A case of erythropoietic protoporphyria.
- Author:
Ha Wook BONG
;
Joo Heung LEE
;
Yoo Shin LEE
;
Jung Ho KIM
- Publication Type:Original Article
- Keywords:
Erythropoietic Protoporphyria;
Free Erythrocyte Protoporphyrin
- MeSH:
Edema;
Erythema;
Hand;
Head;
Heme;
Humans;
Male;
Protoporphyria, Erythropoietic*;
Pruritus;
Sensation;
Skin;
Solar System;
Vomiting;
Young Adult
- From:Korean Journal of Dermatology
1993;31(5):817-822
- CountryRepublic of Korea
- Language:Korean
-
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.
