Barraquer-Simons Syndrome

Acquired partial lipodystrophy

Overview

Type of disease: Rare conditions

Barraquer-Simons syndrome, or acquired partial lipodystrophy, is characterized by the loss of fat from the face, neck, shoulders, arms, forearms, chest and abdomen. Occasionally the groin or thighs are also affected. Onset usually begins in childhood following a viral illness. It affects females more often than males. The fat loss usually has a 18 month course, but can come and go over the course of several years.   Following puberty, affected women may experience a disproportionate accumulation of fat in the hips and lower limbs.  Around 1 in 5 people with this syndrome develop membranoproliferative glomerulonephritis. This kidney condition usually develops more than 10 years after the lipodystrophy’s onset. Autoimmune disorders may also occur in association with this syndrome. Source: Genetic and Rare Diseases Information Center (GARD), supported by ORDR-NCATS and NHGRI.

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