Methylprednisolone Therapy in Acute Hemorrhagic Edema of Infancy

We present a case of an 18-month-old boy who showed severe clinical signs indicative of acute hemorrhagic edema of infancy (AHEI) with painful purpuric skin affection primarily of the face and marked edema of the ears. The histological findings were diagnostic for leukocytoclastic vasculitis and thus met the histological criteria for AHEI. Indicative of infection as causative agent for the condition were symptoms of gastroenteritis. High-dose intravenous corticosteroids led to a fast resolution of symptoms and normalization of laboratory parameters. AHEI is usually not described as being very responsive to corticosteroids. The case presented here indicates that severe cases of AHEI can be treated with high-dose intravenous corticosteroids resulting in significant relief and shortening of the symptoms. Clinical followup showed no underlying malignancy or other severe chronic systemic diseases thus confirming earlier reports that AHEI is not associated with such conditions. The differential diagnoses with AHEI are discussed. 1. Introduction Acute hemorrhagic edema of infancy (AHEI) is an uncommon benign form of cutaneous small-vessel leukocytoclastic vasculitis, which typically affects children from 4 to 24 months of age [1]. AHEI was first described by Snow in 1913 [2]. A case series and systematic review by Fiore et al. [3] has reported approximately 300 patients with AHEI, with a male predominance. The pathogenesis is not fully understood, but a prodromal phase with various infections has been documented in children with AHEI. These include upper respiratory infections, pharyngitis, conjunctivitis, otitis media, bronchitis, urinary tract infections, and pneumonia [4]. AHEI is characterized by the clinical triad of fever; edema of the face, auricles, and extremities; and rosette-shaped purpura. Unlike Henoch-Sch？nlein purpura (HSP) visceral involvement is infrequent [1]. A correct diagnosis of the disorder is important to distinguish it from other vasculitides. The clinical features of AHEI may be confused with the symptoms seen in (HSP), erythema multiforme (EM), meningococcemia, and septicemia. The diagnostic criteria for AHEI are (1) age younger than 2 years; (2) purpuric or ecchymotic “bruise-like” skin lesions with edema of the face, auricles, and extremities with or without mucosal involvement; (3) lack of systemic disease or visceral involvement and spontaneous recovery within a few days or weeks [5]. AHEI is self-limiting (lasting from one to three weeks) and is not usually considered responsive to corticosteroids [1]. We present a severe case