Erythropoietic protoporphyria and terminal hepatic failure.
Bruch-Gerharz D, Bolsen K, Gerharz CD, Goerz G
We report on a 44-year-old patient with erythropoietic protoporphyria who could effectively control his photosensitivity for 22 years with oral carotinoids. The clinical course of his disorder was complicated by liver involvement, initially expressed as marginally raised serum transaminase levels for several years. Terminal hepatic failure with fatal outcome developed 22 years after manifestation of his liver function abnormalities. Hepatic involvement represents an inconstant and unpredictable feature of erythropoietic protoporphyria, determining the prognosis of an otherwise clinically benign disorder.