blaschkoid

Blaschkoid
  • 文章类型: Journal Article
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    色素性扁平苔藓(LPP)是一种罕见的慢性扁平苔藓。LPP分布往往是对称的,经常在脸上看到,脖子,和树干。LPP的非典型临床模式,包括分段,zosteriform,线性,在文献中报道。在这里,我们报告了一名16岁女性罕见的LPP表现,沿Blascko线呈单侧线性模式。
    Lichen planus pigmentosus (LPP) is a rare chronic variant of lichen planus. LPP distribution tends to be symmetrical and is frequently seen on the face, neck, and trunk. Atypical clinical patterns of LPP, including segmental, zosteriform, and linear, were reported in the literature. Herein, we report a rare presentation of LPP in a 16- year-old female with a unilateral linear pattern along the Blascko lines.
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  • 文章类型: Journal Article
    背景:色素镶嵌(PM),也被称为Blaschkoid色素沉着,是一种罕见的色素异常。尽管已经发表了一些病例报告,描述了与PM相关的皮外表现,关于PM患者的临床特征的研究很少。
    目的:描述PM患者的临床特征。
    方法:这项描述性横断面研究是在皮肤科医生和儿科医生检查的47名儿童中进行的。PM的模式和位置,记录了色素沉着的类型和皮肤外表现.
    结果:最常见的PM模式是窄带PM,其次是宽带和棋盘图案。后备箱是受影响最严重的地区,其次是腿和胳膊。51.1%的PM表现为色素沉着减退,色素沉着过度占27.6%,以及21.2%的低/色素沉着。40.4%的患者存在伴随疾病:神经精神疾病是最常见的,其次是内分泌或血液系统疾病和生长/发育迟缓。
    结论:PM与一些皮外发现有关,但仍存在一些争论,这些关联是否反映了不同的PM表型,或者它们是否只是巧合。我们的研究表明,PM患者的皮外受累很频繁,因此需要对PM患者进行仔细检查。
    BACKGROUND: Pigmentary mosaicism (PM), also known as Blaschkoid dyspigmentation, is a rare pigmentary anomaly. Although several case reports have been published describing extracutaneous manifestations associated with PM, there are very few studies on the clinical characteristics of patients with PM.
    OBJECTIVE: To describe the clinical characteristics of patients with PM.
    METHODS: This descriptive cross-sectional study was conducted among 47 children examined by a dermatologist and a pediatrician. The pattern and location of the PM, type of pigmentation and extracutaneous manifestations were documented.
    RESULTS: The most common pattern of PM was narrow-band PM, followed by broad-band and checkerboard patterns. The trunk was the most affected region, followed by the legs and arms. PM manifested as hypopigmentation in 51.1% of cases, as hyperpigmentation in 27.6%, and as hypo/hyperpigmentation in 21.2%. Accompanying diseases were present in 40.4% of patients: neuropsychiatric diseases were the most common, followed by endocrinological or hematological diseases and growth/developmental delay.
    CONCLUSIONS: PM has been associated with several extracutaneous findings but there is still some debate whether these associations reflect different PM phenotypes or whether they are simply coincidental. Our study suggests that extracutaneous involvement in PM patients is frequent, thus warranting careful examination of PM patients.
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  • 文章类型: Journal Article
    扁平苔藓是一种独特的皮肤粘膜疾病,具有公认的临床和组织病理学标准。苔藓样爆发与扁平苔藓非常相似,有时与扁平苔藓难以区分。以前相关的系统性药物包括药物,病毒感染和疫苗。最近出现了与COVID-19疫苗相关的扁平苔藓和苔藓样反应的散发性病例报告。在这里,我们回顾了世界文献(31例患者),并将其扩展为15例出现疫苗诱导的苔藓样皮疹(V-ILE)的患者。讨论了临床和组织病理学发现的范围,重点是其病变表现在胚胎融合系中的子集,称为Blaschko系。在研究的46例患者中,有7例出现了这种罕见的Blaschkoid分布。感兴趣的,所有7个都与COVID-19mRNA疫苗相关。我们认为,应提高怀疑指数,并应特别询问患者的疫苗接种史。在早期诊断时,在几乎所有有或没有色素沉着过度的患者中,V-ILE易于治疗并迅速解决。关于其免疫病理学和炎症信号通路的其他调查研究可能会提供对与COVID-19疫苗接种相关的其他Th1驱动的自身免疫现象的见解。
    Lichen planus is a distinctive mucocutaneous disease with well-established clinical and histopathologic criteria. Lichenoid eruptions closely resemble lichen planus and may sometimes be indistinguishable from it. Systemic agents previously associated have included medications, viral infections and vaccines. Sporadic case reports of lichen planus and lichenoid reactions associated with COVID-19 vaccines have recently emerged. Herein, we review the world literature (31 patients) and expand it with a case series of 15 patients who presented with vaccine-induced lichenoid eruption (V-ILE). The spectrum of clinical and histopathologic findings is discussed with emphasis on the subset whose lesions manifested in embryologic fusion lines termed lines of Blaschko. This rare Blaschkoid distribution appeared in seven of the 46 patients studied. Of interest, all seven were linked to the mRNA COVID-19 vaccines. We believe that all lichenoid eruptions should be approached with a heightened index of suspicion and patients should be specifically questioned with regards to their vaccination history. When diagnosed early in its course, V-ILE is easily treated and resolves quickly in almost all patients with or without hyperpigmentation. Additional investigative studies regarding its immunopathology and inflammatory signaling pathways may offer insight into other Th1-driven autoimmune phenomena related to COVID-19 vaccination.
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  • 文章类型: Journal Article
    玫瑰糠疹(PR)是一种常见的丘疹鳞状疾病,可能与人疱疹病毒7和6的原发感染或内源性再激活有关。基于形态学,分布,和病变的大小,已经描述了PR的几种非典型变体。遵循Blaschko的PR代表了文献中很少描述的非典型变体。这里,我们描述了一个患有糖尿病的孩子,他在对称的情况下发展了PR,Blaschkolinear配置。
    Pityriasis rosea (PR) is a common papulosquamous disorder that may be related to primary infection or endogenous reactivation of human herpesvirus 7 and 6. Based on morphology, distribution, and size of lesions, several atypical variants of PR have been described. PR following the lines of Blaschko represents an atypical variant that has seldom been described in the literature. Here, we describe a child with diabetes who developed PR in a symmetrical, blaschkolinear configuration.
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  • 文章类型: Journal Article
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  • 文章类型: Case Reports
    Cutaneous conditions can follow Blaschko\'s lines on the skin, which are thought to reflect patterns of cell migration and clonal expansion during embryonic development of the epidermis. These diseases are hypothesized to be caused by genetic mosaicism resulting from processes such as lyonization or somatic postzygotic mutation. Lichen striatus and blaschkitis are two such acquired inflammatory skin disorders that are distinguished in the literature by age of onset, location, and histopathological features. Lichen striatus is typically observed on the extremities of children and is characterized by lichenoid papules that appear in a linear distribution along Blaschko\'s lines. Microscopic examination typically shows spongiosis, as well as lichenoid and periadnexal inflammation. Blaschkitis more commonly occurs in adults and frequently involves the truncal areas, including the chest and abdomen. Microscopic examination typically shows spongiotic dermatitis. We describe a young man with a linear eruption extending from the flexor aspect of his right wrist to his central chest, which has features of both lichen striatus and blaschkitis. Both lichen striatus and blaschkitis are self-limited diseases that may resolve within months. It has been suggested that lichen striatus and blaschkitis are not separate entities, but rather the two endpoints within the spectrum of blaschkolinear acquired inflammatory skin eruption (BLAISE). The overlapping features of lichen striatus and blaschkitis in our patient demonstrate the spectrum of clinical and pathologic features in patients with BLAISE.
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  • 文章类型: Case Reports
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  • 文章类型: Case Reports
    Goltz syndrome (focal dermal hypoplasia) is an X-linked dominant disorder that is classically associated with yellowish papules representing fat herniation (superficial adipocytes). We report a series of three cases, with clinicopathologic correlation of biopsies from Blaschkoid streaks. A range of histopathologic features, including some underreported findings (increased papillary dermal blood vessels, decreased thickness of the dermis, and adipocytes high in the dermis), are reproducible and can strongly point to the correct diagnosis of Goltz syndrome.
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