Journal
ACTA PHARMACOLOGICA SINICA
Volume 33, Issue 2, Pages 155-172Publisher
ACTA PHARMACOLOGICA SINICA
DOI: 10.1038/aps.2011.153
Keywords
adiposis dolorosa; Dercum's disease; lipedema; multiple symmetric lipomatosis; familial multiple lipomatosis; familial partial lipodystrophy; lymph; lymphatics
Funding
- UCSD NIDDK Diabetes and Endocrinology Research Center [P30 DK063491]
- UCSD General Clinical Research Center [5M01RR000827]
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Rare adipose disorders (RADs) including multiple symmetric lipomatosis (MSL), lipedema and Dercum's disease (DD) may be misdiagnosed as obesity. Lifestyle changes, such as reduced caloric intake and increased physical activity are standard care for obesity. Although lifestyle changes and bariatric surgery work effectively for the obesity component of RADs, these treatments do not routinely reduce the abnormal subcutaneous adipose tissue (SAT) of RADs. RAD SAT likely results from the growth of a brown stem cell population with secondary lymphatic dysfunction in MSL, or by primary vascular and lymphatic dysfunction in lipedema and DD. People with RADs do not lose SAT from caloric limitation and increased energy expenditure alone. In order to improve recognition of RADs apart from obesity, the diagnostic criteria, histology and pathophysiology of RADs are presented and contrasted to familial partial lipodystrophies, acquired partial lipodystrophies and obesity with which they may be confused. Treatment recommendations focus on evidence-based data and include lymphatic decongestive therapy, medications and supplements that support loss of RAD SAT. Associated RAD conditions including depression, anxiety and pain will improve as healthcare providers learn to identify and adopt alternative treatment regimens for the abnormal SAT component of RADs. Effective dietary and exercise regimens are needed in RAD populations to improve quality of life and construct advanced treatment regimens for future generations.
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