GLUCAGONOMA

By Gary M. White, MD

Necrolytic migratory erythema or glucagonoma


Necrolytic migratory erythema (NME) is a rare paraneoplastic skin condition and is most commonly seen as a cutaneous manifestation of a glucagon-secreting, alpha cell tumor of the pancreas, although some patients may have necrolytic migratory erythema without a glucagonoma. A similar rash occurs in infants with zinc deficiency.

Clinical

An annular, erosive or bullous eruption develops periorally and in the groin in NME. Weight loss, anemia, diabetes mellitus and glossitis may occur. The diagnosis is made by finding elevated levels of serum glucagon and confirmed with imaging of the abdomen/pancreas.

Workup

Serum zinc, amino acids and free fatty acid levels should be measured. Abdominal CT and celiac arteriography are valuable for imaging the pancreatic tumor and possible liver metastasis.

Treatment

Referral to an oncologist is appropriate. Work up for and treatment of any pancreatic tumor should be done. If no glucagon-secreting tumor is found, or the tumor cannot be removed, administration of zinc, amino acids (oral or IV) and free fatty acids may be effective. The administration of amino acids IV resulted in resolution within 24 hours of the skin and mucosal lesions in one patient with NME [JAMA Derm 2016;152;345]. The somatostatin analogue, octreotide has been used for metastatic disease. One should make sure there is no nutritional deficiency (e.g. zinc) with the need for supplementation.

Additional Pictures

Necrolytic migratory erythema or glucagonoma Necrolytic migratory erythema or glucagonoma

Necrolytic migratory erythema or glucagonoma Necrolytic migratory erythema or glucagonoma Necrolytic migratory erythema or glucagonoma Necrolytic migratory erythema or glucagonoma

NECROLYTIC MIGRATORY ERYTHEMA WITHOUT GLUCAGONOMA

Various reports have described patients with the clinical and histologic features of NME without glucagonoma--so called pseudoglucagonoma syndrome. Suspected causes have included chronic liver disease, intestinal malabsorption (e.g. from pancreatic insufficiency or gluten-sensitive enteropathy leading to decreased levels of amino acids, zinc and/or essential fatty acids), and heroin use. Hypoalbuminemia is common in those patients with chronic liver disease.

Treatment

Improved nutrition with zinc, protein and essential fatty acid supplementation should be tried and may help.

RegionalDerm

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