17. Papillon-Lefevre syndrome

Papillon-Lefevre syndrome is a rare autosomal recessive disorder and is characterized by the diffuse palmoplantar hyperkeratosis with rapid destruction of the periodontal support of both the primary and secondary dentition. Patient is often completely or partially edentulous at the stage of primary (4-5 yrs) and permanent dentition (13-15 yrs). Though, exact etiopathogenesis is unknown; three major factors have been identified as accountable for its initiation and progression. These are hereditary, immunological and microbial factors. Total rehabilitation in such cases is often difficult for health care professionals. However, correct diagnosis, perfect treatment planning and appropriate prosthodontic rehabilitation of such unfortunate individuals can improve their oral functioning, esthetics and overall social life. Here is a clinical report of such a rare condition with special focus on its diagnosis, characterization and rehabilitation by removable prosthodontics.