[Porphyria cutanea tarda in a chronic hemodialysis patient]

Abstract

About 3 years after commencing hemodialysis for chronic renal failure, a 39-year-old man developed cutaneous lesions of the face and hands which were compatible with porphyria cutanea tarda (PCT) clinically and histologically. There was no evidence of familial PCT, excessive alcohol consumption, iron overload, chronic liver disease. It was impossible to measure urinary porphyrins because of anuria. Fecal and erythrocytic porphyrins were within normal limits. Plasma porphyrins, however, were markedly elevated, and in an assay using high performance liquid chromatography, uroporphyrin accounted for 72% and 7-carboxyl porphyrin for 24%. Review of past reports of PCT associated with hemodialysis revealed that the most characteristic feature was a significant increase of plasma porphyrins, mainly uroporphyrin. This increase is difficult to explain by accumulation due to failure of adequate clearance. Participation of factors affecting uroporphyrinogen decarboxylase activity seems likely.