Polymyositis Presenting with Isolated Periorbital Edema: a Case-Based Review

Abstract

Periorbital edema is an uncommon presenting sign in idiopathic inflammatory myopathies, which generally manifest with symmetric proximal muscle weakness associated with chronic muscle inflammation. We report the first documented case of polymyositis with an initial presentation of isolated periorbital edema. A 72-year-old African-American male with a history of chronic hypertension presented with marked bilateral periorbital edema. Inflammatory myopathy was not suspected until the patient was hospitalized months later with muscle weakness and dysphagia. Magnetic resonance imaging and electromyography studies were consistent with severe chronic muscle wasting. Polymyositis was confirmed with a muscle biopsy. We identified 16 cases of edema-associated polymyositis documented in the literature between 1982 and 2020. The average age at diagnosis was 57 years, with a median time of four weeks between disease onset and diagnosis. Ninety-three percent involved male patients, despite the higher female prevalence of the disease. Over half of all cases involved bulbar myopathic symptoms, while 20% were associated with malignancy. These cases followed a more rapid clinical course requiring aggressive therapeutic approaches, which was evident in our patient. An atypical presentation of polymyositis should be suspected in the setting of isolated subcutaneous edema, which may be a marker of an aggressive form of the disease. Early identification is crucial in order to intervene prior to life-threatening disease-associated morbidities such as dysphagia or malignancy.