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Clinical challenges and recent advances in the diagnosis of bullous pemphigoid 大疱性类天疱疮诊断的临床挑战和最新进展
Pub Date : 2013-08-01 DOI: 10.1586/17469872.2013.816497
S. Tambe, S. Häfliger, L. Borradori
Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease associated with autoantibodies directed against two components of junctional adhesion complexes in stratified epithelia, BPAG2 and BPAG1-e. BP typically develops in the elderly and manifests with widespread eczematous, urticated and bullous lesions. In up to 20% of the affected patients, obvious blistering is lacking and the clinical features of BP are misleading and closely mimic a variety of other inflammatory conditions. Diagnosis of BP, which should be rather called cutaneous pemphigoid, relies on direct immunofluorescence microscopy studies to detect tissue-bound IgG and/or C3 immune deposits along the epidermal basement membrane zone. Here, the clinical presentations of BP and the diagnostic algorithm with the immunopathological studies available to classify affected patients are reviewed. Finally, the need for widely accepted criteria for the classification of BP patients is emphasized, since in a number of patients with featu...
大疱性类天疱疮(BP)是一种自身免疫性表皮下起疱性疾病,与针对分层上皮中连接粘附复合物BPAG2和BPAG1-e两种成分的自身抗体相关。BP通常发生于老年人,表现为广泛的湿疹、荨麻疹和大疱性病变。在多达20%的受影响的患者中,没有明显的起泡,BP的临床特征具有误导性,并且与各种其他炎症非常相似。BP的诊断,更应该被称为皮肤类天疱疮,依赖于直接免疫荧光显微镜研究,检测表皮基底膜区组织结合的IgG和/或C3免疫沉积物。在这里,BP的临床表现和诊断算法与免疫病理研究可用于分类受影响的患者进行综述。最后,强调需要广泛接受的BP患者分类标准,因为在许多具有特征的患者中…
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引用次数: 6
Efinaconazole 10% solution: a new topical antifungal therapy for onychomycosis 10%艾非那康唑溶液:一种治疗甲真菌病的新型局部抗真菌药物
Pub Date : 2013-08-01 DOI: 10.1586/17469872.2013.814872
B. Elewski
Onychomycosis is a common nail infection for which treatment options are limited, and no new treatment have been introduced for over 10 years. While patients might prefer a topical therapy, efficacy with ciclopirox and amorolfine lacquers has been disappointing especially in moderate to severe disease. Efinaconazole 10% solution is a new triazole antifungal, specifically developed for the topical treatment of onychomycosis. Its physicochemical properties, antifungal activity and formulation are all important aspects of the development program and are reviewed here. Efinaconazole 10% solution may provide the first viable alternative to oral therapy for onychomycosis. Mycologic cure rates are comparable to those seen with oral itraconazole, and greater than reported with ciclopirox lacquer.
甲真菌病是一种常见的指甲感染,治疗方法有限,10多年来没有引入新的治疗方法。虽然患者可能更喜欢局部治疗,但环匹罗和阿莫罗芬漆的疗效令人失望,特别是在中度至重度疾病中。10%艾非那康唑溶液是一种新型三唑类抗真菌药物,专门用于局部治疗甲癣。它的理化性质,抗真菌活性和配方都是开发计划的重要方面,在这里进行综述。10%艾非那康唑溶液可能提供口服治疗甲癣的第一个可行的替代方案。真菌学治愈率与口服伊曲康唑相当,比环匹罗漆的治愈率更高。
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引用次数: 2
Challenges involved in the diagnostic interpretation of FISH for melanocytic neoplasms 在黑素细胞肿瘤的FISH诊断解释中涉及的挑战
Pub Date : 2013-08-01 DOI: 10.1586/17469872.2013.814861
Chelsea Cooper, L. Sholl, P. Gerami
At the molecular level, melanoma and nevi exhibit distinct chromosomal copy number aberrations. As a result, the addition of molecular techniques such as FISH may improve the diagnostic procedures for melanocytic neoplasms. FISH probes targeting 6p25, 11q13, 6q23, 9p21, 8q24 and CEP 6 are currently used in the diagnosis of melanoma. While these assays can be of great utility, there are challenges that may emerge in optimizing interpretation. In this review, the authors discuss technical challenges commonly associated with the FISH protocol such as overdigestion, underdigestion and excessive background. The authors also present challenges associated with interpretation of the FISH for chromosomal copy number changes and offer suggestions as to how the effects of these difficulties can be minimized.
在分子水平上,黑色素瘤和痣表现出明显的染色体拷贝数畸变。因此,诸如FISH之类的分子技术的加入可能会改善黑色素细胞肿瘤的诊断程序。FISH探针靶向6p25、11q13、6q23、9p21、8q24和CEP 6,目前用于黑色素瘤的诊断。虽然这些分析方法非常实用,但在优化解释方面可能会出现挑战。在这篇综述中,作者讨论了通常与FISH协议相关的技术挑战,如消化过度、消化不足和过量的背景。作者还提出了与FISH解释染色体拷贝数变化相关的挑战,并就如何将这些困难的影响降至最低提出了建议。
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引用次数: 0
Etanercept for the treatment of psoriasis 依那西普用于治疗牛皮癣
Pub Date : 2013-08-01 DOI: 10.1586/17469872.2013.814880
M. Ara, E. Daudén
Etanercept is a systemic tumor necrosis factor-α inhibitor indicated for use in the treatment of rheumatoid arthritis, polyarticular juvenile idiopathic arthritis, psoriatic arthritis, ankylosing spondylitis and plaque psoriasis. A considerable number of studies have demonstrated the efficacy and safety of etanercept in these indications, thus offering a new and promising treatment option. The drug was approved by the Food and Drug Administration in the United States and by the European Medicines Evaluation Agency in Europe for treating moderate-to-severe psoriasis in 2004. This article reviews the pharmacology, clinical efficacy and safety of etanercept for psoriasis and recent findings since its approval. The number of studies of medium- and long-term efficacy and safety is increasing steadily; these studies support the use of etanercept in a maintenance setting, with an apparently more favorable risk: benefit ratio than traditional systemic treatments.
依那西普是一种全身性肿瘤坏死因子-α抑制剂,用于治疗类风湿关节炎、多关节幼年特发性关节炎、银屑病关节炎、强直性脊柱炎和斑块性银屑病。相当多的研究已经证明依那西普在这些适应症中的有效性和安全性,从而提供了一个新的和有希望的治疗选择。2004年,该药被美国食品和药物管理局(fda)和欧洲药品评估局(European Medicines Evaluation Agency)批准用于治疗中重度牛皮癣。本文综述了依那西普治疗银屑病的药理学、临床疗效和安全性及其获批以来的最新研究进展。关于中长期疗效和安全性的研究正在稳步增加;这些研究支持在维持环境中使用依那西普,显然比传统的全身治疗具有更有利的风险:效益比。
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引用次数: 2
Managing mycetoma: guidelines for best practice 足菌肿管理:最佳实践指南
Pub Date : 2013-06-01 DOI: 10.1586/EDM.13.31
A. Fahal, A. Elkhawad
Mycetoma is a serious health problem that is endemic in many tropical and subtropical regions and characterized by devastating deformities, disability and high morbidity. It is a badly neglected disease worldwide. To date, mycetoma’s true incidence, prevalence and route of infection are not well understood; likewise, its susceptibility, resistance and response to medical treatment, which negatively affects patient management. Current treatment, in particular for eumycetoma, has proven to be quite ineffective, have serious side effects and is expensive for patients and health authorities in endemic areas. In general, the eumycetoma treatment outcome is disappointing and is associated with a low cure rate and high amputation and recurrence rates. Late patient presentation is a major problem in mycetoma due to the lack of health education, poor patient socioeconomic status and poor treatment compliance. This is aggravated by a lack of national and international disease awareness, which has led to a massive k...
足菌肿是一个严重的健康问题,在许多热带和亚热带地区流行,其特点是破坏性畸形、残疾和高发病率。在世界范围内,这是一种被严重忽视的疾病。迄今为止,足菌肿的真实发病率、流行率和感染途径尚不清楚;同样,它的易感性,耐药性和对医疗的反应,这对患者的管理产生了负面影响。事实证明,目前的治疗方法,特别是针对真菌肿的治疗方法,相当无效,而且有严重的副作用,对流行地区的患者和卫生当局来说费用昂贵。一般来说,真菌性肿的治疗结果令人失望,并且与低治愈率、高截肢率和复发率有关。由于缺乏健康教育,患者社会经济地位差和治疗依从性差,患者出现晚是足菌肿的主要问题。由于缺乏国家和国际疾病意识,这种情况更加严重,这导致了大规模的疾病。
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引用次数: 6
Developments into understanding the pathogenesis of systemic sclerosis 系统性硬化症发病机制的研究进展
Pub Date : 2013-06-01 DOI: 10.1586/EDM.13.8
N. Hunzelmann
Systemic sclerosis (SSc) is a severe, chronic autoimmune disease of the connective tissue. The disease typically becomes clinically apparent on the skin and subsequently spreads to several internal organs, in particular the gastrointestinal system, lung, heart and kidney (in decreasing frequency). The pathogenesis evolves via activation of the vascular and immune system, finally leading to a fibrotic response of the connective tissue and resulting in progressive dysfunction of the affected organs. Although the etiology still remains elusive, the knowledge of genetic factors associated with SSc has increased remarkably in recent years. It can be shown that SSc shares a number of genetic risk factors with other autoimmune diseases, in particular lupus erythematosus. New pathways such as Wnt signaling have been identified, which improve our understanding of the initiation of fibrosis in this still enigmatic disease. The enhanced insight into distinct steps of the organotypic pathophysiology of SSc, for examp...
系统性硬化症(SSc)是一种严重的结缔组织慢性自身免疫性疾病。这种疾病通常在临床上表现为皮肤,随后扩散到几个内脏器官,特别是胃肠道系统、肺、心脏和肾脏(频率逐渐降低)。其发病机制通过血管和免疫系统的激活而演变,最终导致结缔组织的纤维化反应,并导致受影响器官的进行性功能障碍。虽然病因仍然难以捉摸,但近年来对与SSc相关的遗传因素的了解显著增加。可以证明,SSc与其他自身免疫性疾病,特别是红斑狼疮,具有许多相同的遗传风险因素。新的通路如Wnt信号已经被发现,这提高了我们对这种仍然神秘的疾病中纤维化起始的理解。例如,对SSc器官型病理生理的不同步骤的深入了解……
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引用次数: 1
Hormonal acne: leading to a paradigm shift in the management of acne 荷尔蒙痤疮:导致痤疮管理的范式转变
Pub Date : 2013-06-01 DOI: 10.1586/EDM.13.21
Jayakar Thomas, K. Parimalam, R. Sindhu
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引用次数: 1
Comorbidities of psoriasis 牛皮癣的合并症
Pub Date : 2013-06-01 DOI: 10.1586/EDM.13.23
S. Churton, Kristen Doud, N. Korman
Psoriasis is a chronic, immune-mediated inflammatory skin disease that is associated with multiple comorbidities including psoriatic arthritis, cardiometabolic diseases, malignancies, nonalcoholic fatty liver disease, autoimmune diseases, inflammatory-bowel disease, obstructive sleep apnea and chronic obstructive pulmonary disease. Cardiovascular disease including myocardial infarction, stroke and subclinical atherosclerosis as well as obesity, metabolic syndrome, diabetes, hypertension and hyperlipidemia, are associated with psoriasis. Shared inflammatory pathways may be the basis for these disease associations, especially in the case of cardiovascular disease and its risk factors. Reduced quality of life, depression, anxiety, smoking and alcoholism are also seen in psoriasis patients. Awareness and understanding of the relationships between psoriasis and its comorbidities is important in managing patients with psoriasis. The multiple comorbidities associated with psoriasis will be discussed here, focusi...
银屑病是一种慢性、免疫介导的炎症性皮肤病,与多种合并症有关,包括银屑病关节炎、心脏代谢疾病、恶性肿瘤、非酒精性脂肪性肝病、自身免疫性疾病、炎症性肠病、阻塞性睡眠呼吸暂停和慢性阻塞性肺病。包括心肌梗死、中风、亚临床动脉粥样硬化在内的心血管疾病,以及肥胖、代谢综合征、糖尿病、高血压和高脂血症,都与牛皮癣有关。共同的炎症通路可能是这些疾病关联的基础,特别是在心血管疾病及其危险因素的情况下。生活质量下降、抑郁、焦虑、吸烟和酗酒也见于牛皮癣患者。认识和理解牛皮癣及其合并症之间的关系对管理牛皮癣患者很重要。银屑病的多重合并症将在此讨论,重点是…
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引用次数: 1
Mycosis fungoides in children and adolescents 儿童和青少年蕈样真菌病
Pub Date : 2013-06-01 DOI: 10.1586/EDM.13.29
A. Nanda, H. Al-Ajmi
Mycosis fungoides (MF) is a low-grade lymphoproliferative disorder that is histologically characterized by an epidermotropic proliferation of small or medium-sized atypical T lymphocytes with cerebriform nuclei and is primarily a disease of the elderly. It is rare in children and adolescents; in large series of MF published in the literature, only a small percentage of patients were observed to have onset of MF below adolescence. It was believed that a younger age of onset of MF may herald a poorer prognosis. Recently, with improved knowledge of the basic nature of the disease and refined diagnostic facilities, a few series of MF among children and adolescence have appeared and have drawn more awareness to the behavior of MF in this age group. This review summarizes the clinicoepidemiological features, treatment and prognosis of MF among children and adolescents based on the current literature.
蕈样真菌病(MF)是一种低级别淋巴细胞增生性疾病,其组织学特征是具有脑状核的中小型非典型T淋巴细胞表皮性增生,主要发生在老年人身上。在儿童和青少年中很少见;在大量发表的MF文献中,只有一小部分患者在青春期以下发病。据信,年轻的发病年龄可能预示着较差的预后。近年来,随着对该病基本性质认识的提高和诊断设备的完善,儿童和青少年中出现了一些系列MF,并引起了人们对该年龄组MF行为的更多认识。本文结合文献对儿童青少年MF的临床流行病学特点、治疗及预后进行综述。
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引用次数: 9
Should there be an app for that? Controversies of diagnosing melanoma with your smartphone 应该有这样的应用吗?用智能手机诊断黑色素瘤的争议
Pub Date : 2013-06-01 DOI: 10.1586/EDM.13.22
J. F. Moreau, L. Ferris
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引用次数: 0
期刊
Expert Review of Dermatology
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