Differential clinical expression of multiple symmetric lipomatosis in men and women.

BACKGROUND: 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. OBJECTIVE: To provide a detailed description of the clinical aspects of MSL in women. PATIENTS: A total of 11 women and 58 men with MSL. MEASUREMENTS: 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. RESULTS: 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. CONCLUSIONS: 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.

Multiple symmetric lipomatosis: clinical aspects and outcome in a long-term longitudinal study.

BACKGROUND: Multiple symmetric lipomatosis (MSL) is a rare disease characterized by the growth of uncapsulated masses of adipose tissue. MSL is associated with high ethanol intake and complicated by somatic and autonomic neuropathy and by the infiltration of the adipose tissue at the mediastinal level. To date, the disease is considered as slowly progressive, but long-term longitudinal data are still lacking. In this study, a long-term follow-up of a large series of MSL patients is presented. METHODS: We studied 31 patients with MSL (30 males and one female) first evaluated at our institution from 1973 to 1992. All patients were followed until 1998-1999 or until death, with a mean follow-up of 14.5+/-5.0 y (range 4-26 y). Both at baseline and during follow-up, the location and the size of the subcutaneous lipomatous fat depots, the presence and the extension of deeply localized lipomatous tissue, and the presence and the severity of both somatic and autonomic neuropathy were evaluated. RESULTS: Eight MSL patients died during follow-up (25.8% of patients). A sudden death was proved to be the cause of death in three patients. All these three patients had severe autonomic neuropathy and none had coronary disease, acute myocardial infarction or other cardiac abnormalities. No signs or symptoms of coronary heart disease were present in the whole series. In addition to this high fatality rate, a substantial morbidity related to the occupation of the mediastinal space by the lipomatus tissue and to somatic neuropathy was also observed. CONCLUSIONS: MSL is associated with a significant morbidity and mortality. Therefore, the definition of 'benign symmetric lipomatosis', still adopted by several authors, cannot be justified.

Lipoprotein(a) and lipoprotein profile in healthy centenarians: a reappraisal of vascular risk factors.

In this study we assessed whether widely accepted risk factors for atherosclerotic vascular diseases such as lipoprotein(a) [Lp(a)], a cholesterol-rich lipoprotein under strict genetical control, and other lipid parameters change with age. The variations of blood levels and the pathophysiological role of Lp(a) in old people, and particularly in the oldest old, are unknown. Accordingly, we measured Lp(a) levels as well as total, LDL, and HDL cholesterol (CT), and triglycerides (TG) in sera from 75 healthy centenarians, 114 randomly selected subjects under 65 years, 73 randomly selected elderly people, and 30 healthy selected elderly people. The results showed that Lp(a) serum levels did not vary by age group, including centenarians. Remarkably, one-quarter of the centenarians had high Lp(a) serum levels even though they never suffered from atherosclerosis-related diseases. At variance with young and aged people, centenarians with high Lp(a) serum levels also had high plasma concentrations of the proinflammatory cytokine IL-6, suggesting that genetic control of the Lp(a) serum level may attenuate with age and that environmental factors such as chronic subclinical inflammatory processes may play a role. We also showed that most centenarians are paradoxically characterized by low HDL-CT and relatively high TG levels, which together are considered to be strong risk factors for coronary heart disease. On the whole, these data support the hypothesis that a continuous and complex reshaping of lipid metabolism occurs in physiological aging, likely contributing to successful aging.