Abstract

Porhyria cutanea tarda (PCT) is a rare, inherited or acquired disorder due to decreased activity or deficiency of uroporphyrinogen decarboxylase (UROD), one of the enzymes in the haem synthetic pathway. It is characterized by cutaneous manifestations such as erosions, blisters and bulae in the dorsum of the hand, forearm, elbows and knees; and painful indolent sores that heal with dyspigmented and scarring lesions. A 25 year old sales man presented with a 7 month history of recurrent blistering of the skin of the extremities- hands, elbows, knees and feet which occurred spontaneously or following trivial trauma. There was no family history of similar skin symptoms. Examination showed broken and fresh blisters of varying sizes with some healed lesions on the dorsum of the hands, over the elbow and knee joints, and toes. Serum ferritin was 360μ/L (40- 340 μ/L), urine uroporphyrinogen was positive (+++) and Hepatitis C antibodies screening was positive. Some improvement of the cutaneous lesion was noticed following commencement of therapeutic phlebotomy. Keywords: Porhyria, blisters, Hepatitis C virus, uroporphyrinogen

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