A 46-year-old man had a 7-year history of severe
rash, which was then diagnosed as
necrolytic migratory erythema. He had a
weight loss of 6 kg, abnormal
glucose tolerance test findings,
anemia,
glossitis,
hair loss, and
hypoproteinemia. Plasma
amino acids levels were significantly decreased, and the fasting plasma
glucagon (IRG) level was high at 5000 to 8000 pg/ml. Circulating IRG significantly increased after oral
glucose loading, meal ingestion, and
arginine infusion, and decreased with
somatostatin infusion and
insulin-induced
hypoglycemia. No other gut or pancreatic
hormone levels in plasma were elevated. Plasma IRG was eluted by gel-filtration, mainly in the position of true
glucagon (MW 3500) by antiserum 30K. The
rash was markedly improved after infusion of
amino acids. Computerized tomography (CT) scan and celiac angiography revealed a large pancreatic
tumor with multiple liver and
lymph node metastases. The pancreatic
tumor was totally resected, and was identified as
glucagonoma by immunohistochemical technique. Since the plasma IRG levels remained high after surgery, the patient received dimethyltriazenoimidazole carboxamide
therapy. After several courses of this treatment, plasma IRG levels decreased to 1000 to 2000 pg/ml, and the hepatic
metastases were remarkably diminished in size.