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$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.