An Unusual Cause of Dysphagia

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Abstract

Scleredema adultorum of Buschke is a rare scleroderma mimic characterized by woody induration of the skin involving the back of the neck, interscapular region, upper and mid back, and sometimes the face, upper chest, and upper arms. Dysphagia is rare and not well characterized. We describe a case of scleredema that developed significant dysphagia affecting both the oropharyngeal phase and the esophageal phase of deglutition. Diagnostic evaluation revealed mild-moderate oropharyngeal and severe esophageal dysmotility. This is the first reported case of scleredema that exhibited severe dysmotility due to involvement of the smooth muscle of the entire esophagus. This case adds another sclerosing skin disorder to the list of causes of significant symptomatic dysphagia due to pharyngoesophageal dysmotility: systemic sclerosis, scleromyxedema, and chronic graft-versus-host disease. We hypothesize that dysmotility resulted from infiltration of the pharynx and esophagus with acid mucopolysaccharides. However, in the absence of histopathologic evidence, this is purely speculative. To develop an effective therapeutic approach, further studies are needed to understand the underlying pathophysiology of this uncommon but debilitating complication of scleredema.

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