A RARE CASE OF PAPILLON-LEFEVRE SYNDROME: A CASE REPORT

Papillon Lefevre syndrome is an extremely rare autosomal recessive disorder characterized by diffuse palmoplantar hyperkeratosis, rapidly progressive and devastating periodonitis, pyodermas. The etiopathogenesis of the multifactorial, with genetic immunological factors playing a major role. Consanguinity contributing factor. Genetic mutations gene 11q14- q21 encoding for cathepsin-c, lysosomal protease that activates enzymes involved in variety inflammatory immune processes. Management directed towards halting periodontal destruction using conventional treatment, systemic antibiotics, oral hygiene instructions, antiseptic mouth rinses. Palmoplantar hyperkeratosis usually treated topical application emollients, keratolytic agents, steriods retinoids.