Hrev_master Emergency Care Journal 2024; volume 20:12738 [Emergency Care Journal 2024; 20:12738] [page 109] A dark and black esophagus Flavio Scarilli, Davide Tizzani Emergency Medicine 2, Ospedale San Giovanni Bosco, Torino, Italy Question What disorder arises with this dark and black esophagus? 1. Caustic ingestion 2. Melanoma 3. Gurvits syndrome 4. Acanthosis nigricans Answer The correct diagnosis is Gurvits Syndrome also known as Acute Esophageal Necrosis (AEN) or “black esophagus” due to the asso- ciated colour change.1 Firstly described in 1990,2 AEN is a rare syndrome with a male preponderance, with an incidence four times greater than that for women and a peak incidence during the sixth decade of life.3 The prevalence is only 0.001~0.2%, while its mortality rate is up to 32%.4 Most of the cases are fatal due to per- foration, stricture, bleeding, and infections. The etiology is not completely clear. According to a “2 hit hypothesis”, AEN can arise when an acute event occurs and causes local hypoperfusion and subsequently necrosis.5 Combined effects of vascular compro- mise, impaired defence mechanism of the esophagus, and expo- sure to gastric acids all contribute to AEN.6 The major risk factors include male sex, chronic diseases (diabetes mellitus with, hyper- A 70-year-old Italian man presented to our emergency depart- ment for dyspnea and confusion. He was affected by diabetes mellitus type 2, but he stopped his medication 10 days before. At admission, his vital signs were blood pressure of 60/50 mmHg, heart rate of 130 bpm, peripheral oxygen saturation of 94% in the Venturi Mask FiO2 40%, respiratory rate of 40/min, and body temperature of 36°C. Physical examination revealed hypop- erfusion with a high Mottling Score and dark stools with a posi- tive hydrogen peroxide reaction. He denied abdominal pain, nau- sea, and vomiting. Laboratory findings showed leucocytosis (White Blood Cells, WBC, 31,580/mm3; N 91.6%), Hemoglobin (Hb) 14.5 g/dL, Hematocrit Test (Hct) 49%, hyperglycaemia (>700 mg/L), and normal coagulation time. Arterial blood gas documented a metabolic acidosis with pH 6.95, Partial Pressure of Carbon Dioxide (pCO2) 27.5 mmHg, HCO3 7.1 mmol/L, lactate 8.14 mmol/L (normal value <2), and elevated anion gap metabolic. He was first treated with IV therapy as follows omeprazole 80 mg, tranexamic acid 1 g, Ringer acetate 1000 cc, sodium bicarbonate 8.4% 100 mL, and magnesium sulfate 2 g. Then, a continuous IV infusion of Ringer 150 mL/h and omeprazole 8 mg/h was started. An Esophagogastroduodeno- scopy (EGD) was arranged and showed circumferential blacken- ing of the distal half of the esophagus. Correspondence: Flavio Scarilli, Emergency Medicine 2, Ospedale San Giovanni Bosco, Torino, Italy. E-mail: flavio19sx@gmail.com Key words: black esophagus, Gurvits, ketoacidosis, diabetes, AEN. Contributions: all the authors approved the final version of the manu- script, agreed to be held accountable for all aspects, and equally con- tributed to the work. Conflicts of interest: the authors declare no potential conflict of interest. Funding: none. Availability of data and materials: all data underlying the findings are fully available upon reasonable request to the Corresponding Author. Ethics approval and consent to participate: not necessary. The patient provided consent for access to medical records at the time of admission. Received: 21 June 2024. Accepted: 3 July 2024. Early view: 29 July 2024. This work is licensed under a Creative Commons Attribution 4.0 License (by-nc 4.0). ©Copyright: the Author(s), 2023 Licensee PAGEPress, Italy Emergency Care Journal 2024; 20:12738 doi:10.4081/ecj.2023.12738 Publisher's note: all claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organiza- tions, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its man- ufacturer is not guaranteed or endorsed by the publisher. Non -co mmerc ial us e o nly tension, renal failure, vascular diseases), alcohol use disorder and/or recreational drug use (e.g., cocaine), malnutrition, and can- cer.1 In a recently published systemic review, alcohol and hyper- tension were common comorbidities, and the overall mortality rate amounted to 32%.7 The most common presenting symptoms are hematemesis, shock, melena, abdominal, or substernal pain. Diagnosis is made by EGD that shows circumferential dark pig- mentation of the distal third of the esophagus, which is relatively less vascular, and necrosis that stops abruptly at the gastroe- sophageal junction. Colour change depends on the location of the lesion, degree of necrosis, and pattern of necrosis. Biopsy is rec- ommended but not required for a definite diagnosis. AEN has no specific treatment. Antibiotics, antifungals and nutritional support should be considered on an individual basis. Candidiasis may occur in conjunction with AEN, whilst it is not thought to be causative, treatment is considered prudent given the poor progno- sis associated with this condition.8The patient was admitted to the High Dependency Unit and was treated with IV fluid and amine support, piperacillin-tazobactam, oxacillin, fluconazole, proton pump inhibitors, and total parental nutrition. One week later, an EGD showed a lighter esophageal mucosa with some white exu- date spots (Figure 1). After two weeks, EGD documented fibrin deposition associated with reepithelialisation (Figure 2). The patient was released after two more weeks, when an EGD showed total resolution of AEN with no indication of stricture development or perforation (Figure 3). References 1. Gurvits GE, Shapsis A, Lau N, et al. Acute esophageal necro- sis: a rare syndrome. J Gastroenterol 2007;42:29-38. 2. Goldenberg SP, Wain SL, Marignani P. Acute necrotizing esophagitis. Gastroenterology 1990;98:493-6. 3. Gurvits GE. Black esophagus: acute esophageal necrosis syn- drome. World J Gastroenterol 2010;16:3219-25. 4. Akaishi R, Taniyama Y, Sakurai T, et al. Acute esophageal necrosis with esophagus perforation treated by thoracoscopic subtotal esophagectomy and reconstructive surgery on a sec- ondary esophageal stricture: a case report. Surg Case Rep 2019;5:73. 5. Ziegler PE, Rosario Lora D, DeMeo M. An unusual case of black esophagus. ACG Case Rep J 2023;10:e01202. 6. Averbukh LD, Mavilia MG, Gurvits GE. Acute Esophageal Necrosis: a case series. Cureus 2018;10:e2391. 7. Abdullah HM, Ullah W, Abdallah M, et al. Clinical presenta- tions, management, and outcomes of acute esophageal necro- sis: a systemic review. Expert Rev Gastroenterol Hepatol 2019;13:507-14. 8. Beg S, Rowlands D. Acute Oesophageal Necrosis: a case report and review of the literature. BJMP 2015;8:a829 Images in emergency Figure 1. EGD showing a lighter esophageal mucosa with some white exudate spots. Figure 2. EGD documenting fibrin deposition associated with reepithelialisation. Figure 3. EGD after two weeks showing total resolution of AEN with no indication of stricture development or perforation. [page 110] [Emergency Care Journal 2024; 20:12738] Non -co mmerc ial us e o nly