Acute hemorrhagic edema of infancy

Abstract

In infants less than 2 years of age, acute hemorrhagic edema of infancy (AHEI) is characterized by the rapid onset of purpuric annular lesions associated with initially local edemas of the extremities. In most cases, this disorder is benign, without visceral involvement and diagnosis is based on clinical examination. There are no specific laboratory data and the histological analysis of these lesions (which is unnecessary in the majority of cases) is usually unspecific (the patient sometimes has an unspecified leucocytoclasic vasculitis). This condition is of uncertain nosologic status, mimiking rheumatoid purpura. Sometimes the patient had had rhino-pharyngeal episode in previous days, suggesting a viral cause. There is a clear discrepancy between the good general condition and the profuse and spectacular appearance of the lesions. Children's monitoring in the first days must be rigorous but complications are exceptional (acute intussusception'). Patient's outcome is marked by spontaneous regression within 12 days. Treatment is based on careful monitoring of the child's general status. Fever, purpuric lesions extension and, especially, signs of alteration of the general state may suggest the diagnosis of purpura fulminans. Another differential diagnosis is acute hemorrhagic or ecchymotic urticaria. We report the case of a 3-month old infant with diffuse pseudo-roundel like lesions in a context of apyrexy and preservation of the general state. Patient's outcome was favorable, without receiving any treatment. This confirm the diagnosis of AHEI.