Iron Therapy for Hepatic Dysfunction in Erythropoietic Protoporphyria

doi:10.1059/0003-4819-105-1-27

Iron Therapy for Hepatic Dysfunction in Erythropoietic Protoporphyria

Cleveland, Ohio

Abstract

Erythropoietic protoporphyria, caused by an inherited deficiency of ferrochelatase, results in increased free protoporphyrin concentrations in erythrocytes and plasma. Cutaneous photosensitivity is the primary clinical manifestation, but in some patients, protoporphyrin accumulation in the liver may lead to fatal hepatic failure. No treatment exists for this lethal complication. We report the case of a patient with erythropoietic protoporphyria and abnormal results of liver function tests in whom oral iron therapy led to a substantial decrease in free erythrocyte and stool protoporphyrin levels with return of liver function to normal. Iron therapy should be considered for preventing potentially fatal hepatic failure in patients with erythropoietic protoporphyria.

Article and Author Information

▸From the Department of Medicine, Cleveland Metropolitan General Hospital, Department of Dermatology, University Hospitals of Cleveland, and Department of Dermatology, Wade Park Veterans Administration Hospital, Case Western Reserve University; Cleveland, Ohio.
Grant support: in part by grant FD-R-000069-01-0 from the Food and Drug Administration and grants ES-1900 and RR-00080 from the National Institutes of Health.
▸Requests for reprints should be addressed to Gary M. Brittenham, M.D.; Cleveland Metropolitan General Hospital, 3395 Scranton Road; Cleveland, OH 44109.