Papillon Lefevre syndrome is an extremely rare autosomal recessive disorder characterized by diffuse palmoplantar hyperkeratosis, rapidly progressive and devastating periodonitis, pyodermas. The etiopathogenesis of the multifactorial, with genetic immunological factors playing a major role. Consanguinity contributing factor. Genetic mutations gene 11q14- q21 encoding for cathepsin-c, lysosomal ...

⁎ Corresponding author. Tel.: +44 01334 463044. E-mail address: [email protected] (C.E. Lefevre 1090-5138/$ – see front matter © 2013 Elsevier Inc. Al http://dx.doi.org/10.1016/j.evolhumbehav.2013.03.005 Please cite this article as: Lefevre, C.E., et a Human Behavior (2013), http://dx.doi.org/1 Article history: Initial receipt 1 August 2012 Final revision received 25 March 2013 Available o...

papillon-Lefèvre Syndrome (PLS) Is A Very Rare Autosomal Recessive Disorder Characterized By Palmoplantar Hyperkeratosis And Severe Early Onset Of Destructive Periodontitis Leading To Premature Loss Of Both Primary And Permanent Dentitions. Here We Are Presenting Case Report Of Siblings Who Presented With Palmoplantar Hyperkeratosis And Aggressive Periodontitis.