Esophagitis Dissecans Superficialis: An Uncommon Endoscopic Diagnosis

Abstract

A 72 year old female with a history of cervical nerve impingement presented with sudden onset of odynophagia. On presentation to the emergency room, the patient reported 6 hours of severe odynophagia which was dull at rest but spastic with swallowing. Of note, the patient had recently started taking cyclobenzaprine at bedtime and had completed a course of prednisone three weeks prior for neck pain attributed to her cervical nerve impingement. She denied fevers, chills, nausea, vomiting, regurgitation, dyspepsia and abdominal pain. She had no prior surgeries. Physical exam revealed an afebrile woman in no apparent distress with normal oropharyx, pulmonary and abdominal exam. Initial laboratory studies revealed CBC and CMP within normal limits. Plain films of the neck revealed no radiopaque foreign bodies or soft tissue swelling. EGD showed long strands of friable tissue paper-like mucosa overlying normal appearing esophageal mucosa. There were no signs of esophageal narrowing or retained food. Biopsies showed diffuse superficial necrosis of the squamous mucosa with separation of the necrotic upper portion from the unremarkable viable deeper layers. Additionally, few intraepithelial eosinophils were noted. These findings were most consistent with esophagitis dissecans superficialis(EDS). The patient completed a month course of twice daily proton pump inhibitor with complete resolution of her symptoms. EDS is a desquamative disorder of the esophagus with a benign natural history that is under-recognized with less than 100 published cases. Currently the etiology of EDS is poorly understood and it has a wide range of associations. The most common pathogenesis is thought to besecondary to injury of the esophageal mucosa, specifically from medications and other caustic agents. Case reports previously had focused on the histological constellation of parakeratosis, strips of epithelium with distinct necrotic edge, and lack of inflammatory infiltrates. More recent studies have proposed a clinicopathologic diagnosis of EDS with the major diagnostic criteria being 3 key endoscopic findings of the esophageal mucosa: (1)strips of sloughed esophagus, (2)normal underlying mucosa, (3)lack of ulceration/friability of adjacent esophageal mucosa. Increased awareness of the clinical, endoscopic, and histologic features of EDS should result in greater detection rates, which will prevent unnecessary investigation and therapies while aiding in the understanding of its etiology.Figure 1Figure 2