Glucagonoma Syndrome- a Case Report

The glucagonoma syndrome is characterized by dermatitis, glucose intolerance, hypoaminoacidemia, and hyperglucagonemia secondary to an alpha-cell tumor of the pancreas. The classical symptoms are associated with alpha-cell pancreatic islet cell tumor or 'glucagonoma'. Other clinical features include anemia, glossitis, and weight loss. A 65-year-old woman with the syndrome came for medical attention for a skin rash, glossitis, and weight loss. A skin biopsy was suggestive of necrolytic migratory erythema. Necrolytic migratory erythema is considered to be a paraneoplastic dermatosis. To our knowledge, it is rarely reported in the literature. Skin symptoms are important; often they are the clue to the diagnosis of glucagonoma syndrome. On ultrasound there was hypoechoic mass in the distal pancreas so she was advised CT scan of the mass. CT scan showed high density area 4.5 cms in diameter with calcification in the tail of the pancreas. She later on passed away because of late presentation, delayed diagnosis and delayed treatment. The diagnosis of necrolytic migratory erythema is a matter of great importance, since it might be an auxiliary tool for the early detection of glucagonoma.