A case of erythropoietic protoporphyria

Bong, Ha Wook; Lee, Joo Heung; Lee, Yoo Shin; Kim, Jung Ho

Abstract

Erythropoietic protoporphyria, sometimes also called erythrohepitic protoporphyria or simple protoporphyria, is a heritable detect of heme synthesis in which the last enzyme of the heme synthetic pathway, ferrochelatase(or heme synthetase), is functioning subopt,imally. A 23-year-old male has experi nced erythema and edema on the fae and hands during or immediately after sun exposure, since 3 years of age. The skin lesions have been accompanied by severe itching, a buring sensation and pain. Severe episodes were followed hy head iche and vomiting. We have seen a case of erythropoietic protoporphyria presenting clinically and histo athologically, wit.h a skin lesion on the sun exposured area, and free erthrocyte protoporphyrin serologically.