Lupus

Tuberculosis of the skin is a chronic disease that occurs with exacerbations and relapses. Factors contributing to the development of exacerbation and relapse are insufficient duration of the main course of treatment, inadequacy of anti-relapse treatment, poor tolerance of anti-tuberculosis drugs, developing resistance to them strains of mycobacteria.

In other words, skin tuberculosis is a syndrome of skin lesion in tuberculosis along with other evolutionary syndromes of extrapulmonary tuberculosis. This circumstance determines the unity of their pathogenetic mechanisms. This also explains the other features of skin tuberculosis, namely, the variety and "blurring" of the forms, a periodically observed sharp decrease in the incidence. The discrepancy between the clinical picture of various forms and pathogenetic notions about the periods of the development of the disease does not allow to develop a single generally accepted classification of skin tuberculosis.

Tuberculosis of the skin is distinguished by the duration of the course. Often, it is diagnosed late, and it is not amenable to treatment, which leads to the accumulation of contingent patients. The disease itself, its complications and consequences often remain for life, leading to visible cosmetic defects and even to disfigurement. Over 80% of all cases of skin tuberculosis are diagnosed within a period of more than 5 years from the onset of the disease. The reason for this is that. That doctors of the general network and even phthisiatricians are extremely little aware of the clinical manifestations, methods of diagnosis and treatment of skin tuberculosis. And if the latter is true for extrapulmonary tuberculosis in general, then phthisiodermatology is in the worst situation.

[1], [2], [3], [4], [5]

Causes of Tuberculosis of Skin

Tuberculosis of the skin is most often the result of lymphogenous or hematogenous spread of infection, per conutuitatem, less often - exogenous.

Provocative role in the development of tuberculosis is played by a decrease in nonspecific resistance of the organism, acute infections, trauma, functional disorders of the nervous system, endocrine disorders, primarily diabetes mellitus, malnutrition, hypovitaminosis, pregnancy, corticosteroid and cytostatic therapy.

There is no generally accepted classification of skin tuberculosis. Based on data on the routes of infection and the spread of tuberculosis infection, the state of immunity and allergies, taking into account the period of the disease, scientists divided the various manifestations of cutaneous tuberculosis into two groups:

1. tuberculosis of the skin, developed in previously uninfected individuals, including the primary affect. Primary complex, primary affect at the BCG vaccination site, miliary tuberculosis, collicative tuberculosis (primary hematogenous scrofuloderma), and
2. tuberculosis of the skin developed in previously infected individuals, including predominantly local forms, for example, tuberculosis lupus, warty tuberculosis, scrofuloderma, ulcer peri-lateral tuberculosis, predominantly disseminated - papulonecrotic tuberculosis, lichen scrotal, erythema compacted, lupus disseminated miliary.

Currently, there are 4 types of mycobacteria: human, bovine, avian and cold-blooded. Human and bovine types are pathogenic for humans. Tuberculosis is affected by men and women in equal proportions. But men are ill, as a rule, warty, and women - with lupus forms of tuberculosis. Healthy skin is an unfavorable environment for the life of mycobacteria. Development of skin tuberculosis, as a rule, is promoted by: hormonal dysfunction, hypo- or avitaminosis, diseases of the nervous system, metabolic disorders (water and mineral), unsatisfactory social conditions and infectious diseases. Tuberculosis recurs in the winter and autumn periods. Exacerbations occur more often in patients with tuberculous lupus erythematosus and Bazin, less often in patients with papulonecrotic tuberculosis.

All of the above factors reduce the immune system and susceptibility to mycobacteria tuberculosis. Tuberculosis of the skin is the most common opportunistic infection in HIV-infected patients, especially in developing countries. Infection occurs endoexogenously and by autoinoculation.

Depending on the mode of infection, the skin tuberculosis is classified as follows:

Exogenous infection:

• Primary skin tuberculosis (tuberculosis chancre) develops at the site of introduction of the pathogen into the skin of unvaccinated and not infected people with tuberculosis;
• warty tuberculosis of the skin develops at the site of the pathogen in the skin of people who have been ill or have tuberculosis.

Endogenous infection:

• tuberculosis lupus (lupoid tuberculosis);
• scrofuloderma (secondary scrofuloderma);
• colliquative skin tuberculosis (primary scrofuloderma);
• miliary tuberculosis of the skin;
• Ulcerative tuberculosis of the skin and mucous membranes (tuberculosis Yarischa-Chiari).

Sometimes, skin tuberculosis develops after BCG vaccination and is called post-vaccination.

[6], [7]

Histopathology of skin tuberculosis

The process is localized in the upper part of the dermis, but can spread to the subcutaneous tissue. It is represented by an epithelioid-cell granuloma with giant cells of Langhans, surrounded by a lymphocytic shaft. In areas of healing, fibrosis is observed.

Histogenesis of skin tuberculosis

The main factors affecting the development of the pathological process are the massiveness of infection and virulence of bacteria, the state of immune reactivity of the organism. Tuberculosis inflammation is considered as a classic example of inflammation on the immune basis. T cells, specifically sensitized to mycobacterial antigens, are considered as the central link in the manifestation of resistance of the organism to the causative agent of the infection. The role of humoral immunity in the formation of resistance to tuberculosis is not yet clear, as to the role of autoimmune reactions. There is evidence of a high value of the allergic component in the development of disseminated forms of skin tuberculosis. The most studied in this disease are the cellular mechanisms of immunity, primarily the T-system of immunity. According to the data of M.P., Elshanskaya and V.V. Erokhina (1984), in the early stages of experimental tuberculosis, the thymus-dependent zones of the spleen and lymph nodes are expanded due to infiltration by their lymphocytes and the development of blast-transformation, increased migration from the thymus of lymphocytes, EG Isaeva and NA Laptev (19S4) were observed in the development process tuberculosis phase changes in the activity of various T-cell subpopulations. At the same time, short-term stimulation of T-helper function in the initial stages of the disease was replaced by the accumulation of T suppressors during the generalization of the process. With the function of the T-system of immunity, the most characteristic for tuberculosis of HRT and the granulomatous reaction are closely related, which develops under conditions of a prolonged persistence of myco-factors in macrophagocytes.

The tuberculosis granuloma consists predominantly of epithelioid cells, among which are giant Pirogov-Langhans cells surrounded by a shaft of mononuclear cells containing lysosomal enzymes that then develop into macrophages. In the phagosomes of the latter, in the electron microscope study, mycobacteria are detected. Casey necrosis is often found in the center of the tuberculosis granuloma, which is also an expression of delayed type hypersensitivity. It should be noted that granulomatous inflammation is not observed at all stages of the development of the tuberculosis process, not with all clinical forms of skin tuberculosis. So, a specific tubercle infiltrate is most typical for tuberculous lupus. At other forms granulomatous structures usually are combined with nonspecific inflammatory infiltrate.

In the early phase of the inflammatory reaction in the skin at the site of the introduction of mycobacteria, nonspecific phenomena of exudation and alteration are most pronounced, neutrophil granulocytes predominate in infiltrates, and a few lymphocytes.

Polymorphism of clinical and histological manifestations of skin tuberculosis depends largely on the general condition of the body, primarily on its immunity, the age of the patients, the presence or absence of foci of infection in other organs and systems, skin properties, primarily microcirculatory disorders. It is not excluded that every form of skin tuberculosis can be controlled by genetic factors that, acting against a predisposition to tuberculosis, can lead to its development in a certain area, for example, in the skin.

Classification of skin tuberculosis

All the numerous forms of the disease are divided into two clearly defined groups.

• True skin tuberculosis, also called localized, true, bacterial or granulomatous.
• Skin lesions as a result of allergic ("paraspecific" according to AI Strukov) immune inflammation, mainly in the form of allergic vasculitis, called disseminated, hyperergic cutaneous tuberculosis and classified by J. Darje as "tuberculides."

The overwhelming majority (more than 70%) of cases of skin tuberculosis belongs to the 1st group; It should be noted that lichenic tuberculosis of the skin (lichen scrofulosorum) occupies an intermediate position and is often placed in the tuberculosis group.

Diseases included in the 2 nd group are well-known allergic vasculitis, devoid of specific features. The pathomorphological and clinical picture of these forms differs in a certain uniqueness, and along with changes in the nonspecific character, tubercular tubercles can also be histologically detected.

The insufficiently studied miliary disseminated lupus of the face (lupus miliaris disseminatits) stands apart. Close to the 1 st, but attributed by some authors to the 2-nd group. There are also skin diseases, the tuberculous etiology of which is not proven. This is an acute or chronic erythema nodosum, nodular vasculitis, ring-shaped granuloma, rosace-like tuberculosis of Lewandowski and a number of allergic vasculitis associated with tuberculosis infection indirectly.

In domestic literature for the convenience of prakticheskih doctors, skin tuberculosis is classified as follows: localized forms (tuberculosis lupus, colliquative, warty, miliary-ulcer tuberculosis), disseminated forms (papulonecrotic, inductive, lichenoid).

[8], [9], [10], [11], [12]

Primary skin tuberculosis

Synonyms: tuberculous chancre; primary tuberculous affect. Mostly children are ill. Usually at the site of infection after 2-4 weeks after infection appears asymptomatic reddish-brown papule of a dense consistency, turning into a superficial painless ulcer, which in some patients takes a shankraiform appearance (tuberculous chancre). After 2-4 weeks, lymphangitis and lymphadenitis appear. After a few months, the primary focus is healing with the formation of a scar, but there may be generalization of the process with the development of disseminated forms.

Pathomorphology

At the early stage of the process, the changes are non-specific, characterized by tissue destruction, in which numerous mycobacteria are found, by infiltration with neutrophilic granulocytes. Later, the infiltrate is dominated by monocytes and macrophages, then epithelioid cells appear, among them giant cells of Pirogov-Langhans are found. The number of epithelioid cells increases, and mycobacteria decreases, after a while the fibroplastic transformation of the focus and scar formation occurs.

Tuberculosis of the skin acute miliary disseminated

Very rare form, occurs against the background of general disseminated tuberculosis as a result of hematogenous dissemination. Characterized by the appearance on the skin of the trunk and extremities of symmetrical small reddish-brown or cyanotic patchy-papular rashes, pustular, vesicular, hemorrhagic elements, sometimes - nodular formations, including subcutaneous ones.

Pathomorphology

The central part of the papule is a microabscess containing neutrophilic granulocytes, necrotic cell debris and a large number of mycobacterium tuberculosis, surrounded by a macrophage zone. With an easily flowing form, the histological pattern is similar to that described above, but mycobacteria in the lesion are almost not found.

Tuberculous lupus (lupus vulgaris)

It is one of the most common form of skin tuberculosis. The disease often begins at school age and in women. It is characterized by the appearance of several specific soft tubercles (lupus), located in the dermis, pink color with clear boundaries 2-3 mm in diameter. Elements are often localized on the face (nose, upper lip, ears), but can also occur in other areas. Lupomas are prone to peripheral growth, forming continuous lesions (flat form). With diascopy (pressing glass), the color of the tubercle becomes yellowish (the phenomenon of "apple jelly"), and when pressed on the tubercle with a buttoned probe, extreme softness is found and it easily fails, leaving a depression in the tubercle (symptom of the "probe" or Pospelov's symptom). Lupoma can be resolved either by the dry way, when the tubercles are fibrous with the destruction of collagen and elastic fibers and the formation of scar tissue atrophy resembling a crumpled tissue paper, or under the influence of various injuries, when the tubercles can ulcerate (ulcerous form) to form superficial with soft uneven edges and easily bleeding ulcers. In clinical practice, tumorous, verrocose, mutilating and other forms of tuberculous lupus are found. Some patients are affected by the mucous membrane of the nasal cavity, hard and soft palate, lips, gums. Vulgar lupus occurs chronically, torpid, with deterioration in the cold season and may be complicated by the development of lupusurcinoma.

Collicative tuberculosis of the skin (scrofuloderma)

It occurs in persons, especially among children, suffering from tuberculosis of the subcutaneous lymph nodes, from which the mycobacteria are introduced into the skin. In the submaxillary region, on the neck and limbs, dense, painless, deep-lying skin and rapidly growing nodes appear, reaching 3-5 cm in diameter and tightly bonded to the proper tissues. The skin above the nodes becomes bluish. Then the central part of the elements is softened and deep soft almost painless ulcers are formed, which are joined together by fistulous passages, from which bloody contents are excreted with the inclusion of necrotic tissue. Ulcers have dented edges, sluggish granulations. After the healing of ulcers, there are very characteristic "torn", "bridge-shaped" scars of irregular shape.

Secondary scrofuloderma

In contrast to hematogenous collicative tuberculosis, scrofuloderma occurs again from the lymph nodes affected by tuberculosis or other extrapulmonary forms of tuberculosis. It is more common in children. The nodes are located deep, in places of localization of the lymph nodes, most often cervical, or around the fistula with osteoarticular tuberculosis. When they are opened, deep ulcers develop, after the healing of which remain bridged, bridged, fringed scars. On the scars often appear tubercles, may be observed verrukoznye (fungous) foci.

The pathomorphology of the primary and secondary scrophuloderm is similar. In the upper parts of the dermis, the changes are predominantly nonspecific (foci of necrobiosis surrounded by a mononuclear infiltrate), in its deeper parts and in the subcutaneous tissue there are tuberculoid structures with marked necrosis and significant inflammatory infiltration. Mycobacteria are usually found in the superficially located parts of the affected area.

Warty tuberculosis of the skin

Often occurs with exogenous infection in the skin and occurs in pathologists, workers in slaughterhouses, veterinarians in contact with tuberculosis patients with animals. Clinically begins with the appearance of small, painless grayish-red verrux cells surrounded by a narrow inflammatory fringe, rounded, oval or polycyclic outlines covered with thin scales. Gradually increasing in size and merging, they form a continuous warty, sometimes papillomatous lesion, sharply outlined, irregular, polycyclic outlines of a brownish-red color with horny layers, surrounded by a corolla of bluish-red erythema. When regressing in place of the focus, a scar is formed. Rare variants are keloid-like, sclerotic, vegetative, similar to warty tuberculous lupus. On the fingers, back and palm surfaces of the hands, the soles appear painless nodules (or tubercles) of pinkish-cyanotic or reddish color with a cyanotic abscess, surrounded by a narrow inflammatory fringe. In the central part there are warty growths with horny masses.

Pathomorphology

Expressed acanthosis, hyperkeratosis and papillomatosis. Under the epidermis - an acute inflammatory infiltrate consisting of neutrophilic granulocytes and lymphocytes, abscesses are noted in the upper parts of the dermis and inside the epidermis. In the middle of the dermis are tuberculoid structures with a small caseous in the center. Mycobacteria are much larger than in tuberculosis lupus, they can be easily found in sections stained using the Tsilya-Nielsen method.

Miliary-ulcer tuberculosis

Occurs in weakened patients with active tuberculosis of the lungs, intestines and other organs. As a result of autoinoculation with urine, faeces, sputum, containing a large number of mycobacteria, skin lesions occur. Conventional localization is the mucous membranes of natural openings (mouth, nose, anus) and the surrounding skin. Appear small yellowish-red tubercles, which quickly ulcerate, merge with each other, forming painful superficial, easily bleeding ulcers with an uneven bottom and minute abscesses ("Trela's grains").

Tuberculosis of the skin papulonecrrotic

It occurs more often in women. It is characterized by disseminated soft rounded hemispherical papules (more precisely, tubercles) from the pinhead to the pea, a brownish reddish or cyanotic reddish color. Elements are painless, have a dense consistency, smooth or slightly flaky surface. They are localized absent-mindedly on the legs, hips, buttocks, extensor surfaces of the upper limbs, mainly in the joint region.

In the central part of the elements a necrotic scab is formed, after which the "stamping" scars remain.

Tuberculosis of the skin indurative (erythema inductive Bazena)

More common in young women. On the shins, thighs, upper limbs, abdomen appear dense, soldered to the skin, malo-painful knots 1-3 cm in diameter. At first the skin over the nodes is not changed, then becomes reddish with a cyanotic shade. Over time, the site resolves and in its place remains a sunken brownish area of cicatricial atrophy. In some patients ulceration of the nodes is observed and painful shallow ulcers are formed, which differ in the torpid current.

Tuberculosis lichenoid (lichen scurf)

It occurs in patients with tuberculosis of internal organs. On the skin of a monster, less often - limbs and faces appear miliary papules of a soft consistency, a yellowish-brown color or the color of normal skin. They tend to group, disappear without a trace. Sometimes scales are observed in the center of the element. Occurring with this form of tuberculosis, flat papules resemble red flat lichen. Clinically manifested in the form of asymptomatic lichenoid, follicular or perifollicular rashes, often with horny scales on the surface, yellowish brown, reddish or pale pink. When merging and close arrangement of the elements, large foci of oval or ring-shaped lesions may appear. Regressing, the tubercles leave superficial scars.

Pathomorphology

In the dermis, mainly epithelioid-cell granulomas are found, located mainly perifollicularly, as a rule, without caseous necrosis in the center and weak lymphocytic reaction around them.

Tuberculosis of Lupus (syn. Tuberculosis of the skin is lupoid)

The causative agent enters the skin lymph-hematogenously from other foci of tuberculosis infection in the body. The primary element is the tubercle (lupoma). A characteristic feature is a soft consistency, which is revealed when the probe is pressed, which, as it were, tears the tubercle, falling into it (the "probe symptom"). With diascopy, the color of the lupus changes to yellowish-brown (the phenomenon of "apple jelly"). The most common form is flat lupus. The surface of the hearth is usually smooth, but there may be warty growths resembling a warty tuberculosis of the skin, a pronounced ginkreratosis reminiscent of the skin horn. Quite often there is ulceration. With a tendency to spread over the surface, new elements appear. Serpiginiruyuschie foci, and deep into - the destruction of subcutaneous tissue, cartilaginous part of the nose, ears, rejection of phalanges of the fingers, etc. Complication of tuberculous lupus can be lupus carcinoma. In the place of regressed foci, there is a superficial scar, in the zone of which, as well as around, the appearance of new lupas is characteristic. Rare variants of lupus erythematosus are tumorous, verruzic, early infiltrative, erythematous, sarcoid-like.

Pathomorphology

In the dermis most often show specific changes in the form of tuberculous tubercles and tuberculoid infiltrates. Tubercular tubercles consist of clumps of epithelioid cells with varying degrees of necrosis, surrounded by a mononuclear cell shaft. As a rule, among the epithelioid elements there is a different number of giant cells such as Pirogov-Langhanea. Tuberculoid infiltrate is a diffuse infiltration of the dermis by mononuclear cells. Among which are epithelioid tubercles of various sizes. Sometimes the infiltrate spreads to the deep sections of the dermis and the subcutaneous fat layer. At the same time, destruction of the skin appendages and necrosis in the epithelioid tubercles are observed. In some cases, especially with ulceration. In the dermis, a nonspecific inflammatory infiltrate predominates, tuberculoid granulomas are less common. Changes in the epidermis are secondary, its atrophy and destruction, acanthosis, hyperkeratosis, and sometimes parakeratosis are observed. At the edges of ulcerative lesions, pseudoepithelial hyperplasia, the development of cancer, are possible. Mycobacterium in the lesions with this form of tuberculosis is very small, they are not always visible in slices. Even in infected guinea pigs, tuberculosis does not always develop.

Tuberculosis lupus should be differentiated from diseases in which tuberculoid structures are detected in the skin (syphilis, leprosy, fungal lesions). The most difficult to distinguish this disease from sarcoidosis is the absence of absolute histological criteria. It should be borne in mind that in sarcoidosis, granulomas are located in the thickness of the dermis and are separated from the epidermis by a strip of unaltered collagen. In addition, with sarcoidosis, granulomas consist mainly of epithelioid cells, there are almost no lymphoid elements, necrosis is extremely rare.

Tuberculosis of skin and mucous membranes ulcerous periapine

A rare, exogenously emerging form of tuberculosis of the mucous membranes and adjacent areas of the skin due to massive autoinoculation of the infection with progressive exudative tuberculosis of internal organs (lungs, digestive tract, urinary system). Men are more often ill. On the mucous membranes, around the natural openings, less often on the operating wounds, there are multiple miliary nodules, rapidly disintegrating with the formation of small superficial but sharply painful ulcers, with an uneven granular bottom surrounded by an inflammatory rim. Ulcers can fuse.

Pathomorphology

A nonspecific inflammatory infiltrate with a predominance of neutrophilic granulocytes is found around the ulcer. In deeper parts of the dermis, in most cases, tuberculoid granulomas are detected, usually with necrosis in the center.

Tuberculosis of the skin is papulonecrotic (folliclis, acnitis Barthelemy)

At the heart of the disease is allergic vasculitis, which develops as a result of sensitization of mycobacteria tuberculosis or products of their vital activity. This form of tuberculosis occurs in adolescents and young people, more often female. Eruptions are localized mainly on the skin of the extensor surfaces of the extremities and buttocks. In the central part of most elements, necrosis develops with the formation of a crater-like sore covered with a tightly fitting crust surrounded by a slightly protruding rim. After healing, there remain characteristic, as if stamped scars, often surrounded by a narrow pigmented rim. Characteristic polymorphism of the rash, caused by the existence of papules in different stages of development.

Pathomorphology

In the center of the focus is a site of necrosis of the epidermis and the upper part of the dermis, surrounded by a zone of nonspecific inflammatory infiltrate, in the peripheral parts of which there are typical tuberculoid structures with pronounced caseous necrosis. Vascular changes are noted in the form of thickening of their walls and infiltration by inflammatory elements, i.e. Develops vasculitis, which, probably, is the cause of necrosis.

Tuberculosis of the skin condensed (condensed erythema Bazena)

This form is based on dermo-hypodermal allergic vasculitis, caused by increased sensitivity to mycobacteria tuberculosis, which get into the skin mostly hematogenously. It develops mainly in girls and young women suffering from impaired peripheral circulation and hypofunction of the sexual glands. Clinically characterized by the presence of symmetrical, deeply located, few knots of a dough or a tight-elastic consistency with a diameter of 1-5 cm, which is predominantly on the legs in the region of the gastrocnemius mouse. Lymphangites frequently associated with lesions are often observed. After the regression of the nodes, pigmentation and the street degree of atrophy remain. Approximately 30% of the cases are ulcerated. After healing, retracted scars with hyperpigmentation around the periphery remain.

Pathomorphology

In fresh elements, changes are limited to the subcutaneous fat layer, although the infiltrate may be located in the dermis. Characterized by the granulomatous structure of the infiltrate, changes in blood vessels and foci of necrosis. Sometimes the infiltrate can be nonspecific, but among the inflammatory elements you can see small tuberculoid-type foci. There are marked changes in the vessels in the form of thrombovasculitis of small arteries and veins, which often leads to necrosis. Differentiated erythema from knotty due to the presence of a more massive infiltrate and foci of caseous necrosis, which is not present with erythema nodosum.

Tuberculosis of the face skin miliary disseminated

A rare type of tuberculosis, probably, is a localized version of papulonecrotic skin tuberculosis. Characterized by the presence on the linden of an isolated mil paired papules yellowish-reddish or reddish-brown in color, hemispherical with a pustulose-centered center, soft consistency, giving the phenomenon of apple jelly during diascopy. Rashes, usually superficial. There is polymorphism at the expense of different stages of development of elements. After the regression, the scars are left behind.

Pathomorphology

In the superficial layers of the dermis there are typical tuberculoid structures of granuloma with necrosis to the center.

Differential diagnosis is carried out with tubercular syphilis, skin cancer, leishmaniasis, deep mycoses, angiitis of the skin.

Treatment of skin tuberculosis

They conduct complex therapy with the use of tuberculostatic agents, drugs aimed at increasing the immunity system, normalizing metabolic disorders. Tuberculostatic drugs on the therapeutic effect are divided into the following groups:

1. the most effective drugs: isoniazid, rifampicin;
2. medications of average efficiency: ethambutol, streptomycin, protionamide (ethionamide), pyraziumamide, kanamycia, florimycin (viomycin);
3. preparations of moderate activity: PASK, thibon (thioacetazone).

The use of vitamins (especially group B), antioxidants (a-tocopherol, sodium thiosulfate, dibunol), immunomodulators (immunomodulin, sodium nucleate, thymalin), anabolic steroids, physiotherapeutic measures (UV irradiation in suberythmic doses, electrophoresis ) and therapeutic nutrition.