Acute Hemorrhagic Edema of Infancy - Case Report

Abstract

Acute hemorrhagic edema of infancy (AHEI) is a rare vasculitis of small dermal vessels with characteristic presentation in infants aged up to 24 months. It manifests as a sudden occurrence of palpable purpuric skin lesions, swellings in hands, feet, face and auricles, and mild fever. The children affected with AHEI are almost always in good general health and with normal laboratory parameters. Approximately 400 cases have been described in the literature so far. However, the etiology is still unknown. Most evidence suggests infections or vaccination as the principal triggers. Extra cutaneous manifestations are recorded in only about 10% of AHEI patients. The majority of the affected children undergoes recovery spontaneously and without any complications within 1-3 weeks, with or without any treatment. AHEI is usually diagnosed on the clinical grounds only and the diagnostic procedure rarely requires a skin biopsy. The current literature indicates the use of corticosteroids and/or antihistamines as a therapy, but there is still a lot of controversy about these therapeutic measures. This paper presents the case of AHEI with its typical clinical manifestations that resolved in a rapid spontaneous recovery without the use of any treatment within a week. AHEI is a rare syndrome that pediatricians should be well familiar with in order to differentiate it from other potentially severe diseases that have similar cutaneous manifestations, but also to avoid unnecessary investigations and therapy.