cutaneous manifestations

皮肤表现
  • 文章类型: Letter
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  • 文章类型: Journal Article
    背景:皮肌炎(DM)是一种罕见的全身性自身免疫性疾病,其特征是独特的衰弱性皮疹和骨骼肌无力。目前尚不清楚现有的临床结果评估(COA)措施是否包括优先考虑患者的概念以及完全捕获DM活动性皮肤表现改善所必需的概念。本研究旨在开发皮肤皮肌炎研究者全球评估(CDM-IGA),一个从头的IGA,用于成人DM的临床试验。
    方法:八名DM临床专家参加了60分钟的定性访谈,包括概念启发和认知汇报方法。概念启发包括开放式问题和随访探针,以探索临床医生治疗DM患者的经验,症状对患者生活质量的影响,和疾病的严重程度特征来探讨DM的进展。认知汇报要求临床专家对CDM-IGA进行审查,旨在评估DM皮肤疾病活动的严重程度。面试后,举行了与三名临床专家的协商一致会议,就与CDM-IGA有关的任何未决问题达成一致。
    结果:使用九位临床专家的意见迭代开发了CDM-IGA。所有临床医生提供的反馈都认为红斑是DM的主要活性皮肤表现,应该是CDM-IGA的主要特征,按红斑的颜色和程度分开。为了确定皮肤疾病的严重程度,专家建议增加一个叫做二次变化的指标,结合了侵蚀/溃疡和苔藓化,这可以修改病人的最终分数。三位临床专家建议,支持不同肤色红斑评估的照片指南可能是有益的。
    结论:开发了一种新型CDM-IGA,用于成年DM患者的临床试验,基于迭代开发过程,该过程结合了DM临床专家和重要的成年DM患者的定性反馈。
    BACKGROUND: Dermatomyositis (DM) is a rare systemic autoimmune disease characterized by a distinctive debilitating skin rash and skeletal muscle weakness. It is unclear if existing clinical outcome assessment (COA) measures include the concepts of priority to patients and those necessary to fully capture improvements in the active cutaneous manifestations of DM. This study aimed to develop the Cutaneous Dermatomyositis Investigator Global Assessment (CDM-IGA), a de novo IGA, for use in clinical trials of adult DM.
    METHODS: Eight DM clinical experts participated in 60-min qualitative interviews consisting of concept elicitation and cognitive debriefing methodologies. Concept elicitation comprised open-ended questions with follow-up probes to explore clinicians\' experiences of treating patients with DM, the impact of symptoms on patients\' quality of life, and the severity levels of disease characteristics to explore DM progression. Cognitive debriefing required the clinical experts to perform a review of the CDM-IGA, designed to assess the severity of cutaneous disease activity of DM. After the interviews, a consensus meeting with three clinical experts was held to agree on any outstanding issues relating to the CDM-IGA.
    RESULTS: The CDM-IGA was iteratively developed using the opinions of nine clinical experts. Feedback provided by all clinicians agreed that erythema was the main active cutaneous manifestation of DM and should be the primary characteristic on the CDM-IGA, split by erythema color and extent. To determine cutaneous disease severity, experts suggested adding a metric called secondary changes, which combined erosion/ulceration and lichenification, which could modify the patient\'s final score. Three clinical experts suggested that a photo-guide to support assessments of erythema across different skin tones could be beneficial.
    CONCLUSIONS: A novel CDM-IGA was developed for use with adult patients with DM in clinical trials, based on an iterative development process that combined qualitative feedback from clinical experts of DM and importantly adult patients living with DM.
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  • 文章类型: Case Reports
    PSTPIP1相关的骨髓相关蛋白血症炎性(PAMI)综合征是一种罕见的自身炎症性疾病,通常在儿科患者中出现。我们介绍了一个18岁女性的病例,有生长失败的病史,免疫球蛋白A肾病,和炎症性关节炎,他们出现在儿科皮肤科诊所,发现痤疮,银屑病样皮炎,和化脓性汗腺炎,其临床,遗传,实验室检查结果与PAMI综合征最为一致。我们进行了文献综述,以更好地描述这种罕见的皮肤病。识别在PAMI综合征中看到的独特皮肤发现可以帮助将其与其他炎症性疾病区分开来。加快诊断和治疗。
    PSTPIP1-associated myeloid-related proteinemia inflammatory (PAMI) syndrome is a rare autoinflammatory disorder often arising in pediatric patients. We present a case of an 18-year-old female with a past medical history of growth failure, immunoglobulin A nephropathy, and inflammatory arthritis who presented to a pediatric dermatology clinic with findings of acne, psoriasiform dermatitis, and hidradenitis suppurativa, whose clinical, genetic, and laboratory findings were most consistent with PAMI syndrome. We conducted a literature review to better characterize this rare condition in the context of dermatologic findings. Recognition of the distinctive skin findings seen in PAMI syndrome can help distinguish it from other inflammatory disorders, enabling expedited diagnosis and treatment.
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  • 文章类型: Journal Article
    Yao综合征(YAOS)是一种与核苷酸结合寡聚化结构域(NOD2)基因相关的新型全身性自身炎性疾病。它的特点是周期性发烧,胃肠道(GI)症状,关节炎,和皮炎,在其他症状中。关于这种疾病的文献很少,对其皮肤病学表现知之甚少。对现有文献进行了综述,以描述Yao综合征的皮肤表现。85.7%的患者有皮肤表现,具有涉及面部的红斑和斑块的共同特征性描述,树干,腹部,和四肢。根据我们对姚氏综合征的治疗方式的回顾,泼尼松是一种适当的初始方法,口服柳氮磺吡啶和其他缓解疾病的抗风湿药物作为适当的次要选择。YAOS应考虑在鉴别诊断患者出现皮肤皮疹,特别是在并发关节症状的情况下,周期性发烧,和胃肠道症状。
    Yao syndrome (YAOS) is a novel systemic autoinflammatory disease linked to the nucleotide-binding oligomerization domain (NOD2) gene. It is characterized by periodic fevers, gastrointestinal (GI) symptoms, arthritis, and dermatitis, among other symptoms. A sparse literature exists on this disease, and little is known about its dermatological manifestations. A review of available literature was performed to characterize the cutaneous manifestations of Yao syndrome. Cutaneous manifestations were documented in 85.7% of patients, with common characteristic descriptions of erythematous patches and plaques involving the face, trunk, abdomen, and extremities. Based on our review of treatment modalities employed for Yao syndrome, prednisone is an appropriate initial approach, with oral sulfasalazine and other disease-modifying antirheumatic drugs serving as appropriate secondary options. YAOS should be considered in the differential diagnosis of patients presenting with a dermatitic rash, especially in the context of concurrent articular symptoms, periodic fever, and GI symptoms.
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  • 文章类型: Journal Article
    先天性角化病(DC)是一种端粒病,在多个专业中提出诊断和治疗挑战;然而,微妙的皮肤病学体征可以早期发现,改变患者预后。根据研究中我们患者的临床标准进行特定的DC基因测序。随后,进行了主要遗传数据库中的交叉检查信息。此外,对文献进行了广泛的回顾,以组织DC的主要皮肤病学方面。我们报道了DC的一种新变体。此外,我们分享10有用和实用的信息,为皮肤科医生和任何专家照顾这一组患者。
    Dyskeratosis congenita (DC) is a telomeropathy presenting diagnostic and therapeutic challenges across multiple specialties; yet, subtle dermatological signs enable early detection, altering patient prognosis. A specific DC genetic sequencing was performed according to the clinical criteria of our patient in study. Subsequently, cross-checked information in the main genetic databases was carried out. Additionally, an extensive review of the literature was made to organize the main dermatological aspects in DC. We report a novel variant of DC. Additionally, we share 10 useful and practical messages for dermatologists and any specialist caring for this group of patients.
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  • 文章类型: Journal Article
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  • 文章类型: Journal Article
    背景技术肥胖是一种医学状况,其特征是过量脂肪的积累会对健康产生负面影响。导致预期寿命下降和健康问题加剧。肥胖患者经历由皮肤感染引起的皮肤变化,机械摩擦,以及各种皮肤肥大状况,如纤维瘤和黑棘皮病。大约60-70%的患有肥胖症的患者表现出一系列的皮肤变化。目的本研究的主要目的是确定与肥胖相关的各种类型的皮肤状况,并探讨其与体重指数(BMI)的关系。方法学这是一项横断面观察性研究。这项研究包括BMI大于30kg/m2的肥胖患者,他们访问了钦奈Saveetha医学院和医院的皮肤科门诊部,印度。我们在这项研究中招募了100名患者。获得同意后,人口统计信息,高度,体重,进行了皮肤检查,并记录了结果。统计分析采用卡方检验,其中P<0.05被认为是显著的。对独立样本进行t检验以分析定量变量。结果出现时的平均年龄为39.3岁,标准差为9.9。平均BMI为34.3,标准差为2.6。在所有患者中,34%属于31-40岁年龄组,在41-50岁年龄组中紧随其后的是30%,23%属于19-30岁年龄组,11%属于51-60岁年龄组,2%属于>60岁年龄组。大多数患者(63%)患有I类肥胖(BMI30.00-34.99),34%患有II类肥胖(BMI35.00-39.99),3%的人患有III类肥胖(BMI>40.00)。总体上最常见的皮肤表现是顶索,其次是黑棘皮病,茎纹,感染,牛皮癣。结论肥胖被认为是一个重要的公共卫生问题,其与皮肤问题的关联对许多临床医生来说具有实际的重要性。
    Background Obesity is a medical condition characterized by the accumulation of excess fat that can negatively impact health, resulting in a decreased life expectancy and heightened health issues. Obese patients experience skin changes caused by skin infections, mechanical friction, and various skin hypertrophic conditions like fibromas and acanthosis nigricans. Approximately 60-70% of patients suffering from obesity exhibit a range of skin changes.  Objective The main objective of the present study is to identify the various types of skin conditions linked to obesity and investigate their relationship with body mass index (BMI).  Methodology This is a cross-sectional observational study. This study included obese patients with a BMI greater than 30 kg/m2 who visited the dermatology outpatient department at Saveetha Medical College and Hospital in Chennai, India. We enrolled 100 patients in this study. After obtaining consent, demographic information, height, weight, and cutaneous examination were conducted, and the results were documented. Statistical analysis was conducted using the chi-squared test, where P<0.05 was considered significant. The t-test for independent samples was done to analyze quantitative variables. Results The mean age at presentation was 39.3, and the standard deviation was 9.9. The average BMI was 34.3, and the standard deviation was 2.6. Of the total patients, 34% belonged to the 31-40-year age group, which was followed by 30% in the 41-50-year age group, 23% belonged to the 19-30-year age group, 11% belonged to the 51-60-year age group, and 2% belonged to the >60-year age group. Most patients (63%) had Class I obesity (BMI 30.00-34.99), 34% had Class II obesity (BMI 35.00-39.99), and 3% had Class III obesity (BMI >40.00). The most common cutaneous manifestation overall was acrochordons, followed by acanthosis nigricans, striae distensae, infections, and psoriasis.  Conclusion Obesity is identified as a significant public health issue, and its association with skin problems is of practical importance for many clinicians.
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    文章类型: Review
    COVID-19是由SARS-CoV-2引起的以呼吸道症状为特征的传染病,发烧,和寒战。[1]虽然这些全身性症状是众所周知和众所周知的,也有COVID-19患者的皮肤病学表现的报道。这些表现包括冻疮样病变,斑丘疹病变,荨麻疹病变,坏死,和其他类似水痘的激词.[2]这些病变的发病机制尚不清楚,但COVID-19感染诱导的促凝和促炎状态可能导致各种皮肤表现.[3]药物相互作用和并发的超敏反应也被假定。[4]这篇综述旨在汇编和分析各种回顾性研究和病例报告,以总结与COVID-19感染相关的皮肤病学病变的临床表现,并提出进一步的研究领域。
    COVID-19 is an infectious disease caused by SARS-CoV-2 that is characterized by respiratory symptoms, fever, and chills.[1] While these systemic symptoms are widely known and well understood, there have also been reports of dermatological manifestations in patients with COVID-19. These manifestations include chilblain-like lesions, maculopapular lesions, urticarial lesions, necrosis, and other varicella-like exanthems.[2] The pathogenesis of these lesions are not well understood, but the procoagulant and pro-inflammatory state induced by COVID-19 infections may be contributing to varied cutaneous manifestations.[3] Drug interactions and concurrent hypersensitivity reactions have also been postulated.[4] This review aims to compile and analyze various retrospective studies and case reports to summarize the clinical presentation of dermatological lesions associated with COVID-19 infections and suggest further areas of research.
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  • 文章类型: Case Reports
    COVID-19是一场始于2020年的全球大流行。大流行期间,患者出现COVID-19感染的各种皮肤表现。目前,COVID-19感染在全球范围内仍然存在,虽然程度较小。该病例报告描述了一名21岁的男性患者,他的手和脚踝外侧出现丘疹囊泡三天。该患者还出现了8天的呼吸道症状,并检测出COVID-19阳性。重要的是要了解与COVID-19相关的皮肤表现,因为这可以帮助诊断和理解病情。
    COVID-19 is a global pandemic that started in 2020. During the pandemic, patients have presented with various cutaneous manifestations of COVID-19 infections. Currently, COVID-19 infections are still present worldwide, although to a lesser extent. This case report describes a 21-year-old male patient who presented with papulovesicles on his hands and lateral aspects of his ankles for three days. The patient also experienced respiratory symptoms for eight days and tested positive for COVID-19. It is important to have an understanding of the skin manifestations associated with COVID-19, as this can aid in the diagnosis and comprehension of the condition.
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  • 文章类型: Journal Article
    肤色(SOC)的患者在许多情况下表现出不同的疾病,然而,缺乏有关此类感染患者皮肤表现的表现和诊断的信息。因此,我们进行了范围审查,以调查和总结与SOC患者感染性疾病的临床表现和皮肤表现诊断有关的信息,重点是以下病毒,细菌,毒素介导的,感染疾病和真菌感染:人类免疫缺陷病毒,带状疱疹,脓疱病,猩红热,莱姆病,中毒性休克综合征,疮,立克次体,和皮肤真菌感染。这项范围审查确定了有关SOC患者皮肤感染性疾病表现的文献空白,例如缺乏视觉示例和对常见感染性疾病的更精确描述。缺乏描述SOC患者感染性疾病皮肤表现的高质量文献和教育资源可能会导致护理障碍;因此,传染病和皮肤病学需要更多的研究和合作。
    Patients with skin of color (SOC) present diseases differently in many circumstances, yet there is a lack of information regarding the presentation and diagnosis of cutaneous manifestations in such patients experiencing infectious diseases. Therefore, we conducted a scoping review to investigate and summarize information pertaining to the clinical presentation and diagnosis of cutaneous manifestations of infectious diseases in patients with SOC focusing on the following viral, bacterial, toxin-mediated, and infestation diseases and fungal infections: human immunodeficiency virus, shingles, impetigo, scarlet fever, Lyme disease, toxic shock syndrome, scabies, rickettsioses, and cutaneous fungal infections. This scoping review identified literature gaps regarding cutaneous manifestations of infectious diseases in patients with SOC such as a lack of visual examples and more precise descriptions of common infectious diseases. The lack of better-quality literature and educational resources describing cutaneous manifestations of infectious diseases in patients with SOC may contribute to care barriers; therefore, more research and collaboration are needed in the specialties of both infectious diseases and dermatology.
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