A Case of Epidermolysis Bullosa Acquisita with Pseudomembranous Esophageal Involvement / 대한피부과학회지

ABSTRACT

Epidermolysis bullosa acquisita (EBA) is an acquired subepidermal blistering disorder characterized by autoantibodies to anchoring fibril (type VII) collagen. EBA is known to have a wide clinical spectrum that includes a non-inflammatory mechanobullous presentation, an inflammatory vesiculobullous eruption akin to bullous pemphigoid, and a mucosal centered disease with scarring that is reminiscent of cicatrical pemphigoid. Patients with EBA often have lesions on the oral mucosa, but esophageal involvement has not been well documented. We report a case of EBA in a 63-year-old woman who had a pseudomembranous esophageal lumen. To our knowledge, this is the first report of EBA with pseudomembranous esophageal involvement.