Porphyria cutanea tarda in a chronic hemodialysis patient

Abstract

End-stage renal failure and long-term hemodialysis (HD) treatment promote the development of genetically conditioned porphyria cutanea tarda (PCT). Iron overload is often asso-ciated with this disease and is thought to play a role in its pathogenesis. We report a case of HD-related PCT, which improved with deferoxamine treatment. A 33-year-old woman, with end-stage renal failure on HD since 1998, presented with a history of blisters on the face and dorsum of the hands, of several months duration. Laboratory analysis showed: hemoglobin 10.4 g/dL; a moderate hepatic cytolysis; ferritin 1300 μg/L (Nl: 8-120 μg/L) and negative serology for HIV, HBV and HCV. Porphyrin analyses showed a PCT pattern. Skin biopsy findings and direct immunofluo-rescence were consistent with PCT. The patient received deferoxamine (40 mg/kg intravenously every week for 6 weeks) which led to dramatic improvement of the symptoms. Several treatments are proposed in the management of dialysis-related PCT. This case confirms that deferoxamine can induce rapid and prolonged remission.