先天性色素痣合并白癜风2例

报告2例白癜风合并先天性色素痣。例1的初发白斑在左上肢先天性毛痣周围,表现为晕痣,1个月后身体其他部位出现多发白斑;例2为节段型白癜风患者,除了在身体其他部位出现白斑外,在右足背先天性色素痣周围出现白斑,表现为痣周围白癜风。白癜风合并先天性色素痣这一现象值得进一步研究。     

Reconstitution of the histologic characteristics of a giant congenital nevomelanocytic nevus employing the athymic mouse and a cultured skin substitute

This study addresses the development of an animal model for human giant congenital nevomelanocytic nevi (GCNN). Skin grafts were made from 1) non-involved split-thickness skin from a 12-month-old GCNN patient, 2) nevus split-thickness skin from the same GCNN patient, 3) nevus full-thickness skin, and 4) cadaveric human split-thickness skin. For groups 1) and 2), human epidermal and dermal cells were enzymatically isolated and expanded in tissue culture. Composite grafts were made by placing the cultured dermal cells into a collagen-glycosaminoglycan (GAG) matrix, followed by placement of the epidermal cells onto the opposite, laminated side of the matrix. All grafts were placed onto full-thickness wounds of athymic mice and biopsies were obtained from 6 to 38 weeks later for light microscopy including S-100 immunoperoxidase staining, and electron microscopy. The GCNN cultured skin mice (group 2) developed black, raised skin in the healed wounds. None of the group 1 mice developed lesions, grossly or histologically. All of the nevus full-thickness mice retained the nevus grossly. Histopathologic examination at 38 weeks of the black, raised plaques of group 2 demonstrated a reconstituted dermis similar to group 3. Nevus cells were larger and more epithelioid in the upper dermis, as seen with true GCNN. These nevomelanocytes were not seen in the dermis at 24 weeks, suggesting that the nevus cells migrated from the epidermal component of the cultured graft to the dermis during this time frame (24-38 weeks). The melanocyte identity of these cells was confirmed with S-100 immunoperoxidase staining and electron microscopy. These findings are unique to this composite cultured graft system. The ability to culture specific types of melanocytes and place them int skin substitutes on athymic mice provides a basis for the study of GCNN and melanocyte biology in vivo.     

E-cadherin expression in the subepithelial nevus cells of the giant congenital nevocellular nevi (GCNN) correlates with their migration ability in vitro

BACKGROUND: Giant congenital nevocellular nevi (GCNN) are histologically characterized by the broad distribution of nevus cells in the epidermis and dermis. OBJECTIVE: To characterize E-cadherin in GCNN and define its role in nevic cell migrations. METHODS: Twenty-four cases were immunohistochemically examined and in five cases cells were isolated for primary culture for migration assays. RESULTS: The nevus cells in the superficial region showed the immunoreactivity of E-cadherin in a membranous pattern, but those in the deep part of dermis had little immunoreactivity. Ultra-structural analysis of the superficial nevus cells revealed that E-cadherin immunodeposits in the fibrillar processes around the cell body in a spotted pattern. This distribution pattern is quite different from that in the adherens junction of skin squamous epithelial cells. Boyden chamber experiments were performed using primary cultures of intradermal nevus cells. EDTA pretreatment reduced cell migration to the E-cadherin positive side when the E-cadherin positive population was relatively large in the primary cultures. CONCLUSIONS: These results indicate that E-cadherin in the nevus cells may affect nevus cell motility rather than intercellular attachment.     

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。     

Resolution and Recurrence of Vitiligo Following Excision of Congenital Melanocytic Nevus

Abstract: Vitiligo associated with halo congenital melanocytic nevus (CMN) is rare. There are limited reports in the literature, especially with regard to CMN excision. We present the case of a 5‐year‐old girl who presented with vitiligo of the periorbital and axillary regions and halo formation around CMN of the buttock. The lesion was excised, and all areas of vitiligo improved, but 18 months postoperatively, a halo of depigmentation appeared around the excision scar and later in the periorbital and axillary regions. In review of literature, there is only one report of excision of halo CMN and resultant improvement of vitiligo. Although initial resolution of vitiligo in this case was promising, the recurrence indicates that this complex process is not reliably controlled with excision of the inciting lesion.     

Elevated serum antimelanocyte antibody level in cerebriform intradermal nevus with vitiligo

We describe a rare, but typical case of cerebriform intradermal nevus associated with vitiligo. A 45-year-old man had a patch of alopecia over his vertex scalp for 15 years. The microscopic findings of the biopsy revealed a typical deep-seated intradermal nevus and neuroid differentiation with a few pigments. Three hypopigmented patches developed on the forehead, cheek and index finger five years after the scalp lesion, with loss of both melanocytes and melanins. In addition, no dopa reactions were present. Compared to normal controls, the serum anti-melanocyte antibody level in the patient was elevated as determined by cellular enzyme-linked immunosorbent assay (cellular ELISA). This is the first reported case with elevation of serum antimelanocyte antibody level of cerebriform intradermal nevus with vitiligo. This antibody's presence may be related to the occurrence of the vitiligo in patient with cerebriform intradermal nevus.     

Association of giant congenital melanocytic nevus, halo nevus and vitiligo in a 75-year-old patient

A giant congenital melanocytic nevus represents a rare condition. The halo phenomenon may be seen in congenital or acquired melanocytic nevi. In the literature, association of halo nevus and giant congenital melanocytic nevus is rare and the association of both with vitiligo even more rare. A 75-yearold woman at first consultation complained of a hyperchromic bluish-brown hairy macula on the lower back, buttocks and thighs present since birth and an achromic halo of onset three years ago. The histological features were consistent with congenital melanocytic nevus and halo nevus, respectively. After two years the patient developed achromic areas in normal skin, histologically consistent with vitiligo. The authors emphasize the rarity of this triple combination, the patient's age and the absence of malignant degeneration to date.     

Resolution of vitiligo following excision of halo congenital melanocytic nevus: a rare case report

Halo congenital melanocytic nevus (CMN) associated with vitiligo is rare, especially with regard to CMN excision. Only two reports of excision of halo CMN following repigmentation of vitiligo are found in the literature. We present a case of a girl with halo CMN and periorbital vitiligo. The halo CMN was excised and followed by spontaneous improvement of vitiligo. The result suggests excision of the inciting lesion may be a promising way to control vitiligo.     

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.     

不同治疗方法对晕痣疗效的影响因素分析

目的 探讨晕痣临床特征及疗效影响因素。方法 对2016年2 - 11月门诊晕痣患者临床资料进行前瞻性研究,分析影响晕痣疗效相关因素。结果 250例患者共293处皮损,单发219例（87.6%）,多发31例（12.4%）;皮损位于躯干部154处（52.6%）、面颈部127处（43.3%）,直径5 ~ 20 mm。248例（99.2%）晕痣在自然病程中未完全自发消退。122例（48.8%）患者伴发白癜风。单因素分析显示,年龄、皮损数目、伴发白癜风和治疗方法是晕痣疗效的影响因素,多因素logistic回归分析显示年龄 ≤ 19岁或 ≥ 40岁、病程 > 1年、皮损单发、不伴发白癜风、接受祛痣治疗是晕痣治疗有效的独立影响因素。结论 大部分晕痣不能完全自发性消退。CO2激光或手术祛痣联合外用药是治疗晕痣的有效方法,未伴发白癜风晕痣患者可优先祛痣,伴白癜风者可在白癜风稳定后祛痣。多发晕痣或白癜风面积较大者更易复发,应密切随访。     

Halo congenital nevus.

A case of halo congenital nevus is reported in a fifteen year old Mexican-American girl with pre-existing vitiligo.     

Behavior of pigment cells on lesions of the pigmented venus with vitiligo.

When vitiligo occurred on lesions of the pigmented nevus, the behavior of pigment cells in this nevus was investigated. Three cases of giant hairy nevi, seven cases of moles, three cases of Mongolian spots and eleven specimens in nine cases of halo nevi were used. Giant hairy nevi combining with vitiligo showed intensive decreases in nevus cells, particularly superficial A and B-type nevus cells. The epidermal dopa-positive melanocytes and melanin granules in the epidermis decreased, but still remained. On the other hand, moles in vitiligo showed an almost complete disappearance of epidermal dopa-positive melanocytes and melanin granules in the epidermis; nevus cells in the dermis decreased only slightly. Mongolian spots with vitiligo showed an epidermis similar to vitiligo, but the dermal melanocytes were hardly changed. Halo nevi exhibited an intensive decrease and degeneration of nevus cells and marked lymphocytic infiltration. Some of them showed disappearance of epidermal dopa-positive melanocytes and melanin granules in the epidermis. The characteristic findings of vitiliginous skin are mostly restricted to epidermis. In contrast, however, it is interesting to note that, on the lesions of nevocellular nevi with vitiligo, the dermis also exhibited some decrease and degeneration of nevus cells and lymphocytic infiltration.     

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.     

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.     

A rare combination of sebaceoma with carcinomatous change (sebaceous carcinoma), trichoblastoma,and poroma arising from a nevus sebaceus

We report a case of a 48‐year‐old Malay female who presented with multiple tumors arising from a large nevus sebaceus on her right parieto‐temporal scalp. Histologically, the tumors corresponded to a sebaceoma with carcinomatous change, a poroma and a trichoblastoma. Immunohistochemical staining of the sebaceous tumor with p53 showed strong within the areas of carcinomatous change, while there was negative to weak staining within the sebaceoma‐like areas. A discussion on the potential secondary neoplasms from a nevus sebaceus ensues, with a review of this literature on multiple tumors from a nevus sebaceus.     

Development of Halo Nevus Around Nevus Spilus as a Central Nevus,and the Concurrent Vitiligo

Halo nevus is a benign melanocytic nevus that is surrounded by a hypopigmented zone. The most frequent association with halo nevus is vitiligo, and this also appears in nearby regions, as well as at other remote sites. Although the mechanism for developing the depigmentation around nevus spilus is uncertain an immunologic process may be responsible for the finding of inflammatory infiltrates of the upper dermis in the depigmented lesions. We report here on a 13-year-old boy who showed a depigmented zone around a nevus spilus on the right side of his neck with simultaneous vitiligo lesions on the face.     

153例儿童白癜风临床分析

分析了１５３例儿童白癜风的临床资料。结果显示儿童白癜风发病年龄早，以５－７岁娄病高峰平均发病年龄６、７岁，伴晕痣患儿病情发展快。其中节段性白癜风占１７．６５％。其临床表现不同于非节段性白癜风。     

巨大先天性色素痣皮损处出现白毛1例

报告巨大先天性色素痣皮损处出现白毛1例。患者男，34岁。患巨大先天性色素痣34年。2年前发现胸部色素痣上部分胸毛变白，患者无白瘢风、晕痣及皮肤色素减退。先天性巨大色素痣上出现白毛值得研究。     

Clinical course of 44 cases of localized type vitiligo

Vitiligo is often classified into three types, generalized, segmental, and localized, on the basis of their distribution pattern. It is also classified into type A (non-dermatomal or non-segmental) and type B (dermatomal or segmental) vitiligo on the basis of both the distribution pattern and physiological function. The natural courses of type A and type B vitiligo are characteristic and quite different from each other. Whereas type A vitiligo appears at any age and progresses throughout the patient's life span, type B vitiligo affects the young and stabilizes within a few years. Segmental type vitiligo corresponds to type B, and generalized type vitiligo is the late stage of type A. However, no one has observed the course and character of localized type vitiligo, and its nosological position in A/B classification is unclear. We followed 44 cases of localized type vitiligo for periods of 6 months to 8 years. In 3 of the 44 patients, new white patches developed within the same dermatome as their affected areas in the first 12 months and stabilized in a short period. Therefore, these patients were diagnosed as type B vitiligo. In 15 patients, vitiligo developed in other dermatomal areas; the earliest case at 9 months, and others later on. The new white patches continue to develop for a long period, so these patients were diagnosed with type A vitiligo. In 26 of the 44 patients, the vitiligo remained localized within the period of observation. It is concluded that most localized type vitiligo is the early stage of type A, but a small number of cases belong to the early stage of type B.     

白癜风患者406例临床调查分析

目的探讨白癜风患者的临床特点、发病机理,以期对临床诊断、判断疾病转归及治疗提供依据。方法对406例白癜风门诊患者进行问卷调查,对各种涉及的因素及临床表现进行统计分析。结果白癜风患者以16～20岁为发病高峰,该年龄段占发病总数的16.34%。男女发病之比为1.16:1,白癜风各型中散发型244例(60.10%),节段型8例(1.97%),有家族史者64例(15.76%)。伴晕痣28例(6.90%),伴发其他疾病者64例(15.76%)。结论白癜风发病高峰为青少年;有家族史患者发病年龄早于无家族史患者;合并晕痣患者首发年龄早于无合并晕痣患者;各型中以散发型患病人数最多,节段型最少;皮疹瘙痒和同形反应多见于进展期;部分患者白斑有自愈倾向,且自愈与精神因素关系密切。