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Barraquer-Simons syndrome: a rare form of acquired lipodystrophy.

BMC Res Notes

March 2016

Department of Endocrinology, Diabetes and Metabolism, Centro Hospitalar São João, Alameda Prof. Hernâni Monteiro, 4200, Porto, Portugal.

Background: Human lipodystrophies are uncommon disorders, with important clinical consequences, which are often undiagnosed. The Barraquer-Simons syndrome is a form of partial symmetric lipodystrophy of unknown etiology, characterized by the loss of subcutaneous adipose tissue, limited to upper part of the body. Insulin resistance and metabolic complications are less common than with other lipodystrophy subtypes.

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Do you know this syndrome?

An Bras Dermatol

January 2012

Tropical Medicine Foundation of the Amazonas (FMTAM) – Manaus (AM), Brazil.

Barraquer-Simons syndrome, also called acquired partial lipodystrophy or cephalothoracic lipodystrophy, is a rare form of progressive lipodystrophy, characterized by symmetrical lipoatrophy of subcutaneous adipose tissue starting in the head and spreading to the thorax, upper and lower limbs and thighs. In this work, we report the case of a patient with Barraquer-Simons syndrome without systemic complications.

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[Primary lipodystrophies].

Ann Endocrinol (Paris)

February 2007

Inserm, U680, 75012 Paris, France.

Primary lipodystrophies represent a heterogeneous group of very rare diseases with a prevalence of less than 1 case for 100.000, inherited or acquired, caracterized by a loss of body fat either generalized or localized (lipoatrophy). In some forms, lipoatrophy is associated with a selective hypertrophy of other fat depots.

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Barraquer-Simons syndrome, a disorder of unknown etiology, is characterized by a cephalothoracic lipodystrophy. We present 2 patients treated with a bilateral free transverse rectus abdominis myocutaneous (TRAM) flap to restore facial contour. Our technique of using a muscle component to fill the cheek defect was based on our experience with free muscle transfer in facial reanimation.

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Barraquer-Simons syndrome. Report of a case and review of the literature.

Rev Med Chir Soc Med Nat Iasi

July 2002

Faculty of Medicine, Department of Endocrinology, University of Medicine and Pharmacy Gr. T. Popa, Iaşi.

Among lipodystrophies, the Barraquer-Simons syndrome or "parital lipodystrophy of the cephalothoracic type" is a rare condition of unknown origin characterized by a progressive atrophy of the subcutaneous fat of the upper part of the body. We report a 34-year-old woman with progressive loss of subcutaneous fat during 20 years, first affecting the face and spreading to the upper part of the body, equally presenting hypertrophy of the lower body fatty tissue. An overview of this rare syndrome is made.

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