A 61-year-old homeless man with a past medical history of chronic alcohol abuse was found deceased in his room at a shelter. He was found sitting on a toilet, which contained large amounts of black, tarry blood. During post-mortem examination, diffuse circumferential black-appearing distal esophageal mucosa with no involvement of the gastric mucosa was noted (Image 1). Esophageal histology showed diffuse basophilic and gangrenous mucosal necrosis with deposition of brown and black pigment (Image 2).
The correct answer is ...
Acute esophageal necrosis.
Acute esophageal necrosis (AEN) is a rare and deadly disease that can cause life-threatening esophageal hemorrhage. AEN is associated with poor nutritional status and multiple comorbidities, including alcoholic cirrhosis, binge drinking, diabetic ketoacidosis, and infection. AEN is characterized by circumferential black discoloration of the distal esophagus with underlying friable hemorrhagic tissue with a sharp transition to normal-appearing mucosa at the gastroesophageal junction. Histologically, severe esophageal mucosal necrosis is seen in AEN. Inflammation and partial destruction of adjacent muscle fibers and blood vessel thrombosis or occlusion may also be seen. The pathophysiology is hypothesized to result from a combination of esophageal mucosal injury and microvascular thrombosis.
Although esophageal variceal rupture can cause acute life-threatening gastrointestinal hemorrhage, especially in alcoholic patients, AEN presents with different gross and histologic findings. Large, dilated submucosal veins that protrude into the esophageal lumen and impart a mottled blue discoloration to the mucosa are seen on gross examination. Histologically, dilated, thin-walled veins in the submucosa are seen with overlying mucosal erosion and are more characteristic of esophageal varices.
Although the ingestion of caustic substances can cause severe esophageal damage with mucosal sloughing and black discoloration, this can be distinguished from AEN based on history as well as the presence of oropharyngeal burns.
Although focal or scattered black discoloration of the distal esophagus can be seen in esophageal melanosis, it is associated with underlying chronic esophagitis. Additionally, melanocytes are seen on histology rather than mucosal necrosis.
Edwin Lin, M.D., Ph.D.
Resident, Anatomic and Clinical Pathology
Catherine Hagen, M.D.
Senior Associate Consultant, Anatomic Pathology
Assistant Professor of Laboratory Medicine and Pathology
Mayo Clinic College of Medicine and Science