Necrobiosis Lipoidica

脂类坏死病
  • 文章类型: Case Reports
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  • 文章类型: Multicenter Study
    脂肪坏死病(NL)是一种皮肤病,其特征是肉芽肿性炎症的发展,导致胶原蛋白变性,随后形成黄褐色毛细血管扩张斑块,通常位于中年女性的胫骨前皮肤上。由于其稀有性和病因不明,NL的治疗选择没有很好的标准化。其中,光动力疗法(PDT)是一种新兴的工具,尽管其疗效主要是在单例病例报告或小病例系列中进行评估。这项研究报告了在Messina和Reggio-Emilia大学医院皮肤科接受PDT治疗的NL患者队列的现实生活经历。从2013年到2023年,17名患者被招募-5名男性(29%)和12名女性(71%),年龄在16至56岁之间(平均年龄:42±13岁)。NL的中位持续时间为8年。总体完全清除率(>75%病变减少)为29%,而部分清除率(病变减少25-75%)为59%,12%的人是无应答者。这项研究增加了文献中关于PDT治疗NL有效性的少量证据。患者治疗反应的变异性强调了对个性化方案的需求,优化光敏剂,光源,和剂量测定。治疗方案和共识指南的标准化对于确保研究的可重复性和可比性至关重要。
    Necrobiosis Lipoidica (NL) is a dermatological condition characterized by the development of granulomatous inflammation leading to the degeneration of collagen and subsequent formation of yellowish-brown telangiectatic plaques usually localized on the pretibial skin of middle-aged females. Due to its rarity and unclear etiopathogenesis, therapeutic options for NL are not well-standardized. Among them, photodynamic therapy (PDT) is an emerging tool, although its efficacy has primarily been evaluated in single case reports or small case series. This study reports the real-life experience of a cohort of NL patients treated with PDT at the Section of Dermatology of the University Hospital of Messina and Reggio-Emilia. From 2013 to 2023, 17 patients were enrolled -5 males (29%) and 12 females (71%) aged between 16 and 56 years (mean age: 42 ± 13 years), with a median duration of NL of 8 years. The overall complete clearance (>75% lesion reduction) was 29%, while the partial clearance (25-75% lesion reduction) was 59%, with 12% being non-responders. This study adds to the little amount of evidence present in the literature regarding the effectiveness of PDT in the treatment of NL. Variability in treatment responses among patients underscores the need for personalized protocols, optimizing photosensitizers, light sources, and dosimetry. The standardization of treatment protocols and consensus guidelines are essential to ensure reproducibility and comparability across studies.
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  • 文章类型: Journal Article
    脂肪坏死病(NL)是一种罕见的肉芽肿性疾病,其病因尚未完全了解。经典的,NL与胰岛素依赖型糖尿病有关。该疾病通常对常规治疗无效,并对患者的生活质量产生不利影响。一线药物通常是外用皮质类固醇,但患者对他们的反应不同程度的成功。其他选择包括他克莫司,光疗,环孢菌素,富马酸酯,和生物制剂(阿达木单抗,依那西普,和英夫利昔单抗)。我们的综述旨在为难治性病变患者提供可能有效的新治疗方法。描述假定的病因,并为临床医生提供诊断指导。该综述得出结论,Janus激酶抑制剂和生物制剂如ustekinumab和苏金单抗可以有效地用于顽固性NL患者。另一种有希望的治疗选择是tapinarof(芳基烃受体激动剂)。然而,仍需要对更大的患者组进行研究,以评估不同治疗方案的有效性,并确定一致的NL治疗方案.建议提高各种专科医师对脂类坏死病的认识,因为早期诊断的病变通常对治疗有更好的反应。
    Necrobiosis lipoidica (NL) is a rare granulomatous disease of a not fully understood etiopathogenesis. Classically, NL is associated with insulin-dependent diabetes mellitus. The disease often fails to respond to conventional treatments and adversely affects patients\' quality of life. First-line medications are usually topical corticosteroids, but patients respond to them with varying degrees of success. Other options include tacrolimus, phototherapy, cyclosporine, fumaric acid esters, and biologics (adalimumab, etanercept, and infliximab). Our review aims to present new therapeutic approaches potentially effective in patients with refractory lesions, describe the presumed etiopathogenesis, and provide diagnostic guidance for clinicians. The review concludes that Janus kinase inhibitors and biologics such as ustekinumab and secukinumab can be used effectively in patients with recalcitrant NL. Another promising treatment option is tapinarof (an aryl hydrocarbon receptor agonist). However, studies on larger groups of patients are still needed to evaluate the effectiveness of different therapeutic options and to define consistent treatment regimens for NL. It is advisable to improve the awareness of physicians of various specialties regarding necrobiosis lipoidica as lesions diagnosed earlier usually have a better response to treatment.
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  • 文章类型: Journal Article
    背景:脂类坏死菌病(NL)是一种罕见的肉芽肿性皮肤病,对女性有好感,常伴有糖尿病(DM)。本文旨在全面回顾有关NL的文献,专注于它与DM的联系,甲状腺疾病,和代谢综合征。
    方法:从开始到2023年10月,利用PubMed对英语文献进行了系统搜索。我们确定了530项研究,并根据临床意义选择了19项。统计支持,以及与论文目标的相关性。
    结果:NL和DM共存很普遍,率从11%到65.71%不等。NL可能先于DM诊断,并且在此类患者中观察到NL与每日胰岛素需求增加之间存在相关性。NL被认为是DM并发症的潜在预后标志物;然而,最近的研究质疑这种联系,强调需要进一步研究。NL和甲状腺疾病的研究表明,尤其是自身免疫性甲状腺炎.关于NL和代谢综合征,NL患者中代谢综合征的患病率明显高于普通人群.此外,糖尿病患者溃疡NL通常表现为高血压或肥胖,关于高血压和肥胖对NL溃疡的潜在影响的问题。
    结论:需要进一步的研究来解开NL与各种合并症之间的复杂联系。
    BACKGROUND: Necrobiosis lipoidica (NL) is a rare granulomatous skin disorder with a predilection for females, often associated with diabetes mellitus (DM). This paper aims to comprehensively review the literature on NL, focusing on its association with DM, thyroid disorders, and the metabolic syndrome.
    METHODS: A systematic search was conducted in English-language literature from inception to October 2023, utilizing PubMed. We identified 530 studies and selected 19 based on clinical significance, statistical support, and relevance to the paper\'s goals.
    RESULTS: The coexistence of NL and DM is prevalent, with rates ranging from 11% to 65.71%. NL may precede DM diagnosis and a correlation between NL and increased daily insulin requirements has been observed in such patients. NL is suggested as a potential prognostic marker for DM complications; however, recent studies question this association, highlighting the need for further research. Studies in the context of NL and Thyroid Disease indicate a correlation, especially with autoimmune thyroiditis. Regarding NL and Metabolic Syndrome, the prevalence of metabolic syndrome among NL patients is notably higher than in the general population. Additionally, DM patients with ulcerated NL commonly exhibit hypertension or obesity, raising questions about the potential influence of hypertension and obesity on NL ulcerations.
    CONCLUSIONS: Additional research is required to untangle the complex connections between NL and various comorbidities.
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  • 文章类型: Letter
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    脂类坏死菌病是一种罕见的皮肤肉芽肿性疾病,主要影响糖尿病患者。该疾病的穿孔类型是一种不常见的变体,对治疗具有抵抗力,并且可以使用皮肤镜检查容易地识别。
    穿孔性坏死病(NL)是一种罕见的NL变体,主要影响糖尿病(DM)患者。皮肤镜检查有助于区分这种类型的疾病。射孔NL的扩散形式主要发生在DM设置中。在这里,我们介绍了一名24岁的1型DM女性的播散性穿孔NL病例。
    UNASSIGNED: Necrobiosis lipoidica is a rare cutaneous granulomatous disease that mainly affects diabetic patients. The perforating type of the disease is an uncommon variant that is resistant to therapy and can be easily identified using dermoscopy.
    UNASSIGNED: Perforating necrobiosis lipoidica (NL) is a rare NL variant that primarily affects patients with diabetes mellitus (DM). Dermoscopy helps to differentiate this type of disease. The disseminated form of perforating NL mainly occurs in the setting of DM. Here we present a case of disseminated perforating NL in a 24-year-old woman with type 1 DM.
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  • 文章类型: Systematic Review
    背景:脂类坏死病(NL)是一种罕见的,特发性,和难以治疗的胶原变性疾病,其治疗方案研究甚少。由于其反复出现的性质,溃疡的风险,高发病率,有必要了解现有的治疗方式,以更好地为临床护理提供信息。
    目的:这篇综述旨在描述文献中报道的治疗NL的治疗方式。
    方法:通过搜索2016年1月至2022年5月在PubMed和Scopus上发表的出版物,对治疗方法进行了文献检索。鉴于高质量证据有限,纳入病例报告和系列.仅包括提供有关尝试治疗和结果的信息的出版物。
    结果:共确定了60篇新文章(54例病例报告,两个案例系列,和四项回顾性队列研究)。这些研究累计报道了274名患者,并涵盖了包括光疗在内的治疗方法。外用皮质类固醇,局部钙调磷酸酶抑制剂,生物制剂,免疫抑制剂,JAK抑制剂,联合疗法,和其他几个人。光动力疗法的证据最多(80例患者中有72例得到改善),基于UVA的光疗(33个中的12个),外用皮质类固醇(46个中的21个),压缩疗法(20个中的15个),和局部钙调磷酸酶抑制剂(17个中的11个)。还描述了几种较新的治疗方法,包括ustekinumab和JAK抑制剂。
    结论:本系统综述对最近发表的NL治疗方法进行了全面总结。由于现有数据主要来自病例报告和系列,没有评估统计结论。需要更多的具有标准化终点的随机对照试验来比较治疗效果。
    BACKGROUND: Necrobiosis lipoidica (NL) is a rare, idiopathic, and recalcitrant disease of collagen degeneration for which treatment options have been poorly studied. Due to its recurring nature, risk for ulceration, and high morbidity, there is a need to understand existing treatment modalities to better inform clinical care.
    OBJECTIVE: This review aims to describe the therapeutic modalities reported in the literature for the treatment of NL.
    METHODS: A literature search of treatments was performed by searching for publications between January 2016 and May 2022 on PubMed and Scopus. Given the limited high-quality evidence, case reports and series were included. Only publications presenting information on both attempted treatments and outcomes were included.
    RESULTS: A total of 60 novel articles were identified (54 case reports, two case series, and four retrospective cohort studies). These studies cumulatively reported on 274 patients and covered treatments including phototherapy, topical corticosteroids, topical calcineurin inhibitors, biologics, immunosuppressants, JAK inhibitors, combination therapies, and several others. The greatest amount of evidence was found for photodynamic therapy (improvement in 72 of 80 patients), UVA-based phototherapy (12 of 33), topical corticosteroids (21 of 46), compression therapy (15 of 20), and topical calcineurin inhibitors (11 of 17). Several newer treatments were also described, including ustekinumab and JAK inhibitors.
    CONCLUSIONS: This systematic review provides a comprehensive summary of recently published treatments for NL. As the existing data comes predominantly from case reports and series, statistical conclusions are not assessed. A greater number of randomized controlled trials with standardized endpoints are necessary to compare treatment efficacy.
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  • 文章类型: Journal Article
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