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Glucagonomas result in a migratory necrolytic erythema, weight loss, glucose intolerance, hypoamino-acidemia, and normochromic, normocytic ane-mia.23,24 Other less common features include thromboembolic phenomena, neuropsychiatry disturbances, diarrhea, and nonspecific abdominal pain. Glucagonomas occur in 3% of all patients with MEN type I. The age of onset is middle age or later. They typically occur in the tail of the pancreas. They are often large at presentation, 5 to 10 cm, and malignancy is common. Patients present with signs and symptoms of hyperglycemia, and usually diabetes mellitus precedes the diagnosis of glucagonoma. However, Cushing's syndrome is a far more common cause of hyperglycemia in MEN type I. The diagnosis is made by a glucagon level > 1,000 pg/mL often in the presence of the characteristic skin rash.

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