The disease dermatomyositis is a systemic pathology that primarily affects muscle tissue and skin. The term “myositis” literally means the presence of an inflammatory process in the muscles, in this case of an autoimmune nature. Inflammation leads to the fact that muscle tissue dies, being replaced by connective tissue, and becomes unable to perform its previous functions.

Localization and prevalence

The disease dermatomyositis is most common in southern European countries. The greatest number of cases is observed in spring and summer, which may indirectly indicate the pathogenic influence of solar radiation. Women get sick more often than men. Pathology usually manifests itself at a young (15-25 years) or elderly (over 60 years) age. The incidence rate of dermatomyositis in children is 1.4-2.7:100,000, in adults 2-6.2:100,000.

Causes of dermatomyositis

At the moment, the causes of the disease are not exactly clear. Scientists suggest that factors such as:

1. Increased insolation.
2. Recent infectious diseases.
3. Hypothermia.
4. Pregnancy.
5. Use of medications.
6. Viruses.
7. Vaccinations.
8. Malignant neoplasms.

Symptoms of dermatomyositis

Dermatomyositis disease symptoms

The first manifestation of the disease is usually weakness in the neck, upper and lower extremities. In this case, the muscles of the hands and feet are most often not affected, so it is easy for patients to stand on tiptoes, but it is difficult to climb stairs. Patients report discomfort similar to pain after physical activity. Long rest and gentle treatment do not lead to improvements. If left untreated, death occurs due to damage to the diaphragm and respiratory arrest.

A very characteristic skin manifestation of dermatomyositis is the symptom of “purple glasses” - redness and swelling of the skin on the upper eyelids. The remaining manifestations are diverse and nonspecific: patients experience erythematous areas of the skin, rashes in the form of blisters, itching, and papules. Open areas of the body are usually affected.

Juvenile dermatomyositis often occurs acutely or subacutely, which is an unfavorable sign. However, with the right therapy, dermatomyositis can be converted into a chronic form or cured.

Diagnosis of dermatomyositis

The diagnosis is made based on the patient’s complaints, medical history (relationship with a recent viral infection, hypothermia or other risk factor), and laboratory test results. In the blood of patients there is an increase in leukocytes, eosinophilia, and sometimes an acceleration of ESR. In a biochemical blood test, the number of enzymes increases:

• aldolases;
• lactate dehydrogenase;
• creatine phosphokinase;
• aspartate transferase;
• alanine aminotransferase.

Urinalysis shows an increase in creatinine.

Patients with suspected systemic dermatomyositis are prescribed the following studies:

1. Electroneuromyography (ENMG). Needed to determine the cause of weakness. In some cases, it occurs against the background of damage to nervous rather than muscle tissue.
2. Computed tomography (CT) of the hip. Allows you to visually assess the condition of the patient’s muscles: if there is inflammation, their increase due to swelling will be visible. If possible, a CT scan or chest x-ray should be done to identify lung involvement.
3. Muscle biopsy. It is a reference research method. Under a microscope, the doctor will see confirmation of the autoimmune cause of inflammation.
4. Differential diagnosis with oncomyositis. The disease may not be primary (idiopathic) dermatomyositis, but concomitant in the presence of malignant neoplasms, so the doctor conducts a full examination of the patient to exclude oncopathology.

Treatment of dermatomyositis

Treatment is aimed at stopping inflammation and preventing the degeneration of muscle tissue into connective tissue. The following medications are used for this:

1. High doses of glucocorticosteroids (prednisolone, dexamethasone) for a long time (2-3 months). The dosage is reduced gradually, to one tablet per week. It is possible to completely abandon steroid hormones only in case of stable remission.
2. Cytostatics. Prescribed in case of ineffectiveness of glucocorticosteroids.
3. B vitamins, ATP, proserin and cocarboxylase help restore the functional activity of muscles.
4. Plasmapheresis is a procedure that helps remove immune complexes from the blood that damage tissue.

When treating dermatomyositis, it is important to follow a regimen (avoid overheating and hypothermia, minimize physical activity) and diet (limit sweets when using steroid hormones). The doctor prescribes physical therapy to prevent the development of contractures.

Treatment of dermatomyositis with folk remedies

It is possible to treat dermatomyositis with folk remedies.

• Compresses:

1. Pour 1 tablespoon of willow buds and leaves into 100 ml of hot water, let it brew for 1 hour. Apply to affected skin.
2. Pour 1 tablespoon of marshmallow into 100 ml of hot water and let it brew for 1 hour. Apply to affected skin.

• Ointments:

1. Mix 1 part willow buds with 1 part butter until a homogenized mass is formed. Rub into affected skin.
2. Melt the fat in a water bath and mix with tarragon seeds in a 1:1 ratio. Place the mixture in the oven for 6 hours at 160° C. Cool. Rub into affected skin.

• Medicinal mixtures with anti-inflammatory properties: St. John's wort, sage, linden, calendula, chamomile. Brew and drink instead of tea.
• Mumiyo, 2 tablets on an empty stomach in the morning for 1 month.

Prognosis and complications

The prognosis is satisfactory. In the absence of therapy, death occurs within the first two years due to damage to the respiratory muscles. The severe course of the disease is complicated by contractures and deformities of the limbs, leading to disability.

Prevention

Specific prevention has not been developed. Primary prevention includes avoiding risk factors for developing the disease and general hardening of the body. When symptoms are identified, prevention comes down to preventing relapses and complications.

Photo

Disease dermatomyositis skin manifestations photo

Contents of the article

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

Classification of dermatomyositis

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. Begins with progressive weakness in the shoulder girdle. Women aged 30-50 years are most often affected. Often combined with rheumatoid arthritis and Raynaud's syndrome (30% of patients).
Type 2. The classic type of dermatomyositis, accounting for 40% of forms of dermatomyositis.
Type 3. Polymyositis or dermatomyositis is combined with malignant neoplasms in 20% of cases.
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; damage to blood vessels such as allergic vasculitis (poor prognosis), muscles, skin and gastrointestinal tract. This type of dermatomyositis is represented by two options: the first is childhood dermatomyositis of the Banker-Victor type, the second is Brunsting type 2.
Type 5. Polymyositis or dermatomyositis in combination with other collagen diseases.

Etiology and pathogenesis of dermatomyositis

The etiology and pathogenesis have not been sufficiently studied. 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 has been noted: bronchial asthma, diffuse neurodermatitis, urticaria, and seasonal rhinitis are detected in relatives. The role of viruses is suspected, but not proven, since in dermatomyositis virus-like inclusions are found in the nuclei and cytoplasm of myocytes and epidermal cells. There is a point of view that considers dermatomyositis as a process that occurs as a result of allergies 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 immune complex vasculitis; the latter is proven by the detection of immunoglobulins and components of the complement system in the walls of skeletal muscle vessels. 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 lesions, the presence of lymphocellular infiltration, immunocomplex vasculitis, hypergammaglobulinemia, autoantibodies, circulating and fixed IR, cytotoxic activity of lymphocytes, the connection of dermatomyositis with other autoimmune diseases, the possibility of creating an experimental model, etc.
Pathomorphology. In the muscles of patients with dermatomyositis, necrosis, phagocytosis and regeneration, atrophy and degeneration of muscle fibrils, vacuolization, and perivascular infiltrates from mononuclear cells develop. In the dermis and epidermis, atrophy of the epidermis, degeneration of its basal layer, swelling of the upper layer of the dermis, inflammatory infiltrates and fibrinoid deposits are found, in the subcutaneous tissue - panniculitis and mucoid cell degeneration. Visceral pathology is manifested by vasculitis and mildly expressed inflammatory-sclerosing processes in the stroma.

Dermatomyositis Clinic

There are two age peaks in the incidence of dermatomyositis: the first in children at 5-15 years of age, the second in adults at 50-60 years of age. Depending on the form of the disease, certain characteristic features predominate; the main ones are the pathology of the skin and striated muscles. The disease most often begins gradually - with mild weakness, moderate myalgia and arthralgia, changes in the skin and localized swelling, less often - acute febrile temperature (38-39 ° C), diffuse erythema and muscle pain. General 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's papules, which are scaly purple-red skin lesions located on the extensor surfaces; swelling of the face; diffuse erythema; atrophic poikiloderma; vesicles and bubbles; calcinosis 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 of dermatomyositis - falling while walking, inability to lift the head from the pillow, comb one's hair, raise one's leg on a step, etc. Impaired functions of the facial muscles creates a certain mask-like appearance of the face - an “alabaster face”. 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 limitation of movements and is a sign of the disease becoming chronic.
Joint lesions are characterized by arthralgia, less commonly arthritis; dysfunction of the joints is mainly associated with muscle pathology.
Visceral changes mainly depend on damage to the muscles: cardiac (focal and diffuse myocarditis, cardiomyopathies), respiratory muscles (aspiration pneumonia), pharyngeal ring (increasing dysphagia). Dr. symptoms of visceral pathology are vasculitis: lungs (allergic pulmonary vasculitis), digestive tract (gastrointestinal bleeding, gastric perforation), which are especially often observed in childhood dermatomyositis of the Banker-Victor type.
In the acute course of dermatomyositis, death can occur within a year from the onset of the disease; in chronic cases, remission is long-term. A poor prognosis for children under two years of age with an acute onset of the disease, severe muscle weakness, rapid progression of the process with an ESR increased to 80 mm/h. Some patients experience spontaneous remissions. The prognosis for work ability is poor.

Diagnosis of dermatomyositis

Five main diagnostic criteria for dermatomyositis have been identified: symmetrical and progressive weakness of skeletal muscles (the process may involve the respiratory and swallowing muscles); typical histological picture with muscle biopsy (necrosis of muscle bundles with phagocytosis, regeneration with basophilia and inflammatory exudation); increased levels of creatine phosphokinase and aldolase in muscle tissue; electromyogram disturbance; characteristic skin lesions (periorbital edema with heliotrope and Gottron's papules). To make a diagnosis of dermatomyositis, four criteria must be present, and polymyositis - three.

Differential diagnosis of dermatomyositis

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

Treatment of dermatomyositis

To treat dermatomyositis, large doses of glucocorticosteroid drugs are used, preferably methylprednisolone, which causes muscle weakness to a lesser extent; triamcinolone, which increases 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 therapeutic effect occurs. Doses of glucocorticosteroid drugs should be adequate to the severity of the process: for an acute course - 80-100 mg of prednisolone, for subacute - 60, for chronic exacerbation - 30-40 mg per day. After the onset of the therapeutic effect, the dose is reduced to a maintenance dose - in acute and subacute cases, 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. Nonsteroidal anti-inflammatory drugs, aminoquinoline derivatives, can be used in the treatment of dermatomyositis, the latter for many years. In acute cases of dermatomyositis, bed rest is indicated, followed by exercise therapy, gymnastics, massage, physiotherapeutic procedures, and spa treatment.

Prevention of dermatomyositis

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

Dermatomyositis is a chronic rheumatological inflammatory disease of the muscles and skin. Another name for the disease is polymyositis, which is mainly used to refer to an illness without symptoms of skin lesions (25% of all cases). Dermatomyositis is a rare disease; on average, according to world statistics, it is diagnosed in 5 people per million people per year. Children under 15 years of age and people over 55 years of age are most often affected. Women get sick 2 times more often than men. In this article we will look at the symptoms and treatment of this disease.

Why does dermatomyositis develop?

Dermatomyositis develops in individuals with a genetic predisposition.

As with other rheumatological diseases, the underlying cause of the disease has not been found. A connection is expected with the following factors:

1. Chronic viral infection (eg, Coxsackie virus, herpes zoster).
2. , 30% of patients with dermatomyositis have an oncological diagnosis. What matters here is both the autoimmune reaction (when the body attacks both the tumor cells and its own cells) and the direct toxic effect of the decay products of tumor cells.
3. Genetic predisposition. In people with dermatomyositis, blood tests show an accumulation of HLAB8, which is associated with a variety of immune disorders.

Clinical variants of dermatomyositis

Classification of forms of dermatomyositis:

1. Primary idiopathic polymyositis. The term "idiopathic" means that the cause of the disease or condition is unknown.
2. Primary idiopathic dermatomyositis.
3. Dermatomyositis in combination with tumors.
4. Dermatomyositis in combination with.
5. Dermatomyositis in combination with.

Dermatomyositis in women

Polymyositis most often affects women between 30 and 50 years of age. Typical manifestations: gradual increase in symptoms, skin rashes, arthralgia.

Dermatomyositis affects mainly women of the same age group, but, unlike polymyositis, the disease begins and proceeds acutely, with severe musculocutaneous syndrome.

Dermatomyositis in children

In children, the most common variant is dermatomyositis in combination with vasculitis. The disease is acute and often recurs.

Dermatomyositis, combined with tumors, affects boys and girls equally.

Course of the disease

The course of the disease determines the volume and nature of treatment. Highlight:

• Acute course of dermatomyositis. After just six months, the patient becomes involved in the process of b ABOUT most of the muscles. Because of this, the person can no longer move, swallow, and sometimes even speak. The patient suffers from fever and poisoning from toxic products of the breakdown of his own muscles. The cause of death at this stage is aspiration pneumonia (for example, when vomit enters the lungs) or due to heart damage.
• Subacute course. Dermatomyositis regularly recurs, worsening the patient's condition. Manifestations of damage to internal organs gradually increase. The patient also becomes immobilized over time. With specific treatment, long-term remission (periods of relative health) is possible. Its duration depends on the patient’s condition and how carefully he follows medical instructions. With proper attention to oneself, the patient can live for many years with only slightly limited movements.
• Chronic course. The most favorable variant of the course of dermatomyositis. The disease affects only some muscle groups, so the patient feels relatively well and is able to work productively and live a full life. The exception is young men, who may develop large areas of calcification in the skin and muscles. This leads to immobility of a limb or joint, which means it significantly worsens the patient’s quality of life.

Symptoms of dermatomyositis

Muscle manifestations

1. Muscle pain during movement and at rest.
2. Myalgia that occurs when pressure is applied to a muscle.
3. Increasing muscle weakness, leading to disability of the patient. Over time, weakness increases so that the patient loses the ability to stand up, sit down, and eat independently. In the end, he finds himself completely immobilized.
4. The pathological process also extends to the facial muscles, so the patient is completely deprived of the opportunity to express his emotions through facial expressions.
5. The muscles of the larynx, pharynx, and soft palate are affected. Because of this, a person’s voice changes, and problems with swallowing food and water may occur.
6. Damage to the intercostal muscles and diaphragm leads to respiratory failure, hypoventilation and the development of pneumonia.

Skin lesions

1. In 40% of patients, erythema occurs on exposed parts of the body (face, neck, limbs).
2. Rashes such as papules and large blisters (bulls).
3. Telangiectasia.
4. Hyperkeratosis (excessive keratinization).
5. Hyperpigmentation.
6. Purple puffy spots around the eyes – dermatomyositis glasses.
7. Scaly red spots over the joints of the hands - Gottron's syndrome.

Raynaud's syndrome

Raynaud's syndrome is accompanied by numbness, a feeling of coldness, a feeling of pins and needles and is accompanied by pain in the hands; During the period between attacks, the hands may remain cold and cyanotic. In addition to the extremities, manifestations of the syndrome can be observed in the area of ​​the tip of the nose, chin, earlobes and tongue. The duration of the attack ranges from several minutes to several hours.

Raynaud's syndrome occurs in 10% of patients.

Joint lesions

1. When moving, pain occurs in the joints, which torments and limits the patient.
2. Sometimes the muscles are affected so quickly and severely that a person cannot bend his arm at the elbow or his leg at the knee precisely because of the formation of “ankylosis of a muscular nature.” Ankylosis is the inability to move in a joint.

Damages of the cardiovascular system

Damage to the gastrointestinal tract

Occurs in 50% of patients. Anorexia,... Due to damage to smooth muscles, hypotension of the esophagus, edema and necrosis in the walls of the stomach and intestines can develop.

Diagnosis of dermatomyositis

Blood tests

Increased levels of leukocytes (a sign of inflammation), eosinophils (a sign of an allergic reaction), high ESR, anemia. High levels of creatine phosphokinase, C-reactive protein, fibrinogen.

Muscle biopsy

The histologist detects thickening of muscle fibers and areas of necrosis in the tissue sample.

The diagnosis is made based on the presence of the following symptoms:

1. Progressive muscle weakness.
2. Skin syndrome (typical manifestations of dermatomyositis).
3. Increased muscle enzyme activity (high levels of creatine phosphokinase).
4. Typical changes in muscle tissue determined by biopsy results.

Treatment of dermatomyositis

The drugs of choice in the treatment of patients with dermatomyositis are. These are substances with high anti-inflammatory activity. They are especially effective when administered in a dose adequate for the stage of the disease, preferably in the early stages.

The main glucocorticosteroid in the treatment of dermatomyositis is prednisolone. It is prescribed in tablets, at a dose of up to 100 mg per day, in 4-6 doses. Usually, within 1-2 weeks of hormone therapy, the patient’s condition improves significantly: the timbre of the voice is restored, the patient stops choking when eating, pain and weakness in the muscles decrease.

After achieving the maximum effect, the dose of prednisolone begins to be gradually reduced. This is done in order to find the number of tablets per day that will help the patient maintain a satisfactory condition and avoid periods of exacerbation.

Unfortunately, prednisolone, like other glucocorticosteroids, has a number of side effects:

1. Education .
2. Attachment of infections.

A disease characterized by muscle damage with manifestations of deviations 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.

The disease occurs predominantly in adults over the age of 40, but inflammatory damage to the muscular system in children from 5 to 15 years is also possible. The disease in childhood is called juvenile dermatomyositis. Often, signs of malaise occur 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 rate of death. Thus, it is worth having an idea about dermatomyositis, its causes, symptoms and treatment methods, which this article will cover.

Species

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

1. Primary or idiopathic dermatomyositis, which is characterized by signs of independent occurrence not associated with early pathologies.
2. Secondary or paraneoplastic arises on the basis of the prevailing pathological abnormalities that arose as a result of previous illnesses. Often it is the secondary type that is most common.

Depending on the worsening of the disease, there are three degrees of complications, which are characterized by corresponding symptoms.

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

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

Reasons

Dermatomyositis belongs to a number of diseases, the causes of which remain at an insufficiently studied level. But this does not mean that there are no assumptions. Inflammatory disorders of the muscular system are multifactorial diseases, that is, they have different causes. The greatest likelihood of provoking dermatomyositis is determined by the predominance of infectious factors. Relevant studies were carried out on this matter, which proved the reliability of the statement.

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

• group A streptococci;
• hormonal medications;
• vaccines against and.

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

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

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

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

1. Pronormal- characterized by predominant severity over a period of several days to a month.
2. manifest- an advanced stage, which includes the occurrence of muscle, skin and other syndromes.
3. Dystrophic- the most difficult stage of the disease, caused by the occurrence of a general malaise of the body.

Causes of juvenile disease

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

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 that the child could have suffered from 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, which can result in death if appropriate measures are not taken.

Symptoms

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

The disease is characterized by a gradual progression. First of all, the general symptoms are the person’s complaints about general weakness. This weakness occurs due to damage to the muscles of the limbs. Symptoms of weakness occur unnoticed and may take years, so it is almost impossible to determine the presence of dermatomyositis based on such signs.

It’s another matter if the illness has an acute course, in which case the person experiences, in addition to general weakness, pain in the muscles. The pain is pronounced and appears within 2 weeks. This is characterized by an increase in temperature, which leads to a complete loss of strength. 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. Complete weakness of the body occurs, it becomes difficult for a person to get out of bed and perform various physical activities. The disease goes so deep that the neck muscles are unable to function. Most often, the patient is in a horizontal rather than a vertical position. With the localization of the disease, a disorder of the muscle tissue of the esophagus, pharynx, and larynx occurs, which is reflected in the form of speech impairment, coughing and difficulty eating. When swallowing food, a sharp cutting pain occurs in the throat. If you examine the oral cavity, you can observe the appearance of swelling, redness and dryness. Rarely there is a negative effect on the eye muscles.

Skin ailments. The occurrence of skin syndrome gives a clear picture of the predominance of dermatomyositis. Among the abnormalities on the skin, it is worth highlighting the following signs:

• the appearance of a rash on the face in the area of ​​the upper eyelids, nose, nasolabial fold. The rash spreads throughout the body: on the sternum, back, knees and elbows. The rash appears especially clearly on the upper extremities;
• roughening of the palms due to their redness and further peeling of the skin;
• nails become brittle and erythema occurs. Often there is separation of the nails on the toes and less often on the hands;
• the skin throughout the body becomes dry and red as the disease progresses;
• the further picture contributes to the occurrence of atrophy.

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

Joints. Joint pain rarely occurs when bending/extending the arms and legs. The wrist, 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, general weakness of the body occurs. It is possible to develop joint deformation, which is successfully prevented by taking glucocorticosteroids.

Mucous membranes. There is hyperemia, swelling of the palate, etc. Irritation of the back wall of the pharynx occurs, which leads to difficulty swallowing food.

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

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

In rare cases, it can be observed, which depends rather on the cause that led to the ailment of muscle tissue.

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

Gastrointestinal tract. The patient loses his appetite, which is reflected in weight loss, and abdominal pain occurs. Abdominal pain has a dull form of manifestation that can last for a long time. The causes of these pains lie in muscle ailments: the pharynx, esophagus and gastrointestinal tract. An X-ray examination reveals an increase in liver size.

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

Symptoms in children

Juvenile dermatomyositis in children begins with damage to internal organs. Symptoms of the disease differ from adults, first of all, in 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 on the face and extremities, where erythema occurs and turns red. Mostly on the face in children, erythema occurs around the eyes, which can lead to swelling and pain when blinking. If the child, even with such symptoms, is not treated, then the erythema spreads throughout the body.

Muscle dystrophy and partial lipodystrophy - in frequent cases, occur in childhood. When the muscles are damaged, a child experiences weakness, fatigue, lack of desire for active games, etc. Based on the first signs, parents do not exclude the possibility of the child becoming infected and are trying to find the cause.

Important! At the first noticeable symptoms of the disease, you should immediately show the child to the doctor to identify the disease.

Parents notice a complete loss of appetite in the child, which is caused by the development of aspiration. When swallowing food, a painful sensation occurs, and food can enter the respiratory tract, which can lead to the development of pneumonia.

Calcification also occurs quite often in children, which develops in 40% of patients with dermatomyositis. Calcinosis 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 ​​muscle fibers. Their deposition in more traumatic places cannot be ruled out:

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

In this case, calcification becomes diffuse in nature, that is, it is determined by the duration of its occurrence.

When the muscles of the diaphragm are damaged, there is a possibility of respiratory failure, which affects primarily the heart muscle. The first symptoms do not indicate the exact disease of dermatomyositis, so diagnostic studies will be required to clarify.

Diagnostics

Diagnosis of dermatomyositis includes collecting data on symptoms, as well as conducting a survey, laboratory and instrumental studies. Such 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. Based on 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 detect the presence of respiratory failure.
• Immunological study. A high titer of rheumatoid factor is detected.
• Muscle biopsy. If all of the above studies do not allow us to build a picture of the disease, then the decisive method is a biopsy. It is performed under local anesthesia; a sample of muscle tissue is taken from the patient using a special device for examination. After taking the sample, a microscopic examination is performed to determine the presence of inflammation.

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

Treatment

After excluding tumor and infectious diseases, it is necessary to begin direct treatment of dermatomyositis. The main effective drugs in the treatment of this disease are glucocorticosteroids. Moreover, it must be taken in high doses, but always as prescribed by a doctor. One of these drugs is prednisolone, which is prescribed depending on the nature of the manifestation of the disease.

Depending on the nature of the disease, the dosage is selected in the following quantities:

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

If the dose was prescribed correctly, then after seven days you can observe inhibition of the symptoms of the disease (intoxication). After two weeks, swelling disappears, erythema becomes pale and creatinuria decreases.

Juvenile signs of the disease in children are 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 drug has no effect, then a decision is made to gradually increase the dosage. The prescribed dosage is used for 1.5–2 months, after which the amount of the drug is gradually reduced over 2 years.

In addition to glucocorticosteroids, the possibility of a doctor prescribing cytostatic drugs: Methotrexate and Azathioprine cannot be ruled out.

Take a closer look at the effects of these drugs.

Methotrexate is started at a dosage of no more than 7.5 mg/week. After which 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. Afterwards, the positive dynamics of the drug’s effects are reviewed and, if present, the dosage is reduced. Treatment can last up to two to three years.

The following persons should not 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 efficiency. The dosage starts at 2 mg/day and continues until positive changes appear. These changes appear after about 7–8 months, after which it is worth reducing the dosage of the drug.

In addition to the above drugs, fortified products 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 prompt 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.