Sclerodermoid changes of porphyria cutanea tarda: possible relationship to urinary uroporphyrin levels.
From 1950 to 1982, fifteen patients were seen at the Mayo Clinic with a diagnosis of sclerodermoid changes of porphyria cutanea tarda. Fourteen patients had changes similar to scleroderma limited to the skin, and one patient had scleroderma-like skin changes accompanied by visceral abnormalities. Both light-exposed and unexposed areas of the body were affected. Areas of involvement included the chest, the V-shaped area of the neck, and the back, face, and shoulders. In six patients, morpheaform changes represented the presenting cutaneous sign of porphyria cutanea tarda. Follow-up examination, after treatment that included abstinence from alcohol and phlebotomy, revealed that the sclerodermoid skin changes had disappeared in six patients and improved in four. Generally, the degree of improvement of the sclerodermoid changes was proportional to the reduction of the urinary uroporphyrin levels toward normal (p = 0.02).
D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011164:Porphyrias / Q000473:pathology* / Q000652:urine; D011166:Porphyrins / Q000652:urine*; D012594:Scleroderma, Localized / Q000473:pathology; D012867:Skin / Q000473:pathology*; D012871:Skin Diseases / Q000473:pathology* / Q000652:urine*; D014578:Uroporphyrins / Q000652:urine*
D011166:Porphyrins; D014578:Uroporphyrins