Porphyria cutanea tarda in a chronic hemodialysis patient

Abstract EN

End-stage renal failure and long-term hemodialysis (HD) treatment promote the development of genetically conditioned porphyria cutanea tarda (PCT).

Iron overload is often associated with this disease and is thought to play a role in its pathogenesis.

We report a case of HDrelated 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 ?g/L (Nl: 8-120 ?g/L) and negative serology for HIV, HBV and HCV.

Porphyrin analyses showed a PCT pattern.

Skin biopsy findings and direct immunofluorescence 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.