In the case of cocaine-induced systemic vasculitis, the clinical and laboratory findings are essentially indistinguishable from primary, idiopathic granulomatosis with polyangiitis (GPA), which is characterized by skin lesions, nasal and palate destruction, pauci-immune glomerulonephritis with crescents and/or necrotizing lesions, and positive laboratory test for cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA) associated with proteinase-3 (PR3) antibodies [2-4]. This evidence concerns the gene PRTN3 and necrotizing vasculitis.