Glucagonoma Clinical Presentation

  • Author: Luigi Santacroce, MD; Chief Editor: George T Griffing, MD   more...
 
Updated: May 23, 2011
 

History

  • Glucagonoma initially manifests with a nonspecific clinical scenario characterized, in most cases, by weight loss, diabetes mellitus, diarrhea, and stomatitis.
  • The cutaneous lesions in this phase of the disease can easily be confused with nonspecific dermatitis, which occurs more often in patients with diabetes mellitus.
  • Poorly defined symptoms of this condition sometimes make it difficult to establish a correct differential diagnosis (eg, with a pancreatic adenocarcinoma that causes weight loss, the early stages of dyspepsia, and the later stages of jaundice).
  • The so-called 4D syndrome consists of diabetes, dermatitis, DVT (deep vein thrombosis), and depression.
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Physical

  • The most frequent clinical sign observed in patients with glucagonoma is necrolytic migratory erythema (NME), which is present in 80% of cases and is characterized by an erythematous and swollen area of skin.
    • The symptoms that appear on the skin include (in order of appearance) maculopapular, ringed lesions and blisters that breach after a few days, as well as, possibly, pustular evolution due to bacterial superinfection. The lesions are often confluent, evolve in a period of 1-2 weeks, and are strongly pruritic and painful. They heal with hyperpigmentation.
    • NME initially occurs in areas of the skin that are subject to friction and pressure, such as the feet and legs, the hands and forearms, and the buttocks, pubic area, groin area, and perineal area. Mucocutaneous lesions are frequently observed as atrophic glossitis, commissural cheilitis, stomatitis, balanoposthitis or vulvovaginitis, and ungual and hair dystrophies.
  • Aside from NME, symptoms often observed in glucagonoma are diabetes or glucose intolerance (in 80-90% of cases) and a weight loss of 5-15 kg (in most patients).
  • Symptoms that occur less frequently are venous thromboses, in particular DVT (in approximately 40% of cases); intestinal motility alterations with diarrhea and abdominal pains (in approximately 20% of cases); cachexia; anemia; scotomata and other optical disturbances; and psychical disturbances, such as insomnia, depression, disorientation, lethargy, and bradylalia.
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Causes

The causes of this pathology remain unknown, although some genetic factors could play an important role, especially in patients who have a family history of multiple endocrine neoplasia type 1 syndrome.

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Contributor Information and Disclosures
Author

Luigi Santacroce, MD  Assistant Professor, Medical School, State University at Bari, Italy

Disclosure: Nothing to disclose.

Coauthor(s)

Laura Diomede  University of Bari School of Medicine, Italy

Disclosure: Nothing to disclose.

Mini R Abraham, MD  Consulting Staff, Overland Park Medical Specialists

Mini R Abraham, MD is a member of the following medical societies: American Association of Clinical Endocrinologists and Endocrine Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Frederick H Ziel, MD  Associate Professor of Medicine, David Geffen School of Medicine at UCLA; Physician-In-Charge, Endocrinology/Diabetes Center, Director of Medical Education, Kaiser Permanente Woodland Hills; Chair of Endocrinology, Co-Chair of Diabetes Complete Care Program, Southern California Permanente Medical Group

Frederick H Ziel, MD is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Endocrinology, American College of Physicians, American College of Physicians-American Society of Internal Medicine, American Diabetes Association, American Federation for Medical Research, American Medical Association, American Society for Bone and Mineral Research, California Medical Association, Endocrine Society, and International Society for Clinical Densitometry

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: eMedicine Salary Employment

Romesh Khardori, MD, PhD, FACP  Former Professor, Department of Medicine, Former Chief, Division of Endocrinology, Metabolism, and Molecular Medicine, Southern Illinois University School of Medicine

Romesh Khardori, MD, PhD, FACP is a member of the following medical societies: American Association of Clinical Endocrinologists, American College of Physicians, American Diabetes Association, and Endocrine Society

Disclosure: Nothing to disclose.

Mark Cooper, MBBS, PhD, FRACP  Head, Diabetes & Metabolism Division, Baker Heart Research Institute, Professor of Medicine, Monash University

Disclosure: Nothing to disclose.

Chief Editor

George T Griffing, MD  Professor of Medicine, St Louis University School of Medicine

George T Griffing, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Medical Practice Executives, American College of Physician Executives, American College of Physicians, American Diabetes Association, American Federation for Medical Research, American Heart Association, Central Society for Clinical Research, Endocrine Society, International Society for Clinical Densitometry, and Southern Society for Clinical Investigation

Disclosure: Nothing to disclose.

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A section of a glucagonoma mass with several fiber bundles and solid cellular strands (125 X). Courtesy of Professor Pantaleo Bufo, University of Foggia, Italy.
A section of a glucagonoma mass with irregular aspects of fiber bundles and cellular strands (400 X). Courtesy of Professor Pantaleo Bufo, University of Foggia, Italy.
 
 
 
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