lichen sclerosus

硬化性苔藓
  • 文章类型: Journal Article
    以前的观察性研究发现硬化苔藓(LS)与代谢状态有关,如糖尿病(DM)和体重指数(BMI)。然而,也有一些研究表明LS与DM和BMI无关.观察结果背后的机制尚不清楚。因此,这种关系的因果关系仍然未知。在这项研究中,进行了双向双样本孟德尔随机化(MR)来研究DM,BMI,和LS。
    与DM(包括1型和2型糖尿病)相关的工具变量,和BMI从全基因组关联研究(GWAS)和GWAS荟萃分析中确定。LS的GWAS数据来自2022年发布的第八版FinnGen生物数据库。反向方差加权(IVW),加权中位数,和MR-Egger方法用于进行双向双样本MR分析。此后,研究了异质性和水平多效性,以确定结果是否受单核苷酸多态性(SNP)的影响.
    我们发现缺乏证据证明DM的因果关系,和BMI在LS上的逆方差加权(1型糖尿病,OR=0.97,95%CI=0.91-1.04,p=0.429;2型糖尿病,OR=0.91,95%CI=0.82-1.00,p=0.0511;BMI,OR=0.92,95%CI=0.73-1.15,p=0.4554)。在另一个方向,结果还显示,LS对DM和BMI无显著因果效应.
    这项MR分析表明,DM和BMI与LS在两个方向上都没有显着的因果关系,这与之前报告的正相关的观察性研究相矛盾。潜在的混杂因素可能有助于先前观察到的关联,需要进一步的研究。
    UNASSIGNED: Previous observational studies have found that lichen sclerosus (LS) is associated with metabolic statuses, such as diabetes mellitus (DM) and body mass index (BMI). However, there are also some studies showing that LS is not related to DM and BMI. The mechanism behind observational results is still unclear. Therefore, the causality of this relationship remains unknown. In this study, a bidirectional two-sample Mendelian randomization (MR) was conducted to investigate the correlation between DM, BMI, and LS.
    UNASSIGNED: The instrumental variables related to DM (including type 1 and type 2 diabetes), and BMI were identified from genome-wide association studies (GWAS) and a GWAS meta-analysis. The GWAS data for LS was from obtained the eighth edition of the FinnGen biological database released in 2022. Inverse variance weighted (IVW), weighted median, and MR-Egger methods were used to conduct a bidirectional two-sample MR analysis. Thereafter, the heterogeneity and horizontal pleiotropy were examined to determine whether the results were affected by a single-nucleotide polymorphism (SNP).
    UNASSIGNED: We found a lack of evidence for the causal association of DM, and BMI on LS in inverse variance weighted (type 1 diabetes, OR=0.97, 95% CI=0.91-1.04, p=0.429; type 2 diabetes, OR=0.91, 95% CI=0.82-1.00, p=0.0511; BMI, OR=0.92, 95% CI=0.73-1.15, p=0.4554). In the other direction, the results also showed that LS had no significant causal effect on DM and BMI.
    UNASSIGNED: This MR analysis demonstrated no significant causal relationship between DM and BMI with LS in both directions, which contradicts previous observational studies reporting a positive association. Potential confounding factors may contribute to previously observed associations, and further research is necessary.
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  • 文章类型: Journal Article
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  • 文章类型: Case Reports
    特纳综合征(TS)是一种罕见的临床病症,与完全或部分缺失相关,或者X染色体的结构异常,主要表现为身材矮小和骨骼异常,女性促性腺激素性性腺功能减退和不孕症。皮肤经常参与TS,特别是自身免疫性疾病,如白癜风和硬化性苔藓(LS)。这里,我们介绍了一个10岁的中国女孩,患有外阴LS(VLS)和外阴LS,多年来被误诊为湿疹和白癜风。为了充分控制LS并减轻父母对局部皮质类固醇潜在副作用的担忧,给她开了他克莫司软膏治疗外皮病变,和光动力疗法(PDT)用于外阴病变。对于PDT方案,我们使用5-氨基乙酰丙酸(ALA)作为光敏剂,以633nm的红光以60mW/cm2照射病变面积,每次30分钟。每隔2周治疗6次后,瘙痒和病变严重程度均获得了令人满意的缓解。到目前为止,TS指南没有将LS作为一种常见的合并症来引起注意.然而,准确的诊断和有效的治疗是必不可少的LS,以避免发展唇萎缩的可能性,附着力,甚至外阴癌.根据我们的研究,PDT能明显缓解主观症状,具有良好耐受性的VLS的客观病变严重程度和组织病理学变化,因此也可以是TS患者中这种合并症的安全有效的治疗选择。
    Turner syndrome (TS) is a rare clinical condition associated with a completely or partially absence, or structural abnormality of an X chromosome, mainly representing as short stature and skeletal anomalies, female hypergonadotropic hypogonadism and infertility. Skin is frequently involved in TS, especially autoimmune diseases like vitiligo and lichen sclerosus (LS). Here, we present a 10-year-old Chinese girl with TS combined with both vulvar LS (VLS) and extragenital LS, who had been misdiagnosed as eczema and vitiligo for years. In order to control LS sufficiently and allay the parents\' concerns of potential side effects of topical corticosteroids, she was prescribed with tacrolimus ointment on the extragenital lesions, and photodynamic therapy (PDT) for vulvar lesions. For PDT regimen, we used 5-aminolevulinic acid (ALA) as photosensitizer and 633 nm red light to irradiate the lesion area at 60 mW / cm2 for 30 min each time. After 6 times of treatment at 2-week intervals, a satisfactory remission of both pruritus and lesion severity was achieved. So far, the guideline on TS did not include LS as a common comorbidity to raise attention. However, accurate diagnosis and effective treatment are essential for LS to avoid the possibilities of developing labial atrophy, adhesion, or even vulvar cancer. Based on our research, PDT can significantly relieve subjective symptoms, objective lesion severity and histopathological changes of VLS with good tolerance, and therefore can also be a safe and effective therapeutic alternative in such comorbidity in TS patients.
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  • 文章类型: Journal Article
    外阴硬化性苔藓(VLS)是一种发病机制不明的慢性炎症性皮肤病,以女孩外阴和肛门区域周围的瓷白色萎缩性斑块为特征。通过这种交流,我们对16名临床和组织学证实为VLS的女性女孩进行了研究,描述了反射共聚焦显微镜(RCM)中病变的主要识别特征,并阐明了RCM发现与组织学之间的对应关系。我们推荐RCM,一种非侵入性技术,作为VLS的补充诊断工具。
    Vulvar lichen sclerosus (VLS) is a chronic inflammatory dermatosis of unknown pathogenesis, characterized by porcelain-white atrophic plaques around the vulvar and anal areas in girls. With this communication, we performed the study on 16 female girls with clinically and histologically confirmed VLS, described the main identifying characteristics of the lesions in reflectance confocal microscopy (RCM) and elucidated the corresponding relationship between RCM findings and histology. We recommend RCM, a noninvasive technique, as a complementary diagnostic tool for VLS.
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  • 文章类型: Journal Article
    本文介绍了一种基于多光子显微镜(MPM)检查的方法,该方法由双光子激发荧光(TPEF)和二次谐波产生(SHG)组成。通过MPM从未染色的病变组织获取亚细胞分辨率图像,然后进行光谱分析以量化TPEF和SHG信号。此外,使用U-Net对TPEF图像中的弹性纤维进行分割,以与SHG图像中的胶原纤维结合以进行精确的纤维定量。分割预测在几个评价指标上表现优异。mIoU,mPA,F1评分分别达到0.8516、0.9281和0.941。定量分析表明,与硬化性苔藓病例相比,硬体中胶原纤维的增加。同时,根据MPM成像描绘了硬化性苔藓真皮中弹性纤维的大量减少。因此,MPM与组织病理学检查相当,我们的实验结果准确区分了硬皮和苔藓硬化。
    This paper describes a methodology to differentiate morphea from lichen sclerosus based on examination with multiphoton microscopy (MPM) composed of two-photon excited fluorescence (TPEF) and second harmonic generation (SHG). Subcellular-resolution images were acquired by MPM from unstained lesion tissues then process spectral analysis to quantify the TPEF and SHG signals. Moreover, U-Net was employed to segment elastic fiber in TPEF images to combine with collagen fiber in SHG images for precise fiber quantification. Predictions of segmentation showed excellent performance on several evaluation indicators. The mIoU, mPA, and F1 score reach 0.8516, 0.9281, and 0.941. The quantitative analysis demonstrated the increase of collagen fibers in morphea compared to that in lichen sclerosus cases. Meanwhile, the great diminution of elastic fiber in the dermis of lichen sclerosus was depicted based on MPM imaging. Thus, MPM was comparable to the histopathological examination and our experimental results accurately distinguish between morphea and lichen sclerosus.
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  • 文章类型: Journal Article
    硬化性苔藓(LS)是一种慢性炎症性皮肤病,主要发生在肛门生殖器区域并引起瘙痒,酸痛,萎缩和疤痕,这可能会导致女性阴蒂掩埋,男性包茎。光动力疗法(PDT)在过去几年中已被建议作为一种替代的非侵入性治疗LS,但目前尚无荟萃分析来评估其疗效和安全性.
    评估5-氨基乙酰丙酸光动力疗法(ALA-PDT)治疗LS的疗效和安全性。
    我们使用Cochrane协作法和PRISMA指南进行了荟萃分析。在PubMed进行了系统的文献检索,EMBASE,科克伦图书馆,万方数据,截至2020年6月30日的CBM和CNKI。随机对照试验(RCTs)与ALA-PDT进行了比较,用于治疗LS的皮质类固醇或他克莫司软膏。根据Cochrane手册对每个试验的偏倚风险进行评级。使用具有95%置信区间(CI)的风险比(RR)来表达比较结果。
    我们包括4个RCT,共有184名参与者。荟萃分析显示ALA-PDT治疗LS优于外用软膏(总有效率:RR1.38[95%CI1.19-1.60])。
    当前有限的证据支持ALA-PDT治疗LS的有效性和安全性。不良反应包括疼痛、肿胀,随着治疗的持续进行,发红和去角质会减少。必然需要进一步的大样本的高质量RCT。
    UNASSIGNED: Lichen sclerosus (LS) is a chronic inflammatory dermatosis that occurs mainly in the anogenital area and causes itching, soreness, atrophy and scarring, which may result in burying of the clitoris in females and phimosis in males. Photodynamic therapy (PDT) has been suggested during the past years as an alternative non-invasive treatment for LS, but there is still no meta-analysis to evaluate its efficacy and safety.
    UNASSIGNED: To assess the efficacy and safety of 5-aminolevulinic acid photodynamic therapy (ALA-PDT) for treatment of LS.
    UNASSIGNED: We undertook a meta-analysis using the methodology of the Cochrane Collaboration and the guideline of PRISMA. A systematic literature search was carried out in PubMed, EMBASE, The Cochrane Library, WanFang Data, CBM and CNKI up to 30 June 2020. Randomized controlled trials (RCTs) were compared with ALA-PDT, corticosteroids or tacrolimus ointments for treating LS. The risk of bias for each trial was rated according to the Cochrane Handbook. Risk ratios (RR) with 95% confidence intervals (CI) were utilized to express the comparative outcomes.
    UNASSIGNED: We included 4 RCTs with a total of 184 participants. The meta-analysis showed ALA-PDT was better than topical ointments in treating LS (total effective rate: RR 1.38 [95% CI 1.19-1.60]).
    UNASSIGNED: The current limited evidence supports the efficacy and safety of ALA-PDT in treating LS. The adverse reactions included pain, swelling, redness and exfoliation which would decrease with the continuing sessions of treatment. Further high-qualified RCTs of large samples are necessarily needed.
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  • 文章类型: Journal Article
    比较外用糖皮质激素与CO2点阵激光治疗,探讨不同病理类型非肿瘤性外阴上皮病变治疗的疗效差异,为这些疾病的治疗提供科学依据。本文旨在研究不同病理类型的非肿瘤性外阴上皮病变的疗效差异,为此类疾病的治疗提供科学依据。
    2016年11月至2018年7月,我院外阴活检证实178例慢性外阴单纯性苔藓(LSC)或硬化性苔藓。最后,160名患者被纳入本试验。将患者分为2组:一组用局部激素治疗,一组用CO2晶格激光治疗。每组80例,包括40例LSC和40例硬化性苔藓。患者早晚在患处涂抹1克孕酮乳膏和倍他米松乳膏,分别,一天一次,三个月。采用患者总体变化印象(PGI-C)主观症状改善量表和临床疗效评价量表进行疗效评价。应用该公式计算疗效指数。
    治疗1、3和6个月时的PGI-C评分显示,激光治疗组的疗效明显优于糖皮质激素治疗组。3个月和6个月的临床疗效评分量表显示,激光治疗的疗效明显高于糖皮质激素治疗(分别为P=0.006和P=0.002)。在糖皮质激素组中,治疗1个月和3个月后,不同病理亚型的临床疗效差异显着。LSC的治疗效果优于硬化性苔藓。经过3个月和6个月的治疗,LSC的临床效果优于硬化性苔藓(3个月:95%vs.75%;6个月:和95%vs.70%)。
    超脉冲CO2点阵激光治疗外阴上皮非肿瘤样病变疗效优于糖皮质激素治疗。
    UNASSIGNED: A comparison of topical glucocorticoids with CO2 fractional laser treatment was conducted to investigate the differences in the efficacy of non-neoplastic vulvar epithelial lesion treatments in different pathological types and to provide a scientific basis for the management of these disorders. This paper was to study the difference of curative effect of different pathological types of non-tumor vulvar epithelial lesions and provide scientific basis for the treatment of these diseases.
    UNASSIGNED: From November 2016 to July 2018, 178 cases of vulvar lichen simplex chronicus (LSC) or lichen sclerosus were confirmed with vulvar biopsy at our institute. Finally, 160 patients were enrolled in this trial. The patients were divided into 2 groups: a group treated with topical hormone and a group treated with CO2 lattice laser therapies. There were 80 cases in each group, including 40 with LSC and 40 with lichen sclerosus. Patients applied 1 gram of progesterone cream and betamethasone cream to the affected area in the morning and evening, respectively, once a day for 3 months. The efficacy was evaluated with the Patient Global Impression of Change (PGI-C) subjective symptom improvement scale and clinical efficacy evaluation scale. The formula was applied to calculate the curative effect index.
    UNASSIGNED: The PGI-C scores at 1, 3, and 6 months of treatment showed that the laser treatment group had remarkably superior outcomes to the glucocorticoid treatment group. The clinical efficacy score scale at 3- and 6-month treatments indicated a significantly greater curative effect in the laser than in the glucocorticoid treatment (P=0.006 and P=0.002 respectively). In the glucocorticoid group, the clinical effects of different pathological subtypes were significantly different following the 1- and 3-month treatments. The efficacy of treatment for LSC was better than that for lichen sclerosus. Following the 3- and 6-month treatments, the clinical effect for LSC was better than that of lichen sclerosus (3 months: 95% vs. 75%; 6 months: and 95% vs. 70%).
    UNASSIGNED: Ultrapulse CO2 lattice laser was more effective than was glucocorticoid therapy in the treatment of vulvar epithelial non-tumor-like lesions.
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  • 文章类型: Journal Article
    长段苔藓硬化(LS)尿道狭窄是泌尿科医师面临的挑战。有限的数据可供外科医生在Kulkarni和Asopa尿道成形术之间做出手术决定。在这项回顾性研究中,我们调查了这两种手术在LS型尿道狭窄患者中的结局.2015年1月至2020年12月,77例LS尿道狭窄患者在泌尿外科接受了Kulkarni和Asopa尿道成形术,上海市第九人民医院,上海交通大学医学院(上海,中国)。在77名患者中,42人(54.5%)接受了Asopa手术,35人(45.5%)接受了Kulkarni手术。Kulkarni组的总并发症发生率为34.2%,Asopa组为19.0%,差异无统计学意义(P=0.105)。在并发症中,除术后鼻道狭窄(P=0.020)外,尿道狭窄复发(P=0.724)和龟裂(P=0.246)的发生率无统计学差异。然而,两组无复发生存率差异有统计学意义(P=0.016).Cox生存分析显示使用抗血小板/抗凝治疗(P=0.020),糖尿病(P=0.003),当前/以前吸烟(P=0.019),冠心病(P<0.001),狭窄长度(P=0.028)可能导致更高的并发症风险比。即便如此,这两种技术在LS型尿道狭窄的手术治疗中仍能提供可接受的结果,具有各自的优势。应根据患者特征和外科医生的喜好综合考虑手术选择。此外,我们的结果表明,使用抗血小板/抗凝治疗,糖尿病,冠心病,当前/以前吸烟,和狭窄长度可能是并发症的促成因素。因此,我们建议LS患者进行早期干预,以获得更好的治疗效果.
    Long-segment lichen sclerosus (LS) urethral stricture is a challenge for urologists. Limited data are available for surgeons to make a surgical decision between Kulkarni and Asopa urethroplasty. In this retrospective study, we investigated the outcomes of these two procedures in patients with LS urethral stricture. Between January 2015 and December 2020, 77 patients with LS urethral stricture underwent Kulkarni and Asopa procedures for urethroplasty in the Department of Urology, Shanghai Ninth People\'s Hospital, Shanghai Jiao Tong University School of Medicine (Shanghai, China). Of the 77 patients, 42 (54.5%) underwent the Asopa procedure and 35 (45.5%) underwent the Kulkarni procedure. The overall complication rate was 34.2% in the Kulkarni group and 19.0% in the Asopa group, and no difference was observed ( P = 0.105). Among the complications, no statistical difference was observed in the incidence of urethral stricture recurrence ( P = 0.724) or glans dehiscence ( P = 0.246) except for postoperative meatus stenosis ( P = 0.020). However, the recurrence-free survival rate between the two procedures was significantly different ( P = 0.016). Cox survival analysis showed that antiplatelet/anticoagulant therapy use ( P = 0.020), diabetes ( P = 0.003), current/former smoking ( P = 0.019), coronary heart disease ( P < 0.001), and stricture length ( P = 0.028) may lead to a higher hazard ratio of complications. Even so, these two techniques can still provide acceptable results with their own advantages in the surgical treatment of LS urethral strictures. The surgical alternative should be considered comprehensively according to the patient characteristics and surgeon preferences. Moreover, our results showed that antiplatelet/anticoagulant therapy use, diabetes, coronary heart disease, current/former smoking, and stricture length may be contributing factors of complications. Therefore, patients with LS are advised to undergo early interventions for better therapeutic effects.
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  • 文章类型: Journal Article
    背景:女孩的外阴硬化性苔藓(VLS)表现为瘙痒,排尿困难,和便秘,并可能导致外阴结构的丧失。在临床表现模糊的患者中,反射共聚焦显微镜(RCM)可能是一种有用的非侵入性诊断工具。本研究的目的是描述VLS的RCM特征,并探讨RCM在治疗监测中的临床应用价值。
    方法:本研究纳入了16例VLS患者。所有患者均定期用RCM进行临床评估,并根据患者的临床表现和RCM特征给予不同的治疗方案。
    结果:RCM可以观察到VLS的一些主要关键诊断特征,包括圆形到椭圆形的囊肿样结构,具有中低折射的角质物质(75%),表皮厚度变薄(100%),真皮乳头周围环状结构的破坏(100%),无序分布的粗中折射纤维材料(100%),多边形,丰满,高折射细胞结构和线性低折射小管结构(100%)。所有这些特征与组织病理学特征具有高度的一致性。根据临床表现和RCM特征,采用个体化治疗方案后,临床表现有所改善。
    结论:RCM允许可视化VLS的主要关键诊断特征,并代表客观治疗监测的有效选择。
    BACKGROUND: Vulvar lichen sclerosus (VLS) in girls presents with itching, dysuria, and constipation and may result in the loss of vulvar architecture. In patients with an ambiguous clinical presentation, reflectance confocal microscopy (RCM) could be a helpful noninvasive diagnostic tool. The aim of this study was to describe the RCM characteristics of VLS and explore the clinical application value of RCM in therapeutic monitoring.
    METHODS: Sixteen patients with VLS were included in the study. All patients were periodically evaluated clinically with RCM, and different treatment regimens were given based on the patient\'s clinical appearances and RCM features.
    RESULTS: Some major key diagnostic features of VLS can be observed by RCM, including round to oval cyst-like structures with medium-to-low-refractive keratinoid substances (75%), thinning of the epidermal thickness (100%), destruction of the ring-like structures around dermal papillae (100%), disorderly distributed coarse medium-refractive fibrous material (100%),polygonal, plump, high-refractive cellular structures and linear low-refractive canalicular structures (100%). All of these characteristics had a high correspondence with histopathological features. The clinical manifestations improved after individualized treatment regimens based on the clinical appearances and RCM features.
    CONCLUSIONS: RCM allows the visualization of major key diagnostic features of VLS and represents a valid option for objective therapeutic monitoring.
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  • 文章类型: Journal Article
    背景:硬化外阴苔藓(VLS)是一种慢性炎症性疾病,最初涉及肛门生殖器区域。无创评估对于VLS的精确管理至关重要。我们旨在分析高频超声(HFUS)特征并将HFUS与组织病理学变化相关联。
    方法:从2020年8月至2021年9月,对40例经组织病理学证实的VLS病变患者进行回顾性分析。临床表现,评估了皮肤镜图像以及20和50MHzHFUS图像.HFUS评估包括表皮形态,低回声真皮带厚度,和低回声真皮带内部回声。我们将HFUS图像与组织病理学进行了比较,Pearson相关系数用于评估低回声真皮带厚度与组织病理学深度之间的关系。
    结果:100%(40/40)VLS病变中存在低回声真皮带。组织病理学深度与相应的低回声真皮带厚度之间存在显著的线性正相关,皮尔逊相关系数为0.685(p<0.001)。此外,95%(38/40)的病变显示光滑的表皮,低回声真皮带的内部回声在60%(24/40)病变中评估为均匀,在40%(16/40)病变中评估为不均匀。
    结论:HFUS特征,以及可测量的低回声真皮带厚度,可以为VLS的精确诊断和治疗监测提供有价值的信息。
    BACKGROUND: Vulvar lichen sclerosus (VLS) is a chronic inflammatory disease initially involving anogenital areas. Noninvasive assessment is essential for precise management in VLS. We aim to analyze high-frequency ultrasound (HFUS) features and correlate HFUS with histopathological changes.
    METHODS: Forty patients with histopathologically confirmed VLS lesions were retrospectively identified from August 2020 to September 2021. The clinical manifestations, dermoscopic images as well as both 20 and 50 MHz HFUS images were assessed. HFUS assessment included epidermal morphology, hypoechoic dermal band thickness, and hypoechoic dermal band internal echo. We compared HFUS images with histopathology, and Pearson\'s correlation coefficient was used to assess the relationship between hypoechoic dermal band thickness and histopathological depth.
    RESULTS: Hypoechoic dermal band was present in 100% (40/40) VLS lesions. There was a significant linear positive correlation between the histopathological depth and corresponding hypoechoic dermal band thickness, with a Pearson correlation coefficient of 0.685 (p < 0.001). Besides, 95% (38/40) lesions revealed smooth epidermis, and the internal echo of hypoechoic dermal band was assessed as homogeneous in 60% (24/40) and inhomogeneous in 40% (16/40) lesions.
    CONCLUSIONS: HFUS characteristics, as well as measurable hypoechoic dermal band thickness, may provide valuable information in the precise diagnosis and the treatment monitoring of VLS.
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