关键词: 掌跖角化- 牙周破坏综合征, 常染色体隐性遗传, 组织蛋白酶C, 多形核白细胞

Abstract:

Papillon- Lefèvre syndrom（e PLS）is an extremely rare inherited disease as an autosomal recessive trait. The disorder is characterized by diffuse palmoplantar keratoderma and premature loss of both deciduous and permanent teeth. This paper described a case of PLS with classic clinical features and briefly reviewed the relevant literature.

Key words: Papillon- Lefèvre syndrome, autosomal recessive, cathepsin C, polymorphonuclear leukocyte