Papillon-Lefevre syndrome: clinical presentation and a brief review.
The Papillon-Lefevre syndrome (PLS) was first described by Papillon and Lefevre in 1924. It is an autosomal recessive disorder characterized by a diffuse palmoplanter hyperkeratosis and rapidly progressive and devastating periodontitis, affecting the primary as well as the permanent dentition, attributed to a point mutation of the cathepsin C gene. This paper presents a clinical presentation and a brief review of its etiology and treatment modalities.
Dhanrajani
Parmanand J
PJ
HCF Dental Centre, Sydney, NSW, Australia. p1954sa@yahoo.com.au
D010520:Aggressive Periodontitis / Q000276:immunology / Q000382:microbiology; D001419:Bacteria / Q000276:immunology; D020789:Cathepsin C / Q000235:genetics; D055113:Chronic Periodontitis / Q000276:immunology / Q000382:microbiology; D017742:Dental Care for Chronically Ill; D005808:Genes, Recessive / Q000235:genetics; D006801:Humans; D007962:Leukocytes / Q000276:immunology; D010214:Papillon-Lefevre Disease / Q000175:diagnosis* / Q000235:genetics; D017354:Point Mutation / Q000235:genetics
D020789:Cathepsin C