Putting the Pieces Together: Necrolytic Migratory Erythema and the Glucagonoma Syndrome
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- Halvorson, S.A.C., Gilbert, E., Hopkins, R.S. et al. J GEN INTERN MED (2013) 28: 1525. doi:10.1007/s11606-013-2490-5
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Glucagonomas are slow-growing, rare pancreatic neuroendocrine tumors. They may present with paraneoplastic phenomena known together as the “glucagonoma syndrome.” A hallmark sign of this syndrome is a rash known as necrolytic migratory erythema (NME). In this paper, the authors describe a patient with NME and other features of the glucagonoma syndrome. The diagnosis of this rare tumor requires an elevated serum glucagon level and imaging confirming a pancreatic tumor. Surgical and medical treatment options are reviewed. When detected early, a glucagonoma is surgically curable. It is therefore imperative that clinicians recognize the glucagonoma syndrome in order to make an accurate diagnosis and refer for treatment.