blisters

水泡
  • 文章类型: English Abstract
    Mycosis fungoides (MF) is defined as the most common cutaneous T‑cell lymphoma (CTCL). The bullous form is considered one of its numerous variants. Only a few cases of this rare entity have been described. We report the case of a man with an aggressive course of bullous MF, which led to lethal outcome within a few weeks due to a fulminant sepsis.
    UNASSIGNED: Die Mycosis fungoides (MF) ist das häufigste kutane T‑Zell-Lymphom (CTCL). Die bullöse Form gilt als eine ihrer zahlreichen Varianten. In der Literatur sind nur wenige Fälle dieser seltenen Entität beschrieben. Wir berichten von einem männlichen Patienten mit fulminantem Verlauf einer bullösen MF, die aufgrund der infausten septischen Gesamtkonstellation innerhalb weniger Wochen zum Exitus letalis führte.
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  • 文章类型: Journal Article
    偶氮聚合物是光响应材料,有望转化体外细胞培养系统。通过精确的光照,它们有助于基板图案的形成和擦除,允许动态控制和创建细胞和材料之间的活动接口。然而,这些材料表现出在水溶液的存在下从支撑玻璃局部分离的趋势,如细胞培养基,由于水泡的形成,它们是在偶氮聚合物膜-玻璃界面处产生的充满液体的空腔。这些水泡阻碍了偶氮材料表面的精确结构化,限制它们在细胞存在下用于表面光活化。在这项研究中,我们提出了一种经济有效且易于实施的方法,通过玻璃基板的硅烷功能化来改善偶氮聚合物-玻璃界面的稳定性。这种方法被证明是有效的防止水泡形成,从而能够原位动态调节偶氮聚合物表面以进行活细胞实验。此外,我们证明了用于诱导偶氮聚合物表面变化的光照条件不会诱导光毒性效应。因此,这种方法促进了光可转换偶氮聚合物细胞培养平台的开发,用于研究多个原位刻录和擦除周期对细胞功能的影响,同时保持生理湿润的微环境。
    Azopolymers are light-responsive materials that hold promise to transform in vitro cell culture systems. Through precise light illumination, they facilitate substrate pattern formation and erasure, allowing for the dynamic control and creation of active interfaces between cells and materials. However, these materials exhibit a tendency to locally detach from the supporting glass in the presence of aqueous solutions, such as cell culture media, due to the formation of blisters, which are liquid-filled cavities generated at the azopolymer film-glass interface. These blisters impede precise structurization of the surface of the azomaterial, limiting their usage for surface photoactivation in the presence of cells. In this study, we present a cost-effective and easily implementable method to improve the azopolymer-glass interface stability through silane functionalization of the glass substrate. This method proved to be efficient in preventing blister formation, thereby enabling the dynamic modulation of the azopolymer surface in situ for live-cell experiments. Furthermore, we proved that the light-illumination conditions used to induce azopolymer surface variations do not induce phototoxic effects. Consequently, this approach facilitates the development of a photoswitchable azopolymer cell culture platform for studying the impact of multiple in situ inscription and erasure cycles on cell functions while maintaining a physiological wet microenvironment.
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  • 文章类型: Review
    大疱疮是一种罕见的遗传性皮肤病,可引起水疱。在真皮-表皮交界处或附近编码结构蛋白的基因被连续或主要突变,这是EB的主要原因。在这里,两名中国男孩被诊断出患有这种疾病,每个基因都有不同的变异,作为EB遗传咨询的参考。皮肤护理显著影响其预后和生活质量。
    方法:两个中国男孩,表型正常的父母,被诊断出明显的水疱症状,一种患有显性营养不良性大疱性表皮松解症,另一种患有严重形式的单纯性大疱性表皮松解症。第一位患者在COL7A1等位基因中有G到A变异,在核苷酸位置6163,被命名为“G2055A”。由于COL7A1等位基因在三螺旋结构域具有甘氨酸取代,因此先证为营养不良性大疱性表皮松解症的杂合。在他的母亲身上发现了一个类似的变种,表明了它对后代的潜在传播。另一名患者患有严重的单纯大疱性表皮松解症,罕见的c.377T>A变体导致氨基酸p.Leu126Arg(NM_000526.5(c.377T>G,p.Leu126Arg)在角蛋白14基因中。在先前的文献中,角蛋白14与良好的预后相关。然而,我们患有这种罕见变异的患者在21日龄时不幸死于脓毒症。据报道该变体仅发生一次。
    结论:我们的研究表明,患有COL7A1c.6163G>A和KRT14c.377T>A变异的大疱性表皮松解症患者具有不同的临床表现,与显性形式的营养不良性EB相比,其表型更为温和。因此,c.6163G>A患者预后较好。此外,c.377T>A患者比c.6163G>A基因变异的患者更容易感染。基因检测对于确定负责的特定变体和改善治疗方案至关重要。
    UNASSIGNED: Bullosa is a rare hereditary skin condition that causes blisters. Genes encoding structural proteins at or near the dermal-epidermal junction are mutated recessively or dominantly, and this is the primary cause of EB. Herein, two Chinese boys were diagnosed with the condition, each with a different variant in a gene that serves as a reference for EB genetic counseling. Skincare significantly impacted their prognosis and quality of life.
    METHODS: Two Chinese boys, with phenotypically normal parents, have been diagnosed with distinct blister symptoms, one with Dominant Dystrophic Epidermolysis Bullosa and the other with a severe form of Epidermolysis Bullosa Simplex. The first patient had a G-to-A variant in the COL7A1 allele, at nucleotide position 6163 which was named \"G2055A\". The proband is heterozygous for Dystrophic Epidermolysis Bullosa due to a COL7A1 allele with a glycine substitution at the triple helix domain. A similar variant has been discovered in his mother, indicating its potential transmission to future generations. Another patient had severe Epidermolysis Bullosa Simplex with a rare c.377T > A  variant resulting in substitution of amino acid p.Leu126Arg (NM_000526.5 (c.377T > G, p.Leu126Arg) in the Keratin 14 gene. In prior literature, Keratin 14 has been associated with an excellent prognosis. However, our patient with this infrequent variant tragically died from sepsis at 21 days old. There has been a reported occurrence of the variant only once.
    CONCLUSIONS: Our study reveals that Epidermolysis Bullosa patients with COL7A1 c.6163G > A and KRT14 c.377T>A variants have different clinical presentations, with dominant forms of Dystrophic EB having milder phenotypes than recessive ones. Thus, the better prognosis in the c.6163G > A patient. Furthermore, c.377T>A patient was more prone to infection than the patient with c.6163G>A gene variant. Genetic testing is crucial for identifying the specific variant responsible and improving treatment options.
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  • 文章类型: Case Reports
    球形类天疱疮引起的吞咽困难是一种罕见但重要的表现。一名77岁的大疱性类天疱疮患者,无神经系统病史,表现为严重的口咽吞咽困难,可归因于潜在的起泡和水肿,根据纤维内窥镜吞咽检查的记录。
    Dysphagia resulting from bulbous pemphigoid is a rare but significant manifestation. A 77-year-old with bullous pemphigoid with no neurological history presented with severe oropharyngeal dysphagia, attributable to underlying blistering and edema, as documented on a fiberoptic endoscopic swallow examination.
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  • 文章类型: Case Reports
    大疱性类天疱疮(BP)是一种复杂的自身免疫性水疱疾病,在合并症人群中发病率增加,尤其是在老年人中。药物诱导的BP的发生与潜在的遗传易感性有关,引发增强的免疫反应,自身抗体的形成,以及基底膜区内抗原特性的改变。有超过90种确定的能够沉淀BP的药物,我们介绍了一例87岁女性患者的合并症,她经历了从氯沙坦到沙库巴曲/缬沙坦的药物变化.三个月后,她的下肢出现红斑,进展为伴有瘙痒的全身性皮疹。在接下来的一个月里,这些病变演变成紧张的水泡,伴有浆液性内容和剧烈疼痛。怀疑药物改用沙库巴曲/缬沙坦是原因,药物被停用了,开始免疫调节治疗,导致病变的显着改善。
     Bullous pemphigoid (BP) is a complex autoimmune blistering disease with an increased incidence in the comorbid population, particularly among older adults. The occurrence of drug-induced BP is associated with an underlying genetic predisposition, triggering an enhanced immune response, the formation of autoantibodies, and alterations in antigenic properties within the basement membrane zone. With over 90 identified drugs capable of precipitating BP, we present the case of an 87-year-old woman with comorbidities who experienced a medication change from losartan to sacubitril/valsartan. Three months later, erythematous lesions appeared on her lower limbs, progressing to a generalized rash accompanied by itching. Over the following month, these lesions evolved into tense blisters with serous content and intense pain. Suspecting the medication switch to sacubitril/valsartan as the cause, the drug was discontinued, and immunomodulatory treatment was initiated, resulting in a notable improvement in the lesions.
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  • 文章类型: Journal Article
    澳大拉西亚的龙虾和小龙虾会发展为尾扇坏死(TFN综合征)。已经进行了许多尝试来寻找原发性病原体或将该综合征与商业活动联系起来,但是解决方案仍然难以捉摸。TFN综合症是一个“邪恶的问题”,由于不完整和矛盾的信息形成了一个没有简单解决方案的潜在结果矩阵而难以或不可能解决的问题。回顾文献显示,有时据报道TFN综合征与telson和尾足类动物的无菌水泡有关,这些水泡可能会破裂,从而导致环境真菌和/或细菌菌群的入侵。之前是否形成水泡,或者因为,感染是未知的。TFN综合征有时在囚禁中发展,有时需要事先侮辱Telson和uropods,并且在野外的患病率是零散的。文献显示,与TFN综合征相关的水泡的原因仍然是一个谜,为此,我们提出了几个可能的启动因素。我们强烈敦促研究人员不要就TFN综合征的病因得出结论。如果不仔细探索替代病因,同时认识到“相关性不等于因果关系”这一古老的教训,就无法解释。
    Lobsters and crayfish in Australasia can develop a condition known as Tail Fan Necrosis (TFN syndrome). Many attempts have been made to find a primary pathogen or link the syndrome to commercial activities, but a solution remains elusive. TFN syndrome is a \'wicked problem\', a problem difficult or impossible to solve because of incomplete and contradictory information forming a matrix of potential outcomes with no simple solution. Reviewing the literature shows TFN syndrome is sometimes reported to develop in association with sterile blisters on the telson and uropods which may rupture permitting invasion by environmental fungal and/or bacterial flora. Whether blisters form prior to, or because of, infection is unknown. TFN syndrome sometimes develops in captivity, sometimes requires a previous insult to the telson and uropods, and prevalence is patchy in the wild. The literature shows the cause of blisters associated with TFN syndrome remains an enigma, for which we suggest several possible initiating factors. We strongly urge that researchers not \'jump to conclusions\' as to the aetiology of TFN syndrome. It cannot be explained without carefully exploring alternative aetiologies whilst being cognisant of the age-old lesson that \'correlation does not equal causation\'.
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  • 文章类型: Journal Article
    表演艺术家,比如舞者,歌手,演员和音乐家,依靠他们的身体来成功地执行他们的艺术形式。然而,有关影响舞者缺乏的皮肤病学状况的文献。通过电子邮件向舞蹈专业人员分发了一份匿名的REDCap®安全调查,舞蹈未成年人,和舞蹈教师/教授在五个弗吉尼亚本科院校。关于人口统计的回应,舞蹈风格,和皮肤病在2个月内记录。当被问及发展为皮肤病时,57(59%)的调查参与者报告患有皮肤病,比如痤疮,湿疹,多汗症,和足底疣。当被问及皮肤病加剧或被认为是由舞蹈引起的时,56(59%)报告水泡,老茧,皮肤分裂,指甲/脚感染,向内生长的指甲,和地板烧伤。这项研究证明了两个主要发现:跳舞可能会加剧当前的皮肤疾病,某些皮肤状况可能是由跳舞引起的。此外,赤脚跳舞的常见做法可能有助于某些皮肤状况的发展。限制包括样本量,响应偏差,和缺乏验证的调查。
    Performing artists, such as dancers, singers, actors and musicians, rely on their physical bodies to successfully execute their artforms. However, literature regarding dermatologic conditions that impact dancers is lacking. An anonymous REDCap® secure survey was distributed by email to Dance Majors, Dance Minors, and Dance Instructors/Professors at five Virginia undergraduate institutions. Responses regarding demographics, style of dance, and dermatological diseases were recorded over a 2 month period. When asked about developing skin disease, 57 (59%) of survey participants reported experiencing skin diseases, such as acne, eczema, hyperhidrosis, and plantar warts. When asked about skin diseases exacerbated or believed to be caused from dancing, 56 (59%) reported blisters, callouses, skin splitting, nail/foot infection, ingrown nails, and floor burns. This study demonstrates two main findings: dancing may exacerbate current skin disorders and some skin conditions may be caused by dancing. Additionally, the common practice of dancing barefoot likely contributes to the development of certain skin conditions. Limitations include sample size, response bias, and lack of validation of the survey.
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  • 文章类型: Journal Article
    背景:数字健康中的可穿戴传感器可能会通过使用可穿戴贴片对皮肤造成刺激的风险。关于患者和产品相关因素如何影响皮肤刺激的风险知之甚少。带传感器的阿立哌唑片(AS,AbilifyMyCite;OtsukaAmericaPharmaceutical,Inc)是一种数字医疗系统,用于治疗精神分裂症患者,I型双相情感障碍,和重度抑郁症.AS包括阿立哌唑片剂,带有嵌入的可摄取事件标记,通过可穿戴贴片附着在皮肤上的可穿戴传感器,一个智能手机应用程序,和基于Web的门户。不断改进最终产品,开发了可穿戴补丁的连续迭代,包括葡萄干补丁版本4(RP4),其次是一次性可穿戴传感器版本5(DW5),然后可重复使用的可穿戴传感器版本2(RW2)。
    目的:本分析汇集了使用RP4、DW5和RW2可穿戴式AS贴片的成年参与者临床研究的安全性数据,并评估了与使用可穿戴贴片相关的不良事件。
    方法:分析了2010年5月至2020年8月在18-65岁成年人中进行的12项研究的安全性数据。所有研究都评估了安全性,在不到2周的研究中,还特别检查了与AS组件使用相关的人为因素。健康的志愿者或精神分裂症患者,I型双相情感障碍,纳入或重度抑郁症;暴露于至少1个可穿戴贴片的患者被纳入安全性分析.评估与使用可穿戴贴片相关的不良事件。磨损,水泡,皮炎,变色,红斑,刺激,疼痛,瘙痒,皮疹,和皮肤反应被分组为皮肤刺激事件(SIE)。所有统计分析均为描述性。
    结果:分析包括763名参与者(平均[SD]年龄42.6[12.9]岁;白人:n=359,47.1%;男性:n=420,55%)。参与者是健康志愿者(n=269,35.3%)或精神分裂症患者(n=402,52.7%),双相I型障碍(n=57,7.5%),或重度抑郁障碍(n=35,4.6%)。总的来说,13.6%(104/763)的参与者报告至少1个SIE,所有这些都位于可穿戴贴片站点。≥1个斑块相关SIE的发生率为18.1%(28/155),14.2%(55/387),和9.2%(28/306)的参与者分别使用RP4、DW5和RW2。SIE相关治疗中止的发生率较低,1.9%(3/155)报告,3.1%(12/387),使用RP4、DW5和RW2的参与者分别为1.3%(4/306)。
    结论:从RP4到RW2的可穿戴贴片版本报告的SIE发生率表明,从报告的不良事件中获得的信息可能为产品设计和开发提供了信息,这可以提高连续产品的耐受性和耐磨性。
    背景:Clinicaltrials.govNCT02091882,https://clinicaltrials.gov/study/NCT02091882;Clinicaltrials.govNCT02404532,https://clinicaltrials.gov/NCT0585/CTtrigoal91928/
    BACKGROUND: Wearable sensors in digital health may pose a risk for skin irritation through the use of wearable patches. Little is known about how patient- and product-related factors impact the risk of skin irritation. Aripiprazole tablets with sensor (AS, Abilify MyCite; Otsuka America Pharmaceutical, Inc) is a digital medicine system indicated for the treatment of patients with schizophrenia, bipolar I disorder, and major depressive disorder. AS includes aripiprazole tablets with an embedded ingestible event marker, a wearable sensor attached to the skin through a wearable patch, a smartphone app, and a web-based portal. To continuously improve the final product, successive iterations of wearable patches were developed, including raisin patch version 4 (RP4), followed by disposable wearable sensor version 5 (DW5), and then reusable wearable sensor version 2 (RW2).
    OBJECTIVE: This analysis pooled safety data from clinical studies in adult participants using the RP4, DW5, and RW2 wearable patches of AS and evaluated adverse events related to the use of wearable patches.
    METHODS: Safety data from 12 studies in adults aged 18-65 years from May 2010 to August 2020 were analyzed. All studies evaluated safety, with studies less than 2 weeks also specifically examining human factors associated with the use of the components of AS. Healthy volunteers or patients with schizophrenia, bipolar I disorder, or major depressive disorder were enrolled; those who were exposed to at least 1 wearable patch were included in the safety analysis. Adverse events related to the use of a wearable patch were evaluated. Abrasions, blisters, dermatitis, discoloration, erythema, irritation, pain, pruritus, rash, and skin reactions were grouped as skin irritation events (SIEs). All statistical analyses were descriptive.
    RESULTS: The analysis included 763 participants (mean [SD] age 42.6 [12.9] years; White: n=359, 47.1%; and male: n=420, 55%). Participants were healthy volunteers (n=269, 35.3%) or patients with schizophrenia (n=402, 52.7%), bipolar I disorder (n=57, 7.5%), or major depressive disorder (n=35, 4.6%). Overall, 13.6% (104/763) of the participants reported at least 1 SIE, all of which were localized to the wearable patch site. Incidence of ≥1 patch-related SIEs was seen in 18.1% (28/155), 14.2% (55/387), and 9.2% (28/306) of participants who used RP4, DW5, and RW2, respectively. Incidence of SIE-related treatment discontinuation was low, which is reported by 1.9% (3/155), 3.1% (12/387), and 1.3% (4/306) of participants who used RP4, DW5, and RW2, respectively.
    CONCLUSIONS: The incidence rates of SIEs reported as the wearable patch versions evolved from RP4 through RW2 suggest that information derived from reported adverse events may have informed product design and development, which could have improved both tolerability and wearability of successive products.
    BACKGROUND: Clinicaltrials.gov NCT02091882, https://clinicaltrials.gov/study/NCT02091882; Clinicaltrials.gov NCT02404532, https://clinicaltrials.gov/study/NCT02404532; Clinicaltrials.gov NCT02722967, https://clinicaltrials.gov/study/NCT02722967; Clinicaltrials.gov NCT02219009, https://clinicaltrials.gov/study/NCT02219009; Clinicaltrials.gov NCT03568500, https://clinicaltrials.gov/study/NCT03568500; Clinicaltrials.gov NCT03892889, https://clinicaltrials.gov/study/NCT03892889.
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  • 文章类型: Journal Article
    Pachyonychia Congenita Project (PC Project) is an international patient advocacy organization dedicated to patients who suffer from pachyonychia congenita (PC). This condition is a painful and debilitating skin disorder caused by a mutation in one of five keratin genes: KRT6A, KRT6B, KRT6C, KRT16,or KRT17. Through two primary programs, namely the International Pachyonychia Congenita Consortium (IPCC) and the International Pachyonychia Congenita Research Registry (IPCRR), PC Project provides comprehensive patient support and diagnostics while uniting patients, researchers, physicians, and industry partners on a global level to advance research and drug development for meaningful treatments and, ultimately, a cure for PC.
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  • 文章类型: Journal Article
    在目前的研究中,大块钨材料表面暴露于氢,氘,以及射频域(13.56MHz)中的氦等离子体,使用空心阴极配置,输入功率为250W。喷射的材料以各种距离(从6mm至40mm)收集在钛基底上。因此,研究暴露的钨材料的表面变化(起泡发生,粉尘形成,或纳米结构),随着结晶度,取决于等离子体的暴露时间(每种等离子体类型从30分钟到120分钟)。此外,对收集的材料进行分析(形态学,结构,和统计调查)的灰尘和灰尘膜状外观。使用两种方法分析等离子体放电:发射光谱,和单个朗缪尔探测器,为了强调所用等离子体的性质(冷放电,~2eV),随着钨发射的存在(例如,WI406.31nm,WI421.31nm)在等离子体寿命期间。通过使用专用协议,建立了一种在氢和氘等离子体放电中获得与聚变相关的钨表面的方法。通过使用实现的方法,本文介绍了获得新的钨形态的可能性,即,蒲公英般的形状,通过使用氦等离子体,其中可以找到W18O49化合物。
    In the current study, bulk tungsten material surfaces are exposed to hydrogen, deuterium, and helium plasmas in the radiofrequency domain (13.56 MHz) at an input power of 250 W using the hollow-cathode configuration. The ejected material is collected on titanium substrates at various distances (from 6 mm up to 40 mm). Therefore, the exposed tungsten materials are investigated for surface changes (blister occurrence, dust formation, or nano-structuration), along with the crystallinity, depending on the plasma\'s exposure times (from 30 min up to 120 min for each plasma type). Also, the collected materials are analyzed (morphological, structural, and statistical investigations) for dust and dust film-like appearance. Plasma discharges are analyzed using two methods: optical emission spectroscopy, and single Langmuir probes, to emphasize the nature of the used plasmas (cold discharges, ~2 eV), along with the presence of tungsten emission (e.g., WI 406.31 nm, WI 421.31 nm) during the plasma lifetime. By using a dedicated protocol, a method was established for obtaining fusion-relevant tungsten surfaces in the hydrogen and deuterium plasma discharges. By using the implemented method, the current paper introduces the possibility of obtaining a new tungsten morphology, i.e., the dandelion-like shape, by using helium plasma, in which the W18O49 compound can be found.
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