Multidisciplinary management of persistent dysphagia in mucous membrane pemphigoid: a case report

Abstract

: Mucous membrane pemphigoid (MMP) is a chronic, autoimmune, subepithelial blistering disease with a predilection for mucosal surfaces. Patients often present with lesions in more than one site, including the mouth, eyelids, scalp, genitals, larynx, or esophagus. The evolution of active erosions into scars is the hallmark of this disease, which leads to loss of tissue function and increased patient morbidity. Esophageal involvement is seen in about 2–7% patients with MMP and can result in debilitating esophageal strictures and stenosis requiring intermittent, and often frequent, esophageal dilation. Typically, esophageal disease activity coincides with dermatologic activity and therefore management is primarily driven by Dermatology. Though there is no definitive treatment for MMP, symptomatic treatment is accomplished with use of immune therapies including systemic steroids. We present a case of MMP with persistent dysphagia despite escalation of immunosuppression which was adequate enough to successfully treat dermatologic manifestation of MMP. Despite maximizing systemic treatment of MMP and multiple repeated endoscopic esophageal dilations, our patients esophageal MMP and dysphagia persisted. Ultimately, with the addition of swallowed fluticasone as a result of Dermatology and Gastroenterology co-management, the patient achieved complete response. To the best of our knowledge, our case is the first to describe the use of swallowed steroids (fluticasone or budesonide) in a patient with refractory esophageal MMP. 5