Leg Lymphedema: Non-thrombotic Lower Limb Edema

Lymphedema can be a symptom of various conditions such as cardiovascular, liver, kidney, or hormonal disorders, malnutrition, or vitamin deficiencies. It can also result from the use of certain medications or occur idiopathically (without a known cause).

In the course of glomerulonephritis (primary or secondary), edema may present as a symptom of nephrotic or nephritic syndrome. Edema may also occur during acute or chronic kidney failure.

Causes of  Leg Lymphedema

Lymphedema is a swelling of the skin and subcutaneous tissue caused by the accumulation of lymph (a fluid containing water, proteins, lymphocytes, and metabolic waste) in the intercellular spaces. 

It arises from pathological changes in the lymphatic vessels that lead to obstruction, underdevelopment, or destruction of these vessels. Normally, the lymphatic system transports lymph from the tissues back to the circulatory system. If the vessels are damaged for any reason, lymph leaks into the surrounding tissues and accumulates, leading to swelling.

Lymphedema may result from congenital malformations of the lymphatic system or damage caused by other diseases, such as chronic venous insufficiency, inflammation of lymph vessels or nodes, or trauma.

Initially, these changes are reversible and may subside with proper treatment. However, if the edema persists for a long time, the condition worsens and the affected area may not return to normal. Impaired lymph drainage leads to connective tissue overgrowth, skin thickening, recurrent lymph vessel infections, and progressive subcutaneous tissue hypertrophy.

It most commonly affects the limbs, both upper and lower, and it is estimated that approximately 140 million people worldwide are affected by this condition.

Types of Lymphedema

There are two primary types of lymphedema: primary and secondary.

• Primary lymphedema is caused by abnormal development of lymphatic vessels and may be congenital:
  • Early-onset – rare, typically observed at birth or during childhood.
  • Late-onset (about 80% of primary lymphedema cases) – usually affects the lower limbs, with a higher frequency in females.
• Secondary lymphedema is more common and results from injury, infection, surgery, or tumors.
  Any adult-onset lymphedema should prompt an evaluation for possible malignancies that may be causing it.

Symptoms of Lymphedema

Chronic leg lymphedema is firm and rubbery, and only slightly reduces after resting with the affected limb elevated. In early stages, pressing the swollen area may leave a dent. Edema typically begins around the ankles or top of the foot and gradually involves the entire limb, sometimes extending to the genitals or buttocks. The Stemmer sign (inability to pinch a skin fold at the base of the second toe) is often present.

Over time, the condition may lead to:

• Fibrosis, hardening, and loss of skin elasticity,
• Loss of sweat and sebaceous glands,
• Hair loss,
• Skin changes (thickening, keratinization, lymph leakage),
• Increased susceptibility to infections, especially in the foot and lower leg,
• Brittle, yellowing toenails.

In very advanced stages, elephantiasis (extreme swelling and skin thickening) may develop. The disease progresses slowly but steadily. Lymphedema is usually painless, though it can be debilitating. In rare cases, lymphangiosarcoma, a malignant tumor, may develop after many years of chronic lymphedema, particularly in the lower limbs.

Prevention and Treatment 

Lymphatic system failure may be mechanical or dynamic. Dynamic insufficiency occurs when a healthy lymphatic system cannot cope with excessive fluid load – in such cases, treating the underlying disease is the priority.

For preventive and conservative treatment of lymphedema, the following measures are recommended:

• Avoid prolonged standing or sitting,
• Elevate the affected limb,
• Maintain proper skin hygiene,
• Use graduated compression (compression therapy),
• Undergo manual lymph drainage (massage),
• Avoid injuries to the affected limb.

It is also crucial to prevent and properly treat skin infections.

In cases of extensive lymphedema, microsurgical procedures may be considered. For cases resistant to compression therapy, liposuction has shown effective treatment results.