Juvenile dermatomyositis (DMJ) - Clinical signs

Dermatomiozitei juvenile is very diverse symptoms. The disease can have an insidious onset or may be acute, fulminating.
The clinical picture is dominated by muscle damage manifested by three characteristic signs: muscle pain (myalgia), muscle swelling and muscle fatigue (muscle fatigue) to small efforts. These disorders cause muscular system installing a symmetric motor deficit in muscle belts with a mild impairment of distal muscles of the limbs about 25% of cases. Child's usual activities are limited: can not ride a bicycle, can not run, difficulty in climbing stairs and can sin u-pick arms overhead.In maifestare disease process may be affected striated muscles of the body either.Extrinsic muscles of the eyeball Afecatarea cause diplopia. Can be affected muscles of the neck flexors, muscles of the pharynx by setting up post dysphagia, dysphonia, impairment of sphincter incontinence cause sphincter.Muscle and motor deficits in time will cause the appearance of muscular dystrophy and muscle retractions.
Joint damage in the disease consists of immobilization of joints affected by muscle spasm.
By clinical inspection of the skin sick reamarca presence of a rash of purple hue, which alternate with areas of bluish atrophic, compared with vitiligo. Symptoms worsen skin exposure to sunlight. Frequently affected areas are found: periorbital, nose root, eminent malaria, appearing scalp alopecia areas. Edema in subcutaneous tissue cause skin thickening.
Juvenile dermatomyositis for proper characteristic signs are red-purple coloration of the upper eyelid and heliotrope rash. And mucous membranes are affected, especially the mouth is thin, atrophy and facilitating erosive lesions, sometimes with plates of leukoplakia.The metacarpophalangeal joints, interphalangeal, elbow Gottron sign appears, it is the plates covered with psoriasiform scaling.
Another characteristic sign is the appearance teliangiectaziilor periunghiale disease.There is no proportionality between the severity of skin lesions and muscle.
The percentage of 80% of cases of vital functions is installed disorders manifested by: type restrictive respiratory failure, although not clinically manifest dyspnea or other respiratory disorders.Cardiac consists of: left ventricular hypertrophy evidenced by electrocardiogram (ECG), myocarditis, arrhythmias.
Affecting the digestive system is suggested by the installation of dysphagia, reflux and slow motility highlight of the intestinal absorption, for this purpose is D-xylose test is positive or erratic absorption of prednisone demonstrated, leading to prolongation of its serum half-life .
The kidney is remarkable decrease creatinine clearance below 70 ml / minute.