掌跖角化—牙周破坏综合征临床诊断与治疗

Clinical diagnosis and treatment in Papillon-Lefevre Syndrome

目的:了解掌跖角化一牙周破坏综合征(PLS)临床诊断与治疗。方法:收集并调查一例掌跖角化一牙周破坏综合征病例,进行家谱分析,对其进行全口义齿修复。并对相关文献进行回顾。结果:掌跖角化一牙周破坏综合征是一种罕见的以严重牙周组织破坏及掌跖、膝盖、肘部等部位皮肤过度角化为特征的常染色体隐性遗传病,发病年龄早,最终可导致全口牙列缺失。全口义齿修复可获得较满意的效果。结论:早期发现、早期治疗对改善PLS的预后是非常重要的。

Objective： To understand clinical diagnosis and treatment in Papillon-Lefevre syndrome （PLS）. Method： This paper described a case of PLS, performed genealogies analysis and complete denture restoration, then briefly reviewed the relevant literature. Result： Papillon-Lefevre syndrome is a rare autosomal recessive disorder, characterized by hyperker- atosis involving the palms, soles,elbows,knees and severe early-onset periodontitis,finally leading to loss of teeth. Conclu- sion： Early detection, early treatment is the key to improve the prognosis of PLS.