中华皮肤科杂志 ›› 2025, Vol. 58 ›› Issue (8): 699-708.doi: 10.35541/cjd.20250080
中华医学会皮肤性病学分会 中国医师协会皮肤科医师分会
收稿日期:
2025-02-19
修回日期:
2025-06-17
发布日期:
2025-08-05
通讯作者:
肖嵘;高兴华;陆前进
E-mail:xiaorong65@csu.edu.cn; gaobarry@hotmail.com; qianlu5860@pumcderm.cams.cn
基金资助:
Chinese Society of Dermatology; China Dermatologist Association
Received:
2025-02-19
Revised:
2025-06-17
Published:
2025-08-05
Contact:
Xiao Rong; Gao Xinghua; Lu Qianjin
E-mail:xiaorong65@csu.edu.cn; gaobarry@hotmail.com; qianlu5860@pumcderm.cams.cn
Supported by:
摘要: 【摘要】 局限性硬皮病(LS)是一种以真皮及皮下组织纤维化为主要特征的罕见皮肤病,严重者可影响患者容貌、皮肤功能及生活质量。目前国内缺乏LS的相关诊治指南或共识,临床上该病的诊断与治疗缺乏一定的规范性。为提升我国皮肤科临床医生对LS的认识,规范诊治行为,中华医学会皮肤性病学分会、中国医师协会皮肤科医师分会组织国内皮肤病学领域的权威专家,通过系统检索文献,结合国内外研究现状与进展,对LS临床分型、诊断和鉴别诊断、评估方法、治疗方案等进行系统梳理与总结,达成共识,以期为该病规范化诊疗提供指导意见。
中华医学会皮肤性病学分会 中国医师协会皮肤科医师分会. [开放获取] 局限性硬皮病诊疗专家共识(2025版)[J]. 中华皮肤科杂志, 2025,58(8):699-708. doi:10.35541/cjd.20250080
Chinese Society of Dermatology, China Dermatologist Association. Expert consensus on the diagnosis and treatment of localized scleroderma (2025 edition)[J]. Chinese Journal of Dermatology, 2025, 58(8): 699-708.doi:10.35541/cjd.20250080
[1] | Peterson LS, Nelson AM, Su WP, et al. The epidemiology of morphea (localized scleroderma) in Olmsted County 1960-1993[J]. J Rheumatol, 1997,24(1):73⁃80. |
[2] | Keum H, Chen HW, Haley RW, et al. Incidence and prevalence of morphea[J]. JAMA Dermatol, 2024,160(10):1128⁃1130. doi: 10.1001/jamadermatol.2024.2993. |
[3] | Zulian F, Athreya BH, Laxer R, et al. Juvenile localized scleroderma: clinical and epidemiological features in 750 children. An international study[J]. Rheumatology (Oxford), 2006,45(5):614⁃620. doi: 10.1093/rheumatology/kei251. |
[4] | Murray KJ, Laxer RM. Scleroderma in children and adolescents[J]. Rheum Dis Clin North Am, 2002,28(3):603⁃624. doi: 10.1016/s0889⁃857x(02)00010⁃8. |
[5] | Herrick AL, Ennis H, Bhushan M, et al. Incidence of childhood linear scleroderma and systemic sclerosis in the UK and Ireland[J]. Arthritis Care Res (Hoboken), 2010,62(2):213⁃218. doi: 10.1002/acr.20070. |
[6] | 刘盈, 王召阳, 徐子刚, 等. 幼年局限性硬皮病临床特点分析[J]. 中华实用儿科临床杂志, 2024,39(6):445⁃449. doi: 10. 3760/cma.j.cn101070⁃20231227⁃00451. |
[7] | 周海燕, 唐鹤菡, 陈冲, 等. 线状硬斑病107例临床及影像学特点回顾性分析[J]. 中华皮肤科杂志, 2021,54(9):798⁃803. doi: 10.35541/cjd.20201056. |
[8] | Hua VK, Chang J, Laxer RM, et al. Disabling pansclerotic morphoea: a century of discovery[J]. Br J Dermatol, 2025,192(4):585⁃596. doi: 10.1093/bjd/ljae443. |
[9] | Kurzinski KL, Zigler CK, Torok KS. Prediction of disease relapse in a cohort of paediatric patients with localized scleroderma[J]. Br J Dermatol, 2019,180(5):1183⁃1189. doi: 10.1111/bjd.17312. |
[10] | Sator PG, Radakovic S, Schulmeister K, et al. Medium⁃dose is more effective than low⁃dose ultraviolet A1 phototherapy for localized scleroderma as shown by 20⁃MHz ultrasound assessment[J]. J Am Acad Dermatol, 2009,60(5):786⁃791. doi: 10.1016/j.jaad.2008.12.013. |
[11] | Pain CE, Murray A, Dinsdale G, et al. Non⁃invasive imaging and clinical skin scores in juvenile localized scleroderma[J]. Rheumatology (Oxford), 2024,63(5):1332⁃1340. doi: 10.1093/rheumatology/kead380. |
[12] | Chai K, Zhu R, Luo F, et al. Updated role of high⁃frequency ultrasound in assessing dermatological manifestations in autoimmune skin diseases[J]. Acta Derm Venereol, 2022,102:adv00765. doi: 10.2340/actadv.v102.1969. |
[13] | Lepri G, Hughes M, Allanore Y, et al. The role of skin ultrasound in systemic sclerosis: looking below the surface to understand disease evolution[J]. Lancet Rheumatol, 2023,5(7):e422⁃e425. doi: 10.1016/S2665⁃9913(23)00100⁃5. |
[14] | 柴可, 喻江帆, 林彩虹, 等. 基于高频超声的局限性硬皮病皮损分期预测模型的建立及验证[J]. 中华皮肤科杂志, 2023,56(11):1008⁃1015. doi: 10.35541/cjd.20220813. |
[15] | Wang YK, Hao JC, Liu J, et al. Dermoscopic features of morphea and extragenital lichen sclerosus in Chinese patients[J]. Chin Med J (Engl), 2020,133(17):2109⁃2111. doi: 10.1097/CM9. 0000000000000977. |
[16] | Szczepanik⁃Kułak P, Michalak⁃Stoma A, Krasowska D. Usefulness of dermoscopy in localized scleroderma (LoS, Morphea) diagnosis and assessment⁃monocentric cross⁃sectional study[J]. J Clin Med, 2022,11(3):764. doi: 10.3390/jcm110 30764. |
[17] | Martini G, Murray KJ, Howell KJ, et al. Juvenile⁃onset localized scleroderma activity detection by infrared thermography[J]. Rheumatology (Oxford), 2002,41(10):1178⁃1182. doi: 10.1093/rheumatology/41.10.1178. |
[18] | Ranosz⁃Janicka I, Lis⁃Święty A, Skrzypek⁃Salamon A, et al. Detecting and quantifying activity/inflammation in localized scleroderma with thermal imaging[J]. Skin Res Technol, 2019,25(2):118⁃123. doi: 10.1111/srt.12619. |
[19] | Szczepanek M, Frątczak A, Polak K, et al. Narrow⁃band reflectance spectrophotometry and infrared thermography for assessment of skin lesions in localized scleroderma[J]. J Eur Acad Dermatol Venereol, 2022,36(12):2451⁃2458. doi: 10. 1111/jdv.18483. |
[20] | Wang X, Liu M, Ye T, et al. A stretchable hardness sensor for the assessment of skin disease in systemic sclerosis[J]. RMD Open, 2023,9(4):e003512. doi: 10.1136/rmdopen⁃2023⁃003512. |
[21] | Kreuter A, Moinzadeh P, Kinberger M, et al. S2k guideline: diagnosis and therapy of localized scleroderma[J]. J Dtsch Dermatol Ges, 2024,22(4):605⁃620. doi: 10.1111/ddg.15328. |
[22] | Christen⁃Zaech S, Hakim MD, Afsar FS, et al. Pediatric morphea (localized scleroderma): review of 136 patients[J]. J Am Acad Dermatol, 2008,59(3):385⁃396. doi: 10.1016/j.jaad.2008.05.005. |
[23] | Vilela FA, Carneiro S, Ramos⁃e⁃Silva M. Treatment of morphea or localized scleroderma: review of the literature[J]. J Drugs Dermatol, 2010,9(10):1213⁃1219. |
[24] | Kroft EB, Berkhof NJ, van de Kerkhof PC, et al. Ultraviolet A phototherapy for sclerotic skin diseases: a systematic review[J]. J Am Acad Dermatol, 2008,59(6):1017⁃1030. doi: 10.1016/j.jaad.2008.07.042. |
[25] | Scharffetter K, Wlaschek M, Hogg A, et al. UVA irradiation induces collagenase in human dermal fibroblasts in vitro and in vivo[J]. Arch Dermatol Res, 1991,283(8):506⁃511. doi: 10. 1007/BF00371923. |
[26] | Gruss C, Reed JA, Altmeyer P, et al. Induction of interstitial collagenase (MMP⁃1) by UVA⁃1 phototherapy in morphea fibroblasts[J]. Lancet, 1997,350(9087):1295⁃1296. doi: 10. 1016/s0140⁃6736(05)62472⁃5. |
[27] | Yin L, Yamauchi R, Tsuji T, et al. The expression of matrix metalloproteinase⁃1 mRNA induced by ultraviolet A1 (340⁃400 nm) is phototherapy relevant to the glutathione (GSH) content in skin fibroblasts of systemic sclerosis[J]. J Dermatol, 2003,30(3):173⁃180. doi: 10.1111/j.1346⁃8138.2003.tb00368.x. |
[28] | 中国研究型医院学会皮肤科学专业委员会, 中国医师协会皮肤科医师分会. 长波紫外线1皮肤科临床应用专家共识(2022)[J]. 中华皮肤科杂志, 2022,(11). doi: 10.35541/cjd. 20220423. |
[29] | Knobler R, Geroldinger⁃Simić M, Kreuter A, et al. Consensus statement on the diagnosis and treatment of sclerosing diseases of the skin, part 1: Localized scleroderma, systemic sclerosis and overlap syndromes[J]. J Eur Acad Dermatol Venereol, 2024,38(7):1251⁃1280. doi: 10.1111/jdv.19912. |
[30] | Shalaby SM, Bosseila M, Fawzy MM, et al. Fractional carbon dioxide laser versus low⁃dose UVA⁃1 phototherapy for treatment of localized scleroderma: a clinical and immunohistochemical randomized controlled study[J]. Lasers Med Sci, 2016,31(8):1707⁃1715. doi: 10.1007/s10103⁃016⁃2041⁃5. |
[31] | Eisen D, Alster TS. Use of a 585 nm pulsed dye laser for the treatment of morphea[J]. Dermatol Surg, 2002,28(7):615⁃616. doi: 10.1046/j.1524⁃4725.2002.01318.x. |
[32] | 闫小宁, 张建荣, 李美红, 等. 热敷药"治疗局限性硬皮病的临床研究[J]. 世界中西医结合杂志, 2012,7(4):340⁃342. doi: 10.3969/j.issn.1673⁃6613.2012.04.025. |
[33] | 周英. 电针配合刺络拔罐治疗局限性硬皮病52例[J]. 上海针灸杂志, 2008,(11):29. doi: 10.13460/j.issn.1005⁃0957.2008. 11.016. |
[34] | 王红, 韩慧, 吴霞. 火针与薄芝糖肽注射液局部注射治疗局限性硬皮病的疗效比较[J]. 中医临床研究, 2021,(12). doi: 10.3969/j.issn.1674⁃7860.2021.12.037. |
[35] | Zulian F, Martini G, Vallongo C, et al. Methotrexate treatment in juvenile localized scleroderma: a randomized, double⁃blind, placebo⁃controlled trial[J]. Arthritis Rheum, 2011,63(7):1998⁃2006. doi: 10.1002/art.30264. |
[36] | Zulian F, Vallongo C, Patrizi A, et al. A long⁃term follow⁃up study of methotrexate in juvenile localized scleroderma (morphea)[J]. J Am Acad Dermatol, 2012,67(6):1151⁃1156. doi: 10.1016/j.jaad.2012.03.036. |
[37] | Kroft EB, Creemers MC, van den Hoogen FH, et al. Effectiveness, side⁃effects and period of remission after treatment with methotrexate in localized scleroderma and related sclerotic skin diseases: an inception cohort study[J]. Br J Dermatol, 2009,160(5):1075⁃1082. doi: 10.1111/j.1365⁃2133. 2008.09017.x. |
[38] | Weibel L, Sampaio MC, Visentin MT, et al. Evaluation of methotrexate and corticosteroids for the treatment of localized scleroderma (morphoea) in children[J]. Br J Dermatol, 2006,155(5):1013⁃1020. doi: 10.1111/j.1365⁃2133.2006.07497.x. |
[39] | Arthur M, Fett NM, Latour E, et al. Evaluation of the effectiveness and tolerability of mycophenolate mofetil and mycophenolic acid for the treatment of morphea[J]. JAMA Dermatol, 2020,156(5):521⁃528. doi: 10.1001/jamadermatol. 2020.0035. |
[40] | Martini G, Saggioro L, Culpo R, et al. Mycophenolate mofetil for methotrexate⁃resistant juvenile localized scleroderma[J]. Rheumatology (Oxford), 2021,60(3):1387⁃1391. doi: 10.1093/rheumatology/keaa392. |
[41] | Li SC, Torok KS, Ishaq SS, et al. Preliminary evidence on abatacept safety and efficacy in refractory juvenile localized scleroderma[J]. Rheumatology (Oxford), 2021,60(8):3817⁃3825. doi: 10.1093/rheumatology/keaa873. |
[42] | Lythgoe H, Baildam E, Beresford MW, et al. Tocilizumab as a potential therapeutic option for children with severe, refractory juvenile localized scleroderma[J]. Rheumatology (Oxford), 2018,57(2):398⁃401. doi: 10.1093/rheumatology/kex382. |
[43] | McGaugh S, Kallis P, De Benedetto A, et al. Janus kinase inhibitors for treatment of morphea and systemic sclerosis: a literature review[J]. Dermatol Ther, 2022,35(6):e15437. doi: 10.1111/dth.15437. |
[44] | Chimenti MS, Teoli M, Di Stefani A, et al. Resolution with rituximab of localized scleroderma occurring during etanercept treatment in a patient with rheumatoid arthritis[J]. Eur J Dermatol, 2013,23(2):273⁃274. doi: 10.1684/ejd.2013.1929. |
[45] | Diab M, Coloe JR, Magro C, et al. Treatment of recalcitrant generalized morphea with infliximab[J]. Arch Dermatol, 2010,146(6):601⁃604. doi: 10.1001/archdermatol.2010.120. |
[46] | Moinzadeh P, Krieg T, Hunzelmann N. Imatinib treatment of generalized localized scleroderma (morphea)[J]. J Am Acad Dermatol, 2010,63(5):e102⁃104. doi: 10.1016/j.jaad.2010.02.030. |
[47] | Koschitzky M, Khattri S. Apremilast as a treatment for morphea: A case series[J]. JAAD Case Rep, 2022,19:58⁃63. doi: 10.1016/j.jdcr.2021.11.009. |
[48] | Song P, Gocke C, Wigley FM, et al. Resolution of pansclerotic morphea after treatment with antithymocyte globulin[J]. Nat Rev Rheumatol, 2009,5(9):513⁃516. doi: 10.1038/nrrheum. 2009.159. |
[49] | Kumar AB, Blixt EK, Drage LA, et al. Treatment of morphea with hydroxychloroquine: a retrospective review of 84 patients at Mayo Clinic, 1996⁃2013[J]. J Am Acad Dermatol, 2019,80(6):1658⁃1663. doi: 10.1016/j.jaad.2019.01.040. |
[50] | 王特, 刘艺, 陈秋朴, 等. 中医内治法治疗硬皮病研究进展[J]. 天津中医药, 2021,38(3):400⁃403. |
[51] | Strong AL, Rubin JP, Kozlow JH, et al. Fat grafting for the treatment of scleroderma[J]. Plast Reconstr Surg, 2019,144(6):1498⁃1507. doi: 10.1097/PRS.0000000000006291. |
[52] | O'Brien JC, Nymeyer H, Green A, et al. Changes in disease activity and damage over time in patients with morphea[J]. JAMA Dermatol, 2020,156(5):513⁃520. doi: 10.1001/jamadermatol. 2020.0034. |
[53] | Mertens JS, Seyger MM, Kievit W, et al. Disease recurrence in localized scleroderma: a retrospective analysis of 344 patients with paediatric⁃ or adult⁃onset disease[J]. Br J Dermatol, 2015,172(3):722⁃728. doi: 10.1111/bjd.13514. |
[1] | 徐经纬 陈爽 郭克磊 韩立 卞华. 微小RNA调控系统性硬皮病纤维化相关信号通路的研究进展[J]. 中华皮肤科杂志, 2025, 58(8): 788-793. |
[2] | 罗帅寒天 龙海 陆前进. 2024年系统性红斑狼疮研究新进展[J]. 中华皮肤科杂志, 2025, 58(8): 777-780. |
[3] | 中国医师协会皮肤科医师分会痤疮学组 中国研究型医院学会皮肤科学专业委员会 中国中西医结合学会皮肤性病专业委员会痤疮学组. [开放获取] 寻常痤疮临床严重度分级及疗效评价中国专家共识(2025版)[J]. 中华皮肤科杂志, 2025, 58(8): 709-714. |
[4] | 薛珂 陈佳 李斌. 系统性红斑狼疮靶向治疗研究进展[J]. 中华皮肤科杂志, 2025, 58(8): 781-784. |
[5] | 陆前进 曹淑梅 蒋娇. 红斑狼疮研究的现状与挑战[J]. 中华皮肤科杂志, 2025, 58(8): 715-728. |
[6] | 柏琪 朱明芳 邬清婷 姬孝天 杨慧怡 马莉苹 周佳欣. 青藤碱对DNCB诱导的特应性皮炎样模型小鼠皮损改善的作用研究[J]. 中华皮肤科杂志, 2025, 58(8): 759-766. |
[7] | 林尽染 梁晓进 刘庆梅 吴文育. 雄激素性秃发与代谢综合征的关联:从发病机制到治疗策略[J]. 中华皮肤科杂志, 2025, 58(7): 591-594. |
[8] | 吕书影 王英 林文君 杨顶权. 联合托法替布治疗21例匐行性斑秃回顾性分析[J]. 中华皮肤科杂志, 2025, 58(7): 630-635. |
[9] | 王琴 林尽染 刘庆梅 吴文育. 口服米诺地尔在斑秃治疗中的应用[J]. 中华皮肤科杂志, 2025, 58(7): 653-656. |
[10] | 中华医学会皮肤性病学分会毛发学组. 毛发扁平苔藓/前额纤维化性秃发诊疗中国专家共识(2025版)[J]. 中华皮肤科杂志, 2025, 58(7): 583-590. |
[11] | 魏然 李钦峰. 儿童斑秃新型靶向药物治疗研究进展[J]. 中华皮肤科杂志, 2025, 58(7): 650-653. |
[12] | 陶秋薇 陈启韬 邵光辉 李煜乾 朱麒麟 朱晶 李中明 杜旭峰. 硬斑病性秃发研究进展[J]. 中华皮肤科杂志, 2025, 58(7): 664-667. |
[13] | 张莉 宋秀祖. 血管内皮生长因子在雄激素性秃发中的研究进展[J]. 中华皮肤科杂志, 2025, 58(7): 683-685. |
[14] | 邵光辉 李煜乾 陈启韬 朱麒麟 朱晶 李中明 杜旭峰 范卫新. 先天性三角形秃发研究进展[J]. 中华皮肤科杂志, 2025, 58(7): 668-671. |
[15] | 李翔倩 张建中 周城. 系统小分子药物和生物制剂治疗斑秃的临床研究进展[J]. 中华皮肤科杂志, 2025, 58(7): 646-649. |
|