P1859 - Pemphigus Vulgaris and Esophageal Dissecans: A Rare Association Explored

Kazi Khan, MD¹, Mohammad Aasim Khan, MBBS², Abdulaziz Siddiqui, MD¹, Matthew Kobeszko, MD, MBA, MSc¹, Aboud Affi, MD¹, Biana Leybishkis, MD^1
1Aurora Healthcare, Milwaukee, WI; ²Khyber Medical College & Advocate Health Aurora St. Luke’s Medical Center, Milwaukee, WI

Introduction: Esophageal Dissecans Superficialis (EDS) is a rare condition characterized by the separation of the esophageal mucosa and submucosa, leading to erosive esophagitis. This case report highlights the uncommon association between EDS and Pemphigus Vulgaris (PV), a bullous autoimmune disease. We present the clinical features, diagnostic challenges, and treatment approach in a patient with concurrent PV and EDS, shedding light on this intriguing connection.

Case Description/Methods: An 82-year-old man with a medical history of atrial fibrillation and follicular lymphoma presented with mucosal erythema, dysphagia, dysphonia, and reduced oral intake. Initial evaluations suggested various possibilities, including oral thrush and hairy-cell leukoplakia (A), but workup failed to provide a definitive diagnosis. Esophagogastroduodenoscopy (EGD) revealed EDS with severe erosive esophagitis (B) and erythematous vocal cords (C). Biopsies were negative for fungal, malignant, and autoimmune etiologies. Further investigation led to the identification of pemphigus vulgaris via autoantibody panel testing. The patient had an excellent response to pulse-dose 1mg/kg methylprednisolone and transitioned to oral prednisone taper with outpatient rituximab infusions. Due to non-adherence, he currently continues his prednisone taper 3 months later without readmission for dysphagia symptoms and follows with dermatology.

Discussion: EDS, characterized by the separation of esophageal mucosal layers, is a rare condition with diverse etiological associations. While the exact pathogenesis remains uncertain, there is evidence linking EDS to bullous autoimmune diseases such as PV. The presence of esophageal symptoms in patients with PV ranges from 46-87%. Diagnosis of EDS in the context of PV can be challenging, requiring a multidisciplinary approach and careful consideration of clinical features, endoscopic findings, and autoimmune panel testing. Timely recognition and appropriate management with immunosuppressive agents can lead to significant improvement in symptoms and endoscopic outcomes.

Our report emphasizes the rare association between pemphigus vulgaris and esophageal dissecans. It underscores the importance of considering autoimmune etiologies in patients presenting with esophageal dissecans, particularly in cases with atypical features or unresponsiveness to conventional therapies. Reporting this rare association will facilitate improved diagnostic accuracy and tailored treatment strategies in the future.




Disclosures:


Kazi Khan, MD¹, Mohammad Aasim Khan, MBBS², Abdulaziz Siddiqui, MD¹, Matthew Kobeszko, MD, MBA, MSc¹, Aboud Affi, MD¹, Biana Leybishkis, MD¹. P1859 - Pemphigus Vulgaris and Esophageal Dissecans: A Rare Association Explored, ACG 2023 Annual Scientific Meeting Abstracts. Vancouver, BC, Canada: American College of Gastroenterology.