Management of Papillon Lefevre Syndrome

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 of teeth, and finally spontaneous exfoliation without showing definite signs of root resoption by about age f ive. After an edentulous interval, the same process begins shortly after the second dentition. PLS (Papillon Lefevre Syndrome) is transmitted as an autosomal recessive trait. In addition, some patients manifest excessive sweating, the growth of f ine body hair and the development of dirty colored skin on the affected parts. Genetic analysis of several affected families suggests that the disorder may result from mutations of a gene that regulates production of an enzyme known as cathespin C. A case of PLS is presented along with management of this condition. The result was satisfactory with limited resources. The patient was satisf ied with improved esthetic , speech and chewing abilit ies. Conclusion:Any young patient who exhibits palmar hyperkeratosis should be examined carefully for periodontal breakdown