Elephantitis (Elephantiasis)

What is Elephantitis?

Elephantiasis is a condition presenting as marked enlargement of certain regions of the body, most common of which are the upper and lower extremities, and genitalia. Usually misspelled and pronounced as elephantitis, the disease is also characterised by thickened and inflamed skin and its underlying tissues. [1, 2, 3, 4]

Elephantiasis: Evolving Through Time

The first ever case of elephantiasis can be dated back to the period before the Biblical era. The term was first coined by Celsus, as an alternative name for the illnesses sarcocele, leontiasis, and satyriasis. [5, 6]

At present, complete eradication of the disease is still not within reach. It is unfortunate that even at this very moment, the risk of elephantiasis lurks in almost a billion people worldwide. More than 80 countries are endemic for the illness. It is of major medical concern for over 120 million people, and among them, over 40 million are already debilitated by this particular illness. In 2007, increased initiation of treatment was done in almost 50 countries, with 27 of which already has strategies on further prevention of disabilities. [4, 7, 8]

In Africa, more than 382 million are exposed to elephantitis in almost 40 of its countries. In the Southern part of America, 11 million people are at risk, with Brazil, Guyana, Haiti and Dominican Republic reported to have cases of active transmission. While in Asia, nine out of eleven countries are endemic. Egypt, Yemen and Sudan, on the other hand, are the areas endemic for the disease in the Eastern Mediterranean. These are facts proving that this illness does not choose any particular race, and that anywhere, people could be at risk. [7]

How Does One Get Elephantiasis?

In contrary to popular belief, elephantiasis can be brought about either by parasitic or nonparasitic infections.

Lymphatic Filariasis

Elephantiasis presenting in lieu of parasitic infections are called lymphatic filariasis. These occur due to infestation of mosquito borne parasitic worms such as Brugia timori, Brugia malayi and Wuchereria bancrofti. [9]

Infections are transmitted by larvae thru mosquitoes. These larvae are transported to the lymphatic system, where they mature into reproductive adult worms and reside for a number of years. Female worms generate microfilariae, which eventually are ingested by mosquitoes and become the highly infective filariform worms. As mosquitoes bite a human prey, the infective filariform is then transferred to the host, assuming infection. [7, 8]

In time, these worms would be trapped in the lymphatic system of their human host. These would then spread into the circulation, block lymphatic flow all through the body, and cause an imbalance in the lymphatics. This disequilibrium of the fluid between the blood and tissues of the body will, in turn, produce enlargement of the certain parts of the body, particularly the extremities and genitalia. Hence, elephanthiasis ensues. [7, 8, 10, 11]

A diagram showing how the worms causing lymphatic filariasis travels from mosquitoes to its host, humans.

Source: www.sciencedaily.com

Podoconiosis

Elephantiasis without the presence of a parasitic infestation is termed as Nonfilarial Elephantiasis or Podoconiosis. This disease is thought to be caused by an inherent abnormal reaction to inflammation brought about by red clays, mainly composed of the alkalis, sodium and potassium, found in volcanic deposits. [4, 7]

Irritant clay components made of aluminum, magnesium, silicon and iron are absorbed through the feet. These have been microscopically noted on the macrophages of the lymph nodes found in the lower extremities, reducing and destroying the lumen of the lymphatics. Subsequently, blockage of the lymphatic flow can be observed. Hence, lymphedema, enlargement of the genitals and extremities, and elephantiasis eventually follow. [12]

An image of how podoconiosis is gotten, through irritant clay soils.

Source: www.demotix.com

 Elephantitis Balls (Testicular elephantiasis)

Elephantiasis in males will often manifest itself in the genitals in the form or testicular elephantiasis (elephantitis balls) or penile elephantiasis. In mild cases this can cause swollen testicles that will be the size of a tennis ball and in worse cases the testicle can be the size of a football or larger.

What Are the Signs and Symptoms of Elephantiasis?

Lymphatic Filariasis

The disease is characterized by noticeable swelling of a body part, most common of which involves the extremities and the genitals. Within such body parts, abnormal unregulated accumulation of lymph in the tissues ensue, causing edema and severe swelling. There is usually thickening of the skin, which is pebbly in character, with ulcer and hyperpigmentation. [9, 13]

Male genitalia may also be affected. Enlargement of scrotum and hydrocele may be visible. The penile skin may be thickened, retracted, tender and swollen. Spermatic cords may also be noted to be thickened. [7, 9]

Among females, the vulva is the most common part involved. Aside from lymph node enlargement at the lower extremities, a mass with thickened and ulcerated skin covering may also expand along the thighs. Breasts may also be involved. [7, 9]

These signs are usually associated with constitutional signs and symptoms such as body malaise, fever and chills. [9]

Podoconiosis

Before elephantiasis is observed, a prodromal phase may be noted. This is characterized by pruritus and burning sensation of the foot and leg, pedal edema, pronounced markings in the skin, splaying of the forefoot, rigid toes and skin thickening. After which, swelling, of either soft or hard character, may be noted. Occasional episodes of fever and leg tenderness may also be present, hinting the development of fibrosed, hard extremities. [7, 12]

Podoconiosis starts in the foot and ascends to the knee, however, rarely involving the genitalia. This is in contrast to lymphatic filariasis, wherein changes are usually noticed in the groin. [7]

A photo depiciting a patient with podoconiosis.

Source: www.nejm.org

Diagnosing Elephantiasis

Diagnosis is usually based not only on history and clinical findings, but also in location and presence of microfilariae. [7, 13]

Elephantiasis among persons residing in areas of high altitudes, of more than a thousand meters above sea level, is likely due to podoconiosis. [7]

In doing a microscopic examination , blood is extracted usually at night due to the nocturnal periodicity of microfilariae. Presence of microfilariae confirms lymphatic filariasis. Its absence, on the other hand, indicates podoconiosis. [8, 13]

How is Elephantiasis Treated and Prevented?

To eliminate lymphatic filariasis and the adult worms that cause it, the medication Diethylcarbamazine (DEC) is given annually to patients and persons exposed. This may not eradicate the worms, but it reduces transmission of the disease. For those patients with allergic reactions to DEC, ivermectin is given. Both of these are usually used in conjunction with albendazole during the Mass Drug Administration (MDA) program of the WHO. [7, 8]

No specific treatment for podoconiosis is at hand. Prevention is still the key in managing this not so well documented disease. [7]

The methods of preventing and managing podoconiosis are also similar to those of lymphatic filariasis. [7, 8, 13]

• Avoid exposure to mosquitoes and irritant clay soils.
• Wear long sleeves, boots and socks.
• Use mosquito repellants.
• Daily bathing with soap and water is recommended.
• Leg elevation and exercises are advised.
• In reducing swelling, compression bandages are effective.
• Disinfection of wounds with the use of antibacterial ointments.
• If aforementioned medical therapeutics are not working, surgery is the next option.
• Immediate referral to a physician when signs and symptoms are noted is a must.

References:

1. http://www.en.wikipedia.org
2. McNeil, D. Beyond Swollen Limbs, a Disease’s Hidden Agony. The New York Times. 2006 April.
3. http://www.rarediseases.org
4. http://www.news-medical.net
5. Raymond, F. Histoire de l’Elephantiasis. Lausanne, Marseilles, 1767.
6. Hadju, S. A Note From History: Elephantiasis. Annals of Clinical And Laboratory Science. 2002; 32(2): 207-9.
7. http://www.who.int
8. http://www.cdc.gov
9. http://www.elephantiasis.freeyellow.com
10. Saladin, K. Anatomy and Physiology: The Unity of Form and Function. 20007. McGraw-Hill.
11. Niwa, S. Prevalence of Vizcarrondo Worms in Early Onset Lymphatic Filariasis: A Case Study in Testicular Elephantiasis. University of Puerto Rico Medical Journal. 22:187-93.
12. Davey, G. Podoconiosis: Non-Infectious Geochemical Elephantiasis. Transactions of the Royal Society of Tropical Medicine and hygiene. 2007; 101: 1175-80.