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...

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...

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 (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-Lefevre syndrome (PLS) is a rare autosomal recessive heterogeneous trait which is characterized by erythematous palmoplantar hyperkeratosis, early-onset periodontitis, and associated calcification of dura mater. The etiology of PLS is multifactorial with genetic, immunological, and microbial factors playing a role in etiopathogenesis. Recently identified genetic defect in PLS has been ...

Papillon-Lefèvre syndrome is a rare autosomal recessive disorder caused by cathepsin C gene mutation leading to the deficiency of cathepsin C enzymatic activity. The disease is characterized by palmoplantar hyperkeratosis, periodontopathy and precocious loss of dentition, and increased susceptibility to infections. Pyogenic liver abscess is an increasingly recognized complication. Three cases o...

Five cases of Papillon-Lefevre syndrome occurring in 2 families were seen to have variable clinical features. Two of these cases treated with etretinate showed a good response.

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...