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
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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: Journal Article
    红皮银屑病(EP)是一种严重的银屑病,会影响多个器官,包括心血管系统.然而,很少有研究关注这种情况。本研究旨在评估EP患者心力衰竭的患病率和相关因素。并测量成纤维细胞生长因子23(FGF23)的血清浓度,慢性心力衰竭的潜在预测因子。
    我们回顾性研究了2005年1月至2021年10月在北京协和医院住院的EP患者。测量心力衰竭的患病率和相关因素。此外,收集17例患者的外周血样本,并与8名健康对照者的样本相匹配。用ELISA法测定血清FGF23浓度。
    我们研究了225例EP患者,男女比例为2.7:1,平均年龄为47.6±16.7岁。25名(11.1%)参与者在住院期间被诊断为心力衰竭。EP和心力衰竭患者年龄较大(58.2岁vs.46.2年,p=0.001);冠心病病史的患病率较高(32.0%vs.21.5%,p<0.001),发烧(48.0%vs.23.0%,p=0.007),感染(56.0%vs.35.5%,p=0.046);较高的hsCRP浓度(43.2mg/Lvs.8.2mg/L,p=0.005);贫血患病率较高(60.0%vs.22.0%,p<0.001),低蛋白血症(64.0%vs.42.0%,p=0.037),和高脂血症(40.0%vs.20.0%,p=0.023)比没有心力衰竭的人。EP患者的血清FGF23浓度显着高于对照组(493.1pg/mlvs.277.8pg/ml,p=0.027),治疗后显著降低(395.7pg/mlvs.463.1pg/ml,p=0.022)。
    临床医生应该意识到EP患者心力衰竭的风险,尤其是那些高龄和有冠心病史的人,严重的全身症状,高浓度的炎症生物标志物,和营养不良。
    UNASSIGNED: Erythrodermic psoriasis (EP) is a severe form of psoriasis that affects multiple organs, including the cardiovascular system. However, few studies have focused on this condition.This study is aimed to assess the prevalence and factors associated with heart failure in EP patient, and to the measure the serum concentrations of fibroblast growth factor 23 (FGF23), a potential predictor of chronic heart failure.
    UNASSIGNED: We retrospectively studied patients with EP hospitalized at Peking Union Medical College Hospital between January 2005 to October 2021. The prevalence of heart failure and associated factors was measured. In addition, peripheral blood samples were collected from 17 patients and matched with samples from eight healthy controls, and their serum concentrations of FGF23 were measured by ELISA.
    UNASSIGNED: We studied 225 patients with EP, with a male: female ratio of 2.7:1 and a mean age of 47.6 ± 16.7 years. Twenty-five (11.1%) participants were diagnosed with heart failure during their hospital stay. The patients with EP and heart failure were older (58.2 years vs. 46.2 years, p = 0.001); had a higher prevalence of a history of coronary heart disease (32.0% vs. 21.5%, p < 0.001), fever (48.0% vs. 23.0%, p = 0.007), infection (56.0% vs. 35.5%, p = 0.046); higher hsCRP concentration (43.2 mg/L vs. 8.2 mg/L, p = 0.005); and higher prevalence of anemia (60.0% vs. 22.0%, p < 0.001), hypoalbuminemia (64.0% vs. 42.0%, p = 0.037), and hyperlipidemia (40.0% vs. 20.0%, p = 0.023) than those without heart failure. The serum FGF23 concentration was significantly higher in patients with EP than controls (493.1 pg/ml vs. 277.8 pg/ml, p = 0.027), and was significantly lower after treatment (395.7 pg/ml vs. 463.1 pg/ml, p = 0.022).
    UNASSIGNED: Clinicians should be aware of the risk of heart failure in patients with EP, and especially those of advanced age and with a history of coronary heart disease, severe systemic symptoms, high concentrations of inflammatory biomarkers, and poor nutritional status.
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  • 文章类型: Case Reports
    甲基丙二酸血症(MMA)是一种常染色体隐性遗传疾病,由甲基丙二酰辅酶A变位酶活性降低或辅酶钴胺素代谢紊乱引起,皮肤表现是这种疾病的罕见临床体征。在这里,我们描述了一个用缺乏支链氨基酸的配方喂养MMA的中国男孩,表现为红皮病和肢端皮炎肠病样皮疹,纯合无义突变c.742C>T(p。Gln248*)在MMAA基因中鉴定。谱系表现出非孟德尔遗传模式,这归因于先证者4q26-q34.1号染色体上的母本单亲二体,通过染色体微阵列分析证实。我们的案例强调了由于MMA的治疗性氨基酸限制,皮肤变化与营养缺乏之间的关联。
    Methylmalonic acidemia (MMA) is an autosomal recessive genetic disorder caused by decreased activity of methylmalonyl-CoA mutase or metabolic disturbance of its coenzyme cobalamin, cutaneous manifestations are rare clinical signs in this disease. Herein, we describe a Chinese boy with MMA fed with a formula lacking branched-chain amino acids presenting with erythroderma and acrodermatitis enteropathica-like rash, a homozygous nonsense mutation c.742C>T (p.Gln248*) was identified in the MMAA gene. The pedigree exhibited a non-Mendelian inheritance pattern which was attributed to maternal uniparental disomy on chromosome 4q26-q34.1 of the proband, confirmed by chromosomal microarray analysis. Our case highlights the association between skin changes and nutritional deficiency due to therapeutic amino acid restrictions in MMA.
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  • 文章类型: Journal Article
    BACKGROUND: To explore the clinical manifestations, diagnosis, and treatment of patients with acquired immunodeficiency syndrome (AIDS) complicated with drug-induced erythroderma.
    METHODS: The clinical data of 12 AIDS patients with drug-induced erythroderma in our hospital were retrospectively analyzed. The general information, offending medications, complications, modified severity-of-illness score for toxic epidermal necrolysis (SCORTEN) scores, and disease outcome spectrums were analyzed.
    RESULTS: Drug-induced erythroderma was mostly caused by antiviral drugs, antituberculosis drugs, antibiotics, traditional Chinese medicine, and immune checkpoint inhibitors. The spectrum of sensitizing drugs was broad, the clinical situation was complex, and infections were common. The affected areas were greater than 40% body surface area in all patients. The modified SCOTERN score averaged 3.01±0.99. All patients were treated with glucocorticoids, and nine patients were treated with intravenous immunoglobulin (IVIG) pulse therapy at the same time. The average time to effectiveness was 7.08±2.23 days, and the average hospital stay was 17.92±8.46 days. Eleven patients were cured, and one patient died of secondary multiple infections, who had a modified SCORTEN score of 5 points. The mortality rate in this study was 8.3%.
    CONCLUSIONS: The clinical situation of AIDS patients with drug-induced erythroderma in hospitalized patients is complex and the co-infection rate is high. The use of modified SCORTEN score may objectively and accurately assess the conditions, and the use of glucocorticoid combined with IVIG therapy may improve the prognosis.
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  • 文章类型: Journal Article
    克唑替尼是一种多靶点受体酪氨酸激酶抑制剂,对ALK重排的非小细胞肺癌(NSCLC)患者的治疗具有重要意义。据报道,严重的红皮病和中毒性表皮坏死松解症与克唑替尼治疗有关。潜在的机制尚未研究。在这项研究中,我们测试了克唑替尼对永生人角质形成细胞(HaCaT)和人原代角质形成细胞的毒性.我们发现克唑替尼直接对这两种细胞产生细胞毒性,这可能是病理的临床特征的解释。观察到细胞凋亡和Z-VAD-FMK,pan-caspase抑制剂几乎可以完全逆转克唑替尼的凋亡诱导作用.然而,线粒体功能障碍和DNA损伤不参与克唑替尼诱导的细胞凋亡,表明内源性凋亡途径与这种皮肤毒性无关。进一步研究表明,克唑替尼能显著增加外源性凋亡途径的信号蛋白cleaved-caspase-8,以浓度和时间依赖性的方式。此外,我们发现克唑替尼的靶点与HaCaT细胞凋亡无关。总的来说,我们的发现首次报道角质形成细胞凋亡是克唑替尼诱导的皮肤毒性的关键原因。我们还揭示了由于检测到的上调的裂解的半胱天冬酶-8,克唑替尼通过外在凋亡途径诱导凋亡。同时,细胞凋亡独立于线粒体功能障碍,DNA损伤及相关药物靶点抑制。
    Crizotinib is a multi-target receptor tyrosine kinase inhibitor which is of great importance for the management of ALK-rearranged non-small cell lung cancer (NSCLC) patients. Serious erythroderma and toxic epidermal necrolysis have been reported associated with crizotinib treatment. The underlying mechanisms have not been examined. In this study, we tested the toxicity of crizotinib on immortal human keratinocytes (HaCaT) and human primary keratinocytes. We found that crizotinib directly cause cytotoxic on these two cells, which could be the explanation of the clinical characteristic of pathology. Apoptosis was observed and Z-VAD-FMK, a pan-caspase inhibitor can almost totally reverse the apoptosis induction effect of crizotinib. However, mitochondrial dysfunction and DNA damage were not involved in crizotinib-induced apoptosis, indicating the intrinsic apoptosis pathway have no connection with this cutaneous toxicity. Further studies showed that crizotinib significantly increased cleaved-caspase-8, a signaling protein of extrinsic apoptosis pathway, in a concentration and time-dependent manner. Moreover, we found the targets of crizotinib were not involved in HaCaT cells apoptosis. Collectively, our findings first report keratinocytes apoptosis is the key cause of crizotinib-induced cutaneous toxicity. We also reveal crizotinib induce apoptosis through the extrinsic apoptosis pathway due to detected up-regulated cleaved-caspase-8. Meanwhile, the apoptosis is independent of mitochondrial dysfunction, DNA damage and related drug targets inhibition.
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  • 文章类型: Case Reports
    副肿瘤性红皮病的皮肤症状可能是恶性肿瘤的唯一症状。尽管已经报道了许多与恶性肿瘤相关的病例,癌症相关红皮病的发病机制尚不清楚。在此,我们介绍了一名患有肺大细胞神经内分泌癌(LCNEC)和当代严重红皮病的患者。患者出现皮肤红斑和全身鳞屑,手术3周后皮肤病变完全恢复。神经元特异性烯醇化酶(NSE,在术前皮肤的原发癌和基底细胞中均发现2阳性)。三个月后,术后皮肤活检显示接近正常的皮肤组织,伴有NSE阴性表达。手术后九个月,癌症在肝脏和大脑中复发,最初的症状是皮肤红斑和鳞屑。肝活检组织病理显示LCNEC和3+NSE阳性表达。皮肤活检组织显示NSE2+阳性染色。化疗两个周期后的评估显示红皮病明显改善,肿瘤体积减小。然而,由于严重的骨髓抑制而终止化疗时,患者出现红皮病反复恶化。手术后11个月,患者死于癌症恶病质和多器官功能衰竭。据我们所知,这是第一例与肺LCNEC相关的副肿瘤性红皮病。此外,我们首次发现NSE在基底细胞中的沉积可能是红皮病的重要致病因素。
    The cutaneous symptom of the paraneoplastic erythroderma can be the only symptom of a malignancy. Although many cases associated with malignancies have been reported, the pathogenesis of cancer related erythroderma is still unclear. Herein we presented a patient with large cell neuroendocrine carcinoma (LCNEC) of the lung and contemporary severe erythroderma. The patient suffered from skin erythema and scaling all over the body and the cutaneous lesions recovered completely after 3 weeks of surgery. Strong expression of neuron-specific enolase (NSE, 2+ positive) was found in both primary cancer and basal cells of the preoperative skin. Three months later, postoperative skin biopsy presented nearly normal skin tissues, accompanied with a negative expression of NSE. Nine months after surgery, cancer recurred in the liver and brain with the first symptom of skin erythema and scaling. The pathology of liver biopsy tissues illustrated the LCNEC and 3+ positive expression of NSE. The skin biopsy tissues showed 2+ positive stain of NSE. Evaluation after two cycles of chemotherapy showed marked improvement in erythroderma and reduction of tumor volume. However, the patient experienced recurrent worsening of erythroderma when chemotherapy was terminated due to severe myelosuppression. Eleven months after surgery, the patient died of cancer cachexia and multiple organ failure. To our knowledge, this was the first case of paraneoplastic erythroderma associated with LCNEC of lung. Furthermore, we firstly discovered that the deposition of NSE in basal cells might be a crucial pathogenic factor of erythroderma.
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  • 文章类型: Case Reports
    Erythroderma is occasionally associated with lung cancer. Here we report a 69-year-old male who has history of melanoma from 8 years ago presented with erythroderma associated with skin, soft tissue and pulmonary infection. CT chest showed a single enlarging right upper lobe lung nodule. Biopsy showed evidence of adenocarcinoma, and eventually proven to be metastatic melanoma. The patient improved after antibiotics and antifungal treatment. To our knowledge, this is the first case of melanoma metastasis presented as erythroderma as paraneoplastic syndrome.
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