Clinical features of the glucagonoma syndrome:
(1) unexplained weight loss
(2) necrolytic migratory erythema, often involving the lower legs and feet and often with burning and itching
(3) glucose intolerance or diabetes
(4) thromboembolic disease
(5) cheilitis or stomatitis
(6) perianal and/or genital lesions
(7) anemia
(8) diarrhea
The skin lesions may precede the diagnosis of an islet cell tumor by months or years.
Laboratory features:
(1) increased serum fasting glucagon levels
(2) hypoaminoacidemia
Differential diagnosis: The fasting glucagon level may be increased up to 2.5 times the upper limit of normal in patients with systemic stress responses (burns, ketoacidosis, trauma, sepsis, starvation) or organ failures (renal, hepatic).
