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Papillon-Lefevre Syndrome is a rare autosomal recessive disorder of Palmoplantar Keratinisation (PPK) in which there is a unique association of the palmoplantar hyperkeratosis and premature loss of both deciduous and permanent teeth. In most cases, the PPK is noted within the first 3 years of life. Usually the patient becomes edentulous by 20 years of age.
A case of 13 years old female with AS sydrome is discussed in this article.