lichen sclerosus

硬化性苔藓
  • 文章类型: Case Reports
    脓包是指脓液在阴道腔内积聚。先前尚未描述过苔藓硬化和绝经后出血(PMB)背景下的Pyocolpos。一名69岁的第3段患者,有PMB病史,并有长期的苔藓硬化病史。由于阴道粘连,无法进行阴道检查。外貌显示阴蒂结构的丧失。进一步成像显示子宫内膜厚度为4-5毫米,后外宫颈局灶性异常与后穹窿扩张的出血性囊性病变相容,和骨盆内的一些自由液体。由于阴道完全消失,因此放弃了宫腔镜检查。经过多学科评估,患者进行了全腹部子宫切除术,在进入金库的开口处注意到了一个pyocoppos的存在。我们以前没有发现任何与硬化苔藓相关的脓疱病病例报告。硬化苔藓的长期病史可能导致流出道阻塞,其次被感染并缓慢发展为pyocoppos的形成。如果在术前诊断出pyocoppos,则可以探索其他管理选择。对于长期患有硬化性苔藓病史且在影像学上表现出腹痛和盆腔肿块的患者,应考虑使用Pyocoppos。
    Pyocolpos refers to the buildup of pus within the vaginal cavity. Pyocolpos in the background of lichen sclerosis and postmenopausal bleeding (PMB) has not been previously described. A 69-year-old para 3 patient presented with a history of PMB with a long-standing history of lichen sclerosis. The vaginal examination was impossible due to vaginal adhesions. Vulval appearances revealed the loss of the clitoral architecture. Further imaging revealed an endometrial thickness of 4-5 mm, a focal abnormality within the posterior ectocervix compatible with a hemorrhagic cystic lesion distending the posterior fornix, and some free fluid within the pelvis. A hysteroscopy was abandoned as the vagina was completely obliterated. After a multidisciplinary assessment, the patient had a total abdominal hysterectomy, and the presence of a pyocolpos was noticed at the opening into the vault. We could not find any previous case reports of pyocolpos that are associated with lichen sclerosus. The long-standing history of lichen sclerosus may have caused an obstruction of the outflow tract, which was secondarily infected and slowly progressed into the formation of pyocolpos. Other management options could have been explored if the diagnosis of pyocolpos had been made preoperatively. Pyocolpos should be considered in patients with a history of a long-standing lichen sclerosus who present with abdominal pain and a pelvic mass on imaging.
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  • 文章类型: Journal Article
    背景:外阴狭窄是一种损害性功能的衰弱状况,排尿,以及接受妇科检查的能力。本研究的目的是描述毛细血管穿支会阴皮瓣(CPPF)矫正外阴狭窄的技术。
    方法:我们回顾性研究了通过手术分离和CPPF重建治疗的外阴狭窄患者。该程序涉及外阴分离并产生随后的缺陷,用皮瓣修复,横向收获到大阴唇,包括毛细血管穿孔器,并通过皮下隧道转移以修复外阴缺损。用布拉德福德量表评估功能结果,使用学生t检验比较术前和术后评分。
    结果:共纳入13例患者,三例外阴癌治疗后狭窄,十例硬化苔藓。总的来说,我们分析了29个皮瓣,平均尺寸为15.6cm2。我们总是在皮瓣中只包括一个穿孔器,没有术后并发症。所有患者的狭窄都得到了解决,手术后一年没有复发.术前狭窄的平均严重程度为2.3±0.6,干预后降低至0.3±0.4,表明显著改善(p<0.01)。
    结论:CPPF已被证明是一种快速,安全的外阴狭窄重建方法。
    BACKGROUND: Vulvar stenosis is a debilitating condition that compromises sexual function, urination, and the ability to undergo gynecological examinations. The purpose of this study is to describe the technique of capillary perforator perineal flaps (CPPF) for the correction of vulvar stenosis.
    METHODS: We retrospectively examined patients with vulvar stenosis treated through surgical separation and reconstruction with CPPF. The procedure involved vulvar separation with the creation of a subsequent defect, repaired using a flap, harvested laterally to the labia majora including a capillary perforator and transferred through a subcutaneous tunnel to repair the vulvar defect. The functional outcome was evaluated with the Bradford scale, comparing the preoperative and postoperative scores using the Student\'s t-test.
    RESULTS: thirteen patients were included, three with stenosis following treatment for vulvar cancer and ten due to lichen sclerosus. In total, we analyzed 29 flaps, with an average size of 15.6 cm2. We always included just one perforator in the flap and no postoperative complications. Stenosis was resolved in all patients, with no recurrences one year after the surgery. The preoperative average severity of the stenosis was 2.3 + 0.6, reducing to 0.3 + 0.4 post-intervention, indicating a significant improvement (p < 0.01).
    CONCLUSIONS: CPPF has proven to be a quick and safe method for the reconstruction of vulvar stenosis.
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  • 文章类型: Journal Article
    背景:关于硬化性苔藓(LS)的发病率和合并症的数据,基于经过验证的全国人口登记册,仍然稀缺。目的:探讨瑞典LS合并症的发生率和相关性,强调其与恶性肿瘤和自身免疫性疾病的潜在联系。方法:从2001年1月1日至2021年1月1日,使用国家患者登记册进行了一项基于人群的回顾性开放队列研究,以确定所有诊断为LS(ICD-10代码L90.0)的个体。该研究包括154,424名LS患者和一个由463,273人组成的性别和年龄相匹配的对照组,以评估各种癌症和癌前疾病的发生率和优势比。结果:瑞典的LS发病率为每年每10万人80.9,女性发病率(114.4)高于男性(47.2)。LS患者外阴癌的比值比增加(OR=8.3;95%CI=7.5-9.0),阴茎癌(OR=8.9;95%CI=7.3-11.0),前列腺癌(OR=1.2;95%CI=1.1-1.2),睾丸癌(OR=1.4;95%CI=1.1-1.7),膀胱癌(OR=1.1;95%CI=1.1-1.2),乳腺癌(OR=1.4;95%CI=1.3-1.4),外阴白斑(OR=253.5;95%CI=221.9-289.6),和阴茎白斑(OR=5.1;95%CI=4.9-5.4)。结论:这项研究强调了LS患者中各种癌症和癌前病变之间的关联显着增加,强调了对有效治疗和勤奋随访的迫切需要。LS与自身免疫性疾病之间的关联进一步需要进行全面的调查以了解潜在的机制和临床管理意义。未来的研究对于证实这些发现和阐明LS在癌症发展中的作用至关重要。
    Background: Data on the incidence and comorbidity of Lichen sclerosus (LS), based on validated nationwide population-based registries, remains scarce. Objective: To explore the incidence and association of comorbidities with LS in Sweden, emphasizing its potential links to malignancies and autoimmune disorders. Methods: A population-based retrospective open cohort study was conducted using the National Patient Register to identify all individuals diagnosed with LS (ICD-10 code L90.0) from 1 January 2001 to 1 January 2021. The study included 154,424 LS patients and a sex and age matched control group of 463,273 individuals to assess the incidence and odds ratios for various cancers and premalignant conditions. Results: The incidence of LS in Sweden was 80.9 per 100,000 person per year, with higher incidence in females (114.4) than in males (47.2). LS patients showed an increased odds ratio for vulvar cancer (OR = 8.3; 95% CI = 7.5-9.0), penile cancer (OR = 8.9; 95% CI = 7.3-11.0), prostate cancer (OR = 1.2; 95% CI = 1.1-1.2), testicular cancer (OR = 1.4; 95% CI = 1.1-1.7), bladder cancer (OR = 1.1; 95% CI = 1.1-1.2), breast cancer (OR = 1.4; 95% CI = 1.3-1.4), leukoplakia of the vulva (OR = 253.5; 95% CI = 221.9-289.6), and leukoplakia of the penis (OR = 5.1; 95% CI = 4.9-5.4). Conclusions: This study underscores the significantly increased association of various cancers and premalignant conditions in LS patients, highlighting the critical need for efficacious treatment and diligent follow-up. The association between LS and autoimmune diseases further necessitates comprehensive investigation to understand the underlying mechanisms and clinical management implications. Future research is essential to confirm these findings and elucidate the role of LS in cancer development.
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  • 文章类型: Journal Article
    硬化外阴苔藓(VLS)是一种慢性进行性,淋巴细胞介导的炎症性疾病,其发病机制复杂且尚未完全阐明。
    在当前的研究中,我们首次研究了从组织学证实的VLS的女性个体获得的病灶皮肤中白介素17(IL-17)和S100A7的表达。
    在我们的研究中,我们使用从具有组织学证实的VLS的女性患者获得的皮肤活检(n=20)和来自健康的年龄和性别匹配的个体(整形外科程序)的皮肤样本(n=10)作为对照。用免疫组织化学方法评估IL-17和S100A7的组织表达。
    与健康对照的正常皮肤相比,在VLS病变皮肤中显示IL-17表达的细胞数量显著更高(p<0.0001)。在VLS病变皮肤中,IL-17在表皮中和上真皮中的炎性浸润物内的细胞中表达。与健康对照的正常皮肤相比,VLS病变皮肤中显示S100A7表达的细胞数量显著更高(p<0.0001)。在VLS病变皮肤中,S100A7由表皮中的基底上角质形成细胞表达。S100A7也由真皮中的炎性浸润物内的细胞表达。
    我们的研究结果可能提示IL-17和S100A7参与了VLS的发病机制。更好地了解这种疾病可能会导致新的发展,有效的治疗策略,例如使用熟知的生物制剂IL-17抑制剂类。
    UNASSIGNED: Vulvar lichen sclerosus (VLS) is a chronic progressive, lymphocyte-mediated inflammatory disease whose pathogenesis is complex and not fully elucidated.
    UNASSIGNED: In the current study we have investigated for the first time the expression of interleukin-17 (IL-17) and S100A7 in lesional skin obtained from female individuals with histologically confirmed VLS.
    UNASSIGNED: In our study we used skin biopsies obtained from female patients with histologically confirmed VLS (n = 20) and skin samples from healthy age- and gender-matched individuals (plastic surgery procedures) (n = 10) serving as controls. The tissue expressions of IL-17 and S100A7 were assessed with an immunohistochemical method.
    UNASSIGNED: The number of cells showing IL-17 expression was significantly higher in VLS lesional skin as compared to normal skin of healthy controls (p < 0.0001). In VLS lesional skin, IL-17 was expressed in the epidermis and by cells within the inflammatory infiltrate in the upper dermis. The number of cells showing S100A7 expression was significantly higher in VLS lesional skin as compared to normal skin of healthy controls (p < 0.0001). In VLS lesional skin, S100A7 was expressed by suprabasal keratinocytes in epidermis. S100A7 was also expressed by cells within the inflammatory infiltrate in the dermis.
    UNASSIGNED: The results of our study may suggest the involvement of IL-17 and S100A7 in the pathogenesis of VLS. The better understanding of this disease may lead to the development of novel, effective therapeutic strategies e.g. using well-known biologics IL-17 inhibitors class.
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  • 文章类型: Journal Article
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  • 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: Journal Article
    在这份简短的信件中,我们对以前发表的病例报告中的描述提供了我们的专家解释.我们认为这个病例描述了一个患有慢性病的病人,治疗不足的男性生殖器硬化性苔藓。如果未选中,在这种情况下,苔藓硬化可以导致永久性的建筑变化和受损的组织,因此可能会导致继发感染,包括细菌,如溶血葡萄球菌。
    In this short letter of correspondence, we provide our specialist interpretation of what has been described in a previously published case report. We argue that this case describes a patient with chronic, undertreated male genital lichen sclerosus. If left unchecked, as in this case, lichen sclerosus can cause permanent architectural changes and damage to the affected tissues, and can thus predisposes to secondary infections, including bacterial, such as with Staphylococcus haemolyticus.
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  • 文章类型: Journal Article
    有关新生儿男性包皮环切术(NMC)的道德和法律论点相反,值得对该主题进行首次系统回顾。我们对PubMed进行了符合PRISMA标准的关键字搜索,EMBASE,Scopus,LexisNexis,和其他数据库,并确定了61篇符合纳入标准的文章。在这些文章的参考书目中,我们确定了58篇相关文章和28个互联网项目。我们发现了高质量的证据,表明NMC是一种低风险的手术,可提供即时和终生的医疗和健康益处,并且很少导致对性功能或愉悦的不良影响。鉴于这些证据,我们得出结论,从《联合国儿童权利公约》的角度来看,劝阻或否认NMC是不道德的,强调健康权。Further,判例法支持NMC的合法性。我们发现,相反,反对NMC的伦理论据依赖于医学证据的扭曲。因此,NMC,由经验丰富的操作员使用可用的安全预防措施,似乎既合法又道德。与这个结论一致,我们审查的所有循证儿科政策都将NMC描述为低风险且对公共卫生有益.我们计算出,美国的NMC从80%减少到10%将大大增加不良医疗状况的病例。因此,本研究结果支持基于证据的NMC政策声明,并且与不鼓励NMC的非基于证据的政策不一致。在平衡,这里回顾的论点和证据表明,NMC是一种在生命早期的医学上有益和道德的公共卫生干预措施,因为它减少了痛苦,死亡,cases,以及包皮环切术患者一生中治疗不良医疗状况的费用。
    Contrasting ethical and legal arguments have been made concerning neonatal male circumcision (NMC) that merit the first systematic review on this topic. We performed PRISMA-compliant keyword searches of PubMed, EMBASE, SCOPUS, LexisNexis, and other databases and identified 61 articles that met the inclusion criteria. In the bibliographies of these articles, we identified 58 more relevant articles and 28 internet items. We found high-quality evidence that NMC is a low-risk procedure that provides immediate and lifetime medical and health benefits and only rarely leads to later adverse effects on sexual function or pleasure. Given this evidence, we conclude that discouraging or denying NMC is unethical from the perspective of the United Nations Convention on the Rights of the Child, which emphasizes the right to health. Further, case law supports the legality of NMC. We found, conversely, that the ethical arguments against NMC rely on distortions of the medical evidence. Thus, NMC, by experienced operators using available safety precautions, appears to be both legal and ethical. Consistent with this conclusion, all of the evidence-based pediatric policies that we reviewed describe NMC as low-risk and beneficial to public health. We calculated that a reduction in NMC in the United States from 80% to 10% would substantially increase the cases of adverse medical conditions. The present findings thus support the evidence-based NMC policy statements and are inconsistent with the non-evidence-based policies that discourage NMC. On balance, the arguments and evidence reviewed here indicate that NMC is a medically beneficial and ethical public health intervention early in life because it reduces suffering, deaths, cases, and costs of treating adverse medical conditions throughout the lifetimes of circumcised individuals.
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  • 文章类型: Journal Article
    背景:包茎是无法完全缩回包皮并露出龟头。包茎的治疗根据患者的年龄和疾病的严重程度而变化;目前有大量的保守或手术治疗。
    目的:为成人包茎的治疗提供第一篇综述。
    方法:PubMed,Cochrane和Embase搜索同行评审的研究,在2001年1月至2022年12月间发布的是使用搜索术语“包茎和治疗”进行的。
    结果:最初通过数据库搜索确定了总共288种出版物。最终有30份手稿有资格纳入本评论。保守治疗是一种选择。它包括局部类固醇应用和用于温和包皮扩张的新型医用硅管(Phimostop™)应用。关于手术方法,黄金标准治疗以包皮环切术为代表,其中去除包皮后的组织合成也可以用倒刺缝线获得,纤维蛋白胶或订书钉。与传统的包皮环切术相比,激光包皮环切术在手术时间和术后并发症发生率方面似乎具有更好的效果。还描述了几种包皮成形术和使用原位装置(压碎包皮并同时产生止血)的技术。这些原位装置似乎是可行的,与传统包皮环切术相比,它们在治疗包茎方面是安全有效的,同时也减少了手术时间。患者满意度,主要介绍了手术治疗的并发症及对性功能的影响。
    结论:成人包茎的治疗有许多保守和手术治疗方法。正确治疗的选择取决于包茎的等级,结果,并发症,和成本效益。
    BACKGROUND: Phimosis is the inability to completely retract the foreskin and expose the glans. The treatment of phimosis varies depending on the age of the patient and the severity of the disease; a great number of conservative or surgical treatments are currently available.
    OBJECTIVE: To provide the first review summarizing the available options for the treatment of adult phimosis.
    METHODS: A PubMed, Cochrane and Embase search for peer-reviewed studies, published between January 2001 and December 2022 was performed using the search terms \"phimosis AND treatment\".
    RESULTS: A total of 288 publications were initially identified through database searching. Thirty manuscripts were ultimately eligible for inclusion in this review. Conservative treatment is an option. and it includes topical steroid application and the new medical silicon tubes (Phimostop™) application for gentle prepuce dilation. Concerning the surgical approach, the gold-standard treatment is represented by circumcision in which tissue synthesis after prepuce removal can be also obtained with barbed sutures, fibrin glues or staples. Laser circumcision seems to be providing superior outcomes in terms of operative time and postoperative complication rate when compared to the traditional one. Several techniques of preputioplasty and use of in situ devices (which crush the foreskin and simultaneously create haemostasis) have been also described. These in situ devices seem feasible, safe and effective in treating phimosis while they also reduce the operative time when compared to traditional circumcision. Patient satisfaction rates, complications and impact on sexual function of the main surgical treatments are presented.
    CONCLUSIONS: Many conservative and surgical treatments are available for the treatment of adult phimosis. The choice of the right treatment depends on the grade of phimosis, results, complications, and cost-effectiveness.
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