Management of papillon lefevre syndrome

ABSTRACT

It is a rare, autosomal recessive disorder occurring between the first and fifth years of life and is characterized by palmoplantar keratoderma and periodontitis followed 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 five. 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 fine 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 satisfied with improved esthetic, speech and chewing abilities