CASE REPORT A case of acute esophageal necrosis (black esophagus) in a young man with Down syndrome Maria Raffaella Ambrosio • Bruno Jim Rocca • Maria Grazia Mastrogiulio • Alia Lutfi • Stefano Lazzi • Sergio Antonio Tripodi Received: 5 February 2013 / Accepted: 28 August 2013 / Published online: 19 September 2013 Ó The Japan Esophageal Society and Springer Japan 2013 Abstract 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 repar- ative mechanisms present in debilitating diseases. We describe the case of an 18-year-old man affected by Down syndrome, presenting with a streptococcus b-hemolytic group A infection of the upper airways. Although the patient was intensively treated with antibiotics, he devel- oped 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 esoph- ageal 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. Keywords Black esophagus Á Down syndrome Á Streptococcus infection Introduction Acute esophageal necrosis (AEN), commonly referred to as ‘black esophagus’, is a rare entity whose cause is still unclear. It may originate from a combination of an ische- mic insult due to hemodynamic alteration with low-flow states, a corrosive injury by gastric contents in the setting of esophageal gastroparesis and gastric outlet obstruction, and a decreased function of mucosal barrier systems and reparative mechanisms in debilitating diseases [1]. The onset of this lesion is usually dramatic and the presenting symptom is generally hematemesis. The long-term prog- nosis is poor: more than one-third of patients die and others develop esophageal stenosis [2]. The hallmark of this syndrome is a circumferential mucosal discoloration of the distal esophagus, which may extend proximally for a variable length while it abruptly stops at the esophageal-gastric junction. It is characterized by mucosal necrosis, hence the black color of the lesion, and inflammation, in the absence of evident causative agents [3, 4]. What is known about AEN is mainly based on case reports, which illustrate the endoscopic appearance and the clinical pattern and course of the syndrome, but do not detail its gross and histopathological findings, in fact it has been described only twice in the pathology literature [5]. Here, we illustrate an autoptic case of AEN in a young man with Down syndrome. Case report An 18-year-old man affected by Down syndrome presented with severe cough, tonsillitis, fatigue and fever. Physical examination revealed a moderately distended abdomen, M. R. Ambrosio (&) Á B. J. Rocca Á M. G. Mastrogiulio Section of Pathology, Department of Medical Biotechnology, University of Siena, Via delle Scotte 6, 53100 Siena, Italy e-mail: maradot@libero.it A. Lutfi Lega Tumori Senese, Siena, Italy S. Lazzi Á S. A. Tripodi Section of Pathology, Azienda Ospedaliera Universitaria Senese, Viale Bracci, 1, 53100 Siena, Italy 123 Esophagus (2014) 11:136–139 DOI 10.1007/s10388-013-0396-2