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

  • Clinical Practice: Clinical Vignettes
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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.

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Conflict of Interest

The authors declare that they do not have a conflict of interest.

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Authors and Affiliations

  1. Division of Hospital Medicine, Department of Medicine, Oregon Health & Science University, 3181 SW Sam Jackson Park Rd, BTE-119, Portland, OR, 97239, USA

    Stephanie A. C. Halvorson MD

  2. Department of Surgery, Oregon Health & Science University, Portland, OR, USA

    Erin Gilbert MD & Brett Sheppard MD

  3. Department of Dermatology, Oregon Health & Science University, Portland, OR, USA

    R. Samuel Hopkins MD & Helen Liu MD

  4. Knight Cancer Institute, Oregon Health & Science University, Portland, OR, USA

    Charles Lopez MD, PhD

  5. Division of Endocrinology, Diabetes and Clinical Nutrition, Department of Medicine, Oregon Health & Science University, Portland, OR, USA

    Michael Chu MD

  6. Oregon Health & Science University, Portland, OR, USA

    Marie Martin BA

Authors
  1. Stephanie A. C. Halvorson MD
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  2. Erin Gilbert MD
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  3. R. Samuel Hopkins MD
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  4. Helen Liu MD
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  5. Charles Lopez MD, PhD
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  6. Michael Chu MD
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  8. Brett Sheppard MD
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Correspondence to Stephanie A. C. Halvorson MD.

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Halvorson, S.A.C., Gilbert, E., Hopkins, R.S. et al. Putting the Pieces Together: Necrolytic Migratory Erythema and the Glucagonoma Syndrome. J GEN INTERN MED 28, 1525–1529 (2013). https://doi.org/10.1007/s11606-013-2490-5

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  • DOI: https://doi.org/10.1007/s11606-013-2490-5

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