系统性硬化病患者的皮肤表现及其临床意义  

Skin manifestations of patients with systemic sclerosis and their clinical significance

作　　者：崔祥祥 杨骥 屠文震[3] 李明 

机构地区：[1]上海市儿童医院皮肤科,200062 [2]复旦大学附属中山医院皮肤科 [3]上海市中西医结合医院硬皮病专科

出　　处：《中华皮肤科杂志》2018年第1期14-19,共6页Chinese Journal of Dermatology

基　　金：国家自然科学基金（81573043）;上海市自然科学基金（15ZR1406500）

摘　　要：目的 探讨系统性硬化病（SSc）患者的皮损特点及其与临床分型、自身抗体、内脏器官损害之间的关系。方法 对2012—2014年复旦大学附属中山医院皮肤科及上海市中西医结合医院硬皮病专科确诊的120例SSc患者进行分析。结果 120例中，皮损发生率依次为雷诺现象118例（98.3%）、皮肤硬化116 例（96.7%，指背硬化101例，占84.2%）；皮肤肿胀90例（75.0%，手指肿胀84例，占70%）；皮肤异色症77例（64.2%）、口唇变薄75例（62.5%）、毛细血管扩张74例（61.7%）、口周放射性条纹63例（52.5%）、面具脸57例（47.5%）、甲小皮增生49例（40.8%）、甲皱出血点35例（29.2%）、指尖凹陷25例（20.8%）、指腹萎缩24例（20.0%）、指末节缩短24例（20.0%）、指尖溃疡15例（12.5%）。抗Scl-70抗体阳性42例（35.0%），抗着丝点抗体阳性31例（25.8%）。手指肿胀、指尖溃疡、指腹萎缩在抗Scl-70抗体阳性组发生率高于抗Scl-70抗体阴性组（P〈0.05）。指背硬化、皮肤异色症、指尖溃疡、指腹萎缩在抗Scl-70阳性组发生率高于抗着丝点抗体阳性组（P〈0.05）。主要内脏器官损害发生率依次为肺间质病变50%（44/88）、心脏受累47.8%（55/115）、肺动脉高压35.7%（41/115）、食管受累28.3%（34/120）、肾脏受累9.2%（11/120）。弥漫皮肤型系统性硬化病（dcSSc）患者心脏受累和皮肤异色症发生率明显高于局限皮肤型系统性硬化病（lcSSc）患者（P〈0.01）。手指肿胀、指背硬化、皮肤异色症、毛细血管扩张、口唇变薄、口周放射性条纹在SSc 患者早期发生率较高，手指肿胀、指背硬化与肺动脉高压发生率相关性较高；毛细血管扩张、指尖凹陷、指尖溃疡与肺间质病变发生率相关性较高；指腹萎缩与心脏累及发生率相关性较高，差异均具有统计学意义。结论 雷诺现象、手指肿胀、指背硬化、皮肤异色症、毛细血管�Objective To investigate features of skin lesions of patients with systemic sclerosis （SSc）, and to analyze the relationship of skin manifestations with clinical classification, autoantibodies and internal organ involvement. Methods Clinical data were collected from 120 patients with SSc in Department of Dermatology of Zhongshan Hospital Affiliated to Fudan University and Department of Scleroderma of Shanghai TCM-integrated Hospital between 2012 and 2014, and analyzed retrospectively. Results Among the 120 patients with SSc, 118 （98.3%） had Raynaud′s phenomenon, 116 （96.7%） had skin sclerosis, including 101（84.2%） with sclerosis of skin over the dorsum of fingers, 90 （75%） had swollen skin, including 84（70%）with swollen fingers, 77 （64.2%） had poikiloderma, 75 （62.5%） had thinned lip, 74 （61.7%） had telangiectasia, 63 （52.5%） had radial furrowing around the mouth, 57 （47.5%）had mask-like face, 49（40.8%） had hyperplasia of nail cuticle, 35 （29.2%） had petechiae of the nailfolds, 25 （20.8%） had depressed fingertip, 24 （20.0%） had atrophy of the finger pulp, 24（20.0%） had distal finger shortening, and 15 （12.5%） had fingertip ulcer. Anti-Scl-70 antibody and anti-centromere antibody （ACA） were positive in 42 （35.0%） and 31 （25.8%） patients respectively. The incidence of swollen fingers, fingertip ulcer, atrophy of the finger pulp was significantly higher in the anti-Scl-70 antibody-positive group than in the -negative group（P 〈 0.05）, and the incidence of sclerosis of skin over the dorsum of fingers, poikiloderma, fingertip ulcer and atrophy of the finger pulp was all significantly higher in the anti-Scl-70 antibody-positive group than in the ACA-positive group（P 〈 0.05）. The main internal organ involvement included interstitial lung disease （50%, 44/88）, cardiac involvement （47.8%, 55/115）, pulmonary arterial hypertension （35.7%, 41/115）, esophageal involvement （28.3%, 34/120） and kidney involvement （9.2%,

关 键 词：硬皮病 系统性 皮肤表现 自身抗体 早期诊断 纤维化 内脏器官 

分 类 号：R593.2[医药卫生—内科学]