Lymphedema classification

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Editors-in-Chief: Benoit Blondeau, M.D. [1]; C. Michael Gibson, M.S., M.D. [2]

Classification

Lymphedema may be classified as primary or secondary.

Staging and severity

Stages

Whether primary or secondary, lymphedema develops in a number of stages, from mild to severe. The stages were defined in the following manner by the Fifth WHO Expert Committee on Filariasis:

• Stage 0 (latent): The lymphatic vessels have sustained some damage which is not yet apparent. Transport capacity is still sufficient for the amount of lymph being removed. Lymphedema is not present.

• Stage 1 (spontaneously reversible): Tissue is still at the "pitting" stage -- when pressed by the fingertips, the affected area indents and holds the indentation. Usually upon waking in the morning, the limb or affected area is normal or almost normal in size.

• Stage 2 (spontaneously irreversible): The tissue now has a spongy consistency and is "non pitting" -- when pressed by the fingertips, the tissue bounces back without any indentation. Fibrosis found in Stage 2 Lymphedema marks the beginning of the hardening of the limbs and increasing size.

• Stage 3 (lymphostatic elephantiasis): At this stage, the swelling is irreversible and usually the limb(s) or affected area is very large. The tissue is hard (fibrotic) and unresponsive; some patients consider undergoing reconstructive surgery called "debulking". This remains controversial, however, since the risks may outweigh the benefits, and the further damage done to the lymphatic system may in fact make the lymphedema worse.

Grades

Lymphedema can also be categorized by its severity (usually referenced to a healthy extremity):

• Grade 1 (mild edema): Lymphedema involves the distal parts such as a forearm and hand or a lower leg and foot. The difference in circumference is less than 4 centimeters, and other tissue changes are not yet present.

• Grade 2 (moderate edema): Lymphedema involves an entire limb or corresponding quadrant of the trunk. Difference in circumference is more than 4 but less than 6 centimeters. Tissue changes, such as pitting, are apparent. The patient may experience erysipelas.

• Grade 3a (severe edema): Lymphedema is present in one limb and its associated trunk quadrant. The difference in circumference is greater than 6 centimeters. Significant skin alterations, such as cornification or keratosis, cysts and/or fistulae, are present. Additionally the patient may experience repeated attacks of erysipelas.

• Grade 3b (massive edema): The same symptoms as Stage 3a except that two or more extremities are affected.

• Grade 4 (gigantic edema): Also known as elephantiasis. In this stage of lymphedema, the affected extremities are huge due to almost complete blockage of the lymph channels. Elephantiasis may also affect the head and face.