title: Glucagonoma syndrome associated with necrolytic migratory erythema.
abstract: BACKGROUND
  glucagonoma is a pancreatic neuroendocrine tumor derived from alpha-cells of the islets of Langerhans. It is marked by tumoral autonomous production of glucagon and characterized, among other symptoms, by necrolytic migratory erythema, an erythematous circinate lesion with areas of necrosis and sloughing. This is a rare disease with worldwide incidence estimated at 1 case per 20 million people.
  
  
  METHODS
  we report a case of glucagonoma associated necrolytic migratory erythema in a male patient, 56 years, with signs of skin lesions mainly on his legs and groin, hyperglycemia and weight loss. Biopsies of the skin lesions were performed and imaging of the abdomen showed a mass of 10 x 9 cm, at the pancreatic region. The patient was subjected to body-caudal pancreatectomy and splenectomy with autotransplant of the spleen in the greater omentum. The histopathologic report indicated a tumor in the pancreatic alpha cells. Immunohistochemistry showed expression of glucagon and chromogranin A in most tumor cells, consistent with the diagnosis of glucagonoma. The patient presented 3 years of outpatient follow-up with no complications.
  
  
  CONCLUSIONS
  the necrolytic migratory erythema is important for the clinical recognition of glucagonoma, and its early diagnosis is essential for a successful curative therapy.
authors.affiliation: Universidade Federal do Ceará, Fortaleza, CE, Brazil.
authors.forename: Florentino de Araújo
authors.initials: Fde A
authors.lastname: Cardoso Filho
authors.affiliation: Universidade Federal de São Paulo, São Paulo, SP, Brazil.
authors.forename: Roney Gonçalves Fechine
authors.initials: RG
authors.lastname: Feitosa
authors.affiliation: Hospital do Servidor Público do Estado de São Paulo, São Paulo, SP, Brazil.
authors.forename: Carolina Oliveira Costa
authors.initials: CO
authors.lastname: Fechine
authors.affiliation: Dr. César Cals de Oliveira Hospital, Fortaleza, CE, Brazil.
authors.forename: Carlos Márcio Melo de
authors.initials: CM
authors.lastname: Matos
authors.affiliation: Universidade Christus, Fortaleza, CE, Brazil.
authors.forename: Amanda Linhares
authors.initials: AL
authors.lastname: Cardoso
authors.affiliation: Universidade de Fortaleza, Fortaleza, CE, Brazil.
authors.forename: Daniel Linhares
authors.initials: DL
authors.lastname: Cardoso
mesh_terms: D001706:Biopsy; D005935:Glucagonoma / Q000150:complications*; D006801:Humans; D008297:Male; D008875:Middle Aged; D058568:Necrolytic Migratory Erythema / Q000209:etiology* / Q000473:pathology; D010190:Pancreatic Neoplasms / Q000150:complications*; D012867:Skin / Q000473:pathology