Esophageal mucosal pemphigoid and esophageal stricture

Abstract

A 71-year old man came to our department because of odynophagia and dysphagia, with a history of bullous pemphigoid for 4 years. Several small ulcers were dispersed in the buccal mucosa, left cornea (A), and balanus skin. Two tense transparent blisters in both the anterior part of the chest and the posterior part of the left shoulder were found. The Nikolsky sign, dislodgement of the top layers of skin through the application of lateral pressure to intact the border of a blister, was negative. Endoscopy showed upper esophagus stricture and total esophageal transparent blisters (B, C), some of which were already bursting (D, E). Biopsy revealed slight chronic inflammations and squamous cell hyperplasia accompanied by eosinophilic infiltration. Laboratory studies demonstrated high levels of antibullous pemphigoid-180 antibodies 25.57 U/mL, immunoglobulin-G 18.5 g/L, immunoglobulin-A 4.150 g/L, and immunoglobulin-E 2550 IU/mL. The diagnosis of esophageal mucosal pemphigoid was made, and treatment with γ-globulin combined with prednisone was initiated. One week later, the patient was asymptomatic, and BP-180 antibodies decreased to 13.06 U/mL. No recurrence occurred after a follow-up time of 1.5 years.