S4696 Beyond the Skin: A Rare Case of Diffuse Upper Gastrointestinal Tract Involvement in Stevens-Johnson Syndrome (SJS)

Introduction: Steven-Johnson Syndrome (SJS) is a rare but life-threatening mucocutaneous reaction that is typically seen as an adverse reaction to certain drugs. It is characterized by diffuse erythema, bull’s eye rash, epidermal necrosis and sloughing of the mucocutaneous membranes. The lesions commonly affect the skin and oropharyngeal tracts. Gastrointestinal (GI) tract is a rare location for the lesion to affect but SJS involvement of this tract presents its own unique complications. The current case highlights 1 such unique presentation where SJS lesions had an extensive upper GI tract involvement, leading to severe upper GI bleeding. Case Description/Methods: A 74-year-old woman with a history of HIV and hypertension was admitted to the burn intensive care unit due to a diffuse bullous rash following recent exposure to vancomycin. She was diagnosed with SJS and was receiving supportive care. On hospital day 14, she developed severe hematemesis and hemoglobin dropped from 7.7 to 3.3 g/dL. The patient was stabilized on 4 units of RBCs and GI team was consulted. esophagogastroduodenoscopy (EGD) showed LA Grade D esophagitis in the middle and lower thirds of the esophagus (mucosal breaks involving 75% of the circumference). esophagogastroduodenoscopy revealed a 3 cm hiatal hernia, non-bleeding gastric ulcers and duodenal erosions at the bulb. The EGD findings were all consistent with SJS-related mucosal injury. Biopsies were deferred due to mucosal fragility. The patient’s bleeding had self resolved and no active bleeding was observed during or after the procedure. Conservative management for the patient was successful. Discussion: GI involvement can be observed in around 10% of patients with SJS/TEN. The mortality rate for SJS/TEN patients who present with GI involvement has been estimated at approximately 45%. Most common fatal GI complications are ulceration, bleeding, or perforation. The underlying pathologic mechanism involving immune-mediated epithelial apoptosis extends beyond the skin to mucosal linings. Prompt intensive care unit management and urgent EGD was essential for diagnosis and successful management. Further research should attempt to describe the frequency with which different GI segments are affected and their typical severity.