CIMDL can mimic systemic diseases with positive anti-neutrophil cytoplasmic antibodies (ANCA), such as granulomatosis with polyangiitis (GPA).<h4>Case report</h4>In this article, we describe the case of a young woman who presented with nasal perforation induced by cocaine, along with positive perinuclear ANCA test (proteinase 3 antigen), misdiagnosed as limited GPA. This evidence concerns the gene PRTN3 and granulomatosis with polyangiitis.