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It is important as well to be aware that when you have lymphedema, even in one limb there is always the possibility of another limb being affected at some later time. This “inactive” period referred to as the latency stage. It is associated with hereditary forms of lymphedema.
Lymphatic transport capacity is reduced No visible/palpable edema Subjective complaints are possible
(Spontaneously Irreversible Lymphedema) Accumulation of protein rich edema fluid Pitting becomes progressively more difficult Connective tissue proliferation (fibrosis)
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.
Stage 1 (mild) - Upon waking in the morning the limb or affected area is almost a normal size. The tissue is still in a “pitting stage” ( when pressed by a finger the area indents and holds the indentation).
Stage 2 (moderate) - The tissue is “non-pitting” (when pressed by a finger the tissue bounces back without leaving an indentation). The tissue has a spongy consistency.
Stage 3 (severe) - The tissue at this stage is hard (fibrotic) and will be only slightly responsive to the touch. The swelling is almost irreversible and the limb is very large and swollen. Infections are possible at any stage of lymphedema but occurrence becomes greater as stages progress. A swollen limb, left untreated, becomes hard (fibrotic) and full of lymph fluid which is high in protein and a perfect medium for bacteria and infections.
Discussion Stage Four:
There have been some voices raised about a Stage Four classification for lymphedema. From my personal experiences, I concur. Both my legs are classified as Stage Three, yet the difference between the two is very dramatic.
Stage Four Classification
The limb is so densely fibrotic that it is not possible to make any indentation when pressed. It becomes impossible for ultrasound testing to pick even the blood pulse. The skin becomes brittle and even the slight of bumps causes a serious, extensively weeping wound. Because of the hardness of the tissue, it has become a total septic foci for bacteria and constant cellulitis and systemic infections become the norm. The only treatment for cellulitis is an extended period of IV antibiotics.
There is no treatment option for Stage Four. The complete focus is on management, containment of infections, prevention if at all possible of amputation.
AUTHOR: Salvador Nieto, M.D, FICA.
SALVADOR NIETO FOUNDATION “for the development of lymphedematology”. Av. Santa Fe 2679, 2º “D”; (1425) Buenos Aires. Argentina. PAPER PRESENTED IN THE 19º INTERNATIONAL CONGRESS OF LYMPHOLOGY - SEPTEMBER 4-6, 2003 - FREIBURG, GERMANY.
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Updated September 13, 2007