Despite the rapid development of science and medicine, there are still areas that are not fully explored. These areas include rheumatology. This is a field of medicine that studies systemic connective tissue diseases. Among them are dermatomyositis, lupus erythematosus, scleroderma, rheumatoid arthritis, etc. Despite the fact that all of the above 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 ailment often affects children and young people. Pathology includes a complex of symptoms that make it possible to make a diagnosis: dermatomyositis. Photos of the main manifestations of the disease are quite informative, since the ailment has a pronounced clinical picture. A preliminary diagnosis can be made after an ordinary examination, by changing the appearance of the patient.
Dermatomyositis - what is it?
According to the histological structure, several types of tissues are distinguished. They form all organs and functional systems. The largest area has connective tissue, which consists of the skin, muscles, as well as joints and ligaments. Some diseases affect all these structures, so they are referred to as systemic pathologies. Such ailments include dermatomyositis. Symptoms and treatment of this disease are 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 extend the phases of the calming down 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 muscles, skin, and joints. You can identify the ailment after a full assessment of the clinical picture and the implementation of special diagnostic procedures.
Causes of the disease
The etiology of some pathologies is still being clarified by scientists. Dermatomyositis also belongs to such diseases. Symptoms and treatment, photos of the affected areas - this is the information that is available in large quantities in the medical literature. However, the exact causes of the disease are not mentioned anywhere. There are many hypotheses about the origin of systemic lesions of connective tissue. Among them are genetic, viral, neuroendocrine and other theories. Provoking factors include:
- The use of toxic drugs, as well as vaccination against infectious diseases.
- Prolonged hyperthermia.
- Hypothermia of the body.
- Stay in the sun.
- Infection with rare viruses.
- Menopause and puberty, as well as pregnancy.
- Stressful effects.
- A burdened family history.
It should be borne in mind that such factors do not always cause this disease. Therefore, scientists still can not determine how the pathological process starts. Doctors agree that dermatomyositis is a polyetiological chronic disease. In most cases, the disease occurs during the transitional age.
The mechanism of development of dermatomyositis
Given the fact 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 protective system begins to work incorrectly. Immune cells, which must fight infections and other harmful agents, begin to perceive their own tissues as foreign substances. As a result, the inflammatory process begins in the body. A similar reaction is called autoimmune aggression and is observed with 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 at peak age periods, when there is a hormonal surge in the body. It should be noted that autoimmune aggression directed against their own tissues is only the main stage of pathogenesis, but not the etiology of the disease.
Clinical manifestations of pathology
Since the disease relates 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, unpleasant sensations are not necessarily noted in one place, but can migrate. First of all, the striated muscles, responsible for movements, suffer. Pain occurs in the muscles of the neck, shoulder girdle, upper and lower extremities. A sign of autoimmune muscle damage is the progressive course of the 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, smooth muscle tissue is also involved in the autoimmune process. This leads to respiratory failure, the functioning of the digestive tract and the genitourinary system. Due to damage to smooth muscles, the following symptoms of dermatomyositis develop:
- Dysphagia. It occurs as a result of inflammatory changes and sclerosis of the pharynx.
- Speech impairment. It occurs due to damage to the muscles and ligaments of the larynx.
- Respiratory failure. It develops due to damage to the diaphragm and intercostal muscles.
- Congestive pneumonia. It is a complication of the pathological process, developing 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 visualize the appearance of a patient suffering from this pathology. Skin symptoms include:
- Erythema. This manifestation is considered particularly specific. It is characterized by the occurrence of periorbital purple swelling around the eyes, called the "symptom of glasses."
- Signs of dermatitis are the appearance on the skin of areas of redness, various rashes.
- Hyper- and hypopigmentation. Dark and light areas can be seen on the skin of patients. In the affected area, the epidermis becomes dense and rough.
- Erythema on the fingers, redness of the palmar surface of the hand and striated nails. The totality of these manifestations is called the "Gottron symptom."
In addition, mucous membranes are affected. 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 disruption, etc.
Clinical forms and stages of the disease
The disease is classified according to several characteristics. Depending on the cause of the pathology, the ailment is divided into the following forms:
- Idiopathic, or primary dermatomyositis. It is characterized by the fact that it is impossible to identify the connection 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 starting factor in the development of autoimmune damage to connective tissue.
- Children's, or juvenile dermatomyositis. This form is similar to idiopathic muscle damage. Unlike adult dermatomyositis, the study reveals calcification of skeletal muscle.
- Combined autoimmune process. It is characterized by signs of dermatomyositis and other pathologies of connective tissue (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 subacute dermatomyositis, the symptoms are less pronounced. The disease is characterized by a cyclical course with the development of exacerbations and episodes of remission. Chronic autoimmune process proceeds in a milder form. Typically, a lesion is present in a particular muscle group and does not go 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 precursor period. It is characterized by the occurrence of muscle pain and weakness. The second stage is the manifest period. It is characterized by an exacerbation of 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 respiratory and swallowing disorders, muscle dystrophy and cachexia.
Diagnostic criteria of pathology
Several criteria are needed to make a diagnosis of dermatomyositis. The recommendations developed by the Ministry of Health include not only instructions for treating the disease, but also for identifying it. The main criteria for pathology include:
- Damage to muscle tissue.
- Skin manifestations of the disease.
- Changes in laboratory indicators.
- Electromyography data.
Damage to the striated muscle means the clinical symptoms of pathology. Among them - hypotension, muscle weakness, soreness and impaired motor function. The listed symptoms indicate myositis, which may be limited or common. This is the main symptom of pathology. In addition to the clinical picture, changes in the muscles should be reflected in laboratory data. Among them - an increase in the level of enzymes in the biochemical analysis of blood and tissue transformation, confirmed morphologically. The methods of instrumental diagnostics include electromyography, due to which a violation of contractility of the muscles is detected. Another main criterion for pathology is skin changes. If there are three of these indicators, you can make a diagnosis: 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, with a combination of these signs and 2 main criteria, it can be confirmed that the patient suffers from dermatomyositis. The rheumatologist is involved in the identification of this pathology.
Dermatomyositis: Symptoms
Treatment is based on the symptoms of the disease, which include not only the characteristic clinical manifestations, but also test data, as well as electromyography. Only with a full examination can you identify all the criteria and make a diagnosis. Their combination allows us to judge the presence of dermatomyositis. Diagnostics includes interrogation and examination of the patient, and then - the implementation of special studies.
First of all, the specialist draws attention to the characteristic appearance of the patient. Especially pronounced dermatomyositis in children. Parents often bring their babies for examination due to the appearance of purple swelling around the eyes, the appearance of areas of peeling on the skin and redness 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, an acceleration of ESR, moderate anemia and leukocytosis is observed. 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 in the blood of a large amount of hapto and myoglobin.
- Increased levels of sialic acids and seromucoid.
- Increased fibrinogen content.
- Increased ALT, AST, and the aldolase enzyme.
All these indicators indicate acute damage to 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 test is histology. It is carried out quite often, not only for the diagnosis of pathology, but also to exclude the malignant process. With dermatomyositis, inflammation of muscle tissue, fibrosis and fiber atrophy are noted. Calcification is detected by x-ray.
Treatment of the disease includes a number of measures to cope with autoimmune aggression or temporarily stop it. Therapy should be based on clinical guidelines created by specialists.
Differential diagnosis
Dermatomyositis is differentiated with other lesions of muscle tissue, as well as with systemic inflammatory pathologies of connective tissue. It should be noted that hereditary pathologies of muscles are often manifested at an earlier age, have a rapid course and can be combined with various malformations. Diagnostic criteria of each of these diseases allow distinguishing dermatomyositis from other systemic processes.
Methods of treating pathology
How is dermatomyositis treated? Clinical recommendations contain instructions that all rheumatologists follow. Treatment of pathology begins with hormonal 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 end, chemotherapy is prescribed in low doses. Among them are medicines “Cyclosporin” and “Methotrexate”. Frequent exacerbations serve as an indication for plasmapheresis and immunoglobulin injections.
Preventive measures for dermatomyositis
It is impossible to diagnose the disease in advance, therefore, primary prevention has not been developed. To prevent exacerbations, constant medical supervision and the use of hormonal drugs are necessary. To improve the quality of life, you should abandon the possible harmful effects and engage in physiotherapy.
We examined what the symptoms and treatment of dermatomyositis are. Photos of patients with manifestations of this disease were also presented in the review.