摘要
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.
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.
