A 46-year-old woman comes to the office due to dysphagia. The patient has had difficulty swallowing both liquids and solid foods as well as frequent episodes of regurgitation and cough while eating. She has also had weakness in her extremities and a rash. The patient has no prior medical problems and takes no medications. Vital signs are within normal limits. Physical examination shows an erythematous eruption on the upper eyelids. The oropharynx is clear and cardiopulmonary and abdominal examinations are unremarkable. She has difficulty lifting her arms above her head and standing from a sitting position. Which of the following is the most likely cause of this patient's dysphagia?

Question

A 46-year-old woman comes to the office due to dysphagia.  The patient has had difficulty swallowing both liquids and solid foods as well as frequent episodes of regurgitation and cough while eating.  She has also had weakness in her extremities and a rash.  The patient has no prior medical problems and takes no medications.  Vital signs are within normal limits.  Physical examination shows an erythematous eruption on the upper eyelids.  The oropharynx is clear and cardiopulmonary and abdominal examinations are unremarkable.  She has difficulty lifting her arms above her head and standing from a sitting position.  Which of the following is the most likely cause of this patient's dysphagia?

Quizplus · Accepted Answer

This patient has painless proximal muscle weakness; in association with the heliotrope rash on her eyelids, this presentation is consistent with dermatomyositis, an inflammatory myopathy that primarily affects striated muscle and skin. Diagnostic findings include elevated muscle enzymes (eg, creatinine kinase) and positive autoantibodies (ie, antinuclear antibody [high sensitivity, low specificity], anti-Jo-1 [low sensitivity, high specificity]). Muscle biopsy in dermatomyositis shows perimysial inflammatory infiltrates and atrophy involving the fibers around the periphery of muscle fascicles (perifascicular atrophy). The oropharynx and upper esophagus also contain skeletal muscle that can be affected in dermatomyositis and polymyositis, leading to dysphagia and pulmonary aspiration. Other important complications of inflammatory myopathies include interstitial lung disease and myocarditis (due to involvement of striated cardiac muscle). (Choice A) Progressive systemic sclerosis (scleroderma) commonly affects the esophagus, leading to atrophy and fibrous replacement of the distal esophageal muscularis propria. Clinical features include dysphagia, reflux esophagitis, and aspiration. However, the dermal manifestations include sclerodactyly, digital ulcers, and calcinosis cutis, rather than a heliotrope rash. Also, dermatomyositis affects only the striated muscle in the oropharynx and upper esophagus (eg, choking, aspiration), whereas scleroderma can affect the lower esophagus. (Choice B) Eosinophilic esophagitis typically presents with dysphagia, epigastric pain, recurrent esophageal reflux, and food impaction. Skin rash and extremity weakness would be unexpected. (Choice C) Achalasia is caused by inflammation and degeneration of the esophageal myenteric plexus, resulting in impaired relaxation of the smooth muscle in the distal esophagus and lower esophageal sphincter. It typically presents with dysphagia, regurgitation, and heartburn but is not associated with rash or skeletal muscle weakness. (Choice D) Zenker diverticulum develops immediately above the upper esophageal sphincter, with posterior mucosal herniation between the fibers of the cricopharyngeus muscle. Although it presents with dysphagia, regurgitation, and aspiration, it is not associated with rash or muscular weakness. Educational objective: Dermatomyositis is characterized by proximal muscle weakness resembling polymyositis, with additional inflammatory features affecting the skin (eg, heliotrope rash, Gottron papules). In both conditions, involvement of striated muscle in the oropharynx and heart can lead to dysphagia, pulmonary aspiration, and myocarditis.

A 46-Year-Old Woman Comes to the Office Due to Dysphagia