Background: Glucagonoma is a rare functional pancreatic neuroendocrine tumor. Necrolytic migratory erythema (NME) is a paraneoplastic manifestation of glucagonoma and is often the first presenting symptom. The misdiagnosis of this rare entity can affect the patient’s quality of life and his life expectancy. Case Presentation: We report a case of a 48-year-old man presenting with pruritic scaly rash, ulcerated skin lesions, and periorbital edema, who was diagnosed and treated for atopic eczema for a 7-year period. Despite treatment, his dermatitis and general conditions progressively worsened, until he was admitted to the emergency department due to weight loss and fatigue. An uncontrolled diabetes was found and the computer tomography scan of the abdomen revealed a tumor located in the tail of the pancreas with liver metastases. Subsequently, liver biopsy and high levels of glucagon confirmed the diagnosis of glucagonoma. The patient was treated with short-acting subcutaneous octreotide for 2 weeks and then with intramuscular slowrelease octreotide every 28 days, with a significant improvement of the symptoms. Conclusion: This case provides further knowledge about NME, since a timely recognition and treatment of this misleading disease can prevent morbidity from the dermatitis and mortality from the malignancy itself.

Keywords: Glucagonoma, neuroendocrine tumors, glucagonoma syndrome, necrolytic migratory erythema (NME), quality of life, dupilumab, somatostatin analogs (SSA).