A case of acute esophageal necrosis (black esophagus) in a young man with Down syndrome

Acute esophageal necrosis, commonly referred to as 'black esophagus', is a rare clinical entity arising from a combination of ischemic insult, corrosive injury and decreased function of mucosal barrier systems and reparative mechanisms present in debilitating diseases. We describe the case of an 18-year-old man affected by Down syndrome, presenting with a streptococcus β-hemolytic group A infection of the upper airways. Although the patient was intensively treated with antibiotics, he developed a streptococcal toxic shock-like syndrome and died 5 days after admission. At autopsy, circumferential black discoloration of the esophageal mucosa that extended along the entire esophagus and ended abruptly at the esophageal-gastric junction was found. Neither ulceration nor esophageal perforation were present. Black esophagus is well known to the gastroenterology community, whereas it has been described only twice in the pathology literature. To the best of our knowledge, this is the first case ever reported complicating a streptococcal infection. © 2013 The Japan Esophageal Society and Springer.