An Elderly Woman With an Uncommon Cause of Dysphagia

Abstract

Question: A 68-year-old woman with a past medical history of asthma and vulvar lichen planus is referred to us for symptoms of dysphagia. Her dysphagia is to solids only. Symptoms started 1 year before presentation and have been progressive in nature. There was no associated odynophagia, regurgitation, unintentional weight loss, melena, or hematochezia. She has mild symptoms of gastroesophageal reflux disease that are well controlled with once daily proton pump inhibitor therapy. Her medications include daily maximum dose inhaled steroids for asthma. She has no history of smoking or alcohol abuse. On oral examination, no lesions including oral thrush were noted. Our patient underwent a barium esophagram that showed a distal esophageal stricture (Figure A, arrows). An upper endoscopy was pursued that showed significant mucosal changes with white plaques, ulcers, and scarring causing luminal narrowing and friability (Figure B–E). Multiple large unstained lesions on Lugol's chromoscopy were noted (Figures F, G). Multiple biopsies were obtained from the proximal to the distal esophagus (Figure H, I). Grocott’s methenamine silver stain was negative for fungal pathogens. Based on the clinical scenario, endoscopic findings and pathology images, what is the most likely diagnosis? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. Our patient’s dysphagia is secondary to esophageal leukoplakia (also known as “epidermoid metaplasia”). Esophageal biopsies (Figure H, I) reveal marked hyperkeratosis and prominent granular cell layer consistent with esophageal leukoplakia. A repeat upper endoscopy with biopsies was performed without evidence of underlying carcinoma or dysplasia. The patient was placed on twice daily maximum dose proton pump inhibitor therapy. Her symptoms improved. Esophageal dilation was deferred considering extreme mucosal friability and improvement of symptoms with medical therapy. She is enrolled currently in an active surveillance program consisting of repeat upper endoscopies and biopsies for early detection of dysplasia. Esophageal leukoplakia is extremely rare and poorly described in the literature. It is characterized by the presence of mucosal white plaques that cannot be scraped. In a recent case series of esophageal leukoplakia in 18 patients from multiple medical centers, it was noted that the majority of the patients were smokers or exposed to secondhand smoke (61%).1Singhi A.D. Arnold C.A. Crowder C.D. et al.Esophageal leukoplakia or epidermoid metaplasia: a clinicopathological study of 18 patients.Mod Pathol. 2014; 27: 38-43Crossref PubMed Scopus (33) Google Scholar There is an unclear association between esophageal leukoplakia and squamous cell dysplasia/carcinoma. For example, the presence of high grade dysplasia or squamous cell carcinoma in 18 esophageal leukoplakia patients was 17% in 1 series.1Singhi A.D. Arnold C.A. Crowder C.D. et al.Esophageal leukoplakia or epidermoid metaplasia: a clinicopathological study of 18 patients.Mod Pathol. 2014; 27: 38-43Crossref PubMed Scopus (33) Google Scholar Another small series identified 4 patients with epidermoid metaplasia and all 4 had either synchronous or metachronous squamous cell carcinoma.2Ezoe Y. Fujii S. Muto M. et al.Epidermoid metaplasia of the esophagus: endoscopic feature and differential diagnosis.Hepatogastroenterology. 2011; 58: 809-813PubMed Google Scholar All 4 patients in that case series were heavy alcohol drinkers. Our case is unique in that this is a rare condition and our patient lacked risk factors such as smoking or alcohol abuse.