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Necrolytic Migratory Erythema

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Deadly Dermatologic Diseases

Abstract

Necrolytic migratory erythema (NME) is a major component of glucagonoma syndrome, a rare paraneoplastic syndrome consisting of the classic triad of diarrhea, diabetes mellitus, and rash associated with serum hyperglucagonemia. Originally described by Becker et al. in 1942, NME has an incidence of approximately 1 in 20 million. There is no ethnic or gender predilection, and peak age of onset is during the fourth and fifth decades. NME occurs in 70 % of those with glucagonoma syndrome. Glucagonomas are associated with type 1 multiple endocrine neoplasia (MEN I) syndrome sequence and/or Zollinger-Ellison hypergastrinemia syndrome in a minority of the cases (about 5 % of cases). The most common etiology involves the elaboration of glucagon from an islet cell tumor of the pancreas but may rarely follow the metabolic consequences of cirrhosis, pancreatic insufficiency, or celiac disease.

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References

  1. Leichter SB. Clinical and metabolic aspects of glucagonoma. Medicine (Baltimore). 1980;59(2):100–13.

    Article  CAS  Google Scholar 

  2. Hashizume T, et al. Glucagonoma syndrome. J Am Acad Dermatol. 1988;19(2 Pt 2):377–83.

    Article  CAS  PubMed  Google Scholar 

  3. Frankton S, Bloom SR. Gastrointestinal endocrine tumours. Glucagonomas Baillieres Clin Gastroenterol. 1996;10(4):697–705.

    Article  CAS  PubMed  Google Scholar 

  4. van Beek AP, et al. The glucagonoma syndrome and necrolytic migratory erythema: a clinical review. Eur J Endocrinol. 2004;151(5):531–7.

    Article  PubMed  Google Scholar 

  5. Lobo I, et al. Glucagonoma syndrome and necrolytic migratory erythema. Int J Dermatol. 2010;49(1):24–9.

    Article  PubMed  Google Scholar 

  6. Tierney EP, Badger J. Etiology and pathogenesis of necrolytic migratory erythema: review of the literature. MedGenMed. 2004;6(3):4.

    PubMed  PubMed Central  Google Scholar 

  7. Prout TM, Taylor AJ. Case of the season: glucagonoma syndrome. Semin Roentgenol. 2005;40(1):4–7.

    Article  PubMed  Google Scholar 

  8. Chastain MA. The glucagonoma syndrome: a review of its features and discussion of new perspectives. Am J Med Sci. 2001;321(5):306–20.

    Article  CAS  PubMed  Google Scholar 

  9. Boden G. Insulinoma and glucagonoma. Semin Oncol. 1987;14(3):253–62.

    CAS  PubMed  Google Scholar 

  10. Doll DC. Necrolytic migratory erythema. Arch Dermatol. 1980;116(8):861–2.

    Article  Google Scholar 

  11. Balachandran A, et al. Pancreatic neuroendocrine neoplasms: diagnosis and management. Abdom Imaging. 2013;38(2):342–57.

    Article  PubMed  Google Scholar 

  12. Thorisdottir K, et al. Necrolytic migratory erythema: a report of three cases. J Am Acad Dermatol. 1994;30(2 Pt 2):324–9.

    Article  CAS  PubMed  Google Scholar 

  13. Lakdawala N, et al. The role of nutrition in dermatologic diseases: facts and controversies. Clin Dermatol. 2013;31(6):677–700.

    Article  PubMed  Google Scholar 

  14. Sahoo MK, et al. Necrolytic migratory erythema associated with glucagonoma syndrome diagnosed by (6)(8)Ga-DOTANOC PET-CT. Asia Pac J Clin Oncol. 2014;10(2):190–3.

    Article  PubMed  Google Scholar 

  15. Goodenberger DM, et al. Necrolytic migratory erythema without glucagonoma. Arch Dermatol. 1979;115(12):1429–32.

    Article  CAS  PubMed  Google Scholar 

  16. Halvorson SA, et al. Putting the pieces together: necrolytic migratory erythema and the glucagonoma syndrome. J Gen Intern Med. 2013;28(11):1525–9.

    Article  PubMed  PubMed Central  Google Scholar 

  17. Fedeles F, et al. Nutrition and bullous skin diseases. Clin Dermatol. 2010;28(6):627–43.

    Article  PubMed  Google Scholar 

  18. Nakashima H, et al. Necrolytic migratory erythema without glucagonoma in a patient with short bowel syndrome. J Dermatol. 2006;33(8):557–62.

    Article  CAS  PubMed  Google Scholar 

  19. Mignogna MD, Fortuna G, Satriano AR. Small-cell lung cancer and necrolytic migratory erythema. N Engl J Med. 2008;359(25):2731–2.

    Article  CAS  PubMed  Google Scholar 

  20. Kheir SM, et al. Histologic variation in the skin lesions of the glucagonoma syndrome. Am J Surg Pathol. 1986;10(7):445–53.

    Article  CAS  PubMed  Google Scholar 

  21. Hendricks WM. Pellagra and pellagralike dermatoses: etiology, differential diagnosis, dermatopathology, and treatment. Semin Dermatol. 1991;10(4):282–92.

    CAS  PubMed  Google Scholar 

  22. Stratigos JD, Katsambas A. Pellagra: a still existing disease. Br J Dermatol. 1977;96(1):99–106.

    Article  CAS  PubMed  Google Scholar 

  23. Wong PW, Pillai PM. Clinical and biochemical observations in two cases of hartnup disease. Arch Dis Child. 1966;41(218):383–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  24. Neldner KH, Hambidge KM, Walravens PA. Acrodermatitis enteropathica. Int J Dermatol. 1978;17(5):380–7.

    CAS  PubMed  Google Scholar 

  25. Graves K, Kestenbaum T, Kalivas J. Hereditary acrodermatitis enteropathica in an adult. Arch Dermatol. 1980;116(5):562–4.

    Article  CAS  PubMed  Google Scholar 

  26. Wermers RA, et al. The glucagonoma syndrome. Clinical and pathologic features in 21 patients. Medicine (Baltimore). 1996;75(2):53–63.

    Article  CAS  Google Scholar 

  27. Hobday TJ, et al. Multicenter phase II trial of temsirolimus and bevacizumab in pancreatic neuroendocrine tumors. J Clin Oncol. 2014;33(14):1551–6.

    Article  PubMed  PubMed Central  Google Scholar 

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Correspondence to Michael B. Morgan .

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Schuering, R., Morgan, M.B. (2016). Necrolytic Migratory Erythema. In: Crowe, D., Morgan, M., Somach, S., Trapp, K. (eds) Deadly Dermatologic Diseases. Springer, Cham. https://doi.org/10.1007/978-3-319-31566-9_23

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  • DOI: https://doi.org/10.1007/978-3-319-31566-9_23

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