Porphyria cutanea tarda in Ethiopia

Abstract

The clinical, biochemical and histological features of 75 Ethiopians with porphyria cutanea tarda (PCT) are described. PCT in Ethiopia is definitely related to alcohol abuse and there is no clinical evidence for hereditary predisposition. Significant elevation of transaminases and bromsulphthalein retention, moderate to marked hepatic siderosis and inflammation with little or no fibrosis, suggestive of mild or likely reversible parenchymal changes, characterize the liver affection in these patients. A similar study of 18 patients with hyperpigmentation of the face and hands but without blisters, an unexplained but common feature of liver disease in Ethiopia, revealed that serum iron and urinary uroporphyrin levels were normal in eight but significantly elevated in ten (56%). Neither elevated serum iron and hepatic siderosis nor increased urinary uroporphyrin completely explains the hyperpigmentation in this group of patients.