Severe dermatomyositis with pronounced generalized subcutaneous edema and dysphagia: A rare manifestation of a highly active disease

Abstract

Severe subcutaneous edema is rare in dermatomyositis (DM). Such patients usually have a highly active disease, which requires aggressive treatment. To date, there are 14 reported cases of generalized edema secondary to adult DM. We described a severe case of DM manifesting generalized edema, oropharyngeal dysphagia, and dysarthria. A 44-year-old female presented with a typical rash of DM, proximal muscle weakness, and marked swelling of the limbs and face. The findings in the skin biopsy, muscle enzymes, and electromyography were consistent with DM. No internal malignancy was detected. After a brief initial response to oral dexamethasone, the patient experienced a sudden worsening of muscle weakness with dysarthria and an inability to swallow even saliva. A magnetic resonance imaging study revealed edema of the subcutaneous tissue and muscles. The symptoms improved gradually in 2 months after intravenous pulse corticosteroid therapy. Generalized subcutaneous edema is a very rare manifestation of DM that can occur as a presenting symptom. It appears to be a hallmark of a severe DM that requires prompt and aggressive treatment. Additional cases are needed to establish guidelines for treatment of this rare variant.