Dermatomyositis - What Is It? Causes, Forms, Symptoms And Treatment

2024 Author: Josephine Shorter | [email protected]. Last modified: 2024-01-07 17:49

Dermatomyositis: 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, the connective tissue and skin are affected. The dermis swells, red-purple spots are formed on it.

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

Content:

• What is dermatomyositis?
• The main causes of dermatomyositis
• Classification and forms of dermatomyositis
• Dermatomyositis symptoms
• Features of the course of dermatomyositis in children
• Which doctor should I go to?
• Diagnostics
• Dermatomyositis treatment
• Forecast and prevention
• Forecast and prevention

What is dermatomyositis?

Dermatomyositis is a pathology that is accompanied by inflammation of the connective tissue, muscles, skin and internal organs. The disease tends to constantly progress.

If the patient does not develop skin symptoms, then the doctors diagnose not dermatomyositis, but polymyositis.

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

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 old and older people over 55 years old. Men get sick 2 times than women.

The main causes of dermatomyositis

As already mentioned, the causes of dermatomyositis have not yet been established. Therefore, pathology is referred to as multifactorial disorders. The antibodies produced by the body begin to attack their own tissues. Mostly the endothelium of blood vessels suffers.

Scientists suggest that the development of the disease may be associated with neuroendocrine disorders. Confirmation of this fact is that dermatomyositis most often develops precisely 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 towards this version.

The peak incidence of dermatomyositis occurs precisely during seasonal outbreaks of ARVI.

Studies conducted on the detection of the causes of dermatomyositis have revealed the 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 dermatomyositis.
• Bacteria. Vaccines against measles and typhoid can provoke dermatomyositis. There is also the likelihood of autoimmune inflammation while taking dietary supplements containing growth hormone. These are such agents 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 proteins in the cytoplasm and RNA (ribonucleic acids), which are part of muscle tissue. These reactions lead to an imbalance between T- and B-lymphocytes, and also provoke a 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 illness.

These include:

• Overheating or hypothermia of the body.
• Physical trauma received.
• 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 is 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 gonads, adrenal glands and hypothalamus decreases. Skeletal muscles become weak, skin and mucous membranes are affected.
• 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. Elements of a rash with a purple color appear on the skin. The proximal muscles are weakened, and purple edema is visible around the eyes. Symptoms of secondary dermatomyositis may resemble those of primary dermatomyositis, but in case of a tumor form of the disease, therapy will be ineffective.
• Children's (juvenile) dermatomyositis. It is diagnosed in 20-30% of cases. Calcification of muscle tissues is revealed in patients. The disease itself has an acute onset, which is accompanied by muscle pain, fever and rashes.
• Mixed dermatomyositis, which is combined with other diseases of the connective tissue.

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

Stages of dermatomyositis development:

1. Prodromal stage. Symptoms of the disease are vivid and may persist for days or months.
2. Manifest stage. The patient develops skin, muscle and other symptoms of pathology.
3. Dystrophic stage. It is the most difficult, since the body as a whole suffers with it.

Dermatomyositis symptoms

The disease was first described in detail in 1940.

Now her symptoms are well known to doctors:

• At an early stage of the development of the disease, a person's body temperature rises. It can persist at subfebrile levels, and can reach 38 ° C and above.
• The photosensitivity of the skin is increased. When sunlight hits the dermis, it begins to peel off, reddens.
• The patient begins to lose hair on his head, and the areas of baldness become red.
• A person complains of pain in the limbs and hips.
• The weight starts to go away quickly.
• The inner lining of the eyelids becomes edematous, the cornea and the area under the eyes also suffer.
• In the area of the joints, foci of inflammation are formed. The skin above them swells, becomes glossy, begins to atrophy. Then the patient develops poikiloderma, which is manifested by hyperpigmentation of the skin and expansion of capillaries.
• In the mouth, foci of inflammation are formed. They can be red or bluish in color. The tongue and gums become swollen, 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 expression on the patient's face changes, it becomes as if the person is always afraid of something. The supraorbital arches of the patient are raised high.

Sometimes dermatomyositis is called a lilac disease, since the pathology is accompanied by the formation of lilac spots on the skin.

Symptoms of skin lesions

With dermatomyositis, the skin always suffers.

This is manifested by symptoms such as:

• The appearance of heliotropic rash. It has a bright red color, is localized in the eyes and looks like glasses. Also, rashes form on the wings of the nose, on the cheekbones, in the neckline, on the back, in its upper part.
• Gottron's sign. The rash will be visible around the joints of the hands. It has a bright red color. The elements of the rash may be flat or raised. The rash is often 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 cracked.
• Other skin symptoms: itchy skin, photodermatitis, spider veins.

Symptoms of muscle damage

The main symptom of the disease is muscle tissue damage.

Pathological manifestations will be as follows:

• Muscle weakness, which is progressive. It affects both the upper and lower limbs.
• Muscle movement is difficult, patients find it difficult to climb stairs, get on public transport, get out of bed.
• Patients often fall due to muscle weakness.
• When the neck muscles are affected, it becomes difficult for a person to lift his head off the pillow and even keep it in weight.
• Muscles often swell.
• Due to the defeat of the muscles of the pharynx, throat and esophagus, a person's voice disappears, he has difficulty swallowing food, and often chokes.
• Muscle atrophy develops with prolonged progression of the disease. They become thinner and lose their natural functions.

Symptoms of joint damage

First of all, small joints are affected in patients. Knees and elbows are less frequently affected. Therefore, the disease is often confused with rheumatoid arthritis. If the patient does not receive treatment, then he develops deforming arthritis, accompanied by joint subluxation. However, when passing X-ray, erosion is not found on them.

Symptoms of lesions of the mucous membranes

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

Calcification

Calcification 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, above the elbows and knees, above the joints of the fingers, in the buttocks.

Respiratory system symptoms

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

This is manifested by symptoms such as:

• The mobility of the chest is limited, ventilation of the lungs is impaired. This is due to the fact that the tissues of the diaphragm and intercostal muscles are affected. 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 full respiratory function.

Symptoms of damage to the cardiovascular and urinary system

Most often, patients do not complain of any pathological symptoms from the heart and blood vessels. Although during the passage of diagnostics, patients have a heart rhythm disorder and a deterioration in conduction.

Fibrous changes in the heart muscle and myocarditis do not develop often. However, when congestive heart failure occurs, they can appear. Patients are diagnosed with Raynaud's syndrome, petechiae, vascular infarction of the periungual bed.

Renal failure is rarely diagnosed. The same goes for 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

The kidneys in patients most often do not suffer, although the possibility of pyelonephritis should not be ruled out. Also, the patient may be diagnosed with polyneuritis. Such deviations are revealed most often in the hospital, when undergoing a comprehensive examination.

Dermatomyositis can provoke various pathologies of the endocrine and reproductive system. Sometimes girls become sterile. Violations of urinary function are also possible.

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 of a child can rise to 39 ° C, fatigue increases.
• The baby is losing weight quickly.
• Skin manifestations are the same as in adults, but the rash is more intense.
• Petechiae form on the nails and eyelids of the child.
• The child complains of pain in the body, abdomen, back, neck, arms and legs. Muscle weakness increases.
• The joints swell, contractures develop. It is difficult for the child to straighten the knees and elbows.
• Calcification most often accompanies the childhood form of dermatomyositis.

Dermatomyositis in childhood often has a malignant course. Doctors attribute this fact to the fact that the growth processes have not yet been completed, and muscle tissue is actively atrophying.

Which doctor should I go to?

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

Diagnostics

Patient diagnosis is reduced to the following points:

• The patient is interviewed.
• The patient is examined, the condition of his muscles and joints is assessed, and 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.
• Undergoing MRI. The study will detect swelling of muscle tissue.
• Needle electromyography. This will diagnose spontaneous muscle activity.
• 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 muscles. They will be located in their deep structures.
• Computed tomography makes it possible to diagnose alveolar fibrosis and pneumosclerosis.
• Histological examination. Cross striation disappears, monocytes are infiltrated, their structure is disturbed, fibrosis develops.
• Muscle biopsy. In the collected material, infiltrates containing mononuclear cells and areas of necrosis are revealed. If the disease has a severe course, then blood vessels are affected by blood clots, normal muscle cells are reborn 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. Protein appears in it.
  3. Blood chemistry. The level of creatine phosphokinase, lactate dehydrogenase, ALT and AST increases. This indicates damage to muscles of various localization.

It is important to distinguish dermatomyositis from oncomyositis. So, it can be an independent disease, or it can be a consequence of a cancerous tumor growing in the body. Therefore, a full examination of the patient is so important.

Dermatomyositis treatment

The approach to the therapy of dermatomyositis should be comprehensive. The patient is prescribed medications, physiotherapy techniques, dietary food. Sometimes surgery is required. If the work of the swallowing muscles is disrupted, then the patient should work with a speech therapist. In Moscow, genetically engineered biological products are used to treat pathology. Traditional medicine methods turn out to be useless. They can be used only after a medical consultation and in order to prevent the recurrence of pathology.

The basis of therapy is the use of glucocorticosteroids. The drug of choice is Prednisolone.

Maintenance treatment lasts from 3 to 5 years. The drugs allow restraining the pathological reactions of the immune system, preventing inflammation from developing in muscles, skin, and internal organs. The rate of calcification formation slows down, blood microcirculation and metabolic processes in cells improve.

Drug treatment

Medicines allow you to eliminate the inflammatory response, prevent muscle tissue from transforming into connective tissue.

The patient may be prescribed drugs such as:

• High-dose glucocorticosteroids (Prednisolone and Dexamethasone). They will need to be taken within 2-3 months. Then the dose is gradually reduced, bringing to 1 tablet in 7 days. After achieving a stable remission, you can completely cancel the drug.
• Cytostatics. They are used when steroid hormone therapy has failed to improve.
• Papaverine and other antispasmodics. They allow you to get rid of pain in the abdomen, blood vessels, etc.
• Folic acid, antacids, coating preparations. They are prescribed in order to reduce the negative manifestations of other drugs.
• Delagil, Hydroxychloroquine and other aminoquinoline drugs. They help soften the patient's skin.
• Immunoglobulin. This drug is administered intravenously. Its use improves the effect of the use of glucocorticosteroids.
• Retabolil and other anabolic steroids. They help to strengthen the muscles.
• Neostigmine or Proserin, vitamins of group B. These drugs are aimed at correcting muscle functions.
• Etidronic acid prevents the progression of calcification. In addition, Na2 EDTA, Probenecid are injected intravenously. Inside, Colchicine is taken, and Trilon-B ointment is topically applied.
• Pentoxifylline and other vascular drugs can normalize blood microcirculation.
• Anticoagulants (Warfarin, Aspirin, Heparin) are prescribed for antiphospholipid syndrome.
• With tissue necrosis and infectious complications, antibacterial drugs are used.

Medicines are prescribed for courses. After a stable remission is achieved, the drugs are gradually canceled. Stable remission is understood as the absence of symptoms of the disease for a year or more.

It is not recommended to replace Prednisolone with drugs from the NSAID group, as this worsens the prognosis and increases the likelihood of complications.

Features of therapy

Features of the treatment of dermatomyositis:

• In recent years, genetically engineered biological products have been introduced into practice. They are appointed on an individual basis.
• Since glucocorticosteroids lead to damage to the mucous membrane of the stomach and intestines, drugs must be taken to protect them. It can be Omeprazole, Ranitidine. Vitamin D, calcium supplements and bisphosphonates prevent osteoporosis from developing.
• If the patient is shown to take Metipred, then he should not eat sugar and sweet dishes. This will avoid the development of diabetes.
• If an exacerbation of pathology occurs, then a person needs to comply with bed rest. The return to active life should be smooth, it is important to engage in physiotherapy exercises.

GIBP comes from the English "genetically" - genetically, "engineered" - engineering, "biological" - biological, "preparations" - drugs. They are monoclonal antibodies that are of human, murine, humanized and chemical origin. These immunoglobulins are immune cells that are obtained in the laboratory.

Physiotherapy treatment

After it is possible to achieve the extinction of the acute process, the patient is prescribed physiotherapy. They prevent the formation of contracture, lengthen the stage of remission, and prevent muscles from atrophying.

It is possible to use such techniques 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 medicinal baths, water aerobics, massage and other physiotherapy.

Surgery

Surgical intervention 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 pressure sores and trophic ulcers.
• Contractures, bone deformities.
• Anorexia.
• Calcification.

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

The likelihood of developing such dangerous pathologies as:

• Pneumonia, which will be provoked by difficulty in swallowing food. Food particles will enter the patient's respiratory tract and lead to their inflammation. Coughing does not help to push foreign bodies out. Inflammation forms around them, which is able to capture large areas of the lung.
• Heart rhythm disorder due to congestive heart failure. Blood circulation slows down, blood clots begin to form in the vessels. They clog them and can cause the tissue that feeds the affected vessels to die off.
• Ulcerative defects of the gastrointestinal tract with bleeding. The gastric wall and intestines may be affected. 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.

Forecast and prevention

In past years, the forecast has been very unfavorable. Within 1-5 years, about 65% of patients died. The modern treatment regimen allows you to stop the progression of dermatomyositis and save a person's life.

No preventive measures have been developed. After the diagnosis of dermatomyositis, the patient is registered with a rheumatologist. He must follow all the doctor's recommendations and take the drugs prescribed by him.

Self-medication leads to the progression of the disease. About 40% of these patients die from internal bleeding or from respiratory failure. The prognosis of survival in people taking immunosuppressive therapy is improving. However, deformation of the arms and legs cannot be avoided. If the patient develops a cancerous tumor, then the prognosis is determined by oncopathology.

Article author: Mochalov Pavel Alexandrovich | d. m. n. therapist

Education: Moscow Medical Institute. IM Sechenov, specialty - "General Medicine" in 1991, in 1993 "Occupational Diseases", in 1996 "Therapy".