Inflammatory Linear Verrucous Epidermal Nevus with a Novel CSPP1 Mutation: A Case Report and Review of the Literature

Background Inflammatory linear verrucous epidermal nevus(ILVEN)is a rare type of epidermal nevus that occurs in infancy and develops along Blaschko’s lines.Due to its rarity and clinical/histological similarities with other cutaneous disorders,correct diagnosis is sometimes difficult.In the present case,the whole exome sequencing of blood found no germline mutation,but the sequencing of the biopsy specimen revealed a novel potential pathogenic somatic mutation,C>T at rs200881715(p.R698X)in the cspp1 gene,which may provide new insight into the mechanism of ILVEN.Patient concerns A 10-year-old girl presented with linear,pruritic,erythematous,scaly,and exophytic verrucous skin lesions on her left thigh,buttock,and hand unilaterally along Blaschko’s lines from the third day after birth.Diagnosis Biopsy revealed psoriasiform acanthosis,papillary hyperplasia with orthohyperkeratosis,and parakeratosis of squamous epithelium,which are histological characteristics of ILVEN.Interventions Owing to the large lesion size and significant refractory response to previous treatments,multiple-stage surgical resections with a tissue expander were performed.Outcomes After surgical resection and defect reconstruction with a tissue expander,the outcome was satisfactory for the patient and her family.No recurrence was detected during the follow-up visits.Conclusion ILVEN is a rare type of cutaneous hyperplastic disease,and treatment is extremely challenging.Biopsy should be performed at the diagnostician’s discretion or if refractory to conservative treatments.Timely diagnosis and surgical intervention could result in satisfactory outcomes.

比较Becker's痣用铒:YAG和Q开关钕∶YAG治疗的临床和组织病理学结果

Background: Becker’ s naevus (BN) may represent a distressing cosmetic handicap and a challenging issue regarding treatment. Objectives: To compare clinical and histopathological findings of patients with BN treated with two different lasers: an erbium: yttrium-aluminium-garnet (Er: YAG) system vs. a neodymium: YAG (Nd: YAG) laser. Patients and methods: In this prospective and comparative study we present the clinical and histopathological evolution during a 2-year follow-up of 22 patients with BN treated with only one pass of the Er: YAG laser (n=11) or three treatment sessions with the Q-switched Nd: YAG system (n=11). Results: Clinical evaluation 2 years after treatment with the Er: YAG laser showed complete clearance (100% ) in 54% of the patients (n=6) and clearance of>50% in 100% of the subjects. In relation to Nd: YAG laser treatments our results echo those of other authors. Numerous sessions are necessary to get an acceptable clinical clearance rate. Only one patient showed marked clearance(51-99% )after three treatment sessions. Moderate (26- 50% ) and mild (1-25% ) clearance was observed in 45- 5% (n=5) and 27-3% (n=3) of the patients. Conclusions: Both Er: YAG and Nd: YAG are safe tools to treat BN. However, in terms of pigment removal, one pass with Er: YAG is a superior technique to three treatment sessions with the Nd: YAG.

双侧分布的Becker痣并发颈部白色纤维性丘疹病

报告1例呈双侧分布的Becker痣并发颈部白色纤维性丘疹病。患儿男,14岁。8年前左侧颈肩交界处出现淡黄褐色色素沉着斑,逐渐向左肩胛、胸部、上肢扩展,中央毳毛增多、增粗。5年前右乳房处出现类似皮损。半年前左侧躯体皮损处陆续出现散在的白色丘疹。皮肤科检查:胸部皮损面积约20cm×20cm,肩胛约10cm×15cm,其上密集分布长约2cm粗的黑体毛。以右侧乳头为中心的皮损面积约10cm×10cm,呈褐色。左颈侧皮损散在的圆形、黄白色丘疹,左侧乳房无丘疹。皮损组织病理检查:表皮棘层肥厚,基底层黑素增加。真皮中上部胶原明显增生、致密。立毛肌数量增多。

Becker痣24例分析

目的 探讨Becker痣的临床特点、合并症和组织病理表现。方法 回顾性分析24例Becker痣临床和病理资料。结果 24例均为散发病例,发病年龄主要集中在10～20岁(83.33%),发病部位以躯干前部和肩胛部居多(45.83%),典型皮损为较大的单侧分布的多毛的色素沉着斑,组织病理主要表现是表皮不同程度角化过度和棘层肥厚,表皮突较规则地向下延伸,基底层黑素颗粒增多,真皮网状层常见较大的形态不规则的平滑肌纤维束,伴有并发症共5例(20.83%)。结论 根据Becker痣典型皮损特点结合组织病理表现临床可以确诊,但是做为一种遗传相关的错构瘤可伴发其他皮肤肌肉骨骼异常,需仔细探明。

右美托咪定联合表面麻醉在Becker痣激光治疗中的应用

目的评价右美托咪定联合复方利多卡因乳膏表面麻醉在激光治疗Becker痣中的麻醉效果。方法选择30例Becker痣患者,随机分成两组各15例,实验组采用右美托咪定联合5%复方利多卡因乳膏麻醉,对照组采用5%复方利多卡因乳膏行表面麻醉,两组均采用Q开关Nd:YAG激光垂直移动照射术区,终点反应以治疗区域出现灰白为宜。采用视觉模拟评分法(VAS)对患者进行评估,采用调查问卷调查患者对麻醉方法的认可情况,连续监测并记录患者麻醉前、使用右美托咪定后、激光治疗即刻、激光术后的心率(HR)、动脉血氧饱和度(Sp O2)、平均动脉压(MAP),对比观察两组临床疗效。结果术后实验组的VAS评分显著低于对照组(P<0.001),实验组的麻醉效果优于对照组。两组患者对麻醉方法的认可率比较差异有统计学意义(P<0.05),实验组患者对麻醉方法的认可率高于对照组。经过3次治疗后,实验组患者总有效率为73.3%,对照组总有效率为40%,两组比较差异无统计学意义(P>0.05)。实验组患者使用右美托咪定后均有不同程度的心率减慢和平均动脉压下降,但保持平稳,动脉血氧饱和度未见异常。对照组患者在激光治疗过程中,出现不同程度的心率加快和平均动脉压升高,但未超出正常波动范围,动脉血氧饱和度未见异常。结论右美托咪定联合5%复方利多卡因乳膏用于激光治疗Becker痣的麻醉效果优于单用乳膏组。

Becker痣综合征1例

患者,女,22岁。右肩胛及胸部淡褐色不规则色素沉着斑伴右侧乳房发育不全10余年。胸部X线结果示:脊柱下胸段稍侧弯。诊断:Becker痣综合征。

S B Stekin不等式的证明和几何意义

探讨了S B Stekin不等式与Becker-Stark不等式之间的关系,利用微分法、积分法和级数理论给出了S B Stekin不等式的几个新的证法,并阐述了S B Stekin不等式的几何意义.

Becker痣的诊疗进展

目的回顾色素性毛表皮痣病因与发病机制、临床表现与治疗的相关文献,探讨临床治疗策略。方法对色素性毛表皮痣相关文献进行描述与总结。结果色素性毛表皮痣目前病因及发病机制尚不清楚,是一种良性皮肤错构瘤,可伴发皮肤肌肉骨骼发育异常,称为Becker痣综合征;单纯皮肤症状可尝试采用激光治疗,合并其他发育缺陷可采用手术治疗。结论色素性毛表皮痣病因及发病机制尚不清楚,激光是常用治疗单纯皮肤异常的方法。

Non-Invasive Evaluation and Differential Diagnosis for Pediatric Leukoderma in a Single Institute

Background: Although vitiligo shows a similar clinical picture to other disorders in which patients exhibit depigmented spots, there are few reports on the differentiation of these conditions from each other. Purpose: We aimed to investigate the differences and similarities between vitiligo and nevus depigmentosus in children based on the clinical course and characteristics. Materials and Methods: We conducted an examination of the disease type, treatment, progress, and differentiation of 85 cases of vitiligo and nevus depigmentosus that developed before 15 years of age among patients who visited the Osaka University Department of Dermatology with a chief complaint of white patches from January 2010 to March 2017. Results: The median age at the onset of symptoms of 79 patients with vitiligo was 4.5 years and while that of 6 patients with nevus depigmentosus was 0.8 years, indicating that the onset of nevus depigmentosus occurred earlier. While vitiligo often developed in the head and neck regions, with a tendency for depigmented spots in multiple regions, nevus depigmentosus did not show this tendency in these regions. Upon performing Wood’s lamp observation in 38 cases of vitiligo, 35 cases were found to be fluorescent chalky white and in 3 cases histologically diagnosed as vitiligo, no clear fluorescent color was observed. All 6 patients diagnosed with nevus depigmentosus exhibited a non-fluorescent off-white color under a Wood’s lamp. Conclusions: This study indicates that non-invasive observation using a Wood’s lamp is useful in the differential diagnosis of pediatric leukoderma, in addition to the subsequent clinical course, such as the presence or absence of lesional repigmentation and/or enlargement.

Classification of and the latest photoelectric therapy for epidermal nevi

An epidermal nevus(EN)is a common disease of various types,including verrucous EN,nevus sebaceous,inflammatory linear verrucous EN,Becker nevus,smooth muscle hamartoma,and nevus comedonicus.EN syndrome is a serious and difficult-to-treat syndrome that is complicated by two or more abnormal organs or systems.To date,the mechanism of an EN is still unclear,and the therapies remain unsatisfactory.The most frequently used treatments are surgery and laser therapy.Several drugs have also been tested.An EN requires more attention,and further research is needed to provide appropriate and effective treatments for each patient.

色素性毛表皮痣的光学治疗现状

色素性毛表皮痣也称为贝克尔痣,临床主要表现为较大面积的褐色斑片及合并多毛表现,病理上除色素增加外,也有表皮增厚,表皮突下延的情况。本病治疗比较困难,激光是目前的主要治疗手段,特别是近年来新型皮秒激光、不同激光联合治疗等方面较既往疗效显著,本文就色素性毛表皮痣及其光学治疗现状进行综述,以期为临床提供更好的治疗选择。

颧部褐青色痣黑素细胞特性及来源的研究

目的为了进一步证实颧部褐青色痣的组织病理特点,并初步探讨颧部褐青色痣黑素细胞的来源。方法选取颧部褐青色痣、皮内痣、蓝痣及黑变病标本各10例,所有病理切片标本均进行HE染色和免疫组化法检测CD68、S-100、Melan-A、NSE、C-Kit(CD117)在黑素细胞上的表达。结果①颧部褐青色痣的HE染色:在真皮浅层有散在的含黑素颗粒的梭形细胞分布,其细胞长轴与表皮平行,主要分布于真皮浅层。真皮含黑素颗粒细胞分为散在或弥漫的分布。②免疫组化:颧部褐青色痣、皮内痣和蓝痣各组中银染色、S-100、Melan-A、NSE、CD68免疫组化均为阳性,其中Melan-A染色在颧部褐青色痣和皮内痣组显示有表皮基底层黑素细胞增加;黑变病组仅有CD68阳性。C-kit阳性在颧部褐青色痣组有9例、蓝痣组有8例、皮内痣组有5例。结论颧部褐青色痣的表皮基底黑素细胞增加,真皮内既有黑素细胞,也有噬黑素细胞。其黑素细胞是一种具有痣细胞、神经嵴来源细胞及早期胚胎细胞的特性。

LightSheer半导体激光对Becker痣脱毛疗效的分析

目的评价LightSheer半导体激光对Becker痣的脱毛疗效,分析影响脱毛疗效的因素及不良反应。方法对进行脱毛治疗的75例Becker痣患者,采用800 nm LightSheer半导体激光脱毛仪进行≥3次治疗,分析疗效。结果 75例患者中,女13例,男62例,皮损位于面颈部45例、躯干20例、四肢10例。治疗后毛发减少>75%12例,50%~75%28例,26%~49%29例,≤25%6例。<18岁患者治疗过程中可能出现毛发增多、增粗。单因素Logistic回归分析显示,就诊年龄、毛发密度及治疗次数对脱毛疗效有影响,性别、皮损部位、颜色对脱毛疗效无影响。多因素Logistic回归分析显示,与就诊年龄<18岁、治疗3~4次相比,就诊年龄≥18岁、治疗≥5次对脱毛疗效差异有统计学意义(P=0.018和P<0.001)。结论 LightSheer半导体激光治疗Becker痣,就诊年龄、初诊时毛发密度、治疗次数对脱毛疗效有影响,其治疗Becker痣可有效改善局部毛发量,无明显不良反应。

皮秒与纳秒Q开关翠绿宝石激光治疗褐青色痣的疗效比较

目的比较皮秒与纳秒Q开关翠绿宝石激光治疗褐青色痣的疗效及不良反应。方法 30例褐青色痣患者随机一侧面部接受皮秒Q开关翠绿宝石激光治疗,另一侧面部接受纳秒Q开关翠绿宝石激光治疗,1次治疗后6个月随访。采用4分法评估疗效,改善>75%为4分,改善51%~75%为3分,改善26%~50%为2分,改善≤25%为1分。观察色素沉着等不良反应。结果 30例褐青色痣患者1次治疗6个月后,皮秒Q开关翠绿宝石激光侧疗效评分:4分1例(3.30%),3分11例(36.70%),2分9例(30.00%),1分9例(30.00%);纳秒Q开关翠绿宝石激光侧4分0例,3分1例(3.30%),2分9例(30.00%),1分20例(66.70%)。皮秒Q开关翠绿宝石激光侧色素沉着发生率为43.33%(13/30),低于纳秒Q开关翠绿宝石激光[83.33%(25/30)](P<0.05)。结论皮秒Q开关翠绿宝石激光疗效显著,色素沉着发生率低,是治疗褐青色痣的理想选择。

色素性毛表皮痣的发病机制及治疗进展

色素性毛表皮痣是以皮肤褐色斑片伴毛发生长为特点的色素性皮肤病,作为损容性皮肤病的一种,严重影响患者的皮肤外观及心理健康。其发病机制尚不十分明确,既往研究认为可能与雄激素及其受体相关,近年来有学者提出染色体突变和遗传可能也参与了发病。在治疗上主要包括手术切除后植皮、液氮、化学磨削以及近年来提出的光电治疗等,但其复发率高给治疗带来了一定难度。因此,本文就色素性毛表皮痣近年来的研究进展作一综述。

30例色素性毛表皮痣临床、病理及误诊分析

目的:探讨30例色素性毛表皮痣(BN)患者的临床、病理表现及误诊情况,以提高对BN的认识。方法:对我院诊治的30例色素性毛表皮痣患者的临床资料进行回顾性分析,包括临床、病理、误诊及治疗情况。结果:30例患者在出生后不久或青春期发病,男性较多(66.7%),皮损好发于上肢及胸部,单侧分布,青春期发病的男性及幼年发病的女性毛发较多。病理可见表皮突规则延长,角质形成细胞中黑素小体增加,黑素细胞数目正常或轻度增加。常伴淋巴细胞浸润,立毛肌增生。初诊时有10例误诊,分别误诊为融合性网状乳头瘤病、色素性痒疹、咖啡斑等。光电技术治疗色素性毛表皮痣因病例数较少,经验不足,依据目前的治疗效果,提示对脱毛的治疗效果较好,对色素的改善不明显。结论:BN容易误诊,临床特点及组织病理学检查为诊断本病的依据,临床医生要加强对该病的认识。

气球状细胞痣的病理诊断及鉴别诊断

目的:探讨皮肤气球状细胞痣的病理诊断及鉴别诊断。方法:对1例皮肤气球状细胞痣应用免疫组化SP(streptavidin-perosidase)法观察,并进行临床病理分析。结果:皮肤组织真皮内见胞浆透亮的细胞排列成大小不等由胶原纤维分隔的小叶,痣细胞绝大部分为体积较大、胞质丰富且呈透明状或细颗粒状气球状痣细胞。胞核小而圆,大小一致,多位于细胞的中央,无异型性及核分裂像。免疫组化染色提示气球状细胞痣S-100(++)、Vimentin(++)、Melan-A(++)、Ki-67<5%,HMB-45仅在真皮浅层的灶性气球状细胞浆呈弱阳性表达。结论:气球状细胞痣极为罕见,需要与皮肤的多种透明细胞肿瘤进行鉴别,正确诊断主要依赖组织病理及免疫组化结果。

Clinical Application of CLSM,Dermoscope,and Wood’s Lamp in Diagnosis and Differential Diagnosis of Five Hypopigmentation Disorders:A Cross-Sectional Study

Objective:Noninvasive instruments including confocal laser scanning microscope(CLSM),dermoscope,and Wood’s lamp afford the advantage of obtaining skin microanatomical images quickly,in real-time,and dynamically.However,further understanding is needed regarding the differences between various techniques for diagnosis on hypopigmentation diseases.This study was aimed to observe the characteristics of hypopigmentation diseases by using CLSM,dermoscope,and Wood’s lamp,in order to provide evidence for clinician to choose appropriate instruments for assistant diagnosis.Methods:In total,data from 397 patients with vitiligo,nevus anemicus,achromic nevus,pityriasis alba,and senile leukoderma were collected from January 2019 to January 2021 in affiliated hospital of Xuzhou Medical University.CLSM,dermoscope,and Wood’s lamp were used to analyze the characteristics of lesions and the differences between the diseases.Enumeration data were presented as frequencies and percentages.Results:CLSM showed that the pigment ring of vitiligo was absent(126/126,100.0%),senile leukoderma had hypopigmentation,skin atrophy and thinning,and flat epidermis mutation(79/79,100.0%).In dermoscope,vitiligo showed a milky white hypopigmented area(113/126,89.8%),nevus anemicus showed a clear but irregular(38/45,84.4%)white hypopigmentation area(41/45,91.1%),pityriasis alba showed a clear and light white hypopigmented area,with a grayish-white chaff-like scaly appearance(98/107,91.6%),senile leukoderma showed a white radially hypopigmentation area with a small amount of pigmentation(79/79,100.0%).Under Wood’s lamp,bright blue-white patches with clear boundaries can be observed in vitiligo,nevus anemicus,pityriasis alba(93/107,86.9%)and senile leukoderma appeared clear bluish-white patches,achromic nevus appeared unclear bluish-white patches,respectively.Conclusion:Vitiligo,nevus anemicus,achromic nevus,pityriasis alba,and senile leukoderma all belong to hypopigmentation diseases,however,they have own imaging characteristics under CLSM,dermoscope,and Woods lamp techniques,which may be used to help the diagnosis and differential diagnosis of these diseases.

环境保护

20多年前，当社会上掀起了以社会与环境的标准来引导投资的运动时，Joan BaVaria就是这类运动的先锋人物。她经常根据公司的行为，不断对它们加以诱惑、赞扬和抨击。单是2001年，她牵头成立的组织CERES就敦促艾克森美孚石油公司(ExxonMobil)的股东在年会上确定了公司的环保目标。同时，CERES也威胁要终止与通用汽车公司的合作关系，因为通用公司反对为运动休闲车制定更为严格的燃料节约标准。

激光美容相关常见疾病诊疗指南(2024版)

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