Glucagonoma differential diagnosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2]

Overview

Glucagonoma must be differentiated from certain skin lesions (acrodermatitis enteropathica, psoriasis, pellagra, eczema) and other causes of hyperglucagonemia (infection, diabetes mellitus, Cushing syndrome, renal failure, acute pancreatitis, severe stress, and prolonged fasting).

Differentiating Glucagonoma from other Disease

Glucagonoma must be differentiated from certain skin lesions in which necrolytic migratory erythema can be found and other causes of hyperglucagonemia:[1][2]

Disease Clinical Picture Investigations Pictures
History Symptoms Signs
Glucagonoma
  • The first peak for the development of psoriasis occurs between 20 years to 35 years and the second peak is between 40 years to 65 years of life.
  • Patients with early disease onset often have a positive family history of psoriasis, frequent association with histocompatibility antigen (HLA)- Cw6, and more severe disease. Those with onset after the age of 40 usually have a negative family history and a normal frequency of the HLA- Cw6 allele.[2]
  • A typical patient of psoriasis will present with a history of a long-term erythematous scaly area with ocular and joint involvement depending upon the clinical subtype and chronicity of the disease. There may be multiple relapses and remissions.
  • Past medical history of the patient may include viral or bacterial infection, diabeteshypertensionchronic kidney disease and/or obesity due to an association of psoriasis with these conditions.[3]
  • A social history of the patient may indicate smoking, excessive alcohol consumption and/or a recent stressful event if life associated with an acute exacerbation of psoriasis.[4]
  • A long-term history of erythematous scaly area, which may involve multiple areas of the body    
  • Pain, which has been described by patients as unpleasant, superficial, sensitive, itchy, hot or burning (especially in erythrodermic psoriasis and in some cases of traumatized plaques or in the joints affected by psoriatic arthritis) 
  • Pruritus (especially in eruptive, guttate psoriasis)
  • High fever
  • Dystrophic nails
  • Long-term rash with recent presentation of arthralgia
Skin biopsy

Perivascular and dermal inflammatory cell infiltration

Vascular dilation

Absent granular layer

Elongation of dermal papillae

Parakeratosis

Spongiform pustules of Kogoj (pathognomic of psoriasis)

Munro's micro abscesses (pathognomic of psoriasis)

Edema of dermal papillae

In psoriasis, skin biopsy of the affected area of skin shows that the epidermal/supra-papillary thickness ratio is increased

Basal cell layer is expanded

Leukocytosis

End-stage liver disease
Pemphigus foliaceus 
Pustular psoriasis
Acrodermatitis enteropathica
Pellagra
Chronic eczema
kwashiorkor

References

  1. Glucagonoma. Wikipedia. https://en.wikipedia.org/wiki/Glucagonoma. accessed on October 10, 2015
  2. Fang S, Li S, Cai T (2014). "Glucagonoma syndrome: a case report with focus on skin disorders". Onco Targets Ther. 7: 1449–53. doi:10.2147/OTT.S66285. PMC 4140234. PMID 25152626.


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