Kelsey Collins, MD1, Ariela Hazan2, Hayato Mitaka, MD1, Erica Grabscheid, MD, FHM1
1Mount Sinai Beth Israel Medical Center / Icahn School of Medicine at Mount Sinai, New York, NY; 2Icahn School of Medicine at Mount Sinai, New York, NY
Introduction: Bullous pemphigoid (BP) and mucous membrane pemphigoid (MMP) are rare autoimmune conditions. The conditions affect the skin and mucosa respectively; immunoglobulins and complement proteins are deposited in the epidermal/ mucosal basement membranes, promoting subepithelial blister formation. 85% of patients with MMP have lesions in the oral cavity, but only 4% have esophageal involvement.
A 37-year-old man with a history of BP (diagnosed via skin biopsy), sickle cell trait, and smoking, presented with 5 months of progressive dysphagia, oral lesions, and weight loss. The dysphagia began with solid foods and progressed to liquids. His ability to initiate swallowing was intact, but he felt as though food was stuck mid-esophagus. Physical exam revealed a thin male with several active bullous lesions on the face and chest, with additional lesions on the hard palate, gingiva, and posterior oropharynx. An esophagram identified a severe serpiginous stricture in the cervical esophagus (C4-5) approximately 3 cm long. An EGD was then attempted with an XP/ stricture endoscope which showed multiple ulcerations and bullae in the upper esophagus; however, the stricture was too narrow for the endoscope to pass. IV steroids were initiated in order to reduce the inflammation contributing to the stricture. The EGD was re-attempted, however the stricture remained too narrow. The patient was discharged with a course of steroids and outpatient follow up. The patient returned 2 weeks later with recurrent dysphagia. He was restarted on the IV steroids and started on IV Diflucan for oral candidiasis. Dilatation of the esophagus was considered, however given the degree, size, and location of the stricture, dilatation was deemed too high risk. He was taken for surgical placement of a gastrostomy tube and tube feeds were ordered. The patient was discharged home with instructions to continue steroids and follow up with GI for a repeat EGD/ possible esophageal dilatation. The use of biologics and/ or immunosuppressants would be considered as future alternative treatments.
Discussion: Bullous pemphigoid and mucous membrane pemphigoid, typically seen in the elderly, are rare conditions. This case highlights a young adult with BP and MMP simultaneously, resulting in significant esophageal involvement; the resulting dysphagia/ severe esophageal stricture required artificial feeding assistance.
Citation: Kelsey Collins, MD; Ariela Hazan; Hayato Mitaka, MD; Erica Grabscheid, MD, FHM. P0335 - A RARE CASE IN A RARER PLACE: A SEVERE ESOPHAGEAL STRICTURE IN A PATIENT WITH BOTH BULLOUS AND MUCOUS MEMBRANE PEMPHIGOID. Program No. P0335. ACG 2019 Annual Scientific Meeting Abstracts. San Antonio, Texas: American College of Gastroenterology.