ABSTRACT

Papillon–Lefèvre syndrome is a form of a palmoplantar keratoderma of autosomal recessive inheritance characterized by diffuse palmoplantar keratoderma and premature destruction of the periodontium of the deciduous and permanent teeth. The prevalence is estimated to be 1 per 4 millions people. A 12-year-old male patient with gradual thickening of palms and soles starting a few months after birth had psoriasiform lesions over the knees and elbows. He was diagnosed as Papillon Lefevre syndrome with associated features of premature tooth loss and periodontitis.