Dermatomyositis: symptoms

Dermatomyositis - symptoms, treatment, forms, stages, diagnosis

Dermatomyositis (generalized fibromyositis, generalized myositis, angiomyositis, sclerodermatomyositis, poikilodermatomyositis, polymyositis) is a systemic inflammatory disease that affects muscle tissue, skin, capillaries and internal organs.

Source: guardianlv.com

Causes and risk factors

The main role in the pathological mechanism of the development of dermatomyositis belongs to autoimmune processes, which can be considered as a malfunction of the immune system.

Under the influence of provoking factors, she begins to perceive smooth and transversely hairy muscle fibers as foreign and to develop antibodies against them (autoantibodies).

They not only affect the muscles, but also are deposited in the blood vessels.

It is suggested that the development of dermatomyositis may be due to neuroendocrinal factors. This is partly confirmed by the development of the disease in the transitional periods of life (with puberty, menopause).

Predisposing factors:

• some viral infections (Coxsackie virus, picornaviruses);
• malignant neoplasms;
• supercooling;
• hyperinsolation (prolonged exposure to the sun);
• stress;
• allergic reactions;
• hyperthermia;
• pregnancy;
• drug provocation, including vaccination.

Forms of the disease

Depending on the cause of the onset, the following forms of dermatomyositis are distinguished:

• idiopathic (primary) - the disease begins on its own, without any connection with any factors, it is not possible to find out the cause;
• secondary tumor (paraneoplastic) - develops against the background of malignant tumors;
• children's (juvenile);
• combined with other pathologies of connective tissue.

By the nature of the inflammatory process, dermatomyositis is acute, subacute and chronic.

Stages of the disease

In the clinical picture of dermatomyositis, several stages are distinguished:

1. Prodromal period - there are nonspecific harbingers of the disease.
2. Manifest period - is characterized by an expanded clinical picture with bright symptoms.
3. The terminal period is characterized by the development of complications [eg, dystrophy, depletion (cachexia)].

Symptoms

One of the earliest nonspecific signs of dermatomyositis is the weakness of the muscles of the lower extremities, which gradually increases with time. Also, the manifest period of the disease may be preceded by Raynaud's syndrome, polyarthralgia, skin rashes.

The main symptom of dermatomyositis is the defeat of skeletal (transverse-striated) musculature. Clinically, this is manifested by the growing weakness of the muscles of the neck, upper limbs, which in time makes it difficult to perform the most routine, routine actions.

As the dermatomyositis progresses, the muscles of the pharynx, the upper part of the digestive tract, the diaphragm, the intercostal muscles are drawn into the pathological process. As a result, there are:

• speech function disorders;
• dysphagia;
• impaired ventilation;
• relapsing congestive pneumonia.

Dermatomyositis is characterized by skin manifestations:

• erythematous-spotted rash;
• periorbital edema;
• a symptom of Gottron (perigranular erythema, striated nail plate, redness of the palms, erythematous flaky spots on the skin of the fingers);
• alternation of sites of skin atrophy and hypertrophy, pigmentation and depigmentation.

The defeat of the mucous membranes against the background of dermatomyositis leads to the development of:

• hyperemia and swelling of the pharyngeal walls;
• stomatitis;
• conjunctivitis.

Systemic manifestations of dermatomyositis include lesions:

• joints (phalanx, wrist, elbow, shoulder, ankle, knee);
• heart - pericarditis, myocarditis, myocardiofibrosis;
• lungs - pneumosclerosis, fibrosing alveolitis, interstitial pneumonia;
• organs of the gastrointestinal tract - hepatomegaly, dysphagia;
• nervous system - polyneuritis;
• kidney - glomerulonephritis with impaired renal excretory function;
• endocrine glands - a decrease in the function of the sex glands and adrenal glands.

Features of the course of dermatomyositis in children

In comparison with adult patients, dermatomyositis begins more sharply in children. For the prodromal period are characteristic:

• general malaise;
• increased body temperature;
• myalgia;
• decreased muscle strength;
• arthralgia;
• general weakness.

The clinical picture of juvenile dermatomyositis combines signs of damage to various organs and systems, but the most pronounced inflammatory changes in the skin and muscles.

In children and adolescents against the background of dermatomyositis, intramuscular, intraspecific and intradermal calcifications usually located in the projection of large joints, buttocks, shoulder girdle and the pelvic area.

Diagnostics

The main diagnostic criteria for dermatomyositis:

• clinical symptoms of damage to the muscular system and skin;
• characteristic pathomorphological changes in muscle fibers;
• electromyographic changes;
• increased activity of serum enzymes.

Auxiliary (additional) diagnostic markers of dermatomyositis include calcinosis and dysphagia.

The diagnosis of dermatomyositis is made when:

• Skin rash, combined with three any major criteria;
• skin manifestations, two main and two additional criteria.

To confirm the diagnosis, a laboratory and instrumental examination is carried out:

• a general blood test (an increase in ESR, leukocytosis with a shift of the leukocyte formula to the left);
• biochemical blood test (to increase the level of aldolase, transaminases, seromucoid, haptoglobin, sialic acids, myoglobin, fibrinogen, α2- and γ-globulins);
• immunological examination of blood (revealed the presence of nonspecific antibodies to the endothelium, myosin, thyroglobulin, increased levels of myositis-specific antibodies, a small amount of antibodies to DNA and LE cells, a decrease in IgA level with a simultaneous increase in IgM and IgG, a decrease in the number of T-lymphocytes, a decrease in the titer complement);
• histological examination of the musculoskeletal biopsy specimen (loss of transverse striation, inflammatory infiltration of myocytes, degenerative changes, severe fibrosis);
• electromyography (fibrillar oscillations are detected at rest, polyphasic short-wave changes, increased muscular excitability).

Treatment

Therapy of dermatomyositis is aimed at suppressing the activity of autoimmune inflammatory process and is usually carried out by corticosteroids with a long course (1-2 years). If necessary, the scheme may include non-steroidal anti-inflammatory drugs, in particular salicylates.

If corticosteroid therapy is ineffective, cytostatics with a pronounced immunosuppressive effect are prescribed.

To improve the contractile function of muscles use injections of Proserin, B vitamins, cocarboxylase, ATP.

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In recent years in the complex treatment of dermatomyositis began to use plasmapheresis, lymphocytapheresis.

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For the prevention of the formation of muscle contractures, regular sessions of physiotherapy exercises are shown.

Possible complications and consequences

In the absence of adequate therapy, dermatomyositis slowly progresses, leading to severe muscle weakness, damage to internal organs. This leads to disability of patients, and in severe cases - death.

Long-term corticosteroid therapy of dermatomyositis can cause a number of pathologies:

• arterial hypertension;
• obesity;
• osteoporosis;
• diabetes.

Forecast

In the absence of adequate treatment, about 40% of patients die in the first two years from the date of diagnosis; the cause is gastrointestinal bleeding and respiratory failure.

Immunosuppressive therapy significantly improves long-term prognosis. However, even against its background, some of the patients develop persistent joint contractures, deformities of the upper and lower extremities occur.

Prevention

Primary preventive measures for dermatomyositis have not been developed. Secondary prevention is aimed at preventing exacerbations of the disease and reducing the activity of the inflammatory process. It includes:

• sanitation of foci of chronic infection;
• restriction of physical activity;
• avoid excessive insolation and hypothermia;
• adherence to the daily routine;
• clinical examination of rheumatologist;
• careful adherence to the prescribed by the doctor scheme of drug therapy.

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A source: http://www.neboleem.net/dermatomiozit.php

Dermatomyositis

Dermatomyositis- diffuse inflammatory pathology of connective tissue with a progressive course, characterized by the damage of smooth and pseudo-striated muscle fibers with impaired motor functions, skin involvement, small vessels and internal bodies. In the absence of skin syndrome, the presence of polymyositis is indicated. Clinic of dermatomyositis is characterized by polyarthralgia, expressed by muscle weakness, fever, erythematous-spotted rash, skin calcifications, visceral symptoms. Diagnostic criteria for dermatomyositis are clinical, biochemical, electromyographic indicators. The main therapy is hormonal, the course of the dermatomyositis is undulating.

Dermatomyositis- diffuse inflammatory pathology of connective tissue with a progressive course, characterized by the damage of smooth and cross-striated muscle fibers with impaired motor functions, skin involvement, small vessels and internal bodies. In the absence of skin syndrome, the presence of polymyositis is indicated. Clinic of dermatomyositis is characterized by polyarthralgia, expressed by muscle weakness, fever, erythematous-spotted rash, skin calcifications, visceral symptoms. Diagnostic criteria for dermatomyositis are clinical, biochemical, electromyographic indicators. The main therapy is hormonal, the course of the dermatomyositis is undulating.

The etiological relationship of dermatomyositis with a viral infection (picornaviruses, Coxsackie viruses) and genetic conditioning is assumed.

Chronic persistence of viruses in muscles and antigenic similarity between viral and muscular structures causes an immune response with the formation of autoantibodies to muscle tissue.

Starting points to the development of dermatomyositis can serve as hypothermia, infectious exacerbation, stress, hyperthermia, hyperinsurance, drug provocation (vaccination, allergy).

Classification of dermatomyositis

Dermatomyositis and polymyositis belong to the group of idiopathic inflammatory myopathies. Secondary paraneoplastic (tumor) dermatomyositis occurs in 20-30% of cases. The course of dermatomyositis can be acute, subacute or chronic.

In the development of pathology, a period of nonspecific precursors (prodromal), clinical manifestations (manifest) and stage of complications (terminal, dystrophic, cachectic) are distinguished. Dermatomyositis can occur with varying degrees of activity of inflammation (from I to III).

The clinic of dermatomyositis develops gradually. At the beginning of the disease there is a progressive weakness in the muscles of the limbs, which can grow for years.

Acute onset is less common for dermatomyositis. The main clinical manifestations may be preceded by the appearance of skin rashes, polyarthralgia, Raynaud's syndrome.

There is a weakness in the muscles of the neck, proximal parts of the lower and upper extremities, which makes it difficult to perform daily activities.

With severe lesions, patients hardly lift themselves up in bed, can not hold their heads, move independently and keep objects in their hands.

The involvement of the musculature of the pharynx and the upper digestive tract is manifested by speech disorders, swallowing disorders, poperyvaniem; the defeat of the diaphragm and intercostal muscles is accompanied by a violation of the ventilation of the lungs, the development of stagnant pneumonia.

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A characteristic sign of dermatomyositis is the defeat of the skin with various manifestations.

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There is a development of periorbital edema, erythematous-spotted rash over the upper eyelids, in the region of cheekbones, nasolabial folds, wings of the nose, upper back, sternum, joints (knee, elbow, metacarpophalangeal, interphalangeal).

Typically, the presence of Gotthron's symptom - flaky erythematous spots on the skin of the fingers, flaking and redness of the palms, fragility and striation of the nails, okolonogevoy erythema.

A classic symptom of dermatomyositis is the alternation of foci of depigmentation on the skin and pigmentation in combination with telangiectasia, dryness, hyperkeratosis and atrophy of skin areas (poikilodermatomyositis).

With juvenile dermatomyositis, intradermal, intrafacial and intramuscular calcifications may appear in the projection of the pelvic, upper arm, buttocks, and joints. Subcutaneous calcifications can lead to ulceration of the skin and the release of calcium deposits outwards in the form of a crumbly mass.

Among the systemic manifestations of dermatomyositis, there are cardiac lesions (myocardiofibrosis, myocarditis, pericarditis); of the lung (interstitial pneumonia, fibrosing alveolitis, pneumosclerosis); Gastrointestinal tract (dysphagia, hepatomegaly); kidneys (glomerulonephritis), the nervous system (polyneuritis), endocrine glands (hypofunction of the adrenal glands and genital glands).

Diagnosis of dermatomyositis

The main diagnostic markers of dermatomyositis are the characteristic clinical manifestations of skin and muscular damage; pathomorphological transformation of muscle fibers; increased serum enzyme levels; typical electromyographic changes. Additional (auxiliary) criteria for diagnosis of dermatomyositis include dysphagia and calcification.

The reliability of the diagnosis of dermatomyositis is not in doubt with the presence of 3 basic diagnostic criteria and skin rash or 2 basic, 2 auxiliary criteria and skin manifestations.

The probability of dermatomyositis can not be ruled out when skin lesions are detected; with the totality of any 2 other main manifestations, as well as the combination of any main and 2 auxiliary criteria.

To establish the fact of polymyositis, it is necessary to have 4 diagnostic criteria.

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The picture on the part of the blood is characterized by moderate degree of anemia, leukocytosis, neutrophilic shift of the leukocyte formula to the left, the increase of ESR in accordance with the activity of the process.

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Biochemical markers of dermatomyositis are an increase in the level of α2 and γ-globulins, fibrinogen, myoglobin, sialic acids, haptoglobin, seromucoid, transaminases, aldolase, reflecting the severity of muscular damage tissue.

Immunological study of blood in dermatomyositis reveals a reduced complement fissure, a decrease in the number of T-lymphocytes, an increase in the level of IgG and IgM immunoglobulins as a result of a decrease IgA, a small number of LE cells and antibodies to DNA, a high content of myositis-specific antibodies, the presence of non-specific antibodies to thyroglobulin, myosin, endothelium, etc.

In the study of musculoskeletal biopsies, a picture of severe myositis, fibrosis, degeneration, inflammatory infiltration of muscle fibers, loss of transverse striation is determined.

On radiographs of soft tissues are visible areas of calcification; when lung radiography is determined by an increase in the size of the heart, calcification of the pleura, interstitial fibrosis of the lung tissue. In the bones, moderate osteoporosis is revealed.

When the respiratory muscles and palatine muscles are affected, it is necessary to ensure the functions of adequate breathing and swallowing.

To suppress inflammatory phenomena in dermatomyositis, corticosteroids (prednisolone) are administered under the control of serum enzymes and the clinical state of the patient.

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In the course of treatment, the optimal dosage of corticosteroids is selected, the drugs are taken for a long time (1-2 years). It is possible to carry out steroid pulse therapy. The anti-inflammatory scheme with dermatomyositis can be supplemented by the appointment of salicylates.

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In case of ineffectiveness of corticosteroid therapy of dermatomyositis, immunosuppressors of cytostatic action (methotrexate, cyclosporine, azathioprine) are prescribed.

To control skin manifestations of dermatomyositis, 4-aminoquinoline derivatives (hydroxychloroquine); for normalization of muscular functions - injections of neostigmine, ATP, cocarboxylase, vitamins of group B.

In the treatment of dermatomyositis, intravenous immunoglobulin, lymphocytapheresis and plasmapheresis sessions are used. In order to prevent muscle contractures, a complex of exercise therapy is prescribed.

Prognosis and prevention of dermatomyositis

With the running course of dermatomyositis, the lethality in the first 2 years of the disease reaches 40%, mainly due to the defeat of respiratory muscles and gastrointestinal bleeding.

With a severe prolonged nature of dermatomyositis, contractures and limb deformities develop, leading to disability.

Timely intensive corticosteroid therapy inhibits the activity of the disease and significantly improves the long-term prognosis.

A source: http://www.krasotaimedicina.ru/diseases/rheumatology/pseudotrichiniasis

Dermatomyositis: symptoms and treatment

Dermatomyositis is a chronic rheumatological inflammatory disease of muscles and skin. Another name for the disease is polymyositis, mainly it is used to refer to a disease 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. Most often ill children under 15 years and people older than 55 years. Women are sick twice as often as men.

In this article, we will examine the symptoms and treatment of this disease.

Why does dermatomyositis develop?

Dermatomyositis develops in people with a genetic predisposition.

Just as in the case of other rheumatological diseases, the main cause of the disease is not found. The connection with the following factors is supposed:

1. Chronic viral infection (eg, Coxsackie virus, shingles, influenza, rubella).
2. Malignant neoplasms, 30% of patients with dermatomyositis have an oncological diagnosis. It is important here as an autoimmune reaction (when the organism attacks both tumor cells and its cells), as well as the direct toxic effect of decay products of tumor cells.
3. Genetic predisposition. In individuals with dermatomyositis, an accumulation of HLAB8 is noted in the blood test, 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 onset of the disease or condition is unknown.
2. Primary idiopathic dermatomyositis.
3. Dermatomyositis in combination with tumors.
4. Dermatomyositis in combination with vasculitis.
5. Dermatomyositis in combination with diffuse connective tissue diseases.

Dermatomyositis in women

More often polymyositis affects women from 30 to 50 years. Typical manifestations: a gradual increase in symptoms, skin rashes, Raynaud's syndrome, arthralgia.

Dermatomyositis affects mainly women of the same age group, but, unlike polymyositis, the disease begins and proceeds acutely, there is a severe skin and muscle syndrome.

Dermatomyositis in children

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

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

Course of the disease

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

• Acute flow of dermatomyositis. Within six months, the patient is involved in the process of most of the muscles. Because of this, a person can no longer move, swallow himself, and sometimes speak. The patient suffers from fever and poisoning with toxic products of decay of his own musculature. The cause of death at this stage are aspiration pneumonia (for example, when vomit enters the lungs) or cardiovascular failure in the presence of a heart attack.
• Subacute current. Dermatomyositis recurs regularly, worsening the patient's condition. Gradually, the manifestations of internal organ damage increase. The patient also becomes immobilized over time. Against the background of specific treatment, a prolonged remission (periods of relative health) is possible. The duration of it depends on the patient's condition and on how carefully he carries out the medical prescriptions. With due attention to himself, the patient can live for many years, being only slightly limited in movements.
• Chronic course. The most favorable variant of the course of dermatomyositis. The disease affects only a few muscle groups, so the patient feels relatively well and is able to work productively and live a full life. Exceptions are young men, who can form large areas of calcification in the skin and muscles. This leads to the immobility of the limb or joint, and therefore significantly worsens the quality of life of the patient.

Symptoms of dermatomyositis

Muscular manifestations

1. Pain in the muscles during movement and at rest.
2. Myalgia, arising when pressing on a muscle.
3. The increasing weakness of the muscles, leading to disability of the patient.

   Over time, weakness grows so that the patient loses the ability to get up, sit down, and eat. In the end, it turns out to be completely immobilized.

4. The pathological process extends to the muscles of the face, so the patient is completely deprived of the opportunity to express their emotions with the help of facial expressions.
5. 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.

6. The defeat of the intercostal muscles and diaphragm leads to a violation of breathing, hypoventilation and the development of pneumonia.

Skin lesions

1. In 40% of patients on the open parts of the body (face, neck, limbs) there is erythema.
2. Eruptions such as papules and large blisters (bullae).
3. Teleangiectasia.
4. Hyperkeratosis (excessive keratinization).
5. Hyperpigmentation.
6. Purple swelling around the eyes - dermatomyositis eyeglasses.
7. Scaly red spots over the joints of the hands - Gottron's syndrome.

Raynaud's syndrome

Reynaud's syndrome is accompanied by numbness, a feeling of cold, a sense of creepy and accompanied by pain in the hands; in the period between attacks, the hands may remain cold and cyanotic.

In addition to the limbs, the manifestations of the syndrome can be noted in the tip of the nose, chin, earlobes and tongue. The duration of the attack ranges from several minutes to several hours.

Reynaud's syndrome occurs in 10% of patients.

Lesion of joints

1. When moving, there are pains in the joints, which torments and limits the patient.
2. Sometimes the muscles are affected so quickly and strongly that a person can not bend a hand in the elbow or a leg in the knee precisely because of the formation of "ankylosis of the muscular nature". Ankylosis is the impossibility of movement in the joint.

Disorders of the cardiovascular system

In 30% of patients, myocarditis develops (inflammation of the heart muscle).

It is manifested by rapid heart rate, reduced blood pressure, muffling heart tones and the appearance of systolic noise in auscultation, changes in the electrocardiogram (disturbances of excitability and conductivity).

Lung lesions

More often manifest as a complication of the disease. Basically, patients develop aspiration pneumonia or congestive pneumonia due to reduced ventilation and hypodynamia.

Lesion of the gastrointestinal tract

It occurs in 50% of patients. Anorexia, gastritis, colitis, abdominal pain are noted. Because of the defeat of smooth muscles, hypotension of the esophagus, edema and necrosis can develop in the walls of the stomach and intestines.

Diagnosis of dermatomyositis

Blood tests

Elevated 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 discovers a thickening of the muscle fibers and the necrosis zone in the tissue sample.

The diagnosis is made on the basis of the following symptoms:

1. Progressive weakness in the muscles.
2. Skin syndrome (typical for dermatomyositis manifestations).
3. Increased activity of muscle enzymes (high level of creatine phosphokinase).
4. Typical changes in muscle tissue established by biopsy results.

Treatment of dermatomyositis

Drugs of choice in the treatment of patients with dermatomyositis are glucocorticosteroids. These are substances with high anti-inflammatory activity. They are especially effective when prescribed in an adequate dose for the disease stage, preferably in the early stages.

The main glucocorticosteroid in the treatment of dermatomyositis is prednisolone. It is prescribed in tablets, in a dose of up to 100 mg per day, in 4-6 receptions.

Usually for 1-2 weeks of therapy with hormones, the patient's condition improves significantly: the voice's tone is restored, the patient ceases to swallow food, edema, pain and weakness in 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 state and avoid periods of exacerbation.

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

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Immunosuppressive drugs.

These drugs are prescribed to fight the immune system, which affects their own tissues. The medicine of choice is azathioprine. Assign it to 2-3 mg per kg of body weight of the patient. Azathioprine is usually introduced into the treatment regimen if therapy with one prednisolone does not give the desired effect.

Prognosis of patients with dermatomyositis

Mortality in this disease remains quite high. Everything depends on what kind of dermatomyositis it is and what current it is.

The worst treatment is dermatomyositis in cancer patients, but in this case, the severity of the condition and the severity of the prognosis are due more to the presence of the tumor than the damage to the muscles and skin.

To which doctor to apply

When the muscles are affected, it is necessary to consult a rheumatologist. Additionally, the following specialists are needed: infectious diseases specialist, oncologist, gastroenterologist, cardiologist, pulmonologist, dermatologist.

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A source: https://myfamilydoctor.ru/dermatomiozit-simptomy-lechenie/

Dermatomyositis: a fatal disease with unknown causes

With early detection of dermatomyositis, adult survival is 80% within the first 5 years and 73% within 8 years
If we do not meet examples of any disease throughout life, we think that they are not at all.

However, in the population of people there are very rare, little-known outside the medical community, diseases with unknown causes for modern medicine and a significant probability of death outcome.

One of these diseases - dermatomyositis - is devoted to this article.

General characteristics of the disease

Dermatomyositis is a complex disease of muscle tissue of unknown origin with characteristic cutaneous manifestations.

This disorder is rare:

• from 1 to 10 cases per one million adults;
• from 1 to a case for one million children.

Earlier recognition and therapy can reduce complications.

Correlation of this disorder with other connective tissue diseases, as well as with malignant formations, makes the delivery of the correct diagnosis extremely important for successful treatment. You can see the material about Lyell's syndrome.

The lack of a clear understanding of the causes of dermatomyositis is complemented by the described cases of disappearance of all symptoms without special therapy

The prospect of curing the disease is extremely small: the disease reacts poorly to therapy. The high probability of malignant neoplasms complicates therapy.

The etiology of dermatomyositis remains unknown. In studies with different degrees of probability,that the causes of the disease can be:

• genetic changes;
• environmental factors in the form of a virus, infection, drugs;
• wrong autoimmune reaction of the body.

The average age of adult patients at the time of diagnosis is 40 years. For 1 man with a diagnosis of dermatomyositis, there are 2 women with the same diagnosis.

The average age of minors diagnosed with dermatomyositis is 5 to 14 years. This group has a better therapeutic prognosis than the adult group.

Treatment of dermatomyositis requires a comprehensive approach of doctors in a number of specialties depending on the concomitant complications

Symptoms of dermatomyositis

In the scientific medical literature 87 symptoms of dermatomyositis were described.

Most of them are complex complications associated with this disease. Consider the most important symptoms in more detail. You can get acquainted with the material on the prevention and treatment of rhinophyma.

The main symptoms of dermatomyositis include two groups:

• skin manifestations,
• muscle weakness.

In the scientific medical literature 87 symptoms of dermatomyositis were described. Most of them are complex complications associated with this disease

As a rule, these symptoms are not simultaneous. First, there are skin lesions, then - muscle damage.

1. Skin changes manifest themselves in the form of a symmetrically located purple or red (from light to dark tones) rash, localized on the face, areas around the nails, on the articular folds of the fingers, elbows and knees, and also on the chest and back. These changes are the essence of Gottron's symptom. Skin manifestations of the disease significantly precede the defeat of the muscles (with a difference of several months to several years).
2. Muscle weakness, as a rule, takes place in the shoulder girdle, the pelvic region, affects other skeletal muscles. This symptom progresses, eventually leads to difficulties in the implementation of daily activities, for example, in movement or in manipulation of hands. Muscle weakness also affects the muscles of the throat, esophagus and lungs. In the complex, a muscular symptom can lead, in addition to the list enumerated above:

• to dysphonia (change or disappearance of the voice);
• to dysphagia (difficulty or inability to swallow);
• to difficulty breathing and other problems of the respiratory apparatus;
• to muscle pain.

The average age of adult patients at the time of diagnosis is 40 years

Associated symptoms of dermatomyositis include:

• cardiomyopathy;
• myocardial infarction;
• pneumonia, developing against the background of weakness of the respiratory muscles;
• fibrosis;
• atrophy of muscle tissue;
• calcification of muscle tissue;
• joint damage, leading to pain and mobility limitation;
• lesions of the gastrointestinal tract;
• inflammation of the eyes, nystagmus, optic nerve atrophy and other eye complications;
• malignant neoplasms.

Previously, recognition and therapy can reduce complications

Diagnostics

1. The blood test shows a high concentration of muscle enzymes of the creatine kinase and aldolase, which indicates muscle damage.
2. Immunological research can detect specific antibodies that indicate an abnormal autoimmune reaction.
3. Magnetic resonance imaging allows you to diagnose the presence of inflammation of muscle tissue.
4. A biopsy of the muscle tissue as an instrumental method of confirming the diagnosis.

Muscular tissue of a person (video)

Treatment

Treatment of dermatomyositis requires a comprehensive approach of doctors in a number of specialties, depending on the attendant complications.Treatment if necessary includes three components:

• physical therapy;
• drug treatment;
• surgical intervention.

Dermatomyositis is a complex disease of muscle tissue of unknown origin with characteristic cutaneous manifestations

Look at each item in more detail.

1. Physical therapy is aimed at preventing muscle atrophy, especially relevant for patients with calcification. It can be focused on passive stretching of muscles or it can include active physical exercises that counteract the increasing atrophy. When the muscles of the throat are affected, special exercises are prescribed to compensate for degenerative changes.
2. Medical treatment of dermatomyositis includes:

• immunosuppressive treatment;
• treatment with drugs specifically directed at certain functions of the body, against specific complications, etc .;
• intravenous immunoglobulin, containing healthy antibodies that can block own antibodies that damage the muscles and skin of the patient - effective action is required regularly;
• surgical intervention is used, for example, to remove painful calcium deposits;
• hormone-dependent treatment with corticosteroid drugs.

Forecast

The lack of a clear understanding of the causes of dermatomyositis is complemented by the described cases of disappearance of all symptoms without special therapy.

In these cases, muscle weakness, cutaneous manifestations and other concomitant symptoms of dermatomyositis slowly developed, after which they disappeared by themselves and did not recur.

In overwhelming cases, dermatomyositis is a disease that is difficult to treat. Malignant neoplasms, cardiac and pulmonary insufficiencies, infections represent the most common causes of death from this disease.

With early detection of dermatomyositis, adult survival is 80% for the first 5 years and 73% for 8 years. For the children's group of patients, the death rate is lower - from 3% to 10% - while 2/3 children develop severe complications in the form of calcification.

In conclusion, we note the trivial truth, which, as never before, is relevant for this disease. Early diagnosis at the stage of skin dermatitis makes it possible, if not cure, then a significant slowdown in the progress of dermatomyositis, as well as periods of prolonged remission.

A source: http://AntiRodinka.ru/dermatomiozit-smertelnoe-zabolevanie-s-neizvestnimi-prichinami

How to treat dermatomyositis

Dermatomyositis or Wagner's disease is a severe autoimmune disease in which the connective tissue of a person, in particular his muscular tissue, is damaged.

Juvenile dermatomyositis often develops, from which children and adolescents suffer.

In this pathological process, there is a weakening of the motor activity, a disruption of the normal operation of internal organs, and the destruction of small vessels.

There are also characteristic skin manifestations of the disease in the form of rashes. The disease is characterized by a gradual increase in symptoms characteristic of dermatomyositis, and treatment should be cyclic and aimed at reducing manifestations of the disease.

Anti-inflammatory drugs are used for therapy. You can treat the disease with folk remedies. Such therapy is safer and does not cause side effects, because it is based on medicinal herbs.

Causes of the disease

The disease develops in men and women. Juvenile dermatomyositis occurs in children and adolescents aged 4 to 14 years. In adults, the peak incidence is 45-60 years.

Wagner's disease or dermatomyositis is a diffuse autoimmune disease in which the connective, in particular, muscle tissue is affected. With dermatomyositis, immune complexes are developed against cells of smooth and striated muscle.

The exact causes of the disease are not established. There is a theory that the disease develops after a viral infection. This is due to the fact that viral antigens are similar to antigens of the human muscle tissue.

It is also believed that there is a genetic predisposition to the development of dermatomyositis.

The autoimmune process proceeds wavy. Exacerbation of pathology can occur against the background of the action of the stress of stress factors:

• hypothermia or overheating of the body;
• infectious disease or exacerbation of a chronic illness;
• stress, emotional stress;
• vaccination;
• exacerbation of the allergic process.

Dermatomyositis in children is a fairly rare pathology. More often such a disease occurs in southern Europe. Exacerbation of dermatomyositis occurs frequently in the spring-summer period. It is believed that solar radiation can provoke it.

In general, the causes of juvenile dermatomyositis are the same as those of adults: infectious, in in particular, viral diseases, exacerbation of chronic infections, allergic processes, action stress. The disease is considered severe and in children it can be more acute.

Without proper treatment, a child may die.

Symptoms of dermatomyositis

The disease is characterized by a paroxysmal course, the symptoms gradually increase.

Some patients report an acute onset of the disease, but on the whole this manifestation of pathology is not typical. Symptoms of dermatomyositis can grow for years.

Even with acute onset of the disease over time, the symptoms are smoothed out, and dermatomyositis passes into a chronic form with periodic exacerbations.

With acute and subacute start of the process in the first days, a person has pain in muscles and joints, and there are swelling. Also note the increase in temperature.

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Symptoms of the pathology are mainly associated with the defeat of the musculature.
A symptom characteristic of the disease: it is the muscular weakness of the arms and legs, which gradually increases.

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Another characteristic feature is an increase in the muscle volume of the upper and lower extremity belts. In this case, a traffic violation occurs.

It becomes difficult for a patient to sit down and get up, raise arms and legs, lift and turn his head.

In severe cases, a person can completely stop moving.

If in the autoimmune process the facial muscles have suffered a defeat, the person's facial expression is disrupted, his face does not express emotions and resembles a mask.

Dysphagia can also occur with the defeat of swallowing muscles.

If the inflammation develops on the oculomotor muscles, visual disturbances occur.

At the first stages of the development of the disease, edema of the muscles develops, the person experiences pain. Immune complexes cause cell destruction. This leads to progressive degeneration and atrophy of muscle tissue. Muscle fibers are replaced with fibrous tissue.

Sometimes a person develops calcinosis - the deposition of calcium salts in tissues. This process can extend to subcutaneous fatty tissue.

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Such deposits of salts can break out, forming ulcers on the skin with the release of the contents in the form of white crumbs.

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Often this symptom is accompanied by juvenile dermatomyositis, which develops in children and adolescents.

In addition to muscles with dermatomyositis, joints can be affected. The person experiences pain, the mobility of the joint is limited.

The defeat of the skin is manifested by characteristic symptoms:

• rash on the face, chest, upper back and around the joints;
• swelling of the eyelids;
• redness and flaking of the skin of the fingers and hands;
• fragility and stratification of nails, redness of the skin around the nail plate;
• the skin becomes spotty: foci of increased pigmentation alternate with
• depigmented skin;
• dry skin;
• skin atrophy.

Also, the mucous membranes are affected. The patient can develop:

• conjunctivitis;
• stomatitis;
• inflammation and swelling of the mucous membranes of the mouth and larynx.

In addition to striated muscles, smooth muscles and muscle tissue of the heart are affected. As the disease develops, a person can develop:

• heart failure, non-infectious inflammation of the heart or myocardium envelope;
• lung damage caused by hypoventilation: pneumonia, sclerosis of lung tissue;
• disruption of the gastrointestinal tract: increased liver, impaired swallowing;
• kidney damage and the development of the inflammatory process in them;
• inflammation of nerve fibers;
• dysfunction of the glands of internal secretion and hormonal imbalance.

Classification of the disease

Excrete acute, subacute and chronic dermatomyositis.

In acute disease, the patient develops a generalized lesion of the striated musculature. Often this leads to complete immobility, disruption of the breathing process and swallowing.

The patient raises the temperature, the symptoms of intoxication increase, skin manifestations appear. Without proper therapy, a fatal outcome occurs in a few months.

The cause of death is pneumonia or cardiopulmonary insufficiency.

A subacute type of disease is characterized by a cyclic course with a gradual worsening of the symptoms of the disease. The patient is afflicted with damage to the muscles and internal organs.

The increase in symptoms occurs slowly enough, and the patient maintains his long-term ability to work.

But it is important to conduct adequate treatment of the disease, otherwise the development of symptoms will gradually lead to impaired motor activity or complete immobility.

Diagnosis of the disease

Suspected dermatomyositis can be based on the characteristic symptoms of damage to muscle tissue and skin manifestations. To confirm the diagnosis, a laboratory blood test is performed. The analysis shows the development of the inflammatory process and anemia.

Immunological study shows the presence in the serum of antibodies to antigens of the human muscle tissue.

To confirm the diagnosis, a muscle biopsy is performed, which shows the fibrosis and destruction of the fibers and the presence of an inflammatory process in the muscles.

Treatment of dermatomyositis

Autoimmune diseases are extremely difficult to treat. Traditional medicine recommends treating the disease with drugs that suppress immunity.

Against the backdrop of such therapy, secondary infectious diseases often develop, since the defenses of the body are not enough to cope with the infection.

There is also a popular treatment of the disease. Therapy with folk remedies is safe, because it is based on natural products and medicinal herbs.

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With dermatomyositis treatment is aimed at reducing the inflammatory process, for this purpose take decoctions of medicinal herbs, and also use external means.

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Admission of folk potions does not cause side effects, it is recommended to take them during the remission period to prevent recurrence of the disease.

Decoctions for oral administration:

1. Oats. 2 cups of oats are added to 5 cups of boiling milk, simmer for 3 minutes, then insist for 2 hours and filtered. Take 100 ml of this decoction three times a day. The treatment lasts a month, then make a one-month break and repeat the course.
2. Cowberry. In 200 ml of boiling water steamed 1 tbsp. l. leaves of cowberry, boil on low heat for 5 minutes, the loan is cooled and filtered. Drink all the broth during the day. Treatment lasts 2-3 months.
3. Hop. In 200 ml of boiling water steamed 2 tbsp. l. shredded hop cones, insist 2 hours, then filter. Drink 50 ml of infusion 3 times a day.
4. Dandelion. In therapy, the roots of this plant are used. 1 tbsp. l. chopped roots pour 1 cup boiling water, boil over low heat for 5 minutes, then insist 2 hours and filter. Take 100 mg of the drug twice a day. The therapy lasts three weeks, after which they do a weekly break and repeat the course.

Outdoor folk remedies:

1. Willow. Fresh leaves and buds of this plant are ground and poured with boiling water (100 ml of water per 1 tbsp. l. vegetable raw materials), insist the hour. Use this tool for compress on the skin in places of manifestation of the disease.
2. You can also prepare ointment based on willow buds. 100 g of oil is melted, 2 tbsp. l. crushed willow buds, kept in a water bath for 15 minutes, then poured into a glass container. Ointment is stored in the refrigerator and rubbed into affected areas of the skin twice a day.
3. Altey. 1 tbsp. l. roots of this plant cut and poured 100 ml of boiling water, insist half an hour and used for compresses.
4. Tarragon (tarragon or wormwood tarragon). Prepare ointment from the seeds of this plant. 100 g of interior jtut is melted in a water bath and the ground tarragon seeds are added in the ratio:. The mixture is kept in an oven at 150 ° C for 5 hours, then filtered. Store the ointment in a glass container in the refrigerator. Lubricate affected areas of the skin three times a day.
5. In a similar way, you can prepare ointments from cocklebur, celandine and linseed. They are also used to lubricate the affected skin.

Important in the treatment and way of life.

1. To slow down the course of the disease, you need to exercise physical exercises on the muscles, move more, if possible, play sports.
2. Nutrition of the patient with dysphagia should be wiped and liquid. It is recommended to refuse to eat after 7 pm.
3. Provoke an exacerbation of the disease can ultraviolet study. Such patients are prohibited from going to the solarium and being in the sun during the period of maximum activity (from 11 to 17 hours).

Prognosis and prevention

If the disease is not treated, then the death in the first two years occurs in 40% of cases. The cause of death is a violation of breathing, pneumonia or heart failure.

If the smooth muscles of the internal organs are affected, gastrointestinal bleeding may develop.

Long-term course of the disease leads to disruption of motor activity, disability or complete immobility.

Unfortunately, there is no specific prophylaxis of the disease, because it is not exactly established what is the cause of this pathology.

For prevention purposes, it is important to prevent the development of viral infections. To provoke the disease may exacerbation of a chronic disease and allergic process.

It is important to identify and treat these diseases in a timely manner.

A source: http://nmed.org/dermatomiozit.html