Differential clinical expression of multiple symmetric lipomatosis in men and women

Department of Medical and Surgical Sciences, University of Padova, Italy.
International Journal of Obesity (Impact Factor: 5). 11/2003; 27(11):1419-22. DOI: 10.1038/sj.ijo.0802427
Source: PubMed


Multiple symmetric lipomatosis (MSL) is a rare disease characterised by the growth of uncapsulated masses of adipose tissue. MSL is associated with high ethanol intake and complicated by somatic and autonomic neuropathy and the infiltration of adipose tissue at the mediastinal level. To date, the disease is considered as largely more prevalent in men.
To provide a detailed description of the clinical aspects of MSL in women.
A total of 11 women and 58 men with MSL.
Morphological aspect of patient, location of the lipomatous masses, alcohol intake, extension of lipomatous tissue to the mediastinum, association with somatic and autonomic neuropathy, and metabolic profile.
All female patients had the obesity-like appearance of type II MSL and the most frequent locations of lipomatous masses in women were at the proximal arms (90.9%) and legs (54.5%). Very few female patients (27.3%) presented with the submental deposition of lipomatous tissue typical of both type I (97.3%; P<0.001) and type II (66.7%; P<0.05) male subjects. An extension of the lipomatus tissue around the upper airways, associated with compression or dislocation of deeply located mediastinal structures, was observed less frequently in women than in men. The presence of a high ethanol intake, the association with somatic and autonomic neuropathy and the occurrence of a characteristic metabolic pattern (high HDL-cholesterol, low LDL-cholesterol, high uric acid) were similar in men and in women.
Female patients with MSL had a sex-specific morphological aspect, characterised by a low occurrence of the typical 'Madelung collar' and a usual obesity-like appearance.

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    • "RADs include Dercum's disease (DD), multiple symmetric lipomatosis (MSL) and familial multiple lipomatosis (FML). These disorders differ in whether or not pain is present and in the location of fatty masses [10,16,19]. The pathophysiology of RADs is believed to be different from the generalized accumulation of fat in obesity because the abnormal SAT follows discrete patterns, can occur in both obese and non-obese individuals [10,20], may include loss of normal fat (as in MSL)[21], and may involve alterations of the lymphatic system [21,22]. "
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    ABSTRACT: Elevated stearoyl-CoA desaturase activity has been described in obese states, with an increased desaturation index (DI) suggesting enhanced lipogenesis. Differences in the DI among various phenotypes of abnormal adiposity have not been studied. Abnormal accumulation of subcutaneous adipose tissue occurs in rare adipose disorders (RADs) including Dercum's disease (DD), multiple symmetric lipomatosis (MSL), and familial multiple lipomatosis (FML). Examining the DI in subcutaneous fat of people with DD, MSL and FML may provide information on adipose tissue fatty acid metabolism in these disorders. The aims of this pilot study were: 1) to determine if differences in adipose tissue DIs are present among RADs, and 2) to determine if the DIs correlate to clinical or biochemical parameters. Subcutaneous adipose tissue was obtained from human participants with DD (n = 6), MSL (n = 5), FML (n = 8) and obese Controls (n = 6). Fatty acid composition was determined by gas chromatography/mass spectrometry. The DIs (palmitoleic/palmitic, oleic/stearic, vaccenic/stearic ratios) were calculated from the gas chromatogram peak intensities. SCD1 gene expression was determined. Spearman's correlations between the DIs and available clinical or biochemical data were performed. In DD subjects, the vaccenic/stearic index was lower (p < 0.05) in comparison to Controls. Percent of total of the saturated fatty acid myristic acid was higher in DD compared with Controls and FML. Percent of monounsaturated vaccenic acid in DD trended lower when compared with Controls, and was decreased in comparison to FML. In MSL, total percent of the polyunsaturated fatty acids was significantly lower than in the Control group (p < 0.05). In the total cohort of subjects, the palmitoleic/palmitic and oleic/stearic DIs positively correlated with age, BMI, and percent body fat. The positive associations between the DIs and measures of adiposity (BMI and percent body fat) support increased desaturase activity in obesity. The lower vaccenic/stearic DI in DD SAT compared with Controls suggests presence of other factors involved in fat accumulation in addition to lifestyle. Other mechanisms driving fat accumulation in DD such as inflammation or lymphatic dysfunction should be investigated.
    Lipids in Health and Disease 02/2012; 11(1):19. DOI:10.1186/1476-511X-11-19 · 2.22 Impact Factor
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    • "MSL type III is clinically similar to and may be instead, the RAD, lipedema (see below). Women tend to have Type II and III MSL with widespread altered SAT41. "
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    ABSTRACT: 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.
    Acta Pharmacologica Sinica 02/2012; 33(2):155-72. DOI:10.1038/aps.2011.153 · 2.91 Impact Factor
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    ABSTRACT: Benign symmetric lipomatosis is a rare syndrome characterized by gradual appearance of encapsulated masses of adipose tissue, affecting the upper part of the trunk, neck, and limbs. For the peculiarity of its clinical features, this condition received comparative denominations from its original authors, which are classical and still appropriate. Based on a report of a typical case of the condition, some aspects of the syndrome are exem- plified, as the 'horse-collar' and the 'pseudoathletic' appearance.
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