morphea

硬斑
  • 文章类型: Case Reports
    近年来,自体脂肪移植在局限性硬皮病(LoS)患者面部病变中的应用已有报道。
    作者报告了一例自体脂肪移植后活动性局部硬皮病恶化的病例。
    一名男子表现为颈部和面部皮肤萎缩和色素沉着,有LoS病史。出现在1.5年前,病变的大小和形状逐渐增大。在患者被告知疾病活动期可能的手术风险后,获得了同意。他接受了自体脂肪移植到右脸颊,大约30毫升科尔曼脂肪移植。
    治疗1个月后,皮肤色素沉着和萎缩逐渐恶化,伴有轻微红斑增加和病灶扩大。治疗6个月后,局部硬皮病相关评分恶化。
    有不同的因素,如全身用药可影响自体脂肪移植对局部硬皮病的治疗。同时,考虑到6个月随访期的限制,获得长期随访数据对于评估持续结局和潜在并发症是必要的.
    需要更多的临床研究来确定疾病不活动与应用任何外科手术之间的时间间隔,以避免重新激活。
    UNASSIGNED: The application of autologous fat transplantation in facial lesions of patients with localized scleroderma (LoS) has been reported in recent years.
    UNASSIGNED: The authors report a case of worsening of active localized scleroderma after autologous fat transplantation.
    UNASSIGNED: A man presented with neck and facial skin atrophy and pigmentation with a history of LoS. Appearing 1.5 years ago, the lesion had progressively grown in size and shape. Consent was obtained after the patient was informed of the possible surgical risks during the active phase of the disease. He underwent autologous fat grafting into the right cheek with about 30 ml Coleman fat graft.
    UNASSIGNED: Skin dyspigmentation and atrophy progressively deteriorated 1 month into therapy, with slightly increased erythema and enlargement of the lesion. Six months after the therapy, the localized scleroderma-related score worsened.
    UNASSIGNED: There are different factors, such as that systemic medications could affect the treatment of localized scleroderma by autologous fat transplantation. Meanwhile, considering the limitation of the 6-month follow-up period, obtaining long-term follow-up data is necessary to evaluate sustained outcomes and potential complications.
    UNASSIGNED: More clinical research is needed to determine the time interval between disease inactivity and the application of any surgical procedures to avoid reactivation.
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  • 文章类型: Case Reports
    Morphea,是真皮和皮下组织的慢性炎症性疾病。研究表明,硬皮病与I型糖尿病(T1D)之间存在联系。COVID-19可引起自身免疫性疾病,如硬皮病,T1D,系统性红斑狼疮,和其他人。一名正在接受胰岛素治疗的12岁1型糖尿病女孩被带到诊所进行代谢评估。病人有硬结,皮肤硬度,和皮肤红斑检查。T1D的发作是在轻度COVID-19感染后发生的。在T1D发作后3个月合并了硬皮病征。被称为“长期COVID,“COVID-19急性期之后的这个疾病阶段很可能是自身免疫激活的结果。正如接受评估的患者所揭示的,文献中已证明COVID-19会导致自身抗体的产生,并导致或恶化具有遗传易感性的人的自身免疫性疾病。
    Morphea, is a chronic inflammatory disease of the dermis and subcutaneous tissue. Research has indicated a connection between morphea and Type I Diabetes (T1D). COVID-19 can cause autoimmune diseases like scleroderma, T1D, systemic lupus erythematosus, and others. A 12-year-old girl with type 1 diabetes who was on insulin therapy was brought into the clinic for a metabolic evaluation. The patient had induration, skin hardness, and cutaneous erythema upon inspection. The onset of T1D was following a mild COVID-19 infection. Signs of morphea merged 3 months after the onset of T1D. Known as \"long-term COVID,\" this sickness phase that follows the acute stage of COVID-19 is most likely the result of autoimmune activation. As this patient under evaluation reveals, COVID-19 has been demonstrated in the literature to cause the production of autoantibodies and to either cause or worsen autoimmune disorders in people who have a genetic susceptibility.
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  • 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: Case Reports
    Morphea,一种局部硬皮病,可以通过引起皮肤收紧和变色来显著影响个体。我们描述了一名22岁女性的病例,该女性在右臀区肌肉注射史后,右臀区出现进行性皮肤变化和不适。临床检查提示为硬伤,促使我们进行核磁共振,以更好地了解她的病情的程度和性质。MRI结果显示皮肤层增厚和炎症迹象,帮助我们区分活动性炎症和纤维化.这个案例说明了MRI如何为有效管理角膜提供重要的见解。
    Morphea, a form of localized scleroderma, can significantly affect individuals by causing skin tightening and discoloration. We describe the case of a 22-year-old woman who presented with progressive skin changes and discomfort in her right gluteal region following a history of an intramuscular injection in the right gluteal region. Clinical examination suggested morphea, prompting us to conduct an MRI to better understand the extent and nature of her condition. The MRI results revealed thickening of the skin layers and signs of inflammation, helping us differentiate between active inflammation and fibrosis. This case illustrates how MRI can provide crucial insights for managing morphea effectively.
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  • 文章类型: Case Reports
    Parry-Romberg综合征是一种罕见的神经皮肤疾病,其特征是进行性半面部萎缩。我们提出一个14岁的案例,一个已知的线性角膜病病例,出现癫痫发作并经评估被诊断为Parry-Romberg综合征。它会对审美幸福感产生深远的影响,并具有显着的社会心理发病率。本病例报告旨在强调涉及风湿病学家的有效多学科团队方法,皮肤科医生,神经科医生,和眼科医生,最终在我们的病人的疾病的细致管理。
    Parry-Romberg syndrome is a rare neurocutaneous disease characterized by progressive hemifacial atrophy. We present the case of a 14-year-old, a known case of linear morphea, who presented with seizure and on evaluation was diagnosed with Parry-Romberg syndrome. It causes a profound impact on aesthetic well-being and has a significant psychosocial morbidity. This case report aims to highlight the effective multidisciplinary team approach involving a rheumatologist, dermatologist, neurologist, and ophthalmologist which ultimately culminated in the meticulous management of the disease in our patient.
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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: Case Reports
    线形硬伤是硬伤最致残的亚型,这可能会导致一系列的异常表现和后遗症。为进一步探讨线形硬伤的临床特点,我们对过去2年来在我们科室诊断为线性角膜病的22例患者进行了回顾性研究.他们的基线临床信息,皮肤表现,对并发症及治疗效果进行分析。这里,我们报告了6例特殊的线形性角膜病,通常发生在年轻女性的单侧上肢,沿着桡神经的分布传播,并经常在关节上传播,这增加了神经肌肉骨骼疾病的发病率。而不是传统的外用药物,全身泼尼松和甲氨蝶呤联合治疗可改善其皮肤损伤和并发症.识别这种特殊类型的线性角膜能够早期诊断和积极的治疗计划,这有助于改善症状并避免功能性后遗症。
    Linear morphea is the most disabling subtype of morphea, which may cause a series of excutaneous manifestations and sequelae. To futher explore the clinical characteristics of linear morphea, we conducted a retrospective study of 22 patients diagnosed with linear morphea in our department during the past 2 years. Their baseline clinical information, skin manifestations, complications and therapeutic effect were analyzed. Here, we report six cases of a special linear morphea, usually occurring on the unilateral upper limbs of young women, spreading along the distribution of the radial nerve and frequently progressing across the joint, which increases the incidence of neuromusculoskeletal disorders. Instead of traditional topical drugs, a combination of systemic prednisone and methotrexate improved their skin lesions and complications. Recognition of this special type of linear morphea enables earlier diagnosis and active treatment plan, which contributes to ameliorate the symptoms and avoid functional sequelae.
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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: 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: Journal Article
    背景:迄今为止,没有公认的结果监测措施,关于硬伤的具体监测标准的共识仍然难以捉摸。一些研究已经评估了皮肤超声在角膜中的标准有效性。所以,在这项研究中,我们接近的超声检查结果在角膜病变。
    方法:这是一项在2021年12月至2023年5月之间进行的回顾性分析研究。在皮肤科门诊对患者进行临床评估,然后进行高频超声(HF-US)评估,并选择纳入本研究。病变也通过组织病理学证实。在病变部位和对称未受累的另一侧进行超声检查评估。记录真皮厚度和真皮回声。通过使用双尾学生t检验进行组差异的统计学分析。小于0.05的P值被认为是统计学上显著的。
    结果:研究中纳入了27例患者的41例炎症期的角膜病变。角膜病变的平均真皮厚度为1107.97±414.3,对照侧的平均真皮厚度为1094.65±331.06,这两个变量之间的差异无统计学意义。病变的平均真皮密度为49.13±18.97,对照侧的平均真皮密度为52.22±25.33。这两个变量之间的差异没有统计学意义。
    结论:这项研究表明,HF-US表明在组织病理学证实的炎症阶段,角膜病变的真皮厚度增加,真皮密度降低。
    BACKGROUND: To date, there are no accepted outcome measures to monitor morphea, and consensus on specific monitoring criteria for morphea remains elusive. A few studies have assessed the criterion validity of skin ultrasound in morphea. So, in this study, we approach ultrasound findings in morphea lesions.
    METHODS: This was a retrospective-analytical study conducted between December 2021 and May 2023. Patients were clinically evaluated at a dermatology outpatient clinic and then referred for high-frequency ultrasound (HF-US) evaluation and were selected to be included in this study. The lesions were confirmed by histopathology as well. Sonographic evaluations were performed on the lesion site and the symmetrical uninvolved other side. Dermal thickness and dermal echogenicities were recorded. Statistical analysis of group differences was performed by using the 2-tailed Student t-test. A p-value of less than 0.05 was considered statistically significant.
    RESULTS: Forty-one morphea lesions in the inflammatory phase of 27 patients were included in the study. The mean dermal thickness of morphea lesions was 1107.97 ± 414.3 and the mean dermal thickness of the control side was 1094.65 ± 331.06, The difference between these two variables was not statistically significant. The mean dermal density of lesions was 49.13 ± 18.97 and the mean dermal density of the control side was 52.22 ± 25.33. The difference between these two variables was not statistically significant.
    CONCLUSIONS: This study shows that HF-US indicated increasing dermal thickness and reducing the dermal density of the morphea lesions in the inflammatory phase confirmed with the histopathology.
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