Lymphedema: The Basics
What is lymphedema?
Lymphedema is a chronic swelling that happens when lymph fluid is not moving well and protein-rich fluid accumulates in the tissues.
The lymph system consists of lymph nodes and vessels. The lymph vessels run parallel to the blood vessels. The lymph system helps to manage fluid volume and fight disease. Lymph nodes and vessels drain excess fluid from the entire body and return it to the blood near the heart. Lymph nodes filter viruses, dying cells, foreign matter, and bacteria to fight infection. The lymph system also recycles proteins through the body. Lymphedema develops slowly unless there is trauma or infection to the area of the body where your lymph nodes and vessels are not working.
Types of Lymphedema
In some cases, people inherit or are born with a poorly working lymph system. This is called primary lymphedema. Lymphedema can also occur as a result of damage, such as trauma, surgery or radiation for cancer treatment, to the lymph nodes or lymph vessels. Damaged leg veins or a history of blood clots can cause a back up of blood and fluid in the small vessels of the body and progress to the lymph system. These are all causes of secondary lymphedema.
Your risk for developing lymphedema increases with the greater the number of lymph nodes removed, receiving radiation therapy, being overweight, having active cancer, or having an infection/injury to the body part at risk for swelling. Not all swelling in the body is lymphedema. Be sure to discuss swelling anywhere in your body with your physician/nurse or therapist, even if the swelling comes and goes. For more information on lymphedema risk, see the article on risk reduction for lymphedema.
What are some signs of lymphedema development?
Signs of lymphedema development include slow progression of swelling, which typically begins at the far end of your limb (hands and feet) and then moves up the arm or leg. Skin can be pitting (when a finger is pressed into the area the indentation remains). In stage I, this swelling may reduce with elevation. In stage II and III lymphedema, there is progressive hardening of the tissues in the affected area. With increased protein-rich swelling, there is a risk for bacterial growth and infection.
What skin changes are seen with lymphedema?
Skin changes may occur with the development of lymphedema. Local infections, often called "cellulitis," may cause the limb to become red, hot, painful and swollen. This infection can spread quickly, so early diagnosis and treatment is the key to keeping the infection and swelling under control.
Lymphedema can be pitting or non-pitting edema (swelling). Pitting edema is present when a mark is left in the skin by applying pressure from a finger, hand or object. This is often seen with a recent accumulation of fluid and protein.
Hyperkeratosis and papillomas may also be seen in someone with lymphedema. Hyperkeratosis is an overgrowth of the skin like a very thick callus. Papillomas are raised wart-like growths that can appear on the skin of people with longstanding lymphedema.
How is lymphedema diagnosed?
Your medical history is the key to the diagnosis of lymphedema. Your provider will look at your history from cancer diagnosis to treatment along with any past surgeries or medical history. These factors will be used to help determine your risk for lymphedema
Providers will use clinical and diagnostic tools to determine a specific diagnosis of lymphedema. Some of these tests are also used to rule out other causes of swelling.
- Look and feel of the skin.
- Stemmer sign is a sign that indicates a hardening or loss of flexibility of the folds of skin on the top of the toes and fingers. The skin cannot be pinched and lifted or is difficult to lift. A negative Stemmer sign does NOT indicate that there is no lymphedema.
- Swelling in the limb. Swelling can be determined by a few methods – but most providers will take measurements around your arm or leg at standard spots along both limbs. For example, patients with arm lymphedema may be measured at the knuckles, wrist, elbow and upper arm. They will compare both sides to determine if one is larger than the other.
- Diagnostic tests such as Doppler ultrasound, lymphoscintigraphy, CT, and MRI are examples of some of the tests that may be used to determine if lymphedema is present. These tests are also used to rule out local infection (phlebitis) or blood clot (DVT) as the cause of the swelling. X-rays, bone scans, or blood work may be done to rule out a recurrence of cancer.
When these tests are negative for other causes, the patient history and physical examination are usually sufficient to make the diagnosis of lymphedema
Specific tests of the lymphatic system are usually reserved for cases where the diagnosis is unclear or if more specific information about the lymphatic structure is necessary. Diagnostic tests may include lymphoscintigraphy, ultrasound, CT or MRI. Lymphoscintigraphy is a nuclear medicine test where an agent is injected into the lymphatic system. This fluid is then followed as it is carried through the body and is used to determine the structure and function of the lymphatics. Ultrasound can visualize enlarged lymph nodes. Computerized tomography (CT) scans are useful to look for soft tissue growths in the lymph nodes or to determine metastatic disease.
What are the stages of lymphedema?
There are 4 stages of lymphedema based on how long the fluid has been in the body part and what type of changes are seen as a result of the fluid.
In the pre-stage, also called the latency stage, the body is starting to have trouble moving the lymphatic fluid in the damaged area. You may start to have symptoms like clothing feeling too tight or pain in the area. The lymphedema is treatable and reversible at this stage.
In Stage I, there is very little swelling. The swelling that does exist may come and go. At this stage, the lymphedema is said to be reversible. There may be times when there is NO swelling.
In Stage II, there is considerably more swelling, because the fluid is in the tissues. In this stage, there is obvious swelling of the limb. The skin may feel tighter and may be shiny. The tissue may also feel thick and firm to the touch. The tissue may form pits when pressed. These changes are caused by scar tissue, or fibrosis, beginning to form. The fluid CAN be moved out of the tissue, but it won't happen without help from the outside.
Stage III lymphedema is a severe and prolonged accumulation of edema or swelling in the body part. The skin can become hardened with a leathery feel. The color of the skin can become darker with a brownish tint. There may also be papillomas of the skin, which are small bumps that form on the skin and may leak fluid. This stage is termed Lymphostatic elephantiasis. It is not possible for the body to clear the fluid on its own. Even with intense therapy, this fluid is difficult to remove. However, since the skin is such a dynamic organ, tissue changes and softening may be seen with treatment. Learn more about lymphedema treatment.
Resources for More Information
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