Blaschko lines

Blaschko 线
  • 文章类型: Journal Article
    背景:Blaschko线性银屑病(BLP)的特征是银屑病皮肤病变沿Blaschko线的线性分布。BLP可分为I型和II型,主要依据临床表现。BLP容易引起患者的心理负担和医师的临床困惑。这里,我们总结了临床病例,以更好地了解BLP。
    方法:受试者包括访问我们皮肤科的BLP患者以及从PubMed和Wanfang数据库获得的文献报道的患者。定量数据以平均值±SD(标准偏差)表示,定性数据以频率表示。采用学生t检验来比较均值,而卡方检验用于分析定性数据。
    结果:共有74例BLP患者(我们的5例患者,包括文献中的69个),Ⅰ型61例,Ⅱ型13例。我们总结BLP的特点如下:(1)男性个体更频繁,尤其是II型;(2)发病早于经典银屑病;(3)主要分布于单侧,且对左或右部位无偏好;(4)无症状或轻微瘙痒;(5)银屑病家族史大多为阴性;(6)指甲/头皮可能受累(主要为II型);(7)可能的外源性触发或加重因素;(8)可能伴随的经典斑块或点滴状银屑病病变,尤其是II型;(9)符合经典银屑病的组织病理学特征;(10)抗银屑病治疗反应相对良好,尽管对于II型叠加区域较差。
    结论:本研究分析了BLP的临床特征和治疗方面。与已发表的研究相比,我们有了新的发现,比如性别偏见。除了传统的抗银屑病治疗,个性化选择生物制剂也可能是一个有前途的选择。皮肤科医生应该认识和理解这种疾病的意义,并为患者提供适当的心理咨询和临床治疗。
    BACKGROUND: Blaschko linear psoriasis (BLP) is characterized by the linear distribution of psoriatic skin lesions along the Blaschko lines. BLP can be divided into type I and type II, mainly on the basis of clinical manifestations. BLP can easily cause psychological burdens in patients and clinical confusion for physicians. Here, we summarize clinical cases to provide a better understanding of BLP.
    METHODS: The subjects included patients with BLP who visited our dermatology departments and those reported in the literature obtained from the PubMed and Wanfang databases. Quantitative data were presented as means ± SD (standard deviation), and qualitative data were represented by the frequency. Student\'s t test was employed to compare means, whereas chi-square tests were used for analyzing qualitative data.
    RESULTS: A total of 74 patients with BLP (5 our patients, 69 from literature) were included, with 61 type I and 13 type II patients. We summarize BLP\'s characteristics as follows: (1) More frequent in male individuals, especially in type II; (2) Earlier onset than classical psoriasis; (3) Mainly distributed unilaterally, and no preference for left or right site; (4) Asymptomatic or slight pruritus; (5) Mostly negative family history of psoriasis; (6) Possible involvement of the nails/scalp (mainly for type II); (7) Possible exogenous triggering or aggravation factors; (8) Possible concomitant classical plaque or guttate psoriasis lesions, especially in type II; (9) Conforming to histopathology features of classical psoriasis; (10) Relatively favorable response to antipsoriatic treatment, although poor for superimposed areas in type II.
    CONCLUSIONS: This study analyzed the clinical characteristics and therapeutic aspects of BLP. Compared with published studies, we have new findings, such as gender bias. Besides traditional antipsoriatic treatment, a personalized selection of biologics may also be a promising choice. Dermatologists should recognize and understand the significance of this disease, and provide patients with appropriate psychological counseling and clinical treatments.
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  • 文章类型: Journal Article
    背景:纹状体苔藓是一种良性皮肤病,主要影响儿童。这种情况模仿许多其他皮肤病。
    目的:本文的目的是使儿科医生熟悉扁平苔藓的临床表现,以避免误诊,不必要的调查,不必要的转介,以及纹状体地衣管理不善.
    方法:于2023年6月在PubMed临床查询中使用关键术语“纹状体苔藓”进行了搜索。搜索策略包括所有观察性研究,临床试验,以及在过去十年内发表的评论。本评论仅包括在英语文献中发表的论文。从上述搜索中检索到的信息用于本文的汇编。
    结果:扁平苔藓是一种良性的自限性T细胞介导的皮肤病,其特征是主要见于儿童的线性炎性丘疹。发病通常是突然的,症状很少或没有。典型的扁平苔藓纹状体的喷发由离散的,肤色,粉色,红斑,或者暴力,平顶,稍微升高,融合形成暗红色的光滑或有鳞丘疹,可能有鳞状,在几天到几周内中断或连续的带。尽管身体的任何部分都可能参与其中,四肢是好的部位。通常,皮疹是孤独的,单边,并遵循Blaschko的路线。在深色皮肤的个体中,皮肤病变可能在发病时色素减退。指甲可能会单独受到影响,或者,更常见的是,伴随着纹状体苔藓的皮肤病变。扁平苔藓纹状体的鉴别诊断很多,其他疾病的显着特征在本文中得到了强调。
    结论:纹状体苔藓是一种自限性疾病,通常在一年内消退,没有残留的疤痕,但可能有短暂的炎症后色素沉着减退或色素沉着过度。因此,治疗可能是不必要的。对于希望接受美容治疗或瘙痒对症治疗的患者,可以使用低至中效价的局部皮质类固醇或局部免疫调节剂。褪色乳膏可用于炎症后色素沉着过度。
    BACKGROUND: Lichen striatus is a benign dermatosis that affects mainly children. This condition mimics many other dermatoses.
    OBJECTIVE: The purpose of this article is to familiarize pediatricians with the clinical manifestations of lichen striatus to avoid misdiagnosis, unnecessary investigations, unnecessary referrals, and mismanagement of lichen striatus.
    METHODS: A search was conducted in June 2023 in PubMed Clinical Queries using the key term \"Lichen striatus\". The search strategy included all observational studies, clinical trials, and reviews published within the past ten years. Only papers published in the English literature were included in this review. The information retrieved from the above search was used in the compilation of this article.
    RESULTS: Lichen striatus is a benign self-limited T-cell mediated dermatosis characterized by a linear inflammatory papular eruption seen primarily in children. The onset is usually sudden with minimal or absent symptomatology. The eruption in typical lichen striatus consists of discrete, skin- colored, pink, erythematous, or violaceous, flat-topped, slightly elevated, smooth or scaly papules that coalesce to form a dull red, potentially scaly, interrupted or continuous band over days to weeks. Although any part of the body may be involved, the extremities are the sites of predilection. Typically, the rash is solitary, unilateral, and follows Blaschko lines. In dark-skinned individuals, the skin lesions may be hypopigmented at onset. Nails may be affected alone or, more commonly, along with the skin lesions of lichen striatus. The differential diagnoses of lichen striatus are many and the salient features of other conditions are highlighted in the text.
    CONCLUSIONS: Lichen striatus is a self-limited condition that often resolves within one year without residual scarring but may have transient post-inflammatory hypopigmentation or hyperpigmentation. As such, treatment may not be necessary. For patients who desire treatment for cosmesis or for the symptomatic treatment of pruritus, a low- to mid-potency topical corticosteroid or a topical immunomodulator can be used. A fading cream can be used for post-inflammatory hyperpigmentation.
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  • 文章类型: Case Reports
    色素性扁平苔藓(LPP)是一种罕见的扁平苔藓,通常会影响皮肤较黑的中年人。与经典扁平苔藓相比,LPP与更长的临床病程有关,这在临床上是有区别的。它在儿童中的发生并不常见,在文献中很少报道该人群的病例。我们报告了一名7岁的沙特阿拉伯女性患者中罕见的单侧BlaschkoidLPP。
    Lichen planus pigmentosus (LPP) is a rare form of lichen planus that typically affects middle-aged people with darker-pigmented skin. LPP is associated with a longer clinical course than classical lichen planus, which distinguishes it clinically. Its occurrence in children is uncommon, with few reported cases in this population in the literature. We report a rare presentation of unilateral blaschkoid LPP in a seven-year-old Saudi Arabian female patient.
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  • 文章类型: Case Reports
    Moulin的线性福皮病(LAM)是一种罕见的获得性皮肤病。临床上,LAM的特征是色素沉着和萎缩性单侧带状或线性皮肤病,遵循Blaschko系。病变不表现为硬结或硬化。其病程无症状,无全身参与或进展。LAM的病因尚不清楚。根据其皮肤病变沿Blaschko线分布的特征,有学者推测其发病机制可能与染色体镶嵌有关。我们在此提供LAM在一名29岁的中国女性中的病例报告,该女性沿左后腰的Blaschko线持续分布着褐色斑块,臀部,和抗核抗体阳性的下肢(ANA,1:320,核仁型)和免疫球蛋白M升高(3.47g/L)。包括这个案子,据报道,共有6例LAM患者的血清免疫学指标异常。
    Linear atrophoderma of Moulin (LAM) is a rare acquired skin disease. Clinically, LAM is characterized by hyperpigmented and atrophic unilateral band-like or linear dermatoses of variable size following the Blaschko lines. The lesions do not present induration or sclerosis. Its course is asymptomatic without systemic involvement or progression. The etiology of LAM is still unclear. Based on the characteristics of its skin lesions distributed along the Blaschko lines, some scholars speculate that its pathogenesis may be related to chromosome mosaicism. We hereby present a case report of LAM in a 29-year-old Chinese female who had persistent brown patches distributed along the Blaschko lines on the left lower back, buttocks, and lower limbs with positive antinuclear antibody (ANA, 1:320, nucleolar type) and elevated immunoglobulin M (3.47 g/L). Including this case, a total of 6 LAM cases have been reported to have abnormal serum immunological markers.
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  • 文章类型: Review
    迄今为止,仅报告了21例出生后诊断为马赛克三体性12的病例。最常见的表型表现是发育迟缓,畸形面部特征,先天性心脏缺陷,数字改动,和色素性疾病。在本报告中,本文描述了3例无亲缘关系的新出现的镶嵌三体性12患者的详细临床和基因概况,并与以前报道的病例进行了比较.
    在本报告中,我们包括临床,细胞遗传学,和三名墨西哥患者的分子描述出生后诊断为镶嵌三体12。在表型水平,三名患者出现发育迟缓,畸形面部特征,先天性心脏缺陷和皮肤色素异常。特别是,患者1表现出独特的眼部改变为双侧扩张症,三排上睫毛,和数字异常。在患者2多余的皮肤中,严重的听力损失,并观察到低张力,3例患者表现为过度远调和远视。具有播散性色素异常的色素沉着过度是所有这些中的共同特征。细胞遗传学研究是在严格的分析标准下进行的,从三个不同的组织中筛选50-100个中期,在所分析的三种不同组织中的至少一种中显示出12三体镶嵌性。有了SNParray,先前未被细胞遗传学检测到的低水平马赛克拷贝数变异的存在,排除了12号染色体的单亲染色体。STR标记允许确认不存在单亲二倍体,并知道多余染色体12的亲本起源。
    详细的临床,细胞遗传学,以及这三个新患者的分子描述,有助于相关信息更准确地描绘一组表现出异质性表型的患者,虽然共享相同的染色体改变。检测镶嵌三体性12的可能性与用于揭示低水平染色体镶嵌性的方法的灵敏度直接相关。以及在合适的组织中进行分析的可能性。
    To date, only twenty-one cases diagnosed postnatally with mosaic trisomy 12 have been reported. The most frequent phenotypic manifestations are developmental delay, dysmorphic facial features, congenital heart defects, digital alterations, and pigmentary disorders. In the present report, detailed clinical and genetic profiles of three unrelated new patients with mosaic trisomy 12 are described and compared with previously reported cases.
    In the present report, we include the clinical, cytogenetic, and molecular description of three Mexican patients diagnosed postnatally with mosaic trisomy 12. At phenotypic level, the three patients present with developmental delay, dysmorphic facial features, congenital heart defects and skin pigmentary anomalies. Particularly, patient 1 showed unique eye alterations as bilateral distichiasis, triple rows of upper lashes, and digital abnormalities. In patient 2 redundant skin, severe hearing loss, and hypotonia were observed, and patient 3 presented with hypertelorism and telecanthus. Hyperpigmentation with disseminated pigmentary anomalies is a common trait in all of them. The cytogenetic study was carried out under the strict criteria of analysis, screening 50-100 metaphases from three different tissues, showing trisomy 12 mosaicism in at least one of the three different tissues analyzed. With SNParray, the presence of low-level mosaic copy number variants not previously detected by cytogenetics, and uniparental disomy of chromosome 12, was excluded. STR markers allowed to confirm the absence of uniparental disomy as well as to know the parental origin of supernumerary chromosome 12.
    The detailed clinical, cytogenetic, and molecular description of these three new patients, contributes with relevant information to delineate more accurately a group of patients that show a heterogeneous phenotype, although sharing the same chromosomal alteration. The possibility of detecting mosaic trisomy 12 is directly associated with the sensitivity of the methodology applied to reveal the low-level chromosomal mosaicism, as well as with the possibility to perform the analysis in a suitable tissue.
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  • 文章类型: Journal Article
    未经证实:色素失禁症(IP)是一种罕见的X连锁生殖道皮肤病,其特征是有许多发现。皮肤活检和组织病理学分析被认为是诊断IP的次要标准。我们认为皮肤镜检查可以帮助早期诊断IP。
    UNASSIGNED:通过观察皮肤变化的皮肤镜检查结果,获得早期诊断IP的经验。
    UASSIGNED:我们修订了确诊的IP病例,并使用皮肤镜检查进行了检查,比较组织病理学和皮肤镜结果。
    未经证实:第一阶段在红斑皮肤上呈线性排列的单发和成组囊泡。早期II期在红斑或色素沉着的皮肤上出现星形疣状病变。在发育良好的病变中,点状血管围绕着角化部分,一些有血栓形成的毛细血管,像病毒性疣.阶段III在着色区域上呈现线性棕色点。在所有检查的色素Blaschko线性变化中,皮肤镜图像是均匀的。第四阶段在色素减退的皮肤上出现许多点状血管。在所有四个阶段中,末端毛发都很少或不存在。在III和IV阶段,周围的正常皮肤有毛囊周围色素沉着。
    UNASSIGNED:与炎症性皮肤病和色素沉着的皮肤镜检查相比,所有四个阶段的皮肤镜检查都非常具体。第三阶段有非常接近的临床,组织学和皮肤镜模拟,需要用强制性基因检测仔细检查。IV期的皮肤镜检查与组织病理学发现紧密对应,并且作为揭示潜在IP基因携带者的快速工具可能至关重要。除临床检查外,还应使用皮肤镜检查,因为这两种方法是互补的。
    UNASSIGNED: Incontinentia pigmenti (IP) is a rare X-linked geno-dermatosis characterized by numerous findings. Skin biopsy and histopathological analysis are considered as minor criteria for the diagnosis of IP. We assume that dermoscopy can assist the earlier diagnosis of IP.
    UNASSIGNED: To gain experience in earlier diagnosis of IP by observing dermoscopic findings of cutaneous changes.
    UNASSIGNED: We revised confirmed cases of IP and examined them using dermoscopy, comparing histopathological and dermoscopic results.
    UNASSIGNED: Stage I presented solitary and grouped vesicles in linear arrangement on erythematous skin. Early stage II presented star-shaped verrucous lesions on erythematous or pigmented skin. In well-developed lesions, dotted vessels surround keratotic part, some with thrombosed capillaries, resembling a viral wart. Stage III presented linear brown dots on the pigmented areas. Dermoscopic image was uniform in all the examined pigmented Blaschko linear changes. Stage IV presented numerous dotted vessels on the hypopigmented skin. Terminal hair was scarce or absent in all four stages. The surrounding normal skin had perifollicular depigmentations in stages III and IV.
    UNASSIGNED: Dermoscopy of all four stages is very specific compared to the dermoscopy of inflammatory dermatoses and pigmentations. Stage III has very close clinical, histological and dermoscopic mimickers and needs to be carefully examined with obligatory genetic testing. Dermoscopy of the stage IV closely corresponds to histopathological findings and may be crucial as a quick tool in revealing potential IP gene carriers. Dermoscopy should be used in addition to clinical examination since the two methods are complementary.
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  • 文章类型: Case Reports
    炎性获得性Blaschko线性皮肤病(IABLD)是一个连续的概念,涉及诸如纹状体苔藓等疾病,Blaschkitis,和特应性皮炎。然而,在Blaschko系表现出严重程度增加的特应性皮炎很少单独报道。在这里,我们报告了一例罕见的特应性皮炎病例,伴有沿着Blaschko线的继发性结节性痒疹,这对拓宽IABLD的概念可能是有价值的。一名28岁的男性出现了多个,瘙痒,沿Blaschko线的左下肢褐色结节3至4年。患者自幼患有特应性皮炎。组织病理学检查结果显示,致密性角化过度,高颗粒症,海绵状体,表皮有不规则的棘皮病.真皮上部显示有垂直排列的胶原纤维和血管周围淋巴组织细胞浸润的纤维化。我们诊断该病例为沿Blaschko线的继发性结节性痒疹,伴有先前的特应性皮炎。我们假设患者的潜在特应性皮炎严重程度沿Blaschko线增加,和结节性痒疹是由于频繁的刮擦而发生的。局部用甲基强的松龙乳膏改善病变,口服抗组胺药和病灶内注射曲安奈德。
    Inflammatory acquired Blaschko-linear dermatoses (IABLD) are a continuous concept involving diseases such as lichen striatus, blaschkitis, and atopic dermatitis. However, atopic dermatitis that showed increase in severity along Blaschko lines is rarely reported on its own. Herein, we report a rare case of atopic dermatitis with secondary prurigo nodularis along Blaschko lines, which may be valuable in broadening the concept of IABLD. A 28-year-old male presented with multiple, pruritic, brownish nodules on the left lower extremity along Blaschko lines for 3 to 4 years. The patient had atopic dermatitis since childhood. Histopathologic findings revealed compact orthohyperkeratosis, hypergranulosis, spongiosis, and irregular acanthosis in the epidermis. Fibrosis with vertically arranged collagen fibers and perivascular lymphohistiocytic infiltration were shown in the upper dermis. We diagnosed the case as secondary prurigo nodularis along Blaschko lines, accompanied by the preceding atopic dermatitis. We hypothesized that the patient\'s underlying atopic dermatitis increased in severity along Blaschko lines, and prurigo nodularis occurred due to frequent scratching. The lesions improved with topical methylprednisolone cream, oral antihistamines and intralesional triamcinolone injection.
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  • 文章类型: Case Reports
    色素扁平苔藓是扁平苔藓的一种罕见变种。这是一种病因不明的获得性色素性疾病。它的特征是深棕色和石板灰色斑和斑块。钉子,头皮,口腔粘膜通常可以幸免,不像扁平苔藓.色素性扁平苔藓通常累及头颈部区域以及腋窝等杂物区域,乳房下和腹股沟区。它可能与自身免疫性疾病相关,内分泌疾病,和其他变种的扁平苔藓,如纤维性脱发的头皮。色素性扁平苔藓的各种临床模式,包括带状疱疹,线性,segmental已经出版了。组织病理学,它的特征是表皮角化过度,高颗粒症,不同程度的苔藓样浸润取决于病变的年龄,和突出的黑色素失禁。最近关于被认为与色素性扁平苔藓相似的红斑的更新,结论是它们可以在临床基础和组织病理学上进行区分。表皮角化过度,高颗粒症,凋亡细胞,苔藓样皮炎,食管附件浸润,和纤维化和明显的浅表真皮黑色素失禁有助于区分色素扁平苔藓和持久性红斑。在胚胎发生期间,细胞迁移并遵循以Blaschko命名的发育系,一位德国皮肤科医生,首先注意到他们。Blaschko'slines(BL),不要跟随神经,血管,或者淋巴通路.它们背面呈V形,腹部呈S形,在四肢上呈线性。我们报告了一例因BL引起的色素性扁平苔藓,这是该疾病的罕见表现和相关的抗核抗体(ANA)阳性,而没有任何结缔组织疾病的明显表现。
    Lichen planus pigmentosus is a rare variant of lichen planus. It is an acquired pigmentary disorder of unknown etiology. It is characterized by dark brown and slate gray macules and patches. The nails, scalp, and oral mucosa are usually spared, unlike lichen planus. Lichen planus pigmentosus commonly involves the head and neck region as well as intertriginous areas such as the axillae, inframammary and inguinal regions. It can be associated with autoimmune diseases, endocrinopathies, and other variants of lichen planus such as fibrosing alopecia of the scalp. Variable clinical patterns of lichen planus pigmentosus including zosteriform, linear, and segmental had been published. Histopathologically, it is characterized by hyperkeratosis of the epidermis, hypergranulosis, variable degrees of lichenoid infiltration depending on the age of the lesion, and prominent melanin incontinence. Recent updates on erythema dyschromicum perstans that were considered similar to lichen planus pigmentosus, concluded that they could be differentiated on clinical bases as well as histopathology. Epidermal hyperkeratosis, hypergranulosis, apoptotic cells, lichenoid dermatitis, periappendageal infiltrate, and fibrosis with marked superficial dermal melanin incontinence aid to differentiate lichen planus pigmentosus from erythema dyschromicum perstans. During embryogenesis, cells migrate and follow developmental lines named after Blaschko, a German dermatologist, who first noted them. Blaschko\'s lines (BL), do not follow neural, vascular, or lymphatic pathways. They appear as V-shaped on the back, S-shaped on the abdomen, and linearly on limbs. We report a case of lichen planus pigmentosus over BL that is a rare presentation of the disease and associated positive antinuclear antibody (ANA) without overt manifestations of any connective tissue disease.
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  • 文章类型: Journal Article
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  • 文章类型: Journal Article
    METHODS: Different clinical and histological variants of lichen planus (LP) exist, such as lichen planopilaris, pigmentosus, linear, or atrophic LP. Recently, some cases came to our attention of hyperpigmented and atrophic linear lesions of the face with lichenoid histology, suggesting a combination of these different variants. We carried out a single-center, retrospective descriptive study of 6 similar cases selected from our database and compared them with a literature review.
    RESULTS: There were 4 males and 2 females of mean age 42 years. Each had linear lesions located on one side of the face. All lesions were initially itchy; they appeared hyperpigmented in all patients and atrophic in 5 cases. Biopsies indicated lichen planopilaris in 5 patients, with deep peri-eccrine involvement in 4 of them. Only 2 of the 6 patients had extra-facial lesions.
    UNASSIGNED: We found 24 cases in the literature having similar clinical and histological aspects. Men aged around 37 years seemed particularly affected. An atrophic course was noticed in 10 patients. Such a clinicopathological picture may suggest differential diagnoses like lichen striatus, lupus erythematosus, lichen sclerosus atrophicus, or Moulin\'s linear atrophoderma. Early histopathological examination could be of precious assistance in allowing the initiation of effective treatment immediately as of the initial inflammatory phase, thereby limiting the risk of cosmetic sequelae such as atrophy or residual pigmentation.
    CONCLUSIONS: We describe a form of facial lichen planus that is highly particular in terms of its follicular tropism, its blaschkoid distribution, its pigmented character, and its atrophic progression.
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