Pseudoporphyria or porphyria cutanea tarda? Diagnostic and treatment difficulties.
نویسندگان
چکیده
Fig. 1. Erosions on the forehead. Fig. 2. Erosions, milia and scarring on the dorsum of the hand. Sir, Porphyria cutanea tarda (PCT) is a vesiculobullous skin disorder of heme biosynthesis in which there is an enzyme defect of uroporphyrinogen decarboxylase (Uro-D), leading to accumulation of porphyrins mainly in urine but also in stool and plasma. Pseudoporphyria is clinically similar to PCT, but usually lacks the biochemical abnormalities, and is difficult to distinguish clinically and histologically. This case report shows how challenging differential diagnosis between PCT and pseudoporphyria can be, especially in the setting of renal failure and haemodialysis, and it emphasizes the treatment difficulties with renal failure patients and pseudoporphyria. A qualitative and quantitative examination of urine, plasma and stool porphyrins and Uro-D enzyme test in red blood cells is required.
منابع مشابه
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Pseudoporphyria is a rare bullous dermatosis that clinically and histopathologically is similar to porphyria cutanea tarda. It mainly affects patients with chronic renal failure on peritoneal dialysis or hemodialysis. Medications can also be involved in the etiology. Diagnosis and management of this condition is a challenge for dermatologists. The authors report a case of pseudoporphyria relate...
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ورودعنوان ژورنال:
- Acta dermato-venereologica
دوره 88 4 شماره
صفحات -
تاریخ انتشار 2008