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.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
References
Leichter SB. Clinical and metabolic aspects of glucagonoma. Medicine (Baltimore). 1980;59(2):100–13.
Hashizume T, et al. Glucagonoma syndrome. J Am Acad Dermatol. 1988;19(2 Pt 2):377–83.
Frankton S, Bloom SR. Gastrointestinal endocrine tumours. Glucagonomas Baillieres Clin Gastroenterol. 1996;10(4):697–705.
van Beek AP, et al. The glucagonoma syndrome and necrolytic migratory erythema: a clinical review. Eur J Endocrinol. 2004;151(5):531–7.
Lobo I, et al. Glucagonoma syndrome and necrolytic migratory erythema. Int J Dermatol. 2010;49(1):24–9.
Tierney EP, Badger J. Etiology and pathogenesis of necrolytic migratory erythema: review of the literature. MedGenMed. 2004;6(3):4.
Prout TM, Taylor AJ. Case of the season: glucagonoma syndrome. Semin Roentgenol. 2005;40(1):4–7.
Chastain MA. The glucagonoma syndrome: a review of its features and discussion of new perspectives. Am J Med Sci. 2001;321(5):306–20.
Boden G. Insulinoma and glucagonoma. Semin Oncol. 1987;14(3):253–62.
Doll DC. Necrolytic migratory erythema. Arch Dermatol. 1980;116(8):861–2.
Balachandran A, et al. Pancreatic neuroendocrine neoplasms: diagnosis and management. Abdom Imaging. 2013;38(2):342–57.
Thorisdottir K, et al. Necrolytic migratory erythema: a report of three cases. J Am Acad Dermatol. 1994;30(2 Pt 2):324–9.
Lakdawala N, et al. The role of nutrition in dermatologic diseases: facts and controversies. Clin Dermatol. 2013;31(6):677–700.
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.
Goodenberger DM, et al. Necrolytic migratory erythema without glucagonoma. Arch Dermatol. 1979;115(12):1429–32.
Halvorson SA, et al. Putting the pieces together: necrolytic migratory erythema and the glucagonoma syndrome. J Gen Intern Med. 2013;28(11):1525–9.
Fedeles F, et al. Nutrition and bullous skin diseases. Clin Dermatol. 2010;28(6):627–43.
Nakashima H, et al. Necrolytic migratory erythema without glucagonoma in a patient with short bowel syndrome. J Dermatol. 2006;33(8):557–62.
Mignogna MD, Fortuna G, Satriano AR. Small-cell lung cancer and necrolytic migratory erythema. N Engl J Med. 2008;359(25):2731–2.
Kheir SM, et al. Histologic variation in the skin lesions of the glucagonoma syndrome. Am J Surg Pathol. 1986;10(7):445–53.
Hendricks WM. Pellagra and pellagralike dermatoses: etiology, differential diagnosis, dermatopathology, and treatment. Semin Dermatol. 1991;10(4):282–92.
Stratigos JD, Katsambas A. Pellagra: a still existing disease. Br J Dermatol. 1977;96(1):99–106.
Wong PW, Pillai PM. Clinical and biochemical observations in two cases of hartnup disease. Arch Dis Child. 1966;41(218):383–8.
Neldner KH, Hambidge KM, Walravens PA. Acrodermatitis enteropathica. Int J Dermatol. 1978;17(5):380–7.
Graves K, Kestenbaum T, Kalivas J. Hereditary acrodermatitis enteropathica in an adult. Arch Dermatol. 1980;116(5):562–4.
Wermers RA, et al. The glucagonoma syndrome. Clinical and pathologic features in 21 patients. Medicine (Baltimore). 1996;75(2):53–63.
Hobday TJ, et al. Multicenter phase II trial of temsirolimus and bevacizumab in pancreatic neuroendocrine tumors. J Clin Oncol. 2014;33(14):1551–6.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2016 Springer International Publishing Switzerland
About this chapter
Cite this chapter
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
Download citation
DOI: https://doi.org/10.1007/978-3-319-31566-9_23
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-31564-5
Online ISBN: 978-3-319-31566-9
eBook Packages: MedicineMedicine (R0)