lichen sclerosus et atrophicus

硬化性苔藓
  • 文章类型: Journal Article
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  • 文章类型: Letter
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  • 文章类型: Systematic Review
    推测了硬化性苔藓(LS)的合并症,但尚未进行详细分析。这篇综述的目的是确定LS患者的循证相关疾病,并探讨性别依赖性筛查方案的潜在需求。全面搜索MEDLINE,Embase,从开始到2024年2月29日的PsycINFO数据库使用关键搜索词LS及其所有同义词进行。使用DerSimonian和Laird随机效应模型生成合并症的合并优势比和95%置信区间。共有21项病例对照研究符合纳入标准。在分析的75种合并症中,只有16人(21.3%)在两种性别中进行了研究,没有显示基于性别的对比协会。女性和男性LS患者均显示出常见皮肤病的几率显着增加(即,扁平苔藓,白癜风,斑秃,特应性皮炎,和牛皮癣),各种心血管危险因素(即,原发性高血压,肥胖,血脂异常,糖尿病,和2型糖尿病),生殖器疣,与对照组相比,甲状腺功能减退。总的来说,目前缺乏数据并不支持性别依赖筛查策略的实施.调查结果确实如此,然而,与一系列潜在的严重合并症存在重大关联,这需要进一步阐明和临床警惕。
    The comorbidities of lichen sclerosus (LS) are presumed but have not been analysed in detail. The purpose of this review was to identify evidence-based associated diseases in patients with LS and explore the potential need for sex-dependent screening protocols. A comprehensive search of the MEDLINE, Embase, and PsycINFO databases from inception to 29 February 2024 was conducted using the key search terms LS and all its synonyms. Pooled odds ratios and 95% confidence intervals of comorbidities were generated using the DerSimonian and Laird random-effects model. A total of 21 case-control studies met the inclusion criteria. Of the 75 comorbidities analysed, only 16 (21.3%) were studied in both sexes, revealing no contrasting associations based on sex. Both female and male LS patients showed significantly increased odds of common dermatological conditions (i.e., lichen planus, vitiligo, alopecia areata, atopic dermatitis, and psoriasis), various cardiovascular risk factors (i.e., essential hypertension, obesity, dyslipidaemia, diabetes mellitus, and diabetes mellitus type 2), genital warts, and hypothyroidism compared with controls. Overall, the scarcity of data currently does not support the implementation of sex-dependent screening strategies. The findings do, however, present significant associations with a range of potentially serious comorbidities, which warrants further elucidation and clinical vigilance.
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  • 文章类型: Case Reports
    硬化性苔藓(LS)是一种主要影响生殖器的慢性炎症性皮肤病,通常特征为珍珠白色丘疹和斑块。虽然主要影响女性,LS可以在所有年龄组中出现,在青春期前女孩和绝经后妇女中观察到双峰分布。此病例报告显示了一名10岁女孩的特殊性外LS,在她的前臂和小腿上展示了类似扁平苔藓的色素沉着斑块和皱纹斑块。组织病理学分析证实LS,揭示独特的表皮变化和淋巴细胞浸润。没有粘膜受累和独特的临床表现将该病例与典型的LS表现区分开。局部用丙酸氯倍他索治疗显示瘙痒的显着改善。婚外LS很少见,特别是在儿童中,其诊断需要综合的临床病理相关性。报告的病例为这种罕见的变异提供了宝贵的见解,强调准确诊断和量身定制治疗策略的重要性。此外,它强调了高效外用皮质类固醇治疗这种疾病的疗效.
    Lichen sclerosus (LS) is a chronic inflammatory dermatosis primarily affecting the genitalia, commonly characterized by pearly-white papules and plaques. Although predominantly affecting females, LS can manifest across all age groups, with a bimodal distribution observed in prepubescent girls and postmenopausal women. This case report presents an unusual instance of exclusive extragenital LS in a 10-year-old girl, showcasing hyperpigmented patches and wrinkled plaques resembling lichen planus on her forearms and lower legs. Histopathological analysis confirmed LS, revealing distinctive epidermal changes and lymphocytic infiltrates. The absence of mucosal involvement and unique clinical presentation differentiated this case from typical LS manifestations. Treatment with topical clobetasol propionate demonstrated significant improvement in pruritus. Extragenital LS is infrequent, particularly among children, and its diagnosis necessitates a comprehensive clinicopathological correlation. The reported case contributes valuable insights into this uncommon variant, emphasizing the importance of accurate diagnosis and tailored treatment strategies. Additionally, it highlights the efficacy of high-potency topical corticosteroids in managing this condition.
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  • 文章类型: Case Reports
    硬化性苔藓是一种病因不明的慢性炎症性皮肤病,主要影响所有性别个体的生殖器表皮,在绝经后妇女和青春期前女孩中观察到较高的患病率。此外,在诊断为生殖器硬化性苔藓的患者中,约有20%出现生殖器外表现。值得注意的是,以卵泡为中心的生殖器外硬化性苔藓是罕见且不寻常的,现有文献中仅记录了有限的实例。
    我们报告了一名33岁的女士,其背足有多个无症状的病变1年,左手有类似的病变4个月。检查:卵泡中心,闪亮,萎缩性丘疹在双脚的背部合并成网状斑块,很少有光泽,平顶,左手背部有粉红色丘疹。进行了皮肤活检,并证实了生殖器外硬化性苔藓的诊断。
    以卵泡为中心的生殖器外硬化性苔藓是一种罕见且罕见的临床变异。临床病理相关性对于建立正确的诊断是必要的。
    这里,我们提出了一种不寻常的外皮硬化苔藓,我们强调了在鉴别诊断点滴状肢部皮肤病变时考虑它的重要性。我们还从文献中回顾和总结相关案例,希望对医生有所帮助,尤其是皮肤科医生,考虑并迅速达到诊断并提供适当的管理。我们也希望带来新的见解,并扩大未来的研究工作,特别是关于硬化性苔藓和萎缩性皮肤病。
    UNASSIGNED: Lichen sclerosus is a chronic inflammatory dermatological condition of unknown etiology, primarily impacting the genital epidermis in individuals of all genders, with a higher prevalence observed among postmenopausal women and prepubescent girls. Additionally, extragenital manifestations occur in approximately 20% of the patients diagnosed with genital lichen sclerosus. Notably, folliculocentric extragenital lichen sclerosus is rare and unusual, with only limited instances documented in existing literature.
    UNASSIGNED: We report a 33 years old lady presented with multiple asymptomatic lesions on the dorsal feet for 1 year and similar lesions on the left hand for 4 months. On examination: folliculocentric, shiny, atrophic papules coalescing into reticulated plaques over the dorsum of both feet and few shiny, flat-topped, pink papules over the dorsum of the left hand. A skin biopsy was performed and confirmed the diagnosis of extragenital lichen sclerosus.
    UNASSIGNED: Acral folliculocentric extragenital lichen sclerosus is an unusual and rare clinical variant. Clinicopathologic correlation is necessary to establish the correct diagnosis.
    UNASSIGNED: Herein, we present an unusual presentation of extragenital lichen sclerosus, and we highlight the importance of considering it in the differential diagnosis of guttate acral skin lesions. We also review and summarize relevant cases from the literature in hope to aid physicians, especially dermatologists, to consider and swiftly reach the diagnosis and offer appropriate management. We also hope to bring about new insights and broaden future research efforts regarding lichen sclerosus especially and atrophic skin disease in general.
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  • 文章类型: Case Reports
    硬化性苔藓(LSA)是一种慢性炎症性皮肤病变,病因不明。它更常见于生殖器区域,特别是在青少年中,绝经前妇女和绝经后妇女。LSA难以治疗并且经常复发。LSA的主要治疗包括施用有效的局部皮质类固醇。Dupilumab越来越多地用于治疗非特应性皮炎患者的瘙痒,但很少有关于其用于治疗LSA的报道。这里,我们介绍了一名61岁女性患有广泛外阴瘙痒的LSA病例。超过四个月的dupilumab治疗,观察到显著的治疗效果,包括外阴皮肤变薄和瘙痒缓解,无不良反应。
    Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory skin lesion with an undefined cause. It is more commonly found in the genital area, particularly in adolescents, premenopausal women and postmenopausal women. LSA is difficult to treat and often recurs. The primary treatment for LSA involves the administration of potent topical corticosteroids. Dupilumab is increasingly being used for the treatment of itching in non-atopic dermatitis patients but there are few reports on its use for the treatment of LSA. Here, we present a case of LSA in a 61-year-old woman with extensive vulvar itching. Over four months of dupilumab therapy, significant therapeutic effects were observed, including vulvar skin thinning and pruritus relief without adverse reactions.
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  • 文章类型: Case Reports
    硬化性苔藓(LSA)是一种慢性炎症性疾病,最常见的特征是位于女性生殖器上的萎缩性皮肤斑块。很少,LSA可能存在于外源性;然而,关于生殖器和生殖器外LSA之间的时间关系尚不清楚。Morphea,也被称为局限性硬皮病,是一种罕见的以硬化斑块为特征的炎症性皮肤病。调查人员争论LSA和硬皮病是否存在相同的疾病谱,LSA代表了累及生殖器的角膜浅层变体,或者如果它们是不同但巧合的实体。尽管研究人员已经描述了LSA和硬伤发生在同一患者的不同位置,很少有报道描述LSA和硬伤发生在同一病变和腹股沟褶皱。在这里,我们报告了一例62岁的女性,在腹股沟皱褶上有外缘LSA-mr骨重叠,三个月后发展为生殖器LSA。同一斑块中的LSA-硬伤,初次检查时没有生殖器病变的迹象,随着生殖器LSA的发展,尤其不常见。在三个月的时间内,生殖器外LSA-硬骨重叠到生殖器LSA的时间进展是对文献的重要贡献。因为以前没有讨论外生殖器和生殖器LSA之间的时间关系。
    Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory disorder, most often characterized by atrophic skin plaques located on female genitalia. Infrequently, LSA may present extragenitally; however, much is unknown about the temporal relationship between genital and extragenital LSA. Morphea, also known as localized scleroderma, is a rare inflammatory skin condition characterized by sclerotic plaques. Investigators debate whether LSA and morphea exist on the same spectrum of disease, with LSA representing a superficial variant of morphea involving genitalia, or if they are distinct but coincidental entities. Although researchers have described LSA and morphea occurring in different locations on the same patient, few reports describe LSA and morphea occurring in the same lesion and in the inguinal folds. Herein, we report a case of a 62-year-old woman with extragenital LSA-morphea overlap in the inguinal folds, who three months later developed genital LSA. Extragenital LSA-morphea in the same plaque, with no signs of genital lesions on initial exam, with later development of genital LSA, is especially uncommon. The temporal progression of extragenital LSA-morphea overlap to genital LSA over a three-month period is an important contribution to the literature, as the temporal relationship between extragenital and genital LSA is not previously discussed.
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  • 文章类型: Clinical Trial
    背景:硬化性苔藓是一种炎症性疾病,女性肛门生殖器区域的瘢痕性皮肤病,可能导致疼痛和性功能障碍。在保守治疗难以治疗的部分病例中,手术可以提供显著的症状改善。这项研究的目的是通过提供使用闭孔前动脉穿支(aOAP)皮瓣的专门重建方法的手术结果,扩大这些患者的手术治疗选择范围。
    方法:采用aOAP皮瓣剥皮外阴切除术切除受影响的外阴前庭组织并随后进行单阶段重建后的性结局进行了回顾性队列研究。附加程序,如欧米茄圆顶(OD)皮瓣,疤痕手术和阴蒂再暴露,在指示时进行。
    结果:在2014年至2022年之间,共有61例患者接受了手术治疗,并回顾性纳入本研究。53例(87%)进行了外阴切除术和随后的双侧aOAP皮瓣重建。与基线相比,在1年的随访中,性交困难和无法进行性交的患病率显着降低(p<0.001)。有几个未成年人,需要二次干预的可逆性并发症。
    结论:这项研究的结果表明性功能有了实质性的改善,表现为性交困难的显着减少和进行性交的能力增强。硬化性苔藓和萎缩性苔藓患者的组织质量改变和长期使用可的松可能会使该患者人群发生轻微术后并发症的风险更高。
    背景:DRKS00033261。
    BACKGROUND: Lichen sclerosus et atrophicus is an inflammatory, scarring dermatosis of the female anogenital area and may lead to pain and sexual dysfunction. In select cases which are refractory to conservative therapy, surgery may provide significant symptom improvement. The objective of this study was to expand the range of surgical treatment options for these patients by presenting the operative outcomes of a specialised reconstructive method using the anterior obturator artery perforator (aOAP) flap.
    METHODS: A retrospective cohort study was conducted on sexual outcomes following the excision of affected vulvovestibular tissue by skinning vulvectomy and subsequent single-stage reconstruction using the aOAP flap. Additional procedures, such as the Omega-Domed (OD) flap, scar surgery and clitoral re-exposure, were performed when indicated.
    RESULTS: Between 2014 and 2022, a total of 61 patients were surgically treated and retrospectively included in this study. Vulvectomy and subsequent reconstruction with bilateral aOAP flaps were performed in 53 (87%) cases. There was a significant reduction in the prevalence of dyspareunia and inability to have sexual intercourse at the 1-year follow-up compared to baseline (p < 0.001). There were several minor, reversible complications that required secondary intervention.
    CONCLUSIONS: The outcomes of this study indicate a substantial improvement in sexual function, evidenced by a significant reduction in dyspareunia and an increased ability to engage in sexual intercourse. Altered tissue quality in patients with lichen sclerosus et atrophicus and long-term cortisone application may predispose this patient population to a higher risk of minor post-operative complications.
    BACKGROUND: DRKS00033261.
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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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