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:据估计,大约每200个男孩中就有一个被诊断为硬化性苔藓(LS),以前称为BXO(干燥龟头炎)。然而,严重的进行性疾病很少见,尿道组织管理不善可能导致LS的进展。
    方法:回顾了当前有关严重硬化性苔藓治疗的文献,同时回顾了我们对7名年龄在6至10岁之间的严重硬化性苔藓患者的治疗,这些患者需要手术干预。这些患者是在繁忙的儿科实践中确定的,在四年的时间里看到了5507名患者。根据硬化性苔藓的病理生理学,尿道解剖,我们的管理开发了一种用于医疗和外科管理的算法。
    结果:所有患者都接受了局部类固醇的初始药物治疗。三名患者接受了尿道动员和连续活检。其中一名患有严重疾病的患者需要第二次远端尿道动员。三名患者接受了包皮环切术治疗,一个在复杂的重建后对局部类固醇反应良好。无术后尿道疾病。
    结论:硬化性苔藓影响鳞状上皮,但如果通过Koebner现象受到创伤,则可以扩展到未受影响的组织。目前尚无治疗该疾病的随机对照试验。因此,适当的早期治疗避免尿道扩张或切口可以防止尿道向下延伸,这可能导致严重的狭窄疾病。几位作者认为这是最糟糕的狭窄疾病之一。根据疾病的病理生理学和我们治疗患者的4年经验,我们提出了一种管理男孩严重硬化性苔藓的算法。诊断为男孩硬化性苔藓需要高度怀疑,如果怀疑,应进行早期活检。如果在包皮环切术或切肉术前被发现,最初的治疗应该是医学的。如果患者局部类固醇治疗失败,包皮环切术和活检是最初推荐的手术方法.Optimally,第一次切肉手术的活检确定了诊断。如果疾病持续存在,尿道动员可能是一种治愈性治疗,因为它可以促进尿道健康,并允许完全切除远端鳞状上皮,而不是创伤性的重复扩张或切口。
    结论:本文总结了有关严重LS的处理的现有文献,并根据疾病的病理生理学和我们的经验提供了流程图。
    BACKGROUND: It is estimated that approximately one out of 200 boys has the diagnosis of lichen sclerosus (LS), previously referred to as BXO (balanitis xerotica obliterans). Severe progressive disease is rare however, mismanagement of urethral tissues may contribute to progression of LS.
    METHODS: The current literature regarding the management of severe lichen sclerosus was reviewed alongside our management of seven patients with ages ranging from six to ten years of age with severe lichen sclerosus who required surgical intervention. These patients were identified out of a busy pediatric practice that saw 5507 patients during the four-year span. Based on the pathophysiology of lichen sclerosus, urethral anatomy, and our management an algorithm was developed for medical and surgical management.
    RESULTS: All patients received initial medical treatment with topical steroids. Three patients underwent urethral mobilization and serial biopsy. One of these patients with severe disease required a second distal urethral mobilization. Three patients were treated with circumcision, and one is responding well to topical steroids after complex reconstruction. None developed postoperative urethral disease.
    CONCLUSIONS: Lichen sclerosus affects squamous epithelium but can extend to unaffected tissue if traumatized via the Koebner phenomenon. There are no randomized control trials for the management of the disease. Thus, appropriate early management with avoidance of urethral dilation or incision may prevent extension down the urethra that can lead to severe stricture disease. Several authors identified this as one of the worst forms of stricture diseases to manage. Based on the pathophysiology of the disease and our 4-year experience treating patients, we propose an algorithm for management of severe lichen sclerosus in boys. The diagnosis of lichen sclerosus in boys requires a high level of suspicion, and early biopsies should be obtained if suspected. If identified before circumcision or meatotomy, initial treatment should be medical. If the patient fails topical steroid therapy, circumcision and biopsy are the initial recommended surgical approach. Optimally, a biopsy with the first meatotomy establishes the diagnosis. If the disease persists, urethral mobilization may represent a curative treatment as it advances healthy urethra and allows complete removal of distal squamous epithelium instead of traumatic repeated dilations or incisions.
    CONCLUSIONS: This paper summarizes the available literature on the management of severe LS and provides a flow diagram based on the pathophysiology of the disease and our experience sever cases.
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  • 文章类型: Journal Article
    目的:我们描述了接受包皮环切术治疗硬化性苔藓(LS/BXO)的男孩的饮食结局,重点是那些在包皮环切术时接受了切肉手术/子宫移植术的男孩以及与包皮环切术后干预相关的因素。
    方法:回顾性回顾了2011年至2020年间接受包皮环切术的患者的组织学证实的LS。通过Chi2和多变量分析进行统计检验。
    结果:382例患者接受包皮环切术平均9.1年(SD2.9)。在包皮环切术时,在213/365(58%)中记录了龟头上的LS。在74/382(19%)中记录了大量受累;25/382(6.5%)进行了切肉手术,94/382(25%)的饮食校准/扩张和234/367(64%)的手术后局部类固醇处方。LS龟头或受累的患者更有可能进行切肉手术(p=0.0013)并接受术后类固醇(OR5,p=0.0001)。包皮环切术后,40/382(10%)需要1次后续手术的中位数(范围1-5),10人(2.6%)接受扩张,30(7.4%)进行了切肉手术。在包皮环切术中进行切肉手术的患者在随后的切肉手术中的比值比(OR)为1.2(p=0.027)。根据任何后续手术的要求进行的分析确定,在包皮环切术中进行切肉手术的OR为3.1(p=0.022),接受术后类固醇的OR为6.0(p=<0.001)。
    结论:对于需要肠道干预的LS,包皮环切术后的肠道狭窄影响了10%的男孩。包皮环切术时的切肉术增加了随后进行饮食干预的可能性,因此不建议使用。
    方法:三级。
    OBJECTIVE: We describe meatal outcomes for boys undergoing circumcision to treat Lichen Sclerosus (LS/BXO) with a focus on those who underwent meatotomy/meatoplasty at circumcision and factors associated with post-circumcision meatal intervention.
    METHODS: Retrospective review of patients undergoing circumcision for histologically confirmed LS between 2011 and 2020. Statistical testing was by Chi2 and multivariate analysis.
    RESULTS: 382 patients underwent circumcision at a mean of 9.1 years (SD 2.9). At circumcision, LS on the glans was documented in 213/365 (58%). Meatal involvement was documented in 74/382 (19%); 25/382 (6.5%) had a meatotomy, 94/382 (25%) had meatal calibration/dilatation and 234/367 (64%) were prescribed post-operative topical steroids. Patients with LS glans or meatal involvement were more likely to have a meatotomy (p = 0.0013) and to receive post-operative steroids (OR 5, p = 0.0001). Post circumcision, 40/382 (10%) required a median of 1 subsequent procedure (range 1-5), 10 (2.6%) underwent dilatation, 30 (7.4%) had a meatotomy. Patients undergoing meatotomy at circumcision had an odds ratio (OR) of 1.2 for subsequent meatotomy (p = 0.027). Analysis based on requirement for any subsequent procedure identified an OR of 3.1 for having had a meatotomy at circumcision (p = 0.022) and an OR of 6.0 of receiving post-operative steroids (p=<0.001).
    CONCLUSIONS: Meatal stenosis following circumcision for LS requiring meatal intervention affected 10% of boys. Meatotomy at circumcision increased the likelihood of subsequent meatal intervention and is therefore not recommended.
    METHODS: Level III.
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  • 文章类型: Letter
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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
    目的:评估高效局部使用类固醇对苔藓硬化相关外阴癌复发风险的影响。
    方法:这是一项回顾性队列研究,评估患有硬化苔藓(LS)相关外阴鳞状细胞癌(VSCC)的患者。人口统计学和临床结果数据在两个比较组之间进行了比较:接受类固醇治疗的患者,主要是氯倍他索,以及LS相关外阴癌治疗后未接受类固醇治疗的患者。使用Fisher精确检验或卡方检验比较分类变量。使用双侧学生t检验比较连续变量。使用Kaplan-Meier生存图分析复发时间(TTR)和总生存期(OS),并使用Mantel-Cox对数秩检验进行比较。进行Cox比例风险回归模型以产生TTR和OS的风险比。<0.05的p值被认为是统计学上显著的。
    结果:共纳入49例患者,36例患者接受类固醇治疗,13例患者为期待管理组。诊断年龄中位数为68岁。平均BMI为31.7+/-7.0。中位随访时间为41个月。大多数患者被诊断为I期VSCC。两组外阴癌的人口统计学或肿瘤学管理没有差异。与未接受类固醇治疗的患者相比,接受类固醇治疗的患者总体复发减少,12例患者(33.3%)与9例患者(69.2%)(p=0.048)。
    结论:治疗硬化性苔藓相关的外阴鳞状细胞癌后高效局部使用类固醇与复发风险降低和中位复发时间延长相关。
    OBJECTIVE: To evaluate the impact of high-potency topical steroid use on risk of recurrence of lichen sclerosus-associated vulvar cancer.
    METHODS: This is a retrospective cohort study evaluating patients with lichen sclerosus (LS)- associated vulvar squamous cell cancer (VSCC). Demographic and clinical outcome data were compared between two comparison groups: patients who received steroids, mainly clobetasol, and patients who did not receive steroids following treatment of LS-related vulvar cancer. Categorical variables were compared using Fisher\'s exact test or chi-square test. Continuous variables were compared using a two-sided student\'s t-test. Time to recurrence (TTR) and overall survival (OS) were analyzed using Kaplan-Meier survival plot and compared using Mantel-Cox log rank test. Cox proportional hazard regression models were conducted to generate hazard ratios for both TTR and OS. A p value of <0.05 was considered statistically significant.
    RESULTS: A total of 49 patients were included, with 36 patients receiving steroid treatment and 13 patients in the expectant management group. The median age of diagnosis was 68. The average BMI was 31.7 +/- 7.0. The median length of follow up was 41 months. The majority of patients were diagnosed with stage I VSCC. There was no difference in demographics or oncologic management of vulvar cancer between the two cohorts. Overall recurrence was decreased among patients who received steroid treatment when compared to patients who did not, 12 patients (33.3%) versus 9 patients (69.2%) respectively (p = 0.048).
    CONCLUSIONS: High-potency topical steroid use following treatment of lichen sclerosus-associated vulvar squamous cell carcinoma is associated with decreased risk of recurrence and prolonged median time to recurrence.
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