Rheumatic consequences of cocaine are rare but well documented. These disorders primarily manifest as organ specific vasculitis (such as cerebral vasculitis), systemic vasculitis, or cocaine-induced midline destructive lesions (CIMDL) [
1]. 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]. CIMDL can closely mimic the upper airway lesions seen in GPA with reports ranging from severe sinusitis to nasal and palatal perforation; however, the pathophysiology is thought to be secondary to chronic cocaine exposure, leading to progressive mucosal and perichondrial injury and subsequent ischemic necrosis and perforation of the nasal septum [
5,
6]. Histologically, CIMDL is distinguished from GPA by the lack of granulomatous inflammation and vasculitis on biopsy, and serologic differences have been reported [
7,
5,
8,
9]. While these pathologies are rare, it is important to recognize the association with cocaine use, as cessation of the drug is often the only treatment needed.
Since 2005, a new vasculitic syndrome appears to be occurring with increasing frequency in patients who use cocaine, characterized by positive perinuclear (p)-ANCA serologies, neutropenia/agranulocytosis, and purpuric lesions involving the face, especially the ear lobes, with varying frequencies of glomerulonephritis and lung hemorrhage. The increased prevalence appears to be correlated with the escalating abuse of levamisole-adulterated cocaine [
1]. This review will summarize the range of clinical and pathologic findings seen in the setting of levamisole-adulterated cocaine exposure with an emphasis on the morphologic features observed in patient biopsies.