cutaneous manifestations

皮肤表现
  • 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
    自身免疫性多内分泌病-念珠菌病-外胚层营养不良(APECED),或多腺自身免疫综合征1型(PAS-1/APS-1),是一种罕见的常染色体隐性遗传疾病,与自身免疫调节因子(AIRE)基因突变有关。这篇综述详细分析了APECED的皮肤表现,关注慢性粘膜皮肤念珠菌病(CMC),斑秃(AA),和白癜风。甲状旁腺功能减退的经典三联征,肾上腺功能不全,和CMC作为诊断的基石。然而,APECED的不同临床谱,尤其是它的皮肤表现,提出了诊断挑战。CMC,通常是早期的征兆,不同人群的患病率不同,包括芬兰语(100%),爱尔兰人(100%)沙特阿拉伯(80%)意大利(60-74.7%),北美(51-86%),和克罗地亚(57.1%)人口。同样,AA患病率在不同人群中有所不同。白癜风也表现出不同地区的患病率。该评论综合了截至2023年10月以英语发表的14项重要人类研究的叙事分析所产生的当前知识。此外,本文强调了早期发现和监测的重要性,强调皮肤表现为关键诊断指标。持续的研究和临床警惕对于解开这种罕见的自身免疫综合征的复杂性和加强患者护理至关重要。
    Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED), or polyglandular autoimmune syndrome type 1 (PAS-1/APS-1), is a rare autosomal recessive disorder linked to mutations in the autoimmune regulator (AIRE) gene. This review provides a detailed analysis of cutaneous manifestations in APECED, focusing on chronic mucocutaneous candidiasis (CMC), alopecia areata (AA), and vitiligo. The classic triad of hypoparathyroidism, adrenal insufficiency, and CMC serves as a diagnostic cornerstone. However, the varied clinical spectrum of APECED, particularly its cutaneous presentations, poses a diagnostic challenge. CMC, often an early sign, varies in prevalence across populations, including Finnish (100%), Irish (100%), Saudi Arabian (80%), Italian (60-74.7%), North American (51-86%), and Croatian (57.1%) populations. Similarly, AA prevalence varies in different populations. Vitiligo also exhibits variable prevalence across regions. The review synthesizes the current knowledge arising from a narrative analysis of 14 significant human studies published in English up to October 2023. Moreover, this paper underscores the importance of early detection and monitoring, emphasizing cutaneous manifestations as key diagnostic indicators. Ongoing research and clinical vigilance are crucial for unraveling the complexities of this rare autoimmune syndrome and enhancing patient care.
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  • 文章类型: Journal Article
    背景:关于肝细胞癌(HCC)副肿瘤皮肤表现的临床意义的公开数据仍然很少。方法:2022年12月对MEDLINE进行系统检索。纳入标准包括报告肝癌患者的研究,有副肿瘤皮肤表现的人。感兴趣的结果包括存活和对癌症定向和/或皮肤定向治疗的响应。结果:共有48项研究包括60例HCC患者。最常见的皮肤异常是皮肌炎,圆形糠疹,和卟啉症。大多数皮肌炎患者有潜在的病毒性肝炎,虽然所有报告的卟啉症和棘皮病病例都与HCC的代谢原因有关,比如脂肪变性。副肿瘤性皮肤变化在转移性疾病患者中更为常见。轮曲糠疹与最低的死亡风险相关,(OR:0.05,95%CI:0.003至0.89;p=0.04),而皮肌炎的死亡风险具有统计学意义(OR:3.37,95%CI:1.01-12.1;p=0.03)。大多数患者的皮肤异常有所改善,癌症定向治疗后。结论:在疾病负担较高的患者中,副肿瘤皮肤表现更频繁。尤其是存在转移。某些皮肤表现具有预后意义。
    Background: There remains a scarcity of published data on the clinical significance of paraneoplastic cutaneous manifestations in hepatocellular carcinoma (HCC). Method: A systematic search of MEDLINE was performed in December 2022. Inclusion criteria comprised studies reporting on patients with HCC, who had paraneoplastic cutaneous manifestations. Outcomes of interests comprise survival and response to cancer-directed and/or skin directed therapy. Results: A total of 48 studies comprising 60 HCC patients were included in the analysis. The most frequent reported skin abnormalities were dermatomyositis, pityriasis rotunda, and porphyria. Most patients presented with dermatomyositis had underlying viral hepatitis, while all reported porphyria and acanthosis cases were associated with metabolic causes of HCC, such as steatosis. Paraneoplastic skin changes were more common in patients with metastatic disease. Pityriasis Rotunda was associated with the lowest risk of death, (OR: 0.05, 95% CI: 0.003 to 0.89; p = 0.04), while dermatomyositis had a statistically significant higher risk of death (OR: 3.37, 95% CI: 1.01-12.1; p = 0.03). Most patients showed an improvement in their cutaneous abnormalities, following cancer-directed therapy. Conclusion: Paraneoplastic cutaneous manifestations are reported more frequently in patients with a higher burden of disease, especially presence of metastases. Certain cutaneous manifestations have prognostic implication.
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  • 文章类型: Journal Article
    背景:环境氯乙烯(VC)暴露可能会导致严重的急性和慢性皮肤病。因为现有文献主要集中在职业环境中的暴露,我们对大规模化学品泄漏的医疗后果的理解仍然存在差距。
    目的:本研究旨在研究在工业泄漏和其他环境灾难的背景下,VC暴露的潜在皮肤病学表现,并强调此类释放对公共卫生和司法的影响。
    方法:在这篇叙述性综述中,相关证据为基础,同行评审的科学来源,灰色文学,和媒体报道是通过使用与VC相关的预定关键字搜索词搜索PubMed和Google的搜索来识别的,VC溢出和释放,火车脱轨,皮肤病,公共卫生,以及弱势和边缘化人群。
    结果:接触性皮炎和冻伤可能会出现,强调快速净化的重要性。由于环境水库的持续存在而导致的慢性VC暴露的长期表现包括雷诺病,硬化皮肤变化,肢端骨溶解,和皮肤恶性肿瘤.皮肤表现的临床严重程度受个体易感性和持续时间的影响。强度,和曝光的途径。此外,VC的化学释放更频繁地影响有色人种和社会经济地位较低的群体,导致暴露相关疾病的发生率更高。
    结论:随着危险化学品的环境释放事件变得越来越普遍,并且由于皮肤相对于其他器官与环境毒素的接触增加,迫切需要更好地了解大规模的短期和长期健康影响,有毒物质暴露,强调需要持续的临床警惕。皮肤科医生和公共卫生官员还应致力于更好地了解危险化学品暴露对低收入和少数群体的不成比例影响可能加剧现有健康差距的方式。在这里,我们描述了有毒物质释放对健康的影响,并特别考虑了边缘化和弱势群体。除了法律和监管框架之外,我们主张改善公共卫生措施,不仅减轻了未来环境灾难的风险,但也要确保对它们做出及时有效的反应。
    BACKGROUND: Environmental vinyl chloride (VC) exposure may result in serious acute and chronic dermatological conditions. Because existing literature largely focuses on exposures in occupational settings, a gap persists in our understanding of the medical consequences of large-scale chemical spills.
    OBJECTIVE: This study aims to examine the potential dermatological manifestations of VC exposure in the context of industrial spills and other environmental disasters and to highlight the public health and justice implications of such releases.
    METHODS: In this narrative review, relevant evidence-based, peer-reviewed scientific sources, gray literature, and media reports were identified via searches of search PubMed and Google using predetermined keyword search terms related to VC, VC spills and releases, train derailment, cutaneous disease, public health, and vulnerable and marginalized populations.
    RESULTS: Contact dermatitis and frostbite may arise acutely, highlighting the importance of swift decontamination. Long-term manifestations from chronic VC exposure due to persistence in environmental reservoirs include Raynaud disease, sclerodermatous skin changes, acro-osteolysis, and cutaneous malignancies. The clinical severity of cutaneous manifestations is influenced by individual susceptibility as well as duration, intensity, and route of exposure. Additionally, chemical releases of VC more frequently impact Communities of Color and those of lower socioeconomic status, resulting in greater rates of exposure-related disease.
    CONCLUSIONS: With environmental release events of hazardous chemicals becoming increasingly common and because the skin has increased contact with environmental toxins relative to other organs, an urgent need exists for a greater understanding of the overall short- and long-term health impacts of large-scale, toxic exposures, underscoring the need for ongoing clinical vigilance. Dermatologists and public health officials should also aim to better understand the ways in which the disproportionate impacts of hazardous chemical exposures on lower-income and minority populations may exacerbate existing health disparities. Herein, we describe the health implications of toxic releases with particular consideration paid to marginalized and vulnerable populations. In addition to legal and regulatory frameworks, we advocate for improved public health measures, to not only mitigate the risk of environmental catastrophes in the future, but also ensure timely and effective responses to them.
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  • 文章类型: Journal Article
    疟疾是由疟原虫属原生动物引起的媒介传播的热带感染,并通过受感染的按蚊叮咬传播。这种疾病通常以发烧为特征,水肿,血小板减少症,低血糖,贫血,和肌痛;然而,感染的皮肤表现并不常见,也往往被忽视。进行了文献检索,重点是疟疾患者已注意到使用病例报告和当前可用文献呈现的各种皮肤病理。我们描述了与疟疾相关的各种皮肤表现,比如暴发性紫癜,高热性荨麻疹,皮肤利什曼病合并感染,传染性荨麻疹,间日疟原虫诱导的严重血小板减少症,肢端皮肤坏死,和网状红斑,以及这些皮肤表现如何提供对患者预后的洞察。必须强调有关这些皮肤表现的文件和警惕,因为它们可能导致更好的患者结果和对患者潜在疟疾的更深入的了解。
    Malaria is a vector-borne tropical infection caused by protozoa of the genus Plasmodium and is transmitted by the bite of an infected Anopheles mosquito. The disease is commonly characterized by fever, edema, thrombocytopenia, hypoglycemia, anemia, and myalgias; however, the infection\'s cutaneous presentations are not commonly emphasized and tend to be overlooked. A literature search was conducted that focused on the various skin pathologies that malaria patients have been noted to present with using case reports and currently available literature. We describe the various skin manifestations associated with malaria, such as purpura fulminans, febrile urticaria, cutaneous leishmaniasis co-infections, urticaria infectiosum, vivax-induced severe thrombocytopenia petechiae, acral skin necrosis, and reticulated erythema, and how each of these skin manifestations may provide insight into the patient\'s prognosis. Documentation and vigilance regarding these cutaneous manifestations must be emphasized as they may lead to better patient outcomes and a stronger understanding of the patient\'s underlying malaria.
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  • 文章类型: Journal Article
    疟疾是由各种形式的原生动物疟原虫引起的威胁生命的寄生虫病,并由雌性按蚊传播。寄生虫感染在90个国家流行,每年报告约5亿例病例,估计每年死亡150万至270万人。历史上,抗疟药物的使用有望用于疟疾的化学预防和治疗,降低年死亡率。值得注意的是,这些抗疟药与各种不良反应有关,包括肠胃不适和头痛.然而,这些抗疟药可能导致的不良皮肤表现缺乏文献记载和了解.我们的目的是描述研究较少的疟疾治疗的不良皮肤病理学,以更好地教育医生对患者的正确治疗。我们的叙述性综述描述了与特定抗疟药治疗相关的皮肤表现及其相关的预后和治疗。讨论的皮肤病理包括水性瘙痒(AP),掌plant剥脱,Steven-Johnson综合征,中毒性表皮坏死松解症,皮肤血管炎,牛皮癣,瘀斑,热带苔藓样皮炎.需要对抗疟药的皮肤不良事件进行进一步的研究和警惕的记录,以防止潜在的危及生命的不良结果。
    Malaria is a life-threatening parasitic disease caused by various forms of the protozoa Plasmodium and is transmitted by the female Anopheles mosquito. The parasitic infection is endemic in 90 countries, with approximately 500 million cases reported annually and an estimated annual mortality of 1.5-2.7 million individuals. Historically, the use of antimalarial drugs has been promising for the chemoprophylaxis and treatment of malaria, mitigating the annual mortality rate. Notably, these antimalarial drugs have been associated with various adverse effects, including gastrointestinal upset and headaches. However, the adverse cutaneous manifestations these antimalarial drugs may lead to are poorly documented and understood. We aim to describe the lesser-studied adverse cutaneous pathologies of malaria treatment to better educate physicians on the proper treatment of their patients. Our narrative review describes the skin manifestations associated with specific antimalarial treatments and their associated prognoses and treatments. The cutaneous pathologies discussed include aquagenic pruritus (AP), palmoplantar exfoliation, Steven-Johnson syndrome, toxic epidermal necrolysis, cutaneous vasculitis, psoriasis, ecchymosis, and tropical lichenoid dermatitis. Further studies and vigilant documentation of the cutaneous adverse events of antimalarial drugs need to be performed and emphasized to prevent potential life-threatening adverse outcomes.
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  • 文章类型: Journal Article
    随着冠状病毒病(COVID-19)疫苗的继续使用,皮肤科医生在识别和治疗与疫苗相关的皮肤表现(CM)中起着至关重要的作用。文献中报道的COVID-19疫苗的不良皮肤反应范围从常见的荨麻疹到罕见的水疱性反应。在这项研究中,我们进行了(1)范围审查,以评估水泡的发生,丘疹囊泡,和COVID-19疫苗的大疱性CM及其各自的治疗方法,(2)叙述性综述,讨论COVID-19疫苗的其他常见和不常见CM。36篇文章被纳入范围审查,和66篇文章在叙事审查。我们发现囊泡,丘疹囊泡,大疱性病变很少见,报道主要是在第一剂Moderna或辉瑞疫苗之后。36项研究中有11项报告了与带状疱疹病毒的激活或再激活一致的囊泡反应。大多数水疱性和大疱性病变是自限性的或用局部皮质类固醇治疗。其他CM包括注射部位,荨麻疹或麻痹症反应,血管炎,中毒性表皮坏死松解症,以及牛皮癣等新发皮肤病的爆发。CMs的治疗包括局部或口服皮质类固醇,抗组胺药,或者在自我限制的情况下没有治疗。虽然大多数CM是良性的和可治疗的,关于全身性皮质类固醇和免疫抑制疗法对COVID-19疫苗免疫原性的影响的数据有限.一些研究报告了使用大剂量皮质类固醇后疫苗的免疫原性降低。在向免疫抑制患者推荐COVID-19疫苗时,医生可以参考当地指南,以及使用皮质类固醇管理COVID-19疫苗的CM时。
    As coronavirus disease (COVID-19) vaccines continue to be administered, dermatologists play a critical role in recognizing and treating the cutaneous manifestations (CM) associated with the vaccines. Adverse cutaneous reactions of COVID-19 vaccines reported in the literature range from common urticarial to rare vesiculobullous reactions. In this study, we performed a (1) scoping review to assess the occurrences of vesicular, papulovesicular, and bullous CMs of COVID-19 vaccines and their respective treatments, and (2) a narrative review discussing other common and uncommon CMs of COVID-19 vaccines. Thirty-six articles were included in the scoping review, and 66 articles in the narrative review. We found that vesicular, papulovesicular, and bullous lesions are infrequent, reported mostly after the first dose of Moderna or Pfizer vaccines. Eleven of the 36 studies reported vesicular reactions consistent with activation or reactivation of the herpes zoster virus. Most vesicular and bullous lesions were self-limited or treated with topical corticosteroids. Other CMs included injection-site, urticarial or morbilliform reactions, vasculitis, toxic epidermal necrolysis, and flaring of or new-onset skin diseases such as psoriasis. Treatments for CMs included topical or oral corticosteroids, antihistamines, or no treatment in self-limited cases. Although most CMs are benign and treatable, the data on the effect of systemic corticosteroids and immunosuppressive therapies on the immunogenicity of COVID-19 vaccines is limited. Some studies report reduced immunogenicity of the vaccines after high-dose corticosteroids use. Physicians may consult local guidelines where available when recommending COVID-19 vaccines to immunosuppressed patients, and when using corticosteroids to manage the CMs of COVID-19 vaccines.
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  • 文章类型: Journal Article
    色情成瘾是一个越来越令人关注的领域,特别是由于互联网上色情材料的无处不在。即便如此,没有正式的精神疾病诊断和统计手册(DSM-5)将强迫性色情作为行为成瘾。虽然色情成瘾的社会心理影响已经被研究,过度使用色情制品带来的直接皮肤伤害和行为改变的风险仍有待观察。成年男性是从事危险和暴力性行为的大多数案例,与色情消费的关联不清楚。成年女性可能会复制类似于色情内容中看到的阴毛修剪图案,即使对阴唇成形术的态度尚不清楚。最后,经常接触色情制品的青少年被发现复制色情材料中的性活动,并有较早的性活动。在评估的文献中,色情材料与直接皮肤病之间的联系仍然是进一步研究的主要领域。
    Pornography addiction is an area of increasing concern, particularly due to the ubiquitous nature of pornographic material on the Internet. Even so, there is no formal Diagnostic and Statistical Manual of Mental Disorders (DSM-5) inclusion of compulsive pornography use as a behavioral addiction. Although the psychosocial impacts of pornography addiction have been studied, the risk of direct skin injuries and behavioral changes brought about by excessive pornography usage remain to be seen. Adult males constitute the majority of cases of engaging in risky and violent sexual behaviors with an unclear association with pornography consumption. Adult females may be likely to copy pubic hair trimming patterns analogous to those seen in pornographic content, even though attitudes towards labiaplasty are unclear. Finally, adolescents regularly exposed to pornography have been found to replicate sexual activity seen in pornographic material and have earlier sexual activity. In the literature evaluated, an association between pornographic material and direct cutaneous disease remains a major area of further research.
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  • 文章类型: Systematic Review
    2019年冠状病毒病(COVID-19)患者的皮肤表现可能对识别潜在的严重病例具有预后价值。这项系统评价调查了老年患者的皮肤病学特征是否与COVID-19结局相关。文献检索于2022年5月11日从数据库中进行,手工搜索,并追踪引文。根据资格标准进行选择后,剩余的记录使用国家心脏进行评估,肺,和血液研究所研究质量评估工具或纽卡斯尔-渥太华量表。随后使用Cochran-Mantel-Haenszel检验分析合并的个体数据,以计算每种皮肤病变类型的严重程度和死亡率的比值比(OR)和置信区间(CI)。我们纳入了70篇文章,包括180和117个条目,关于疾病严重程度和死亡率的信息。进一步分析显示,血管类型是确诊的COVID-19老年患者中最常见的皮肤病变(46.2%),并与发展为晚期疾病的风险增加(OR7.32,95%CI3.39-15.81)和随后终止疾病的风险增加(OR5.73,95%CI2.46-13.36)相关。在斑丘疹型中观察到相反的现象(严重程度OR0.27,95%CI0.14-0.52;死亡率OR0.10,95%CI0.03-0.36)。总之,皮肤表现可预测老年组COVID-19的严重程度和死亡率.
    Cutaneous manifestations in coronavirus disease 2019 (COVID-19) patients may possess prognostic value for identifying potentially severe cases. This systematic review investigated whether dermatological features are associated with COVID-19 outcomes in elderly patients. Literature retrieval was conducted on May 11, 2022, from databases, hand-searching, and tracing citations. Following selection against eligibility criteria, the remaining records were evaluated utilizing the National Heart, Lung, and Blood Institute Study Quality Assessment Tool or Newcastle-Ottawa Scale. The pooled individual data were subsequently analyzed using the Cochran-Mantel-Haenszel test to calculate the odds ratio (ORs) and confidence intervals (CIs) for the severity and mortality of each skin lesion type. We incorporated 70 articles, including 180 and 117 entries, with information regarding disease severity and mortality. Further analysis revealed that vascular type was the skin lesion most frequently noticed in confirmed COVID-19 elderly patients (46.2%) and was associated with an increased risk of developing advanced disease (OR 7.32, 95% CI 3.39-15.81) and the ensuing termination (OR 5.73, 95% CI 2.46-13.36). The converse phenomenon was observed in maculopapular type (severity OR 0.27, 95% CI 0.14-0.52; mortality OR 0.10, 95% CI 0.03-0.36). In conclusion, skin manifestations may predict COVID-19 severity and mortality in the senior group.
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  • 文章类型: Journal Article
    镰状细胞病是一种常见且高度病态的遗传病,其特征是多系统受累,包括许多皮肤表现。长期以来,持续和复发性的腿部溃疡一直被认为是镰状细胞疾病患者的皮肤病学标志,但是最近有关于罕见病原体感染和肝体血管病变发作的描述。文献中对该人群的考虑越来越多,包括对常见治疗如羟基脲的皮肤反应以及镰状细胞疾病和斑块状牛皮癣等疾病之间交集的临床管理。最近的研究还表明,通过不清楚的机制,镰状细胞疾病患者对皮肤癌变的抵抗力增加。然而,尽管关于镰状细胞病的皮肤表现和考虑的知识体系正在慢慢扩大,这与这些患者面临的相当大的疾病和症状负担不符。需要更多的研究来更好地描述我们对镰状细胞病的这些皮肤表现的理解,以改善结果和进一步的管理。
    Sickle cell disease is a common and highly morbid genetic condition that is characterized by multi-system involvement, including numerous cutaneous manifestations. Persistent and recurrent leg ulceration has long been considered the dermatological hallmark of those with sickle cell disease, but there have been recent descriptions of associated infection with rare pathogens and episodes of liverdoid vasculopathy. Growing considerations for this population in the literature include cutaneous reactions to common treatments such as hydroxyurea and clinical management of the intersection between sickle cell disease and conditions like plaque psoriasis. Recent studies have also demonstrated an increased resistance to skin carcinogenesis for those with sickle cell disease through unclear mechanisms. However, though the body of knowledge regarding cutaneous manifestations and considerations of sickle cell disease is slowly expanding, it does not match the considerable disease and symptom burden faced by these patients. More research is needed to better delineate our understanding of these cutaneous manifestations of sickle cell disease to improve outcomes and further management.
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