Secondary hemosiderosis presented by porphyria cutanea tarda in akidney dialysis patient: A case report

摘要

A 68-year-old woman with chronic kidney disease receiving dialysis and ironsupplementation presented to our hospital with painful blisters, fragile skin,and changes to skin pigmentation on the dorsal side of both upper and lowerlimbs. Skin biopsy findings and an increase in urine porphyrins confirmed thediagnosis of porphyria cutanea tarda. Upon examination, extremely high serumferritin levels (6000 µg/L) suggested iron overload. Oral iron supplementationwas immediately discontinued, and the patient received treatment with the ironchelators deferoxamine, 10 mg/kg/day intravenously for 4 days, and deferasirox,540 mg/day orally. After a 4-month follow-up, ferritin levels were normal(97.7 µg/L) and the cutaneous manifestations of porphyria cutanea tarda hadimproved. Complete remission has been maintained for the last 2 years, and thepatient’s liver and heart function are normal. This case of porphyria cutaneatarda caused by secondary hemosiderosis highlights the potential toxicity ofiron accumulation as a result of excessive iron supplementation. Although notapproved for the treatment of patients on hemodialysis, we report the efficacyof deferasirox without any adverse effects in this case. We also stress theimportance of the close monitoring of serum iron levels in kidney dialysis—andindeed all iron-supplemented—patients to avoid potential hepatic, cardiac, andendocrine damage.