Limited scleroderma in children. Symptoms of localized scleroderma

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

Keywords: Scleroderma - etiology - pathogenesis - autoimmune 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, DCTs include SSc, systemic lupus erythematosus (SLE), dermatomyositis, periarteritis nodosa and rheumatoid arthritis are formidable diseases that require a specific strategy and tactics for patient management and 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. think that increased level Collagen proteins, being a source of active antigenic stimulation, create a background against which autoimmune reactions are realized if there is a genetic predisposition. The emerging vicious circle of mutual influence of lymphoid and collagen-synthesizing cells leads to the progression of the fibrotic process.
With scleroderma, a variety of other autoimmune disorders are observed: various autoantibodies, a decrease in the level of T-lymphocytes with unchanged or increased content of B-lymphocytes, a decrease in the function of T-suppressors with unchanged or increased function T-helper cells, decreased functional activity natural killers.
In 20 - 40% of cases with plaque scleroderma, antinuclear antibodies are detected, in 30 - 74% of patients with scleroderma, circulating immune complexes.

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 OS classification is based on 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, the function of the sebaceous glands and hair growth are impaired.
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. The sequential formation of vesicular-bullous and erosive-ulcerative lesions is 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. The consequence of a long progressive course may also be functional impairment 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 the intensity of the lesion, are subject to instrumental examination in order to early diagnosis 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, assessing the condition of internal organs using instrumental methods in children with OS it 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 the possible development of a 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 heart lesions (scleroderma heart - disturbance of 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, disorders rhythm, 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, correction functional disorders nervous, endocrine, 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.
Among 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 by applying its powder (0.5 - 1.0 g) to the moistened saline solution napkin. Apply a napkin to the lesion, fixing it with a bandage for half a day. The course of applications continues for 2 - 3 weeks.
Favorable influence Electrophoresis with a 0.5% solution of zinc sulfate helps resolve 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 circulation and trophic processes in the lesions contribute to the resolution of 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 stimulate 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 is allowed or reduced to subclinical signs usually around 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 provided that scleroderma is diagnosed early and the necessary comprehensive course of treatment is carried out in a timely manner.

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Systemic scleroderma(DM) is a systemic disease of connective tissue and small vessels, characterized by widespread fibrosclerotic changes in the skin, stroma of internal organs and widespread vasospastic disorders such as Raynaud's syndrome. DM ranks second in frequency among DBST.

Girls are more often affected (in focal forms the ratio of boys to girls is 1.5:1, in systemic forms it is 15:1).

Etiology and pathogenesis of the disease complex and far from fully understood. The etiology of SSc can be represented as a combination of genetic predisposition with the influence of unfavorable exo- and endogenous factors. Environmental factors include long-term cooling, vibration, mental stress, contact with viruses and toxins (during the production of polyvinyl chloride).

Among the genetic aspects, the association of certain antigens and alleles of the HLA histocompatibility system should be noted; 90% of patients have chromosomal abnormalities (chromatid breaks, the presence of marginal fragments and ring chromosomes).

The pathogenesis of diabetes is associated with excessive activation of fibroblasts and excessive formation of immature collagen fibers, the development of unregulated fibrosis, and then sclerosis of internal organs.

An increase in the functional activity of fibroblasts and other collagen-forming cells (especially smooth muscle cells of the vascular wall) leads to increased production of soluble immature collagen types I and III, which is accompanied by damage to endothelial vascular cells with their replacement by smooth muscle cells (collagen producers), as a result of which the ability of blood vessels to spasm increases, and internal choroid hyperplastic.

In addition, damage to the endothelium itself leads to adhesion and aggregation of platelets, leukocytes, erythrocytes with the development of intravascular stasis, coagulation, microthrombosis, which is clinically realized by generalized Raynaud's syndrome.

In addition to the vascular mechanism, the participation of immunocompetent cells in the local and general pathogenesis of the disease and their relationship with fibroblasts has been proven; the presence of various immune and autoimmune reactions, including the detection of SSc-specific anticentromere antibodies (ACAs) and antipolymerase-1 antibodies (ATA), antinuclear autoantibodies and antibodies to various components of connective tissue.

Classification of diabetes identifies clinical forms (focal and systemic), various variants of the course, stages, and activity of the disease.

Acute course DM is characterized by severe fibrous peripheral lesions already in the first year of the disease, often with the development of glomerulosclerosis (scleroderma kidney) and death.

Subacute course(more often in children) is characterized by the presence of dense swelling of the skin with subsequent induration (thickening), recurrent polyarthritis (rheumatoid type), less often myositis, polyserositis, visceritis (interstitial pneumonia with outcome in pneumosclerosis, myocardosis with the development of primary cardiosclerosis, sclerodermic esophagitis, duodenitis, glomerulonephritis). Vasomotor trophic disorders are not clearly expressed.

Chronic course(more in adults) is characterized by progressive vasomotor disorders such as Raynaud's syndrome and resulting trophic disorders over a number of years.

Subsequently, they prevail in the picture of the disease with gradual thickening of the skin and periarticular tissues, the formation of contractures, osteolysis and slow sclerosis of internal organs (esophagus, lungs, heart).

Among the stages of SSD, there are stage I of initial manifestations (mainly articular in subacute and vasospastic in chronic cases), stage II of generalization of the process (with polysyndromic and polysystemic lesions and less pronounced therapeutic effect) and stage III of advanced changes, terminal (with a predominance of severe sclerotic, dystrophic or vascular-necrotic processes and dysfunction of organs).

According to activity, grade I is distinguished (minimal, with chronic and subacute course, against the background effective treatment), II degree (moderate, with subacute and exacerbation of chronic course) and III degree (maximum, with acute and subacute course).

Clinical picture of diabetes. Focal diabetes can manifest itself in the form of plaque and linear forms.

At plaque form on early stages observe the appearance of yellowish-pinkish erythematous plaques that evolve into firm, waxy or yellowish-white (ivory) focal lesions (sometimes with a rim purple), various localizations (usually on the limbs and torso).

IN initial stage linear SD the pattern of skin changes is similar, but a linear configuration quickly appears, which looks like a wide stripe, often located along the neurovascular bundle of either limb.

A special local form of SD is located on the forehead and is called a “saber strike”. The linear form is not limited to damage only to the skin - all underlying tissues (fiber, muscles, fascia and even bones) are involved in the pathological process, which leads to large deformations.

In addition to skin lesions, with focal DM, arthritis with morning stiffness, limitation of movements, but without pronounced inflammatory changes can be observed.

In some patients, Raynaud's syndrome can be detected, characterized by a three-phase vasomotor reaction (whitening - cyanosis - hyperemia) after cooling, excitement, or fatigue. At first, Raynaud's syndrome occurs episodically, involving the distal parts of several fingers, and then all fingers and toes are involved, less often the nose, lips, and ears.

In 20% of patients with focal diabetes, radiological signs of esophageal motility disorders can be detected in the absence of clinical manifestations esophagitis. These changes are unstable (they are not detected during repeated examination) and cannot indicate systemic diabetes.

System SD It is extremely rare in children. Initial manifestation In this case, Raynaud's syndrome occurs, which can last for several months or even years. Other manifestations may also be observed: a feeling of numbness, paresthesia of the limbs, face, torso (especially after hypothermia); stiffness in the hands, contractures of the fingers, discomfort in the joints; “unreasonable” fever (initially low-grade); “unreasonable” weight loss.

The diagnosis is established when diffuse thickening of the fingers and toes appears, and these changes become proximal to the metocarpo- and metotarsophalangeal joints (this sign is practically pathognomonic for distinguishing between systemic and local forms of diabetes). Skin lesions in diabetes go through three phases:

1. Edema phase (early): occurs due to damage vascular endothelium and increased permeability of the vascular wall, which leads to bilateral swelling of the hands and face with further thickening of the skin.

2. Induration (hardening) phase: characterized by increased collagen synthesis - the skin color changes (“salt and pepper”), the skin becomes taut, shiny, the pattern of blood vessels is clearly visible, telangiectasias appear (persistent dilation of capillaries with the formation of dark red spots on the skin) . Subcutaneous calcification appears in places frequently exposed to trauma (fingertips, elbows, knees). As it progresses, trophic disorders develop - baldness, nail deformation, ulcerations, ulcers.

3. Phase of atrophy of the skin and appendages with characteristic adhesion of the skin to the underlying tissues. The skin is thinned and shiny. The nose is pointed. There is a purse-shaped fold around the mouth. Due to thickening of the skin on the fingers and hands, flexion contractures develop, followed by shortening of the fingers with osteolysis of individual flanks

As a rule, with systemic diabetes, internal organs are also involved in the process - the esophagus (esophagitis), lungs (pulmonary tissue fibrosis), heart (peri- and myocarditis), kidneys (nephritis with the development of malignant hypertension).

Clinical and laboratory diagnostics of diabetes is important for establishing the activity of the process. There are moderate increase in ESR, hyper-g-globulinemia, scleroderma ATs are detected.

Diagnostic criteria for diabetes proposed by A.T. Mazi et al. (1980):

Big Criterion: proximal scleroderma - symmetrical thickening and induration of the skin of the fingers, as well as areas of the skin located proximal to the metacarpophalangeal and metatarsophalangeal joints; changes may affect the face, neck, torso, chest and abdomen.

Small criteria: 1. Sclerodactyly (the changes listed above, limited to the involvement of only the fingers).

2. Scarring on the fingertips or loss of substance in the fingertips.

3. Bilateral basal pulmonary fibrosis - bilateral mesh or linear nodular shadows, most pronounced in the basal areas of the lungs (changes like “honeycomb” lung are possible), and there is no connection between these changes and primary lung damage.

To make a diagnosis, it is necessary to have a major criterion or at least two minor ones (method sensitivity 97%, specificity 98%).

Dermatomyositis in children

Dermatomyositis(DM) is a systemic non-purulent inflammatory disease of the skeletal and smooth muscle and skin with typical skin rashes. In ¼ of cases the disease is limited to the muscular system (polymyositis).

DM is a rare disease (prevalence 0.5-0.8 per 100,000 population). All age groups are affected (the peak occurs at the age of 1-15 years), girls are more often affected (the ratio of girls to boys is 1.5:1.0).

Etiology of DM not installed. There is evidence of the relationship between DM and enteroviruses (Coxsackie, ECHO) and Toxoplasma. A possible genetic predisposition is assumed - associations of DM with the HLA DQA1 antigen (59-100%) and the TNF-308A allele and their correlation with myositis-specific antibodies (AT) were identified. Predisposition to the disease is realized in combination with constitutional ones (high threshold of stigmatization, frequency hypermobility syndrome) and environmental (infectious, endocrine) factors.

Pathogenesis. It is believed that the virus can have a direct damaging effect on muscle tissue, acting through an immune response to viral antigens located on the surface of muscle fibers, through the mechanism of antigenic mimicry. Immune vasculopathy is believed to play a central role in the pathogenesis of DM. First of all, the endothelium of small-caliber vessels (capillaries, venules and small arteries) in the skin, muscles, and gastrointestinal tract suffers. When damaged, endothelial cells swell and become necrotic, narrowing, along with thrombosis, the lumen of blood vessels, causing tissue ischemia.

Immune disorders have been proven at the cellular and humoral levels: the formation of Mi-2 (antinuclear antibodies - AT) - in 20% of cases, AT to muscle tissue, the vascular wall; cytotoxic lymphocytes and lymphokines. According to some data, myositis-specific antibodies are detected in the active phase of the disease and rarely (in 1/3 of cases) when the activity subsides. The result is the development of an inflammatory and degenerative pathological process in the muscles, systemic vasculitis.

Classification of DM distinguishes acute, subacute and chronic course of the disease.

At acute course after 3-6 months from the onset of the disease, a catastrophically increasing generalized lesion of the striated muscles is observed, up to complete immobility, the development of dysphagia and dysarthria. There is a general severe febrile-toxic condition with various skin rashes. The cause of death is usually aspiration pneumonia or pulmonary-heart failure caused by damage to the lungs or heart. The prognosis improves with treatment with large doses of glucocorticoids (GC).

Subacute course characterized by cyclicality, but still adynamia, damage to the skin and internal organs are steadily increasing. With the use of GC, recovery is possible with the preservation of pronounced amyotrophies, contractures, and calcifications that impair the ability to work.

Chronic course is the most favorable, only certain muscle groups are affected, and therefore, despite a significant number of exacerbations, general state patients remain satisfactory, they remain able to work for a long time.

Clinical picture of DM is distinguished by its diversity due to generalized damage to the microvasculature, a progressive inflammatory-necrotic process in striated and smooth muscles, and progressive muscle weakness. With the polymorphism of clinical symptoms, the leading ones in DM are skin and muscle syndromes.

Damage to skeletal muscles. The cardinal symptom of DM is symmetrical weakness of the proximal muscle groups of the limbs and trunk muscles of varying severity. The muscles most often affected are the shoulder and pelvic girdles, neck flexors and abdominal muscles.

Usually, parents begin to notice that the child has difficulty performing actions that he previously performed without difficulty: climbing stairs, getting up from a low chair, from bed, from a potty, from the floor. It is difficult for a child to sit on the floor from a standing position; he has to lean on a chair or his knees to lift the toy from the floor. Progression leads to the fact that the child cannot hold his head up well, cannot dress himself or comb his hair. Often parents consider these symptoms to be a manifestation of general weakness and do not pay attention to them. With severe muscle weakness, the child often cannot lift his head or leg from the bed, sit up from a lying position, and in more severe cases cannot walk. Involvement of the intercostal muscles and diaphragm can lead to respiratory failure, and the pharyngeal muscles to dysphagia and dysphonia.

Often patients complain of muscle pain. The pain can come from the muscles, joints, fascia, ligaments or tendons. Symptoms of muscle damage may precede skin manifestations.

Skin changes. The classic cutaneous manifestations of DM are Gottron's sign and heliotrope rash. Gottron's symptom consists of erythematous and sometimes scaly skin elements, nodules and plaques that rise above the surface of the skin of the extensor joints - interphalangeal, metacarpophalangeal, elbow, knee, ankle. The classic heliotrope rash is a purple or erythematous periorbital skin rash on the upper eyelids and the space between the upper eyelid and eyebrow (the “purple glasses” sign), often in combination with periorbital edema. The erythematous rash can also be located on the face, chest and neck, not upper back and upper arms (shawl symptom), abdomen, buttocks, thighs, legs. An early sign of the disease may be changes in the nail bed, such as hyperemia of the periungual ridges and overgrowth of the cuticle. Skin manifestations may precede muscle involvement by a year or more. Isolated skin syndrome at its onset is more common than the “muscular” or “musculocutaneous” onset of the disease.

Soft tissue calcification usually occurs in the 2-3rd year of the disease, but is possible in the 5-8th and even 10th year of the disease. More often this manifestation of DM occurs in preschool age, with a continuously recurrent, undulating and chronic course. Calcification is the deposition of deposits of calcium salts (hydroxyapatites) in the skin, subcutaneous fat, muscles or intermuscular fascia in the form of single nodules, large tumor-like formations, superficial plaques, or may be widespread. If calcifications are located superficially, it is possible inflammatory reaction surrounding tissues, suppuration and rejection in the form of crumbly masses. Deeply located calcifications can only be detected by x-ray.

Heart damage. System muscle process and systemic vasculopathy cause frequent involvement of the myocardium in the pathological process, although all three membranes of the heart and coronary vessels may suffer, up to the development of a heart attack. However, the low severity of clinical symptoms and their nonspecificity explain the difficulty of clinical diagnosis of carditis. IN active period Patients experience tachycardia, muffled heart sounds, expansion of the boundaries of the heart, and cardiac arrhythmias. Echocardiography in the case of myocarditis reveals expansion of the cavities of the heart, thickening of the walls, decreased contractile and pumping functions of the myocardium, and in the presence of pericarditis, separation of the pericardial layers.

Damage to the gastrointestinal tract. The main cause of gastrointestinal damage in DM is widespread vasculitis with the development of trophic disorders, impaired innervation and damage to smooth muscles. Always alarming in the DM clinic is the appearance of complaints of pain in the throat and along the esophagus, aggravated by swallowing, abdominal pain, which is of a mild, diffuse nature. Pain syndrome can have several causes at its core. The most serious is the development of esophagitis, gastroduodenitis, enterocolitis, due to catarrhal inflammation or an erosive-ulcerative process. In this case, minor or profuse bleeding may be observed, perforations are possible, leading to mediastinitis, peritonitis, which can cause the death of the child.

Other clinical manifestations. In addition to the general symptoms listed above (fever, general weakness), DM is characterized by severe dystrophy due to the general severity of the disease, muscle dystrophy and sclerosis, and atrophy of subcutaneous fat. With DM, lesions are observed in the mucous membranes of the oral cavity, upper respiratory tract, conjunctiva, and vagina.

Joint syndrome with DM it can manifest itself as arthralgia, limited mobility in joints, and morning stiffness. Exudative changes in joints are less common. More typical is the formation of tendon-muscular contractures, often in the wrist, elbow, hip and knee joints, which, with persistent rehabilitation measures in remission, have the possibility of complete reverse dynamics.

Lung damage. Defeat respiratory system occurs quite often and is due to the involvement of the respiratory muscles in the process with the development of respiratory failure and pharyngeal muscles with impaired swallowing and the possible development aspiration pneumonia. Infrequently, “antisynthetase syndrome” occurs, which is characterized by seasonality, acute onset of myositis, Raynaud’s syndrome, “mechanic’s hand” (hyperkeratosis, roughness and cracks in the skin of the palms).

Current of DM can be acute (in 10.8% of cases), subacute (83%) and primary chronic (6.2%).

Acute course accompanied by high fever, prostration, muscle tightening, and soreness. Sometimes the pathological process is limited only to muscle tissue (polymyositis), but more often skin changes typical of DM also appear. The most serious complication of the acute onset of DM is damage to the muscles of the palate and respiratory muscles with the appearance of the inability to swallow and a sharp decrease in the volume of excursion chest.

At the most frequent subacute course typical skin changes develop - bright red or violet-bluish erythema around the eyes (“heliotropic eyelids” or “dermatomyositis glasses”) and on open areas of the body (décolleté type), swelling of the eyelids and supraorbital region, hermitema scaly rashes over small joints hands, elbows, knee joints(Gottron's symptom), redness and peeling of the skin of the palms ("mechanic's hands"), vertical nail telangiectasia, skin calcification. Less common are generalized rashes, more pronounced on the chest. This form of onset is characterized by arthralgia.

At primary chronic course characterized by a gradual onset and slow progression of symptoms over several years in the form of dermatitis, hyperpigmentation, hyperkeratosis, and minimal visceral pathology. General dystrophic changes, muscle atrophy and sclerosis predominate, and there is a tendency to develop calcifications and contractures.

Laboratory and instrumental data. In acute cases, signs of laboratory activity are detected (increased ESR, leukocytosis); in subacute cases, they may be absent.

Due to muscle damage in the blood, the content of myospecific enzymes (creatine phosphokinase, g-glutamate aminotransferase, lactate dehydrogenase) increases more than 50 times; evidence of increased muscle catabolism is an increase in the concentration of muscle creatinine. Myoglobin is detected in the urinary sediment, the ratio of the concentration of creatine to the sum of the concentrations of creatine and creatinine changes (exceeds 40%).

Immunological studies reveal high titers of rheumatoid factor (ABs that react with the Fe fragment of Ig G), and antinuclear antibodies are positive.

Electromyography of the affected muscles describes increased muscle excitability, action potentials with low amplitude, polyphasic action potentials, fibrillation (signs of myositis).

To clarify the diagnosis, it is possible to perform a muscle biopsy (deltoid or femoral quadriceps) to identify characteristic pathomorphological changes.

Diagnosis of DM established on the basis of the development in a child under 16 years of typical clinical symptom complex. The differential diagnosis of DM is carried out with a large group of inflammatory myopathies (Duchenne muscular dystrophy, myasthenia gravis, infectious myositis, toxic and drug-induced myopathies, etc.). Most often, differential diagnosis must be made between DM and myositis syndrome in other rheumatic diseases.

The generally accepted diagnostic criteria are those proposed by Bohan and Peter (1975), revised and expanded by Tahimoto et al. (1995). According to this classification, the diagnosis of DM requires at least one of the skin criteria in combination with 4 of the criteria for polymyositis.

Diagnostic criteria for DM and polymyositis (Tahimoto et al., 1995):

Skin criteria:

1) heliotrope rash (red-purple edematous erythema on the upper eyelids)

2) Gottron's sign (red-violet atrophic erythema over the extensor surfaces of the finger joints)

3) erythema of the extensor surface of the joints

Criteria for polymyositis: the following:

1. Weakness of the proximal muscles for at least 1 month.

2. Myalgia for 1 month in the absence of sensory impairment.

3. The ratio of the concentration of creatine in the urine to the sum of the concentrations of creatine and creatinine in the urine is more than 40%.

4. A significant increase in the blood level of creatine phosphokinase or transaminases in the absence of other reasons.

5. Degenerative changes in muscle fibers during biopsy.

The diagnosis is considered reliable if 4 signs are present, probable - if 3 are present, possible - 2.

Similar criteria are given by N.P. Shabalov (2001):

1. Classic dermatomyositis rash (main criterion).

2. Symmetrical proximal muscle weakness.

3. Increased levels of muscle enzymes in blood serum.

4. Electromyographic findings characteristic of DM.

5. Typical findings in muscle biopsies for DM (muscle biopsy is performed only in the absence of the 2nd, 3rd or 4th signs).

Diagnosis requires the presence of a primary criterion (characteristic rash) in combination with any of 3 other criteria.

Periarteritis nodosa

Periarteritis nodosa(UP) – systemic necrotizing vasculitis of the type of segmental damage to small and medium-sized arteries with the formation of aneurysmal protrusions. Belongs to the group of systemic vasculitis with exceptional clinical polymorphism. Mostly boys and young men are affected.

Etiology of UP. UP develops after respiratory (including streptococcal) infections, drug intolerance (sulfonamides, penicillins, iodides, thiouracil, bismuth preparations, hypothiazide), after the administration of vaccines and serums. Very often, markers of chronic hepatitis B are found in UP.

Pathogenesis of UP. A variety of pathogenic factors ( streptococcal infection, viruses, serotherapy, antibiotics, sulfonamides) indicate that the hyperergic reaction of the body is of decisive importance in the development of UP. According to modern concepts, with UP there is both an immediate and delayed hypersensitivity reaction with a violation of humoral and cellular immune mechanisms. The main changes during a hyperergic reaction develop in small and medium-sized arteries. Immune complexes play an important role, leading to the activation of complement and the accumulation of leukocytes in the zone of their fixation (in medium and small arteries). In the acute stage, neutrophils infiltrate all layers of the vessel wall, which leads to its degeneration. As the process becomes chronic, the vascular wall becomes infiltrated with mononuclear cells with the development of fibrinoid necrosis, which leads to narrowing of the vessel lumen, thrombosis, and infarction. As healing proceeds, collagen is deposited in the affected area, which leads to further occlusion of the vessel.

Thus, with UP, simultaneous damage to the vascular endothelium (deposition of immunocomplexes), the internal elastic membrane (polymorphic cell inflammation) and perivascular tissue (infiltration, scarring) occurs.

A characteristic morphological feature of UP are distinct thickenings of the affected arteries (“periarteritis nodosa”), aneurysms with a diameter of up to 1 cm, found in the vessels of the kidneys, heart, central nervous system, and abdominal organs.

UE classification. In children, the following clinical variants of the disease are distinguished: with predominant damage to peripheral vessels, with predominant damage to internal organs and isolated damage to the skin or internal organs. According to the course – acute, subacute and chronic. Clinical syndromes are also distinguished: skin, thromboangitis, muscle, articular, neurological, cardiac, abdominal, renal and pulmonary. Complications: cerebral hemorrhage, pulmonary hemorrhage, rupture of a coronary aneurysm, rupture of the liver, spleen, kidney, perforation of an intestinal ulcer, peritonitis. Outcomes: complete remission, relative clinical and laboratory remission, disability.

Clinical picture of UP. Due to the extent of damage to small and medium arteries, the clinical picture is polymorphic. The onset is usually acute, with fever, muscle pain and rapidly increasing weight loss, weakness, lack of appetite, sweating, pain various localizations. Against this background, clinical syndromes characteristic of UP are identified - leading ones, which determine the severity of the patient’s condition, and accompanying ones, reflecting the systemic nature of the lesion. In children, skin, thromboangitis, muscle, articular, neurological and cardiac syndromes are more common. Abdominal, renal and pulmonary syndromes are observed less frequently.

Cutaneous and thromboangitic syndromes are caused mainly by damage to small and medium-sized peripheral arteries and are characterized by a wide variety of rashes, often hemorrhagic (erythematous, maculopapular, hemorrhagic, urticarial), subcutaneous and intradermal nodules, livedo, local edema, necrosis, gangrene and subsequent necrosis at the site of the rash, atrophy and even gangrene.

Most often found livedo(“stasis syndrome”), which manifests itself in the first days or at the height of the disease against the background of fever, it may be preceded by hyperesthesia. Livedo is a persistent and long-lasting skin symptom, shaped like a network or tree branches of a purplish-cyanotic color and localized on the extensor surfaces of the distal arms and legs, sometimes on the hips, buttocks, shoulders, back, face.

Painful subcutaneous nodules up to 1 cm in diameter, palpated along the affected vessels (granulomas or aneurysms), which give the name to the disease, are located in the area of ​​the forearms, legs, thighs, abdomen, face, scalp, are not always determined. The number of nodules is variable - from single to multiple; sizes from millet to pea and nut. They usually regress within 1-2 weeks.

Local edema located above large joints or spread to the hands, feet, lower back, face, similar to Quincke's edema. As the skin in the area of ​​edema progresses, it becomes cyanotic and cold, then diffuse hemorrhages occur, in place of which dry necrosis. In severe cases it develops distal gangrene.

Along with necrotic changes in the skin and distal gangrene, at the height of the process activity, damage to the mucous membranes, stomatitis, wedge-shaped necrosis of the tongue, necrosis is observed soft palate, necrotizing tonsillitis.

Joint and muscle syndromes– manifest themselves in the form of symmetrical arthralgia and myalgia of a paroxysmal nature. These lesions are characterized by complete functional reversibility.

Neurological syndrome. The nervous system is affected simultaneously at all levels or sequentially at different levels. Based on the brain vascular disorders lies a combination of two interacting factors - arterial hypertension due to kidney damage and cerebral vasculitis. Symptoms of the lesion develop acutely, often in the form of a transient disorder cerebral circulation and cerebral vascular crisis (headache, vomiting, meningeal syndrome, epileptiform seizures, convulsive syndrome, loss of consciousness from several hours to 2 days followed by aphasia, mental disorders). Against the background of a cerebral crisis, symptoms of focal brain damage may appear, mainly with motor disturbances. In addition, there may be signs of damage to the optic and auditory nerves. The interest of the diencephalic-hypothalamic region is evidenced by such clinical symptoms as anorexia, progressive cachexia, diffuse symmetric marbling of the skin, and severe sweating.

Damage to the peripheral nervous system is less common. It manifests itself with symptoms of mononeuritis, asymmetric polyneuritis, and polyradiculoneuritis.

A abdominal syndrome. Abdominal syndrome may be caused by vasospasm, compensated by impaired mesenteric circulation, intestinal paresis, arteritis of the vessels feeding appendix and gallbladder, infarction and intestinal necrosis, peritonitis.

At abdominal syndrome Along with skin rashes, there are paroxysmal abdominal pains against the background of increased body temperature, without clear localization and are often accompanied by dyspeptic symptoms (anorexia, vomiting, alternating diarrhea with constipation). As the process progresses painful attacks occur more often and become more persistent and the picture develops acute abdomen.

Quite quickly, damage to internal organs occurs - kidneys (in the form of a hematuric form of nephritis with persistent arterial hypertension), heart (myocarditis), gastrointestinal tract (ulcerative lesions with possible intestinal bleeding), nervous system (peripheral neuritis, cerebral infarctions, convulsions, psychoses), lungs (bronchial asthma syndrome with persistent eosinophilia, pulmonary vasculitis with hemoptysis, shortness of breath and influenza-type infiltrates), joints (arthralgia, migratory arthritis of large joints), muscles (myalgia), etc. Persistent hyperthermia is characteristic (antibiotics are ineffective in 2/3 of patients). Weight loss in patients (up to cachexia) correlates with the activity of the process.

Painful subcutaneous nodules up to 1 cm in diameter, palpated along the affected vessels (granulomas or aneurysms), which give the name to the disease, are detected only in 5-10% of cases.

Clinical and laboratory diagnostics of UP. The disease is characterized by high laboratory activity. In the peripheral blood, leukocytosis, thrombocytosis, an increase in ESR are determined, in the blood serum - an increase in urea, in the urinary sediment - proteinuria, hematuria.

Immunological studies reveal positive markers in ½ cases viral hepatitis B. Rheumatoid and antinuclear factors are in low titer or absent. A high complement titer is characteristic when the skin or kidneys are affected.

Visceral angiography reveals microaneurysms of the affected arteries.

The main clinical and diagnostic criteria for UP include:

1. Persistent fever and weight loss in a patient with signs of systemic pathology.

2. Unexplained ischemic damage to the heart and central nervous system (coronary artery disease, myocardial infarction, cerebral vascular crisis).

3. Clinical signs of acute abdomen (appendicular arteritis, acute perforated intestinal ulcer, multiple intestinal infarctions).

4. Active urinary sediment and/or acute arterial hypertension.

5. Myopathy or neuropathy, hyperesthesia.

6. Skin changes (including purpura, livedo tree, necrosis of the skin and mucous membranes, acute dry gangrene of the fingers, subcutaneous or intradermal nodules).

7. Laboratory data: significant leukocytosis, increase in ESR to 50-70 mm/hour, dysproteinemia, CRP, increased levels of seromucoid, fibrinogen.

Treatment of DBST in children. The goal of treatment for DBST is to achieve and maintain long-term remission of diseases to increase the duration and improve the quality of life of patients.

Basic principles of treatment: 1) an individual approach in choosing treatment methods, taking into account the clinic, the degree of activity and the nature of the course of the disease, the response of the child’s body to treatment; 2) complexity of therapeutic effects; 3) programmaticity (correctness and sequence of implementation of all components of the therapeutic program chosen for treatment); 4) continuity (timely transition from intensive immunosuppressive therapy to supportive therapy, taking into account the stage of the disease); 5) monitoring the effectiveness and safety of the therapy; 6) phasing (stationary and ambulatory treatment); 7) duration and continuity of treatment.

Efficiency is determined by treatment started as early as possible after diagnosis. Since the etiology of DBST has not been definitively established, therapy is based on pathogenetic principles.

Treatment of patients with DBST should be carried out in a hospital, preferably in a specialized department.

At the onset of the disease, bed rest is prescribed, but in the absence of damage to internal organs and serious functional impairment, strict adherence to bed rest is not necessary.

First-line drugs for DBST are glucocorticoids(GCs), which have anti-inflammatory, immunomodulatory and anti-destructive effects. For scleroderma, it is prescribed only according to indications (rapidly progressive diffuse or widespread forms).

GCs are taken taking into account the circadian (physiological rhythm) rhythm of their release in the morning, which reduces the degree of inhibition of the hypothalamic-pituitary-adrenal system. When prescribing a large daily dose of GC, it is divided into 3-4 doses, 2/3 of it is prescribed in the first half of the day.

When choosing an individual dose of GCs, they are guided by the severity of the condition and individual characteristics of the patient, the degree of activity and leading clinical symptoms.

Summary

The work is devoted to current problems of diagnosis and treatment of scleroderma in children. Modern data on the pathogenesis, epidemiology, classification, and nomenclature of the disease are provided. For illustration, we present our own clinical observations authors. Although the prognosis for treatment effectiveness is initial stage It is difficult to manage patients with scleroderma; the effectiveness of treatment is largely determined not only by the early start of treatment, but also by its continuity.

Keywords

Children, scleroderma.

Scleroderma (SD) is a polysyndromic disease manifested by progressive fibrosis of the skin, internal organs and vascular pathology.

Pathogenesis

The pathogenesis of diabetes includes the following main points:

1. Violation of fibroblast function: acceleration of collagen biosynthesis, formation of abnormal collagen fibers.

2. Damage to small vessels: obliteration of small arteries, arterioles, capillaries, disruption of microcirculation, disruption of the structure and function of the affected tissue.

3. Autoimmune changes: formation of autoantibodies to collagen, cell nuclei, vascular endothelium, muscles.

Classification and nomenclature

DS in children has traditionally been classified as juvenile localized sclerosis (JLS) and juvenile systemic sclerosis (JSS). JLS was further classified as focal (morphea), localized or generalized and linear DS, including en coup de sabre lesions on the forehead and Parry-Romberg hemifacial atrophy. JLS has been suggested to be a benign, self-limiting condition with manifestations limited to the skin and/or subcutaneous tissue. However latest research showed that mainly in the localized form there may be arthritis and neurological lesions, not necessarily related to the side of the skin lesions, suggesting a systemic autoimmune process. F. Zulian et al. suggest that within the classification of childhood scleroderma there is a third class - juvenile localized scleroderma with extracardiac manifestations.

Limited scleroderma can be plaque-like or streak-like. It is divided into a focal form (1-5 foci), disseminated (6-30 foci), widespread - confluent (plaques and stripes affect the face, limbs and a significant part of the body) and generalized (without damage to internal organs) (Nikitina, 1980).

Epidemiology

SD - rare disease with a frequency of 0.05 per 100,000 children. The average age of patients is 8 years, 90% of pediatric patients have diffuse lesions. The involvement of internal organs in the pathological process differs from those in adults. Survival rates are higher in pediatric patients than in adults. Most patients who died during the first 5 years had diffuse lesions.

Some authors identify a Borrelia-associated form of plaque focal scleroderma, characterized by the onset of the disease at an early age, infection with B. Burgedorfi and a pronounced autoimmune phenomenon, which is manifested by a high titer of antinuclear antibodies. The disease has severe course and requires treatment of both the infection and skin inflammation.

The question of congenital scleroderma is raised. As stated by Dr. Lawrence Schachner at the University of Miami's annual pediatric conference, Congenital localized scleroderma is a rare diagnosis that may be overlooked in young children. He reported that in a multinational analysis of case histories of 750 children with juvenile linear diabetes, it was found that in 6 patients (0.8%) clinical and serological signs of scleroderma were first detected immediately after birth. The ratio of “girls to boys” was 2: 1. Parents described the skin lesions at birth as erythematous and slightly pigmented and firm to the touch in 2 and simply erythematous in 1. The linear type of lesions was present in all 6 children, and in 4 of them the lesions captured the face in the form of a “saber strike.” For staging correct diagnosis in children with congenital localized diabetes it took an average of almost 4 years (L. Schachner, 2006).

Clinical features of scleroderma in children

Juvenile localized scleroderma is generally considered a disease limited to the skin and subcutaneous tissue. A group of authors (F. Zulian et al.) and the Juvenile Scleroderma Working Group of the European Pediatric Rheumatology Society (PRES) studied the prevalence and clinical symptoms of extracutaneous manifestations in large group children with juvenile localized scleroderma. 750 patients were under observation. In 168 of them (22.4%), 193 extracutaneous lesions were identified, including articular (47.2%), neurological (17.1%), vascular (9.3%), ocular (8.3%) , gastrointestinal (6.2%), respiratory (2.6%), cardiac (1%) and renal (1%). Other autoimmune conditions were present in 7.3% of cases. Neurological lesions included epilepsy, vasculitis, CNS, peripheral neuropathy, vascular abnormalities, headache and perceptual disturbances. Typical manifestations were episcleritis, uveitis, xerophthalmia, glaucoma, and papiledema. More than a quarter of these patients had articular, neurological and ocular lesions, regardless of the location of the skin manifestations. Raynaud's syndrome was observed in 16 patients. Respiratory pathology manifested itself in restrictive lung disease. Gastrointestinal lesions in 12 patients manifested themselves exclusively as gastroesophageal reflux. 30 patients (4%) had multiple extracutaneous manifestations, but systemic sclerosis (JSS) developed in only 1 child. In patients with extracutaneous manifestations, the levels of antinuclear antibodies and rheumatoid factor were significantly higher than in children with only skin lesions. However, Scl1-70 and anticentromere markers of CC were not significantly increased. Laboratory diagnostics include: indicators of the acute phase of inflammation, high level anticentromere antibodies (Scl-70), anti-DNS topoisomers, anti-PmScl, anti-Ul-nRNP, anti-Fibrillarin, anti-RNS I, II, III and others, skin biopsy results. The authors conclude that the subgroup of children with JSS and extradermal lesions, although at very low risk of developing JSS, should be carefully studied, treated more aggressively, and monitored very closely.

Compared with adults, children with an attack of juvenile systemic sclerosis more often have mixed (overlap) syndromes with polymyositis (PM) - dermatomyositis, a higher incidence of skeletal muscle involvement, the presence of anti-PM-DM and anti-U1-RNP antibodies, fatal cardiac pathology and more high survival rate.

A multifactorial analysis of factors influencing survival in diabetes, based on a study of 134 case reports from 40 centers, gave the following results: 16 patients died, 4 within 1 year after diagnosis and 10 within 5 years. Fibrosis on chest x-ray, elevated creatinine levels, and pericarditis were noted as significant predictors of mortality. All patients with a fatal outcome were affected by a diffuse form of the disease and most of them showed rapid progression and early signs of involvement of internal organs in the process. The author concludes that children with diabetes may have two possible courses: the rapid development of internal organ failure, leading to a serious condition and ultimately death, and a slow course of the disease with low mortality.

Treatment of scleroderma in children

The pathogenetic links of various forms of DM are only partially determined, but the main defect in DM is abnormal collagen deposits, which ultimately lead to fibrosis of the skin, as well as internal organs - the heart, lungs - in JSS. Therefore, therapeutic interventions for the treatment of diabetes can be divided into 3 main groups: antifibrotics, anti-inflammatory drugs and vasodilators. For localized forms of the disease, anti-inflammatory drugs, analogues of vitamin D and ultraviolet radiation are under study. However, the infrequency of diabetes in children, plus the fact that the disease very often shows spontaneous remission, makes randomized controlled trials very difficult. For this reason, most data on therapeutic programs for this disease in children have been derived from studies in adults. Moreover, it has been proven that not a single therapeutic method for JLS and JSS was not very effective or significantly changed the course of the disease. However, modern therapeutic strategies must be initiated early in the disease to achieve maximum positive clinical effect.

New treatments for this are being studied complex disease, such as autologous stem cell transplantation and cytokine-correcting therapy.

In order to study the features of the course of diabetes at the present stage and the effectiveness of therapy, we conducted a special study, during which we studied the features of the disease of 3 children who were treated for diabetes in the somatic department of the City Children's Clinical Hospital No. 16 in Kharkov. Of these, 2 girls and 1 boy aged 9-14 years.

U it was established that the first early signs The diseases in all children were focal lesions of the skin and subcutaneous tissues, localized on one side (according to rheovasography). Pathological process accompanied by damage to the gastrointestinal tract (in the form chronic gastroduodenitis, duodenogastric reflux, biliary dyskinesia in 1 child), kidney damage (in the form of dismetabolic nephropathy in all children), heart damage (in the form of dysplastic cardiopathy, vegetative-vascular dysfunction in 2 children), damage to the thyroid gland (in the form of diffuse goiter, euthyroidism in 1 child), changes in the functional adhesiveness of platelets (in the form of thrombocytopathy) in all children studied. In a clinical blood test, an increase in ESR to 20-30 mm/h was noted in 3 children. Rheumatoid factor and antinuclear antibodies were detected in 1 child. All children had changes in cellular and humoral immunity, an increase in the level of CEC, natural and lymphocytotoxic antibodies; a biopsy of depigmented skin areas was performed in 1 child. Treatment was carried out with agents including 5% unithiol solution 0.1 mg/kg IM No. 20-25, lidase 32-64 IU subcutaneously or intramuscularly every other day No. 14, long-term Wobenzym, antiplatelet agents (Trental 10 - 15 mg/kg IV No. 10-12, then peros, or chimes 5-10 mg/kg), ACE inhibitors, anticoagulants, NSAIDs, cardiotropic drugs, drugs that improve tissue metabolism and microcirculation. Positive dynamics in the condition appeared 10-15 days after the start of therapy.

conclusions

X-ray examinations can detect motility disorders of the esophagus and small intestine. A functional study of the lungs, ECG, and radiography can detect damage to the cardiac and respiratory systems. If the kidneys are damaged, changes in urine tests and impaired renal function are noted.

The effectiveness of treatment is largely determined not only by the early start of therapy, but also by its continuity, which is important to consider when managing a child.

Bibliography

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Scleroderma, a pathology of connective tissue, most often manifests itself in girls, and is less often diagnosed in boys. A number of reasons cannot be listed as etiological factors, since they have not yet been established. According to statistics, scleroderma in children ranks 2nd among all connective tissue diseases.

To date, the etiology of the disease has not yet been established. The triggering mechanism is:

• Stressful conditions.
• Hypothermia.
• Endocrine system disorders.
• Lupus erythematosus.
• Infections of a viral or bacterial nature.
• Genetic predisposition.

At focal form noted increased production collagen, responsible for skin elasticity. If there is an excess amount of it, the skin thickens and becomes rough.

Characteristic manifestations

Systemic scleroderma develops extremely rarely in children. Its manifestation is characterized primarily by Raynaud's syndrome and lasts from 2 months to 4 years. Other accompanying signs of pathology include:

• Numbness.
• Loss of body weight.
• Paresthesia of the face, limbs, trunk.
• Causeless fever.
• Stiffness in the area of ​​the hands.
• Contracture of fingers.

Over time, the entire skin is subject to diffuse damage, which provokes subcutaneous calcifications and telangiectasias. First of all, the disease affects the hands and face. After which the pathology spreads to the neck, legs, chest and abdomen. In almost all cases, internal organs are also affected. Esophagitis begins to develop in the esophageal system. Heart disorders are indicated by myocarditis and pericarditis.

Focal scleroderma in children is divided into several types, each of which has its own associated symptoms.

The plaque form at the initial stage of the disease is accompanied by the appearance of erythematous spots of a yellowish-pink color. Subsequently, they transform into lesions, become dense and waxy, and have an ivory color with a purple rim. In most cases, their location is on the legs, arms and torso.

The linear form of the pathology is marked by the same changes as the plaque form. But over time, a linear type configuration stands out noticeably. It is presented in the form of a wide strip located along the neurovascular bundle of a limb.

The appearance of scleroderma in a child is possible in the forehead and scalp. This phenomenon gave rise to the name “saber strike.”

Not only the skin, but also tissues can be affected, which provokes extensive deformations.

Besides that focal scleroderma in children it affects the skin; arthritis, accompanied by limited movement and stiffness, is also possible.

A person with scleroderma feels stiffness in their joints

Therapeutic measures

Therapy for focal disease in children consists of an integrated approach and a long course. The number of courses is at least 6, the break is up to 60 days. If the progression of the disease decreases, the interval between sessions can increase to 4 months.

In case of active disease, the following groups of drugs are prescribed:

• Antihistamines - Tavegil, Pipolfen.
• Vascular - Nicotinic acid, Aescusan, Trental, Madecassol.
• Antibiotics – Oxacillin, Amoxicillin.
• Calcium ion antagonists – Corinfar, Verapamil.
• Agents that suppress excess collagen synthesis are Aloe, Lidaza, Actinohyal.

In the presence of lichen sclerosus, Actovegin, cream containing vitamin E, and Trental are added to the course of treatment.

As local therapy, ointment applications and physiotherapy are used. Prescribed drugs:

• Trypsin.
• Unithiol.
• Troxevasin.
• Heparin and Butadione ointments.

Lidase can be used for phonophoresis or electrophoresis.

• Laser therapy.
• Magnetic therapy
• Vacuum decompression.

Treatment of systemic scleroderma in children involves taking medications such as:

• Vasodilators – Papaverine, Anginin.
• Vitamins A, B, E.
• Antiplatelet agents – Curantil.
• Anti-inflammatory – Indomethacin.
• Immunosuppressants.

A significant role in this type of disease is given to therapeutic exercises, physiotherapy and massage. Such procedures help improve blood supply to tissues and expand movements.

In addition to all the above measures, patients must follow a nutritious diet.

The final stage of treatment for scleroderma in children can be supplemented with radon or hydrogen sulfide baths.

IN Lately some experts are inclined to reduce the amount of medication used. Such drugs can be replaced with agents of broad action, for example, systemic polyenzymes, Wobenzym.

Modern medicine also offers such a procedure as hyperbaric oxygenation. Thanks to this method, the tissue is saturated with oxygen, which activates metabolism in mitochondria, improves blood microcirculation and has an antimicrobial effect.

Scleroderma in children- the second most common disease among diffuse connective tissue diseases in children; it is based on damage to the connective tissue with a predominance of fibrosis and vascular pathology of the type of a peculiar obliterating endarteritis.

This disease in childhood is more often recorded in girls than in boys.

Types of scleroderma

• systemic
• focal

Plaque

Linear

What provokes / Causes of Scleroderma in children:

The cause of the disease remains a mystery to researchers to this day. There can be two mechanisms in the development of the disease: immune and vascular. In the immune system, antibodies to collagen are formed as a result of a cyclical autoimmune process, which also occurs in other diffuse connective tissue diseases in children and adults. If speak about vascular mechanism, altered endothelial cells play a role here. Researchers also say that two mechanisms can develop at once.

Pathogenesis (what happens?) during Scleroderma in children:

With the systemic form of scleroderma in children, collagen and other proteins of the intercellular substance are excessively formed and deposited in the skin and other organs.

Vascular damage

Fibrosis in the systemic form of the disease develops after damage to small arteries, capillaries of the skin, kidneys, gastrointestinal tract, lungs and heart of the child. The blood flow is disrupted, and Raynaud's syndrome occurs, which is considered in most cases the first sign of the disease. After damage to the endothelium and basement membrane, the intima thickens, the lumen of the vessels narrows and becomes overgrown.

The symptoms described above progress, because the number of small vessels becomes less and less. As a result, chronic ischemia of the skin and internal organs occurs. Capillaroscopy of the nail folds reveals a decrease in the number of capillaries, as well as the expansion and tortuosity of the remaining capillaries. Unaffected capillaries grow, and telangiectasia appears.

Endothelial damage

In systemic scleroderma, endothelial damage can be caused by various factors. In some cases, serum contains granzyme A, an enzyme that leads to damage to the basement membrane of blood vessels. In other cases, the serum damages the endothelium.

Vascular spasm

Following this, restoration of blood flow can trigger mechanisms leading to fibrosis and vascular occlusion. Vasoactive substances play a significant role in the development of vascular spasm.

Damage to sensory nerves

This factor also leads to vasospasm, since it causes a lack of vasodilating neuropeptides. In many cases, endothelial damage is accompanied by an increase in the level of the factor coagulation VIII and von Willebrand factor in serum.

Immune disorders in systemic scleroderma

Impaired cellular immunity plays a role in the development of fibrosis in systemic scleroderma in children. The main role belongs to T-helpers. The immunoregulatory index in the blood of a sick child, which is determined by the ratio of CD4 and CD8 lymphocytes, is higher than normal.

Adhesion molecules play a role in pathogenesis, mediating the interaction of T lymphocytes with the endothelium. The level of IR-1 and TNFa is increased in the blood serum, which may indicate activation of monocytes. Along with many other functions, these cytokines have the ability to activate fibroblasts. Skin fibrosis can also occur with the participation of mast cells. Their increased content is found in the dermis, even outside the lesion. One of the reasons for mast cell degranulation may be their interaction with activated T lymphocytes.

With systemic scleroderma in children, there are disorders of not only cellular, but also humoral immunity. The vast majority of patients have antinuclear antibodies in their blood serum.

Fibroblast dysfunction

In the culture of fibroblasts isolated from the affected areas of the skin of patients with systemic scleroderma, excessive collagen synthesis continues. In patients with systemic scleroderma, type VII collagen is located throughout the entire thickness of the dermis, which is not the norm. It is believed that this is the reason for the thickening of the skin and its adhesion to tissues that lie at deeper levels.

Fibroblasts, which are located in the affected and unaffected areas of the child’s skin, carry platelet-derived growth factor receptors, which is also not the norm. More than 90% of patients with systemic scleroderma have chromosomal abnormalities:

• ring chromosomes
• acentric fragments
• chromatid breaks

Pathological anatomy of systemic scleroderma

Leather

At the onset of the disease, the skin has an increased content of monocytes, T-lymphocytes, mast and plasma cells. Further, the epidermis becomes thinner, parallel to it in the dermis there are thickened homogeneous bundles of collagen. They grow into the subcutaneous tissue, which causes adhesion to the underlying tissues. Skin appendages atrophy, interpapillary wedges of the epidermis disappear.

In the gastrointestinal tract, fibrosis is not as pronounced as in the skin. The middle mucosa becomes very thin and lower thirds esophagus, in the mucous layer the amount of collagen is higher than normal. The muscular lining of the esophagus and other parts of the gastrointestinal tract are affected. Due to thinning of the mucous membrane, ulcers occur. In the later stages of scleroderma, dilatation of the affected areas of the gastrointestinal tract occurs.

Lungs

Diffuse interstitial and peribronchial fibrosis appears, proliferation of the bronchial epithelium increases, and the walls of the alvioles thicken. Rupture of the walls of the alveoli can lead to the appearance of small cysts and bullous emphysema.

Musculoskeletal system

Swelling of the synovial membrane is detected if there is arthritis, and it is also infiltrated with lymphocytes and plasma cells. Fibrin is deposited in the thickness of the synovial membrane, on its surface, on tendon sheaths. In later stages of the disease there may be fibrosis of the synovium.

Myopathy

With the systemic form of scleroderma, interstitial and perivascular lymphocytic infiltration, degeneration of muscle fibers and interstitial fibrosis are manifested. There may also be thickening of the walls of arterioles and a decrease in the number of capillaries.

Heart

In the myocardium, degeneration of cardiomyocytes and interstitial fibrosis are recorded, which is most pronounced around the vessels. Fibrosis of the cardiac conduction system causes AV block and arrhythmias.

Kidneys

With systemic scleroderma, more than 50% of sick children are almost affected. Histology shows intimal hyperplasia of the interlobular arteries, fibrinoid necrosis of the glomeruli and afferent arterioles, and thickening of the glomerular basement membrane. There may also be glomerulosclerosis and small infarcts in the renal cortex. If a child with scleroderma has Raynaud's syndrome, then renal blood flow is reduced.

Other organs

In this disease, the liver is affected in rare cases. But primary biliary cirrhosis may develop. Fibrosis of the thyroid gland can occur both against the background of chronic lymphocytic thyroiditis and in its absence.

Symptoms of Scleroderma in children:

Focal scleroderma

For the plaque form, yellowish-pink erythematous plaques appear at the onset of the disease, which then become focal lesions - hard, waxy or ivory-colored, and may have a purple rim. Most often, such lesions are located on the arms, legs, and torso.

For linear scleroderma at the onset of the disease, the same (described above) nature of changes in the skin is typical. But soon a linear configuration appears. it looks like a wide strip, often located along the neurovascular bundle of any of the limbs. Scleroderma can appear on the forehead and scalp in what is called a “saber strike.” With linear scleroderma in children, not only the skin is affected, but also the tissues that are under it, which causes large deformations. If the face and head are affected, the child may experience uveitis and epileptic seizures.

In addition to skin lesions, there may also be arthritis with morning stiffness and limitation of movements, but with them inflammatory changes are not pronounced. In the focal form of scleroderma, Raynaud's syndrome may occur.

Systemic scleroderma

This form of the disease occurs in extremely rare cases in childhood. Raynaud's syndrome always appears first with a duration of 2 months to 3-4 years. Other manifestations include:

• parasthesia of the limbs, torso, face
• numb feeling
• "unreasonable fever", initially low-grade fever
• contracture of fingers
• stiffness in the hands
• joint discomfort
• weight loss

Over time, diffuse damage to all skin integuments develops, in which telangiectasias and subcutaneous calcifications may appear. In most cases, the skin of the face and arms are affected first, and then it affects the neck, abdomen, chest and legs. Internal organs are almost always affected. Esophagitis develops in the esophagus. Changes in the heart are manifested by pericarditis or myocarditis.

Diagnosis of Scleroderma in children:

If a child has skin changes typical for scleroderma, diagnosis is not difficult. The disease should be distinguished from the following syndromes: Buschke syndrome, China syndrome, as well as eosinophilic fasciitis. The diagnosis is aided by the simultaneous development of inductive edema of the neck, face, shoulders with reverse development without skin atrophy.

Treatment of Scleroderma in children:

The main therapy is local application means to improve microcirculation. Doctors may prescribe dimexide, heparin ointment or demixide + heparin to the child. If Raynaud's syndrome is present, doctors can prescribe antiplatelet agents to the patient - aspirin in doses of 10-15 mg/kg body weight per day, chimes, drugs containing nicotinic acid, nifedipine.

If skin changes with scleroderma in children progress, doctors prescribe glucocorticoids in moderate dosage - at the rate of 0.5 mg per 1 kg of body weight per day. D-penicillamine can be prescribed, in a similar dosage as for JCA. Treatment of systemic scleroderma is not complete without the use of corticosteroids at the rate of 0.5-1 mg/kg body weight per day, D-penicillamine. In recent years, methotrexate has been used with increasing success.

Prevention of Scleroderma in children:

Prevention of scleroderma in children is to protect the skin from frostbite, avoid injury to the skin and mucous membranes, and burns. The child should be as nervous as possible. When diagnosing scleroderma in a child, he must be constantly under medical supervision.