掌跖角化-牙周破坏综合征1例  

A case report of Papillon-Lefèvre syndrom

作　　者：胡婷婷 孙瑜[1] 叶芳[1] HU Tingting;SUN Yu;YE Fang(330006,Department of Periodontics Affiliated Stomatological Hospital of Nanchang University,Key Laboratory of Oral Biomedicine,Jiangxi Province,China)

机构地区：[1]南昌大学附属口腔医院牙周病科.江西省口腔生物医学重点实验室,330006

出　　处：《实用口腔医学杂志》2018年第6期857-860,共4页Journal of Practical Stomatology

摘　　要：掌跖角化-牙周破坏综合征(Papillon-Lefèvre syndrome,PLS)是一种在临床上以早发的快速性牙周组织破坏,掌跖等部位皮肤为主的过度角化为特征的常染色体隐性遗传病,起病早,常致乳牙及恒牙过早脱落。PLS病例极少,本文1例具有典型PLS临床特点的病例报道。Papillon-Lefèvre syndrome(PLS)is an autosomal recessive disorder,charaterized by hyperkeratosis involving the main parts of palmoplantar skin and severe early-onset periodontitis,usually leading to the premature loss of both primary and secondary teeth.PLS is a rare disease,this paper describes a case of PLS with representative clinical features,pathogenesis and treatment.

关 键 词：掌跖角化-牙周破坏综合征(PLS) 常染色体隐性遗传 

分 类 号：R781.4[医药卫生—口腔医学]