You've noticed swelling in a larger or smaller part of your body, but you're not sure what lymphoedema is? We'll explain it all to you.
What is lymphoedema?
Lymphoedema is a chronic and progressive condition characterised by an excessive accumulation of lymphatic fluid in the tissues, leading to localised swelling of the limb concerned (oedema).
This accumulation results from a deficiency in the lymphatic system, either due to congenital malformations and genetic mutations (primary lymphoedema), or following alteration or obstruction of the lymphatic pathways, for example following trauma (such as surgery), malignant disease, venous disease, infection, etc. (secondary lymphoedema)(1).
This chronic, progressive disease has a significant impact on the lives of sufferers, not least because it requires lifelong treatment. It can cause discomfort, difficulty getting around, a poor self-image, recurrent skin infections, etc. if it is not properly managed(1).
Lymphoedema is caused by an inability of the lymphatic system to drain lymph fluid correctly, leading to an accumulation of fluid in the interstitial tissues (oedema). There are two types of lymphoedema:
Primary lymphoedema: This is the consequence of congenital anomalies (developmental abnormalities) affecting the lymphatic system and the development of lymphatic vessels and lymph nodes(1,2). This type of lymphoedema is rare and affects more women (2/3 of cases)(3,10). Its true prevalence is unknown in France, but it is estimated to affect less than 1 in 2,000 people(10).
Secondary lymphoedema: This is more common than primary lymphoedema and is caused by alteration or obstruction of the lymphatic ducts, for example following trauma (such as surgery), malignant disease, venous disease, infection, obesity etc(1,2).
In developed countries, most cases of secondary lymphoedema are due to a malignant tumour or the treatment of a malignant tumour, and breast cancer is the cancer most often associated with secondary lymphoedema(10).
What are the symptoms of lymphoedema?
The main symptoms of lymphoedema include :
Swelling and heaviness Lymphedema is mainly characterised by localised swelling, often of the limbs (arms or legs), but can also affect the genitals, head, neck and trunk(2). This swelling (mild or severe) is associated with a feeling of heaviness. The swelling is usually unilateral (affecting only one side of the body) but may be bilateral(1,10).
Discomfort and pain Lymphoedema can be painful and/or uncomfortable, particularly when the swelling becomes more pronounced and complications arise. Pain is thus secondary to lymphoedema. This may be due to a feeling of tension in the skin, joint pain, inflammation, etc(1,2,3,10).
Stiffness and limitation of movement Swelling can lead to a loss of mobility in the affected area, particularly in severe cases. This can affect daily activities such as walking or lifting(1,10,8).
Skin changes As lymphoedema progresses, the skin may become thicker, firmer, and tissue fibrosis may appear. The skin in the lymphoedema area also becomes vulnerable to infections such as erysipelas(2,3,9).
These symptoms can develop gradually and must be managed quickly to limit the progression of lymphoedema.
How does lymphoedema develop?
Stage 0
This is the ‘latent’ or ‘subclinical’ stage. Lymphatic transport capacity is reduced, but oedema is not visibly apparent. Although the disease is asymptomatic in the majority of cases, some patients may feel a sensation of heaviness in the affected limb, tightness or stiffness. This stage may last for months or years.
Stage 1
The accumulation of lymphatic fluid is visible but fades as the limb is raised. The oedema is therefore ‘spontaneously reversible’. The oedema is painless and generally less than 20% larger than the healthy limb. Pressure with a finger on the swollen area may leave an imprint on the skin, known as the cup sign. At this stage, the skin and tissues are not permanently damaged.
Stage 2
The affected limb appears even more swollen. The swelling of the limb no longer diminishes as it becomes more elevated. The cup sign is clearly present. At this stage, there is a 20-40% increase in volume compared with the healthy limb. The skin and tissues begin to thicken as the volume of the limb increases.
Stage 3
This is the most severe stage of lymphoedema. The oedema is ‘permanent and irreversible’. Skin changes intensify, and the skin becomes drier, thicker and harder (fibrosis). The affected limb is generally more than 40% larger, with a significant loss of mobility. The cup sign is no longer present. Hyperpigmentation appears with an increase in skin folds, as well as fat deposits and warty growths.
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What are the consequences of lymphoedema?
Lymphoedema has significant consequences for patients' physical and psychological health.
Chronic swelling and functional impairment Lymphedema causes persistent and progressive swelling of the affected limbs. Combined with fibrosis of the tissues, this often leads to significant functional impairment. Lymph fluid accumulates in the tissues, causing a sensation of heaviness, stiffness and discomfort. Musculoskeletal disorders can also occur as a result of this swelling(1,10).
Tissue changes Over time, the retention of lymphatic fluid will lead to fibrosis (tissue thickening), inflammation and fatty tissue deposition, making treatment more difficult and limiting patients' mobility. This complication aggravates swelling and also contributes to functional discomfort(1,3).
Infectious complications Skin problems are common in lymphoedema patients. Swelling can produce deep skin folds where fungal and bacterial infections can develop(9). Lymphatic stasis is also associated with local immunosuppression in the area concerned, and local inflammatory changes, as well as an increased risk of infection: bacterial (mainly erysipelas), fungal infections (intertrigo), and even viral infections(2,10,12).
Psychological impact and quality of life Lymphoedema profoundly affects patients' quality of life, mobility and self-perception, and can lead to anxiety, depression and reduced self-esteem, contributing to an overall deterioration in emotional and social well-being(10,12).
What are the treatments and management of lymphoedema?
The main aims of lymphoedema management are to reduce and then stabilise the volume, prevent complications (such as erysipelas) and facilitate their management, encourage patient autonomy and improve quality of life(3).
Early and rapid treatment of lymphoedema should be initiated as soon as it is diagnosed, to reduce the risk of developing irreversible tissue changes, such as fibrosis and fat deposition(3).
The treatment, known as complete (or complex) decongestive therapy (CDT) is carried out in 2 distinct phases: the intensive phase (=reduction phase), then the maintenance phase.
Intensive/reduction phase: This aims to reduce the volume of lymphoedema by 30 to 40% and to teach the patient to self-manage and take preventive measures. This phase includes the round-the-clock use of compression bandages for 1 to 3 weeks, physical exercise with bandaging, manual lymphatic drainage and skin care(3,13).
Maintenance phase: the aim of this phase is to achieve long-term stabilisation of lymphoedema volume, following the reduction phase.
This phase mainly involves the use of high-pressure compression garments during the day (the highest pressure tolerated). Other components of the maintenance phase still include physical activity, less frequent bandaging than during the intensive phase, the possible use of manual lymphatic drainage, meticulous ongoing skin care and weight control(3,13).
Other conservative techniques may be used, although there is no solid evidence of their efficacy, such as the use of intermittent pneumatic compression (IPC), or balneotherapy. Therapeutic education is also necessary to help patients manage their condition(3,13).
Recurrent intensive phases may be necessary to optimise lymphoedema management.
Surgical interventions may be considered in advanced cases, but these practices remain controversial depending on their type due to insufficient evaluation of their indications, methodology and adverse effects. They include reconstruction surgery, skin resection surgery and liposuction. Surgical techniques aim to reduce tissue volume and improve lymphatic drainage(3,14).
FAQ about lymphoedema
Our medical team answers the questions you may have.
Several techniques can be used to drain lymphatic fluid and prevent it from accumulating in the tissues (as in the case of lymphoedema). Manual lymph drainage stimulates lymph circulation and helps to drain excess fluid towards the lymph nodes. Compression encourages the drainage of lymphatic fluid by applying constant pressure to the tissues, and appropriate physical exercise will stimulate the function of the muscles, which act like a ‘pump’ to help circulate lymph throughout the body. Intermittent pneumatic compression (IPC) devices (compression devices with inflatable chambers) are widely used, although there is no solid evidence for this
Lymph is a clear liquid that circulates in the lymphatic system, a network of vessels and lymph nodes distributed throughout most of the body. Lymph has three main functions.
Firstly, it drains excess fluid and proteins from the tissues into the blood. Blood capillaries naturally leak fluid and proteins into the interstitial spaces (the spaces between tissue cells). This excess fluid is absorbed by the lymphatic capillaries and then transported by the network of lymphatic vessels to the venous system, where it is reintroduced into the bloodstream, thus preventing accumulation. At the same time,
lymph contributes to immune protection by carrying immune cells; as it passes through the lymph nodes, it is filtered to detect and eliminate any pathogens. Finally, the lymphatic system is essential for absorbing fats and certain vitamins from the digestive system and transporting them to the bloodstream(2).
Primary lymphedema corresponds to a chronic increase in volume of a limb following the build-up of lymphatic fluid. It is related to a malformation of part or all of the lymphatic system.
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Ly, C., Kataru, R., & Mehrara, B. (2017). Inflammatory Manifestations of Lymphedema. International Journal of Molecular Sciences, 18. https://doi.org/10.3390/ijms18010171.
Cormier J., Damstra R., Brorson H., Suami H., Chang D., 2012, Surgical Intervention: A position document on surgery for lymphoedema. https://www.lympho.org/publications
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