Papillon-Lefèvre syndrome: a case report. 1998

L Cooke, and S Bonnett, and L Briggs
Community Dental Department, North Downs Community Health Trust, Yateley Medical Centre, Hampshire.

This case report discusses a very rare condition; Papillon-Lefèvre Syndrome. This is an autosomal recessive condition in which the main features are hyperkeratosis of the palms and the soles and severe periodontal destruction.

UI MeSH Term Description Entries
D010214 Papillon-Lefevre Disease Rare, autosomal recessive disorder occurring between the first and fifth years of life. It is characterized by palmoplantar keratoderma with periodontitis followed by the premature shedding of both deciduous and permanent teeth. Mutations in the gene for CATHEPSIN C have been associated with this disease. Haim-Monk Syndrome,Keratosis Palmoplantar Periodontopathy,Keratosis Palmoplantaris with Periodontopathia,Papillon Lefevre Disease,Papillon-Lefevre Syndrome,Haim Monk Syndrome,Keratosis Palmoplantar Periodontopathies,Palmoplantar Periodontopathies, Keratosis,Papillon Lefevre Syndrome
D010510 Periodontal Diseases Pathological processes involving the PERIODONTIUM including the gum (GINGIVA), the alveolar bone (ALVEOLAR PROCESS), the DENTAL CEMENTUM, and the PERIODONTAL LIGAMENT. Parodontosis,Pyorrhea Alveolaris,Disease, Periodontal,Diseases, Periodontal,Parodontoses,Periodontal Disease
D011862 Radiography, Panoramic Extraoral body-section radiography depicting an entire maxilla, or both maxilla and mandible, on a single film. Orthopantomography,Panoramic Radiography,Pantomography,Orthopantomographies,Panoramic Radiographies,Pantomographies,Radiographies, Panoramic
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man

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