Erythoropietic protoporphyria, sometimes also called erythrohepatic protoporphyria or simple protoporphyria, is a heritable defect of heme synthesis in which the last enzyme of the heme synthetic pathway, ferrochelatase(or heme synthetase), is
functioning suboptimally.
A 23-year-old male has experienced erythema and edema on the face 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 by headache and vomiting. We have seen a case of erythropoietic protoporphyria presenting clinically and histopathologically, with a skin lesion on the sun exposured area, and free erthrocyte protoporphyrin serologically
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