Porphyria cutanea tarda in human immunodeficiency virus-seropositive men: case report and literature review.
Porphyria cutanea tarda (PCT) is a disorder of heme synthesis characterized by (a) a diminished activity of uroporphyrinogen decarboxylase biochemically and (b) cutaneous lesions secondary to a delayed type of photosensitivity clinically. A human immunodeficiency virus (HIV)-infected patient with PCT is reported and the world literature is reviewed. To date, 17 HIV-seropositive men with PCT have been described. The initial appearance of PCT occurred before or concurrent with the diagnosis of HIV infection in 71% of these individuals (12 men). The median age at onset of PCT was 36 years (range of 20 to 69 years); the median age for the detection of HIV infection was 35 years (range of less than 20 to 71 years). All of these patients had elevated levels of urine porphyrins and blisters on their dorsal hands. Abnormal liver function tests, erosions, hyperpigmentation, hypertrichosis, and skin fragility were also present in some of the men. Polycythemia, serologic evidence of increased iron stores, scarring, milia, and sclerodermoid changes were rarely observed. Successful therapeutic approaches for PCT in men with HIV infection included (a) elimination of PCT-precipitating agents, (b) avoidance of sun exposure, and (c) periodic phlebotomy. Multiple hypotheses for an etiologic role of the HIV and/or an HIV-associated infection, directly or indirectly, in the pathogenesis of PCT in HIV-seropositive men have been suggested.
Cohen
P R
PR
Department of Dermatology, University of Texas Medical School, Houston 77030.
D000328:Adult; D006679:HIV Seropositivity / Q000150:complications*; D006801:Humans; D008297:Male; D011164:Porphyrias / Q000150:complications*; D012871:Skin Diseases / Q000150:complications*