Lymphoedema arises when the lymphatic system is unable to fulfill its function of regulating fluid balance in the body. This results in oedema which can occur anywhere in the body but mainly in the limbs. It also causes changes to occur in the skin and body tissue.
There are two types of lymphoedema:
Primary and Secondary
Primary Lymphoedema or hereditary lymphoedema occurs when a person is born with an abnormal lymphatic system which impairs its function. Swelling may be present at birth or become apparent later in life.
It is divided into three categories:
Conatal Lymphoedema: This means that it is present or noticeable at the time of birth, and does not resolve. It is important that the child is seen by a doctor, as not all oedema or limb size difference is lymphoedema. Differences in the size of limbs may also be caused by malformation of blood vessels, or other reasons.
Lymphoedema Preacox: This is when lymphoedema is first noted around puberty for the first time. Usually it begins slowly, and may fluctuate on a daily basis. However with time it progresses and remains swollen. If untreated it becomes progressively worse.
Lymphoedema Tarda: This is when the lymphoedema is first seen when a person is in their mid-thirties or later. It is not well understood why it would only present at this time, even though the underdevelopment of lymph nodes has been present since birth. Again, it usually starts slowly, and may fluctuate initially but then the swelling remains and worsens over time (if left untreated).
Secondary: Infection or Trauma
It is difficult to identify who is most at risk of developing the condition, but an infection or trauma to the limb or area of cancer treatment is often identified as being the catalyst. In some cases it may not appear for many years and not everyone who has damage to their lymphatics will automatically go on to get lymphoedema. However some patients who have not had lymph nodes removed may be at risk of developing the condition due to the trauma and infection in the area.
If lymphoedema is not treated or controlled it may lead to repeated infections (cellulitis) and the skin tissue hardens (fibrosis) leading to more complex skin and lymph problems. Occasionally the skin breaks and lymph fluid leaks out (lymphorrhea).