A 67-year-old woman had a 4-year history of dermatomyositis manifesting as a heliotrope rash, shawl sign, Gottron papules, proximal upper and lower extremity muscle weakness, and dysphagia. The creatine kinase level was 424 U/L (reference range, 2-150 U/L) and the erythrocyte sedimentation rate was 40 mm/h (reference range, 0-25 mm/h). Results of the remainder of the laboratory examination, including globulin levels, were within normal limits. Recent screening colonoscopy, mammography, Papanicolaou smear, and cancer antigen 125 levels showed no evidence of malignancy. She was refractory to a treatment regimen of prednisolone, 60 mg/d; mycophenolate mofetil, 750 mg twice daily; and chloroquine, 250 mg/d. She had not tolerated azathioprine or methotrexate, and trials of quinacrine and hydroxychloroquine had failed. She had chronic hypertension, which was controlled with ramipril, 5 mg/d. She was a nonsmoker, with no personal or family history of hypercoagulability.