Barium studies in esophageal cicatricial pemphigoid

Cicatricial pemphigoid (CP) (benign mucous membrane pemphigoid) is a rare, blistering disease of skin and mucous membrane. The disease rarely extends to involve the esophagus, and there are only a few cases reported in the radiological literature. The aims of this study were to document the frequency of esophageal involvement and to describe the findings on upper GI barium studies.

A total of 197 patients with CP were seen at our institution from 1981 to 1991. The clinical and radiological findings of these patients were reviewed and compared with findings reported in the literature.

Esophageal involvement was documented in seven patients. Cervical esophageal webs were found in five of the seven patients. Two patients had single esophageal webs while three had multiple webs. Frank strictures of the esophagus were also seen in five patients. These were most common in the cervical esophagus, but strictures were also found in the mid and lower esophagus. Two of the strictures resulted in significant dysphagia and required multiple endoscopic dilatations. One of the dilatations was complicated by mucosal injury, and follow-up barium examination showed dissection of the esophageal mucosa from the cervical esophagus to the esophagogastric junction. One patient demonstrated intramural pseudodiverticulosis in the cervical esophagus. Functional disturbances demonstrated on barium studies included tracheal aspiration in two patients and nasopharyngeal reflux in three.

CP involves the esophagus in approximately 5% of cases. The hypopharynx and cervical esophagus are most commonly involved, but any portion of the esophagus may be involved, and multiple levels of involvement may be seen. Cervical esophageal webs, often multiple or complex, are the most common appearance on barium studies, but frank strictures are also found. Secondary manifestations of esophageal involvement include nasopharyngeal reflux, tracheal aspiration, and intramural pseudodiverticulosis.