Palmoplantar keratosis-periodontal destruction syndrome (Papillon-Lefèvre syndrome, PLS) is a rare autosomal recessive disease characterized by early periodontitis and hyperkeratosis of the palmoplantar skin occurring in deciduous and permanent teeth. It was first reported and named by Papillon and Lefèvre in France in 1924. The incidence rate of this disease is about 1 to 4 parts per million, 1/3~2/3 of the families are consanguineous marriages, and the incidence rate of this disease is the same for men and women[1]。 One case was admitted to the Department of Children's Stomatology, Stomatology Hospital, Peking University School of Stomatology in 2004, and followed up for 10 years. The report is now as follows.
