Barraquer-Simons Syndrome

Abstract Background Barraquer-Simons syndrome (BSS) is a rare acquired lipodystrophy characterized by gradually symmetric subcutaneous fat loss in a craniocaudal distribution, associated with hypocomplementemia, diabetes and hypertriglyceridemia. Few investigators have studied body fat distribution...

Full description

Saved in:
Bibliographic Details
Published in:The American journal of the medical sciences 2016-09, Vol.352 (3), p.280-284
Main Authors: Small, Juan E., MD, Jassam, Yasir N., MD, Small, Kirstin M., MD, Chea, Pauley, MD, Popov, Veljko, MD, Li, Sui, MD, Srinivasan, Jayashri, MD
Format: Article
Language:English
Subjects:
Adult
Aged
Barraquer-Simons
Diagnosis, Differential
Endocrinology
Female
Humans
Hypocomplementemia
Internal Medicine
Lipodystrophies
Lipodystrophy - pathology
Magnetic Resonance Imaging
Phenotype
Subcutaneous Fat - pathology
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Abstract Background Barraquer-Simons syndrome (BSS) is a rare acquired lipodystrophy characterized by gradually symmetric subcutaneous fat loss in a craniocaudal distribution, associated with hypocomplementemia, diabetes and hypertriglyceridemia. Few investigators have studied body fat distribution with cross-sectional imaging techniques. Methods We present 2 cases of BSS with emphasis on phenotypic analysis through cross-sectional imaging. Results For the first time, we demonstrate bone marrow involvement and deep cervical and axillary fat sparing of Barraquer-Simons using magnetic resonance imaging. Conclusion Phenotypic analysis in lipodystrophies such as Barraquer-Simons is an essential guide for future experiments. Therefore, careful analysis of cross-sectional imaging should be conducted in future studies as areas of involvement or fat sparing may be overlooked. The major contributions of our work are that this is the first time that deep cervical or nuchal and axillary fat sparing and bone marrow involvement has been documented in BSS.
ISSN:0002-9629
1538-2990
DOI:10.1016/j.amjms.2016.05.007