Necrolytic Migratory Erythema: Complete Healing after Surgical Removal of Pancreatic Carcinoma

Necrolytic migratory erythema is considered an obligatory cutaneous paraneoplastic sign associated with glucagonoma. Glucagonoma syndrome is defined by the presence of an alpha-cell secreting tumor of the pancreas, elevated levels of glucagon, and a characteristic rash called necrolytic migratory erythema. Although necrolytic migratory erythema is a specific finding in glucagonoma syndrome, it may occur in other settings, unassociated with an alpha-cell pancreatic tumor (pseudoglucagonoma syndrome). The rarity of glucagonoma imposes a challenge, with most patients being diagnosed after a long period of treatment for their skin rash. The main prognostic sign of glucagonoma are the subsequent metastases that come late in the course of the disease. Herein, we present a 55-year-old female patient with a 5-year history of unrecognized cutaneous and systemic manifestations of glucagonoma syndrome. Based on the investigations, the diagnosis of glucagonoma syndrome without metastases was established. After surgical removal of pancreatic carcinoma/glucagonoma, complete healing and a long disease-free period was achieved. Appropriate awareness of the characteristics of necrolytic migratory erythema in physicians/dermatologists often leads to an early diagnosis of glucagonoma syndrome and enhances the chances of a favorable outcome.