S4438 Esophageal Cast After Balloon Dilation in a Patient With Esophageal Stricture From Epidermolysis Bullosa Acquisita

Introduction: Epidermolysis bullosa acquisita (EBA) is a rare, chronic autoimmune blistering disorder characterized by autoantibodies targeting type VII collagen, a key component of anchoring fibrils in the basement membrane. While primarily affecting the skin (non-inflammatory type), the mucosal involvement (inflammatory type) is possible. Esophageal involvement is particularly rare & may lead to serious complications such as dysphagia & strictures. Case Description/Methods: A 42-year-old African American woman with a known diagnosis of EBA presented with progressive dysphagia to both solids & liquids, choking episodes & occasional inability to tolerate secretions for the past 6 months. Her diet was reduced to pureed soups & nutritional shakes. An initial endoscopy (EGD) revealed a severe esophageal stricture at 25 cm from the incisors, characterized by sloughing mucosa & inability to advance even a pediatric scope. She was treated with intermittent prednisone, which provided partial symptomatic relief & was started on dupilumab by dermatology. The patient noted a reduction in blistering of the skin, but dysphagia persisted. She subsequently underwent a second EGD & at 25 cm from the incisor, there was a circumferential stricture with a residual diameter of 5 mm. Using a guidewire, a through-the-scope balloon was advanced & the stricture was carefully dilated to 10 mm. The inflated balloon was used as a guide & the endoscope was carefully advanced to the stomach. After deflating the balloon, we found a tubular cast of the esophageal lumen, which was carefully retrieved with a biopsy forceps & submitted for histology. The histology revealed features of EBA involving the esophagus. The patient was started on a proton pump inhibitor twice daily & oral budesonide. She subsequently had severe odynophagia after the procedure, which improved after the third repeat dilation to 15 mm the following week. Discussion: Our first-ever case highlights a rare but significant complication of EBA: esophageal involvement leading to dysphagia. EBA-associated strictures may mimic other causes of esophageal narrowing and are often diagnosed only after extensive evaluation. Treatment typically includes immunosuppressants such as corticosteroids, azathioprine, or biologic agents. Gastroenterologists need to recognize the rare complication of esophageal cast during dilations for strictures in patients with EBA to decrease post-procedural complications and improve outcomes.

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Publication Details

Journal
The American Journal of Gastroenterology
Published
2025-10-01
DOI
https://doi.org/10.14309/01.ajg.0001145212.67652.01
Primary Topic
Autoimmune Bullous Skin Diseases
Type
article
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article

S4438 Esophageal Cast After Balloon Dilation in a Patient With Esophageal Stricture From Epidermolysis Bullosa Acquisita

Kwabena O. Asafo-Agyei, Kristi Randhawa, Sajid Ali, Bianca Afonso et al.
The American Journal of Gastroenterology
Autoimmune Bullous Skin Diseases
article

S4438 Esophageal Cast After Balloon Dilation in a Patient With Esophageal Stricture From Epidermolysis Bullosa Acquisita

Kwabena O. Asafo-Agyei, Kristi Randhawa, Sajid Ali, Bianca Afonso, Amit Hudgi, Sourabh Sharma, Subbaramiah Sridhar, Anam Herekar, Leonard Little
article en

Abstract

Introduction: Epidermolysis bullosa acquisita (EBA) is a rare, chronic autoimmune blistering disorder characterized by autoantibodies targeting type VII collagen, a key component of anchoring fibrils in the basement membrane. While primarily affecting the skin (non-inflammatory type), the mucosal involvement (inflammatory type) is possible. Esophageal involvement is particularly rare & may lead to serious complications such as dysphagia & strictures. Case Description/Methods: A 42-year-old African American woman with a known diagnosis of EBA presented with progressive dysphagia to both solids & liquids, choking episodes & occasional inability to tolerate secretions for the past 6 months. Her diet was reduced to pureed soups & nutritional shakes. An initial endoscopy (EGD) revealed a severe esophageal stricture at 25 cm from the incisors, characterized by sloughing mucosa & inability to advance even a pediatric scope. She was treated with intermittent prednisone, which provided partial symptomatic relief & was started on dupilumab by dermatology. The patient noted a reduction in blistering of the skin, but dysphagia persisted. She subsequently underwent a second EGD & at 25 cm from the incisor, there was a circumferential stricture with a residual diameter of 5 mm. Using a guidewire, a through-the-scope balloon was advanced & the stricture was carefully dilated to 10 mm. The inflated balloon was used as a guide & the endoscope was carefully advanced to the stomach. After deflating the balloon, we found a tubular cast of the esophageal lumen, which was carefully retrieved with a biopsy forceps & submitted for histology. The histology revealed features of EBA involving the esophagus. The patient was started on a proton pump inhibitor twice daily & oral budesonide. She subsequently had severe odynophagia after the procedure, which improved after the third repeat dilation to 15 mm the following week. Discussion: Our first-ever case highlights a rare but significant complication of EBA: esophageal involvement leading to dysphagia. EBA-associated strictures may mimic other causes of esophageal narrowing and are often diagnosed only after extensive evaluation. Treatment typically includes immunosuppressants such as corticosteroids, azathioprine, or biologic agents. Gastroenterologists need to recognize the rare complication of esophageal cast during dilations for strictures in patients with EBA to decrease post-procedural complications and improve outcomes.

The American Journal of GastroenterologyVol. 120(10S2)
Openalex Percentile: Top 54%
Autoimmune Bullous Skin Diseases
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