Dermatomyositis secondary. Dermatomyositis: a difficult test that requires accurate diagnosis and competent treatment

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

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

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

Dermatomyositis is a systemic increasing pathology, due to which muscle tissue and skin cover are primarily affected, the functioning of organs is disrupted, which may be accompanied by a purulent infection. In a quarter of patients, skin diseases are not observed. In this case, mean polymyositis. Dermatomyositis belongs to a class of inflammatory neuromuscular diseases. In 25% of cases, secondary tumor pathology is observed. The disease can be acute, subacute, or chronic.

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

Causes of pathology

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

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

Risk factors

Dermatomyositis most often occurs women than men.

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

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

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

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

Endocrine diseases. Malfunction of the thyroid gland.

Hormonal failure, menopause, pregnancy.

permanent being under the scorching sun or, conversely, a long stay in the cold.

Symptoms of pathology

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

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

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

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

In the mucous membrane, the processes of stomatitis and conjunctivitis occur, swelling is manifested. Sometimes you can find a disease of the joints of the shoulders, elbows, hands, knees and ankles. Juvenile dermatomyositis is accompanied by intramuscular and intradermal calcification in the shoulders, pelvis and buttocks. Calcifications provoke the formation of ulcers on the skin and the release of calcium deposits.

Systemic symptoms of pathology affect the work:

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

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

Symptoms include the following.

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

Table. Variants of skin lesions in dermatomyositis.

Variants of skin lesions	Description
Periorbital edema (heliotrope)	Purple or dark blue rash around the eyes.
Erythematous photosensitivity rash	Erythema of the face in the knees, elbows, neck and décolleté.
Gottron's papules	Dense erythematous rashes, often localized over the metacarpophalangeal and proximal interphalangeal joints, over the flexion surfaces of the elbow and knee joints.
"Mechanic's Hand"	Peeling, painful cracks on the skin of the hands.
capillaries of the nail bed	Dilated and tortuous capillaries of the nail bed.
Poikiloderma	Hyperpigmentation and atrophy with the presence of spider veins, peeling, which determines the mottled nature of the affected area of ​​the skin.

Diagnosis of dermatomyositis

• laboratory factors;
• muscle biopsy.

Polymyositis should be suspected when a patient has proximal muscle weakness, a manifestation without underlying pain in the muscles. The accuracy of diagnosing a disease becomes higher if the presence of the following problems can be confirmed:

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

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

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

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

Note! Temporary monitoring of creatine kinase activity provides good results for disease monitoring. In acute muscle atrophy, the activity of the enzyme can maintain normal levels, despite the activity of myositis. MRI information, elevated creatine kinase levels, and muscle biopsies help identify myopathy and polymyositis.

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

Screening is strongly recommended by some health authorities for patients 40 years of age or older with dermatomyositis and patients over 60 years of age with polymyositis. As a rule, in people of this age group with existing diseases, malignant tumors are very often found.

Screening involves a physical examination, including:

• examination of the pelvis, mammary glands and rectum;
• laboratory and biochemical blood tests;
• mammography;
• Analysis of urine;
• chest x-ray;
• other research.

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

Possible Complications

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

Treatment of the disease

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

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

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

If there is an inappropriate response to therapy with this drug and if hormonal myopathy or other problems appear, you need to reduce the amount of medication used or stop prednisone. In this case, treatment with immunosuppressants should be considered. Many specialists prescribe combined treatment in the initial stages of therapy.
Some patients take one methotrexate for 5 years or more. Therapy with intravenous drugs can bring results in patients who do not accept medical treatment. But their considerable price makes it impossible to organize comparative studies. Myositis, similar to malignancy, most often differs in greater refractoriness in relation to corticosteroids. Myositis, similar to a tumor, may subside if it is removed.

Important! Patients with an autoimmune disease are more prone to atherosclerosis and should therefore be monitored regularly. Permanent and long-term treatment requires patients to prevent osteoporosis.

Necessary patient care

1. The sleeping place of a person with dermatomyositis should be comfortable, soft and keep warm. Affected joints should be protected from possible microtraumas.
2. For medical purposes, apply a warm compress to the joints.
3. It is necessary to constantly monitor the intake of drugs by patients, remind them of the need to take them, monitor compliance with the dosage. Track possible deviations from the usual state and manifested complications during treatment.
4. Patient hygiene control. If necessary, provide assistance during the change of bed and personal linen, taking a bath. If there are pains in the joints and muscles, excessive weakness, the patient is unable to perform these simple actions alone.
5. The patient's diet should contain the necessary vitamins, especially vitamins B, C and D, and not include many fatty acids. It is worth reducing the use of table salt. Food should be sufficiently high-calorie and well digestible by the stomach, the diet should be balanced.
6. Regular exercise therapy. Physical exercises should be aimed at working with the affected muscles and joints, individual muscle groups. If dermatomyositis is detected, it is worth performing special gymnastic exercises for the muscles of the face. During exercise, active and passive loads occur on all joints. Carrying out medical procedures should take place at the time of weak activity of immunoinflammatory processes with full observation of the patient's condition.
7. Given the fact that treatment requires considerable time, there is a possibility of various complications from the used therapeutic agents (especially when using cytostatics and glucocorticosteroids). It is worth paying special attention to constant conversations with the patient, during which it is worth conveying the necessary meaning of the therapy being carried out, convincing the patient to show more tolerance and restraint, directing them to positive thoughts and charging them with good emotions. It is equally important to talk with the patient's family. It is worth bringing them up to date and highlighting the essence and nuances of the disease, the established course of treatment, familiarize them with possible complications and convince them to be tolerant when providing the patient with the necessary assistance and support.
8. It is worth helping the patient with the optimal organization of his day, think over his working time and periods of rest. It is better to refuse night work, minimize physical activity and protect from emotional outbursts.

Prevention and prognosis of dermatomyositis

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

Non-specific ways to prevent dermatomyositis include: observation by a rheumatologist, control of medication.

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

Women should coordinate pregnancy with a rheumatologist.

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

Summing up

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

Juvenile dermatomyositis- a disease from the group of diffuse connective tissue diseases with a predominant lesion of the proximal skeletal muscles, the development of muscle weakness, as well as purple erythema on the skin. Since the etiology of the disease is unclear, juvenile dermatomyositis is included in the heterogeneous group of idiopathic inflammatory myopathies with a leading clinical manifestation - inflammatory skeletal muscle damage. According to R.L. Woltman (1994), in addition to juvenile dermatomyositis, this group also includes other myopathies.

Symptoms of juvenile dermatomyositis

In children, dermatomyositis often begins acutely or subacutely; at the onset of the disease, fever, weakness, malaise, weight loss, myalgia, arthralgia, and a progressive decrease in muscle strength often occur. The clinical picture of dermatomyositis is usually polysyndromic, but the most characteristic changes in the skin and muscles.

Skin lesion

Skin lesions are a characteristic sign of dermatomyositis. Skin manifestations of dermatomyositis include erythematous rashes with a purple tint on the face in the paraorbital region (a symptom of "dermatomyositis glasses"), in the décolleté area, over the metacarpophalangeal and proximal interphalangeal joints of the hands (Gottron's sign) and over large joints of the extremities, primarily the elbows and knee. In the acute period, patients often have superficial skin necrosis at the sites of injury, and subsequently develop atrophy with areas of depigmentation. Some patients observe redness, peeling and cracking of the skin of the palms ("mechanic's hand").

In children with dermatomyositis, bright livedo usually occurs, especially in the area of ​​the shoulder and pelvic girdle, capillaritis of the palms and feet, and telangiectasia. Generalized vascular lesions are especially characteristic of preschool children.

In acute and subacute course, pronounced trophic disorders are observed in the form of xeroderma, brittle nails, and alopecia.

Subcutaneous tissue injury

Over the affected muscles of the limbs and on the face, a testy or dense edema often appears. Perhaps the development of partial lipodystrophy of the face and limbs, usually combined with muscle atrophy.

Muscle damage

Usually, at the beginning of the disease, patients with dermatomyositis complain of fatigue during physical exertion, muscle pain that occurs spontaneously and is aggravated by palpation and movement. Dermatomyositis is characterized by a symmetrical lesion, primarily of the proximal muscles of the limbs, as a result of which children cannot carry a briefcase in their hands, it is difficult for them to raise their hands up and hold them in this position, they cannot comb their hair (“comb symptom”), get dressed (“symptom shirts”), quickly get tired when walking, often fall, cannot climb stairs, get up from a chair, raise and hold their legs. With severe damage to the muscles of the neck and back, patients cannot tear their heads off the pillow, turn around and get out of bed. In the most severe cases, generalized muscle weakness develops with an emphasis on the proximal group, as a result of which patients can be almost completely immobilized.

When the muscles of the larynx and pharynx are affected, a nasal and hoarse voice appears, as well as a violation of swallowing, which can lead to aspiration of food and saliva. With damage to the facial muscles, a mask-like face is noted, with damage to the oculomotor muscles - diplopia and ptosis of the eyelids. Severe damage to the diaphragm and intercostal muscles leads to respiratory failure. In the outcome of polymyositis, muscle hypotrophy develops.

In children, unlike adults, persistent, sometimes painful tendon-muscle contractures are often formed, which sharply limit the range of motion.

Joint damage

Joint damage is observed in more than 75% of patients. Develop arthralgia or polyarthritis. The most commonly affected joints are the small joints of the hands (mainly proximal interphalangeal), knee and elbow. Articular changes are characterized by moderate defiguration and pain on palpation and movement. In most cases, articular syndrome quickly stops during treatment, only 25% of patients note the formation of contractures, deformities and subluxations in the interphalangeal joints with some limitation of functionality.

Calcinosis

Calcinosis in dermatomyositis in children occurs 3-4 times more often than in adults. It develops in almost 40% of patients, mainly in the period from 1 to 5 years after the onset of the disease. Calcifications can be limited in the form of individual foci or plates and localized subcutaneously or in the connective tissue around muscle fibers, they can also be located in areas of the greatest trauma - around the knee or elbow joints, along the Achilles tendon, on the hips, buttocks, shoulders. In patients with continuously relapsing dermatomyositis, calcification is usually diffuse.

Damage to internal organs

With dermatomyositis, myocarditis most often develops, manifested mainly by rhythm and conduction disturbances, and a decrease in the contractility of the heart muscle. In 25% of patients, pericarditis develops with mild symptoms that quickly disappear after the start of treatment with glucocorticoids.

Lung damage (pneumonitis) is associated with vascular-interstitial changes and is clinically manifested by an unproductive cough, shortness of breath, intermittent wheezing during auscultation. Prognostically unfavorable is the development of diffuse alveolitis with the formation of an alveolar-capillary block, the rapid development of pulmonary insufficiency and death. Damage to the lungs in dermatomyositis can also be due to the development of aspiration and banal hypostatic pneumonia due to damage to the muscles involved in swallowing and breathing. Often, pleurisy is found in children, with a high degree of activity of the process, sometimes accompanied by the formation of exudate.

Kidney damage is rare. The renal syndrome is represented by a transient urinary syndrome, in some cases accompanied by impaired renal function up to the development of acute renal failure due to massive myoglobinuria.

Often in children with a high activity of the process, esophagitis, gastroduodenitis, enterocolitis occur; the development of an erosive-ulcerative process, complicated by perforation and bleeding, is possible. Occasionally, a pseudo-abdominal syndrome is observed, resulting from damage to the muscles of the anterior abdominal wall, with edema, induration and severe pain during breathing and palpation.

Laboratory research

In a laboratory study, patients in the active period of the disease usually reveal an increase in ESR, moderate anemia, in some patients - moderate leukocytosis, hypergammaglobulinemia.

Among the biochemical parameters, characteristic changes reflecting damage to skeletal muscles include an increase in the activity of creatine phosphokinase, as well as aldolase. In addition, patients often show an increase in the concentration of LDH and aminotransferases in the blood serum. Some patients develop myoglobinuria.

The detection of myositis-specific antibodies is important primarily for classification, i.e. clarification of the clinical and immunological subtype of dermatomyositis and polymyositis. In some patients, antibodies to tRNA aminoacyl synthetases are detected, primarily antibodies to histidyl-tRNA synthetase (Jo-1). In the presence of these antibodies in the blood, an antisynthetase syndrome develops, characterized by an acute onset of myositis, interstitial lung damage, fever, symmetrical arthritis, Raynaud's syndrome, skin lesions of the hands like a "mechanic's hand", an incomplete response to the use of glucocorticoids and frequent development of exacerbations against the background of a decrease in their doses, the debut of the disease mainly in the spring.

Diagnosis of juvenile dermatomyositis

The following criteria for the diagnosis of dermatomyositis have been developed ( Tanimoto et al., 1995).

Skin lesion.

1. Heliotrope rash - red-violet erythematous rash on the eyelids.

   Gottron's sign - red-violet scaly atrophic erythema or spots on the extensor surface of the hands over the metacarpophalangeal and proximal interphalangeal joints.

   Erythema on the extensor surface of the limbs, over the elbow and knee joints.

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

Localization and prevalence

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

Causes of dermatomyositis

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

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

Symptoms of dermatomyositis

Disease dermatomyositis symptoms

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

A very characteristic skin manifestation of dermatomyositis is a symptom of "purple glasses" - redness and swelling of the skin on the upper eyelids. Other manifestations are diverse and nonspecific: patients have erythematous skin areas, rashes in the form of vesicles, itching, papules. Usually exposed areas of the body are affected.

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

Diagnosis of dermatomyositis

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

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

In the analysis of urine, an increase in the content of creatinine is observed.

Patients with suspected systemic dermatomyositis are assigned the following studies:

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

Treatment of dermatomyositis

Treatment is aimed at stopping inflammation and preventing the degeneration of muscle tissue into connective tissue. For this, the following drugs are used:

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

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

Treatment of dermatomyositis with folk remedies

It is possible to treat dermatomyositis with folk remedies.

• Compresses:

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

• Ointments:

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

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

Prognosis and complications

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

Prevention

Specific prophylaxis has not been developed. Primary prevention includes the avoidance of risk factors for the development of the disease, the general hardening of the body. When symptoms are detected, prevention is reduced to preventing relapses and complications.

A photo

Disease dermatomyositis skin manifestations photo

is an inflammatory myopathy of childhood with a predominant lesion of the proximal muscles of the limbs, the development of systemic vasculitis and characteristic skin changes. There are specific skin manifestations in the form of an erythematous rash around the eyes, as well as in the neck and large joints (knee and elbow). It is accompanied by progressive muscle weakness, including the smooth muscles of the respiratory and digestive organs, as well as the striated muscle tissue of the heart. Joint damage and calcification are noted. Juvenile dermatomyositis is confirmed by detecting myositis antibodies in the blood. Treatment is with corticosteroids and cytostatics.

ICD-10

M33.0 Juvenile dermatomyositis

General information

Causes

The etiology of the disease is currently unknown. The detection of familial cases suggests a possible genetic predisposition. A viral infection plays a certain role, since some viruses (coxsackie viruses of groups A and B, picornaviruses) are often found in the blood of sick children. In addition, juvenile dermatomyositis can debut on the background of a viral disease or a short time after recovery. The main role in the pathogenesis is assigned to the autoimmune reaction of the body to its own myocytes, as well as to the smooth muscle cells of the vascular wall.

Symptoms of juvenile dermatomyositis

The disease usually begins subacutely. The first symptom may be muscle weakness. The weakening of muscle strength in juvenile dermatomyositis is more characteristic of the proximal muscles of the limbs, so at first it becomes difficult for the child to raise his arms (for example, to comb) and climb stairs.

Over time, weakness sometimes progresses so much that the patient cannot sit up from a lying position and even tear his head off the pillow. Pressing on the muscles in this case causes discomfort and is painful. Muscle weakness can also spread to the smooth muscles of the internal organs, which makes it difficult to breathe and swallow, and there may be cases of choking.

In about half of patients, juvenile dermatomyositis debuts with skin lesions. Skin manifestations are very specific. So, erythematous rashes on the eyelids and around the eyes are characteristic - a symptom of "dermatomyositis" glasses. A similar skin lesion is observed in the area of ​​​​the joints, as a rule, knee and elbow, as well as around the neck and in the area of ​​small joints of the hand. Often, thinning of the epidermis and superficial necrosis of skin areas are noted at the sites of the lesion. Telangiectasias may also occur here as a sign of systemic vasculitis. For juvenile dermatomyositis, vascular damage is characteristic, which is manifested by symptoms from the intestines: stool retention, esophagitis, gastritis, perforations are occasionally possible.

Over the inflamed muscles, areas of lipodystrophy are often formed, against which the relief of the muscles becomes more noticeable. There is also synovial edema around the altered muscles. The skin over the affected areas looks pasty. Edema is also noted in the internal organs, for example, in the heart sac (pericarditis) and around the lungs. Very often, juvenile dermatomyositis is accompanied by calcification. Calcifications are located between muscle fibers or subcutaneously, often found around the joints. The joints themselves are usually also affected, however, polyarthritis quickly stops on the background of therapy.

Complications

Frequently, juvenile dermatomyositis is associated with cardiac weakness and myocarditis. Subsequently, the development of dilated cardiomyopathy with symptoms of congestive heart failure is possible. Diagnosable pneumonia may be associated with weakness of the intercostal muscles (hypostatic pneumonia) and accidental ingestion of food into the lungs due to difficulty swallowing. Such a diverse clinical picture of juvenile dermatomyositis is based on the same vasculitis with damage to muscle tissue. Thus, inflammatory changes can be observed in muscle fibers located in any organs.

Diagnostics

Some symptoms of the disease are pathognomonic and greatly simplify the correct diagnosis. These signs include a characteristic lesion of the periorbital region, the skin around the neck and over large joints. However, as mentioned above, skin manifestations in juvenile dermatomyositis are expressed only in half of the diseased children.

At the same time, muscle weakness is a very non-specific symptom characteristic of many connective tissue diseases and neurological pathologies. In particular, it is noted in myasthenia gravis, progressive muscular dystrophies. In addition, against the background of viral diseases, muscle weakness may be the result of general intoxication. To confirm the diagnosis of juvenile dermatomyositis, you must:

1. Blood study. Specific antibodies are detected in the blood, the concentration of products with steroid hormones is determined. There is a successful experience of connecting cytostatics to treatment. Since juvenile dermatomyositis is an autoimmune disease, there is a positive effect.

   In the remission phase, regular monitoring of the state of the muscles and muscle strength is necessary. Skin manifestations are reduced by taking vitamin D and using sunscreen. The child continues to take corticosteroids on an ongoing basis. Physical activity and gymnastics are also shown.

   Forecast

   The prognosis of the disease is doubtful. In recent decades, the mortality rate from juvenile dermatomyositis has decreased significantly and currently does not exceed 1% among affected children. There are also cases of almost complete restoration of muscle strength. At the same time, constant intake of corticosteroids can lead to chronic pathologies of the stomach and intestines, as well as to the development of depressive conditions (the effect of steroid hormones on the nervous system). An unfavorable prognosis is observed in cases of early onset and continuously relapsing course of juvenile dermatomyositis. However, timely diagnosis of the disease provides a 90% chance of successful treatment.

The content of the article

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

Classification of dermatomyositis

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

Etiology and pathogenesis of dermatomyositis

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

Clinic of dermatomyositis

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

Diagnosis of dermatomyositis

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

Differential diagnosis of dermatomyositis

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

Treatment of dermatomyositis

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

Prevention of dermatomyositis

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