P-ANCA Positive Cocaine Associated Vasculitis: A Case Report

Antineutrophilic cytoplasmic antibodies (ANCA) are classically associated with primary vasculitic syndromes, including Churg-Strauss syndrome, microscopic polyangiitis, and Wegeners granulomatosis. Recently, drug induced ANCA positive vasculitis has been reported, most frequently in association with propylthiouracil in Grave’s disease. Cocaine use is known to be associated with pulmonary and systemic eosinophilia as well as various vascular pathology, including vasospasm, atherosclerosis, and vasculitis. We report a case of cocaine associated perinuclear antineutrophilic cytoplasmic antibody (p-ANCA) positive vasculitis. A 46-year-old man with a history of crack cocaine abuse presented with arthralgia, lower extremity edema, and multiple purpuric, tender papules and plaques over the hands and lower extremities. Peripheral blood studies revealed hypereosinophilia, and serologic tests demonstrated a p-ANCA titer of 1:2560. A skin biopsy demonstrated injured small dermal vessels with a perivascular infiltrate of neutrophils, karyorrhectic debris, erythrocytes, and abundant eosinophils. His symptoms and eosinophilia resolved with cessation of cocaine use and intravenous dexamethasone. Following a 2 year period of reported abstinence, the patient relapsed on cocaine and presented with similar clinical, serologic, and histologic findings. Although p-ANCA has been reported in patients with cocaine associated midline destructive lesions, to our knowledge, this is the first report of cocaine associated p-ANCA positive systemic vasculitis.