A Case Series of Esophageal Pemphigus Vulgaris—A Rare Cause of Dysphagia and Odynophagia

Background and Aim: Pemphigus vulgaris (PV) is characterized by intraepithelial blister formation, due to acantholysis by immunoglobulin-G antibodies against the keratinocyte cell surface of mucous membranes and skin. It may present with dysphagia, odynophagia, and rarely hematemesis, though majority are asymptomatic. Very few cases have been reported with esophageal involvement in PV. Esophageal involvement of PV is usually overlooked, as routine endoscopy is not performed for cutaneous manifestations. This study emphasizes on early identification through endoscopic evaluation and histological confirmation which is crucial for appropriate management.

Methods: Patients presenting with complaints of recurrent oral ulcers, dysphagia, odynophagia associated with or without bullous skin lesions, vomiting or weight loss were clinically evaluated. Three such patients were evaluated, upper gastrointestinal endoscopy was performed and biopsy of the affected tissue was taken and serologic testing was done for antibodies to desmoglein 1 and 3. The results showed characteristic findings of PV on endoscopy—(an almost normal appearance of the esophageal mucosa on initial passage of the endoscope, followed by the appearance of erosions and sheets of sloughed mucosa on withdrawal of the instrument suggestive of blistering disease) and histopathological examination—(histopathologic findings of a suprabasal cleft, cells with a ‘‘tombstone’’ appearance, and acantholysis are diagnostic of PV; positive direct and indirect immunofluorescence confirms the diagnosis) along with elevated titers of desmoglein antibodies.

Results: All three patients were treated after confirming the diagnosis of PV. Treatment consisted of oral steroids, azathioprine, proton pump inhibitors, sucralfate, and local anesthetic agents with good results and without complications.

Conclusion: Aim of reporting this article is physician and dermatologist’s awareness for PV, a rare cause in patients with recurrent or nonhealing oral and esophageal ulcers associated with odynophagia/dysphagia. High index of clinical suspicion is required for early identification of esophageal lesion of PV, which warrants significantly different management.

Publication History

Article published online:
22 April 2024