PPARG-related familial partial lipodystrophy

Orpha code: 79083OMIM code: 604367

Definition

A rare familial partial lipodystrophy characterized by adult onset of distal lipoatrophy with gluteofemoral fat loss, as well as increased fat accumulation in the face and trunk and visceral adiposity. Additional manifestations include diabetes mellitus, atherogenic dyslipidemia, eyelid xanthelasmas, arterial hypertension, cardiovascular disease, hepatic steatosis, acanthosis nigricans on axillae and neck, hirsutism, and muscular hypertrophy of the lower limbs.

Disease data
Classification

Disease

Synonyms
FPLD3
FplD zależna od PPARG
FplD3
Rodzinna częściowa lipodystrofia typu 3
Familial partial lipodystrophy type 3
PPARG-related FPLD
ORPHA code
79083
OMIM code
604367
ICD10 code
E88.1
ICD11 code
-

No additional description.

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