Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P01275 (glucagon)
 26,492 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two patients with clinical and histologic findings consistent with necrolytic migratory erythema are presented. Unlike previously described patients with this disorder, neither patient had substantially elevated glucagon levels nor an associated pancreatic islet cell tumor. The cause of the skin disease in these patients remains unknown but may be related to the underlying small-bowel disorder present in both.
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PMID:Necrolytic migratory erythema without glucagonoma. 53 89

In this paper two patients with uncommon syndromes, viz. acrodermatitis enteropathica-like eruption due to acute zinc deficiency, when on long-term intravenous hyperalimentation for Crohn's disease, and necrolytic migratory erythema as a consequence of a malignant glucagon secreting alpha-cell tumour of the pancreas (glucagonoma syndrome) are reported. Attention is paid to the many common features of the skin lesions in both syndromes. This is discussed in detail. Both patients passed through a catabolic stage. Laboratory investigations, however, failed to demonstrate a common biochemical mechanism which might be responsible for the skin lesions. Administration of zinc in the first patient and surgical treatment of the second patient results in rapid clearing of the skin lesions and other symptoms.
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PMID:Zinc deficiency syndrome versus glucagonoma syndrome. 53 35

Necrolytic migratory erythema is a distinctive cutaneous eruption that occurs in patients with malignant glucagon-secreting tumors of the pancreas. Recognition of this erosive dermatitis as a cutaneous manifestation of an internal malignancy can result in tumor detection and surgical removal prior to metastasis. The case history of a forty-year-old diabetic woman with necrolytic migratory erythema associated with a metastatic glucagon-secreting islet cell tumor of the pancreas is presented. Prior to diagnosis, she had been treated with topical steroids and Mycostatin powder for a recurrent perioral, acral, and intertriginous dermatitis. Because of apparent responsiveness to these medications, the diagnosis of necrolytic migratory erythema was not considered, and the diagnosis of pancreatic carcinoma was delayed for over one year. Wider recognition of the distinctive clinical and histopathologic features of necrolytic migratory erythema should result in earlier detection and possible surgical cure of the associated glucagonoma.
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PMID:Necrolytic migratory erythema: unresolved problems in diagnosis and pathogenesis. A case report and literature review. 156 84

A 49-year-old woman suffered from recurrent episodes of necrolytic migratory erythema over the lower legs, lower abdomen, and buttocks for more than two years. Stomatitis, glossitis and vaginitis were the accompanying symptoms and signs during each episode. The result of skin biopsy revealed superficial necrosis in the upper half of the epidermis. Laboratory examinations revealed mild glucose intolerance and hypoaminoacidemia. Fasting plasma glucagon level measured by radioimmunoassay was 890 pg/mL. Oral glucose loading test showed a paradoxical increase in plasma glucagon level up to 1,500 pg/mL. Abdominal echo, computerized axial tomography, and celiac angiography demonstrated a hypervascular tumor, 4 cm in diameters, located at the pancreatic head. Glucagonoma syndrome was confirmed and diagnosed. The patient underwent surgical resection of the tumor mass. Necrolytic migratory erythema disappeared thereafter, and the plasma glucagon level declined to 120 pg/mL. Histologically, the tumor revealed an islet cell carcinoma composed of moderately uniform cells with a few mitosis, arranged in cords and nests. Abundant characteristic secretory granules of the pancreatic A cell were found within the tumor cells by electron microscopic examination.
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PMID:[Necrolytic migratory erythema as the first manifestation of glucagonoma]. 168 96

The glucagonoma syndrome is characterized by elevated serum glucagon, a pancreatic alpha-cell tumor, anemia, hypoaminoacidemia, and necrolytic migratory erythema. Necrolytic migratory erythema may cause marked morbidity and is frequently misdiagnosed. A 42-year-old white woman with a 1 1/2-year history of refractory dermatitis (most severe on the lower extremities) had the glucagonoma syndrome. Her severe morbidity was markedly relieved with the administration of intravenous amino acids. This therapy was successful in controlling the necrolytic migratory erythema through recurrences after somatostatin (SMS 201-995), surgical debulking, and chemotherapy proved inadequate.
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PMID:Treatment of necrolytic migratory erythema in glucagonoma syndrome. 176 71

A newly recognized disease in dogs, ulcerative dermatosis associated with diabetes mellitus (diabetic dermatopathy), was diagnosed in 2 dogs with pancreatic endocrine tumors that had immunohistologic evidence of glucagon production. Dogs developed diabetes mellitus in the later stages of the illness, months after the skin disease was first observed. Liver disease was identified and characterized by high serum alkaline phosphatase and alanine transaminase activities. Clinically, erythema and crusting involved the footpads, the face, perioral and genital skin, and ventrum. Histologically, skin lesions were intercellular and intracellular edema and necrosis of the upper half of the epidermis and diffuse parakeratosis. Clinically and histologically, skin lesions closely resembled necrolytic migratory erythema of people, a skin disease that usually is associated with a glucagon-secreting pancreatic endocrine tumor and diabetes mellitus (glucagonoma syndrome): The morphologically descriptive term, superficial necrolytic dermatitis, was preferred over the previously proposed names hepatocutaneous syndrome and diabetic dermatopathy, which each connote only a single feature of the disease.
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PMID:Glucagon-producing pancreatic endocrine tumors in two dogs with superficial necrolytic dermatitis. 227 59

The glucagon-producing pancreatic tumors or glucagonomas are among the rarest forms of islet cell tumors; most are malignant and usually produce a definite clinical syndrome. Mild diabetes mellitus, weight loss, and anemia usually accompany the syndrome. However, only the presence of a peculiar cutaneous rash (necrolytic migratory erythema) and the finding of hyperglucagonemia on assay are reliable diagnostic features of the syndrome. Selective, celiac axis arteriography is the most valuable preoperative technique for localizing these neoplasms and their common liver metastases. Immunohistochemical and ultrastructural examinations are particularly helpful in defining the tumor cell nature (alpha-2 islet cell) and the peptide content (glucagon). When the tumor is benign (less than 30%), complete operative removal results in lasting cure; for malignant forms, surgical therapy is mainly palliative, and adjunctive chemotherapy should be administered. In this report, the importance of clinical recognition and operative and chemotherapeutic responses is illustrated in two patients. In each case, the characteristic dermatitis, diabetes mellitus, weight loss, anemia, and elevated plasmatic glucagon were present. Both patients had their tumors localized by selective angiography and underwent operative removal of the primary pancreatic lesion. In the case of benign glucagonoma, surgical excision was curative. In the malignant one, cytoreductive surgery plus adjunctive chemotherapy (dimethyltriazenomidazole-carboxamide resulted in prolonged survival and significant clinical improvement. Follow-up with serum glucagon assay has been useful in monitoring recurrence.
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PMID:Response of glucagonomas to surgical excision and chemotherapy. Report of two cases and review of the literature. 254 27

We report 1 patient with a necrolytic migratory erythema, a high plasma glucagon concentration and a metastatic pancreatic endocrine tumor who has now been treated effectively for 33 months with the somatostatin analogue octreotide (SMS 201-995) (400 micrograms/day). The results of SMS 201-995 in the treatment of glucagonoma syndrome are reviewed.
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PMID:Octreotide (SMS 201-995) in the treatment of metastatic glucagonoma: report of one case and review of the literature. 254 11

Necrolytic migratory erythema is the distinctive skin rash of the glucagonoma syndrome. Its presence is virtually pathognomonic of a glucagon-producing pancreatic islet cell neoplasm. Results of a study of a patient with hyperglucagonemia and necrolytic migratory erythema complicating untreated celiac disease are reported. Whereas pancreatic glucagon was only mildly elevated, there was marked elevation of enteroglucagon. Immunofluorescence staining demonstrated numerous (19.6 cells per square millimeter of mucosa) enteroglucagon-positive small intestinal crypt cells. Treatment with gluten-free diet not only resulted in resolution of malabsorption and improvement in small intestinal histology but was paralleled by disappearance of necrolytic migratory erythema, normalization of plasma glucagon levels, and marked reduction in the number of enteroglucagon-producing crypt cells (0.2/mm2 mucosa). The findings demonstrate that necrolytic migratory erythema is not an exclusively paraneoplastic phenomenon and that it can occur in association with excess production of enteroglucagon by the intestinal mucosa.
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PMID:Necrolytic migratory erythema with elevated plasma enteroglucagon in celiac disease. 270 19

A 62-year-old man had necrolytic migratory erythema in association with hyperglucagonemia and multiple hepatic tumors. A diagnosis of metastatic glucagonoma was made. He was treated with human lymphoblastoid interferon given subcutaneously in the dose of 2.8 to 7.1 X 10(6) IU/day. This produced a considerable fall in plasma glucagon and resolution of the associated rash. The treatment was continued for 10 weeks over a 4-month period. Computed tomography demonstrated a reduction in hepatic tumor mass.
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PMID:Hepatic tumors with hyperglucagonemia. Response to treatment with human lymphoblastoid interferon. 284 25


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