Clinical Challenges

What's your diagnosis?

A 70-year-old man with a history of rectal cancer was referred to our clinic for chronic dysphagia and odynophagia. He did not have fevers or an allergic history. Physical examination was unremarkable except for multiple erosions in the oral cavity. Upper gastrointestinal endoscopy revealed multiple erosions in the palate and laryngopharynx (Figure A), a web stricture in the cervical esophagus (Figure B), and multiple scars in the thoracic esophagus.

Laboratory examination showed normal results including a normal white blood cell count (8010/mcL; eosinophils 360/mcL), hemoglobin level (14.0 g/dL), mean corpuscular volume (97.8 fL), serum iron level (140 mcg/dL), and ferritin level (50.5 mg/L). His dysphagia gradually worsened and he finally could not take pills nor solid food. Two weeks after the first endoscopy, a second endoscopic examination was performed and it showed exacerbation of esophageal stricture and appearance of a bloody blister (Figure C).
What is the diagnosis?


Previously published in Gastroenterology

Mucous membrane pemphigoid with esophageal web stricture.

Additional laboratory examination showed that his serum anti-BP180 antibody level was high (11.7 U/mL; normal range, <9.0 U/mL). Biopsy specimens taken from the laryngopharyngeal erosion showed subepithelial blister formation and it was consistent with pemphigoid pathologically (Figure D). He did not have cutaneous lesions and was diagnosed with mucous membrane pemphigoid (MMP). After performing endoscopic dilation, prednisolone (20 mg/d) was administered orally. Three months after starting the prednisolone treatment, follow-up endoscopy showed improvements of the laryngopharyngeal erosions (Figure E) and esophageal blister on the web. However, esophageal narrowing remained, and thus endoscopic balloon dilation was performed (Figure F-H). Three months after the dilation, the narrowing improved (Figure I).

MMP is an autoimmune blistering disease that induces the formation of mucous membrane subepithelial bullae. Basement membrane zone components such as collagen XVII (also known as BP180) are targets of autoantibodies in MMP. Symptomatic esophageal involvement affects 5.4% of patients with MMP and dysphagia is the most frequent symptom. 1 Endoscopic findings include erosion, web stricture, subepithelial hematomas, and scars. 2,3 Endoscopic dilation is sometimes necessary for the treatment of severe esophageal strictures. 1

1. Zehou O et al. Br J Dermatol. 2017 Oct;177(4):1074-85.
2. Sallout H et al. Gastrointest Endosc. 2000 Sep;52(3):429-33.
3. Gaspar R et al. Gastrointest Endosc. 2017 Aug;86(2):400-2.

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