Scleredema adultorum of Buschke with upper esophageal involvement

A 55-yr-old white female with biopsy proven scleredema adultorum was evaluated for progressive dysphagia. Esophageal motility studies revealed persistently high upper esophageal pressures without response to deglutition, and minimal peristalsis in the upper body of the esophagus. A cine-esophagram revealed massive aspiration of barium into the nasopharynx and lungs, concommitant with stenosis in the upper esophagus. The patient died presumptively from aspiration. This patient exemplifies a potentially lethal complication of scleredema adultorum i.e., upper esophageal infiltration with resultant aspiration.