Primary vs. Secondary Lymphedema: Symptoms, Causes and Treatment


Uncomfortable and even painful swelling can occur in the body for many reasons, including lymphedema. Primary lymphedema is a relatively rare condition, affecting 1 in 100,000 people, while secondary lymphedema is more common. One in 1,000 people in the U.S. will experience secondary lymphedema. 

How does this disease develop? What are the risk factors? The answers to these questions are different depending on the type of lymphedema — primary or secondary. Learn about both types of the disease below and how to manage the condition. 

What Is Lymphedema?

Lymphedema is a chronic condition that causes swelling in the body. The swelling most commonly occurs in the arms and legs, but it can happen anywhere in the body, including the hands, chest, neck, face and genitals. A collection of excess lymph fluid causes swelling.

Lymph fluid is a vital part of the lymphatic system, which also includes lymph nodes, lymphatic vessels, adenoids, the tonsils, the thymus and the spleen. The lymphatic system moves lymph fluid, which contains white blood cells, throughout the body. This process helps remove toxins and waste from the body, making the lymphatic system part of our immune system.

Lymphedema occurs when lymph accumulates and the lymphatic system is unable to drain it. The lymph nodes may have been damaged or blocked. Without draining, this high-protein fluid builds up and causes the swelling associated with lymphedema.

Certain conditions can increase the risk of developing lymphedema. Obesity and older age are considered risk factors for lymphedema. Additionally, certain types of arthritis are associated with a higher risk of lymphedema. 

Symptoms of Lymphedema 

Lymphedema is most easily recognized by visible swelling. But, swelling, particularly in the early stages of the condition, may be difficult to spot. If the arm changes even just two centimeters in diameter, this could suggest the development of lymphedema. If you know you are at risk of developing lymphedema, take note if an item of clothing that previously fit well feels tight on your arms or legs. 

You may experience other symptoms of this condition before you can see swelling. You can experience swelling, along with other symptoms, in varying levels of severity throughout your entire experience with the condition. 

In addition to swelling, symptoms of lymphedema include:

  • Tingling: A tingling sensation in the affected area of the body could indicate lymphedema. You may feel this in the arms, underarms, hands, breasts or chest. This sensation can sometimes be described as "pins and needles" or as a sensation of increased warmth. People with lymphedema may also experience shooting pains in different areas of the body. 
  • Numbness: You may experience a feeling of numbness in the affected areas of your body, whether it is in the limbs or elsewhere. The feeling may come and go. 
  • Achiness: Lymphedema can cause flu-like symptoms, such as body aches. You may also experience feelings of tenderness in the affected areas and develop a fever. 
  • Feelings of fullness and tightness: The swelling caused by lymphedema may be accompanied by feelings of fullness or tightness. You might feel like you have less flexibility in your joints of the affected limbs. 
  • Changes in the skin: Lymphedema can also affect the skin. You may notice areas of the skin that are red or have a rash. Hardening, itching and pain on the skin are also common symptoms. 

Your symptoms can help your doctor make a formal lymphedema diagnosis, but they may order tests to understand the cause of the condition. Imaging tests like an MRI scan, CT scan and Doppler ultrasound can provide a visual of the lymphatic system and pinpoint any blockages responsible for the fluid buildup. 

Causes of Lymphedema 

Various factors, including genetic conditions, infection and cancer treatment, can cause lymphedema. Primary and secondary lymphedema have varying causes. So what is the difference between primary and secondary lymphedema?

Primary Lymphedema

Primary lymphedema occurs because of a genetic condition. Aplasia, a birth anomaly where organs or limbs do not develop, can affect the lymphatic system. In this case, parts of the system, such as lymph nodes, never developed in the body. Hypoplasia, where parts of the lymphatic system are underdeveloped, and hyperplasia, where lymph collectors are larger than normal, can also cause lymphedema. 

Aplasia, hypoplasia and hyperplasia can affect many different parts of the body and can be related to a number of other conditions. These terms do not necessarily mean someone has or will have lymphedema. The three main types of primary lymphedema are:

  • Congenital lymphedema: Congenital lymphedema, also known as Milroy's disease, is present at birth due to genetic factors. Congenital lymphedema is also associated with other genetic conditions, including Noonan syndrome and Turner syndrome. 
  • Lymphedema praecox: Lymphedema praecox is another congenital condition, but the symptoms typically do not become apparent until puberty or later. This condition is also referred to as Meige disease, and it is the most common type of primary lymphedema. Swelling typically begins at the feet and progresses upward. 
  • Lymphedema tarda: Lymphedema tarda, or late-onset lymphedema, is the third type of primary lymphedema. As the name suggests, this condition develops later in life. People with lymphedema tarda will not experience symptoms until after age 35. 

Secondary Lymphedema 

Secondary lymphedema is acquired, meaning another disease or outside factor causes it. Some of the potential causes of secondary lymphedema include: 

  • Cancer: Cancer can play a role in disrupting the lymphatic system and causing lymphedema. When a tumor grows, it can put pressure on lymph vessels, making it more difficult for lymph fluid to flow through them. Cancer cells can also block the flow of lymph. Breast cancer is the most common type of cancer related to secondary lymphedema. 
  • Cancer treatment: Cancer treatment can also cause secondary lymphedema. In some cases, cancer patients need to have the lymph nodes in their underarms removed to prevent the spread of cancer. Lymph node removal is associated with an increased risk of developing secondary lymphedema. Radiation therapy, particularly when directed at the chest and underarms, can also increase the risk. Surgery on the chest, such as surgery to address breast cancer, can also lead to secondary lymphedema. Scar formation following surgery can also affect the lymphatic system. 
  • Infection: Infection following surgery could increase the risk of secondary lymphedema. Infection at the incision site can cause inflammation and stress the lymphatic system as it tries to handle increased blood and fluid flow. 
  • Trauma: The lymphatic system rests less than half an inch beneath the surface of the skin. Traumatic injury, such as the crushing of a limb or severe burns, can damage the lymphatic system and increase the risk of secondary lymphedema. 
  • Parasites: Lymphatic filariasis is a type of disease caused by a parasite. The parasite, Wuchereria bancrofti, is transmitted via mosquito bite. Once inside the body, the worms live in the lymph system. This type of secondary lymphedema is considered a neglected tropical disease. 
  • Other health conditions: Conditions that affect other organs in the body can play a role in causing secondary lymphedema. Kidney failure, for example, affects the body's ability to remove excess fluid. If fluid builds up in the body, it can stress the lymphatic system and increase the chances of lymphedema. Conditions affecting the heart and vascular system can also decrease the body's ability to move fluid throughout the body, leading to stress on the lymphatic system. 

Can Lymphedema Be Cured?

Lymphedema cannot be cured at this time. It is possible to reverse the effects of mild lymphedema, or stage 1, but the risk for the return or worsening of symptoms is still possible. Right now, treatment focuses on managing symptoms to improve quality of life. 

Early detection is key in treating lymphedema. If this condition is left untreated, the severe swelling it causes can lead to permanent changes to the body's tissue. If you know you are at risk for developing lymphedema, there are some steps you can take to watch for symptoms. You can engage in self-lymphatic massage to help promote proper drainage, for instance. 

Following cancer treatment and surgery, you can take steps to help prevent secondary lymphedema, including:

  • Protecting your limbs: Take extra steps to protect the arms or legs targeted by radiation or affected by surgery. Try to avoid getting small cuts or scrapes on those limbs, and avoid wearing tight clothing that could constrict those limbs. 
  • Skincare: A thorough skincare routine can help you recognize any subtle changes to your skin, and keeping the skin clean can reduce the risk of infection. 
  • Elevation: Elevate your affected limbs if possible. For example, you can rest your arm above the level of your heart while you are sitting and relaxing in the evening. 

You can also watch for any of the symptoms associated with the condition, even mild swelling, and talk to your doctor immediately. Monitoring and early detection can help prevent lymphedema from becoming more serious. Ask your doctor if they have experience treating lymphedema or search for a provider who does. Lymphedema can sometimes be difficult to diagnose, and not all providers specialize in treating this condition. 

The symptoms of lymphedema can come and go. Some people need continuous treatment, while others may only require treatment when symptoms flare up. 

Lymphedema Treatment and Management

Although primary and secondary lymphedema are chronic conditions, there are several approaches to treating it and managing symptoms, including:

  • Compression: Compression is one of the frontline treatments for managing the swelling caused by lymphedema. Lymphedema therapists can teach you how to wrap bandages around the affected area of the body to help move the excess fluid. Treatment may also involve wearing compression garments. You may use the garments by themselves or attached to a device that can apply additional pressure to promote movement of the fluid. 
  • Massage: Manual lymphatic drainage is a massage technique that helps move the fluid from the affected area of the body to other body parts with working lymph vessels. The massage is a gentle treatment your therapist can perform. Your therapist can also teach you how to do the technique at home. 
  • Exercise: Gentle forms of exercise can help promote fluid drainage in the affected areas. Your therapist can teach you how to do these exercises on your own. 
  • Complete decongestive therapy (CDT): CDT is an integrative approach to lymphedema treatment. It combines manual lymphatic drainage, compression therapy, different types of exercise and skincare. The first phase of this treatment approach focuses on decongestion, while the second is targeted at maintenance. Those with a history of blood clots, heart failure, high blood pressure or diabetes might not be recommended CDT. 
  • Surgery: Surgery may be suggested in more severe cases of lymphedema. Lymphatic bypass involves connecting lymphatic vessels to direct fluid away from obstructions and encourage proper draining. Healthy, functioning lymph nodes can also be transferred from other parts of the body to the affected areas. 

Post Acute Medical's Lymphedema Management Program

Whether you suffer from primary or secondary lymphedema, we can help. Post Acute Medical's Lymphedema Management Program focuses on addressing your symptoms to improve quality of life. Here is what you can expect from a treatment plan developed through our program:

  • Drainage: Our providers are trained in manual massage techniques to promote the drainage of excess fluid causing swelling. 
  • Compression therapy: Compression therapy involves wrapping the affected limb to direct the flow of fluid away from that part of the body. Our providers can show you the proper wrapping technique. 
  • Exercise: Regular exercise can help to improve lymph drainage. Our providers will work with you to establish an exercise regimen to help you stay fit and improve your range of motion. 
  • Education: Lymphedema is a chronic condition, which means you need to find the treatment that works best for you. Our team provides education about your different options to help you learn about the approaches and lifestyle changes that work for you. 

We also work closely with you to provide support for the psychological impact of lymphedema. The effects on your physical appearance and the pain caused by the disease can be significant. We help you to develop coping skills and improve your self-esteem. 

Find a Post Acute Medical Location Near You

Lymphedema is a chronic condition that can have a serious impact on your quality of life. Treatment can help you to manage the condition and alleviate your symptoms. Post Acute Medical has several hospitals specializing in this condition, including:

  • PAM Rehabilitation Hospital of Allen
  • PAM Rehabilitation Hospital of Clear Lake
  • PAM Rehabilitation Hospital of Round Rock
  • PAM Rehabilitation Hospital of Victoria
  • PAM Specialty and Rehabilitation Hospital of New Braunfels
  • PAM Specialty Hospital of Covington
  • PAM Specialty Hospital of Lufkin 
  • PAM Specialty Hospital of Hammond
  • PAM Specialty Hospital of San Antonio 
  • PAM Specialty Hospital of Tulsa
  • PAM Specialty Hospital of Wilkes-Barre
  • Rehabilitation Hospital of Overland Park
  • Warm Springs Rehabilitation Hospital of Thousand Oaks

Find a location near you to start treating your condition and receive the support you need to improve your quality of life.