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Acute hemorrhagic edema of infancy: a variant of Henoch-Schönlein purpura?

Acute Hemorrhagic Edema of Infancy (AHEI) is a rare leukocytoclastic vasculitis and there are around 100 cases described in the English language literature. The typical cutaneous lesion is a palpable purpura localized on the face, ears, and extremities and resembles a medallion. It is a vasculitis of small vessels, mainly seen in children less than 2 years of age. It is usually self-limited and benign, despite its appearance. We report a case of an infant who presented with swelling of hands and feet, purpuric lesions on face and fever, and compare with other previously described cases, accordingly to the literature review. The rarity may be associated with underdiagnosis or mistaken diagnosis of Henoch-Schönlein purpura. AHEI is preceded in the majority of cases by infections, immunizations or drugs. Mucosal and visceral involvement is seldom seen. No treatment is currently recommended. The alert for this vasculitis aims to help the diagnosis, making it earlier and preventing unnecessary concern and treatment.

acute hemorrhagic edema of infancy; vasculitis; purpura


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