Primary and secondary lymphoedema have different causes.

Primary lymphoedema

Primary lymphoedema is caused by alterations (known as mutations) in genes responsible for the development of the lymphatic system. Mutated genes interfere with the development of the lymphatic system, resulting in the loss of its ability to drain fluid properly.

Due to the genetic nature of primary lymphoedema, the condition usually runs in families. However, not everyone born to a parent with primary lymphoedema will develop the condition.

Primary lymphoedema is more common in females.

Secondary lymphoedema

Secondary lymphoedema can have different causes. The most common are explained below.

Surgical treatment of cancer

Cancer spreads around the body via the lymphatic system and so part of the treatment can involve surgically removing the cancerous lymph nodes. The surgeon will try to ensure the rest of your lymphatic system is still able to operate normally, although this is not always possible.

There is a significant risk of lymphoedema occurring as a complication of treatment for:

• breast cancer 
• skin cancer
• gynaecological cancers, such as cervical cancer and vulval cancer
• genitourinary cancers, such as prostate cancer or penile cancer

Radiotherapy

Radiotherapy uses controlled doses of high-energy radiation to destroy cancerous tissue. However, it can also damage healthy tissue. If it is necessary to use radiotherapy to destroy cancerous cells present in your lymph nodes or vessels, there is a risk that the nodes and vessels will become permanently damaged. 

Infections

In some cases, an infection can cause lymphoedema.

Cellulitis is a bacterial skin infection that can cause lymphoedema, it is also sometimes a complication of the condition. A severe cellulitis infection can damage the tissue around lymph nodes or vessels, leading to scarring.

Another infectious cause of lymphoedema is a parasite infection called filariasis. This can be common in parts of the developing world, such as parts of India, but is not a risk in the UK.

Inflammation

Conditions that cause tissue to become inflamed (red and swollen) can also permanently damage the lymphatic system. Health conditions that can cause lymphoedema include:

• rheumatoid arthritis – which causes pain and swelling in the joints
• eczema – which causes the skin to become itchy, reddened, dry and cracked

Venous diseases 

Venous diseases, which affect the flow of blood through the veins, can cause lymphoedema in some people. The abnormal or damaged veins can result in excess blood or fluid building up in tissues, which causes tissue damage. This can affect the drainage of the lymphatic system.

Some venous diseases that can lead to lymphoedema include:

• deep vein thrombosis (DVT) – a blood clot in one of the deep veins in the body
• varicose veins (swollen and enlarged veins) – where poor drainage of blood in the veins causes higher vein pressure and more fluid passes into the tissues

Trauma and injury

In a small number of cases, lymphoedema can be caused by a general injury or trauma, such as a burn or extensive scarring.

Reduced movement

The movement of the surrounding muscles when you exercise or move helps lymph drainage. Therefore, reduced movement can lead to lymphoedema because the fluid in the lymphatic system does not get moved on, causing swelling.

People who are paralysed may be at risk of developing lymphoedema as a result of being unable to move. Someone who is chair-bound for a long time due to an illness may also be at risk of lymphoedema in their legs because they are not moving. 

Cellulitis is the most common complication of lymphoedema, but the condition can also cause psychological issues.

These complications are explained below.

Cellulitis

Many people with lymphoedema experience repeated episodes of cellulitis. Cellulitis is a bacterial infection of the deep layer of skin (dermis) and the layer of fat and soft tissues (the subcutaneous tissues) that lie underneath the skin.

Symptoms of cellulitis include:

• redness and a feeling of heat in the skin
• pain
• generally feeling unwell

Cellulitis can usually be successfully treated with antibiotics (medicines which treat bacterial infections).

Psychological effects

Living with a chronic (long-term) condition that affects your appearance can cause a great deal of distress. Lymphoedema can cause increased distress because many people will develop the condition after treatment for cancer.

If you have been feeling particularly down for the last few months and you no longer take pleasure in the things you usually enjoy, you may be experiencing a period of depression. If this is the case, talk to your GP or members of your lymphoedema treatment team because there are effective treatments for depression.

Talking to other people who also have lymphoedema can be reassuring and decrease feelings of isolation, stress and anxiety. The Lymphoedema Support Network offers information and advice and can put you in touch with a support group in your area.

The Complex Decongestive Therapy plan has proved successful in treating lymphoedema. If you persevere with treatment, your symptoms should become less noticeable and troublesome.

Read more information about treatment for lymphoedema.

If you are at risk of developing lymphoedema because you have had treatment for cancer, you may be offered an assessment for lymphoedema as part of your aftercare treatment plan. Otherwise, see your GP if you experience symptoms of swelling.

There are a number of specialist lymphoedema treatment centres in England. Your GP may refer you to one of these for further assessment.

In many cases, it is possible to make a diagnosis of lymphoedema by:

• asking about your symptoms and medical history
• measuring the distance around the affected limb to see if it is enlarged 

Other tests may be recommended to assess the extent and severity of your symptoms. These tests are explained below.

Tape measure

A tape measure is used at 4cm (1.6in) intervals up the leg to measure the limb circumference and then calculate limb volume.

Water displacement method

The water displacement method is based on the principle that you can calculate the volume of an object by measuring how much water it displaces (the amount of water that is pushed out of the way when an object is placed in water).

You will be asked to place the affected limb in a tank of water and the amount of water that is displaced will then be measured. This measurement can be used to calculate the volume of your limb.

Perometry

Perometry is a technique that uses infrared light to measure the volume of your limb. This process can accurately calculate how swollen your affected limb is.

Bioimpedance test

During a bioimpedance test, electrodes (small metallic discs) are placed on different parts of your body. The electrodes release a small and painless electric charge that is measured using a handheld device. Changes in the strength of the current can indicate the presence of fluid in your tissue.

Imaging tests

Imaging tests may also be used to help with the diagnosis. These include:

• a lymphoscintigraph – where you are injected with a radioactive dye that can be tracked using a special scanner, this shows how the dye moves through your lymphatic system and can check for any blockages
• a magnetic resonance imaging (MRI) scan – which uses a strong magnetic field and radio waves to produce detailed images of the inside of your body
• an ultrasound scan – which uses high-frequency sound waves to create an image of the inside of your body
• a computerised (axial) tomography (CT or CAT) scan – which uses X-rays and a computer to create detailed images of the veins or lymph nodes

These scans can be used to create a clearer picture of the affected tissue. They are also useful for ruling out another condition called lipoedema. Lipoedema is swelling of the limbs as a result of abnormal fat deposits.

Lymphoedema is a chronic (long-term) condition that causes swelling in the body’s tissue. This can lead to pain and a loss of mobility.

Lymphoedema usually affects the arms or legs, although in some cases there may be swelling in the:

• chest
• head
• genitals

Lymphoedema is caused by damage or disruption to the lymphatic system.

One function of the lymphatic system is to drain excess fluid from tissues. If the lymphatic system is disrupted or damaged, it can lose this ability and the excess fluid will cause the tissue to swell.

Read more about the symptoms of lymphoedema.

Types of lymphoedema

There are two main types of lymphoedema:

• Primary lymphoedema – which develops at birth or shortly after puberty and is caused by faulty genes.
• Secondary lymphoedema – caused by damage to the lymphatic system as a result of an infection, injury, trauma, or cancer.

Secondary lymphoedema often develops as a side effect of cancer treatment. Surgery is often necessary to remove lymph glands to prevent a cancer from spreading, this can damage the lymphatic system. 

Radiotherapy, where controlled doses of high-energy radiation are used to destroy cancer cells, can also damage the lymphatic system.

Read more about the causes of lymphoedema.

Who is affected?

It is estimated that 1 in 10,000 people are affected by primary lymphoedema.

Secondary lymphoedema is a relatively common condition, affecting an estimated 100,000 people in the UK.

Secondary lymphoedema occurs more frequently in women, possibly because it can sometimes be a side effect of breast cancer treatment.

Cancer Research UK estimates than one in five women may have lymphoedema in their arm after they have had radiotherapy or lymph nodes removed to treat breast cancer.

If you are at risk of developing lymphoedema due to cancer treatment, you may be offered an assessment as part of your aftercare. Read more about how lymphoedema is diagnosed.

Treating lymphoedema

There is no cure for lymphoedema, but it is possible to control the symptoms using a combination of different techniques, such as massage and compression garments.

There are also things you can do to help prevent the condition getting worse. This includes taking care of your skin to avoid infection and having a healthy diet and lifestyle.

If you have received treatment for cancer, these measures may also help to prevent lymphoedema.

Read more about how lymphoedema is treated and preventing lymphoedema.

Complications

People with lymphoedema are more vulnerable to infection. This is because infection-fighting white blood cells, called lymphocytes, which travel in the lymphatic system, are prevented from reaching the part of the body where they are needed.

A bacterial infection of the skin called cellulitis is one of the most commonly reported infections in people with lymphoedema.

Read more about complications of lymphoedema.

If you have received treatment for cancer, the following steps could help prevent lymphoedema. If you already have lymphoedema, this advice may stop it getting worse.

Skin care

The part of your body affected by lymphoedema is more vulnerable to infection because it does not receive a regular supply of infection-fighting cells (lymphocytes). Any cuts in your skin can allow bacteria to enter your body and may quickly develop into an infection.

You can avoid infections and help prevent lymphoedema by:

• not having injections or blood pressure readings in the affected area
• treating cuts and scratches immediately with an antiseptic cream so they do not become infected
• using insect repellents to prevent insect bites
• moisturising the skin daily to keep it supple (your GP may prescribe a suitable cream)
• avoiding very hot baths and showers (the heat from saunas, steam rooms and sun beds may also increase the swelling)
• using sun cream with a high sun protection factor (SPF) to prevent sunburn
• wearing gloves for gardening and household tasks to avoid cuts
• cutting your nails with nail clippers and using hand cream regularly
• using anti-fungal powder to prevent athlete’s foot (a condition that makes the skin red, flaky and itchy)
• seeing a chiropodist for foot and nail care (tell them you have lymphoedema)
• wearing shoes that fit correctly and provide support at the top of your feet
• using an electric razor if you need to shave to reduce the risk of cutting yourself
• not wearing tight-fitting clothes or jewellery

Diet and lifestyle

A healthy diet is also important in controlling lymphoedema. Being overweight puts increased strain on areas of the body that are already swollen. This can make it more difficult to fit compression garments over the affected limbs.

People who have a body mass index (BMI) of 30 or more are classed as obese and may be at increased risk of developing lymphoedema.

Use the healthy weight calculator to work out your BMI. Read more information about obesity.

Some people find that alcohol and heat cause an increase in swelling, while some have reported that air travel makes their symptoms worse.

The main symptom of lymphoedema is swelling in all or part of a limb, which can cause problems with wearing jewellery or watches, or fitting into clothes or shoes.

Other symptoms include:

• the affected limb feeling heavy and aching
• the affected limb losing some of its mobility
• pain in the affected limb
• painful joints, such as the elbow or knee, caused by swelling in the limb
• repeated skin infections in the affected limb
• the skin on the affected limb becoming hard and tight, and blisters or wart-like growths developing on the surface

The start of symptoms

In around 1 in 10 people, primary lymphoedema can cause symptoms that are present from birth. Most people with primary lymphoedema will experience symptoms after puberty.

Secondary lymphoedema, which is caused by an illness or injury, can appear at any time. For example, if you have had breast cancer treated with radiotherapy, you may need to take precautions against lymphoedema for the rest of your life.

Read more information and advice about preventing lymphoedema.

The recommended treatment for lymphoedema is a treatment plan called complex decongestive therapy (CDT). It is also known as decongestive lymphatic therapy (DLT).

CDT is not a cure for lymphoedema, but it can help control the symptoms of swelling and pain. Although CDT takes time and effort, it can be used to effectively control your lymphoedema.

Complex decongestive therapy (CDT)

There are four components to CDT treatment:

• Manual lymphatic drainage (MLD) is a specialised massage technique designed to stimulate the flow of fluid and reduce swelling.
• Multilayer lymphoedema bandaging (MLLB) uses bandages and compression garments to move fluid out of the affected limb.
• Remedial exercises designed to activate muscles in the limb to improve lymph drainage. 
• Skin care is required to prevent infection.

Each of these treatments is described in more detail below.

CDT begins with an intensive phase of therapy, during which you may receive daily treatment for up to six weeks. This is followed by the second phase, known as the maintenance phase. During the maintenance phase, you will be encouraged to take over your own care using simple lymph drainage techniques (MLD administered by either yourself or a carer) and exercise while wearing compression garments.

You will then have regular six-monthly review meetings to check how your treatment is progressing.

Manual lymphatic drainage (MLD)

During manual lymphatic drainage (MLD), your lymphoedema therapist will use special massage techniques to move fluid from the swollen areas into working lymph nodes where it can be drained.

It is important you receive MLD from a trained lymphoedema therapist and not a regular masseur. This is because the technique requires a detailed knowledge of the lymphatic system to be performed correctly.

Your lymphoedema therapist will also teach you a range of massage techniques you can use during the maintenance phase.

MLD may not be suitable if you have certain health conditions. For example:

• current or suspected deep vein thrombosis (DVT)
• a blood clot in one of the deep veins in the body
• swelling in your legs due to heart failure
• cancer in the affected area

Multilayer lymphoedema bandaging (MLLB)

Unlike the blood circulation system, there is no central pump, such as the heart, to move fluid around the lymphatic system. Instead, the lymphatic system uses the massaging effect of the muscles surrounding the lymph vessels and nodes to move the fluid.

The aim of multilayer lymphoedema bandaging (MLLB) is to support the muscles during exercise and encourage them to move fluid out of the affected limb. MLLB is often used after a session of MLD to prevent fluid accumulating in the limb again.

You will be taught how to correctly apply your own bandages and compression garments so you can continue to use MLLB during the maintenance period.

Remedial exercises

Remedial exercises are designed to strengthen muscles involved in lymph drainage. You will be given an exercise plan tailored to your requirements and ability.

Skin care

Taking good care of your skin is important because it will reduce your risk of developing an infection, such as cellulitis. You should be given a daily routine to follow, where you are required to meticulously clean your skin and check for any cuts, abrasions or signs of infection.

Read information about preventing lymphoedema for more advice about skin care.

Surgery

In some cases, a surgical procedure using liposuction (where a tube is used to suck fat out of tissue) can be used to treat lymphoedema.

Liposuction is used to remove excess fat from the affected limb to help reduce swelling. Once the surgery is complete, you will have to wear a compression garment on the affected limb for at least a year.

The National Institute for Health and Clinical Excellence (NICE) has announced that liposuction for chronic (long-term) lymphoedema is acceptable in terms of clinical safety. However, NICE states there is currently no evidence about its long-term effectiveness or whether there are risks of long-term complications.

Access to liposuction for lymphoedema may be limited depending on what is available from your local primary care trust.