$Unique_ID{BRK03698} $Pretitle{} $Title{Elephantiasis} $Subject{Elephantiasis Elephantitis Lymphedema, Hereditary Lymphedema, Secondary Filariasis} $Volume{} $Log{} Copyright (C) 1990 National Organization for Rare Disorders, Inc. 689: Elephantiasis ** IMPORTANT ** It is possible that the main title of the article (Elephantiasis) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Elephantitis Information on the following diseases can be found in the Related Disorders section of this report: Lymphedema, Hereditary Lymphedema, Secondary Filariasis General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Elefantiasis is a rare disorder of the lymphatic system. 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. For more information on this disorder, choose "Hereditary Lymphedema" as your search term in the Rare Disease Database. 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. For more information on this disorder, choose "Filariasis" as a search term in the Rare Disease Database. Therapies: Standard Treatment of elefantiasis usually involves surgery to remove excess skin. In extreme cases, the amputation of an entire limb may be necessary. In cases where the male genitals have been affected, reconstructive surgery on the penis and scrotum has been successful. Anti-streptococcal antibiotics are used to relieve infection. Lymphatic tissue is removed by surgery or radiation therapy. Therapies: Investigational 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. Resources For more information on Elephantiasis, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 NIH/National Institute of Allergy and Infectious Diseases 9000 Rockville Pike Bethesda, MD 20892 (301) 496-5717 Centers for Disease Control (CDC) 1600 Clifton Road, NE Atlanta, GA 30333 (404) 329-3534 References THE MERCK MANUAL, Volume 1, 14th Ed.: Robert Berkow, M.D. ed.-in-chief; Merck Sharp & Dohme Laboratories, 1982. Pp. 372. ELEPHANTIASIS NOSTRAS: AN EIGHT-YEAR OBSERVATION OF PROGRESSIVE NONFILARIAL ELEPHANTIASIS OF THE LOWER EXTREMITY. L.J. Sanders et al.; CUTIS (Nov. 1988; 42(5)). Pp. 406-411. ELEPHANTIASIS NOSTRAS--A CASE REPORT: S.A. Baughman et al.; ANGIOLOGY (Feb. 1988 39(2)). Pp. 164-168.