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Background: Papillon-Lefevre syndrome is a rare autosomal recessive genetic disorder which is characterized by palmar plantar hyperkeratosis with precocious progressive periodontal disease that results in premature exfoliation of primary and permanent dentitions. Consanguinity of parents is evident in about one third of cases.
Context: Here we report a case of 22 year old male who presented with Papillon-Lefevre syndrome. The etiopathogenesis, investigations, differential diagnosis, complications and management of this syndrome are also discussed here.
Importance: It is a serious condition leading to social stigma as the individual suffering from this disorder often faces an edentulous state at a very early age. Early recognition and treatment especially with oral retinoids have shown promising results to some extent. However stem cell therapy can be expected to be a turning point in the dental treatment of such patients.
Aggressive periodontitis, palmar and plantar hyperkeratosis