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Lymphoedema

Lymphoedema develops through the accumulation of protein-rich fluid in the tissues. Lymph fluid (lymph, Latin: lympha = clear water) cannot be sufficiently transported away by the impaired lymphatic system and accumulates as a result. Lymphoedema can develop in all parts of the body but affects the legs in a majority of cases. 

Self-check

Lymphoedema or lipoedema?

To illustrate the differences between the two conditions, lymphoedema and lipoedema, we have drawn up a comparison of the most important distinguishing criteria.

LYMPHOEDEMALIPOEDEMA

Appearance of the oedema

asymmetricalsymmetrical
Painful sensitivity to pressurenoyes
Bruises (haematoma)noyes
Erysipelascommonno

Swelling on

back of the foot and/or hand

yesno

Important: This self-check is not a replacement for seeing a medical specialist. It is crucial that you consult a medical specialist to obtain a correct diagnosis.

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Good to know

  • A positive Stemmer’s sign may indicate a leg lymphoedema. You can easily test this yourself. 
  • Try to lift the skin on your second toe with your index finger and thumb. If you can lift the skin and it is easy to hold on to, this is a negative Stemmer’s sign. In this case it is unlikely that you have lymphoedema. 
  • If you cannot lift the skin, this is a positive Stemmer’s sign and indicates that it is likely you have lymphoedema. 
  • Compare the skin folds on the second toes on both feet. On a foot with a positive Stemmer’s sign, the fold is significantly thicker.
  • A negative Stemmer’s sign does not always mean that lymphoedema can be excluded. If you have other symptoms, it is essential that you consult a specialist physician to examine the problem.


Lymphoedema

In many cases, lymphoedema is not accompanied by pain but can lead to swellings with tightness in the affected areas that often results in major movement restrictions and further complications.  Both women and men are afflicted by lymphoedema, with a higher number of women being affected.

The most common triggers for lymphoedema can be found in previous ailments such as, for example, breast cancer in women. A congenital disposition (e.g. too few lymph vessels) is a lot less frequent. Lymphoedema does not develop immediately following an operation involving the lymphatic system. This often happens years later, i.e. with a long delay of the onset.

Therefore it can happen that the disorder is not immediately recognised as lymphoedema (stage 0). During the time that passes without treatment, the condition of the lymphoedema can deteriorate and make later treatment more difficult. 

Lymphoedema can be divided into two types based on their different causes:

Primary lymphoedema

Primary lymphoedema can develop immediately after birth or during the course of your life and is due to the following causes:

  • Congenital (inherited) absence of lymph vessels 
  • Over- or underdevelopment of lymph vessels and lymph nodes
  • Hardening of lymph nodes

Secondary lymphoedema


Secondary lymphoedema is a lot more common than primary lymphoedema. It is always due to a preceding cause that led to damage or an impairment of the lymphatic system. Secondary lymphoedema can develop in any area of the body and is due to a variety of causes. For example:

  • Injuries causing damage to lymph vessels or nodes
  • Surgical procedures requiring the removal of lymph nodes or sectioning of lymph vessels (e.g. breast cancer surgery)
  • Inflammations due to viruses, bacteria, fungi, etc.
  • Untreated chronic venous insufficiency
  • Tumours, malignancies, radiation, infections, insect bites, etc.

Mixed forms of oedema

In addition to the primary and secondary types of lymphoedema, mixed forms of oedema can also develop:

Phlebolymphoedema 

Phlebolymphoedema almost exclusively develops on the legs. It is the consequence of a phlebological disease, i.e. a disorder of the venous system. Phlebolymphoedema can be caused by, for example, varicose veins or also a thrombosis. If an oedema that developed as a consequence of a venous disorder (phleboedema) remains untreated, the lymphatic system will become overloaded over time. The tissues harden and a phlebolymphoedema will develop. 


Lipo-lymphoedema

Lipo-lymphoedema can develop in the advanced stages and as a complication of lipoedema (explanation from page 20 onwards in this brochure). The lymphatic vessels become constricted due to an overgrowth of fatty tissue. Lymph can no longer drain sufficiently and accumulates in the tissues. In contrast to normal development, lymphoedema associated with lipoedema usually develops symmetrically. 

Symptoms

The symptoms of lymphoedema can be categorised into internally experienced symptoms and externally visible symptoms. You can use the following signs to identify whether you are potentially suffering from lymphoedema.

The symptoms of lymphoedema

Symptoms that are visible on the outside
These signs are clearly evident on the affected parts and can, to a varying extent, have the following characteristics:

  • Self-test
  • Swelling of a limb that is not the same on both sides but asymmetrical (e.g. just one leg)
  • The back of the foot and/or hand is often swollen as well
  • Skin discolourations and other skin changes
  • Susceptibility to inflammations and skin irritations
  • Clearly visible wrinkles and furrows
  • Possible movement restrictions


Internally experienced symptoms
In addition to the visible signs, there are other symptoms that could indicate lymphoedema:

  • Tired, heavy or painful limbs
  • Feeling of tension and tightness
  • Tingling or twinges
  • Slight numbness of the affected body part
  • Quicker fatigue of the affected leg or arm

If you notice one or more of the signs of lymphoedema, please consult a medical specialist. Early diagnosis has a positive effect on the progression of the disorder.

Thumb test

Press your thumb into the affected tissue for approx. 10 seconds. If the indentation does not disappear immediately, once you have removed the thumb, this would indicate lymphoedema. 

Stages

Lymphoedema can be divided into four stages, depending on severity. The stage also determines the type of treatment of the lymphoedema, which should commence immediately upon diagnosis to counteract any enlargement or deterioration. 

Woman with umbrella

Stage 0 – latency stage

  • Damage to the lymphatic system is present and known
  • Oedema not (yet) visible


Stage I – spontaneously reversible stage

  • Soft swelling
  • Elevation results in a reduction of the oedema
  • Finger pressure leaves a visible indentation


Stage II – spontaneously irreversible stage

  • Hardening of the connective tissue
  • Elevation does not result in a reduction of the oedema
  • Creating an indentation with the finger is scarcely possible


Stage III – elephantiasis

  • The volume of the affected body part has increased dramatically
  • Hardened skin with skin changes
  • Severely restricted mobility 


Treatment concept

Complex decongestive therapy (CDT) is the only treatment method that has been proven to contribute to an improvement of the condition or counteract a possible deterioration of lymphoedema. This includes manual lymphatic drainage (MLD), compression treatment tailored to the particular stage of the lymphoedema, skincare and decongestive exercises, as well as the necessary degree of personal initiative on the part of the patient. Consistent and sustained treatment is the only way of reducing the severity of lymphoedema and maintaining it in the long term. 

Surgical methods

  • Resection methods
  • Drainage methods
  • Reconstructive methods

All of these measures and methods are subject to highly special conditions and requirements and their benefits and risks must always be assessed.

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Good to know

We have compiled some tips on how you can do something good for your body in addition to the lymphoedema treatment you are receiving. Support your well-being through appropriate exercise and a healthy diet. Find out what is most helpful for you personally and what does you good! 

Complications

If lymphoedema remains untreated or is treated inadequately or in the wrong way, skin abnormalities, hardening of the tissues and other complications can develop leading to additional problems.

The skin on the body parts affected by lymphoedema can dry out and become scaly. Protein that cannot be sufficiently transported away by the lymphatic system collects in the tissues. This can lead to inflammation. New connective tissue forms and the oedema consequently gets bigger and hardens (fibrosis).

Because the immune system of lymphoedema patients is impaired in the affected area, the organism is more prone to bacterial skin infections such as erysipelas or fungal infections (mycosis). These require immediate medical treatment.