Glucagonoma differential diagnosis: Difference between revisions
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|Glucagonoma | |Glucagonoma<sup>[[Glucagonoma history and symptoms#cite note-pmid6268399-4|[4]]]</sup><sup>[[Glucagonoma history and symptoms#cite note-pmid4793623-2|[2]]]</sup><sup>[[Glucagonoma history and symptoms#cite note-pmid86066272-3|[3]]]</sup><sup>[[Glucagonoma laboratory tests#cite note-pmid15313692-1|[1]]]</sup><sup>[[Glucagonoma laboratory tests#cite note-pmid8606627-3|[3]]]</sup><sup>[[Glucagonoma laboratory tests#cite note-pmid17873310-4|[4]]]</sup><sup>[[Glucagonoma CT#cite note-pmid9574609-4|[4]]]</sup> | ||
|A family history of [[multiple endocrine neoplasia type 1]] | |A family history of [[multiple endocrine neoplasia type 1]] | ||
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* [[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]] | * [[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]] | ||
* [[Weight loss]] | * [[Weight loss]] | ||
* [[Glucose intolerance]] | * [[Glucose intolerance]] | ||
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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]]. | ||
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* Unilateral/bilateral [[sensory loss]] in the upper/lower extremity | * Unilateral/bilateral [[sensory loss]] in the upper/lower extremity | ||
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* '''Serum glucagon''' | * '''Serum glucagon''' | ||
** Increased plasma glucagon levels (>500 pg/mL). | ** Increased plasma glucagon levels (>500 pg/mL). | ||
** Concentrations above 1000 pg/mL are diagnostic of glucagonoma. | ** Concentrations above 1000 pg/mL are diagnostic of glucagonoma. | ||
* 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. | * 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. | ||
|[[File:NEM1.jpg|center|250px]] | |[[File:NEM1.jpg|center|250px]] | ||
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|[[Pemphigus foliaceus]] | |[[Pemphigus foliaceus]].<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><sup>[[Pemphigus foliaceus#cite note-Bolognia-2|[2]]]</sup><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 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> | |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 | Mucosal involvement is absent even with widespread disease | ||
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* The main symptom is a cutaneous lesion that usually develops in a seborrheic distribution | * The main symptom is a cutaneous lesion that usually develops in a seborrheic distribution; The scalp, face, and trunk are common sites of involvement. | ||
* 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. | ||
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* The skin lesions usually consist of small, scattered superficial blisters that rapidly evolve into scaly, crusted erosions | * 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. | * Occasionally, pemphigus foliaceus progresses to involve the entire skin surface as an exfoliative erythroderma. | ||
|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]] | ||
|[[File:Pemphigus foliaceus08.jpg|center|250px]] | |[[File:Pemphigus foliaceus08.jpg|center|250px]] | ||
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|[[Psoriasis|Pustular psoriasis]] | |[[Psoriasis|Pustular psoriasis]]<sup>[[Psoriasis history and symptoms#cite note-pmid1390163-2|[2]]]</sup><sup>[[Psoriasis history and symptoms#cite note-pmid24790463-3|[3]]]</sup> | ||
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* Positive family history of psoriasis, frequent association with [[histocompatibility]] [[antigen]] (HLA)- Cw6. | * Positive family history of psoriasis, frequent association with [[histocompatibility]] [[antigen]] (HLA)- Cw6. | ||
* A history of a long-term [[erythematous]] scaly area with [[ocular]] and [[joint]] involvement. | * 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]]. | ||
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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 burning. | ||
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|[[File:Pus.png|center|250px]] | |[[File:Pus.png|center|250px]] | ||
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|[[Acrodermatitis enteropathica]] | |[[Acrodermatitis enteropathica]]<ref name="pmid6696358">{{cite journal| author=Prasad AS, Cossack ZT| title=Zinc supplementation and growth in sickle cell disease. | journal=Ann Intern Med | year= 1984 | volume= 100 | issue= 3 | pages= 367-71 | pmid=6696358 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=6696358 }}</ref><ref name="pmid1940572">{{cite journal| author=Meftah S, Prasad AS, Lee DY, Brewer GJ| title=Ecto 5' nucleotidase (5'NT) as a sensitive indicator of human zinc deficiency. | journal=J Lab Clin Med | year= 1991 | volume= 118 | issue= 4 | pages= 309-16 | pmid=1940572 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1940572 }}</ref><ref name="pmid9481631">{{cite journal| author=Kiliç I, Ozalp I, Coŝkun T, Tokatli A, Emre S, Saldamli I et al.| title=The effect of zinc-supplemented bread consumption on school children with asymptomatic zinc deficiency. | journal=J Pediatr Gastroenterol Nutr | year= 1998 | volume= 26 | issue= 2 | pages= 167-71 | pmid=9481631 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9481631 }}</ref> | ||
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* An [[autosomal]] [[recessive]] disorder characterized by periorificial and acral [[dermatitis]], [[alopecia]], and [[diarrhea]]. | * An [[autosomal]] [[recessive]] disorder characterized by periorificial and acral [[dermatitis]], [[alopecia]], and [[diarrhea]]. | ||
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* A low plasma [[zinc]] usually is defined as a value less than 60 mcg/dL. | * 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. | * 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]]. | * 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. | * Depressed serum [[alkaline phosphatase]] levels for age provide supportive evidence for [[zinc]] deficiency. | ||
|[[File:Acrodermatitis enteropathica 05.png|center|250px]] | |[[File:Acrodermatitis enteropathica 05.png|center|250px]] | ||
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|[[Pellagra]] | |[[Pellagra]]<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><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> | ||
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* It is a niacin deficiency disease characterized by a [[Photosensitivity|photosensitive]] pigmented [[dermatitis]], [[diarrhea]], and [[dementia]]. | * It is a niacin deficiency disease characterized by a [[Photosensitivity|photosensitive]] pigmented [[dermatitis]], [[diarrhea]], and [[dementia]]. | ||
* Hisotry of [[Alcoholic|alcoholics]], [[bariatric surgery]], [[anorexia nervosa]], or [[Malabsorption|malabsorptive disease]]. | * Hisotry of [[Alcoholic|alcoholics]], [[bariatric surgery]], [[anorexia nervosa]], or [[Malabsorption|malabsorptive disease]]. | ||
* Dietary deficiency especially in infants | * Dietary deficiency especially in infants | ||
* Past history of [[Carcinoid syndrome]] | * Past history of [[Carcinoid syndrome]] | ||
* Prolonged use of [[isoniazide]] | * Prolonged use of [[isoniazide]] | ||
* A family history of [[Hartnup disease]] | * A family history of [[Hartnup disease]] | ||
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Revision as of 15:34, 9 October 2017
Glucagonoma Microchapters |
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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] Mohammed Abdelwahed M.D[3]
Overview
Glucagonoma must be differentiated from certain skin lesions in which necrolytic migratory erythema can be found such as acrodermatitis enteropathica, psoriasis, pellagra, and eczema. Glucagonoma should be differentiated from other causes of hyperglycemia include 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 such as:[1]
Disease | Clinical Picture | Investigations | Pictures | ||
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History | Symptoms | Signs | |||
Glucagonoma[4][2][3][1][3][4][4] | A family history of multiple endocrine neoplasia type 1 |
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Pemphigus foliaceus.[2][2][3]
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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 |
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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[2][3] |
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Acrodermatitis enteropathica[5][6][7] |
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Pellagra[8][9] |
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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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Differentiating glucagonoma from other causes of hyperglycemia:
Glucagonoma can be differentiated from other causes of hyperglycemia which include: [10][11][12]
- Type 2 DM
- MODY-DM
- Psychogenic polydipsia
- Diabetes insipidus
- Transient hyperglycemia
- Steroid therapy
- Renal tubular acidosis type-1
- Glucagonoma
- Cushing's syndrome
- Hypothyroidism
- Wolfram syndrome
- Alstrom syndrome
Disease | History and symptoms | Laboratory findings | Additional findings | |||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Polyuria | Polydipsia | Polyphagia | Weight loss | Weight gain | Serum glucose | Urinary Glucose | Urine PH | Serum Sodium | Urinary Glucose | 24 hrs cortisol level | C-peptide level | Serum glucagon | ||
Type 1 Diabetes mellitus | ✔ | ✔ | ✔ | ✔ | - | ↑ | ↑ | Normal | Normal | N/↑ | Normal | ↓ | Normal | Auto antibodies present (Anti GAD-65 and anti insulin anti bodies) |
Type 2 Diabetes mellitus | ✔ | ✔ | ✔ | ✔ | - | ↑ | ↑ | Normal | Normal | ↑ | Normal | Normal | ↑ | Acanthosis nigricans |
MODY | ✔ | ✔ | ✔ | - | ✔ | ↑ | ↑ | Normal | Normal | ↑ | Normal | Normal | N | - |
Psychogenic polydipsia | ✔ | ✔ | - | - | - | Normal | Normal | Normal | ↓ | Normal | Normal | Normal | Normal | - |
Diabetes insipidus | ✔ | ✔ | - | - | - | Normal | Normal | Normal | ↑ | Normal | Normal | Normal | Normal | - |
Transient hyperglycemia | - | - | - | - | - | ↑ | ↑ | Normal | Normal | ↑ | Normal | Normal | N/↑ | In hospitalized patients especially in ICU and CCU |
Steroid therapy | ✔ | - | - | - | ✔ | ↑ | ↑ | Normal | Normal | ↑ | ↑ | N/↑ | N/↑ | Acanthosis nigricans, |
RTA 1 | - | - | - | ✔ | - | Normal | Normal | ↑ | Normal | ↑ | Normal | Normal | Normal | Hypokalemia, nephrolithiasis |
Glucagonoma | - | - | - | - | - | ↑ | Normal | Normal | Normal | - | Normal | Normal | ↑ | Necrolytic migratory erythema |
Cushing syndrome | - | - | - | - | ✔ | ↑ | - | Normal | ↓ | N/↑ | ↑ | Normal | Normal | Moon face, obesity, buffalo hump, easy bruisibility |
References
- ↑ 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.
- ↑ Barrett TG (2007). "Differential diagnosis of type 1 diabetes: which genetic syndromes need to be considered?". Pediatr Diabetes. 8 Suppl 6: 15–23. doi:10.1111/j.1399-5448.2007.00278.x. PMID 17727381.
- ↑ Type 1 Diabetes mellitus "Dennis Kasper, Anthony Fauci, Stephen Hauser, Dan Longo, J. Larry Jameson, Joseph Loscalzo"Harrison's Principles of Internal Medicine, 19e Accessed on December 27th,2016
- ↑ "namrata".