弹性纤维假黄瘤29例临床与病理分析  

Clinical and pathological analysis of 29 cases of pseudoxanthoma elasticum

作　　者：吴正胜[1] 晋亮[1] 马东来[2] 阎衡[3] 耿松梅[4] 陈柳青[5] 谭德友[6] 李勇 袁伟[8] 颜廷凯[8] 杨璐婷[1] 张伟[8] 王刚[1] 

机构地区：[1]第四军医大学西京皮肤医院,西安710032 [2]中国医学科学院北京协和医学院北京协和医院皮肤科 [3]第三军医大学西南医院皮肤科 [4]西安交通大学第二附属医院皮肤科 [5]武汉市第一医院皮肤科 [6]广东省佛山市第二人民医院皮肤科 [7]湖北省宜昌市第二人民医院皮肤科 [8]遵义医学院附属医院皮肤科

出　　处：《中华皮肤科杂志》2013年第10期698-701,共4页Chinese Journal of Dermatology

摘　　要：目的探讨弹性纤维假黄瘤患者的临床与组织病理学特点。方法回顾性分析29例弹性纤维假黄瘤患者的临床资料，并对其皮肤组织病理切片、弹性纤维染色、钙染色等进行综合分析。结果男：女为1：8．7；平均发病年龄（22．6±11．2）岁；在29例弹性纤维假黄瘤患者中，发病部位位于颈部29例（100％），腋窝11例（37．9％），腹部5例（17．2％），腹股沟4例（13．8％）。29例HE染色均可见到真皮中层特征性的卷曲样结构，弹性纤维染色证实是弹性纤维肿胀、断裂、卷曲和变性，钙染色证实变性的弹性纤维上有钙的沉积。5例患者进行了眼底和心血管系统的检查，其中2例可见眼底以视盘为中心的放射状血管条纹改变，较严重的1例可见眼底的少量出血和视力轻微受损，5例患者心血管系统均未见异常；29例患者中有1例伴甲状腺功能亢进。结论弹性纤维假黄瘤患者临床表现具有一定的特征性，结合组织病理学检查、弹性纤维染色，一般能及时做出诊断，避免漏诊和误诊。Objective To investigate the clinical and pathological features of pseudoxanthoma elasticum. Methods Clinical data on 29 cases of pseudoxanthoma elasticum were retrospectively collected. Clinical manifestations, treatment outcome and findings from histopathological examination, Verhoeff-van Gieson staining and von Kossa＇s staining were studied. Results Among these patients, the ratio of male to female was 1 ： 8.7, with an average age at onset of （22. 6 ＋ 11.2） years. Lesions were distributed in the neck （29 cases, 100%）, axillary fossa （11 cases, 37.9%）, abdomen （5 cases, 17.2%）, or groin （4 cases, 13.8%）. Hematoxylin-eosin （HE） staining of lesions consistently revealed characteristic curl-like structures in mid dermis, which were confirmed to be swelling, disrupted, curled, and degenerated elastic fibers by Verhoeff-van Gieson staining, yon Kossa＇s staining showed calcium deposition on the degenerated elastic fibers. Of five patients receiving fundus ocnli examination and cardiovascular system examination, two had angioid streaks radiating from the video disc, one even had mild retinal hemorrhage and vision impairment, none showed cardiovascular abnormalities. Hyperthyroidism was detected in one of the 29 patients. Conclusions Pseudoxanthoma elasticum has distinct clinical manifestations, and it is usually not difficult to diagnose pseudoxanthoma elasticum according to clinical manifestations as well as findings from histopathological examination and Verhoeff-van Gieson staining.

关 键 词：假黄瘤 弹性 病理学 诊断 

分 类 号：R739.5[医药卫生—肿瘤]