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Acquired generalized lipodystrophy information


Acquired generalized lipodystrophy
Other namesLawrence-Seip syndrome

Acquired generalized lipodystrophy (AGL), also known as Lawrence syndrome[1] and Lawrence–Seip syndrome,[1] is a rare skin condition that appears during childhood or adolescence, characterized by fat loss affecting large areas of the body, particularly the face, arms, and legs.[2]: 496  There are four types of lipodystrophy based on its onset and areas affected: acquired or inherited (congenital or familial), and generalized or partial. Both acquired or inherited lipodystrophy present as loss of adipose tissues, in the absence of nutritional deprivation. The near-total loss of subcutaneous adipose tissue is termed generalized lipodystrophy while the selective loss of adipose tissues is denoted as partial lipodystrophy.[3] Thus, as the name suggests, AGL is a near-total deficiency of adipose tissues in the body that is developed later in life. It is an extremely rare disease with only about 100 cases reported worldwide.[4][5] There are three main etiologies of AGL suspected: autoimmune, panniculitis-associated, or idiopathic.[6] After its onset, the disease progresses over a few days, weeks, months, or even in years.[6] Clinical presentations of AGL are similar to other lipodystrophies, including metabolic complications and hypoleptinemia.[6][5] Treatments are also similar and mainly supportive for symptomatic alleviation. Although HIV- or drug-induced lipodystrophy are types of acquired lipodystrophy, their origins are very specific and distinct and hence are usually not discussed with AGL (see HIV-associated lipodystrophy).

  1. ^ a b Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L., eds. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 978-1-4160-2999-1. [page needed]
  2. ^ James, William D.; Berger, Timothy G.; Elston, Dirk M., eds. (2006). Andrews' Diseases of the Skin: Clinical Dermatology (Ninth ed.). Saunders Elsevier. ISBN 978-0-7216-2921-6.
  3. ^ Brown, Rebecca J.; Chan, Jean L.; Jaffe, Elaine S.; Cochran, Elaine; DePaoli, Alex M.; Gautier, Jean-Francois; Goujard, Cecile; Vigouroux, Corinne; Gorden, Phillip (2016-01-02). "Lymphoma in acquired generalized lipodystrophy". Leukemia & Lymphoma. 57 (1): 45–50. doi:10.3109/10428194.2015.1040015. PMC 4755279. PMID 25864863.
  4. ^ Garg, Abhimanyu (2004-03-18). "Acquired and Inherited Lipodystrophies". New England Journal of Medicine. 350 (12): 1220–1234. doi:10.1056/nejmra025261. PMID 15028826.
  5. ^ a b Vantyghem, Pr Marie-Christine, ed. (January 2009). "Orphanet: Acquired generalized lipodystrophy". www.orpha.net. Retrieved 2017-11-07.
  6. ^ a b c Hussain, Iram; Garg, Abhimanyu (2016). "Lipodystrophy Syndromes". Endocrinology and Metabolism Clinics of North America. 45 (4): 783–797. doi:10.1016/j.ecl.2016.06.012. PMC 4947059. PMID 27823605.

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