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: 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: 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
    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
    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: Case Reports
    嗜酸性筋膜炎可能是一种使人衰弱的诊断,并且由于其与其他硬化性疾病(包括硬皮病)的相似性,通常会延迟。如在组织病理学上的结合-下硬化的皮肤和组织层的炎症和硬化增厚。延迟治疗可导致关节挛缩和皮肤残余硬度,具有美容和功能意义。因此,在病程早期发现明确的诊断和与其他硬化性疾病的鉴别具有重要意义。我们介绍了一个77岁的女性,她的背部和四肢出现了全身性皮疹,和进行性疼痛症状,关节挛缩,和有限的运动,鉴于嗜酸细胞性筋膜炎和硬皮病之间的临床和组织学相似之处,这突出了诊断和治疗方面的挑战。
    Eosinophilic fasciitis can be a debilitating diagnosis and is often delayed given its similarities to other sclerotic conditions including morphea, such as bound-down indurated skin and inflammation and sclerotic thickening of tissue layers on histopathology. Delaying treatment can lead to joint contracture and residual hardness in skin which has both cosmetic and functional implications. Therefore, finding the definitive diagnosis and differentiating from other sclerotic diseases is important early in the disease course. We present a case of a 77-year-old female with a generalized rash on her back and extremities, and progressive symptoms of pain, joint contractures, and limited movement, which highlights the challenges in diagnosis and management given clinical and histological parallels between eosinophilic fasciitis and morphea.
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  • 文章类型: Case Reports
    背景:肿瘤坏死因子α(TNFα)是参与某些炎性疾病发病的关键细胞因子,如类风湿性关节炎(RA),脊椎关节病,和炎症性肠病。在过去的二十年里,TNFα抑制剂(TNFi)彻底改变了上述疾病的治疗和结果。然而,TNFi的使用与许多自身免疫现象和矛盾的皮肤表现的发展相关,这些症状可能与TNFi有效使用的临床适应症类型相同.因此,它们可能表现为关节炎,葡萄膜炎,结肠炎,牛皮癣,和其他一些皮肤临床表现,其中包括湿疣的发展,局限性硬皮病皮肤病变。
    方法:我们描述了一名58岁的女性,患有血清阴性RA,甲氨蝶呤难治,他接受了ABP-501(Hefiya)治疗,阿达木单抗(ADA)生物仿制药发展成椭圆形,约3.5cm大小的深层皮肤损伤,在开始治疗3个月后,影响她背部的左侧部分与角膜相容。ADA生物仿制药停产,两个月后,她的皮肤有了很大的改善.
    结论:这是在TNFi生物仿制药期间首次报道的硬伤表现,因为患者没有其他硬伤发展的触发因素,如创伤和感染。治疗使用TNFi生物仿制药的患者的医生应该意识到矛盾的皮肤反应,在他们当中,密切监测,一分钟和仔细的临床检查,和后续检查是必需的。
    BACKGROUND: Tumor necrosis factor alpha (TNFα) is a pivotal cytokine involved in the pathogenesis of certain inflammatory diseases, such as rheumatoid arthritis (RA), spondyloarthropathies, and inflammatory bowel diseases. In the last two decades, TNFα inhibitors (TNFi) have revolutionized the treatment and outcome of the above disorders. However, the use of TNFi has been associated with the development of many autoimmune phenomena and paradoxical skin manifestations that may present as the same type of clinical indications for which the TNFi effectively used. Thus, they may display as arthritis, uveitis, colitis, psoriasis, and several other cutaneous clinical manifestations, among them the development of morphea, a localized scleroderma skin lesion.
    METHODS: We describe a 58-year-old woman with seronegative RA, refractory to methotrexate, who was treated with ABP-501 (Hefiya), an adalimumab (ADA) biosimilar and developed an oval-shaped, deep skin lesion of approximately 3.5cm in size, affecting the left part of her back compatible with morphea 3 months after the initiation of therapy. ADA biosimilar was discontinued and two months later, she had substantial skin improvement.
    CONCLUSIONS: This is the first report of morphea manifestation during TNFi biosimilar since the patient had no other trigger factors for morphea development like trauma and infections. Physicians dealing with patients treated with TNFi biosimilars should be aware of paradoxical skin reactions, among them morphea; thus, close monitoring, a minute and careful clinical examination, and a follow- up check are required.
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  • 文章类型: Case Reports
    硬皮病是一种罕见的疾病,影响结缔组织,导致皮肤硬化,有时导致器官损伤。硬皮病有两种主要形式:局部硬皮病,或者是硬皮,通常有一个温和和有限的过程,只影响皮肤和/或下面的组织,和系统性硬化症,涉及皮肤硬化和内脏器官问题。局部硬皮病的病因未知。最近的研究表明,这种形式可能具有不同的严重程度,并可能影响某些器官。为了避免由于局部硬皮病的高发病率引起的并发症,建议早期治疗。在这篇文章中,我们介绍了局部硬皮病患者治疗的主要方面和细节。
    Scleroderma is an uncommon disease that affects the connective tissue, causing skin hardening and sometimes organ damage. There are two main forms of scleroderma: localised scleroderma, or morphea, which usually has a mild and limited course and only affects the skin and/or the tissues below it, and systemic sclerosis, which involves skin hardening and internal organ problems. The cause of localised scleroderma is unknown. Recent studies suggest that this form can have different levels of severity and can affect some organs. To avoid complications due to the high morbidity of localised scleroderma, early treatment is recommended. In this article, we present the main aspects and details of the management of patients with localised scleroderma.
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  • 文章类型: Case Reports
    硬化性苔藓(LSA)是一种病因不明的炎症性皮肤病,通常影响生殖器区域,外性参与并不常见。在同一病变中,LSA和硬脊膜的共存很少见。本研究旨在证明LSA和硬体可能具有相似的病理过程。我们介绍了一例53岁的女性患者,其生殖器外病变的临床表现和组织病理学特征均为LSA和硬伤。最后,这两种疾病可能位于同一疾病谱上。
    Lichen sclerosus et atrophicus (LSA) is an inflammatory dermatosis of unknown etiology, usually affecting the genital region, with extragenital involvement being uncommon. The coexistence of LSA and morphea in the same lesion is rare. The present study aims to demonstrate that LSA and morphea might share similar pathologic processes. We present a case of a 53-year-old female patient with extragenital lesions with clinical appearance and histopathological features of both LSA and morphea. Finally, the two diseases might lie on the same disease spectrum.
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