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Necrolytic migratory erythema — ABIM Board MCQ

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HardDermatologyNecrolytic migratory erythemaABIM Board

A 59-year-old has new diabetes, weight loss, glossitis, diarrhea and an annular erosive rash involving the perioral and intertriginous regions. CT shows a pancreatic-tail mass and fasting glucagon is markedly elevated. What is the unifying diagnosis?

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Correct answer: C — Functional glucagon-secreting pancreatic neuroendocrine tumor syndrome

The best answer is “Functional glucagon-secreting pancreatic neuroendocrine tumor syndrome”. The combination of hyperglucagonemia, diabetes, weight loss and necrolytic migratory erythema is the glucagonoma syndrome from a functional pancreatic neuroendocrine tumor, often arising in the tail. The rash distribution and systemic metabolic syndrome are not explained by IgA disease, zinc excess, herpes or an incidental nonfunctioning cyst.

Reference: NCI Pancreatic Neuroendocrine Tumor Treatment PDQ: https://www.cancer.gov/types/pancreatic/hp/pnet-treatment-pdq