Acute hemorrhagic edema of young children (cockade purpura and edema): a case series and systematic review.

BACKGROUND: Acute hemorrhagic edema is an uncommon leukocytoclastic small-vessel vasculitis of young children.

OBJECTIVE: To determine clinical features and outcome of acute hemorrhagic edema of young children.

METHODS: Seven new cases are reported. A search of the literature revealed 287 published cases.

RESULTS: The 294 children (boys, 67%) ranged in age between 2 and 60 months (median, 11 months) and were in good general condition. In 195 children the disease developed after a simple acute infection. The exanthemata included large, round, red to purpuric plaques predominantly over the cheeks, ears, and extremities and mostly tender edema of the distal extremities, ears, and face. Involvement of body systems other than skin was rare. The children recovered spontaneously without sequelae.

LIMITATIONS: Results of this review must be viewed with an understanding of the limitations of the analysis process, which incorporated data exclusively from single case reports or case series.

CONCLUSIONS: Acute hemorrhagic edema of young children is a very benign vasculitis. Physicians might rapidly develop the skills necessary to diagnose this condition.