Systemic scleroderma in children. Pathological anatomy of systemic scleroderma

For citation: Grebenyuk V.N. LIMITED SCLERODERMA IN CHILDREN // BC. 1998. No. 6. S. 2

Key words: Scleroderma - etiology - pathogenesis - auto immune disorders- classification - clinical forms - lichen sclerosus - penicillin - lidase - biostimulants - pyrogenic preparations - vasoprotectors.

The article describes limited scleroderma in children: etiology, pathogenesis, classification of the disease, clinical forms and manifestations. Practical recommendations on diagnostic and medical approaches are given.

Key words: Scleroderma - etiology - pathogenesis - autoimmune disorders - classification - clinical forms - lichen sclerosus et atrophicus - penicillin - lidase - biostimulants - pyrogenic agents - vasoprotectors.

The paper outlines localized scleroderma in children, its etiology, pathogenesis, classification, clinical forms, and manifestations. Practical guidelines for diagnostic and medical approaches are given.

V. N. Grebenyuk, prof., Dr. med. Sci., Head of the Department of Pediatric Dermatology, TsNIKVI, Ministry of Health of the Russian Federation

Prof. V.N.Grebenyuk, MD, Head, Department of Pediatric Dermatology, Central Research Dermatovenereologic Institute, Ministry of Health of the Russian Federation

Introduction

Limited scleroderma (OS) in children is a serious modern medical and social problem. Unlike systemic scleroderma (SS), in which various organs are involved in the pathological process, OS is "limited" to lesions of the skin only. At the same time, the disease often acquires a systemic character, i.e. becomes an ssd. However, the opinion that these two diseases essentially represent a single pathological process is not shared by all researchers. Some authors believe that OS and SJS are not identical, and differentiate them by pathogenesis, clinic, and course. And in this case, SJS is classified as diffuse connective tissue disease (DCTD), but OS is not.
As is known, DZT include SJS, systemic lupus erythematosus (SLE), dermatomyositis, periarteritis nodosa and rheumatoid arthritis - formidable diseases that require a specific strategy and tactics for managing patients, conducting an intensive therapeutic and prophylactic complex. SJS is the second most common disease after SLE from the CTD group (from 32 to 45 cases per 100,000 population). It should be emphasized once again that the possibility of OS transition to SDS cannot be ignored.
In childhood, OS dominates. It occurs in children more than 10 times more often than SLE. Girls get sick more than boys more than 3 times.
The disease can occur at any age, even in newborns, usually beginning gradually, without any subjective sensations and disturbance of the general condition. Due to the tendency of a growing organism to a widespread pathology, to pronounced exudative and vascular reactions in children, this disease often exhibits a tendency to a progressive course, extensive damage, although in the early stages it can manifest itself as single foci. In the last decade, the incidence of this pathology in children has increased. OS is characterized by predominantly localized foci of chronic inflammation and fibrous-atrophic lesions of the skin and mucous membranes.
The first description of a disease similar to scleroderma, known to ancient Greek and Roman doctors, belongs to Zacucutus Zusitanus (1634). Alibert (1817) significantly supplemented the characteristics of this disease, for which E. Gintrac proposed the term "scleroderma".

Etiology and pathogenesis

The etiology of scleroderma has not yet been definitively established. The hypothesis of infectious genesis is interesting in the historical aspect, however, the role of Koch's bacillus, pallidum spirochete, pyococci as a possible root cause of scleroderma has not been confirmed. The significance of Borrelia burgdorferi in the development of this disease is also not convincing. Although structures resulting from the indirect influence of a viral infection were found in cells of various tissues in patients with scleroderma, the virus was not isolated.
The role of genetic factors is not excluded.
Multifactorial inheritance is assumed.
The pathogenesis of scleroderma is associated mainly with the hypotheses of metabolic, vascular and immune disorders.
The occurrence of scleroderma is also affected by disorders of the autonomic nervous system and neuroendocrine disorders.
Connective tissue metabolism disorders are manifested by hyperproduction of collagen by fibroblasts, an increased content of hydroxyproline in blood plasma and urine, a violation of the ratio of soluble and insoluble fractions of collagen, and accumulation of copper in the skin.
Changes in microcirculation are of particular pathogenetic importance in scleroderma. They are based mainly on lesions of the walls of small arteries, arterioles and capillaries, proliferation and destruction of the endothelium, intimal hyperplasia, sclerosis.

Atrophoderma Pasini - Pierini is combined with a strip-like form (in the lumbar region).

Data from clinical and laboratory studies of immune disorders (with changes in both humoral and cellular immunity) indicate their importance in the pathogenesis of scleroderma.
More than 70% of patients with scleroderma have autoantibodies circulating in the blood. In the blood and tissues, an increased content of CD4 + is detected. -lymphocytes and high levels of interleukin-2 (IL - 2) and IL-2 receptors. A correlation has been established between the activity of T-helpers and the activity of the scleroderma process.
R.V. Petrov considers scleroderma as an autoimmune disease, in which the violations are based on the interaction of autoantigens with lymphoid cells. At the same time, T-helpers, activated by exogenous or endogenous factors, produce lymphokines that stimulate fibroblasts. V.A. Vladimirtsev et al. It is believed that an increased level of collagen proteins, being a source of active antigenic stimulation, creates a background against which, with a genetic predisposition, autoimmune reactions are realized. The emerging vicious circle of mutual influence of lymphoid and collagen-synthesizing cells leads to the progression of the fibrous process.
A variety of other autoimmune disorders are observed in scleroderma: various autoantibodies, a decrease in the level of T-lymphocytes with unchanged or elevated content B-lymphocytes, a decrease in the function of T-suppressors with unchanged or increased function of T-helpers, a decrease in the functional activity of natural killers.
In 20 - 40% of cases with plaque scleroderma, antinuclear antibodies are detected, in 30 - 74% of patients with scleroderma, circulating immune complexes are detected.

Classification

The variety of clinical forms and variants of OS, as well as the presence of obliterated (abortive) manifestations of the disease, different degrees of involvement of the skin and underlying tissues in the pathological process make it difficult to diagnose.
A classification of OS based on clinical principle is practically acceptable.
I. Plaque form with its variants (varieties):
1) indurative-atrophic (Wilson);
2) surface "lilac" (Gugereau);
3) keloid-like;
4) knotty, deep;
5) bullous;
6) generalized.
II. Linear form (strip-shaped):
1) saber;
2) ribbon-like;
3) zosteriform.
III. Lichen sclerosus (white spot disease).
IV. Idiopathic atrophoderma Pasini - Pierini.

Clinic

In the dynamics of development, foci of scleroderma usually go through three stages: erythema, skin thickening and atrophy. In some clinical forms, induration is not always pronounced or even absent.
A feature of OS is its clinical diversity. The plaque form is characterized by the appearance on various parts of the skin (in some cases, on the mucous membranes). The plaques are round-oval, less often with irregular outlines. Their size is from one to several centimeters in diameter. The color of the skin in the lesions is pinkish-lilac, liquid. In the center of the plaque, dermatosclerosis is usually formed in the form of a disk of compacted or dense skin, waxy-gray or ivory, with a smooth shiny surface. On the periphery of the focus, there is often a border of a liquid, pinkish-bluish color with a purple tint, which is an indicator of the activity of the process.

Multifocal plaque scleroderma (against the background of congestive hyperemia and pigmentation, foci of dermatosclerosis).

Peripheral plaque growth and the appearance of new lesions usually occur slowly and are not accompanied by subjective sensations. In the foci and adjacent areas of the skin, pigmentation and telangiectasias may occur.
On the affected skin, sweating is reduced or absent, the function of sebaceous glands and hair growth.
An extremely rare type of OS is a bullous, erosive-ulcerative form, which usually occurs against the background of skin sclerosis in the periarticular areas. It can appear on any focus of scleroderma. Consistent formation of vesicular-bullous and erosive and ulcerative lesions associated with dystrophic changes in sclerotic skin. Trauma and secondary infection may play a causal role.

Several foci of plaque scleroderma with severe dermatosclerosis; on the edge of some of them there is a border of pink-brownish color.

With superficial lilac plaque OS (Gugereau), a barely noticeable superficial induration is observed, the skin in the focus is pinkish-lilac with a more intense color at the border of the focus.
With a strip-like form of OS, the foci are linear, in the form of stripes, more often localized along one limb, often along the course of the neurovascular bundle. They can also be located circularly on the trunk or limbs. On the face, scalp, localization of lesions, often cicatricial-saber-shaped (resembling a scar after a saber strike), is noted, which is not uncommon in this form. A dense strand of sclerosed skin can have a different length and width, a brownish color, and a shiny surface.
In the place of its localization on the scalp, there is no hair growth. Vertically, the focus can pass from the scalp, crossing the forehead, back of the nose, lips, chin. Often the mucous membrane of the oral cavity is involved in the process.
When the process is resolved, the surface of the focus is smoothed out, a retraction is formed due to atrophy of the skin, muscles and bone tissue.
Lichen sclerosus (LS) of Zumbusch (synonyms: white spot disease, guttate scleroderma) is considered as a disease that is close in clinic to limited superficial scleroderma, but not completely identical to it.
Clinical manifestations: whitish, almost milky papules with a diameter of 1 - 3 mm, usually rounded in outline, located on unchanged skin. At the beginning of their appearance, they have a reddish color, sometimes surrounded by a barely noticeable lilac border. There may be a recess in the center of the elements. At the confluence of grouped papules, foci with scalloped outlines are formed. These lesions are localized more often on the neck, trunk, genitals, as well as in other areas of the skin and mucous membranes. The rash tends to resolve spontaneously, leaving atrophic hypopigmented or amelanotic patches. Their surface is shiny and wrinkled. Usually the rash is not accompanied by subjective sensations.
The clinical variant of LAL is a plaque-like form with foci reaching several centimeters in size, with rounded or irregular outlines. The skin in such foci is thinned, easily gathers into folds like crumpled tissue paper. In the pemphigodous form, bubbles the size of a pea are formed, transparent contents appear through their thin cover. When the bubbles burst, erosions form.
Diagnosis of OS presents certain difficulties in the early stages of the disease. This is evidenced by frequent cases of diagnostic errors. Delayed by months, and sometimes even years, recognition of the disease carries the risk of developing severe forms that can lead to disability. A consequence of a long progressive course can also be a functional insufficiency of the skin and the musculoskeletal system.
Under the influence of treatment, rarely spontaneously, lesions are resolved (densification, redness, shine disappear) with the outcome in skin atrophy, often leaving vitiliginous or pigmented spots.
Outwardly, the skin resembles parchment. Vellus hair is absent in residual lesions. There is a thinning not only of the skin, but also of the underlying tissues. After the resolution of the scleroderma process in superficial plaque lesions, skin changes are much less pronounced.

Survey

All children with OS, regardless of the clinical form of the disease and the intensity of the lesion, are subject to instrumental examination for the purpose of early diagnosis of visceral pathology, detection of signs of systemic disease. And given the possibility of a latent course of SJS, especially in the early stages of its occurrence, the assessment of the state of internal organs using instrumental methods in children with OS should be carried out at least once every 3 years.
Knowing about the frequent subclinical course of SJS in children or even about the absence of its clinical signs, which are usually nonspecific, the doctor should be alert to possible development systemic process not only with multifocal and widespread manifestations, but also with single limited plaques.
For many years of observation N.N. Uvarova 173 children with SJS, clinically and instrumentally examined, in 63% of cases the disease began with skin lesions (skin syndrome). At the same time, skin changes at the height of the systemic process were noted in all patients. T.M. Vlasova, during a clinical and instrumental examination in 51 (25.1%) of 203 children with OS, revealed visceral changes, i.e. signs of a systemic process. Among them are heart lesions (scleroderma heart - impaired atrioventricular and intraventricular conduction, sinus tachycardia, arrhythmia, S-T interval shift), lungs (increased bronchopulmonary pattern, diffuse or focal pneumosclerosis, cysts in the lungs - "cellular" lung, thickening of the interlobar pleura ), gastrointestinal tract (gastritis, colitis, atony of the esophagus and stomach, arrhythmias, evacuations), kidneys (decrease in effective renal plasma flow, proteinuria).
M.N. Nikitina, when examining 259 children with OS, similar visceral disorders were found. Clinically, it is impossible to draw a line between OS and skin syndrome in SJS.
Children suffering from OS, in the process of multi-course treatment and observation, should be under the constant supervision of a pediatrician, dermatologist and consult other specialists according to indications.

Treatment

The treatment of OS children remains a difficult task. It should be comprehensive and stage-by-course. At the same time, a differentiated approach is important, which takes into account the anamnesis and the results of clinical and laboratory examination, which allows you to prescribe adequate therapeutic measures. In particular, they include sanitation of the body, correction of functional disorders of the nervous, endocrine, immune systems, as well as pathogenetic drugs.
In the progressive stage, inpatient treatment is preferable using penicillin, lidase for dermatosclerosis, dimexide (DMSO), and vitamins. With the stabilization of the pathological process with a tendency to resolve induration and sclerosis, enzyme preparations, immunomodulators, antispasmodics, biostimulants, and pyrogenic preparations are indicated. They fix and enhance the therapeutic effect, and also have a rehabilitation effect of physiotherapy and spa treatment.
Penicillin is recommended to be administered in the progressive stage of the disease at 1 million IU / day in 2-3 injections, for a course of up to 15 million IU in 2-3 courses with an interval between them of 1.5-2 months. Less commonly used semi-synthetic penicillins (ampicillin, oxacillin).
It is assumed that the therapeutic effect of penicillin is due to its structural component - penicillamine, which inhibits the formation of insoluble collagen. The sanitizing effect of penicillin is also allowed in the presence of a focal infection.
From enzyme preparations lidase and ronidase containing hyaluronidase are widely used. The therapeutic effect is associated with the properties of drugs to improve microcirculation in tissues and contribute to the resolution of sclerosis in the foci. For a course of 15 - 20 injections. Lidazu injected intramuscularly in 1 ml with 32 - 64 UE in 1 ml of 0.5% solution of novocaine. The therapeutic effect is increased by combining parenteral administration of the drug with electrophoretic. Courses are repeated after 1.5 - 2 months in the presence of dermatosclerosis.
Ronidase is applied externally, applying its powder (0.5 - 1.0 g) to a napkin moistened with saline. Apply a napkin to the lesion, fixing with a bandage for half a day. The course of applications is continued for 2-3 weeks.
A favorable effect on the resolution of scleroderma lesions has electrophoresis with a 0.5% solution of zinc sulfate. The procedures are carried out every other day for 7-20 minutes, for a course of 10-12 sessions.
Biostimulants (splenin, vitreous body, aloe), activating metabolic processes in the connective tissue, promote tissue regeneration and increase the body's reactivity. Splenin injected 1 - 2 ml intramuscularly, the vitreous body - 1 - 2 ml subcutaneously, aloe - 1 - 2 ml subcutaneously, for a course of 15 - 20 injections.
Pyrogenic drugs increase the body's resistance, stimulate the T-cell link of immunity. Of these drugs, pyrogenal is more commonly used. It is usually used after 2 days on the third intramuscularly, starting from 10 - 15 MPD. The dose, depending on the temperature reaction, is increased by 5-10 MPD. The course consists of 10 - 15 injections.
Immunocorrective effect is exerted by immunomodulators, in particular taktivin and timoptin. Under their influence, normalization of a number of immune parameters and collagen formation occurs. Taktivin is administered daily under the skin, 1 ml of a 0.01% solution for 1 to 2 weeks, 2 to 3 times a year. Timoptin is administered subcutaneously every 4th day for 3 weeks (at the rate of 2 μg per 1 kg of body weight).
Angioprotectors, improving peripheral blood circulation and trophic processes in the lesions, contribute to the resolution of sclerotic changes in the skin. From this group, they use: pentoxifylline (0.05 - 0.1 g 2 - 3 times a day), xanthinol nicotinate (1/2 - 1 tablet 2 times a day), nikospan (1/2 - 1 tablet 2 - 3 times a day), apressin (0.005 - 0.015 g 2 - 3 times a day). One of these drugs is taken in a course lasting 3 to 4 weeks.
DMSO is prescribed externally in the form of a 33 - 50% solution 1 - 2 times a day in repeated monthly courses with intervals between them of 1 - 1.5 months. Compressive dressings or applications are applied to dermatosclerotic plaques until they are noticeably resolved. The drug, penetrating deep into the tissues, has a pronounced anti-inflammatory effect, inhibits the hyperproduction of collagen.
Solcoseryl (a protein-free cattle blood extract), administered intramuscularly at 2 ml per day (20-25 injections per course), improves microcirculation and activates trophic processes in the focus.
Outwardly, in addition to DMSO and Ronidase, drugs are used that improve metabolic processes in the skin and stimulating regeneration: solcoseryl (jelly and ointment), 2% troxevasin gel, vulnuzan ointment, actovegin (5% ointment, jelly), 5% parmidine ointment. Apply one of these funds 2 times a day, rubbing into the lesions. You can alternate these drugs every week, the duration of local applications is 1-1.5 months. Effective in the treatment of children with scleroderma, also madecassol. This herbal preparation regulates the quantitative and qualitative formation of connective tissue, inhibits the excessive formation of collagen.
Adequate external treatment in combination with vasodilators is of great importance in the treatment of vulvar LAL and allows you to refuse from multi-course treatment with penicillin and lidase.
In most girls, the disease has a favorable outcome. The process resolves or decreases to subclinical signs, usually by the time of menarche. The course of other forms of OS is less predictable. A decrease in disease activity, stabilization of the scleroderma process and its regression are usually noted under the condition of early diagnosis of scleroderma and timely implementation of the necessary complex course treatment.

Literature:

1. Dovzhansky S.I. // Scleroderma. - Publishing House of the Saratov University. - 1979. - S. 195.
2. Gintrac M. Note sur la sclerodermie. Rev med Chir 1847;2:263-7.
3. Wienenecke R, Schliipen EM, Zochling N, et al. No evidence for Borrilia burgdorferi - specific DNA in lesions of localized scleroderma. J In vest Derm 1995;104:23-6.
4. Guseva N.G. // Scleroderma group of diseases. - Ter. arch. - 1988. - No. 8. - S. 20-26.
5. Shlyapak E.A., Kuznetsov B.G. // Hormonal shifts in children with scleroderma during spa treatment // Questions of balneology, physiotherapist. and treat. physical education. - 1985. - 6. - S. 40-42.
6. Jablonska S, Bubnow B, Lukusiak. Auswertung von Chronaxie messungen bei Scherodermie. Derm Wschir 1957; 136(31): 831-37.
7. Medsger TA. Systemic sclerosis (scleroderma); eosinophilic fuseiitis and calanosis. In: McCarty D.J., editor. Arthritis and Allied Conditions, 11th edition. Philadelphia: Lea & Febiger 1989;1118-65.
8. Korn J.H. // Immunologic aspects of scleroderma. - Curr. Opur Rheumatol, 1991; 3:947-52.
9. Ferri C, Bernini L, Cecchetti R, et al. Cutaneous and serological subsets of systemic sclerosis. Their relevance in diagnosis, severity and prognosis of the disease. J Rheumatol 1991;18:1826-32.
10. Steen V. Treatment of systemic sclerosis. Curr Opin Rheum 1991;3:979-85.
11. Petrov R.V. // Immunology and immunogenetics. - M. - 1976.
12. Vladimirtsev V.A., Avdeeva Zh.I., Guseva N.G. et al. // Study of cellular immune response to type I collagen in patients with systemic scleroderma. - Q. rheumatism - 1982. - I. - S. 33-38.
13. Kalamkaryan A.A., Dolbin A.G., Fedorova E.G., Zaretskaya Yu.M. // HLA antigens in patients with focal scleroderma - atrophoderma Pasini - Pierini. - Mater. Plenum of the All-Union dermatovenereologists. - M. - 1977.
14. Suchkova T.N. // Violation of T-cell immunity in patients with focal scleroderma // Vestn. dermatol. - 1986. - II. - S. 12-16.
15. Fedorova E.G. // Quantification of populations of T-, B-, null lymphocytes in patients with focal scleroderma // Vestn. dermatol. - 1980. - 6. - S. 17-19.
16. Butov Yu.S. // Antinuclear antibodies in patients with systemic diseases // Vestn. dermatol. - 1980. - II. - 17-21.
17. Suvorov A.P., Zavyalov A.I., Grashkina I.G. // Limited scleroderma. - Methodical. recommendation, Saratov. - 1990. - S. 25.
18. Sonnichsen N. Vergleichende Unier suchuiigen zur pathogenese der Lupuserythematodes, der Scherodermic and der Dermatomyasitis - Dtsch Gesundheitswes 1983;39(14):526-30.
19. Ganchev B. // Dermato-venereological terminology. Sofia. - 1968.
20. Jablonska S. Classification of Scleroderma. Clinic in Dermatology 1994;12(2):225-8.
21. Uvarova N.N. // Clinical picture and the course of systemic scleroderma in children. - Abstract. dis. doc. honey. Sciences. - M. - 1989. - S. 47.
22. Vlasova T.M. // Features of limited scleroderma in children and its possible connection with systemic scleroderma. - Abstract. dis. cand. honey. Sciences. - M. - 1984. - S. 26.
23. Nikitina M.N. // Limited scleroderma in children. Questions of the clinic, pathogenesis and treatment. - Abstract. dis. doc. honey. Sciences. - M. - 1980. - S. 37.

People of any age are affected. So, in the sanatorium "Red Storm" in Sochi, S. I. Dovzhansky from 1962 to 1965 observed 115 children with various forms of this disease, which amounted to slightly less than 3% of the total number of patients skin diseases. A. A. Studnitsin says that scleroderma occurs frequently in childhood, and recently cases of this disease have become more frequent.

Conventionally, two clinical forms of this disease are distinguished: diffuse (systemic) and focal (limited). Until now, the question of the relationship between the systemic and focal forms of the disease has been discussed. So, if according to A. A. Studnitsky both forms are a single process, then G. Ya. Vysotsky claims that these are different independent diseases.

Pathogenesis and etiology

To date, the etiology of the disease remains unclear, as for the pathogenesis, there are also many questions. At the same time, in the formation of the sclerosing process, great importance is attached to the infectious-allergic concept.

The development of virology and electron microscopy has led to an increase in the cases of detection of waste products of viruses in the tissues and blood of patients with scleroderma. So, during an electron microscopic examination of muscle tissues biopsied from patients, J. Kudejko found cellular inclusions resembling viruses.

It is rather difficult to compile a list of all kinds of neuroendocrine, visceral, metabolic disorders that can be attributed to the pathogenesis of scleroderma. Known a large number of cases of this severe dermatosis in patients with functional disorders of the thyroid, genital, parathyroid glands, after hypothermia, injuries and so on. It is believed that this disease can develop as an orthodox allergic reaction in response to the penetration of heterogeneous proteins into cells and, accordingly, the formation of aggressive autoantibodies. Actually, this is precisely what can explain the cases of the disease after vaccinations, the introduction of therapeutic sera, and blood transfusions.

Various metabolic, endocrine, genetic, neurological pathological factors in combination with the damaging effects of exogenous factors (radiation exposure, cooling, trauma) contribute to the emergence, formation of deep autoimmune and dysproteinemic processes that are localized in the system of connective tissue of the skin, blood vessels, internal organs.

The limited form of scleroderma includes patchy, strip-like, plaque forms. Scleroderma systemic may also appear in different forms.

Limited scleroderma. It begins with the appearance of an edematous spot, which early stages characterized by a pale pink or mauve color. The boundaries of the foci are indistinct, and the sizes can vary over a fairly wide range - from a coin to the palm of an adult. It is characterized by an edematous-dense consistency. Over time, the color in the center of the spot becomes paler, approaching the color of ivory, while a pinkish-bluish halo remains along the edges. With the loss of inflammatory color, the lesion becomes dense in consistency, then the density increases. The surface of the affected skin becomes shiny, while there is a smoothness of the skin pattern, lack of hair, dryness due to the lack of sebum and sweating, and reduced sensitivity. The skin is very difficult to fold.

Further, the disease proceeds according to the atrophic type: the lilac ring disappears, the seals become less pronounced, and the infiltrate is replaced by scar connective tissue. Summing up, we can say that in the clinical course of the plaque form of scleroderma, three stages are distinguished: inflammatory edema; the appearance of a seal; atrophy. As a rule, foci of plaque scleroderma are located on the neck, trunk, lower and upper limbs, and sometimes on the face.

As for the second variety of focal scleroderma, strip-like (ribbon-like, linear), it is localized most often in the face, mainly on the forehead. It is this form of the disease that can most often be observed in children. The disease also begins with the appearance of an erythematous spot, which gradually passes into the stage of edema, then compaction and atrophy. In addition to the face, foci of scleroderma can be localized along the limbs and along the trunk along the reflexogenic zones of Zakharyin-Ged, nerve trunks.

Superficially localized areas of linear and plaque scleroderma regress without pronounced atrophy or leave mild dyschromia as a result. However, in most patients (children) with both forms of the disease, there is a deep lesion of the underlying tissues with the development of ulceration, as well as mutations.

The disease of white spots can be characterized by the formation of atrophic depigmented spots of various sizes with clear boundaries of oval or round outlines. They are distinguished by a shiny, wrinkled surface with a smoothed skin pattern and the absence of vellus hair. Shoulders, forearms, neck, upper chest can be noted as places of localization. Patients complain about mild itching in the area of ​​localization, a feeling of constriction.

Systemic or diffuse scleroderma

This disease usually occurs after stressful situations, injuries, cooling with consequences (ARVI, influenza, tonsillitis, herpes simplex, shingles). It is characterized in the prodromal period by ailments, chills, pain in the joints, muscles, insomnia, headaches, fever, severe fatigue, combined with coldness, blanching of the skin of the face, feet, hands.

The disease begins with symptoms of Raynaud's syndrome: vascular spasms, a feeling of coldness, cyanosis, numbness, pain, paresthesia, combined with soreness and stiffness of the joints of the hands. Further, there is a thickening of the skin of the fingers on the hands - the skin becomes stretched, smooth, cold, acquires a pale red tint. Often the fingers are fixed in a bent position.

With systemic, diffuse scleroderma on initial stage the hands and face are affected, then the limbs and torso. With the progression of the disease, a change in skin color from whitish-gray to yellowish is observed, thickening increases, and vellus hair falls out. The fingers of the feet and hands become thinner and sharper, the movements of the joints become difficult, the skin is fixed to the underlying tissues. Rigidity, tension, pallor of the skin, its cooling are aggravated by numbness, paresthesia. The skin peels off in places, ulcerations, cracks form, mutations develop, the fingers become like drumsticks or fingers of labor.

As a result of atrophic and sclerotic lesions of the skin, facial muscles, subcutaneous tissue, the nose becomes sharp, the cheeks sink, the mouth opening becomes folded, narrows, and the lips become thinner. The face becomes monochromatic (bronze), mask-like, amimic. Very often, the mucous membranes of the tongue and mouth are also involved in the process. The border of the lips can peel off, sores and cracks appear. Difficulty eating and swallowing. The atrophic process captures the aponeurosis on the scalp, manifestations are visible, multiple telangiectasias, hair falls out.

There are three stages of the disease: edema, induration and atrophy, which only emphasizes the clinical similarity of the diffuse form with limited forms of the disease. However, in the case of the systemic form, lesions of the gastrointestinal tract, cardiovascular system, damage to the lungs, endocrine glands and kidneys, bones, joints, and muscles come to the fore.

As for the diagnosis during the development of clinical symptoms, it does not present any particular difficulties in view of characteristic appearance lesions. However, at the initial stage of the focal plaque form of the disease, when only inflammatory edema is observed, the diagnosis is complicated, and it is necessary histological examination. It's not easy to hold differential analysis during the primary manifestations of the diffuse form of scleroderma - at this stage, the symptoms of the disease are similar to the symptoms of Raynaud's disease.

Treatment of scleroderma

Treatment of children begins with the appointment of vitamins A, E, C, which contribute to the normalization of the connective tissue. Since inhibition of hyaluronidase activity is observed, it is optimal to use enzymes - ronidase, vitreous body, lidase. Antibiotics, usually penicillin, are prescribed for any form of the disease.

N. A. Slesarenko and S. I. Dovzhansky treat patients with proteolytic enzymes, prescribing intramuscular injections of chymotrypsin and crystalline trypsin every other day for a course of 10-15 injections. Proteolytic enzymes are also administered by electrophoresis or ultrasound.

Availability endocrine disorders in children with scleroderma - an indication for the appointment of drugs of the pituitary gland, sex hormones, parathyroid glands, thyroid gland. Due to pronounced changes in microcirculation in any form of the disease, vasodilators are also used in complex therapy - noshpu, complamin, andekalik, nikospan, depopadutin.

With the appearance of inflammatory edema, characteristic of the initial stage of a disease such as scleroderma, treatment is carried out with glucocorticoids - urbazoom, prednisolone, triamcinolone, dexamethasone - both inside and into the lesions intradermally in small doses. Low molecular weight dextrans, the introduction of which is pathogenetically justified, being hypertonic solutions, can cause an increase in plasma volume, reduce blood viscosity, and improve blood flow. Thiol compounds are able to break down collagen, therefore, unithiol is often used in the treatment, which not only improves the general condition, but also reduces skin density, the growth zone of lesions, ensures the disappearance of pain in muscles and joints, and improves the activity of the liver and heart.

Various means physiotherapy used in the treatment of the disease include diadynamic Bernard currents, ultrasound, indirect and local diathermy, electrophoresis and phonophoresis of lidase, ichthyode, potassium iodide, ozokerite, paraffin applications, therapeutic mud, radon and hydrogen sulfide baths. Remedial gymnastics, oxygen-thalassotherapy, massage are also useful.

focal scleroderma ends with recovery. As for the systemic, diffuse form of scleroderma, it occurs for a long time, with periods of remission, which are replaced by relapses of the disease, which makes it difficult to predict the outcome of treatment. Patients with any form of the disease are subject to clinical examination.

Egallohit cream, which contains green tea extract, is very effective. Main active substance of this cream is epigallocatechin-3-gallate. Egallochit is characterized by pronounced antioxidant and restorative properties, promoting healing, as well as preventing the appearance of pathological scars of various origins.

The cream is able to activate the natural processes of skin regeneration, in addition, it inhibits the processes premature aging, normalizes metabolic processes, and also increases the resistance of the skin to the negative effects of the external environment.

Egallohit is used as a prophylactic agent for the formation of keloid, hypertrophic, atrophic scars. Is different high efficiency with focal scleroderma, vitiligo, skin sarcoidosis as part of complex therapy - the course of application is at least 3 months.

Popular

And focal (limited). It is the systemic course of this disease that is considered the most dangerous, since in this case, internal organs, musculoskeletal system and fabrics. Localized scleroderma is characterized by less aggressive manifestations, accompanied by the appearance of patches (stripes or spots) on the skin. white color, which somewhat resemble scars, and has a favorable prognosis. This ailment is more often detected in the fair sex and 75% of patients are women 40-55 years old.

In this article, we will introduce you to the causes, symptoms, and treatments for localized scleroderma. This information will be useful for you, you will be able to suspect the onset of the development of the disease in time and ask the doctor about treatment options.

In recent years, this skin disease has become more common, and some experts say that its course is more severe. It is possible that such conclusions were the result of non-compliance with the terms of treatment and medical examination of patients.

Causes and mechanism of the development of the disease

The exact causes of focal scleroderma are not yet known. There are a number of hypotheses that indicate the possible influence of some factors contributing to the appearance of changes in collagen production. These include:

• leading to the production of antibodies against your own skin cells;
• the development of neoplasms, the occurrence of which in some cases is accompanied by the appearance of foci of scleroderma (sometimes such foci appear several years before the formation of a tumor);
• concomitant connective tissue pathologies: , rheumatoid arthritis, etc .;
• genetic predisposition, since this disease is often observed among several relatives;
• past viral or bacterial infections: human papillomavirus, streptococci;
• hormonal disorders: abortion, pregnancy and lactation;
• excessive exposure to the skin of ultraviolet rays;
• severe stress or choleric temperament;
• traumatic brain injury.

With focal scleroderma, there is an excessive synthesis of collagen, which is responsible for its elasticity. However, with the disease, due to its excess amount, thickening and coarsening of the skin occurs.

Classification

There is no single classification system for focal scleroderma. Specialists will more often use the system proposed by Dovzhansky S.I., which most fully reflects all the clinical variants of this disease.

1. Plaque. It is divided into indurative-atrophic, superficial "lilac", nodular, deep, bullous and generalized.
2. Linear. It is divided into the "saber strike" type, flight-shaped or strip-shaped and zosteriform.
3. White spot disease (or lichen scleroatrophic, guttate scleroderma, lichen white Tsimbusha).
4. Idiopathic atrophoderma Pasini-Pierini.
5. Parry-Romberg face hemiatrophy.

Symptoms

plaque form

Among all clinical options The most common form of focal scleroderma is the plaque form. A small number of foci appear on the patient's body, which go through three phases in their development: spots, plaques and areas of atrophy.

Initially, several or one lilac-pink spot appears on the skin at once, the size of which can be different. After some time, a smooth and shiny area of ​​yellow-white compaction appears in its center. A lilac-pink border is preserved around this island. It can increase in size, these signs can be used to judge the activity of the scleroderma process.

On the plaque formed, hair loss occurs, the excretion stops sebum and sweat, the skin pattern disappears. A piece of skin in this area cannot be taken with your fingertips in a fold. This appearance and signs of a plaque can persist for a different time, after which the lesion undergoes atrophy.

Linear (or strip shape)

This type of focal scleroderma is rarely seen in adults (it usually occurs in children). The difference between its clinical manifestations and the plaque form is only in the form skin changes- they look like stripes of white color and in most cases are located on the forehead or limbs.

white spot disease

This type of focal scleroderma is often combined with its plaque form. With it, small scattered or grouped spots with a diameter of about 0.5-1.5 cm appear on the patient's body. They can be located on different areas body, but are usually localized on the neck or torso. In women, such lesions can be observed in the area of ​​the labia.

Pasini-Pierini idiopathic atrophoderma

With this type of focal scleroderma, spots with irregular contours are located on the back. Their size can reach up to 10 or more centimeters.

Pasini-Pierini idiopathic atrophoderma is more common in young women. The color of the spots is close to a bluish-violet hue. Their center sinks a little and has a smooth surface, and a lilac ring may be present along the contour of skin changes.

For a long time after the appearance of spots, there are no signs of compaction of lesions. Sometimes these skin changes can be pigmented.

Unlike the plaque variety of focal scleroderma, Pasini-Pierini idiopathic atrophoderma is characterized by the main lesion of the skin of the trunk, and not the face. In addition, rashes with atrophoderma do not regress and gradually progress over several years.

Parry-Romberg facial hemiatrophy

This rare type of focal scleroderma is manifested by an atrophic lesion of only one half of the face. Such a focus can be located both on the right and on the left. Skin tissues and subcutaneous fat undergo dystrophic changes, and muscle fibers and bones of the facial skeleton are involved in the pathological process less often or to a lesser extent.

Parry–Romberg facial hemiatrophy is more common in women, with onset between the ages of 3 and 17 years. The pathological process reaches its activity by the age of 20 and in most cases lasts up to 40 years. Initially, foci of changes of a yellowish or cyanotic hue appear on the face. Gradually, they thicken and over time undergo atrophic changes, representing a serious cosmetic defect. The skin of the affected half of the face becomes wrinkled, thinned and hyperpigmented (focal or diffuse).

There is no hair on the affected half of the face, and the tissues underlying the skin are amenable to gross changes in the form of deformations. As a result, the face becomes asymmetrical. The bones of the facial skeleton can also be involved in the pathological process if the onset of the disease began in early childhood.

Discussions of specialists around the disease

There is an ongoing debate among scientists about the possible relationship between systemic and localized scleroderma. According to some of them, systemic and focal forms are varieties of the same pathological process in the body, while others believe that these two diseases are very different from each other. However, this opinion does not yet find exact confirmation, and statistics indicate the fact that in 61% of cases focal scleroderma transformed into a system.

According to various studies The following 4 factors contribute to the transition of focal scleroderma to systemic scleroderma:

• the development of the disease before the age of 20 or after 50;
• plaque or linear form of focal scleroderma;
• increase in anti-lymphocyte antibodies and large-dispersed immune circulating complexes;
• the severity of dysimmunoglobulinemia and lack of cellular immunity.

Diagnostics

Diagnosis of focal scleroderma is difficult due to the similarity of the signs of the initial stage of this disease with many other pathologies. That is why differential diagnosis is carried out with the following diseases:

• undifferentiated form of leprosy;
• kraurosis of the vulva;
• keloid-like nevus;
• Shulman's syndrome;
• scleroderma-like form;

In addition, the patient is assigned the following laboratory tests:

• skin biopsy;
• Wasserman reaction;
• blood biochemistry;
• general blood analysis;
• immunogram.

Carrying out a skin biopsy allows with a 100% guarantee to make the correct diagnosis of "focal scleroderma" - this method is the "gold standard".

Treatment

Treatment of focal scleroderma should be comprehensive and long-term (multi-course). With the active course of the disease, the number of courses should be at least 6, and the interval between them should be 30-60 days. When the process of progression of foci is stabilized, the interval between sessions can be 4 months, and with residual manifestations of the disease, courses of therapy are repeated for preventive purposes once every six months or 4 months and they include drugs to improve the microcirculation of the skin.

In the stage of the active course of focal scleroderma, the treatment plan may include such drugs:

With scleroatrophic lichen, creams with vitamins F and E, Solcoseryl, Retinol palmate, Actovegin may be included in the treatment plan.

If the patient has limited foci of scleroderma, then treatment may be limited to the appointment of phonophoresis with Trypsin, Ronidase, Chemotrypsin or Lidase and vitamin B12 (in suppositories).

For local treatment of focal scleroderma, ointment applications and physiotherapy should be used. As local preparations are usually used:

• Troxevasin;
• Heparin ointment;
• Theonicol ointment;
• Heparoid;
• Butadion ointment;
• Dimexide;
• Trypsin;
• Chymotrypsin;
• Ronidase;
• Unithiol.

Lidaza can be used to perform phonophoresis or electrophoresis. Ronidase is used for applications - its powder is applied to a napkin soaked in saline.

In addition to the above physiotherapy techniques, patients are recommended to perform the following sessions:

• magnetotherapy;
• ultraphonophoresis with Hydrocortisone and Kuprenil;
• laser therapy;
• vacuum decompression.

At the final stage of treatment, procedures can be supplemented with hydrogen sulfide or radon baths and massage in the area of ​​scleroderma foci.

In recent years, for the treatment of focal scleroderma, many experts recommend reducing the volume of drugs. They can be replaced by means that combine several expected effects. These drugs include Wobenzym (tablets and ointment) and systemic polyenzymes.

With the modern approach to the treatment of this disease, the treatment plan often includes such a procedure as HBO ( hyperbaric oxygenation), which contributes to the saturation of tissues with oxygen. This technique allows you to activate metabolism in mitochondria, normalizes lipid oxidation, has antimicrobial action, improves blood microcirculation and accelerates the regeneration of affected tissues. This method of treatment is used by many specialists who describe its effectiveness.

scleroderma (sclerodermia; Greek skleros hard, dense + derma skin; syn. scleroderma). The term "scleroderma" was first proposed by Gentrak (E. Gintrac) in 1847. Distinguish between systemic and limited S. Systemic S. is characterized by generalized progressive sclerosis of the skin and internal organs, limited - mainly by focal lesions of the skin without signs of systemicity.

Systemic scleroderma

Systemic scleroderma (sclerodermia systemica; syn.: progressive, universal, generalized, diffuse scleroderma, progressive systemic sclerosis) belongs to the group of rheumatic diseases, in particular to diffuse connective tissue diseases (see Collagen diseases). It is a polysyndromic disease, manifested by progressive fibrosis of the skin, internal organs (heart, lungs, gastrointestinal tract, kidneys), a kind vascular pathology type ob-l iterative endarteriolitis with widespread vasospastic disorders.

The incidence, according to various researchers, ranges from 0.27-1.2 per 100 thousand population. Mortality, according to Mazi (AT Masi) et al., is 0.14-0.53 per 100 thousand. Mostly women are ill. According to various statistics, the ratio between the incidence of women and men is 3:1 - 7:1. The average age of patients is 20-50 years. By domestic classification N. G. Guseva (1975), distinguish between acute (rapidly progressive), subacute and chronic systemic S. (the last two variants of the course are more common); typical S. with a characteristic generalized skin lesion and its atypical forms with focal skin lesions; S. with a primary lesion of internals; C., combined with others rheumatic diseases. Rodnan (G. P. Rodnan) and others distinguish the following forms of systemic S.: classic shape with diffuse skin lesions; CREST syndrome - a combination of calcification (see), Raynaud's syndrome (see below), lesions of the esophagus, sclerodactyly and telangiectasias (see); the name of the syndrome is formed from the first letters of the names of its constituent symptoms; S., combined with other rheumatic diseases.

The first descriptions of defeat of separate internals at S. and attempts to present it as generalized process belong to Stephen (J. L. Steven), W. Osler (1898), A. E. Yanishevsky and G. I. Markelov (1907). P. Klemperer's doctrine of collagen diseases served as a powerful impetus for the study systemic manifestations this disease. In 1945, R. H. Goetz proposed the term "progressive systemic sclerosis". The subsequent study of the wedge, manifestations of the disease contributed to the improvement of diagnosis, including atypical and early versions S., served as the basis for further pathogenetic and therapeutic research, for the creation of classifications that summarize the works of the monographic plan, of which the works of E. M. Tareeea, N. G. Guseva, G. Ya. Vysotsky, S. I deserve the most attention. Dovzhansky, Yablonskaya (St. Jablon-ska), Rodnan (G. P. Rodnan), Leroy (E. C. LeRoy), etc.

Etiology

The etiology is not clear; the probability of a viral and hereditary origin of the disease is discussed. The possible involvement of a viral infection in the etiology of systemic S. is indirectly indicated by the detection of virus-like particles in the affected tissues, a virus-specific enzyme (reverse transcriptase) in the bone marrow, and an increase in the titer of antiviral antibodies in the blood serum of patients. The possibility of transplacental "vertical" and "horizontal" transmission of the virus, integration of the virus with the cell genome, activation of a latent viral infection is discussed.

The concept of hereditary transmission of systemic S. is based on Ch. arr. on existence of family cases of a disease, frequent detection immunol. disorders in clinically healthy relatives of patients, a high frequency of chromosomal aberrations (see Mutation) in patients with systemic S.

Cooling, vibration, trauma, contact with certain chemicals. agents (silicon dust, vinyl chloride, etc.), infections, neuroendocrine disorders that precede the development of systemic S. in a number of patients, can be considered as provoking factors. They retain their significance in the theory of polygenic multifactorial inheritance of systemic C.

Pathogenesis

The pathogenesis is complex and includes characteristic changes connective tissue metabolism (see) with an increase in collagen biosynthesis (see) and neofibrillogenesis as the basis of generalized fibrosis, immune disorders and damage to the vascular, microcirculatory bed with the development of a kind of scleroderma angiopathy (eidarteriolitis obliterans, capillary reduction, common vasospastic reactions).

Systemic S. is characterized by hyperactivity of fibroblasts with excessive collagen and fibril formation in violation of intercellular and interstitial interaction of connective tissue components. There is an increase in the content of hydroxyproline (see Proline) in the urine and blood plasma of patients, a significant increase in the rate of collagen biosynthesis in the skin, an increase in the soluble fraction of collagen and the enzyme protocollagen-proline hydroxylase in some patients, ultrastructural signs of increased functional activity of skin fibroblasts and enhanced neofibrillogenesis. Scleroderma-like syndrome in the treatment of bleomycin is also associated with excessive collagen production due to the stimulating effect of the drug on fibroblasts. In the study of a monolayer culture of skin fibroblasts in patients with systemic S., a phenotypically stable hyperproduction of connective tissue components, Ch. arr. collagen, a violation of the functional properties of the fibroblast membrane (an abnormal reaction to adrenaline, etc.) was revealed. Changes in the functions of collagen-synthesizing cells with reduced or “defective” perception of the signal from the regulatory systems of the body can lead to anomalies in the processes of fibril formation (aggregation of collagen fibers, assembly of fibrils, etc.) and tissue fibrosis characteristic of systemic S..

Systemic S. is also characterized by impaired humoral and cellular immunity (see), as evidenced by the combination with various autoimmune diseases and syndromes - hemolytic anemia (see), Hashimoto's thyroiditis (see Hashimoto's disease), Sjogren's syndrome (see Sjogren's syndrome) etc. It often reveals: anti-nucleolar and antinuclear antibodies, including antibodies to the Scleroderma-70 antigen, anticentromeric (to centromeric chromatin) autoantibodies; antibodies and cellular immune responses to collagen; a decrease in the content of T-suppressors with a normal content of B-lymphocytes in the blood; cytopathic effect of lymphocytes; the similarity of skin and vascular changes in systemic S. with the reactions observed during bone marrow transplantation, etc.

Violations of microcirculation (see) and actually scleroderma angiopathy, which plays a leading role in the origin of many wedges, manifestations of systemic S., and often determines the prognosis, in particular during the development of the so-called. true scleroderma kidney.

The blood serum of patients with systemic S. has cytotoxic activity against the endothelium, damage to which is accompanied by adhesion and platelet aggregation (see), activation of coagulation (see), fibrinolysis (see), release of inflammatory mediators (see), increased permeability vascular wall with its subsequent plasma impregnation and deposition of fibrin. Inflammatory mediators enhance endothelial destruction, microthrombosis, and intravascular coagulation, maintaining injury. The subsequent repair of the vascular wall is accompanied by the reduplication of basement membranes, intimal migration and proliferation of smooth muscle cells. The latter, being a kind of fibroblasts, are capable of synthesizing predominantly type III collagen and are largely responsible (under the indicated conditions) for the development of vascular and perivascular fibrosis.

Thus, the microvasculature plays the role of a target organ where contact with a hypothetical damaging agent is made, and it actively participates, along with connective tissue and immune system, in development of patol, characteristic of a system scleroderma. process.

pathological anatomy

Systemic S. is morphologically characterized by severe fibrosis of various organs and tissues. At the heart of tissue damage is vascular damage and excessive production of collagen (see).

The most characteristic changes are observed in the skin. Both with systemic and limited S., there are three stages of skin changes: 1) the stage of dense edema; 2) the stage of induration; 3) stage of atrophy. In the stage of dense edema, signs of increased vascular permeability predominate (see). Hydropic dystrophy of the cells of the basal layer of the epidermis (see. Vacuolar dystrophy), expansion of lymph, crevices, slight disintegration of the collagen bundles of the dermis due to edema, vasculitis (see), telangiectasia (see), inflammatory infiltration around the vessels, skin appendages and in the subcutaneous fiber. Among the cells of the inflammatory infiltrate in the affected tissues, there is a sharp predominance of T-lymphocytes and macrophages with signs of intense phagocytosis (see). Thickened hyalinized bundles of collagen fibers are found in the stage of dense edema only in the deep sections of the reticular (reticular) layer of the dermis. Fleischmajer (R. Fleischmajer) et al. (1980) using immunofluorescence (see) and electron microscopy (see) found that sclerosis begins around the capillaries and near the subcutaneous tissue. Fibroblasts in areas of fibrosis have a developed rough endoplasmic reticulum (see), surrounded by accumulations of thin fibrils (diam. 10-30 nm); there is an increase in the number of thin collagen fibers, immature bundles to-rykh are similar to those that are detected in the skin during the embryonic period.

The stage of induration (Fig. 1) is characterized by sclerosis of the papillary and reticular layers of the dermis with desolation of capillaries, sclerosis of the walls of blood vessels, a decrease in the number of cells, thickening of collagen bundles of the reticular layer and hyaline (see), atrophy of the epidermis and skin appendages, sclerosis and hyalinosis of the subcutaneous fiber. Vasculites are rare at this stage. Cellular infiltrates are usually scanty, represented by 3-5 cells of the lymphoid type.

The stage of atrophy develops many years after the onset of the disease. At gistol. Examination of the skin and subcutaneous tissue reveals fields of hyalinized tissue with diffuse atrophy of the epidermis, alignment of the papillae, abrupt emptying of the vessels of the microcirculatory bed, a decrease in the number of cells, and atrophy of the skin appendages. These skin changes are accompanied by necrosis (see) and trophic ulcers (see). With Tibierzh-Weissenbach syndrome (see below), lime deposits are detected in the subcutaneous tissue. In areas of externally unchanged skin, there is a thickening of collagen bundles in the deep part of the reticular layer of the dermis.

At an active current patol. process vasculitis of arterioles and small arteries have a proliferative character with a circular growth of the inner membrane (Fig. 2). Electron microscopy in the capillaries of the affected tissues reveals vacuolization and destruction of the endothelium, as well as a multilayer basement membrane. According to K meadows (N. K lug) et al. (1977) and others, IgM and complement deposits were found in the walls of small arteries and capillaries in the walls of small arteries and capillaries, as well as under the sarcolemma of muscle fibers.

Skin lesions in systemic S. are often combined with damage to the joints, bones, and muscles. When the joints are affected, exudative-proliferative synovitis is found (see) with fibrinous deposits on the surface of the synovial layer of the joint capsule, focal proliferation of synoviocytes, single productive vasculitis, moderate angiomatosis, lymphoid-macrophage infiltration in the subsynovial and fibrous layers. Articular cartilage in systemic S. loses its elasticity, becomes brittle and wears out quickly; periarticular osteoporosis is noted (see). In the absence of signs of arthritis in the joint cavity, there is virtually no synovial fluid, macroscopically, the synovial layer of the joint capsule becomes dense, devoid of villi. At gistol. the study is difficult to find its organ-specific features: synoviocytes are mostly absent, the synovial layer is covered with hyaline-like masses, the subsynovial layer is represented by fibrous connective tissue poor in blood vessels with extensive fields of hyalinosis. At the system S. which is followed by a myopathic syndrome, gistol. study skeletal muscle reveals a picture hron. myositis (see) with a variety of muscle fibers, hydropic dystrophy and myolysis of some of them, perivascular infiltrates from lymphocytes, macrophages, polynuclear cells, vasculitis, proliferation of granulation and fibrous connective tissue in the endo- and perimysium. More typical is fibrosing interstitial myositis (Fig. 3) with severe sclerosis, lipomatosis, hyalinosis of the epi- and perimysium, sclerosis of the vessel walls, desolation of the capillary bed, small-focal perivascular lymphoid-macrophage infiltration, single vasculitis, focal perifascicular or diffuse atrophy of muscle fibers.

In went.-kish. tract marked atrophy of the mucous membrane and smooth muscles, sclerosis and hyalinosis of the submucosa and serous membrane, sometimes with the development of erosions and ulcers. Atrophy of the smooth muscles of the circular layer is especially pronounced. In subacute systemic S., esophagitis is found (see), enteritis (see Enteritis, Enterocolitis), colitis (see) with proliferative, less often destructive-proliferative vasculitis of the mesentery arteries and the walls of the esophagus and intestines. In the liver, there is periductal, perivascular, less often intralobular fibrosis, sclerosis and hyalinosis of the vessel walls, fatty degeneration hepatocytes. Less often meet hron. active hepatitis (see), primary biliary and macronodular cirrhosis of the liver (see).

In the lungs there is a picture of interstitial pneumonia (see) and basal pneumosclerosis (see). Subpleural localization patol prevails. process; while foci of sclerosis alternate with emphysematous areas and small cysts.

Damage to the heart is morphologically characterized by diffuse small-focal or large-focal cardiosclerosis (see), myocardial hypertrophy of both the right and left ventricles, adhesive pericarditis (see). In 1/3 of cases, there is a diffuse thickening of the endocardium, both parietal and valvular, sometimes with the development of heart defects. In the subacute course of systemic S., a kind of interstitial myocarditis is found (see) with edema and proliferation of connective tissue, proliferative, less often destructive-proliferative in a sculitis of small branches of the coronary (coronary) arteries and arterioles. Occasionally, hyalinosis of the inner and outer shells of the main trunks of the coronary arteries is detected.

With the so-called true scleroderma kidney thrombosis, heart attacks, necrosis of its cortical substance are noted. At gistol. the study determined the proliferation of the intima, mucoid edema, thrombovasculitis of the interlobular arteries, fibrinoid necrosis of the leading arterioles, inflammatory infiltration, degeneration and necrosis of the epithelium of the tubules. Occasionally, fibrinoid necrosis and "wire loops" in the glomeruli of the renal corpuscles occur. However, more often with systemic S., in the kidneys there is a picture of focal or chronic intracapillary proliferative-membranous glomerulonephritis (see). In the outcome of the latter, secondary wrinkling of the kidneys may develop.

C lesion is associated with vasculitis, sclerosis and hyalinosis of the vessel walls. n. With. In autonomic nerve endings, nodes of the sympathetic trunk and autonomic centers of the brain stem, dystrophic changes are detected. In case of development in systemic S. of polyneuritis (see) or polyneuropathy (see Neuropathy, in neurology), both vasculitis of small vessels supplying nerves and sclerosis of epineurium, perineurium of nerve trunks and destruction of axons are noted.

Clinical picture

The clinical picture is polysyndromic, reflecting the systemic, progressive nature of the disease. Systemic S. more often begins gradually with vascular disorders characteristic of Raynaud's disease (see Raynaud's disease), moderate arthralgia (see), less often with arthritis (see Arthritis), dense swelling of the fingers with limited movement and a tendency to form contractures ( cm.); in some cases - with damage to internal organs ( digestive system, heart, lungs). Much less often, there is an acute polysyndromic onset of the disease, often with an increase in body temperature up to 38 ° and above, a rapidly progressive course and generalization of the process in the first 3-6 months. from the onset of the disease. Of the general manifestations of the disease, the most characteristic is a significant, sometimes catastrophic weight loss observed during the period of generalization or rapid progression of the disease. Half of the patients have subfebrile temperature.

Rice. 7. The hand of a patient with sclerodactyly: areas of depigmentation and hyperpigmentation of the skin, deformity and shortening of the fingers due to osteolysis. Rice. 8. Masculine face in a patient with systemic scleroderma. Rice. 9. Face of a patient with systemic scleroderma: pallor of the skin of the face, telangiectasia. Rice. 10. Fingers of a patient with systemic scleroderma: thinning, focal hyperpigmentation, skin tightness, which makes it shiny ("sucked fingers"); a scar at the site of former necrosis at the base of the second finger and fresh necrosis in the area of ​​the interphalangeal joint of the second finger. Rice. 11. The distal part of the foot of a patient with systemic scleroderma: partial amputation of the And finger, dystrophic changes in the nails. Rice. 12. The thigh of a patient with plaque scleroderma: a skin lesion in the form of an ivory-colored area with a shiny surface and a lilac rim.

One of the important diagnostic signs of systemic S. is a characteristic skin lesion that changes the appearance in 80-90% of patients, but at the onset of the disease is observed only in 1/3 of cases. Chl is localized. arr. on the hands - sclerodactyly (printing. Fig. 7), on the face - masking (printing. Fig. 8), upper half of the body, feet; less often (mainly with a rapidly progressive course) there is diffuse lesion skin. Along with the characteristic sclerodermic changes in the skin, passing through the stages of dense edema, induration (see) and atrophy (see), hyperpigmentation is noted, often alternating with areas of depigmentation (see Skin dyschromia), telangiectasia (tsvetn. Fig. 9), trophic disorders(nail deformity, baldness). In some patients, there is a skin lesion according to the type of limited C. Often there is a lesion of the mucous membranes - hron. conjunctivitis (see), atrophic and subatrophic rhinitis (see), stomatitis (see), pharyngitis (see) and damage to the salivary glands, in some cases Sjogren's syndrome (see Sjogren's syndrome).

Raynaud's syndrome is an early and frequent sign of systemic S., occurs, according to various researchers, in 70-90% of patients. Unlike Raynaud's disease, Raynaud's syndrome with systemic S. is more common: vascular changes are noted on the hands, feet, sometimes in the face, similar changes are in the lungs and kidneys. Often, Raynaud's syndrome long precedes articular and skin manifestations or develops simultaneously with them. Factors such as cooling, vibration, emotional lability aggravate existing microcirculation disorders, contribute to the progression of Raynaud's syndrome and the occurrence of vascular-trophic changes (printing. Fig. 10) - repeated ulceration of the tissues of the fingertips up to the development of gangrene (see) .

The defeat of the musculoskeletal system is noted in all patients with systemic S. and is one of the reasons for the disability of these patients. Articular syndrome is often observed; it is one of the initial signs of the disease. There are three main variants of it: 1) polyarthralgia; 2) polyarthritis with a predominance of exudative-proliferative (rheumatoid-like) or fibrous-inductive changes; 3-) periarthritis with deformity of the joints and the development of contractures, mainly due to damage to the periarticular tissues. Muscle damage in systemic S. is manifested more often by fibrous interstitial myositis with the development of contractures, less often by true myositis with progressive muscle weakness and movement disorders, as in dermatomyositis (see).

Changes in bones in the form of osteolysis (see), more often distal (nail) phalanges are characteristic, which manifests itself clinically in the form of shortening (tsvetn. fig. 11) and deformation of the fingers and toes. Systemic S. is characterized by soft tissue calcification, known as Tibjerzh-Weissenbach syndrome. Deposits of calcium salts are localized mainly in the area of ​​the fingers and periarticularly - around the elbow, shoulder and hip joints, in the subcutaneous tissue, sometimes along the fascia and muscle tendons. Tissue calcification develops gradually, usually not earlier than 5 years from the onset of the disease. More often, tissue calcification does not cause discomfort and is detected only radiographically, and when it is localized in the fingers, it is detected by deformation of the latter. With a more rapid, more often by the type of individual exacerbations, development of the process, infiltration of tissues with a pronounced pain syndrome, deterioration of the general condition and sometimes a feverish reaction are detected. With a superficial location, foci of calcification can open with the release of a white, crumbly or liquid mass.

The defeat of the digestive tract, especially the esophagus and intestines, is observed in 60-70% of cases and has a characteristic clinical and radiological picture. Changes from a gullet can be noted at early stages of a disease; they are manifested by dysphagia (see), weakening of peristalsis (see), expansion of the upper third and narrowing of the lower third of the esophagus, rigidity of its walls. Later, the phenomena of reflux esophagitis join (see Esophagitis), which is accompanied in a number of cases by the development of peptic ulcers (see), strictures, hiatal hernia (see). Scleroderma bowel disease is manifested by dilatation duodenum, duodenitis (see), sacculation of the colon, malabsorption syndrome (see Malabsorption syndrome) and persistent constipation, sometimes with symptoms of partial intestinal obstruction (see).

Damage of a liver is shown by its increase, in some cases - a skin itch, periodically arising jaundice that testifies to hron. hepatitis (see) or cirrhosis. Changes in the pancreas are rarely detected, mainly in functional studies.

Lung damage is observed in approximately 2/3 of patients; it is characterized by the gradual development of diffuse peumosclerosis (compact, rarely cystic) with predominant localization in the basal regions, as well as the presence adhesive process and thickening (fibrosis) of the pleura. Wedge, signs of a pneumosclerosis (see) in an initial stage are insignificant or absent, while functional disturbances and rentgenol. the changes are already there. Therefore, the use of these research methods for the early diagnosis of scleroderma pneumofibrosis is recommended. The severity and severity of pneumofibrosis is primarily due to the activity of the scleroderma process. Patients with subacute S. have interstitial pneumonia (see). With severe pneumofibrosis, bronchiectasis, emphysema, perifocal pneumonia, and respiratory failure develop.

Damage to the heart, in particular the myocardium, is the leading sign of damage to internal organs in systemic S. both in frequency and significance, since in some cases it leads to death. Scleroderma cardiosclerosis (see), which underlies myocardial damage, is characterized by an increase in the size of the heart, rhythm disturbance (more often - extrasystole) and conduction, weakening contractile function with areas of adynamia, detected by X-ray kymography (see) and especially clearly by echocardiography (see). Large-focal myocardial fibrosis is accompanied by heart attack-like changes on the ECG and in some cases can lead to the development of a kind of "callosal" heart aneurysm. With systemic S., it is possible to damage the endocardium of the valves with the formation of heart disease, more often the left atrioventricular - mitral (see. Acquired heart defects), To-ry is characterized by a relatively benign course with a rare development of decompensation. Wedge, and rentgenol. the picture of heart disease is not always clear due to simultaneous damage to the myocardium and pericardium. Scleroderma pericarditis (see) is predominantly adhesive in nature, although the section quite often registers an increase in fluid in the pericardial cavity (transudation disorders).

In 1/3 of patients, usually with subacute and chronic course of systemic S., a subclinical form of kidney damage is detected, which is detected during functional studies, for example, renography using 131 I hippuran (see Radioisotopy renography), as well as signs latent and, rather seldom, hypertensive, nephrotic or mixed type (at a subacute course) glomerulonephritis (see).

Described as. true scleroderma kidney - a condition characterized by catastrophic acuteness of the disease (2-4 weeks) and death. It is characterized by proteinuria (see), signs of rapidly increasing kidney failure(see) - azotemia (see), oliguria (see) and terminal anuria (see), arterial hypertension (see Arterial hypertension), retinopathy (see) and encephalopathy (see). Similarity of nek-ry pathogenetic lines and morfol is noted. signs of a true scleroderma kidney with malignant arterial hypertension. With severe arterial hypertension is found high level renin in blood plasma. The true scleroderma kidney develops, as a rule, in acute, rapidly progressive systemic S. and is the main cause of death in patients with this variant of the course of the disease.

Damage to the nervous system in systemic S. is common. The leading syndrome is neuro-circulatory dystonia (see). Already in the early stages of the disease, secretion is disturbed sweat glands: first there is hyperhidrosis of the palms and axillary areas(see Hyperhidrosis), and then reduced sweating in areas of skin atrophy. Vegetative-vascular and related trophic disorders are manifested by peeling of the skin, hyperkeratosis (see), hair and eyelash loss, impaired nail growth, increased sensitivity to cold, a decrease in skin temperature by 1-2 °, the absence of local and reflex dermographism (see).

At system S. the polyneuropathic syndrome quite often meets (see. Polyneuritis ). According to N. G. Guseva, it is observed in 1/3 of cases of the disease. Basically, the polyneuropathic syndrome is manifested by sensory disturbances, patients complain of paresthesia (see) in the arms and legs, sometimes in pain. The study reveals pain along the nerve trunks, hyperesthesia, and sometimes hypesthesia or hyperpathy in the distal extremities in the form of "gloves" and "socks". Movement disorders with S. are not characteristic, although, according to V. V. Mikheev, the development of atrophic paresis of the hands and paralysis of the feet is possible. Despite the frequent absence of gross paresis and sensitivity disorders, early extinction of tendon reflexes in the arms and legs is quite typical, up to complete areflexia (see). The presence of symptoms of Lasegue tension is characteristic (see Radiculitis).

Defeat c. n. With. is rare. It is manifested by a meningo-encephalitic syndrome (see Encephalitis) or vascular disorders of a hemorrhagic or ischemic nature. Acute violation cerebral circulation(see) can be fatal. Meningoencephalitic syndrome is characterized by headaches, dizziness and mild focal symptoms. Quite typical is a change in the psyche with anxiety-depressive reactions, sometimes the development of an acute psychotic state with delirium, auditory and olfactory hallucinations, and amnesia. The pressure of the cerebrospinal fluid is increased, its protein content is increased. Nipple edema may develop optic nerve(optic disc, T.).

The spinal cord is rarely affected, there are single descriptions of the development of symptoms of myelitis (see) and myelopolyradiculoneuritis (see). These phenomena are due to vascular disorders associated with the underlying disease.

There are three main variants of the course of systemic S.: acute (rapidly progressing), subacute and chronic, to-rye differ from each other in the activity and speed of progression patol. process, severity and nature of peripheral (skin, articular, etc.) and visceral manifestations. For the most frequent hron. the course is characterized by progressive vasomotor disorders (Raynaud's syndrome) and the pronounced trophic disorders caused by them. They are often the only manifestation of the disease for a number of years and further prevail in the picture of the disease. At hron. During the course of laboratory tests, they usually remain within the normal range or close to them, with the exception of moderate hyperproteinemia and hypergammaglobulinemia in 1/3 of patients.

The subacute course is characterized by the presence of dense edema of the skin with its subsequent induration, recurrent polyarthritis (sometimes like rheumatoid), less often - myositis with myasthenic syndrome, polyserositis (see), visceral pathology - interstitial pneumonia with the subsequent development of pneumosclerosis, cardiosclerosis, scleroderma esophagitis (see .), duodenitis (see), hron. glomerulonephritis, as well as vasomotor and trophic disorders.

The acute, rapidly progressive course is characterized by an unusually rapid (already in the first year of the disease) development of diffuse S., steady progression of lesions of internal organs, rapidly increasing fibrosis of organs and tissues, and severe vascular pathology with frequent kidney damage like a true scleroderma kidney.

Diagnosis

The diagnosis at the developed picture of a disease does not cause difficulties; he is based on arr. on a wedge, S.'s manifestations in combination with laboratory, radiological and morphological (skin biopsy) data.

In accordance with the criteria of the American rheumatological association(1979) the diagnosis of "definite" systemic S. can be established in the presence of a "major" criterion, which is considered to be proximal (in relation to the fingers) scleroderma skin changes, or - two of the three "small" criteria - sclerodactyly, trophic ulcers of the fingertips , bilateral basal pulmonary fibrosis.

Early diagnosis of systemic S. is based on the presence of Raynaud's syndrome in combination with persistent arthralgias (less commonly, arthritis) and (or) moderate flexion contractures, dense swelling of the fingers, face, and, more rarely, characteristic lesions of internal organs (esophagus, lungs, heart).

Blood at systemic S. is changed a little, only some patients have hypochromic anemia (see), leukopenia (see), more often - leukocytosis (see). Accelerated ROE along with an increase in the content of fibrinogen (see), alpha 2-globulins (see. Globulins), ceruloplasmin, the appearance of a C-reactive protein (see) reflects the activity of patol. process. In the red bone marrow, a plasmacytic and reticulocyte reaction is often detected. Approximately half of patients with systemic S. have hyper-gammaglobulinemia, causing a tendency to hyperproteinemia; in some cases - monoclonal gammopathy. According to various researchers, in 40-60% of cases, rheumatoid factor (see), antinuclear antibodies (in 36-91%) and LE cells (in 2-7% of cases) are found in the blood serum of patients, which brings this disease closer to rheumatoid arthritis (see) and systemic lupus erythematosus (see). Systemic S. is characterized by the presence of special antinuclear antibodies to the so-called. Scleroderma-70 antigen and anticentromeric antibodies (the latter are detected mainly in the CREST syndrome, i.e., the chronic course of the disease). Some patients have cryoglobulinemia. In 40-60% of patients with systemic S., an increase in the content of hydroxyproline in blood plasma and urine is detected, which indicates pronounced disorders of collagen metabolism.

X-ray. the research at system S. has important a wedge, value since, specifying a picture of a disease, promotes the solution of a question of the diagnosis. Use of various rentgenol. methods depends on which organs and systems are to be studied.

Typical for systemic S. changes in the soft tissues, bones and joints (Fig. 4) are areas of calcification (see) in the subcutaneous tissue, mainly the end sections of the fingers, less often - the feet, the area of ​​the elbow, knee and other joints. Osteolysis (see) is observed in the nail phalanges of the fingers, feet, coronoid processes of the branches of the lower jaw, the distal sections of the radius and ulna, the posterior sections of the ribs and some other bones. There are periarticular osteoporosis (see), narrowing of the joint spaces, sometimes single erosions on the surface of the articular cartilage and bone ankylosis (see), more often in the joints of the wrist.

Of great importance for the diagnosis of systemic S. is rentgenol. research went. - kish. path, because it allows you to identify one of the most specific signs of the disease - a decrease in tone and a weakening of peristalsis, which leads to an expansion of the lumen of the organ and prolonged stasis of barium suspension. Most often, such changes occur in the esophagus, duodenum and jejunum (Fig. 5), less often in the stomach and large intestine.

When the lungs are affected radiographically in the basal sections diffuse and cystic pneumosclerosis is determined (see), often combined with moderate emphysema of the lungs (see), as well as signs of adhesive (adhesive) pleurisy (see).

X-ray. symptoms of heart damage are detected in almost 100% of cases and are characterized by changes in its configuration due to an increase in the size of the left ventricle and right sections (due to the development of pneumosclerosis and pulmonary hypertension). Typical is a decrease in the amplitude of the pulsation up to the zones of adynamia (Fig. 6), which is well detected by X-ray kymography (see). There may be signs of damage to the valvular apparatus, mainly in the form of insufficiency of the left atrioventricular (mitral) valve, in some cases, stenosis of the left atrioventricular orifice and aortic valve insufficiency (changes in configuration, size of the heart cavities, as well as the nature of the heart pulsation).

Systemic S. should be differentiated from diseases of the so-called. scleroderma group (limited S., eosinophilic fasciitis, scleredema Bushke), with others diffuse diseases connective tissue, rheumatoid arthritis (see), with a group of pseudosclerodermic conditions.

Features a wedge, pictures make it relatively easy to distinguish between systemic and limited S., however, one should keep in mind the possibility of focal skin lesions in systemic S. Differential diagnosis with eosinophilic fasciitis is based on the diffuse induration of the deep layers of the fascia and subcutaneous tissue, which is characteristic of the latter (established by biopsy), predominantly in the forearms, less often in the legs, torso, eosinophilia of the blood and often tissues, as well as the absence of Raynaud's syndrome and damage to internal organs in eosinophilic fasciitis. With scleredema Bushka, in contrast to systemic S., the initial localization of the process is noted in the neck and face; predominantly subcutaneous tissue is affected.

At rheumatoid arthritis, especially in juvenile rheumatoid arthritis, Raynaud's syndrome, thinning and trophic changes in the skin of the fingers are possible. On the other hand, in some cases, with systemic S., polyarthritis develops, resembling joint damage in rheumatoid arthritis. Difficulties in differential diagnosis in these cases can be resolved by taking into account characteristic symptoms and process dynamics.

The nature of the disease as a whole, as well as the features of vasospastic disorders and damage to internal organs, usually make it possible to distinguish systemic S. from dermatomyositis (see) and poikilodermatomyositis (see) even in the presence of similar features (flexion contractures of the limbs, masking of the face, dysphagia). Polymyositis (see Myositis) may be a manifestation of systemic S., but unlike skeletal muscle damage in dermatomyositis, it rarely prevails in the picture of the disease and only for a short time. Differential diagnosis with systemic lupus erythematosus (see) is usually not difficult. It should be borne in mind that antinuclear factor, antibodies to DNA (usually in a small titer), single LE cells can be observed with systemic S. more often than subacute course.

Pseudosclerodermic syndromes in primary amyloids, congenital metabolic disorders - porphyria (see), phenylketonuria (see), hepato-cerebral dystrophy (see), with certain endocrinopathies, for example, Werner's syndrome (see Werner's syndrome) and paraneoplastic syndromes (see), are characterized by hl. arr. musculoskeletal, less often - vascular symptoms, reminiscent, but not identical to the manifestations of systemic S. Atypism of peripheral and the absence of lesions of internal organs characteristic of S., along with a wedge, features of iseudoscleroderma-syndromes, are the basis of differential diagnosis.

Systemic S. should be differentiated from such dermatol. diseases, as hron. atrophic acrodermatitis (see) and scleroatrophic lichen with a predominant lesion of the mucous membranes and their secondary progressive sclerosis, which may be accompanied by a narrowing of the lumen of the esophagus, vagina. Full wedge, examination of the patient, specification of character local lesion and dynamics patol. processes make it possible to distinguish between these diseases.

Treatment

Treatment of patients with systemic S. is carried out for a long time (for years). When choosing a complex to lay down. measures, it is necessary to take into account the nature of the course, activity and stage of the disease. Of the drugs used are D-penicillamine, unithiol, corticosteroids, aminoquinoline drugs, immunosuppressants, non-steroidal anti-inflammatory, vasodilators and antiaggregants, lidase, dimethyl sulfoxide, less often centrally acting muscle relaxants, colchicine, heparin, grpseofulvin, etc.

D-penicillamine inhibits the maturation and, in part, the biosynthesis of collagen. Applies Ch. arr. in acute and subacute course of the disease in gradually increasing doses: starting from 300 mg to 1-2 g per day, followed by a transition to a maintenance dose (300 mg per day). Treatment is carried out for a long time - for 2-3 years (sometimes up to 5). In 1/3 of patients, adverse reactions are observed: dermatitis, dyspeptic disorders, loss of taste, fever, leukopenia and thrombocytopenia, drug nephropathy. With long-term treatment with D-penicillamine, indurative skin changes, articular and vascular syndromes are clearly reduced. The effect of the drug on visceral pathology is less clear. In some observations, under the influence of treatment, the transition of an acute course to subacute and even chronic was noted.

Unithiol, like D-penicillamine, contains sulfhydryl groups and affects collagen metabolism; it can be applied in complex treatment C. Prescribe repeated courses of treatment with unithiol; administer 5 ml of 5% solution intramuscularly, for a course of 10-12 injections.

Glucocorticosteroids (mainly prednisolone) are prescribed in the presence of a wedge, and a lab. signs of activity patol. process, in acute and subacute course and rarely (short courses lasting 1-2 months) with exacerbation of chronic C. The initial dose is 30-40 mg per day (in combination with D-penicillamine - 20 mg); it is used for 1-2 months. before achievement a wedge, effect. Subsequently, when the process stabilizes, the dose of the drug is gradually reduced to a maintenance dose (20-15-10 mg per day). Glucocorticosteroids are used for a long time; adverse reactions are rare. Glucocorticosteroids are effective in articular, skin and vascular syndromes, nek-ry visceral manifestations (myocarditis, interstitial pneumonia). They are not indicated in the development of a true scleroderma kidney.

Aminoquinoline derivatives (chloroquine, rezoquine, plaquenil) are used as the main type of treatment for subacute and especially hron. during systemic C. Assign 0.25 g of chloroquine or 0.4 g of plaquenil per day for a long time (2-3 years) under the control of blood tests and the supervision of an oculist. These drugs have a positive effect mainly in the articular syndrome.

Non-steroidal anti-inflammatory drugs (acetylsalicylic acid, brufen, voltaren, indomethacin, etc.) are prescribed to patients with systemic S. most often with articular syndrome. Cytostatic immunosuppressants (azathioprine, cyclophosphamide, chlorbutine, etc.) are used relatively rarely in systemic S., Ch. arr. at high activity patol. process that is not amenable to the effects of corticosteroids, or in case of contraindications to their treatment. Azathioprine is preferred for kidney damage like glomerulonephritis. It is prescribed at 1-3 mg per 1 kg of body weight (weight) of the patient (50-200 mg per day) for 2-3 months. under the control of blood tests. Of the vasodilators and antiplatelet agents in systemic S., complamin, anginin, andecalin, nicotinic acid preparations, griseofulvpi, chimes, low molecular weight dextran, etc. are used. Hyperbaric oxygenation improves peripheral circulation (see), balneotherapy and physiotherapy.

With polyneuropathy, in addition to these drugs, vitamins of group B and repeated courses of adenyl 1 ml 2 times a day for 1 month are prescribed, as well as massage and exercise therapy.

With the development of a true scleroderma kidney, massive antihypertensive therapy is necessary, including inhibitors of the renin-angiotensin system, repeated hemodialysis (see), in some cases a kidney transplant is indicated (see).

Lidazu apply at hron. during systemic S. repeated courses in the form of subcutaneous injections of 64 - 128 IU (for a course of 12-14 injections) or electrophoresis on the affected skin.

Dimethyl sulfoxide is prescribed in the form of applications to the affected areas of the skin, it can be combined with nicotinic acid preparations, Trilon B, analgesics.

In the presence of calcification, treatment with Na2 EDTA, which has a chelating effect, is indicated.

San.-kur. treatment with the use of balneotherapy (radon, hydrogen sulfide, carbonic baths), mud therapy, etc. is shown in Ch. arr. at hron. during systemic S. In the absence of contraindications, early inclusion in the therapeutic complex of massage and to lay down is possible. physical education.

Forecast and Prevention

The prognosis is determined by the nature of the course, the timeliness of diagnosis and the adequacy of therapy. At hron. During the course, the prognosis is favorable, with subacute - satisfactory, with acute - unfavorable, especially in cases of development of a true scleroderma kidney.

Prevention consists in the elimination of external factors that provoke the development of systemic S. in persons "threatened" in relation to the development of the disease: cooling, vibration, exposure to chemical. substances, including silicon dust, allergenic influences, etc. The group "threatened" in relation to the development of systemic S. includes persons with a tendency to vasospastic reactions, with limited S. or recurrent polyarthralgia, relatives of patients with collagen diseases. Secondary prevention, designed to prevent exacerbations and progression of the disease, includes early diagnosis and timely adequate treatment of exacerbations of the disease in a hospital and outpatient settings, medical examination, carrying out rehabilitation measures, including the stage of resort treatment (mainly in chronic course). Proper employment of patients and the exclusion of the above factors that provoke the development of S. and its exacerbations are necessary. In the acute and subacute course of systemic S., patients, as a rule, are unable to work and must be transferred to disability, and in chronic cases, they are limitedly able to work.

Correct timely treatment and employment can improve the prognosis and preserve the working capacity of some patients with systemic C.

Features of systemic scleroderma in children

Systemic S. is rare in children. The disease usually begins between the ages of 5 and 10 years. Girls get sick 5 times more often. Provoking factors, in addition to those that cause disease in adults, are acute childhood infections, the introduction of vaccines and sera.

Skin changes typical for systemic S. as the first symptom of the disease are noted only in half of the patients. The successive change of stages of skin changes can not always be traced. In the same patient, a combination of dense skin edema with induration, induration with atrophy, or the presence of all three stages at the same time is possible. As in adults, in addition to the typical changes in the skin in the form of limited S., trophic disorders, and pigmentation disorders occur in children. Telangiectasias in children are rare. Vascular syndrome in the form of vasospastic crises (Raynaud's syndrome) as the first sign of the disease occurs approximately 3 times less frequently than in adults, but in the future the frequency vascular manifestations is growing. With a progressive process, the formation of trophic ulcers is possible (in 20% of patients). The articular syndrome is similar to that in adults. Already in the early stages of the disease, severe joint-muscular contractures often appear. Clinic muscle lesions, as well as the frequency of true myositis are the same as in adults. Osteolysis and calcification are 2 times less common than in adults, however, unlike adults, they may appear in children. earlier periods - in the 2-3rd year of the disease.

Damage to the internal organs in children, as a rule, is not very pronounced, progressing slowly. However, with the help of instrumental research methods, a high frequency and prevalence of visceral pathology is revealed. Most often observed changes in the heart. The myocardium is affected in all patients, the pericardium - somewhat less often, but 4 times more often than in adults, the endocardium - in V3 sick children. Damage to the lungs in frequency is in second place (approximately 70% of patients). An early sign lung lesions are functional disorders, in particular a regional decrease in the vital capacity of the lungs, detected using radiopneumography (see Pulmonary ventilation). Damage of a gullet in the form of disturbance of motility is diagnosed rentgenol. method in half of the children. Kidney damage is clinically detected in approximately 40% of patients and is more often characterized by mild changes in the urine (transient albuminuria, slight changes in sediment).

In children, the same variants of the course of systemic S. are observed, as in adults. Subacute and chronic course occurs with about the same frequency. Perhaps an acute course with a fatal outcome in the first three years of the disease. Chron. long-term forms of the disease isolated syndrome Raynaud, rare in children.

Complications are most often associated with the addition of a secondary infection - infection of ulcers, pyelonephritis (see), less often pneumonia (see), sepsis (see). As rare complication meet patol. fractures of the bones of the lower extremities, not associated with hormonal treatment.

The differential diagnosis should be carried out with scleredema (see) and phenylketonuria (see). The first is characterized by the phasing of the appearance of induration, the absence of fibrosis and atrophy of soft tissues, articular and vasospastic manifestations; patol. processes in the internal organs proceed benignly and subside as the skin induration disappears. With phenylketonuria, accompanied by thickening of the skin and muscles, there is a lag in mental and physical development, as well as an increase in the content of phenylalanine in the blood and its detection in the urine.

The principles of systemic S.'s therapy in children are the same as in adults.

The prognosis is most serious when the disease develops at an early age and depends on the speed of development and severity of muscle and joint damage, the depth and prevalence of vascular disorders, and the addition of a secondary infection. With the progression of visceral lesions, the prognosis is aggravated.

Prevention is similar to S.'s prevention at adults; it is necessary to conduct a thorough and adequate treatment of childhood infections, follow the rules for routine vaccinations.

Limited scleroderma

Limited scleroderma (sclerodermia circumscripta; syn.: focal S., localized S., keloid-like S., Addison's keloid). As well as at system S., patol. the process in the skin with limited S. goes through three stages: dense edema, induration, and atrophy. In some cases, in addition to the skin, the underlying muscles are affected with the development of limited myosclerosis. The nature skin lesions There are several variants of limited S.

Blyashechny S. (sclerodermia placata) is observed most often. It usually develops gradually, for no apparent reason, has a long course with periods of exacerbations and remissions. It is characterized by the formation on the lateral surface of the trunk, back, lower back or proximal parts of the limbs of one or more spots of various sizes, oval or irregularly shaped pinkish with various shades(purple, lilac). The spots gradually increase in size, and after a few weeks sclerotic changes develop in their central part, as a result of which a smooth, dense, like cardboard, shiny, ivory-colored plaque is formed, somewhat protruding above the level of the surrounding skin (tsvetn. Fig. 12). On the periphery of the plaque there is purple ring-shaped area, gradually turning into normal skin. This zone indicates the progression of the process. The formed plaque slowly increases in size, it can form areas of pigmentation and telangiectasia. In rare cases, there are multiple lesions (generalized or disseminated plaque S.). After a few years, the lesion imperceptibly resolves and undergoes atrophy, leaving a slightly pigmented retraction of the skin. Atrophied skin, resembling crumpled tissue paper, easily gathers into a fold. It is extremely rare that blisters with hemorrhagic contents appear in the area of ​​plaques (bullous-hemorrhagic plaque S.) or areas of superficial ulceration. Varieties of plaque S. are superficial limited S., with a cut on the skin, small dark-colored spots with a lilac tint develop without signs of compaction and infiltration, as well as knotted shape(tuberous, keloid-like) in the form of protruding nodes. In the area of ​​scleroderma plaques, hair falls out, the secretion of the sebaceous and sweat glands decreases.

Tape-like, or strip-shaped, S. (sclerodermia striata) differs in a linear form of the centers of defeat of skin and quite often involvement in patol. the process of underlying tissues (subcutaneous tissue, muscles). S.'s centers are located along one of extremities, sometimes on the course of nerves (sclerodermia zoniformis) or circularly, encircling a trunk, all extremity or a finger (sclerodermia anularis). When tendons, ligaments and muscles are affected, retractions and contractures are formed, which limit the range of motion in the joints. Localization of ribbon-like S. is possible on the face (in the region of the bridge of the nose and forehead) and the scalp (reminiscent of a scar from a saber strike). Nek-ry researchers refer to limited S. progressive atrophy of the face - Parry's disease - Romberg (see Hemiatrophy).

Teardrop scleroderma is characterized by the appearance of small, several millimeters in diameter, whitish spots, round or polygonal in shape, sometimes surrounded by a narrow pink border. The spots are often located in groups, can merge, forming large foci of scalloped outlines. After a few years, skin atrophy develops on the spots (see). Rashes are localized on the neck, upper chest or back, less often on the limbs. Although most researchers consider white spot disease to be a type of limited S., there is an opinion about its possible connection with lichen planus (see Lichen red flat).

The diagnosis is made on the basis of a wedge, data.

For the treatment of limited S., lidase is widely used, to-ruyu is administered subcutaneously or intramuscularly at 64 IU every other day, for a course of 12-15 injections. Repeated courses are carried out after a 2-3-month break. Also effective are lidase electrophoresis and compresses with Ronidase on the skin lesion. Apply intradermal or subcutaneous injection of hydrocortisone suspension into the lesions, 1-2 ml with 0.25% solution of novocaine, 2 times a week, 6-8 injections; phonophoresis of hydrocortisone suspension; lubrication of the foci with dimethyl sulfoxide in its pure form or chipping of the foci with 1-5% solution of dimethyl sulfoxide. Improvement can also be achieved by repeated novocaine blockade of the nodes of the sympathetic trunk, by taking ganglioblocking substances (pahikarpin). General strengthening treatment is carried out (vitamins of group B, A, PP, C). Thermal procedures (baths, mud therapy, paraffin therapy), light massage, sea and hydrogen sulfide baths (Sochi-Matsesta, Pyatigorsk) are effective at the stage of skin compaction, to lay down. physical training.

The prognosis for limited S. is favorable, reliable cases of its transition to systemic S. are not described.

Patients with limited S. are subject to dispensary registration and observation. At the same time carry out sanitation of the centers hron. infections, treatment of concomitant diseases. Sick limited scleroderma work in cold rooms is contraindicated, as well as work associated with skin trauma, vibration.

Bibliography: Nazarova V. D. and Balabanova R. M. Features of microcirculatory disorders in the cat with systemic scleroderma, Ter. arch., vol. 51, no. 6, p. 77, 1979; Vysotsky G. Ya. Systemic and focal scleroderma, L., 1971, bibliogr.; Gusev H. M. and Guseva N. G. X-ray cinematography in the diagnosis of heart damage in systemic scleroderma, Ter. arch., vol. 48, no. 5, p. 125, 1976; Gusev II. G. Systemic scleroderma, M., 1975, bibliogr.; Davidovsky L. Ya. Collagen diseases in children, Alma-Ata, 1979, bibliogr.; D about in-zhansky S. I. Scleroderma, Saratov, 1979, bibliogr.; To at x and E. and Yablonska D. Damage of lungs at a scleroderma, Klin, medical, t. 32, 1978; Multivolume Guide to Pediatrics, ed. Yu. F. Dombrovskaya, vol. 7, p. 286, M., 1964; Rakhmanov V.A. To the mechanism of action of lidase in the treatment of patients with scleroderma, Vestn. dermis, and ven., No. 6, p. 3.1959; StrukovA. I., K about p e in and T. N. and K and to t at r-s to-and y L. V. Immunopathology of collagen diseases, Klin, medical, t. 20, 1974, bibliogr.; Tareev E. M. Collagenoses, p. 162, M., 1965; A p-s e 1 1 V. M., N a s s e h G. A. a. B y-waters E. G. Scleroderma in childhood, Ann. rheum. Dis., v. 35, p. 189, 1976; Barnett A. J. Immunology in scleroderma, Med. J. Aust., v. 2, p. 138, 1978; D a b i c h L., S u 1 1 i v a n D. B. a. C a & s i d y J. T. Scleroderma in the child, J. Pediat., v. 85, p. 770, 1974; G er t 1 er W. Systematische Dermatologie, Bd 1, S. 354, Lpz., 1970; I s h i k a-w a H. a. o. An approach to experimental scl-eroderma, using urinary glycosamin-glycans from patients with systemic scleroderma, Acta derm.-venereol. (Stockh.), v. 55, p. 97, 1975 Twardzina i stany rzekomotwardzinowe, Warszawa, 1963; To lug H., Barth e 1m e s H. u. T h o r m a n n T. Immunmorphologische und ultrastruktu-relle Befunde an der Muskulatur bei progres-siver Sklerodermie, Z. ges. inn. Med., Bd 32, S. 357, 1977; Lapenas D., Rodnan G. P. a. Cavallo T. Immunopathology of the renal vascular lesion of progressive systemic sclerosis (scleroderma), Amer. J. Path., v. 91, p. 243, 1978; Masi A. T. a. D'Ange-1 on W. A. ​​Epidemiology of fatal systemic sclerosis (diffuse scleroderma), Ann. intern. Med., v. 66, p. 870, 1967; Meds-g e r T. A. a. M a s i - A. T. Epidemiology of systemic sclerosis (scleroderma), ibid., v. 74, p. 714, 1971; Velayos E. E. a. Cohen B. S. Progressive systemic sclerosis, Amer. J. Dis. Child., v. 123, p. 57, 1972.

H. G. Guseva; T. H. Kopieva (path. An.), V. V. Mikheev, N. A. Ilyina (neur.), H. N. Uvarova (features of systemic scleroderma in children), Yu. S. Khomyakov (rents.) , O. K. Shaposhnikov (limited scleroderma)

Thank you

Oh those kids! How much they have to experience even before they enter adulthood. Even such a terrible disease as scleroderma and that did not bypass them. Dear parents, a disease called scleroderma is really dangerous .. site) will try to tell you as much information as possible regarding the course, manifestations, and methods of treating scleroderma in children. You need to know everything about all the diseases that your baby may have, so stay tuned.

To begin with, we note that scleroderma is a very rare disease, and most often they suffer from the fair sex, whose age varies between thirty and fifty years. In fact, scleroderma has been known since ancient times. Despite this, this ailment has not yet been properly studied. Scleroderma surprises scientists with its manifestation, cause and course more and more. Moreover, today there are not so many specialists who are studying this disease. But let's get back to the main point.

So, scleroderma in children.

What is scleroderma?

Scleroderma is an inflammatory disease of the connective tissue that is chronic and in most cases affects only the human skin. Despite this, many vital important organs. In medicine, scleroderma in children is called childhood scleroderma or juvenile scleroderma.

How often does this disease occur in children?

In fact, scleroderma in childhood is an extremely rare occurrence. Every year, from two to twelve children per one million suffer from this disease. Many of you now may think that this does not threaten their children. You may be right, but there is still a risk. As for childhood scleroderma, it can take two forms. This plaque And linear scleroderma. The first form of this disease occurs mainly in girls, but the second - in boys. However, in all cases, this disease develops subacutely and affects both the skin and subcutaneous tissue.

Symptoms

As for the symptoms of scleroderma in children, this disease begins to manifest itself through oval or strip-shaped spots, which can be different in size. At the very beginning, these areas are slightly reddish and puffiness is noted on them. Then they become denser and acquire a shade of ivory. As a result, their atrophy occurs. Prolonged absence treatment leads to involvement in the existing inflammatory process of other parts of the skin. Most often, atherosclerosis also affects the internal organs, but not in the case of children. If we talk about childhood scleroderma, then it almost never affects the internal organs.

Diagnostics

Diagnosing juvenile scleroderma is quite difficult. This is explained by the fact that the symptoms of this disease are very similar to the symptoms of other ailments. However, today there are already some methods for diagnosing this disease, with the help of which it can still be detected at a very early stage of development. As for the treatment of scleroderma in children, by analyzing the basic therapy, scientists were able to reveal that gold salts do an excellent job with scleroderma in children. That is why for the treatment of this disease is most often used auranofin as the main drug.

Dear parents, we once again remind you that changes in the general condition of the child must be constantly monitored. Also do not forget that the child should only lead a healthy lifestyle.

Before use, you should consult with a specialist.

Reviews

I've been sick since 2 years old. Now 37. If you list what you tried and what doctors you went to. Incl. Institute. Sechenov in Moscow. Zero results. I have linear scleroderma. I have reconciled and I live further. I have not met a single doctor who would say sensible. And how much lidase I was instructed in childhood and electrophoresis. Scary to imagine

My daughter at 6 months had a spot at 1.5, she was diagnosed with systemic scleroderma, we are already being treated for 1.5
Please tell me clinics or methods of treatment

My daughter fell ill 4 months ago, focal plaque scleroderma, observed in the department of rheumatology in Samara, were seen by dermatologists, but their treatment with penicillin and lidase, the department recommended medakassol, began to inject methodoject to stop the process, smear with egallochite, solcoseryl and karepoin on queues, write if anyone has something new in treatment, maybe someone in Moscow will recommend where to turn and to whom ....

Good afternoon, my daughter has linear scleroderma, we have been treated since 2010, there are no results. We are already 9 years old. I want to hear advice and am waiting for an answer, help

I am 13 years old, I have been suffering from focal scleroderma for 8 years now, help me, tell me if clay cures this sore

My daughter is 4.5 years old and diagnosed with focal scleroderma, common in Kyrgyzstan, children are not treated, and in Moscow, how much will the treatment cost

My son is 5 years old in June 2013, on his upper legs there were spots with a nail, one on the outer and inner sides of the total 4. I went to the skin clinic. They determined that it was not lichen. The doctor could not say anything more. Now August and the spots have increased to 2 ruble coins. maybe it's scleroderma

Please tell me where you can cure scleroderma in St. Petersburg? Are there branches of the Sechenov Clinic for Children's Diseases in St. Petersburg?

My daughter is now 14 years old and has been suffering from scleroderma for 4 years. We live in Kazakhstan before, under the USSR, such patients from all over the Union were treated in Moscow, but now Russia is like a foreign country for us and treatment is paid or according to a quota. Our authorities do not draw up a quota and we are treated at home, but there is no improvement. I ask you for help, can someone help or tell me what to do and where to turn. The child has a whole life ahead of him, and it seems to me that if it is still at the initial stage, there is at least something else that can be done. We are being treated with methojet, drinking cuprenil, all this with loading doses, smearing madecasol, nothing helps. Help us how we can get from Kazakhstan to the Sechenov Institute for treatment in Moscow and how much it will cost.

I have been ill with scleroderma since childhood, I was treated in Almaty at the Institute of Pediatrics and now in Astana at the national center I drink cuprenil

I had never heard of this disease before, but about a year ago I began to notice that some spots began to appear on my daughter's leg. She was then 3 years old. The spots appeared and then disappeared. After some time, they went to the clinic. Several diagnoses were made, but not one was confirmed. They did a skin biopsy, after which they made this terrible diagnosis. As a treatment, we take Kuprenil and a combination of ointments, different every month. A few months later, back to the hospital for a checkup. doctors reassured - they said that not everything is so scary, mild form but we are still worried. Heard about the Institute. Sechenov, but we can’t get there, since we live in Kazakhstan. Maybe someone could cure a child in Kazakhstan, write where and how, I will be very grateful.

My son is 13 years old, fell ill with scleroderma at the age of 11. For 1.5 years they drove from one hospital to another, they injected penicillin and lidase, but the spots increased every day, and then we somehow miraculously ended up in Moscow at the Sechenov Institute. Thank God the spots have stopped.

My son is 17 years old. The diagnosis is focal scleroderma. Hyperpigmentation all over the body. Organs are not involved. They prescribed treatment with penicillamine and a bunch of ointments. At the same time, the leading rheumatologist said that he sees this for the first time. Analyzes are all normal. What and where. If they expose a photo of scleroderma and focus 2 or 3. Then you can do some kind of compresses. And my son has it all over his body. Who else can you contact?

I was diagnosed with this at the age of 12. They prescribed very expensive and rare medicines. My mother was unable to pay for the treatment due to her difficult financial situation. And we refused further treatment. The doctors said that life remained without treatment for 10 years no more. Now I am 40. I have two magnificent daughters. I don’t know how life will turn out further, but I am full of optimism. In fact, the disease itself (except for a visual cosmetic skin defect) does not bring any inconvenience. We will live!

My son got sick with scleroderma after a flu shot, in 2002. They were treated in Moscow, at the Sechenov Clinic for Children's Diseases. I was lucky, having arrived in Moscow with a sick child after two weeks of visiting various clinics, institutions, committees, and the Ministry of Health was told the address of Sechenov's clinic. All the years of my son's illness I studied this issue, I can say with confidence that this is the only place where your children can be helped. They helped us and hundreds of children whom I saw entering our department for six years. With this disease, in no case should you take a biopsy, injure the affected areas and inject injections into them, of course, you should not sunbathe. Treatment with penicillin, lidase is the last century, many side effects, there are no significant improvements (with me in 2005, a girl was admitted to the department after four years of treatment with penicillin at TsNIKVI, Moscow, there are no improvements, her face is deformed, her lips do not close on the right side (you can’t look at this without tears). Previously, they also treated in the clinic penicillin, etc. but the results were poor, many children died (especially from lupus), doctors looked for new methods, and eventually abandoned the old protocols.We were treated with prednisolone, cuprenil, vascular drugs, doses are selected very carefully, in some regions they are trying to prescribe a similar treatment, but probably there is not enough experience, they brought “healed” children, it seems that the drugs are correct, but horse doses, it’s also impossible. , but these are trifles - the main thing is the gifted opportunity for my child to live. Children there from all over Russia and the CIS also call from abroad for advice. The mistake of parents is that many do not the disease is terrible, the children were brought to the clinic half “ossified”, the danger is that even if you then have a remission, the affected area still stops growing, the result is mutilated faces, arms, legs of children. Now we do not take drugs, we control the tests in dynamics. I wish all mothers to help their children, do not be afraid, the main thing is not to give up.

By the way, it is very strange, but no one mentioned such a medicine as Piascledin. He helped me a lot too. When the disease began, he was not in Russia, they were brought from France through Germany. Piascledin is now commercially available. It is better to address in Institute of Rheumatology. Antibiotics and hormonal preparations were not recommended to me at all. And a cream - though what. Maybe an allergy develops. Another ban on solariums and outdoor activities under the sun. In summer, do not go outside without a cream with protection less than 30! And so every day I apply a cream of the Pure Line series for dry skin (wheat germ) with vitamins A and B to the affected area (medial part of the thigh).