What is lymphedema?

Lymphedema is a long-term (chronic) disease where a protein-rich fluid called lymph collects in the tissues and causes swelling. Lymphedema occurs either because the lymphatic system isn’t working properly due to diseases or injuries (secondary lymphedema) or because it’s not developed properly due to genetic reasons (primary lymphedema).

Illustration of a woman with lymphedema, showing visible swelling and deformity in one leg.

What causes lymphedema?

Primary and secondary lymphedema can have different causes.

Causes of primary lymphedema:

Aplasia: Parts of the lymphatic system are missing
Hypoplasia: Lymphatic vessels are underdeveloped or present in below-average numbers. This is the most common cause of primary lymphedema.
Hyperplasia: Lymphatic vessels are larger than normal and don't work as well as normal-sized vessels.
Fibrosis of lymph nodes: hardening and scarring of the tissue

Causes of secondary lymphedema:

Cancer and its treatment
Obesity
Trauma
Infectious diseases (e.g. parasites)

In both cases, missing or damaged lymph vessels or lymph nodes cause a blockage of the lymphatic system. The lymph is unable to drain properly and collects in the tissues. This results in swelling in the affected areas.

Signs and symptoms of lymphedema

The most common signs and symptoms of lymphedema are:

Swelling of your arm(s), leg(s) or other parts of the body
Feeling of heaviness or tightness
Aching or discomfort
Less mobility and range of motion
Clothing or jewelry feel tighter than usual
Skin changes such as hard (fibrotic) skin in advanced disease

There is no specific test or tool to diagnose lymphedema, which leaves many patients without a correct diagnosis for a long time.

Diagram showing both phases of Complex/Complete Decongestive Therapy and their elements.

How can lymphedema be treated?

The gold standard for treating lymphedema is called Complex/Complete Decongestive Therapy (CDT). The aim of CDT is to reduce swelling, improve the condition of the skin and increase mobility. It comprises two parts:

Phase I – Decongestion: Intensive treatment phase to reduce the swelling as much as possible, improve the limb shape and skin condition
Phase II – Maintenance: Long-term control to maintain and maximize the achievements of Phase I

Both phases have several elements in common. These are compression therapy, Manual Lymphatic Drainage (MLD), skin care, and exercise. Self-Management plays an important role as well – it means both a deep understanding of the disease and ways to manage your lymphedema on your own and together with your carers.