Progressive lipodystrophy

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Synonym(s)

Progressive partial lipodystrophy; Barra-quer-Simons syndrome; acquired partial lipodystrophy; cephalothoracic dystrophy; acquired progressive lipodystrophy

Definition

Disorder characterized by progressive and symmetric loss of subcutaneous fat

Pathogenesis

May be associated with glomerulonephritis, low C3 levels, and the presence of a C3 nephritic factor

Clinical manifestation

Onset between 5 and 15 years of age; slow, insidious loss of subcutaneous fat, initially limited to the face, sometimes extending to the upper portion of the body, giving the patient a cachetic appearance

Differential diagnosis

HIV-associated lipodystrophy; Cockayne syndrome; generalized lipodystrophies such as Berardinelli-Seip syndrome; Werner syndrome; hypothalamus tumor

Therapy

Subcutaneous fat injections from unaffected areas; temporal muscle flaps; silicone filling material