Erythropoietic Protoporphyria

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Abstract
Histological and histochemical studies on specimens of skin from four patients with erythropoietic protoporphyria, whose lesions consisted of thickened eczematized plaques on sunlight exposed areas, showed that with hematoxylin and eosin staining large amounts of hyaline material invariably surrounded the blood vessels of the upper corium. Histochemical studies indicated that this material contains an acidic carbohydrate-protein-lipid complex which includes an acid mucopolysaccharide. Skin from a covered area appeared normal clinically and histologically. This material may possibly arise from combination of degradation products of protoporphyrin and components of perivascular connective tissue. When an eruption is present on patients suspected of having erythropoietic protoporphyria, diagnostic help may be obtained by cutaneous biopsy.

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