BIOMAT Database Logo BIOMAT Database Logo

The glucagonoma syndrome. A distinctive cutaneous marker of systemic disease. 1978

K H Swenson, and R B Amon, and J M Hanifin

The glucagonoma syndrome is a rare clinical condition characterized by a distinctive cutaneous eruption associated with a glucagon-secreting islet cell neoplasm of the pancreas. A 19-year-old woman manifested typical features of this condition: a polymorphous skin eruption with characteristic distribution of lesions in perioral and paragenital regions; lesions in sites of cutaneous trauma; a skin biopsy that showed epidermal cleavage; glossitis; weight loss; mild anemia; abnormal glucose tolerance test results. Plasma glucagon levels, determined by radioimmunoassay, were approximately five times normal. Angiography indicated a pancreatic tumor with liver metastases. Islet cell origin was confirmed histologically. It is hoped that wider recognition of the distinctive clinical features of this syndrome will result in earlier detection and possible surgical cure of the underlying malignancy.

UI MeSH Term Description Entries
D007516 Adenoma, Islet Cell A benign tumor of the pancreatic ISLET CELLS. Usually it involves the INSULIN-producing PANCREATIC BETA CELLS, as in INSULINOMA, resulting in HYPERINSULINISM. Islet Cell Tumor,Islet of Langerhans Tumor,Nesidioblastoma,Pancreatic Islet Cell Tumors,Island Cell Tumor,Adenomas, Islet Cell,Island Cell Tumors,Islet Cell Adenoma,Islet Cell Adenomas,Islet Cell Tumors,Langerhans Tumor Islet,Nesidioblastomas,Tumor Islet, Langerhans,Tumor, Island Cell,Tumor, Islet Cell,Tumors, Island Cell,Tumors, Islet Cell
D010190 Pancreatic Neoplasms Tumors or cancer of the PANCREAS. Depending on the types of ISLET CELLS present in the tumors, various hormones can be secreted: GLUCAGON from PANCREATIC ALPHA CELLS; INSULIN from PANCREATIC BETA CELLS; and SOMATOSTATIN from the SOMATOSTATIN-SECRETING CELLS. Most are malignant except the insulin-producing tumors (INSULINOMA). Cancer of Pancreas,Pancreatic Cancer,Cancer of the Pancreas,Neoplasms, Pancreatic,Pancreas Cancer,Pancreas Neoplasms,Pancreatic Acinar Carcinoma,Pancreatic Carcinoma,Acinar Carcinoma, Pancreatic,Acinar Carcinomas, Pancreatic,Cancer, Pancreas,Cancer, Pancreatic,Cancers, Pancreas,Cancers, Pancreatic,Carcinoma, Pancreatic,Carcinoma, Pancreatic Acinar,Carcinomas, Pancreatic,Carcinomas, Pancreatic Acinar,Neoplasm, Pancreas,Neoplasm, Pancreatic,Neoplasms, Pancreas,Pancreas Cancers,Pancreas Neoplasm,Pancreatic Acinar Carcinomas,Pancreatic Cancers,Pancreatic Carcinomas,Pancreatic Neoplasm
D005260 Female Females
D005934 Glucagon A 29-amino acid pancreatic peptide derived from proglucagon which is also the precursor of intestinal GLUCAGON-LIKE PEPTIDES. Glucagon is secreted by PANCREATIC ALPHA CELLS and plays an important role in regulation of BLOOD GLUCOSE concentration, ketone metabolism, and several other biochemical and physiological processes. (From Gilman et al., Goodman and Gilman's The Pharmacological Basis of Therapeutics, 9th ed, p1511) Glucagon (1-29),Glukagon,HG-Factor,Hyperglycemic-Glycogenolytic Factor,Proglucagon (33-61),HG Factor,Hyperglycemic Glycogenolytic Factor
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D012877 Skin Manifestations Dermatologic disorders attendant upon non-dermatologic disease or injury. Manifestation, Skin,Manifestations, Skin,Skin Manifestation
D013577 Syndrome A characteristic symptom complex. Symptom Cluster,Cluster, Symptom,Clusters, Symptom,Symptom Clusters,Syndromes

Related Publications

K H Swenson, and R B Amon, and J M Hanifin
[Glucagonoma syndrome : cutaneous manifestations].
January 1978, Annales de dermatologie et de venereologie,
K H Swenson, and R B Amon, and J M Hanifin
Necrolytic migratory erythema. Distinctive dermatosis of the glucagonoma syndrome.
June 1977, Archives of dermatology,
K H Swenson, and R B Amon, and J M Hanifin
Adult-onset Still's disease as a cutaneous marker of systemic disease.
January 2019, Clinics in dermatology,
K H Swenson, and R B Amon, and J M Hanifin
Necrolytic migratory erythema: a cutaneous clue to glucagonoma syndrome.
September 2004, Journal of the European Academy of Dermatology and Venereology : JEADV,
K H Swenson, and R B Amon, and J M Hanifin
Necrolytic migratory erythema: the outermost marker for glucagonoma syndrome.
September 2007, Archives of dermatology,
K H Swenson, and R B Amon, and J M Hanifin
Cutaneous bacillary angiomatosis: a marker of systemic disease in HIV.
April 2009, South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde,
K H Swenson, and R B Amon, and J M Hanifin
Glucagonoma and the glucagonoma syndrome.
March 2018, Oncology letters,
K H Swenson, and R B Amon, and J M Hanifin
[Glucagonoma without the glucagonoma syndrome].
May 1985, Minerva chirurgica,
K H Swenson, and R B Amon, and J M Hanifin
A review of cutaneous manifestations within glucagonoma syndrome: necrolytic migratory erythema.
June 2018, International journal of dermatology,
K H Swenson, and R B Amon, and J M Hanifin
[Glucagonoma without glucagonoma syndrome].
January 2010, Srpski arhiv za celokupno lekarstvo,
Copied contents to your clipboard!