Papillon-Lefévre 综合征(PLS)为法国学者 Papillon 和 Lefévre 于1924年首先描述,至今世界文献共报告100余例,分散于世界各地,其发病率在一般人群中估计为1～4/百万。近些年来,对这一遗传性综合征有了一些新的认识,特别是在治疗方面,取得了可喜的进展。临床表现PLS 又称掌跖角化伴发牙周病综合征,典型的临床特征一般都在3岁前开始出现,无性别和种族差异。主要有以下两个方面的表现:①皮肤:皮肤病变往往先于口腔病变 Papillon-Lefévre Syndrome (PLS) was first described by the French scholar Papillon and Lefévre in 1924. To date, more than 100 cases of Papillon-Lefévre syndrome have been reported in the world and the incidence is estimated to be 1 to 4 per million in the general population. In recent years, some new understanding has been given to this hereditary syndrome, especially in the area of ​​treatment, and remarkable progress has been made. Clinical manifestations of PLS, also known as palmoplantar keratosis with periodontal syndrome, the typical clinical features are generally started before the age of 3, no gender and racial differences. Mainly in the following two aspects of performance: ① skin: skin lesions tend to precede the oral lesions