|Year : 2012 | Volume
| Issue : 3 | Page : 182-186
Combined orthodontic and periodontic therapy in a patient with Papillon-Lefèvre syndrome
Padmalatha Challa1, Chandra S Gandikota2, Suhas Tarlapally3, Naveen Rayapudi1
1 Department of Orthodontics, Mamata Dental College, Giri Prasad Nagar, Khammam, Hyderabad, India
2 Department of Orthodontics, Panineeya Dental College, Dilsukhagar, Hyderabad, India
3 Department of Orthodontics, Mamata Dental College, Khammam, Private Practice, Hyderabad, India
|Date of Web Publication||15-Oct-2012|
Department of Orthodontics, Mamata Dental College, Khammam, Andhra Pradesh
Source of Support: None, Conflict of Interest: None
Papillon-Lefèvre 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.
Keywords: Adjunctive orthodontic treatment, aggressive periodontitis, Papillon-Lefèvre syndrome
|How to cite this article:|
Challa P, Gandikota CS, Tarlapally S, Rayapudi N. Combined orthodontic and periodontic therapy in a patient with Papillon-Lefèvre syndrome. J NTR Univ Health Sci 2012;1:182-6
|How to cite this URL:|
Challa P, Gandikota CS, Tarlapally S, Rayapudi N. Combined orthodontic and periodontic therapy in a patient with Papillon-Lefèvre syndrome. J NTR Univ Health Sci [serial online] 2012 [cited 2021 Sep 23];1:182-6. Available from: https://www.jdrntruhs.org/text.asp?2012/1/3/182/102450
| Introduction|| |
Papillon-Lefèvre syndrome (PLS) is an autosomal recessive disorder. The two cardinal diagnostic features of the syndrome are palmoplantar keratosis and an early-onset form of aggressive periodontitis with premature loss of primary and permanent dentitions. Males and females are affected equally, and no racial predominance seems to exist.  The rate of parental consanguinity is far greater than that for the general population. ,
A close relationship between the presence of Aggregatibactor actinomycetemcomitans (Aa) and periodontal destruction in PLS patients has been suggested by microbiologic monitoring and clinical examination following various treatment modalities.  An improvement in clinical symptoms has been demonstrated with simultaneous elimination of Aa from the gingival crevice and mouth rinse. 
An extensive review of the literature showed that most studies of PLS focused on the genetic basis , and the periodontal management of the syndrome. [6-8] The gene responsible for PLS was mapped to chromosome 11q14 q21.  The common features of PLS include palmoplantar hyperkeratosis associated with severe, early onset periodontitis and premature loss of primary and permanent teeth. Common cutaneous changes include well-demarcated erythematous hyperkeratotic lesions on the palms, soles, dorsum of the hands, and the interphalangeal joints. Periodontal literature shows that it is possible to successfully maintain a healthy periodontium in these patients with early treatment and preventive measures. , This includes oral hygiene instructions, use of mouth rinse, frequent debridement, multiple antibiotic regimens, periodontal surgery, and extraction of hopeless teeth. , The present case report is an attempt for esthetic and functional rehabilitation of a young patient diagnosed as PLS with aggressive periodontitis, by an interdisciplinary approach involving adjunctive orthodontics, periodontal therapy, and maintenance.
| Case Report|| |
A female patient aged 24 years was referred to Department of Orthodontics, Mamata dental college, Khammam. The patient was referred by department of dermatology, Mamata medical college, wherein she was diagnosed with PLS. On physical examination, the patient exhibited hyperkeratosis of palms and soles as well as knees and elbows. The patient was under oral retinoids for the treatment of hyperkeratosis. She presented with a past dental history of flap surgery 8 months ago. Periodontal therapy included full thickness mucoperiosteal flap elevation in all four quadrants thorough scaling and root planning.
Extra oral examination showed convex profile, incompetent lips, lower lip trap, acute nasolabial angle [Figure 1] with interlabial gap of 7 mm and incisal show of 5 mm [Figure 2]. Intraoral examination revealed missing 31, 41, and 42, [Figure 3] which has resulted in habitual forward posturing of the tongue but without any significant tongue thrusting habit. There was pathological migration of 11 and 21. Angle's Class I malocclusion with proclined upper and lower anterior teeth [Figure 4] and [Figure 5]. Generalized loss of interdental papilla and gingival recession were present. Hypermobility of the teeth with furcation involvement in relation to 36, 37, 46, and 47. A panoramic radiograph was obtained which revealed generalized interdental and vertical bone loss in relation to 36,37,38,46, and 47 [Figure 6].
| Treatment|| |
Orthodontic treatment was carried out to improve the esthetics and function. Bonding of the upper arch was carried out with MBT 0.022″ slot bracket system (Ormco corp). In view of periodontal status of the patient 0.013 Copper Nickel Titanium wire was used to deliver light forces, followed by 17 × 25 mil HANT wire for alignment and bite opening. Later leveling and aligning was achieved in lower arch using the same sequence. Anterior spaces were closed in the upper arch with rectangular 19 × 25 mil stainless steel (SS) wires with active tiebacks [Figure 7]. The patient was advised to perform tongue exercises as a part of conscious remodeling of tongue position. Angle's Class I molar and Class I canine relation were maintained throughout the treatment [Figure 8] and [Figure 9]. In the lower arch the edentulous space was treated by removable prosthesis keeping in mind the financial constraints of the patient. The lower lip trap was relieved as a result of reduced overjet [Figure 10]. The incisal visibility was reduced posttreatment [Figure 11]. During the whole period of orthodontic treatment the patient had regular evaluation for periodontal status to evaluate bleeding on probing, attachment loss, pocket depth. The total treatment duration was 16 months.
| Discussion|| |
Patients with PLS usually present with a range of periodontal problems starting from primary dentition stage. This is mainly due to lack of functional cathepsin C, which results in a reduced host response against plaque bacteria. Cathepsin C is a cysteine-lysosomal protease coded by the CTSC gene.  The exact cause of periodontal disease in PLS has not been found but it has been attributed to neutrophil defects which causes decreased neutrophil chemotaxis and phagocytosis, Bacterial infection with Aa and natural killer cell defect Cytotoxicity.  Early treatment and compliance with the prevention program are the major determinants for preserving permanent teeth in young PLS patients.
In the present case, the patient had already lost her lower anterior teeth and there was pathological migration of anterior teeth along with generalized bone loss and mobility of the entire dentition. She underwent flap surgery 8 months before she presented to orthodontic treatment and was under periodontal maintenance thereafter. Keeping her periodontal status in mind, orthodontic treatment was started with Cu-Niti wires. These wires produce more constant force over long activation spans, which is usually required in cases with weak periodontal status.
The case was followed up for a period of 15 months postdebond. To assess the stability of the treatment, reduced bleeding on probing, sulcus depth, probing pocket depth, and gain in clinical attachment were assessed at every 2 month postdebond follow-up similar to the protocol followed by Closs et al. 
| Conclusion|| |
The adjunctive orthodontic treatment used in the present case report improved the esthetics by reducing the incisal visibility; function by relieving lip trap; and stability by decreasing overjet. Thus the goals of the orthodontic treatment were achieved and were equally appreciated by the patient. Considering the aggressive nature of periodontitis seen in PLS patients, we advocate a comprehensive periodic follow up and supportive therapy for overall successful rehabilitation.
| Acknowledgements|| |
The authors thank Dr Yudhistar, Dr Arpita, Dr Sarita for their valuable suggestions.
| References|| |
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11]