CAJ | 학술논문

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.
목적 탐토탄성섬유가황류환자적림상여조직병이학특점.방법 회고성분석29례탄성섬유가황류환자적림상자료,병대기피부조직병리절편、탄성섬유염색、개염색등진행종합분석.결과 남∶녀위1∶8.7；평균발병년령(22.6±11.2)세；재29례탄성섬유가황류환자중,발병부위위우경부29례(100％),액와11례(37.9％),복부5례(17.2％),복고구4례(13.8％).29례HE염색균가견도진피중층특정성적권곡양결구,탄성섬유염색증실시탄성섬유종창、단렬、권곡화변성,개염색증실변성적탄성섬유상유개적침적.5례환자진행료안저화심혈관계통적검사,기중2례가견안저이시반위중심적방사상혈관조문개변,교엄중적1례가견안저적소량출혈화시력경미수손,5례환자심혈관계통균미견이상；29례환자중유1례반갑상선공능항진.결론 탄성섬유가황류환자림상표현구유일정적특정성,결합조직병이학검사、탄성섬유염색,일반능급시주출진단,피면루진화오진.
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.von Kossa's staining showed calcium deposition on the degenerated elastic fibers.Of five patients receiving fundus oculi 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.