局限性硬皮病522例临床分析

发布时间：2019-04-07 19:59

【摘要】：目的:从流行病学资料、临床表现及分型、实验室资料等方面对局限性硬皮病进行统计分析,进一步研究其临床分型及临床特点。方法:对北京协和医院皮肤科2013年1月—2014年4月门诊就诊的522例局限性硬皮病患者的临床资料进行回顾性分析。结果:患者平均发病年龄为(19.6±16.1)岁,平均病程为(5.6±6.3)年,男女比例为1.0∶2.1。临床亚型中,斑块状硬斑病(44.4%)最常见,其次为线状硬斑病(32.6%)、深部硬斑病(17.0%)、泛发性硬斑病(6.1%)、致残性硬斑病(4.0%)、点滴状硬斑病(2.3%)、瘢痕性硬斑病(0.6%)、大疱性硬斑病(0.2%)。7.3%表现为混合型。30.69%的患者抗核抗体(ANA)阳性,其中泛发性硬斑病、混合型硬斑病、深部硬斑病阳性率较高。结论:局限性硬皮病临床表现上差异较大。实验室检查中,ANA可能与疾病的严重程度相关。组织病理方面,深部硬斑病、部分线状硬斑病、部分泛发性硬斑病和致残性硬斑病累及筋膜,其他亚型间皮损组织病理上无明显差别。
[Abstract]:Aim: to study the clinical classification and clinical characteristics of localized scleroderma by statistical analysis of epidemiological data, clinical manifestations and typing, laboratory data and so on. Methods: the clinical data of 522 patients with localized scleroderma from Department of Dermatology of Peking Union Hospital from January 2013 to April 2014 were analyzed retrospectively. Results: the mean age of onset was (19.6 卤16.1) years, the mean course of disease was (5.6 卤6.3) years, and the ratio of male to female was 1.0 卤2.1. Among the clinical subtypes, plaque scleroderma (44.4%) was the most common disease, followed by linear scleroderma (32.6%), deep scleroderma (17.0%), generalized scleroderma (6.1%) and disability scleroderma (4.0%). Drip scleroderma (2.3%), cicatricial scleroderma (0.6%), bullous scleroderma (0.2%). 7.3% showed mixed type. 30.69% of the patients were positive for anti-nuclear antibody (ANA), including generalized scleroderma. The positive rate of deep scleroderma was higher than that of mixed scleroderma. Conclusion: the clinical manifestations of localized scleroderma are different. In laboratory tests, ANA may be associated with the severity of the disease. In histopathology, deep scleroderma, partial linear scleroderma, partial generalized sclerosclerosis and crippled scleroderma involved fascia, but there was no significant difference in histopathology among other subtypes of mesotheliocutaneous lesions.
【作者单位】：中国医学科学院北京协和医学院北京协和医院皮肤科;
【分类号】：R593.25

【参考文献】