Juvenile (children) dermatomyositis - diseasefrom the group of diffuse diseases of the connective tissue with a primary lesion of the proximal skeletal muscle and the development of muscle weakness, as well as a purple erythema on the skin.
Children often begins acutely or dermatomyositissubacute in onset of the disease often arise fever, fatigue, malaise, weight loss, myalgia, arthralgia, progressive decrease in muscle strength.
The clinical picture of dermatomyositis usually polisindromna, but the most characteristic changes in the skin and muscles.
Skin lesions - a characteristic featuredermatomyositis. By cutaneous manifestations of dermatomyositis include erythematous rash with a purple tinge on his face (a symptom of "dermatomiozitnyh points") in the neck, above the metacarpophalangeal joint of the hands and legs of the large joints, especially elbow and knee. In the acute phase patients often note the superficial skin necrosis at the site of injury and subsequently developed atrophy with areas of depigmentation. Some patients have redness, flaking and cracking of the skin of palms ( "mechanical hand").
The defeat of the vessels is especially true for children of preschool age.
Over the muscles of the affected limb, and often appears on the face or tight testovatoy edema. Perhaps the development of muscle atrophy.
Typically, in early disease patientsdermatomyositis complain of fatigue during exercise, muscle pain, arising spontaneously and aggravated by palpation and movement. For dermatomyositis is characterized by symmetrical defeat primarily of proximal limb muscles, so that children can not wear a bag in his hands, it is difficult to raise their hands up and hold it in this position, they can not independently comb your hair ( "symptom comb"), dress ( "symptom shirt "), quickly get tired of walking, often fall, can not climb stairs, get up from the chair lift and hold up. In severe lesions of the neck and back muscles patients can not take his head from the pillow, turn around and get out of bed. In the most severe cases, develops generalized muscle weakness with a focus on the proximal group, so that patients can be almost completely immobilized.
With the defeat of the larynx and pharynx appearstwang and hoarseness and swallowing disorder that can lead to aspiration of food and saliva. If it affects the facial muscles note masklike face, with the defeat of the eye muscles. Heavy defeat of the diaphragm and intercostal muscles lead to respiratory failure. The outcome of polymyositis develops muscle wasting.
Children, unlike adults, are often formed persistent, sometimes painful tendon-muscle contraction, sharply limiting the range of motion.
The defeat of the joints is observed in more than 75%patients. Develop arthralgia or arthritis. Most often affects the small joints of the hands (especially the proximal interphalangeal), knee and elbow. Articular changes are characterized by moderate defiguratsiey and tenderness to palpation and movement. In most cases, the articular syndrome quickly docked during treatment, only 25% of patients noted the formation of contractures, deformities and subluxations in the interphalangeal joints with some limited functionality.
Calcification in dermatomyositis in children occurs3-4 times more frequently than in adults. It occurs in almost 40% of patients mainly in the period from 1 year to 5 years after the onset of the disease. Calcinates may be limited in the form of individual lesions or plates and localized subcutaneous or in the connective tissue around the muscle fibers, they can also be located in areas of greatest trauma - around the knee or elbow joints, along the Achilles tendon on the thighs, buttocks, shoulders. Patients with dermatomyositis continuously relapsing course of calcification usually has a diffuse character.
In most developed dermatomyositismyocarditis, manifested mainly arrhythmias and conduction, decreased cardiac contractility. In 25% of patients with mild pericarditis develops severe symptoms quickly disappear after the start of treatment.
The defeat of the lungs manifested non-productive cough, shortness of breath, wheezing fickle. Often children show pleurisy.
The prognosis of juvenile dermatomyositis lessfavorable than in dermatomyositis in adults. Lethal outcomes noted mainly in the first years after the onset of the disease on the background of the high activity of the process and crisis course. Patient survival 5 years after diagnosis averages more than 90%. In the early diagnosis and effective long-term treatment in the majority of patients manage to achieve long-term remission for many years. The worst prognosis was observed in children infected at an early age, and in patients with severe lesions of the gastrointestinal tract and lungs.