Papillon-Lefèvre syndrome: A case report of two siblings and review of the literature
Keywords:
Papillon-Lefèvre syndrome, periodontitis, palmoplantar keratodermaAbstract
Papillon-Lefèvre 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.References
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