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
    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: 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:皮肤僵硬综合征(SSS)是一种罕见的疾病,其特征是皮肤硬化。已经报道了广泛和节段性SSS的病例。
    目的:报告一系列SSS的临床和组织病理学特征。
    方法:我们回顾性分析了从皮肤科收集的广泛和节段性SSS的临床和组织病理学特征。我们还比较了节段性SSS和硬皮之间的组织病理学。
    结果:31例,包括三个广泛的SSS和28个分段SSS,被收集。广泛的SSS的皮肤病变通常表现为集中在腰部的皮肤硬化,臀部,大腿,四肢近端,和肩膀有特定的异常步态和姿势。节段性SSS的皮肤病变通常表现为累及大腿的硬化斑块,腰部和臀部,与多毛症有关,色素沉着和鹅卵石外观。节段性SSS通常不会引起关节受限或严重的身体不适。组织病理学,SSS显示真皮或皮下组织中的成纤维细胞增殖和胶原硬化。与硬皮相比,SSS显示出更明显的成纤维细胞增殖,并且完全没有淋巴细胞浸润。
    结论:分段SSS代表SSS的主要变体。组织病理学,SSS显示成纤维细胞的增殖,硬化和没有炎症。
    BACKGROUND: Stiff skin syndrome (SSS) is a rare disease characterized by sclerosis of the skin. Cases of both widespread and segmental SSS have been reported.
    OBJECTIVE: To report the clinical and histopathological characteristics of a large series of SSS.
    METHODS: We retrospectively analysed the clinical and histopathological characteristics of widespread and segmental SSS collected from a dermatology department. We also compared histopathology between segmental SSS and morphea.
    RESULTS: Thirty-one cases, including three widespread SSS and 28 segmental SSS, were collected. Skin lesions of widespread SSS generally showed skin sclerosis concentrating over the lumbar, buttocks, thighs, proximal part of limbs, and shoulders with specific abnormal gait and posture. Skin lesions of segmental SSS generally showed sclerotic plaques involving the thigh, lumbar area and buttocks, associated with hypertrichosis, hyperpigmentation and a cobblestone appearance. Segmental SSS did not typically cause joint limitation or serious physical discomfort. Histopathologically, SSS showed proliferation of fibroblasts and sclerosis of collagen in the dermis or subcutaneous tissue. Compared with morphea, SSS showed more prominent proliferation of fibroblasts and completely lacked lymphocyte infiltration.
    CONCLUSIONS: Segmental SSS represents the major variant of SSS. Histopathologically, SSS shows proliferation of fibroblasts, sclerosis and an absence of inflammation.
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  • 文章类型: Journal Article
    目的:评价部分铒:钇铝石榴石(Er:YAG)激光治疗小鼠模型的效率和机制。
    背景:Morphea是一种罕见的自身免疫性疾病,其特征是皮肤中胶原蛋白过度沉积。分数Er:YAG激光治疗是一种很有前途的治疗方法,尽管关于治疗效果和潜在机制的研究有限。
    方法:通过皮下注射博来霉素(BLM)建立小鼠角膜模型。总共24只小鼠接受分级Er:YAG激光治疗,每周一次,持续4周。采用的客观测量方法是超声成像测量真皮厚度。主观测量包括根据调整后的局部角膜皮肤评估工具(LoSCAT)进行评分;苏木精和伊红(H&E)染色以评估纤维化的组织学等级;定量形态计量学研究,以通过免疫组织化学确定转化生长因子-β1(TGF-β1)和基质金属蛋白酶-1(MMP1)的表达。
    结果:在这项自我对照研究中,分数Er:YAG激光治疗可显着改善硬伤的严重程度,包括较低的临床评分(p<0.01),真皮厚度降低(p<0.001),纤维化组织学分级下降(p<0.001),MMP1增加(p<0.001),TGF-β1表达降低(p<0.01)。
    结论:我们发现分数阶Er:YAG激光治疗角膜有良好的临床效果,超声波,和组织病理学疗效,这可能是未来有希望的治疗方法。
    OBJECTIVE: To assess the efficiency and the mechanism of fractional erbium:yttrium aluminum garnet (Er:YAG) laser for the treatment of morphea in mouse model.
    BACKGROUND: Morphea is a rare autoimmune disease characterized by excessive collagen deposition in skin. Fractional Er:YAG laser treatment is a promising treatment to improve morphea, despite limited studies about the therapeutic effect and underlying mechanism.
    METHODS: The mouse model of morphea was established by subcutaneously injecting with bleomycin (BLM). A total of 24 mice received fractional Er:YAG laser treatment once a week for 4 weeks. Objective measurement employed was ultrasonic imaging to measure dermal thickness. Subjective measures included scoring according to the adjusted Localized morphea Cutaneous Assessment Tool (LoSCAT); hematoxylin and eosin (H&E) staining to evaluate the histological grade of fibrosis; and quantitative morphometric studies to determine the expression of transforming growth factor-β1 (TGF-β1) and matrix metalloproteinase-1 (MMP1) by immunohistochemistry.
    RESULTS: In this self-controlled study, fractional Er:YAG laser treatment significantly ameliorate the severity of morphea, including lower clinical score (p < 0.01), decreased dermal thickness (p < 0.001), declined histological grade of fibrosis (p < 0.001), increased MMP1 (p < 0.001), and reduced TGF-β1 (p < 0.01) expression.
    CONCLUSIONS: We found that fractional Er:YAG laser treatment of morphea has good clinical, ultrasonic, and histopathologic efficacy, which may be a promising treatment in the future.
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  • 文章类型: Journal Article
    本文介绍了一种基于多光子显微镜(MPM)检查的方法,该方法由双光子激发荧光(TPEF)和二次谐波产生(SHG)组成。通过MPM从未染色的病变组织获取亚细胞分辨率图像,然后进行光谱分析以量化TPEF和SHG信号。此外,使用U-Net对TPEF图像中的弹性纤维进行分割,以与SHG图像中的胶原纤维结合以进行精确的纤维定量。分割预测在几个评价指标上表现优异。mIoU,mPA,F1评分分别达到0.8516、0.9281和0.941。定量分析表明,与硬化性苔藓病例相比,硬体中胶原纤维的增加。同时,根据MPM成像描绘了硬化性苔藓真皮中弹性纤维的大量减少。因此,MPM与组织病理学检查相当,我们的实验结果准确区分了硬皮和苔藓硬化。
    This paper describes a methodology to differentiate morphea from lichen sclerosus based on examination with multiphoton microscopy (MPM) composed of two-photon excited fluorescence (TPEF) and second harmonic generation (SHG). Subcellular-resolution images were acquired by MPM from unstained lesion tissues then process spectral analysis to quantify the TPEF and SHG signals. Moreover, U-Net was employed to segment elastic fiber in TPEF images to combine with collagen fiber in SHG images for precise fiber quantification. Predictions of segmentation showed excellent performance on several evaluation indicators. The mIoU, mPA, and F1 score reach 0.8516, 0.9281, and 0.941. The quantitative analysis demonstrated the increase of collagen fibers in morphea compared to that in lichen sclerosus cases. Meanwhile, the great diminution of elastic fiber in the dermis of lichen sclerosus was depicted based on MPM imaging. Thus, MPM was comparable to the histopathological examination and our experimental results accurately distinguish between morphea and lichen sclerosus.
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  • 文章类型: Journal Article
    背景:局限性硬皮病(LS)的治疗和疗效评估仍然困扰着许多临床工作者。
    目的:通过超声检查评价甲氨蝶呤治疗LS的疗效。
    方法:一项前瞻性研究纳入了10例接受MTX治疗至少6个月的患者。通过临床评分和15-MHz超声检查评估治疗结果。安全性评估包括药物不良反应监测和临床实验室检查。
    结果:10例患者中有8例仅使用MTX实现临床缓解。1例患者经MTX联合糖皮质激素后缓解,而另一种在霉酚酸酯和皮质类固醇治疗后没有改善。MTX的有效率为80%。9例患者随着局部硬皮病皮肤评估工具的减少而得到显着改善(LoSCAT皮肤活动的平均评分从5.2降至1.0,p<0.001,LS皮肤损伤的平均评分从4.3降至2.3,p=0.002)。在8例患者中,通过超声评估的皮肤病变与正常皮肤之间的厚度平均差异从0.13cm降低到0.04cm(p=0.009)。无严重不良反应发生。
    结论:甲氨蝶呤是治疗LS患者安全有效的药物。超声检查可以被认为是评估LS的有效评估工具。
    BACKGROUND: The treatment and curative effect evaluation of localized scleroderma (LS) still perplexes many clinical workers.
    OBJECTIVE: To investigate the efficiacy of methotrexate in the treatment of LS by the evaluation of ultrasonography.
    METHODS: A prospective study enrolled 10 patients treated with MTX for at least 6 months was conducted. Treatment outcome was evaluated by a clinical score and 15-MHz ultrasonography. Safety assessment included the monitoring of adverse drug reactions and clinical laboratory examinations.
    RESULTS: Eight of the 10 patients achieved clinical remission only with MTX. One patient was relieved after MTX combined with corticosteroids, while another one does not improve after the treatment of mycophenolate mofetil and corticosteroids. The effective rate of MTX is 80%. Nine patients were significantly improved with a decrease of the Localized Scleroderma Cutaneous Assessment Tool (the mean score of the LoSCAT cutaneous activity dropped from 5.2 to 1.0, p < 0.001, the mean score of the LS cutaneous damage dropped from 4.3 to 2.3, p = 0.002). The average difference of thickness between skin lesions and normal skin evaluated by ultrasonography decreased from 0.13 cm to 0.04 cm (p = 0.009) in eight patients. No serious adverse reactions occurred.
    CONCLUSIONS: Methotrexate is a safe and effective treatment for patients with LS. Ultrasonography can be considered as an efficient assessment tool for evaluation LS.
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  • 文章类型: Journal Article
    Morphea是一种以皮肤硬化为特征的自身免疫性疾病。根据疾病进展情况,硬伤可分为炎症,硬化,和萎缩性阶段。皮肤镜检查和高频超声(HF-US)已应用于许多炎症性疾病的无创评估。但是对不同阶段的角膜皮肤影像学特征的研究还很有限。分析不同病期的皮肤镜和HF-US特征,探讨其在该病分期中的辅助价值。我们随访了2018年4月至2021年7月间经组织病理学证实的34例患者,这些患者接受了皮肤镜检查以及50和20MHzHF-US.Fisher精确检验用于评估不同病期患者的皮肤镜和HF-US特征的差异。7名患者被归类为炎症阶段,20为硬化阶段,七个为萎缩期。炎性病变最常见的皮肤镜特征是红色无结构区域(100%)和线性弯曲血管(85.7%)。在100%和90%的硬化病变中可以看到白云和闪亮的白色条纹,分别。在萎缩性病变中,色素结构(100%)和红色无结构区域(85.7%)是主要特征。在HF-US检查中,炎性病变显示附件周围的低回声性(85.7%),低回声真皮(71.4%),真皮和皮下脂肪之间的边界不清楚(71.4%)。在硬化期的病变中,主要的HF-US特征包括高回声真皮(85.0%),真皮的声衰减(70.0%),真皮和皮下脂肪之间的边界不清楚(85.0%)。所有萎缩性病变均表现为高回声真皮,28.6%显示真皮和皮下脂肪之间的边界不清楚。皮肤镜检查和HF-US可以揭示不同阶段的特征,并与组织病理学表现出良好的对应关系。皮肤镜检查和HF-US可以为硬伤的分期提供重要信息。
    Morphea is an autoimmune disease characterized by skin sclerosis. According to the disease progression, morphea can be divided into inflammatory, sclerotic, and atrophic stages. Dermoscopy and high-frequency ultrasound (HF-US) have been applied in the noninvasive evaluation of many inflammatory diseases, but studies on the skin imaging features of the different stages of morphea are limited. To analyze the dermoscopic and HF-US features of the different stages of morphea and explore their auxiliary value in staging the disease, we followed 34 patients with histopathology-confirmed morphea between April 2018 and July 2021 who underwent dermoscopy and 50 and 20 MHz HF-US. Fisher\'s exact test was used to assess the differences in dermoscopic and HF-US features among patients with different stages of morphea. Seven patients were classified as the inflammatory stage, 20 as the sclerotic stage, and seven as the atrophic stage by histopathology. The most common dermoscopic features of inflammatory lesions were red structureless areas (100%) and linear curved vessels (85.7%). White clouds and shiny white streaks could be seen in 100% and 90% of sclerotic lesions, respectively. Among atrophic lesions, pigmentary structures (100%) and red structureless areas (85.7%) were the main features. In the HF-US examination, inflammatory lesions showed hypoechogenicity around the appendages (85.7%), a hypoechogenic dermis (71.4%), and an unclear boundary between the dermis and the subcutaneous fat (71.4%). Among lesions of the sclerotic stage, the main HF-US characteristics included a hyperechogenic dermis (85.0%), acoustic attenuation of the dermis (70.0%), and an unclear boundary between the dermis and the subcutaneous fat (85.0%). All atrophic lesions showed a hyperechogenic dermis, and 28.6% showed an unclear boundary between the dermis and the subcutaneous fat. Dermoscopy and HF-US can reveal the characteristic features of the different stages of morphea and show good correspondence with the histopathology. Dermoscopy and HF-US can provide important information for the staging of morphea.
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  • 文章类型: Journal Article
    局限性硬皮病(LS)是一种以皮肤硬化为主要表现的自身免疫性疾病。目前,对于LS没有特定的治疗方法。已在多项研究中证明了消融点阵激光(AFL)治疗LS的有效性。将消融分数Er:YAG激光治疗与局部甲氨蝶呤相结合可能会对LS患者产生治疗益处。比较AFL辅助给药甲氨蝶呤在成人LS患者中的疗效和安全性。我们将患者随机分为AFL治疗组和甲氨蝶呤剥蚀点阵激光辅助给药(AFL+MTX)治疗组.激光和辅助给药治疗每4周一次,为期4个月,22名患者完成了试验。使用超声测量真皮厚度和组织学纤维化程度以及局部硬皮病皮肤评估工具(LoSCAT)评分来评估治疗效果。治疗结果表明,AFL和AFL辅助甲氨蝶呤给药治疗LS均有效,激光联合甲氨蝶呤治疗在改善临床表现(p值=0.042)和皮肤厚度(p值=0.016)方面更有效。两组均未发生严重不良反应。总之,AFL和甲氨蝶呤的辅助给药是LS的有效和安全的治疗方法。
    Localized scleroderma (LS) is an autoimmune disease with sclerosis of the skin as the main manifestation. Currently, there is no specific treatment for LS. The effectiveness of ablative fractional laser (AFL) therapy for LS has been demonstrated in several studies. Combining ablative fractional Er:YAG laser therapy with topical methotrexate may yield therapeutic benefits for patients with LS. To compare the efficacy and safety of AFL-assisted delivery of methotrexate in adults with LS, we randomly divided patients into an AFL therapy group and an ablative fractional laser-assisted delivery of methotrexate (AFL+MTX) therapy group. Laser and assisted drug delivery treatment were given every four weeks for four months, and 22 patients completed the trial. Ultrasound measurements of dermal thickness and histological fibrosis degree and the Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) score were used to assess therapeutic effects. Treatment results showed that both AFL and AFL-assisted methotrexate delivery were effective in treating LS, and the laser combined with methotrexate therapy was more effective in improving clinical appearance (p value = 0.042) and dermal thickness (p value = 0.016). No serious adverse reaction occurred in either group. In conclusion, AFL and assisted delivery of methotrexate are effective and safe treatments for LS.
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  • 文章类型: Journal Article
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