Paraneoplastic Dermatomyositis presenting as Supraglottitis and acute heart failure; a case report.

Abstract

Dermatomyositis is a rare autoimmune condition with a highly variable clinical presentation characterized by skin changes and skeletal myopathy. It is frequently associated with underlying malignancy. Rare manifestations including myocarditis and upper airway oedema have been described. We present an unusual case of paraneoplastic dermatomyositis which manifested as acute airway compromise and heart failure in a previously healthy 77-year-old man. The combination of symmetrical proximal myopathy, dysphagia, rash and myocarditis suggested inflammatory myositis. The clinical diagnosis was supported by positive laboratory findings including anti-TIF1 antibodies and characteristic changes on cardiac and musculoskeletal MRI. Endoscopic oesophageal biopsy demonstrated high-grade adenocarcinoma. To our knowledge this is the first reported case of acute supraglottitis as a presentation of paraneoplastic dermatomyositis. Dermatomyositis should be considered in the differential of all patients presenting with muscle weakness and rash, even if other symptoms are dominant at the time of presentation.