Systemic scleroderma in children. Pathological anatomy of systemic scleroderma

For quotation: Grebenyuk V.N. LIMITED SCLERODERMA IN CHILDREN // Breast cancer. 1998. No. 6. S. 2

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

The article describes limited scleroderma in children: etiology, pathogenesis, classification of the disease, clinical forms and manifestations. Practical recommendations on diagnostic and drug 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., doctor of medicine. Sciences, Head of the Department of Pediatric Dermatology of the Central Scientific Research Institute of Medical Sciences of the 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 (LS) in children is a serious modern medical and social problem. Unlike systemic scleroderma (SSc), in which various organs are involved in the pathological process, OS is “limited” to affecting only the skin. At the same time, the disease often becomes systemic in nature, i.e. becomes 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 SSD are not identical, and distinguish them by pathogenesis, clinical picture and course. And in this case, SSD is classified as diffuse connective tissue diseases (DCT), but OS is not.
As is known, DTDs include SSc, systemic lupus erythematosus (SLE), dermatomyositis, periarteritis nodosa and rheumatoid arthritis - formidable diseases that require a specific strategy and tactics for patient management, an intensive treatment and prophylactic complex. SSc is the second most common disease after SLE from the DTD group (from 32 to 45 cases per 100 thousand population). It should be emphasized once again that the possibility of transitioning OS to SSD cannot be ignored.
In childhood, OS dominates. It occurs in children more than 10 times more often than SLE. Girls get sick more than 3 times more often than boys.
The disease can occur at any age, even in newborns, usually starting gradually, without any subjective sensations or disturbances in the general condition. Due to the tendency of the growing organism to widespread pathology, to pronounced exudative and vascular reactions in children, this disease often shows a tendency towards a progressive course, extensive damage, although in the early stages it can manifest itself in single foci. In the last decade, the incidence of this pathology in children has increased. OS is characterized mainly by 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 expanded 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 from a historical perspective, but the role of Koch's bacillus, pallidum spirochete, and pyococci as a possible root cause of scleroderma has not been confirmed. The role of Borrelia burgdorferi in the development of this disease is also not convincing. Although structures resulting from the indirect influence of viral infection were found in the cells of various tissues of patients with scleroderma, the virus was not isolated.
The role of genetic factors cannot be ruled out.
Multifactorial inheritance is assumed.
The pathogenesis of scleroderma is associated mainly with hypotheses of metabolic, vascular and immune disorders.
The occurrence of scleroderma is also influenced by disorders of the autonomic nervous system and neuroendocrine disorders.
Disorders of connective tissue metabolism are manifested by hyperproduction of collagen by fibroblasts, 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 primarily on lesions of the walls of small arteries, arterioles and capillaries, proliferation and destruction of the endothelium, intimal hyperplasia, and sclerosis.

Pasini-Pierini atrophoderma 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. Increased levels of CD4+ are detected in the blood and tissues -lymphocytes and high levels of interleukin-2 (IL-2) and IL-2 receptors. A correlation has been established between the activity of T-helper cells and the activity of the scleroderma process.
R.V. Petrov considers scleroderma as an autoimmune disease in which the disturbances are based on the interaction of autoantigens with lymphoid cells. At the same time, T-helpers, activated by exo- 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 autoimmune reactions are realized due to genetic predisposition. The emerging vicious circle of mutual influence of lymphoid and collagen-synthesizing cells leads to the progression of the fibrotic process.
In scleroderma, a variety of other autoimmune disorders are observed: various autoantibodies, decreased levels of T-lymphocytes with unchanged or increased content B-lymphocytes, decreased function of T-suppressors with unchanged or increased function of T-helpers, decreased functional activity of natural killer cells.
In 20-40% of cases with plaque scleroderma, antinuclear antibodies are detected, and 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 erased (abortive) manifestations of the disease, varying degrees of involvement of the skin and underlying tissues in the pathological process make its diagnosis difficult.
A practically acceptable classification of OS is based on the clinical principle.
I. Plaque form with its variants (varieties):
1) indurative-atrophic (Wilson);
2) superficial “lilac” (Guzhero);
3) keloid-like;
4) knotty, deep;
5) bullous;
6) generalized.
II. Linear shape (strip):
1) saber-shaped;
2) ribbon-like;
3) zosteriform.
III. Lichen sclerosus (white spot disease).
IV. Idiopathic atrophoderma Pasini - Pierini.

Clinic

In the dynamics of development, scleroderma lesions 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 its appearance on various parts of the skin (in some cases, on the mucous membranes). Plaques are round-oval in shape, less often with irregular outlines. Their size ranges 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 usually forms in the form of a disk of compacted or dense skin, waxy-grayish or ivory in color, with a smooth shiny surface. Along the periphery of the lesion 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 growth of the plaque and the appearance of new lesions usually occur slowly and are not accompanied by subjective sensations. Pigmentation and telangiectasias may occur in the lesions and adjacent areas of the skin.
On the affected skin, sweating is reduced or absent, function is impaired sebaceous glands and hair growth.
An extremely rare type of OS is the bullous, erosive-ulcerative form, which usually occurs against the background of sclerosis of the skin in the periarticular areas. It can appear at any site of scleroderma. Consecutive formation of vesicular-bullous and erosive and ulcerative lesions associated with dystrophic changes in sclerotic skin. Trauma and secondary infection may play a causative role.

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

With superficial lilac plaque OS (Gugerot), a barely noticeable superficial compaction is observed, the skin in the lesion is pinkish-lilac with a more intense color at the border of the lesion.
In the strip-like form of OS, the lesions are linear, in the form of stripes, often localized along one limb, often along the neurovascular bundle. They can also be located circularly on the torso or limbs. On the face and scalp, localization of lesions that are not uncommon in this form is noted, often cicatricial-saber-shaped (resembling a scar after a saber strike). A dense strand of sclerotic skin can have different lengths and widths, a brownish color, and a shiny surface.
There is no hair growth where it is located on the scalp. Vertically, the lesion can extend from the scalp, crossing the forehead, bridge of the nose, lips, and chin. Often the mucous membrane of the oral cavity is involved in the process.
When the process resolves, the surface of the lesion is smoothed out, and a recess is formed, caused by atrophy of the skin, muscles and bone tissue.
Lichen sclerosus (LS) of Tsumbusha (synonyms: white spot disease, guttate scleroderma) is considered as a disease that is clinically close to limited superficial scleroderma, but is not completely identical to it.
Clinical manifestations: whitish, almost milky papules with a diameter of 1 - 3 mm, usually round in shape, located on unchanged skin. At the beginning of their appearance, they are reddish in color, sometimes surrounded by a barely noticeable lilac border. There may be a recess in the center of the elements. When grouped papules merge, lesions with scalloped outlines are formed. These lesions are most often localized on the neck, torso, genitals, as well as on other areas of the skin and mucous membranes. The lesions tend to resolve spontaneously, leaving atrophic hypopigmented or amelanotic macules. Their surface is shiny and wrinkled. Usually the rash is not accompanied by subjective sensations.
The clinical variety of SAL is the plaque form with lesions reaching several centimeters in size, with round or irregular outlines. The skin in such lesions is thinned and easily gathers into folds like crumpled tissue paper. With the pemphigoid form, bubbles the size of a pea are formed; transparent contents are visible through their thin cover. When bubbles rupture, 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 recognition of the disease by months and sometimes years carries the risk of developing severe forms that can lead to disability. A long-term progressive course can also result in functional insufficiency of the skin and musculoskeletal system.
Under the influence of treatment, rarely spontaneously, the lesions resolve (induration, redness, shine disappear) resulting in skin atrophy, often leaving vitiligo or pigment spots.
Externally, the skin resembles parchment. There are no vellus hairs in residual lesions. There is a thinning of not only the skin, but also the underlying tissues. After resolution of the scleroderma process in superficial plaque lesions, skin changes are much less pronounced.

Survey

All children suffering from OS, regardless of the clinical form of the disease and intensity of the lesion, are subject to instrumental examination for the purpose of early diagnosis of visceral pathology, identifying signs of systemic disease. And given the possibility of a latent course of SSD, especially in the early stages of its occurrence, assessment of the condition 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 SSD in children or even the absence of its clinical signs, which are usually nonspecific, the doctor should be wary of possible development systemic process not only with multifocal and widespread manifestations, but also with single limited plaques.
Over many years of observation N.N. Uvarova 173 children with SSD, clinically and instrumentally examined, in 63% of cases the disease began with skin lesions (cutaneous 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, revealed visceral changes in 51 (25.1%) of 203 children with OS, i.e. signs of a systemic process. Among them are lesions of the heart (scleroderma heart - impaired atrioventricular and intraventricular conduction, sinus tachycardia, arrhythmia, shift of the S - T interval), 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, rhythm disturbances, evacuation), kidneys (decreased effective renal plasma flow, proteinuria).
M.N. Nikitina, when examining 259 children with OS, found similar visceral disorders. Clinically, it is impossible to distinguish between OS and skin syndrome in SSc.
Children suffering from OS, during multi-course treatment and observation, should be under constant supervision of a pediatrician, dermatologist and consult other specialists as indicated.

Treatment

Treatment of children with OS remains a difficult task. It must be comprehensive and stage-by-course. In this case, a differentiated approach is important, which takes into account the anamnesis and the results of clinical and laboratory examination, which makes it possible to prescribe adequate treatment measures. They, in particular, include the sanitation of the body, the correction of functional disorders of the nervous, endocrine, and immune systems, as well as pathogenetic drugs.
In the advanced stage, inpatient treatment with penicillin, lidase for dermatosclerosis, dimexide (DMSO), and vitamins is preferable. When the pathological process is stabilized with a tendency towards resolution of induration and sclerosis, enzyme preparations, immunomodulators, antispasmodics, biostimulants, and pyrogenic drugs are indicated. Physiotherapy and sanatorium-resort treatment consolidate and enhance the therapeutic effect, and also have a rehabilitation effect.
Penicillin is recommended to be administered in the progressive stage of the disease at 1 million units/day in 2 - 3 injections, for a course of up to 15 million units in 2 - 3 courses with an interval between them of 1.5 - 2 months. Semi-synthetic penicillins (ampicillin, oxacillin) are used less frequently.
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 in the presence of focal infection is also allowed.
From enzyme preparations Lidase and Ronidase containing hyaluronidase are widely used. The therapeutic effect is associated with the properties of the drugs to improve microcirculation in tissues and promote the resolution of sclerosis in lesions. There are 15 - 20 injections per course. Lidase is administered intramuscularly, 1 ml with 32 - 64 UE in 1 ml of 0.5% novocaine solution. The therapeutic effect increases when parenteral administration of the drug is combined with electrophoretic administration. The courses are repeated after 1.5 - 2 months in the presence of dermatosclerosis.
Ronidase is used externally, applying its powder (0.5 - 1.0 g) to a napkin moistened with saline solution. Apply a napkin to the lesion, fixing it with a bandage for half a day. The course of applications continues for 2 - 3 weeks.
Electrophoresis with a 0.5% solution of zinc sulfate has a beneficial effect on the resolution of scleroderma lesions. The procedures are carried out every other day for 7 - 20 minutes, for a course of 10 - 12 sessions.
Biostimulants (splenin, vitreous, aloe), activating metabolic processes in connective tissue, promote tissue regeneration and increase the body's reactivity. Splenin is administered 1 - 2 ml intramuscularly, vitreous - 1 - 2 ml subcutaneously, aloe - 1 - 2 ml subcutaneously, for a course of 15 - 20 injections.
Pyrogenic drugs increase the body's resistance and stimulate the T-cell component of the immune system. Of these drugs, pyrogenal is most often used. It is usually used after 2 days on the third intramuscular injection, starting from 10 - 15 MTD. Depending on the temperature response, the dose is increased by 5 - 10 MTD. The course consists of 10 - 15 injections.
Immunomodulators, in particular taktivin and timoptin, have an immunocorrective effect. Under their influence, a number of immune parameters and collagen formation are normalized. Taktivin is administered daily under the skin, 1 ml of a 0.01% solution for 1 - 2 weeks, 2 - 3 times a year. Timoptin is prescribed subcutaneously every 4th day for 3 weeks (at the rate of 2 mcg per 1 kg of body weight).
Angioprotectors, improving peripheral blood circulation and trophic processes in lesions, help resolve sclerotic skin changes. From this group use: pentoxifylline (0.05 - 0.1 g 2 - 3 times a day), xanthinol nicotinate (1/2 - 1 tablet 2 times a day), nikoshpan (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. Compress bandages or applications are applied to dermatosclerotic plaques until they noticeably resolve. The drug, penetrating deep into the tissue, has a pronounced anti-inflammatory effect and inhibits collagen hyperproduction.
Solcoseryl (cattle blood extract freed from protein), administered intramuscularly at 2 ml per day (20 - 25 injections per course), improves microcirculation and activates trophic processes in the lesion.
Externally, 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 remedies 2 times a day, rubbing into the affected areas. You can alternate these drugs every week; the duration of local applications is 1 - 1.5 months. Madecassol is also effective in treating children with scleroderma. This herbal preparation regulates the quantitative and qualitative formation of connective tissue and inhibits excessive collagen formation.
Adequate external treatment in combination with vasodilators is of great importance in the treatment of vulvar SAL and makes it possible to avoid multi-course treatment with penicillin and lidase.
For 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 subject to early diagnosis of scleroderma and timely implementation of the necessary comprehensive course of treatment.

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People of any age are susceptible. Thus, in the “Red Storm” sanatorium 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 is common in childhood, and recently cases of this disease have become more frequent.

Conventionally, there are two clinical forms of this disease: 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 represent 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, many questions remain here as well. 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 cases of detection of waste products of viruses in the tissues and blood of patients with scleroderma. Thus, during an electron microscopic examination of muscle tissue biopsied from patients, J. Kudejko discovered cellular inclusions resembling viruses.

It is quite 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 occurrence of this severe dermatosis in patients with functional disorders of the thyroid, reproductive, 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 cases of the disease after vaccinations, administration of therapeutic serums, 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 and formation of deep autoimmune and dysproteinemic processes that are localized in the connective tissue system of the skin, blood vessels, and internal organs.

Limited forms of scleroderma include spotted, stripe, and plaque forms. Systemic scleroderma may also manifest itself in various ways.

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 lesions are unclear, and the sizes can vary quite widely - from a coin to the palm of an adult. It is characterized by a 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 at the edges. With the loss of inflammatory coloring, the lesion becomes dense in consistency, then the density increases. The surface of the affected skin becomes shiny, while a smoothed skin pattern, lack of hair, dryness due to lack of sebum and sweating, and decreased sensitivity are noticed. The skin is very difficult to gather into a 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. To summarize, we can say that in the clinical course of the plaque form of scleroderma there are three stages: inflammatory edema; the appearance of compaction; atrophy. As a rule, foci of plaque scleroderma are located on the neck, torso, lower and upper extremities, and sometimes on the face.

As for the second type of focal scleroderma, strip-like (ribbon-shaped, linear), it is most often localized in the facial area, 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 hardening and atrophy. In addition to the face, foci of scleroderma can be localized along the limbs and along the body along the Zakharyin-Ged reflexogenic zones and 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, deep damage to the underlying tissues is observed with the development of ulceration, as well as mutations.

White spot disease can be characterized by the formation of atrophic depigmented spots of varying 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. Places of localization include the shoulders, forearms, neck, and upper chest. Patients complain about slight itching in the area of ​​localization, a feeling of constriction.

Systemic or diffuse scleroderma

This disease usually occurs after stressful situations, injuries, cold with consequences (ARVI, flu, sore throat, herpes simplex, shingles). In the prodromal period, it is characterized by ailments, chills, pain in the joints, muscles, insomnia, headaches, fever, severe fatigue in combination with cold weather, pallor of the skin of the face, feet, and hands.

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

For systemic, diffuse scleroderma on initial stage The hands and face are affected, then the limbs and torso. As the disease progresses, a change in skin color from whitish-gray to yellowish is observed, thickening increases, and vellus hair falls out. The fingers and toes become thinner and sharper, joint movements become difficult, and the skin becomes fixed to the underlying tissues. Rigidity, tension, pale skin, and coldness are aggravated by numbness and paresthesia. The skin peels off in places, ulcers and cracks form, mutilations develop, and the fingers become like drumsticks or fingers of labor.

As a result of atrophic and sclerotic damage to the skin, facial muscles, and subcutaneous tissue, the nose becomes sharper, the cheeks recede, the mouth becomes folded, narrows, and the lips become thinner. The face becomes monochromatic (bronze), mask-like, and mimic. Very often, the mucous membranes of the tongue and mouth are also involved in the process. The edges of the lips may peel off, ulcers and cracks appear. Eating and swallowing are difficult. The atrophic process involves the aponeurosis on the scalp, ulcers, multiple telangiectasia are visible, and hair falls out.

There are three stages of the disease: swelling, 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, lesions of 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 due to 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. Not easy to carry out 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 for children begins with the administration of vitamins A, E, C, which help normalize the condition of connective tissue. Since inhibition of hyaluronidase activity is observed, it is optimal to use enzymes - ronidase, vitreous, lidase. For any form of the disease, antibiotics are prescribed, usually penicillin.

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 introduced using electrophoresis or ultrasound.

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

When inflammatory edema, characteristic of the initial stage of a disease such as scleroderma, appears, treatment is carried out with glucocorticoids - urbazoom, prednisolone, triamcinolone, dexamethasone - both orally and intradermally into the lesions in small doses. Low molecular weight dextrans, the introduction of which is justified pathogenetically, being hypertonic solutions, can cause an increase in plasma volume, reduce blood viscosity, and improve its flow. Thiol compounds are capable of breaking down collagen, so unithiol is often used in 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 functioning 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, ichthyodine, potassium iodide, ozokerite, paraffin application, therapeutic mud, radon and hydrogen sulfide baths. Therapeutic gymnastics, oxygen thalassotherapy, and massage are also useful.

Focal scleroderma ends with recovery. As for the systemic, diffuse form of scleroderma, it occurs over a long period of time, with periods of remissions that are followed 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 medical examination.

Egallohit cream, which contains green tea extract, is very effective. Main active substance This cream contains epigallocatechin-3-gallate. Egallohit has 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 skin’s resistance to negative environmental influences.

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

Popular

And focal (limited). The systemic course of this disease is considered to be the most dangerous, since in this case the pathological process involves internal organs, musculoskeletal system and fabrics. Focal scleroderma is characterized by less aggressive manifestations and is accompanied by the appearance of patches (stripes or spots) on the skin. white, which somewhat resemble scars, and has a favorable prognosis. This disease 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 of focal scleroderma. This information will be useful for you; you will be able to suspect the onset of the disease in time and ask your doctor questions about treatment options.

In recent years, this skin disease has become more common, and some experts note 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 development of the disease

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

• , leading to the production of antibodies against one’s 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 imbalances: abortion, pregnancy and lactation;
• excessive exposure of the skin to ultraviolet rays;
• severe stress or choleric temperament;
• traumatic brain injuries.

With focal scleroderma, there is excess synthesis of collagen, which is responsible for its elasticity. However, with the disease, due to its excess amount, the skin thickens and becomes rough.

Classification

There is no uniform classification system for focal scleroderma. Specialists more often use the system proposed by S. I. Dovzhansky, which most fully reflects all 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 sclerosis, guttate scleroderma, lichen white of Tsimbusha).
4. Idiopathic atrophoderma Pasini-Pierini.
5. Parry-Romberg facial hemiatrophy.

Symptoms

Plaque form

Among all clinical options of focal scleroderma, the most common is the plaque form. A small number of lesions 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 vary. After some time, a smooth and shiny yellow-white area of ​​compaction appears in its center. A lilac-pink border remains around this island. It can increase in size; these signs can be used to judge the activity of the scleroderma process.

Hair loss occurs on the resulting plaque, and hair loss stops. sebum and sweat, the skin pattern disappears. The area of ​​skin in this area cannot be folded with your fingertips. This appearance and signs of the plaque can persist for various periods of time, after which the lesion undergoes atrophy.

Linear (or strip-shaped)

This type of focal scleroderma is rarely observed in adults (it is usually detected in children). The difference between its clinical manifestations and the plaque form is only in the form skin changes– they look like white stripes 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 labia area.

Idiopathic atrophoderma Pasini-Pierini

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

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

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

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

Parry-Romberg facial hemiatrophy

This rare type of focal scleroderma manifests itself as atrophic lesions of only one half of the face. Such a focus can be located both on the right and on the left. Skin tissue and subcutaneous fat tissue undergo dystrophic changes, and muscle fibers and bones of the facial skeleton are involved in the pathological process less frequently or to a lesser extent.

Parry-Romberg facial hemiatrophy is more common in women, and the onset of the disease occurs 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 in a yellowish or bluish tint appear on the face. Gradually they become denser and over time undergo atrophic changes, which represent a serious cosmetic defect. The skin of the affected half of the face becomes wrinkled, thinned and hyperpigmented (focally or diffusely).

There is no hair on the affected half of the face, and the tissues under the skin are susceptible 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 among specialists around the disease

Among scientists, debate continues about the possible relationship between systemic and limited 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 sharply different from each other. However, this opinion has not yet found precise confirmation, and statistics indicate the fact that in 61% of cases focal scleroderma transforms into a systemic one.

According to various studies The transition of focal scleroderma to systemic scleroderma is facilitated by the following 4 factors:

• development of the disease before the age of 20 or after 50;
• plaque or linear form of focal scleroderma;
• increased antilymphocyte antibodies and large-dispersed immune circulating complexes;
• severity of disimmunoglobulinemia and lack of cellular immunity.

Diagnostics

Diagnosis of focal scleroderma is complicated by 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 syndrome;
• scleroderma-like form;

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

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

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

Treatment

Treatment of focal scleroderma should be complex and long-term (multi-course). If the disease is active, the number of courses should be at least 6, and the interval between them should be 30-60 days. When the progression of lesions has stabilized, the interval between sessions can be 4 months, and in case of residual manifestations of the disease, courses of therapy are repeated for preventive purposes once every six months or 4 months and include medications to improve microcirculation of the skin.

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

For lichen sclerosus, the treatment plan may include creams with vitamins F and E, Solcoseryl, Retinol palmiate, Actovegin.

If the patient has limited foci of scleroderma, then treatment may be limited to prescribing 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. The following are usually used as local medications:

• Troxevasin;
• Heparin ointment;
• Teonicol ointment;
• Heparoid;
• Butadione ointment;
• Dimexide;
• Trypsin;
• Chymotrypsin;
• Ronidase;
• Unithiol.

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

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

• magnetic therapy;
• ultraphonophoresis with Hydrocortisone and Cuprenil;
• 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 where scleroderma foci are located.

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

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

SCLERODERMA (scleroderma; Greek skleros hard, dense + derma skin; syn. scleroderma). The term “scleroderma” was first proposed by E. Gintrac in 1847. A distinction is made between systemic and limited sclerosis. Systemic sclerosis is characterized by generalized progressive sclerosis of the skin and internal organs, while limited sclerosis is characterized by predominantly focal skin lesions 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 peculiar vascular pathology type of iterating endarteriolitis with widespread vasospastic disorders.

The incidence, according to various researchers, ranges from 0.27-1.2 per 100 thousand population. Mortality, according to A. T. Masi et al., is 0.14-0.53 per 100 thousand. Mostly women are affected. 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 progressing), subacute and chronic systemic S. (the last two variants of the course are more common); typical S. with characteristic generalized skin lesions and its atypical forms with focal skin lesions; S. with predominant damage to internal organs; S., combined with others rheumatic diseases. Rodnan (G. P. Rodnan) and others identify the following forms of systemic S.: classic shape with diffuse skin lesions; CREST syndrome - a combination of calcification (see), Raynaud's syndrome (see below), damage to the esophagus, sclerodactyly and telangiectasia (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 damage to individual internal organs in S. and attempts to present it as a generalized process belong to Steven (J. L. Steven), W. Osler (1898), A. E. Yanishevsky and G. I. Markelov (1907). P. Klemperer's teaching about collagen diseases served as a powerful impetus for the study systemic manifestations of this disease. In 1945, R. N. Goetz proposed the term “progressive systemic sclerosis.” 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 summarizing monographic works, of which the works of E. M. Tareea, N. G. Guseva, G. Ya. Vysotsky, S. I. deserve the greatest attention. Dovzhansky, Jablonskaya (St. Jablon-ska), Rodnan (G. P. Rodnan), Leroy (E. C. LeRoy), etc.

Etiology

The etiology is not clear; the likelihood of a viral and hereditary origin of the disease is discussed. The possible participation of a viral infection in the etiology of systemic S. is indirectly evidenced by the detection of virus-like particles in 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, and activation of latent viral infection is being discussed.

The concept of hereditary transmission of systemic S. is based on Ch. arr. on the presence of family cases of the disease, frequent detection of 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 (silica dust, vinyl chloride, etc.), infections, neuroendocrine disorders, which precede the development of systemic S. in a number of patients, can be considered as provoking factors. They retain their importance in the theory of polygenic multifactorial inheritance of systemic S.

Pathogenesis

The pathogenesis is complex, it 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, microvasculature with the development of a kind of scleroderma angiopathy (obliterating eidarteriolitis, reduction of capillaries, widespread vasospastic reactions).

Systemic S. is characterized by hyperactivity of fibroblasts with excessive collagen and fibril formation when intercellular and interstitial interaction of connective tissue components is disrupted. 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 increased neofibrillogenesis. Scleroderma-like syndrome during treatment with bleomycin is also associated with excess production of collagen due to the stimulating effect of the drug on fibroblasts. When studying a monolayer culture of skin fibroblasts of patients with systemic S., a phenotypically stable overproduction of connective tissue components was discovered, Ch. arr. collagen, a violation of the functional properties of the fibroblast membrane was revealed (abnormal reaction to adrenaline, etc.). Changes in the functions of collagen-synthesizing cells with reduced or “defective” signal perception from the body’s regulatory systems can lead to an anomaly 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 disorders of 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), Sjögren's syndrome (see Sjögren's syndrome) etc. With it, the following are often detected: anti-nucleolar and antinuclear antibodies, including antibodies to the Scleroderma-70 antigen, anticentromere (to centromere chromatin) autoantibodies; antibodies and cellular immune responses to collagen; 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.

Disorders of microcirculation (see) and scleroderma angiopathy itself, 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 aggregation of platelets (see), activation of coagulation (see), fibrinolysis (see), release of inflammatory mediators (see), increased permeability vascular wall with subsequent plasmatic impregnation and fibrin deposition. Inflammatory mediators enhance endothelial destruction, microthrombosis and intravascular coagulation, maintaining damage. Subsequent repair of the vascular wall is accompanied by reduplication of basement membranes, intimal migration and proliferation of smooth muscle cells. The latter, being a type of fibroblasts, are capable of synthesizing predominantly type III collagen and are largely responsible (under these 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 occurs, and it is actively involved, along with connective tissue and immune system, in the development of patol characteristic of systemic scleroderma. process.

Pathological anatomy

Systemic S. is morphologically characterized by pronounced fibrosis of various organs and tissues. Tissue damage is based on vascular damage and excessive production of collagen (see).

The most characteristic changes are observed in the skin. Both with systemic and limited S., three stages of skin changes are distinguished: 1) the stage of dense edema; 2) induration stage; 3) stage of atrophy. In the stage of dense edema, signs of increased vascular permeability predominate (see). Hydropic degeneration of the cells of the basal layer of the epidermis (see Vacuolar degeneration), expansion of lymphatic fissures, slight disintegration of collagen bundles of the dermis due to edema, vasculitis (see), telangiectasia (see), inflammatory infiltration around the vessels, skin appendages and in the subcutaneous tissue are detected. 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 parts of the reticular (reticular) layer of the dermis. R. Fleischmajer et al. (1980), using immunofluorescence (see) and electron microscopy (see), it was established 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 clusters of thin fibrils (diameter 10-30 nm); there is an increase in the number of thin collagen fibers, immature bundles of which are similar to those that are detected in the skin during the embryonic period.

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

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

With active course of patol. process, vasculitis of arterioles and small arteries are proliferative in nature with circular growth of the inner membrane (Fig. 2). Electron microscopy reveals vacuolation and destruction of the endothelium, as well as a multilayer basement membrane, in the capillaries of affected tissues. According to N. Klug et al. (1977) and others, during an immunofluorescent study of material obtained from a biopsy of kidneys, muscles and skin, deposits of IgM and complement were found 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 joints, bones and muscles. When joints are affected, exudative-proliferative synovitis (see) is detected 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. In systemic articular cartilage, articular cartilage loses elasticity, becomes brittle, and quickly wears out; 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 articular capsule becomes dense, devoid of villi. With histol. Research has difficulty finding its organ-specific features: synoviocytes are absent over a large area, the synovial layer is covered with hyaline-like masses, the subsynovial layer is represented by vascular-poor fibrous connective tissue with extensive fields of hyalinosis. With systemic S., accompanied by myopathic syndrome, gistol. study skeletal muscles reveals a picture hron. myositis (see) with different caliber muscle fibers, hydropic dystrophy and myolysis of part of them, perivascular infiltrates of 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 vascular walls, emptying of the capillary bed, small-focal perivascular lymphoid-macrophage infiltration, isolated vasculitis, focal perifascicular or diffuse muscle atrophy fibers

In yellowish-kish. tract there is 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 the subacute course of systemic S., esophagitis (see), enteritis (see Enteritis, Enterocolitis), colitis (see) with proliferative, less often destructive-proliferative vasculitis of the arteries of the mesentery and the walls of the esophagus and intestines are found. In the liver, periductal, perivascular, and less often intralobular fibrosis, sclerosis and hyalinosis of the vessel walls are observed, fatty degeneration hepatocytes. Chron is less common. active hepatitis (see), primary biliary and large-nodular cirrhosis of the liver (see).

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

Heart damage 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, diffuse thickening of the endocardium, both parietal and valvular, occurs, sometimes with the development of heart defects. In the subacute course of systemic S., a peculiar interstitial myocarditis (see) is found with edema and proliferation of connective tissue, proliferative, and less often destructive-proliferative in the small branches of the coronary (coronary) arteries and arterioles. Occasionally, hyalinosis of the inner and outer membranes of the main trunks of the coronary arteries is detected.

With the so-called In true scleroderma kidneys, thrombosis, infarction, and necrosis of its cortex are observed. With histol. The study determined intimal proliferation, mucoid edema, thrombovasculitis of interlobular arteries, fibrinoid necrosis of afferent arterioles, inflammatory infiltration, dystrophy and necrosis of the tubular epithelium. Occasionally, fibrinoid necrosis and “wire loops” in the glomeruli of renal corpuscles occur. However, more often with systemic S., a picture of focal or chronic intracapillary proliferative-membranous glomerulonephritis is noted in the kidneys (see). As a result of the latter, secondary shrinkage of the kidneys may develop.

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

Clinical picture

The clinical picture is polysyndromic and reflects the systemic, progressive nature of the disease. Systemic S. 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 limitation of movements and a tendency to form contractures ( cm.); in some cases - with damage to internal organs ( digestive system, heart, lungs). Much less often, acute polysyndromic onset of the disease is observed, often with an increase in body temperature to 38°C and above, a rapidly progressing 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 significant, sometimes catastrophic, weight loss, observed during the period of generalization or rapid progression of the disease. Half of the patients have low-grade fever.

Rice. 7. Hand of a patient with sclerodactyly: areas of depigmentation and hyperpigmentation of the skin, deformation and shortening of the fingers due to osteolysis. Rice. 8. Mask-like appearance of the face in a patient with systemic scleroderma. Rice. 9. Face of a patient with systemic scleroderma: pale facial skin, telangiectasia. Rice. 10. Fingers of a patient with systemic scleroderma: thinning, focal hyperpigmentation, skin tension, which gives it shine (“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. Distal part of the foot of a patient with systemic scleroderma: partial amputation of the finger, dystrophic changes in the nails. Rice. 12. Thigh of a patient with plaque scleroderma: a skin lesion in the form of an ivory-colored area of ​​compaction 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 is observed in only 1/3 of cases at the onset of the disease. Ch. is localized. arr. on the hands - sclerodactyly (color fig. 7), on the face - mask-like (color fig. 8), the upper half of the body, feet; less often (mainly with a rapidly progressive course) observed diffuse lesion skin. Along with the characteristic scleroderma 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 (color. Fig. 9), trophic disorders(deformation of nails, baldness). In some patients, skin lesions of the limited C type are observed. Damage to the mucous membranes is often noted - 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.; it occurs, according to various researchers, in 70-90% of patients. Unlike Raynaud's disease, Raynaud's syndrome in systemic S. is more common: vascular changes are noted on the hands, feet, and sometimes in the face, similar changes are observed 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 (color Fig. 10) - repeated ulceration of the tissues of the fingertips up to the development of gangrene (see) .

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

Characteristic changes in bones are in the form of osteolysis (see), most often of the distal (nail) phalanges, 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 Tibierge-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 no earlier than 5 years from the onset of the disease. More often, tissue calcification does not cause discomfort and is detected only by x-ray, and if it is localized in the fingers, by the deformation of the latter. With a more rapid development of the process, often in the form of individual exacerbations, tissue infiltration with severe pain, deterioration of the general condition and sometimes a febrile reaction is revealed. When located superficially, foci of calcification may open with the release of a white crumbly or liquid mass.

Damage to the digestive tract, especially the esophagus and intestines, is observed in 60-70% of cases and has a characteristic clinical and radiological picture. Changes in the esophagus can be observed in the early stages of the 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 (see Esophagitis) are added, which is accompanied in a number of cases by the development of peptic ulcers (see), strictures, hiatal hernia (see). Scleroderma intestinal lesions manifest as 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 to the liver is manifested by its enlargement, in some cases by skin itching, periodically occurring jaundice, which indicates hron. hepatitis (see) or cirrhosis. Changes in the pancreas are rarely detected, mainly during functional studies.

Lung damage is observed in approximately 2/3 of patients; it is characterized by the gradual development of diffuse pyeumosclerosis (compact, less often cystic) with predominant localization in the basal sections, as well as the presence adhesive process and thickening (fibrosis) of the pleura. Wedge, signs of pneumosclerosis (see) in the initial stage are insignificant or absent, while functional disorders and rentgenol. changes are already in place. Therefore, the use of these research methods for the early diagnosis of scleroderma pulmonary fibrosis is recommended. The degree of severity and severity of pneumofibrosis is determined, first of all, by the activity of the scleroderma process. In patients with subacute S., interstitial pneumonia is observed (see). With severe pulmonary fibrosis, bronchiectasis, emphysema, perifocal pneumonia, and respiratory failure develop.

Damage to the heart, in particular to 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. Sclerodermic cardiosclerosis (see), which underlies myocardial damage, is characterized by an increase in heart size, rhythm disturbances (more often - extrasystole) and conductivity, weakening contractile function with areas of adynamia revealed by x-ray kymography (see) and especially clearly by echocardiography (see). Large-focal myocardial fibrosis is accompanied by infarction-like changes on the ECG and in some cases can lead to the development of a kind of “calloused” cardiac aneurysm. With systemic S., damage to the endocardium of the valves is possible with the formation of a heart defect, most often the left atrioventricular - mitral (see Acquired heart defects). This is characterized by a relatively benign course with the 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. Sclerodermic pericarditis (see) is predominantly adhesive in nature, although the section often records 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, revealed during functional studies, for example, renography using 131 I hippuran (see Renography radioisotope), as well as signs latent and, relatively rarely, hypertensive, nephrotic or mixed type (with subacute course) glomerulonephritis (see).

Described so-called. true scleroderma kidney is a condition characterized by catastrophic severity of disease development (2-4 weeks) and death. It is characterized by proteinuria (see), signs of rapidly increasing renal failure(see) - azotemia (see), oliguria (see) and terminal anuria (see), arterial hypertension (see Arterial hypertension), retinopathy (see) and encephalopathy (see). There is a similarity between certain pathogenetic features and morphol. signs of true scleroderma kidney with malignant arterial hypertension. For severe arterial hypertension is discovered high level renin in blood plasma. True scleroderma kidney develops, as a rule, in acute, rapidly progressing systemic S. and is the main cause of death in patients with this variant of the disease.

Damage to the nervous system with systemic S. is common. The leading syndrome is neurocirculatory dystonia (see). Already in the early stages of the disease, secretion is impaired sweat glands: first there is hyperhidrosis of the palms and axillary areas(see Hyperhidrosis), and then decreased sweating in areas of skin atrophy. Vegetative-vascular and associated 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°, absence of local and reflex dermographism (see).

With systemic S., polyneuropathic syndrome often occurs (see Polyneuritis). According to N.G. Guseva, it is observed in 1/3 of cases of the disease. Basically, polyneuropathic syndrome is manifested by sensory disturbances, patients complain of paresthesia (see) in the arms and legs, sometimes of pain. The examination reveals pain along the nerve trunks, hyperesthesia, and sometimes hypoesthesia or hyperpathia in the distal parts of the extremities in the form of “gloves” and “socks”. Movement disorders with S. are not typical, 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 severe paresis and sensitivity disorders, early extinction of tendon reflexes in the arms and legs, 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 meningo-encephalitic syndrome (see Encephalitis) or vascular disorders of a hemorrhagic or ischemic nature. Acute disorder cerebral circulation(see) can be fatal. Meningoencephalitic syndrome is characterized by headaches, dizziness and mild focal symptoms. A fairly typical change in the psyche with anxiety-depressive reactions, sometimes the development of an acute psychotic state with delirium, auditory and olfactory hallucinations, and amnesia. Cerebrospinal fluid pressure is increased and its protein content is increased. Nipple swelling may develop optic nerve(optic disc, T.).

The spinal cord is rarely affected; there are isolated descriptions of the development of symptoms of myelitis (see) and myelopolyradiculoneuritis (see). These phenomena are caused by vascular disorders associated with the underlying disease.

There are three main variants of the course of systemic S.: acute (rapidly progressing), subacute and chronic, which differ from each other in activity and speed of progression patol. process, degree of severity and nature of peripheral (skin, joint, etc.) and visceral manifestations. For the most frequent hron. The course is characterized by progressive vasomotor disorders (Raynaud's syndrome) and the resulting pronounced trophic disorders. They are often the only manifestation of the disease for a number of years and subsequently prevail in the picture of the disease. With chronic During the course of the disease, laboratory tests usually remain within normal limits 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 swelling of the skin with subsequent induration, recurrent polyarthritis (sometimes of the rheumatoid type), less often - myositis with myasthenic syndrome, polyserositis (see), visceral pathology - interstitial pneumonia with the subsequent development of pneumosclerosis, cardiosclerosis, scleroderma esophagitis (see .), duodenitis (see), chronic. glomerulonephritis, as well as vasomotor and trophic disorders.

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

Diagnosis

The diagnosis with a detailed picture of the disease does not cause difficulties; it is based ch. arr. on the wedge, manifestations of S. 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 “minor” criteria - sclerodactyly, trophic ulcers of the fingertips , bilateral basal pulmonary fibrosis.

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

Blood in systemic S. is changed little, only in some patients there is hypochromic anemia (see), leukopenia (see), and somewhat 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 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 hypergammaglobuliemia, which causes 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 anticentromere antibodies (the latter are mainly detected in 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 the blood plasma and urine is detected, which indicates severe disturbances in collagen metabolism.

X-ray research in systemic S. is important wedge, because, clarifying the picture of the disease, it helps to resolve the issue of diagnosis. Use of various rentgenol. methods depend on which organs and systems are being studied.

Typical for systemic S. changes in 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, areas of the elbows, knees and other joints. Osteolysis (see) is observed in the nail phalanges of the fingers and toes, the coronoid processes of the branches of the lower jaw, the distal parts of the radius and ulna, the posterior parts of the ribs and certain 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. tract, since it allows us to identify one of the most specific signs of the disease - decreased tone and weakened peristalsis, which leads to 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 colon.

When the lungs are affected, diffuse and cystic pneumosclerosis (see), often combined with moderate pulmonary emphysema (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 pulsation down to zones of adynamia (Fig. 6), which is clearly revealed by X-ray kymography (see). Signs of damage to the valve apparatus may be observed, mainly in the form of insufficiency of the left atrioventricular (mitral) valve, in some cases, stenosis of the left atrioventricular orifice and insufficiency of the aortic valve (changes in the 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 in the ear), with others diffuse diseases connective tissue, rheumatoid arthritis (see), with a group of pseudoscleroderma conditions.

Features of the wedge, the pictures make it relatively easy to distinguish between systemic and limited S., however, one should keep in mind the possibility of focal skin lesions with systemic S. Differential diagnosis with eosinophilic fasciitis is based on the diffuse induration of the deep layers of the fascia and subcutaneous tissue, characteristic of the latter (established by biopsy), mainly in the area of ​​the forearms, less often - 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 of the ear, in contrast to systemic S., the initial localization of the process is noted in the neck and face; The subcutaneous tissue is predominantly affected.

At rheumatoid arthritis, especially with 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, reminiscent of 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 characteristics 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, mask-like appearance of the face, dysphagia). Polymyositis (see Myositis) can be a manifestation of systemic S., but unlike damage to skeletal muscles in dermatomyositis, it rarely prevails in the picture of the disease and only for a short period of time. Differential diagnosis with systemic lupus erythematosus (see) is usually not difficult. It should be taken into account that antinuclear factor, antibodies to DNA (usually in small titres), and single LE cells can be observed in systemic S., more often in the subacute course.

Pseudoscleroderma syndromes with primary amyloids, inborn errors of metabolism - porphyria (see), phenylketonuria (see), hepatocerebral dystrophy (see), with certain endocrinopathies, for example, Werner syndrome (see Werner syndrome) and paraneoplastic syndromes (see), are characterized by ch. arr. skin-articular-muscular, less often - vascular symptoms, reminiscent, but not identical to the manifestations of systemic S. Atypia of peripheral and the absence of lesions of internal organs characteristic of S., along with wedge, features of pseudosclerodermic syndromes, are the basis of differential diagnosis.

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

Treatment

Treatment of patients with systemic S. is carried out for a long time (years). When choosing a complex to treat. measures must take into account the nature of the course, activity and stage of the disease. Among the drugs used are D-penicillamine, unithiol, corticosteroids, aminoquinoline drugs, immunosuppressants, non-steroidal anti-inflammatory, vasodilator and disaggregating agents, lidase, dimethyl sulfoxide, less often - centrally acting muscle relaxants, colchicine, heparin, grpzeofulvin, etc.

D-penicillamine inhibits the maturation and, in part, the biosynthesis of collagen. Sec. applies. 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 changes in the skin, joint 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, a transition from acute to subacute and even chronic was noted.

Unithiol, like D-penicillamine, contains sulfhydryl groups and affects collagen metabolism; it can be used in complex treatment C. Repeated courses of treatment with unithiol are prescribed; 5 ml of 5% solution is administered intramuscularly, for a course of 10-12 injections.

Glucocorticosteroids (mainly prednisolone) are prescribed if there is a wedge, and a lab. signs of pathol activity. process, in acute and subacute cases 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. until the wedge effect is achieved. 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 for articular, skin and vascular syndromes, certain visceral manifestations (myocarditis, interstitial pneumonia). They are not indicated for the development of true scleroderma kidney.

Aminoquinoline derivatives (chloroquine, resoquine, plaquenil) are used as the main type of treatment for subacute and especially chronic. during systemic S. Prescribe 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 ophthalmologist. These drugs have a positive effect mainly in case of articular syndrome.

Nonsteroidal 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, chlorobutin, etc.) are used for systemic S. relatively rarely, Ch. arr. with high activity patol. a process that is not amenable to the effects of corticosteroids, or in case of contraindications to treatment with them. Azathioprine is preferred for kidney damage such as glomerulonephritis. It is prescribed at a dose of 1-3 mg per 1 kg of the patient’s body weight (50-200 mg per day) for 2-3 months. under the control of blood tests. Vasodilators and disaggregants for systemic S. include complamin, angina, andecalin, nicotinic acid preparations, griseofulvpi, chimes, low-molecular dextran, etc. Hyperbaric oxygenation (see), balneotherapy and physiotherapy improve peripheral circulation.

For polyneuropathy, in addition to these drugs, B vitamins 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 required, including inhibitors of the renin-angiotensin system, repeated hemodialysis (see), in some cases a kidney transplant is indicated (see).

Lidaza is used for chronic during systemic S. with repeated courses in the form of subcutaneous injections of 64 - 128 IU (per course of 12-14 injections) or electrophoresis on the affected areas of the 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, and analgesics.

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

San.-kur. treatment using balneotherapy (radon, hydrogen sulfide, carbon dioxide baths), mud therapy, etc. is shown in Chapter. arr. with chronic during systemic S. In the absence of contraindications, early inclusion of massage and treatment in the therapeutic complex is possible. physical education.

Prognosis and Prevention

The prognosis is determined by the nature of the course, timeliness of diagnosis and adequacy of therapy. With chronic during the course of the disease, the prognosis is favorable, in subacute cases it is satisfactory, in acute cases it is unfavorable, especially in cases of the development of a true scleroderma kidney.

Prevention consists of eliminating external factors that provoke the development of systemic S. in individuals “threatened” with respect to the development of the disease: cooling, vibration, exposure to chemicals. substances, including silica dust, allergenic influences, etc. The group “threatened” with respect to the development of systemic S. includes persons with a tendency to vasospastic reactions, with limited S. or recurrent polyarthralgia, and 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 hospital and outpatient setting, clinical examination, rehabilitation measures, including the stage of resort treatment (mainly in chronic cases). Proper employment of patients and the exclusion of the above factors that provoke the development of S. and its exacerbations are necessary. In acute and subacute course of systemic S., patients, as a rule, are incapacitated and must be transferred to disability, and in chronic cases, they are of limited ability to work.

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

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 the disease in adults, are acute childhood infections, the administration of vaccines and serums.

Skin changes typical for systemic S. as the first symptom of the disease are observed in only half of the patients. It is not always possible to trace the successive change in the stages of skin changes. The same patient may have a combination of dense skin edema with induration, induration with atrophy, or the presence of all three stages simultaneously. Just like in adults, in children, in addition to the typical ones, there are skin changes in the form of limited S. and trophic disorders, pigmentation disorders. Telangiectasias are rare in children. 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 later the frequency vascular manifestations is growing. With a progressive process, the formation of trophic ulcers is possible (in 20% of patients). The joint syndrome is similar to that in adults. Already in the early stages of the disease, severe joint and muscle contractures often appear. Clinic muscle lesions, as well as the frequency of true myositis are the same as in adults. Osteolysis and calcification occur 2 times less frequently than in adults, however, unlike adults, they may appear in children. earlier periods - in the 2-3rd year of illness.

Lesions of internal organs in children, as a rule, are mild and progress slowly. However, with the help of instrumental research methods, a greater frequency and prevalence of visceral pathology is revealed. The most common changes observed are in the heart. The myocardium is affected in all patients, the pericardium is affected somewhat less frequently, but 4 times more often than in adults, the endocardium is affected in V3 sick children. Lung damage is in second place in frequency (in 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 to the esophagus in the form of impaired motility is diagnosed by 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, minor 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 approximately the same frequency. An acute course with a fatal outcome is possible in the first three years of the disease. Chron. forms of the disease that last for a long time with isolated syndrome Raynaud's, 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). Patol occurs as a rare complication. fractures of the bones of the lower extremities not associated with hormonal treatment.

Differential diagnosis should be made with scleredema (see) and phenylketonuria (see). The first is characterized by the staged appearance of induration, the absence of fibrosis and atrophy of soft tissues, articular and vasospastic manifestations; pathol. processes in the internal organs proceed benignly and subside as the skin induration disappears. With phenylketonuria, accompanied by hardening 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 treatment for systemic S. 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 damage to muscles and joints, the depth and prevalence of vascular disorders, and the addition of a secondary infection. As visceral lesions progress, the prognosis becomes worse.

Prevention is similar to S.'s prevention in adults; Careful and adequate treatment of childhood infections should be carried out, and the rules for routine vaccinations should be followed.

Limited scleroderma

Limited scleroderma (scleroderma circumscripta; syn.: focal S., localized S., keloid-like S., Addison's keloid). As with systemic 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.

Plaque S. (sclerodermia placata) is observed most often. It usually develops gradually, for no apparent reason, and has a long course with periods of exacerbations and remissions. Characterized by the formation on the lateral surface of the body, back, lower back or proximal limbs of one or several spots of various sizes, oval or irregular in shape, pinkish in color 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, cardboard-like, shiny, ivory-colored plaque is formed, slightly protruding above the level of the surrounding skin (color book fig. 12). At the periphery of the plaque there is purple a ring-shaped area that gradually turns into normal skin. This zone indicates the progression of the process. The formed plaque slowly increases in size, and areas of pigmentation and telangiectasia may form on it. In rare cases, multiple lesions are observed (generalized or disseminated plaque S.). After a few years, the lesion quietly resolves and undergoes atrophy, leaving a slightly pigmented recession of the skin. Atrophied skin, resembling crumpled tissue paper, easily folds. It is extremely rare that blisters with hemorrhagic contents (bullous-hemorrhagic plaque S.) or areas of superficial ulceration appear in the area of ​​plaques. Varieties of plaque S. are superficial limited S., in which small dark-colored spots with a lilac tint develop on the skin without signs of compaction and infiltration, as well as knotty form(tuberous, keloid-like) in the form of protruding nodes. In the area of ​​scleroderma plaques, hair falls out, and the secretion of sebaceous and sweat glands decreases.

Ribbon-shaped, or strip-shaped, S. (sclerodermia striata) is distinguished by the linear shape of skin lesions and often involvement in patol. process of the underlying tissues (subcutaneous tissue, muscles). S.'s foci are located along one of the limbs, sometimes along the nerves (sclerodermia zoniformis) or circularly, encircling the torso, the entire limb or finger (sclerodermia anularis). When tendons, ligaments and muscles are damaged, retractions and contractures are formed, limiting the range of motion in the joints. Localization of ribbon-like S. is possible on the face (in the area of ​​the bridge of the nose and forehead) and the scalp (reminiscent of a scar from a saber blow). Some researchers classify progressive facial atrophy as limited S. - Parry-Romberg disease (see Hemiatrophy).

Guttate 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 and can merge, forming large foci with scalloped outlines. After a few years, skin atrophy develops at the spots (see). The rashes are localized on the neck, upper chest or back, less often on the extremities. 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 planus).

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

For the treatment of limited S., lidase is widely used, which is administered subcutaneously or intramuscularly at 64 units every other day, for a course of 12-15 injections. Repeated courses are carried out after a 2-3 month break. Lidase electrophoresis and compresses with ronidase on the skin lesion are also effective. Apply intradermal or subcutaneous injection into the lesions of a suspension of hydrocortisone, 1 - 2 ml with 0.25% novocaine solution, 2 times a week, 6-8 injections; phonophoresis of hydrocortisone suspension; lubricating the lesions with dimethyl sulfoxide in its pure form or injecting the lesions with 1-5% dimethyl sulfoxide solution. Improvement can also be achieved by repeated novocaine blockades of the nodes of the sympathetic trunk, taking ganglion-blocking substances (pachycarpine). General strengthening treatment is carried out (vitamins B, A, PP, C). In the stage of skin thickening, thermal procedures (baths, mud therapy, paraffin therapy), light massage, sea and hydrogen sulfide baths (Sochi-Matsesta, Pyatigorsk), medical treatment are effective. physical training.

The prognosis for limited S. is favorable; no reliable cases of its transition to systemic S. have been described.

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

Bibliography: Nazarova V.D. and Balabanova R.M. Features of microcirculatory disorders in the cat with systemic scleroderma, Ter. arkh., t. 51, no. 6, p. 77, 1979; Vysotsky G. Ya. Systemic and focal scleroderma, L., 1971, bibliogr.; Gusev N. M. and Guseva N. G. X-ray cinematography in the diagnosis of heart damage in systemic scleroderma, Ter. arkh., t. 48, no. 5, p. 125, 1976; Guseva II. G. Systemic scleroderma, M., 1975, bibliogr.; Davidovsky L. Ya. Collagen diseases in children, Alma-Ata, 1979, bibliogr.; D o v-zhansky S.I. Scleroderma, Saratov, 1979, bibliogr.; K ukh and E. and Jablonska D. Lung damage in scleroderma, Klin, med., t. 56, JM# 4, p. 32, 1978; Multi-volume guide to pediatrics, ed. Yu. F. Dombrovskaya, t. 7, p. 286, M., 1964; Rakhmanov V.A. and X m e l n i t s k i y R.Kh. To the mechanism of action of lidase in the treatment of patients with scleroderma, Vestn. derm, i ven., No. 6, p. 3.1959; StrukovA. I., K o p e v a T. N. and K a k t u r-s k-i y L. V. Immunopathology of collagen diseases, Klin, med., t. 52, No. 1, p. 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 e r t 1 e r W. Systematische Dermatologie, Bd 1, S. 354, Lpz., 1970; I s h i k a-w a H. a. o. An approach to experimental scleroderma, using urinary glycosamin-glycans from patients with systemic scleroderma, Acta derm.-venereol. (Stockh.), v. 55, p. 97, 1975 J abl6nska St. Twardzina i stany rzekomotwardzinowe, Warszawa, 1963; K lug H., Barth e 1 m 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 about 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.

N. G. Guseva; T. N. Kopyeva (pat. an.), V. V. Mikheev, N. A. Ilyina (neur.), N. N. Uvarova (features of systemic scleroderma in children), Yu. S. Khomyakov (rent.) , O.K. Shaposhnikov (limited scleroderma)

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

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

So, scleroderma in children.

What is scleroderma?

Scleroderma is an inflammatory disease of connective tissue that is chronic and in most cases affects only the human skin. Despite this, many vitally important important organs. In medicine, scleroderma in children is called scleroderma of childhood 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 million suffer from this disease. Many of you may now think that this is not a threat to their children. It is possible that you are right, but there is still a risk. As for childhood scleroderma, it can have 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 vary in size. At the very beginning, these areas are slightly reddish and there is swelling on them. Then they become denser and take on an ivory hue. As a result, their atrophy occurs. Long absence treatment leads to the involvement of other areas of the skin in the existing inflammatory process. Most often, atherosclerosis also affects internal organs, but not in the case of children. If we talk about childhood scleroderma, it almost never affects 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 basic therapy, scientists were able to reveal that gold salts cope well with scleroderma in children. That is why it is most often used to treat this disease. auranofin as the main drug.

Dear parents, we remind you once again 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 a specialist.

Reviews

I have been sick since I was 2 years old. Now 37. If you list what you tried and what doctors you went to. Incl. Institute named after Sechenov in Moscow. Zero results. I have linear scleroderma. I accepted it and moved on with my life. I haven’t met a single doctor who said anything sensible. And how much lidase and electrophoresis were taught to me in childhood. Scary to imagine

My daughter developed a 1.5 spot at 6 months old and was diagnosed with systemic scleoderma and is already being treated for 1.5
Please tell me clinics or treatment methods

4 months ago my daughter fell ill with focal plaque scleroderma, we are being seen in the rheumatology department in Samara, we visited dermatologists but they were treated with penicillin and lidase, the department recommended Medacassol, they started injecting Methodject to stop the process, we applied egallochite, solcoseryl and carepoin according to queue, write if anyone has anything new in treatment, maybe someone can recommend in Moscow where to turn and to whom...

Good afternoon, my daughter has linear scleroderma, we have been undergoing treatment 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, help, tell me if clay cures this sore

My 4.5-year-old daughter is diagnosed with focal scleroderma, which is widespread; in Kyrgyzstan they don’t treat children, but in Moscow how much will treatment cost?

My son is 5 years old, in June 2013, spots the size of a fingernail appeared on the upper part of his legs, one on the outer and inner sides for a total of 4. I went to a skin clinic. They determined that it was not lichen. The doctor couldn’t say anything more. Now it’s 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 of 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. Previously, during 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 based on a quota. Our authorities do not issue a quota and we are receiving treatment at home, but there is no improvement. I ask you for help, maybe someone can help or tell me what to do and where to go. The child has his whole life ahead of him, and it seems to me that if it’s still at the initial stage, at least something else can be done. We are being treated with Methodject, drank cuprenil, all of this in loading doses, we apply madecasol, nothing helps. Help us how we can get from Kazakhstan for treatment in Moscow at the Sechenov Institute and how much it will cost.

I have been suffering from scleroderma since childhood, I was treated in Almaty at the Institute of Pediatrics, and now in Astana at the national center I take 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 3 years old then. The spots appeared and disappeared. After some time we went to the clinic. Several diagnoses were made, but none were confirmed. They did a skin biopsy, after which they made this terrible diagnosis. As treatment we take Cuprenil and a combination of ointments, different every month. A few months later I went back to the hospital for a check-up. the doctors reassured me - they said that not everything was so scary, light form, but we still worry. I heard about the institute. Sechenov, but we can’t get there, since we live in Kazakhstan. Maybe someone was able to cure a child in Kazakhstan, write where and how, I will be very grateful.

My son is 13 years old, he fell ill with scleroderma at the age of 11. For 1.5 years we were driven from one hospital to another, injected with pinicillin and lidase, but the spots grew larger every day, and then by some miracle we ended up in Moscow at the Sechenov Institute. Thank God the spots stopped.

My son is 17 years old. Diagnosis: Focal scleroderma. Hyperpigmentation throughout the body. No organs involved. They prescribed treatment with the drug penicillamine D and a bunch of ointments. At the same time, the leading rheumatologist said that he had seen this for the first time. The tests are all normal. What and where. If they show a photo of scleroderma and lesion 2 or 3. Then you can do some kind of compresses. And my son has it all over his body. Who else can I contact?

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

After receiving a flu shot, my son fell ill with scleroderma in 2002. We were treated in Moscow, at the Sechenov Clinic of 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 suggested the address of Sechenov’s clinic. All the years of my son’s illness I have been studying this issue, I can say with confidence that this is the only place where they can help your children. They helped us and hundreds of children whom I saw entering our department over the course of six years. With this disease, under no circumstances should you take a biopsy, injure the affected areas or give injections in them, and of course you should not sunbathe. Treatment with penicillin, lidase is a thing of the past, many side effects, there are no significant improvements (in my time in 2005, a girl was admitted to the department after four years of treatment with penicillin at the TsNIKVI in Moscow, there was no improvement, her face was deformed, her lips did not close on the right side (you can’t look at this without tears). Previously, the clinic also treated penicillin, etc. but the results were bad, 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 doctors they are trying to prescribe a similar treatment, but probably they don’t have enough experience, they brought in “cured” children, it seems the drugs are correct, but the doses are too high, that’s also not possible. We were able to achieve remission, and for six years we went there several times a year, we took the medicine every day for several years , but these are little things - the main thing is the given opportunity for my child to live. Children there from all over Russia and the CIS also call from abroad for consultations. The parents’ mistake is that many do not consider the disease to be terrible, children were brought to the clinic half “ossified”, the danger is that , that even if you then go into remission, the affected area still stops growing, resulting in disfigured faces, arms, and legs of children. Now we don’t take medications, we monitor the tests over time. I wish all mothers to help their children, don’t be afraid, the main thing is don’t give up.

By the way, it’s very strange, but no one mentioned such a medicine as Piascledine. He really helped me. When the disease began, he was not in Russia; he was brought from France through Germany. Piaskledin is now available for sale. It is better to contact the Institute of Rheumatology. Antibiotics and hormonal drugs were not recommended to me at all. And it depends on what kind of cream it is. An allergy may also develop. There is also a ban on solariums and active recreation under the sun. In the summer, don’t go outside without a cream with protection less than 30! And so every day I apply the Clean Line series cream for dry skin (wheat germ) with vitamins A and B to the affected area (medial part of the thigh).