Acute hemorrhagic edema of infancy: a vasculitis with good prognosis
Aims: To report two cases of acute hemorrhagic edema of infancy, a rare vasculitis characterized by purpuric skin lesions and peripheral edema without systemic involvement (excluding fever), which has a sudden onset and an usually benign course, with spontaneous resolution.
Cases description: The authors describe two cases of male infants, 11 and 12 months old, who were admitted to the emergency department with fever and progressively worsening purpuric lesions. The diagnosis of acute hemorrhagic edema of infancy was made by exclusion and sustained by the fact that, despite the impressive skin presentation, both infants had a good general state and presented the classic triad of this entity: fever, peripheral edema, and purpuric lesions on the face, ears and extremities. Both boys presented total regression of the lesions in about one week, without sequelae.
Conclusions: There are just over 100 cases of hemorrhagic edema of infancy reported worldwide. The rarity of this vasculitis may be due to underdiagnosis or mistaken diagnosis of other leukocytoclastic vasculitis, mostly Henoch-Schönlein purpura. In addition to this, there are other conditions to consider in the differential diagnosis, such as meningococcemia, Kawasaki disease and erythema multiforme, which have similarities, but at the same time distinctive features that allow to exclude them. Timely diagnosis of acute hemorrhagic edema of childhood is crucial to avoid unnecessary therapies and supplementary tests, as well as to reassure the family about the good prognosis of the disease.
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