Elephant Legs

Elephantiasis is a rare disease caused by impaired lymph flow. Let's consider features of the given pathology, kinds, stages, methods of diagnostics and treatment.

According to the International Classification of Diseases ICD-10, elephant leg is included in the category IX Diseases of the circulatory system (I00-I99):

I95-I99 Other and unspecified diseases of the circulatory system.

• I97 Circulatory system disorders after medical procedures, not elsewhere classified.
  • I97.2 Syndrome of postmastectomy lymphatic edema (elephantiasis, obliteration of lymphatic vessels, mastectomy).

Elephility of the lower extremities arises from the stagnation of lymph. Lymph fluid performs important functions in the body. It cleanses tissues and cells from accumulated toxins and normalizes the water balance. Colorless liquid provides blood to all organs and systems, participates in the formation of immunity.

With persistent edema, the metabolic products are not removed from the tissues, protein compounds disintegrate and provoke the formation of fibrin. This leads to the appearance of a coarse connective tissue between the muscles. The extremities increase in size, acquiring a cylindrical shape that resembles the legs of an elephant. The skin on the affected tissues is ulcerated, covered with cracks, rashes and warts.

The disease can occur with congenital weakness of the lymphatic system, trauma, parasitic and bacterial infections. In 70% of the disorder appears only on one leg, cases of bilateral lymphedema are extremely rare. But the problem is not only in the deformation of the limbs. Pathology affects internal organs and systems, disrupting the functioning of the whole organism. In particularly difficult cases, elephantiasis affects not only the legs, but also the hands, face, mammary glands, genitals.

Epidemiology

With the problem of lymphatic edema, more than 300 million people - 13% of the world's population. All of these are at increased risk of developing elephantiasis. At the same time, medical statistics indicate that only infection with filaria in the tropics causes disease in 100 million people.

In countries of Europe and on continents with a moderate climate, the disease is of low prevalence. Here elephantiasis arises from a number of other factors, both congenital and acquired.

According to statistical indicators, lymphedema most often affects the lower extremities - about 95% of all cases of the disease. Less likely to diagnose persistent swelling of the hands, mammary glands, face, genitals. In 70% of cases, the problem is one-sided.

[1], [2], [3], [4],

Causes of the elephantiasis

Elephantiasis is associated with pathological changes in the lymphatic system. The accumulation of fluid arises from the obstruction or narrowing of the lymphatic ducts. The reasons for elephant leg depend on the type of disease.

Secondary elephantiasis, that is, acquired, is directly related to the violation of the lymphatic system of different etiologies and can occur at any age.

• Tumor lesions and removal of lymph nodes, chemotherapy. The affected lymph node misses the lymph with certain disorders. The liquid accumulates in the vessels, stretches them and even gets into the tissues. Prolonged stasis provokes severe edema and germination of connective tissue.
• Erysipelas and phlegmon caused by streptococcal infection. Microorganisms multiply in lymphatic capillaries, and the toxins released by them cause allergic reactions. The immune system is struggling with the problem, provoking increased cell division and tissue increase in the volume.
• Damage to lymphatic vessels occurs with frostbite, extensive injuries, burn injuries. A large amount of lymph stagnates in the tissues, which causes persistent edema.
• Varicose veins and postthrombophlebitis syndrome. Defeat of deep veins disrupts the functioning and nutrition of soft tissues. Gradually, changes affect the lymph vessels, violating their patency, which leads to stagnation of lymph. In the modified cells, pathogenic microorganisms multiply causing the intoxication of the organism. This leads to tissue overgrowth and skin rashes.
• Parasitic infestations with an insect bite are another reason for elephantiasis. Mosquitoes and mosquitoes can infect humans with filarias, worms that parasitize in lymphatic vessels. Helminths intertwine into coils, clogging and stretching the lumen of the vessels. Toxico-allergic reaction of the body is accompanied by edema and proliferation of connective tissues.

Primary lymphostasis (congenital) can be associated with such causes:

• Increased production of lymph.
• Nonne-Milroy-Meizh syndrome (trophism of tissues).
• Shershevsky-Turner syndrome (chromosomal pathology).
• Anomalies in the development of lymphatic vessels (aplasia, dysplasia, hypoplasia, hyperplasia).
• Disorders of the central nervous system.
• Endocrine disorders.
• Insufficient valve device.

In 3-5% of cases, it is difficult to establish primary causes, so this form of the disease is called idiopathic. Congenital pathology very often leads to the defeat of both limbs.

[5], [6], [7], [8], [9], [10]

Risk factors

Elephant disease develops because of many causes associated with both congenital and acquired factors.

The occurrence of the disease can be influenced by such risk factors as:

• Oncological pathologies with lymphatic vessels.
• Chemotherapy or radiation exposure.
• Venereal diseases.
• Disturbances of blood circulation.
• Diseases of the hematopoietic system.
• Phlebeurysm.
• Surgical interventions with removal of lymph nodes.
• Systemic lupus erythematosus.
• Autoimmune pathologies.
• Parasitic infestations.
• Strong frostbite.
• Injuries of soft tissues of lower extremities.
• Overweight, obesity.
• Chronic eczema.

The aforementioned diseases are dangerous not only for the high risk of developing elephantiasis, but also for a significant disruption of the normal functioning of the body.

[11], [12], [13], [14], [15],

Pathogenesis

The lymphatic system participates in metabolic processes and purification of cells from toxins. It consists of vessels, nodes, trunks and capillaries. Free passage of fluid along the vascular bed provides normal lymph flow.

The mechanism of development of elephantiasis is associated with inadequate functions of the lymphatic system and a violation of the outflow of fluid. Normally, the tissues of the lower extremities daily synthesize about 2 liters of lymph, but with vascular blockages, stagnation occurs, which are manifested by persistent edema.

The pathogenesis of elephantiasis is based on the consistent development of such pathological changes:

• Impaired lymph drainage.
• Stagnation of fluid in tissues.
• Lymphogenous edema due to impregnation of tissues with proteins.
• Pathological restructuring of the lymphatic system.
• Fibrotic processes involving the dermis, subcutaneous tissue, fascia.

Infringement of a current of a liquid entails increase of an intralymphatic pressure and decrease in resorption (absorption). The tissues accumulate liquid and protein. Protein compounds break up and transform into fibrin fibers. Fibroblasts penetrate into the changed tissues and form collagen fibers. Against this background, serious disturbances occur in the cells of connective tissue.

Fibrous changes in elephantiasis capture the skin, the subcutaneous fat layer, muscles, fascia, the walls of arterial, venous and lymphatic vessels. Increasing edema worsens hemo and lymphodynamics. In the tissues accumulate metabolic products, there is hypoxia. This leads to a weakening of the protective properties of the immune system. There is a soft pastous edema. With its strengthening, the dermis becomes easily vulnerable. Against this background, soft tissue pathologies and trophic disorders develop. Rapid progression of lymphostasis leads to deformation of the damaged limb.

[16], [17], [18], [19], [20],

Symptoms of the elephantiasis

Signs of elephantiasis completely depend on the causes, type and stage of the pathological process. Symptoms of elephant legs arise as the disease progresses, we consider the main ones:

• Edema often appears only on one limb. One-sided lesion is typical for the acquired form of the disease. With congenital pathologies, lymphostasis of two legs is possible simultaneously.
• As the swelling increases, a feeling of raspiraniya in the limbs. Discomfort is accompanied by increased fatigue and a deterioration in overall well-being.
• Puffiness appears on the foot or hand, that is, below the affected area of the lymphatic vessels. Gradually, she turns to the ankle, and then to the thigh. Swelling is soft, so when pressing on the tissue appear pits.
• Violation of the outflow of lymph leads to the multiplication of pathogens in the lymphatic system and the thickness of the skin. Lymph nodes filtering lymph on the affected area, increase in size and become inflamed.
• The fluid in the intermuscular space and the subcutaneous fatty tissue are gradually replaced by a connective tissue. Because of this, the leg becomes hard to the touch, that is, the swelling solidifies. The skin is almost impossible to gather into a crease, and when pressing, there are no pits left.
• Persistent progression of the edema leads to deformity of the limb. On the ankle, all protuberances are smoothed, the leg acquires a cylindrical shape. The finiteness increases in volume several times.
• Violation of blood circulation entails atrophy of sebaceous and sweat glands (their secret protects the dermis from bacteria and viruses). Because of the violation of the protective layer, various eruptions appear on the skin, papillomas, warts, ulcers, ulcerous lesions, cracks. The presence of bacterial flora is dangerous by the development of allergic reactions.
• Excess volume of lymphatic fluid begins to be excreted through the skin. On the tissues fistula is formed, through which a yellowish liquid flows. Most often, the holes are localized in places with thin skin, that is, on interdigital folds.
• Because of the disturbed circulation in the tissues, a lot of melanin begins to be produced. On the extremities appear brown spots. Increased cell division leads to growth of formless tubercles and other growths, separated by transverse folds.

The above-described symptom complex can be accompanied by violations from other organs of the system, significantly worsening well-being.

[21], [22], [23],

Edema of the legs with elephantiasis

Lymphedema is a pathological condition with progressive edema of the soft tissues of the affected area. Edema of the legs with elephantiasis develops as a result of disturbance of the lymph flow through the lymphatic vessels. This pathology can be associated with both congenital and those acquired factors.

There are several types of lymphatic edema, consider them:

• Mechanical - appear after traumatization of tissues.
• Cachectic - are associated with cardiovascular pathologies and exhaustion of the body.
• Stagnant - increased capillary pressure, pronounced vascular permeability and decreased albumin level.
• Neuropathic - endocrine pathology, alcoholism.
• Hydramic - the accumulation of lymph is caused by kidney diseases.

With mild elephantiasis, edema disappears after a full rest and wearing compression linen. At moderate severity, persistent non-edema is observed with an increase in connective tissue. The skin becomes stretched, compacted. The patient complains of painful sensations and the extension of the legs, the general state of health worsens. There may be temporary convulsions and paresthesia.

Severe edema, that is, the last stage of elephantiasis, leads to irreversible lesions of lymph flow, fibrocystic changes in tissues. The limb is severely deformed and can not function normally. Because of this, contractures develop, deforming osteoarthritis, eczema, erysipelas, trophic ulcers. Another danger of persistent edema is an increased risk of lymphosarcoma.

[24], [25], [26], [27],

First signs

A feature of lymphedema is that at first its symptoms are so blurred that the patient does not attach much importance to it. In the evening on feet and ankles there is a small puffiness, which is often written off for fatigue during the day. Edema is especially noticeable in hot weather, after prolonged physical exertion and during the menstrual cycle. In this case, the joint retains normal mobility and pain in the leg does not arise.

The first signs of elephantiasis:

• Periodic swelling of one or two extremities.
• Edema is particularly noticeable at the end of the day, but completely disappears after an overnight rest.
• Puffiness increases with the vertical position of the limbs, after increased physical exertion and with limited mobility.
• At an early stage of irreversible tissue proliferation and other pathological changes does not occur.

In this case, the above described symptoms of the disease can persist for many years, flowing with a deterioration in overall well-being and weakness.

[28], [29], [30]

Stages

Symptom complex of elephantiasis of lower extremities has such stages:

1. At first, small swelling appears. They are associated with the growth of fibrous tissue and a violation of tissue metabolism. Edema begins with the foot and gradually passes above the knee to the thigh.

• Unsymmetric swelling on the extremities.
• Soft edema (after pressing on the skin remains a pit).
• Fabrics look very pale, easily shifted, but with difficulty folding.
• There are rashes and itchy skin.

The duration of the first stage is about 6-8 months.

2. Symptoms become more pronounced. The swollen area thickens and constantly increases in size.

• Larger lymphatic vessels are probed.
• Edema affects not only the feet, but the shin and thigh.
• Movement of the joints is limited.
• There are no painful sensations, but there is slight discomfort.
• The skin of the affected limb is strongly stretched and immovable.
• The tissues are very sensitive, even a slight pressure causes discomfort.
• The affected leg is significantly enlarged in size.

The second stage begins 2-7 years after the onset of lymphedema.

3. This stage is considered the most severe and incurable. The skin strongly coarsens, on it there are various neoplasms (warts, papillomas, blisters, ulcers). Affected limbs are deformed, they form wrinkles that impede movement.

• Thickening of the stratum corneum of the epidermis.
• Various neoplasms and cracks on the tissues.
• Rupture of lymphatic vessels, lymph flow through the fistula.
• Lymph nodes are enlarged, inflamed and very painful.
• The leg has a cylindrical shape and is increased 2-3 times in comparison with the healthy one.
• Blood poisoning.
• Atrophy of muscle tissue and cell death.

The third stage develops in 7-15 years from the moment of appearance of the first signs of the disease.

If the elephant leg is identified in the early stages, then medication in combination with physiotherapy can restore the patient's condition. The last stage can not be corrected. In this case, the treatment is aimed at alleviating the patient's morbid state.

[31], [32], [33]

Forms

Elephant legs arise because of a variety of different factors. The types of the disease depend on its etiology, therefore they distinguish such forms of lymphostasis:

1. Primary (idiopathic) - is associated with congenital functional disorders of the lymphatic system. Pathology occurs when:

• Hypoplasia of lymph nodes and vessels.
• Hyperplasia of the lymphatic tract.
• Insufficient valves.
• Lymphocsyaccording.

The first signs appear even in childhood, but as they grow older they become worse.

2. Secondary - is associated with traumatic injuries of the limbs, malfunctions in the lymphatic system and other pathological processes in the body. Can be of inflammatory and non-inflammatory origin.

Elephant legs has several types, based on the deformation of the limbs:

• I degree - edema and easy deformation of the foot.
• II degree - the pathological process passes to the foot and shin.
• III degree - there is a persistent edema of the foot, shin, thigh.
• IV degree - defeat of the foot, shin, thigh in combination with trophic disorders (cracks, papillomatosis, lymphorrhea).

The disease is also divided according to the age criterion. Isolate adolescent lymphostasis 15-30 years and later - after 30 years. According to the clinical course, there is a stable, slowly and rapidly progressing. By duration: acute, latent, transient and chronic elephantiasis.

[34], [35], [36], [37],

Complications and consequences

The human lymphatic system consists of nodes and vessels. Lymphatic vessels pass parallel to the blood vessels and flow into the lymph nodes, filtering out viruses, dying cells, bacteria and other pathogens. With lymphedema, the fluid does not move through the vessels, but accumulates in the tissues, causing persistent edema.

• The consequences and complications of elephantiasis in the first stage are associated with secondary skin infections. Against this background, very often deep vein thrombosis develops.
• In the second stage, because of the proliferation of connective tissue, the edema hardens, the tissues are tightened and painful sensations arise. If at this stage do not start treatment, then the elephant disease will progress, worsening the patient's quality of life.
• The sick limb is strongly deformed, therefore its functioning is broken. In addition to mobility problems, cosmetic defects are also observed. Due to impaired blood supply in the area of the edema, reddish areas are formed, which gradually turn into trophic ulcers.

In patients with chronic elephantiasis, which lasts more than 10 years, there is a risk of developing lymphangiosarcoma (lymphatic cancer). The prognosis of this complication is very poor, since even with amputation of the affected limb the risk of a lethal outcome is high enough. Infectious processes trigger another complication - sepsis, that is, blood infection.

[38], [39], [40], [41], [42], [43], [44], [45]

Diagnostics of the elephantiasis

The complex of diagnostic studies for elephantiasis largely depends on the causes of the disease. If elephant legs are caused by erysipelas, the diagnosis and treatment is infectious. When infecting with filaria, you should consult a parasitologist. All other cases are handled by a surgeon.

Diagnosis begins with the collection of anamnesis and questioning the patient:

• When the swelling began to appear.
• Are the tissues restored after a long rest.
• Whether the edema passes if the limb is in a raised position.
• Whether there are diseases of veins or erysipelatous inflammation.
• Recent visits to tropical countries (risk of infection with filarias).
• Whether swelling causes joint pain or impaired mobility.
• Presence of cardiovascular, renal or hepatic diseases.

The next stage is laboratory diagnosis: a clinical and biochemical analysis of blood and urine. In instrumental examinations, ultrasound examination of the veins of the lower limbs, abdominal organs and small pelvis is shown.

Also carried out magnetic resonance imaging, duplex scanning of the vessels of the extremities, X-ray studies. Differential diagnostics with diseases with similar symptoms is mandatory.

[46], [47], [48], [49], [50], [51],

Analyzes

Laboratory diagnosis of lower limb lymphostasis begins with a general blood test. The study is conducted to count all types of blood cells and their characteristics. The analysis is aimed at determining the level of eosinophils, albumin and the degree of coagulation of the blood. According to the results, the doctor can draw conclusions about the presence of inflammatory processes in the body.

Serologic examination of blood serum is also shown. This analysis is prescribed for suspected elephantiasis caused by infection with filaria. In this case, specific antibodies to parasites can be detected. Analyzes are carried out at all stages of treatment to monitor the patient's condition and the effectiveness of the prescribed therapy.

[52], [53], [54]

Instrumental diagnostics

To confirm lymphedema of the lower extremities, determine its type and stage, the patient is assigned a set of instrumental studies. Diagnosis consists of:

1. Radiography (angiography) - is performed to visualize changes in soft tissues. In the picture there may be signs of osteoporosis, thickening of the bone (the last stage of the disease), layering at the bone surface, deposition of potassium in parasitic invasions.
2. Ultrasound examination - reveals areas of constriction and blockage in the lymphatic vessels, the presence of thrombi, the destruction of valves in large vessels. Expanded varicose areas with disturbed lymphatic fluid flow can also be detected.
3. Magnetic resonance imaging - layered visualization of the cut of the affected limb. For elephantiasis is characterized by the presence of such symptoms:

• Constriction or blockage of the blood / lymph vessels.
• Varicose enlargement of lymphatic capillaries and their rupture.
• Strong growth of coarse fibers of connective tissue.
• Clusters of filaria in the lumen of the vessels and calcium deposits due to their death.
• Reduced density of subcutaneous fat (first stages).
• Germination of tissues with fibrous fibers (last stages).

4. Doppler of the vessels of the extremities - reveals the enlargement of lymph nodes and their puffiness, the presence of thrombophlebitis and varicose veins.
5. Thermography - the study of the affected limb is carried out with infrared radiation. The presence of lymphostasis is indicated by a decrease in the temperature of the diseased area in comparison with healthy tissues by 1.5 degrees, circulatory disorders. Also, there may be a local increase in temperature in the foci of inflammation.
6. Lymphoscintigraphy - a special drug is introduced into the lymphatic vessels, which confirms pathological changes. The disease is characterized by a slowing of the speed of the drug and its slow resolution in tissues.
7. Blister test of McClure-Aldrich - physiological saline is injected into diseased and healthy tissues so that a small blister forms on the skin. With elephantiasis, the defect disappears within 5-10 minutes, since the affected tissues have an increased ability to absorb liquid. While on a healthy leg the drug dissolves within an hour.

Based on the results of instrumental diagnosis, the doctor makes a treatment plan or appoints additional examinations.

Differential diagnosis

Despite the fact that the main symptom of elephant leg is an increase in the affected limbs in the volume, differential diagnosis of the disease can be significantly complicated. This is due to the fact that there are many other pathologies with a similar course.

Elephantiasis is differentiated with the lymphatic form of the arteriovenous fistula. This pathology is characterized by elongation and thickening of the limb, increased temperature and spots on the skin, increased oxygenation of venous blood.

With a common hemangiomatosis, the limbs have multiple swelling with a soft-elastic consistency. Edema is painful on palpation and causes discomfort during exercise. The skin is very thin and pigmented, their temperature is increased.

Mandatory lymphedema is compared with such diseases:

• Osteo-painful form of post-thrombophlebitic syndrome.
• Parks-Weber-Rubashov Syndrome.
• Clippel-Trenone syndrome.
• Gemangioma.
• Obesity.
• Tumor lesions of the extremities.
• Metastatic and traumatic lesions of the lymphatic tract.
• Hysterical edemas.
• Neurofibromatosis.
• Diseases of the cardiovascular system, kidneys.
• Erythromelalgia.

With obesity, diffuse lipomatosis of the legs is characterized by the appearance of a symmetrical edema of a soft consistency. Skin covers are not changed and are easy to assemble. Fingers and feet are normal size, but with pressure on the swelling there is soreness. The mechanism of the development of obesity is associated with violations of the CNS and endocrine glands.

Post-thrombotic disease is characterized by a mild painful edema, with a pressure on which appears a fossa. Tissues of a cyanotic shade, a network of enlarged subcutaneous veins is visible. When palpation of the muscles of the shin, there are sharp pains.

Elephant legs differentiate with myxedema. This disorder is a specific edema with the deposition of the mucinous substance in the tissues due to damage to the thyroid gland. Protein deposits disrupt the structure and elasticity of the skin. With thyrotoxicosis, the pathological process proceeds locally, affecting the pre-tyibe region.

Treatment of the elephantiasis

Restoration of normal lymph drainage is the main goal of treatment of lymphostasis. This can be achieved through an integrated approach that aims at:

• Enhance the protective properties of the immune system.
• Strengthening of the vascular walls.
• Acceleration of biological and metabolic processes.

Prevention

To prevent lymphostasis of the lower limbs, there is a set of preventive measures. Particular attention should be given to people at risk for developing the disease: patients with extensive injuries, burns and frostbite, who have undergone removal of lymph nodes, erysipelas or thrombophlebitis. People with obesity, severe sunburn and skin fungal infections.

Prophylactic recommendations:

• Comprehensive examination of the body to identify pathological factors that cause persistent edema.
• Observance of personal hygiene. It is necessary to thoroughly wash the lower extremities 2 times a day, wipe them well, wear socks, stockings or pantyhose from natural materials.
• Treat any tissue damage with antiseptics, for example a solution of brilliant green.
• Regular physical activity - gymnastics, swimming.
• Refusal from bad habits: alcoholism, abuse of sleeping pills or antidepressants, smoking.
• A balanced diet with a minimum amount of salt. Compliance with the drinking regime.

If on legs or foots the edemas often began to arise, it is necessary to address to the surgeon. Timely diagnosis and treatment will eliminate the disease at an early stage and avoid the development of serious complications.

[55], [56], [57],

Forecast

With timely diagnosis and treatment, elephantiasis has a favorable prognosis. Conservative therapy at an early stage of the disease prevents its further progression. A good prognosis is provided by surgical treatment in combination with medical and physiotherapy methods.

If the elephant leg is detected in late, rapidly progressing stages, then its prognosis deteriorates substantially. This is associated with a risk of complications, the most dangerous of which are oncological lesions of lymph nodes and sepsis.

[58],