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Diseases

Genetic and Rare Diseases Information Center (GARD)

Barraquer-Simons syndrome


Other Names for this Disease
  • APL
  • Lipodystophy partial progressive
  • Lipodystrophy cephalothoracic type
  • Lipodystrophy partial acquired
  • Partial acquired lipodystrophy
Related Diseases
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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.[1] Occasionally the groin or thighs are also affected. Onset usually begins in childhood following a viral illness.[1] 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.[1]  Following puberty, affected women may experience a disproportionate accumulation of fat in the hips and lower limbs.[1] 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.[1]
Last updated: 7/29/2011

References

  1. Acquired: Partial Lipodystrophy (Barraquer-Simons Syndrome). The University of Texas Southwestern Medical Center at Dallas Web site. 2007; http://www.utsouthwestern.edu/media/files/2400/Partial-Lipo-Barraquer-Simons-Syndrome.pdf. Accessed 7/29/2011.
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Basic Information

  • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
  • Genetics Home Reference (GHR) contains information on Barraquer-Simons syndrome. This website is maintained by the National Library of Medicine.
  • The National Organization for Rare Disorders (NORD) is a federation of more than 130 nonprofit voluntary health organizations serving people with rare disorders. Click on the link to view information on this topic.

In Depth Information

  • Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
  • Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine. 
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs.  Access to this database is free of charge.
  • PubMed is a searchable database of medical literature and lists journal articles that discuss Barraquer-Simons syndrome. Click on the link to view a sample search on this topic.

Diagrams/Images

Other Names for this Disease
  • APL
  • Lipodystophy partial progressive
  • Lipodystrophy cephalothoracic type
  • Lipodystrophy partial acquired
  • Partial acquired lipodystrophy
Related Diseases
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.