S4374 Esophageal Bullous Pemphigoid

Introduction: Bullous pemphigoid (BP) is an autoimmune condition which typically affects adults over 60 years old and involves IgG antibodies against hemidesmosomes in the basement membrane of the skin. Esophageal BP is rare and can occur in 4% of patients, typically manifesting as gastrointestinal bleeding, dysphagia or odynophagia. Case Description/Methods: An 82-year-old man with a history of cerebrovascular accident, dementia, and gastroesophageal reflux disease, who initially presented to his primary care provider with dermatitis on the dorsum of his feet bilaterally. He improved with antifungals and corticosteroids but subsequently presented 2 months later due to 2 weeks of hemoptysis. A laryngoscopy demonstrated multiple dilated vessels in the pharynx and fresh blood pooling around the esophageal orifice. Esophagogastroduodenoscopy revealed ulceration in the posterior hypopharynx, desquamation of the proximal esophagus, severe distal esophagitis, and erythema in the gastric cardia and fundus. Esophageal biopsies showed patchy chronic inflammation and gastric biopsies confirmed H. pylori infection. He was treated with amoxicillin and omeprazole with eradication. He subsequently developed taut blisters involving the arms, legs and glans penis. A diagnosis of BP was confirmed with a punch biopsy of the skin which revealed linear depositions of IgG and C3 along the BM zone, positive anti-BP 180 antigen, and a positive immunobullous panel. He received 1 month of prednisone 40 mg daily followed by mycophenolate 1000 mg in the morning and 500 mg at night. Given recurrent blisters, his mycophenolate dosage was increased to 1000 mg BID and an additional course of prednisone was prescribed which then resulted in remission. Discussion: BP is associated with blister formation due to the disadhesion of the subepidermal layer of the skin. Oral mucous membranes may be involved. While esophageal BP is rare, it may manifest as strictures, ulcers or hemorrhagic bullae. Esophagogastroduodenoscopy may be challenging due to the formation of bullae and mucosal sloughing with contact. The diagnosis of BP is typically confirmed with a biopsy of the lesion and direct immunofluorescence. Treatment involves systemic steroids. Azathioprine and rituximab have been utilized as a steroid-sparing therapy. In cases of significant gastrointestinal bleeding, argon plasma coagulation, which does not require contact with mucosa, may be used for treatment. Clinicians should include BP in the differential in a patient with desquamative esophageal lesions and cutaneous abnormalities.