RESUMO
27 cases with a diagnosis of Juvenile Dermatomyositis were studied from a total of 1307 patients who suffered from connective tissue diseases; 19 of which met the diagnosis criteria of Bohan and Peters. Females were the most frequently affected. 52.63% of the cases presented the first symptoms between 5 and 9 years of age. Skin lesions, debility in inferior extremities and fever were the most frequent motives of consultation. The Aldolase and LDH were the muscular enzymes whose values increased in the majority of the cases. The electromyography was more sensible than the muscular biopsy in the diagnosis of the disease. We suggest that the epidemiologic and clinical characteristics found in our study should be taken into account for further accurate diagnosis of Juvenile Dermatomyositis in Venezuela.