Cases reported "Glucagonoma"

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1/6. necrolytic migratory erythema.

    BACKGROUND: necrolytic migratory erythema is considered to be a paraneoplastic dermatosis. The classical symptoms are associated with alpha-cell pancreatic islet cell tumor or 'glucagonoma'. Generally, extracutaneous hallmarks of this disease include weight loss, diabetes, anaemia and diarrhoea. observation: We report a case of a 39-year-old woman with a 3-year history of recalcitrant psoriasiform eruption, who had no other associated symptoms on routine examination. Histologic examinations suggested necrolytic migratory erythema. Abdominal computer tomography was performed, which revealed a tumor in the tail of the pancreas. After distal resection of the pancreas her skin symptoms resolved in a few days time. histology was consistent with glucagonoma. She is clinically well and symptomless and no signs of metastasis after 4 years. CONCLUSIONS: It is infrequent to have only necrolytic migratory erythema, hyperglucagonaemia and islet-cell tumor but no other extracutaneous symptoms in glucagonoma syndrome. To our knowledge, ours is the second such case reported in the literature. skin symptoms are important, often they are the clue to the diagnosis of glucagonoma syndrome.
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keywords = dermatosis
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2/6. Effect of somatostatin on skin lesions and concentrations of plasma amino acids in a patient with glucagonoma-syndrome.

    A case of glucagonoma syndrome in a 58-year-old male patient who had the typical skin lesions associated with severe hypoaminoacidemia is described. The decrease in amino acids has been proposed to be causally related to the dermatosis. Furthermore, it has been shown previously that somatostatin rapidly improves skin lesions in glucagonoma patients. Therefore, plasma amino acid levels were determined before and during an infusion of somatostatin prior to surgical removal of the tumor in the tail of the pancreas. During somatostatin infusion in combination with total parenteral nutrition, 10 out of 22 amino acids were in the normal range. Thus it seems unlikely that normalization of amino acid levels is responsible for the rapid improvement in skin lesions in glucagonoma patients. On the other hand it cannot be excluded that partial normalization of amino acids contributed to the observed healing process. Nevertheless, somatostatin administered prior to surgery is a useful therapeutic regimen in these patients.
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keywords = dermatosis
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3/6. glucagonoma syndrome. A clinicopathologic, immunocytochemical, and ultrastructural study.

    necrolytic migratory erythema has been considered to be a paraneoplastic dermatosis. Although some individuals have had no associated neoplasm, a pancreatic glucagonoma is typically found in the vast majority. We describe a 71-year-old woman with findings strongly suggestive of the glucagonoma syndrome. The plasma glucagon level was modestly elevated. Special studies of the pancreas, including computerized axial tomography and selective angiography, did not demonstrate a tumor. Surgery, however, revealed a 5-cm tumor in the head of the pancreas. Although immunocytochemical stains showed only rare, weakly reactive cells, the electron microscopic study confirmed the diagnosis of an islet cell tumor and revealed characteristic morphologic features of glucagonoma.
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keywords = dermatosis
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4/6. glucagonoma syndrome: in vitro evidence that glucagon increases epidermal arachidonic acid.

    A 63-year-old white woman with perioral dermatitis, a sore tongue, and an erythematous dermatosis in the inframammary and perineal regions underwent surgical removal of a pancreatic glucagonoma. The patient's plasma and pooled normal human plasma containing Sigma glucagon were fed to human keratinocyte cultures and increased arachidonic acid levels by 300% and 200%, respectively, when compared to pooled normal human plasma with no added commercial glucagon. These experiments suggest that glucagon may increase inflammatory mediators such as arachidonic acid and its metabolites in the epidermis, causing the skin lesions seen in the glucagonoma syndrome.
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keywords = dermatosis
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5/6. necrolytic migratory erythema without glucagonoma in patients with liver disease.

    BACKGROUND: necrolytic migratory erythema (NME) is an uncommon inflammatory dermatosis with a distinct clinical and histologic appearance. NME is usually associated with glucagonoma. Only a few cases of NME in the absence of glucagonoma have been previously reported. OBJECTIVE: We sought to understand further the pathogenesis of NME by analyzing data from three patients. methods: Three patients were examined both clinically and histopathologically. RESULTS: Each patient had an extensive erythematous scaling eruption in intertriginous, perioral, and acral areas, and a markedly red, smooth tongue. skin biopsy specimens showed confluent parakeratosis, epidermal pallor, papillary edema, and a lymphohistiocytic infiltrate. 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. CONCLUSION: In the absence of glucagonoma, hepatocellular dysfunction and hypoalbuminemia appear to be the most common factors associated with NME.
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ranking = 1
keywords = dermatosis
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6/6. Iatrogenic necrolytic migratory erythema: a case report and review of nonglucagonoma-associated necrolytic migratory erythema.

    necrolytic migratory erythema is characterized by waves of irregular erythema in which a central bulla develops, and subsequently erodes and becomes crusted. It usually occurs in patients with an alpha-islet cell tumor of the pancreas. However, necrolytic migratory erythema has also been observed in patients without an associated glucagonoma. We describe a woman with iatrogenic necrolytic migratory erythema. She received intravenous glucagon for hypoglycemia associated with an insulin-like growth factor ii-secreting hemangiopericytoma. After chemotherapy, she developed necrolytic migratory erythema. The characteristics of the previously reported patients with nonglucagonoma-associated necrolytic migratory erythema are reviewed. In patients with nonglucagonoma-associated necrolytic migratory erythema, the dermatosis-related conditions most commonly observed were celiac disease or malabsorption, cirrhosis, malignancy, and pancreatitis; less common conditions included hepatitis, inflammatory bowel disease, heroin abuse, and odontogenic abscess. Although the pathogenesis of necrolytic migratory erythema remains unknown, hyperglucagonemia appears to have had a causative role in the development of this dermatosis in our patient. patients who develop necrolytic migratory erythema should be evaluated for the presence of a glucagonoma; if a glucagonoma is ruled out, evaluation for other conditions known to occur with necrolytic migratory erythema, such as liver disease, malabsorptive disorders, and nonislet-cell tumors is warranted.
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keywords = dermatosis
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