It is a chronic, inflammatory disease that is defined as the accumulation of high molecular weight proteins and other elements (water, salts, electrolytes, hyaluronic acid, etc.) in the interstitial space, as a result of a dynamic and / or mechanical alteration of the lymphatic system. , causing a progressive and evolutionary increase in the limb or body region with decreased functional and immune capacity, weight gain, and morphological changes (Definition according to the 3º Latin American Consensus for the Treatment of Lymphedema).
The International Society of Lymphology (ISL) defines it as an abnormal accumulation of tissue proteins, edema, chronic inflammation, and fibrosis.
It is a dysfunction in the Lymphatic System that can be congenital (hereditary) or ideopathic (appear without apparent cause).
It usually affects the lower limbs and is more common in women. It is cataloged within the Rare or Infrequent Diseases.
There are three clinical forms of Primary Lymphedema depending on the age of onset:
● Congenital lymphedema: It manifests from birth (between 0-2 years). Incidence of 1/6000. In case it is hereditary, it is recognized as Milroy's Disease
● Early lymphedema: It manifests from 2 to 35 years, with a peak of onset at 17-18 years and more often in females (due to hormonal changes). In case it is hereditary, it is recognized as Meige's Disease
● Late Lymphedema. It manifests after 35 years. It has a peak of appearance at 50 years old.
There are numerous causes for this type of lymphedema caused by injury, obstruction or infiltration of the vessels or lymph nodes such as:
● Surgery, biopsy and dissection in oncology.
● Parasitic cause (Filariase).
● Cancer and metastasis.
● Radiotherapy.
● Trauma and burns.
● Infection and inflammation (post erysipelas or lymphangitis).
● Paralysis.
● Chronic venous insufficiency.
● Mycosis: which causes skin lesions.
The ISL (International Society of Lymphology) specifies that within each stage, there are several degrees of volumetric differences to classify functional severity:
Mild edema (Less than 20% of member volume increase).
Moderate Edema (Between 20 - 40% of the member volume increase).
Severe edema (More than 40% member volume increase).
Staging of the ISL (International Society of Lymphology) to describe the stages of physical development of the disease:
Stadium 0 - Sub-clinical
Damage to the Lymphatic System, not apparent. The swelling is not yet evident, subtle changes in tissue composition. Lymphedema is not noticeable and may take months or years for the edema to manifest.
Stage 1
Relatively high fluid accumulation in proteins. The edema only appears in the afternoon and the swelling disappears when the limb rises. The edema leaves fovea.
Stage 2 - Precoz
Lymphedema does not go away after a moderate and prolonged rest. The swelling persists. Skin hard and just lets go of it by pressing hard. Elevation of the limb by itself has no effect on swelling.
Stage 2 - Late
It may or may not stop when excess fat and fibrosis of moderate and severe consistency appear.
Stage 3 - Lymphostatic Elephantiasis
Inflammation associated with complications such as, increased fat deposition and fibrosis, skin lesions with warts (papillomatosis), small vesicles (cysts), ruptures in the skin with lymph exudation (fistulas) and permanent induration.
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