morphea

硬斑
  • 文章类型: Journal Article
    局限性硬皮病(LS),通常被称为morphea,由于其慢性,提出了一个重大的临床挑战,炎症性质影响皮肤和潜在的下层组织。这篇系统的综述探讨了激光治疗和注射填充剂相结合的创新方法,特别是透明质酸,用于治疗LS。我们遵循PRISMA指南进行了全面的文献综述,检查MEDLINE/PubMed的文章,以评估这些治疗方法在改善LS患者的美学和功能结局方面的综合疗效。搜索产生了64篇文章,选择了6名患者进行深入分析,共9名患者,涵盖一系列患者人口统计学和治疗类型。我们的评论重点介绍了部分CO2激光治疗促进长期组织重塑的情况,以及透明质酸填充剂有效解决皮肤萎缩和体积损失的情况。增强即时和持久的审美改善。这些治疗方法之间的协同作用表明了一种有希望的双重方法,旨在最大限度地提高LS患者的审美结果和生活质量。这篇综述强调了进一步研究以建立全面、循证临床路径整合两种治疗方法来管理LS,从而提高患者的满意度和解决这种具有挑战性的皮肤病的多面性。
    Localized scleroderma (LS), commonly known as morphea, presents a significant clinical challenge due to its chronic, inflammatory nature affecting the skin and potentially underlying tissues. This systematic review explores the innovative approach of combining laser therapy and injectable fillers, specifically hyaluronic acid, for the treatment of LS. We conducted a comprehensive literature review following PRISMA guidelines, examining articles from MEDLINE/PubMed to assess the combined efficacy of these treatments in improving both esthetic and functional outcomes for LS patients. The search yielded 64 articles, with six selected for in-depth analysis for a total of nine patients, covering a range of patient demographics and treatment types. Our review highlights cases where fractional CO2 laser therapy promoted long-term tissue remodeling and instances where hyaluronic acid fillers effectively addressed skin atrophy and volume loss, enhancing both immediate and long-lasting esthetic improvements. The synergy between these treatments suggests a promising dual approach, aiming to maximize esthetic outcomes and to improve the quality of life for LS patients. This review underscores the necessity of further research to establish a comprehensive, evidence-based clinical pathway integrating both treatments for managing LS, thereby enhancing patient satisfaction and addressing the multifaceted nature of this challenging dermatological condition.
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  • 文章类型: Journal Article
    背景:目前对局部硬皮病(LS)的治疗已被证明可以阻止疾病活动,但是对这些治疗的患者体验知之甚少,对于未来临床试验的最佳测量策略也没有达成共识.
    目的:对结果和措施类型的文献进行范围审查(即临床医生-,患者-,和护理人员报告的)用于已发表的LS治疗研究。
    方法:在在线数据库中搜索与评估LS治疗效果相关的文章,特别关注儿科。
    结果:在168项研究中,最常用的结局是通过临床医生报告的评估测量的皮肤疾病活动度和损伤.最常引用的测量是局部硬皮病皮肤评估工具(LoSCAT)。很少使用患者报告的结果测量(PROM)。
    结论:一些研究只是模糊地报告了所使用的措施,并且该审查产生的临床试验数量很少。
    结论:除了用临床医生报告的措施评估疾病活动外,该领域可以通过包括症状和功能的高质量PROM来获得关于患者体验的关键知识。需要使用各种结果和措施进行更多的临床试验,以确定最适合LS患者的治疗过程。
    BACKGROUND: Current treatment for localized scleroderma (LS) has been shown to halt disease activity, but little is still known about patient experiences with these treatments, nor is there consensus about optimal measurement strategies for future clinical trials.
    OBJECTIVE: Conduct a scoping review of the literature for the types of outcomes and measures (i.e. clinician-, patient-, and caregiver-reported) utilized in published treatment studies of LS.
    METHODS: Online databases were searched for articles related to the evaluation of treatment efficacy in LS with a special focus on pediatrics.
    RESULTS: Of the 168 studies, the most common outcomes used were cutaneous disease activity and damage measured via clinician-reported assessments. The most frequently cited measure was the Localized Scleroderma Cutaneous Assessment Tool (LoSCAT). Few patient-reported outcome measures (PROMs) were used.
    CONCLUSIONS: Some studies only vaguely reported the measures utilized, and the review yielded a low number of clinical trials.
    CONCLUSIONS: In addition to evaluating disease activity with clinician-reported measures, the field could obtain critical knowledge on the patient experience by including high-quality PROMs of symptoms and functioning. More clinical trials using a variety of outcomes and measures are necessary to determine the most suitable course of treatment for LS patients.
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  • 文章类型: Journal Article
    Morphea,自身免疫性进行性疾病,会显著影响患者的健康,然而治疗选择,虽然扩张,表现出有限的功效。疾病管理的持续挑战围绕监测疾病活动和衡量治疗有效性。为了解决这个问题,已经设计了各种临床评估工具,每个都有其固有的局限性。角膜的成像领域已经经历了值得注意的扩展,超声检查(US)成为量化疾病活动和评估治疗结果的有效且具有成本效益的途径。然而,对其应用的证据支持仍然模棱两可。我们的目的是探索和分析有关超声在硬皮治疗中的应用的现有证据。
    我们使用PubMedMedline进行了全面的文献综述,以评估有关美国在角膜管理中的效用的证据。
    共16项研究纳入我们的综述。
    尽管提出的研究有其自身的局限性,累积发现表明超声的潜力,特别是当与多普勒耦合时,为了促进分期,评估疾病活动,并纵向评估患者的治疗效果。
    UNASSIGNED: Morphea, an autoimmune progressive disorder, can significantly impact patient well-being, yet therapeutic options, though expanding, exhibit limited efficacy. A persistent challenge in disease management revolves around monitoring disease activity and gauging treatment effectiveness. To address this, various clinical assessment tools have been devised, each with its inherent limitations. The realm of imaging in morphea has undergone noteworthy expansion, with ultrasonography (US) emerging as an efficacious and cost-effective avenue for quantifying disease activity and evaluating therapeutic outcomes. However, the evidential support for its application remains equivocal. Our aim was to explore and analyze the existing evidence concerning the utility of ultrasound in the management of morphea.
    UNASSIGNED: We conducted a comprehensive literature review using PubMed Medline to assess evidence concerning US utility in morphea management.
    UNASSIGNED: Sixteen total studies were included in our review.
    UNASSIGNED: Although the studies presented carry their own limitations, cumulative findings indicate the potential of ultrasound, particularly when coupled with Doppler, in facilitating staging, assessing disease activity, and longitudinal assessment of therapeutic efficacy in patients with morphea.
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  • 文章类型: Letter
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  • 文章类型: Journal Article
    偏侧肌痉挛(HMS)是一种罕见的运动障碍,其特征是单侧颌骨闭合肌肉的阵发性痉挛或抽搐。本研究旨在综合评价HMS患者的临床特征。回顾性分析了17例新诊断为HMS的患者(12例女性和5例男性;发病平均年龄:46.7岁)的数据。从电子医疗数据库开始到2023年11月30日,进行了文献检索。对相关文献中引用的文章进行了手动搜索。分析了57项研究中的117例病例(72例女性和45例男性;平均发病年龄:37.1岁)。受累肌肉为咬肌(97.4%),颞肌(47.9%),内侧翼状体(6%)。在23.9%的患者中观察到硬皮病或硬皮病,面部偏侧萎缩占27.4%。在17.9%的病例中,Parry-Romberg综合征复杂或可疑。典型的肌电图检查结果包括痉挛期间没有沉默期(23.9%)和多个运动单位电位的不规则短暂爆发。口服药物,如氯硝西泮或卡马西平,缓解了一些患者的症状,但通常不令人满意。肉毒杆菌毒素疗法在大多数情况下是有效的。最近,微血管减压术的应用越来越广泛,在某些情况下导致完全救济。总之,目前有非常有效的模式,有必要提高对HMS的认识,以确保医疗和牙科专业人员都能准确诊断和治疗HMS。
    Hemimasticatory spasm (HMS) is a rare movement disorder characterized by paroxysmal spasms or twitches of the unilateral jaw-closing muscles. This study aimed to comprehensively evaluate the clinical features of patients with HMS. Data from 17 patients newly diagnosed with HMS (12 females and 5 males; mean age at onset: 46.7 years) who visited our department were retrospectively analyzed, and a literature search based on electronic medical databases from their inception until November 30, 2023, was conducted. A manual search was conducted for articles cited in the related literature. A total of 117 cases (72 females and 45 males; mean age at onset: 37.1 years) from 57 studies were analyzed. The muscles involved were the masseter (97.4%), temporalis (47.9%), and medial pterygoid (6%). Morphea or scleroderma was observed in 23.9% of the patients, and facial hemiatrophy in 27.4%. In 17.9% of the cases, Parry-Romberg syndrome was either complicated or suspected. Typical electromyographic findings included the absence of a silent period during spasms (23.9%) and irregular brief bursts of multiple motor unit potentials. Oral medicines, such as clonazepam or carbamazepine, alleviated the symptoms for some patients but were often unsatisfactory. Botulinum toxin therapy was effective in most cases. Recently, microvascular decompression surgery is increasingly being used, resulting in complete relief in some cases. In conclusion, highly effective modalities are currently available, and it is necessary to raise awareness of HMS to ensure that it can be diagnosed and treated accurately by both medical and dental professionals.
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  • 文章类型: Review
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  • 文章类型: Journal Article
    Morphea是一种自身免疫性纤维化皮肤病。嗜酸性筋膜炎(EF)被认为属于严重的硬皮病谱。我们进行了一项范围审查,评估了硬伤/EF患者的继发性癌症风险。副肿瘤性硬伤/EF和硬伤/EF继发于癌症治疗。搜索是使用MEDLINE进行的,Embase,Cochrane数据库,用于从开始到2022年9月发表的文章,遵循系统评价和范围评价荟萃分析的首选报告项目(PRISMA-ScR)指南,没有语言或日期限制。包括200项研究。其中,32项研究报道了硬伤/EF患者的继发性癌症,副肿瘤性硬伤/EF为45,癌症治疗诱导的硬伤/EF为125。虽然目前的证据仍然有限,数据表明,在硬伤患者中,继发性皮肤和胰腺恶性肿瘤的风险增加,特别是广义子类型。EF的数据不足。另一方面,副肿瘤性角膜是轶事,而一些观察性研究表明,约10%的EF病例可能是副肿瘤,主要在血液系统恶性肿瘤的背景下。放射疗法引起的硬伤很少见,在~0.2%的治疗患者中观察到,通常局限于治疗部位,除了预先存在自身免疫的患者。虽然报道了化疗引起的病例,免疫治疗的硬伤/EF病例正在出现,并且优先使用PD-1而不是CTLA-4抑制剂。这项研究受到所包括文章类型的限制(病例报告,病例系列和观察性研究),因此,需要对这一重要课题进行更多的研究。
    Morphea is an autoimmune fibrotic skin disease. Eosinophilic fasciitis (EF) is considered to belong to the severe spectrum of morphea. We conducted a scoping review assessing the risk of secondary cancer among morphea/EF patients, paraneoplastic morphea/EF and morphea/EF developing secondary to cancer therapy. The search was conducted using MEDLINE, Embase, Cochrane databases for articles published from inception to September 2022 following the Preferred Reporting Items for Systematic reviews and Meta-Analyses for Scoping Reviews (PRISMA-ScR) guidelines with no language or date restrictions. Two hundred and one studies were included. Of these, 32 studies reported on secondary cancer in morphea/EF patients, 45 on paraneoplastic morphea/EF and 125 on cancer-treatment-induced morphea/EF. While the current evidence remains limited, data suggest an increased risk of secondary cutaneous and possibly pancreatic malignancy in morphea patients, particularly the generalized subtype. There were insufficient data for EF. On the other hand, paraneoplastic morphea was anecdotal, whereas several observational studies suggested that ~10% of EF cases may be paraneoplastic, primarily in the context of hematologic malignancies. Radiotherapy-induced morphea is rare, seen in ~0.2% of treated patients and is usually localized to the treatment site, except in patients with pre-existing autoimmunity. While chemotherapy-induced cases are reported, immunotherapy morphea/EF cases are emerging and are preferentially seen with PD-1 and not CTLA-4 inhibitors. This study is limited by the type of articles included (case reports, case series and observational studies), and hence, additional research on this important topic is needed.
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  • 文章类型: Case Reports
    在这项研究中,我们报告了一例罕见的军刀政变,皮肤下的左额骨增生,在磁共振成像筛查和术前评估期间检测到。一名27岁女性,有13年的前额和头皮进行性软组织凹陷病史,并在疾病进入静止期之前用传统中草药治疗。患者接受了连续的长脉冲激光治疗和自体脂肪移植,结果令人满意。据我们所知,这是第一次在患有encoupdesabre的患者中发现软组织病变下的骨性增生。
    In this study, we report a rare case of en coup de sabre with hyperplasia of the left frontal bone beneath skin lesion, which is detected during magnetic resonance imaging screening and preoperative evaluation. A 27-year-old woman with 13-year history of progressive soft tissue depression in the forehead and scalp, and was treated by traditional Chinese herb before the disease went into stationary stage. The patient underwent serial long-pulsed laser treatments and autologous fat grafting with satisfactory outcome. To our knowledge, this is the first time that bony hyperplasia beneath the soft tissue lesion was found in a patient with en coup de sabre.
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  • 文章类型: Journal Article
    背景:局限性硬皮病,被称为morphea,是以皮肤和软组织的炎症和纤维化为特征的结缔组织病症。为了监测疾病活动,没有普遍接受的验证结果指标。除了评估结果指标的临床评分外,成像模式越来越多地用于评估患者,例如高频超声检查(美国),剪切波弹性成像(SWE),磁共振成像(MRI)。然而,目前尚不清楚这些成像方式在监测角膜活动方面的准确性。
    目的:回顾有关影像学检查方法在评估硬伤患者中的作用的文献。
    方法:在本研究中,我们在PubMed/Medline数据库中搜索了从开始到2023年2月发表的文章.
    结果:美国三类共23篇原创文章,弹性成像,包括MRI。
    结论:关于美国,标准,包括增加真皮厚度,增加了皮下组织的回声,真皮回声减少,当使用高频探头(18-20MHz)彩色多普勒超声检查时,是活动性角膜病变的指标。此外,评估SWE的研究,一种定量评估组织硬度的新方法,表现出活动性病变的真皮僵硬度增加。
    结论:研究表明,MRI可以帮助确定疾病的深度,特别是作为一线和后续诊断工具,尤其是在广义和深层的角膜。此外,脑部MRI可能对出现新的或恶化的神经系统症状的局部颅面硬皮病患者有用.
    BACKGROUND: Localized scleroderma, known as morphea, is a connective tissue disorder characterized by inflammation and fibrosis of the skin and the soft tissue. There exist no universally accepted validated outcome measures in order to monitor the disease activity. Besides clinical scores to evaluate outcome measures, imaging modalities are increasingly utilized in assessing patients with morphea, such as high-frequency ultrasonography (US), shear-wave elastography (SWE), and magnetic resonance imaging (MRI). However, the accuracy of these imaging modalities in monitoring morphea activity is not yet clear.
    OBJECTIVE: To review the literature regarding the role of imaging modalities in assessing patients with morphea.
    METHODS: In this study, we searched the PubMed/Medline database for articles published from inception until February 2023.
    RESULTS: A total number of 23 original articles in three categories of US, elastography, and MRI were included.
    CONCLUSIONS: Regarding US, criteria, including increased dermal thickness, increased echogenicity of the subcutaneous tissue, and decreased dermal echogenicity, were indicators of active morphea lesions when using high frequencies probe (18-20 MHz) color Doppler sonography. Moreover, studies evaluating SWE, a novel method to quantitatively assess tissue stiffness, demonstrated increased dermal stiffness in active lesions.
    CONCLUSIONS: Studies showed that MRI can help to determine the depth of disease, particularly as a first-line and follow-up diagnostic tool, especially in generalized and deep morphea. In addition, brain MRI may be useful for patients with localized craniofacial scleroderma experiencing new or worsening neurological symptoms.
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