1791 Esophageal Nikolsky’s Sign: A Rare Finding in a Patient With Bullous Pemphigoid

Abstract

INTRODUCTION: Bullous pemphigoid (BP) is a chronic relapsing autoimmune blistering disease characterized by circulating antibodies directed against specific epitopes of hemidesmosomes involved in anchoring the epidermis to the dermal layer. We report a case of acute onset esophageal bullae identified in a patient with active skin BP seen only upon withdrawal of the upper endoscope, not present on insertion. This sign is analogous to Nikolsky’s sign where pressure or shearing results in formation of new bullae. CASE DESCRIPTION/METHODS: 57-year-old Caucasian female with Type II DM, BP, CKD stage 3a presented to hospital for 5-6 episodes of melanotic stools for two days. She was diagnosed in September 2017 with BP by skin biopsy and direct immunofluorescence (Figure 1). At the time of presentation, she was normotensive, afebrile, heart rate 102 beats/min and respiratory rate of 14 breaths/min. Skin examination revealed tense bullae on upper extremities (Figure 2). Abdominal exam was notable for tenderness to palpate in the epigastric region. Digital rectal exam revealed melena. Laboratory findings showed hemoglobin 5.7 mg/dl, hematocrit 17.5 mg/dl. The patient received 1 unit of packed RBC, started on a proton pump inhibitor infusion and continued on mycophenolate with oral prednisone. She underwent an upper gastrointestinal endoscopy which revealed segments of sloughing mucosa in the esophagus. Additionally, a large bulla ranging from 20 to 25 cm was seen only upon withdrawal of the endoscope and not during initial insertion (Figure 3). She was discharged home on a high dose oral proton pump inhibitor, mycophenolate, prednisone, and topical steroid. DISCUSSION: Bullous Pemphigoid (BP) is the most common type of acute bullous dermatoses (ABD) affecting older adults without gender predilection. Esophageal manifestations of BP are rare and range from dysphagia, hematemesis or melena. Nikolsky sign is a specific skin finding of autoimmune blistering diseases but is only seen in 56% of the cases. The use of esophagogastroduodenoscopy can be challenging in these patients because of bullae formation after light contact between esophageal mucosa and the endoscope. Only gentle advancement and withdrawal of the endoscope is advised to prevent adverse events such as blood blisters, frank bleeding, or perforation. In cases of significant gastrointestinal bleeding and/ or hemodynamic instability, therapeutic endoscopy can be used to achieve hemostasis.