Introduction: Esophagitis Dissecans Superficialis (EDS), characterized by sloughing of the esophageal epithelial lining, is a rare endoscopic finding. Causes include medications, hot beverages, celiac disease, collagen vascular diseases, idiopathy, and autoimmune bullous dermatoses (ABDs), such as Bullous Pemphigoid (BP) and Pemphigus Vulgaris (PV). Symptoms include dysphagia, odynophagia, and heartburn; vomiting of mucosal casts and hematemesis rarely occurs. We present a patient with BP and EDS with upper gastrointestinal bleeding (UGIB). Case: A 54 year-old female with a medical history of end stage renal disease, due to hypertension, status post renal allograft transplant, with graft failure on hemodialysis, presented to the emergency department (ED) with hematemesis. For 2 weeks she had worsening oral ulcerations and hematuria and 6 hours prior, she had severe nausea, burning chest pain, and 4-5 episodes of bright red vomitus. With 1 episode, she saw a “½ foot” long grayish-white soft tissue-like material. In the ED, she was hypertensive and tachycardic, with cutaneous blisters at various stages of healing. Hemoglobin was 7.1 g/dL, from 8.1 g/dL 2 weeks prior. An urgent EGD revealed mucosal sloughing involving the entire esophagus and posterior oro-pharynx, with associated clots and bleeding. Biopsy was deferred. Further history indicated she had a 4 month history of skin blisters and oral ulcerations, occurring after her transplant immunosuppresants were withdrawn, and was diagnosed with BP. Workup revealed a BP180 IgG > 150 units (normal < 9) and skin biopsy showed linear basement membrane pattern staining for IgG and C3 antibody. Her esophageal findings were attributed to her underlying BP. With PPI therapy, high-dose methylprednisolone, and mycophenolate mofetil she had significant improvement with resolution of hematemesis. A repeat endoscopic evaluation was declined. Discussion: Hematemesis with vomiting of esophageal mucosal casts is a rarely reported presentation of EDS. EDS has many potential causes, and recently, the pathophysiology and relationship between EDS and ABDs such as BP and PV have been increasingly acknowledged in the literature. Conclusion: EDS should be considered in patients who present with hematemesis and/or report of vomiting esophageal mucosal casts; endoscopic findings of exfoliative esophagitis support the diagnosis. These clinical findings should lead to consideration of ABDs as an etiology, to guide appropriate management decisions.
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