Dermatomyositis: analysis of 109 patients surveyed at the Hospital das Clínicas (HCFMUSP), São Paulo, Brazil.

BACKGROUND: Dermatomyositis affects striated muscles, skin and other organs. OBJECTIVE: To characterize the disease from January 1992 to December 2002, assessing its classification, cutaneous and systemic manifestations, and also laboratory results, therapeutic and prognostic findings compared to those in the literature. METHODS: Data were obtained from medical records of 109 patients who were classified into five groups: 23 juvenile dermatomyositis; 59 primary idiopathic dermatomyositis; 6 amyopathic dermatomyositis; 7 dermatomyositis associated with neoplasms and 14 dermatomyositis associated with other connective tissue diseases. RESULTS: Sixty patients were classified as "definite" diagnosis; 33 as "possible"; four as "probable" and 12 and as amyopathic. The average age at diagnosis was 36 years. Cutaneous manifestations occurred in all patients; the most frequent symptom was loss of proximal muscle strength; the most common pulmonary disorder was interstitial lung disease, and gastritis was the most prevalent digestive manifestation. Tumors were documented in 6.42% of cases. Lactate dehydrogenase was the muscle enzyme most frequently elevated in the majority of cases. Skin biopsies were performed in 68 patients; muscle biopsies in 53; and electroneuromyographies in 58 patients. The most commonly used treatment was corticotherapy and the mortality rate was 14.7%. CONCLUSION: in this sample, the disease appeared in younger individuals, was more frequent in women and the association with cancer was small.

Dermatomyositis: analysis of 109 patients surveyed at the Hospital das Clínicas (HCFMUSP), São Paulo, Brazil

BACKGROUND: Dermatomyositis affects striated muscles, skin and other organs. OBJECTIVE: To characterize the disease from January 1992 to December 2002, assessing its classification, cutaneous and systemic manifestations, and also laboratory results, therapeutic and prognostic findings compared to those in the literature. METHODS: Data were obtained from medical records of 109 patients who were classified into five groups: 23 juvenile dermatomyositis; 59 primary idiopathic dermatomyositis; 6 amyopathic dermatomyositis; 7 dermatomyositis associated with neoplasms and 14 dermatomyositis associated with other connective tissue diseases. RESULTS: Sixty patients were classified as "definite" diagnosis; 33 as "possible"; four as "probable" and 12 and as amyopathic. The average age at diagnosis was 36 years. Cutaneous manifestations occurred in all patients; the most frequent symptom was loss of proximal muscle strength; the most common pulmonary disorder was interstitial lung disease, and gastritis was the most prevalent digestive manifestation. Tumors were documented in 6.42% of cases. Lactate dehydrogenase was the muscle enzyme most frequently elevated in the majority of cases. Skin biopsies were performed in 68 patients; muscle biopsies in 53; and electroneuromyographies in 58 patients. The most commonly used treatment was corticotherapy and the mortality rate was 14.7%. CONCLUSION: in this sample, the disease appeared in younger individuals, was more frequent in women and the association with cancer was small. .

Ceratodermia palmoplantar de Unna-Thost associada a pseudo-ainhum: relato de um caso / Unna-Thost type palmoplantar keratoderma associated with pseudoainhum: a case report

Os autores relatam um caso de ceratodermia palmoplantar hereditária de Unna-Thost, de apresentação clínica atípica, bem como complicação do tipo estrangulamento ainhumóide no quinto dedo de ambos os pés. Os autores citam ainda outras ceratodermias palmoplantares hereditárias que podem cursar com pseudo-ainhum, bem como os critérios clínicos básicos que diferenciam essas manifestações.

Lúpus eritematoso sistêmico associado a síndorme nefrótica e trombose na veia cava inferior / Systemic lupus erythematosus associated with nephrotic syndrome and vena cava inferior thrombosis

O lúpus eritematoso é doença auto-imune, cujas manifestações variam de lesões cutâneas isoladas ao envolvimento de vários sistemas. A síndrome nefrótica ocorre em carca de 25 por cento dos casos. Caracteriza-se por proteinúria, hipoaluminemia, hipercolesterolemia e edema periférico. Um estado hipercoagulável acompanha essa síndrome, freqüentemente determinando complicações trombóticas. O caso relatado refere-se a uma paciente de 32 anos que apresentava lúpus eritematoso cutâneo crônico e, após seis anos de evolução, desenvolveu síndorme nefrótica complicada por trombose na veia cava inferior. Foi tratada com prednisona, enalapril,furosemida, albumina intravenosa, heparina e, posteriormente, warfarin, apresentando boa resposta. O caso enfatiza a necessidade de seguimento dos portadores de lúpus eritematoso cutâneo crônico a fim de serem detectados sinais de sistematização da doença

Síndrome de Sneddon: relato de um caso e revisão da literatura / Sneddon's syndrome: a case report and revision onf literature

A síndrome de Sneddon caracteriza-se pela associação de livedo racemoso e lesões vasculares cerebrais, podendo acometer outros órgãos internos. Afeta artérias de pequeno e médio calibre da derme profunda e o tecido celular subcutâneo superior, iniciando o processo por uma inflamação do endotélio, que evolui com oclusão, fibrose e atrofia dos vasos. Em alguns casos foi relatada a ocorrência de anticorpos antifostolipídeos. sua evolução é lenta e progressiva, e não há tratamento reconhecidamente efetivo, Relatamos um caso dessa síndrome em uma paciente de 48 anos, em que não foram detectados anticorpos antifosfolipídeos