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Acquired generalized lipodystrophy

MedGen UID:: 543499
•Concept ID:: C0271693
•: Disease or Syndrome

Synonyms:	Acquired Generalized Lipodystrophy; acquired generalized lipodystrophy; Acquired lipoatrophic diabetes; acquired lipoatrophic diabetes; Acquired lipodystrophic diabetes; Acquired total lipoatrophy; Lawrence syndrome; Lawrence-Seip syndrome
SNOMED CT:	Acquired total lipoatrophy (86907008); Acquired generalized lipodystrophy (86907008); Acquired lipodystrophic diabetes (86907008)
Modes of inheritance:	Not genetically inherited MedGen UID: 988794 •Concept ID: CN307044 • Finding Source: Orphanet clinical entity without genetic inheritance. See: This record Not genetically inherited (Orphanet)

Monarch Initiative:	MONDO:0019193
Orphanet:	ORPHA79086

Definition

A rare lipodystrophic syndrome characterized by loss of adipose tissue, and is a syndrome of insulin resistance that leads to increased cardiovascular risk. Acquired generalized lipodystrophy is related to a selective loss of subcutaneous adipose tissue occurring exclusively at the extremities (face, legs, arms, palms and sometimes soles). [from ORDO]

Term Hierarchy

GTR
MeSH
Orphanet

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVAcquired generalized lipodystrophy

Acquired lipodystrophy
- Acquired generalized lipodystrophy

Follow this link to review classifications for Acquired generalized lipodystrophy in Orphanet.

Professional guidelines

PubMed

Diagnosis and treatment of lipodystrophy: a step-by-step approach.

Araújo-Vilar D, Santini F
J Endocrinol Invest 2019 Jan;42(1):61-73. Epub 2018 Apr 27 doi: 10.1007/s40618-018-0887-z. PMID: 29704234 Free PMC Article

Long-term effectiveness and safety of metreleptin in the treatment of patients with generalized lipodystrophy.

Brown RJ, Oral EA, Cochran E, Araújo-Vilar D, Savage DB, Long A, Fine G, Salinardi T, Gorden P
Endocrine 2018 Jun;60(3):479-489. Epub 2018 Apr 12 doi: 10.1007/s12020-018-1589-1. PMID: 29644599 Free PMC Article

Clinical Features and Management of Non-HIV-Related Lipodystrophy in Children: A Systematic Review.

Gupta N, Asi N, Farah W, Almasri J, Barrionuevo P, Alsawas M, Wang Z, Haymond MW, Brown RJ, Murad MH
J Clin Endocrinol Metab 2017 Feb 1;102(2):363-374. doi: 10.1210/jc.2016-2271. PMID: 27967300 Free PMC Article

See all (3)

Recent clinical studies

Etiology

A Concerted Vision to Advance the Knowledge of Diabetes Mellitus Related to Immune Checkpoint Inhibitors.

Deligiorgi MV, Trafalis DT
Int J Mol Sci 2023 Apr 21;24(8) doi: 10.3390/ijms24087630. PMID: 37108792 Free PMC Article

Lipodystrophy for the Diabetologist-What to Look For.

Patni N, Garg A
Curr Diab Rep 2022 Sep;22(9):461-470. Epub 2022 Jul 11 doi: 10.1007/s11892-022-01485-w. PMID: 35821558 Free PMC Article

Generalized lipoatrophy syndromes.

Sorkina E, Chichkova V
Presse Med 2021 Nov;50(3):104075. Epub 2021 Sep 22 doi: 10.1016/j.lpm.2021.104075. PMID: 34562560

Acquired generalized lipodystrophy under immune checkpoint inhibition.

Haddad N, Vidal-Trecan T, Baroudjian B, Zagdanski AM, Arangalage D, Battistella M, Gautier JF, Lebbe C, Delyon J; PATIO group
Br J Dermatol 2020 Feb;182(2):477-480. Epub 2019 Aug 9 doi: 10.1111/bjd.18124. PMID: 31077337

Lymphoma in acquired generalized lipodystrophy.

Brown RJ, Chan JL, Jaffe ES, Cochran E, DePaoli AM, Gautier JF, Goujard C, Vigouroux C, Gorden P
Leuk Lymphoma 2016;57(1):45-50. Epub 2015 May 12 doi: 10.3109/10428194.2015.1040015. PMID: 25864863 Free PMC Article

See all (18)

Diagnosis

Lipodystrophy for the Diabetologist-What to Look For.

Patni N, Garg A
Curr Diab Rep 2022 Sep;22(9):461-470. Epub 2022 Jul 11 doi: 10.1007/s11892-022-01485-w. PMID: 35821558 Free PMC Article

Generalized lipoatrophy syndromes.

Sorkina E, Chichkova V
Presse Med 2021 Nov;50(3):104075. Epub 2021 Sep 22 doi: 10.1016/j.lpm.2021.104075. PMID: 34562560

Diagnosis and treatment of lipodystrophy: a step-by-step approach.

Araújo-Vilar D, Santini F
J Endocrinol Invest 2019 Jan;42(1):61-73. Epub 2018 Apr 27 doi: 10.1007/s40618-018-0887-z. PMID: 29704234 Free PMC Article

Acquired Generalized Lipodystrophy Following Immune Thrombocytopenia.

Cunningham J, Nadal R, Broome C
Am J Med 2017 Oct;130(10):e445-e446. Epub 2017 May 24 doi: 10.1016/j.amjmed.2017.04.035. PMID: 28549922

Lipodystrophies.

Garg A
Am J Med 2000 Feb;108(2):143-52. doi: 10.1016/s0002-9343(99)00414-3. PMID: 11126308

See all (28)

Therapy

A Concerted Vision to Advance the Knowledge of Diabetes Mellitus Related to Immune Checkpoint Inhibitors.

Deligiorgi MV, Trafalis DT
Int J Mol Sci 2023 Apr 21;24(8) doi: 10.3390/ijms24087630. PMID: 37108792 Free PMC Article

Lipodystrophy for the Diabetologist-What to Look For.

Patni N, Garg A
Curr Diab Rep 2022 Sep;22(9):461-470. Epub 2022 Jul 11 doi: 10.1007/s11892-022-01485-w. PMID: 35821558 Free PMC Article

Leptin induces TNFα-dependent inflammation in acquired generalized lipodystrophy and combined Crohn's disease.

Ziegler JF, Böttcher C, Letizia M, Yerinde C, Wu H, Freise I, Rodriguez-Sillke Y, Stoyanova AK, Kreis ME, Asbach P, Kunkel D, Priller J, Anagnostopoulos I, Kühl AA, Miehle K, Stumvoll M, Tran F, Fredrich B, Forster M, Franke A, Bojarski C, Glauben R, Löscher BS, Siegmund B, Weidinger C
Nat Commun 2019 Dec 10;10(1):5629. doi: 10.1038/s41467-019-13559-7. PMID: 31822667 Free PMC Article

Diagnosis and treatment of lipodystrophy: a step-by-step approach.

Araújo-Vilar D, Santini F
J Endocrinol Invest 2019 Jan;42(1):61-73. Epub 2018 Apr 27 doi: 10.1007/s40618-018-0887-z. PMID: 29704234 Free PMC Article

Dysregulation of growth hormone in acquired generalized lipodystrophy.

Sahhar NN, Ahmad AT, Ammari FL, Ajlouni KM
Saudi Med J 2004 Nov;25(11):1766-7. PMID: 15573230

See all (22)

Prognosis

Lymphoma in acquired generalized lipodystrophy.

A Report of Three Cases With Acquired Generalized Lipodystrophy With Distinct Autoimmune Conditions Treated With Metreleptin.

Lebastchi J, Ajluni N, Neidert A, Oral EA
J Clin Endocrinol Metab 2015 Nov;100(11):3967-70. Epub 2015 Sep 21 doi: 10.1210/jc.2015-2589. PMID: 26390101 Free PMC Article

A Novel Syndrome of Generalized Lipodystrophy Associated With Pilocytic Astrocytoma.

Patni N, Alves C, von Schnurbein J, Wabitsch M, Tannin G, Rakheja D, Garg A
J Clin Endocrinol Metab 2015 Oct;100(10):3603-6. Epub 2015 Aug 7 doi: 10.1210/jc.2015-2476. PMID: 26252356 Free PMC Article

Type 1 diabetes associated with acquired generalized lipodystrophy and insulin resistance: the effect of long-term leptin therapy.

Park JY, Chong AY, Cochran EK, Kleiner DE, Haller MJ, Schatz DA, Gorden P
J Clin Endocrinol Metab 2008 Jan;93(1):26-31. Epub 2007 Oct 16 doi: 10.1210/jc.2007-1856. PMID: 17940115 Free PMC Article

Generalized lipodystrophy, congenital and acquired (lipoatrophy).

Seip M, Trygstad O
Acta Paediatr Suppl 1996 Jun;413:2-28. doi: 10.1111/j.1651-2227.1996.tb14262.x. PMID: 8783769

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Clinical prediction guides

Autoantibodies Against Perilipin 1 as a Cause of Acquired Generalized Lipodystrophy.

Corvillo F, Aparicio V, López-Lera A, Garrido S, Araújo-Vilar D, de Miguel MP, López-Trascasa M
Front Immunol 2018;9:2142. Epub 2018 Sep 19 doi: 10.3389/fimmu.2018.02142. PMID: 30283460 Free PMC Article

Clinical Features and Management of Non-HIV-Related Lipodystrophy in Children: A Systematic Review.

Bone imaging findings in genetic and acquired lipodystrophic syndromes: an imaging study of 24 cases.

Teboul-Coré S, Rey-Jouvin C, Miquel A, Vatier C, Capeau J, Robert JJ, Pham T, Lascols O, Berenbaum F, Laredo JD, Vigouroux C, Sellam J
Skeletal Radiol 2016 Nov;45(11):1495-506. Epub 2016 Sep 8 doi: 10.1007/s00256-016-2457-9. PMID: 27631079

Leptin in congenital and HIV-associated lipodystrophy.

Tsoukas MA, Farr OM, Mantzoros CS
Metabolism 2015 Jan;64(1):47-59. Epub 2014 Aug 12 doi: 10.1016/j.metabol.2014.07.017. PMID: 25267014

Generalized lipodystrophy, congenital and acquired (lipoatrophy).

Seip M, Trygstad O
Acta Paediatr Suppl 1996 Jun;413:2-28. doi: 10.1111/j.1651-2227.1996.tb14262.x. PMID: 8783769

See all (14)