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] 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
History Symptoms Signs
Glucagonoma[2][3][4][5] A family history of multiple endocrine neoplasia type 1
  • Serum glucagon
    • Increased plasma glucagon levels (>500 pg/mL)
    • Concentrations above 1000 pg/mL are diagnostic of glucagonoma
  • CT scan is used to determine:
    • The location of the tumor
    • Metastasis (usually liver metastasis)
      • Appear isodense with the liver on a non-contrasted study
Pemphigus foliaceus.[6][2][7][8] 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

  • Cutaneous lesion that usually develops in a seborrheic distribution
  • The scalp, face, and trunk are common sites of involvement
  • 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
  • The skin lesions usually consist of small, scattered superficial blisters
    • Lesions rapidly evolve into scaly, crusted erosions
    • Positive Nikolsky sign


Autoimmune IgG build up in the epidermis, then nearly almost all of the antibodies are aimed against desmoglein 1
Courtesy:http://www.atlasdermatologico.com.br/index.jsf
Pustular psoriasis[2][3]
Courtesy:http://www.atlasdermatologico.com.br/index.jsf
Acrodermatitis enteropathica[9][10][11]
  • Symptoms appear in infants after breast milk weaning
  • The appearance of erythematous patches and plaques of dry, scaly skin
  • Diarrhea
Courtesy:http://www.atlasdermatologico.com.br/index.jsf
Pellagra[12][13] Symmetric hyperpigmented 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 
Courtesy:http://www.atlasdermatologico.com.br/index.jsf
Chronic eczema (atopic dermatitis)
  • 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 hyperactivity to various environmental stimuli
  • Exposure to:
Courtesy:http://www.atlasdermatologico.com.br/index.jsf

Differentiating glucagonoma from other causes of hyperglycemia:

Glucagonoma can be differentiated from other causes of hyperglycemia which include:[14][15][16]

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

  1. 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.
  2. Wilkinson DS (1973). "Necrolytic migratory erythema with carcinoma of the pancreas". Trans St Johns Hosp Dermatol Soc. 59 (2): 244–50. PMID 4793623.
  3. Wermers RA, Fatourechi V, Wynne AG, Kvols LK, Lloyd RV (1996). "The glucagonoma syndrome. Clinical and pathologic features in 21 patients". Medicine (Baltimore). 75 (2): 53–63. PMID 8606627.
  4. Zhang M, Xu X, Shen Y, Hu ZH, Wu LM, Zheng SS (2004). "Clinical experience in diagnosis and treatment of glucagonoma syndrome". HBPD INT. 3 (3): 473–5. PMID 15313692.
  5. Kindmark H, Sundin A, Granberg D, Dunder K, Skogseid B, Janson ET, Welin S, Oberg K, Eriksson B (2007). "Endocrine pancreatic tumors with glucagon hypersecretion: a retrospective study of 23 cases during 20 years". Med. Oncol. 24 (3): 330–7. PMID 17873310.
  6. Bystryn JC, Rudolph JL (2005). "Pemphigus". Lancet. 366 (9479): 61–73. doi:10.1016/S0140-6736(05)66829-8. PMID 15993235.
  7. 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.
  8. 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.
  9. Prasad AS, Cossack ZT (1984). "Zinc supplementation and growth in sickle cell disease". Ann Intern Med. 100 (3): 367–71. PMID 6696358.
  10. 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.
  11. 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.
  12. 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.
  13. 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.
  14. 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.
  15. 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
  16. "namrata".

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