Case Presentation: A 29-year-old woman with poorly-controlled type I diabetes presented to the emergency department with nausea, vomiting, lethargy, and odynophagia for two days prior to admission. Her physical exam was notable for dry mucus membranes, ketotic breath odor, and mid-epigastric tenderness. Laboratory studies showed a blood glucose of 421 mg/dL with an anion gap of 21 and a β-Hydroxybutyric acid >9.0. She was admitted for diabetic ketoacidosis (DKA) attributed to medical non-adherence and treated supportively with insulin infusion, IV fluid resuscitation, and anti-emetics. Approximately 24 hours into her hospitalization, she experienced an episode of ~100 cc of frank hematemesis. She was taken urgently for upper endoscopy, which revealed class D esophagitis, sloughing of the friable mucosa, and adherent blood clots that could not be displaced with washing. No aggressive measures were taken due to the unhealthy appearance of the mucosa. The patient was treated with blood transfusion, bowel rest, IV proton pump inhibitor, and sucralfate. Repeat endoscopy 72 hours later demonstrated atrophic, scarred appearance of the esophageal mucosa with significant improvement from the previous procedure; biopsies were taken at this time. Pathology revealed severe extensive acute esophagitis with fibrinous ulcer debris and giant cells with polarizable material consistent with acute esophageal necrosis (AEN). H. pylori and fungal stains were negative. The patient’s diet was slowly advanced, and she was discharged home. Repeat endoscopy ~5 months subsequently demonstrated severe fibrosis and narrowing of the midesophagus (with an estimated luminal diameter of ~2.0 millimeters) requiring dilation under fluoroscopic guidance.

Discussion: Acute esophageal necrosis (also known as necrotizing esophagitis or “black esophagus”) is an endoscopic diagnosis characterized by circumferential hyperpigmentation or blackening of the esophageal mucosa. This phenomenon is most commonly observed in the area of the distal esophagus to the gastroesophageal junction. Acute esophageal necrosis was first recognized in 1990 by Goldenberg et al and has an estimated prevalence of ~0.2%. Incidence of AEN is highest in males in their sixth decade of life. The pathophysiology of AEN is thought to be multifactorial, related to local ischemia, increased exposure to acidic gastric contents, reduced esophageal motility, and diminished mucosal defense mechanisms in the setting of critical illness. The distal esophagus is considered a “watershed” area compared to the proximal and middle esophagus, making it vulnerable to ischemic injury. Potential complications of AEN include esophageal perforation, mediastinal abscess, esophageal stricture, superinfection, and death. Nasogastric tube placement is contraindicated in AEN given the friability of the esophageal mucosa and associated risk of esophageal perforation.

Conclusions: Acute esophageal necrosis is a rare, under-recognized cause of upper gastrointestinal (GI) bleeding associated with numerous clinical scenarios, including diabetic ketoacidosis, sepsis, alcohol intoxication, caustic ingestions, hypoperfusion, vasculopathy, and malignancy. Limited data exists regarding the clinical characteristics, endoscopic findings, and treatment of AEN. While AEN is an exceedingly rare disorder, the condition’s mortality approaches ~30%, suggesting that internists should include AEN in their differential for patients presenting with DKA and upper GI bleeds.

IMAGE 1: Acute Esophageal Necrosis Images