Rationale: Glucagonoma is a rare neuroendocrine tumor of the pancreas. Glucagonoma syndrome is often misdiagnosed as other skin lesions by clinicians due to a typical clinical sign of necrolytic migratory erythema (NME) with severe erythematous rash.
Patient concerns: A 48-year-old female patient was admitted to our department because she presented with unclear recurrent severe erythematous rash. The patient was diagnosed as skin disease.
Diagnoses: Histopathologic examination revealed a pancreatic glucagonoma. Immnohistochemical staining of tumor tissue was positive for glucagon.
Interventions: The distal pancreatectomy plus splenectomy was performed in 2017.
Outcomes: The skin lesions disappeared after surgery. She was followed up and showed no recurrence until now.
Lessons: Clinicians should consider the diagnosis of glucagonoma according to the typical initial symptoms. Early diagnosis is very important to provide a better prognosis. A multidisciplinary approach is effective in patients with unresectable metastatic tumors.