ABSTRACT
Eythropoietic protoporphyria (EPP) is a rare genetic disease characterized by a defect in the enzyme ferrochelatase, which inserts iron into protoporphyrin to make heme. The genetic defect causes the accumulation of protoporphyrin in erythrocytes and plasma, resulting in painful photosensitization of any skin exposed to sunlight, and also in some cases, to strong artificial light. One of the functions of the carotenoid pigments present in all green plants and photosynthetic microorganisms is to protect these organisms against photosensitization by their own chlorophyll; the chlorophyll molecule consists of protoporphyrin and an atom of magnesium. The author relied upon this protective function of carotenoids to develop a treatment for the photosensitivity associated with EPP. The author will describe here the development of this treatment, and the use of carotenoids in the treatment of EPP and certain other photosensitivity diseases of the skin.
