Cocaine is known to cause thrombotic complications. Its use has also been associated with a variety of rheumatologic manifestations, most notably cutaneous vasculopathy. We report clinical features and laboratory findings of cocaine-related cutaneous vasculopathy.


Here we describe 8 patients with cocaine-related cutaneous vasculopathy.


Our patients most commonly presented with purpuric lesions and ulcers with areas of skin necrosis. Extracutaneous features included fatigue and arthritis in most patients, as well as weight loss in 3 patients, pneumonia in 3, diffuse reactive lymphadenopathy in 1, and acute upper airway obstruction in 2. Laboratory abnormalities included elevated inflammatory markers and positive perinuclear antineutrophil cytoplasmic antibodies (ANCAs) in all patients, and antimyeloperoxidase antibodies in 7 of 8 patients. Cytoplasmic ANCA was positive in 5 of 8, equivocal in 2 of 8, and negative in 1 of 8 patients. Anti–proteinase 3 antibodies were found in 6 of 8 patients. Six of 8 patients had positive antinuclear antibodies and 5 of 8 had positive anti–double-stranded DNA antibodies (4 of 5 were at low titers). Two patients previously thought to have systemic lupus erythematosus had low complement levels. Lupus anticoagulant was positive in 3 of 8 and equivocal in 5 of 8. Anticardiolipin IgG was positive in 1 of 8 and IgM was positive in 6 of 8; β2-glycoprotein I IgM was positive in 2 of 8. Cold agglutinins were strongly positive in 6 of 6 patients in whom they were measured. Skin disease improved in 3 patients who appeared to have stopped using cocaine. It was chronic and progressive in 4 patients who continued to use cocaine, 2 of whom died presumably due to multidrug overdose.


This report outlines clinical features and distinctive laboratory findings that, when present in the right clinical setting, should prompt consideration of cocaine-related cutaneous vasculopathy.