Keywords
Acanthosis nigricans
Cyclophosphamide
Hyperinsulinemia
Continuous subcutaneous insulin infusion system
CSII
How to Cite
Abstract
Introduction: Type B insulin resistance syndrome (TBIRS) is an immune-mediated disorder characterized by severe acanthosis nigricans and difficult-to-control diabetes mellitus. This condition represents a complex clinical challenge.
Objective: To describe the case of a patient compatible with Type B insulin resistance syndrome, highlighting the challenges in glycemic control and the response to immunosuppressive therapy combined with a hybrid continuous subcutaneous insulin infusion system.
Case Presentation: A 57-year-old woman with a history of limited cutaneous systemic sclerosis and type 2 diabetes mellitus presented with severe insulin resistance, acanthosis nigricans, and markedly elevated blood glucose levels. Anti-insulin receptor antibodies were consistently negative. The patient received multiple treatments, including plasmapheresis, immunosuppressive therapy, and continuous subcutaneous insulin infusion using a MiniMed® 780G system, resulting in improved continuous glucose monitoring metrics and a reduction in daily insulin requirements.
Discussion: This case suggests a possible association between systemic sclerosis and Type B insulin resistance syndrome despite persistently negative anti-insulin receptor antibodies. A false-negative result may be explained by prior immunomodulatory treatments. The combination of immunosuppressive therapy and a hybrid closed-loop continuous subcutaneous insulin infusion system demonstrated favorable clinical outcomes.
Conclusion: This case report contributes to the. understanding of Type B insulin resistance syndrome and highlights the potential benefit of integrating immunosuppressive therapies with advanced insulin delivery technologies. Further studies are needed to define their role in the management of patients with severe insulin resistance
References
. Kahn CR, Flier JS, Bar RS, et al. The Syndromes of Insulin Resistance and Acanthosis Nigricans. N Engl J Med 1976;294:739-45. DOI: 10.1056/NEJM197604012941401
. Ogawa W, Araki E, Ishigaki Y, Hirota Y, Maegawa H, Yamauchi T, Yorifuji T, Katagiri H. New classification and diagnostic criteria for insulin resistance syndrome. Endocr J. 2022 Feb 28;69(2):107-113. DOI: 10.1507/endocrj.EJ21-0725
. Willard DL, Stevenson M, Steenkamp D. Type B insulin resistance syndrome. Curr Opin Endocrinol Diabetes Obes. 2016 Aug;23(4):318-23. DOI: 10.1097/MED.0000000000000263
. Musso C, Cochran E, Moran SA, et al. Clinical course of genetic diseases of the insulin receptor (type A and Rabson-Mendenhall syndromes): a 30-year prospective. Medicine (Baltimore) 2004; 83:209–222. DOI: 10.1097/01.md.0000133625.73570.54
. Angelidi AM, Filippaios A, Mantzoros CS. Severe insulin resistance syndromes. J Clin Invest. 2021 Feb 15;131(4):e142245. DOI: 10.1172/JCI142245
. Martins LM, Fernandes VO, Carvalho MMD, Gadelha DD, Queiroz PC, Montenegro Junior RM. Type B insulin resistance syndrome: a systematic review. Arch Endocrinol Metab. 2020 Aug;64(4):337-348. DOI: 10.20945/2359-3997000000257
. Alvarez-Payares JC, Ribero D, Rodríguez L, Builes CE, Prieto C, Arango C, Gamboa JG, Alvarez-Payares C. Late Systemic Lupus Erythematosus-Associated Insulin Resistance Syndrome: A Rare Cause of De Novo Diabetes Mellitus. Case Rep Med. 2022 Oct 14; 2022:4655804. DOI: 10.1155/2022/4655804
. Gómez, A. M., Gómez, C., Leguizamón, G., & Imitola, A. (2018). Hiperglucemia refractaria al tratamiento por causa de resistencia a la insulina tipo B: reto diagnóstico. Rev Colomb Endocrinol Diabet Metab. 2018 Nov;5(4):48-51. Link: https://www.endocrino.org.co/revistaacevol5N4-Art8
. Hirota Y, Suwanai H, Yamauchi T, Kadowaki T. Clinical Features of Type B Insulin Resistance in Japanese Patients: Case Report and Survey-Based Case Series Study. J Diabetes Res. 2020 Apr 4;2020:4359787. DOI: 10.1155/2020/4359787
. Zhang S, Wang G, Wang J. Type B insulin resistance syndrome induced by systemic lupus erythematosus and successfully treated with intravenous immunoglobulin: case report and systematic review. Clin Rheumatol. 2013 Feb;32(2):181-8. DOI: 10.1007/s10067-012-2098-x
. Kura MM, Sanghavi SA. Acral Acanthosis Nigricans in a Case of Scleroderma. Indian J Dermatol. 2015 Jul-Aug;60:423. DOI: 10.4103/0019-5154.160540
. Yang GQ, Li YJ, Dou JT, Wang BA, Lu JM, Mu YM. Type B insulin resistance syndrome with Scleroderma successfully treated with multiple immune suppressants after eradication of Helicobacter pylori infection: a case report. BMC Endocr Disord. 2016;16:20. DOI: 10.1186/s12902-016-0099-5
. Cochran E, Brown RJ, Gorden P. Other Antibodies Resulting in Diabetes Mellitus: Type B Insulin Resistance and Insulin Autoimmune Syndrome. AACE Clin Case Rep. 2016 Summer;2(3):e274-e275. DOI: 10.4158/ep151122.co
. Censi S, Mian C, Betterle C. Insulin autoimmune syndrome: from diagnosis to clinical management. Ann Transl Med. 2018 Sep;6(17):335. DOI: 10.21037/atm.2018.07.32
. Cappellani D, Macchia E, Falorni A, Marchetti P. Insulin Autoimmune Syndrome (Hirata Disease): A Comprehensive Review Fifty Years After Its First Description. Diabetes Metab Syndr Obes. 2020 Apr 1;13:963-978. DOI: 10.2147/DMSO.S219438
. Sjöholm Å, Pereira MJ, Nilsson T, Linde T, Katsogiannos P, Saaf J, Eriksson JW. Type B insulin resistance syndrome in a patient with type 1 diabetes. Endocrinol Diabetes Metab Case Rep. 2020 Apr 29;2020:19-0157. DOI: 10.1530/EDM-19-0157
. Osanami A, Kanda M, Sato T, Akazawa C, Baba S, Komatsu H, Murase K, Yamashita T, Yano T. Case report: Successful combination therapy with double-filtration plasmapheresis and rituximab under the condition of the use of a sensor-augmented pump for type B insulin resistance syndrome. Front Endocrinol (Lausanne). 2022 Sep 9;13:997296. DOI 10.3389/fendo.2022.997296
Almurashi AM, Rodriguez E, Garg SK. Emerging Diabetes Technologies: Continuous Glucose Monitors/Artificial Pancreases. J Indian Inst Sci. 2023 Mar 28:1-26. doi: 10.1007/s41745-022-00348-3. Epub ahead of print. PMID: 37362851; PMCID: PMC10043869. DOI: 10.1007/s41745-022-00348-3
. Klubo-Gwiezdzinska J, Lange M, Cochran E, Semple RK, Gewert C, Brown RJ, Gorden P. Combined Immunosuppressive Therapy Induces Remission in Patients With Severe Type B Insulin Resistance: A Prospective Cohort Study. Diabetes Care. 2018 Nov;41(11):2353-2360. DOI: 10.2337/dc18-0884
. Malek R, Chong AY, Lupsa BC, Lungu AO, Cochran EK, Soos MA, Semple RK, Balow JE, Gorden P. Treatment of type B insulin resistance: a novel approach to reduce insulin receptor autoantibodies. J Clin Endocrinol Metab. 2010 Aug;95(8):3641-7. DOI: 10.1210/jc.2010-0167
This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.
Copyright (c) 2026 Revista Colombiana de Endocrinología, Diabetes & Metabolismo