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Journal of Indian Society of Pedodontics and Preventive Dentistry Official publication of Indian Society of Pedodontics and Preventive Dentistry
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Year : 2019  |  Volume : 37  |  Issue : 3  |  Page : 303-307

Cu-sil dentures: A novel approach of Papillon–Lefèvre syndrome management

1 Department of Pedodontics and Preventive Dentistry, Christian Dental College, Ludhiana, Punjab, India
2 Department of Dentistry, Rajiv Gandhi Institute of Medical Sciences, Adilabad, Telangana, India

Date of Web Publication30-Sep-2019

Correspondence Address:
Dr. Shreya Tyagi
Department of Pedodontics and Preventive Dentistry, Christian Dental College, Brown Road, Ludhiana - 141 008, Punjab
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/JISPPD.JISPPD_256_18

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Papillon–Lefèvre syndrome (PLS) is a rare genetic disorder characterized by palmoplantar keratosis and premature loss of primary and permanent dentition. Its onset can be as early as 1–4 years of age. The genetic disorder is mutation in the cathepsin C gene. Hereby, we discuss the fabrication of Cu-sil dentures for the prosthetic rehabilitation of a 14-year-old girl with PLS. The case report describes the procedure and associated relevant information regarding the management protocols.

Keywords: Cu-sil dentures, palmoplantar keratosis, Papillon–Lefèvre syndrome

How to cite this article:
Tyagi S, Thomas AM, Balla V, Kundra R. Cu-sil dentures: A novel approach of Papillon–Lefèvre syndrome management. J Indian Soc Pedod Prev Dent 2019;37:303-7

How to cite this URL:
Tyagi S, Thomas AM, Balla V, Kundra R. Cu-sil dentures: A novel approach of Papillon–Lefèvre syndrome management. J Indian Soc Pedod Prev Dent [serial online] 2019 [cited 2022 Aug 17];37:303-7. Available from: http://www.jisppd.com/text.asp?2019/37/3/303/268177

   Introduction Top

Papillon–Lefèvre syndrome (PLS) is an autosomal recessive disorder characterized by palmoplantar hyperkeratosis and aggressive periodontitis leading to premature loss of primary and permanent teeth.[1] PLS was first described by two French physicians, Papillon and Lefèvre in 1924.[2] The prevalence is approximately one case in 1–4 million people. The most frequent cause of its expression is consanguineous marriages.[3]

It is a rare genetic disorder caused by loss-of-function mutations in the cathepsin C gene – the encoder for cysteine lysosomal protease, an enzyme highly expressed in the cells of the immune system, lungs, kidneys, and epithelial tissues, thereby deactivating it.[1]

Palmoplantar keratosis usually begins from 1 to 4 years of age, and juvenile periodontitis can be seen as early as 2 years of age.[2] Dural calcification was added as another component of diagnosis by Gorlin et al.[4] By the age of 4 or 5 years, all the primary teeth may have exfoliated. Gingiva becomes normal following the exfoliation of primary teeth. However, this state relapses after the eruption of permanent teeth. Consequently, partial or complete edentulous stage appears by the age of 14–16 years.[5]

In about 20% of patients, infections other than periodontitis can also be seen.[1] Increased incidence of infections led to the hypothesis that an underlying immune disorder is an important primary or secondary etiological factor in predisposing Papillon–Lefèvre patients to periodontitis.[3]

There is increased percentage of Gram-negative microorganisms in these patients. Actinobacillus actinomycetemcomitans is a common pathogenic organism responsible for periodontal destruction.[3] Organisms such as Porphyromonas gingivalis, Prevotella intermedia, Prevotella loescheii, Bacteroides gracilis, and Fusobacterium nucleatum are also involved in periodontitis.[6]

Many treatment options have been described by the authors for the prosthetic rehabilitation of PLS patients, but Cu-sil dentures have been fabricated for these patients for the first time. It is a transitional denture with holes to accommodate the existing natural teeth. A resilient liner is used to fill the gap between the denture base and the tooth so that it seals and does not allow food and fluids to enter below the denture base and also gives cushioning and splinting effect.[7] A Cu-sil denture provides a good retention and stability. In accordance with future needs, relining is possible.[8] The case report is necessary to bring to the notice that for PLS patients in whom the remaining alveolar bone does not allow the placement of dental implant and the conventional complete dentures are not retentive owing to resorbed ridges, Cu-sil dentures can serve as a better treatment option. The remaining teeth have been utilized for the retention of the dentures for the first time in PLS patients. Till date, no case report has mentioned about the Cu-sil denture as one of the treatment modalities in these patients.

   Case Report Top

A 14-year-old girl reported to the Department of Pedodontics with a chief complaint of loss of multiple teeth and difficulty in chewing food. The family history revealed consanguineous marriage of the parents. The patient was lean and thin. She had a senile facial appearance due to multiple exfoliated teeth. There was slight dystrophy of nails, but the hair was normal. Well-demarcated white keratotic lesions were present on the dorsal surface of hands [Figure 1] and feet [Figure 2]. On the plantar surface of feet, fissuring was present [Figure 3]. The skin was dry and rough on palpation. Only five teeth were present in the oral cavity, i.e., 17, 27, 37, 43, and 47 [Figure 4]. There was Grade 3 mobility in 43, so it was extracted. Orthopantomogram revealed severe alveolar bone resorption giving the teeth a “floating in air” appearance [Figure 5]. Intracranial calcifications were absent on lateral cephalogram [Figure 6].
Figure 1: Well-demarcated keratotic lesions on the dorsal surface on hands

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Figure 2: Lesions on the dorsal surface on feet

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Figure 3: Fissuring on the plantar surface of feet

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Figure 4: Preoperative intraoral view

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Figure 5: Orthopantomogram showing “floating in air appearance of teeth” and atrophied jaws

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Figure 6: Lateral cephalogram showing no intracranial calcification

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For the fabrication of Cu-sil dentures, preliminary impressions were made with alginate (Zelgan, Dentsply) [Figure 7], and final impressions were made with putty and light body polyvinyl siloxane elastomeric impression material (Dentsply). Casts were poured with Type III dental stone (Gypstone, Prevest Denpro Ltd.,) [Figure 8]. Occlusal rims were prepared on temporary denture bases which were fabricated using autopolymerizing acrylic resins (DPI RR Cold Cure). After the bite registration was recorded [Figure 9], the setup was mounted on a three-point articulator. Teeth arrangement was done followed by wax trial. The wax-up of the dentures was done leaving holes corresponding to the remaining natural teeth 17, 27, 37, and 47. The dentures were cured with heat-cure acrylic resin (DPI Heat Cure, DPI) [Figure 10]. Finishing and polishing of the dentures were done. The space in maxillary and mandibular partial dentures around the remaining teeth was widened to give a clearance of 4–5 mm around the teeth. Acrylic-based soft liner (Coe-Soft, GC Soft Liner) was applied to denture and placed into oral cavity to occupy space between denture and natural teeth [Figure 11]. The dentures were inserted in patient's mouth and held in position. After setting of the material, dentures were removed. The excess liner material was trimmed, and dentures were finally inserted in the patient's mouth. Occlusion was checked, and postinsertion instructions were given [Figure 12].
Figure 7: Preliminary diagnostic impression

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Figure 8: Maxillary and mandibular master casts

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Figure 9: Bite registration

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Figure 10: Cu-sil dentures with holes

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Figure 11: Resilient denture liner

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Figure 12: Postoperative intraoral view

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   Discussion Top

For the treatment of PLS patients, a multidisciplinary approach involving a team of dermatologist, pediatrician, and dental team is required for the overall care. Retinoids including etretinate, acitretin, and isotretinoin are beneficial in the treatment of both hyperkeratosis and periodontitis. Different treatment modalities such as conventional periodontal treatment in the form of scaling and root planning; oral hygiene instructions, 0.2% chlorhexidine gluconate mouth rinses; and a course of antibiotic therapy have been suggested to control the active periodontitis.[9] For the successful management of PLS cases, following guidelines have been proposed: it includes extraction of the deciduous dentition and construction of complete dentures 3 months after the removal of primary teeth. It also includes a prophylactic dose of antibiotic for 10 days immediately after the denture insertion, and the denture bases are adjusted during the emergence of the permanent dentition followed by another dose of antibiotic.[10]

For the rehabilitation of edentulous PLS patient, conventional complete dentures, implant-supported complete dentures, overdentures, or a combination of these can be utilized.[9] As proposed by Ullbro et al., Woo et al., and Etoz et al., the use of implants could considerably enhance future therapeutic options. According to Senel et al., the treatment of these patients is complicated because the patients become edentulous at a young age, and alveolar ridges are severely atrophic because of early-onset periodontitis.[11]

In the present case, the patient underwent treatment for the cutaneous lesions but was not relieved and thus discontinued the treatment. For the prosthetic rehabilitation, the treatment plan was planned by the multidisciplinary team. The remaining alveolar bone was not sufficient enough for the implant, so removable prosthesis was chosen as the treatment option. The fabrication of Cu-sil denture was planned, and the remaining teeth 17, 27, 37, and 47 were retained to record the centric relation and vertical dimension. The Cu-sil dentures thus fabricated maintained the proprioception by remaining natural teeth and provided the psychological benefit of avoiding extraction. No antibiotic was prescribed to the patient during the treatment, but the patient was instructed to maintain strict oral hygiene regimen. The patient was kept on regular follow-up visits.

The limitation of Cu-sil dentures is the short functional duration of soft liner. It can deteriorate over a period of time which includes changes in the physical properties (hardening, cracking, or tearing), and thus, the prosthesis needs to be monitored at regular follow-up appointments.[12]

   Conclusion Top

Prosthodontic rehabilitation of PLS patients can improve their oral functioning, appearance, self-confidence, and minimize the onset of emotional and psychological problems. In conclusion, the fabrication of Cu-sil denture was preferred to provide the psychological benefit of preserving the remaining teeth. Successful periodontal management of PLS patients is challenging, and it is imperative to maintain the oral hygiene status to achieve the long-term success of the treatment.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Ullbro C, Twetman S. Review paper: Dental treatment for patients with Papillon-Lefèvre syndrome (PLS). Eur Arch Paediatr Dent 2007;8:4-11.  Back to cited text no. 1
Rai R, Kumar A, Deshpande V. Papillon-Lefèvre syndrome – The prosthodontic management. J Pierre Fauchard Acad 2014;28:23-7.  Back to cited text no. 2
Hart TC, Shapira L. Papillon-lefèvre syndrome. Periodontol 2000 1994;6:88-100.  Back to cited text no. 3
Gorlin RJ, Sedano H, Anderson VE. The syndrome of palmar-plantar hyperkeratosis and premature periodontal destruction of the teeth. A clinical and genetic analysis of the Papillon-Lef'evre syndrome. J Pediatr 1964;65:895-908.  Back to cited text no. 4
Karayilmaz H, Gungor O, Yalcin H, Hatipoǧlu M. Oro-dental characteristics of three siblings with Papillon–Lefevre syndrome. Niger J Clin Pract 2017;20:256.  Back to cited text no. 5
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Robertson KL, Drucker DB, James J, Blinkhorn AS, Hamlet S, Bird PS, et al. A microbsiological study of Papillon-Lefévre Syndrome in two patients. J Clin Pathol 2001;54:371-6.  Back to cited text no. 6
Hiremath S, Jairaj A. Cu-sil denture – A space maintainer for function in paediatric patients. J Clin Diagn Res 2017;11:ZD09-11.  Back to cited text no. 7
Jain AR. Cu-sil denture for patients with few remaining teeth-A case report. J Adv Pharm Technol Res 2017;7:332-4.  Back to cited text no. 8
Sreeramulu B, Shyam ND, Ajay P, Suman P. Papillon-Lefèvre syndrome: Clinical presentation and management options. Clin Cosmet Investig Dent 2015;7:75-81.  Back to cited text no. 9
Baer PN, McDonald RE. Suggested mode of periodontal therapy for patients with Papillon-Lafevre syndrome. Periodontal Case Rep 1981;3:10.  Back to cited text no. 10
Senel FC, Altintas NY, Bagis B, Cankaya M, Pampu AA, Satiroglu I, et al. A 3-year follow-up of the rehabilitation of Papillon-Lefèvre syndrome by dental implants. J Oral Maxillofac Surg 2012;70:163-7.  Back to cited text no. 11
Revathi K, Reddy SS, Reddy KK. Enhancing retention of partial dentures using elastomeric retention rings. Indian J Dent Res 2015;26:328-32.  Back to cited text no. 12
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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], [Figure 12]

This article has been cited by
1 Papillon–Lefèvre syndrome (PLS) with novel compound heterozygous mutation in the exclusion and Peptidase C1A domains of Cathepsin C gene
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