A case of epidermolysis bullosa acquisita with esophageal stenosis by bulla in esophagus

Abstract

A 27-year-old woman was diagnosed with epidermolysis bullosa acquisita (EBA) in 2007 and received medical treatment since that time. In April 2011, she developed erythematous bullous eruption and erosion covering her whole body, and was admitted as an emergency to the department of dermatology in our hospital. After admission, the patient had dysphagia and vomiting. Upper gastrointestinal endoscopy showed mucosal edema and stenosis associated with esophageal erosion in the middle esophagus. Dysphagia improved after treatment of the EBA, mucosal edema disappeared and the esophageal erosion visible on upper gastrointestinal endoscopy regressed. In July 2011, the patient had acute onset discomfort in her throat and dysphagia recurred. Upper gastrointestinal endoscopy revealed esophageal stenosis─with circumferential extent of one half─around a tense bulla in the upper esophagus. The bulla was ruptured by pressure from the endoscope, removing the stenosis. Subsequently dysphagia quickly disappeared, and the patient was discharged symptom-free. This is the first case of esophageal stenosis caused by a bulla in the esophagus, where the clinical condition may have been due to a previous esophageal erosion and ulcer lesion.