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.

Additional Metadata
Keywords Erythropoietic protoporphyria, Liver disease, Blood transfusion, Protoporphyrin IX, Treatment
Persistent URL dx.doi.org/10.3748/wjg.v25.i30.4292, hdl.handle.net/1765/121079
Journal World Journal of Gastroenterology
Citation
Wensink, D., Wagenmakers, M., Wilson, J.H.P, & Langendonk, J.G. (2019). Letter to the editor: Diagnosis of erythropoietic protoporphyria with severe liver injury - a case report. World Journal of Gastroenterology, 25(30), 4292–4293. doi:10.3748/wjg.v25.i30.4292