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:

Disease	Clinical Picture			Investigations	Pictures
	History	Symptoms	Signs
Glucagonoma	A family history of predisposing genetic disorders such as multiple endocrine neoplasia type 1	Necrolytic migratory erythema (NME) is a classical symptom observed in patients with glucagonoma and is present in 80% of cases. Associated NME is characterized by the spread of erythematous blisters and swelling across areas subject to greater friction and pressure, including the lower abdomen, buttocks, perineum, and groin.[2] Weight loss[3] Glucose intolerance[4]	Rash: Erythematous, ring shaped rash that blisters, erodes, and crusts over suggesting necrolytic migratory erythema. Muscle atrophy may be present. Unilateral calf or thigh erythema, swelling, and erythema. Hyporeflexia may be present Unilateral/bilateral sensory loss in the upper/lower extremity may be present	Serum glucagon[1] Normal glucagon level is less than 50 pg/mL. Increased plasma glucagon levels (>500 pg/mL).[3] Concentrations above 1000 pg/mL are diagnostic of glucagonoma.[4] CT scans are used to determine the location of the tumor, show the organs nearby, determining the stage of cancer and in determining whether surgery is a good treatment option. Sensitivity is >80 percent but it is decreased for tumors smaller than 2 cm.[2] Liver metastases may appear isodense with the liver on a non-contrasted study.[4]
End-stage liver disease
Pemphigus foliaceus	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.	Pemphigus foliaceus is a superficial variant of pemphigus that presents with cutaneous lesions. 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. Pain or burning sensations frequently accompany the cutaneous lesions. Systemic symptoms are usually absent.	Positive Nikolsky sign.	Autoimmune IgG build up in the epidermis, then nearly almost all of the antibodies are aimed against desmoglein 1
Pustular psoriasis	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 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	Papulosquamous disease with variable morphology, distribution, severity, and course Scaling papules and plaques Koebner phenomenon: appearance of new psoriatic lesions at the site of skin injury Woronoff’s ring: ring of peripheral blanching skin around a psoriatic plaque Auspitz’s sign: small bleeding points are seen upon disruption of a psoriatic scale.	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
Acrodermatitis enteropathica	It is an autosomal recessive disorder characterized by periorificial and acral dermatitis, alopecia, and diarrhea. The genetic base is a mutation of SLC39A4 which encodes a transmembrane protein that serves as a zinc uptake protein.	Symptoms appear in infants after breast milk weaning. The appearance of erythematous patches and plaques of dry, scaly skin. Diarrhea may occur.	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.	Measurement of zinc in plasma, erythrocytes, neutrophils, lymphocytes, and hair. A low plasma zinc usually is defined as a value less than 60 mcg/dL. Zinc levels in neutrophils or lymphocytes may be more sensitive than plasma zinc. The criteria for zinc deficiency are decreased zinc level in either lymphocyte. Depressed serum alkaline phosphatase levels for age provide supportive evidence for zinc deficiency.
Pellagra	It is Niacin deficincy disease characterized by a photosensitive pigmented dermatitis (typically located in sun-exposed areas), diarrhea, and dementia. Hisotry of alcoholics, bariatric surgery, anorexia nervosa, or malabsorptive disease. Dietary deficiency especially in infants Past history of Carcinoid syndrome, in which metabolism of tryptophan is to 5-OH tryptophan and serotonin rather than to nicotinic acid. This leads to the deficiency of active forms of niacin and the development of pellagra. Prolonged use of isoniazid. A family history of Hartnup disease which is a congenital defect of a membrane transport in the intestinal and renal cells normally responsible for the absorption of tryptophan.	Photosensitivity Pigmented dermatitis (typically located in sun-exposed areas) Diarrhea Dementia Glossitis Insomnia Weakness Mental confusion	The most characteristic finding is the presence of a symmetric hyper pigmented rash, similar in color and distribution to a sunburn, which is present in the exposed areas of skin.	Niacin status can be assessed by measuring urinary N-methylnicotinamide or by measuring the erythrocyte NAD/NADP (ratio).
Chronic eczema (atopic dermatitis)	Atopic dermatitis is a chronic pruritic inflammatory skin disease that occurs most frequently in children but also affects adults. A family history of atopy (eczema, asthma, or allergic rhinitis) and the loss-of-function mutations in the filaggrin (FLG) gene, involved in the skin barrier function, are major risk factors for atopic dermatitis. History of dermatitis involving the skin creases. Personal or family history of asthma or hay fever.	Symptoms beginning in a child before the age of 2 years or, in children <4 years, dermatitis affecting the cheeks or dorsal aspect of extremities. Dry skin and severe pruritus that is associated with cutaneous hyperreactivity to various environmental stimuli including exposure to food and inhalant allergens, irritants, and infection.	Visible dermatitis involving flexural surfaces. Presence of generally dry skin within the past year Erythema, papulation, oozing and crusting, excoriation.	Raised IgE or an eosinophilia. Radioallergosorbent Test Paper Radioimmunosorbent Test: in the test, blood is mixed separately with many different allergens and the antibody levels measured. High levels of antibodies in the blood signify an allergy to that substance. Skin biopsy which is a procedure that removes a small piece of the affected skin that is sent for microscopic examination in a pathology laboratory.

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