Dermatologic and oral findings in a cohort of 47 patients with Papillon-Lefèvre syndrome

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 ev...

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Bibliographic Details
Published in:Journal of the American Academy of Dermatology 2003-03, Vol.48 (3), p.345-351
Main Authors: Ullbro, Christer, Crossner, Claes-Göran, Nederfors, Tommy, Alfadley, Abdullah, Thestrup-Pedersen, Kristian
Format: Article
Language:English
Subjects:
Adolescent
Adult
Age Distribution
Biological and medical sciences
Child
Child, Preschool
Cohort Studies
Dermatology
Dyskeratosis
Female
Humans
Incidence
Male
Medical sciences
Papillon-Lefevre Disease - diagnosis
Papillon-Lefevre Disease - epidemiology
Papillon-Lefevre Disease - therapy
Periodontal Diseases - diagnosis
Periodontal Diseases - epidemiology
Periodontal Diseases - therapy
Probability
Prognosis
Psoriasis - diagnosis
Psoriasis - epidemiology
Psoriasis - therapy
Quality of Life
Risk Assessment
Severity of Illness Index
Sex Distribution
Sickness Impact Profile
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Summary: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. (J Am Acad Dermatol 2003;48:345-51.)
ISSN:0190-9622
1097-6787
1097-6787
DOI:10.1067/mjd.2003.197