Definition
Lymphedema is not a disease in itself, but a symptom of many other diseases. It is an underfunction of the lymphatic system. The lymph can no longer be completely removed and accumulates in the tissue.
Lymphedema is chronic at the affected site. The causes can be diseases, but also surgical interventions and malformations. As a preventative measure, those affected can wear compression stockings and should avoid anything that leads to lymph formation.
The causes
The causes of lymphedema can be used to divide lymphedema into a primary and a secondary form. The primary form is very rare and refers to a congenital form in which parts of the lymph vessels are not attached. This includes Milroy’s and Meige’s disease.
A complete absence of the lymphatic system is incompatible with life. The secondary form refers to all other causes of lymphedema, which have in common a mechanical, acquired outflow disorder. Tumors of the lymphatic system or surrounding tissue can be an obstacle to outflow.
Mechanical obstacles can also result from injuries or operations. Lymphedema is particularly common during tumor removal, as parts of the lymphatic system are often removed as well. A chronic underfunction of the veins can also result in a disturbed lymphatic drainage.
A special form of lymphedema is elephantiasis, a disease caused by parasites. Elephantiasis belongs to the tropical diseases and does not normally occur in Europe. Lymphedema can also occur in combination with other oedemas.
Irradiation as part of tumor therapy can damage lymph vessels. In most cases, this is not the sole cause of lymphedema. The tumor itself can already restrict the drainage pathways, so that radiation is an intensifying factor. Patients suffering from Hodgkin’s disease are more likely to suffer from lymphedema due to radiation.
The stages of lymphedema
Lymphedema can be divided into three to four stages, with stage zero showing no symptoms. The first stage is a completely reversible edema, which occurs during physical exertion and usually only appears in the afternoon or evening. The lymphedema is very rich in protein and still soft.
The affected areas can be pressed in with the fingers and the marks remain visible for a short time. The mobility of the joints may be restricted and affected persons report sensory disturbances and dullness. Fibrosclerotic – scarred and hardened – changes in the tissue are usually not yet present or only locally limited.
The swellings can disappear if they are elevated at night. Depending on the cause, certain body regions are affected. Early therapy can prevent the lymphedema from the first stage from progressing to the next stage.
Patients who discover these symptoms in themselves should consult a physician, as the cause should be sought. The initially reversible lymphedema can change into irreversible, chronic edema. The tissue changes fibrosclerotically (hardened and scarred connective tissue) and permanently.
Furthermore, a new formation of fatty tissue begins in the affected body region. The oedemas are no longer soft and can be pushed away, but become hard and firm. Swelling due to elevation can no longer be observed.
The restriction of movement of the joints increases and the sensitivity of the skin decreases. The skin is also brittle and cracked and can be painful. Treatment can only lead to a reduction in symptoms and cannot completely stop the lymphedema.
A high risk of relapse remains permanently. The damage to the connective tissue is not reversible. In the absence of treatment and skin care, the lymphedema can reach the stage of elephantiasis.
Elephantiasis describes a clinical picture characterized by massive swelling of body parts as a result of lymph congestion. The third stage of lymphedema is normally only reached if no therapy is initiated in the early stages. Therefore, the third stage is almost only found in developing countries and is mainly a consequence of the parasitic disease elephantiasis (this disease is caused by a massive swelling of body parts due to lymphostasis).Several liters of lymph fluid accumulate in the tissue, making normal movement impossible and disfiguring the affected body parts.
The skin also shows strong changes. On the affected body part, the skin is cracked and blisters, scars, fistulas and papillomatosis form. In extreme cases, the skin becomes dry and greyish and looks like elephant skin, which gives the stage its name.
Furthermore it comes to wound healing disorders. In the absence of treatment, a lymphangiosarcoma, a malignant tumor, may develop. In lymphedema, as in many diseases, the prognosis is strongly dependent on the time of diagnosis and the start of therapy. In later stages, only relief from late effects and pain can be achieved.