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色素脱失痣:67例患者的临床特征及组织病理学特点

Nevus depigmentosus: clinical features and histopathologic characteristics in 67 patients.

作者信息

Lee H S, Chun Y S, Hann S K

机构信息

Department of Dermatology, Yonsei University College of Medicine, Seoul, Korea.

出版信息

J Am Acad Dermatol. 1999 Jan;40(1):21-6. doi: 10.1016/s0190-9622(99)70524-4.

Abstract

BACKGROUND

Nevus depigmentosus is defined as a congenital nonprogressive hypopigmented macule or patch that is stable in its relative size and distribution throughout life. The pathogenesis and histopathologic characteristics of nevus depigmentosus is not yet fully established.

OBJECTIVE

The purpose of this study was to investigate the clinical and histopathologic characteristics of nevus depigmentosus as well as its pathogenesis.

METHODS

A clinical survey was done with 67 patients diagnosed as having nevus depigmentosus. Two skin biopsy specimens each were taken from 18 patients: one from the central part of the depigmented lesion and another from the border of the lesion, including perilesional normal skin. The sections were stained with hematoxylin-eosin, Fontana-Masson, and S-100 protein. Ultrastructural evaluation was also done to detect changes of the melanocytes.

RESULTS

The lesions were mostly present before 3 years of age (92.5%), but some lesions also appeared later in childhood (7.5%). The back and buttocks were the most commonly affected sites, followed by the chest and the abdomen, the face, the neck, and the arms. Forty patients (59.7%) had the isolated type of nevus depigmentosus and 27 patients (40.3%) had the segmental type. Histopathologic studies showed that the staining ability of Fontana-Masson in nevus depigmentosus lesions decreased compared with perilesional normal skin. However, there were no changes in the numbers of melanocytes identified as S-100-positive cells in the basal layer. Electronmicroscopic studies revealed a great reduction in the number of melanosomes in melanocytes and some membrane-bound aggregated melanosomes were observed in keratinocytes.

CONCLUSION

The results of this study support the hypothesis that nevus depigmentosus is caused by the functional defects of melanocytes and the morphologic abnormalities of melanosomes.

摘要

背景

色素脱失痣被定义为一种先天性、非进行性色素减退斑或斑片,其相对大小和分布在一生中保持稳定。色素脱失痣的发病机制和组织病理学特征尚未完全明确。

目的

本研究旨在探讨色素脱失痣的临床和组织病理学特征及其发病机制。

方法

对67例诊断为色素脱失痣的患者进行了临床调查。从18例患者身上各取两块皮肤活检标本:一块取自色素减退病变的中央部分,另一块取自病变边界,包括病变周围的正常皮肤。切片进行苏木精-伊红染色、Fontana-Masson染色和S-100蛋白染色。还进行了超微结构评估以检测黑素细胞的变化。

结果

病变大多在3岁前出现(92.5%),但也有一些病变在儿童期后期出现(7.5%)。背部和臀部是最常受累的部位,其次是胸部和腹部、面部、颈部和手臂。40例患者(59.7%)为孤立型色素脱失痣,27例患者(40.3%)为节段型。组织病理学研究表明,与病变周围正常皮肤相比,色素脱失痣病变中Fontana-Masson染色能力降低。然而,基底层中被鉴定为S-100阳性细胞的黑素细胞数量没有变化。电子显微镜研究显示黑素细胞中黑素小体数量大幅减少,并且在角质形成细胞中观察到一些膜结合的聚集黑素小体。

结论

本研究结果支持色素脱失痣是由黑素细胞功能缺陷和黑素小体形态异常引起的这一假说。

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