Diseases connective tissue - This is quite a frequent phenomenon. One of these diseases is dermatomyosis. Dermatomiosit can occur at any age. It does not depend on sexuality - manifests both in women and men. Symptoms of dermatomyositis are diverse. Dependes on which organs and systems are affected by the pathological process. Modern medicine allows you to diagnose this disease in the early stages when the designated treatment will be most effective.

Show all

What is it?

Dermatomyositis is an inflammatory disease of the connective tissue. With this pathology, smooth muscles, skeletal muscles and leather are affected. Often painful changes are subject to internal organs - systemic inflammatory disease of the skeletal and smooth muscles and skin; less often notes involvement in the pathological process internal organs.

If pathological changes do not affect the skin, they talk about polyimosit.

A year is fixed from two to ten cases of a dermatomyositis per 1 million people. More often, women suffer from this disease. If we talk about the dermatomyosit, which arose in childhoodHere, here the ratio of sick boys and girls is the same. More often, dermatomyositis manifests in the pre-career period or in the elderly.



Causes and mechanism for the development of the disease

There is no unambiguous answer to the question about the causes of the dermatomyositis. Currently, doctors are assumed that an important factor is infectious agents. It has been established that the peak of the dermatomyositis is falling on the ORVI epidemic season.

There are indisputable data on genetic predisposition to this pathology. But it is important to understand that the disease itself is inherited, but immune disorders that can lead to dermatomyositis.

Pathological immune responses contribute to the development of dermatomyositis. In the dermatomyositis, the muscle tissue is in a state of "oversaturation" of immunity cells. In the "failure" mode, both cellular and humoral immunity works.

Activated macrophages, T-lymphocytes and in-lymphocytes, antibodies infiltrate the muscle. T cells have the properties of toxicity against myocytes (muscle cells). In turn, the reactions of humoral immunity lead to damage to the microcirculatory vascular bed of muscle tissue.



Symptoms and clinical picture

The clinic of dermatomositis is diverse and does not have clear characteristics during the manifestation.

Most of the diseases are marked:

• unsatisfactory overall well-being;
• prostration;
• the feeling of weakness in the muscles, which increases for several weeks;
• skin lesion.

In children and in youthful age, there is more often a sharp field of illness.

Arise:

• severe muscular pain;
• temperature rise to febrile values;
• fast loss of body weight.

Symptoms with antisyestase syndrome:

• lung damage;
• damage to hands;
• fever;
• symmetric arthritis.

Rare symptoms of the onset of the disease:

• defeating the muscles of the pharynx and larynx (difficulties in playing sounds, swallowing);
• the emergence of aspiration pneumonia.

These symptoms by mistake can be taken for violation of blood circulation in the brain.





Muscle defeat

The main sign of this rheumatological disease is the growing weakness of muscles.

Muscular disorders during dermatomyositis are presented in Table:

Sick appear growing pain in muscles, weakness.

In the absence of qualified treatment, muscle tissue atrophy is developing.

Leather

One of the components of the dermatomyositis is the damage to the skin.

Skin manifestations of dermatomyositis are presented in the table:

Sustaines

More often amazed small joints, less often - elbow and knee. Sometimes this feature is mistakenly regarded as a manifestation of rheumatoid arthritis.

In the absence of treatment, it is sometimes developing deforming arthritis with joint ventrics. But there are no erosion on the radiograph.

Calcine

It is characteristic of juvenile dermatomyositis, more often than later stages of the disease.

Calcium inclusions are localized:

• around muscular fiber;
• in subcutaneous tissue;
• over elbow joints, knees, finger joints in their trauma;
• in buttocks.

Respiratory system

The breathing apparatus is attributed to: the pathology of the lungs, pleura and respiratory muscles.

Pathology of respiratory organs:

Symptomatic man feels shortness of breath, breathing failure, progressive cough.

Cardiovascular and urinary system

As a rule, the symptoms of the lesion of the heart and blood vessels is absent. Sometimes during the examination, arrhythmias and conductivity disorders are found.

Myocarditis and fibrous changes in the heart muscle are rare. They can manifest itself a stagnant heart failure. The vascular defeats include: Reyno syndrome, phetechial rash, infarction of the vessels of the olomogte bed.

Renal pathology occurs infrequently. The development of nephrotic syndrome and renal failure. The initial changes are manifested by proteinuria.

Diagnostic events

Diagnosis of the patient with suspicion of dermatomyositis includes:

• conversation with patients;
• inspection;
• laboratory diagnostics (general blood test, general urine analysis, biochemical blood test);
• immunological analysis;
• magnetic resonance tomography;
• needle electromiography;
• muscle biopias.

Diagnostics of dermatomyositis:

Research method	What changes are detected
General blood analysis	No specific changes, it is possible to increase the erythrocyte sedimentation rate (ESO) and anemia of easy degree
General urine analysis	The presence of a protein in the urine
Blood biochemical study	Increased KFK level (creatinophosphokinease), KFK-MV, hepatic enzymes
Immunological analysis	Specific antibodies are found
Needle Mioelectronography	Fixed spontaneous muscular activity
Magnetic resonance Tomography (MRI)	Muscle swelling is revealed
Biopsy	In the muscular bioptate there is infiltration of mononuclears and local necrotic changes; During the launched state, there are vessel thrombosis, substitution of myocytes for fat cells, muscle atrophy

Diagnostic criteria of dermatomyositis:

Criteria number	Criterion	Signs of defeat
1	Skin lesion	Grotton syndrome. Erythema on the extensitive surface above the elbows and knees. Heliotropic rash
2	Increased KFK or Aldlase	Confirmed by laboratory analyzes
3	Malgia	Pain in muscles at rest, with a strengthening during palpation
5	Moogenic pathology	Spontaneous reductions in muscle fiber at electromyography
6	The presence of specific antibodies	Confirmed by immunological analysis
7	Arthritis without destructions for radiograph	Sustav pain
8	Signs of general inflammation of the body	Resistant body temperature increase. Increase the rate of sedimentation of red blood cells, etc.
9	Confirmed laboratory Miositis	Availability of infiltrate, edema, necrotic cells, muscle atrophy

The diagnosis of "dermatomyomy" is raised if there is at least one sign of skin lesion and four and more other criteria. For polyimosit, it is necessary to have four or more criteria without damage to the skin.

Treatment

Treatment of dermatomyositis must be comprehensive. Apply basic therapy, auxiliary therapy and rehabilitation methods.

Basis therapy implies glucocorticosteroids short action. This preparation is prednisone.

Doses of the drug and treatment diagram is assigned only by a doctor!

If after a month of treatment of positive dynamics is not observed, then the doctor increases the dosage of the drug. When the effect of the prednisiolon dose is reduced to the supporting level. With the juvenile mositis and the rapid progression of dermatomyositis in adults, pulse therapy is used.

• cytostatic (methotrexate, azathioprine, cyclophosphamide, plaque, etc.);
• immunosuppressants (Mikofenotala Mofetin);
• immunoglobulins;
• fNO-ALPA inhibitors;
• calcium preparations.

Sometimes plasmapheresis use.

Rehabilitation and forecast

Rehabilitation activities depend on the stage of the disease. They are represented various species medical physical education And aimed at relaxation and strengthening muscles.

Dermatomiositis is a solved problem of modern rheumatology. Five-year survival with dermatomyositis is 90%. With timely appeal for medical help, the forecast for life is favorable. It is important that the treatment of dermatomyositis is qualified and adequate to the stage of the disease.

Dermatomiositis (cipher M33.0, M33.1 and M33.9 in the ICD-10) - systemic disease from the group of pathologies of connective tissue. In the process of its development, the functionality of skeletal muscles, joints, internal organs is worst, muscles are destroyed, bones are deformed. During the dermatomyositis, symptoms on the skin (photo) immediately appear, and the treatment begin immediately, otherwise a person dies or becomes disabled.

a brief description of

Dermatomyomyomy is a disease at which skin tissues are inflated, smooth-leasive fibers, skeletal muscles. Later they are destroyed, atrophy. This causes disorder of the functions of the joints, spine, as well as vessels, internal organs. These consequences of the disease can end with massive bleeding, respiratory failure or complex infectious pathology.

The causes and pathogenesis of such a disease as a dermatomyodeism to the end are not studied. Doctors are inclined to viral, autoimmune and genetic etiology of the disease. The provoking factors include the infection of the cokesaw (CoxSsackievirus), hormonal disorder, neoplasms, long-term supercooling or overheating, drug intolerance.

Varieties of dermatomyositis

Doctors use the generally accepted separation of the disease for the flow on chronic, protracted, sharp or shape. According to stages, it is distinguished by the long-standing, manifest and terminal periods.

Dermatomiositis has such varieties:

• amiopathic (absent muscular syndrome);
• primary;
• youth (juvenile);
• tumor (combined with neoplasm, paranoplastic);
• associated with connecting tissue diseases;
• associated with vasculitis.

The intensity of the development of the pathological process allocate four classes. During remission it is missing (zero degree of activity), when chronic flow The minimum (III) or average pronounced (II), and in the acute period - high (I).

Symptoms of dermatomyositis

For typical dermatomyositis, extensive inflammatory-degenerative changes in the tissues of the epidermis and muscles are characteristic. Acute or prostrate the form suddenly develops. Inflammation covers all the muscles of the body in the first 6-12 months from the beginning of the prodromal period. This disrupts the work of the internal organs, which entails the connection of secondary diseases.

Acute dermatomyomyomy manifests:

Chronic dermatomaisuit is characterized by many years of development, a wave-like light or medium-free course of the disease during relapse and long-term remission. Distrophic changes of muscles predominate from symptoms, pyachilodermia, contracture and calcification.

Clinical manifestations of dermatomyositis

In a typical dermatomyositis, doctors detect skin, muscular and articular syndromes. The patient marks spilled pain in articulations and body, deterioration of motor functions, a bluish-purple rash, violation of the internal organs.

Clinical signs that are distinguished by dermatomyosis:

• inflammation of muscles (myiositis);
• photodermatitis;
• swelling, redness of the skin (symptom of points);
• vascular meshes, bruises;
• dark spots;
• depigmented skin sections;
• thickening of the epidermis;
• erythema at open areas of the body and / or under the hair;
• gottron syndrome (tubercles and peeling reddish stains on the fingers, hips and the legs);
• excessive drying of the skin on the palms.

The dermatomyositis is also observed fragility and / or the allocated nails, the growth of the cuticle. With inflammatory processes, the swelling appears on their hands, legs, the face becomes thought-out and reminds the mask.

In the event of damage to the muscles of the internal organs:

The common symptoms of dermatomositis include both Schogren syndromes, Reyno. They manifest themselves with increased dry mucous eye, respiratory tract, Gastrointestinal tract, circulatory disorders in feet and brushes.

Muscle destruction during dermatomy

Muscles are destroyed due to prolonged inflammation of their fabric. Man begins to bend limbs with difficulty, lean, get up from bed. Later, due to the muscular weakness, the work of the speech apparatus worsens, the tone of the muscle of the esophagus disappears, the functions of the vessels, hearts, kidneys and other organs disappears.

The destruction of muscle structures is accompanied by pain syndrome during rest and when driving. There are also signs of intoxication: indisposition, temperature rise, chills. The pain in the muscles increases during physical exertion or when the body is pressed.

Other symptoms

In the acute phase in adults less often than in children, fever begins and the temperature is raised above 38º C. When dermatomyositis can fall out, shortness of breath, swallowing, respiratory functions are worse, dizziness and other signs of hypoxia appear.

Other symptoms during dermatomyositis:

• pain in all joints;
• reducing the amplitude of movements due to contracture;
• deterioration of diction and swallowing function;
• secondary diseases (pneumonia, body failure, gastritis, and so on);
• reducing body weight.

During dermatomyosit, they are also tested under the skin of a tubercle, or a seal is noticeable on the radiograph. These are calcium deposits formed in the tissues of the epidermis, inside the muscles, along the structures of the ligorous tendon apparatus. A ulcer can appear around the salt approval.

How the dermatomyode is manifested in children

In a child, against the background of intensive growth, symptoms of the disease is pronounced than adults. With juvenile acute dermatomyositis in children, the temperature increases to 39º C, high muscle fatigue, fever appears. The child also has a sharply reduced weight, bright rash arises on the skin or spots, similar to pink deprived or dermatitis.

In children, there are very pronounced inflammation, accompanied by severe pain in all muscles and joints even at rest. The child can not move, blend elbows, knees, raise her head over the pillow, he swells the joints. With juvenile dermatomyositis faster and more often than in adults, calculations are formed, vasculitis develops. Disease is clearly associated with DR 3, NLA B8 markers.

Diagnosis of dermatomyositis

With dermatomyosit medical help Contact rheumatologist. The patient must examine the Andrologist (men), gynecologist (women), urologist, cardiologist, angiologist, neurologist, other profile doctors.

Diagnostic methods:

• biochemical studies of urine, blood;
• blood test with oncomarckers;
• general urine tests, blood;
• immunological tests;
• eNMG electromyography;
• biopsy of inflamed muscle tissue, histological studies;
• P-spectroscopy;
• CT or MRI;
• radiography.

During the studies, calcinities, follicle dystrophy, dysfagia, deterioration of collagen structures, changing the sebaceous glands are found. From external signs celebrate edema around the eyes, appearance vascular stars, spots on the skin (erythema, pigmentation, rash).

Other diagnostic signs that are distinguished by dermatomyosis:

The diagnosis of dermatomyosis confirms the presence of skin and muscle syndrome. If there are no changes in the tissues of the epidermis, it means that the patient develops polyimiositis.

Dermatomiositis: Treatment

When dermatomyosit, complex treatments are used: medicines, physiotherapy, diet, surgical interventions, classes with a speech therapist (if swallowing muscles are affected). In Moscow, use genetically engineering biopreparations. Folk ways are ineffective: with the permission of the doctor, they are used in conjunction with drugs for the prevention of relapse.

Medicia treatment

In medicinal therapy, pharmaceuticals are used with immunosuppressive, pathogenetic and anti-inflammatory effect. These are glucocorticosteroids (cannot be replaced by NSAIDs), cytostatic, aminohinoline medical products, anabolics, immunoglobulin, folic acid.

The basic and supporting therapy is used for 3-5 years. Medicines suppress autoimmune inflammation in muscles, skin, organ tissues, prevents the formation of calcinates, restores microcirculation and cellular metabolism.

What medicines are used in the treatment of such a disease as dermatomyomyosis:

During the course you need to alternate high and maintenance dosages of medical preparations. Medicia treatment Terminated after the rescue is shown when the year and no longer manifests the symptoms of dermatomyositis and the analyzing indicators do not deteriorate.

Physiotherapeutic treatment

Physiolesis is used after eliminating exacerbation (pain, inflammation). It warns relapse, muscle dystrophy or contracture.

What methods of therapy are prescribed in the damping phase of dermatomyositis:

To secure the results of the main therapy of dermatomyositis, it is recommended to relax in the sanatorium. In healthy, it is necessary to make rape, sulfur and other healing baths, to engage in water gymnastics, passing common massage sessions and use other physiotherapy methods.

Surgical treatment of dermatomyositis

Surgical methods of treatment corrected the effects of dermatomyositis caused by disabilities. Operations are carried out to eliminate muscle contractures, removal of calcinates or neoplasms, as well as in order to purify necrotic sites and other infectious foci.

Forecast Dermatomiota

Today, the forecasting output forecast for juvenile dermatomyositis decreased from 11% to 1.5%, and cases of deep disability - from 16.9% to 5%. In other children, the action of medicines are achieved by a resistivity or full cure. These indicators have achieved thanks to a timely examination, improvement of equipment and medical preparations.

If the patient was engaged in self-medication without proper diagnosis, the dermatomyode is rapidly progressing and 40% of patients dies from internal bleeding or respiratory failure. Immunosuppressive therapy increases a favorable survival forecast, but does not prevent the deformation of the limbs. In tumor form, the patient's life depends on the effectiveness of oncotherapy.

Dermatomiota prevention

When dermatomyosit, it is necessary to support the treatment with glucocorticosteroids, anti-inflammatory preparations, vitamin and mineral complexes. A person with this diagnosis cannot be missed by a control medical examination at the rheumatologist, an oculist, a dermatologist, it is recommended to regularly donate blood for analyzes on oncology.

1. It is necessary to treat inflammatory pathologies in a timely manner, to make the prevention of ARVI, on the recommendation of the doctor to purify infectious foci.
2. Need to do a massage, exercise physical leaps and swimming with gentle exercise.
3. A man with a dermatomyositis can not smoke, overheat in the sun or hypother, women are not recommended to do abortions.
4. It is necessary to eat more protein food, limit the consumption of salt, carbohydrate products.

The long-term use of glucocorticosteroids during dermatomyositis can be complicated by obesity, hypertension, diabetes or ostepode. Therefore, preventive measures are also taken against their development.

Among the diseases of the connecting tissues there is a formidable pathology - a systemic dermatomyroid, which is also called the disease of the Wagner or a purple disease ( last name due to characteristic purple skin of skin spots and erythem). Her danger in defeat is not only skin, but also muscles (both skeletal and smooth), internal organs (heart, lungs, gastrointestinal, endocrine glands), vessels and nerves. Consider the dermatomyositis, its symptoms and treatment, but at first a photo: brush hands with a sick with a dermatomyositis.

What is dermatomyosis (DM)

Dermatomiositis is a rare inflammatory disease of soft tissues with skin manifestations.

DM is sick several people out of 100,000, and adults are twice as much more often than children, and the exposure of women of this disease is somewhat higher. More accurate figures: children are about three people. out of 100 thousand; Men are about 6 out of 100 thousand.

Causes of dermatomyositis

It has a greater spread of dermatomyositis in southern and south-European countries in spring and summer, because of which the assumption is made about the possible effect of sunlight on the development of pathology. Also, empirical observations helped establish a connection between infectious diseases, vaccinations and some drugs and developing after a while (hidden period can reach three months) dermatomyositis.

It was found that the development of DM contributes to:

• of viral diseases - influenza, parvovirus, hepatitis B and a, lush, poliomyelitis, rhinitis;
• bacterial - borreliosis, hemolytic streptococcus;
• vaccinations - Cory vaccinations, pigs, typhoids, cholera;
• medicinal preparations - Penicilline, STG (somatotropic hormone).

Types of dermatomyositis

Dermatomiositis is classified by the type and nature of the flow.

• idiopathic (the cause of primary pathology is unknown);
• paranoplastic (caused by the oncological process);
• juvenile or children's (observed mainly in adolescence and childhood);
• polyimiosit (without skin manifestations, but with symptoms of diffuse damage to many tissues);
• dermatopolimiosit (present both skin lesions and other systemic fabrics).

By the nature of the flow:

• acute (with a rapid increase of symptoms);
• subacute (moderate symptomatomy);
• asymptomic (without explicit clinical signs, the diagnosis is established on the basis of x-ray and functional diagnostics);
• chronic (slow development of the disease).

Symptoms of dermatomyositis

DM usually begins with skin lesion. Symptoms of muscle pathologies can join in a few months, or even years. At the same time, the development of myositis in the skin and muscles is nonsense, that is, an exceptionally rare phenomenon.

The main signs of DM is a rash different types.

• The symptom of the Gottron is a nodule or plaques, sometimes with the symptoms of peeling, or large spots of red or pink, localized on bending articular surfaces, mainly hands (interphalangeal or millatoplange) brushes, as well as elbow and knee joints.
• Heliotropic lilac-colored rash by type of glasses near the eye, under the eyebrows, or mantle on the top area of \u200b\u200bthe limbs, back and clavicle. Skin rashes are also possible on the abdomen, buttocks, hips and lower legs. There are also dark burgundy branching erythema.
• The initial symptom of dermatomyositis is redness and swelling of the skin at the borders of the nails.
• In dermatomyositis, burdened with calcine, purulent wounds appear, filled with a crumb-like mass - a sign of depositing calcium deposits under the skin. Calcia is observed five times more often at juvenile dm.

Symptoms of polymositis (PM)

Polyimiosit is capable of most often hit the muscles of the skeleton, joints, light, heart, endocrine system.

In the connecting tissues, structural transformations begin, they are compacted, smallers, and then calcification can join this process. The formation of calcium salts sediments may occur quite deeply and not to appear externally, but calcine always gives a typical picture of single or multiple nodes on an x-ray.

Defeating skeletal muscles

• With the damage to skeletal muscles, which occurs mainly symmetrically, muscle atrophy develops in the patient, muscle fibrotization and education of contractures are possible.
• They suffer more often shoulder, hip and abdominal muscles (mostly press), as well as cervical flexors. This is manifested in difficulties in certain movements requiring efforts, such as lifting up the stairs or attempt to rise from the seating, especially with low.
• The most unfavorable form of muscular polyimositis with the lesion of the respiratory muscles. At the same time, besides the threat of respiratory failure, the patient changes the voice (hoarse or bent).

Pulmonary form of polymositis

The pulmonary form can be a complication of polyimositis and is considered the most dangerous.

Come to her:

• the damage to the polyimositis of the respiratory muscles and M - C diaphragm, which led to insufficient ventilation of the lungs;
• viral and tank. infections;
• entering water or food into the lungs of the esophagus;
• interstitial pneumonia;
• malignant tumors and metastases in the lungs.

Symptoms of pulmonary polymositis:

• superficiality and respiratory rapidity;
• progressive shortness of breath;
• hypoxemia (reduction of O2 in the blood);
• noises in the lungs;
• fibrosis of pulmonary fabric and alveoli.

Acute pulmonary myositis has an unfavorable forecast: with it, breathing insufficiency is growing very quickly, and up to 10% of cases ends with fibrosis of the lungs, which means the limited possibility of the respiratory function.

The asymptomic form is peculiar to the early stages of paraneoplastic PM. When developing a malignant process, the symptoms appear:

• inspiratory shortness of breath;
• supersonic dry cough;
• in the lower departments, noises and wheezing (crepitiation) are listed.

Articular polyimiosit

The articulated PM is most often developing due to infections and affects periarticular connecting tissues (articular muscles, ligaments, tendons, capsules). It is characterized by pains, stagnation of joints, especially in the morning. Sometimes it comes to the joint, because of which it can swollen.

This form of PM is reversible and has a favorable forecast: after the antibacterial / antivirus therapy, the taking cytostatics, the articual manifestations are regressing.

Heart polyimiosit

Usually, this disease is manifested in the defeat of myocardium, but all three heart shells may suffer, as well as coronary arteries.

Symptoms with heart PM:

• cardiac rhythm;
• arrhythmia;
• muffled heart tones;
• with an unfavorable outcome of the heart attack.

Defeat GTC

Gastrointestinal polyimiositis is caused by the damage to the blood and nervous vessels, and the smooth muscles of the gastrointestinal tract.

As a result, the power of the mucous membrane and intestinal peristalsis is disturbed:

• the symptoms of gastritis and colitis begin to manifest;
• on the inner walls of the stomach and the intestine, erosion and ulcers are formed;
• the danger of gastrointestinal bleeding and perforation increases, which can lead to peritonitis and fatal outcome.

Failures of the endocrine system

Endocrine disorders are possible with inflammation of vessels (vasculite), which are caused directly by the systemic mositis itself, as well as in the form of the effects of GKS therapy, the main method of treatment with symptoms of polyimositis and dermatomyositis.

First of all, the pituitary glands, adrenal glands, sex glands are affected.

Diagnostics during dermatomyositis

The main methods of examination in the diagnosis of DM:

• Oak and bhak (general and biochemical analyzes blood;
• immunological analyzes;
• the biopsy of pathological connective tissues (is the most verification method).

Diagnostic pattern with DM as follows:

• EE and leukocytes are slightly increased;
• the enzymes of muscle damage to KFK (creatinophosphokinesis) and LDH (lactate dehydrogenase) may exceed ten times;
• also increased aldola, alt, asthma.

Treatment of dermatomyositis

Medication tools

Treat inflammation of connecting tissues at DM is necessary with:

• glucocorticosteroids;
• immunosuppressants (methotrexate, sulfasalazine, azathioprine, cyclophosphomide, etc.);
• antibiotics, antivirus drugs;
• antihistamine.

Comprehensive supporting therapy

With a polyimosite, affecting the organs, also conduct comprehensive supporting therapy and prevention of possible complications:

• Drugs are prescribed to normalize blood circulation and exchange in soft tissues, antihypoxants, vitamin complexes.
• In the treatment of acute forms of pulmonary polymositis, urgent IVL may be required, with a gastrointestinal damage - an emergency operation, and in heart disease - measures to prevent infarction or post-infarction therapy.

Surgery

Surgical intervention is necessary in the arrangements of connective tissues, their fibrosis or calcine if they affect the nerves, causing pain and limiting mobility, or overlap vessels.

The article will tell about such a rare and dangerous inflammatory disease as dermatomyomyomy. Knowing the symptoms of its occurrence, you will be able to prevent the development of pathology in a timely manner. You will also learn what medications you can cure a tightened disease.

Dermatomiositis: General Information

Dermatomiosit (Wagner-Unferricht Syndrome) - Rare inflammatory chronic disease. Developing in the human body, it modifies the skin and muscles, affecting their motor function. If the disease strikes a smooth and skeletal muscles, it does not affect the skin, which happens in 25% of all clinical cases, then it is diagnosed as a polyimiosite (another name of the dermatomyositis).

For reference! In a year, dermatomyomyomy is diagnosed in 5 people per 1 million population. The female floor is more prone to this type of disease than male. In adults, the disease occurs more often than children.

Symptoms of the disease

First detailed description The dermatomyositis was published in 1940:

1. The disease begins with a subfebrile temperature (37.1 - 38 ° C).
2. The patient has photosensitivity of the skin (excessive sensitivity to the sunny light causes redness, peeling of the skin).
3. The hair falls out, foci of redness appear on the skin of the head.
4. Pains in the field of hips and limbs are felt.
5. The patient quickly loses weight.
6. The lesion of the skin is happening: the inner shell of the eyelids and the cornea will swell (the outer sheath of the eyes), as well as the area under the eyes.
7. On the skin in the joints of the joints appear anomalous redness with glossy glitter. After these foci disappear, the skin no longer looks like before, it is atrophy (the epidermis is thinned, becomes wrinkled and dehydrated). Later is developing porchilodemia (hyperpigmentation, smaller expansion blood vessels).
8. On the mucosa of the oral cavity, inflamed foci (from black and red to blue-red) are formed. Language, lips and lower gums swell, painful ulcers appear on them. Thickened and lins the mucous cheek, surface of the language and corners of the lips.
9. Facial facial is broken, it acquires the expression of fear, the forehead is highlighted.

For reference! Due to the fact that blister-purple spots occur on the skin on the skin, the pathology received an additional name - "Lilk Disease".

Pathological changes of muscles

Following the skin atrophy, muscle defeat occurs. Such a state makes it necessary to know the feeling of pain and the inability to relax the muscles of the body. The patient's condition is deteriorating, muscle atrophy occurs, their physical strength decreases. The thrust muscles are almost all the time in abbreviated state, which does not allow blood to fully deliver nutrients to muscle fibers.

Changing the functions of smooth muscles

In addition to lesions of the skin and skeletal muscle tissue, the dermatomyosis can cause the pathology of the heart muscle. As a result, a patient may appear tachycardia (rapid heartbeat), arrhythmia (impaired frequency), extrasystolia (improper reduction of cardiac cameras).

A negativeness may affect the disease and on the smooth muscles of the body, in particular the tract (gastro intestinal tract), causing dysfagia (swallowing disorder), painful spasms in the stomach, ulcerative formations on the mucous membrane.

Other violations in the body

• Vessels. In some cases, dermatomyosis (Wagner syndrome) causes the spasm of the brain vessels, causing oxygen starvation of its cells.
• Lungs. Pathology can also affect the lungs and provoke diseases such as bronchopneumonia (inflammation of the walls of the bronchial tree) and penetration into light foreign bodies due to swallowing disorder (aspiration).
• Lymphosystem (part of the cardiovascular system). During the dermatomyosit, the lymphatic system may be out, which threatens the organism the occurrence of malignant and benign tumors, as well as an increase in the spleen.
• Nerves. In addition to internal organs, dermatomyomyosis adversely affects nervous system patient. Such a state makes itself felt a violation of the psyche and pain when pressing on large nerves, which are located in the lumbar-sacral zone in the neck and limbs.
• Skeleton. If dermatomyomyomy is lasting for several months, then pathological changes affect the skeleton. In the patient against the background of inactivity, the skeleton atrophy occurs, which affects the functions of the musculoskeletal system.
• Eyes. Atrophic foci occurs in the eye day, the retina is affected, which becomes the cause of vision disorder.

Main reasons

To date, the exact reasons that can provoke the occurrence of dermatomyositis do not exist. Medicine scientists attributed this type of pathology to the multifactorial group of diseases (with hereditary predisposition). A huge role in the development of the disease is played:

• Infectious factors. This is confirmed by the results of the studies conducted, which have proven that patients who took several times for 3 months some infectious disease (For example: chlamydia, raw tit), the chances of the appearance of dermatomyositis increase.
• Viral diseases. The influenza virus, pebornaviruses (cause inflammation in the intestines), parvoviruses (striking joints without degenerative changes, skin), predispose to the appearance of DM.
• Bacterial pathogens. Reason: Vaccination against Tifa and Corey, Taking Badics based on Growth Hormone ("Nexropin", "Jintopin").
• Pathogenetic factor. An autoimmune reaction (a condition in which human immunity attacks healthy cells of its own organism) works against the contents of muscle tissue cells (proteins, ribonucleic acids). Such reactions lead to impaired balance between lymphocytes and do not allow too aggressive immune response.

Other predisposing factors are:

• Supercooling of the body.
• Sunny / thermal blow.
• Injuries of physical and mental type.
• Hereditary predisposition.
• Exacerbation of focal infection.
• Allergies to drugs.

Taking into account the fact that the factors that can provoke the development of Wagner syndrome (dermatomyodeism), too much and they are different, scientists decided to distribute the disease in classes.

Classification of the disease

The existing forms of dermatomyositis are distinguished with regard to the origin and course of the disease:

• Primary.
• Secondary (tumor).
• Children's (juvenile).
• Polymizit.

Primary (clinical picture)

It is characterized by the weakening of skeletal muscles, manifestations on mucous membranes and skin (100% defeat, swelling, redness).

Pathology develops gradually, but may also be acute and chronic:

1. In the first case, the disease exhibits themselves with swelling and hyperemia (blood vessel overflow) around the eyes and in open parts of the body (face, neck, handbrushes). Pain in the field of joints and muscles, muscle weakness. The appearance of a subfebrile temperature (37.1 - 38 ° C).
2. With acute dermatomyositis (wagner syndrome), a patient has a fever with a temperature of 38 - 39 ° C. Dramatically worsen general state Health. Pronounced inflamed foci on the skin of the face, limbs and torso arise. Muscles are intensively weakened, up to the loss of motor functions.
3. The chronic form of dermatomyositis is expressed by the appearance of rashes on the skin even before the smooth muscles lesion, which develops gradually and is not so pronounced, as at the first two forms.

Secondary syndrome

Pathology makes it necessary to know the purple rash on the skin and the weakening of the proximal (nearest to the body) muscles. There is a puffiness with a purple tint around the eye.

In 25% of clinical cases, patients with dermatomyositis are combined with the appearance of malignant neoplasms. In some patients, the disease develops before the appearance of neoplasm (tumors) for 30-60 days, in others at the same time with the advent of the neoplasm, in the rest - after the tumor was diagnosed. Doctors explain this factor with age feature and the presence of rheumatic diseases.

The combination of dermatomyositis with a tumor is observed at the male floor 3 times more often than women. Although the female floor prevails among patients with polyimositis.

The development of tumor dermatomyositis is explained by several factors:

• A possible cross reaction between muscle and tumor antigens (antibody manufacturers cause an immune response of the body).
• Activation of a hidden viral infection (for example, cytomegalovirus, herpes of different species) with the subsequent immune response and development of Wagner syndrome.

The clinical symptoms of the secondary DM can coincide with the primary (idiopathic), but in this case the difference is reduced to the absence of a patient's reaction on the treatment carried out.

Children's dermatomyosis

In children, Wagner syndrome most often appears at the age of 4 - 10 years, equally both boys and girls. In almost half of the clinical cases, pathology begins acutely (there is a sharp pain in muscles, rashes, temperature). Symptoms and the course of the disease are similar to the painting of the development of dermatomyositis in adults.

Distinctive features are reduced to:

1. Acute start of pathology.
2. The strong damage to the shallow brain vessels.
3. The presence of fluid in edema regions, including in the inflamed joints.
4. To the consequence in the form of sediments of salts in soft tissues.

The children's dermatomyomyosis begins with fever, the appearance of a sharp pain in the muscles. Patient feels general weaknessFor the first two days, weight is noticeably losing weight. Separate faces of the face of the face acquire a purple shade, or purple and red rashes appear in the area around the eyes, in some cases the rash is brightly noticeable on the cheeks, neck, hands, footsteps, rear and in front of the chest.

For reference! In parallel, the swelling of the skin and subcutaneous fiber can be developed, the tissues of enveloping joints can be inflated (with the appearance of liquid in them).

Muscle weakness increases, it leads to patient immobility. Often, this condition is accompanied by a feeling of pain, resulting in a need for a clarification of the diagnosis. Symptoms of the disease begin to resemble the polyarthritis (inflammatory joint disease).

Although, if during this period, dysphagia (swallowing violation) and dysphony (voice problems) makes itself felt about himself, the specialists may not doubt the diagnosis of dermatomyodeism. If only the last two violations are not the result of a neurological disease. For this reason, the diagnosis cannot be delivered without differential diagnosiswhich allows you to calculate the only probable disease in the patient.

The main reason that can lead to death in children is an intensive change in the muscles of the breathing apparatus with the subsequent occurrence of respiratory failure. If the condition described is accompanied by pneumonia congestion (inflammation of the lungs with blood circulation stagnation) or aspiration (infectious-toxic damage, when inhaled foreign bodies) Character, then with insufficient treatment, sometimes with acute or sluggish the course of the disease comes a fatal outcome.

Polymizit (type DM)

Pathology amazes skeletal muscle tissue, mainly limbs. Accompanied by pain syndrome, weakening and muscle atrophy. When polyimosit, pathological changes also cover the smooth muscles of lungs and hearts.

In order to diagnose the disease, we will take advice from the rheumatologist, therapist, dermatologist. You will be appointed:

• For testing for laboratory research.
• Electromyography (for evaluation functional state muscles).
• Biopsy (take muscle tissue to study).
• Ultrasound and ECG (for inspection of internal organs).

As for treatment, it is carried out with the help of steroid hormones (Prednisolone tablets). But if the result is absent, which happens in 50% of cases, then the patient is prescribed immunosuppressants ("Methotrexate" for intravenous administration).

Important! As an alternative, Azatioprin can be used, but with a condition for the monthly assessment of the liver condition by means of a biochemical test.

As for the forecast of polymositis, the most unfavorable exodus expects patients with acute form Diseases, especially with illiterate treatment of the body. The patient's death may occur as a result of complications in the form of ease of pulmonary failure or development of pneumonia.

More about the symptoms, diagnosis and treatment of polyimositus will tell a specialist in the proposed video material. From it you will learn from which doctors you need to get advice, what tests need to be passed to confirm the diagnosis.

Treatment methods

After the exact diagnosis, the patient with the dermatomyositis is prescribed treatment with the use of a glucocorticosteroid drug, usually, prednisolone pills.

• Acute course of the disease - 80-100 mg / day
• Subighteous type of pathology - 60 mg / day.
• Chronic dermatomyositis - 30-40 mg / day.

If the dose of the hormonal drug is chosen correctly, then after 7 days the patient's condition begins to come back to normal. Initially, the symptoms of intoxication disappear, then after 2 weeks, the swelling is gradually from the skin. Redness disappear.

The dose of the drug can be increased in the absence of due effect. In this case, the maximum dosage of the patient can not take more than 60 days, after which the dose is reduced to supporting.

Note!Medical practice shows that the treatment of dermatomyositis lasts about 2-3 years.

With the stability of the body to glucocorticosteroids, the patients are prescribed cytostatic, which inhibit pathological processes in the body:

In the treatment of acute and subacute type of pathology, the preparation of "prednisolone", during the dosage period, patients prescribe the reception of chinoline means ("delagil", "Plaquenil"), which have the property to normalize the body's immune response. If the disease is chronic, then quinoline drugs are attributed initially.

In addition to these drugs for the treatment of dermatomyositis, vitamins of groups B, "adenosynthithic acid" (energy supplier), non-steroidal anti-inflammatory drugs ("ibuprofen", "diclofenac") can be assigned.

Prediction and prevention

The launched shape of the dermatomyositis threatens to a person with a fatal outcome. In the first two years, death is overtaking 40% of patients. The reason is reduced to the damage of the respiratory muscle and the emergence of gastrointestinal bleeding. Heavy pathology with a protracted character causes a narrowing and deformation of the limbs, which becomes the cause of disabilities.

Timely treatment of steroid drugs ("prednisone") improves the forecast for survival. This applies to the prevention, then special measures that are able to prevent the occurrence of Wagner's syndrome has not been detected. The secondary prevention of the disease, which is carried out to avoid relapse, requires that the patient stood on a dispensary accounting by the rheumatologist and supported his condition through medication prescribed by the doctor.

Question answer

What doctor to contact when suspicion of dermatomyomyode?

Mandatory to the rheumatologist. This specialist treats the damage to the connective tissue, joint diseases, inflammation of the vessels of the skin.

Is it possible to refuse hormonal therapy at DM due to the occurrence side Effects And only immunoglobulins are treated?

It is impossible, since this type of pathology is treated with large doses of glucocorticosteroids in combination with a long admission. Immunoglobulins should go in a complex with hormonal means, as they only contribute to the normalization of immunity.

Hormones wash out calcium from the bones, is it possible to somehow prevent this problem?

To this end, with the knowledge of the doctor, you can take 2 tab / day "Calc Chicken Advance", put the course of omega 3 (fish oil) for 2-3 capsules per day. Eat every day solid cheese and sesame seeds.

What to remember:

1. Dermatomiositis is a serious inflammatory disease, so at the first symptoms of its development, refer to a rheumatologist.
2. Leaving the disease without treatment, you increase the risk of fatal outcome.
3. Cure dm without use hormonal drugs It is impossible.
4. Cancellation of high doses of drugs should occur gradually.
5. Preventive measures with respect to DM E exists, but after the treatment of pathology, it is necessary to register and take appointed medicines to support health.

Dermatomiositis is the result of a painful reaction immune system on the own fabrics. Symptoms are associated not only with skin and muscles, but also with light, heart and digestive system. In some cases, the disease occurs in parallel with the development of malignant neoplasms.

Dermatomiositis is a progressive disease, which primarily affects muscle and skin tissue vessels. Inflammatory symptoms can lead to ulcerations, scarring and skin necrosis, muscle weakness, interstitial changes in lungs and heart problems.

Dermatomiode - symptoms

Symptoms of dermatomyositis appear gradually, increasing their severity with relapses, between which a temporary decrease in the intensity of the disease can be observed.

General ailments are nonspecific. Weakness, repeating subfebrile states or fever, lack of visible cause, weight loss are the most common.

Special symptoms associated with dermatophytes, which can be originally recognized, belong to the skin. They are so-called Papula Gottron: blue, in advanced shape with a small attic in the middle. They are often ulcerated, heal, leaving scars or discoloration or atrophic skin smoothing. Their typical location is the dorsal surfaces of the hands near Metarpographic and interphalangeal joints. They are also observed on the front surface of the knees and on elongated areas of skin elbows.

The appearance of symptoms on the patient's hands is characteristic. Inflammatory changes also focus around the nails, forming dark red or blue furrows on the surface of the fingertips. This symptom is called the "man's hand".

Skin Symptoms: A. Gottron B. Papula B. Ocolone TuelGelectasia F. Pihalodermatomyomy

Other symptoms of dermatomyositis include, for example, intensively painted erythematous spots with a slightly raised surface and high heat due to edema. You can see them around the eyes, on the forehead, on the cheeks and ears and in places subject to sunlight. During periods of flashes, stains can be mixed together, creating large surfaces (for example, the so-called neck scarf). Typical provisions are also the side surfaces of the hips, lumbar region Spins, scalp. Intense inflammatory changes can lead to local hair loss.

During the dermatomyositis, changes occur in the blood vessels, the flow of which is irregular and winding. Numerous extensions appear, i.e. telegangectasia.

Muscular symptoms of dermatomyositis

Dermatomiosit causes muscle weakness in 30-40% of patients. On early stages Diseases, it basically affects the muscles of the shoulder belt, hands and hips. There are problems with raising hands above your head, holding more heavy items, upstairs up the stairs or uneven surfaces, as well as difficulty stand still. As the disease progressing, changes also affect the distal muscles - further from the body. The patient has problems with accurate hand movements, capturing objects.

The consequence of the disease is associated with:

• problems while maintaining the right position of the head,
• swallowing
• emission of the contents of the stomach in the esophagus with the symptoms of heartburn (reflux),
• reduced myocardial reduction and even stagnant heart failure,
• savs of tendons with the type of symptom Rhine (paroxysmal pallor, ischemia, cooling,
• which pass temporary hyperemia, swelling and severe pain).

Light symptoms of dermatomyositis

Dermatomiositis initially gives symptoms in the light type of interstitial inflammation, which is transformed into fibrosis with the atelectasis and dilatation (expansion) of the bronchi. This leads to a decrease in the effectiveness of respiratory activity, cough and an increase in chronic shortness of breath.

Dermatomiode - Diagnostics

The diagnosis of dermatomyositis is based primarily at the level of characteristic antibodies and histopathological assessment of skin sections or muscles. Examination (non-unambiguous diagnosis):

• blood biochemistry (evaluation of inflammation markers),
• diagnostic visualization (computed tomography, magnetic resonance),
• electrocardiography (ECG) and electromyography,
• lungs.

Dermatomiositis - reasons

The main cause of the dermatomyositis is the effect of antibodies produced by the body on its own tissues, mostly endothelium of blood vessels. The study shows that the beginning of the disease process often correlates with such factors as:

Genetic factors are also important.

Dermatomiosis - forecast

When dermatomyosit, the forecast depends on the intensity and degree of illness and the individual response to drugs. Most patients survive for many years. In the case of lungs, serious complications may arise in the form of recurrent inflammation and chronic respiratory failure or even death. Despite the anti-inflammatory and immunosuppressive treatment, the disease usually progresses, and muscle weakness and lesions of the skin gradually increase.