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What is lymphoedema?
Literally, lymphoedema means edema (swelling) that arises out of lymph fluid accumulation. Typically, it produces swelling in the arms and legs. There are many reasons that can lead to such swelling.
What causes lymphoedema?
Depending upon the causes, lymphoedema can be of two broad types:
Primary: 
- Primary lymphoedema can be present at birth (called congenital) or it may develop during puberty or adulthood. The exact cause of primary lymphoedema is not known. There will be a history of several other family members being affected by the same disease.
Secondary:
- Diseases: Certain disease conditions like elephantiasis or filariasis cause lymphoedema in endemic countries. This disease is caused by parasites and is transmitted by mosquitoes. It is the commonest cause of secondary lymphoedema.
- Surgery: Surgeries which involve removal of lymph nodes, usually during cancer treatment; e.g. radical mastectomy (removal of breast tissue and surrounding lymph nodes in a breast cancer patient) and radical prostatectomy (removal of a cancerous prostate gland and surrounding lymph nodes) can cause lymphoedema of the corresponding arm and leg respectively.
- Medication: Drugs such as megestrol, tamoxifen, and other cancer medications, radiation therapy to the lymph nodes can also cause lymphoedema.
- Trauma: damage to the lymphatic system, particularly to the lymph nodes owing to trauma may also lead to lymphoedema.
What are the signs and symptoms of lymphoedema?
- The earliest symptom is pitting edema which is characteristically, not painful when touched. Eventually, this progresses to a non-pitting swelling. The swelling generally follows ‘the glove and sock pattern,’ which means the condition gradually affects the tips of toes and gradually ascends upwards and then affects the tips of the fingers too.
- The affected arms or legs may appear several times larger than the normal. This can be quite embarrassing to the patient and often the first reason to seek treatment.
- The skin becomes red and thickens and may have the appearance as that of an orange peel.
- If left unattended, the skin may develop cracks and ulcers, and lymph may start oozing out. This may cause secondary infections of the skin and lead to serious diseases like cellulitis. Utmost care of skin is required at this stage.
How is lymphoedema diagnosed?
Diagnosis of lymphoedema is largely clinical. Diagnostic tests will be required in cases where the cause of lymphoedema is not obvious. Imaging techniques are used to locate the blockages in the lymphatic system. These include:
- MRI scan
- C T scan
- Doppler ultrasound
- Lymphoscintigraphy (Radionuclide imaging of the lymphatic system)
How is the staging of lymphoedema done?
The staging of lymphoedema is done as per the criteria by “International Society of Lymphology”. The stages are as follows:
- Stage 1: It is the pre-clinical stage. There is no swelling in this stage. The patient is just prone to developing a swelling and is under observation.
- Stage 2: Mild swelling can be seen. Difference between circumference of the affected arm/leg and the normal counterpart is less than 4 cm. A small dent can be seen if one presses the affected area. This is known as pitting edema. Elevating the arm/leg helps reduce the swelling, but the swelling returns back as soon as the normal position is restored.
- Stage 3: Moderate swelling can be seen. In this stage, the swelling is non-pitting i.e. there is no dent formed on applying pressure. The difference between circumferences of affected and normal extremities is more than 4 cm. The tissue under the skin gets inflamed and hardens.
- Stage 4: Severe enlargement of the affected part is seen. The skin is leathery and wrinkled, and the limb misshapen. It is more commonly associated with filariasis but not so common in post-surgery patients. It may be very difficult to walk in case the leg is affected, due to the heaviness. Face, and genitals may also be involved.