Paediatric necrolytic migratory erythema as a presenting sign of glucagonoma syndrome

Summary Glucagonoma syndrome is an extremely rare pancreatic neuroendocrine tumour often associated with necrolytic migratory erythema. While glucagonomas are neoplasms of adulthood, we report the first case in a paediatric patient. We present the case of a 15‐year‐old girl with a 4‐year history of a rash, consistent with necrolytic migratory erythema, found to have a localized glucagonoma. Immediately following resection of the tumour, there was complete resolution of her rash and systemic symptoms. Detection of the cutaneous rash of necrolytic migratory erythema can aid in the early diagnosis of a glucagonoma, as well as the prevention of metastatic disease. To our knowledge, this is the first reported paediatric patient with glucagonoma syndrome. What's already known about this topic? Necrolytic migratory erythema often presents as the first clinical sign in glucagonoma syndrome. The majority of cases occur in the fifth decade of life, with no paediatric cases previously reported. What does this study add? We present the first reported case of a paediatric patient with necrolytic migratory erythema as the presenting sign of a localized glucagonoma. Recognition of necrolytic migratory erythema in patients of all ages can aid in the early diagnosis and treatment of glucagonomas.