Endoscopic Nikolsky Sign.

A 54-year-old Asian woman with a history of pemphigus vulgaris presented with a 1-month history of sore throat, odynophagia, and dysphagia. She had been in remission for 2 years and had received corticosteroids and rituximab in the past. Upper endoscopy revealed superficial ulcers in the posterior oropharynx and aryepiglottic folds. The esophageal mucosa appeared normal on initial visualization, but multiple large bullae were then noted to develop with endoscopic contact (Figure A). This was consistent with endoscopic Nikolsky sign. Bullae were both fluid and blood filled, and ruptured with biopsy with sloughing off of the mucosa. Esophageal biopsies revealed focal acantholysis (Figure B). There were intracellular deposits of IgG (Figure C) and C3 in the squamous mucosa with positive direct immunofluorescence consistent with pemphigus vulgaris. The patient was started on prednisone and rituximab with progressive improvement and resolution of symptoms. Pemphigus vulgaris is an autoimmune disease characterized by blistering in the skin and mucosa. Esophageal involvement is rare, but should be considered in the differential diagnosis with endoscopic findings of bullae or exfoliation in the esophagus. Endoscopists must be aware of this entity and submit specimens in appropriate media (eg, Michel medium) for direct immunofluorescence staining to aid diagnosis.