It presents with annular eruptions of migrating erythematous papules and plaques with superficial epidermal necrosis, central flaccid bullae, and crusted erosions located primarily in the intertriginous areas. [ncbi.nlm.nih.gov]

• Splenectomy

  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. [ncbi.nlm.nih.gov]

• Congestive Heart Failure

  Having reached the final stage of the disease, which was further complicated by congestive heart failure, the patient died one year later. [ncbi.nlm.nih.gov]

• Sepsis

  He died due to internal bleeding and sepsis after surgery. Presentation of diffuse malignant glucagonoma with tumor emboli but no metastasis or NME is unusual. [ncbi.nlm.nih.gov]

• Internal Bleeding

  He died due to internal bleeding and sepsis after surgery. Presentation of diffuse malignant glucagonoma with tumor emboli but no metastasis or NME is unusual. [ncbi.nlm.nih.gov]

• Polydipsia

  She had been experiencing weight loss, polydipsia, polyphagia, postprandial emesis, excessive hair loss and abdominal pain for two months. [ncbi.nlm.nih.gov]

• Liver Dysfunction

  Two patients had alcoholic liver disease and one had liver dysfunction as a result of hemochromatosis. Serum albumin level was depressed, and liver enzyme values were increased in all three patients. Glucagonoma was undetectable in these patients. [ncbi.nlm.nih.gov]

• Mucocutaneous Rash

  Abstract Necrolytic migratory erythema is a rare paraneoplastic dermatosis that may be the first clinical manifestation of the glucagonoma syndrome, a disorder characterized by mucocutaneous rash, glucose intolerance, hypoaminoacidemia, hyperglucagonaemia [ncbi.nlm.nih.gov]

• Liver Biopsy

  The patients were subjected to skin biopsy examination, CT scan of the pancreas and a liver biopsy. Liver function tests, serum glucagon, glucose, amino acids and zinc were measured. [ncbi.nlm.nih.gov]

Treatment with the long-acting somatostatin analog Octreotide is a potential therapy to help ameliorate skin symptoms. [ncbi.nlm.nih.gov]

Early recognition of NME can lead to possible cure, while delayed identification of the disease is associated with metastatic disease and a poor prognosis for the patient. [ncbi.nlm.nih.gov]

At least 50% of these tumors are metastatic at the time of diagnosis and, therefore, generally carry a poor prognosis. [emedicine.com]

METHODS: We performed a PubMed review of the literature on the etiology and pathogenesis of NME to identify case reports and reviews published in both the internal medicine and dermatology literature. [ncbi.nlm.nih.gov]

Etiology Hyperglucagonemia can be found as part of a polyfunctional endocrine tumor or an exclusively glucagon-producing tumor. The tumor may be part of a clinical syndrome (eg, Zollinger-Ellison syndrome ), or it may be asymptomatic. [emedicine.com]

Epidemiology Frequency Glucagonomas are quite rare; since 1942, more than 300 cases have been reported worldwide. Their annual incidence has been estimated at 1 in 20 million. [emedicine.com]

However, publications have reported that neither glucagonoma nor hyperglucagonemia is necessary for NME. [4] Pseudoglucagonoma syndrome refers to NME in the absence of a glucagon-secreting tumor. [5, 6] Pathophysiology Although the pathogenesis of NME [emedicine.com]

Early recognition and treatment may prevent metastatic disease and ensure its cure with resolution of the cutaneous and catabolic manifestations. [ncbi.nlm.nih.gov]