Erythropoietic Protoporphyria with Severe Cholestasis

Abstract

A 74-year-old woman with erythropoietic protoporphyria (EPP) with hepatic dysfunction is reported. She had been photosensitive for two years prior to admission. Physical examination revealed hepatosplenomegaly and erosions on her face. Moderate increases in serum bilirubin and biliary tract enzymes were noted. Histology of the biopsied liver revealed moderate fibrosis and dilatation of bile canaliculi containing orange pigment. Marked increases in protoporphyrin in erythrocytes and feces were noted. The patient died of hepatic failure a year and a half after admission with maximum serum bilirubin of 34 mg/dl. This patient seems to be the oldest among reported EPP cases with liver dysfunction. © 1994, The Japanese Society of Internal Medicine. All rights reserved.