A 59-year-old man presented with a history of dysphagia and generalized myalgia and muscle weakness and a rash on the face, neck, and upper arms. Serum muscle enzymes, myoglobin, C-reactive protein, and erythrocyte sedimentation rate were elevated and antinuclear antibodies positive. Electromyographic conduction studies showed pathological changes on arm and leg muscles and magnetic resonance imaging of the oral and neck muscles. A diagnosis of dermatomyositis with severe esophageal involvement was established. Treatment with prednisolone was started and methotrexate added. Enteral feeding with a percutaneous endoscopic gastrostomy was started and a therapy with intravenous immunoglobulin (IVIG) initiated, which caused a rapid improvement of the patient's ability to swallow. This case demonstrates a patient with polymyositis/dermatomyositis who showed steroid-resistant life-threatening esophageal impairment. IVIG resulted in a dramatic improvement of symptoms.

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