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January 1993

Picture of the Month

Author Affiliations

From the Departments of Laboratory Medicine (Dr Rector), Dermatology (Drs DeLoach-Banta and Barrett), and Clinical Investigation (Dr Rector), Naval Hospital, San Diego, Calif.

Am J Dis Child. 1993;147(1):73-74. doi:10.1001/archpedi.1993.02160250075023

An adolescent with a long history of burning and stinging of the skin on exposure to sunlight had the cutaneous changes noted in Figs 1 through 3. Figure 4 is a representative sample of a stained histologic section from a skin biopsy.

Denouement and Discussion 

Erythropoietic Protoporphyria 

Manifestations  Erythropoietic protoporphyria (EPP) is an inherited disorder of porphyrin metabolism characterized by the accumulation of excessive amounts of protoporphyrin in the skin and other tissues. It is the most common form of porphyria and usually presents in childhood, although, rarely, the disorder may not become apparent until late adulthood.1 The most common clinical presentation is a burning or stinging sensation of the skin on sun exposure. The history of skin discomfort usually far outweighs the clinical findings. Itching, edema, and erythema of the skin also are frequent occurrences after exposure to UV light. With more prolonged exposure to sunlight, plaquelike edema and wheals may develop, followed

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