A Unique Case Report of Papillon Lefevere Syndrome in a 13-Year Old Child

Abstract

Papillon-Lefever syndrome (PLS) or palmoplantar keratoderma with periodontitis (Rapini, Ronald P; 2007) is an autosomal recessive genetic disorder caused by deficiency in cathepsin C [1,2]. Two French physician Papillon & LeFevre were the first one to characterize this disease in a brother & sister suffering from palmoplantar hyperkeratosis associated with early onset of periodontitis & premature loss of primary & permanent dentition
(P-L 1924) [1,3]. The third component that is Dural calcification in this disease was added by Gorlin et al 1964 (Gorlin 1964) [1]. Other symptoms include hyperhidrosis, arachnodactyly, increased susceptibility infection & mental retardation (Hattab FN 1995, sollecito TP 2005) [1-6]