Management of Lymphedema

Primary

Primary Lymphedema is rare and results from abnormal development of the lymphatic system.

Symptoms may appear starting at birth or later in life.

Precox or Meige’s syndrome
It appears in adolescence or up to the third decade of life and represents 77-94% of cases. It often occurs in women (female to male ratio 10:1) and this may be why estrogen plays a role.
Patients show smaller and fewer lymph vessels compared to healthy individuals.

Tarda
Appears after the age of 35 or in the last stages of life, usually in 10% of cases of primary lymphedema. Patients with this form of lymphedema have abnormal and non-functional lymph vessels.

Hypotrichosis lymphedema telangiectasia:
It is the rarest form of primary lymphedema. It is caused by mutations in the transcription factor SOX18.

Congenital or Milroy’s Disease
It appears at birth or in the first 2 years of life. It is more common in men than in women.

Secondary

Secondary Lymphedema is more common. This form is caused by damage to the lymphatic system from cancer, radiation therapy, surgery, lymph nodes removal, scar tissue, or parasites (rarely).

It is the most common form of lymphatic dysfunction.

For the developed countries, causes are iatrogenic with the main one’s being surgeries, lymph node exclusion and radiation therapy. For the tropical countries the most common cause is lymphatic filariasis (elephantiasis), a parasitic infection, which is caused by parasites such us Wuchereria bancrofti, Brugia malayi and Brugia timori. This parasite causes an inflammatory reaction which stimulates the production of VEGFs resulting in the hyperplasia, blockage and fibrosis of the lymph nodes.