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What is lymphedema

Damage to the lymphatic vessel system disturbs the capacity to transport waste. Protein and tissue fluid can no longer be carried away and instead accumulate in interstitial tissue, this causes the tissue to swell, so-called lymphedema. The latter always manifests itself in that part of the body where lymph draining ability is impaired. Especially affected are arms and legs. Lymphedema can also occur on organs, the head or genitals. It afflicts women more frequently than men.
Depending on the cause, a distinction is made between primary and secondary lymphedema.

Primary lymphedema

Lymphedema is described as "primary" when already at birth a malformation of the lymphatic vessels exists.
Possible causes of primary lymphedema could be:

  • Complete absence of lymphatic vessels, usually in individual parts of the body (aplasia/atresia)
  • Lymphatic vessels are too narrow or too few (hypoplasia)
  • Distended vessels (hyperplasia, lymphangiectasis)
  • Hardening of lymph nodes (lymph node fibrosis)
  • Absence of lymph nodes (lymph node agenesia)

Primary lymphedema tends to develop between puberty and the age of 35. However, it can already be present at birth or develop after the age of 35. Primary lymphedema can be hereditary but usually occurs sporadically.

Secondary lymphedema

One speaks of "secondary lymphedema" when damage to the lymph function was sustained through external causes. It is then the result of damage to the lymph vessels or lymph nodes.
The causes of secondary lymphedema are:

  • Injury
  • malignant disease (eg a tumour)
  • Inflammation of the skin (eg erysipelas)
  • Parasites
  • Operations and/or radiation

The principal cause of secondary lymphedema in western industrial nations are operations for cancer in which lymph nodes are partially or completely removed as well as radiation treatment.

Lymphedemas differ in appearance, site of the disease and shape. Unfortunately lymphedema is often diagnosed too late. Many afflicated persons are not receiving appropriate treatment and consequently the number of patients in Germany is according to the German Society for Vascular Disease (Deutsche Gefäßliga) estimated to be approx 80,000.