Dermatomyositis or Wagner's disease. Dermatomyositis: a difficult test that requires accurate diagnosis and competent treatment Dermatomyositis treatment

• Redness of the skin
• Weakness
• Elevated temperature
• Abdominal pain
• Loss of appetite
• Dyspnea
• Muscle weakness
• Joint pain
• Dry mouth
• Skin rashes
• Dry skin
• Cough
• Peeling skin
• Pain when swallowing
• Muscle pain
• Hoarseness of voice
• Prostration
• Brittle nails
• Redness of the oral mucosa
• Swelling of the oral mucosa

A disease characterized by muscle damage with manifestations of abnormalities in motor functions and the formation of edema and erythema on the skin is called Wagner's disease or dermatomyositis. If there are no skin syndromes, then the disease is called polymyositis.

• Causes
• Juvenile disease
• Symptoms
• In children
• Diagnostics
• Treatment
• Prevention

The disease occurs predominantly in adults aged 40 years, but inflammatory lesions of the muscular system in childhood from 5 to 15 years are not excluded. The disease in childhood is called juvenile dermatomyositis. Often, signs of malaise are found in women and girls, which is due to the physiological structure of the body. The disease is especially often diagnosed during puberty, as a result of which the disease itself is provoked through hormonal development.

Dermatomyositis is a rare disease, but with severe symptoms and a high degree of death. Thus, it is worth having an idea of \u200b\u200bdermatomyositis, its causes, symptoms of manifestation and methods of treatment, which will be discussed in this article.

Depending on the signs of the pathological process, this disease is of two types:

Primary or idiopathic dermatomyositis, which is characterized by signs of independent occurrence not associated with early pathologies.

Secondary or paraneoplastic arises on the basis of the prevailing pathological abnormalities that have arisen as a result of the transferred ailments. Often, it is the secondary species that is found most often.

Depending on the aggravation of the disease, three degrees of complication are distinguished, which are characterized by the corresponding symptoms.

Depending on the signs of dermatomyositis, the following types are distinguished:

• Acutecharacterized by sudden onset;
• Subacute, which is characterized by an aggravation of the acute form, provoked as a result of the lack of appropriate treatment;
• Chronic, as a consequence, arises from the failure to take appropriate measures to get rid of the disease.

Causes

Dermatomyositis refers to a number of diseases, the causes of which remain at an insufficiently understood level. But this does not mean that there are no assumptions. Inflammatory disorders of the muscular system are multifactorial diseases, that is, having various causes. The greatest likelihood of provoking dermatomyositis is due to the predominance of infectious factors. On this occasion, relevant studies were carried out, which proved the reliability of the statement.

A significant role in the development of malaise is played by viral diseases provoked by the ingestion of picornaviruses, parvoviruses, and also the influenza virus. Bacterial pathogens occupy an honorable place among the causes of the formation of inflammatory processes of smooth and skeletal muscles. These pathogens include:

Streptococcus hemolytic group A

• group A streptococci;
• hormonal drugs;
• typhoid and measles vaccines.

The pathogenetic factor causing the disease is also an autoimmune reaction with the formation of autoantibodies. These antibodies predominantly target cytoplasmic proteins and RNA (ribonucleic acids), which form the basis of muscle tissue. Such reactions cause an imbalance between T and B lymphocytes, and also lead to the rejection of T-suppressor function.

In addition to the above reasons, there are a number of trigger (secondary) factors that also tend to form dermatomyositis in humans. These factors include:

• hypothermia of the body;
• overheating;
• hereditary predisposition;
• mental and physical trauma;
• allergic reactions to drugs;
• exacerbation of foci of infections.

Thus, all of the above reasons cause the onset of dermatomyositis, which is characterized by the following periods of course:

Prodnormal - characterized by predominant severity for several days to a month.

Manifested - an advanced stage, which includes the occurrence of muscle, skin and other syndromes.

Dystrophic - the most difficult stage of the disease, due to the onset of general malaise of the body.

Causes of juvenile illness

The causes of childhood dermatomyositis also remain unclear, but they differ in some way from adults. First of all, signs of dermatomyositis occur in children aged 4 to 10-15 years, but the peak of localization of the disease occurs at the age of 7.

Juvenile dermatomyositis occurs as a result of children's exposure to the sun, that is, through the action of radiation rays. Doctors also do not exclude infectious diseases with which the child could be ill from the very beginning of birth. Especially if infectious diseases have become chronic.

The juvenile species is special, since the child's body is not yet ready for such serious tests, as a result of which the result can be fatal if the appropriate measures are not taken.

Symptoms of the disease

The presence of a disease in a person can be determined by the following characteristic features, described below.

The disease is characterized by a gradual course. First of all, the general symptoms are the person's complaints of general weakness. This weakness occurs due to damage to the muscles of the limbs. Symptoms of weakness appear imperceptibly, it may take years, so it is almost impossible to determine the presence of dermatomyositis by such signs.

Another thing is if the ailment has an acute nature of the course, in which case the person experiences, in addition to general weakness, pain in the muscles. The pains are pronounced and appear within 2 weeks. In this case, an increase in temperature is characteristic, which leads to a complete breakdown. In rare cases, the acute form is caused by the appearance of a skin rash and polyarthralgia.

Let us consider in more detail which organs and systems are affected by dermatomyositis with characteristic symptoms.

Muscular system... Since dermatomyositis is a disease of the muscular system, they are the first to suffer accordingly. There is a complete weakness of the body, it becomes difficult for a person to get out of bed, to perform various physical actions. The disease goes so deep that the muscles in the neck are unable to function. Most often, the patient is in a horizontal rather than vertical position. With the localization of the disease, a disorder of the muscle tissues of the esophagus, pharynx, larynx occurs, which is reflected in the form of speech impairment, the appearance of cough and difficulty in eating. When food is swallowed, a sharp cutting pain in the throat occurs. If you examine the oral cavity, you can observe a picture of the appearance: swelling, redness and dryness. Rarely, there is a negative effect on the eye muscles.

Skin ailments... The onset of skin syndrome gives a clear picture of the predominance of dermatomyositis. Among the abnormalities on the skin, the following signs should be highlighted:

• the appearance of a rash on the face in the upper eyelids, nose, nasolabial fold. The rash spreads throughout the body: to the sternum, back, knee and elbow joints. A rash on the upper extremities is especially pronounced;
• coarsening of the palms due to their redness and further peeling of the skin;
• nails become brittle, erythema occurs. Often there is a stratification of toenails and less often on the hands;
• the skin throughout the body becomes dry and red during the course of the disease;
• further picture contributes to the occurrence of atrophy.

Dermatomyositis symptoms

The manifestation of the first pathological abnormalities on the skin should cause embarrassment in the patient and lead him to a dermatologist to determine the disease.

Joints... Rarely, joint pain occurs when flexing / extending the arms and legs. Carpal, elbow, wrist, shoulder and knee joints are also affected. Swelling occurs in the joints, limiting their mobility. Along with pain in the muscles and pain in the joints, there is a general weakness of the body. The formation of joint deformity is possible, which is successfully prevented by taking glucocorticosteroids.

Mucous membranes... There is hyperemia, edema of the palate, stomatitis and conjunctivitis. The back wall of the throat is irritated, which leads to difficulty in swallowing food.

Heart failure... The disease is so serious that it often affects the heart muscle. In this case, such diseases arise:

• myocarditis and myocardiofibrosis;
• tachycardia;
• atrioventricular block of varying degrees of complexity.

In rare cases, pericarditis may occur, which depends rather on the cause that led to the malaise of the muscle tissues.

Lungs... The disease leads to the development of pneumonia of the lungs in a person, which almost always ends in failure. There is also alveolitis, damage to the intercostal muscles, the integrity of the diaphragm is disrupted, aspiration is formed at the time of swallowing. The result is shortness of breath, cough, hoarseness, and dry mouth.

Gastrointestinal tract... The patient's appetite disappears, which is reflected in weight loss, and abdominal pain occurs. Abdominal pain has a dull form of manifestation, which can be observed for a long time. The causes of these pains are inherent in muscle ailments: the pharynx, esophagus and gastrointestinal tract. On x-ray examination, an increase in the size of the liver is observed.

CNS and kidneys... It is observed only in rare cases. Glomerulonephritis can be diagnosed in the kidneys, and polyneuritis in the central nervous system. These diseases are diagnosed exclusively in a hospital. Dermatomyositis contributes to the disruption of the endocrine system and genitals. There is a violation of urination and the development of infertility in girls.

Symptoms in children

Juvenile dermatomyositis symptoms

Juvenile dermatomyositis in children begins with damage to internal organs. The symptoms of the disease differ from adults, first of all, the duration of the formation of the disease. The first characteristic signs of the presence of the disease in children are damage to the skin. All skin syndromes begin in the face and extremities, where red erythema occurs. Mostly on the face of children, erythema occurs around the eyes, which can lead to their swelling and pain when blinking. If the child, even with such symptoms, is not treated, then erythema spreads throughout the body.

Muscle dystrophy and partial lipodystrophy - in frequent cases, they occur in childhood. With muscle damage, a child experiences weakness, fatigue, a lack of desire for active games, etc. Parents on the first signs do not exclude the possibility of infection of the child and try to find the cause.

Important! At the first noticeable symptoms of the disease, it is worth immediately showing the child to the doctor to identify the disease.

Parents notice a complete disappearance of the child's appetite, which is caused as a result of the development of aspiration. When swallowing food, a painful sensation arises, while food can enter the respiratory tract, which is fraught with the development of pneumonia.

Children also often have calcification, which develops in 40% of patients with dermatomyositis. Calcification is the deposition of calcium salts in soft tissues and organs. Salts can be deposited subcutaneously or in the connective tissue in the area of \u200b\u200bmuscle fibers. Their deposition in more traumatic places is not excluded:

• in the area of \u200b\u200bthe joints;
• along the Achilles tendon;
• on the hips;
• on the buttocks and shoulders.

In this case, calcification becomes diffuse, that is, it is caused by the duration of the course.

When the muscles of the diaphragm are damaged, there is a possibility of the formation of respiratory failure, which is reflected, first of all, on the heart muscle. The first symptoms do not indicate the exact disease of dermatomyositis, therefore, diagnostic tests will be required to clarify.

Diagnostics

Diagnosis of dermatomyositis includes collecting data on symptoms, as well as conducting a survey, laboratory and instrumental studies. These studies include:

• X-ray... X-rays determine the presence of calcifications, an increase in the size of the heart muscle and signs of osteoporosis.
• Blood test... The analysis reveals the composition of creatine phosphokinase, aldolase and ESR. By the increased amount of these components, the doctor determines the presence of the disease.
• Electrocardiography... This study allows you to determine the presence of conduction disorders and arrhythmias.
• Spirography... Allows you to identify the presence of respiratory failure.
• Immunological research... A high titer of rheumatoid factor is revealed.
• Muscle biopsy... If all of the above studies do not allow to build a picture of the disease, then a biopsy is the decisive method. It is performed under local anesthesia, and a sample of muscle tissue is collected from the patient using a special device for examination. After the sample is taken, microscopic examination is performed to determine the presence of inflammation.

Computer spirography

After the diagnosis is made, the doctor will make an appropriate decision on the choice of treatment for the disease.

Treatment of the disease

After excluding tumor and infectious diseases, it is necessary to proceed with the direct treatment of dermatomyositis. The main effective drugs in the treatment of this disease are glucocorticosteroids. Moreover, the reception must be carried out in high doses, but always according to the doctor's prescription. One of these drugs is prednisone, which is prescribed depending on the nature of the manifestation of the disease.

Depending on the nature of the disease, the dosage is chosen in the following amount:

• For acute symptoms - 80-100 mg / day;
• In subacute form - 60 mg / day;
• In the chronic form - 30-40 mg / day.

The drug Prednisolone

If the dose was correctly prescribed, then after seven days inhibition of the symptoms of the disease (intoxication) can be observed. After two weeks, the edema disappears, the erythema turns pale and the creatinuria decreases.

The juvenile symptom of the disease in children is also treated with prednisolone, but in different dosages. For children, the dosage of the drug is 10–20 mg / day, and a positive effect is observed after three days.

If the form of the disease was determined incorrectly and the effect of the drug is absent, then a decision is made to gradually increase the dosage. The prescribed dosage is applied for 1.5–2 months, after which the amount of the drug is gradually reduced over 2 years.

In addition to glucocorticosteroids, the likelihood of prescribing by a doctor and cytostatic drugs is not excluded: Methotrexate and Azathioprine.

The drug Methotrexate

Consider in more detail the effect of these drugs.

Methotrexate is started at a dosage of no more than 7.5 mg / week. After that, the dose is gradually increased at the discretion of the doctor by 0.25 mg per week. The drug works effectively, but the first changes can be noticed no earlier than six months later. After the positive dynamics of the effect of the drug is revised and, if available, the dosage is reduced. Treatment can last up to two to three years.

The following persons are not allowed to use methotrexate:

• pregnant women;
• people with kidney and liver diseases;
• people with bone marrow diseases.

Azathioprine is prescribed due to the presence of contraindications to methotrexate. It has a more gentle effect, but less effective. Dosage starts at 2 mg / day and continues until positive changes appear. These changes appear in about 7-8 months, after which it is worth reducing the dosage of the drug.

In addition to the above drugs, fortified agents are also used that have a positive effect on treatment - these are B vitamins, cocarboxylase, ATP and non-steroidal anti-inflammatory drugs.

Prevention

In addition to treatment, a disease called dermatomyositis must be prevented by avoiding hypothermia and timely treatment for infections. It is also not allowed to take medications spontaneously and without prescription, especially unfamiliar ones. It is also necessary to maintain hygiene and cleanliness in the house, especially where there are small children.

What to do?

If you think that you have Dermatomyositis and the symptoms characteristic of this disease, then doctors can help you: a rheumatologist, a dermatologist.

The diagnosis is dermatomyositis, or Wagner's disease is difficult to treat. However, if the disease is detected at the first manifestation of signs, you can completely get rid of it.

Therefore, it is worthwhile to figure out what dermatomyositis is and how it affects the muscles and skin of a person. Let's analyze what the disease is, symptoms and treatment, and also see what the disease looks like in the photo.

Dermatomyositis and polymyositis

Dermatomyositis is a disease in which motor functions are impaired, with severe redness of the skin manifested, caused by the expansion of capillaries. But, if the skin is not affected, then the disease is called polymyositis (systemic muscle inflammation in which the striated muscles of the legs and arms are affected).

The disease can be at a young age, but it is called juvenile dermatomyositis. Together with polymyositis, systemic rheumatic diseases are formed, characterized by muscle inflammation and pain in muscle tissues.

Symptoms and skin manifestations

The treatment of the disease is undulating and growing. Let's analyze the forms of the disease:

Various signs of an inflammatory nature appear on the skin, which manifests itself individually or collectively at various stages. Skin changes in dermatomyositis disease appear earlier in muscle tissues - within a couple of months or even years.

Consider the main symptoms of the disease:

• rashes in the form of papules, blisters;
• swelling of the skin and subcutaneous hemorrhage;
• redness on the eyelids, around the eyes, above the lips, on the cheekbones, in the back, neck, on the side walls of the nose;
• scarlet, pinkish, peeling spots over the joints of the fingers, knees;
• excessive dryness of the skin;
• fragility of the nail plates.

When muscle tissue is disturbed, weakness and low temperature appear. From the first signs of the disease to the manifested symptoms in the bulk of the situation, a period of up to 6 months passes. The doctor describes the increase in violations in the variant of such signs in the patient:

• painful sensations appear in the muscles at rest, as well as during pressure;
• increasing weakness of the muscles of the neck, arms and legs, abdominal zone;
• the patient cannot hold his head;
• abnormal enlargement and hardening of muscles;
• bad speech;
• inability to swallow due to the fact that the patient has a weakening of the muscles of the throat and alimentary tract.

You can also characterize the appearance of the disease by other signs and highlight the following symptoms:

• temperature up to 39 degrees;
• loss of strands;
• breathing is heavy with wheezing;
• oxygen starvation;
• pneumonia;
• fibrosis in the alveolar walls;
• joint pain and weakness;
• diseases of the digestive system;
• protein in the urine due to the destruction of muscle protein compounds.

Causes, treatment, consequences

When it is impossible to determine the cause of Wagner's disease, then this type of doctor refers to a variant of idiopathic dermatomyositis. It can start acutely, but often develops slowly, differing in skin and muscle manifestations:

The formation of the disease is greatly influenced by the presence of an allergen. Sometimes there is a transitional form, between dermatomyositis and connective tissue disease (scleroderma), which indicates susceptibility to allergens and heredity.

The main hazards affecting the onset of the disease can be identified:

• frostbite;
• allergic response to medications;
• hormonal disbalance.

The photo shows the symptoms of dermatomyositis, treatment depends on the specifics of the disease. When signs of muscle damage are expressed, you need to go to a rheumatologist. Also, consultation is needed with such doctors:

• a doctor specializing in the treatment of various infections;
• a doctor who diagnoses and treats benign and malignant neoplasms;
• a doctor specializing in skin conditions.

The disease must be treated with glucocorticosteroids. These substances are very effective in relieving inflammation, so it is best to start taking medications at the first manifestations of the disease.

Of the glucocorticoids, the drug Prednisolone is more often used. It is prescribed for admission for 2 weeks. As a result, over time, speech function returns, skin edema, soreness, and weakness decrease.

Rituximab is used to treat rheumatoid arthritis.

For rashes, your doctor may prescribe hydroxychloroquine.

Diagnostics

Before prescribing drugs, the doctor must be sure of the diagnosis of dermatomyositis, so a diagnosis is necessary. Today there are a lot of devices with which you can conduct a survey. The result of the examinations will determine what treatment the specialist prescribes, especially when it comes to dermatomyositis. Consider the main methods of examination:

Complementary treatments

Often the doctor prescribes other methods, not medication, that are used in the treatment of dermatomyositis. It is important to follow the recommendations of a specialist. Depending on the severity of the signs, doctors prescribe additional therapy options:

• treating dermatomyositis with exercise to improve muscle function and maintain muscle elasticity
• speech therapy. Due to the disease of dermatomyositis, the patient has a weakness of the swallowing muscles.

When the patient's situation improves, you need to visit a nutritionist, because in this case, foods that are easy to digest should prevail in the diet.

Ailment in children

For juvenile (children's) dermatomyositis, symptoms of muscle inflammation, weakness are characteristic, which subsequently leads to a limitation of physical activity. A characteristic feature of the disease in children, which distinguishes it from dermatomyositis of an adult, is the formation of the disease without the appearance of tumors.

The reasons for the appearance in children are considered more often from the side of the influence of infections. There is a judgment that the disease of dermatomyositis at a young age is due to heredity. Irradiation of surfaces with sunlight is of great importance in the formation of the disease. The main signs of childish (juvenile) dermatomyositis include muscle inflammation, muscle weakness, dermatological rashes, skin diseases, diseases of the lungs and intestinal tract.

As a result of the extremely rapid spread of the disease in the child's body, it is children who die more often than adults. Fatalities were recorded during the first years of the disease. Naturally, if you correctly approach the treatment process and follow the doctor's recommendations throughout the disease, drink the necessary medications and improve muscle function in every possible way, then the disease can be overcome. On average, treatment lasts for three years, but in some cases - up to 15 years.

Treatment with folk remedies

Dermatomyositis is difficult to treat, however, with patience, you can achieve results with the use of folk remedies.

It should be understood that plant treatment is used during a period of decreasing signs and pronounced symptoms. Treatment is carried out in the spring and in the fall to prevent the onset of an exacerbation. The course of treatment lasts for a month.

Consider alternative methods of treatment:

1. Treatment as applying compresses and lotions. To prepare the compress, you need ingredients such as willow leaves and buds (1 tablespoon each). All components are filled with water and brewed. After cooling, it can be applied to painful areas of the body.
2. You can also use the following recipe and make lotions: take marshmallow (1 tbsp. L.) And fill it with a glass of boiling water, brew.
3. For the preparation of ointments, you will need willow and butter. After preparation, the medicine can be applied to the affected areas.
4. This composition of ingredients is excellent for dermatomyositis disease: oats (500 g), milk (liter or one and a half). Send the acquired composition over low heat and cook for two minutes. After the tincture has cooled, it must be filtered. The treatment lasts for a month, you can drink up to a glass of broth a day.

Forecast and prevention

Today, thanks to the use of effective drugs, the development of the disease of dermatomyositis is inhibited, and with the supervision of a qualified doctor, improvement occurs quickly.

So, when the doctor has prescribed the exact dosage of the drug, you do not need to reduce the intake of the amount of the drug yourself. It is because of the decrease in dosage that the patient's position is aggravated.

Dermatomyositis of the protracted stage of the disease, despite the therapy, has a high probability of the formation of complications.

The earlier the diagnosis is determined, the treatment is prescribed, the higher the likelihood of a complete recovery of the patient. The child can also end up in absolute recovery or in stable remission.

Measures that would prevent the formation of the disease have not yet been created. However, in clinics, such preventive measures include the following actions:

• supportive drug therapy;
• periodic examinations by doctors, especially by a dermatologist and rheumatologist;
• tests to exclude tumors;
• treatment of inflammatory diseases;
• getting rid of the sources of the infectious process in the body.

Polymyositis and dermatomyositis are rheumatic diseases, the features of which are the appearance of inflammation and the transformation of muscles (polymyositis) or muscles and skin. A more common dermatological sign is heliotropic rash. For any of these symptoms listed above, it is imperative to go to the clinic in order to exclude the appearance of the disease. Below are photos of dermatomyositis in different parts of the body.

The content of the article

Dermatomyositis (synonyms of the disease: Wagner-Unferricht-Hepp disease, poikilomyositis) - severe generalized inflammation of the striated muscles and skin with degenerative changes and scar formation, characterized by impaired motor function and related to collagen diseases.
Dermatomyositis was first isolated as a separate nosological unit in 1887 by Unferricht. The disease is relatively rare (occurs three times less often than systemic lupus erythematosus), affects women twice more often than men. Dermatomyositis is a disease in which muscles (the leading symptom) and skin are affected. Polymyositis is a condition in which skin changes are insignificant or absent, symptoms of muscle damage predominate - myalgia, muscle weakness, deformation and muscle atrophy. Dermatomyositis and polymyositis are clinical variants of the same process.

Dermatomyositis classification

Dermatomyositis is a heterogeneous disease, so its classification is difficult. According to the clinical classification of Bohan and Peter (1975), dermatomyositis is divided into five types.
Type 1. Primary idiopathic polymyositis, the most common form, accounting for 30-60% of myopathies. It begins with progressive weakness in the shoulder girdle. More often women 30-50 years old are ill. Often combined with rheumatoid arthritis and Raynaud's syndrome (30% of patients).
Type 2. The classic type of dermatomyositis, accounts for 40% of forms of dermatomyositis.
Type 3. Polymyositis or dermatomyositis in 20% of cases is combined with malignant neoplasms.
Type 4. In 15% of cases, dermatomyositis affects children aged 5-15 years. Features of this form: early onset of calcification (good prognosis) and muscle weakness; vascular damage by the type of allergic vasculitis (poor prognosis), muscles, skin and gastrointestinal tract. This type of dermatomyositis is represented by two options: the first is children's dermatomyositis of the Bunker-Victor type, the second is Brunsting's type 2.
Type 5. Polymyositis or dermatomyositis in combination with other collagen diseases.

Etiology and pathogenesis of dermatomyositis

The etiology and pathogenesis are not well understood. There is a connection between dermatomyositis and the histocompatibility antigen HLA - B8. A hereditary predisposition of patients with dermatomyositis to autoimmune diseases and allergic diseases was noted: bronchial asthma, diffuse neurodermatitis, urticaria, seasonal rhinitis are detected in relatives. The role of viruses is suspected, but not proven, since with dermatomyositis, virus-like inclusions are found in the nuclei and cytoplasm of myocytes and epidermal cells. There is a point of view that dermatomyositis is a process that occurs as a result of allergy to various antigens - tumor, infectious, etc.
The pathogenesis of dermatomyositis is associated with the formation of immune complexes (type III immunological damage according to Jell and Coombs), which are deposited in the walls of blood vessels, causing immunocomplex vasculitis; the latter is proved by the detection of immunoglobulins and components of the complement system in the vascular walls of the skeletal muscles. This mechanism is especially important for dermatomyositis in childhood. In the pathogenesis of polymyositis, the main role belongs to cytotoxic lymphocytes, which cause necrosis of muscle fibrils. The autoimmune genesis of dermatomyositis is indicated by the systemic nature of the lesions, the presence of lymphocellular infiltration, immunocomplex vasculitis, hypergammaglobulinemia, autoantibodies, circulating and fixed IR, cytotoxic activity of lymphocytes, the relationship of dermatomyositis with other autoimmune diseases, etc.
Pathomorphology. In the muscles of patients with dermatomyositis, necrosis, phagocytosis and regeneration, atrophy and degeneration of muscle fibrils, vacuolization, perivascular infiltrates from mononuclear cells develop. In the dermis and epidermis, atrophy of the epidermis, degeneration of its basal layer, edema of the upper layer of the dermis, inflammatory infiltrates and fibrinoid deposits are found, in the subcutaneous tissue - panniculitis and mucoid degeneration of cells. Visceral pathology is manifested by vasculitis and mild inflammatory-sclerosing processes in the stroma.

Dermatomyositis clinic

There are two age peaks in the incidence of dermatomyositis: the first in children at the age of 5-15, the second in adults at the age of 50-60. Depending on the form of the disease, certain characteristic features prevail, the main ones are the pathology of the skin and striated muscles. The disease begins most often gradually - with mild weakness, moderate myalgia and arthralgia, changes in the skin and localized edema, less often acute febrile temperature (38-39 ° C), diffuse erythema and muscle pain. Common symptoms: muscle pain, weakness, fatigue, anorexia, emaciation, temperature reaction.
Skin lesions are characterized by symptoms pathognomonic for dermatomyositis: periorbital edema with heliotrope (bluish-purple
coloring); Gottron papules, which are scaly purple-red lesions of the skin located on the extensor surfaces; swelling of the face; diffuse erythema; atrophic poikiloderma; vesicles and blisters; calcification of the skin; telangiectasia; hyperkeratosis of the nail bed; hives; hypertrichosis; itchy skin; alopecia; photodermatitis.
Muscle lesions are characterized by muscle weakness and pain. The muscles of the neck and pharynx are the first to be involved in the process, later - the shoulder and pelvic girdles, which creates a picture of muscle weakness typical for dermatomyositis - falling when walking, inability to tear off the head from the pillow, combing your hair, raising your leg up a step, etc. creates some mask-like face - "alabaster face". The involvement of the pharyngeal muscles in the process causes dysphagia, and the intercostal and diaphragmatic muscles contribute to the development of pneumonia. Calcification often leads to restriction of movement and is a sign of the transition of the disease into a chronic form.
Joint lesions are characterized by arthralgias, less often arthritis, their dysfunctions are mainly associated with muscle pathology.
Visceral changes mainly depend on muscle damage: cardiac (focal and diffuse myocarditis, cardiomyopathy), respiratory muscles (aspiration pneumonia), pharyngeal ring (increasing dysphagia). Dr. symptoms of visceral pathology are vasculitis: lungs (allergic pulmonary vasculitis), digestive tract (gastrointestinal bleeding, stomach perforation), which are especially often observed in infantile dermatomyositis of the Bunker-Victor type.
In the acute course of dermatomyositis, death can occur a year after the onset of the disease, with chronic remissions long. Poor prognosis in children under two years of age with acute onset of the disease, severe muscle weakness, rapid progression of the process with ESR increased to 80 mm / h. Some patients experience spontaneous remissions. The prognosis for work capacity is poor.

Diagnosis of dermatomyositis

There are five main diagnostic criteria for dermatomyositis: symmetric and progressive weakness of skeletal muscles (the process may involve the respiratory and swallowing muscles); typical histological picture in muscle biopsy (necrosis of muscle bundles with phagocytosis, regeneration with basophilia and inflammatory exudation); increased levels of creatine phosphokinase and aldolase in muscle tissue; violation of electromyogram; characteristic skin lesions (periorbital edema with heliotrope and Gottron papules). To make a diagnosis of dermatomyositis, four criteria are required, polymyositis - three.

Differential diagnosis of dermatomyositis

Dermatomyositis should be differentiated from collagen diseases, infectious (mononucleosis, trichinosis, brucellosis, typhoid fever), dermatological (neurodermatitis, photodermatoses, t oxermia) and neuroinfectious diseases, sarcoidosis, endocrinopathies, and endocrine diseases.

Dermatomyositis treatment

For the treatment of dermatomyositis, large doses of glucocorticosteroid drugs are used, preferably methylprednisolone, which causes muscle weakness to a lesser extent; triamcinolone, which enhances myopathy, is undesirable. The average dose of hormones is 60-80 mg of prednisolone or 48-64 mg of methylprednisolone daily for a long time (two to three months) until the onset of a therapeutic effect. Doses of glucocorticosteroid drugs should be adequate to the acuteness of the process: in acute course - 80-100 mg of prednisolone, with subacute - 60, with exacerbation of chronic - 30-40 mg per day. After the onset of the therapeutic effect, the dose is reduced to a maintenance dose - in acute and subacute course of 30-40 mg in the first year and 20-10 - in the second and third. If the effect after 3-4 months of treatment is insufficient, immunosuppressants are prescribed - cyclophosphamide, azathioprine - at a dose of 2 mg per 1 kg of body weight. Non-steroidal anti-inflammatory drugs, aminoquinoline derivatives can be used in the treatment of dermatomyositis, the latter for many years. In the acute course of dermatomyositis, bed rest is indicated, in the future - exercise therapy, gymnastics, massage, physiotherapy, spa treatment.

Prevention of dermatomyositis

Prevention of the disease consists in early diagnosis, timely and active therapy in a hospital setting, dispensary observation and adequate supportive therapy. It is necessary to exclude allergenic factors that can exacerbate the process.

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Before the era of corticosteroids prognosis dermatomyositis (DM)was considered unfavorable, fatal in almost 2/3 of patients. With the use of corticosteroid drugs, the prognosis of the disease improved significantly, although the opinions of scientists about the effectiveness of treatment were divided. A number of authors, evaluating positively corticosteroids in DM, note only a moderate improvement in prognosis, but most emphasize the high effectiveness of this type of therapy.

When studying the survival rate of 144 long-term patients with DM, the 5 and 10-year survival rates of patients were 73 and 66%, respectively. The prognostic value of the age of the patients has been established: the most favorable prognosis is in persons with illnesses under the age of 20, the lowest survival rate is noted in the older age groups.

If the levels of 5 and 10-year survival in patients of the first group were 100%, then in patients over 50 they were 57 and 38%. Deterioration of the prognosis of dermatomyositis in the elderly is also noted by other authors. So, in the observations of M. Hochberg et al. (1983) the 8-year survival rate of patients with DM (polymyositis) was 56.7% in people over 45 years old and 96.6% in the group of patients under 45 years old. It is quite obvious that the worsening prognosis in older age groups is due to an increase in the number of patients with tumor DM.

Comparison of 5 and 10-year survival of patients with idiopathic (89 and 81%) and tumor (15 and 11%) dermatomyositis clearly illustrates the poor prognosis of the latter. In addition, the often more severe course of DM, often complicated by the development of pneumonia, in the elderly should be taken into account.

There were no significant differences in the survival rate of patients with DM (polymyositis) depending on gender.

The nature of the course of the disease plays a significant role in determining the prognosis, which is also well illustrated by the survival rates. So, according to M.A.Zhanuzakov (1987), the 5 and 10-year survival rate of patients with chronic dermatomyositis remained at the level of 100%, and in acute and subacute course it was 71 and 63%, respectively.

Flow

In active forms of DM, of course, the prognosis is also determined by the duration of the disease (before the start of adequate therapy), the severity of muscle and visceral manifestations. So, in the presence of immobility, the 5 and 10-year survival rates were 77 and 69%, and while maintaining the range of movements required for self-service, it was equal to 95 and 88%. In the presence of dysphagia, the same indicators were 76 and 70%, and in patients without dysphagia - 97 and 88%. The addition of pneumonia is even more prognostically unfavorable: in the group of DM patients with pneumonia, 5 and 10-year survival rates decreased to 66 and 32% compared to 93 and 89% in the absence of pneumonia.

Treatment

An important factor that improved the prognosis of patients with acute and subacute idiopathic dermatomyositis should be considered timely and adequate treatment, primarily with sufficiently high doses of corticosteroids (at least 1 mg / kg of body weight). Such treatment led to the preservation of 5 and 10-year survival rates at the level of 96 and 90%, while in patients who did not receive adequate therapy (insufficient doses and / or the duration of treatment), these indicators were 70 and 56%.

In tumor DM, surgical intervention combined with corticosteroid treatment is decisive. This tactic contributed to the preservation of the survival rate after 5 and 10 years in this category of patients at the level of 32 and 27%.

Of the 209 patients with DM observed by E.M. Tareev and A.P. Solovieva (1985) for 25 years, there were 162 patients with idiopathic dermatomyositis (I group) and 40 patients with tumor DM (group II). Most of the patients in group I received adequate drug therapy, including corticosteroids, which led to a relatively favorable prognosis.

Out of 162 patients with idiopathic DM, 17 died (10.5%), and in 5 of them the cause of death was not directly related to the underlying disease (myocardial infarction, complications of influenza, etc.), in 8 it was due to complications of corticosteroid therapy (gastrointestinal bleeding , pancreatic necrosis, infection).

In group II 36 (40 patients with paraneoplastic dermatomyositis) died; in 4, timely removal of the tumor led to a cure. In some of the operated patients, relapses or neoplasia of other localization occurred, which was accompanied by the activation and growth of DM signs, although during the period of severe tumor intoxication, DM symptoms often clearly decreased.

In retrospective observations, J. Benbassat et al. (1985) 94 patients with dermatomyositis (polymyositis) for the purpose of analyzing the prognostic factors of the disease, the mortality rate was 32.6%, and it was also the highest in the group of patients with tumor DM (polymyositis). The most common causes of death were malignant tumors, pulmonary complications, and ischemic heart disease. The highest mortality was observed during the first year from the moment of diagnosis.

Prognostically unfavorable factors include:uncontrolled activity of the process and the inability to achieve remission of the disease, old age, as well as such clinical and laboratory signs as skin rashes, dysphagia, fever above 38 ° C and leukocytosis. Sex, the presence of arthritis or arthralgias, Raynaud's syndrome, ECG changes, histological changes in muscle biopsy, an increase in the level of muscle enzymes in the blood serum, an increase in ESR, changes in electromyogram, hemoglobin level, and the presence of antinuclear antibodies did not affect survival.

Thus, summarizing our own observations and literature data, we can conclude that the causes of death in patients with idiopathic DM (polymyositis) are often complications of the disease (most often hypostatic and aspiration pneumonia) or treatment, changes in the general condition (cachexia, dystrophy) or internal organs ( heart with the development of heart failure, etc.). Often, death is associated with the addition of a concomitant disease (infection, etc.) against the background of the general serious condition of the patient.

When paraneoplastic dermatomyositis (polymyositis), the cause of death is usually a malignant tumor, although other complications should be considered. As an illustration, we present the data of A.P. Solovieva (1980), who analyzed the causes of death of 23 patients with tumor and 14 idiopathic DM.

In general, the prognosis of the disease has improved significantly, due to more timely diagnosis and active treatment of patients. Outcomes of DM in 130 patients, followed by A.P. Solovieva (1980), are presented in table. 6.3.

Naturally, the term "recovery" is used to a certain extent conditionally, since patients, even having returned to an active lifestyle, require further (at least once a year) observation and employment with the exception of physical activity, night shifts, business trips, chemical and temperature effects, any allergenic factors, etc. e. Similarly, all unfavorable factors should be eliminated in all patients with dermatomyositis, which is a kind of prevention of exacerbation of the disease.

In case of acute and subacute course, patients are transferred to I or II group of disability, and only after a year or more, when a stable effect is achieved, the question of resuming studies or work (with the above restrictions) can be discussed. In the chronic course of DM (PM), it is possible to maintain labor activity, subject to medical supervision and the necessary medical procedures.

Table 6.3. Outcomes of dermatomyositis in 100 patients with idiopathic and 30 patients with tumor forms

Exodus	Flow		Flow	Total
	idiopathic		tumor
	dermatomyositis		dermatomyositis
	spicy	chron-	spicy	Abs.	O / / O
	and subacute	rationally	and subacute	Number
Recovery, remission	14	6	3	41
Significant improvement	48
	16	-	-	161
Improvement	2	-	4	61
Death	14	-	23	3728,4

Sigidin Ya.A., Guseva N.G., Ivanova M.M.

Dermatomyositis is an inflammatory diffuse disease of connective tissues, smooth and skeletal muscle fibers, skin and internal organs. If there is no skin symptom, they speak of polymyositis. Dermatomyositis manifests itself in the following symptoms: muscle weakness, fever, migraines, polyarthralgia. The diagnosis of pathology is based on biochemical and laboratory results. An effective way to alleviate or eliminate problems is hormonal, the course of the disease is wave-like.

There is a connection between dermatomyositis with viral infection and genetic factors. The chronic survival of microbes in muscle tissues, the similarity of the viral and muscle systems leads to the emergence of autoantibodies to the muscles. The starting point for the formation of dermatomyositis can be an exacerbation of an infectious virus, severe depression, hypothermia, allergy, vaccination, hyperthermia.

In the article, we will consider the following: dermatomyositis: symptoms and treatment of this pathology, diagnosis and causes of its development.

Dermatomyositis is a systemic intensifying pathology, due to which muscle tissues and skin are affected in the first place, the functioning of organs is disrupted, which can be accompanied by a purulent infection. In a quarter of patients, skin diseases are not observed. In this case, polymyositis is meant. Dermatomyositis belongs to the class of inflammatory neuromuscular diseases. Secondary tumor pathology is observed in 25% of cases. The disease can be acute, subacute, or chronic.

The development of dermatomyositis is accompanied by a prodromal stage, clinical symptoms and an exacerbation period. The disease can pass with different manifestations of inflammatory activity (from 1 to 3).

Causes of pathology

To date, the reason is undisclosed. It is believed that the main prerequisite for the appearance of pathology is viral infection, but not a single clinical and laboratory study has yet been able to prove this with 100% probability. An important component is the autoimmune process. 15% of the considered myopathies are associated with oncological pathologies, in which we are most often talking about dermatomyositis.

The disease begins to manifest itself as mild weakness, malaise, fever, loss of weight and appetite, skin disease with a subsequent increase in the ailment in the hips and shoulders. Dermatomyositis can proceed slowly, over several months, or immediately take an acute form, which most often occurs in the younger generation.

Risk factors

Dermatomyositis most often overtakes womenthan men.

Table. Distribution of 150 patients with dermatomyositis (polymyositis) by clinical group and gender.

Most women get sick in age from 40 to 60 years old, children - at the age of 5-15.

Most often people suffer job which is associated with the toxic effects of harmful substances.

The disease is accompanied by acute or chronic viral and bacterial infections.

Endocrine diseases. Malfunction of the thyroid gland.

Hormonal disruption, menopause, pregnancy.

Permanent being under the scorching sun or, on the contrary, a long stay in the cold.

Symptoms of pathology

The clinic of the disease has a gradual development. At the onset of dermatomyositis, you can find increasing muscle tissue weakness, which can worsen over several years. Acute development is not particularly characteristic of dermatomyositis. The main symptoms may be accompanied by skin irritation, Raynaud's syndrome.

There is weakness in the neck, arms, which can interfere with the performance of daily work. With more advanced forms of the disease, it is difficult for patients to get out of bed, to keep their head suspended, and it is difficult to walk on their own.

The defeat of the muscles of the throat, upper digestive canals leads to indistinct speech, difficulty in swallowing and passing food. Violation of the diaphragm and intercostal tissues leads to malfunctions of the lungs and the development of pneumonia. A characteristic feature of dermatomyositis is the infection of the skin. There is swelling of the face, a rash on the cheeks, in the area of \u200b\u200bthe nasolabial folds, chest, shoulder blades.

The most often observed symptom of Gottron, which is manifested by peeling of the skin on the hands, red spots on the palms, brittle and exfoliating nail plates. A natural symptom of dermatomyositis is a change in pigmentation and depigmentation on the skin, accompanied by dryness, atrophy and hyperkeratosis.

The processes of stomatitis and conjunctivitis occur in the mucous membrane, swelling manifests itself. Occasionally, a disorder of the joints of the shoulders, elbows, hands, knees and ankles can be found. Juvenile dermatomyositis is accompanied by intramuscular and intradermal calcification in the shoulders, pelvis, and buttocks. Calcifications provoke the formation of ulcers on the skin and the release of calcium deposits.

Systemic symptoms of pathology affect work:

• heart, leading to myocarditis and myocardiofibrosis;
• lungs, which causes pneumosclerosis and pneumonia:
• Gastrointestinal tract, provoking hepatomegaly and dysphagia;
• nervous system;
• endocrine glands, which provokes disruption of the gonads and adrenal glands;
• kidney, causing glomerulonephritis.

Dermatomyositis begins to manifest itself with the usual, at first glance, frivolous problems, such as: weakness, loss of appetite and weight, fever, the appearance of a minor skin rash. Gradually, the disease begins to progress and acquire more serious forms of manifestation. Pathology can proceed slowly and drag on for several months or take on an acute form, which is usually found in people in their youth.

Among the symptoms are the following.

1. Subcutaneous calcification - in most cases occurs in children.
2. Antisynthetase syndrome. Manifested by acute fever, lung disease, and arthritis symmetry. As a rule, with such a symptom, antibodies to Jo-1 are detected in the blood. Pathology is actively manifested in the spring.

Table. Variants of skin lesions in dermatomyositis.

Skin lesion options	Description
Periorbital edema (heliotrope rash)	A purple or dark blue rash around the eyes.
Erythematous photosensitive rash	Erythema of the face in the knees, elbows, neck and décolleté.
Gottron papules	Dense erythematous rash, often localized over the metacarpophalangeal and proximal interphalangeal joints, over the flexor surfaces of the elbow and knee joints.
"Mechanic's hand"	Peeling, painful cracks in the skin of the hands.
Capillaries of the nail bed	Expanded and convoluted capillaries of the nail bed.
Poikiloderma	Hyperpigmentation and atrophy with the presence of spider veins, peeling, which determines the variegated nature of the affected area of \u200b\u200bthe skin.

Diagnosis of dermatomyositis

• laboratory factors;
• muscle biopsy.

Polymyositis should be suspected when a patient is found to have proximal muscle weakness, manifestation without based pain in the muscles. The accuracy of diagnosing a disease becomes better if the following problems can be confirmed:

• proximal muscle weakness;
• skin rash;
• excessive work of muscle enzymes. In the absence of an excessive amount of creatine kinase, it is worth testing for an increase in aldolase or aminotransferase, which are not so specific compared to creatine kinase;
• abnormalities in muscle tissue, which can be detected by doing MRI and electromyography;
• abnormalities found on muscle biopsy.

A muscle biopsy will help eliminate clinically similar symptoms, including myositis and rhabdomyolysis, which are caused by a viral infection. The existing disorders detected during histological examination may be of a different nature, but they are common: places of degeneration and regeneration of muscle tissues, inflammatory reactions.
Before starting treatment, it is worth making sure that the diagnosis is accurate. This can be done using histological verification. It is carried out in order to exclude even the slightest likelihood of the presence of other muscle pathologies. To increase the awareness of histological examination, a biopsy should be taken from a muscle corresponding to as many signs as possible:

• weakness at the time of clinical trials;
• changes obtained with electromyography;
• detection of inflammatory reactions after MRI.

With the help of clinical studies, one can be convinced, or, on the contrary, refute the likelihood of the presence of a disease, assess the degree of its complication, and trace cross-violations. It is worth identifying autoantibodies. The probability of detecting autoantibodies is 75%. A more complete study of antinuclear antibodies is very important in detecting cross-complications that are characteristic of other autoimmune pathologies. Myositis-specific autoantibodies are present in approximately 25% of patients. It is still not known what connection is present between antibodies and pathogenesis.

Note! Temporary observation of creatine kinase activity provides good results for disease monitoring. In acute muscle atrophy, the activity of the enzyme can remain normal, regardless of the activity of myositis. The information obtained during the MRI, the overestimated values \u200b\u200bof the work of creatine kinase and muscle biopsy help determine myopathy and polymyositis.

Doctors often insist on cancer screening, since dermatomyositis can be observed in malignant tumors

Some public health authorities strongly recommend screening for patients aged 40 or over with dermatomyositis and patients over 60 years of age with polymyositis. As a rule, in people of this age group with existing diseases, malignant tumors are very often found.

Screening refers to a physical examination that includes:

• examination of the pelvis, mammary glands and rectum;
• laboratory and biochemical blood test;
• mammography;
• analysis of urine;
• chest x-ray;
• other studies.

Important! The need for additional examination is assigned based on the history and physical data. Sometimes a CT scan of the pelvis and thoracic region is prescribed. Young patients who do not have clinical symptoms of malignant tumors do not need screening.

Possible complications

1. Difficulty swallowing. The defeat of the gastric muscles.
2. Disease of the respiratory tract, the appearance of shortness of breath due to damage to the pectoral muscles, which in the worst case can lead to respiratory arrest.
3. Aspiration pneumonia. Due to the fact that the swallowing process is disturbed, what is contained in the stomach can enter the respiratory organs on inhalation, which will provoke the appearance of pneumonia.
4. Calcium deposits. Calcium can be deposited in the skin, muscle and connective tissues.
5. Cardiovascular diseases. Myocarditis, arrhythmia and heart failure.
6. Vascular diseases. White skin on the fingers and nose during cold periods is indicative of Raynaud's syndrome.
7. Kidney problem. The appearance of renal failure.
8. High probability of oncopathology.

Treatment of the disease

In many cases, immunosuppressants are prescribed. Motor processes should be moderate until the inflammatory process slows down. In the initial stages of treatment, it is recommended to use glucocorticoids. Adults with acute illness are prescribed 50 mg of prednisone by mouth once a day. Continuous monitoring of the activity of creatine kinase is considered an effective indicator of treatment: in a larger number of patients, after 2-3 months, its decrease or the achievement of permissible norms is observed with a further increase in muscle strength.

When the enzyme activity has stabilized, the amount of prednisone used is reduced. If the enzyme action is increased, the dose is increased again. If complete recovery is achieved, the agent is gradually withdrawn from the patient's regimen under the strict supervision of his further condition. But in most cases, adult patients require long-term treatment with prednisone. For children, a dose of 40-50 mg per day is intended. Discontinuation of the drug by children is allowed after remission.

There are cases when in patients who have been using a large dosage of glucocorticoid for a long time, weakness increases as a result of the appearance of steroid myopathy.

In case of an inappropriate reaction to therapy with this drug and when hormonal myopathy or other problems appear, it is necessary to reduce the amount of the drug used or abandon prednisolone. In this case, treatment with immunosuppressants is worth considering. Many specialists prescribe combination therapy in the initial stages of therapy.
Some patients take methotrexate alone for 5 years or more. Intravenous drug therapy can be beneficial for patients who do not accept medication. But their considerable price does not make it possible to organize comparative studies. Myositis, similar to a malignant tumor, is most often distinguished by a greater refractoriness in relation to corticosteroids. Myositis, similar to a tumor, may subside if removed.

Important! Patients with an autoimmune disease are more prone to atherosclerosis, so they should be monitored regularly. Continuous and long-term treatment requires prevention of osteoporosis from patients.

Necessary patient care

1. The sleeping place of a person with dermatomyositis should be comfortable, soft and keep warm. The affected joints should be protected from possible microtrauma.
2. Apply a warm compress to the joints as directed by a doctor.
3. It is necessary to constantly monitor the intake of medications by patients, remind them of the need to take them, and monitor compliance with the dosage. Track possible deviations from the usual state and emerging complications during treatment.
4. Patient hygiene control. If necessary, provide assistance when changing bed and personal linen, taking a bath. If there are pains in the joints and muscles, excessive weakness, the patient is unable to perform these simple actions alone.
5. The patient's diet should contain essential vitamins, especially vitamins B, C and D, and not include a lot of fatty acids. It is worth reducing the use of table salt. The food should be high in calories and well absorbed by the stomach, the diet should be balanced.
6. Regular performance of remedial gymnastics. Physical exercises should be aimed at working with the affected muscles and joints, individual muscle groups. If dermatomyositis is detected, it is worth performing special gymnastic exercises for the muscles of the face. During the exercise, active and passive loads are exerted on all joints. Carrying out of medical procedures should take place at the time of weak activity of immuno-inflammatory processes with full observation of the patient's condition.
7. Given that treatment takes a long time, there is a likelihood of various complications from the therapeutic agents used (especially when using a cytostatic and a glucocorticosteroid). It is worth paying special attention to constant conversations with the patient, during which it is worth conveying the necessary meaning of the therapy being carried out, convincing the patient to show more tolerance and restraint, directing to positive thoughts and charging with good emotions. It is equally important to talk to the patient's family. It is worth bringing them up to date and highlighting the essence and nuances of the disease, the established course of treatment, familiarizing them with possible complications and convincing them to be tolerant when providing the patient with the necessary help and support.
8. It is worth helping the patient with the optimal organization of his day, thinking over his working hours and rest periods. It is better to refuse night work, minimize physical activity and protect from emotional outbursts.

Prevention and prognosis of dermatomyositis

In severe and advanced forms of the disease, the risk of death in the first couple of years of the development of pathology is close to 30-40% of all cases, especially as a result of respiratory tract disease and stomach hemorrhage. With all the severity of the disease, contractures appear and the limbs are gradually taken away, which in the future ends with the patient becoming disabled. Timely assistance and the establishment of a therapeutic course will help prevent the development of the disease and improve further prognosis.

Non-specific methods for the prevention of dermatomyositis include: observation by a rheumatologist, monitoring of drug intake.

Patients should avoid reasons that provoke an exacerbation of the disease, namely: prolonged exposure to the sun and cold, colds, abortions, depression, constant contact with chemicals, taking medications that are allergic.

Women should check their pregnancy with a rheumatologist.

An important role is played by the timely detection and treatment of malignant tumors.

Summing up

Dermatomyositis is an extremely unpleasant disease, which is not very easy to get rid of. However, the sooner the patient seeks help and begins therapy, the easier this illness will pass for him and the faster he will be able to live a full life again.