Despite the rapid development of science and medicine, there are still areas that have not been fully explored. One such area is rheumatology. This is a field of medicine that studies systemic diseases of the connective tissue. Among them are dermatomyositis, lupus erythematosus, scleroderma, rheumatoid arthritis, etc. Despite the fact that all of these pathologies have long been described and known to doctors, the mechanisms and causes of their development have not been fully elucidated. In addition, doctors still have not found a way to cure such diseases. Dermatomyositis is one of the types of systemic pathological processes of connective tissue. This disease often affects children and young people. Pathology includes a set of symptoms that make it possible to make a diagnosis: dermatomyositis. Photos of the main manifestations of the disease are quite informative, since the disease has a pronounced clinical picture. A preliminary diagnosis can be made after an ordinary examination, by changing the patient's appearance.
Dermatomyositis - what is it?
According to the histological structure, they distinguishseveral types of fabrics. They form all organs and functional systems. The largest area is the connective tissue, which consists of the skin, muscles, as well as joints and ligaments. Some diseases affect all these structures, so they are classified as systemic pathologies. Dermatomyositis should be attributed to such ailments. Symptoms and treatment of this disease is studied by the science of rheumatology. Like other systemic diseases, dermatomyositis can affect the entire connective tissue. A feature of the pathology is that most often there are changes in the skin, smooth and striated muscles. With progression, superficial vessels and articular tissue are involved in the process.
Unfortunately, dermatomyositis is a chronic pathology that cannot be completely cured. The disease has periods of exacerbation and remission. The task of doctors today is to prolong the phases of the remission of the pathological process and stop its development. In the clinical picture of dermatomyositis, skeletal muscle damage comes first, leading to impaired movement and disability. Over time, other connective tissues are involved, namely smooth muscle, skin, and joints. It is possible to identify the disease after a full assessment of the clinical picture and the performance of special diagnostic procedures.
Causes of disease development
The etiology of some pathologies is still being investigated by scientists. Dermatomyositis is one such disease. Symptoms and treatment, photos of the affected areas arethe information that is available in the medical literature in large quantities. However, the exact causes of the disease are not mentioned anywhere. There are many hypotheses about the origin of systemic connective tissue lesions. Among them are genetic, viral, neuroendocrine and other theories. Provoking factors include:
- Use of toxic drugs and vaccination against infectious diseases.
- Prolonged hyperthermia.
- Hypocooling of the body.
- Stay in the sun.
- Infection with rare viruses.
- Climacteric and puberty, as well as pregnancy.
- Stress effects.
- Complicated family history.
It should be taken into account that such factors do not always cause this disease. Therefore, scientists still cannot determine how the pathological process starts. Doctors agree that dermatomyositis is a polyetiological chronic disease. In most cases, the disease occurs during adolescence.
Mechanism of development of dermatomyositis
Given that the etiology of dermatomyositis is not fully understood, the pathogenesis of this disease is difficult to study. It is known that pathology develops as a result of an autoimmune process. Under the influence of a provoking factor, the body's defense system begins to work incorrectly. Immune cells that have to fight infections and other harmful agents begin to perceive their own tissuesfor foreign substances. As a result, an inflammatory process begins in the body. Such a reaction is called autoimmune aggression and is observed in all systemic pathologies.
It is still not clear what exactly starts the process. It is believed that the neuroendocrine system plays an important role in it. After all, dermatomyositis most often develops during peak age periods, when there is a hormonal surge in the body. It should be noted that autoimmune aggression directed against one's own tissues is only the main stage of pathogenesis, but not the etiology of the disease.
Clinical manifestations of pathology
Since the disease refers to systemic processes, the manifestation of dermatomyositis can be different. The severity of symptoms depends on the nature of the course of the disease, stage, age and individual characteristics of the patient. The first sign of pathology is myalgia. Muscle pain appears suddenly and is intermittent. Also, discomfort is not necessarily noted in one place, but can migrate. First of all, the striated muscles responsible for movement suffer. Pain occurs in the muscles of the neck, shoulder girdle, upper and lower extremities. A sign of autoimmune muscle damage is a progressive course of pathology. Gradually, unpleasant sensations intensify, and motor function suffers. If the severity of the disease is severe, over time, the patient completely loses his ability to work.
In addition to skeletal muscle damage, the autoimmune processsmooth muscle tissue is also involved. This leads to impaired breathing, the functioning of the digestive tract and the genitourinary system. Due to smooth muscle damage, the following symptoms of dermatomyositis develop:
- Dysphagia. Occurs as a result of inflammatory changes and sclerosis of the pharynx.
- Speech disorder. Occurs due to damage to the muscles and ligaments of the larynx.
- Impaired breathing. It develops due to damage to the diaphragm and intercostal muscles.
- Congestive pneumonia. It is a complication of the pathological process that develops due to impaired mobility and damage to the bronchial tree.
Often, the autoimmune process is directed not only to the muscles, but also to other connective tissues present in the body. Therefore, skin manifestations are also referred to as symptoms of dermatomyositis. Photos of patients help to better imagine the appearance of a patient suffering from this pathology. Skin symptoms include:
- Erythema. This manifestation is considered especially specific. It is characterized by the appearance of periorbital purplish edema around the eyes, called "spectacle symptom".
- Signs of dermatitis - the appearance on the skin of areas of redness, various rashes.
- Hyper- and hypopigmentation. On the skin of patients, dark and light areas can be seen. In the affected area, the epidermis becomes dense and rough.
- Erythema on the fingers, redness of the palmar surface of the hand and striation of the nails. The combination of these manifestations is called the “symptom of Gottron.”
Besides being amazedmucous membranes. This is manifested by signs of conjunctivitis, pharyngitis and stomatitis. The systemic symptoms of the disease include various lesions, covering almost the entire body. These include: arthritis, glomerulonephritis, myocarditis, pneumonitis and alveolitis, neuritis, endocrine dysfunction, etc.
Clinical forms and stages of the disease
The disease is classified according to several criteria. Depending on the cause that caused the pathology, the disease is divided into the following forms:
- Idiopathic or primary dermatomyositis. It is characterized by the fact that it is impossible to identify the relationship of the disease with any provoking factor.
- Paraneoplastic dermatomyositis. This form of pathology is associated with the presence of a tumor process in the body. It is oncological pathology that is the triggering factor in the development of autoimmune lesions of the connective tissue.
- Children's or juvenile dermatomyositis. This form is similar to idiopathic muscle damage. Unlike adult dermatomyositis, calcification of the skeletal muscle is revealed on examination.
- Combined autoimmune process. It is characterized by signs of dermatomyositis and other connective tissue pathologies (scleroderma, systemic lupus erythematosus).
According to the clinical course of the disease, there are: acute, subacute and chronic process. The first is considered the most aggressive form, as it is characterized by the rapid development of muscle weakness and complications from the heart and respiratory system. With subacutedermatomyositis symptoms are less pronounced. The disease is characterized by a cyclic course with the development of exacerbations and episodes of remission. Chronic autoimmune process proceeds in a milder form. Usually the lesion is in a particular muscle group and does not pass to the rest of the muscles. However, with a long course of the disease, calcification of the connective tissue often occurs, leading to impaired motor function and disability.
In cases where only muscles are involved in the pathological process, without skin and other manifestations, the disease is called polymyositis. There are 3 stages of the disease. The first is called the period of harbingers. It is characterized by muscle pain and weakness. The second stage is the manifest period. It is characterized by an exacerbation of the pathology and the development of all symptoms. The third stage is the terminal period. It is observed in the absence of timely treatment or severe forms of dermatomyositis. The terminal period is characterized by the appearance of symptoms of complications of the disease, such as impaired breathing and swallowing, muscle dystrophy and cachexia.
Diagnostic criteria for pathology
Multiple criteria are needed to make a diagnosis of dermatomyositis. The recommendations developed by the Ministry of He alth include not only instructions for treating the disease, but also for identifying it. The main criteria for pathology include:
- Destruction of muscle tissue.
- Skin manifestations of the disease.
- Laboratory changesindicators.
- Electromyography data.
Striated muscle disease refers to the clinical symptoms of pathology. Among them are hypotension, muscle weakness, soreness and impaired motor function. These symptoms indicate myositis, which may be limited or widespread. This is the main symptom of pathology. In addition to the clinical picture, changes in the muscles should be reflected in the laboratory data. Among them are an increase in the level of enzymes in a biochemical blood test and tissue transformation, confirmed morphologically. The methods of instrumental diagnostics include electromyography, due to which a violation of muscle contractility is detected. Another main criterion for pathology is skin changes. In the presence of the three listed indicators, a diagnosis can be made: dermatomyositis. Symptoms and treatment of the disease are described in detail in the clinical guidelines.
In addition to the main criteria, there are 2 additional signs of pathology. These include impaired swallowing and calcification of muscle tissue. The presence of only these two symptoms does not allow a reliable diagnosis. However, when these features are combined with the 2 main criteria, it can be confirmed that the patient is suffering from dermatomyositis. A rheumatologist is engaged in the identification of this pathology.
Dermatomyositis symptoms
Treatment is carried out on the basis of the symptoms of the disease, which include not only characteristic clinical manifestations, but also test data, as well as electromyography. Only with a full examinationall criteria can be identified and a diagnosis can be made. Their combination allows us to judge the presence of dermatomyositis. Diagnosis includes interviewing and examining the patient, and then performing special studies.
First of all, the specialist draws attention to the characteristic appearance of the patient. Dermatomyositis is especially pronounced in children. Parents often bring babies for examination due to the appearance of purple swelling around the eyes, the appearance of areas of peeling on the skin and reddening of the palms. In the medical literature, you can find many photos of patients, because the disease has various manifestations.
In the general blood test, there is an acceleration of ESR, moderate anemia and leukocytosis. This data indicates an inflammatory process in the body. One of the main laboratory tests is a biochemical blood test. In the presence of dermatomyositis, the following changes are expected:
- Increased levels of gamma and alpha-2 globulins.
- The appearance of large amounts of hapto- and myoglobin in the blood.
- Increased levels of sialic acids and seromucoid.
- Increase in fibrinogen content.
- Increase in ALT, AST and the enzyme - aldolase.
All these indicators indicate an acute lesion of muscle tissue. In addition to the study of biochemical data, an immunological study is carried out. It allows you to confirm the aggression of cells, which normally should protect the body from foreign particles. Another laboratory study is histology. It is carried out quiteoften not only for the diagnosis of pathology, but also to exclude a malignant process. With dermatomyositis, inflammation of the muscle tissue, fibrosis and atrophy of the fibers are noted. Calcification is detected by x-ray.
Treatment of the disease includes a number of activities to cope with autoimmune aggression or temporarily stop it. Therapy should be based on clinical guidelines created by specialists.
Differential Diagnosis
Dermatomyositis is differentiated from other lesions of the muscle tissue, as well as from systemic inflammatory pathologies of the connective tissue. It should be noted that hereditary muscle pathologies often appear at an earlier age, have a rapid course and can be combined with various malformations. Dermatomyositis can be distinguished from other systemic processes by the diagnostic criteria for each of these diseases.
Methods of treating pathology
How is dermatomyositis treated? Clinical guidelines contain instructions that all rheumatologists follow. Pathology treatment begins with hormone therapy. The drugs "Methylprednisolone" and "Hydrocortisone" are used. If the disease progresses against the background of the systemic use of these medicines, pulse therapy is prescribed. It involves the use of hormones in high dosages.
If necessary, cytostatic therapy is carried out, aimed at suppressing autoimmune aggression. To this endprescribe chemotherapy drugs in low doses. Among them are the medicines "Cyclosporine" and "Methotrexate". Frequent exacerbations serve as an indication for plasmapheresis sessions and immunoglobulin injections.
Preventive measures for dermatomyositis
It is impossible to diagnose the disease in advance, so primary prevention has not been developed. To prevent exacerbations, constant dispensary observation and the use of hormonal drugs are necessary. To improve the quality of life, one should abandon possible harmful effects and engage in physical therapy.
We looked at the symptoms and treatment of dermatomyositis. Photos of patients with manifestations of this disease were also presented in the review.
