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

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。

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.