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: Case Reports
    硬化性苔藓(LS)是一种典型的生殖器区域的慢性炎症性皮肤病,罕见地累及外生区,尤其是面部。LS治疗管理具有挑战性,和常见的疗法,包括局部和全身皮质类固醇,局部钙调磷酸酶抑制剂,手术往往是无效的。在这里,我们介绍了一例LS发生在1名36岁女孩中,她的面部受累对全身性糖皮质激素和外用他克莫司治疗产生抵抗.考虑到一个敏感地区的参与,病人的年龄很小,以及使用光动力疗法治疗面部皮肤疾病的一致临床经验,我们开始使用5-氨基乙酰丙酸(ALA)-光动力疗法(PDT),每月一次,剂量为37J/cm2.我们将我们的病例与文献中的其他八名面部LS患者进行了比较,并进行了不同的治疗。
    Lichen sclerosus (LS) is a chronic inflammatory dermatosis typical of the genital region, with rare involvement of extragenital areas and particularly the face. LS therapeutic management is challenging, and common therapies including topical and systemic corticosteroids, topical calcineurin inhibitors, surgery are often ineffective. Herein, we present a case of LS occurred in a 36-year-old girl with facial involvement resistant to therapy with systemic corticosteroids and topical tacrolimus. Considering the involvement of a sensitive area, the young age of the patient, and the consistent clinical experience in using photodynamic therapy for the treatment of facial skin disease, we started a treatment with topical 5-aminolevulinic acid (ALA)-photodynamic therapy (PDT) with a dosage of 37 J/cm2 once a month. We compared our case with eight other facial LS patients from the literature and treated differently.
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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: Case Reports
    阴道硬化性苔藓(LS)是一种极为罕见的实体。经典的,LS被称为慢性,炎症性皮肤病,在绝经后妇女中观察到的肛门生殖器皮肤明显好感,通常在临床上表现为白色,萎缩性斑块.这里,我们报道了一例61岁的患者,该患者在阴道近距离放射治疗后3年接受随访,作为子宫内膜腺癌的辅助治疗.对该病变进行活检,并在组织学分析中确认为阴道LS。虽然先前已观察到LS会影响肛门生殖器区域以外的粘膜区域,比如嘴巴,报道的阴道LS病例在文献中非常罕见。我们的案例强调了这种疾病的未被认识到的位置以及辐射作为潜在的危险因素。
    Vaginal lichen sclerosus (LS) is an extremely rare entity. Classically, LS is referred to as a chronic, inflammatory skin disease with a distinct predilection for the anogenital skin that is observed in post-menopausal women and typically manifests clinically as white, atrophic plaques. Here, we report a case of a 61-year-old patient who presented for a follow-up visit three years after vaginal brachytherapy as an adjuvant treatment for endometrial adenocarcinoma. This lesion was biopsied and confirmed to be vaginal LS on histological analysis. While LS has been previously observed to impact mucosal areas outside of the anogenital region, such as the mouth, reported cases of vaginal LS are very rare in the literature. Our case highlights both the underrecognized location of this disease as well as radiation as a potential risk factor.
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  • 文章类型: Journal Article
    背景:硬化性苔藓(LS)是一种慢性,自身免疫性皮肤病主要位于女性的肛门生殖器区域。近年来,人类微生物群在自身免疫性疾病发病机理中的作用,包括LS,收到了兴趣。
    目的:该研究旨在评估和比较尿液的成分,与未受影响的对照组相比,患有LS的女性的阴道和肠道微生物群。
    方法:被诊断为LS的女性(n=16)和匹配的对照组(n=14)被纳入研究。每个参与者,中游尿液,上下阴道拭子,以及粪便样本,被收集。使用V4高变区的16S核糖体RNA(rRNA)基因测序评估微生物群组成。
    结果:我们在四个解剖壁龛中都没有观察到LS特异性聚类,使用层次聚类分析或加权β多样性指标。然而,对于未加权的UniFrac,当将患有LS的女性与对照组进行比较时,观察到泌尿和下阴道微生物群存在显著差异.这些发现表明,虽然这两个群体的微生物群以相同的细菌为主,变异确实发生在数量较少的细菌中。LEfSe分析显示,与对照组相比,LS女性的泌尿和下阴道微生物群中链球菌属的相对丰度更高。此外,与对照组相比,在患有LS的女性的肠道微生物群中观察到更高的Euryarchaeota门相对丰度。
    结论:在这项研究中,我们证明了尿液中不那么丰富的细菌之间的几个差异,将LS女性与对照组进行比较时,阴道和粪便微生物区系较低。然而,需要进一步的研究来评估这些微生物群差异是致病的还是仅仅是潜在LS疾病的结果.
    BACKGROUND: Lichen sclerosus (LS) is a chronic, autoimmune skin disease predominantly located in the anogenital region in women. In recent years, the role of the human microbiota in the pathogenesis of autoimmune diseases, including LS, has received interest.
    OBJECTIVE: The study aimed to evaluate and compare the composition of the urinary, vaginal and gut microbiota in women with LS versus non-affected controls.
    METHODS: Women diagnosed with LS (n = 16) and matched controls (n = 14) were enrolled in the study. From each participant, midstream urine, upper and lower vaginal swabs, as well as faecal samples, were collected. The microbiota composition was assessed using 16S ribosomal RNA (rRNA) gene sequencing of the V4 hypervariable region.
    RESULTS: We observed no LS-specific clustering in either of the four anatomic niches, using either hierarchical cluster analysis or weighted beta diversity metrics. However, for unweighted UniFrac, significant differences in the urinary and lower vaginal microbiota were observed when comparing women with LS to controls. These findings indicate that while the two groups have microbiota dominated by the same bacteria, variations do occur amongst less abundant bacteria. The LEfSe analysis revealed a higher relative abundance of the genus Streptococcus in the urinary and lower vaginal microbiota in women with LS compared to controls. Additionally, a higher relative abundance of phylum Euryarchaeota was observed in the gut microbiota in women with LS compared to controls.
    CONCLUSIONS: In this study, we demonstrated several differences amongst less abundant bacteria in the urinary, lower vaginal and faecal microbiota when comparing women with LS to controls. However, further research is required to assess whether these microbiota differences are causative or merely a result of the underlying LS disease.
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  • 文章类型: Journal Article
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    大疱性类天疱疮(BP)代表最常见的自身免疫性大疱性疾病,其特征在于IgG自身抗体靶向胶原蛋白XVII(BP180)。据报道,BP与其他炎症性皮肤病有关。这里,我们描述了一名女性患者的血压异常发生,该女性患者伴有全身性硬皮病(局限性硬皮病,LoS)和皮肤和生殖器硬化性苔藓(LiS)。BP的发生与血清抗BP180IgG自身抗体水平升高有关,在临床缓解后下降。自身免疫性大疱性疾病和硬化性皮炎是免疫学上不同的实体,其关联很少被描述。在这项研究中,我们对LoS或LiS患者发生BP的病例进行了文献综述.Further,我们讨论了可能有利于我们患者出现BP的免疫机制。
    Bullous pemphigoid (BP) represents the most common autoimmune bullous disease and is characterized by IgG autoantibodies targeting collagen XVII (BP180). BP has reportedly been occurred in association with other inflammatory skin diseases. Here, we describe the unusual occurrence of BP in a female patient with a concomitant history of generalized morphea (localized scleroderma, LoS) and cutaneous and genital lichen sclerosus (LiS). The occurrence of BP was associated with elevated serum levels of anti-BP180 IgG autoantibodies, which decreased upon clinical remission. Autoimmune bullous diseases and sclerosing dermatitis are immunologically distinct entities, whose association has been rarely described. In this study, we provide a literature review on cases of BP developed in patients with either LoS or LiS. Further, we discussed immunological mechanisms which may have favored the emergence of BP in our patient.
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  • 文章类型: Case Reports
    扁平苔藓硬化(LS)是一种阴险的,慢性,以萎缩性为特征的复发性皮肤病,瓷器出现的斑块。它通常出现在肛门生殖器区域,但是有些病例可以出现在生殖器外区域,有各种各样的表现,包括bullous变体。局部皮质类固醇是一线治疗,通常是诱导LS缓解的最有效治疗方法。然而,有一部分患者对局部用类固醇反应不佳.在这里,我们报告了一例经点阵CO2激光(FxCO2)和随后的halcinonide溶液湿敷成功治疗的外大疱性LS病例.
    Lichen sclerosis (LS) is an insidious, chronic, relapsing skin disease characterized by atrophic, porcelain-appearing plaques. It usually arises in the anogenital area, but some cases can present in extragenital regions with a variety of presentations, including a bullous variant. Topical corticosteroids are a first-line therapy and are usually the most effective treatment to induce remission of LS. However, there is a subset of patients that does not respond well to topical steroids. Herein, we report an extragenital bullous LS case successfully treated with a fractional CO2 laser (FxCO2) and subsequent wet dressing of halcinonide solution.
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  • 文章类型: Letter
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