Combined orthodontic and periodontic therapy in a patient with Papillon-Lefèvre syndrome

Papillon-Lefevre Syndrome is a rare autosomal recessive disorder with an onset usually by 2-3 years of age. The disease is mainly characterized by severe early onset of periodontitis, and hyperkeratosis characteristically involving the palms and soles and sometimes the knees, elbows, knuckles, and back. Periodontitis affects both the primary and secondary dentitions, resulting in premature tooth loss of both dentitions. The primary teeth erupt at the expected age and in the normal sequence. The teeth are typically of normal form and structure. Eruption of the primary dentition in to the oral cavity is accompanied by severe gingival inflammation and generalized aggressive periodontitis. Chewing can be painful due to tooth mobility. With loss of the primary dentition, gingival inflammation resolves. After the eruption of secondary teeth, the same cycle of events begins and without treatment most of the secondary teeth may be lost at an early age. Orthodontic treatment in periodontal compromised cases is most challenging, light forces and controlled mechanics are important for adjunctive orthodontic treatment.