PAPILLON-LEFEVRE-SYNDROME - AN ACROECTODE RMAL DYSPLASIA WITH PREMATURE LOSS OF THE TEETH, WHICH CAN BE TREATED BY RETINOIDS

Papillon-Lefevre syndrome is a rare autosomal recessive disorder chara cterized by transgredient palmoplantar keratosis and premature loss of the deciduous and permanent teeth. We report on two siblings, who wer e treated with 0.5 mg acitretin/kg body weight/day and concomitant per iodontal therapy since November 1992. The dermatological and dental fi ndings transiently improved, but a constant success with preservation of die permanent teeth can probably only be achieved by longterm treat ment. In regard of the bone toxicity of the retinoids it is necessary to carefully weigh benefits against risks of treatment.