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Juvenile dermatomyositis
Last reviewed: 23.04.2024
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Juvenile dermatomyositis (juvenile idiopathic dermatomyositis, juvenile dermatopoly myositis) is a serious progressive systemic disease with a predominant lesion of the striated muscles, skin and vessels of the microcirculatory bed.
ICD-10 code
- M33.0. Youthful dermatopoly myositis.
Epidemiology of juvenile dermatomyositis
The prevalence of juvenile dermatomyositis is 3.2 per 1,000,000 children under the age of 17 with variations in different ethnic groups. The disease debuts at any age, but most often it begins at the age of 4-10 years. Girls are more often ill.
Causes of juvenile dermatomyositis
The etiology is unknown. According to modern ideas, juvenile dermatomyositis is a multifactorial disease that develops as a result of antigenic stimulation of an autoimmune response according to the type of molecular mimicry under the influence of environmental factors, in all probability, in genetically predisposed individuals.
Symptoms of juvenile dermatomyositis
The clinical picture of juvenile dermatomyositis is diverse due to the generalized lesion of the microcirculatory bed, but the leading syndromes are cutaneous and muscular.
Classic skin manifestations of juvenile dermatomyositis - a symptom of Gottron and heliotrope rash. Gottron's symptom is erythematous, sometimes flaky skin elements (Gotthron's feature), nodules and plaques (Gottron's papules) that rise above the surface of the skin of the extensor surfaces of proximal interphalangeal, metacarpophalangeal), elbows, knees, and rarely ankle joints. Sometimes Gottron's symptom is represented only by a mild erythema, subsequently completely reversible. Most often, the erythema is located above the proximal interphalangeal and metacarpophalangeal joints and subsequently leaves behind the scars.
Where does it hurt?
What's bothering you?
Classification of juvenile dermatomyositis
Juvenile dermatomyositis is isolated in a separate form in the widely used classification of idiopathic inflammatory myopathies according to A. Bohan and JB Peter. Differences of juvenile dermatomyositis from adults dermatomyositis - common vasculitis, expressed by myalgia, more frequent involvement in the process of internal organs, high incidence of calcification, lack of association with the neoplastic process (except for single cases).
Classification of idiopathic inflammatory myopathies by A. Bohan and JB Peter (1975).
- Primary idiopathic polymyositis.
- Primary idiopathic dermatomyositis.
- Tumor dermatomyositis / polymyositis.
- Juvenile dermatomyositis / polymyositis associated with vasculitis.
- Dermatomyositis / polymyositis in combination with other systemic diseases of connective tissue.
Diagnosis of juvenile dermatomyositis
The ECG reveals signs of metabolic disturbances in the myocardium, tachycardia. With myocarditis, the conduction delay, extrasystoles, and decreased electrical activity of the myocardium were recorded. Occasionally, ischemic changes in the cardiac muscle are observed-the display of generalized vasculopathy affecting the coronary vessels.
How to examine?
What tests are needed?
Treatment of juvenile dermatomyositis
The patient with juvenile dermatomyositis shows early activation to prevent the formation of severe muscular dystrophy, contractures and osteoporosis. As the activity of the disease subsides, the dosed physical exercise is prescribed. Massage is not carried out until the inflammatory activity in muscles is completely suppressed. In the period of remission, rehabilitation therapy in special sanatoriums (sulphurous, radon, rap baths) is possible to reduce the severity of contractures.
Prevention of juvenile dermatomyositis
Primary prevention of juvenile dermatomyositis is not developed. To prevent recurrence of the disease, an adequate reduction in the dose of basic drugs, primarily glucocorticosteroids, is of paramount importance. The main factors leading to exacerbation of juvenile dermatomyositis are rapid reduction and premature elimination of glucocorticosteroids, insolation and vaccination, and infectious diseases.
Forecast
In recent years, due to improved diagnosis and the expansion of the arsenal of medicines, the prognosis of juvenile dermatomyositis has improved significantly. With timely initiated and adequately conducted treatment, most patients succeed in achieving a stable clinical and laboratory remission. According to LA Isaeva, and MA. Zhvania (1978), who observed 118 patients, lethal outcomes were noted in 11% of cases, deep disability - in 16.9% of children. In recent decades, severe functional insufficiency develops in juvenile dermatomyositis in no more than 5% of cases, the share of deaths does not exceed 1.5%.
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