Identification of Esophagitis Dissecans Superficialis by Endoscopy

Abstract

An 88-year-old woman with multiple medical issues including atrial fibrillation, left ventricular systolic dysfunction, chronic congestive heart failure, mitral regurgitation, cardiac arrest, syncope, hypertension, and severe diverticulosis was referred to endoscopy for melena. At endoscopy, white, linear, desquamating plaques were identified in the distal esophagus (Figure A). The oral mucosa was normal, and the only other finding was mild gastric mucosal erythema. There was no history of cutaneous bullous disorders. Medications included allopurinol, amlodipine, furosemide, lisinopril, metoprolol, rivaroxaban, tramadol, and acetaminophen. Distal esophageal biopsies revealed a “two-toned” appearance in intact squamous mucosa fragments (Figure B). The basal layer remained basophilic, whereas the superficial layer was markedly eosinophilic with crowded, pyknotic nuclei, with pockets of edema separating the morphologically distinct areas. In other foci the superficial layer, marked by extensive parakeratosis, was sloughed from the basal layer, correlating to the desquamating plaques identified at endoscopy (Figure C). These endoscopic and histologic features are those of esophagitis dissecans superficialis (EDS) (aka sloughing esophagitis). This is a rare benign condition in which vertical strips of sloughing squamous mucosa create the endoscopic appearance described as “gift-wrap ribbons.” Cases of patients coughing up or vomiting the sloughed mucosa have been reported. Presenting symptoms include dysphagia, odynophagia, and heartburn, although many patients are asymptomatic and undergo endoscopy for unrelated reasons. Although no one specific etiology has been identified, a recent study found that patients with EDS were older and were more likely to be chronically debilitated, to have died since biopsy, and to be taking 5 or more medications when compared with the control population. They were more likely to have hypertension and heart conditions such as valvular disease, arrhythmia, and congestive heart failure. Furthermore, central nervous system suppressants and medications associated with esophageal injury (iron, Kcl, aspirin) were specifically found to be associated with EDS. Cutaneous bullous disorders, particularly pemphigus vulgaris (PV), can cause features identical to EDS. Esophageal involvement in PV may be more prevalent than previously recognized. Absence of cutaneous or oral pharyngeal lesions and immunofluorescence can assist in ruling out PV. Diagnostic considerations to exclude include candidiasis, pill esophagitis, mucosal trauma (hot beverages, chemical irritants), celiac disease, processing artifact, and rigid endoscopy with esophageal dilation. No specific therapy is recommended for treating EDS, and the clinical course is driven by the patient’s underlying disease (possibly self-limited). There are no published data to guide follow-up. Our patient’s advanced age, polypharmacy, and debilitating chronic medical conditions fit well with EDS, although definitive cause remains unknown. Melena was attributed to rivaroxaban, which resolved after discontinuation. Omeprazole was prescribed for gastritis