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A patient with dermatomyositis with both cutaneous and muscle disease refractory to prednisone, azathioprine, intravenous immune globulin, and mycophenolate mofetil developed fever and splenomegaly and was diagnosed with Janus Kinase 2 mutation (JAK2 V617F) post-polycythemia myelofibrosis. The initial dose of ruxolitinib therapy (5 mg twice daily) was advanced to 15 mg twice daily.
She gained weight, her strength improved, and the rash (heliotrope and photodistributed poikiloderma) completely resolved. Mycophenolate was stopped first, followed by prednisone and then intravenous immunoglobulin. Ruxolitinib is a JAK 1 and 2 inhibitor whose mechanism of action involves blocking interferon-beta–induced signal transducers and the activation of tr…