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
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Clinical Picture
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Investigations
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Pictures
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| History
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Symptoms
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Signs
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| Glucagonoma
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- 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, diabetes, hypertension, chronic 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]
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- 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
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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
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| End-stage liver disease
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| Pemphigus foliaceus
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It is an autoimmune blistering disease of the skin with characteristic lesions that are scaly, crusted erosions, often on an erythematous base.[1]
Mucosal involvement is absent even with widespread disease.[2]
The pathway is most likely either of three mechanisms:
- Steric hindrance of the desmoglein 1: The antibody caps off the site for intracellular binding to another keratinocyte.
- Activation of an endocytic pathway: The antibody activates a pathway which causes an internalization of desmoglein 1, which in turn causes a loss of adhesion.
- Disruption of function: In this case, the antibody blocks the desmoglein 1 from being formed into a desmosome. This, in turn, causes a loss of adhesion with acantholysis as a result.
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- Pemphigus foliaceus is a superficial variant of pemphigus that presents with cutaneous lesions. [1]
- Pemphigus foliaceus usually develops in a seborrheic distribution.
- The scalp, face, and trunk are common sites of involvement. The skin lesions usually consist of small, scattered superficial blisters that rapidly evolve into scaly, crusted erosions
- The skin lesions may remain localized or may coalesce to cover large areas of skin. Occasionally, pemphigus foliaceus progresses to involve the entire skin surface as an exfoliative erythroderma [9].
- Pain or burning sensations frequently accompany the cutaneous lesions. Systemic symptoms are usually absent.
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Positive Nikolsky sign [10]
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Autoimmune IgG build up in the epidermis, then nearly almost all of the antibodies are aimed against desmoglein 1
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| Pustular psoriasis
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| Acrodermatitis enteropathica
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- Symptoms appear in infants after breast milk weaning.
- The appearance of erythematous patches and plaques of dry, scaly skin.
- Diarrhea may occur.
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- Erythematous patches and plaques of dry, scaly skin.
- The lesions may appear eczematous or may evolve further into crusted vesicles, bullae or pustules.
- The lesions are frequent around natural orifices like the mouth perioral and anus peri-anal, and also in hands, feet, and scalp.
- There may be suppurative inflammation of the nail fold surrounding the nail plate - known as paronychia.
- Alopecia of the scalp, eyebrows, and eyelashes may occur.
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| Pellagra
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| Chronic eczema
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| kwashiorkor
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References
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