What are the cutaneous findings in erythropoietic protoporphyria?
| | Fig. 17.8 Erythropoietic protoporphyria. Young child with intense photosensitivity of hands and feet (associated with wearing sandals) manifesting as tense blisters, crusting, and very early thickening of the skin. (Courtesy of the Fitzsimons Army Medical Center teaching files.) | Patients with erythropoietic protoporphyria usually have complaints beginning in childhood, though cases presenting in adult life are well documented. Photosensitivity may be severe, with almost immediate burning and stinging of the exposed skin following sun exposure. Erythema, edema, hivelike lesions, vesicles, and purpura may then develop, particularly on the nose, cheeks, and dorsal hands (Fig. 17-8). With time, these areas develop atrophic, waxy scars. The skin over the knuckles may become thickened, wrinkled, and shiny, giving the appearance of very aged hands.
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