Glucagonoma Clinical Presentation

Updated: May 14, 2015
  • Author: Luigi Santacroce, MD; Chief Editor: George T Griffing, MD  more...
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Presentation

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. [9]

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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