P3995 - Cicatricial Pemphigoid Presenting With Odynophagia

Claire Beamish, MD, Sara Zelman, MD, Mary Drinane, MD
Dartmouth Hitchcock Medical Center, Lebanon, NH

Introduction: Cicatricial pemphigoid (CP) is a rare, autoimmune blistering disease characterized by subepidermal blistering and linear IgG and/or IgA deposits along the basement membrane. Blisters primarily affect the mucous membrane, namely the oropharynx. In spite of extensive mucosal involvement, the esophagus is rarely affected. We present a patient diagnosed with cicatricial pemphigoid with atypical esophageal involvement.

Case Description/Methods: The patient is a 67-year-old female who initially presented to her local emergency department for redness in both eyes and a new erythematous patch on the nasal apex. The patient was diagnosed with suspected conjunctivitis and non-bullous impetigo for which she was treated with oral antibiotics. The patient subsequently developed a diffuse, pruritic rash and painful oral ulcers, limiting oral intake. The patient returned to the emergency department. Physical exam was notable for bilateral conjunctival erythema, singular erosion on the posterior hard palate, and diffuse tense bullae with erosions (Figures A, B). Lab values showed mild leukocytosis and elevated inflammatory markers. Patient was started on corticosteroids for suspicion of bullous pemphigoid vs. pemphigus vulgaris. Upper scope was performed, which revealed diffuse, friable mucosa with sloughing and ulcerations localized to the upper esophagus (Figure C). Esophageal biopsies showed granulation tissue with fibrinopurulent exudate consistent with ulcers. Patient then underwent skin biopsies. Hematoxylin and Eosin staining showed subepidermal splitting and intraepidermal vesicles; direct immunofluorescence confirmed linear IgG and IgA along the intraepidermal zone, confirming diagnosis of CP. Patient remained on corticosteroid taper with addition of daily dapsone with marked improvement.

Discussion: A high burden of mucosal involvement is a defining characteristic of the CP. However, esophageal involvement is rare, which is reflected in the scarcity of available literature. In the cases of CP with esophageal involvement, it typically presents in later stages with esophageal webs and esophageal strictures. This is the focus of most published case reports to date. This case is unique in that it captured esophageal involvement early in the disease course before any evidence of esophageal webs or strictures. This case highlights the need for a multidisciplinary approach in patients with suspected CP, particularly in those with otherwise unexplained dysphagia or odynophagia.



Disclosures:


Claire Beamish, MD, Sara Zelman, MD, Mary Drinane, MD. P3995 - Cicatricial Pemphigoid Presenting With Odynophagia, ACG 2024 Annual Scientific Meeting Abstracts. Philadelphia, PA: American College of Gastroenterology.