Lymphoedema is a long-term condition that causes swelling in parts of the body when the lymphatic system, a crucial network that helps fight infections and remove excess fluid, doesn’t work properly. This chronic swelling most commonly affects the arms and legs, though it can develop in other areas such as the chest, neck, or genitals. The condition cannot be cured, but with proper management and early treatment, its symptoms can be controlled and complications prevented.
What Is Lymphoedema?
Lymphoedema refers to the accumulation of protein-rich fluid in body tissues, resulting in swelling that can range from mild to severe. The condition develops when something disrupts the normal functioning of the lymphatic system, which is part of your body’s immune system and circulatory network. This system is responsible for collecting excess fluid, proteins, and toxins from cells and tissues and returning them to the bloodstream.[1][2]
The lymphatic system consists of lymph vessels, lymph nodes, and organs spread throughout the body. Lymph fluid contains white blood cells called lymphocytes, which attack viruses and bacteria when they enter the body. When lymph vessels or nodes become blocked or damaged, the lymph fluid has nowhere to drain and begins to accumulate in the surrounding tissue, causing swelling.[5]
Lymphoedema can affect any part of the body, but most frequently develops in the arms or legs. It may also occur in the breast, chest wall, abdomen, neck, genitals, face, and even inside the mouth or throat. The swelling can significantly impact a person’s ability to perform daily activities and often causes psychological distress due to visible changes in appearance.[1][3]
Types of Lymphoedema
There are two main types of lymphoedema: primary and secondary. Each type has different underlying causes and typically appears at different stages of life.
Primary Lymphoedema
Primary lymphoedema is rare and occurs due to inherited or congenital conditions that affect the development of the lymphatic system. These genetic mutations cause malformations in the lymphatic vessels, preventing them from working properly. Primary lymphoedema affects approximately 1 in 100,000 people in the United States.[2][5]
This type can be further divided into three categories based on when symptoms first appear. Congenital lymphoedema is present at birth or develops within the first two years of life. Some infants may be born with Milroy’s disease, an inherited form of the condition. Lymphoedema praecox, also called Meige disease, typically develops during puberty, pregnancy, or by age 35. Finally, lymphoedema tarda is a rare, late-onset form that begins after age 35, usually affecting only the legs.[2][5]
Secondary Lymphoedema
Secondary lymphoedema is far more common than primary lymphoedema. It occurs when the lymphatic system is damaged or blocked due to external factors such as surgery, radiation therapy, trauma, infection, inflammation, or cancer. This type of lymphoedema can also result from prolonged inactivity or lack of limb movement.[3][4]
Secondary lymphoedema is most frequently seen in people who have undergone cancer treatment, particularly breast cancer surgery involving lymph node removal. However, it can also affect individuals treated for other cancers, including vulval, penile, prostate, bowel, melanoma, head and neck cancers, and other cancers involving the lymphatic system. Not everyone who has cancer treatment develops lymphoedema, but the risk increases with certain procedures.[3][7]
Epidemiology
Lymphoedema is thought to affect more than 200,000 people in the United Kingdom alone. While primary lymphoedema is extremely rare, secondary lymphoedema is significantly more common. Globally, it is estimated that lymphoedema affects roughly 1 in 1,000 Americans, with worldwide estimates ranging from 140 million to 250 million people.[3][17]
Secondary lymphoedema often develops in people with cancers that involve the lymphatic system or who have had treatment affecting the lymph nodes. Breast cancer survivors face a particularly high risk, with estimates suggesting that between 15 and 40 percent of those treated for breast cancer will develop breast cancer-related lymphoedema at some point. For patients who undergo sentinel lymph node biopsy (removal of fewer than four lymph nodes) without radiation, the lifetime risk is approximately 6 percent. However, for those who have axillary lymph node dissection (removal of more than four lymph nodes), the risk increases to 15 to 25 percent.[6][7]
Lymphoedema can develop soon after cancer treatment or may not appear until months or even years later. The condition typically progresses slowly over time, though in some cases swelling can develop suddenly. Radiation therapy can also increase the risk of lymphoedema, with effects roughly equivalent to surgery, even if no surgical removal of lymph nodes occurred.[6][7]
Causes
Lymphoedema develops when something blocks or disrupts the flow of lymph fluid through the lymphatic system. The underlying causes differ depending on whether the lymphoedema is primary or secondary.
In primary lymphoedema, the root cause is a genetic mutation that affects how the lymphatic system develops. People with this form are born with abnormalities in their lymphatic vessels, though symptoms may not appear until later in life. These genetic conditions are inherited and passed down through families.[5]
Secondary lymphoedema has multiple potential causes. Cancer surgery is one of the most common, particularly procedures that involve removing lymph nodes to check whether cancer has spread. When lymph nodes are removed, the pathways that normally drain lymph fluid are disrupted, causing fluid to accumulate in nearby tissues. Radiation therapy used to treat cancer can cause scar tissue that blocks lymph vessels, preventing proper drainage.[4][7]
Tumors themselves can press against or block lymph nodes and vessels, restricting the flow of lymph fluid. Physical trauma or injury to the body can damage lymphatic structures. Infections, particularly a parasitic infection called filariasis, can also cause lymphoedema. Obesity is another contributing factor, as excess weight can interfere with lymphatic function. Inflammation of the limbs and prolonged periods of inactivity or lack of movement can further contribute to the development of secondary lymphoedema.[4][7]
Risk Factors
Several factors can increase a person’s likelihood of developing lymphoedema. Understanding these risk factors helps individuals and healthcare providers identify those who may need closer monitoring or preventive measures.
The most significant risk factor for secondary lymphoedema is cancer treatment. People who have had surgery that removes or damages lymph nodes are at elevated risk. The more lymph nodes removed, the higher the risk becomes. Radiation therapy to areas where lymph nodes are located also increases risk, as it can cause scarring and damage to lymphatic vessels.[6][7]
The type and location of cancer also influence risk. Individuals treated for breast cancer, particularly those who undergo axillary lymph node dissection, face substantial risk. People with cancers in the abdomen or genital area, including vulvar, vaginal, ovarian, endometrial, cervical, prostate, and colorectal cancers, may develop lymphoedema in the legs, genitals, or abdomen. Those treated for head and neck cancers may experience swelling in the face, neck, or under the chin. Melanoma and sarcoma can also lead to lymphoedema, especially after surgery or radiation.[7]
Having an infection in the area where lymph nodes were removed or damaged increases risk. Wounds that heal slowly after surgery can contribute to lymphoedema development. Advanced cancer that has spread to lymph nodes presents additional risk. Being overweight or having obesity interferes with lymphatic function and significantly raises the likelihood of developing the condition. Previous surgeries or radiation treatments in areas near lymph nodes also elevate risk.[7]
For primary lymphoedema, the main risk factor is having a family history of the condition, as it is inherited through genetic mutations. Children born to parents with certain genetic conditions affecting lymphatic development are at risk of developing primary lymphoedema at some point in their lives.[5]
Symptoms
The main symptom of lymphoedema is swelling in one or more parts of the body. This swelling may be subtle at first, making it difficult to notice in the early stages. Some people may not realize they have unusual swelling until the condition has progressed. In other cases, swelling can appear suddenly and become quite noticeable quickly.[1][2]
In the early stages, the swelling may be soft and easy to push in with a finger, leaving a temporary dent in the skin. This is called pitting edema. The swelling may worsen during the day and improve overnight when the affected limb is elevated. Without proper treatment, the swelling typically becomes more severe and persistent over time. The affected area may no longer pit when pressed, and the skin may feel harder and thicker.[3][4]
People with lymphoedema often notice that their affected arm or leg looks larger than the other one. Clothing, shoes, jewelry, and watches may feel tighter than usual or no longer fit properly. The swollen limb may feel heavy, full, or tight. Some individuals experience an aching sensation or discomfort in the affected area. Movement can become difficult, as joints in the swollen limb may feel stiff or have a reduced range of motion.[1][2][3]
Skin changes are common as lymphoedema progresses. The skin in the swollen area may appear red, puffy, or discolored. It can become thicker, leathery, or develop a texture like orange peel. Folds may develop in the skin, and in some cases, wart-like growths can appear. Fluid may occasionally leak through the skin. Some people experience a tingling sensation, itching, or burning feeling in the affected area.[1][3][4]
When lymphoedema affects the head and neck, symptoms may include swelling or a feeling of heaviness in the face, mouth, or throat. This can lead to problems with swallowing and speech. Lymphoedema can also develop inside the body, such as in the throat, where it may not be visible from the outside.[3][7]
Many people with lymphoedema experience psychological and emotional effects. The visible changes in appearance can cause feelings of self-consciousness, embarrassment, depression, anger, or anxiety. The condition may interfere with daily activities, work, and social interactions, significantly affecting quality of life.[2][5]
Prevention
While lymphoedema cannot always be prevented, there are steps individuals can take to reduce their risk or delay its onset, particularly after cancer treatment. Early intervention and lifestyle modifications can make a significant difference in managing or avoiding the condition.
Before undergoing surgery or radiation therapy for cancer, it is important to discuss with your doctor what measures can be taken to lower the chances of developing lymphoedema. Healthcare teams plan treatments to minimize damage to lymph nodes whenever possible. Techniques such as sentinel lymph node biopsy, which removes fewer lymph nodes than traditional methods, can reduce risk.[3][19]
Protecting the skin from injury is crucial, as even minor cuts, burns, insect bites, or scrapes can lead to infection in areas where lymphatic function is compromised. Keeping the skin clean, dry, and well-moisturized helps prevent cracking and reduces infection risk. Using sunscreen protects skin from burns, and wearing gloves while gardening or doing household chores prevents accidental cuts.[3][18]
Maintaining a healthy weight through balanced diet and regular exercise is one of the most effective preventive measures. Excess weight places additional strain on the lymphatic system and increases the risk of developing lymphoedema. Regular physical activity and movement help stimulate lymph flow and prevent fluid buildup. Exercise should be gentle and gradually increased, particularly for those at risk.[4][18]
Avoiding tight or restrictive clothing, jewelry, and accessories that could compress lymphatic vessels is important. Blood pressure measurements, injections, and blood draws should be performed on the arm that is not at risk whenever possible. Elevating the at-risk limb whenever possible, especially at night, can help prevent fluid accumulation.[18]
Monitoring for early signs of swelling allows for prompt treatment, which is essential for preventing progression. People who have had cancer treatment should regularly check their limbs for any changes in size, heaviness, or tightness. If any symptoms appear, contacting a doctor immediately can lead to earlier intervention and better outcomes.[7][19]
Pathophysiology
The pathophysiology of lymphoedema involves changes in the normal structure and function of the lymphatic system, leading to chronic accumulation of protein-rich fluid in body tissues. Understanding these changes helps explain why symptoms develop and progress over time.
The lymphatic system normally functions as a drainage network. Lymph capillaries in the skin and tissues collect excess fluid, proteins, cellular debris, bacteria, and other substances that need to be removed from tissues. This lymph fluid travels through progressively larger vessels, passing through lymph nodes where it is filtered and harmful substances are removed. Eventually, the cleaned lymph fluid returns to the bloodstream through connections to veins in the chest.[5][11]
In lymphoedema, this drainage process is disrupted. When lymph vessels are damaged, blocked, or absent due to developmental abnormalities, lymph fluid cannot flow properly. The capacity of the lymphatic system to transport fluid becomes overwhelmed by the amount of fluid that needs to be drained. As a result, protein-rich lymph fluid accumulates in the spaces between cells in the tissue, called the interstitium.[5][11]
The accumulation of protein-rich fluid triggers a series of changes in the affected tissue. The excess protein attracts more water into the tissue, increasing swelling. Over time, the persistent presence of lymph fluid causes inflammation in the surrounding tissue. This chronic inflammation leads to the formation of scar tissue, a process called fibrosis. The affected tissue becomes harder and thicker as fibrosis progresses.[11]
Fat tissue also accumulates in areas affected by lymphoedema, contributing to increased limb size. The combination of fluid accumulation, fibrosis, and fat deposition creates the characteristic appearance of advanced lymphoedema, where the limb or affected area is significantly enlarged and the skin becomes thick and difficult to pinch.[11]
The impaired lymphatic function also affects the immune system. Because lymph fluid contains infection-fighting white blood cells and the lymph nodes filter out harmful substances, damage to the lymphatic system compromises the body’s ability to fight infections in the affected area. This is why people with lymphoedema are much more vulnerable to bacterial skin infections such as cellulitis. These infections can further damage lymphatic vessels, creating a cycle where each infection worsens the lymphoedema.[2][3]
In primary lymphoedema, the pathophysiology begins with structural abnormalities in lymphatic vessels present from birth. These vessels may be too few in number, too narrow, or improperly formed, limiting their ability to drain lymph fluid effectively. In secondary lymphoedema, the pathophysiology involves acquired damage to previously normal lymphatic structures through surgery, radiation, trauma, or other external causes.[5]
Once lymphoedema develops and tissue changes occur, the condition becomes chronic and progressive. Early intervention is crucial because once significant fibrosis and tissue changes have occurred, they are difficult or impossible to reverse. This is why early diagnosis and treatment are so important for managing lymphoedema effectively.[5][11]