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Idiopathic localized lipodystrophy

MedGen UID:
633314
•Concept ID:
C0473566
•
Acquired Abnormality
Synonyms: idiopathic localized lipodystrophy; Localized idiopathic lipoatrophy
SNOMED CT: Localized idiopathic lipoatrophy (238897006)
 
Monarch Initiative: MONDO:0019554
Orphanet: ORPHA90158

Definition

Idiopathic localized lipodystrophy is a rare, acquired, localized lipodystrophy characterized by asymptomatic, well-demarcated, depressed, lipoatrophic lesions of variable size, with normal overlying skin without antecedent inflammation or a known identifiable cause (autoimmune disease, drug injection, injury, etc). [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIdiopathic localized lipodystrophy

Recent clinical studies

Etiology

Subcutaneous tissue involvement in idiopathic inflammatory myopathies: Systematic literature review including three new cases and hypothetical mechanisms.
Suzon B, Goulabchand R, Louis-Sidney F, Maria A, Najjari R, Chauvet E, Le Quellec A, Bessis D, Guilpain P
Autoimmun Rev 2023 Apr;22(4):103284. Epub 2023 Feb 1 doi: 10.1016/j.autrev.2023.103284. PMID: 36736986
Localized abdominal idiopathic lipodystrophy.
Serrão VV, Feio AB
Dermatol Online J 2008 Jul 15;14(7):15. PMID: 18718199
Localized involutional lipoatrophy: a clinicopathologic study of 16 patients.
Dahl PR, Zalla MJ, Winkelmann RK
J Am Acad Dermatol 1996 Oct;35(4):523-8. doi: 10.1016/s0190-9622(96)90673-8. PMID: 8859277

Diagnosis

Subcutaneous tissue involvement in idiopathic inflammatory myopathies: Systematic literature review including three new cases and hypothetical mechanisms.
Suzon B, Goulabchand R, Louis-Sidney F, Maria A, Najjari R, Chauvet E, Le Quellec A, Bessis D, Guilpain P
Autoimmun Rev 2023 Apr;22(4):103284. Epub 2023 Feb 1 doi: 10.1016/j.autrev.2023.103284. PMID: 36736986
Bilateral idiopathic localised involutional lipoatrophy in a child.
Tersigni C, Caporalini C, Buccoliero AM, Coronella G, de Martino M, Bassi A
Arch Dis Child 2019 Aug;104(8):774. Epub 2018 May 4 doi: 10.1136/archdischild-2018-315084. PMID: 29728417
Cosegregation of focal segmental glomerulosclerosis in a family with familial partial lipodystrophy due to a mutation in LMNA.
Thong KM, Xu Y, Cook J, Takou A, Wagner B, Kawar B, Ong AC
Nephron Clin Pract 2013;124(1-2):31-7. Epub 2013 Sep 26 doi: 10.1159/000354716. PMID: 24080738
Localized abdominal idiopathic lipodystrophy.
Serrão VV, Feio AB
Dermatol Online J 2008 Jul 15;14(7):15. PMID: 18718199
Localized involutional lipoatrophy with epidermal and dermal changes.
Abbas O, Salman S, Kibbi AG, Chedraoui A, Ghosn S
J Am Acad Dermatol 2008 Mar;58(3):490-3. doi: 10.1016/j.jaad.2007.06.030. PMID: 18280348

Therapy

Localized involutional lipoatrophy: a clinicopathologic study of 16 patients.
Dahl PR, Zalla MJ, Winkelmann RK
J Am Acad Dermatol 1996 Oct;35(4):523-8. doi: 10.1016/s0190-9622(96)90673-8. PMID: 8859277

Prognosis

Subcutaneous tissue involvement in idiopathic inflammatory myopathies: Systematic literature review including three new cases and hypothetical mechanisms.
Suzon B, Goulabchand R, Louis-Sidney F, Maria A, Najjari R, Chauvet E, Le Quellec A, Bessis D, Guilpain P
Autoimmun Rev 2023 Apr;22(4):103284. Epub 2023 Feb 1 doi: 10.1016/j.autrev.2023.103284. PMID: 36736986
Localized abdominal idiopathic lipodystrophy.
Serrão VV, Feio AB
Dermatol Online J 2008 Jul 15;14(7):15. PMID: 18718199

Clinical prediction guides

Subcutaneous tissue involvement in idiopathic inflammatory myopathies: Systematic literature review including three new cases and hypothetical mechanisms.
Suzon B, Goulabchand R, Louis-Sidney F, Maria A, Najjari R, Chauvet E, Le Quellec A, Bessis D, Guilpain P
Autoimmun Rev 2023 Apr;22(4):103284. Epub 2023 Feb 1 doi: 10.1016/j.autrev.2023.103284. PMID: 36736986
Localized involutional lipoatrophy: a clinicopathologic study of 16 patients.
Dahl PR, Zalla MJ, Winkelmann RK
J Am Acad Dermatol 1996 Oct;35(4):523-8. doi: 10.1016/s0190-9622(96)90673-8. PMID: 8859277

Recent systematic reviews

Subcutaneous tissue involvement in idiopathic inflammatory myopathies: Systematic literature review including three new cases and hypothetical mechanisms.
Suzon B, Goulabchand R, Louis-Sidney F, Maria A, Najjari R, Chauvet E, Le Quellec A, Bessis D, Guilpain P
Autoimmun Rev 2023 Apr;22(4):103284. Epub 2023 Feb 1 doi: 10.1016/j.autrev.2023.103284. PMID: 36736986

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