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Causes and Types of Lymphedema

The most common causes of lymphedema are damages to the lymphatic system due to injuries or other diseases (secondary lymphedema). A primary lymphedema is a rare type of lymphedema that mainly occurs in early life.

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Primary and secondary lymphedema are the two main types of lymphedema. In both cases, missing or damaged lymph vessels or lymph nodes cause a blockage of the lymphatic system. The protein-rich fluid called lymph or lymphatic fluid is unable to drain properly and collects in the tissues. This results in swelling in the affected areas.

Causes of primary lymphedema

Primary lymphedema is a rare disease that occurs when the lymphatic system has not developed adequately (or is not functioning as it should) from birth due to a genetic reason. Primary lymphedema develops as soon as the lymph vessel system can no longer cope with the strain. This usually happens in early life. It is called 'primary' because the impairment is not due to a ‘secondary’ cause like an illness or an injury.

Primary lymphedema can have different causes:

There are different types of primary lymphedema and there are many known syndromes that have been linked with it, including Noonan Syndrome, Prader Willi, Yellow Nail Syndrome, and Turner Syndrome.

An example for the congenital onset lymphedema is Milroy’s disease where the lymphedema is noticed at birth or shortly afterwards. The late onset primary lymphedema (e.g. Meige disease) presents at a later stage, with Meige disease usually around puberty.

Causes of secondary lymphedema

Secondary lymphedema is more common than primary lymphedema and is caused by another disease or an injury. The lymphedema develops either because the lymphatic system is damaged or there is too much lymph fluid that cannot be drained properly (overload).

Damages to the lymphatic system can be a result of:

An overload of the lymphatic system can be a result of:

All these causes affect the lymphatic system. They either impair the flow, increase higher lymph production, damage the lymph vessels or cause pressure on the lymphatic system.