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Familial partial lipodystrophy, Köbberling type

Disease definition

Familial partial lipodystrophy, Köbberling type, is a very rare form of familial partial lipodystrophy (FPLD; see this term) of unknown etiology characterized by lipoatrophy that is confined to the limbs and a normal or increased fat distribution of the face, neck, and trunk. Arterial hypertension and diabetes have also been associated. Inheritance is thought to be autosomal dominant.


Classification level: Disorder
  • Synonym(s):
    • FPLD1
    • Familial partial lipodystrophy type 1
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal dominant 
  • Age of onset: Childhood
  • ICD-10: E88.1
  • OMIM: 608600
  • UMLS: C1720859
  • MeSH: -
  • GARD: 12598
  • MedDRA: -

Detailed information


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