Scarring alopecia

瘢痕性脱发
  • 文章类型: Journal Article
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  • 文章类型: Journal Article
    毛囊角化病(KFSD)是一种罕见的X连锁遗传性疾病,其特征是滤泡性角化过度-畏光-脱发三联症。临床异质性使诊断困难。探讨KFSD的临床病理特征和镜下特征,进一步明确诊断的必要条件。我们对KFSD患者进行了回顾性研究.临床信息,组织学特征,和三镜检查结果进行了评估。八名患者来自七个不同的家庭。两名女性是来自同一家庭的母亲和女儿,其他六名患者是男性,代表散发病例。脱发的平均发病年龄为21.25岁。头皮毛发的参与导致头皮中线进行性瘢痕性脱发并伴有不同程度的炎症是病理特征。它通常在青春期后开始。与毛发相关的毛囊角化过度病变影响了所有患者。然而,畏光不是一个恒定的特征。组织病理学检查显示毛囊疾病伴有急慢性炎症反应。卵泡变化,包括融合漏斗,外根鞘伸入卵泡管,观察到由角蛋白闭塞引起的峡部毛囊扩张。三镜特征包括毛囊周围鳞屑,簇绒的毛发,和卵泡开口的丧失。总之,末梢毛发受累,无论是头皮毛发,眉毛,或者睫毛,绒毛毛囊角化过度是KFSD的诊断依据。我们假设组织病理学的卵泡变化是引发可变炎症和进一步卵泡破坏的主要事件。
    Keratosis follicularis spinulosa decalvans (KFSD) is a rare X-linked hereditary disorder characterized by the triad of follicular hyperkeratosis-photophobia-alopecia. The clinical heterogeneity makes the diagnosis difficult. To investigate the clinicopathologic and trichoscopic features of KFSD and to further clarify the essential requisites for the diagnosis, we conducted a retrospective study of patients with KFSD. The clinical information, histologic features, and trichoscopic findings were evaluated. Eight patients were from seven separate families. Two females were mother and daughter from the same family and the other six patients were male and represented sporadic cases. The average age of onset of alopecia was 21.25 years. Involvement of the scalp hairs leading to progressive scarring alopecia on the midline of the scalp with variable degrees of inflammation was the pathognomonic feature. It typically began after puberty. Vellus hair-associated follicular hyperkeratosis affected all of the patients. However, photophobia was not a constant feature. Histopathologic examination revealed disorders of the hair follicle with an acute-chronic inflammatory response. Follicular changes including fused infundibulum, the protrusion of the outer root sheath into the follicular canal, and a dilatation of the follicles at the isthmus level caused by the occlusion of keratin were observed. The trichoscopic features included perifollicular scaling, tufted hairs, and loss of follicular openings. In conclusion, terminal hair involvement, either scalp hairs, eyebrows, or eyelashes, and the hyperkeratosis of the follicle of vellus hairs is the diagnostic basis of KFSD. We hypothesize that follicular changes in histopathology are the primary event that trigger variable inflammation and further follicular destruction.
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  • 文章类型: Journal Article
    目的:皮肤红斑狼疮(CLE)是一种异质性皮肤病。两种最常见的亚型是盘状LE(DLE),其特征是瘢痕性皮肤损伤和急性CLE(ACLE),表现为短暂可逆的皮肤损伤。尚不清楚是什么原因导致皮肤病变的差异。研究表明,在人体组织中存在组织特异性5-羟甲基胞嘧啶(5hmC)修饰区,这可能会影响组织相关疾病。这里,我们旨在评估5hmc在DLE和ACLE病变中的表达,并探讨5hmc与DLE瘢痕损害的关系。
    方法:84个CLE样本纳入研究。我们评估了皮肤损伤评分并回顾了组织病理学切片。免疫组化染色检测5hmc在附件和附件周围炎症细胞中的表达。通过多光谱免疫组织化学染色研究了5hmc在食管附件淋巴细胞中的表达。
    结果:疤痕/萎缩是区分DLE和ACLE的最显著损害。所有DLE瘢痕性脱发(DLESA)患者均存在滤泡周围炎性浸润。DLESA的附件和附件周围炎症细胞中5hmc的表达明显高于ACLE。在IFN-α/β受体(IFNAR)的染色中观察到类似的表达模式。附件中5hmc的表达与附件周围炎症细胞的表达呈正相关。在由CD4+细胞组成的毛囊周围的淋巴细胞簇中有5hmc表达增加,CD8+细胞,DLESA病变中的CD19+细胞。
    结论:这些数据表明5hmc的表达模式与组织病理学特征分布密切相关,以及DLESA中的I型干扰素(IFNs)信号,支持5hmc在附件损伤和附件周围炎症放大中的重要性,从而为DLE的瘢痕损伤和CLE皮肤病变的异质性提供了新的见解。
    OBJECTIVE: Cutaneous lupus erythematosus (CLE) is a heterogenous skin disease. The two most common subtypes are discoid LE (DLE) characterized by scarring skin damage and acute CLE (ACLE) presenting with transiently reversible skin lesions. It remains unknown what causes the difference of skin lesions. Studies have shown the existence of tissue-specific 5-Hydroxymethylcytosine (5 hmC)-modified regions in human tissues, which may affect the tissue-related diseases. Here, we aim to assess the expression of 5 hmc in DLE and ACLE lesions and explore the relationship of 5 hmc with scarring damage in DLE.
    METHODS: 84 CLE samples were included in the study. We evaluated the skin damage score and reviewed the histopathologic sections. Immunohistochemical staining was performed to detect the expression of 5 hmc in the appendage and periappendageal inflammatory cells. The 5 hmc expression in periappendageal lymphocytic cells was investigated by multi-spectrum immunohistochemistry staining.
    RESULTS: Scarring/atrophy was the most significant damage in differentiating the DLE from ACLE. Perifollicular inflammatory infiltration was present in all patients with DLE scarring alopecia (DLESA). The 5 hmc expression in the appendage and periappendageal inflammatory cells was significantxly increased in DLESA than ACLE. Similar expression pattern was seen in the staining of IFN-alpha/beta Receptor (IFNAR). The expression of 5 hmc in the appendage was positively correlated with that in the periappendageal inflammatory cells. There was an increased 5 hmc expression in lymphocytes cluster around hair follicle consisting of CD4+ cells, CD8+ cells, and CD19+ cells in DLESA lesions.
    CONCLUSIONS: These data demonstrate a close association of the expression pattern of 5 hmc with the histopathological characteristic distribution, and with the type I interferons (IFNs) signals in DLESA, supporting the importance of 5 hmc in the amplification of appendage damage and periappendageal inflammation, thereby offering a novel insight into the scarring damage of DLE and the heterogeneity of CLE skin lesions.
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  • 文章类型: Journal Article
    背景:瘢痕性脱发可显著影响儿童的情绪。毛囊单位切除(FUE)和毛囊单位移植(FUT)已用于瘢痕治疗。
    目的:本研究旨在评估毛囊单位毛发移植治疗儿童瘢痕性脱发的安全性和可行性。
    方法:共有9名儿童(7名男性和2名女性)患有瘢痕性脱发,年龄从5岁开始,2个月到12年,本研究包括10个月。瘢痕形成时间7个月~5年。站点是顶点(2),眉毛(3),正面发际线(3),和时间区域(2)。
    结果:本组9例患儿随访6~34个月,治疗方案如下:FUE(5例),FUT(3例),和FUT结合FUE(1例)。治疗期间无明显并发症发生。移植的头发长得很好,方向和形状令人满意,生存率>90%。
    结论:对于手术后烧伤和瘢痕性脱发的儿童,毛发移植可以显著改善其外观,手术风险低,患者满意度高。
    BACKGROUND: Scarring alopecia can significantly affect children emotionally. Follicular unit excision (FUE) and follicular unit transplantation (FUT) have been applied for scar treatment.
    OBJECTIVE: This study aimed to evaluate the safety and feasibility of follicular unit hair transplantation in treating scarring alopecia in children.
    METHODS: A total of nine children (seven males and two females) with cicatricial alopecia, ranging in age from 5 years, 2 months to 12 years, 10 months were included in this study. Scar formation time ranged from 7 months to 5 years. Sites were vertex (2), eyebrow (3), frontal hairline (3), and temporal regions (2).
    RESULTS: Nine children in this group were followed up for 6-34 months with the following treatment options: FUE (5 cases), FUT (3 cases), and FUT combined with FUE (1 case). No significant complications were observed during the treatment. The transplanted hair grew well, the direction and shape were satisfactory, and the survival rate was >90%.
    CONCLUSIONS: For children with burn trauma and cicatricial alopecia after surgery, hair transplantation can significantly improve their appearance with low surgical risk and high patient satisfaction rate.
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  • 文章类型: Journal Article
    Scarring alopecia in systemic lupus erythematosus (SLE) patients caused reduced life quality and prolonged disease course. This case-control study aims to survey the prevalence of scarring alopecia during the disease course of SLE and evaluate the risk factors for scarring alopecia in Chinese SLE patients.
    SLE patients in Chinese SLE treatment and Research group (CSTAR) were recruited. Scarring alopecia was defined according to SLICC/ACR-DI which was collected during follow-up visits or via self-reported questionnaires. We collected demographic characteristics, common comorbidities, autoantibody profiles, disease activity status, major organ involvements, and treatment strategies of these patients at registry. Univariate and multivariate logistic regression analyses were used to investigate the risk factors for scarring alopecia.
    We recruited 4792 SLE patients, and 374 (7.80%) patients had scarring alopecia. Mucocutaneous lesions (OR 2.062, p < 0.001), high SLICC/ACR-DI (OR 1.409, p < 0.001), and positive anti-Sm (OR 1.374, p = 0.029) were risk factors for scarring alopecia, while renal (OR 0.714, p = 0.028) and cardio-respiratory involvements (OR 0.347, p = 0.044), and immunosuppressant treatment (OR 0.675, p < 0.001) were significantly negative associated with it.
    The prevalence of scarring alopecia in SLE patients is 7.80%. Active treatment strategies should be adopted to prevent scarring alopecia occurring.
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