Dermatomiosite é doença idiopática inflamatória crônica que afeta musculatura estriada, pele e outros órgãos. Este trabalho tem o objetivo de caracterizar os pacientes com a doença encontrados no HC-FMUSP no período de janeiro de 1992 a dezembro de 2002, avaliando a classificação, as manifestações cutâneas e sistêmicas, achados laboratoriais, terapêuticos e evolutivos em relação à literatura. Este estudo foi baseado nos dados obtidos dos prontuários de 109 pacientes com critérios diagnósticos definidos por Bohan e Peter e modificados por Drake. Os pacientes foram divididos em cinco grupos: 23 dermatomiosites juvenis, 59 dermatomiosites primárias idiopáticas, 6 dermatomiosites amiopáticas, 7 dermatomiosites associada a neoplasias e 14 dermatomiosites associadas a outras doenças do tecido conectivo. Sessenta pacientes foram caracterizados como diagnóstico definido, 33 como possíveis, 4 como prováveis e 12 como amiopáticos. A maior representação foi do sexo feminino (85/109) e a idade média do diagnóstico da doença foi de 36 anos. Manifestações cutâneas foram observadas em todos os pacientes; em relação às alterações sistêmicas a manifestação muscular mais freqüente foi a perda de força proximal (88%); manifestação pulmonar mais comum foi a pneumopatia intersticial (16,5%) e a manifestação digestiva mais observada foi a gastrite (20,2%). Documentaram-se neoplasias durante o seguimento da doença em 6,42% dos casos, sendo mais freqüente nos pacientes acima de 60 anos (71,4%).A enzima muscular alterada na maioria dos casos foi a desidrogenase lática (78,6%). Realizou-se biópsia cutânea em 68 pacientes com alterações compatíveis ao exame anatomopatológico em 78% dos casos. Dentre os casos, 53 pacientes se submeteram à biópsia muscular e 96% deles apresentaram miosite ao exame anatomopatológico. Das 58 eletroneuromiografias efetuadas, mostrou-se padrão miopático compatível com a enfermidade em 81% dos casos. A terapia mais utilizada foram os esteróides e a mortalidade foi de 14,7%, sendo as causas de óbito mais freqüentes a neoplasia maligna, septisemia e infecção pulmonar. / Dermatomyositis is a chronic idiopathic inflammatory disorder which affects the striated skeletal muscles, the skin, and other organs. This present study aims at characterizing the patients who have been affected by this disease and were under treatment at HCFMUSP from January, 1992 to December, 2002 Its classification, the cutaneous and systemical manifestations, as well as laboratorial, therapeutical and evolutive findings were checked out in accordance with the literature. The base for this study was the data obtained from the analysis of 109 patients´records who met not only Bohan\'s and Peter\'s criteria for dermatomyositis diagnosis but also the ones modified by Drake. The patients were divided into five groups: 23 juvenile dermatomyositis, 59 idiopatic primary dermatomyositis, 6 amyopatic dermatomyositis, 7 dermatomyositis associated to malignancy and 14 dermatomyositis associated to connective tissue disorder Sixty of these patients were classified as definite diagnostic, 33 as possible, 4 as probable and 12 as amyopathic. Most patients were female (85/109) and the average age for the diagnosis of the illness was 36. Skin manifestations were observed in all patients, while regarding the systemical alterations, the most frequent was the loss of proximal strength (88%). As the lung manifestation, the most common was an interstitial pneumopathy (16,5%) and as the digestive manifestation, the one which was more commonly observed was gastritis (20,2%). Neoplasies were documented after the following up of the disease in 6,42% of the cases, which was more frequent in patients over 60 years of age (71,4%). The muscle enzyme modified in the majority of the cases was the lactic dehydrogenize (78,6%). Skin biopsy procedure was carried out in 68 patients who showed compatible changes to anatomo-pathological exam in 78% of the cases; 53 patients went under muscular biopsy and 96 % of them proved to have myositis after anatomo-pathological exams. From the fifty-eight electroneuromiographies that were carried out, 81% of the cases showed to have compatible myopathic patterns. Steroidal drugs were the most common therapy used. The mortality rate was 14,7% and the most usual causes of death were malignant neoplasm, sepsis and pulmonary infection.

Dermatomiosite é doença idiopática inflamatória crônica que afeta musculatura estriada, pele e outros órgãos. Este trabalho tem o objetivo de caracterizar os pacientes com a doença encontrados no HC-FMUSP no período de janeiro de 1992 a dezembro de 2002, avaliando a classificação, as manifestações cutâneas e sistêmicas, achados laboratoriais, terapêuticos e evolutivos em relação à literatura. Este estudo foi baseado nos dados obtidos dos prontuários de 109 pacientes com critérios diagnósticos definidos por Bohan e Peter e modificados por Drake. Os pacientes foram divididos em cinco grupos: 23 dermatomiosites juvenis, 59 dermatomiosites primárias idiopáticas, 6 dermatomiosites amiopáticas, 7 dermatomiosites associada a neoplasias e 14 dermatomiosites associadas a outras doenças do tecido conectivo. Sessenta pacientes foram caracterizados como diagnóstico definido, 33 como possíveis, 4 como prováveis e 12 como amiopáticos. A maior representação foi do sexo feminino (85/109) e a idade média do diagnóstico da doença foi de 36 anos. Manifestações cutâneas foram observadas em todos os pacientes; em relação às alterações sistêmicas a manifestação muscular mais freqüente foi a perda de força proximal (88%); manifestação pulmonar mais comum foi a pneumopatia intersticial (16,5%) e a manifestação digestiva mais observada foi a gastrite (20,2%). Documentaram-se neoplasias durante o seguimento da doença em 6,42% dos casos, sendo mais freqüente nos pacientes acima de 60 anos (71,4%).A enzima muscular alterada na maioria dos casos foi a desidrogenase lática (78,6%). Realizou-se biópsia cutânea em 68 pacientes com alterações compatíveis ao exame anatomopatológico em 78% dos casos. Dentre os casos, 53 pacientes se submeteram à biópsia muscular e 96% deles apresentaram miosite ao exame anatomopatológico. Das 58 eletroneuromiografias efetuadas, mostrou-se padrão miopático compatível com a enfermidade em 81% dos casos. A terapia mais utilizada foram os esteróides e a mortalidade foi de 14,7%, sendo as causas de óbito mais freqüentes a neoplasia maligna, septisemia e infecção pulmonar. / Dermatomyositis is a chronic idiopathic inflammatory disorder which affects the striated skeletal muscles, the skin, and other organs. This present study aims at characterizing the patients who have been affected by this disease and were under treatment at HCFMUSP from January, 1992 to December, 2002 Its classification, the cutaneous and systemical manifestations, as well as laboratorial, therapeutical and evolutive findings were checked out in accordance with the literature. The base for this study was the data obtained from the analysis of 109 patients´records who met not only Bohan\'s and Peter\'s criteria for dermatomyositis diagnosis but also the ones modified by Drake. The patients were divided into five groups: 23 juvenile dermatomyositis, 59 idiopatic primary dermatomyositis, 6 amyopatic dermatomyositis, 7 dermatomyositis associated to malignancy and 14 dermatomyositis associated to connective tissue disorder Sixty of these patients were classified as definite diagnostic, 33 as possible, 4 as probable and 12 as amyopathic. Most patients were female (85/109) and the average age for the diagnosis of the illness was 36. Skin manifestations were observed in all patients, while regarding the systemical alterations, the most frequent was the loss of proximal strength (88%). As the lung manifestation, the most common was an interstitial pneumopathy (16,5%) and as the digestive manifestation, the one which was more commonly observed was gastritis (20,2%). Neoplasies were documented after the following up of the disease in 6,42% of the cases, which was more frequent in patients over 60 years of age (71,4%). The muscle enzyme modified in the majority of the cases was the lactic dehydrogenize (78,6%). Skin biopsy procedure was carried out in 68 patients who showed compatible changes to anatomo-pathological exam in 78% of the cases; 53 patients went under muscular biopsy and 96 % of them proved to have myositis after anatomo-pathological exams. From the fifty-eight electroneuromiographies that were carried out, 81% of the cases showed to have compatible myopathic patterns. Steroidal drugs were the most common therapy used. The mortality rate was 14,7% and the most usual causes of death were malignant neoplasm, sepsis and pulmonary infection.