Granulomatosis with polyangiitis. A diagnostic challenge in pediatric patients

Granulomatosis with polyangiitis is a systemic autoimmune disease characterized by the presence of granulomatous necrotizing vasculitis with kidney, respiratory, and vascular involvement, whose peak incidence is in the fifth and sixth decades of life. Its localized presentation without systemic involvement is infrequent. We present the case of a 10-year-old patient with recurrent eyelid edema as the initial symptom in the setting of granulomatosis with systemic polyangiitis. Although rare, this disease can affect children, as in the case we present, and given its low incidence and atypical presentation it is sometimes a diagnostic challenge.