acquired partial lipodystrophy

(redirected from Barraquer-Simons Syndrome)
Also found in: Wikipedia.

acquired partial lipodystrophy

A rare, acquired, partial lipodystrophy of unknown aetiology and onset during childhood, eventually leading to complete loss of subcutaneous fat in the face, neck, trunk and upper limbs. It is more common in females, and may be associated with glomerulonephritis, diabetes, hyperlipidaemia and complement deficiency.

Management
Free-fat transplantation.
References in periodicals archive ?
The Barraquer-Simons syndrome, APL or cephalothoracic lipodystrophy, was described by the Spanish physician Barraquer in 1906 and the German physician Simons in 1911 [1].
On release, the patient was diagnosed with the following: chronic bronchitis, situs inversus totalis, polinodular goiter, Barraquer-Simons syndrome, insulin-dependent type 2 non-obese diabetes complicated with polyneuropathie, hypertriglyceridemia, liver steatosis, essential hypertension stage 3 well drug controlled, obstructive hypertrophic cardiomyopathy, ischemic heart disease, heart failure class III NYHA, rheumatoid arthritis stage II, polymyositis, osteoporosis.
Differential diagnosis in the infant include: short syndrome, neonatal progeroid syndrome, neurometabolic lysosomal storage disorder (Gaucher type 2, Krabbe disease), Russell diencephalic syndrome; in older children, Dunningan lipodistrophy, Rabson-Mendenhall syndrome, insulin-dependent diabetes mellitus; and in adults, Barraquer-Simons syndrome, AIDS, partial lipodistrophy and Lawrence syndrome (9).