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Introduction Papillon Lefevre syndrome (PLS) is a rare condition with an estimated incidence of 1-4 per million.  Approximately 300 cases have been reported worldwide and none have been reported from South Africa (Dhanrajani, 2009). This autosomal recessive genetic disorder, which was first described by Papillon and Lefevre in 1924, is associated with the loss-of-functional mutation [...]