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