A rare case of lymphocytic esophagitis in a patient with recurrent and severe oral ulcers

Document Type

Conference Proceeding

Publication Date


Publication Title

Am J Gastroenterol


Introduction: We describe a unique case of lymphocytic esophagitis with non-necrotizing granulomas in a patient with a three-year history of recurrent oral ulcers that did not resolve with standard medical therapy. Case Description/Methods: A 37-year-old female with a history of iron deficiency anemia presents with recurring intermittent episodes of severe oral ulcers that began after a 6-month course of antibiotics for hiradenitis suppurativa. She reported weight loss due to odynophagia and oral pain. An oral ulcer biopsy revealed dense lymphocytic and plasmocytic infiltrate with collections of histiocytic granulomas and multinucleated giant cells. Ulcer outbreaks coincided with stress and menstruation, but a gynecological etiology was unremarkable. Colonoscopy locally was non diagnostic for Crohn's disease (CD). An obvious rheumatological etiology was not found. The patient was placed on fexofenadine once daily and a second biopsy of the oral ulcers revealed connective tissue focally surfaced by fragments of squamous epithelium, erosive lichen planus and a robust influx of mixed chronic inflammation including non-necrotizing granulomas. Further rheumatologic and dermatologic evaluation suggested an oral manifestation of Behcet's disease. Given persistent odynophagia, esophagogastroduodenoscopy was performed. Small non-bleeding erosions in the lower third of the esophagus was found on endoscopy. Biopsies were significant for lymphocytic esophagitis with nonnecrotizing granulomas and mycobacteria and fungus stains were negative. Differential diagnosis by pathology included sarcoidosis, Behcet's disease and inflammatory bowel disease. Acid-fast and fungal stains were negative. Repeat colonoscopy including ileoscopy was negative. Management considerations by rheumatology included genetic testing for HLA-B51, steroids and colchicine. Discussion: Granulomatous esophagitis is a rare manifestation of systemic autoimmune disease including Behcet's, Crohn's Disease and sarcoidosis. Although only seen in 5% of CD cases, esophageal manifestations can present as granulomatous esophagitis. Pyogenic granulomatous esophagitis is typically managed with systemic corticosteroids or endoscopic resection. In our case, evaluation for Crohn's disease was unremarkable and the presence of pathergy and involvement of non-keratinized squamous mucosa is suggestive of an oral manifestation of Behcet's disease despite absent genital ulcers and join pain.

PubMed ID

Not assigned.





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