The etiology of immunoglobulin A vasculitis (IgAV) remains unclear, but the formation of galactose-deficient IgA1 (Gd-IgA1) and its associated immune complexes is believed to play a significant role in the pathogenesis of systemic vasculitis and tissue damage, thus representing a crucial mechanism in the development of IgAV (10). The gene discussed is IGHA1; the disease is necrotizing vasculitis.