Lymphedema: symptoms and causes
Do you have persistent asymmetrical swelling in one or more of your limbs? Is the affected arm or leg free of pain, but you have a feeling of tension in it? Do other family members suffer from swollen limbs? Have you needed a cancer treatment or undergone an operation where lymphnodes had to be removed? Did you have inflammation in the affected arm or leg beforehand?
If yes, then your lymphatic system is potentially damaged and you have developed lymphedema.
What is lymphedema?
Lymphedema is a chronic inflammatory tissue disorder. It occurs as a consequence of hereditary or acquired damage of the lymphatic drainage system. In healthy patients, there is a balance between the lymph that forms and its removal. Lymphedema leads to an imbalance – there is more lymph present than can be transported away. Local accumulations of fluid and tissue swelling develop. The longer this state exists, the more damage occurs in the tissues as a consequence.
Lymphedema is a protein-rich edema. It arises locally and usually affects one arm or leg. Less often, it is also found in other body parts, such as the face, trunk or genital area. The individual extent can vary.
What forms are recognized?
Primary lymphedema
Primary lymphedema occurs without external factors. This concerns a congenital disease owing to an incorrectly or incompletely formed lymphatic network. The initial manifestation usually occurs in the time from puberty to early adulthood, but may also be present from birth or in early childhood. The first appearance of the disease is favoured by events connected with a high lymph load, that is, with an increased formation of lymph. This includes pregnancy, trauma to the arm or leg or an overload (e.g. a job requiring standing, excessive physical exertion).
Secondary lymphedema
Secondary lymphedema results from external damage to the lymphatic system (lymph vessels / lymphnodes) through injuries, inflammation (e.g. after insect bites), severe wounds or burns. Severe obesity, operations or irradiation, but also a tumour that presses on the lymphnode, can cause lymphedema.
Recognizable symptoms of lymphedema
Lymphedema of the hands and feet is characterised by a positive Stemmer sign (image, right): if the skin folds above the second and third toes or finger is prevalent, thickened and difficult or impossible to raise, the Stemmer sign is positive.
Further symptoms of lymphedema depend on the localisation and extent:
- Box toes
- Asymmetrical swelling
- Deepened natural skin folds, especially at the base joints
- Swelling on the back of the foot and hand
- Very smooth, firm skin in the swollen areas
- Painless, pulling sensation
- Repeated skin infections
- Hardening of the skin, connective and fatty tissue in advanced stages
- Lymph cysts with lymph leakage from the tissue to the outside
- Chronic wounds
Indentable edema
The stages of lymphedema
Lymphedema can be subdivided into four stages. Each stage has a different effect on the affected person's body. They are classified according to the severity and range from lymphedema stage 0 (clinically not identifiable or only with difficulty) up to lymphedema stage 3, which can be accompanied by severe symptoms and physical restrictions.
Stage 0
No visible swelling despite delayed lymph transport and subtle changes to the tissue fluid. It can take months to years until an edema is visible as such. This stage can only be shown with a special radiological examination (called lymphoscintigraphy).
Stage 1
In stage 1, an indentable swelling is visible temporarily; however, this recedes without further measures when the affected limb is raised.
Stage 2
The swelling is permanent, raising the limb is insufficient for a complete reduction and there are the first signs of tissue hardening.
Stage 3
Skin changes arise in addition to the persistent and usually progressive swelling, e.g. blisters, fistulae or wart-like changes. Skin infections and erysepilas occur as complications of the untreated or poorly compensated lymphedema.
Mixed forms of lymphedema: phlebo-lymphedema
Phlebödeme entstehen durch Venenerkrankungen wie z. B. Beinvenenthrombosen oder ein Krampfaderleiden. A backlog in the venous system leads to more fluid in the intercellular space. Phlebedema occurs if this additional fluid cannot be sufficiently drained by the lymphatic system. Over time, the venous disease can also lead to damage to the lymphatic vessel system. The edema then becomes a mixed form described as phlebo-lymphedema.
Experts also employ lymphedema therapy in this case, use skin care, lymph drainage and compression therapy and recommend a specific movement program.