Necrolytic migratory erythema (NME) is a classical symptom observed in patients with glucagonoma and is present in 80% of cases. Associated NME is characterized by the spread of erythematous blisters and swelling across areas subject to greater friction and pressure, including the lower abdomen, buttocks, perineum, and groin.
Glucagonoma is usually malignant (cancerous). The cancer tends to spread and get worse. The cancer affects the islet cells of the pancreas. As a result, they produce too much of a hormone called glucagon. The excess glucagon causes symptoms such as glucose intolerance and hyperglycemia (elevated blood sugar).
Glucagonomas not associated with the syndrome are diagnosed in various ways. The tumor may appear as a malignant pancreatic tumor discovered because of local growth, with or without metastases. Glucagonoma very rarely is a member of multiple endocrine neoplasia type 1 (MEN I) syndromes, and, in such cases, it appears as a single lesion and is biologically inactive. Similar to other islet cell tumors, the primary and metastatic lesions are slow growing.
Patients usually present with nonspecific complaints, such as weight loss, diabetes, diarrhea, and stomatitis. Unexplained weight loss and the onset of NME, especially with new-onset diabetes mellitus, often hasten the correct diagnosis. Still, because mild early lesions may exhibit only subtle changes in histology and because inadequate sampling can miss the diagnostic changes, the skin eruption itself often is interpreted as a nonspecific dermatitis. It is not uncommon for several years to elapse before the correct diagnosis is found.
The most important of these factors is the reduced blood concentration of glucose. Acetylcholine and catecholamines elevate serum levels of glucagon and somatostatin, and serotonin reduces these levels. Physiological glucagon activity includes (1) glycogenolysis activation with contemporary glycolysis inhibition and activation of the gluconeogenesis; (2) stimulation of lipolysis and catecholamines secretion; (3) inhibition of the gastric secreting activity, the pancreatic secreting activity, and the gastrointestinal motility; and (4) the stimulation of urinary excretion of water and phosphates as well as sodium, calcium, and magnesium ions.
Currently, 72 months after transplantation, our patient is in complete remission, which has been verified by somatostatin receptor scintigraphy monitoring, computed tomographic scanning and glucagon serum control. Increased awareness of the clinical symptoms and visible polymorphic mucocutaneous and nonspecific histopathologic features of glucagonoma syndrome is needed to avoid unnecessary delay in the diagnosis of this syndrome.
Weight loss induced by the catabolic effects of glucagon. Antibiotics, steroids, and both amino acid and zinc supplementation may improve the skin rash when it is severe, but cure of the rash is achieved only with the return of glucagon levels to normal. Octreotide also is useful in helping to improve the perioperative condition of these patients. Prophylactic measures to prevent venous thrombosis, including low-dose subcutaneous heparin or intermittent pneumatic compression stockings, are mandatory for all patients during the perioperative period.
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