PORPHYRIA CUTANEA-TARDA - CLINICAL-FEATURES AND LABORATORY FINDINGS IN 40 PATIENTS

Clinical and laboratory findings in 40 patients with porphyria cutanea tarda observed for periods ranging from four months to seven years are presented. These data indicate alterations in the previously established typical patient profile as well as in the factors associated with the development of porphyria cutanea tarda. Earlier age at onset, diminution of alcohol ingestion as the major etiologic factor and an increased incidence in females suggest new environmental influences. Aside from ethanol, use of estrogens for oral contraception, for postmenopausal symptoms or for the treatment of prostatic carcinoma was the most frequently associated etiologic factor. Cutaneous features in these patients included bullae (85 per cent), increased skin fragility (75 per cent), facial hypertrichosis (63 per cent), hyperpigmentation (55 per cent), sclerodermoid changes (18 per cent) and dystrophic calcification with ulceration (8 per cent). Associated findings included diabetes mellitus (15 per cent), systemic lupus erythematosus (5 per cent), elevated serum iron level (62 per cent) and abnormal liver function test results (60 per cent). Liver biopsy specimens obtained from 34 patients all showed histologic abnormalities. The treatment of choice was phlebotomy. In 32 patients so treated, clinical remissions lasting an average of 30.9 months, were achieved. Ten patients (31 per cent) had a relapse, but second remissions were achieved with additional phlebotomies. Other therapeutic modalities utilized included chloroquine and plasmapheresis. © 1979.