Year : 2005 | Volume
: 23 | Issue : 2 | Page : 96--98
Prosthodontic rehabilitation in Papillon-Lefevre syndrome: A case report
Veena Jain, R Gupta, H Parkash
Department of Prosthodontics, Centre for Dental Education and Research, AIIMS, New Delhi, India
Department of Prosthodontics, Centre for Dental Education and Research, AIIMS, New Delhi
Papillon-Lefevre syndrome is a rare recessive genetic disorder. The clinical manifestations include palmar-plantar hyperkeratosis with precocious rapidly progressive periodontal disease that results in premature exfoliation of primary and permanent dentitions. Patients are often edentulous at an early age. Here is the report of prosthodontic rehabilitation of a case of Papillon-Lefevre syndrome in a 6-year-old child.
|How to cite this article:|
Jain V, Gupta R, Parkash H. Prosthodontic rehabilitation in Papillon-Lefevre syndrome: A case report.J Indian Soc Pedod Prev Dent 2005;23:96-98
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Jain V, Gupta R, Parkash H. Prosthodontic rehabilitation in Papillon-Lefevre syndrome: A case report. J Indian Soc Pedod Prev Dent [serial online] 2005 [cited 2021 Jul 28 ];23:96-98
Available from: https://www.jisppd.com/text.asp?2005/23/2/96/16451
Papillon-Lefevre syndrome is a rare syndrome of autosomal recessive inheritance also known as Palmoplantar Keratoderma with Periodontosis, Palmar-plantar Hyperkeratosis and Concomitant Periodontal Destruction, Keratoris Palmoplantaris with Periodontopathia, Hyperkeratosis Palmoplantaris with Periodontosis. Palmar-plantar hyperkeratosis and severe periodontal destruction involving both the primary and permanent dentition characterize it.  Gorlin et al. have added the third component of dural calcification, which was observed in significant number of cases reviewed by them. However, this calcification is a variable finding and their significance is unknown. 
The clinical features usually become apparent from approximately 1-5 years of age. Genetic predisposition with greater frequency of occurrence in consanguineous offsprings has been reported.  There is no predominance for sex, as males and females are equally affected.  The frequency of disorder is estimated approximately 1-4 per million persons in the general population and the carrier frequency appears to be 2-4 per 1000 population. ,
A case is reported of a 6-year-old male child with only mandibular first molars present when first seen. History revealed loss of all primary teeth at the age of 4 years. Child was first sibling out of two children. No other family member or relatives suffered from the similar condition. No history of consanguinity of his parents was present. The pregnancy and delivery was uneventful. Patient's mother noticed reddening of palms and soles at 6 months of age [Figure 1]. Gradually the skin thickened and became rough and scaly. Lesions were well demarcated from adjacent skin. Patient's overall physical and mental development was normal with normal development of hair, nails and sebaceous glands. Intra-oral examination revealed completely edentulous upper arch and partially edentulous lower arch with only mandibular first molars present [Figure 2]. Premature loss of teeth and loss of alveolar bone had resulted in decreased facial height and a senile appearance. Radiographic examination revealed generalized loss of alveolar bone [Figure 3]. X-ray skull and CT scan was within normal limits. Considering the present state of the child, rehabilitation with maxillary complete denture and mandibular partial denture was planned. Necessary preventive measures, i.e. scaling and oral tetracycline therapy for 10 days were accomplished to preserve the mandibular first molars and prevent the further resorption of alveolar bone.
Usual steps of denture fabrication were followed. Putty and light body polyvinyl siloxane impression material was used for making the final impression [Figure 4]. Smaller size and more translucent teeth were selected. Spacing was given in between the teeth resembling the deciduous dentition [Figure 5] and [Figure 6]. Teeth were contoured to simulate the deciduous dentition. Usual recall schedule was followed up and necessary adjustments were done. Parents were informed regarding oral rehabilitation with implants at later stage.
The dental surgeons are the first to diagnose Papillon-Lefevre syndrome because of the severe periodontitis affecting the patients. Oral manifestation of Papillon-Lefevre syndrome appears almost simultaneously with the onset of palmar-plantar hyperkeratosis.  Once the primary teeth have erupted, rapidly progressing periodontitis occurs. It is manifested by markedly reddened, swollen gingiva and fetor-ex-oris with pus exudation from deep periodontal pockets. Premature rapid destruction of alveolar bone around teeth causes premature shedding of teeth resulting in edentulism. ,, 'Gingiva reverts back to normal health after exfoliation of teeth. The same cycle is repeated with the eruption of permanent teeth. Without treatment, most of the secondary (permanent) teeth may also be lost by approximately at 17 years of age. ,
The pathogenesis of this syndrome remains relatively obscure. Immunologic, genetic and microbiologic factors have been proposed. Various etiopathogenic factors seen associated with the syndrome are presence of virulent gram-negative anaerobic pathogens at the site of lesion such as bacterocides gingivalis, capnocytophaga, spirochetes and actinobacillus actinomycetem comitans. Second there is impairment of neutrophil chemotaxis and phagocytosis; third defective immune-mediated mechanism with suppression of helper to suppressor T-cell ratio, deficient monocytic function and elevation of IgG (serum). ,, Recent research has shown that a disorder in the activity of enzyme Cathepsin C, a lysosomal protease is a consistent finding in patients with PLS.  The PLS debilitates individuals socially, psychologically and physically. Thus oral rehabilitation in such patients is a must. Prosthetic replacement in PLS is age specific, specialty job involving initial replacement with complete or partial dentures with future consideration for implant-supported prosthesis.' Prosthodontic rehabilitation provides a psychological boost up to the patient as well as to the parents by restoring not only the esthetic appearance but also the function.
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