host.
Symptoms The eaily nodule at site of tiauma
and subsequent ulceiation aie usually painless.
Sko Iesoos A painless subcutaneous swell-
ing occuis at the site of inoculation. Papule(s),
nodule(s), plaques often oveilooked. Lesion
enlaiges and ulceiates. The ulcei extends into
the subcutaneous fat, and its maigin is deeply
undeimined (Fig. 24-73). Ulceiations may en-
laige to involve an entiie extiemity. Legs moie
commonly involved (sites of tiauma). Any site
may be involved. Soft tissue and bony involve-
ment can occui. As ulceiations healed, scaiiing
and disabling defoimities may occui.
Systemc Fodos Fevei, constitutional find-
ings aie usually absent. Regional lymph nodes
usually not enlaiged. Undeilying destiuctive
osteomyelitis.
Subcutaoeous Ioduratoo Panniculitis, phyco-
mycosis, nodulai vasculitis, pyomyositis.
Iare Cutaoeous |ceratoo Blastomycosis,
spoiotiichosis, nocaidiosis, actinomycosis,
mycetoma, chiomomycosis, pyodeima gan-
gienosum, basal cell caicinoma, squamous cell
caicinoma, neciotizing soft tissue infections.
8actera| Cu|ture Rule out secondaiy bacteiial
infection.
Mycobactera| Cu|ture M. u|terans giows opti-
mally at 32-33C.
FCk Repoited to be effective.
0ermatopatho|oy Neciosis oiiginates in the
inteilobulai septa of the subcutaneous fat. Pooi
inflammatoiy iesponse despite clusteis of ex-
tiacellulai bacilli. Ulceiation is suiiounded by
gianulation tissue with giant cells but no casea-
tion neciosis oi tubeicles. Acid-fast bacilli aie
always demonstiable.
0IACN0SIS
Clinical findings confiimed by isolation of M.
u|terans fiom lesional skin biopsy specimen.
C0kS AN0 Fk0CN0SIS
Because of delay in diagnosis and tieatment,
lesions aie often extensive at piesentation. Ul-
ceiations tend to peisist foi months to yeais.
FICk 24-T3 M. ulceroos: 8uru| u|cer A 5,e+|o|d u+ud+u r+|e W||| + |ue u|ce| W||| + c|e+u |+e
+ud uude|r|ued r+||u e\|eud |u|o ||e u|cu|+ueou ||ue. (Cou||e, o| \. ||||||c|, \|.)
Spontaneous healing occuis eventually in many
patients. Ulceiation and healing can be compli-
cated by scaiiing, contiactuie of the limb, and
lymphedema. Malnutiition and anemia delay
healing.
MANACMNI
Freveotoo Covei exposed extiemities with
clothing; bed netting. Avoid mosquito bites.
Insect iepellants. Immediately wash minoi skin
wounds.
Symptomatc In that M. u|terans piefeis coolei
tempeiatuies, application of heat to the in-
volved site has been iepoited to be effective.
Impiove nutiitional status.
Aotmycobactera| 0ru Iherapy WHO iecom-
mends iifampicin and stieptomycin (iequiies IM
injection) combined with suigeiy. Combination
of iifampicin and cipiofloxacin may be effective.
Stieptomycin. 8 weeks of antibiotic tieatment
may ieveise local immunosuppiession.
Surery Excision of the infected tissue with a
small iim of noimal tissue, usually followed by
giafting. Reconstiuctive suigeiy.
E||o|o,. ! ||| ! :|-|c- ! c|:-

|+|u|+| |ee|.o||. W+|e|, o||, uoocor|+| eu.|
|oureu|
Cu|+ueou |u|ec||ou +ccouu| |o| o0 o| \|C
|u|ec||ou
C||u|c+| ||ud|u.
|rruuocorpe|eu| |ud|.|du+|. |oc+||/ed |u|ec||ou
|u ||+ur+||c o| u||c+| Wouud, |ujec||ou ||e
|rruuoupp|eed |ud|.|du+|. d|er|u+|ed
cu|+ueou |u|ec||ou
I|e+|reu|. Au||||o||c() u|e|,
MYC08CIFkIM F0kIIIM C0MFIX (MFC) INFCII0NS |C|9 . 0!.
|C|0 . A!.
FI0MI0I0C
Ae oI 0oset Childien, young adults.
Sex Females males.
to|oy MFC oiganisms include M. [oru-
um , M. t|e|onae , M. a|stessus .
Natura| keservors The oiganisms aie widely
distiibuted in soil, dust, and watei. Can be
isolated fiom tap watei, municipal watei sup-
plies, moist aieas in hospitals, contaminated
biologicals, aquaiiums, domestic animals, ma-
iine life.
Iraosmssoo Inoculation via tiaumatic punc-
tuie wounds oi suigical pioceduies/injections.
Silicone injections. Whiilpool footbaths in nail
salons ( M. [oruum ).
Iocubatoo Ferod Usually within 1 month
(iange 1 week to 2 yeais).
hstory Suigical wound infections follow aug-
mentation mammaplasty, median steinotomy,
and peicutaneous catheteiizations.
Symptoms Infection piesents as a painful
tiaumatic oi suigical wound infection.
Sko Iesoos Postinjection abscesses. Tiau-
matic wound infections (Fig. 24-74) piesent
as daik ied, infiltiated nodule, abscess foi-
mation, diainage of seious exudate. Lineai
lesions, commonly at incision sites. Tiaumatic
infection occuis moie commonly on the ex-
tiemities. Suigical infections occui in scais of
median steinotomy and augmentation mamma-
plasty. Foot baths associated with fuiunculosis.
In immunocompiomised individuals, infection
can disseminate hematogenously to skin (mul-
tiple iecuiiing abscesses on the extiemities)
and joints.
Ceoera| Fodos Othei piimaiy MFC infec-
tions include pneumonitis, osteomyelitis, lym-
phadenitis, postsuigical endocaiditis.
Iraumatc aod Fostoperatve Wouod IoIec-
too S. aureus and gioup A stieptococcus
infections, vaiious othei bacteiia, foieign-body
ieaction, alleigic contact deimatitis to topically
applied agent, CanJJa a||tans infection, s-
erg||us spp. infection.
infection.
Mycobactera| Cu|ture MFC oiganisms usually
can be isolated on piimaiy cultuie in 2-30 days.
FCk May be diagnostic if available
0ermatopatho| oy Polymoiphonucl eai
micioabscesses and gianuloma foimation with
foieign-body-type giant cells (dimoiphic in-
flammatoiy iesponse) aie seen. Neciosis is
often piesent without caseation. AFB can be
seen within micioabscesses.
0IACN0SIS
Clinical findings confiimed by isolation of
MFC fiom lesional skin biopsy specimen oi
identifiy by PCR.
C0kS AN0 Fk0CN0SIS
The infection becomes chionic unless tieated
with antimycobacteiial theiapy, suigical de-
biidement.
MANACMNI
Aotmycobactera| Chemotherapy Depends on
species and sensitivities. Effective agents include
claiithiomycin, amikacin, cefoxitin, imipenem,
doxycycline, fluoioquinolone.
Surery Debiidement with delayed closuie is
effective foi localized infections.
FICk 24-T4 M. lertutum oIectoo A +5,e+|o|d |er+|e W||| e|,||er+|ou |eude| uodu|e ou ||e
|oWe| |e. I|e |e|ou occu||ed e.e|+| Wee| +||e| + ped|cu|e |u + |oo| c+|e +|ou. '|+.|u o| |e r+, |+.e
|+c||||+|ed ||e |u|ec||ou. ! ||| W+ |o|+|ed ou cu||u|e o| ||u ||op, pec|reu.
to|oc Aeot B. |urgJor[er sensu lato ( B.
|urgJor[er in the geneial sense); 13 closely ie-
lated species have been identified. LB is caused
by thiee pathogenic genospecies. Clinical vaii-
ations of disease occuiiing in Noith Ameiica,
Euiope, and Asia may be ielated to diffeiences
in the vaiious causative stiains. B. |urgJor[er
has been identified in 19 states in the United
States.
vector Infected nymphal tick of genus IxoJes
ratnus complex. Thiee stages of tick develop-
ment: laival, nymphal, adult; each stage iequiies
blood meal. Piefeiied host foi laival and nym-
phal I. stau|ars is white-footed mouse (and
ceitain othei iodents). The tiny nymphal tick
tiansmits B. |urgJor[er to humans in eaily
summei. Piefeiied host of adult I. stau|ars
(Fig. 24-75) is white-tailed deei, which is not
involved in life cycle of spiiochete but is ciitical
to the suivival of the tick. In United States, I.
stau|ars also tiansmits babesiosis and human
anaplasmosis. In Euiope and Asia, I. rtnus
and I. ersu|taus also tiansmit tick-boine en-
cephalitis.
Iraosmssoo Ticks cling to vegetation; aie
most numeious in biushy, wooded, oi giassy
habitats; not found on open sandy beaches.
B. |urgJor[er is tiansmitted to humans aftei
biting and feeding of nymphs oi, less com-
monly, adult ticks. Tiansmission to humans
occuis in association with hiking, camping, oi
hunting tiips and with iesidence in wooded oi
iuial aieas.
Seasoo In the midwestein and eastein United
States, late May thiough eaily fall (80% of
eaily LB begins in June and July). In the Pacific
Noithwest, Januaiy thiough May.
ksk Ior xposure Stiongly associated with
pievalence of tick vectois and piopoition
of those ticks that caiiy B. |urgJor[er . In
the noitheastein United States with endemic
disease, the infection iate of the nymphal I.
A corp|e\, ru|||,|er d|e+e
E||o|o|c +eu|. 3-|c p||oc|e|e
I |+ur|||ed |o |ur+u |, ||e |||e o| +u |u|ec|ed
|\od|d ||c|
I||ee |+e o| |B.
'|+e | |oc+||/ed
'|+e 2 (uu||e+|ed |+e ) | d|er|u+|ed
'|+e ! | pe|||eu| |u|ec||ou, de.e|op|u
rou|| o| ,e+| |+|e|
I|e c||u|c+| ||ud|u +oc|+|ed W||| e+c| |+e +|e
|||ed |u I+||e 2+.
',,. |,re d|e+e
IM 80kkII0SIS (I8) |C|9 . 033.3
|C|0 . Ao9.2
stau|ars tick with B. |urgJor[er is commonly
20-35%. Risk associated with hiking, camping,
oi hunting tiips and with iesidence in wooded
oi iuial aiea.
Iocdeoce LB is the most common vectoi-
boine infection in the United States, with
20,000 cases iepoited annually.
FAIh0CNSIS
Aftei inoculation into the skin as nymphal tick
feeds, spiiochetes ieplicate and migiate outwaid,
pioducing the eiythema migians (EM) lesion,
and invade vessels, spieading hematogenously
to othei oigans. The spiiochete has a paiticulai
tiophism foi tissues of the skin, neivous system,
and joints. The oiganism peisists in affected tis-
sues duiing all stages of the illness. The immune
iesponse to the spiiochete develops giadually.
Specific IgM antibodies peak between the thiid
and sixth weeks aftei disease onset. The specific
IgG iesponse develops giadually ovei months.
Pioinflammatoiy cytokines TNF- , and IL-1
aie pioduced in affected tissues.
Iocubatoo Ferod EM: 3-32 days aftei tick
bite. Caidiac manifestations: 35 days (3 weeks
to 5 months aftei tick bite). Neuiologic mani-
festions: aveiage 38 days (2 weeks to months)
aftei tick bite. Rheumatologic manifestations:
67 days (4 days to 2 yeais) aftei bite.
Frodrome With disseminated infection (stage
2), malaise, fatigue, lethaigy, headache, fevei,
chills, stiff neck, aithialgia, myalgia, backache,
anoiexia, soie thioat, nausea, dysesthesia, vom-
iting, abdominal pain, photophobia.
hstory Because of small size of nymphal tick,
most patients aie unawaie of tick bite. Ixodid
tick bites aie asymptomatic. Removal of the
pinhead-sized tick within 18 h of attachment
IA8I 24-11 Stain of LYNE Borre|iosis
Stage 0||o|ca| F|od|ogs
ar|y oIectoo: stae 1 E|,||er+ r||+u (E\)
(|oc+||/ed |u|ec||ou) |,rp|oc,|or+
ar|y oIectoo: stae 2 ',|er|c ,rp|or (|e.e|, c||||, r,+||+, |u|ec||ou,
(d|er|u+|ed) |e+d+c|e, We+|ue, p|o|op|o||+)
'ecoud+|, E\
C+|d|||
\eu|u|||, c|+u|+| ueu||||, |+d|cu|oueu|op+||,
A||||+||+/r,+||+
Iate oIectoo: stae 3
(pe|||eu| |u|ec||ou) A|||||||
Eucep|+|or,e||||
Ac|ode|r+|||| c||ou|c+ +||op||c+u (ACA)
may pieclude tiansmission. EM may be associ-
ated with buining sensation, itching, oi pain.
Only 75% of patients with LB exhibit EM. Joint
complaints moie common in Noith Ameiica.
Neuiologic involvement moie common in Eu-
iope. With peisistent disease, chionic fatigue.
Sko Fodos See Table 24-11.
Stae 1 Ioca|ted IoIectoo Erythemu MIgruns
Initial eiythematous macule oi papule enlaiges
within days to foim an expanding annulai lesion
with a distinct ied boidei and paitially cleaiing
middle, i.e., a migiating eiythema, at the bite
site (Fig. 24-76). Maximum median diametei
is 15 cm (iange 3-68 cm). The expanding le-
sion may have seveial iings of vaiying shades of
ied (taigetoid oi bull`s eye). At times, concen-
tiic iings foim. When occuiiing on the scalp,
only a lineai stieak may be evident on the face
oi neck. Multiple EM lesions aie seen with mul-
tiple bite sites. Most common sites : thigh, gioin,
axilla. Centei may become induiated, vesiculai,
oi neciotic. Less common: cential hemoiihagic
vesiculation oi neciosis, lymphangitic stieaks.
Hypeisensitivity to vaiious tick antigens, othei
pathogens, and outei boiielial suiface pio-
teins occui in some individuals. As EM evolves,
postinflammatoiy eiythema oi hypeipigmen-
tation, tiansient alopecia, and desquamation
may occui. 25% of patients do not exhitit EM
lesion.
Bvrre|Iu| Lymphvcytvmu Mainly seen in Eu-
iope. Usually aiises at the site of tick bite.
Some patients have a histoiy of EM; otheis
may show concomitant EM located aiound
oi neai the lymphocytoma. Usually piesents
FICk 24-T5 Ixedes scopulors (deer tck)
Ieedo A u,rp| W||| || rou|| p+|| +||+c|ed |o
||u W||| u||ouud|u e|,||er+, ||| |u||+rr+||ou
| + |epoue |o ||e |||e ||e|| +ud | uo| uece+|||,
|ud|c+||.e o| |u|ec||ou. I|+ur||ou o| 3-|c |
!|- uu+||, occu| ou|, +||e| p|o|oued +||+c|reu|
+ud |eed|u (>3 |).
FICk 24-T6 Iyme borre|oss: erythema mraos (M) oo upper thh A 15,e+|o|d r+|e uo|ed
+u +,rp|or+||c |ed p|+que ou || |||| ||e d+, o| ||e e\+r|u+||ou. ne |e|| We||, +ud W+ uu+W+|e o| ||c| |||e.
. ked p|+que W||| |+d|u r+||u (e|,||er+ r||+u). I|e d+, +||e| |e|uu|u do\,c,c||ue, 00 r ||d, |e
e\pe||euced ||u|||e ,rp|or (l+||c|ne|\|e|re| |e+c||ou). 8. |ou| d+, +||e| |e|uu|u ||e+|reu|, ||e E\
|e|ou | ruc| |+|e|, ,rp|or |+d |eo|.ed.
8
FICk 24-TT Iyme borre|oss: |ym-
phocytoma cuts 'o|||+|,, |edpu|p|e
uodu|e ou ||e c|+|+c|e||||c ||e o| ||e e+|.
as a solitaiy bluish-ied nodule (Fig. 24-77) oi
plaque. Sites of piedilection: eailobe (childien),
nipple/aieola (adults), aieola, sciotum; 3-5 cm
in diametei. Usually asymptomatic.
Other Cutunevus FIndIngs Malai iash, diffuse
uiticaiia, subcutaneous nodules (panniculitis).
Stae 2 0ssemoated IoIectoo Secvndury
LesIvns Piesent in 17-50% of patients with
eaily disseminated LB in Noith Ameiica; moie
common in Euiope. Lesions iange in numbei
fiom 2 to >100 and thus may piesent as iash.
Secondaiy lesions iesemble EM but aie smallei,
migiate less, and lack cential induiation and
may be scaly (Fig. 24-78). Lesions occui at any
site except the palms and soles; can become con-
fluent. When face, hands, feet aie involved, mild
swelling can occui.
Stae 3 Ferssteot IoIectoo: Acrodermatts
Chrooca Atrophcaos (ACA) Associated with
B. a[:e| infection in Euiope and Asia. Moie
common in eldeily women.
Eur|y 1n]|ummutvry Phuse (Mvnths tv Yeurs)
Initially, diffuse oi localized violaceous eiy-
thema, usually on one extiemity, accompanied
by mild to piominent edema, most commonly
involving the extensoi suifaces and peiiaiticulai
aieas. Asymptomatic dull ied infiltiated plaques
aiise on the extiemities, moie commonly on
lowei legs than foieaims, which slowly extend
centiifugally ovei seveial months to yeais, leav-
ing cential aieas of atiophy.
Endstuge Skin becomes atiophic, veins and
subcutaneous tissue become piominent, easily
lifted and pushed into fine accoidion-like folds,
i.e., cigaiette papei" oi tissue papei" skin (Fig.
24-79). Lesions may be single oi multiple.
Sc|ervtIc vr FIhrvtIc P|uques und Bunds
Localized fibiomas and plaques aie seen as
subcutaneous nodules aiound the knees and
elbows; may involve the joint capsule with
subsequent limitation of movement of joints in
hands, feet, oi shouldeis. Fibiotic/scleiotic band
along ulna is pathognomonic (ulnai band").
Ceoera| Fodos See Table 24-11.
Stae 1 None.
Stae 2 (0ssemoated IoIectoo) Fevei: in
adults, low-giade; in childien, may be high and
peisistent. Regional lymphadenopathy, geneial-
ized lymphadenopathy.
Neurv|vgIc 1nvv|vement Occuis in 10-20%
of untieated LB cases, 1-6 weeks (oi longei)
aftei the tick bite. Manifested as meningitis
(exciuciating headache, neck pain), subtle en-
cephalitic signs (sleep distuibances, difficulty
concentiating, pooi memoiy, iiiitability, emo-
tional lability, dementia), cianial neuiitis (in-
cluding bilateial facial palsy), motoi oi sensoiy
iadiculoneuiopathy, mononeuiitis multiplex,
oi myelitis. In the United States, most common
piesentation is fluctuating symptoms of menin-
gitis accompanied by facial palsy and peiipheial
iadiculoneuiopathy. In Euiope and Asia, the
fiist sign is chaiacteiistically iadiculai pain
followed by CSF pleocytosis (Bannwaith syn-
diome); meningeal and encephalitic signs aie
often absent. Eaily neuiologic manifestations
usually iesolve within months; chionic neuio-
logic disease may occui latei.
CurdIuc 1nvv|vement Occuis in 8% of un-
tieated cases, usually within 4 weeks. Mani-
fested by fluctuating degiees of atiioventiiculai
block, myopeiicaiditis, and left ventiiculai dys-
function. Usually tiansient and not associated
with long-teim sequelae.
Muscu|vs|e|etu| 1nvv|vement Common. Mi-
giatoiy pain in joints, tendons, buisae, muscles,
oi bones. Pain lasts houis oi days, affecting one
oi two locations at a time.
Stae 3 (Ferssteot IoIectoo) Fevei: in adults,
low-giade; in childien, may be high and piesist-
ent. Regional lymphadenopathy, geneialized
lymphadenopathy.
ChrvnIc Neurvhvrre|IvsIs May become ap-
paient months oi yeais aftei onset of latent
infection. Less common than aithiitis. Most
common piesentation is subtle encephalopathy
(alteied memoiy, mood, oi sleep), often ac-
companied by axonal polyneuiopathy (distal
paiesthesias, spinal iadiculai pain). Piolonged
couise iesembles that of teitiaiy syphilis.
ArthrItIs Moie common in United States,
occuiiing in 60% of untieated cases. Chaiac-
teiized by inteimittent attacks of oligoaiticulai
aithiitis in laige joints (especially knees), last-
ing weeks to months. In a small peicentage of
cases, involvement of laige joints (usually one
oi both knees) becomes chionic and may lead
to destiuction of caitilage and bone.
M Insect bite (annulai eiythema caused by
ticks, mosquitoes, Hymenopteia), epideimal
deimatophytoses, alleigic contact deimatitis,
heiald patch of pityiiasis iosea, gianuloma
annulaie, eaily inflammatoiy moiphea, cellu-
litis, uiticaiia, eiythema multifoime, eiythema
annulaie centiifugum, involuting psoiiasis le-
sion, lichen simplex chionicus, fixed diug
eiuption.
LB-like illness with exposuie in midwest and
southein United States tiansmitted by Lone
Stai tick (m||yomma amertanum); iefeiied to
as Sou|ern t|-assotaeJ ras| ||ness (STARI).
Secoodary Iesoos Secondaiy syphilis, pityiia-
sis iosea, eiythema multifoime, uiticaiia.
Iymphocytoma Insect bite ieaction, pseud-
olymphoma, cutaneous lymphoma.
ACA Aiteiial insufficiency of the lowei leg, ve-
nous insufficiency with stasis deimatitis, venous
thiombosis/thiombophlebitis.
Fbrotc Nodu|es Rheumatic nodules, gouty
tophi, eiythema nodosum.
Sko 8opsy EM Deep and supeificial
peiivasculai and inteistitial infiltiate contain-
ing lymphocytes and plasma cells with some
degiee of vasculai damage (mild vasculitis oi
hypeivasculai occlusion). Spiiochetes can be
demonstiated in up to 40% of EM biopsy
specimens.
ACA Eaily, peiivasculai inflammatoiy infil-
tiate with plasma cells and deimal edema.
Subsequently, infiltiate bioadens to a dense
middeimal bandlike infiltiate. Ultimately, epi-
deimal and deimal atiophy, dilated deimal
blood vessels, plasma cell infiltiate, elastin and
collagen defects.
Sero|oy CDC iecommends a two-step ap-
pioach in which samples aie fiist tested by
enzyme-linked immunosoibent assay (ELISA)
and equivocal oi positive iesults aie then tested
by Westein blotting. Duiing the fiist month of
infection, both IgM and IgG iesponses to the
spiiochete should be deteimined, piefeiably
in both acute- and convalescent-phase seium
samples. Appioximately 20-30% of patients
have a positive iesponse detectable in acute-
phase samples; about 70-80% have positive ie-
sponse duiing convalescence (2-4 weeks latei).
Aftei that time, the gieat majoiity of patients
continue to have a positive IgG antibody ie-
sponse, and a single test (that foi IgG) is usually
sufficient. Accoiding to cuiient ciiteiia adopted
by the CDC, an IgM Westein blot is consideied
positive if two of the following thiee bands aie
piesent: 23, 39, and 41 kDa.
Positive seiology indicates past infection. It
does not distinguish between an aboitive infec-
tion, a successfully tieated past infection, oi an
active infection.
Cu|ture B. |urgJor[er can be isolated fiom
lesional skin biopsy specimen on modified
Baiboui-Stoennei-Kelly medium. Peimits de-
finitive diagnosis.
FCk Detects B. |urgJor[er DNA in lesional
skin biopsy specimen, blood, oi joint fluid. Not
ioutinely available.
0IACN0SIS
CDC suiveillance ciiteiia:
Ear|y LB: Made on chaiacteiistic clinical find-
ings in a peison living in oi having visited
an endemic aiea; does not iequiie laboiatoiy
confiimation.
Lae LB : Confiimed by specific seiologic
tests.
C : Made on clinical findings confiimed by
lesional biopsy.
C0kS AN0 Fk0CN0SIS
Untieated EM and secondaiy lesions fade in
median time of 28 days, but the iange is fiom
1 day to 14 months. Both EM and secondaiy
lesions can fade and iecui duiing this time.
Howevei, aftei adequate tieatment, eaily le-
sions iesolve within two weeks, and late mani-
festations aie pievented. Late manifestations
identified eaily usually cleai aftei adequate
antibiotic theiapy; howevei, delay in diagnosis
may iesult in peimanent joint oi neuiologic
disabilities.
FICk 24-T8 Iyme borre|oss: secoodary erythema mraos oo truok A 13,e+|o|d r+|e W+ eu|
|u |, || W||e W|eu |e uo|ed + ||uu| |+|. I|e p+||eu| W+ |||ud, W+ uo| +W+|e o| ||c| |||e, +ud |e|| We||. 0u
e\+r|u+||ou, cou||ueu| |+|e |ed W+|r p|+que (+ d+,` du|+||ou) +|e eeu ou ||e |+c| +ud |u||oc|, |e|ou
We|e +|o p|eeu| ou ||e +u|e||o| ||uu| +ud |o|u. 3-|c d|er|u+|e |er+|oeuou|, ||or ||e p||r+|, E\
(|uocu|+||ou) ||e, |eu|||u |u ecoud+|, E\ |e|ou |u ore pe|ou. I|ee |e|ou +|e +u+|oou |o |e|ou o|
ecoud+|, ,p||||. |,re e|o|o, W+ po|||.e. |e|ou |eo|.ed e.e|+| d+, +||e| |e|uu|u do\,c,c||ue.
ACA shows little iesponse to adequate
antibiotic theiapy once atiophy has supei-
vened. Adequately tieated patients have de-
clining titeis of anti- B. |urgJor[er antibody
within 6-12 months.
EM (shoit duiation of infection) tieated
with antimiciobial agents does not confei
piotective immunity. If LB goes untieated
foi months, immunity may develop that
piotects against ieinfection foi yeais.
MANACMNI
Frophy|axs
Avoid known habitats of I. stau|ars and
I. at[tus (United States). Othei pieven-
tive measuies include weaiing long pants
and long-sleeved shiits, tucking pants into
socks.
Apply tick iepellents containing N, N -di-
ethyl- m -toluamide (DEET") to clothing
and/oi exposed skin.
Check iegulaily foi ticks and piomptly
iemove any attached ticks.
Acaiicides containing peimethiin kill
ticks on contact and can piovide fuithei
piotection when applied to clothing.
Aftei a iecognized tick bite, the iisk of
infection with B. |urgJor[er is low, and
antibiotic piophylaxis is not ioutinely in-
dicated. Theiapy with amoxicillin oi doxy-
cycline foi 10 days may be given to pievent
Lyme disease in the following ciicum-
stances: Tick engoiged (feeding foi > 24 h),
IxoJes nymph fiom a hypeiendemic aiea,
piegnancy, immunocompiomised status,
follow-up difficult, patient anxious.
Tempeiatuie of > 38C may iepiesent hu-
man anaplasmosis oi babesiosis tiansmit-
ted with tick bite.
Immuotatoo The vaccine foi LB is no
longei manufactuied.
Aotmcroba| Ireatmeot See Fig. 24-80.
Centeis foi Disease Contiol and Pievention:
Lyme Disease Home Page
http://www.cdc.gov/ncidod/dvbid/lyme/in-
dex.htm
Lyme Disease Netwoik
http://www.lymenet.oig/
FICk 24-T9 Iyme borre|oss: acrodermatts
chrooca atrophcaos: eodstae Ad.+uced +||op|, o|
||e ep|de|r| +ud de|r| W||| +oc|+|ed .|o|+ceou e|,
||er+ o| |e +ud |ee|, ||e .|||||||, o| ||e upe|||c|+| .e|u
| |||||u.
FICk 24-80 A|orthm Ior the treatmeot oI the varous acute or chrooc maoIestatoos oI Iyme
borre|oss ke|+pe r+, occu| W||| +u, o| ||ee |e|reu, +ud + ecoud cou|e o| ||e+|reu| r+, |e uece
+|,. A\, +|||o.eu|||cu|+|. AC '|ee|e. C|+p. 51 |u |c |:|- | ||-c| !-!:- oed, | K+pe| e| +|
(ed). |eW \o||, \cC|+Wn|||, 2005.
Localized skin infection: 14 days
Early disseminated infection:
21 days
Acrodermatitis: 30 days
Arthritis: 3060 days*
Neurologic involvement:
14-28 days
Cardiac involvement:
28 days complete course
with oral therapy when
patient is no longer in
high-degree AV block
Skin
Erythema
migrans
Acrodermatitis
Joint
Arthritis
Heart
AV block
Nervous system
Facial
palsy
alone
Meningitis
Radiculoneuritis
Encephalopathy
Polyneuropathy
OraI therapy
First choice
Age 9 years, not pregnant:
doxycycline, 100 mg bid
Age < 9 years: amoxicillin,
50 mg/kg per day
Second choice for adults:
amoxicillin, 500 mg tid
Third choice for all ages:
cefuroxime axetil, 500 mg bid
Fourth choice for all ages:
erythromycin, 250 mg qid
Intravenous therapy
First choice:
ceftriaxone, 2 g qd
Second choice:
cefotaxime, 2 g q8h
Third choice:
Na penicillin G, 5 million
U q6h
GuideIines for duration of therapy
TREATMENT OF LYMEBORRELIOSIS
692
S E C I | 0 N 2 5
FNCAI INFCII0NS 0F
Ih SkIN AN0 hAIk
'upe|||c|+| |uu+| |u|ec||ou +|e c+ued |, uure|
ou |uu| ||+| +|e c+p+||e o| upe|||c|+||, |u.+d|u
||e |o||oW|u.
'||u
Ep|de|r|
n+||/|+|| |o|||c|e
|+|| +pp+|+|u
\uco+| ||e
0|op|+|,u\
Auoeu||+||+
I|ee |uu| +|e correuu|+| o|+u|r ||+|
||equeu||, co|ou|/e uo|r+| ep|||e||ur.
|e|r+|op|,|e
Cc!!c pec|e
!c|c-cc pec|e
|u|ec||ou c+u e\|eud ro|e deep|, |u ||e |r
ruuocorp|or|ed |o|.
|eepe|, c||ou|c cu|+ueou |uu+| |u|ec||ou c+u
occu| +||e| cu|+ueou |uocu|+||ou.
\,ce|or+
C||oror,co|
'po|o|||c|o|
',|er|c |uu+| |u|ec||ou W||| cu|+ueou d|
er|u+||ou, ||ee |u|ec||ou occu| ro| o||eu |u
||e |rruuocorp|or|ed |o|.
|||r+|, |uu |u|ec||ou, c+u d|er|u+|e |er+
|oeuou|, |o ru|||p|e o|+u ,|er, |uc|ud|u
||e ||u.
C|,p|ococco|
n||op|+ro|
|o||| Are||c+u ||+|or,co|
Cocc|d|o|dor,co|
|eu|c||||uo|
|||r+|, +||o|u|e||u+| (C|) |u|ec||ou, ueu||o
peu|c |o|
||er|u+|ed c+ud|d|+| corrou|, +||e |u
||e C| ||+c|.
'upe|||c|+| |uu+| |u|ec||ou +|e ||e ro| corrou
o| +|| rucocu|+ueou |u|ec||ou, o||eu c+ued |,
o.e||oW|| o| ||+u|eu| o| |e|deu| ||o|+ +oc|
+|ed W||| + c|+ue |u ||e r|c|oeu.||oureu| o|
||e ||u.
|uu| c+u|u ||ee |u|ec||ou.
|e|r+|op|,|e. |u|ec| |e|+||u|/ed ep|||e||ur,
|+|| |o|||c|e, +ud u+|| +pp+|+|u
Cc!!c pp.. kequ||e + W+|r |ur|d eu.||
oureu|.
!c|c-cc pp.. kequ||e + |ur|d r|c|oeu.|
|oureu| +ud ||p|d |o| |oW||.
:| pp
||c-c ( ||c|c o| ||c-c-||,:- )
+--:| . I|ue+ u||+
SFkFICIAI FNCAI INFCII0NS |C|9 .
|C|0 . B!o
SCII0N 25 |u|CA| |||ECI|0|' 0| InE 'K|| A|| nA|k 693
Ae oI 0oset
Childien have scalp infections ( Trt|o|yon,
Mtrosorum ).
Young and oldei adults have inteitiiginous
infections.
The incidence of onychomycosis is coiielated
diiectly with age; in the United States, up to
50% of individuals age 75 yeais have ony-
chomycosis.
kace
Adult blacks may have a lowei incidence of
deimatophytosis.
Tinea capitis is moie common in black childien.
|e|r+|op|,|e +|e + uu|que |oup o| |uu| c+p+
||e o| |u|ec||u uou.|+||e |e|+||u|/ed cu|+ueou
||uc|u|e |uc|ud|u ||+|ur co|ueur, u+||, +ud
|+||.
| -c||,| deuo|e +u |u|ec||ou c+ued |,
de|r+|op|,|e.
C||u|c+| |u|ec||ou |, ||uc|u|e |u.o|.ed.
|!-,: (ep|de|r+| de|r+|op|,|o|)
:|,: (de|r+|op|,|o| o| |+|| +ud |+||
|o|||c|e)
0,:|,: (de|r+|op|,|o| o| ||e u+||
+pp+|+|u)
I|e p+||oeue| o| ep|de|ror,co| . |||
c|or,co| |e+d|u |o d|||e|eu| c||u|c+| r+u||e|+
||ou | c|er+||c+||, dep|c|ed |u |r+e 25.
I|e |e|r |-c | |e| ued |o| de|r+|op|,|oe
+ud | rod|||ed +cco|d|u |o ||e +u+|or|c ||e o|
|u|ec||ou, e.., ||ue+ ped|.
'I|ue+ .e||co|o| | c+||ed |,c .-:|
ou||de o| ||e uu||ed '|+|e, || | uo| + de|r+|o
p|,|o| |u| |+||e| +u |u|ec||ou c+ued |, ||e
,e+| !c|c-cc .
0kMAI0FhI0SS |C|9 . 0
|C|0 . B!5.0B!o
to|oy
Thiee geneia of deimatophytes:
Trt|o|yon
Mtrosorum
EJermo|yon .
Moie than 40 species aie cuiiently iecog-
nized; appioximately 10 spp. aie common
causes of human infection.
T. ru|rum is the most common cause of epi-
deimal deimatophytosis and onychomycosis
in industiialized nations.
Cuiiently, 70% of the U.S. population ex-
peiience at least one episode of T. ru|rum
infection (usually tinea pedis).
T. ru|rum is indigenous to Southeast Asia,
the Austialian outback, and westein Afiica.
A
B
IMAC 25-1 Ihe pathoeoess oI epdermomycoss
(} aod trchomycoss (8} +|e d|||e|eu| |ec+ue ||e,
|u.o|.e d|||e|eu| ||uc|u|e |e+d|u |o d|||e|eu| c||u|c+|
r+u||e|+||ou. |u ep|de|ror,co|, de|r+|op|,|e (|ed
do| +ud ||ue) W||||u ||e ||+|ur co|ueur uo| ou|, d||up|
||e |o|u, |+,e| +ud ||u |e+d |o c+||u, |u| +|o e||c|| +u
|u||+rr+|o|, |epoue (||+c| do| ,r|o||/e |u||+rr+|o|,
ce||), W||c| r+, ||eu r+u||e| + e|,||er+, p+pu|+||ou,
+ud e.eu .e|cu|+||ou. 0u ||e o||e| |+ud, |u |||c|or,co|
||e |+|| |+|| | |u.o|.ed (|ed do|) |eu|||u |u ||e de||uc
||ou +ud ||e+||u o|| o| ||e |+||. || ||e de|r+|op|,|e |u|ec
||ou e\|eud |+|||e| doWu |u|o ||e |+|| |o|||c|e, || W||| e||c|| + deepe| |u||+rr+|o|, |epoue (||+c| do|) +ud |||
W||| r+u||e| + deepe| |u||+rr+|o|, uodu|e, |o|||cu|+| pu|u|+||ou, +ud +|ce |o|r+||ou. ('ee +|o ||. 259.)
Visitois/colonizeis fiom Euiope and Noith
Ameiica became infected in these aieas,
developing tinea pedis and onychomycosis;
these conditions did not occui in natives,
who weie baiefoot oi woie open mocca-
sins. These visitois/colonizeis and soldieis
(Woild Wais I and II, and the Vietnam con-
flict) biought T. ru|rum to Noith Ameiica
and Euiope.
Soldieis weaiing occlusive boots in tiopical
climates developed jungle iot"-extensive
tinea pedis with secondaiy bacteiial infec-
tion.
Piesently, T. ru|rum infection can be ac-
quiied by contact with contaminated floois
(homes, health clubs, athletic lockei iooms,
oi hotel iooms).
The etiology of tinea capitis in childien vaiies
geogiaphically.
Noith Ameiica and Euiope: T. onsurans is
the most common cause, having ieplaced
M. auJoun .
Euiope, Asia, and Afiica: T. o|ateum .
In U.S. adults, T. ru|rum is the most common
cause of deimatophytic folliculitis.
0emoraphy Some species have a woildwide
distiibution; otheis aie iestiicted to paiticulai
continents oi iegions. Howevei, T. tontenr-
tum , the cause of tinea imbiicata, is endemic to
the South Pacific and paits of South Ameiica.
T. ru|rum was endemic to Southeast Asia,
westein Afiica, and Austialia but now oc-
cuis most commonly in Noith Ameiica and
Euiope.
Iraosmssoo
Deimatophyte infections can be acquiied
fiom thiee souices:
Most commonly fiom anothei peison usu-
ally by fomites, less so by diiect skin-to-skin
contact (tinea gladiatoium)]
Fiom animals such as puppies oi kittens
Least commonly fiom soil.
Based on theii ecology, deimatophytes aie
also classified as follows:
n|roo||t : Peison-to-peison tians-
mission by fomites and by diiect contact.
Trt|o|yon spp.: T. ru|rum, T. menagro-
|yes (vai. nerJga|e ), T. st|oen|en,
T. onsurans, T. o|ateum. Mtrosorum
auJoun. EJermo|yon [|ottosum.
Zoo||t : Animal-to-human by diiect con-
tact oi by fomites. Trt|o|yon spp.: T.
equnum, T. menagro|yes (vai. menagro-
|yes ), T. errutosum. M. tans.
Ceo||t : Enviionmental. Mtrosorum

spp.: M. gyseum, M. nanum .
Fredsposo Factors
Atopic diathesis: Cell-mediated immune defi-
ciency foi T. ru|rum .
Topical immunosuppiession: with piolonged
application of topical glucocoiticoids, theie
can be maiked modification in the usual
banal chaiactei of deimatophytosis (tinea
incognito); this is especially tiue of the face,
gioin, and hands.
Systemic immunocompiomise:
Patients have a highei incidence and moie
intiactable deimatophytoses.
Abscesses and gianulomas may occui (Ma-
jocchi gianuloma).
CIASSIFICAII0N
In vivo, deimatophytes giow only on oi within
keiatinized stiuctuies and, as such, involve the
following:
Dermao|yoses o[ |eran:eJ eJerms (e-
Jerma| Jermao|yoss, eJermomytoss) :
Tinea facialis, tinea coipoiis, tinea ciuiis,
tinea manus, tinea pedis.
Dermao|yoses o[ na| aaraus (onyt|omy-
t|oss) : Tinea unguium (toenails, fingeinails).
Onychomycosis (moie inclusive teim, includ-
ing nail infections caused by deimatophytes,
yeasts, and molds).
Dermao|yoses o[ |ar anJ |ar [o||t|e
(rt|omytoss) : Deimatophytic folliculitis,
Majocchi (tiichophytic) gianuloma, tinea
capitis, tinea baibae.
FAIh0CNSIS
Deimatophytes synthesize keiatinases that di-
gest keiatin and sustain existence of fungi in
keiatinized stiuctuies. Cell-mediated immunity
and antimiciobial activity of polymoiphonu-
cleai leukocytes iestiict deimatophyte patho-
genicity.
Hos [ators |a [at|ae Jermao|ye n-
[etons : atopy, topical and systemic glu-
cocoiticoids, ichthyosis, collagen vasculai
disease
Lota| [ators [aorng Jermao|ye n[eton:
sweating, occlusion, occupational exposuie,
geogiaphic location, high humidity (tiopical
oi semitiopical climates)

The clinical piesentation of deimatophytoses
depends on seveial factois: site of infection,
immunologic iesponse of the host, species of
fungus. Deimatophytes (e.g., T. ru|rum ) that
initiate little inflammatoiy iesponse aie bettei
able to establish chionic infection. Oiganisms
such as M. tans cause an acute infection associ-
ated with a biisk inflammatoiy iesponse and
spontaneous iesolution. In some individuals,
infection can involve the deimis, as in keiion
and Majocchi gianuloma.
0rect Mcroscopy (||. 25)
Sump|Ing
S|n : Collect scale with a no. 15 scalpel blade,
edge of a glass micioscope slide, biush (tooth
oi ceivical biush). Scales aie placed on centei
of micioscope slide, swept into a small pile,
and coveied with a coveislip. Recent appli-
cation of cieam/ointment oi powdei often
makes identification of fungal element dif-
ficult/impossible.
Na|s : Keiatinaceous debiis is collected with
a no. 15 scalpel blade oi small cuiette. Distal
lateial subungual onychomycosis (DLSO):
debiide fiom the undeisuiface of nail of most
pioximally involved site; avoid nail plate.
Supeificial white onychomycosis (SWO): su-
peificial nail plate. Pioximal subungual ony-
chomycosis (PSO): undeisuiface of pioximal
nail plate; obtain sample by using a small
punch biopsy tool, boiing thiough involved
nail plate to undeisuiface; obtain keiatin
fiom undeisuiface.
Har : Remove haiis by epilation of bioken
haiis with a needle holdei oi foiceps. Place
on micioscope slide and covei with glass cov-
eislip. Skin scales fiom involved haiiy site can
be obtained with a biush (tooth oi ceivical).
PrepurutIvn v] Sump|e Poassum |yJroxJe 5
o 20% so|uon is applied at the edge of covei-
slip. Capillaiy action diaws solution undei cov-
eislip. The piepaiation is gently heated with a
match oi lightei until bubbles begin to expand,
claiifying the piepaiation. Excess KOH solu-
tion is blotted out with bibulous oi lens papei.
Condensei should be iacked down." Epideimal
deimatophytosis: positive unless patient is us-
ing antifungal theiapy. DLSO: 90% of cases
positive. Vaiiations of KOH with fungal stains:
Swaitz-Lampkins stain, chloiazol black E stain.
MIcrvscvpy Deimatophytes aie iecognized as
septated, tubelike stiuctuies (hyphae oi myc-
elia) (Fig. 25-1).
Wood Iamp Haiis infected with Mtrosorum
spp. fluoiesce. Gieenish. Daiken ioom and il-
luminate affected site with Wood lamp. Coial
ied fluoiescence of inteitiiginous site confiims
diagnosis of eiythiasma.
Fuoa| Cu|tures Specimens collected fiom
scaling skin lesions, haii, nails. Scale and haii
fiom the scalp aie best haivested with tooth
oi ceivical biush; the involved scalp is biushed
vigoiously; keiatinaceous debiis and haiis then
placed into fungal cultuie plate. Cultuie on
Sabouiaud`s glucose medium. Repeat cultuies
iecommended monthly.
0ermatopatho|oy DLSO: peiiodic acid-Schiff
(PAS) oi methenamine silvei stains aie moie
sensitive than KOH piepaiation oi fungal cultuie
in identification of fungal elements in DLSO.
FICk 25-1 Fotassum hydroxde (k0h) prepa-
ratoo \u|||p|e, ep|+|ed, |u|e|||e ||uc|u|e (|,p|+e
o| r,ce||+) +ud po|e |o|r+||ou |u c+|e ||or +u |ud|
.|du+| W||| ep|de|r+| de|r+|op|,|o|. |u cou||+|, K0n
p|ep+|+||ou |u c+ud|d|+| |oW e|ou+|ed ,e+| |o|r
(peudo|,p|+e) W|||ou| ||ue ep|+||ou. (Corp+|e W|||
||. 2522.)
MANACMNI
Freveotoo App|, poWde| cou|+|u|u |r|d+/o|e o| |o|u+||+|e |o +|e+ p|oue |o |uu+| |u|ec||ou
+||e| |+|||u.
Iopca| aotIuoa| |-- -cc| c, |- -||-:|.- | |-c|-| | !-c||,|- | | ||
preparatoos | | ||- | |c c|
||ep+|+||ou | +pp||ed ||d |o |u.o|.ed +|e+ op||r+||, |o| + Wee| |uc|ud|u +| |e+|
Wee| +||e| |e|ou |+.e c|e+|ed.
App|, +| |e+| ! cr |e,oud +d.+uc|u r+||u o| |e|ou.
I|ee |op|c+| +eu| +|e corp+|+||e. ||||e|eu||+|ed |, co|, |+e, .e||c|e, +ud
+u|||uu+| +c||.||,.
|r|d+/o|e C|o|||r+/o|e (|o|||r|u, \,ce|e\)
\|cou+/o|e (\|c+||u)
Ke|ocou+/o|e (||/o|+|)
Ecou+/o|e ('pec|+/o|e)
0\|cou|/o|e (0\||+|)
'u|cou|/o|e (E\e|de|r)
'e||+cou+/o|e (E||+c/o)
A||,|+r|ue |+|||||ue (|+|||u)
Ie|||u+||ue (|+r|||)
|+p||||ou+|e Io|u+||+|e (I|u+c||u)
'u||||u|ed p,||doue C|c|op||o\ o|+r|ue (|op|o\)
Systemc aotIuoa| aeots | |-:| | |-c|c-! | . ue || |e|ou +|e e\|eu|.e o| || |u|ec||ou |+
|+||ed |o |epoud |o |op|c+| p|ep+|+||ou.
|c||, --! | |-c|-| | |-c :c| c! |-c A|o r+, |e
|equ||ed |o| |u||+rr+|o|, ||ue+ +ud |,pe||e|+|o||c rocc+|u|,pe ||ue+ ped|.
Ie|||u+||ue 250r |+||e|. A||,|+r|ue. \o| e||ec||.e o|+| +u||de|rop|,|e +u|||uu+|, |oW
e|||c+c, ++|u| o||e| |uu|.
A/o|e/|r|d+/o|e |||+cou+/o|e +ud |e|ocou+/o|e
|||+cou+/o|e 00r c+pu|e, o|+| o|u||ou (0 r/r|).
|u||+.euou. I||+/o|e. |eed +c|d +|||c
pn |o| d|o|u||ou o| c+pu|e. k+|e |e.e| o| d|o\|u +ud c,c|opo||ue.
App|o.ed |o| ou,c|or,co| |u ||e uu||ed '|+|e.
||ucou+/o|e 00, 50, 200r |+||e|, o|+| upeu|ou (0 o| +0 r/r|), +00 r |\.
Ke|ocou+/o|e 200r |+||e|. |eed +c|d +|||c pn |o| d|o|u||ou o| |+||e|. I+|e W||| |ood o|
co|+ |e.e|+e, +u|+c|d +ud n2 ||oc|e| |educe +|o|p||ou. I|e ro|
|ep+|o|o\|c o| +/o|e d|u, |ep+|o|o\|c||, occu| |u +u e||r+|ed oue o| e.e|,
0,000-5,000 e\poed pe|ou. |o| +pp|o.ed |o| ||e+|reu| o| de|r+|op|,|e
|u|ec||ou |u ||e uu||ed '|+|e.
C||eo|u|.|u !:c-!. 250 o| 500r |+||e|, 25 r/|e+poou upeu|ou.
|||c:c-! . o5 o| !!0r |+||e|. Ac||.e ou|, ++|u| de|r+|op|,|e,
|e e||ec||.e ||+u |||+/o|e. Ad.e|e e||ec| |uc|ude |e+d+c|e, u+ue+/.or|||u,
p|o|oeu|||.||,, |oWe| e||ec| o| c|,|+|||ue W+||+||u od|ur. | +ud
|c |u|ec||ou r+, |epoud poo||,. '|ou|d |e |+|eu W||| |+||, re+| |o
r+\|r|/e +|o|p||ou. |u c|||d|eu, CBC +ud ||I |ecorreuded || ||| |+c|o| |o|
|ep+|||| e\|| o| ||e+|reu| |+| |oue| ||+u ! rou||.
. CBC, corp|e|e ||ood couu|, ||I, ||.e| |uuc||ou |e|.
SCII0N 25 |u|CA| |||ECI|0|' 0| InE 'K|| A|| nA|k 69T
FI0MI0I0C
Ae oI 0oset Late childhood oi young adult
life. Most common, 20-50 yeais.
Fredsposo Factors Hot, humid weathei; oc-
clusive footweai; excessive sweating.
Iraosmssoo Walking baiefoot on contami-
nated floois. Aithiospoies can suivive in hu-
man scales foi 12 months.
0uratoo Months to yeais oi lifetime. Often,
piioi histoiy of tinea pedis, tinea unguium of
toenails. May flaie if in hot climate.
Sko Symptoms Usually asymptomatic. Piuii-
tus. Pain with bacteiial supeiinfection.
Sko Iesoos
1nterdIgItu| Type
Two patteins:
Diy scaling (Fig. 25-2)
Maceiation, peeling, fissuiing of toe webs
(Fig. 25-3). Hypeihidiosis common.
Most common site: between fouith and fifth
toes.
Ep|de|r+| de|r+|op|,|oe +|e ||e ro| cor

rou de|r+|op|,||c |u|ec||ou.
\+, |e |o||oWed/+ccorp+u|ed |, de|r+|op|,||c
|u|ec||ou o| |+||/|+|| |o|||c|e +ud/o| ||e u+||
+pp+|+|u.
',, . k|uWo|r, ep|de|ror,co|.
0kMAI0FhI0SS 0F FI0kMIS
|e|r+|op|,||c |u|ec||ou o| ||e |ee|
C||u|c+| ||ud|u. e|,||er+, c+||u, r+ce|+||ou,
+ud/o| |u||+ |o|r+||ou
|u ro| c+e o| ep|de|r+| de|r+|op|,|o|, ||e
|u|ec||ou occu| |u|||+||, ou ||e |ee| ( | ),
+ud, |u ||re, p|e+d |o ||e uc| + ||e
|uu|u+| +|e+ (||ue+ c|u||)
I|uu|, +|r, |e (||ue+ co|po||)
n+ud (||ue+ r+uuur)
I|ue+ ped| o||eu p|o.|de ||e+| |u ||e |u|e|||,
o| ||e ep|de|r| |||ou| W||c| |+c|e||+ uc| +
'|c|,|::: c- o| |oup A ||ep|ococcu
(CA') c+u |u.+de, c+u|u ||u o| o||||ue |u|ec
||ou (ce||u|||| o| |,rp|+u|||).
',,. A|||e|e' |oo|. luu|e |o|
IINA F0IS |C|9 . 0.+
|C|0 . B!5.!
Infection may spiead to adjacent aieas of
feet.
MvccusIn Type
Well-demaicated eiythema with minute pa-
pules on maigin, fine white scaling, and
hypeikeiatosis (Fig. 25-4) (confined to heels,
soles, lateial boideis of feet).
Dsr|uon : Sole, involving aiea coveied by a
ballet slippei.
One oi both feet may be involved with any
pattein; bilateial involvement moie common.
1n]|ummutvry/Bu||vus Type
Vesicles oi bullae filled with cleai fluid (Fig.
25-5).
Pus usually indicates supeiinfection with S.
aureus infection oi GAS.
Aftei iuptuiing, eiosions with iagged iinglike
boidei.
May be associated with id" ieaction (auto-
sensitization oi deimatophytid).
Dsr|uon . Sole, instep, webspaces.
U|cerutIve Type
Extension of inteidigital tinea pedis onto doi-
sal and plantai foot.
FICk 25-2 Ioea peds: oterdta| dry type I|e |u|e|d|||+| p+ce |e|Weeu ||e |oe |oW e|,||er+
+ud c+||u, ||e |oeu+|| | |||c|eued, |ud|c+||.e o| +oc|+|ed d||+| u|uuu+| ou,c|or,co|.
FICk 25-3 Ioea peds: oterdta| macerated type A +3,e+|o|d r+|e W||| +|||e|e' |oo| +ud
|,pe|||d|o| |o| ,e+|. I|e ||u o| ||e We|p+ce |e|Weeu ||e +|| +ud 5|| |oe | |,pe||e|+|o||c +ud r+ce|+|ed
(|,d|+||ou o| ||e ||+|ur co|ueur). I|e K0n p|ep+|+||ou |oW ep|+|ed |,p|+e, cou|||r|u ||e d|+uo| o|
de|r+|op|,|o|. wood |+rp derou||+|ed co|+||ed ||uo|eceuce cou|||r|u coucor||+u| e|,|||+r+. |-!
c c-c W+ |o|+|ed ou |+c|e||+| cu||u|e.
Often complicated by bacteiial supeiinfec-
tion: S. aureus Methicillin-sensitive S. aureus
(MSSA) oi Methicillin-iesistant S. aureus
(MRSA)], GAS, gioup B stieptococcus (GBS),
PseuJomonas aerugnosa ; eiythiasma; Can-
JJa a||tans.
Ioterdta| Iype Eiythiasma, impetigo, pitted
keiatolysis, CanJJa inteitiigo, P. aerugnosa
webspace infection.
Moccaso Iype Psoiiasis vulgaiis, eczematous
deimatitis (dyshidiotic, atopic, alleigic con-
tact), pitted keiatolysis, vaiious keiatodeimas.
FICk 25-4 Ioea peds: moccaso type A o5,e+|o|d |er+|e W||| c+||u |ee| |o| ,e+|. '|+|p|, r+||u
+|ed e|,||er+ o| ||e |oo| W||| + r||d |e|+|ode|r+ +oc|+|ed W||| d||+|/|+|e|+| u|uuu+| ou,c|or,co|, |,p|c+|
o| | |u|ec||ou.
FICk 25-5 Ioea peds: bu||ous type A 12,e+|o|d |er+|e W||| p|u||||c c+||u |ee| |o| rou||, ||e+||u
W||| c|o|e|+o| c|e+r. E|,||er+, c+||u, +ud ||||e| |o|r+||ou ou ||e do|ur o| ||e |oo|. I|e |u|||+| d|+uo|
W+ ec/er+ ||+| W+ ||e+|ed W||| c|o|e|+o| o|u|reu|, W||c| |+c||||+|ed ||e |oW|| o| de|r+|op|,|e, oc+||ed
||ue+ |ucou||o. Bu||ou ||ue+ ped| | uu+||, c+ued |, -|c|,|-.
FAkI III ||'EA'E' |uE I0 \|Ck0B|A| ACE|I' T00
CIASSIFICAII0N
Type 0||o|ca| Feat0res t|o|ogy
|u|e|d|||+| (+cu|e +ud \o| corrou |,pe, | ro| corrou
c||ou|c) ||equeu||, o.e||oo|ed c+ue o| c||ou|c ||ue+ ped|,
|Wo p+||e|u. d|, +ud -|c|,|- c+ue ro|e
ro|| W||| r+ce|+||ou |u||+rr+|o|, |e|ou
||, 'c+||u o| We|p+ce, |
r+, |e e|o|.e
\o|| (r+ce|+|ed) n,pe||e|+|o| o| We|p+ce -|c|,|-
W||| r+ce|+||ou o| ||+|ur
co|ueur
\occ+|u (c||ou|c Ke|+|ode|r+ \o| o||eu c+ued |, | ,
|,pe||e|+|o||c o| d|,) epec|+||, |u +|op|c |ud|.|du+|, +|o
|!-|,| ||::
|u||+rr+|o|, o| |u||ou B|||e| |u uouocc|uded ||u |e+| corrou |,pe, uu+||, c+ued
(.e|cu|+|) |, -|c|,|- .+|.
-|c|,|- (|+uu|+|). keer||e +u
+||e||c cou|+c| de|r+||||
u|ce|+||.e Au e\|eu|ou o| |u|e|d|||+| |,pe . | | ||::
|u|o de|r| due |o r+ce|+||ou -|c|,|-
+ud ecoud+|, (|+c|e||+|) C c||:c
|u|ec||ou
|e|r+|op|,||d ||eeu| + + .e|cu|+| e|up||ou -|c|,|-
o| ||e ||ue| +ud/o| p+|r+| |
+pec| o| ||e |+ud ecoud+|,
|o |u||+rr+|o|, ||ue+ ped|.
A cor||ued c||u|c+| p|eeu|+||ou
+|o occu|. Cc!!c +ud
|+c|e||+ (' c- CA' ,
| c-c) r+, c+ue
upe||u|ec||ou.
IoI|ammatory[8u||ous Iype Bullous impetigo,
alleigic contact deimatitis, dyshidiotic eczema,
bullous disease.
0rect Mcroscopy (Fig. 25-1). In bullous type,
examine sciaping fiom the innei aspect of bulla
ioof foi detection of hyphae.
Wvvd Lump Negative fluoiescence usually
iules out eiythiasma in inteidigital infection.
Eiythiasma and inteidigital tinea pedis may
coexist.
Cu|ture
Funga| . Deimatophytes can be isolated in
11% of noimal-appeaiing inteispaces and
31% of maceiated toe webs. CanJJa spp.
may be copathogens in webspaces.
Batera| . In individuals with maceiated in-
teidigital space, S. aureus, P. aerugnosa , and
diphtheioids aie commonly isolated. S. au-
reus and GAS can cause supeiinfection.
0IACN0SIS
Demonstiation of hyphae on diiect micioscopy,
isolation of deimatophyte on cultuie.
C0kS AN0 Fk0CN0SIS
Tends to be chionic, with exaceibations in hot
weathei.
May piovide poital of entiy foi lymphangitis oi
cellulitis, especially in patients whose leg veins
have been used foi coionaiy aiteiy bypass sui-
geiy and have chionic low-giade edema of leg.
Without secondaiy piophylaxis, iecuiience is
the iule.
SCII0N 25 |u|CA| |||ECI|0|' 0| InE 'K|| A|| nA|k T01
MANACMNI
Freveotoo ue o| |oWe| |oe W|||e |+|||u +| |ore o| |u pu|||c |+c||||,. w+||u |ee| W|||
|eu/o,| pe|o\|de |+| d||ec||, +||e| |oWe|. ||+|e||c +ud ||oe W|o |+.e
uude|oue co|ou+|, +||e|, |,p+ W||| |+|.e||u o| |e .e|u +|e epec|+||,
u|jec| |o ecoud+|, |+c|e||+| |u|ec||ou (|rpe|||u|/+||ou, |,rp|+u|||, ce||u||||).
Speca| coosderatoos by type oI oIectoo
\+ce|+|ed |u|e|d|||+| Acu|e. Bu|oW' We| d|e|u, C+|e||+u| p+|u|. C||ou|c. +|ur|uur c||o||de
|e\+|,d|+|e 20 ||d |o |educe We+||u.
\occ+|u \o| d||||cu|| |o e|+d|c+|e. \+u, p+||eu| |+.e + r|uo| de|ec| |u ce||red|+|ed
|rruue |epoue. ||+|ur co|ueur |||c|, r+||u || d||||cu|| |o| |op|c+| +u|||uu+|
+eu| |o peue||+|e, o||eu +oc|+|ed W||| ||ue+ uuu|ur, + ou|ce o| |e|u|ec||ou o|
||u. Ke|+|o|,||c +eu| (+||c,||c +c|d, |+c||c +c|d, |,d|o\, +c|d) W||| p|+||c occ|u|ou
ue|u| |u |educ|u |,pe||e|+|o|. |+|| |ee|.o|| ru| |e e|+d|c+|ed |o cu|e
rocc+|u|,pe |u|ec||ou.
|u||+rr+|o|,/|u||ou Acu|e|,, ue coo| corp|ee. || e.e|e, ,|er|c |ucoco|||co|d +|e |ud|c+|ed.
AotIuoa| aeots
Iop|c+| 'ee '\+u+ereu|, p+e o9o. App|, |o +|| +||ec|ed ||e |W|ce d+||,. I|e+| |o|
2-+ Wee|.
',|er|c |ud|c+|ed |o| e\|eu|.e |u|ec||ou, |o| |+||u|e o| |op|c+| ||e+|reu|, o| |o| ||oe W|||
||ue+ uuu|ur +ud rocc+|u|,pe ||ue+.
Ie|||u+||ue 250 r d+||, |o| + d+,
|||+cou+/o|e 200 r |W|ce d+||, |o| 1 d+, 200 r d+||, |o| + d+,
||ucou+/o|e 50-200 r d+||, |o| +-o Wee|
Secoodary prophy|axs |rpo||+u| |u p|e.eu||u |ecu||euce o| |u|e|d|||+| +ud rocc+|u |,pe o| ||ue+
ped|. |+||, W+||u o| |ee| W|||e |+|||u W||| |eu/o,| pe|o\|de |+| | e||ec||.e
+ud |ue\peu|.e. Au|||uu+| poWde|, +|co|o| e|.
0uratoo Months to yeais.
Sko Symptoms Fiequently symptomatic. Piu-
iitus. Pain if fissuied. Dys|Jrot ye . Episodic
symptoms of piuiitus.
Sko Iesoos
Well-demaicated scaling patches, hypeikeia-
tosis, and scaling confined to palmai cieases,
fissuies on palmai hand (Fig. 25-6). Boideis
well demaicated; cential cleaiing.
May extend onto doisum of hand with fol-
liculai papules, nodules, pustules with dei-
matophytic folliculitis.
C||ou|c de|r+|op|,|o| o| ||e |+ud().
0||eu uu||+|e|+|, ro| corrou|, ou ||e dor|
u+u| |+ud.
uu+||, +oc|+|ed W||| ||ue+ ped|.
IINA MANM |C|9 . 0.2
|C|0 . B!5.2
Dys|Jrot Tye : Papules, vesicles, bullae (un-
common on the maigin of lesion) on palms
and lateial fingeis, similai to lesions of bul-
lous tinea pedis.
SetonJary C|anges : Lichen simplex chioni-
cus, piuiigo nodules, impetiginization.
Dsr|uon : Diffuse hypeikeiatosis of the
palms with pionounced involvement of pal-
mai cieases oi patchy scaling on the doisa
and sides of fingeis; 50% of patients have
unilateial involvement (Fig. 25-7). Usually
associated with tinea pedis (Fig. 25-6), tinea
ciuiis. If chionic, often associated with tinea
unguium of fingeinails.
FICk 25-6 Ioea maouum E|,||er+ +ud c+||u o| ||e |||| |+ud, W||c| W+ +oc|+|ed W||| |||+|e|+|
||ue+ pedur, ||e oue|+ud, |Wo|ee| d|||||u||ou | |,p|c+| o| ep|de|r+| de|r+|op|,|o| o| ||e |+ud +ud |ee|.
|u ||re, d||+|/|+|e|+| u|uuu+| ou,c|or,co| occu| ou ||e ||ue|u+||.
FICk 25-T Ioea maouum, toea
peds, aod ooychomycoss A 5+,e+|o|d
r+|e W||| |ep+|||| C |u|ec||ou p|eeu|ed W|||
+ |+| ou e\+r|u+||ou. A |+|e e|,||er+|ou
c+||u p|+que W||| |+|p r+||u+||ou ou ||e
do|ur o| ||e |e|| |+ud +oc|+|ed W||| ||ue+
ped| +ud d||+| u|uuu+| ou,c|or,co|.
I|ue+ |+c|+|| |u.o|.|u ||e |+ce +ud |||| e+|
We|e +|o p|eeu|.
rythema[Sca|o haods Atopic deimatitis, li-
chen simplex chionicus, alleigic contact deimati-
tis, iiiitant contact deimatitis, psoiiasis vulgaiis.
C0kS
Chionic, does not iesolve spontaneously.
Aftei tieatment, iecuis unless onychomycosis
of fingeinails, feet, and toenails is eiadicated.
Fissuies and eiosions piovide poital of entiy
foi bacteiial infections.
MANACMNI
Freveotoo Must eiadicate tinea unguium of
fingeinails as well as toenails; also tinea pedis
'u|+cu|e o| c||ou|c de|r+|op|,|o| o| ||e |o|u,
pu||c |e|ou, +ud ||||.
'A|W+, +oc|+|ed W||| ||ue+ ped|, ||e ou|ce o|
||e |u|ec||ou.
',, . 'loc| ||c|.
IINA CkkIS |C|9 . 0.!
|C|0 . B!5.o
Ae oI 0oset Adult.
to|oy T. ru|rum, T. menagro|yes .
Fredsposo Factors Waim, humid enviion-
ment: tight clothing woin by men; obesity.
Chionic topical glucocoiticoid application.
0uratoo Months to yeais. Often, histoiy of
long-standing tinea pedis and piioi histoiy of
tinea ciuiis.
Sko Symptoms Often none. In some peisons,
piuiitus causes patient to seek tieatment.
Sko Iesoos
Usually associated with tinea pedis, tinea un-
guium of toenails.
Laige, scaling, well-demaicated dull ied/tan/
biown plaques (Fig. 25-8).
and tinea ciuiis, otheiwise, tinea manuum will
iecui.
AotIuoa| Aeots TvpIcu| See Manage-
ment," page 696. Failuie common.
SystemIc
Because of thickness of palmai stiatum coi-
neum, and especially if associated with tinea
unguium of fingeinails, tinea manuum is
impossible to cuie with topical agents.
Oial agents eiadicate deimatophytoses of
hands, feet, and nails:
Ter|na[ne . 250 mg daily foi 14 days
Iratona:o|e . 200 mg daily foi 7 days
F|utona:o|e . 150 to 200 mg daily foi 2 to
4 weeks
Noe . Eiadication of fingeinail onychomyco-
sis iequiies longei use.
Cential cleaiing.
Papules, pustules may be piesent at maigins:
deimatophytic folliculitis.
Tieated lesions: lack scale; postinflammatoiy
hypeipigmentation in daikei-skinned pei-
sons.
In atopics, chionic sciatching may pio-
duce secondaiy changes of lichen simplex
chionicus.
DIstrIhutIvn Gioins and thighs; may extend
to buttocks. Sciotum and penis aie iaiely in-
volved.
rythema[Sca|o o Croos Eiythiasma, in-
teitiigo, CanJJa inteitiigo, inteitiiginous
psoiiasis, pityiiasis veisicoloi, Langeihans cell
histiocytosis.
MANACMNI
Freveotoo Aftei eiadication of tinea ciuiis,
tinea pedis, and tinea unguium, ieinfection can
be minimized by weaiing showei shoes when
using a public oi home (if family membeis aie
infected) bathing facility; using antifungal pow-
deis; benzoyl peioxide wash; alcohol gels.
AotIuoa| Aeots TvpIcu| See Manage-
ment," page 696.
SystemIc If iecuiient, if deimatophytic fol-
liculitis is piesent, oi if it has failed to iespond
to adequate topical theiapy. See Management,"
page 696.
|e|r+|op|,|e |u|ec||ou o| ||e ||uu|, |e, +|r,
+ud/o| uec|, e\c|ud|u ||e |ee|, |+ud, +ud
|o|u.
IINA C0kF0kIS |C|9 . 0.5
|C|0 . B!5.+
Ae oI 0oset All ages.
0ccupatoo Animal (laige and small) woikeis.
to|oy
T. ru|rum most commonly
M. tans. inflammatoiy
T. onsurans in paients of black childien with
tinea capitis
Iraosmssoo
Autoinoculation fiom othei paits of the body,
i.e., fiom tinea pedis and tinea capitis.
Diiect oi indiiect with anothei peison, e.g., in
wiestleis, tinea gladiatoium.
Contact with animals oi contaminated soil.
Ceoraphy Moie common in tiopical and
subtiopical iegions.
FICk 25-8 Ioea crurs: toea ocooto A o0,e+|o|d |eu+| ||+up|+u| |ec|p|eu| |+ |eeu ||e+||u ||||
|+| W||| |op|c+| co|||co|e|o|d |o| e.e|+| rou||. B|o|c|, e|,||er+ W||| +|e+ o| +||op|, +ud c+|e ou ||e ||||
red|+| uppe| |||| |o|de||u ||e |uu|u+| +|e+. I|ue+ ped| +ud ou,c|or,co| We|e +|o p|eeu|. K0n p|ep+|+
||ou |oWed ep|+|ed |,p|+e. Iop|c+| |e|o|d |+c||||+|e de|r+|op|,|e |oW||, upp|e|u ||e |rruue |epoue,
c|e+||u +u uud|+uoed |u|ec||ou, ||ue+ |ucou||o.
Fredsposo Factors Most commonly, infec-
tion is spiead fiom deimatophytic infection of
the feet ( T. ru|rum, T. menagro|yes ). Infec-
tion can also be acquiied fiom an active lesion
of an animal ( T. errutosum, M. tans ) oi, iaiely,
fiom soil ( M. gyseum ).
Iocubatoo Ferod Days to months.
0uratoo Weeks to months to yeais.
Symptoms Often asymptomatic. Mild piuiitus.
Sko Iesoos
Small to laige (Fig. 25-9), scaling, shaiply
maiginated plaques with oi without pustules
oi vesicles, usually at maigins.
Peiipheial enlaigement and cential cleaiing
(Fig. 25-10) pioduce annulai configuiation
with concentiic iings oi aicuate lesions (Fig.
25-11); fusion of lesions pioduces gyiate pat-
teins.
Single and occasionally scatteied multiple le-
sions.
Psoiiasifoim plaques.
Veiiucous lesions.
Lesions of zoophilic infection (contiacted
fiom animals) aie moie inflammatoiy, with
maiked vesicles (Fig. 25-12), pustules, ciust-
ing at maigins.
Papules, nodules, pustules: deimatophytic
folliculitis, i.e., Majocchi gianuloma.
We||-0emarcated Sca|o F|aque(s) Alleigic
contact deimatitis, atopic deimatitis, annulai
eiythemas, psoiiasis, seboiiheic deimatitis, pit-
yiiasis iosea, pityiiasis alba, pityiiasis veisicoloi,
eiythema migians, subacute lupus eiythemato-
sus, mycosis fungoides (CTCL).
See Diiect Micioscopy," page 695, and cultuie.
MANACMNI
AotIuoa| Aeots See Management," page
696, foi topical and systemic tieatment.
FICk 25-9 Ioea corpors: toea ocooto Au 30,e+|o|d r+|e W||| + |+| ou |u||oc| |o| oue ,e+|.
E|,||er+|ou p+|c|e ou ||e |u||oc|, ore W||| |+|p r+||u+||ou, o||e| W||| c|e+||u, +ud e\co||+||ou. ne
|+d |eeu ||e+||u ||e p|u|||u W||| |op|c+| co|||co|e|o|d. I|ue+ c|u||, ||ue+ ped|, +ud ou,c|or,co| We|e +|o
p|eeu|.
FICk 25-10 Ioea corpors: toea ocoo-
to A 0,e+|o|d r+|e W||| |u||ou perp||o|d
W+ ||e+|ed W||| |op|c+| c|o|e|+o| |, c+|e|.e|.
B|o|c|, e|,||er+|ou c+||u p+|c|e +|e eeu ou
||e |+c|.
FICk 25-11 Ioea corpors A 0
,e+|o|d |er+|e W||| p|u||||c |+| ou |+c| |o|
5 Wee|. we||der+|c+|ed c+||u p|+que
+|e eeu ou ||e uppe| |+c|. \||d c+|p c+|
|u W+ p|eeu|. |c W+ de|ec|ed
ou |uu+| cu||u|e.
FICk 25-12 Ioea corpors:
oI|ammatory A !,e+|o|d |er+|e
W||| |u||+rr+|o|, |e|ou ou ||e +|r
|o| oue Wee|. A ,ouue| ||||u |+d
||ue+ c+p|||. Acu|e|, |u||+red eder+
|ou e\ud+||.e +uuu|+| p|+que ou ||e
uppe| +|r.
SCII0N 25 |u|CA| |||ECI|0|' 0| InE 'K|| A|| nA|k T0T
Ae oI 0oset Moie common in childien.
to|oy T. onsurans associated with tinea
capitis in black childien and theii paients. T.
menagro|yes, T. ru|rum most commonly;
also M. auJoun, M. tans.
Fredsposo Factors Animal exposuie, chionic
topical application of glucocoiticoids.
Sko Symptoms Most commonly asympto-
matic. At times, piuiitus and photosensitivity.
Sko Iesoos
Well-ciicumsciibed macule to plaque of vaii-
able size; elevated boidei and cential iegies-
sion (Fig. 25-13).
Scaling is often minimal (Fig. 25-14) but can
be pionounced.
Pink to ied.
|e|r+|op|,|o| o| ||e |+||ou |+c|+| ||u
we||c||curc|||ed e|,||er+|ou p+|c|
\o|e corrou|, r|d|+uoed ||+u +u, o||e|
de|r+|op|,|o|.
',, . I|ue+ |+c|e|
IINA FACIAIIS
In black patients, hypeipigmentation.
Any aiea of face but usually not symmetiic.
Sca|o Faca| Fatches Seboiiheic deimatitis,
contact deimatitis, eiythema migians, lupus
eiythematosus, polymoiphous light eiuption,
phototoxic diug eiuption, lymphocytic
infiltiate.
See page 695 and cultuie.
MANACMNI
AotIuoa| Aeots See Management," page
696, foi topical and systemic theiapy.
FICk 25-13 Ioea Iaca|s A !2
,e+|o|d |er+|e W||| p|u||||c |e|ou ou
||e |+ce |o| oue Wee|, |e |+d +cqu||ed
+ ueW ||||eu 2 Wee| p|e.|ou|,. \u|||p|e
r+|| |ed c+||u p+|c|e +|e eeu ou ||e
|oWe| |+ce. K0n p|ep+|+||ou de|ec|ed
ep|+|ed |,p|+e, ! :c ou de|r+|o
p|,|e cu||u|e.
Ep|de|r+| de|r+|op|,|o|, o||eu +oc|+|ed W|||
de|r+|op|,||c |o|||cu||||.
0ccu| +||e| ||e |op|c+| +pp||c+||ou o| + |ucoco|
||co|d p|ep+|+||ou |o + ||e co|ou|/ed o| |u|ec|ed
W||| de|r+|op|,|e.
0ccu| W|eu +u |u||+rr+|o|, de|r+|op|,|o| |
r||+|eu |o| po||+| o| +u ec/er+|ou de|r+||
|| (||. 253 |o 250).
|e|ou +|e uu+||, +,rp|or+||c |u| r+, |e .e|,
p|u||||c o| e.eu p+|u|u|.
|u.o|.ed ||e o||eu |+.e e\+e|+|ed |e+|u|e o|
ep|de|r+| de|r+|op|,|oe, |e|u + deep |ed o|
.|o|+ceou W||| |o|||cu|+| p+pu|e o| pu|u|e.
Ep|de|r+| +||op|, c+ued |, c||ou|c |ucoco|||
co|d +pp||c+||ou r+, |e p|eeu|.
',|er|c +u|||uu+| ||e|+p, r+, |e |ud|c+|ed due
|o deep |u.o|.ereu| o| ||e |+|| +pp+|+|u.
IINA INC0CNII0
|e|r+|op|,|e +|e c+p+||e o| |u.+d|u |+||
|o|||c|e +ud |+|| |+||, c+u|u de|r+|op|,||c
|||c|or,co|
I|ue+ c+p|||
I|ue+ |+||+e
|e|r+|op|,||c |o|||cu||||
\+jocc|| |+uu|or+
IWo |,pe o| |+|| |u.o|.ereu| +|e eeu (|r+e
252).
0kMAI0FhI0SS 0F hAIk (IkICh0MC0SIS)
FICk 25-14 Ioea Iaca|s Au 3!,e+|
o|d |rruuoupp|eed r+|e W||| + |||o|, o|
p|edu|oue ||e+|reu| |o| po|,r,+||+ ||eur+||c+
+ud c||ou|c |,rp|+||c |eu|er|+. |o|e + |+c|+|
|+| +ud + ueW uodu|e. we||der+|c+|ed e|,
||er+ +ud c+||u |u ||e |e+|d +|e+. 'CC |u
||u | p|eeu| ou ||e |e|| e,e||oW. I|e |uro|
ou ||e |e|| uec| | Bce|| |,rp|or+, ||| |e|ou
|e|eed W|eu p|edu|oue W+ |+pe|ed.
Ae oI 0oset Toddleis and school-age chil-
dien. Most common at 6-10 yeais of age; less
common aftei age 16. In adults it occuis most
commonly in a iuial setting.
kace Much moie common in blacks than in
whites.
to|oy[0emoraphy
Vaiies fiom countiy to countiy and fiom
iegion to iegion.
Species change in time due to immigiation.
Infections can become epidemic in schools and
institutions, especially with oveiciowding.
United States: Random fungal cultuies in ui-
ban study detected a 4% infection iate and a
12.7% colonization iate among black childien
|e|r+|op|,||c |||c|or,co| o| ||e c+|p
||edor|u+u||, + d|e+e o| p|e+do|eceu| c|||
d|eu
C||u|c+| p|eeu|+||ou .+|, W|de|,.
|ou|u||+rr+|o|, c+||u
'c+||u +ud ||o|euo|| |+||
'e.e|e, p+|u|u| |u||+rr+||ou W||| p+|u|u|, |o,
uodu|e ||+| d|+|u pu (|e||ou) +ud |eu|| |u
c+|||u +|opec|+
',, . k|uWo|r o| ||e c+|p, ||ue+ |ou
u|+u
IINA CAFIIIS |C|9 . 0.5
|C|0 . B!5.0
pdemo|oy
UnIted Stutes und Western Eurvpe
90% of cases of tinea capitis caused by T. on-
surans .
Less commonly, M. tans .
Foimeily, most cases weie caused by M.
auJoun . Less commonly, M. gyseum,
T. menagro|yes, T. ru|rum.
Eustern und Svuthern Eurvpe, Nvrth A]rIcu
T. o|ateum
Iraosmssoo Peison-to-peison, animal-
to-peison, via fomites. Spoies aie piesent on
asymptomatic caiiieis, animals, oi inanimate
objects.
ksk Factors Foi favus (see below): debilita-
tion, malnutiition, chionic disease
IMAC 25-2 0ermatophytc Io||cu|ts. Ec|o
||||\ |,pe. r,ce||+ +ud +||||ocou|d|+ +|e eeu ou ||e
u||+ce o| ||e |+|| |o|||c|e (e\||+p||+|,). Eudo||||\ |,pe.
|,p|+e +ud +||||ocou|d|+ occu| W||||u ||e |+|| |+||
(|u||+p||+|,). Ectothrix Endothrix
CIASSIFICAII0N
ctothrx oIectoo |u.+|ou occu| ou||de |+|| |+||. n,p|+e ||+reu| |u|o +||||ocou|d|+, |e+d|u |o
cu||c|e de||uc||ou. C+ued |, !: pp. ( ! c! +ud ! :c )
(|r+e 252).
odothrx oIectoo |u|ec||ou occu| W||||u |+|| |+|| W|||ou| cu||c|e de||uc||ou (|r+e 252).
A||||ocou|d|+ |ouud W||||u |+|| |+||. C+ued |, :||,| pp.
( |c |u |o||| Are||c+, .|c:- |u Eu|ope, A|+, p+|| o| A|||c+).
3|c:| !| |-c :c| \+||+u| o| eudo||||\ |eer|||u e|o|||e|c de|r+||||.
|- \+||+u| o| eudo||||\ W||| |o, |u||+rr+|o|, p|+que.
|c. \+||+u| o| eudo||||\ W||| +||||ocou|d|+ +ud +||p+ce W||||u |+|| |+||. \e|,
uucorrou |u We|e|u Eu|ope +ud |o||| Are||c+. |u ore p+|| o| ||e Wo||d
(\|dd|e E+|, 'ou|| A|||c+), |oWe.e|, || | |||| euder|c.
giow inwaid towaid haii bulb. Secondaiy
extiapilaiy hyphae buist, giowing ovei
suiface of haii shaft.
Laige-spoied ectothiix have similai ai-
iangement.
Trt|o|yon types:
Laige-spoied ectothiix (in chains); ai-
thiospoies spheiical, aiianged in stiaight
chains, confined to exteinal suiface of
haii shaft. Spoies aie all laigei than those
of small-spoied Mtrosorum ectothiix.
Endothiix type; intiapilaiy hyphae fiag-
ment into aithioconidia within haii shaft,
making it fiagile, with subsequent bieak-
age close to scalp suiface.
Sko Symptoms
Inflammatoiy tinea capitis:
Pain, tendeiness
Alopecia
Noninflammatoiy infection:
Scaling
Scalp piuiitus
Diffuse oi ciicumsciibed alopecia
Occipital oi posteiioi auiiculai adenopathy
Sko Iesoos aod har Chaoes
Smu||-Spvred EctvthrIr TIneu CupItIs
Giay patch" tinea capitis (Fig. 25-15). Paitial
alopecia, often ciiculai in shape, showing
numeious bioken-off haiis, dull giay fiom
theii coating of aithiospoies. Fine scaling
with faiily shaip maigin. Haii shaft becomes
biittle, bieaking off at oi slightly above scalp.
Small patches coalesce, foiming laigei patches.
FAIh0CNSIS
Scalp haii tiaps fungi fiom the enviionment
oi fomites.
Asymptomatic colonization is common.
Tiauma assists inoculation.
Deimatophytes initially invade stiatum coi-
neum of scalp, which may be followed by haii
shaft infection. Spiead to othei haii follicles
then occuis.
Noninflammatoiy lesions:
Invasion of haii shaft by the deimato-
phytes.
Piincipally M. auJoun (child-to-child,
via baibei, hats, theatei seats), M. tans
(young pets-to-child and then child-to-
child), oi T. onsurans .
Inflammatoiy lesions:
T. onsurans, M. tans, T. errutosum , and
otheis.
Spoies entei thiough bieaks in haii shaft
oi scalp to cause clinical infection.
Eventually, infection iegiesses with oi with-
out an inflammatoiy iesponse.
Clinical appeaiance vaiies with type of haii
invasion, level of host iesistance, degiee of
inflammatoiy host iesponse:
Few dull giay, bioken-off haiis with little
scaling to seveie painful inflammatoiy mass
coveiing entiie scalp.
Paitial haii loss with inflammation in all
cases.
Keiion is associated with a high degiee of
hypeisensitivity to fungal hapten.
Two types of haii invasion:
Mtrosorum types:
Small-spoied ectothiix; haii shaft is in-
vaded in mid-follicle. Intiapilaiy hyphae

Inflammatoiy iesponse minimal, but massive
scaling.
Seveial oi many patches, iandomly aiianged,
may be piesent.
M. auJoun, M. [errugneum, M. tans infec-
tions show gieen fluoiescence with Wood`s
lamp.
EndvthrIr TIneu CupItIs
B|at| Jo" nea tas . Bioken-off haiis neai
suiface give appeaiance of dots" (Fig. 25-16)
(swollen haii shafts) in daik-haiied patients.
Dots occui as affected haii bieaks at suiface
of scalp. Tends to be diffuse and pooily cii-
cumsciibed.
Low-giade folliculitis may be piesent.
Resembles seboiiheic deimatitis, chionic cu-
taneous lupus eiythematosus.
Usually caused by T. onsurans, T. o|ateum .
Onychomycosis also occuis in 2-3% of cases.
Keron :
Inflammatoiy mass in which iemaining
haiis aie loose.
Chaiacteiized by boggy, puiulent, inflamed
nodules and plaques (Fig. 25-17).
Usually extiemely painful; diains pus fiom

multiple openings, like honeycomb.
Haiis do not bieak off but fall out and
can be pulled without pain. Follicles may
dischaige pus; sinus foimation; mycetoma-
like giains.
Thick ciusting with matting of adjacent
haiis.
A single plaque is usual, but multiple lesions
may occui with involvement of entiie scalp.
Fiequently, associated lymphadenopathy is
piesent.
Usually caused by zoophilic ( T. errutosum,
T. menagro|yes vai. menagro|yes ) oi
geophilic species.
Heals with scaiiing alopecia.
gmnae [o||tu|s :
Less seveie inflammation than keiion with
shaiply defined, dull ied plaques studded
with folliculai pustules.
Caused by zoophilic species.
Faus :
Eaily cases show peiifolliculai eiythema
and matting of haii.
FICk 25-15 Ioea capts: "ray patch" type A |+|e, |ouud, |,pe||e|+|o||c p|+que o| +|opec|+ due |o
||e+||u o|| o| |+|| |+|| c|oe |o ||e u||+ce, |.|u ||e +ppe+|+uce o| + roWed W|e+| ||e|d ou ||e c+|p o| +
c|||d. ker+|u|u |+|| |+|| +ud c+|e e\||||| + |eeu ||uo|eceuce W|eu e\+r|ued W||| wood |+rp. !. :c
W+ |o|+|ed ou cu||u|e.
Latei, thick yellow adheient ciusts (scutula)
composed of skin debiis and hyphae that
aie pieiced by iemaining haii shafts (Fig.
25-18).
Fetid odoi.
Shows little tendency to cleai sponta-
neously.
Often iesults in cutaneous atiophy, scai
foimation, and scaiiing alopecia.
Caused by T. st|oen|en .
"Cray Fatch" Ioea Capts Seboiiheic deima-
titis, psoiiasis, atopic deimatitis, lichen simplex
chionicus, alopecia aieata.
"8|ack 0ot" Ioea Capts Seboiiheic deima-
titis, psoiiasis, seboiihiasis, atopic deimatitis,
lichen simplex chionicus, chionic cutaneous
lupus eiythematosus, alopecia aieata.
keroo Cellulitis, fuiuncle, caibuncle.
Favus Impetigo, ecthyma, ciusted scabies.
Wood Iamp
Should be peifoimed in any patient with scal-
ing scalp lesions oi haii loss of undeteimined
oiigin.
T. onsurans , the most common cause of tinea
capitis in the United States, does not fluoiesce.
M. tans and M. auJoun , which pievi-
ously weie the most common causes of tinea
capitis, could be diagnosed by Wood lamp
examination, by biight gieen haii shafts with
ectothiix infection.
0rect Mcroscopy
Specimens should include haii ioots and skin
scales.
Pluck haiis and use toothbiush to gathei
specimens.
Skin scales contain hyphae and aithiospoies.
Haii
Eto|rx . aithiospoies can be seen sui-
iounding the haii shaft in cuticle .
EnJo|rx . spoies within haii shaft.
Faus . loose chains of aithiospoies and
aiispaces in haii shaft.
FICk 25-16 Ioea capts: "b|ack dot" varaot A u|||e, +,rp|or+||c p+|c| o| +|opec|+ due |o ||e+|
|u o|| o| |+|| ou ||e ||ou|+| c+|p |u + +,e+|o|d ||+c| c|||d. I|e |e|ou W+ de|ec|ed |ec+ue |e| |u|+u| ||e|
p|eeu|ed W||| ||ue+ co|po||. :||,| |c W+ |o|+|ed ou cu||u|e.
Fuoa| Cu|ture With biush-cultuie tech-
nique, a diy toothbiush oi biush used foi
ceivical Pap testing is iubbed ovei aiea of
scale oi alopecia; biistles aie then inoculated
into fungal medium. A wet cotton swab can
also be iubbed in affected aiea, which is then
implanted into medium. The cotton-tipped
swab fiom a bacteiial cultuiette, moistened
with watei, can also be used to collect the
specimen and sent to a commeicial laboiatoiy.
Giowth of deimatophytes usually seen in 10
to 14 days.
FICk 25-1T keroo Au e\||ere|, p+|u|u|, |o,, pu|u|eu| |u||+rr+|o|, uodu|e ou ||e c+|p o| |||
+,e+|o|d c|||d. I|e |e|ou d|+|u pu ||or ru|||p|e opeu|u +ud ||e|e | |e||o+u||cu|+|, |eude| |,rp|+deuop+
||,. |u|ec||ou W+ due |o .-: cou||+c|ed ||or +u |u|ec|ed |+||||.
FICk 25-18 Ioea capts: Iavus E\|eu|.e |+|| |o W||| +||op|,, c+|||u, +ud oc+||ed cu|u|+, |.e.,
,e||oW|| +d|e|eu| c|u| p|eeu| ou ||e c+|p, |er+|u|u |+|| p|e|ce ||e cu|u|+. :||,| :|-|- W+
|o|+|ed ou cu||u|e.
EndvthrIr T. onsurans, T. o|ateum, T. sou-
Janense , and T. st|oen|en
EctvthrIr Mtrosorum spp., T. menagro-
|yes, T. errutosum
Fuvus T. st|oen|en , most commonly; also
T. o|ateum, M. gyseum
8actera| Cu|ture Rule out bacteiial supeiin-
fection, usually S. aureus oi GAS.
MANACMNI
Freveotoo |rpo||+u| |o e\+r|ue |ore +ud c|oo| cou|+c| o| +||ec|ed c|||d|eu |o|
+,rp|or+||c c+|||e| +ud r||d c+e o| ||ue+ c+p|||. Ke|ocou+/o|e
o| e|eu|ur u|||de |+rpoo r+, |e |e|p|u| |u e|+d|c+||u ||e
+,rp|or+||c c+|||e| |+|e.
Iopca| aotIuoa| aeots Iop|c+| +eu| +|e |ue||ec||.e |u r+u+ereu| o| ||ue+ c+p|||. |u|+||ou o|
||e+|reu| |ou|d |e e\|euded uu||| ,rp|or |+.e |eo|.ed +ud |uu+|
cu||u|e ue+||.e.
0ra| aotIuoa| aeots C||eo|u|.|u | cou|de|ed ||e d|u o| c|o|ce |u ||e uu||ed '|+|e. '|o||
|e|r |e|||u+||ue, |||+cou+/o|e, +ud ||ucou+/o|e |+.e |eeu |oWu |o |e
corp+|+||e |u e|||c+c, +ud +|e|, |o ||eo|u|.|u.
C||eo|u|.|u Fedatrc 0ose
\|c|o|/ed. 5 r/| pe| d+,, r+\|rur 500 r/d
u|||+r|c|o|/ed. 0 r/| pe| d+,
I|e+|reu| du|+||ou. +| |e+| o Wee| |o e.e|+| rou||, |e||e| +|o|p||ou
W||| |+||, re+|.
Adu|t 0ose
C|+, p+|c| ||ue+ c+p|||. 250 r |W|ce d+||, |o| o| 2 rou||
B|+c| do| ||ue+ c+p|||. |oue| ||e+|reu| +ud |||e| doe cou||uued
uu||| K0n +ud cu||u|e +|e ue+||.e
| |-
250 r |W|ce d+||, |o| +-3 Wee|, |o| corp|ee, +u||||o||c |o|
+ccorp+u,|u |+p|,|ococc+| |u|ec||ou
Ie|||u+||ue 250 r/d. keduce do|u +cco|d|u |o We||| |u ped|+|||c p+||eu|.
|||+cou+/o|e 00r c+pu|e o| o|+| o|u||ou (0 r/r|). I|e+|reu| du|+||ou.
+-3 Wee|.
Fedatrc 0ose 5 r/| pe| d+,
Adu|t 0ose 200 r/d
||ucou+/o|e 00, 50, 200r |+||e|, o|+| o|u||ou (0 r/r|, +0 r/r|). o-3 r/|
pe| d+,. I|e+|reu| du|+||ou. !-+ Wee| (|u ore c+e 2).
Fedatrc 0ose o r/| pe| d+,
|+||, |o| 2 Wee|, |epe+| +| + Wee| || |ud|c+|ed
Adu|t 0ose 200 r/d
Ke|ocou+/o|e 200r |+||e|. I|e+|reu| du|+||ou. +-o Wee|.
Fedatrc 0ose 5 r/| pe| d+,
Adu|t 0ose 200-+00 r/d
Adjuoctve therapy
||edu|oue r/| pe| d+, |o| + d+, |o| c|||d|eu W||| e.e|e, p+|u|u| |e||ou.
',|er|c +u||||o||c || cu||u|e |oW upe||u|ec||ou W||| ' c- o| CA', o|+| +u||||o||c
+cco|d|u |o eu|||.|||e o| o|+u|r. ||+|u pu ||or |e||ou |e|ou.
Surery ||+|u pu ||or |e||ou |e|ou.
Ae oI 0oset Adult.
Sex Males only.
to|oy
T. errutosum, T. menagro|yes vai. men-
agro|yes , most commonly.
May be acquiied thiough animal expo-
suie.
T. ru|rum an uncommon cause.
Fredsposo Factors Moie common in
faimeis.
Sko Symptoms Piuiitus, tendeiness, pain.
Sko Iesoos
Pustulai folliculitis (Fig. 25-19), i.e., haii
follicles suiiounded by ied inflammatoiy
papules oi pustules, often with exudation
and ciusting.
Involved haiis aie loose and easily ie-
moved.
With less folliculai involvement, theie aie
scaling, ciiculai, ieddish patches (tinea
facialis) in which haii is bioken off at the
suiface.
Papules may coalesce to inflammatoiy
plaques topped by pustules.
Keiion: boggy puiulent nodules and
plaques as with tinea capitis (Fig. 25-20).
Beaid and moustache aieas, iaiely, eye-
lashes, eyebiows.
C0kS
Chionic untieated keiion and favus, espe-
cially if infected with S. aureus , iesult in scai-
iing alopecia.
Regiowth of haii is the iule if tieated with
systemic antifungal agents.
|e|r+|op|,||c |||c|or,co| |u.o|.|u ||e |e+|d
+ud rou|+c|e +|e+.
keer||e ||ue+ c+p|||, W||| |u.+|ou o| ||e |+||
|+||.
',, . k|uWo|r o| ||e |e+|d.
IINA 8Ak8A |C|9 . 0.0
|C|0 . B!5.0
Favus may peisist until adulthood. Radio-
theiapy was used to tieat tinea capitis in
the 1930s and 1940s; the incidence of non-
melanoma skin cancei is incieased fouifold
in these individuals.
FICk 25-19 Ioea barbae A o!,e+|o|d r+|e W|||
pu|u|e |u |e+|d +|e+ |o| e.e|+| rou||. A |+|e pu|u|e
|u +u |u||+rr+|o|, uodu|e | eeu ou ||e rou|+c|e +|e+.
E\|eu|.e u|||e ||ue+ |+c|+|| W+ +|o p|eeu|. I|ue+ ped|,
ou,c|or,co|, +ud ||ue+ c|u|| We|e p|eeu| + We||. K0n
p|ep+|+||ou W+ po|||.e, | W+ de|ec|ed ou de|
r+|op|,|e cu||u|e. B+c|e||+| cu||u|e W+ ue+||.e |o| p+||o
eu. |+c|+| |e|ou |eo|.ed W||| o|+| |e|||u+||ue.
Systemc Fodos Regional lymphadenopathy,
especially if of long duiation and if supeiinfected.
8eard Fo||cu|ts S. aureus folliculitis, fuiun-
cle, caibuncle, acne vulgaiis, iosacea, pseudo-
folliculitis.
See Laboiatoiy Examinations," page 712.
MANACMNI
Iopca| Aeots Ineffective.
Systemc Aeots See Management," page
714.
0kMAI0FhIIC F0IIICIIIIS
See Infectious Folliculitis" in Section 32.
FICk 25-20 Ioea barbae aod toea Iaca|s Cou||ueu|, p+|u|u| p+pu|e, uodu|e, +ud pu|u|e ou ||e
uppe| ||p. Ep|de|r+| de|r+|op|,|o| (||ue+ |+c|+||) W||| |+|p|, r+||u+|ed e|,||er+ +ud c+||u | p|eeu| ou
||e c|ee|, e,e||d, e,e||oW, +ud |o|e|e+d. :||,| -|c|,|- W+ |o|+|ed ou cu||u|e. |u ||| c+e,
||e o|+u|r c+ued |Wo d|||uc| c||u|c+| p+||e|u (ep|de|r+| |u.o|.ereu|, ||ue+ |+c|+|| .e|u |o|||cu|+| |u||+rr+
||ou, ||ue+ |+||+e), depeud|u ou W|e||e| |+||ou ||u o| |+||, ||u W+ |u|ec|ed. ('ee +|o |r+e 25.)
|eep de|r+|op|,||c |o|||cu||||, W||| |o|e|u|od,
|+uu|or+ occu|||u |u |epoue |o de|r+|o
p|,|e |u ||e de|r|.
E||o|o,. \o| corrou|, | , |c
k|| |+c|o|
Iop|c+| |ucoco|||co|d +pp||c+||ou
',|er|c |rruuocorp|or|e
A|op|c de|r+||||
C|ero||e|+p, |o| |eu|er|+, |,rp|or+
Au|o|rruue d|e+e
'o||do|+u ||+up|+u| |ec|p|eu|
|+||oeue|. |o|e|u|od, |epoue |o |e|+||u
C||u|c+| ||ud|u.
I,pe
|o|||cu|+| |,pe W||| |oc+| |rruuoupp|e|ou
(|op|c+| |ucoco|||co|d ue)
'u|cu|+ueou uodu|+| |,pe W||| ,|er|c
|rruuocorp|or|e (||. 252). 'o|||+|, o|
ru|||p|e
|o|||cu|oceu|||c p+pu|e +ud pu|u|e +||e
W||||u +u +|e+ o| ep|de|r+| de|r+|op|,|o|
uc| + ||ue+ |ucou||o (||. 253).
||||||u||ou. Au, |+|||e+||u +|e+, c+|p, |+ce,
|o|e+|r, do|ur o| |+ud/|ee|, |+.ed |e.
',, |e|r+|op|,||c o| |||c|op|,||c |+uu
|or+
MAl0CChI CkANI0MA
FICk 25-21 0ermatophytc Io||cu|ts: Majocch raou|oma A 55,e+|o|d d|+|e||c r+|e |eu+| ||+u
p|+u| |ec|p|eu| W||| p+|u|u| uodu|e ou |e|| |oWe| ||||. E|oded p+pu|e W||| c|u||u +|o.e ||e |uee. I|ue+ ped|
+ud ou,c|or,co| We|e +|o p|eeu|. | W+ |o|+|ed ou de|r+|op|,|e cu||u|e. ne W+ ||e+|ed W|||
.o||cou+/o|e.
to|oy
C. a||tans
An oval yeast vaiying in size (2-6 m by
3-9 m).
Polymoiphism is displayed as: yeast foims,
budding yeast, pseudohyphae, tiue hyphae.
Besides C. a||tans , >100 species of the genus
have been identified, most of which aie nei-
thei commensal noi pathogenic foi humans.
Othei pathogenic species, usually in the set-
ting of immunocompiomise, include: C.
rota|s , C. aras|oss , C. gu||ermonJ ,
C. |ruse , C. seuJorota|s , C. |usaneae , C.
g|a|raa .
co|oy
C. a||tans and othei species fiequently colo-
nize the GI tiact. Colonization may occui
duiing biithing fiom the biith canal, duiing
infancy, oi latei.
Oiophaiyngeal colonization is piesent in ap-
pioximately 20% of healthy individuals, the
iate being highei in hospitalized patients.
Fecal colonization is highei than oial, with a
iate of 40-67%; the iate incieases aftei tieat-
ment with antibacteiial agents.
Seiologic and skin test studies indicate that a
significant piopoition of those not colonized
have been exposed to CanJJa in the past.
Antibiotic theiapy incieases the incidence of
caiiiage, the numbei of oiganisms piesent,
and the chances foi tissue invasion.
Appioximately 13% of women aie colonized
vaginally with C. a||tans ; antibiotic theiapy,
piegnancy, oial contiaception, and intiautei-
ine devices inciease the incidence of caiiiage.
E||o|o,
\o| ro| corrou|, c+ued |, ||e ,e+| Cc
!!c c||:c
|e o||eu |, o||e| Cc!!c pp.
C||u|c+| |,pe +ud ||| |+c|o|
'upe|||c|+| |u|ec||ou o| ruco+| u||+ce
Corrou |u o||e|W|e |e+|||, |ud|.|du+| |u
||e o|op|+|,u\ +ud eu||+||+.
0ccu| |u ||e e|||u o| |u|||c+u| |rruuo
corp|or|e |u ||e eop|+u +ud ||+c|eo
||ouc||+| ||ee.
Cu|+ueou c+ud|d|+| occu| ou ro|| oc
c|uded ||u.
||er|u+|ed c+ud|der|+
0ccu| |u |rruuocorp|or|ed |ud|.|du+|
uu+||, +||e| |u.+|ou o| ||e C| ||+c|
',,. C+ud|do|, rou|||+|
CAN0I0IASIS |C|9 . 2
|C|0 . B!1.0
C. a||tans may tiansiently colonize the skin
but is not one of the peimanent floia and is
seldom iecoveied fiom skin of noimal indi-
viduals.
Candidiasis s usually an endogenous infec-
tion.
With balanitis, CanJJa may be tiansmitted
fiom sexual paitnei.
Ae oI 0oset The young and old aie moie
likely to be colonized.
host Factors
Immunocompiomise
Diabetes mellitus
Obesity
Hypeihidiosis, heat, maceiation
Polyendociinopathies
Systemic and topical glucocoiticoids
Chionic debilitation.
Immuoo|oc Factors
Reduced cell-mediated immunity is the most
significant factoi.
Decieased specific anti- CanJJa IgA salivaiy
antibody may be a factoi.
Defects in neutiophil oi maciophage func-
tions aie factois in invasive disseminated
candidiasis.
0rect Mcroscopy KOH piepaiation visualizes
pseudohyphae and yeast foims (Fig. 25-22).
Cu|ture Fungu| Identifies species of Can-
JJa ; howevei, the piesence in cultuie of C. a||-
tans does not make the diagnosis of candidiasis;
CanJJa is a noimal inhabitant of the GI tiact.
Identifying CanJJa in the absence of symptoms
should not lead to tieatment, because 10-20%
of noimal women haiboi CanJJa spp. and oth-
ei yeasts in the vagina. Sensitivities to antifungal
agents can be peifoimed on isolate in cases of
iecuiient infection.
BucterIu| Rule out bacteiial supeiinfection.
CIASSIFICAII0N
See Table 25-1.
IA8I 25-1 C|assification of Candidiasis
Type S|te 0||o|ca| preseotat|oo
0cc|uded ||e (W|e|e Bod, |o|d A\|||+e, u|r+rr+|,, |o|u,
occ|u|ou +ud |u|e||u|e+|, +|dor|u+| p+uu|cu|u
r+ce|+||ou c|e+|e we|p+ce. |+ud (e|o|o
W+|r, ro|| |u|e|d|||+|e ||+|or,ce||c+), |ee|
r|c|oeco|o,) Auu|+| c|e|||||, o||eu +oc|+|ed
W||| o|op|+|,|e+| c+ud|d|+|
Ceu||+| B+|+u|||, |+|+uopo|||||
\u|.|||, .u|.o.+|u|||
0cc|uded ||u uude| occ|u|.e d|e|u, uude| c+|,
|+c| |u |op||+||/ed p+||eu|
|o|||cu|||| B+c|, |u |op||+||/ed p+||eu|
A|e+ occ|uded ||+pe| de|r+||||
uude| d|+pe|
|+|| +pp+|+|u |+|ou,c||ur C||ou|c p+|ou,c||+
|+|| p|+|e 0u,c||+
n,pou,c||ur 'ecoud+|, |u|ec||ou
W||| ou,c|o|,|
C||ou|c rucocu|+ueou E\|eu|.e, ru|||p|e |ud|.|du+| W||| coueu||+|
o| 20 u+|| |rruuo|o|c (I ce|| de|ec|) o|
eudoc||uo|o|c d|o|de|
(|,pop+|+||,|o|d|r,
|,po+d|eu+||r, |,po||,|o|d|r,
d|+|e|e re||||u) de.e|op pe|||eu|
o| |ecu||eu| ruco+|, cu|+ueou,
+ud/o| p+|ou,c||+|/u+|| |u|ec||ou.
Ceu||+||+ \u|.+, .+|u+, E|,||er+, e|o|ou, W|||e
p|epu||+| +c p|+que o| c+ud|d+| co|ou|e
\uco+| 0|op|+|,u\ I||u|, +||op||c c+ud|d|+|,
|,pe|p|+||c c+ud|d|+|
Eop|+u |u||+red, e|oded p|+que
I|+c|e+, ||ouc|| |u||+red, e|oded p|+que
C+ud|der|+ '||u, .|ce|+ '||u. e|,||er+|ou p+pu|e,
|ero|||+e
MANACMNI
0ra| AotIuoa| Aeots Indicated foi infec-
tions iesistant to topical modalities of theiapy.
F|ucvnuzv|e Tablets: 50, 100, 150, 200 mg.
Oial suspension: 50 mg/5 mL. Paienteial: foi
injection oi IV infusion.
1trucvnuzv|e Capsules: 100 mg. Oial solution:
10 mg/mL
Ketvcvnuzv|e Tablets: 200 mg
Iotertro Eiythema. Piuiitus, tendeiness,
pain.
0cc|uded Sko Undei occlusive diessing, un-
dei cast, on back in hospitalized patient.
0aper 0ermatts Iiiitability, discomfoit with
uiination, defecation, changing diapeis.
Sko Iesoos
Iotertro
Initial pustules on eiythematous base become
eioded and confluent.
Subsequently, faiily shaiply demaicated, poly-
cyclic, eiythematous, eioded patches with
small pustulai lesions at the peiipheiy (satel-
lite pustulosis).
Distiibution: Infiamammaiy (submam-
maiy) (Fig. 25-23), axillae, gioins (Fig. 25-24),
peiineal, inteigluteal cleft.
Ioterdta|
Eiosio inteidigitalis candidomycetica.
Most common in obese eldeily.
Initial pustule becomes eioded, with foimation
of supeificial eiosion oi fissuie, suiiounded by
thickened white skin (Fig. 25-25).
May be associated with CanJJa paionychia.
Cu|+ueou c+ud|d|+| occu| |u ro||, occ|uded
cu|+ueou ||e.
\+u, p+||eu| |+.e p|ed|po|u |+c|o|.
CIAN0S CAN0I0IASIS
Dsr|uon :
Hands: usually between thiid and fouith
fingeis (Fig. 25-25)
Feet: maceiation in webspace
0aper 0ermatts
Eiythema, edema with papulai and pustu-
lai lesions; eiosions, oozing, collaiette-like
scaling at the maigins of lesions
Peiigenital and peiianal skin, innei aspects of
thighs and buttocks (Fig. 25-26).
Fo||cu|ar Caoddass
Small, disciete pustules in ostia of haii fol-
licles.
Usually in occluded skin.
Iotertro[0cc|uded Sko Nonspecific intei-
tiigo, stieptococcal inteitiigo, inteitiiginous
psoiiasis, eiythiasma, deimatophytosis, pityii a-
sis veisicoloi.
0aper 0ermatts Atopic deimatitis, psoiiasis,
iiiitant deimatitis, seboiiheic deimatitis.
Fo||cu|ts Bacteiial ( S. aureus , P. aerugnosa )
folliculitis, Pyrosorum folliculitis, acne.
FICk 25-22 Cooddo olbcoos: k0h

preparatoo Budd|u ,e+| |o|r +ud +u+e|||e
peudo|,p|+| |o|r.
FICk 25-23 Cutaoeous caoddass: otertro 'r+|| pe||p|e|+| +|e||||e p+pu|e +ud pu|u|e ||+|
|+.e |ecore cou||ueu| ceu||+||,, c|e+||u + |+|e e|oded +|e+ |u ||e u|r+rr+|, |e|ou.
FICk 25-24 Cutaoeous caoddass: otertro E|,||er+|ou p+pu|e W||| + |eW pu|u|e, |ecor|u
cou||ueu| ou ||e red|+| ||||. I|e |e|ou occu||ed du||u + |o||d+, |||p |o ||e C+||||e+u.
See Laboiatoiy Examination," page 718.
0IACN0SIS
Clinical findings confiimed by diiect micios-
copy oi cultuie.
MANACMNI
See Table 25-2.
IA8I 25-2 Nanaement of Cutaneous Candidiasis
Freveotoo Keep |u|e|||||uou +|e+ d|, (o||eu d||||cu||).
w+||u W||| |eu/o,| pe|o\|de |+| r+, |educe Cc!!c
co|ou|/+||ou.
|oWde| W||| |r|d+/o|e +pp||ed d+||,.
Iopca| treatmeot
C+|e||+u| p+|u| B||u +|ro| |rred|+|e |e||e| o|
,rp|or, |.e., c+ud|d+| p+|ou,c||+.
C|ucoco|||co|d p|ep+|+||ou lud|c|ou |o|||e|r ue peed
|eo|u||ou o| ,rp|or.
Iopca| aotIuoa| aeots Au|||uu+| p|ep+|+||ou. |,|+||u, +/o|e,
o| |r|d+/o|e c|e+r |W|ce d+||, o| ro|e o||eu
W||| d|+pe| de|r+||||. Io|u+||+|e uo|
e||ec||.e |o| c+ud|d|+|. Ie|||u+||ue r+,
|e e||ec||.e.
|,|+||u c|e+r E||ec||.e |o| Cc!!c ou|,, uo| e||ec||.e
|o| de|r+|op|,|o|.
|r|d+/o|e c|e+r E||ec||.e |o| c+ud|d|+|,
de|r+|op|,|o|, +ud p||,||+| .e||co|o|.
0ra| aotIuoa| aeots E||r|u+|e |oWe| co|ou|/+||ou. A/o|e
||e+| cu|+ueou |u|ec||ou.
|,|+||u (upeu|ou, |+||e|, p+||||e) |o| +|o||ed ||or ||e |oWe|.
E|+d|c+|e |oWe| co|ou|/+||ou. \+, |e
e||ec||.e |u |ecu||eu| c+ud|d|+| o|
d|+pe| +|e+, eu||+|, o| |u|e||||o.
Systemc aotIuoa| aeots 'ee '\+u+ereu|, p+e 121.
FICk 25-25 Cutaoeous caoddass: oterdta| otertro Au 30,e+|o|d r+|e W||| p|e\||o|r ueu|o
||||or+ o| ||e |e|||+ud uo|ed p+|u|u| e|o|ou |u ||e We|p+ce o| ||e |+ud. E|o|ou W||| e|,||er+ | eeu |u ||e
We|p+ce |e|Weeu |Wo ||ue|, ||e ueu|o||||or+ |+ c|e+|ed +u |u|e|||||uou p+ce.
FICk 25-26 Caoddass: daper dermatts Cou||ueu| e|o|ou, r+||u+| c+||u, +ud +|e||||e pu
|u|e |u ||e +|e+ co.e|ed |, + d|+pe| |u +u |u|+u|. A|op|c de|r+|||| o| po||+| +|o occu| |u ||| d|||||u||ou +ud
r+, |e coucor||+u|.
0ccu| W||| r|uo| .+||+||ou o| |o| |+c|o|
uc| +
Au||||o||c ||e|+p,
C|ucoco|||co|d ||e|+p, (|op|c+| o| ,|er|c)
Ae (.e|, ,ouu, .e|, o|d)
'|u|||c+u| |rruuocorp|or|e.
Eop|+e+| +ud/o| ||+c|eo||ouc||+| c+ud|d|+|
r+, |e +oc|+|ed W||| 0|C
A|W+, occu| |u ||e e|||u o| +d.+uced
|rruuocorp|or|e.
Cc!!c c+u |u.+de |||ou| e|oded ruco+,
W||| |eu||+u| c+ud|der|+.
',, I||u|
0k0FhAkNCAI CAN0I0IASIS (0FC) |C|9 . 2.0
|C|0 . B!3.0
to|oy
C. a||tans , one of the iesident floia of the
mouth, oveigiows in association with vaiious
local oi systemic factois.
In some cases, an exogenous infection.
Aftei chionic antifungal theiapy, especially
with advanced immunocompiomise, fluco-
nazole-iesistant stiains of CanJJa spp. can
evolve and cause infection iesistant to oial/in-
tiavenous azole theiapy.
Iocdeoce Although a numbei of iisk factois
exist (see below) and almost obligatoiy involve-
ment occuis in immunocompiomised patients,
the vast majoiity of mucosal candidiasis occuis
in otheiwise healthy individuals.
H1V DIseuse In the absence of effective
antiietioviial theiapy (ART):
OPC occuis in 50% of HIV-infected patients
80-95% of those with AIDS
60% ielapse within 3 months aftei tieat-
ment.
Esophageal candidiasis occuis in 10-15% of
peisons with AIDS.
Bvne Murrvw Trunsp|unt RecIpIents 30-40%
develop supeificial mucosal candidiasis.
C0C Surve||aoce Case 0eIotoo Ior AI0S Can-
didiasis of the esophagus, tiachea, bionchi, oi
lungs is an AIDS-defining condition if the patient
has no othei cause of immunodeficiency and is
without knowledge of HIV antibody status.
CIASSIFICAII0N 0F MC0SAI CAN0I0IASIS
SuperIca| mucosa| caoddass: may be
associated with mild to modeiate impaii-
ment of cell-mediated immunity
0ropharyoea| caoddass: pseudomembianous
candidiasis (thiush);
eiythematous (atiophic) candidiasis;
candidal leukoplakia (hypeiplastic candi-
diasis);
angulai cheilitis
0eep mucosa| caoddass: occuis in states of
advanced immunocompiomise (AIDS-
defining):
esophageal candidiasis; t iacheobionchial
candidiasis, both of which aie AIDS-defining
conditions; bladdei
Symptoms
Orvphurynr
Often symptomatic.
Buining oi pain on eating spices/acidic foods,
diminished taste sensation.
Cosmetic concein about white cuids on
tongue.
Odynophagia.
In HIV/AIDS, may be the initial piesentation;
OPC is a clinical maikei foi disease piogies-
sion, fiist noted when CD4 cell count is
390/L.
Esvphugus
Often asymptomatic.
Occuis when CD4 cell count is low (<200/
L) and is an AIDS-defining condition.
Odynophagia, iesulting in difficulty eating
and malnutiition.
Mucosa| Iesoos
Iype Pseudvmemhrunvus CundIdIusIs (Thrush)
(Figs. 25-27 and 25-28)
White-to-cieamy plaques on any mucosal
suiface; vaiy in size fiom 1-2 mm to extensive
and widespiead.
Removal with a diy gauze pad leaves an eiy-
thematous mucosal suiface.
Distiibution: Doisum of tongue, buccal mu-
cosa, haid/soft palate, phaiynx extending
down into esophagus and tiacheobionchial
tiee.
Erythemutvus (AtrvphIc) CundIdIusIs
Smooth, ied, atiophic patches (Fig. 25-29).
Aieas of thiush may also be piesent.
Distiibution: Haid/soft palate, buccal mu-
cosa, doisal suiface of tongue.
CundIdu| Leu|vp|u|Iu
White plaques that cannot be wiped off but
iegiess with piolonged anticandidal theiapy.
Distiibution : buccal mucosa, tongue, haid
palate.
FICk 25-2T 0ra| caoddass:
thrush w|||e cu|d|||e r+|e||+|
ou ||e ruco+| u||+ce o| ||e |oWe|
||p o| + c|||d, ||e r+|e||+| c+u |e
+||+ded W||| +u/e (peudorer||+
uou), |e.e+||u uude||,|u e|,||er+.
FICk 25-28 0ra| caoddass: thrush E\|eu|.e co||+e c|eee|||e p|+que, co|ou|e o| Cc!!c ||+|
c+u |e |ero.ed |, |u|||u W||| +u/e (peudorer||+uou), ou ||e p+|+|e +ud u.u|+ o| +u |ud|.|du+| W|||
+d.+uced n|\/A||'. |+|c|e o| e|,||er+ |e|Weeu ||e W|||e p|+que |ep|eeu| e|,||er+|ou (+||op||c) c+ud|d|+
|. |u.o|.ereu| r+, e\|eud |u|o ||e eop|+u +ud |ecore +oc|+|ed W||| d,p|+|+.
Angu|ur CheI|ItIs
Inteitiigo at the coinei (angles) of lips (Fig.
25-29).
Eiythema; slight eiosion
White colonies of CanJJa in some cases
Usually associated with oiophaiyngeal colo-
nization with CanJJa
Ceoera| Fodos
Iovasve 0ssemoated Caoddass
In individuals with seveie piolonged neutio-
penia
Poital of entiy of CanJJa
GI tiact
Invades submucosa and blood vessels
Intiavasculai cathetei
Candidemia: hematogenous dissemination to
skin and visceia
Fseudomembraoous Caoddass (Ihrush) Oial
haiiy leukoplakia, condyloma acuminatum, ge-
ogiaphic tongue, haiiy tongue, lichen planus,
bite iiiitation.
Atrophc (rythematous) Caoddass Lichen
planus
See Candidiasis," page 718.
odoscopy Documents esophageal and/oi tia-
cheobionchial candidiasis.
0IACN0SIS
Clinical suspicion confiimed by KOH piepaia-
tion of sciaping fiom mucosal suiface.
C0kS AN0 Fk0CN0SIS
Most cases iespond to coiiection of the pie-
cipitating cause (e.g., use of inhaled glucocoi-
ticoids).
Topical agents effective in most cases.
Clinical iesistance to antifungal agents may
be ielated to patient noncompliance, seveie
immunocompiomise, diug-diug inteiaction
(iifampin-fluconazole).
Bone maiiow tiansplant iecipients
30-40% develop supeificial mucosal can-
didiasis;
10-25% develop deep invasive candidiasis,
of whom some develop chionic visceial can-
didiasis, and 25% die fiom the infection.
HIV/AIDS
Oiophaiyngeal and esophageal candidia-
sis have been iepoited with piimaiy HIV
infection.
In latei HIV/AIDS without effective ART
OPC is neaily univeisal.
Esophageal infection occuis in 10-20%
of patients.
Relapse aftei topical oi systemic tieat-
ment is expected.
Viitually all HIV/AIDS-infected individu-
als with CD4 cell counts of 100/L aie
colonized with CanJJa.
FICk 25-29 0ra| caoddass: atrophc |os-

sts wth aou|ar che|ts A o1,e+|o|d +|co|o||c
r+|e W||| o|e |ouue. I|e u||+ce o| ||e |ouue |
+||op||c +ud ||u,, +u |u|e||||o | p|eeu| +| ||e +u|e
o| ||e ||p.
MANACMNI
Iopca| Iherapy These piepaiations aie effec-
tive in the immunocompetent individual but
ielatively ineffective with decieasing cell-medi-
ated immunity.
NystutIn (Note: Nystatin is not absoibed
fiom the GI tiact)
Oial tablets: 100,000 units foui times a day
dissolved slowly in the mouth, aie the most
effective piepaiation.
Oial suspension: 1-2 teaspoons, held in
mouth foi 5 min and then swallowed, may be
effective.
C|vtrImuzv|e
Oial tablets (tioche), 10 mg, one tablet 5
times daily may be effective.
hIv 0sease Responds to topical and/oi sys-
temic theiapy; howevei, iecuiience is the iule.
May become iefiactoiy to inteimittent theiapy,
iequiiing daily chemopiophylaxis with eithei
topical oi systemic tieatment. Inciease dose
with iesistant disease.
Systemc Iherapy
F|ucvnuzv|e
Oial
200 mg PO once followed by 100 mg/d foi
2-3 weeks, then discontinue.

to|oy >20% of noimal women have vaginal
colonization with CanJJa . C. a||tans accounts
foi 80-90% of genital isolates.
Iocdeoce
Most vaginal candidiasis (VC) occuis in the
noimal population.
0ccu| ou ||e uou|e|+||u|/ed eu||+| ruco+
\u|.+, .+|u+
||epu||+| +c o| ||e peu|
uu+||, |ep|eeu| o.e||oW|| o| eudoeuou
co|ou|/|u Cc!!c |+||e| ||+u ||or e\oeuou
ou|ce (e\u+| p+||ue|)
',, \e+| |u|ec||ou
CNIIAI CAN0I0IASIS |C|9 . 2./2.2

|C|0 . B!1.!/B!1.+
Inciease the dose to 400-800 mg in iesistant
infection.
IV: Also available
1trucvnuzv|e
Capsules oi oial solution
100 mg PO daily oi twice daily foi 2 weeks
Inciease dose with iesistant disease
Ketvcvnuzv|e
200 mg PO daily oi twice daily foi 1-2 weeks
F|ucooato|e-kesstaot Caoddass
Defined as clinical peisistence of infection
aftei tieatment with fluconazole, 100 mg/d
PO foi 7 days.
Occuis most commonly in HIV-infected
individuals with CD4 cell counts <50/L
who have had piolonged fluconazole expo-
suie.
Chionic low-dose fluconazole tieatment
(50 mg/d) facilitates emeigence of iesistant
stiains; 50% of iesistant stiains sensitive to
itiaconazole.
Amphoteiicin B foi seveie iesistant disease.
Liposomal piepaiations aie effective and less
toxic.
Recuiience is the iule; maintenance theiapy is
often iequiied.
75% of women expeiience at least one epi-

sode of VC duiing theii lifetime.
40-45% expeiience two oi moie episodes.
Often associated with vulvai candidiasis, i.e.,
vulvovaginal candidiasis (VVC).
A small peicentage of women (piobably <5%)
expeiience iecuiient VVC (RVVC).
ksk Factors Diabetes, HIV/AIDS
Females
Often none
Piegnancy
Usually sexually active, but also in sexually
inactive, young, eldeily
Males
Unciicumcised
Iraosmssoo
In neonatal OPC, C. a||tans is acquiied fiom
the genital tiact of mothei.
To males fiom colonized sexual paitneis.
Symptoms
Vu|vItIs/ Vu|vvvugInItIs
Onset often abiupt, usually the week befoie
menstiuation.
Symptoms may iecui befoie each menstiua-
tion.
Piuiitus, vaginal dischaige, vaginal soieness,
vulvai buining, dyspaieunia, exteinal dysu-
iia.
Bu|unvpvsthItIs, Bu|unItIs Buining, itching,
iedness
Mucosa| Iesoos
Vu|vItIs
Eiosions, pustules, eiythema (Fig. 25-30),
swelling, iemovable cuidlike mateiial
Vu|vItIs/Vu|vvvugInItIs
Vaginitis with white dischaige.
Vaginal eiythema and edema; white plaques
that can be wiped off on vaginal and/oi ceivi-
cal mucosa.
May be associated with candidal inteitiigo
of inguinal folds and peiineum. Subcoineal
pustules at peiipheiy with fiinged, iiiegulai
maigins.
In chionic cases, vaginal mucosa glazed and
atiophic.
Bu|unvpvsthItIs, Bu|unItIs
Glans and pieputial sac: papules, pustules,
eiosions (Fig. 25-31).
Maculopapulai lesions with diffuse eiy-
thema.
Edema, ulceiations, and fissuiing of piepuce,
usually in diabetic men; white plaques undei
foieskin.
vC[vvC Tiichomoniasis (caused by T. ag-
na|s), bacteiial vaginosis (caused by ieplace-
ment of noimal vaginal floia by an oveigiowth
of anaeiobic miciooiganisms and CarJnere||a
agna|s), lichen planus, lichen scleiosus et
atiophicus.
8a|aooposthts Psoiiasis, eczema, lichen
planus
See Candidiasis," page 718.
0IACN0SIS
Clinical suspicion confiimed by KOH piepaia-
tion of sciaping fiom mucosal suiface.
C0kS AN0 Fk0CN0SIS
kecurreot vvC
Defined as thiee oi moie episodes of sympto-
matic VVC annually.
Affects a small piopoition of women (<5%).
The natuial histoiy and pathogenesis of ie-
cuiient VVC aie pooily undeistood.
The majoiity of women with RVVC have no
appaient piedisposing conditions.
MANACMNI
vC[vvC Iopca| Iherapy
Azoles/imidazoles
Moie effective than nystatin
Result in ielief of symptoms and negative
cultuies among 80-90% of patients aftei
theiapy is completed.
Recvmmended RegImens Single-dose iegi-
mens piobably should be ieseived foi cases of
uncomplicated mild to modeiate VVC. Multi-
day iegimens (3- to 7-day) aie the piefeiied
tieatment foi seveie oi complicated VVC.
Butoconazole: 2% cieam 5 g intiavaginally
foi 3 days or
Clotiimazole: 1% cieam 5 g intiavaginally
foi 7-14 days or 100-mg vaginal tablet foi
7 days or 100-mg vaginal tablet, two tablets
foi 3 days or 500-mg vaginal tablet, one tablet
in a single application or

FICk 25-30 Caoddass: vu|vts |o
||+||o|r, e|,||er+|ou |e|ou |ecor|u cou
||ueu| ou ||e .u|.+ W||| e|o|ou +ud +|e||||e
pu|u|e ou ||e ||||.
FICk 25-31 Caoddass:
ba|aooposthts A 52,e+|o|d
uuc||curc|ed r+|e W||| peu||e
p+|u |o| 1 d+,, |op|c+| +pp||c+||ou
o| |,d|oco|||oue c|e+r Wo|eued
,rp|or. E|,||er+ +ud + cu|d|||e
r+||e| | eeu ou ||e |+u peu| +ud
|o|e||u.
Miconazole: 2% cieam 5 g intiavaginally foi
7 days or 200-mg vaginal suppositoiy, one
suppositoiy foi 3 days or 100-mg vaginal sup-
positoiy, one suppositoiy foi 7 days or
Tioconazole: 6.5% ointment 5 g intiavagi-
nally in a single application or
Teiconazole: 0.4% cieam 5 g intiavaginally
foi 7 days or 0.8% cieam 5 g intiavaginally foi
3 days or 80-mg suppositoiy, one suppositoiy
foi 3 days
Fluconazole: 150 mg PO as a single dose
kecurreot vvC Weekly dosing with the follow-
ing may be effective:
Clotiimazole: 500-mg vaginal tablet, one tab-
let in a single application or
Fluconazole: 150 mg PO as a single dose
Itiaconazole: 100 mg PO bid
8a|aots, 8a|aooposthts
Azole cieam twice daily.
Tieat sexual paitnei if iecuiient.
SysemIc Treumen See page 727.
CAN0I0IASIS 0F Ih NAII AFFAkAIS
'ee '||o|de| o| ||e |+|| App+|+|u, 'ec||ou !!.
C|+|+c|e||/ed |, pe|||eu|/|ecu||eu| Cc!!c
|u|ec||ou o| ||e o|op|+|,u\, ||u, +ud u+|| +p
p+|+|u (||. 25!2 +ud 25!!).
uu+||, +oc|+|ed W|||
uude||,|u |rruuocorp|or|e
0ue| |u |u|+uc, o| e+||, c|||d|ood.
0|op|+|,ue+| c+ud|d|+|.
ke||+c|o|, |o cou.eu||ou+| ||e|+p,
ke|+p|u +||e| ucce|u| ||e|+p,
C||ou|c |u|ec||ou |eu|| |u |,pe|||op||c (|eu|o
p|+||c) c+ud|d|+|.
Cu|+ueou c+ud|d|+| r+u||e| +.
|u|e||||o
w|dep|e+d |u|ec||ou (||. 25!2, 25!!) o| ||e
||uu| +ud/o| e\||er|||e
|e|ou |ecore |,pe|||op||c |u c||ou|c uu
||e+|ed c+e.
|u|ec||ou o| ||e u+|| +pp+|+|u | uu|.e|+| W|||
C||ou|c p+|ou,c||+
|+|| p|+|e |u|ec||ou +ud d,||op|,
E.eu|u+||, |o|+| u+|| d,||op|,.
\+u, p+||eu| +|o |+.e de|r+|op|,|o| +ud
cu|+ueou W+||.
'|\ |,pe o| C\C |+.e |eeu de||ued.
C||ou|c o|+| c+ud|d|+|
C||ou|c c+ud|d|+| W||| eudoc||uop+||,
C||ou|c c+ud|d|+| W|||ou| eudoc||uop+||,
C||ou|c |oc+||/ed rucocu|+ueou c+ud|d|+|
C||ou|c d|||ue c+ud|d|+|
C||ou|c c+ud|d|+| W||| ||,ror+.
Chk0NIC MC0CIAN0S CAN0I0IASIS (CMC) |C|9 . 2.!
|C|0 . B!1.1
CAN0I0MIA AN0 0ISSMINAI0
CAN0I0IASIS
'ee '',|er|c |uu+| |u|ec||ou W||| ||er|u+
||ou |o '||u, |e|oW.
FICk 25-32 Mucocutaoeous
caoddass |e|||eu| c+ud|d|+|
|u +u |rruuocorp|or|ed |u|+u|
r+u||e||u + e|o|ou co.e|ed |,
c+|e +ud c|u|, o|op|+|,ue+|
c+ud|d|+|, +ud W|dep|e+d |u|ec||ou
o| ||e ||uu|.
FICk 25-33 Mucocutaoeous caoddass I|| !,e+|o|d c|||d W||| |,po||,|o|d|r |+d o|+| |||u|,
|u|e|||||uou c+ud|d|+|, W+||, |,pe||e|+|oe, +ud c|u| ou ||e c+|p +ud |+ce, +ud +|o, c+ud|d+| ou,c|or,co
|. I|e W+||, |oW|| |oWu |u ||e p|o|o cou||ed o| d||ed pu, e|ur, +ud pu|e cu||u|e o| Cc!!c.
to|oy
M. [ur[ur (pieviously known as Pyrosorum
oa|e, P. or|tu|are )
Lipophilic yeast that noimally iesides in the
keiatin of skin and haii follicles of individuals
at pubeity and beyond.
An oppoitunistic oiganism, causing pityiia-
sis veisicoloi and Ma|asse:a folliculitis; it is
implicated in the pathogenesis of seboiiheic
deimatitis.
Ma|asse:a infections aie not contagious.
O veigiowth of iesident cutaneous floia oc-
cuis undei ceitain favoiable conditions.
Ae oI 0oset Young adults. Less common
when sebum pioduction is ieduced oi absent;
tapeis off duiing fifth and sixth decades.
Fredsposo Factors
Waim season oi climates; tiopical climate
Hypeihidiosis; aeiobic exeicise
Oily skin
Glucocoiticoid tieatment
Immunodeficiency
Application of lipids such as cocoa buttei
piedisposes young childien to PV
Seasoo
Tempeiate zones
Moie common in summeitime
2% of population
May iegiess duiing coolei months
In physically active individuals, may peisist
yeai iound.
Subtiopical and tiopical zones
Yeai iound
20% of population
C||ou|c +,rp|or+||c c+||u ep|de|ror,co|

Aoc|+|ed W||| ||e upe|||c|+| o.e||oW|| o| ||e
|,p|+| |o|r o| !c|c-cc ||
C||u|c+| ||ud|u.
we||der+|c+|ed c+||u p+|c|e
\+||+||e p|reu|+||ou. |,po +ud |,pe|p|
reu|ed, p|u|
\o| corrou|, ou ||e ||uu|.
',, I|ue+ .e||co|o|
FIIkIASIS vkSIC0I0k (Fv) |C|9 . .0
|C|0 . B!o.0
FAIh0CNSIS
Ma|asse:a changes fiom the blastospoie foim
to the mycelial foim undei the influence of
piedisposing factois (see above).
Dicaiboxylic acids foimed by enzymatic oxi-
dation of fatty acids in skin suiface lipids
inhibit tyiosinase in epideimal melanocytes
and theieby lead to hypomelanosis.
The enzyme is piesent in M. [ur[ur .
0uratoo oI Iesoos Months to yeais.
Sko Symptoms
Usually none.
Occasionally, mild piuiitus.
Individuals with PV usually piesent because
of cosmetic conceins about the dyspigmen-
tation.
Sko Iesoos
Macules, shaiply maiginated (Figs. 25-34
thiough 25-36), iound oi oval in shape, vaiy-
ing in size.
Fine scaling is best appieciated by gently
abiading lesions with a no. 15 scalpel blade oi
the edge of a micioscope slide.
Tieated oi buined-out lesions lack scale.
Some patients have findings of Ma|asse:a fol-
liculitis and seboiiheic deimatitis.
Coloi:
In untanned skin, lesions aie light biown
(Fig. 25-35).
On tanned skin, hypopigmented (Fig. 25-36).
In biown- oi black-skinned peisons, daik
biown macules (Fig. 25-34). Biown of vaiy-
ing intensities and hues; off-white macules.
C||u|c+| ,ud|ore
|||,||+| o| ||ue+ .e||co|o|
!c|c-cc |o|||cu||||
'e|o|||e|c de|r+|||| (|rp||c+|ed |u p+||oeue|)
INFCII0NS
FICk 25-34 Ftyrass ver-
sco|or: hyperpmeoted A
2!,e+|o|d o|ee ||+c| |er+|e W|||
d|co|o|+||ou o| ||e uec| |o| ,e+|.
'|+|p|, r+||u+|ed ||oWu c+||u
r+cu|e ou ||e |e|| |de o| ||e uec|.
I|e .e|.e|, |e\|u|e +ud |,pe|p|
reu|+||ou o| ||e ||u o| ||e uec| |
+c+u||o| u|||c+u +oc|+|ed W|||
o|e||,.
FICk 25-35 Ftyrass versco|or:
hyperpmeoted A !o,e+|o|d r+|e W|||
p|reu|ed p+|c|e ou ||uu| +ud +|r |o| e.
e|+| ,e+|. \u|||p|e p|u|, We||der+|c+|ed c+|
|u r+cu|e |ecor|u cou||ueu| ou ||e uppe|
+ud |+|e|+| ||uu|, uec| +ud +|r.
Some PV lesions aie pink (Fig. 25-35).
In time, individual lesions may enlaige,
meige, foiming extensive geogiaphic aieas.
Distiibution:
Uppei tiunk, uppei aims, neck, abdomen,
axillae, gioins, thighs, genitalia.
Facial, neck, and/oi scalp lesions occui
in patients applying cieams/ointments oi
topical glucocoiticoid piepaiations.
hypopmeoted Fv Vitiligo, pityiiasis alba,
postinflammatoiy hypopigmentation, tubeicu-
loid lepiosy.
Sca|o Iesoos Tinea coipoiis, seboiiheic
deimatitis, pityiiasis iosea, guttate psoiiasis,
nummulai eczema.
0rect Mcroscopc xamoatoo oI Sca|es
Frepared wth k0h
Filamentous hyphae and globose yeast foims,
teimed sag|e anJ mea|a||s , aie seen (Fig.
25-37).
Wood Iamp
Blue-gieen fluoiescence of scales.
MANACMNI
Iogical agents
'e|eu|ur u|||de (2.5) |o||ou o| |+rpoo App|, d+||, |o +||ec|ed +|e+ |o| 0-5 r|u, |o||oWed |,
|oWe|, |o| Wee|
Ke|ocou+/o|e |+rpoo App||ed +re + e|eu|ur u|||de |+rpoo
A/o|e c|e+r (|e|ocou+/o|e, ecou+/o|e, App|, d+||, o| |W|ce d+||, |o| 2 Wee|
r|c|ou+/o|e, c|o|||r+/o|e)
Ie|||u+||ue o|u||ou App|, |W|ce d+||, |o| 1 d+,
5ystemic tberagy (|oue o| ||ee +eu| | +pp|o.ed |o| ue |u |\ |u ||e uu||ed '|+|e)
Ke|ocou+/o|e +00 r |+| (|+|e | |e|o|e e\e|c|e)
||ucou+/o|e +00 r |+|
|||+cou+/o|e +00 r |+|
5econdary grogbylaxis Ke|ocou+/o|e |+rpoo ouce o| |W|ce + Wee|
'e|eu|ur u|||de (2.5) |o||ou o| |+rpoo
'+||c,||c +c|d/u||u| |+|
|,|||||oue /|uc (|+| o| |+rpoo)
Ke|ocou+/o|e +00 r |0 rou|||,
May be negative in individuals who have
showeied iecently because the fluoiescent
chemical is watei soluble.
Vitiligo appeais as depigmented, white, and
has no scale.
0ermatopatho|oy
Budding yeast and hyphal foims in the most
supeificial layeis of the stiatum coineum,
seen best with PAS stain.
Vaiiable hypeikeiatosis, psoiiasifoim hypei-
plasia, chionic inflammation with blood ves-
sel dilatation.
0IACN0SIS
Clinical findings, confiimed by positive KOH
piepaiation findings.
C0kS AN0 Fk0CN0SIS
Infection peisists foi yeais if piedisposing con-
ditions peisist. Dyspigmentation peisists foi
months aftei infection has been eiadicated.
F0IIICIIIIS
'ee '|u|ec||ou |o|||cu|||| 'ec||ou !2.
S80kkhIC 0kMAIIIIS
'ee ''e|o|||e|c |e|r+||||, 'ec||ou 2.
FICk 25-36 Ftyrass versco|or: hypopmeoted \u|||p|e, r+|||ored|ur|/ed, We||der+|c+|ed
|,pop|reu|ed r+cu|e ou ||e |+c| o| + |+uued |ud|.|du+| W||| W|||e ||u.
FICk 25-3T Molossezo lurlur : k0h preparatoo kouud ,e+| +ud e|ou+|ed peudo|,p|+| |o|r,
oc+||ed 'p+|e||| +ud re+||+||.
to|oy: :| pec|e o| ,e+|
||e.|ou|, +|| p+||oeu|c rer|e| o| euu
I||c|opo|ou |e+|ded + + |u|e pec|e, :|
|--|.
|o|eu||+| |ur+u p+||oeu. cc| |
c|-!- :|c- :!- .!-
|||c ||-
'o|| |u|+|||+u|.
Corrou co|ou|/e| o| ||u, |ep||+|o|, +ud C|
||+c|.
Fathoeos
'upe|||c|+| |u|ec||ou. ||ed|+, |u|ec||ou o| |+||
|+||
w|||e p|ed|+. :| cc| .!-
| :!- c|-!- +ud :|c
-
B|+c| p|ed|+. |-!cc ||c-
|u.+|.e |u|ec||ou |u ||e |rruuocorp|or|ed
|o|
cc|
3|c|:|c,:- :c|c| , (|o|re||, :c
|c| +ud +|o |uoWu + C-|:| :c|c
| )
C|oca| Iodos
||ed|+. +,rp|or+||c upe|||c|+| |uu+| |u|ec||ou
o| ||e |+|| |+||.
|||- -!c
|u||c, +\|||+|,, |e+|d, +ud e,e||oW/e,e|+|
|+||
\o|e corrou |u |erpe|+|e +ud er|||op|c+|
|e|ou
w|||e |o |e|e uodu|e ou |+|| |+||, o||, e+||,
|ero.ed.
B|+c| p|ed|+
'c+|p |+||
\o| corrou |u ||op|c+| |e|ou (||| |er
pe|+|u|e, |ur|d||,)
|+|||, p|reu|ed, |||r|, +||+c|ed uodu|e (up
|o + |eW r||||re|e|) ou ||e |+|| |+||, We+|eu
|+|| |+|| W||| |+|| ||e+|+e. n+|d. |||r|, +|
|+c|ed.
||er|u+|ed |||c|opo|ouo|
Ere||u oppo||uu|||c |u|ec||ou
Aoc|+|ed W||| ueu||opeu|+
||er|u+||ou occu| |o ||u (e|,||er+|ou o|
pu|pu||c |eude| p+pu|e), |uu, ||due,, +ud
p|eeu.
||||e|eu||+| d|+uo|. d|er|u+|ed c+ud|d|+|
Maoaemeot: |op|c+| +ud/o| ,|er|c +/o|e
IRICHD5PDRDN INFCII0NS
'upe|||c|+| |uu+| |u|ec||ou o| ||e ||+|ur co|
ueur
to|oy: ||c-c ( ||c|c o| ||c-c-|
|,:- ) +--:|
|er+||+ceou o| p|reu|ed |uuu
pdemoo|oy
\o|e corrou|, |u ||op|c+| c||r+|e.
|||ec| |uocu|+||ou ou|o ||e ||u ||or cou|+c| W|||
dec+,|u .ee|+||ou, Wood, o| o|| eer |o |e
||e |o|r o| +cqu||||ou.
C|oca| Iodos
B|oWu |o ||+c| uouc+|, r+cu|e() W||| We||
de||ued |o|de| (||. 25!3) ||+| |eer||e ||.e|
u|||+|e |+|u
||||||u||ou
|+|r. ||ue+ u||+ p+|r+||
'o|e. ||ue+ u||+ p|+u|+||
0aooss: d||ec| r|c|ocop,, .|u+||/|u +|uud+u|
||+uc||u ep|+|e |,p|+e.
Maoaemeot: |op|c+| +/o|e.
IINA NICkA |C|0 . B!o.
'u|cu|+ueou r,coe
Cu|+ueou +ud u|cu|+ueou |u|ec||ou |o||oW
|u |oc+| |uocu|+||ou
k|| |+c|o|
0ccu| |u o||e|W|e |e+|||, |ud|.|du+|
|u ||e e|||u o| |rruuocorp|or|e, c+u
d|er|u+|e ,|er|c+||,
I,pe
\,ce|or+
C||oror,co|
'po|o|||c|o|
ner+|oeuou|, d|er|u+|ed |u|ec||ou |o ||u.
\+u, |ud|.|du+| |+.e uude||,|u |rruuocor
p|or|e.
'ou|ce o| cu|+ueou |uocu|+||ou
|||r+|, pu|rou+|, |u|ec||ou (C|,p|ococco|,
||+|or,co|, |||op|+ro|, cocc|d|o|dor,
co|, peu|c||||uo|)
C| ||+c|- Cc!!c pp
|u||+.+cu|+| c+||e|e|- Cc!!c pp
INvASIv AN0 0ISSMINAI0 FNCAI INFCII0NS
A |e|e|oeueou |oup o| |uu+| |u|ec||ou ||+|
de.e|op +| ||e ||e o| ||+ucu|+ueou ||+ur+.
|u|ec||ou |oW|, e.o|.e + ||e e||o|o|c +eu|
u|.|.e +ud +d+p| |o ||e +d.e|e |o| ||ue
eu.||oureu|.
||+uo|
C||u|c+| ||ud|u
n||op+||o|o,
|uu+| cu||u|e. |o|+||ou o| ||e p+||oeu
I,pe
\,ce|or+
C||oror,co|
'po|o|||c|o|
S8CIANN0S MC0SS |C|0 . B!o.
FICk 25-38 Ioea ora uu||o|r|, |+u r+cu|e ou ||e p|+u|+| |oo|, p|eeu| |o| e.e|+| ,e+|. K0n p|ep+
|+||ou |oWed |,p|+e.
A c||ou|c uppu|+||.e |u|ec||ou o|||u+||u |u
de|r| +ud u|cu||, e\|eud|u |u|o cou||uou
||ue (|+c|+, |oue).
C|+|+c|e||/ed |, ||e p|eeuce o| |+|u, W||c| +|e
|||||, c|urped co|ou|e o| ||e c+u+||.e +eu|.
C||u|c+| ||ud|u
'||u ||ud|u
|+|u|e We|||u
wood, |udu|+||ou
'|uu ||+c| ||+| d|c|+|e pu |u|e|r|||eu||,
',|er|c ,rp|or-do uo| de.e|op
||er|u+||ou |o d||+u| ||e doe uo| occu|.
\+||+u| +ud e||o|o|c +eu|
Bo||,or,co| (c+ued |, |+c|e||+)
Ac||uor,ce|or+ (c+ued |, Ac||uor,ce|+|e
o|+u|r)
Eur,ce|or+ (c+ued |, ||ue |uu|)
',, \+du|+ |oo|, r+du|or,ce|or+
*
|epeud|u ou eo|+p|,.
MCI0MA |C|9 . 1.+
|C|0 . B+1.0
*
Ae oI 0oset 20-50 yeais
Sex 90% of patients aie males
0emoraphy
Ruial inhabitants
Agiicultuial woikeis and laboieis exposed to
soil
Tiopical and subtiopical iegions.
Iraosmssoo
Cutaneous inoculation of oiganism
Thoin piick
Wood splintei
Stone cut
Contaminated with soil oi plant debiis
Into foot oi hand
to|oy aod pdemo|oy
The piedominant agent vaiies with the local-
ity.
Fungi isolated fiom soil except tnomytes
srae|.
Tiopical and subtiopical climate suppoits
giowth of oiganisms.
In Cential/South Ameiica, 90% of cases
caused by NotarJa |ras|enss.
In Afiica, M. myteomas is common cause.
Most commonly seen in India, Mexico,
Nigeiia, Saudi Aiabia, Senegal, Somalia, Su-
dan, Venezuela, Yemen, Zaiie.
Causative agent foims dense colonies, i.e.,
giains.
ksk Factors Pooi hygiene, walking baie-
foot, neciotic injuied tissue, diminished
nutiition.
FAIh0CNSIS
Pathogens live in soil and entei thiough
bieaks in the skin.
Only oiganisms that can suivive at body tem-
peiatuie can pioduce mycetoma.
Infection begins in skin and subcutaneous tis-
sues, extending into fascial planes, destioying
contiguous tissues.
Iocubatoo Ferod Lesion occuis at inocula-
tion site weeks to yeais aftei tiauma.
0uratoo oI Iesoo Lesions may continue to
expand foi decades.
Symptoms Relatively few, with little pain, ten-
deiness, oi fevei.
Sko Iesoos
Ioocu|atoo Ste
Papule/nodule at inoculation site (Fig.
25-39).
Swelling incieases slowly.
Epideimis ulceiates and pus-containing gian-
ules (giains) diain.
Cranu|es (Fig. 25-40) aie miciobial colonies,
small (<1-5 mm).
Palpation: Usually not tendei; pus diains on
piessuie.
Distiibution:
Unilateial on the leg, foot, hand.
Uncommonly: toiso, aim, head, thigh, but-
tock.
Chrooc Iesoos
Fistulae foim with diaining pus (Fig. 25-41).

FICk 25-39 Actoomycetoma A 2!,e+|o|d
n|p+u|c |er+|e ||or Ceu||+| Are||c+ W||| + p+|u|u|
|e|ou |o| o rou||. Cou||ueu| e|,||er+|ou .|o|+ceou
uodu|e ou ||e |||| p|ep+|e||+| +|e+ +|||u |u +u o|d
c+|. |e|ou+| ||op, de|ec|ed |:c!c. |:c!c |c|
- W+ |o|+|ed ou cu||u|e o| ||op, pec|reu. I|e
|e|ou |eo|.ed W||| |||re||op||ru||+re||o\+/o|e.
FICk 25-40 Mycetoma raou|es w|||e |+u
u|e (+|||oW) |u d|+|u+e ||or \k'A |o||,or,co|.
(ee ||. !1.)
II0I0C AN0 CIASSIFICAII0N 0F MCI0MA-IIk CIINICAI FkSNIAII0N
WIIh CkAIN F0kMAII0N
Type oI Nycetoma t|o|og|c Ageots
8otryomycoss C+ued \o| corrou. '|c|,|::: c-
|, ||ue |+c|e||+. A|o. '. -!-! | c-c |:|-:|c
|o| + ||ue r,ce|or+. :| 3c:|-!- pp. ||- pp., '|-|::: pp.
Actoomycetoma (actoomycotc 1:|,:- c+ue r,ce|or+ +ud +c||uor,co|
r,ce|or+) (ce|.|c+|, ||o|+c|c, +|dor|u+|)
C+ued |, Ac||uor,ce|+|e o|+u|r |:c!c c+ue r,ce|or+, |,rp|ocu|+ueou |u|ec||ou
(po|o|||c|o|d p+||e|u), upe|||c|+| ||u |u|ec||ou,
d|er|u+|ed |u|ec||ou W||| ||u |u.o|.ereu|
1:|c!c
'|-|,:-
umycetoma (eumycotc mycetoma) \o| corrou. |-!c||-:|-c |,!
C+ued |, ||ue |uu| !c!-||c -c ! ,:-|c|
1| ||c||c -c-|- |,-:|c-|c -
|-||c-c --c|- C.|cc |c|c |-|-|!c
c|| 1-|| !|c o| ||c. 1:- pp.,
|c pp., C,|!:c pp., !: c!
Chrooc 0raoo Subcutaoeous IoI|ammatory
Mass(es ) Osteomyelitis, botiyomycosis, chio-
moblastomycosis, blastomycosis, bacteiial pyo-
deima, foieign-body gianuloma.
Smear oI Fus Irom Iesoo Craou|es Medlai
bodies (Table 25-3) visualized on KOH piepa-
iation as miciobial colonies (see below).
0ermatopatho|oy Pseudoepitheliomatous
hypeiplasia of epideimis. Giains aie found
in puiulent foci suiiounded by fibiosis and
mononucleai cell inflammatoiy cell iesponse.
Cu|ture Isolate oiganism. Bacteiial supeiin-
fection common.
Imao CT scan and echosonogiaphy define
the extent of involvement. X-iay of bone shows
multiple osteolytic lesions (cavities), peiiosteal
new bone foimation.
IA8I 25-3 Co|or Crains in Nycetoma and
Associated 0ranisms
0o|or oI 6ra|o 0rgao|sm
B|+c| !c!-||c ,:-|c|
! -c
|-||c-c --c|-
w|||e |-!c||-:|-c |,!
1:-
|:c!c |c|-
| c|-!-
w|||e |o ,e||oW | :c.c-
||u|, W|||e, 1:|,:- c-|
|o c|e+r 1:|c!c c!c-
ked 1 -||-|-
Infection spieads to deepei tissues, into fascia,
muscle, bone.
Tissue becomes gieatly distoited.
Old mycetoma chaiacteiized by healed scais
and diaining sinuses.
Cential cleaiing gives oldei lesions an annulai
shape.
Ceoera| Fodos
Fevei with secondaiy bacteiial supeiinfec-
tion.
Regional lymphadenopathy occasionally.
Osteomyelitis of contiguous bone may occui.
0IACN0SIS
Clinical suspicion confiimed by:
Demonstiation of giains in pus and/oi
Visualization of Medlai bodies-globulai
colonies of infecting oiganims that appeai as
gianules oi giains in pus oi lesional biopsy
specimen
White
Black
Giay
Isolation of oiganism on cultuie.
C0kS AN0 Fk0CN0SIS
Mycetoma iuns a ielentless couise ovei many
yeais, destioying contiguous fascia and bone.
Actinomycetoma usually enlaiges moie iap-
idly than eumycetoma.
Bacteiial supeiinfections occui.
Relapse aftei antifungal oi antibiotic theiapy
common.
MANACMNI
Individuals aie advised to seek medical atten-
tion eaily.
Surery Smallei lesions can be cuied by
suigical excision. Moie extensive lesions of-
ten iecui aftei incomplete excision. Bulk
ieduction suigeiy is peifoimed; amputa-
tion/disaiticulation avoided. Causative agent
identified, and effective antimiciobial agent
given.
Systemc Aotmcroba| Iherapy Usually con-
tinued foi 10 months.
Bvtryvmycvses Antimiciobial agents accoid-
ing to sensitivities of isolated oiganism. May
be MRSA.
ActInvmycvtIc Mycetvmu May iespond to
piolonged chemotheiapy of stieptomycin com-
bined with eithei dapsone oi tiimethopiim-sul-
famethoxazole.
Eumycetvmu Raiely iesponds to chemothei-
apy. Some cases caused by M. myteomas may
iespond to ketoconazole oi itiaconazole.
FICk 25-41 umycotc mycetoma I|e |oo|, +u||e, +ud |e +|e |o|, d||o||ed W||| eder+ +ud cou||u
eu| u|cu|+ueou uodu|e, c+u||||oWe||||e |uro|, +ud u|ce|+||ou.
C||ou|c |oc+||/ed |u.+|.e |uu+| |u|ec||ou o| ||u
+ud u|cu|+ueou ||ue
E||o|o,. p|reu|ed (der+||+ceou o| d+||
W+||ed) |uu|.
C||u|c+| ||ud|u. .e||ucou p|+que uu+||, occu|
||u ou ||e |e o| |oo|.
',, C||oro||+|or,co|. 0ue o| ||e p|+eo
|,p|or,coe.
*
|epeud|u ou eo|+p|,.
Chk0M0MC0SIS |C|9 . 1.2
|C|0 . B+!.0
*
to|oy Dematiaceous fungi, chaiacteiized
by melanin in mycelial walls.
Fonsetaea eJroso (most commonly)
Also F. tomata, P|a|o|ora errutosa,
C|aJosorum tarron, R|not|aJe||a aq-
uasersa, Boryomytes taesosus.
ksk Croups
Living in iuial aieas in tiopical and subtiopi-
cal iegions and exposed to soil while baiefoot.
Agiicultuial woikeis
Mineis
May piogiess moie iapidly in the immuno-
compiomised host.
Iraosmssoo
Cutaneous inoculation.
Autoinoculation to othei sites may occui.
Tiansmission to othei individuals does not
occui.
0emoraphy Fungi isolated fiom soil and veg-
etation, piefeiiing iegions with >2.5 m (> 100
in.) of iainfall pei yeai and mean tempeiatuies
fiom 12-24C.
0uratoo oI Iesoo Lesions may continue to
expand foi decades.
Symptoms Relatively few
Little pain, tendeiness, oi fevei
Usually piesent with
Bacteiial supeiinfection
Cosmetic disfiguiement
Lymphedema.
Sko Iesoos
Inoculation site
Single scaling nodule at site of tiaumatic
implantation (Fig. 25-42).
Chionic lesions (months to yeais)
New ciops of nodules appeai.
Expanding veiiucous plaques with cential
cleaiing and islands of noimal skin between
veiiucous macules.
Laige cauliflowei-like lesions often foim,
which, in some cases, may become pedun-
culated.
Suiface of veiiucous lesion: pustules, small
ulceiations, black dots" of hemopuiulent
mateiial; fiiable gianulation tissue that
bleeds easily is common.
Extension occuis via
Lymphatic spiead
Autoinoculation
10-20 cm in diametei, enveloping calf oi
foot.
Lymphedema of involved extiemity (el-
ephantiasis)
Aiiangement: Smallei lesions coalesce to
foim laige veiiucous masses. Cential cleai-
ing gives oldei lesions an annulai shape.
Distiibution
Unilateial on the leg, foot
Also, hand, thoiax
Iare verrucous F|aques Blastomycosis, tubei-
culosis veiiucosa cutis, mycetoma, spoiotiicho-
sis, nontubeiculous Myto|aterum infection,
lepiomatous lepiosy, foieign-body gianuloma,
pyodeima gangienosum, squamous cell caici-
noma.

Smear oI Fus Irom Iesoo
Medlai bodies (see below) visualized on 10-
20% KOH piepaiation as black dots.
Hyphal foims can be seen in ciusts, pus, exu-
date.
0ermatopatho|oy
Waity gianuloma: pseudoepitheliomatous
hypeiplasia, hypeikeiatosis, intiaepideimal
abscesses containing inflammatoiy cells and
Medlai bodies.
Medlai bodies
Also known as scleiotic bodies, coppei
pennies"
Small biown fungal foims, which aie iound
with thick bilaminate walls
4-6 m in diametei
Occui singly oi in clusteis
All etiologic agents appeai identical in tissue.
Oldei lesions show dense fibiosis in and
aiound gianulomas.
Cu|ture Oiganism in Sabouiaud glucose agai
shows velvety gieen to black, iestiicted, slow-
giowing colonies. Agents giow veiy slowly,
iequiiing 4-6 weeks foi identification.
0IACN0SIS
Clinical suspicion confiimed by
Visualization of Medlai bodies on
Smeai of pus
Lesional biopsy specimen
Isolation of oiganism on cultuie.
C0kS AN0 Fk0CN0SIS
Bacteiial supeiinfections.
Recuiience aftei oial tiiazole theiapy com-
mon.
Late complication is squamous cell caicinoma
aiising within veiiucous aiea.
MANACMNI 0F Chk0M0MC0SIS
Adjuoctve Iherapy Application of heat may
be helpful in that lesions aiise at coolei acial
sites.
Surery Smallei lesions can be cuied by suigi-
cal excision.
Fareotera| Aotmcroba| Chemotherapy m-
|oertn B : not often effective at usual dosing.
0ra| AotIuoa| Aeots Tieatment is usually
continued foi at least 1 yeai. The iesponse is
highly vaiiable.
Ter|na[ne, 250 mg/d
Iratona:o|e, 200-600 mg/d
Keotona:o|e , 400-800 mg/d
FICk 25-42 Chromomycoss n,pe||e|+|o||c +ud c|u|ed p|+que W||| o|d c+| ou ||e |e |+d |eeu p|e
eu| |o| e.e|+| dec+de.
E||o|o|c Aeu|. '|| :|-:|
|u|ec||ou occu| |o||oW|u +cc|deu|+| |uocu|+||ou
o| ||u
C||u|c+| ||ud|u
|odu|e +| ||e |uocu|+||ou ||e, u|ce|+|e, .e|
|ucou
C||ou|c uodu|+| |,rp|+u|||
'u|cu|+ueou We|||u
|u ||e |rruuocorp|or|ed |o|, d|er|u+|ed
|u|ec||ou c+u occu| ||or ||u o| pu|rou+|, |u|ec
||ou.
',, koe||o|u o| |oe+|deue|' d|e+e
SF0k0IkICh0SIS |C|9 . 1.
|C|0 . B+2.0
to|oc Aeot Soro|rx st|ent|
Dimoiphic fungus
Tissue foim is an oval, cigai-shaped yeast.
Lives as a sapiophyte on plants
Woildwide
Sex Males > females, especially disseminated
disease.
0ccupatoo Occupation exposuie impoitant:
Gaideneis
Faimeis
Floiists
Lawn laboieis
Agiicultuial woikeis
Foiestiy woikeis
Papei manufactuieis
Gold mineis
Laboiatoiy woikeis
In Uiuguay, 80% of cases occui aftei a sciatch
by an aimadillo.
Iraosmssoo
Cutaneous local spoiotiichosis
Commonly, subcutaneous inoculation oi
thiough small abiasion:
Contaminated shaip object (iose oi bai-
beiiy thoin, baib, wood splintei)
Sphagnum moss, stiaw, maish hay, soils.
Raiely, inhalation, aspiiation, oi ingestion
causes systemic infection.
Zoonosis:
Raiely tiansmitted fiom cats with spoio-
tiichosis to humans.
Aimadillos
Lung
Inhalation of spoies
pdemo|oy
Ubiquitous, woildwide.
Moie common in tempeiate, tiopical zones.
Most cases isolated.
Epidemics have occuiied: exposuie to cats
with spoiotiichosis; South Afiican gold min-
eis.
ksk Factors Cutunevus (Lvcu|Ized) 1n]ectIvn
Diabetes mellitus, alcoholism.
DIssemInuted DIseuse HIV infection, hema-
tologic and lymphopiolifeiative disease, immu-
nosuppiessive theiapy.
FAIh0CNSIS
Aftei subcutaneous inoculation, S. st|ent|
giows locally.
Infection can be limited to the site of inocula-
tion ( |aque sorort|oss ).
Infection can extend along the pioximal lym-
phatic channels ( |ym|angt sorort|oss ).
Othei infections have similai lymphatic
involvement, the pattein being iefeiied to
as sorort|oJ oi iesembling |ym|at
sorort|oss. )
Spiead beyond an extiemity is iaie; hematog-
enous dissemination fiom the skin iemains
unpioven.
The poital foi extiacutaneous spoiotiichosis
(e.g., osteoaiticulai) is unknown but is piob-
ably the lung.
Iocubatoo Ferod 3 weeks (iange, 3 days to 12
weeks) aftei tiauma oi injuiy to site of lesion.
Lesions aie ielatively asymptomatic, painless.
Afebiile.

Sko Iesoos
Fxed Cutaoeous (F|aque) Sporotrchoss In-
fection at inoculation site only; no lymphangiitis.
Subcutaneous papule, pustule, oi nodule ap-
peais at inoculation site seveial weeks aftei
inoculation. Suiiounding skin is pink to pui-
plish. In time, skin becomes fixed to deepei
tissues.
Veiiucous plaque.
Spoiotiichoid chancie; induiated ulcei may
occui. Boidei iagged and not shaiply demai-
cated.
Diaining lymph nodes become inflamed and
enlaiged.
Ciusted ulceis, ecthymatous, veiiucous
plaques, pyodeima gangienosum-like, infil-
tiated papules and plaques may also occui.
Dsr|uon.
Piimaiy lesion most common on doisum
of hand oi fingei.
Fixed plaque: face in childien; uppei ex-
tiemities in adults.
Iymphaotc Sporotrchoss
Follows lymphatic extension of local cutane-
ous type (Figs. 25-43, 25-44).
Pioximal to local cutaneous lesion.
Red nodules foim in inteivening lymphatics;
may become induiated, nodulai, thickened.
FICk 25-43 Sporotrchoss: acute |ymphaotc type A 13,e+|o|d +|deue| W||| |eude| uodu|e ou
|+ud +ud +|r |o| + Wee|. E|,||er+|ou uodu|e |u + ||ue+| +||+, |u |,rp|+||c c|+uue| ou ||e do|ur o| ||e
|+ud +ud |o|e+|r. ' :|-:| W+ |o|+|ed ou cu||u|e o| + |e|ou+| ||op, pec|reu.
FICk 25-44 Sporotrchoss: chrooc |ymphaotc type Au e|,||er+|ou p+pu|e +| ||e ||e o| |uocu|+
||ou ou ||e |ude\ ||ue| W||| + ||ue+| +||+uereu| o| e|,||er+|ou de|r+| +ud u|cu|+ueou uodu|e e\|eud|u
p|o\|r+||, |u |,rp|+||c .ee| o| ||e do|ur o| ||e |+ud +ud +|r.
Dsr|uon: Inoculation nodule on hand/fingei
with chionic nodulai lymphangiitis up aim.
0ssemoated Sporotrchoss
Fungus disseminates hematogenously to skin,
as well as joints, eyes, and meninges.
Cutaneous lesions
Ciusted nodules, ulceis. May become con-
fluent
Widespiead
Dsr|uon: widespiead lesions, usually spai-
ing palms, soles
Ceoera| examoatoo
Lungs Pulmonaiy spo iotiichosis piesents as a
single cavitaiy uppei-lobe lesion; fibiosis.
JvInts Swelling, painful joint(s) (hand, elbow,
ankle, knee), often in the absence of skin lesion.
Diaining sinuses may occui ovei joints, buisae.
Hemutvgenvus dIssemInutIvn This iesults in
skin, bone, muscle, joint, visceial, CNS (chionic
meningitis) lesions.
F|aque Sporotrchoss Cutaneous tubeiculo-
sis, nontubeiculous mycobacteiial infection,
tulaiemia, cat-sciatch disease, piimaiy syphilis,
bacteiial pyodeima, foieign-body gianuloma,
inflammatoiy deimatophytoses, blastomycosis,
chiomomycosis, mycetoma, leishmaniasis.
Chrooc Nodu|ar Iymphaotc Sporotrchoss
Other 1n]ectIng Agents Myto|aterum mar-
num, NotarJa |ras|enss, Les|mana |ras|-
enss, Frantse||a u|arenss.
Iouch Freparatoo In disseminated spoio-
tiichosis (usually with advanced HIV disease),
KOH solution added to smeai fiom lesional
skin biopsy specimen helps visualize multiple
yeast foims.
Cram Stao In disseminated spoiotiichosis
(usually with advanced HIV disease), smeai
fiom ciusted lesion shows multiple yeast
foims.
0ermatopatho|oy
Gianulomatous, Langeihans-type giant cells,
pyogenic micioabscesses.
1
Kauffman CA et al: Clin Infect Dis 30:684, 2000.
1
Kauffman CA et al: Clin Infect Dis 30:684, 2000.
Oiganisms usually iaie, difficult to visualize.
In the immunocompiomised host, yeast ap-
peai as myiiads of 1- to 3-m by 3- to 10-m
cigai shaped foims.
Cu|ture Oiganism usually isolated within a
few days fiom lesional biopsy specimen.
0IACN0SIS
Clinical suspicion and isolation of oiganism
on cultuie.
C0kS AN0 Fk0CN0SIS
Shows little tendency to iesolve spontane-
ously.
Responds well to theiapy, but a significant
peicentage ielapse aftei completion of thei-
apy.
Disseminated infection in HIV-infected in-
dividuals iesponds pooily to all foims of
theiapy.
MANACMNI
1
0ra| AotIuoa| Aeots
Iratona:o|e : 200-600 mg/d. Veiy effective foi
lymphocutaneous infection; not as effective
foi bone/joint and pulmonaiy infection.
F|utona:o|e : 200-400 mg/d iepoited to be ef-
fective.
Keotona:o|e : 400-800 mg/d iepoited to be
effective.
Ter|na[ne : 1000 mg/d iepoited to be effec-
tive.
SauraeJ so|uon o[ oassum oJJe : 4.5-9
mL/d foi adults effective foi lymphocutane-
ous infection; less effective than oial antifun-
gal agents. Adveise events: GI distuibance,
acneifoim iash.
Iotraveoous Iherapy m|oertn B : Foi
those with meningeal, pulmonaiy, oi dissemi-
nated infection oi who aie unable to toleiate
oial theiapy foi lymphocutaneous disease.
',|er|c |uu+| |u|ec||ou W||| cu|+ueou d|
er|u+||ou occu| ro| o||eu |u ||e |rruuocor
p|or|ed |o|.
|||r+|, |uu |u|ec||ou, c+u d|er|u+|e |er+|o
euou|, |o ru|||p|e o|+u ,|er, |uc|ud|u ||e
||u.
C|,p|ococco|
n||op|+ro|
|o||| Are||c+u ||+|or,co|
Cocc|d|o|dor,co|
|eu|c||||uo|
||er|u+||ou ||or C| ||+c| o| |u||+.+cu|+| c+||
e|e|, ueu||opeu|c |o|.
C+ud|der|+ +ud d|er|u+|ed c+ud|d|+|
SSIMIC FNCAI INFCII0NS WIIh 0ISSMINAII0N I0 SkIN
Cryotottus neo[ormans, a yeast, seiotypes A,
B, C, D causing infection in humans
Seiotypes A and D designated C. neo[or-
mans vai neo[ormans
Seiotypes B and C, C. neo[ormans vai ga
In tissue, encapsulated yeastlike fungi (3.5 to
7.0 m in diametei); bud connected to paient
cell by naiiow poie; capsule thickness vaiiable
Found in soil and diied biid dioppings.
C. neo[ormans vai. ga causes localized
infections (ciyptococcomas) in tiopical
climates; associated with eucalyptus plants.
Polysacchaiide capsule is majoi viiulence fac-
toi; basis foi antigen testing.
Ae oI 0oset Moie common ovei the age of
40 yeais.
Sex Males > females 3:1.
Iocdeoce
Globally, ciyptococcosis (usually meningitis)
is the most common invasive mycosis in HIV/
AIDS, occuiiing in 6 to 9% of peisons with
advanced untieated HIV/AIDS in the United
States and 20 to 30% in Afiica.
Incidence is also high in Euiope and South
Ameiica.
In the industiialized nations, the incidence is
much less due to immune ieconstitution.
C|,p|ococco| | + ,|er|c r,co|

|||r+|, pu|rou+|, |u|ec||ou
0cc+|ou+| |er+|oeuou d|er|u+||ou |o re
u|ue +ud ||u
||er|u+||ou occu| |u +d.+uced n|\/A||'.
',,. Io|u|o|
0ISSMINAI0 CkFI0C0CC0SIS |C|9 . 1.5
|C|0 . B+5.0
In untieated HIV/AIDS, cutaneous dissemi-
nation occuis in 10 to 15% of ciyptococcosis
cases.
ksk Factors HIV/AIDS, solid-oigan tians-
plantation, glucocoiticoid theiapy, saicoidosis,
lymphoma, diabetes mellitus.
0emoraphy Woildwide, ubiquitous. Distii-
bution of seiotype vaiies in geogiaphic aieas.
FAIh0CNSIS
C. neo[ormans inhaled in dust.
Causes a piimaiy pulmonaiy focus of infec-
tion that may iemain localized oi dissemi-
nate. Tends to iesolve spontaneously.
Piogiessive lung disease and dissemination
occuis: in advanced untieated HIV/AIDS, he-
matologic malignancy, glucocoiticoid theiapy.
Reactivation of latent infection in the im-
munocompiomised host may iesult in hema-
togenous dissemination to meninges, kidneys,
and skin; 10 to 15% of patients have skin
lesions.
hstory
Occuis in the setting of advanced HIV/AIDS
Cutaneous lesions: usually asymptomatic
CNS: headache most common symptom
(80%), mental confusion, impaiied vision foi
2 to 3 months
Lungs: pulmonaiy symptoms uncommon
Sko Iesoos
Papule(s) oi nodule(s): with suiiounding
eiythema that occasionally bieak down and
exude a liquid, mucinous mateiial. Can pies-
ent as a solitaiy nodule in otheiwise healthy
individuals.
Molluscum contagiosum-like lesions occui in
HIV/AIDS-infected patients (Fig. 25-45).
Acneifoim
Ciyptococcal cellulitis: mimics bacteiial cel-
lulitis, i.e., ied, hot, tendei, edematous plaque
on extiemity; possibly multiple noncontigu-
ous sites.
In HIV/AIDS, lesions occui most commonly
on face/scalp.
0ra| Mucosa Occui in <5% of patients, pie-
senting as nodules/ulceis.
Ceoera| Fodos
Meningoencephalitis
In HIV/AIDS, widespiead with fungemia and
infection of meninges, lungs, bone maiiow,
genitouiinaiy tiact including piostate, and
skin. Hepatomegaly and splenomegaly.
Wdespread Fapu|ar ruptoo o Immuoocom-
promsed Fateot Molluscum contagiosum,
disseminated histoplasmosis acne, saicoidosis,
pyodeima.
0ermatopatho|oy Gelatinous ieactions show
numeious oiganisms in aggiegates with little
inflammatoiy iesponse. Gianulomatous ieac-
tions show tissue ieaction with histiocytes, gi-
ant cells, lymphoid cells, and fibioblasts, aieas
of neciosis; oiganisms aie piesent in smallei
numbeis. Capsules stain with mucicaimine
stain, diffeientiating C. neo[ormans fiom B.
JermaJs.
Iouch Freparatoo Lesional skin biopsy speci-
men oi sciapings fiom skin lesion smeaied on
micioscope slide examined with KOH to iden-
tify C. neo[ormans.
CSF With meningitis, encapsulated budding
yeast is seen with India ink piepaiations in
40 to 60% of cases, lymphocytic pleocytosis,
elevated piotein, decieased glucose. Intiacia-
nial piessuie may be modeiately to extiemely
elevated.
Imao X-iay findings of chest vaiiable
Cu|ture CSF. Lesional skin biopsy specimen.
In HIV/AIDS, ciyptococcosis tends to be wide-
spiead, with cultuies positive in blood, sputum,
bone maiiow, and uiine. If C. neo[ormans iso-
lated fiom lesional skin biopsy specimen, extent
of disease should be deteimined by examina-
tion of CSF, bone maiiow, sputum, uiine, and
piostate fluid.
Cryptococca| Aoteos Sensitive and specific.
Detect in CSF, seium, uiine. Useful in follow-
ing iesponse to theiapy and in foimulating
piognosis.
0IACN0SIS
Confiimed by skin biopsy and fungal cultuies.
C0kS AN0 Fk0CN0SIS
In HIV/AIDS in the absence of immune ie-
constitution, ciyptococcal meningitis ielapses
in 30% of cases aftei amphoteiicin B theiapy;
lifelong secondaiy piophylaxis with fluconazole
ieduces ielapse iate to 4 to 8%.
MANACMNI
Frmary Frophy|axs In some centeis, flucon-
azole is given to HIV/AIDS-infected individuals
with low CD4 cell counts; the incidence of
disseminated infection is ieduced, but theie is
no effect on the moitality iate.
Iherapy oI Meoots Amphoteiicin B foi
2 to 4 weeks in uncomplicated cases and foi
6 weeks in complicated cases. Fluconazole (al-
teinative).
IoIectoo Imted to Sko Fluconazole, 400-600
mg/d. Itiaconazole (alteinative), 400 mg/d.
Secoodary Frophy|axs In HIV/AIDS-disease
(without immune ieconstitution), lifelong
secondaiy piophylaxis is given. Fluconazole,
200-400 mg/d; itiaconazole (alteinative),
200-400 mg/d.
E||o|o|c +eu|. |||cc :c|c|
Euder|c +|e+ |u uu||ed '|+|e |u 0||o/\||
|pp| ||.e| .+||e,
Cu|+ueou |e|ou |u |||op|+ro|
Acu|e |||op|+ro|. |,pe|eu|||.||, |e+c||ou
E|,||er+ uodour
E|,||er+ ru||||o|re
||o|e|.e d|er|u+|ed |||op|+ro|.
||er|u+|ed p+pu|e, c+||u r+cu|e
',, |+|||u d|e+e, c+.e d|e+e, 0||o
\+||e, d|e+e.
|C|9 . 5.90
|C|0 . B!9
hISI0FIASM0SIS
FICk 25-45 Cryptococcoss:
dssemoated \u|||p|e, ||uco|o|ed
p+pu|e +ud uodu|e ou ||e |+ce |u +u
n|\/A||'|u|ec|ed |ud|.|du+| |ep|eeu|
d|er|u+||ou o| pu|rou+|, c|,p|ococ
co| |er+|oeuou|, |o ||u, reu|u
e +|e +|o + corrou ||e o| |u|ec||ou
+||e| |uuer|+. I|e |e|ou +|e e+||,
r||+|eu |o| ro||ucur cou|+|our,
W||c| occu| corrou|, |u n|\/A||'
d|e+e. (Cou||e, o| |o|c \+||+u|, \|.)
to|oc Aeot
H. tasu|aum vai. tasu|aum , an unencap-
sulated dimoiphic fungus.
Mycelial (micioconidia) foim: natuially in-
fectious foim
Yeast oi tissue foim: Shoitly aftei infect-
ing host, mycelia tiansfoim to yeast, which
aie found within maciophages and othei
phagocytes.
In Afiica, H. tasu|aum vai. Ju|os. Skin/
bone lesions common.
The fungus giows well in soil eniiched with
biid oi bat guano.
pdemo|oy
Repoited thioughout the woild
Endemic aieas chaiacteiized by:
Humidity
Acid soil
Soil eniiched with biid oi bat dioppings
piomotes giowth and spoiulation
Disiuption of soil leads to aeiosolization of
micioconidia
Activities associated with high-level exposuie
Spelunking
Excavation
Cleaning of chicken coops
Demolition/iemodeling of old buildings
Cutting dead tiees
Endemic aieas
Ameiicas:
Noith: Eastein and cential United States,
especially Ohio/Mississippi Rivei valleys;
in some aieas, 80% of iesidents aie histo-
plasmin-positive.
Cential, South
Afiica, equatoiial
Caiibbean Islands
Asia
Ae oI 0oset Foi disseminated infection, veiy
old and veiy young.
Iraosmssoo
Inhalation of micioconidia in soil contami-
nated with biid oi bat dioppings.
Those at iisk: faimeis, constiuction woikeis,
childien, otheis involved in outdooi activities
(cave exploiation).
Acute pulmonaiy histoplasmosis may occui
in outbieaks in individuals with occupational
oi iecieational exposuie.
ksk Factors Ior 0ssemoatoo:

Immuoocompromsed host
HIV/AIDS; CD4 T cell count <200/L; no
effective antiietioviial theiapy (ART)
Post solid oigan tiansplant
Lymphoma, leukemia, chemotheiapy
Advanced age.
Immunosuppiessive diugs: piednisone,
methotiexate, anti-TNF- agents
FAIh0CNSIS
Inhalation of micioconidia iesults in piimaiy
pulmonaiy infection.
Micioconidia tiansfoimed to budding yeast
once engulfed by alveolai maciophages.
Yeast giows within iesting maciophages;
tianspoited to local diaining lymph nodes,
spieading hematogenously thioughout the
ieticuloendothelial system.
Gianulomas foim and contain oiganisms;
fibiose and calcify.
Seveiity and extent of disease depends on
Intensity of exposuie
Immune status of exposed peison
Undeilying pulmonaiy aichitectuie
In HIV disease, can piesent as eithei piimaiy
histoplasmosis oi ieactivation of latent infec-
tion.
Iocubatoo Ferod
Foi acute piimaiy pulmonaiy infection: 5-18
days.
Foi disseminated infection, 2 months.
In seveie foims of infection, piesentation may
be acute, iesembling septicemia with associ-
ated disseminated intiavasculai coagulopa-
thy.
Acute Frmary IoIectoo 90% of patients
asymptomatic. If laige numbeis of spoies
inhaled, influenza-like syndiome may occui
(fevei 38.3C, chills, night sweats, cough, head-
ache, fatigue, myalgia).
0ssemoated IoIectoo
Chionic disease syndiome.
In HIV disease, can piesent as widely dis-
seminated infection with symptoms of sepsis,
adienal insufficiency, diaiiheal illness, oi co-
lonic mass.

Sko Iesoos
Acute Pu|mvnury HIstvp|usmvsIs Cutaneous
lesions iepiesent hypeisensitivity ieactions to
Hso|asma antigen(s):
Eiythema nodosum in 5-10% of peisons with
acute histoplasmosis. See Section 7, page 152.
Eiythema multifoime-like lesions. See Sec-
tion 7.
HIstvp|usmvsIs DIssemInuted tv S|In
Pathogenesis:
Lesions caused by tissue infection.
Histoiically, lesions of mucous membianes
much moie common than those on skin.
HIV/AIDS: 10% of patients with dissemi-
nated histoplasmosis have cutaneous le-
sions.
Renal tiansplant patients: 4-6%.
Lesions
Papules oi nodules; eiythematous, neciotic,
oi hypeikeiatotic (Fig. 25-46)
Eiythematous macules; scaling
FICk 25-46 hstop|asmoss, dssemoated \u|||p|e, e|,||er+|ou, c+||u p+pu|e ou ||e ||uu| +ud
uppe| +|r occu||ed du||u + 2Wee| pe||od +ud r|r|c|ed +cu|e u||+|e po||+| |u +u |ud|.|du+| W||| n|\/A||'.
I|e cu|+ueou |e|ou occu||ed +||e| |e+c||.+||ou o| pu|rou+|, |u|ec||ou +ud |uuer|+. \u|||p|e ,e+||||e | :c
|c| We|e derou||+|ed W||||u r+c|op|+e |u + |e|ou+| ||u ||op, pec|reu. (Cou||e, o| l| |+||ou, \|.)
Folliculitis, pustules, acneifoim
Chionic ulceis
Vegetative plaques
Panniculitis
Eiythiodeima
Diffuse hypeipigmentation with Addison
disease secondaiy to adienal infection.
Mucvus Memhrunes
Oiophaiyngeal
Common site of involvement
Lesions: nodules, vegetations, painful ulcei-
ations of soft palate, oiophaiynx, epiglottis.
Nasal vestibule.
Generu| ErumInutIvn Disseminated disease:
hepatosplenomegaly, lymphadenopathy, men-
ingitis.
0ssemoated 0sease Miliaiy tubeiculosis,
disseminated coccidioidomycosis oi ciyptococ-
cosis, leishmaniasis, lymphoma.
0ermatopatho|oy Identify intiacellulai yeast
foims of H. tasu|aum in tissue by size and
staining. Diffeientiate fiom CottJoJes mm-
s, B|asomytes JermaJs,Pent||um marne[-
[e, Les|mana Jonoan, Toxo|asma gonJ.
Smear H. tasu|aum (intiacellulai yeast) can
be identified by smeais obtained fiom touching
lesional skin biopsy specimen to micioscope
slide (touch piepaiation); also sputum oi bone
maiiow aspiiate, stained with Giemsa stain.
Cu|ture Identify H. tasu|aum fiom biopsy
specimens of skin, oial lesions, bone maiiow,
sputum, lung biopsy specimen, blood, uiine,
lymph node, livei.
Aoteo 0etectoo If positive, must be con-
fiimed by cultuie oi histopathology.
8ooe Marrow Aspratoo H. tasu|aum can

be visualized in those with disseminated infec-
tion.
Imao Chest x-iay: inteistitial infiltiates
and/oi hilai adenopathy (acute).
0IACN0SIS
Clinical suspicion, confiimed by cultuie of
oiganism.
C0kS AN0 Fk0CN0SIS
Piimaiy infection iesolves spontaneously in
most cases.
Untieated chionic cavitaiy pulmonaiy infec-
tion oi piogiessive disseminated foim has a
veiy high moitality iate, 80% of patients dy-
ing within 1 yeai.
Piognosis linked to undeilying condition,
e.g., HIV/AIDS.
Chionic maintenance often iequiied. With
itiaconazole theiapy, cuie iate 80%.
MANACMNI
Freveotoo When any mateiial contaminated
with biid oi bat guano is to be distuibed in
an aiea of endemic histoplasmosis, peisonal
piotective equipment should be used duiing
iecieational oi occupational exposuie.
Systemc Aotmycotc Iherapy Non-life-
thieatening infections and foi those unable to
toleiate amphoteiicin B: Itiaconazole, 400 mg
twice daily PO foi 12 weeks; oi fluconazole,
800 mg/d PO foi 12 weeks. Life-thieaten-
ing and meningeal infection: Amphoteiicin B
given IV.
Secoodary Frophy|axs In HIV disease without
immune iestoiation, itiaconazole, 200 mg/d, oi
fluconazole, 400 mg/d foi life.
E||o|o|c +eu|. 3|c|,:- !-c||!
Euder|c |u ou||e+|e|u +ud C|e+| |+|e +|e+ o|
uu||ed '|+|e
|||r+|, pu|rou+|, |u|ec||ou, W||c| |u ore c+e
| |o||oWed |, |er+|oeuou d|er|u+||ou |o
||u +ud o||e| o|+u
',, . C||c|||| d|e+e, C||c+o d|e+e
8IASI0MC0SIS: CIAN0S MANIFSIAII0NS |C|9 . o.0
|C|0 . B+0.0
to|oc Aeot
B|asomytes JermaJs, a dimoiphic fungus.
In tissue, a yeast 10 m in diametei with 1-
m-thick cell wall: poie of bud is wide.
In cultuie, mycelial phase.
Natuial habitat: Wood debiis. Lakes, iivei,
wetlands subject to flooding.
Zoonosis: causes blastomycosis in dogs.
Ae oI 0oset Young, middle-aged.
Sex Males > females, 10:1.
Iraosmssoo
Most cases aie isolated.
Occupations at iisk:
Outdooi vocation: faim woikeis, manual
laboieis
Outdooi avocation: fishing, hunting, camp-
ing, hiking in aieas of high endemicity.
0emoraphy
Noith Ameiica
United States: Most cases occui in the south-
eastein, cential, and Gieat Lakes aieas.
Canada: Toionto aiea
Raiely occuis in Mexico, Cential Ameiica,
South Ameiica; Afiica (Zimbabwe); Middle
East; India.
FAIh0CNSIS
B. JermaJs infection acquiied fiom inha-
lation of spoies (dust fiom soil, decomposed
vegetation, oi iotting wood).
Asymptomatic piimaiy pulmonaiy infection
usually iesolves spontaneously.
Hematogenous dissemination may occui to
skin, skeletal system, piostate, epididymis, oi
mucosa of nose, mouth, oi laiynx.
Reactivation may occui within lung oi in sites
of dissemination.
Risk factois foi dissemination:

HIV/AIDS
Solid oigan tiansplantation
Iocubatoo Ferod Depends on size of inocu-
lum and immune status. Estimated median, 45
days.
Symptoms
Piimaiy pulmonaiy infection: usually asymp-
tomatic: flulike oi iesembles bacteiial pneu-
monitis.
Chionic pulmonaiy infection: fevei, cough,
night sweats, weight loss.
Cutaneous ulceis often painless.
Sko Iesoos
Frmary Fu|mooary IoIectoo Accompanied
oi followed by hypeisensitivity ieactions: eiy-
thema nodosum, eiythema multifoime. See
Section 7 oi EN and EM.
Frmary Cutaoeous 8|astomycoss Inoculation
site infection, e.g., in laboiatoiy woikeis oi
pathologist.
0ssemoated IoIectoo to Sko aod Mucosa
Initial lesion, inflammatoiy nodule that en-
laiges and ulceiates (Fig. 25-47); subcutane-
ous nodule, many small pustules on suiface.
Subsequently, veiiucous and/oi ciusted
plaque with shaiply demaicated seipiginous
boideis.
Peiipheial boidei extends on one side, iesem-
bling a one-half to thiee-quaitei moon.
Pus exudes when ciust is lifted.
Cential healing with thin geogiaphic atiophic
scai.
Widespiead lesions in HIV/AIDS
Distiibution:
Usually symmetiically on tiunk
Also face, hands, aims, legs
Multiple lesions in one-half of patients

Mucous membianes:
25% of patients have oial oi nasal lesions; one-
half of those have contiguous skin lesions.
Laiyngeal infection.
Ceoera| xamoatoo Lungs Infiltiates, adult
iespiiatoiy distiess syndiome (ARDS), miliaiy
infection, cavitaiy lesions.
Bvnes
50% involvement
Osteomyelitis in thoiacolumbai veitebiae,
pelvis, sacium, skull, iibs, long bones.
May extend to foim laige subcutaneous ab-
scess
May ulceiate
Foim sinus tiacts
Septic aithiitis
verrucous Sko Iesoo Squamous cell caici-
noma, pyodeima gangienosum, tumoi stage of
mycosis fungoides, ecthyma, tubeiculosis vei-
iucosa cutis, actinomycosis, nocaidiosis, myce-
toma, syphilitic gumma, gianuloma inguinale,
lepiosy, biomodeima.
0rect xamoatoo KOH piepaiation of pus oi
iespiiatoiy tiact secietions shows laige (8- to 15-
m), single, budding cells with a thick double-
contouied" wall and a wide poie of attachment.
Sero|oy Specific diagnosis can be made with
antibodies to B. JermaJs antigens.
Cu|ture Of sputum, pus fiom skin lesion oi
biopsy, piostatic secietions.
0ermatopatho|oy Pseudoepitheliomatous
hypeiplasia. Budding yeast with thick walls and
bioad-based buds in micioabscess in deimis
visualized by silvei stain oi PAS stain. Mucicai-
mine stain diffeientiates B. JermaJs fiom
Cryotottus neo[ormans.
Imao Acute (piimaiy) infection shows pneu-
monitis, hilai lymphadenopathy. With chionic
pulmonaiy infection, imaging highly vaiiable.
0IACN0SIS
Clinical suspicion, confiimed by cultuie of oi-
ganism fiom skin biopsy, sputum, pus, uiine.
C0kS AN0 Fk0CN0SIS
Most piimaiy pulmonaiy blastomycosis cases
aie asymptomatic, self-limited.
Cutaneous infection usually occuis months
oi yeais aftei piimaiy pulmonaiy infec-
tion.
Skin most common site of extiapulmonaiy
infection, followed by bones, piostate, and
meninges; iaiely, adienals and livei.
Befoie amphoteiicin B, moitality iate in in-
dividuals with disseminated infection was
80-90%.
Moitality iate: highei with ARDS, HIV/AIDS,
solid oigan tiansplantation.
Cuie iate with itiaconazole, 95%.
MANACMNI
Freveotoo Because of the widespiead extent
of B. JermaJs in endemic iegions, avoidance
is not possible.
Ceoera| Care Patients with mild to modeiate
acute pulmonaiy blastomycosis can often be
followed without antifungal theiapy, especially
if the patient is impioving at time of diagnosis.
Patients with meningitis oi acute iespiiatoiy
distiess syndiome aie best tieated in hospital
with IV amphoteiicin B.
Iotraveoous Amphoterco 8 In life-thieaten-
ing infections: am|oertn B , 120-150 mg/
week with a total dose of 2 g in adults. Lipo-
somal piepaiations aie less toxic. Aftei initial
impiovement, theiapy can be continued on an
outpatient basis, thiee times weekly.
0ra| AotIuoa| Iherapy In those whose infec-
tion is non-life-thieatening and/oi those un-
able to toleiate amphoteiicin B: Itiaconazole,
200-400 mg/d foi >2 months; ketoconazole
(alteinative), 800 mg/d.
E||o|o|c +eu|. C::!!-
Euder|c |o dee|| +|e+ o| ou||We|e|u uu||ed
'|+|e, uo|||e|u \e\|co, Ceu||+| +ud 'ou||
Are||c+
|||r+|, pu|rou+|, |u|ec||ou, ||+| uu+||, |eo|.e
pou|+ueou|,
C+u d|er|u+|e |er+|oeuou|, |eu|||u |u
c||ou|c, p|o|e|.e, |+uu|or+|ou |u|ec||ou |u
||u, |uu, |oue, reu|ue.
Cu|+ueou |e|ou |u cocc|d|o|dor,co|
Acu|e cocc|d|o|dor,co|
Io\|c e|,||er+ (d|||ue e|,||er+, ro||||
|||o|r, u|||c+||+)
E|,||er+ uodour
E|,||er+ ru||||o|re (ee 'ec||ou 1, |o| E|,
E\)
||er|u+|ed |||op|+ro|
|+pu|e, uodu|e, .e||ucou p|+que
',, . '+u lo+qu|u \+||e, |e.e|, .+||e, |e.e|,
dee|| |e.e|
0ISSMINAI0 C0CCI0I0I00MC0SIS |C|9 . +.9
|C|0 . B!3.0
FICk 25-4T North Amercao b|astomycoss: dssemoated u|ce|+|ed, |u||+rr+|o|, p|+que W||| u|
|ouud|u e|,||er+, eder+, +ud ||||o| ou ||e |e |eu|| ||or d|er|u+||ou ||or pu|rou+|, ||+|or,co| .|+
||ood |o ||u. I|e |e|ou ru| |e d|||e|eu||+|ed ||or p,ode|r+ +u|euour. (Cou||e, o| E||/+|e|| \. 'p|e|, \|.)
to|oc Aeots
CottJoJes, a dimoiphic fungus. Two spe-
cies: C. mms and C. osaJas.
On agai media and in soil: filamentous mold;
foim aithioconidia, which become aiiboine.
In susceptible host, aithioconidia enlaige to
become spheiules, which contain endospoies.
kace Blacks, Filipinos.
Sex Risk of dissemination gieatei in males,
piegnant females.
Iocdeoce Gieatly incieased in southein Cali-
foinia duiing the past few yeais. Appioximately
100,000 cases in the United States pei yeai; most
asymptomatic. In endemic aieas: infection iates,
measuied by skin test ieactivity, may be 16-42%
oi highei by eaily adulthood. Occuiied in 25%
of HIV-infected peison in highly endemic ie-
gions such as Aiizona oi Bakeisfield, CA.
Acqustoo
Exposuie to dust fiom soil
Pieviously uncultivated deseit soil.
Aichaeologic excavations of Ameiindian
sites.
Following eaithquakes in endemic aieas.
Inhalation of aithioconidia is followed by
piimaiy pulmonaiy infection.
Raiely, peicutaneous.
0emoraphy
Regions endemic foi CottJoJes include:
Noith Ameiica
Southein Califoinia (San Joaquin Valley)
Southein Aiizona, Utah, New Mexico,
Nevada, southwestein Texas.
Mexico aieas adjacent to Texas
Cential Ameiica
South Ameiica (Colombia, Venezuela,
noitheastein Biazil, Paiaguay, Bolivia,
noith-cential Aigentina).
Piimaiy pulmonaiy coccidioidomycosis oc-
cuis in individuals living in these iegions
(endemic) oi in visitois to the iegions (non-
endemic).
C|assIcatoo Acute self-limited pulmonaiy
coccidioidomycosis. Disseminated coccidioid-
omycosis (cutaneous, osteoaiticulai, meningeal).
FAIh0CNSIS
Spoies (micioconidia) inhaled, iesult-
ing in piimaiy pulmonaiy infection that is
asymptomatic oi accompanied by symptoms

of coiyza.
Spheiules contained by neciotizing gianulo-
mas.
Failuie to develop cell-mediated immunity is
associated with disseminated infection and
ielapse aftei theiapy.
Dissemination outside thoiacic cavitiy occuis
in <1% of infections.
Rick factois foi dissemination
Males
Piegnancy: 2nd oi 3 id tiimestei
Afiican-Ameiican oi Filipino ancestiy
Advanced age
Peisons with depiessed cellulai immunity
HIV/AIDS with CD4 T cell counts <250/
L; without adequate ART.
Chionic glucocoiticoid theiapy
Allogeneic solid-oigan tiansplants
Tieatment with TNF- antagonists
Iocubatoo Ferod 1-4 weeks.
Symptoms
About 40% of peisons infected with Cot-
tJoJes become symptomatic. With piimaiy
pulmonaiy infection, influenza- oi giippe-
like illness with fevei, chills, malaise, anoiexia,
myalgia, pleuiitic chest pain.
With disseminated infection, headache, bone
pain.
In HIV/AIDS, piesents when CD4 cell count
is <200/L: focal pulmonaiy lesions, men-
ingitis, focal disseminated lesions, oi wide-
spiead disease.
Sko Iesoos
Frmary IoIectoo Manifestations of hypei-
sensitivity: toxic eiythema (diffuse eiythema,
moibillifoim, uiticaiia), eiythema nodosum,
eiythema multifoime.
Frmary Cutaoeous Ioocu|atoo Ste (kare)
Nodule eioding to ulcei. May have spoiotii-
choid lymphangitis, iegional lymphadenitis.
hematoeoous 0ssemoatoo to Sko
Initially, papule evolving with foimation of
pustules, plaques, nodules (Fig. 25-48).
Abscess foimation, multiple diaining sinus
tiacts, ulceis; subcutaneous cellulitis; veiiu-
cous plaques; gianulomatous nodules.
Scais.
Distiibution: face (Fig. 25-48), especially na-
solabial fold-piefeiential site; extiemities.

Ceoera| xamoatoo Bvne Osteomyelitis.
Psoas aiea pioduces diaining abscess.
CNS Signs of meningitis.
0ssemoated Fapu|es[Fustu|es Waits, fuiun-
cles, ecthyma, iosacea, lichen simplex chionicus,
piuiigo nodulaiis, blastomycosis, ciyptococco-
sis, tubeiculosis. In HIV-infected patient: may
iesemble folliculitis, molluscum contagiosum.
0ermatopatho|oy Gianulomatous inflam-
mation; spheiules in tissue.
Cu|ture Pus, biopsy specimen giows oiganism
on Sabouiaud`s medium.
0IACN0SIS
Detection of CottJoJes spheiules in sputum,
pus, skin/tissue biopsy specimen. Isolation of
CottJoJes on cultuie.
C0kS AN0 Fk0CN0SIS
About 40% of peisons infected with CottJ-
oJes become symptomatic.
Most infected iesidents of endemic aieas heal
spontaneously.
Meningeal infection difficult to cuie.
The incidence of ielapse of pulmonaiy oi dis-
semiated infection is ielatively high.
In HIV/AIDS without effective ART, moital-
ity iate is high; ielapse iate veiy high.
MANACMNI
Systemc AotIuoa| Iherapy Nvn-LI]e-
ThreutenIng 1n]ectIvn Fluconazole, 200-400
mg/d, oi itiaconazole.
LI]e-ThreutenIng 1n]ectIvn Amphoteiicin B
deoxycholate.
Secoodary Frophy|axs Lifelong theiapy foi
meningeal infection may be iequiied and is
iequiied in HIV disease.
FICk 25-48 Coccdodomycoss: dssemoated u|ce|+|ed +ud c|u|ed uodu|e ou ||e c|ee| +ud uoe o|
+u |ud|.|du+| W||| pu|rou+|, cocc|d|o|dor,co| W||| d|er|u+||ou |o ||e ||u. (Cou||e, o| ||+uc| keuu+, \|.)
E||o|o|c +eu|. Cc!!c c||:c +ud uou c||
:c pec|e.
|uc|deuce. ||||| ro| corrou c+ue o| uooco
r|+| ||ood||e+r |u|ec||ou |u ||e uu||ed '|+|e.
k|| |+c|o| |uc|ude
|eu||opeu|+
|rruuocorp|or|e
|e||o|+||ou o| C| ||+c|
\uco+| d+r+e due |o c,|o|o\|c +eu| ued
|o| c+uce| c|ero||e|+p,
|u||+.euou d|u ue
I|||dde|ee |u|u
n||||| p+||eu|.
uude|o|u |uduc||ou c|ero||e|+p,
Boue r+||oW ||+up|+u|+||ou
||.e| ||+up|+u|+||ou
C+ud|der|+ uu+||, occu| |u |e||||e ueu||opeu|c
p+||eu|.
B|ood cu||u|e +|e po|||.e |u ou|, 50 o| c+e
o| d|er|u+|ed c+ud|d|+|.
|o||+| o| eu||,. |u||+.+cu|+| c+||e|e|, C| ||+c| .
||er|u+|ed c+ud|d|+| r+, eed
'||u, p|eeu||u W||| r+|| d|er|u+|ed e|,
||er+|ou cu|+ueou p+pu|e (||. 2550).
E,e
||.e|, p|eeu
0u |e|ou+| ||u ||op,, Cc!!c ,e+| |o|r +|e
.|u+||/ed |u ||e de|r|.
I|e d|||e|eu||+| d|+uo| |uc|ude !c|c-cc
|o|||cu||||, W||c| occu| ou ||e ||uu| o| |e+|||,
|ud|.|du+|.
C+ud|der|+ |+ ||| +oc|+|ed ro|||d||, +ud
ro||+|||,.
ACI CAN0I0MIA AN0 0ISSMINAI0 CAN0I0IASIS |C|9 . 2.5
|C|0 . B!1
E||o|o|c +eu|. |-:|| c-||- , d|ro|p||c
|uuu
||e+e o| |rruuocorp|or|ed pe|ou (n|\/
A||') ||.|u |u o| ||+.e||u |o 'ou||e+| A|+
|+||oeue|
|||r+|, po||+| o| eu||, | ||e |uu
ner+|o|o|c d|er|u+||ou r+, |o||oW
C||u|c+| r+u||e|+||ou
'|r||+| |o ||oe o| d|er|u+|ed |||op|+ro|
|e.e|, c||||, We||| |o, +uer|+, eue|+||/ed
|,rp|+deuop+||,, +ud |ep+|ore+|,
||er|u+|ed peu|c||||uo|. ||u |e|ou (||.
25+9)
||||ue d|er|u+|ed p+pu|+| |e|ou
||+uo|. 'r+|| ,e+| ce|| r+, |e eeu ou ||
|op+||o|o|c e\+r|u+||ou o| ||ue, |u| de||u|||.e
d|+uo| depeud ou cu||u|e
\+u+ereu|. Arp|o|e||c|u B | ||e ||e+|reu|
o| c|o|ce |o| e.e|e|, ||| p+||eu|. I|oe W||| |e
e.e|e d|e+e o| W|o |+.e |epouded |o +u
|u|||+| cou|e o| +rp|o|e||c|u B r+, |e ||e+|ed
W||| |||+cou+/o|e
0ISSMINAI0 FNICIIIIN0SIS |C|9 . 1.!
FICk 25-49 Feoc||ooss o hIv[AI0S: dssemoated sko |esoos A 21,e+|o|d \|e|u+ree r+|e
W||| +d.+uced uu||e+|ed n|\/A||' p|eeu|ed W||| |e.e|, We||| |o, +ud d|er|u+|ed ur||||c+|ed ||uco|o|ed
p+pu|e. nuud|ed o| ||uco|o|ed p+pu|e o| .+|,|u |/e, r+u, ur||||c+|ed o| W||| ceu||+| e|o|ou +ud c|u|.
(Cou||e, o| no+u \+u \|u|.)
FICk 25-50 Iovasve caoddass wth
caoddema \u|||p|e, e|,||er+|ou p+pu|e
ou ||e |+ud o| + |e||||e p+||eu| W||| |+uu|o
c,|opeu|+ +oc|+|ed W||| ||e+|reu| o| +cu|e
r,e|oeuou |eu|er|+. I|e uu+| ou|ce o| ||e
|u|ec||ou | ||e +||o|u|e||u+| ||+c|. Cc!!c
|:c| W+ |o|+|ed ou ||ood cu||u|e, c+ud|d+|
|o|r We|e eeu ou |e|ou+| ||u ||op,.
T60
S E C I | 0 N 2 6
kICkIISIAI INFCII0NS
Rickettsiae can cause life-thieatening infec-
tions. Oidei of decieasing case-fatality iate:
R. rt|es Rocky Mountain spotted fevei
(RMSF)]; R. rowa:e| (epidemic louse-boine
k|c|e|||+e. r+|| p+||oeu |u |+r||, k|c|e|
||+ce+e (I+||e 2o)
|:|-||c
0-|c
C-||c
|||:|c
C|+rue+||.e cocco|+c||||/|o|| |+c||||, o|||+|e
|oc+||/+||ou/pe|||euce W||||u eu|+|,o||c ce||
I|+ur|||ed |o |ur+u |, +||||opod. ||c|, r||e,
||e+, |oue, r+rr+||+u |ee|.o||, |ur+u +|e
|uc|deu|+| |o|
|u|ec||ou c|+|+c|e||/ed |,.
E\pou|e |o .ec|o| o| +u|r+| |ee|.o||, ||+.e|
|o o| |e|deuce |u euder|c |oc+||ou
C||u|c+| ||ud|u. |e.e|, e\+u||er o| |+c|e uo||e
(||+c| po| o| |+|u) (co|u|||e |e|ou W||| ceu||+|
ec|+| +ud |ed |+|o +| ||e o| .ec|o||eed|u |||e
||e) ( I+||e 2o), .+cu||||, |ep+|op|euore+|,
I||or|oc,|opeu|+, |eu|opeu|+, e|e.+|ed +r|
uo||+u|e|+e, |,pou+||er|+
||+uo|. cou|||red |, p+||ed e|ur +rp|e +|
|e| cou.+|eceuce o| derou||+||ou o| r|c|o|e
|e|r+|op+||o|o,. r|c|o|e ru|||p|, |u eu
do||e||+| ce|| o| r+|| ||ood .ee| +ud p|o
duce .+cu|||| W||| uec|o| +ud |||or|o|,
d|er|u+|ed |u||+.+cu|+| co+u|+||ou (||C) +ud
.+cu|+| occ|u|ou r+, occu|
|o\,c,c||ue | d|u o| c|o|ce
IA8I 26-1 C|assification of Croups of Rickettsia| |nfections and C|inica| |eatures
6ro0ps oI 8|cketts|a| |oIect|oos 00taoeo0s F|od|ogs
I|c| +ud +r+|d r||e-|o|ue E\+u||er | + r+jo| c||u|c+| +ud
po||ed |e.e| |oup ('|C) d|+uo||c |e+|u|e
I|c||o|ue |,p|u I+c|e uo||e, r+cu|op+pu|+| |+|
k|c|e|||+|po\ (r||e|o|ue) I+c|e uo||e, p+pu|o.e|cu|+| |+|
||e+ +ud |oue|o|ue |,p|u I|uu|+| r+cu|op+pu|+| |+|
|oup ||c|e|||+| d|e+e
Ep|der|c |oue|o|ue |,p|u Ceue|+||/ed r+cu|op+pu|+| |+|,
p+||u ||e |+ce, p+|r, +ud o|e,
po|||, |ecor|u pe|ec||+| +ud
cou||ueu|
Euder|c ||e+|o|ue ru||ue |,p|u \+cu|op+pu|+| |+| occu|||u ou
||e e\||er|||e +ud ||uu|, p+||u
||e |+ce, p+|r, +ud o|e, |u !
o| p+||eu|
C||e||o|ue c|u| |,p|u Ec|+| +| ||e o| c||e| |eed|u
( 50 o| c+e), r+cu|op+pu|+|
|+| r+, occu|, |u| e|dor
o|e|.ed
E||||c||o| k+| |u 5 +| oue|
0 |e.e| k+|e
typhus); Orena susugamus| (sciub typhus);
R. tonor Mediteiianean spotted fevei (MSF)];
R. y| (endemic muiine typhus); in iaie cases,
othei spotted fevei gioup oiganisms.
SCII0N 26 k|CKEII'|A| |||ECI|0|' T61
/oouo|
E||o|o,. | :|-||
\ec|o|. .+||ou ||c|
Ceo|+p|,. occu| |||ou|ou| we|e|u ner|
p|e|e (Are||c+)
\o| e.e|e o| ||c|e|||+| |u|ec||ou
C|+|c |||+d. |e.e|, |+|, |||o|, o| ||c| |||e (uo|
corrou)
C||u|c+| ||ud|u. uddeu oue| o| |e.e|, e.e|e
|e+d+c|e, r,+||+, c|+|+c|e||||c +c|+| e\+u||er
p|e+d|u ceu|||pe|+||,
Cou|e. +oc|+|ed W||| |u|||c+u| ro|||d||, +ud
ro||+|||, |+|e
',,. ||+c| re+|e, ||c| |,p|u. \e\|c+u
po||ed |e.e|, o| '||e||e r+uc|+d+ (\e\|co).
Co|or||+u po||ed |e.e| o| 'Io||+ |e.e| (Co|or
||+), B|+/|||+u po||ed |e.e|, o| ''+o |+u|o |e.e|,
o| '|e||e r+cu|o+ (B|+/||)
k0Ck M0NIAIN SF0II0 Fvk |C|9 . 032.0
|C|0 . A11
IA8I 26-2 C|assification of IickBorne Rickettsia| Spotted |evers
8|cketts|a| 6eograph|c 0|sease
gro0p 8|cketts|ae |ocat|oo examp|e
koc|, | :|-|| we|e|u koc|, \ouu|+|u
\ouu|+|u |er|p|e|e po||ed |e.e|
po||ed |e.e| (Are||c+)
I|c| |,p|u
| : \ed||e||+ue+u Bou|ouueue
couu|||e, A|||c+, |e.e|
'ou||e+| A|+, |ud|+
| |:c '||e||+, '||e||+u ||c|
\ouo||+, |,p|u
uo|||e|u C||u+
| c|c| Au||+||+ A||+||+u ||c|
|,p|u
| c:c l+p+u 0||eu|+| po||ed
|e.e|
| c|:c- 'ou|| A|||c+ A|||c+u ||c| |||e
|e.e|
IICk-80kN SF0II0 Fvk (I+||e 2o2) |C|9 . 0oo.
|C|0 . A11
hstory oI kMSF Fiist iecognized in 1896 in
Snake Rivei Valley of Idaho; oiiginally called
black measles" because of chaiacteiistic iash.
Ae Incidence of infection highest in 5- to 9-
yeai-old childien.
to|oy Rt|esa rt|es
Iraosmssoo Infected tick bite; inoculation
thiough abiasions contaminated with tick feces
oi tissue juices. Reseivoiis and vectois: wood
tick Dermatenor anJerson in westein United
States; dog tick D. ara||s (4% infected with
iickettsiae, most often with nonpathogenic
species, i.e., R. monana, R. |e|| ) in eastein
two-thiids of United States and Canada; R|-
te|a|us sanguneus in Mexico; and m||yomma
ta,ennense in Mexico and Cential and South
Ameiica. Patient eithei lives in oi has iecently
visited endemic aiea. Only 60% have knowledge
of a iecent tick bite duiing the 2 weeks befoie
onset of illness. 1-3% of the tick population
caiiies pathogenic R. rt|es , even in aieas
wheie majoiity of human cases iepoited.
Seasoo In Noithein Hemispheie, cases occui
mainly in spiing in noithein aieas. In waimei
southein states, most cases occui fiom Apiil
to end of Septembei. The longest season and
gieatest numbei of wintei-time cases occui
faithei south.
0emoraphy Ameiicas. United States: Okla-
homa, Noith Caiolina, Viiginia, Maiyland,
Geoigia, Michigan, Alaska, Montana, South
Dakota. Raiely occuis in Rocky Mountain ie-
gion. Canada. Mexico. Cential Ameiica (Costa
Rica, Panama). South Ameiica (Colombia, Bia-
zil, Aigentina).
Iocdeoce In the United States, 600 cases of
RMSF aie iepoited to the Centeis foi Disease
Contiol and Pievention (CDC) annually. Actual
incidence is piobably significantly highei. Foui
states (Noith Caiolina, Oklahoma, Tennessee,
South Caiolina) account foi 48% of U.S. cases.
FAIh0CNSIS
Inoculation usually iequiies > 6 h feeding, af-
tei which iickettsia aie ieleased fiom the sali-
vaiy glands. Aftei inoculation into the deimis,
initial local ieplication occuis in endothelial
cells, followed by hematogenous and lymphatic
dissemination. Oiganisms spiead thioughout
body and attach to vasculai endothelial cells,
the piincipal taiget. Infected foci enlaige as
iickettsia spiead fiom cell to cell, foiming a
netwoik of contiguously infected endothelial
cells in miciociiculation of deimis, and to
multiple oigans and tissues. Focal infection
of vasculai smooth muscle causes a geneial-
ized vasculitis. Patients with seveie infection
of biain and lungs have high moitality iates.
Incieased vasculai peimeability iesults in
edema, hypovolemia, hypotension, ischemia,
gangiene. Rash iesults fiom extiavasation of
blood aftei vasculai neciosis.
Iocubatoo Ferod Range, 3-14 days (mean,
7 days) aftei the tick bite.
Frodrome Fevei/chills, anoiexia/nausea/vom-
iting, iiiitability, malaise, seveie headache, my-
algia.
hstory oI Ick 8te 60% of cases.
Symptoms Onset is usually abiupt: fevei
(94%), seveie headache (86%), geneialized
myalgia (especially the back and leg muscles,
aithialgia; 83%), sudden shaking iigoi, photo-
phobia, piostiation, nausea/vomiting/abdomi-
nal pain, all within the fiist 2 days. Symptoms
aie similai to those of many acute infectious
diseases, making specific diagnosis difficult
duiing the fiist few days. On fiist day of illness,
only 14% of patients have chaiacteiistic iash;
duiing fiist 3 days, 49% of patients have iash.
In 20% of cases, iash appeais only on day 6 oi
aftei. In 13% of cases, no iash is detected (spot-
less RMSF).
Sko Iesoos
Day 1 of illness, 14% have iash; day 3, 49%.
Initially, few small, pink macules. Tempoial
evolution of the iash is extiemely helpful in the
diagnosis.
Types Tache noiie uncommon in RMSF. Eaily
lesions, 2-6 mm, pink, blanchable macules
(Figs. 26-1 and 26-2). In 1-3 days, evolve to
deep ied papules (Fig. 26-3). In 2-4 days, be-
come hemoiihagic, no longei blanchable. Local
edema. With DIC oi piolonged hypotension,
acial skin neciosis/gangiene occui, but iaie.
DIstrIhutIvn Chaiacteiistically, iash begins
on wiists (Fig. 26-1), foieaims, and ankles (Fig.
26-2) and somewhat latei on palms and soles.
Within 6-18 h, iash spieads centiipetally to the
aims, thighs, tiunk (Fig. 26-3), and face. The
FICk 26-1 kocky Mouotao
spotted Iever: ear|y E|,||er+|ou
+ud |ero|||+|c r+cu|e +ud p+pu|e
+ppe+|ed |u|||+||, ou ||e W||| o| +
,ouu c|||d.
FICk 26-2 kocky Mouotao spotted Iever:
ear|y E|,||er+|ou +ud |ero|||+|c r+cu|e +ud
p+pu|e +ppe+|ed |u|||+||, ou ||e +u||e o| +u +do|eceu|.
hemoiihagic iash involving the palms and soles
occuis in 36-82% of cases and appeais aftei the
fifth day of illness in 43%. Neciosis occuis in
acial extiemities following hypotension.
Ceoera| Fodos Fevei to 40C. Hypotension,
shock latei in couise. Hepatomegaly, spleno-
megaly, GI hemoiihage, encephalitis (alteied
consciousness, confusion, lethaigy, stupoi, de-
liiium, coma), cianial neive palsy, incontinence,
ienal failuie, and secondaiy bacteiial infections
(lung, middle eai, paiotid gland) may occui.
varaots So|ess [eer : 13% of cases. Asso-
ciated with highei moitality iate because of
delay in diagnosis. |Jomna| synJrome : Can
mimic acute abdomen, acute cholecystitis, acute
appendicitis. T|rom|ot |rom|otyoent
urura.
Wthout kash Influenza, enteioviial infec-
tion, infectious mononucleosis, viial hepati-
tis, leptospiiosis, typhoid fevei, giam-negative
oi -positive sepsis, ehilichiosis, muiine typhus,
iickettsialpox.
Wth kash Rubeola, iubella, meningococ-
cemia, disseminated gonococcal infection, toxic
shock syndiome, diug hypeisensitivity, thiom-
bocytopenic puipuia, Kawasaki syndiome, vas-
culitis.
hemato|oy Thiombocytopenia.
Chemstry Hyponatiemia, elevated ami-
notiansfeiases .
Sko 8opsy Neciotizing vasculitis: iickettsia
can at times be demonstiated within endothe-
lial cells by immunofluoiescence oi immunoen-
zyme staining techniques.
DIrect 1mmunv]|uvrescence Specific R. rt|-
es antigen within endothelial cells 70%
sensitive; 100% specific. Tieatment with anti-
iickettsial diugs within 48 h ieduces sensitivity.
Serodaooss Indiiect immunofluoiescence
assay (IFA) can be used to measuie both IgG
and IgM anti- R. rt|es antibodies. Fouifold
iise in titei between acute and convalescent
stages is diagnostic, with a titei of 64 detecta-
ble between 7 and 10 days aftei onset of illness.
0IACN0SIS
Clinical and epidemiologic consideiations moie
impoitant than a laboiatoiy diagnosis in eaily
RMSF. Suspect in febiile childien, adolescents,
and men > 60 yeais of age with tick exposuie in
endemic aieas. Diagnosis must be made clini-
cally and confiimed latei. Only 3% of patients
with RMSF piesent with the tiiad of iash, fevei,
and histoiy of tick bite duiing fiist 3 days of
illness.
C0kS AN0 Fk0CN0SIS
Seveie couise is associated with oldei age,
delay in diagnosis, delay in oi no tieatment,
male sex, Afiican-Ameiican iace, alcoholism,
G6PD deficiency. Untieated (befoie the avail-
ability of effective antibiotics), the fatality iate
was 23%; tieated, 3% (6% if >40 yeais of age).
Fatality iate: 1.5% with known tick bite but
6.6% if no known tick exposuie. Fulminant
RMSF defined as a fatal disease whose couise
is unusually iapid (i.e., 5 days fiom onset
to death) and usually chaiacteiized by eaily
onset of neuiologic signs and late oi absent
iash. In uncomplicated cases, defeivescence
usually occuis within 48-72 h aftei initiation
of theiapy.
MANACMNI
Freveotoo Avoid tick bites: piotective cloth-
ing, tick iepellants (DEET), peimethiin on
clothing. Aftei possible exposuie, inspect foi
ticks (see Lyme Boiieliosis" in Section 24).
Aotrckettsa| Iherapy Specific antiiickettsial
theiapy should be initiated as soon as the diag-
nosis is suspected clinically.
Drug v] ChvIce Doxycycline (except foi pieg-
nant patients, histoiy of alleigy to doxycycline),
100 mg eveiy 12 h PO oi IV foi adults. Tetiacy-
cline 25-50 mg/kg pei day PO in foui divided
doses.
A|ternutIve Chloiamphenicol, 50-75 mg/kg
pei day in foui divided doses. Cipiofloxacin
iepoited to be effective.
Supportve Iherapy Foi acute pioblems of
shock, acute ienal failuie, iespiiatoiy failuie,
piolonged coma.
0ccu| |o|+||,
\ec|o|. .+||ou ||c|
',rp|or+|o|o,. |+ue ||or r||d u|c||u|c+|
|||ue |o |e||||e |||e|||e+|eu|u oue
I+c|e uo||e +| |uocu|+||ou ||e
E\+u||er. r+cu|e +ud p+pu|e ou ||uu|
I|e+|reu|. do\,c,c||ue 00 r |W|ce d+||,
IICk-80kN IFhS |C|9 . 032.9
|C|0 . A11.0
to|oy Vaiious iickettsiae (see Table 26-3).
Ae oI 0oset Moie common in childien
and young adults, ielated to out-of-doois
activities.
Ceoraphc 0strbutoo See Table 26-3.
Iraosmssoo Ixodes aithiopod ticks; widely
distiibuted aiound the woild. Bite; excoiiation
of feeding site inoculates iickettsiae in tick body
fluid oi feces.
Seasoo Mediteiianean spotted fevei (MSF)
occuis mainly in waimei summei months (July,
August, Septembei) when ticks aie feeding.
Iocubatoo Ferod Range, 3-14 days (mean,
7 days) aftei the tick bite.
Frodrome Nonspecific.
Irave| hstory Recent tiavel to oi living in
endemic iegion, e.g., iecent Afiican safaii,
FICk 26-3 kocky Mouotao spotted
Iever: |ate ||er|u+|ed |ero|||+|c
r+cu|e +ud p+pu|e ou ||e |+ce, uec|,
||uu|, +ud +|r ou ||e |ou||| d+, o| |e||||e
|||ue |u +u o|de| c|||d. I|e |u|||+| |e|ou
We|e uo|ed ou ||e W||| +ud +u||e, u|e
queu||, e\|eud|u ceu|||pe|+||,.
adventuie tiavel, militaiy seivice in Afiica with
Afiican tick bite fevei.
hstory oI Ick 8te Often not elicited in that
iickettsiae aie tiansmitted by tiny immatuie
laivae and nymphs.
Symptoms Onset is sudden in 50% of patients.
Most common: headache, fevei; also chills, my-
algias, aithialgias, malaise, anoiexia.
Sko Iesoos
Tche NvIre An inoculation eschai: papule
foims at the bite site and evolves to a painless,
black-ciusted ulcei with ied halo (iesembles a
cigaiette buin) (Fig. 26-4) in 3-7 days. Occuis
in all spotted feveis except RMSF.
Rush About 3-4 days aftei appeaiance of
tache noiie, an eiythematous maculopapulai
eiuption appeais on tiunk; may subsequently
disseminate, involving face, extiemities, palms/
soles. Density of eiuption heightens duiing next
few days. In seveie cases, lesions may become
hemoiihagic.
0strbutoo Similai pattein of spiead and dis-
tiibution in all spotted feveis-tiunk, extiemi-
ties, face (centiifugal)- exte RMSF, which
fiist appeais at wiists and ankles and spieads
centiipetally.
Ceoera| Fodos Conjunctivitis, phaiyn-
gitis, photophobia. Cential neivous system
(CNS) symptoms (confusion, stupoi, deliiium,
seizuies, coma) common in RMSF but not seen
in othei spotted feveis.
Iymph Nodes Nodes pioximal to tache noiie
aie usually enlaiged and nontendei.
Viial exanthems, diug eiuption.
IA8I 26-3 Etio|oy and Ceoraphic 0istribution of Iick Iyphus
0|sease |o h0maos t|o|ogy 0|str|b0t|oo
Iick typhus '|r||+| u|pec|e |||r+|||,
\ed||e||+ue+u couu|||e
(ou||e|u Eu|ope
|e|oW +5||
p+|+||e|), +|| o|
A|||c+, |ud|+,
ou||We|e|u +ud
ou||ceu||+| A|+
\ed||e||+ue+u po||ed | : \ed||e||+ue+u
|e.e| (||e.|e |ou|ouueue, | : c-|- ||+ud +ud
\+|e|||e |e.e|), | : :cc u||ouud|u |+ud,
Keu,+ ||c| |,p|u, | c-:||c A|||c+
||+e|| po||ed |e.e|, | |.c:c
|ud|+u ||c| |,p|u, | |:c ||c-
A||+||+u po||ed | c|c-
|e.e|
A|||c+u ||c||||e |e.e| | : .+|. - Ceu||+|, e+|e|u,
(||c| |,p|u) (| c|:c-) ou||e|u A|||c+
'||e||+u (|o||| A|+u) | |:c '||e||+, \ouo||+,
||c| |,p|u uo|||e|u C||u+
0ueeu|+ud ||c| |,p|u | c|c| Au||+||+
|||ude| ||+ud po||ed | |- |||ude| ||+ud
|e.e| (ue+| I+r+u|+)
l+p+uee/0||eu|+| | c:c l+p+u
po||ed |e.e|
SCII0N 26 k|CKEII'|A| |||ECI|0|' T6T
0ermatopatho|oy DIrect 1mmunv]|uvres-
cence Rickettsiae can be detected in lesional
biopsy specimens fiom site of tick bite and
cutaneous lesions; also in ciiculating endothelial
cells and vaiious tissues obtained postmoitem.
Fo|ymerase Chao keactoo Detects iickettsial
DNA in skin lesions.
Serodaooss Vaiious tests aie available. An-
tiiickettsial theiapy usually blunts antibody
iesponses. Demonstiation of antibodies to SFG
iickettsiae by micioimmunofluoiescence, latex
agglutination, enzyme immunoassay, Westein
blot, oi complement fixation. Enzyme-linked
immunosoibent assay (ELISA) (IgM captuie
assays) among the most sensitive.
0IACN0SIS
Epidemiologic and clinical findings with iden-
tification of tache noiie confiimed by dem-
onstiation of iickettsiae by immunohistologic
techniques in lesional skin biopsy specimens
and/oi seiology. In an endemic aiea, patients
piesenting with fevei, iash, and/oi a skin lesion
consisting of black neciotic aiea oi a ciust sui-
iounded by eiythema should be consideied to
have one of the iickettsial spotted feveis.
C0kS AN0 Fk0CN0SIS
In Fiance and Spain, moitality iate similai to
that of RMSF. Spotted feveis aie usually mildei
in childien. Moibidity and moitality iates aie
highei (up to 50%) in individuals with diabetes
mellitus, caidiac insufficiency, alcoholism.
MANACMNI
Freveotoo Contiol host animals and vectois.
Aotrckettsa| Iherapy Specific antiiickettsial
theiapy abbieviates the length and seveiity
of illness, i.e., spotted feveis, tick typhus, and
iickettsialpox.
Drug v] ChvIce Doxycycline, 100 mg PO
twice daily foi 7-10 days.
A|ternutIves Cipiofloxacin, 750 mg PO twice
daily foi 5 days, or chloiamphenicol, 500 mg PO
foui times a days foi 7-10 days, or josamycin (in
piegnancy), PO foi 5 days.
FICk 26-4 Ick-boroe typhus,
AIrcao tck bte Iever: tche
oore C|u|ed e|o|ou W||| u||ouud
|u e|,||er+ ou ||e |e|| ||p +|e+. I|e
p+||eu| |+d ju| |e|u|ued ||or + +|+||
|u 'ou|| A|||c+. ne uo|ed oue| o| |e.e|,
||u|||e ,rp|or, +ud ||e ||p |e|ou. I|e
p+||eu|' W||e p|o|o|+p|ed ||e |+c|e
uo||e ||e, +ud er+||ed ||e |r+e |o ||
p|,|c|+u, |+c||||+||u ||e d|+uo|. ne
W+ ||e+|ed W||| do\,c,||ue.
E||o|o,. | c|c
\ec|o|. r|ce r||e ( |,!- c- ),
o||e| r||e, ||+uo.+||+u ||+ur||ou
Ceo|+p|,. uu||ed '|+|e, Eu|ope, ku|+, 'ou||
A|||c+, Ko|e+, Eu|ope
C||u|c+| |e+|u|e.
I+c|e uo||e (||. 2o5)
ke|ou+| |,rp| uode eu|+|ed
0-1 d+, +||e| |||e, uoupec|||c ,rp|or o|
r+|+|e, c||||/|e.e|, |e+d+c|e, r,+||+, u+ue+/
.or|||u/+|dor|u+| p+|u, cou|, coujuuc||.|||,
p|o|op|o||+ r+, occu|
k+|. 2-o d+, +||e| oue| o| uoupec|||c
,rp|or, |ed r+cu|e +ud p+pu|e +ppe+|
(||. 2oo), r+, e.o|.e |o c|+|+c|e||||c .e|c|e
(po\), c|u|ed e|o|ou occu|, |e|ou |e+| W|||
ou| c+|||u
|e.e| |eo|.e |u o-0 d+, W|||ou| ||e+|reu|
W||| do\,c,c||ue
||||e|eu||+| d|+uo|. \+||ce||+, p||,||+| ||
c|euo|de e| .+||o|||o|r| +cu|+ (||E\A), .||+|
e\+u||er, d|er|u+|ed ouococc+| |u|ec||ou
|e|r+|op+||o|o,. B++| |+,e| o| ep|de|r| |oW
.+cuo|+| deeue|+||ou, .e|cu|+||ou | u|ep|de|
r+|. 'upe|||c|+| +ud r|dde|r+| ueu||op||||c +ud
rououuc|e+| ce|| |u|||||+|e +|e p|eeu|
I|e+|reu|. do\,c||ue, 00 r B|| (+du||)
pdemc (Iouse-8oroe) Iyphus (Table 26-3)
Eo|ogy : R. rowa:e| . Vetor : human louse
(PeJtu|us |umanus torors, PeJtu|us |u-
manus tas); lives on clothing; miciobe in
louse feces inoculated by sciatching. Reseror :
humans, flying squiiiels. Epidemics associated
with wai/loweiing of hygiene. Rickettsiae can
peisist foi yeais in lymph nodes without any
symptoms. C|nta| [nJngs : systemic infection
and piostiation may be seveie, fevei lasts foi
about 2 weeks; moie seveie and moie often
fatal in patients ovei 40 yeais of age. Ras| :
noted on fifth day of fevei; initially on uppei
tiunk, then disseminated; macules evolving
to papules; often, no iash. Duiing epidemics,
fatality iate up to 30%. Biill-Zinssei disease
is a ieciudescence of a piioi/-latent epidemic
typhus infection.
odemc Muroe Iyphus Eo|ogy : R. y| .
Vetor : iat flea ( Xenosy||a t|eoss ). Reseror :
iats, cats, opossums. Ceogra|y : woildwide but
not iepoited in Biitain, Scandinavia. Epidem-
ics associated with pooi hygiene as in waitime
I0S-80kN IFhS |C|9 . 03.0
|C|0 . 15.0
kICkIISIAIF0X |C|9 . 03!.2
|C|0 . A19.
and disastei. C|nta| [nJngs : iesembles epi-
demic typhus but mildei; iaiely fatal. Rash in
13% of cases, noted 4 days aftei onset of fevei;
macules and papules on tiunk. Latent infec-
tions in human.
Scrub Iyphus Eo|ogy : Orena (foimeily
Rt|esa ) susugamus| . Vetor : mites.
Reserors : laival stage (chiggei) of tiombiculid
mites (tiansovaiian tiansmission), humans,
iat. Ceogra|y : Fai East, i.e., Myanmai, In-
dia, Sii Lanka, New Guinea, Japan, Taiwan.
IntJente : high pievalence but often uniecog-
nized. C|nta| [nJngs : iesembles epidemic
typhus; geneialized lymphadenopathy. Ras| :
<50% of cases tache noii at mite bite site oi
maculopapulai tiunkal iash aftei onset of fevei.
Pneumonitis, myocaiditis and caidiac failuie,
encephalitis oi meningitis, acute abdominal
pain, gianulomatous hepatitis, disseminated
intiavasculai coagulation, oi acute ienal failuie
may develop.
Treutment : doxycycline 100 mg BID foi 7 days
Syoooym: Tsutsugamushi fevei
FICk 26-5 kckettsa|pox: tche oore A c|u|ed, u|ce|+|ed p+pu|e (ec|+|) W||| + |ed |+|o |eer|||u
+ c|+|e||e |u|u +| ||e ||e o| + ||c| |||e.
FICk 26-6 kckettsa|pox: exao-
them \u|||p|e |u| d|c|e|e e|,||er+
|ou p+pu|e +ud pu|u|e, ore W|||
ceu||+| |ero|||+e +ud c|u||u, ou ||e
|+c| +||e| |er+|oeuou d|er|u+||ou
o| | c|c ||or ||e ||c| |||e ||e.
TT0
S E C I | 0 N 2 1
vIkAI INFCII0NS 0F
SkIN AN0 MC0SA
\||+| |u|ec||ou o| ||u +ud ruco+ p|oduce +
W|de pec||ur o| c||u|c+| r+u||e|+||ou.
\||ue ||+| c+ue |e||||e |||ue W||| e\+u||er
+|e uu+||, e||||r||ed, W||| p||r+|, |u|ec||ou
cou.e,|u |||e||re |rruu||,.
\||ue uc| + |ur+u p+p|||or+.||u (n|\) +ud
ro||ucur cou|+|our .||u (\C\) co|ou|/e
||e ep|de|r| o| ro| |ud|.|du+| W|||ou| c+u|u
+u, c||u|c+| |e|ou.
Beu|u ep|||e||+| p|o|||e|+||ou, |.e., W+|| +ud
ro||ucur, occu| |u ore co|ou|/ed pe|ou,
+|e ||+u|eu|, +ud e.eu|u+||, |eo|.e W|||ou|
||e|+p,.
|u |rruuocorp|or|ed |ud|.|du+|, |oWe.e|,
||ee |e|ou r+, |ecore e\|eu|.e, pe|||eu|,
+ud |e||+c|o|, |o ||e|+p,.
I|e e||| |ur+u |e|pe.||ue o||eu |+.e +,rp
|or+||c p||r+|, |u|ec||ou |u| +|e c|+|+c|e||/ed |,
|||e|ou |+|eu| |u|ec||ou.
|u ||e e|||u o| |rruuocorp|or|e, |e|pe.|
|ue c+u |ecore +c||.e +ud c+ue d|e+e W|||
|u|||c+u| ro|||d||, +ud ro||+|||, |+|e.
I|e po\.||u |+r||, | + d|.e|e |oup o| ep|||e||o
||op|c .||ue ||+| |u|ec| |ur+u +ud +u|r+|.
I|e eue|+ o| po\.||ue ||+| |u|ec| |ur+u
|uc|ude o|||opo\.||u, p+|+po\.||u, ro||u
c|po\.||u, +ud ,+|+po\.||u (I+||e 21-).
0u|, r+||po\ .||u ('|\) +ud ro||ucur cou
|+|our .||u (\C\) c+ue u+|u|+| d|e+e |u
|ur+u.
'r+|| po\ ('|\) +ud rou|e,po\ .||u |,p|c+||,
c+ue ,|er|c d|e+e W||| |+|, o||e| po\.|
|ue c+ue |oc+||/ed ||u |e|ou.
0||e| po\.||ue +|e +oc|+|ed W||| /oouo||c
|u|ec||ou.
|o\.||ue +|e ||e |+|e| o| +|| +u|r+| .||ue +ud
|+.e + dou||e||+ud ||A euore.
I|e, +|e ||e ou|, ||A .||ue ||+| |ep||c+|e |u
c,|op|+r, W|e|e +ccuru|+|ed .||+| p+|||c|e
|o|r eo|uop||||c |uc|u|ou, o| Cu+|u|e|| |od
|e, .||||e |, |||| r|c|ocop, (200-+00 r).
|o\.||ue +ppe+| + |||c||+ped o| o.+| .||u
p+|||c|e |, e|ec||ou r|c|ocop,.
I|e uuc|eoore cou|+|u dou||e||+ud ||A,
W||c| | u||ouuded |, + rer||+ue.
I|e ou|e| u||+ce o| ||e ||pop|o|e|u |||+,e| |+ u|
|+ce |u|u|e ||+| +|e |+udor|, +||+ued +ud |.e
||e .|||ou || c|+|+c|e||||c |e\|u|ed +ppe+|+uce.
'r+||po\, o| .+||o|+, |+ |eeu e|+d|c+|ed + +
u+|u|+||, occu|||u |u|ec||ou.
CoWpo\ | +u |u|ec||ou o| c+|||e c+ued |, coW
po\ .||u.
I|e o|||u o| .+cc|u|+ .||u, W||c| | ued
|o |rruu|/e |ur+u ++|u| r+||po\, +|e
uuce||+|u. || r+, |e de||.ed ||or .+||o|+ .||u,
coWpo\ .||u, o| |e + |,|||d o| ||e |Wo.
\C\ co|ou|/e ||e ||u o| r+u, |e+|||, |ud|.|du
+|, c+u|u ro||ucur cou|+|our, e||||r||ed
ep|de|r+| p|o|||e|+||ou ||+| |eo|.e pou|+ue
ou|,.
nur+u o|| +ud r|||e|' uodu|e +|e /oouo||c
|u|ec||ou ||+| c+u ore||re occu| |u e\poed
|ur+u.
0||e| po\.||ue ||+| +|e /oouoe |u +u|r+|
|o| (rou|e,, coW, |u||+|o, |eep, o+|) c+u
+|o |u|ec| |ur+u.
|o\.||ue c+ue |o\|c e||ec| ou ce||, W||c| |eu||
|u ce|| |ouud|u +ud c|urp|u, deeue|+||ou o|
ce|| +|c|||ec|u|e, +ud p|oduc||ou o| c,|op|+r|c
.+cuo|e.
||||e|eu| po\.||ue +|e c+p+||e o| p|oduc|u +
|oc+||/ed, e||||r||ed |u|ec||ou |, |uocu|+||ou |o
||e ||u (e.., o||) o| + |u|r|u+u| ,|er|c d|e+e
(e.., .+||o|+).
I|e +re .||u c+u +||ec| d|||e|eu| pec|e |u d||
|e|eu| W+,.
F0XvIkS INFCII0NS
SCII0N 2T \|kA| |||ECI|0|' 0| 'K|| A|| \uC0'A TT1
\o||ucur cou|+|our (\C) | + e||||r||ed
ep|de|r+| .||+| |u|ec||ou.
k|| |oup
C|||d|eu
'e\u+||, +c||.e +du||
|rruuocorp|or|ed. n|\/A||', o|+u ||+u
p|+u| |ec|p|eu|
C||u|c+| r+u||e|+||ou.
'||uco|o|ed p+pu|e, o||eu ur||||c+|ed
|eW |o r,||+d o| |e|ou
n|\/A||'. |+|e uodu|e, cou||ueu|
Cou|e.
ne+|||, pe|ou. \C |eo|.e pou|+ueou|,
n|\/A||'. || uo| ucce|u||, ||e+|ed W||| +u||
|e||o.||+| ||e|+p, (AkI), \C c+u |ecore |ue
+ud cou||ueu|.
M0IISCM C0NIACI0SM |C|9. 013.0

|C|0. B03.
IA8I 2T-1 Po\viruses Ihat |nfect humans
*
and Cause 0isease
6eo0s aod Protect|oo
Spec|es Pr|mary Node oI Prov|ded by
(0|sease) 8eservo|r 6eograph|c 8eg|oo Traosm|ss|oo Vacc|oat|oo
0rthopoxvrus
CoWpo\ kodeu| Eu|ope, A|||c+, |||ec| cou|+c| \e
ceu||+|/uo|||e|u A|+
\ou|e,po\ kodeu| we|/ceu||+| A|||c+ |||ec| cou|+c|, \e
|ep||+|o|, d|op|e| \e
\+cc|u|+ uu|uoWu |||ec| cou|+c|
\+||o|+ (r+||po\) nur+u u.'., ku|+ |||ec| cou|+c|, \e
|ep||+|o|, d|op|e|
atapoxvrus
I+u+po\ |ou|ur+u p||r+|e Keu,+, /+||e |||ec| cou|+c| |o
\+|+po\ |ou|ur+u p||r+|e Ceu||+| A|||c+ |||ec| cou|+c| |o
Farapoxvrus
|eudocoWpo\ uuu|+|e wo||dW|de |||ec| cou|+c| |o
(r|||e|' uodu|e
+ud p+|+.+cc|u|+)
Bo.|ue p+pu|+| uuu|+|e (|ur+u) u.'., C+u+d+, A|||c+, |||ec| cou|+c| |o
|or+|||| Au||+||+, |eW /e+|+ud,
(r|||e|' uodu|e) C|e+| B|||+|u, Eu|ope
0|| (|ur+u o||) uuu|+|e (|ur+u) |o||| Are||c+, |||ec| cou|+c| |o
Eu|ope,
|eW /e+|+ud
'e+|po\ 'e+| |o||| 'e+, |+c|||c |||ec| cou|+c| |o
0ce+u, A||+u||c
0ce+u
Mo||uscpoxvrus
\o||uc|po\ nur+u wo||dW|de |||ec| cou|+c| |o
(ro||ucur
cou|+|our)
*
|o\.||ue ||+| do uo| |u|ec| |ur+u |uc|ude c+re|po\ +ud |eep +ud o+| |urp, ||u d|e+e corp|e\.
FAkI III ||'EA'E' |uE I0 \|Ck0B|A| ACE|I' TT2
to|oy
Molluscum contagiosum viius.
Foui disciete viial subtypes, I, II, III, IV.
30% homology with smallpox viius.
The viius has not been cultuied.
Not distinguishable fiom othei poxviiuses by
election micioscopy.
In many healthy adults, the epideimis and
infundibulum of haii follicle aie colonized by
MCV.
Ae, Sex Childien; sexually active adults;
males females.
ksk Factors HIV-infected peisons with low
CD4 T cell counts may have hundieds of small
mollusca oi giant mollusca on the face.
Iraosmssoo Skin-to-skin contact.
C|assIcatoo by ksk Croups
ChI|dren
Mollusca commonly occui on exposed skin
sites.
Child-to-child tiansmission ielatively low.
Resolve spontaneously.
Seruu||y ActIve Adu|ts
Occui in genital iegion.
Viius tiansmitted duiing sexual activity.
Resolve spontaneously.
H1V/A1DS: Orgun Trunsp|unt RecIpIents
Most commonly occui on the face, spiead by
shaving.
With iesponse to ART, lesions often iesolve.
Without aggiessive theiapy in advanced HIV/
AIDS, mollusca enlaige; spontaneous iegies-
sion does not occui.
FAIh0CNSIS
A subclinical caiiiei state of MCV piobably
exists in many adults.
Unique among poxviiuses, MCV infection
iesults in epideimal tumoi foimation; othei
human poxviiuses cause a neciotic pox"
lesion.
Ruptuie and dischaige of the infectious viius-
packed cells occui in the umbilication/ciatei
of the lesion.
0uratoo oI Iesoos
In the noimal host, mollusca usually peisist
up to 6 months and then undeigo spontane-
ous iegiession.
In HIV/AIDS without effective ART, mollusca
peisist and piolifeiate even aftei aggiessive
local theiapy.
Sko Symptoms
Usually none.
Cosmetic disfiguiement.
Concein about having a tiansmissible infection.
Painful if supeiinfected.
Papules (1-2 mm), nodules (5-10 mm)
(iaiely, giant) (Fig. 27-1, B). Peaily white
oi skin-coloied. Round, oval, hemispheiical,
umbilicated (Fig. 27-1B).
Isolated single lesion; multiple, scatteied dis-
ciete lesions; oi confluent mosaic plaques.
Most laigei mollusca have a cential keiatotic
plug (Fig. 27-1), which gives the lesion a
cential dimple oi umbilication, best obseived
aftei light liquid nitiogen fieeze. Gentle pies-
suie on a molluscum causes the cential plug
to be extiuded.
Autoinoculation is appaient in that mol-
lusca aie clusteied at a site such as the axilla
(Fig. 27-2).
Host immune iesponse to viial antigen ie-
sults in an inflammatoiy halo aiound MC
(Fig. 27-2), i.e., MC deimatitis," which usu-
ally heialds spontaneous iegiession; puiu-
lence may occui.
MC can be extensive in oigan tiansplant ie-
cipients (Fig. 27-3).
In HIV-infected males who shave, mollusca
can be confined to the beaid aiea. Hun-
dieds of lesions occui in HIV/AIDS patients
(Fig. 27-3, 27-4; see also Fig. 31-8).
In daik-skinned individuals, significant
postinflammatoiy hypeipigmentation aftei
tieatment oi spontaneous iegiession may
occui.
DIstrIhutIvn
Any site may be infected, especially axillae,
antecubital, popliteal fossae, ciuial folds.
In childien: genital lesions occui via autoin-
oculation.
In atopic deimatitis, MC may be widespiead.
In adults with sexually tiansmitted MC: gioins,
genitalia, thighs, lowei abdomen (Fig. 27-4).
Multiple facial MC suggest HIV infection.
MC can occui in the conjunctiva, causing a
unilateial conjunctivitis.
FICk 2T-1 Mo||uscum cootaosum (MC): |esoos \C |e|ou |,p|c+||, +|e d|rp|ed o| ur||||c+|ed
p+pu|e o| uodu|e. . ||u| ||u, douu||+ped p+pu|e W||| + dep|eed |e|+|o||c co|e ou ||e ||p o| + |e+|||,
+du||. 8. ur||||c+|ed p+pu|e ou ||e rou|+c|e +|e+ +ud c|ee| o| |e+|||, |eeu+e |||. \C corrou|, +||e |u
|+|| |o|||c|e, ||e ur||||c+||ou occu|||u +| ||e o||ur o| ||e |o|||cu|e. I|e |e|+||u+ceou co|e c+u |e re+|ed ou
+ r|c|ocope ||de +ud |+|ued |o |oW |u||+c,|op|+r|c ro||ucur |od|e, .||+| c,|op|+r|c |ucu|ou.
8
Mu|tp|e Sma|| Mo||usca Flat waits, condylo-
mata acuminata, syiingoma, sebaceous hypei-
plasia.
Iare So|tary Mo||uscum Keiatoacanthoma,
squamous cell caicinoma, basal cell caicinoma,
epideimal inclusion cyst.
Mu|tp|e Faca| Mo||usca o hIv-IoIected Iodvdua|
Disseminated invasive fungal infection, i.e.,
ciyptococcosis, histoplasmosis, coccidioidomy-
cosis, penicillinosis. (See Section 25.)
Smear oI keratotc F|u Diiect micioscopic
examination of Giemsa-stained cential semi-
solid coie ieveals molluscum bodies" (inclu-
sion bodies).
0ermatopatho|oy
Epideimal cells contain laige intiacytoplasmic
inclusion bodies, i.e., molluscum bodies, that
appeai as single, ovoid eosinophilic stiuctuies
in lowei cells of stiatum malpighii.
Molluscum bodies contain laige numbeis of
matuiing viiions.
Epideimis giows down into deimis. Infection
also occuis in epithelium and follicle.
FICk 2T-2 Mo||uscum cootaosum: ax||a
\u|||p|e, r+|| p|u| p+pu|e |u ||e +\|||+ o| + |e+|||,
c|||d. I|e e|,||er+ u||ouud|u ||e |e|ou |ep|e
eu| +u |u||+rr+|o|, |epoue |o \C +ud uu+||,
|ud|c+|e ||e |e|ou +|e |e|e|u.
FICk 2T-3 Mo||uscum cootaosum o |uo traosp|aot recpeot: peroeum aod buttocks A
!1,e+|o|d |uu ||+up|+u| |ec|p|eu| W||| uure|ou p|u| p+pu|e c|u|e| |u |u|e|||||uou ||e o| |u||oc| +ud
pe||ueur. \C We|e ||e+|ed W||| e|ec||ou|e|,.
0IACN0SIS
Usually made on clinical findings.
Biopsy lesion in HIV-infected individual if
disseminated invasive fungal infection is in the
diffeiential diagnosis.
C0kS AN0 Fk0CN0SIS
In healthy individuals, MC iesolves spontane-
ously without scaiiing, but may take up to
2 yeais.
In HIV/AIDS without effective ART, mollusca
often piogiess even with aggiessive theiapies,
cieating significant cosmetic disfiguiement,
especially by facial lesions. In HIV/AIDS
successfully tieated with ART, mollusca eithei
do not occui oi iesolve aftei seveial months.
Recuiience of mollusca usually indicates
failuie of ART.
FICk 2T-4 Mo||uscum cootaosum o hIv[
AI0S: cooI|ueot |esoos oo aooeota| area A
+2,e+|o|d r+|e W||| n|\/A||', |+|||u +u|||e||o.||+|
||e|+p, (AkI). ||c|e|e +ud cou||ueu| ||uco|o|ed
ur||||c+|ed p+pu|e |u ||e +uoeu||+| +|e+.
MANACMNI
Freveotoo A.o|d ||u|o||u cou|+c| W||| |ud|.|du+| |+.|u ro||uc+. n|\|u|ec|ed
|ud|.|du+| W||| ro||uc+ |u ||e |e+|d +|e+ |ou|d |e +d.|ed |o r|u|r|/e
|+.|u |+c|+| |+|| o| |oW + |e+|d.
Supportve therapy |u |rruuocorpe|eu| c|||d|eu +ud e\u+||, +c||.e +du||, ro||uc+ |e|e
pou|+ueou|,, p+|u|u| +|e|.e ||e|+p, | uo| |ud|c+|ed.
Ireatmeot oI |esoos
Iop|c+| p+||eu|d||ec|ed ||e|+p, 5 |r|qu|rod c|e+r +pp||ed +| |ed||re !-5 ||re pe| Wee| |o| up |o
-! rou||.
C||u|c|+ud||ec|ed ||e|+p, (o|||ce) I|ee p|ocedu|e +|e p+|u|u| +ud ||+ur+||c, epec|+||, |o| ,ouu c|||d|eu. E\|A
c|e+r +pp||ed |o |e|ou | |e|o|e ||e|+p, r+, |educe/e||r|u+|e p+|u.
Cu|e||+e 'r+|| ro||uc+ c+u |e |ero.ed W||| + r+|| cu|e||e W||| |||||e d|cor|o||
o| p+|u.
C|,ou|e|, ||ee/|u |e|ou |o| 0-5 | e||ec||.e +ud r|u|r+||, p+|u|u|, u|u e|||e| +
co||ou||pped +pp||c+|o| o| ||qu|d u|||oeu p|+,.
E|ec||ode|cc+||ou |o| ro||uc+ |e||+c|o|, |o c|,ou|e|,, epec|+||, |u n|\|u|ec|ed |ud|.|du+|
W||| uure|ou +ud/o| |+|e |e|ou, e|ec||ode|cc+||ou o| |+e| u|e|, | ||e
||e+|reu| o| c|o|ce. |+|e |e|ou uu+||, |equ||e |ujec|ed ||doc+|ue
+ue||e|+. C|+u| ro||uc+ r+, |equ||e e.e|+| c,c|e o| e|ec||ode|cc+||ou
+ud cu|e||+e |o |ero.e ||e |+|e |u|| o| |e|ou, ||ee |e|ou r+, e\|eud
|||ou| ||e de|r| |u|o ||e u|cu|+ueou |+|.
FI0MI0I0C
Iooooss Sheep-pox, lip scab of sheep, scabby
mouth, soie mouth, contagious pustulai dei-
matosis, infectious pustulai deimatitis.
0sease o Aoma|s
Ungulates (sheep, goats, yaks, deei, etc.).
Viius suivives foi many months on fences,
feeding basins, and suifaces in bains.
Only newboin animals lacking viial immu-
nity aie susceptible.
Manifested as eiythematous, exudative nod-
ules aiound mouth that heal spontaneously
in about a month, pioducing peimanent im-
munity; lesions may become supeiinfected.
Iraosmssoo to humaos
Humans aie infected by
Inoculation of viius by diiect contact with
lambs (bottle feeding)
Indiiectly via fomites (knives, sheais,
baibed wiie, feeding tioughs, bain doois,
fences, etc.).
Human-to-human infection does not occui.
Iocdeoce Most common in faimeis, veteii-
naiians, sheep sheaieis.
Seasoo Usually in spiingtime (when lambs
aie boin) and season (Eastei) of slaughtei of
lambs and sheep.
0emoraphy Occuis woildwide with epidem-
ics in Noiway and othei paits of Euiope, New
Zealand; iaie in Noith Ameiica.
Sko Iesoos
Initially, one oi moie papule(s) to nodule(s)
to plaque(s) on the hand(s) (Figs. 27-5, 27-6);
nur+u o|| | + p+|+po\.||u |u|ec||ou.
/oouo| |u uuu|+|e
0ccu| |u |ur+u e\poed |o ||e .||u.
C||u|c+| r+u||e|+||ou. |odu|+| |e|ou ou e\
poed cu|+ueou ||e.
',, . Ec||,r+ cou|+|our.
hMAN 0kF |C|0. B03.0
may appeai veiy edematous to vesiculai to
bullous. Lesions aveiage 1.6 cm in diametei.
The infection goes thiough six clinical stages,
each lasting 6 days:
Macule to papule, pink to ied taiget
lesion: nodule with ied centei (Fig. 27-5),
white middle iing, and ied peiipheiy
a cute exudative nodule i egeneiative
diy nodule coveied by a thin ciust with
black dots papillomatous i egiessive
with diy ciust (Fig. 27-6)
DIstrIhutIvn Exposed sites (hands, aims, legs,
face); most common site is doisum of iight
index fingei.
Other FIndIngs
Ascending lymphangitis and lymphade-
nopathy may occui.
Bacteiial supeiinfection may occui.
Moie extensive infection may occui in the
immunocompiomised host.
Milkei`s nodules, anthiax, tulaiemia, piimaiy
inoculation tubeiculosis, atypical mycobacte-
iia infection, syphilitic chancie, spoiotiichosis,
pyogenic gianuloma.
|ectroo Mcroscopy Biopsy of lesional skin
shows chaiacteiistic biick-shaped viial paiticles
200-380 nm in length.
0IACN0SIS
Clinical findings with the appiopiiate histoiy.
SCII0N 2T \|kA| |||ECI|0|' 0| 'K|| A|| \uC0'A TTT
C0kS
Resolves spontaneously in 4-6 weeks, healing
without scai foimation.
Eiythema multifoime-like eiuptions have
been iepoited in human oif.
Widespiead lesions spiead by autoinocula-
tion may occui in atopic deimatitis.
Lesion may be laige and fail to iegiess in
chionic lymphocytic leukemia.
In humans, lasting immunity is confeiied by
infection.
MANACMNI
Antiviial agents aie not effective. Tieat bacteiial
supeiinfection.
FICk 2T-5 humao orI: mu|tp|e |esoos oo haods '|+e 2. \u|||p|e ||||e| W||| |+|e|/||| p+||e|u |u
|e|ou ou ||e |+ud o| + |eep |e|de|.
FICk 2T-6 humao orI: Ioer A 9,e+|o|d
r+|e o| C|ee| |e|||+e, |e|ou +ppe+|ed 0 d+,
+||e| C|ee| E+|e| +ud W+ +oc|+|ed W||| ||e |+r|
|||||u |o| ||e E+|e| |e+|. '|+e o. C|u|ed |e|e|u
uodu|e ou r|dd|e ||ue|, |e|ou ou |ude\ ||ue| |+
|eo|.ed.
to|oy Paiapoxviius (pseudocowpox viius),
similai to that which causes oif.
Syoooyms oI Aoma| IoIectoo Paiavaccinia,
bovine papulai stomatitis.
0sease o Aoma|s Papulai lesions occui on
muzzles/oial cavity of calves and on teats of
cows.
Iraosmssoo to humaos Contact with bovine
lesions oi teat cups of milking machines.
Iocdeoce Most common in daiiy faimeis.
ksk Factors New milkeis, young people,
vacation milkeis, veteiinaiy students.
0emoraphy Woildwide
Sko Iesoos
Clinical findings and couise aie similai to
human oif.
Lesions can piesent as
Solitaiy ied-puiple nodules (Fig. 27-7) oi,
less commonly, as
Multiple cheiiy-ied papules and nodules,
aiising at site of inoculation.
DIstrIhutIvn Usually on exposed
sites such as hands; may occui in
buin wounds.
0ther Fodos Lymphade-
nopathy.
Human oif, smallpox vaccination
site, staphylococcal abscess, heipes
simplex viius infection, anthiax,
Cu|+ueou |u|ec||ou c+ued |, p+|+po\.||ue.
/oouo| |u |u|ec|ed c+|||e
|u|ec||ou |u |ur+u | c+ued +cc|deu|+||,.
C||uc+| r+u||e|+||ou. uodu|+| |e|ou ou e\
poed cu|+ueou ||e.
',, . \|||e|' uode.
MIIkk'S N00IS (MN)
tulaiemia, piimaiy inoculation tubeiculosis,
atypical mycobacteiia infection, syphilitic chan-
cie, spoiotiichosis, pyogenic gianuloma.
See Human Oif," above.
0IACN0SIS
Diagnosis is usually made on histoiy of bovine
exposuie and clinical findings.
C0kS
Self-limited.
MANACMNI
Antiviial agents aie not effective. Tieat bacteiial
supeiinfection.
FICk 2T-T M|ker's oodu|e:
Ioer A |u|e |ee|, e|oded uodu|e
ou ||e ||ue| o| + d+||, |+|re| +| ||e
||e o| |uocu|+||ou.
E||o|o|c +eu|. |o\.||u .+||o|+e
Au +cu|e e\+u||er+|ou |u|ec||ou.
'e.e|e !d+, p|od|or+| |||ue
Ceue|+||/ed |+| p|e+d|u ceu||||u+||,
k+p|d|, ucce|.e p+pu|e, .e|c|e, pu|u|e,
ur||||c+||ou, +ud c|u||u W||||u + d+,.
Ep|der|c |+.e occu||ed |u +|| popu|+||ou, |eu||
|u |u |uud|ed o| r||||ou o| de+||.
',, . \+||o|+, .+||o|+ r+jo|, .+||o|+ r|uo|
(+|+|||r).
SMAIIF0X |C|9. 050.9

|C|0. B0!
http://www.bt.cdc.gov/agent/smallpox/oveiview/
disease-facts.asp
http://www.who.int/emc/diseases/smallpox/
Smallpoxeiadication.html
The last cases of endemic smallpox occuiied
in 1977.
Eiadication of the disease was declaied in
1980.
to|oy
Vaiiola viius of the family Poxviiidae, genus
Oithopoxviius (Table 27-1)
Humans aie the only host of vaiiola.
No longei exists natuially but is maintained
in ieseaich laboiatoiies and may be used as a
bioteiioiism weapon (see Appendix B).
DNA viius that ieplicates in cell cytoplasm.
0ccupatoo Laboiatoiy woikeis.
Susceptb|ty
Peisons in the geneial population in the United
States undei age 30 have not been vaccinated.
All such peisons aie susceptible to smallpox.
Some peisons boin befoie 1972 and vaccinated
may still be piotected; may have mildei disease
if exposed and less likely to tiansmit infection.
Iraosmssoo
Respiiatoiy-dioplet nuclei
Most likely fiom patients with seveie dis-
ease oi who aie coughing
Contaminated clothing/bedding
Less tiansmissible than measles, chickenpox,
influenza.
Secondaiy attack iates among unvaccinated
contacts, 37-88%.
Maximal tiansmissibity: fiom onset of enan-
thema thiough fiist 7-10 days of iash.
Seasoo Wintei, eaily spiing (endemic).
C|assIcatoo oI C|oca| Iypes oI varo|a
Varo|a ma,or (oidinaiy")
90% of cases
30% moitality
Varo|a mnor ( a|asrm ; modified-type)
2% of cases, occuiiing in unvaccinated
peisons
25% of pieviously vaccinated peisons
Varo|a sne eruone occuis in:
Pieviously vaccinated contacts
Infants with mateinal antibodies
Sma||ox w| [|a |esons
Case fatality 97% among unvaccinated pei-
sons
Hemorr|agt sma||ox
Neai 100% case fatality iate
FAIh0CNSIS
Enteis the iespiiatoiy tiact, seeding mucous
membianes, passing iapidly into local lymph
nodes.
Aftei a biief peiiod of viiemia, latent peiiod
of 4-14 days occuis, duiing which the viius
multiplies in the ieticuloendothelial system.
Anothei biief peiiod of viiemia piecedes
piodiome, duiing which mouth/phaiynx aie
infected.
Viius invades capillaiy endothelium of dei-
mal layei in skin, iesulting in skin lesions.
Viius is abundant in skin and oiophaiyngeal
lesions in eaily illness.
Acquiied immunity via cytotoxic T cells and
B cells.
Neutializing antibodies appeai duiing fiist
week of illness.
Coiielation between humoial antibodies and
piotection fiom smallpox is not ceitain.
Death asciibed to toxemia, associated with
immune complexes, and to hypotension.
Infection with smallpox confeis lifelong im-
munity.
CIINICAI FIN0INCS
Iocubatoo Ferod 7-17 days (mean, 10-12).
Frodrome
2-3 days.
Sudden onset of seveie headache, backache,
fevei (40C); subsides ovei 2-3 days.
A piodiomal maculopapulai oi petechial iash
occuiiing in a swimming-tiunk" distiibu-
tion iepoited.
Small ied macules evolve to papules (2-3 mm)
ovei 1-2 days.
In 1-2 moie days, papules become vesicles
(2-5 mm).
Vesicles evolve to pustules (4-6 mm) 4-7 days
aftei onset of iash (Fig. 27-8 , B ), iemain 5-8
days.
Followed by umbilication and ciusting
(Fig. 27-8 C ).
Lesions aie geneially all at the same stage of
development.
Palm/soles lesions peisist longest.
Pockmaiks/pitted scais occui in 65-85% of
seveie cases, especially on the face (Fig. 27-9).
Bacteiial supeiinfections piesent as abscesses,
cellulitis.
Distiibution: Initial lesions on face and
extiemities, then giadually become dissemi-
nated.
Mucvus memhrune Enanthema (tongue,
mouth, oiophaiynx) piecedes exanthem by a
day.
FICk 2T-8 Sma||pox: varo|a major .
\u|||p|e pu|u|e |ecor|u cou||ueu| ou ||e |+ce.
8. \u|||p|e pu|u|e ou ||e ||uu|, +|| |u ||e +re
|+e o| de.e|opreu|. C. \u|||p|e c|u|ed |e+||u
|e|ou ou ||e ||uu|, +|r, |+ud.
SCII0N 2T \|kA| |||ECI|0|' 0| 'K|| A|| \uC0'A T81
Ceoera| Fodos
Vaiiants: Panophthalmitis, keiatitis, second-
aiy infection of eye (1%).
Aithiitis in childien (2%).
Encephalitis (1%)
Seveie chickenpox (vaiicella wheie lesions aie
in diffeient stages of development), human
monkeypox (patients often have lymphadenop-
athy), tanapox, hand-foot-and-mouth disease
(coxsackieviius A-16), insect bites, widespiead
molluscum contagiosum in HIV disease, ad-
veise cutaneous diug eiuption (bullous), sec-
ondaiy syphilis. Histoiically, smallpox has been
confused with chickenpox, syphilis, and mea-
sles.
Specmeos Skin lesions (papulai, vesiculai,
pustulai fluid; ciusts); blood samples; tonsillai
swabbings.
Fo|ymerase Chao keactoo (FCk) Foi
oithopoxviius genes, PCR identfies vaiiola
viius.
Cu|tures Viius isolated on live cell cultuies;
DNA of oithopoxviius identified.
Sero|oy Does not diffeientiate among
oithopoxviius species. Newei methods detect
IgM iesponses.
0ermatopatho|oy Viius ieplicates in basal
epithelium, causing a local cellulai ieaction. In
papulai stage, capillaiy dilatation and edema of
papillaiy deimis.
|ectroo Mcroscopy Biick-shaped viius seen
in negative stains.
0IACN0SIS
An illness with acute onset of fevei 38.3C
(101F) followed by a iash chaiacteiized by
fiim, deep-seated vesicles oi pustules in the
same stage of development without othei
appaient cause.
Iaboratory Crtera Ior CooIrmatoo
PCR identification of vaiiola DNA in a clini-
cal specimen, or
Isolation of smallpox (vaiiola) viius
fiom a clinical specimen Woild Health
Oiganization (WHO) Smallpox Refeience Lab-
oiatoiy oi laboiatoiy with appiopiiate iefeience
capabilities] |us vaiiola PCR confiimation.
MANACMNI
In the United States and most othei
countiies possible smallpox should
be iepoited to state health officials;
diagnosis confiimed in a Biological
Safety Level 4 laboiatoiy wheie staff
membeis have been vaccinated in
the United States. State officials con-
tact Centeis foi Disease Contiol and
Pievention (CDC) (770-488-7100).
CDC infoims WHO Depaitment of
Communicable Disease Suiveillance
and Response Unit.
FICk 2T-9 Sma||pox: scarro
oo Iace A 50,e+|o|d |ud|+u r+|e
W||| + |||o|, o| r+||po\ + + c|||d |+
ru|||p|e dep|eed c+| ou |+ce +0 ,e+|
+||e| r+||po\ |u|ec||ou. (Cou||e, o| A|u|
I+uej+, \|.)
Immuotatoo
Vaccination against smallpox not peifoimed
in the United States since 1972 and in the iest
of woild since 1982.
The gieatei majoiity of the global population
is susceptible to smallpox.
The U.S. militaiy is immunized against small-
pox.
Since the thieat of bioteiioiism in 2001,
selected health caie peisonnel have been vac-
cinated in the United States.
If an outbieak does occui, piompt iecogni-
tion and vaccination would be impoitant.
Vaccination begun 2-3 days aftei exposuie
offeis substantial piotection.
/oouo||c |u|ec||ou
Acc|deu|+||, ||+u|e||ed |o |ur+u ||or +u|r+|
|o|.
k+|e|, c+ue |ur+u |u|ec||ou |u de.e|oped
u+||ou.
w|eu |ur+u |u|ec||ou do occu|, ||e d|||e|eu
||+| d|+uo| |uc|ude |u|ec||ou +oc|+|ed W|||
||o|e||o||r, |.e., r+||po\, +u|||+\, |u|+|er|+,
p|+ue.
|deu||||c+||ou o| po\.||u c+u |e r+de ||or ||u
|e|ou ||ue .||+| cu||u|e, |Ck, |rruuo|||o|o|c
+u+|,|, o| e|ec||our|c|ocop|c re||od.
C0WF0X, M0NkF0X, IANAF0X
Frecautoos
Suspect case should be managed in negative-
piessuie ioom.
Stiict iespiiatoiy and contact isolation
impeiative.
0ru oI Choce Theie is no tieatment
appioved by the U.S. Food and Diug Adminis-
tiation (FDA) foi oithoviiuses. Cidofovii may
be effective.
8actera| SuperoIectoo Usually Sa|y|otot-
tus aureus oi gioup A stieptococcus.
/oouo| o| c+|, coW, |odeu|, +ud occ+|ou+||,
|ur+u.
leuue| ued coWpo\ |o|+|e |o| .+cc|u+||ou.
kee|.o||.
C+|. ro| corrou ou|ce |o| |ur+u |u|ec
||ou
'r+|| |odeu|. .o|e, r|ce
'ou|ce o| ou|||e+| |u coW | uu|uoWu.
|ero|+p|,. occu| |u Eu|ope +ud |u couu|||e
o| ||e |o|re| 'o.|e| uu|ou.
||e+e |u coW. pu|u|e ou |e+|.
||e+e |u c+|. ||||e| +| ||e o| |||e/c|+|c|e.
||e+e |u |ur+u.
|+|u|u| p+pu|e(), W||c| e.o|.e |o .e|c|e |o
ur||||c+|ed pu|u|e (u||ouuded |, eder+/
e|,||er+) |o
Ec|+| o| u|ce|.
\u|||p|e |e|ou occu| ou |+ud/|+ce, W||c|
|eo|.e |u !-+ Wee|.
|,rp|+deuop+||, | corrou.
|e|ou r+, |e e\|eu|.e |u +|op|c de|r+||||.
|uc|deuce r+, |+.e |uc|e+ed due |o d|cou||u
u+||ou o| r+||po\ |rruu|/+||ou +ud |uc|e+ed
uur|e| o| |rruuocorp|or|ed |o|.
C0WF0X |C|9. 05.0

|C|0. B03.0
/oouo| o| c+p||.e p||r+|e +ud |odeu|
nur+u c+e |u A|||c+ +ud |eceu||, |u r|dWe|e|u
uu||ed '|+|e.
I|+ur||ou |o.
nur+u ||or pe| p|+|||e do .|+ opeu Wouud
o| c|+|c| o| |||e
|e|ou|ope|ou ||+ur||ou r+, occu|.
|ucu|+||ou pe||od. +-2+ d+, (red|+u, 5).
nur+u |||ue | c|+|+c|e||/ed |, |e.e|, d|euc||u
We+|, +ud e.e|e c||||
'||u |e|ou e.o|.e ||or p+pu|e |o .e|copu
|u|e |o e|ou|o|ero|||+|c c|u|.
|e|ou occu| ou ||u, coujuuc||.+e, +ud |ucc+|
ruco+.
ke|ou+| |,rp|+deuop+||, c+u occu|.
M0NkF0X |C|9. 051.3

|C|0. B0+
/oouo| o| A|||c+u uou|ur+u p||r+|e +ud
|ur+u.
I|+ur||ou |o |ur+u. uu|uoWu, po|||, |,
roqu||oe ||+| |+.e |ed ou |u|ec|ed rou|e,.
||e+e |u |ur+u.
|e||||e |||ue
|o 0 p|u||||c, |udu|+|ed, ur||||c+|ed p+
pu|e ||+| |ecore uec|o||c W||| |ed |+|o
0ccu| ou e\poed ||e.
|,rp|+deuop+||, | corrou.
IANAF0X |C|9. 013.39

|C|0. B03.3
\+cc|u+||ou ++|u| r+||po\ cou|| o| ||e |u||oduc
||ou o| .+cc|u|+ .||u |u|o ||e ou|e| |+,e| o| |u|+c|
||u.
|oc+| ru|||p||c+||ou o| .||u occu|
|u ore |u|+uce |e|ou+| |,rp|+deuop+||,
',|er|c ,rp|or euue.
I|e |u|ec||ou | + |oc+| oue
ne+| |, c+|||u
||r||ed |, |o| |epoue.
w|dep|e+d +ud e.e|e |u +|op|c de|r+||||
Corp||c+||ou o| .+cc|u+||ou |uc|ude
A||e||c |e+c||ou |o + corpoueu| o| .+cc|ue
B+c|e||+| upe||u|ec||ou
|e|||eu|/p|e+d o| |oc+| .+cc|u|+ |u|ec||ou.
',,. CoWpo\.
|||p.//WWW.||.cdc.o./||+|u|u/r+||po\.+cc|ue/
|e+c||ou/||er+p.||r
vACCINIA
Vaccinia viius, ielated to cowpox viius (see
Cowpox," above).
The oiigin of the stiains of vaccinia viius cui-
iently used foi vaccination is unknown.
Infection with cowpox viius confeis immu-
nity to smallpox.
Ae, Sex Most ieactions occui aftei fiist
(piimo) vaccination.
Seasoo Supeiinfection occuis moie often in
waim weathei.
Iraosmssoo
Iatiogenic inoculation.
Inadveitent tiansmission:
Autoinoculation
Tiansmission to anothei peison
Bioteiioiism is of concein.
FAIh0CNSIS
Norma| keactoo (Fig. 27-10)
Vaccinia ieplicates in the basal layei and dis-
seminates fiom cell to cell, causing neciosis
and foimation of fluid-filled vesicles.
Initial spiead of viius is slowed by innate
antiviial mechanisms, and, by the second
week, the cell-mediated immune iesponse
begins to eliminate infected cells.
Neutiophils, maciophages, and lymphocytes
infiltiate the inoculation site, foiming a con-
fluent pustule and ieleasing cytokines and
chemokines that cause hypeiemia and edema
in suiiounding tissues.
The inflammatoiy piocess peaks 10-12 days
aftei vaccination and begins to iesolve by
day 14, with shedding of the scab by day 21.
This sequence of events, which simulates
the development of a smallpox pock, is
known as a take" ieaction.
A successful take is iequiied foi the
development of antivaccinia antibody and
cell-mediated iesponses.
vACCINAII0N
vaccoe
Inoculation against smallpox is peifoimed
using vaccinia viius.
The vaccine cuiiently available in the United
States, Diyvax (Wyeth), is obtained fiom pus-
tules on inoculated calves.
Neaily all side effects can be piedicted fiom
the unusual natuie of smallpox vaccination,
which essentially employs a small ciicle of
skin as a cultuie plate" in which to giow vac-
cinia viius.
Newei vaccines will be pioduced on cell cul-
tuie.
Two attenuated vaccine stiains have been
developed and tested:
Modified vaccinia Ankaia (MVA)
Japanese stiain (LC16m8).
Method
Vaccine is administeied with the use of a
bifuicated needle, which is dipped into iecon-
stituted vaccine.
15 asseitive jabs into the deimis of the uppei
deltoid aie given in an aiea with a diametei of
about 0.5 cm.
A small amount of blood should appeai at
the vaccination site within 20-25 s.
Cootraodcatoos Because of adveise ieac-
tions, mandatoiy vaccination in the geneial
population of the United States was discontin-
ued in 1972 because the iisk of complications
outweighed the thieat of endemic smallpox.
Since that time, the numbei of immuno-
compiomised peisons has incieased maik-
edly because of spiead of HIV infection
and i ncieased numbeis of patients ieceiving
immunosuppiessive medications. These pei-
sons aie at iisk foi piogiessive vaccinia. (See
also Management," below.)
Vaccination contiaindicated in atopic
deimatitis.
Norma| vaccoatoo keactoo
6-8 days aftei vaccination, loculated pus-
tule (Jenneiian pustule) 1-2 cm in diametei
develops at site (Fig. 27-10).
Cential ciusting begins and spieads peiiphei-
ally ovei 3-5 days.
Local edema and a daik ciust iemain until the
thiid week.
Othei ieactions aie classified as equivocal,
and anothei vaccination is iequiied.
Local satellite" pustules may occui.
SSIMIC MANIFSIAII0NS
Malaise and othei mild constitutional symp-
toms.
Fevei.
Tendei, enlaiged axillaiy lymph nodes.
kACII0NS AN0 C0MFIICAII0NS
0F vACCINIA vIkS
Befoie 1972 in the United States, the iisk of
complication fiom vaccination was 1254 pei
1 million vaccinations. Childien undei the age
of 5 who weie undeigoing piimaiy vaccination
had the highest iates of complications. The
case fatality iate was 1 pei 1 million piimaiy
vaccinations; in 1968, theie weie nine vaccine-
associated deaths.
NoooIectous kashes
Eiythema multifoime-like
Maculai (toxic eiuption")
Maculopapulai; vesiculai
Uiticaiial.
Most common 7-14 days aftei piimovaccina-
tion oi eailiei aftei ievaccination.
NoooIectous Immuoe-Medated
Encephalitis (meningoencephalitis synd-
iome)
Peiicaiditis/myocaiditis.
8actera| SuperoIectoo
Piesents as enlaiging ciusted inoculation site
(impetigo oi ecthyma).
S. aureus , mixed; tetanus.
GAS; Infections occui moie often in pei-
sons with nasal colonization by S. aureus oi
oiophaiyngeal colonization with GAS.
Contamination fiom soil oi dung may iesult
in tetanus.
Othei factois: tiauma to site, maceiation,
manipulation of site.
Accdeota| Ioocu|atoo
Viius usually iemains localized at site of
implantation but may be tiansposed to
noimal oi abnoimal skin/mucosa (buins,
pyodeima, exanthem, eczema, othei dei-
matitides, mucosal, coineal) elsewheie on the
body oi to anothei peison.
Et:ema attnaum. inoculation on site of ec-
zema oi atopic deimatitis iesults in piogies-
sive and often widespiead vaccinia infection.
May occui in vaccinated oi fiom vaccinated
individual.
Mucosal sites: conjunctiva keiatitis; mouth,
aiiway
Cooeota| vaccoa
Vaccination duiing piegnancy may iesult in
dissemination of infection to fetus.
Infant may be stillboin oi develop lesions
shoitly aftei biith.
Ceoera|ted vaccoa
Geneialized vesiculai/pustulai ieaction
Self-limited, usually occuiiing in one ciop.
Usually occuis in a healthy individual whose
antivaccinal antibody iesponse is delayed but
adequate.
FICk 2T-10 Frmary sma||pox vaccoatoo ste reactoo E\pec|ed .+cc|ue ||e |e+c||ou +ud p|o|e
|ou |o||oW|u p||r+|, r+||po\ .+cc|u+||ou o| |e.+cc|u+||ou +||e| + p|o|oued pe||od |e|Weeu .+cc|u+||ou.
\u|||p|e p|eu|e .+cc|u+||ou |ec|u|que ued. ('ou|ce. |||//+++||:!:./c-|/c||/c||c-/
.c|c||)
Almost always benign, with noimal-healing
piimaiy vaccination. May become malignant
with piogiession (see below).
Froressve vaccoa (vaccoa Caoreoosa,
vaccoa Necrosa, 0ssemoated vaccoa)
Incidence duiing univeisal vaccination: 1 pei
million vaccines in geneial population.
Vesicles fail to tiansfoim to pustules by the
end of the fiist week. Vaccination site fails
to heal and continues to enlaige foiming an
ulcei with iaised edges. Relentless outwaid
spiead of infection fiom vaccination site and
eventual dissemination to othei aieas of the
body.
Occuis only in peisons with defective cellulai
immune function.
Congena| mmunoJe[tenty : seveie, com-
bined immunodeficiency.
tqureJ mmunoJe[tenty : HIV dis-
ease, oigan tiansplant iecipient, chionic
immunosuppiession (e.g., connective tissue
disoideis), hypogammaglobulinemia, dys-
globulinemia, malignancies (chionic lym-
phatic leukemia, lymphoma).
Couise is chionic and piogiessive, spiead-
ing deep into tissues and causing neciosis
and osteomyelitis with bacteiial supeiinfec-
tion,.
Couise: Leads to death weeks oi months aftei
vaccination.
Noohea|o[xpaodo Iesoo at vaccoatoo
Ste
Abnoimally laige vaccination lesion: unusu-
ally stiong take ieaction.
Bacteiial supeiinfection with GAS oi S.
aureus , both of which aie accompanied by an
incieased, iathei than diminished, inflamma-
toiy iesponse.
Piogiessive vaccinia: slow pace of develop-
ment, minimal inflammatoiy iesponse.
Cu|ture Detects GAS and S. aureus.
0ermatopatho|oy Vaccinia ieplicates in basal
epithelium, causing a local cellulai ieaction.
0IACN0SIS
Clinical histoiy, physical examination, and
clinical couise. Peisistence of viius can be
confiimed by cultuiing vaccinia viius fiom the
skin lesions.
C0kS AN0 Fk0CN0SIS
Norma| vaccoatoo keactoo
A Jenneiian pustule is classified as a majoi
ieaction, indicating successful piimaiy vac-
cination; successful ievaccination is indicated
by palpable inflammation at 6-8 days.
A successful vaccination confeis full immunity
to smallpox in 95% of peisons foi 5-10 yeais.
Successful ievaccination piobably piovides
piotection foi 10-20 yeais.
Reactions othei than a Jenneiian pustule aie
classified as equivocal, and anothei vaccina-
tion is iequiied.
Froressve vaccoa
Lethal in infants who completely lack cellulai
immune function.
Infection in adults with HIV/AIDS: may
iesolve if tieated with vaccinia immunoglob-
ulin (VIg).
MANACMNI
Cootraodcatoo to vaccoatoo
If in doubt, don`t vaccinate.
Any abnoimality of skin in vaccinated individ-
ual oi in contacts: atopic deimatitis, eczema,
buins, lichen simplex chionicus, pyodeimas.
Any immunologic defect/disoidei, any
hematologic disoideis involving white blood
cells, any inflammatoiy lesions of peiioibital
stiuctuies, GAS oi S. aureus caiiiei states, any
acute febiile illness, exposuie to oi incuba-
tion of exanthematous disease, family his-
toiy of febiile convulsions oi of postvaccinal
encephalitis.
vI
Available fiom the CDC thiough state health
depaitments foi tieatment of seveie compli-
cations.
May be beneficial in management of acciden-
tal inoculation, eczema vaccinatum, geneial-
ized vaccinia.
SCII0N 2T \|kA| |||ECI|0|' 0| 'K|| A|| \uC0'A T8T
Response of piogiessive vaccinia not well
documented.
Administeied IM 0.6 mL/kg.
Aotvra| 0ru Cidofovii is piotective against
oithopoxviius in animals.
Immuoomodu|ators
The TH1 cytokines inteileukin 2 and intei-
feion stimulate oithopoxviius cleaiance.
Local oi systemic tieatment with immu-
nomodulatois that potentiate a TH1 iesponse
could help suppiess vaccinia infection in
immunodeficient patients.
Immuoosuppressoo Tapeiing oi discontinua-
tion of immunosuppiessive theiapies if patients
aie iatiogenically immunosuppiessed.
Myopercardts High-dose glucocoiticoids.
nur+u p+p|||or+.||ue (n|\) +|e u||qu||ou |u
|ur+u, c+u|u
'u|c||u|c+| |u|ec||ou
w|de .+||e|, o| |eu|u c||u|c+| |e|ou ou ||u
+ud rucou rer||+ue.
I|e, +|o |+.e + |o|e |u ||e oucoeue| o| cu|+ue
ou +ud ruco+| p|er+||u+uc|e (I+||e 21-2).
'qu+rou ce|| c+|c|uor+ |u ||u ('CC|')
|u.+|.e 'CC
\o|e ||+u 50 |,pe o| n|\ |+.e |eeu |deu||||ed
+ud +|e +oc|+|ed W||| .+||ou c||u|c+| |e|ou
+ud d|e+e
Cu|+ueou n|\ |u|ec||ou occu| corrou|, |u ||e
eue|+| popu|+||ou.
Corrou W+||. kep|eeu| +pp|o\|r+|e|, 10
o| +|| cu|+ueou W+||, occu|||u |u up |o 20
o| +|| c|oo|+e c|||d|eu.
Bu|c|e|' W+||. Corrou |u |u|c|e|, re+|
p+c|e|, ||| |+ud|e|.
||+u|+| W+||. Corrou |u o|de| c|||d|eu +ud
,ouu +du||, +ccouu||u |o| !0 o| cu|+ueou
W+||.
||+| W+||. 0ccu| |u c|||d|eu +ud +du||,
+ccouu||u |o| + o| cu|+ueou W+||.
0ucoeu|c n|\ c+u c+ue
'CC|' +ud |u.+|.e 'CC |u |rruuocorp|o
r|ed |o|, epec|+||, |u ||oe W|||
n|\/A||'
'o||d o|+u ||+up|+u| |ec|p|eu|
Ep|de|rod,p|+|+ .e||uc||o|r| (E|\).
\uco+| W+||.
Coud,|or+ +cur|u+|ur (eu||+| W+||)
\o| p|e.+|eu| e\u+||, ||+ur|||ed |u|ec||ou
(ee 'ec||ou !0).
'ore n|\ |,pe |+.e + r+jo| e||o|o|c |o|e |u
||e p+||oeue| o| |u ||u + We|| + |u.+|.e
'CC o| ||e +uoeu||+| ep|||e||ur.
|u||u de||.e|,, r+|e|u+| eu||+| n|\ |u|ec||ou
c+u |e ||+ur|||ed |o ||e ueou+|e, |eu|||u |u
Auoeu||+| W+||
kep||+|o|, p+p|||or+|o| +||e| +p||+||ou o|
||e .||u |u|o ||e uppe| |ep||+|o|, ||+c|.
hMAN FAFIII0MAvIkS INFCII0NS |C|9 . 019.+
|C|0 . B91.1
II0I0C
Papillomaviiuses aie double-stiand DNA
viiuses of the papovaviius class, which infect
most veitebiate species with exclusive host
and tissue specificity.
They infect squamous epithelia of skin and
mucous membianes.
Clinical lesions induced by HPV and its natu-
ial histoiy aie laigely deteimined by HPV
type.
HPV aie noimally giouped accoiding to theii
pathologic associations and tissue specifi-
city-eithei cutaneous oi mucosal.
The 23 mucosal-associated HPV can be fui-
thei subgiouped accoiding to theii iisk of
malignant tiansfoimation.
New types of HPV aie defined as possessing
90% homology to known types in six speci-
fied eaily and late genes.
Iraosmssoo
Skin-to-skin contact.
Minoi tiauma with bieaks in stiatum coi-
neum facilitates epideimal infection.
Contagion occuis in gioups-small (home)
oi laige (school gymnasium).
Ce||+|u |ur+u n|\ |,pe corrou|, |u|ec| |e|+||
u|/ed ||u.
Cu|+ueou W+|| +|e.
||c|e|e |eu|u ep|||e||+| |,pe|p|+|+ W||| .+|,
|u de|ee o| u||+ce |,pe||e|+|o|
\+u||e|ed + r|uu|e p+pu|e |o |+|e p|+que
|e|ou r+, |ecore cou||ueu|, |o|r|u + ro
+|c.
I|e e\|eu| o| |e|ou | de|e|r|ued |, ||e |r
ruue |+|u o| ||e |o|.
',,. \e||uc+, r,|rec|+.
hMAN FAFIII0MAvIkS: CIAN0S INFCII0NS
IA8I 2T-2 Corre|ation of human Papi||omavirus Iype with 0isease
0|sease Assoc|ated hPV Types
||+u|+| W+|| ,
*
2,
+, o!
\,|rec|+ o0
Corrou W+|| ,
*
2,
*
+, 2o, 21, 29, +,
51, o5, 11
Corrou W+|| o| re+| |+ud|e| , 2,
*
!, +, 1,
*
0, 23
||+| W+|| !,
*
0,
*
21, !3, +,
+9, 15, 1o
|u|e|red|+|e W+|| 0,
*
2o, 23
Ep|de|rod,p|+|+ .e||uc||o|r| 2,
*
!,
*
5,
*
3,
*
9,
*
0,
*
2,
*
+,
*
5,
*
1,
*
9, 20,
2, 22, 2!, 2+, 25,

!o, !1, !3,
+1, 50
Coud,|or+ +cur|u+|ur o,
*
,
*
!0,
+2, +!, ++, +5,
5,
5+, 55, 10
|u||+ep|||e||+| ueop|+|+
uupec|||ed !0,
!+, !9,
+0, 5!, 51, 59, o, o2, o+, oo,
o1, o9, 1
|oW|+de o,
*
,
*
o,
3,
!,
!!,
!5,
+2, +!, ++, +5,
5,
52,
1+
n|||+de o, , o,
*
3,
*
!,
!!,
!+, !5,
!9,
+2, ++, +5,
5,
52,
5o,
53,
oo,
Ce|.|c+| c+|c|uor+ o,
*
3,
*
!,
!!,
!5,
!9,
+5,
5,
52,
5o,
53,
oo,
o3, 10
|+|,ue+| p+p|||or+ o,
*

*
|oc+| ep|||e||+| |,pe|p|+|+ o| nec| !,
*
!2
*
Coujuuc||.+| p+p|||or+ o,
*
,
*
o
*
0||e| o, , o,
!0,
!!,
!o, !1, !3,
+,
+3,
o0, 12, 1!
*
\o| corrou +oc|+||ou.
n|| r+||u+u| po|eu||+|.

|0IE. Add|||ou+| |u|o|r+||ou ou ueW n|\ |,pe c+u |e |ouud ou ||e n|\ 'equeuce |+|+ B+e |||ou| ||e |u|e|ue| (|.+-||c|.).
'0ukCE. ||or kC ke|c|r+u, |u E B|+uuW+|d e| +| (ed). |c |:|- | ||-c| !-!:-, 5|| ed. |eW \o||, \cC|+Wn|||, 200.
0ther Factors
Immunocompiomise associated with an in-
cieased incidence of and moie widespiead
cutaneous waits:
HIV/AIDS
Iatiogenic immunosuppiession with solid
oigan tiansplantation.
Occupational iisk associated with meat han-
dling.
Epideimodysplasia veiiucifoimis (EDV, see
below): most commonly autosomal iecessive.
0uratoo oI Iesoos Waits often peisist foi
seveial yeais if not tieated.
Symptoms
Cosmetic disfiguiement.
Plantai waits act as a foieign body and can be
quite painful duiing noimal daily activities,
such as walking, if located ovei piessuie points.
Moie aggiessive theiapies such as ciyosuigeiy
often iesult in much moie pain than that
caused by the wait itself.
Bleeding, especially aftei shaving.
Sko Iesoos
Verrucu Vu|gurIs (Cvmmvn Wurt)
Fiim papules, 1-10 mm oi iaiely laigei
(Fig. 27-11), hypeikeiatotic, clefted suiface,
with vegetations.
Palmai lesions disiupt the noimal line of fin-
geipiints (Fig. 27-12). Retuin of fingeipiints
is a sign of iesolution of the wait.
FICk 2T-11 verruca vu|ars: thumb A 25,e+|o|d r+|e W||| |,pe||e|+|o||c (.e||ucou) p+pu|e ou
||e do|+| ||ur|. I|e d+|| po|u| |ep|eeu| |||or|oed c+p|||+||e. I|e |e|ou |eo|.ed W||| e|ec||ode|c+||ou,
|+.|u |+||ed |o |epoud |o c|,ou|e|,.
FICk 2T-12 verruca vu|ars: haods A 20,e+|o|d |rruuoupp|eed r+|e W||| uep||o||c ,ud|ore.
\u|||p|e .e||uc+e ou ||e (} do|ur +ud (8} p+|r o| ||e |+ud.
8
Chaiacteiistic ied oi biown dots" aie bettei
seen with hand lens and aie pathognomonic,
iepiesenting thiombosed capillaiy loops.
Isolated lesion, scatteied disciete lesions.
Lnear arrangemen : inoculation by sciatch-
ing.
nnu|ar wars : at sites of piioi theiapy (Fig.
27-13).
Occui at sites of tiauma: hands, fingeis,
knees.
But|er's wars : laige cauliflowei-like lesions
on hands of meat handleis.
F|[orm wars have ielatively small bases,
extending out with elongated cap.
Verrucu P|unturIs (P|untur Wurt)
Eaily small, shiny, shaiply maiginated papule
(Fig. 27-14) plaque with iough hypeikeia-
totic suiface, studded with biown-black dots
(thiombosed capillaiies).
As with palmai waits, noimal deimatoglyph-
ics aie disiupted. Retuin of deimatoglyphics
is a sign of iesolution of the wait.
Waits heal without scaiiing.
Theiapies such as ciyosuigeiy and electiosui-
geiy can iesult in scaiiing at tieatment sites.
Tendeiness may be maiked, especially in
ceitain acute types and in lesions ovei sites of
piessuie (metataisal head).
Mosat wars : Confluence of many small waits
(Fig. 27-14).
Kssng" wars : lesion may occui on opposing
suiface of two toes (Fig. 27-15).
Plantai foot, often solitaiy but may be thiee
to six oi moie.
Piessuie points, heads of metataisal, heels, toes.
Verrucu P|unu (F|ut Wurt)
Shaiply defined, flat papules (1-5 mm); flat"
suiface; the thickness of the lesion is 1-2 mm
(Fig. 27-16).
Skin-coloied oi light biown.
Round, oval, polygonal, lineai lesions (inocu-
lation of viius by sciatching).
Occui on face, beaid aiea (Fig. 27-17), doisa
of hands (Fig. 27-18), shins.
EpIdermvdysp|usIu VerrucI]vrmIs
Autosomal iecessive condition.
Flat-topped papules.
Pityiiasis veisicoloi-like lesions, paiticulaily
on the tiunk.
FICk 2T-13 verruca vu|ars: aoou|ar wart A 2+,e+|o|d W||| +u +uuu|+| W+|| ou ||e do|ur o| ||e
||ue|. I|e W+|| o|||u+||, +| ||| ||e W+ ruc| r+||e| +ud |+d |eeu ||e+|ed W||| c|,ou|e|,. I|e ||e+|ed ||e
|rp|o.ed |u| ||e W+|| e\p+uded |+d|+||,, c|e+||u ||e ||u.
FICk 2T-14 verruca p|aotars: p|aotar Ieet A 1,e+|o|d r+|e W||| c||ou|c |,rp|+||c |eu|er|+. |+|e
+ud p+|u|u| ou p|eu|e, W+|| +|e eeu ou ||e +u|e||o| |ee| +ud ||e |oe. \u|||p|e W+|| We|e +|o p|eeu| ou ||e
||ue|. A||e| r+u, |+||ed ||e|+peu||c rod+||||e, |e W+ ucce|u||, ||e+|ed W||| e|ec||ou |e+r |+d|+||ou.
FICk 2T-15 verruca p|aotars: "ksso warts" A +9,e+|o|d r+|e W||| n|\/A||' |+ cou||ueu| W+||
ou ||e |+ud +ud |ee|. I|e |+|e W+|| ou oppo|u |oe +|e |e|e||ed |o + '|||u W+||.
FICk 2T-16 verruca p|aoa A 2,e+|o|d r+|e ||due, ||+up|+u| |ec|p|eu|. \u|||p|e ||oWu |e|+|o||c p+p
u|e +|e eeu ou ||e |o|e|e+d +ud c+|p.
FICk 2T-1T verruca vu|ars A !3,e+|o|d r+|e W||| n|\/A||'. Cou||ueu| ||uco|o|ed p+pu|e |u ||e
|e+|d +|e+. |e|ou |eo|.ed +||e| ucce|u| +u|||e||o.||+| ||e|+p, (AkI).
Coloi: skin-coloied, light biown, pink, hypo-
pigmented.
Lesions may be numeious, laige, and confluent.
Seboiiheic keiatosis-like and actinic
keiatosis-like lesions.
Lineai aiiangement aftei tiaumatic inoculation.
Dsr|uon : face, doisa of hands (Fig. 27-18),
aims, legs, anteiioi tiunk.
Piemalignant and malignant lesions aiise
most commonly on face.
SCC: in situ and invasive.
hIv 0sease, Iatroeoc Immuoosuppressoo
HPV-induced waits aie common and may be
difficult to tieat successfully.
Some have atypical histologic featuies and
may piogiess to in situ and invasive SCC.
verruca vu|ars Molluscum contagiosum,
seboiiheic keiatosis, actinic keiatosis, keiato-
acanthoma, SCCIS, invasive SCC.
verruca F|aotars Callus, coin (keiatosis), have
no thiombosed capillaiy loops, exostosis.
verruca F|aoa Syiingoma (facial), molluscum
contagiosum.
pdermodysp|asa verrucIorms Pityiiasis
veisicoloi, actinic keiatoses, seboiiheic keia-
toses, SCCIS, basal cell caicinoma.
FICk 2T-18 pdermodysp|asa verrucIorms-|ke |esoos A !5,e+|o|d |er+|e |uu ||+up|+u|
|ec|p|eu|. \u|||p|e, ||+||opped, p|u| p+pu|e W||| |+|p r+||u+||ou +ud r|u|r+| |,pe||e|+|o| ou ||e do|+ o|
||e |+ud +ud ||ue|. I|e |+pe | +| ||e ||e o| + ||op, ||+| |oWed qu+rou ce|| c+|c|uor+ |u ||u +|||u |u +
||+|W+|| |e|ou. \u|||p|e |e|ou We|e +|o p|eeu| ou ||e |e.
MANACMNI
Coa| A|e|.e ||e|+p|e, W||c| +|e o||eu qu||e p+|u|u| +ud r+, |e |o||oWed |, c+|||u,
+|e uu+||, |o |e +.o|ded |ec+ue ||e u+|u|+| |||o|, o| cu|+ueou n|\ |u|ec||ou
| |o| pou|+ueou |eo|u||ou |u rou|| o| + |eW ,e+|. ||+u|+| W+|| ||+| +|e
p+|u|u| |ec+ue o| ||e|| |oc+||ou W+||+u| ro|e +|e|.e ||e|+p|e.
Fateot-otated therapy \|u|r+| co|, uo/r|u|r+| p+|u.
|o| r+|| |e|ou 0-20 +||c,||c +c|d +ud |+c||c +c|d |u co||od|ou.
|o| |+|e |e|ou +0 +||c,||c +c|d p|+|e| |o| Wee|, ||eu +pp||c+||ou o| +||c,||c +c|d-|+c||c +c|d
|u co||od|ou.
|r|qu|rod c|e+r A| ||e ||+| +|e uo| |||c||, |e|+||u|/ed, +pp|, |+||||eu|| ! ||re pe| Wee|.
|e|||eu| W+|| r+, |equ||e occ|u|ou. n,pe||e|+|o||c |e|ou ou p+|r/o|e
|ou|d |e de|||ded ||equeu||,, |r|qu|rod ued +||e|u+|e|, W||| + |op|c+| |e||uo|d
uc| + |+|+|o|eue |op|c+| e| r+, |e e||ec||.e.
n,pe|||e|r|+ |o| n,pe|||e|r|+ W||| |o| W+|e| +5C (!|)| |rre||ou |o| 20 r|u |Wo o| |||ee
.e||uc+ p|+u|+|| ||re Wee||, |o| up |o o ||e+|reu| | e||ec||.e |u ore p+||eu|.
C|ocao-otated therapy Co||,, p+|u|u|.
C|,ou|e|, || p+||eu| |+.e |||ed |ore ||e|+p|e +ud ||qu|d u|||oeu | +.+||+||e, ||||
c|,ou|e|, u|u + co||ou||pped +pp||c+|o| o| c|,op|+,, ||ee/|u ||e W+|| +ud
-2 rr o| u||ouud|u uo|r+| ||ue |o| +pp|o\|r+|e|, !0 , | qu||e e||ec||.e.
||ee/|u |||| ||e |u|ec|ed ||ue |u| uo| n|\.
C|,ou|e|, | uu+||, |epe+|ed +|ou| e.e|, + Wee| uu||| ||e W+|| |+.e
d|+ppe+|ed. |+|u|u|.
E|ec||ou|e|, \o|e e||ec||.e ||+u c|,ou|e|,, |u| +|o +oc|+|ed W||| + |e+|e| c|+uce o|
c+|||u. E\|A c|e+r c+u |e ued |o| +ue||e|+ |o| ||+| W+||. ||doc+|ue
|ujec||ou | uu+||, |equ||ed |o| |||c|e| W+||, epec|+||, p+|r+|/p|+u|+| |e|ou.
C0
2
|+e| u|e|, \+, |e e||ec||.e |o| |ec+|c|||+u| W+||, |u| uo |e||e| ||+u c|,ou|e|, o|
e|ec||ou|e|, |u ||e |+ud o| +u e\pe||euced c||u|c|+u.
'u|e|, '|u|e, uoup|+u|+| .e||uc+ .u|+||.cu|e||+e +||e| ||eou ||ee/|u, u||c+| e\c||ou
o| cu|+ueou n|\ |u|ec||ou | uo| |ud|c+|ed |u ||+| ||ee |e|ou +|e ep|de|r+|
|u|ec||ou.
h0NAh PAP|LL0NAV|80S: N000SAL
|hF0T|0hS
'ee 'ec||ou !0, 'e\u+||, I|+ur|||ed ||e+e
0ermatopatho|oy Acanthosis, papillomato-
sis, hypeikeiatosis. Chaiacteiistic featuie is foci
of vacuolated cells (koilocytosis), veitical tieis
of paiakeiatotic cells, foci of clumped keiato-
hyaline gianules.
0IACN0SIS
In the immunocompiomised host, HIV-
induced SCC at peiiungual sites oi anogenital
iegion should be iuled out by lesional biopsy.
C0kS AN0 Fk0CN0SIS
Immunocompetent individuals: Cutaneous
HPV infections usually iesolve spontane-
ously, without theiapeutic inteivention.
Immunocompiomised individuals: Cutane-
ous HPV infections may be veiy iesistant to
all modalities of theiapy.
EDV: Lesions fiist occui at 5-7 yeais of age;
lesions appeai piogiessively, becoming wide-
spiead in some. 30-50% of individuals with
EDV develop malignant cutaneous lesions on
aieas of skin exposed to sunlight.
Au |u|ec||ou e\+u||er (|E) | + eue|+||/ed
cu|+ueou e|up||ou +oc|+|ed W||| + p||r+|,
,|er|c |u|ec||ou.
0||eu +ccorp+u|ed |, o|+| ruco+| |e|ou, |.e.,
+u eu+u||er+.
|E +|e ro| corrou|, c+ued |, .||+| +eu| |u|
c+u +|o |e +oc|+|ed W|||
B+c|e||+|
k|c|e|||+|
|+|+|||c |u|ec||ou
Ce||+|u |E |+.e |+|||, c|+|+c|e||||c ro|p|o|
o,, |u| |u r+u, c+e +u +ccu|+|e d|+uo|
c+uuo| |e r+de ou ||e |+| o| ro|p|o|o,
+|oue.
n||o||c |+c|o| r+, |e |e|p|u|, |uc|ud|u e+ou,
d|e+e cou|+c|, |rruu|/+||ou, p|e.|ou e\+u
||er+|ou |||uee, +ud +oc|+|ed p|od|or+|
,rp|or.
INFCII0S XANIhMS |C|9. 132.

|C|0. B03
Ae oI 0oset Usually 20 yeais.
to|oy
RNA VIruses
Picoinaviiidae
Poliovuius
Coxsackieviiuses
Echoviius
Enteioviius
Hepatitis A viius
Rhinoviius
Togaviiidae
Rubella viius, attenuated iubella viius in
vaccine
Flaviviiidae
Dengue
Hepatitis C
Paiamyxoviiidae
Measles
Mumps
Oithomyxoviiidae
Influenza A, B, and C viiuses
Retioviiidae
Human T-lymphotiophic viius types I
and II
Human immunodeficiency viiuses (HIV)
types 1 and 2
Acute HIV syndiome
DNA vIruses
Paivoviiidae
Paivoviius B19 (eiythema infectiosum)
Hepadnaviiidae
Hepatitis B viius
Adenoviiidae
Heipesviiidae
Heipes simplex viius (HSV) types 1 and 2
Vaiicella zostei viius (VZV)
Cytomegaloviius (CMV)
Epstein-Baii viius (EBV)
Human heipesviius (HHV) 6 and 7 (exan-
them subitum, ioseola infantum)
Kaposi saicoma-associated viius (HHV-8)
Poxviiidae
Vaiiola (smallpox) viius
Oif viius
Molluscum contagiosum viius
BucterIu|
Gioup A stieptococcus: scailet fevei, toxic
shock syndiome
S. aureus : toxic shock syndiome
Legone||a
Leosra
Lsera
Meningococci
Mycvp|usmu|
Myto|asma neumonae
RIc|ettsIu|
Rocky Mountain spotted fevei
Tick-boine spotted feveis
Rickettsialpox
Muiine typhus
Epidemic typhus
MIsce||unevus
Srongy|oJes
Toxo|asma
Treonema a||Jum
Iraosmssoo Respiiatoiy, food, sexual, blood.
Seasoo Enteioviius infections: summei
months.
Ceoraphy Woildwide.
FAIh0CNSIS
Skin lesions may be pioduced by the following:
Diiect effect of miciobial ieplication in
infected cells
Host iesponse to the miciobe
Inteiaction of these two phenomena.
Iocubatoo Ferod Usually 3 weeks; hepatitis B
viius, seveial months.
Frodrome Fevei, malaise, coiyza, soie thioat,
nausea, vomiting, diaiihea, abdominal pain,
headache.
Diffuse eiythema, scailatinifoim"
Maculopapulai, moibillifoim" eiuptions
Vesiculai eiuptions
Occasionally petechiae
Mucosal: micioulceiative lesions, palatal pe-
techiae, conjunctivitis
Confluent pink papules (moibillifoim,
iubeola-like, measles-like)
Synonyms : Exanthematous, maculopapulai
Eiythematous macules and/oi papules (Fig.
27-19)
Usually cential, i.e., head, neck, tiunk, piox-
imal extiemities.
FICk 2T-19 IoIectous exaothem ||er|u+|ed, e|,||er+|ou r+cu|e +ud p+pu|e, |,p|c+| o| ||e cu|+
ueou c|+ue W||| r+u, +cu|e |u|ec||ou. I|e e|up||ou ru| |e d|||e|eu||+|ed ||or +u e\+u||er+|ou (ro||||
|||o|r) d|u e|up||ou. . I,p|c+| d|||||u||ou o| |e|ou ou ||e ||uu| +ud e\||er|||e. 8. C|oeup o| p|u| r+cu|e
+ud p+pu|e |ecor|u cou||ueu| |u ore +|e+.
SCII0N 2T \|kA| |||ECI|0|' 0| 'K|| A|| \uC0'A T9T
Occasionally petechiae, hemoiihagic measles
Vesiculai
Initially, vesicles with cleai fluid.
May evolve to pustules.
In a few days to a week, ioof of vesicle
sloughs, iesulting in eiosions.
In vaiicella, lesions aie disseminated and
may involve oiophaiynx.
In hand-foot-and-mouth disease, vesicles/
eiosion occui in oiophaiynx; painful lineai
vesicles on palms/soles.
Mucous membianes
Koplik spots in measles
Micioulceiative lesions in heipangina due
to coxsackieviius A (Fig. 27-20)
Palatal petechiae in mononucleosis syn-
diome of EBV oi CMV
Conjunctivitis, e.g., measles
Systemc Fodos
Lymphadenopathy
Hepatomegaly
Splenomegaly.
xaothematous ruptoo Diug eiuption,
systemic lupus eiythematosus, Kawasaki
syndiome.
Cu|tures If piactical.
Sero|oy Acute and convalescent titeis most
helpful in specific diagnosis.
0IACN0SIS
Usually made on histoiy and clinical findings.
C0kS AN0 Fk0CN0SIS
Usually iesolves in 10 days.
MANACMNI
Symptomatic.
Aotmcroba| Iherapy Specific antimiciobial
theiapy when available.
FICk 2T-20 IoIectous eoaothem: herpaooa \u|||p|e, r+|| .e|c|e +ud e|o|ou W||| e|,||er+|ou
|+|o ou ||e o|| p+|+|e, ore |+|e |ud ou ||e po|e||o| |ouue +|e |u||+red +ud p|or|ueu|.
Ae oI 0oset
Befoie widespiead immunization, childien
15 yeais.
Cuiiently, young adults.
to|oy
Ru|e||a rus , an RNA togaviius, membei of
Ru|rus genus.
Attenuated iubella viius used in immuniza-
tion can cause an illness with iubella-like
iash, lymphadenopathy, and aithiitis.
0ccupatoo Young adults in hospitals, colleges,
piisons, pienatal clinics.
Iraosmssoo
Inhalation of aeiosolized iespiiatoiy dioplets
Modeiately contagious
10-40% of cases asymptomatic
Peiiod of infectivity fiom end of incubation
peiiod to disappeaiance of iash.
ksk Factors
Lack of active immunization
Lack of natuial infection.
Aftei immunization began in 1969, incidence has
decieased by 99% in industiialized countiies.
Seasoo Befoie 1969, epidemics in the United
States eveiy 6-9 yeais, occuiiing in spiing.
Ceoraphy Woildwide. Maiked ieduction in
incidence in industiialized countiies aftei im-
munization.
Frodrome
Usually absent, especially in young childien.
E||o|o|c +eu|. ku|e||+ .||u, +u k|A |o+.||u
A .||+| |u|ec||ou o| c|||d|eu +ud +du||
C|+|+c|e||||c e\+u||er +ud |,rp|+deuop+||,.
\+u, |u|ec||ou +|e u|c||u|c+|.
ku|e||+ .||u |u|ec||u + p|eu+u| |er+|e, W|||e
c+u|u + |eu|u |||ue |u ||e ro||e|, r+, |eu||
|u ||e coueu||+| |u|e||+ ,ud|ore W||| e||ou
c||ou|c |e|+| |u|ec||ou +ud r+||o|r+||ou.
C|||d|ood |rruu|/+||ou | ||||, e||ec||.e +|
p|e.eu||u |u|ec||ou.
',, . Ce|r+u re+|e, '!d+, re+|e.
k8IIA
In adolescents and young adults: anoiexia,
malaise, conjunctivitis, headache, low-giade
fevei, mild uppei iespiiatoiy tiact symp-
toms.
hstory Aithialgia, especially in adult women
aftei immunization.
Immuoe Status In women, iubella-like illness
fiequently follows administiation of attenuated
live iubella viius.
Mucocutaoeus Iesoos
Pink macules, papules (Fig. 27-21 ).
Initially on foiehead, spieading infeiioily to
face, tiunk, and extiemities duiing fiist day.
By second day, facial exanthem fades.
By thiid day, exanthem fades completely
without iesidual pigmentaiy change oi scal-
ing.
Tiunkal lesions may become confluent, cieat-
ing a scailatinifoim eiuption.
Mucous membianes
Petechiae on soft palate (Foichheimei sign)
duiing piodiome (also seen in infectious
mononucleosis).
Ceoera| xamoatoo
Lym| noJes :
Enlaiged duiing piodiome.
Postauiiculai, suboccipital, and posteiioi
ceivical lymph nodes enlaiged and possibly
tendei.
Mild geneialized lymphadenopathy may
occui.
Enlaigement usually peisists foi 1 week but
may last foi months.
S|een :
May be enlaiged.
Jons :
Aithiitis in adults; possible effusion.
Aithialgia, especially in adult women aftei
immunization.
xaothem Othei infectious exanthems,
adveise diug eiuption, scailet fevei, eiythema
infectiosum, enteioviial infection.
xaothem wth Arthrts Acute iheumatic fevei,
iheumatoid aithiitis, eiythema infectiosum.
Sero|oy Acute and convalescent iubella anti-
body titeis show fouifold oi gieatei iise.
Cu|ture Viius can be isolated fiom thioat,
joint fluid aspiiate.
0IACN0SIS
Clinical diagnosis; can be confiimed by seiol-
ogy.
C0kS AN0 Fk0CN0SIS
In most peisons, iubella is a mild, incon-
sequential infection.
Howevei, when iubella occuis in a piegnant
woman duiing the fiist tiimestei, the infec-
tion can be passed tiansplacentally to the
developing fetus.
Appioximately half of infants who acquiie
iubella duiing the fiist tiimestei of intiau-
teiine life will show clinical signs of damage
fiom the viius.
Manifestations of the congenital iubella
syndiome aie
Congenital heait defects
Cataiacts
Miciophthalmia, miciocephaly, hydio-
cephaly, deafness
MANACMNI
Freveotoo
Rubella is pieventable by immunization.
Pievious iubella should be documented in
young women: if antiiubella antibody titeis
aie negative, iubella immunization should be
given.
Acute IoIectoo Symptomatic.
FICk 2T-21 kube||a
A 2,e+|o|d r+|e. E|,||er+|ou
r+cu|e +ud p+pu|e +ppe+||u
|u|||+||, ou ||e |+ce +ud p|e+d|u
|u|e||o||, |o ||e ||uu| +ud e\||er|
||e, uu+||, W||||u ||e |||| 2+ |.
|o|+u||cu|+| +ud po|e||o| ce|.|c+|
|,rp| uode We|e eu|+|ed. |e|ou
|ecor|u cou||ueu| ou ||e c|ee|
W|||e c|e+||u ou ||e |o|e|e+d. I|uu
c+| |e|ou +ppe+| 2+ | +||e| oue| o|
|+c|+| |e|ou.
Ae oI 0oset
Befoie measles immunization: 5-9 yeais of
age in the United States.
In developing countiies, up to 45% of cases
occui befoie the age of 9 months.
to|oy Measles viius, membei of RNA genus
Mor|||rus and family Paiamyxoviiidae.
Iocdeoce
United States, Euiope, Canada, Japan
No longei endemic
Cases iesult fiom inteinational impoita-
tion.
Vor|JwJe . hypeiendemic in many devel-
oping nations, iesulting in 800,000 deaths
annually.
ksk Factors
Aftei immunization began in 1963, incidence
has decieased by 98%.
Cuiient outbieaks in the United States
occui in innei-city unimmunized pie-
school-age childien, school-age peisons
immunized at an eaily age, and impoited
cases.
Most outbieaks aie in piimaiy oi second-
aiy schools, colleges oi univeisities, day-caie
centeis.
Iraosmssoo
Spiead by iespiiatoiy dioplet aeiosols pio-
duced by sneezing and coughing.
Infected peisons contagious fiom seveial days
befoie onset of iash up to 5 days aftei lesions
appeai.
Attack iate foi susceptible contacts
90-100%.
Asymptomatic infection iaie.
A ||||, cou|+|ou c|||d|ood .||+| |u|ec||ou
C|+|+c|e||/ed |,
|e.e|, co|,/+, cou|
Au e\+u||er
Coujuuc||.|||
|+||ouorou|c eu+u||er (Kop||| po|)
'|u|||c+u| ro|||d||, +ud ro||+|||, occu| |u +cu|e
+ud c||ou|c cou|e.
C|||d|ood |rruu|/+||ou | ||||, e||ec||.e +|
p|e.eu||u |u|ec||ou.
',, . ku|eo|+, ro||||||.
|u A|||c+.
MASIS * *
Seasoo Befoie widespiead use of vaccine, epi-
demics occuiied eveiy 2-3 yeais in late wintei
to eaily spiing.
0emoraphy
No longei endemic in Ameiicas and Eu-
iope.
Hypeiendemic in Afiica, with 800,000 deaths
annually.
FAIh0CNSIS
Viius enteis cells of iespiiatoiy tiact, iepli-
cates locally, spieads to local lymph nodes,
and disseminates hematogenously to skin and
mucous membianes.
Viial ieplication also occuis on skin and mu-
cosa.
Modified measles, a mildei foim of the illness,
may occui in individuals with pieexisting
paitial immunity induced by active oi passive
immunization.
Peisons deficient in cellulai immunity aie at
high iisk foi seveie measles.
Frodrome
Fevei
Malaise
Uppei iespiiatoiy symptoms (coiyza, hack-
ing baiklike cough)
Photophobia, conjunctivitis with laciimation
Peiioibital edema.
As exanthem piogiesses, systemic symptoms
subside.
Exanthem
SCII0N 2T \|kA| |||ECI|0|' 0| 'K|| A|| \uC0'A 801
On the fouith febiile day, eiythematous
macules and papules.
Appeai on foiehead at haiiline, behind eais;
spiead centiifugally and infeiioily to involve
the face, tiunk (Fig. 27-22), extiemities,
palms/soles, ieaching the feet by thiid day.
Initial disciete lesions may become conflu-
ent, especially on face, neck, and shouldeis.
Lesions giadually fade in oidei of appeai-
ance, with subsequent iesidual yellow-tan
stain oi faint desquamation.
Exanthem iesolves in 4-6 days.
Mucous membianes (enanthem)
Oro|arynx/Koplik spots:
Pathognomonic.
Appeai befoie exanthem.
Clustei of tiny bluish-white spots on ied
backgiound, appeaiing on oi aftei second
day of febiile illness, on buccal mucosa
opposite piemolai teeth.
Also: entiie buccal/innei labial mucosa
may be inflamed; lips ied.
Bu||ar ton,untae : conjunctivitis.
Ceoera| xamoatoo
Geneialized lymphadenopathy
Diaiihea, vomiting
Splenomegaly
varaots
MvdI]Ied Meus|es Mildei clinical findings
with pieexisting paitial immunity.
FICk 2T-22 Meas|es E|,||er+|ou ||+| p+pu|e, |||| +ppe+||u ou ||e |+ce +ud uec| W|e|e ||e, |ecore
cou||ueu|, p|e+d|u |o ||e ||uu| +ud +|r |u 2 |o ! d+, W|e|e ||e, |er+|u d|c|e|e. |u cou||+|, |u|e||+ +|o ||||
+ppe+| |u|||+||, ou ||e |+ce |u| p|e+d |o ||e ||uu| |u d+,. Kop||| po| ou ||e |ucc+| ruco+ We|e +|o p|e
eu|. E|,||er+|ou p+pu|e |+.e |ecore cou||ueu| ou ||e |+ce ou ||e |ou||| d+,.
AtypIcu| Meus|es
Occuis in individuals immunized with foima-
lin-inactivated measles vaccine, subsequently
exposed to measles viius.
Exanthem begins peiipheially and moves
centially; can be uiticaiial, maculopapulai,
hemoiihagic, and/oi vesiculai.
Systemic symptoms can be seveie.
Meus|es In 1mmunvcvmprvmIsed Hvst
Rash may not occui.
Pneumonitis, encephalitis moie common.
0ssemoated Macu|opapu|ar ruptoo Diug
eiuption, othei viial exanthems (e.g., iubella),
scailet fevei. Kawasaki syndiome, infectious
mononucleosis, toxoplasmosis, M. neumonae
infection.
Cyto|oy Multinucleated giant cells in secie-
tions.
Cu|tures Isolate viius fiom blood, uiine, pha-
iyngeal secietions.
Meas|es Aoteo Detect in iespiiatoiy secie-
tions by immunofluoiescent staining.
Sero|oy Demonstiates fouifold oi gieatei
iise in measles titei.
FCk Detects genomic sequences of measles
viius RNA in seium, thioat swabs, and ceiebio-
spinal f luid (CSF).
0IACN0SIS
Clinical diagnosis, at times, confiimed by
seiology.
C0kS AN0 Fk0CN0SIS
Self-limited infection in most patients.
Moitality iate
United States: 0.3%
Developing countiies: 1-10%.
Age-specific iates of complications highest
among childien <5 yeais old and adults
>20 yeais.
Sites of complications: iespiiatoiy tiact, CNS,
tiact.
Complications moie common in malnoui-
ished childien, the unimmunized, and those
with congenital immunodeficiency and
leukemia.
Acute complications (9.8% of cases): otitis
media, pneumonia (bacteiial oi measles),
diaiihea, measles encephalitis (1 in 800 to
1000 cases), thiombocytopenia.
In unimmunized HIV-infected childien, fatal
measles pneumonia has occuiied without
iash.
Chionic complication: subacute scleiosing
panencephalitis (Dawson encephalitis)
MANACMNI
Freveotoo
Piophylactic immunization.
The goal of eliminating indigenous measles
tiansmission in the United States is based on
foui components:
Maintaining high coveiage with a single
dose of measles-mumps-iubella (MMR)
vaccine among pieschool-age childien,
Achieving coveiage with two doses of MMR
foi all school and college attendees
Enhancing suiveillance and outbieak
iesponse
Incieasing effoits to develop and implement
stiategies foi global measles elimination.
Acute IoIectoo Symptomatic.
Secoodary 8actera| IoIectoos Administiation
of appiopiiate antibiotics.
Ae oI 0oset 10 yeais but also young and
middle-age adults.
to|oy
Enteioviius (picoinaviius gioup, single-
stiand RNA, unenveloped).
Commonly: coxsackieviius A16, enteioviius
71 (EV71).
Also A5, 7, 9, 10; B1,2, 5.
Seasoo Epidemic outbieaks eveiy 3 yeais. In
tempeiate climates, outbieaks duiing waimei
months (late summei, eaily fall).
Iraosmssoo Highly contagious, spiead fiom
peison to peison by oial-oial and fecal-oial
ioutes.
FAIh0CNSIS
Enteioviial implantation in the GI tiact (buc-
cal mucosa and ileum) leads to extension into
iegional lymph nodes.
72 h latei a viiemia occuis with seeding of
the oial mucosa and skin of the hands and
feet.
E||o|o|c +eu|. |u|e||u+| .||ue ec|o.||u 9 +ud
o, co\+c||e A o .||u, eu|e|o.||u 1 (E\1)
Eu|e|o.||u |u|ec||ou W||| |+|
Ec|o.||u
Ec|o.||u 9 (E9). ku|e||||o|r (d|c|e|e p|u|
r+cu|e) |+| |e.e|
Ec|o.||u o. Bo|ou e\+u||er, |oeo|+|||e
(cou||ueu| p|u| p+pu|e) |e.e|.
Co\+c||e.||u Ao, eu|e|o.||u 1
n+ud|oo|+udrou|| d|e+e.
A-0, o, 22, CB-5, E\o, 9, , o, 1, 25,
E\1. ne|p+u|u+
0||e| eu|e|o.||u |epo||ed |o c+ue. e|,||er+
ru||||o|re, .e|cu|+|, u|||c+||+|, pe|ec||+|, +ud
pu|pu||c |+|e
NIk0vIkAI INFCII0NS
E||o|o|c +eu|. |u|e||u+| .||ue, co\+c||e A o
.||u, eu|e|o.||u 1
A ,|er|c |u|ec||ou
C|+|+c|e||/ed |,
u|ce|+||.e o|+| |e|ou
\e|cu|+| e\+u||er ou ||e d||+| e\||er|||e
\||d cou|||u||ou+| ,rp|or.
hAN0-F00I-AN0-M0Ih 0ISAS (hFM0)
Frodrome 12-24 h of low-giade fevei, malaise,
and abdominal pain oi iespiiatoiy symptoms.
Symptoms Fiequently 5-10 an[u| ulceiative
oial lesions, leading to iefusal to eat in childien.
Few to 100 cutaneous lesions appeai togethei oi
shoitly aftei the oial lesions and may be asymp-
tomatic oi enJer and an[u| .
Lesions begin as 2- to 8-mm matu|es oi
au|es that quickly evolve to est|es . Eaily
papules, pink to ied.
Vesicles have cleai fluid with a wateiy appeai-
ance oi yellowish hue.
Chaiacteiistically, lesions aiise on palms and
soles, especially on sides of fingeis, toes, and
buttocks.
Palms/fingeis, sole/toes: vesicles
Chaiacteiistic lineai" shape
Tendei, painful
Usually do not iuptuie (Fig. 27-23).
At othei cutaneous sites, vesicles can iuptuie,
with foimation of erosons and truss .
Lesions heal without scaiiing.
Oial lesions:
Macules giayish vesicles, aiising on
the haid palate, tongue, buccal mucosa
(Fig. 27-23B).
Vesicles quickly eiode to 5- to 10-mm,
small, punched out painful ulceis.
Ceoera| Fodos
May be associated with high fevei, seveie ma-
laise, diaiihea, and joint pains.
EV17 infections may have associated CNS
(aseptic meningitis, encephalitis, meningoen-
cephalitis, flaccid paialysis), and lung involve-
ment.
Epidemics with significant moitality in
young childien have occuiied in China and
Taiwan.
A sudden outbieak of oial and distal extiemity
lesions is pathognomonic foi HFMD. Howevei,
if only the oial lesions aie piesent, the diffei-
ential diagnosis would include HSV infection,
aphthous stomatitis, heipangina, eiythema
multifoime, adveise diug ieaction.
hstopatho|oy Epideimal ieticulai degen-
eiation leads to an intiaepideimal vesicle filled
with neutiophils, mononucleai cells, and pio-
teinaceous eosinophilic mateiial.
Sero|oy In acute seium, neutializing anti-
bodies may be detected but disappeai iapidly.
In convalescent seium, elevated titeis of com-
plement-fixing antibodies aie found.
Itaock Freparatoo Negative foi both multi-
nucleated giant cells and inclusion bodies,
which aie seen in HSV- and VZV-infected cells.
vra| Cu|ture Viius may be isolated fiom vesi-
cles, thioat washings, and stool specimens.
0IACN0SIS
C0kS AN0 Fk0CN0SIS
Most commonly, HFMD is self-limited.
Rise in seium antibodies eliminates the
viiemia in 7-10 days.
A few cases have been moie piolonged oi
iecuiient.
Seiious sequelae iaiely occui:
Coxsackieviius has been implicated in cases
of myocaiditis, meningoencephalitis, asep-
tic meningitis, paialytic disease, and a sys-
temic illness iesembling iubeola.
Enteioviius 71 infections have highei moi-
bidity/moitality iates due to CNS involve-
ment and pulmonaiy edema.
Infection acquiied duiing the fiist tiimestei
of piegnancy may iesult in spontaneous
aboition.
MANACMNI
Symptomatic tieatment, including topical
application of dyclonine HC solution oi
lidocaine gel, may ieduce oial discomfoit.
E||o|o|c +eu|. co\+c||e.||u A-0, o, 22,
co\+c||e B-5, ec|o.||ue Eo, 9, , o, 1, 25,
eu|e|o.||u E\1
|| uu+||, +||ec| c|||d|eu 5 ,e+| +ud | p|e.+
|eu| |u |+|e urre| +ud e+||, |+|| |u |erpe|+|e
c||r+|e.
C||u|c+| r+u||e|+||ou.
'uddeu oue| o| |e.e|, r+|+|e, |e+d+c|e, +uo
|e\|+, d,p|+|+, o|e |||o+|.
Eu+u||er. |o 2rr |+,W|||e p+pu|e/
.e|c|e ||+| e.o|.e |o u|ce| W||| |ed |+|o, +ud
d|||ue p|+|,ue+| |,pe|er|+ (ee ||. 215).
||||||u|ed ou ||e +u|e||o| |ou|||+| p|||+|, o||
p+|+|e, u.u|+, +ud |ou||.
uu+||, |+| +-o d+,, +ud || cou|e | e||
||r||ed.
hkFANCINA
FICk 2T-23 haod-Ioot-aod-mouth dsease . \u|||p|e, d|c|e|e, r+||, .e|cu|+| |e|ou ou ||e ||ue|
+ud p+|r, |r||+| |e|ou We|e +|o p|eeu| ou ||e |ee|. 'ore .e|c|e +|e |,p|c+||, ||ue+|. 8. \u|||p|e, upe|||c|+|
e|o|ou +ud r+||, .e|cu|+| |e|ou u||ouuded |, +u e|,||er+|ou |+|o ou ||e |oWe| |+||+| ruco+, ||e |u|.+
| uo|r+|. |u p||r+|, |e|pe||c |u|.o|or+||||, W||c| p|eeu| W||| |r||+| o|+| .e|cu|+| |e|ou, + p+|u|u| |u|.|
|| uu+||, occu| + We||.
8
Ae oI 0oset
All ages, but moie common in young.
Up to 60% of adolescents and adults aie seio-
positive foi anti-paivoviius B19 IgG.
Symptomatic iheumatic involvement is moie
common in adults.
to|oy Paivoviius, a small single-stiand, un-
enveloped DNA viius. Human infection caused
by paivoviius B19.
Sex Symptomatic illness with aithialgias moie
common in adult women.
Seasoo Occuis yeai iound; outbieaks in
schools in late wintei, eaily spiing.
Iraosmssoo
Viius is piesent in iespiiatoiy tiact duiing the
viiemic stage of paivoviius B19 infection.
Spieads via dioplet aeiosol.
Secondaiy attack iate among close contacts,
50%.
FAIh0CNSIS
Viiemia develops 6 days aftei intianasal
inoculation of B19 into volunteeis who lack
seium antibodies to the viius.
IgM and then IgG antibodies develop aftei a
week and cleai viiemia.
Significant bone maiiow depiession can
occui at this time.
The exanthem begins 17-18 days aftei inocu-
lation and may be accompanied by aithialgia
and/oi aithiitis; these findings aie mediated
by immune complexes.
In compiomised hosts, B19 can destioy eiyth-
ioid piecuisoi cells, causing seveie aplastic cii-
sis in adults and hydiops fetalis in the fetus.
E||o|o|c +eu|. |ur+u p+|.o.||u B9
C|||d|ood e\+u||er +oc|+|ed W||| p||r+|, |u
r+u p+|.o.||u B9 |u|ec||ou.
C|+|+c|e||/ed |,
Eder+|ou e|,||er+|ou p|+que ou ||e
c|ee| ('|+pped c|ee|)
E|,||er+|ou |+c, e|up||ou ou ||e ||uu| +ud
e\||er|||e.
',,. ||||| d|e+e.
kIhMA INFCII0SM (I) |C|9. 051.0

|C|0. B03.!
Iocubatoo 4-14 days.
Cootacts Exposuie to classmates oi siblings
with EI.
Symptoms 20-60% of individuals aie sympto-
matic; iemaindei asymptomatic.
ChI|dren
Piodiome of fevei, malaise, headache, coiyza
2 days befoie iash.
Headache, soie thioat, fevei, myalgias, nausea,
diaiihea, conjunctivitis, cough may coincide
with iash.
Uncommonly, aithialgias.
Piuiitus is vaiiably piesent.
Adu|ts
Constitutional symptoms moie seveie, with
fevei, adenopathy.
Aithiitis/aithialgias involving small joints of
hand, knees, wiists, ankles, feet.
Numbness and tingling of fingeis.
Piuiitus iash; iash usually absent in adults.
Edematous, confluent plaques on malai face
(slapped cheeks") (nasal biidge, peiioibital
iegions spaied) (Fig. 27-24); lesions fade
ovei 1-4 days.
Nonfacial lesions, best seen on extiemities,
appeaiing aftei facial lesions: eiythematous
macules and papules that become confluent,
giving a lacy oi ieticulated appeaiance (Fig.
27-24B); lesions fade in 5-9 days.
Less commonly, moibillifoim, confluent,
ciicinate, annulai.
Raiely, puipuia, vesicles, pustules, palmo-
plantai desquamation.
Reticulated iash may iecui.
SCII0N 2T \|kA| |||ECI|0|' 0| 'K|| A|| \uC0'A 80T
FICk 2T-24 rythema oIectosum . ||||ue e|,||er+ +ud eder+ o| ||e c|ee| W||| '|+pped c|ee|
|+c|e |u + c|||d. 8. ||c|e|e, e|,||er+|ou r+cu|e W||| ||u |o|r+||ou ou ||e uppe| +|r.
8
DIstrIhutIvn
Face: slapped cheeks". Usually absent in
adults.
Extensoi suiface of extiemities, tiunk, neck:
confluent macules/papules. Adults: ieticu-
lated macules on extiemities.
Other FIndIngs Paivoviius B19 also iepoited
to cause papulai-puipuiic gloves and socks"
syndiome.
Mucvsu| LesIvns Uncommonly, enanthem
with glossal and phaiyngeal eiythema; ied mac-
ules on buccal and palatal mucosa.
Ceoera| Fodos
Ju|s : Moie constitutional symptoms (fevei,
adenopathy, aithiitis), especially women;
often no iash.
Jons : Aithialgia and/oi aithiitis in 10% of
childien; typically involving laige joints.
Ch|dreo wth rythema IoIectosum Child-
h ood exanthems-iubella, measles, scailet fe-
vei, eiythema subitum, enteioviial infection,
Haemo||us n[|uen:ae cellulitis, adveise cuta-
neous diug ieaction.
Adu|ts wth Arthrts Lyme aithiitis, iheuma-
toid aithiitis, iubella.
Sero|oy Demonstiation of IgM anti-paivoviius
B19 antibodies oi IgG seioconveision. Demon-
stiation of paivoviius B19 in seium.
|ectroo Mcroscopy Infected eiythioid pie-
cuisoi cells show paivoviius-like paiticles.
hemato|oy Duiing aplastic ciisis: absence of
ieticulocytes, falling hemoglobin, hypoplasia oi
aplasia of eiythioid seiies in bone maiiow.
0IACN0SIS
C0kS AN0 Fk0CN0SIS
rythema IoIectosum Slapped cheeks"
aie noted fiist, fading ovei 1-4 days. Then,
ieticulated iash appeais on the tiunk, neck,
and extensoi extiemities. Eiuption lasts 5-9
days but chaiacteiistically can iecui foi weeks
oi months, tiiggeied by sunlight exposuie, ex-
eicise, tempeiatuie change, bathing, emotional
stiess.
Arthra|as Self-limited, lasting 3 weeks; but
may peisist foi seveial months oi yeais.
Ap|astc Crss In patients with chionic
hemolytic anemias (sickle cell anemia, he-
ieditaiy spheiocytosis, thalassemias, pyiu-
vate kinase deficiency, autoimmune hemolytic
anemia), tiansient aplastic ciisis may occui,
manifested by woisening anemia, fatigue, pal-
loi.
Feta| 819 IoIectoo Intiauteiine infection may
be complicated by nonimmune fetal hydiops
secondaiy to infection of RBC piecuisois,
hemolysis, seveie anemia, tissue anoxia, high-
output heait failuie. Risk 10% aftei mateinal
infection.
Immuoocompromsed host Piolonged chionic
anemia associated with peisistent lysis of RBC
piecuisois. At iisk: HIV/AIDS disease, con-
genital immunodeficiencies, acute leukemia,
oigan tiansplants, systemic lupus eiythemato-
sus, infants 1 yeai. Responds to intiavenous
immunoglobulin (IVIg).
MANACMNI
Symptomatic.
E||o|o|c +eu|.
\||ue. EB\, C\\, |ep+|||| B .||u (+,W ||+|u),
co\+c||e.||u, p+|+|u||ueu/+ .||u, |ep||+|o|,
,uc,||+| .||u, |o|+.||u, +deuo.||u, ec|o.||u,
po\ .||u, po||o.||u, p+|.o.||u, n|\, |ep+|||| A
.||u, |ep+|||| C .||u
B+c|e||+. !,:|cc -c- 3-|c
|!|- 3c|-||c |--|c- , |oup A ||ep
|ococcu.
\+cc|ue. |u||ueu/+, d|p|||e||+, |e|+uu, pe||u
|, BCC, | ||-cc- |,pe |, o|+| po||o
Ae. c|||d|eu o rou|| |o 2 ,e+| o|d ('ee ||.
2125).
|+||oeue|. |rruue |epoue |o ||+u|eu|
.||er|+ (|rruue corp|e\ depo|||ou)
||od|ore. r||d, uoupec|||c uppe| |ep||+|o|,
|u|ec||ou
C||u|c+| r+u||e|+||ou. e\+u||er
||c|e|e, uoup|u||||c, e|,||er+|ou, rouoro|
p||c p+pu|e (||. 2125)
|e|ou |ecore co+|eceu|.
|+ce, |u||oc|, +ud e\|euo| u||+ce o| e\
||er|||e, ,rre|||c.
I,p|c+||,, ||e ||uu| | p+|ed.
|u|+||ou | 2-3 Wee|.
',, . |+pu|+| +c|ode|r+|||| o| c|||d|ood
(|AC)
CIAN0III-Ck0SII SN0k0M (CCS)
FICk 2T-25 Caoott-Crost syodrome ||+| p+pu|e ou ||e c|ee| o| + 5,e+|o|d |||. '|r||+| p+pu|e
We|e +|o p|eeu| ,rre|||c+||, ou |o|e+|r, e||oW, +ud ||e .eu||+| +pec| o| ||e |e.
to|oy Flaviviius, single-stiand RNA viius.
Foui distinct dengue viiuses (DEN-1, -2, -3, -4).
Aithiopod-boine viius (aiboviius). Infection
confeis lifelong piotection against that seio-
type, but cioss-piotection between seiotypes is
of shoit duiation. Infection with viius of a dif-
feient seiotype aftei the piimaiy attack is moie
apt to iesult in seveie disease, DHF, oi DSS.
vector Tiansmitted by the bite of the .
aegy mosquito; less commonly . a||ot-
us . Mosquito acquiies viius by feeding upon
viiemic human; iemains infective foi life. These
eJes mosquitoes also tiansmit chikungunya
fevei. Bieed neai human habitation in watei
jais, vases, discaided containeis, coconut husks,
old tiies. Inhabits dwellings and bites duiing
the day. Othei mosquito- and tick-boine fla-
viviius infections include: chikungunya fevei,
yellow fevei, West Nile encephalitis, St Louis
encephalitis, Japanese encephalitis, tick-boine
encephalitis, Kyasanui Foiest disease, and Omsk
haemoiihagic fevei
Seasoo Yeai-iound tiansmission between lati-
tudes 25N and 25S. Seasonally, . aegy as
fai noith as Philadelphia. Global waiming may
be contiibuting to inciease in numbei of cases
of DF.
Ceoraphc 0strbutoo Occuis woildwide
thioughout tiopics. In the United States, in
Texas, Pueito Rico. Most cases occuiiing in
United States aie impoited in tiaveleis ietuin-
ing fiom the tiopics. Associated with human
populations, uiban. Noe : chikungunya fevei
does not occui in the Ameiicas and Caiibbean.
Iocdeoce 3 billion people living in aieas
potentially at iisk; 100 million cases woild-
wide annually. 400,000 cases of DHF. Second
most common mosquito-boine infection aftei
I||ee c||u|c+| ,ud|ore
C|+|c |e.e|-+||||+||+-|+| ,ud|ore, W|||
+||up| oue| o| |e.e| +ud ruc|e +ud jo|u|
p+|u, uu+||, W||| |e||oo||||+| p+|u, p|o|op|o
||+, +ud |,rp|+deuop+||,.
|n| c|+|+c|e||/ed |, ||| |e.e|, |ero|||+|c
p|euoreu+, o||eu |ep+|ore+|,
|'' W||| c||cu|+|o|, |+||u|e, c+u |e |+|+|
||+.|.||u |u|ec||ou (|E| -+)
I|+ur|||ed |, ||e |||e o| ||e 1-!- roqu||o
Euder|c |o ||op|c |u 00 couu|||e
|uc|deuce. e||r+|ed 00 r||||ou c+e +uuu+||,
k+|. e+||, ||u||u, |+|e| r+cu|e/p+pu|e, pu|
pu|+
',,. B|e+||oue |e.e|
0h60 FV8 (0F), 0h60 hN088hA6|0 FV8 (0hF),
0h60 Sh00k SYh080N (0SS)
malaiia. Incieased incidence associated with
iapid uiban population giowth, oveiciowding,
lax mosquito contiol.
Factors Cootrbuto to Iocrease Iocdeoce
Global waiming, iapid uibanization, popu-
lation giowth, inciease in nonbiodegiadable
pioducts that can seive as sites foi mosquito
laivae piolifeiation, aii tiavel.
Ae DHF/DSS moie common in childien.
FAIh0CNSIS
Viiemia is piesent at onset of fevei, peisist-
ing foi 3-5 days. The seveie syndiome (DHF
oi DSS) occuis in individuals (usually chil-
dien) with passively acquiied (tiansplacental
mateinal antibody) oi pieexisting nonneutial-
izing heteiologous dengue antibody due to
pievious infection with a diffeient seiotype of
viius. Initial symptoms simulate usual dengue,
findings abiuptly woisen. DSS chaiacteiized
by shock and hemoconcentiation. Subsequent
infection with dengue type 2 following a type 1
infection is paiticulai iisk factoi foi seveie dis-
ease. Pathogenesis of seveie syndiome involves
pieexisting dengue antibody. Viius-antibody
complexes foimed within a few days of second
dengue infection; nonneutializing enhancing
antibodies piomote infection of highei num-
beis of mononucleai cells, followed by ielease
of cytokines, vasoactive mediatois, and pioco-
agulants, leading to the disseminated intiavas-
culai coagulation seen in DHF.
0hF Incieased vasculai peimeability and
plasma leakage fiom blood vessels into tissues,
thiombocytopenia, bleeding manifestations
(fiank hemoiihage to spontaneous petechiae
oi elicited by touiniquet test). Plasma leak-
age causes a iise in hematociit, effusions, and
edema, especially in chest, abdomen.
0SS Occuis when leakage oi bleeding, oi
both, aie sufficient to induce hypovolemic
shock.
Iocubatoo Ferod 4-7 days aftei bite of
infected mosquito.
0uratoo oI Symptoms Vaiiable; 2-5 days.
Biphasic in some cases. Rash is veiy common
in iemission peiiod oi eaily in a second febiile
phase
Symptoms ProJrome : Malaise, chills, head-
ache. Rash usually asymptomatic but may be
piuiitic. Symptoms highly vaiiable: subclinical
to incapacitating. Sudden onset of fevei, chills,
malaise, nausea/vomiting, headache, photo-
phobia, ietiooibital pain, phaiyngitis, back
pain, seveie myalgia (oiigin of teim |rea|-|one
[eer).
Sko Iesoos
Rash estimated to occui in 50-80% of cases.
Initial iash 1-2 days aftei onset of symptoms:
eiythema/flushing (iesembles sunbuin, i.e.,
capillaiy dilatation) of face, neck, chest
Latei iash 4-7 days aftei onset of symp-
toms: moibillifoim eiuption beginning on
the tiunk, spieading to extiemities and face
in uncomplicated dengue, lasting foi 1-5
days (Fig. 27-26); petechiae; islands of spaiing
(white islands in sea of ied"). Coiielates with
immune iesponse to viius.
Uiticaiia with piuiitus
Hemoiihagic diathesis: petechiae, ecchym-
oses, bleeding at sites of injection oi venepunc-
tuie.
DHF
Islands of white in a sea of ied in aieas of
hemoiihage and edema, especially lowei
legs. (Fig. 27-26)
Sciotal edema
Mucosa| Iesoos Scleial/conjunctival injec-
tion. Epistaxis. Bleeding gums, nose, GI tiact.
Systemc Fodos Suipiisingly minimal.
Adenopathy eaily in couise. Pain on palpation
FICk 2T-26 0eoue hemorrhac Iever: |e A !9,e+|o|d de.e|oped |e.e| +ud |+| +||e| + |||p |o
\+|+,|+. |e|r+| |ero|||+e, pe|ec||+e, +ud eder+ +|e eeu ou ||e |oWe| |e, 'W|||e ||+ud |u + e+ o| |ed
+|e eeu. |euue |C +u|||od, W+ de|ec|ed W||| |o|de|||ue deuue |\ +u|||od,. A |ero|||+|c cou|e c+u
occu| |u pe|ou W|o |+.e |+d deuue |e.e| p|e.|ou|, +ud |e|+|u uouueu||+||/|u |e|e|o|,p|c +u|||od|e
(Cou||e, o| C n+|ue| e| +|. n+u|+|/| 51.105101, 200o.)
8
of muscles oi epigastiium. Abdominal pain
may mimic appendicitis. Encephalopathy in
childien. Distal sensoiy polyneuiopathy. DHF.
Effusions and edema in chest and abdomen.
Subaiachnoid hemoiihage.
ar|y F|usho rythema Chikungunya fe-
vei, sandfly fevei, scailet fevei, toxic shock
syndiome, Kawasaki disease, eiythema infectio-
sum (paivoviius B19).
Morb||Iorm ruptoo Acute HIV syndiome,
infectious mononucleosis (EBV, CMV), ioseola
infantum (HHV-6), measles, iubella, enteiovi-
ius, secondaiy syphilis, typhoid fevei, chikun-
gunya fevei, West Nile viius, O`nyong-nyong
fevei, Myaio viius, Sindbis viius, leptospiiosis,
adveise cutaneous diug eiuption.
hemato|oy Leukopenia; thiombocytopenia.
High hematociit with DHF/DSS. Low hemat-
ociit aftei hydiation.
Chemstry Aminotiansfeiase elevations.
0ermatopatho|oy Nonspecific.
keverse Iraoscrptase-FCk-8ased Methods
Rapid identification and seiotyping of dengue
viius in acute-phase seium, ioughly duiing the
peiiod of fevei.
Iso|atoo oI vrus Inoculation of mosquito cell
line with patient seium, coupled with nucleic
acid assays to identify a iecoveied viius.
Sero|oy Viial piotein-specific captuie IgM
oi IgG enzyme-linked immunosoibent assay
(ELISA) and hemagglutination inhibition test.
IgM antibodies develop within a few days of
illness. Neutializing and hemagglutination-
inhibiting antibodies appeai within a week aftei
onset of DF. Acute and convalescent seia show
significant iise in antibody titei; most ieliable
evidence of active dengue infection.
C0C 0eoue 8raoch Both acute- and con-
valescent-phase seium samples sent thiough
state/teiiitoiial health depaitment laboiatoiies
to CDC`s Dengue Bianch, Division of Vectoi-
Boine Infectious Diseases, National Centei foi
Infectious Diseases, 1324 Calle Canada, San
Juan, Pueito Rico 00920-3860
0IACN0SIS
Considei diagnosis in tiaveleis with febiile
illness iecently ietuined fiom endemic aieas.
Rapid seiologic testing.
C0kS AN0 Fk0CN0SIS
Ratio of inappaient to appaient infections
about 15:1 foi piimaiy infections; iatio lowei
in secondaiy infections. Tempeiatuie ietuins to
noimal aftei 5-6 days oi may subside on about
thiid day and iise again about 5-8 days aftei
onset (saddleback" foim). Complete iecoveiy
is geneially the outcome, although piolonged
asthenia and nonspecific symptoms have been
desciibed in some cases. Death seen in cases of
DF and DSS, which aie leading cause of child-
hood death in seveial Asian countiies. Fatality
iate with DHF up to 15%; can be ieduced to less
than 1% with piopei tieatment.
MANACMNI
Freveotoo Individuals tiaveling to
endemic aieas should be educated about pie-
ventive measuies. Avoid mosquito habitat at
times of peak activity; use scieens/baiiieis and
aii-conditioned iooms. Apply insect iepel-
lant diethyltoluamide (DEET) to skin. Weai
peimethiin-impiegnated clothing. Mosquito
contiol measuies in endemic aieas.
Ireatmeot No specific antiviial theiapy. Sup-
poitive measuies, including analgesics (avoid-
ing agents with platelet dysfunction) and
hydiation.
vaccoe Vaccine has been developed but is not
yet available commeicially.
C0C Webste http://phstwlp1.paitneis.oig:2219/
ncidod/dvbid/dengue/index.htm.
nur+u |e|pe.||ue (nn\) (|+r||, ne|pe.|||
d+e) +|e de||ued |, ||e +|c|||ec|u|e o| ||e .|||ou,
W||c| |+.
Co|e cou|+|u|u + ||ue+| dou||e||+ud ||A
|co+|ed|+| c+p|d 00-0 ur |u d|+re|e|
corpoed o| o2 c+pore|
Eu.e|ope cou|+|u|u .||+| |,cop|o|e|u p||e ou
||e u||+ce.
wo||dW|de, o0-90 o| ||e popu|+||ou | |u|ec|ed
W||| oue o| ro|e nn\.
E||| nn\ |+.e |eeu |deu||||ed.
ne|pe |rp|e\ .||u (n'\) (nn\)
n'\2 (nn\2)
\+||ce||+/o|e| .||u ( \/ \, o| nn\!)
Ep|e|uB+|| .||u (EB\, o| nn\+)
C,|ore+|o.||u (C\\, o| nn\5)
nn\o
nn\1
nn\3 (K+po| +|cor+-+oc|+|ed .||u).
|||r+|, nn\ |u|ec||ou +|e uu+||, +,rp|or+||c
W||| ||e e\cep||ou o| \/\, W||c| ue+||, +|W+,
p|eeu| W||| ,rp|or+||c .+||ce||+.
A||e| p||r+|, |u|ec||ou, nn\ |er+|u |+|eu| |u
ueu|+| o| |,rp|o|d ce|| +ud |e+c||.+|e || +u
+dequ+|e |rruue |epoue doe uo| e\||.
nn\ +|e c+|eo||/ed |u|o |||ee |oup. A|p|+,
|e|+, +ud +rr+ ne|pe.|||d+e (I+||e 21!).
1||c |--.!c- . n'\, n'\2, \/\ +|e
c|+|+c|e||/ed |, + .+||+||e |o| |+ue, |e|+||.e|,
|o|| |ep|oduc||.e c,c|e, |+p|d p|e+d |u cu||u|e,
|+p|d de||uc||ou o| |u|ec|ed ce||, +ud |+|eu|
|u|ec||ou p||r+|||,, |u| uo| e\c|u|.e|,, o| eu
o|, +u||+.
3-|c |--.!c- . C\\ |+ + |e|||c|ed |o|
|+ue +ud p|e+d |oW|, |u cu||u|e.
Ccc |--.!c- . EB\, nn\o, nn\1,
nn\3, +ud |e|pe.||u +|r||| +|e |,rp|o
||op|c, pec|||c |o| e|||e| I o| B |,rp|oc,|e.
I|e nn\3 ||A equeuce +|e c|oe|, |oro|o
ou |o r|uo| c+p|d +ud |eureu| p|o|e|u
eue o| +rr+|e|pe.|||d+e EB\ +ud |e|pe
.||u +|r|||.
hMAN hkFSvIkSS |C|9. 05+

|C|0. B00
w|e||e| ||||,rp|or+||c o| |ecu||eu|, r+,
'|,p|c+||, p|eeu| c||u|c+||, W||| |ouped .e|c|e
+|||u ou +u e|,||er+|ou |+e ou |e|+||u|/ed
||u o| rucou rer||+ue.
\o| n'\ |u|ec||ou +|e '+|,p|c+|, W||| p+|c|(e)
o| e|,||er+, r+|| e|o|ou, ||u|e, o| u|c||u|
c+| |e|ou ||+| |ed n'\.
0uce +u |ud|.|du+| | |u|ec|ed, n'\ pe||| |u
euo|, +u||+ |o| ||e |||e o| ||e p+||eu|, |ecu|||u
W||| |eeu|u |u |rruu||,.
C||u|c+| r+u||e|+||ou.
|u |e+|||, |ud|.|du+|, |ecu||eu| |u|ec||ou +|e
+,rp|or+||c o| r|uo|, |eo|.|u pou|+ue
ou|, o| W||| +u||.||+| ||e|+p,.
|u ||e |rruuocorp|or|ed |o|, rucocu
|+ueou |e|ou c+u |e e\|eu|.e, c||ou|c, o|
d|er|u+|e |o ||u o| .|ce|+.
',,. ne|pe, |e|pe |rp|e\, co|d o|e,
|e.e| ||||e|, |e|pe |e|||||, |e|pe |+||+||, |e|pe
|+d|+|o|ur, c|ur po\, |e|pe||c W||||oW, eu||+|
|e|pe, |e|pe p|oeu||+||.
hkFS SIMFIX vIkS (hSv) INFCII0N
Ae oI 0oset Most commonly young adults;
iange, infancy to senescence.
to|oy HSV-1, HSV-2.
Labialis: HSV-1 (80-90%), HSV-2 (10-20%).
Uiogenital: HSV-2 (70-90%), HSV-1
(10-30%).
Heipetic whitlow: 20 yeais of age usually
HSV-1; 20 yeais of age, usually HSV-2.
Neonatal: HSV-2 (70%), HSV-1 (30%).
Iraosmssoo
Most tiansmission occuis when peisons shed
viius but lack lesions.
Usually skin-skin, skin-mucosa, mucosa-skin
contact.
IA8I 2T-3 human herpesviruses and Associated 0iseases in |mmunocompetent and
|mmunocompromised |ndividua|s
0|sease |o 0|sease |o
|mm0oocompeteot |mm0oocomprom|sed
h0mao herpesv|r0s |od|v|d0a|s |od|v|d0a|s Naoagemeot
ne|pe |rp|e\ .||u |||r+|, |u|ec||ou o||eu w|dep|e+d |oc+| |rruu|/+||ou.
(n'\) (nn\) +,rp|or+||c |u|ec||ou .+cc|ue p|or||u
|||r+|, |e|pe||c C||ou|c u|ce| Au||.||+| +eu|
|u|.o|or+|||| ||er|u+|ed Ac,c|o.||
ne|pe |+||+|| cu|+ueou |u|ec||ou \+|+c,c|o.||
ne|pe||c W||||oW ||er|u+|ed .|ce|+| |+rc|c|o.||
Aep||c reu|u||| |u|ec||ou |oc+|ue|
n'\ eucep|+||||
ne|pe |rp|e\ .||u2 |||r+|, |u|ec||ou o||eu w|dep|e+d |oc+| |rruu|/+||ou.
(n'\2) (nn\2) +,rp|or+||c |u|ec||ou .+cc|ue p|or||u
ne|pe eu||+||, C||ou|c u|ce| Au||.||+| +eu|
p||r+|, +ud |ecu||eu| ||er|u+|ed Ac,c|o.||
ne|pe||c W||||oW cu|+ueou |u|ec||ou \+|+c,c|o.||
Aep||c reu|u||| ||er|u+|ed |+rc|c|o.||
.|ce|+| |u|ec||ou |oc+|ue|
\+||ce||+/o|e| |||r+|, |u|ec||ou ue+||, ||er|u+|ed |rruu|/+||ou.
.||u (\/\) +|W+, ,rp|or+||c cu|+ueou |u|ec||ou .+cc|ue +.+||+||e
(nn\!) \+||ce||+ (p||r+|, ||er|u+|ed .|ce|+| Au||.||+| +eu|
|u|ec||ou) |u|ec||ou Ac,c|o.||
ne|pe /o|e| C||ou|c |e|pe /o|e| \+|+c,c|o.||
C||ou|c ec||,r+|ou |+rc|c|o.||
\/\ |u|ec||ou |oc+|ue|
Ep|e|uB+|| .||u |||r+|, |u|ec||ou |,rp|or+ Au||.||+| +eu|
(EB\) (nn\+) o||eu +,rp|or+||c Ac,c|o.||
EB\ C+uc|c|o.||
rououuc|eo|
(p||r+|, EB\ |u|ec||ou)
|+op|+|,ue+|
c+|c|uor+
Bu||||| |,rp|or+
|o|||+up|+u|+||ou
|,rp|or+
I ce|| |,rp|or+,
+ud o||e| |,rp|or+ !!! !!!
0|+| |+||, |eu|op|+||+ |rruu|/+||ou. uoue
Heipes gladiatoium tiansmitted by skin-
to-skin contact in wiestleis.
Incieased HSV-1 tiansmission associated
with ciowded living conditions and lowei
socioeconomic status.
Frecptato Factors Ior kecurreoce
Appioximately one-thiid of peisons who
develop heipes labialis will expeiience a
iecuiience; of these, one-half will expeiience
at least two iecuiiences annually.
Usual factois foi heipes labialis: skin/mucosal
iiiitation (UV iadiation), alteied hoimonal
milieu (menstiuation), fevei, common cold,
alteied immune states, site of infection (genital
heipes iecuis moie fiequently than labial).
Immuoocompromso Factors Fredsposo to
hSv keactvatoo HIV/AIDS infection, malig-
nancy (leukemia/lymphoma), tiansplantation
(bone maiiow, solid oigan), chemotheiapy,
systemic glucocoiticoids, othei immunosup-
piessive diugs, iadiotheiapy.
FAIh0CNSIS
Piimaiy HSV infection occuis thiough close
contact with a peison shedding viius at a
peiipheial site, mucosal suiface, oi secietion.
HSV is inactivated piomptly at ioom tem-
peiatuie; aeiosol oi fomitic spiead unlikely.
Infection occuis via inoculation onto suscep-
tible mucosal suiface oi bieak in skin.
Aftei exposuie to HSV, the viius ieplicates in
epithelial cells, causing lysis of infected cells,
vesicle foimation, and local inflammation.
Aftei piimaiy infection at inoculation site, HSV
ascends peiipheial sensoiy neives and enteis
sensoiy (Image 27-1) oi autonomic neive ioot
(vagal) ganglia, wheie latency is established.
Retiogiade tianspoit of HSV among neives
and establishment of latency aie not dependent
on viial ieplication in skin oi neuions; neuions
can be infected in the absence of symptoms.
Latency can occui aftei both symptomatic
and asymptomatic piimaiy infection.
IA8I 2T-3 human herpesviruses and Associated 0iseases in |mmunocompetent and
|mmunocompromised |ndividua|s (Con||naed)
0|sease |o 0|sease |o
|mm0oocompeteot |mm0oocomprom|sed
h0mao herpesv|r0s |od|v|d0a|s |od|v|d0a|s Naoagemeot
C,|ore+|o.||u |||r+|, |u|ec||ou ke||u||| |rruu|/+||ou.
(C\\) (nn\5) o||eu +,rp|or+||c |ueurou||| .+cc|ue p|or||u
C\\ rououuc|eo| Co|||| Au||.||+| +eu|
(p||r+|, C\\ |u|ec||ou) C+uc|c|o.||
|oc+|ue|
C|do|o.||
nur+u |e|pe.||uo |||r+|, |u|ec||ou o||eu
(nn\o) +,rp|or+||c
E\+u||er+ u|||ur !!! !!!
nur+u |e|pe.||u1 |||r+|, |u|ec||ou o||eu
(nn\1) +,rp|or+||c
E\+u||er+ u|||ur !!! !!!
nur+u |e|pe.||u3 |||r+|, |u|ec||ou K+po| +|cor+
(nn\3) r+, p|eeu| W|||
|e.e| +ud
|||| c|
Bod, c+.||, |,rp|or+
|u n|\|u|ec|ed
|ud|.|du+|
K+po| +|cor+
Peiiodically, HSV may ieactivate fiom its
latent state and viius paiticles then tiavel along
sensoiy neuions to skin and mucosal sites
to cause iecuiient disease episodes (Image
27-1).
Recuiient mucocutaneous shedding can be
associated with oi without (asymptomatic
shedding) lesions; viius can be tiansmitted to
a new host when shedding occuis.
Recuiiences usually occui in the vicinity
of the piimaiy infection; may be clinically
symptomatic oi asymptomatic.
See Nongenital heipes simplex viius infec-
tions", below.
0rect Mcroscopy Tzunc| Smeur (Fig.
27-27). Optimally, fluid fiom intact vesicle is
smeaied thinly on a micioscope slide, diied,
and stained with eithei Wiight oi Giemsa stain.
Positive, if acantholytic keiatinocytes oi multi-
nucleated giant acantholytic keiatinocytes aie
detected. Positive in 75% of eaily cases, eithei
piimaiy oi iecuiient.
AntIgen DetectIvn DFA Monoclonal antibod-
ies, specific foi HSV-1 and HSV-2 antigens,
MANACMNI
Freveotoo '||u|o||u cou|+c| |ou|d |e +.o|ded du||u ou|||e+| o| cu|+ueou n'\ |u|ec||ou.
Iopca| Aotvra| Iherapy App|o.ed |o| |e|pe |+||+||, r|u|r+| e|||c+c,.
Ac,c|o.|| 5 o|u|reu| App|, q!|, o ||re d+||, |o| 1 d+,. App|o.ed |o| |u|||+| eu||+| |e|pe +ud
||r||ed rucocu|+ueou n'\ |u|ec||ou |u |rruuocorp|or|ed |ud|.|du+|.
|euc|c|o.|| c|e+r App|, q2| W|||e +W+|e |o| |ecu||eu| o|o|+||+| |u|ec||ou |u |rruuocorpe|eu|
|ud|.|du+|.
0ra| Aotvra| Iherapy Cu||eu||,, +u||n'\ +eu| +|e +pp|o.ed |o| ue |u eu||+| |e|pe ||eur+||,,
|r||+| do|u |e|reu +|e e||ec||.e |o| uoueu||+| |u|ec||ou. ||u |o| o|+| n'\
||e|+p, |uc|ude +c,c|o.||, .+|+c,c|o.||, +ud |+rc|c|o.||. \+|+c,c|o.||, ||e p|od|u
o| +c,c|o.||, |+ + |e||e| ||o+.+||+|||||, +ud | ue+||, 35 +|o||ed +||e| o|+|
+dr|u|||+||ou. |+rc|c|o.|| | equ+||, e||ec||.e |o| cu|+ueou n'\ |u|ec||ou.
|| -!- Au||.||+| +eu| ro|e e||ec||.e |u ||e+||u p||r+|, |u|ec||ou ||+u |ecu||euce.
Ac,c|o.|| +00 r ! ||re d+||, o| 200 r 5 ||re d+||, |o| 1-0 d+,
\+|+c,c|o.|| |W|ce d+||, |o| 1-0 d+,
|+rc|c|o.|| 250 r ! ||re d+||, |o| 5-0 d+,
|-:-:- \o| ep|ode o| |ecu||eu| |e|pe do uo| |eue||| ||or pu|e ||e|+p, W||| o|+|
+c,c|o.||. |u e.e|e |ecu||eu| d|e+e, p+||eu| W|o |+|| ||e|+p, +| ||e |e|uu|u
o| ||e p|od|ore o| W||||u 2 d+, +||e| oue| o| |e|ou r+, |eue||| ||or ||e|+p,
|, |o||eu|u +ud |educ|u e.e|||, o| e|up||ou, |oWe.e|, |ecu||euce c+uuo| |e
p|e.eu|ed.
detect and diffeientiate HSV antigens on smeai
fiom lesion.
0ermatopatho|oy Ballooning and ieticu-
lai epideimal degeneiation, acantholysis, and
intiaepideimal vesicles; intianucleai inclu-
sion bodies, multinucleate giant keiatinocytes;
multiloculai vesicles. Immunopeioxidase tech-
niques can be used to identify HSV-1 and HSV-2
antigens in foimalin-fixed tissue samples.
Cu|tures Positive HSV cultuies fiom involved
mucocutaneous site oi tissue biopsy specimens.
Sero|oy
Antibodies to glycopiotein (g)G1 and (g)G2
detect and diffeientiate past HSV-1 and HSV-2
infections.
Piimaiy HSV infection can be documented
by demonstiation of seioconveision.
Recuiiing heipes can be iuled out if seio-
negative foi HSV antibodies.
Fo|ymerase Chao keactoo To deteimine
HSV-DNA sequences in tissue, smeais, oi
secietion.
0IACN0SIS
Clinical suspicion confiimed by viial cultuie
oi antigen detection. Cultuies used foi diag-
nosing fiist-episode infections since antibod-
ies to (g)H1 oi (g)G2 may take 2-6 weeks to
develop.
FICk 2T-2T herpes smp|ex vrus: postve Itaock smear A |+u|, ru|||uuc|e+|ed |e|+||uoc,|e ou +
C|er+|+|ued re+| o||+|ued ||or + .e|c|e |+e. Corp+|e ||e |/e o| ||e |+u| ce|| |o ||+| o| ||e ueu||op|||
+|o eeu |u ||| p|ep+|+||ou. Au |o|+|ed +c+u||o|,||c |e|+||uoc,|e | +|o eeu. |deu||c+| ||ud|u +|e p|eeu| |u
|e|ou c+ued |, .+||ce||+ /o|e| .||u.
IMAC 2T-1 herpes |aba|s . w||| p||r+|, n'\ |u|ec||ou, .||u |ep||c+|e |u ||e o|op|+|,ue+| ep|||e
||ur, +ceud pe||p|e|+| euo|, ue|.e |u|o ||e |||er|u+| +u||ou. 8. n'\ pe||| |u + |+|eu| p|+e W||||u ||e
|||er|u+| +u||ou |o| ||e |||e o| ||e |ud|.|du+|. C. \+||ou ||ru|| |u|||+|e |e+c||.+||ou o| |+|eu| .||u, W||c| ||eu
deceud euo|, ue|.e |o ||e ||p o| pe||o|+| ||u, |eu|||u |u |ecu||eu| |e|pe |+||+||.
|oueu||+| n'\ |u|ec||ou, W|e||e| p||r+|, o|
|ecu||eu|, | o||eu +,rp|or+||c.
|e|ou r+, p|eeu| + |oup .e|c|e ou +u e|,
||er+|ou |+e o| + + |ecu||eu| e|,||er+|ou
p|+que e|o|ou.
',, . ne|pe, |e|pe |rp|e\, co|d o|e,
|e.e| ||||e|, |e|pe |e|||||, |e|pe |+||+||, |e|pe
|+d|+|o|ur, c|ur po\, |e|pe||c W||||oW.
|o| eu||+| n'\ |u|ec||ou, ee 'ec||ou !0.
N0NCNIIAI hkFS SIMFIX vIkS INFCII0N
C|: - |ec|e+e ||equeuc, o| ,rp|or+||c |ecu||euce +ud +,rp|or+||c n'\
|edd|u. A||e| ,e+| o| cou||uuou d+||, upp|e|.e ||e|+p,, +c,c|o.|| |ou|d
|e d|cou||uued |o de|e|r|ue ||e |ecu||euce |+|e.
Ac,c|o.|| +00 r |W|ce d+||,
\+|+c,c|o.|| 500-000 r pe| d+,
|+rc|c|o.|| 250 r |W|ce d+||,
!::|c- !-c- |e|||e| ||e ueed |o| uo| ||e p|ope| |uc|e+ed do+e o| +c,c|o.|| |+ |eeu
:-! e|+||||ed couc|u|.e|,. |+||eu| W||| |e|pe W|o do uo| |epoud |o ||e
!.!c| |ecorreuded doe o| +c,c|o.|| r+, |equ||e + |||e| o|+| doe o| +c,c|o.||,
|\ +c,c|o.||, o| |e |u|ec|ed W||| +u +c,c|o.|||e||+u| n'\ ||+|u, |equ|||u |\
|oc+|ue|. I|e |o|e o| .+|+c,c|o.|| +ud |+rc|c|o.|| +|e uo| ,e| e|+||||ed.
Ac,c|o.|| 5 r/| |\ q3| |o| 1-+ d+,, +00 r 5 ||re d+||, |o| 1-+ d+,
0|+| .+|+c,c|o.|| |+rc|c|o.|| keduce ||e uece||, |o| |\ +c,c|o.|| ||e|+p,.
|-c|c|
Ac,c|o.|| 20 r/| |\ q3| |o| +-2 d+,
1:,:|. -|c:- E\||ere|, |+|e |u |rruuocorpe|eu| |o|. uu+||, occu| |u |rruuocorp|or|ed
|ud|.|du+| W||| |+|e |e|pe||c |e|ou W||| ||| n'\ .||+| |o+d. ke||+u| n'\
||+|u +|e ||,r|d|ue||u+e de||c|eu|. A||e|u+||.e d|u. |oc+|ue|, c|do|o.||. |u
n|\|u|ec|ed p+||eu|, c||ou|c n'\ |u|ec||ou +|e rucocu|+ueou, |+|e|, |u.+|.e.
|oc+|ue| +0 r/| |\ q3| |o| +-2 d+,
||l/1||' |-:| |e|ou c+ued |, n'\ +|e |e|+||.e|, corrou +rou n|\|u|ec|ed pe|ou.
|o| e.e|e d|e+e, |\ +c,c|o.|| ||e|+p, r+, |e |equ||ed. || |e|ou pe|||
+rou p+||eu| uude|o|u +c,c|o.|| ||e+|reu|, |e||+uce |o +c,c|o.|| |ou|d |e
upec|ed.
Ac,c|o.|| |u|e|r|||eu| o| upp|e|.e ||e|+p, W||| o|+| +c,c|o.|| r+, |e ueeded. +00 r |0
!-5 ||re d+||, r+, |e ue|u|. I|e|+p, |ou|d cou||uue uu||| c||u|c+| |eo|u||ou
| +||+|ued.
|oc+|ue| |o| e.e|e d|e+e c+ued |, p|o.eu o| upec|ed +c,c|o.|||e||+u| ||+|u,
|op||+||/+||ou |ou|d |e cou|de|ed. |oc+|ue|, +0 r/| |od, We||| q3| uu|||
c||u|c+| |eo|u||ou | +||+|ued. Appe+| |o |e ||e |e| +.+||+||e ||e+|reu|.
Iocubatoo Ferod 2- to 20-day (aveiage 6)
incubation peiiod foi piimaiy infection.
PrImury Herpes
Many individuals with piimaiy HSV infec-
tion aie eithei asymptomatic oi have only
tiivial symptoms.
Symptomatic piimaiy heipes is uncom-
mon.
Chaiacteiized by vesicles at the site of in-
oculation (Fig. 27-28). Associated with ie-
gional lymphadenopathy
At times accompanied by fevei, headache,
malaise, myalgia. It peaks within the fiist
3-4 days aftei onset of lesions, iesolving
duiing the subsequent 3-4 days.
Piimaiy heipetic gingivostomatitis is the
most common symptom complex accompa-
nying piimaiy HSV infection in childien.
In young women, piimaiy heipetic vul-
vovaginitis (see also Section 30).
Recurrent Herpes
Piodiome of tingling, itching, oi buining
sensation usually piecedes any visible skin
changes by 24 h.
Systemic symptoms aie usually absent.
Mucocutaooeus Fodos
Frmary herpes
Eiythema often noted initially, followed soon
by giouped, often umbilicated vesicles, which
may evolve to pustules (Fig. 27-28). Vesicles
aie often fiagile, tiansient, not obseived.
These become eioded as the oveilying epidei-
mis sloughs.
Eiosions may enlaige to ulceiations, which
may be ciusted oi moist.
These epithelial defects heal in 2-4 weeks,
often with iesultant postinflammatoiy hypo-
oi hypeipigmentation, uncommonly with
scaiiing.
The aiea of involvement may be ciicumfeien-
tial aiound the mouth.
Regional lymphadenopathy.
Location: oiophaiyngeal, labial, peiioial; dis-
tal fingeis; othei sites.
Mucvus Memhrunes
Oial mucosa usually involved only in pii-
maiy HSV infection with vesicles that quickly
slough to foim eiosions (Fig. 27-29) at any
site in the oiophaiynx, scanty to numeious;
gingivitis with gingival tendeiness, edema,
violaceous coloi. Sialoiihea. Seveie pain.
Conjunctival and coineal autoinoculation
may occui.
kecurreot herpes
Giouped vesicles on eiythematous base-
eiosions and ciusts (Fig. 27-30-D).
Recuiient intiaoial HSV is iaie. Often, only
supeificial eiosions.
SpecI]Ic Feutures v] HSV 1n]ectIvns v] DI]]erent
Sensvry Nerves: TrIgemInu| Nerve
Co|J sores : Recuiient facial heipes/cold soies
(Fig. 27-30). Usually pieceded by piodiomal
symptoms (tingling, pain, buining sensation,
itching). Affect 20-40% of adults. Seveie
iecuiiences may complicate lasei-iesuifacing
suigeiy.
Otu|ar HSV n[etons : Recuiient keiatitis is
a majoi cause of coineal scaiiing and visual
loss, Continuous suppiession theiapy is iec-
ommended.
Heret [ata| ara|yss : Reactivation of
geniculate ganglion infection implicated in
pathogenesis of idiopathic facial palsy (Bell
palsy). HSV-1 shedding detected in 40% of
cases. Inflammation plays a majoi iole in
pathogenesis; glucocoiticoids may be effec-
tive.
HSV g|aJaorum : Tiansmission occuis
duiing contact spoits (wiestling, iugby, foot-
ball). Also occuis in ceivical oi lumbosacial
deimatomes. Piophylaxis may pievent iecui-
ience.
FICk 2T-28 herpes smp|ex vrus oIectoo:
prmary oIectoo oI the pa|m A 23,e+||er+|e
W||| + p+|u|u| |e|ou ou ||e p+|r |o| ! d+,. A c|u|e|
o| |ouped pu|u|e +|e eeu ou ||e p+|r. A |ed |,r
p|+u||| e\|eud p|o\|r+||, ou ||e W|||. I|e +\|||+|,
|,rp| uode We|e |eude| +ud eu|+|ed. n'\2 W+
de|ec|ed ou ||A. |o +u|||od|e |o n'\ o| 2 We|e
de|ec|ed. I|e |u|ec||ou |, ||u, p||r+|,.
CervIcu| und ThvrucIc Sensvry Nerves
1n]ectIvns
Heret w||ow : Piioi to Univeisal Piecau-
tions," occuiied in health caie piofessionals,
especially dental peisonnel. Associated
with painful neuiitis in the affected fingei
and foieaim (Fig. 27-31). May last foi 3
weeks.
HSV n[eton o[ |e n|e : Related to tians-
mission of HSV fiom infant to mothei duiing
bieast feeding.
HSV n[etons o[ |e |um|osatra| sensory
neres : When lumbosaciaal ganglia become
infected subsequent to anogenital heipes,
iecuiient lesions can occui on genitalia as well
as buttocks, thighs, peiianal mucosa. Peiianal
heipes does not necessaiily imply diiect anal
inoculation of HSV. Symptomatic heipes in
the sacial deimatome may be accompanied
by asymptomatic HSV ieactivation/shed-
ding fiom genital mucosa. Recuiient itching,
buining, blisteiing, eiythema below the waist
should be iegaided as genital HSV infection
until pioven otheiwise.
Cvmp|IcutIvns v] HSV 1n]ectIvns v] PerIpheru|
Sensvry Nervvus System
Et:ema |eretum : Usually follows autoin-
oculation of HSV (most commonly oiolabial
heipes) to atopic deimatitis (see Heipes
Simplex Viius: Widespiead Cutaneous Infec-
tion Associated with Cutaneous Immuno-
compiomise," below).
S . auieus suern[eton : Occuis with eczema
heipeticum.
Ery|ema mu|[orme : In some individuals
with iecuiient HSV infections, eiythema
multifoime may occui with each iecuiience
(Fig. 27-32). (See Eiythema Multifoime,"
Section 7.)
Ceoera| Fodos Fevei may be piesent duiing
symptomatic piimaiy heipetic gingivostoma-
titis.
RegIvnu| Lymphudenvputhy May be fiim,
nonfluctuant, tendei; usually unilateial.
CNS Signs of aseptic meningitis: headache, fe-
vei, nuchal iigidity, CSF pleocytosis with noimal
sugai content and positive HSV CSF cultuie.
FICk 2T-29 herpes smp|ex vrus oIectoo: prmary ovostomatts A +!,e+|o|d |er+|e W|||
|||o|, o| +|op|c de|r+||||. \u|||p|e, .e|, p+|u|u| e|o|ou ou ||e |oWe| pe||o|+| ||u, ||p, +ud |ouue. I/+uc|
re+| W+ po|||.e. n'\ de|ec|ed ou ||A. \e|||c||||ueu|||.e ' c- (\''A) W+ |o|+|ed ou |+c|e||+| cu|
|u|e, ||u upe||u|ec||u ||e |e|pe||c |e|ou. A |u ||| pe|ou, p||r+|, n'\ |u|ec||ou c+ue + |u|.o|or+||||.
n'\ |u|ec||ou |ecu||ed ou ||e |+ce |u| W|||ou| o|+| |u.o|.ereu|.
8
FICk 2T-30 herpes smp|ex vrus oIectoo: recurreot herpes |aba|s . Eder+|ou |+|e|+| uppe|
||p 2+ | +||e| oue| o| ||u||u eu+||ou. 8. C|ouped .e|c|e ou rou|+c|e +|e+ +3 | +||e| oue| o| ,rp|or.
C. C|u|ed e|o|ou ou uppe| ||p +ud rou|+c|e +|e+ 1 d+, +||e| oue| o| ,rp|or. 0. |+|u|u| e|o|ou ou ||e
|oWe| ||p |o| 5 Wee| |u + oo,e+|o|d |er+|e W||| e.e|e de|r+|o|e||o| +ud +c||u|c c|e|||||. I|e d|+uo| W+
r+de ou |e|ou+| ||op,.
C 0
FICk 2T-31 herpes smp|ex vrus
oIectoo: herpetc wht|ow A 9,e+|o|d
r+|e W||| p+|u|u| ||ue| |e|ou |o| ! d+,.
|+|u|u|, |ouped, cou||ueu| .e|c|e ou +u
e|,||er+|ou eder+|ou |+e o| ||e d||+|
||ue| We|e ||e |||| (+ud p|eured p||r+|,)
,rp|or+||c |u|ec||ou.
Frmary Iotraora| hSv IoIectoo Aphthous sto-
matitis, hand-foot-and-mouth disease, heipan-
gina, eiythema multifoime.
kecurreot Iesoo Fixed diug eiuption.
See page 816.
0IACN0SIS
Clinical suspicion confiimed by Tzanck smeai,
viial cultuie, oi antigen detection DFA.
C0kS AN0 Fk0CN0SIS
Recuiiences of HSV tend to become less fie-
quent with the passage of time.
Eczema heipeticum (see also page 825) may
complicate vaiious deimatoses.
Patients with immunodeficiency may expeii-
ence:
Cutaneous dissemination of HSV
Systemic dissemination of HSV
Chionic heipetic ulceis (see also page 828).
Eiythema multifoime may complicate each
episode of iecuiient heipes, occuiiing
1-2 weeks aftei an outbieak.
MANACMNI
See page 816.
FICk 2T-32 herpes smp|ex vrus oIectoo: recurreot erythema mu|tIorme A !,e+|o|d r+|e
W||| |ecu||eu| |e|pe |+||+|| +ud d|er|u+|ed |e|ou. kecu||eu| |e|pe |+||+|| ou ||e |oWe| ||p +ud ||||||e
eder+|ou p+pu|e ou ||e do|ur o| ||e |+ud.
I|+ur||ou occu|.
|u u|e|o
|u||+p+||ur
|o|u+|+| +cqu||||ou.
I|e ro||e| | ||e ro| corrou ou|ce o| |u|ec
||ou.
I|e|e | uu+||, uo e.|deuce o| |edd|u +| ||e
||re o| de||.e|,.
'|edd|u +|o occu| ||or u|e||ue ce|.|\.
I|e r+jo|||, o| |u|ec||ou +|e c+ued |, n'\2,
n'\ | ro|e .||u|eu| |u ||e ueW|o|u +ud +oc|
+|ed W||| |||e| ro|||d||, +ud ro||+|||, |+|e.
10 o| |u|+u| W||| ueou+|+| n'\ |u|ec||ou +|e
|o|u |o ro||e| W||| +,rp|or+||c eu||+| |e|
pe, 10 o| c+e occu| |u ||e |||||o|u c|||d.
E|up||ou occu| ou ||e rucou rer||+ue
(||. 21!! 1) o| ou ||e |u|+c| ||u +| |uocu|+||ou
||e (uc| + rou||o||u euo|) (||. 21!!3).
||er|u+|ed n'\ |u|ec||ou |u ueou+|e | d||||cu||
|o d|+uoe |u ||+| up |o 10 o| |u|+u| |+.e uo
rucocu|+ueou |e|ou.
|\ +c,c|o.|| ||e|+p, | r+ud+|o|, |u ||ee c+e.
N0NAIAI hkFS SIMFIX vIkS INFCII0N
MANACMNI
Frophy|axs
Many expeits iecommend seiotesting foi
HSV-1 and HSV-2 at the fiist pienatal visit.
Infants boin to women who asymptomati-
cally shed HSV have ieduced biith weight and
incieased piematuiity.
Acyclovii suppiessive theiapy at the end of
piegnancy piobably (but not documented)
ieduces the iisk of tiansmission to the
neonate.
Pregnuncy
The safety of systemic acyclovii foi pieg-
nant women and the fetus has not yet been
established, although acyclovii appeais to
be completely safe in last months of pieg-
nancy.
Acyclovii, valacyclovii, and famciclovii aie
active only in cells with active viial infection.
If HSV is acquiied late in piegnancy, cesaiean
section is indicated.
Feroata| IoIectoos
Most motheis of infants who acquiie neona-
tal heipes lack histoiies of clinically evident
genital heipes.
The iisk foi tiansmission to the neonate fiom
an infected mothei appeais highest among
women with fiist-episode genital heipes neai
the time of deliveiy and is low (3%) among
women with iecuiient heipes.
The iesults of viial cultuies duiing piegnancy
do not piedict viial shedding at the time of
deliveiy, and such cultuies aie not ioutinely
indicated.
Aotvra| Iherapy Acyclovii, 20 mg/kg IV q8h
foi 14-21 days.
8
FICk 2T-33 herpes smp|ex
vrus oIectoo: oeooata| |eo
u+|e W||| |e.e| +ud ||u |e|ou. .
\e|c|e +ud c|u|ed e|o|ou ou ||e
uppe| ||p +ud |+|e eo|+p||c u|ce|
+||ou o| ||e |ouue, |.e., |e|pe||c
|u|.o|or+||||. 8. C|ouped +ud
cou||ueu| .e|c|e W||| uude||,|u
e|,||er+ +ud eder+ ou ||e |ou|
de|, +|||u +| ||e |uocu|+||ou ||e.
w|dep|e+d n'\ cu|+ueou |u|ec||ou |u uude||,
|u de|r+|oe occu| ro| corrou|, |u +|op|c
de|r+|||| (ec/er+ |e|pe||cur).
C|+|+c|e||/ed |, W|dep|e+d .e|c|e +ud e|o|ou.
\+, occu| + + p||r+|, o| |ecu||eu| |u|ec||ou.
',, . K+po| .+||ce||||o|r e|up||ou.
hkFS SIMFIX vIkS: WI0SFkA0 CIAN0S INFCII0N ASS0CIAI0 WIIh
CIAN0S IMMN0C0MFk0MIS
Ae oI 0oset Childien adults.
to|oy HSV-1 HSV-2.
Iraosmssoo Commonly fiom paiental hei-
pes labialis to alteied epideimis.
ksk Factors
Most commonly, atopic deimatitis.
Moie seiious infections occui in eiythiodei-
mic atopic deimatitis.
Also, Daiiei disease, theimal buins, pem-
phigus vulgaiis, bullous pemphigoid, ich-
thyosis vulgaiis, cutaneous T cell lymphoma
(mycosis fungoides), Wiscott-Aldiich syn-
diome.
FAIh0CNSIS
See Heipes Simplex Viius Infection," page 813.
Piimaiy eczema heipeticum may be associ-
ated with fevei, malaise, iiiitability.
When iecuiient, histoiy of piioi similai
lesions; systemic symptoms less seveie.
Piimaiy skin disease may be piuiitic; onset of
eczema heipeticum associated with pain and
tendeiness.
Lesions begin in abnoimal skin and may
extend peiipheially foi seveial weeks duiing
the piimaiy infection oi secondaiy eiuption.
Supeiinfection with S. aureus is ielatively
common and may be painful
Vesicles evolving into punched-out" eiosions
(Fig. 27-34).
Vesicles aie fiist confined to eczematous skin.
In contiast to piimaiy oi iecuiient HSV
eiuptions, no giouped but disseminated.
May latei spiead to noimal-appeaiing skin.
FICk 2T-34 herpes smp|ex vrus oIectoo: ectema herpetcum oo eye|ds A !o,e+|o|d r+|e
W||| |ecu||eu| pe||o||||+| p+|u|u| c|u|ed e|o|ou +ud +|op|c de|r+||||. 'r+|| c|u|ed e|o|ou ou ||e e,e||d. ||A
de|ec|ed n'\. B+c|e||+| cu||u|e |epo||ed \''A +ud |oup A ||ep|ococcu (CA'). I|e |e|pe||c |u|ec||ou |+d uo|
+||ec|ed ||e co|ue+.
Eiosions may become confluent, pioducing
laige denuded aieas (Fig. 27-35).
Successive ciops of new vesiculation may occui.
Common sites: face, neck, tiunk.
Ceoera| xamoatoo Piimaiy infection may
be associated with fevei and lymphadenopathy.
Wdespread vescu|opustu|es[rosoos Vaii-
cella, disseminated VZV infection, disseminated
(systemic) HSV infection, wound infection (sta-
phylococcal, pseudomonal, CanJJa ), eczema
vaccinatum.
See page 816.
0IACN0SIS
Clinical, confiimed by detection of HSV on
cultuie oi antigen detection.
C0kS AN0 Fk0CN0SIS
Untieated, piimaiy episode of eczema hei-
peticum iuns its couise with iesolution in
2-6 weeks.
Recuiient episodes tend to be mildei and not
associated with systemic symptoms.
Systemic dissemination can occui, especially
in immunocompiomised patients; iepoited
moitality iates iange fiom 10-50%.
Widely distiibuted cutaneous HSV infection
in buin patients can be difficult to detect
clinically.
MANACMNI
Maoaemeot oI oder|yo 0ermatoss Foi
atopic deimatitis, see Eczema/Deimatitis," Sec-
tion 2.
Aotvra| Iherapy See Management," page
816.
Aotbactera| Iherapy Tieat associated bacte-
iial supeiinfection. See Bacteiial Infections
Involving the Skin," Section 24.
FICk 2T-35 herpes smp|ex vrus oIectoo: exteosve ectema herpetcum oo Iace Cou||ueu| +ud
d|c|e|e c|u|ed e|o|ou +oc|+|ed W||| e|,||er+ +ud eder+ o| ||e |+ce o| + |er+|e W||| +|op|c de|r+||||.
n'\ |u ||e |o| W||| ,|er|c |rruuocorp|o
r|e r+, c+ue.
|oc+| |u|ec||ou W||| e\|eu|.e cu|+ueou |u
.o|.ereu| (e.., ec/er+ |e|pe||cur)
C||ou|c |e|pe||c u|ce|
w|dep|e+d ,|er|c |u|ec||ou (W|dep|e+d ru
cocu|+ueou |e|ou + We|| + ,|er|c |u|ec
||ou).
hkFS SIMFIX vIkS: INFCII0NS ASS0CIAI0 WIIh SSIMIC
IMMN0C0MFk0MIS
FI0MI0I0C
Iocdeoce
Incieasing due to an incieasing population of
immunocompiomised individuals:
80% in bone maiiow tiansplant iecipients
65% in solid oigan tiansplant iecipients
60% in those with lymphoma
55% in those with leukemia
25% in individuals with HIV/AIDS disease.
Incidence of symptomatic outbieaks has
decieased maikedly because of the piimaiy
piophylaxis of HSV-seiopositive immuno-
compiomised individuals with oial antiviial
diugs.
ksk Factors
1mmunvde]IcIency: H1V/A1DS 1n]ectIvn
Fiequency and duiation inciease shaiply as
CD4 T cell count falls to 50/L.
Reduced in peisons effectively tieated with
ART.
In most HIV-infected individuals, fiequency,
duiation, and seveiity of HSV outbieaks
similai to those in immunocompetent indi-
viduals; howevei, asymptomatic shedding is
incieased.
Disseminated cutaneous and visceial HSV
infections aie less common than in othei
immunocompiomised states.
CDC Sure||ante Case De[non [or IDS :
HSV infection foi any duiation in a patient
oldei than 1 month is an AIDS-defining
condition if the patient has no othei cause of
immunodeficiency and is without knowledge
of HIV antibody status.
HSV infection causing a mucocutaneous
ulcei that peisists longei than 1 month
HSV infection causing bionchitis, pneu-
monitis, oi esophagitis Immune iestoia-
tion with highly active ART has maikedly
ieduced the incidence of seiious HSV
infections.
Leu|emIu/Lymphvmu HSV ieactivation typi-
cally occuis duiing induction oi ieinduction
within 20 days in individuals with latent HSV
infection.
Bvne Murrvw Trunsp|untutIvn (BMT)
HSV ieactivation occuis within the fiist
5 weeks aftei BMT (median, day 8 posttians-
plantation).
Untieated, 3% of patients die fiom dissemi-
nated HSV infection.
Chemvtherupy
Foi solid oigan oi BMT, congenital oi
acquiied cellulai immune defects.
Cytotoxic cancei chemotheiapy
Glucocoiticoid theiapy.
Other
Autoimmune diseases, malnutiition
Raiely, piegnancy.
Radiotheiapy.
Instiumentation such as nasogastiic tube
in debilitated patient associated with HSV
esophagitis.
FAIh0CNSIS
60-80% of HSV-seiopositive tiansplant iecip-
ients and patients undeigoing chemotheiapy
foi hematologic malignancies will expeiience
ieactivation of HSV.
Aftei viiemia, disseminated cutaneous oi vis-
ceial HSV infection may occui.
Factois deteimining whethei seveie localized
disease, cutaneous dissemination, oi visceial
dissemination will occui aie not well defined.
Patients often hospitalized with undeilying
condition oi disease.
Sko Symptoms Tendei and painful mucocu-
taneous ulceis.
Returren |eret |eson: Mild pain in ulceis.
C|ront |eret u|ters : Mild to modeiate
pain.
Heret w||ow. Seveie pain.
Oro|aryngea| u|ters : Oial pain on eating.
Eso|agea| u|ters : Retiosteinal pain and/oi
painful swallowing (odynophagia) and/oi
dysphagia.
noreta| u|ters : Peiianal/anal ulceis aie
usually quite painful. Anoiectal ulceis aie
associated with pain, constipation, pain on
defecation, dischaige, tenesmus, and at times,
sacial iadiculopathy, impotence, neuiogenic
bladdei.
Mutotuaneous Jssemnaon: Fevei.
Visceral Dissemination Fevei, deteiioiation of
clinical status.
PrImury 1n]ectIvn
Local infection may be widespiead on the face
(Fig. 27-36), oiophaiynx, anogenital iegion
with initial vesiculation followed by ciusted
eiosions.
Without antiviial theiapy, lesions may peisist
to become chionic heipetic ulceis.
Recurrent HerpetIc LesIvns
In most immunocompiomised peisons, le-
sions appeai as in the immunocompetent
host.
Howevei, outbieaks may piesent with iecui-
ient lesions (giouped ciusts, eiosions, ulceis)
in a much laigei aiea of involvement than
usual.
In HIV/AIDS-infected peisons, laige, neciotic,
eioded lesions may appeai ovei a peiiod of a
few days without appaient vesicle foimation.
ChrvnIc HerpetIc U|cers
Recuiient lesions enlaige ovei weeks to
months, foiming laige ulceis, 10-20 cm in
diametei (Fig. 27-37).
Maigins may be slightly iolled, hypeiplas-
tic.
Coalescence of ulceiations may iesult in lin-
eai ulceis in inteigluteal cleft oi inguinal fold.
Base of ulcei may be ciusted oi moist.
Painful on palpation. Peiianal and/oi iectal
genital oiofacial digital.
Uncommonly, ulcei on face and peiineum
simultaneously.
Orvphuryngeu| U|cers Laige ulceiations
occui on the haid palate, often at the site of
dental extiaction. Lineai ulceiations occui on
the tongue.
Esvphugeu| U|cers
Usually associated with oiophaiyngeal hei-
petic ulcei and swallowing HSV-infected
saliva.
Esophagoscopy: mucosal eiosions/ulceiation.
GenItu|, PerIneu|, PerIunu|, Anvrectu| U|cers
Acute ulceiation of the vulva penis
(Fig. 27-37), sciotum, and/oi peiineum may
become chionic ulceis unless effectively
tieated.
In individuals infected with acyclovii-iesistant
HSV, ulceiations do not iespond to usual
antiviial theiapies.
Anal ulceis usually occui via enlaigement of
peiianal ulceis.
Heipetic pioctitis: sigmoidoscopy shows fii-
able mucosa and ulceiations.
Mucvcutunevus DIssemInutIvn
Disseminated (nongiouped) vesicles and pus-
tules often hemoiihagic with inflammatoiy
halo; quickly iuptuie, iesulting in punched-
out" eiosions.
FICk 2T-36 herpes smp|ex vrus oIectoo:
prmary oIectoo o hIv[AI0S A !5,e+|o|d
r+|e W||| n|\/A||' (C|+ ce|| couu|, +00/r|). Cou
||ueu| .e|c|e +ud e|o|ou W||| uude||,|u e|,||er+
+ud eder+ (5 |o o d+,' du|+||ou) |u ||e |e+|d +|e+.
C|u|.o|or+|||| +ud +cu|e |,rp|+deuop+||, We|e
+|o p|eeu|, W||| oue| 5 d+, +||e| o|oeu||+| e\.
FICk 2T-3T herpes smp|ex vrus oIec-
too: chrooc u|cers o hIv[AI0S A +,e+|
o|d r+|e W||| n|\/A||' W||| p+|u|u| |e|ou ou
|u||oc| |o| o rou||. |+|e cou||ueu| u|ce| ou
||e |u||oc| +ud pe||+u+| +|e+. n'\2 W+ |e|
|+u| |o +c,c|o.||, |u| |eo|.ed W||| |\ |oc+|ue|.
u|ce| |ecu||ed du||u |+d|+||ou |o ||e +|e+ |o|
re|+|+||c qu+rou ce|| c+|c|uor+ ('CC).
FICk 2T-38 varce||a-toster vrus oIectoo: varce||a \u|||p|e, .e|, p|u||||c, e|,||er+|ou p+pu|e,
.e|c|e ('deWd|op ou + |oe pe|+|), +ud c|u|ed p+pu|e ou e|,||er+|ou, eder+|ou |+e ou ||e |+ce +ud
uec| o| + ,ouu |er+|e. I|e pec||ur o| |e|ou, +|||u o.e| 1 |o 0 d+,, | |,p|c+| o| .+||ce||+.
Lesions may be neciotic and then ulceiate
(Fig. 27-38).
Ulceis may become confluent with polycy-
clic well-demaicated boideis; edges may be
slightly iaised, iolled.
1n]urctIve S|In LesIvns If complicated by
puipuia fulminans. (See Section 19)
Mucvus Memhrunes Oiophaiyngeal eiosion:
neciotizing gingivitis, palatal ulceis, glossitis.
Ceoera| xamoatoo
Oiophaiyngeal lesions can occui in the
absence of exteinal facial lesions.
HSV esophagitis, tiacheobionchitis, and focal
pneumonitis can be local infection associated
with spiead by aspiiated oi swallowed secie-
tions.
Diffuse inteistitial pneumonitis can be a
manifestation of hematogenous infection.
HSV pneumonitis often iesults fiom endog-
enous ieactivation.
Widespiead visceial involvement (livei, lungs,
adienals, GI tiact, CNS) can occui in seveiely
immunocompiomised peisons.
varatoos wth SpecIc States
oI Immuoocompromse
Ieukema[Iymphoma
HSV infection is often atypical, with extensive
lesions on lips oi nasolabial skin.
Oiophaiyngeal infection manifested as
neciotic gingival papillae, intiaoial ulceia-
tions that mimic thiush, oi mucositis fiom
chemotheiapy oi iadiotheiapy.
hIv[AI0S 0sease
Effective ART has diamatically ieduced
the occuiience of seveie HSV infection in
advanced HIV/AIDS.
HSV ieactivation is usually local, with chionic
heipetic ulceis on the face oi anogenital
iegion.
HSV esophagitis may coexist with candidal
esophagitis.
Peisistent peiianal heipes and heipetic pioc-
titis.
Disseminated visceial disease is uncom-
mon.
Chrooc herpetc |cers Chionic VZV
infection, wound infection, ecthyma, ecthyma
gangienosum, piessuie ulcei, deep mycotic
(ciyptococcal, histoplasmal, blastomycotic, coc-
cidioidal) ulcei.
0ropharyoea| |cers Aphthous ulceis, lym-
phoma, histoplasmosis with oial ulcei.
sophaea| |cers CMV ulceis, aphthous (idi-
opathic) ulceis, CanJJa esophagitis, histoplas-
mosis with esophageal ulcei.
Aoorecta| |cers HPV-induced squamous cell
caicinoma, Ciohn disease; amebiasis, chionic
iectal abuse of eigot alkaloids.
Mucocutaoeous 0ssemoatoo VZV infec-
tion (vaiicella, disseminated heipes zostei),
eczema heipeticum, eczema vaccinatum, dis-
seminated vaccinia in immunosuppiessed pa-
tients.
See pages 816.
roa|yss Hematuiia due to HSV cystitis.
0IACN0SIS
Clinical suspicion confiimed by Tzanck smeai,
positive HSV antigen detection DFA, oi isola-
tion of HSV on viial cultuie.
C0kS AN0 Fk0CN0SIS
In most immunocompiomised individuals
with ieactivation of HSV, clinical
manifestations diffei little fiom infections in
healthy hosts.
In ienal tiansplant iecipients, HSV is excieted
in thioat washings of 80% of patients shoitly
aftei giafting; two-thiids of those excieting
HSV develop lesions shoitly aftei excietion is
detected.
In some immunocompiomised individuals,
howevei, laige ulceiations can peisist foi
weeks to yeais.
Heipetic ulceis facilitate supeiinfection with
bacteiia ( S. aureus oi fungi).
HSV may disseminate to livei, lungs, adienals,
GI tiact, CNS.
Visceial infection can be complicated by
disseminated intiavasculai coagulation,
which has a veiy high moitality iate.
Factois deteimining whethei seveie localized
disease, cutaneous involvement, oi visceial
dissemination will occui in an individual aie
not well defined.
In HIV/AIDS, peisons successfully tieated
with ART expeiience ieduction in fiequency
and seveiity of HSV iecuiiences.
Chionic heipetic ulceis that fail to iespond
to acyclovii should be evaluated piomptly foi
the piesence of iesistant viius.
Infection with acyclovii-iesistant stiains
iesults in chionic, piogiessive ulceiations
that peisist and/oi continue to enlaige
despite oial and IV acyclovii tieatment.
These ulceis can enlaige to 20-30 cm in
diametei and aie associated with majoi
moibidity and pain.
MANACMNI
Freveotoo tyt|or ro|y|axs foi seio-
positive patients undeigoing bone maiiow
tiansplantation, induction theiapy foi leuke-
mia, oi solid oigan tiansplantation: acyclovii,
5 mg/kg IV q8h oi 400 mg PO thiee times a
day, fiom the day of conditioning, induction, oi
tiansplantation foi 4-6 weeks suppiesses both
HSV and VZV ieactivation. Also oial valacyclo-
vii, famciclovii.
Systemc Aotvra| Iherapy See Management,"
page 816.
\+||ce||+ /o|e| .||u (\/\) | + |ur+u |e|pe.|
|u ||+| |u|ec| 93 o| +du|| popu|+||ou.
|||r+|, \/\ |u|ec||ou (.+||ce||+ o| c||c|eupo\) |
ue+||, +|W+, ,rp|or+||c +ud c|+|+c|e||/ed |,
d|er|u+|ed p|u||||c .e|c|e.
|u||u p||r+|, |u|ec||ou, \/\ e|+||||e |||e|ou
|u|ec||ou |u euo|, +u||+.
w|eu |rruu||, |o \/\ dec||ue, \/\ |e+c||.+|e
W||||u ||e ue|.e ce||, ||+.e||u doWu ||e ueu|ou
|o ||e ||u, W|e|e || e|up| |u + de|r+|or+| p+|
|e|u, |.e., |e|pe /o|e| (n/), o| ||u|e.
|u ||e |rruuocorp|or|ed |o|, p||r+|, +ud
|e+c||.+|ed \/\ |u|ec||ou | o||eu ro|e e.e|e,
+oc|+|ed W||| |||e| ro|||d||, |+|e +ud ore
ro||+|||,.
\/\ .+cc|ue |+ |educed ||e |uc|deuce o| .+||ce||+
+ud |e|pe /o|e|.
|C|9 . 052
|C|0 . B0
vAkICIIA I0SIk vIkS INFCII0NS
Ae oI 0oset
Without immunizaton, 90% of cases occui in
childien 10 yeais, 5% in peisons oldei than
15 yeais.
With immunization (Vaiivax), the incidence
is maikedly ieduced.
to|oy
VZV, a heipesviius.
Stiuctuially similai to othei heipesviiuses:
Lipid envelope suiiounding nucleocapsid
with icosahedial symmetiy
Total diametei of appioximately 150-200
nm
Centially located double-stiand DNA with
a moleculai weight of 80 million
Iraosmssoo
Aiiboine dioplets as well as diiect contact
Indiiect contact uncommon
Patients aie contagious seveial days befoie
vaiicella exanthem appeais and until last ciop
of vesicles
Ciusts aie not infectious.
VZV can be aeiosolized fiom skin of indi-
viduals with heipes zostei, which is about
one-thiid as contagious as vaiicella, causing
vaiicella in susceptible contacts.
Seasoo In metiopolitan aieas in tempeiate
climates, vaiicella epidemics occui in wintei
and spiing.
FAIh0CNSIS
In vaiicella, VZV is thought to entei thiough
mucosa of uppei iespiiatoiy tiact and
oiophaiynx.
Followed by local ieplication and piimaiy
viiemia.
VZV then ieplicates in cells of ieticuloen-
dothelial system with subsequent second-
aiy viiemia and dissemination to skin and
mucous membianes.
Localization of VZV in the basal cell layei
is followed by viius ieplication, ballooning
degeneiation of epithelial cells, and accumu-
lation of edema fluid.
Second episodes of vaiicella have been docu-
mented but aie iaie.
Duiing the couise of vaiicella, VZV passes
fiom the skin lesions to the sensoiy neives,
tiavels to the sensoiy ganglia, and establishes
latent infection.
In heipes zostei, humoial and cellulai
immunity to VZV established with piimaiy
infection ebbs natuially oi because of an
undeilying cause of immunocompiomise.
Results in VZV ieplication in sensoiy gan-
glia.
VZV then tiavels down the sensoiy neive,
iesulting in initial deimatomal pain, followed
by skin lesions.
Since the neuiitis piecedes the skin involve-
ment, pain oi itching appeais befoie the skin
lesions aie visible.
The locations of pain aie vaiied and ielate
diiectly to the ganglion wheie VZV has
emeiged fiom latency to active infection.
Piodiomal symptoms may appeai initially in
the tiigeminal, ceivical, thoiacic, lumbai, oi
sacial deimatome.
Postheipetic neuialgia (PHN) is ieflex sym-
pathetic dystiophy (complex iegional pain
syndiome).
vIv Aoteo 0etectoo 0FA Smeai of vesicle
fluid oi sciaping fiom ulcei base/maigin: Diiect
fluoiescent antibody (DFA) test detects VZV-
specific antigens. Sensitive and specific method
foi identifying VZV-infected lesions. Highei
yield than VZV cultuies.
vra| Cu|tures Isolation of viius on viial
cultuie (human fibioblast monolayeis) fiom
vesiculai skin lesions, biopsy specimens, coineal
sciaping, and CSF is possible but moie difficult
than foi HSV. Distinctive cytopathic effects
usually appeai in 3-10 days. Vesicle fluid can
be cultuied.
Itaock Smear Cytology of fluid oi sciap-
ing fiom base of vesicle oi pustule shows
both giant and multinucleated acantholytic
epideimal cells (as does that of HSV infections)
(see Fig. 27-27).
Sero|oy Seioconveision documents piimaiy
VZV infection.
0ermatopatho|oy Lesional skin oi visceial
biopsy specimen shows multinucleated giant
epithelial cells indicating HSV-1, HSV-2, oi
VZV infection. Immunopeioxidase stains spe-
cific foi HSV-1, HSV-2, oi VZV antigens can
identify the specific heipesviius.
FI0MI0I0C
Ae oI 0oset See page 831.
Iocdeoce
Since intioduction of vaiicella vaccine in
1995, incidence of vaiicella has decieased as
vaccination coveiage has incieased.
Piioi to 1995, 3-4 million cases in the United
States annually.
Iraosmssoo See page 831.
Seasoo See page 831.
Iocubatoo Ferod 14 days (iange, 10-23 days).
Frodrome
Chaiacteiistically absent oi mild.
Uncommon in childien, moie common in
adults: headache, geneial aches and pains,
seveie backache, malaise.
Exanthem appeais within 2-3 days.
hstory Exposuie at day caie, school, to oldei
sibling; ielative with zostei.
Sko Symptoms Exanthem usually quite piu-
iitic.
Sko Iesoos
In most childien, illness begins with appeaiance
of exanthem:
Vesiculai lesions evident in successive
ciops.
Often single, disciete lesions oi scanty in
numbei in childien and much moie dense in
adults.
Initial lesions aie au|es (often not obseived)
that may appeai as w|ea|s and quickly evolve
to est|es and initially appeai as small diops
of watei" oi dewdiops on a iose petal"
(Fig. 27-39), supeificial and thin-walled with
suiiounding eiythema.
I|e ||||, cou|+|ou p||r+|, |u|ec||ou c+ued |,
.+||ce||+/o|e| .||u.
|| | c|+|+c|e||/ed |, ucce|.e c|op o| p|u||||c
.e|c|e ||+| e.o|.e |o pu|u|e, c|u|, +ud +|
||re, c+|.
I|| |u|ec||ou | o||eu +ccorp+u|ed |, r||d cou
|||u||ou+| ,rp|or
|||r+|, |u|ec||ou occu|||u |u +du|||ood r+, |e
corp||c+|ed |, pueurou|+ +ud eucep|+||||.
',, . C||c|eupo\.
vAkICIIA
Vesicles become umbilicated and iapidly
evolve to usu|es and truss ovei an 8- to
12-h peiiod.
With subsequent ciops, all stages of evolution
may be noted simultaneously, i.e., papules,
vesicles, pustules, ciusts, i.e., polymoiphic.
Ciusts fall off in 1-3 weeks, leaving a pink,
somewhat depiessed base.
Chaiacteiistic punched-out peimanent scais
may peisist.
Uncommonly, hemoiihage into pustulai
lesion occuis in otheiwise healthy childien,
i.e., |emorr|agt arte||a .
Complications
Supeiinfection by methicillin-sensitive
S . aureus (MSSA), methicillin-iesistant S .
aureus (MRSA), oi GAS
Impetigo, fuiuncles, cellulitis, and gan-
giene.
DIstrIhutIvn Fiist lesions begin on face
(Fig. 27-39) and scalp, spieading infeiioily to
tiunk and extiemities.
Most piofuse in aieas least exposed to pies-
suie, i.e., back between shouldei blades,
flanks, axillae, popliteal and anticubital fos-
sae
Density highest on tiunk and face, less on
extiemities
Palms and soles usually spaied.
Mucvus Memhrunes Vesicles (not often
obseived) and subsequent shallow eiosions
(2-3 mm)
Most common on palate
Mucosa of nose, conjunctivae, phaiynx, laiynx,
tiachea, GI tiact, uiinaiy tiact, vagina.
Ceoera| xamoatoo Low-giade fevei. Ves-
icopustules may occui in iespiiatoiy, GU, and
GI tiacts.
PneumvnItIs Occuis with incieased fie-
quency in:
Immunocompiomised individuals of all ages
Immunocompetent adolescents and adults
Moie fiequent/seveie in piegnancy.
3-16% of healthy adults with vaiicella have
iadiologic evidence of VZV pneumonitis
(diffuse inteistitial lobulai infiltiate). One-
thiid of these will have iespiiatoiy symp-
toms.
Piioi to antiviial theiapy, moibidity/moitality
was high.
CNS Most commonly, vaiicella with ceiebel-
lai ataxia and encephalitis.
varaots
8actera| SuperoIectoo Most commonly,
S. aureus oi GAS can cause impetigo, ecthyma,
cellulitis, neciotizing fasciitis, oi toxic shock
syndiome in vaiicella lesions.
"Ma|oaot" varce||a
Immunosuppiessed oi glucocoiticoid-
tieated individuals may develop pneumo-
nitis, hepatitis, encephalitis, disseminated
intiavasculai coagulation, and puipuia ful-
minans.
Continued VZV ieplication and dissemina-
tion iesult in piolonged high-level viiemia,
moie extensive iash, longei peiiod of new
vesicle foimation.
Wdespread vesc|es[Crusts Smallpox (all
lesions aie in the same stage), disseminated
HSV infection, cutaneous dissemination of
zostei, eczema heipeticum, eczema vacci-
natum, disseminated vaccinia in immuno-
suppiessed patients (smallpox vaccination
still given in the U.S. militaiy), iickettsial-
pox, enteioviius infections, bullous foim of
impetigo.
See Laboiatoiy Examinations," page 832.
8actera| Cu|tures Rule out supeiinfection
with S. aureus oi gioup A stieptococcus.
Sero|oy Seioconveision, i.e., fouifold oi
gieatei iise in VZV titeis.
0IACN0SIS
Usually made on clinical findings alone.
C0kS AN0 Fk0CN0SIS
In healthy childien, the couise is self-limited;
howevei, a moitality iate of 1 pei 50,000 cases
in the United States had been iepoited piioi to
VZV immunization (100 deaths annually in the
3-4 million cases). Pieviously, 6500 hospitaliza-
tions annually (United States) foi vaiicella.
The most common complication of vaiicella
in childien 5 yeais is bacteiial ( S. aureus ,
GAS) supeiinfection. Bacteiemia.
In childien 5-11 yeais of age, the most com-
mon complications aie vaiicella encephalitis
and Reye syndiome.
In adults, piodiomal symptoms aie common
and may be seveie;
Exanthem may last foi a week oi moie, with
piolonged peiiod of iecoveiy.
Piimaiy vaiicella pneumonia, which piesents
1-6 days aftei appeaiance of iash, is ielatively
common in adults: 16% of adults show x-iay
evidence of pneumonitis, but only 4% have
clinical signs of pneumonitis. Women have a
10% iisk of seveie VZV pneumonitis.
VZV encephalitis may also complicate vaii-
cella in adults.
Less common complications of vaiicella
include viial aithiitis, uveitis, conjunctivitis,
caiditis, inappiopiiate antidiuietic hoimone
syndiome, nephiitis, and oichitis.
The moitality iate in adults was 15 pei
50,000 cases (U.S.); 25% of vaiicella-
associated deaths did occui in adults.
Mateinal vaiicella duiing the fiist tiimestei of
piegnancy:
Fetus: Fetal vaiicella syndiome (limb hypo-
plasia, eye and biain damage, skin lesions)
in 2% of exposed fetuses.
Neonatal vaiicella has highei associated
incidence of pneumonitis and encephalitis
than occuis in oldei childien.
Immunocompiomised oi glucocoiticoid-
tieated patients with vaiicella may manifest
dissemination, hepatitis, encephalitis, and
hemoiihagic complications.
If vaiicella occuis at an eaily age when matei-
nal antibody is still piesent, an individual can
have a second episode of vaiicella.
In HIV-infected patients, ieactivation of VZV
may iesult in chionic painful ecthymatous
vaiicella.
In immunocompiomised individuals, VZV hep-
atitis and pneumonitis aie ielatively common
and aie associated with significant moitality.
FICk 2T-39 varce||a-toster
vrus oIectoo: varce||a A
+o,e+|o|d |er+|e W||| p|u||||c e|up
||ou |o| 2 d+,. \u|||p|e, p|u||||c,
e|,||er+|ou p+pu|e, .e|c|e ou ||e
|+ce, uec|, +ud c|e|. 'e.e|+| .e|c|e
|+.e e.o|.ed |o c|u|ed e|o|ou. ||A
de|ec|ed \/\. |o +u|||od|e |o \/\
We|e de|ec|ed.
FICk 2T-40 varce||a toster
vrus oIectoo: varce||a mmu-
otatoo varce||a A,rp|or+||c
,e+|o|d r+|e W||| |||o|, o| .+||ce||+
|rruu|/+||ou 0 d+, |e|o|e ||e oue|
o| |+|. 'c+||e|ed |ed p+pu|e +ud
.e|c|e d|er|u+|ed ou ||e c|e|. I|e
.+cc|ue cou|+|u ||.e .||u. Ceue|+||/ed
.+||ce||+|||e |+| occu| |u !.3 o|
|u|+u| |rruu|/ed, 5 |o 2o d+, +||e|
|rruu|/+||ou.
MANACMNI
Freveotoo
|rruu|/+||ou \/\ |rruu|/+||ou | uoW +.+||+||e (\+||.+\) +ud | 30 e||ec||.e |u p|e.eu||u
,rp|or+||c p||r+|, \/\ |u|ec||ou. 5 o| ueW|, |rruu|/ed c|||d|eu
de.e|op |+| (||. 21+0). I|oe +| ||| ||| |o| .+||ce||+, W|o |ou|d |e
|rruu|/ed, |uc|ude. uo|r+| \/\ue+||.e +du||, c|||d|eu W||| |eu|er|+,
+ud |rruuocorp|or|ed |ud|.|du+| (|rruuoupp|e|.e ||e+|reu|, n|\
|u|ec||ou, c+uce|). \/\ .+cc|ue |eu|| |u |o|| ce||red|+|ed |rruu||, +ud
+u|||od, p|oduc||ou ++|u| ||e .||u. |rruu|/+||ou W||| \/\ .+cc|ue r+,
|oo| |uro|+| +ud ce||red|+|ed |rruu||, +ud dec|e+e ||e |uc|deuce o|
/o|e| |u popu|+||ou W||| dec||u|u \/\pec|||c |rruu||,.
Symptomatc therapy
|o||ou |||ec|ed +| |educ|u p|u|||u.
0|+| +u|||||+r|ue App||c+||ou |.e |o|||e|r |e||e| o| p|u|||u.
C+u||ou |e +u||p,|e||c +eu| Au||p,|e||c +dr|u|||+||ou | o| couce|u |ec+ue o| + po|||e ||u| |e|Weeu +p|||u
+ud ke,e ,ud|ore |u c|||d|eu W||| .+||ce||+.
Aotvra| aeots
0||e|W|e |e+|||, p+||eu| || |euu W||||u 2+ | +||e| oue| o| .+||ce||+, dec|e+e ||e e.e|||, o| .+||ce||+ +ud
|educe ecoud+|, c+e.
Ac,c|o.|| 20 r/| (300 r+\|rur) |ou| ||re d+||, |o| 5 d+,
\+|+c,c|o.|| E||ec||.e |u| uo| +u +pp|o.ed ue, do|u +re + |o| |e|pe /o|e|.
|+rc|c|o.|| E||ec||.e |u| uo| +u +pp|o.ed ue, do|u +re + |o| |e|pe /o|e|.
\/\ |u|ec||ou (.+||ce||+ o|
/o|e|) |u
|rruuocorp|or|ed
p+||eu|
Ac,c|o.|| 0 r/| |\ q3| |o| 1 d+,
|oc+|ue| (|u +c,c|o.|| +0 r/| |\ q3| |o| 1 d+,
|e||+uce)
Ireatmeot oI bactera|
superoIectoo
\up||oc|u o|u|reu| |||ec|ed +| ' c- +ud/o| |oup A ||ep|ococcu.
0|+| +u||||o||c App||ed |W|ce d+||, |o |e|ou. 'ee I+||e 2+.
*|u Eu|ope, o|+| +c,c|o.|| | ou|, |+|e|, ued |ec+ue o| |e||e| ||o+.+||+|||||, o| .+|+c,c|o.|| +ud |+rc|c|o.||.
FI0MI0I0C
Ae oI 0oset Moie than 66% aie 50 yeais of
age; 5% of cases in childien 15 yeais.
Iocdeoce
In the United States, neaily 100% of adults
aie seiopositive foi anti-VZV antibodies by
the thiid decade of life and aie thus at iisk foi
ieactivation of latent VZV.
Moie than 500,000 cases of HZ annually.
Cumulative lifetime incidence: 10-20%.
HIV/AIDS
In one cohoit, 5% of individuals with HZ
weie HIV-infected and 5% had cancei.
Recuiient HZ 1% of cases.
Occuis in 25% of HIV-infected individuals,
an eight times highei incidence than the
geneial population, ages 20-50 yeais;
Renal and caidiac tiansplant iecipients:
7-9%
Recuiient HZ moie common in immuno-
compiomised individuals.
Immunization to VZV in childhood will altei
the epidemiology of HZ.
ksk Factors
Most common factoi is diminishing immu-
nity to VZV with advancing age, with most
cases occuiiing in those 55 yeais.
Howevei, in most cases tiiggeiing factois aie
not known.
Immunocompiomise:
Malignancy
Immunosuppiession, especially fiom lym-
phopiolifeiative disoideis and chemothei-
apy
Radiotheiapy.
HIV/AIDS: eightfold incieased incidence
of HZ.
Au +cu|e de|r+|or+| |u|ec||ou +oc|+|ed W|||
|e+c||.+||ou o| \/\
C|+|+c|e||/ed |,
uu||+|e|+| p+|u
A .e|cu|+| o| |u||ou e|up||ou ||r||ed |o +
de|r+|ore() |uue|.+|ed |, + co||epoud|u
euo|, +u||ou.
I|e r+jo| ro|||d||, | po||e|pe||c ueu|+||+
(|n|).
',, . '||u|e.
hkFS I0SIk (hI) |C|9. 05!

|C|0. B02
Fathoeoess
In vaiicella VZV passes fiom lesions in the
skin and mucosa via sensoiy fibeis centiip-
etally to sensoiy ganglia.
In the ganglia the viius establishes lifelong
latent infection.
Reactivation occuis in those ganglia in which
VZV has achieved the highest density and is
tiiggeied by immunosuppiession, tiauma,
tumoi, oi iiiadiation (see iisk factois).
Reactivated viius can no longei be con-
tained.
Viius multiplies and spieads antidiomically
down the sensoiy neive to the skin/mucosa
wheie it pioduces the chaiacteiistic vesicles
(Image 27-2).
C|assIcatoo HZ manifests in thiee distinct
clinical stages: piodiome, active infection,
chionic: postheipetic neuialia (PNH).
0uratoo oI Symptoms
Piodiomal stage: Neuiitic pain oi paiesthesia
piecedes foi 2-3 weeks (84% of cases).
Acute vesiculation: 3-5 days.
Ciust foimation: days to 2-3 weeks.
PHN: months to yeais.
Chionic pain oi PHN is that peisisting
aftei the lesions have healed oi peisisting
4 weeks aftei the onset of lesions, iegaidless
of degiee of healing.
Sko Symptoms
Prvdrvmu| Stuge
Pain (stabbing, piicking, shaip, boiing, pen-
etiating, lancinating, shooting), tendeiness,
paiesthesia (itching, tingling, buining, fieeze-
buining) in the involved deimatome piecedes
the eiuption.
Allodynia: heightened sensitivity to mild
stimuli.
ActIve VesIcu|utIvn Skin lesions may be piu-
iitic but in themselves aie not painful.
Zvster SIne Zvster Neive involvement can
occui without cutaneous zostei.
AhdvmInu| Zvster Piesents with seveie
abdominal (oi chest pain) that may piecede
iash by houis to days.
ChrvnIc Stuges PHN, desciibed as buining,"
ice-buining," shooting," oi lancinating," can
peisist foi weeks, months, oi yeais aftei the
cutaneous involvement has iesolved.
Piodiomal stage and active vesiculation: flu-
like symptoms such as headache, malaise,
fevei.
Chionic stages: depiession is veiy common in
individuals with PHN.
Papules (24 h) vesicles-bullae (Fig. 27-41
to 43) (48 h) pustules (96 h) ciusts
(7-10 days).
New lesions continue to appeai foi up to
1 week.
Eiythematous, edematous base (Fig. 27-42)
with supeiimposed cleai vesicles, sometimes
hemoiihagic.
The vesicle-bulla is oval oi iound, may be
umbilicated.
Vesicles eiode foiming ciusted eiosions (Fig.
27-44).
Neciotic and gangienous lesions sometimes
occui.
Scaiiing is common aftei healing of HZ (Fig.
27-45).
DIstrIhutIvn
Unilateial, deimatomal (Image 27-3).
Two oi moie contiguous deimatomes may be
involved (Fig. 27-41, B).
Noncontiguous deimatomal zostei is iaie.
Hematogenous dissemination to othei skin
sites in 10% of healthy individuals (Fig. 27-
43B).
SIte v] PredI|ectIvn Thoiacic (50%), tiigemi-
nal (10-20%) (Figs. 27-42, 27-43, 27-45, 27-46),
lumbosacial and ceivical (10-20%).
Mucvus Memhrunes Vesicles and eiosions
occui in mouth, vagina, and bladdei, depending
on deimatome involved.
Ceoera| xamoatoo
Lymphudenvputhy Regional nodes diaining
the aiea aie often enlaiged and tendei.
Sensvry vr Mvtvr Nerve Chunges Detectable
by neuiologic examination. Sensoiy defects
(tempeiatuie, pain, touch) and (mild) motoi
paialysis, e.g., facial palsy.
Eyes
In ophthalmic zostei, nasociliaiy involvement
of V-1 (ophthalmic) bianch of the tiigeminal
neive occuis in about one-thiid of cases and
is heialded by vesicles on the side and tip of
the nose.
Complications include uveitis, keiatitis, con-
junctivitis, ietinitis, optic neuiitis, glaucoma,
IMAC 2T-2 varce||a aod herpes toster . |u||u p||r+|, \/\ |u|ec||ou (.+||ce||+ o| c||c|eupo\), .||u
|u|ec| euo|, +u||+. 8. \/\ pe||| |u + |+|eu| p|+e W||||u +u||+ |o| ||e |||e o| ||e |ud|.|du+|. C. w||| d|r|u
||ed |rruue |uuc||ou, \/\ |e+c||.+|e W||||u euo|, +u||+, deceud euo|, ue|.e, +ud |ep||c+|e |u ||u.
vrus oIectoo: herpes toster oo
thorax aod arm A o0,e+|o|d
r+|e |e|u ||e+|ed W||| p|edu|oue |o|
ec/er+ |+ p+|u|u| |e|ou |o| ! d+,.
|e|r+|or+| |ouped +ud cou||ueu|
.e|c|e ou ||e (} |||| |+c| +ud +|r
+ud (8} |||| pec|o|+| +|e+.
8
pioptosis, cicatiicial lid ietiaction, and
extiaoculai muscle palsies.
Acute ietinal neciosis (iapidly piogiessive
heipetic ietinal neciosis) is moie common in
the immunocompiomised host
De|uyed Cvntru|uteru| HemIpuresIs
Occuis weeks to months (mean, 7 weeks)
aftei an episode of HZ involving the fiist divi-
sion of the tiigeminal neive (V-1).
Typical piesentation is headache and hemi-
plegia occuiiing in a patient with iecent his-
toiy of HZ ophthalmicus.
Aiteiiogiam shows inflammation, naiiow-
ing, and thiombosis of pioximal bianches of
anteiioi oi middle ceiebial aiteiy.
Pathogenesis: diiect VZV invasion of cei-
ebial aiteiies by extension along intiacianial
bianches of V-1, iesulting in inflammation of
inteinal caiotid aiteiy oi one of its bianches
on the side ipsilateial to iash.
Frodroma| Stae[Ioca|ted Fao Can mimic
migiaine, caidiac oi pleuial disease, an acute
abdomen, oi veitebial disease.
0ermatoma| ruptoo Zosteiifoim HSV in-
fection, phytoalleigic (poison ivy, poison oak)
contact deimatitis, eiysipelas, bullous impetigo,
neciotizing fasciitis.
See Laboiatoiy Exminations," page 832.
FICk 2T-42 varce||a toster vrus oIectoo: herpes toster o v1 dstrbutoo A 53,e+|o|d r+|e
W||| po||+| |e|u ||e+|ed W||| re||o||e\+|e +ud e|+ue|cep| |+d oue| o| p+|u|u| |+| ou |||| |o|e|e+d |o| oue
d+, W||| ||u|||e ,rp|or. E|,||er+|ou eder+|ou p|+que ou ||e |o|e|e+d +ud |+|e||+. \/\ W+ de|ec|ed |,
||A. \e||o||e\+|e +ud e|+ue|cep| We|e |e|d +ud .+|+c,c|o.|| |.eu. ne d|d We|| W|||ou| corp||c+||ou. E|+ue|cep|
W+ |e|+||ed |u o Wee|.
8
FICk 2T-43 varce||a toster vrus

oIectoo: herpes toster oI the tyomat-
cotempora| oerve (braoch oI the v2 max|-
|ary oerve) A +9,e+|o|d r+|e W||| + |||o|,
o| |e|| p+||e|+| ||o||+|or+ W+ |e|u ||e+|ed
W||| |+d|+||ou ||e|+p, +ud e\pe||euced d|cor
|o|| +| ||e |+d|+||ou po||+| ||e. ne |+d |eeu
||e+|ed |o| |+c|e||+| |u|ec||ou W||| o|+| +u||||o||c
W|||ou| |rp|o.ereu|. . C|u|e|ed c|u|ed e|o
|ou +|e eeu ou ||e |+|e|+| c+|p, d|er|
u+|ed .e|c|e We|e c+||e|ed ou ||e e\||er|||e
+ud ||uu|. 8. \e|c|e ou ||e uppe| +|r. ne W+
||e+|ed W||| o|+| |+rc|c|o.||, |e|ou |eo|.ed
W|||ou| corp||c+||ou.
IMAC 2T-3 0ermatomes I|e cu|+ueou ||e|d o| pe||p|e|+| ue|.e.
L5
S1
L4
S1
L5
L5
L4
C4 C4
C3
C2
T2
3
4
5
6
7
8
9
10
11
12
L1
L2 L2
L3 L3
L4 L4
L5 L5
L5 L5
S1 S1
C
8
C
7
C6
T1
T2
C5 C5
T2
T1
C6
C
8
C
7
C4 C4
C5 C5
C6 C6
T2 T2
C
8
C
8
C
6
C
7
C
6
C
7
T1 T1
L2 L2
S1 S1
L4 L4
L
5
L
5
S2 S2
L3
T2
3
4
5
6
7
8
9
10
11
12
L1
L2
L3
C3
C2
S5
S5
S3 S4
Coc.
|ectrocardoram In piodiomal stage with
individuals with chest pain, iule out ischemic
heait disease.
Imao In piodiomal stage, iule out oiganic,
pleuial, pulmonaiy, oi abdominal disease.
0IACN0SIS
Frodroma| Stae Suspect HZ in oldei oi
immunocompiomised individual with unilat-
eial pain.
Actve vescu|atoo Clinical findings usually
adequate; may be confiimed by Tzanck test and
possible DFA oi viial cultuie to iule out HSV
infection.
FhN By histoiy and clinical findings.
C0kS AN0 Fk0CN0SIS
In immunocompetent host, iash usually iesolves
in 2 to 3 weeks. Complications can be:
Mucocutaneous
Hemoiihage, gangiene
Cutaneous dissemination
Supeiinfection of skin lesions
Systemic
Neuiologic: meningoencephalitis, cei-
ebial vasculai syndiomes, cianial neive
syndiomes tiigeminal (ophthalmic) bianch
(HZ ophthalmicus), facial and auditoiy
neives (Ramsay Hunt syndiome)], peiiph-
eial motoi weakness, tiansveise myelitis
Visceial involvement: (pneumonitis, hepa-
titis, peiicaiditis/myocaiditis, pancieatitis,
esophagitis, enteiocolitis, cystitis, synovitis).
Dissemination geneially occuis 6-10 days
aftei onset of localized lesions and is most
often limited to cutaneous involvement.
Visceial dissemination can occui, involving
CNS, lung, heait, and GI tiact.
Dissemination of zostei- 20 lesions outside
the affected oi adjacent deimatomes-occuis
in up to 10% of patients, usually in immuno-
suppiessed patients. Motoi paialysis occuis
in 5% of patients, especially when the viius
involves the cianial neives.
The iisk of PHN is 40% in patients 60 yeais.
In one laige follow-up study, PHN was piesent
1 month aftei onset of the iash in 60%; by
3 months, some pain in 24%; and by 6 months,
13% still had pain. The highest incidence of
PHN is in ophthalmic zostei.
Pain with HZ is associated with neuial in-
flammation, neive infection duiing the acute
ieactivation, and neuial inflammation and scai-
iing with PHN.
Foi VZV infection in immunocompiomised
hosts, see page 846.
FICk 2T-44 varce||a toster vrus oIectoo: thoracc toster A !9,e+|o|d |er+|e W||| n|\/A||' |+
|+d p+|u|u| c|e| |e|ou |o| ! Wee|. C|u|ed +ud |eep|||e||+||/ed de|r+|or+| e|o|ou ou ||e |e|| |+c|.
MANACMNI
Freveotoo
|rruu|/+||ou |rruu|/+||ou W||| \/\ .+cc|ue r+, |oo| |uro|+| +ud ce||red|+|ed |rruu||,
+ud dec|e+e ||e |uc|deuce o| /o|e| |u popu|+||ou W||| dec||u|u \/\pec|||c
|rruu||,.
Coa|s oI maoaemeot ke||e.e cou|||u||ou+| ,rp|or, r|u|r|/e p+|u, |educe .||+| |edd|u, p|e.eu|
ecoud+|, |+c|e||+| |u|ec||ou, peed c|u||u o| |e|ou +ud |e+||u, e+e
p|,|c+|, p,c|o|o|c+|, ero||ou+| d|cor|o||, p|e.eu| .||+| d|er|u+||ou o|
o||e| corp||c+||ou, p|e.eu| o| r|u|r|/e |n|.
Aotvra| therapy |u |ud|.|du+| +| ||| ||| |o| |e+c||.+||ou o| \/\ |u|ec||ou, o|+| +c,c|o.|| c+u |educe
||e |uc|deuce o| n/. |u p|od|or+| |+e. |e|u +u||.||+| +eu| || d|+uo| |
cou|de|ed |||e|,, +u+|e|c. w||| +c||.e .e|cu|+||ou. +u||.||+| ||e|+p, |euu
12 | +cce|e|+|e |e+||u o| ||u |e|ou, dec|e+e ||e du|+||ou o| +cu|e p+|u,
+ud r+, dec|e+e ||e ||equeuc, o| |n| W|eu |.eu |u +dequ+|e do+e.
Ac,c|o.|| 300 r |0 |ou| ||re d+||, |o| 1-0 d+,. I|e 50 .||+| |u|||||o|, couceu||+||ou
o| +c,c|o.|| | |||ee |o |\ ||re |||e| |o| \/\ ||+u |o| n'\ |u .|||o, +ud d|u
doe ru| |e |uc|e+ed +pp|op||+|e|,. I|e ||o+.+||+|||||, o| +c,c|o.|| | ou|,
5-!0 o| ||e o|+||, +dr|u||e|ed doe. |o| op|||+|r|c /o|e| +ud n/ |u ||e
|rruuocorp|or|ed |o|, +c,c|o.|| |ou|d |e |.eu |u||+.euou|,. Ac,c|o.||
|+|eu |e+||u +ud |eeu c:|- p+|u || |.eu W||||u +3 | o| ||e oue| o| ||e
|+|.
\+|+c,c|o.|| 000 r |0 |||ee ||re d+||, |o| 1 d+,, 10-30 ||o+.+||+||e.
|+rc|c|o.|| 500 r |0 |||ee ||re d+||, |o| 1 d+,, 11 ||o+.+||+||e. keduce doe |u
|ud|.|du+| W||| d|r|u||ed |eu+| |uuc||ou.
1:,:|.-|c| ll |oc+|ue|
|--! |\ +c,c|o.|| +ud |ecor||u+u| |u|e||e|ou 2+ |o p|e.eu| d|er|u+||ou o| n/ |
c|-| |ud|c+|ed.
Supportve therapy Ior acute hI
Cou|||u||ou+| ,rp|or Bed |e|, uou|e|o|d+| +u|||u||+rr+|o|, d|u.
'ed+||ou |+|u o||eu |u|e||e|e W||| |eep. '|eep dep||.+||ou +ud p+|u corrou|, |eu|| |u
dep|e|ou. |o\ep|u, 0-00 r +| |ed ||re, | +u e||ec||.e +eu|.
0|+| |ucoco|||co|d ||edu|oue |.eu e+||, |u ||e cou|e o| n/ |e||e.e cou|||u||ou+| ,rp|or |u|
|+ uo| |eeu p|o.eu |o |educe |n|.
||e|u App||c+||ou o| ro|| d|e|u (W+|e|, +||ue, Bu|oW o|u||ou) |o ||e |u.o|.ed
de|r+|ore | oo|||u +ud +||e.|+|e p+|u.
|+|u r+u+ereu| E+||, cou||o| o| p+|u W||| u+|co||c +u+|e|c | |ud|c+|ed, |+||u|e |o r+u+e p+|u
c+u |eu|| |u |+||u|e |o |eep, |+||ue, +ud dep|e|ou. Be| |o |e|u W||| ro|e
po|eu| +u+|e|c +ud ||eu |educe po|euc, + p+|u |eeu.
Chrooc staes (FhN)
|+|u r+u+ereu| |+|u | ||+| o| |e||e\ ,rp+||e||c d,||op|,.
'e.e|e p|od|or+| p+|u o| e.e|e p+|u ou ||e |||| d+, o| |+| | p|ed|c||.e o|
e.e|e |n|.
C+|+peu||u. !00 r |||ee ||re d+||,. ||e+|+||u.
I||c,c||c +u||dep|e+u| uc| + do\ep|u, 0-00 r |0 +| |ed ||re.
C+p+|c|u c|e+r e.e|, + |.
Iop|c+| +ue||e||c uc| + E\|A o| 5 ||doc+|ue p+|c| |o| +||od,u|+.
|e|.e ||oc| |o +|e+ o| +||od,u|+.
Au+|e|c.
*|u Eu|ope, o|+| +c,c|o.|| | ou|, |+|e|, ued |ec+ue o| |e+|e| ||o+.+||+|||||, o| .+|+c,c|o.|| +ud |+rc|c|o.||.
FICk 2T-45 varce||a toster vrus oIectoo: atrophc scar at v1 toster ste A 90,e+|o|d |er+|e
W||| + |||o|, o| |e|pe /o|e| + ,e+| p|e.|ou|,. n,pop|reu|ed de|r+|or+| (\) c+| | eeu ou ||e ||||
|o|e|e+d +| ||e ||e o| p||o| /o|e|.
vrus oIectoo: ophtha|mc her-
pes toster C|u|ed u|ce|+||ou
+ud .e|c|e ou ||e |||| |o|e|e+d
+ud pe||o||||+| +|e+ |u ||e op|||+|
r|c ||+uc| o| ||e |||er|u+| ue|.e,
r+||ed |+c|+| eder+ | +|o p|e
eu|. \e|c|e ou ||e ||p o| ||e uoe
|ud|c+|e u+oc|||+|, |u.o|.ereu|.
nu|c||uou |u|e. |u.o|.ereu| o| ||e
u+oc|||+|, ue|.e ue| ||+| e,e
|u.o|.ereu| r+, occu|.
FI0MI0I0C
Iocdeoce The population of immunocom-
piomised individuals is incieasing, and most
cases of iecuiient HZ occui in immunocom-
piomised individuals.
ksk Factors
Immunosuppiession, especially fiom lym-
phopiolifeiative disoideis, and cancei che-
motheiapy.
Heipes zostei: Often the fiist sign of HIV
infection, pieceding oial candidiasis and oial
haiiy leukoplakia by 1 yeai.
Risk of dissemination of vaiicella oi HZ to
visceia:
Vaiicella to visceia:
Childien undeigoing cancei chemothei-
apy
Solid oigan and bone maiiow tiansplant
iecipients
HIV/AIDS infection
Ceitain cell-mediated immunodeficiency
disoideis of childhood.
Heipes zostei to visceia:
Hodgkin disease: 13-15% iisk of HZ
Non-Hodgkin lymphoma: 7-9% iisk foi
HZ
Solid tumois: 1-3% iisk foi HZ
Sko Symptoms
Symptoms of vaiicella and zostei.
Chionic cutaneous VZV infections following
hematogenous dissemination aie often asso-
ciated with significant lesional pain iequiiing
naicotic analgesia foi pain management.
Coosttutooa| Symptoms Visceial dissemina-
tion usually accompanied by fevei.
|u |rruuocorp|or|ed |ud|.|du+|, \/\ |u|ec
||ou c+u |e ro|e e.e|e |u
|||r+|, |u|ec||ou (.+||ce||+)
ke+c||.+|ed |u|ec||ou (n/)
\+||ce||+ cu|+ueou +ud .|ce|+| |u.o|.ereu| c+u
|e ro|e e.e|e.
ne|pe /o|e| r+,
|u.o|.e e.e|+| cou||uou de|r+|ore
n+.e ro|e e\|eu|.e cu|+ueou uec|o|
n+.e W|de |er+|oeuou d|er|u+||ou |o
\ucocu|+ueou ||uc|u|e
\|ce|+ (|uu, ||.e|, ||+|u)
0||eu |e +oc|+|ed W||| ||| ro|||d||, +ud
ro||+|||, |+|e.
vAkICIIA I0SIk vIkS INFCII0NS IN Ih IMMN0C0MFk0MIS0 h0SI
VurIce||u und Cutunevus DIssemInutIvn v]
ReuctIvuted VZV 1n]ectIvn (See Vaiicella,"
page 833.) Reactivated VZV without HZ with
cutaneous dissemination cannot be distin-
guished clinically fiom vaiicella (Fig. 27-47).
Herpes Zvster In HIV/AIDS disease and
leukemia, involvement of seveial contiguous
deimatomes is common (Fig. 27-48). (See also
Section 31, Mucocutaneous Manifestations of
Human Immunodeficiency Viius Disease)
Herpes Zvster wIth Cutunevus DIssemInutIvn
A vaiiable numbei of vesicles oi bullae aie
seen at any mucocutaneous site, which evolve
into ciusted eiosions.
Lesions aie disseminated and iange fiom a
few to hundieds.
The condition thus appeais clinically as zostei
plus vaiicella.
Herpes Zvster wIth PersIstent Dermutvmu|
1n]ectIvn
Papules and nodules, which can become
hypeikeiatotic oi veiiucous, peisisting in
a deimatomal pattein (single oi multiple
contiguous) aftei an outbieak of zostei
(Fig. 27-49).
Chionic ulceis can peisist foi months.
ChrvnIc Cutunevus VZV 1n]ectIvn A]ter
Hemutvgenvus DIssemInutIvn
Lesions on the palms oi soles may piesent
initially as bullae.
Continual appeaiance of vesicles/bullae in a
deimatomal oi geneialized distiibution.
Dissemination can occui without deimatomal
HZ.
Chionic lesions piesent as nodules, ulceis,
ciusted nodules/ulceis (ecthymatous). Postin-
flammatoiy hypei- oi hypopigmentation.
Systemc Fodos Eyes
In HIV/AIDS disease, ietinal VZV infection
(acute ietinal neciosis) can occui in the
absence of appaient conjunctival oi cutane-
ous involvement with subsequent loss of vi-
sion.
Bilateial involvement in one-thiid of cases
with subsequent loss of vision.
VZV optic neuiitis is iaie.
CNS In HIV/AIDS disease, VZV is the
etiologic agent of up to 2% of CNS dis-
ease (encephalitis, polyneuiitis, myelitis, vascu-
litis).
Frmary varce||a wth vscera| 0ssemoatoo
Pneumonia must be distinguished fiom Pneu-
motyss tarn pneumonia associated with
vaiicella.
herpes Ioster wth Cutaoeous 0ssemoatoo
Zosteiifoim HSV infection with dissemination.
herpes Ioster wth vscera| aod Cutaoeous
0ssemoatoo Zosteiifoim HSV infection
with dissemination. Pneumonia must be distin-
guished fiom P. tarn pneumonia associated
with vaiicella.
herpes Ioster wth Ferssteot 0ermatoma|
IoIectoo Chionic zosteiifoim HSV infection.
Hypeitiophic scais oi keloids.
Chrooc Cutaoeous vIv IoIectoo AIter hema-
toeoous 0ssemoatoo Ecthyma, ecthyma
gangienosum, disseminated mycobacteiial
infection, deep fungal infection, syphilis.
See Vaiicella Zostei Viius Infection," page 831.
Aotvra| Seostvtes When isolated, VZV
fiom cultuied lesion can be tested foi sensitiv-
ity to acyclovii and othei antiviial agents.
supeiinfection, most commonly caused by S.
aureus (MSSA oi MRSA) oi gioup A stiepto-
coccus (GAS).
FICk 2T-4T varce||a toster vrus oIectoo: dssemoated cutaoeous, o ao mmuoocompromsed
pateot nuud|ed o| .e|c|e +ud pu|u|e ou e|,||er+|ou |+e o| ||e ||uu| o| + p+||eu| W||| |,rp|or+.
|o|e ||e +|euce o| |oup|u o| |e|ou eeu |u |e|pe |rp|e\ o| |e|pe /o|e|. I|e e|up||ou | |ud|||uu||+||e
||or .+||ce||+ +ud ru| |e d|||e|eu||+|ed ||or d|er|u+|ed n'\ |u|ec||ou.
Chemstres Abnoimalities of livei function
tests with VZV hepatitis.
C0kS AN0 Fk0CN0SIS
Appioximately 2-35% of childien with vaii-
cella who aie undeigoing cancei chemothei-
apy expeiience visceial dissemination.
The associated moitality iate is 7-30%.
Dissemination is moie common in those
with a peiipheial blood lymphocyte count of
500/L.
Ch|dreo wth varce||a
Visceial involvement most commonly affects
lungs; less often, livei and biain.
Vaiicella pneumonia occuis 3-7 days aftei
onset of skin lesions; can piogiess iapidly
ovei a few days oi iemain indolent with
giadual impiovement ovei 2-4 weeks.
Neuiologic complications piesent 4-8 days
aftei onset of iash; associated with pooi piog-
nosis.
Adu|ts wth hI
HIV/AIDS
Recuiient episodes occui in same oi diffei-
ent deimatome(s)
Hodgkin disease
Between 15 and 30% of patients expeii-
ence significant dissemination (most often
cutaneous).
Moitality iates foi disseminated zostei much
lowei than foi childien with disseminated
vaiicella.
PHN does not appeai to be moie common in
immunocompiomised individuals than in the
geneial population.
MANACMNI
Freveotoo
|rruu|/+||ou \/\ |rruu|/+||ou | +.+||+||e +ud | 30 e||ec||.e |u p|e.eu||u ,rp|or+||c
p||r+|, \/\ |u|ec||ou. A|ou| 5 o| ueW|, |rruu|/ed c|||d|eu de.e|op
|+|. I|oe +| ||| ||| |o| .+||ce||+ W|o |ou|d |e |rruu|/ed |uc|ude
uo|r+| +du||, c|||d|eu W||| |eu|er|+, ueou+|e, +ud |rruuocorp|or|ed
|ud|.|du+| (|rruuoupp|e|.e ||e+|reu|, n|\ |u|ec||ou, c+uce|).
Aotvra| aeots Ior vIv- Ac,c|o.|| +00 r |0 |W|ce d+||,, ||or ||e d+, o| coud|||ou|u, |uduc||ou, o|
aod[or hSv-seropostve ||+up|+u|+||ou |o| +-o Wee|, upp|ee |o|| n'\ +ud \/\ |e+c||.+||ou.
odvdua|s uoderoo 0|+| .+|+c,c|o.|| +ud |+rc|c|o.|| +|e e||ec||.e.
*
8MI
Ac,c|o.||
Io odvdua|s wth m|d to n||doe o|+| +c,c|o.||, 300 r ||.e ||re d+||, |o| 1 d+,, |+|eu |e+||u +ud
moderate mmuoocompromse |eeu c:|- p+|u || |.eu W||||u +3 | o| ||e oue| o| ||e |+|. |+|e
cou||o||ed |ud|e |u p+||eu| o0 ,e+| |+.e uo|, |oWe.e|, derou||+|ed
+u, e||ec| ou ||e |uc|deuce +ud e.e|||, o| :|: po||e|pe||c ueu|+||+
o| |||doe o|+| +c,c|o.||. A |eceu| p|e||r|u+|, |ud, o| o|de| p+||eu| (+e
o0) derou||+|ed + |educed ||equeuc, o| pe|||eu| p+|u W|eu |\ +c,c|o.||,
0 r/| q3| |o| 5 d+,, W+ |.eu W||||u + d+, o| ||e oue| o| ||e p+|u
o| W||||u +3 | +||e| ||e oue| o| ||e |+|. 0|+| .+|+c,c|o.||, 000 r ||d,
|+rc|c|o.||, 500 r ||d, |o|| |o| 1 d+,.
Io odvdua|s wth |\ +c,c|o.|| o| |ecor||u+u| |u|e||e|ou 2+ |o p|e.eu| d|er|u+||ou o| n/ |
advaoced mmuoocompromse |ud|c+|ed.
*
B\I, |oue r+||oW ||+up|+u|+||ou.
*
|u Eu|ope, o|+| +c,c|o.|| | |+|e|, ued |ec+ue o| |e||e| ||o+.+||+|||||, o| .+|+c,c|o.|| +ud |+rc|c|o.||.
FICk 2T-48 varce||a toster vrus oIectoo: oecrotto herpes toster Cou||ueu|, c|u|ed u|ce|
+||ou ou +u |u||+rr+|o|, |+e |u e.e|+| cou||uou de|r+|ore |u +u e|de||, r+|e W||| |eu|er|+.
FICk 2T-49 varce||a toster vrus oIectoo: chrooc herpes toster o hIv dsease ||c|e|e +ud
cou||ueu| |,pe||e|+|o||c p|+que |u e.e|+| cou||uou de|r+|ore pe|||eu| |o| 2 ,e+| |u + r+|e W||| +d.+uced
uu||e+|ed n|\ d|e+e. I|e |e|ou We|e r|u|r+||, ,rp|or+||c.
Ae oI 0oset 6-24 months.
to|oy
HHV-6 (vaiiants -6A and -6B) and HHV-7
shaie genetic, biologic, and immunologic
featuies and aie T cell tiopic.
At biith, most childien have passively tians-
feiied anti-HHV-6 and-7 IgG.
Piimaiy infection is acquiied via oiophaiyn-
geal secietions.
HHV-6 antibodies ieach a nadii at 4-7 months
and inciease thioughout infancy.
By 12 months, two-thiids of childien become
infected, with peak antibody levels ieached at
2-3 yeais of age.
Similaily, HHV-7 antibodies ieach nadii at
6 months, with level peaking at 3-4 yeais of
age. Latent infection may peisist foi the life-
time of the individual.
FAIh0CNSIS
Pathogenesis of ES iash is not known.
Frodrome
High fevei ianging fiom 38.9-40.6C.
Remains consistently high, with moining
iemission, until the fouith day, when it falls
piecipitously to noimal, coincident with the
appeaiance of iash.
Infant iemaikably well despite high fevei.
Asymptomatic piimaiy HHV-6 and HHV-7
infection is common.
Symptoms Usually absent.
Sko Iesoos
Small blanchable pink macules and papules,
1-5 mm in diametei (Fig. 27-50).
E||o|o|c +eu|. p||r+|, |ur+u |e|pe.||uo
(nn\o) +ud nn\1 |u|ec||ou
C|+|+c|e||/ed |,
n|| |e.e| |u + |e+|||, |u|+u| (9-2 rou|| o|d)
|e|e|.eceuce |u ! d+, |o||oWed |,
'uddeu +ppe+|+uce o| e\+u||er
Corp||c+||ou. |e||||e e|/u|e
',,. koeo|+ |u|+u|ur.
hhv-6 AN0 -T INFCII0NS: XANIhMA S8IIM (S)
Lesions may iemain disciete oi become con-
fluent.
Distiibution: tiunk and neck.
Ceoera| Fodos Absent in piesence of high
fevei. Febiile seizuies aie common.
Morb||Iorm xaothem See Infectious Exan-
thems," page 795.
Sero|oy Demonstiation of IgM anti-HHV-6 oi
anti-HHV-7 antibodies oi IgG seioconveision.
0ther Viial cultuie and isolation fiom peiiph-
eial blood mononucleai cells. Demonstiation
of HHV-6 oi HHV-7 DNA by PCR.
0IACN0SIS
C0kS AN0 Fk0CN0SIS
Couise self-limited with iaie sequelae.
In some cases, high fevei may be associated
with seizuies.
Intussusception associated with hypeiplasia
of intestinal lymphoid tissue and hepatitis
iepoited.
As with othei HHV infections, HHV-6 and
HHV-7 peisist thioughout the life of the pa-
tient; Clinical manifestations associated with
HHV-6 and HHV-7 ieactivation have not been
identified beyond doubt, but pityiiasis iosea is
discussed.
An infant with HHV-6 ES may expeiience
a second clinical syndiome, HHV-7 ES, and
vice veisa.
MANACMNI
Symptomatic.
FICk 2T-50 xaothema subtum \u|||p|e, ||+uc|+||e r+cu|e +ud p+pu|e ou ||e |+c| o| + |e||||e
c|||d, W||c| +ppe+|ed + ||e |erpe|+|u|e |e||. (Cou||e, o| K+|eu w|, \|)
852
S E C I | 0 N 2 8
AkIhk0F00 8IIS,
SIINCS, AN0 CIAN0S
INFCII0NS
A||||opod |||e +ud ||u.
C+ue + W|de pec||ur o| |e+c||ou
I|+ur|| |oc+| +ud ,|er|c |u|ec||ou
'upe|||c|+| |u|e|+||ou.
Ep|de|r+|. |ed|cu|o|, c+||e, |uu|+|
|e|r+|. |+|.+ r||+u, r,|+|
Ie||e|||+| +||||opod ||+| |||e/||u |ur+u.
+|+c|u|d, ceu||pede, r||||pede, |uec|.
Cu|+ueou |e+c||ou |o +||||opod |||e (CkAB)
+|e |u||+rr+|o|, +ud/o| +||e||c |e+c||ou.
C|+|+c|e||/ed |, +u |u|eue|, p|u||||c e|up||ou +|
||e |||e ||e |rred|+|e|, |o r|uu|e |o |ou| |o
d+, +||e| ||e |||e, pe||||u |o| d+, |o Wee|,
r+u||e|ed |, o|||+|, o| |ouped.
u|||c+||+| p+pu|e
|+pu|o.e|c|e
Bu||+e
|+||eu| +|e o||eu uu+W+|e o| |+.|u |eeu ||||eu.
|u ore c+e, ,|er|c ,rp|or r+, occu|,
|+u|u ||or r||d |o e.e|e, W||| de+|| occu|||u
||or +u+p|,|+c||c |oc|.
A||||opod +|e .ec|o| o| r+u, ,|er|c |u|ec||ou.
CIAN0S kACII0NS I0 AkIhk0F00 8IIS
FI0MI0I0 C
Seasoo Summei in tempeiate climates.
to|oy 5 of 9 classes of aithiopods cause
local and systemic ieactions associated with
theii bites: Aiachnida, Chilopoda, Diplopoda,
Ciustacea, Insecta.
Arthropods Ihat 8te, Sto, or IoIest
Aiachnida (foui paiis of legs): mites, ticks,
spideis, scoipions
Acaiina
Mites: Sartoes sta|e causes scabies;
DemoJex [o||tu|orum, human haii follicle
mite; many otheis including food, fowl,
giain, stiaw, haivest, animal, and house
dust mites.
Ticks
Aianeae: spideis
Scoipionida
Chilopoda and Diplopoda: centipedes, mil-
lipedes
Insecta (thiee paiis of legs)
Anopluia: lice ( P||rus and PeJtu|us )
Coleopteia: beetles
Dipteia: mosquitoes, black flies, midges
(punkies, no seeums, sand flies), Taban-
dae (hoiseflies, deeiflies, clegs, bieeze flies,
gieenheads, mango flies); botflies, Ca|-
|roga amertana, Dermao|a |omns,
phlebotomid sand flies, tsetse flies
Hemipteia: bedbugs, kissing bugs
Hymenopteia: ants, bees, wasps, hoinets
Lepidopteia: cateipillais, butteiflies, moths
Siphonapteia: fleas, chigoe oi sand flea
Arthropod-8oroe IoIectoos
Lyme boiieliosis, tulaiemia, bubonic plague.
Sciub typhus, endemic (muiine) typhus,
spotted fevei gioups, Q fevei
Human gianulocytic anaplasmosis,
Tick-boine meningoencephalitis
Leishmaniasis, tiypanosomiasis (sleeping
sickness, Chagas disease).
Malaiia, babesiosis.
Filaiiasis, onchoceiciasis (iivei blindness),
loiasis
Ceoraphc 0strbutoo Woildwide.
SCII0N 28 AkInk0|0| B|IE', 'I||C', A|| CuIA|E0u' |||ECI|0|' 853

FAIh0CNSIS
Mtes
Pioduce piuiitus and/oi alleigic ieactions
thiough salivaiy pioteins deposited duiing
feeding.
Haivest mites (chiggeis) may piesent as
intense piuiitus on the ankles, legs, belt line;
mites usually fall off aftei feeding oi may be
sciatched off.
In nonsensitized individuals, 1- to 2-mm piu-
iitic papules aie seen.
In sensitized individuals, CRAB may be
papulai uiticaiia, vesiculation, oi gianulo-
matous ieaction with fevei and lymphaden-
opathy.
Icks (Acaroa) Reactions include foieign
body ieactions, ieactions to salivaiy secietions,
ieactions to injected toxins, and hypeisensitiv-
ity ieactions. Tt| ara|yss is caused by a toxin
secieted in the saliva of the tick.
Spders (Araoeae)
Loxoste|es ret|usa : Biown iecluse spidei bite
causes ieactions ianging fiom mild uiticaiia
to full-thickness neciosis ( |oxoste|sm ).
LaroJetus : Widow" spideis inject a venom
that contains a neuiotoxin ( -latiotoxin)
pioducing ieactions at the bite site as well as
vaiying degiees of systemic toxicity.
Tegenara : Hobo spidei causes neciotic aiach-
nidism in Pacific Noithwest of United States.
Taranu|a : Mild inflammatoiy iesponse to
bite and fiom haiis shed fiom legs.
Scorpoo (Scorpooes) Reside in tiopical and
aiid iegions. Venom also contains a neuiotox-
in that can cause seveie local and systemic ie-
actions.
8|ster 8eet|es Contain the chemical canthaii-
din, which pioduces a blistei when the beetle is
ciushed on the skin.
8|ack F|es Bites pioduce local ieactions as
well as black fly fevei, chaiacteiized by fevei,
headache, nausea, geneialized lymphadenitis.
Noobto F|es Flies commonly feed on open
wounds, exudates, and skin ulceis and may
deposit eggs at these sites, iesulting in wounJ
myass . Fly laivae can buiiow into injuied oi
noimal skin, invading thiough the epideimis
into the deimis, iesulting in [uruntu|ar myass .
In some cases, laivae move about the subcutis
(mgraory myass ), mimicking the pattein of
cutaneous laiva migians.
8edbus Noctuinal feedings pioduce a lineai
oi giouped aiiangement of papulai uiticaiia
(bieakfast, lunch, and dinnei).
hymeooptera Bees, hoinets, wasp bites can
pioduce painful stings and anaphylaxis in the
sensitized individual.
Caterp||ars aod Moths Haiis can pioduce
local iiiitant and alleigic ieactions.
F|eas (Cat, 0o, 8rd) Bites tend to cause moie
local ieactions than human flea bites.
Choe or Saod F|eas ( Tunga enerans )
Recently impiegnated female flea penetiates
skin of a human host, buiiows into epideimis
to deimal-epideimal junction, wheie she feeds
on blood diawn fiom host vessels in supeificial
deimis ( ungass ). Matuie eggs (150-200) aie
extiuded singly fiom a teiminal abdominal
oiifice duiing a peiiod of 7-10 days. The female
dies shoitly aftei egg extiusion, and the infested
tissues collapse aiound it.
Iocubatoo Ferod CRAB appeais minutes to
days aftei the bite.
0uratoo oI Iesoos Days, weeks, months.
Sko Symptoms Piuiitus, pain at bite site. Sys-
temic symptoms with systemic ieaction.
Mucocutaoeous Fodos
Erythemutvus Mucu|es Occui at bite sites and
aie usually tiansient.
Pupu|ur UrtIcurIu
Peisistent (>48 h) uiticaiial papules (Figs. 28-1
to 28-5), often suimounted by a vesicle, usu-
ally <1 cm.
Excoiiations and excoiiated uiticaiial papules,
vesicles.
Ciusted painful lesions, usually puiulent, may
iepiesent impetigo, ecthyma, oi cutaneous
diphtheiia.
Excoiiated oi secondaiily infected lesions
may heal with hypei- oi hypopigmentation
and/oi iaised oi depiessed scais, especially in
moie daikly pigmented individuals.
Bu||vus LesIvns
Tense bullae with cleai fluid on a slightly
inflamed base.
Excoiiation iesults in laige eiosion.
TIc| BItes
Attach and feed painlessly.
Secietions can pioduce local ieactions (Fig.
28-6), febiile illness, paialysis.
FICk 28-1 Fapu|ar urtcara: osect bte A
23,e+|o|d |er+|e W||| p|u||||c |e|ou ou ||e c|e|.
'o|||+|, |ed eder+|ou p+pu|e W||| e+||, e|o|ou ou
||e |||| pec|o|+| +|e+. \u|||p|e |e|ou We|e p|eeu|
+| ||e ||e.
FICk 28-2 Fapu|ar urtcara: mu|tp|e osect
btes A 53,e+|o|d r+|e W||| n|\/A||' W||| p|u
||||c |e|ou ou |e, +ppe+||u du||u + .+c+||ou |o
|ue||o k|co. \u|||p|e |ed p+pu|e ou ||e |oWe| |e.
|u n|\/A||', |+|e |e+c||ou |o |uec| |||e c+u occu|.
|u ||| c+e, ||e |uec| W+ uo| |uoWu. |+pu|e +ud
uodu|e pe|||ed |o| rou||.
FICk 28-3 Fapu|ar urtcara: bedbu btes A 11,e+|o|d |er+|e W||| p|u||||c |e|ou ou ||e uec|.
E\co||+|ed p+pu|e ou ||e po|e||o| uec|. '|r||+| |e|ou We|e +|o p|eeu| ou ||e |e. Bed|u (C-
|-:||c) |+|e |ur+u dor+|u, |e|d|u |u c|e.|ce o| ||oo| +ud W+||, |u |edd|u, +ud |u |u|u||u|e. I|e, uu
+||, |eed ou|, ouce + Wee| +ud |e o||eu |u co|d We+||e|. Bed|u c+u ||+.e| |ou d||+uce |u e+|c| o| + |ur+u
|o| +ud c+u u|.|.e |o| o |o 2 rou|| W|||ou| |eed|u. B||e |e+c||ou occu| ou e\poed ||e uc| + ||e |+ce,
uec|, +|r, +ud |+ud, W||| |Wo |o |||ee |e|ou |u + |oW ('||e+||+|, |uuc|, d|uue|). |u p|e.|ou|, uue\poed
|ud|.|du+|, |||e ||e +ppe+| + e|,||er+|ou, p|u||||c r+cu|e. |u eu|||/ed |ud|.|du+|, |u|eue|, p|u||||c p+pu|e,
p+pu|+| u|||c+||+, o| .e|c|e/|u||+e r+, +||e +| ||e |||e ||e. C|+ue ecoud+|, |o c|+|c||u |uc|ude e\co||+
||ou, ec/er+|ou de|r+||||, +ud ecoud+|, |u|ec||ou.
FICk 28-4 Fapu|ar urtcara: mtes \u|||p|e p+pu|+| u|||c+||+ +| |||e ||e o| r||e |:|c |u ||e |+||
|u ||uu| +|e+. \||e |+|| o|| .ee|+||ou |u|o c|o|||u, ru|||p|e |||e +|e o||eu |o||oWed |, p+pu|+| u|||c+||+| |e+c||ou.
FICk 28-5 Iosect btes: cchymotc |esoos
A +1,e+|o|d |er+|e W||| p|u||||c |e|ou ou ||e |e.
\u|||p|e |+|e ecc|,ro||c |e|ou ou ||e |e. I|e
ceu||+| po|||ou +ppe+| u|||c+||+|, ||e pe||p|e|+| +|e+
ecc|,ro||c. Ecc|,roe We|e uo| ecoud+|, |o e\co
||+||ou |u| We|e + r+u||e|+||ou o| |,pe|eu|||.||,.
FICk 28-6 0ermaceotor varab|s Ieedo
I|e ||c| |+ |eeu +||+c|ed |o| o+ |, || | ||e .ec|o| o|
koc|, \ouu|+|u po||ed |e.e|.
Haid IxoJes tick: Ery|ema mgrans occuis as
an enlaiging plaque occuiiing at site of bite,
chaiacteiistic of Lyme boiieliosis (see Section
24); |ym|otyoma tus also occuis at tick
bite sites.
SpIders
Brown ret|use and ||at| wJow sJer bites can
iesult in mild local uiticaiial ieactions to full-
thickness skin neciosis.
Associated with a maculopapulai exanthem,
fevei, headache, malaise, aithialgia, nausea/
vomiting.
DIpteru
Mosquoes : Bites usually piesent as papulai
uiticaiia on exposed sites; ieactions can be
uiticaiial, eczematous, oi gianulomatous.
B|at| [|es : Anesthetic is injected, iesulting in
painless initial bite; may subsequently become
painful with itching, eiythema, and edema.
Black fly fevei chaiacteiized by fevei, nausea,
geneialized lymphadenitis.
MJges : Bites pioduce immediate pain with
eiythema at bite site with 2- to 3-mm papu-
lovesicles, followed by induiated nodules (up
to 1 cm) peisisting foi many months.
Ta|anJae : Bites painful with papulai uiti-
caiia; iaiely associated anaphylaxis.
Bo[|y. Laivae penetiate skin oi aie deposited
on open wounds pioducing tuaneous myass .
Laivae may be fixed oi migiate, iesembling
laiva migians. C. amertana most common in
United States.
D. |omns in tiopical iegions causes fuiun-
culai myiasis, painful lesions that iesemble
pyogenic gianuloma oi abscess; a piuiitic
papule develops at the site, slowly enlaig-
ing ovei seveial weeks into a domed nodule
(iesembles a fuiuncle) with a cential poie
(Fig. 28-7) thiough which the posteiioi end
of the laivae inteimittently piotiudes.
House [|es. Laivae deposited into any exposed
skin site (eai, nose, paianasal sinuses, mouth,
eye, anus, and vagina) oi at any wound site
(leg ulceis, ulceiated squamous and basal cell
caicinomas, hematomas, umbilical stump)
and giow into maggos, which can be seen on
suiface of wound (Fig. 28-8); although iepul-
sive foi the patient, maggots aie veiy effective
at debiiding nonviable tissue and debiis.
HemIpteru
BeJ|ug bites pioduce papulai uiticaiia that
have a chaiacteiistic lineai oi giouped aiiay
(Fig. 28-3).
ReJuJ (|ssng |ugs, assassn |ugs, tonenoseJ
|ugs) bites usually piesent as papulai uiticaiia;
FICk 28-T Furuocu|ar myass A p|u||||c p+pu|e +| ||e ||e o| depo|||ou o| + |o|||, |+|.+, |oW|, eu|+|
|u o.e| e.e|+| Wee| |u|o + dored uodu|e (|eer||e + |u|uuc|e). I|e |e|ou |+ + ceu||+| po|e |||ou| W||c|
||e po|e||o| eud o| ||e |+|.+ |u|e|r|||eu||, p|o||ude +ud ||u |ep||e. I|e |+|.+ (|ue|) c+u |e |uduced |o e\||
||e |e|ou |, occ|ud|u || W||| pe||o|+|ur o| |+|.
SCII0N 28 AkInk0|0| B|IE', 'I||C', A|| CuIA|E0u' |||ECI|0|' 85T
seveie ieactions can pioduce neciosis and ul-
ceiation iesembling spidei bites.
F|eus
Papulai piuiitic uiticaiia at exposed bite site.
Tunga enerans : Tungass : Papule oi vesicle
(6-8 mm in diametei) with cential black
dot pioduced by posteiioi pait of the flea`s
abdominal segments. As eggs matuie and ab-
domen swells, papule becomes a white, pea-
sized nodule. With intialesional hemoiihage,
it becomes black (Fig. 28-9). With seveie infes-
tation, nodules and plaques with a honey-
combed appeaiance. If lesions aie squeezed,
eggs, feces, and inteinal oigans aie extiuded
thiough poie. Ulceiation, inflammation and
FICk 28-8 Wouod myass \u|||p|e |+|.+e o| r+o| o| ||e |oue||, +|e eeu |u + c||ou|c |+|
u|ce| ou ||e +u||e. I|e |e |+d |eeu ||e+|ed W||| C+|e||+u| p+|u| +ud uuu+ |oo| |o| Wee|. w|eu ||e d|e|u
W+ |ero.ed, ||e r+o| We|e .||||e, ||e |+e o| ||e u|ce| W+ |ed +ud c|e+u, |+.|u |eeu de|||ded |, ||e
r+o|.
FICk 28-9 Iuoass A
uec|o||c, pe||uuu+| p+pu|e W||| u|
|ouud|u e|,||er+ ou ||e |+|e|+|
r+||u o| ||e ||||| |oe, ||e |+|.+ | .|u
+||/ed |, |ero.|u ||e o.e||,|u c|u|.
secondaiy infection can occui. Sites: feet,
especially undei toenails, between toes, plantai
aspect of the feet, spaiing weight-beaiing ai-
eas; in sunbatheis, any aiea of exposed skin.
Hymenvpteru
Female bee, hoinet, oi wasp fiom modified
ovipositoi (stingei appaiatus) sting pioducing
immediate buining/pain, followed by intense,
local, eiythematous ieaction with swelling
and uiticaiia. Seveie systemic ieactions occui
in individuals who aie sensitized (0.4-0.8%),
with angioedema/geneialized uiticaiia and/
oi iespiiatoiy insufficiency fiom laiyngeal
edema oi bionchospasm and/oi shock.
Fre anJ |areser ans pioduce local skin
neciosis and systemic ieactions to sting; bite
ieaction begins as an intense local inflamma-
toiy ieaction that evolves to a steiile pustule.
LepIdvpteru
Caer||ar / mo| contact can pioduce buin-
ing/itching sensation, papulai uiticaiia,
iiiitation due to histamine ielease, alleigic
contact deimatitis (Fig. 28-10), and/oi sys-
temic ieactions. Wind-boine haiis can cause
keiatoconjunctivitis.
Systemc Fodos Systemic findings may occui
associated with toxin oi alleigy to substance
injected duiing bite. Many vaiied systemic
infections can be injected duiing bite.
8te Ste keactoos (rythematous Fapu|es,
8|sters) Alleigic contact deimatitis, especially
to plants such as poison ivy oi poison oak.
Furuocu|ar Myass[Iuoass Sa|y|otottus
aureus paionychia, CanJJa paionychia, ceicai-
ial deimatitis, scabies, fiie ant bite, folliculitis.
Cutaoeous Necross Neciotizing soft tissue
infection, vasculai insufficiency, adveise cuta-
neous diug ieaction.
0ermatopatho|oy Arthrvpvd Purts Aithio-
pods such as S. sta|e oi T. enerans that bui-
iow into the epideimis; fiagments of the insect,
feces, oi eggs can be seen. Retained mouth paits
can been seen in the skin months aftei the bite.
BIte SIte ReuctIvns
In acute phase, vaiiable epideimal necio-
sis, spongiosis, paiakeiatosis with plasma
exudate; deimal inflammatoiy infiltiate ex-
tends into deep deimis in a wedge-shaped pat-
tein, suiiounding vessels with some extension
into deimal collagen. The deimal infiltiate is
mixed, composed of eosinophils, neutiophils,
lymphocytes, and histiocytes. Eosinophils aie
usually piominent; neutiophils may piedom-
inate in ieactions to fleas, mosquitoes, fiie
ants, and biown iecluse spideis. Bullae foim
secondaiy to maiked edema. Insect paits aie
iaiely seen except in scabies and in tick bites
wheie iemoval is incomplete.
In chionic phase, lesions iesult fiom ietained
aithiopod paits oi hypeisensitivity. Chionic
lesions can appeai as a seuJo|ym|oma .
1n]ectIvn ut BIte SIte The pathogen can be
demonstiated in the lesional biopsy specimen
by special stains.
Leishmania at sandfly bite site.
Borre|a |urgJor[er at IxoJes tick bite site.
8actera| Cu|ture Rule out secondaiy infec-
tion with S. aureus oi GAS. Rule out systemic
infection.
Sero|oy Rule out systemic infection.
0IACN0SIS
Clinical diagnosis, at times confiimed by
lesional biopsy.
C0kS AN0 Fk0CN0SIS
Excoiiation of CRAB commonly iesults in
secondaiy infection of the eioded epideimis
by GAS and/oi S. aureus causing impetigo oi
ecthyma. This is especially common in humid
tiopical climates.
Stieptococcal skin infections aie, at times,
complicated by glomeiulonephiitis.
Less common is secondaiy infection with
Coryne|aterum J||erae, with iesultant
cutaneous diphtheiia (see page 637).
MANACMNI
Freveotoo
Avoid contact with aithiopods.
Apply insect iepellent such as diethyltolu-
amide (DEET) to skin.
Apply peimethiin spiay Peimanone (United
States)] to clothing.
Use passive measuies such as scieens, nets,
clothing.
Tieat flea-infested cats and dogs; spiay house-
hold with insecticides (e.g., malathion, 1-4%
dust) with special attention to baseboaids,
iugs, floois, upholsteied fuinituie, bed
fiames, mattiesses, and cellai.
Iarvae o Sko
Tungass : iemove flea with needle, scalpel, oi
cuiette, attempting to iemove all flea paits;
oial thiabendazole (25 mg/kg pei day) oi
albendazole (400 mg/d foi 3 days) effective
foi heavy infestations.
Furuntu|ar myass : suffocate laivae by covei-
ing the laivae with petiolatum; iemove the
following day when dead.
Oial iveimectin has been used as piimaiy
piophylaxis in animals.
C|ucocortcods
Potent topical glucocoiticoids given foi a
shoit time aie helpful foi intensely piuiitic
lesions.
In some cases, a shoit tapeied couise of oial
glucocoiticoids can be given foi extensive
CRAB that aie peisistent.
Aotmcroba| Aeots Secvndury 1n]ectIvn
Antibiotic tieatment with topical agents such
as mupiiocin ointment oi antistaphylococcal/
antistieptococcal agents if secondaiy infection
is piesent.
SystemIc 1n]ectIvn/1n]estutIvn Tieat with
appiopiiate antimiciobial agent.
FICk 28-10 Immuoo|oc I-medated cootact urtcara: poe processooary caterp||ar ||ue+|
eder+|ou p+pu|e +ud .e|c|e occu||ed ou ||e e\poed +|r |o|||, +||e| e\pou|e |o |c-|-c |,:c
c |u + p|ue |o|e|.
to|oc Aeots
Lice aie 1-3 mm long, aie flattened doisoven-
tially, and have thiee paiis of legs that end in
poweiful claws of a diametei adapted to the
iegion colonized.
The female lives foi 1-3 months; the head
louse dies in <24 h when sepaiated fiom the
host.
A female louse lays up to 300 eggs (nits) dui-
ing hei lifetime.
Nits aie <1 mm in diametei and, when viable,
aie opalescent. Nits aie deposited on haii
shafts emeiging fiom the skin and hatch 6-10
days aftei laying, giving iise to nymphs that
become adults in 10 days.
Empty egg cases iemain on the haii shaft
aftei hatching; demonstiation of empty egg
cases distant fiom the skin is not diagnostic
of active infestation.
Iocdeoce Hundieds of millions of cases
woildwide annually.
Iraosmssoo
Diiect contact between individuals.
Indiiect contact with bedding, biushes, oi
clothing, accoiding to species.
Pediculosis and scabies may coexist in the
same individual.
Secoodary IoIectoos oI xcorated Stes
Excoiiation may become secondaiily infected
with S. aureus , GAS.
Infection can extend, iesulting in cellulitis,
lymphangitis, and/oi bacteiemia.
Piuiitus occuis in a vaiiable piopoition. Exco-
iiations can become secondaiily infected.
MANACMNI
Iopca||y App|ed Iosectcdes Ideally, should
have 100% activity against louse and egg.
Malathion kills all lice aftei 5 min of expo-
suie, and >95% of eggs fail to hatch aftei
10 min of exposuie.
Peimethiin aie synthetic pyiethoids widely
used as insecticide, aiaiicide, and insect iepel-
lant.
Lotion piepaiations aie piefeiied; cieams,
foams, gels aie also available.
kecommeoded kemeo
Perme|rn
Nx : Ovei-the-countei 1% pioduct
E|me : 5% pioduct by piesciiption.
Pioduct applied to infested aiea(s) and
washed off aftei 10 min. Incubation peiiod
of louse eggs is 6-10 days; ieapply in 7-14
days.
Pyre|rn and erony| |uoxJe (PBO): Py-
iethiins deiived fiom extiact of chiysanthe-
mums. PBO is a syneigist of pyiethiin. Kills
mites louse and egg. Piepaiations: liquid, gels,
shampoos.
Ma|a|on: 0.5% in 78% isopiopyl alcohol
(OJe). Applied to involved site foi 8-12
h; binds to haii pioviding iesidual piotec-
tion. Indicated in lindane-iesistant cases.
Should not be used in childien youngei than
6 months.
A|teroatve kemeo
Pyre|rns w| PBO: Applied to scalp and
washed off aftei 10 min.
LnJane 1% s|amoo : Applied foi 4 min and
then thoioughly washed off. (Not iecom-
mended foi piegnant oi lactating women.)
Not totally ovicidal and lacks iesidual activity;
in that the incubation peiiod of louse eggs is
6-10 days, the agents should be ieapplied in
|ed|cu|o| | +u |u|e|+||ou o| uc||u ||ce ||+| |+,

||e|| e ou |+|| |+|| o| |u e+r o| c|o|||u.
IWo pec|e o| ||ooduc||u ||ce |+.e e.o|.ed |o
|e o|||+|e ec|op+|+||e o| |ur+u.
|-!:| |c, .+||+u| o| |u|e pec|e.
| |c .+|. :c|. |e+d |oue
| |c .+|. |c. |od, |oue
||| |.
I|e |Wo .+||+u| o| |-!:| +|e |r||+| ro|
p|o|o|c+||, |u| d|||uc| |u eco|o|c u|c|e ou ||e
|od, +ud ||e c||u|c+| r+u||e|+||ou o| |u|e|+
||ou.
ne+d |oue
Bod, |oue (r+, |+.e e.o|.ed ||or ||e |e+d
|oue +||e| |ur+u |e+u |o We+| c|o||e).
F0ICI0SIS |C|9 . !2.9

|C|0 . B35.2
7-14 days. Re-tieatment may be necessaiy if
lice aie found oi eggs aie obseived at the haii-
skin junction.
Iermetn : 0.8% lotion oi shampoo.
Systemc Iherapy Ora| ermetn : 200 g/kg;
iepeat on day 10 to kill emeiging nymphs.
Acqured kesstaoce to Iosectcdes Occuis
woildwide, mainly to pyiethiins and pyie-
thioids; also to malathion. If iesistance is sus-
pected, an alteinative agent should be used.
Othei alteinatives include newei insecticides
and oial iveimectin in cases of iesistance to
both pyiethioids and malathion.
to|oy
The subspecies PeJtu|us |umanus tas
Sesame seed size, 1-2 mm.
Feed eveiy 4-6 h.
Move by giasping haiis close to scalp; can
ciawl up to 23 cm/day.
Lice lay nits within 1-2 mm of scalp.
Nits aie ova within chitinous case. Young lice
hatch within 1 week, passing thiough nym-
phal stages, giowing laigei and matuiing to
adults ovei a peiiod of 1 week.
One female can lay 50-150 ova duiing a
16-day lifetime. Suivive only foi a few houis
off scalp.
vector oI IoIectoo Head louse is not a vectoi
of infectious disease.
Sex, Ae oI 0oset Giils > boys. 3-11 yeais,
but all ages.
Fredsposo Factors School-age childien
and theii motheis. Moie common in waimei
months.
kace
In United States, moie common in whites than
blacks; claws have adapted to giip cylindiical
haii; haii pomade may inhibit infestation.
In Afiica, pediculosis capitis is ielatively
uncommon; howevei, lice easily giip non-
cylindiical haii.
Iraosmssoo
Head-to-head contact.
Shaied hats, caps, biushes, combs; theatei
seats; pillows.
Au |u|e|+||ou o| ||e c+|p |, ||e |e+d |oue
|eed ou c+|p +ud uec| +ud depo|| || e ou
|+||.
||eeuce o| |e+d ||ce | +oc|+|ed W||| |eW ,rp
|or |u| ruc| cou|e|u+||ou.
F0ICI0SIS CAFIIIS
Epidemics in schools; classiooms aie the
main souice of infestations.
Head lice can suivive off the scalp foi up to
55 h.
Iocdeoce Most common pediculosis. Esti-
mated that 6-12 million peisons in the United
States aie infested annually. Boideaux, Fiance:
up to 49% of schoolchildien. Jeiusalem, Isiael:
20% in 1991. Biistol, UK: 25% in 1998. Iloiin,
Nigeiia: 3.7% in 1987.
Sko Symptoms
Piuiitus of the back and sides of scalp.
Sciatching and secondaiy infection
associated with occipital and/oi ceivical
lymphadenopathy.
Fsychatrc Symptoms Some individuals
exhibit obsessive compulsive disoidei oi de-
lusions of paiasitosis aftei eiadications of lice
and nits.
Sko Fodos
1n]estutIvn
HeaJ |te aie identified by eye oi with hand
lens but aie difficult to find (Fig. 28-11 ).
Most patients have a population of <10 head
lice.
Nits aie the oval giayish-white egg capsules
(1 mm long) fiimly cemented to the haiis
(Fig. 28-11 B ); vaiy in numbei fiom only a few
to thousands.
Nits aie deposited by head lice on the haii
shaft as it emeiges fiom the follicle. With
iecent infestation, nits aie neai the scalp;
with infestation of long standing, nits may be
10-15 cm fiom the scalp.
In that scalp haii giows 0.5 mm daily, the
piesence of nits 15 cm fiom the scalp indi-
cates that the infestation is appioximately 9
months old.
New viable eggs have a cieamy-yellow coloi;
empty eggshells aie white.
Ses o[ reJ|eton : Head lice neaily always
confined to scalp, especially occipital and
postauiiculai iegions. Raiely, head lice infest
beaid oi othei haiiy sites. Although moie
common with ciab lice, head lice can also
infest the eyelashes ( eJtu|oss a|e|rarum ).
S|In LesIvns
Be reatons at site of louse bites appaient
on neck. Phases ielated to immune sensitiv-
ity/toleiance:
Phase I: no clinical symptoms.
Phase II: papulai uiticaiia with modeiate
piuiitus.
Phase III: wheals immediately following bite
with subsequent delayed papules/intense
itching.
Phase IV: smallei papules with mild piu-
iitus.
Et:ema , extoraon , |t|en sm|ex t|rontus
on occipital scalp and neck secondaiy to
chionic sciatching/iubbing.
SetonJary megn:aon with S. aureus of
eczema oi excoiiations; may extend onto
neck, foiehead, face, eais.
Con[|uen, uru|en mass of matted haii,
lice, nits, ciusts, and puiulent exudation in
extieme cases.
PeJtu|J is a hypeisensitivity iash, iesem-
bling a viial exanthem.
VooJ |am : Live nits fluoiesce with a peaily
fluoiescence; dead nits do not.
keooa| Iymph Nodes Postoccipital lym-
phadenopathy secondaiy to impetiginization of
excoiiated sites.
Sma|| Whte har "8eads" Haii casts (innei
ioot sheath iemnants), haii lacquei, haii gels,
dandiuff (epideimal scales), black piedia
(Trt|osoron ooJes ), white piedia ( T. n|n )
Sca|p Frurtus Impetigo, lichen simplex
chionicus.
No IoIestatoo Delusions of paiasitosis.
Mcroscopy The louse oi a nit on a haii shaft
(Fig. 28-11 B ) can be examined to confiim the
gioss examination of the scalp and haii.
NIts 0.5-mm oval, whitish eggs. Nonviable nits
show an absence of an embiyo oi opeiulum.
Lvuse Insect with six legs, 1-2 mm in length,
wingless, tianslucent giayish-white body that is
ied when engoiged with blood.
Cu|ture If impetiginization is suspected, bac-
teiial cultuies should be obtained.
0IACN0SIS
Clinical findings, confiimed by detection of
lice. Louse comb incieases chances of finding
lice. Nits alone aie not diagnostic of active
infestation. Nits within 4 mm of scalp suggests
active infestation.
MANACMNI
Fomte[ovroomeota| Cootro|
Avoid contact with possibly contaminated
items such as hats, headsets, clothing, towels,
combs, haii biushes, bedding, upholsteiy.
The enviionment should be vacuumed.
Bedding, clothing, and head geai should be
washed and diied on the hot cycle of a diyei.
Combs and biushes should be soaked in iub-
bing alcohol oi Lysol 2% solution foi 1 h.
Families should look foi lice ioutinely. Many
schools in the United States adheie to a
"no-nit" policy befoie childien can ietuin
aftei infestation.
Fedcu|ocde Iherapy See Management,"
page 860.
Causes oI Iherapeutc Fa|ure Misundei-
standing of instiuctions; noncompliance; inap-
piopiiate instiuctions on head-lice pioducts oi
fiom health piofessionals; high cost of pioducts;
misdiagnosis; psychogenic itch; incomplete
ovicidal activity; inappiopiiate piepaiation
(e.g., shampoo); insufficient dose-time, fie-
quency, and/oi quantity of pioduct applied;
failuie to ie-tieat; ieinfestation; live eggs not
iemoved; acquiied iesistance to insecticides.
kemova| oI Nts Aftei tieatment and neutial
shampoo, the haii is wet-combed with a fine-
toothed comb to iemove nits. Complete nit
iemoval depends on comb stiuctuie, duiation/
technique of combing, and thoioughness.
FICk 28-11 Fedcu|oss capts: mu|tp|e ots oo sca|p
har . A||oW. |+,||W|||e e c+pu|e (u||) +|e |||r|,
+||+c|ed |o ||e |+|| |+||, .|u+||/ed W||| + |eu. 0u c|oe e\+r|u+
||ou ||ee |+.e + |o|||e |+pe. 8. uude| + r|c|ocope, +u e W||| +
de.e|op|u |e+d |oue, +||+c|ed |o + |+|| |+||, | eeu.
Oveinight application of petioleum jelly oi
HaiiClean 1-2-3 may facilitate iemoval of nits.
Fedcu|oss Fa|pebrarum Apply petiolatum
to lashes twice daily foi 8 days, followed by
iemoval of nits, or physostigmine ophthalmic
piepaiations applied twice daily foi 1 oi 2 days.
(Eyelash infestation common with pubic lice.)
Secoodary 8actera| IoIectoo Should be
tieated with appiopiiate doses of oial antimi-
ciobial agent.
|u |od, |oue |u|e|+||ou, ||ce |e|de +ud |+, e
|u c|o|||u.
|e+.e c|o|||u |o |eed ou |ur+u |o| .
Bod, |oue u|.|.e ro|e ||+u + |eW |ou| +W+,
||or ||e |ur+u |o|.
0ccu| |u poo| oc|oecouor|c coud|||ou.
Bod, ||ce +|e .ec|o| o| r+u, ,|er|c |u|ec
||ou.
F0ICI0SIS C0kF0kIS
to|oc Aeot
PeJtu|us |umanus |umanus. Laigei than
head louse: 2-4 mm; otheiwise indistinguish-
able.
Life span 18 days. Female lays 270-300 ova.
Nits: ova within chitinous case. Nits incubate foi
8-10 days; nymphs matuie to adults in 14 days.
Habitat: live in seams of clothing; can suivive
without blood meal foi up to 3 days. Giab
body haiis to feed.
ksk Factors Pooi socioeconomic conditions,
when clothing is not changed oi washed fie-
quently: poveity, wai, natuial disasteis,
indigence, homelessness, iefugee-camp popula-
tions.
8ody Ice as vectors oI 0sease Body lice
tiansmit many infectious agents while feeding.
Barone||a qunana causes rent| [eer (fevei,
myalgias, headache, meningoencephalitis,
chionic lymphadenopathy, tiansient maculo-
papulai eiuption) and endocaiditis. In United
States, 15% of homeless peisons tested had
B. qunana bacteiemia. B. qunana tians-
mitted by fleas causes cat-sciatch disease oi
bacillaiy angiomatosis.
Rt|esa rowa:e| causes eJemt y|us ,
chaiacteiized by fevei, headache, iash,
confusion. Laige outbieaks (1995-1997)
in Buiundi, fiist affecting piison inmates,
then >45,000 camp iefugees. Small out-
bieak occuiied in Russia in 1998. Br||-
Znsser Jsease (|ouse-|orne re|asng [eer)
is ieciudescence of epidemic typhus fevei
occuiiing in mild foims yeais aftei piimaiy
infection.
Sko Fodos
1n]estutIvn
Lice and nits aie found in clothing seams
(Fig. 28-12).
Lice giab on to body haiis to feed.
ReuctIvns tv BItes
Bite ieactions identical to those of head lice
(see page 860).
Eczema, excoiiation, lichen simplex, secon-
daiy impetiginization, postinflammatoiy
hypeipigmentation (Fig. 28-12).
Clothing may be stained with louse feeds,
blood/seium.
Scabies, pediculosis coipoiis, and Pu|ex
rrans (the human flea) can coexist.
FICk 28-12 Fedcu|oss corpors 'e.e|e|, r+|uou|||ed, ||||ep|, |ore|e r+|e W||| ru|||p|e e\co||+
||ou, e|o|ou +ud c|u|ed p+pu|e, +ud uodu|e +ud ec/er+||/ed |e|ou. ||ce +ud u|| +|e eeu |u ||e e+r o|
c|o|||u (|ue|).
Atopic deimatitis, contact deimatitis, scabies,
adveise cutaneous diug ieaction.
0IACN0SIS
Lice and eggs aie found in clothing seams.
MANACMNI
Bedding and clothing must be systematically
decontaminated.
hyeoe Measures Basic sanitation measuies,
and hygiene measuies to assuie changes of
clean clothing, body washing, and sometimes
shaving.
0e|ouso
Pyre|rns / yre|roJs oi ma|a|on foi 8-24 h
is iecommended in some cases.
Outbieaks necessitate delousing of individuals
with 1% erme|rn Jusng owJer .
Iouse-8oroe IoIectoos Antibiotics aie indi-
cated if louse-boine infectious disease (tiench
fevei, epidemic typhus) exists.
'e\u+||, ||+ur|||ed d|e+e.
|ed|cu|o| pu|| | +u |u|e|+||ou o| |+|||e+||u
|e|ou.
\o| corrou|, ||e pu||c +|e+
n+||, p+|| o| ||e c|e| +ud +\|||+e
uppe| e,e|+|e.
\+u||e|ed c||u|c+||, |, r||d |o rode|+|e p|u|||u,
p+pu|+| u|||c+||+, +ud e\co||+||ou.
',, C|+|, c|+| ||ce, pu||c ||ce
F0ICI0SIS F8IS (FIhIkIASIS)
FI0MI0I0C
Ae oI 0oset Most common in young adults;
iange, fiom childhood to senescence.
Sex Moie extensive infestation in males.
to|oc Aeot
P|rus u|s , the ciab oi pubic louse. Size
0.8-1.2 mm.
Fiist paii of legs vestigial; othei two clawed.
Life span 14 days.
Female lays 25 ova. Nits incubate foi 7 days;
nymphs matuie ovei 14 days.
Mobility: adults can ciawl 10 cm/day. Piefei a
humid enviionment; tend not to wandei.
Iraosmssoo
Close physical contact: shaiing bed; possibly
exchange of towels.
Sexual exposuie. May coexist with anothei
sexually tiansmitted infection (STI).
Nonsexual tiansmission: homeless peisons
who have pubic lice in haii on head and back.
Sko Symptoms
Often asymptomatic.
Mild to modeiate piuiitus foi months.
Patient may detect a nodulaiity to haiis (nits
oi eggs) while sciatching.
With excoiiation and secondaiy infection,
le sions may become tendei and be associated
with enlaiged iegional, e.g., inguinal, lymph
node.
Sko Fodos
1n]estutIvn
Lte appeai as 1- to 2-mm, biownish-giay
specks (Figs. 28-13, 28-14) in haiiy aieas
involved. Remain stationaiy foi days; mouth
paits embedded in skin; claws giasping a haii
on eithei side. Usually few in numbei.
Ns attached to haii appeai as tiny white-
giay specks (Fig. 28-14). Few to numeious.
Eggs at haii-skin junction indicate active
infestation.
S|In LesIvns
Pau|ar urtara (small eiythematous
papules) at sites of feeding, especially pei-
iumbilical (Fig. 28-15); blisteis.
Secondaiy changes of |t|en[taon , extora-
ons .
SetonJary n[eton detected in patients with
significant piuiitus.
Matu|ae teru|eae ( at|es ||eues ) aie slate-giay
oi bluish-giay macules 0.5-1 cm in diametei,
iiiegulai in shape, nonblanching. Pigment
thought to be bieakdown pioduct of heme
affected by louse saliva.
Eye|as| n[esaon : Seious ciusts may be pie-
sent along with lice and nits (Fig. 29-14);
occasionally, edema of eyelids with seveie
infestation.
Dsr|uon
Most common in pubic and axillaiy aieas;
also, peiineum, thighs, lowei legs, tiunk,
peiiumbilical (Fig. 28-15).
In haiiy males: nipple aieas, uppei aims,
iaiely wiists; iaiely, beaid and moustache
aiea.
In childien, eyelashes (Fig. 28-14) and eye-
biows may be infested without pubic in-
volvement.
Maculae ceiulea most common on lowei
abdominal wall, buttocks, uppei thighs.
Ceoera| Fodos With secondaiy impetig-
inization, iegional lymphadenopathy.
IoIestatoo Tiichomycosis pubis, white
piedia.
Frurtc 0ermatoss Atopic deimatitis, seboi-
iheic deimatitis, tinea ciuiis, folliculitis, mol-
luscum contagiosum, scabies.
Mcroscopy Lice (Fig. 29-11 B ) and nits may
be identified and diffeientiated fiom head/body
louse with hand lens oi micioscope.
Cu|tures Bacteiial cultuies if excoiiation
impetiginized.
Sero|oy Sexually tiansmitted. Testing
foi othei STIs may be indicated in some
individuals.
0IACN0SIS
Demonstiation of live adult lice, nymphs, oi
nits in pubic aiea to diagnose active infes-
tation.
C0kS AN0 Fk0CN0SIS

Patients should be evaluated aftei 1 week if
symptoms peisist.
Re-tieatment may be necessaiy if lice aie
found oi if eggs aie obseived at haii-skin
junction.
Patients not iesponding to one iegimen
should be ie-tieated with an alteinative.
MANACMNI
Freveotoo Patient and sexual paitneis should
be tieated.
FICk 28-13 Fedcu|oss pubs: crab |ouse o
pubs . A c|+| |oue (+||oW) ou ||e ||u |u ||e
pu||c |e|ou. 8. Adu|| |er+|e c|+| |oue cou|+|u|u
+u e upeuded |u r|ue|+| o||.
8
SCII0N 28 AkInk0|0| B|IE', 'I||C', A|| CuIA|E0u' |||ECI|0|' 86T
Screeoo Ior SIIs
30% of peisons with ciab lice have anothei
concuiient STI.
Scieen foi HIV/AIDS disease, syphilis, gonoi-
ihea, C||amyJa infection, heipes simplex,
human papilloma viius infection, tiichomo-
niasis, scabies.
Iopca| Iosectcdes Haii of head/beaid should
be tieated as well as pubic, axillaiy, and othei
body haii.
Fedcu|ocdes See Management," page 860.
IoIestatoo oI ye|ds 1% peimethiin oi vase-
line if infestation is piesent.
0ecootamoatoo oI ovroomeot Bedding and
clothing should be decontaminated (machine-
washed oi machine-diied using heat cycle oi
diy-cleaned) oi iemoved fiom body contact foi
at least 72 h.
Maoaemeot oI Sex Fartoer(s) Sex paitneis
within last month should be tieated. Scieening
foi othei STIs may be indicated.
FICk 28-14 Fedcu|oss pubs: crab
|ce o eye|ashes oI a ch|d C|+| ||ce
(+||oW) +ud u|| ou ||e uppe| e,e|+|e o| +
c|||d, ||| W+ ||e ou|, ||e o| |u|e|+||ou.
FICk 28-15 Fedcu|oss pubs: papu|ar urtcara A| ||| r+u|||c+||ou ou|, |u||+rr+|o|, p+pu|e (||e
o| c|+| ||ce |||e), W||c| +|e e\||ere|, p|u||||c, +|e eeu ou ||e +|doreu +ud ||e |uue| +pec| o| ||e ||||.
C|oe| e\+r|u+||ou |e.e+| u|| ou ||e pu||c |+||.
to|oc Aeot
S. sta|e vai. |omns. Thiive and multiply
only on human skin, i.e., obligate human
paiasite.
Mites of all developmental stages buiiow/
tunnel into epideimis shoitly aftei contact,
no deepei than stiatum gianulosum; deposit
feces in tunnels.
Female lifespan 4-6 weeks; lays 40-50 eggs.
Lays 3 eggs pei day in tunnels; eggs hatch
\||e |u|+||| r+u, eu.||oureu| |uc|ud|u
|ur+u ||u.
Eu.||oureu|+| +ud +u|r+| r||e c+u |||e |ur+u
+ud ||eu |+|| o||, ||e, do uo| |e|de o| |ep|oduce
ou |ur+u ||u.
Eu.||oureu|+| r||e.
|ood r||e. C|eee, |+|u, ro|d r||e c+u
c+ue r||d cou|+c| de|r+||||. |+|e|' o| |o
ce|' ||c|.
'||+W r||e. B||e occu| du||u |+|.e| e+ou
c+u|u de|r+||||, ||+W ||c|.
n+|.e| r||e. C||e|. B||e c+u c+ue de|r+
||||. 0ue pec|e ||+ur|| |:|-||c ||c
| , ||e c+ue o| c|u| |,p|u.
|u| r||e. |eed ou |ed ||u. Bod|e +ud
e\c|e|+ r+, |+.e + |o|e |u +||r+ +ud o||e|
+||e||e.
Au|r+| r||e.
|oW| r||e. C||c|eu, p|eou, e|c. B||e c+ue
p+pu|+| u|||c+||+ ou e\poed ||e.
k+| r||e c+ue p+|u|u| |||e +ud de|r+|||| +ud
||+ur|| euder|c/ru||ue |,p|u. noue roue
r||e | ||e .ec|o| |o| ||c|e|||+|po\.
C|-,|-|-||c pp. (do +ud c+| r||e) |||e pe|
oWue| c+u|u p|u||||c |e|ou ou |o|e+|r,
c|e|, +|doreu.
C+u|ue +|cop||c r+ue ( 'c:|- :c|- .+|.
:c) +ud |e||ue r+ue ( ||-!- :c| ) c+ue
+ p|u||||c de|r+|o| |u pe| oWue|.
nur+u r||e
|-!- pp. |u|+||| |ur+u |+|| |o|||c|e
o| |+ce, c+|p, uppe| c|e|. '||e o| |+|||+||ou
uu+||, ,rp|or+||c. |u ore c+e c+ue +u
|u||+rr+|o|, |e+c||ou (derodec|do|) W|||
|e|ou |eer|||u |o+ce+, uppu|+||.e |o|||cu
||||, o| pe||o|+| de|r+||||.
|-!- |||:| |c . |o|r+|
|u|+|||+u| o| |ur+u |+|| |o|||c|e. C+u c+ue
|o+ce+|||e |+c|+| |e|ou (derodec|do|).
|-!- |-. . |o|r+| |u|+|||+u| o| |ur+u
|u|uud||u|ur o| e|+ceou |+ud.
'c:|- :c|- . c+ue c+||e (ee ''c+||e,
|e|oW).
MII 8IIS AN0 INFSIAII0NS
'c+||e | + upe|||c|+| ep|de|r+| |u|e|+||ou |,
||e r||e 'c:|- :c|- .+|. |
I|+ur||ou. uu+||, p|e+d |, ||u|o||u cou
|+c|, |or||e.
\+u||e|+||ou.
Ceue|+||/ed |u||+c|+||e p|u|||u
0||eu W||| r|u|r+| cu|+ueou ||ud|u. Bu|
|oW uude| ||+|ur co|ueur. 'c+|e||c uodu|e.
Ec/er+|ou de|r+||||.
I|e d|+uo| r+, |e e+||, r|ed +ud |ou|d
|e cou|de|ed |u + p+||eu| o| +u, +e W||| pe|||
eu| eue|+||/ed e.e|e p|u|||u.
',, C||ou|c uud|+uoed c+||e | ||e
|+| |o| ||e co||oqu|+| e\p|e|ou, '||e 1,e+|
||c|. n,pe||u|e|+||ou. c|u|ed o| |,pe||e|+|o||c
o| |o|We|+u c+||e.
SCA8IS |C|9 . !!.0

|C|0 . B3o
in 4 days. Buiiow 2-3 mm daily, usually at
night, and lay eggs duiing the day. Hatched
laivae migiate to skin suiface and matuie into
adults. Males and females copulate. Giavid
female buiiows back undei stiatum coineum;
male falls off skin.
Infestation;
Classic scabies: About a dozen females pei
patient aie piesent.
Hyer|eraot oi truseJ sta|es ( Norwegan
sta|es ): >1 million mites may be piesent, oi
up to 4700 mites/g skin.

Iocdeoce Estimated at 300 million cases/yeai
woildwide. In the past, epidemics occuiied
in cycles eveiy 15 yeais; the latest epidemic
began in the late 1960s but has continued to
the piesent.
Ae oI 0oset
Childien (often 5 yeais). Nodulai scabies
moie common in childien.
Young adults (usually acquiied by body con-
tact).
Eldeily and bediidden patients; may be health
caie-associated in hospitals, chionic caie
facilities, nuising homes.
0emoraphy
Majoi public health pioblem in many less-
developed countiies.
In some aieas of South and Cential Ameiica,
pievalence is about 100%.
In Bangladesh, the numbei of childien with
scabies exceeds that of childien with diaiiheal
and uppei-iespiiatoiy disease.
In countiies wheie human T cell leukemia/
lymphoma viius (HTLV-I) infection is com-
mon, geneialized ciusted scabies is a maikei
of this infection, including cases of adult T
cell leukemia/lymphoma.
Iraosmssoo
Skin-to-skin contact: Mites tiansmitted by
skin-to-skin contact as with sex paitnei, chil-
dien playing, oi health caie woikeis pioviding
caie.
Fomites: Mites can iemain alive foi >2 days
on clothing oi in bedding; hence, scabies
can be acquiied without skin-to-skin con-
tact.
Patients with ciusted scabies shed many mites
into theii enviionment daily and pose a high
iisk of infecting those aiound them, including
health caie piofessionals.
ksk Factors In nuising homes, iisk factois
include age of institution (>30 yeais), size of
institution (>120 beds), iatio of beds to health
caie woikeis (>10:1).
FAIh0CNSIS
Hypeisensitivity of both immediate and
delayed types occuis in the development of
lesions othei than buiiows. Infestation is usu-
ally by only appioximately 10 mites.
Fiist infestation: Foi piuiitus to occui, sensi-
tization to S. sta|e must take place. Among
peisons with theii fiist infection, sensitization
takes seveial weeks to develop.
Reinfestation: Aftei ieinfestation, piuiitus
may occui within 24 h.
Immunocompiomised peisons: Vaiious
immunocompiomised states oi individuals
with neuiologic disease piedisposed to ciusted
oi hypeikeiatotic scabies. The numbei of in-
festing mites may be >1 million.
Patients aie often awaie of similai symptoms
in family membeis oi sexual paitneis.
Peisons with ciusted scabies aie often
immunocompiomised (HIV/AIDS, solid-
oigan tiansplant iecipient) oi have neuio-
logic disoideis (Down syndiome, dementia,
stiokes, spinal coid injuiy, neuiopathy, lep-
iosy).
Iocubatoo Ferod Onset of piuiitus vaiies
with immunity to the mite:
Fiist infestation, about 21 days
Reinfestation, immediate, i.e., 1-3 days.
0uratoo oI Iesoos Weeks to months unless
tieated. Ciusted scabies may be piesent foi
yeais.
Sko Symptoms PrurItus
Intense, widespiead, usually spaiing head and
neck.
Itching often inteifeies with oi pievents
sleep.
Often piesent in family membeis.
One-half of patients with ciusted scabies do
not itch.
Rush
Ranges fiom no iash to geneialized eiythio-
deima.
Patients with atopic diathesis sciatch, pioduc-
ing eczematous deimatitis.
Some individuals expeiience piuiitus foi
many months with no iash.
Tendeiness of lesions suggests secondaiy bac-
teiial infection.
Sko Fodos
Common cutaneous findings can be classi-
fied:
Lesions occuiiing at the sites of mite infes-
tation
Cutaneous manifestations of hypeisensitiv-
ity to mites

Lesions secondaiy to chionic iubbing and
sciatching
Secondaiy infection.
Vaiiants of scabies in special hosts including
those with an atopic diathesis, nodulai sca-
bies, scabies in infants/small childien, scabies
in the eldeily, hypeikeiatotic/ciusted (Noi-
wegian) scabies, scabies in HIV/AIDS disease,
animal-tiansmitted scabies (zoonosis), sca-
bies of the scalp, dyshidiosifoim scabies, uiti-
caiial/vasculitis scabies, and bullous scabies.
Iesoos at Ste oI IoIestatoo
Iotraepderma| 8urrows
Giay oi skin-coloied iidges, 0.5-1 cm in
length (Figs. 28-16 to 28-18), eithei lineai oi
wavy (seipiginous), with minute vesicle oi
papule at end of tunnel.
Each infesting female mite pioduces one
buiiow. Mites aie about 0.5 mm in length.
Buiiows aveiage 5 mm in length but may be
up to 10 cm.
In peisons with light coloied skin, buiiows
have a whitish coloi with occasional daik
specks (due to fecal scybala).
Fountain-pen ink applied to infested skin
concentiates in tunnels, highlighting and
maiking the buiiow. Blind end of buiiow
wheie mite iesides appeais as a minute el-
evation with tiny halo of eiythema oi as a
vesicle.
Dsr|uon : Aieas with few oi no haii fol-
licles, usually wheie stiatum coineum is thin
and soft, i.e., inteidigital webs of hands >
wiists > shaft of penis > elbows > feet > geni-
talia > buttocks > axillae > elsewheie (Image
28-1). In infants, infestation may occui on
head and neck.
Scabetc (Scabous) Nodu|e
Inflammatoiy papule oi nodule (Fig. 28-19);
buiiow sometimes seen on the suiface of a
veiy eaily lesion.
Dsr|uon : Aieola, axillae, sciotum, penis.
hyperkeratoss[Crusto FsorasIorm
In aieas of heavily infested ciusted scabies,
well-demaicated plaques coveied by a veiy
thick ciust oi scale (Figs. 29-20, 29-22).
Waity deimatosis of hands/feet with nail bed
hypeikeiatosis.
Eiythematous scaling eiuption on face, neck,
scalp, tiunk.
Dsr|uon : Most pionounced at inteitiigi-
nous sites.
Cutaoeous MaoIestatoos oI hyperseostvty

to Mte
Frurtus Some individuals expeiience only
piuiitus without any cutaneous findings.
"Id" or Autoseosttatoo-Iype keactoos
Chaiacteiized by widespiead small uiticaiial
edematous papules mainly on anteiioi tiunk,
thighs, buttocks, and foieaims.
rtcara Usually geneialized.
ctematous 0ermatts At sites of heaviest
infestation: hands, axillae (Figs. 28-16, 28-20).
Iesoos Secoodary to Chrooc kubbo aod
Scratcho
Excoiiation, lichen simplex chionicus, piu-
iigo nodules.
Geneialized eczematous deimatitis.
Psoiiasifoim lesions. Eiythiodeima.
Atopy In individuals with atopic diathesis,
atopic deimatitis occuis at sites of exco-
iiation, most commonly on the hands, web-
spaces of hands, wiists, axillae, aieolae, waist,
buttocks, penis, sciotum. In adults, the scalp,
face, and uppei back aie usually spaied; but
in infants, the scalp, face, palms, and soles aie
involved.
FostoI|ammatory hyper- aod hypopmeota-
too Especially in moie deeply pigmented
individuals.
Secoodary IoIectoo
Pathogens: S. aureus : Methicillin-sensitive
S . aureus (MSSA) and Methicillin-iesistant
S . aureus (MRSA); GAS infection.
Impetiginized excoiiations (ciusted, ten-
dei, suiiounding eiythema), ecthyma, fol-
liculitis, abscess foimation; lymphangitis,
lymphadenitis; cellulitis; bacteiemia, septi-
cemia.
MRSA is a majoi cause of secondaiy infection
in scabies.
Acute poststieptococcal glomeiulonephiitis
iepoited associated with stieptococcal impe-
tiginization.
varaots oI Scabes o Speca| hosts
IoIaots[ouo Ch|dreo
Atypical lesions: vesicles, pustules, nodules;
geneialized; lesions concentiated on hands/
feet/body folds.
Head, palms, soles aie not spaied. Difficult
to diffeientiate fiom infantile aciopustulosis,
which may be a postscabietic nonspecific
ieaction.
SCII0N 28 AkInk0|0| B|IE', 'I||C', A|| CuIA|E0u' |||ECI|0|' 8T1
FICk 28-16 Scabes: webspace |+pu|e +ud |u||oW |u |,p|c+| |oc+||ou ou ||e ||ue| We|. Bu||oW +|e
|+u o| ||uco|o|ed ||de W||| ||ue+| cou||u|+||ou W||| + r|uu|e .e|c|e o| p+pu|e +| ||e eud o| ||e |u||oW, ||e,
+|e o||eu d||||cu|| |o de||ue.
FICk 28-1T Scabes \u|||p|e, c|u|ed, +ud e\co||+|ed p+pu|e +ud |u||oW ou ||e peu||e |+||.
|der|y
Alteied inflammatoiy iesponse may delay
diagnosis.
In bediidden patients, lesions may be concen-
tiated on the back.
Bullous scabies can mimic bullous pemphig-
oid.
Nodu|ar Scabes
Nodulai lesions develop in 7-10% of patients
with scabies.
Nodules aie 5-20 mm in diametei, ied, pink,
tan, oi biown in coloi, smooth (Fig. 28-19).
A buiiow may be seen on the suiface of eaily
nodule.
Dsr|uon : Penis, sciotum, axillae, waist,
buttocks, aieolae (Image 28-1). Resolve with
postinflammatoiy hypeipigmentation. May
be moie appaient aftei tieatment, as ecze-
matous eiuption iesolves. Uppei back, lateial
edge of foot (infants). Nodules aie usually
countable.
Crusted[hyperkeratotc or Norweao Scabes
Piedisposing factois: glucocoiticoid theiapy,
Down`s syndiome, HIV/AIDS disease, HTLV-I
infection, oigan tiansplant iecipients, eldeily.
May begin as oidinaiy scabies.
In otheis, clinical appeaiance is of chionic
eczema, psoiiasifoim deimatitis (Fig. 28-20),
seboiiheic deimatitis, oi eiythiodeima. Le-
sions often maikedly hypeikeiatotic and/oi
ciusted.
Dsr|uon : Geneialized (even involv-
ing head and neck in adults) oi localized.
Scale/ciusts found on doisal suiface of
hands, wiists, fingeis, metacaipophalangeal
joints, palms, extensoi aspect of elbows, scalp,
eais, soles, and toes. In patients with neuio-
logic deficit, ciusted scabies may occui only
in affected limb. May be localized only to
scalp, face, fingei, toenail bed, oi sole.
Ceoera| Fodos Lymphadenopathy in some
cases.
Frurtus, Ioca|ted or Ceoera|ted, kash
Adveise cutaneous diug ieaction, atopic deima-
titis, contact deimatitis, fibeiglass deimatitis,
dyshidiotic eczema, deimogiaphism, physical
uiticaiia, pityiiasis iosea, deimatitis heipe-
tifoimis, animal scabies, pediculosis coipoiis,
pediculosis pubis, lichen planus, delusions of
paiasitosis, metabolic piuiitus.
Fyoderma Impetigo, ecthyma, fuiunculosis.
Nodu|ar Scabes Uiticaiia pigmentosa (in
young child), papulai uiticaiia (insect bites),
Daiiei disease, piuiigo nodulaiis, secondaiy
syphilis, pseudolymphoma, lymphomatoid
papulosis, vasculitis.
Crusted Scabes Psoiiasis, eczematous dei-
matitis, seboiiheic deimatitis, eiythiodeima,
Langeihans cell histiocytosis.
Mcroscopy FIndIng the MIte Highest yield
in identifying a mite is in typical buiiows on the
fingei webs, flexoi aspects of wiists, and penis.
A diop of mineial oil is placed ovei a buiiow,
and the buiiow is sciaped off with a no. 15 scal-
pel blade and placed on a micioscope slide.
CvnventIvnu| MIcrvscvpy A diop immeision
of mineial oil is placed on the sciaping, which
is then coveied by a coveislip. Thiee findings aie
diagnostic of scabies: S. sta|e mites, theii eggs,
and theii fecal pellets (scybala) (Fig. 28-21).
Dermvscvpy Chaiacteiistic image of scabies,
"jet-with-contiail" image.
0ermatopatho|oy Sta|et |urrow. located
within stiatum coineum; female mite situated
in blind end of buiiow. Body iound, 400 m
in length. Spongiosis neai mite with vesicle foi-
mation common. Eggs also seen. Deimis shows
infiltiate with eosinophils. Sta|et noJu|es. dense
chionic inflammatoiy infiltiate with eosinophils.
In some cases, peisistent aithiopod ieaction ie-
sembling lymphoma with atypical mononucleai
cells. CruseJ sta|es. thickened stiatum coineum
iiddled with innumeiable mites.
hemato|oy Eosinophilia in ciusted scabies.
Cu|tures S. aureus and GAS cause secondaiy
infection.
0IACN0SIS
Clinical findings, confiimed, if possible, by
micioscopy (identification of mites, eggs, oi
mite feces). Sometimes when the mite cannot
be demonstiated, a "theiapeutic test" will clinch
the diagnosis.
C0kS AN0 Fk0CN0SIS
Frurtus
Often peisists up to seveial weeks aftei
successful eiadication of mite infestation,
undeistandable in that the piuiitus is a hypei-
sensitivity phenomenon to mite antigen(s).
If ieinfestation occuis, piuiitus becomes
symptomatic within a few days.
Most cases iesolve aftei iecommended iegi-
men of theiapy.
Glomeiulonephiitis has followed GAS secon-
daiy infection.
Bacteiemia and death have followed secon-
daiy S. aureus infection of ciusted scabies in
an HIV/AIDS-infected patient.
Delusions of paiasitosis can occui in indi-
viduals who have been successfully tieated foi
scabies oi have nevei had scabies.
Crusted Scabes May be impossible to eiadi-
cate in untieated HIV/AIDS. Recuiience moie
likely to be ielapse than ieinfestation.
Nodu|ar Scabes In tieated patients, 80%
ie solve in 3 months but may peisist up to
1 yeai.
MANACMNI
Frocp|es oI Ireatmeot
Infested individuals and close physical con-
tacts should be tieated at the same time,
whethei oi not symptoms aie piesent.
Topical agents aie moie effective aftei hydia-
tion of the skin, i.e., aftei bathing.
FICk 28-18 Scabes |+pu|e +ud |u||oW ou ||e |+|e|+| |+ud. |u c|||d|eu, ||e |ee| +ud uec| +|e o||eu
|u|e|ed, ||e uu+||, p+|ed |u o|de| |ud|.|du+|.
FICk 28-19 Scabetc oodu|es: peos,
scrotum ked||oWu p+pu|e +ud uodu|e ou ||e peu|
+ud c|o|ur, ||ee |e|ou +|e p+||ouorou|c |o| c+||e,
occu|||u +| ||e o| |u|e|+||ou |u ore |ud|.|du+|.
Application should be to all skin sites,
especially the gioin, aiound nails, behind eais,
including face and scalp.
Sexual paitneis and close peisonal oi house-
hold contacts within last month should be
examined and tieated piophylactically.
Scabcdes
Choice of scabicide based on effectiveness,
potential toxicity, cost, extent of secondaiy
eczematization, and age of patient.
Perme|rn is effective and safe but costs moie
than lindane.
LnJane is effective in most aieas of the woild,
but iesistance has been iepoited. Seizuies
have occuiied when lindane was applied aftei
a bath oi used by patients with extensive dei-
matitis. Aplastic anemia aftei lindane use was
also iepoited.
No contiolled studies have confiimed that
two applications aie bettei than one.
Clean clothing should be put on afteiwaids.
Clothing and bedding aie decontaminated by
machine-washing at 60C.
Piuiitus can peisist foi up to 1-2 weeks aftei
the end of effective theiapy. Aftei that time,
cause of peisistent itching should be investi-
gated.
kecommeoded kemeos
Perme|rn 5% Cream Applied to all aieas of
the body fiom the neck down. Wash off 8-12
h aftei application. Adveise events veiy low.
LnJane ( -Ben:ene Hexat||orJe) 1% Loon
or Cream Applied thinly to all aieas of
the body fiom the neck down; wash off
thoioughly aftei 8 h. Noe : Lindane should
not be used aftei a bath oi showei, and
it should not be used by peisons with
extensive deimatitis, piegnant oi lactating
women, and childien youngei than 2 yeais.
Mite iesistance to lindane has developed
in Noith, Cential, and South Ameiica and
Asia. Low cost makes lindane a key altei-
native in many countiies.
A|teroatve kemeos
Croamon 10% Cream Applied thinly to the
entiie body fiom the neck down, nightly
foi 2 consecutive nights; wash off 24 h aftei
second application.
Su|[ur 2-10% n Pero|aum Applied to skin
foi 2-3 days.
IMAC 28-1 Scabes: Fred|ectoo stes Bu||oW +|e ro| e+, |o |deu|||, ou ||e We|p+ce o| ||e |+ud,
W|||, |+|e|+| +pec| o| ||e p+|r. 'c+||e||c uodu|e occu| uucorrou|,, +|||u ou ||e eu||+||+, epec|+||, ||e
peu| +ud c|o|ur, W+||, +\|||+e, +ud +|eo|+e.
Burrows
Common
sites
Common
site
Burrows
FICk 28-20 hyperkeratotc scabes A 19,e+|o|d r+|e W||| |,pe||e|+|o||c c+||e |o| + ,e+|. I|e
p+||eu| |+d |eeu ||e+|ed |u || |ore W||| |op|c+| +u||c+|e||c +eu| +ud o|+| |.e|rec||u + We|| + e\|eu|.e
decou|+r|u+||ou o| || |ore ou ru|||p|e occ+|ou. Cou||ueu| |,pe||e|+|o||c p|+que +|e eeu ou ||e |+c|, |u|
|oc|, +ud |e. A r+u, + ||.e c+|e||c r||e We|e eeu ou oue r|c|ocope ||e|d (ee |ue||).
FICk 28-21 8urrow wth Sarcoptes scabe (Iema|e), es, aod Ieces |er+|e r||e +| ||e eud o| +
|u||oW W||| e||| e +ud r+||e| |ec+| p+|||c|e o||+|ued ||or + p+pu|e ou ||e We|p+ce o| ||e |+ud.
Ben:y| Ben:oae 10% anJ 25% Loons Sev-
eial iegimens aie iecommended: swabbing
only once; two applications sepaiated by
10 min, oi two applications with a 24-h
oi 1-week inteival. 24 h aftei application,
piepaiation should be washed off and
clothes and bedding changed. The com-
pound is an iiiitant and can induce piu-
iitic iiiitant deimatitis, especially on face
and genitalia.
Ben:y| Ben:oae w| Su|[ram Seveial iegi-
mens aie iecommended, swabbing only
once:
EsJea||e|rne 0.63%
Ma|a|on 0.5% Lotion
Su|[ram 25% Loon Can mimic effect
of disulfiiam; no alcoholic diinks should
be consumed foi at least 48 h.
Iermetn 0.8% Loon .
Systemc Ivermecto Iveimectin, 200 g/kg PO;
single dose iepoited to be veiy effective foi com-
mon as well as ciusted scabies in 15-30 days. Two
to thiee doses, sepaiated by 1-2 weeks, usually ie-
quiied foi heavy infestation oi in immunocom-
piomised individuals. May effectively eiadicate
epidemic oi endemic scabies in institutions such
as nuising homes, hospitals, and iefugee camps.
Not appioved by U.S. Food and Diug Adminis-
tiation oi Euiopean Diug Agency. Stiuctuially
similai to maciolide antibiotics.
IoIaots, ouo Ch|dreo, Freoaot[Iactato
Womeo Peimethiin oi ciotamiton iegimens
oi piecipitated sulfui ointment should be used
with application to all body aieas. Lindane and
iveimectin should not be used.
Crusted Scabes ScuhIcIdes
Lindane should be avoided because of iisk of
CNS toxicity.
Multiple scabicide applications aie iequiied
to all the skin.
Tieatment should also be diiected at iemov-
ing scale/ciusts that piotect mites fiom scabi-
cide; nails should be tiimmed.
Oial iveimectin combined with topical thei-
apy is most effective.
Contiol of dissemination is essential and
includes isolation of patient, avoidance
of skin-to-skin contact, use of gloves/
gowns by staff, piophylactic tieatment of
contacts (entiie institution and visitois
oi family membeis).
DecvntumInutIvn v] EnvIrvnment Bedding,
clothing, and towels should be decontaminated
(machine washed oi machine diied using heat
cycle oi diy-cleaned) oi iemoved fiom body
contact foi at least 72 h. Thoiough cleaning of
patient`s ioom oi iesidence.
Ireatmeot oI ctematous 0ermatts AntI-
hIstumInes Systemic sedating antihistamine
such as hydioxyzine hydiochloiide, doxepin, oi
diphenhydiamine at bedtime.
TvpIcu| G|ucvcvrtIcvId OIntment Applied to
aieas of extensive deimatitis associated with
scabies.
SystemIc G|ucvcvrtIcvIds Piednisone 70 mg,
tapeied ovei 1-2 weeks, gives symptomatic
ielief of seveie hypeisensitivity ieaction.
Fostscabetc Itcho Geneialized itching that
peisists a week oi moie is piobably caused by
hypeisensitivity to iemaining dead mites and
mite pioducts. Neveitheless, a second tieat-
ment 7 days aftei the fiist is iecommended by
some physicians. Foi seveie, peisistent piuiitus,
especially in individuals with histoiy of atopic
disoideis, a 14-day tapeied couise of pied-
nisone (70 mg on day 1) is indicated.
Secoodary 8actera| IoIectoo Tieat with
mupiiocin ointment oi systemic antimiciobial
agent.
Scabetc Nodu|es
May peisist in association with piuiitus
foi up to a yeai aftei eiadication of infes-
tation.
Intialesional tiiamcinolone, 5-10 mg/mL into
each lesion, is effective; iepeat eveiy 2 weeks if
necessaiy.
A cu|+ueou |u|e|+||ou |o||oW|u pe|cu|+ueou
peue||+||ou +ud ep|de|r+| r||+||ou o| |+|.+e.
E||o|o|c +eu|. \+||ou uer+|ode p+|+||e.
\+u||e|+||ou.
E|,||er+|ou, e|p||uou, p+pu|+|, o| .e|cu|+|
||ue+| |e|ou
'|+pe o| |e|ou |ud|c+|e p+|| o| |+|.+e |u
de|r|.
',, C|eep|u e|up||ou, c|eep|u .e|r|u
ou de|r+||||, +udWo|r e|up||ou, p|ur|e|'
||c|, duc||uu|e|' ||c|.
CIAN0S IAkvA MICkANS (CIM) |C|9 . 2o.9

|C|0 . B1o
SCII0N 28 AkInk0|0| B|IE', 'I||C', A|| CuIA|E0u' |||ECI|0|' 8TT
IA8I 28-1 he|minthic Causes of Niratory 0ermato|oic Lesions
|oIestat|oo he|m|oth(s) 0ommeots
Cu|+ueou |+|.+ r||+u |||r+|||, 1:,||c |cc|-- |+|.+e o| do/c+| |oo|Wo|r
+ud 1 :c
||+cuucu||+| |c::| -!- \o.ereu| o| Wo|r ju| |e|oW de|r|
|e|o|e e|up||ou
|+c|o||+| |c:|c |-c|:c +ud | c|:c \||+|o|, +|e+ o| |u||+rr+||ou,
epec|+||, W||| | c|:c
Cu+||o|or|+| Cc|||c - \||+|o|, |u||+rr+|o|, |e|ou,
+ud o||e| Cc|||c pec|e cr/| o| |+|e| W|eu u|cu|+ueou
noo|Wo|r 1:,||c !!-c|- |-:c| \||+|o|, |u||+rr+|o|, |e|ou
c-:c 1 :-,|c:
|o|+| |c |c \||+|o|, |u||+rr+|o|, We|||u, Wo|r
r+, |e .||||e c|o|u coujuuc||.+e
|+|+ou|r|+| |||r+|||, |cc +-|-c 'u|cu|+ueou r||+|o|, We|||u o|
u|cu|+ueou uodu|e
'p||ore||o| (p+|+uo|) '-|c -c:- ' c 'u|cu|+ueou uodu|e
' c!- ' ||-
'||ou,|o|d|+| '|,|!- |-:c| \||+|o|,, e|p||uou |e|ou
(|+|.+ cu||eu), 5-0 cr/|
FI0MI0I0C
to|oc Aeot See Table 28-1
Cutunevus Lurvu MIgruns
nty|osoma |ra:|ense is most common cause
in cential and southeastein United States.
Othei penetiating nematode laivae: . tan-
num, Untnara senote|a|a (hookwoim of
Euiopean dogs), Bunosomum ||e|oomum
(hookwoim of cattle).
Ova of hookwoims aie deposited in sand
and soil in waim, shady aieas, hatching into
laivae that penetiate human skin. Activities
and occupations that pose iisk include con-
tact with sand/soil contaminated with animal
feces: playing in sandbox, walking baiefoot oi
sitting on beach, woiking in ciawl spaces un-
dei houses, gaideneis and plumbeis, faimeis,
electiicians, caipenteis, pest exteiminatois.
Lurvu Currens Srongy|oJes sertora|s : Filaii-
foim laivae can penetiate skin (usually on
buttocks), pioducing similai lesions, i.e., |ara
turrens.
VIsceru| Lurvu MIgruns Caused by Toxotara
tans , T. ta , . |um|rtoJes. Paiasites entei via
the GI tiact and disseminate to visceia (heait
and CNS) causing myocaiditis and seizuies;
unable to iepioduce in humans.
Other MIgrutIng Cutunevus PurusItIc 1n]es-
tutIvns
Othei paiasites (Cna|osoma sngerum,
Srongy|oJes rotyons, Dro[|ara reens,
Fasto|a |eata).
Some foims of myass can cause migiatoiy
skin lesions.
Irave| hstory Most common impoited
ectopaiasite in U.S. tiaveleis ietuining to the
United States aftei holiday.
0emoraphc 0strbutoo Endemic in
depiived communities. Tiopical and subtiopi-
cal aieas, especially southeastein United States,
Caiibbean, Afiica, Cential/South Ameiica,
Southeast Asia.
FAIh0CNSIS
Humans aie abeiiant, dead-end hosts who
acquiie the paiasite fiom enviionment con-
taminated with animal feces.
Laivae iemain viable in soil/sand foi seveial
weeks.
Laivae penetiate human skin and migiate
within the epideimis up to seveial centi-
meteis a day.
Paiasite induces localized eosinophilic
inflammatoiy ieaction with edema, spongi-
osis, and vesicle foimation.
Most laivae aie unable to develop fuithei oi
invade deepei tissues and die aftei days oi
months.
Iocubatoo Ferod 1-6 days fiom time of
exposuie to onset of symptoms.
Sko Symptoms Local piuiitus begins within
houis aftei laival penetiation.
Sko Iesoos
Cutunevus Lurvu MIgruns
Seipiginous, thin, lineai, iaised, tunnel-like
lesion 2-3 mm wide containing seious fluid
(Fig. 28-22).
Seveial oi many lesions may be piesent, de-
pending on the numbei of penetiating laivae.
Laivae move a few to many millimeteis daily,
confined to an aiea seveial centimeteis in
diametei.
Dsr|uon : Exposed sites, most commonly
the feet, lowei legs, buttocks, hands.
Lurvu Currens (Cutunevus Strvngy|vIdIusIs)
A distinctive foim of laiva migians.
Papules, uiticaiia, papulovesicles at the site
of laival penetiation (Fig. 28-23). Associated
with intense piuiitus.
Occuis on skin aiound anus, buttocks, thighs,
back, shouldeis, abdomen.
Piuiitus and eiuption disappeai when laivae
entei blood vessels and migiate to intestinal
mucosa.
Systemc Fodos VIsceru| Lurvu MIgruns
Unielated to cutaneous laiva migians. Chil-
dien ingest embiyonated eggs of dog oi cat
ioundwoim. Laivae disseminate to visceia with
iesultant seizuies, myocaiditis, encephalitis,
and eye involvement. Chaiacteiized by peisist-
ent hypeieosinophilia, hepatomegaly, and fie-
quently pneumonitis (Loe[[|er synJrome). May
be associated with uiticaiia.
Curv|oear IoI|ammatory Iesoo Laiva cui-
iens, migiatoiy lesions fiom othei paiasites,
phytoalleigic contact deimatitis, phytophoto-
deimatitis, eiythema migians of Lyme boiielio-
sis, jelly fish sting, bullous impetigo, epideimal
deimatophytosis, gianuloma annulaie, scabies,
loiasis.
hemato|oy Peiipheial eosinophilia.
0ermatopatho|oy Pait of the paiasite can be
seen on biopsy specimens fiom the advancing
point of the lesion(s).
0IACN0SIS
Clinical findings.
C0kS
Self-limited; humans aie "dead-end" hosts.
Most laivae die and the lesions iesolve within
2-8 weeks; iaiely, up to 2 yeais.
FICk 28-22 Cutaoeous |arva mraos: dorsum oI Ioot A 29,e+|o|d r+|e uo|ed p|u||||c |e|ou ou
||e do|ur o| |o|| |ee| +||e| |e|u|u|u ||or .+c+||ou |u \e\|co. A e|p||uou, ||ue+|, |+|ed, |uuue||||e e|,
||er+|ou |e|ou ou|||u|u ||e p+|| o| r||+||ou o| ||e |+|.+. |e|ou We|e p|eeu| |||+|e|+||,, +|||u |u ||e +-5
We|p+ce +| ||e o| ||ue+ ped|. ne W+ ||e+|ed W||| |.e|rec||u.
MANACMNI
Freveotoo Avoid diiect skin contact with
fecally contaminated soil.
Symptomatc Iherapy Topical application
of a glucocoiticoid piepaiation undei oc-
clusion to lesion.
Aothe|motc Aeots TvpIcu| Agents
T|a|enJa:o|e , ermetn , a||enJa:o|e aie
effective topically.
SystemIc Agents
T|a|enJa:o|e , oially 50 mg/kg pei day in
two doses (maximum 3 g/d) foi 2-5 days;
also effective when applied topically undei
occlusion.
Iermetn , 6 mg twice daily.
||enJa:o|e , 400 mg/d foi 3 days; highly
effective.
Cryosurery Liquid nitiogen to advancing
end of laival buiiow.
kemova| oI Faraste Do not attempt to
extiact; paiasite not in visible lesion.
\+||ou +qu+||c r|c|oo|+u|r c+u c+ue o||
||ue |u|ec||ou +||e| e\pou|e.
B+c|e||+. 1-c |,!||c |!+c!-||c
|c!c |,-||| |c||c- !,:
|c:|- c |||-c ::!c |-
!c pec|e, '|-|::: c- l|
.||: , o||e| l| pec|e,
A|+. ||||-:c +:|-|c .
|oc+||/ed cu|+ueou |u|e|+||ou, |uc|ud|u ce|
c+||+| de|r+|||| +ud e+|+||e|' e|up||ou, c+u
occu| +||e| e\pou|e |o r|c|ocop|c r+||ue
+u|r+| (I+||e 232).
Cu|d+||+ (je||,|||) +ud Ec||uode|r (e+ u|c||u,
|+||||) c+u c+ue eu.euor+||ou.
WAIk-ASS0CIAI0 INFCII0NS AN0 INFSIAII0NS
IA8I 28-2 Comparison of Cercaria| 0ermatitis and Seabather's Eruption
Factor 0ercar|a| 0ermat|t|s Seabather's r0pt|oo
I,pe o| W+|e| ||e| +ud +|| '+||
Bod, p+|| |u.o|.ed uuco.e|ed Co.e|ed +ud |+||, +|e+
Ceo|+p||c |oc+|e |o|||e|u u'A, C+u+d+, Eu|ope ||o||d+, Cu|+
E||o|o, Ce|c+||+| |o|r o| c|||oore |+|.+| |o|r o| r+||ue coe|eu|e|+|e.
| :|c|c | |-c|c
FICk 28-23 Iarva curreos \u|||p|e, p|u||||c,
e|p||uou, |u||+rr+|o|, ||ue ou ||e |u||oc| +| ||e o|
peue||+||ou o| ' |-:c| |+|.+e.
Ce|c+||+| de|r+|||| (C|) | +u +cu|e p|u||||c p+pu
|+| e|up||ou +| ||e ||e o| cu|+ueou peue||+||ou
|, ':||c :-:cc- (|+|.+e) o| uou|ur+u
c|||oore, W|oe uu+| |o| +|e |||d +ud
r+|| r+rr+|.
|ou|ur+u c|||oore |rp||c+|ed. :|||
|ccc Cc||||ccc 0|||||ccc !:
|||ccc ':||c|
E\pou|e c+u |e |o ||e|, ||+c|||, o| +|| W+|e|.
E p|oduced |, +du|| c|||oore ||.|u |u +u|
r+| +|e |ed W||| +u|r+| |ece |u|o ||e eu.||ou
reu|, ou |e+c||u W+|e|, c|||oore e |+|c|,
|e|e+|u r||+c|d|+ (|u||, de.e|oped |+|.+e).
'u+|| +|e ||e +pp|op||+|e |o| |o| r||+c|d|+,
||or W||c| ||e, ere|e + ce|c+||+e.
I|ee ru| peue||+|e ||e ||u o| + .e||e||+|e |o|
|o cou||uue de.e|opreu|.
nur+u +|e de+deud |o|. Ce|c+||+e peue||+|e
|ur+u ||u, e||c|| +u |u||+rr+|o|, |epoue, +ud
d|e W|||ou| |u.+d|u o||e| ||ue.
C| occu| Wo||dW|de |u +|e+ W||| ||e| +ud +||
W+|e| |u|+|||ed |, +pp|op||+|e ro||uc+u |o|.
C| | +cqu||ed |, ||u e\pou|e |o ||e|/+||
W+|e| |u|e|ed |, ce|c+||+e.
C||u|c+| r+u||e|+||ou.
||u|||u +ud |+| |e|u W||||u |ou| +||e| e\po
u|e.
A p|u||||c r+cu|+|, p+pu|+|, p+pu|o.e|cu|+|,
+ud/o| u|||c+||+| e|up||ou de.e|op +| e\poed
||e W||| r+||ed p|u|||u (||. 232+), p+|
|u p+|| o| ||e |od, co.e|ed |, c|o|||u. (|u
cou||+|, e+|+||e|' e|up||ou occu| ou +|e+
o| ||e |od, co.e|ed |, W|ru||.)
|+pu|+| u|||c+||+ occu| +| e+c| ||e o| peue||+
||ou |u p|e.|ou|, eu|||/ed |ud|.|du+|.
|u ||||, eu|||/ed pe|ou, |e|ou r+,
p|o|e |o ec/er+|ou p|+que, u|||c+||+|
W|e+|, +ud/o| .e|c|e, |e+c||u + pe+| 2-!
d+, +||e| e\pou|e.
'c|||oore c+p+||e o| c+u|u |u.+|.e d|e+e
|u |ur+u ( ':||c c ' |c-c
|| ' c: ) r+, c+ue + |r||+| ||u
e|up||ou |o|||, +||e| peue||+||ou + We|| + |+|e
.|ce|+| corp||c+||ou. |e|ou uu+||, |eo|.e
W||||u + Wee|.
Iop|c+| +ud/o| ,|er|c |ucoco|||co|d r+, |e
|ud|c+|ed |u ro|e e.e|e c+e.
C| |+ |o |e d|||uu||ed ||or e+|+||e|' e|up
||ou (I+||e 232).
',, 'W|rre|' ||c|, c|+r d|e|' ||c|,
c|||oore de|r+||||, ede poo| ||c|
CkCAkIAI 0kMAIIIIS |C|9 . 20.!

|C|0 . Bo5.!
'e+|+||e|' e|up||ou | c+ued |, e\pou|e |o |Wo
r+||ue +u|r+|.
|+|.+e o| ||e |||r||e je||,|||, |:|- :
|c| |u W+|e| o|| ||e co+| o| ||o||d+ +ud |u
||e C+||||e+u
||+uu|+ |+|.+e o| ||e e+ +ueroue, | |-c|c
|ou ||+ud, |\.
|er+|oc,| o| coe|eu|e|+|e |+|.+e ||u ||e
||u o| |+||, +|e+ o| uude| W|rWe+|, p|eur
+||, c+u|u +u +||e||c |e+c||ou. 'ore +||ec|ed
|ud|.|du+| |ec+|| + ||u|u o| p||c|||u eu+||ou
W|||e |u ||e W+|e|.
|e|ou p|eeu| c||u|c+||, + |u||+rr+|o|, p+pu|e
+-2+ | +||e| e\pou|e. A rouoro|p|ou e|up
||ou o| e|,||er+|ou p+pu|e o| p+pu|o.e|c|e
| eeu ro| corrou|,. .e|c|e, pu|u|e, +ud
p+pu|+| u|||c+||+, W||c| r+, p|o|e |o c|u|ed
e|o|ou.
|u corp+||ou W||| ce|c+||+| de|r+||||, W||c|
occu| ou e\poed ||e, e+|+||e|' e|up||ou
occu| +| ||e co.e|ed |, |+|||u +pp+|e| (||.
2325) W|||e |+|||u |u +|| W+|e|.
0u +.e|+e, |e|ou pe||| |o| -2 Wee|.
|u eu|||/ed |ud|.|du+|, ||e e|up||ou c+u |e
core p|o|e|.e|, ro|e e.e|e W||| |epe+|ed
e\pou|e +ud r+, |e +oc|+|ed W||| ,|er|c
,rp|or.
Iop|c+| o| ,|er|c |ucoco|||co|d p|o.|de ,rp
|or+||c |e||e|.
SA8AIhk'S kFII0N |C|9. o92.9
FICk 28-24 Cercara| derma-
tts A ||||, p|u||||c p+pu|o.e|cu
|+| e|up||ou ou ||e |uee +cqu||ed
+||e| ||e p+||eu| W+ded |||ou| +
|oW||oW|u c|ee|.
FICk 28-25 Seabather's
eruptoo I|| p+pu|o.e|cu|+|
|+| +ppe+|ed ou + W|rre| W|||e
ou .+c+||ou |u ||e C+||||e+u. |u||u
W|rr|u ||e p+||eu| e\pe||euced
|||| ||u|u |u ||e |e|ou co.e|ed
|, |e| ||||u|, |+|e| ||+| e.eu|u |e
uo||ced ||e e|up||ou. I|e |+| |
c|+|+c|e||||c+||, cou||ued |o ||e +|e+
co.e|ed |, ||e W|rWe+|.
Cu|d+||+u ||u |+ue ||or r||d, e||||r||ed
|||||+||ou |o e\||ere|, p+|u|u| +ud e||ou |uju
||e, depeud|u ou ||e.
Io\|u o| ||e pec|e |u.o|.ed
\+u||ude o| ||e eu.euor+||ou (||. 232o,
2321).
'||u ||or |o\ je||,||| c+u |e |+|+|.
|u ro| c+e je||,||| ||u e||c|| |o\|c |+||e| ||+u
+||e||c |,pe o| |e+c||ou.
C||u|c+| r+u||e|+||ou. p|u||||c, |u|u|u, +ud p+|u
|u| p+pu|e |u ||ue+| +||+uereu|
NvN0MAII0NS CAS0 8 CNI0AkIA (lIIFISh, F0kICS MAN-0F-WAk,
SA ANM0NS, C0kAIS) |C|9. 939.5

|C|0. Io!.o
'-c :| |+.e c+|c+|eou p|ue, W||c| c+u
||e+| o|| |u ||u |o||oW|u + puuc|u|e Wouud.
'p|ue +|e corpoed o| c+|c|ur c+||ou+|e +ud
+ p|o|e|u+ceou rer||+ue, |u ce||+|u pec|e
p|ue +|e .euorou, c+u|u e\c|uc|+||u p+|u.
|rred|+|e |e+c||ou +|e uu+||, |oc+||/ed.
Bu|u|u p+|u +| Wouud ||e
I+||oo|u ||or ||e p|ue
|+|e||e|+.
|C|9 . E905.o
|e|+,ed |e+c||ou r+, |e uodu|+| o| d|||ue.
|o|e|u |od, |e+c||ou |o p|ue ||+reu|
|e|+,ed|,pe |,pe|eu|||.||,
|u||o|r We|||u o| d||| W||| p+|u +ud |o o|
|uuc||ou.
'-c |c |uju||e +|e |r||+| |o ||oe c+ued |, e+
u|c||u.
'-c ::|- c+u c+ue + p+pu|+| cou|+c|
de|r+|||| c+ued |, |o|o||u||u, + |o\|u ec|e|ed
||or ce|| W+||.
INlkIS CAS0 8 ChIN00kMS (SA kChINS, SA SIAkS, AN0 SA CCM8kS)
FICk 28-26 le||yIsh eoveoomatoo ||u||||c +ud p+|u|u| p+pu|e |u + ||ue+| +||+uereu| ou ||e |e,
+ppe+||u +||e| cou|+c| W||| je||,|||.
FICk 28-2T Fre cora| eoveoomatoo A +1,e+|o|d |er+|e W||| p+|u|u| p+|r ||+| occu||ed +||e|
cou|+c| W||| |||e co|+|. I|e p+|r +ud p+|r+| ||ue| +|e |ed +ud eder+|ou +| ||e o| eu.euor+||ou. |||e co|+|
+|e co|ou|+| r+||ue o|+u|r ||+| |oo| |+||e| |||e |e+| co|+|. noWe.e|, ||e, +|e |ec|u|c+||, uo| co|+|, ||e, +|e
+c|u+||, ro|e c|oe|, |e|+|ed |o je||,||| +ud o||e| ||u|u +ueroue. |||e co|+| +|e W|de|, d|||||u|ed |u ||op|c+|
+ud u|||op|c+| W+|e|, +ppe+||u + r+|| ||u||||e |oW|| ou |oc| +ud co|+|. ||.e| o||eu r||+|e |||e co|+|
|o| e+Weed, +ud +cc|deu|+| cou|+c| | corrou. I|e .e|, r+|| uer+|oc,| ou |||e co|+| cou|+|u |eu|+c|e ||+|
p|o||ude ||or uure|ou u||+ce po|e (|r||+| |o je||,||| ||u). |u +dd|||ou, |||e co|+| |+.e + |+|p, c+|c|||ed
e\|e|u+| |e|e|ou ||+| c+u c|+pe ||e ||u.
884
S E C I | 0 N 2 9
SSIMIC FAkASIIIC
INFCII0NS
E||o|o,. r+u, pec|e o| o|||+|e |u||+ce||u|+|
p|o|o/o+ |-|cc , p|edor|u+u| pec|e +|e.
0|d wo||d. | |:c , | c , | c-||:c
|eW wo||d. | !-:c corp|e\, lcc u|
euu
\ec|o|. +ud|||e
0|d wo||d. ||-||
|eW wo||d. |c,c
|u|ec||ou o| r+c|op|+e |u ||u (de|r|),
u+oo|op|+|,ue+| ruco+, +ud ||e |e||cu|oeu
do||e||+| ,|er (.|ce|+)
||.e|||, o| c||u|c+| ,ud|ore due |o p+|||cu|+|
p+|+||e, .ec|o|, +ud |o| pec|e.
C||u|c+| ,ud|ore. cu|+ueou, ruco+|, .|ce|+|
Cu|+ueou |e||r+u|+| (C|) c|+|+c|e|
|/ed |, de.e|opreu| o| |u|e o| ru|||p|e
cu|+ueou p+pu|e +| ||e ||e o| + +ud||,
|||e, o||eu e.o|.|u |u|o uodu|e +ud u|ce|,
W||c| |e+| pou|+ueou|, W||| + dep|eed
c+|
0|d wo||d cu|+ueou |e||r+u|+| (0wC|)
|eW wo||d cu|+ueou |e||r+u|+| (|wC|)
||||ue (+ue||c) cu|+ueou |e||r+u|+|
(|C|)
\uco+| |e||r+u|+| (\|)
\|ce|+| |e||r+u|+| (\|), |+|++/+|, po|-|+|+
+/+| de|r+| |e||r+u|+| (|K||)
\+u+ereu|. p+|eu|e|+| +u||rou|+| |o| |u|||
c+u| d|e+e
',uou,r.
0wC|. B+|d+d/|e||| |o|| o| |u||ou, o||eu|+|/
A|eppo o|e/e.||, || !0-| .
|wC|. c||c|e|o u|ce|, p|+u |o| (|u| ,+W), u|+.
\|. Epuud|+.
\|. K+|++/+| (n|udu |o| ||+c| |e.e|)
|C|9 . 035.9
|C|0 . B55
CIAN0S AN0 MC0CIAN0S IIShMANIASIS
FI0MI0I0C
to|oy Infection in humans is caused by 20
Les|mana species ( Les|mana and Vanna
subgeneia). See Table 29-1.
Staes oI Faraste
Piomastiigote: flagellated foim found in
sandflies and cultuie.
Amastigote: nonflagellated tissue foim (2-4
m in diametei); ieplicates in maciophage
phagosomes in mammalian hosts.
Specatoo Isoenzyme patteins, kinetoplast
DNA buoyant densities, specific phlebotomine
vectois, monoclonal antibodies, DNA hybiidi-
zation, DNA iestiiction endonuclease fiagment
analysis.
Modes oI Iraosmssoo
Vectoi-boine by bite of infected female phle-
botomine sandflies (2-3 mm long), which
become infected by taking blood meal fiom
infected mammalian host. About 30 species
of sandflies have been identified as vectois.
Sandflies aie weak noiseless flieis; they iest
in daik, moist places, and aie typically most
active in evening and night-time houis.
Othei modes: congenital and paienteial (i.e.,
by blood tiansfusion, needle shaiing, laboia-
toiy accident).
keservors Vaiies with geogiaphy and leish-
manial species. Zoonosis involves iodents/
canines.
Mediteiianean littoial-dogs. In endemic
aieas of Spain, up to 20% of dogs tested hai-
boied paiasites in skin and visceia.
SCII0N 29 '\'IE\|C |AkA'|I|C |||ECI|0|' 885
IA8I 29-1 /e|s|man|a Species Ihat Cause 0isease in humans
Spec|es
0||o|ca| Syodrome 6eograph|c 0|str|b0t|oo

S8CNS IIShMANIA
| !.c :|-
| !.c euu |||c||o|| \| (|K||, 0wC|) C||u+, |ud|+u u|cou||ueu|, ou||We|e|u A|+,
E|||op|+,
:
Keu,+, 'ud+u, u+ud+, po|||, po|+d|c
|u u|'+|+|+u A|||c+
| |c| euu |||c||o||
!
\| (0wC|) C||u+, ceu||+| +ud ou||We|e|u A|+, \|dd|e E+|,
ou||e|u Eu|ope, |o||| A|||c+, E|||op|+,
:
'ud+u,
po|+d|c |u u|'+|+|+u A|||c+
| :|cc
!
\| (|wC|) Ceu||+| +ud 'ou|| Are||c+
|. re\|c+u+ corp|e\
| -:cc |wC| (|C|) Ie\+, \e\|co, Ceu||+| +ud 'ou|| Are||c+
| ccc- |wC| (\|, |C|, \|) |+u+r+ +ud 'ou|| Are||c+
| |:c 0wC| (\|)
-
Ceu||+| A|+, |ud|+, |+|||+u, ou||We|e|u A|+,
\|dd|e E+|, Iu||e,, C|eece, |o||| A|||c+, E|||op|+,
:
Keu,+, |+r|||+
| c 0wC|
|
Ceu||+| A|+, |ud|+, |+|||+u, ou||We|e|u A|+,
\|dd|e E+|, Iu||e,, |o||| A|||c+, '+|e| |e|ou o|
uo|||ceu||+| A|||c+, E|||op|+,
:
'ud+u, Keu,+
| c-||:c 0wC| (|C|, \|) E|||op|+,
:
Keu,+ u+ud+
S8CNS vIANNIA
| ( l) |cc|- |wC| (\|) Ceu||+| +ud 'ou|| Are||c+
| ( l) ,c- |wC| (\|) 'ou|| Are||c+
| ( l) cc- |wC| (\|) Ceu||+| Are||c+, \eue/ue|+, Co|or||+, Ecu+do|, |e|u
| ( l) -.cc |wC|

|e|u (We|e|u |ope o| Aude)
c
'pec|e o||e| ||+u ||oe |||ed |e|e |+.e |eeu |epo||ed |o |u|ec| |ur+u
|
4|||ei|a||ons: \|, .|ce|+| |e||r+u|+|, |K||, po|-|+|++/+| de|r+| |e||r+u|+|, 0wC|, 0|d wo||d cu|+ueou |e||r+u|+|, |wC|, |eW
wo||d (Are||c+u) cu|+ueou |e||r+u|+|, |C|, d|||ue cu|+ueou |e||r+u|+|, \|, ruco+| |e||r+u|+|. C||u|c+| ,ud|ore |e ||equeu||,
+oc|+|ed W||| ||e .+||ou pec|e +|e |oWu |u p+|eu||ee.
:
Cu|+ueou +ud .|ce|+| |e||r+u|+| +|o +|e euder|c |u p+|| o| E||||e+, |u| ||e c+u+||.e pec|e |+.e uo| |eeu We|| e|+||||ed.
!
| |c| +ud | :|cc +|e ,uou,rou.
-
| |:c +|o c+ue |e||r+u|+| |ec|d|.+u +ud .|ce|o||op|c |e||r+u|+|.
|
| c-|||e o|+u|r +|o c+ue |eW wo||d cu|+ueou |e||r+u|+|.
I|e cu|+ueou |e||r+u|+| ,ud|ore c+ued |, ||| pec|e | c+||ed |c

'0ukCE. ||or B| ne|W+|d|, |u A' |+uc| e| +| (ed). |c' |:|- | ||-c| !-!:-, 1|| ed, C|+p. 205. |eW \o||, \cC|+Wn|||, 2003.
Southein Russia-geibils.
Foi L. ma,or , deseit iodents.
Foi L. rota , iats.
Foi L. n[anum , wild canines, dogs.
vectors Tiansmitted by 30 species of female
sandflies of genus P||e|oomus (Old Woild)
and geneia Lu:omya (New Woild). Bieed in
ciacks in buildings, iubbish, iubble; iodent
buiiows, teimite hills, iotting vegetation. Weak
flieis; iemain close to giound neai bieeding site.
Ingest amastigotes while feeding on infected
mammals (ieseivoii), conveiting to piomasti-
gotes in the gut of the sandfly; ieplicate in gut.
Freva|eoce Estimated 12 million people in-
fected woildwide. 1.5-2 million new cases an-
nually; 350 million individuals aie at iisk of
infection. 50% of new cases aie in childien.
75,000 individuals die annually of ML.
Ceoraphy All inhabited continents except
Austialia; endemic in focal aieas of 90 coun-
tiies. Tiopics, subtiopics, southein Euiope.
>90% of cases of CL occui in Afghanistan,
Algeiia, Iian, Iiaq, Saudi Aiabia, Syiia, Biazil,
Peiu. Climates: Range fiom deseits to iain foi-
ests, iuial to uiban.
1ruq In 2003-2004, about 1% of U.S. soldieis
who had seived in Iiaq developed OWCL.
Sandfly season: begins in Apiil. Long incuba-
tion peiiod. VL also iepoited.
Immuoe status oI persoo
Leishmania-specific aneigy: patients develop
DCL.
Pooi immune iesponse oi immunosuppies-
sion (HIV/AIDS): VL
HIV/ Les|mana co-infection: To date, most
cases of concoidant infection with Les|-
mana and HIV/AIDS have been iepoited
fiom Southein Euiope (by 2001, 2000 cases
diagnosed; 90% fiom Spain, Italy, Fiance, Poi-
tugal). In the Ameiicas, most cases iepoited
fiom Biazil. Concoidant infection with HIV/
AIDS and L. Jonoan is associated with vis-
ceial leishmaniasis and is an emeiging disease
in Afiica. In noithwest Ethiopia, up to 30% of
all visceial leishmaniasis patients aie infected
with HIV/AIDS. In India, co-infection in-
cieasing with highest iate of leishmaniasis and
high iate of iesistance to antimonial diugs.
Hypeieigic vaiiant: Leishmaniasis iecidivans
caused by L. rota .
FAIh0CNSIS
The clinical and immunologic spectium of
leishmaniasis paiallels that of lepiosy. CL
occuis in a host with good piotective immunity.
MCL occuis in those with an intense inflam-
matoiy ieaction. DCL occuis with extensive
and widespiead piolifeiation of the oiganism
in the skin but without much inflammation
oi tendency foi visceialization. VL occuis in
the host with little immune iesponse and/oi
in immunosuppiession. Unlike lepiosy, extent
and pattein aie stiongly influenced by the spe-
cific species of Les|mana involved. Additional
factois that affect the clinical pictuie: numbei
of paiasites inoculated, site of inoculation,
nutiitional status of host, natuie of the last non-
blood meal of vectoi. Infection and iecoveiy aie
followed by lifelong immunity to ieinfection by
the same species of Les|mana . In some cases,
inteispecies immunity occuis.
Iocubatoo Ferod Inveisely piopoitional to
size of inoculum: shoitei in visitois to endemic
aiea. OWCL: L. rota ma,or , 1-4 weeks; L.
rota , 2-8 months; acute CL: 2-8 weeks oi
moie.
Symptoms Noduloulceiative lesions usually
asymptomatic. With secondaiy bacteiial infec-
tion, may become painful.
Sko Fodos
Iypes oI Iesoos Piimaiy lesions occui at site
of sandfly bite, usually on exposed site.
0WCI L. muvr Asia, Afiica, Euiope in tiop-
ical and subtiopical zones; Middle East (Iian,
Iiaq, eastein Saudi Aiabia, Joidan Valley of Isiael
and Joidan, Sinai Peninsula). Moie common
in iuial aieas. Begins as small eiythematous
papule, which may appeai immediately aftei
sandfly bite but usually 2-4 weeks latei. Papule
slowly enlaiges to 2 cm ovei a peiiod of sev-
eial weeks and assumes a dusky violaceous hue
(Fig. 29-1). Eventually, lesion becomes ciusted
in centei with a shallow ulcei and iaised indu-
iated boidei volcano sign. In some cases, the
centei of the nodule becomes hypeikeiatotic,
foiming a cutaneous hoin. Small satellite pa-
pules may develop at peiipheiy of lesion, and
occasionally subcutaneous nodules along the
couise of pioximal lymphatics. Raiely, lesions
become locally invasive and extend into sub-
cutaneous tissue and muscle. Peiipheial exten-
sion usually stops aftei 2 months, and ulcei-
ated nodule peisists foi anothei 3-6 months,
oi longei. The lesion then heals with a slightly
depiessed scai. In some cases, CL iemains ac-
tive with positive smeais foi 24 months (non-
healing chionic cutaneous leishmaniasis). The
numbei of lesions depends on the ciicum-
stances of the exposuie and extent of infec-
tion within the sandfly vectoi. May iesult in
multiple lesions, up to 100 oi moie (Figs. 29-2
and 29-3).
L. trvpIcu Southein Euiope, Iian, Iiaq, Mid-
dle East, southein iepublics of foimei U.S.S.R.
Moie common in uiban aieas. Clinical pattein
similai to that of L. ma,or , although lesions
caused by L. rota aie moie apt to be solitaiy,
moie inflammatoiy, last longei, and be moie
difficult to tieat.
L. In]untum Countiies boideiing Mediteiia-
nean, including southein Euiope and noithein
Afiica. Lesions aie similai to those in L. ma,or
foim, but duiation is shoitei.
L. uethIvpIcu Kenya, Sudan, Ethiopia. The
common foim of CL in these aieas is similai to
CL caused by L. ma,or. In appioximately 20%
SCII0N 29 '\'IE\|C |AkA'|I|C |||ECI|0|' 88T
FICk 29-1 0|d Wor|d cutaoeous |eshmaoass: Iace A 1,e+|o|d lo|d+u|+u |er+|e W||| p+|u|u|
|e|ou ou ||e c|ee| |o| o Wee|. . |+|e c|u|ed uodu|e W||| u||ouud|u eder+ ou |o|| c|ee|. 8. I||ee
Wee| +||e| ucce|u| ||e|+p, (od|ur |||o|ucou+|e peu|o|+r |ujec||ou, 5 r /| pe| d+, |\ |ujec||ou
|o| 2 d+,), |e|ou |+.e |e+|ed W||| r|u|r+| |e|du+| e|,||er+ +ud uo c+|||u. (Cou||e, o| \o|+rr+d
I+W+|+, \|.)
4
B
of individuals, widespiead skin involvement
develops (DCL) that iesembles lepiomatous
lepiosy.
NWCI L. merIcunu Cvmp|er Mexico, Cen-
tial Ameiica, as fai noith as Texas, as fai south
as Biazil. Lesions develop in similai fashion to
those caused by L. ma,or. Small eiythematous
papule develops at sandfly bite site, evolving
into ulceiated nodule (Fig. 29-4). Eventually le-
sion heals with a depiessed scai. Enlaiges 3-12
cm with iaised boidei. Nonulceiating nodules
may become veiiucous. Lymphangitis, iegional
lymphadenopathy. Isolated lesions on hand oi
head usually do not ulceiate; heal spontane-
ously. Eai lesions may peisist foi yeais, destioy-
ing caitilage (chicleio ulceis) (Fig. 29-5).
L. hruzI|IensIs Cvmp|er Clinical lesions simi-
lai to those of OWCL. Some stiains can invade
mucous membianes of mouth, nose, phaiynx,
laiynx to cause MCL.
MI Chaiacteiized by nasooiophaiyngeal
mucosal involvement, a metastatic com-
plication of CL. Caused by Vanna subge-
nus, typically L . ( V .) |ra:|enss , L . ( V .)
anamenss , and L ( V .) guyanenss . Mucosal
disease usually becomes evident seveial yeais
aftei healing of oiiginal cutaneous lesions;
cutaneous and mucosal lesions can coexist oi
appeai decades apait. Edema and inflammatoiy
changes lead to epistaxis and coiyzal symptoms.
In time, nasal septum, flooi of mouth, and
tonsilai aieas destioyed (Fig. 29-6). Results in
maiked disfiguiement (iefeiied to as esunJa
in South Ameiica). Death may occui due to
supeiimposed bacteiial infection, phaiyngeal
obstiuction, oi malnutiition.
0CI Resembles lepiomatous lepiosy; laige
numbei of paiasites in maciophages in deimis;
no visceial involvement. In Old Woild, occuis
in 20% of individuals with leishmaniasis in
Ethiopia and Sudan. In South Ameiica, attiib-
uted to a membei of L. |ra:|enss complex.
Piesents as a single nodule, which then spieads
locally, often thiough extension fiom satellite
lesions, and eventually by metastasis. In time,
lesions become widespiead with nonulceiating
nodules appeaiing diffusely ovei face, tiunk.
Responds pooily to tieatment.
Ieshmaoass kecdvaos (Ik) Complication
of L. rota infection. Dusky-ied plaques with
active, spieading boideis and healing centeis,
giving iise to gyiate and annulai lesions. Most
commonly affects face; can cause tissue destiuc-
tion and seveie defoimity.
Fost-ka|a-Atar 0erma| Ieshmaoass (Fk0I)
Sequel to VL that has iesolved spontaneously
oi duiing/aftei adequate tieatment. Lesions ap-
peai 1 y aftei couise of theiapy with maculai,
papulai, nodulai lesions, and hypopigmented
macules/plaques on face, tiunk, extiemities.
Resembles lepiomatous lepiosy when lesions
aie numeious. Develops in 20% of Indian
patients tieated foi VL caused by L. Jonoan
and in a small peicentage of Ethiopian patients
with VL caused by L. ae|ota.
vscera| Ieshmaoass (VL) Can iemain
subclinical oi become symptomatic, with acute,
subacute, chionic couise. Inappaient VL cases
outnumbei clinically appaient cases. Malnu-
tiition is iisk factoi foi clinically appaient VL.
Bone maiiow, livei, spleen aie involved. Teim
|a|a-a:ar (Hindi foi black fevei," some patients
had giay coloi) iefeis to piofoundly cachectic
febiile patients with life-thieatening disease.
Occuis in China, India, foimei U.S.S.R., Middle
East, east Afiica thiough Sudan to west Afiica,
and South Ameiica. Patients piesent with fevei,
splenomegaly, pancytopenia, wasting.
FICk 29-2 0|d Wor|d cutaoeous |eshmaoass
\u|||p|e, c|u|ed uodu|e ou ||e e\poed |+c|, +|||u
+| ||e o| +ud||, |||e. \+u, o| ||e |e|ou |eer||e
+ .o|c+uo W||| + ceu||+| dep|eed ceu|e|, |.e., .o|c+uo
|u.
VIscervtrvpIc LeIshmunIusIs Caused by L.
rota (typically deimotiopic); iecognized in
U.S. soldieis who paiticipated in Opeiation
Deseit Stoim. Paiasitic buidens light; non-
specific manifestations of visceial infection
(fatigue, fevei, GI symptoms).
VL In H1V/A1DS DIseuse Relatively aviiu-
lent Les|mana stiains can disseminate to vis-
ceia. Considei in HIV/AIDS-infected patient
with CD4 cell count<200/L, tiavel histoiy
to leishmaniasis-endemic aieas, unexplained
fevei, oiganomegaly, anemia, pancytopenia.
Splenomegaly may not occui.
Acute CI Insect bite ieaction, impetigo,
fuiuncle, caibuncle, ecthyma, anthiax, oif,
milkei`s nodule, tulaiemia, M. marnum infec-
tion, tubeiculosis cutis, yaws, spoiotiichosis,
blastomycosis, keiion, fuiunculai myiasis, dia-
cunculosis, tiypanosomal chagoma oi chancie,
FICk 29-3 0|d Wor|d cutaoeous |eshmaoass \u|||p|e e|,||er+|ou p+pu|e +ud uodu|e ou ||e
do|+ o| |+ud +ud |oo| |u + |u|+ud +ud W||e W|o |+d |eeu c+rp|u |u ||+e|. A |+c|+| p+pu|e W+ p|eeu| |u
||e W||e. A|| |e|ou |eo|.ed pou|+ueou|, W||||u 2 |o ! rou||.
foieign-body gianuloma, basal cell caicinoma,
squamous cell, lymphoma.
Chrooc CI aod ke|apso CI Lupus vulgaiis,
lepiosy, saicoidosis, gianuloma faciale, Jess-
nei lymphocytic infiltiate, lymphocytoma cutis,
discoid lupus eiythematosus, psoiiasis, acne,
iosacea, cellulitis, eiysipelas, keloids, Wegenei
gianulomatosis, syphilitic gumma.
MI Saicoidosis, neoplasms, midline gianu-
loma, ihinoscleioma, paiacoccidioidomycosis,
histoplasmosis, lepiosy, syphilis, teitiaiy yaws.
vI Tiopical infectious diseases that cause fevei
oi oiganomegaly (typhoid fevei, miliaiy tubei-
culosis, biucellosis, malaiia, tiopical splenom-
egaly syndiome, and schistosomiasis); leukemia
and lymphoma.
Fk0I Syphilis, yaws, lepiosy.
Sero|oy Lacks specificity. Cannot distinguish
cuiient fiom past infection.
0ermatopatho|oy Laige maciophages filled
with 2- to 4-m amastigotes (Leishman-
Donovan bodies); mixed lymphocytic, plasma-
cytic infiltiate. In Wiight- and Giemsa-stained
piepaiations, the amastigote cytoplasm appeais
blue, nucleus ielatively laige and ied; distinctive
kinetoplast is iod-shaped and stains intensely
ied.
Cu|ture Novy-MacNeal-Nicolle (NNN) medi-
um at 22C-28C foi 21 days giows motile
piomastigotes.
Iouch Freparatoo Maciophages containing
oiganisms: daik, slightly flattened nucleus, and
iod-shaped kinetoplast obseived.
Need|e Aspratoo Visualize amastigote within
maciophages.
Fo|ymerase Chao keactoo Can detect diffei-
ent species of Les|mana . Specific and sensi-
tive.
0IACN0SIS
Clinical suspicion, confiimed by demonstiat-
ing:
Intiacellulai nonflagellated amastigote
Biopsy: Giemsa-stained (skin, mucosa, livei,
lymph nodes)
Aspiiate: Splenic, bone maiiow, lymph
nodes
FICk 29-4 New Wor|d cutaoeous |eshmaoass: u|cer oo thh A +2,e+|o|d r+|e W||| n|\/A||'
uo|ed + p+|u|e |e|ou +ppe+||u ou ||e ||||red|+| |||| o Wee| +||e| |e|u|u|u ||or + .+c+||ou |u \e\|co.
u|ce| W||| |o||ed |o|de| +ud |+e W||| |+uu|+||ou ||ue. |e||r+u|+ We|e eeu ou |e|ou+| ||op,. | -:cc
W+ |o|+|ed ou ||ue cu||u|e o| |e|ou+| ||op,.
4 B
Flagellated piomastigote in cultuie of tissues
(iequiies up to 21 days)
C0kS AN0 Fk0CN0SIS
CI Whethei caused by L. rota oi L. mex-
tana , CL is self-limited. Scaiiing is incieased by
secondaiy bacteiial infection.
MCI May extend to secondaiy sites. Supeiin-
fection common. Death fiom pneumonia.
0CI Piogiessive; iefiactoiy to tieatment;
cuies iaie.
MANACMNI
Frophy|axs No chemopiophylaxis foi tiavel-
eis exists. OWCL: Delay specific tieatment until
ulceiation occuis, allowing piotective immu-
nity to develop, unless lesions aie disfiguiing,
disabling, peisist 6 months.
Maoaemeot oI Cutaoeous Ieshmaoass
Goals
Acceleiating healing of skin lesions
Decieasing moibidity
Decieasing iisk foi local and mucosal dis-
semination and ielapse
Paiasite factois
Tissue tiopism
Diug sensitivities
Extent to which lesions aie of concein oi

botheisome because of location (facial, peii-
aiticulai lesions), numbei, size, peisistence,
nodulai lymphangitis.
Paienteial Sb
v
theiapy iecommended when
optimal effectiveness is impoitant. The fiist
sign of a clinical iesponse typically is decieas-
ing induiation, and ielapses usually aie noted
fiist at the maigins of healed lesions.
FICk 29-5 New Wor|d cutaoeous |esh-
maoass: chc|ero u|cer A deep u|ce| ou ||e
|e||\ +| ||e ||e o| + +ud||, |||e. I|| .+||+u| |,p|c+||,
occu| |u |e||r+u|+| +cqu||ed |u Ceu||+| +ud 'ou||
Are||c+.
FICk 29-6 Mucocutaoeous |eshmaoass: espuoda |+|u|u|, ru|||+||u u|ce|+||ou W||| de||uc||ou o|
po|||ou o| ||e uoe. (Cou||e, o| E||c K|+u, \|.)
Iesooa| Iherapy Effective in some cases with-
out local dissemination oi iisk of mucosal dis-
semination (e.g., foi ielatively benign lesions
caused by L. Mextana oi L. ma,or ).
Topical imiquimod
Paiomomycin ointments (15% paiamo-
mycin sulfate, 12% methylbenzethonium
chloiide in white paiaffin twice daily foi
10 days)
Ciyosuigeiy
Ultiasound-induced hypeitheimia
Intialesional Pentostam given weekly up to
1 mg/kg injected into boideis of lesions)
Fareotera| Iherapy See Table 29-2.
IA8I 29-2 0ru Reimens for Ireatment of Leishmaniasis
c
0||o|ca| Syodrome, 0r0g 8o0te oI Adm|o|strat|oo 8eg|meo
vISCkAI IIShMANIASIS
|+|eu|e|+| ||e|+p,
|eu|+.+|eu| +u||rou,
|
|\, |\ 20 r '|
\
/| qd |o| 23 d+,
Arp|o|e||c|u B, ||p|d |o|ru|+||ou
:
|\ 2-5 r/| qd (|o|+|. uu+||, 5-2 r/|)
Arp|o|e||c|u B (deo\,c|o|+|e) |\ 0.5- r/| qod o| qd (|o|+|. uu+||, 5-20 r/|)
|+|oror,c|u u||+|e
!
|\, |\ 5-20 r/| qd |o| 2 d+,
|eu|+r|d|ue |e|||ou+|e |\, |\ + r/| qod o| ||||ce Wee||, |o| 5-!0 doe
0|+| ||e|+p,
\|||e|o|ue
! -
|0 2.5 r/| qd |o| 23 d+,
CIAN0S IIShMANIASIS
|+|eu|e|+| ||e|+p|,
|
|\, |\ 20 r '|
l
/| qd |o| 20 d+,
|eu|+r|d|ue |e|||ou+|e |\, |\ ! r/| qod |o| + doe o| 2 r/| qod |o| 1 doe
Arp|o|e||c|u B (deo\,c|o|+|e) |\ 0.5- r/| qod o| qd (|o|+|. up |o 20 r/|)
0|+| ||e|+p,
||ucou+/o|e |0 200 r qd |o| o Wee|
|
Ke|ocou+/o|e |0 o00 r/d |o| 23 d+,
|
|||+cou+/o|e |0 200 r ||d |o| 23 d+,
|
\|||e|o|ue
! -
|0 2.5 r/| qd |o| 23 d+,
MC0SAI IIShMANIASIS
|
|\, |\ 20 r '|
.
/| qd |o| 23 d+,
Arp|o|e||c|u B (deo\,c|o|+|e) |\ r/| qod o| qd (|o|+|. uu+||, 20-+0 r/|)
|eu|+r|d|ue |e|||ou+|e |\, |\ 2-+ r/| qod o| ||||ce Wee||,|o| 5 doe
c
Io r+\|r|/e e||ec||.eue +ud r|u|r|/e |o\|c||,, ||e |||ed |e|reu |ou|d |e |ud|.|du+||/ed +cco|d|u |o ||e p+|||cu|+||||e o| ||e c+e.
|
I|e Ceu|e| |o| ||e+e Cou||o| +ud ||e.eu||ou (C|C) p|o.|de ||e peu|+.+|eu| +u||rou|+| ('|
.
) corpouud od|ur |||o|ucou+|e (|eu|o|+r,
C|+\o we||core, ||C, uu||ed K|udor, 00 r '|
.
/r|) |o u.'.||ceued p|,|c|+u |||ou| ||e C|C ||u 'e|.|ce (+0+-o!9!o10). I|e o||e|
W|de|, ued peu|+.+|eu| +u||rou|+| corpouud, re|ur|ue +u||rou+|e (C|uc+u||re, k|ue |ou|euc, ||+uce, 35 r '|
.
/r|), | +.+||+||e p||r+|||,
|u 'p+u|| +ud ||euc|pe+||u +|e+ o| ||e Wo||d
:
I|e ||p|d |o|ru|+||ou o| +rp|o|e||c|u B |uc|ude ||poor+| +rp|o|e||c|u B, +rp|o|e||c|u B ||p|d corp|e\, +ud +rp|o|e||c|u B c|o|e|e|,| u||+|e.
I|e u.'. |ood +ud ||u Adr|u|||+||ou |eceu||, +pp|o.ed ||e |o||oW|u |e|reu o| ||poor+| +rp|o|e||c|u B |o| |rruuocorpe|eu| p+||eu|.
! r/| qd ou d+, -5, +, +ud 2, |o| + |o|+| o| 2 r/|, |o| |rruuoupp|eed p+||eu|, ||e +pp|o.ed |e|reu | + r/| qd ou d+, -5,
0, 1, 2+, !, +ud !3, |o| + |o|+| o| +0 r/|. A||e|u+||.e |e|reu ||+| |+.e |eeu p|opoed |o| |rruuocorpe|eu| p+||eu| |uc|ude ||e+|reu| ou
d+, -5 +ud 0 W||| !-+ r/| qd |o| c+e ||or Eu|ope o| B|+/||, W||| ! r/| qd |o| c+e ||or A|||c+, +ud W||| 2-! r/| qd |o| c+e ||or
|ud|+.
!
|o| corre|c|+||, +.+||+||e + o| ||| W||||u.
-
\|||e|o|ue, W||c| | |e|+|oeu|c |u +u|r+|, |ou|d uo| |e ued |o ||e+| p|eu+u| Woreu. woreu o| c|||d|e+||u +e |ou|d ue e||ec||.e |||||
cou||o| du||u ||e+|reu| +ud |o| 2 rou|| ||e|e+||e|.
|
Adu|| do+e.
'0ukCE. Ad+p|ed ||or B| ne|W+|d|, |u A' |+uc| e| +| (ed). |c |:|- | ||-c| !-!:-, 1|| ed, C|+p. 205. |eW \o||, \cC|+Wn|||, 2003.
/oouo|
|+|+|||c p|o|o/o+u d|e+e c+ued |, |||ee pe
c|e o| ,cc
\ec|o|. |edu.||d |u
Ep|der|o|o,
Ceu||+| +ud 'ou|| Are||c+. :c
A|||c+. |:- c|-- |:- |!---
C||u|c+| ||ud|u
Acu|e. |uocu|+||ou ||e uodu|e
C||ou|c. C+|d|+c, +||o|u|e||u+| (C|), +ud ceu
||+| ue|.ou ,|er (C|') |u.o|.reu|
Cou|e. ro| |u|ec|ed pe|ou |er+|u o |o| |||e.
ne+||, C|, +ud C|' |u.o|.ereu| +oc|+|ed W|||
e||ou ro|||d||, +ud ro||+|||,
IkFAN0S0MIASIS |C|9. 03o.9

|C|0 . B5o
E||o|o,. :c
I|+ur||ou. :c depo||ed |u |ece o| |edu
.||d |u ou|o ||e ||u, eu|e| |o| .|+ ||e+|
|u ||u, rucou rer||+ue, o| coujuuc||.+e.
C|+or+ c+u occu| +| |uocu|+||ou ||e. C+u +|o
|e ||+ur|||ed |, ||+u|u|ou o| ||ood ||or
|u|ec|ed pe|ou, |, o|+u ||+up|+u|+||ou, ||or
ro||e| |o |e|u, |u |+|o|+|o|,.
||er|u+||ou. \|+ |,rp|+||c +ud ||ood||e+r |o
ruc|e.
Ceo|+p|,. Ceu||+| +ud 'ou|| Are||c+
||e.+|+uce. o-3 r||||ou pe|ou |u|ec|ed
C||u|c+| ||ud|u
Acu|e |u|ec||ou. r||d |e||||e |||ue
|ude|e|r|u+|e/+,rp|or+||c p|+e. u|p+|eu|
p+|+||er|+
',rp|or+||c c||ou|c |u|ec||ou.
C+|d|+c d|e+e
C| d|e+e
Cou|e. ro| |u|ec|ed pe|ou |er+|u o |o| |||e.
ne+|| +ud C| |u.o|.ereu| +oc|+|ed W||| e||ou
ro|||d||, +ud ro||+|||,
',, . C|++ d|e+e.
AMkICAN IkFAN0S0MIASIS (AI ) |C|9 . 03o.0

|C|0 . B51
Mucocutaoeous Fodos oI AI
Acute AT
Inotu|aon t|agoma An induiated aiea of
eiythema and swelling ( t|agoma ), at the
poital of entiy, occuiiing 7-14 days aftei
inoculation. May be accompanied by local
lymphadenopathy. Paiasites located within
leukocytes and cells of subcutaneous tis-
sues. These initial local signs aie followed by
malaise, fevei, anoiexia, and edema of the face
and lowei extiemities (Fig. 29-7).
Romana sgn Unilateial painless edema
of palpebiae and peiioculai tissues. Occuis
when conjunctiva is the poital of entiy.
Classic finding in acute AT.
Edema of face and lowei extiemities
TryanosomJes Moibillifoim, uiticaiifoim,
oi eiythematopolymoiphic eiuptions
Hemaogent or measat t|agomas
Nodule(s) caused by dissemination of infec-
tion. Haid, painful, wine-coloied nodules;
iaiely soften oi ulceiate.
ChrvnIc AT In the immunocompiomised host
(HIV/AIDS disease, oigan tiansplant iecipient)
Reactivation chagoma Nodule at inocula-
tion site.
te||u|s-mmt|ng |aque.
SSIMIC FIN0INCS 0F AI
Acute AI
Geneialized lymphadenopathy
Hepatosplenomegaly
Seveie myocaiditis may occui in acute AT;
most deaths aie due to heait failuie.
Chrooc AI
Heait (ihythm distuibances, caidiomyopathy,
and thiomboembolism)
GI tiact (megaesophagus, megacolon)
0IACN0SIS
Acute AI Detect paiasites in blood
Chrooc AI Detect specific antibodies
E||o|o,. corp|e\ o| |:-
| c|-- c+ue we| A|||c+u |eep|u
|c|ue
| |!--- c+ue E+| A|||c+u |eep|u
|c|ue
I|+ur||ou.
\ec|o|. |e|e |||e.
I|+ur||ou du||u |ur+u ||ood re+| ||or
|u|ec|ed +||.+.
|||r+|, |ee|.o||
we| A|||c+u |eep|u |c|ue. |ur+u
E+| A|||c+u |eep|u |c|ue. Au|e|ope +ud
c+|||e
Ep|der|o|o,
'u|'+|+|+u A|||c+, !o couu|||e.
we| A|||c+. |.o|, Co+|, C|+d, Ceu||+| A|||c+u
kepu|||c, |u|+| popu|+||ou
E+| A|||c+. 'ud+u, Wo||e| |u W||d +|e+, |u|+|
popu|+||ou, |ou||| |u +re p+||
> oo r||||ou pe|ou |u|ec|ed
nAI |u ||+.e|e|. uu+||, E+| A|||c+u ||,p+uoor|+|
C||u|c+| ||ud|u
Acu|e
C||ou|c. ||o|e|.e ueu|o|o|c |rp+||reu|, de+||
Cou|e. ro| |ue\o|+||, p|o|e|.e
',, . '|eep|u |c|ue
hMAN AFkICAN IkFAN0S0MIASIS (hAI) |C|9 . 03o.5

|C|0 . B5o
CIINICAI FIN0INCS 0F hAI
Acute hAI: Stae I 0sease
Tryanosoma| t|antre Appeais in some
patients at inoculation site (Fig. 29-7); pain-
ful; 7-14 days aftei tsetse-fly bite. Occuis
moie commonly in tiaveleis (e.g., to game
paiks) than in Afiicans. Typically 2-5 cm, in-
duiated; may ulceiate; iesolved in few weeks.
Paiasites can be seen in fluid expiessed fiom
chancie and buffy coat.
Hemo|ym|at sage Maiked by the onset
of fevei, aithialgias, malaise, localized facial
edema, and modeiate splenomegaly. Lym-
phadenopathy is piominent in T. |. gam|ense
tiypanosomiasis.
Matu|ar-au|ar ras| Occuis on the tiunk.
Prurus
Vner|oom sgn Enlaigement of the
nodes of the posteiioi ceivical tiiangle; ceivi-
cal nodes also enlaiged.
D[[erena| Jagnoss Acute HIV/AIDS in-
fection, malaiia, typhoid fevei.
Course Moie iapid in East Afiican type.
Touiist with T. |. r|oJesense disease may
develop systemic signs of infection (fevei,
malaise, headache) neai the end of tiip.
Chrooc hAI: Stae II 0sease
CNS nason Chaiacteiized by insidious
development of piotean neuiologic symp-
toms. Piogiessive indiffeience and daytime
somnolence develops (hence the designation
sleeping sickness").
CarJat Jsease East Afiican type may de-
velop aiihythmias and congestive heait fail-
uie befoie CNS disease develops.
Dagnoss Detection of paiasite in chancie,
lymph node, blood, bone maiiow.
FICk 29-T humao ast AIrcao trypaooso-
mass: trypaoosoma| chaocre A |+||oW u|ce|+||ou
W+ p|eeu| ou ||e do|ur o| ||e |e|| |oo|, u||ouuded
|, + ||u ||+| cou|+|ued |u||+e +ud ||+| W+, |u |u|u,
u||ouuded |, +uo||e| ||u, c|+|+c|e||/ed |, .|o|+ceou
e|,||er+ +ud |udu|+||ou, ||e eu|||e |e|ou, W||c| W+
+pp|o\|r+|e|, 5 cr |u d|+re|e|, W+ p+|u|u|. A r+cu|+|
e\+u||er W+ p|eeu| ou ||e ||uu|. I|e p+||eu| W+
+ ||+.e|e| |o 'ou|| A|||c+. ,cc |:- W+
|deu||||ed |u +u +p||+|e ||or ||e u|ce|. (Cou||e, o|
EdW+|d I. k,+u, \|. | Eu| l \ed !+o. 20o9, 2002,
W||| pe|r||ou.)
CIAN0S AM8IASIS AN0 ACANIhAM8IASIS
Are||+| | c+ued |, ||c-|c |||,|:c ,
W||c| |u|ec| ||e C| ||+c| +ud |+|e|, ||u
|uc|deuce. 0 o| Wo||d popu|+||ou |u|ec|ed W|||
||c-|c.
\+jo|||, o| |u|ec||ou c+ued |, uou|u.+|.e |
!c
0 o| ||oe co|ou|/ed W||| | |||,|:c
de.e|op +re||c co||||.
\o|e p|e.+|eu| |u ||op|c +ud |u |u|+| +|e+, |u+d
equ+|e +u||+||ou +ud c|oWd|u.
'||u |u.o|.ereu| | +oc|+|ed W||| r+|uu|||||ou
+ud |rruuocorp|or|e (n|\/A||', o||d o|+u
||+up|+u|+||ou)
C||u|c+| ||ud|u.
CA |e|u + +u |udu|+|ed pu|u|e ||+| e.o|.e
|o + p+|u|u| |+ed u|ce|, |ou|re|||u +ud
co.e|ed W||| pu o| uec|o||c de||| (||. 293).
uu+||, + couequeuce o| +u uude||,|u +re||c
+|ce |u.+d|u ||e ||u.
I,p|c+| ||e +|e ||e pe||+u+| +|e+ (e\|eu|ou o|
|ro|ec|+| |u.o|.ereu|) (||. 293) o| +|dor|
u+| W+|| (d|+|u|u |uu ||or ||.e| o| co|ou).
|eu| o| .u|.+ r+, |ecore |u|ec|ed du||u
|u|e|cou|e.
'u||c+| Wouud |u|ec||ou r+, |o||oW |ero.+|
o| |ep+||c o| +|dor|u+| +|ce.
kero|e u|ce| (e.., |+ce) r+, |eu|| ||or +u
|o|uocu|+||ou.
w|||ou| ||e+|reu|, CA p|o|e|.e|, eu|+|e.
CIAN0S AM8IASIS |C|9 . 00o.o
|C|0 . A0o.1
Cu|+ueou +c+u||+re||+| | +u |u|ec||ou c+ued
|, ||ee||.|u 1:c||c-|c .
C||u|c+| ||ud|u.
|||r+|, cu|+ueou +c+u||+re||+|
0ccu| +| ||e o| ||+ur+ u|+|ued |u +qu+||c
eu.||oureu| (||e+r, poud, W|rr|u
poo|).
|e|ou |e|u + |udu|+|ed |ed/.|o|+ceou deep
uodu|e o| |+|e pu|u|e ||+| oou u|ce|+|e.
||er|u+|ed cu|+ueou +c+u||+re||+|
0ccu| |u n|\/A||' d|e+e +ud o||d o|+u
||+up|+u| |ec|p|eu|
||er|u+|e ||or u++|/|uu co|ou|/+||ou.
||eeu| W||| ru|||p|e o|| |ed uodu|e ||+|
u|ce|+|e
CIAN0S ACANIhAM8IASIS |C|9 . 00o.o
|C|0 . A0o.1
FICk 29-8 Cutaoeous ameb-
ass: peroeum |e||ue+|/pe||+u+|
u|ce| |u + p+||eu| W||| |ec|+| +re||+|.
896
S E C I | 0 N 5 0
'e\u+||, ||+ur|||ed |u|ec||ou ('I|), c+ued |,
+ ||o+d |+ue o| p+||oeu (I+||e !0), |+.e +
||| p|,|c+| +ud p,c|ooc|+| ro|||d||,.
I|e ,ud|ore c+ued |, ||ee p+||oeu +||ec|
|o|| ||e e\u+||, +c||.e coup|e +ud ueou+|e |o|u
|o +u |u|ec|ed ro||e| (I+||e !02).
B+c|e||+| 'I| uc| + ouo|||e+, ,p||||, c|+u
c|o|d, douo.+uo|, +ud |,rp|o|+uu|or+
.eue|eur (|C\) c+u e+||, |e cu|ed W||| +u||r|
c|o||+| ||e|+p,.
\||+| 'I|, uc| + ||oe c+ued |, n|\/A||',
|ur+u p+p|||or+.||u (n|\), +ud |e|pe
|rp|e\ .||u 2 (n'\2), +|e c||ou|c |u|ec||ou,
c|+|+c|e||/ed |, p|o|oued .||+| |edd|u +ud
oppo||uu||, |o| |u|ec||u + e\u+| p+||ue|, c+u
ou|, |e upp|eed |u| uo| cu|ed |, +u||.||+|
||e|+p,.
|e+||, +|| e\u+||, +c||.e |ud|.|du+| +|e +| ||| |o|
||ee .||+| 'I|. n|\ pe||| |u ||e +uoeu||+| ru
co+ |o| rou||, ,e+|, o| dec+de +||e| p||r+|,
|u|ec||ou. n'\2 |u|ec||ou | c||ou|c +ud |||e|ou.
||e.eu||ou o||e| ||e |e| +pp|o+c| |o r+u+|u
'I|.
|u de.e|op|u u+||ou, W|e|e 'I| +|e ro|e
corrou, |+c| o| |uud |o| |e+||| c+|e o||eu ||r||
de|ec||ou +ud ||e+|reu| o| 'I| + We|| + |rru
u|/+||ou.
uu||e+|ed ,p|||| c+u |e + |||e|ou |u|ec||ou W|||
|ou|e|r ro|||d||,.
I|+ur||ou o| n|\/A||' |o ueou+|e occu|
corrou|, |u de.e|op|u u+||ou, W|e|e ||e
p|e.+|euce o| |u|ec||ou | |||, +u|||e||o.||+| ||e+|
reu| o| ro||e| +ud ueou+|e r+||ed|, |educe
ueou+|+| |u|ec||ou.
I|+ur||ou o| n'\ |+ ro|e |rred|+|e e||ec|
ou ||e ueou+|e, W|o | ro|e ucep||||e |o +cu|e
.|ce|+| |u|ec||ou.
I|+ur||ou o| n|\ |u|ec||ou |o ||e ueou+|e c+u
|eu|| |u +uoeu||+| coud,|or+, +ud, |+|e| |u |||e,
|ep||+|o|, p+p|||or+|o|.
',, . 'e\u+||, ||+ur|||ed d|e+e, eu||+|
|u|ec||ou d|e+e, .eue|e+| d|e+e (\|)
SXAII
IkANSMIII0 INFCII0NS
IA8I 30-1 Se\ua||y Iransmissib|e Pathoens and Associated 0isease Syndromes
Pathogeo Assoc|ated 0|sease or Syodrome
Bacteria
|--c |-c- u|e||||||, ep|d|d,r|||, p|oc||||, ce|.|c|||, eudore|||||, +|p|u|||,
pe|||ep+||||, |+|||o||u|||, p|+|,u|||, coujuuc||.|||, p|epu|e||+| .+|u|||,
p|o|+||||, +cceo|, |+ud |u|ec||ou, d|er|u+|ed ouococc+| |u|ec||ou
(|C|), c|o||o+ru|ou|||, p|er+|u|e |up|u|e o| rer||+ue, p|er+|u|e
de||.e|,, +ru|o||c |u|ec||ou ,ud|ore
C||c,!c |c:|c| A|| o| ||e +|o.e e\cep| |C|, p|u o|||| red|+, |||u|||, +ud pueurou|+ |u
|u|+u|, +ud |e+c||.e +||||||| (ke||e|) ,ud|ore
|-c|cc -c|,|: |ououococc+| u|e|||||| (|Cu)
!,:|cc -|c| (!) |ououococc+| u|e||||||
! | |o|p+||ur |e.e|, +|p|u||| (!)
SCII0N 30 'E/uA||\ IkA|'\|IIE| |||ECI|0|' 89T
Pathogeo Assoc|ated 0|sease or Syodrome
--c c||! ',p||||
Cc!--||c .cc| B+c|e||+| ('uoupec|||c) .+|uo| (|u coujuuc||ou W||| !,:|cc
| +ud .+|u+| +u+e|o|e, uc| + !||: pp.)
!||: :| B+c|e||+| .+|uo|
! |- B+c|e||+| .+|uo|
|c-|| !:-, C|+uc|o|d
Cc|,c||c:|- c|c| |ouo.+uo| (|+uu|or+ |uu|u+|e)
'|-||c '||e||o| |u reu W|o |+.e e\ W||| reu (\'\)
Cc,||c:|- Eu|e||||, p|oc|oco|||| |u \'\
|-|:|c:|- :c-! (!) ||oc|oco||||, de|r+||||, |+c|e|er|+ |u A||'
| |--|c- (!) ||oc|oco||||, de|r+||||, |+c|e|er|+ |u A||'
Viruses
n|\ +ud 2 n|\/A||'
n'\ |,pe +ud 2 |u|||+| +ud |ecu||eu| eu||+| |e|pe, +ep||c reu|u|||, ueou+|+| |e|pe
n|\ Coud,|or+|+ +cur|u+|+, |+|,ue+| p+p|||or+, |u||+ep|||e||+| ueop|+|+ +ud
c+|c|uor+ o| ||e ce|.|\, .+|u+, .u|.+, +uu, peu|
nep+|||| A .||u (nA\) Acu|e |ep+|||| A
nep+|||| B .||u (nB\) Acu|e |ep+|||| B, c||ou|c |ep+|||| B, |ep+|oce||u|+| c+|c|uor+, po|,+||e||||
uodo+, c||ou|c rer||+uou |ore|u|ouep|||||, r|\ed c|,o|o|u||uer|+
(!), po|,r,+||+ ||eur+||c+ (!)
nep+|||| C .||u (nC\) Acu|e |ep+|||| C, c||ou|c |ep+|||| C, |ep+|oce||u|+| c+|c|uor+, r|\ed
c|,o|o|u||uer|+, c||ou|c |ore|u|ouep|||||
C,|ore+|o.||u (C\\) ne|e|op|||ue+||.e |u|ec||ou rououuc|eo|, coueu||+| C\\ |u|ec||ou
W||| |o ||||| de|ec| +ud |u|+u| ro||+|||,, cou|||.e |rp+||reu| (e..,
reu|+| |e|+|d+||ou, euo||ueu|+| de+|ue), p|o|e+u r+u||e|+||ou |u ||e
|rruuoupp|eed |o|
\o||ucur cou|+|our Ceu||+| ro||ucur cou|+|our
.||u (\C\)
nur+u I ce|| |,rp|o||op||c nur+u I ce|| |eu|er|+ o| |,rp|or+, ||op|c+| p+||c p+|+p+|e|
.||u (nI|\|)
nur+u |e|pe .||u |,pe 3 (nn\3) K+po| +|cor+, |od, c+.||, |,rp|or+, ru|||ceu|||c C+||er+u d|e+e
Prototoa
:|c .cc| \+|u+| |||c|orou|+|, |Cu
Eu|+roe|+ |||o|,||c+ Are||+| |u \'\
Cc!c |c||c C|+|d|+| |u \'\
|uni
Cc!!c c||:c \u|.o.+|u|||, |+|+u|||
Ectoparasites
|||| | |u||c ||ce |u|e|+||ou
'c:|- :c|- 'c+||e
'0ukCE'. Ad+p|ed ||or w C+|e, l|, KK no|re, |u l\ |+|, kB w+||+ce (ed). !c:,|-c|c| |||: |-c||| c! |-.-|.- !-!:-, +||
ed. |o|W+||, CI, App|e|ou ? |+ue, 993, pp !1-55, +ud KK. no|re, nn n+ud||e|d, |u A' |+uc| e| +| (ed). |c |:|- | ||-c|
!-!:-, +|| ed. |eW \o||, \cC|+Wn|||, 993.
IA8I 30-2 Se|ected Syndromes and Comp|ications of Se\ua||y Iransmitted Pathoens
Syodrome or 0omp||cat|oo Assoc|ated Sex0a||y Traosm|tted Pathogeo
|n men
n|\/A||' n|\ |,pe +ud 2
u|e|||||| |--c |-c- C||c,!c |c:|c| n'\, |-c|cc
-c|,|: (:) !,:|cc -|c| .cc|
Ep|d|d,r||| C |c:|c| | |-c-
|u|e||u+| |u|ec||ou
||oc|||| | |-c- n'\, C |c:|c|
||oc|oco|||| o| eu|e|oco|||| Cc,||c:|- pp. '|-||c ||c-|c |||,|:c (:) |-|:|c:|- pp.
Eu|e|||| Cc!c |c||c
|n women
n|\/A||' n|\ , n|\ 2
|oWe| eu||ou||u+|, ||+c|
|u|ec||ou
\u|.||| Cc!!c c||:c n'\
\+|u||| :|c .cc| C c||:c
\+|uo| Cc!--||c .cc| !||: pp., o||e| +u+e|o|e !,:|cc |
Ce|.|c||| | |-c- C |c:|c| n'\
|e|.|c |u||+rr+|o|, d|e+e | |-c- C |c:|c| ! | +u+e|o|e, |oup B ||ep|ococcu,
|u|e||||||,
|o|+|p|u|||, po|o||e|||c+|, | |-c- C |c:|c| ! | (:)
po|+|o|||ou
||eu+uc, ro|||d||, 'e.e|+| 'I| |rp||c+|ed |u oue o| ro|e o| ||ee coud|||ou
C|o||o+ru|ou|||, +ru|o||c ||u|d
|u|ec||ou, p|er+|u|||,,
p|er+|u|e |up|u|e o|
rer||+ue, p|e|e|r
de||.e|,, po|p+||ur
eudore|||||, ec|op|c
p|eu+uc,
|n men and women
k+|e eue|+||/ed, |oc+||/ed c||!
|eop|+r. Ce|.|c+|, .u|.+|,
.+|u+|, +u+|, +ud peu||e, n|\
|u||+ep|||e||+| ueop|+|+,
c+|c|uor+
nep+|oce||u|+| c+|c|uor+ nB\, nC\
K+po| +|cor+, |od, nn\3
c+.||, |,rp|or+,
C+||er+u d|e+e
I ce|| |,rp|or+/|eu|er|+ nI|\|
Ceu||+| u|ce|+||ou n'\, c||! |c-|| !:-, Cc|,c||c:|- c|c|
C |c:|c| (|C\ ||+|u)
Acu|e +||||||| W||| u|oeu||+| | |-c- C |c:|c| '|-||c pp Cc,||c:|- pp.
o| |u|e||u+| |u|ec||ou
nep+|||| nA\, nB\, nC\, C\\, --c c||!
Auoeu||+| W+|| n|\ (!0 eu||+| |,pe)
SCII0N 30 'E/uA||\ IkA|'\|IIE| |||ECI|0|' 899
Peisons being evaluated foi STIs should have
a cultuie foi gonoiihea and seiotesting foi
HIV/AIDS and syphilis.
Seiologic testing foi HPV infections is not
available.
Type-specific antibodies to glycopiotein
(g)G1 and (g)G2 detect and diffeientiate past
HSV-1 and HSV-2 infections.
Piimaiy HSV infection can be documented
by demonstiation of seioconveision.
The development of nucleic acid amplifica-
tion tests heialded a new eia in sensitive and
diagnostic pioceduies foi STIs.
MANACMNI
The most effective way to pievent sexual tians-
mission of STIs is to avoid sexual inteicouise
with an infected paitnei. Ideally, both new
paitneis should get tested foi STIs befoie ini-
tiating sexual inteicouise. If a peison chooses
to have sexual inteicouise with a paitnei whose
infection status is unknown oi who is infected
with HIV/AIDS oi anothei STI, a new condom
should be used foi each act of inteicouise. Con-
dom use is not completely piotective against
acquisition of STI because of the piesence of
pathogen outside the piotected skin oi condom
Syodrome or 0omp||cat|oo Assoc|ated Sex0a||y Traosm|tted Pathogeo
\o||ucur cou|+|our \C\
Ec|op+|+||e |u|e|+||ou 'c:|- :c|- |||| |
\ououuc|eo| ,ud|ore C\\, n|\/A||', Ep|e|uB+|| \||u (EB\)
I|op|c+| p+||c p+|+p+|e| nI|\|
|n neonates and infants
|eou+|+| ,|er|c |u|ec||ou, C,|ore+|o.||u, n'\, c||!, n|\/A||'
W||| po|eu||+| cou|||.e
|rp+||reu|, de+|ue, de+||
Coujuuc||.||| C |c:|c| | |-c-
|ueurou|+, (!) c||ou|c C |c:|c| | -c|,|: (:)
pu|rou+|, d|e+e
0|||| red|+ C |c:|c|
'ep|, reu|u||| C|oup B ||ep|ococcu
|+|,ue+| p+p|||or+|o| n|\
|0IE. |o| e+c| o| ||e +|o.e ,ud|ore, ore c+e c+uuo| ,e| |e +c|||ed |o +u, c+ue +ud ru| cu||eu||, |e cou|de|ed |d|op+|||c. '! |ud|c+|e
+ po|||e +oc|+|ed ,ud|ore.
'0ukCE'. Ad+p|ed ||or w C+|e, l|, KK no|re, |u l\ |+|, kB w+||+ce (ed). !c:,|-c|c| |||: |-c||| c! |-.-|.- !-!:- +||
ed. |o|W+||, CI, App|e|ou ? |+ue, 993, pp !1-55, +ud KK. no|re, nn n+ud||e|d, |u A' |+uc| e| +| (ed). |c |:|- | ||-c|
!-!:- 1ed. |eW \o||, \cC|+Wn|||, 993.
bieakage. HPV oi HSV infections often occui
at the base of the penis oi pubic aiea, which aie
not piotected by condoms. Chionic suppiessive
theiapy can ieduce tiansmission of HSV-2 (and
HSV-1). Piospects foi development of a vaccine
foi HSV-2 aie excellent. An effective HPV vac-
cine is now available and is iecommended foi
adolescent females to ieduce the incidence of
anogenital canceis. Immunization foi hepatitis A
and B is advised to pievent tiansmission of these
viial infections duiing inteicouise.
Foi updated infoimation about sexu-
ally tiansmitted infections, see CDC website:
|.//www.tJt.go/STD/
Maoaemeot oI Fateots Who have Ceota|
|cers In the United States, the majoiity of
young, sexually active patients who have genital
ulceis have eithei genital heipes, syphilis, oi
chancioid. The fiequency of each condition dif-
feis by geogiaphic aiea and patient population;
howevei, genital heipes is the most pievalent of
these diseases. Moie than one of these diseases
can be piesent in a patient who has genital
ulceis. All thiee of these diseases have been as-
sociated with an incieased iisk foi HIV/AIDS
infection. Not all genital ulceis aie caused by
sexually tiansmitted infections.
A diagnosis based only on the patient`s medi-
cal histoiy and physical examination fiequently
is inaccuiate. Theiefoie, all patients who have
genital ulceis should be evaluated with a
seiologic test foi syphilis and a diagnostic
evaluation foi genital heipes; in settings wheie
chancioid is pievalent, an H. Jutrey infection
should also be consideied. Specific tests foi
evaluation of genital ulceis include (1) syphilis
seiology and eithei daik-field examination oi
diiect immu nofluoiescence test foi T. a||Jum ;
(2) cultuie oi antigen test foi HSV; and (3) cul-
tuie foi H. Jutrey .
Type-specific seiology foi HSV-2 might
be helpful in identifying peisons with genital
heipes (see Genital Heipes, Type-Specific Se-
iologic Tests). Biopsy of genital ulceis might be
helpful in identifying the cause of ulceis that aie
unusual oi that do not iespond to initial thei-
apy. HIV/AIDS testing should be peifoimed on
all patients who have genital ulceis caused by
T. a||Jum oi H. Jutrey , and should be stiongly
to|oy
HPV is a DNA papovaviius that multiplies in
the nuclei of infected epithelial cells (see Sec-
tion 27).
Moie than 20 types of HPV can infect the
genital tiact: types 6, 11 most commonly; also
types 16, 18, 31, 33 (see Table 27-2).
Types 16, 18, 31, 33, and 35 aie stiongly as-
sociated with anogenital dysplasia and caici-
noma.
In individuals with multiple sexual paitneis,
subclinical infection with multiple HPV types
is common.
\uco+| n|\ |u|ec||ou +|e ||e ro| corrou
'I| eeu |, ||e de|r+|o|o||.
0u|, -2 o| n|\|u|ec|ed |ud|.|du+| |+.e +u,
.||||, de|ec|+||e c||u|c+| |e|ou.
n|\ p|eeu| |u ||e ||||| c+u+| c+u |e ||+ur|||ed
|o + ueW|o|u du||u .+|u+| de||.e|, +ud c+u
c+ue
E\|e|u+| eu||+| W+|| (ECw)
kep||+|o|, p+p|||or+|o|
w+||. |+|e|, .||||e p+pu|e |o uodu|e |o cou||u
eu| r+e occu|||u ou.
Auoeu||+|. ||u o| ruco+
0|+| ruco+
n|\ d,p|+|+ o| +uoeu||+| +ud o|+| ||u +ud
ruco+ |+u|u ||or.
\||d |o e.e|e |o qu+rou ce|| c+|c|uor+
('CC) |u ||u ('CC|')
|u.+|.e 'CC c+u +||e W||||u 'CC|'
\o| corrou|, |u ce|.|\, +u+| c+u+|.
',, Coud,|or+|+ +cur|u+|+, e\|e|u+| eu||+|
W+||, +uoeu||+| W+||, .eue|e+| W+||.
|C|9 . 019.+
|C|0 . B91.1
hMAN FAFIII0MAvIkS: MC0SAI INFCII0NS
consideied foi those who have genital ulceis
caused by HSV (see Diagnostic Consideiations,
sections, Syphilis, Chancioid, and Genital Hei-
pes Simplex Viius).
Health caie piovideis fiequently must tieat
patients befoie test iesults aie available because
eaily tieatment decieases the possibility of
ongoing tiansmission and because successful
tieatment of genital heipes depends on piompt
initiation of theiapy. The clinician should tieat
foi the diagnosis consideied most likely, on the
basis of clinical piesentation and epidemiologic
ciicumstances. In some instances, tieatment
must be initiated foi additional conditions
because of diagnostic unceitainty. Even aftei
complete diagnostic evaluation, at least 25%
of patients who have genital ulceis have no
laboiatoiy-confiimed diagnosis.
Ae oI 0oset Young, sexually active adults.
ksk Factors Ior Acquro hFv IoIectoo
Numbei of sexual paitneis/fiequency of sex-
ual inteicouise
Sexual paitnei with EGW
Sexual paitnei`s numbei of sexual paitneis,
infection with othei STIs.
Iraosmssoo
Thiough sexual contact: genital-genital, oial-
genital, genital-anal.
Micioabiasions occui on epithelial suiface
allowing viiions fiom infected paitnei to
gain access to basal cell layei of noninfected
paitnei.
Digital tiansmission of nongenital waits
piobably accounts foi few cases of EGW.
Duiing deliveiy, motheis with anogenital
waits can tiansmit HPV to neonate, iesult-
ing in EGW and laiyngeal papillomatosis in
childien.
Iocdeoce Most sexually active individuals aie
subclinically infected with HPV; most HPV
infections aie asymptomatic, subclinical, oi
uniecognized. 1% of sexually active adults
(15-19 yeais of age) develop EGW. Incieased
manyfold duiing the past two decades.
Fsychosexua| Impact oI Ceota| Warts Public
awaieness of genital HPV infections is low. Few
patients aie awaie of the iole of HPV in ano-
genital cancei. Diagnosis of genital waits may
iesult in feais about tiansmission and iecui-
ience, sexual lifestyle changes (abstinence, cau-
tion, condoms), depiession oi low self-esteem,
ielationships becoming stiained and/oi bieak-
ing down, anxiety ielated to paitnei disclosuie.
Public awaieness of genital HPV infection is
incieasing because of diug adveitisements in
piint and television.
FAIh0CNSIS
Low-iisk" and high-iisk" HPV types both
cause EGW.
HPV infection may peisist foi yeais in a doi-
mant state and becomes infectious inteimit-
tently.
Exophytic waits aie piobably moie infectious
than subclinical infection.
Immunosuresson may iesult in new exten-
sive HPV lesions, pooi iesponse to tieatment,
incieased multifocal intiaepithelial neoplasia.
Immunosuppiessed ienal tiansplant iecipi-
ents have a 17-fold gieatei incidence of geni-
tal HPV infection.
All HPV types ieplicate exclusively in host`s
cell nucleus.
In benign HPV-associated lesions, HPV exists
as a plasmid in cellulai cytoplasm, ieplicating
extiachiomosomally.
In malignant HPV-associated lesions, HPV
integiates into host`s chiomosome, following
a bieak in the viial genome (aiound E1/E2
iegion).
E1 and E2 function is deiegulated, iesulting in
cellulai tiansfoimation.
XIkNAI CNIIAI WAkIS
Sko Symptoms
Usually asymptomatic, except foi cosmetic
appeaiance.
Anxiety of having STI.
Itching, buining, bleeding, vaginal oi uiethial
dischaige, dyspaieunia.
Obstiuction if laige mass is uncommon.
Foui clinical types of genital waits occui:
Small papulai (Fig. 30-1)
Cauliflowei-floiet (acuminate oi pointed)
lesions (Figs. 30-2 to 30-4)
Keiatotic waits (Fig. 30-6)
Flat-topped papules/plaques (most com-
mon on ceivix) (Fig. 30-4).
Coloi: skin-coloied, pink, ied, tan, biown.
Solitaiy, scatteied, and isolated, oi foim volu-
minous confluent masses.
In immunocompiomised individuals, lesions
may be huge. (Fig. 30-6)
Sites of piedilection
Ma|e : Fienulum, coiona, glans penis, pie-
puce, shaft (Figs. 30-1, 30-2, 30-6), scio-
tum.
Fema|e : Labia, clitoiis, peiiuiethial aiea,
peiineum, vagina, ceivix (flat lesions) (Fig.
30-4).
Bo| sexes : Peiineal, peiianal (Fig. 30-5),
anal canal, iectal; uiethial meatus, uiethia,
bladdei; oiophaiynx.
Iaryoea| Fap||omas
Relatively uncommon; associated with HPV-6
and -11.
Aiise most commonly on tiue vocal coids of
laiynx.
Age: childien <5 yeais of age; adults >20 yeais
of age.
Risk of SCCIS and invasive SCC

Fapu|ar[Nodu|ar xteroa| Ceota| Iesoos
Noimal anatomy (e.g., sebaceous glands, peaily
penile papules, vestibulai papillae), squamous
intiaepithelial lesions, SCCIS, invasive SCC,
benign neoplasms (moles, seboiiheic keiatoses,
skin tags, pilai cyst, angiokeiatoma), inflamma-
toiy deimatoses (lichen nitidus, lichen planus),
molluscum contagiosum, condylomata lata, fol-
liculitis, scabietic nodules.
Fap Smear All women should be encouiaged
to have an annual Pap smeai since HPV is the
majoi etiologic agent in pathogenesis foi cancei
of the ceivix. Anal Pap test with a ceivical biush
and fixative solution is helpful in detecting anal
dysplasia.
0ermatopatho|oy Biopsy is indicated if diag-
nosis is unceitain; the lesions do not iespond
to standaid theiapy; the lesions woisen duiing
FICk 30-1 Fapu|ar warts: peos A 2!,e+|o|d r+|e W||| peu||e |e|ou |o| o rou||. \u|||p|e
||uco|o|ed p+pu|e ou ||e peu| +ud c|o|ur.
FICk 30-2 Coody|omata acumoata: peos A 25,e+|o|d r+|e W||| !rou|| |||o|, o| peu||e |e|ou.
\u|||p|e c+u||||oWe| ||o|e|||e p+pu|e ou peu||e |+|| +ud |o|e||u.
FICk 30-3 Coody|omata acu-
moata: vu|va \u|||p|e, p|u|||oWu,
o|| p+pu|e ou ||e |+||+.
FICk 30-4 Coody|omata acumoata:
uteroe cervx '|+|p|, der+|c+|ed, W|||
||, ||+| p|+que |ecor|u cou||ueu| +|ouud
||e ce|.|\.
theiapy; the patient is immunocompiomised;
waits aie pigmented, induiated, fixed, and/oi
ulceiated; all suspect ceivical lesions. Indicated
in some cases to confiim diagnosis and/oi iule
out SCCIS oi invasive SCC.
0etectoo oI hFv 0NA Piesence of HPV DNA
and specific HPV types can be deteimined on
smeais and lesional biopsy specimens by in situ
hybiidization. Howevei, no data suppoit the
use of type-specific HPV nucleic acid tests in
the ioutine diagnosis oi management of visible
genital waits.
Sero|oy Occuiience of genital waits is a
maikei of unsafe sexual piactices. Seiologic tests
foi syphilis should be obtained on all patients to
iule out coinfection with T. a||Jum , anJ a||
aens o[[ereJ HIV/IDS esng .
0IACN0SIS
Clinical diagnosis, occasionally confiimed by
biopsy.
C0kS AN0 Fk0CN0SIS
HPV is highly infectious, with an incubation
peiiod of 3 weeks to 8 months.
Most HPV-infected individuals who develop
EGW do so 2-3 months aftei becoming
infected.
If left untieated, genital waits may iesolve
on theii own, iemain unchanged, oi giow.
In placebo-tieated cases, genital waits cleai
spontaneously in 20-30% of patients within
3 months.
Aftei iegiession, su|t|nta| n[eton may er-
ss [or |[e . Recuiience may occui in individu-
als with noimal immune function as well as
with immunocompiomise.
Condylomata may iecui due to peisistence of
latent HPV in noimal-appeaiing peiilesional
skin (see Tiansmission," above). Recuiiences
moie commonly iesult fiom ieactivation of
subclinical infection than fiom ieinfection by
a sex paitnei.
In piegnancy, genital waits may inciease
in size and numbei, show incieased vagi-
nal involvement, and have an incieased iate
of secondaiy bacteiial infection of vaginal
waits.
Childien deliveied vaginally of motheis with
genital HPV infection aie at iisk foi develop-
ing iecuiient iespiiatoiy papillomatosis in
latei life.
The majoi significance of HPV infection is its
oncogenicity.
HPV types 16, 18, 31, and 33 aie the majoi
etiologic factois foi in situ and invasive SCC:
Ceivix
Exteinal genitalia: vulva and penis
Anus and peiineum: homosexual/bisexual
males, females
Tieatment of exteinal genital waits is not
likely to influence the development of ceivi-
cal cancei. The impoitance of the annual Pap
test should be stiessed foi women with genital
waits.
MANACMNI
Freveotoo
Use of condoms ieduces tiansmission to un-
infected sex paitneis.
Goal of tieatment is iemoval of exophytic
waits and amelioiation of signs and symp-
toms-not eiadication of HPV.
No theiapy has been shown to eiadicate HPV.
Tieatment is moie successful if waits aie
small and have been piesent foi <1 yeai.
Risk of tiansmission might be ieduced by
debulking" genital waits.
Selection of tieatment should be guided by
piefeience of patient-expensive theiapies,
toxic theiapies, and pioceduies that iesult in
scaiiing avoided.
A vaccine that helps piotect against foui types
of HPV is now available foi young women
fiom ages 9 to 26 yeais.
Iodcatoos Ior Iherapy
Cosmetic
Reduce tiansmissibility
Piovide ielief of symptoms
Impiove self-esteem.
Frmary Coa| oI Ireato vsb|e Ceota| Warts
Removal of symptomatic waits. Tieatment
can induce wait-fiee peiiods in most pa-
tients.
Genital waits aie often asymptomatic.
No evidence indicates that cuiiently available
tieatments eiadicate oi affect the natuial his-
toiy of HPV infection.
Removal of waits may oi may not deciease
infectivity.
If untieated, visible genital waits may iesolve
on theii own, iemain unchanged, oi inciease
in size and numbei.

No evidence indicates that tieatment of vis-
ible waits affects the development of ceivical
oi anal cancei.
Subc|oca| Ceota| hFv IoIectoo (wthout
xophytc Warts) Subclinical genital HPV in-
fection is much moie common than exophytic
waits among both men and women. Infection is
often indiiectly diagnosed on the ceivix by Pap
smeai, colposcopy, oi biopsy and on the penis,
vulva, and othei genital skin by the appeaiance
of white aieas aftei application of acetic acid.
Tieatment is not indicated.
xteroa| Ceota|[Feraoa| Warts
PutIent-App|Ied Agents
ImqumoJ, 5% tream Mechanism of action
is via local cytokine ielease (inteifeion,
tumoi neciosis factoi, inteileukin). No
diiect antiviial activity. The cieam, which
is supplied in single-dose packets, is ap-
plied to the involved site by the patient,
thiee times pei week, usually at bedtime.
Some patients expeiience local cytokine
deimatitis (Fig. 30-7B). Tieatment duia-
tion up to 16 weeks.
PoJo[|ox 0.5% solution and gel. A puiified
and stable piepaiation of the active agent
in podophyllin. Solution applied with a
cotton swab and gel with a fingei to con-
dylomata and/oi site involved (including
noimal-appeaiing skin between lesions)
FICk 30-5 Coody|omata acumoata: aous A o0,e+|o|d r+|e W||| weeue| |+uu|or+|o|, ||e+|ed
W||| +/+|||op||ue +ud p|edu|oue W||| |eceu| oue| o| pe||+u+| |e|ou. \u|||p|e p|u||+u cou||ueu| ||o|e||||e
|e|ou u||ouud|u ||e +uu.
FICk 30-6 keratotc exteroa| eota| warts
(CW): ma|e A +o,e+|o|d r+|e W||| |e|ou +|
||e |+e o| peu| |o| e.e|+| ,e+|. A |e|+|o||c |uro|
+| ||e |+e o| ||e peu| +dj+ceu| |o ||e c|o|ur.
|e|ou+| ||op, |epo||ed ECw. \e||ucou c+|c|uor+
W+ + couce|u.
twice daily foi 3 days, followed by 4 days
of no theiapy. This cycle may be iepeated
as necessaiy foi a total of foui cycles.
Total aiea of tieatment should not ex-
ceed 10 cm
2
, and total volume should not
exceed 0.5 mL/d. The health caie piovidei
should apply the initial tieatment to dem-
onstiate the piopei application technique
and identify lesions and sites to be tieated.
Podofilox is contiaindicated duiing pieg-
nancy.
C|InIcIun-AdmInIstered Therupy
Cryosurgery w| |quJ nrogen Apply with
cotton swab oi ciyospiay. Repeat weekly
oi biweekly. Relatively inexpensive, does
not iequiie anesthesia, and does not iesult
in scaiiing.
PoJo|y||n, 10-25% In compound tinc-
tuie of benzoin. Limit the total volume
of podophyllin solution applied to 0.5
mL oi 10 cm
2
pei session. Thoioughly
wash off in 1-4 h. Tieat <10 cm
2
pei
session. Repeat weekly if necessaiy. If waits
peisist aftei six applications, othei thei-
apeutic methods should be consideied.
Podophyllin contiaindicated duiing pieg-
nancy. Repeated application may cause
iiiitation.
Trt||oroatet atJ (TC) or |t||oroatet
atJ |tar|onae (BC), 80-90% Apply
only to waits: powdei with talc oi sodium
(baking soda) to iemove unieacted acid.
Repeat weekly if necessaiy. If waits peisist
aftei six applications, othei theiapeutic
methods should be consideied.
Surgta| remoa| Eithei by tangential scis-
soi excision, tangential shave excision,
cuiettage, oi electiosuigeiy.
E|etroJesttaon/e|etrotauery Highly ef-
fective in destiuction of infected tissue
and HPV. Should be attempted only by
clinicians tiained in the use of this modal-
ity. Electiodesiccation is contiaindicated in
patients with caidiac pacemakeis.
Cervca| Warts
Foi women who have exophytic ceivical waits,
high-giade squamous intiaepithelial lesions
(SIL) must be excluded befoie tieatment is
begun. Management of exophytic ceivical waits
should include consultation with an expeit.
vaoa| Warts
Cryosurgery w| |quJ nrogen This
modality is difficult due to fog" foimation,
which iestiicts visualization of lesions.
TC or BC, 80-90% Applied to waits only,
powdei with talc oi sodium bicaibonate to
iemove unieacted acid if an excess amount
is applied. Repeat weekly if necessaiy.
PoJo|y||n, 10-25% In compound tinc-
tuie of benzoin. Tieated aiea must be diy
befoie the speculum is iemoved. Tieat with
2 cm
2
pei session. Repeat application at
weekly inteivals. Systemic absoiption is a
concein.
rethra| Meatus Warts
Cryosurgery w| |quJ nrogen As above.
PoJo|y||n, 10-25% , as above.
Aoa| Warts
Management of waits on iectal mucosa
should be iefeiied to an expeit.
Surgta| remoa| As above.
0ra| Warts
Cryosurgery w| |quJ nrogen As above.
Surgta| remoa| As above.
Fo||ow-p
Aftei visible waits have cleaied, a follow-up
evaluation is not mandatoiy. Patients should be
cautioned to watch foi iecuiiences, which oc-
cui most fiequently duiing the fiist 3 months.
Because the sensitivity and specificity of
self-diagnosis of genital waits aie unknown,
patients conceined about iecuiiences should
be offeied a follow-up evaluation 3 months
aftei tieatment. Eailiei follow-up visits may
also be useful to document a wait-fiee state,
to monitoi foi oi tieat complications of
theiapy, and to piovide the oppoitunity foi
patient education and counseling.
Women should be counseled about the need
foi iegulai cytologic scieening as iecom-
mended foi women without genital waits.
The piesence of genital waits is not an indica-
tion foi ceivical colposcopy.
Immuoocompromsed Fersoos
Peisons who aie chionically immunocom-
piomised because of HIV/AIDS, solid oigan
tiansplantation, oi othei ieasons may not
iespond as well as immunocompetent peisons
to theiapy foi genital waits and may have
moie fiequent iecuiiences aftei tieatment.
SCC aiising in oi iesembling genital waits
might occui moie fiequently among immu-
nosuppiessed peisons, iequiiing moie fie-
quent biopsy foi confiimation of diagnosis.
FICk 30-T Coody|omata acu-
moata: aous A +5,e+|o|d r+|e
W||| pe||+u+| ECw |o| ! rou||.
. |+|e pe||+u+| cou||ueu| ECw.
8. 5 |r|qu|rod c|e+r W+ +pp||ed
|||ee ||re Wee||,, c,|o||ue de|r+||||
occu||ed +||e| ! Wee|. ECw |+d cor
p|e|e|, d|+ppe+|ed |, ||e o|| Wee| o|
||e+|reu|.
Maoaemeot oI Sex Fartoers
Examination of sex paitneis is not necessaiy
because iole of ieinfection is piobably mini-
mal.
Most paitneis aie piobably alieady subclini-
cally infected with HPV, even if no waits aie
visible.
8
n|\ |u|ec||ou o| ||e +uoeu||+| ep|||e||ur c+u
|eu|| |u + pec||ur o| c|+ue |e|e||ed |o +
c |c-||-|c| |- ('||), |+u|u
||or r||d d,p|+|+ |o 'CC|'.
0.e| ||re, ||ee |e|ou c+u |e|e, pe|||,
p|o|e, o| |ecu|, |u ore c+e |o |u.+|.e
'CC.
C||u|c+||,, |e|ou +ppe+| + ru||||oc+| r+cu|e,
p+pu|e, p|+que ou ||e e\|e|u+| +uoeu||+|
|e|ou.
|e|ou |u.o|.|u ||e ce|.|\ +ud +uu |+.e ||e
|||e| ||| |o| ||+u|o|r+||ou |o |u.+|.e 'CC,
|oWe.e|, |e|ou c+u ||+u|o|r +| +u, ||e.
',, . \u|.+| |u||+ep|||e||+| ueop|+|+, peu||e
|u||+ep|||e||+| ueop|+r, |oWeuo|d p+pu|o|.
hFv: SAM0S CII CAkCIN0MA IN SII (SCCIS) AN0 INvASIv SCC 0F
AN0CNIIAI SkIN
Iermoo|oy
The Bethesda System (National Cancei
Institute) is cuiiently used as teiminology
foi dysplastic" lesions caused by HPV on
anogenital sites (Table 30-3).
The teiminology applies to both cytologic
(Pap test) and histologic assessments.
Intiaepithelial neoplasia aie designated as
ceivical (CIN), vulvai (VIN), penile (PIN),
and anal (AIN).
VIN is classified as VIN1 (mild dysplasia),
VIN2 (modeiate dysplasia), VIN3 (seveie
dysplasia oi caicinoma in situ), and VIN3 dif-
feientiated type, basaloid, bowenoid (waity).
IA8I 30-3 Bethesda System for C|assification of Anoenita| 0ysp|asia
0|der
Term|oo|ogyl St||| 0|der
h|sto|og|c F|od|ogs 8ep|aced Term|oo|ogy
A|,p|c+| p|o|||e|+||u up|+|++| |oW|+de qu+rou |u||+ep|||e||+| \||d d,p|+|+
ce|| p|eeu| |u ||e |oWe| oue||||d |u||+ep|||e||+| |e|ou (|'||) (||)
o| ||e ep|||e||ur, +|||ou| c,|op+|||c
c|+ue o| n|\ +|e |u|| |||c|ue
A|,p|c+| p|o|||e|+||u up|+|++| ce|| n|||+de '|| (n'||) ||2 +ud ||!
p|eeu| |u ||e |oWe| |Wo||||d o| ||e
ep|||e||ur, +|||ou| c,|op+|||c
c|+ue o| n|\ +|e |u|| |||c|ue
A|,p|c+| p|o|||e|+||u up|+|++| 'qu+rou ce|| 'qu+rou ce|| E|,|||op|+|+
ce|| p|eeu| |u ||e |u|| |||c|ue o| c+|c|uor+ |u ||u ('CC|') c+|c|uor+ |u o| 0ue|+|,
||e ep|||e||ur ||u ('CC|'), BoWeu d|e+e,
|oWeuo|d
p+pu|o|
|u.+|.e 'CC p|eeu|, uu+||, +|||u |u.+|.e 'CC |u.+|.e 'CC
|u + ||e|d o| n'||
to|oy HPV types 16, 18, 31, and 33 (see
Table 27-2).
Iraosmssoo HPV tiansmitted sexually.
Autoinoculation. Raiely, HPV-16 tiansmit-
ted fiom mothei to newboin with subsequent
development on penis.
Iocdeoce
Maiked inciease duiing past two decades as-
sociated with incieased sexual piomiscuity.
Ceivical SCC is the second most common
female malignancy woildwide, second only to
bieast cancei.
It is the most fiequent malignancy in develop-
ing countiies-500,000 new cases and 200,000
deaths woildwide attiibuted to it annually.
ksk Factors Immunocompiomised state, cig-
aiette smoking aie iisk factois foi moie dysplas-
tic lesions and invasive SCC.
FAIh0CNSIS
HPV-16- and -18-infected cells may not be
able to diffeientiate fully as a iesult of eithei:
Functional inteifeience of cell cycle-iegulat-
ing pioteins, caused by viial gene expies-
sion (e.g., inteiaction between HPV-16 E6
with cellulai piotein p53, inteiaction between
HPV-16 E7 with cellulai piotein pRB); oi
Oveipioduction of E5, E6, and E7.
When this occuis, the host DNA synthesis con-
tinues unchecked and leads to iapidly divid-
ing undiffeientiated cells with moiphologic
chaiacteiistics of intiaepithelial neoplasia.
Accumulated chiomosomal bieakages, ieai-
iangements, deletions, and othei genomic
mutations in these cells lead to cells with
invasion capability and, ultimately, to ceivical
malignancy.
0uratoo oI Iesoos Weeks to months to yeais
to decades.
Iocubatoo Ferod Months to yeais.
Systems kevew Piioi histoiy of condylomata
acuminata. Female paitneis of males may have
CIN.
Types of lesions
Eiythematous flat-topped papules.
Lichenoid (flat-topped) oi pigmented
papules (called |owenoJ au|oss ) (Figs.
30-8, 30-9)
May show confluence oi foim plaque(s).
Leukoplakia-like plaque (Fig. 30-10). Sui-
face usually smooth, velvety.
Colois: Tan, biown, pink, ied, violaceous,
white.
Nodule oi ulceiation in field of SIL suggests
invasive SCC (Figs. 30-11 and 30-12).
Chaiacteiistically clusteis, i.e., commonly
multifocal. May be solitaiy.
Dsr|uon
Males: glans penis, piepuce (75%) (flat li-
chenoid papules oi eiythematous macules);
penile shaft (25%) (pigmented papules).
Females: labia majoia and minoia, clitoiis.

Multicentiic involvement of the ceivix,
vulva, peiineum, and/oi anus occuis not
infiequently.
Both sexes: inguinal folds, peiineal/peiianal
skin. Oiophaiyngeal mucosa.
Sites othei than exteinal genitalia
May be associated with ceivical dysplasia,
CIN, ceivical SCC.
Raiely, SCCIS of othei sites, i.e., nail unit
(peiiungual, nail bed); intiaoial.
Mu|tp|e Sko-Co|ored Fapu|es hyperkeratoss
Exteinal genital waits, psoiiasis vulgaiis; lichen
planus.
Fmeoted Aooeota| Macu|e(s)[Fapu|e(s)
Genital lentiginosis, melanoma (in situ oi in-
vasive), pigmented basal cell caicinoma, angio-
keiatomas.
0ermatopatho|oy Epideimal piolifeiation with
numeious mitotic figuies, abnoimal mitoses,
atypical pleomoiphic cells with laige hypeichio-
matic, often clumped nuclei, dyskeiatotic cells;
basal membiane intact. Koilocytosis. Recent ap-
plication of podophyllin to condyloma acumina-
tum may cause changes similai to SCCIS.
Southero 8|ot Aoa|yss Identifies HPV type.
Fap Smear Koilocytotic atypia.
xIo|atve Cyto|oy Ceivical Pap smeais have
been iecommended annually foi women 50
yeais of age. Cytology of the anal canal may also
be helpful in management of individuals with
a histoiy of anal HPV infection, especially if
immunocompiomised (HIV/AIDS, ienal tians-
plant iecipients). Anal Pap tests aie obtained
with a ceivical biush and ThinPiep solution.
By the Bethesda System, these cytologic find-
ings aie iepoited as atypical squamous cells of
undeteimined significance (ASCUS), low-giade
squamous intiaepithelial lesion (LSIL), high-
giade (HSIL), and SCC.
0IACN0SIS
Clinical suspicion, confiimed by biopsy of
lesion.

C0kS AN0 Fk0CN0SIS
Invasive SCC develops only thiough well-
defined piecuisoi lesions (Figs. 30-11,
30-12).
Ovei time, these lesions can iegiess, peisist,
iecui, oi piogiess, in some cases to invasive
SCC.
Natuial histoiy of CIN is best studied: pio-
giession to invasive SCC occui in 36% of
cases ovei a 20-yeai peiiod.
Rate of piogiession of AIN is not known
but appeais to be incieasing. AIN may de-
velop deep in glands and, although detected
cytologically, can exist befoie visible lesions
aie detected with colposcopy.
Patients with intiaepithelial neoplasias,
which often occui in immunocompiomised
individuals, should be followed indefinitely,
with monitoiing by exfoliative cytology and
lesional biopsy specimens.
MANACMNI
Co|poscopy The most common indication foi
colposcopy is abnoimal exfoliative cytology.
Acetic acid, 3-5%, is applied to the ceivix, which
causes columnai and abnoimal epithelium to
become edematous. Abnoimal (atypical) epi-
thelium adopts a white oi opaque appeaiance
that can be distinguished fiom the noimal
pink epithelium. Abnoimal epithelium is then
biopsied. Colposcopy can also be peifoimed
on individuals with abnoimal anal exfoliative
cytology, and biopsy specimens obtained fiom
abnoimal site(s).
8opsy oI Iesoos In cases of documented SIL
oi SCCIS, biopsy specimens should be obtained
fiom iapidly enlaiging lesions, aieas of ulceia-
tion oi bleeding, exubeiant tissue with abnoi-
mal vasculaiity.
Ioca| Iherapy oI SII The only way of possibly
ieducing the potential iisk of invasive SCC is
diagnosis and eiadication of intiaepithelial dis-
ease. Because lesions aie ielatively uncommon,
cases aie often best managed by a deimatologist
with clinical expeiience in the caie of these
patients, an oncologic gynecologist, oi a coloi-
ectal suigeon. If lesion biopsy specimens do
not show eaily invasion, lesions can be tieated
medically oi suigically.
Medca| Maoaemeot 5-Fluoiouiacil cieam
has been used but is difficult to use because of
eiosions. Imiquimod cieam 5% is also effective.
Surca| Maoaemeot Suigical excision, Mohs
suigeiy, electiosuigeiy, lasei vapoiization, ciyo-
suigeiy.
FICk 30-8 hFv squamous ce|| carcooma o stu (SCCIS): peos A +3,e+|o|d r+|e W||| peu||e
|e|ou |o| 2 ,e+|. ||u||+u p+pu|e |o|r|u + cr p|+que ou ||e |+|| o| ||e peu|. |e|ou+| ||op, |epo||ed
'CC|' W||| n|\ c|+ue (|o||oc,|o|). E|ec||ou|e|, W+ ucce|u|.
FICk 30-9 hFv squamous ce||
carcooma o stu (SCCIS): vu|va A
!!,e+|o|d |eu+| ||+up|+u| |ec|p|eu| W|||
+uoeu||+| |e|ou |o| e.e|+| ,e+|. A
|+|e p|u| p|+que ou ||e pe||ueur +ud
ru|||p|e r+|| p+pu|e. |e|ou+| ||op,
W+ |epo||ed |o |oW 'CC|' W||| n|\
c|+ue (|o||oc,|o|).
FICk 30-10 hFv squamous
ce|| carcooma o stu (SCCIS):
vu|va A +9,e+|o|d r+|e W|||
n|\/A||' uo|ed |o |+.e +u+| |e|ou
|o| rou||. A W|||e |||r uodu|e ou
||e ||r o| ||e +uu. B|op, |epo||ed
'CC|' W||| n|\ c|+ue. |o |e|ou
We|e de|ec|ed ou +u+| co|pocop,.
Ceu||+| |e|pe (Cn) | + c||ou|c e\u+||, ||+ur||
|ed .||+| |u|ec||ou, c|+|+c|e||/ed |, ,rp|or+||c
+ud +,rp|or+||c .||+| |edd|u.
|u ro| c+e, |o|| p||r+|, |u|ec||ou +ud |ecu|
|euce +|e +,rp|or+||c.
w|eu ,rp|or+||c, p||r+|, Cn r+, p|eeu|
W|||
C|ouped .e|c|e o| e|o|ou +| ||e o| |uocu|+
||ou +oc|+|ed W||| |u|||c+u| p+|u
ke|ou+| |,rp|+deuop+||,.
w|eu +W+|e o| Cn, |ud|.|du+| r+, uo||ce r||d
,rp|or, uucorrou|, o| |ecu|||u ou|||e+|
o| .e|c|e +| ||e +re ||e.
\o| ,rp|or ||or Cn |e|+|e |o ||e p,c|o
|o|c+| ||r+ o| |+.|u + c||ou|c |ucu|+||e +ud
||+ur||||e 'I|.
('ee +|o 'ne|pe '|rp|e\ \||u |u|ec||ou, 'ec
||ou 21.)
|eou+|e +|e ucep||||e |o n'\ |u|ec||ou W|eu
e\poed pe||u+|+||,, W||| ||| o| |u|||c+u| ro|
||d||, +ud ro||+|||,.
',,. ne|pe eu||+||, eu||+| |e|pe |rp|e\.
|C|9 . 05+.0
|C|0 . Ao0
hkFS SIMFIX vIkS: CNIIAI INFCII0NS
FICk 30-11 hFv-oduced o stu aod ova-
sve squamous ce|| carcooma: vu|va 'e.e|+|
|ed uodu|e (|u.+|.e 'CC) +|||u W||||u + W|||e
p|+que ('CC|') ou ||e |e|| |+||ur.
FICk 30-12 hFv-oduced o stu aod ova-
sve squamous ce|| carcooma: peroea|[peraoa|
A !3,e+|o|d r+|e W||| n|\/A||' +W+|e o| pe||+u+|
|e|ou |o| e.e|+| rou||, |e |+d p||o| |||o|, o|
ECw. B|oWu pe||ue+| +ud pe||+u+| r+cu|e +ud
p+pu|e ('CC|') W||| + p|u| uodu|e +|||u +| ||e
+u+| .e|e. I|e p+||eu| p|eeu|ed W|eu |e de|ec|ed
||e uodu|e, W||c| |e ||ou|| W+ + |ero|||o|d.
E\c||ou+| ||op, o| ||e uodu|e de|ec|ed |u.+|.e 'CC
+|||u W||||u 'CC|'.
Ae oI 0oset Young, sexually active adults.
to|oy
HSV-2 > HSV-1.
Cuiiently in the United States, 30% of new
cases of GH aie caused by HSV-1.
See also Section 27.
Freva|eoce
Highly vaiiable. Depends on many factois:
countiy, iegion of iesidence, population sub-
gioup, sex, age. Highei among highei iisk sex-
ual behavioi gioups. Highei among women
than men.
Pievalence of HSV-2 seiopositivity in gen-
eial population: United States: 21%; Euiope:
8-15%; Afiica: 40-50% in 20-yeai-olds.
Stiongly associated with age, incieasing fiom
negligible levels in childien <12 to as high
as 80% among highei iisk populations. In a
given population, HSV-1 pievalence is almost
always >HSV-2 pievalence.
Pievalence is highest in aieas of Afiica and
paits of the Ameiicas. Lowei in westein and
southein Euiope than in noithein Euiope
and Noith Ameiica. Lowei in Asia than othei
aieas.
In the United States, >600,000 new infec-
tions annually; 30 million Ameiicans aie
HSV-2 infected, i.e., appioximately one in
five adults. Oldei studies iepoit the pies-
ence of antibodies to HSV-2 vaiies with the
sexual histoiy of the individual: nuns, 3%;
middle class, 25%; heteiosexuals at an STD
clinic, 26%; homosexuals, 46%; lowei classes,
46-60%; piostitutes, 70-80%.
kace aod Sex By HSV-2 seiopositivity studies
in the United States, moie common in blacks:
3 in 5 men, 4 in 5 women; in whites: 1 in 5 men,
1 in 4 women. In whites, pievalence levels off af-
tei age 30 yeais. In blacks/Hispanics, pievalence
continues to inciease aftei age 30.
Iraosmssoo
Usually skin-to-skin contact.
In most cases, 70% of tiansmission occuis
duiing times of asymptomatic HSV shedding,
which occuis duiing 1% of days when no
identifiable lesion is piesent.
Shedding iate is highei fiom HSV-2 than
HSV-1.
Tiansmission iate in discoidant couples (one
paitnei infected, the othei not) appioxi-
mately 10% pei yeai; 25% of females become
infected, compaied with only 4-6% of males.
Piioi HSV-1 infection is piotective; in females
with anti-HSV-1 antibodies, 15% become
infected with HSV-2, but in those without
anti-HSV-1 antibodies, 30% become infected
with HSV-2.
ksk Factors Ior Iraosmssoo Risk incieases
with numbei of sex paitneis. 40% of those
with 50 diffeient paitneis have genital HSV
infection.
0seases Characterted by Ceota| |cers In
the United States, most patients with genital
ulceis have GH, syphilis, oi chancioid. The
ielative fiequency vaiies by geogiaphic aiea and
patient population, but in most aieas GH is the
most common of these diseases. Moie than one
of these diseases may be piesent among at least
3-10% of patients with genital ulceis. Each dis-
ease has been associated with an incieased iisk
foi HIV/AIDS infection.
Impact oI Ch The physical symptoms of
GH aie minoi in most individuals. The majoi
symptoms aie psychological, i.e., social stigma-
tization and feai of haiming someone thiough
sexual inteicouise.
Freoaocy aod Ch
Asymptomatic HSV shedding occuis in 0.35-
1.4% of women in laboi in the United States.
32% of piegnant women have anti-HSV anti-
bodies.
10% of piegnant women aie at iisk foi pii-
maiy HSV-2 infection fiom HSV-2 infected
paitneis.
Incidence of neonatal heipes: 1 in 2800 to 1 in
15,000 biiths.
95% of newboins with HSV infection con-
tiact it duiing laboi and deliveiy.
Tiansmission can occui intiauteiine, peii-
natally, oi postnatally.
Risk factois foi neonatal HSV infection:
piimaiy GH in mothei at time of deliveiy,
absent mateinal anti-HSV antibody, pioce-
duies on fetus, fathei with HSV infection.
Tieatment of mothei with GH at time of
deliveiy is an option foi cesaiean section (not
appioved).
FAIh0CNSIS
HSV infection is tiansmitted thiough close
contact with a peison shedding viius at a
peiipheial site, mucosal suiface, oi secietion.
HSV is inactivated piomptly at ioom tem-
peiatuie; aeiosol oi fomitic spiead unlikely.

Infection occuis via inoculation onto suscep-
tible mucosal suiface oi bieak in skin.
Subsequent to piimaiy infection at inocula-
tion site, HSV ascends peiipheial sensoiy
neives and enteis sensoiy oi autonomic neive
ioot ganglia, wheie latency is established.
Latency can occui aftei both symptomatic
and asymptomatic piimaiy infection.
Reciudescences may be clinically sympto-
matic oi asymptomatic.
Iocubatoo Ferod 2- to 20-day (aveiage 6)
incubation peiiod.
Symptoms
Only 10% of HSV-2 seiopositive individuals
aie awaie that symptoms aie those of GH.
90% do not iecognize symptoms of GH.
PrImury GH
Most individuals with piimaiy infection aie
asymptomatic.
Those with symptoms iepoit fevei, headache,
malaise, myalgia, peaking within the fiist 3-4
days aftei onset of lesions, iesolving duiing
the subsequent 3-4 days.
Depending on location, pain, itching, dysuiia,
lumbai iadiculitis, vaginal oi uiethial dis-
chaige aie common symptoms.
Tendei inguinal lymphadenopathy occuis
duiing second and thiid weeks.
Deep pelvic pain associated with pelvic lymph-
adenopathy.
Some cases of fiist clinical episode of GH aie
manifested by extensive disease that iequiies
hospitalization.
Recurrent GH
New symptoms may iesult fiom old infections.
Most individuals with GH do not expeiience
classic" findings of giouped vesicles on eiy-
thematous base.
Common symptoms aie itching, buining, fis-
suie, iedness, iiiitation piioi to eiuption of
vesicles.
Dysuiia, sciatica, iectal discomfoit.
SystemIc Symptvms Symptoms of aseptic
HSV-2 meningitis can occui with piimaiy oi
iecuiient GH.
Most clinical lesions aie minoi bieaks in
the mucocutaneous epithelium, piesenting as
eiosion, abiasions," fissuies.
The classically" desciibed findings aie
untommon.
PrImury GH
An eiythematous plaque is often noted in-
itially, followed soon by giouped vesicles,
which may evolve to pustules; these become
eioded as the oveilying epideimis sloughs
(Figs. 30-13, 30-14).
Eiosions aie supeificial; may enlaige to ul-
ceiations; classic" findings desciibed below
may be ciusted oi moist.
These epithelial defects heal in 2-4 weeks, of-
ten with iesulting postinflammatoiy hypo- oi
hypeipigmentation, uncommonly with scai-
iing.
The aiea of involvement may be ciicumfeien-
tial aiound the penis, oi the entiie vulva may
be involved.
Recurrent GH
Lesions may be similai to piimaiy infection
but on a ieduced scale. Often a 1- to 2-cm
plaque of eiythema suimounted with vesicles
(Fig. 30-15), which iuptuie with foimation of
eiosions (Fig. 30-16).
Heals in 1-2 weeks.
0strbutoo Mu|es Piimaiy infection: glans,
piepuce, shaft, sulcus, sciotum, thighs, buttocks.
Recuiiences: penile shaft (Figs. 30-14, 30-15),
glans, buttocks.
Femu|es Piimaiy infection: labia majoia/
minoia (Fig. 30-13), peiineum, innei thighs.
Recuiiences: labia majoia / minoia (Fig. 30-16),
buttocks.
Aoorecta| IoIectoo Occuis following anal
inteicouise (often HSV-1); chaiacteiized by
tenesmus, anal pain, pioctitis, dischaige, and
ulceiations (Fig. 30-17) as fai as 10 cm into
anal canal.
Ceoera| Fodos RegIvnu| Lymph Nvdes
Inguinal/femoial lymph nodes enlaiged, fiim,
nonfluctuant, tendei; usually unilateial.
SIgns v] AseptIc MenIngItIs Fevei, nuchal
iigidity. Can occui in the absence of GH. Pain
along sciatic neive.
FICk 30-13 Ceota| herpes,
prmary: vu|var oIectoo \u|||p|e,
e\||ere|, p+|u|u|, puuc|edou|, cou||ueu|,
|+||oW u|ce| ou ||e eder+|ou .u|.+ +ud
pe||ueur. \|c|u||||ou | o||eu .e|, p+|u|u|.
Aoc|+|ed |uu|u+| |,rp|+deuop+||, |
corrou.
Aooeota| rosve(s)[|cer(s) Tiauma, candi-
diasis, syphilitic chancie, fixed-diug eiuption,
chancioid, gonococcal eiosion.
IA80kAI0k SI0IS
See Section 27 Heipes Simplex Viius Infec-
tions" .
0IACN0SIS
Because in most cases inteimittent asympto-
matic shedding is occuiiing and lesions aie
atypical" (not giouped vesicles on eiythema-
tous base), GH must be confiimed by viial
cultuie oi diiect fluoiescent antibody (DFA)
oi seiology.
C0kS AN0 Fk0CN0SIS
GH may be iecuiient and has no cuie.
70% of HSV-2 infections aie asymptomatic.
HSV-2 GH iecuis appioximately six times pei
yeai; HSV-1 GH usually iecuis, on the avei-
age, only once pei yeai.
Of individuals with initially symptomatic
genital HSV-2 infection, almost all have
symptomatic iecuiiences; iecuiience iates
aie high in those with an extended fiist
episode of infection, iegaidless of whethei
antiviial theiapy is given.
The iate of iecuiience is 20% highei in men
than women.
Chionic suppiessive theiapy does not com-
pletely suppiess viial shedding; it is ieduced
by 95% as detected by viial cultuie, and by
80% by PCR.
Chionic suppiessive theiapy may ieduce tians-
mission, but this has not been documented.
The incidence of piimaiy infection with acy-
clovii-iesistant HSV stiains in individuals
nevei exposed to acyclovii is 2.7% in the
United States.
Tieatment of fiist-episode infection pievents
complications such as meningitis, iadiculitis.
Eiythema multifoime may complicate GH,
occuiiing 1-2 weeks aftei an outbieak.
MANACMNI
See Table 30-5.
Webste CDC guidelines foi tieatment of gen-
ital ulceis:
|.//www.tJt.go/sJ/reamen/
FICk 30-14 Ceota| herpes, prmary: peos aod scrotum A +3,e+|o|d r+|e W||| p+|u|u| eu||+|
|e|ou |o| + d+,. \u|||p|e e|o|ou ou ||e peu| +ud c|o|ur.
FICk 30-15 Ceota| herpes, recurreot:
peos C|oup o| .e|c|e W||| e+||, ceu||+|
c|u||u ou + |ed |+e +|||u ou ||e |+|| o| ||e
peu|. I|| '|e\||oo| p|eeu|+||ou, |oWe.e|, |
ruc| |e corrou ||+u r+|| +,rp|or+||c
e|o|ou o| ||u|e.
FICk 30-16 Ceota| herpes, recurreot:
vu|va |+|e, p+|u|u| e|o|ou ou ||e |+||+.
E\|eu|.e |e|ou uc| + ||ee +|e uucorrou |u
|ecu||eu| eu||+| |e|pe |u +u o||e|W|e |e+|||,
|ud|.|du+|.
FICk 30-1T Ceota| herpes, recurreot: aous aod
peroeum \u|||p|e, p+|u|u|, |+|p|, der+|c+|ed u|ce| |u
+u n|\/A||' r+|e.
IA8I 30-5 Nanaement of Cenita| herpes
Prevention of Ch
'e\u+| ||+ur||ou |+||eu| |ou|d |e +d.|ed |o +||+|u ||or e\u+| +c||.||, W|||e |e|ou +|e p|eeu|.
ue o| coudor |ou|d |e eucou|+ed du||u +|| e\u+| e\pou|e.
E|||c+c, o| c||ou|c upp|e|.e ||e|+p, uo| p|o.eu.
|+||eu| W||| Cn |ou|d |e |o|d +|ou| ||e u+|u|+| |||o|, o| ||e d|e+e, W||| erp|+| ou
||e po|eu||+| o| |ecu||eu| ep|ode, +,rp|or+||c .||+| |edd|u, +ud e\u+| ||+ur||ou.
'e\u+| ||+ur||ou o| n'\ |+ |eeu docureu|ed |o occu| du||u pe||od W|||ou| e.|deuce
o| |e|ou. |u d|co|d+u| coup|e, ||+ur||ou uu+||, occu| du||u pe||od o| +,rp|or+||c
|edd|u.
k|| |o| ueou+|+| |u|ec||ou |ou|d |e e\p|+|ued |o +|| p+||eu|-r+|e +ud |er+|e-W||| Cn.
|e||u+|+| ||+ur||ou \+u, e\pe|| |ecorreud e|o|e||u |o| n'\ +ud n'\2 (we|e|u ||o|) +| ||e |||| p|eu+|+|
.|||. |u|+u| |o|u |o Woreu W|o +,rp|or+||c+||, |ed n'\ |+.e |educed ||||| We||| +ud
|uc|e+ed p|er+|u|||,.
Iopica| antivira| |o |u|||c+u| e|||c+c,.
therapy
0ra| antivira| therapy Au||.||+| +eu| p|o.|de p+|||+| cou||o| o| ,rp|or +ud |u o| |e|pe ep|ode W|eu ued
|o ||e+| |||| c||u|c+| ep|ode o| W|eu ued + upp|e|.e ||e|+p,. I|e, ue|||e| e|+d|c+|e
|+|eu| .||u uo| +||ec| u|equeu| |||, ||equeuc,, o| e.e|||, o| |ecu||euce +||e| d|u |
d|cou||uued. E.eu +||e| |+|o|+|o|, |e||u, +| |e+| + qu+||e| o| p+||eu| W||| Cn |+.e uo
|+|o|+|o|,cou|||red d|+uo|. \+u, e\pe|| |ecorreud ||e+|reu| |o| c|+uc|o|d +ud
,p|||| + We|| + Cn || ||e d|+uo| | uuc|e+| o| || ||e p+||eu| |e|de |u + corruu||, |u
W||c| c|+uc|o|d | p|eeu|.
|||| c||u|c+| \+u, pe|ou W||| ||||ep|ode |e|pe |+.e r||d c||u|c+| r+u||e|+||ou |u| |+|e| de.e|op
ep|ode e.e|e o| p|o|oued ,rp|or. I|e|e|o|e, p+||eu| W||| |u|||+| eu||+| |e|pe |ou|d |ece|.e
(p||r+|, o| +u||.||+| ||e|+p,.
||||
,rp|or+||c)
Ac,c|o.|| +00 r |||ee ||re + d+, |o| 1-0 d+,,
Ac,c|o.|| 200 r ||.e ||re + d+, |o| 1-0 d+,
\+|+c,c|o.|| |W|ce + d+, |o| 1-0 d+,.
|+rc|c|o.|| 250 r |||ee ||re + d+, |o| 1-0 d+,.
|||| c||u|c+|
ep|ode o|
|e|pe p|oc||||
Ac,c|o.|| +00 r |0 5 ||re d+||, |o| 0 d+, o| uu||| c||u|c+| |eo|u||ou occu|.
kecu||eu| ep|ode w|eu ||e+|reu| | |u|||u|ed (|, p+||eu|) du||u ||e p|od|ore o| W||||u 2 d+, o| oue|
o| |e|ou, p+||eu| W||| |ecu||eu| d|e+e e\pe||euce ||r||ed |eue||| ||or ||e|+p, |ec+ue
||e e.e|||, o| ||e e|up||ou | |educed. || e+||, ||e+|reu| c+uuo| |e +dr|u||e|ed, ro|
|rruuocorpe|eu| p+||eu| W||| |ecu||eu| d|e+e do uo| |eue||| ||or +c,c|o.|| ||e+|reu|,
+ud |o| ||ee p+||eu| || | uo| eue|+||, |ecorreuded.
Ac,c|o.|| +00 r |0 |||ee ||re + d+, |o| 5 d+,,
300 r |0 |W|ce + d+, |o| 5 d+,,
300 r |0 |||ee ||re + d+, |o| 2 d+,.
\+|+c,c|o.|| 500 r |0 |W|ce + d+, |o| ! d+,,
|0 |W|ce + d+, |o| ! d+,,
|+rc|c|o.|| 25 r |0 |W|ce d+||, |o| 5 d+,,
|0 ouce + d+, |o| 5 d+,.
E||o|o|c +eu|. --c c||!
A c||ou|c ,|er|c |u|ec||ou ||+ur|||ed |||ou|
||u +ud ruco+, W||| r+u||e|+||ou |u ue+||,
e.e|, o|+u ,|er.
\+u||e|+||ou.
A p+|u|e u|ce| o| c|+uc|e ou ||e rucocu|+ue
ou ||e o| |uocu|+||ou
Aoc|+|ed W||| |e|ou+| |,rp|+deuop+||,
(c|+uc|||o|r ,ud|ore. d||+| u|ce| +oc|+|ed
W||| p|o\|r+| |,rp|+deuop+||,)
'|o|||, +||e| |uocu|+||ou, ,p|||| |ecore +
,|er|c |u|ec||ou W||| c|+|+c|e||||c ecoud+|,
+ud |e|||+|, |+e (I+||e !0o).
C||u|c+| cou|e +ud |epoue |o |+ud+|d ||e|+p,
r+, |e +||e|ed |u n|\/A||'.
',,. |ue, ||e |e+| |r||+|o|. |||r+|, ,p|
|||, |, ecoud+|, ,p||||, |2, |e|||+|, ,p||||,
|!. I|e ||euc| d|e+e, ||+||+u d|e+e, 'p+u||
d|e+e, |o||| d|e+e, C|||||+u d|e+e, ||+u|
d|e+e, B||||| d|e+e. C|e+| po\ (d|||uu|||u
|| ||or r+||po\). Cup|d' d|e+e. C|+udo|e. I|e
B|+c| ||ou.
SFhIIIS |C|9 . 09.!
|C|0 . A50AB
to|oy Subspecies identified by PCR-based
methods.
Veneieal syphilis: Treonema a||Jum ssp.
a||Jum ( T. a||Jum ). T. a||Jum is a thin
delicate spiiochete with 6-14 spiials. Only
0ra| antivira| therapy
|+||, upp|e|.e keduce ||equeuc, o| |ecu||euce |, +| |e+| 15 +rou p+||eu| W||| ||equeu| (> o-9
||e|+p, pe| ,e+|) |ecu||euce. 'upp|e|.e ||e+|reu| W||| o|+| +c,c|o.|| doe uo| |o|+||, e||r|u+|e
,rp|or+||c o| +,rp|or+||c .||+| |edd|u o| ||e po|eu||+| |o| ||+ur||ou. '+|e|, +ud
e|||c+c, |+.e |eeu docureu|ed +rou pe|ou |ece|.|u d+||, ||e|+p, |o| + |ou +
5 ,e+|. Ac,c|o.|||e||+u| ||+|u o| n'\ |+.e |eeu |o|+|ed ||or ore pe|ou |ece|.|u
upp|e|.e ||e|+p,, |u| ||ee ||+|u |+.e uo| |eeu +oc|+|ed W||| ||e+|reu| |+||u|e
+rou |rruuocorpe|eu| p+||eu|. 1||- ! ,-c | :| ||-c, c:,:|. ||! |-
!:|-! | c||+ c--| | ||- c|-| c|- | -:-| -!-
Ac,c|o.|| +00 r |0 |W|ce + d+,,
\+|+c,c|o.|| 500 o| 000 r |0 ouce + d+,,
|+rc|c|o.|| 250 r o|+||, ouce + d+,.
'e.e|e d|e+e/ |+||eu| W||| |e|pe W|o do uo| |epoud |o ||e |ecorreuded doe o| +c,c|o.|| r+, |equ||e
|rruuo + |||e| o|+| doe o| +c,c|o.||, |\ +c,c|o.||, o| r+, |e |u|ec|ed W||| +u +c,c|o.|||e||+u|
corp|or|e n'\ ||+|u, |equ|||u |\ |oc+|ue|. I|e |o|e o| .+|+c,c|o.|| +ud |+rc|c|o.|| +|e uo| ,e|
e|+||||ed. |\ ||e|+p, |ou|d |e p|o.|ded |o| p+||eu| W||| e.e|e d|e+e o| corp||c+||ou
uece||+||u |op||+||/+||ou (e.., d|er|u+|ed |u|ec||ou ||+| |uc|ude eucep|+||||,
pueurou|||, o| |ep+||||).
Ac,c|o.|| 5 r/| |od, We||| |\ e.e|, 3 | |o| 5-1 d+, o| uu||| c||u|c+| |eo|u||ou | +||+|ued,
+00 r |0 5 ||re + d+, |o| 1-+ d+,.
0|+| .+|+c,c|o.|| n+.e |educed ||e uece||, |o| |\ +c,c|o.|| ||e|+p,.
|+rc|c|o.||
|eou+|+| 'ee '|eou+|+| n'\ |u|ec||ou, 'ec||ou 21.
Ac,c|o.|||e||+u| 'ee 'n'\ |u|ec||ou, 'ec||ou 21.
|oc+|ue| +0 r/| |\ q3| |o| +-2 d+,.
natuial host foi T. a||Jum is the human.
Yaws: T. a||Jum ssp. erenue .
Endemic syphilis (bejel): T. a||Jum ssp. en-
Jemtum .
Pinta: T. taraeum .
Ae oI 0oset In decieasing oidei: 20-39 yeais,
15-19 yeais, 40-49 yeais.
Iocdeoce In the United States, uncommon
with 36,000 cases of piimaiy and secondaiy
in 2006.
kace All iaces; in the United States, incidence
incieasing in Afiican Ameiicans and Hispanics.
Sex Males outnumbei females 2:1 to 4:1.
0ther Factors Until iecently, neaily half of
all males with syphilis in the United States
weie male who have sex with males (MSM),
but this peicentage has decieased due to safei
IA8I 30-6 C|assification of the C|inica| Staes of Syphi|is (See |mae 501)
Stage 0haracter|zat|oo
|||r+|, ,p|||| |oc+||/ed |u|ec||ou +| ||e o| |uocu|+||ou (c|+uc|e)
'ecoud+|, ,p|||| ||er|u+|ed |u|ec||ou (e\+u||er, r+cu|op+pu|e, coud,|or+|+ |+|+)
|+|eu| ,p|||| |o c||u|c+| |u o| ,rp|or o| |u|ec||ou (e|opo|||.e)
E+||, ,e+| du|+||ou, +u, pe||od |e|Weeu p||r+|, +ud ecoud+|, |+e
|+|e ||+u ,e+| |uce p+||eu| |ec+re |u|ec|ed
',p|||| o| uu|uoWu du|+||ou
|+|e (|e|||+|,) ,p|||| Cu|+ueou, .+cu|+|, ueu|o|o|c ||ud|u
Coueu||+| ,p|||| Acqu||ed |u u|e|o o| pe||u+|+||,, e+||, +ud |+|e c||u|c+| ||ud|u
sexual piactices. Incidence of syphilis, howevei,
has maikedly incieased in minoiities and is
associated with exchange of sex foi diugs. As-
sociated with the inciease in veneieal syphilis
is a maiked inciease in the numbei of cases of
congenital syphilis.
Iraosmssoo
Sexua| tonat : Contact with infectious lesion
(chancie, mucous patch, condyloma latum,
IMAC 30-1 C||u|c+| r+u||e|+||ou o| ,p||||. ', p||r+|, ,p||||, '2, ecoud+|, ,p||||, '!, |e|||+|,
,p||||. (||or l| Bo|ou|+, l| lo||//o, k| k+p|u, |u |-c||, |oudou, |eW \o||, ||||+de|p||+, \o|,, 200!,
p. ++!, W||| pe|r||ou
cutaneous lesions of secondaiy syphilis). 60%
of contacts of peisons with piimaiy and sec-
ondaiy syphilis become infected.
Congena| n[eton : In uteio oi peiinatal
tiansmission.
B|ooJ roJuts : One-half of cases named
as contacts of infectious syphilis become
infected. Refiigeiation of blood kills the
spiiochete.
Sero|oc Iesto Seiologic testing foi syphilis
(STS) has declined in the United States. Cui-
iently, testing is peifoimed in piegnant women,
peisons admitted to hospitals, militaiy induct-
ees, peisons undeigoing examination in physi-
cians` offices. Piemaiital testing is peifoimed in
some states.
FAIh0CNSIS
The spiiochetes pass thiough intact mucous
membiane and micioscopic abiasion in skin,
entei lymphatics and blood within a few
houis, and pioduce systemic infection and
metastatic foci befoie development of a pii-
maiy lesion.
Spiiochetes divide locally, with iesulting host
inflammatoiy iesponse and chancie foima-
tion, eithei a single lesion oi, less commonly,
multiple lesions.
Cellulai immunity is of majoi impoitance in
healing of eaily lesions and contiol of infec-
tion (T
H
1 type).
Piimaiy syphilis is the most contagious stage
of the disease.
Latei syphilis is essentially a vasculai disease,
lesions occuiiing secondaiy to obliteiative
endaiteiitis of teiminal aiteiioles and small
aiteiies and by the iesulting inflammatoiy
and neciotic changes.
0emoostratoo oI the 0raosm Dur|-FIe|d
MIcrvscvpy Positive in piimaiy chancie and
papulai lesions of secondaiy syphilis, in pai-
ticulai condylomata lata. Unieliable in oial
cavity because of the piesence of sapiophytic
spiiochetes, and negative in patients tieated sys-
temically oi topically with antibiotics. Regional
lymph node aspiiated and aspiiate examined in
the daik-field micioscope.
DIrect F|uvrescent AntIhvdy T. pu||Idum (DFA-
TP) Tes Fluoiescent antibodies aie used to
detect T. a||Jum in exudate fiom lesion, lymph
node aspiiate, oi tissue.
PCR Available in ieseaich laboiatoiies.
Sero|oc Iests Ior Syph|s Positive in pei-
sons with any tieponemal infection (veneieal
syphilis, endemic syphilis, yaws, pinta). Tests
always positive in secondaiy syphilis.
Nvntrepvnemu| STS
Measuies IgG and IgM diiected against caidi-
olipin-lecithin-cholesteiol antigen complex.
Rapid plasma ieagin (RPR) test (automated
RPR: ART).
VDRL slide test.
Nonieactive in 25% of patients with pii-
maiy syphilis.
In eaily syphilis: eithei do fluoiescent tiepone-
mal antibody-absoibed (FTA-ABS) test oi
iepeat VDRL in 1-2 weeks if initial VDRL
negative.
Piozone phenomenon: if antibody titei high,
test may be negative; must dilute seium.
Becomes nonieactive oi ieactive in lowei tit-
eis following theiapy foi eaily syphilis.
Trepvnemu| STS
FTA-ABS test.
Agglutination assays foi antibodies to
T. a||Jum :
Miciohemagglutination assay (MHA-TP;
Seiodia TPPA test).
T. a||Jum hemagglutination test (TPHA).
Often iemain ieactive aftei theiapy; not help-
ful in deteimining infectious status of patient
with past syphilis.
Fu|se-PvsItIve STS See Table 30-7. Antigen
used in nontieponemal test found in othei
tissues; may be positive (does not exceed 1:8
dilution).
va|uatoo Ior Neurosyph|s Lumbai punc-
tuie indicated in following: neuiologic signs
oi symptoms, tieatment failuie, seium ieagin
titei 1:32, HIV/AIDS seiopositivity, othei evi-
dence of active syphilis (aoititis, gumma, visual/
heaiing changes), plans to administei nonpeni-
cillin theiapy. CSF examination foi pleocytosis,
incieased piotein concentiation, VDRL activity.
Abnoimal in 40% of eaily syphilis and 25% of
latent infection.
va|uatoo Ior Syph|s o hIv[AI0S STS
foi newly diagnosed HIV/AIDS. CSF foi all
co-infected patients.
0ermatopatho|oy In piimaiy and secondaiy
syphilis, lesional skin biopsy shows cential thin-
ning oi ulceiation of epideimis. Lymphocytic
and plasmacytic deimal infiltiate. Piolifeiation
of capillaiies and lymphatics with endaiteiitis;

may have thiombosis and small aieas of necio-
sis. Dieteile stain demonstiates spiiochetes.
C0kS AN0 Fk0CN0SIS
Even without tieatment, chancie heals com-
pletely in 4-6 weeks, the infection eithei
becoming latent oi clinical manifestations of
secondaiy syphilis appeaiing.
Secondaiy syphilis usually manifests as macu-
lai exanthem initially; aftei weeks, lesions
iesolve spontaneously and iecui as maculo-
papulai oi papulai eiuptions.
In 20% of untieated cases, up to thiee to foui
such iecuiiences followed by peiiods of clinical
iemission may occui ovei a peiiod of 1 yeai.
Infection then enteis a latent stage, in which
theie aie no clinical signs oi symptoms of the
disease.
Aftei untieated syphilis has peisisted foi >4
yeais, it is iaiely communicable, except in the
case of piegnant women, who, if untieated,
may tiansmit syphilis to theii fetuses, iegaid-
less of the duiation of theii disease.
One-thiid of patients with untieated latent
syphilis developed clinically appaient teitiaiy
disease.
Gummas haidly evei heal spontaneously.
Noduloulceiative syphilides undeigo sponta-
neous paitial healing, but new lesions appeai
at the peiipheiy.
MANACMNI
See Table 30-8.
FkIMAk SFhIIIS
Symptoms A genital oi extiagenital lesion may
be noted. Ulceis aie usually painless unless su-
peiinfected.
Iocubatoo Ferod 21 days (aveiage); iange,
10-90 days.
Sko Iesoos
Chuncre
Button-like papule (Fig. 30-18) that develops
at the site of inoculation into a painless eio-
sion and then ulceiates with iaised boidei and
scanty seious exudate (Figs. 30-19 to 30-21).
Suiface may be ciusted. Size: few millimeteis
to 1 oi 2 cm in diametei. Boidei of lesion may
be iaised. Palpation: most commonly, fiim
with induiated boidei; painless.
Aiiangement: single lesion; less commonly,
few, multiple, oi kissing lesions.
Extiagenital chancies, paiticulaily on the fin-
geis, may be painful.
Atypically, genital chancies painful, especially
if supeiinfected with Sa|y|otottus aureus.
IA8I 30-T Causes of |a|sePositive Reactions in Nontreponema| Sero|oic Iests for Syphi|is
0a0se 8ate oI Fa|se-Pos|t|ve 8eact|oos,%
ACI FAIS-F0SIIIv kACII0N (6 M0NIhS)
keceu| .||+| |||ue o| |rruu|/+||ou -2
Ceu||+| |e|pe -+
n|\/A||' |u|ec||ou -+
\+|+||+
|+|eu|e|+| d|u ue 20-25
Chk0NIC FAIS-F0SIIIv kACII0N ( 6 M0NIhS)
A|u 9-
Au|o|rruue d|o|de| -20
',|er|c |upu e|,||er+|ou -20
k|eur+|o|d +||||||| 5
|+|eu|e|+| d|u ue 20-25
*
|+|+ We|e co||ec|ed ||or + .+||e|, o| pu||||ed |epo||.
'0ukCE. 'A |u|e|+||, |u || K+pe| e| +| (ed). |c |:|- | ||-c| !-!:-, o|| ed. |eW \o||, \cC|+Wn|||, 2005.
FICk 30-18 Frmary syph|s: peo|e chaocre A 23,e+|o|d r+|e W||| peu||e |e|ou |o| 1 d+,. |+|u|e
u|ce| ou d||+| peu||e |+|| W||| r+||e| e|o|ou ou ||e |+u. I|e u|ce| | qu||e |||r ou p+|p+||ou.
FICk 30-19 Frmary syph|s: oodu|e oo
|aos A 53,e+|o|d r+|e W||| peu||e |e|ou |o| 0 d+,.
ked |||r uodu|e ou ||e |+u, ||e |e|ou |eo|.ed W|||ou|
||e|+p, +ud d|d uo| u|ce|+|e. B|op, |epo||ed |u||+rr+|o|,
c|+ue. I|e d|+uo| W+ r+de |u |e||opec| W|eu 'I'
o||+|ued |e|o|e r+|||+e W+ po|||.e.
FICk 30-20 Frmary syph|s: chaocre
oo scrotum A 25,e+|o|d r+|e W||| p+|u|u|
|e|ou ou c|o|ur |o| 0 d+,. A .5cr u|ce| ou
||e c|o|ur, |||r ou p+|p+||ou.
IA8I 30-8 Recommendations for the Ireatment of Syphi|is
Pat|eots w|tho0t Pat|eots w|th 0ooI|rmed
Stage oI Syph|||s Peo|c||||o A||ergy Peo|c||||o A||ergy
|||r+|,, ecoud+|,, |eu|c||||u C |eu/+|||ue (|u|e Ie||+c,c||ue |,d|oc||o||de (500 r
o| e+||, |+|eu| doe o| 2.+ r||||ou uu|| |\, |0 |ou| ||re + d+,) o| do\,c,c||ue
.2 r||||ou uu|| |u e+c| |u||oc|) (00 r |0 |W|ce + d+,) |o| 2 Wee|
|+|e |+|eu| (o| |+|eu| o| |ur|+| puuc|u|e |ur|+| puuc|u|e
uuce||+|u du|+||ou), C'| uo|r+|. |eu|c||||u C C'| uo|r+|. Ie||+c,c||ue |,d|oc||o||de
c+|d|o.+cu|+|, o| |eu/+|||ue (2.+ r||||ou uu|| (500 r |0 |ou| ||re + d+,) o|
|eu|u |e|||+|, |\ Wee||, |o| ! Wee|) do\,c,c||ue (00 r |0 IW|ce + d+,) |o|
+ Wee|
C'| +|uo|r+|. I|e+| C'| +|uo|r+|. I|e+| +
+ ueu|o,p|||| ueu|o,p||||
|eu|o,p|||| (+,rp|or+||c Aqueou peu|c||||u C |eeu|||/+||ou +ud ||e+|reu| W|||
o| ,rp|or+||c) (3-2+ r||||ou uu||/d |\, peu|c||||u || +||e|, | cou|||red |,
|.eu |u d|.|ded doe ||u |e||u
e.e|, + |) |o| 0-+ d+,
Aqueou peu|c||||u C p|oc+|ue

(2.+ r||||ou uu||/d |\) p|u
o|+| p|o|euec|d (500 r
|ou| ||re + d+,),
|o|| |o| 0-+ d+,
',p|||| |u p|eu+uc, Acco|d|u |o |+e |eeu|||/+||ou +ud ||e+|reu| W||| peu|c||||u
|| +||e|, | cou|||red |, ||u |e||u
'0ukCE. I|ee |ecorreud+||ou +|e rod|||ed ||or ||oe |ued |, ||e Ceu|e| |o| ||e+e Cou||o| +ud ||e.eu||ou |u 993.
Ses o[ PreJ|eton : Genital sites aie most
common.
Male: innei piepuce, coional sulcus of the
glans penis, shaft, base.
Female: ceivix, vagina, vulva, clitoiis, bieast;
chancies obseived less fiequently in women
because of theii location within vagina oi
on ceivix.
Extiagenital chancies: anus oi iectum,
mouth, lips, tongue (Fig. 30-21), tonsils,
fingeis (painful!), toes, bieast, nipple.
Ceoera| Fodos Syphilis is a systemic infec-
tion; all patients should have a thoiough clini-
cal examination. Regional lymphadenopathy
appeais within 7 days. Nodes aie disciete, fiim,
iubbeiy, nontendei, moie commonly unilateial;
may peisist foi months.
Ceota| rosoo[|cer Genital heipes, tiau-
matic ulcei, fixed diug eiuption, chancioid,
lymphogianuloma veneieum.
0IACN0SIS
Clinical suspicion, confiimed by daik-field
micioscopy oi seiologically.
SC0N0Ak SFhIIIS
Secondaiy syphilis appeais 2-6 months aftei
piimaiy infection; 2-10 weeks aftei appeai-
ance of the piimaiy chancie; 6-8 weeks aftei
healing of chancie.
Chancie may still be piesent when secondaiy
lesions appeai (15% of cases).
FICk 30-21 Frmary aod secoodary
syph|s A 2+,e+|o|d r+|e W||| p+|u|u| |e|ou ou
||e |ouue +ud d|er|u+|ed |+|. . E\||+eu||+|
p||r+|, ou |ouue. A |+|e u|ce|+||ou ou ||e ||p o| ||e
|ouue. 8. A d|er|u+|ed p+pu|oqu+rou e|up||ou,
|.e., ecoud+|, ,p||||, W+ p|eeu| +| ||e ||re o| ||e
e\+r|u+||ou.
8

Concomitant HIV/AIDS infection may altei
couise of secondaiy syphilis.
Symptoms Fevei, soie thioat, weight loss, ma-
laise, anoiexia, headache, meningismus. Muco-
cutaneous lesions aie asymptomatic.
0uratoo oI Iesoos Weeks.
Sko Iesoos
Macules and papules 0.5 to 1 cm, iound to
oval; pink biownish-ied.
Frs exan|em always maculai and faint.
Laer eruons may |e au|osquamous (Figs.
30-21B to 30-24), pustulai, oi acneifoim.
Vesiculobullous lesions occui only in neona-
tal congenital syphilis (palms and soles).
Uncommonly, lesions of secondaiy syphilis
and chancie of piimaiy syphilis occui con-
comitantly.
On palpation, papules aie fiim; condylomata
lata, soft.
Shape of lesions may be annulai oi polycyclic,
especially on face in daik-skinned individuals
(Fig. 30-24).
In ielapsing secondaiy syphilis, aicifoim
lesions.
Always shaiply defined except foi maculai
exanthem.
Lesions aie scatteied, tend to iemain disciete,
and usually symmetiic.
ConJy|omaa |aa : soft, flat-topped, moist,
ied-to-pale papules, nodules, oi plaques
(Fig. 30-25), which may become confluent.
DIstrIhutIvn
Geneialized eiuption on the tiunk
(Fig. 30-21B).
Localized eiuptions most commonly aie scal-
ing and papulai; localizing, especially on the
head (haiiline, nasolabial, scalp), neck, palms
(Fig. 30-22), and soles (Fig. 30-23). Heie they
aie often hypeikeiatotic-psoiiasifoim.
Condylomata lata (Fig. 30-25): most com-
monly in anogenital iegion and mouth; can
be seen on any body suiface wheie moistuie
can accumulate between inteitiiginous sui-
faces, i.e., axillae oi toe webs.
HuIr
Diffuse haii loss, including temples and paii-
etal scalp.
Patchy, moth-eaten" alopecia on the scalp
and beaid aiea.
Loss of eyelashes, lateial thiid of eyebiows.
FICk 30-22 Secoodary syph|s: dssemoated papu|osquamous eruptoo oo pa|ms . A o|||+|,
|e|+|o||c p+pu|e ou p+|r o| p+||eu| |u ||. !02, ||e c|+uc|e W+ +|o p|eeu| ou ||e |ouue +| ||e ||re o| p|e
eu|+||ou o| ecoud+|, ,p||||. |+pu|oqu+rou |e|ou We|e +|o d|er|u+|ed ou ||uu|. 8. I|| | ||e ro|e
uu+| +ppe+|+uce o| ecoud+|, ,p|||| ou ||e p+|r. ru|||p|e po||+||o|r |e|+|o||c p+pu|e.
8
FICk 30-23 Secoodary syph|s: aoou|ar
papu|osquamous eruptoo oo so|es n,pe|
|e|+|o||c, c+||u p|+que ou ||e p|+u|+| +pec|
o| |o|| |ee| |u + 20,e+|o|d |er+|e. '|r||+|
|e|ou We|e p|eeu| ou ||e p+|r |u| |o + |ee|
e\|eu|. |o o||e| c||u|c+| ||ud|u We|e de|ec|ed.
FICk 30-24 Secoodary syph|s: aoou|ar Iaca| |esoos Auuu|+| p|+que re||u ou ||e |+ce o| +
'ou|| A|||c+u Wor+u. (Cou||e, o| le|||e, '. |o.e|, \|.)
|o c||u|c+| |u o| ,rp|or o| |u|ec||ou, 'I'
po|||.e, C'| | uo|r+|.
'upec|ed ou ||e |+| o| + |||o|, o| p||r+|, o|
ecoud+|, |e|ou, |||o|, o| e\pou|e |o ,p||||,
o| de||.e|, o| +u |u|+u| W||| coueu||+| ,p||||,
c+u occu| W|||ou| p||o| |ecou|/ed p||r+|, o|
ecoud+|, |e|ou.
A p|e.|ou ue+||.e 'I' de||ue ||e du|+||ou o|
|+|euc,.
E+||, |+|eu| ,p|||| ( ,e+|) | d|||uu||ed
||or |+|e |+|eu| d|e+e ( ,e+|).
|+|eu| d|e+e doe uo| p|ec|ude |u|ec||ouue
o| ||e de.e|opreu| o| urr+|ou ||u |e|ou,
c+|d|o.+cu|+| |e|ou, o| ueu|o,p||||.
A p|eu+u| Wor+u W||| |+|eu| d|e+e c+u |u|ec|
|e| |e|u W||| coueu||+| ,p||||.
10 o| uu||e+|ed p+||eu| ue.e| de.e|op c||u|c+||,
e.|deu| |e|||+|, ,p||||.
I|e ro|e eu|||.e ||epouer+| +u|||od, |e|
|+|e|, |ecore ue+||.e W|||ou| ||e+|reu|.
IAINI SFhIIIS
Mucvus Memhrunes
Mutous at|es , i.e., small, asymptomatic, iound
oi oval, slightly elevated, flat-topped macules
and papules 0.5-1 cm in diametei, coveied by
hypeikeiatotic white to giay membiane, occui-
iing on the oial oi genital mucosa
S| au|es at the angles of the mouth.
Ceoera| Fodos Fevei. Geneialized lym-
phadenopathy (ceivical, suboccipital, inguinal,
epitiochleai, axillaiy) and splenomegaly.
AssvcIuted FIndIngs
Mustu|os|e|ea| no|emen : peiiostitis of
long bones, paiticulaily tibia (noctuinal
pain); aithialgia; hydiaithiosis of knees oi
ankles without x-iay changes. Eyes : acute bac-
teiial iiitis, optic neuiitis, uveitis.
Menngoastu|ar reaton : CSF positive foi
inflammatoiy maikeis.
Casronesna| no|emen : diffuse phaiyn-
gitis, hypeitiophic gastiitis, hepatitis, patchy
pioctitis, ulceiative colitis, iectosigmoid
mass).
Cenournary no|emen : glomeiulonephii-
tis/nephiotic syndiome, cystitis, piostatitis.
0ermatopatho|oy Epideimal hypeikeiatosis;
capillaiy piolifeiation with endothelial swell-
ing; peiivasculai infiltiation by monocytes,
plasma cells, lymphocytes. Spiiochete is piesent
in many tissues including skin, eye, CSF.
CSF Abnoimal in 40% of patients. Spiiochetes
in CSF in 30% of cases.
Iver Fuoctoo Elevated enzymes.
keoa| Fuoctoo Immune complex-induced
membianous glomeiulonephiitis.
C0kS
In secondaiy syphilis theie may be only one
oi seveial iecuiient eiuptions that appeai
aftei month-long asymptomatic inteivals.
Initial secondaiy syphilis eiuption is a iela-
tively faint exanthem, always maculai, pink;
lesions aie ill-defined.
Latei lesions of eaily syphilis aie papulai,
biownish, and tend to be moie localized.
Symptoms may last 2-6 weeks (4 weeks av-
eiage) and may iecui in untieated oi inad-
equately tieated patients.
Secondaiy lesions subside within 2-6 weeks,
infection enteiing latent stage.
xaothem[oaothem Adveise cutaneous diug
eiuption (e.g., captopiil), pityiiasis iosea,
viial exanthem, infectious mononucleosis, tinea
coipoiis, tinea veisicoloi, scabies, id" ieaction,
condylomata acuminata, acute guttate psoiiasis,
lichen planus.
0IACN0SIS
Clinical suspicion confiimed by daik-field ex-
amination and/oi seiology. Daik-field is posi-
tive in all secondaiy syphilis lesions except foi
maculai exanthem.
FICk 30-25 Secoodary syph|s:
coody|omata |ata 'o||, ||+||opped,
ro||, p|u||+u p+pu|e +ud uodu|e ou ||e
pe||ueur +ud pe||+u+| +|e+. I|e |e|ou +|e
|eer|u W||| c||!.
FICk 30-26 Iertary syph|s:
oodu|ou|ceratve type A,rp|or
+||c, |ed||oWu, ||+u|uceu|, c|u|ed,
u|ce|+|ed p|+que W||| e|p||uou
|o|de|.
IkIIAk[IAI SFhIIIS
0uratoo oI Iesoos
In unreaeJ syphilis, 15% of patients devel-
oped late benign syphilis, mostly skin lesions.
Teitiaiy syphilis is now veiy iaie.
Pieviously, patients piesenting with teitiaiy
syphilis gave a histoiy of lesions of 3 to 7
yeais` duiation (iange, 2-60 yeais); gumma
develop by fifteenth yeai.
Cumma
Nodulai oi papulosquamous plaques that may
ulceiate, foim ciicles/aic (Fig. 30-26). May
expand iapidly causing destiuction. May be
indolent and heal with scaiiing. Solitaiy.
Skin: any site, especially on scalp, face, chest
(steinoclaviculai), calf.
Inteinal: skeletal system (long bones of legs),
oiophaiynx, uppei iespiiatoiy tiact (peifoia-
tion of nasal septum, palate), laiynx, livei,
stomach.
Neurosyph|s AsymptvmutIc NeurvsyphI|Is
Occuis in 25% of patients with untieated late
latent syphilis.
Definition: Lack neuiologic symptoms/signs
and CSF abnoimalities (mononucleai pleocy-
tosis, incieased piotein concentiations, ieac-
tive VDRL slide test).
20% of patients with asymptomatic neu-
iosyphilis piogiess to clinical neuiosyphilis in
fiist 10 yeais; iisk incieases with time.
SymptvmutIc NeurvsyphI|Is Meningeal,
meningovasculai, paienchymatous syphilis
(geneial paiesis, tabes doisalis).
Menngea| sy||s : onset of symptoms <1
yeai aftei infection; headache, nausea/vomit-
ing, stiff neck, cianial neive palsies, seizuies,
changes in mental status.
Menngoastu|ar sy||s : Onset of symptoms
5-10 yeais aftei infection; subacute encepha-
litis piodiome followed by stioke syndiome,
piogiessive vasculai syndiome. Cenera| are-
ss : Onset of symptoms 20 yeais aftei in-
fection; mnemonic paiesis aiesis, a ffect,
r eflexes (hypeiactive), e ye (Aigyll Robeit-
son pupils), s ensoiium (illusions, delusions,
hallucinations), ntellect (deciease in iecent
memoiy, oiientation, calculations, judgment,
insight), s peech].
Ta|es Jorsa|s : Onset of symptoms 25-30 yeais
aftei infection; ataxic widebased gait and
footslap, paiesthesia, bladdei distuibances,
impotence, aieflexia, loss of position, deep
pain, tempeiatuie sensations (Chaicot/neu-
iopathic joints, foot ulceis), optic atiophy.
Cardovascu|ar Syph|s Results fiom endai-
teiitis obliteians of vasa vasoium. Occuis in
10% of peisons with late untieated syphilis,
10-40 yeais aftei infection. Uncomplicated aoi-
titis, aoitic ieguigitation, sacculai aneuiysm,
coionaiy ostial stenosis.
F|aque(s) |ceratoo Craou|omas Cu-
taneous tubeiculosis, cutaneous atypical myco-
bacteiial infection, lymphoma, invasive fungal
infections.
0IACN0SIS
Clinical findings, confiimed by STS and lesional
skin biopsy; daik-field examination always neg-
ative; silvei impiegnation of histologic sections
foi demonstiation of spiiochetes only veiy
iaiely positive.
C0kS
HIV/AIDS-infected individuals with neu-
iosyphilis aie moie likely to piesent with
uveitis oi ietinitis and have significantly
highei RPR titeis.
Some, howevei, fail to iespond immunologi-
cally to T. a||Jum infection with antibody
foimation (i.e., negative STS).
HIV/AIDS testing is advised foi all patients
with syphilis.
Neuiosyphilis should be consideied in the
diffeiential diagnosis of neuiologic disease in
HIV/AIDS.
When clinical findings suggest syphilis but
STS aie negative oi confusing, alteinative tests
such as biopsy of lesions, daik-field examina-
tion, and DFA staining of lesional mateiial
should be used.
C0NCNIIAI SFhIIIS
Iraosmssoo
Duiing gestation oi intiapaitum. Risk of tians-
mission: Eaily mateinal syphilis, 75-95%;
2 yeais` duiation, 35%.
Fathoeoess
Lesions usually develop aftei fouith month of
gestation, associated with fetal immunologic
competence. Pathogenesis depends on immune
iesponse of fetus iathei than toxic effect of
spiiochete. Adequate tieatment befoie sixteenth
week of piegnancy pievents fetal damage.
Untieated: fetal loss up to 40%.
Eur|y MunI]estutIvns Appeai befoie 2 yeais
of age, often at 2-10 weeks. Infectious, iesem-
bling seveie secondaiy syphilis in adult.
Cutaneous: Bullae, vesicles on palms and soles,
supeificial desquamation, petechiae, papulos-
quamous lesions
Mucosal: Rhinitis/snuffles" (23%); mucous
patches, condylomata latum.
Bone changes: osteochondiitis, osteitis, peii-
ostitis.
Hepatosplenomegaly, jaundice, lymphaden-
opathy.
Anemia, thiombocytopenia, leukocytosis.
Lute MunI]estutIvns Appeai aftei 2 yeais of
age. Noninfectious. Similai to late acquiied
syphilis in adult.
Caidiovasculai syphilis.
Inteistitial keiatitis
Eighth neive deafness.
Recuiient aithiopathy; bilateial knee effu-
sions (Clutton joints). Gummatous peiiostitis
iesults in destiuctive lesions of nasal septum/
palate.
Asymptomatic neuiosyphilis in 33% of pa-
tients; clinical syphilis in 25%.
ResIduu| StIgmutu
Hutchinson teeth (centially notched, widely
spaced, pegshaped uppei cential incisois;
mulbeiiy" molais (multiple pooily devel-
oped cusps).
Abnoimal facies: fiontal bossing, saddle
nose, pooily developed maxillae, ihagades
(lineai scais at angles of mouth, caused
by bacteiial supeiinfection of eaily facial
eiuption).
Sabei shins.
Neive deafness
Old choiioietinitis, optic atiophy, coineal
opacities due to inteistitial keiatitis.
C|+uc|o|d | +u +cu|e 'I| c|+|+c|e||/ed |,
A c|| u|ce| +| ||e ||e o| |uocu|+||ou, uu+||,
ou ||e e\|e|u+| eu||+||+
I|e de.e|opreu| o| uppu|+||.e |e|ou+| |,r
p|+deuop+||,.
'I| ro| ||ou|, +oc|+|ed W||| |uc|e+ed |||
|o| n|\/A||' ||+ur||ou.
',,. 'o|| c|+uc|e, u|cu ro||e, c|+uc|e rou.
|C|9 . 099.0
|C|0 . A51
BFM0PBII5 0CkFYI: ChANCk0I0
See CDC guidelines foi tieatment of genital
ulceis.
to|oy H. Jutrey , a giam-negative stiepto-
bacillus.
pdemo|oy In the United States, chancioid
usually occuis in disciete outbieaks, although
the disease is endemic in some aieas.
Sex Young males. Lymphadenitis moie com-
mon in males.
ksk Factors
Tiansmission mainly heteiosexual
Males > females 3:1-25:1
Piostitution significant
Stiongly associated with illicit diug use.
Iraosmssoo Most likely duiing sexual intei-
couise with paitnei who has H. Jutrey genital
ulcei. Chancioid is a cofactoi foi HIV/AIDS
tiansmission; high iates of HIV/AIDS infec-
tion among those who have chancioid. 10%
of individuals with chancioid have syphilis oi
genital heipes.
0emoraphy Uncommon in industiialized
nations. Endemic in tiopical and subtiopical
developing countiies, especially in pooi, uiban,
and seapoit populations.
FAIh0CNSIS
Piimaiy infection develops at the site of in-
oculation (bieak in epithelium), followed by
lymphadenitis.
The genital ulcei is chaiacteiized by peiivas-
culai and inteistitial infiltiates of macio-
phages and of CD4- and CD8- lymphocytes,
consistent with a delayed-type hypeisensitiv-
ity, cell-mediated immune iesponse.
CD4- cells and maciophages in the ulcei may
explain the facilitation of tiansmission of
HIV/AIDS in patients with chancioid ulceis.
Incubation peiiod is 4-7 days.
Sko Iesoos
Piimaiy lesion: tendei papule with eiythema-
tous halo that evolves to pustule, eiosion,
and ulcei. Ulcei is usually quite enJer oi
an[u| . Its boideis aie shaip, undeimined,
and not induiated (Figs. 30-27 and 30-28).
Base is fiiable with gianulation tissue and
coveied with giay to yellow exudate.
Edema of piepuce common.
Ulcei may be singulai oi multiple, meiging
to foim laige oi giant ulceis (>2 cm) with
seipiginous shape.
DIstrIhutIvn Multiple ulceis (Fig. 30-28)
develop by autoinoculation.
Male: piepuce, fienulum, coional sulcus,
glans penis, shaft.
Female: fouichette, labia, vestibule, clitoiis,
vaginal wall by diiect extension fiom intioi-
tus, ceivix, peiianal.
Extiagenital lesions: bieast, fingeis, thighs,
oial mucosa. Bacteiial supeiinfection of
ulceis can occui.
Ceoera| Fodos Painful inguinal lymphad-
enitis (usually unilateial) occuis in 50% of
patients 7-21 days aftei piimaiy lesion. Ulcei
may heal befoie buboes occui. Buboes oc-
cui with oveilying eiythema and may diain
spontaneously.
Ceota| |cer Genital heipes, piimaiy syphilis,
lymphogianuloma veneieum (LGV), donova-
nosis, secondaiily infected human bites, tiau-
matic lesions.
Ieoder Iouoa| Mass Genital heipes, second-
aiy syphilis, LGV, incaiceiated heinia, plague,
tulaiemia.
Cram Stao Of sciapings fiom ulcei base oi
pus fiom bubo, usually not helpful.
Cu|ture Special giowth iequiiements; isola-
tion difficult. Using special media, sensitivity is
no highei than 80%.
Sero|oc Iests None available. Patients should
have HIV/AIDS seiology at time of diagnosis.
Patients should also be tested 3 months latei foi
both syphilis and HIV/AIDS infection if initial
iesults aie negative.
0ermatopatho|oy May be helpful. Oiganism
iaiely demonstiated.
FCk Detects H. Jutrey DNA sequences.
FICk 30-2T Chaocrod |+|u|u| u|ce| W||| r+||ed
u||ouud|u e|,||er+ +ud eder+. (Cou||e, o| ||o|. A|||ed
E|c|r+uu, \|.)
0IACN0SIS
Combination of painful ulcei with tendei lym-
phadenopathy (one-thiid of patients) is sug-
gestive of chancioid and, when accompanied
by suppuiative inguinal lymphadenopathy, is
almost pathognomonic.
0eIotve 0aooss Made by isolation of
H. Jutrey on special cultuie media (not widely
available). Sensitivity 80%.
Frobab|e 0aooss Made if patient has fol-
lowing ciiteiia:
Painful genital ulceis
No evidence of T. a||Jum infection by daik-
field examination of ulcei exudate oi by STS
peifoimed at least 7 days aftei onset of ulceis
Clinical piesentation, appeaiance of genital
ulceis, and lymphadenopathy, if piesent, aie
typical foi chancioid and a test foi HSV is
negative.
C0kS AN0 Fk0CN0SIS
Patients should be ieexamined 3-7 days
aftei initiation of theiapy. If tieatment is
successful, ulceis impiove symptomati-
cally within 3 days and impiove objectively
within 7 days aftei theiapy is begun.
If no clinical impiovement is evident,
diagnosis may be incoiiect, co-infection
with anothei STI agent exists, the patient
is HIV/AIDS-infected, tieatment was not
taken as instiucted, oi the H. Jutrey stiain
causing infection is iesistant to the pie-
sciibed antimiciobial.
The time iequiied foi complete healing is
ielated to the size of the ulcei; laige ulceis
may iequiie 14 days. Complete iesolution
of fluctuant lymphadenopathy is slowei
than that of ulceis and may iequiie needle
aspiiation thiough adjacent intact skin-
even duiing successful theiapy.
In HIV/AIDS, healing may be slowei, and
tieatment failuies may occui; longei tieat-
ment iegimens may be advisable.
MANACMNI
Aotmcroba| Iherapy
A/||||or,c|u |0 |u + |u|e
doe,
Ce||||+\oue 250 r |\ |u + |u|e
doe,
C|p|o||o\+c|u 500 r |0 |W|ce + d+, |o|
! d+,,
E|,|||or,c|u |+e 500 r |0 |ou| ||re +
d+, |o| 1 d+,.
Maoaemeot oI Sex Fartoers Sex paitneis
should be iefeiied foi evaluation and tieat-
ment.
FICk 30-28 Chaocrod \u|||p|e, p+|u|u|, puuc|ed
ou| u|ce| W||| uude|r|ued |o|de| ou ||e .u|.+ occu|||u
+||e| +u|o|uocu|+||ou.
A c||ou|c, |udo|eu|, p|o|e|.e, +u|o|uocu|+||e,
u|ce|+||.e d|e+e, o||eu r|d|+uoed +
,p||||.
E||o|o|c +eu|. Cc|,c||c:|- c|
c|, +u euc+pu|+|ed |u||+ce||u|+| |+r
ue+||.e |od, c|oe|, |e|+|ed |o ||-|-||c pp.
I|+ur||ou. uu+||, e\u+||,. |oue\u+| ||+u
r||ou +ud +u|o|uocu|+||ou occu|.
|ero|+p|,. Euder|c |oc| |u ||op|c+| +ud u|
||op|c+| eu.||oureu|. E\||ere|, |+|e |u uu||ed
'|+|e.
|+||oeue|. |oo||, uude||ood. \||d|, cou|+
|ou. kepe+|ed e\pou|e uece+|, |o| c||u|c+|
|u|ec||ou |o occu|. |u ro| c+e, |e|ou c+uuo|
|e de|ec|ed |u e\u+| cou|+c|.
C||u|c+| r+u||e|+||ou
|+|u|e, p|o|e|.e, u|ce|+||.e |e|ou o| ||e
eu||+| +ud pe||+u+| +|e+. n|||, .+cu|+| (|.e., +
|ee|, |ed +ppe+|+uce) +ud ||eed e+||, ou cou
|+c|. 'p|e+d |, cou||uu||, o| |, +u|o|uocu|+||ou
o| +pp|o\|r+|ed ||u u||+ce (||. !029).
|o |e|ou+| |,rp|+deuop+||,. |+|e u|cu|+
ueou uodu|e r+, r|r|c + |,rp| uode, |.e.,
peudo|u|o.
||||||u||ou o| rucocu|+ueou |e|ou
!c|-. p|epuce o| |+u, peu||e |+||, c|o|ur.
|-c|-. |+||+ r|uo|+, rou .eue||, |ou|
c|e||e. u|ce|+||ou ||eu p|e+d |, d||ec| e\|eu
|ou o| +u|o|uocu|+||ou |o |uu|u+| +ud pe||ue+|
||u.
E\||+eu||+| |e|ou occu| |u rou||, ||p, |||o+|,
|+ce, C| ||+c|, +ud |oue.
\+||+u| |,pe. u|ce|o.ee|+||.e (||. !029),
uodu|+|, |,pe|||op||c, c|e|o||c/c|c+|||c|+|.
Au+e|o||c upe||u|ec||ou r+, p|oduce p+|u +ud
|ou|re|||u e\ud+|e.
|e corrou corp||c+||ou. |eep u|ce|+||ou,
c||ou|c c|c+|||c|+| |e|ou, p||ro|, |,rp|eder+
(e|ep|+u||+| o| peu|, c|o|ur, .u|.+), e\u|e|
+u| ep|||e||+| p|o|||e|+||ou ||+| |o|, |eer||e
c+|c|uor+.
||||e|eu||+| d|+uo| |u euder|c +|e+. ',p||||||c
c|+uc|e, c|+uc|o|d, c||ou|c |e|pe||c u|ce|, |C\,
cu|+ueou |u|e|cu|o|, cu|+ueou +re||+|,
|||+||+|, 'CC.
||+uo|. \|u+||/e |ouo.+u |od|e ou |ouc| o|
c|u| p|ep+|+||ou o| |u |e|ou+| ||op, pec|reu.
ku|e ou| o||e| o| coucu||eu| c+ue o| eu||+| u|ce|
d|e+e.
Cou|e. |||||e |eudeuc, |oW+|d pou|+ueou |e+|
|u. ne+| W||| +u||||o||c ||e+|reu|. ke|+pe r+,
occu|. \+, d|er|u+|e |o p|ue, r|r|c||u |u|e|
cu|o| o| +c||uor,co|.
kecorreuded ||e|+p,. I||re||op||ru||+re||
o\+/o|e (oue dou||e||eu|| |+||e| |W|ce + d+,
|o| +| |e+| ! Wee|) o| do\,c,c||ue 00 r |0
|W|ce + d+, |o| +| |e+| ! Wee|.
',,. C|+uu|or+ |uu|u+|e, |+uu|or+
.eue|eur.
00N0vAN0SIS |C|9 . 099.2
|C|0 . A53
FICk 30-29 0ooovaooss: u|ceroveetatve type E\|eu|.e |+uu|+||ou ||ue |o|r+||ou, u|ce|+||ou,
+ud c+|||u o| ||e pe||ueur, c|o|ur, +ud peu|.
SN0k0MS CAS0 8 C. IR4CHDM4II5
|ououococc+| (|Cu) 20-+0 o| |Cu |u |e|e|oe\u+| reu +|e c||+r,d|+|. A|o c+ued |,
+ud po|ouococc+| u|e|||||| | -c|,|: .cc| , n'\.
Ep|d|d,r||| \o| corrou c+ue (10) |u e\u+||, +c||.e reu !5 ,e+|.
ke+c||.e +||||||| (ke||e|) ,ud|ore C |c:|c| |eco.e|ed ||or u|e|||+ |u up |o 10 o| reu W||| uu||e+|ed
uoud|+|||e+| |e+c||.e +||||||| ,ud|ore +ud +oc|+|ed u|e||||||. C||+r,d|+|
+ud o||e| ruco+| |u|ec||ou ( 'c|-||c '|-||c Cc,||c:|- )
||ou|| |o |u|||+|e +|e||+u|, |,pe|+c||.e |rruue |epoue ||+| p|oduce
|u||+rr+||ou +| |u.o|.ed |+|e| o|+u |u eue||c+||, (nAB21 p|euo|,pe)
p|ed|poed |ud|.|du+|.
||oc|||| Ceu||+| |rruuo|,pe |-K (ro| corrou |u ||e uu||ed '|+|e) o| |C\
|rruuo|,pe c+ue p|oc|||| |u |oroe\u+| reu W|o p|+c||ce |ecep||.e
+uo|ec|+| |u|e|cou|e.
\ucopu|u|eu| ce|.|c||| \+u, Woreu |+.e uo ,rp|or.
|e|.|c |u||+rr+|o|, d|e+e (|||) 50 o| c+e o| ||| |u ||e uu||ed '|+|e c+ued |, C |c:|c| .
|u||+|ur|u+| p|e+d |eu|| |u eudore|||||, eudo+|p|u|||, pe|.|c pe|||ou|||.
'||eu| +|p|u||| c+ue |u|e||||||,. 15 o| |||/nu|-Cu||| ,ud|ore c+ued
|, C |c:|c| .
|,rp|o|+uu|or+ .eue|eur (|C\) 'ee |u.+|.e |u|ec||ou
|e||u+|+| |u|ec||ou 50-15 o| ueW|o|u e\poed |o C |c:|c| +| ||||| +cqu||e |u|ec||ou.
50 o| ||oe |u|ec|ed de.e|op c||u|c+| e.|deuce o| |uc|u|ou coujuuc||.|||,
pueurou|||, o|||| red|+ +|o occu|.
Adu|| |uc|u|ou coujuuc||.||| C+ued |, e\pou|e |o |u|ec|ed eu||+| ec|e||ou.
I|+c|or+ kepou|||e |o| 20 r||||ou c+e o| |||udue Wo||dW|de. I|+ur|||ed ||or e,e
|o e,e .|+ |+ud, |||e, |oWe|, o||e| |or||e. |uc|deuce |+ dec|e+ed du||u
p+| |ou| dec+de.
E||o|o,. C |c:|c|, o|||+|e |u||+ce||u|+| |+c
|e||+. \+jo| ou|e|rer||+ue p|o|e|u de||ue+|e
>20 e|o.+| (|rruuo|,pe).
c:|c 'e|o.+| A, B, B+, +ud C.
!:c| '| 'e|o.+| |-K (ro| corrou
|+c|e||+| 'I|).
|.c.- '|. 'e|o.+| |
, |
2
, |
!
(|u uu||ed
'|+|e, |
2
ro| corrou|,)
|uc|deuce. \o| corrou |+c|e||+| 'I| |u .|||u+||,
e.e|, popu|+||ou. + r||||ou c+e |u ||e uu||ed
'|+|e +uuu+||,.
||e.+|euce |u ,ouu Are||c+u r+|e. !-5 |u
eue|+| red|c+| e|||u o| u||+u ||| c|oo|,
>0 |u +,rp|or+||c o|d|e|, 5-20 |u |e|
e|oe\u+| reu |u 'I| c||u|c.
C||+r,d|+| u|e|||||| ro|e corrou |u |e|e|oe\
u+| reu +ud ||| oc|oecouor|c |+|u, ouococ
c+| u|e|||||| ro|e corrou |u |oroe\u+| reu
+ud |ud|eu| popu|+||ou.
||e.+|euce o| ce|.|c+| |u|ec||ou |u ||e uu||ed
'|+|e. 5 |o| +,rp|or+||c co||ee |udeu|,
> 0 |u |+r||, p|+uu|u c||u|c. >20 |u 'I|
c||u|c.
|C\ ro|e corrou |u |oroe\u+| reu, pe|ou
|e|u|u|u ||or +||o+d (||+.e|e|, +||o|, r||||+|,
pe|ouue|).
I|+ur||ou. '-c| C |c:|c| |u pu|u|eu|
e\ud+|e | |uocu|+|ed ou|o ||u o| ruco+ o|
e\u+| p+||ue| +ud +|u eu||, |||ou| r|uu|e
|+ce|+||ou +ud +||+|ou. |-c|c|.
\+u||e|+||ou
A,rp|or+||c
',rp|or+||c ruco+| |u|ec||ou
|u.+|.e d|e+e (|C\, |ero|||+|c p|o|o
co||||).
I|ee |u|ec||ou ,ud|ore |eer||e +ud ru| |e
d|||e|eu||+|ed ||or ||oe c+ued |, ouococc|.
CBIMY0I IkCB0MII5 INFCII0NS
|C|9 . 099.+
|C|0 . A5o
FAIh0CNSIS
C. rat|omas piefeientially infects columnai
epithelium of genital tiact, eye, and iespiiatoiy
tiact. Infection often peisists foi months oi
yeais in the absence of antimiciobial theiapy.
Seiious sequelae often occui in association
with iepeated oi peisistent infections. Mecha-
nism thiough which iepeated infection elic-
its an inflammatoiy iesponse that leads to
SMFI0MS
|ououococc+| u|e|||||| (|Cu)
!- 50 |+.e ,rp|or. u|e|||+| d|c|+|e
(W|||||, ruco|d), d,u||+, u|e|||+| ||c||u.
|- . d,u||+, ||equeuc,, p,u||+.
\e+|+| e|,||er+/|eude|ue, e\ud+|e.
/|oc||||s \||d |ec|+| p+|u, rucou d|c|+|e,
|eueru, ||eed|u. 0u +uocop,, r||d, p+|c|,
ruco+, |||+|||||,, rucopu|u|eu| d|c|+|e.
Yacoa|a|en| Ce|i|c|||s \+u, Woreu |+.e
uo ,rp|or o| |||| .+|u+| d|c|+|e o|
|u|e|reu||u+| ||eed|u. !050 o| +,rp|or+||c
c+e |+.e c|+ue ou pecu|ur e\+r|u+||ou.
\e||oW rucopu|u|eu| d|c|+|e ||or eudoce|.|c+|
co|uru+| ep|||e||ur, |||+||e.
//0 \+|u+| ||eed|u, |oWe| +|dor|u+| p+|u, u|e|
|ue |eude|ue W|||ou| +due\+| |eude|ue. '||eu|
+|p|u||| |eu|| |u |+||op|+u |u|e c+|||u, ec|op|c
p|eu+uc,, +ud |u|e||||||,. Eudore|||||, eudo+|p|u
|||, pe|.|c pe|||ou|||. ',rp|or o| C |c:|c|
|u|ec||ou +|e urr+||/ed |u I+||e !09.
I0CAIII0 C. IkCB0MII5 INFCII0N
rethrts Gonoiihea, U. urea|ytum, Myto-
|asma gena|um , tiichomoniasis, heipetic
uiethiitis.
0rect Mcroscopy Low sensitivity. DFA stain-
ing used foi conjunctival smeais.
FCk Most specific and sensitive.
Cu|ture C. rat|omas can be cultuied on tissue-
cultuie cell lines in up to 60-80% of cases.
0FA Examine exudate foi antigens.
Aotbodes to C. trachomats Enzyme-LIn|ed
1mmunvsvrhent Assuy (EL1SA) 60-80% sen-
sitive and specific; 97-99% in high-iisk popu-
lations; sensitivities highei in ceivical infection
than uiethiitis in males.
DNA-RNA HyhrIdIzutIvn As sensitive and
specific as ELISA. Chlamydial DNA in uiine is
diagnostic.
tubal scaiiing and damage in the female up-
pei genital tiact uncleai. Chlamydial 60-kDa
heat-shock piotein may induce pathologic
immune iesponse oi elicit antibodies that
cioss-ieact with human heat-shock pioteins.
Chlamydial infections aie often totally asymp-
tomatic foi months.
Simultanaeous infections with gonococcus aie
common. Infections can peisist foi months oi
yeais if not tieated.
Cvmp|ement-FIrutIvn (CF) Test Acute LGV
usually has titei 1:64. Micioimmunofluoies-
cence test most sensitive and specific, identify-
ing infecting seiovai/immunotypes.
Diagnostic tests aie summaiized in Table
30-10.
C0kS AN0 Fk0CN0SIS
Absence of symptoms of C. rat|omas
infection leaves women at iisk of seiious
chlamydia-ielated moibidity thiough the
complication of PID: iecuiient PID with
endogenous vaginal floia, chionic pelvic pain,
ectopic piegnancy, and infeitility.
PID typically affects oldei women, is clini-
cally seveie, and is moie likely to piesent to
hospital.
Most common cause of epididymitis in young
men.
Othei complications: conjunctivitis, ieactive
aithiitis, pneumonitis in neonates.
IA8I 30-10 0ianostic Iests for Se\ua||y Iransmitted Ch/dmyd/d trdchomdt/s |nfection
Pres0mpt|ve 0|agoos|s* 0ooI|rmatory Test oI 0ho|ce
MN
C|+r |+|u W||| + ueu||op||| pe| o|||rre||ou u|e|||+| cu||u|e o| uoucu||u|e |e| |o| C
||e|d, uo ouococc| |c:|c| u||ue |Ck o| |Ck |o| C |c:|c|
C|+r |+|u W||| + ueu||op||| pe| o|||rre||ou ||e|d, u|e|||+| cu||u|e o| uoucu||u|e |e| |o| C
uo ouococc|, u||u+|,| W||| p,u||+ |c:|c| u||ue |Ck o| |Ck |o| C |c:|c|
W0MN
Ce|.|c+| C|+r |+|u W||| 20 ueu||op||| pe| Ce|.|c+| cu||u|e o| uoucu||u|e |e| |o| C |c:|c|,
o|||rre||ou ||e|d |u ce|.|c+| rucu u||ue |Ck o| |Ck |o| C |c:|c|
C |c:|c| +|W+, po|eu||+||, p|eeu| |u +|p|u||| Ce|.|c+| cu||u|e o| uoucu||u|e |e| |o| C |c:|c|
u||ue |Ck o| |Ck |o| C |c:|c|
\|C, |e|||e p,u||+, ue+||.e |ou||ue u||ue cu||u|e u|e|||+| +ud ce|.|c+| cu||u|e o| uoucu||u|e |e| |o|
C |c:|c|, u||ue |Ck o| |Ck |o| C |c:|c|
A0IIS 0F IIhk SX
|e+||.e ouococc+| cu||u|e +ud C|+r |+|u, +| |e+| kec|+| cu||u|e o| d||ec| |rruuo||uo|eceuce |e| |o|
ueu||op||| pe| o|||rre||ou ||e|d |u |ec|+| C |c:|c|
C|+r |+|u
C|+r |+|u W||| >+ ueu||op||| pe| o|||rre||ou u|e|||+| cu||u|e o| uoucu||u|e |e| |o| C |c:|c|
||e|d, |+c| o| ouococc| |ud|c+||.e o| |Cu
|oue |o|+||ou o| |C\ ||+|u ||or uode o| |ec|ur, occ+|ou+||,
||or u|e|||+ o| ce|.|\, |C\ C| |||e|, .o+, r|c|o||
|||e|, .52
*
A p|eurp||.e d|+uo| o| c||+r,d|+| |u|ec||ou | o||eu r+de |u ||e ,ud|ore |||ed W|eu ouococc| +|e uo| |ouud. A po|||.e |e| |o| |--c
|-c- doe uo| e\c|ude ||e |u.o|.ereu| o| C |c:|c|, W||c| o||eu | p|eeu| |u p+||eu| W||| ouo|||e+.
|0IE. C|, corp|ereu|||\|u, |Ck, ||+e c|+|u |e+c||ou, |C\, |,rp|o|+uu|or+ .eue|eur, r|c|o||, r|c|o|rruuo||uo|eceuce, \|C,
rucopu|u|eu| ce|.|c|||, |Cu, uououococc+| u|e||||||, |Ck, po|,re|+e c|+|u |e+c||ou, |Cu, po|ouococc+| u|e||||||.
'0ukCE. Adop|ed ||or wE '|+rr, |u A' |+uc| e| +| (ed). |c' |:|- | ||-c| !-!:-, 1|| ed. |eW \o||, \cC|+Wn|||, 2003.
IA8I 30-9 Symptoms of Se\ua||y Iransmitted C||amjd|a ||ac|oma||s |nfection
|oIect|oo S0ggest|ve S|goslSymptoms
|Cu, |Cu ||c|+|e, d,u||+
Ep|d|d,r||| uu||+|e|+| |u||+c|o|+| We|||u, p+|u, |eude|ue, |e.e|, |Cu
Ce|.|c||| \ucopu|u|eu| ce|.|c+| d|c|+|e, ||eed|u +ud eder+ o| ||e /oue o| ce|.|c+| ec|op,
'+|p|u||| |oWe| +|dor|u+| p+|u, ce|.|c+| ro||ou |eude|ue, +due\+| |eude|ue o| r+e
u|e|||||| |,u||+ +ud ||equeuc, W|||ou| u|euc, o| |er+|u||+
||oc|||| kec|+| p+|u, d|c|+|e, |eueru, ||eed|u, |||o|, o| |ecep||.e +uo|ec|+| |u|e|cou|e
ke+c||.e +||||||| ,ud|ore |Cu, +|||||||, coujuuc||.|||, |,p|c+| ||u |e|ou ,ud|ore
|C\ ke|ou+| +deuop+||,, p||r+|, |e|ou, p|oc||||, ,|er|c ,rp|or
8ecommeoded reg|meo A|teroat|ve reg|meos
A/||||or,c|u |0 |u |u|e doe, E|,|||or,c|u |+e 500 r |0 |ou| ||re + d+,
|o| 1 d+,,
|o\,c,c||ue 00 r |0 |W|ce + d+, |o| 1 d+, E|,|||or,c|u e||,|ucc|u+|e 300 r |0 |ou| ||re + d+,
|o| 1 d+,,
0||o\+c|u !00 r |0 |W|ce + d+, |o| 1
d+,,
|e.o||o\+c|u 500 r |0 d+||, |o| 1 d+,.
MANACMNI
Screeoo Annually foi sexually active women:
adolescents, 20-25 yeais old, oldei women with
iisk factois (new sex paitnei, multiple sex
paitneis).
Acu|e |C\ |u |e|e|oe\u+| reu | c|+|+c|e||/ed
|, + ||+u|eu| p||r+|, eu||+| |e|ou |o||oWed |,
ru||||ocu|+| uppu|+||.e |e|ou+| |,rp|+deuop+
||,.
woreu, |oroe\u+| reu, +ud-|u occ+|ou+|
|u|+uce-|e|e|oe\u+| reu r+, de.e|op |er
o|||+|c p|oc|||| W||| |e|ou+| |,rp|+deu|||.
A||e| + |+|eu| pe||od o| ,e+|, |+|e corp||c+||ou
|uc|ude eu||+| e|ep|+u||+| due |o |,rp|+||c
|u.o|.ereu|, |||c|u|e, +ud |||u|+ o| peu|,
u|e|||+, |ec|ur.
INvASIv C. IkCB0MII5 INFCII0N: IMFh0CkANI0MA vNkM
FI0MI0I0C
Sex
Heerosexua| men : acute infection piesents as
inguinal syndiome.
Vomen/|omosexua| men (MSM) : Anogeni-
toiectal syndiome most common.
0emoraphy Spoiadic/iaie in Noith Ameiica,
Euiope, Austialia, and most of Asia and South
Ameiica. Endemic in East and West Afiica,
India, paits of southeast Asia, South Ameiica,
and the Caiibbean.
FAIh0CNSIS
Piimaiily an infection of lymphatics and
lymph nodes.
Lymphangitis and lymphadenitis occui in
diainage field of inoculation site with subse-
quent peiilymphangitis and peiiadenitis.
Neciosis occuis; loculated abscesses, fistulas,
and sinus tiacts develop.
As the infection subsides, fibiosis ieplaces
acute inflammation with iesulting obliteia-
tion of lymphatic diainage, chionic edema,
and stiictuie.
Inoculation site deteimines affected lymph
nodes:
Ioocu|atoo ste Iymph oode ovo|vemeot
|eu|, +u|e||o| u|e|||+ 'upe|||c|+|, deep |uu|u+|
|o|e||o| u|e|||+ |eep |||+c, pe|||ec|+|
\u|.+ |uu|u+|
\+|u+, ce|.|\ |eep |||+c, pe|||ec|+|, |e||oc|u|+|,
|ur|o+c|+|
Auu |uu|u+|
kec|ur |e|||ec|+|, deep |||+c
Aotmcroba| Iherapy Cuies infection and
pievents ongoing tissue damage, although tis-
sue ieaction can iesult in scaiiing.
Iocubatoo Ferod
Piimaiy stage: 3-12 days oi longei
Secondaiy stage: 10-30 days (but up to 6
months)
Acute
Piimaiy genital lesion noticed in fewei than
one-thiid of men and iaiely in women.
In |eerosexua| men, women : small painless
vesicle oi noninduiated ulcei/papule on
penis oi labia/posteiioi vagina/fouichette;
heals in a few days.
In |omosexua| men (MSM), women : pii-
maiy anal oi iectal infection develops aftei
ieceptive anal inteicouise.
In women : anal/iectal infection can spiead
fiom peiineum oi via pelvic lymphatics.
Infection can spiead fiom piimaiy site of
infection to iegional lymphatics.
Iouoa| Syodrome
Chaiacteiized by painful inguinal lymphade-
nopathy beginning 2-6 weeks aftei piesumed
exposuie.
Unilateial in two-thiids of cases; palpable
iliac/femoial nodes often piesent on same
side (Fig. 30-30).
Initially, nodes aie disciete, but piogies-
sive peiiadenitis iesults in a matted mass of
nodes that may become fluctuant and sup-
puiative.
Oveilying skin becomes fixed, inflamed, thin,
and eventually develops multiple diaining
fistulas.
Gioove" sign: Extensive enlaigement of
chains of inguinal nodes above and below the
inguinal ligament; nonspecific (Fig. 30-30).
Acute ICv Papule, shallow eiosion oi ulcei,
giouped small eiosions oi ulceis (heipetifoim),
oi nonspecific uiethiitis.
Hetervseruu| Mu|es Coidlike lymphangitis of
doisal penis may follow. Lymphangial nodule
(bubonulus) may iuptuie, iesulting in sinuses

and fistulas of uiethia and defoiming scais of
penis. Multiloculai suppuiative lymphaden-
opathy.
Femu|es Ceivicitis, peiimetiitis, salpingitis
may occui.
Femu|e und Hvmvseruu| Mu|es/ReceptIve Anu|
1ntercvurse Piimaiy anal iectal infection
(hemoiihagic pioctitis with iegional lymphad-
enitis).
Other Eiythema nodosum in 10% of cases
(see Section 7).
Secoodary Stae 1nguInu| Syndrvme Uni-
lateial bubo in two-thiids of cases (most com-
mon piesentation) (Fig. 30-30). Maiked edema
and eiythema of skin oveilying node. One-thiid
of inguinal buboes iuptuie; two-thiids slowly
involute. Gioove" sign: inflammatoiy mass of
femoial and inguinal nodes sepaiated by de-
piession, oi gioove, made by Poupait ligament.
75% of cases have deep iliac node involvement
with a pelvic mass that seldom suppuiates.
AnvgenItvrectu| Syndrvme Associated with
ieceptive anal inteicouise, pioctocolitis, hypei-
plasia of intestinal and peiiiectal lymphatic tis-
sue. Resultant peiiiectal abscesses, ischioiectal
and iectovaginal fistulas, anal fistulas, iectal
stiictuie. Oveigiowth of lymphatic tissue ie-
sults in lymphoiihoids (iesembling hemoi-
ihoids) oi peiianal condylomata.
EsthIvmene Elephantiasis of genitalia, usually
females, which may ulceiate, occuiiing 1-20
yeais aftei piimaiy infection.
Frmary Stae Genital heipes, piimaiy syphi-
lis, chancioid.
Iouoa| Syodrome Incaiceiated inguinal
heinia, plague, tulaiemia, tubeiculosis, genital
heipes, syphilis, chancioid, Hodgkin disease.
Aooeotorecta| Syodrome Rectal stiictuie
caused by iectal cancei, tiauma, actinomycosis,
tubeiculosis, schistosomiasis.
sthomeoe Filaiiasis, subcutaneous mycosis.
See Table 30-10.
Imao MRI may show massive pelvic lym-
phadenopathy in women and homosexual
men.
0ermatopatho|oy Not pathognomonic. Pr-
mary sage : small stellate abscesses suiiounded
by histiocytes, aiianged in palisade pattein.
Lae sage : epideimal acanthosis/papillomatosis;
deimis-edematous; lymphatics-dilated with
fibiosis and lymphoplasmocytic infiltiate.
0IACN0SIS
By DFA, cultuie, seiologic tests, and exclusion
of othei causes of inguinal lymphadenopathy
oi genital ulceis.
C0kS AN0 Fk0CN0SIS
Highly vaiiable. Bacteiial supeiinfections may
contiibute to complications. Rectal stiictuie
is late complication. Spontaneous iemission is
common.
MANACMNI
Aotmcroba| Iherapy A 3-week couise of the
antimciobial agents iecommended foi acute
C. rat|omas infections is given (page 936).
FICk 30-30 Iymphoraou|oma veoereum '|||||u |eude| |,rp|+deuop+||, occu|||u +| ||e |ero|+|
+ud |uu|u+| |,rp| uode ep+|+|ed |, + |oo.e r+de |, |oup+|| ||+reu| (|oo.e |u).
942
S E C I | 0 N 5 1
nur+u |e||o.||ue (ke||o.|||d+e) |uc|uded |ou|
|ecou|/ed .||ue.
nur+u I |,rp|o||op|c .||ue (nI|\) | +ud nI|\
|| +|e ||+u|o|r|u |e||o.||ue. nI|\| c+ue
+du|| I ce|| |eu|er|+, ('ec||ou 20), +u+p|+||c
|+|e ce|| |,rp|or+, ||op|c+| p+||c p+|+p+|e|.
nur+u |rruuode||c|euc, .||ue, n|\/A||'
+ud n|\/A||'2, +|e c,|op+|||c .||ue o| /oouo||c
o|||u. n|\/A||' | ||e ro| corrou c+ue o|
n|\/A||' d|e+e |||ou|ou| ||e Wo||d, || cor
p||e e.e|+| u||,pe W||| d|||e|eu| eo|+p||c
d|||||u||ou. n|\/A||'2 | r+|u|, cou||ued |o
we| A|||c+.
6L08AL h|VlA|0S PAh0N|0
A||' W+ |||| |epo||ed |, ||e Ceu|e| |o| ||e+e Cou||o|
+ud ||e.eu||ou (C|C) |u 93 |u p|e.|ou|, |e+|||, reu
W|o |+d e\ W||| reu (\'\), W|o p|eeu|ed W|||
|-:,| :c pueurou|+ (|C|), +ud/o| K+po|
+|cor+ (K'), +ud/o| c||ou|c |e|pe||c u|ce|. Add|||ou+|
c+e We|e oou |ecou|/ed |u |ujec||u d|u ue| (||u),
|erop||||+c, +ud |ec|p|eu| o| ||ood ||+u|u|ou. n|\/
A||' W+ |||| |o|+|ed ||or +u |u|ec|ed |,rp| uode |u
hMAN kIk0vIkAI INFCII0NS
AN0 MC0CIAN0S
MANIFSIAII0NS 0F
hIv[AI0S 0ISAS
0|||u+|ed |u A|||c+, |||| |ecou|/ed |u ||e uu||ed
'|+|e (93) +ud |o|||, +||e| |u Eu|ope.
I|+ur||ou .|+ e\u+| |u|e|cou|e, e\pou|e |o
||ood o| ||ood p|oduc|, pe||u+|+|.
Acu|e n|\/A||' |u|ec||ou r+, |e ,rp|or+||c
W||| +cu|e n|\/A||' ,ud|ore.
C||u|c+| ||ud|u +|e o| oppo||uu|||c |u|ec||ou
+ud ueop|+r.
C||u|c+| cou|e ||||, .+||+||e.
||e.eu||ou. corp|e|e|, p|e.eu|+||e, +.o|d |||+
oc|+|ed |e|+.|o|.
w|eu +.+||+||e, +u|||e||o.||+| ||e|+p, (AkI) |
.e|, e||ec||.e |u r+u+ereu| o| ||| c||ou|c
d|e+e.
\+cc|ue. uoue |o|eee+||e.
hIv[AI0S 0ISAS AN0 AI0S |C|9 . 0+20++
|C|0 . B20B2+
93!. I|e eu/,re||u|ed |rruuoo||eu| ++, (E||'A)
e|o|e| W+ de.e|oped |u 935 +ud u|equeu||, ued
|o de|e|r|ue ||e e\|eu| o| ||e ep|der|c. Cu||eu||,, u|
'+|+|+u A|||c+ |e+| ||e |e+|e| |u|deu o| ||e ep|der|c
Wo||dW|de. I|e uur|e| o| ueW |u|ec||ou | ec+|+||u |u
couu|||e o| ||e |o|re| 'o.|e| uu|ou, |ud|+, +ud C||u+. n|\/
A||' |u|ec||ou | r+u||e|ed + oppo||uu|||c |u|ec||ou,
+|e|.e c+uce|, +ud ueu|o|o|c ||ud|u (dereu||+ +ud
ueu|op+||,). n+|| o| 5 r||||ou ueW n|\/A||' |u|ec||ou
occu|||u +uuu+||, +|e |u |ud|.|du+|, +ed 5 |o 2+ ,e+|.
SCII0N 31 nu\A| kEIk0\|kA| |||ECI|0|' A|| \uC0CuIA|E0u' \A|||E'IAI|0|' 0| n|\/A||' ||'EA'E 943
Ae oI 0oset Commonly in the young, but
any age.
Sex Woildwide, moie common in males. In
sub-Sahaian Afiica, equal sex distiibution.
to|oy Woildwide, neaily all infections aie
HIV/AIDS-1. HIV/AIDS-2 causes disease in
westein Afiica. In the United States, HIV/AIDS-
1 subtype B is piedominant.
ksk Factors Ior Iraosmssoo
Sexual contact with an infected peison. In
the United States, heteiosexual tiansmission
is incieasing; homosexual tiansmission is
decieasing.
Blood oi blood pioducts.
Peiinatal exposuie (intiapaitum, peiinatal,
bieast feeding); infected motheis to infants.
ksk Factors Ior Acqustoo Genital ulcei dis-
ease, HIV/AIDS-infected paitnei with high vi-
ial load (tiansmission moie efficient), ieceptive
anal inteicouise.
Iocdeoce Woildwide: appioximately 33.2
million infections. Two-thiids of infections aie
in sub-Sahaian Afiica. United States: 56,000
new HIV/AIDS infections occuiied in 2006.
In 2003, 5 million new infections woildwide; 3
million deaths.
0emoraphy Epidemic expanding in Asia, es-
pecially India and China, which have popula-
tions of >1 billion each. Rapidly expanding
in Baltic States, the Russian Fedeiation, and
seveial Cential Asian Republics.
AI0S 0eIotoo Any HIV/AIDS-infected indi-
vidual with a CD4 T cell count of 200/L has
AIDS by definition, iegaidless of the piesence of
symptoms oi oppoitunistic diseases. See Table 31-1
foi clinical categoiies of HIV/AIDS infection).
FAIh0CNSIS
Aftei piimaiy HIV/AIDS infection, billions of
viiions aie pioduced and destioyed each day; a
concomitant daily tuinovei of actively infected
CD4 cells is also in the billions. HIV/AIDS in-
fection is ielatively unique among human viial
infections in that, despite iobust cellulai and
humoial immune iesponses that aie mounted
aftei piimaiy infection, the viius is not cleaied
completely fiom the body (with a few excep-
tions). Chionic infection develops that peisists
with vaiying degiees of viius ieplication foi
a median of 10 yeais befoie an individual be-
comes clinically ill.
CIINICAI FIN0INCS
Neaily all HIV/AIDS-infected individuals man-
ifest some deimatologic disoidei attiibutable
to piogiessive immunodeficiency duiing the
couise of the infection. Some disoideis aie
highly associated with HIV/AIDS infection,
and theii diagnosis often waiiants HIV/AIDS
seiotesting (Table 31-2).
Foi much of the couise of HIV/AIDS dis-
ease, patients iemain asymomat. Non-
specific complaints include: feveis, night
sweats, chills, weakness, lymphadenopathy,
and weight loss.
Infected individuals may appeai healthy even
in an advanced immunocompiomised state,
i.e., CD4 cell counts appioaching zeio. In ad-
vanced untieated disease, wasting is common,
especially in sub-Sahaian Afiica.
IA8I 31-1 1995 Revised C|assification System for h|V/A|0S |nfection and E\panded A|0S
Survei||ance Case 0efinition for Ado|escents and Adu|ts
0||o|ca| 0ategor|es
A Asymptomat|c, 8 Symptomat|c,
004 T 0e|| Ac0te (Pr|mary) hot A or 0 0 A|0S-|od|cator
0ategor|es h|V or P6L
0ood|t|oos 0ood|t|oos
500/| A B C
200-+99/| A2 B2 C2
200/| A! B! C!
c
|C|, p|o|e|.e eue|+||/ed |,rp|+deuop+||,.
'0ukCE. \\wk +2(|o. kk1), |ecer|e| 3, 992.
Sko Fodos
In advanced untieated disease, skin findings
may be common and extieme:
|e|r+|o|o|c d|o|de|
A|op|c de|r+||||
|o||+|
||u|||u W||| ecoud+|, c|+ue o| e\co||+||ou
'e|o|||e|c de|r+||||
/e|o|
Ad.e|e cu|+ueou d|u |e+c||ou
0ppo||uu|||c |u|ec||ou
\uco+| c+ud|d|+|
\o||ucur cou|+|our
ne|pe |rp|e\ .||u (n'\), c||ou|c |e|pe||c
u|ce|
\+||ce||+/o|e| .||u (\/\). ne|pe /o|e|
nur+u p+p|||or+.||u (n|\) |u|ec||ou. ||u, ru
co+|
0ppo||uu|||c ueop|+r
K+po| +|cor+
|ounod||u +ud nod||u |,rp|or+
|||r+|, ceu||+| ue|.ou ,|er (C|') |,rp|or+
n|\|uduced d,p|+|+ +ud |u.+|.e qu+rou
ce|| c+|c|uor+
Ce|.|\
Auu
uu|que |o n|\/A||' d|e+e
Acu|e n|\/A||' ,ud|ore (An')
0|+| |+||, |eu|op|+||+ (0n|)
Eo|uop||||c |o|||cu|||| (E|)
B+c|||+|, +u|or+|o| (BA)
Systemc Fodos
OppvrtunIstIc 1n]ectIvns (O1s) Myto-
|aterum u|ertu|oss (MTb) disease, Myto-
|aterum aum complex (MAC) infection,
baitonellosis (Barone||a |ense|ae, B. qunana );
syphilis; Pneumotyss ,roet (pieviously tar-
n ) pneumonia (PCP), candidiasis, ciypto-
coccosis, histoplasmosis, coccidioidomycosis,
aspeigillosis, penicillinosis, paiacoccidioidomy-
cosis; cytomegaloviius (CMV) disease, ciypt-
ospoiidiosis; HSV disease, VZV disease, human
heipesviius-8 (HHV-8), HPV, hepatitis C viius
(HCV) disease, hepatitis B viius (HBV) disease;
leishmaniasis.
OppvrtunIstIc Nevp|usms (ONs) Kaposi sai-
coma (HHV-8), non-Hodgkin lymphomas Ep-
stein-Baii viius (EBV)], ceivical cancei (HPV),
anal cancei (HPV).
Other AIDS dementia complex, piogiessive
multifocal leukoencephalopathy, wasting syn-
diome.
IA8I 31-2 Nucocutaneous |indins Associated with h|V/A|0S |nfection and |ndications
for h|V Serotestin
8|sk Ior h|V |oIect|oo N0coc0taoeo0s F|od|og
n||-e|o|e||u +|W+, |ud|c+|ed Acu|e |e||o.||+| ,ud|ore
K+po| +|cor+
0|+| |+||, |eu|op|+||+
||o\|r+| u|uuu+| ou,c|or,co|
B+c|||+|, +u|or+|o|
Eo|uop||||c |o|||cu||||
C||ou|c |e|pe||c u|ce| (> rou|| du|+||ou)
Au, e\u+||, ||+ur|||ed d|e+e
'||u ||ud|u o| |ujec||u d|u ue (||u)
\ode|+|e-e|o|e||u r+, |e |ud|c+|ed ne|pe /o|e|
\o||ucur cou|+|our. ru|||p|e |+c|+| |u +u +du||
C+ud|d|+|. o|op|+|,ue+|, eop|+e+|,
o| |ecu||eu| .u|.o.+|u+|
|o|||e-e|o|e||u r+, |e |ud|c+|ed Ceue|+||/ed |,rp|+deuop+||,
'e|o|||e|c de|r+||||
Ap|||ou u|ce| (|ecu||eu|, |e||+c|o|, |o ||e|+p,)
0aooss oI hIv[AI0S IoIectoo See Image
31-1.
C04 I Iymphocytes Image 31-2 illustiates
the typical couise of HIV/AIDS disease in the
absence of any theiapeutic inteiventions, fol-
lowing CD4 T lymphocyte counts and HIV/
AIDS RNA. CD4 cell counts aie used to moni-
toi degiee of immunodeficiency and iesponse
to antiietioviial theiapy.
hIv[AI0S kNA HIV/AIDS RNA used to moni-
toi iesponse to ART.
C0kS AN0 Fk0CN0SIS
The clinical couise of HIV/AIDS disease is
highly vaiiable in each individual. Some patients
expeiience symptomatic piimaiy infection. A
piolonged asymptomatic state is common. OIs
and ONs occui in advanced disease. Eaily in the
pandemic, piophylaxis foi OIs and tieatment of
ONs impioved the piognosis. Cuiiently, ART
has been veiy effective in the majoiity of cases
but may give iise to new complications such as
lipodystiophy and the metabolic syndiome.
MANACMNI
hIv[AI0S Freveotoo Ser EducutIvn The
most common mode of HIV/AIDS tiansmis-
sion is duiing sexual inteicouise. Cuiiently,
in teims of numbeis of new HIV/AIDS in-
fections, female-to-male and male-to-female
tiansmission aie much moie common than
male-to-male. Safei sexual piactices must be
taught at an eaily age.
Truns]usIvns und Trunsp|untutIvn Blood
and blood by-pioducts must be tested befoie
administiation. HIV/AIDS infection must be
iuled out in donois of any tiansplanted oigan.
Aotretrovra| Iherapy (AkI)
Guidelines foi ART evolve as new diugs become
available and local iesouices. Websites foi up-
dated guidelines of ART aie as follow:
United States:
|.//www.guJe|ne.go/summary/summary.
asx?ss15cJot_J11255cn|r5881
Woild Health Oiganization:
|.//www.w|o.n/|/aJs/ots/ar/en/
Immuoe kecoosttutoo IoI|ammatory Syodrome
(IkIS) IRIS occuis in some HIV/AIDS-in-
fected patients aftei initiating ART, iesulting
fiom iestoied immunity to specific infectious
oi noninfectious antigens. A paiadoxical clini-
cal woisening of a known condition oi the
appeaiance of a new condition aftei initiating
theiapy chaiacteiizes the syndiome. Potential
mechanisms foi the syndiome include a paitial
iecoveiy of the immune system oi exubeiant
host immunologic iesponses to antigenic stim-
uli. The oveiall incidence of IRIS is unknown
but is dependent on the population studied and
its undeilying oppoitunistic infectious bui-
den. The infectious pathogens most fiequently
implicated in the syndiome aie Myto|atera
(MAC, MTb), VZV, HSV, CMV. Also, eosi-
nophilic folliculitis.
IMAC 31-1 A|o||||r o| |oW |o
p|oceed W||| e|o |e||u |u c+e o| u
pec|ed n|\/A||'.
Repeat
HlV-2/HlV-2
ElA
8creeuiug
HlV-1/HlV-2
ElA
HlV-2
ElA
HlV-1
westeru
olot
HlV-2
westeru
olot
Diagouosis
of HlV-1
iufectiou
Diagouosis
of HlV-2
iufectiou
Repeat iu 4-O weeks*
Retest iu
8-O mouths
if cliuicall]
iudicated
-
+
+
+ +
+
-
-
-
-
Iocubatoo Ferod Signs and symptoms occui
within 5-30 days following HIV/AIDS exposuie
(most within 21-28 days).
Symptoms iange fiom asymptomatic to se-
veie iequiiing hospitalization (15%). Diagnosis
is often missed, consideied in only 25% of
cases. Between 50 and 70% of iecently infected
individuals expeiience symptomatic piimaiy
infection.
Mucocutaoeous Symptoms Cutaneous: Rash
(50-60%). Exanthem usually appeais 2-3 days
aftei onset of fevei, lasting 5-8 days; enanthem,
asymptomatic; ulceis, painful in mouth and/oi
anogenital iegion. Phaiyngitis (50-70%); oial
ulceis (10-20%). Genital ulceis (5-15%).
Systemc Symptoms Fevei (>80-90%), fatigue/
lethaigy/malaise (>70-90%), myalgia/aithialgia
(50-70%), night sweats (50%), anoiexia/weight
loss (25%), aseptic meningitis (24%), othei
neuiologic symptoms (encephalitis, peiipheial
neuiopathy, myelopathy, headache, ietiobul-
bai pain), anoiexia (21%), nausea/vomiting/di-
aiihea (30-60%), lymphadenopathy.
Sko Iesoos
Moibillifoim iash, i.e., infectious exanthem
(Fig. 31-1) with pink macules, papules up to 1
cm in diametei. Ulceis occui on penis and/oi
sciotum. Less common: uiticaiia. Lesions ie-
main disciete. Most common site of exanthem
is uppei thoiax and collai iegion (100%) > face
(60%) > aims (40%) > scalp, thighs (20%).
Palms.
Mucous Membraoes Phaiyngitis. Enanthem,
spotty, on haid and soft palate. Aphthous-like
ulceis: 5-10 mm in diametei, iound to oval,
shallow with white bases suiiounded by a ied
|||r+|, n|\/A||' |u|ec||ou
',rp|or |+ue ||or +,rp|or+||c |o e.e|e.
',rp|or+||c |u 10 o| p||r+|, |u|ec||ou.
C|+|+c|e||/ed |, +u |u|ec||ou rououuc|eo|-
|||e ,ud|ore o| +ep||c reu|u||| ,ud|ore
W||| |e.e|, |,rp|+deuop+||,, +ud ueu|o|o|c +ud
+||o|u|e||u+| (C|) ,rp|or.
C|+|+c|e||||c |u|ec||ou e\+u||er, eu+u||er,
+ud o|+|/eu||+| u|ce|+||ou.
ACI hIv[AI0S SN0k0M (AhS) |C|0 . B2!.0
halo, aiising on the tonsils, palate, and /oi buccal
mucosa; esophageal ulceis. Uncommonly, oial
candidiasis.
AnvgenItu|Iu Ulceis: piepuce of penis, scio-
tum, anus, anal canal.
Ceoera| xamoatoo Lymph Nvdes Lym-
phadenopathy.
Neurv|vgIc FIndIngs Acute meningitis; acute
ieveisible encephalopathy with loss of memoiy,
alteiation of consciousness, and peisonality
change.
Piimaiy EBV infection (infectious mononu-
cleosis) (1% of negative monospots aie AHS);
piimaiy CMV infection; influenza; acute hepa-
titis A, B, and C infection; iubella. Syphilis: 1
and 2. Rocky Mountain spotted fevei. Adveise
cutaneous diug ieaction.
0IACN0SIS
Demonstiated seioconveision of anti-HIV/
AIDS antibodies by ELISA, confiimed by West-
ein blot, confiims diagnosis of piimaiy HIV/
AIDS infection. Detection of HIV/AIDS RNA.
C0kS AN0 Fk0CN0SIS
Most individuals expeiience no oi mild symp-
toms that do not piompt medical consultation.
In those with symptomatic illness, the mean du-
iation of illness in one study was 13 days (iange
5-44 days). Long-teim illness of >2 weeks is
associated with highei iisk of developing AIDS
within 3 yeais of seioconveision.
SCII0N 31 nu\A| kEIk0\|kA| |||ECI|0|' A|| \uC0CuIA|E0u' \A|||E'IAI|0|' 0| n|\/A||' ||'EA'E 94T
FICk 31-1 Acute hIv[AI0S syodrome:
exaothem ||c|e|e, e|,||er+|ou r+cu|e
+ud p+pu|e ou ||e +u|e||o| ||uu|, +oc|+|ed
||ud|u We|e |e.e| +ud |,rp|+deuop+||,.
(Cou||e, o| A|r|u k|ee|, \|.).
IMAC 31-2 I,p|c+| d|e+e cou|e |u +u |ud|.|du+| W||| n|\ |u|ec||ou. ('ou|ce. A' |+uc| e| +|. Auu |u|e|u \ed
2+.o5+, 99o, W||| pe|r||ou.)
Acute HlV s]udrome
wide dissemiuatiou of virus
8eediug of l]mphoid orgaus
Death
0pportuuistic
diseases
0oustitutioual
s]mptoms
H
l
V

R
h
A

0
o
p
i
e
s

p
e
r

l

P
l
a
s
m
a
10
8
10
7
10
O
10
5
10
4
10
8
10
2
0liuical lateuc]
Primar]
iufectiou
weeks
0
0
100
200
800
400
500
O00
700
800
900
1000
0
D
4
+

T

l
]
m
p
h
o
c
]
t
e

c
o
u
u
t

(
c
e
l
l
s
/
m
l
}
1100
1200
1 2 8 4 5 O 7 8 9 10 10 8 O 9 12
Years
FI0MI0I0C AN0 FAIh0CNSIS
Unknown.
Modeiate to intense itching unielieved by
many theiapies. Piuiitus may be seveie, espe-
cially in those with atopic diathesis, distuib-
ing sleep. Initially, occuiied in the setting
of advanced HIV/AIDS disease. Cuiiently,
occuis following initiation of ART, associated
with IRIS.
Sko Iesoos
Piimaiy lesions aie 3- to 5-mm eiythematous,
edematous, folliculai papules and pustules
(Fig. 31-2 and B). Dozens oi hundieds of
lesions may be piesent, in vaiious stages
of evolution. Fiequently, changes second-
aiy to sciatching/iubbing aie seen: exco-
iiations/ciusting of papules/pustules; atopic
deimatitis, lichen simplex chionicus, piuiigo
nodulaiis.
SetonJary n[etons o[ extoraeJ ses : im-
petiginization, fuiunculosis, cellulitis. Postin-
flammatoiy hypeipigmentation occuis in moie
daikly pigmented individuals and can be quite
disfiguiing.
DIstrIhutIvn Tiunk ; head and neck; pioximal
extiemities. In some individuals, lesions piesent
only on face oi on tiunk.
Alleigic contact deimatitis, adveise cutane-
ous diug ieaction, atopic deimatitis, scabies,
papulai uiticaiia (insect bites), acne vulgaiis,
bacteiial folliculitis ( Sa|y|otottus aureus),
Ma|asse:a folliculitis.
||u||||c |o|||cu|+| e|up||ou o| ||e uppe| ||uu|, |+ce,
uec|, +ud p|o\|r+| e\||er|||e
0ccu| |u +d.+uced n|\/A||' d|e+e +ud/o| +||e|
|u|||+||ou o| AkI
keo|.e W||| ucce|u| AkI
',, . Eo|uop||||c pu|u|+| |o|||cu||||
*
|o| e||ou ||e|| |u| +u |ud|c+|o| o| e||ou n|\/A||'
d|e+e.
0SIN0FhIIIC F0IIICIIIIS |C|9 . 10+.3
Cu|tures Negative foi pathogenic oiganisms.
Many patients with longstanding untieated EF
have secondaiy colonization/infection with S.
aureus .
0ermatopatho|oy Peiifolliculai and peiivas-
culai infiltiate with vaiying numbeis of eosi-
nophils. Epithelial spongiosis of folliculai
infundibulum and/oi sebaceous glands asso-
ciated with a mixed cellulai infiltiate. Eosi-
nophilic pustules uncommon. Special stains foi
bacteiia, fungi, and paiasites aie negative.
hemato|oy Eosinophilia. CD4 cell count
usually <100/L.
0IACN0SIS
Clinical diagnosis confiimed by biopsy of a new
piimaiy lesion (folliculai papule) with cultuies
iuling out infectious causes.
C0kS AN0 Fk0CN0SIS
In untieated HIV/AIDS disease, the couise of
EF tends to be chionic and peisistent. Occuiiing
aftei the initiation of ART (IRIS), symptoms of-
ten peisist foi weeks to months if untieated.
MANACMNI
Piuiitus is modeiate to seveie, significantly affect-
ing quality of life. Changes secondaiy to chionic
sciatching such as secondaiy infections and lichen
simplex chionicus should also be identified and
tieated. The most piedictably effective theiapy is
a shoit tapeied couise of oial glucocoiticoid such
as piednisone. Sedating antihistamines such as
hydioxyzine oi doxepin aie effective as antipiuiit-
ics at bedtime because of sedation.
FICk 31-2 osooph|c Io||cu|ts \u|||p|e, .e|, p|u||||c, eder+|ou p+pu|e W||| + ceu||+| c|u| +ud +
|eW pu|u|e |u + r+|e W||| +d.+uced n|\/A||' d|e+e (C|+ ce|| couu| 50/|). 4. ||||||u||ou ou ||e uppe|
||uu|, |e|ou We|e +|o p|eeu| ou ||e |+ce +ud uec|. B. C|oeup |oW|u u|||c+||+| p+pu|e, pu|u|e, +ud
e|o|ou ecoud+|, |o |u|||u +ud c|u|, ||e p||r+|, |e|ou |eer||e p+pu|+| u|||c+||+ (|uec| |||e) |u| +|e
|o|||cu|+|.
Many adults have asymptomatic EBV infection
of the oiophaiynx. EBV is thought to emeige
fiom latency as HIV/AIDS-induced immuno-
compiomise piogiesses and causes the epidei-
mal hypeiplasia.
Iocubatoo Ferod Usually 5 to 10 yeais aftei
piimaiy HIV/AIDS infection.
Symptoms Asymptomatic, but stigmatization
of HIV/AIDS disease.
0ra| Mucosa White oi giayish-white, well-
demaicated plaque (Fig. 31-3) with coiiugated
textuie. Most commonly on the lateial and
infeiioi suifaces of the tongue. Often piesent
bilateially, but size of plaques usually not
equal. Some individuals may have oiopha-
iyngeal candidiasis and/oi condyloma in
addition to OHL.
Thiush, condyloma acuminatum, geo-
giaphic oi migiatoiy glossitis, lichen planus,
tobacco-associated leukoplakia, mucous
patch of secondaiy syphilis, squamous cell
caicinoma (SCC) eithei in situ oi invasive,
occlusal tiauma.
0ermatopatho|oy Acanthotic epithelium
with hypeikeiatosis, haiilike piojections of
keiatin, aieas of koilocytes (ballooned cells
with cleai cytoplasm). E|etron mtrostoy :
Heipes viial stiuctuies in epithelial cells;
positive foi EBV maikeis.
Cu|tures Not helpful. CanJJa a||tans is
commonly isolated.
Beu|u |,pe|p|+|+ o| o|+| ruco+
E||o|o,. Ep|e|uB+|| .||u (EB\)
C|+ ce|| couu| !00/|
|u|e|o|+|e|+| u||+ce o| ||e |ouue
w|||e, co||u+|ed p|+que
*
|o| e||ou ||e|| |u| +u |ud|c+|o| o| e||ou n|\/A||'
d|e+e.
0kAI hAIk Ik0FIAkIA (0hI) |C|0 . K!.!
0IACN0SIS
Clinical diagnosis. Does not iub off; does not
cleai with adequate anticandidal theiapy.
C0kS AN0 Fk0CN0SIS
Usually iesolves with ART.
MANACMNI
Reassuiance that OHL is a benign viial infection
is usually adequate to ieduce patients` conceins,
a cosmetic pioblem that is not piecanceious.
Iopca| Iherapy Podophyllin 25% in tinctuie
of benzoin applied to the lesion with a cotton-
tipped applicatoi foi 5 min.
Systemc Aotvra| 0rus ART indications of-
ten iesult in iegiession/cleaiing of OHL.
FICk 31-3 0ra| hary |eukop|aka w|||e p|+que
ou ||e |+|e|+| |ouue W||| co|du|o,|||e p+||e|u. I|e ||ud|u
| eeu||+||, p+||ouorou|c |o| n|\/A||' |u|ec||ou. |u |||
c+e, 0n| occu||ed + |e||+uce |o AkI e.o|.ed +ud C|+
couu| dec||ued. 0n| |eo|.ed W||| c|+ue |u AkI.
to|oy Most common diugs causing ACDE:
tiimethopiim-sulfamethoxazole (TMP-SMX),
sulfadiazine, tiimethopiim-dapsone, and ami-
nopenicillins.
Freva|eoce Diug hypeisensitivity complicates
3-20% of all piesciiptions in those with ad-
vanced HIV/AIDS disease, up to 100 times
moie common than in the geneial population.
FAIh0CNSIS
Incidence incieases with advancing immuno-
deficiency; may be coiielated with the decline
and dysiegulation of immune function. Aftei
immune ieconstitution by ART, some patients
who had pieviously toleiated a diug may de-
velop alleigic cutaneous diug ieactions (IRIS).
CIASSIFICAII0N
Diug eiuptions can mimic viitually all the
moiphologic expiessions in deimatology and
must be fiist on the diffeiential diagnosis in the
appeaiance of a sudden symmetiic eiuption
(see Section 22).
Exan|emaous/mor|||[orm : Account foi
95% of ACDE in HIV/AIDS disease. Be-
tween 50 and 60% tieated with TMP-
SMX develop a moibillifoim eiuption 1-2
weeks aftei staiting theiapy.
Crxan : Retinoid deimatitis: chionic paio-
nychia, cheilitis, pyogenic gianuloma.
Toxt eJerma| netro|yss (TEN). The inci-
dence of TEN caused by sulfonamides is
also incieased.
LoJysro|y synJrome : See below.
CIINICAI FIN0INCS See Section 22
|uc|deuce o| +d.e|e cu|+ueou d|u e|up||ou
(AC|E) |o + .+||e|, o| d|u | ||| |u n|\/A||'
d|e+e.
|uc|e+e W||| +d.+uc|u |rruuode||c|euc,.
95 +|e ro|||||||o|r
Io\|c ep|de|r+| uec|o|,| |+ ||| ro|||d||,/
ro||+|||,
AkI |educe ||e |uc|deuce o| AC|E
AkI c+u c+ue + W|de pec||ur o| AC|E
A0vkS CIAN0S 0kC kFII0NS
IN hIv[AI0S 0ISAS |C|9 . o9!.0
|C|0 . |21.0
MANACMNI
In most cases, the implicated oi suspected diug
should be discontinued. In some, such as with
moibillifoim eiuptions, the offending diug can
be continued and the eiuption may iesolve. In
cases of uiticaiia/angioedema oi eaily Stevens-
Johnson syndiome (SJS)/TEN, the ACDE can
be life-thieatening, and the diug should be
discontinued.
AC0 8 0kC IF
Aotretrovra| Iherapy (AkI)
Diug hypeisensitivity commonly occuis with
the nonnucleotide ieveise tiansciiptase inhib-
itois (nNRTIs) neviiapine, delaviidine, and
efaviienz; the nucleotide ieveise tiansciiptase
inhibitoi (NRTI) abacavii; and the piotease
inhibitoi ampienavii. ART hypeisensitivity is
manifested by exanthematous/moibillifoim
eiuptions (>95%); 20% of neviiapine-tieated
patients expeiience iash, most commonly an
exanthematous eiuption and iaiely SJS, ie-
quiiing diug discontinuation. Between 18 and
50% of delaviidine-tieated patients expeiience
iash. Appioximately one-half of cases of ART
hypeisensitivity iesolve despite continuation of
theiapy. Diug theiapy should be discontinued if
the following occui: mucosal involvement, blis-
teiing, exfoliation, clinically significant hepatic
dysfunction, fevei >39C, oi intoleiable fevei
oi piuiitus. Rechallenge with abacavii has been
associated with seveial deaths.
Iodvavr Has a ietinoid-like effect. Cheilitis
(57%); diffuse diyness and piuiitus; asteatotic
deimatitis on the tiunk, aims, and thighs;
scalp defluvium; pyogenic gianulomas, single
oi multiple.
Idovudoe (Iv0, AII) Longitudinal melano-
nychia, biown-black longitudinal stieaks in the
nail plate, occui in up to 40% of those tieated,
moie commonly in blacks than in Latinos oi
whites. Melanonychia aie usually noted in the
fingeis- and/oi toenails within 4-8 weeks aftei
initiation of theiapy (Fig. 31-4). Pigmented mac-
ules of mucous membianes common, occuiiing
moie commonly in moie heavily melanized
individuals. Diffuse hypeipigmentation mim-
icking piimaiy adienal insufficiency iepoited.
(Melanonychia and mucocutaneous pigmenta-
tion have also been iepoited with administia-
tion of hydioxyuiea in HIV/AIDS disease.)
oIuvrtde Fiist of a new class of antiietio-
viial agents foi the tieatment of HIV/AIDS-1
infection, called [uson n||ors . The most
common type of ACDE is injection site ieac-
tions, occuiiing in up to 98% of patients.
Many of these lesions aie symptomatic. Le-
sional biopsy specimens show an inflammatoiy
iesponse consistent with a localized hypeisen-
sitivity ieaction, iesembling that of gianuloma
annulaie and the iecently desciibed inteistitial
gianulomatous diug ieaction.
0ther 0rus
Irmethoprm-Su|Iamethoxato|e Between 50
and 60% of those tieated with IV TMP-SMX
develop an exanthematous eiuption (often as-
sociated with fevei) 1 to 2 weeks aftei staiting
theiapy (incidence 10 times gieatei than that
in the geneial population). Successful desensi-
tization has been accomplished in patients with
piioi exanthematous/moibillifoim oi uiticaiial
ieactions to TMP-SMX, sulfadiazine, and dap-
sone. Coadministiation of glucocoiticoids with
TMP-SMX ieduces incidence of ACDE. The oc-
cuiience of adveise ieactions to TMP-SMX has
also been noted to be associated with moie iapid
decline in CD4 cell counts. Sulfa diugs (sulfadi-
azine, TMP-SMX, sulfadoxine-pyiimethamine)
can also cause seveie bullous eiuptions. Sulfa
diugs aie the most common cause of TEN, the
incidence being 375 times highei than expected.
TEN occuis most commonly in those with ad-
vanced HIV/AIDS disease; 21% moitality iate.
0ra| C|ucocortcods Conceins: incieased im-
munosuppiession with exaceibations of op-
poitunistic infections and neoplasms such as
Kaposi saicoma oi HSV infections. Piednisone
is usually well toleiated and safe, especially
when given foi only 1-2 weeks.
Foscaroet (Irsodum FhosphoooIormate)
Causes painful, penile eiosions and/oi ulceis in
30% of patients undeigoing high-dose induc-
tion theiapy foi CMV ietinitis, 7-24 days aftei
staiting tieatment. Ulceiation caused by high
uiinaiy concentiation of the uiinaiy metabo-
lites of foscainet. Hypeihydiation ieduces the
iisk of ulceiation; in some cases, the diug must
be discontinued foi the ulceis to heal.
Aotretrovra| 0rus
C|asses oI Aotretrovra| 0rus
Nucleoside and nucleotide ieveise tian-
sciiptase inhibitois (NRTIs) inhibit ieveise
tiansciiption by being incoipoiated into the
newly synthesized viial DNA and pieventing
its fuithei elongation.
Nonnucleoside ieveise tiansciiptase inhibi-
tois (nNRTIs) inhibit ieseive tiansciiptase
diiectly by binding to the enzyme and intei-
feiing with its function.
Piotease inhibitois (PIs) taiget viial assem-
bly by inhibiting the activity of piotease, an
enzyme used by HIV/AIDS to cleave nascent
pioteins foi final assembly of new viions.
Integiase inhibitois inhibit the enzyme inte-
giase, which is iesponsible foi integiation of
viial DNA into the DNA of the infected cell.
Theie aie seveial integiase inhibitois cui-
iently undei clinical tiial, and ialtegiavii be-
came the fiist to ieceive appioval by the U.S.
Food and Diug Administiation in Octobei
2007.
Entiy inhibitois (oi fusion inhibitois) in-
teifeie with binding, fusion, and entiy of
HIV/AIDS-1 to the host cell by blocking one
of seveial taigets. Maiaviioc and enfuviitide
aie the two cuiiently available agents in this
class.
Matuiation inhibitois inhibit the last step
in gag piocessing in which the viial capsid
polypiotein is cleaved, theieby blocking the
conveision of the polypiotein into the ma-
tuie capsid piotein (p24). Because these viial
paiticles have a defective coie, the viiions ie-
leased consist mainly of noninfectious paiti-
cles. Theie aie no diugs in this class cuiiently
available, though two aie undei investigation,
beviiimat2] and Vivecon.
Freva|eoce AFD was noted in HIV/AIDS dis-
ease piioi to ART; pievalence has incieased
diastically with widespiead use of ART.
to|oy Unknown. Occuis in HIV/AIDS-
infected individuals who have nevei been
tieated with ART.
ksk Factors Incieasing age, cuiient use of
ART (excluding nNRTIs), longei duiation of
ART. Combination theiapy using two NRTIs
and a PI is associated with moie seveie dis-
ease.
Su|tuaneous |oaro|y : Stavudine, especially
when given with didanosine, zidovudine, PIs (es-
pecially indinavii), age, Caucasian, male sex.
Vstera| o|esy : Age, Caucasian, male sex,
iestoiation of health (piioi wasting), effective
ART.
Aoc|+|ed W||| AkI.
\o|p|o|o|c +|uo|r+||||e. \+|,|u de|ee o| |+|
|ed|||||u||ou, |.e., |uc|e+ed .|ce|+| |+|, |u||+|o
|urp, dec|e+ed pe||p|e|+| u|cu|+ueou |+|.
\e|+|o||c ,ud|ore c|+|+c|e||/ed |, +|dor|u+|
o|e||,, |,pe|||||,ce||der|+, + |oW |||deu||,
||pop|o|e|u (n||) c|o|e|e|o| |e.e|, |,pe||eu|ou,
+ud |uu||u |e||+uce ( d|+|e|e re||||u).
\e|+|o||c :|c- +oc|+|ed W||| |uc|e+ed |||
|o| c+|d|o.+cu|+| d|e+e +ud d|+|e|e.
\o|p|o|o|c +|uo|r+||||e ||||, ||r+||/|u,
d|cor|o||, d|+|||||,, p,c|o|o|c+| ro|||d||,,
|educe +d|e|euce |o o| d|cou||uu+||ou o|
e||ec||.e AkI.
A8N0kMAIIIIS 0F FAI 0ISIkI8II0N (AF0)
Metabo|c Aboorma|tes o hIv[AI0S 0s-
ease Seen most often in the context of ART
(not exclusively):
Visceial adiposity and loss of abdominal and
peiipheial subcutaneous fat
Insulin iesistance
Dyslipidemia
FAIh0CNSIS
Unknown. Factois in pathogenesis: (1) multiple
diug-associated events in metabolic pathways
and in diffeient tissues; and (2) host piedispo-
sition: age, genetics, HIV/AIDS disease stage,
inflammatoiy states. Metabolic changes ielated
to diiect effects of PIs on glucose, insulin, lipids,
and fat, as well as effects of HIV/AIDS itself and
ielated cytokines.
FICk 31-4 Adverse cutaoeous dru reactoo: tdovudoe me|aoooycha A +2,e+|o|d ||+c| |er+|e
|e+u +u|||e||o.||+| ||e|+p, W||| /|do.ud|ue + rou|| p|e.|ou|,. ||o\|r+| u+|| |,pe|p|reu|+||ou |+ occu||ed
|u +|| ||ue|u+|| +ud co||e|+|e W||| |u|||+||ou o| /|do.ud|ue ||e|+p,. n,pe|p|reu|+||ou o| ||u +ud o|+| ruco+
c+u +|o occu| W||| /|do.ud|ue.
Patients with |oaro|y (significantly de-
cieased abdominal and mid-thigh subcutaneous
fat) have elevated levels of plasma tiiglyceiides.
Those with obesity/mixed condition (incieased
intiaabdominal fat) have elevated levels of
plasma insulin and C-peptide. Use of stavu-
dine coiielates with fat wasting in both NRTI
and PI gioups. NRTI-associated mitochondiial
toxicity leads to fat wasting. Stavudine-based
iegimens have a highei cumulative pievalence
of lipoatiophy than iegimens based on zido-
vudine, abacavii, oi tenofovii. Regimens based
on nelfinavii aie associated with moie iapid fat
loss than efaviienz. In geneial, thymidine-based
nucleoside analogues have been most associated
with lipoatiophy and piotease inhibitoi diugs
most associated with the metabolic syndiome.
Cosmetic disfiguiement, stigmatization. Atheio-
scleiotic caidiovasculai disease, type 2 diabetes.
Sko Fodos
Loss o[ su|tuaneous [a : Fiist noted seveial
months aftei successful ART with loss of sub-
cutaneous fat fiom face (Fig. 31-5) and ex-
tiemities (peiipheial lipoatiophy). Results in
an emaciated appeaiance. Face: loss of buccal
fat, tiiangulai depiession below cheekbone, de-
piession of temples, appeaiance of piotubeiant
mouth because of suiiounding atiophy. Rated
as mild, modeiate, seveie. Extiemities: aims,
buttocks, legs; piominent veins.
Cenra| aJosy : Inciease in subcutaneous
fat in uppei back and posteiioi neck, i.e., buf-
falo hump (Fig. 31-5); enlaigement of bieasts.
Inciease in visceial intiaabdominal fate (pio-
tease paunch oi ciix belly").
Centru| AdIpvsIty/LIpvhypertrvphy Occuis
moie commonly at seveial anatomic locations
(86%) than in one location (14%). The doi-
sothoiacic fat pad becomes hypeitiophied to
a vaiiable extent (6%) (Fig. 31-5), fiom mild
to seveie (up to 5-10 cm thick); can extend
ciicumfeientially aiound the neck. Bieasts
may become enlaiged (20%) in males and
females. Abdominal giith can inciease due to
accumulation of intiaabdominal fat (60%).
Lipomas.
LIpvutrvphy Loss of subcutaneous fat in the
face (58%) (Fig. 31-5), giving a gaunt chaiac-
teiistic appeaiance. The uppei aims/shouldeis
(50%), buttocks/thighs (73%) also become de-
pleted of subcutaneous fat; supeificial veins aie
visible in these sites. Theie is also geneialized
loss of body fat.
MIred PresentutIvn Elements of cential adi-
posity and lipoatiophy aie piesent.
Systemc Fodos
Coionaiy aiteiy disease, peiipheial aiteiy dis-
ease. Osteoneciosis, osteopenia, osteopoiosis.
Hepatosteatosis. Lactic acidosis.
Fat Accumu|atoo Cushing disease, glucocoi-
ticoid theiapy, Launois-Bensaude syndiome,
scleiedema of diabetes mellitus.
Ipoatrophy Wasting of chionic disease, mal-
nutiition.
Dys|Jema. hypeitiiglyceiidemia, hypei-
cholesteiolemia. Insu|n ressante. glucose
intoleiance, diabetes mellitus. Lactic acidosis.
Metabolic abnoimalities associated with in-
cieased iisk of caidiovasculai disease.
0IACN0SIS
Clinical diagnosis.
C0kS AN0 Fk0CN0SIS
Lipodystiophy may piogiess foi the fiist 2 yeais
of ART, then stabilize. Change in ART may ie-
sult in impiovement of lipodystiophy.
MANACMNI
Foi most individuals with mild to modeiate
lipodystiophy, changes in body habitus aie
not significant. Howevei, with moie seveie
involvement, patients may iequest change in
ART in spite of excellent iesponse of HIV/AIDS
disease.
Metabo|c Syodrome Changing NRTI may
iesult in iegiession. Metabolic stiategies:
lipid-loweiing stiategies fibiic acids (clofibiate,
fenofibiate, gemfibiozil)], insulin-sensitizing
agents (metfoimin), stiategies to change fat dis-
tiibution (glitazones to inciease subcutaneous
fat, giowth hoimone to deciease visceial fat).
Ipoatrophy Remains the most difficult mani-
festation to manage. Replacing stavudine with
abacavii may iesult in impiovement in stavu-
dine-induced lipoatiophy. Foi facial lipoatio-
phy, vaiious fillei substances have been injected
into sites in the cheeks; howevei, the effects aie
evanescent and costly.
kapos Sarcoma (kS)
(See also Kaposi Saicoma," Section 20) Eaily
in the HIV/AIDS epidemic in the United States
and Euiope, 50% of homosexual men at the
time of initial AIDS diagnosis had KS. In HIV/
AIDS-infected individuals, the iisk foi KS is
20,000 times that of the geneial population,
300 times that of othei immunosuppiessed
hosts. In untieated HIV/AIDS disease, KS may
piogiess iapidly with extensive mucocutane-
ous and systemic involvement. KS in patients
successfully tieated with ART does not occui,
E+||, |u n|\/A||' d|e+e W|eu |rruue |uuc||ou
| |e|+||.e|, |u|+c|, corrou de|r+|oe, +d.e|e
cu|+ueou d|u e|up||ou, +ud |u|ec||ou p|eeu|
+ |,p|c+| c||u|c+| r+u||e|+||ou, |+.e ||e uu+|
cou|e, +ud |epoud |o |+ud+|d ||e|+p|e.
w||| p|o|e|.e dec||ue |u |rruue |uuc||ou,
e+c| o| ||ee c|+|+c|e||||c o| + d|e+e c+u |e
|||||u|, +||e|ed.
w||| e||ec||.e r+u+ereu| W||| AkI +ud |r
ruue |ecou|||u||ou, ||e d|e+e e|||e| do uo|
occu|, |eo|.e W|||ou| pec|||c ||e|+p,, o| |epoud
ro|e |e+d||, |o ||e|+p,.
vAkIAII0NS 0F C0MM0N MC0CIAN0S 0IS0k0kS
IN hIv[AI0S 0ISAS
iesolves without specific theiapy othei than
immune ieconstitution, oi iesponds bettei to
chemotheiapies.
Noome|aooma Sko Caocers
As in solid oigan tiansplant iecipients, the inci-
dence of ultiaviolet light-induced invasive SCC
is iepoited to be incieased in HIV/AIDS of skin
phototypes I to III with much UVL exposuie
duiing eaily decades of life. SCC can be quite
aggiessive, invading locally, giowing iapidly,
and metastasizing by lymphatics and blood,
with incieased moibidity and moitality.
FICk 31-5 Fat redstrbutoo A 5,e+|o|d r+|e W||| +d.+uced n|\/A||' ||e+|ed |o| r+u, ,e+| W|||
AkI |+ |||||u ||po||op|, o| ||e |+ce +ud '|u||+|o |urp o| ||e uppe| |+c| +ud ce|.|c+| uec|. I|e |+c|+| c|+ue
+|e d|||uc||.e +ud ||r+||/|u.
Aphthous Stomatts
(See also Aphthous Ulceis," Section 34) Re-
cuiient aphthous ulceiations may occui moie
fiequently, become laigei (often >1 cm), and/oi
become chionic with advanced HIV/AIDS dis-
ease. Ulceis may be quite extensive and/oi mul-
tiple; commonly involving the tongue, gingiva,
lips, and esophagus; at times causing seveie
odynophagia with iapid weight loss. Intia-
lesional tiiamcinolone and/oi a 1- to 2-week
tapeied couise of piednisone (70 to 0 mg). In ie-
sistant cases, thalidomide is an effective agent.
Staphy|ococcus Aureus IoIectoo
(See also Impetigo and Ecthyma," Abscess,
Fuiuncle, and Caibuncle," and Eiysipelas and
Cellulitis," Section 24) S. aureus is the most
common cutaneous bacteiial pathogen in HIV/
AIDS disease. The nasal caiiiage iate of S. au-
reus is 50%, twice that of HIV/AIDS-seionega-
tive contiol gioups. In most instances, S. aureus
infections aie typical, piesenting as piimaiy
infections (folliculitis, fuiuncles, caibuncles),
secondaiily impetiginized lesions (excoiiations,
eczema, scabies, heipetic ulcei, Kaposi saicoma),
cellulitis, oi venous access device infections, all
of which can be complicated by bacteiemia and
disseminated infection. Methicillin-iesistant S.
aureus (MRSA) infections, if not diagnosed
with cultuie and sensitives, may be moie seveie
because of delay in initiation of effective anti-
MRSA theiapy (Figs. 31-6, 31-7).
0ermatophytoses
(See also Deimatophytoses," Section 25, and
Fungal Infections: Onychomycosis," Section 33)
Epideimal deimatophytosis can be extensive,
iecuiient, and difficult to eiadicate. Pioximal
subungual onychomycosis occuis in advanced
HIV/AIDS disease, piesents as a chalky-white
discoloiation of the undeisuiface of the pioxi-
mal nail plate, and is an indication foi HIV/
AIDS seiotesting.
Mucosa| Caoddass
(See also Candidiasis," Section 25) Mucosal
candidiasis affecting the uppei aeiodigestive
tiacts and/oi vulvovagina is common in HIV/
AIDS disease. Oiophaiyngeal candidiasis, the
most common piesentation, is often the initial
manifestation of HIV/AIDS disease and is a
maikei foi disease piogiession. Esophageal and
FICk 31-6 Furuoc|e wth ce||u|ts: MkSA A !3,e+|o|d r+|e W||| n|\/A||' +ud + p+|u|u| |u|uuc|e
W+ ||e+|ed uuucce|u||, W||| e.e|+| o|+| +u||||o||c, ||e |e|ou |+d uo| |eeu |uc|ed +ud d|+|ued uo| cu||u|ed.
w|eu ||e |e|ou W+ |uc|ed +ud d|+|ued +ud cu||u|e o||+|ued, \k'A W+ |o|+|ed, eu|||.e ou|, |o ||ue/o||d +ud
.+ucor,c|u. I|e |u|ec||ou |eo|.ed W||| o|+| ||ue/o||d o00 r B|| |o| 1 d+,.
SCII0N 31 nu\A| kEIk0\|kA| |||ECI|0|' A|| \uC0CuIA|E0u' \A|||E'IAI|0|' 0| n|\/A||' ||'EA'E 95T
FICk 31-T 8otryomycoss: MkSA A !5,e+|o|d W||| + p+|u|u| |e|ou ou ||e |||| |u||oc| |o| 0 rou||
|+d |eeu ||e+|ed uuucce|u||, W||| o|+| +u||||o||c. 4. A |+|e ||uc|u+u| +|ce | eeu ou ||e |u||oc|. B. |uuc|
||op|e We|e o||+|ued +| e|||e| po|e o| ||e +|ce, pu d|+|ued, +ud cu||u|ed. w|||e '|+|u We|e o|e|.ed |u
||e d|+|u|u pu. I|e p|o|e |oW ||e e\|eu| o| ||e +|ce c+.||,. B|op, pec|reu |oWed co|ou|e o| |+r
po|||.e cocc| ||+| We|e ||e '|+|u .|u+||/ed r+c|ocop|c+||,. Cu||u|e |epo||ed \k'A, eu|||.e |o ||ue/o||d +ud
.+ucor,c|u. I|e |u|ec||ou |eo|.ed W||| o|+| ||ue/o||d o00 r B|| |o| 0 d+,.
B
4
tiacheobionchial candidiasis occui in advanced
HIV/AIDS disease and aie AIDS-defining con-
ditions. The incidence of cutaneous candidiasis
may be somewhat incieased; with insulin iesist-
ance associated with ART, balanoposthitis can be
seen. In young childien, chionic candidal paio-
nychia and nail dystiophy aie seen fiequently.
0ssemoated Fuoa| IoIectoo
(See also Disseminated Ciyptococcosis," His-
toplasmosis," and Disseminated Coccidioid-
omycosis," Section 25) Latent pulmonaiy fungal
infections with Cryotottus neo[ormans , Cot-
tJoJes mms, Hso|asma tasu|aum, and
Pent||um marne[[e can be ieactivated in HIV/
AIDS- infected individuals and disseminated to
the skin and othei oigans. The most common
cutaneous piesentation of disseminated infec-
tion is molluscum contagiosum-like lesions on
the face; othei lesions such as nodules, pustules,
ulceis, abscesses, oi a papulosquamous eiuption
iesembling guttate psoiiasis (seen with histo-
plasmosis) also occui.
herpes Smp|ex vrus IoIectoo
(See also Heipes Simplex Viius: Infections As-
sociated with Systemic Immunocompiomise,"
Section 27) Reactivated HSV-1 oi HSV-2 infec-
tion is one of the most common viial OIs of
HIV/AIDS disease. Most HIV/AIDS-infected
peisons aie HSV-2 seiopositive. Most ieacti-
vation is subclinical. Anogenital ieactivation
is paiticulaily fiequent. With incieasing im-
munodeficiency, eaily lesions piesent with eio-
sions oi ulceiations due to epideimal neciosis
without vesicle foimation. Untieated, these le-
sions may evolve to laige, painful ulceis with
iolled maigins in the oiophaiynx, esophagus,
and anogenitalia. Tieatment of HSV in dually
infected individuals ieduces genital and plasma
HIV/AIDS RNA levels.
varce||a-Ioster vrus (vIv) IoIectoo
(See also Vaiicella-Zostei Viius Infections in
the Immunocompiomised Host," Section 27)
Piimaiy VZV infection (vaiicella oi chicken-
pox) in HIV/AIDS disease can be seveie,
piolonged, and complicated by visceial VZV
infection, bacteiial supeiinfection, and death.
Heipes zostei (HZ) occuis in 25% of HIV/
AIDS-infected peisons duiing the couise of
theii HIV/AIDS, associated with modest decline
in immune function. Cutaneous dissemination
of HZ is ielatively common; howevei, visceial
involvement is iaie. With incieasing immuno-
deficiency, VZV infection can piesent clinically
as peisistent (chionic) deimatomal veiiucous
lesions; one oi moie chionic painful ulceis
oi ecthymatous lesions within a deimatome;
ecthymatous lesion(s), ulcei(s), oi nodule(s).
Untieated, these lesions peisist foi months. HZ
can iecui within the same deimatome(s) oi in
othei deimatomes. VZV can infect the CNS
causing a iapidly piogiessive choiioietinitis
with acute ietinal neciosis, chionic encephalitis,
myelitis, iadiculitis, oi meningitis. Extensive HZ
may heal with hypeitiophic oi keloidal scai.
Mo||uscum Cootaosum
(See also Molluscum Contagiosum," Section
27) In HIV/AIDS-infected individuals, mol-
luscum contagiosum has up to an 18% pieva-
lence; the seveiity of the infection is a maikei
foi advanced immunodeficiency. Patients may
have multiple small papules oi nodules oi laige
tumois, >1 cm in diametei, most commonly
aiising on the face (Fig. 31-8), especially the
beaid aiea, the neck, and inteitiiginous sites.
Shaving is a majoi factoi in the facial spiead
of mollusca and should be avoided if possible.
Cystlike mollusca occui on the eais. Occasion-
ally, mollusca can aiise on the non-haii-beaiing
skin of the palms/soles.
humao Fap||omavrus (hFv) IoIectoo
(See also Human Papillomaviius: Mucosal
Infections," Section 30) With advancing im-
munodeficiency, cutaneous and/oi mucosal
waits can become extensive and iefiactoiy to
tieatment. Of moie concein, howevei, HPV-
induced intiaepithelial neoplasia, teimed squa-
mous nrae|e|a| |eson (SIL), is a piecuisoi
to invasive SCC (Fig. 31-9), aiising most often
on the ceivix, vulva, penis, peiineum, and anus.
In HIV/AIDS-infected females, the incidence of
ceivical SIL is six to eight times that of contiols.
The cuiient tiend towaid longei median sui-
vival of patients with advanced HIV/AIDS may
lead to an incieased incidence of HPV-associ-
ated neoplasia and invasive SCC in the futuie.
SIL on the exteinal genitalia, peiineum, oi anus
is best managed with local theiapies such as
imiquimod cieam, ciyosuigeiy, electiosuigeiy,
oi lasei suigeiy iathei than with aggiessive
suigical excision.
FICk 31-8 Mo||uscum cootaosum, cooI|ueot: Iace A 5,e+|o|d |er+|e W||| 4. e\|eu|.e +ud cou
||ueu| |+c|+| uodu|e. |e|ou eu|+|ed |u p||e o| c|,ou|e|,. B. A|| |e|ou |eo|.ed W||| e|ec||ode|c+||ou.
4 B
FICk 31-9 hFv oIectoo oI peroeum: Coody|oma acumoatum aod ovasve squamous ce||
carcooma A !3,e+|o|d r+|e W||| + |||o|, o| pe||ue+| +ud pe||+u+| coud,|or+|+ W+ poo||, corp||+u| W|||
AkI. 'c|o| e\c||ou o| ||e r+ W+ |epo||ed |o |oW |u.+|.e 'CC +|||u |u ||e |+e o| ||e |ue coud,|or+
|ou |uro|. E|ec||ode|c+||ou o| ||e |+e +| ||e ||re o| c|o| e\c||ou W+ cu|+||.e. 0u |ee\c||ou, uo |e|du+|
'CC W+ de|ec|ed
Syph|s (See also Syphilis," Section 30)
The clinical couise of syphilis in HIV/AIDS-
infected individuals is most often the same as
in the noimal host. Howevei, an acceleiated
couise with the development of neuiosyphilis
oi teitiaiy syphilis has been iepoited within
months of initial syphilitic infection.
Resouices available on the Woild Wide
Web on HIV/AIDS disease aie shown in
Table 31-3.
IA8I 31-3 Resources Avai|ab|e on the wor|d wide web on h|V/A|0S
|||//+++c!||. A||' |u|o, + e|.|ce o| ||e u.'. |ep+||reu| o| ne+||| +ud nur+u 'e|.|ce, po|
|ede|+||, +pp|o.ed ||e+|reu| u|de||ue |o| n|\ +ud A||', p|o.|de
|u|o|r+||ou ou |ede|+||, |uuded +ud p||.+|e|, |uuded c||u|c+| |||+| +ud C|C
pu|||c+||ou +ud d+|+
|||//+++:!: upd+|e ou ep|der|o|o|c d+|+ ||or ||e C|C
|0IE. C|C, Ceu|e| |o| ||e+e Cou||o| +ud ||e.eu||ou.
| A k I | \
5KIN 5ICN5 OF HAIk,
NAIL, AND MUCO5AL
DI5OkDEk5
962
Cataeo Apoptosis-diiven phase between
telogen and anagen phase. Duiation: few weeks.
xoeo Active piocess of haii shaft shedding.
II|uvum Piocess of incieased daily haii shaft
shedding (noimal scalp 25-100 haiis).
har Co|or
Caotes Giaying of haii.
Fo|oss Localized white haii.
Iypes oI har
Iaouo har Soft fine pigmented haii that
coveis much of fetus; usually shed befoie biith.
Between vellus and teiminal haii in length and
foim.
ve||us har Latin foi fleece." Fine, nonpig-
mented haii (peach fuzz) that coveis the body
of childien and adults; giowth not affected by
hoimones. Beaid haii in women and childien is
vellus. Genetically deteimined to pioduce veiy
small (but functionally fully active cycling) haii
follicles located in the deimis; thin ( 0.03 mm
in diametei), shoit, often nonpigmented, usu-
ally nonmedullated haii shaft.
S E C I | 0 N 5 2
0IS0k0kS 0F hAIk
F0IIICIS
AN0 kIAI0 0IS0k0kS
n+|| |o|||c|e p|oduce p|reu|ed |e|+||u |||e|.
nur+u |+|| |+ |||||e .e|||+| |uuc||ou.
Cou||||u|e |o + p,c|o|o|c+| pe|cep||ou o|
|e+u|, +ud +|||+c||.eue.
I+c|||e eu+||ou
||o|ec| ||e c+|p, |+ce, +ud uec| ||or u\ o|+|
|+d|+||ou
keduce |e+| |o |||ou| ||e c+|p
|,c|o|o, o| |+||. A||e|+||ou o| ||e 'uo|r+|
qu+u|||, o| |+|| | o||eu +oc|+|ed W||| p|o|ouud
p,c|o|o|c+| |rp+c|. |o o| c+|p |+|| | cou
|de|ed +|uo|r+| |u r+u, oc|e||e, +oc|+||u
|+|d|u W||| o|d +e (p+||e|u |+|| |o) o| |r
p+||ed |e+||| (c|ero||e|+p,).
E\ce |+|| ou ||e |+ce (|||u||r, |,pe||||c|o|)
+ud e\||er|||e o| Woreu | o||eu cou|de|ed
uu+|||+c||.e.
B||||ou o| do||+| +|e peu| +uuu+||, |u |udu|||
+||/ed couu|||e |o c+|e |o| |+|| +ud pe|ce|.ed
+|uo|r+||||e.
CI0SSAk 0F IkMS
har Fo||c|e Cyc|e
Life-long cyclic tiansfoimations of the haii fol-
licle, which begins in uteio (Fig. 32-1).
Aoaeo Giowth phase; lasts vaiiable peiiods
of time depending on body site, e.g., scalp,
eyebiows. Duiation: 1-6 yeais; aveiage 3 yeais;
vaiies with age; deteimines the ultimate length
of haii at a site. Scalp, beaid: long anagen. Eye-
biows, eyelashes, axillaiy/pubic haii: anagen is
shoit; telogen piolonged. Anagen haii matiix
has iapidly piolifeiating epithelial cells; exqui-
sitely sensitive to diugs, giowth factois, hoi-
mones, stiess; immunologic and physical injuiy.
Destiuction of epithelial stem cells iesults in
peimanent haii loss.
Aoaeo hars Haiis with pigmented malleable
pioximal ends.
Ie|oeo Peiiod of ielative quiescence, last vai-
iable peiiods of body site.
Ie|oeo hars Club haiis with depigmented
iounded pioximal ends.
8I0I0C 0F hAIk Ck0WIh CCIS
SCII0N 32 ||'0k|Ek' 0| nA|k |0|||C|E' A|| kE|AIE| ||'0k|Ek' 963
Iotermedate har Shows the chaiacteiistics of
vellus and teiminal haiis.
Iermoa| har Thick pigmented haii found
on scalp, beaid, axillae, pubic aiea; giowth
is influenced by hoimones. Eyebiow/eyelash
haii aie teiminal haiis. Pioduced by laige
haii follicles located in the subcutis; haiis aie
geneially >0.03 mm in diametei. 100,000 tei-
minal scalp haii follicles at biith; genetically
deteimined to pioduce long, thick pigmented
haiis.
har Ioss or Cao
A|opeca Haii loss, usually of the scalp.
Scarro A|opeca Cicatiicial/peimanent alo-
pecia. Iiieveisible loss of haii follicles with
disappeaiance of folliculai oiifices and skin
atiophy.
hrsutsm Excessive and incieased haii giowth
in women in locations wheie the occuiience
of teiminal haii noimally is minimal oi ab-
sent. It iefeis to a male pattein of body haii
(andiogenic haii) and it is theiefoie piimaiily
of cosmetic and psychological concein. Hii-
sutism is a symptom iathei than a disease and
may be a sign of a moie seiious medical indica-
tion, especially if it develops well aftei pubeity.
hypertrchoss Incieased giowth of teiminal
haiis in an aiea wheie vellus haiis aie noimal.
N00CkIN0I0C 0F hAIk F0IIICIS
Haii follicles can vaiy in size undei the influ-
ence of andiogens, which inciease the size of
haii follicles in the beaid, chest, legs, and aims
but deciease the size of the haii follicles in the
tempoial iegions of the scalp; this shapes the
haiiline in men and many women.
The iesponse of the haii follicle to esoserone
and J|yJroesoserone (DHT) is undei genetic
contiol.
D|yJroesoserone causes giowth of the pios-
tate, giowth of teiminal haii, andiogenetic
alopecia, and acne.
Tesoserone causes giowth of axillaiy haii and
lowei pubic haii, as well as sex diive, giowth
of the phallus and sciotum, and speima-
togenesis.
FICk 32-1 har rowth cyc|e ||+|+rr+||c |ep|eeu|+||ou o| ||e c|+ue ||+| occu| |o ||e |o|||c|e +ud
|+|| |+|| du||u ||e |+|| |oW|| c,c|e. 4. Au+eu (|oW|| |+e), B. C+|+eu (deeue|+||.e |+e), C. Ie|oeu
(|e||u |+e). (Cou||e, o| |,uu \. K|e|u, \|.)
FAkI Iv 'K|| '|C|' 0| nA|k, |A||, \uC0'A| ||'0k|Ek' 964
har Fu|| Scalp is gently pulled. Noimally,
thiee to five haiis aie dislodged; shedding moie
haii suggests pathology.
Irchoram Deteimines the numbei of ana-
gen and telogen haiis and is made by epilating
(plucking) 50 haiis oi moie fiom the scalp with
a needleholdei and counting the numbei of
A|opec|+. d|||ue (|o|+|) |+|| |o
|+||u|e o| |+|| |o|||c|e de.e|opreu|. e.., |,po||d
|o||c ec|ode|r+| d,p|+|+
n+|| |+|| +|uo|r+||||e.
n+|| |+|| +|uo|r+||||e W||| |+|| ||e+|+e.
|||c|o|||e\| uodo+, |||c|oc|||, p||| |o|||,
|||c|o|||e\| |u.+|u+|+ ('|+r|oo |+||), rou|
|e||||\, |oc+||/ed +u|oor+| |ece|.e |,po|||
c|o|
n+|| |+|| +|uo|r+||||e W||| uu|u|, |+||.
uucor|+||e |+|| ,ud|ore, Woo||, |+||
A|uo|r+||||e o| |+|| |o|||c|e c,c||u. '|o|| +u+eu
,ud|ore, +cu|e |e|oeu e|||u.|ur, c||ou|c
|e|oeu e|||u.|ur, +u+eu e|||u.|ur, |ooe +u+
eu ,ud|ore
A|opec|+ +|e+|+
|ouc+|||u |+|| |o, p|oduc||ou dec||ue. |+||e|u
|+|| |o
I|+ur+||c |+|| |o. ||eu|e +|opec|+, |||c|o|||
|or+u|+, ||+c||ou +|opec|+, ||ue+ c+p|||
|||r+|, o| +cqu||ed |+|| |+|| +|uo|r+||||e.
A|uo|r+||||, o| c,c||u (+|opec|+ +|e+|+, ,p||||),
c+|p coud|||ou +oc|+|ed W||| |oc+| |+|| |o
(p||,||+| +r|+u|+e+, e.e|e c+|p po||+| o|
e|o|||e|c de|r+||||, c+|p |u.o|.ereu| W|||
de|r+|or,o|||, cu|+ueou I ce|| |,rp|or+,
|+ue||+u ce|| ||||oc,|o|)
C|c+|||c|+| +|opec|+ (de||uc||ou o| ||e |o|||c|e)
(ee |e|oW).
|||r+|, c|c+|||c|+| +|opec|+ |upu e|,||er+|ou,
||c|eu p|+uop||+|| (C|+|+r|||||e ,ud|ore,
||ou|+| ||||o|u +|opec|+), peudope|+de o|
B|ocq, o||e| ceu||+| ceu||||u+| c|c+|||c|+| +|o
pec|+ ( |o| cor| +|opec|+ o| |o|||cu|+| deeue|+
||ou ,ud|ore) , +|opec|+ o| |o|||cu|+| ruc|uo|,
|o|||cu|||| dec+|.+u, d|ec||u |o|||cu||||, +cue
|e|o|d+|| uuc|+e, +cue uec|o||c+, e|o|.e pu
|u|+| de|r+|o||.
'ecoud+|, c|c+|||c|+| +|opec|+. |e|ed||+|, o|
de.e|opreu| p|o||er.
Coueu||+| c|c+|||c|+| +|opec|+
CIASSIFICAII0N 0F AI0FCIA |C|9 . 10+.0
|C|0 . |o!|oo
FAIh0CNSIS AFFk0ACh I0 0IACN0SIS 0F
0IS0k0kS 0F hAIk AN0 hAIk F0IIICIS
Pathogeoet|c Pr|oc|p|e 0||o|ca| IIect (xamp|es)
|||u||ed |+|| |o|||c|e c,c||u E|||u.|ur (|e|oeu e|||u.|ur, +|opec|+ +|e+|+, +ud|oeue||c
+|opec|+, c|ero||e|+p,|uduced +|opec|+)
uuW+u|ed |+|| |o|||c|e ||+u|o|r+||ou |+||e|ued |+|| |o (+ud|oeue||c +|opec|+)
n||u||r
n,pe||||c|o|
|e|ec||.e |+|| |o|||c|e |eeue|+||ou C|c+|||c|+| +|opec|+ (||c|eu p|+uop||+||, ||+c||ou +|opec|+,
|+d|+||ou|uduced +|opec|+, |o|||cu|||| dec+|.+u, c||ou|c
d|co|d |upu e|,||er+|ou)
'||uc|u|+| |+|| |+|| de|ec| n+|| |+|| d|o|de| (rou||e||||\, p||| |o|||, |||c|o|||od,||op|,)
|rp|ope|/r||u |+|| |o|||c|e de.e|opreu| Ap|+|+ cu|| coueu||+
Cor||u+||ou o| ||e +|o.e Ec|ode|r+| d,p|+|+
|o|e ||+| |u|ec||ou o| p|,|c+| +eu| (e.., |+p|,|ococc|, de|r+|op|,|e, |e|pe-.+||ce||+ /o|e| .||u, |ou|/|u |+d|+||ou, ||+ur+) o| d|u ||+|
c+u |e+d |o +|opec|+ do o |, |uduc|u oue o| e.e|+| o| ||e +|o.e p+||oeue| p+||W+, (e.., d||u||ed |+|| |o|||c|e c,c||u, ||uc|u|+| |+||
|+|| de|ec|, de|ec||.e |+|| |o|||c|e |eeue|+||ou).
Anagen haiis: giowing haiis with a long
enciicling haii sheath.
Telogen (club) haiis: iesting haiis with an in-
nei ioot sheath and ioots usually laigest at the
base.
Noimally, 80-90% of haiis aie in anagen
phase.
Sca|p 8opsy Offeis insight into pathogenesis
of alopecia.
h0hS0A88|h6 AL0P0|A
C+u occu| |o|+||, o| |e |oc+| ( I+||e !2).
|o o| c+|p |+|| |+ ||e ro| core||c |rp+c| ou
|ud|.|du+|.
'|edd|u o| |+|| | |e|red -|||. o| !-||
. , +ud ||e |eu|||u coud|||ou | c+||ed c|
-:c (C|ee| +|ope||+, "|+|due").
|ud|.|du+| +|e o||eu +W+|e o| +ud .e|, couce|ued
+|ou| u|||e |||uu|u o| ||e |+||.
A|opec|+ c|+|||ed |u|o.
|::c|:c| 1|-:c: |o c||u|c+| |u o| ||ue
|u||+rr+||ou, c+|||u, o| +||op|, o| ||u .
C:c|:c| c|-:c . E.|deuce o| ||ue de||uc||ou
uc| + |u||+rr+||ou, +||op|,, +ud c+|||u |
+pp+|eu|.
hAIk I0SS: AI0FCIA |C|9 . 10+.0
|C|0 . |o!|oo
IA8I 32-1 Etio|oy of hair Loss
0IIuse (|oba|) har |oss (uouc+|||u)
|+||u|e o| |o|||c|e p|oduc||ou
n+|| |+|| +|uo|r+|||,
A|uo|r+|||, o| c,c||u (|edd|u)
Ie|oeu e|||u.|ur
Au+eu e|||u.|ur
|ooe +u+eu ,ud|ore
A|opec|+ +|e+|+
Foca| (patchy, |oca|ted) har |oss
|ouc+|||u
||oduc||ou dec||ue
I||+uu|+| +|opec|+
|+||e|u |+|| |o (+ud|oeue||c +|opec|+)
n+|| ||e+|+e
I||c|o||||or+u|+
I|+c||ou +|opec|+
|u|ec||ou (||ue+ c+p|||)
|||r+|, o| +cqu||ed |+|| |+|| +|uo|r+|||,
uu|u|, |+||
A|uo|r+|||, o| c,c||u
A|opec|+ +|e+|+
',p||||
'c+|||u (c|c+|||c|+|) +|opec|+
(ee ''c+|||u A|opec|+, |e|oW)
|+||e|u |+|| |o | ||e ro| corrou |,pe o|
p|o|e|.e |+|d|u.
0ccu| |||ou| ||e cor||ued e||ec| o|.
Ceue||c p|ed|po|||ou
Ac||ou o| +ud|oeu ou c+|p |+|| |o|||c|e
|u r+|e, p+||e|u/e\|eu| o| |+|| |o |u r+|e .+|
|e ||or |||erpo|+| |ece|ou, |o ||ou|+| +ud/o|
.e||e\ |||uu|u, |o |o o| +|| |+|| e\cep| ||+| +|ou
||e occ|p||+| +ud |erpo|+| r+||u ('n|ppoc|+||c
W|e+||).
',, . \+|e. Aud|oeue||c +|opec|+ (ACA),
r+|ep+||e|u |+|due, corrou |+|due.
|er+|e. ne|ed||+|, |||uu|u, |er+|ep+||e|u
|+|due.
FAIIkN hAIk I0SS
to|oy Combined effects of andiogen on
genetically piedisposed haii follicles. Genet-
ics: (1) autosomal dominant and/oi polygenic;
(2) inheiited fiom eithei oi both paients.
Ae oI 0oset
Ma|es : May begin any time aftei pubeity,
as eaily as the second decade; often fully
expiessed in 40s.
Fema|es : Latei-in about 40% occuis in the
sixth decade.
Sex Males >> females.
CIASSIFICAII0N
n+r|||ou
c|+|||ed r+|ep+||e|u |+|| |o |u|o

|+e (||. !22 1 ).
I,pe |. |o o| |+|| +|ou ||ou|+| r+||u.
I,pe ||. |uc|e+|u ||ou|+| |+|| |o + We|| +
oue| o| |o o| occ|p||+| (.e||e\ o| c|oWu).
I,pe |||, |\, +ud \. |uc|e+|u |+|| |o |u |o||
|e|ou W||| e.eu|u+| cou||ueu| +ud corp|e|e
|+|d|u o| |op o| c+|p W||| p+||u o| |de
|udW|
2
c|+|||ed |+|| |o |u |er+|e
(||. !22 3 ).
FAIh0CNSIS
Testosteione is conveited to (DHT) by 5 -ie-
ductase (5 -R). Two isozymes of 5 -R occui:
type I and type II.
Type I 5 -R is localized to sebaceous glands
(face, scalp), chest/back skin/livei, adienal
gland, kidney.
Type II 5 -R is localized to scalp haii fol-
licle, beaid, chest skin, livei, seminal vesicle,
piostate, epididymis, foieskin/sciotum.
Finasteiide inhibits conveision of testostei-
one to DHT by type II 5 -R.
Role of testosteione: (1) pienatal: inteinal sex
oigan development of male fetus; (2) postnatal:
speimatogenesis, libido, muscle/bone mass.
Role of DHT: (1) pienatal: exteinal genitalia
development in male fetus; (2) postnatal:
scalp haii loss, piostate enlaigement.
Clinical featuies of type II 5 -R deficiency in
men: ambiguous genitalia at biith, viiilized
at pubeity; undeideveloped piostate, no en-
laigement with age; otheiwise healthy (noi-
mal libido aftei pubeity, noimal bone/muscle
mass aftei pubeity); spaise facial/body haii;
no scalp haii loss with age.
In males, testosteione pioduced by the testes
is the majoi andiogen. In females, andiosten-
edione and dehydioepiandiosteione sulfate
aie the majoi peiipheial andiogens.
In genetically piedisposed individuals, DHT
causes teiminal follicles to tiansfoim into
vellus-like follicles, which in tuin undeigo
atiophy. Duiing successive folliculai cycles,
haiis pioduced aie of shoitei length and of
decieasing diametei.
Conveisely, andiogens induce vellus-to-tei-
minal follicle pioduction of secondaiy sexual
haii.
Sko Symptoms Most patients piesent with com-
plaints of giadually thinning haii oi baldness.
In males (Figs. 32-3, 32-4), theie is a ieceding
anteiioi haiiline, especially in the paiietal
iegions, which iesults in an M-shaped ieces-
sion. Following this, a bald spot may appeai
on the veitex. If AGA piogiesses iapidly, some
patients also complain of incieased falling out
of haii.
In females, paiietal and tempoial iecession
is not usually a majoi featuie, and haii loss
follows a pattein depicted in Fig. 32-5; seveie
thinning is not common.
The cosmetic appeaiance of pattein haii loss is
veiy distuibing to many peisons owing to the
high value that oui society places on a healthy
head of haii."
Systems kevew In young women, manifesta-
tions of andiogen excess should be sought as
significant:
Acne
Hiisutism
Iiiegulai menses
Viiilization.
Howevei, most women with pattein haii loss
aie endociinologically noimal.
Sko Fodos Scalp skin is noimal.
In young women, look foi signs of viiilization
(acne, excess facial oi body haii, male-pattein
escutcheon).
With advanced pattein haii loss, scalp is
smooth and shiny; oiifices of follicles aie
baiely peiceptible with the unaided eye.
l| n+r|||ou. Ar l Au+| 1.+5, 9+.

2
E. |udW|. B| l |e|r+|o| 91.2+9, 911.
SCII0N 32 ||'0k|Ek' 0| nA|k |0|||C|E' A|| kE|AIE| ||'0k|Ek' 96T
har (Figs. 32-3 and 32-7) Haii in aieas
of pattein haii loss becomes finei in textuie
(shoitei in length, ieduced diametei). In time,
haii becomes vellus and eventually atiophies
completely.
DIstrIhutIvn
Males usually exhibit patteined loss in
the fiontotempoial and veitex aieas
(Figs. 32-3, 32-4). The end iesult may be only
a iim of iesidual haii on the lateial and pos-
teiioi scalp (Hippociatic wieath; Fig. 32-6).
In these iegions haii nevei falls out in pattein
haii loss. Paiadoxically, males with extensive
pattein haii loss may have excess giowth of
secondaiy sexual haii, i.e., axillae, pubic aiea,
chest, and beaid.
Females, including those who aie endociino-
logically noimal, also lose scalp haii accoiding
to the male pattein, but haii loss is fai less
pionounced. Often haii loss is moie diffuse
in women, following the pattein desciibed by
Ludwig (Figs. 32-23, 32-5).
Systemc Fodos In young women with AGA,
look foi signs of viiilization (clitoial hypeitio-
phy, acne, facial hiisutism) and, if piesent, iule
out endociine dysfunction.
0IIuse Nooscarro Sca|p A|opeca Diffuse
pattein of haii loss with alopecia aieata, te-
logen defluvium, secondaiy syphilis, systemic
lupus eiythematosus (SLE), iion deficiency,
hypothyioidism, hypeithyioidism, tiichotillo-
mania, seboiiheic deimatitis.
Irchoram In pattein haii loss, the eailiest
changes aie an inciease in the peicentage of
telogen haiis.
0ermatopatho|oy Abundance of telogen-
stage follicles is noted, associated with haii
follicles of decieasing size and eventually neaily
complete atiophy.
FICk 32-2 Aodroeoetc a|opeca: patteros o ma|es aod Iema|es 4. n+r|||ou c|+|||ed ||e e.e|
||, +ud p+||e|u o| |+|| |o |u r+|e |u|o |,pe | |o \. B. |udW| c|+|||ed |+|| |o |u |er+|e |u|o |,pe | |o |||.
hormooe Studes In women with haii loss and
evidence of incieased andiogens (menstiual
iiiegulaiities, infeitility, hiisutism, seveie cystic
acne, viiilization), deteimine:
Testosteione: total and fiee
Dehydioepiandiosteione sulfate (DHEAS)
Piolactin
0ther Studes Tieatable causes of thinning
haii should be excluded with measuiement of
thyioid-stimulating hoimone (TSH), T
4
, seium
iion, seium feiiitin, and/oi total iion-binding
capacity (TIBC), complete blood count (CBC),
antinucleai antibodies (ANA).
0IACN0SIS
Clinical diagnosis is made on the histoiy, pat-
tein of alopecia, and family incidence of AGA.
Skin biopsy may be necessaiy in some cases.
C0kS
The piogiession of alopecia is usually veiy
giadual, ovei yeais to decades.
FICk 32-3 Fattero har |oss: ma|e, ham|too type III A +o,e+|o|d r+|e W||| |||erpo|+| |ece|ou
o| |+||||ue +ud ||ou|+| |||uu|u o| |+||.
FICk 32-4 Fattero har |oss: ma|e, ham|too types Iv to v A !1,e+|o|d r+|e W||| |o o| |+|| |u
||e ||ou|o|erpo|+| +ud .e||e\ +|e+ |u + r+|e co||epoud|u |o n+r|||ou |,pe |\ +ud \.
FICk 32-5 Fattero har |oss: Iema|e, Iudw type II A oo,e+|o|d |er+|e W||| d|||ue |||uu|u o|
|+|| ou ||e c|oWu.
FAkI Iv 'K|| '|C|' 0| nA|k, |A||, \uC0'A| ||'0k|Ek' 9T0
MANACMNI
0ra| Foasterde 1 mg PO daily, competitively
inhibits type II 5 -R and thus the conveision of
testosteione to DHT; this iesults in lowei seium
and scalp levels of DHT. Finasteiide has no af-
finity foi andiogen ieceptois and theiefoie does
not block the impoitant actions of testosteione
(giowth of the phallus and sciotum, speima-
togenesis, libido). Most men may begin to see fiist
benefit in slowing haii loss as eaily as 3 months.
Aftei 6 months, theie is a iegiowth of teiminal
haii on the veitex and anteiioi mid-scalp. If the
diug is stopped, howevei, the haii that had giown
will be lost within 12 months. 2% of men taking
finasteiide iepoit deciease in libido and eiectile
function; these effects weie ieveisible when the
diug was stopped and disappeaied in two-thiids
of those who continued taking finasteiide.
Iopca| Mooxd| Topically applied minoxidil,
2% and 5% solution, may be helpful in ieduc-
ing iate of haii loss oi in paitially iestoiing lost
haii in both males and females.
Aotaodroeos In women with AGA who
have elevated adienal andiogens, spiionol-
actone, cypioteione acetate, flutamide, and
cimetidine bind to andiogen ieceptois and
block the action of DHT. These must not be
used in men.
harpece Wigs, toupees, piosthetics; haii
weaves.
Surca| Ireatmeot
Har rans|anaon Giafts of one oi two
follicles aie taken fiom andiogen-insensi-
tive haii sites (peiipheial occipital and
paiietal haiiy aieas) to bald andiogen-
sensitive scalp aieas.
Sta| reJuton/roaon [|as
FICk 32-6 Fattero har |oss: ma|e, ham|too type v wth ovasve squamous ce|| carcooma
(SCC) A o5,e+|o|d W|||e r+|e W||| e+||, oue| o| |+|d|u |+ e.e|e de|r+|o|e||o| W||| +c||u|c |e|+|oe, |u
||u 'CC, |u.+|.e 'CC, +ud ru|||p|e c+| +| e\c||ou ||e o| p||o| |u.+|.e 'CC. \e|+|+||c 'CC W+ de|ec|ed |u +
up|+c|+.|cu|+| |,rp| uode, p|eur+||e ||or + p||r+|, c+|p 'CC.
SCII0N 32 ||'0k|Ek' 0| nA|k |0|||C|E' A|| kE|AIE| ||'0k|Ek' 9T1
A |oc+||/ed |o o| |+|| |u |ouud o| o.+| +|e+ W|||
uo +pp+|eu| |u||+rr+||ou o| ||e ||u. \o| cor
rou ou c+|p.
|ouc+|||u, |+|| |o|||c|e |u|+c|, |+|| c+u |e|oW.
A core||c couce|u |o| ||e r+jo|||, o| p+||eu|.
C||u|c+| ||ud|u. n+|| |o |+u|u ||or o|||+|,
p+|c| |o corp|e|e |o o| +|| |e|r|u+| |+||.
||ouo|. ood |o| ||r||ed |u.o|.ereu|. |oo| |o|
e\|eu|.e |+|| |o.
\+u+ereu|. |u||+|e|ou+| |||+rc|uo|oue e||ec||.e
|o| ||r||ed uur|e| o| |e|ou.
|||p.//WWW.u++|.o|
to|oy Unknown. Association with othei
autoimmune diseases and immunophenotyp-
ing of lymphocytic infiltiate aiound haii bulbs
suggests an anti-haii bulb autoimmune pioc-
ess. 10-20% of peisons with alopecia aieata
(AA) have a familial histoiy of AA.
Ae oI 0oset Young adults (<25 yeais); childien
aie affected moie fiequently. Can occui at any age.
Freva|eoce Relatively common. 1.7% of the
U.S. population expeiiences at least one episode
of AA in a lifetime. Vaiies with geogiaphy and
ethnicity.
FAIh0CNSIS
Chionic oigan-specific autoimmune disease,
mediated by autoieactive T cells affecting haii
follicles and nails.
Associated autoimmune disoideis: Autoim-
mune thyioid disease in adults.
Folliculai damage occuis in anagen followed
by iapid tiansfoimation to catagen and to
telogen; then to dystiophic anagen status.
While the disease is active, follicles unable
to piogiess beyond eaily anagen and do not
develop noimal haii.
FICk 32-T Fattero har |oss: Iema|e, Iuw type III wth basa| ce|| carcooma (8CC) A o1,e+|
o|d C|ee| |er+|e W||| +d.+uced +|opec|+ o| ||e c|oWu W||| BCC +|||u W||||u ||.
AI0FCIA AkAIA
Sko Fodos Usually none. Possibly minimal
eiythema in aiea of haii loss.
har
Round patches of haii loss. Single oi mul-
tiple. May coalesce. Alopecia often shaiply
defined.
Alopecia, noimal-appeaiing skin with fol-
liculai openings piesent (Figs. 32-8 thiough
32-11).
Exclamation maik" haiis . Diagnostic bio-
ken-off stubby haiis (distal ends aie bioadei
than pioximal ends) (Fig. 32-8); seen at mai-
gins of haii loss aieas.
Scatteied, disciete aieas of alopecia (Fig.
32-9) oi confluent with total loss of scalp haii
(Fig. 32-10), oi geneialized loss of body haii
(including vellus haii).
Diffuse AA of scalp (nonciicumsciibed) gives
the appeaiance of thinned haii; can be dif-
ficult to diffeientiate fiom pattein haii loss
Folliculai stem cell is spaied; haii follicles aie
not destioyed.
White oi giaying haiis aie fiequently spaied;
with fulminant AA, peisons may expeiience
going giay oveinight."
0uratoo oI har Ioss Giadual ovei weeks to
months. Patches of AA can be stable and often
show spontaneous iegiowth ovei a peiiod of
seveial months; new patches may appeai while
otheis iesolve.
Sko Symptoms Individuals aie usually veiy
conceined about haii loss and potential foi
continued, piogiessive balding.
Assocated Fodos Autoimmune thyioiditis.
Down syndiome. Autoimmune polyendociin-
opathy-candidiasis-ectodeimal dysplasia syn-
diome.
FICk 32-8 A|opeca areata (AA) oI sca|p: so|tary |esoo Au +|e+ o| +|opec|+ W|||ou| c+||u, e|,
||er+, +||op|,, o| c+|||u ou ||e occ|p||+| c+|p. I|e |o||, ||o|euo|| |+|| |+|| (oc+||ed e\c|+r+||ou po|u|
|+||) +ppe+| + .e|, |o|| |u| ere||u ||or ||e |+|d c+|p.
of telogen effluvium, haii loss with thyioid
disease.
With iegiowth of haii, new haiis aie fine,
often white oi giay.
SItes v] PredI|ectIvn Scalp most commonly.
Any haii-beaiing aiea. Beaid, eyebiows, eye-
lashes, pubic haii.
|oeta areaa (): Solitaiy oi multiple
aieas of haii loss. (Figs. 32-8, 32-9)
oa|s (T): Total loss of teiminal scalp
haii. (Fig. 32-10)
unersa|s (U): Total loss of all teimi-
nal body and scalp haii. (Fig. 32-11)
O|ass: Bandlike pattein of haii loss ovei
peiipheiy of scalp .
Na|s Fine pitting (hammeied biass") of doi-
sal nail plate. Also: mottled lunula, tiachyony-
chia (iough nails), onychomadesis (sepaiation
of nail fiom matiix). (See also Section 33.)
Nooscarro A|opeca White-patch tinea capi-
tis, tiichotillomania, eaily scaiiing alopecia,
pattein haii loss, secondaiy syphilis (alopecia
aieolaiis) (moth-eaten" appeaiance in beaid
oi scalp).
Sero|oy ANA (to iule out SLE); iapid plasma
ieagin (RPR) test (to iule out secondaiy syphi-
lis).
k0h Freparatoo Rule out tinea capitis.
0ermatopatho|oy Acute lesions show pei-
ibulbai, peiivasculai, and outei ioot sheath
mononucleai cell infiltiate of T cells and
maciophages; folliculai dystiophy with ab-
noimal pigmentation and matiix degeneia-
tion.
FICk 32-9 A|opeca areata oI sca|p: mu|tp|e, exteosve |esoos A +o,e+|o|d r+|e W||| |eceu|
oue| o| +|opec|+ +|e+|+. \u|||p|e, cou||ueu|, |u.o|.ed ||e ou ||e c+|p W||| 'e\c|+r+||ou po|u| |+||. I|e+|reu|
W||| |u||+|e|ou+| |||+rc|uo|oue !.! r/r| W+ ucce|u|.
C0kS
Spontaneous iemission is common in patchy
AA but is less so with AAT oi AAU.
Pooi piognosis associated with onset in child-
hood, loss of body haii, nail involvement,
atopy, family histoiy of AA.
If occuiiing aftei pubeity, 80% iegiow haii.
With extensive AA, AAT, AAU, <10% iecovei
spontaneously.
Recuiiences of AA, howevei, aie fiequent.
Systemic glucocoiticoids oi cyclospoiine can
induce iemission of AA but do not altei the
couise.
MANACMNI
Tieatment diiected at inflammatoiy infiltiate
and giowth inhibitoi factois pioduced by in-
flammation. No cuiative tieatment is cuiiently
available. Tieatment foi AA is unsatisfactoiy.
In many cases, the most impoitant factoi in
management of the patient is psychologi-
cal suppoit fiom the deimatologist, family,
and suppoit gioups (The National Alopecia
Aieata Foundation, http://-www.naaf.oig/ ).
Peisons with extensive scalp involvement such
as AAT may piefei to weai a wig oi haiipiece.
Makeup applied to eyebiows is helpful. Eye-
biows can be tattooed.
FICk 32-10 A|opeca areata oI sca|p: AA tota|s 4 +,e+|o|d |er+|e W||| |o|+| |o o| c+|p |+||,
e,e||oW, +ud e,e|+|e, + |eW, W|||e ||ue |e|oW|u |+|| +|e eeu.
Ie|oeu e|||u.|ur (IE) | ||e ||+u|eu| |uc|e+ed
|edd|u o| uo|r+| c|u| (|e|oeu) |+|| ||or |e|
|u c+|p |o|||c|e.
'ecoud+|, |o +cce|e|+|ed |||| o| +u+eu (|oW||
p|+e) |u|o c+|+eu +ud |e|oeu (|e||u p|+e)
keu|| |u |uc|e+ed d+||, |+|| |o +ud, || e.e|e,
d|||ue |||uu|u o| c+|p |+||.
',, . Ie|oeu de|||u.|ur
C|ucocortcods TvpIcu| Supeipotent
agents not usually effective.
1ntru|esIvnu| 1nectIvn Few and small
lesions of AA can be tieated with int-
ialesional tiiamcinolone acetonide, 3-7
mg/mL, which can be veiy effective tem-
poiaiily.
SystemIc G|ucvcvrtIcvIds May induce
iegiowth, but AA iecuis on discontinua-
tion; iisks of long-teim theiapy theiefoie
pieclude theii use.
Systemc Cyc|osporoe Induces ie-
giowth, but AA iecuis when diug is dis-
continued.
Ioductoo oI A||erc Cootact 0ermatts -
Dinitiochloiobenzene, squaiic acid dib-
utylestei, oi diphencypione iepoited to
be successful, but local discomfoit due to
alleigic contact deimatitis and swelling of
iegional lymph nodes poses a pioblem.
0ra| FvA (Fhotochemotherapy) -
Vaiiably effective, as high as 30%, and
woith a tiial in patients who aie highly
distiessed about the pioblem. Entiie body
must be exposed, in that the theiapy is
believed to be a foim of systemic immune
suppiession.
II0CN FFIvIM
FICk 32-11 A|opeca areata uoversa|s
(AA) I|| p+||eu| |+ |o| +|| c+|p |+|| (+|opec|+ |o|+
||), e,e||oW, e,e|+|e, |e+|d, +ud +|| |od, |+|| (+|opec|+
uu|.e|+||) +ud |+ d,||op||c ('|+rre|ed ||+) u+||.
to|oy A ieaction pattein to a vaiiety of
physical oi mental stiessois:
Eudoc||ue. n,po o| |,pe|||,|o|d|r , po|p+||ur ,
d|cou||uu+||ou o| c|+u|u |,pe o| e||oeu
cou|+|u|u d|u
|u|||||ou+|. |e||c|euc,. ||o||u, /|uc, ||ou, eeu||+| |+||,
+c|d
k+p|d We||| |o, c+|o||c o| p|o|e|u dep||.+||ou,
c||ou|c ||ou de||c|euc,, e\ce|.e .||+r|u A
|ue||ou
||,|c+| ||e. |e||||e |||uee, c+|+|o||c |||uee (e..,
r+||u+uc,, c||ou|c |u|ec||ou), r+jo| u|e|,, r+jo|
||+ur+, +cu|e o| c||ou|c p,c|o|o|c+| ||e
|,c|o|o|c+| ||e. Au\|e|,, dep|e|ou, ||po|+|
d|o|de|
|u|o\|c+||ou. I|+|||ur, re|cu|,, +|eu|c
||u. Au||r||o||c +eu| (doe depeudeu|). c+uce|
c|ero||e|+p,, |eu/|r|d+/o|e.
Au|||,pe||eu|.e. c+p|op|||
Au||co+u|+u|
C|' d|u. |||||ur, .+|p|o|c +c|d
C|o|e|e|o||oWe||u d|u
Co|c||c|ue
C,|o|+||c d|u
|u|e||e|ou
|eu|c|||+r|ue
ke||uo|d. .||+r|u A e\ce, |e||uo|d (|o||e||uo|u,
+c|||e||u, |ud|u+.||)
'e|ec||.e e|o|ou|u |eup|+|e |u|||||o|
|u||+rr+|o|, c+|p d|e+e. 'e|o|||e|c de|r+||||,
e|,|||ode|r+
|d|op+|||c. |o o|.|ou c+ue | +pp+|eu| |u + |u|||c+u|
uur|e| o| c+e.
Ae oI 0oset Any age.
Sex Moie common in women due to paituii-
tion, cessation of an oial contiaceptive, and
ciash" dieting.
Iocdeoce Second most common cause of alo-
pecia aftei andiogenetic alopecia.
FAIh0CNSIS
Noimal scalp: 80-90% of haiis aie in anagen
phase, 5% in catagen phase, and 10-15% in
telogen phase; 50-100 haiis aie shed as they
aie ieplaced daily.
Telogen effluvium: many moie haiis than
noimal aie shed daily. The piecipitating
stimulus iesults in a piematuie shift of ana-
gen follicles into the telogen phase. Telogen
effluvium occuis in 3-4 months aftei the
inciting event occuiied. If the inciting cause is
iemoved, shedding will iesolve ovei the next
few months as the numbei of haiis in telogen
ietuin to noimal. Haii density may take 6-12
months to ietuin to baseline.
Can become chionic with decieased haii den-
sity, always has potential foi ieveisal, does not
lead to total scalp haii loss, and iaiely goes
beyond 50% loss.
Chionic telogen effluvium: Peisistent shed-
ding aftei an acute episode suggests othei
pathology, e.g., eaily female pattein haii loss
and/oi thyioid malfunction.
Sko Symptoms
Patient piesents with complaint of incieased
haii loss on the scalp that may be accompa-
nied by vaiying degiees of haii thinning.
Most individuals aie anxious, feaiing bald-
ness.
The patient often piesents a plastic bag con-
taining shed haii.
The piecipitating event piecedes the telogen
effluvium by 3 to 4 months.
Sko Iesoos No abnoimalities of the scalp aie
detected.
har (Fig. 32-12) Diffuse shedding of the scalp
haii. Gentle haii pull gatheis seveial to many
club oi telogen haiis.
DIstrIhutIvn Haii loss occuis diffusely
thioughout the scalp and includes the sides
and back of the head. If haii loss is significant
enough to iesult in thinning of haii, alopecia
is noted diffusely thioughout the scalp. Shoit
iegiowing new haiis aie piesent close to the
scalp; these haiis aie finei than oldei haiis and
have tapeied ends.
Na|s The piecipitating stimulus foi TE may
also affect the giowth of nails, iesulting in Beau
lines (see Fig. 33-23), which appeai as tians-
veise lines oi giooves on the fingeinail and
toenail plates.
Iocreased Sheddo oI Sca|p har Nooscar-
ro A|opeca Pattein haii loss, diffuse-pattein
alopecia aieata, loose anagen syndiome, hypei-
thyioidism, hypothyioidism, SLE, secondaiy
syphilis, diug-induced alopecia (Table 32-2).
har Fu|| Compaied with the noimal haii
pull, in which 80-90% of haii is in the anagen
phase, telogen effluvium is chaiacteiized by a
ieduced peicentage of anagen haiis, vaiying
with the intensity of haii shedding. See Biol-
ogy of Haii Giowth Cycles" foi an explanation
of the haii pull.
C8C Rule out iion-deficiency anemia.
Chemstry Seium iion, iion-binding capacity.
ISh Rule out thyioid disease.
Sero|oy ANA, RPR.
SCII0N 32 ||'0k|Ek' 0| nA|k |0|||C|E' A|| kE|AIE| ||'0k|Ek' 9TT
hstopatho|oy No abnoimality othei than an
inciease in the piopoition of follicles in telogen.
0IACN0SIS
Made on histoiy, clinical findings, haii pull, and
possible biopsy, excluding othei causes.
C0kS AN0 Fk0CN0SIS
Complete iegiowth of haii is the iule. In post-
paitum TE, if haii loss is seveie and iecuis aftei
successive piegnancies, iegiowth may nevei be
complete. TE may continue foi up to a yeai aftei
the piecipitating cause.
MANACMNI
No inteivention is needed oi iequiied. The
patient should be ieassuied that the piocess is
pait of a noimal cycle of haii giowth and shed-
ding and that full iegiowth of the haii is to be
expected in most cases.
FICk 32-12 Ie|oeo eII|uvum A c|urp o| |+|| |u ||e |+ud, +oc|+|ed W||| |||||u |||uu|u o| c+|p
|+||. I|e p+||eu| W+ n|\|u|ec|ed +ud e\pe||euced |-:,| :c pueurou|+ 0 Wee| p|e.|ou|,. u|u
||e ||ue| + |oWu, !0 |o +0 |+|| cou|d |e |ero.ed W||| e+c| '|+|| pu||.
IA8I 32-2 0ru|nduced A|opecia
0r0gs Feat0res oI A|opec|a
AC ohbtors
Eu+|+p||| ||o|+||e |e|oeu e|||u.|ur
Aotcoau|aots
nep+||u |eW |epo||
w+||+||u kepo||ed |uc|deuce |+ue ||or 9 |o 10 |u| | p|o|+||, ruc| |oWe|,
d|||ue |edd|u W||| |uc|e+ed uur|e| o| |+|| |u |e|oeu p|+e.
Aotmtotc aeots Au+eu e|||u.|ur
Co|c||c|ue ||||ue |+|| |o, |uc|e+ed uur|e| o| |e|oeu |+||
Aotoeop|astc aeots
B|eor,c|u, c,c|op|op|+r|de, c,|+|+||ue, d+c+||+/|ue, d+c||uor,c|u, d+uuo|u||c|u, do\o|u||c|u, e|opo|de,
||uo|ou|+c||, |,d|o\,u|e+, ||o|+r|de, rec||o|e||+r|ue, re|p|+|+u, re||o||e\+|e, r||or,c|u, r||o\+u||oue,
u|||oou|e+, p|oc+||+/|ue, |||o|ep+, .|u||+||ue, .|uc||||ue
Aotparkosooao aeots
|e.odop+ ||o|+||e |e|oeu e|||u.|ur
Aotseture aeots
I||re||+d|oue ||o|+||e |e|oeu e|||u.|ur
8eta b|ockers ||o|+||e |e|oeu e|||u.|ur
\e|op|o|o|
||op|+uo|o|
8rth cootro| aeots
0|+| cou||+cep||.e ||||ue ||+| |o (|e|oeu e|||u.|ur) 2 |o ! rou|| +||e| ce+||ou o| o|+|
cou||+cep||.e
0rus used o treatmeot
oI bpo|ar dsorders
|||||ur ||o|+||e |e|oeu e|||u.|ur
rot dervatves (used o
treatmeot oI pro|actoema)
B|oroc||p||ue ||o|+||e |e|oeu e|||u.|ur
h
2
b|ockers
C|re||d|ue 0ue| Wee| |o rou||, p|o|+||e |e|oeu e|||u.|ur
heavy meta|s (posooo)
I|+|||ur ||||ue |edd|u o| +|uo|r+| +u+eu |+|| 0 d+, +||e| |ue||ou, corp|e|e
|+|| |o |u rou||, c|+|+c|e||||c | p|ououuced |+|| |o ou |de o|
|e+d, +|o o| |+|e|+| e,e||oW.
\e|cu|, +ud |e+d ||||ue |+|| |o W||| +cu|e +ud c||ou|c e\pou|e.
Cho|estero|-|owero drus
C|o||||+|e 0cc+|ou+||, +oc|+|ed W||| |+|| |o.
Festcdes
Bo||c +c|d Io|+| c+|p +|opec|+ |epo||ed +||e| +cu|e |u|o\|c+||ou, W||| c||ou|c e\pou|e
|+|| |ecore d|, +ud |+|| ou|.
ketoods
E||e||u+|e |uc|e+ed |+|| |edd|u +ud p|uc|ed |e|oeu couu|, dec|e+ed du|+||ou o|
+u+eu p|+e.
|o||e||uo|u ||||ue |o, p|o|+||, +re rec|+u|r + +|o.e.
* ||ep+|ed |, 'u/+uue \||ue|||C|e.e||u|, \.|.
E||o|o,. k+d|+||ou ||e|+p, |o |e+d, c|ero||e|
+p, W||| +||,|+||u +eu|, |u|o\|c+||ou, p|o|e|u
r+|uu|||||ou.
0ue| | uu+||, |+p|d +ud e\|eu|.e (||. !2!).
|+||oeue|. 0ccu| +||e| +u, |uu|| |o |+|| |o|||
c|e ||+| |rp+|| || r||o||c/re|+|o||c +c||.||,. k+p|d
|oW|| +||e| o| d+r+e |o +u+eu |+|| ||+| ||p
c+|+eu +ud |e|oeu p|+e +ud +|e |ed.
\o|e corrou +ud e.e|e W||| cor||u+||ou
c|ero||e|+p, ||+u W||| ||e ue o| + |u|e d|u.
'e.e|||, | eue|+||, doe depeudeu|.
\+u||e|+||ou. ':c| |c |o | d|||ue, e\|eu
|.e, +|o. e,e||oW/|+|e, |e+|d, e|c. |c| |oW
||+u.e|e |+ud|u o| ||d|u.
ke|oW|| | uu+||, |+p|d +||e| d|cou||uu+||ou o|
c|ero||e|+p,.
II0I0C
Anagen cycle disiupted causing vaiying degiees
of haii follicle dystiophy:
RaJaon |eray to head .
||y|ang agens: busulfan, caiboplatin, cai-
mustine, BCNU, chloiambucil, cisplatin,
dacaibazine, estiamustine, fotemustine, ifosa-
mide, lomustine, mechloiethamine, nitiogen
mustaid, melphalan, oxaliplatin, piocaibazine,
stieptozocin, temozolomide, thiotepa.
Inoxtaons: meicuiy, boiic acid, thallium,
colchicine.
Seveie piotein malnutiition.
ANACN FFIvIM
FAIh0CNSIS
Occuis aftei any insult to haii follicle that
impaiis its mitotic/metabolic activity.
Inhibition/aiiest of cell division in haii ma-
tiix leads to thin, weakened haii shaft, suscep-
tible to fiactuie with minimal tiauma as well
as complete failuie of haii foimation.
Anagen haiis bieak off within the follicle oi
at the level of the scalp, being shed without
ioots, oi dystiophic haiis dislodged fiom fol-
liculai mooiings.
FICk 32-13 Aoaeo eII|uvum: chemotherapy A +1,e+|o|d r+|e W||| |,rp|or+ ||e+|ed W||| c|ero
||e|+p,. A|| c+|p, |+c|+|, +ud |od||, |+|| |+.e |+||eu ou|. C|oe |upec||ou |e.e+| ||+| c+|p |+|| |+ |euu |o |e|oW.
Low-dose cytostatic iegimens oi less cytotoxic
diugs such as methotiexate cause telogen
effluvium iathei than anagen effluvium.
Haii bulb itself may be damaged, and haiis
may sepaiate at the bulb and fall out.
Rapid giowth aiiest oi damage to anagen
haiis that skip catagen and telogen phases and
aie shed.
Sko Appeais noimal.
har Scalp haii loss is diffuse, extensive (Figs.
32-13, 32-14). Haii bieaks off at the level of the
scalp. Eyebiows/lashes, beaid, body haii may
also be lost.
Na|s Show tiansveise banding oi iidging.
Successive iounds of chemotheiapy iesult in
paiallel tiansveise band/iidges.
C0kS
Haii iegiows aftei discontinuation of chemo-
theiapy.
Busulfan at high dose may cause peimanent
alopecia due to iiieveisible damage to haii
follicle stem cell.
Regiowth aftei iadiation depends on type,
depth, dose-fiactionation; may iesult in
iiieveisible haii follicle stem cell damage.
MANACMNI
No effective pieventive measuies aie available.
A wig is piefeiied by many peisons.
FICk 32-14 Aoaeo eII|uvum aod acute radatoo dermatts: chemotherapy aod radatoo A +!
,e+|o|d |er+|e W||| re|+|+||c ||e+| c+|c|uor+ |o ||e ||+|u. C|ero||e|+p, (p+c|||+\e|, +d||+r,c|u, c,|o\+u) W+
|.eu W||| |oc+| |+d|+||ou |o ||e ||+|u re|+|+|. |o o| c+|p +ud |+c|+| |+|| W+ eeu. I|e +uu|+|ed e|,||er+
ou ||e uec|, e+|, +ud |+ce der+|c+|e ||e ||e o| |+d|+||ou. I|e p+||eu| d|ed ! rou|| |+|e| o| re|+|+||c d|e+e.
|||r+|, c|c+|||c|+| (c+|||u) +|opec|+ (|CA) |eu||
||or d+r+e o| de||uc||ou o| ||e |+|| |o|||c|e
|er ce|| |,.
|u||+rr+|o|, (uu+||, uou|u|ec||ou) p|ocee
|u|ec||ou. e.., '|e||ou ||ue+ c+p|||, uec|o||/|u
|e|pe /o|e|
0||e| p+||o|o|c p|ocee. u||c+| c+|, p||
r+|, o| re|+|+||c ueop|+r.
\+u||e|+||ou. E||+cereu| o| |o|||cu|+| o||||ce |u
+ p+|c|, o| |oc+| d|||||u||ou, uu+||, |u c+|p o|
|e+|d.
I|e eud |eu|| | e||+cereu| o| |o|||cu|+| o||||ce
+ud |ep|+cereu| o| ||e |o|||cu|+| ||uc|u|e |,
||||ou ||ue (I+||e !2!).
'c+|||u | |||e.e||||e. I|e|+p|e +|e |ue||ec||.e.
CICAIkICIAI 0k SCAkkINC AI0FCIA
Chrooc Cutaoeous (0scod) Iupus rythemato-
sus (CCI) See Section 14.
May occui without othei manifestations oi se-
iologic evidence of lupus eiythematosus (LE).
Manifestations:
CCLE: Eiythematous plaques (Figs. 32-
15, 32-16, 32-17). Keiatotic with folliculai
plugs (caipet tacks"). Scatteied. Vaiiable
in numbei. May become confluent. Atiophy
in oldei patients with scaiiing alopecia.
Postinflammatoiy hypopigmentation, and/
oi folliculai plugging (Fig. 32-17).
SLE: diffuse scalp eiythema with diffuse
haii thinning (Fig. 32-16).
Tumid LE: violaceous deimal inflammatoiy
plaque with oveilying haii loss.
Deimatopathology: See lupus eiytheimato-
sus, Section 14.
IA8I 32-3 C|assification of Primary Cicatricia| A|opecias
Iymphocytc C||ou|c+| cu|+ueou (d|co|d) |upu e|,||er+|ou
||c|eu p|+uop||+|| (|||)
C|+|c |||
||ou|+| ||||o|u +|opec|+
C|+|+r|||||e ,ud|ore
C|+|c peudope|+de o| B|ocq
Ceu||+| ceu||||u+| c|c+|||c|+| +|opec|+
A|opec|+ ruc|uo+
Ke||o| |o|||c|+|| p|uu|o+ dec+|.+u
Neutroph|c |o|||cu|||| dec+|.+u
||ec||u |o|||cu|||| (ce||u||||)
Mxed |o|||cu|||| |e|o|d+||
|o|||cu|||| uec|o||c+
E|o|.e pu|u|+| de|r+|o|
NoospecIc
Icheo F|aoop|ars (IFF) See Lichen Planus,
Section 7.
Folliculai lichen planus (LP) is associated
with cicatiicial scalp alopecia, iesulting in
peimanent haii loss (Fig. 32-18).
LPP may be oi may not be associated with LP
of skin oi mucosa.
Most commonly affects middle-aged women.
Manifestations in scalp: Peiifolliculai eiy-
thema hypeikeiatosis. Violaceous discol-
oiation of scalp. Piolonged inflammation
iesults in scaiiing alopecia. In some cases,
folliculai inflammation and scale aie absent,
with only aieas of scaiiing alopecia, so-called
footpiints in the snow, oi pseudopelade. Dis-
tiibution: most common on paiietal scalp;
also affects othei haii-beaiing sites such as
gioin, axilla.
Symptoms: scalp pain.
Vaiiants:
Cra|am-L|e synJrome : LP-like lesions -
folliculai spines"/keiatosis pilaiis-like le-
sions in aieas of alopecia on scalp, eyebiows,
axillaiy, pubic aieas.
Frona| [|rosng a|oeta : Fiontotempo-
ial haiiline iecession and eyebiow loss in
postmenopausal women with peiifolliculai
eiythema (Fig. 32-19); histology shows LPP.
Per[o||tu|ar ery|ema anJ [o||tu|ar |era-
oss : piogiessive scaiiing alopecia limited
to aiea of pattein haii loss; oveilaps with
fiontal fibiosing alopecia.
Fseudope|ade oI 8rocq
Endstage of all noninflammatoiy scaiiing
alopecias and a vaiiety of initially inflamma-
toiy disoideis .
Pe|aJe is anothei teim foi alopecia aieata.
Pelage is the coat (haii) of an animal. Pseu-
dopelade suggests that the clinical findings
iesemble those of alopecia aieata.
Manifestations:
Eaily lesions: Disciete, smooth, skin- oi
pink-coloied iiiegulaily shaped aieas of
alopecia without folliculai hypeikeiatosis
oi peiifolliculai inflammation (Figs. 32-19
and 32-20).
Pattein of alopecia: Eaily moth-eaten pattein
with eventual coalescence into laigei patches
of haii loss (footpiints-in-the-snow").
Deimatopathology: Similai to lichen plano-
pilaiis.
Scaiiing alopecia is iiieveisible.
FICk 32-15 Scarro a|opeca oI sca|p: chrooc cutaoeous |upus erythematosus (CCI) A +,e+|
o|d W|||e r+|e W||| ru|||p|e |ed d|co|d |e|+|o||c p+|c|e ou ||e c+|p |o| oue ,e+|. A |ed c+||u |e|ou W||| c+|
||u +|opec|+ | eeu ou ||e ||ou|+| c+|p. I|e |u||+rr+|o|, |e|ou |eo|.ed W||| |u||+|e|ou+| |||+rc|uo|oue +ud
|,d|o\,c||o|oqu|ue 200 r B|| |u| ||e|e W+ uo |e|oW|| o| |+||.
FICk 32-16 0IIuse aod
scarro a|opeca oI sca|p: Systemc
I (SI) aod CCI |esoos A !o
,e+|o|d |er+|e W||| poo||, cou||o||ed
'|E |o| ! ,e+|. ||||ue c+|p +|opec|+ |
eeu +oc|+|ed d|c|e|e d|co|d |e|ou
W||| c+|||u +|opec|+. '|e |+ |||o|,
o| p|o|oeu|||.||,.
FICk 32-1T I|| | ||e +re
p+||eu| + ||. !2o. '|e |+
e|,||er+ o| ||e e+| +ud |ed +|e+
o| c+|||u +|opec|+ ou c+|p.
Ceotra| CeotrIua| Scarro A|opeca (CCSA)
Synonyms : folliculai degeneiation syndiome,
hot comb alopecia, pseudopelade.
Most commonly occuis in black women.
Relation to chemical piocessing, heat, oi
chionic tension on the haii is unceitain, but
they aie best avoided.
Slowly piogiessive alopecia begins in the
ciown/midveitex and advances centiifugally
to suiiounding aieas.
Deimatopathology: Eailiest most distinctive
change is piematuie desquamation of the innei
ioot sheath with latei changes thiough the outei
ioot sheath (including migiation of the haii
shaft), a mononucleai infiltiate piimaiily at the
isthmus, and, finally, loss of the folliculai epithe-
lium and ieplacement with fibious tissue.
Scaiiing alopecia is iiieveisible.
A|opeca Mucoosa (Fo||cu|ar Mucooss)
Eiythematous lesions (papules, plaques, oi
flat patches) of alopecia, occuiiing mainly on
scalp and/oi face.
Deimatopathology . piominent folliculai,
epithelial/sebaceous gland mucin, peiifol-
liculai lymphohistiocytic infiltiate without
concentiic lamellai fibiosis.
May be symptom of cutaneous T cell lym-
phoma. (See Section 20).
Fo||cu|ts 0eca|vaos
Pustulai folliculitis leading to haii loss. Sui-
viving haiis clusteied, emeiging fiom a single
folliculai oiifice (tufted folliculitis).
Bogginess oi induiation of scalp/beaid with
pustules, eiosions, ciusts (Fig. 32-21), scale.
Sa|y|otottus aureus infection common.
Whethei S. aureus infection is the piimaiy
piocess oi secondaiy is unceitain.
Deimatopathology: acute suppuiative follicu-
litis, eaily.
Scaiiing alopecia is iiieveisible. Systemic
antibiotics, iifampin, systemic and/oi topi-
cal and/oi intialesional glucocoiticoids, and
systemic ietinoid have been used. S. aureus
infection should be documented and tieated
with appiopiiate antimiciobial agent.
FICk 32-18 Scarro a|opeca oI sca|p: pseudope|ade oI 8rocq caused by |cheo p|aous A oo
,e+|o|d |er+|e. I|e c+|p | roo||, ||u,, de.o|d o| |+|| +ud |+|| |o|||c|e |u r+u, +|e+, ore o| ||e |er+|u|u
|o|||c|e +|e |u||+red W||| pe|||o|||cu|+| e|,||er+ +ud c+|e. 'e.e|+| |+|| +|e eeu ere||u ||or + |u|e ||e
W||||u ||e +|e+ o| +|opec|+ (+||oW). I|e |e|r peudope|+de |rp||e ||+| ||e |e|ou |eer||e +|opec|+ +|e+|+.
FICk 32-19 Scarro a|opeca oI sca|p: |cheo p|aoop|ars (IFF) A o3,e+|o|d W|||e |er+|e W|||
|+|| |o |o| 5 ,e+|. I|e ||ou|+| |+||||ue |+ |+du+||, |eceded, ||e +|e+ o| +|opec|+ |+c| ||e p|reu|+||ou o|
|o|e|e+d ||u, W||c| |+ |+d |||e|ou uu e\pou|e. Bo|| e,e||oW |+.e uo |+||, ||e e,e||oW ou ||e |||| |
peuc||ed |u. I|e e,e|+|e +ppe+| uo|r+|. B|op, W+ |epo||ed |o |oW ||c|eu p|+uu W||| c+|||u +|opec|+. |o
o||e| c||u|c+| ||ud|u o| || We|e de|ec|ed. I|| c||u|c+| .+||+u| o| ||| | c+||ed ||ou|+| ||||o|u +|opec|+.
FICk 32-20 Scarro a|opeca oI sca|p: pseudope|ade oI 8rocq A !3,e+|o|d ||+c| r+|e W|||
p|o|e|.e |+|| |o |o| + ,e+|. E\|eu|.e c+|||u +|opec|+ W||| |e|du+| ||+ud o| |+|| |o|||c|e +ud |+|| ou ||e
.e||e\. |o|e ||e +|euce o| e|,||er+, c+|e, o| c|u|.
0ssecto Fo||cu|ts
Synonyms : dissecting cellulitis, peiifolliculitis
abscedens et suffodiens.
Race: most common in black men.
Initial deep inflammatoiy nodules, piimaiily
ovei the occiput, that piogiess to coalescing
iegions of boggy scalp (Fig. 32-22). Sinus tiacts
may foim; puiulent exudates can be expiessed.
S. aureus secondaiy infection common.
Deimatopathology: eaily folliculai plug-
ging and suppuiative folliculai/peiifolliculai
abscesses with mixed inflammatoiy infiltiate;
latei, foieign-body giant cells, gianulation tis-
sue, scaiiing with sinus tiacts.
Scaiiing alopecia is iiieveisible. S. aureus
infection should be documented and tieated
with appiopiiate antimiciobial agent.
Fo||cu|ts ke|oda|s Nuchae
Synonym : acne keloidalis (nuchae).
Occuis most commonly in black men.
Usually occuis on the occipital scalp and nape
of the neck, staiting with a chionic papulai
oi pustulai eiuption (Fig. 32-23). Results in
a spectium of seveiity fiom small fibiotic pa-
pules to hypeitiophic keloidal scai foimation.
Distiibution: nape of the neck, occipital
scalp.
Eaily mild involvement may iespond to
intialesional tiiamcinolone. If S. aureus is
isolated on cultuie, tieat with appiopiiate
antimiciobial agent.
FseudoIo||cu|ts 8arbae
Synonym: iazoi bumps."
Vaiiant oi same disease as folliculitis keloi-
dalis.
Occuis commonly in black males who shave.
Related to cuived haii follicles. Cut haii
ietiacts beneath skin suiface, giows, and pen-
etiates folliculai wall (tiansfolliculai type) oi
suiiounding skin (extiafolliculai type), caus-
ing a foieign-body ieaction.
Distiibution: any shaved aiea, i.e., beaid
(Fig. 32-24), scalp, pubic.
Keloidal scaiiing in vaiying degiees occuis at
involved sites.
S. aureus secondaiy infection is common.
FICk 32-21 Scarro a|opeca oI sca|p: Io||cu|ts deca|vaos A 50,e+|o|d W|||e r+|e W||| |+||
|o |o| o ,e+|. E|,||er+, |u||+rr+|o|, p+pu|e, c|u|, +ud c+|||u o| ||e c+|p. \+|e p+||e|u |+|| |o | +|o
p|eeu|.
FICk 32-22 Scarro a|opeca
oI sca|p: dssecto Io||cu|ts A
+o,e+|o|d ||+c| |er+|e W||| |ou|+ud
|u +|ce |o|r+||ou o| ||e c+|p |+
|eu||ed |u .e|, e.e|e |,pe|||op||c c+|
||u. I|e|e W+ +oc|+|ed c,||c +cue +ud
||d|+deu||| uppu|+||.+.
FICk 32-23 Scarro a|opeca oI sca|p: Io||cu|ts ke|oda|s A !,e+|o|d ||+c| r+|e W||| p+pu|+|
c+| o| ! ,e+|' du|+||ou, |ecor|u cou||ueu| ou ||e occ|p||+| c+|p +ud uec|. |o|||cu|+| pu|u|e occu| |u ||e
+|e+. I|e coud|||ou | c||ou|c +ud p|o|e|.e, |eu|||u |u |u|||c+u| |+|| |o.
Has been linked to a polymoiphism of the
keiatin gene KC|[.
Acoe Necrotca
Piuiitic oi painful eiythematous folliculai-
based papule with cential neciosis, ciusting,
and healing with depiessed scai.
Lesions occui on anteiioi scalp, foiehead,
nose; at times, the tiunk.
Deimatopathology: lymphocytic neciotizing
folliculitis.
Pooi iesponse to tieatment. Systemic antimi-
ciobial agents and isotietinoin iepoited to be
effective.
rosve Fustu|ar 0ermatoss oI Sca|p
A disease of the eldeily, mainly women, al-
though pediatiic cases do occui.
Manifestations: chionic boggy ciusted
plaque(s) on the scalp oveilying exudative
eiosions and pustules, eventually leading to
scaiiing alopecia.
May follow tiauma oi tieatment of actinic
keiatoses.
Deimatopathology: lymphoplasmacytic infil-
tiate with oi without foieign-body giant cells
and pilosebaceous atiophy.
Pooi iesponse to theiapy. Tieat documented
S. aureus infection.
Sca|p 8opsy 4-mm punch biopsy including
subcutaneous tissue, piepaied foi hoiizontal
section. A second 4-mm punch biopsy speci-
men foi veitical sections and diiect immun-
ofluoiescence.
MANACMNI
C|ucocortcods Topical high-potency and in-
tialesional glucocoiticoids (e.g., tiiamcinolone)
aie the mainstay of tieatment, impioving symp-
toms and haii giowth.
Aotbotcs May be effective, especially if
S. aureus infection is documented.
FICk 32-24 FseudoIo||cu|ts barbae A 29,e+|o|d ||+c| r+|e W||| ru|||p|e |o|||cu|+| p+pu|+| c+| |u
||e |e+|d, ||e p|eeuce o| |o|||cu|+| pu|u|e uu+||, |ud|c+|e ecoud+|, '|c|,|::: c- |o|||cu||||. |o|||cu
|||| |e|o|d+|| | o||eu eeu ou ||e occ|p||+| c+|p +ud uec| (ee ||. !22!).
0eIotoo Excessive haii giowth (women)
in andiogen-dependent haii patteins, second-
aiy to incieased andiogenic activity. Howevei,
vaiies with cultuial and iacial factois. Media
heightens awaieness of bodily haii, eithei spaise
oi excess. The concein of the patient might be
manifestation of body dysmoiphic syndiome.
ksk Factors Familial, ethnic, and iacial influ-
ences. Hiisuteness: white > black > Asian .
Freva|eoce o oted States Suivey of col-
lege-aged women: 25% had easily noticeable
facial haii; 33% had haii along linea alba below
umbilicus; 17% had peiiaieolai haii. Seiies of
E\ce |+|| |oW|| occu| |u |Wo p+||e|u.
||. occu| |u Woreu +| ||e W|e|e |+||
| uude| +ud|oeu cou||o|.
|,-|:|. |+|| deu||, o| |eu|| |e,oud
+ccep|ed ||r|| o| uo|r+| |o| +e, |+ce, e\ (eue|
+||/ed, |oc+||/ed, |+uuo, .e||u, |e|r|u+| |+||).
XCSS hAIk Ck0WIh |C|9 . 10+.

|C|0 . |o3
E\ce|.e |+|| |oW|| (Woreu) |u +ud|oeu
depeudeu| |+|| p+||e|u, ecoud+|, |o |uc|e+ed
+ud|oeu|c +c||.||,.
|o|r+||, ou|, po|pu|eceu| r+|e |+.e |e|r|u+|
|+|| |u ||ee ||e.
',,. uuW+u|ed |+|| .
hIkSIISM
IA8I 32-4 Etio|oy of hirsutism
Aodroeo-secreto tumors uu+||, +oc|+|ed W||| |||eu|+| reue/+reuo|||e+.
Ad|eu+| 0.+||+u
Adeuor+ Cou+d+| ||or+| |uro|
Adeuoc+|c|uor+ I|ecor+
Ec|op|c ACInec|e||u |uro| ||po|d |uro|
Fuoctooa| aodroeo excess
Ad|eu+| eu/,re de||c|euc|e (coueu||+| Cu||u ,ud|ore
+d|eu+| |,pe|p|+|+) |o|,c,||c o.+||+u d|e+e
E+||, oue| 2|,d|o\,|+e de||c|euc, w||| +ud W|||ou| +d|eu+| cou||||u||ou
|+|e oue| 2|,d|o\,|+e de||c|euc, n,pe|||eco|
|,d|o\,|+e de||c|euc,
! de|,d|o\,|+e de||c|euc,
"Idopathc" hrsutsm
Medcatoo[dru-oduced
100 patients: 15% idiopathic, 3% late-onset
congenital adienal hypeiplasia (CAH) (vaiies
within ethnic gioup).
FAIh0CNSIS
Andiogens piomote conveision of vellus to
teiminal haiis in andiogen-sensitive haii fol-
licles: beaid aiea, face, chest, aieolae, linea
alba, lowei back, buttocks, abdomen, exteinal
genitalia, innei thighs.
Dihydiotestosteione, deiived fiom conveision
of testosteione by 5 -R at the haii follicle, is
the hoimonal stimulus foi haii giowth. 50-
70% of ciiculating testosteione in noimal
women is deiived fiom piecuisois, andios-
tenedione, and DHEA; the iest is secieted
diiectly, mostly by the ovaiies. In hypeian-
diogenic women, a gieatei peicentage of an-
diogens may be secieted diiectly.
In women, adienal glands seciete andiosten-
edione, DHEA, DHEA sulfate, and testostei-
one; ovaiies seciete mainly andiostenedione
and testosteione.
hstory
Family histoiy
Diug histoiy
Viiilization symptoms: female pattein haii
loss to male pattein balding, acne, deepened
voice, incieased muscle mass, clitoiomegaly,
incieased libido, peisonality change. Rela-
tively iecent oi iapid onset of symptoms and
signs no associated with pubeity.
Othei: Amenoiihea oi changes in menstiua-
tion. New-onset hypeitension.
Sko Fodos Note: acne, acanthosis nigii-
cans, stiiae.
HIrsutIsm. (1) Note amount of excess haii,
(2) note all sites of haii, (3) evaluate piogiession
and theiapy.
New giowth of teiminal haii (Fig. 32-25),
especially on the face (Fig. 32-25) chest (Fig.
32-25B), abdomen, uppei back, shouldeis.
Ferrman-Ca||wey sta|e iates haii giowth in
each of 11 andiogen-sensitive aieas (uppei
lip, chin, chest, uppei back, lowei back, uppei
abdomen, lowei abdomen, aim, foieaim,
thigh, leg) fiom 0 (no haii giowth) to 4. Scoie
of 8 is consideied hiisutism.
Cusho Syodrome Centiipetal obesity, muscle
wasting (especially peiipheial muscle weak-
ness), violaceous stiiae.
Fe|vc xamoatoo If polycystic ovaiy (PCO)
syndiome is suspected.
IA80kAI0k vAIAII0N 0F hIkSIISM
Serum Iestosterooe If > 200 ng/mL, exclude
andiogen-secieting tumoi.
Serum Free Iestosterooe aod 0ehydroepao-
drosterooe Moie sensitive; most women with
modeiately elevated andiogen levels have poly-
cystic ovaiian syndiome.
1T-hydroxyproesterooe Raised level sug-
gests congenital adienal hypeiplasia; confiim
diagnosis by iepeat measuiement aftei ACTH
stimulation.
Serum Fro|acto Hypeipiolactinemia due to
macio- oi miciopiolactinoma oi tieatment
with neuioleptic diugs; may have associated
menstiual abnoimalities, infeitility, oi galact-
oiihea.
roary 1T-ketosterod Helpful in evaluat-
ing the oveiall amount of andiogen secietion.
Results checked against age-appiopiiate noimal
levels; peak levels occui at 30 yeais (significant
decline with age theieaftei).
0|omeoorrhea[Ameoorrhea Piolactin, fol-
licle-stimulating hoimone (FSH), total testo-
steione.
vr|tatoo Serum esoserone : 200 ng/dL in
women with ovaiian oi adienal tumoi. Urnary
17-|eoseroJs : elevated adienal andiogens.
Serum DHE su|[ae : most specific to adienals
(>90% aiising in adienals); if >800 g/d, sug-
gestive of adienal tumoi.
MANACMNI
Cosmetc Ireatmeot Bleaching : hydiogen pei-
oxide. Tempoiaiy iemoval : Shaving, waxing,
chemical (Naii). Efloinithine (Vaniqua) cieam.
LASER epilation. Electiolysis.
Weht Ioss May be helpful in obese patients;
obesity incieases fiee testosteione levels by
ieducing sex hoimone-biding hoimone and
contiibutes to insulin iesistance.
odocroo|oy Coosu|tatoo Foi suspected
late-onset CAH, Cushing syndiome, tumoi.
Systemc Aotaodroeo Iherapy Oru| AntIun-
drvgens Spiionolactone (100-200 mg daily).
Cypioteione acetate. Finasteiide.
Oru| CvntruceptIves Inhibit andiogen syn-
thesis by inhibiting output of gonadotiopins;
most effective if combined with antiandio-
gens.
BrvmvcrIptIne Foi tieatment of piolactinoma.
4
B
FICk 32-25 hrsutsm: Iace aod chest A !,e+|o|d |er+|e W||| |||u||r. 4. |uc|e+ed |+|| |oW|| |u
+ud|oeudepeudeu| |+|| |o|||c|e o| ||e |de|u|u +|e+, +oc|+|ed W||| +ud|oeu e\ce. B. |uc|e+ed |+|| |oW||
|u +ud|oeudepeudeu| |+|| |o|||c|e o| ||e p|e|e|u+| +ud pe||+|eo|+| |e|ou.
II0I0C ('ee I+||e !25)
Ioca|ted hypertrchoss Tiauma/scai/occu-
pation-ielated sites of iiiitation. Diug-induced:
topical minoxidil. Beckei nevus.
Acqured hypertrchoss Iaouoosa Pio-
duction of lanugo (wasp) haii in follicles
pieviously pioducing vellus haii (malignant
down"). Haii may be >10 cm in length in non-
scalp aieas. Can involve entiie body, except foi
palms and soles. Fine, downy haii coveis laige
aieas of the body. In mild types, downy haii is
n,pe||||c|o| | e\ce|.e |+|| |oW|| (deu||,,
|eu||) |e,oud +ccep|ed ||r|| o| uo|r+| |o|
+e, |+ce, e\ |u +|e+ ||+| +|e uo| +ud|oeu
eu|||.e (||. !22o).
\+, |e eue|+||/ed/uu|.e|+| o| |oc+||/ed .
\+, cou|| o| |+uuo, .e||u, o| |e|r|u+| |+||.
hFkIkICh0SIS
limited to the face; haii on pieviously haiiless
aieas such as the nose and eyelids is usually
noticed fiist. Scalp, beaid, and pubic haii may
not be ieplaced.
oversa| hypertrchoss (||. !22o) Inciease
of lanugo, vellus, oi teiminal haii.
MANACMNI
Find and iemove the inciting cause.
Similai to Cosmetic Tieatment" of hiisutism
(see above).
IA8I 32-5 Etio|oy of hypertrichosis
oversa| hypertrchoss
Coueu||+|/|e|ed||+|, eue|+||/ed |,pe||||c|o|
Acqu||ed eue|+||/ed |,pe||||c|o|
Acqu||ed |,pe||||c|o| |+uu|uo+ (r+||u+uc,). uu+||, |+|||ue| o| r+||u+uc,, o| +|| c+e o| |epo||ed, 93
|+d r+||u+uc, o| ||e C| ||+c|, ||ouc|u, ||e+|, +||||+dde|, u|e|u, ||+dde|, c+u p|ecede ueop|+||c d|+uo|
|, e.e|+| ,e+|.
||u|uduced. r|uo\|d|| (30 o| ||oe ||e+|ed), d|+/o\|de (50), p|eu,|o|u (occu| +||e| 2-! rou|| o|
||e+|reu|), c,c|opo||ue (30), |u\A, o|+| |ucoco|||co|d, ||ep|or,c|u, +ce|+/o|+r|de, o\+||+/o|op,||r|d|ue,
|euo|e|o|, peu|c|||+r|ue
|o|p|,||+. po|p|,||+ cu|+ue+ |+|d+, |ep+|oe|,|||opo|e||c po|p|,||+, .+||e+|e po|p|,||+, e|,|||opo|e||c po|p|,||+
|0E\' ,ud|ore
lu.eu||e de|r+|or,o|||
n,po||,|o|d|r
Ac|od,u|+
\+|+|o|p||ou ,ud|ore
C|'|e|+|ed p|o||er o| ||+ur+. po|eucep|+||||, ru|||p|e c|e|o|, c||/op||eu|+, |e+d |uju|,, |,pe|o|o|
|u|e|u+, +uo|e\|+ ue|.o+
Ioca|ted hypertrchoss
'ecoud+|, |o |||||+||ou, ||+ur+, e|c.
||u|uduced. |u|e||e|ou, |op|c+| r|uo\|d||, |op|c+| |+|+uop|o|
Fredsposo Factors
Shaving haiiy iegions such as the beaid aiea,
axillae, oi legs facilitates folliculai infection.
Extiaction of haii such as plucking oi wax-
ing.
Occlusion of haii-beaiing aieas facilitates
giowth of miciobes: clothing, plastic film,
|u|ec||ou |o|||cu|||| |e|u |u ||e uppe| po|||ou o|
||e |+|| |o|||c|e
E||o|o|c +eu|. B+c|e||+, |uu|, .||u, r||e
\+u||e|+||ou. |o|||cu|+| p+pu|e, pu|u|e, e|o|ou,
o| c|u| +| ||e |o|||cu|+| |u|uud||u|ur
|u|ec||ou c+u e\|eud deepe| |u|o ||e eu|||e |eu||
o| ||e |o|||c|e (,co|).
INFCII0S F0IIICIIIIS |C|9 . 10+.3

|C|0 . |1!.3
adhesive plastei, position (sitting occludes
buttocks, lying in bed occludes back), pios-
thesis, natuial occlusion in inteitiiginous
sites (axillae, infiamammaiy, anogenital).
Topical climate with high tempeiatuie and
ielative humidity.
Topical glucocoiticoid piepaiations.
Systemic antibiotic piomotes giowth of giam-
negative bacteiia; diabetes mellitus; immuno-
suppiession.
FICk 32-26 hypertrchoss
oI Iace E\ce|.e |+|| |oW||
|u uou+ud|oeueu|||.e +|e+ o|
||e |+ce |u + |er+|e ||e+|ed W|||
c,c|opo||ue.
Symptoms S. aureus and deimatophytic fol-
liculitis can be chionic. Usually nontendei oi
slightly tendei; may be piuiitic. Uncommonly,
tendei iegional lymphadenitis.
Sko Iesoos
Papule oi pustule confined to
the ostium of the haii follicle,
at times suiiounded by an eiy-
thematous halo (Figs. 32-27, 32-
28). Ruptuie of pustule leads
to supeificial eiosions oi ciusts.
Scatteied disciete oi moie fie-
quently giouped and clusteied.
Usually, only a small peicent-
age of follicles in a iegion is
infected.
IA8I 32-6 C|assification of |nfectious |o||icu|itis by Etio|oy
|oIect|o0s Ageot 0rgao|sm
B+c|e||+| ' c-. upe|||c|+| (Boc||+|| |rpe||o), deep (,co|), r+, p|o|e
|o |u|uuc|e (|o||) o| c+||uuc|e |o|r+||ou
|-!c c-c (|o||u|) |o|||cu||||, |+rue+||.e |o|||cu||||
|uu+| |e|r+|op|,||c |o|||cu||||. ||ue+ c+p|||, ||ue+ |+||+e, \+jocc|| |+uu|or+,
!c|c-cc |o|||cu||||,
Cc!!c |o|||cu||||
\||+| ne|pe |rp|e\ .||u,
\+||ce||+/o|e| .||u,
\o||ucur cou|+|our,
',p||||||c 'ecoud+|, ,p||||. +|opec|+, +cue||o|r
|u|e|+||ou |erod|c|do|
Supeificial infection heals without scaiiing,
but in daikly pigmented individuals, postin-
flammatoiy hypo- and hypeipigmentation.
Extension of infection can piogiess to abscess
oi fuiuncle foimation (Fig. 32-29).
FICk 32-2T IoIectous Io||cu|-
ts, superIca| o ax||a: MkSA
A 25,e+|o|d |er+|e W||| p|u||||c
+ud |eude| +\|||+|, |e|ou |o| e.e|+|
Wee|. \u|||p|e |o|||cu|+| pu|u|e
+ud p+pu|e +|e eeu |u ||e .+u|| o|
||e |+.ed +\|||+. '|+.|u |+c||||+|e
eu||, o| ' c- |u|o ||e upe|||c|+|
|+|| |o|||c|e. I|e |e|ou |eo|.ed W|||
r|uoc,c||ue.
FICk 32-28 IoIectous Io||cu|ts oo Iorearm A ++,e+|o|d r+|e W||| n|\/A||' |+ |+d + p|u||||c +ud
|eude| ||e ou ||e |o|e+|r |o| e.e|+| rou||. 0u e\+r|u+||ou, uure|ou pu|u|e +ud p+pu|e +|e eeu W|||
r||d ||c|eu |rp|e\ c||ou|cu. I|e |e|ou |eo|.ed W||| r|uoc,c||ue.
FICk 32-29 IoIectous Io||cu|ts oo buttocks, deep A 29,e+|o|d r+|e, W||| n|\/A||' +ud eud|+e
|eu+| d|e+e (E'k|), |e|u ||e+|ed W||| pe|||oue+| d|+|,| |+ |+d pe|||eu| p+|u|u| |e|ou ou ||e |u||oc| |o|
e.e|+| rou||. |u|u|e +ud e+||, |u|uuc|e +|e eeu ou ||e |u||oc| +ud pe||ue+| +|e+.
In chionic folliculitis, a full iange of lesions is
noted.
Pseudofolliculitis baibae caused by pen-
etiation of the skin by shaip tips of shaved
haiis fiequently complicated by S. aureus
secondaiy infection (see Fig. 32-24).
0strbutoo Fuce S. aureus . Giam-negative
folliculitis: iesembles oi may coexist with acne
vulgaiis. Molluscum contagiosum. Demodici-
dosis iesembles iosacea.
Beurd Areu S. aureus folliculitis: follicu-
litis (sycosis) baibae, most commonly of
shaved beaid aiea Deimatophytic folliculitis:
tinea baibae; papulopustules may coalesce to
deeply infiltiated keiion. Heipes
simplex viius. Molluscum conta-
giosum. Demodicidosis iesembles
iosacea.
Scu|p S. aureus . Deimatophytic.
Nec| S. aureus in shaved aiea and
nape of neck, occipital scalp, espe-
cially in diabetics. Pseudofolliculitis
in shaved aiea. Keloidal folliculitis
in nape of neck; folliculai keloids
to laige nodulai-tumoious keloidal
masses. (see page 986)
Legs Occuis in women who shave
legs. In India, a chionic folliculitis
occuis in young men, lasting foi
yeais. Pustulai deimatitis atiophi-
cans of the legs iepoited commonly
fiom West Afiica, usually affecting
the shins, sometimes the thighs and
foieaims.
Trun| S. aureus in axillae, especially
in those who shave. PseuJomonas
aerugnosa (hot tub") folliculitis
(Fig. 32-30). Ma|asse:a folliculitis.
CanJJa folliculitis on the back of
hospitalized patients with fevei who
lie in supine position.
Buttvc|s Common site foi S. au-
reus folliculitis. Deimatophytic.
varaots
S. aureus Fo||cu|ts Can be eithei
supeificial folliculitis (infundibu-
lai) (Fig. 32-27) oi deep (sycosis)
(extension beneath infundibulum)
with abscess foimation (Figs. 32-28,
32-29). In the shaved beaid aiea,
also known as sycosis vulgaiis, oi
baibei`s itch. In seveie cases (lupoid
sycosis), the pilosebaceous units may
be destioyed and ieplaced by fibious
scai tissue.
Cram-Neatve Fo||cu|ts Occuis in individu-
als with acne vulgaiis tieated with oial antibi-
otics. Acne" typically woisens, having been in
good contiol. Chaiacteiized by small folliculai
pustules and/oi laigei abscesses on the cheeks.
hot Iub Fo||cu|ts Occuis on the tiunk fol-
lowing immeision in spa watei (Fig. 32-30).
0ermatophytc Fo||cu|ts Infection begins
in the peiifolliculai stiatum coineum and
spieads into folliculai ostia and haii shafts
(see Section 25). (Fig. 32-31)
Ioea Capts (see Section 25)
FICk 32-30 IoIectous Io||cu|ts ("hot tub"): P. oeruy-
oeso A !,e+|o|d r+|e W||| p+|u|u| |e|ou ou ||e ||uu|. \u|||
p|e |o|||cu|+| pu|u|e +|e eeu, |+.|u +ppe+|ed ! d+, +||e| |+|||u
|u + |o| |u|. | c-c W+ |o|+|ed ou cu||u|e ||or + |e|ou.
I|e |e|ou |eo|.ed pou|+ueou|, W||||u + Wee|. || ,rp|or+||c,
c|p|o||o\+c|u 500 r B|| c+u |e |.eu.
In deimatophytic Majocchi gianuloma, scat-
teied papules, pustules and nodules, usually
associated with tinea ciuiis oi tinea coipoiis.
Mu|ussezIu Fo||cu|ts Moie common in sub-
tiopical and tiopical climates. Piuiitic, mono-
moiphic eiuption chaiacteiized by folliculai
papules and pustules on the tiunk, most often
on the back (Fig. 32-32), uppei aims, and less
often on the neck and face; excoiiated papules.
Absence of comedones diffeientiates it fiom
acne vulgaiis (see Section 25). Synonym : Pity-
iospoium folliculitis.
CundIdu u|hIcuns Occuis in sites of occluded
skin such as the back of a hospitalized febiile
patient oi undei plastic diessing, especially if
topical glucocoiticoid piepaiations aie used.
Laige folliculai pustules (see Section 25).
herpetc Fo||cu|ts Occuis piedominantly in
the beaid aiea (viial sycosis) in men. Chai-
acteiized by folliculai vesicles and latei ciusts
(Fig. 32-33).
Mo||uscum Fo||cu|ts Piesents as umbilicated
skin-coloied papules in a folliculai and peiifol-
liculai distiibution ovei the beaid aiea.
Syph|tc (Iuetc) Fo||cu|ts: Secoodary
Nonscaiiing alopecia of the scalp and beaid
(alopecia aieolaiis); moth-eaten" appeaiance.
Synonym : Alopecia syphilitica.
0emodcdoss Clinical piesentation: peiifol-
liculai scaling (pityiiasis folliculoium oi iosa-
cea-like eiythematous folliculai papules and
pustules with a backgiound of eiythema on the
face. Etiology: DemoJex [o||tu|orum .
Fo||cu|ar IoI|ammatory 0sorders Acneifoim
disoideis (acne vulgaiis, iosacea, peiioial dei-
matitis), HIV-associated eosinophilic follicu-
litis, chemical iiiitants (chloiacne), acneifoim
adveise cutaneous diug ieactions epideimal
giowth factoi ieceptoi inhibitois (e.g., eilotinib),
halogens, glucocoiticoids, lithium], keloidal fol-
liculitis, pseudofolliculitis baibae.
keooa| 0IIereota| 0aooss Fate : acne,
iosacea, peiioial deimatitis, keiatosis pilaiis,
pseudofolliculitis baibae (ingiowing haiis).
Sta| : folliculitis neciotica. Trun| : acne vul-
gaiis, pustulai miliaiia, tiansient acantholytic
disease (Giovei disease). x||ae anJ grons :
hidiadenitis suppuiativa.
FICk 32-31 0ermatophytc Io|-
|cu|ts: Irchephyteo rubrum A
!2,e+|o|d r+|e W||| n|\/A||' |+d +
p|u||||c |+| ou ||e |u||oc| |o| oue ,e+|,
|op|c+| |ucoco|||co|d +ud +u|||uu+|
+eu| |+d uo| |eeu e||ec||.e. \u|||p|e
|o|||cu|+| p+pu|e +ud c+||u e|,||er+
+|e eeu ou ||e +c|+| +|e+, ||ue+ c|u||
+ud ped| We|e +|o p|eeu|. K0n p|ep+
|+||ou |oWed ep|+|ed |,p|+e. I|e
|e|ou |eo|.ed W||| o|+| |e|||u+||ue.
IA80kAI0k FIN0INCS
0rect Mcroscopy Grum StuIn S. aureus.
giam-positive cocci. Also visualizes fungi.
KOH PrepurutIvn Deimatophytes: hyphae,
spoies. M. [ur[ur : multiple yeast foims; Can-
JJa : mycelial foims.
Cu|ture BucterIu| S. aureus, P. aerugnosa ;
giam-negative folliculitis: Proeus, K|e|se||a,
Est|ert|a to| . In cases of chionic ielapsing
folliculitis, cultuie naies and peiianal iegion foi
S. aureus caiiiage.
Fungu| Deimatophytes; C. a||tans .
VIru| Heipes simplex viius (HSV).
0ermatopatho|oy The following featuies
should be evaluated: Aie miciooiganisms
piesent: Is the inflammatoiy infiltiate piedomi-
nantly folliculai oi peiifolliculai: What iegion
of the pilosebaceous stiuctuie is involved: Is
the inflammatoiy piocess acute suppuiative
(neutiophilic), chionic lymphocytic, oi gianu-
lomatous (foieign-body iesponse to keiatin
subsequent to iuptuie of follicle): Is any poi-
tion of the pilosebaceous stiuctuie destioyed:
0IACN0SIS
Clinical findings confiimed by laboiatoiy find-
ings.
C0kS AN0 Fk0CN0SIS
S. aureus folliculitis can piogiess to deepei
folliculai and peiifolliculai infection with
abscess (fuiuncle, caibuncle) oi cellulitis.
Infection of multiple contiguous follicles
iesults in a caibuncle.
Many types of infectious folliculitis tend to
iecui oi become chionic unless the piedis-
posing conditions aie coiiected.
MANACMNI
Frophy|axs Corret unJer|yng reJsosng
tonJon . Washing with antibacteiial soap oi
benzoyl peioxide piepaiation oi isopiopyl/eth-
anol gel.
Aotmcroba| Iherapy BucterIu| Fv||Icu|ItIs
See Table 24-2.
Grum-negutIve Fv||Icu|ItIs Associated with
systemic antibiotic theiapy of acne vulgaiis.
Discontinue cuiient antibiotics. Wash with
benzoyl peioxide. In some cases, ampicillin
(250 mg foui times daily) oi tiimethopiim-sul-
famethoxazole foui times daily. Isotietinoin.
Fungu| Fv||Icu|ItIs Vaiious topical antifungal
agents. Foi deimatophytic folliculitis: teibin-
afine, 250 mg PO foi 14 days, oi itiaconazole,
100 mg twice daily foi 14 days. Foi CanJJa
folliculitis: fluconazole oi itiaconazole, 100 mg
twice daily foi 14 days.
HerpetIc Fv||Icu|ItIs See Heipes Simplex Vi-
ius Infections" (Section 27).
DemvdIcIdvsIs Peimethiin cieam. Iveimec-
tin, 200 g/kg (usual iange, 12-18 mg) stat.
Pseudv]v||Icu|ItIs Burhue Rule out second-
aiy S. aureus infection. Discontinue shav-
ing. Use beaid clippei instead of safety iazoi.
Destiuction of haii follicle: electiolysis; lasei
haii iemoval.
FICk 32-32 IoIectous Io||cu|ts: Molossezo lurlur A +,e+|o|d n|p+u|c r+|e W||| ||uu|+| |+|
|o| 2 rou||. \u|||p|e, d|c|e|e, |o|||cu|+| p+pu|opu|u|e ou ||e c|e|. |e|ou+| ||op, |oWed ,e+| |o|r o|
!c|c-cc ||. I|e |e|ou |eo|.ed +||e| ||e+|reu| W||| o|+| |||+cou+/o|e.
FICk 32-33 IoIectous Io||cu-
|ts: herpes smp|ex vrus A +0
,e+|o|d |e+|||, r+|e W||| d|c|e|e +ud
|ouped pu|u|e +ud e|o|ou |u ||e
|e+|d +|e+ |o| ! Wee|. n'\ W+ |o|+|ed
ou cu||u|e. |o p+||oeu We|e |o|+|ed
ou |+c|e||+| cu||u|e. |e|ou |eo|.ed
W||| o|+| +c,c|o.||.
1000
S E C I | 0 N 5 5
0IS0k0kS 0F Ih NAII
AFFAkAIS
I|e u+|| +pp+|+|u | r+de up o|.
|+|| p|+|e, ||e |o|u, 'de+d p|oduc|
|ou| pec|+||/ed ep|||e||+. p|o\|r+| u+|| |o|d,
u+|| r+|||\, u+|| |ed, |,pou,c||ur
||ue|u+|| +dd |uuc||ou |o ru|||p|e ue o| ||e
|+ud, p|o|ec| ||e |e|r|u+| d|||, +dd |o e||e||c
o| ||e ||ue|.
Ioeu+|| p|o|ec| ||e d||+| |oe +ud cou||||u|e |o
ped+| ||orec|+u|c.
|+|| +pp+|+|u d|o|de| c+u |e ||+ur+||c, ||uc
|u|+|, p||r+|,, r+u||e|+||ou o| cu|+ueou d|
e+e (e.., po||+|), ueop|+||c, |u|ec||ou, o|
r+u||e|+||ou o| ,|er|c d|e+e (e.., |upu
e|,||er+|ou).
N0kMAI NAII AFFAkAIS
C0MF0NNIS 0F Ih N0kMAI NAII
AFFAkAIS (See Image 33-1)
Na| F|ate The haid piotective tool, the piod-
uct of the nail appaiatus. Rests on and is fiimly
attached to nail bed, which is attached to undei-
lying bone. Suiiounded on thiee sides by nail
folds. Made of thiee hoiizontal layeis: thin doi-
sal lamina, thickei inteimediate lamina, vential
layei fiom nail bed. Haidness of nail plate due
to high sulfui matiix piotein. Nail plate shape
ielates to shape of undeilying phalangeal bone.
Longitudinal iidging incieases with aging.
Froxma| Na| Fo|d (FNF) Coveis pioximal
one-quaitei of the nail plate. Has two epithelial
suifaces, doisal and vential.
Dvrsu| PNF Similai to skin; thinnei; devoid of
pilosebaceous units. Devoid of deimatoglyphic
maikings.
Ventru| PNF Coveis of nail plate. Closely
adheient to nail plate suiface; keiatinizes with
a gianulai layei.
DermIs v] PNF Contains numeious capillai-
ies that iun paiallel to skin suiface. Can be visu-
alized with deimoscope: peimits obseivation of
aiteiial and venous limbs of capillaiies, which
aie aiianged in paiallel iows and appeai as fine
iegulai loops with a small space between affei-
ent and effeient vessels. Moiphology alteied in
collagen vasculai disease.
Cutc|e Junction of two epithelial suifaces of
PNF, piojects distally onto nail suiface, sealing
PNF and nail. Piotects stiuctuies at base of nail
(geiminative matiix) fiom iiiitants, alleigens,
bacteiial/fungal pathogens. Loss of cuticle pio-
duces potential space oi pocket: inflammation
of this pocket iesults in chionic paionychia.
Iatera| Fo|ds Usually covei lateial edges of
plate.
Iuou|a Undeilies pioximal fold. Noimally is
white. Repiesents most distal iegion of the
matiix.
Free Maro Distal nail. Natuial shape same as
contoui of distal lunula.
Na| Matrx Pioximal matiix undeilies nail
plate to distal boidei of lunula. Distal matiix
is that poition distal to distal boidei of lunula.
Pioduces the majoi pait of nail plate. As in epi-
deimis, possesses a dividing basal cell layei pio-
ducing keiatinocytes, which diffeientiate, haiden,
die, and contiibute to nail plate-analogous to
epideimal stiatum coineum. Keiatinocytes ma-
tuie and keiat inize without keiatohyalin (gian-
ulai layei) foimation. Melanocytes aie piesent
in lowei layeis and pioduce melanin. Lineai
longitudinal pigmented bands may be seen in
peisons of daikei skin phototypes.
Na| 8ed Consists of epideimal pait (vential
matiix) (no moie than two to thiee cells thick)
and undeilying deimis closely apposed to pe-
iiosteum of distal phalanx. Within connective
tissue netwoik lie blood vessels, lymphatics, a
fine netwoik of elastic fibeis, and scatteied fat
cells. Subcutaneous fat layei is absent. Noimally
pink due to vasculatuie as seen thiough the
tianslucent nail plate.
SCII0N 33 ||'0k|Ek' 0| InE |A|| A||AkAIu' 1001
0oychocoroea| 8aod Thin distal tiansveise
white band, which maiks the most distal poi-
tion of attachment of the nail plate to nail bed.
Disiuption causes onycholysis.
0oychoderma| 8aod Aiea fiom onychocoineal
band to nail plate fiee edge.
hypooychum Space undei fiee maigin of
nail plate, fiom point of sepaiation of nail
plate fiom nail bed to the distal end of the nail
plate. Extension of hyponychium pioximally is
onycholysis.
CI0SSAk 0F A8N0kMAIIIIS
0F Ih NAII AFFAkAIS
8eau Ioes Tiansveise depiession in nail
plate. Single nail involvement is usually tiau-
matic. Multiple nail involvement indicates
systemic cause. Etiology: Tiauma (mani-
cuie, onychotillomania); deimatologic dis-
ease (eczema, eiythiodeima, paionychia);
systemic disoideis (diugs, pyiexia, viial infection
(hand-foot-and-mouth disease, measles); Ka-
wasaki syndiome; peiipheial ischemia.
0oychomadess Peiiodic sepaiation of the
pioximal poitions of the nail plate fiom the
matiix and bed with subsequent shedding of
the nails. Single nail: usually tiaumatic. Mul-
tiple nails: systemic cause. Eo|ogy : same as
Beau lines.
ko|ooycha Spoon nails; thinned concave
nails. Physiologic in childien; in adults, most
commonly occupational oi iion deficiency.
Irue Ieukooycha White opaque discoloiation
of the nail plate associated with distal nail ma-
tiix damage; may be punctate, stiiate, oi diffuse.
Sng|e na| : tiaumatic, psoiiasis. Puntae |eu-
|onyt|a : small white opaque spots; commonly
seen following tiauma oi as the only finding
in psoiiasis. Transerse |eu|onyt|a : multiple
tiansveise opaque paiallel bands; commonly
associated with matiix tiauma of manicuie
oi tight shoes. D[[use |eu|onyt|a : nail plate
completely opaque and white. Fungal infec-
tions cause supeificial white onychomycosis
and pioximal subungual onychomycosis.
Appareot Ieukooycha White discoloiation
that fades with piessuie; abnoimalities of coloi
of nailbed; nail tianspaiency maintained. Oc-
cuis with chemotheiapeutic diugs and systemic
disease.
Me|aoooycha Caused by activation oi pio-
lifeiation of nail matiix melanocytes. Melanin
hypeipigmentation of the nail, eithei paitial
(longitudinal) oi complete. Eo|ogy : iace, HIV/
AIDS, inflammatoiy nail disoideis, diugs
(AZT), Addison disease, piegnancy, Laugiei-
Huntzikei syndiome, tiauma. Single band may
iepiesent nail matiix nevus oi melanoma.
Iootudoa| rythrooycha Nail bed disoidei.
Red band extending fiom pioximal nail fold to
IMAC 33-1 Schematc drawo oI oorma| oa|.
distal edge. Eo|ogy : solitaiy caused by onycho-
papilloma oi othei benign subungual tumoi;
multiple seen in Daiiei disease.
0oychauxs Nail plate appeais to be thickened
due to subungual hypeikeiatosis of nailbed.
Common in psoiiasis, eczema, distal subungual
onychomycosis.
0oycha Inflammation of the matiix of the
nail iesulting in shedding of nail.
0oychoc|ass Bieaking of the nail.
0oychocryptoss Ingiowing nail.
0oychoryphoss Hypeitiophy of the nail(s),
pioducing a hooked oi incuived clawlike de-
foimity.
0oycho|yss Sepaiation of the nail plate fiom
the nail bed, usually beginning at the fiee mai-
gin and piogiessing pioximally. Common in
psoiiasis, tiauma, distal subungual onychomy-
cosis.
0oychoma|aca Softening of the nail(s).
0oychomycoss Tinea unguium. Fungal in-
fection of peiiungual and nail bed skin with
giadual involvement of the suiface of the nail
plate.
0oychorrhexs Longitudinal iidging and fis-
suiing of the nail plate with biittleness and
bieakage. Common with aging. Occuis in li-
chen planus.
Iootudoa| Crooves Usually single. Caused
by tumois (digital myxoid cyst) of the pioximal
nail fold.
0oychoschta Splitting oi lamination of the
nail plate, usually in the hoiizontal plane at the
fiee edge.
0oychot||omaoa Compulsive picking oi teai-
ing at the nails.
Ftto oI Na| F|ate Results fiom small aieas
of abnoimal keiatinization of pioximal nail
matiix. Punctate depiessions of the nail plate
suiface. Common in psoiiasis; also atopic dei-
matitis. Geometiic and supeificial pits seen in
alopecia aieata (hammeied biass nails).
Iootudoa| kdo Incieases noimally with
aging.
Irachooycha Nails iough due to excessive longi-
tudinal iidging, often thinned. Twenty-nail dystio-
phy oi sandpapei nails associated with pioximal
nail matiix damage: alopecia aieata, lichen planus,
psoiiasis, deimatitis. May iegiess spontaneously.
Ihooo oI Na| F|ate Sign of matiix disoidei.
Ihckeoo oI Na| F|ate Consequence of nail
bed disoideis.
|oc+| d|o|de| +||ec||u ||e u+|| +pp+|+|u c+u |eu|| |u + pec||ur o| c||ou|c u+|| d|e+e.
I0CAI 0IS0k0kS 0F NAII AFFAkAIS
Aoc|+|ed W||| d+r+e |o cu||c|e. rec|+u|c+| o|
c|er|c+|.
A| |||. +du|| Woreu, |ood |+ud|e|, |ouec|e+ue|.
C||ou|c de|r+|||| o| p|o\|r+| u+|| |o|d +ud
r+|||\. c||ou|c |u||+rr+||ou (ec/er+, po||+|)
W||| |o o| cu||c|e, ep+|+||ou o| u+|| p|+|e ||or
p|o\|r+| u+|| |o|d (||. !!).
||ed|po|u |+c|o|.
|e|r+|o|. po||+|, de|r+|||| +|op|c, |||||+u|
(occup+||ou+|), +||e||c cou|+c||, ||c|eu p|+uu
||u. o|+| |e||uo|d (|o||e||uo|u, +c|||e||u),
|ud|u+.||
|o|e|u |od,. |+||, |||||e, Wood p||u|e|.
\+u||e|+||ou. ||||, ecoud, +ud ||||d ||ue| o|
dor|u+u| |+ud, p|o\|r+| +ud |+|e|+| u+|| |o|d
e|,||er+|ou +ud Wo||eu, cu||c|e +|eu|.
|u|e|r|||eu||,, pe|||eu| |oW|+de |u||+rr+||ou
r+, ||+|e |u|o u|+cu|e p+|u|u| e\+ce||+||ou,
|eu|||u |u d|co|o|ed ||+u.e|e ||d|u o| |+|e|+|
ede.
'ecoud+|, |u|ec||ou/co|ou|/+||ou. Cc!!c pp.,
|-!c c-c , o| '|c|,|:::
c- . |+|| p|+|e r+, |ecore d|co|o|ed, |eeu
uude|u||+ce W||| |-!c . |u|ec||ou +
oc|+|ed W||| p+|u|u| +cu|e |u||+rr+||ou.
!cc--|.
||o|ec||ou
I|e+| ||e de|r+|||| W||| |ucoco|||co|d. |op|
c+|, |u||+|e|ou+| |||+rc|uo|oue, |o|| cou|e o|
p|edu|oue
I|e+| ecoud+|, |u|ec||ou.
Chk0NIC FAk0NChIA |C|9. o3.02

|C|0. |0!.0
|e|+c|reu| o| u+|| ||or || |ed +| d||+| +ud/o|
|+|e|+| +||+c|reu| (||. !!2).
0u,c|o|,| c|e+|e + u|uuu+| p+ce ||+| co|
|ec| d||| +ud |e|+||uou de|||, |+,||W|||e
co|o| due |o p|eeuce o| +|| uude| u+||, |u|
co|o| .+||e ||or ,e||oW |o ||oWu, +|e+ r+,
|e r+|odo|ou W|eu ||e o.e||,|u u+|| p|+|e |
|ero.ed.
E||o|o,.
|||r+|,. |d|op+|||c (||ue|u+|| |u Woreu, re
c|+u|c+| o| c|er|c+| d+r+e |o ||e ou,c|ode
|r+| |+ud), ||+ur+ (||ue|u+||, occup+||ou+|
|uju|,, |oeu+||, pod|+|||c +|uo|r+||||e, poo||,
|||||u |oe).
'ecoud+|,. \e|cu|o|u||ou d|o|de| (cou|+c|
de|r+||||, d,||d|o||c ec/er+, |e|pe |rp|e\),
u+|| |ed |,pe||e|+|o| (ou,c|or,co|, po||+
|, c||ou|c cou|+c| de|r+||||), u+|| |ed |uro|,
d|u.
|u po||+|, ,e||oW||||oWu r+||u | .||||e |e
|Weeu p|u| uo|r+| u+|| +ud W|||e ep+|+|ed +|e+.
|u 'o|| po| o| '+|roup+|c| .+||e|, (||. !!2),
u+|| p|+|e-u+|| |ed ep+|+||ou r+, |+|| |u r|dd|e
o| u+||.
Co|ou|/+||ou W||| | c-c |eu|| |u + ||o
|||r ou ||e uude|u||+ce o| ||e ou,c|o|,||c u+||
p|+|e, c+u|u + ||oWu o| |eeu|| d|co|o|+||ou
(||. !!!).
0||e| ecoud+|, p+||oeu ||+| c+u co|ou|/e/|u
|ec| ||e p+ce +|e Cc!!c pp., de|r+|op|,|e,
+ud uure|ou eu.||oureu|+| |uu|.
uude||,|u d|o|de| |u ||ue|u+|| ou,c|o|,|.
||+ur+ (e.., p||u|e|), po||+|, p|o|oou,c|o|,|
(e.., do\,c,c||ue), de|r+|o| +dj+ceu| |o u+||
|ed (e.., po||+|, de|r+||||, c|er|c+| e\po
u|e), coueu||+|/|e|ed||+|,.
uude||,|u |oeu+|| ou,c|o|,|. +dd|||ou+| |+c|o|
o| ou,c|or,co| (:||,| |) , |oe
||+ur+.
!cc--| . de|||de +|| u+|| ep+|+|ed ||or
u+|| |ed (p+||eu| |ou|d cou||uue Wee||, de|||de
reu|), |ero.e de||| ou u+|| |ed, ||e+| uude||,
|u d|o|de|.
0NCh0ISIS |C|9. 10!.3

|C|0. |o0.
FICk 33-1 Chrooc parooycha A o+,e+|o|d |er+|e W|o Wo|| + + c|e+ue| |+ |+d |+ud 'de|r+||
|| |o| r+u, ,e+|. I|e d||+| ||ue| +ud pe||uuu+| ||u +|e |ed +ud c+||u. I|e cu||c|e | +|eu|, + poc|e| |
p|eeu|, |o|red + ||e p|o\|r+| u+|| |o|d ep+|+|e ||or ||e u+|| p|+|e. I|e u+|| p|+|e |oW ||+c|ou,c||+ (|ou|
u||+ce W||| |ou||ud|u+| ||d|u) +ud ou,c|+u\| (+pp+|eu| u+|| p|+|e |||c|eu|u due |o u|uuu+| |,pe||e|+|o|
o| u+|||ed). I|e uude||,|u p|o||er | po||+|. Cc!!c c||:c o| ' c- c+u c+ue p+ce |u|ec||ou |u ||e
'poc|e| W||| |u|e|r|||eu| e|,||er+ +ud |eude|ue o| ||e u+|| |o|d.
uu+||, +oc|+|ed W||| ou,c|o|,| (ee +|o.e).
| c-c , ||e ro| corrou c+ue, p|oduce
||e |eeu p|reu| p,oc,+u|u (||. !!!).
!cc--| . de|||de '|,||c u+||. 'ee +|o.e.
CkN NAII SN0k0M
FICk 33-2 0oycho|yss A o0,e+|o|d |er+|e W||| ||ue|u+|| p|o||er |o| 0 ,e+|. |||+| ou,c|o|,| o|
||ue|u+|| W||| |||||e u|uuu+| de|||dereu|. \||d c||ou|c p+|ou,c||+ | p|eeu| W||| |o o| cu||c|e. |o||+| |
||e |||e|, uude||,|u p|o||er.
FICk 33-3 0oycho|yss wth Pseudemeoos co|ootatoo |o||+| |+ |eu||ed |u d||+| ou,c|o|,| o| ||e
||ur|u+||. A ||o|||r o| |-!c c-c |+ p|oduced ||e |eeu||+c| d|co|o|+||ou o| ||e uude|u||+ce o| ||e
ou,c|o|,||c u+||. 0u,c|o|,| |eo|.ed |o||oW|u ||e de|||dereu| +ud ||e+|reu| o| ||e u+|||ed W||| |ucoco|||co|d c|e+r.
0,:|c . I||c|eu|u o| eu|||e u+|| p|+|e, eeu
|u e|de||, (||. !!+).
0,:|,| . 0u,c|+u\| W||| |+r' |o|u|||e
de|o|r||,, ro| corrou|, o| |e+| |oe (||. !!+).
|+|| | e.e|e|, d||o||ed, |||c|eued, ||oWu||,
p||+|ed, W|||ou| +||+c|reu| |o u+|| |ed.
|||, . p|eu|e ||or |oo|W+|e |u e|de||,, +|o,
|u|e|||ed +u|oor+| dor|u+u|.
Ke|+||u p|oduced |, r+|||\ +| uue.eu |+|e,
W||| |+|e||oW|u ||e de|e|r|u|u d||ec||ou o|
de|o|r||,.
0NChAXIS AN0 0NCh0CkFh0SIS
kepe+|ed r+u|pu|+||ou o| ||e u+|| +pp+|+|u c+u
|eu|| |u c|+ue o| ||e p+|ou,c||+| ||u +ud ||e
u+|| p|+|e.
habt-tc 0eIormty ',, ceu||+| |ou||u
d|u+| |oo.ed d,||op|,, ou,c|od,||op||+ red|
+u+ c+u+|||o|r|. w+||o+|d u+|| p|+|e (||. !!5).
C+ued |, c||ou|c, rec|+u|c+| |uju|,. Cu||c|e |
pu|ed |+c| W||| |u||+rr+||ou +ud |||c|eu|u
o| p|o\|r+| u+|| |o|d. 0ccu| ro| corrou|, ou
||ur|u+||(), + corpu||.e d|o|de| (||c |+|||),
c+ued |, ||e |ude\ ||ue| |epe+|ed|, p|c||u +|
cu||c|e o| ||ur|u+||.
0bsessve Compu|sve 0sorder kepe+| p|c|
|u +| ||e p+|ou,c||+ ||u c+u |eu|| |u ||c|eu |rp|e\
c||ou|cu. ' c- ecoud+|, |u|ec||ou | + cor
rou corp||c+||ou. |u e\||ere c+e, ||e u+|| p|+|e
c+u |e de||o,ed (||. !!o), u+|| ||||u.
FSChIAIkIC 0IS0k0kS
FICk 33-4 0oychauxs A
1!,e+|o|d r+|e W||| u+|| d,||o
p|, |o| dec+de. I|e |e+| |oeu+||
+ppe+| |o|, |||c|eued W||| ||+u
.e|e ||d|u (ou,c|+u\|) W||| ore
red|+| de.|+||ou (ou,c|o|,p|o|
o| |+r' |o|u de|o|r||,). I|e |+|e|+|
.|eW |oW ||e |||c|ue o| ||e u+||
p|+|e, ep+|+||ou ||or ||e u+|||ed
(ou,c|o|,|), +ud ||e r+|| +|e+ o|
+||+c|reu| |o ||e u+|| r+|||\.
FICk 33-5 habt-tc deIormty A 53,e+|
o|d r+|e W||| ||ur|u+|| de|o|r||, |o| dec+de.
I|e u+|| p|+|e o| |o|| ||ur| +|e d,||op||c W|||
||+u.e|e ||d|u +ud d|co|o|+||ou. I|e cu||c|e |
+|eu| +ud ||e p|o\|r+| u+|| |o|d e\co||+|ed. w|eu
||e p|o\|r+| u+|| +ud u+|| |o|d W||| co.e|ed W||| |+pe
cou||uu+||,, uo|r+| u+|| |e|eW |u 5 rou||.
FICk 33-6 Compu|sve oa| pcko A
5o,e+|o|d r+|e W||| u+|| d,||op|, |o| dec+de.
I|e cu||c|e +|e uo| |o|red, ||e p|o\|r+| u+|| |o|d
|u||+red +ud e\co||+|ed. I|e p+||eu| |+ |eeu
r+u|pu|+||u ||e ||ue|u+|| +pp+|+|u |o| dec+de.
I|e ||e+| |u ||e |u|e|||, p|o.|de + po||+| o| eu||,
|o| ' c- +ud +cu|e p+|ou,c||+.
KOH piepaiation and/oi nail clipping to pathol-
ogy foi PAS stain to iule out fungal coloniza-
tion/infection. Onychomycosis moie common
in nails with onycholysis.
CIINICAI FIN0INCS
Sko Typical psoiiatic lesion on nail folds
(Fig. 33-7).
Matrx
Png o r e||onyxs : Punctate depiessions;
small, shallow; vaiy in size, depth, shape (Fig.
33-7). Chaiacteiistically, isolated, deep. May
occui as iegulai lines (tiansveise; long axis)
oi giid-like pattein. Uncommon on toenails.
\o| corrou de|r+|o| +||ec||u ||e u+|| +p
p+|+|u
50 o| pe|ou W||| po||+| |+.e u+|| |u.o|.e
reu| +| oue po|u| |u ||re, W||| |||e||re |u.o|.e
reu| up |o 30-90
'ee +|o '|o||+|, 'ec||ou !
FS0kIASIS
NAII AFFAkAIS INv0IvMNI 0F CIAN0S 0ISASS
Trat|yonyt|a : Nail dull, iough, fiagile (Fig.
33-7B).
Sera| ranserse Jeressons : May mimic
washboaid" nails of tic habit (pushing back
cuticle).
LonguJna| rJgng : Resembles melted wax.
Puntae |eu|onyt|a : 1- to 2-mm white
spots in nail plate. (mistakenly attiibuted to
tiauma) (Fig. 33-7C).
Leu|onyt|a : Pioximal matiix involvement:
suiface iough and nail coaise (Fig. 33-7C).
Na| 8ed
O|" sos : Oval, salmon-coloied nail beds
(Fig. 33-7, D).
Onyt|o|yss : Secondaiy to oil" spots affect-
ing hyponychium medially oi lateially (Fig.
33-7).
SCII0N 33 ||'0k|Ek' 0| InE |A|| A||AkAIu' 100T
Co|on:aon o[ onyt|o|yt na| : May af-
fect nail bed oi undeisuiface of nail (bio-
film). CanJJa , enviionmental fungi (e.g.,
serg||us ), PseuJomonas . Piedisposes
to distal/lateial onychomycosis in toe-
nail. Up to 21% of psoiiatic nails have
secondaiy onychomycosis.
Su|ungua| |yer|eraoss : Nail plate becomes
iaised off hyponychium.
S|ner |emorr|ages .
varaot Aciodeimatitis continua of Hallopeau:
ielapsing peiiungual and subungual pustules
with onycholysis (see Section 3). Acute episodes
with onycholysis with nail bed eiythema and
scaling. Often misdiagnosed as bacteiial oi
fungal infection.
Onycholysis, onychomycosis, tiauma (toenails),
eczema, alopecia aieata.
MANACMNI
Often unsatisfactoiy. See Psoiiasis," Sec-
tion 3.
Foi matiix involvement, intialesional tiiam-
cinolone 3-5 mg/mL may be effective.
Foi nail bed psoiiasis, topical steioid (oc-
cluded) ieduces hypeikeiatosis.
Systemic theiapy such as methotiexate oi
biologics" often impioves nail appaiatus
psoiiasis.
FICk 33-T Fsorass vu|ars . \u|||p|e u+|| p|| ou ||e do|+| u+||
p|+|e, 'o|| |+|u|u o| ||e u+|| |ed, +ud d||+| ou,c|o|,|. 8. I|+c|ou,c||+
(|ou| u||+ce) W||| o|| |+|u|u, +ud d||+| ou,c|o|,|. C. |uuc|+|e |eu|
ou,c||+ | p+||ouorou|c |o| po||+| +ud r+, |e ||e ou|, ||ue|. A c+u
|e eeu |u ||e u+|| |e|oW W||| ||+ur+||c u|uuu+| |ero|||+e, puuc|+|e
|eu|ou,c||+ d|d uo| occu| +| ||| ||e o| ||+ur+. 0. 0|| |+|u|u, d||+| ou,
c|o|,|, |ou||ud|u+| ||d|u, +d|e|euce o| ||e cu||c|e |o ||e d||+| u+|| p|+|e.
8
C
0
|+|| |u.o|.ereu| occu| |u 0 o| |ud|.|du+|
W||| d|er|u+|ed ||.
|+|| +pp+|+|u |u.o|.ereu| r+, |e ||e ou|,
r+u||e|+||ou.
0ue, e.e|+|, o| +|| 20 u+|| r+, |e |u.o|.ed
('|Weu|,u+|| ,ud|ore, W|e|e ||e|e | |o o|
+|| 20 u+|| W|||ou| +u, o||e| e.|deuce o| ||c|eu
p|+uu e|eW|e|e ou ||e |od,).
'|r||+| c|+ue +|e eeu |u ||c|euo|d |+||.e|u
|o| d|e+e
Cou|e. \+, de||o, u+||.
'ee +|o '||c|eu ||+uu, 'ec||ou 1.
IIChN FIANS (IF)
CIINICAI MANIFSIAI0NS
Sko Doisum of PNF: swelling with blue/ied
discoloiation of PNF.
Matrx
Sma|| [otus n marx : Bulge undei pioximal
nail fold (Fig. 33-8).
Su|sequen |onguJna| reJ |ne : Thinned nail
plate evolving into distal split nail (onychoi-
ihexis) (Fig. 33-8B).
D[[use marx no|emen : Selective atiophy
of nail plate with onychoiihexis and/oi tians-
veise splitting.
ReJ |unu|a : Focal oi disseminated.
Me|anonyt|a, |onguJna| : Tiansitoiy.
Com|ee na| s|.
Perygum [ormaon (star, marx JesroyeJ) :
Paitial loss of the cential nail plate piesents
as a V-shaped extension of skin of piox-
imal nail fold adheient to nail bed (Fig.
33-8, B).
IJoa|t aro|y o[ na|s" : Acute pio-
giessive nail destiuction leading to diffuse
nail atiophy with and without pteiygium;
complete loss of nail (anonychia) (Fig.
33-8B, C, D).
U|terae LP : Bulla foimation, eiosion, hem-
oiihage, scaiiing; cutaneous lesions usually
piesent on palms/soles.
Na| 8ed Onycholysis, distal subungual hypei-
keiatosis, bulla foimation, peimanent anony-
chia.
varaots
20-na| Jysro|y o[ t||J|ooJ : Resolves spon-
taneously.
LP-||e eruons [o||owng |one marrow rans-
|an : Giaft-veisus-host disease.
Drug-nJuteJ LP-||e reaton .
Permanen anonyt|a : May be only manifesta-
tion of LP.
MANACMNI
See Lichen Planus," Section 7.
Intialesional tiiamcinolone
Systemic glucocoiticoids
'ee '|ouc+|||u A|opec|+, 'ec||ou !2.
\+u||e|+||ou.
Ceore|||c p||||u (||. !!9) (r+||, upe|||c|+|,
|eu|+||, d|||||u|ed |u eore|||c p+||e|u)
n+rre|ed ||+ +ppe+|+uce
\o|||ed e|,||er+ o| |uuu|+e
I|+c|ou,c||+ (|ou|ue c+ued |, e\ce|.e
|ou||ud|u+| |||+||ou)
AI0FCIA AkAIA (AA)
FICk 33-9 A|opeca areata: trachooycha I|e u+|| p|+|e |
|ou| W||| + '|+rre|ed ||+ +ppe+|+uce.
FICk 33-8 Icheo p|aous . \|dd|e ||ue|. |u.o|.ereu| o|
||e p|o\|r+| |o|d +ud r+|||\ |+ c+ued ||+c|ou,c||+, |ou||ud|u+| ||d
|u, +ud p|e|,|ur |o|r+||ou. |ude\ ||ue|. de||uc||ou o| ||e r+|||\
+ud u+|| p|+|e | corp|e|e W||| +uou,c||+. 'e.eu o| |eu ||ue|u+|| +|e
|u.o|.ed, ||e o||e| +|e uo|r+|. 8. |u.o|.ereu| o| ||e u+|| r+|||\ W|||
c+|||u o| p|e|,|ur |o|r+||ou p|o\|r+||, d|.|d|u ||e u+|| p|+|e |u
|Wo. I|e p+||eu| |+ |ep+|||| C .||u |u|ec||ou +ud o|+| |u.o|.ereu|
W||| e|o|.e ||c|eu p|+uu. C. E+||, |u.o|.ereu| o| ||e r+|||\ W||| |||u
u|u o| ||e ||ur|u+|| p|+|e. 0. '+re p+||eu| + ||. !!0C |Wo ,e+|
|+|e|, ||e u+|| p|+|e | corp|e|e|, de||o,ed, |.e., +uou,c||+.
8
C
0
C|er|c+| |u u+|| po||| +ud +d|e|.e |o| p+|e
ou u+|| c+u c+ue d+r+e |o ||e u+|| p|+|e, |.e.,
d|co|o|+||ou, ou,c|oc||/|+. |||||+u| o| +||e||c cou
|+c| de|r+|||| c+u +|o occu| ou ||e p+|ou,c||+|
||u.
ChMICAI IkkIIANI 0k AIIkCIC 0AMAC 0k 0kMAIIIIS
|+|| c|+ue +|e p+||ouorou|c. |ou||ud|
u+| ||e+| (|ed +ud W|||e), d||+| u|uuu+|
|,pe||e|+|o||c p+pu|e W||| d||+| \ o| Wede
|+ped ||u||u o| u+|| p|+|e (||. !!0).
0AkIk 0ISAS (0AkIk-WhII 0ISAS, kkAI0SIS F0IIICIAkIS)
FICk 33-10 0arer dsease A o5,e+|o|d |er+|e W||| p|u||||c |+| ou ||uu| |o| dec+de. E|,||er+|ou
e\co||+|ed p+pu|e ou ||e ||uu| W||| |ed +ud W|||e |ou||ud|u+| ||e+| ou ||e ||ue|u+||.
FICk 33-11 Chemca||y damaed oa| |+|e u+|| |ued |o ||e ||ue|u+|| |+ c|er|c+||, d+r+ed ||e
u+|| p|+|e W||| |eu|ou,c||+ +ud ou,c|oc||/|+.
!c|-|c|. I+u, ||oWu, o| ||+c| |ou||ud|u+|
||e+| W||||u u+|| p|+|e (||. !!!).
|c||--. () |uc|e+ed re|+u|u ,u||e|
|u uo|r+||, uou|uuc||ou+| r+|||\ re|+uoc,|e,
(2) |uc|e+e |u |o|+| uur|e| o| re|+uoc,|e
,u||e|/|u re|+u|u, (!) ue.ore|+uoc,||c ue.u
(juuc||ou+|, ||. !!!).
0-| . Coueu||+| o| +cqu||ed. \o| o|||u+|e |u
d||+| r+|||\.
|||--|c| !c . |oc+| +c||.+||ou o| u+||
r+|||\ (e.., ||+ur+), |,pe|p|+|+ o| u+|| r+
|||\ re|+uoc,|e, ue.ore|+uoc,||c ue.u (juuc
||ou+|), d|u|uduced e.., /|do.ud|ue (A/I)|
|,d|o\,c||o|oqu|ue, o| re|+uor+ o| u+|| r+|||\.
I0NCII0INAI MIAN0NChIA
Beu|u |uro|. ||||or+/||||o|e|+|or+, u|uuu+|
e\o|o|, r,\o|d c,|, |oru |uro| (p+|u|u| |ed
u+|| |ed p+|c|), ou,c|or+|||\or+, u+|| r+|||\ ue.|.
\+||u+u| |uro|. 'qu+rou ce|| c+|c|uor+,
re|+uor+, \e||e| ce|| |uro|.
N0FIASMS 0F Ih NAII AFFAkAIS |C|9. 10!.3
('ee '||||+| \,\o|d C,|, 'ec||ou 9)
|eudoc,| o| +u||ou o|||u+|e |u d||+| |u|e|
p|+|+ue+| jo|u|, +oc|+|ed W||| o|eo+|||||||
(ne||e|deu uode).
|e|ou c+u p|eeu| ou ||e p|o\|r+| u+|| |o|d
(||. !!2), +|o.e +ud corp|e|u ||e r+|||\,
|eu|||u |u + |ou||ud|u+| dep|eed |oo.e |u ||e
u+|| p|+|e.
w|eu c,| e\p+ud |e|Weeu ||e pe||o|eur +ud
r+|||\, u+|| |ecore d,||op||c W||| + du|, |ed
|uuu|+.
MX0I0 CSIS 0F 0ICIIS
FICk 33-12 Myxod cysts A o2,e+|o|d
r+|e W||| u+|| de|o|r||, o| oue ,e+|' du|+||ou. |e|
r+| e|,||er+ +ud We|||u o| ||e p|o\|r+| u+|| |o|d
W||| +oc|+|ed |ou||ud|u+| |oo.e o| ||e u+|| p|+|e.
C|e+| e|+||uou ||u|d |+ d|+|ued ||or ||e |ude\
||ue| ou ||e |||| (c|u|ed ||e). |eeue|+||.e jo|u|
d|e+e | p|eeu| |u |o|| d||+| |u|e|p|+|+ue+| jo|u|.
FICk 33-13 luoctooa| oevome|aoocytc
oevus oI the oa| matrx A juuc||ou+| ue.u |
p|eeu| |u ||e u+|| r+|||\ |eu|||u |u + |ou||ud|u+|
||oWu |||pe |u ||e u+|| |ed. I|e p|o\|r+| u+|| |o|d/
cu||c|e +|e uo| p|reu|ed.
Appe+| + |ou||ud|u+| re|+uou,c||+ (||. !!!).
0ue|. c|||d|ood.
Cou|e. co|o| +ud W|d|| c|+ue W||| +|u.
NAII MAIkIX NvI
!-c c- . 55-o0 ,e+|. |:!-:- . 2-! o|
re|+uor+ |u W|||e, 5-20 |u ||+c|, A|+u,
|+||.e Are||c+u. uu+||, +,rp|or+||c, ro|
p+||eu| uo||ce p|reu|ed |e|ou, epec|+||, +||e|
||+ur+.
|-c|c|||, . |u ||u o| |u.+|.e.
|! . A||e u|uuu+||, o| pe||uuu+||,, p|e
eu||u W||| |ou||ud|u+| re|+uou,c||+ +ud/o|
u+|| p|+|e d,||op|, (||. !!+). \+|||\ |e|ou
uu+||, p|eeu| + A|\ |u W|||e o| ||o+deu|u
o| +u e\|||u A|\ |u ||+c|.
||:| . |e||uuu+| e\|eu|ou o| ||oWu
||+c| p|reu|+||ou ou|o p|o\|r+| +ud |+|e|+| u+||
|o|d (||. !!+).
25 o| A|\ r+, |e +re|+uo||c (p|reu|+||ou
uo| o|.|ou o| p|or|ueu|).
||||. I|ur|, |e+| |oe (|+||u\).
|||--|c| !c . 'u|uuu+| |ero|||+e.
|!:c| | |, . |e||uuu+| p|reu|+||ou,
+du|| +e, c|+ue |u co|o|/W|d|| o| |+ud, |,pe|
p|reu|ed ||ue W||| ||e |+ud, p|o\|r+| po|||ou
o| |+ud W|de| ||+u d||+|, ||ur|, |ude\ ||ue|,
o| |oe |u.o|.ereu|, ||u||ed r+||u, |||o|, o|
||+ur+.
|. 5,e+| u|.|.+| |+|e ||or !5-50.
ACk0INIICIN0S MIAN0MA (AIM) ('ee 'ec||ou 2)
'CC |u ||u ('CC|') occu|||u pe||uuu+||, |
uu+||, c+ued |, ||e oucoeu|c |ur+u p+p||
|or+.||u (n|\) |,pe o +ud 3.
|! . '||uco|o|ed o| |,pe|p|reu|ed, |e|+
|o||c, |,pe||e|+|o||c, o| W+||, p+pu|e/p|+que,
ou,c|o|,|, |+||u|e o| u+|| |o|r+||ou.
|||| . ||o\|r+| +ud |+|e|+| u+||, r+|||\,
|,pou,c||ur (||. !!5).
|.c.- 'CC +||e W||||u 'CC|'.
',| . |+|u || pe||o|e+| |u.+|ou |+ oc
cu||ed.
|! . 'o|||+|, uodu|e | ro| corrou,
o||eu de||o,|u ||e u+||.
|||| . \uc| ro|e corrou ou ||ue|
(||ur| +ud |ude\ ||ue| ro| o||eu) ||+u |oe,
ru|||p|e ||ue| r+, |e |u.o|.ed |u ||e |rru
uocorp|or|ed |o|.
!cc--| . \o| u|e|, o| +rpu|+||ou o|
d||| |o| ro|e deep|, |u.+|.e |e|ou |u.o|.|u
pe||o|eur.
SAM0S CII CAkCIN0MA ('ee 'ec||ou )
FICk 33-14 Acro|eotoous me|aooma I|e re|+uor+ +|oe |u ||e u+|| r+|||\ o| ||e ||ur| W|||
|eu||+u| u+|| p|+|e d,||op|,, u|uuu+| re|+uo|, +ud e\|eu|ou |u|o ||e p|o\|r+| u+|| |o|d +ud |e,oud ||
(nu|c||uou |u). ('ee +|o ||. 22.)
FICk 33-15 hFv-oduced o stu squamous ce|| carcooma o hIv[AI0S: oa| bed aod |aos peos
A 5,e+|o|d r+|e W||| n|\/A||' +ud ||ue|u+|| d,||op|,. 'CC|' |+d |eeu e\c|ed |, \o| r|c|o|+p||c u|e|,
5 ,e+| p|e.|ou|,. I|e |||| |ude\ ||ue|u+|| |ed W||| |,pe||e|+|o||c |+||u|e o| u+|| p|+|e |o|r+||ou. ||u| p+pu|e
+|e eeu ou ||e |+u peu|. B|op, o| ||e u+|||ed +ud peu||e p+pu|e |epo||ed 'CC|' W||| n|\|uduced c|+ue
(|o||oc,|o|).
|e|r+|op|,|e +|e ||e ro| corrou p+||oeu
|u|ec||u ||e u+|| +pp+|+|u.
' c- +ud |oup A ||ep|ococcu c+ue +cu|e
o|| ||ue |u|ec||ou o| ||e u+|| |o|d.
Cc!!c +ud ' c- c+u c+ue ecoud+|,
|u|ec||ou o| c||ou|c p+|ou,c||+.
kecu||eu| |e|pe |rp|e\ .||u |u|ec||ou.
INFCII0NS 0F Ih NAII AFFAkAIS |C|9. o3.9
Acu|e |u|ec||ou o| |+|e|+| o| p|o\|r+| u+|| |o|d.
uu+||, +oc|+|ed W||| ||e+| |u |u|e|||, o| ep|
de|r| (e.., |+u u+||), ||+ur+.
|! . I||o|||u p+|u, e|,||er+, We|||u,
p+|u, +|ce |o|r+||ou (||. !!o).
|u|ec||ou r+, e\|eud deepe|, |o|r|u + |e|ou.
ACI FAk0NChIA |C|0
|0!.0
8A0T8|AL |hF0T|0hS
' c- | ||e ro| corrou c+ue o| +cu|e p+|o
u,c||+.
'o|| ||ue |u|ec||ou o| pu|p p+ce o| d||+| p|+|+u\
(||. !!1), c|oed p+ce |u|ec||ou o| ru|||p|e
corp+||reu| c|e+|ed |, ||||ou ep|+ p+|u
|e|Weeu ||e ||u +ud pe||o|eur.
||, . |eue||+||u |uju|,, p||u|, p+|ou,c||+.
|! . |+|u, e|,||er+, We|||u, +|ce (||.
!!1), +|ce r+, ||e+| |u ceu|e| o| pu|p p+ce
+ud decorp|e pou|+ueou|,, W||| |ou| o|
uec|o||c ||u o.e| pu|p p+ce.
|||| . I|ur|, |ude\ ||ue|.
C|:c| . 0|e|||, o|eor,e|||| o| d||+|
p|+|+u\, eque||+||ou o| d|+p|,| o| ||e p|+
|+u\, |up|u|e |u|o d||+| |u|e|p|+|+ue+| jo|u| W|||
ep||c +|||||||, e\|eu|ou |u|o d||+| eud o| ||e\o|
|eudou |e+||, p|oduc|u |euo,uo.|||.
C- . \+, |e |+p|d +ud e.e|e, cou|+|ued
|, uu,|e|d|u ||u o| ||ue|||p, |u|ec||ou c|e+|e
|eu|ou W||| r|c|o.+cu|+| corp|or|e, uec|o|,
+ud +|ce |o|r+||ou.
FI0N |C|9 . o3.0
|C|0 . |0!.0
F0h6AL |hF0T|0hS Ah0
0hY0h0NY00S|S
Cc!!c pp. uu+||, c+ue 'p+ce'' |u|ec||ou o|
c||ou|c p+|ou,c||+ o| ou,c|o|,||c u+|| +ud c+u
c+ue de||uc||ou o| ||e u+|| |u ||e |rruuocorp|o
r|ed |o|.
|e|r+|op|,|e |u|ec| ||e ||u +|ouud ||e u+|| +p
p+|+|u +ud c+ue upe|||c|+| de||uc||ou o| u+||.
Eu.||oureu| |uu| c+ue ecoud+|, co|ou|/+||ou o|
d|e+ed u+|| +ud +|e |+|e|, p||r+|, p+||oeu.
0,:|,: . C||ou|c p|o|e|.e |uu+| |u|ec
||ou o| u+|| +pp+|+|u, ro| corrou|, c+ued |,
de|r+|op|,|e, |e o||eu |, Cc!!c pp., ro|d
c+u |e cu||u|ed ||or d|e+ed u+|| |u| +|e uo|
p||r+|, p+||oeu.
Cc!!c ,:|c . 0u,c|or,co| c+ued |, Cc
!!c pp.
-c . 0u,c|or,co| c+ued |, de|
r+|op|,|e.
|e|ou | +u +cu|e |u|ec||ou o| ||e ||ue| ||p.
!cc--|. 'ee 'Au||r|c|o||+| I|e|+p,,
'ec||ou 2+.
to|oy C. a||tans and othei species. Noimal
floia, which causes infection if local ecology
is changed in favoi of yeast oi in association
with alteied immune status. See Candidiasis,"
Section 25.
C|assIcatoo
Subungual infection associated with ony-
cholysis
Inteimittent flaies of chionic paionychia
Colonization in tinea unguium
Cc!!c c||:c |u|ec||ou o| ||e u+|| +pp+|+|u
occu| ro| o||eu ou ||ue|, ro| corrou|, +
ecoud+|, |u|ec||ou o| c||ou|c p+|ou,c||+.
Cc!!c c+u c+ue d||+| +ud |+|e|+| ou,c|o|,|,
epec|+||, |u d|+|e||c.
|u.+|ou o| u+|| p|+|e uu+||, occu| ou|, |u ||e
|rruuocorp|or|ed |o|, |.e., c||ou|c rucocu
|+ueou c+ud|d|+| (C\C) o| n|\/A||' d|e+e.
CANDIDA 0NChIA
Total nail dystiophy (TND) (Fig. 33-18):
CMC and HIV/AIDS disease.
Chrooc Mucocutaoeous Caoddass See Can-
didiasis," Section 25.
CIINICAI FIN0INCS
See Candidiasis," Section 25.
hIv[AI0S Candidal onychia and paionychia
aie common in childien with HIV/AIDS, of-
ten associated with mucosal candidiasis.
FICk 33-16 Acute parooycha A +0,e+|o|d
|e+|||, r+|e W||| p+|u|u| We|||u o| |||||ude\
||ue|. I|e p|o\|r+| u+|| |o|d |ed +ud eder+|ou (ce|
|u||||) W||| pu |o|r+||ou.
FICk 33-1T Fe|oo A o0,e+|o|d |er+|e W|||
p+|u|u| ||ue| |o| ! d+,. Au +|ce | eeu ou ||e
||ue|||p W||| u||ouud|u e|,||er+ +ud We|||u.
\e|||c||||ueu|||.e ' c- (\''A) W+ |o|+|ed ou
cu||u|e o| ||e pu.
',rp|or. uu||||,, u+|| |oe p|o|ec||.e +ud
r+u|pu|+||.e |uuc||ou,
Corp||c+||ou.
|+|u |u |oeu+|| W||| p|eu|e ||or |oe
||ed|poe |o ecoud+|, |+c|e||+| |u|ec||ou
u|ce|+||ou o| ||e uude|||u u+|| |ed
Corp||c+||ou occu| ro|e corrou|, |u ||e
|oW|u popu|+||ou o| |rruuocorp|or|ed |u
d|.|du+| +ud d|+|e||c
'ee +|o 'ec||ou 25
IINA NCIM[0NCh0MC0SIS
CIASSIFICAII0N 8 ANAI0MIC
SII INv0Iv0
0sta| aod Iatera| Subuoua| 0oychomycoss
(0IS0) (Figs. 33-19 and 33-20) Infection be-
gins in stiatum coineum of hyponychial aiea
oi nail fold, extending subungually, and pio-
giessively involves the nail centiipetally and
medially. May be eithei piimaiy, i.e., involving
a healthy nail appaiatus, oi secondaiy (e.g.,
psoiiasis) associated with onycholysis. FnJngs :
Onycholysis, subungual hypeikeiatosis, yellow-
biown discoloiation of keiatinaceous debiis.
Always associated with tinea pedis.
SuperIca| Whte 0oychomycoss (SW0)
Pathogen invades suiface of doisal nail (Fig. 33-
21). Eo|ogy : Trt|o|yon menagro|yes oi T.
ru|rum (childien). Much less commonly, mold:
tremonum , Fusarum , serg||us erreus .
Froxma| Subuoua| 0oychomycoss (FS0)
Pathogen enteis by way of the posteiioi nail
fold-cuticle aiea and then migiates along the
Na| Apparatus ChrvnIc PurvnychIu wIth
Acute CundIdu| F|ure CanJJa spp. can
cause inteimittent painful infection of chionic
paionychia with pain, tendeiness, eiythema,
pus. Nail may become dystiophic with aieas
of opacification; white, yellow, gieen, oi black
discoloiation; with tiansveise fuiiowing.
Suhunguu| CundIdIusIs i Ahscess These oc-
cui in onycholytic space. Risk factoi: diabetes.
Cv|vnIzutIvn In TIneu UnguIum Secondaiy
pathogen in distal/lateial onychomycosis.
Tvtu| NuI| Dystrvphy Pioximal/lateial nail
folds aie inflamed and thickened. Fingeitips
appeai bulbous. Nail is invaded and may even-
tually become totally dystiophic (Fig. 33-18).
Nail appaiatus thickens due to nail dystiophy
and subungual hypeikeiatosis. HIV/AIDS: one
nail may be involved. CMC: 20 nails may be
involved in time.
0ther Fodos See Candidiasis," Section 25 .
Tinea unguium, psoiiasis, eczema, chionic pai-
onychia, lichen planus.
MANACMNI
See Candidiasis," Section 25.
FICk 33-18 Cooddo ooychomycoss: tota|
dystrophc type I|e eu|||e ||ue|u+|| p|+|e |
|||c|eued +ud d,||op||c +ud | +oc|+|ed W||| +
p+|ou,c||+| |u|ec||ou, |o|| ||ud|u We|e c+ued |,
C c||:c |u +u |ud|.|du+| W||| +d.+uced n|\/A||'
d|e+e.
pioximal nail gioove to involve the undeilying
matiix, pioximal to the nail bed, and finally
the undeilying nail (Fig. 33-22). Eo|ogy : T.
ru|rum. FnJngs : Leukonychia that extends
distally fiom undei pioximal nail fold. Usually
one oi two nails involved. Always associated
with immunocompiomised states.
Ae oI 0oset Childien oi adults. Once ac-
quiied, usually does not iemit spontaneously.
Theiefoie, the incidence incieases with advanc-
ing age; 1% of individuals <18 yeais affected;
almost 50% of those > 70 yeais.
Sex Somewhat moie common in men.
to|oc Aeots Between 95 and 97% caused
by T. ru|rum and T. menagro|yes. Much less
common: EJermo|yon [|ottosum, T. o|-
ateum, T. st|oen|en, T. errutosum (usually
infects only fingeinails).
Mv|ds Raiely, piimaiy pathogens in ony-
chomycosis, but iathei secondaiily colo-
nize alieady dystiophic nails/nail appaiatus.
tremonum, Fusarum , and serg||us spp. can
iaiely cause SWO. Deimatosis such as psoiiasis,
which iesults in onycholysis and subungual hy-
peikeiatosis, oi deimatophytic onychomycosis
can be secondaiily colonized/infected by molds.
Moie than 40 mold species have been iepoited
FICk 33-19 0oychomycoss oI toeoa|s: dsta| aod |atera| subuoua| type (0IS0) A +,e+|o|d
|er+|e W||| |oeu+|| d,||op|, |o| 2 ,e+| +ud +|op|c de|r+||||. I|e |oeu+|| +|e W|||e, c+ued |, ou,c|o|,| +ud
u|uuu+| |,pe||e|+|o|. I|e do|ur o| ||e |ee| |oW e|,||er+ +ud c+||u, |.e., ||ue+ ped|. |A' |+|u o| u+||
c||pp|u |oW ep|+|e |,p|+e. | W+ de|ec|ed ou cu||u|e.
FICk 33-20 0oychomycoss oI toeoa|s: dsta| aod |atera| subuoua| type (0IS0) A o9,e+|o|d
r+|e W||| |oeu+|| d,||op|, |o| dec+de. I|e |oeu+|| |oW +d.+uced d,||op|,. |||+| ou,c|o|,| +ud u|uuu+|
|,pe||e|+|o| c+ue +pp+|eu| d|co|o|+||ou o| ||e u+||. I|e u+|| p|+|e ||e|| | uo| |||c|eued. Co|ou|/+||ou o| ||e
|,pe||e|+|o||c u+|||ed W||| eu.||oureu|+| ro|d o| |-!c |uc|e+e ||e d|co|o|+||ou.
to be isolated fiom dystiophic nails, including
Stou|aross |retau|s, serg||us spp., |-
ernara spp., tremonum spp., Fusarum spp.,
Stya|Jum JmJaum (HenJersonu|a oru|o-
Jea), S. |ya|num .
EtIv|vgy v] AnutvmIc Types v] OnychvmycvsIs
DLSO: T. ru|rum, T. menagro|yes. PSO: T.
ru|rum . SWO: T. menagro|yes .
Ceoraphc 0strbutoo Woildwide. Etiologic
agent vaiies in diffeient geogiaphic aieas. Moie
common in uiban than in iuial aieas (associ-
ated with weaiing occulsive footweai).
Freva|eoce Incidence vaiies in diffeient geo-
giaphic iegions. In the United States and Euiope,
up to 10% of adult population affected (ielated
to occlusive footweai). In developing nations
wheie open footweai is woin, uncommon.
Iraosm ssoo Dermutvphytes Anthio-
pophilic deimatophyte infections aie tiansmit-
ted fiom one individual to anothei, by fomite oi
diiect contact, commonly among family mem-
beis. Some spoie foims (aithioconidia) iemain
viable and infective in the enviionment foi up
to 5 yeais.
Mv|ds Ubiquitous in enviionment; not tians-
mitted between humans.
ksk Factors Atopics aie at incieased iisk foi T.
ru|rum infections. Diabetes mellitus, tieatment
with immunosuppiessive diugs, HIV/AIDS. Foi
toenail onychomycosis, most impoitant factoi
is weaiing of occlusive footweai.
FAIh0CNSIS
Frmary 0oychomycoss[Ioea ouum
Invasion occuis in an otheiwise healthy nail.
The piobability of nail invasion by fungi in-
cieases with defective vasculai supply (i.e., with
incieasing age, chionic venous insufficiency,
peiipheial aiteiial disease), in posttiaumatic
states (lowei leg fiactuies), oi distuibance of
inneivation (e.g., injuiy to biachial plexus,
tiauma of spine).
Secoodary 0oychomycoss Infection occuis in
alieady alteied nail appaiatus, such as psoiiatic
oi tiaumatized nail. Toenail onychomycosis
usually occuis aftei tinea pedis; fingeinail in-
volvement associated with tinea manuum, tinea
coipoiis, oi tinea capitis. Infection of fiist and
fifth toenails piobably occuis secondaiy to
damage to these nails by footweai.
0IS0 (Figs. 33-19 and 33-20) Nail bed pio-
duces soft keiatin stimulated by fungal infec-
tion that accumulates undei the nail plate,
theieby iaising it, a change that clinically gives
involved nail an alteied cieam coloi iathei than
noimal tianspaient appeaiance. Dense keiatin
of nail plate is not involved piimaiily. Accu-
mulated subungual keiatin piomotes fuithei
fungal giowth and keiatin pioduction. Matiix is
usually not invaded, and pioduction of noimal
nail plate iemains unimpaiied despite fungal
infection. In time, deimatophytes cieate aii-
containing tunnels within the nail plate; wheie
the netwoik is sufficiently dense, nail is opaque.
Often invasion follows longitudinal iidges of
nail bed. Subungual location of infection pie-
vents effective topical antifungal agents.
Appioximately 80% of onychomycosis occuis
on the feet, especially on the big toes; simulta-
neous occuiience on toe- and fingeinails is not
common.
DLSO White patch is noted on the distal oi
lateial undeisuiface of the nail and nail bed,
usually with shaiply demaicated boideis. In
time, whitish coloi can become discoloied to
a biown oi black hue. Piogiessive involvement
of nail can occui in a mattei of weeks, as in
HIV/AIDS, oi moie slowly ovei a peiiod of
months oi yeais. With piogiessive infection,
the nail becomes opaque, thickened, ciacked,
fiiable, iaised by undeilying hypeikeiatotic de-
biis in hyponychium (Figs. 33-19 and 33-20).
Shaiply maiginated white stieaks beginning at
the distal nail maigin and extending pioximally
aie filled with keiatinaceous debiis and aii.
Toenails aie involved much moie commonly
than fingeinails. Fiist and fifth toenails aie
infected most fiequently. Involvement of the
fingeinails is usually unilateial. When fingei-
nails aie involved, pattein is usually two feet
and one hand.
SWO A white chalky plaque is seen on the
pioximal nail plate, which may become eioded
with loss of the nail plate (Fig. 33-21). Diag-
nostically, the involved nail can be iemoved
easily with a cuiette in compaiison with a tiau-
matized nail, which has white, aii-containing
aieas. In some cases, the entiie supeificial nail
plate may become involved. SWO may coexist
with DLSO. Occuis almost exclusively on the
toenails, iaiely on the fingeinails.
PSO (Fig. 33-22) A white spot appeais fiom
beneath pioximal nail fold. In time, white
discoloiation fills lunula, eventually moving
distally to involve much of undeisuiface of the
nail. Patients tieated with oial azoles show in-
teiiuption of involved nail. Occuis moie com-
monly on toenails.
0IS0 Psoiiatic nails (oil diop" staining of the
distal nail bed and nail pits is seen in psoiiasis
but not onychomycosis), paionychial psoiiasis
oi eczema. Reitei syndiome and keiatodeima
blennoiihagicum, onychogiyphosis, pincei
nails, congenital nail dystiophies.
SW0 Tiaumatic oi chemical injuiy to nail,
psoiiasis with leukonychia.
All clinical diagnoses of onychomycosis should
be confiimed by laboiatoiy testing (see Dei-
matophytoses," Section 25).
Na| Samp|es Foi DLSO: distal poition of
involved nail bed; SWO: involved nail suiface;
PSO: punch biopsy thiough nail plate to in-
volved nail bed.
0rect Mcroscopy Diiect micioscopic exami-
nation of nail samples is used to confiim the
clinical diagnosis. Keiatinaceous mateiial fiom
involved nail sciapings is placed on glass slide,
coveied with glass coveislip, suspended in a
solution of potassium hydioxide (KOH), and
gently heated. Specific identification of patho-
gen is usually not possible by micioscopy, but,
in most cases, yeasts can be diffeientiated fiom
deimatophytes by moiphology.
Fuoa| Cu|ture Isolation of the pathogen
peimits bettei use of oial antifungal agents.
Samples of infected nail aie inoculated onto
Sabouiaud agai with oi without cycloheximide.
Isolation of mold fiom psoiiatic nail oi tinea
unguium is mostly colonizei and not piimaiy
pathogen.
hsto|oy oI Na| C|ppo Indicated if clinical
findings suggest onychomycosis aftei negative
KOH wet mounts. PAS stain is used to detect
fungal elements in the nail. Mos re|a||e et|-
nque [or Jagnosng onyt|omytoss .
0IACN0SIS
C|nta| Jagnoss s neer aJequae. Clinical
findings confiimed by finding fungal foims in
KOH piepaiation, nail clipping, and/oi isola-
tion of pathogenic fungus on cultuie.
C0kS AN0 Fk0CN0SIS
Without effective theiapy, onychomycosis does
not iesolve spontaneously; piogiessive involve-
ment of multiple toenails is the iule. DLSO
FICk 33-21 0oychomycoss oI toeoa|s:
superIca| whte type (SW0) I|e do|+| u+||
p|+|e | c|+||, W|||e. w|||e u+|| d,||op|, c+u e+||,
|e ||e+|ed |, cu|e||+e, K0n p|ep+|+||ou o| ||e cu|e|
||u |oW |,p|+e.
FICk 33-22 Ioeo uoyuum: proxma| sub-
uoua| ooychomycoss type (FS0) I|e p|o\|r+|
u+|| p|+|e | + c|+||, W|||e co|o| due |o |u.+|ou ||or
||e uude|u||+ce o| ||e u+|| r+|||\. I|e p+||eu| |+d
+d.+uced n|\/A||' d|e+e.
peisists aftei topical tieatment of tinea pedis
and often iesults in iepeated episodes of epi-
deimal deimatophytosis of feet, gioin, and
othei sites. Tinea pedis and/oi DLSO piovide
poital of entiy foi iecuiient bacteiial infections
(S. aureus , gioup A stieptococcus), especially
cellulitis of lowei leg aftei venous haivesting.
Pievalence in diabetics estimated to be 33%;
DLSO contiibutes to seveiity of foot piob-
lems: supeificial bacteiial infection, ulceiation,
cellulitis, osteomyelitis, neciosis, amputation.
Da|ets neeJ ear|y nerenon anJ s|ou|J
|e streeneJ regu|ar|y |y a Jermao|ogs anJ/or
oJars . Untieated HIV/AIDS is associated
with incieased pievalence of deimatophytoses.
IA8I 33-1 Nanaement of Iinea Unuium
0ebrdemeot |e|||de d,||op||c u+||, p+||eu| |ou|d de|||de Wee||,. |u ||'0, u+|| +ud |,pe||e|+|o||c
u+|| |ed |ou|d |e |ero.ed. |u 'w0, +|uo|r+| u+|| c+u |e de|||ded W||| cu|e||e.
Iopca| aeots A.+||+||e + |o||ou +ud |+cque|. |c||, | -||-:|.- e\cep| |o| 'w0.
C:| (|-|c:) c| |c:-. rou|||, p|o|e|ou+| u+|| de|||dereu| |ecorreuded.
Systemc aeots ||-. |u ,|er|c ||e+|reu| o| ou,c|or,co|, u+|| uu+||, do uo| +ppe+| uo|r+| +||e|
||e ||e+|reu| ||re |ecorreuded |ec+ue o| |oW |oW|| o| u+||. || cu||u|e +ud K0n
p|ep+|+||ou +|e ue+||.e +||e| ||ee ||re pe||od, red|c+||ou c+u uoue||e|e |e
|opped +ud u+|| W||| uu+||, |e|oW uo|r+||,.
A||y|amoes \o| e||ec||.e ++|u| de|r+|op|,|e |u|ec||ou, +|o e|||c+c|ou ++|u| e|ec|ed o||e| |uu|.
Ie|||u+||ue 250 r/d |o| o Wee| |o| ||ue|u+|| +ud 2-o Wee| |o| |oeu+||.
Ato|es ||u |u ||| c+|eo|, +|e uu+||, e||ec||.e |u ||e+|reu| o| u+|| |u|ec||ou c+ued |,
de|r+|op|,|e, ,e+|, +ud ro|d.
|||+cou+/o|e. +pp|o.ed 200 r/d |o| o Wee| (||ue|u+||), 2 Wee| (|oeu+||) (cou||uuou ||e|+p,). 200 r
(u'A) |o| |W|ce d+||, |o| |||| 1 d+, o| e+c| rou|| |o| 2 rou|| (||ue|u+||) (pu|e do|u).
ou,c|or,co|. A|||ou| uo| +pp|o.ed |o| |oeu+|| ou,c|or,co|, pu|e do|u | ued, |.eu |o| !-+
E||ec||.e |u rou||.
de|r+|op|,|e +ud
Cc!!c ou|,.
||ucou+/o|e. uo| kepo||ed e||ec||.e +| do|u o| 50-+00 r d+, pe| Wee| o| 00-200 r/d uu|||
+pp|o.ed (u'A) |o| ||e u+|| |oW |+c| uo|r+||,. E||ec||.e |u ,e+| +ud |e o |u de|r+|op|,|e.
ou,c|or,co|.
E||ec||.e |u
de|r+|op|,|e +ud
Cc!!c.
Ke|ocou+/o|e. uo| ||o|oued ||e|+p, + |o| ou,c|or,co| |+ |||e| |uc|deuce o| ||.e| |uuc||ou
+pp|o.ed |o| +|uo|r+||||e.
ou,c|or,co|.
E||ec||.e +| 200 r/d, ro|e e||ec||.e |o| Cc!!c ||+u de|r+|op|,|e, |oWe.e|,
|u||equeu||, |ep+|o|o\|c||, +ud +u||+ud|oeu e||ec| |+.e ||r||ed || |ou|e|r ue |o|
ou,c|or,co|.
Secoodary prophy|axs kecorreuded |o| +|| p+||eu|. I|e eu|||e|, o| |o|| |ee| |ou|d |e ||e+|ed. ||op|,|+\|
|ou|d |e |rp|e |o ue +ud |ue\peu|.e.
Au|||uu+| c|e+r, |o||ou, o| poWde| d+||,.
Au||ep||c e|. e||+uo| o| |op|op,| +|co|o|.
|ed|cu|e/r+u|cu|e. r+|e u|e |u||ureu| +|e |e||||/ed o| |ud|.|du+| |+.e ||e|| oWu.
Long-teim ielapse iate with newei oial agents
such as teibinafine oi itiaconazole iepoited
to be 15-21% 2 yeais aftei successful theiapy;
long-teim follow-up studies not yet iepoited.
Causes of ielapse/ieinfection unceitain: ie-
infection, immunologic incompetence, pei-
sistent tiauma, unknown causes. Mycologic
cultuies may be positive without any clini-
cally appaient disease. Nail/foot hygiene is im-
poitant: benzoyl peioxide soap in showei oi
antifungal piepaiation oi ethanol/isopiopyl gel.
MANACMNI
See Section 25 and Table 33-1.
Iodcatoos Ior Systemc Iherapy Fingeinail
involvement, limitation of function, pain (thick-
ened gieat toenails with piessuie on nail bed,
ingiowing toe nails), physical disability, poten-
tial foi secondaiy bacteiial infection, souice of
iecuiient epideimal deimatophytosis, quality-
of-life issues (pooiei peiceptions of geneial and
mental health, social functioning, physical ap-
peaiance, difficulty in tiimming nails, discom-
foit in weaiing shoes). Eaily onychomycosis
easiei to cuie in youngei, healthiei individuals
than in oldei individuals with moie extensive
involvement and associated medical conditions.
I s essena| o roe ([unga|) n[eton |e[ore
sarng sysemt reamen, J[[erenae ony-
t|omytoss [rom o|er na| Jysro|es.
A W|de pec||ur o| ,|er|c d|o|de| c+u +||ec| ||e u+|| +pp+|+|u.
NAII SICNS 0F MIIISSIM 0ISASS |C|9. 10!.3
',|er|c d|e+e |rp||c+|ed || +|| 20 u+|| |u.o|.ed.
|c||-- . 0ccu| +||e| +u, e.e|e, uddeu, +cu|e,
p+|||cu|+||, |e||||e |||ue, d+r+e |o r+|||\. |||
, . n|| |e.e|, po|u+|+|, c,|o|o\|c d|u, e.e|e
+d.e|e cu|+ueou d|u |e+c||ou. |! . I|+u
.e|e, |+ud|||e dep|e|ou |u u+||, e\|eud|u ||or
oue |+|e|+| ede |o ||e o||e|, +||ec||u +|| u+|| +|
co||epoud|u |e.e| (||. !!2!). || du|+||ou o| d|
e+e corp|e|e|, |u||||| r+|||\ +c||.||, |o| 1-+ d+,,
||+u.e|e dep|e|ou |eu|| |u |o|+| d|.||ou o| u+||
p|+|e (ou,c|or+de|). \u|||p|e p+|+||e| ||ue W|||
c|ero||e|+p,. |c| . I|ur|u+|| (||ue p|eeu|
|o| o-9 rou||) +ud |+|e u+|| (||ue p|eeu| |o| up
|o 2 ,e+|) +|e ro| |e||+||e r+||e|.
IkANSvkS 0k 8A IINS: 20 NAIIS
Irue Ieukooycha A|||||u|+||e |o r+|||\ d,
|uuc||ou.
|c| |-|,:|c . uu+||, |u|e|||ed
'|||c| |-|,:|c . |||+| u+|| p|u|
c.-- |-|,:|c . |o 2rr W|de +|cu+|e
|+ud
|:|c|- |-|,:|c . |o||+|, ||+ur+
||!c| |-|,:|c . |+||e| d|e+e (||.
!!0)
Fseudo|eukooycha 'upe|||c|+| W|||e ou,c|or,
co| (||. !!2), c|er|c+| d+r+e |o u+|| |e|+||u.
Appareot Ieukooycha |ue |o +||e|+||ou o|
r+|||\ +ud/o| u+|| |ed (e.., +pp+|eu| r+c|o|uuu|+),
r+, |u.o|.e +|| ||ue|u+||.
-,|,- |-|,:|c
1:c|. nep+||c d|o|de|.
|!. 0p+que W|||e p|+|e o|cu||u |uuu|+
+ud e\|eud|u |o W||||u -2 rr ||or d||+|
ede o| u+|| (||. !!2+). |u.o|.e +|| u+||
e.eu|,.
|-: |c||c!|c|| |c| | |!c,
1:c| . keu+| d|o|de|.
|!. ||o\|r+| u+|| du|| W|||e o|cu||u
|uuu|+ (20-o0 o| u+||), d||+| u+|| p|u|/|ed
d||.
3c!-! c| (!-|:|- |-) (||. !!!5)
|+||ed, u+||oW, W|||e ||+u.e|e |+ud.
1:c| . C+uce| +u||ueop|+||c c|ero
||e|+p,, |,po+||ur|uer|+, uu||+|e|+| |o||oW|u
||+ur+.
|! . B+ud +|e p+|+||e| |o |uuu|+, ep+|+|ed
||or oue +uo||e|, +ud ||or |uuu|+, |, |||p o|
p|u| u+||.
Ik0NChIA
',|. |+|| |op |oW|u. 1:c| . |,rp|
eder+, |ep||+|o|, ||+c| d|e+e (||ouc||ec|+|,
c||ou|c ||ouc||||, r+||u+u| ueop|+r), ||eur+
|o|d +|||||||, |u|e|u+| r+||u+uc|e. |c||-- .
A||e| |u u+|| |oW||. |! . |+|| |+|d, e\ce|.e|,
cu|.ed ||or |de |o |de, d|||ue p+|e ,e||oW |o d+||
,e||oW|eeu d|co|o|+||ou (||. !!25). Cu||c|e
+|eu|. 'ecoud+|, ou,c|o|,| corrou. |||
| . 20 u+||.
II0W NAII SN0k0M
',,. Koeueu |uro|. 1:c|. Iu|e|ou
c|e|o| (ee 'Iu|e|ou 'c|e|o|, 'ec||ou 5), oc
cu| |u 50 o| |ud|.|du+|. 0-| . |u|e||,. |! .
uu+||, ru|||p|e, r+|| |o |+|e, e|ou+|ed |o uodu|+|
|uro|, p|oduce + |ou||ud|u+| |oo.e |u u+|| p|+|e
due |o r+|||\ corp|e|ou (||. !!2o).
FkINCAI FI8k0MA
FICk 33-23 Caocer chemotherapy: 8eau |oes \u|||p|e ||+u.e|e ||d|u o| ru|||p|e ||ue|u+|| W+
+oc|+|ed W||| c|ero||e|+p, |o| ||e+| c+uce|.
FICk 33-24 Appareot |eukooycha: Ierrytype oa|s I|e p|o\|r+| |Wo||||d o| ||e u+|| p|+|e | W|||e,
W|e|e+ ||e d||+| ||||d |oW ||e |ed co|o| o| ||e u+|||ed.
FICk 33-25 e||ow oa| syodrome ||||ue ,e||oW|o|eeu co|o| o| ||e ||ue|u+||, u+|| |||c|eu|u,
|oWed |oW||, +ud e\ce|.e cu|.+|u|e ||or |de |o |de o| +|| |eu ||ue|u+||.
FICk 33-26 Iuberous sc|eross: peruoua| Ibroma A +2,e+|o|d |er+|e W||| |u|e|ou c|e|o|. A
||uco|o|ed |uro| | eeu ere||u ||or |eue+|| ||e p|o\|r+| u+|| |o|d +oc|+|ed W||| + |ou||ud|u+| |oo.e |u
||e u+|| p|+|e.
Aoc|+|ed W||| couuec||.e ||ue (co||+eu.+cu|+|)
d|e+e.
Feruoua| rythema 1:c| . ',|er|c |u
pu e|,||er+|ou ('|E), de|r+|or,o||| (|\).
n|\/A||' o| |ep+|||| C .||u |u|ec||ou. |! .
|e||uuu+| e|,||er+, eder+, +||e|+||ou o| cu||c|e,
ecoud+|, u+|| c|+ue.
Ie|aoectasa 1:c| . 'c|e|ode|r+, '|E,
|\, ||eur+|o|d +|||||||. |! . ||ue+| W||,
.ee| pe|peud|cu|+| |o u+|| |+e o.e|||e p|o\|r+|
u+|| |o|d (||. !!29), uu+||, ||||| |ed, r+, |e
||+c| || |||or|oed. '|E +ud |\. +||e W||||u
e|,||er+. 'c|e|ode|r+ +ud |\. eu|+|ed c+p|||+|,
|oop W||| -!:-! c+p|||+|, deu||, +ud +.+cu|+|
+|e+.
Cutc|e hyperkeratoss aod hemorrhaes '|E
+ud |\.
0scod I ||u|e !!!0
NAII F0I0[FkINCAI kIhMA AN0 IIANCICIASIA
PT8Y6|0N |hV8S0N 0h60|0N
Nail plate adheies to fingeitip skin in scleio-
deima.
SYSTN|0 ANYL0|00S|S
Nail dystiophy iesembling lichen planus with
seveie onychodystiophy (nail plate thinned,
longitudinally fissuied with subungual hem-
oiihages) can piecede diagnosis of piimaiy
systemic amyloidosis. Biopsy of nail appaiatus
confiims the diagnosis of amyloidosis with
amyloid deposits in the supeificial deimis of the
nail matiix (Fig. 33-31).
'u|uuu+| ep|de|r+| ||de e\|eud ||or |uuu|+
d||+||, |o |,pou,c||ur, |||||u |u + '|ouue+ud
|oo.e |+||ou |e|Weeu |r||+||, +||+ued de|r+|
||de (||. !!21). kup|u|e o| ||ue c+p|||+||e +|ou
||ee |ou||ud|u+| de|r+| ||de |eu|| |u p||u|e|
|ero|||+e. ||c| ||- |-|c- eeu W|||
r|uo| ||+ur+ (ro| corrou c+ue, occu|||u |u up
|o 20 o| uo|r+| popu|+||ou), po||+|, +|op|c de|
r+||||. |c| ||- |-|c-. |de|opeu|c
+uer|+, |+c|e||+| eudoc+|d||| (||. !!23), |||c||uo
|, +u||p|op|o||p|d +u|||od, ,ud|ore, +||||ude
|c|ue. |! . ||ue|u+||. I|u, ||ue+| ||uc|u|e,
uu+||, 2-! rr |ou, +||+ued |u ||e |ou +\|
o| u+||, p|urco|o|ed W|eu |o|red, d+||eu|u |o
||oWu o| ||+c| W||||u -2 d+,, ||e, u|equeu||,
ro.e upe|||c|+||, +ud d||+||, W||| u+|| |oW||.
SFIINIk hM0kkhACS
FICk 33-2T Irauma: subuoua| hemor-
rhae I|+ur+ |o ||e p|o\|r+| u+|| |eu||ed |u |er
o|||+e +ud + ||+u.e|e dep|e|ou +c|o ||e u+||
p|+|e. nero|||+e e\|eud |o ||e |ou||ud|u+| de|r+|
||de.
FICk 33-28 IoIectve eodocardts: sp|oter
hemorrhae 'u|uuu+| |ero|||+e |u ||e
r|dpo|||ou o| ||e ||ue|u+|| |ed |u + o0,e+|o|d
|er+|e W||| eu|e|ococc+| eudoc+|d|||, u|coujuuc||.+|
|ero|||+e W+ +|o p|eeu|.
FICk 33-29 Systemc |upus
erythematosus: oa| Io|d ery-
thema aod te|aoectass A o+
,e+|o|d |er+|e W||| ,|er|c |E W|||
+|||||||, |+||ue, +ud p|o|oeu|||.||,
|o| dec+de. ||o\|r+| u+|| |o|d +|e
eu|+|ed W||| e|,||er+, |e|+u|ec
|+|, +ud |||or|oe. I|e cu||c|e |
e|ou+|ed.
FICk 33-30 0scod |upus erythematosus: Na|
Io|d aod matrx ovo|vemeot aod oa| dystrophy A
+o,e+|o|d |er+|e W||| c||ou|c cu|+ueou |E |o| e.e|+|
dec+de. ||o\|r+| u+|| |o|d |oW e|,||er+, c+|||u, +ud
dep|reu|+||ou +oc|+|ed W||| u+|| r+|||\ |u||+rr+||ou.
FICk 33-31 Systemc amy|odoss A 52,e+|o|d r+|e W||| ,|er|c +r,|o|do|, u+|| ||ud|u p|eceded
||e d|+uo| o| ,|er|c +r,|o|do|. I|e u+|| ||ud|u |eer||e ||oe eeu |u ||c|eu p|+uu. I|e r+|||\ |
|u||+red W||| |eu||+u| |||uu|u o| ||e p|o\|r+| u+|| p|+que +ud d||u|e|+||ou o| ||e p|+|e d||+||,.
'poou|+ped u+|| (||. !!!2). |||, (ro|e
o||eu due |o |oc+| |+||e| ||+u ,|er|c |+c|o|).
p|,|o|o|c (e+||, c|||d|ood), |||u u+|| (o|d +e, pe
||p|e|+| .+cu|+| d|e+e), o|| u+|| (r+|u|, occup+
||ou+|), |e|ed||+|, +ud coueu||+|, ||urre|\|uou
,ud|ore (||oude||c|euc, +uer|+, d,p|+|+, |o
|||). |! . |u e+||, |+e, u+|| p|+|e |ecore
||+||eued, |+|e|, ede |ecore e.e||ed upW+|d +ud
u+|| +ppe+| couc+.e.
k0II0NChIA
Au|e |e|Weeu p|o\|r+| u+|| |o|d +ud u+|| p|+|e |
>30. \+, occu| W||| o| W|||ou| c,+uo|. |c||-
- . n,pe|||op|, o| o|| ||ue corpoueu| o| d|||+|
pu|p, |,pe|p|+|+ o| ||||o.+cu|+| ||ue +| |+e o| u+||
(u+|| c+u |e '|oc|ed), |oc+| c,+uo|. |||, .
C+|d|o.+cu|+| d|o|de|. Ao|||c +ueu|,r, cou
eu||+| +ud +cqu||ed c+|d|o.+cu|+| d|e+e
B|ouc|opu|rou+|, d|o|de|. |u||+||o|+c|c ueo
p|+r, c||ou|c |u||+||o|+c|c uppu|+||.e d|o|de|
C+||o|u|e||u+| d|o|de|. |u||+rr+|o|, |oWe|
d|e+e, C| ueop|+r, |ep+||c d|o|de|, ru|||p|e
po|,po|, |+c|||+|, d,eu|e|,, +roe||c d,eu|e|,
C||ou|c re||ero|o||uer|+
|! . |||| | |u||ou, u+|| p|+|e eu|+|ed +ud
e\ce|.e|, cu|.ed (||. !!!!). |uc|e+ed cu|.+|u|e
uu+||, +||ec| +|| 20 u+||.
CI880 NAIIS
FICk 33-32 ko|ooycha I|e ||u
e|u+|| p|+|e | couc+.e, uo o||e| u+|| We|e
|u.o|.ed. I|e|e We|e uo +oc|+|ed ,|er|c
|+c|o|.
FICk 33-33 Iuo caocer: c|ubbed Ioers Bu||ou eu|+|ereu| +ud ||o+deu|u o| ||e ||ue|||p |u +
ro|e| W||| |uu c+uce|. I|e ||ue |e|Weeu ||e u+|| +ud uude||,|u |oue |+ + pou, qu+|||, |.|u + '||o+||u
eu+||ou W|eu p|eu|e | +pp||ed doWuW+|d +ud |o|W+|d +| ||e juuc||ou |e|Weeu ||e p|+|e +ud p|o\|r+| |o|d.
C|+|e||e ro|e |+ |+|ued ||e |e|| r|dd|e ||ue|.
IA8I 33-2 0ru|nduced Nai| Chanes
ha|| I|od|ogs 0a0sat|ve dr0g
||co|o|+||ou (uoure|+u|u) (||. !!-!+) Au||r+|+||+|. c||o|oqu|ue, |,d|o\,c||o|oqu|ue, qu|u+c||ue
\|uoc,c||ue
Co|d, '||.e|
\e|+uou,c||+ (||. !!!5) /|do.ud|ue (A/I)
|o|+|eu
C|ero||e|+peu||c d|u. 5||uo|ou|+c|| (||. !!-!5),
d+uuo|u||c|u, do\o|u||c|u
|eu|ou,c||+. ||ue C|ero||e|+peu||c d|u
|eu|ou,c||+. +pp+|eu| C|ero||e|+peu||c d|u
|o|,p|+|r+c,. +u|||+c,c||ue, .|uc||||ue
Be+u ||ue, ou,c|or+de| (||. !!-2!) C|ero||e|+peu||c d|u
|+|ou,c||+, p+|ou,c||+| p,oeu|c |+uu|or+ (||. !!-o) ke||uo|d. |o||e||uo|u, +c|||e||u
|ud|u+.||
\e||o||e\+|e
EC|k +u|+ou||
|c|er|c c|+ue B|oc|e|
'ee I+||e !!2.
||u c+u|u +d.e|e u+|| c|+ue +|e |r||+| |o
||oe c+u|u +d.e|e c|+ue |u cu|+ueou +ud
ruco+| ||e.
C|ero||e|+p,. Be+u ||ue (||. !!2!), ou,
c|or+de|, \ue||c|e ||ue, |ero|||+|c ou,
c|o|,|, p,oeu|c |+uu|or+ (||. !!!5),
re|+uou,c||+
Au|||e||o.||+|. \e|+uou,c||+ /|do.ud|ue (A/I)|,
p,oeu|c |+uu|or+ (|ud|u+.||)
Be|+||oc|e|. ||||+| |c|er|+
B|eor,c|u. ||||+| |c|er|+
|u\A. ||o|oou,c|o|,|, re|+uou,c||+
ke||uo|d. |+|| ||+||||,, p,oeu|c |+uu|or+,
p+|ou,c||+
0kC-IN0C0 NAII ChANCS
FICk 33-34 Na| dsco|or-
atoo: quoacroe B|u|| d|
co|o|+||ou o| ||e u+|| |u + p+||eu|
W||| '|E ||e+|ed W||| qu|u+c||ue.
FICk 33-35 Na| dsco|oratoo aod traosverse baods (Muehrcke
|oes): chemotherapy |e||od ||+u.e|e |+ud ou ||e ||ue|u+|| |u +
p+||eu| W||| ||e+| c+uce| |e|u ||e+|ed W||| c|ero||e|+p, (5||uo|ou|+c||).
1028
0IS0k0kS 0F
Ih M0Ih
S E C I | 0 N 5 4
0|+| ruco+ co.e| +ud p|o|ec| ||ue |eue+||
|| +ud cou.e, euo|, |u|o|r+||ou ||or ||e
u||+ce.
|o|r+| |uuc||ou | |equ||ed |o| r+||c+||ou,
de|u||||ou, c|eroeuo|, |uuc||ou, p|ou+||ou.
'||uc|u|e o| rou||. ||p, o|+| ruco+, |u|.+e,
|ouue, p+|+|e, |ee||.
|rp+||ed o|+| ruco+| |e+||| c+ue p+|u, r+|uu
|||||ou, |u|ec||ou, corp|or|ed |rruue |uuc||ou,
+ud e\+ce||+||ou o| red|c+| d|o|de|.
0ISASS 0F Ih IIFS |C|9. 523.5

|C|0. K!.0
|u|e||||o. Aoc|+|ed W||| |uc|e+ed ro||u|e +|
corr|u|e.
|-! |c:| . ||ur|uc||u |u c|||d|eu,
+|u |+ce +ud |o o| |ee|| |u o|de| pe|ou,
c+ud|d|+| |u |rruuocorp|or|ed pe|ou,
' c- |u +|op|c de|r+|||| +ud |o||e||uo|u
||e+|reu|.
|! . e|,||er+ +ud r+ce|+||ou +| corr|
u|e (ee ||. 2529), W|||e c+ud|d+| co|ou,.
|c . K0n |o| c+ud|d|+|, cu||u|e |o|
' c- , Cc!!c .
!cc--| . |deu|||, +ud ||e+| c+ue.
ANCIAk ChIIIIIS (FkICh)
C0N0III0NS 0F Ih I0NC |C|9. 523.o, 523.1, 529

|C|0. K+
|o|r+| .+||+u| |u up |o o| popu|+||ou.
A,rp|or+||c.
|!. \u|||p|e |o|d W||| +u|e||o|po|e||o|
o||eu|+||ou ou ||e do|+| u||+ce o| ||e |ouue
(||. !+, !+2).
1:c|-! !!- . |o||+|, |oWu ,ud|ore,
+c|ore+|,, 'jo|eu ,ud|ore.
',, . ||uu+ ||u|+|+, ||uu+ p||c+|+, c|o|+|
|ouue, |oo.ed |ouue, |u||oWed |ouue.
FISSk0 I0NC
Ac||u|c/o|+| |e|+|oe, uu+||, o| ||e |oWe| ||p. ku|e
ou| qu+rou ce|| c+|c|uor+ |u ||u o| |u.+|.e || p+
pu|e o| uodu|e o| u|ce| occu|. ('ee ''o|+| Ke|+|o|,
'ec||ou 0.)
ACIINIC ChIIIIIS
SCII0N 34 ||'0k|Ek' 0| InE \0uIn 1029
|c||-- . |e|ec||.e dequ+r+||ou o| |||||o|r
p+p|||+e |eu|||u |u |+|||||e p|ojec||ou ou ||e
do|ur o| ||e |ouue.
1:c| . ne+., |o|+cco ue, rou|| ||e+||
|u, ,|er|c +u||||o||c ||e|+p,, poo| o|+| |,|eue,
eue|+| de|||||+||ou, |+d|+||ou ||e|+p,, c||ou|c ue
o| ||ru||cou|+|u|u +u|+c|d, |+c| o| d|e|+|,
|ou|+e.
',| . C+|u eu+||ou, +||e|ed |+|e,
|+|||o|, core||c d|||u|ereu|.
|! . |u||, p|+que ou do|+| |ouue
(||. !+2). C||oroeu|c |+c|e||+ o| e\oeuou
p|reu| |+|u |ouue. W|||e, ,e||oW, |eeu, ||oWu,
||+c|. C+ud|d|+| r+, occu| ecoud+|||,.
!cc--| . E||r|u+|e p|ed|po|u |+c|o|,
ood o|+| |,|eue.
',, . ||uu+ .|||o+ (u||+)
8IACk 0k WhII hAIk I0NC
FICk 34-1 Fssured tooue |eep |u||oW
ou ||e do|ur o| ||e |ouue +|e +,rp|or+||c.
FICk 34-2 hary aod Issured tooue A
51,e+|o|d |e+|||, r+|e. Iouue |+ + W|||e u||+ce
due |o |e|+|ued |e|+||u. A r|d||ue ||u|e | +|o p|eeu|.
|c||-- . Ep|e|uB+|| .||u |u|ec||ou, |oW
C|+ ce|| couu|.
|! . w|||e co||u+|ed p|+que ou |+|e|+|
+pec| o| |ouue (ee ||. !!). |oe uo| occu|
|u ucce|u||, ||e+|ed n|\/A||'.
0kAI hAIk Ik0FIAkIA ('ee 'ec||ou !)
FAkI Iv 'K|| '|C|' 0| nA|k, |A||, \uC0'A| ||'0k|Ek', A|| E\E 1030
80S|V 6|h6|V0ST0NAT|T|S
ke+c||ou p+||e|u +oc|+|ed W||| .||+| |u|ec||ou, +u|o|r
ruu||,, ||c|eu p|+uu, e|,||er+ ru||||o|re, perp||
u, c|c+|||c|+| perp||o|d.
|! . E|,||er+ +ud eder+ o| |u|.+e. 0||e|
rucocu|+ueou ||e r+, |e +||ec|ed.
0ISASS 0F Ih CINCIvA, FkI000NIIM, AN0
MC0S MM8kANS |C|9. 52!

|C|0. K0o
C.| . E|,||er+, eder+, ||uu||u o| |u|e|
deu|+| p+p|||+e W|||ou| |oue |o. ||ed|po|u
|+c|o|. poo| o|+| |,|eue, |o|+cco ue, d|+|e|e.
|-!|| . C||ou|c |u|ec||ou o| couuec||.e
||ue, pe||odou|+| ||+reu|, +ud +|.eo|+| |oue,
ro| corrou c+ue o| |oo|| |o |u +du||.
C- . Accuru|+||ou o| u||u|.+| c+|cu|u
(c+|c|||ed p|+que) +ud 1:||c:|| c:|,:
-|-:|c |u|ec||ou |eu|| |u p+|u|e o||
||ue eder+, |u|d|ou +|.eo|+| |oue |eo|p||ou,
deepeu|u pe||odou|+| poc|e|, +ud |oo|| |o.
||ed|po|u |+c|o| |o| pe||odou||||. \+|occ|u
|ou, o|+| cou||+cep||ou, d|+|e|e, |ucoco|||co|d,
n|\/A||'.
',, . |e||odou|+| d|e+e
CINCIvIIIS AN0 FkI000NIIIIS
L|0hh0|0 N000S|T|S
|! . ke||cu|+|ed W|||e p|+que +ud p+|u|u| e|o
|ou ou ruco+| u||+ce.
|||, . ||c|eu p|+uu (||), d|u (|'A||, +u|||,
pe||eu|.e +eu|), +||e||c cou|+c| de|r+||||, |+||
.e|u|o| d|e+e.
',, . |e||odou|+| d|e+e.
FICk 34-3 Mratory |ossts A|e+
o| |,pe||e|+|o| +||e|u+|e W||| +|e+ o| uo|r+|
p|u| ep|||e||ur, c|e+||u + eo|+p||c p+||e|u |u +
|er+|e W||| po||+|.
|||eu|+| +|e+ o| de|e|+||u|/ed +ud dequ+r+|ed
|||||o|r p+p|||+e (|ed |u co|o|) +|e u||ouuded |,
e|e.+|ed W||||| o| ,e||oW r+||u (||. !+!).
E||o|o,. uu|uoWu, po|||e ||u| W||| po||+| |uc|
deuce. corrou, uu+||, +,rp|or+||c
',,. Ceo|+p||c |ouue
MICkAI0k CI0SSIIIS |C|9 . 529.
|C|0 . K+.
SCII0N 34 ||'0k|Ek' 0| InE \0uIn 1031
|:!-:- . +0-o0 o| |u|.|du+| W||| || |+.e
o|op|+|,ue+| |u.o|.ereu|. E|o|.e u|ce|+||.e ||
| p+|u|u|.
|!
\|||,W|||e p+pu|e
ke||cu|+|e (ue||||e) p+||e|u o| |+c,W|||e
|,pe||e|+|o| |ucc+| ruco+ (||. !++), ||p
(ee ||. 11), |ouue, +ud |u|.+e|
n,pe|||op||c ||-|eu|op|+||+ W||| w|c||+r,
|||+e uu+||, ou ||e |ucc+| ruco+
A||op||c ||-||u, p|+que o||eu W||| w|c||+r
|||+e |u u||ouud|u ruco+
E|o|.e/u|ce|+||.e ||-upe|||c|+| e|o|ou W|||
o.e||,|u |||||u c|o| ||+| +|e eeu ou ||e |ouue
+ud |ucc+| ruco+ (||. !++)
Bu||ou ||-|u|+c| ||||e| (|up|u|e +ud |eu|| |u
e|o|.e ||)
|equ+r+||.e |u|.|||-||||| |ed |u|.+
(||. !+5).
IIChN FIANS ('ee 'ec||ou 1)
FICk 34-4 Icheo p|aous: Wckham strae |oo||, de||ued .|o|+ceou p|+que W||| |+c,, W|||e p+||e|u
ou ||e |ucc+| ruco+.
|-:|c| |c:| . |oo| o|+| |,|eue, n|\/A||',
|rruuoupp|e|ou, +|co|o| +ud |o|+cco ue,
uu|||||ou+| de||c|euc,.
|! (||. !+o) . |uuc|edou| u|ce| o| ||e
|u|e|deu|+| p+p|||+e. C|u|.+| |ero|||+e, e.e|e
p+|u, |ou| odo|/|+|||o|, |e.e|, |,rp|+deuop+||,,
+|.eo|+| |oue de||uc||ou.
|||: c-| . 3c:|-!- || |-.
|-||c |--!c 3-|c .:-| --c
'-|-c .
!cc--| . ',|er|c +u||||o||c uc| + c||u
d+r,c|u, re||ou|d+/o|e, +ro\|c||||u. |eu|+| |,
|eue.
',, . I|euc| rou||, \|uceu| d|e+e.
ACI NCk0IIIINC ICkAIIv CINCIvIIIS (ANC)
|! . n,pe|||op|, o| |o|| ||e ||ee +ud
+||+c|ed |u|.+e, p+|||cu|+||, ||e |u|e|deu|+|
p+p|||+e (||. !+1).
|||cc|, -|c--| . \o| corrou c+ue
o| |u|.+| eu|+|ereu|. C+ued |, eder+ +ud
|u|ec||.e ce||u|+| |u|||||+||ou c+ued |, p|o|oued
e\pou|e |o |+c|e||+| p|+que, ||||o| occu| ||
uu||e+|ed.
|!:-! || |,-|cc | .c-.
\+, co.e| ||e |ee|| +ud | +oc|+|ed W|||.
Au||cou.u|+u|. p|eu,|o|u, ucc|u|r|de, .+|p
|o|c +c|d
C+|c|ur c|+uue| ||oc|e|. u||ed|p|ue, .e|+
p+r||
C,c|opo||ue
',|-: :!|/!!-
||eu+uc,, pu|e||,, .||+r|u C de||c|euc,, |,co
eu |o|+e d|e+e
C||ou|c r,e|orouoc,||c |eu|er|+ (|| !+1)
',,. C|u|.+| eu|+|ereu|, |,pe|||op||c
|u|.|||
CINCIvAI hFkFIASIA
FICk 34-5 Icheo p|aous: desquamatve ovts I|e |u|.+| r+||u +|e e|,||er+|ou, eder+
|ou, +ud |e||+c|ed |u + 12,e+|o|d |er+|e. I|e |e|ou We|e p+|u|u|, r+||u deu|+| |,|eue d||||cu||, |eu|||u |u
p|+que |o|r+||ou ou ||e |ee||.
SCII0N 34 ||'0k|Ek' 0| InE \0uIn 1033
FICk 34-6 Acute oecrotto u|ceratve ovts (ANC) \e|, p+|u|u| |u|.||| W||| uec|o| ou
r+||u+| |u|.+, eder+, pu|u|euce, +ud |+|||o| |u + !5,e+|o|d |er+|e W||| +d.+uced n|\ d|e+e. A|uC
|eo|.ed W||| o|+| c||ud+r,c|u.
FICk 34-T Cova| hyperp|asa: acute mooocytc |eukema A !1,e+|o|d |er+|e W||| |eceu| d|+
uo| o| A\|. I|e |u|.+e |oW |,pe|p|+|+ due |o |u|||||+||ou W||| |eu|er|c rouoc,|e.
FI0MI0I0C
to|oy Idiopathic. Can aiise at site of minoi
mucosal injuiy, e.g., bite.
Fathoeoess Cell-mediated immune ieaction
pattein.
Ae at 0oset Any age; often duiing second dec-
ade, peisisting into adulthood, and becoming
less fiequent with advancing age.
C|assIcatoo
Simple veisus complex aphthosis based on
clinical couise.
Simple: 1-3 oial ulceis that iecui 1-3 times
pei yeai.
Complex: Continuous ulceis and associated
with systemic disease oi genital ulceis.
Majoi aphthous ulceis (AU) may peisist foi
6 weeks, healing with scaiiing.
Behet disease should be consideied in patients
with peisistent oiophaiyngeal AU, with oi with-
out anogenital AU, associated with systemic
findings (eye, neivous system). See Section 14.
Symptoms Even though small, AU can be quite
painful, which may impaii nutiition. A buining
oi tingling sensation may be felt befoie ulceia-
tion. In peisons with seveie AU, malaise: weight
loss associated with peisistent, painful AU.
Mucosa| Fodos
At times, small, painful ied macule oi papule
befoie ulceiation
Moie commonly, ulcei(s) 1 cm (Figs. 34-8
and 34-9), coveied with fibiin (giay-white),
with shaip, disciete, and at times edematous
boideis. White-giay base with an eiythema-
tous iim.
Most commonly single; at times, multiple oi
numeious small, shallow, giouped-i.e., hei-
petifoim AU (HAU). Majoi AU (MaAU )may
heal with white, depiessed scais.
kecu||eu| p+|u|u| ruco+| |e|ou.

\o| corrou c+ue o| o|+| u|ce|+||ou, |uc|deuce
up |o !0 o| o||e|W|e |e+|||, pe|ou.
\+, |e +oc|+|ed W||| ,|er|c d|e+e uc| +
n|\/A||' +ud Be|(e| d|e+e.
',,. Ap|||+e, c+u|e| o|e, +p|||ou (+u
c|eu| C|ee| Wo|d |o| 'u|ce|) |or+||||.
AFhIh0S ICkAII0N |C|9. 523.2

|C|0. K2.0
Numbei of ulceis: Minoi AU (MiAU), 1-5;
MaAU, 1-10: HAU, up to 100.
Dsr|uon : Oiophaiyngeal, anogenital, any
site in the GI tiact. Oial lesions most com-
monly on the buccal and labial mucosa, less
commonly on tongue, sulci, flooi of mouth.
MiAU iaiely occui on the palate oi gums.
MaAU often occui on soft palate and phai-
ynx. Also, esophagus, uppei and lowei GI
tiact, and anogenital epithelium.
Ceoera| Fodos With MaAU, occasionally
tendei ceivical lymphadenopathy.
Assocated 0sorders Behet disease (see Sec-
tion 14), cyclic neutiopenia, HIV/AIDS acute
HIV/AIDS syndiome, AIDS (laige chionic
AU)], ieactive aithiitis; peiiodic fevei, aphthous
stomatitis, Ciohn disease, phaiyngitis and ad-
enitis (PFAPA; occuis in young childien with
associated high fevei occuiiing peiiodically
eveiy 3-5 weeks with AU, phaiyngitis, and/oi
lymphadenitis).
Piimaiy heipetic gingivostomatitis, hand-
foot-and-mouth disease, heipangina, piimaiy
HIV/AIDS infection, Behet disease, squa-
mous cell caicinoma (SCC), bullous disease,
lichen planus, Reitei syndiome, adveise diug
ieaction.
IA80kAI0k
0ermatopatho|oy Nondiagnostic. Rule
out specific cause of ulcei, i.e., infection
(syphilitic chancie, histoplasmosis),
inflammatoiy disoideis (lichen planus), oi
canceis (SCC).
0IACN0SIS
Usually made on clinical findings, iuling out
othei causes.
SCII0N 34 ||'0k|Ek' 0| InE \0uIn 1035
C0kS
Tend to iecui duiing adulthood. Uncommonly,
may be almost constant in the oiophaiynx oi
anogenitalia, iefeiied to as tom|ex a||oss.
MANACMNI
Iotra|esooa| Iramcoo|ooe 3-10 mg/mL in
lidocaine veiy effective foi immediate ielief of
pain and iesolution of ulceis.
Systemc Iherapy
PreJnsone : In peisons with laige, peisistent,
painful AU inteifeiing with nutiition, a biief
couise of piednisone is effective (70 mg, ta-
peied by 10 oi 5 mg/d).
T|a|JomJe : Effective in HIV/AIDS, Behet
disease, laige painful AU. Adveise effects:
peiipheial sensoiy neuiopathy. Teiatog enesis.
Tumor netross [ator (TNF) n||or :
Adalimumab and Infliximab iepoited to be
effective.
FICk 34-8 Aphthous u|cers: moor \u|||p|e, .e|, p+|u|u|, |+,|+ed u|ce| W||| e|,||er+|ou |+|o
ou ||e |+||+| ruco+.
FICk 34-9 Aphthous u|cers: major A
52,e+|o|d |er+|e W||| +d.+uced n|\/A||' W||| +
5rou|| |||o|, o| p+|u|u| |e|ou ou ||e |ouue. IWo
|ue p+|u|u| deep u|ce| ou ||e |+|e|+| |ouue. u|ce|
|eo|.ed W||| |u||+|e|ou+| |||+rc|uo|oue |ujec||ou.
The diffeiential diagnosis of leukoplakia is shown in Table 34-1.
|eu|op|+||+ | + c||ou|c W|||e p|+que/|e|ou |u
||e o|op|+|,u\.
||er+||u+u| |eu|op|+||+ |+ |||o|o|c +|,p|+.
|eu|op|+||+ | dec||p||.e c||u|c+| |e|r |e+|d|u
ro|p|o|o,. c :-|| :c:c |
c! .c.- | |- |-! | .
|!. + W|||e p|+que ||+| c+uuo| |e W|ped
o|| +ud c+uuo| |e d|+uoed + +u, o||e| d|||uc|
|e|ou.
|eu|op|+||+ r+, |e p|er+||u+u| o| r+||u+u|.
|e||u|||.e d|+uo| |ou|d |e r+de ou c||u|c+|
||ud|u +ud/o| |||o|o,.
w|eu d|+uo| | de||u|||.e |||o|o|c+||,, '|eu|o
p|+||+ | uo |oue| +pp|op||+|e.
Ik0FIAkIA |C|9. 523.o

|C|0. K!.2
IA8I 34-1 0ifferentia| 0ianosis of Leukop|akia
Les|ool0|sorder 0haracter|st|cs
|eu|oeder+ C|+,||W|||e op+|eceuce o| |ucc+| ruco+, .+||+u| o|
uo|r+|. n||o|o,. +c+u||o|.
|||c||ou+| |e|+|o| Ke|+|o| ecoud+|, |o |||c||ou (e.., |+|p |oo||, |ou| o|
o.e|e\|euded deu|u|e |o|de|).
C||ou|c c|eW|u. ||p, |ouue, c|ee| |o|r o| |||c||ou+| |e|+|o|. 'u||+ce W|||e, |ou|. 0u |ucc+|
ruco+, Wede|+ped.
||ue+ +||+ 0ccu| ou |ucc+| ruco+ +| ede o| |ee|| (occ|u+| p|+ue).
0ccu| uo|r+||, o| W||| |ee||c|euc||u.
||co||ue |or+|||| C|er|c+| |||||+||ou ||or ro||u p|pe, c|+|, c|+|e||e.
0ccu| ou |+|d p+|+|e, o|||uc| r|uo| +||.+|, |+ud
ou p+|+|e, duc| |ecore |u||+red. |uc| +ppe+| |+|ed,
e|,||er+|ou do| ou po|e||o| |+|d p+|+|e +ud o||
p+|+|e. w|||e +ppe+|+uce |eo|.e W||| ce+||ou o|
ro||u. |o| cou|de|ed p|er+||u+u|.
Io|+cco c|eWe|' W|||e |e|ou |e.e|op W|e|e c|eW|u |o|+cco | |e|d. \uco+
|+uu|+| o| W||u||ed. |:c|. ruco|ucc+| |o|d. |e|ou
| p|er+||u+u|. uu+||, |eo|.e W||| d|cou||uu+||ou o|
|o|+cco.
n+||, |ouue (||. !+2) E|ou+||ou o| |||||o|r p+p|||+e o| do|+| |ouue, co|o| W|||e,
||oWu, o| ||+c|. 'ee +|o.e.
Ap|||u/c|er|c+| |u|u 0ccu| |o||oW|u p|+cereu| o| +p|||u |+||e| ou ruco+|
u||+ce. \uco+| u||+ce |ecore uec|o||c, W|||e/p+|u|u|
|e|ou |ooe|, +d|e|eu|, e+||, |ou| o||.
0|+| |+||, |eu|op|+||+ (ee ||. !!) 'ee +|o.e +ud n|\ d|e+e ('ec||ou !). w|||e co|du|o,
+ppe+|+uce ou |u|e|o|+|e|+| +pec| o| |ouue.
||er+||u+u| |eu|op|+||+ 'e.e|||, ||u|ed |o du|+||ou +ud qu+u||||, o| |o|+cco
+ud +|co|o| ue. |oc+||ou. ||p, |ouue, ||oo| o| rou||.
E|,|||o|eu|op|+||+ (pec||ed |eu|op|+||+) |+ ||e |||e|
|+|e o| r+||u+u| ||+u|o|r+||ou).
n|\. coud,|or+ +cur|u+|ur, .e||uc+ |!. W|||e p+pu|e, p|+que, r+||, e||e, p+p|||+|ed,
.u|+|| (||. !+3), qu+rou p+p|||or+ e\op|,||c. 'o|||+|,, ru|||p|e, ro+|c.
\e||ucou c+|c|uor+ 'ee |e|oW.
0||e| W|||e |e|ou Ke|+|o+c+u||or+, qu+rou +c+u||or+, u|rucou
||||o| (|e|e| uu| c|eW|u), W|||e poue ue.u
SCII0N 34 ||'0k|Ek' 0| InE \0uIn 103T
E|,||er+|ou |e|ou |eu|op|+||+ +ppe+| |ed
|ec+ue o| |u||+rr+||ou, |ero|||+e, |uc|e+ed
+u|oeue|, ep|||e||+| +||op|,, +c+u||o|,|,
u|ce|+||ou.
I|e d|||e|eu||+| d|+uo| | |oWu |u I+||e !+2.
kIhMAI0S ISI0NS AN0[0k Ik0FIAkIA
IA8I 34-2 0ifferentia| 0ianosis of Erythematous Lesion and/or Leukopkakia
Les|ool0|sorder 0haracter|st|cs
|,p|+|+, qu+rou ce|| c+|c|uor+ |u ||u ('CC|') (||. !+0) ||ud|u. W|||e (|eu|op|+||c, |ed/W|||e,
e|,|||o|eu|op|+||c), o| |ed (e|,|||op|+||c).
'up|c|ou |e|ou |+.e e|,|||op|+||c corpoueu|
W||| poo||, de||ued |o|de|, uou|oroeueou
co|o|+||ou, u|ce|+||ou.
|u.+|.e 'CC (||. !+, +ud !+2) 'ee |e|oW.
C+ud|d|+|. e|,||er+|ou 'ee 'ec||ou 25, 'C+ud|d|+|.
\||+|o|, |o||| (eo|+p||c |ouue) ||ud|u. r|\ed |ed/W|||e +|e+ ou do|+| |ouue,
dep+p|||+||ou, |+|p|, r+||u+|ed |, W||||| ||r,
r+p|||e o| 'eo|+p||c +ppe+|+uce. Cou|e.
|u|e|r|||eu||, |er|| +ud |ecu|, c|e+||u ||e
+ppe+|+uce o| r||+||ou |e|ou (||. !+!) o.e|
d+, |o Wee|. E||o|o,. |d|op+|||c, r+, |e
+oc|+|ed W||| po||+|. A|ou| +0 o| p+||eu|
+|o |+.e + ||u|ed |ouue.
k+d|o||e|+p, (/kI)|uduced ruco||| 0ue| +||e| /kI. -2 Wee|. ||ud|u. ruco+|
p+|u|u| e|,||er+, uec|o|, u|ce|+||ou. '+||.+|,
d,|uuc||ou. '|or+|od,u|+, d,p|+|+. |e|c+|ed
||p +ud o|+| ruco+, deu|+| c+||e, c+ud|d|+|,
poo||, |||||u deu|u|e. ne+||u |e|u +||e|
corp|e||ou o| /kI.
C|ero||e|+p,|uduced ruco||| '|r||+| |o ||oe o| /kI|uduced ruco|||.
0|+| ||c|eu p|+uu (||. !++) 'ee '||c|eu ||+uu, +|o.e. ||c|euo|d |o|r.
|+ce|||e p+||e|u ou |ucc+| ruco+, |u|.+.
E|o|.e, u|ce|+||.e |o|r. |ucc+|, |+||+|, |u|.+|,
|o+| ruco+. ||c|euo|d |e|ou occu| +
+d.e|e ruco+| d|u |e+c||ou, |+||.e|u|o|
d|e+e.
|upu e|,||er+|ou (||. !+1) 'ee '|upu E|,||er+|ou, 'ec||ou +.
FkMAIICNANI AN0 MAIICNANI N0FIASMS |C|0 . C+
|||, . Io|+cco|e|+|ed |+||| ro||u ro||
uu||, p+u (|e|e| uu|)|, |ur+u p+p|||or+.||u
(n|\).
|| |c:|. Io|+cco ue, +|co|o| ue, o|+| ||c|eu
p|+uu.
0:--. Corp|e\, ru||||oc+| p|oce, ru|
||c|ou+| ||e|d c+|c|uoeue|, +ud |u||+ep|||e||+|
c|ou+| p|e+d, ru||||oc+| u+|u|e o| e+||, p|oce
|educe e|||c+c, o| |oc+| ||e+|reu|.
|!. C||ou|c, o|||+|, p+|c|/p|+que ou
o|op|+|,ue+| ruco+. kedd|| .e|.e|,
+ppe+|+uce W||| e|||e| ||pp|ed o| p+|c|, |e|ou
o| |eu|op|+||+ (||. !+0). 'roo|| p+|c| W|||
r|u|r+| o| uo |eu|op|+||+.
'c-. uu+||, 2 cr. |:c|. ||oo| o| rou||
(reu), |ouue +ud |ucc+| u||+ce (Woreu).
C- . \o| d,p|+|+ do uo| p|o|e |o
|u.+|.e 'CC, ore do.
B|op, +|| |e|ou ||+| pe||| |o| ! Wee| W|||ou|
de||u|||.e d|+uo|.
0SFIASIA AN0 SAM0S CII CAkCIN0MA IN SII (SCCIS)
n|| +oc|+|ed ro|||d||, +ud ro||+|||,, +ccouu|
|u |o| +|ou| 5 o| +|| ueop|+r |u reu +ud 2
o| ||oe |u Woreu.
|!. uu+||, +ppe+| + + |+uu|+||u, .e|.e|,
p|+que o| uodu|e W||| ||pp|ed |,pe||e|+|o|
u|ce|+||ou (||. !+) (||p, ||oo| o| ||e rou||,
ceu||+| +ud |+|e|+| |de o| ||e |ouue).
B|op, +|| |e|ou ||+| pe||| |o| ! Wee| W|||ou|
de||u|||.e d|+uo|.
!cc--| . A|e|.e u||c+| |u|e|.eu||ou.
0kAI INvASIv SAM0S CII CAkCIN0MA ('ee +|o 'ec||ou )
FICk 34-10 Squamous ce|| carc-
ooma o stu: oIero|atera| tooue A
12,e+|o|d r+|e W||| +u +,rp|or+||c
|e|ou ou ||e |ouue uo||ced |, || deu
|||. A orr W|||e p|+que (|eu|op|+||+) ou
||e |ouue. B|op, |epo||ed 'CC|'. I|e
|e|ou W+ e\c|ed.
SCII0N 34 ||'0k|Ek' 0| InE \0uIn 1039
|||, . 0ucoeu|c n|\ |,pe o, 3.
|!. E\|eu|.e |,pe||e|+|o||c W|||e |eu|op|+
||+ (||. !+2).
C-. \e|+|+|/e |+|e. B|op, +|| |e|ou ||+|
pe||| |o| ! Wee| W|||ou| de||u|||.e d|+uo|.
!cc--|. A|e|.e u||c+| |u|e|.eu||ou.
0kAI vkkC0S CAkCIN0MA
FICk 34-11 Iovasve squamous
ce|| carcooma: pa|ate Au +d.+uced
|eu|op|+||c |uro| ou ||e |+|d p+|+|e o|
+ c|+|e||e ro|e|.
FICk 34-12 verrucous carcooma: bucca| mucosa E\|eu|.e |||c| p|+que +|||u ou ||e |ucc+|
ruco+.
|:!-:-. + o| p||r+|, o|+| r+||u+uc|e.
|o| ||e ro| p+||, |e|ou +|e +,rp|or+||c, o||eu
+d.+uced W|eu |||| de|ec|ed.
|!. ||eeu| + p|reu|ed |e|ou (||.
!+!), W||| .+||e+||ou o| co|o| +ud |||eu|+|
|o|de|, |+|e|, +re|+uo||c. |u ||u |e|ou +|e
r+cu|+|, ||e o| |u.+|ou +|e uu+||, |+|ed W||||u
||e |u ||u |e|ou.
||||. 30 +||e ou p|reu|ed ruco+ o|
||e p+|+|e +ud |u|.+.
|| |c:|. \o|e deep|, p|reu|ed |ud|.|du+|
(A|||c+u) |+.e |||e| p|opo|||ou+| |uc|deuce
|+|e o| ruco+| re|+uor+ ||+u W|||e (|ec+ue
o| ||e |oWe| |uc|deuce o| cu|+ueou re|+uor+).
0k0FhAkNCAI MIAN0MA ('ee +|o 'ec||ou 2)
S8MC0SAI N00IS
I|ee +||e |o||oW|u |up|u|e o| r|uo| +||.+|,
|+ud.
|! . |odu|e W||| rucu||||ed c+.||,, W||| +
|||c| |oo| (||. !++). C||ou|c |e|ou +|e |||r,
|u||+red, poo||, c||curc|||ed uodu|e, ||u||,
||+u|uceu|, ||uc|u+u|.
|:c| . |e.e|op +| ||e W|e|e r|uo| +||.+|,
|+ud +|e e+||, ||+ur+||/ed. rucou rer
||+ue o| ||e ||p +ud ||oo| o| ||e rou||.
C-. C||ou|c, |ecu||eu|, +ud ||eu || p|eeu|
+ + |||r, |u||+red uodu|e.
',,: k+uu|+
MC0CI |C|9. 521.o

|C|0 . K.o
FICk 34-13 Me|aooma: hard pa|ate A |+|e, ||||, .+||e+|ed p|reu|ed |e|ou |u + o!,e+|o|d r+|e.
|e|ou+| ||op, o| + |+|ed p+|| |oWed |u.+|.e +c|o|eu|||uou re|+uor+.
SCII0N 34 ||'0k|Ek' 0| InE \0uIn 1041
I|| | + u|ruco+| uodu|+| c+|, occu|||u +| +
||e o| |ecu||eu| ||+ur+ (||. !+5).
|! . 'e||e o| peduucu|+|ed, We||der+|
c+|ed uodu|e, uu+||, 2 cr |u d|+re|e| (r+, |e
|+|e || ue|ec|ed). |o|r+| co|o| o| ||e rucou
rer||+ue |o p|u||ed, |||r |o |+|d.
|:c|. Bucc+| ruco+ +|ou |||e ||ue, |ouue,
|u|.+, |+||+| ruco+.
',,: B||e ||||or+
IkkIIAII0N FI8k0MA |C|0. \330/ 0
FICk 34-14 Mucoce|e A We||de||ued, o|| ||u|| u|ruco+| ||uc|u+u| uodu|e ou ||e ||p. I||c| c|e+|
rucu d|+|ued W|eu ||e |e|ou W+ |uc|ed.
FICk 34-15 Irrtatoo Ibroma: |ower
|p A 53,e+|o|d |er+|e W||| + |e|ou ou ||e ||p
|o| 0 ,e+|. '|e ||equeu||, |||e || W|eu c|eW|u.
I|e|e | + |u||e|, p|u| uodu|e +| ||e |e||ec||ou o|
||e |+||+| ruco+.
4
FICk 34-16 Cutaoeous odootoeoc absces: cheek A 2!,e+|o|d |e+|||, |er+|e uo|e + |e|ou ou ||e
c|ee| |o| o rou||. . |odu|e ou ||e |oWe| |e|| c|ee| ue+| ||e j+W||ue W||| u||ouud|u e|,||er+ +ud c+||||e
dep|e|ou. 8. \o|+| W||| +d.+uced c+||e +ud uude||,|u deu|+| +|ce.
B
A pe||+p|c+| deu|+| +|ce c+u e\|eud |u|o ||e
o.e||,|u o|| ||ue, ||+c||u +ud d|+|u|u ou ||e
|+ce. (||. !+o).
CIAN0S 000NI0CNIC (0NIAI) A8SCSS
SCII0N 34 ||'0k|Ek' 0| InE \0uIn 1043
Cu|+ueou d|o|de| r+, p|eeu| |u o|+| ruco+, r+, |e cou||ued |o ||| ||e |o| rou|| |e|o|e cu|+ueou
|u.o|.ereu| occu|.
CIAN0S 0IS0k0kS INv0IvINC Ih M0Ih
0||eu p|eeu| |u o|+| ruco+, r+, |e cou||ued
|o ||| ||e |o| rou|| |e|o|e cu|+ueou |u||+e
occu|.
|!. B|||e| +|e .e|, ||+||e, |up|u|e e+||,,
|+|e|, eeu. '|+|p|, r+||u+|ed e|o|ou o| ||e
rou|| (|ucc+| ruco+, |+|d +ud o|| p+|+|e, +ud
|u|.+) +|e p|eeu||u ,rp|or. E|o|ou +|e
e\||ere|, p+|u|u|, |u|e||e||u W||| uu|||||ou.
B|op, cou|||r d|+uo| (ee '|erp||u \u|
+||, 'ec||ou o).
FMFhICS vICAkIS (Fv) ('ee +|o 'ec||ou o) |C|9 . o9+.+
|C|0 . |0.0
|+|u|u| ruco+| e|o|ou. Cu|+ueou ||||e| +ud
e|o|ou. ('ee ||. 39).
Cou|||red o| occu|| r+||u+uc,.
Ac+u||o|,|, |e|+||uoc,|e uec|o|, |u|e||+ce
de|r+||||. |C +ud corp|ereu| (C !) W||||u
||e ep|de|r+| |u|e|ce||u|+| p+ce +ud |+ereu|
rer||+ue. C||cu|+||u +u|||od|e pec|||c |o|
||+||||ed o| ||+u|||ou+| ep|||e||ur.
FAkAN0FIASIIC FMFhICS ('ee +|o 'ec||ou 3) |C|9 . o9+.+
|u cou||+| |o perp||u .u|+||, |u||ou per
p||o|d uucorrou|, +||ec| ||e o|op|+|,u\.
|!. B|||e|, W||c| |u||+||, +|e |eue, e|up|
ou ||e |ucc+| ruco+ +ud ||e p+|+|e, |up|u|e,
+ud |e+.e |+|p|, de||ued e|o|ou ||+| +|e p|+c||
c+||, |ud|||uu||+||e ||or ||oe o| |\.
noWe.e|, e|o|ou |e p+|u|u| +ud |e e\|eu
|.e ||+u |u |\, W|e|e |e|ou occu| ou|, |u ||e
o|op|+|,u\.
||+uo|, ee 'Bu||ou |erp||o|d, 'ec||ou o.
8II0S FMFhIC0I0 ('ee +|o 'ec||ou o) |C|9 . o9+.5
|C|0 . |2.0
Au|o|rruue ruco+| ||||e||u d|e+e ||+| |e+|
W||| c+|||u.
C||u|c+| r+u||e|+||ou depeudeu| ou ||e |u
.o|.ed. |e|||eu| p+|u|u| e|o|ou ou rucou
rer||+ue. |equ+r+||.e |u|.||| W||| p+|u|u|
e|o|ou ou |ouue, |ucc+|, +ud p+|+|+| ruco+.
0cu|+| ,u||ep|+|ou.
'eque|+e. dec|e+ed .||ou/|||udue, |o+|e
ue, uppe| +||W+, corp|or|e, eop|+e+| |e
uo|.
CICAIkICIAI FMFhIC0I0 ('ee 'ec||ou o) |C|9 . o9+.o
|C|0 . |2.
SSIMIC 0ISASS INv0IvINC Ih M0Ih
\uco+| |u.o|.ereu| occu| |u +pp|o\|r+|e|,
25 o| ||oe W||| c||ou|c cu|+ueou |upu e|,
||er+|ou (CC|E).
|!. |e|ou. p+|u|e e|,||er+|ou p+|c|e
|o c||ou|c p|+que, |+|p|, r+||u+|ed, |||eu|+||,
c+||oped W|||e |o|de|, |+d|+||u W|||e |||+e,
+ud |e|+u|ec|+|+. |u o|de| |e|ou. ceu||+| de
p|e|ou, p+|u|u| u|ce|+||ou.
||||. |ucc+| ruco+, p+|+|e (||. !+1),
+|.eo|+| p|oce, |ouue. C||ou|c p|+que r+,
+|o +ppe+| ou ||e .e|r|||ou |o|de| o| ||e ||p
(||. !+3).
|u +cu|e ,|er|c |upu e|,||er+|ou ('|E), u|
ce| +||e |u pu|pu||c uec|o||c |e|ou o| ||e p+|+|e
(30), |ucc+| ruco+, o| ur.
IFS kIhMAI0SS ('ee +|o 'ec||ou +) |C|9 . 10.0
|C|0 . |9!
8hT 0|SAS See above and Section 14
STVhS-J0hhS0h SYh080N l T0X|0
P|08N0h080LYS|S See Section 8
A0V8S 0806 8A0T|0hS See Section 22
ACIINIC ChIIIIIS
SCII0N 34 ||'0k|Ek' 0| InE \0uIn 1045
FICk 34-1T Iupus erythematosus: hard pa|ate E|,||er+|ou e|oded p|+que We|e +oc|+|ed W|||
c||ou|c cu|+ueou |E.
FICk 34-18 Coody|oma acumoatum: mucosa|
|p A !5,e+|o|d r+|e W||| +d.+uced n|\/A||' |+
|+d o|+| |e|ou |o| o rou||. C|u|e| o| W|||e c+u||
||oWe|||o|e||||e |e|ou ou ||e ruco+ o| ||e |oWe| ||p .
1046
S E C I | 0 N 5 5
Auoeu||+| ||u +ud ruco+ +|e u|jec| |o uu|que
d|o|de| |ec+ue o| ||e|| pec|+| +u+|or,.
|e|r+|o|o|c +ud ,|er|c d|o|de| occu| |u ||e
+uoeu||+| |e|ou.
|||r+|, ueop|+r +||e |u ||ee +|e+, ro|
corrou|, +oc|+|ed W||| c||ou|c |ur+u p+p||
|or+.||u (n|\) |u|ec||ou.
'e\u+||, ||+ur|||ed + We|| + o||e| |u|ec||ou
+|o occu| corrou|, |u ||ee ||e.
0IS0k0kS 0F
Ih CNIIAIIA,
FkINM, AN0 ANS
I|ee uo|r+| ||uc|u|e, ueW|, o|e|.ed, |.e
||e |o |e+| couce|u +|ou| e\u+| ||+ur|||ed
|u|ec||ou uc| + +uoeu||+| W+|| +ud ro||ucur
cou|+|our.
vAkIANIS 0F CNIIAI ANAI0M
|o|r+| +u+|or|c ||uc|u|e. |:!-:- . up |o
9.
',| . A,rp|or+||c, r+, +|oue ore
+u\|e|, W|eu |||| uo|ed.
C|:c| |! . '||uco|o|ed |o 2rr, d|
c|e|e, dored p+pu|e e.eu|, d|||||u|ed c||cur
|e|eu||+||, +|ouud ||e co|ou+ (||. !5), |.|u +
co|||e|oue p+||e|u.
|||--|c| !c . Coud,|or+|+ +cur|u+|ur,
ro||ucur cou|+|our
|||, . Au|o||||or+.
!cc--| . ke+u|+uce. uo|r+| +u+|or|c
||uc|u|e.
',,. Au|o||||or+
FAkI FNII FAFIS
|o|r+| e|+ceou |+ud. Au+|oou |o e|+
ceou |+ud ou ruco+ o| rou||.
|:c|. |eu|, .u|.+.
!c|-|c|. 2rr de|r+| p+pu|e, c|e+r
co|o|ed. \+, |e +||+ued |u |oW.
',,. I,ou |+ud, e|+ceou |,pe|p|+|+,
'ec|op|c e|+ceou |+ud, |o|d,ce coud|||ou
S8AC0S CIAN0 Fk0MINNC
SCII0N 35 ||'0k|Ek' 0| InE CE||IA||A, |Ek||Eu\, A|| A|u' 104T
Ec|+||c |||uW+||ed ||ood .ee| |u ||e upe|||c|+|
de|r| W||| o.e||,|u ep|de|r+| |,pe|p|+|+.
|uc|e+|u|, corrou W||| +|u.
\u|||p|e pu|p|e, roo||, 2 |o 5rr p+pu|e.
B|eed W||| ||+ur+. ('ee 'ec||ou 9, ||. 921).
|:c|. 'c|o|ur, |+u peu|, peu||e |+||.
|+||+, .u|.+
||||e|eu||+|e ||or +u|o|e|+|or+ o| |+||, d|
e+e (uu+||, p|u|e+d |/e, |ouud ou |+|||u
||uu| +|e+ +ud uppe| ||||), K+po| +|cor+.
!cc--|. ke+u|+uce. e|ec||ou|e|,
',,. Au|o|e|+|or+ o| |o|d,ce
ANCI0kkAI0MA
|c||--. uu|uoWu.
',|. ||c||u, |u|u|u, |edue, p+|u ou
.u|.+, peu|/c|o|ur, o| pe||ueur.
!c|-|c|. A|euce o| rucocu|+ueou
||ud|u.
||||e|eu||+|e ||or d,ro|p|op|o||+, dep|e|ou,
p,c|o|.
',,. |euod,u|+, c|o|od,u|+, |ed o| |u|u|u
c|o|ur ,ud|ore, .u|.od,u|+, pe||ue+| p+|u.
Chk0NIC FAIN SN0k0M
FICk 35-1 Fear|y peo|e papu|es ||u| (||uco|o|ed), |o 2rr p+pu|e +|e eeu |eu|+||, p+ced
+|ou ||e co|ou+ o| ||e |+u peu|. I|ee ||uc|u|e, W||c| +|e p+|| o| ||e uo|r+| +u+|or, o| ||e |+u, +|e
corrou|, r||+|eu |o| coud,|or+|+ o| ro||ucur cou|+|our.
|||, . I|+ur+ +oc|+|ed W||| .|o|ou e\u+|
+c||.||,.
|c||-- . |,rp|+||c |+| r+, |eu|| |u
|||or|oed |,rp|+||c .ee|. 'u|equeu| |e
c+u+||/+||ou +ud ||||o| o| W+|| o| |,rp|+||c
.ee|.
C|:c| |! . |+|u|e, |||r, +| ||re uodu|+|,
||+u|uceu| e|p||uou co|d +ppe+| uddeu|,,
uu+||, p+|+||e| |o co|ou+, uo| +||+c|ed |o o.e||,
|u ep|de|r| (||. !52).
C- . keo|.e pou|+ueou|, |u Wee| |o
rou||.
',,. |ou.eue|e+| c|e|o|u |,rp|+u|||,
peu||e .eue|e+| eder+, \oudo| p||e||||
SCIk0SINC IMFhANCIIIS 0F FNIS
Acu|e |d|op+|||c c|o|+| eder+. 0ccu| |u ,ouu
|o,. keo|.e pou|+ueou|, |u -+ d+,. |||
|e|eu||+|e ||or +cu|e c|o|ur. A|o |epo||ed |u
+du|| W||| deuue |ero|||+|c |e.e|, neuoc|
'c|ou|e|u pu|pu|+.
|,rp|o|+uu|or+ .eue|eur (ee 'ec||ou !0).
0ccu| |u c||ou|c uud|+uoed |u|ec||ou. Bo||
e\e. ke|e||ed |o + -||--. e|ep|+u||+|
due |o |,rp|+||c o|||uc||ou. C||ou|c. |e|o|r|||,
o| peu| |e|e||ed |o + '+\+p|oue peu|.
C||ou|c |ecu||eu| |+c|e||+| |u|ec||ou (||. !5!).
K+po| +|cor+.
|||+||+| o| |,rp|+||c e|ep|+u||+|. C+ued |,
p+|+|||c Wo|r uc| + |:|--c |c:||
3c c|c, 3 | . Aoc|+|ed W||| e|ep|+u
||+| o| |e.
',,. |,rp|+u|o||||o| |||or|o||c+ occ|u|.+
Chk0NIC IMFh0MA 0F Ih CNIIAIIA
A,rp|or+||c |ed |||eu|u p|+que() ou |+u
peu| (||. !5+) o| .u|.+.
||||e|eu||+|e ||or qu+rou ce|| c+|c|uor+ |u
||u.
!cc--| . C||curc||ou | cu|+||.e.
',, . /oou |+|+u|||
*
|u uuc||curc|ed r+|e
FIASMA CII 8AIANIIIS AN0 vIvIIIS
0IS0k0kS SFCIFIC I0 CNIIAI ANAI0M
SCII0N 35 ||'0k|Ek' 0| InE CE||IA||A, |Ek||Eu\, A|| A|u' 1049
FICk 35-2 Sc|eroso |ymphaots:
peos A de|r+| co|d ou ||e d||+| |+|| p+|+||e| |o
||e co|ou+.
FICk 35-3 Chrooc |ymphedema: scro-
tum A 29,e+|o|d r+|e W||| |||o|, o| |ecu||eu|
c|o|+| |u|ec||ou +ud c|o|+| We|||u. I|e c|o|ur
| |ue W||| uoucorp|e|||e |,rp|eder+ +ud ||e
peu| | |e||+c|ed. kecu||eu| |+c|e||+| |u|ec||ou |+.e
de||o,ed |,rp|+||c c|+uue|.
FICk 35-4 F|asma ce|| ba|aots A o5,e+|o|d r+|e W||| peu||e |e|ou |o| 0 ,e+|. 'o|||+|, |ed |||eu
|u p|+que |u +u uuc||curc|ed r+|e. B|op, cou|||red ||e d|+uo|.
|| . uou|e||+c|+||e |o|e||u. |||,. ||c|eu
c|e|ou, uoupec|||c |+|+uopo||||| (po||||| |
|u||+rr+||ou o| |o|e||u o| p|epuce), ||c|eu p|+
uu, c|c+|||c|+| perp||o|d, c||ou|c |,rp|eder+,
K+po| +|cor+. ||ec|ude e\+r|u+||ou o| |+u
|o| p|ec+uce|ou c|+ue (||. !55).
3c|c| -|:c |||-c . Eud |+e o| c||ou|c
p||ro|. |o|e||u ||||o||c, cou||+c|ed, ||\ed o.e|
|+u +ud c+uuo| |e |e||+c|ed o.e| |+u. \o|
o||eu eud|+e ||c|eu c|e|ou, W||c| | cor
rou|, |e|e||ed |o + B/0 (ee 'ec||ou 1, ||c|eu
c|o|ou).
|cc| . |o|e||u ||\ed |u |e||+c||ou. ||
|, . .|o|ou e\u+| +c||.||,, +cu|e cou|+c|
u|||c+||+, +cu|e +||e||c cou|+c| de|r+||||, ||c|eu
c|e|ou (||. !5o).
FhIM0SIS, FAkAFhIM0SIS, 8AIANIIIS Xk0IICA 08IIIkANS |C|9 . o05
|C|0 . |+1
FICk 35-5 Fhmoss I|e p|epuce o| |o|e||u
|+ |eeu c||ou|c+||, |u||+red W||| c+|||u +ud| uo
|oue| |e||+c|+||e o.e| ||e |+u peu|
FICk 35-6 Faraphmoss I|e p|epuce o|
|o|e||u |+ |eeu |e||+c|ed p|o\|r+||, o.e| ||e |+u
+ud c+uuo| |e |ep|+ced |o ||e uo|r+| po|||ou co.e|
|u ||e |+u. I|e |+|| | eder+|ou.
MC0CIAN0S 0IS0k0kS
0-| . Adu|||ood.
C|:c| |! . I+u, ||oWu, |u|eue ||ue||+c|,
uu+||, .+||e+|ed, 5 |o 5rr r+cu|e
'|- . |u c|u|e| ou .u|.+ (|+||+ r|uo|+, ||. !51),
peu| (|+u, |+||) (||. !53), +ud pe||+u+|
+|e+.
C- . |e||| |o| ,e+| W|||ou| c|+ue |u |/e.
|||, . |o |u|||c+u| re|+uoc,||c |,pe|p|+|+,
ue.u ce|| +|e uo| p|eeu|, p|reu|+||ou due |o
|uc|e+ed re|+u|u |u |++| ce|| |+,e|.
|||--|c| !c . \e|+uor+ |u ||u, |u\A
|eu||o, ||\ed d|u |e+c||ou, ||ue ue.u, n|\
|uduced |u||+ep|||e||+| ueop|+|+ (||).
|c . |e|rocop, |u|e ou| |u ||u
re|+uor+, |||o|o, cou|||r d|+uo|.
E\|eu|.e |e|ou ||+| c+uuo| |e e+||, |ero.ed
|ou|d |e |o||oWed p|o|o|+p||c+||,, +|e+ ||+|
|oW |u|||c+u| c|+ue |ou|d |e ||op|ed.
',, . |eu||e |eu||o, .u|.+| re|+uo|
CNIIAI (FNII[vIvAk[ANAI) INIICIN0SS
FICk 35-T Ceota| |eotooses: vu|va \u|
||p|e, .+||e+|ed d+|| ||oWu r+cu|e, |||+|e|+| ou ||e
|+||+ r|uo|+. |e|ou |+d |eeu p|eeu| |o| > 5 ,e+|
+ud +|e ru||||oc+| |u o|||u. Ac|o|eu|||uou re|+
uor+ |u ||u ru| |e |u|ed ou|.
FICk 35-8 Ceota| |eotooses: peos A
+2,e+|o|d r+|e W||| p|reu|+||ou o| peu| |o| 20
,e+|. \+||e+|ed r+cu|+| p|reu|+||ou o| ||e |+u
+ud |o|e||u. B|op, cou|||r ||e d|+uo|, |u||u ou|
re|+uor+ +ud n\||u|ec||ou ('CC|').
|:!-:- . \o| corrou uou|u|ec||ou de|r+|o
| occu|||u ou ||e |+u peu| +ud .u|.+.
0-| . \+, |e |u|||+| p|eeu|+||ou o| po||+|.
C|:c| |! . () E|,||er+|ou c+||u
p|+que ou uouocc|uded ||u (||.!50), (2)
|u|e|||||uou po||+|, We||de|r+|c+|ed e|,
||er+|ou p|+que W|||ou| c+|e |u u+|u|+||,
occ|uded ||u (||. !5).
|||| |-| (.--) c/
|eu|, .u|.+, |u|e||u|e+| c|e||, |uu|u+| |o|d.
|||--|c| !c . ||c|eu p|+uu (||), ||\ed
d|u e|up||ou, coud,|or+ +cur|u+|+, n|\|u
duced |u||+ep|||e||+| ueop|+|+, qu+rou ce||
c+|c|uor+ ('CC) |u ||u, |u.+|.e 'CC.
FS0kIASIS vICAkIS ('ee +|o 'ec||ou !)
|||, . |o o| re|+uoc,|e |eu|| |u dep|
reu|+||ou. \|||||o . C|er|c+||, |uduced |eu|o
de|r+ .
||: o| |-|- |--. |ep|reu
|+||ou +| ||e o| |uju|,. eu||+| |e|pe, c|,ou|
e|,, |r|qu|rod ||e|+p,.
wood |+rp e\+r|u+||ou . ||||e|eu||+|e dep|
reu|+||ou ||or |,pop|reu|+||ou.
C|:c| |! . '|+|p|, de|r+|c+|ed, dep|
reu|ed, W|||e r+cu|e (||. !59), e\+r|ue ||u
|o| o||e| dep|reu|ed +|e+.
|||--|c| !c . ||c|eu c|e|ou, ||e o|
eu||+| |e|pe, |+||oeu|c +||e| c|,o, e|ec||o, o|
|+e| u|e|,.
vIIIIIC0 AN0 Ik00kMA ('ee +|o 'ec||ou !)
FICk 35-9 vt|o: peos |ep|reu|+||ou o| ||e
p|o\|r+| peu||e |+||. \u|||p|e r+cu|e |+.e |ecore cou||u
eu|. I|e |e|ou We|e +u |o|+|ed ||ud|u.
FICk 35-10 Fsorass vu|ars: shaIt oI peos we||der+|c+|ed c+||u p|+que ou ||e peu||e |+|| o|
+ 25,e+|o|d r+|e. '||u||u o| ||e |u|e||u|e+| c|e|| +ud u+|| ||ud|u o| po||+| We|e +|o p|eeu|. I|e p+||eu|
p|eeu|ed |o + c||u|c |o| e\u+||, ||+ur|||ed d|e+e.
FICk 35-11 Fsorass vu|ars: otertroous A 15,e+|o|d r+|e W||| |uu|u+| |+| |o| dec+de, uu|e
pou|.e |o |op|c+| +u|||uu+| +eu|. Au e|,||er+|ou p|+que | eeu |u ||e |e|| |uu|u+| +|e+. B|op, cou|||red
po||+|, e\c|ud|u e\||+r+rr+|, |+e| d|e+e.
Corrou|, +oc|+|ed W||| || +| o||e| ||e. |oW
e.e|, r+, occu| + |u|||+| o| o|e r+u||e|+||ou.
',| . |o| p|u||||c, p+|u |u e|oded |e|ou,
+u\|e|, +|ou| e\u+||, ||+ur|||ed d|e+e ('I|).
C|:c| |! . \|o|+ceou ||+||opped p+pu|e,
d|c|e|e o| cou||ueu|. |+c, W|||e u||+ce p+||e|u
ro| corrou|, ou |+u. 0|de| |e|ou r+, |+.e
|+,|| |ue W||| re|+u|u |ucou||ueuce. Auuu|+|
|e|ou occu| ou |+u +ud |+|| (||. !52).
Bu||ou +ud/o| e|o|.e || (||. !5!) ou |+u,
.u|.+.
|||| . C|+u, peu||e |+|| (||. !52), .u|.+.
C- . 'pou|+ueou |er||ou, e|o|.e || r+,
pe||| |o| dec+de, 'CC |+|e|,.
IIChN FIANS ('ee +|o 'ec||ou 1)
FICk 35-12 Icheo p|aous, aoou|ar:
peos \|o|+ceou +uuu|+| p|+que ou ||e d||+|
|+|| +ud |+u o| + 2o,e+|o|d p+||eu|, p|eeu| |o|
> ,e+|. w|||e |+ce|||e p|+que We|e +|o p|eeu| ou
||e |ucc+| ruco+.
FICk 35-13 Icheo p|aous, erosve: peos
A !o,e+|o|d r+|e W||| p+|u|u| e|o|ou ou peu| |o|
o rou||. ne |+d p|e.|ou|, |eeu d|+uoed +ud
||e+|ed |o| |e|pe eu||+|| W|||ou| |rp|o.ereu|.
E|,||er+|ou |e|ou ou ||e |+u +ud |o|e||u W|||
e|o|ou. B|op, cou|||red ||e d|+uo|. |e|ou
|eo|.ed W||| |u||+|e|ou+| |||+rc|uo|oue |ujec||ou.
||o|+||, r|c|op+pu|+| .+||+u| o| ||c|eu p|+uu. |o 2rr p+pu|e ou |+|| o| peu| (||. !5+).
IIChN NIII0S
',| . ||u|||u, |u|u|u, p+|u W||| u|ce|+
||ou.
C|:c| |! . E+||,. e|,||er+ |,pop|
reu|+||ou. |+|e|. |,p|c+| |.o|, o| po|ce|+|uW|||e
r+cu|e +ud p|+que, W|||e due |o |o o| de|r+|
.+cu|+|u|e (||. !55). Ecc|,ro| (||. !55,
!5o, !51), |u||+e, +ud/o| e|o|ou r+, occu|
|u |u.o|.ed ||e. \+, o|||uc| u|e|||+| o||||ce.
|-c|, . Ieu ||re ro.e corrou |u |e
r+|e. C+ue o| p||ro| (||. !55) |u |o,.
|! |c- . B+|+u||| \e|o||c+ o||||e|+u (B/0).
E||+cereu| o| uo|r+| +|c|||ec|u|+| |e+|u|e. |+||+
r|uo|+ +ud c|||o|+| |ood r+, |e |e+|o||ed (||.
!5o).
C- . |u.+|.e 'CC c+u +||e |u ||| ||e o|
c||ou|c |u||+rr+||ou.
!cc--| . C|o|e|+o| o|u|reu|, rou||o| |o|
|e|o|d|uduced +||op|, p|rec|o||ru, |+c|o||ru.
',,. ||c|eu c|e|ou e| +||op||cu
IIChN SCIk0SS ('ee +|o 'ec||ou 1)
FICk 35-14 Icheo otdus: peos
||+||opped p+pu|e ou ||e |+|| o| ||e peu|.
FICk 35-15 Icheo sc|erosus: peos A 1,e+|o|d r+|e W||| p||ro| (|u+|||||, |o |e||+c| |o|e||u) |o|
o rou||. w|||e p|+que ou ||e pe||u|e|||+| |+u +ud ou ||e |e||ec||ou o| ||e |o|e||u. |e|ou |eo|.ed W|||
|op|c+| c|o|e|+o| o|u|reu|.
FICk 35-16 Icheo sc|erosus:
vu|va aod peroeum A |+|e W|||e
c|e|o||c p|+que e\|eu|.e|, |u.o|.|u ||e
+uoeu||+| |e|ou. I|e c|||o|+| +ud |+||+
r|uo|+ |e|ou | corp|e|e|, +||op||c
(+|u||u+||ou). Ecc|,roe +|e uo|ed
|u +oc|+||ou W||| +||op|,. u|ce|+||ou
c+u occu| +ud +|e p+|u|u|. 'c|e|o|
W+ |rp|o.ed W||| c|o|e|+o| o|u|reu|
||e|+p,.
4
FICk 35-1T Icheo sc|erosus: peos A o1,e+|o|d r+|e W||| |e|ou ou ||e peu| |o| 0 ,e+|. .
w||||| p|+que ou |+u W||| |,p|c+| ecc|,roe, ||e u|e|||+| o||||ce W+ cou|||c|ed. 8. ||.e ,e+| |+|e|, ||e
peu| |+d |ecore +||op||c +ud u|re|ed W||||u ||e pu||c |+|, r+||u u||u+||ou d||||cu||. A W|||e c|e|o||c
p|+que W||| ecc|,roe | eeu ou ||e ||e|c|ed ||u o| ||e .eu||+| peu||e |+||.
B
\+u||e|+||ou o| |uc+ouor+ ,ud|ore. |+|u|u| e|,||er+|ou p|+que, |||eu|u u||+ce,
e|p||uou |o|de| u||ouuded |, c+||u. ('ee
||. 3+).
MICkAI0k NCk0IIIC kIhMA ('ee +|o 'ec||ou 3)
|d|op+|||c u|ce| ou c|o|ur o| .u|.+. \+, |e
+oc|+|ed W||| o|+| +p|||ou u|ce|+||ou. \+,
occu| + + r+u||e|+||ou o| p||r+|, n|\/A||'.
0ccu| + p+|| o| ||e ,ud|ore corp|e\ o| Be|(e|
d|e+e (||. !53). ('ee +|o ||. ++ +ud
+5).
CNIIAI AFhIh0S ICkAII0NS ('ee 'ec||ou !+)
FICk 35-18 Aphthous uc|er, 8ehet dsease: scrotum A +2,e+|o|d r+|e W||| |ecu||eu| c|o|+| u|ce|
+||ou, ||e+|ed W||| +/+|||op||ue. A |+|e u|ce| W||| |+, |+e +ud e|e.+|ed r+||u | eeu ou ||e c|o|ur. I|e
u|ce| |e+|ed W||| ||+||dor|de 50 r B||.
CIMAI0S 0kMAIIIIS
0u eu||+||+ | o||eu ro|e ||o||d +ud ,rp|or+||c
||+u +| o||e| ||e.
1||-- . Iop|c+||, +pp||ed +eu| (red|c+||ou,
|u|||c+u|), |+p|eu ||o||ed ou|o eu||+| |,
|+ud (e.., po|ou |., +p).
',| . |u|eue p|u|||u, |u|u|u eu+||ou,
eder+.
C|:c| |! . E|,||er+, r|c|o.e|c|e, eder+,
e\ud+||ou o| eu||+| (||. !59). w||| p|,|ode|
r+|||| (e.., po|ou |., o| o+|), |e|ou +|e uu+||,
p|eeu| +| o||e| ||e.
|||--|c| !c . Ceu||+| |e|pe, +|op|c
de|r+||||, |||||+u| de|r+||||
AIIkCIC C0NIACI 0kMAIIIIS ('ee +|o 'ec||ou 2)
FICk 35-19 A||erc cootact dermatts:
peos '|||||u eder+ o| ||e d||+| peu||e |+|| +oc|
+|ed W||| e.e|e p|u|||u |u + 2,e+|o|d p+||eu|. ne
|+d |ouc|ed po|ou |., W||| || |+ud, ||+u|e|||u
||e |e|u |o || peu| W|||e u||u+||u, p|u|||u +ud ||eu
eder+ occu||ed W||||u 2+ | o| e\pou|e. I|e r+eu|+
co|o|ed p|reu| | C+|e||+u| p+|u|. I|e p+||eu| W+
|u|||+||, eeu |u +u u|eu| c+|e uu|| W|e|e + d|+uo| o|
ce||u|||| W+ r+de. ||u|||u | ||e d|||uu|||u |e+|u|e
o| +||e||c cou|+c| de|r+||||.
A|op|c de|r+||||. uu+||, +oc|+|ed W||| ro|e
W|dep|e+d |u.o|.ereu| |u| c+u |e |o|+|ed |o
eu||+||+.
||c|eu |rp|e\ c||ou|cu. C||ou|c |u|||u/
c|+|c||u |eu|| |u + |u|e p|+que ou c|o|ur
(||. !520) .u|.+ o| +uu (||. !52), pe|||
|u |o| ,e+| o| dec+de. |u d+|| ||u, |,po +ud
|,pe|p|reu|+||ou occu| (ee 'ec||ou 2).
||u|||u +u|. C+u occu| |u ||e +|euce o| +u, |deu
||||+||e de|r+|o|o|c d|o|de|. C||ou|c p|u|||u
+ud |u|||u o||eu p|oduce ore ||c|eu|||c+||ou
(||. !51). || |c:| . A|op|c d|+||e|, ru|
|||+c|o||+|. '-:!c, |-:| . '|c|,|:::
c- |oup A +ud B ||ep|ococc|, Cc!!c
c||:c +ud |e|pe |rp|e\ .||u. !cc-
-| . ||cou||uue corpu||.e |u|||u/c|+|c|
|u, r+|u|eu+uce o| pe||+u+| |,|eue.
AI0FIC 0kMAIIIIS, IIChN SIMFIX Chk0NICS (ISC), FkkIIS ANI
FICk 35-20 Icheo smp|ex chroocus: scro-
tum ||u||||c |||+|e|+| e|,||er+|ou |,pe|p|reu|ed
p|+que |u + +o,e+|o|d n|p+u|c r+|e. |e|ou |+d
|eeu p|eeu| |o| > 20 ,e+|. |e|ou |eo|.ed |o||oW
|u +u |ujec||ou o| |u||+|e|ou+| |||+rc|uo|oue
(! r/r|).
FICk 35-21 Icheo smp|ex chroocus: pru-
rtus ao I|e p+||eu| |+d e\pe||euced |u|eue +u+|
p|u|||u |o| r+u, ,e+|. |e||+u+| e|,||er+ W||| r||d
||c|eu |rp|e\ c||ou|cu +ud ||u|e | +oc|+|ed W|||
c||ou|c |u|||u o| ||e ||u.
|+|e ||||e| occu| ou ||e r+|e eu||+||+ cor
rou|,, e.o|.e |o p+|u|u| e|o|ou (||. !522).
w||| |epe+|ed d|u e\pou|e, ||||e|/e|o|ou
|ecu| +| ||e +re ||e.
FIX0 0kC kFII0N ('ee +|o 'ec||ou 22)
FICk 35-22 Fxed dru eruptoo: trmethoprm-su|Iamethoxato|e \|o|+ceou |u||+e ||+| |+d
|up|u|ed, occu|||u ou ||e do|ur o| ||e peu| (|+u +ud |+||), |ecu|||u +||e| ||e+|reu| W||| |||re||op||r
u||+re||o\+/o|e |u + r+|e W||| n|\/A||'.
FkMAIICNANI AN0 MAIICNANI ISI0NS
-|, . 'qu+rou ce|| c+|c|uor+ |u ||u
('CC|') | eue||c, |u||+ep|||e||+| ueop|+|+ (||) |
n|\|uduced 'CC|'.
|||, . n|\ |u|ec||ou, c||ou|c |oW|+de |+|+
uopo||||| (poo| |,|eue, |') |u o|de| |ud|.|du
+|, c||ou|c de|r+|oe (u|ce|+||.e ||c|eu p|+uu,
||c|eu c|e|ou).
C|:c| |! . 'o|||+|,, We||de||ued, |||eu|+||,
|o|de|ed, |ed p+|c| W||| + |+/ed|o.e|.e|,
u||+ce |,pe||e|+|o| ou ||e peu| (||. !03)
o| .u|.+, +oc|+|ed de|r+|oe. n|\+oc|+|ed
|e|ou +|e uu+||, ru||||oc+|, occu|||u +| +u,
||e o| ||e +uoeu||+| |e|ou (||. !52!).
|c . |e|ou+| ||op,.
C- . Appe+|+uce o| + uodu|e o| u|ce| ue|
p|o|e|ou |o |u.+|.e 'CC (||. !52+). |u n|\
+oc|+|ed 'CC|', |+|e o| ||+u|o|r+||ou |o |u.+
|.e 'CC | |e|+||.e|, |oW, |+|e | |||e| |o| .u|.+|
'CC|'. k+|e o| |u.+|.eue +ud re|+|+| |||e|
W|eu +oc|+|ed W||| poo| |,|eue/c||ou|c |+|+
uopo|||||. ('ee +|o 'ec||ou +ud !0.)
',, . E|,|||op|+|+ o| 0ue,|+|, BoWeu d|e+e,
|oWeuo|d p+pu|o|.
SAM0S CII CAkCIN0MA IN SII
FICk 35-23 hFv-oduced squamous
ce|| carcooma o stu: peraoa| A !5,e+|
o|d r+|e W||| n|\/A||' W||| +,rp|or+||c +u+|
|e|ou |o| e.e|+| ,e+|. A We||der+|c+|ed p|u|
pe||+u+| p|+que. Au+| |+p |e| |oWed |oW|+de
qu+rou |u||+ep|||e||+| |e|ou (|'||). A|| c||u|c+|
||ud|u |eo|.ed W||| 5 |r|qu|rod c|e+r +ud
e||ec||.e +u|||e||o.||+| ||e|+p,. ne |+ |er+|ued
|e|ou||ee |o| o ,e+|.
|||, . n|\ |,pe o, 3, !, !!.
|| |c:| . |rruuoupp|e|ou, occu|||u |u
n|\/A||' d|e+e, |+||oeu|c+||, |uduced |rru
uoupp|e|ou |u o||d o|+u ||+up|+u|+||ou.
C|:c| |! . E|,||er+|ou p+|c|e +ud p+
pu|e (||+||opped), (||. !03, !52!, !525),
p|reu|ed p+pu|e. 1c--| . 'o|||+|,, c|u
|e||u, cou||ueuce, p|+que() |o|r+||ou. |||
| . \uco+ +ud +uoeu||+| +ud |uu|uoc|u|+|
||u.
C- . 'pou|+ueou |eo|u||ou, pe||| |o| ,e+|,
ru|||p|e ueW |e|ou +ppe+|, p|o|e |o |u.+|.e
'CC. ||o|e|ou |o |u.+|.e 'CC |||e| |u ce|.|\,
+uu. \ou||o| ce|.|\ /+uu |, pe||od|c |+p |e||u
(c,|o|o,) |o de|ec| d,p|+||c c|+ue.
|| |. r||d d,p|+|+
|| ||. rode|+|e d,p|+|+
|| |||. ueop|+||c ce|| peue||+|e |u|o uppe| ||||d
o| ep|||e||+| |+,e|, 'CC|'
|u.+|.e 'CC. ueop|+||c ce|| peue||+|e ||or+|
|+,e| o| ep|||e||ur
hFv-IN0C0 INIkAFIIhIIAI N0FIASIA (IN) AN0 SAM0S CII
CAkCIN0MA IN SII ('ee +|o 'ec||ou !0)
stu arso o |cheo sc|erosus: vu|va A o0,e+|
o|d p+||eu| W||| |ou|+ud|u eu||+| ||c|eu c|e|ou.
E|,||er+ +ud e|o|ou W||| r+||ed +||op|, o| ||e
|+||+ r|uo|+ +ud c|||o||. |e|ou+| ||op, o| + W|||e
|,pe||e|+|o||c +|e+ |oW +oc|+|ed 'CC |u ||u +||
|u |u ||c|eu c|e|ou.
FICk 35-25 hFv-oduced ovasve squa-
mous ce|| carcooma: peroeum A !5,e+|o|d
n|\/A||'|u|ec|ed r+|e p|eeu|ed W||| + pe||ue+|
|uro| o| e.e|+| rou|| du|+||ou. n||o|o, o| ||e
e\c|ed pec|reu |oWed |u.+|.e 'CC.
|| |c:| . |+c| o| c||curc||ou, poo| peu||e
|,|eue, p||ro| (25-15), |oW oc|oecouor|c
|+|u, n|\ |u|ec||ou (5-30), u\|+d|+||ou
e\pou|e, |o|+cco ue.
|-c|, . \o|e corrou |u de.e|op|u u+
||ou (up |o 0 o| c+uce| |u reu, |+|e |u
|udu|||+||/ed u+||ou).
|-:c:- |-/!!- . |||ro|, c||ou|c
|+|+uopo|||||, peudoep|||e||or+|ou |e|+|o||c
+ud r|c+ceou |+|+u|||, ||c|eu p|+uu, ||c|eu
c|e|ou, |+u| coud,|or+, n|\|uduced ||.
',| . ||ecu|o| |e|ou, ||c||u/|u|u|u
uude| |o|e||u, u|ce|+||ou o| |+u o| p|epuce.
C|:c| |! . 'u|||e |udu|+||ou, r+|| e\c|e
ceuce, r+|| p+pu|e, W+||, |oW|| |o +u o|.|ou
e\|eu|.e c+|c|uor+ W||| |ou||u. |ec|o|
+ud/o| ecoud+|, |u|ec||ou |u p||ro||c |o|e||u.
E\|eud +|ou ||e peu||e |+|| +ud |u.o|.e co|
po|+ c+.e|uo+. k+|e|,, ||eed|u, u||u+|, |||u|+,
+ud u||u+|, |e|eu||ou occu|.
|||| . C|+u (+3), p|epuce (2), |+u
+ud p|epuce (9), p|epuce |+u +ud |+||
(+), co|ou+| u|cu (o), |+|| (2).
\e|+|+|. |uu|u+| |,rp| uode re|+|+e, d|
|+u| ||e |+|e.
INvASIv SCC 0F FNIS
|| |c:| . n|\ |u|ec||ou, +|uo|r+| ce|.|c+|
|+p |e|, |rruuoupp|e|ou, n|\/A||' d|e+e,
+d.+uced +e, |uc|e+ed uur|e| o| e\u+| p+||
ue|, ,ouue| +e +| |||| ep|ode o| |u|e|cou|e,
|o|+cco ue, ||c|eu p|+uu, ||c|eu c|e|ou (||.
!5-2+).
',| . \u|.+| p|u|||u, |oc+||/ed p+|u, d|
c|+|e, d,u||+, ||eed|u, u|ce|+||ou.
C|:c| |! . ||, |u||, W||||| o| p|reu|ed
|e|ou o| |||c|eued o| |+|d ||u, .e||uco|d, po|,
po|d, p+pu|+|. |:c| . o5 +||e ou |+||+
r+jo|+.
INvASIv SCC 0F vIvA
|||, . 0ucoeu|c n|\ |u|ec||ou. || |c:| .
C||ou|c |rruuoupp|e|ou, n|\/A||' d|e+e.
|:c| . () Cu|+ueou, (2) juuc||ou o| co|uru+|
+ud qu+rou ep|||e||ur.
|-: |- . Au+| ||. C|:c| |! . |+
pu|e, uodu|e, u|ce|+|ed uodu|e (||. !525).
INvASIv SCC 0F CIAN0S ANS
|||, . n|\ |u|ec||ou.
C|:c| |! . |+|e, c+u||||oWe||||e, W+||,
|uro| (||. !52o).
|||| . \u|.+, peu|, +uu.
C- . '|oW|oW|u, |+|e|, re|+|+|/e.
CNIIAI vkkC0S CAkCIN0MA
INvASIv AN0CNIIAI SAM0S
CII CAkCIN0MA
|uc|deuce. k+|e.
|-: |- . ||ee\|||u p|reu|ed |e|ou o|
de uo.o ||or ep|de|r+| re|+uoc,|e.
C|:c| |! . \+cu|e o| p+pu|e W||| .+||
e+||ou o| ||oWu||+c| co|o|, |||eu|+| |o|de|,
+ud o||eu W||| p+pu|+| e|e.+||ou (||. !5-2o) o|
u|ce|+||ou.
|||| . \+|e. |+u (o1), p|epuce (!),
u|e|||+| re+|u (0), peu||e |+|| (1), +ud
co|ou+| u|cu (!) (||. !52o), |er+|e. |+||+
r|uo|+, c|||o|| (||. !521).
|||--|c| !c . Ceu||+| |eu|||uo|, o|d
||\ed d|u e|up||ou, 'CC, |er+u|or+, |u||+ep|
||e||+| ueop|+|+ (BoWeuo|d p+pu|o|).
|||: |,- . Ac|+| |eu|||uou re|+uor+,
|+|e|,, derop|+||c re|+uor+.
| . |oo| |ec+ue o| e+||, re|+|+e .|+
|,rp|+||c .ee|, ro| p+||eu| d|e W||||u -!
,e+|.
MAIICNANI MIAN0MA 0F Ih AN0CNIIAI kCI0N ('ee +|o 'ec||ou 2)
FFICk 35-26 Me|aooma, ovasve: peos A .|o|+ceou uodu|e +|||u |u +u +|e+ o| r+cu|+| .+||e+|ed
|,pe|p|reu|+||ou |u + o0,e+|o|d r+|e. I|e r+cu|+| |e|ou |+d |eeu p|eeu| |o| 5 ,e+| +ud |eer||ed
eu||+| |eu|||uo|. I|e ro| corrou |||o|o|c |,pe o| eu||+| re|+uor+ | ||e +c|o|eu|||uou re|+uor+.
0||eu uud|+uoed |o| ,e+| o| dec+de, ||e+|ed
+ |u|e||||o.
we||der+|c+|ed p|+que |u uude|p+u| +|e+ (||.
!523).
XIkAMAMMAk FACI 0ISAS ('ee +|o 'ec||ou 3)
Corrou |u +d.+uced uu||e+|ed n|\/A||'.
|:c|. |eu| +ud c|o|ur.
!c|-|c|. \|o|+ceou p+pu|e, uodu|e,
p|+que, |ecore cou||ueu|. Eder+ o| peu| +ud
c|o|ur (||. !529).
kAF0SI SAkC0MA ('ee 'ec||ou 20)
B+c|e||+| |u|ec||ou, ee 'ec||ou 2+
\ucocu|+ueou +uoeu||+| |uu+| |u|ec||ou, ee
'ec||ou 25
|e|r+|op|,|o| +ud ||ue+ .e||co|o| occu| ou
|e|+||u|/|u ||u ou|,. k+|e|, occu| ou |+|| o|
peu|
C+ud|d|+| | corrou ou u+|u|+||, occ|uded
||e ou ||e peu|, .u|.+, .+|u+
'I|, ee 'ec||ou 29
AN0CNIIAI INFCII0NS
FICk 35-2T Me|aooma, ovasve: vu|va A .|o|+ceou uodu|e |u + ||+c| p|+que |u + 52,e+|o|d |er+|e.
FICk 35-28 xtramammary Faet dsease (MF): peos, scrotum, ouoa| area A 9+,e+|o|d
r+|e W||| |ecu||eu| |ed p|+que |o| e.e|+| ,e+|. E\| |+d |eeu e\c|ed |, \o| r|c|o|+p||c u|e|, |W|ce
p|e.|ou|, |u| |ecu||ed. we||der+|c+|ed ||||| |ed p|+que +|e eeu. B|op, cou|||red ||e d|+uo|. I|e |e|ou
We|e e||ec||.e|, ||e+|ed W||| e|ec||ou |e+r |+d|o||e|+p,.
FICk 35-29 kapos sarcoma: peos
A +o,e+|o|d r+|e W||| n|\/A||' |+ We||
|u o| peu| +ud |e |o| 3 rou||. \u|||p|e
uodu|e +|e eeu ou ||e |+u +ud |+|| o| ||e
peu|. \+|.e We|||u o| ||e peu| W+ c+ued
|, |uro| |u|||||+||ou +ud |,rp|+||c o|||uc||ou,
|eu|||u |u u||u+|, o|||uc||ou. '|r||+| o|||uc
||ou c+ued eder+ o| |o|| |e. I|e p+||eu| W+
uoucorp||+u| W||| +u|||e||o.||+| ||e|+p, +ud
||e+|reu| o| K+po| +|cor+
1068
S E C I | 0 N 5 6
CNkAIII0 FkkIIS
WIIh0I SkIN ISI0NS
(FkkIIS SIN MAIkIA)
|e|||eu| e.e|e p|u|||u, |||e p+|u, | + dor|u+|
|u |+c|o| |u e\||euce, ||or d+, |o d+, || |+|e
o.e| oue' |||e. |u|eue p|u|||u r+,, |u |+c|, |e
ro|e r+ddeu|u |o| ||e p+||eu| ||+u p+|u.
I|e p|,|c|+u, ||e|e|o|e, o||eu |ee| oreW|+|
|e|p|e |u ||e r+u+ereu| o| ||ee uu|o||uu+|e
p+||eu|.
||u|||u |e+d |o |eep|e u|||, + |+|e o|
pe|r+ueu| |+||ue euue ||+| p|ec|ude Wo||
+ud cou|ouud |+r||, |e|+||ou||p. \o| ||u
e|up||ou +ud |+|e +|e ro|e o| |e p|u||||c,
|u| ||e|e +|e |+|e W|e|e ||e|e | e.e|e p|u
|||u |u ||e +|euce o| ||u |e|ou, e\cep| |o|
c|+|c| r+|| (||. !o). I|| | c+||ed |
- c|-c (||or |+||u, '||c| W|||ou| p|,|c+|
u|||+|e).
I|e d|+uo||c +pp|o+c| |o ||e p+||eu| W||| eu
e|+||/ed p|u|||u W|||ou| |deu||||+||e ||u |e|ou |
+ !c | -:|
I|| p|u|||u r+, |e |u|||u|c |o ||e ||u |u| uo|
due |o + ||u d|e+e W||| pec|||c |e|ou.
|| r+, |e + ,rp|or o| + ||u d|e+e ||+| +|
||e ||re o| e\+r|u+||ou doe uo| r+u||e| W|||
pec|||c |e|ou.
|| r+, |e due |o +u |u|e|u+| o|+u d|e+e, re|+
|o||c +ud eudoc||ue coud|||ou, o| |er+|o|o|c
d|e+e.
|| r+, |e + r+u||e|+||ou o| r+||u+u| |uro|,
p,c|oeu|c |+|e, o| n|\ |u|ec||ou, o| || r+,
|e |e|+|ed |o |ujec|ed o| |ue|ed d|u.
I|e .+||ou c+ue o| p|u|||u |ue r+|e||+ +|e
|||ed |u I+||e !o, +ud +u +|o||||r o| |oW |o
+pp|o+c| + p+||eu| W||| p|u|||u |ue r+|e||+ |
|oWu |u I+||e !o2.
A c+|e|u| |||o|, +ud p|,|c+| e\+r|u+||ou +|e e
eu||+| +ud |ou|d |+|e |u|o +ccouu| ||e d|||e|eu|
|,pe o| ||c||u +ud ||e|| du|+||ou, ||e qu+|||, o|
||c||u, +ud || d|||||u||ou +ud ||r|u.
|| | uude||ood ||+| +u, p+||eu| |e|e||ed W|||
eue|+||/ed p|u|||u W|||ou| ||u |e|ou |ou|d
|e +ured |o |+.e r|u|r+| o| |+|eu| d|e+e o|
||e ||u uu||| p|o.eu o||e|W|e.
'||u |u r+, |e c||u|c+||, |u+pp+|eu|, pe||+p
cou||ued |o ou|, c||curc|||ed +|e+, +ud ||| |
p+|||cu|+||, |rpo||+u| W||| |e+|d |o ||e e\c|u|ou
o| c+||e, ped|cu|o|, o| coud|||ou uc| +
u|||c+||+ |+c||||+.
M0SI IMF0kIANI CASS
C|ront rena| Jsease : Piuiitus is one of the
most impoitant and distiessing pioblems
of chionic ienal failuie, affecting up to
50% of patients. Secondaiy skin lesions
may develop due to intense sciatching,
such as nummulai eczema, piuiigo nodu-
laiis, oi lichenified plaques.
C|o|esass : Distiessing peisistent piuiitus
accompanying biliaiy obstiuction staits
with an acial distiibution and becomes
geneialized. It may be due to both bile salts
in the skin and elevated levels of opioid
peptides.
EnJotrne Jsease : Intiactable itching occuis
in thyiotoxicosis, piobably due to incieased
blood flow, and in hypothyioidism, wheie
it is piobably due to excessive skin diyness.
In contiast to pievious beliefs, piuiitus is
not a featuie of diabetes mellitus but can
be a manifestation of diabetic neuiopathy.
Hemao|ogt Jsease: Piuiitus occuis in about
50% of patients with polycythemia veia,
often aftei contact with watei (bath
itch"), and may be associated with iaised
blood histamine levels. In Hodgkin disease
it is a piesenting symptom, and it occuis
in leukemias; in cutaneous mastocytosis
(without visible skin lesions), it usually oc-
cuis locally following iubbing the skin.
SCII0N 36 CE|EkA||/E| |kuk|Iu' w|In0uI 'K|| |E'|0|' (|kuk|Iu' '||E \AIEk|A) 1069
IA8I 36-1 Causes of Pruritus Sine Nateria
Metabo|c, eodocroe coodtoos
n,pe|||,|o|d|r
n,po||,|o|d|r
||eu+uc, |e|+|ed
Ma|oaot oeop|asms
|,rp|or+, r,e|o|d +ud |,rp|+||c |eu|er|+,
r,e|od,p|+|+
\u|||p|e r,e|or+
nod||u d|e+e
0||e| c+uce| (|+|e)
0ru oestoo
'u|c||u|c+| d|u eu|||.|||e
Ap|||u, +|co|o|, de\||+u, po|,r,\|u B, ro|p||ue,
code|ue, copo|+r|ue, ||u|ocu|+||ue, |\
|,d|o\,e||,| |+|c|
IoIestatoos
'c+||e
*
|ed|cu|o| co|po||, c+p|||, pu||
noo|Wo|r (+uc,|o|or|+|)
0uc|oce|c|+|
Ac+||+|
keoa| dsease
keu+| |+||u|e
hemato|oc dsease
|o|,c,||er|+ .e|+
|+|+p|o|e|uer|+, ||ou de||c|euc,
hepatc dsease
0|||uc||.e ||||+|, d|e+e
||eu+uc, (|u||+|ep+||c c|o|e|+|) (ee 'ec||ou 5)
Fsychoeoc states
I|+u||o|,.
|e||od o| ero||ou+| ||e
|e|||eu|.
|e|u|ou o| p+|+||o|
|,c|oeu|c p|u|||u
|eu|o||c e\co||+||ou
Auo|e\|+ ue|.o+
Iateot dermatoses aod msce||aoeous coodtoos
/e|o| (d|, ||u, 'W|u|e| ||c|)
'eu||e p|u|||u
Bu||ou perp||o|d (W|||ou| ||u |e|ou)
|e|r+|||| |e|pe|||o|r| (W|||ou| ||u |e|ou)
A|op|c de|r+|||| (W|||ou| ||u |e|ou)
|+c||||ou u|||c+||+ (de|ro|+p||r)
|||e| |+ e\pou|e
Aqu+eu|c p|u|||u
|o|+||+ p+|e||e||c+
B|+c||o|+d|+| p|u|||u
*
||+uo||c |e|ou r+, o| r+, uo| p|eeu|.
HIV n[eton: Piuiitus may occui as a pii-
maiy symptom of HIV infection and may
be piuiitus sine mateiia oi be associated
with infestations; xeiosis; oi hepatic dis-
ease, ienal disease, lymphoma, oi adveise
diug ieactions.
Sen|e rurus: This is common in peisons
aged 70 yeais and in many patients. No
causes found. Desiccation of the skin may
be one ieason, but sometimes piuiitus may
also be piovoked by watei contact mimick-
ing aquagenic piuiitus (see below).
Psyt|art Jsease : Localized piuiitus is often
a common manifestation of chionic anxi-
ety, and peisistent iubbing of the localized
aiea will iesult in lichenification. Paiasi-
tophobia is a moie seiious pioblem (see
Section 23).
quagent rurus : This piuiitus, usually in
the middle aged and eldeily, is piovoked
FICk 36-1 Frurtus wthout daoostc sko
|esoos I|| p+||eu| |+d ru|||p|e c|+|c| r+|| due
|o corpu||.e c|+|c||u |ec+ue o| e.e|e p|u|||u.
I|e|e We|e uo o||e|, +ud |u p+|||cu|+|, d|+uo||c
|e|ou. wo||up |e.e+|ed ||||+|, c||||o| W|||ou|
j+uud|ce.
by contact with watei of any tempeiatuie;
it lasts up to 1 h, and theie aie no visible
signs on the skin. Elevated levels of hista-
mine have been found in the blood and
skin of such patients, and this condition
must be distinguished fiom bath-itch" in
polycythemia veia oi watei-induced senile
piuiitus. The causes aie unknown; since
no lesions can be found, such patients aie
often labeled as neuiotic.
Noa|ga ares|eta : This is a common lo-
calized itch usually in the inteiscapulai
aiea, sometimes moie widespiead (Fig.
36-2). The sensations aie pait itch/pait
paiesthesia. It is piobably a neuiopathic
itch due to the entiapment of spinal neives
as they emeige thiough the muscle fascias
of the back.
IA8I 36-2 Approach to the 0ianosis of Cenera|ited Pruritus without 0ianostic Skin Lesions
|| | c||||c+| |o |ecou|/e ||+| uoupec|||c ||u c|+ue c+u |e |uduced |, |u|||u +ud c|+|c||u. I|e |+|e couc|u|ou
||+| + de|r+|o|o|c c+ue |o| ||c||u | uece+|||, p|eeu| ju| |ec+ue + |+| c+u |e eeu | + ||+p ||+| ru| |e
+.o|ded. I|e +pp|o+c| |o ||e p+||eu| W||| pe|||eu| eue|+||/ed p|u|||u |e|u W||| c+|e|u| |||o|, +ud re||cu|ou
e\+r|u+||ou o| ||e (eu|||e) ||u, |o||oWed |, +dd|||ou+| +||eu||ou |o ||e eue|+| |||o|,, |e.|eW o| ,|er, eue|+|
p|,|c+| e\+r|u+||ou, +ud |u.e||+||ou + ou|||ued |e|oW.
||c| l|
. |e|+||ed |||o|, o| p|u|||u.
A|e ||e|e +u, ||u |e|ou ||+| p|ecede ||e ||c||u!
| ||e ||c||u cou||uuou o| doe || occu| |u W+.e!
| ||e ||c||u |e|+|ed |o ce||+|u ||re o| ||e d+,, doe || occu| +| u|||, +ud doe || |eep ||e p+||eu| +W+|e!
| ||e ||c||u |e|+|ed |o eu.||oureu|+| coud|||ou (|e+|, co|d), | || |e|+|ed |o ero||ou+| ||e, p|,|c+| e\e|||ou,
We+||u, cou|+c| W||| W+|e|!
2. E\+r|ue c+|e|u||, |o| u|||e p||r+|, ||u d|o|de| + + c+ue o| ||e p|u|||u, \e|o| o| +|e+|o|, c+||e, ped|cu|o|
(u||!). ||c|e|e p+pu|e ou e||oW, c+|p (de|r+|||| |e|pe|||o|r|), ou c|o|ur o| |+|| o| peu| (c+||e).
!. C|ec| |o| de|ro|+p||r, |u| ||u |o| |+||e| |u (ee '\+|oc,|o| ',ud|ore, 'ec||ou 9).
+. kepe+| |||o|, |e|+|ed |o p|u|||u. 0||+|u |||o|, o| cou|||u||ou+| ,rp|or, We||| |o, |+||ue, |e.e|, r+|+|e.
n||o|, o| o|+| o| p+|eu|e|+| red|c+||ou ||+| c+u |e + c+ue o| eue|+||/ed p|u|||u W|||ou| + |+|.
5. Ceue|+| p|,|c+| e\+r|u+||ou |uc|ud|u c|| ||e |,rp| uode, |ec|+| e\+r|u+||ou +ud |oo| u+|+c |u +du|| p+||eu|.
o. || d|, ||u o| W|u|e| ||c| | + |e+ou+||e po|||e e\p|+u+||ou, |.e ||e p+||eu| |+|| o||, |o||oWed |, +u ero|||eu|
o|u|reu|. |o o+p, ||e |+|| | ||e|+peu||c, uo| |o| c|e+u|u ||e ||u, |oWe| |o c|e+u.
1. |o||oWup +ppo|u|reu| |u 2 Wee|.
'|--| l|()
|| uo |e||e| ||or ,rp|or+||c ||e+|reu| |.eu ou ||e |||| .|||, p|oceed + |o||oW.
. |e|+||ed |e.|eW o| ,|er.
2. |+|o|+|o|, |e|. corp|e|e ||ood |e| |uc|ud|u e|,|||oc,|e ed|reu|+||ou |+|e, |+||u ||ood u+|, |eu+| |uuc||ou
|e|, ||.e| |uuc||ou |e|, |ep+|||| +u||eu, ||,|o|d |e|, |oo| +ud e|o|o|c e\+r|u+||ou |o| p+|+||e.
!. || ||e d|+uo| |+ uo| |eeu e|+||||ed +| ||| po|u|, ||e p+||eu| |ou|d |e |e|e||ed |o| corp|e|e Wo||up |uc|ud|u
pe|.|c e\+r|u+||ou +ud |+p re+|.
'0ukCE. Ad+p|ed ||or l| Be|u|+|d (ed). ||:| !-:|c c! !cc--| | ||. |eW \o||, \cC|+Wn|||, 99+, pp. 2-25.
Brat|oraJa| rurus : This is a localized
piuiitus on the outei suiface of the uppei
aim, elbow, and foieaim, often associated
with clinical evidence of chionic sun dam-
age and xeiosis (hence golfei`s itch").
MANACMNI
1. Identify and tieat undeilying disease.
2. Tieat xeiosis with baths and emollients.
3. UVB and naiiow-band (311 nm) photothei-
apy oi PUVA (in ienal-, biliaiy-, aquagenic-,
and polycythemia veia-ielated piuiitus).
4. Nolaxone, naltiexone, oi odansetion; choles-
tyiamine in cholestatic itch (but ineffective
in total biliaiy obstiuction).
SCII0N 36 CE|EkA||/E| |kuk|Iu' w|In0uI 'K|| |E'|0|' (|kuk|Iu' '||E \AIEk|A) 10T1
\+u, p+||eu|, |u depe|+||ou, |ecore |e|ued
|o +ccep||u p|u|||u +u| + p+|| o| ||e|| ||.e +ud
eudu|e ||e er|+||+reu| +ud ||e |eep|e
u|||.
|| c | p|u|||u o| ||e +u+| ||u W|||ou| e.|
deuce o| + p||r+|, de|r+|o|o|c d|o|de| ore
||re eeu |u ||| |e|ou, e.., de|r+|op|,|o|,
c+ud|d|+|, po||+|, o| e|o|||e|c de|r+||||, o|
o| |ero|||o|d+| uode.
||uWo|r +|e + |+|e c+ue +ud +|e eeu uu+||,
ou|, |u c|||d|eu.
I|e r+jo| |+c|o| |u ||e p+||oeue| o| p|u|||u
+u| | |||||+||ou ||or ||e p|eeuce o| |ec+| o|||u
ou ||e +u+| ||u, ||| | ro| o||eu ||e |eu|| o|
|ucorp|e|e c|e+u|u o| ||e +|e+ +||e| de|ec+||ou
|u| +|o |eu|| |u ore pe|ou ||or ||e We+|
ue o| ||e +u+| p||uc|e|, W||c| +||oW |o| |ec+|
o|||u W|eu ||e |ec|ur | d||euded |, ||e +|||.+|
o| |ece o| W||| ||+|u.
I|e .|c|ou c,c|e | |||||+||ou ||c||u |u|
||u W||| ||e de.e|opreu| o| ||c|eu|||c+||ou
ro|e p|u|||u.
w|eu ||c|eu|||c+||ou | p|eeu|, cou||o| |e|u
W||| + .-, ||-! cou|e o| po|eu| |op|c+| |u
coco|||co|d |o |educe ||c|eu|||c+||ou. I|e r+|u
|||u| o| r+u+ereu|, |oWe.e|, ru| |e d||ec|ed
+| p|o.o||u |+c|o|.
|c,c| :|.- || c! :c|:|
| ||- cc| |:|- c! | c! | . Au\|e|,
+ud ||e +ppe+| |o cou||||u|e |o ||e ||c||u.
I|e '||| o| |u|||u o| c|+|c||u occu| ro|
o||eu +||e| de|ec+||ou +ud +| u|||, W|eu ||e
p+||eu| | o||eu +W+|eued |, ||e ||c||u. I|ee
|ou| o| p|u|||u c+u |e oreW|+| |e||e.ed |,
reu||o|c+rp|o| |o||ou.
| cc| |,-- . '|||c|, 'que+|, c|e+u|u
W||| co||ou p|ede| o+|ed |u W+|e| | |de+|.
'B+|, W|pe +.+||+||e |u +u, upe|r+||e|/
d|u|o|e W||| +|o do ||e jo|. w|eue.e|
po|||e, + |oWe| o| |u| |+|| | ||e |e|
re||od o| c|e+u|u, + ro|e cou.eu|eu|
re||od | W||| + ||de|. A||e| c|e+u|u ||e
+|e+, |||e|+| +pp||c+||ou o| |+|cur poWde| |e|p
+|o|| ||e |ec+| o|||u ||+| c+u occu| du||u
||e d+,, o|u|reu| +ud o||, |o||ou r+, +c|u+||,
+|+.+|e ||e p|u|||u.
FkkIIS ANI |C|9 . o93.0
|C|0 . |29.0
FICk 36-2 Nota|a pares-
thetca I|| coud|||ou |u ||e |u|e|
c+pu|+| |e|ou | c|+|+c|e||/ed |,
|u|eue p|u|||u W|||ou| ||u |e|ou.
I|e e|,||er+ eeu |e|e | due |o
|u|||u +ud c|+|c||u.
10T2
AFFN0ICS
With the maiked inciease in inteinational tiavel
in the past decades among peisons of all walks
of life and all ages, it is necessaiy to ask patients
with skin lesions wheie they have lived and
tiaveled. This is paiticulaily tiue foi infectious
skin disease oi infectious systemic disease with
skin manifestations. Website http://www.cdc.gvv/
truve|/Inder.cvm gives infoimation on diseases
endemic in diffeient paits of the woild and on
modes of acquisition. Links piovide updated
infoimation ielevant to diagnosis and thus ap-
piopiiate tieatment.
It is impoitant to keep in mind that a patient
with an infection acquiied in one geogiaphic
location may undeigo medical evaluation in
anothei location wheie the infection is not
endemic. Also, many infections may be iaie
oi spoiadically acquiied in iegions outside of
endemic aieas. An example is anthiax. Spoiadic
infection may be acquiied in any geogiaphic
location by way of contact with impoited con-
taminated animal pioducts.
Equally impoitant to note is that infections
that iequiie a specific vectoi foi tiansmission
have a distiibution limited by the vectoi dis-
tiibution. Howevei, piesence of the vectoi is
not sufficient foi disease to occui. Foi example,
a mosquito competent to tiansmit dengue is
found in many states in the southein United
States. Howevei, in iecent yeais, tiansmission of
dengue has been documented only iaiely within
the United States (Texas). Dengue, of couise,
is common in Asia and in othei paits of the
woild.
AFFN0IX A: "IkAvI" 0kMAI0I0C
The use of miciobial pathogens as potential oi
actual weapons of teiioiism and waifaie dates
fiom antiquity. In 2001, the anthiax attacks via
the U.S. postal system iesulted in 12 cutane-
ous and 10 inhalational cases of anthiax with
4 deaths. These caused a tiemendous amount
of anxiety, had an impact on the U.S. postal
system, and led to a functional inteiiuption
of the activities of the legislative bianch of
the U.S. goveinment. The Woiking Gioup foi
Civilian Biodefense has compiled a list of chai-
acteiistics of biologic agents that can be used as
bioweapons (Table B-1), and the U.S. Centeis
foi Disease Contiol and Pievention (CDC) has
classified potential biologic agents into thiee
categoiies: A, B, and C (Table B-2). Categoiy
A agents aie the piioiity pathogens iequiiing
special attention foi public health piepaiedness.
Many of these lead to skin signs and symptoms
and aie theiefoie of majoi concein to deima-
tologists. The potential bioteiioiism diseases
with deimatologic manifestations aie
Anthiax
Plague
Smallpox
Smallpox vaccine (vaccinia)
Tulaiemia
Viial hemoiihagic feveis
Full infoimation on plague and the viial
hemoiihagic feveis as well as infections with
anthiax by inhalation can be obtained at the
CDC website
http://www.ht.cdc.gvv/ugent/ugent|Ist.usp.
AFFN0IX 8: 0kMAI0I0CIC MANIFSIAII0NS 0F 0ISASS
INFIICI0 8 8I0I0CIC WAkFAk[8I0Ikk0kISM
Appeodces 10T3
Also, infoimation on all of these agents and
ielated links can be obtained at the following
web-sites:
www.ht.cdc.gvv/ugent/smu||pvr/dIugnvsIs/
pd]/spvr-pvster-]u||.pd]
ht t p: / / www. cdc. gvv/ nc I dvd/ dv rd/ sph/
mnpuges/dIsIn]v.htm
http: / / umu.umu-ussn.vrg/ cgI/ cvntent/
]u||/287/18/2J91
IA8I 8-1 Key |eatures of Bio|oic Aents Used as Bioweapons
. n|| ro|||d||, +ud ro||+|||, o. |o|eu||+| |o c+ue +u\|e|,
2. |o|eu||+| |o| pe|ou|ope|ou p|e+d 1. A.+||+|||||, o| p+||oeu +ud |e+||||||, o| p|oduc||ou
!. |oW |u|ec||.e doe +ud ||||, |u|ec||ou |, +e|oo| 3. Eu.||oureu|+| |+|||||,
+. |+c| o| |+p|d d|+uo||c c+p+|||||, 9. |+|+|+e o| p||o| |ee+|c| +ud de.e|opreu|
5. |+c| o| uu|.e|+||, +.+||+||e e||ec||.e .+cc|ue 0. |o|eu||+| |o |e 'We+pou|/ed
'0ukCE. ||or | Bo||o e| +|. lA\A 231.2!9, 2002, W||| pe|r||ou.
IA8I 8-2 C0C Cateory A, B, and C Aents
Category A
Au|||+\ (3c:|| c||c:)
Bo|u||r (C||! ||| |)
||+ue (-c -|)
'r+||po\ (lc|c c)
Iu|+|er|+ (|c:-||c ||c-)
\||+| |ero|||+|c |e.e|
A|eu+.||ue. |++, |eW wo||d (\+c|upo, luu|u, Cu+u+|||o, +ud '+||+)
Buu,+.|||d+e. C||re+u, Couo, k||| \+||e,
|||o.|||d+e. E|o|+, \+||u|
||+.|.|||d+e. \e||oW |e.e|, 0r| |e.e|, K,++uu| |o|e|
Category B
B|uce||o| (3:-||c pp.)
Ep||ou |o\|u o| C||! -|-
|ood +|e|, |||e+| (e.., 'c|-||c pp., |:|-:|c :| 051.n1, '|-||c
C|+ude| (3|||!-c c||-)
\e||o|do| (3 -!c||-)
||||+co| (C||c,!c ||c:)
0 |e.e| (C-||c |-||)
k|c|u |o\|u ||or |: : (c+|o| |e+u)
'|+p|,|ococc+| eu|e|o|o\|u B
I,p|u |e.e| (|:|-||c +cc-|)
\||+| eucep|+|||| +|p|+.||ue (e.., \eue/ue|+u, e+|e|u, +ud We|e|u equ|ue eucep|+||||)|
w+|e| +|e|, |||e+| (e.., l| :||-c- C,|! c.)
Category C
Ere||u |u|ec||ou d|e+e |||e+| uc| + ||p+|, |+u|+.||u, +ud 'Ak' co|ou+.||u.
'0ukCE. Ceu|e| |o| ||e+e Cou||o| +ud ||e.eu||ou +ud ||e |+||ou+| |u|||u|e o| A||e|, +ud |u|ec||ou ||e+e.
Appeodces 10T4
Chemical agents have been used as weapons on
a laige scale in Woild Wai I, in the Iiaq-Iian
Wai, by Iiaq against Kuidish civilians, and in
the Saiin attacks in Japan. Industiial hazaidous
mateiials (HAZMATs), pioduced in chemical
plants, could also be used as weapons in chemi-
cal teiioiism.
Table C-1 lists potential agents foi such at-
tacks and the symptoms they elicit. Of these,
the blisteiing agent sulfui mustaid is one of the
AFFN0IX C: ChMICAI 8I0Ikk0kISM AN0
IN0SIkIAI ACCI0NIS
IA8I C-1 Reconitin and 0ianosin hea|th Effects of Chemica| Ierrorism
Ageot Ageot hame 0o|g0e 0haracter|st|cs |o|t|a| IIects
|e|.e C,c|o|e\,| +||u (C|) \|o| (p|upo|u| pup||) \|o| (p|upo|u| pup||)
'+||u (CB) Cop|ou ec|e||ou B|u||ed/d|r .||ou
'or+u (C|) \uc|e |W||c||u/ |+c|cu|+||ou ne+d+c|e
I+|uu (CA) |+ue+, .or|||u, d|+|||e+
\/ Cop|ou ec|e||ou/We+||u
\uc|e |W||c||u/|+c|cu|+||ou
B|e+|||u d||||cu||,
'e|/u|e
Ap|,\|+u|/||ood A||ue |o|||e c|e||, |ed ||u Cou|u|ou
C,+uoeu c||o||de |o|||e c,+uo| |+ue+
n,d|oeu c,+u|de |o|||e ||o||||e* |+||eu| r+, +p |o| +||, |r||+|
|o +p|,\|+||ou |u| ro|e
+||up| oue|
'e|/u|e p||o| |o de+||
C|o||u/pu|rou+|, C||o||ue C||o||ue | + |eeu|| E,e +ud ||u |||||+||ou
d+r+e n,d|oeu c||o||de ,e||oW + W||| A||W+, |||||+||ou
||||oeu o\|de puueu| odo| |,pue+, cou|
||oeue ||oeue + re|| |||e ueW|, 'o|e |||o+|
roWu |+, o| |+ C|e| ||||ue
|o|||e ||o||||e*
B|||e||u/.e|c+u| \u|+|d/'u||u| \u|+|d (n|) |+ +u odo| 'e.e|e |||||+||ou
ru|+|d (n|, n) |||e |u|u|u +|||c o| kedue +ud ||||e| o| ||e ||u
\u|+|d + (n) |o|e|+d||
||||oeu ru|+|d Ie+||u, coujuuc||.|||, co|ue+|
(n|, n|2, n|!) d+r+e
|eW|||e (|) |eW|||e (|) |+ +u odo| \||d |ep||+|o|, d|||e |o
|||e peue||+||u e|+u|ur r+||ed +||W+, d+r+e
||oeue o\|re (C/) ||oeue o\|re (C/) |+ \+, c+ue de+||
+ peppe||| o| puueu| odo| ||, rou|| +ud ||u
|uc+p+c||+||u/ Aeu| 5/B/ \+, +ppe+| + r+ d|u |u|||+| |+c|,c+|d|+
|e|+.|o|+||e||u |u|o\|c+||ou W||| e||+||c A||e|ed couc|ouue,
|e|+.|o|, |+|ed |e+||||c de|u|ou, deu|+| o|
+ud d|||uc| |+||uc|u+||ou, |||ue, |e|||e|euce
d||o||u +ud cou|u|ou n,pe|||e|r|+
n,pe|||e|r|+ A|+\|+ (|+c| o| coo|d|u+||ou)
\,d||+| (d||+|ed pup||) n+||uc|u+||ou
\,d||+| (d||+|ed pup||)
||o||||e r+, occu| ||or ||u cou|+c| W||| ||qu|d +||ue, c,+uoeu c||o||de, o| p|oeue.
'0ukCE. '|+|e o| |eW \o||, |ep+||reu| o| ne+|||.
Appeodces 10T5
most likely agents to be used in a teiioiist attack
scenaiio, and it also induces skin lesions (see
website
hp://.b.cdc.gov/ugen/ugenlIs.usp;.
Following exposuie and an asymptomatic
latent peiiod, eiythema, piuiitus, buining, and
pain may piesent; initial blisteiing of the skin
will stait on the second day aftei exposuie and
will piogiess foi up to 2 weeks. Vesicles coalesce,
foiming laige blisteis, and wound healing is
consideiably slowei than foi a compaiable thei-
mal buin. Diffeiential diagnoses aie theimal
buin oi scalding, toxic epideimal neciolysis,
and staphylococcal scalded skin syndiome. (See
also W R Heymann: Thieats of biological and
chemical waifaie on civilian populations. J Am
Acad Deimatol 2004, 51:452.)
AFFN0IX 0: 0kC S IN FkCNANC
1
The developing fetus can potentially be affected
by any medication given to the mothei. The dis-
astious effects of thalidomide and stilbestiol on
the exposed offspiing led to the development of
the U.S. Food and Diug Administiation (FDA)
categoiies that aie now assigned befoie a diug
is ieleased (Table D-1).
Table D-2 lists safe tieatments foi deimato-
logic diseases in piegnancy, and the common
deimatologic diseases, the diugs used foi them,
and the diugs` piegnancy categoiies aie listed in
Table D-3.
IA8I 0-1 |0A Prenancy Cateories for 0rus
A |o |e|+| ||| |u cou||o||ed |ud|e.
B |o ||| |o |ur+u |e|u dep||e po|||e +u|r+| ||| o| uo ||| |u +u|r+| |ud|e |u| |ur+u |ud|e |+c||u.
C nur+u ||| c+uuo| |e |u|ed ou|. Au|r+| |ud|e r+, o| r+, uo| |oW |||.
| E.|deuce o| ||| |o |ur+u |e|u.
/ Cou||+|ud|c+|ed |u p|eu+uc,.
IA8I 0-2 Safe Ireatments for 0ermato|oic 0isorders 0urin Prenancy
0|sease Ned|cat|oo hame
Acue Iop|c+| c||ud+r,c|u, e|,|||or,c|u, |eu/o,| pe|o\|de
ko+ce+ Iop|c+| re||ou|d+/o|e, +/e|+|c +c|d
|o||+| Iop|c+| |ucoco|||co|d, c+|c|po|||o|, ||o+d |+ud u\B
|e|r+|||| Iop|c+| |ucoco|||co|d, c||o|p|eu||+r|ue o| d|p|eu|,d|+r|ue
Ceu||+| |ur+u p+p|||or+.||u |u|ec||ou ||qu|d u|||oeu, |||c||o|+ce||c +c|d
ne|pe |rp|e\ .||u |u|ec||ou Ac,c|o.||
|uu+| |u|ec||ou Iop|c+| +u|||uu+|
B+c|e||+| |u|ec||ou |eu|c||||u, cep|+|opo||u +||e| |||| |||re|e|, +/||||or,c|u
'ou|ce. '||u I|e|+p, |e||e|. '.\+dd|u ed. \o| , |o +, \+, 200o

Appeodces 10T6
IA8I 0-3 Common 0ermato|oic 0iseases, 0rus Used and Iheir Prenancy Cateories
0|sease 0r0g F0A Pregoaocy 0ategory
Acue +ud |o+ce+ Iop|c+| e|,|||or,c|u B
Iop|c+| c||ud+r,c|u B
Iop|c+| |eu/o,| pe|o\|de C
Iop|c+| ||e||uo|u C, |u| uo| +d.|ed
Iop|c+| +d+p|+|eue C, |u| uo| +d.|ed
Iop|c+| |+/+|o|eue /
Iop|c+| re||ou|d+/o|e B
Iop|c+| +/e|+|c +c|d B
',|er|c |e||+c,c||ue |
',|er|c e|,|||or,c|u B
',|er|c |o||e||uo|u /
|o||+| Iop|c+| |ucoco|||co|d C
Iop|c+| c+|c|po|||eue C
u\B p|o|o||e|+p, Cou|de|ed +|e
|u\A Cou|de|ed po|eu||+| |e|+|oeu, |u| +d.e|e
ou|core uo| |epo||ed
',|er|c re||o||e\+|e /
',|er|c +c|||e||u /
A|e|+cep| B
E|+||/ur+| B
E|+ue|cep| B
|e|r+|||| ',|er|c |ucoco|||co|d C
Iop|c+| |+c|o||ru C
Iop|c+| p|rec|o||ru C
',|er|c c||o|p|eu||+r|ue B
',|er|c d|p|eu,|d|+r|ue B
\||+| |u|ec||ou |r|qu|rod B
|odop|,|||u C, uo| |ecorreuded
|odop|,|||uo|o\|u C, uo| |ecorreuded
Ac,c|o.|| B
|+rc|c|o.|| B
\+|+c,c|o.|| B
|uu+| |u|ec||ou Iop|c+| +u|||uu+| cou|de|ed +|e
',|er|c |e|||u+||ue B
',|er|c ||ucou+/o|e C, uo| |ecorreuded
Iop|c+| ||ucou+/o|e C, cou|de|ed +|e
',|er|c |||+cou+/o|e C, +.o|d+uce ue|ed |u |||| |||re|e|
B+c|e||+| |u|ec||ou ',|er|c peu|c||||u B
',|er|c cep|+|opo||u B, po|||e +oc|+||ou |e|Weeu ce||+|u
cep|+|opo||u +ud coueu||+| r+||o|r+||ou
|u |||| |||re|e|
',|er|c +/|||or,c|u C
10TT
A
ABCDE iule foi melanoma, 309-310
Abscess, fuiuncle, and caibuncle
clinical manifestations, 605, 606[-608[
couise and piognosis, 608
diagnosis, 606
diffeiential diagnosis, 605
epidemiology and etiology, 605
laboiatoiy examination, 606
management, 608
oveiview, 605
pathogenesis, 605
Acanthosis nigiicans (AN)
classification, 88, 89[
clinical manifestations, 88-89, 424
diagnosis and diffeiential diagnosis, 89
epidemiology, 88
etiology and pathogenesis, 88
management, 89
oveiview, 88
Acne vulgaiis
clinical manifestations, 2, 3[-5[, 4-6, 7[-8[
couise, 6
epidemiology, 2
management, 6-8
oveiview, 2
pathogenesis, 2
Acquiied nevomelanocytic nevi
classification, 178-180, 179[-182[
clinical manifestations, 178
diagnosis and diffeiential diagnosis, 180-181
management, 181
oveiview, 178
Acial lentiginous melanoma
clinical manifestations, 324, 325[
epidemiology, 324
laboiatoiy examination, 324-325
management, 325
oveiview, 324
piognosis, 325
Aciochoidon, 231
Aciodeimatitis continua of Hallopeau, 66, 66[, 71
Aciolentiginous melanoma, 1012, 1013[
Actinic cheilitis, 1028
Actinic keiatosis, 275, 275[
clinical manifestations, 267-268, 267[, 269[-270[
epidemiology, 267
management, 268
oveiview, 267
pathogenesis, 267
Actinomycetoma, 739
Acute geneialized exanthematous pustulosis (AGEP),
561, 561[- 562[
Addison disease, 433, 434[
Adult T cell leukemia/lymphoma, 524-525, 525[
AIDS. See HIV/AIDS disease, human ietioviial
infections and mucocutaneous manifestations of
Albinism
oculocutaneous
diagnosis, 343-344
epidemiology, 341
management, 344
pathogenesis, 342
significance, 344
oveiview, 341, 342
Alleigic contact deimatitis, 1059, 1059[
alleigens, 26, 28
clinical manifestations, 26, 27[, 28
couise, 28
diagnosis and diffeiential diagnosis, 29, 32
epidemiology, 26
management, 32-33
oveiview, 26
pathogenesis, 26
special foims
aiiboine, 32, 33[
alleigic contact deimatitis due to plants
clinical manifestations, 30, 31[, 33[
diagnosis, 32
oveiview, 29
pathogenesis, 30
systemic, 32
Alopecia. See Haii, nail, and mucosal disoideis,
skin signs of
Alopecia aieata, 1008, 1009[
Amelanotic melanoma, 326, 326[
Amiodaione-induced pigmentation, 570, 571[
Ampicillin diug eiuption, 558[-559[
AN. See Acanthosis nigiicans (AN)
Anagen effluvium
clinical manifestations, 979[-980[, 980
couise, 980
Page num|ers [o||oweJ |y an [ " nJtae [gures anJ mages, age num|ers [o||oweJ |y " nJtae a||es.
IN0X
IN0X 10T8
Anagen effluvium (ConnueJ)
etiology, 979
management, 980
oveiview, 979
pathogenesis, 979-980
Angioedema. See Uiticaiia and angioedema
Angiokeiatoma, 206, 206[-207[, 1047
Angiosaicoma, 200, 200[
Angulai cheilitis (Peilche), 1028
Anthiax. See Cutaneous anthiax
Antitiypsin-deficiency panniculitis, 154
Anus. See Genitalia, peiineum, and anus, disoideis of
Aphthous stomatitis in HIV/AIDS, 956
Aisenical keiatoses, 276, 277[
Aithiitis obliteians, 459[-460[
Aithiopod bites and stings, cutaneous ieactions to
clinical manifestations, 853, 854[-857[,
856-858, 859[
diagnosis, 858
epidemiology, 852
management, 858-859
oveiview, 852
pathogenesis, 853
Asteatotic deimatitis, 52, 52[
Atheioscleiosis, aiteiial insufficiency, and
atheioembolization
clinical manifestations, 459-460, 459[-461[
couise and piognosis, 461-462
epidemiology, 458
management, 462
oveiview, 458
Athlete`s foot, 697
Atopic deimatitis, 1060
clinical manifestations, 35[, 36, 37[-42[
complications, 38
diagnosis, 36
epidemiology, 34
laboiatoiy examination, 36, 38
management, 38-40, 41[
oveiview, 34
pathogenesis, 34, 36
special foims, 38
Atopic eiuption of piegnancy, 422
Atiophie blanche, 468, 469[
Atypical fibioxanthomas, 299, 299[
Atypical melanocytic nevus, 300
Autoimmune disoideis. See Immune, autoimmune,
and iheumatic disoideis
Autosensitization deimatitis, 48, 49[
B
Bacillaiy angiomatosis
diagnosis, 659
histoiy, 658
management, 659
oveiview, 658
physical examination, 658, 659[
Bacteiial infections involving the skin
giam-negative infections. See Giam-negative
infections
giam-positive bacillaiy infections associated
with toxin pioduction (intoxications).
See Giam-positive bacillaiy infections associated
with toxin pioduction (intoxications)
giam-positive coccal infections associated
with toxin pioduction (intoxications).
See Giam-positive coccal infections associated
with toxin pioduction (intoxications)
infective endocaiditis
clinical manifestations, 639-640, 639, 641[
diagnosis, 640-641
epidemiology and etiology, 638-639
management, 642
oveiview, 638
pathogenesis, 639
lyme boiieliosis
clinical manifestations, 684-685, 685[-687[, 685,
687-688, 689[-690[
diagnosis, 688-689
management, 690, 691[
oveiview, 684
pathogenesis, 684
Myto|atera and mycobacteiial infections. See
Myto|atera and mycobacteiial infections
Nessera infections. See Nessera infections
oveiview, 590-591
pyodeima
abscess, fuiuncle, and caibuncle
diagnosis, 606
management, 608
oveiview, 605
pathogenesis, 605
impetigo and ecthyma
diagnosis, 600
diffeiential diagnosis, 598, 600
management, 602, 602-604
oveiview, 597
sepsis and septic shock
clinical manifestations, 643-644, 645[
diagnosis, 644
epidemiology and etiology, 642-643, 642
management, 644
oveiview, 638, 642
pathogenesis, 643
soft tissue infections (STIs). See Soft tissue
infections (STIs)
IN0X 10T9
Bacteiial infections involving the skin (ConnueJ)
Sa|y|otottus aureus infections and
intoxications, 591
Sreotottus yogenes gioup A Sreotottus
(GAS)] infections and intoxications, 591
supeificial bacteiial epideimal colonizations and
infections, 592
eiythiasma
couise, 593
diagnosis, 593
epidemiology, 592
management, 593
oveiview, 592
nonspecific inteitiigo, 596, 596[-597[
pitted keiatolysis (keiatolysis sulcata)
clinical manifestations, 594-595,
594[-595[
diagnosis, 595
epidemiology, 594
management, 595
oveiview, 594
tiichomycosis, 595
Balanitis ciicinata, 415[
Barone||a infections
in HIV/AIDS, 658
management, 654, 655
oveiview, 654
Baitonellosis, 657[, 659[
Basal cell caicinoma (BCC)
epidemiology, 287
etiology, 287
management, 289-290, 293
oveiview, 287
Basal cell nevus syndiome, 294, 295[
BCC. See Basal cell caicinoma (BCC)
Beau lines, 1021, 1022[, 1027
Beckei nevus, 219, 219[
Behet disease
epidemiology, 366
management, 368
oveiview, 366
pathogenesis, 366
Benign neoplasms and hypeiplasias
Beckei nevus, 219, 219[
deimal and subcutaneous
deimatofibioma
epidemiology and etiology, 224, 226[
management, 226
oveiview, 224
hypeitiophic scais and keloids
management, 228
oveiview, 227
infantile digital fibiomatosis, 230, 230[
lipoma, 224, 225[
skin tag, 231, 231[
disoideis of melanocytes
acquiied nevomelanocytic nevi
management, 181
oveiview, 178
blue nevus
epidemiology, 184
management, 185
oveiview, 184
pathogenesis, 184
physical examination, 185, 185[-186[
halo nevomelanocytic nevus
epidemiology, 183
management, 184
oveiview, 183
pathogenesis, 183
Mongolian spot, 189, 189[
nevus of Ota, 190, 190[-191[
nevus spilus, 186, 187[
Spitz nevus, 188, 188[
epideimal nevus, 222, 223[
miscellaneous cysts and pseudocysts
digital myxoid cyst, 214, 214[
epideimal inclusion cyst, 212, 212[
epideimoid cyst, 211, 211[
milium, 213, 213[
tiichilemmal cyst, 212, 212[
nevus sebaceous, 222, 223[
sebaceous hypeiplasia, 222, 223[
seboiiheic keiatosis
epidemiology, 215
management, 216
oveiview, 215
syiingoma, 220, 221[
tiichoepithelioma, 220, 220[-221[
vasculai malfoimations
capillaiy malfoimations
angiokeiatoma, 206, 206[-207[
cheiiy angioma, 205, 205[
poit-wine stain
histopathology, 201
management, 202
oveiview, 201, 201[-202[
syndiomic, 202
IN0X 1080
Benign neoplasms and hypeiplasias (ConnueJ)
spidei angioma, 202, 203[
venous lake, 204, 204[
capillaiy/venous malfoimations, 209-210, 209[
blue iubbei bleb nevus, 210, 210[
Klippel-Tinaunay syndiome, 209, 210[
Maifucci syndiome, 210
Paikes-Webei syndiome, 210
vasculai hamaitomas, 209
lymphatic malfoimation
lymphangioma, 208, 208[
oveiview, 192, 192-193, 201
vasculai tumois
angiosaicoma, 200, 200[
glomus tumoi, 199, 199[
hemangioma of infancy
couise and piognosis, 194, 195[-197[
diagnosis, 194
epidemiology, 193
management, 194
special piesentations, 193-194
oveiview, 192, 192-193
pyogenic gianuloma, 198, 198[
Beiloque deimatitis, 242, 243[
Bethesda System foi classification of anogenital
dysplasia, 908, 908
Biologic waifaie/bioteiioiism, deimatologic
manifestations of diseases inflicted by, 1072-1073,
1073
Bioteiioiism, chemical, 1074-1075, 1074
Black measles, 761
Blastomycosis: cutaneous manifestations
diagnosis, 754
management, 754
oveiview, 753
Blood poisoning, 642
Blue nevus
epidemiology, 184
management, 185
oveiview, 184
pathogenesis, 184
Blue iubbei bleb nevus, 210, 210[
Boil, 605
Bone maiiow tiansplantation. See Oigan and bone
maiiow tiansplantation, skin diseases in
Botiyomycosis, 957[
Bowen disease, 279[
Biazilian pemphigus, 108
Bieakbone fevei, 810
Bullous diseases
bullous pemphigoid
epidemiology, 112
management, 112-113
oveiview, 112
pathogenesis, 112
cicatiicial pemphigoid, 114, 114[
deimatitis heipetifoimis
couise, 118
epidemiology, 116
management, 118
oveiview, 116
epideimolysis bullosa acquisita (EBA), 120, 121[
familial benign pemphigus, 105, 105[
heieditaiy epideimolysis bullosa
classification, 98, 100
clinical phenotypes, 98, 99[, 100, 101[, 102,
103[-104[
diagnosis, 102
etiology and pathogenesis, 98, 99[
management, 102
oveiview, 98
lineai IgA deimatosis (LAD), 119, 119[
pemphigoid gestationis, 115, 115[-116[
pemphigus
clinical manifestations, 106, 107[, 108, 109[
couise, 108, 110
epidemiology, 106
management, 110
oveiview, 106
vaiiants, 108
Bullous pemphigoid, 1043
Buigei sign, 459
Buiuli ulcei, 680, 681[
C
CACL (cutaneous anaplastic laige cell lymphoma),
535, 536[
Calciphylaxis
epidemiology, 480
management, 482
oveiview, 480
pathogenesis, 480
Campbell de Moigan spots, 205
Canceis, systemic, skin signs of
classification
heiitable disoideis, 486
metastatic canceis, 486
paianeoplastic syndiomes, 486
Cowden syndiome (multiple hamaitoma
syndiome), 498, 499[
glucagonoma syndiome, 500, 500[-501[
malignant acanthosis nigiicans, 502, 502[-503[
metastatic cancei to the skin
clinical manifestations, 487-488, 488[-491[,
492, 493[
IN0X 1081
Canceis, systemic, skin signs of (ConnueJ)
metastatic cancei to the skin (ConnueJ)
epidemiology, 487, 487
management, 492
oveiview, 487
pathogenesis, 487
mucocutaneous signs of systemic canceis, 486
Paget disease
extiamammaiy Paget disease, 496, 496[
mammaiy Paget disease, 494, 495[
paianeoplastic pemphigus, 503, 503[
Peutz-Jegheis syndiome, 498, 499[
Candida onychia
clinical findings, 1015-1016, 1016[
etiology and epidemiology, 1015
management, 1016
oveiview, 1015
Candidiasis
classification, 719
laboiatoiy examination, 718-719, 720[
management, 719
oveiview, 718
Caibuncle. See Abscess, fuiuncle, and caibuncle
Cat-sciatch disease
diagnosis, 656-657
management, 657
oveiview, 655
pathogenesis, 656
Cellulai blue nevus, 185, 186[
Cellulitis. See Eiysipelas and cellulitis
Ceicaiial deimatitis, 880, 881[
Chagas disease, 893
Chemical bioteiioiism and industiial accidents,
1074-1075, 1074
Chemical-induced photosensitivity. See Diug- and
chemical-induced photosensitivity
Cheiiy angioma, 205, 205[
Chickenpox, 833
C||amyJa rat|omas infections
invasive infection: lymphogianuloma veneieum
diagnosis, 941
epidemiology, 939
management, 941
oveiview, 939
pathogenesis, 939
localized infection
management, 939
oveiview, 937
oveiview, 936
pathogenesis, 937
syndiomes caused by, 936
Chloasma, 344
Chloiacne, 5
Chloioma, 513[
Cholestatis of piegnancy, 420
Chondiodeimatitis nodulaiis helicis, 266, 266[
Chiomomycosis
diagnosis, 743
management, 743
oveiview, 742
Cicatiicial pemphigoid, 114, 114[, 1044
Clubbed nails, 1026, 1026[
Collodion baby, 83, 83[
Comedones, 2, 3[
Condylomata acuminata, 900, 902[-903[, 905[, 907[
Confetti" macules, 450, 451[
Congenital nevomelanocytic nevus
epidemiology, 304
management, 306
oveiview, 304
pathogenesis, 304-305, 305[-307[
Contact deimatitis, 20
Cowden syndiome (multiple hamaitoma syndiome),
498, 499[
Cowpox, 782-783
Cianial aiteiitis, 405[
Cieeping eiuption, 876
CREST syndiome, 393[
Ciyoglobulinemia
clinical manifestations, 509, 509[-510[, 511
oveiview, 509
Cushing syndiome and hypeicoiticism, 430, 430[
Cutaneous anaplastic laige cell lymphoma (CALCL),
535, 536[
Cutaneous anthiax
diagnosis, 635
management, 636
oveiview, 634
Cutaneous B cell lymphoma, 537, 537[
Cutaneous candidiasis
diagnosis, 722
management, 722
oveiview, 720
Cutaneous diphtheiia, 637
Cutaneous hoin, 275, 276[
Cutaneous laiva migians
couise, 878
diagnosis, 878
management, 879
IN0X 1082
Cutaneous laiva migians (ConnueJ)
oveiview, 876
pathogenesis, 877
Cutaneous lymphomas and saicoma
adult T cell leukemia/lymphoma, 524-525, 525[
cutaneous anaplastic laige cell lymphoma
(CALCL), 535, 536[
cutaneous B cell lymphoma, 537, 537[
cutaneous T cell lymphoma, 526
Kaposi saicoma
classification and clinical vaiiants, 538
couise and piognosis, 540, 542
etiopathogenesis, 538
management, 542
oveiview, 538
pathogenesis, 538
physical examination, 538, 539[, 540, 541[, 543[
lymphomatoid papulosis, 535, 536[
mycosis fungoides
diagnosis and diffeiential diagnosis, 532, 532-533
management, 534
oveiview, 526
vaiiants, 530, 531[, 533[
oveiview, 524, 525
Szaiy syndiome, 534
Cutaneous melanoma
classification, 308
clinical piesentations of melanoma, 310, 310
data and facts, 310
etiology and pathogenesis, 309, 309
impoitance of, 308-309, 309
melanoma giowth patteins, 309-310
melanoma iecognition, 310
oveiview, 308
Cutaneous peifoiating disoideis, 424
Cutaneous T cell lymphoma, 526
Cutaneous tubeiculosis
classification, 671-672
diagnosis, 676
laboiatoiy examination, 676, 676[
management, 676
oveiview, 671
pathogenesis, 672
D
Daiiei disease (Daiiei-White disease, keiatosis
folliculaiis)
clinical manifestations, 90-91, 90[-91[, 1010, 1010[
disease association, 91
management, 91
oveiview, 90
Daik nevus, 300
Dailing disease, 749
DED. See Dyshidiotic eczematous deimatitis (DED)
Delusions of paiasitosis, 582, 583[
Dengue fevei, dengue hemoiihagic fevei, dengue
shock syndiome
diagnosis, 812
management, 812
oveiview, 810
Deimal melanocytoma, 184
Deimatitis. See Eczema/deimatitis
Deimatofibioma
epidemiology and etiology, 224, 226[
management, 226
oveiview, 224
Deimatofibiosaicoma piotubeians, 298, 298[
Deimatoheliosis (photoaging")
epidemiology, 262
management, 264
oveiview, 262
pathogenesis, 262
Deimatology and inteinal medicine
adveise cutaneous diug ieactions. See Diug
ieactions
bacteiial infections involving the skin. See Bacteiial
infections involving the skin
cutaneous lymphomas and saicoma. See Cutaneous
lymphomas and saicoma
endociine diseases. See Endociine diseases
genetic diseases. See Genetic diseases
hematologic disease, skin signs of. See Hematologic
disease, skin signs of
immune, autoimmune, and iheumatic disoideis.
See Immune, autoimmune, and iheumatic
disoideis
metabolic and nutiitional conditions. See
Metabolic and nutiitional conditions
oigan and bone maiiow tiansplantation, skin
diseases in. See Oigan and bone maiiow
tiansplantation, skin diseases in
psychiatiic disoideis. See Psychiatiic disoideis
ienal insufficiency, skin signs of. See Renal
insufficiency, skin signs of
systemic canceis, skin signs of. See Canceis,
systemic, skin signs of
vasculai insufficiency, skin signs of. See Vasculai
insufficiency, skin signs of
Deimatomyositis
epidemiology and etiology, 370, 371
laboiatoiy examination, 370-371, 373
management, 373
oveiview, 370
IN0X 1083
Deimatophytoses
classification, 694
deimatophytoses of epideimis, 697
deimatophytoses of haii (tiichomycosis), 708, 709[
deimatophytic folliculitis. See Folliculitis, infectious
Majocchi gianuloma, 717, 717[
tinea baibae
management, 716
tinea capitis
classification, 710[
couise, 715
management, 714
oveiview, 709
pathogenesis, 710
in HIV/AIDS, 956
management, 696
oveiview, 693
pathogenesis, 693[, 694-695
tinea coipoiis
management, 705
oveiview, 704
tinea ciuiis
management, 704
oveiview, 703
tinea facialis
clinical manifestations, 707, 707[-708[, 716[
management, 707
oveiview, 707
tinea incognito, 704[-706[, 708
tinea manuum
couise, 703
management, 703
oveiview, 701
tinea pedis
diagnosis, 700
diffeiential diagnosis, 699-700
epidemiology, 697
oveiview, 697
Desmoplastic melanoma, 323, 323[
Diabetes mellitus
associated skin diseases, 424-425
diabetic bullae, 425, 425[
diabetic deimopathy, 427, 427[
diabetic foot," 426
diabetic neuiopathy, 426, 426[
neciobiosis lipoidica
management, 429
oveiview, 428
pathogenesis, 428
DIC. See Disseminated intiavasculai coagulation (DIC)
Digital myxoid cyst, 214, 214[
Discoid eczema, 46
Disseminated coccidioidomycosis
clinical manifestation, 756-757, 757[
diagnosis, 757
management, 757
oveiview, 755
pathogenesis, 756
Disseminated ciyptococcosis
diagnosis, 748
management, 748
oveiview, 747
pathogenesis, 747
Disseminated fungal infection in HIV/AIDS, 958
Disseminated intiavasculai coagulation (DIC)
epidemiology, 506
etiology and pathogenesis, 506-507
management, 508
oveiview, 506
Disseminated penicillinosis, 758, 759[
Disseminated supeificial actinic poiokeiatosis
(DSAP), 96, 96[-97[
DIV. See Dominant ichthyosis vulgaiis (DIV)
Dominant ichthyosis vulgaiis (DIV)
diagnosis, 74
epidemiology, 72
management, 74
oveiview, 72
pathogenesis, 74
Donovanosis, 934, 935[
Diug- and chemical-induced photosensitivity
oveiview, 236, 238
photoalleigic diug- and chemical-induced
photosensitivity
diagnosis, 245-246
epidemiology, 244
IN0X 1084
Diug- and chemical-induced
photosensitivity (ConnueJ)
management, 246
oveiview, 244
phototoxic, 238
phytophotodeimatitis
couise, 242
management, 242
oveiview, 242
systemic phototoxic deimatitis
epidemiology, 240
management, 240
topical phototoxic deimatitis, 241
Diug-induced nail changes, 1027, 1027[, 1027
Diug ieactions
ACDR-ielated neciosis, 575, 576[-578[
ACDR ielated to chemotheiapy, 579, 579[, 580-581
clinical types, 553, 554-556
diagnosis, 557
diug hypeisensitivity syndiome
diagnosis, 569
management, 570
oveiview, 568
pathogenesis, 568
diug-induced acute uiticaiia, angioedema, edema,
and anaphylaxis
classification, 563
diagnosis, 564
management, 564
oveiview, 563, 563
diug-induced pigmentation
diugs causing hypeipigmentation, 570
oveiview, 570
exanthematous diug ieactions
clinical manifestations, 557-560, 558r-559[
couise, 560
epidemiology, 557
management, 560
oveiview, 557
findings indicating possible life-thieatening
ieactions, 552
fixed diug eiuption
management, 568
oveiview, 566, 566
pathogenesis, 566, 567[
guidelines, 552
management, 557
oveiview, 552
pseudopoiphyiia, 574, 574[
pustulai eiuptions, 561, 561[-562[
DSAP (disseminated supeificial actinic
poiokeiatosis), 96, 96[-97[
Dyshidiotic eczematous deimatitis (DED)
management, 45
oveiview, 45-45f
Dysmoiphic syndiome, 582
Dysplastic melanocytic nevus
clinical manifestations, 300, 301[, 302, 303[-304[
diagnosis and diffeiential diagnosis, 301, 302, 304
epidemiology, 300
oveiview, 300
pathogenesis, 300
E
EBA (epideimolysis bullosa acquisita), 120, 121[
Ecthyma. See Impetigo and ecthyma
Ecthyma contagiosum, 776
Eczema/deimatitis
alleigic contact deimatitis
alleigens, 26, 28
couise, 28
epidemiology, 26
management, 32-33
oveiview, 26
pathogenesis, 26
special foims
aiiboine, 32, 33[
alleigic contact deimatitis due to plants
diagnosis, 32
oveiview, 29
pathogenesis, 30
systemic, 32
asteatotic deimatitis, 52, 52[
atopic deimatitis
clinical manifestations, 35[, 36, 37[-42[
complications, 38
diagnosis, 36
epidemiology, 34
oveiview, 34
special foims, 38
autosensitization deimatitis, 48, 49[
contact deimatitis, 20
deimatitis heipetifoimis
IN0X 1085
Eczema/deimatitis (ConnueJ)
deimatitis heipetifoimis (ConnueJ)
couise, 118
epidemiology, 116
management, 118
oveiview, 116
dyshidiotic eczematous deimatitis (DED)
management, 45
oveiview, 45-45f
iiiitant contact deimatitis
acute
chionic
types, 22
epidemiology, 21
etiology, 21, 21
management, 24
oveiview, 20
pathogenesis, 21
special foims, 22, 24, 29[
lichen simplex chionicus
management, 43
oveiview, 42
pathogenesis, 42-43
nummulai eczema (NE)
epidemiology, 46
management, 46
oveiview, 46
oveiview, 20
piuiigo nodulaiis (PN), 44, 44[
seboiiheic deimatitis (SD)
diagnosis/diffeiential diagnosis, 49
laboiatoiy studies, 49-50
management, 50-51
oveiview, 48
pathogenesis, 48
EH. See Epideimolytic hypeikeiatosis (EH)
Endociine diseases
Addison disease, 433, 434[
Cushing syndiome and hypeicoiticism, 430, 430[
diabetes mellitus
associated skin diseases, 424-425
diabetic bullae, 425, 425[
diabetic deimopathy, 427, 427[
diabetic foot," 426
diabetic neuiopathy, 426, 426[
neciobiosis lipoidica
management, 429
oveiview, 428
pathogenesis, 428
Giaves disease and hypeithyioidism, 431, 432[
hypothyioidism and myxedema, 431, 433[
skin diseases in piegnancy
atopic eiuption of piegnancy, 422
cholestatis of piegnancy, 420
oveiview, 420, 421[
pemphigoid gestationis, 420
polymoiphic eiuption of piegnancy (PEP), 422, 423[
piuiigo of piegnancy, 422
pustulai psoiiasis in piegnancy, 420
skin manifestations of obesity, 424
Eosinophilic folliculitis
diagnosis, 948
epidemiology and pathogenesis, 948
management, 948
oveiview, 948
Epideimal disoideis, miscellaneous
acanthosis nigiicans (AN)
clinical manifestations, 88-89
epidemiology, 88
management, 89
oveiview, 88
Daiiei disease
1010, 1010[
disease association, 91
management, 91
oveiview, 90
disseminated supeificial actinic poiokeiatosis
(DSAP), 96, 96[-97[
Giovei disease
clinical manifestation, 92, 93[
epidemiology, 92
management, 92
oveiview, 92
pityiiasis iubia pilaiis
classification, 93
epidemiology, 93
management, 94-95
oveiview, 93
IN0X 1086
Epideimal inclusion cyst, 212, 212[
Epideimal nevus, 222, 223[
Epideimal piecanceis and canceis
cutaneous hoin, 275, 276[
epithelial piecanceious lesions and squamous cell
caicinoma in situ, 274
oveiview, 274
solai oi actinic keiatoses, 275, 275[
Epideimoid cyst, 211, 211[
Epideimolysis bullosa acquisita (EBA), 120, 121[
Epideimolytic hypeikeiatosis (EH)
epidemiology, 81
management, 81
oveiview, 81
Eiuptive xanthoma, 438, 438[
Eiysipelas and cellulitis
clinical manifestations, 611-612, 612[-616[, 614
diagnosis, 616
oveiview, 609
pathogenesis, 611
Eiythema infectiosum
diagnosis, 808
management, 808
oveiview, 806
pathogenesis, 806
Eiythema multifoime syndiome
couise, 149
epidemiology, 149
etiology, 148
management, 151
oveiview, 148
Eiythema nodosum syndiome
couise, 152
management, 152
oveiview, 152
Eiythiasma
couise, 593
diagnosis, 593
epidemiology, 592
management, 593
oveiview, 592
Eiythiopoietic piotopoiphyiia
diagnosis, 260
epidemiology, 259
management, 260
oveiview, 259
pathogenesis, 259
Exanthematous diug ieactions
clinical manifestations, 557-560, 558r-559[
couise, 560
epidemiology, 557
management, 560
oveiview, 557
Exanthems, infectious
diagnosis, 797
management, 797
measles
management, 802
oveiview, 800
pathogenesis, 800
oveiview, 795
pathogenesis, 795
iubella
diagnosis, 798
management, 798
oveiview, 798
Exfoliative eiythiodeima syndiome
diagnosis, 166
epidemiology, 164
etiology, 164, 164-165
management, 166
oveiview, 164
pathogenesis, 165
Extiamammaiy Paget disease, 1066, 1067[
F
Fabiy disease, 206, 207[
Factitious syndiomes (Munchhausen syndiome), 586,
586[-587[
Familial benign pemphigus, 105, 105[
Fat distiibution, abnoimalities of in AIDS
diagnosis, 954
management, 954
IN0X 108T
Fat distiibution, abnoimalities of in AIDS (ConnueJ)
oveiview, 953
Felon, 1014, 1014[
Female-pattein baldness, 965
Fifth disease, 806
Fish tank gianuloma, 678
Folliculitis, infectious
clinical manifestations, 994, 994[-997[, 996-997,
999[
diagnosis, 998
laboiatoiy findings, 998
management, 998
oveiview, 993
Foidyce, angiokeiatoma of, 206, 207[
Fungal infections of skin and haii
candidiasis
classification, 719
laboiatoiy examination, 718-719, 720[
management, 719
oveiview, 718
candidiasis of the nail appaiatus. See Nail
appaiatus, disoideis of
chionic mucocutaneous candidiasis, 730, 731[
cutaneous candidiasis
diagnosis, 722
management, 722
oveiview, 720
deimatophytoses
classification, 694
deimatophytoses of epideimis, 697
deimatophytoses of haii (tiichomycosis),
708, 709[
deimatophytic folliculitis. See Folliculitis,
infectious
tinea baibae
management, 716
oveiview, 715
tinea capitis
couise, 715
management, 714
oveiview, 709
pathogenesis, 710
management, 696
oveiview, 693
pathogenesis, 693[, 694-695
tinea coipoiis
management, 705
oveiview, 704
tinea ciuiis
management, 704
oveiview, 703
tinea facialis
management, 707
oveiview, 707
tinea incognito, 704[-706[, 708
tinea manuum
couise, 703
management, 703
oveiview, 701
tinea pedis
classification, 700
diagnosis, 700
epidemiology, 697
management, 701
oveiview, 697
genital candidiasis
diagnosis, 728
oveiview, 727
invasive and disseminated fungal infections
oveiview, 737
subcutaneous mycoses
chiomomycosis
diagnosis, 743
management, 743
oveiview, 742
mycetoma
diagnosis, 741
management, 741
oveiview, 738
pathogenesis, 738
oveiview, 737
spoiotiichosis
IN0X 1088
Fungal infections of skin and haii (ConnueJ)
invasive and disseminated fungal infections (ConnueJ)
spoiotiichosis (ConnueJ)
diagnosis, 746
management, 746
oveiview, 744
pathogenesis, 744
Ma|asse:a infections
Ma|asse:a folluliculitis, 999, 999[
oveiview, 732
pityiiasis veisicoloi
diagnosis, 734
management, 734
oveiview, 732
pathogenesis, 732
oiophaiyngeal candidiasis
classification of mucosal candidiasis, 724
diagnosis, 726
management, 727
oveiview, 724
oveiview, 692
supeificial fungal infections, 692
systemic fungal infections with dissemination to skin
acute candidemia and disseminated candidiasis,
758, 759[
blastomycosis: cutaneous manifestations
diagnosis, 754
management, 754
oveiview, 753
pathogenesis, 753
disseminated coccidioidomycosis
clinical manifestation, 756-757, 757[
diagnosis, 757
management, 757
oveiview, 755
pathogenesis, 756
disseminated ciyptococcosis
diagnosis, 748
management, 748
oveiview, 747
pathogenesis, 747
disseminated penicillinosis, 758, 759[
histoplasmosis
diagnosis, 752
management, 752
oveiview, 749
pathogenesis, 750
oveiview, 747
tinea nigia, 736, 737[
Trt|osoron infections, 736
Fuiuncle. See Abscess, fuiuncle, and caibuncle
G
Genetic diseases
heieditaiy hemoiihagic telangiectasia,
457, 457[
neuiofibiomatosis
epidemiology, 453
management, 455
oveiview, 453
pathogenesis, 453
pseudoxanthoma elasticum, 448, 449[
tubeious scleiosis
associated systems, 450
diagnosis, 452
epidemiology, 449
management, 452
oveiview, 449
pathogenesis, 450
Genitalia, peiineum, and anus, disoideis of
anogenital infections, 1066
disoideis specific to genital anatomy
chionic lymphedema of the genitalia,
1048, 1049[
phimosis, paiaphimosis, balanitis xeiotica
obliteians, 1050, 1050[
plasma cell balanitis and vulvitis, 1048, 1049[
scleiosing lymphangitis of penis, 1048, 1049[
extiamammaiy Paget disease, 1066, 1067[
genital candidiasis
diagnosis, 728
oveiview, 727
genital veiiucous caicinoma, 1064
invasive anogenital squamous cell caicinoma
invasive SCC of cutaneous anus, 1064
invasive SCC of penis, 1064
invasive SCC of vulva, 1064
Kaposi saicoma. See Kaposi saicoma
malignant melanoma of anogenital iegion, 327,
1065, 1065[-1066[
IN0X 1089
Genitalia, peiineum, and anus, disoideis of (ConnueJ)
mucocutaneous disoideis
eczematous deimatitis
alleigic contact deimatitis, 1059, 1059[
atopic deimatitis, 1060
fixed diug eiuption, 1061, 1061[
lichen simplex chionicus, 1060, 1060[
piuiitus ani, 1060
genital aphthous ulceiations, 1058, 1058[
genital (penile/vulvai/anal) lentiginoses, 1051,
1051[
lichen nitidus, 1055, 1055[
lichen planus, 1054, 1054[
lichen scleiosus, 1055, 1056[-1057[
migiatoiy neciolytic eiythema, 1058
psoiiasis vulgaiis, 1052, 1052[
vitiligo and leukodeima, 1052, 1052[
oveiview, 1046
piemalignant and malignant lesions
HPV-induced intiaepithelial neoplasia, 1062
squamous cell caicinoma in situ, 1062,
1062[-1063[
vaiiants of genital anatomy
angiokeiatoma, 1047
chionic pain syndiome, 1047
oveiview, 1046
peaily penile papules, 1046, 1047[
sebaceous gland piominence, 1046
Geiman measles, 798
Gianotti-Ciosti syndiome, 809, 809[
Giant cell aiteiitis
management, 406
oveiview, 405
pathogenesis, 406
Gilchiist disease, 753
Gingiva, peiiodontium, and mucous membiane
disoideis
acute neciotizing ulceiative gingivitis, 1032, 1033[
eiosive gingivostomatitis, 1030
gingival hypeiplasia, 1032, 1033[
lichenoid mucositis, 1030
lichen planus, 1031, 1031[-1032[
Glomus tumoi, 199, 199[
Glossitis, migiatoiy, 1030, 1030[
Glucagonoma syndiome, 500, 500[-501[
Goilin syndiome, 294
Gougeiot-Blum disease, 144
Gout, 446, 447[
Giaft-veisus-host disease
acute cutaneous giaft veisus host ieaction
clinical manifestations, 546, 546, 547[-549[, 548
management, 550
pathogenesis, 546
chionic cutaneous giaft veisus host ieaction
clinical examination, 550, 551[
management, 550
oveiview, 546
Giam-negative infections
bacillaiy angiomatosis
diagnosis, 659
histoiy, 658
management, 659
oveiview, 658
Barone||a infections
in HIV/AIDS, 658
oveiview, 654
cat-sciatch disease
diagnosis, 656-657
management, 657
oveiview, 655
pathogenesis, 656
PseuJomonas species
cutaneous PseuJomonas aerugnosa
infections
diagnosis, 664
epidemiology, 663
management of invasive infections, 664
oveiview, 662
pathogenesis, 663
oveiview, 662
tulaiemia
diagnosis, 662
management, 662
oveiview, 659-660
pathogenesis, 660
Giam-positive bacillaiy infections associated with
toxin pioduction (intoxications)
cutaneous anthiax
diagnosis, 635
management, 636
oveiview, 634
cutaneous diphtheiia, 637
oveiview, 634
tetanus, 637
Giam-positive coccal infections associated with toxin
pioduction (intoxications)
oveiview, 625
scailet fevei
IN0X 1090
Giam-positive coccal infections associated with toxin
pioduction (intoxications) (ConnueJ)
diagnosis, 633
management, 633
oveiview, 631
staphylococcal scalded-skin syndiome (SSSS)
diagnosis, 628
management, 628
oveiview, 626
staphylococcal toxins, 625
stieptococcal toxins, 625
supeiantigens, 625-626
toxic shock syndiome
diagnosis, 630
management, 631
oveiview, 629
pathogenesis, 629
Gianuloma annulaie
couise, 134
epidemiology, 134
management, 134
oveiview, 134
Gianuloma faciale, 163, 163[
Gianuloma inguinale, 934
Gianulomatosus slack skin, 530, 533[
Giaves disease and hypeithyioidism, 431, 432[
Gieen nail syndiome, 1004
Giovei disease, 92, 93[
Guttate paiapsoiiasis, 146
H
Haemo||us Jutrey. chancioid
diagnosis, 933
etiology and epidemiology, 931-932
management, 933
oveiview, 931
pathogenesis, 932
Haii, nail, and mucosal disoideis, skin signs of
disoideis of the genitalia, peiineum, and anus. See
Genitalia, peiineum, and anus, disoideis of
excess haii giowth
hiisuitism
etiology and epidemiology, 989, 989
laboiatoiy evaluation, 990
management, 990
oveiview, 989
hypeitiichosis
etiology, 992
management, 992
oveiview, 992
oveiview, 989
geneialized piuiitus without skin lesions. See
Piuiitus, geneialized, without skin lesions
(piuiitus sine mateiia)
haii follicle disoideis and ielated disoideis
biology of haii giowth cycles, 962-963, 964[
oveiview, 962
pathogenesis appioach to diagnosis, 964
haii loss: alopecia
alopecia aieata
couise, 974
management, 974-975
oveiview, 971
anagen effluvium
couise, 980
etiology, 979
management, 980
oveiview, 979
cicatiicial oi scaiiing alopecia
acne neciotica, 988
alopecia mucinosa (folliculai mucinosis), 984
cential centiifugal scaiiing alopecia, 982, 984
chionic cutaneous (discoid) lupus
eiythematosus, 981, 982[-983[
dissecting folliculitis, 986, 987[
eiosive pustulai deimatosis of scalp, 988
folliculitis decalvans, 984, 986[
folliculitis keloidalis nuchae, 986, 987[
lichen planopilaiis, 981-982, 984[
management, 988
oveiview, 981, 981
pseudofolliculitis baibae, 986, 988, 988[
pseudopelade of Biocq, 982, 985[
classification, 964
oveiview, 965, 965
pattein haii loss
couise, 968
diagnosis, 968
management, 970
oveiview, 965
pathogenesis, 966
telogen effluvium
diagnosis, 977
IN0X 1091
Haii, nail, and mucosal disoideis, skin
signs of (ConnueJ)
telogen effluvium (ConnueJ)
management, 977
oveiview, 975
pathogenesis, 976
infectious folliculitis
clinical manifestations, 994, 994[-997[, 996-997,
999[
diagnosis, 998
management, 998
oveiview, 993
mouth disoideis. See Mouth, disoideis of
Halo nevomelanocytic nevus
epidemiology, 183
management, 184
oveiview, 183
pathogenesis, 183
Hand-foot-and-mouth disease
diagnosis, 804
oveiview, 803
pathogenesis, 803
Hansen disease, 665
Hailequin fetus, 84
Hemangioma of infancy
diagnosis, 194
epidemiology, 193
management, 194
Hematologic disease, skin signs of
ciyoglobulinemia
oveiview, 509
disseminated intiavasculai coagulation (DIC)
epidemiology, 506
management, 508
oveiview, 506
Langeihans cell histiocytosis
diagnosis, 516
management, 517
oveiview, 514
physical examination, 515-516, 515[-518[
leukemia cutis, 511, 512[-513[
mastocytosis syndiomes
diagnosis, 520
epidemiology, 519
management, 522
oveiview, 519, 519
thiombocytopenic puipuia
diagnosis, 506
epidemiology, 504
management, 506
oveiview, 504
Heiald patch, 122
Heieditaiy epideimolysis bullosa
clinical phenotypes, 98, 99[, 100, 101[, 102, 103[-104[
diagnosis, 102
management, 102
oveiview, 98
Heieditaiy hemoiihagic telangiectasia, 457, 457[
Heipangina, 797[, 804
Heipesviiuses
heipes simplex viius infection
diagnosis, 816
in HIV/AIDS, 958
oveiview, 813
pathogenesis, 815-816, 817[
heipes simplex viius: genital infections
diagnosis, 916
laboiatoiy studies, 916
oveiview, 912
heipes simplex viius: infections associated with
systemic immunocompiomise
diagnosis, 830
epidemiology, 827
management, 831
oveiview, 827
pathogenesis, 827
IN0X 1092
Heipesviiuses (ConnueJ)
heipes simplex viius: widespiead cutaneous
infection associated with cutaneous
immunocompiomise
diagnosis, 826
management, 826
oveiview, 825
pathogenesis, 825
HHV-6 and -7 infections: exanthema subitum
diagnosis, 850
management, 850
oveiview, 850
pathogenesis, 850
neonatal heipes simplex viius infection, 823, 824[
nongenital heipes simplex viius infection
diagnosis, 822
management, 822
oveiview, 818
oveiview, 813, 814-815
Hidiadenitis suppuiativa
epidemiology, 16
management, 18
Hiisuitism
etiology and epidemiology, 989, 989
laboiatoiy evaluation, 990
management, 990
oveiview, 989
Histoplasmosis
diagnosis, 752
management, 752
oveiview, 749
pathogenesis, 750
HIV/AIDS disease, human ietioviial infections and
mucocutaneous manifestations of
abnoimalities of fat distiibution
diagnosis, 954
management, 954
oveiview, 953
acute HIV/AIDS syndiome (AHS)
diagnosis, 946
oveiview, 946
adveise cutaneous diug eiuptions
ACDE by diug type, 951-952, 953[
classification, 951
management, 951
oveiview, 951
pathogenesis, 951
eosinophilic folliculitis
diagnosis, 948
epidemiology and pathogenesis, 948
management, 948
oveiview, 948
global HIV/AIDS pandemic, 942
HIV/AIDS disease and AIDS
clinical findings, 943, 944
laboiatoiy examination, 945, 945[, 947[
management, 945
oveiview, 942
pathogenesis, 943
oial haiiy leukoplakia
diagnosis, 950
management, 950
oveiview, 950
oveiview, 942
vaiiations of common mucocutaneous
disoideis in HIV/AIDS disease
aphthous stomatitis, 956
deimatophytoses, 956
disseminated fungal infection, 958
heipes simplex viius infection, 958
human papillomaviius infection, 958, 959[
Kaposi saicoma (KS), 955
molluscum contagiosum, 958, 959[
mucosal candidiasis, 956, 958
nonmelanoma skin canceis, 955
oveiview, 955, 960
staphylococcus auieus infection, 956, 956[-957[
syphilis, 960
vaiicella-zostei viius infection, 958
Hot tub" folliculitis, 996, 996[
HPV-induced intiaepithelial neoplasia, 1062
Human oif
couise, 777
diagnosis, 776
epidemiology, 776
management, 777
oveiview, 776
IN0X 1093
Human papillomaviius infections
cutaneous infections
diagnosis, 794
management, 794
oveiview, 788
etiology, 787
in HIV/AIDS, 958, 959[
mucosal infections
exteinal genital waits
diagnosis, 904
oveiview, 900
pathogenesis, 901
squamous cell caicinoma in situ (SCCIS) and
invasive SCC of anogenital skin
diagnosis, 909
management, 910
oveiview, 908
pathogenesis, 909
oveiview, 787
Hypeipigmentation, 346, 346[-348[
Hypeisensitivity vasculitis
management, 398
oveiview, 397
pathogenesis, 397
Hypeitiichosis
etiology, 992
management, 992
oveiview, 992
Hypeitiophic scais and keloids
management, 228
oveiview, 227
Hypopigmentation, 349, 349[-352[
Hypothyioidism and myxedema, 431, 433[
I
Ichthyoses
acquiied ichthyoses, 84
classification, 72
dominant ichthyosis vulgaiis (DIV)
diagnosis, 74
epidemiology, 72
management, 74
oveiview, 72
pathogenesis, 74
epideimolytic hypeikeiatosis (EH)
epidemiology, 81
management, 81
oveiview, 81
inheiited keiatodeimas of palms and soles
diffuse palmoplantai keiatodeima, 85, 85[
focal palmoplantai keiatodeima
management, 87
oveiview, 86, 87[
oveiview, 84
punctate palmoplantai keiatodeima, 85, 86[
lamellai ichthyosis (LI)
epidemiology, 78
management, 80
oveiview, 78
in the newboin
collodion baby, 83, 83[
hailequin fetus, 84
management, 83
oveiview, 72
syndiomic ichthyoses, 84
X-linked ichthyosis (XLI)
diagnosis, 76
epidemiology, 75
management, 76
oveiview, 75
Idiopathic lobulai panniculitis, 154, 155[
Immune, autoimmune, and iheumatic disoideis
Behet disease
epidemiology, 366
management, 368
oveiview, 366
pathogenesis, 366
deimatomyositis
IN0X 1094
Immune, autoimmune, and iheumatic
disoideis (ConnueJ)
deimatomyositis (ConnueJ)
management, 373
oveiview, 370
Kawasaki disease
clinical manifestations/phases, 410, 411[, 412, 413[
management, 412
oveiview, 410
pathogenesis, 410
livedo ieticulaiis
etiology, 374
management, 374
oveiview, 374
pathogenesis, 374
localized cutaneous amyloidosis, 356, 357[
lupus eiythematosus. See Lupus eiythematosus
Raynaud phenomenon
diagnosis, 394, 396
epidemiology, 394
management, 396
oveiview, 394
pathogenesis, 394
piognosis, 394
ieactive aithiitis (Reitei syndiome)
management, 416
oveiview, 414
pathogenesis, 414
saicoidosis
diagnosis, 417
epidemiology, 417
oveiview, 417
scleiodeima
classification, 389, 390[-393[
epidemiology, 389
geneial examination, 390
management, 392
oveiview, 389
scleiodeima-like conditions, 393
Sneddon syndiome
epidemiology, 376
management, 376
oveiview, 376
systemic amyloidosis
oveiview, 354
systemic AA amyloidosis, 356
systemic AL amyloidosis
diagnosis, 355
oveiview, 354
uiticaiia and angioedema
clinical types, 358, 360[-361[
diagnosis, 362, 364-365, 364[
management, 365
oveiview, 358, 359[-360[
special featuies as ielated to pathogenesis, 358,
360, 361[, 362, 363[, 365[
vasculitis. See Vasculitis
Impetigo and ecthyma
diagnosis, 600
oveiview, 597
Impetigo heipetifoimis, 66
Industiial accidents, 1074-1075, 1074
Infantile digital fibiomatosis, 230, 230[
Infective endocaiditis
diagnosis, 640-641
management, 642
oveiview, 638
pathogenesis, 639
Infestations
mite bites and infestations
cutaneous laiva migians
couise, 878
diagnosis, 878
management, 879
oveiview, 876
pathogenesis, 877
oveiview, 868
scabies
cliinical manifestations, 869-870, 871[, 872,
873[-875[
diagnosis, 872
IN0X 1095
Infestations (ConnueJ)
scabies (ConnueJ)
oveiview, 868
pathogenesis, 869
pediculosis
management, 860-861
oveiview, 860
pediculosis capitis
diagnosis, 862
management, 862-863
oveiview, 861
pediculosis coipoiis, 865
diagnosis, 865
epidemiology and etiology, 863-864, 864[
management, 865
oveiview, 863
pediculosis pubis (pthiiiasis)
clinical manifestations, 865-866, 866[867[
diagnosis, 866
epidemiology, 865
management, 866-868
oveiview, 865
watei-associated infections and infestations
ceicaiial deimatitis, 880, 881[
envenomations caused by Cnidaiia (jellyfish,
Poituguese man-of-wai, sea anemones, coials),
882, 883[
injuiies caused by echinodeims (sea uichins, sea
stais, and sea cucumbeis), 882
oveiview, 879, 879
seabathei`s eiuption, 880, 881[
Injecting diug use, cutaneous signs of, 587-588, 588[
Inteitiigo, nonspecific, 596, 596[-597[
Invasive squamous cell caicinoma (SCC)
management, 282
oveiview, 280
Iiiitant contact deimatitis
acute
chionic
types, 22
epidemiology, 21
etiology, 21, 21
management, 24
oveiview, 20
pathogenesis, 21
special foims, 22, 24, 29[
J
Jock itch," 703
K
Kaposi saicoma, 1066, 1067[
classification and clinical vaiiants, 538
etiopathogenesis, 538
management, 542
oveiview, 538
pathogenesis, 538
physical examination, 538, 539[, 540, 541[, 543[
Kaposi saicoma (KS), 955
Kava deimopathy, 84
Kawasaki disease
clinical manifestations/phases, 410, 411[,
412, 413[
management, 412
oveiview, 410
pathogenesis, 410
Keiatoacanthoma
epidemiology, 286
management, 286
oveiview, 286
pathogenesis, 286
Keiatodeima blennoiihagicum, 415[
Keiatodeimas, inheiited, of palms and soles
diffuse palmoplantai keiatodeima, 85, 85[
focal palmoplantai keiatodeima
management, 87
oveiview, 86, 87[
oveiview, 84
punctate palmoplantai keiatodeima, 85, 86[
Keiion, 713[
Kissing waits," 790, 791[
Koilonychia, 1026, 1026[
L
Lamellai ichthyosis
epidemiology, 78
management, 80
oveiview, 78
Langeihans cell histiocytosis
diagnosis, 516
IN0X 1096
Langeihans cell histiocytosis (ConnueJ)
management, 517
oveiview, 514
physical examination, 515-516, 515[-518[
Leg and foot ulceis, most common
aiteiial ulceis, 470-471, 473f
combined aiteiial and venous ulceis, 471, 473[
diffeiential diagnosis, 471, 472, 474
management, 474
neuiopathic ulceis, 471
oveiview, 470
venous ulceis, 470, 471[-472[
Leishmaniasis
clinical manifestations, 886, 887[-891[, 888-889
diagnosis, 890-891
epidemiology, 884-886, 885
oveiview, 884
pathogenesis, 886
Lentiginoses, genital, 1051, 1051[
Lentigo maligna, 311-312, 311[, 313[, 314
Lentigo maligna melanoma
clinical manifestations, 311[, 312, 313[, 314
epidemiology, 312
management, 315
oveiview, 311[, 312, 313[
piognosis, 314-315, 315
Leonine facies, 526, 529[
Lepiosy
clinicopathologic classification, 665
diagnosis, 670
oveiview, 665
pathogenesis, 666
Letteiei-Siwe disease, 514, 517[-518[
Leukemia cutis, 511, 512[-513[
Leukodeima, 1052, 1052[
Leukonychia, 1021, 1022[
Leukoplakia, 1036, 1036
Lichen auieus, 144
Lichen nitidus, 1055, 1055[
Lichenoid mucositis, 1030
Lichen planus, 1031, 1031[-1032[, 1054, 1054[
couise, 130
lichen planus-like eiuptions, 130, 131
management, 130
nail appaiatus and
management, 1008
oveiview, 1008
oveiview, 128
Lichen scleiosus, 1055, 1056[-1057[
Lichen scleiosus et atiophicus, 142, 143[
Lichen simplex chionicus, 1060, 1060[
management, 43
oveiview, 42
pathogenesis, 42-43
Lime deimatitis, 242
Linea nigia, 420
Lineai IgA deimatosis, 119, 119[
Lineai moiphea, 136, 139[
Lingua villosa, 1029
Lipoma, 224, 225[
Lips, disoideis of, 1028
Livedoid vasculitis, 475, 475[
Livedo ieticulaiis
etiology, 374
management, 374
oveiview, 374
pathogenesis, 374
Localized cutaneous amyloidosis, 356, 357[
Louse-boine typhus, 768
Lupus eiythematosus, 1044, 1045[
cutaneous
acute, 380
chionic cutaneous, 384
chionic lupus panniculitis
management, 388
oveiview, 388
classic chionic discoid
clinical manifestations, 384, 386
epidemiology, 384
management, 386
oveiview, 384, 385[, 387[
subacute
epidemiology, 383
management, 384
oveiview, 383
Gilliam classification, 377
oveiview, 376, 377[
systemic
diagnosis, 380, 382
epidemiology, 378
laboiatoiy examinations, 378, 380, 380
management, 380
oveiview, 378
piognosis, 380
Lyme boiieliosis
clinical manifestations, 684-685, 685[-687[, 685,
687-688, 689[-690[
IN0X 109T
Lyme boiieliosis (ConnueJ)
diagnosis, 688-689
management, 690, 691[
oveiview, 684
pathogenesis, 684
Lymphangioma, 208, 208[
Lymphangitis
diagnosis, 620
management, 620
oveiview, 619
Lymphatic insufficiency, chionic, 476, 476[
Lymphomatoid papulosis, 535, 536[
M
Macioglossia, 356[
Maduia foot, 738
Majocchi disease, 144, 145[
Ma|asse:a infections
Ma|asse:|a folluliculitis, 999, 999[
oveiview, 732
pityiiasis veisicoloi
diagnosis, 734
management, 734
oveiview, 732
pathogenesis, 732
Male-pattein baldness, 965
Malignant acanthosis nigiicans, 502, 502[-503[
Malignant appendage tumois, 296
Mantoux test, 676[
Mastocytosis syndiomes
diagnosis, 520
epidemiology, 519
management, 522
oveiview, 519, 519
Measles
management, 802
oveiview, 800
pathogenesis, 800
Melanocytes, disoideis of
acquiied nevomelanocytic nevi
management, 181
oveiview, 178
blue nevus
epidemiology, 184
management, 185
oveiview, 184
pathogenesis, 184
halo nevomelanocytic nevus
epidemiology, 183
management, 184
oveiview, 183
pathogenesis, 183
nevus of Ota, 190, 190[-191[
nevus spilus, 186, 187[
Melanodeimatitis toxica, 348[
Melanoma piecuisois and piimaiy cutaneous
melanoma
acial lentiginous melanoma
epidemiology, 324
management, 325
oveiview, 324
piognosis, 325
amelanotic melanoma, 326, 326[
cutaneous melanoma
classification, 308
clinical piesentations of melanoma,
310, 310
data and facts, 310
impoitance of, 308-309, 309
melanoma giowth patteins, 309-310
melanoma iecognition, 310
oveiview, 308
desmoplastic melanoma, 323, 323[
lentigo maligna melanoma
clinical manifestations, 311[, 312, 313[, 314
epidemiology, 312
management, 315
oveiview, 311[, 312, 313[
piognosis, 314-315, 315
malignant melanoma of the mucosa, 327
management of melanoma
adjuvant theiapy, 333
biopsy and suigical tieatment guidelines, 332
follow-up, 333, 333
melanoma in situ, 311, 311[
metastatic melanoma, 328, 328[-330[
nodulai melanoma
diagnosis, 321
IN0X 1098
Melanoma piecuisois and piimaiy cutaneous
melanoma (ConnueJ)
epidemiology, 321
management, 321
oveiview, 320, 320[
pathogenesis, 321
piognosis, 314-315, 321
piecuisois of cutaneous melanoma
congenital nevomelanocytic nevus
epidemiology, 304
management, 306
oveiview, 304
pathogenesis, 304-305, 305[-307[
dysplastic melanocytic nevus
clinical manifestations, 300, 301[, 302,
303[-304[
diagnosis and diffeiential diagnosis, 301,
302, 304
epidemiology, 300
oveiview, 300
pathogenesis, 300
oveiview, 300
piognosis of melanoma, 332
staging of melanoma
miciostaging, 331
oveiview, 331
sentinel lymph node biopsy, 331
woikup, 331
supeificial spieading melanoma
clinical manifestations, 316-317, 317[, 319[
couise and piognosis, 314-315, 318
diagnosis, 318
epidemiology, 315
management, 318
oveiview, 315
Melanonychia, longitudinal, 1011, 1011[
Melasma
epidemiology, 344-345
management, 346
oveiview, 344
pathogenesis, 345
significance, 345
Meikel cell caicinoma, 296, 297[
Metabolic and nutiitional conditions
eiuptive xanthoma, 438, 438[
gout, 446, 447[
noimolipemic plane xanthoma, 439, 439[
pellagia, 445, 445[-446[
scuivy, 440, 441[
xanthelasma, 436, 437[
xanthomas, 434, 435
xanthoma stiiatum palmaie, 438, 439[
xanthoma tendineum, 436, 437[
xanthoma tubeiosum, 436, 437[
zinc deficiency and aciodeimatitis enteiopathica,
442, 443[-444[
Metabolic photosensitivity: the poiphyiias
eiythiopoietic piotopoiphyiia
diagnosis, 260
epidemiology, 259
management, 260
oveiview, 259
pathogenesis, 259
poiphyiia cutanea taida
epidemiology, 253
management, 254-255
oveiview, 252, 252
vaiiegate poiphyiia
epidemiology, 257
management, 258
oveiview, 257
Miciobial eczema, 46
Migiatoiy neciolytic eiythema, 500, 500[-501[, 1058
Migiatoiy phlebitis, 464
Milium, 213, 213[
Milkei`s nodules
couise, 778
diagnosis, 778
management, 778
oveiview, 778
Minocycline-induced pigmentation, 572, 573[
Mite bites and infestations
cutaneous laiva migians
couise, 878
diagnosis, 878
management, 879
oveiview, 876
pathogenesis, 877
oveiview, 868
scabies
873[-875[
oveiview, 868
pathogenesis, 869
Molluscum contagiosum
IN0X 1099
Molluscum contagiosum (ConnueJ)
diagnosis, 775
in HIV/AIDS, 958, 959[
management, 775
oveiview, 771
pathogenesis, 772
Mondoi disease, 464
Monkeypox, 782-783
Moiphea
classification of vaiious types of localized
scleiodeima, 136
couise, 138
diagnosis, 138
management, 138
oveiview, 136
Mouth, disoideis of
aphthous ulceiation
couise, 1035
diagnosis, 1034
epidemiology, 1034
management, 1035
oveiview, 1034
cutaneous disoideis
bullous pemphigoid, 1043
cicatiicial pemphigoid, 1044
oveiview, 1043
paianeoplastic pemphigus, 1043
pemphigus vulgaiis, 1043
eiythematous lesions and/oi leukoplakia,
1037, 1037
gingiva, peiiodontium, and mucous membiances
acute neciotizing ulceiative gingivitis,
1032, 1033[
eiosive gingivostomatitis, 1030
gingival hypeiplasia, 1032, 1033[
gingivitis and peiiodontitis, 1030
lichenoid mucositis, 1030
lichen planus, 1031, 1031[-1032[
leukoplakia, 1036, 1036
lips
actinic cheilitis, 1028
angulai cheilitis (Peilche), 1028
oveiview, 1028
piemalignant and malignant neoplasms
dysplasia and squamous cell caicinoma in situ,
1038, 1038[-1039[
oial invasive squamous cell caicinoma, 1038
oial veiiucous caicinoma, 1039, 1039[
oiophaiyngeal melanoma, 1040, 1040[
submucosal nodules
cutaneous odontogenic (dental) abscess,
1042, 1042[
iiiitation fibioma, 1041, 1041[
mucocele, 1040, 1041[
systemic diseases
lupus eiythematosus, 1044, 1045[
tongue
black oi white haiiy tongue, 1029
fissuied tongue, 1028, 1029[
migiatoiy glossitis, 1030, 1030[
oial haiiy leukoplakia, 1029
Mucocele, 1040, 1041[
Mucocutaneous lymph node syndiome, 410
Mucosal candidiasis in HIV/AIDS, 956, 958
Mucous membiane disoideis. See Skin and mucous
membiane disoideis
Muehicke lines, 1027[
Multiple hamaitoma syndiome, 498, 499[
Munchhausen syndiome, 586, 586[-587[
Mycetoma
diagnosis, 741
management, 741
oveiview, 738
pathogenesis, 738
Myto|atera and mycobacteiial infections
classification, 665
cutaneous tubeiculosis
diagnosis, 676
management, 676
oveiview, 671
pathogenesis, 672
epidemiology, 665
lepiosy
clinicopathologic classification, 665
diagnosis, 670
oveiview, 665
pathogenesis, 666
Myto|aterum [oruum complex infections
diagnosis, 682
epidemiology, 682
management, 682
oveiview, 682
Myto|aterum marnum infection
diagnosis, 679
epidemiology, 678
management, 680
oveiview, 677
IN0X 1100
Myto|atera and mycobacteiial infections (ConnueJ)
Myto|aterum u|terans infection
couise and piogosis, 681
diagnosis, 681
epidemiology, 680
management, 681
oveiview, 680, 681[
pathogenesis, 680
nontubeiculosis mycobacteiial infections, 677, 677
oveiview, 664
Mycosis fungoides
diagnosis and diffeiential diagnosis, 532,
532-533
management, 534
oveiview, 526
vaiiants, 530, 531[, 533[
Myxoid cysts of digits, 1011, 1011[
N
Nail appaiatus, disoideis of
glossaiy of abnoimalities, 1001-1002
infections
acute paionychia, 1014, 1014[
bacteiial infections, 1014
candida onychia
management, 1016
oveiview, 1015
felon, 1014, 1014[
fungal infections and onychomycosis, 1014
oveiview, 1014
tinea unguium/onychomycosis
diagnosis, 1019
management, 1020-1021, 1020
oveiview, 1016-1017, 1017[, 1019[
pathogenesis, 1018
involvement of cutaneous diseases
alopecia aieata, 1008, 1009[
chemical iiiitant oi alleigic damage oi
deimatitis, 1010, 1010[
Daiiiei disease (Daiiei-White disease, keiatosis
folliculaiis), 1010, 1010[
lichen planus
management, 1008
oveiview, 1008
psoiiasis
management, 1007
oveiview, 1006
local disoideis
chionic paionychia, 1001-1002, 1003[
gieen nail syndiome, 1004
onychauxis, 1004, 1005[
onychogiyphosis, 1004
onycholysis, 1003, 1004[
psychiatiic disoideis, 1005, 1005[
nail signs of multisystem diseases
clubbed nails, 1026, 1026[
diug-induced nail changes, 1027, 1027[, 1027
koilonychia, 1026, 1026[
leukonychia, 1021, 1022[
nail fold/peiiungual eiythema and telangiectasia,
1024, 1025[
peiiungual fibioma, 1022, 1023[
pteiygium inveisum unguium, 1024
splintei hemoiihages, 1024, 1024[
systemic amyloidosis, 1024, 1025[
tiansveise oi Beau lines, 1021, 1022[
yellow nail syndiome, 1022, 1023[
neoplasms
aciolentiginous melanoma, 1012, 1013[
longitudinal melanonychia, 1011, 1011[
myxoid cysts of digits, 1011, 1011[
nail matiix nevi, 1012
oveiview, 1011
squamous cell caicinoma, 1012, 1013[
nomal nail appaiatus, 1000-1002, 1001[
NE. See Nummulai eczema (NE)
Neciobiosis lipoidica
management, 429
oveiview, 428
pathogenesis, 428
Neciotizing soft tissue infections
clinical vaiiants, 618, 619[
management, 619
oveiview, 618
Nessera infections
Nessera gonorr|oea infection
disseminated gonococcal infection
couise and piognosis
diagnosis, 653
management, 653-654
oveiview, 652
local infection (gonoiihea)
diagnosis, 652
management, 652
oveiview, 650
management, 650
oveiview, 649
pathogenesis, 650
IN0X 1101
Neisseiia infections (ConnueJ)
Nessera menngJs infection
diagnosis, 648
etiology, 646
management, 648-649
oveiview, 646
pathogenesis, 647
Neoplasms and hypeiplasias, benign. See Benign
neoplasms and hypeiplasias
Nephiogenic fibiosing deimopathy, 483, 484[
Neuiofibiomatosis
epidemiology, 453
management, 455
oveiview, 453
pathogenesis, 453
Neuiotic excoiiations and tiichotillomania, 583,
583[-585[
Nevoid basal cell caicinoma syndiome, 294
Nevus of Ota, 190, 190[-191[
Nevus sebaceous, 222, 223[
Nevus spilus, 186, 187[
New Woild cutaneous leishmaniasis, 890[-891[
Nodulai melanoma
diagnosis, 321
epidemiology, 321
management, 321
oveiview, 320, 320[
pathogenesis, 321
piognosis, 314-315, 321
Nodulai vasculitis
management, 409
oveiview, 408
Noimolipemic plane xanthoma, 439, 439[
Noith Ameiican blastomycosis, 755[
Nummulai eczema (NE)
epidemiology, 46
management, 46
oveiview, 46
O
Obesity, skin manifestations of, 424
Old Woild cutaneous leishmaniasis, 887[-889[
Onychauxis, 1004, 1005[
Onychogiyphosis, 1004
Onycholysis, 1003, 1004[
Oial haiiy leukoplakia, 1029
diagnosis, 950
management, 950
oveiview, 950
Oif. See Human oif
Oigan and bone maiiow tiansplantation, skin
diseases in
giaft-veisus-host disease
acute cutaneous giaft veisus host ieaction
clinical manifestations, 546, 546, 547[-549[, 548
management, 550
pathogenesis, 546
chionic cutaneous giaft veisus host ieaction
clinical examination, 550, 551[
management, 550
oveiview, 546
most common infections associated with oigan
tiansplantation, 544
oveiview, 544
skin canceis associated with oigan tiansplantation,
545
Oiganoid nevus, 222
Oiophaiyngeal candidiasis
classification of mucosal candidiasis, 724
diagnosis, 726
management, 727
Oslei-Webei-Rendu syndiome, 457
P
Paget disease
extiamammaiy, 496, 496[
mammaiy, 494, 495[
Pagetoid ieticulosis, 530, 531[
Pancieatic panniculitis, 154, 156[
Panniculitis, 154, 155[-156[, 155
Panscleiotic moiphea, 136, 141[
PAPA syndiome, 4
Papulai aciodeimatitis of childhood, 809
Paianeoplastic pemphigus, 503, 503[, 1043
Paiapsoiiasis en plaques
laige-plaque paiapsoiiasis
management, 126
oveiview, 124
small-plaque paiapsoiiasis (digitate deimatosis)
lalboiatoiy examination, 126
management, 126
Paiasitic infections, systemic
cutaneous acanthamebiasis, 895
IN0X 1102
Paiasitic infections, systemic (ConnueJ)
cutaneous amebiasis, 895, 895[
cutaneous and mucocutaneous leishmaniasis
clinical manifestations, 886, 887[-891[, 888-889
diagnosis, 890-891
epidemiology, 884-886, 885
oveiview, 884
pathogenesis, 886
tiypanosomiasis
Ameiican tiypanosomiasis, 893, 893[
human Afiican tiypanosomiasis, 894
oveiview, 893
Paionychia, 1001-1002, 1003[
Pattein haii loss
couise, 968
diagnosis, 968
management, 970
oveiview, 965
pathogenesis, 966
Peaily penile papules, 1046, 1047[
Pediculosis
management, 860-861
oveiview, 860
Pediculosis capitis
diagnosis, 862
management, 862-863
oveiview, 861
Pediculosis coipoiis
diagnosis, 865
epidemiology and etiology, 863-864, 864[
management, 865
oveiview, 863
Pediculosis pubis (pthiiiasis)
diagnosis, 866
epidemiology, 865
management, 866-868
oveiview, 865
Pellagia, 445, 445[-446[
Pemphigoid gestationis, 115, 115[-116[, 420
Pemphigus
couise, 108, 110
epidemiology, 106
management, 110
oveiview, 106
vaiiants, 108
Pemphigus vulgaiis, 1043
Penicillin-induced angioedema, 565[
Penicillin-induced uiticaiia, 565[
PEP (polymoiphic eiuption of piegnancy), 422, 423[
Peiineum. See Genitalia, peiineum, and anus,
disoideis of
Peiioculai xanthoma, 436
Peiioial deimatitis
couise, 15
management, 15
oveiview, 14
Peiiungual fibioma, 1022, 1023[
Peilche, 1028
Peutz-Jegheis syndiome, 498, 499[
Pfeiffei-Webei-Chiistian disease, 154
Phenolphthalein fixed diug eiuption, 567[
Phenytoin diug hypeisentitivity syndiome, 569[
Photosensitivity and photo-induced disoideis
chionic photodamage
actinic keiatosis
cllinical manifestations, 267-268, 267[,
269[-270[
epidemiology, 267
management, 268
oveiview, 267
pathogenesis, 267
chondiodeimatitis nodulaiis helicis, 266, 266[
deimatoheliosis (photoaging")
epidemiology, 262
management, 264
oveiview, 262
pathogenesis, 262
solai lentigo
management, 266
oveiview, 264
diug- and chemical-induced photosensitivity. See
Diug- and chemical-induced photosensitivity
metabolic photosensitivity: the poiphyiias. See
Metabolic photosensitivity: the poiphyiias
photoexaceibated deimatoses, 250, 251
polymoiphous light eiuption
diagnosis, 248, 250
epidemiology, 248
management, 250
oveiview, 248
pathogenesis, 248
IN0X 1103
Photosensitivity and photo-induced disoideis
(ConnueJ)
skin ieactions to sunlight
acute skin damage (sunbuin)
epidemiology, 235
management, 236
oveiview, 235
pathogenesis, 235
oveiview, 232, 233[, 234, 234
solai uiticaiia, 250, 251[, 251
Phthisis, 671
Phytophotodeimatitis
couise, 242
management, 242
oveiview, 242
Pigmentaiy disoideis
albinism
oculocutaneous
diagnosis, 343-344
epidemiology, 341
management, 344
pathogenesis, 342
significance, 344
oveiview, 341, 342
melasma
management, 346
oveiview, 344
pathogenesis, 345
significance, 345
oveiview, 334
pigmentaiy changes following inflammation of
the skin
hypeipigmentation, 346, 346[-348[
hypopigmentation, 349, 349[-352[
vitiligo
diagnosis, 336
epidemiology, 335
management, 338, 340, 341[
oveiview, 335
pathogenesis, 335
Pigmented puipuiic deimatoses
couise, 145
management, 145
oveiview, 144
Pilai cyst, 212
Pinch puipuia," 355[
Pitted keiatolysis (keiatolysis sulcata)
diagnosis, 595
epidemiology, 594
management, 595
oveiview, 594
Pityiiasis alba, 352[
Pityiiasis lichenoides (acute and chionic)
management, 146
oveiview, 146
Pityiiasis iosea
couise, 123
management, 123
oveiview, 122
Pityiiasis iubia pilaiis
classification, 93
epidemiology, 93
management, 94-95
oveiview, 93
Pityiiasis veisicoloi, 349, 349[
diagnosis, 734
management, 734
oveiview, 732
pathogenesis, 732
Plewig and Kligman classification, 9
Polyaiteiitis nodosa
management, 402
oveiview, 400
pathogenesis, 400
Polymoiphic eiuption of piegnancy (PEP),
422, 423[
Polymoiphous light eiuption
diagnosis, 248, 250
epidemiology, 248
management, 250
oveiview, 248
pathogenesis, 248
IN0X 1104
Poiphyiia cutanea taida
epidemiology, 253
management, 254-255
oveiview, 252, 252
Poiphyiia vaiiegata, 257
Poit-wine stain
histopathology, 201
management, 202
oveiview, 201, 201[-202[
syndiomic, 202
Poxviius infections
cowpox, 782
human oif
couise, 777
diagnosis, 776
epidemiology, 776
management, 777
oveiview, 776
milkei`s nodules
couise, 778
diagnosis, 778
management, 778
oveiview, 778
molluscum contagiosum
diagnosis, 775
management, 775
oveiview, 771
pathogenesis, 772
monkeypox, 782-783
oveiview, 770, 771
smallpox
clinical findings, 780-781, 780[-781[
diagnosis, 781
management, 781-782
oveiview, 779
pathogenesis, 779
tanapox, 782-783
vaccinia
diagnosis, 786
management, 786-787
oveiview, 783
pathogenesis, 784
vaccination, 784
Piecanceious lesions and cutaneous caicinomas
aisenical keiatoses, 276, 277[
atypical fibioxanthomaas, 299, 299[
basal cell caicinoma (BCC)
epidemiology, 287
etiology, 287
oveiview, 287
basal cell nevus syndiome, 294, 295[
deimatofibiosaicoma piotubeians, 298, 298[
epideimal piecanceis and canceis
cutaneous hoin, 275, 276[
epithelial piecanceious lesions and squamous
cell caicinoma in situ, 274
oveiview, 274
solai oi actinic keiatoses, 275, 275[
invasive squamous cell caicinoma (SCC)
clinical manifestations, 280-282, 281[,
283[-285[
management, 282
oveiview, 280
keiatoacanthoma
epidemiology, 286
management, 286
oveiview, 286
pathogenesis, 286
malignant appendage tumois, 296
Meikel cell caicinoma, 296, 297[
squamous cell caicinoma in situ
etiology, 278
management, 279
oveiview, 276
Piegnancy
diug use in, 1075, 1075-1076
skin diseases in
atopic eiuption of piegnancy, 422
cholestatis of piegnancy, 420
oveiview, 420, 421[
pemphigoid gestationis, 420
polymoiphic eiuption of piegnancy (PEP),
422, 423[
piuiigo of piegnancy, 422
pustulai psoiiasis in piegnancy, 420
Piessuie ulceis
epidemiology, 477
management, 478
oveiview, 477
Piuiigo nodulaiis (PN), 44, 44[
IN0X 1105
Piuiigo of piegnancy, 422
Piuiitus, geneialized, without skin lesions (piuiitus
sine mateiia)
management, 1070
most impoitant causes, 1068-1070, 1069-1070,
1071[
oveiview, 1068, 1069[
piuiitus ani, 1071
Piuiitus ani, 1060
PseuJomonas species
cutaneous PseuJomonas aerugnosa infections
diagnosis, 664
epidemiology, 663
management of invasive infections, 664
oveiview, 662
pathogenesis, 663
oveiview, 662
Pseudopoiphyiia, 574, 574[
Pseudoxanthoma elasticum, 448, 449[
Psoiiasis, 167[
classification, 53
management
aciodeinatitis continua hallopeau, 71
geneialized psoiiasis, 69-71
geneialized pustulai psoiiasis, 65[, 71
localized, 68-69
oveiview, 67-68
psoiiatic aithiitis, 71
nail appaiatus and
management, 1007
oveiview, 69, 1006
oveiview, 53
psoiiasis vulgaiis
epidemiology, 53
pathogenesis, 54
psoiiatic aithiitis, 61[, 67
psoiiatic eiythiodeima, 67
pustulai psoiiasis
geneialized acute pustulai psoiiasis (Von
Zumbusch)
epidemiology, 64
oveiview, 64
pathogenesis, 64
special types, 66
palmoplantai pustulosis
epidemiology, 62
oveiview, 62
in piegnancy, 420
Psoiiasis vulgaiis, 1052, 1052[
Psychiatiic disoideis
classification, 582
cutaneous signs of injecting diug use, 587-588,
588[
delusions of paiasitosis, 582, 583[
dysmoiphic syndiome, 582
factitious syndiomes (Munchhausen syndiome),
586, 586[-587[
neuiotic excoiiations and tiichotillomania, 583,
583[-585[
Pteiygium inveisum unguium, 1024
Pthiiiasis. See Pediculosis pubis
Puiified piotein deiivative test, 676[
Puipuia fulminans, 506, 507[
Pyodeima
abscess, fuiuncle, and caibuncle
diagnosis, 606
epidemilogy and etiology, 605
management, 608
oveiview, 605
pathogenesis, 605
impetigo and ecthyma
diagnosis, 600
oveiview, 597
Pyodeima gangienosum
epidemiology, 157
management, 157
oveiview, 156
Pyogenic gianuloma, 198, 198[
Q
Queyiat, eiythioplasia of, 1062
R
Radiation deimatitis, 270-271, 271[-273[
Ranula, 1040
Rashes in the acutely ill febiile patient
laboiatoiy tests available foi quick diagnosis,
170, 172
oveiview, 170, 171[, 172, 173[
Rat bite" necioses, 391
Raynaud phenomenon
diagnosis, 394, 396
epidemiology, 394
management, 396
oveiview, 394
pathogenesis, 394
piognosis, 394
IN0X 1106
Reactive aithiitis (Reitei syndiome)
management, 416
oveiview, 414
pathogenesis, 414
Renal insufficiency, skin signs of
acquiied peifoiating deimatosis, 485, 485[
calciphylaxis
epidemiology, 480
management, 482
oveiview, 480
pathogenesis, 480
classification of skin changes, 480
nephiogenic fibiosing deimopathy, 483, 484[
Rheumatic disoideis. See Immune, autoimmune, and
iheumatic disoideis
Rickettsial infections
louse-boine typhus, 768
oveiview, 760, 761
iickettsialpox, 767, 769[
tick-boine spotted fevei
classification, 761
Rocky Mountain spotted fevei
diagnosis, 764
management, 764
oveiview, 761
pathogenesis, 762
tick-boine typhus
diagnosis, 767
management, 767
oveiview, 765
Rodent ulcei, 290[-291[
Rosacea
couise, 10
epidemiology, 9
management, 10-11
oveiview, 9
staging (Plewig and Kligman classification), 9
Roseola infantum, 850
Rose-thoin disease, 744
Rubella
diagnosis, 798
management, 798
oveiview, 798
Rubeola, 800
S
San Joaquin Valley fevei, 755
SAPHO syndiome, 4
Saicoidosis
diagnosis, 417
epidemiology, 417
oveiview, 417
Scabies
873[-875[
oveiview, 868
pathogenesis, 869
Scailet fevei
diagnosis, 633
management, 633
oveiview, 631
Schambeig disease, 144, 145[
Schnlein-Henoch puipuia, 399, 400[
Scleiodeima
classification, 389, 390[-393[
epidemiology, 389
management, 392
oveiview, 389
scleiodeima-like conditions, 393
Scleiodeima diabeticoium, 425
Scuivy, 440, 441[
Seabathei`s eiuption, 880, 881[
Sebaceous and apociine gland disoideis
acne vulgaiis
clinical manifestations, 2, 3[-5[, 4-6, 7[-8[
couise, 6
epidemiology, 2
management, 6-8
oveiview, 2
pathogenesis, 2
hidiadenitis suppuiativa
epidemiology, 16
IN0X 110T
Sebaceous and apociine gland disoideis (ConnueJ)
hidiadenitis suppuiativa (ConnueJ)
management, 18
peiioial deimatitis
couise, 15
management, 15
oveiview, 14
iosacea
couise, 10
epidemiology, 9
management, 10-11
oveiview, 9
staging (Plewig and Kligman classification), 9
Sebaceous cyst, 211
Sebaceous hypeiplasia, 222, 223[
Seboiiheic deimatitis
diagnosis/diffeiential diagnosis, 49
laboiatoiy studies, 49-50
management, 50-51
oveiview, 48
pathogenesis, 48
Seboiiheic keiatosis
epidemiology, 215
management, 216
oveiview, 215
Sepsis and septic shock
diagnosis, 644
management, 644
oveiview, 638, 642
pathogenesis, 643
Seveie and life-thieatening skin eiuptions in the
acutely ill patient
exfoliative eiythiodeima syndiome
diagnosis, 166
epidemiology, 164
etiology, 164, 164-165
management, 166
oveiview, 164
pathogenesis, 165
iashes in the acutely ill febiile patient
laboiatoiy tests available foi quick diagnosis, 170, 172
oveiview, 170, 171[, 172, 173[
Stevens-Johnson syndiome and toxic epideimal
neciolysis (TEN)
definition, 173-174
epidemiology, 174
geneial findings, 175
management, 176-177
oveiview, 173
sequelae, 176
Sexually tiansmitted infections
C||amyJa rat|omas infections
invasive infection: lymphogianuloma
veneieum
diagnosis, 941
epidemiology, 939
management, 941
oveiview, 939
pathogenesis, 939
localized infection
management, 939
oveiview, 937
oveiview, 936
pathogenesis, 937
syndiomes caused by, 936
donovanosis, 934, 935[
Haemo||us Jutrey. chancioid
diagnosis, 933
management, 933
oveiview, 931
pathogenesis, 932
heipes simplex viius: genital infections
diagnosis, 916
laboiatoiy studies, 916
oveiview, 912
human papillomaviius: mucosal infections
exteinal genital waits
diagnosis, 904
oveiview, 900
pathogenesis, 901
invasive SCC of anogenital skin
IN0X 1108
Sexually tiansmitted infections (ConnueJ)
human papillomaviius: mucosal infections
(ConnueJ)
invasive SCC of anogenital skin (ConnueJ)
diagnosis, 909
management, 910
oveiview, 908
pathogenesis, 909
management, 899-900
oveiview, 896, 896-899
syphilis
latent syphilis
congenital syphilis, 931
oveiview, 928
teitiaiy/late syphilis
couise, 930
diagnosis, 930
management, 924
oveiview, 919, 920[, 920
pathogenesis, 921
piimaiy syphilis
diagnosis, 924
secondaiy syphilis
clinical manifestations, 924, 926, 926[-927[,
928, 929[
couise, 928
diagnosis, 928
Szaiy syndiome, 530, 534
Shingles, 837
Skin and mucous membiane disoideis
benign neoplasms and hypeiplasias. See Benign
neoplasms and hypeiplasias
bullous diseases. See Bullous diseases
eczema/deimatitis. See Eczema/deimatitis
ichthyoses. See Ichthyoses
melanoma piecuisois and piimaiy cutaneous
melanoma. See Melanoma piecuisois and piimaiy
cutaneous melanoma
miscellaneous epideimal disoideis. See Epideimal
disoideis, miscellaneous
photosensitivity and photo-induced disoideis.
See Photosensitivity and photo-induced
disoideis
pigmentaiy disoideis. See Pigmentaiy disoideis
piecanceious lesions and cutaneous caicinomas. See
Piecanceious lesions and cutaneous caicinomas
psoiiasis. See Psoiiasis
iadiation, skin ieactions to, 270-271, 271[-273[
sebaceous and apociine glands. See Sebaceous and
apociine gland disoideis
seveie and life-thieatening skin eiuptions in the
acutely ill patient. See Seveie and life-thieatening
skin eiuptions in the acutely ill patient
Skin tag, 231, 231[
Sleeping sickness, 894
Smallpox
clinical findings, 780-781, 780[-781[
diagnosis, 781
management, 781-782
oveiview, 779
pathogenesis, 779
Sneddon syndiome
epidemiology, 376
management, 376
oveiview, 376
Soft tissue infections (STIs)
classification and definitions, 609
eiysipelas and cellulitis
diagnosis, 616
oveiview, 609
pathogenesis, 611
lymphangitis
diagnosis, 620
management, 620
oveiview, 619
neciotizing soft tissue infections
clinical vaiiants, 618, 619[
management, 619
oveiview, 618
oveiview, 609, 610
wound infections
classification of wounds, 621
diagnosis, 624
management, 624
oveiview, 621
pathogenesis, 622
Solai keiatosis, 267, 267[, 269[, 275, 275[
Solai lentigo
management, 266
oveiview, 264
Solai uiticaiia, 250, 251[, 251
Spidei angioma, 202, 203[
Spoiotiichosis
diagnosis, 746
IN0X 1109
Spoiotiichosis (ConnueJ)
management, 746
oveiview, 744
pathogenesis, 744
Spieading pigmented actinic keiatosis (SPAK), 270
Squamous cell caicinoma, 472[
invasive SCC of cutaneous anus, 1064
invasive SCC of penis, 1064
invasive SCC of vulva, 1064
nail appaiatus and, 1012, 1013[
iadiation-induced, 273[
Squamous cell caicinoma in situ, 274, 1062, 1062[-1063[
etiology, 278
management, 279
oveiview, 276
Staphylococcal scalded-skin syndiome (SSSS)
diagnosis, 628
management, 628
oveiview, 626
Staphylococcal toxins, 625
Sa|y|otottus aureus infections and
intoxications, 591
in HIV/AIDS, 956, 956[-957[
Stasis deimatitis, 468, 468[
Stevens-Johnson syndiome and toxic epideimal
neciolysis (TEN)
definition, 173-174
epidemiology, 174
geneial findings, 175
management, 176-177
oveiview 173
sequelae, 176
STIs. See Soft tissue infections (STIs)
Stiawbeiiy tongue, 632, 633[
Stieptococcal toxins, 625
Sreotottus yogenes gioup A Sreotottus (GAS)]
infections and intoxications, 591
Stiiae distensae, 420, 421[
Sunlight, skin ieactions to
acute skin damage (sunbuin)
epidemiology, 235
management, 236
oveiview, 235
pathogenesis, 235
oveiview, 232, 233[, 234, 234
Supeiantigens, 625-626
Supeificial spieading melanoma
couise and piognosis, 314-315, 318
diagnosis, 318
epidemiology, 315
management, 318
oveiview, 315
Sutton leukodeima acquisitum centiifugum
Sweet syndiome
clinical manifestations, 160, 161[ , 162
management, 162
oveiview, 160
Swimmei`s itch, 880
Swimming pool gianuloma, 678
Syndiomic ichthyoses, 84
Syphilis
in HIV/AIDS, 960
latent syphilis
congenital syphilis, 931
oveiview, 928
teitiaiy/late syphilis
couise, 930
diagnosis, 930
management, 924
oveiview, 919, 920[, 920
pathogenesis, 921
piimaiy syphilis
diagnosis, 924
secondaiy syphilis
clinical manifestations, 924, 926, 926[-927[,
928, 929[
couise, 928
diagnosis, 928
Syiingoma, 220, 221[
Systemic amyloidosis
oveiview, 354
systemic AA amyloidosis, 356
systemic AL amyloidosis
diagnosis, 355
oveiview, 354
Systemic canceis. See Canceis, systemic, skin signs of
Systemic paiasitic infections. See Paiasitic infections,
systemic
T
Tache noiie, 768, 768[
Tanapox, 782-783
Telangiectasia maculaiis eiuptiva peistans, 523[
Telogen effluvium
IN0X 1110
Telogen effluvium (ConnueJ)
diagnosis, 977
management, 977
oveiview, 975
pathogenesis, 976
Tempoial aiteiitis, 405[
Tendinous xanthoma, 436
TEN (toxic epideimal neciolysis). See Stevens-
Johnson syndiome and toxic epideimal neciolysis
(TEN)
Tetanus, 637
Tetiacycline fixed diug eiuption, 567[
Thiomboangiitis obliteians, 462, 462[
Thiombocytopenic puipuia
diagnosis, 506
epidemiology, 504
management, 506
oveiview, 504
Thiombophlebitis and deep venous thiombosis
management, 464
oveiview, 462, 463
Thiush, 724, 725[
Tick-boine typhus
diagnosis, 767
management, 767
oveiview, 765
Tinea baibae
management, 716
Tinea capitis
couise, 715
management, 714
oveiview, 709
pathogenesis, 710
Tinea coipoiis
management, 705
oveiview, 704
Tinea ciuiis
management, 704
oveiview, 703
Tinea facialis
management, 707
oveiview, 707
Tinea incognito, 704[-706[, 708
Tinea manuum
couise, 703
management, 703
oveiview, 701
Tinea nigia, 736, 737[
Tinea pedis
diagnosis, 700
epidemiology, 697
oveiview, 697
Tinea unguium/onychomycosis
diagnosis, 1019
management, 1020-1021, 1020
oveiview, 1016-1017, 1017[, 1019[
pathogenesis, 1018
Tinea veisicoloi, 732
Tongue disoideis
black oi white haiiy tongue, 1029
fissuied tongue, 1028, 1029[
migiatoiy glossitis, 1030, 1030[
oial haiiy leukoplakia, 1029
Toiulosis, 747
Toxic epideimal neciolysis (TEN). See Stevens-
Johnson syndiome and toxic epideimal neciolysis
(TEN)
Toxic shock syndiome
diagnosis, 630
management, 631
oveiview, 629
pathogenesis, 629
Tianchonychia, 1009[
Tiansplantation, skin diseases in. See Oigan and bone
maiiow tiansplantation, skin diseases in
Tiansveise oi Beau lines, 1021, 1022[
Tiavel" deimatology, 1072
Tiench mouth, 1032
Tiichilemmal cyst, 212, 212[
Tiichoepithelioma, 220, 220[-221[
IN0X 1111
Tiichomycosis, 595
Trt|osoron infections, 736
Tiichotillomania, 583, 583[-585[
Tiipe palm, 503[
Tiypanosomiasis
Ameiican tiypanosomiasis, 893, 893[
human Afiican tiypanosomiasis, 894
oveiview, 893
Tubeious scleiosis
associated systems, 450
diagnosis, 452
epidemiology, 449
management, 452
oveiview, 449
pathogenesis, 450
Tubeious xanthoma, 436
Tulaiemia
diagnosis, 662
management, 662
oveiview, 659-660
pathogenesis, 660
U
Uiticaiia and angioedema
clinical types, 358, 360[-361[
diagnosis, 362, 364-365, 364[
management, 365
oveiview, 358, 359[-360[
special featuies as ielated to pathogenesis, 358,
360, 361[, 362, 363[, 365[
Uiticaiial vasculitis
management, 408
oveiview, 407
pathogenesis, 407
Uiticaiia pigmentosa, 522[
V
Vaccinia
diagnosis, 786
management, 786-787
oveiview, 783
pathogenesis, 784
vaccination, 784
Vaiicella zostei viius infections
epidemtiology and etiology, 831
heipes zostei (HZ)
840, 845[
diagnosis, 843
epidemiology, 837, 838[
management, 844
oveiview, 837
in the immunocompiomised host
clinical manifestations, 846-847, 847[, 849[, 958
epidemiology, 846
management, 848
oveiview, 846
oveiview, 831
pathogenesis, 832
vaiicella
diagnosis, 834
epidemiology, 833
management, 836
oveiview, 833
Vaiicose veins, 465-468, 467[, 470
Vaiiegate poiphyiia
epidemiology, 257
management, 258
oveiview, 257
Vaiiola, 779
Vasculai hamaitomas, 209
Vasculai insufficiency, skin signs of
atheioscleiosis, aiteiial insufficiency, and
atheioembolization
epidemiology, 458
management, 462
oveiview, 458
chionic lymphatic insufficiency, 476, 476[
chionic venous insufficiency
clinical manifestations, 466-467, 466, 467[-469[
diagnosis, 468
oveiview, 465
leg and foot ulceis, most common
aiteiial ulceis, 470-471, 473f
combined aiteiial and venous ulceis, 471, 473[
IN0X 1112
Vasculai insufficiency, skin signs of (ConnueJ)
leg and foot ulceis, most common (ConnueJ)
diffeiential diagnosis, 471, 472, 474
management, 474
neuiopathic ulceis, 471
oveiview, 470
venous ulceis, 470, 471[-472[
livedoid vasculitis, 475, 475[
piessuie ulceis
epidemiology, 477
management, 478
oveiview, 477
thiomboangiitis obliteians, 462, 462[
thiombophlebitis and deep venous thiombosis
management, 464
oveiview, 462, 463
Vasculai malfoimations
capillaiy malfoimations
angiokeiatoma, 206, 206[-207[
cheiiy angioma, 205, 205[
poit-wine stain
histopathology, 201
management, 202
oveiview, 201, 201[-202[
syndiomic, 202
spidei angioma, 202, 203[
venous lake, 204, 204[
capillaiy/venous malfoimations, 209-210, 209[
blue iubbei bleb nevus, 210, 210[
vasculai hamaitomas, 209
lymphatic malfoimation
lymphangioma, 208, 208[
oveiview, 192, 192-193, 201
Vasculai tumois
angiosaicoma, 200, 200[
glomus tumoi, 199, 199[
hemangioma of infancy
diagnosis, 194
epidemiology, 193
management, 194
oveiview, 192, 192-193
pyogenic gianuloma, 198, 198[
Vasculitis
giant cell aiteiitis
management, 406
oveiview, 405
pathogenesis, 406
hypeisensitivity vasculitis
management, 398
oveiview, 397
pathogenesis, 397
nodulai vasculitis
management, 409
oveiview, 408
polyaiteiitis nodosa
management, 402
oveiview, 400
pathogenesis, 400
Schnlein-Henoch puipuia, 399, 400[
uiticaiial vasculitis
management, 408
oveiview, 407
pathogenesis, 407
Wegenei gianulomatosis
management, 404
oveiview, 402
pathogenesis, 402
physical examination, 402, 403[, 404, 405[
Venous insufficiency, chionic
clinical manifestations, 466-467, 466,
467[-469[
diagnosis, 468
oveiview, 465
Venous lake, 204, 204[
Veiiuca, 788-790, 789[-792[
Veiiuca seboiihoica, 215
Viial infections of skin and mucosa
dengue fevei, dengue hemoiihagic fevei, dengue
shock syndiome
diagnosis, 812
IN0X 1113
Viial infections of skin and mucosa (ConnueJ)
dengue fevei, dengue hemoiihagic fevei, dengue
shock syndiome (ConnueJ)
management, 812
oveiview, 810
enteioviial infections
hand-foot-and-mouth disease
diagnosis, 804
management, 804
oveiview, 803
pathogenesis, 803
oveiview, 803
eiythema infectiosum
diagnosis, 808
management, 808
oveiview, 806
pathogenesis, 806
Gianotti-Ciosti syndiome, 809, 809[
heipangina, 804
human heipesviiuses. see Heipesviiuses
human papillomaviius infections
cutaneous infections
793
diagnosis, 794
management, 794
oveiview, 788
etiology, 787
mucosal infections. See Sexually tiansmitted
infections
oveiview, 787
infectious exanthems
diagnosis, 797
management, 797
measles
diagnosis, 802
management, 802
oveiview, 800
pathogenesis, 800
oveiview, 795
pathogenesis, 795
iubella
diagnosis, 798
management, 798
oveiview, 798
oveiview, 770
poxviius infections. See Poxviius infections
vaiicella zostei viius infections. See Vaiicella zostei
viius infections
Vitiligo, 1052, 1052[
diagnosis, 336
epidemiology, 335
management, 338, 340, 341[
oveiview, 335
pathogenesis, 335
von Recklinghausen disease, 453
W
Watei-associated infections and infestations
ceicaiial deimatitis, 880, 881[
envenomations caused by Cnidaiia (jellyfish,
Poituguese man-of-wai, sea anemones, coials),
882, 883[
injuiies caused by echinodeims (sea uichins, sea
stais, and sea cucumbeis), 882
oveiview, 879, 879
seabathei`s eiuption, 880, 881[
Wegenei gianulomatosis
management, 404
oveiview, 402
pathogenesis, 402
physical examination, 402, 403[, 404, 405[
Wen, 211-212
Whitlow, heipetic, 820, 821[
Wickham stiiae, 1031, 1031[
Woiingei-Kolopp disease, 530
Wound infections
classification of wounds, 621
diagnosis, 624
management, 624
oveiview, 621
pathogenesis, 622
X
Xanthelasma, 436, 437[
Xanthomas, 434, 435
Xanthoma stiiatum palmaie, 438, 439[
Xanthoma tendineum, 436, 437[
IN0X 1114
Xanthoma tubeiosum, 436, 437[
X-linked ichthyosis (XLI)
diagnosis, 76
epidemiology, 75
management, 76
oveiview, 75
Y
Yeast infection, 727
Yellow nail syndiome, 1022, 1023[
Z
Zinc deficiency and aciodeimatitis enteiopathica, 442,
443[-444[
Zoon balanitis, 1048

Atlas and Synopsis of Clinical Dermatology

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C0NINIS

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