Sign characteristic of dermatomyositis. Juvenile dermatomyositis. Possible complications and consequences

The diagnosis of dermatomyositis or Wagner's disease is difficult to treat. However, if the disease is detected at the first manifestation of symptoms, you can completely get rid of it.

Therefore, it is worth understanding what dermatomyositis is and how it affects the muscles and skin of a person. Let's analyze what the disease is, symptoms and treatment, and also see what the disease looks like in the photo.

Dermatomyositis and polymyositis

Dermatomyositis is a disease in which motor functions are affected, with a strong reddening of the skin caused by the expansion of capillaries. But, if the skin is not affected, then the disease is called polymyositis (systemic inflammation of the muscles, in which the striated muscles of the legs and arms are affected).

The disease can be at a young age, but it is called juvenile dermatomyositis. Together with polymyositis, systemic rheumatic diseases are formed, characterized by muscle inflammation and pain in muscle tissues.

Symptoms and skin manifestations

Treatment of the disease is undulating and growing. Let's analyze the forms of the disease:

There are various signs of an inflammatory nature on the skin, which manifests itself individually or in combination at various stages. Skin changes in dermatomyositis disease appear earlier in muscle tissues - within a couple of months and even years.

Consider the main symptoms of the disease:

• rashes in the form of papules, blisters;
• swelling of the skin and subcutaneous hemorrhages;
• redness on the eyelids, near the eyes, above the lips, on the cheekbones, in the back, neck, on the side walls of the nose;
• scarlet, pinkish, peeling spots over the joints of the fingers, knees;
• excessive dryness of the skin;
• fragility of the nails.

When muscle tissue is disturbed, weakness and low temperature appear. From the first signs of the disease to the symptoms manifested in the bulk of the situation, a period of up to 6 months passes. The doctor describes the increase in violations in the variant of such signs in a patient:

• there are painful sensations in the muscles at rest, as well as during pressure;
• increasing weakness of the muscles of the neck, arms and legs, abdominal zone;
• the patient cannot hold his head;
• abnormal enlargement and hardening of muscles;
• bad speech;
• inability to swallow due to the fact that the patient has a weakening of the muscles of the throat and the alimentary tract.

You can also characterize the appearance of the disease by other signs and highlight the following symptoms:

• temperature up to 39 degrees;
• loss of strands;
• heavy breathing with wheezing;
• oxygen starvation;
• pneumonia;
• fibrosis in the alveolar walls;
• joint pain and weakness;
• diseases of the digestive system;
• protein in the urine due to the destruction of muscle protein compounds.

Causes, treatment, consequences

When it is impossible to determine the cause of Wagner's disease, then the doctor refers to this type of variant of idiopathic dermatomyositis. It may begin acutely, but more often develops slowly, differing in skin and muscle manifestations:

The presence of an allergen has a great influence on the formation of the disease. Sometimes there is a transitional form, between dermatomyositis and connective tissue disease (scleroderma), indicating susceptibility to allergens and heredity.

The main dangers that affect the appearance of the disease can be identified:

• frostbite;
• allergic response to medications;
• hormonal disbalance.

The photo shows the symptoms of dermatomyositis, treatment depends on the specifics of the disease. When signs of muscle damage are expressed, you need to go to a rheumatologist. Also, consultation is necessary with such doctors:

• a doctor specializing in the treatment of various infections;
• a doctor who diagnoses and treats benign and malignant neoplasms;
• doctor who specializes in skin diseases.

The disease should be treated with glucocorticosteroids. These substances are very effective in relieving inflammation, so it is best to start taking drugs at the first manifestations of the disease.

Of the glucocorticoids, the drug Prednisolone is more often used. It is prescribed for admission within 2 weeks. As a result, speech function returns over time, skin swelling, soreness, and weakness decrease.

Rituximab is used in the treatment of rheumatoid arthritis.

For a rash, your doctor may prescribe Hydroxychloroquine.

Diagnostics

Before prescribing drugs, the doctor must be convinced of the diagnosis of dermatomyositis disease, therefore, diagnosis is necessary. To date, there are many devices with which you can conduct an examination. The result of the examinations will determine what treatment the specialist will prescribe, especially when it comes to dermatomyositis disease. Consider the main methods of examination:

Complementary Therapies

Often the doctor prescribes other non-drug methods that are used in the treatment of dermatomyositis. It is important to follow the recommendations of a specialist. Depending on the severity of the symptoms, doctors prescribe additional treatment options:

• treatment of dermatomyositis with exercise to improve muscle function and maintain muscle tissue elasticity;
• speech therapy. Due to the disease of dermatomyositis, the patient has weakness of the swallowing muscles.

When the patient's condition improves, you need to visit a nutritionist, because in this case, foods that are easily digestible should prevail in the diet.

Illness in children

Juvenile (children's) dermatomyositis is characterized by symptoms of muscle inflammation, weakness, which subsequently leads to limitation of physical activity. A characteristic feature of the disease in children, which distinguishes it from adult dermatomyositis, is the formation of the disease without the occurrence of tumors.

The causes of appearance in children are considered more often from the side of the influence of infections. There is a judgment that the disease of dermatomyositis at a young age is due to heredity. Of great importance in the formation of the disease is the irradiation of surfaces with sunlight. The main signs of childish (juvenile) dermatomyositis include muscle inflammation, muscle weakness, dermatological rashes, skin diseases, diseases of the lungs and intestinal tract.

As a result of the extremely rapid spread of the disease in the children's body, it is children who die more often than adults. Deaths were recorded during the first years of the disease. Naturally, if you competently approach the treatment process and follow the doctor's recommendations throughout the entire disease, drink the necessary medicines and improve muscle function in every possible way, then the disease can be overcome. On average, treatment lasts for three years, but in some cases - up to 15 years.

Treatment with folk remedies

Dermatomyositis is difficult to treat, however, with patience, you can achieve results with the use of folk remedies.

It must be understood that herbal treatment is used during a period of decreasing signs and pronounced symptoms. Treatment is carried out in spring and autumn to prevent the occurrence of an exacerbation. The course of treatment lasts for a month.

Consider folk methods of treatment:

1. Treatment as application of compresses and lotions. To prepare a compress, you need ingredients such as willow leaves and buds (1 tbsp each). All components are filled with water and brewed. After cooling, it can be applied to painful areas of the body.
2. You can also use the following recipe and make lotions: take marshmallow (1 tablespoon) and pour it with a glass of boiling water, brew.
3. To prepare ointments, you will need willow and butter. After preparation, the medicine can be applied to the affected areas.
4. Excellent help with dermatomyositis disease is the following composition of ingredients: oats (500 g), milk (liter or one and a half). Send the purchased composition to a small fire and cook for two minutes. After the tincture has cooled, it must be filtered. Treatment lasts for a month, a day you can drink up to a glass of broth.

Forecast and prevention

Today, thanks to the use of effective drugs, the development of the disease of dermatomyositis is restrained, and with the supervision of a qualified doctor, improvement occurs quickly.

So, when the doctor has prescribed the exact dosage of the drug, you do not need to reduce the amount of the drug yourself. It is because of the decrease in dosage that the patient's situation is aggravated.

Dermatomyositis of the protracted stage of the disease, despite therapy, has a high probability of developing complications.

The earlier the diagnosis is determined, the treatment is prescribed, the higher the probability of a complete recovery of the patient. The child may also end up with an absolute recovery or a stable remission.

Measures that would prevent the formation of the disease have not been created to date. However, in clinics, such preventive measures include the following actions:

• maintenance drug therapy;
• periodic examinations by doctors, especially a dermatologist and rheumatologist;
• testing to exclude tumors;
• treatment of inflammatory diseases;
• getting rid of the sources of the infectious process in the body.

Polymyositis and dermatomyositis are rheumatic diseases characterized by inflammation and transformation of muscles (polymyositis) or muscles and skin. A more characteristic dermatological sign is a heliotrope rash. With any of these symptoms listed above, it is imperative to contact the clinic to exclude the appearance of the disease. Below are photos of dermatomyositis in different parts of the body.

Dermatomyositis (DM) is a systemic progressive disease, characterized by a predominant lesion of striated and smooth muscles with impaired motor function, as well as skin. In 60% of the population with classic DM, lesions of the skin and muscles appear simultaneously; it is extremely rare that a form of DM manifests itself as a lesion of the skin only. DM is common in all climatic and geographical zones of the earth and ranks third among systemic connective tissue diseases after systemic scleroderma. The disease can develop at any age and is more common in women. The incidence rate in the population is 1.8 cases per 100,000 patients per year. In the etiopathogenesis of the disease, immune and infectious theories are significant. This article describes a case of a rare form of amyopathic DM, which is characterized by skin lesions, without muscle damage typical of DM. According to various sources, the duration of myositis skin lesions ranges from 6 to 24 months. and more. More common in Asian populations. Actualized issues of etiology and pathogenesis are considered, a modern international classification is given, an algorithm for the diagnostic search and treatment of this disease is described.

Keywords: autoimmune disease, dermatomyositis, amyopathic dermatomyositis, skin symptoms, heliotrope rash, Gottron's symptom, shawl sign, holster symptom, mechanic's hand, Thibierge-Weissenbach syndrome, muscle weakness, topical treatment.

For citation: Orlova E.V., Plieva L.R., Pyatilova P.M., Novosartyan M.G. Dermatomyositis: a clinical case and literature review // BC. Medical review. 2017. No. 11. pp. 850-852

Dermatomyositis: a clinical case and literature review
Orlova E.V., Plieva L.R., Pyatilova P.M., Novosartian M.G.

First Moscow State Medical University named after I.M. Sechenov

Dermatomyositis (DM) is a systemic progressive disease characterized by a predominant lesion of the striated and smooth musculature with impaired motor function and skin lesion. In 60% of the population with classical DM the lesions of the skin and muscles appear simultaneously, the form of DM which manifests itself only by the skin lesion is extremely rare. DM is common in all climatic and geographical third zones of the earth and takes the place after systemic scleroderma among systemic diseases of connective tissue. The disease can develop at any age, and it is more common in women. The incidence rate in the population is 1.8 cases per 100,000 patients per year. The immune and infectious theories are significant in the etiopathogenesis of the disease. This article describes the case of a rare form of amiopathic dermatomyositis, which is characterized by skin lesions, without a typical DM lesion of the muscles. According to various data, the duration of skin lesions without symptoms of myositis is 6 to 24 months and more. It is more common in the Asian population. The article considers the actual issues of etiology and pathogenesis, the modern international classification, the algorithm of diagnostic search and treatment of this disease.

key words: autoimmune disease, dermatomyositis, amiopathic dermatomyositis, skin symptoms, heliotrope rash, Gottron sign, "shawl" symptom, "holster" symptom, "Mechanic hand", Tibierge-Weissenbach syndrome, muscle weakness, topical treatment.
For quote: Orlova E.V., Plieva L.R., Pyatilova P.M., Novosartian M.G. Dermatomyositis: a clinical case and literature review // RMJ. 2017. No. 11. P. 850–852.

The article is devoted to the problem of dermatomyositis

Dermatomyositis (DM) is an autoimmune disease characterized by skin lesions and muscle weakness. The incidence of DM is 1.8 cases per 100,000 patients per year. Women get sick 2 times more often than men. The peak incidence falls on the age of 40 to 50 years.

Etiopathogenesis

Clinical picture

Diagnostics

Treatment of DM requires an individual approach, taking into account the severity, duration and nature of the disease. The drugs of choice are short-acting glucocorticoids: prednisolone, methylprednisolone. With resistance to high doses of glucocorticoids, cytostatics may be used. The most commonly used are methotrexate and azathioprine.

Clinical observation

A differential diagnosis was made between amyopathic dermatomyositis and erythematosis.
An additional laboratory and instrumental examination was carried out.
Deep diagnostic biopsy of subcutaneous fat and adjacent muscle tissue in the focus in the shoulder area:
- the result of a histological examination of the biopsy specimen: the epidermis with a focal reduction in layers, slight hyperkeratosis, acanthosis, the dermoepidermal junction is sealed, in the dermis minor lymphomacrophage infiltrates are located perivascularly or near the hair follicles. Conclusion: changes are nonspecific;
- data of immunohistochemical study of the biopsy: Ig - moderate accumulation in the papillary layer of the dermis (diffuse and granular), no in the basement membrane, widespread fixation in the nuclei of keratinocytes of all layers of the epidermis; IgM - insignificantly in the dermoepidermal zone; IgA - traces in the papillary layer of the dermis, as part of large hyaline bodies; The C3c component of complement is negligible in the papillary and reticular layers of the dermis; fibrin - fixation in the vessels of the dermis. Conclusion: the immunomorphological picture does not contradict the diagnosis of lupus erythematosus.
MSCT of the chest: CT signs of mild lymphadenopathy of the intrathoracic lymph nodes.
ECG: sinus rhythm. Moderate changes in the myocardium.
EMG: in the examined muscles there are signs of an inactive primary muscle process.
Densitometry: indicators within the age norm.
Capillaroscopy: myopathic type (most often such changes occur in DM).
anti-CMV IgG: 616.1 U/mL (>= 6.0 positive), anti-CMV IgM: negative, anti-HSV (types 1 and 2) IgG: 17.7 positivity index (> 1.1 - positive), anti-HSV (type 1 and 2) IgM: negative, anti-EBV IgG-EBNA (nuclear protein): 429 U / ml (>20 - positive), anti-EBV IgM -VCA (capsid protein):<10 Ед/мл.
Biochemical analysis of blood: albumin - 59.8%; α1 - 3.9%; α2 - 9.0%; β1 - 10.4%; γ - 16.9%; CPK total - 94 units / l; AST - 19 units/l; ALT - 21 units/l; LDH - 375 units / l; total bilirubin - 8.1 μmol / l; creatinine - 0.69 mg / dl; albumin - 44.5 g/l; total protein - 69.5 g / l; KA - 2.83; glucose - 8.6 mmol / l; cholesterol - 7.3 mmol / l; triglycerides - 2.80 mmol / l; LDL - 4.14 mmol / l; VLDL - 1.27 mmol / l; HDL - 1.91 mmol / l.
Given the typical clinical picture, capillaroscopy, EMG, the final diagnosis of amyopathic dermatomyositis was made and treatment was carried out: methylprednisolone 24 mg/day and a course of therapeutic plasmapheresis No.

Literature

1. el-Azhary R.A., Pakzad S.Y. Amyopathic dermatomyositis: retrospective review of 37 cases // J Am Acad Dermatol. 2002 Vol. 46. ​​P. 560–565.
2. Jacobson D.L., Gange S.J., Rose N.R., Graham N.M. Epidemiology and estimated population burden of selected autoimmune diseases in the United States // Clin Immunol Immunopathol. 1997 Vol. 84(3). R. 223–243.
3. Bohan A., Peter J.B., Bowman R.L., Pearson C.M. Computer-assisted analysis of 153 patients with polymyositis and dermatomyositis // Medicine (Baltimore). 1977 Vol. 56(4). R. 255–286.
4. Tymms K.E., Webb J. Dermatopolymyositis and other connective tissue diseases: A review of 105 cases // J Rheumatol. 1985 Vol. 12(6). R. 1140–1148.
5. Radenska-Lopovok S.G. The main varieties of inflammatory myopathies: morphological differential diagnosis // Neuromuscular diseases. 2011 #1. pp. 5‒8.
6. Wolf K. et al. Fitzpatrick's Dermatology in Clinical Practice: in 3 volumes / transl. from English. under total ed. acad. A.A. Kubanova. Moscow: Panfilov Publishing House; BINOMIAL. Knowledge Laboratory, 2012. Vol. 2. .
7. Okorokov A.N. Diagnosis of diseases of internal organs. V. 2. Diagnosis of rheumatic and systemic connective tissue diseases. Diagnosis of endocrine diseases. 2000 .
8. Van der Kooi A.J., de Visser M. Idiopathic inflammatory myopathies // Handb Clin Neurol. 2014. Vol. 119. R. 495‒512. doi: 10.1016/B978-0-7020-4086-3.00032-1
9. Rodionov A.N. Dermatovenereology. The Complete Guide for Physicians. St. Petersburg: Science and technology, 2014. 872 p. .
10. Antelava O.A., Radenska-Lopovok S.G., Guseva N.G., Nasonov E.L. Modern approaches to the classification criteria for idiopathic inflammatory myopathies // Scientific and Practical Rheumatology. 2007. No. 5. pp. 41–46. doi: http://dx.doi.org/10.14412/1995-4484-2007-20
11. Sontheimer R.D. A portable digital microphotography unit for rapid documentation of periungualnailfold capillary changes in autoimmune connective tissue diseases // J Rheumatol. March 2004 Vol. 31(3). R. 539‒544.
12. Ghazi E., Sontheimer R.D., Werth V.P. The importance of including amyopathic dermatomyositis in the idiopathic inflammatory myositis spectrum // Clin Exp Rheumatol. 2013 Jan-Feb. Vol. 31(1). R. 128-134. Epub 2012 Nov 22.
13. Hoogendijk J.E., Amato A.A., Lecky B.R. et al. 119th ENMC international workshop: trial design in adult idiopathic inflammatory myopathies, with the exception of inclusion body myositis, 10‒12 October 2003, Naarden, The Netherlands. Neuromuscul Discord. May 2004 Vol. 14(5). R. 337‒345. doi: 10.1016/j.nmd.2004.02.006
14. Dermatovenereology. National leadership. Brief edition / ed. Yu.S. Butova, Yu.K. Skripkina, O.L. Ivanova. M.: GEOTAR-Media, 2013. 514 p. .

Juvenile dermatomyositis- a disease from the group of diffuse connective tissue diseases with a predominant lesion of the proximal skeletal muscles, the development of muscle weakness, as well as purple erythema on the skin. Since the etiology of the disease is unclear, juvenile dermatomyositis is included in the heterogeneous group of idiopathic inflammatory myopathies with the leading clinical manifestation - inflammatory skeletal muscle lesions. According to R.L. Woltman (1994), in addition to juvenile dermatomyositis, this group also includes other myopathies.

Symptoms of juvenile dermatomyositis

In children, dermatomyositis often begins acutely or subacutely; at the onset of the disease, fever, weakness, malaise, weight loss, myalgia, arthralgia, and a progressive decrease in muscle strength often occur. The clinical picture of dermatomyositis is usually polysyndromic, but the most characteristic changes in the skin and muscles.

Skin lesion

Skin lesions are a characteristic sign of dermatomyositis. Skin manifestations of dermatomyositis include erythematous rashes with a purple tint on the face in the paraorbital region (a symptom of "dermatomyositis glasses"), in the décolleté, over the metacarpophalangeal and proximal interphalangeal joints of the hands (Gottron's sign) and over large joints of the extremities, primarily the elbows and knee. In the acute period, patients often have superficial skin necrosis at the sites of injury, and subsequently develop atrophy with areas of depigmentation. Some patients observe redness, peeling and cracking of the skin of the palms ("mechanic's hand").

In children with dermatomyositis, bright livedo usually occurs, especially in the area of ​​the shoulder and pelvic girdle, capillaritis of the palms and feet, and telangiectasia. Generalized vascular lesions are especially characteristic of preschool children.

In acute and subacute course, pronounced trophic disorders are observed in the form of xeroderma, brittle nails, and alopecia.

Subcutaneous tissue injury

Over the affected muscles of the limbs and on the face, a testy or dense edema often appears. Perhaps the development of partial lipodystrophy of the face and limbs, usually combined with muscle atrophy.

Muscle damage

Usually, at the beginning of the disease, patients with dermatomyositis complain of fatigue during physical exertion, muscle pain that occurs spontaneously and is aggravated by palpation and movement. Dermatomyositis is characterized by a symmetrical lesion, primarily of the proximal muscles of the limbs, as a result of which children cannot carry a briefcase in their hands, it is difficult for them to raise their hands up and hold them in this position, they cannot comb their hair (“comb symptom”), get dressed (“symptom shirts”), quickly get tired when walking, often fall, cannot climb stairs, get up from a chair, raise and hold their legs. With severe damage to the muscles of the neck and back, patients cannot tear their heads off the pillow, turn around and get out of bed. In the most severe cases, generalized muscle weakness develops with an emphasis on the proximal group, as a result of which patients can be almost completely immobilized.

When the muscles of the larynx and pharynx are affected, a nasal and hoarse voice appears, as well as a violation of swallowing, which can lead to aspiration of food and saliva. With damage to the facial muscles, a mask-like face is noted, with damage to the oculomotor muscles - diplopia and ptosis of the eyelids. Severe damage to the diaphragm and intercostal muscles leads to respiratory failure. In the outcome of polymyositis, muscle hypotrophy develops.

In children, unlike adults, persistent, sometimes painful tendon-muscle contractures are often formed, which sharply limit the range of motion.

Joint damage

Joint damage is observed in more than 75% of patients. Develop arthralgia or polyarthritis. The most commonly affected joints are the small joints of the hands (mainly proximal interphalangeal), knee and elbow. Articular changes are characterized by moderate defiguration and pain on palpation and movement. In most cases, articular syndrome quickly stops during treatment, only 25% of patients note the formation of contractures, deformities and subluxations in the interphalangeal joints with some limitation of functionality.

Calcinosis

Calcinosis in dermatomyositis in children occurs 3-4 times more often than in adults. It develops in almost 40% of patients, mainly in the period from 1 to 5 years after the onset of the disease. Calcifications can be limited in the form of individual foci or plates and localized subcutaneously or in the connective tissue around muscle fibers, they can also be located in areas of the greatest trauma - around the knee or elbow joints, along the Achilles tendon, on the hips, buttocks, shoulders. In patients with continuously relapsing dermatomyositis, calcification is usually diffuse.

Damage to internal organs

With dermatomyositis, myocarditis most often develops, manifested mainly by rhythm and conduction disturbances, and a decrease in the contractility of the heart muscle. In 25% of patients, pericarditis develops with mild symptoms that quickly disappear after the start of treatment with glucocorticoids.

Lung damage (pneumonitis) is associated with vascular-interstitial changes and is clinically manifested by an unproductive cough, shortness of breath, intermittent wheezing during auscultation. Prognostically unfavorable is the development of diffuse alveolitis with the formation of an alveolar-capillary block, the rapid development of pulmonary insufficiency and death. Damage to the lungs in dermatomyositis can also be due to the development of aspiration and banal hypostatic pneumonia due to damage to the muscles involved in swallowing and breathing. Often, pleurisy is found in children, with a high degree of activity of the process, sometimes accompanied by the formation of exudate.

Kidney damage is rare. The renal syndrome is represented by a transient urinary syndrome, in some cases accompanied by impaired renal function up to the development of acute renal failure due to massive myoglobinuria.

Often in children with a high activity of the process, esophagitis, gastroduodenitis, enterocolitis occur; the development of an erosive-ulcerative process, complicated by perforation and bleeding, is possible. Occasionally, a pseudo-abdominal syndrome is observed, resulting from damage to the muscles of the anterior abdominal wall, with edema, induration and severe pain during breathing and palpation.

Laboratory research

In a laboratory study, patients in the active period of the disease usually reveal an increase in ESR, moderate anemia, in some patients - moderate leukocytosis, hypergammaglobulinemia.

Among the biochemical parameters, characteristic changes reflecting damage to skeletal muscles include an increase in the activity of creatine phosphokinase, as well as aldolase. In addition, patients often show an increase in the concentration of LDH and aminotransferases in the blood serum. Some patients develop myoglobinuria.

The detection of myositis-specific antibodies is important primarily for classification, i.e. clarification of the clinical and immunological subtype of dermatomyositis and polymyositis. In some patients, antibodies to tRNA aminoacyl synthetases are detected, primarily antibodies to histidyl-tRNA synthetase (Jo-1). In the presence of these antibodies in the blood, an antisynthetase syndrome develops, characterized by an acute onset of myositis, interstitial lung damage, fever, symmetrical arthritis, Raynaud's syndrome, skin lesions of the hands like a "mechanic's hand", an incomplete response to the use of glucocorticoids and the frequent development of exacerbations against the background of a decrease in their doses, the debut of the disease mainly in the spring.

Diagnosis of juvenile dermatomyositis

The following criteria for the diagnosis of dermatomyositis have been developed ( Tanimoto et al., 1995).

Skin lesion.

1. Heliotrope rash - red-violet erythematous rash on the eyelids.

   Gottron's sign - red-violet scaly atrophic erythema or spots on the extensor surface of the hands over the metacarpophalangeal and proximal interphalangeal joints.

   Erythema on the extensor surface of the limbs, over the elbow and knee joints.

The article will tell about such a rare and dangerous inflammatory disease as dermatomyositis. Knowing the symptoms of its occurrence, you can prevent the development of pathology in a timely manner. You will also learn what medicines can cure a protracted illness.

Dermatomyositis: general information

Dermatomyositis(Wagner-Unferricht disease/syndrome) - a rare inflammatory disease of a chronic nature. Developing in the human body, it modifies the skin and muscles, affecting their motor function. If the disease affects the smooth and skeletal muscles, while not affecting the skin, which happens in 25% of all clinical cases, then it is diagnosed as polymyositis (another name for dermatomyositis).

For reference! In a year, dermatomyositis is diagnosed in 5 people per 1 million people. The female sex is more susceptible to this type of disease than the male. In adults, the disease occurs more often than in children.

Symptoms of the disease

The first detailed description of dermatomyositis was published in 1940:

1. The disease begins with subfebrile temperature (37.1 - 38 ° C).
2. The patient develops skin photosensitivity (excessive sensitivity to sunlight causes redness, peeling of the skin).
3. Hair falls out, foci of redness appear on the scalp.
4. Pain is felt in the thigh and limbs.
5. The patient quickly loses weight.
6. The skin is damaged: the inner shell of the eyelids and the cornea (the outer shell of the eyes), as well as the area under the eyes, swell.
7. Abnormal redness with a glossy sheen appears on the skin in the area of ​​​​the joints. After these foci disappear, the skin no longer looks like before, it atrophies (the epidermis becomes thinner, becomes wrinkled and dehydrated). Later, poikiloderma develops (hyperpigmentation, dilation of small blood vessels).
8. Inflamed foci (from black-red to blue-red) form on the oral mucosa. Tongue, lips and lower gums swell, painful ulcers appear on them. The mucous membrane of the cheeks, the surface of the tongue and the corners of the lips thicken and exfoliate.
9. The facial expressions are disturbed, it acquires an expression of fear, the forehead is raised high.

For reference! Due to the fact that bluish-purple spots appear on the skin with dermatomyositis, the pathology has received an additional name - “Purple disease”.

Pathological changes in muscles

Following atrophy of the skin, muscle damage occurs. This condition makes itself felt by a feeling of soreness and the inability to relax the muscles of the body. The patient's condition worsens, muscle atrophy sets in, and their physical strength decreases. The flexor muscles are in a contracted state almost all the time, which does not allow the blood to fully deliver nutrients to the muscle fibers.

Changes in smooth muscle function

In addition to damage to the skin and skeletal muscle tissue, dermatomyositis can cause pathology of the heart muscle. As a result, the patient may develop tachycardia (rapid heartbeat), arrhythmia (impaired beat frequency), extrasystole (improper contraction of the heart chambers).

The disease can also negatively affect the smooth muscles of the body, in particular the gastrointestinal tract (gastrointestinal tract), causing dysphagia (swallowing disorder), painful cramps in the stomach, and ulcerative formations on the mucosa.

Other disorders in the body

• Vessels. In some cases, dermatomyositis (Wagner's syndrome) causes spasm of cerebral vessels, causing oxygen starvation of its cells.
• Lungs. Pathology can also affect the lungs and provoke diseases such as bronchopneumonia (inflammation of the walls of the bronchial tree) and the penetration of foreign bodies into the lungs due to swallowing disorders (aspiration).
• Lymphatic system(part of the cardiovascular system). With dermatomyositis, the lymphatic system can fail, which threatens the body with the occurrence of malignant and benign tumors, as well as an enlarged spleen.
• Nerves. In addition to internal organs, dermatomyositis adversely affects the functioning of the patient's nervous system. This condition makes itself felt by a mental disorder and pain when pressing on large nerves, which are located in the lumbosacral zone, in the neck and limbs.
• Skeleton. If dermatomyositis lasts for several months, then pathological changes also affect the skeleton. In a patient against the background of inactivity, skeletal atrophy occurs, which affects the function of the musculoskeletal system.
• Eyes. Atrophic foci appear in the fundus, the retina is affected, which causes visual impairment.

Main reasons

To date, there are no exact causes that can provoke the occurrence of dermatomyositis. Medical scientists attributed this type of pathology to a multifactorial group of diseases (with a hereditary predisposition). A huge role in the development of the disease is played by:

• infectious factors. This is confirmed by the results of studies that have shown that patients who have had an infectious disease several times within 3 months (for example: chlamydia, typhus) increase the chances of developing dermatomyositis.
• Viral diseases. Influenza virus, picornaviruses (cause inflammation in the intestines), parvoviruses (affect the joints without degenerative changes, the skin), predispose to the appearance of DM.
• Bacterial pathogens. Reason: vaccination against typhoid and measles, taking dietary supplements based on growth hormone (Neotropin, Jintropin).
• pathogenic factor. An autoimmune reaction (a condition in which a person's immunity attacks healthy cells of his own body) works against the contents of muscle tissue cells (proteins, ribonucleic acids). Such reactions lead to an imbalance between lymphocytes and do not slow down an overly aggressive immune response.

Other predisposing factors are:

• Hypothermia of the body.
• Sun / heat stroke.
• Injuries of physical and mental type.
• hereditary predisposition.
• Exacerbation of focal infection.
• Allergy to drugs.

Taking into account the fact that there are too many factors that can provoke the development of Wagner's syndrome (dermatomyositis) and they are different, the scientists decided to distribute the disease into classes.

Disease classification

The existing forms of dermatomyositis are distinguished according to the origin and course of the disease:

• Primary.
• Secondary (tumor).
• Children's (juvenile).
• Polymyositis.

Primary (clinical picture)

It is characterized by a weakening of the skeletal muscles, manifestations on the mucous membranes and skin (100% damage, swelling, redness).

Pathology develops gradually, but can also be acute and chronic:

1. In the first case, the disease manifests itself as swelling and hyperemia (overflow of blood vessels) around the eyes and on open parts of the body (face, neck, hands). Pain in the joints and muscles, muscle weakness. The appearance of subfebrile temperature (37.1 - 38 ° C).
2. In acute dermatomyositis (Wagner's syndrome), the patient has a fever with a temperature of 38-39°C. The general state of health deteriorates sharply. There are pronounced inflamed foci on the skin of the face, limbs and torso. Muscles are intensively weakened, up to the loss of motor functions.
3. The chronic form of dermatomyositis is expressed by the appearance of rashes on the skin even before smooth muscle damage, which develops gradually and is not as pronounced as in the first two forms.

secondary syndrome

Pathology makes itself felt with a purple rash on the skin and weakening of the proximal (closest to the body) muscles. Puffiness with a purple tint appears around the eyes.

In 25% of clinical cases in patients, dermatomyositis is combined with the appearance of a malignant neoplasm. In some patients, the disease develops before the appearance of neoplasm (tumor) in 30-60 days, in others simultaneously with the appearance of a neoplasm, in others - after the tumor has been diagnosed. Doctors explain this factor by the age feature and the presence of rheumatic diseases.

The combination of dermatomyositis with a tumor is observed in males 3 times more often than in women. Although it is the female gender that prevails among patients with polymyositis.

The development of tumor dermatomyositis is explained by several factors:

• Possible cross-reaction between muscle and tumor antigens (producers of antibodies that cause the body's immune response).
• Activation of a latent viral infection (for example, cytomegalovirus, herpes of various types) with a subsequent immune response and the development of Wagner's syndrome.

The clinical symptoms of secondary DM may coincide with the primary (idiopathic), but in this case the difference comes down to the lack of response of the diseased organism to the treatment.

Pediatric dermatomyositis

In children, Wagner's syndrome most often appears between the ages of 4 and 10, equally in boys and girls. In almost half of the clinical cases, the pathology begins acutely (sharp muscle pain, rashes, fever appear). The symptoms and course of the disease are similar to the development of dermatomyositis in adults.

Distinctive features come down to:

1. Acute onset of pathology.
2. Severe damage to small vessels of the brain.
3. The presence of fluid in swollen areas, including inflamed joints.
4. To a consequence in the form of salt deposits in soft tissues.

Children's dermatomyositis begins with fever, the appearance of a sharp pain in the muscles. The patient feels general weakness, during the first two days he noticeably loses weight. Separate areas of the skin of the face acquire a lilac hue, or lilac and red rashes appear in the area around the eyes, in some cases the rash is clearly visible on the cheeks, neck, hands, feet, behind and in front of the chest.

For reference! In parallel, it can develop: swelling of the skin and subcutaneous tissue, tissues that envelop the joints can become inflamed (with the appearance of fluid in them).

Muscle weakness increases, this leads to immobility of the patient. Often this condition is accompanied by a feeling of pain, as a result of which sometimes there is a need to clarify the diagnosis. Symptoms of the disease begin to resemble polyarthritis (inflammatory disease of the joints).

Although, if during this period dysphagia (impaired swallowing) and dysphonia (problems with the voice) make themselves felt, then specialists can be sure of the diagnosis of dermatomyositis. Unless the last two disorders are the result of a neurological disease. For this reason, the diagnosis cannot be made without differential diagnosis, which allows to calculate the only probable disease in the patient.

The main cause that can lead to death in children is an intense change in the muscles of the respiratory apparatus, followed by the occurrence of respiratory failure. If the described condition is accompanied by congestive pneumonia (pneumonia with circulatory stagnation) or aspiration (infectious-toxic damage, by inhalation of a foreign body) character, then with insufficient treatment, sometimes with an acute or sluggish course of the disease, death occurs.

Polymyositis (a type of DM)

Pathology affects skeletal muscle tissue, mainly of the limbs. Accompanied by pain, muscle weakness and atrophy. With polymyositis, pathological changes also cover the smooth muscles of the lungs and heart.

In order to diagnose the disease, consult a rheumatologist, therapist, dermatologist. You will be assigned:

• Delivery of analyzes for laboratory tests.
• Electromyography (to assess the functional state of the muscles).
• Biopsy (they will take muscle tissue for study).
• Ultrasound and ECG (for examination of internal organs).

As for the treatment, it is carried out with the help of steroid hormones (Prednisolone tablets). But if there is no result, which happens in 50% of cases, then the patient is prescribed immunosuppressants ("Methotrexate" for intravenous administration).

Important! Alternatively, "Azathioprine" can be used, but with the condition of a monthly assessment of the state of the liver through a biochemical test.

As for the prognosis of polymyositis, the most unfavorable outcome awaits patients with an acute form of the disease, especially with illiterate treatment of the body. The death of the patient may occur as a result of complications such as the occurrence of pulmonary heart disease or the development of pneumonia.

More about the symptoms, diagnosis and treatment of polymyositis will tell the specialist in the proposed video. From it you will find out which doctors you need to get advice from, what tests you need to pass to confirm the diagnosis.

Treatment Methods

After an accurate diagnosis, a patient with dermatomyositis is prescribed treatment with a glucocorticosteroid drug, usually Prednisolone tablets.

• Acute course of the disease - 80-100 mg / day
• Subacute type of pathology - 60 mg / day.
• Chronic dermatomyositis - 30-40 mg / day.

If the dose of the hormonal drug is chosen correctly, then after 7 days the patient's condition begins to return to normal. Initially, the symptoms of intoxication disappear, then after 2 weeks, swelling gradually disappears from the skin. Disappears redness.

The dose of the drug can be increased in the absence of the desired effect. At the same time, the patient cannot take the maximum dosage for more than 60 days, after which the dose is reduced to maintenance.

Note! Medical practice shows that the treatment of dermatomyositis lasts about 2-3 years.

With the body's resistance to glucocorticosteroids, patients are prescribed cytostatics that inhibit pathological processes in the body:

In the treatment of acute and subacute types of pathology with the drug "Prednisolone", during the period of dosage reduction, patients are prescribed quinoline drugs ("Delagil", "Plaquenil"), which have the property of normalizing the body's immune response. If the disease is chronic, then quinoline drugs are prescribed initially.

In addition to these drugs for the treatment of dermatomyositis, B vitamins, Adenosine triphosphoric acid (energy supplier), non-steroidal anti-inflammatory drugs (Ibuprofen, Diclofenac) can be prescribed.

Forecast and prevention

A neglected form of dermatomyositis threatens a person with a fatal outcome. In the first two years of the disease, death overtakes 40% of patients. The reason is reduced to the defeat of the respiratory muscle and the occurrence of gastrointestinal bleeding. Severe pathology with a protracted nature causes narrowing and deformity of the limbs, which causes disability.

Timely treatment of the disease with steroid drugs ("Prednisolone") improves the prognosis for survival. As for prevention, no special measures have been found that can prevent the onset of Wagner's syndrome. Secondary prevention of the disease, which is carried out in order to avoid recurrence, requires that the patient be registered with a rheumatologist and maintain his condition through the medicines prescribed by the doctor.

Question answer

Which doctor should I contact if I suspect dermatomyositis?

Be sure to see a rheumatologist. This specialist treats damage to connective tissue, diseases of the joints, inflammation of the vessels of the skin.

Is it possible to refuse hormonal therapy in DM due to the occurrence of side effects and be treated only with immunoglobulins?

It is impossible, since this type of pathology is treated with large doses of glucocorticosteroids in combination with long-term use. Immunoglobulins should go in combination with hormonal agents, since they only contribute to the normalization of immunity.

Hormones flush out calcium from bones, is there any way to prevent this problem?

For this purpose, with the knowledge of the doctor, you can take 2 tablets / day "Calcemin Advance", drink a course of Omega 3 (fish oil) 2-3 capsules per day. Eat hard cheese and sesame seeds every day.

What to remember:

1. Dermatomyositis is a serious inflammatory disease, so at the first symptoms of its development, you should consult a rheumatologist.
2. Leaving the disease untreated increases the risk of death.
3. It is impossible to cure DM without the use of hormonal drugs.
4. Cancellation of high doses of drugs should occur gradually.
5. There are no preventive measures in relation to DM, but after treating the pathology, it is imperative to register and take prescribed medications in order to maintain health.