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A 36-year-old woman presented to Emergency with 2.5 years duration of waxing and waning severe pain related to confluent necrotic lesions involving her lower extremities. Her medical history was significant for crack cocaine use for 12 years, resulting in a recent admission for febrile neutropenia consistent with levamisole toxicity. Last use was the night prior to this presentation, toxicology screen was positive for cocaine. Physical exam revealed a cachectic, malnourished woman in severe distress with painful non-blanching purpura noted along the helixes of both ears and tip of the nose. Her lower extremities had large, minimally retiform purpuric plaques with surrounding erythema, which later developed hemorrhagic bullae over the subsequent 10-day hospital course. At admission, her absolute neutrophil count was 2,200, reaching a nadir of 1,000. Inflammatory markers included C-reactive protein elevated at 6.9, erythrocyte sedimentation rate greater than 120. No evidence of infectious process was found during the hospital course, including viral serologies and bacterial cultures.ANA was positive (titer, 1:320), as well as P-ANCA positive (titer, 1:2560) with both PR3 and MPO significantly elevated at 441 and 289, respectively. Additional rheumatologic serologies included positive Lupus-like inhibitor, Beta-2 glycoprotein IgM, anti-cardiolipin IgM, and normal IgG levels. A skin biopsy was obtained and histopathology showed dilated venules with abundant small to medium-sized fibrin thrombi. (panel at Left) Direct immunofluorescence pattern had deposition of IgG, IgM, IgA, C3, and fibrinogen in venule walls diagnostic of leukocytoclastic vasculitis. (panel at Left) Treatment with steroids resulted in slow partial resolution of the lesions.


Won poster competition at the American College of Physicians New Mexico Chapter Scientific Meeting, 10/2010, Albuquerque, NM. Presented at the National American College of Physicians Meeting San Diego 04/11.