Acute Hemorrhagic Edema of Infancy (AHEI) is a small vessel vasculitis characterized by palpable purpuric skin lesions, edema, and fever. AHEI normally develops in children between the ages of 4 months to 2 years, as opposed to Henoch-Schönlein Purpura, which is more typical in children 2-10 years of age (peak age 4-6). Triggers can include infections, medications including penicillin, cephalosporins, and Trimethoprim- sulfamethoxazole, and immunizations. Clinical features are often preceded by a mild prodromal illness, followed by the rapid development of palpable purpura, ecchymosis, and petechia over 24-48 hours that is distributed mainly on the extremities and face, specifically the ears, eyelids and cheeks. The mucus membranes and the trunk are spared. Because AHEI is an immune-mediated vasculitis, internal organ involvement is possible, although rare, and can include nephritis, arthritis, and gastrointestinal tract problems. Diagnosis of AHEI is clinical, although other serious conditions must be considered in the differential such as non-accidental trauma, leukemia, and Kawasaki Disease. AHEI is a self-limited disease that resolves spontaneously over 1-3 weeks.
Take-Home Points
- AHEI is characterized by palpable purpuric skin lesions, edema and fever. It is distinguished from HSP clinically primarily by the age of onset, with HSP affecting children usually from age 2-10 years.
- Serious conditions such as non-accidental trauma, leukemia, and Kawasaki Disease should be considered and excluded.