Lymphedema

Lymphedema is a swelling caused by an increased accumulation of tissue water (lymph) and is located under the skin. As soon as more fluid flows into the tissue than the lymphatic channels can remove, the lymph accumulates and the tissue swells.

Lymphedema affects either the extremities (e.g. arms or legs) or the whole body, which is called generalized lymphedema. These edemas can be distinguished by their protein content, which means that they can contain more or less protein. The swelling that develops can result in an undersupply of the affected tissue. Congested fluid then forces itself between cells (e.g. skin) and their supplying blood vessels, which makes it difficult for nutrients to reach them and can thus lead to damage or death. To minimize the damage, lymphedema should be treated as soon as possible.

Furthermore, a distinction can be made between primary (hereditary) and secondary (acquired) lymphedema, which is about twice as common as hereditary lymphedema. Congenital lymphedema mostly affects women from the age of 17.

Lymphatic system
The lymphatic system describes a vascular network, connected by lymphatic channels, which supports the immune defence. It transports tissue fluid and proteins (including pathogens) from intercellular spaces first into veins, then into lymph nodes. There everything is filtered and cleaned. Muscles and joints function as a supporting pump in this circulation.

Lymphedema is an accumulation of water in the tissues. It occurs when too much fluid gets into the space between the tissues (interstitium) or it cannot drain away quickly enough.

There can be four main causes of this:

1. Increased pressure in blood vessels. This presses more fluid into the intercellular space.
2. A lower protein content of the blood (low oncotic pressure). More fluid flows into the intercellular space to compensate for the resulting difference in concentration.
3. The small blood vessels are more permeable than normal. The lymph cannot drain properly.
4. An inflammation or a tumor pressing on the lymphatic channels may be responsible. The higher pressure in the lymphatic system then forces more fluid into the intercellular spaces.

In practice, a distinction is made between primary (hereditary) and secondary (acquired) lymphedema.

Primary lymphedema (hereditary)
Congenital malformations are responsible for one in ten cases of lymphedema. The predisposition to it may either have been inherited, the concomitant of another hereditary disease, or have arisen spontaneously. The majority of cases are caused by incomplete development of the lymphatic vessels in the womb. Approximately 85 percent of those affected are women, most of whom are over the age of 17. Primary edema usually begins at the outermost part of the extremities (e.g. the toes) and then spreads to the trunk. It can occur on one or both sides of the body.

Secondary lymphedema (acquired)
It is about twice as common and, unlike primarylymphedema, it spreads from the trunk of the body toward the extremities. Almost always, lymph cannot drain adequately due to disease or medical treatment. Lymph nodes may have been removed by surgery or lymph vessels may have been destroyed by radiation. Injuries or inflammations are rather the exception. Breast cancer is the leading cause of secondary lymphedema. One in ten women has permanent lymphedema on the arm of the same side of the body after a tumor operation.

Causes of secondary lymphedema

• Surgery (e.g. breast cancer)
• Radiation (e.g. breast cancer)
• Accidents or injuries (trauma)
• Infections
• Inflammations
• Parasitic diseases
• Malignant tumours (which attack lymphatic vessels)
• Self-harm (e.g. cutting off an arm)
• Removal of veins (e.g. for a heart bypass)
• Diseases of the small blood vessels

Lymphedema can occur on one or both sides of the body. The size of the swelling depends on the amount of fluid accumulated. Hereditary lymphedema initially occurs on the outermost parts of the extremities. This typically results in swollen, so-called box toes, as their shape resembles a cuboid. In the affected areas, the skin is often rough due to swelling. Acquired lymphedema first appears in the trunk of the body and spreads from there towards the extremities. In the case of surgery, they may appear either immediately afterwards or within two years.

Symptoms that may indicate lymphedema include

• Swelling on one side, the skin can be dented by finger pressure Quickly
• onset of fatigue in the affected limb
• Increased blood flow (visible veins)
• Skin discoloration and similar changes
• Widened furrows and wrinkles of the skin, can no longer be distinguished from joints (e.g. on toes, fingers)

Four degrees of severity of lymphedema

• Stage 0, latency stage No visible swelling; dysfunction of the lymph vessels detectable (functional lymph scintigraphy)
• Stage 1, Reversible swelling Soft swelling; dimpling when pressure is applied to the tissue, disappears spontaneously after some time; no tissue changes in the skin
• Stage 2, irreversible swelling Swelling is difficult to depress, does not recede even when elevated; fibrosis (excess connective tissue) develops around the edema
• Stage 3, elephantiasis Affected areas are severely enlarged and changed in shape; hardened skin, bulges develop; high risk of infection and deep, poorly healing wounds Feeling of pressure, tension and heaviness (also painful) Slight tingling, stinging or numbness

Lymphedema in the early stages is difficult to see with the naked eye. Doctors help themselves in this case with a measuring tape, with which even slight swellings can be detected. Visible indications are deepened skin folds, discolorations and also rough skin on the back of the fingers and toes, which is difficult or impossible to lift. In order to determine the cause, the physician needs information about recent operations, infections, accidents or illnesses. A further step is a blood test, which provides information about possible inflammations or other diseases through individual values (e.g. protein content, liver values).

Lymphatic drainage

Lymphatic drainage is the most effective form of therapy. A therapist massages the accumulated fluid out of the affected tissue. The treatment is supported by the use of compression bandages, stockings and certain gymnastic exercises. The aim is to reduce skin changes and swelling and to increase the mobility of the limbs. The duration of therapy depends on the success of the treatment. Generally, it is continued until the circumference of the limb cannot be reduced any further. The frequency of drainage depends on the severity of the edema. On average, daily treatments over a period of three to six weeks have proven successful. Wearing specially made compression stockings (also on the arms) is of great importance for success.

It should be noted that stage 1 lymphedema can normally only be reduced to stage 0. Those in stage 2 or 3 almost always require lifelong treatment.

Contraindications for lymphatic drainage are concomitant diseases of high blood pressure such as coronary heart disease (CHD) or diabetes in conjunction with nerve damage (diabetic neuropathy).

Exercise therapy

The ability of the muscles to support the lymphatic system in its pumping function is used here. In particular, the muscle groups of the affected limbs are stimulated. The resulting pressure on veins and lymph vessels supports the removal of fluid accumulations in the tissue. Swimming, cycling or Nordic walking, for example, have proven to be particularly suitable.

Medication

They are only suitable for the treatment of lymphedema with low protein content. So-called diuretics are used, which remove water from the tissue. In the case of protein-rich oedemas, however, these would cause further oedemas or fibrosis (hardened tissue) by increasing the protein concentration.

Operations

There is the possibility of a lymphatic vessel graft into the affected limb. However, these have not proved successful in practice. Thermotherapy This naturopathic treatment is based on the effect of heat and compression. The curative effect has not yet been proven by a study. Tissue suction Another invasive treatment that is rarely used due to the high risk of injuring other tissue.

Early treatment has a good chance of success. In the case of oedema that has already been present for a longer period of time, the skin-related sequelae such as hardening often can no longer be reversed. Special care in skin care is essential in any case. The skin should be cared for carefully and intensively. Special care should be taken with nails, and those affected should not wear any clothing that cuts into the skin. If left untreated, however, lymphedema will always increase in size. The elasticity of the skin decreases and rough patches develop. In addition, the skin over lymphoedema is less sensitive to touch. The mobility of the limbs decreases. Rarely, a malignant change of the edema results, a so-called lymphangiosarcoma.

• Make sure you get enough exercise (without overworking yourself).
• Keep an eye on your weight, as excess weight promotes oedema.
• Do not wear constricting clothing or accessories (too tight underwear, stockings, watch bands).
• Blood pressure measurements, blood draws or injections should only be done on a healthy arm.
• Avoid extreme temperatures (heat, cold) and strong sunlight.
• Take good care of your skin and have any changes (e.g. redness, dandruff) treated at an early stage.