Porphyria cutanea tarda and chronic renal failure.
Bullous dermatosis developed in a patient with renal failure who was on hemodialysis. The results of standard tests for porphyria cutanea tarda--quantitation of urinary and fecal porphyrins by fluorometric assay--were within normal limits or were nondiagnostic. Assay of plasma using high-pressure liquid chromatography, however, revealed that this patient had a porphyrin profile consistent with porphyria cutanea tarda. A porphyria-like bullous eruption may occur in patients with chronic renal failure; however, in only a few cases have laboratory studies confirmed the diagnosis of porphyria cutanea tarda. Our experience suggests that, in patients with renal failure, a plasma assay may be more reliable than tests of the urine and feces in establishing a diagnosis in those patients who actually have disorders of porphyrin metabolism. We also report the results of assay of erythrocyte uroporphyrinogen decarboxylase activity and fecal isocoproporphyrin.
D000328:Adult; D002851:Chromatography, High Pressure Liquid; D003306:Coproporphyrins / Q000378:metabolism; D006801:Humans; D007676:Kidney Failure, Chronic / Q000097:blood / Q000150:complications*; D008297:Male; D011164:Porphyrias / Q000097:blood / Q000175:diagnosis / Q000209:etiology*; D012872:Skin Diseases, Vesiculobullous / Q000209:etiology; D014575:Uroporphyrinogen Decarboxylase / Q000378:metabolism
D003306:Coproporphyrins; C005524:isocoproporphyrin; D014575:Uroporphyrinogen Decarboxylase