Types of Lymphedema and Definitions

Lymphedema is the build-up of protein rich lymphatic fluid in the body due to damage to a part of the lymphatic system or a deficiency of the lymphatic system. Fluid or edema accumulates in one or more regions affected; the head/neck or more commonly a limb and its corresponding quadrant of the trunk. Lymphatic conditions are progressive and generally worsen if not treated properly.

Stage O Lymphedema: Edema is present in the tissue but not yet measurable or visible. Prompt treatment may prevent worsening of lymphedema.

Stage 1 Lymphedema: Edema is now visible and measurable in the affected region with soft pitting present due to a low protein content. Edema may fluctuate throughout the day and often decreases with elevation.

Stage 2 Lymphedema: Pitting is less prevalent as fibrosis or hardening/thickening of the tissue is present due to increased protein and fibrin present. The edema no longer improves with elevation and the size of the affected area increases due to increased fluid accumulation.

Stage 3 Lymphedema: Increased size and fibrosis are present with skin changes including dry/scaly skin (hyperkeratosis), weeping of lymphatic fluid (lymphorrhea), raised areas (papillomatosis), color changes (hyperpigmentation) as well as decreased functional mobility and increased risk of infection and wounds.

Primary Lymphedema is usually due to an anatomical anomaly and can be hereditary or of unknown cause. Often this occurs due to fewer lymph nodes present at birth in one or more of the major lymphatic chains. The symptoms can appear early or late in life; often after puberty, an injury or surgery.

Secondary Lymphedema occurs due to a known trauma; such as surgical lymph node removal, radiation, infection or trauma (burns, injuries, surgeries).

Lipedema is likely a hereditary or primary condition that involves edema in the adipose tissue which is symmetrical but disproportionately distributed, usually in the legs but sparing the feet. This condition affects up to 11% of women and usually becomes apparent around puberty or childbirth. Individuals with Lipedema usually present with varicose veins, lipomas, hemangiomas, hypermobility, easy bruising, tenderness along the shin, and family history of the condition. Lipedema can progress to Lipo-lymphedema which is asymmetrical and involves swelling of the foot/feet.

Cellulitis, an infection of the lymphatic fluid, is a major complication of lymphedema which can cause further damage to the lymphatic system and may progress to sepsis, a blood infection that is potentially fatal.