How Does a Person Get Lymphedema?

Primary lymphedema (also called hereditary lymphedema) is caused by hereditary or genetic abnormalities in the lymphatic system. Secondary lymphedema is caused by damage or trauma to the lymphatic system from cancer, cancer-related surgery, obesity, traumatic injury, radiation therapy, or parasitic infection.

Lymphedema is abnormal swelling in tissues just beneath the skin due to the accumulation of lymph fluid in the body.

The lymph system works to drain and carry fluids and cells from the body’s tissues to help fight infection. When the lymph system is not functioning properly, fluid cannot drain as it should, and swelling can occur. 

Lymphedema can be categorized as primary or secondary. 

Primary lymphedema (also called hereditary lymphedema) is an uncommon disorder caused by hereditary or genetic abnormalities that result in the lymphatic system not forming properly. 

Secondary lymphedema is more common. A person can get secondary lymphedema from damage or trauma to the lymphatic system. Damage to the lymph system can be caused by: 

• Cancer 
• Cancer-related surgery
  • May include surgery to remove lymph nodes or the vessels that carry lymph fluid
• Obesity
• Traumatic injury
• Radiation therapy
• Parasitic infection
  • This is the most common cause of damage to the lymphatic system worldwide, commonly in subtropical areas of Southeast Asia, India, Central America, and Africa
  • Leads to a form of lymphedema called filariasis

When the lymph system is damaged, such as from cancer, surgery, or radiation, lymphedema may not develop right away but can occur years later. 

Lymphedema may be triggered by: 

• Injury to the affected area 
• Wounds or skin punctures, such as insect bites or injections
• Skin infections on the affected area
• Excess load on a limb such as from carrying heavy bags or standing for long periods on a susceptible leg 
• Limb constriction from blood pressure cuffs, tight clothing, and jewelry 
• Air travel, due to changes in air pressure
• Excessive heat exposure

Symptoms of lymphedema may include:

• Swelling of arms, legs, or other body parts 
  • May develop quickly or it may progress slowly and worsen over several months
• Heavy feeling in arms or legs
• Tightness of the skin in the swollen area 
• Clothing fitting more snugly on the affected area
• Thick or leathery appearance of the skin over the affected area 
• Tingling sensation

Lymphedema is diagnosed with a physical examination. If an arm or leg is swollen, it is usually measured to compare it with the other arm or leg. If the swollen extremity is 2 cm (about 4/5 of an inch) larger than the other, it is generally considered lymphedema.

Other tests used to confirm the diagnosis of lymphedema or to determine the cause include: 

• Lymphoscintigraphy 
• Magnetic resonance imaging (MRI) 
• Computed tomography (CT) scans 

There is no cure for lymphedema. The goal of treatment is to relieve symptoms and reduce swelling or prevent it from getting worse. 

Lymphedema treatment may include: 

• Compression sleeves or stockings to help lymph fluid drain
• Exercise to help lymph fluid drain and to reduce swelling
• Weight loss in patients who are overweight or obese
• Manual lymph drainage 
  • A special type of massage therapy that works to help push lymph fluid out of the swollen parts of the body
• Pneumatic pump
• Cancer treatment in cases where a tumor is causing lymphedema
• Surgery in severe cases 
  • Liposuction
  • Lymphatico-venous anastomosis (lymphovenous bypass)
  • Vascularized lymph node transfer surgery (lympho-venous transplant)
  • Charles procedure (skin grafts)