spongiosis

  • 文章类型: Journal Article
    脊椎沉着症定义为表皮中的细胞间水肿和囊泡。组织病理学是诊断海绵状疾病的金标准。湿疹的临床诊断有时不清楚,并与其他皮肤病相混淆;组织病理学通常显示海绵状组织反应模式;此类疾病称为海绵状疾病。对于皮肤科医生来说,使用皮肤镜进行正确的非侵入性诊断是一项挑战,因此我们已经进行了这项研究,以将海绵状疾病的皮肤镜和组织病理学发现相关联,以设定诊断的皮肤镜标准。
    为了研究海绵体病的皮肤镜特征并与临床相关,皮肤镜,和组织病理学发现。
    二百五十二位病人,纳入的病史和临床表现提示湿疹.他们被归类为急性(<6周),亚急性(6周至3个月),和基于持续时间的慢性(>3个月)湿疹。对代表性病变进行皮肤镜检查和皮肤活检。收集数据并使用频率分布和卡方检验进行统计分析。
    我们根据急性,亚急性,慢性有三种模式,临床检查,皮肤镜,和组织病理学。在临床检查中,急性(27.4%),亚急性(42.9%),和慢性(29.7%)皮炎。在皮肤镜上,急性(28.5%),亚急性(40.4%),和慢性(31.1%)皮炎。在组织病理学上,急性(29.5%),亚急性(44.2%),和慢性(26.3%)海绵体。99%的正相关,96.2%,在皮肤镜检查和组织病理学上观察到95%,在急性,亚急性,和慢性湿疹,分别。急性湿疹的皮肤镜检查显示线性血管(100%)和红色背景(100%)。白斑(98.9%)和剥痕(70.1%)。亚急性湿疹的皮肤镜检查显示白色鳞片(99.1%),不规则颜料网络(98.3%),不规则点的血管变化(97.4%),棕白色背景(93.1%),和黑色/棕色/灰色点(91.4%)。慢性湿疹的皮肤镜检查显示为棕白色背景(100%),不规则颜料网络(100%),和黑色/棕色/灰色斑点(100%)。
    与海绵状疾病一致地观察到明确的皮肤镜模式,并且这些可以另外用于设定皮肤镜标准并确认诊断。此外,皮肤镜检查结果与已经确定的组织病理学特征密切相关。
    UNASSIGNED: Spongiosis is defined as intercellular edema and vesicles in the epidermis. Histopathology is the gold standard for the diagnosis of spongiotic disorders. Clinical diagnosis of eczema is sometimes unclear and confused with other dermatoses; histopathology often shows spongiotic tissue reaction patterns; such conditions are called spongiotic disorders. It is challenging for a dermatologist to make the correct diagnosis noninvasively with a dermoscope and thus we have taken up the study to correlate the dermoscopic and histopathological findings in spongiotic disorders to set dermoscopic criteria for the diagnosis.
    UNASSIGNED: To study the dermoscopic features of spongiotic disorders and correlate clinical, dermoscopic, and histopathological findings.
    UNASSIGNED: Two hundred fifty two patients, with history and clinical presentation suggesting eczema were enrolled. They were classified as Acute (<6 weeks), Subacute (6 weeks to 3 months), and Chronic (>3 months) eczemas based on duration. Dermoscopy and skin biopsy were performed on representative lesions. Data were compiled and statistically analyzed using frequency distribution and Chi-square test.
    UNASSIGNED: We correlated the diagnosis based on acute, subacute, and chronic with three modalities, clinical examination, dermoscopy, and histopathology. On clinical examination, acute (27.4%), subacute (42.9%), and chronic (29.7%) dermatitis. On dermoscopy, acute (28.5%), subacute (40.4%), and chronic (31.1%) dermatitis. On histopathology, acute (29.5%), subacute (44.2%), and chronic (26.3%) spongiosis. A positive correlation of 99%, 96.2%, and 95% was observed on dermoscopy and histopathology, in acute, subacute, and chronic eczemas, respectively. Dermoscopy of acute eczemas showed linear vessels (100%) and red background (100%). White-Clods (98.9%) and excoriation marks (70.1%). Dermoscopy of subacute eczemas showed white scales (99.1%), irregular pigment network (98.3%), vascular changes with irregular dots (97.4%), a brown-white background (93.1%), and black/brown/grey dots (91.4%). Dermoscopy of chronic eczema showed brown-white background (100%), irregular pigment network (100%), and black/brown/grey blotches (100%).
    UNASSIGNED: Definitive dermoscopic patterns are observed consistently with spongiotic diseases and these can be used additionally to set dermoscopic criteria and confirm the diagnosis. Also, dermoscopic findings are well correlated with the already established histopathological features.
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  • 文章类型: Journal Article
    特应性皮炎(AD)是一种异质性炎性皮肤病。我们先前的研究表明,在大约60%的AD病例中观察到皮肤中的嗜碱性粒细胞浸润。然而,与嗜碱性粒细胞浸润相关的AD的临床和组织学特征仍不清楚。我们通过免疫组织化学染色检查了2016年4月至2021年9月在东京医科大学附属医院接受皮肤活检以诊断AD的34例患者的38例标本的嗜碱性粒细胞浸润。患者/标本分为两组,17例患者/21例标本与很少或没有嗜碱性粒细胞浸润相关(嗜碱性粒细胞低组),17例患者/17例标本与明显的嗜碱性粒细胞浸润相关(嗜碱性粒细胞高组)。患者的临床特征(年龄,性别,并发症,血液生物标志物,皮肤症状,和治疗)和组间比较标本的组织学特征。高碱性粒细胞患者明显比低碱性粒细胞患者年轻。高嗜碱性粒细胞患者的血液嗜碱性粒细胞计数高于低嗜碱性粒细胞患者。高嗜碱性粒细胞标本中CD4T细胞浸润比低嗜碱性粒细胞标本中更明显。CD4T细胞仅在高嗜碱性粒细胞标本中渗入真皮和表皮。因此,高嗜碱性粒细胞型AD的特征是在年轻患者中与大量辅助性T细胞浸润相关的皮肤病变。
    Atopic dermatitis (AD) is a heterogenous inflammatory skin disorder. Our previous study revealed that basophil infiltration in skin is observed in approximately 60% of AD cases. However, the clinical and histological characteristics of AD associated with basophil infiltration remain unclear. We examined basophil infiltration by immunohistochemical staining of 38 specimens from 34 patients who underwent skin biopsies to diagnose AD from April 2016 to September 2021 at Tokyo Medical and Dental University Hospital. The patients/specimens were divided into two groups, 17 patients/21 specimens associated with little or no basophil infiltration (basophil-low group) and 17 patients/17 specimens associated with marked basophil infiltration (basophil-high group). The clinical characteristics of the patients (age, sex, complications, blood biomarkers, skin symptoms, and treatment) and histological features of the specimens were compared between the groups. Basophil-high patients were significantly younger than basophil-low patients. Blood basophil counts were higher in basophil-high patients than in basophil-low patients. CD4+ T-cell infiltration was more marked in basophil-high specimens than in basophil-low specimens. CD4+ T cells infiltrated into the dermis as well as into the epidermis only in the basophil-high specimens. Thus, basophil-high AD can be characterized by skin lesions associated with abundant helper T-cell infiltration in younger patients.
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  • 文章类型: Journal Article
    消瘦一词是指用于描述以生长迟缓为特征的身体状况的临床体征,通常是多因素起源的。本研究的目的是首次描述一种病理过程,其特征是猪的前脑神经纤维空泡化,表现出浪费而没有明显的神经系统症状。为了表征病变的病理特征,调查了其中八个农场的受影响和未受影响的猪。组织学上,最一致的病变是前脑的神经纤维空泡化,主要位于丘脑核中以及新皮质的白质和灰质之间的过渡区(患病猪40/56,健康猪4/30)。在最严重的情况下,空泡化也涉及中脑,小脑核,在较小程度上,延髓.前脑的空泡化与猪的消瘦和生长迟缓有关。虽然本案的具体原因尚不清楚,在饮用水中预防性使用多种维生素和矿物质复合物改善了病情,强烈暗示了所观察到的状况的代谢起源。
    The term wasting refers to a clinical sign used to describe a physical condition characterized by growth retardation, usually of multifactorial origin. The objective of the present study was to describe for the first time a pathological process characterized by forebrain neuropil vacuolization in pigs showing wasting without conspicuous neurological signs. To characterize the lesions pathologically, affected and non-affected pigs from eight of these farms were investigated. Histologically, the most consistent lesion was neuropil vacuolization of the prosencephalon, mainly located in the thalamic nuclei and in the transition between the white and grey matter of the neocortex (40/56 in sick and 4/30 in healthy pigs). In the most severe cases, the vacuolation also involved the midbrain, cerebellar nuclei and, to a lesser extent, the medulla oblongata. Vacuolization of the forebrain was associated with pigs experiencing marked emaciation and growth retardation. Although the specific cause of the present case remained unknown, the preventive use of multivitamin and mineral complexes in drinking water ameliorated the condition, strongly suggesting a metabolic origin of the observed condition.
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  • 文章类型: Journal Article
    作者描述了外阴高度鳞状上皮内病变(HSIL)与相关表皮水肿的临床病理特征,至少集中,在活检中掩盖它们的基本特征。就各种临床病理特征而言,将11例此类病例(研究组)与33例HSIL病例的对照组进行了比较。在研究组病例中,这些变化是弥漫性的,并累及大部分表皮.低功率印象通常是纤维海绵体(n=11)和/或表面降解(n=3)。在73%的病例中同时存在密集的苔藓样浸润。在6例中,典型的HSIL区域在表皮的基底区域(n=2)或在表皮内(n=4)出现。大功率检查显示了HSIL的特点,在所有情况下,包括嗜铬症和至少中度多态性,但是水肿区通常存在明显减少的非典型性的大区域。在研究组病例中,有丝分裂图明显不明显,尤其是在水肿区。与对照组相比,研究组病例显示出显着的较低的有丝分裂指数,密集苔藓样炎症的频率更高,和较低的上覆角化过度的频率。这两组在各种其他形态学特征方面没有显着差异。两组病例均为p16阻滞阳性,增殖指数较高。研究组和对照组在患者年龄方面没有显着差异,症状持续时间,在出现前一个月使用外阴阴道局部治疗的频率,病灶,硬化性苔藓的病史,或病变的位置。总之,花状水肿可能会掩盖外阴活检中HSIL的基本特征。从业者应该意识到这种潜在的诊断缺陷。
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  • 文章类型: Case Reports
    丙酸血症(PA)是一种常染色体隐性遗传代谢性疾病,由丙酰辅酶A羧化酶(PCC)基因突变引起,影响人体多个系统。这里,我们报告了一名PA患者的神经病理学发现。患者是一名男婴,从产后四天开始,嗜睡和喂养不良。他在三个月大的肝移植后逐渐昏迷并死于并发症。实验室检查结果与PA一致,和遗传分析显示,PCC亚基β基因中的复合杂合突变:c.838dupC(rs769968548)和c.1127G>T(rs142982097)。他死后23小时进行了脑限制性尸检,神经病理学检查显示明显的星形细胞增多,少突胶质细胞损失,神经元丢失,和脑干脱髓鞘,运动皮层,基底神经节,还有丘脑.脊椎沉着症,真空化,并观察到阿尔茨海默氏II型星形胶质细胞和活化的小胶质细胞的出现。这是第一份具有明确遗传原因的PA的大脑尸检报告。
    Propionic acidemia (PA) is an autosomal recessive inheritable metabolic disease caused by mutations in the propionyl CoA carboxylase gene (PCC) that affects multiple systems of the human body. Here, we report neuropathological findings of a PA patient. The patient was a male infant who presented with increasing lethargy and poor feeding from four days postpartum. He gradually became comatose and died from complications after liver transplantation at three months old. The results of laboratory examination were consistent with PA, and genetic analysis revealed compound heterozygous mutations in the gene for PCC subunit beta: c.838dupC (rs769968548) and c.1127G>T (rs142982097). Brain-restricted autopsy was performed 23 h after his death, and the neuropathological examination revealed distinct astrocytosis, oligodendrocytic loss, neuronal loss, and demyelination across the brainstem, motor cortex, basal ganglia, and thalamus. Spongiosis, vacuolization, and the appearance of Alzheimer type II astrocytes and activated microglia were observed as well. This is the first brain autopsy report of PA with a clear genetic cause.
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  • 文章类型: Journal Article
    特应性皮炎(AD)是一种慢性瘙痒性皮肤病,其复杂的发病机制是其异质性的临床表型和基因型。AD的皮肤表现反映了遗传易感性诱导的2型优势免疫轴的细胞因子环境。先天免疫失调,表皮屏障缺损,过敏性炎症。然而,湿疹性皮炎的详细病理机制,这是AD的主要特征,尚不清楚。这篇综述回顾了先前的研究,这些研究表明了该领域的研究进展,并考虑了“海绵状皮炎”的免疫学病理机制,这是湿疹性皮炎的组织病理学标志。该领域的研究揭示了IgE介导的迟发型超敏反应的重要性,Fas/Fas-配体系统,和细胞介导的细胞毒性在海绵状皮炎中诱导角质形成细胞凋亡。最近的研究表明,连同浸润的CD4T细胞,表达IgE的树突状细胞(即炎性树突状表皮细胞和朗格汉斯细胞)捕获特定过敏原(即,屋尘螨)存在于IgE过敏性AD患者的苔藓化湿疹的海绵状表皮中。这些发现表明,IgE介导的迟发型超敏反应在AD皮肤病变中海绵状皮炎的发病机理中起着关键作用。
    Atopic dermatitis (AD) is a chronic pruritic skin disease with a complex pathogenesis underlying its heterogeneous clinical phenotypes and endotypes. The skin manifestation of AD reflects the cytokine milieu of a type-2-dominant immunity axis induced by genetic predisposition, innate immunity dysregulation, epidermal barrier defects, and allergic inflammation. However, the detailed pathomechanism of eczematous dermatitis, which is the principal characteristic of AD, remains unclear. This review examines previous studies demonstrating research progress in this area and considers the immunological pathomechanism of \"spongiotic dermatitis\", which is the histopathological hallmark of eczematous dermatitis. Studies in this field have revealed the importance of IgE-mediated delayed-type hypersensitivity, the Fas/Fas-ligand system, and cell-mediated cytotoxicity in inducing the apoptosis of keratinocytes in spongiotic dermatitis. Recent studies have demonstrated that, together with infiltrating CD4 T cells, IgE-expressing dendritic cells (i.e., inflammatory dendritic epidermal cells and Langerhans cells) that capture specific allergens (i.e., house dust mites) are present in the spongiotic epidermis of lichenified eczema in patients with IgE-allergic AD. These findings suggest that IgE-mediated delayed-type hypersensitivity plays a pivotal role in the pathogenesis of spongiotic dermatitis in the skin lesions of AD.
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  • 文章类型: Journal Article
    天疱疮是由靶向表皮中的桥粒蛋白的自身抗体引起的慢性起泡性疾病。棘管松解可能不存在,天疱疮可能只出现海绵状和囊泡,从而导致湿疹的误诊。在这里,我们做了一个回顾,观察,单中心研究,以建立通过直接免疫荧光证实的天疱疮病例的海绵体病模式。从2001年到2020年免疫病理学诊断的天疱疮标本被检索,并对海绵状体标本进行了以下特征分析:囊泡形成,棘层松解术,海绵状体,表皮中的炎症细胞,真皮发炎.海绵状皮炎病例用作对照。在99个免疫病理学诊断的天疱疮标本中,鉴定出41个海绵状体样品。大约四分之一的标本没有棘皮松解。在病灶周围表皮的中至下三分之一(p=0.030),嗜中性粒细胞或嗜酸性粒细胞的胞吐作用(p=0.016),由淋巴细胞组成的皮肤浸润,嗜酸性粒细胞,和中性粒细胞(p=0.012),与对照组相比,天疱疮中不存在朗格汉斯细胞微脓肿(p<0.001)更为常见。天疱疮中的脊椎病可能与无棘皮松解症患者的湿疹相似。这项研究中微妙的组织学发现提供了诊断线索,并建议应进行进一步的免疫荧光以确认天疱疮的诊断。
    Pemphigus is a chronic blistering disorder caused by autoantibodies that target desmosomal proteins in the epidermis. Acantholysis may be absent, and pemphigus may present only with spongiosis and vesiculation, thereby leading to a misdiagnosis of eczema. Herein, we conducted a retrospective, observational, single-center study to establish a pattern of spongiosis in cases of pemphigus confirmed by direct immunofluorescence. Immunopathologically diagnosed pemphigus specimens from 2001 to 2020 were retrieved, and specimens with spongiosis were analyzed for the following features: vesiculation, acantholysis, spongiosis, inflammatory cells in the epidermis, and inflammation in the dermis. Cases of spongiotic dermatitis were used as control. Out of 99 immunopathologically diagnosed pemphigus specimens, 41 samples with spongiosis were identified. About one quarter of the specimens did not have acantholysis. Spongiosis in the middle to lower thirds of the perilesional epidermis (p = 0.030), exocytosis with either neutrophils or eosinophils (p = 0.016), dermal infiltrates composed of lymphocytes, eosinophils, and neutrophils (p = 0.012), and absence of Langerhans cell microabscesses (p < 0.001) were more common in pemphigus than control. Spongiosis in pemphigus may mimic eczema in patients without acantholysis. The subtle histological findings in this study provide diagnostic clues and suggest that further immunofluorescence should be performed to confirm pemphigus diagnosis.
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  • 文章类型: Case Reports
    天疱疮是一种自身免疫性水疱疾病,其特征是皮肤和粘膜上的病变。迄今为止,在食蟹猴中没有这种疾病的自发病例的报道。本报告描述了食蟹猴自发性天疱疮的组织病理学特征。宏观上,在整个身体的皮肤上观察到发红和瘙痒的鳞屑。组织病理学,表皮显示细胞间水肿,嗜酸性粒细胞和单核细胞浸润表皮。表皮无明显棘皮松解。血管周围区域显示水肿,嗜酸性粒细胞和单核细胞浸润真皮血管。免疫组织化学,表皮中的细胞间区域对免疫球蛋白G和补体成分3呈阳性。血清学上,血清中的抗桥粒蛋白1和桥粒蛋白3抗体均为阴性。从这些发现中,这个病例被诊断为自身免疫性皮肤病,怀疑是天疱疮,并得出结论,病变与人类“疱疹样天疱疮”相似。
    Pemphigus is an autoimmune blistering disease characterized by lesions on the skin and mucous membranes. To date, no spontaneous cases of this disease have been reported in cynomolgus monkeys. This report describes the histopathological characteristics of spontaneous pemphigus in a cynomolgus monkey. Macroscopically, redness and scaling with pruritus were observed on the skin of the entire body. Histopathologically, the epidermis showed intercellular edema, and eosinophils and mononuclear cells infiltrated the epidermis. There was no obvious acantholysis in the epidermis. The perivascular area showed edema, and eosinophils and mononuclear cells infiltrated the vessels in the dermis. Immunohistochemically, the intercellular area in the epidermis was positive for Immunoglobulin G and Complement component 3. Serologically, anti-desmoglein 1 and desmoglein 3 antibodies in the serum were negative. From these findings, this case was diagnosed as an autoimmune skin disease, suspected to be pemphigus, and concluded as lesions being similar to those in human \"pemphigus herpetiformis\".
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  • 文章类型: Journal Article
    Primary mitochondrial diseases are caused by mutations in mitochondrial or nuclear genes, leading to the abnormal function of specific mitochondrial pathways. Mitochondrial dysfunction is also a secondary event in more common pathophysiological conditions, such as obesity and metabolic syndrome. In both cases, the improvement and management of mitochondrial homeostasis remain challenging. Here, we show that beta-resorcylic acid (β-RA), which is a natural phenolic compound, competed in vivo with 4-hydroxybenzoic acid, which is the natural precursor of coenzyme Q biosynthesis. This led to a decrease in demethoxyubiquinone, which is an intermediate metabolite of CoQ biosynthesis that is abnormally accumulated in Coq9R239X mice. As a consequence, β-RA rescued the phenotype of Coq9R239X mice, which is a model of primary mitochondrial encephalopathy. Moreover, we observed that long-term treatment with β-RA also reduced the size and content of the white adipose tissue (WAT) that is normally accumulated during aging in wild-type mice, leading to the prevention of hepatic steatosis and an increase in survival at the elderly stage of life. The reduction in WAT content was due to a decrease in adipogenesis, an adaptation of the mitochondrial proteome in the kidneys, and stimulation of glycolysis and acetyl-CoA metabolism. Therefore, our results demonstrate that β-RA acted through different cellular mechanisms, with effects on mitochondrial metabolism; as such, it may be used for the treatment of primary coenzyme Q deficiency, overweight, and hepatic steatosis.
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  • 文章类型: Journal Article
    BACKGROUND: Inverse psoriasis represents a less commonly described form of psoriasis in intertriginous areas. The pathologic findings of inverse psoriasis are typically grouped in with those of plaque psoriasis, as the histopathologic features specific to inverse psoriasis have not received significant investigation.
    METHODS: A single institution, retrospective cohort study was performed to review biopsy slides for psoriasis occurring in typical intertriginous areas. Patient\'s charts were reviewed and only those where the clinical diagnosis of inverse psoriasis was also favored were included.
    RESULTS: Twelve patients met inclusion criteria: 58% male and 42% female, 18 to 86 years of age. Classic features of psoriasis such as hypogranulosis, confluent parakeratosis, and thinning of the suprapapillary plate were seen in 100%. Regular psoriasiform acanthosis and dilated tortuous dermal vessels were seen in 92%. Neutrophils were present in the scale in 83% and in the dermis in 100%. Features considered atypical for psoriasis included spongiosis in 83%, eosinophils in 67%, and focal serum in the scale in 42%.
    CONCLUSIONS: While inverse psoriasis commonly exhibits features considered to be classic for psoriasis, it is not unusual for inverse psoriasis to show features considered atypical for plaque psoriasis such as dermal eosinophils, epidermal spongiosis, and focal serum in the scale.
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