Glucagonoma syndrome
Keywords:
Glucagonoma syndrome, diabetes mellitus, necrolytic migratory erythemaAbstract
Glucagonoma syndrome is a rare disease in which necrolytic migratory erythema is often one of the early symptoms. Weight loss and diabetes mellitus are two other characteristics of this syndrome. Necrolytic migratory erythema is typically characterized on skin biopsies by necrolysis of upper epidermis with vacuolated keratinocytes. Persistent hyperglucagonemia results in excessive stimulation of basic metabolic pathway which in turn results in diabetes mellitus at the expense of tissue glycogen stores. Deficiencies of various essential nutrients and vitamin B lead to dermatosis. Due to rarity of glucagonoma, it is quite difficult to diagnose this syndrome at early stages.References
Irwin M. Freedberg et al. Skin manifestations of alterations and disorders of the endocrine system, dermatology in general medicine, 2003, 1659-1660.
Becker SW, Kahn, Rothman S. Cutaneous manifestations of internal malignant tumours. Arch Derm Syphilol 1942; 45; 1069-80.
McGavran MH, Unger RH, Recant L et al. A glucagon-secreting alpha-cell carcinoma of the pancreas. N Engl J Med 1966; 274: 1408-13.
Soga J, Yakuwa Y. Glucagonomas / diabetic-dermatogenic syndrome (DDS). J Hepatobiliary Pancreat Surg 1998; 5: 312-9.
Santacrose L. Diomede L. Abraham MR. A review article on glucagonoma [online] last updated Nov 20, 2008. Available from:URL: http://emedicine.medscape.com/article/118899-overview.
Aswani VL, Malkani RH. Case report of glucagonoma syndrome. Indian J Dermatol Venereol Leprol 1996; 62: 246-9.
Remes-Troche JM, García-de-Acevedo B, Zuñiga-Varga J et al. Necrolytic migratory erythema: a cutaneous clue to glucagonoma syndrome. J Eur Acad Dermatol Venereol 2004; 18: 591-5.
Kumar PJ. Clark ML. Pocket Essentials of Clinical Medicine, 4 edn. London: WB Saunders; 2008.
Graham RM. Cox NH: Systemic disease and the skin. Rook’s Text Book of Dermatology, 7th edn. Oxford: Blackwell Science: 2004. p. 59-46.
Wermers RA, Fatourechi V, Wynne AG et al. The glucagonoma syndrome: clinical and pathological features in 21 patients. Medicine 1996; 75: 53-63.
Davidson S, Haslett C, Chilvers ER, eds. Davidson’ Principles and Practice of Medicine, 20 edn. London: Elsevier Science; 2002.
Prinz RA, Dorsch TR, Lawrence AM. Clinical aspects of glucagon-producing Islet cell tumors. Am J Gastroenterol 1981; 76: 125-31.
Chastain MA. The gulcagonoma syndrome: a review of its features and discussion of new perspectives. Am J Med Sci 2001; 321: 306-20.
Akerstrom G, Hellman P, Hessman O et al. Surgical treatment of endocrine pancreatic tumours. Neuroendocrinology 2004; 80 (Suppl 1): 62-6.
Meier RF, Reichert MM. Octreotide: a clinical update. Saudi J Gastroenterol 1998: 4: 147-55.
Ravi Kumar BC, Balachandran C, Sabitha. Glucagonoma syndrome. Indian J Dermatol Venereol Leprol 1999; 65: 227-9.
