A Dialysis Case Presentation and Discussion
Edited by Roger A. Rodby: 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.

Cutaneous Manifestations of ESRD

“…Sclerodermatous plaques, hypertrichosis, and hyperpigmentation in sun-exposed areas are also seen in patients with PCT but not patients with pseudoporphyria. Increased serum levels of iron and ferritin, increased urinary uroporphyrin excretion, and increased levels of plasma uroporphyrin in anuric patients are typical in PCT (63,64). Treatment of PCT includes avoiding triggers, like alcohol, hepatotoxic medications, and sun exposure, and using sunscreen.…”

“…Porphyrins may form complexes with high-molecular weight proteins, which are poorly dialyzed by conventional methods. In hemodialysis (HD) patients, PCT occurs secondary to decreased URO-D activity and poor clearance of the plasma porphyrins (63,64). PCT is uncommon with peritoneal dialysis (PD), most probably because PD achieves more effective clearance of larger molecules (63).…”

“…Alcohol abuse, hepatitis C virus, HIV infection, and iron supplementation contribute to decreased URO-D activity (63). Porphyrins may form complexes with high-molecular weight proteins, which are poorly dialyzed by conventional methods.…”

“…Alternatively, deferoxamine can be used intravenously with each dialysis session in those patients unable to tolerate phlebotomy (63,64). High-flux membrane HD, which is more effective at removing plasma porphyrins, is an effective adjunct (63). Treatment of pseudoporphyria involves stopping/ avoiding any of the medications known to induce the disease (for example, diuretics, antibiotics, and antifungals) as well as sun avoidance and sun protection.…”

“…The combination of erythropoiesisstimulating agents with small volume phlebotomy (50-100 ml once or twice weekly) to decrease hepatic iron stores can induce remission within several months. Alternatively, deferoxamine can be used intravenously with each dialysis session in those patients unable to tolerate phlebotomy (63,64). High-flux membrane HD, which is more effective at removing plasma porphyrins, is an effective adjunct (63).…”