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Diffuse connective tissue diseases

Diffuse diseases of the connective tissue (syn: collagenoses, collagen diseases) is a group concept that combines several diseases in which diffuse damage to the connective tissue and blood vessels is noted. In 1942, Klemperer (P. Klemperer) proposed calling diffuse collagen disease diseases that are anatomically characterized by generalized alteration (damage) of the extracellular components of the connective tissue, primarily collagen fibers and their collagen protein. This group includes systemic lupus erythematosus, systemic scleroderma, dermatomyositis, periarthritis nodosa.

In the development of collagenoses, there are roughly three main periods:

1. Morphological period - was associated with an intensive study of histopathology, in particular fibroinoid changes in collagen fibers, which was observed in this case.

2. Clinical period - was associated with an intensive study of the manifestations of these diseases.

3. The immunological period.

Lupus erythematosus (lupus erytematodes), or scarring penfigoid. The proportion of this disease occupies 1% of all dermatoses. It is most often found in countries with a cold and humid climate, more often among blondes and pale-skinned people who have increased sensitivity to the sun, more often in young women and girls, more often in 20-40 years. In young women and girls, this disease is more common because they have a state of hyperextrogenemia. And then, when there are a lot of estrogens, the elimination (or removal) of the immune complexes that are formed during this disease slows down, hence the great likelihood of various autoimmune diseases.

Etiology. The tuberculous, syphilitic etiology, expressed more than a hundred years ago, is now of more historical interest. Currently, some researchers admit the role of a streptococcal agent, but most are inclined to viral etiology (RNA-type virus), although nothing has been finally determined. The development of the disease is associated with damage to the cellular component of immunity, T-lymphocytes, and above all T-suppressors, are affected, they become few. At the same time, the activity of B-lymphocytes increases, which leads to the development of autoantibodies of the widest spectrum of action. Autoantibodies to almost all body structures are formed: antinuclear (to the nuclei of cells), DNA, microsomes, lysosomes, mitochondria, etc. Autoantibodies to native DNA are the most pathogenetically significant. These antibodies combine with native DNA to form an immune complex, complement is activated, the entire chain of immunological reactions is launched, and these immune complexes are deposited on the basement membranes of various internal organs, including the skin (inflammation? Damage? Lysozyme activity increases? Biologically active release occurs substances — inflammation of almost all organs and tissues occurs).

Provoking factors: insolation, pregnancy and abortion, childbirth, the onset of menstruation in girls (i.e., hyperextrogenemia), various infections, drugs, post-vaccination reaction.

Predisposing factors: the presence of systemic lupus erythematosus in immediate relatives. The family-genetic predisposition of the association of systemic lupus erythematosus with histocompatibility antigens has been proved.

Classification. To date, there is no single classification. Each scientist believes that his proposed classification is the most rational.

I. Chronic form (or discoid).

II. Intermediate forms (so named because they are more often transformed into systemic lupus erythematosus):

? Dissiminated form.

? Centrifugal erythema bietta.

? The deep form of Kaposi-Irgang.

III. Systemic lupus erythematosus.

The discoid form is the most common clinical variety. It most often affects the skin of the face, most often in the form of a butterfly (the back of this “butterfly” is on the nose, and the “wings” on the cheeks). In addition, the forehead, eyebrows, auricles, red border of the lips, oral mucosa, scalp are affected. The course is chronic, laboratory data have a slight deviation from the norm. The diagnosis of discoid lupus erythematosus is based on three symptoms:

1. Erythema.

2. Follicular hyperkeratosis.

3. Cicatricial atrophy.

Accordingly to these symptoms, three stages of the course of this disease are distinguished:

I. Erythematous stage. In the places indicated above, primary elements appear, which are edematous spots, round in shape, with clear outlines, pink-red in color, which gradually increase in size and compact.

II. Infiltrative-hyperkeratotic. The center of the plaque is covered with tight-fitting, very stiff grayish scales. Scraping a fingernail over this focus, or holding a spatula for a patient is unpleasant, painful, which is called the Binier-Meshchersky symptom, as well as removing the scales (not to be confused with the Binier symptom with multi-colored lichen). If the flake is forcibly removed, then on the reverse side there is a phenomenon of hyperkeratotic layering in the form of a cone, reminiscent of a lady's heel, behind a set of follicular hyperkeratosis. If you remove a lot of scales, then the surface of the focus will be exposed, which will resemble a lemon or orange peel - a symptom of Khachaturian.

III. Cicatricial atrophy. The center of the plaque begins to absorb, the skin becomes thinner and easily folds. On the scalp, cicatricial atrophy develops very quickly with the irreversible disappearance of hair follicles.

Thus, in the breeding center, we distinguish three zones:

1. The central part is cicatricial atrophy.

2. Medium - infiltrative-hyperkeratotic.

3. The periphery is the erythematous part.

There are still signs that are very common, but not always, unlike the above. The surface of the focus is a rather colorful phenomenon due to telangiectasias, pinpoint hemorrhages and sites of poikiloderma (“leopard skin” - areas of hyper- and depigmentation). Objectively, patients are concerned about mild itching and a feeling of constriction, but usually the sensations are minimal. The following histological picture is very characteristic:

? Follicular hyperkeratosis is a phenomenon of “ladies' heel”.

? Atrophy of the malpigian layer (the first three layers are malpigian) of the epidermis.

? Vacuum degeneration in the cells of the basal layer (basal epidermal cells).

? Powerful lymphocytic infiltrate around the appendages of the skin (hair, sebaceous and sweat glands).

? Edema of the papillary dermis, expansion of capillaries, large vessels, their edema.

Intermediate forms:

1. Dissiminated (in 12-22% of patients). It looks like a discoid shape, only the foci are smaller in size, but their numbers are larger.

2. Centrifugal erythema Bietta (in 5-11% of patients).
This form was described by Beeth in 1828. But there is no follicular hyperkeratosis, no atrophy. This erythema can be located on the face in the form of a butterfly, but there are no tight-fitting scales, hypertrophy, etc. This form is quickly treatable, but often recurs. Since new foci may appear in new places, this erythema is sometimes called migratory erythema.

3. The deep form of Kaposi-Irgang. It is rare. One or more nodes, the skin is either not changed, or a stagnant bluish color. More often localized in the face, shoulders, buttocks. There is a very long time, calcification of the lesion is possible and, after regression, deep atrophy. Moreover, there are often foci of discoid lupus erythematosus.

Systemic lupus erythematosus. It occurs in 3-6% of patients with lupus erythematosus. Very severe polysyndromic disease. Changing the skin is very polymorphic (blisters, nodules or papura, spots may appear). There is no typical localization, rashes can occur anywhere.

The issue of differential diagnosis between systemic lupus erythematosus and focal lupus is very complex. The American Dermatological Association has developed a number of criteria for the timely diagnosis of systemic lupus erythematosus:

1. Erythema on the face in the form of a “butterfly”.

2. Typical discoid lupus.

3. Raynaud's syndrome - a vasospastic disorder of arterial blood supply to the hands and feet.

4. Alopecia (hair loss on the head).

5. Increased photosensitivity.

6. ulceration in the oral cavity and nasopharynx.

7. Arthritis without deformation of the joints.

8. LE cells (from lupus erytematodes) - lupus erythematosus cells (more than 5-10).

9. False positive reaction of Wasserman (the reaction is positive, but there is no syphilis).

10. Protein in the urine - proteinuria (more than 3.5 g per day).

11. Cylindruria.

12. Pleurisy, pericarditis.

13. Psychosis and convulsions.

14. Pancytopenia (almost all cells of the formed elements become scarce).

Laboratory Confirmations:

1. Complete blood count - increased ESR, pancytopenia, detection of LE cells. LE cell formation pattern: Casiricar antinuclear factor was discovered, which is macroglobulin, which acts on the nucleus of a white blood cell. In this case, the nucleus of the leukocyte begins to increase, homogenize and destructurize, loses its chromatin structure. The white blood cell is destroyed and its nucleus enters the surrounding tissue. In a smear, it will be referred to as a hemotoxylin body. Then new white blood cells approach this nucleus. This formation will be called a socket. Each of these leukocytes begins to phagocytose the remnants of the nucleus, thus the leukocyte, which has absorbed the destructed nucleus and has, besides its own nucleus, a destroyed nucleus and is called an LE cell or lupus erythematosus cell. Detection of at least 5 cells per 1000 leukocytes is diagnostically significant.

2. Biochemical analysis of blood - there is a hyperproteinemia and dysproteinemia of the ratio of globulin fractions, an excess of macroglobulins, in particular gamma globulin fractions. Biochemical signs of inflammation: C-reactive protein appears, the content of sialic acids, fibrin and seromucoids increases.

3. Immunogram - few T-suppressors, many T-lymphocytes. If a radioimmunoassay method is used, then antibodies to native DNA and antinuclear factor Ig G are detected.

4. Serological blood tests - a positive reaction of Wasserman.

Treatment of lupus erythematosus:

1) Remediation of focal infection foci.

2) Protect the skin from excess insolation. This is the main condition for successful treatment, otherwise any therapy will not give results.

3) Antimalarial drugs are most effective. They were proposed in 1940 by academician Prakapchuk. As it turned out, they not only affect the causative agent of malaria, but also have a good effect with lupus erythematosus. These are chingamine, delagil, rezohin, chlorofin and the English plaquenil device. 1 tablet 3 times a day, then switch to a lower dose 2 times a day. A course of 80-100 tablets. The positive effect of these drugs with lupus erythematosus is due to the fact that they have photo-desensitizing effects and stimulate the production of endogenous corticosteroids. The drugs are toxic, therefore, mandatory monitoring of the general blood test is necessary, since they can cause leukopenia; mandatory monitoring of the liver and organs of vision, as retinopathies often develop. A very good Hungarian preparation presocil., Which contains delagil, prednisolone and aspirin.

4) Vitamins. They are prescribed to improve tolerance, increase the effectiveness of the third group of drugs, i.e., antimalarial:

? Nicotinic acid (vitamin B3). Photoprotective effect, enhances the production of endogenous corticosteroids, improves tolerance to the third group of drugs. It is given in tablets, or intramuscularly in the form of solutions.

? Riboflavin (Vitamin B2). It improves tolerance to the third group of drugs, is prescribed in tablets for at least a month.

? Pantothenic acid (vitamin B5). It is prescribed in solutions and tablets. Enhances the production of endogenous corticosteroids, also improves tolerability of the above drugs.

? Vitamin B6 - to prevent intolerance to antimalarial drugs.

? Cyanocobalamin or Vitamin B12 - has a photo-hyposensitizing effect.

? Folic acid.

? Vitamin C or ascorbic acid, which is an antioxidant here, involved in the synthesis of collagen and the production of corticosteroids. In addition, anti-malarial drugs create vitamin C deficiency.

? Vitamin E and Vitamin A, “Better as Avita.” Strong anti-inflammatory effect, and stabilization of cell membranes. This is important because immunocomplexes are being deposited and are being pollinated to target immunological reactions.

5) Antiviral therapy. Interferron, indomethacin. Patients with lupus erythematosus have antibodies detected to prostaglandins. And indomethacin slows down the production of prostaglandins.

6) Immunomodulators. Levomizole (decaries) increases the number of T-suppressors, since there are few of them, and all that activates the cellular immunity link.

7) When attaching a secondary infection, antibiotics can be used very carefully. Tetracyclines and fluoroquinolones should not be prescribed, because they increase the sensitivity of the skin to the sun. Sulfonamides, especially streptomycin, are contraindicated, since they complicate the course, until the transition to the transition to the systemic form.

8) Cytostatics in severe cases.

9) External treatment is auxiliary in nature, therefore, corticosteroid ointments of fluorocor, tosinolar, etc. can be used. With the discoid form, the foci are chipped with either an emulsion or delagil.
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Diffuse connective tissue diseases

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    Diffuse diseases of the connective tissue is a group of diseases that are characterized by a systemic type of inflammation of various organs and systems as a result of the combined development of autoimmune and immunocomplex processes, as well as excessive fibrosis. A feature of this group of diseases is the multifactorial type of predisposition with a specific role of immunogenetic
  2. Diffuse connective tissue diseases
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  10. Cross syndrome and mixed connective tissue disease
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  12. Abstract. Biochemistry of bone and connective tissue., 2008
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  16. CONNECTIVE TISSUE FUNCTION
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  19. Biochemical changes in connective tissue during aging and some pathological processes.
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