Patients manifest with a chronic swelling and fullness of the affected limb. Most commonly it is the lower limb and the upper extremity that are affected; trunk and genitalia are less frequently affected. The patient notices asymmetry of the extremity. This progresses to increased heaviness of the limb, fatigue and difficulty in wearing clothes. There is severe deterioration in performing daily activities. The swelling of the limb is usually painless though some patients do report of constant aching in that area.

As the edema becomes chronic and long standing it develops discoloration, the skin becomes dense and appears thick. The texture appears as that of an orange peel. There is fibrous tissue and scar formation.

In addition to this, recurrent fever with chills, weakness, and repeated cellulitis and infections are common. Locally, there might be fissuring, recurrent ulcer formation, and lymphorrhea [6]. Lymphorrhea constitutes of oozing of clear or straw colored fluid from the affected limb. Inability to pinch the affected skin in between two fingers is called as the positive Stemmer test, and is useful in eliciting the diagnosis for lymphedema.

The degree of severity, from mild to severe forms is accessed through a staging method introduced by the Lee, Morgan and Bergan through the American society of lymphology. This method benefits the physician in indentifying the severity of the case and in providing the necessary intervention accordingly.

• Stage 0: Absent lymphedema. This is a latent stage. Here, the lymphatic vessels are destroyed to a mild degree and hence it is not yet apparent.
• Stage 1: The edema is visible but is reversible. It resolves on elevating the limb. Also, the affected area is normal in morning and swelling appears as the day progresses. 
• Stage 2: The tissues are affected. They become non-pitting on pressure. Lymphedema is evident with increasing swelling and hardening of the skin. 
• Stage 3: Irreversible swelling is seen in this stage. The limb tissue becomes hard and non tender. There is marked heaviness and disability of the limb. There is complete blockage of the lymphatic vessels. This is also called as stage of elephantiasis [4] [7].

Blood tests include complete blood count, liver function tests, urine examination, creatinine and urea levels including electrolytes. These tests help in identifying the underlying infectious etiology.

Imaging studies such as lymphangiography might be done; it is an invasive technique that evaluates the lymphatic channels and their patency. This technique causes a lot of adverse reactions such as atrophy, fibrosis and obliteration; thus it is not advised frequently. Ultrasonography identifies structural and capacitative changes within the lymphatic vessels. A CT scan and MRI are done in cases of a suspected malignancy [8] [9].

Treatment comprises of use of compression bandages and garments including stockings. These are applied for several days so that tension is maintained in order to reduce lymphedema. These bandages should be costume fit and should not produce a tourniquet effect. These should be worn during the day and during activities short bandages are preferred that improve the pumping activities against increasing resistance [5] [9].

CDT is complete decongestive therapy and consists of a combination of exercises, bandaging and after skin care. Here, under expert guidance, manual massaging of the limb is done to improve circulation and lymph flow. The treatment is highly effective and enhances drainage.

Intermittent pump therapy involves use of multi-chambered pump compression that causes effective drainage of the lymph.

Surgery offers palliation. It helps to reduce the load of the swelling and is useful for patients who did not respond to the conservative treatment method. Appropriate hygiene is very important to observe in cases of lymphedema. Repeatedly cleansing of the area is recommended in order to eliminate dead skin and debris and prevent the invasion of bacterial or other microbial infections. Patients are advised to lose weight, monitor the limb regularly and to wear proper clothing and comfortable foot wear [10].

Prognosis depends on the severity of the lymphedema, the general health of the patient and the underlying conditions that have caused it.

Long standing lymphedema may develop into a lymphangiosarcoma. This tumor has a very poor prognosis and requires amputation at its earliest. Kaposi sarcoma, B-cell lymphoma are other tumors found with chronic lymphedema.

Lymphedema may also lead to complications such as cellulitis, bacterial infections or deep vein thrombosis leading to functional impairment and disability [5].

Based on the causes, lymphedema can be categorized into primary and secondary lymphedema.

Primary causes are related to congenital or developmental arrest of the lymphatic system. In congenital lymphedema, there is failure of the lymphatic channels to develop. This is an autosomal-dominant disorder. The genetic defect mutated and is mapped into the telomeric part of chromosome 5. This most commonly affects bilateral lower extremities. Meige disease or lymphedema praecox is a common form of primary lymphedema. This is more frequently found after a birth defect. Lymphedema tarda is due to valvular incompetency. This is found in individuals older than 35 years [2].

Secondary lymphedema is obtained due to underlying conditions such as infection, trauma, obesity or a tumor mass. This condition obstructs the lymph flow or damages the vessels carrying the lymphatic fluid, leading to an abnormal collection, producing a swelling and edema of the affected arm or leg.

Studies reveal that lymphedema usually follows after a breast cancer surgery. Around 80% of the cases show congenital onset. Demographics reveal that lymphedema that is primary in nature is seen from birth up to 1 year of age and the secondary ones can occur at any age.

Amongst the infections, filariasis is the most frequent cause leading to lymphedema. Obesity has been highly associated with lymphedema.

Lymphatics normally drain proteins, lipids and water from the interstial tissues into the circulation filtering via the lymph nodes. This occurs through osmotic mechanism. The flow of the lymph is controlled by the valves within the vessels to prevent the back flow of the fluid, also the surrounding muscle contraction and the pulse pressure are important factors involved with the lymph flow.

In case of lymphedema, the transport volume is reduced. The amount of fluid formation exceeds to the volume of fluid returned [3]. Consequently there is stagnation, edema formation with high concentration of proteins per ml. Eventually, the lymphatic vessels undergo changes in the form of fibrosis, thrombus formation and shunt formation.

Within the interstitial spaces, fluid accumulation leads to inflammation producing increased macrophage activity and collagen deposition. This eventually progresses to a suppressed immune activity that increases probabilities of infection and degeneration.

Locally, the skin appears congested and thick; the characteristic appearance is that of an orange skin called peau d’orange appearance. The epidermal layer of the skin gets destroyed and develops fissures, resulting in leakage of the lymph on the surface of the skin. Bacteria enter and multiply leading to a condition called elephantiasis verrucosa [4].

Those at a risk for developing lymphedema should observe special precautions especially after undergoing a cancer surgery. Injury to the limb from sharp objects should be avoided. Regular exercise and elevation of the affected limb during rest is advisable. Tight garments hamper circulation and cause constriction; hence comfortable size garments should be worn. Lastly, skin care and hygiene must be observed regularly to avoid infection [1] [11].

The lymphatic fluid and vessels essay a very vital role in the body’s immune system. The lymphatic circulation consists of many lymph nodes and lymphatic vessels through which the lymphatic fluids pass [1]. It acts by draining the unwanted lymphatic fluid from tissues of the body in the blood circulation for removal.

When this lymphatic fluid is abnormally accumulated within the interstitium, it results in swelling under the skin leading to edema called as lymphedema.

Lymphedema develops in cases where normal lymph drainage is obstructed due to congenital causes or underlying tumors, after surgery or trauma, the fluid builds up causing increased swelling and discomfort.

Long standing lymphedema increases the risk of infection, cellulitis and fibrosis. Treatment includes use of compression bandages and garments like wearing stockings and bandages. These help in improving lymph drainage and circulation. Secondly, gentle exercises, elevation of the limb while resting are advised to enhance lymph drainage. Surgery is advised in cases when conservative treatment does not respond. Other precautions observed are protection of the limb from any injury especially from sharp objects and needles.

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