Juvenile dermatomyositis - Inflammatory myopathy of childhood with a primary lesion of the proximal limb muscles, the development of systemic vasculitis, and characteristic skin changes. Observed specific cutaneous manifestations in the form of erythematous rash around the eyes, as well as in the neck and large joints (knee and elbow). Accompanied by progressive muscle weakness, including - of smooth muscles of the respiratory system and digestive system, as well as striated muscle tissue of the heart. It noted the defeat of joints and calcification. Juvenile dermatomyositis myositis confirmed after detection of antibodies in blood. The therapy with corticosteroids and cytotoxic drugs.
dermatomyositis ranks third in prevalence of diffuse connective tissue diseases in childhood. The incidence of 2-3 cases per 1 million children. Girls fall ill about 2 times more likely than boys. The peak incidence occurs in the 3-5 and 7-9 years. Pediatrics Advances in recent years have led to a significant reduction in mortality from juvenile dermatomyositis. Despite this, the disease continues to be relevant to pediatrics and rheumatology in connection with developing muscle atrophy, which can become a cause of child disability. In addition, a danger of early disease with acute onset, when the children for one or more years die from cardio-respiratory failure.
telangiectasia as a symptom of systemic vasculitis. For juvenile dermatomyositis is characterized by vascular disease, which is manifested by symptoms of bowel: a delay chair, esophagitis, gastritis, occasionally possible perforation.
Over sore muscles often forming portions lipodystrophy, against which the muscles of the relief becomes more noticeable. Also, there is synovial swelling around the modified muscle. The skin over the wound site looks pasty. Swelling and observed in the internal organs, such as in heart bag (pericarditis) and around the lungs. It is often accompanied by the phenomena of juvenile dermatomyositis calcification. Calcifications located between the muscle fibers either subcutaneously commonly found around the joints. Sami joints are usually also affected, but polyarthritis quickly relieved by the therapy.
Often in juvenile dermatomyositis found heart failure and myocarditis. Later may develop dilated cardiomyopathy with symptoms of congestive heart failure. Diagnosed pneumonia can be associated with the weakness of the intercostal muscles (hypostatic pneumonia) and with the accidental release of food into the lungs due to difficulties in swallowing. It is important to understand that the basis of such a diverse clinical picture of juvenile dermatomyositis is still the same vasculitis with muscle lesion. Thus, inflammatory changes may occur in the muscle fibers, which are located in all organs.
myasthenia gravis, progressive muscular dystrophy. Moreover, against viral diseases muscle weakness may be a consequence of intoxication.
To confirm the diagnosis of juvenile dermatomyositis is necessary to identify specific antibodies in the blood. Also, be sure to determine the concentration of rhabdomyolysis products and inflammation in the muscles. These indicators include the level of creatine phosphokinase and aldolase in serum. Muscle weakness is confirmed instrumentally using electromyography, detecting characteristic changes in juvenile dermatomyositis. Furthermore, always present in the blood common signs of inflammatory reaction with increased amount of leukocytes, C-reactive protein and erythrocyte sedimentation rate.
pediatric rheumatologist using high doses of short-acting corticosteroids. In severe cases, a pulse therapy with steroids. There is a successful experience of connecting to the cytostatic treatment. Because juvenile dermatomyositis relates to autoimmune diseases, there is a positive effect of plasmapheresis. In remission requires regular monitoring of the state of the muscles, muscle strength. Cutaneous manifestations are reduced by receiving vitamin D and the use of sunscreens. Corticosteroids child continues to take on a permanent basis. Also shown is a physical activity and exercises.
Forecast questionable disease. In recent decades, the mortality rate of juvenile dermatomyositis has dropped significantly and is currently less than 1% of affected children. There are also cases of almost complete recovery of muscle strength. Thus chronic administration of corticosteroids may lead to chronic pathologies of the stomach and intestines, as well as to the development of depressive states (the influence of steroid hormones on the nervous system). Prognosis have cases of early debut and continuously relapsing course of juvenile dermatomyositis. At the same time, timely diagnosis of disease provides a 90% chance of successful treatment.