Papillon-Lefevre syndrome: a case report of two siblings and review of the literature

ABSTRACT

Papillon-Lefevre syndrome is a rare autosomal recessive genodermatosis which is characterised by periodontitis, palmoplantar keratoderma and predisposition to pyogenic infections and occurs due to cathepsin C gene mutation [located on chromosome11].The loss of primary teeth usually occurs by the age of 4 years and secondary teeth by second decade. The disorder is associated with significant cosmetic and functional disability