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ELEPHANTIASIS
NORD is grateful to Bernard Zazula, MD, for assistance in the preparation of this report.
SYNONYMS OF ELEPHANTIASIS
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Idiopathic Elephantiasis
DISORDER SUBDIVISIONS
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No subdivisions found.
GENERAL DISCUSSION
Elephantiasis is a condition characterized by gross enlargement of an area of the body, especially the limbs.
Other areas commonly affected include the external genitals. Elephantiasis is caused by obstruction of the
lymphatic system, which results in the accumulation of a fluid called lymph in the affected areas.
Functioning as part of the immune system, the lymphatic system helps to protect the body against infection
and disease. It consists of a network of tubular channels (lymph vessels) that drain a thin watery fluid
known as lymph from different areas of the body into the bloodstream. Obstruction of these vessels results
in the massive swelling and gross enlargement characteristic of elephantiasis.
In areas where filariasis is endemic, the most common cause of elephantiasis is a parasitic disease known as
lymphatic filariasis and, in the medical literature, the terms lymphatic filariasis and elephantiasis may be
used interchangeably. Elephantiasis due to lymphatic filariasis may also be referred to as "true"
elephantiasis. In most areas, the lymphatic damage associated with elephantiasis has other causes including
certain sexually transmitted diseases (e.g., lymphogranuloma venereum); tuberculosis; an infectious
disease called leishmaniasis; repeated streptococcal infections; leprosy; and environmental factors such as
exposure to certain minerals (e.g., silica). In some cases, no cause can be identified (idiopathic).
Recently a team of researchers funded by the National Institute of Allergy and Infectious Diseases (NIAID),
one of the National Institutes of Health (NIH), has recently revealed the genetic secrets of one of these
parasites. The researchers report solving the complete genome of Brugia malayi, one of the worms that
causes the often debilitating disease elephantiasis.
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SYMPTOMS
The main symptom of elephantiasis is gross enlargement and swelling of an area of the body because of the
accumulation of fluid. The arms and legs are the areas most often affected. An entire arm or leg may swell
to several times its normal size resembling the thick, round appearance of an elephant's leg. The skin of the
affected areas usually develops a dry, thickened, pebbly appearance and may become ulcerated, pitted and
darkened (hyperkeratosis). Fever, chills, and a general feeling of ill health (malaise) may also be present.
Elephantiasis may also affect the male and female external 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 thicken. Affected individuals may experience pain and
a burning sensation.
The external parts of the female genital organs (vulva) may also be affected by elephantiasis. A tumorous
mass covered by thickened and ulcerated skin may develop between the thighs and may be accompanied by
enlarged lymph nodes (lymphadenopathy) of the legs. In some women the breasts may become enlarged.
Underlying damage to the lymphatic system may leave individuals susceptible to secondary bacterial and
fungal infections that can greatly worsen the condition. Although the legs, arms and external genitalia are
most often affected, elephantiasis can affect any area of the body.
CAUSES
Elephantiasis is caused by obstruction of the lymph vessels of the lymphatic system. As lymph moves
through the lymphatic system, it is filtered by a network of small structures known as lymph nodes that help
to remove microorganisms (e.g., viruses, bacteria, etc.) and other foreign bodies. Groups of lymph nodes
are located throughout the body, including in the neck, under the arms (axillae), at the elbows, and in the
chest, abdomen, and groin. In addition to the lymph nodes, the lymphatic system includes the spleen, which
filters worn-out red blood cells and produces lymphocytes, and the tonsils, which are masses of lymphoid
tissue in the throat region that help to fight infection. Lymphatic tissues also include the thymus, a relatively
small organ behind the breastbone that is thought to play an important role in the immune system until
puberty, as well as the bone marrow, which is the spongy tissue inside the cavities of bones that
manufactures blood cells. Lymphatic tissue may also be located in other regions of the body, such as the
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skin, small intestine, liver, and other organs.
In underdeveloped regions of South America, Central Africa, Asia, the Pacific Islands and the Caribbean,
obstruction can be caused by a parasitic disease known as lymphatic filariasis. Lymphatic filariasis is caused
by three different species of worms known as Brugia malayi, Brugia timori and Wuchereria bancrofti. These
worms cause damage and inflammation to the lymphatic system. The larval form of the worms is introduced
into the human body through the bite of infected mosquitoes.
Genital elephantiasis can also be caused by bacterial sexually transmitted diseases, specifically
lymphogranuloma venereum (LGV) and donovanosis. The bacterium that results in LGV, Chlamydia
trachomatis serovar L1-L3, damages the lympathic system resulting in lymphatic obstruction in the genitals.
Chronic obstruction eventually results in genital elephantiasis. Donovanosis is caused by the bacterium
Calymmatobacterium (Klebsiella) granulomatosis. Donovanosis causes genital elephantiasis because the
body's immune system response to the bacterium causes inflammation and narrowing (constriction) of the
lymphatic vessels.
Elephantiasis is also associated with a disorder known as podoconiosis. Podoconiosis, sometimes referred to
as nonfilarial elephantiasis, is a disorder caused by the absorption of minute mineral particles from the soil
through the feet of barefoot individuals. It is believed that the mineral particles cause an immune system
response eventually resulting in the formation of inflammatory masses of nodules (granulomas) in the
lymph vessels of the feet and legs.
Additional causes of elephantiasis include a protozoan disease called leishmaniasis, tuberculosis, leprosy,
and a repeated streptococcal infection. Elephantiasis may also occur secondary to trauma, surgery or
radiation. For example, treatment such as the surgical removal of lymph nodes to treat cancer may result in
the accumulation of lymph and subsequent swelling (lymphedema).
AFFECTED POPULATIONS
Elephantiasis occurs with the greatest frequency in tropical regions including Southeast Asia, India, Africa
and South America as a manifestation of lymphatic filariasis.
Elephantiasis due to other causes is rare. Elephantiasis can affect men or women of any age.
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RELATED DISORDERS
Symptoms of the following disorders can be similar to those of elephantiasis. Comparisons may be useful for
a differential diagnosis.
Hereditary lymphedema is an inherited disorder of the lymphatic system that is characterized by abnormal
swelling of certain parts of the body. The lymphatic system is a circulatory network of vessels, ducts, and
nodes that filter and distribute certain fluid (lymph) and blood cells throughout the body. Lymphatic fluid
collects in the soft tissues in and under the skin (subcutaneous) due to the obstruction, malformation, or
underdevelopment (hypoplasia) of various lymphatic vessels. There are three forms of hereditary
lymphedema: congenital hereditary lymphedema or Milroy disease, lymphedema praecox or Meige disease,
and lymphedema tarda. Symptoms include swelling of affected areas (lymphedema) and thickening and
hardening of the skin in affected areas. In most cases, hereditary lymphedema is inherited as an autosomal
dominant trait. (For more information on this disorder, choose "hereditary lymphedema" as your search
term in the Rare Disease Database).
STANDARD THERAPIES
Diagnosis
A diagnosis of elephantiasis is made based upon a thorough clinical evaluation, a detailed patient history and
identification of characteristic symptoms. A variety of tests may be used to determine the underlying cause
of lymphatic damage and subsequent elephantiasis.
Treatment
Treatment of elephantiasis usually involves treating the underlying condition. Lymphatic filariasis is treated
with diethylcarbamazine. LGV is treated with doxycycline. Donovanosis may be treated with azithromycin.
However, in many cases, medical therapy alone is not enough and surgery 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 secondary infection. Lymphatic tissue may be
removed by surgery or radiation therapy.
Other treatment is symptomatic and supportive.
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