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June 1977

Glucagonoma SyndromeReport of Two Cases and Literature Review

Author Affiliations

From the Section of Dermatology (Drs Binnick and Spencer), Hitchcock Clinic and the Department of Medicine (Drs Binnick and Spencer), Dartmouth Medical School, Hanover, NH, and the Department of Medicine (Drs Horton and Dennison), University of Vermont College of Medicine, Burlington.

Arch Dermatol. 1977;113(6):749-754. doi:10.1001/archderm.1977.01640060045003

• The glucagonoma syndrome is characterized by dermatitis, stomatitis, elevated serum glucagon levels, abnormal glucose tolerance, weight loss, and anemia—all in association with a glucagonsecreting alpha-cell tumor of the pancreas. A review of 21 cases showed strikingly similar features. A generalized, symmetrical dermatitis initially appeared to be asteatotic or eczematous over the perineum, buttocks, and lower extremities. Gradually, a more characteristic migratory necrolytic erythema with transient bulla formation and erosions developed in intertriginous and dependent areas. Histologically, the most specific features included necrolysis of the upper epidermis, with liquefaction necrosis of the granular cell layer and subcorneal clefting or blister formation. The dermatologist is often first to examine such patients; early recognition of this syndrome with prompt surgical removal of the primary pancreatic lesion may afford cure of the neoplasm.

(Arch Dermatol 113:749-754, 1977)