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 m...

background: papillon-lefevre syndrome (pls) is a rare autosomal recessive disorder characterized by palmoplantar hyperkeratosis and early development of aggressive pe-riodontitis. although cathepsin c (ctsc) gene mutations have been established in about 70-80% of pls patients, it is assumed that the patients may have dysfunctioning of immune defense mechanisms. objective: to assess the associat...

It is a rare, autosomal recessive disorder occurring between the f irst and fi f th years of l i fe and is characterized by palmoplantar keratoderma and periodontitis fol lowed by the premature shedding of both primary and permanent teeth. The teeth are affected in the order of their eruption, exhibiting inflammation of the periodontal tissue, bleeding of the gums, pocket formation, loosening o...

Papillon-Lefevre syndrome is a very rare syndrome of autosomal recessive inheritance characterized by palmar-plantar hyperkeratosis and early onset of a severe destructive periodontitis, leading to premature loss of both primary and permanent dentitions. Various etiopathogenic factors are associated with the syndrome but a recent report has suggested that the condition is linked to mutations of...

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 wit...

background: papillon-lefevre syndrome (pls) is a rare autosomal recessive disorder characterized by diffused palmoplantar keratoderma and severe periodontitis. increased susceptibility to infections due to impairment of the immune system is considered to be involved in pathoetiology of this disease. objective: according to the crucial function of hla molecules in immune responses and associatio...

Premature tooth loss is a disastrous situation that affects deciduous or permanent teeth era with different causes. It may be attributed to some disorders like Papillon-Lefevre syndrome or coffin-lowry syndrome but because of ambiguous nature, precise diagnosis is not easily possible. Moreover, it has very low incidence and defines by few and limited case series, with vague characters to some e...