Barraquer-Simons syndrome

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Disease Overview

Barraquer-Simons syndrome, or acquired partial lipodystrophy, is characterized by the loss of fat from the face, neck, shoulders, arms, forearms, chest and abdomen.[4008] Occasionally the groin or thighs are also affected. Onset usually begins in childhood following a viral illness.[4008] 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.[4008]  Following puberty, affected women may experience a disproportionate accumulation of fat in the hips and lower limbs.[4008] 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.[4008]


Synonyms

  • APL
  • Lipodystrophy partial acquired
  • Lipodystrophy cephalothoracic type
  • Lipodystophy partial progressive

For more information, visit GARD.

National Organization for Rare Disorders