Porphyria cutanea tarda in a chronic hemodialysis patient.
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 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 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.
Labidi
Jannet
J
Department of Internal Medicine, Hospital Militaire Principal d'instruction, Tunis, Tunisia. labidijannet@yahoo.fr
D000328:Adult; D015415:Biomarkers / Q000097:blood; D001706:Biopsy; D003676:Deferoxamine / Q000627:therapeutic use; D005260:Female; D005293:Ferritins / Q000097:blood; D005455:Fluorescent Antibody Technique; D006454:Hemoglobins / Q000378:metabolism; D006801:Humans; D007676:Kidney Failure, Chronic / Q000150:complications / Q000628:therapy*; D017119:Porphyria Cutanea Tarda / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D006435:Renal Dialysis / Q000009:adverse effects; D012720:Severity of Illness Index; D017262:Siderophores / Q000627:therapeutic use; D012867:Skin / Q000187:drug effects / Q000473:pathology; D013997:Time Factors; D016896:Treatment Outcome
D015415:Biomarkers; D006454:Hemoglobins; D017262:Siderophores; D005293:Ferritins; D003676:Deferoxamine