[Barraquer-Simons syndrome].

Rev Stomatol Chir Maxillofac

Unité de stomatologie et chirurgie maxillo-faciale, service de traumatologie A, université Cadi-Ayyad, CHU Mohammed-VI-Marrakech, Marrakech, Morocco.

Published: June 2011

The Barraquer-Simons syndrome is a rare acquired acral partial lipodystrophy of unknown etiology. It progressively develops during childhood. It leads to a complete loss of subcutaneous fat in the face, neck, trunk and upper limbs. The disease is more common in female than male patients and causes facial dysmorphy. Free fat transplantation seems to be an appropriate technique to treat facial abnormalities encountered in this syndrome.

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http://dx.doi.org/10.1016/j.stomax.2011.03.005DOI Listing

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Article Synopsis
  • Barraquer-Simons syndrome (BSS) is a rare acquired lipodystrophy leading to significant loss of upper body fat, particularly affecting the face and arms, with a possible autoimmune origin.
  • This study evaluated clinical and immunological data from 26 BSS patients to refine diagnostic tools and identify potential triggers or genetic links.
  • Key findings revealed most patients experienced fat loss in childhood, with a notable prevalence of autoimmune markers and metabolic conditions, suggesting BSS is commonly connected to other autoimmune diseases.
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