Cutaneous small vessel vasculitis (CSVV) is a disorder characterized by neutrophilic inflammation of small blood vessels in the skin without associated signs of systemic vasculitis. Most CSVV cases resolve within weeks; however, a subset develop chronicity or complications requiring immunomodulatory therapy. This report presents a case of CSVV treated successfully with leflunomide.
A woman in her 40s presented with extensive 1-mm to 2-mm purpuric macules and papules on her lower extremities and abdomen. She had a 20-year history of intermittent purpuric lesions and had previously failed to tolerate and/or respond to dapsone, colchicine, and methotrexate. She complained of weekly flares of skin burning and itching, with minimal control on prednisone (20 mg/d). Over the past year, her disease had progressed with extension of the purpura to her abdomen and new ulcerations of her lower extremities. Past medical history was notable for migraines, morbid obesity, and joint pain of her fingers, ankles, and knees. Current medications (except prednisone) were started more than 10 years after the onset of the rash and included zolpidem, duloxetine, acetaminophen-hydrocodone, and atenolol. She denied nonsteroidal anti-inflammatory drug, supplement, or herbal remedy use. Physical examination revealed purpuric macules and papules scattered diffusely on the lower extremities, forearms, buttocks, flanks, and abdomen with ulcerations with serum crust (Figure 1A).
Stiegler JD, Sami N. Successful Treatment of Cutaneous Small Vessel Vasculitis With Leflunomide. JAMA Dermatol. 2017;153(9):940–942. doi:10.1001/jamadermatol.2017.1316
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