Stages of Lymphedema

John Fuller Beckwith3/13/2024

Benign lymphedema is typically slow in its progression, usually thought of in terms of fluid accumulation, but more significantly marked by unique changes in skin texture. The slow progression of lymphedema can be understood in distinct phases that may take months or years to be apparent. Malignant lymphedema is an exception in that it is marked by a rapid progression through the stages. It should be remembered that lymphedema is eminently treatable in all stages, even advanced Stage III, and very dramatic improvements can be achieved and maintained if the principles of good treatment are properly implemented and followed.

Stage 0 (Latency/At Risk)

In this stage there is no active lymphedema. However, the risk for developing lymphedema is ever present due to an injury or impairment of lymphatic transport. The normal capacity for lymph drainage has been reduced. In this stage there is an increased risk of infection, which can be well-managed with attention to daily skin and nail care.

Pre-Clinical Stage

In this stage the reduced capacity of lymph transport has been exceeded and at a microscopic level fluid is beginning to accumulate, yet there is no visible swelling in the body part of the body. This stage is highlighted by subjective feelings of changes in the limb or body part (such as achiness, fullness, soreness, heaviness) without outward signs of swelling.

Stage I (Spontaneously Reversible)

Swelling is visibly apparent and measurable. The swelling may be very mild and very limited yet conversely can also be pitting and present in much of the limb or body part. Stage I is marked by edema that will significantly reduce with elevation (or overnight) and may go away to the point of not returning for days or weeks (returning to a stage of latency), hence the edema, “spontaneously reverses.” Many people and clinicians think that the reduction means no treatment is needed, or perhaps they forget that the swelling even happened. However, proper intervention at this point can help prevent further progression of lymphedema. It is generally accepted that without proper intervention, all Stage I lymphedema will progress over time to Stage II.

Stage II (Spontaneously Irreversible)

Swelling is present all the time and though it may fluctuate, it never resolves completely, hence this stage is termed, “spontaneously irreversible,” (i.e., it does not reverse with elevation, though it can reverse with proper treatment). Since the swelling is always present, the process that causes lymphostatic fibrosis begins and progresses. Fibrosis is apparent in an increasing firmness in the tissues and it becomes gradually more difficult to cause pitting in the skin. The skin itself may harden and thicken in some areas, a process involving hyperkeratosis and fibrosis. During this stage, the edema may be persistent yet relatively mild, or can be more moderate, firm and heavy. Difficulty wearing clothes and challenges with some daily activities can be seen in this stage.

Stage III (Severe Lymphedema)

The edema has progressed such that the swelling is severe, or the associated skin changes such as hyperkeratosis and fibrosis are extensive and severe, or both. There can be obvious skin folds and deep creases, pitting becomes impossible or very difficult and the skin has changed significantly. More severe cases may see skin projections called papillomas and there may be areas of weeping fluid. It may be seen in the literature that this stage is termed, “elephantiasis,” since the changes to the skin or limb may bring to mind features of an elephant’s skin.