Lymphedema can be congenital or acquired.

We say that lymphedema has a congenital origin when a child has some alteration of the lymphatic system at birth. This condition may or may not evolve to lymphedema.

It is considered acquired when the subject was born normal, that is, without changes that may lead to the development of lymphedema. However, over the years, some factors can damage the lymphatic system, such as filariasis, surgeries, cancer, etc. and the person evolves with lymphedema.

Congenital lymphedemas are classified according to the age of onset as:

1 - Primary congenital lymphedema, when the disease manifests before the age of two. This can affect the lower limbs, upper limbs, or any part of the body.

2 - Early primary lymphedema manifests between two and 35 years of age and can affect the lower limbs, upper limbs, or any part of the body.

3 - Delayed primary lymphedema develops after the age 35 and can affect the lower limbs, upper limbs, or any part of the body.

One of the most studied forms in the literature is autosomal dominant inheritance in which the individual is already born with edema (Milroy's Disease).

The most common acquired forms of lymphedema are:

1 - Filariasis that can affect any part of the body such as the lower limbs, upper limbs, breast, penis, scrotum or thorax.

2 - After surgeries, such as to treat breast cancer and gynecological cancers, that affect the lymphatic system. This can affect any part of the body including the lower limbs, upper limbs, breast, penis, scrotum or thorax.

3 - After radiotherapy, lymphedema can affect any part of the body such as the lower limbs, upper limbs, breast, penis, scrotum or thorax.

4 - After erysipelas, lymphedema can affect any part of the body such as the lower limbs, upper limbs, breast, penis, scrotum or thorax.

These are the most frequent causes of lymphedema however there are several causes where the lower limbs, upper limbs, breast, thorax, scrotum and penis are the regions of the body most affected.

Function of the lymphatics

The two main functions of the lymphatic system are:

1 - Return macromolecules from the interstitial space back into blood circulation

2 - Immunological function

 

Epidemiological data of the disease

In Brazil, there are no exact numbers of people suffering with lymphedema. However, around the world, it is estimated that lymphatic filariasis affects more than 120 million people in 73 countries with more than 20% of the planet's population living in endemic areas. In Western countries, it is estimated that 600 thousand to 2 million people suffer from lymphedema.

Recent data show that about 25% of women submitted to breast cancer treatment will develop secondary lymphedema.

 

The effects

Lymphedema interferes in physical and psychological and social aspects of the patient’s life. People with lymphedema suffer limitations in the daily life because the affected limb can cause a negative picture in the patients' psychic and affect their quality of life. Lack of guidance in the postoperative period of breast cancer treatment increases the possibility of developing lymphedema.

Early stages of Lower limb lymphedema may go unnoticed, but upper limb lymphedema is more difficult to conceal. Breast cancer-related lymphedema is one type of lymphedema that causes the greatest psychological impact due to the circumstances in which it develops. For this, a psychologist's help is always welcome.

 

Signs and symptoms of lymphedema

Edema is the main clinical manifestation of lymphedema. The swollen limbs may present with a sensation of heaviness, repetitive infections, verrucosities, changes in color, ulcers, etc.

Interdigital mycoses are frequent in lymphedema and require the patient’s daily attention. Sufferers are advised to take care of their feet similar to taking care of the face and every day investigate whether there is a wound, mycosis or any other change that may predispose them to an infection. Erysipelas can occur every time the skin barrier is broken; this is one of the main aggravating factors of lymphedema.

Fever and chills are signs of a probable infection. Patients with lymphedema should seek medical attention immediately to identify what is happening.

Recurrent erysipelas is also a warning sign for the possibility of subclinical lymphedema and worsening of the disease.