Elephantiasis

Elephantiasis, or Lymphatic Filariasis, is a rare disorder of the lymphatic system caused by parasitic worms such as Wuchereria bancrofti, Brugia malayi, and B. timori, all of which are transmitted by mosquitos. Inflammation of the lymphatic vessels causes extreme enlargement of the affected area, most commonly a limb or parts of the head and torso. It occurs most commonly in tropical regions and particularly in parts of Africa

Symptoms

Elephantiasis is characterized by the gross enlargement of a limb or areas of the trunk or head. There is an abnormal accumulation of watery fluid in the tissues (edema) causing severe swelling. The skin usually develops a thickened, pebbly appearance and may become ulcerated and darkened. Fever, chills and a general feeling of ill health (malaise) may be present.

Elephantiasis may also affect the male and female genital organs. In a male, there may be enlargement of the scrotum, and the penis may be retracted under skin which has become thickened, nonelastic, hot and painful. The spermatic cords may become thickened.

The external parts of the female genital organs (vulva) may also be affected by elephantiasis. A long, tumorous mass covered by thickened and ulcerated skin may develop between the thighs. There may also be an enlargement of the lymph nodes of the legs.

Causes
The extreme enlargement of the limbs and other areas of the body characterized by elephantiasis, is the result of obstruction of the lymph flow and possibly of blood circulation. The lymphatic blockage can be due to recurrent attacks of a bacterial infection which causes inflammation of the lymphatic vessels (streptococcal lymphangitis). When the lymphatic obstruction is large enough, back pressure in the lymphatic channels produces dilation of the superficial vessels, resulting in extreme swelling. Without medical intervention, the cycle continues until the affected area is grotesquely enlarged. Death of surrounding tissues may also occur from an obstructed blood supply (gangrene).

Recent studies have shown that a possible cause of elephantiasis in Africa may be related to the red soil on which certain barefooted populations live. It is believed that small chemical particles found in the soil may enter the skin through the bare feet. These particles then lodge in the lymphatic tissues and produce irritating effects. The traumatized tissue is then vulnerable to streptococcal infection.

Affected Population
Elephantiasis is most commonly found in African nations.

Related Disorders
Hereditary Lymphedema is a genetic disorder of the lymphatic system. Major symptoms may include swelling of the tissue under the skin resulting from obstruction, destruction or underdevelopment of lymph vessels and accumulation of excessive lymph fluid.

Secondary Lymphedema is a disorder of the lymphatic system resulting from infection. Symptoms may include sudden onset, chills, high fever and the presence of a red, hot, swollen leg.

Filariasis is a disorder spread to man by mosquito bite. Major symptoms may include chills, fever, headache and elephantiasis.

Standard Therapies
Treatment can include chemotherapy to attack the adult worms as well as symptomatic treatment to repair damage caused by the body's reaction to the presence of dead worms. Drugs including Suramin (Antrypol ), Diethylcarbamazine (DEC, Heterazan, Banocide, and Notezine ), Ivermectin (Mectizan), Metrifonate (Trichlorphon), Mebendazole and Levamisole have shown to be effective in treating conditions associated with a filarial infection.

Failure of the Lymphatic system due to an infection provides an opportunity for microbial infections to develop. These infections can be minimized by proper anisepticalyl hygenic care. Surgery can be performed to remove or bypass damaged lymphatic regions.

Investigational Therapies
It has recently come to my attention via a massage therapy student that lymphatic massage has been successful in treating some cases.

This disease entry is based upon medical information available through July 1990. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.