Eosinophilic granulomatosis with polyangiitis: unusual presentation of a rare disease in pediatric age

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare disorder in pediatric age, accounting for less than 2% of vasculitis in pediatrics. Its etiology is unknown and difficult to diagnose due to the variability in its clinical picture. Patients have a history of asthma, allergic rhinitis and eosinophilia in peripheral blood as a characteristic feature. Microscopically, necrotizing vasculitis that affects small to medium-caliber vessels, the presence of granulomas and extravascular eosinophilia.Although EGPA is a vasculitis associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCA), in children they are only found in less than 40% of cases. We present the case of an 8-year-old schoolgirl with recurrent respiratory symptoms associated with eosinophilia greater than 10%, significant cutaneous manifestations, eosinophilic esophagitis and polyneuropathy, finally diagnosed with EGPA-ANCA-PR3 (ANCA specific for proteinase 3).