The Glucagonoma Syndrome

Swenson, Keith H.; Amon, Robert B.; Hanifin, Jon M.

doi:10.1001/archderm.1978.01640140042010

Cited by 30 publications

(9 citation statements)

References 14 publications

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“…Fusiform keratinocytes with pyknotic nuclei are found, and a mononuclear inflammatory infiltrate is often present. All immunofluorescent studies have been negative (60,65,89). This characteristic histological picture is best seen from biopsy of a fresh lesion (Fig.…”

Section: Cutaneous Lesionsmentioning

confidence: 89%

“…In healthy subjects, plasma glucagon is increased by hypoglycemia (56), mixed meals (57), arginine infusion (41,56,57), or a-adrenergic stimulation (58); is decreased by glucose administration (59,60); and is unchanged (41,(61)(62)(63)(64) by secretin. Less predictable patterns, however, are found in tumor-bearing patients.…”

Section: Summer 1981mentioning

confidence: 99%

“…Almost always the initial lesions appear centrally, around the perineum or on the thighs, and spread peripherally. A purulent crust may develop in later stages and harbor bacteria or fungi (3,13,15,60,79,84,85,87,89,122). Glossitis, stomatitis, and angular chelitis may occur in association with the rash (3, 12-14, 18-20, 34, 35, 51, 65, 71, 77, 79, 83, 84, 86-89, 94, 105, 108).…”

Section: Cutaneous Lesionsmentioning

confidence: 99%

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The Glucagonoma Syndrome: Clinical Features, Diagnosis, and Treatment

1981

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Historical BackgroundIslet cell tumors of the pancreas have been described for over 50 yr. Although carcinoma arising from islet tissue had been noted since the turn of the century (1), not until 1927 was the first case of a hormonally active pancreatic neoplasm, an insulinoma, recorded (2). For many years thereafter, pancreatic endocrine tumors were characterized as either insulin-producing or "nonfunctional," the latter being derived from cells apparently devoid of clinically significant hormonal activity. With time, however, other islet cell secretory products were discovered and their tumors identified with various clinical syndromes. Whereas the hypoglycemia of insulinomas and hyperacidity of gastrinomas figured prominently in the initial recognition of these neoplasms, the clinical signs of glucagon-producing tumors were less readily apparent and not as easily attributable to specific hormone excess.Becker and co-workers (3) are credited with the first description of a glucagonoma. Their case, reported in 1947 but diagnosable only in retrospect, typifies what is now regarded as a classic clinical presentation of the syndrome. The patient, a 45-yr-old woman, sought attention because of a skin rash that, over a period of 8 months, progressed to involve almost her entire body. Weight loss, weakness, and amenorrhea were additional complaints. The rash was symmetrically distributed and characterized by confluent, pruritic areas of macular, erythematous, and vesicular erruptions showing evidence of exfoliation and superficial necrosis. Stomatitis and onchonolysis were also found. Laboratory examination revealed a normochromic anemia, mild fasting hyperglycemia, and hypoproteinemia. Deep venous thrombosis developed while the patient was in hospital, and she succumbed to cardiorespiratory failure. At autopsy, an islet cell neoplasm was discovered "replacing the body

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Section: Cutaneous Lesionsmentioning

confidence: 89%

Section: Summer 1981mentioning

confidence: 99%

Section: Cutaneous Lesionsmentioning

confidence: 99%

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The Glucagonoma Syndrome: Clinical Features, Diagnosis, and Treatment

1981

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“…Cases with glucagonoma syndrome are often detected by dermatologists (Binnick et al, 1977;Church and Crane, 1967;Gossner and Korting, 1960;Kahan et al, 1977 ;Pederson et al, 1976;Sweet, 1974;Swenson et al, 1978;Warin, 1974;Wilkinson, 1971 and1973) because of its peculiar skin lesions, although three of the four cases with glucagonoma reported by Recant et al (1976) lacked skin eruptions. In our case also the syndrome was suspected by a dermatologist.…”

Section: Immunochemical Analysis Of the Tissuesmentioning

confidence: 99%

A case with glucagonoma syndrome -endocrine and metabolic studies-.

et al. 1981

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“…Three of the 27 previously reported cases had a tumor localized to the pancreas, which was resected for cure with prompt regression of the dermatologic manifestations of the disease. In 17 patients in whom sites of metastases were indicated, 16 were confined to or associated with liver involvement. Most commonly, the primary tumor is located in the body or tail of the pancreas: only 2 patients had tumors in the pancreatic head.…”

Section: Fig 7 Electron Micrograph Of Normal Islet Cells With Doublmentioning

confidence: 99%

Pancreatic alpha cell tumors: Case report and review of the literature

Lokich

Anderson

Rossini

et al. 1980

Cancer

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The authors thank Mrs. Nancy Carlson for her technical assistance in electron microscopy, Ms. Rose O'Reilly for preparation of the manuscript, and Dr. Carl Hoar for performing the surgical exploration.Accepted for publication May 2, 1979. secreting islet cell carcinoma and describe the use of streptozotocin, which induced complete remission of the clinical syndrome, reversion of the biochemical abnormalities toward normal, and objective decrease in size of the hepatic metastases. Case ReportA 61-year-old woman with known diabetes mellitus was controlled on diet and oral agents since the initial diagnosis in 1965. In 1977, the control of the diabetes was erratic and the patient was admitted for evaluation and insulin regulation. She had had a 50-lb. weight loss as part of the exacerbation of the diabetes, and she required 40 units of NPH insulin to regulate the blood sugar at that hospitalization. Six month later the patient was readmitted with an intensely pruritic rash, and at that point she gave a history of intermittent pruritis and rash dating back to January 1975. The rash was erythematous and appeared initially in the perineum, buttocks, ankles, and inframammary area (Figs. IA, B, C, D, E). It had been treated with a variety of topical medicaments including cortico-steroids. The rash was intermittent, passing through periods of exacerbation and remission not related specifically to topical therapy and leading to residual hyperpigmentation. The cutaneous lesions were considered characteristic of migratory necrotizing erythema, and, therefore, the patient underwent further diagnostic studies including abdominal ultrasound and pancreatic angiography.

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The Glucagonoma Syndrome

Cited by 30 publications

References 14 publications

The Glucagonoma Syndrome: Clinical Features, Diagnosis, and Treatment

The Glucagonoma Syndrome: Clinical Features, Diagnosis, and Treatment

A case with glucagonoma syndrome -endocrine and metabolic studies-.

Pancreatic alpha cell tumors: Case report and review of the literature

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