title: Papillon-Lefèvre syndrome--successful treatment with a combination of retinoid and concurrent systematic periodontal therapy: case reports.
abstract: Papillon-Lefevre syndrome is a rare autosomal-recessive congenital differentiation disorder; the external signs are hyperkeratosis of the palms and soles. Intraorally, the most salient manifestations are dystrophic periodontal problems that affect both the primary and permanent dentitions and frequently lead to premature tooth loss. Two children were treated with acitretin 0.5 mg/kg of body weight per day from November 1992 to November 1993, and another child since October 1993. Concurrently, the children received professional oral hygiene care (scaling, root planing, and curettage). The combination of retinoid therapy and periodontal treatment improved the dermatologic and periodontal conditions.
authors.affiliation: Department of Periodontology, Heinrich Heine University Düsseldorf, Center of Dentistry, Düsseldorf, Germany.
authors.forename: S
authors.initials: S
authors.lastname: Kressin
authors.forename: A
authors.initials: A
authors.lastname: Herforth
authors.forename: S
authors.initials: S
authors.lastname: Preis
authors.forename: V
authors.initials: V
authors.lastname: Wahn
authors.forename: H G
authors.initials: HG
authors.lastname: Lenard
mesh_terms: D017255:Acitretin / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D017742:Dental Care for Chronically Ill; D005260:Female; D005891:Gingivitis / Q000209:etiology / Q000628:therapy; D006801:Humans; D007641:Keratolytic Agents / Q000627:therapeutic use*; D008297:Male; D010214:Papillon-Lefevre Disease / Q000150:complications / Q000188:drug therapy / Q000628:therapy*; D010510:Periodontal Diseases / Q000188:drug therapy / Q000209:etiology / Q000628:therapy*; D014086:Tooth Mobility / Q000209:etiology / Q000628:therapy
chemical_list: D007641:Keratolytic Agents; D017255:Acitretin