Dermatomyositis as a Paraneoplastic Manifestation of Ovarian Cancer: A Case Report and Review of Literature

Abstract

Dermatomyositis (DM) is a rare autoimmune disease that occurs throughout the world. It is characterized by pathognomonic cutaneous features and symmetrical proximal muscle weakness of striated muscles of limbs, although an amyopathic form of dermatomyositis also exists. The disease is mainly idiopathic but may present as a cutaneous manifestation of cancer. Pathogenesis of DM is poorly understood, but it is believed that it is caused by altered cellular and humoral immunity. Diagnosis is based on a characteristic clinical picture confirmed by biochemistry and biopsy of skin and muscle. Treatment is mainly by steroids, immunosuppressive drugs and management of underlying malignancy. Recurrence of dermatomyositis symptoms is often the first sign of relapse. Prognosis of ovarian cancer in context of dermatomyositis is poor. We hereby report a case of DM who on investigations is diagnosed to have underlying ovarian cancer. She is managed by systemic steroids, chemotherapy and cytoreductive surgery leading to the resolution of disease which again relapsed after 1 year, again preceded by onset of rash and managed by second-line chemotherapy and monoclonal antibody.