Glucagonoma and pseudoglucagonoma syndromes

Necrolytic Migratory Erythema: Case Report and Clinical Review

“…These conditions are collectively referred to as the pseudoglucagonoma syndrome. 4 Reported cases include liver disease/cirrhosis, pancreatic disease, chronic renal failure, Danazol therapy, Cushing syndrome, trauma/burn/surgery, bacteremia, Celiac disease, familial hyperglucagonemia, and decompensated diabetes mellitus. 5 These exceptions being noted, it is important to stress that NME is classically observed in patients with a glucagonoma.…”

“…5 Reports in the literature range from age 19 to 84. 3,6 Some papers have noticed a female preponderance (60% women), 4 however, other reviews note an equal gender distribution. 3,[7][8][9] There have been fewer than 200 cases worldwide, with an incidence estimated at 1 in 20 million.…”

“…3 The tumor is most often identified in the body and tail of the pancreas and often has metastasized at the time of diagnosis. 4 Patients often present emaciated, with alopecia and a chronic dermatitis of many years' duration. The dermatitis has often been misdiagnosed as a chronic candidiasis, acrodermatitis enteropathica, or some other inflammatory skin condition.…”

“…The dermatitis has often been misdiagnosed as a chronic candidiasis, acrodermatitis enteropathica, or some other inflammatory skin condition. 4 Necrolytic migratory erythema is the sine qua non of the glucagonoma syndrome. In a review of 21 GS patients by Wermers et al, 3 all of the patients had NME at one point in their illness while 67% had NME as the presenting complain.…”

Glucagonoma and the glucagonoma syndrome (Review)

“…Mild elevated glucagon levels may be associated with other diseases, including malabsorption, infection or hepatic cirrhosis (35). Glucagon receptor gene mutations may also cause hyperglucagonemia without glucagonoma syndrome (36).…”