Erythroderma

红皮病
  • 文章类型: Case Reports
    背景:挪威sc疮(NS)是一种严重的寄生虫性皮肤病。尽管NS是红皮病的原因之一,它经常被忽视。因此,提高对NS表现为红皮病的认识至关重要。
    方法:我们介绍了一例持续3年以上的NS病例。经过非标准治疗后,患者的皮疹恶化并逐渐发展为红皮病。最后,经皮肤镜检和病理确诊为NS。
    结论:当瘙痒性皮肤病患者存在长期卧床和免疫缺陷等高危因素时,临床医生需要提高对NS的认识,并确保及时诊断和治疗。
    BACKGROUND: Norwegian scabies (NS) is a serious parasitic skin condition. Although NS is one of the causes of erythroderma, it is frequently overlooked. Therefore, it is essential to raise awareness regarding NS presenting as erythroderma.
    METHODS: We present a case of NS that persisted for more than 3 years. After following nonstandard treatment, the patient\'s rash worsened and gradually progressed into erythroderma. Finally, NS was diagnosed by skin microscopy and pathology.
    CONCLUSIONS: When patients with pruritic dermatosis have high-risk factors such as prolonged bed rest and immunodeficiency, clinicians need to enhance their awareness of NS and ensure prompt diagnosis and treatment.
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  • 文章类型: Journal Article
    特应性皮炎(AD),牛皮癣,与红皮病相关的药物反应经常因感染而复杂化。然而,血流感染(BSI)受到的研究关注较少。
    本研究旨在探讨红皮病患者与BSI相关的临床特征和危险因素。
    对141例红皮病病例进行回顾性分析。11例患者被确定为患有BSI。回顾并比较了BSI和非BSI组的临床记录。
    在7.80%(11/141)的红皮病病例中诊断出BSI,在AD中分解为7.14%,2.00%的牛皮癣,药物反应占17.14%。值得注意的是,所有阳性皮肤培养物(7/7)均显示细菌分离株与血液培养物一致。单因素logistic回归分析显示与BSI有几个显著关联,包括温度(≤36.0或≥38.5°C;比值比(OR)=28.06;p<0.001),冷却(OR=22.10;p<0.001),肾脏疾病(OR=14.64;p<0.001),药物反应的病因(OR=4.18;p=0.03),白蛋白(ALB)(OR=0.86;p<0.01),C反应蛋白(CRP)(OR=1.01;p=0.02),白细胞介素6(IL-6)(OR=1.02;p=0.02),和降钙素原(PCT)(OR=1.07;p=0.03)。受试者工作特征(ROC)曲线显示与ALB显著相关(p<0.001;曲线下面积(AUC)=0.80),PCT(p=0.009;AUC=0.74),和CRP(p=0.02;AUC=0.71)。
    提高对BSI风险的认识在红皮病管理中至关重要。有特定危险因素的患者,如体温异常(≤36.0或≥38.5℃),令人不寒而栗的感觉,肾病,有药物反应史,CRP升高(≥32mg/L),PCT升高(≥1.00ng/ml),和低白蛋白(≤31.0g/L),需要密切监测BSI的发展。
    UNASSIGNED: Atopic dermatitis (AD), psoriasis, and drug reactions associated with erythroderma are frequently complicated by infections. However, bloodstream infection (BSI) have received less research attention.
    UNASSIGNED: This study aimed to investigate the clinical characteristics and risk factors associated with BSI in patients with erythroderma.
    UNASSIGNED: A retrospective analysis was conducted on 141 erythroderma cases. Eleven cases were identified as having BSI. Clinical records of both BSI and non-BSI groups were reviewed and compared.
    UNASSIGNED: BSI was diagnosed in 7.80% (11/141) of erythroderma cases, with a breakdown of 7.14% in AD, 2.00% in psoriasis, and 17.14% in drug reactions. Notably, all positive skin cultures (7/7) showed bacterial isolates concordant with blood cultures. Univariate logistic regression analysis revealed several significant associations with BSI, including temperature (≤36.0 or ≥38.5 °C; odds ratio (OR) = 28.06; p < 0.001), chilling (OR = 22.10; p < 0.001), kidney disease (OR = 14.64; p < 0.001), etiology of drug reactions (OR = 4.18; p = 0.03), albumin (ALB) (OR = 0.86; p < 0.01), C-reaction protein (CRP) (OR = 1.01; p = 0.02), interleukin 6 (IL-6) (OR = 1.02; p = 0.02), and procalcitonin (PCT) (OR = 1.07; p = 0.03). Receiver operating characteristic (ROC) curves demonstrated significant associations with ALB (p < 0.001; the area under curve (AUC) = 0.80), PCT (p = 0.009; AUC = 0.74), and CRP (p = 0.02; AUC = 0.71).
    UNASSIGNED: Increased awareness of BSI risk is essential in erythroderma management. Patients with specific risk factors, such as abnormal body temperature (≤36.0 or ≥38.5 °C), chilling sensations, kidney disease, a history of drug reactions, elevated CRP (≥32 mg/L), elevated PCT (≥1.00 ng/ml), and low albumin (≤31.0 g/L), require close monitoring for BSI development.
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  • 文章类型: Case Reports
    红皮病,也被称为剥脱性皮炎,是成人发作的斯蒂尔病(AOSD)的罕见非典型皮肤表现。我们介绍了红皮病与AOSD相关的病例,AOSD是类固醇依赖性的,对托珠单抗治疗有反应。皮疹,瘙痒,添加托珠单抗后,相关的实验室检查结果显着改善,而泼尼松龙成功减量至最低维持水平。据我们所知,这是首次报道托珠单抗对AOSD相关红皮病的唯一治疗作用.
    Erythroderma, also known as exfoliative dermatitis, is a rarely reported atypical cutaneous manifestation of adult-onset Still\'s disease (AOSD). We present the case of erythroderma in association with AOSD that was steroid dependent and responded to tocilizumab therapy. Skin rash, pruritis, and related laboratory findings were significantly improved upon the addition of tocilizumab, while prednisolone was successfully tapered to an ever-lowest maintenance level. To our knowledge, this is the first to report the sole therapeutic effect of tocilizumab in erythroderma related to AOSD.
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  • 文章类型: Journal Article
    红皮病是一种以红斑影响至少90%的皮肤表面积为特征的病症。它可能是由各种潜在条件引起的。由于非特异性的临床和实验室检查结果,确定原因可能会带来挑战。在回顾性研究中,我们在皮肤科确定了212例因红皮病住院的患者,性病,2012年1月至2022年3月期间在弗罗茨瓦夫医科大学学习和变态反应学。临床,实验室,和组织病理学特征,以及病人的管理,被研究过。成年人的平均年龄为61岁(IQR=47-68)。红皮病最常见的原因是银屑病(n=49,24.01%),其次是特应性皮炎(AD)(n=27,13.23%),皮肤T细胞淋巴瘤(CTCL)(n=27,13.23%)。尽管进行了实验室检查和组织病理学检查,39例(19.12%)红皮病的病因尚未确定。在70.59%的患者中,这是红皮病的第一次发作,而29.41%的人经历了反复发作。不管红皮病的病因是什么,患者最常接受全身性抗组胺药治疗(146例,71.57%)和全身性类固醇(132例,64.71%)。特发性红皮病患者构成了最大的诊断和治疗挑战,需要特别彻底的评估。
    Erythroderma is a condition characterized by erythema affecting at least 90% of the skin surface area. It can be caused by various underlying conditions. Due to nonspecific clinical and laboratory findings, determining the cause may pose a challenge. In the retrospective study, we identified 212 patients hospitalized for erythroderma in the Department of Dermatology, Venereology, and Allergology at Wroclaw Medical University between January 2012 and March 2022. Clinical, laboratory, and histopathological features, as well as the management of patients, were studied. The median age of adults was 61 years (IQR = 47-68). The most common causes of erythroderma were psoriasis (n = 49, 24.01%), followed by atopic dermatitis (AD) (n = 27, 13.23%), and cutaneous T-cell lymphomas (CTCL) (n = 27, 13.23%). Despite laboratory tests and histopathological examination, the etiology of erythroderma remained undetermined in 39 cases (19.12%). In 70.59% of patients, it was the first episode of erythroderma, while 29.41% experienced a recurrent episode. Regardless of the etiology of erythroderma, patients were most frequently treated with systemic antihistamines (146 cases, 71.57%) and systemic steroids (132 cases, 64.71%). Patients with idiopathic erythroderma constitute the greatest diagnostic and therapeutic challenge, requiring particularly thorough evaluation.
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  • 文章类型: Case Reports
    人类免疫缺陷病毒(HIV)相关的CD8T细胞皮肤浸润性疾病伴严重红皮病的报道很少。虽然HIV阳性患者容易患上淋巴瘤,通常与爱泼斯坦-巴尔病毒有关,多形性淋巴增生性疾病是罕见的,占病例的5%。我们在此报告一名41岁的HIV阳性男子,他出现了发烧,红皮病,和淋巴结病,并被诊断为两种疾病并存。通过持续的抗逆转录病毒治疗,他的病情明显改善。这种情况表明,HIV诱导的免疫缺陷对两种实体的发病机理至关重要,并且改善免疫缺陷状态是有效的治疗方法。
    Human immunodeficiency virus (HIV)-associated CD8+ T-cell skin infiltrative disease with severe erythroderma has rarely been reported. While HIV-positive patients are prone to develop lymphoma, which is often associated with Epstein-Barr virus, polymorphic lymphoproliferative disorder is rare, accounting for <5% of cases. We herein report a 41-year-old HIV-positive man who presented with a fever, erythroderma, and lymphadenopathy and was diagnosed with the coexistence of both diseases. His condition improved significantly with continued antiretroviral therapy. This case suggests that HIV-induced immunodeficiency is central to the pathogenesis of both entities and that improvement of the immunodeficient state is an effective treatment.
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  • 文章类型: Journal Article
    红皮银屑病是一种急性炎症性疾病,在有寻常型银屑病病史的患者中表现为红斑和鳞屑,累及超过90%的体表面积。如果不及时治疗,在这种情况下,急性皮肤衰竭引起的代谢并发症和感染会导致大量的发病率和死亡率。白细胞介素-17(IL-17)被认为是在牛皮癣中引发炎症级联反应的关键角色。IL-17阻断剂已成功用于寻常型银屑病的治疗。然而,其在不稳定型红皮病型银屑病中的应用仅限于孤立病例报告。
    我们在此报告一项观察性研究,9例不稳定型银屑病红皮病患者通过注射苏金单抗成功治疗,并在接下来的24个月内进行随访。
    在研究期间对9名患者进行了管理,所有患者均获得了成功的结果.在第4周,牛皮癣面积和严重程度指数的反应率从基线提高了至少75%,为33.3%(3/9),在第12周提高到88.9%(8/9)。随访24个月,所有患者均未出现红皮病复发。
    该研究得出结论,苏金单抗是快速的,安全,对银屑病红皮病有效,在24个月的随访中,所有患者均未出现红皮病复发。
    UNASSIGNED: Erythrodermic psoriasis is an acute inflammatory condition presenting as erythema and scaling involving more than 90% of body surface area in patients with a history of psoriasis vulgaris. If not treated promptly, metabolic complications and infections due to acute skin failure can cause significant morbidity and mortality in this condition. Interleukin-17 (IL-17) is considered to be the key player in initiating the inflammatory cascade in psoriasis. IL-17 blockers have been successfully used in the management of psoriasis vulgaris. However, its use in unstable erythrodermic psoriasis is limited to isolated case reports.
    UNASSIGNED: We hereby report an observational study of nine patients of unstable psoriatic erythroderma successfully managed with injection secukinumab and followed up over the next 24 months.
    UNASSIGNED: Nine patients were managed during the study period, and a successful outcome was noted in all the patients. The Psoriasis Area and Severity Index response rate improved by at least 75% from baseline in 33.3% (3⁄9) at week 4 and improved to 88.9% (8⁄9) at week 12. None of the patients had a recurrence of erythroderma till 24 months of followup.
    UNASSIGNED: The study concluded that secukinumab is quick, safe, and efficient in psoriatic erythroderma, and there was no relapse of erythroderma in any of the patients in the 24 months of followup.
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  • 文章类型: Journal Article
    仅根据临床表现很难诊断红皮病的根本原因。皮肤镜检查在诊断各种病因继发的红皮病中的作用正在发展。
    本研究旨在观察不同皮肤疾病继发红皮病的皮肤镜特征,并将其与临床特征和组织病理学进行比较。
    29名连续的红皮病患者被纳入研究。使用HeineDeltaII皮肤镜在偏振模式下以10倍放大倍数对每种情况进行皮肤镜检查。进行组织病理学检查以确认诊断。
    8名患者被诊断为牛皮癣,五个有内源性湿疹,四个患有发痒糠疹(PRP),三个患有脓疱型牛皮癣,两例继发于抗结核治疗的药疹,两个皮肤真菌感染,每个特应性皮炎患者,结痂的疮,天疱疮,嗜酸粒细胞增多和全身症状的药物反应,和霉菌病。在牛皮癣引起的红皮症中观察到特征性皮肤镜特征,PRP,脓疱型银屑病,内源性湿疹,疮,和皮肤癣菌病。仅根据皮肤镜检查很难区分其他疾病,临床-组织病理学相关性对于诊断至关重要。
    即使在相应的红皮病或不稳定阶段,经典皮肤病的皮肤镜特征也得以保留。银屑病继发红皮病的皮肤镜特征,脓疱型银屑病,PRP,内源性湿疹,疮,和皮肤癣菌病有明显的区别,而红皮病的其他原因的皮肤镜特征是重叠的。因此,皮肤镜检查可以作为临床评估红皮病的良好筛查工具。
    UNASSIGNED: It is difficult to diagnose the underlying cause of erythroderma on mere clinical presentation. The role of dermoscopy in diagnosing erythroderma secondary to various etiologies is evolving.
    UNASSIGNED: This study aimed to observe the dermoscopic features of erythroderma secondary to different cutaneous disorders and compare them with clinical features and histopathology.
    UNASSIGNED: Twenty-nine consecutive patients of erythroderma were enrolled in the study. Dermoscopy was performed on every case using a Heine Delta II Dermatoscope with 10x magnification in polarized mode. A histopathological examination was conducted to confirm the diagnosis.
    UNASSIGNED: Eight patients were diagnosed with psoriasis, five with endogenous eczema, four with pityriasis rubra pilaris (PRP), three with pustular psoriasis, two with drug rash secondary to antitubercular therapy, two with dermatophytic infection, one patient each of atopic dermatitis, crusted scabies, pemphigus foliaceous, drug reaction with eosinophilia and systemic symptoms, and mycosis fungoides. Characteristic dermoscopic features were observed in erythroderma due to psoriasis, PRP, pustular psoriasis, endogenous eczema, scabies, and dermatophytosis. Differentiation of other disorders based on dermoscopy alone was difficult, and clinico-histopathological correlation was crucial to reach a diagnosis.
    UNASSIGNED: Dermoscopic features of classical patterns of skin disorders are preserved even in the corresponding erythrodermic or unstable stage. Dermoscopic features of erythroderma secondary to psoriasis, pustular psoriasis, PRP, endogenous eczema, scabies, and dermatophytosis are clearly differentiating, whereas the dermoscopic features in other causes of erythroderma are overlapping. Thus, dermoscopy can be a good screening tool in the clinical assessment of erythroderma.
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  • 文章类型: Review
    背景:皮肤癣菌是世界范围内最常见的真菌病病原体,通常会引起浅表感染。然而,它们可以进入真皮深处,导致侵袭性皮肤癣菌病,如在罕见情况下更深的真皮皮肤癣菌病。红皮病是各种疾病的严重皮肤病学表现,导致全身皮肤发红,但是由于真菌感染引起的红皮病几乎没有报道。在这篇文章中,我们报告了首例红皮病合并红色毛癣菌引起的深层皮肤真菌病(T。rubrum)在重症肌无力患者中。
    方法:一名48岁的男子因身体上有鳞屑和结节的红斑住院一个月。该患者有重症肌无力病史,通过定期服用泼尼松龙控制超过10年,并伴有甲癣和足癣持续超过8年。根据组织病理学检查,真菌培养物,和DNA测序结果,该患者最终被诊断为皮肤癣菌引起的红皮病合并红斑T.rubrum引起的真皮深层皮肤癣菌病。抗真菌治疗2周后,病人恢复良好。
    结论:本病例报告显示,具有长期浅表真菌病病史的免疫抑制患者倾向于发生侵袭性皮肤癣菌感染或播散性真菌感染的风险更高。皮肤科医生应警惕这种情况,并及时治疗浅表性皮肤癣菌病。
    BACKGROUND: Dermatophytes are the most common causative pathogens of mycoses worldwide and usually cause superficial infections. However, they can enter deep into the dermis lead to invasive dermatophytosis such as deeper dermal dermatophytosis on rare occasions. Erythroderma is a severe dermatological manifestation of various diseases resulting in generalized skin redness, but erythroderma due to fungi infections is barely reported. In this article, we reported the first case of erythroderma combined with deeper dermal dermatophytosis due to Trichophyton rubrum (T. rubrum) in a patient with myasthenia gravis.
    METHODS: A 48-year-old man was hospitalized because of erythema with scaling and nodules covering his body for a month. The patient had a history of myasthenia gravis controlled by regularly taking prednisolone for > 10 years and accompanied by onychomycosis and tinea pedis lasting > 8 years. Based on histopathological examinations, fungal cultures, and DNA sequencing results, the patient was finally diagnosed with dermatophyte-induced erythroderma combined with deeper dermal dermatophytosis caused by T. rubrum. After 2 weeks of antifungal treatment, the patient had recovered well.
    CONCLUSIONS: This case report shows that immunosuppressed patients with long histories of superficial mycoses tend to have a higher risk of developing invasive dermatophytic infections or disseminated fungal infections. Dermatologists should be alert to this condition and promptly treat the superficial dermatophytosis.
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  • 文章类型: Case Reports
    红皮病是一个用于描述严重,强烈的皮肤炎症。当与剥脱相关时,该病症也被称为剥脱性皮炎。红皮病有很多原因,如不良药疹,皮炎,牛皮癣,发痒糠疹,免疫性疾病,皮肤T细胞淋巴瘤(Sézary综合征),潜在的系统性恶性肿瘤,移植物抗宿主病,和艾滋病毒感染。许多药物会导致红皮病,包括抗生素,抗癫痫药,血管紧张素转换酶(ACE)抑制剂,和磺胺类药物.这里,我们报告了一例罕见的继发于格列齐特的红皮病,口服抗糖尿病药.这个演讲很少见,因为我们在文献中发现只有一例格列齐特引起红皮病的病例报告。红皮病被认为是需要立即诊断和迅速处理的医疗紧急情况;因此,早期干预应该在怀疑的情况下开始,而不等待皮肤科医生的确认,因为这将大大降低这种可能危及生命的紧急情况的死亡率和发病率。
    Erythroderma is a general term used to describe severe, intense skin inflammation. The condition is also known as exfoliative dermatitis when it is associated with exfoliation. Erythroderma has many causes, such as adverse drug eruption, dermatitis, psoriasis, pityriasis rubra pilaris, immunobullous disease, cutaneous T-cell lymphoma (Sézary syndrome), underlying systemic malignancy, graft versus host disease, and HIV infection. Many medications can cause erythroderma, including antibiotics, antiepileptics, angiotensin-converting enzyme (ACE) inhibitors, and sulfonamides. Here, we report a rare case of erythroderma secondary to gliclazide, an oral antidiabetic. This presentation is rare, as we found only one case report of gliclazide causing erythroderma in the literature. Erythroderma is considered a medical emergency requiring immediate diagnosis and prompt management; therefore, early intervention should start on suspicion without waiting for dermatologist confirmation, as this will significantly reduce the mortality and morbidity of this potentially life-threatening emergency.
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  • 文章类型: Journal Article
    鱼鳞病是由遗传决定的表皮角质化疾病,其特征是存在不同程度的鳞屑,角化过度,和红皮病通常与掌足底角化病有关。已经提出了对这些疾病的不同分类,通常基于涉及的基因和/或临床表现。这些疾病的临床特征在不同的遗传实体中表现出一些表型重叠。主要取决于突变的外显率。在这项研究中,使用临床,遗传,和分子方法,我们分析了一个有两个受影响成员的家庭,他们的临床和组织学特征类似于变异型红皮角化症(EKV)或一种红皮过度角化症伴掌plant角化症。尽管有临床表现,我们证明了受影响的患者在ABCA12基因中的两个不同突变是遗传双杂合子,已知与丑角鱼鳞病有关。为了解释我们患者的轻度表型,我们对皮肤进行了分子表征。在表皮的上层,结果表明,葡萄糖基神经酰胺(GlcCer)的片状存在,它是ABCA12转运的脂质,对皮肤不通透性有重要作用。的确,检测到的两个突变并没有完全消除ABCA12活性,表明轻度表型是由于酶功能的部分丧失,从而产生类似EKVP的中间表型,由于GlcCer沉积物的部分消耗。
    Ichthyoses are genetically determined cornification disorders of the epidermis characterized by the presence of different degrees of scaling, hyperkeratosis, and erythroderma often associated with palmoplantar keratoderma. Different classifications of these diseases have been proposed, often based upon the involved genes and/or the clinical presentation. The clinical features of these diseases present some overlap of phenotypes among distinct genetic entities, depending mainly on the penetrance of mutations. In this study, using a clinical, genetic, and molecular approach, we analyzed a family with two affected members who had clinical and histological features resembling erythrokeratodermia variabilis (EKV) or a type of erythrodermic hyperkeratosis with palmoplantar keratoderma. Despite of the clinical presentation, we demonstrated that the affected patients were genetically double heterozygous for two different mutations in the ABCA12 gene, known to be responsible for harlequin ichthyosis. To explain the mild phenotype of our patients, we performed a molecular characterization of the skin. In the upper layers of the epidermis, the results showed a patchy presence of the glucosyl-ceramides (GlcCer), which is the lipid transported by ABCA12, fundamental in contributing to skin impermeability. Indeed, the two mutations detected do not completely abolish ABCA12 activity, indicating that the mild phenotype is due to a partial loss of function of the enzyme, thus giving rise to an intermediate phenotype resembling EKVP, due to a partial depletion of GlcCer deposition.
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