Papillon-Lefevre syndrome is a rare (1-4 cases per million) autosomal recessive disorder showing predominantly oral and dermatological manifestations in the form of aggressive periodontitis affecting both primary and permanent dentition and palmoplantar hyperkeratosis. Genetic studies have shown that mutations in the major gene locus of chromosome 11q14 with loss of function of cathepsin C gene...

statement of problem: papillon lefevre syndrome (pls) is a rate autosomal recessive disorder, which is characterized by palmar- plantar hyperkeratosis and rapid periodontal destruction of primary and permanent dentitions. purpose: the aim of the present study was to evaluate the peripheral blood neutrophil function including random locomotion, chemotaxis and oxidative mechanism of killing in a ...

Papillon-Lefevre syndrome is a rare autosomal recessive genetic disorder. The clinical manifestations include palmer planter hyperkeratosis with precocious progressive periodontal disease that results in premature exfoliation of primary and permanent dentitions. Patients are often edentulous at an early age. This is a case report of prosthodontic rehabilitation of a 15-year-old girl with Papill...

papillon-lefevre syndrome (pls) is a very rare genetic syndrome, and fewer than 500 cases have been reported in the world. patients exhibit typical cutaneous involvement with hyperkeratosis, especially on the soles and palms, and early shedding of primary teeth. internal organ involvements, such as liver abscesses, have been described in case reports. this  communication  represents  the  first...

Papillon Lefèvre syndrome is a rare disease characterized by skin lesions caused by palmar-plantar hyperkeratosis, and severe periodontal destruction involving both the primary and permanent dentitions. It is transmitted as an autosomal recessive condition and consanguinity of parents is evident in about one-third of cases. Pyogenic liver abscess is an increasingly recognized complication. We r...

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...

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...

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...