硬斑病患者180例临床特征及分类探讨

Clinical features and classification of 180 patients with morphea

目的分析硬斑病患者的临床特征及分类。方法收集2010年1月至2021年7月就诊于复旦大学附属中山医院的180例硬斑病患者流行病学资料、临床表现和实验室数据进行回顾性分析。采用两独立样本t检验比较性别间发病年龄差异, 卡方检验比较不同性别、亚型间临床特征差异。结果 180例患者中, 女性123例, 男性57例, 男女比为1∶2.16。硬斑病发病年龄女性为(28.69 ± 17.97)岁, 男性为(29.90 ± 20.67)岁。其中, 带状硬斑病最为常见(68例, 37.78%), 其次是斑块状硬斑病(63例, 35.00%)、混合型硬斑病(28例, 15.56%)和深部硬斑病(21例, 11.67%)。各年龄段均可发病, 不同临床亚型平均发病年龄差异有统计学意义(F = 5.95, P < 0.001)。男女患者平均发病年龄、亚型比例方面差异均无统计学意义(F = 0.15, P = 0.696;χ^(2) = 2.88, P = 0.410)。Pasini和Pierini皮肤萎缩症(PP萎缩)在本组患者中常见(62例, 34.44%), 表现为斑片状或带状分布, 45例斑片状PP萎缩中26例及17例带状PP萎缩中11例同时合并其他亚型硬斑病。75例患者检测自身抗体, 阳性34例(45.33%), 女性患者自身抗体种类多, 男性则较为单一, 主要为抗核抗体、抗SSA及抗SSB抗体。女性患者合并症种类较多, 但硬化萎缩性苔藓和白癜风是男女患者中最常见的合并症。结论 PP萎缩是硬斑病一个常见的临床亚型, 常合并其他亚型, 可能是中国人硬斑病发病的一个特点。斑块状硬斑病、带状硬斑病、深部硬斑病和混合型硬斑病作为硬斑病大类的分类方法比较合理。

Objective To determine classification and clinical features of morphea.Methods A retrospective analysis was conducted on epidemiological information about clinical manifestations of and laboratory data from 180 patients with morphea,who visited Zhongshan Hospital,Fudan University from January 2010 to July 2021.Two-independent-sample t test was used to compare the age at onset between genders,and chi-square test to analyze differences in clinical characteristics between different genders and subtypes.Results Among the 180 patients,123 were females and 57 were males,with a male-to-female ratio of 1∶2.16.The age at onset of morphea was 28.69±17.97 years for female patients,and 29.90±20.67 years for male patients.Among them,linear morphea was the most common type in this study(68 cases,37.78%),followed by plaque morphea(63 cases,35.00%),mixed morphea(28 cases,15.56%)and deep morphea(21 cases,11.67%).The disease occurred in all age groups,but the age at onset significantly varied among different clinical subtypes(F=5.95,P<0.001).No significant difference was observed in the age at onset or proportion of clinical subtypes between genders(F=0.15,P=0.696;χ^(2)=2.88,P=0.410).Atrophoderma of Pasini and Pierini(APP)was very common(62 cases,34.44%)in the 180 patients,which mainly manifested as plaques or linear lesions,and 26 out of 45 patients with plaque APP and 11 out of 17 with linear APP were both accompanied by other subtypes of morphea.Among the 75 patients tested for autoantibody profiles,34(45.33%)presented with positive results.More diverse types of autoantibodies were found in female patients compared with male patients,and antinuclear antibodies,anti-SSA and anti-SSB antibodies were the most common types.There were various types of comorbidities in female patients,but lichen sclerosus et atrophicus and vitiligo were the most common comorbidities in both genders.Conclusion High incidence and frequent co-occurrence with other subtypes of APP may be the characteristics of Chinese patients with morphea,and it is recommended to classify morphea into plaque,linear,deep and mixed subtypes.