Ketosis-prone Diabetes Presenting with Acute Esophageal Necrosis or
“Black Esophagus”: An Intriguing New Clinical Association

Riccardo   Maria   Pollis; Damiano      Furlanetto; Elena      Pagin; Carla      Scaroni; Mattia      Barbot; Giacomo      Voltan

doi:10.2174/0118715303279019231127065331

Abstract

Background: Ketosis-prone diabetes (KPD) is an intermediate subtype of diabetes mellitus, usually affecting Afro-American adults, presenting with diabetic ketoacidosis (DKA), without the classic phenotype of autoimmune type 1 diabetes. Patients require insulin therapy at onset for the acute decompensation, then usually remain insulin-free for prolonged periods with diet alone or with other antidiabetic drugs. DKA can be rarely complicated by upper gastrointestinal bleeding and mucosal necrosis, a severe complication named acute esophageal necrosis (AEN) burdened by high mortality. The association of KPD presenting with DKA complicated by AEN is here reported for the first time, to the knowledge of the authors, in the medical literature.

Case Presentation: Here we report an interesting case of middle-aged African woman, newly diagnosed with KPD, presenting with DKA hematemesis. The patient was first treated at Intensive Care Unit for the ketoacidosis with intravenous fluids combined with continuous insulin infusion, and then switched to subcutaneous regimen. At the same time, esophagogastroduodenoscopy (EGD) was performed to diagnose acute esophageal necrosis, which was promptly managed with proton pump inhibitors infusion, fasting, and parenteral nutrition. After the correct clinical evaluation, the patient was switched to oral antidiabetic and basal insulin at discharge and an EGD follow-up was scheduled.

Conclusions: KPD remains an under-recognized and under-diagnosed type of diabetes which can present as DKA. Since DKA could be a possible trigger of AEN, a rare but potentially lifethreatening condition, that clinicians should be aware of, in patients presenting with upper gastrointestinal bleeding and ketoacidosis. The prompt management and classification of DKA, combined with the EGD execution for early AEN diagnosis and follow-up, is essential.

« Previous

[1]
Balasubramanyam, A. Syndrome of ketosis-prone diabetes. Trans. Am. Clin. Climatol. Assoc.,  2019, 130, 145-155.
 [PMID:  31516178]

[2]
Mauvais-Jarvis, F.; Sobngwi, E.; Porcher, R. Riveline,] J.P.; Kevorkian, J.P.; Vaisse, C.; Charpentier, G.; Guillausseau, P.J.; Vexiau, P.; Gautier, J.F. Ketosis-prone] type 2 diabetes in patients of sub-Saharan African origin: Clinical pathophysiology and natural history of beta-cell dysfunction and insulin resistance. Diabetes,  2004, 53(3), 645-653.
 [http://dx.doi.org/10.2337/diabetes.53.3.645] [PMID:  14988248]

[3]
Lebovitz, H.E.; Banerji, M.A. Ketosis-prone diabetes (Flatbush Diabetes): An emerging worldwide clinically important entity. Curr. Diab. Rep.,  2018, 18(11), 120.
 [http://dx.doi.org/10.1007/s11892-018-1075-4] [PMID:  30280274]

[4]
Oli, J.M. Remittant diabetes mellitus in Nigeria. Trop. Geogr. Med.,  1978, 30(1), 57-62.
 [PMID:  675828]

[5]
Banerji, M.A.; Chaiken, R.L.; Huey, H.; Tuomi, T.; Norin, A.J.; Mackay, I.R.; Rowley, M.J.; Zimmet, P.Z.; Lebovitz, H.E. GAD antibody negative NIDDM in adult black subjects with diabetic ketoacidosis and increased frequency of human leukocyte antigen DR3 and DR4. Flatbush diabetes. Diabetes,  1994, 43(6), 741-745.
 [http://dx.doi.org/10.2337/diab.43.6.741] [PMID:  8194658]

[6]
Umpierrez, G.E.; Smiley, D.; Kitabchi, A.E. Narrative review: Ketosis-prone type 2 diabetes mellitus. Ann. Intern. Med.,  2006, 144(5), 350-357.
 [http://dx.doi.org/10.7326/0003-4819-144-5-200603070-00011]

[7]
Gaba, R.; Balasubramanyam, A. Covid-19: A new cause of “provoked” A-β+ Ketosis-Prone Diabetes. J. Diabetes Complications,  2022, 36(4), 108147.
 [http://dx.doi.org/10.1016/j.jdiacomp.2022.108147] [PMID:  35153127]

[8]
Tan, H.; Wang, C.; Yu, Y. Ketosis-prone diabetes mellitus in an obese adolescent. A case report. Medicine,  2019, 98(25), e16076.
 [http://dx.doi.org/10.1097/MD.0000000000016076]

[9]
Goldenberg, S.P.; Wain, S.L.; Marignani, P. Acute necrotizing esophagitis. Gastroenterology,  1990, 98(2), 493-496.
 [http://dx.doi.org/10.1016/0016-5085(90)90844-Q] [PMID:  2295407]

[10]
Gurvits, G.E.; Shapsis, A.; Lau, N.; Gualtieri, N.; Robilotti, J.G. Acute esophageal necrosis: A rare syndrome. J. Gastroenterol.,  2007, 42(1), 29-38.
 [http://dx.doi.org/10.1007/s00535-006-1974-z] [PMID:  17322991]

[11]
Gurvits, G.E. Black esophagus: Acute esophageal necrosis syndrome. World J. Gastroenterol.,  2010, 16(26), 3219-3225.
 [http://dx.doi.org/10.3748/wjg.v16.i26.3219] [PMID:  20614476]

[12]
Dias, E.; Santos-Antunes, J.; Macedo, G. Diagnosis and management of acute esophageal necrosis. Ann. Gastroenterol.,  2019, 32(6), 529-540.
 [http://dx.doi.org/10.20524/aog.2019.0418] [PMID:  31700229]

[13]
Gurvits, G.E.; Cherian, K.; Shami, M.N.; Korabathina, R.; El-Nader, E.M.A.; Rayapudi, K. Black esophagus: New insights and multicenter international experience. Dig. Dis. Sci.,  2014, 60(2), 444-453.

[14]
Katz, M.A. Hyperglycemia-induced hyponatremia-calculation of expected serum sodium depression. N. Engl. J. Med.,  1973, 289(16), 843-844.
 [http://dx.doi.org/10.1056/NEJM197310182891607] [PMID:  4763428]

[15]
Graziani, A.; Pollis, R.M.; Maria, B.B.; Scaroni, C.; Sabbadin, C. Ketosis-prone diabetes and hypogonadism: A new clinical association to be aware of? Endocr. Metab. Immune Disord. Drug Targets,  2023, 23(12), 1552-1556.
 [http://dx.doi.org/10.2174/1871530323666230621114503]

[16]
Jerzy, L.; Artur, K.; Felisiak-Golabek, A. Sebastian,] Z.; Piotr, W.; Marek, M.; Jaroslaw, W.; Justyna, S.; Markku, M. Primary malignant melanoma of esophagus: Clinicopathologic characterization of 20 cases including molecular genetic profiling of 15 tumors. Mod. Pathol.,  2019, 32(7), 957-966.

[17]
Kimball, M.W. Pseudomelanosis of the esophagus. Gastrointest. Endosc.,  1978, 24(3), 121-122.
 [http://dx.doi.org/10.1016/S0016-5107(78)73478-4] [PMID:  305381]

[18]
Day, A.; Sayegh, M. Acute oesophageal necrosis: A case report and review of the literature. Int. J. Surg.,  2010, 8(1), 6-14.
 [http://dx.doi.org/10.1016/j.ijsu.2009.09.014] [PMID:  19800431]

[19]
Altenburger, D.L.; Wagner, A.S.; Li, S.; Garavaglia, J. A case of black esophagus with histopathologic description and characterization. Arch. Pathol. Lab. Med.,  2011, 135(6), 797-798.
 [http://dx.doi.org/10.5858/2010-0128-C.1] [PMID:  21631276]

[20]
Burtally, A.; Gregoire, P. Acute esophageal necrosis and low-flow state. Can. J. Gastroenterol.,  2007, 21(4), 245-247.
 [http://dx.doi.org/10.1155/2007/920716]

[21]
Ghoneim, S.; Shah, A.; Dalal, S.; Landsman, M.; Kyprianou, A. Black esophagus in the setting of diabetic ketoacidosis: A rare finding from our institution. Case Rep. Gastroenterol.,  2019, 13(3), 475-480.
 [http://dx.doi.org/10.1159/000504434] [PMID:  31824236]

[22]
Shah, A.R.; Landsman, M.; Waghray, N. A dire presentation of diabetic ketoacidosis with “black esophagus”. Cureus,  2019, 11(5), e4761.
 [http://dx.doi.org/10.7759/cureus.4761] [PMID:  31363442]

[23]
Uhlenhopp, D.J.; Pagnotta, G.; Sunkara, T. Acute esophageal necrosis: A rare case of upper gastrointestinal bleeding from diabetic ketoacidosis. Clin. Pract.,  2020, 10(2), 1254.
 [http://dx.doi.org/10.4081/cp.2020.1254] [PMID:  32670536]

[24]
Vien, L.P.; Yeung, H.M. Acute esophageal necrosis] (Gurvits Syndrome): A rare complication of diabetic] ketoacidosis in a critically ill patient. Case Rep. Med.,  2020, 2020, 1-3.
 [http://dx.doi.org/10.1155/2020/5795847] [PMID:  32180810]

[25]
Kitawaki, D.; Nishida, A.; Sakai, K. Gurvits syndrome: A case of acute esophageal necrosis associated with diabetic ketoacidosis. BMC Gastroenterol.,  2022, 22(1), 277.

[26]
Rigolon, R.; Fossà, I.; Rodella, L.; Targher, G. Black esophagus syndrome associated with diabetic ketoacidosis. World J. Clin. Cases,  2016, 4(2), 56-59.
 [http://dx.doi.org/10.12998/wjcc.v4.i2.56] [PMID:  26881192]

[27]
Tanaka, S.; Fujishiro, M.; Ichijima, R.; Kohno, G.; Abe, M.; Ishihara, H. Acute esophageal necrosis after cellulitis in an obese patient with diabetes mellitus. J. Diabetes Investig.,  2020, 11(1), 250-252.
 [http://dx.doi.org/10.1111/jdi.13104] [PMID:  31218806]

[28]
Carl, G.F.; Hoffman, W.H.; Passmore, G.G.; Truemper, E.J.; Lightsey, A.L.; Cornwell, P.E.; Jonah, M.H. Diabetic ketoacidosis promotes a prothrombotic state. Endocr. Res.,  2003, 29(1), 73-82.
 [http://dx.doi.org/10.1081/ERC-120018678] [PMID:  12665320]

[29]
Carr, M.E. Diabetes mellitus. J. Diabetes Complications,  2001, 15(1), 44-54.
 [http://dx.doi.org/10.1016/S1056-8727(00)00132-X] [PMID:  11259926]

[30]
Vivino, G.R.; Crofton, N.A.; Mussarat, S. Diabetic ketoacidosis and acute mesenteric ischemia in adults: An underreported association. Cureus,  2022, 14(9), e29053.
 [http://dx.doi.org/10.7759/cureus.29053] [PMID:  36249626]

[31]
Dhatariya, K.K.; Glaser, N.S.; Codner, E. Umpierrez,] G.E. Diabetic ketoacidosis. Nat. Rev. Dis. Primers,  2020, 6(1), 40.
 [http://dx.doi.org/10.1038/s41572-020-0165-1] [PMID:  32409703]

[32]
Delaloye, J.; Calandra, T. Invasive candidiasis as a cause of sepsis in the critically ill patient. Virulence,  2014, 5(1), 161-169.
 [http://dx.doi.org/10.4161/viru.26187] [PMID:  24157707]

[33]
Wiggins, A.; Reddy, R. An atypical source of persistent fungemia in the intensive care unit. Am. J. Case Rep.,  2022, 23, e936223.
 [http://dx.doi.org/10.12659/AJCR.936223] [PMID:  35644934]

[34]
Elzouki, A-N.; Eledrisi, M.S. Management of diabetic ketoacidosis in adults: A narrative review. Saudi J. Med. Med. Sci.,  2020, 8(3), 165-173.
 [http://dx.doi.org/10.4103/sjmms.sjmms_478_19] [PMID:  32952507]

Rights & Permissions Print Cite

Journal Information

For Authors

For Editors

For Reviewers

Explore Articles

Open Access

Open Access Articles

For Visitors

DOI https://dx.doi.org/10.2174/0118715303279019231127065331	Print ISSN 1871-5303
Publisher Name Bentham Science Publisher	Online ISSN 2212-3873

Endocrine, Metabolic & Immune Disorders - Drug Targets

Ketosis-prone Diabetes Presenting with Acute Esophageal Necrosis or “Black Esophagus”: An Intriguing New Clinical Association

Abstract Play Pause

Related Journals

Related Books

Abstract