Glucagonoma differential diagnosis: Difference between revisions
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|Glucagonoma | |Glucagonoma | ||
|A family history of | |A family history of [[multiple endocrine neoplasia type 1]] | ||
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* [[Necrolytic migratory erythema]] | * [[Necrolytic migratory erythema]] characterized by the spread of erythematous blisters and swelling across areas subject to greater friction and pressure, including the lower [[abdomen]], [[Buttock|buttocks]], [[perineum]], and [[groin]].<sup>[[Glucagonoma history and symptoms#cite note-pmid4793623-2|[2]]]</sup> | ||
* [[Weight loss]]<sup>[[Glucagonoma history and symptoms#cite note-pmid86066272-3|[3]]]</sup> | * [[Weight loss]]<sup>[[Glucagonoma history and symptoms#cite note-pmid86066272-3|[3]]]</sup> | ||
* [[Glucose intolerance]]<sup>[[Glucagonoma history and symptoms#cite note-pmid6268399-4|[4]]]</sup> | * [[Glucose intolerance]]<sup>[[Glucagonoma history and symptoms#cite note-pmid6268399-4|[4]]]</sup> | ||
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* [[Rash|Rash:]] Erythematous, ring shaped [[rash]] that blisters, erodes, and crusts over suggesting [[necrolytic migratory erythema]]. | * [[Rash|Rash:]] Erythematous, ring shaped [[rash]] that blisters, erodes, and crusts over suggesting [[necrolytic migratory erythema]]. | ||
* Muscle [[atrophy]] may be present. | * Muscle [[atrophy]] may be present. | ||
* Unilateral calf or thigh erythema, swelling | * Unilateral calf or thigh erythema, and swelling. | ||
* Hyporeflexia | * Hyporeflexia | ||
* Unilateral/bilateral [[sensory loss]] in the upper/lower extremity | * Unilateral/bilateral [[sensory loss]] in the upper/lower extremity | ||
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* '''Serum glucagon'''<sup>[[Glucagonoma laboratory tests#cite note-pmid15313692-1|[1]]]</sup> | * '''Serum glucagon'''<sup>[[Glucagonoma laboratory tests#cite note-pmid15313692-1|[1]]]</sup> | ||
** Increased plasma glucagon levels (>500 pg/mL).<sup>[[Glucagonoma laboratory tests#cite note-pmid8606627-3|[3]]]</sup> | ** Increased plasma glucagon levels (>500 pg/mL).<sup>[[Glucagonoma laboratory tests#cite note-pmid8606627-3|[3]]]</sup> | ||
** Concentrations above 1000 pg/mL are diagnostic of glucagonoma.<sup>[[Glucagonoma laboratory tests#cite note-pmid17873310-4|[4]]]</sup> | ** Concentrations above 1000 pg/mL are diagnostic of glucagonoma.<sup>[[Glucagonoma laboratory tests#cite note-pmid17873310-4|[4]]]</sup> | ||
* CT scans are used to determine the location of the tumor, show the organs nearby | * CT scans are used to determine the location of the tumor, show the organs nearby. Liver metastases may appear isodense with the liver on a non-contrasted study.<sup>[[Glucagonoma CT#cite note-pmid9574609-4|[4]]]</sup> | ||
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|[[Pemphigus foliaceus]] | |[[Pemphigus foliaceus]] | ||
| | |Autoimmune blistering disease of the skin with characteristic lesions that are scaly, crusted erosions, often on an erythematous base.<sup>[[Pemphigus foliaceus#cite note-Fitz2-1|[1]]]</sup> | ||
Mucosal involvement is absent even with widespread disease.<sup>[[Pemphigus foliaceus#cite note-Bolognia-2|[2]]]</sup> | Mucosal involvement is absent even with widespread disease.<sup>[[Pemphigus foliaceus#cite note-Bolognia-2|[2]]]</sup> | ||
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* | * The main symptom is a cutaneous lesion that usually develops in a seborrheic distribution:<ref name="pmid15993235">{{cite journal| author=Bystryn JC, Rudolph JL| title=Pemphigus. | journal=Lancet | year= 2005 | volume= 366 | issue= 9479 | pages= 61-73 | pmid=15993235 | doi=10.1016/S0140-6736(05)66829-8 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15993235 }}</ref>the scalp, face, and trunk are common sites of involvement. | ||
<ref name="pmid15993235">{{cite journal| author=Bystryn JC, Rudolph JL| title=Pemphigus. | journal=Lancet | year= 2005 | volume= 366 | issue= 9479 | pages= 61-73 | pmid=15993235 | doi=10.1016/S0140-6736(05)66829-8 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15993235 }}</ref> | * The skin lesions may remain localized or may coalesce to cover large areas of skin. | ||
* The skin lesions may remain localized or may coalesce to cover large areas of skin. | |||
* Pain or burning sensations frequently accompany the cutaneous lesions. Systemic symptoms are usually absent. | * Pain or burning sensations frequently accompany the cutaneous lesions. Systemic symptoms are usually absent. | ||
|Positive Nikolsky sign | | | ||
* The skin lesions usually consist of small, scattered superficial blisters that rapidly evolve into scaly, crusted erosions | |||
* Occasionally, pemphigus foliaceus progresses to involve the entire skin surface as an exfoliative erythroderma.<ref name="pmid159414332">{{cite journal| author=Chams-Davatchi C, Valikhani M, Daneshpazhooh M, Esmaili N, Balighi K, Hallaji Z et al.| title=Pemphigus: analysis of 1209 cases. | journal=Int J Dermatol | year= 2005 | volume= 44 | issue= 6 | pages= 470-6 | pmid=15941433 | doi=10.1111/j.1365-4632.2004.02501.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15941433 }}</ref> | |||
* Positive Nikolsky sign<ref name="pmid21353333">{{cite journal| author=Martin LK, Werth VP, Villaneuva EV, Murrell DF| title=A systematic review of randomized controlled trials for pemphigus vulgaris and pemphigus foliaceus. | journal=J Am Acad Dermatol | year= 2011 | volume= 64 | issue= 5 | pages= 903-8 | pmid=21353333 | doi=10.1016/j.jaad.2010.04.039 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21353333 }}</ref> | |||
|Autoimmune [[IgG]] build up in the [[Epidermis (skin)|epidermis]], then nearly almost all of the antibodies are aimed against [[desmoglein 1]] | |Autoimmune [[IgG]] build up in the [[Epidermis (skin)|epidermis]], then nearly almost all of the antibodies are aimed against [[desmoglein 1]] | ||
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|[[Psoriasis|Pustular psoriasis]] | |[[Psoriasis|Pustular psoriasis]] | ||
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* | * Positive family history of psoriasis, frequent association with [[histocompatibility]] [[antigen]] (HLA)- Cw6.<sup>[[Psoriasis history and symptoms#cite note-pmid1390163-2|[2]]]</sup> | ||
* A | * A history of a long-term [[erythematous]] scaly area with [[ocular]] and [[joint]] involvement. | ||
* Past medical history of the patient may include [[viral]] or [[bacterial]] infection, [[Diabetes mellitus|diabetes]], [[hypertension]], [[chronic kidney disease]] and/or [[obesity]] | * Past medical history of the patient may include [[viral]] or [[bacterial]] infection, [[Diabetes mellitus|diabetes]], [[hypertension]], [[chronic kidney disease]] and/or [[obesity]].<sup>[[Psoriasis history and symptoms#cite note-pmid24790463-3|[3]]]</sup> | ||
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* [[Pain]], which has been described by patients as unpleasant, superficial, sensitive, itchy, hot or burning. | |||
* [[Pain]], which has been described by patients as unpleasant, superficial, sensitive, itchy, hot or | * [[Pruritus]] | ||
* [[Pruritus]] | |||
* High [[fever]] | * High [[fever]] | ||
* Dystrophic nails | * Dystrophic nails | ||
* | * Recent presentation of [[arthralgia]] | ||
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* [[Papulosquamous disorder|Papulosquamous]] [[disease]] with variable morphology, distribution, severity, and course | * [[Papulosquamous disorder|Papulosquamous]] [[disease]] with variable morphology, distribution, severity, and course | ||
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* [[Woronoff|Woronoff’s ring]]: ring of peripheral blanching skin around a psoriatic [[plaque]] | * [[Woronoff|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. | * Auspitz’s sign: small [[bleeding]] points are seen upon disruption of a psoriatic scale. | ||
|'''Skin biopsy''' | | | ||
* '''Skin biopsy''' | |||
Perivascular and [[dermal]] [[Inflammatory cells|inflammatory cell]] infiltration | * Perivascular and [[dermal]] [[Inflammatory cells|inflammatory cell]] infiltration | ||
* [[Vascular]] dilation | |||
[[Vascular]] dilation | * Absent [[granular layer]] | ||
* Elongation of [[dermal]] [[Papilla|papillae]] | |||
Absent [[granular layer]] | * Parakeratosis | ||
* Spongiform [[pustules]] of Kogoj (pathognomic of psoriasis) | |||
Elongation of [[dermal]] [[Papilla|papillae]] | * Munro's micro abscesses (pathognomic of psoriasis) | ||
* [[Edema]] of [[dermal]] [[papillae]] | |||
Parakeratosis | * [[Basal cell layer]] is expanded | ||
* Leukocytosis | |||
Spongiform [[pustules]] of Kogoj (pathognomic of psoriasis) | |||
Munro's micro abscesses (pathognomic of psoriasis) | |||
[[Edema]] of [[dermal]] [[papillae]] | |||
[[Basal cell layer]] is expanded | |||
Leukocytosis | |||
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|[[Acrodermatitis enteropathica]] | |[[Acrodermatitis enteropathica]] | ||
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* | * 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. | * The genetic base is a [[mutation]] of [[SLC39A4]] which encodes a [[transmembrane protein]] that serves as a zinc uptake protein. | ||
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* The lesions may appear eczematous or may evolve further into crusted vesicles, bullae or pustules. | * 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]]. | * The lesions are frequent around natural orifices like the mouth perioral and anus peri-anal, and also in hands, feet, and [[scalp]]. | ||
* | * [[paronychia]] | ||
* Alopecia of the scalp, eyebrows, and | * Alopecia of the scalp, eyebrows, and eyelashes | ||
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* Measurement of zinc in plasma, erythrocytes, neutrophils, lymphocytes, and hair. | * Measurement of zinc in plasma, erythrocytes, neutrophils, lymphocytes, and hair. | ||
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|[[Pellagra]] | |[[Pellagra]] | ||
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* It is | * It is a niacin deficiency disease characterized by a photosensitive pigmented dermatitis, diarrhea, and dementia. | ||
* Hisotry of alcoholics, bariatric surgery, anorexia nervosa, or malabsorptive disease.<ref name="pmid12777163">{{cite journal| author=Prousky JE| title=Pellagra may be a rare secondary complication of anorexia nervosa: a systematic review of the literature. | journal=Altern Med Rev | year= 2003 | volume= 8 | issue= 2 | pages= 180-5 | pmid=12777163 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12777163 }}</ref> | * Hisotry of alcoholics, bariatric surgery, anorexia nervosa, or malabsorptive disease.<ref name="pmid12777163">{{cite journal| author=Prousky JE| title=Pellagra may be a rare secondary complication of anorexia nervosa: a systematic review of the literature. | journal=Altern Med Rev | year= 2003 | volume= 8 | issue= 2 | pages= 180-5 | pmid=12777163 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12777163 }}</ref> | ||
* Dietary deficiency especially in infants | * Dietary deficiency especially in infants | ||
* Past history of Carcinoid syndrome | * Past history of Carcinoid syndrome | ||
* Prolonged use of isoniazid | * Prolonged use of isoniazid<ref name="pmid21128910">{{cite journal| author=Wan P, Moat S, Anstey A| title=Pellagra: a review with emphasis on photosensitivity. | journal=Br J Dermatol | year= 2011 | volume= 164 | issue= 6 | pages= 1188-200 | pmid=21128910 | doi=10.1111/j.1365-2133.2010.10163.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21128910 }}</ref> | ||
* A family history of Hartnup disease | * A family history of Hartnup disease | ||
|N | |||
|Symmetric hyper pigmented rash, similar in color and distribution to a sunburn, which is present in the exposed areas of skin. | |||
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|Niacin status can be assessed by measuring urinary N-methylnicotinamide or by measuring the erythrocyte NAD/NADP (ratio). | |Niacin status can be assessed by measuring urinary N-methylnicotinamide or by measuring the erythrocyte NAD/NADP (ratio). | ||
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|[[eczema|Chronic eczema]] (atopic dermatitis) | |[[eczema|Chronic eczema]] (atopic dermatitis) | ||
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* | * It 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) | * A family history of atopy (eczema, asthma, or allergic rhinitis) | ||
* History of dermatitis involving the skin creases | * History of dermatitis involving the skin creases | ||
* Personal or family history of asthma or hay fever | * Personal or family history of asthma or hay fever | ||
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* 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. | * 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. | ||
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* Erythema, papulation, oozing and crusting, excoriation. | * Erythema, papulation, oozing and crusting, excoriation. | ||
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* Raised [[IgE]] or an [[eosinophilia]] | * Raised [[IgE]] or an [[eosinophilia]] | ||
* [[RAST test|Radioallergosorbent Test]] | * [[RAST test|Radioallergosorbent 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 | * High levels of antibodies in the blood signify an allergy to that substance | ||
* Skin biopsy | * Skin biopsy: a procedure that removes a small piece of the affected skin and sent for microscopic examination in a pathology laboratory. | ||
|} | |} | ||
Revision as of 18:51, 3 August 2017
Glucagonoma Microchapters |
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Glucagonoma differential diagnosis On the Web |
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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 Diseases
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 | ||
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History | Symptoms | Signs | |||
Glucagonoma | A family history of multiple endocrine neoplasia type 1 |
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Pemphigus foliaceus | 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] |
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Autoimmune IgG build up in the epidermis, then nearly almost all of the antibodies are aimed against desmoglein 1 | |
Pustular psoriasis |
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| |
Acrodermatitis enteropathica |
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Pellagra |
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N | 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) |
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References
- ↑ Glucagonoma. Wikipedia. https://en.wikipedia.org/wiki/Glucagonoma. accessed on October 10, 2015
- ↑ 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.
- ↑ Bystryn JC, Rudolph JL (2005). "Pemphigus". Lancet. 366 (9479): 61–73. doi:10.1016/S0140-6736(05)66829-8. PMID 15993235.
- ↑ Chams-Davatchi C, Valikhani M, Daneshpazhooh M, Esmaili N, Balighi K, Hallaji Z; et al. (2005). "Pemphigus: analysis of 1209 cases". Int J Dermatol. 44 (6): 470–6. doi:10.1111/j.1365-4632.2004.02501.x. PMID 15941433.
- ↑ Martin LK, Werth VP, Villaneuva EV, Murrell DF (2011). "A systematic review of randomized controlled trials for pemphigus vulgaris and pemphigus foliaceus". J Am Acad Dermatol. 64 (5): 903–8. doi:10.1016/j.jaad.2010.04.039. PMID 21353333.
- ↑ Prasad AS, Cossack ZT (1984). "Zinc supplementation and growth in sickle cell disease". Ann Intern Med. 100 (3): 367–71. PMID 6696358.
- ↑ Meftah S, Prasad AS, Lee DY, Brewer GJ (1991). "Ecto 5' nucleotidase (5'NT) as a sensitive indicator of human zinc deficiency". J Lab Clin Med. 118 (4): 309–16. PMID 1940572.
- ↑ Kiliç I, Ozalp I, Coŝkun T, Tokatli A, Emre S, Saldamli I; et al. (1998). "The effect of zinc-supplemented bread consumption on school children with asymptomatic zinc deficiency". J Pediatr Gastroenterol Nutr. 26 (2): 167–71. PMID 9481631.
- ↑ Prousky JE (2003). "Pellagra may be a rare secondary complication of anorexia nervosa: a systematic review of the literature". Altern Med Rev. 8 (2): 180–5. PMID 12777163.
- ↑ Wan P, Moat S, Anstey A (2011). "Pellagra: a review with emphasis on photosensitivity". Br J Dermatol. 164 (6): 1188–200. doi:10.1111/j.1365-2133.2010.10163.x. PMID 21128910.