无色素痣的研究进展

无色素痣是一种先天性、非家族性皮肤色素减退性疾病，病因不明。通常表现为先天性、边缘不规则的色素减退斑。白斑相对位置和分布持续终生不变。临床分为孤立型、节段型和系统型3型。组织病理学检查表明，无色素痣皮损内黑素细胞数量正常或减少，但黑素的合成及黑素小体向角质形成细胞内的迁移减少。超微结构观察发现角质形成细胞内有薄膜包绕的聚集的黑素小体。自体表皮移植是治疗无色素痣的一种有效方法。     

无色素痣的临床、组织学和超微结构观察

目的 探讨无色素痣的临床和组织学特征。方法 分析85例无色素痣患者的发病年龄、类型和皮损特点,并对部分患者行皮肤色素测定和反射式共聚焦显微镜（RCM）观察。对其中17例患者的皮损区和正常区皮肤组织进行组织病理检查,透射电镜观察皮损区超微结构。免疫组化法检测皮损区和正常皮肤处酪氨酸酶（TYR）、HMB45、酪氨酸酶相关蛋白1（TRP-1）、TRP-2和CD117表达。结果 85例无色素痣患者中,23例（27.1%）出生时发现皮损,21例（24.7%）出现于3岁以后,最大发病年龄为29岁。皮损分布于躯干部25例（29.4%）,颈部13例（15.3%）;72例（84.7%）皮损边缘不规则,54例（63.5 %）仅有1处皮损。19例无色素痣患者患处的黑素指数（186.56 ± 52.86）和相对黑素指数（80 ± 11）低于正常人皮肤（分别为223.88 ± 63.19和100）,高于12例白癜风患者皮损处（分别为128.57 ± 64.31和60 ± 20）,差异均具有统计学意义（P < 0.01）。反射式共聚焦显微镜示,无色素痣皮损中含黑素细胞数量减少,亮度减低,黑素分布均匀,皮损区与正常皮肤分界区常不清晰。皮损区Fontana-Masson染色示皮损区黑素强度为1810.12 ± 327.96,较正常区（2064.24 ± 260.41）明显减弱。电镜下发现黑素细胞数量减少,黑素小体减少,黑素细胞胞质和树突以及角质形成细胞中可见Ⅱ、Ⅲ期未成熟的黑素小体,角质形成细胞中可见聚集成团的黑素小体。17例患者正常区TYR表达水平为1827.35 ± 307.09,TRP-1为6102.54 ± 1642.64,而皮损区TYR（1477.35 ± 224.05）和TRP-1（5322.33 ± 1565.26）表达下降,正常区与皮损区比较,P均 < 0.01;HMB45、TRP-2、CD117表达两处比较差异均无统计学意义。 结论 无色素痣是一种早期发病、非家族聚集性、稳定的不规则色素减退性疾病,其皮损中黑素细胞和黑素小体数量均减少,可见未成熟黑素小体。相对黑素指数和反射式共聚焦显微镜检查可作为诊断无色素痣的无创性检测方法。     

308单频准分子紫外光治疗无色素痣的临床疗效观察

目的 探讨308单频准分子紫外光对无色素痣的疗效及其不良反应。方法 308单频准分子紫外光对18例躯干部无色素痣的患者进行连续10次治疗,治疗前后检测皮损平均黑素值,随访3个月。结果 同初次治疗前基线值相比,治疗后皮损平均黑素值以次数依赖方式逐渐上升;约6次治疗后,皮损平均黑素值与周围正常皮肤平均黑素值百分率接近100%,随着随访时间的延长,皮损平均黑素值有下降趋势,约1个月后,皮损平均黑素值接近周围正常皮肤,3个月后皮损平均黑素值是正常皮肤的90%。治疗期间未发现水疱和瘢痕形成等不良反应。结论 308准分子紫外光治疗无色素痣安全有效,为稳定疗效可间隔3个月巩固治疗1次。     

个体化培养自体黑素细胞移植治疗白癜风

目的 探讨使用个体化培养基进行自体黑素细胞培养移植治疗白癜风的疗效。方法 负压吸疱获取患者正常表皮片,制成细胞悬液,在Hu16黑素细胞选择性培养基中培养。检测黑素细胞分裂时间（DOT）和黑素含量,根据DOT的大小、黑素含量和细胞形态,调整血清、细胞因子浓度及补充内皮素-1,进行个体化黑素细胞培养。经2 ~ 5次传代后收集黑素细胞,白斑区用超脉冲CO2激光磨削后进行黑素细胞移植,随访观察复色效果。结果 共治疗155例稳定期白癜风患者的204处皮损,进行1次移植119例,进行2 ~ 4次移植36例。应用个体化黑素细胞培养后细胞扩增可达50 ~ 80倍。84.80%的皮损复色面积超过50%,其中52.94%的皮损复色面积超过90%,且复色均匀,未见瘢痕及其他不良反应。性别、年龄、病程长短和皮损面积大小对疗效没有影响。节段型白癜风移植疗效好于寻常型白癜风,两组有效率分别为93.62%和82.16%,痊愈率分别为65.96%和49.04%。手臂和腿部的皮损（不包括肘部和膝盖）移植后痊愈率达73.08%,疗效好于躯干、面颈;肢端皮损疗效最差,痊愈率仅为25.93%。结论 个体化培养技术能提高白癜风患者黑素细胞的培养成功率与细胞扩增倍数。体外培养的自体黑素细胞移植治疗稳定期白癜风疗效肯定,用少量供皮区即可治疗大面积皮损,值得临床应用。     

压力对白癜风负压吸疱自体表皮移植的影响

负压吸疱自体表皮片移植是治疗稳定期白癜风常用方法之一.供皮区一般选择腹部、臀部等不影响美容的部位.我们通过观察不同压力下腹部起疱情况、真表皮分离部位,黑素细胞培养观察黑素细胞数量,寻求腹部起疱最合适的压力,从而提高负压吸疱移植治疗白癜风的疗效.     

正常皮肤负压吸疱疱顶Masson Fontana染色分析

为研究负夺吸疱法表皮移植治疗白癜风的机理,对16例来自白癜风病人色素正常部位皮肤负压吸引产生水疱的疱顶组织进行HE和Masson Fontana染色,观察水疱的位置及有无黑素细胞的存在。结果负压吸引产生的水疱位于棘细胞层,无黑素细胞存在。治疗白癜风表皮移植的机理黑素细胞被移植这一假说不能成立。     

进行性斑状色素减少症的临床及实验研究

目的 探讨进行性斑状色素减少症（PMH）的临床特点和诊断要点。方法 用Wood灯及活体共聚焦激光扫描显微镜（皮肤CT）观察皮损特点、致病菌培养、黑素细胞培养,并应用S-100和TRP-1免疫组化分析皮损区黑素细胞数量、电镜观察其超微结构特征。结果 Wood灯检查示皮损区可见点状红色荧光,皮肤CT观察示皮损区色素环完整,但与周围正常皮肤相比其内所含的黑素颗粒含量减少。致病菌培养可见产红色荧光的革兰阳性棒状杆菌,经鉴定为痤疮丙酸杆菌。S-100染色示皮损区阳性细胞数（8.25 ± 0.96）与周围正常皮肤（8.75 ± 1.71）相比无统计学意义（P > 0.05）。TRP-1染色示皮损区阳性细胞数（4.25 ± 0.96）与周围正常皮肤（4.50 ± 1.29）相比也无统计学意义（P > 0.05）。电镜观察发现,皮损区Ⅳ期黑素小体的数量明显下降,并观察到较多的膜结合体,内含成簇状分布的多个体积较小的Ⅱ ～ Ⅳ期黑素小体。成功培养出黑素细胞,其形态与正常细胞相比未见明显异常。结论 初步提出进行性斑状色素减少症的诊断要点。     

色素障碍性皮肤病

993015 寻常型白癜风皮损的超微结构研究/漆军（解放军总医院）…//解放军医学杂志.-1999,24（1）.-35～37 以透射电镜观察了10例白癜风患者的白斑和边缘处皮损,两部位有明显不同。白斑处无黑素细胞和黑素小体,角朊细胞改变较小;白斑边缘可见黑素细胞空泡变性,黑素小体聚集,核固缩,角朊细胞有明显的空泡变性,郎汉斯细胞略少于白斑区。3例静止期标本中见表皮基层有单一核细胞浸润,真皮浅层见较多单一枝树枝状细胞,但无Birbeck颗粒。作者认为白癜风皮损中的角朊细胞变性系黑素细胞损伤的继发改变,另推测真皮中的树枝状细胞主要是郎汉斯细胞,郎汉斯细胞在白癜风的发病中可能发挥了重要作用。图3参5 （高天文）     

白癜风皮损边缘黑素细胞线粒体超微结构的电镜观察

【摘要】 目的 探讨白癜风患者皮损边缘黑素细胞线粒体结构的变化。方法 在透射电镜下观察健康对照、进展期白癜风及稳定期白癜风患者皮损边缘黑素细胞形态,体视学方法测量线粒体体密度（Vv）、表面积密度（Sv）、数密度（Nv）等参数。结果 健康对照组黑素细胞可见大量黑素小体（28.57 ± 3.21）,以Ⅲ、Ⅳ期为主,线粒体规则分布在细胞内,结构正常、嵴密集,部分细胞胞质内可见自噬小体。进展期和稳定期白癜风黑素细胞内黑素小体数量减少,单位细胞内黑素小体数量分别为22 ± 6.16和17.43 ± 6.24,其中,Ⅲ期黑素小体显著减少,线粒体大小不一、形态多样,大部分线粒体明显肿胀,嵴模糊、排列紊乱甚至断裂,呈空泡状改变,未见线粒体自噬现象。线粒体形态结构定量研究显示,健康对照组Nv、Vv、Sv分别为（7.194 ± 1.434） μm－3、（4.8 ± 1.2）%、（2.42 ± 0.86） μm－1;进展期白癜风组Nv、Vv、Sv分别为（4.055 ± 0.906） μm－3、（7.4 ± 2.1）%、（3.58 ± 1.15） μm－1;稳定期白癜风组Nv、Vv、Sv分别为（5.311 ± 0.873） μm－3、（6.5 ± 1.4）%和（2.82 ± 0.94） μm－1,组间差异有统计学意义（P ＜ 0.05）。结论 白癜风皮损边缘黑素细胞线粒体受损,且进展期损伤程度大于稳定期。 【关键词】 白癜风; 黑素细胞; 线粒体; 显微镜检查,电子,透射     

几种色素减退性皮肤病的共聚焦激光扫描显微镜图像特点

目的 观察白癜风、无色素痣、进行性斑状色素减少症、贫血痣的活体共聚焦激光扫描显微镜（CLSM）特征。方法 用CLSM观察同一层面（基底层真表皮交界处）皮损处、交界处及白斑周边正常皮肤的镜下特征。结果 进展期白癜风白斑区部分区域色素完全缺失,部分区域可见残存色素环,残存之色素环结构欠完整且色素含量降低;交界处界限模糊;白斑周边正常皮肤可见部分色素环失去完整性。稳定期白癜风白斑处色素完全消失;交界处界限清晰;白斑周边正常皮肤色素环完整,折光明亮;恢复期可见到树突状、折光明亮的黑素细胞。无色素痣和进行性斑状色素减少症的CLSM表现相似：白斑处色素环结构完整,色素含量降低,折光减弱。贫血痣白斑处色素环结构和色素含量与周边正常皮肤无明显差异。结论 结合临床表现,CLSM可以作为鉴别诊断白癜风、无色素痣、进行性斑状色素减少症、贫血痣的一种辅助方法。     

无色素痣18例临床分析

目的：总结及分析无色素痣的临床及Wood灯形态特点。方法：采用问卷调查及Wood灯检查对18例无色素痣患者进行临床研究。结果：18例无色素痣患者中男10例（55.5%）,女8例（44.4%）;平均发病年龄11.3个月;孤立型7例（38.9%）,皮节型10例（55.6%）,漩涡状型1例（5.6%）;最常见的累及部位是头面部;1例（5.5%）患者有阳性家族史。所有的患者皮损在Wood灯下均呈现灰白色。结论：无色素痣最常见的累及部位是头颈部;最常见的发病类型是皮节型;Wood灯有辅助诊断价值。     

调QAlexandrite激光对太田痣黑素细胞作用机制的研究

目的 研究调Ｑ Ａｌｅｘａｎｄｒｉｔｅ激光对太田痣黑素细胞的作用机制。方法 在激光照射前及照射后各个阶段,对４例患者分别进行１１例次组织病理学观察和１４例次透射电镜观察。结果激光照射瞬间,真皮黑素细胞破坏,黑素小体空泡化,真皮中形成圆形人泡。表皮无明显损伤。     

CO2点阵激光治疗无色素痣一例首次报道

无色素痣（ND）以往采用308 nm准分子激光或光照射、自体表皮移植等治疗方法取得了一定的疗效,未有报道单用CO₂点阵激光治疗的病例。本篇首次报道1例单用CO₂点阵激光治疗ND的病例,4次治疗后基本复色,无明显不良反应。     

Expression of the c-kit receptor in hypomelanosis: a comparative study between piebaldism, naevus depigmentosus and vitiligo

In order to investigate possible alterations in c-kit protein expression on epidermal melanocytes in different hypopigmentary disorders, we have examined skin specimens from one patient with piebaldism, one patient with naevus depigmentosus, and five patients with vitiligo. Cryosections were examined by immunohistochemistry using monoclonal antibodies against the c-kit protein (YB5.B8) and melanosomes (TA99). In piebaldism, hypomelanotic epidermis contained only a few TA99-positive epidermal melanocytes and no detectable c-kit protein, whereas in naevus depigmentosus the expression of c-kit protein was strong, and TA99 immunoreactivity was faint. In vitiligo lesions, no epidermal immunoreactivity for melanosomes or c-kit protein was found. Normally pigmented skin of all patients showed immunoreactivity of epidermal melanocytes for both c-kit protein and melanosomes. Different hypomelanotic lesions can thus be differentiated by absent melanocyte c-kit protein and low or no expression of melanosomal marker in piebaldism, normal c-kit but low melanosome expression in naevus depigmentosus, and the absence of all melanocyte markers in vitiligo.     

In vivo reflectance confocal microscopy imaging of vitiligo,nevus depigmentosus and nevus anemicus

Background/purpose: Hypopigmentary skin disorders such as vitiligo, nevus depigmentosus and nevus anemicus are common diseases in clinic. The lesions of these diseases could be similar to some extent, although each of them has its own characteristic clinical appearance and histological features. Clinically, the atypical lesions are often difficult to be differentiated. In vivo reflectance confocal microscopy (RCM) is a non‐invasive, repetitive imaging tool that provides real‐time images at a nearly cellular histological resolution. Our aim was to investigate the RCM features of vitiligo, nevus depigmentosus and nevus anemicus. Subjects and Methods: A total of 135 patients with a clinical diagnosis of the aforementioned diseases were included in this study. The RCM images from depigmented skin, border of the white macules, adjacent normal‐appearing skin and distant normal skin for all patients at the dermo‐epidermal junction (DEJ) level were investigated. Results: In the active phase of vitiligo (AVP), the RCM demonstrated a complete loss of melanin in lesional skin in eight (53; 15.1%) patients. In 45 patients (53; 84.9%) of the AVP, part of the bright dermal papillary rings normally seen at the DEJ level disappeared or part of the rings lost their integrity and the content of melanin decreased obviously. In 20 patients (53; 37.7%) of the AVP, highly refractile inflammatory cells could be seen within the papillary dermis in the lesional and adjacent normal‐appearing skin, which may indicate the lesion progresses. In addition, part of the dermal papillary rings showed lack of integrity or their brightness decreased in adjacent normal‐appearing skin in all the patients of the AVP. It is important to know that the RCM demonstrated an ill‐defined border. In the stable phase of vitiligo (SPV), the RCM demonstrates a complete loss of melanin in lesional skin and a clear border in 31 (41; 75.6%) patients; the content of melanin and dermal papillary rings in adjacent normal‐appearing skin show no changes. In 10 (41; 24.4%) patients, the dendritic and highly refractile melanocytes arose in the recovery phase of vitiligo, which may indicate the repigmentation of vitiligo. There are three kinds of repigmentation patterns under RCM: marginal, perifollicular and diffuse. Distant normal skin showed no difference from controls in both the active and the SPV. In all the patients with nevus depigmentosus, the content of melanin decreases obviously but the dermal papillary rings are intact. The dermal papillary rings show no differences between lesional skin and adjacent normal‐appearing skin of nevus anemicus. Conclusion: Considering our results, RCM may be useful to non‐invasively discriminate vitiligo, nevus depigmentosus and nevus anemicus in vivo.     

儿童无色素性痣106例临床分析

为了解无色素性痣的临床特征，分析总结了106例儿童无色素性痣的临床特点，对其中10例做了组织学检查。结果显示本病发病年龄早，79.2%白斑于1岁内发现：躯干部最常见，占43.4%；局限型占60.4%，节段型占39.6%；66.0%白斑边缘很不规则，部分呈锯齿状或泼溅状。组织病理学显示基底层黑素细胞数目无明显减少。其临床特征及组织学改变与其它局限性白斑不同。     

Histopathologic features in vitiligo

Nevus depigmentosus is a congenital disorder characterized by a nonprogressive hypopigmented lesion, which may not be apparent at birth. Thus, it is sometimes difficult to differentiate vitiligo from nevus depigmentosus only by clinical features. We postulated that the histologic changes in lesional and perilesional skin might be different in the 2 conditions. We took biopsies from both lesional and perilesional skin of 100 cases of vitiligo to assess the number of melanocytes, the amount of melanin, dermal inflammatory infiltrate, and other changes. We compared them with 30 cases of nevus depigmentosus. Histologically, lesions of vitiligo showed more basal hypopigmentation and dermal inflammation than perilesional normal skin. With Fontana-Masson staining, 16% of cases of vitiligo showed the presence of melanin. The ratio of pigmented area to epidermal area was 0.06% in vitiligo, whereas 17% in perilesional normal skin and 8.9% in nevus depigmentosus. In NKI/beteb staining, 12% of vitiligo showed the presence of melanocytes, and their average number was 7.68 per square millimeter. The number of melanocytes was also decreased in nevus depigmentosus but not as much as in vitiligo. We also confirmed the presence of melanocytes in 1 of 3 cases of vitiligo by electron microscopy. In conclusion, there are a few melanocytes and melanin in some cases of vitiligo. Therefore, the diagnosis of vitiligo should be made considering these points.     

Treatment of alopecia areata with 308-nm excimer lamp

Alopecia areata is considered to be a T-cell mediated autoimmune disorder. The 308-nm excimer lamp is thought to be capable of inducing T-cell apoptosis in vitro, suggesting that the lamp might be effective for the treatment of alopecia areata. We examined the effectiveness of the 308-nm excimer lamp for the treatment of alopecia areata. We recruited three patients with single alopecia areata lesions that were resistant to conventional treatment. The lesions were exposed to a 308-nm excimer lamp at 2-weekly intervals. Hair regrowth was observed in all three patients after approximately 10 treatment sessions. Our study showed that exposure to the 308-nm excimer lamp effectively induced hair regrowth in solitary alopecia areata lesions. Apart from erythema, there were no significant adverse effects. Therefore, we suggest that it may be considered as a treatment modality for recalcitrant alopecia areata.     

Clinical differences between segmental nevus depigmentosus and segmental vitiligo

Segmental nevus depigmentosus and segmental vitiligo can be difficult to differentiate from each other. Differential diagnosis of these two diseases is important because they have significantly different prognoses and psychological effects. The purpose of this study is to identify clinical clues that may be helpful in differentiating these two diseases. We enrolled 63 patients with segmental nevus depigmentosus and 149 patients with segmental vitiligo. Sex, age of onset, sites involved, dermatomal distribution, margin of lesion and presence of poliosis were evaluated in both groups. The age of onset was less than 10 years in 96.8% of segmental nevus depigmentosus and 28.9% of segmental vitiligo cases. Trunk (36.5%) and cervical (38.1%) dermatomes were the most commonly involved in segmental nevus depigmentosus and face (67.1%) and trigeminal (64.4%) dermatomes in segmental vitiligo. The average number of dermatomes involved in truncal lesions was different in segmental nevus depigmentosus and segmental vitiligo (2.71 vs 1.62, P = 0.001). Segmental vitiligo on the face, neck and trunk appeared closer to the axis than segmental nevus depigmentosus (P < 0.001). Segmental nevus depigmentosus and segmental vitiligo showed significantly different margins (90.5% and 41.6% serrated, respectively; P < 0.001). We observed clinical differences between patients with segmental nevus depigmentosus and those with segmental vitiligo. Distribution (site, distance to axis, dermatome), vertical width, margin of lesion and presence of poliosis can be helpful in differentiating segmental nevus depigmentosus and segmental vitiligo.