Erythropoietic protoporphyria

Erythropoietic protoporphyria (EPP) is an autosomal dominant condition characterized by marked skin photosensitivity.

Patients typically complain of a burning sensation on relatively brief exposure to sunlight. Urticarial or eczematous changes may appear later.

Punctate scars occur in areas of chronic sun exposure such as the malar skin.

Particularly a problem in childhood, the condition can persist through adulthood.

Treatment

• Case reports show benefit from PUVA and UVB which increase tolerance to sunlight.

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