Dermatology Quiz and Case Discussion

From The Child's Doctor, Fall 2004

Anthony J. Mancini, MD Head, Dermatology, Ann & Robert H. Lurie Children's Hospital of Chicago; Professor of Pediatrics and Dermatology, Northwestern University Feinberg School of Medicine
Disclosure: Dr. Mancini has no industry relationships to disclose and does not refer to products that are still investigational or not labeled for the use in discussion.

Daniela Russi, MD Fellow, Pediatric Dermatology, Children's Memorial Hospital

Disclosure: Dr. Russi has no industry relationships to disclose and does not refer to products that are still investigational or not labeled for the use in discussion. Other Disclosure Information

Educational objectives
At the conclusion of this activity, participants will be able to:

Recognize the exanthematous disorder presented in the photographs Describe the epidemiologic concerns associated with the causative agent Evaluate the associated findings and relate the course of the disease

CME credit
Credit statement

A 22-month-old male presented to the dermatology clinic for evaluation of a rash involving both hands. The rash was pruritic and gradually progressing. He was originally treated with hydrocortisone 1% cream and oral diphenhydramine, without improvement. Review of systems was notable for upper respiratory symptoms 3 weeks prior, and negative for fever or gastrointestinal symptoms. Physical examination revealed partially blanchable, erythematous-to-purpuric patches without any truly palpable purpura, involving both palms (Figure 1). His oral mucosa revealed a single superficial ulceration of his right posterior palate. His feet were clear, as was the remainder of his cutaneous examination, and he had no lymphadenopathy.

One week later, a 9-month-old male was referred to the dermatology clinic for a rash involving his hands and feet. His skin eruption was associated with pruritus, and he had had low grade fevers and upper respiratory symptoms for 2 days. Physical examination revealed petechial, erythematous patches of the palms and soles (Figure 2). The remainder of his physical examination was unremarkable, including a normal oral mucosal examination and the absence of lymphadenopathy.

What is the most likely diagnosis in both patients?

a. Palmoplantar hidradenitis b. Hand-foot-and-mouth disease c. Papular-purpuric gloves and socks syndrome d. Rocky Mountain spotted fever Answer: C

Discussion:
Papular-purpuric gloves and socks syndrome (PPGSS) is an acute, self-limited condition initially described in 1990. The characteristic features include purpuric erythema involving the hands and feet (especially palms and soles) in a glove and stocking distribution. It is occasionally associated with

fever and oral lesions. Serological studies have revealed that the vast majority of patients with PPGSS have IgM antibodies to parvovirus B19 (B19). Other infectious agents that have been proposed in association with PPGSS include human herpesvirus 6 (HHV6), human herpesvirus 7 (HHV7), measles virus and cytomegalovirus. PPGSS occurs most often during the spring and summer months. The incubation period in PPGSS is around 10 days. Skin manifestations are quite characteristic, and begin with edema and erythema symmetrically localized to the hands and feet. Subsequently, petechial and purpuric changes appear, and vary from a few millimeters in diameter to larger, confluent patches. Pruritus, tingling, and pain may be reported by the patient. Polymorphous lesions (usually small erosions) of the oral cavity may be present, affecting the hard and soft palate, pharynx, tongue, and inner aspects of the lips. Other occasionally reported signs and symptoms include fatigue , headache, anorexia, arthralgias and lymphadenopathy. Low grade fever is common, often developing 2 to 4 days following the onset of the rash, and usually not exceeding 38.5 degrees Celsius. Laboratory findings in patients with PPGSS are variable, and depend upon the specific etiologic agent. Hematologic findings may include mild and transient abnormalities, including anemia, neutropenia, eosinophilia, monocytosis and thrombocytopenia. In addition, elevation of hepatic transaminases, C-reactive protein, and erythrocyte sedimentation rate have been observed. Skin biopsies, which are rarely necessary, have revealed nonspecific histopathologic features, including lymphocytic perivascular infiltrates, edema, and erythrocyte extravasation within the papillary dermis. Direct immunofluorescence studies have shown deposits of immunoglobulin and C3 in a granular pattern within papillary dermal vessel walls, and B19 DNA has been demonstrated in skin biopsy specimens by polymerase chain reaction (PCR) analysis. These findings suggest that PPGSS may result from a direct viral effect, as well as an immune-mediated vascular response to infection. The timing of the antibody response to B19 in PPGSS appears to differ from that seen in erythema infectiosum (EI). Patients with PPGSS may develop mucocutaneous lesions during the period of viremia (and hence while still considered infectious), and subsequently develop a humoral immune response. In contrast, in patients with EI, development of the skin exanthem coincides with the appearance of antibody and disappearance of viremia. The course of PPGSS is one of spontaneous involution. Resolution of the exanthem occurs over 1 to 2 weeks, during which time desquamation may occur. No residual sequelae are usually found. Treatment is generally supportive, and includes cool fluids and analgesics for oral mucosal discomfort, if necessary. The affected skin surfaces can be treated with cool compresses and oral antihistamines in symptomatic patients. For Further Reading:
[1.] Aractingi S, Bakhos D, Flageul B, et al. Immunohistochemical and virological study of skin in the papular-purpuric gloves and socks syndrome. British Journal of Dermatology 1996;135:599-602. [2.] Larralde M, Enz PA, Sanchez Gomes A, Corbella MC. Papular-purpuric gloves and socks syndrome due to parvovirus B19 infection in childhood. Pediatr Dermatol 1998 Sep-Oct;15(5):413-414.

[3.] Messina MF, Ruggeri C, Rosano M, et al. Purpuric gloves and socks syndrome caused by parvovirus B19 infection. Pediatr Infect Dis J 2003 Aug;22(8):755-756.

[4.] Pavlovic MD. Papular-purpuric gloves and socks syndrome caused by parvovirus B19. Vojnosanit Pregl 2003 Mar-Apr;60(2):223-225.

[5.] Smith PT, Landry ML, Carey H, et al. Papular-purpuric gloves and socks syndrome associated with acute parvovirus B19 infection: case report and review. Clin Infect Dis 1998;27:164-168.

[6.] Vargas-Diez E, Buezo GF, Aragues M, et al. Papular-purpuric gloves-and-socks syndrome. Int J Dermatol 1996;35:626-632. [7.] Veraldi S, Rizzitelli G, Scarabelli G, et al. Papular-purpuric gloves and socks syndrome. Arch Dermatol 1996;132:975-977.

Accreditation Statement

The Northwestern University Feinberg School of Medicine is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians.
Credit Designation Statement

The Northwestern University Feinberg School of Medicine designates this enduring material for a maximum of 2 AMA PRA Category 1 Credit(s). Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Gloves and Socks Syndrome

copyright 1996 - 2013 DermIS

image description
diagnosis Gloves and Socks Syndrome localization back of the hands lesions erythema additional descriptions

http://www.dermis.net/dermisroot/en/12018/image.htm

Gloves and socks syndrome caused by parvovirus B19 infection

M Carlesimo1, E Palese1, E Mari1, V Panasiti1, A Picarelli2, A Rossi1, G Camplone1
Dermatology Online Journal 12 (6): 19

1. Department of Dermatology, University of Rome "La Sapienza" 2. Department of Clinical Sciences, University of Rome "La Sapienza"

Abstract
The papular purpuric gloves and socks syndrome (PPGSS) is an uncommon dermatosis with a typical purpuric exanthem limited to hands and feet; it occurs mainly in young adults. We report a case of a 19year-old man with an acute febrile illness accompanied by purpuric and papular lesions located mostly on the dorsal areas of his hands and feet. Serologic analysis for parvovirus B19 yielded positive results. The diagnosis of PPGSS was made. The eruption cleared without therapy in 12 days with plantar and palmar desquamation. Parvovirus B19 and some other viral infections have been proven to be causative agents of this syndrome.

Introduction The papular purpuric gloves and socks syndrome (PPGSS) is an uncommon dermatosis described for the first time by Harms et al. in 1990 [1]. They named this entity gloves and socks syndrome for the typical disposition of the exanthem. In 1991 Bagot and Revuz observed the association between PPGSS and infection by human parvovirus B19 [2], supporting the hypothesis of a viral etiology. Papular purpuric gloves and socks syndrome is clinically characterized by a monomorphic eruption of purpuric macules and papules that is accompanied by pruritic and painful edema involving the distal extremities in a peculiar gloves and socks distribution.

Some authors report symptoms and signs that occur during the course of this disease such as fever, asthenia, anorexia, arthralgia, lymphadenopathy, mucosal erosions, and perineuritis [3, 4, 5]. The exanthem is self-limited and clears within a period of 1 or 2 weeks [6]. Clinical synopsis In March 2004 a 19-year-old man was seen in our department for a 6day history of papular purpuric lesions located exclusively on the hands (Fig. 1) and feet with a sharp demarcation at the wrists and the ankles. Edema, itching, and pain were also present. The eruption was preceded by fever, abdominal pain, anorexia, and asthenia. Neither oral lesions nor lymphadenopathy were present. Laboratory studies were within normal limits with the exception of a mildly elevated white blood cell count, erythrocyte sedimentation rate at 22 mm/h, and protein C reactive at 2 mg/dl. Figure 1 Serologic markers for Epstein Barr virus, parvovirus, measles virus, cytomegalovirus, hepatitis B and C virus, Rickettsia, HIV, herpes Figure 1: Papular purpuric simplex virus 1 and 2 were tested with an indirect enzyme linked immunosorbent assay on the sixth day and on the eleventh day after lesions presented exclusively onset of the clinical eruption. The results were within normal limits with on the hands with a sharp the exception of assays for parvovirus B19 IgG and IgM confirming demarcation at the wrists acute infection in the second week. The diagnosis of acute parvovirus B19 infection was made on the basis of the presence of specific IgM antibodies in serum samples assayed at two different times during the patient's illness. Based on the clinical picture and the laboratory data the diagnosis of PPGSS was made. The eruption cleared after palmoplantar desquamation in 12 days. Discussion PPGSS is an acute self-limited acral dermatosis that affects children or young adults. Edema and erythema appear on the hands and feet in a "gloves and socks" distribution, followed by the appearance of pruritic or painful purpuric macules and papules on the same sites [3, 4]. Sometimes similar lesions may also be observed on other sites and a variety of oral or genital manifestation have been described [6, 7]. Systemic involvement that may precede or accompany skin manifestations include flu-like symptoms such as fever, asthenia, anorexia, arthralgia, lymphadenopathy, myalgia, respiratory or gastrointestinal symptoms [3, 7, 8]. Laboratory data are nonspecific with occasional leukopenia, neutropenia, eosinophilia, and mild elevation of transaminase levels and erythrocyte sedimentation rate [4, 8]. Histologic findings are non-specific and include necrotic keratinocytes, erythrocyte extravasion, and a superficial and mild-dermal perivascular lymphocytic infiltrate. The illness is self limited, lasting 1-2 weeks; usually the eruption clears with palmar and plantar desquamation. The viral etiology is well documented in the literature [9,10]. B19 is the most frequently reported etiologic agent of PPGSS, whereas occasional cases are associated with Coxsackie B virus, Epstein Barr virus,

Measles virus, Cytomegalovirus, Hepatitis B virus, and Herpes virus 6 infection. These data suggest that PPGSS could be a non- specific manifestation of a viral infection. The pathogenesis of this syndrome is still not completely understood. Parvovirus B19 is a member of the erythrovirus family and is the only parvovirus known to cause disease in humans. Infection with parvovirus B19 during pregnancy can cause several serious complications in the fetus, such as fetal anemia, neurological anomalies, hydrops fetalis, and fetal death because of viral inhibition of erythropoiesis and cytolysis of erythroid progenitor cells [11]. A receptor for Parvovirus B19 is present on bone marrow erythroid precursor cells, endothelial cells of dermal vessels, and keratinocytes. An immunohistochemical study with specific anti-B19V antibodies showed the presence of viral antigens on both epidermal and endothelial cells [12]. Therefore, the mucocutaneous lesions could be virus-mediated, through cytotoxicity, and not antibody-mediated. Cutaneous lesions generally develop on days 10 to 13 after infection [13-15]. In the literature there are some sero-negative cases, perhaps because testing for IgM antibodies was done during the first week of clinic eruption when sometimes antibodies are not yet detectable. Indeed, we perform this test during the second week of clinical eruption. Many clinical entities have been strongly associated with B19 infection such as erythema infectiosum, transient aplastic crisis, arthralgias, rheumatoid-like arthritis, spontaneous abortions, hydrops fetalis, and PPGSS. In a recent paper, however, the sera of 110 children with cutaneous eruptions and an acute systemic illness were analyzed to evaluate the presence of antibodies against parvovirus B19. Specific IgM antibodies to Parvovirus B19 were found in only 3 cases. This data indicates that Parvovirus B19 is not one of the most common causes of acute illness and rash in children [16]. We report this case because of the importance of recognizing Parvovirus B19 illness due to the occasional serious sequelae and because of the increased frequency of this presentation in young adults in recent years [17].

References
1. Harms M, Feldmann R, Saurat JH . Papular-purpuric "gloves and socks" syndrome. J Am Acad Dermatol 1990 Nov;23: 850-854. 2. Bagot M, Revuz J. Papular-purpuric "gloves and socks" syndrome: primary infection with parvovirus B19? J Am Acad Dermatol. 1991 Aug;25(2 Pt 1):341-2. 3. Borradori L, Cassinotti P, Perrenoud D, Frenk E. Papular-purpuric "gloves and socks" sindrome. Int J Dermatol. 1994 Mar;33(3):196-7. 4. Halasz CL, Cormier D, Den M. Petechial glove and sock sindrome caused by parvovirus B19. J Am Acad Dermatol. 1992 Nov;27(5 Pt 2):835-8. 5. Aguilar-Bernier M, Bassas-Vila J, Torne-Gutierrez J.I, Martinez-Garcia G, Aragoneses-Fraile H, Miranda-Romero A. Presence of perineuritis in a case of papuar purpuric gloves and socks syndrome associated with mononeuritis multiplex attributable to B19 parvovirus. J Am Acad Dermatol. 2006; 54:896-899. 6. Smith SB, Libow LF, Elston DM, Bernert RA, Warschaw KE Gloves and socks syndrome: early and late histopathologic features. J Am Acad Dermatol. 2002 Nov; 47(5):749-54.

7. Grilli R, Izquierdo MJ, Farina MC, Kutzner H, Gadea I, Martin L, Requena L. Papular-purpuric "gloves and socks" sindrome: polymerase chain react demonstration of parvovirus B19 DNA in cutaneous lesions and sera. J Am Acad Dermatol. 1999 Nov; 41(5 Pt):793-6. 8. Smith Pt, Landry ML, Carey H, Krasnoff J, Cooney E. Papular-purpuric gloves and socks syndrome associated with acute parvovirus B19 infection: case report and review. Clin Infect Dis. 1998 Jul;27(1):164-8. 9. Vargas-Diez E, Buezo GF, Arageus M, Dauden E, De Ory F. Papular-purpuric gloves-and-socks syndrome. Int J Dermatol. 1996 Aug;132(8):975-7. 10. Stone MS, Murph JR. Papular-purpuric gloves and socks syndrome: a characteristic viral exanthema. Pediatrics. 1993 Dec;92(6):864-5. 11. de Jong EP, de Haan TR, Kroes AC, Beersma MF, Oepkes D, Walther FJ. Parvovirus B19 infection in pregnancy. J Clin Virol. 2006 May;36(1):1-7. 12. Alfadey A, Alijubran A, Hainau B, Alhokail A. Papular-purpuric"gloves and socks" syndrome in a mother and daughter. J Am Acad Dermatol. 2003 Jun; 48(6):941-4. 13. Feldmann R, Harms M, Borradori L, Saurat JH. "Gloves and socks" papulo-purpuric syndrome. Ann Dermatol Venereol. 1993;120(11):810-2. 14. Aractingi S, Bakhos D, Flageul B, Verola O, Brunet M, Dubertret L, Morinet F. Immunohistochemical and virological study of skin in the popular-purpuric gloves and socks syndrome. Br J Dermatol. 1996 Oct;135(4):599-602. 15. Nelson JS, Stone MS. Update on selected viral exanthems. Curr Opin Pediatr. 2000 Aug;12(4):35964. 16. Pruksachatkunakorn C, Apichartpiyakul N, Kanjanaratanakorn K. Parvovirus B19 infection in children with acute illness and rash. Pediatric Dermatology. 2006; 23:216-218. 17. Hsieh MY, Huang PH. The juvenile variant of papular-purpuric gloves and socks syndrome and its association with viral infections. Br J Dermatol. 2004 Jul;151(1):201-6. 2006 Dermatology Online Journal

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Gloves and Socks Syndrome

copyright 1996 - 2013 DermIS

image description
diagnosis Gloves and Socks Syndrome localization feet lesions additional descriptions petechiae

patient information
http://www.dermis.net/dermisroot/en/12022/image.htm

copyright 1996 - 2013 DermIS

image description
diagnosis Gloves and Socks Syndrome localization feet lesions macula / patch additional descriptions petechiae

http://www.dermis.net/dermisroot/en/12025/image.htm

Gloves and Socks Syndrome

copyright 1996 - 2013 DermIS

image description
diagnosis Gloves and Socks Syndrome localization feet lesions macula / patch additional descriptions petechiae

http://www.dermis.net/dermisroot/en/12028/image.htm

Gloves and Socks Syndrome

copyright 1996 - 2013 DermIS

image description
diagnosis Gloves and Socks Syndrome localization palms lesions erythema additional descriptions

http://www.dermis.net/dermisroot/en/12011/image.htm

more information about this diagnose

Gloves and Socks Syndrome (7)

differential diagnoses
Measles (7)Erythema Infectiosum (10)Chemotherapy (0)

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Stevens-Johnson Syndrome

copyright 1996 - 2013 DermIS

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diagnosis Stevens-Johnson Syndrome Stevens-Johnson Syndrome localization palms palms lesions plaque vesicle additional descriptions

http://www.dermis.net/dermisroot/en/30351/image.htm

Stevens-Johnson Syndrome

copyright 1996 - 2013 DermIS

image description
diagnosis Stevens-Johnson Syndrome Stevens-Johnson Syndrome localization palms palms lesions plaque vesicle additional descriptions

http://www.dermis.net/dermisroot/en/30348/image.htm

Stevens-Johnson Syndrome

copyright 1996 - 2013 DermIS

image description
diagnosis Stevens-Johnson Syndrome Stevens-Johnson Syndrome localization lower arms lower arms lesions plaque vesicle additional descriptions

http://www.dermis.net/dermisroot/en/30345/image.htm

Stevens-Johnson Syndrome

copyright 1996 - 2013 DermIS

image description
diagnosis Stevens-Johnson Syndrome Stevens-Johnson Syndrome localization lower arms lower arms lesions plaque vesicle additional descriptions

http://www.dermis.net/dermisroot/en/30342/image.htm

Stevens-Johnson Syndrome

copyright 1996 - 2013 DermIS

image description
diagnosis Stevens-Johnson Syndrome Stevens-Johnson Syndrome Stevens-Johnson Syndrome localization lower arms lower arms lower arms lesions erythema plaque vesicle additional descriptions

http://www.dermis.net/dermisroot/en/30337/image.htm

Stevens-Johnson Syndrome

copyright 1996 - 2013 DermIS

image description
diagnosis Stevens-Johnson Syndrome Stevens-Johnson Syndrome localization lower arms lower arms lesions plaque vesicle additional descriptions

http://www.dermis.net/dermisroot/en/30333/image.htm

Stevens-Johnson Syndrome

copyright 1996 - 2013 DermIS

image description
diagnosis Stevens-Johnson Syndrome Stevens-Johnson Syndrome localization lips (skin) lips (skin) lesions erosion crust additional descriptions

http://www.dermis.net/dermisroot/en/30355/image.htm

Stevens-Johnson Syndrome

copyright 1996 - 2013 DermIS

image description
diagnosis Stevens-Johnson Syndrome localization lesions additional descriptions

http://www.dermis.net/dermisroot/en/30363/image.htm

Stevens-Johnson Syndrome

copyright 1996 - 2013 DermIS

image description
diagnosis Stevens-Johnson Syndrome Stevens-Johnson Syndrome localization lower arms lower arms lesions plaque vesicle additional descriptions

http://www.dermis.net/dermisroot/en/30329/image.htm

more information about this diagnose

Stevens-Johnson Syndrome (55)

differential diagnoses
Gingivostomatitis Herpetica (2)Gingivostomatitis Herpetica Gravis (0)Pemphigus, Paraneoplastic (0)Drug Eruption (43)Lyell Syndrome (20)Pemphigus Vulgaris (43)Pemphigus Mucosae (44)Bullous Pemphigoid (71)Linear IgA Dermatosis (3)Erythema Exsudativum Multiforme, Minor Form (12)Acute Cutaneous Graft-versus-Host Reaction (0)Lichen Planus of the Mucosa, Erosive (30)Lupus Erythematosus Mucosae Oris (8)Epidermolysis Bullosa Hereditaria (36)

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