Glucagonoma (syndrome)

Glucagonoma syndrome is a clinicopathologic entity caused by (generally malignant) tumoral proliferation of alpha islet cells in the pancreas.

The typical dermatological lesion is necrolytic migratory erythema. Blepharitis, stomatitis, painful glossitis, balanitis and vulvovaginitis may also be present. Weight loss and diabetes are evocative and may be combined with venous thrombosis, abdominal pain and psychiatric disorders.

A serum glucagon assay result showing hyperglucagonaemia is suggestive of glucagonoma. The tumour itself is diagnosed via transparietal and endoscopic ultrasound or, where necessary, coeliac arteriography. The rarity of this disease is such that no standard treatment exists.