[Juvenile dermatomyositis in 12 years old girl]

We would like to present a selected case of 12-aged girl, with recognition of dermatomyositis (DM). At the age of 11 in the normally developing child, erythematous-oedematous changes have appeared on the face, particularly intensified in the vicinity of the orbital cavities (so called: pseudoglasses), as well as erythema and teleangiectasis on the dorsum of hands and small-sized diarthroidal joints (the Gottron's symptom). Subsequently, lower physical efficiency and distinctly weakness in the child's extremities occurred. In EMG (quadriceps muscle of the thigh) myogenous traits have been proven. Neurological examination revealed as follows: muscular weakness (adynamia), mainly lower limbs (grade 3 in the Lovett's scale, along with decreased loss of muscles tone), lack of the periosteal reflex near lower limbs, positive Gower's symptom and increased anterior spinal curvature. In the biochemical examinations accelerated erythrocyte sedimentation rate (ESR), and a rise of activity in muscles enzymes were stated. In the child's blood serum, we disclosed antinuclear antibodies ANA (type of granular luminescence, titre 160), to be rather evident to presence of autoimmunological process. During examinations of the musculocutaneus specimen, DM-markers have been detected. Capillaroscopy proved specific presence of numerous vessels, multiple capillary tubes, individual gemmated vessels and completely invisible dermatomyositous border. Patient was treated with per os sterid--Encorton at the initial dose of 2 mg/day, every other day during the lapse of 6 weeks to reach the normal CPK-activity, and consequently clinical picture under "on-line" surveillance, gradually reducing a specific medicine up to maintenance dose through 18 months. At present, the patient is subjected to check-up and monitoring by Neurological Outpatient and Rehabilitain Clinic for Children.