Table I.
Spectrum of mucocutaneous disorders associated with cocaine insufflation
| Disorder | Clinical presentation | Systemic involvement | Serology | Histopathologic features |
|---|---|---|---|---|
| Levamisole-associated vasculitis/vasculopathy4,8,∗ | Inflammatory retiform purpura with predilection for acral sites including ears. | May include fever, arthritis, nephritis, heterogenous pulmonary disease, low complement, lymphopenia, and/or neutropenia. | ANCA positive (85%).8 Often p-ANCA pattern with multiple targets inc. anti-MPO. +ANA and APLs in most. +dsDNA. | LCV with or without thrombotic occlusion, affecting small and medium vessels. Fibrinoid necrosis and sparse inflammation with or without thrombotic occlusion. Pyoderma gangrenosum-like-pattern: neutrophilic dermatosis with vasculitis or vasculopathy. |
| Neutrophilic dermatoses4,9,∗ | Multiple ulcers or vegetative plaques with predilection for the head, ears, and neck. | May include fever, night sweats, arthralgia, leukopenia, and/or renal involvement (commonly pauci-immune crescentic glomerulonephritis and membranous nephropathy). | Often p-ANCA pattern (71%) with antibody targets of anti-MPO and anti-HNE.9 | Neutrophilic dermatosis with thrombotic vasculitis. or Granulomatous pattern resembling superficial granulomatous pyoderma without vasculitis |
| Cocaine-induced midline destructive lesions (CIMDL)4,10,11 | Destructive sinonasal necrosis often leading to septal and/or palatal perforation. | Systemic findings typically absent. | p-ANCA pattern more often than c-ANCA. Antibody targets of anti-PR3 more often than anti-MPO, and anti-HNE (84%).11 | Prominent necrosis with plasma-cell rich infiltrate and LCV with or without thrombosis of venules/arterioles (fibrin deposition or organizing intravascular granulation tissue). No granulomas. |
| Cocaine-associated plasma cell orificial mucositis (CAPCOM)1, 2, 3 | Periorificial vegetative plaques or ulcers, commonly involving nares, upper cutaneous lip, and/or oral mucosa. | Systemic findings typically absent. May present with other cocaine-associated disorders. | c-ANCA more often than p-ANCA pattern. Multiple targets present, including anti-HNE. | Marked epidermal hyperplasia, variable intraepidermal pustules with eosinophils, and a brisk mixed dermal infiltrate with numerous eosinophils and plasma cells. No vasculitis or granulomas. |