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Effects of Oral Zinc in Erythropoietic Protoporphyria
Suhail Shehade, MRCP;
Russell Ead, FRCP
The Skin Hospital Manchester University School of Medicine Chapel Street Manchester, M60 9EP, England
David McDowell, MRCPath;
Mustafa Gebril, PhD;
Cyril Weinkove, PhD
Department of Clinical Biochemistry Hope Hospital Salford, M30 0NJ, England
Arch Dermatol. 1989;125(12):1713-1714.
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To the Editor.—
Erythropoietic protoporphyria (EPP) is an inherited disorder of heme synthesis due to a defect in the enzyme activity of ferrochelatase, which results in the accumulation of free protporphyrin IX in erythrocytes and plasma, and is associated with cutaneous photosensitivity to UV light (around 400 nm). It is one of the two most common porphyrias, rivaling porphyria cutanea tarda in prevalence. Treatment of the cutaneous symptoms of EPP entail avoidance of sunlight and wearing of protective clothing. Beta carotene, used systemically in doses that produce clinical carotenodermia (recommended serum carotene levels above 400 µg/dL), affords most patients limited photoprotection. It has long been recognized that erythrocyte protoporphyrin  is significantly increased in lead toxicity and some anemias without cutaneous photosensitivity.1 In these cases, protoporphyrin is mainly bound to zinc in the form of zinc protoheme, whereas, in EPP, the accumulated protoporphyrin is metal free. The affinity of zinc for protoporphyrin, and the reduction in erythrocyte photohemolysis that results from cheletion of free protoporphyrin, has been previously demonstrated.2
. . . [Full Text PDF of this Article]
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