Leprosy in our time. Leprosy (Hansen's disease, leprosy). Damage to the lymphatic system

Leprosy or leprosy (outdated name), hansenosis, hanseniasis, is an infectious disease that is caused by the mycobacteria Mycobacterium lepromatosis and Mycobacterium leprae and occurs with primary lesions of the skin, peripheral nerves and, sometimes, the upper respiratory tract, the anterior chamber of the eye, testicles, feet and brushes

Currently, leprosy has become a curable disease, because antibiotics can destroy its causative agent. In this article we will introduce you to the causes, symptoms and modern methods of treating this disease.

There are references to this disease in the Bible, manuscripts of Hippocrates and doctors of Ancient India, Egypt and China. Leprosy was called a mournful disease, because in those days it inevitably led to death. In the Middle Ages, quarantine places were opened for such doomed patients - leper colonies. There they said goodbye to life. Those around him also avoided the patient's relatives, because they were afraid of contracting leprosy.

According to the decree of the king in France, patients with leprosy were subjected to a “religious tribunal.” They were taken to the church, where everything was prepared for burial. After this, the patient was placed in a coffin, had a funeral service and was taken to the cemetery. After being lowered into the grave, saying the words: “You are not alive, you are dead to all of us,” and throwing several shovels of earth onto the coffin, the “dead man” was again taken out of the coffin and sent to the leper colony. After such a ceremony, he never returned to his home and did not see any of the family members. Officially, he was considered dead.

Now the prevalence of leprosy has decreased significantly, but the disease is still considered endemic (that is, found in a certain area and renewing itself after some time, and not due to importation from outside). It is usually detected among residents and tourists of tropical countries - Brazil, Nepal, India, the countries of the Western Pacific and East Africa. In Russia, a case of the disease was detected in 2015 in a worker from Tajikistan, who was employed at the construction site of a medical center.

Causes

The causative agent of leprosy is the mycobacteria Mycobacterium lepromatosis and Mycobacterium leprae. After infection, which occurs from a sick person to a healthy person through discharge from the nose and mouth or through frequent contact, a long time passes before the first signs of the disease appear. The incubation period for leprosy can range from six months to several decades (usually 3-5 years).

After this, the patient begins an equally long prodromal (latent) period, manifested in nonspecific symptoms that cannot contribute to the early detection of the disease. This fact, as well as the long incubation period, predisposes to its spread.

Symptoms and types of disease

When infected with leprosy, infection of areas of the body exposed to air cooling is usually observed - the skin, mucous membranes of the upper respiratory tract and superficial nerves. In the absence of timely and correct treatment, the disease causes severe skin infiltration and nerve destruction. In the future, these changes can cause complete deformation of the face, limbs and deformity.

Changes such as the death of fingers on the extremities are provoked not by pathogenic mycobacteria, but by secondary bacterial infections that occur during injuries, caused by loss of sensitivity in the arms and legs affected by leprosy. Such damage goes unnoticed, the patient does not seek medical help, and the infection leads to necrosis.

Leprosy can occur in the following forms:

• tuberculoid;
• lepromatous;
• dimorphic (or borderline);
• mixed (or undifferentiated).

All forms of leprosy have their own characteristic features, but experts also identify a number of common signs of this infectious disease:

• (up to low-grade fever);
• weakness;
• skin manifestations (light or dark spots with impaired sensitivity);
• the appearance of areas of skin infiltration;
• joint pain (especially during movement);
• drooping earlobes and the appearance of a fold between the eyebrows;
• loss of the outer third of the eyebrows;
• damage to the mucous membranes of the nose.

Tuberculoid leprosy

This form of leprosy appears as a hypopigmented spot with clear contours. With any physical impact on it, the patient feels hyperesthesia, that is, an increased sensation of the stimulus.

Over time, the spot increases in size, its edges become raised (in the form of ridges), and the center becomes sunken, undergoing atrophic changes. Its color can vary from bluish to stagnant red. A ring-shaped or spiral pattern appears along the edge.

Within such a spot there are no sweat and sebaceous glands, hair follicles and all sensations disappear. Near the lesion, thickened nerves can be felt. Their changes associated with the disease cause muscle atrophy, which is especially pronounced when the hands are affected. Often the disease leads to contractures not only of the hands, but also of the feet.

Any trauma and compression in the lesions (for example, wearing shoes, socks, clothes) lead to secondary infection and the appearance of neurotrophic ulcers. In some cases, this causes rejection (mutilation) of the phalanges of the fingers.

When the facial nerve is damaged, leprosy is accompanied by eye damage. The patient may develop logophthalmos (inability to completely close the eyelid). This consequence of the disease leads to the development of keratitis and ulcers on the cornea, which in the future can provoke the onset of blindness.

Lepromatous leprosy

This form of leprosy is more contagious than tuberculoid leprosy. It is expressed in the form of extensive and symmetrical skin lesions relative to the median axis of the body. The lesions appear in the form of plaques, spots, papules, lepromas (nodules). Their boundaries are blurred, and the center is convex and dense. The skin between the lesions thickens.

The first symptoms of the disease often include nosebleeds and difficulty breathing. Subsequently, obstruction of the nasal passages, hoarseness and laryngitis may develop. And when the nasal septum is perforated, the back of the nose is depressed in the patient (“saddle nose”). When the anterior chamber of the eye is infected with a pathogen, the patient develops iridocyclitis and keratitis.

Most often, such skin changes are observed on the face, ears, elbows, wrists, knees and buttocks. In this case, the patient feels weakness and numbness, which is caused by nerve damage. In addition to these characteristic symptoms, lepromatous leprosy causes loss of the outer third of the eyebrows. As the disease progresses, the patient experiences enlargement of the earlobes and a “lion face”, caused by thickening of the skin and expressed in distortion of facial expressions and features.

The disease is accompanied by a painless enlargement of the axillary and inguinal lymph nodes. In the later stages of lepromatous leprosy, decreased sensation in the legs appears.

In men, this form of leprosy can lead to development (inflammation of the mammary glands). In addition, compaction and sclerosis of testicular tissue causes the development of infertility.

Dimorphic (or borderline) form

This form of leprosy can combine in its clinical picture signs of tuberculoid and lepromatous leprosy.

Mixed (or undifferentiated) form

This form of leprosy is accompanied by severe nerve damage, most often the ulnar, peroneal and auricular nerves suffer from this process. As a result, loss of pain and tactile sensitivity develops. Violation of the trophism of the limbs leads to a gradual loss of ability to work and disability of the patient. When the nerves responsible for the innervation of the face are damaged, the patient’s diction is impaired and atrophy and paralysis of the facial muscles occurs.

In children, this form of leprosy can occur in the form. In such cases, red spots with torn edges appear on the body. They do not rise above the surface of the skin and are accompanied by weakness and an increase in temperature to low-grade fever.

Diagnostics

An important stage in making a diagnosis is a medical examination.

To diagnose leprosy, your doctor may use several techniques. In this case, an obligatory part of the patient’s interview is to establish places of stay and contacts over the past few years.

The examination plan for a patient with suspected leprosy includes:

• inspection and survey;
• scraping of the oral or nasal mucosa;
• Minor's test to detect decreased sweating in lesions;
• determination of skin sensitivity;
• nicotine test to detect skin reaction to nicotine;
• lepromin test (injection of a special drug into the skin of the forearm to identify the form of leprosy).

The most accessible and fastest specific method for diagnosing leprosy is the lepromin test. To perform it, the patient is injected intradermally in the forearm with Lepromin, a drug based on an autoclaved homogenate of skin lesions of patients with lepromatous leprosy. After 48 hours, a papule or spot appears on the skin, and after 14-28 days a tubercle (sometimes with an area of ​​necrosis) appears. The appearance of these signs is a positive result and indicates the tuberculoid form of leprosy, and if the result is negative, it indicates the lepromatous form or the absence of the disease.

Treatment

Treatment of patients with leprosy is always carried out in a specialized infectious diseases hospital, and after discharge they must undergo regular medical examinations. Its effectiveness largely depends on the timely initiation of therapy at the earliest stages of the disease, i.e., at the time the fact of possible infection is determined. However, in practice, patients more often seek medical help at those stages when symptoms begin to progress. This fact can lead to complications of the disease and its residual manifestations, such as changes in appearance and disability.

For etiotropic therapy aimed at destroying the pathogen, the patient is prescribed a combination of several effective antimicrobial drugs. Medication regimens may differ slightly in different countries.

The etiotropic drug therapy plan may include the following:

• drugs of the sulfonic group: Dapsone, Diaminodiphenylsulfone, Sulfetron, Sulfatine, etc.;
• antibacterial agents: Rifampicin (Rifampin), Ofloxacin, Minocycline, Clofazimine, Clarithromycin, etc.

The World Health Organization recommends combination therapy regimens for all forms of leprosy. For tubercoloid leprosy - Dapsone once a day and Rifampin once a month for six months, and for lepromatous leprosy - Clofasamine and Dapsone once a day and Rifampin once a month for 2 years until negative skin biopsy tests.

Etiotropic treatment is supplemented by taking Rutin, vitamin C, B vitamins and antihistamines (Suprastin, Loratadine, etc.).

In some cases, to inhibit the growth of mycobacteria and accelerate the regeneration of the skin, the patient is prescribed to take Chaulmugra oil (from chaulmugra seeds). This remedy should be taken in gelatin capsules, which protect the gastric mucosa from unwanted irritant effects.

Drugs for the etiotropic treatment of leprosy are taken for long periods (from several months to 2 years), and this contributes to their negative effect on blood composition. In patients, the level of red blood cells and hemoglobin decreases. To eliminate these signs of anemia, the patient needs to organize regular intake of vitamins and a balanced diet to replenish the lack of iron in the blood. In addition, he must take control blood tests at least once a month.

To exclude the development of complications from the visual, respiratory, nervous and musculoskeletal systems, consultations with such specialists are recommended.

Leprosy (or leprosy) is a disease caused by Hansen's bacillus, discovered by him in 1873.

Cellular lipids give different reactions in different people when they are introduced into the cell. This depends on the chemical composition of the lipids.

The bacillus is classified as a gram-positive bacillus, close to tuberculosis. The nervous system and skin are mainly affected. Less commonly, eyes and other organs. Sensation in the hands and feet is often lost. If treatment is not started in time, the face and limbs will become deformed. Death is rare.

Interesting. Among the lepers were historical figures: Louis XI, Henry XIV, Byzantine Emperor Constantine, impressionist Paul Gauguin.

In mild forms of leprosy, there is usually no need for isolation. In more serious cases, special measures are applied.

What types of leprosy is divided into?

There are three options for the development of the disease - depending on the type and degree of tissue damage (skin or nervous system), the possibility of cure or transition to the chronic stage.

Tuberculoid

Considered benign. Characterized by small lesions. In some places the skin turns white and gradually loses sensitivity.

Bacillus rods are not detected. The lepromin test is positive. In this form, the body's resistance is high. Treatment occurs quickly. After healing of the ulcerated areas, depigmented white scars remain in the form of spots.

Sometimes the development of leprosy leads to damage to peripheral nerves. This causes temperature disturbances and pain along the nerve. If the disease has progressed to the chronic stage, then during exacerbations in the rash areas, mycobacteria of leprosy are detected.

Note. Currently, about 11 million people on earth are affected by the bacillus, many of whom have had no skin contact with infected people. It is assumed that infection occurs through damaged skin or respiratory tract.

Lepromatous

This type is malignant. Large areas of rash on the skin. The kidneys, nasal mucosa, and genitals are affected. The face resembles a lion's muzzle.

The rash may be pigmented. They are symmetrically located, most often on the face, buttocks and bends of the limbs. While leprosy is gaining strength, vellus hair is still present.

But after 3-5 years, the loss of eyebrows and eyelashes begins. Erythema areas lose vellus hairs. The mustache and beard may disappear. The tissues of the nose and brow ridges grow. This symptom is characteristic and is called “lion face”.

At the site of infiltrates, nodules appear - lepromas. Leprosy affects the mucous membranes of the respiratory tract. The nose is deformed. The glottis narrows. The eyes suffer. The spread of lepromas continues in the lymph nodes, testicles and liver.

Note. In the Middle Ages, leprosy led to a slow, painful death. The duration of the disease was about 9 years, after which it ended in death. Neither medicine nor priests were able to alleviate the suffering of people.

Uncertain

Instead of the characteristic rashes, there are a few hyperemic spots with blurred boundaries. Hansen's wand is not detected. Histology gives a picture of chronic dermatosis. The patient's condition is good.

The peripheral nervous system is affected. Further development will be shown by a positive or negative reaction to the lepromin test. In the first case, only a tuberculoid scenario is possible, in the second - a lepromatous scenario.

The erased forms include Lucio leprosy. Visually, it resembles scleroderma and myxedema. Characterized by vasculitis, ulcerations, thrombosis of cutaneous blood vessels. Later leads to ichthyosis.

Leprosy: etiology, pathogenesis

It can take 2-3 months from infection with the bacillus to the first manifestations. up to 50 years old. Children and people suffering from chronic alcoholism and drug addiction are especially defenseless. With prolonged contact, they become infected much more often.

Therefore, the practice of immediately separating a newborn from a sick mother and transferring it to a child care facility or healthy relatives has been introduced. Then infection does not occur. Intrauterine infection was not recorded.

Important. Hansen's sticks are spread by airborne droplets (when the patient talks, they spread within a radius of 1-1.5 m) and from lepromas. Less often - with microcracks in the skin. There are known cases of infection during tattooing. Found in all biological fluids.

High natural resistance to leprosy has been observed in healthy people. Not everyone is susceptible to this disease, even under the most unfavorable circumstances.

For some, leprosy occurs as a latent infection.

Diagnostics

An infectious disease doctor prescribes serological, bacterioscopic, histological studies of lesions on the skin, and scrapings from the nasal mucosa.

Differentiate with toxicoderma, tertiary period of syphilis, exudative erythema, tuberculosis, leishmaniasis, systemic lupus erythematosus, dermatomyositis, sarcoidosis, scleroderma, urticaria pigmentosa, vitiligo, trophic ulcers, mycoses.

If nerves are damaged, it is necessary to exclude diseases with similar symptoms.

No misdiagnosis carries such serious consequences for a person as leprosy.

Consultations with other specialists

If there are neurological and amyotrophic manifestations, osteomyelitis, pathology of the eyes and respiratory organs, patients are prescribed consultations with a dermatologist, neurologist, surgeon, ophthalmologist, and otorhinolaryngologist.

Leprosy treatment

The first anti-leprosy drug, developed in 1950, was slow-acting and became increasingly less effective as the bacillus developed resistance to it.

New treatments have emerged, and since the early 1980s, holistic therapy has been the accepted treatment option for leprosy worldwide. This method includes the use of Dapsone, Rifampicin and Clofazimine.

Fact. Complex therapy turned out to be very effective. As a result of its use, the number of leprosy patients decreased from 12 million in 1985 to about 1.3 million in mid-1996.

On a note. In some patients, even 5-10 years after chemotherapy, Hansen's bacillus continues to be detected. Prescribe iron supplements to prevent anemia. Sometimes patients have to take medications for life - unexplained relapses periodically occur.

Physiotherapy is prescribed as soon as the diagnosis is made.

Orthopedic measures are indicated to correct impaired body functions. Modulan also came to the aid of the sick. An extension of the hand is made from it or the desired tool is formed. Patients can hold cutlery and tools, even a hammer.

Is there a great danger of contracting leprosy? Prevention

Lepers have been isolated from healthy people throughout the ages. The Bible gives detailed instructions on the definition of leprosy, quarantine, etc.

Fact. Fear of disease led to cruel laws. This included placing the patient in a freshly dug grave, publicly disowning him, declaring him dead, and lifting him upstairs to be placed in an isolated area. It was allowed to leave after putting on a special robe with slits for the eyes with bells. It seemed as if death itself was coming.

Columns of lepers dressed like this walked throughout medieval Europe. The consequences of the Crusades were an extraordinary spread of leprosy.

In the 20th century, the world's scientists united against leprosy, and leprosy became a controlled infection.

In Russia there are two leper colonies, an immunological laboratory and a leprosy research institute. Cases of infection have become isolated - mostly relatives of patients.

In the fight against leprosy, the practice of preventive measures has developed:

1. Strict registration of patients.
2. Placing them in a leper colony.
3. Medical examination of the patient's family members.
4. Preventative examination of all residents of the locality.
5. Prescribing Dapsone to contact persons.
6. Administration of the BCG vaccine upon contact with patients with a highly contagious form and administration of Lepromin to them.
7. A ban on releasing the bodies of deceased patients to relatives. Instead, they are buried on the territory of the leper colony.

The project involves creating a test to determine predisposition to leprosy.

Interesting. Some doctors performed real feats, infecting themselves and experimenting with cures. It was the same with leprosy, but the researchers were unable to achieve infection. The Norwegian Danielsen and his colleagues injected themselves with the blood and pus of the patient. Even implantation of leproma under the skin did not lead to the disease. Later, the non-viability of rods visible through a microscope was established.

In 1960, an American doctor was able to infect experimental mice, but this did not advance the fight against the disease. Then evidence was obtained of the life of the infection in the environment: soil, water, the body of one type of armadillo and chimpanzee.

Note. Leprosy is now found in the warm climates of Africa, Asia and South America. The further north people live, the fewer episodes of leprosy are recorded. In the United States, 100 new cases are diagnosed annually. The top three countries for leading the disease are India, Brazil and Burma. In Russia, only 11 cases have been identified over the past 10 years.

While this ancient disease still exists, the best preventative measure remains to improve socio-economic and sanitary conditions of life.

Few diseases have such a dismal reputation as leprosy. Firstly, it disfigures people not only severely, but also in a very diverse way, often causing aesthetic shock. Secondly, before the invention of specific chemotherapy in 1943, leprosy was practically incurable. Third, the causes of leprosy have long been mysterious. This disease was specially invented to create the impression of an unpredictable “punishment of the Lord”: it affects people very selectively and, moreover, has a huge incubation period. Until the end of the 19th century, there were serious discussions among doctors about whether leprosy was contagious at all and whether it was caused, for example, by eating fish.

The Greek word for leprosy, “leprosy” (λέπρα), came into scientific use in the 3rd century BC, after the famous seventy interpreters in Alexandria of Egypt translated the Old Testament into Greek. But, of course, this disease was known to people before. In some countries it allows it to forget about itself for a long time, in others it runs rampant. At the beginning of the 20th century, on the eastern outskirts of the Belgian Congo there was a fairly extensive area where 20% of the population, that is, every fifth ( Transactions of the Royal Society of Tropical Medicine and Hygiene, 1923, 16, 8, 440–464). And in West Africa (French Guinea) at one time there was an area where even 32% were affected - every third ( Annales de médecine et de pharmacie coloniales, 1920, 18, 109–137). These numbers are hard to believe, but they are in the literature.

Leprosy is a complex phenomenon. It can be the object of study in a variety of sciences, from molecular biology to cultural studies - just remember books such as “The Name of the Rose” by Umberto Eco or “The History of Madness in the Classical Age” by Michel Foucault.

However, knowing that we live in an evolving world, it is natural to ask the following question: where did leprosy come from? Or, more precisely: where and when did it originate?

Genomics and deduction

“On the Origin of Leprosy” is the title of an article published in 2005 by an international group of microbiologists and geneticists led by Marc Monod, an employee of the famous Pasteur Institute in Paris ( Science, 2005, 308, 5724, 1040–1042). The causative agent of leprosy is a non-motile bacterium, close to the tuberculosis bacillus (they belong to the same genus). In Latin this bacterium is called Mycobacterium leprae. It was discovered by the Norwegian Gerhard Hansen and the German Albert Neisser back in the 70s of the 19th century. And by the beginning of the 21st century, it had been studied well enough to try to solve the question of the origin of leprosy using comparative genomics methods. This is exactly what the Mono group took on.

The genome of the causative agent of leprosy was first fully read in 2001. It is quite small, even by the standards of bacterial genomes, which are always small. This genome has undoubtedly undergone evolution towards simplification: it is not without reason that a significant part of the genes in it have turned into pseudogenes (the so-called non-functional former genes that have survived, but have lost the ability to perform any activity). In addition, comparisons between different populations M. leprae shows that the intraspecific variability of its genome is very low, it is extremely stable in space and time. Finding variable regions in such a genome, from comparisons of which one can draw at least some evolutionary conclusions, turned out to be not so easy.

Recognizing this, Monod's group focused on the most basic components of genetic variation: single-nucleotide polymorphisms (SNPs), which were found in non-coding regions of the genome. Let us remember that nucleotides are individual “letters” of the genetic code. DNA contains only four types of nucleotides, distinguished by a specific functional group, which can be adenine (A), thymine (T), guanine (G) or cytosine (C). Nucleotide substitutions in non-coding regions of the genome do not affect the structure of proteins and therefore can accumulate relatively easily. But in the case of the genome of the causative agent of leprosy, even in such regions, the researchers were able to select only three variable loci for analysis (in Latin, this term simply means “place”).

Well, even meager material can often reveal something important if the deductive method is used correctly. Let us have three single nucleotide loci. How many types of nucleotides are possible at each locus? That's right, four: A, T, G or C. This means that the total number of possible combinations here is 64 (4 to the third power).

The first valuable information the researchers obtained was that in real populations M. leprae out of 64 potentially possible combinations, only four are present: C-G-A, C-T-A, C-T-C and T-T-C. This greatly simplifies the system being studied. All that remains is to understand from which combination all the others originated.

Four lines correspond to hypotheses about the primitiveness of any of the four genetic types of the causative agent of leprosy. In cells shows the number of replacements that would be needed to make each actual type (four columns) from the original one. On right the number of required replacements is summed for all types. The fewer replacements, the more plausible the hypothesis about the primitiveness of this option" border="0">

This is where the deductive method comes in handy. First of all, we see that in three of the four options, C is in the first position (see table). Modern evolutionary studies (especially molecular ones) have adopted the so-called parsimony principle, according to which, other things being equal, one should always choose the version that requires the fewest assumptions about independent events. In this case, this means that C in the first position should be considered a primitive state (it is easy to see that any other version will require the postulation of additional substitutions). Thus, the fourth genetic type, T-T-C, is so far excluded from the candidates for the role of the most ancient.

In the second position, in three out of four options there is T. Similarly, it should be assumed that this state is primitive. Then the first genetic type (C-G-A) is also excluded from the candidates for the role of the most ancient.

This means that the most ancient genetic type of the causative agent of leprosy had C in the first position and T in the second. But C-T-A or C-T-C? The primitiveness of both options is equally probable. The resolution of a purely genetic approach is exhausted here.

However, any evolution occurs not only in the abstract space of genotypes, but also in the ordinary geographical one. Important additional information can be obtained by overlaying genetic types on a world map. Fortunately, Mono’s group obtained samples of bacteria from various countries on Earth.

For convenience, genetic types M. leprae were color coded. The first type (C-G-A) is “yellow”, the second (C-T-A) is “red”, the third (C-T-C) is “purple” and the fourth (T-T-C) is “ green". Judging by genetic considerations, the “red” and “purple” types can equally likely claim the role of the most ancient. Now let's see what their geographic distribution tells us.

Genomics meets geography

First, we give a dry summary of the data obtained.

“Yellow” type: East Africa (southern part), Madagascar, India, Korea, Malaysia, Philippines.

“Red” type: East Africa (Ethiopia, Malawi), Nepal, northeast India.

“Purple” type: North Africa (Morocco), Western Europe, most of the Americas.

“Green” type: West Africa (sub-Saharan), Caribbean islands, Brazil.

There are three types in New Caledonia (“yellow”, “red” and “purple”), but this is a clear consequence of the settlement of the island by different ethnic groups during the colonial period, and therefore we can not be distracted by it.

Which type is the most ancient? If you choose between “red” and “purple” types, then, of course, “red” is preferable. European leprosy is definitely less ancient (for example, in Italy it was completely unknown during the time of Emperor Augustus, that is, at the turn of our era). And in Africa, the “purple” type is found only north of the Sahara, for example in Morocco, where the connection with Europe is relatively close. But the range of the “red” type covers the entire East Africa. So this is the birthplace of leprosy? Quite possible.

True, there is also a hypothesis of the Asian origin of leprosy, which Monod and his co-authors also did not immediately completely reject. But from a genetic point of view, this version is less likely: it involves at least one additional nucleotide substitution. Most likely, the original type was not the “yellow” (Asian) type, but the “red” one. This means that leprosy originated in the same place as the species Homo sapiens: deep in East Africa.

From Africa, leprosy came first of all to the Middle East, and then it had two routes - to Europe or to Asia. Migration towards Europe gave rise to the “purple” type, migration towards Asia - “yellow”. The breeding ground for the latter was primarily the ancient states of the Indian subcontinent and China. There simply weren’t such numbers of people in Europe for a long time, and the conditions for the survival of lepers there were harsher.

It is interesting that the island of the “red” - East African - type is noted precisely on the Indian subcontinent (Nepal, northeast India). Perhaps this is a relic left over from the original migration.

On the other hand, the “yellow” - Asian - line of leprosy has also been found in Africa. But what kind of Africa is this? This is Madagascar and the southern part of East Africa, located approximately opposite it. The current indigenous inhabitants of Madagascar - the Malagasy - are known to be descendants of Indonesians. And in the southern part of East Africa there are old ports focused on trade with Asia - Malindi, Mombasa, Zanzibar. There is no doubt that leprosy was brought here from Asia, through the Indian Ocean.

The fate of the “green” line of leprosy is very interesting. It is genetically very distant from the supposed original “red” type, and its distribution is limited to Western Africa south of the Sahara. How did she get there? Perhaps through ancient continental migrations across Africa from east to west. This continent is not particularly convenient for long-distance travel, so the isolation is understandable. Or maybe the Phoenicians, who sailed on ships along the African coast of the Atlantic, at one time brought leprosy there from the Mediterranean (here one can recall Ivan Efremov’s novel “On the Edge of the Oikumene,” which describes just such travels). This version is indirectly supported by the fact that the “green” type of leprosy pathogen is genetically closer not to “red”, but to “purple” - for the Mediterranean, as well as for Europe, it is the latter that is characteristic.

On the American continents, leprosy is mostly “purple,” which looks completely natural: America was colonized by Europeans, after all. The “green” type of leprosy is found in the Antilles and Brazil, but this is clearly explained by the Atlantic slave trade - slaves were at one time brought mainly from West Africa.

It is noteworthy that immigrants from Europe seem to have managed to infect nine-banded armadillos, which are widespread in South, Central and North America, with leprosy. Dasypus novemcinctus. The nine-banded armadillo is almost the only species other than humans susceptible to this disease. Even natural outbreaks have formed in the southern United States and Mexico. So, armadillos have a genetic type M. leprae- “purple”, exactly as one would expect, based on the fact that leprosy was brought to America by Europeans.

There are still a lot of questions here. But one way or another, we have before us a coherent evolutionary scenario.

...And with archeology

The scheme for the evolution of the causative agent of leprosy, proposed by Monod's group, is beautifully reminiscent of Sherlock Holmes' solution to the problem with the dancing men. It goes without saying that the research didn't stop there. A few years later, the same group published a clarifying paper in which four genetic types M. leprae already divided into 16 subtypes ( Nature Genetics, 2009, 41, 12, 1282–1289). There is nothing fundamentally changing the picture, but there are interesting details. For example, DNA M. leprae, discovered in a leprosy skeleton from Egypt approximately 1,500 years old, turned out to belong not to the “red” type (as one might think), but to the “purple” type. It's the same in Turkey. It turns out that the “purple” type range covers the entire Mediterranean in a ring. In the exchange of leprosy pathogens between the Middle East and Europe - during the Crusades, for example - only the “purple” line of the microbe participated.

As for the “yellow” line, it apparently initially penetrated from Africa to Asia not through the land bridge between them (as, again, it would be easy to think), but by some other route. If the "purple" type M. leprae moved from Egypt through Sinai, Palestine and Syria, then the “yellow” - straight from the Somali Peninsula along the northern coast of the Indian Ocean. Along the Great Arc, as Efremov’s heroes would put it.

Here, however, there is reason to think.

Almost simultaneously with the publication of a new article by Monod’s group, data appeared on the findings of leprosy skeletons in India dating back as far as 2000 years BC ( PloS One, 2009, 4, 5, e5669, see photo). There is no molecular evidence, but the anatomical (more precisely, osteological) looks impressive. It is quite natural that the authors of this discovery questioned the hypothesis of Monod’s group, suggesting that the original type of leprosy pathogen was not “red” (African), but “yellow” (Asian). As we remember, the Mono group itself did not completely reject this version. But what is most interesting: the place where these skeletons were found is not just India, but Western India. This is the area of ​​​​the ancient civilization of the Indus Valley, the same one where the famous disappeared cities of Mohenjo-Daro and Harappa were located. The Sumerians and Akkadians called this country Melukha (History of the Ancient East. Edited by B.S. Lyapustin M., 2009).

At this point, the authors of the Indian discovery talk about the existence in the 2-3 millennia BC of the so-called single sphere of interaction, which included Mesopotamia, Turan, Meluhha and the kingdom of Magan on the Arabian Peninsula. Wherever leprosy originated, it is certain that it spread throughout this area. Urban civilizations were a breeding ground for it.

But from which direction did she come? Alas, there are purely genetic data that force us to reject the hypothesis of the origin of leprosy from India.

"Forever and ever"

One recent work estimates: about 10 million years ago ( PLoS Neglected Tropical Diseases, 2014, 8, 2, e2544). That's a lot! The oldest supposedly upright relative of humans, Sahelanthropus, lived only 6–7 million years ago. And 10 million years ago, our upright posture was just beginning to take shape. And in any case, all the initial stages of human evolution took place in Africa. If the causative agent of leprosy is so ancient, then it could only appear there.

It is known that many infectious diseases were in one way or another acquired by man from animals with whom he had to come into contact ( Nature, 2007, 447, 7142, 279–283). About tuberculosis, which is also caused by a microorganism of the genus Mycobacterium, there is a hypothesis that people got it from ruminant mammals. There is, however, a counter-opinion that this is a very ancient purely human infection that infected ruminants for the second time ( PLoS Pathogens, 2005, 1, 1, e5). There are no such disputes about leprosy, because there are no serious grounds for them. This is a human disease. True, armadillos and very rarely (literally in isolated cases) chimpanzees, as well as some other African monkeys, also suffer from leprosy. But it looks like they all got leprosy, again from people. A leper chimpanzee brought from West Africa has the genetic type M. leprae turned out to be “green”, that is, exactly the one that is common among the local residents ( Future Microbiology, 2011, 6, 10, 1151–1157).

So, leprosy is a specific disease of people. Given its antiquity, it is better to say not “people”, but “hominids” (in the narrow sense of the word, upright primates). What features of their - our - way of life determined its existence?

The great anthropologist Owen Lovejoy associates the emergence of upright walking with a new reproductive strategy that allowed hominids to dramatically increase the size of their populations. With this strategy, females spend most of their lives in a small, safe “nesting zone”, caring for children (they need upright posture to free their hands for this work). Males, not constrained by cubs and females, can greatly expand their territory, making long and risky foraging trips. The new structure of society has created new opportunities, but also new risks. In a herd of monkeys, the likelihood of survival of individuals affected by a severe, slow infection is likely to be low. But in the hominid space, clearly divided into a “nesting zone” (where females live), a foraging and hunting zone (where males go) and the outer absolutely wild world, here the lepers were able to find for themselves at least a gloomy and uncomfortable, but still niche.

“In Bruegel, the lepers are watching the ascent to Calvary, where all the people follow Christ, from afar: this is their place forever and ever,” wrote Michel Foucault. He did not yet know that this “forever and ever” may be measured in millions of years. Leprosy is an ancient shadow of human society. It’s even scary to imagine how ancient it is. One of those products of evolution that you most want to get rid of. Fortunately, modern treatments finally make it possible to do this.

Leprosy is a severe chronic infectious pathology caused by the mycobacterium Mycobacterium leprae hominis. The source of infection is a person with leprosy. Synonyms: leprosy, Hansen's disease, Phoenician anomaly, St. Lazarus disease and others.

There are approximately 11,000,000 people suffering from leprosy worldwide. From a medical and social point of view, leprosy is a serious disease.

Leprosy has been known since ancient times. It was mainly suffered in the countries of the Middle East. Even then, separate closed settlements were built for the sick, where the sick slowly died. There is a well-known case from the New Testament when Jesus healed a whole group of leper patients.

The prejudiced attitude of society towards patients with leprosy, who are subject to complete physical and social isolation, greatly complicates the problem of identifying cases of the disease and combating it. To this should be added the chronic nature of the disease and the lack of confidence that even after prolonged treatment it is possible to achieve complete liberation of the body from mycobacteria.

WHO drew attention to the problem of leprosy in the first decades of its existence. She was inspired by Raoul Follero, who dedicated himself to the fight against leprosy. In honor of Follero, WHO registered back in 1954, January 30, World Leprosy Day. This day should attract the attention of the international community to a comprehensive review and thorough understanding of the problem of leprosy in the world and the possibility of helping such patients.

Etiology, epidemiology of leprosy (leprosy)

Currently, leprosy is widespread in Africa, Southeast Asia, South America and Oceania. Extremely rare - in Europe and North America. The disease can develop at any age and has no racial restrictions. The concentration of leprosy patients in economically undeveloped countries and its connection with overpopulation is noted quite often. Exogenous high-risk factors play an important role in the spread of leprosy.

The exact method of transmission of the disease is unknown, but long-term observations of patients indicate infection through constant contact of a healthy person with a leprosy patient. There is no evidence regarding the transmission of the disease to humans from rodents, fleas, insects and others. An important factor in persistent infection, as well as the inability to trace the source of infection in reported cases, is the fact that asymptomatic contacts can spread mycobacteria from the nasal cavity long before they are diagnosed with leprosy. It is worth noting that mycobacteria leprosy can spread from lepromatous ulcers of treated patients, from mother's milk, and from skin appendages. Lepromatous mycobacteria can probably be transmitted by airborne droplets. They are in soil and water.

Symptoms, diagnosis of leprosy (leprosy)

The disease has a long incubation period, which can last from six months to several decades, more often – 5-7 years. It is asymptomatic. A long latent period is also possible, manifested mainly in general malaise, causeless weakness, chilliness, etc.

There are two polar forms (types) of leprosy - lepromatous and tuberculoid, as well asfour stages of the disease: progressive, stationary, regressive and the stage of residual effects. In addition, intermediate or dimorphic leprosy is possible.

Tuberculoid leprosy

Tuberculoid leprosy usually begins with the appearance of a clearly defined hypopigmented spot, within which hyperesthesia is noted. Subsequently, the spot enlarges, its edges rise, become roll-shaped with a ring-shaped or spiral pattern. The central part of the spot undergoes atrophy and sinks. Within this lesion, the skin is devoid of sensitivity, there are no sweat glands and hair follicles. Near the spot, thickened nerves innervating the affected areas are usually palpated. Nerve damage leads to muscle atrophy; The muscles of the hand are especially affected. Contractures of the hands and feet are common. Injuries and pressure lead to infections of the hands and feet, and neurotrophic ulcers form on the soles. In the future, mutilation of the phalanges is possible. When the facial nerve is damaged, lagophthalmos and resulting keratitis occur, as well as corneal ulcers, leading to blindness.

Lepromatous leprosy

Lepromatous leprosy is usually accompanied by extensive skin lesions that are symmetrical relative to the midline of the body. Lesions can be represented by spots, plaques, papules, nodes (lepromas). They have vague borders and a dense and convex center. The skin between the elements is thickened. The most commonly affected areas are the face, ears, wrists, elbows, buttocks and knees. A characteristic sign is loss of the outer third of the eyebrows. Late stages of the disease are characterized by so-called “lion face” (distortion of facial features and impaired facial expressions due to thickening of the skin), enlargement of the earlobes. The first symptoms of the disease are often nasal congestion, nosebleeds, and difficulty breathing. Complete obstruction of the nasal passages, laryngitis, and hoarseness are possible. Perforation of the nasal septum and deformation of the cartilages lead to retraction of the nasal bridge (saddle nose). Penetration of the pathogen into the anterior chamber of the eye leads to keratitis and iridocyclitis. The inguinal and axillary lymph nodes are enlarged, but not painful. In men, infiltration and sclerosis of testicular tissue lead to infertility. Gynecomastia often develops. Late stages of the disease are characterized by hypoesthesia of the peripheral limbs. Skin biopsy reveals diffuse granulomatous inflammation.

Immunity in leprosy is cellular in nature, it is maximum in patients with tuberculoid leprosy and minimal in lepromatous form. To assess the immune response and differential diagnosis between the two forms of the disease, the lepromin test is used. The reaction to an intradermally administered suspension of mycobacterium leprosy is positive in the tuberculoid form and negative in the lepromatous form.

Leprosy can be diagnosed by the presence of clinical symptoms of the disease. Confirmatory research methods are bacterioscopic and histological.

Treatment and prevention of leprosy (leprosy)

Treatment is a long-term course (up to 3-3.5 years) with the prescription of anti-leprosy drugs of the sulfone group (diaphenylsulfone, solusulfone, diucifone, etc.). The duration of the course is 6 months, the break in treatment is 1 month. Multibacterial leprosy requires initial administration of rifampicin, dapsone or clofazimine, after which switching to sulfone group drugs. Evaluation of the effectiveness of treatment is controlled by bacterioscopic and histological research methods. Currently, there are 4 leper colonies in Russia (a place for detection, treatment, isolation, and prevention of leprosy): in Astrakhan, Krasnodar Territory, Sergiev Posad District of the Moscow Region, Stavropol Territory.

The main problem of WHO is the fight against leprosy at the level of primary prevention. Today, the main goal should be early diagnosis and effective drug therapy. Secondary prevention measures - identifying cases of the disease - are also important. This can be achieved through primary health care with the active participation of the entire population of a country where cases of leprosy have been reported. In places where leprosy is endemic, mass surveys of the population, sanitary and educational work among the population and doctors are carried out. In addition to the epidemiological situation, socio-economic factors are of great importance, which explains the widespread spread of the disease among the poorest populations in Asia and Africa. The health systems of these countries prioritize expanding services to identify and treat leprosy patients and ensure that modern treatment is available to all patients. Prevention of leprosy among medical personnel and other persons who, by the nature of their work, come into contact with patients, consists of strict adherence to sanitary and hygienic rules (frequent hand washing with soap, mandatory sanitation of microtraumas, etc.). Cases of infection of medical personnel are rare.

Leprosy (leprosy) is an infectious disease that affects the skin and peripheral nervous system of humans. The disease leprosy is considered one of the oldest diseases, mentions of which are found in the Old Testament. In those days, people with leprosy were considered “unclean.” Healthy people shunned them, they were persecuted and deprived of the right to a normal life. The peak incidence of leprosy occurred in the 12th-14th centuries, when the infection affected the population of almost all European countries.

To combat leprosy, medieval aesculapians used numerous leper colonies - institutions that identified and treated lepers. Initially, leprosy patients were located on the territory of monasteries, where they were allocated houses and plots for agricultural activities. In fact, the unfortunate people lived in a kind of reservations and did not have the opportunity to communicate with the rest of the world. However, then the isolation of leprosy patients was completely justified and bore fruit. By the 16th century, leprosy had disappeared from Europe. Isolated cases of the disease were recorded for some time on the Mediterranean coast and Scandinavia, but large-scale epidemics never developed.

Today we know almost everything about leprosy. Contrary to popular belief, the infection is not transmitted by simply touching a patient and does not always lead to death. It is known that the disease leprosy threatens only 5-7% of people, and the remaining inhabitants of the Earth have stable immunological protection against the pathogen. As for the method of transmission of infection. In most cases, infection requires prolonged direct skin contact. There is also a theory that leprosy, the symptoms of which can appear 10 years after the infection, enters the body by inhaling bacteria secreted from the mouth or nasal cavity of a sick person. Perhaps it is this assumption that partly explains the fact that today there are about 11 million leprosy patients registered in the world and many of them have not had any skin contact with infected people.

What causes leprosy?

The disease leprosy is caused by rod-shaped microorganisms - Mycobacterium leprae. They were discovered in 1874 by the scientist G. Hansen. These microorganisms have properties close to those of tuberculosis, but do not have the ability to multiply in nutrient media and often do not show themselves in any way for many years. Suffice it to say that the incubation period of the disease is often 15-20 years, which is due to the characteristic features of leprosy. By itself, it is not capable of causing tissue necrosis. This means that the activity of microorganisms must be activated by some external factors, for example, secondary bacterial infection, poor diet, contaminated water or poor living conditions.

A long incubation period and an equally long latent period often lead to the fact that when leprosy is diagnosed, treatment begins too late, since doctors experience objective problems with the early diagnosis of the disease.

Currently, experts know two forms of leprosy:

• lepromatous - the pathogen affects mainly the skin;
• tuberculoid - for the most part the disease affects the peripheral nervous system.

There is also a borderline form of leprosy, which tends to develop into one of the two main types of the disease.

Leprosy symptoms

The tuberculoid form has the following characteristic symptoms of leprosy:

• the appearance of a clearly defined spot, which gradually increases in size;
• absence of hair follicles and sweat glands on the affected skin surface;
• thickened nerves can be clearly felt near the spot;
• amyotrophy;
• formation of neurotrophic ulcers on the soles;
• contractures of the hands and feet.

As the leprosy disease progresses, the symptoms of the disease also increase. Over time, patients develop phalangeal mutilation, corneal ulcers and other lesions of the facial nerve, leading to blindness.

Lepromatous leprosy manifests itself as extensive skin lesions in the form of plaques, papules, spots and nodules. As a rule, such formations appear on the face, ears, elbows, wrists and buttocks. Very often, leprosy is accompanied by loss of eyebrows. Late stages of the disease are characterized by distortion of facial features, enlargement of the earlobes, nosebleeds, and difficulty breathing. Leprosy patients also suffer from laryngitis, hoarseness and keratitis. Infiltration of pathogens into testicular tissue leads to infertility in men.

Treatment of leprosy

For several centuries, chaulmugra oil has been used against the disease leprosy, however, modern medicine has much more effective means, in particular sulfone drugs. They are not specific therapeutic agents, but can stop the development of infection and have a general strengthening effect on the body.

In mild forms of the disease, cure occurs within 2-3 years. Severe leprosy increases this period to 7-8 years. We also add that recently strains of lepta bacteria have been discovered that are resistant to dapsone (the main drug used in modern medicine), so in recent years sulfamine drugs have been used in combination with other drugs. For example, for the lepromatous type of infection, clofamizine is widely used.

Of course, researchers are not going to stop there and are looking for more effective ways to combat leprosy that will reduce treatment time and reduce the severity of symptoms in severely ill patients.

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