Dermatomyositis: the first symptoms, forms and treatment

Dermatomyositis or Wagner-Hepp-Unferricht disease is a severe systemic pathology that is accompanied by damage to smooth and skeletal muscles. The motor function of patients suffers, connective tissue and skin are affected. The dermis swells, red-purple spots form on it.

Dermatomyositis is not common, but this does not reduce the severity of its course. The pathology is poorly tolerated, the number of deaths is high.

What is dermatomyositis?

Dermatomyositis is a pathology that is accompanied by inflammation of the connective tissue, muscles, skin and internal organs. The disease has a tendency to constant progression.

Dermatomyositis is an autoimmune pathology, that is, it develops due to disturbances in the functioning of the immune system. Why this happens remains a mystery to this day. However, the mechanism of development of pathology is clear. The body begins to perceive its own cells as foreign. The immune system produces antibodies against them, which begin to attack the muscles and connective tissue. They become inflamed, causing the onset of symptoms of the disease. Sometimes the immune system begins to attack the internal organs of the patient.

Dermatomyositis is a pathology that is not common. It is diagnosed in 5 out of a million people a year. Most often, dermatomyositis affects children under 15 years of age and the elderly over 55 years of age. Men get sick twice as much as women.

The main causes of dermatomyositis

As already mentioned, the causes of dermatomyositis have not been established to date. Therefore, pathology is referred to as multifactorial disorders. Antibodies that the body produces begin to attack its own tissues. The endothelium of blood vessels suffers mainly.

Scientists suggest that the development of the disease may be associated with neuroendocrine disorders. Confirmation of this fact is that dermatomyositis most often develops during the transitional periods of a person’s life, for example, during menopause or during puberty.

There is also a theory that dermatomyositis is a consequence of infectious diseases. Moreover, most researchers are inclined to this version.

Studies conducted on the discovery of the causes of dermatomyositis have identified risk factors leading to its development:

infectious diseases. It has been proven that the likelihood of dermatomyositis is higher in people who have had some kind of infectious disease (chlamydia, typhus, etc.) several times within 3 months.
Viruses. Picornaviruses, cytomegaloviruses, influenza, Coxsackie virus, hepatitis B virus, HIV, Lyme disease, toxoplasma, parvoviruses can cause the development of dermatomyositis.
bacteria. Measles and typhoid vaccines can provoke dermatomyositis. There is also a possibility of autoimmune inflammation against the background of taking dietary supplements containing growth hormone. These are such means as Jintropin, Neotropin.
Diseases of the thyroid gland.
genetic factors. HLA-B88 accumulates in the blood of patients with dermatomyositis. Scientists believe that this antigen leads to various immune pathologies.
Pathogenetic factors (autoimmune reaction of the body, accompanied by the formation of autoantibodies). They attack cytoplasmic proteins and RNA (ribonucleic acids), which are part of muscle tissue. These reactions lead to an imbalance between T- and B-lymphocytes, and also provoke rejection of the T-suppressor function.

These factors provoke the development of dermatomyositis most often. There are also secondary causes that increase the likelihood of the disease.

These include:

Overheating or hypothermia of the body.
Received physical injuries.
Emotional shock.
Allergy to medications.
The presence of foci of infection in the body.
Alcohol abuse and drug addiction.

Classification and forms of dermatomyositis

Depending on the etiological factor that provoked the development of dermatomyositis, the following forms are distinguished:

Idiopathic form, that is, it is such a dermatomyositis, the causes of which have not been established. It is diagnosed in 30-40% of cases. It often develops after a routine vaccine has been given. If the idiopathic form of the disease manifests itself in a child, then it is called juvenile. In patients with this form of pathology, the functioning of the sex glands, adrenal glands and hypothalamus decreases. Skeletal muscles become weak, skin and mucous membranes suffer.
Secondary tumor (paraneoplastic) dermatomyositis. It manifests itself due to the formation of malignant tumors in the body. This form of the disease is diagnosed mainly in elderly patients. On the skin appear elements of the rash, which have a purple color. The proximal muscles are weakened, purple edema is visible around the eyes. Symptoms of secondary dermatomyositis may resemble the symptoms of primary dermatomyositis, but with a tumor form of the disease, therapy will be ineffective.
Children’s (juvenile) dermatomyositis. It is diagnosed in 20-30% of cases. In patients, calcification of muscle tissue is detected. The disease itself has an acute onset, which is accompanied by muscle pain, fever and rashes.
Mixed dermatomyositis, which is combined with other connective tissue diseases.

Depending on the nature of the course of the disease, acute, subacute and chronic dermatomyositis are distinguished.

Stages of development of dermatomyositis:

prodromal stage. Symptoms of the disease are bright, they can persist for several days or several months.
Manifesto stage. The patient develops skin, muscle and other symptoms of pathology.
dystrophic stage. It is the most difficult, since the body as a whole suffers with it.

The symptom is dermatomyositis

The disease was first described in detail in 1940.

Now its symptoms are well known to doctors:

At an early stage in the development of the disease, a person has an increase in body temperature. It can persist at subfebrile levels, and can reach 38 ° C and above.
The photosensitivity of the skin increases. When sunlight hits the dermis, it begins to peel off, turns red.
The patient’s hair begins to fall out on the head, and the areas of baldness become red.
The person complains of pain in the limbs and hips.
The weight starts to come off quickly.
The inner lining of the eyelids becomes edematous, the cornea and the area under the eyes also suffer.
In the area of uXNUMXbuXNUMXbthe joints, foci of inflammation form. The skin above them swells, becomes glossy in color, begins to atrophy. Then the patient develops poikiloderma, which is manifested by hyperpigmentation of the skin and expansion of capillaries.
Foci of inflammation form in the mouth. They may be red or bluish in color. The tongue and gums become edematous, after which they become covered with ulcers. The cheeks thicken from the inside, The same applies to the mucous membrane of the lips and tongue.
The facial expression of the patient changes, it becomes as if the person is afraid of something all the time. The patient’s brow ridges are raised high.

Symptoms of skin lesions

With dermatomyositis, the skin is always affected.

This is manifested by symptoms such as:

The appearance of a heliotrope rash. It has a bright red color, is localized in the eyes and looks like glasses. Also, rashes are formed on the wings of the nose, on the cheekbones, in the decollete, on the back, in its upper part.
Sign of Gottron. The rash will be noticeable in the area of uXNUMXbuXNUMXbthe joints of the hands. It has a bright red color. The elements of the rash can be flat or protruding. Often such a rash is flaky.
Erythema. It is localized on the extensor surfaces of the knees and palms. Such spots appear due to the expansion of small blood vessels.
Mechanic’s hand. This symptom got its name due to the fact that in patients with dermatomyositis, the skin of the palms becomes red, flaky, and covered with cracks.
Other skin symptoms: skin itching, photodermatitis, the appearance of spider veins.

Dermatomyositis: the first symptoms, forms and treatment

Symptoms of muscle damage

The main symptom of the disease is damage to muscle tissue.

Pathological manifestations will be as follows:

Muscle weakness that is progressive. It affects both the upper and lower extremities.
Muscle movement is difficult, it is difficult for patients to climb stairs, get into public transport, and get out of bed.
Patients often fall due to muscle weakness.
When the neck muscles suffer, it becomes difficult for a person to raise his head from the pillow and even keep it on weight.
Muscles often swell.
Due to damage to the muscles of the pharynx, throat and esophagus, a person loses his voice, he has difficulty swallowing food, and often chokes.
Muscle atrophy develops with prolonged progression of the disease. They become thinner, lose their natural functions.

Symptoms of joint damage

First of all, small joints are affected in patients. The knees and elbows are less commonly affected. Therefore, the disease is often confused with rheumatoid arthritis. If the patient does not receive treatment, then he develops deforming arthritis, accompanied by subluxations of the joints. However, during the passage of x-rays, erosion is not detected on them.

Symptoms of lesions of the mucous membranes

Patients develop conjunctivitis and stomatitis. The upper palate and the back wall of the pharynx become inflamed. This leads to the fact that the patient becomes difficult to eat.

Calcinosis

Calcinosis develops in patients with progressive dermatomyositis. This pathological sign is manifested by the deposition of calcium in uncharacteristic places: around the muscles, under the skin, over the elbows and knees, over the joints of the fingers, in the buttocks.

Symptoms in the defeat of the respiratory system

With dermatomyositis, the respiratory organs are exposed to inflammation: lungs, pleura and respiratory muscles.

This is manifested by symptoms such as:

The mobility of the chest is limited, ventilation of the lungs is disturbed. This is due to the fact that the tissues of the diaphragm and intercostal muscles suffer. All this becomes a favorable background for the development of pneumonia and other infectious diseases of the respiratory system.
Interstitial pulmonary fibrosis is characterized by inflammation of the interstitial tissue of organs. In the future, they undergo fibrosis, that is, the connective fiber becomes larger, it will be rough, dense. Such cells are not able to provide the full function of respiration.

Symptoms of damage to the cardiovascular and urinary system

Most often, patients do not complain about any pathological symptoms of the heart and blood vessels. Although during the passage of the diagnosis in patients, a violation of the heart rhythm and a deterioration in conduction are detected.

Fibrotic changes in the heart muscle and myocarditis develops infrequently. However, if congestive heart failure occurs, they may appear. Patients are diagnosed with Raynaud’s syndrome, petechiae, vascular infarction of the periungual bed.

Renal failure is rarely diagnosed. The same applies to nephrotic syndrome. Kidney damage can be determined by the presence of protein in the urine.

Symptoms of damage to the central nervous system and kidneys

Kidneys in patients most often do not suffer, although the possibility of pyelonephritis should not be ruled out. Also, the patient can be diagnosed with polyneuritis. Such deviations are most often detected in the hospital, during a comprehensive examination.

Dermatomyositis can provoke various pathologies of the endocrine and reproductive systems. Sometimes girls become infertile. It is also possible violations of the urinary function.

Features of the course of dermatomyositis in children

In childhood, dermatomyositis has some features:

The first symptoms of the disease will be more pronounced.
The body temperature in a child can rise to 39 ° C, fatigue increases.
The child is rapidly losing weight.
Skin manifestations are the same as in adults, but the rashes are more intense.
Petechiae form on the nails and on the eyelids of the child.
The child complains of pain in the body, in the abdomen, back, neck, arms and legs. Increasing muscle weakness.
The joints swell, contractures develop. It is difficult for a child to straighten his knees and elbows.
Calcinosis most often accompanies the childhood form of dermatomyositis.

Which doctor should I go to?

If symptoms of dermatomyositis occur, you should visit a rheumatologist. You will also need to consult narrow specialists: andrologist (for men), gynecologist (for women), urologist, dermatologist, angiologist, cardiologist, neurologist, etc.

Diagnostics

Diagnosis of the patient is reduced to the following points:

The patient is spoken to.
The patient is examined, the condition of his muscles, joints is assessed, their mobility is determined. It is important to listen to the heart and lungs.
Delivery of immunological analysis. Specific antibodies will be found in the blood.
Passing an MRI. The study will detect swelling of muscle tissue.
Needle electromyography. In this case, spontaneous muscle activity will be diagnosed.
Spirography. The study makes it possible to diagnose respiratory failure.
ECG. The study can reveal tachycardia, heart rhythm disturbances, deterioration of cardiac conduction.
X-ray examination makes it possible to visualize calcium deposits in the muscles. They will be located in their deep structures.
Computed tomography makes it possible to diagnose alveolar fibrosis and pneumosclerosis.
Histological examination. The transverse striation disappears, monocytes are infiltrated, their structure is broken, fibrosis develops.
Biopsy of muscle tissue. In the taken material, infiltrates containing mononuclear cells and areas of necrosis are detected. If the disease has a severe course, then the vessels are affected by blood clots, normal muscle cells degenerate into fatty compounds, muscles atrophy.
Laboratory diagnostics involves the delivery of the following tests:
1. General blood analysis. The patient has an increase in ESR, as well as mild anemia.
2. General urine analysis. It contains protein.
3. Blood chemistry. Increased levels of creatine phosphokinase, lactate dehydrogenase, ALT and AST. This indicates damage to the muscles of various localization.

Treatment of dermatomyositis

The approach to the treatment of dermatomyositis should be comprehensive. The patient is prescribed medications, physiotherapy techniques, dietary nutrition. Sometimes surgery is required. If the work of the swallowing muscles is disturbed, then the patient should be engaged with a speech therapist. In Moscow, genetically engineered biological products are used to treat pathology.

The basis of therapy is the use of glucocorticosteroids.

Medication

Medicines allow you to eliminate the inflammatory reaction, do not allow muscle tissue to transform into connective tissue.

The patient may be prescribed drugs such as:

Glucocorticosteroids – they will need to be taken within 2-3 months. After achieving a stable remission, it will be possible to completely cancel the drug.
Cytostatics – they are resorted to in the case when steroid hormone therapy has not made it possible to achieve improvement.
Antispasmodics – allow you to get rid of pain in the abdomen, in blood vessels, etc.
Folic acid, antacids, enveloping preparations. They are prescribed in order to reduce the negative manifestations of other drugs.

Medicines are prescribed courses. After it is possible to achieve a stable remission, the drugs are gradually canceled. Under persistent remission understand the absence of symptoms of the disease for a year or more.

Features of therapy

Features of the treatment of dermatomyositis:

In recent years, genetically engineered biological products have entered the practice. They are assigned on an individual basis.
Since glucocorticosteroids cause damage to the mucous membrane of the stomach and intestines, you need to take drugs to protect them.
If an exacerbation of the pathology occurs, then the person needs to observe bed rest. The return to active life should be smooth, it is important to engage in physical therapy.

Physiotherapeutic treatment

After it is possible to achieve the extinction of the acute process, the patient is prescribed physiotherapy. They allow you to prevent the formation of contractures, lengthen the stage of remission, do not allow the muscles to atrophy.

It is possible to use methods such as:

exercise therapy, massage,
Discrete plasmapheresis in combination with pulse therapy, accompanied by intravenous administration of corticosteroids.
Medicinal electrophoresis.
Lymphocytapheresis.

To improve the effect of the treatment, the patient should be placed in a sanatorium. There, he is shown therapeutic baths, water aerobics, massage and other physiotherapy.

Surgery

Surgery allows you to eliminate the consequences of the disease, which can make a person disabled. The help of a surgeon may be required for patients with contractures, calcifications, cancerous tumors, areas of necrosis, etc.

Possible complications and consequences

If a person does not receive treatment, then the likelihood of developing complications such as:

Formation of bedsores and trophic ulcers.
Contractures, bone deformities.
Anorexia.
Calcification.

If the disease has a long course, then it begins to threaten the life of the patient.

The likelihood of developing such dangerous pathologies as:

Pneumonia, which will be provoked by difficulty in swallowing food. Food particles will get into the respiratory tract of the patient and lead to their inflammation. Coughing to push out foreign bodies does not help. Inflammation is formed around them, which can capture large areas of the lung.
Cardiac arrhythmia due to congestive heart failure. Blood circulation slows down, blood clots begin to form in the vessels. They clog them and can cause the death of tissues that feed the affected vessels.
Ulcerative defects of the digestive tract with bleeding. The stomach wall and intestines may be damaged. This will lead to the development of peritonitis and death of the patient.

If the patient receives treatment, then the terrible consequences of the pathology can be avoided, in contrast to Duchenne myopathy.

Prognosis and prevention

In recent years, the prognosis has been very unfavorable. About 1% of patients died within 5-65 years. The modern treatment regimen allows you to stop the progression of dermatomyositis and save a person’s life.

Preventive measures have not been developed. After the diagnosis of dermatomyositis, the patient is registered with a rheumatologist. He must follow all the recommendations of the doctor and take the drugs prescribed by him.