DOI: 10.36347/sjams.2013.v01i05.0059

Pages: 589-592

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. The palmar plantar keratoderma typically has its onset between the ages of 1 and 4 years and severe periodontitis starts at the age of 3 or 4 years. Two cases of Papillon-Lefevre syndrome in the same family (siblings) are presented. A 15 year old girl and 13 year old boy having all the characteristic features of syndrome.