Figure 1. Lower extremity demonstrating lymphedematous limb with swelling on the dorsum of foot, hyperkeratosis and papillomatosis (navel orange for comparison).

Lymphedema is a disease process that results from failure of the lymphatic transport system. It usually manifests as lower extremity swelling which maybe unilateral or bilateral. However, it can also present as swelling to the trunk, head and neck or external genitalia. It is essentially lymphatic fluid which is protein rich accumulating in the interstitial space. Two types of lymphedema can occur: Primary which is usually due to developmental abnormality and is subclassified based on age at onset. Congenital is from birth to age 1, Precox from age 1 to 35, Tarda is age 35 and onwards. Secondary which is due to a disease process. Secondary is the most common type that one will encounter in practice and it is disruption of the lymphatic pathways by disease, surgery or radiotherapy. Worldwide filariasis caused by the parasite Wuchereria bancrofti is the most common cause of lymphedema. In the USA axillary lymph node dissection is most common cause.

Risk factors for developing lymphedema include obesity, delayed wound healing which results in a prolonged inflammatory phase that disrupts the lymphatic circulation, axillary lymph node removal, sentinel lymph node biopsy, and venous insufficiency.

Symptoms of lymphedema include swelling of the affected body part which is often unilateral and involves the whole limb including the digits. Local symptoms are heaviness, tightness, weakness, reduced joint flexibility, aching or discomfort. Because of protein rich exudate there are often recurrent skin infection that occur. In chronic cases there is hardening, thickening, and tightening of the overlying skin.

Signs

Clinically we see swelling in the affected area, non-pitting edema and in the early stages the edema is mild and easily reversed. In the advanced stages the skin becomes indurated and fibrotic. Classic signs are the orange peel appearance of the skin, positive Stemmers sign which is inability to pinch the skin at the base of the second toe, swelling of the dorsum of the foot with squared appearance of the digits. Diagnosis is predominantly clinical and there are no set diagnostic criteria currently.

Management and Treatment

There is no curative treatment, so management is aimed at minimizing discomfort and swelling, and preventing infection.

There are two types of management conservative and surgical. Similar to other chronic diseases patient involvement is essential to achieving therapeutic goals. Complete decongestive therapy is ideal. It is a combination of lymphatic massage, compression, skin care and exercise. There are specialized centers that combine all these treatment modalities in one place.

Cornerstone to treatment is compression. The selection of type and level of compression is guided by patient tolerance, mobility, arterial vascular status, and any underlying conditions the patient may have. Essentially with compression therapy an external pressure is applied to the limb to help mobilize the fluid in the subcutaneous tissue. There are 3 different levels of compression that are achieved by using 2 different kinds of bandages Long stretch or elastic bandages, short stretch or inelastic bandages. Depending on the type of bandage used and the number of layers of bandage applied you achieve different levels of compression. There are 3 levels of compression: Mild compression is from 17 to 25 mm Hg. Moderate from 25-35 mmHg, High from 40-50mmHg.

Patients with lymphedema need to take care of their skin and take precautions to avoid getting infections or aggravating the edema. They must keep the affected area of skin clean and dry and moisturize the skin. They should wear gloves while gardening or cooking to avoid inadvertent injury or burns to the skin. They should avoid tight clothing or jewelry that impedes the circulation. They must not apply external heat to the affected area since this causes vasodilation and aggravates the swelling. Elevate the limb whenever possible to aid return of fluid. Immunization, blood draws and blood pressure measurements in affected limb may predispose to infection or swelling and should be avoided when possible.  A multidisciplinary approach has the most success of managing this chronic recurring condition.