This 26 month old boy developed widespread hemorrhagic papules and plaques on the extremities, buttocks and face with edema of the ears, hands and feet. Histopathologic examination of the skin showed a leukocytoclastic vasculitis and direct immunofluorescence (DIF) was negative. The skin lesions healed in 8 days without necrosis. The morphology, histopathology and course of the skin rash with negative DIF and lack of systemic disease is most consistent with infantile acute hemorrhagic edema (IAHE). Although originally described in the United States in 1913, IAHE has been generally ignored in the English literature. Unlike Henoch-Schonlein purpura, IAHE occurs in children under 2 years old and is not associated with serious visceral disease. For a good review see J Am Acad Dermatol 1991;24:17-22. (Click on the diagnosis for Pubmed search.)
Description
round red purpuric papules and plaques some forming large confluent and reticulated plaques on the limbs, buttocks and face with edema of the ears and extremities, particularly the hands and feet