线状硬斑病107例临床及影像学特点回顾性分析

doi:10.35541/cjd.20201056

中华皮肤科杂志 ›› 2021, Vol. 54 ›› Issue (9): 798-803.doi: 10.35541/cjd.20201056

线状硬斑病107例临床及影像学特点回顾性分析

周海燕¹ 唐鹤菡² 陈冲² 吕玲¹ 吕小岩¹

¹四川大学华西医院皮肤科，成都 610041；²四川大学华西医院放射科，成都 610041
第一作者现在四川大学华西公共卫生学院/华西第四医院皮肤科工作

收稿日期:2020-10-30 修回日期:2021-05-26 发布日期:2021-09-02
通讯作者: 吕小岩 E-mail:lxiaoyan@scu.edu.cn

Clinical and imaging characteristics of 107 patients with linear morphea: a retrospective analysis

Zhou Haiyan¹, Tang Hehan¹, Chen Chong¹, Lyu Ling², Lyu Xiaoyan²

¹Department of Dermatology, West China Hospital, Sichuan University, Chengdu 610041, China; ²Department of Radiology, West China Hospital, Sichuan University, Chengdu 610041, China
Zhou Haiyan is working at West China School of Public Health and West China Fourth Hospital, Sichuan University, Chengdu 610041, China

Received:2020-10-30 Revised:2021-05-26 Published:2021-09-02
Contact: Lyu Xiaoyan E-mail:lxiaoyan@scu.edu.cn

摘要/Abstract

摘要： 【摘要】目的分析不同亚型线状硬斑病（LM）的临床表现、实验室及影像学检查特点, 对LM的诊断和病情评估方法提出建议。方法回顾性分析2018年1月至2019年12月四川大学华西医院皮肤科门诊经临床和/或病理确诊的LM患者的临床资料。结果共纳入107例LM患者，肢体LM 63例，刀劈状硬斑病22例，进行性颜面偏侧萎缩和嗜酸性筋膜炎各11例。88例患者接受改良局限性硬皮病皮肤严重指数（mLoSSI）和皮肤损伤指数（LoSDI）评分，mLoSSI评分为0～51分，LoSDI评分为0～40分。10例嗜酸性筋膜炎患者行血常规检查，4例嗜酸性粒细胞增高。88例LM患者行抗核抗体检查，13例（14.8%）阳性（滴度 ≥ 1∶320）。4例进行性颜面偏侧萎缩患者行核磁共振成像（MRI）检查2例出现同侧大脑半球萎缩及对侧白质区T2高信号；肢体LM 28例中26例（92.9%）MRI表现为肌筋膜增厚，嗜酸性筋膜炎患者11例MRI均可见皮下脂肪间隔和肌筋膜增厚。结论 mLoSSI和LoSDI可对LM的疾病活动性、严重程度及预后做出初步评估；对临床提示有深部受累的患者，建议行MRI检查。

关键词: 线状硬斑病, 肢体线状硬斑病, 刀劈状硬斑病, 进行性颜面偏侧萎缩, 嗜酸性筋膜炎

Abstract: 【Abstract】 Objective To analyze clinical, laboratory and imaging characteristics of different subtypes of linear morphea （LM）, and to propose an appropriate approach to the diagnosis and severity assessment of LM. Methods Clinical data were collected from patients with clinically and/or pathologically confirmed LM in Department of Dermatology, West China Hospital, Sichuan University from January 2018 to December 2019, and retrospectively analyzed. Results A total of 107 patients with LM were enrolled into this study, including 63 with LM of the limbs/trunk , 22 with morphea en coup de sabre, 11 with progressive hemifacial atrophy and 11 with eosinophilic fasciitis. Disease severity was evaluated by using the modified localized scleroderma skin severity index （mLoSSI） and localized scleroderma skin damage index （LoSDI） scores in 88 patients, with the mLoSSI scores ranging from 0 to 51 points, and the LoSDI scores ranging from 0 to 40 points. Routine blood examination of 10 patients with eosinophilic fasciitis showed increased eosinophil counts in 4 patients. Thirteen （14.8%） of 88 patients with LM were positive for antinuclear antibody, with titers of ≥ 1∶320. Magnetic resonance imaging （MRI） examination showed ipsilateral cerebral hemisphere atrophy and contralateral white matter hyperintensity on T2-weighted images in 2 out of 4 patients with progressive hemifacial atrophy, myofascial thickening in 26 out of 28 patients with LM of the limbs/trunk （92.9%）, subcutaneous septal and myofascial thickening in all 11 patients with eosinophilic fasciitis. Conclusions The preliminary assessment of disease activity, severity and prognosis of LM can be made by mLoSSI and LoSDI. MRI examination is recommended for patients with clinical signs of involvement of subcutaneous structures.

Key words: Linear morphea, Linear morphea of the limbs/trunk, Morphea en coup de sabre, Progressive hemifacial atrophy, Eosinophilic fasciitis

周海燕唐鹤菡陈冲吕玲吕小岩. 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

Zhou Haiyan, Tang Hehan, Chen Chong, Lyu Ling, Lyu Xiaoyan. Clinical and imaging characteristics of 107 patients with linear morphea: a retrospective analysis[J]. Chinese Journal of Dermatology, 2021, 54(9): 798-803.doi:10.35541/cjd.20201056

参考文献 29

［1］	Fett N, Werth VP. Update on morphea: part I. Epidemiology, clinical presentation, and pathogenesis［J］. J Am Acad Dermatol, 2011,64（2）:217⁃230. doi: 10.1016/j.jaad.2010.05.045.
［2］	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.
［3］	Leitenberger JJ, Cayce RL, Haley RW, et al. Distinct autoimmune syndromes in morphea: a review of 245 adult and pediatric cases［J］. Arch Dermatol, 2009,145（5）:545⁃550. doi: 10.1001/archdermatol.2009.79.
［4］	Pequet MS, Holland KE, Zhao S, et al. Risk factors for morphoea disease severity: a retrospective review of 114 paediatric patients［J］. Br J Dermatol, 2014,170（4）:895⁃900. doi: 10.1111/bjd. 12758.
［5］	Kreuter A, Krieg T, Worm M, et al. German guidelines for the diagnosis and therapy of localized scleroderma［J］. J Dtsch Dermatol Ges, 2016,14（2）:199⁃216. doi: 10.1111/ddg.12724.
［6］	El⁃Kehdy J, Abbas O, Rubeiz N. A review of Parry⁃Romberg syndrome［J］. J Am Acad Dermatol, 2012,67（4）:769⁃784. doi: 10.1016/j.jaad.2012.01.019.
［7］	Careta MF, Leite Cda C, Cresta F, et al. Prospective study to evaluate the clinical and radiological outcome of patients with scleroderma of the face［J］. Autoimmun Rev, 2013,12（11）:1064⁃1069. doi: 10.1016/j.autrev.2013.05.005.
［8］	Berianu F, Cohen MD, Abril A, et al. Eosinophilic fasciitis: clinical characteristics and response to methotrexate［J］. Int J Rheum Dis, 2015,18（1）:91⁃98. doi: 10.1111/1756⁃185X.12499.
［9］	Schanz S, Fierlbeck G, Ulmer A, et al. Localized scleroderma: MR findings and clinical features［J］. Radiology, 2011,260（3）:817⁃824. doi: 10.1148/radiol.11102136.
［10］	Schanz S, Henes J, Ulmer A, et al. Response evaluation of musculoskeletal involvement in patients with deep morphea treated with methotrexate and prednisolone: a combined MRI and clinical approach［J］. AJR Am J Roentgenol, 2013,200（4）:W376⁃W382. doi: 10.2214/AJR.12.9335.
［11］	李明, 孙建方. 皮肤科结缔组织病诊治［M］. 北京: 北京大学医学出版社, 2017:295⁃297.
［12］	Lebeaux D, Francès C, Barete S, et al. Eosinophilic fasciitis （Shulman disease）: new insights into the therapeutic management from a series of 34 patients［J］. Rheumatology （Oxford）, 2012,51（3）:557⁃561. doi: 10.1093/rheumatology/ker366.
［13］	Wright NA, Mazori DR, Patel M, et al. Epidemiology and treatment of eosinophilic fasciitis: an analysis of 63 patients from 3 tertiary care centers［J］. JAMA Dermatol, 2016,152（1）:97⁃99. doi: 10.1001/jamadermatol.2015.3648.
［14］	Itin PH, Schiller P. Double⁃lined frontoparietal scleroderma en coup de sabre［J］. Dermatology, 1999,199（2）:185⁃186. doi: 10. 1159/000018236.
［15］	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.
［16］	Arkachaisri T, Vilaiyuk S, Li S, et al. The localized scleroderma skin severity index and physician global assessment of disease activity: a work in progress toward development of localized scleroderma outcome measures［J］. J Rheumatol, 2009,36（12）:2819⁃2829. doi: 10.3899/jrheum.081284.
［17］	Arkachaisri T, Vilaiyuk S, Torok KS, et al. Development and initial validation of the localized scleroderma skin damage index and physician global assessment of disease damage: a proof⁃of⁃concept study［J］. Rheumatology （Oxford）, 2010,49（2）:373⁃381. doi: 10.1093/rheumatology/kep361.
［18］	Torok KS, Kurzinski K, Kelsey C, et al. Peripheral blood cytokine and chemokine profiles in juvenile localized scleroderma: T⁃helper cell⁃associated cytokine profiles［J］. Semin Arthritis Rheum, 2015,45（3）:284⁃293. doi: 10.1016/j.semarthrit.2015.06. 006.
［19］	Mertens JS, Seyger M, Thurlings RM, et al. Morphea and eosinophilic fasciitis: an update［J］. Am J Clin Dermatol, 2017,18（4）:491⁃512. doi: 10.1007/s40257⁃017⁃0269⁃x.
［20］	Dańczak⁃Pazdrowska A, Kowalczyk M, Szramka⁃Pawlak B, et al. Interleukin⁃17A and interleukin⁃23 in morphea［J］. Arch Med Sci, 2012,8（6）:1089⁃1095. doi: 10.5114/aoms.2012.32421.
［21］	Arkachaisri T, Fertig N, Pino S, et al. Serum autoantibodies and their clinical associations in patients with childhood⁃ and adult⁃onset linear scleroderma. A single⁃center study［J］. J Rheumatol, 2008,35（12）:2439⁃2444. doi: 10.3899/jrheum.080098.
［22］	Yamane K, Ihn H, Kubo M, et al. Anti⁃U1RNP antibodies in patients with localized scieroderma［J］. Arch Dermatol Res, 2001,293（9）:455⁃459. doi: 10.1007/s004030100254.
［23］	Yimane K, Ihn H, Kubo M, et al. Anti⁃U3 snRNP antibodies in localised scleroderma［J］. Ann Rheum Dis, 2001,60（12）:1157⁃1158. doi: 10.1136/ard.60.12.1157.
［24］	Dupont S, Catala M, Hasboun D, et al. Progressive facial hemiatrophy and epilepsy: a common underlying dysgenetic mechanism［J］. Neurology, 1997,48（4）:1013⁃1018. doi: 10.1212/ wnl.48.4. 1013.
［25］	El⁃Kehdy J, Abbas O, Rubeiz N. A review of Parry⁃Romberg syndrome［J］. J Am Acad Dermatol, 2012,67（4）:769⁃784. doi: 10.1016/j.jaad.2012.01.019.
［26］	Asher SW, Berg BO. Progressive hemifacial atrophy: report of three cases, including one observed over 43 years, and computed tomographic findings［J］. Arch Neurol, 1982,39（1）:44⁃46. doi: 10.1001/archneur.1982.00510130046011.
［27］	Careta MF, Leite Cda C, Cresta F, et al. Prospective study to evaluate the clinical and radiological outcome of patients with scleroderma of the face［J］. Autoimmun Rev, 2013,12（11）:1064⁃1069. doi: 10.1016/j.autrev.2013.05.005.
［28］	Moko SB, Mistry Y, Blandin de Chalain TM. Parry⁃Romberg syndrome: intracranial MRI appearances［J］. J Craniomaxillofac Surg, 2003,31（5）:321⁃324. doi: 10.1016/s1010⁃5182（03）00028⁃3.
［29］	Terstegge K, Kunath B, Felber S, et al. MR of brain involvement in progressive facial hemiatrophy （Romberg disease）: reconsideration of a syndrome［J］. AJNR Am J Neuroradiol, 1994,15（1）:145⁃150.

线状硬斑病107例临床及影像学特点回顾性分析

Clinical and imaging characteristics of 107 patients with linear morphea: a retrospective analysis

PDF

可视化

摘要/Abstract

引用本文

使用本文

参考文献 29

相关文章 1

Metrics

本文评价

推荐阅读 10