OBJECTIVETo study the clinical features, diagnosis, and treatment of glucagonoma syndrome (GS) for providing clues for the recognition of this disorder in clinical practice.

METHODThe clinical and laboratory findings of four confirmed patients with GS were analyzed retrospectively.

RESULTSAll four patients had typical clinical manifestations of necrolytic migratory erythema (NME), elevated glucagon level in serum and hepatic metastasis. The skin rashes disappeared gradually and serum glucagon level decreased after operation and somatostatin treatment.

CONCLUSIONSNME is the most specific clinical finding of GS. Biopsy of the lesions, glucagon level in serum and various radiological examinations should be done in order to confirm the diagnosis. Surgical resection, chemotherapy, and somatostatin are the main therapies of GS.