Letter to the editor: Diagnosis of erythropoietic protoporphyria with severe liver injury - a case report.
Wensink D, Wagenmakers MA, Wilson JP, Langendonk JG
World journal of gastroenterology, 2019 08 14
Abstract
Erythropoietic protoporphyria (EPP) is an extremely rare disease which is often unrecognized as diagnosis. In the recent article Lui et al describe a patient with a new diagnosis of EPP with severe liver injury. Approximately 5%-20% of patients with EPP develop liver manifestations. The most severe complication of EPP is an hepatic crisis, which is a medical emergency requiring urgent treatment. Intensive treatment should consist of (exchange) transfusions and preferably in a center that performs liver transplantations.