Acute haemorrhagic oedema of infancy: Alarming but clinically benign vasculitis.

Abstract

Acute haemorrhagic oedema of infancy (AHEI) is a self-limiting small vessel vasculitis of infants and young children. Although the cutaneous manifestation of the disease looks seemingly worrisome, this is a benign clinical condition. Understanding the typical appearance may be of great importance in clinical practice. We report one such case with typical skin lesions. A 12-month-old boy presented with sudden onset non-pruritic ecchymotic patches on the face, trunk and lower limbs. There was a history of an upper respiratory tract infection 3 weeks prior. This illness was not accompanied by any fever, irritability or joint swelling. On examination, he was active, afebrile and had reddish erythematous patchy lesions on the face, trunk and extremities including palms and soles (Fig. 1). The remainder of the examination was unremarkable. Investigations revealed anaemia with haemoglobin 105 g/L; normal total leukocyte count (9.2 × 109/L) with lymphocytosis and normal platelet count. Erythrocyte sedimentation rate was 17 mm/h in the first hour. Kidney and liver function tests were normal. C-reactive protein level was raised at 35.81 mg/L (normal level <6 mg/L). Imaging of the chest and abdomen was normal. Sudden onset of classical purpuric rash in an afebrile active infant prompted a diagnosis of AHEI. Skin biopsy findings were consistent with leukocytoclastic vasculitis. The lesions were self-limiting and resolved within a few days without any desquamation or hyperpigmentation. No recurrences have occurred over the last 12 months. AHEI is an uncommon small vessel vasculitis that presents with transient haemorrhagic oedema and ecchymosis in young children.1 It can follow infections and administration of drugs or vaccines but in most cases, the aetiology remains uncertain.2 Even though lesions of AHEI may appear alarming to parents, the disorder is benign and self-limiting. Diagnosis is essentially clinical. Skin biopsy shows leukocytoclastic vasculitis.3, 4 Renal involvement, as seen in Henoch-Schonlein purpura, is uncommon in AHEI. It is important for paediatricians to be aware of this condition to avoid unnecessary investigations.