morphea

硬斑
  • 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: Published Erratum
    [这修正了文章DOI:10.3389/fimmu.2024.1351675。].
    [This corrects the article DOI: 10.3389/fimmu.2024.1351675.].
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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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  • 文章类型: Journal Article
    色素沉着障碍在皮肤病学中提出了重大的诊断挑战,有时反映潜在的血液学状况。这篇综述探讨了血液病中色素沉着不足的临床表现,专注于白癜风,疣,和综合征白化病。白癜风,针对黑素细胞的自身免疫性疾病,涉及遗传多态性和免疫反应之间的相互作用,特别是关于CD8+T细胞和IFN-γ。药源性白癜风,特别是通过免疫检查点抑制剂和小分子靶向抗癌疗法,强调了免疫失调的重要性。Morphea,炎症性皮肤病,可能是血液学受累的信号,如在深层和放疗后的病变中所见。综合征白化病,与影响黑色素产生的各种基因突变有关,常表现为血液学异常。治疗方法侧重于针对特定疾病的免疫途径,当这是不可能的时候,管理症状。了解这些皮肤病表现对于及时诊断和治疗血液病至关重要。
    Hypopigmentation disorders pose significant diagnostic challenges in dermatology, sometimes reflecting underlying hematological conditions. This review explores the clinical presentations related to hypopigmentation in hematological disorders, focusing on vitiligo, morphea, and syndromic albinism. Vitiligo, an autoimmune disorder targeting melanocytes, involves interactions between genetic polymorphisms and immune responses, particularly regarding CD8+ T cells and IFN-γ. Drug-induced vitiligo, notably by immune checkpoint inhibitors and small-molecule targeted anticancer therapies, underscores the importance of immune dysregulation. Morphea, an inflammatory skin disorder, may signal hematological involvement, as seen in deep morphea and post-radiotherapy lesions. Syndromic albinism, linked to various genetic mutations affecting melanin production, often presents with hematologic abnormalities. Treatment approaches focus on targeting the immune pathways specific to the condition, and when that is not possible, managing symptoms. Understanding these dermatological manifestations is crucial for the timely diagnosis and management of hematological disorders.
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  • 文章类型: Journal Article
    背景和客观Morphea,或局限性硬皮病(LS),是一种以炎症和硬化为特征的自身免疫性皮肤病。其潜在原因包括感染,遗传易感性,和创伤。这种疾病涉及炎症和纤维化的循环,导致皮肤硬化和疤痕,如果不治疗会导致畸形。探索角膜与类风湿因子(RF)之间联系的研究,与其他自身免疫疾病相关的标志物,正在进行中。这项研究旨在检查射频的作用,通常与类风湿关节炎(RA)相关的标志物,在病情严重的情况下。它专注于评估角膜患者的RF水平及其与疾病严重程度的相关性,打算对病情及其管理提供更深入的见解。方法本研究采用简单的随机横断面分析,以评估RF在2022年10月至2023年12月Al-Sader教学医院皮肤性病科患者中测量硬伤严重程度的作用。我们纳入了临床和实验室确诊的硬伤患者,而排除了其他自身免疫性皮肤病患者,最近的全身性类固醇或免疫抑制治疗,和孕妇。通过使用局部硬皮病皮肤评估工具(LoSCAT)进行疾病严重程度的评估。使用SPSSStatistics版本27(IBMCorp.,Armonk,NY),显著性阈值为p<0.05。结果RF水平升高与病情严重程度增加显著相关,与中度(25.83U/mL)和轻度病例(21.56U/mL)相比,重度病例显示更高的RF水平(平均:30.34U/mL)(p=0.028)。然而,RF水平与人口统计学因素如年龄、性别,或职业。与正常RF水平的患者相比,高RF水平的患者的疾病持续时间更长(平均:57.15年)(25.83年,p=0.020)。在背部的病变分布中观察到显着差异(p=0.002)。Logistic回归分析显示重度硬伤患者RF水平升高的可能性更大[比值比(OR):1.158,p=0.014]。结论本研究丰富了我们对RF在硬皮病中作用的认识,与人口统计学因素没有显着相关性,但表明其在疾病慢性和严重程度中的潜在作用。
    Background and objective Morphea, or localized scleroderma (LS), is an autoimmune skin disorder characterized by inflammation and sclerosis. Its potential causes include infections, genetic predisposition, and trauma. The disease involves cycles of inflammation and fibrosis, leading to skin hardening and scarring, which can cause deformities if untreated. Research exploring the link between morphea and rheumatoid factor (RF), a marker associated with other autoimmune conditions, is ongoing. This study aimed to examine the less-explored role of RF, a marker typically linked to rheumatoid arthritis (RA), in the severity of morphea. It focused on assessing the levels of RF among morphea patients and its correlation with disease severity, intending to provide deeper insights into the condition and its management. Methods This study involved a simple randomized cross-sectional analysis to evaluate the role of the RF in measuring morphea severity among patients at the Dermatology and Venereology Department of Al-Sader Teaching Hospital from October 2022 to December 2023. We included participants with clinically and laboratory-confirmed morphea while excluding those with other autoimmune dermatological diseases, recent systemic steroid or immunosuppressive therapy, and pregnant women. The assessment of disease severity was done by utilizing the Localized Scleroderma Cutaneous Assessment Tool (LoSCAT). Statistical analyses were performed using SPSS Statistics version 27 (IBM Corp., Armonk, NY), with a significance threshold of p<0.05. Results Elevated RF levels were significantly associated with increased morphea severity, with severe cases showing higher RF levels (mean: 30.34 U/mL) compared to moderate (25.83 U/mL) and mild cases (21.56 U/mL) (p = 0.028). However, no significant correlation was found between RF levels and demographic factors such as age, gender, or occupation. Patients with high RF levels had a longer disease duration (mean: 57.15 years) compared to those with normal levels (25.83 years, p = 0.020). Significant differences were observed in lesion distribution on the back (p = 0.002). Logistic regression indicated that severe morphea patients were more likely to have elevated RF levels [odds ratio (OR): 1.158, p = 0.014]. Conclusions This study enriches our understanding of RF\'s role in morphea, revealing no significant correlation with demographic factors but suggesting its potential role in disease chronicity and severity.
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  • 文章类型: Journal Article
    通过超声和局部硬皮病皮肤评估工具评估富血小板血浆恢复硬皮皮变化的功效和安全性。
    通过临床和组织病理学诊断了9例(21个病变)。皮内富含血小板的血浆每周一次注射到角膜病变中,共12个疗程。在基线时评估疾病严重程度和损伤,在上次会议之后(3个月后),并在6个月时使用LoSCAT和高分辨率超声进行随访。健康的相应侧被认为是对照。
    治疗终点后,局部硬皮病皮肤评估工具评分从13±7.28到7.33±6.82有显著改善,随访6个月后达到6.44±7.09,p值分别为0.008和0.014。病变的持续时间与超声评估的改善之间存在显着正相关,p值=0.01。关于不利影响,所有患者报告在注射富含血小板的血浆时都有疼痛;4名患者(45%)报告了短暂的面部水肿,只有两名患者出现一过性红斑。
    自体富血小板血浆是一种安全的技术,具有很好的美学效果,可以填补轮廓缺陷并纠正色素沉着和色素沉着不足,除了软化硬化的病变。
    UNASSIGNED: To evaluate the efficacy and safety of platelet-rich plasma to restore skin changes in morphea by ultrasound and Localized Scleroderma Cutaneous Assessment Tool.
    UNASSIGNED: Nine morphea patients (21 lesions) were diagnosed clinically and by histopathology. Intradermal platelet-rich plasma was injected into morphea lesion once weekly for 12 sessions. The disease severity and damage were evaluated at baseline, after the last session (3 months later), and at 6 months follow-up using the LoSCAT and a high-resolution ultrasound. The healthy corresponding side was considered as a control.
    UNASSIGNED: The Localized Scleroderma Cutaneous Assessment Tool score showed a significant improvement starting from 13 ± 7.28 up to 7.33 ± 6.82 after the therapeutic endpoint, reaching to 6.44 ± 7.09 after 6 months of follow-up with p value = 0.008 and 0.014, respectively. There was a significant positive correlation between the duration of the lesion and the improvement assessed by the ultrasound, with p value = 0.01. Regarding adverse effects, all patients reported having pain during platelet-rich plasma injection; transient edema of the face was reported by four patients (45%), and only two patients showed transient erythema.
    UNASSIGNED: Autologous platelet-rich plasma is a safe technique with great aesthetic outcomes for filling up the contour defects and correcting both hyper and hypopigmentation, in addition to softening the indurated lesions.
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  • 文章类型: Journal Article
    系统性硬化症是一种系统性结缔组织疾病,其主要病理生理机制是内脏器官和皮肤进行性纤维化,导致增厚和硬结。也可能涉及血管。然而,系统性硬皮病不是引起皮肤硬化的唯一疾病。有一组在临床表现上模仿硬皮病的疾病-这些是硬皮病样综合征。可以区分炎症/自身免疫综合征,遗传,新陈代谢,有毒,药物诱导,职业,沉积障碍引起的副肿瘤和综合征。在下面的论文中,我们回顾了有关硬皮病样综合征的文献。我们已经概述了导致每种疾病发展的因素,其发病机制,临床表现,诊断和治疗过程及各证型与系统性硬皮病的差异。
    Systemic sclerosis is a systemic connective tissue disease whose main pathophysiological mechanism is a progressive fibrosis of internal organs and skin leading to thickening and induration. Blood vessels may also be involved. However, systemic scleroderma is not the only disease causing cutaneous sclerosis. There is a group of diseases that mimic scleroderma in their clinical presentation - these are scleroderma-like syndromes. A distinction can be made between syndromes of inflammatory/autoimmune, genetic, metabolic, toxic, drug-induced, occupational, paraneoplastic and syndromes caused by deposition disorders. In the following paper, we have reviewed the literature on scleroderma-like syndromes. We have outlined the factors predisposing to the development of each disease, its pathogenesis, clinical presentation, diagnostic and treatment process and the differences between each syndrome and systemic scleroderma.
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  • 文章类型: Letter
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