• 【与一步式自蚀刻胶粘剂结合在一起的搪瓷中的渗透性起泡。】 复制标题 收藏 收藏
    DOI:10.1177/154405910408300404 复制DOI
    作者列表:Tay FR,Lai CN,Chersoni S,Pashley DH,Mak YF,Suppa P,Prati C,King NM
    BACKGROUND & AIMS: :One-step self-etch adhesives behave as permeable membranes after polymerization, permitting water to move through the cured adhesives. We hypothesize that osmotic blistering occurs in bonded enamel when these adhesives are used without composite coupling. Tooth surfaces from extracted human premolars were bonded with 5 one-step self-etch adhesives. They were immersed in distilled water or 4.8 M CaCl(2), and examined by stereomicroscopy, field-emission/environmental SEM, and TEM. Water blisters were observed in bonded enamel but not in bonded dentin when specimens were immersed in water. They collapsed when water was subsequently replaced with CaCl(2). Blisters were absent from enamel in specimens that were immersed in CaCl(2) only. Water trees were identified from adhesive-enamel interfaces. Osmotic blistering in enamel is probably caused by the low water permeability of enamel. This creates an osmotic gradient between the bonded enamel and the external environment, causing water sorption into the interface.
    背景与目标: :一步式自蚀刻胶粘剂在聚合后表现为可渗透膜,使水能够流过固化的胶粘剂。我们假设,当使用这些粘合剂而没有复合偶联剂时,粘结的瓷釉中会发生渗透性起泡。用5种单步自蚀刻粘合剂将提取的人类前磨牙的牙齿表面粘合在一起。将它们浸入蒸馏水或4.8 M CaCl(2)中,并通过体视显微镜,场发射/环境SEM和TEM进行检查。将标本浸入水中时,在粘合的瓷釉中观察到水泡,但在粘合的牙本质中未观察到水泡。随后将水替换为CaCl(2)时,它们崩溃了。仅浸入CaCl(2)的标本中的搪瓷上没有气泡。从粘合剂-瓷釉界面中鉴定出水树。搪瓷中的渗透性起泡可能是由于搪瓷的低透水性引起的。这会在粘结的瓷釉和外部环境之间产生渗透梯度,从而导致水吸附到界面中。
  • 【自身免疫性水疱病中的新型免疫调节药物。】 复制标题 收藏 收藏
    DOI:10.1016/s0733-8635(05)70305-3 复制DOI
    作者列表:Korman NJ
    BACKGROUND & AIMS: The autoimmune blistering diseases are a fascinating group of diseases characterized by the presence of blisters involving the skin and mucous membranes. Understanding of the diagnosis, pathophysiology, and advances in treatment of these diseases has grown enormously in recent years. In this article, the author discusses the major clinical and immunopathologic findings in bullous pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, linea IgA bullous disease, and pemphigus. The article focuses on the therapeutic management of patients with autoimmune blistering diseases, including the appropriate treatment of patients, with particular emphasis on the use of immunomodulating and immunosuppresive agents.

    背景与目标: 自身免疫性水疱病是一组引人入胜的疾病,其特征在于存在涉及皮肤和粘膜的水泡。近年来,对这些疾病的诊断,病理生理学和治疗进展的了解已大大增加。在本文中,作者讨论了大疱性天疱疮,粘膜膜天疱疮,表皮松解性大疱性水疱,线虫IgA大疱性疾病和天疱疮的主要临床和免疫病理学发现。本文着重于自身免疫性水疱病患者的治疗管理,包括对患者的适当治疗,特别着重于免疫调节剂和免疫抑制剂的使用。

  • 【在自身免疫性水疱性疾病中使用皮质类固醇。】 复制标题 收藏 收藏
    DOI:10.1016/j.iac.2012.04.008 复制DOI
    作者列表:Frew JW,Murrell DF
    BACKGROUND & AIMS: :Corticosteroids, while providing rapid remission and ongoing control of symptoms of autoimmune blistering diseases (AIBD), have numerous potentially serious acute and long-term side effects. Evidence-based medicine has reevaluated the various types of corticosteroids and forms of corticosteroid delivery in AIBD to ascertain whether any advantages of specific delivery systems or regimens exist. Careful monitoring of patients and simple preventive measures are effective in minimizing the adverse outcomes associated with their use. This article outlines the current level of evidence for corticosteroid use in AIBDs, and discusses appropriate investigations and interventions to minimize or prevent the associated adverse effects.
    背景与目标: :皮质类固醇可提供快速缓解并持续控制自身免疫性水疱病(AIBD)症状,但具有许多潜在的严重急性和长期副作用。循证医学已经重新评估了AIBD中各种类型的皮质类固醇和皮质类固醇的递送形式,以确定特定的递送系统或治疗方案是否存在任何优势。仔细监视患者并采取简单的预防措施可有效减少与使用相关的不良后果。本文概述了AIBD中使用皮质类固醇的现有证据水平,并讨论了适当的调查和干预措施,以最大程度地减少或预防相关的不良影响。
  • 【苯妥英钠对减轻大疱性表皮松解的水疱作用4例报道。】 复制标题 收藏 收藏
    DOI: 复制DOI
    作者列表:Sasidharan CK,Vijayakumar M,Vinodkumor MS
    BACKGROUND & AIMS: :To evaluate the effect of phenytoin in reducing of patients with epidermolysis bullosa four newborn babies were studied after thorough clinical evaluation including detailed history and relevant investigations. All of them were put on oral phenytoin sodium, in the usual antiepileptic dose and were followed up. All of the babies had significant reduction in the number of lesions.
    背景与目标: :为了评估苯妥英钠在大疱性表皮松解症患者中的减少作用,经过全面的临床评估,包括详细的病史和相关研究,对4例新生儿进行了研究。所有患者均按常规抗癫痫药剂量口服苯妥英钠,并进行随访。所有婴儿的病灶数量均明显减少。
  • 【针对起泡的化学战剂的医学防御。】 复制标题 收藏 收藏
    DOI: 复制DOI
    作者列表:Smith WJ,Dunn MA
    BACKGROUND & AIMS: :First used in World War I, chemical blistering agents present a serious medical threat that has stimulated renewed interest in the light of extensive use in recent conflicts. Current medical management cannot yet prevent or minimize injury from the principal agent of concern--sulfur mustard. Research directed at this goal depends on defining effective intervention in the metabolic alterations induced by exposure to sulfur mustard.
    背景与目标: :起泡剂首次用于第一次世界大战,由于最近在冲突中被广泛使用,化学起泡剂带来了严重的医学威胁,激发了人们的新兴趣。当前的医学管理尚不能防止或减少主要关注因素-硫芥末的伤害。针对该目标的研究取决于对暴露于硫芥子气引起的代谢变化进行有效干预。
  • 【转移性卵巢癌相关的表皮下水疱性疾病伴有针对p200真皮抗原和层粘连蛋白5的γ2亚基的自身抗体,显示出异常的临床特征。】 复制标题 收藏 收藏
    DOI:10.1111/j.1365-2133.2008.08483.x 复制DOI
    作者列表:Mitsuya J,Hara H,Ito K,Ishii N,Hashimoto T,Terui T
    BACKGROUND & AIMS: :Anti-p200 pemphigoid is an autoimmune subepidermal blistering disease characterized by autoantibodies to an unknown 200-kDa acidic noncollagenous glycoprotein of the lower lamina lucida, whereas antilaminin 5 mucous membrane pemphigoid is characterized by autoantibodies to a major basement membrane extracellular matrix, laminin 5. We report a 64-year-old Japanese woman with a subepidermal blistering disease associated with lymph node metastasis of ovarian clear cell carcinoma 10 years after its surgical treatment. Clinical features showed severe blisters and erosions on multiple mucous membranes (i.e. lip, oral cavity, nose, eye, genitalia and anus) and on both the periungual and subungual regions. This is the first report in which an immunoblot analysis revealed the unusual combination of autoantibodies to both the p200 antigen and the gamma 2 subunit of laminin 5.
    背景与目标: Anti-p200天疱疮是一种自身免疫性表皮下水疱性疾病,其特征是针对下部lucina lucida的未知200 kDa酸性非胶原糖蛋白的自身抗体,而antilaminin 5粘膜天疱疮的特征是针对主要基底膜细胞外基质laminin 5的自身抗体。我们报道了一名64岁的日本女性,患有10年后的卵巢透明细胞癌淋巴结转移相关的表皮下水疱性疾病。临床表现为在多个粘膜(即嘴唇,口腔,鼻子,眼睛,生殖器和肛门)以及在唇周和舌下区域均出现严重的水泡和侵蚀。这是第一份免疫印迹分析显示针对p200抗原和层粘连蛋白5的gamma 2亚基的自身抗体异常组合的报告。
  • 【在表皮祖细胞中转录因子Ovol2的过表达导致皮肤起泡。】 复制标题 收藏 收藏
    DOI:10.1016/j.jid.2017.02.985 复制DOI
    作者列表:Lee B,Watanabe K,Haensel D,Sui JY,Dai X
    BACKGROUND & AIMS: -2
    背景与目标: -2
  • 【一例表皮下水疱病,对多个层粘连蛋白亚单位有自身抗体,后来又发展了与膜性肾小球肾病相关的IV型胶原α-5链的自身抗体。】 复制标题 收藏 收藏
    DOI:10.2340/00015555-2059 复制DOI
    作者列表:Sueki H,Sato Y,Ohtoshi S,Nakada T,Yoshimura A,Tateishi C,Borza DB,Fader W,Ghohestani RF,Hirako Y,Koga H,Ishii N,Tsuchisaka A,Qian H,Li X,Hashimoto T
    BACKGROUND & AIMS: :We report a 68-year-old Japanese female patient with subepidermal blistering disease with autoantibodies to multiple laminins, who subsequently developed membranous glomerulonephropathy. At skin disease stage, immunofluorescence demonstrated IgG anti-basement membrane zone antibodies reactive with dermal side of NaCl-split skin. Immunoblotting of human dermal extract, purified laminin-332, hemidesmosome-rich fraction and laminin-521 trimer recombinant protein (RP) detected laminin γ-1 and α-3 and γ-2 subunits of laminin-332. Three years after skin lesions disappeared, nephrotic symptoms developed. Antibodies to α-3 chain of type IV collagen (COL4A3) were negative, thus excluding the diagnosis of Goodpasture syndrome. All anti-laminin antibodies disappeared. Additional IB and ELISA studies of RPs of various COL4 chains revealed reactivity with COL4A5, but not with COL4A6 or COL4A3. Although diagnosis of anti-laminin γ-1 (p200) pemphigoid or anti-laminin-332-type mucous membrane pemphigoid could not be made, this case was similar to previous cases with autoantibodies to COL4A5 and/or COL4A6.
    背景与目标: :我们报道了一名68岁的日本女性,患有表皮下水疱病,对多种层粘连蛋白具有自身抗体,随后发展成膜性肾小球肾病。在皮肤疾病阶段,免疫荧光显示IgG抗基底膜区抗体可与NaCl分裂的皮肤的真皮侧发生反应。人类皮肤提取物,纯化的层粘连蛋白332,富含半脂质体的级分和层粘连蛋白521三聚体重组蛋白(RP)的免疫印迹检测到层粘连蛋白332的层粘连蛋白γ-1和α-3和γ-2亚基。皮肤病变消失三年后,出现肾病症状。 IV型胶原的α-3链抗体(COL4A3)呈阴性,因此排除了Goodpasture综合征的诊断。所有抗层粘连蛋白抗体均消失。对各种COL4链的RP进行的其他IB和ELISA研究表明,它与COL4A5有反应性,但与COL4A6或COL4A3没有反应性。尽管无法诊断出抗lamininγ-1(p200)天疱疮或抗laminin-332型粘膜天疱疮,但该病例与先前具有COL4A5和/或COL4A6自身抗体的病例相似。
  • 【颗粒酶B抑制可降低自身免疫性水疱性疾病的疾病严重程度。】 复制标题 收藏 收藏
    DOI:10.1038/s41467-020-20604-3 复制DOI
    作者列表:Hiroyasu S,Zeglinski MR,Zhao H,Pawluk MA,Turner CT,Kasprick A,Tateishi C,Nishie W,Burleigh A,Lennox PA,Van Laeken N,Carr NJ,Petersen F,Crawford RI,Shimizu H,Tsuruta D,Ludwig RJ,Granville DJ
    BACKGROUND & AIMS: :Pemphigoid diseases refer to a group of severe autoimmune skin blistering diseases characterized by subepidermal blistering and loss of dermal-epidermal adhesion induced by autoantibody and immune cell infiltrate at the dermal-epidermal junction and upper dermis. Here, we explore the role of the immune cell-secreted serine protease, granzyme B, in pemphigoid disease pathogenesis using three independent murine models. In all models, granzyme B knockout or topical pharmacological inhibition significantly reduces total blistering area compared to controls. In vivo and in vitro studies show that granzyme B contributes to blistering by degrading key anchoring proteins in the dermal-epidermal junction that are necessary for dermal-epidermal adhesion. Further, granzyme B mediates IL-8/macrophage inflammatory protein-2 secretion, lesional neutrophil infiltration, and lesional neutrophil elastase activity. Clinically, granzyme B is elevated and abundant in human pemphigoid disease blister fluids and lesional skin. Collectively, granzyme B is a potential therapeutic target in pemphigoid diseases.
    背景与目标: :类天疱疮疾病是指一组严重的自身免疫性皮肤起泡疾病,其特征在于表皮下起泡和自身抗体诱导的表皮粘附性丧失,免疫细胞在真皮-表皮交界处和上层真皮中浸润。在这里,我们使用三种独立的小鼠模型探讨了分泌免疫细胞的丝氨酸蛋白酶粒酶B在类天疱疮疾病发病机理中的作用。与对照相比,在所有模型中,颗粒酶B的敲除或局部药理抑制作用均显着降低了总的起泡面积。体内和体外研究表明,颗粒酶B通过降解真皮-表皮连接中关键的锚定蛋白(对真皮-表皮的粘附是必需的)而有助于起泡。此外,粒酶B介导IL-8 /巨噬细胞炎性蛋白2分泌,病变中性粒细胞浸润和病变中性粒细胞弹性蛋白酶活性。在临床上,人类天疱疮疾病的水疱液和病灶皮肤中的颗粒酶B升高并且含量很高。总的来说,粒酶B是类天疱疮疾病的潜在治疗靶标。
  • 【遗传性皮肤起泡中角蛋白5的保守螺旋终止肽中的突变。】 复制标题 收藏 收藏
    DOI:10.1038/356244a0 复制DOI
    作者列表:Lane EB,Rugg EL,Navsaria H,Leigh IM,Heagerty AH,Ishida-Yamamoto A,Eady RA
    BACKGROUND & AIMS: :In the hereditary blistering condition epidermolysis bullosa simplex, the skin blisters on trauma following rupture of epidermal basal cells. Clinical variations range from severely incapacitating, especially in early childhood, to mild forms that may not even present clinically. Dowling-Meara epidermolysis bullosa simplex is characterized by clusters of epidermal blisters and keratin clumping in the cytoplasm; recent reports describe potentially causal mutations in keratin 14 (refs 2, 3). Here we describe a 'complementary' mutation at the other end of the other keratin expressed by these cells (K5, coexpressed with K14), a change from a Glu to a Gly in the helix termination peptide, detected by altered antibody binding and confirmed by sequencing using the polymerase chain reaction. The two conserved helix boundary peptides are predicted to be essential for filament assembly, and the requirement for two complementary (type I and type II) keratins is absolute. Epidermolysis bullosa simplex diseases demonstrate the function of the keratin cytoskeleton in resisting compaction stresses which otherwise lead to cell lysis.
    背景与目标: :在大疱性表皮松解的遗传性起泡情况下,表皮基底细胞破裂后,皮肤因创伤而起水泡。临床变化范围从严重丧失工作能力(尤其是在儿童早期丧失工作能力)到可能甚至在临床上都不存在的轻度形式。 Dowling-Meara表皮松解性大疱性单纯疱疹的特征是在细胞质中有表皮水泡簇和角蛋白聚集。最近的报道描述了角蛋白14中潜在的因果突变(参考文献2、3)。在这里,我们描述了由这些细胞表达的其他角蛋白(K5,与K14共表达)另一端的“互补”突变,螺旋终止肽从Glu变为Gly的变化,通过改变抗体结合来检测并通过使用聚合酶链反应进行测序。预测这两个保守的螺旋边界肽对于长丝组装是必不可少的,并且对两个互补的(I型和II型)角蛋白的需求是绝对的。表皮松解性大疱性单纯性疾病证明了角蛋白细胞骨架在抵抗压紧应力方面的功能,否则该压紧应力会导致细胞溶解。
  • 【从茄子茄中分离出的番茄起泡花叶病毒高度分离株。】 复制标题 收藏 收藏
    DOI:10.1007/s11262-016-1288-z 复制DOI
    作者列表:Blawid R,Hayashi EA,Rezende JA,Kitajima EW,Nagata T
    BACKGROUND & AIMS: :The complete genome of a tymovirus infecting Solanum violaefolium was sequenced. The genome comprised 6284 nt, with a 5'-UTR of 137 nt and a comparatively longer 3'-UTR of 121 nt. Sequence analysis confirmed three ORFs encoding a movement protein, a polyprotein, and a coat protein (CP). The isolate was considered to be the Tomato blistering mosaic virus (ToBMV) based on a CP amino acid sequence identity of 95.3 %. The nucleotide sequence of the complete genome of the S. violaefolium isolate, however, differed markedly from the other two reported ToBMV isolates, with identities of 76.6 and 76.3 %, below one of the demarcation criteria of the genus Tymovirus (overall genome identity of 80 %). No recombination signals were detected in the genome of this isolate. The high identity of the CP amino acid sequence and similar host responses suggest that the S. violaefolium isolate belongs to the same species as the Tomato blistering mosaic virus. The sequence analysis of this ToBMV isolate thus suggests that the demarcation criterion of 80 % overall genome sequence identity in the genus Tymovirus may require revision.
    背景与目标: :对感染小菜蛾的鼓膜病毒的完整基因组进行了测序。基因组包含6284nt,具有5'-UTR为137nt和相对更长的3'-UTR为121nt。序列分析证实了三个ORF,它们编码运动蛋白,多蛋白和外壳蛋白(CP)。基于95.3%的CP氨基酸序列同一性,该分离物被认为是番茄起泡花叶病毒(ToBMV)。但是,紫罗兰链球菌分离物完整基因组的核苷酸序列与其他两个报道的ToBMV分离物显着不同,同一性分别为76.6和76.3%,低于胸膜炎病毒属的一种划界标准(总体基因组同一性为80)。 %)。在该分离物的基因组中未检测到重组信号。 CP氨基酸序列的高度同一性和相似的宿主反应表明,紫堇链球菌分离株与番茄起泡花叶病毒属于同一物种。因此,对该ToBMV分离株的序列分析表明,在Tymovirus属中,总基因组序列同一性为80%的分界标准可能需要修改。
  • 【自身免疫性水疱病的结果措施。】 复制标题 收藏 收藏
    DOI:10.1111/1346-8138.12711 复制DOI
    作者列表:Zhao CY,Murrell DF
    BACKGROUND & AIMS: :Outcome measures are crucial in assessing an autoimmune blistering disease's (AIBD) severity as well as its impact on the patient's quality of life (QOL). The standardization of AIBD outcome measures is pivotal to accurately monitor the patient and to pool results from randomized controlled trials for meta-analysis, and thereby provide knowledge of the optimal AIBD therapies. In the past decade, several AIBD severity outcome measures have been developed and validated. For pemphigus severity, the Pemphigus Disease Area Index (PDAI) developed by the International Pemphigus Definitions Group was shown to be the most superior, followed by the Autoimmune Bullous Skin Disorder Intensity Score (ABSIS) by the German group. For bullous pemphigoid severity, the Bullous Pemphigoid Disease Area Index (BPDAI) was shown to be an accurate and valid measure. To quantify the burden of AIBD and its treatments on QOL, the Autoimmune Bullous Disease Quality of Life (ABQOL) and the Treatment of Autoimmune Bullous Disease Quality of Life (TABQOL) were also developed, validated, and are now being validated in multiple languages and cultures.
    背景与目标: :结果措施对于评估自身免疫性水疱病(AIBD)的严重程度及其对患者生活质量(QOL)的影响至关重要。 AIBD结局指标的标准化对于准确监控患者并汇总来自随机对照试验的结果进行荟萃分析至关重要,从而提供了最佳AIBD疗法的知识。在过去的十年中,已经开发并验证了几种AIBD严重程度结果指标。对于天疱疮的严重程度,由国际天疱疮定义小组制定的天疱疮疾病区域指数(PDAI)被证明是最好的,其次是德国小组的自身免疫性大疱性皮肤疾病强度评分(ABSIS)。对于大疱性类天疱疮严重程度,显示了大疱性类天疱疮疾病面积指数(BPDAI)是一种准确有效的方法。为了量化AIBD的负担及其对QOL的治疗,还开发,验证了自身免疫性大疱性疾病生活质量(ABQOL)和自身免疫性大疱性疾病生活质量(TABQOL),并正在以多种语言进行验证。文化。
  • 【老年人水疱疾病:诊断和治疗。】 复制标题 收藏 收藏
    DOI:10.1016/s0733-8635(03)00116-5 复制DOI
    作者列表:Sami N,Yeh SW,Ahmed AR
    BACKGROUND & AIMS: :This article discusses the major blistering diseases in the geriatric population. The diagnosis of both immune- and non-immune-mediated blistering disorders can be confirmed with the help of histologic and immunopathologic studies. Various serologic assays, which are more specific, also can be used to confirm the diagnosis of autoimmune blistering diseases. These techniques have facilitated the diagnosis and allowed the institution of early treatment. The treatment of blistering disorders has included both localized and systemic treatments. Localized treatment involves topical care including the following measures: the prevention of trauma; soaking of blisters in antiseptic (potassium permanganate or aluminum subacetate) solutions; topical and intralesional corticosteroids; and the prevention and early treatment of infections with local or systemic antibiotics. Conventional oral systemic therapies that have proved to be beneficial include systemic corticosteroids, anti-inflammatory agents, and immunosuppressive agents. Because the elderly are more prone to the side effects of these systemic agents, it is crucial that routine hematologic tests be done and monitored until the treatments have been discontinued. Recently, newer alternative treatment modalities have proved to be successful in patients who failed to respond or developed multiple side effects to the conventional oral systemic agents. In conclusion, as clinicians gain a greater understanding into the pathogenesis of these diseases, more specific molecular-targeted treatments will most likely become available.
    背景与目标: :本文讨论了老年人口中的主要水疱病。免疫和非免疫介导的水疱性疾病的诊断都可以通过组织学和免疫病理学研究得到证实。各种更特异性的血清学检测也可用于确认自身免疫性水疱病的诊断。这些技术有助于诊断并允许早期治疗。起泡性疾病的治疗包括局部治疗和全身治疗。局部治疗涉及局部护理,包括以下措施:预防创伤;将水泡浸泡在防腐剂(高锰酸钾或亚乙酸铝)溶液中;局部和病灶内糖皮质激素;以及预防和早期治疗局部或全身性抗生素的感染。已被证明是有益的常规口服全身疗法包括全身性皮质类固醇,抗炎药和免疫抑制剂。由于老年人更容易出现这些全身性药物的副作用,因此至关重要的是,必须进行常规的血液学检查并进行监测,直到停止治疗为止。最近,事实证明,较新的替代治疗方式在对常规口服全身用药无反应或产生多种副作用的患者中很成功。总而言之,随着临床医生对这些疾病的发病机理有了更深入的了解,更有可能使用更具体的针对分子的治疗方法。
  • 【利妥昔单抗在哪里治疗自身免疫性粘膜皮肤水疱性皮肤病?】 复制标题 收藏 收藏
    DOI: 复制DOI
    作者列表:Nigam R,Levitt J
    BACKGROUND & AIMS: :We propose rituximab as a first-line therapy for pemphigus vulgaris and steroid-dependent bullous pemphigoid with or without systemic steroids. A brief review of the literature substantiates the significant risk associated with the use of long term, high-dose prednisone, mycophenolate mofetil (MMF), and azathioprine. No head-to-head studies are available with respect to safety and efficacy of rituximab versus these therapies. When comparing the side effects of rituximab to MMF, both are found to be mild when used as monotherapy in dermatologic patients. The most severe side effects of rituximab include fatal infusion reactions and hypersensitivity, pancytopenia, infection and organ dysfunction. With MMF, malignancy, pancytopenia, infection, and organ dysfunction are the most concerning side effects. The frequencies of these observed adverse events are difficult to compare, but the side effect profiles of rituximab and MMF are clearly similar. Therefore, there is equipoise whether to use rituximab before rather than after MMF and/or systemic corticosteroids.
    背景与目标: :我们建议使用利妥昔单抗作为寻常性天疱疮和类固醇依赖性大疱性类天疱疮的一线治疗,无论是否全身性使用类固醇。对文献的简要回顾证实了长期使用大剂量泼尼松,霉酚酸酯和硫唑嘌呤会带来重大风险。目前尚无关于利妥昔单抗相对于这些疗法的安全性和有效性的正面研究。当比较利妥昔单抗与MMF的副作用时,在皮肤病患者中作为单一疗法使用时,发现两者均较轻。利妥昔单抗最严重的副作用包括致命的输注反应和超敏反应,全血细胞减少,感染和器官功能障碍。对于MMF,恶性,全血细胞减少,感染和器官功能障碍是最令人担忧的副作用。这些观察到的不良事件的发生频率很难比较,但是利妥昔单抗和MMF的副作用曲线非常相似。因此,是否在MMF和/或全身性皮质类固醇之前而不是之后使用利妥昔单抗是有平衡的。
  • 【三代后出现全身斑驳的色素沉着和产后皮肤起泡。】 复制标题 收藏 收藏
    DOI:10.1016/j.jaad.2003.07.015 复制DOI
    作者列表:Westerhof W,Dingemans KP
    BACKGROUND & AIMS: :We describe three generations of a family expressing progressive mottled hypopigmentation and hyperpigmentation on the non-exposed parts of the body from childhood to adult life. At birth, they all had epidermal blistering of the distal extremities. Although the palmoplantar warty keratoses could be related to the bulla formation, the pigmentary changes could not. Otherwise, there were no systemic disorders. Genetic diseases with spotty epidermal hypopigmentation and hyperpigmentation form a long list and the diagnosis is not always easy. Although different diagnostically, the condition resembled an entity described by Siemens in 1922 and epidermolysis bullosa with mottled pigmentation. Molecular biological investigation would be required to characterize the phenotype of this entity, which apparently was a mutation occurring in one family for three generations.
    背景与目标: :我们描述了一个家庭的三代人,从童年到成年,他们在身体的未暴露部位表现出斑驳的色素沉着和色素沉着过度。出生时,他们都患有远端肢体的表皮水泡。尽管掌plant疣状角化病可能与大疱的形成有关,但色素变化却无关。否则,没有全身性疾病。伴有表皮色素沉着过多和色素沉着过多的遗传性疾病的名单很长,诊断并不总是那么容易。尽管在诊断上有所不同,但该病状类似于西门子在1922年所描述的病状和表皮松解性大疱,并带有斑驳的色素沉着。需要进行分子生物学研究来表征该实体的表型,这显然是一个家族中发生了三代的突变。

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