Dermatomyositis Associated with Myelofibrosis following Polycythemia Vera

Abstract

Dermatomyositis (DM) is a unique inflammatory myopathy with clinical findings of proximal muscle weakness, characteristic rash, and elevated muscle enzymes. The association of DM and malignancy, most commonly adenocarcinoma, is well known. There have been few case reports of primary myelofibrosis associated with DM. We present the case of a 69-year-old male with a history of polycythemia vera (PV) who developed proximal muscle weakness, dysphagia, and rash. He was found to have elevated creatinine kinase and skin biopsy was consistent with DM. Due to persistent pancytopenia a bone marrow biopsy was performed and showed postpolycythemic myelofibrosis. To our knowledge, this is the first case reported of this unique association.

POEMS syndrome: a unique presentation and a diagnostic challenge

“…The combination of thrombocytosis and polycythaemia together with splenomegaly prompted a consideration of a myeloproliferative disorder as a cause of the polymyositis which has been described rarely in the literature 1. The unifying diagnosis of POEMS was reached once a plasma cell dyscrasia was identified on biopsy together with the typical sensorimotor axonal and demyelinating polyneuropathy on nerve conduction studies.…”