The glucagonoma syndrome

The glucagonoma syndrome is another of those systemic disorders in which skin manifestations provide a clue to the diagnosis. The patient will most often be a middle-aged woman who has the characteristic, indolent skin lesions in the face of diabetes mellitus and additional features to suggest an occult carcinoma. Marked elevation of the levels of plasma glucagon should confirm the suspicion cure of the skin lesions follows cure of the tumor. Two lines of speculation seem promising. Either the initial event is an overproduction of glucagon and all other observations follow. Or the syndrome is another of the polyendocrine disorders. Cases are still too few to resolve either the pathophysiology, prognosis, or even to guess at the true frequency of the syndrome.