What is Leprechaun?
Leprosy (Hansen’s disease, hansenoz, hanseniaz; outdated name – leprosy, elephantiasis graecorum, lepra arabum, lepra orientalis, Phoenician disease, satyriasis, sorrowful disease Krymka lazy death, St. Lazarus disease and others.) – Chronic granulomatosis (chronic infectious disease ), caused by Mycobacterium Mycobacterium leprae and discovered in 2008 Mycobacterium lepromatosis, occurring with a primary lesion of the skin, peripheral nervous system, sometimes the anterior chamber of the eye, upper respiratory tract above the larynx, testicles, and hands and feet.
If the etiology of leprosy is known, the pathogenesis of the disease, its epidemiology, the conditions of infection and spread are not fully understood.
The largest endemic leprosy foci exist in Karakalpakia, Kazakhstan, the Lower Volga region, the North Caucasus, the Far East, and the Baltic states. Occasional sporadic cases of leprosy are detected in various places. In total, there are a relatively small number of patients in our country, whose treatment is carried out mainly in leprosarium. An important role is played by timely isolation and effective treatment of patients, identification, treatment of sources of infection and examination of all contacts with the patient, especially household. Worldwide, according to incomplete data, there are more than 10 million patients. In Ukraine, leprosariums are organized to isolate and fully cure patients with leprosy and return them to normal life.
Causes of Leprosy
The causative agent of leprosy is bacilli, discovered in 1871 by the Norwegian physician G.Hansen, of the family Mycobacteriacea, called Mycobacterium Leprae hominis. Bacillus leprosy is a gram-positive alcohol-and acid-resistant bacillus, stained by Ziehl-Nielsen, Ehrlich and aniline dyes. In recent years, for bacterioscopic diagnosis of leprosy, Marcinovsky’s staining method has been used. In the process of existence, the mycobacterium leprosy forms a-forms, granular and filterable forms. In biopsy specimens, mycobacteria are found in significant numbers in the form of sticks with slightly pointed ends, with a tendency to parallel arrangement in the form of “cigar packs” or clusters in the form of balls surrounded by a transparent gelatin-like shell. Mycobacterium leprosy does not have a capsule and does not form a spore. Attempts to obtain a pure pathogenic culture of mycobacteria on an artificial nutrient medium have not yet succeeded, it is possible to vaccinate leprosy to armadillosis and some experimental animals (rats, mice) irradiated with X-rays and prepared corticosteroid hormones.
Epidemiology. Socio-economic factors are of major importance, which explains the particularly wide spread of the disease among the poorest people in Asian countries.
A person becomes infected from a patient as a result of prolonged contact, which should be accompanied by sensitization, which progresses with repeated inoculation. Particularly high risk of infection in childhood, but even in this case, children who are prone to allergic diseases have a greater risk. Mycobacterium leprosy is secreted from the patient through the mucous membranes of the respiratory tract or from ulcerated lepromas. Especially a lot of mycobacterium leprosy is found in the nasal mucus and the discharge of mucous membranes of the pharynx, larynx. It is known that one patient, even with a quiet conversation for 10 minutes. can disperse a huge amount of leprous sticks in a radius of 1-1.5 m. Mycobacterium leprosy can also be detected in tears, urine, semen, secretions from the urethra, breast milk and even blood during periods of reactivation of the leprous process.
There is no data on intrauterine infection of the fetus. Children born to parents suffering from leprosy and immediately separated from them remain slaughtered. It is known that in relation to a leprous infection there is a high natural resistance. This explains that not all persons who have even prolonged contact with leprous patients become ill, and in some people leprosy occurs in the form of a latent infection. At the same time, individuals who have had prolonged contact with leprous patients can detect mycobacterium leprosy in punctate lymph nodes, or even re-find them in a scraping of the nasal cavity in the absence of any signs of disease. But at the same time, various factors that weaken the body’s resistance (insufficient and inadequate nutrition, alcoholism, colds, heavy physical exertion), contribute to the infection or the transition process from latent to active form. Given the possibility of more frequent injury to the skin of the lower extremities, it is believed that infection mainly occurs through the skin of the lower extremities. This assumption is based on the frequent detection of mycobacterium leprosy in enlarged femoral lymph nodes in the initial period of the disease. In this case, pathogenetic significance is attached to impaired microcirculation, varicose syndrome and foot mycoses – especially athlete and candidiasis.
Pathogenesis during Leprosy
The gates of infection are the skin and mucous membrane of the upper respiratory tract. From the moment of introduction of microbes to the appearance of the first manifestations, it usually takes from 3 to 5 years, but sometimes several decades. A detailed picture of the disease does not develop in all cases. Only 10–20% of infected individuals develop unobtrusive signs of infection, and only half of them (ie, 5–10% of infected) develop a detailed picture of the disease. The development of a particular clinical form is associated with the genetic characteristics of the organism (in particular, with HLA haplotypes). In patients with leprosy, there is a defect in cellular immunity, significantly different from defects in cellular immunity in HIV-infected individuals. Even with intensive hematogenous dissemination of the pathogen, destructive processes are limited to the skin, peripheral nerves, anterior sections of the eye, testicles, upper and lower limbs. Especially often affected ulnar nerve near the elbow bend. In the case of lepromatous leprosy, mycobacteria are found in the liver, spleen, and bone marrow.
The incubation period, according to various authors, on average lasts from 4 to 6 years. However, the possibility of incubation is established quite reliably both within 2-3 months, and from 10-20 to 50 years. Therefore, leprosy is characterized by duration and significant fluctuations in the timing of the incubation period.
Lepra is considered a low-contagious disease; it is less contagious than tuberculosis. Children are much more sensitive than adults, and in conditions of prolonged contact they become infected with leprosy more quickly and significantly.
Prodromal phenomena are characterized by complaints of patients for indisposition, severe neuralgic pain, pain in the joints, increasing weakness, gastrointestinal disorders. Sometimes paresthesias, hyperesthesia, fever are noted. Already during this period, Hansen sticks can be found on the mucous membrane of the nasal septum. Subsequently, the clinical symptoms of the disease develop, depending on the morphological manifestations of which there are three types of diseases.
Classification. According to the Madrid classification adopted in 1953, 2 polar types of leprosy are distinguished: lepromatous and tuberculoid and 2 intermediate groups: undifferentiated and borderline (dimorphic). The modern classification, which in fact develops Madrid, reflects the immunological reactivity and data of immunobiological, histopathological, bacterioscopic studies. In practice, the following 3 types of leprosy are distinguished: lepromatous, tuberculoid, and undifferentiated or borderline. During each of these types of leprosy, 4 stages are possible: progressive, stationary, regressive, and residual effects. In the progressive and stationary stages, even with rational therapy, leprous reactions are observed, manifested by an exacerbation of the main foci of the disease and polymorphic secondary allergic rashes. Formation of the type of leprosy depends on the degree of immunobiological resistance of the patient. In individuals with symptoms of asthenia, immunodeficiency, accompanied by a negative lepromin test, the most contagious type of leprosy most often develops – lepromatous. In individuals with high immunological reactivity, manifested by a positive lepromin sample, there is a relatively favorable type – tuberculoid. Between these two opposite types, a peculiar intermediate form is often revealed – the undifferentiated type of leprosy. Such a variant of the formation of the disease is likely in individuals with an undetermined nature of immunobiological reactivity. Therefore, the undifferentiated type of leprosy can be further transformed either into a tuberculoid (with a favorable course) or into a lepromatous one.
Lepromatous type of leprosy. In the case of lepromatous type of leprosy, at first, mildly outlined, hardly noticeable reddish spots with a purple or cherry shade appear on the skin. Sensitivity (pain, temperature, tactile) in the area of these spots is not disturbed at first. Gradually, the spots become dense. Often powerful infiltrates are formed. Along with the skin itself, the subcutaneous fatty tissue is also involved in the process; nodes (lepromas) are formed. Such infiltrates and nodes are most often located on the extensor surface of the limbs, on the face in the area of the forehead, eyebrows, cheeks, nose. Facial expression is disturbed, or disfigured, taking the “ferocious” expression (“face of a lion” – fades leonina).
The infiltration of the superciliary arches leads to rather persistent hair loss in the region of the lateral part of the eyebrows. Infiltrative foci, in addition to the face and extensor surface of the extremities, may be in separate areas of the trunk skin, as well as in the internal organs. In addition to spots and infiltrates, there can be tubercles (lepromas) ranging from a match head to a pea, a hemispherical or flattened shape, dense texture, reddish-brown or reddish-purple color, which may later become rusty due to the hemorrhagic component. In the area of the lobes of the auricles, on the distal parts of the extremities of the leprosy are reddish-bluish color. The surface of the hillocks is shiny, smooth, often looking greased (“greasy”). Hillocks may ulcerate.
The resulting ulcers are characterized by dense, sometimes valikoobrazno elevated steep edges, serum detachable, which contains a large number of leprous bacilli. According to Brazilian leprologists (Bekelli, Romberg), 1 cm3 of affected tissue with lepromatous type contains about 1 billion bacilli. Ulcers are gradually performed by granulation and scarring. Nodes and deep infiltrates also often ulcerate, less often resolved without ulceration (in this case, superficial scars remain). In place of ulcerated nodes and deep infiltrates extensive ulcers are formed; however, muscles and bones may be involved in the process. In such cases, the destruction of the joints, small bones, and their falling away (mutilatio) with subsequent disfigurement and severe deformation can be observed.
Most often, the mucous membrane of the nose is involved in the process, especially in the area of the cartilaginous part of the septum. It is from here at the beginning of the disease in the scraping reveal Hanzen sticks.
The lesion of the mucous membrane of the nasal septum is characterized by erythema, infiltration, nasal discharge, layers of crusts, which causes a picture of chronic leprous rhinitis. As a result of the further development of leprous infiltrates in the cartilaginous part of the nasal septum, ulcers and destruction can form: in such cases, characteristic changes in shape occur — the tip of the nose elevated upwards.
Diffuse infiltrates and lepromas can be localized in the tongue in the region of the hard and soft palate, spread to the mucous membrane of the larynx, the vocal cords. As a result, hoarseness can occur, up to persistent aphonia, which in such cases is caused by cicatricial changes of the epiglottis mucosa and vocal cords.
In patients with lepromatous type of leprosy, conjunctivitis, iritis, episcleritis, and keratitis may be observed. As a result of infiltration, clouding, ulceration and scarring of the cornea after leprous keratitis without treatment, loss of vision may occur.
Lymph nodes (especially femoral and inguinal, less often cervical, occipital, submandibular, axillary) are enlarged to the size of a hazelnut, have a tight-elastic consistency, painless, mobile.
Often there is a lesion of the nervous system. These changes can be conditionally divided, firstly, into general disorders of the central nervous system, manifested by the development of variously pronounced neurotic reactions up to neuroses and psychoses; secondly, lesions of the peripheral nervous system, when neuritis and polyneuritis develop. Most often affected nn. ulnaris, auricularis magnus et peroneus. The affected nerve trunks are thickened and easily felt in their respective areas. As a result of lesions of the central and mainly the peripheral nervous system, changes in sensitivity, trophic and motor disorders develop.
Impaired sensitivity in lepromatous leprosy occurs much later than in tuberculoid leprosy. Sensitive nerve endings are affected secondarily as a result of the squeezing action of the primary cellular infiltrates. At first, persistent, rather agonizing neuralgia is noted, and then hyperesthesia, paresthesia, distortion of sensitivity, inadequate reactions to stimuli appear in the corresponding areas of the skin (the cold is felt as heat and vice versa, the response to the unconditioned stimulus is late). In the future, hyperesthesia and paresthesia are replaced by anesthesia, analgesia. The presence of thermal anesthesia and analgesia in the area of individual segments of the limbs (usually distal) and the body leads to frequent burns that patients do not feel. In place of these burns are then cicatricial changes of the skin. Impaired tactile sensitivity in lepromatous type is rare and only in a later period of the disease.
Trophic disorders in patients with leprosy lead to a violation of pigmentation, to severe complications – the development of mutation of the hands and feet. At the same time, due to significant trophic disorders (and not as a result of infiltrative processes), a gradual separation of the bone skeleton of the hands or feet, destruction and deformation of the nails occurs. The hand or foot becomes soft, resembling a seal with a paw or a frog’s paw. Trophic disorders caused by impaired function of the sebaceous and sweat glands. Their hyperfunction is later replaced by hypofunction until the complete cessation of sweating and sebaceous excretion: the skin becomes dry, rough, cracked. In such patients, the Minor’s test turns out to be negative (starch, sprinkled on the skin, lubricated with an alcoholic solution of iodine, when patients are placed in the heat-dry-air chamber does not turn blue due to the lack of function of the sweat glands).
Movement disorders occur as a result of damage to the peripheral nervous system. As a result of uneven atrophy of the extensors of the muscles of the hands, feet, then the forearms and lower legs, the flexor tone prevails. The fingers assume a semi-bent position with an unequal degree of flexion (claw-shaped hand and horse foot). On the back surface of the hands due to the atrophy of small muscles there is a recession of the interosseous spaces. Due to the atrophy of the muscles of tenar and hypotenar, the brush becomes flattened and resembles a monkey’s paw.
Atrophy of the circular muscles of the eye leads to incomplete closure of the eyelids (lagophthalmos). These patients can not spontaneously close the eyelids. In connection with the defeat of the facial nerve, atrophy of the facial muscles occurs and the face takes on a sad, mask-like expression (“St. Anthony’s mask”).
In patients with leprosy, a distinct combination of the above-described sensory, motor and trophic disorders with functional disorders of the central nervous system is possible.
In patients with lepromatous type of leprosy, various internal organs can be affected. First of all, the lungs, liver, and spleen become dense and dense. However, the clinical picture of these disorders is not typical for leprosy. The type of the disease is clarified on the basis of a clinic of specific changes in the skin and mucous membranes, data from a bacterioscopic examination of a nasal septum mucous membrane, scarification, a biopsied skin piece from the lesion, less often an enlarged lymph node.
Along with damage to the nervous system and internal organs, leprosy impairs the function of some endocrine glands, which is expressed in premature aging, early menopause in women, a decrease in sexual function, up to impotence in men. As a result of leprous bilateral orchitis and epididymitis that occurs in some patients with the subsequent development of the sclerotic process, azoospermia occurs. In such cases, the woman’s infertility is due to the absence of sperm from her husband.
Tuberculoid type of leprosy. Tuberculoid type leprosy is characterized by a much more benign course. The skin and peripheral nerves are mainly affected. On the skin appear sharply defined depigmented vitiligo-like spots or bright, with clear borders of a reddish-stagnant color with central fading spots, on the periphery of which in the form of a kind of border are polygonal, flat and dense papules with a violet tinge. The confluent papules form a reddish-purple or reddish-brown color, flat plaques of various sizes, sometimes having a ring-shaped configuration. Depigmentation and atrophy gradually develop in the central part of these plaques.
It is very characteristic of tuberculoid leprosy to early detect violations of pain, temperature, and somewhat later, and tactile sensitivity. In patients with vitiligo, these types of leprosy sensitivity are preserved. In addition, with the introduction of 0.1 ml of histamine intracutaneously at a dilution of 1: 1000, unlike vitiligo, reflex hyperemia does not occur around the blister in the area of the depigmented spot with leprosy (lack of axon-reflex). The affected peripheral nerves are thickened, palpable in the form of dense cords, in places with distinct bulges.
However, diseases of the nerve trunks in tuberculoid leprosy are much easier than lepromatous neuritis and polyneuritis. This type of leprosy is characterized by damage to the skin appendages (hair loss, impaired sweating in the affected areas, etc.).
Leprominic test in patients is characterized by a positive delayed response.
Undifferentiated type of leprosy. The undifferentiated type of leprosy is characterized by the absence of typical rashes.
A small number of soft spots with soft edges, of various sizes and outlines makes it difficult to diagnose. To identify Hansen’s wand in such patients is rarely possible. Histopathological picture is manifested banal nonspecific infiltration, similar to that which can be with a variety of chronic dermatoses. Leprosy sticks in such an infiltrate are most often not detected. These patients are ill-contagious, their general condition and state of health are generally good: In addition to the skin, the peripheral nervous system is involved in the process with an uncertain type of leprosy. Specific neuritis is characterized by thickening of the Schwann shell, accumulation of round-cell infiltrate in the perineurium, the presence of single leprous bacilli in the thickness of individual nerve bundles.
The clinical picture of polyneuritis to a greater extent resembles that of the tuberculoid form, but motor trophic disorders and sensitivity disorders associated with it can be quite pronounced (trophic ulcers, claw-shaped hand, horse foot, etc.). In the past, this form of leprosy was called “spotty-anesthetic” or “nervous.”
Lepromine response in patients with undifferentiated leprosy is different: in some it is negative, in others it is positive. In patients with a negative lepromin reaction, an unspecified type of leprosy can transform into a lepromatous form. Positive leprominic reaction indicates a favorable prognosis. In such patients, transformation into a tuberculoid type is possible.
In children aged mainly from 3 years and older, mixed or dimorphic leprosy is much more common than in adults, when there are simultaneously changes characteristic of lepromatous, tuberculoid and undifferentiated types of leprosy. In addition, in children, leprosy can manifest as erythema nodosum.
Lepra is not by chance called the “great imitator” of various syndromes. At the time of the patient’s initial visit to a doctor, patients show diffuse changes in the color of the skin, single or multiple spotted rashes, varying by location, shape, size and color (erythematous, hypopigmented, hyperpigmented), limited or diffuse skin infiltration, nodes, bumps, papules , rhinitis, loss of eyebrows, eyelashes, amyotrophy, brittle nails, ichthyosis, ulcers, paresthesias and impaired surface sensitivity. There are also signs of peripheral autonomic failure and symptoms of reflex vascular disorders (marbling of the skin, cyanosis, swelling of the hands and feet, disturbed flow and sebaceous excretions, etc.). Thus, the initial manifestations of leprosy are characterized by symptoms characteristic of dermatoses: genodermatosis, sarcoidosis, tuberculosis, allergic raskulitis, dyschromia, etc. At the first treatment of patients in medical institutions, erroneous diagnoses are frequent: toxicidermia, erythema nodosum, neurofibromatosis, dermatomastomy, and dermatomastomy are frequent; smooth skin, eczema, polyneuritis, etc.
Verifying studies of leprosy include a detailed description of the state of innervation in the element under study (temperature, pain and tactile sensitivity is disturbed in leprosy), the presence of thickening of dermal branches of nerves near eruptions and larger regional nerve trunks, but the most important thing is the identification of the causative agent during bacteriological examination and pathological examination (coloring according to Ziehl-Nielsen).
There are certain achievements in the development of test systems for specific serological diagnostics.
All types of leprosy are diagnosed on the basis of the cardinal symptoms of the disease: the presence of spotty, tubercular-knotted elements of a peculiar brownish color with a greasy luster, hair loss with a characteristic sensitivity disorder, existing neurological symptoms with a thickening of the nerve trunks determined by palpation. The diagnosis of leprosy is confirmed by the detection of leprous bacilli in tissue juice, smears from mucous membranes or histological specimens. It is more difficult to confirm the diagnosis of lepromatous and undifferentiated types when mycobacterium leprosy is absent. In these cases, using the complement fixation reaction (RAC) and precipitation reaction.
Quite informative in the diagnosis of early spotted manifestations of the sample with nicotinic acid. After intravenous infusion of 1.0 ml of a 1% nicotinic acid solution, spotty leprous rashes turn red and swell up (inflammation phenomenon) in 1-3 minutes.
The differential diagnosis of lepromatous leprosy is performed with tertiary syphilides, leishmaniasis, and skin tuberculosis (Bazin’s indurative erythema, scrofuloderma). Tuberculoid leprosy is differentiated from lichen planus, vitiligo, chloasma. The main criteria are clinical and morphological symptoms, trophic and neurological symptoms, hypo-or anesthesia, microscopic examination. In patients with tuberculoid and undifferentiated types, the study of the mycobacterium leprosy is best done after taking it for 1-2 days by patient for 5% of potassium iodide (1 tablespoon 3 times a day). This causes an aggravation of the process and may contribute to an easier and faster identification in the nose scraping of Hansen sticks. Also important for the diagnosis is a carefully collected history, living in endemic areas, indicating possible contact with a patient with leprosy.
Etiotropic therapy is used. The main drug is dapsone (4,4-diaminodiphenylsulfone, diphenylsulfone). An adult patient is prescribed from 50 to 100 mg once a day. The treatment is long. Although after 12 weeks most of the microbes die, however, relapses are possible after stopping treatment (sometimes after 5-10 years). Dapson resistant mycobacteria are observed. Rifampicin is a very active drug (after taking 1500 mg of the drug after 5 days, mycobacteria cannot be detected). The drug is prescribed at 600 mg / day. Due to the high cost in developing countries, rifampicin is prescribed 600 mg 2 times a week and even once a month. There are other drugs that are not sufficiently tested. The full course of therapy is 24 months.
Prevention of Leprosy
All family members are examined at least 1 time per year. Newborns are immediately separated from sick mothers with leprosy and transferred to artificial feeding, and more adult children are allowed to study in general schools subject to their clinical and laboratory examination at least 2 times a year. In places endemic for leprosy, mass screening of the population is carried out. Persons in contact with patients are given a leprometin test.
Leprominic test (Mitsuda reaction) – intradermal injection of leprosy in the digested suspension of mycobacterium leprosy.
In persons in contact with patients with leprosy, there is a rather high percentage of Mitsuda’s reaction, which makes it possible to judge the body’s resistance to a leprous infection. Persons who do not respond to the test with lepromein, undergo a thorough examination, active immunoprophylaxis by repeated administration of the BCG vaccine. Immunization (immunization) of the population with BCG vaccine is indicated in areas with a relatively frequent incidence of leprosy.
Family members of the patient are subject to preventive treatment, taking into account the results of the leproma test, if the patient has an active form of leprosy. Patients with leprosy are not allowed to work in the food industry and childcare facilities. According to an international agreement, the transfer of patients with leprosy from one country to another is prohibited. Personal prophylaxis of leprosy in persons who, by the nature of their activities, are in contact with patients, consists in strict observance of basic sanitary and hygienic rules (frequent hand washing with soap, mandatory microtrauma sanitation).