Papillon-Lefèvre syndrome. A clinical and therapeutical contribution.

A clinical study of a patient with the Papillon-Lefèvre syndrome is presented. She had identifiable zones where cutaneous involvement was lacking, probably corresponding to areas free from the effects of attrition or trauma. The patient was treated with oral aromatic retinoid Ro 10-9359. An obvious response with marked improvement of the clinical picture was obtained. Combined treatment of the oral cavity with local and systemic medication was carried out. Beneficial effects on involved integuments and on social repercussions are discussed and a new alternative in the management of these patients is proposed.