中华皮肤科杂志 ›› 2010, Vol. 43 ›› Issue (8): 549-554.

• 论著 • 上一篇    下一篇

无色素痣的临床、组织学和超微结构观察

严昉1,项蕾红1,李颖2,潘展砚3,郑志忠4   

  1. 1. 复旦大学附属华山医院
    2. 山东大学齐鲁医院皮肤科
    3. 上海 同济大学医学院附属同济医院性病实验室
    4. 复旦大学附属华山医院皮肤科
  • 收稿日期:2009-12-18 修回日期:2010-02-21 出版日期:2010-08-15 发布日期:2010-08-10
  • 通讯作者: 严昉 E-mail:yan2fang3@126.com
  • 基金资助:

    常见皮肤恶性肿瘤的在体共聚焦成像及量子点标记的实验研究

Clinical, histopathologic and ultrastructural characteristics of achromic naevus

  • Received:2009-12-18 Revised:2010-02-21 Online:2010-08-15 Published:2010-08-10
  • Contact: Yan Fang E-mail:yan2fang3@126.com

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摘要：

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。

关键词: 无色素痣, 色素减退, 黑素细胞, 黑素, 反射式共聚焦激光扫描显微镜

Abstract:

Objective To study the clinical, histopathologic and ultrastructural characteristics of achromic naevus （AN）. Methods Clinical data, including sex, age, age of onset, pattern of lesions, involved sites, shape and number of lesions and associated systemic diseases, were collected from 85 patients with AN. Skin melanin index was detected in 34 lesions of 19 patients with AN, 30 lesions of 12 patients with vitiligo and 64 contralateral normal skin islands of the 31 patients. Reflectance confocal microscopy （RCM） was performed to analyze the lesion, normal skin and junctional area between lesional and normal skin of 62 patients with AN. Tissue samples were obtained from lesions and perilesional normal skin of 17 patients with AN and subjected to pathological examination as well as ultrastructural study with transmission electron microscopy; also, skin biopsy specimens were immunostained for tyrosinase, HMB45, tyrosinase-related protein-1 （TRP-1）, TRP-2 and CD117. Results Of the 85 patients with AN, 23 （27.1%） developed lesions at birth, and 21 （24.7%） after 3 years of age; 72 （84.7%） had irregularly shaped lesions, 54 （63.5%） had only a single lesion. The mean melanin index and relative melanin index of AN lesions were 186.56 ± 52.86 and 80 ± 11, respectively, significantly lower than those in normal skin islands （223.88 ± 63.19 and 100, both P < 0.01）, but higher than those in depigmented lesions from 12 patients with vitiligo （128.57 ± 64.31 and 60 ± 20, both P < 0.01）. RCM revealed a decline in the number of melanocytes and brightness of melanin caps, even distribution of melanin in lesions, as well as obscure demarcation between lesions and normal skin from patients with AN. Fontana-Masson stain showed that the melanin content was lower in lesions than in perilesional skin （1810.12 ± 327.96 vs 2064.24 ± 260.41） from patients with AN. Microscopic examination demonstrated a decrease in melanocyte and melanosome number, presence of immature melanocytes at stage Ⅱ and Ⅲ in cytoplasm and dendrites of melanocytes and keratinocytes, aggregated melanosomes in affected keratinocytes in lesions of AN. In 17 patients with AN, the relative expression levels of tyrosinase and TRP-1 were 1827.35 ± 307.09 and 6102.54 ± 1642.64, respectively, in normal skin specimens, significantly higher than those in lesional skin （1477.35 ± 224.05, 5322.33 ± 1565.26, both P < 0.01）; no statistical difference was observed in the expression levels of HMB45, TRP-2 or CD117 between lesional and normal skin. Conclusions AN is an early-onset, nonfamilial aggregated, stable leukoderma with irregular margins, and in lesions of AN, the number of both melanocytes and melanosomes is decreased with the presence of immature melanosomes. The measurement of relative melanin index and reflectance confocal microscopy may offer a non-invasive approach to the diagnosis of AN.

Key words: Nevus depigmentosus, Hypopigmentary, Melanocyte, Melanin, Reflectance confocal microscopy (RCM)