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 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.

• Authors: Jannet Labidi
• Journal: Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi Arabia (Saudi J Kidney Dis Transpl) Vol. 21 Issue 5 Pg. 919-22 (Sep 2010) ISSN: 1319-2442 [Print] Saudi Arabia
• PMID: 20814132 (Publication Type: Case Reports, Journal Article)

Chemical References

• Biomarkers
• Hemoglobins
• Siderophores
• Ferritins
• Deferoxamine

Topics

• Adult
• Biomarkers (blood)
• Biopsy
• Deferoxamine (therapeutic use)
• Female
• Ferritins (blood)
• Fluorescent Antibody Technique
• Hemoglobins (metabolism)
• Humans
• Kidney Failure, Chronic (complications, therapy)
• Porphyria Cutanea Tarda (diagnosis, drug therapy, etiology)
• Renal Dialysis (adverse effects)
• Severity of Illness Index
• Siderophores (therapeutic use)
• Skin (drug effects, pathology)
• Time Factors
• Treatment Outcome