Clinical Practice: Clinical Vignettes

Journal of General Internal Medicine

, Volume 28, Issue 11, pp 1525-1529

First online:

Putting the Pieces Together: Necrolytic Migratory Erythema and the Glucagonoma Syndrome

  • Stephanie A. C. HalvorsonAffiliated withDivision of Hospital Medicine, Department of Medicine, Oregon Health & Science University Email author 
  • , Erin GilbertAffiliated withDepartment of Surgery, Oregon Health & Science University
  • , R. Samuel HopkinsAffiliated withDepartment of Dermatology, Oregon Health & Science University
  • , Helen LiuAffiliated withDepartment of Dermatology, Oregon Health & Science University
  • , Charles LopezAffiliated withKnight Cancer Institute, Oregon Health & Science University
  • , Michael ChuAffiliated withDivision of Endocrinology, Diabetes and Clinical Nutrition, Department of Medicine, Oregon Health & Science University
  • , Marie MartinAffiliated withOregon Health & Science University
  • , Brett SheppardAffiliated withDepartment of Surgery, Oregon Health & Science University

Rent the article at a discount

Rent now

* Final gross prices may vary according to local VAT.

Get Access

Abstract

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.

KEY WORDS

glucagonoma neuroendocrine tumors necrolytic migratory erythema