We report on our experience with two patients with glucagonoma syndrome and review the recent dermatologic literature. The clinical features are described with special emphasis on necrolytic migratory erythema, the characteristic cutaneous sign of glucagonoma syndrome. Using our histological and ultrastructural findings, we discuss the pathogenesis of necrolytic migratory erythema. Pathognomonic laboratory data and the diagnostic procedures recommended for the evaluation of patients with glucagonoma syndrome are presented. Finally, we discuss the differential diagnosis and describe therapeutic possibilities in the management of this syndrome.

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