1734 An “Off the Wall” Case of Sloughing Esophagitis

Abstract

INTRODUCTION: Esophagitis dissecans superficialis (EDS), or sloughing esophagitis, is a rare, benign disease characterized by large, longitudinal sheets of sloughing mucosa visualized on upper endoscopy. Symptoms typically vary and include dysphagia, odynophagia, abdominal pain, and dyspepsia. EDS has been associated with medications, chemical and thermal irritants, autoimmune bullous dermatoses, and celiac disease. However, its pathogenesis is still poorly understood. We present a case of EDS in a patient with multiple comorbid autoimmune disorders including type 1 diabetes mellitus, Hashimoto’s thyroiditis, and celiac disease. CASE DESCRIPTION/METHODS: A 22 year old female was admitted with diabetic ketoacidosis (DKA), dysphagia, abdominal discomfort and weight loss. Her medical history included poorly controlled type 1 diabetes mellitus, celiac disease, and Hashimoto’s thyroiditis. Her upper gastrointestinal symptoms led to anorexia and unintentional weight loss. After resolution of DKA, Gastroenterology was consulted for evaluation of her symptoms. Upper endoscopy was performed which revealed mild esophagitis, mild gastritis, and scalloping in the duodenum. Esophageal biopsies demonstrated fragments of squamous mucosa with features consistent with "sloughing esophagitis". Her appetite eventually improved with initiation of proton pump inhibitor therapy and management of her poorly controlled diabetes. On outpatient follow up, her upper GI symptoms continued to improve and her weight stabilized. DISCUSSION: EDS is a rare endoscopic finding of white plaques in vertical columns that represent sloughing of large fragments of squamous tissue. Histologically, the most common features of EDS are parakeratosis, pauci-immune intraepithelial sloughing, and intraepithelial splitting. The pathogenesis of EDS is not fully understood though has been associated with autoimmune skin disorders and celiac disease. There are no specific treatments for EDS as it is usually self-limited. We present a case of sloughing esophagitis in a patient with multiple pre-existing autoimmune diseases including type 1 diabetes mellitus, Hashimoto’s thyroiditis, and celiac disease. This case is unique in that she has a polyglandular like autoimmune disorder. This suggests that autoimmunity may play a role in the pathogenesis of EDS. Interestingly, she lacked all of the dermatologic manifestations that are most frequently associated with EDS.