[MIM 614 103]

Rare disease that could affect up to 11 % of the post-pubertal female population.  It belongs to the group of rare diseases involving adipose tissue such as the diffuse and symmetrical lipomatoses (Madelung and Launois-Bensaude disease) or Dercum disease. A family history is present in 15 % of cases.

This disease  affects almost exclusively women.

Features:

-        onset around puberty

-        symmetric involvement of the lower limbs, from the hips to the ankles, and leaving the feet unaffected; sometimes pseudotumoral masses at the level of the thighs

-        the skin remains supple; little pitting edema

-        elevation of the limbs or weight loss are not effective

-        tenderness on pressure and pinch ("cellulalgia")

-        spontaneous bruising

-        after several years: appearance of secondary lymphedema with a risk of superinfection (erysipela)

Cause: functional failure of the lymphatic system, abnormal production of lymph in the fat cells, role of female hormones ?

Differential diagnosis: obesity, lymphedema

Treatment: liposuction and/or cutaneous resection.

Anesthetic implications:

none

References:

-        Vignes S.
Lipoedème : une entité mal connue.
J Mal Vasc 2012 ; 37 : 213-8.

Updated: February 2019