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September 1989

Erythropoietic Protoporphyria Presenting in Adulthood

Author Affiliations

Department of Dermatology Harvard Medical School and Massachusetts General Hospital Boston, MA 02114

Arch Dermatol. 1989;125(9):1286-1287. doi:10.1001/archderm.1989.01670210124029

To the Editor.—  Erythropoietic protoporphyria (EPP) is an inherited disorder of porphyrin metabolism resulting in the elevation of red blood cell, plasma, and fecal protoporphyrin levels, plus cutaneous photosensitivity. The most common symptoms are painful burning and pruritus of exposed skin, often arising within minutes of exposure to ultraviolet light. Acute skin findings may include erythema or edema. However, skin lesions following attacks may be subtle or absent.1,2The onset of symptoms in EPP is normally in infancy or in early childhood on first exposure to sunlight; it is rare for initial symptoms to occur late in life.3 The most common of the porphyrias to present in adulthood is porphyria cutanea tarda. This porphyria typically presents in middle age, and is commonly associated with alcohol abuse or diabetes mellitus. Skin manifestations include vesicles, bullae, and milia, and scarring in sun-exposed skin, as well as increased skin fragility, sclerotic

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