局限性硬皮病522例临床分析  

Clinical characteristics of 522 patients with localized scleroderma

作　　者：樊雪[1] 朱晨雨[1] 毛笑非[1] 李芸[1] 杜伟[1] 方凯[1] 渠涛[1] 邢泽宇[1] 王菂 孙秋宁[1] 

机构地区：[1]中国医学科学院北京协和医学院北京协和医院皮肤科,北京100730

出　　处：《临床皮肤科杂志》2016年第1期5-10,共6页Journal of Clinical Dermatology

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。Objective： To determine the clinical characteristics of localized scleroderma （LSc）. Methods： We analyzed epi- demiological, clinical and laboratory features of 522 patients with LSc in our hospital from January 2013 to April 2014. Re- suits： The mean age of onset was （19.6±16.1） years; The mean disease duration at diagnosis was （5.6±6.3） years. Male to female ratio was 1.0：2.1. Plaque morphea was the most common subtype （44.4%）, followed by Linear scleroderma（32.6%）, deep morphea（17.0%）, generalized morphea（6.1%）, disabling morphea（4.0%）, guttate morphea（2.3%）, keloid morphea（0.6%） and bullous morphea（0.2%）. Mixed subtype accounted for 7.3%. 30.69% of the patients were antinuclear antigen（ANA） pos- itive. Conlusion： ANA positive rates were higher in generalized morphea, mixed subtype and deep morphea. We concluded that clinical features of localized scleroderma vary greatly. ANA may be associated with the severity of the disease. Histopathologically, deep morphea, linear scleroderma, generalized morphea and disabling morphea can involve the fascia. Oth- er subtypes have no differences in pathological features.

关 键 词：硬皮病 局限性 临床分析 

分 类 号：R593.2[医药卫生—内科学]