Lymphedema: the Growing Awareness

Lymphedema is the swelling of a body part, most often an arm or a leg, caused by the abnormal accumulation of lymph fluid.

Although lymphedema has been observed for centuries, until recently little was understood about the disease. Only in the past 10 to 15 years have clinicians begun to seriously focus on its causes and treatment.

The lymphatic system, which is a part of the larger circulatory system, develops before birth. Fluids and protein, lipids, fat-soluble vitamins, and immune cells circulate within lymphatic vessels and nodes. If the normal flow of lymph fluid is blocked, the skin and tissues become edematous (swollen) and are at a much greater risk for infection.

Primary Lymphedema
Primary lymphedema can be congenital (present at birth) or develop after age 35. Some cases are familial (inherited) but primary lymphedema often occurs due to idiopathic (unknown) causes.

Primary lymphedema is more common in females and occurs more often in the lower extremities.

Types of Primary Lymphedema:

  • Congenital or Hereditary Lymphedema

    • Idiopathic Lymphedema
      Milroy’s Disease (presents at birth)

    • Lymphedema Praecox (present around 19-20)

    • Lymphedema Tarda (present after age 35)

  • Turner’s Disease

  • Parkes-Weber Syndrome

  • Klippel-Trenaunay Syndrome

   

Secondary Lymphedema
Secondary means that a disease occurs as a result of something else. Secondary lymphedema can be caused by injury, or by the scarring or removal of the lymphatic vessels as a result of radiation and/or surgery on or near the lymph nodes. Occasionally, secondary lymphedema is caused by trauma or by a chronic infection of the lymphatic system.

 

Types of Secondary Lymphedema:

  • Postsurgical Lymphedema

  • DES Daughters

  • Traumatic (as the result of injury)

  • Burns

  • RSD (Reflex Sympathetic Dystrophy)

  • AIDS

  • Nephritis

  • Postinflammatory Lymphedema

  • Radiotherapy

  • Liver Disease

  • Filariasis