[Liver cirrhosis in protoporphyria: bile acid therapy and liver transplantation]

Protoporphyria is an important differential diagnosis in patients with jaundice and cutaneous photosensitivity. In 1975 erythropoietic protoporphyria and liver damage of unknown origin were diagnosed in a 24 year old patient with photosensibility since early childhood. Laparoscopic liver biopsy confirmed protoporphyrin-associated liver cirrhosis in 1983. Therapy with ursodeoxycholic acid was initiated and liver function was stable during an 18 month period. In december 1989 the patient developed severe intrahepatic cholestasis with rapid deterioration of liver function. Liver transplantation was performed in 1990. The patient is now five years after transplantation in an excellent clinical condition. This 20 year observation period gives insight in the pathogenesis of protoporphyrin-induced hepatobiliary complications in a latent and overt phase. The relevant diagnostic and prognostic porphyrin parameters and therapy of protoporphyric liver disease are discussed.