A case of erythropoietic protoporphyria with severe liver damage

Abstract

This report describes a patient with erythropoietic protoporphyria (EPP) and severe liver damage. A 42- year-old man who had been diagnosed with EPP at age 10 years was admitted to our hospital for an EPP storm, accompanied by stomachache, constipation and jaundice. Laxatives under light shielding showed no improvement in symptoms and his total bilirubin was elevated to 17.1 mg/d/. Five days after admission, he was administered injections of hemin, an iron-containing protein used to manage porphyria attacks, for a further 5 days. Subsequently, his total bilirubin declined and his stomachache was relieved. Three months after hemin treatment, his liver function and jaundice normalized.