Eponym: Papillon-Lefevre syndrome.

[papillon-lefèvre syndrome]

Papillon-Lefevre Syndrome (PLS ) is a very rare autosomal recessive disorder characterized by palmoplantar hyperkeratosis and severe early onset periodontitis , affecting the primary and permanent dentition . The syndrome was first described by Papillon and Lefevre in 1924 . Genetic , immunologic , and microbiologic factors are suggested as responsible for the initiation and progression of the disease . A point mutation of cathepsin C gene has recently been detected in PLS . A multidisciplinary approach is important for management .The prognosis has improved with the early recognition of the syndrome , effective professional supervision , and home care .