HIV-Associated Generalized Papular Mucinosis and Neurosyphilis

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The association of cutaneous mucinosis with other conditions is frequent. While the rare coexistence of papular mucinosis with HIV infection has already been documented, any relation of the disease to syphilitic infection has not been proved yet. We present on a patient with generalized papular mucinosis, HIV infection and neurosyphilis.

Cutaneous mucinoses are characterized by excessive dermal deposition of acid glycosaminoglycans. Dubreuilh was the first to describe lichen myxoedematosus in 1906, and it was in 1953 when Montgomery and Underwood classified the various clinical forms. Association of cutaneous mucinoses with other conditions, such as paraproteinemia, diabetes mellitus, thyroid dysfunction, hepatitis C and HIV-infection, was found frequently. No definite reference about relation to syphilis infection has been published yet. Etiologic factors of dermal mucin deposition have not been clarified, it is assumed that abnormal activation of mucinproducing dermal fibroblasts by autoantibodies, paraproteins and cytokines, or defective pathways of mucin degradation could be involved. Here we present an uncommon form of papular mucinosis (PM) associated with HIV infection and neurosyphilis.

A 30-year-old, otherwise healthy man presented with widespread skin symptoms of about 12 month’s duration without any subjective complaints. Widespread 3-5 mm, skin coloured papules suggested scleromyxoedema. Skin biopsy revealed alcian blue and PAS positive mucin deposition in the papillary dermis, perivascular and perifollicular inflammatory infiltrate and discrete epidermal vacuolization. Laboratory examination showed elevated liver enzymes and polyclonal hypergammaglobulinaemia, ANA antibodies were negative. The patient refused the offered thorough examination. After a few months he was hospitalised at an ophthalmologic department because of bilateral visual impairment. Panuveitis was diagnosed on the basis of abnormal perimetry and funduscopy findings. TPPA, TP-ELISA and RPR tests were positive, the latter in 1/512 dilution. Anti-HIV ELISA was reactive. On admission the patient showed widespread indurated, skincoloured papules 2-5 mm in diameter, diffuse alopecia, bilat eral inguinal and submandibular lymphadenopathy and he complained of dizziness and headache. No genital symptoms were present.