Clinical Featurs of Acquired Partial Lipodystrophy (APL, Barraquer-Simons Syndrome)

 

Synonym: Barraquer-Simons Syndrome

 

Clinical features of APL:

(1) The onset is during childhood, adolescence or adulthood.

(2) There is a progressive loss of subcutaneous fat in a cephalocaudal fashion affecting the face, neck, arms, thorax and upper abdomen.

(3) There is sparing of the lower extremities, buttocks and intra-abdominal fat.

(4) Variable renal disease including membranoproliferative glomerulonephritis.

 

Laboratory features:

(1) low serum complement 3 (C3)

(2) C3 nephritic factor

 

Risk factors:

(1) autoimmune disease (dermatomyositis, systemic lupus erythematosus)

(2) female gender