title: Dermatologic and oral findings in a cohort of 47 patients with Papillon-Lefèvre syndrome.
abstract: Papillon-Lefèvre syndrome is an autosomal recessive disorder characterized by palmoplantar hyperkeratosis and early development of aggressive periodontal infection. The aims of this study were to rank the severity of dermatologic and oral affections using a semiquantitative scoring system, and to evaluate whether the severity of the dermatologic changes were correlated to age, degree of periodontal infection, or both. The study included 47 patients with Papillon-Lefèvre syndrome. With no exception both skin and oral changes developed early in life. The dermatologic involvement showed no correlation with age, whereas the periodontal infection was significantly worse in young children with deciduous teeth. A strong correlation was found between the condition of feet and hands, although the scores for the feet were significantly higher. No significant correlation could be demonstrated between the level of periodontal infection and severity of skin affections, supporting the concept that these 2 major components of Papillon-Lefèvre syndrome are unrelated to each other.
authors.affiliation: Department of Dentistry, Section of Dermatology, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia. ullbro@kfshrc.edu.sa
authors.forename: Christer
authors.initials: C
authors.lastname: Ullbro
authors.forename: Claes-Göran
authors.initials: CG
authors.lastname: Crossner
authors.forename: Tommy
authors.initials: T
authors.lastname: Nederfors
authors.forename: Abdullah
authors.initials: A
authors.lastname: Alfadley
authors.forename: Kristian
authors.initials: K
authors.lastname: Thestrup-Pedersen
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006801:Humans; D015994:Incidence; D008297:Male; D010214:Papillon-Lefevre Disease / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy; D010510:Periodontal Diseases / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy; D011336:Probability; D011379:Prognosis; D011565:Psoriasis / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy; D011788:Quality of Life; D018570:Risk Assessment; D012720:Severity of Illness Index; D017678:Sex Distribution; D018434:Sickness Impact Profile