acquired partial lipodystrophy

(redirected from Barraquer-Simons Syndrome)
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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 differential diagnosis of our case of progressive hemifacial atrophy includes other forms of juvenile localized scleroderma, Barraquer-Simons syndrome, congenital hemiatrophy, and primary hemifacial hypertrophy.
The product is a recombinant analogue of human leptin and is claimed to be the first and only licensed treatment in Europe indicated as an adjunct to diet as a replacement therapy to treat the complications of leptin deficiency in (LD) patients with confirmed congenital generalised LD (Berardinelli-Seip syndrome) or acquired generalised LD (Lawrence syndrome) in adults and children 2 years of age and above; or with confirmed familial partial LD or acquired partial LD (Barraquer-Simons syndrome), in adults and children 12 years of age and above for whom standard treatments have failed to achieve adequate metabolic control.
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].
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).