Una “vistosa” vasculite con edema, che riguarda gli arti e il viso e risparmia il tronco

Abstract

Acute haemorrhagic oedema of infancy (AHEI) is a small vessel vasculitis that affects boys between 4 months and 2 years. It presents with a characteristic purpuric skin rash but with marked bruising and “typical” localizations in auricles and is often accompanied by oedema of face and extremities. It is typically post-infectious, especially after infections of the upper respiratory tract and rarely can be associated with gastrointestinal, renal, articular or testicular complications. The paper reports the case of a 2-year-and-5-month-old boy who, 5 days after the resolution of a febrile infection of the upper airways (with subsequent detection of positive IgM and IgG serology for SARS-Cov-2), presented with a rash with ecchymotic/purpuric lesions in his lower limbs, face and auricles associated with intense arthralgia, arthritis of a knee and deterioration of general conditions. The diagnosis of acute infantile haemorrhagic oedema was clear but the clinical presentation required an “important” therapeutic approach with intravenous steroids and an infusion of immunoglobulins.