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Topic 26. Cutaneous and mucosal manifestations and treatment of syphilis Syphilis Sexually transmitted disease Infection with the causative organism, Treponema pallidum, may be congenital or acquired through transfusion with contaminated blood, or by accidental inoculation. The most important route, however, is through sexual contact with an infected partner. Famous victims: Beethoven, Gaugin, Heine, Lenin, Napoleon, Hitler, Al Capone, Semmelweis Etiology - Treponema pallidum Motile, corkscrew-shaped, gram-negative bacterium 6-15 m in length, 0.15 m in width 6-14 coils Spirochaetales (coiled hair) Replication: with fission, every 30 hours Oral mucosa: non-pathogenic treponemes!!! Natural course of syphilis Contact (1/3 become infected) (10-90 days) Primary syphilis (syphilitic chancre) (3-12 wks) Secondary syphilis (mucocutaneous, organ) (4-12 weeks) Early latent syphilis (1 yr from contact) Late latent (more than 1 yr) Remission (2/3)

Relapsing (in 25%)

Tertiary syphilis (1/3) Late benign (16%) Cardiovascular (9.6%) Neurosyphilis (6.5%)

Natural course of syphilis Infection incubation period ~ 3 weeks 1 syphilis (syphilitic chancre) spontaneous regression in 2-6 weeks incubation 3-12 weeks 2 syphilis (maculopapular rash) spontaneous regression in 4-12 weeks Latent syphilis (asymptomatic stage, but positive serology) Relapsing syphilis (95% within 2 years) 3 syphilis (gummas, cardiovascular syphilis, neurosyphilis)

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Clinical manifestations Primary syphilis of contacts acquire the disease Incubation period: 10 to 90 days (average 3 weeks) Dusky red macule papule ulcer (chancre) - 1-2 cm, round or oval, firm, rubbery, painless; - Border: sharply defined, raised, indurated; - Base: ham-colored; - Serous exudate spirochetes Women: unilateral labia swelling (edema indurativum) Extragenital chancres Enlargeed, movable, nontender lymph nodes (buboes) Secondary syphilis - the great imitator 3-12 weeks after the appearance of the chancre Macular lesions (roseola syphilitica) - trunk and flexor aspects of extremities Maculopapular lesions transition to papular form (corona veneris) Papular eruptions (papulosquamous, follicular, lenticular, corymbose, nodular and annular) Pustular eruptions malignant syphilis Pigmentary changes (leucoderma colli syphiliticum or necklace of Venus) Mucous membrane lesions extremely infectious (condylomata lata, mucous patches, pharyngitis) Skin appendages (nail, hair) Other - Lymph node enlargement (buboes) - Syphilitic iritis - Hepatitis, glomerulonephritis - Anemia

Latent syphilis No clinical symptoms Reactive serological test Outcome is variable: 1. Persistent latency 2. Relapsing syphilis 3. Progression to tertiary syphilis Diagnosis of exclusion

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Relapsing syphilis 25% of untreated patients 90% within the first year 95% within the first 2 years Tertiary syphilis (~30% of untreated patients) Late benign syphilis 1. Granulomatous reaction to a few treponemes 2. Skin (70%): granulomatous nodules, psoriasiform granulomatous lesions, gummas (nontender pink to dusky red nodules) 3. Mucous membranes (10%): gummas, atrophic scars 4. Bones (10%) nocturnal pain, swelling: gummatous osteitis, periostitis, sclerosing osteitis Cardiovascular syphilis - Aortitis syphilitica (aneurysm!) Neurosyphilis 1. Tabes dorsalis 2. Atrophia n. optici 3. Pachimeningitis 4. General paresis Congenital syphilis (prenatal syphilis) Early prenatal syphilis (<2 years) 1. Rhinitis, perforated nasal septum, saddle nose 2. Bone abnormalities: osteochondritis 3. Mucocutaneous: macules, papules, condylomata lata, rhagades around mouth (Parrots lines) 4. Lymphadenopathy Late prenatal syphilis (>2 years) 1. Stigmata (Hutchinsons teeth, saddle nose, high arched palate) 2. Active disease (interstitial keratitis, retinitis, gummas, Neurosyphilis...) Diagnostic tests Microscopic tests dark field microscopy 1. Test of choice in chancres, condylomata lata, mucous patches 2. Not applicable in case of oral lesions 3. swimming treponemes Direct fluorescence antibody test 1. Exudate on glass slide 2. Fluorescence labeled anti -T. pallidum globulin Serology 1. Nontreponemal/nonspecific tests (cardiolipin, RPR, VDRL) 2. Treponemal/specific tests (FTA, ELISA, Western blots, PCR)

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Differential diagnosis: Chancre: chancroid (multiple and painful), herpes simplex, anal fissure, cervical erosions. Secondary syphilis: 1. Eruption - measles, rubella, drug eruptions, Pityriasis rosea, lichen planus, psoriasis 2. Condylomas - genital warts, haemorrhoids; Oral lesions - aphthous ulcers, candidiasis. Late syphilis: bromide and iodide reactions, other granulomas, erythema induratum Therapy 1. Penicillin 0.078 IU/ml, 15 MU in 15 days - 4 weeks later 15 MU again in 15 days 2. Tetracycline 4x500 mg/day 3. Erythromycin 4x500 mg/day

Jarisch-Herxheimer reaction inflammatory reaction caused by bacteria gradually disappearing and releasing toxins into the body faster than the body can handle with ease (first observed in patients with syphilis who received mercury treatment or antibiotics)