Primary lymphedema

Primary lymphedema is an inherited abnormality of the lymphatic system. Its root causes are  lymph vessels are too few or too small, lymph nodes are too few or too small, lymph vessels and/or lymph nodes fail to function properly, or the causes are a combination of the above disorders 

The lymphatic system has considerable reserve power, which is why it often takes years until its weakness becomes visible. It may be a simple sunburn which triggers system overload and is the last drop “to make the cup overflow”. As a consequence lymphedema will develop.Primary lymphedema usually begins in the distal leg area, i.e. the toes, in rare cases also in the hands. However, it may also develop in any other area of the body. Alongside the swelling, so typical of lymphedema, skin changes will also occur with progressing disease. 

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Thickening of the skin in the second and third toes

The skin in the edema area gradually thickens and coarsens.

If these changes occur at the base of the second toe, we talk of a
“positive Stemmer’s sign”. 

Skin changes in lymphedema

The skin in the affected parts of the body frequently shows the typical changes associated with primary lymphedema:

  • Wart-like lesions (papillomatosis)
  • Excessive growth of horny tissue of the skin (hyperkeratosis)
  • Lymph vesicles
  • Fistula (the name given to an abnormal duct or passageway that links two cavities in the body, frequently opening through the skin)
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These skin changes worsen with the duration and severity of edema. This is why lymphedema must be treated as early as possible to prevent such changes from developing.

Too late a therapy may result in these changes no longer being fully reversible. Primary lymphedema is a chronic disease which cannot be cured, but it can be very well managed by therapy.

 At the Lymphedema Clinic Wittlinger in Walchsee/Austria we treat our patients with the Dr. Vodder Method of Manual Lymph Drainage and compression bandaging. Intensity of treatment will depend on the size of edema and its course. The key to successful therapy is early intervention, plus the patient's compliance with precautionary rules.