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.