Kim Sung Kwon, Kang Hee Young, Lee Eun-So, Kim You Chan
Department of Dermatology, Ajou University School of Medicine, Suwon, Korea.
J Am Acad Dermatol. 2006 Sep;55(3):423-8. doi: 10.1016/j.jaad.2006.04.053. Epub 2006 May 30.
Nevus depigmentosus (ND) is known to be a rare congenital, nonprogressive disorder characterized by a hypopigmented lesion that remains stable over time. There have been only few studies of clinical and histopathologic characteristics of ND, and the etiopathogenesis is not fully established.
The purpose of this study was to investigate the clinical and histopathologic characteristics of ND.
A clinical survey was carried out with 60 patients given the diagnosis of ND. Punch biopsies (2 mm) from lesional and perilesional normal skin were performed. The sections were stained with hematoxylin-eosin, Fontana-Masson, antibodies to S-100 protein, MART-1, NKI/beteb, CD1a, CD3, CD20, and CD68.
The lesions were usually present before the age of 3 years (68.3%), but some lesions appeared later in childhood (31.7%). In all, 27 patients (45%) had one lesion, but there were 14 patients (23.3%) who had more than 10 lesions. Fontana-Masson stain showed that the amount of melanin was significantly decreased in ND skin compared with perilesional normal skin. Melanocyte counts were significantly decreased in ND skin when stained with antibodies to GP-100 and MART-1. However, there were no significant differences in the number of melanocytes identified as S-100 protein-positive cells. There were no significant differences in histologic findings or dermal inflammatory infiltrates between ND skin and perilesional normal skin.
Only 29 patients (48.3%) were followed up, and the average follow-up period after initial diagnosis was relatively short (68 months); therefore, these overall results may not be representative of the clinical course of the patients.
Only 18 patients (30.0%) presented with ND since birth and only 27 patients (45.0%) had one lesion. Both the amount of melanin and the number of melanocytes in ND skin were decreased in patients with ND. Therefore, both clinical and histologic findings should be considered together to make a diagnosis of ND.
色素脱失痣(ND)是一种罕见的先天性、非进行性疾病,其特征为色素减退性损害,且随时间保持稳定。关于色素脱失痣的临床和组织病理学特征的研究较少,其发病机制尚未完全明确。
本研究旨在探讨色素脱失痣的临床和组织病理学特征。
对60例诊断为色素脱失痣的患者进行临床调查。从皮损及皮损周围正常皮肤取2mm钻孔活检组织。切片进行苏木精-伊红染色、Fontana-Masson染色、抗S-100蛋白抗体、MART-1、NKI/beteb、CD1a、CD3、CD20和CD68染色。
皮损通常在3岁前出现(68.3%),但部分皮损在儿童期后期出现(31.7%)。共有27例患者(45%)有1处皮损,但有14例患者(23.3%)有超过10处皮损。Fontana-Masson染色显示,与皮损周围正常皮肤相比,色素脱失痣皮肤中的黑色素量显著减少。用抗GP-100和MART-1抗体染色时,色素脱失痣皮肤中的黑素细胞计数显著减少。然而,作为S-100蛋白阳性细胞识别的黑素细胞数量没有显著差异。色素脱失痣皮肤与皮损周围正常皮肤在组织学表现或真皮炎症浸润方面没有显著差异。
仅29例患者(48.3%)进行了随访,初次诊断后的平均随访期相对较短(68个月);因此,这些总体结果可能不代表患者的临床病程。
仅18例患者(30.0%)自出生即患有色素脱失痣,仅27例患者(45.0%)有1处皮损。色素脱失痣患者的色素脱失痣皮肤中黑色素量和黑素细胞数量均减少。因此,诊断色素脱失痣时应综合考虑临床和组织学表现。