Esophageal Stricture Recalcitrant to Repeated Dilation in Complex Rheumatological Disease

We report a case of severe refractory esophageal strictures in the setting of rheumatological disease found to be secondary to isolated esophageal autoimmune bullous disease. She had a history of Sjogren syndrome and esophageal strictures with many previous dilations. After rheumatological workup, she was diagnosed with mixed connective tissue disease. Biopsy showed complement and immunoglobulin G deposition in the basement membrane consistent with bullous lupus or bullous pemphigoid. She had no cutaneous bullae and was diagnosed with isolated esophageal bullous disease. She required multiple dilatations over several months of treatment and was started on mycophenolate mofetil with clinical improvement.