Lymphedema is classified as primary or secondary. In primary lymphedema there is usually no cause found. In secondary lymphedema, the cause may be an infection, cancer, or surgery
Facts about primary lymphedema
In most cases, the disorder is inherited. The genetic link for primary lymphedema has not been identified and not everyone in the family develops the condition. Most individuals begin to show enlargement of the extremity (usually leg) in their 2nd decade of life.
Primary lymphedema tends to affect more women than men. Most females will notice a slight discrepancy in their legs just after puberty. The swelling may initially start off at the foot or ankle but will gradually affect the entire leg.
Congenital (Milroy’s) disorder usually starts at a younger age. This disorder occurs chiefly because of enlarged lymph nodes in the groin that do not connect with the lymphatic vessels in the leg. When the connection fails, fluid in the leg has nowhere to drain and starts to pool in leg tissues.
Another cause of lymphedema is known as Meige disease (lymphedema praecox). This inherited disorder is seen in the first decade of life or just after puberty. The lymphatic vessels do develop but have no valves. Thus, the fluid keeps on flowing backwards and collects in the extremities. Further lymphatics vessels have very thin wall and cannot actively push out the fluid. With time, the condition progresses and significant swelling of the extremities results.
There are a few individuals who suddenly develop lymphedema of their legs in the 3rd decade of life. Known as lymphedema tarda or delayed onset lymphedema, it is believed to be a mild version of primary lymphedema. Most individuals notice that the leg is gradually increasing in size and that they are no longer able to put on the shoes or wear socks. The disorder affects the entire limb and is frequently mistaken for varicose veins.
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