Bullous skin lesions in a patient undergoing chronic hemodialysis.

Abstract:

:Porphyria cutanea tarda (PCT) is a vesiculobullous skin disorder characterized by a defect in heme biosynthesis. Reduced activity of the hepatic enzyme uroporphyrinogen decarboxylase (URO-D) results in accumulation of photosensitive porphyrins; this ultimately leads to the skin fragility and blistering that is characteristic of this disease. The majority of cases of PCT are associated with acquired deficiencies of the enzyme URO-D, secondary to hepatic injury precipitated by medications or infections. Less commonly, PCT has been documented in patients with end-stage renal disease. The pathogenesis of PCT in long-term hemodialysis (HD) has been attributed to many factors, but the following mechanisms have been implicated: (i) decreased hepatic URO-D activity due to suppressive effects of iron and other hepatotoxins and (ii) poor porphyrin clearance by renal replacement therapies. We report a case of PCT that developed in a patient on maintenance HD for 4 years. He had a history of hepatitis C and evidence of iron overload. However, as the patient was anemic, therapeutic phlebotomy was problematic and therefore erythrocyte-stimulating agents were maximized to mobilize iron stores and allow phlebotomy. With this treatment, the patient's skin lesions improved in conjunction with decreasing ferritin levels.

journal_name

Semin Dial

journal_title

Seminars in dialysis

authors

Ryali ME,Whittier WL

doi

10.1111/j.1525-139X.2009.00672.x

subject

Has Abstract

pub_date

2010-01-01 00:00:00

pages

83-7

issue

1

eissn

0894-0959

issn

1525-139X

pii

SDI672

journal_volume

23

pub_type

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