1.General information
Dermatomyositis (DM) is one of the rare, systemic (affecting more than one organ or area) and severe diseases. In the literature, synonymous names are used - lilac disease, Wagner (Unferricht-Hepp) disease - and the direct translation of the term means inflammatory damage to the skin and muscles. From a clinical and nosological point of view, this should also include damage to connective tissue, blood vessels, lungs, joints, etc., but in this case, of course, the diagnosis would be unacceptably long.
Dermatomyositis affects females twice as often as males. The predominant age intervals for the primary diagnosis of DM are from 5 to 15 and over 40 years. According to the type of course, the disease is divided into acute, subacute and chronic dermatomyositis.
About a third of all patients with dermatomyositis do not exhibit clinically significant skin symptoms; Such patients are diagnosed with polymyositis.
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Symptoms of pathology
The clinical picture of the disease is gradually developing. At the onset of dermatomyositis, one can detect increasing weakness of muscle tissue, which can worsen over several years. Acute development is not particularly characteristic of dermatomyositis. The main symptoms may be accompanied by skin irritation and Raynaud's syndrome.
Raynaud's syndrome
There is weakness in the neck and arms, which can interfere with daily work. In more advanced forms of the disease, patients find it difficult to get out of bed, hold their head up, and find it difficult to walk independently.
Damage to the muscles of the throat and upper digestive canals leads to unclear 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 infection of the skin. Swelling of the face, a rash on the cheeks, in the area of the nasolabial folds, chest, and shoulder blades appear.
2. Reasons
Dermatomyositis is a polyetiological process, i.e. occurs (like rheumatic diseases, to the group of which it is often classified) under the influence of one of many possible causes, or a combination of them, or without any apparent reason at all. Typically, such a statement is alarming, meaning that either the real cause has not yet been established, or the disease is triggered by a rare combination of some unfavorable factors and/or conditions. In the case of EBM, both are true to a certain extent. Today, the theoretical understanding of the etiopathogenesis of dermatomyositis is dominated by infectious-toxic hypotheses, and the role of both viral and bacterial agents is statistically confirmed. In turn, pathogenic viruses and bacteria that cause (or can cause) the development of DM are also very numerous and heterogeneous. In addition, researchers have repeatedly drawn attention to the regional and seasonal dependence of the frequency of occurrence of DM, suggesting a provoking effect of direct intense sunlight.
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Prevention and prognosis of dermatomyositis
In severe and advanced forms of the disease, the risk of death in the first couple of years of development of the pathology is close to 30-40% of all cases, especially as a result of respiratory tract disease and gastric hemorrhage. Despite the severity of the disease, contractures appear and limbs are gradually lost, which later ends with the patient becoming disabled. Timely provision of assistance and establishment of a therapeutic course will help prevent the development of the disease and improve the further prognosis.
If the patient received timely assistance, complications will not arise
No special preventive recommendations for the treatment of pathology have been developed.
3. Symptoms, diagnosis
Many causes - many consequences; in other words, the clinical picture of dermatomyositis is very diverse. On the skin side, the most typical changes are the so-called. Gottron's symptom and heliotrope rash: various spots of a reddish or purple hue, plaques on the outer side of the finger phalanges and other joints, rashes in various areas (neck, back, abdomen, etc.), branching purple-bluish spots, in the early stages - changes in the nails cuticle
Another characteristic symptom of DM is progressive muscle weakness in the limbs, fatigue from less and less exercise, and sometimes muscle pain. As the disease progresses, the muscles of the respiratory system and upper gastrointestinal tract may be involved in the process, which leads to difficulties in breathing, swallowing, and speech. At certain stages, contractures (joint blocking), pneumonia and other areas of inflammation may appear.
In addition to skin and muscle disorders (as axial symptoms), the development of DM with varying frequency is accompanied by damage to the digestive, endocrine, genitourinary, cardiovascular, musculoskeletal systems, calcification (calcification) of soft tissues and other severe, even severe disabilities or death - complications.
There are several types of dermatomyositis: idiopathic (this is the case when the disease has to be explained by a combination of purely individual unfavorable characteristics of the body), tumor, juvenile (childhood and adolescence), combined.
The diagnosis of “dermatomyositis” is established mainly clinically (i.e., by direct examination, study of anamnesis, complaints and dynamics), sometimes only after a series of additional consultations with specialized specialists. In some cases, laboratory analysis methods (usually quite complex), biopsy, and electromyography as a way to study muscle status are also informative and indicated.
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Necessary patient care
- The sleeping place of a person with dermatomyositis should be comfortable, soft and keep warm. Affected joints should be protected from possible microtraumas.
- If prescribed by a doctor, apply a warm compress to the joints.
- It is necessary to constantly monitor the patient’s medications, remind them of the need to take them, and monitor compliance with the dosage. Monitor possible deviations from the usual state and emerging complications during treatment.
- Monitoring the patient's hygiene. If necessary, provide assistance during changing bed and personal linen, and taking a bath. If there is pain in the joints and muscles, excessive weakness, the patient is unable to perform these simple actions alone.
- The patient's diet should contain essential vitamins, especially vitamins B, C and D, and not contain many fatty acids. It is worth reducing the use of table salt. Food should be sufficiently high in calories and well digestible by the stomach, the diet should be balanced.
- Regular performance of therapeutic exercises. Physical exercises should be aimed at working with affected muscles and joints, individual muscle groups. If dermatomyositis is detected, it is worth performing special gymnastic exercises for the facial muscles. During exercise, active and passive loads occur on all joints. Treatment procedures should occur at a time of low activity of immunoinflammatory processes with full monitoring of the patient’s condition.
- Taking into account the fact that treatment requires a lot of time, there is a possibility of various complications arising from the medications used (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 him to positive thoughts and charging him with good emotions. It is equally important to talk to 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, familiarizing them with possible complications and convincing them to be tolerant when providing the patient with the necessary help and support.
- It is worth helping the patient with the optimal organization of his day, thinking about his working hours and rest periods. It is better to refuse night work, minimize physical activity and protect from emotional outbursts.
Patients with dermatomyositis require constant care and monitoring.
4.Treatment
Currently, anti-inflammatory hormonal therapy using glucocorticosteroids is considered the most effective therapeutic strategy for the treatment of dermatomyositis. Means of normalizing metabolism (metabolism), stimulating blood circulation and tissue nutrition are also used. In some cases, physical therapy and massage are indicated for the prevention of joint mobility disorders. If there is no effect from these regimens, rheumatology-specific cytostatics are prescribed. Developing complications are treated symptomatically (for example, when muscle deficiency increases, anabolic hormones can be prescribed; in the case of a tumor, appropriate surgical intervention is performed). An important role is played by vitamin complexes.
In most cases, it is possible to achieve high-quality remission or significant improvement.
Let's sum it 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 faster he will be able to live life to the fullest again.
Unfortunately, it is difficult to prevent such a pathology, since the exact cause of the development of the disease has not yet been discovered. However, if a person leads a healthy lifestyle, the risk of developing such a disease will be minimized.
Pathogenesis
The pathogenetic process begins with a nonspecific effect - hypothermia, excessive solar insolation , vaccination , acute intoxication , trauma and other exogenous factors. It is associated with the presence of infectious agents introduced into the genome of muscle cells, changes in neuroendocrine reactivity or genetic predisposition - the presence of HLA histocompatibility antigens associated with B8 or DR3 .
With dermatomyositis, an immune-inflammatory reaction develops, aimed at the destruction of altered intranuclear structures of muscle and skin cells. Due to cross-reactions, cell populations related to antigens are involved in immune damage. Microphage mechanisms of elimination of immune complexes from the body lead to activation of fibrogenesis and systemic inflammation of small vessels. The hyperreactivity of the immune system is aimed at destroying the intranuclear structure of the virion; it is manifested by the presence in the bloodstream of antibodies Mi2, Jo1, SRP, as well as autoantibodies to nuclear proteins and other soluble antigens.
Classification
In approximately 25-30% of cases, there are no skin symptoms and then they speak of a special case of this systemic disease - polymyositis .
Depending on the origin and course, the following are distinguished:
- primary idiopathic dermatopolymyositis;
- the secondary paraneoplastic or tumor type occurs in individuals over 55 years of age against the background of cancer of the breast , lungs , ovaries , prostate , uterus , stomach , colon , hemoblastosis and other malignant neoplasms;
- juvenile - children's;
- polymyositis , combined with other diffuse connective tissue diseases.
Juvenile dermatomyositis: features of the course
It is distinguished by focal calcification of soft tissues such as muscles, epidermis and subcutaneous fat. Deposits of calcium salts - hydroxyapatites - can be deposited on the surface and deep, forming limited single or diffusely located nodules and pseudotumors. Superficial plaques with calcifications cause inflammatory reactions of surrounding tissues; they rot and are rejected in the form of a crumbly mass.
Subcutaneous calcifications
As in adults, classic rashes and Gottron's sign are observed. Gradually increasing muscle weakness begins with a bizarre gait of the child, which further constrains movement even more, preventing the child from playing, running and caring for himself.
Juvenile dermatomyositis