Dermatomyositis - a rare disease of the connectiveand muscle tissue with impaired motor function, redness of skin, swelling c complicating soft tissue calcification and purulent infections. The disease is more common in women, but sometimes occurs in children.
Causes of this disease are not fully known. Exacerbation of dermatomyositis is prolonged exposure to sunlight and the presence of infectious agents. Some vaccines against mumps, rubella, measles and typhoid fever may also be links of dermatomyositis. This provocateurs disease in adults and children are influenza viruses, parainfluenza and hepatitis B. In addition, the cause may be bacterial pathogens, namely, Lyme disease and streptococcus group A.
The impact of infectious andtoxic-allergic factors, family history, autoimmune shifts play a key role in the progression of dermatomyositis. The pathogenesis of the disease based on the occurrence of immune complexes that hit the wall of small blood vessels.
The clinical manifestations of the disease varied, so common classification divides dermatomyositis origin and current.
- idiopathic - Primary
- paraneoplastic - secondary;
- dermatomyositis in children;
- in combination with other connective tissue diseases.
In course of the disease:
The main symptoms of dermatomyositis and adultchildren are skin rash in the form of red and pink spots and scaly plaques in the extensor surfaces of the joints. In contrast to these cutaneous manifestations, symptoms Gottrona and heliotrope rash in juvenile dermatomyositis sometimes identified dim reddening knots.
The leading features of dermatomyositis, and treatmentThe differential diagnosis of which received considerable attention is the loss of skeletal muscle, lungs, joints, heart, gastrointestinal tract and endocrine organs.
The defeat of the muscles in weaknessmuscle fibers, which greatly limits the possibility of the elementary everyday actions. With the progression of the disease process may involve not only the pelvic and shoulder muscles, but intercostal muscles and the diaphragm muscle, which in turn can lead to respiratory failure. Additionally, degenerative and inflammatory changes in the muscle tissue can cause excessive proliferation of connective tissue in it, and this contributes to the development of muscle contractures.
Pulmonary disease occurs due to weakeningintercostal muscle fibers, which reduces lung ventilation function, thereby greatly complicating the treatment of dermatomyositis. Interstitial pneumonia and fibrosing alveolitis are leading the clinical manifestations of the disease.
soft tissue calcification dermatomyositis feature appears in children. It is based on the deposition of calcium in the muscles and subcutaneous fat.
If it affects the heart in the pathological process involved not only the myocardium, but also all the lining of the heart, and this is manifested by tachycardia heart rhythm disorder.
The involvement in the pathological process smoothmuscles of the intestine - is active dermatomyositis, pathogenesis of which is in violation of circulation in small blood vessels of the mucous membrane of the gastrointestinal tract and manifests gastritis, colitis, ulcer.
Excessive growth of connective tissue in the small and large joints is expressed by stiffness, pain and limitation in the motion.
Endocrine disorders caused by disease progression, change the functional activity of the gonads, adrenal cortex and hypothalamus.
Implemented with lupus erythematosus, systemic sclerosis, myxedema, photodermatosis, trichinosis, polyarteritis nodosa, erythema, etc..
Dermatomyositis, laboratory differentialDiagnosis is made with other cutaneous and systemic diseases is based on the holding of the general, biochemical analysis of blood and histological examination.
In general, the analysis of the blood was moderate growthESR and slight leukocytosis in the biochemical analysis of blood - Increase in liver enzymes. To confirm this diagnosis is used aiming muscle biopsy with subsequent histological examination.
Dermatomyositis involves treatment thatbased on the use of glucocorticoids and cytotoxic drugs to maintain the function of internal organs, as well as medications that improve blood flow in the microvasculature.