title: Eponym: Papillon-Lefevre syndrome.
abstract: 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.
authors.affiliation: Department of Pediatric Gastroenterology, Faculty of Medicine, Gazi University, Besevler, 06500, Ankara, Turkey.
authors.forename: Buket
authors.initials: B
authors.lastname: Dalgıc
authors.forename: Aysegul
authors.initials: A
authors.lastname: Bukulmez
authors.forename: Sinan
authors.initials: S
authors.lastname: Sarı
mesh_terms: D020789:Cathepsin C / Q000235:genetics*; D002648:Child; D042241:Early Diagnosis; D004850:Eponyms; D006801:Humans; D010214:Papillon-Lefevre Disease / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy*; D017354:Point Mutation; D011379:Prognosis
chemical_list: D020789:Cathepsin C