SHORT COMMUNICATION Cephalometric findings in patients with Papillon-Lefèvre syndrome Naif A. BinDayel, a Christer Ullbro, b Lokesh Suri, c andEmadAl-Farra d Boston, Mass, and Riyadh, Saudi Arabia Introduction: Literature regarding oral conditions in patients with Papillon-Lefèvre syndrome (PLS) often coversthe periodontal aspects,but no literature was founddescribing specificcraniofacial findings in this group. Theaimof thisretrospective study was to investigate thecephalometric findings of patients withPLS. Methods: Lateral cephalograms of 8 patients withPLS were traced, andhard-andsoft-tissue variables were analyzed. Results: Class III skeletal relationship was evident (ANB angle, 2° ⫾ 3.1°; Witsappraisal, ⫺9.1mm ⫾ 3.7 mm). Other findings include maxillary retrognathia, decreased lower facial height, retroclined mandibular incisors, and upper lip retrusion. Conclusions: Patients affectedwith PLS have a Class III skeletal pattern. Thesefindings can be o f clinical value not onlyfor diagnosis, but also for proper treatment planning. (AmJ OrthodDentofacial Orthop2008;134:138-44) P apillon-Lefèvre syndrome (PLS) is an autosomal recessive disorder. The 2 cardinal diagnostic features of thesyndrome are palmoplantar ker- atosis and anearly-onset form of aggressive periodon- titis. 1 The palms andsoles have a dry,red, and scaly appearance. Otherareas, including cheeks, eyelids, labial commissures, legs,thighs, knees, and elbows, can be affected by the keratosis, although it varies significantly. 1 Ullbro et al 2 studied 47 patients with PLS andfound no significant correlation between the severity of the skin lesions and the level of periodontal infection. Before tooth eruption, thegingival and mu- cosal surfaces appear normal. As the skin lesions appear, the gingiva becomes inflamed and swollen. Rapid periodontal destruction occurs as teeth erupt. In many uncontrolled situations, most of the primary dentition is lost by age 4 or 5 years, followed byloss of the permanent dentition in the early teens. 1 Histologic examination of extracted teeth from 2 affected persons showed areas of root resorption of various depths and extents, signs of spontaneous repair, and areas with healthy cementum. 3 An extensive review of theliterature showed that most studies of PLS focused on thegenetic basis 4-10 and the periodontal management of the syndrome. 11-18 Thegene responsible forPLSwas mapped to chromo- some 11q14-q21. 19 Periodontal literature shows that it is possible tosuccessfully maintain a healthy periodon- tium in these patients with early treatment and preven- tivemeasures. 15,20 Thisincludes oral hygiene instruc- tions, use of mouth rinse, frequent debridement, multiple antibiotic regimens, periodontal surgery, and extraction of hopeless teeth. 11 An isolated case report of PLS presented the combined periodontal-orthodontic management of a patient aged 7 years 9 months. 21 A stable periodontal situation was achieved after 26 months of combined mechanical and antibiotic therapy. Thisinitial therapy was followed by orthodontic treat- ment with fixed appliance without further pronounced periodontal deterioration. Spaces for eruption of the canines and the premolars were created, in addition to the alignment of teeth. In another case report, a lingual holding arch wasplaced on the first molars once they erupted. 11 Until now, no article has described the craniofacial features of patients with PLS. Ourclinical observation is that patients with PLS have the characteristics of Class III skeletal malocclu- sion. Our aim in this study was therefore to establish a cephalometric baseline for skeletal and soft-tissue vari- ables in PLSpatients. MATERIAL AND METHODS Patient records at King Faisal Specialist Hospital and Research Center (KFSH & RC) in Riyadh, Saudi Arabia, were used for this study. There were 47patients a Postgraduate fellow, School of Dental Medicine, TuftsUniversity, Boston, Mass. b Consultant pedodontist, Department of Dentistry, King FaisalSpecialist Hospital and Research Center, Riyadh, Saudi Arabia; Institute for Postgraduate Dental Education, Jönköping, Sweden. c Assistant professor, Department of Orthodontics, School of Dental Medicine, Tufts University, Boston, Mass. d Consultant orthodontist, Department of Dentistry, King FaisalSpecialist Hospital and Research Center, Riyadh, Saudi Arabia. Reprintrequests to: NaifA. BinDayel, PO Box 613,Riyadh 11321, Saudi Arabia; e-mail,bindayel@hotmail.com. Submitted, March 2007; revised and accepted, January 2008. 0889-5406/$34.00 Copyright © 2008 by the American Association of Orthodontists. doi:10.1016/j.ajodo.2008.01.002 138