Hx: 36 y M cocaine user, severe facial pain, epistaxis, crusting, rhinorrhoea, rash/livedo in thighs; hematuria, proteinuriaLab: ESR 36 mm/h; CPR 44 mg/L; WCC 8.0; p-ANCA+; c-ANCA-; anti-MPO-; anti-PR3-; IgG4 increaseNasal biopsy (n = 3): extensive ulceration, fibrin, proliferative fibrosis; acute/chronic inflammatory cells; no vasculitis (1st); geographic necrosis, fibrinoid necrosis foci (2nd); ulcerated mucosa and granulation; no vasculitis (3rd);Dx: CIMDLs and systemic levamisole vasculitisTx: abstinence, steroids; methotrexate; mycophenolate; rituximab, surgical debridement. This evidence concerns the gene PRTN3 and vasculitis.