Porphyria cutanea tarda in a chronic hemodialysis patient

• Published in: Saudi journal of kidney diseases and transplantation Vol. 21; no. 5; pp. 919 - 922
• Main Author: Labidi, Jannet
• Format: Journal Article
• Language: English
• Published: Riyadh, Saudi Arabia Saudi Center for Organ Transplantation 01-09-2010; Medknow Publications and Media Pvt. Ltd; Wolters Kluwer Medknow Publications
• Subjects: Adult; Biomarkers - blood; Biopsy; Care and treatment; Case studies; Complications and side effects; Deferoxamine; Deferoxamine - therapeutic use; Development and progression; Diseases; Dosage and administration; Female; Ferritins - blood; Fluorescent Antibody Technique; Hemodialysis; Hemoglobins - metabolism; Humans; Kidney Failure, Chronic - complications; Kidney Failure, Chronic - therapy; Kidneys; Patient outcomes; Patients; Porphyria; Porphyria Cutanea Tarda - diagnosis; Porphyria Cutanea Tarda - drug therapy; Porphyria Cutanea Tarda - etiology; Renal Dialysis - adverse effects; Severity of Illness Index; Siderophores - therapeutic use; Skin - drug effects; Skin - pathology; Time Factors; Treatment Outcome; الأمراض; الأمراض الجلدية; الفشل الكلوي; الكلى; المرضى; دراسات الحالة; علم المداواة; غسيل الكلى
• ISSN: 1319-2442; 2320-3838

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.