Treatment of dermatomyositis and its first symptoms. Dermatomyositis affects more than just the skin Dermatomyositis symptoms

What is the difference between dermatomyositis and polymyositis? The etiology of the changes is unknown, but the symptoms have some differences.

Dermatomyositis is characterized by signs from the side skin- erythematous rash and swelling of exposed areas of the body.

Polymyositis is displayed exclusively on the work of the muscles - the inflammatory process has no external signs.

The disease is rare - approximately 1 case per 200,000 of the population, and women are twice as likely to be affected. The most likely periods of life for the manifestation of the disease are from 5 to 15 and from 40 to 60 years. Myositis can be combined with various other pathologies:

a third of all cases develop together with connective tissue lesions: systemic lupus erythematosus, rheumatoid arthritis, scleroderma;
a tenth of the recorded myositis is accompanied by the development of oncological formations.

There is a hereditary predisposition and occurrence against the background of infectious diseases.

The reasons

The etiology of the disease is unknown. On the basis of research, two theories have been put forward, according to which dermato- and polymyositis can be triggered by autoimmune processes or viral damage to muscle tissues.

The pathophysiology of inflammation consists in such changes:

cell damage;
muscle atrophy (the muscles of the face or limbs are least likely to be damaged);
possible malfunctions internal organs due to the inflammatory process in their muscular structure (the pharynx, upper esophagus, intestines, stomach, heart are more often susceptible to this);
the concentration of myoglobin increases (negatively affects the performance of the kidneys);
joint damage;
inflammatory processes in the lungs.

Disease classification

The current classification is:

Primary idiopathic polymyositis. This variety makes up a third of all cases of pathology, characterized by imperceptible progression for a long time, the absence of sudden onset or severe symptoms. Muscle weakness begins with the proximal muscles of the limbs (more often in the femoral and pelvic region, shoulder girdle). The problem may focus on one muscle group. Rarely, aching pain or muscle tenderness on palpation is noted. In 30% of patients, cardiological changes were recorded, in addition, long-term hormone therapy exacerbates heart problems and increases the likelihood of myocardial infarction.
Primary idiopathic dermatomyositis - a type of pathology accounts for 25% of all cases of the disease. Dermatological changes may be: maculopapular rash, erythema (diffuse and localized), exfoliative or scaly eczematous dermatitis. Skin lesions are most often localized on the face (eyelids, bridge of the nose, cheeks, forehead), chest, knees and elbows, around the nail bed, on the knuckles. The rash may be accompanied by tissue edema, ulceration, and subcutaneous calcification.
Dermato- or polymyositis + oncological formations - accounts for about 8% of cases. Malignant cells can provoke the disease or appear some time after its onset (up to 2 years). This type of myositis occurs in the older age group of the population and is most often accompanied by foci of cancer cells in the lungs, chest, gastrointestinal tract, and ovaries.
Children's dermato- or polymyositis + vasculitis - 7% of myositis. The clinical picture manifests itself in the form of changes and disappearance of capillaries during their distribution, necrosis of skin areas, ischemic infarcts in the gastrointestinal tract, kidneys, and brain. The level of deaths in this type of pathology is up to 30%.
Dermato- or polymyositis + collagen diseases. Systemic lesions of the connective tissue in combination with myositis account for 5 of all cases of pathology (combinations with scleroderma, acute rheumatism, rheumatoid arthritis are common).

In addition to this classification, myositis is also distinguished with such concomitant pathologies:

Infections (Coxsackievirus, toxoplasmosis) - infection is accompanied by symptoms such as subfebrile temperature, fever, swelling and chronic fatigue. From the side of the muscles, the symptoms are subtle or absent altogether. A severe degree of the development of the disease is displayed in the form of problems from the central nervous system (focal manifestations, coma, delirium), heart (tachycardia).
Viral focal lesions (presumably - parotitis) are characterized by necrotic changes with involvement of distal muscles in the process.
Sarcoidosis - a quarter of patients with sarcoidosis have regenerating multinucleated myoblasts in skeletal muscles, which makes diagnosis difficult and often leads to misdiagnosis. Symptoms characteristic of polymyositis in this case do not appear.
Focal modular myositis is accompanied by the formation of painful inflamed nodules in the muscle tissue.

Note! Classification is important in making a diagnosis - the choice of a set of therapeutic measures depends on comorbidities.

Symptoms

Symptoms of pathology appear in stages and most often occur after a viral infection:

skin rash of dark color and erythematous type (with dermatomyositis);
the formation of subcutaneous calcifications (typical for children);
weakness of the proximal muscles;
polyarthalgia;
dysphagia;
Raynaud's syndrome;
hyperthermia;
cough and dyspnea (with lung damage);
weight loss;
general weakness, fatigue;
arrhythmia (with damage to the heart muscle);
vomiting with blood, violations of the integrity of the intestine (with damage to the gastrointestinal tract).

Signs of myositis depend on the area and degree of muscle damage.

Photo of dermatomyositis and polymyositis

Figure 1 Dermatomyositis on the back Figure 2 Dermatomyositis on the arms Figure 3 Dermatomyositis on the face
Fig 4 Polymyositis Fig 5 Polymyositis of the hands Fig 6 Polymyositis manifestations on the hands

Diagnostics

It is almost impossible to identify polymyositis at an early stage of occurrence due to a blurred picture of symptoms, moreover, characteristic and many other diseases. Diagnosis will require a comprehensive examination by a neurologist, immunologist, allergist, dermatologist, cardiologist and laboratory tests.

Criteria for the detection of dermato- and polymyositis:

Electromyography: revealed small, short polyphasic motor units, fibrillation.
High levels of muscle enzymes.
Histology: revealed fibril necrosis (1-2 types), inflammatory exudate, perifascial atrophy, phagocytosis, large nuclei in the sarcolemma.
There is symmetry in the weakening of the muscles (anterior flexor necks, pelvic and shoulder girdle). Symptom progresses long time and is accompanied by dysphagia, changes in the respiratory system.
Dermatological symptoms: erythematous rash on the face (butterfly shaped), tissue edema, erythematous scaly dermatitis with manifestation on the hands, elbows, kneecaps, neck and upper chest.

Differential diagnosis is based on the results laboratory research.

Treatment

The basis of therapy for dermato- and polymyositis is the complex use of glucocorticoid drugs in long courses.

Self-medication is unacceptable, since hormonal drugs have a lot of side effects and an individual calculation of the dosage and duration of the course is important.

Drugs used for therapy:

Glucocorticoids (Prednisolone, Methylprednisolone). The dose is individual. It is important that the course ends gradually - with a slow decrease in the amount of medication taken, and does not stop abruptly. If there is no effect, the dosage is increased or the treatment is supplemented with other means.
Vitamin D, calcium - is prescribed as adjuvant therapy to reduce the side effect of hormone-containing drugs.
Immunosuppressants: Azathioprine, Methotrexate (less effective - Cyclosporine, Cyclophosphamide). They are prescribed for low efficiency of glucocorticoids and allow to reduce the amount of the latter taken. The course of treatment is long, the effect is noticeable after 4-5 months of continuous therapy. It is important to remember that these drugs suppress the function of hematopoiesis, reduce immunity and provoke malfunctions in the digestive tract.
Immunoglobulin therapy, plasmapheresis - used for severe forms pathologies to improve the effectiveness of drugs.
Physiotherapy procedures (exercise therapy) to restore the condition of muscle tissue.

In addition, each organism reacts to therapy in its own way - this requires observation, timely correction of the course.

Forecasts

The success of treatment largely depends on the degree of damage to the body at the time of diagnosis and initiation of therapy. Long-term remission over a five-year period is observed in more than 50% of patients (in childhood- up to 80%). But still, overall survival in polymyositis and dermatomyositis is 4 times lower than in the general population.

The highest mortality is observed among women (especially the elderly) with acute and severe course.

Polymyositis and dermatomyositis are difficult to diagnose, but when detected on early stage successfully treatable (provided there are no serious concomitant pathologies, for example, malignant tumors).

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All information is presented for educational purposes. Do not self-medicate, it is dangerous! An accurate diagnosis can only be made by a doctor.

Dermatomyositis is a chronic rheumatological inflammatory disease muscles and skin. Another name for the disease is polymyositis, it is mainly used to refer to an ailment 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 persons over 55 years of age are more often ill. 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 in the case of other rheumatological diseases, the underlying cause of the disease has not been found. It is expected to be associated with the following factors:

Chronic viral infection (for example, Coxsackie virus, herpes zoster,).
, 30% of patients with dermatomyositis have an oncological diagnosis. Here, both the autoimmune reaction (when the body attacks both tumor cells and its own cells) and the direct toxic effect of the decay products of tumor cells are important.
genetic predisposition. In individuals with dermatomyositis, the blood test shows the accumulation of HLAB8, which is associated with a variety of immune disorders.

Clinical variants of dermatomyositis

Classification of forms of dermatomyositis:

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

Dermatomyositis in women

Most often, women from 30 to 50 years old get sick with polymyositis. 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, there is a severe musculoskeletal syndrome.

Dermatomyositis in children

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

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

Course of the disease

The course of the disease determines the scope and nature of the treatment. Allocate:

Acute course of dermatomyositis. Within six months, the patient is involved in the process of O most of the muscles. Because of this, a person can no longer move, swallow himself, and sometimes even speak. The patient suffers from fever and poisoning with toxic decay products of his own muscles. The cause of death at this stage is aspiration pneumonia (for example, when vomit enters the lungs) or against the background of heart damage.
Subacute flow. Dermatomyositis regularly recurs, worsening the patient's condition. Gradually, manifestations of damage to internal organs increase. The patient also becomes immobilized over time. Against the background of specific treatment, a long-term remission (periods of relative health) is possible. Its duration depends on the condition of the patient and on how carefully he fulfills medical prescriptions. With proper attention to yourself, the patient can live for many years, being only slightly limited in movement.
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 exceptions are young men, who may develop large areas of calcification in the skin and muscles. This leads to immobility of the limb or joint, and therefore significantly worsens the quality of life of the patient.

Symptoms of dermatomyositis

Muscular manifestations

Pain in the muscles during movement and at rest.
Myalgia caused by pressure on the muscle.
Increasing muscle weakness, leading to disability of the patient. Over time, weakness increases so that the patient loses the ability to get up, sit down, eat independently. In the end, he is completely immobilized.
The pathological process also extends to the muscles of the face, so the patient is completely deprived of the opportunity to express his emotions through facial expressions.
The muscles of the larynx, pharynx, soft palate are affected. Because of this, a person's voice changes, and there may be problems with swallowing food and water.
The defeat of the intercostal muscles and the diaphragm leads to respiratory failure, hypoventilation and the development of pneumonia.

Skin lesions

In 40% of patients, erythema occurs on open parts of the body (face, neck, limbs).
Rashes such as papules and large blisters (bull).
Telangiectasia.
Hyperkeratosis (excessive keratinization).
Hyperpigmentation.
Purple puffy spots around the eyes - dermatomyositis glasses.
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 goosebumps and is accompanied by pain in the hands; between attacks, the hands may remain cold and bluish. 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 damage

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

Damage to the cardiovascular system

Damage to the gastrointestinal tract

Occurs in 50% of patients. Anorexia is noted. 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

Elevated levels of leukocytes (a sign of inflammation), eosinophils (a sign of 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:

Progressive muscle weakness.
Skin syndrome (manifestations typical of dermatomyositis).
Increased activity of muscle enzymes (high levels of creatine phosphokinase).
Typical changes in muscle tissue, established by the results of a biopsy.

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

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

Education .
Accession of infections.

Dermatomyositis(DM) syn. Wagner's disease, Wagner-Unferricht-Hepp disease - a severe progressive systemic disease of connective tissue, skeletal and smooth muscles with impaired motor function, skin in the form of erythema and edema, and vessels of the microcirculatory bed with damage to internal organs, often complicated by calcification and purulent infection. In 25-30% of patients, skin syndrome is absent. In this case, one speaks of polymyositis (PM).

Frequency DM is considered a rare disease. Women get sick more often. In children, the frequency is 1.4-2.7:1, in adults 2-6.2:1.

Etiology The reasons are unknown. Currently, DM is considered a multifactorial disease. Since DM is more common in southern European countries, and the incidence increases in spring and summer, the role of insolation is not excluded. However, the greatest importance is currently attached to infectious agents. Epidemiological studies indicate the frequent presence of infectious diseases during the 3 months preceding the onset of DM. Influenza, parainfluenza, hepatitis B viruses, picornaviruses, parvovirus, and protozoa (toxoplasma) are considered as etiologically significant. Among bacterial pathogens, the role of borreliosis and group A β-hemolytic streptococcus is emphasized. Other alleged etiological factors include some vaccines (against typhoid, cholera, measles, rubella, mumps) and medicinal substances (D-penicillamine, growth hormone).

Classification

Origin

idiopathic (primary);

paraneoplastic (secondary, tumor);

juvenile dermatopolymyositis (children's)

dermatomyositis (polymyositis) in combination with other diffuse connective tissue diseases.

With the flow

subacute;
chronic.

Clinic Clinical manifestations are diverse, they are caused by a generalized lesion of the microcirculatory bed, but skin and muscle syndrome are the leading ones.

Skin changes The classic skin manifestations are Gottron's sign and heliotrope rash. Gottron's symptom - the appearance of red and pink, sometimes scaly nodules and plaques on the skin in the area of the extensor surfaces of the joints (often interphalangeal, metacarpophalangeal, elbow and knee). Sometimes the symptom of Gottron is presented only by a soft redness, subsequently completely reversible.

Heliotrope rash - is a purple or red skin rash on the upper eyelids and the space between the upper eyelid and eyebrow (a symptom of "purple glasses"), often in combination with swelling around the eyes. The rash can also be located on the face, on the chest and neck (V-shaped), on the upper back and upper arms (shawl symptom), abdomen, buttocks, thighs and legs. Often on the skin of patients there are changes in the type of tree branch (tree-like livedo) of a burgundy-bluish color in the area of the shoulder girdle and proximal limbs.

An early sign of the disease may be changes in the nail bed, such as reddening of the periungual folds and overgrowth of the skin around the nail bed. Skin manifestations in DM often precede muscle damage by several months or even years on average. Isolated skin lesions at the onset are more common than muscle and skin lesions at the same time.

Skeletal muscle damage The cardinal symptom is symmetrical weakness of the muscles of the shoulder and pelvic girdle, neck flexors and abdominal muscles of varying severity. Usually, difficulty in performing daily activities is noticed: climbing stairs, getting up from a low chair, etc. The progression of the disease leads to the fact that the patient does not hold his head well, especially when he lies down or gets up. Terrible symptoms are damage to the respiratory and swallowing muscles. Involvement of the intercostal muscles and diaphragm can lead to respiratory failure. When the muscles of the pharynx are affected, the timbre of the voice changes, nasality, choking begins, and difficulties arise when swallowing food. Often, patients complain of muscle pain, although muscle weakness can occur without pain. Inflammatory changes in the muscles are accompanied by a violation of their blood supply, nutrient delivery, which leads to a decrease in muscle mass, growth of connective tissue in the muscles and the development of tendon-muscle contractures.

Lung damage The defeat of the pulmonary system in patients with dermatomyositis is due to a number of factors: muscular syndrome (hypoventilation), the presence and development of infection, aspiration during swallowing, the development of interstitial pneumonia and fibrosing alveolitis. Muscle weakness that extends to the respiratory muscles, including the diaphragm, causes a decrease in the ventilation function of the lungs. Clinically, this is expressed in frequent and shallow breathing, inspiratory dyspnea, and the development of hypostatic pneumonia. Dysphagia with aspiration of liquid and food into the lungs causes the development of aspiration pneumonia. Pulmonary involvement is detected in 5-46% of patients with dermatomyositis, mainly in the form of interstitial pneumonia, fibrosing alveolitis and fibrosis. Shortness of breath and cough, wheezing and crepitus are observed with severe lung damage. Pulmonary function tests indicate a predominantly restrictive type of impairment with a decrease in total and vital lung capacity, hypoxemia is characterized by a moderate decrease in lung diffusion capacity. There are certain subtypes of interstitial lung disease that should be considered in the diagnosis and treatment of dermatomyositis:

Acute or subacute type with severe rapidly progressive dyspnea and increasing hypoxemia already in the first months of the disease.

Chronic type with slowly progressive dyspnoea.

Asymptomatic type, proceeds subclinically, is detected by X-ray and functional examination of the lungs.

The first type of interstitial lung disease has the worst prognosis and requires early active therapy with glucocorticosteroids, cytostatics, etc. Pulmonary fibrosis develops in 5-10% of patients. It is characterized by increasing inspiratory dyspnea, dry cough, crepitant wheezing in the lower parts of the lungs, and progressive respiratory failure. It is necessary to keep in mind the possibility of developing a tumor, more often metastatic, process in the lungs.

Other symptoms Soft tissue calcification (mainly muscles and subcutaneous adipose tissue) is a feature of the juvenile variant of the disease, observed 5 times more often than with DM in adults, and especially common in preschool age. Calcification can be limited or diffuse, symmetrical or asymmetric, is the deposition of deposits of calcium salts (hydroxyapatites) in the skin, subcutaneous tissue, muscles or intermuscular spaces in the form of single nodules, large tumor-like formations, superficial plaques. With a superficial location of calcifications, an inflammatory reaction of the surrounding tissues is possible, suppuration and rejection of them in the form of crumbly masses. Deeply located muscle calcifications, especially single ones, can only be detected by X-ray examination.

Joint damage Articular syndrome can be manifested by pain and limited mobility in the joints, morning stiffness in both small and large joints. Swelling is less common. As a rule, during treatment, all changes in the joints undergo a reverse development.

Heart failure The systemic muscular process and vascular damage causes frequent involvement of the myocardium in the pathological process, although with DM all three membranes of the heart and coronary vessels can suffer, up to the development of a heart attack. In the active period, patients have tachycardia, muffled heart tones, rhythm disturbances.

Gastrointestinal tract lesions The main cause of damage to the gastrointestinal tract in DM is widespread vascular damage with the development of malnutrition of the mucous membrane, impaired nerve patency and damage to smooth muscles. A clinic of gastritis, colitis, including erosive and ulcerative lesions, is possible. In this case, minor or profuse bleeding may occur, and perforation leading to peritonitis is possible.

Endocrine disorders They are manifested by changes in the functional activity of the gonads, the pituitary-adrenal system, which can be associated both with the severity of the disease and vasculitis, and with ongoing steroid therapy.

Diagnostics The basis for the diagnosis is the clinical picture. In the general blood test with DM, there is only moderate elevated ESR, slight leukocytosis. In the biochemical analysis of blood, an increase in the so-called. “muscle breakdown enzymes” (creatine phosphokinase, lactate dehydrogenase, ALT, AST, aldolase), which is of diagnostic value. In an acute process, CPK and LDH can exceed the norm by 10 times or more. Immunological studies: AT to histidyl tRNA synthetase (Jo 1) Instrumental methods - muscle biopsy - to confirm the diagnosis

Treatment The basis of treatment is glucocorticoids, according to indications of cytostatics (methotrexate, cyclophosphamide, azathioprine) and drugs aimed at eliminating microcirculation disorders, metabolism, maintaining the functions of internal organs, preventing complications of the disease and therapy.

Literature

1. E. I. Alekseeva, S. I. Valieva, T. M. Bzarova, E. G. Chistyakova et al. “Rheumatology” - a collection of methodological materials p. 40-47

2. Pediatric rheumatology: a guide for physicians, ed. A. A. Baranova, L. K. Bazhenova. M., Medicine, 2002.

3. L. A. Saykova, T. M. Alekseeva "Chronic polymyositis" M., Foliant, 2000, 120 p.

Dermatomyositis

Dermatomyositis- inflammatory muscle damage, characterized by damage to the striated and smooth muscles with impaired motor function, as well as skin lesions in the form of redness and swelling, mainly in open areas of the body.

The prevailing age of the development of the disease: two peaks of incidence are determined - at the age of 5-15 and 40-60 years. The predominant gender is female (2:1).

The cause of dermatomyositis is not known. The possible role of viral factors, primarily picornaviruses, is discussed. The presence of a connection between oncology and dermatomyositis suggests an autoimmune reaction due to antigenic masking of tumor tissues and muscle tissue.

Manifestations of dermatomyositis Muscle weakness: difficulty brushing your hair, brushing your teeth, getting up from a low chair, getting into a vehicle

Skin lesions: photodermatitis and "solar" edema of the eye area, redness of the skin of the face and in the "décolleté" area, red scaly rashes over the small joints of the hands, redness and peeling of the skin of the palms (mechanic's hand)

Swallowing disorders. Dryness of mucous membranes

Lung damage. Heart failure

Symmetric joint damage without deformities, most often affecting the small joints of the hands; often develops at the onset of the disease

Carpal tunnel syndrome: swelling of the hands, pain and decreased sensitivity in fingers I-III and fingers IV

Kidney damage

Testing for dermatomyositis

An increase in the content of CPK in the blood serum

Serum aldolase is elevated

Increased serum creatinine (less than 50% of patients)

The presence of myoglobin in the urine

Increase in ESR in general analysis blood

High titers of rheumatoid factor (less than 50% of patients) in serum

Presence of ANAT (more than 50% of patients)

On the ECG - arrhythmias, conduction disturbances

Electromyography - increased muscle excitability

Muscle biopsy (deltoid or quadriceps femoris) - signs of inflammation

X-ray changes in the joints are not typical (in children, the formation of calcifications in soft tissues is possible)

Treatment of dermatomyositis Hormones are the drug of choice in the treatment of dermatomyositis (eg, prednisolone). AT acute stage diseases, the initial dose of prednisolone is 1 mg / kg / day. If there is no improvement within 4 weeks, the dose should be increased by 0.25 mg / kg / month to 2 mg / kg / day with an adequate assessment of clinical and laboratory efficacy. After achieving clinical and laboratory remission (but not earlier than 4-6 weeks from the start of treatment), the dose of prednisolone is gradually reduced (about 1/4 of the daily dose during each month under clinical and laboratory control, with negative dynamics, the dose is again increased). The total duration of treatment for dermatomyositis is approximately 2-3 years.

Methotrexate. When taken orally, the initial dose is 7.5 mg / week with an increase of 0.25 mg / week until the effect is obtained (no more than 25 mg / week). At intravenous administration initial dose of 0.2 mg/kg/week with an increase of 0.2 mg/kg/week (no more than 25 mg/week) until the effect is obtained. With this disease, methotrexate is not administered intramuscularly! The clinical effect of the drug usually develops after 6 weeks, the maximum effect after 5 months. Upon reaching remission, methotrexate is canceled, gradually reducing the dose (by 1/4 per week). In the treatment of dermatomyositis, it is necessary to conduct general blood tests, urine tests, as well as liver function tests. Methotrexate is contraindicated in pregnancy, diseases of the liver, kidneys, bone marrow; incompatible with anticoagulants, salicylates and drugs that inhibit hematopoiesis

Azathioprine (less effective than methotrexate). Dose 2-3 mg / kg / day. The maximum effect usually develops after 6-9 months. Further, the daily dose is reduced by 0.5 mg / kg every 4-8 weeks to the minimum effective. Azathioprine is contraindicated in severe hematopoiesis suppression, severe liver disease, pregnancy

Cyclosporine: initial dose 2.5-3.5 mg/kg, maintenance dose 2-2.5 mg/kg

Cyclophosphamide is used in the development of lung damage at a dose of 2 mg / kg / day.

Aminoquinoline derivatives (hydroxychloroquine 200 mg/day) can control the skin manifestations of dermatomyositis.

Immunoglobulin intravenously at a dose of 0.4-0.5 g / kg (long-term treatment).

Plasmapheresis, lymphocytopheresis

Dermatomyositis, also called Wagner's disease, is a very severe inflammatory disease of muscle tissue, which develops gradually and also affects the skin, causing swelling and erythema, internal organs. In this case, mobility is severely impaired. Very often, this systemic ailment can be complicated by the deposition of calcium in muscle tissues or the development of purulent infectious diseases.

The development of this disease in women occurs 2 times more often than in men. The disease is also selective in the age of the victims, the criteria for its selection indicate children from 5 to 15 years old, or adults from 40 to 60 years old.

Causes

Officially, dermatomyositis is considered a multi-symptom disease. And yet, the long history of its study has not borne fruit in the form of an understanding of its etiology. Therefore, the classification of diseases designates it as idiopathic. Experts believe that the disease can be affected by:

complications after suffering viral infections, such as the flu;
a secondary phenomenon against the background of an existing oncological disease;
genetic predisposition to this disease;
reaction to various drugs;
reaction to vaccination against cholera, measles, mumps, typhoid, rubella;
pregnancy;
insolation;
drug allergy;
injury;
hypothermia;
borreliosis.

Symptoms

In order for the diagnosis of the disease to be successful, you need to pay attention to the following symptoms:

the occurrence of muscle weakness, which can be expressed in difficulties in carrying out the simplest daily activities;
on the skin, the affected areas are visible in the form of photo dermatitis, the occurrence of edema around the eyes, skin color changes to red in the face and décolleté area, the appearance of red rashes over the small joints of the hands, the surface of which is flaky, coarsening and flaking of the skin on the palms, resembling a surface palms of a person working in adverse physical conditions;
difficulty in swallowing;
drying of the surfaces of the mucous membranes of the body;
difficult work of the lungs;
dysfunction of the heart;
at the very beginning of the development of the disease, damage to small joints is most often observed, usually starting on the hands;
swelling of the hands;
occurrence pain and numbness in the fingers;
disruption of the kidneys.

Diagnostics

Diagnosis of a lesion in the event of an ailment is relatively uncomplicated. It includes such criteria.

The most common manifestation of the disease on the surface of the skin may be the appearance of red and pink nodules and plaques, which sometimes peel off. Their location usually occurs in the areas of the extensor joints. Sometimes, instead of them, only redness appears, which can be removed over time. The appearance of a purple rash, located from the edge, is also common. upper eyelid all over the area up to the eyebrow line. It can be combined with edema and represent some kind of purple glasses. This symptom is immediately noticeable, especially when compared with an earlier photo of the patient. Such a rash is based not only in this area, but also spreads over the face, in bulk goes down the neck to the chest, covering the décolleté area, and also appears on the upper back and arms. You can meet her on the stomach, as well as on the entire lower body. When scleroderma develops, dermatomyositis moves to a deeper stage.

At an early stage of the development of the disease, one can notice another important sign, which the patients themselves pay attention to last. These are the changes that affect the nail bed. At the same time, the periungual ridges become red, and skin grows around the bed.

All these manifestations are the first bells that appear long before the onset of muscle damage. Simultaneous damage to both skin and muscle tissue is extremely rare. Timely diagnosis of the disease can help stop or simply slow down its development.

The fact that the muscles are already affected is clearly indicated by muscle weakness. While doing their usual activities, patients notice difficulty in climbing stairs or in the process of putting in order their appearance. It is expressed in the weakness of the muscles at the level of the shoulders and pelvis, the muscles responsible for bending the neck, as well as the abdominal muscles. Developing, the disease can prevent a person from holding his head, especially when he takes a horizontal position or rises from it. When the intercostal muscles are affected, they affect the functioning of the diaphragm. This results in respiratory failure. By affecting the muscles located in the pharynx, the disease changes the timbre of the voice, and also causes difficulty in swallowing. During this period, some patients may experience the appearance of pain in the muscle tissues, although more often it does not happen. Inflammation of the muscles leads to impaired blood supply, muscle mass decreases, connective tissue grows more and more. At this time, tendon-muscle contactures develop. This stage in the development of the disease can complicate polymyositis, in which dermatomyositis will be more painful.

When the disease affects the lungs, respiratory failure can be joined by various infectious diseases, pneumonia and alveolitis. A person begins to breathe often and shallowly, he has shortness of breath. Sometimes fibrosis develops. If the lesion is pronounced, then the patient's constant companion will be shortness of breath, wheezing, crackling sounds in the chest and wheezing. Naturally, the volume of the lungs is sharply reduced.

Sometimes you can observe the deposition of calcium in muscle tissue. It occurs most often at a young age, especially in preschoolers. You can notice it by paying attention to the presence of nodules under the skin, plaques on the surface of the skin, or formations resembling a tumor. If the deposit is on the surface of the skin, then the body tries to get rid of it, which causes suppuration and its rejection in the form of crumbs. Diagnosis of deposits located in deep layers can be successful only with x-ray examination.

Affected joints can hurt, sometimes there is swelling, in the morning there is stiffness in them. Such joints lose their mobility.

The heart is an organ made up of muscles. Therefore, all its membranes suffer, causing tachycardia, muffled tones, disturbances in the rhythm of the heartbeat, heart attacks often occur. Thus, the disease is rapidly fatal if it is not stopped in time.

With damage to the gastrointestinal tract, we can observe clinical picture characteristic of diseases such as colitis or gastritis.

Diagnostic studies show that the activity of the glands responsible for sexual activity and the adrenal glands are dulled.

When diagnosing idiopathic dermatomyositis, you can notice small changes in the analyzes:

ESR in the general blood test is only slightly increased;
there is a slight leukocytosis;
in the blood there is the presence of enzymes resulting from the breakdown of muscles.

All other diagnostic studies are carried out only to confirm the diagnosis of dermatomyositis.

Treatment

The main drugs needed to successfully treat dermatomyositis are glucocorticoids, along with which, if necessary, cytostatics are used. Also in the process of treatment, drugs are involved, the main function of which is the restoration of microcirculation and metabolism in the body. In addition, there is a need for drugs that support the internal organs and help prevent the development of various complications.

Forecast

The prognosis for patients with this disease is not very encouraging. 2 out of 5 patients die within only 2 years after its detection. The main causes of death are problems with respiratory system, heart attack and complications of the gastrointestinal tract.

Dermatomyositis is a recurrent severe and progressive disease of the whole organism with characteristic inflammatory and degenerative changes in the skin, connective tissue, skeletal and smooth muscles, blood vessels and internal organs. We will talk about it in detail today.

Pathogenesis and features of the disease

Self-treatment leads to rapid progression of pathology and life-threatening complications.

Dermatomyositis in children is dedicated to the video below:

Treatment

Medical

Traditionally applied medications 7 types.

Glucocorticosteroids

The most optimal choice, prescribed at the rate of 1 mg per day per 1 kilogram of body weight of an adult patient in the acute stage. In severe cases, the daily dose for a month is increased to 2 mg / kg. When a therapeutic effect is achieved, they very slowly switch to reduced dosages (¼ of the used one). It is unacceptable to quickly reduce the dosage in order to avoid severe exacerbations.

It is highly undesirable to prescribe non-steroidal anti-inflammatory drugs instead of Prednisolone. This dramatically worsens the prognosis and increases the likelihood of serious consequences.

Immunosuppressive cytostatics

Assign with low therapeutic efficacy of steroids. Basic:, (with pulmonary fibrosis).

The initial dose of Methotrexate by mouth is 7.5 mg per week, increased by 0.25 mg per week until the effect is obtained (maximum weekly dose is 25 mg)
Intravenous infusion (no intramuscular methotrexate is administered) begins with 0.2 mg per 1 kg of the patient's weight per week, increasing the dose by 0.2 mg / kg per week.
The expected therapeutic result is observed after 1 - 1.5 months, the maximum healing effect- after 5 months. Reduce the dose very slowly (by a quarter of the one used per week).
The scheme of therapy provides for the joint use of Methotrexate with Prednisolone.
Azathioprine is started at a dose of 2-3 mg/kg per day. The drug gives fewer complications to the blood system, and treatment with them can be long. Because azathioprine is considered less potent than methotrexate, it is often combined with corticosteroids.
The introduction of vitamin B 9 (folic acid), reduces the risk side effects especially those associated with impaired liver function.

Other means

Aminoquinoline drugs in low doses. Prescribed for mitigation skin manifestations as maintenance therapy, usually with chronic course and in combination with other medicines. Basic:, Hydroxychloroquine 200 mg / day.
Intravenous infusion of immunoglobulin at a dose of 0.4 - 0.5 grams per kilogram per day is carried out in order to increase positive reaction patient on standard therapy hormonal means. In many patients, immunoglobulin reduces inflammation by influencing the immune system.
Prozerin(during remission), cocarboxylase, Neostigmine, ATP, B vitamins in injections, to normalize muscle functions.
Anabolic steroid such as Nerobol, Retabolil, are more often used as muscle tissue-strengthening agents for a long-term course of Prednisolone.
If small calcifications form, a certain therapeutic result is achieved when internal application Colchicine, Probenecid, intravenous administration of Na 2 EDTA, local application of Trilon B.

Therapeutic

and lymphocytapheresis is used mainly in patients with severe course, difficult to respond to traditional treatment, with signs of vasculitis and severe muscle pathology.
Therapeutic exercise, designed to prevent muscle contractures, is mandatory, especially in childhood, but only in the period of remission.

Surgical

Sometimes single subcutaneous calcifications are surgically removed. But this is not very effective, and the main task is early detection and prevention of salt deposits, especially in childhood dermatomyositis, using high-dose hormone therapy, sometimes even "aggressive".
The same scheme is used to suppress the growth of tumor formations in paraneoplastic dermatomyatosis. Surgery, combined with medication, very often helps to eliminate or significantly reduce the severity of abnormal manifestations.

Features of therapy

Recently, the use of new, genetically engineered biological products has begun, but strictly individually and according to a scheme developed by a specialist doctor.
Given that Prednisolone and Metipred give serious side effects, prescribe drugs that protect the gastric mucosa (gastroprotectors), including omeprazole, ranitidine, calcium and vitamin D preparations, bisphosphonates to prevent osteoporosis.

During the course of Metipred, it is not allowed to consume sugar and sweet foods in order to avoid the body's tolerance to glucose.
With exacerbations, rest is strictly indicated. When the process subsides, you can gradually practice small physical exercise, study physical therapy, but very carefully so as not to provoke an exacerbation of the disease.

Disease prevention

Measures that could prevent the development of dermatomyositis have not yet been developed. Secondary prevention measures, after the diagnosis of the disease, include:

maintenance treatment with corticosteroids,
control examinations by a dermatologist, rheumatologist,
cancer tests,
timely treatment of any inflammatory diseases,
elimination of foci of infection in the body.

Complications

With long-term current dermatomyositis without treatment, the following develop:

and trophic ulcers;
contractures, bone deformities;
loss of muscle mass;
calcification.

Most serious complications, threatening the patient with advanced dermatomyositis, from which, without appropriate treatment, up to 40% of patients die in the first 2 years:

aspiration pneumonia, alveolar fibrosis;
destruction of the muscles of the respiratory organs, esophagus and pharynx;
gastrointestinal bleeding;
cardiac pathologies;
general dystrophy, exhaustion

Forecast

Previously, pathology led to the death of almost 2/3 of patients. Today, the use of corticosteroids gives a pronounced therapeutic result, suppresses the aggressiveness of the disease and, if used correctly, significantly improves the long-term prognosis.

Dermatomyositis may present one episode, passing into the stage (inactive course (remission) within 2 years after the first signs, and then - do not give relapses.
With a polycyclic flow long periods of remission alternate with relapses. This often occurs if the dose is drastically reduced or Prednisolone is discontinued.
Chronically flowing dermatomyositis despite treatment has a higher chance of developing complications.

The earlier an accurate diagnosis is made and treatment started, the better the long-term prognosis. In children, dermatomyositis can result in an almost complete cure or sustained remission.

Even more about dermatomyositis and related ailments will tell the video below: