[Risk of liver failure in erythropoietic protoporphyria. Be alert for sings of cholestatic development!]. / Risk för leversvikt vid erytropoetisk protoporfyri. Var uppmärksam på kolestatisk utveckling!

ABSTRACT

Erythropoietic protoporphyria (EPP) presents clinically as a painful skin reaction to sun-light exposure. The profoundly disabling psychosocial consequences of the disease often go unnoticed by the physician, and the need to monitor the patient for hepatic complications is not generally recognised. The article describes the clinical and biochemical course in a 51-year-old man with EPP, who within a few days developed signs of acute hepatic failure. The case emphasises the importance of a well designed monitoring programme that allows close evaluation of the patient's current porphyrin metabolism, and indicates what measures should be considered.