Acantholysis

棘层松解
  • 文章类型: Journal Article
    天疱疮是一种影响皮肤和粘膜的自身免疫性疾病,由天疱疮IgG的沉积诱导,其主要靶向桥粒蛋白1和3(Dsg1和3)。这种自身抗体导致Dsg1和3之间的空间干扰和细胞粘附的丧失,产生棘皮松解.该分子及其细胞效应在临床上反映为表皮内起泡。寻常型天疱疮IgG(PV-IgG)结合涉及p38MAPK信号传导依赖性胱天蛋白酶-3激活。本工作评估了PV-IgG对依赖于caspase-3的HaCaT细胞粘附的体外作用。PV-IgG诱导的细胞脱离和凋亡变化,如膜联蛋白荧光测定法所示。在用caspase-3-shRNA预处理的细胞中,PV-IgG诱导的caspase-3的作用受到抑制,和正常IgG(N-IgG)作为对照对上述参数没有相关影响。结果表明,shRNA降低了caspase-3的表达,如通过qRT-PCR和通过蛋白质印迹和免疫荧光测量,并增加细胞粘附。总之,shRNA防止了体外细胞脱离和PV-IgG诱导的凋亡对HaCaT细胞的后期影响,进一步了解caspase-3细胞粘附依赖性在天疱疮疾病中的分子作用。
    Pemphigus is an autoimmune disease that affects the skin and mucous membranes, induced by the deposition of pemphigus IgG, which mainly targets desmogleins 1 and 3 (Dsg1 and 3). This autoantibody causes steric interference between Dsg1 and 3 and the loss of cell adhesion, producing acantholysis. This molecule and its cellular effects are clinically reflected as intraepidermal blistering. Pemphigus vulgaris-IgG (PV-IgG) binding involves p38MAPK-signaling-dependent caspase-3 activation. The present work assessed the in vitro effect of PV-IgG on the adherence of HaCaT cells dependent on caspase-3. PV-IgG induced cell detachment and apoptotic changes, as demonstrated by annexin fluorescent assays. The effect of caspase-3 induced by PV-IgG was suppressed in cells pre-treated with caspase-3-shRNA, and normal IgG (N-IgG) as a control had no relevant effects on the aforementioned parameters. The results demonstrated that shRNA reduces caspase-3 expression, as measured via qRT-PCR and via Western blot and immunofluorescence, and increases cell adhesion. In conclusion, shRNA prevented in vitro cell detachment and the late effects of apoptosis induced by PV-IgG on HaCaT cells, furthering our understanding of the molecular role of caspase-3 cell adhesion dependence in pemphigus disease.
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  • 文章类型: Case Reports
    我们介绍了两名患有瘙痒的中年患者,硬皮头皮糜烂。皮肤活检在直接免疫荧光上显示表皮棘皮松解伴IgG和C3细胞间沉积物,导致诊断局限性寻常型天疱疮。低剂量口服泼尼松和病灶内曲安奈德后,病变无复发。
    We present two middle-aged patients with pruritic, crusted scalp erosions. Skin biopsy showed epidermal acantholysis with IgG and C3 intercellular deposits on direct immunofluorescence, leading to the diagnosis of localized pemphigus vulgaris. Resolution of the lesions without relapse occurred after low doses of oral prednisone and intralesional triamcinolone acetonide.
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  • 文章类型: Case Reports
    红斑天疱疮是一种罕见的自身免疫性大疱性皮肤病,组织学,和与红斑狼疮和天疱疮重叠的血清学特征。自身抗原是角质形成细胞中的桥粒蛋白3,桥粒蛋白1和桥粒粘附蛋白。当这些债券被破坏时,它导致角质形成细胞的棘皮松解,导致层之间的流体收集。因此,患者将在临床上出现小的松弛性大疱,结痂和鳞屑,主要在脂溢区。我们报告了一个21岁的女性出现多个角化过度斑块的病例,主要是在脂溢区,包括脸,胸部,和肘部。对患者进行了进一步评估,根据临床和实验室调查,诊断与抗双链脱氧核糖核酸(抗-dSDNA)和抗核抗体(ANA)阳性相关的天疱疮.然后使用免疫抑制剂治疗患者,整个过程在这个案例报告中已经详细说明了。
    Pemphigus erythematosus is an uncommon autoimmune bullous skin disorder with clinical, histological, and serological characteristics that overlap with lupus erythematosus and pemphigus foliaceus. The autoantigens are desmoglein 3, desmoglein 1, and desmosomal adhesion proteins in keratinocytes. When these bonds are disrupted, it causes acantholysis of keratinocytes, leading to the fluid collection between layers. Hence, the patient will present clinically with small flaccid bullae with crusting and scaling, mainly on the seborrheic areas. We report the case of a 21-year-old female presenting to us with multiple hyperkeratotic plaques, mainly on the seborrheic areas, including the face, chest, and elbows. The patient was evaluated further, and based on clinical and laboratory investigations, the diagnosis of pemphigus erythematosus associated with anti-double-stranded deoxyribonucleic acid (anti-dSDNA) and anti-nuclear antibody (ANA) positivity was made. The patient was then managed using immunosuppressant therapy, and the entire course has been detailed in this case report.
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    短暂性棘层松解性皮肤病,也被称为格罗弗病,是一种获得性皮肤病,其特征是瘙痒的突然出现,红斑丘疹,或者囊泡,主要影响树干。最常见于50岁以上的男性。组织学通常显示表皮内的局灶性棘皮松解的模式,变性细胞,包括军团和谷物,和上部真皮中可变的血管周围淋巴细胞浸润。虽然它的病因还不是很清楚,公认的触发因素包括过热,出汗,阳光照射,和某些药物,如化疗药物。最近,在严重急性呼吸综合征冠状病毒2(SARS-CoV-2)感染患者和COVID-19疫苗接种后,已经描述了格罗弗病和格罗弗样皮肤疹的单独报告。我们报告了一名65岁男子的病例,该男子接受二级护理,有9天的胸部和背部剧烈瘙痒皮疹史。关于内部医疗检查,他被发现患有SARS-CoV-2感染,并因2019年冠状病毒病(COVID-19)相关肺炎而迅速恶化,需要住院10天进行支持性护理。他的皮肤病变的诊断检查证实了短暂性棘皮松解性皮肤病(格罗弗病),在口服皮质类固醇一个疗程后解决。这一病例强调了格罗弗的疾病与COVID-19之间的罕见但重要的关联,为不断发展的关于SARS-CoV-2感染相关皮肤病变的文献提供了有价值的见解,并强调了考虑将SARS-CoV-2筛查作为对出现Grover样皮疹的患者的诊断检查的一部分的重要性。
    Transient acantholytic dermatosis, also known as Grover\'s disease, is an acquired dermatological condition characterised by the sudden emergence of pruritic, erythematous papules, or vesicles, primarily affecting the trunk. It is observed most commonly in men older than 50 years. Histology typically demonstrates a pattern of focal acantholysis within the epidermis, dyskeratotic cells including corps ronds and grains, and a variable perivascular lymphocytic infiltrate in the upper dermis. While its aetiology is not well understood, recognised triggers include excessive heat, sweating, sun exposure, and certain drugs, such as chemotherapy agents. More recently, isolated reports of Grover\'s disease and Grover-like skin eruptions have been described in patients with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and following COVID-19 vaccination. We report the case of a 65-year-old man who presented to secondary care with a nine-day history of an intensely pruritic rash over his chest and back. On internal medical workup, he was found to have SARS-CoV-2 infection and rapidly deteriorated due to coronavirus disease 2019 (COVID-19)-associated pneumonia, necessitating a 10-day hospital admission for supportive care. Diagnostic workup of his skin lesions confirmed transient acantholytic dermatosis (Grover\'s disease), which resolved following a course of oral corticosteroids. This case underscores the rare but significant association between Grover\'s disease and COVID-19, contributing valuable insights to the evolving body of literature on cutaneous lesions associated with SARS-CoV-2 infection, and highlighting the importance of considering SARS-CoV-2 screening as part of the diagnostic workup for patients presenting with Grover-like skin eruptions.
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  • 文章类型: Case Reports
    脂溢性天疱疮(SP)代表了天疱疮(PF)的局部和表面形式,由于临床相似性,通常被误认为是其他皮肤病,例如脂溢性皮炎(SD)。此外,由于历史术语和重叠的临床特征,SP可能在概念上与红斑天疱疮(PE)混淆。我们提供了一个案例研究,该案例研究是一名38岁的女性,最初被诊断为SD,但后来通过详细的临床和组织病理学分析确定为SP。我们讨论了准确诊断SP的挑战,强调将其与PE和其他棘皮松解性皮肤病区分开的重要性。此外,我们强调了局部治疗在管理SP方面的有效性,与PE通常需要的全身治疗相反。我们的发现强调了进一步研究以优化SP管理策略的必要性,并强调了精确术语在临床实践和研究中的重要性。
    Seborrheic pemphigus (SP) represents a localized and superficial form of pemphigus foliaceus (PF) often mistaken for other dermatological conditions such as seborrheic dermatitis (SD) due to clinical similarities. Additionally, SP may be conceptually confused with pemphigus erythematosus (PE) due to historical terminology and overlapping clinical features. We present a case study of a 38-year-old female initially diagnosed with SD but later identified as SP through detailed clinical and histopathological analysis. We discuss the challenges in accurately diagnosing SP, emphasizing the importance of distinguishing it from PE and other acantholytic dermatoses. Furthermore, we highlight the effectiveness of topical treatment in managing SP, contrary to the systemic therapy often required for PE. Our findings underscore the necessity for further research to optimize management strategies for SP and emphasize the significance of precise terminology in clinical practice and research.
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  • 文章类型: Journal Article
    达里病(DD)是一种罕见的遗传性皮肤病。关于这一主题的文献绝大多数以罕见临床表现的病例报告为主,其中简要提到了组织病理学特征。这项研究的目的是记录DD的组织病理学。根据12年的临床病理相关性,对诊断为Darier病的皮肤活检进行了各种表皮和真皮特征的回顾。包括16名患者,最常出现在第三个十年,轻微的女性偏爱。最常见的临床表现是色素沉着过度,过度角化,丘疹和斑块(91%),69%的人影响躯干。除了经典的鼻上棘皮裂隙,我们注意到一些不寻常的特征:没有角化不全(19%),角状薄片状图案(62%),滤泡棘皮松解(13%)和单个活检中的多个受累灶(63%)。诸如生殖异常细胞的存在和最小的真皮淋巴细胞浸润等特征与先前的文献一致。本研究的局限性在于样本量小。最后,病理学家必须意识到Darier病的组织病理学变化,特别是当遇到非典型临床表现时。达里尔样模式在几种棘皮松解疾病中相遇,临床病理相关性是最终诊断的关键。
    UNASSIGNED: Darier disease (DD) is a rare genodermatosis. Literature on this topic is overwhelmingly dominated by case reports with rare clinical presentations, which have mentioned the histopathologic features briefly. The aim of this study was to document the histopathology of DD. Skin biopsies diagnosed as Darier disease based on clinicopathologic correlation over 12 years were reviewed for various epidermal and dermal features. There were 16 patients included, who most commonly presented in the third decade, with slight female predilection. The most common clinical presentation was hyperpigmented, hyperkeratotic, papules and plaques (91%), with 69% affecting the trunk. In addition to the classic suprabasal acantholytic clefts, we noted some unusual features: absence of parakeratosis (19%), a cornoid lamella-like pattern (62%), follicular acantholysis (13%) and multiple foci of involvement within a single biopsy (63%). Features such as the presence of dyskeratotic cells and minimal dermal lymphocytic infiltrates were concordant with previous literature. The limitation of this study was the small sample size. To conclude, pathologists must be aware of the variations in histopathology of Darier\'s disease, especially when challenged with atypical clinical presentations. The Darier-like pattern is met within several acantholytic diseases, and clinicopathologic correlation has the last word in arriving at a diagnosis.
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  • 文章类型: Case Reports
    寻常型天疱疮,一种罕见的危及生命的自身免疫性疾病,表现为疼痛的皮肤和粘膜病变,导致棘皮松解引起的起泡疮。这项研究深入研究了临床表现,危险因素,以及与寻常型天疱疮相关的诊断复杂性,重点是一个代表性的案例,强调其认可和管理方面的挑战。我们探索了一个60岁男性寻常型天疱疮的病例,最初的表现包括头皮上的黄结皮病变,进展为胸部的非瘙痒病变,脖子,和腹股沟区。进行了多学科医学检查,包括血清学测试,成像,以及传染病和皮肤病专家的咨询。通过三个4毫米穿孔活检的组织病理学检查确定了明确的诊断。该病例强调了寻常型天疱疮的多态性质,具有不同的临床表现和诊断挑战。胸部和颈部病变上的Nikolsky信号阳性,加上在常规牙科手术中观察到的口腔粘膜受累,例证了其表现形式的复杂性。复杂的诊断涉及传染病的阴性结果,肾功能下降,炎症标志物升高,需要一种协作方法来进行准确的诊断。寻常型天疱疮需要全面了解其各种介绍和医学专业之间的合作,以进行准确的诊断和量身定制的管理。治疗涉及全身性糖皮质激素和免疫调节剂。该病例强调需要继续研究以提高这种罕见的自身免疫性疾病的诊断准确性并完善治疗干预措施。
    Pemphigus vulgaris, a rare and life-threatening autoimmune disorder, presents with painful skin and mucosal lesions, leading to blistering sores attributed to acantholysis. This study delves into the clinical manifestations, risk factors, and diagnostic intricacies associated with pemphigus vulgaris, with a focus on a representative case highlighting the challenges in its recognition and management. We explore the case of a 60-year-old male with pemphigus vulgaris, whose initial presentation involved yellow-crusting lesions on the scalp progressing to non-pruritic lesions on the chest, neck, and inguinal areas. A multidisciplinary medical workup was conducted, encompassing serological tests, imaging, and consultations with infectious disease and dermatology specialists. The definitive diagnosis was established through histopathological examination of three 4-mm punch biopsies. The case underscores the polymorphic nature of pemphigus vulgaris, with diverse clinical presentations and diagnostic challenges. The positive Nikolsky sign on the chest and neck lesions, coupled with oral mucosal involvement observed during a routine dental procedure, exemplifies the complexity of its manifestations. Diagnostic intricacies involved negative results for infectious diseases, declined kidney function, and elevated inflammatory markers, necessitating a collaborative approach for accurate diagnosis. Pemphigus vulgaris demands a comprehensive understanding of its varied presentations and collaboration among medical specialties for accurate diagnosis and tailored management. Treatment involves systemic glucocorticoids and immunomodulators. The presented case underscores the need for continued research to enhance diagnostic accuracy and refine therapeutic interventions for this rare autoimmune disorder.
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  • 文章类型: Journal Article
    关于口腔中鳞状细胞癌的透明细胞变体的报道仅有限。本研究涉及显示口腔鳞状细胞癌的透明细胞和棘皮变体的组织病理学特征的病例。已对文献进行了回顾,以了解这些变化的发病机理。此外,已经给出了一个假设,即明确的细胞变化可能是棘皮分解过程级联的结果,而不是一个单独的实体。因此,需要更多的研究来证实这一假设并了解病变的预后。
    Only limited cases have been reported about the clear cell variant of squamous cell carcinoma occurring in the oral cavity. The present study regards the case showing the histopathological features of both the clear cell and acantholytic variants of oral squamous cell carcinoma. A review of the literature has been done to understand the pathogenesis of those changes. Also, a hypothesis has been given that the clear cell changes could be the consequences of the cascades of the acantholytic process and not a separate entity. Therefore, more research is required to confirm this hypothesis and understand the prognosis of the lesion.
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  • 文章类型: Journal Article
    Galli-Galli病(GGD)是一种罕见的遗传性皮肤病,表现出常染色体显性遗传,外显率可变。GGD通常表现为红斑,丘疹,弯曲区域的网状色素沉着过度。存在一个独特的非典型变体,主要在树干上有棕色斑疹,下肢,和四肢,在弯曲区域明显没有标志性的网状色素沉着。这篇综述包括详细的文献检索,并审查了自1982年GGD首次描述以来的案例。它旨在综合当前关于GGD的知识,涵盖其病因,临床表现,组织病理学,诊断,和治疗。这篇综述的一个重要方面是对遗传的探索,组织病理学,GGD和Dowling-Degos病(DDD)之间的临床相似之处,这是另一种罕见的常染色体显性遗传病,特别关注它们在KRT5和POGLUT1基因中的共有突变。这支持了GGD和DDD可能是相同病理条件下不同表型表达的假设。尽管它们传统上被认为是独立的实体,上棘皮松解是GGD的显着特征。最后,这篇综述讨论了现有的治疗方法,强调缺乏既定的指导方针和各种治疗方法的有效性有限。
    Galli-Galli disease (GGD) is a rare genodermatosis that exhibits autosomal dominant inheritance with variable penetrance. GGD typically manifests with erythematous macules, papules, and reticulate hyperpigmentation in flexural areas. A distinct atypical variant exists, which features brown macules predominantly on the trunk, lower limbs, and extremities, with a notable absence of the hallmark reticulated hyperpigmentation in flexural areas. This review includes a detailed literature search and examines cases since GGD\'s first description in 1982. It aims to synthesize the current knowledge on GGD, covering its etiology, clinical presentation, histopathology, diagnosis, and treatment. A significant aspect of this review is the exploration of the genetic, histopathological, and clinical parallels between GGD and Dowling-Degos disease (DDD), which is another rare autosomal dominant genodermatosis, particularly focusing on their shared mutations in the KRT5 and POGLUT1 genes. This supports the hypothesis that GGD and DDD may be different phenotypic expressions of the same pathological condition, although they have traditionally been recognized as separate entities, with suprabasal acantholysis being a distinctive feature of GGD. Lastly, this review discusses the existing treatment approaches, underscoring the absence of established guidelines and the limited effectiveness of various treatments.
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