J Interdiscip Dentistry
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CASE REPORT
Year : 2012  |  Volume : 2  |  Issue : 2  |  Page : 122-127

Rehabilitation of three siblings with Papillon-Lefevre syndrome


1 Department of Prosthodontics and Geriatric Dentistry, SDM College of Dental Sciences & Hospital, Sattur, Dharwad, India
2 Department of Oral and Maxillofacial Surgery, Sri Sai College of Dental Surgery, Opp. Shiv Sagar, Kothrepally, Vikarabad, India
3 Department of Prosthodontics, SDM College of Dental Sciences & Hospital, Sattur, Dharwad, India
4 Department of Oral and Maxillofacial Surgery, SDM College of Dental Sciences & Hospital, Sattur, Dharwad, India

Correspondence Address:
Gouri V Anehosur
Department of Prosthodontics and Geriatric Dentistry, SDM College of Dental Sciences & Hospital, Sattur, Dharwad
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2229-5194.100606

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Papillion-Lefevre Syndrome is a rare autosomal recessive disorder characterized by severe early onset periodontopathia and palmoplantar hyperkeratosis leading to premature loss of primary and permanent dentition. PLS is caused by mutations in cathepsin C (CTSC) gene. It is transmitted with an estimated frequency of one to four per million individuals. In this case report, we present clinical and other relevant investigations of three siblings with symptoms typical of Papillion-Lefevre Syndrome. The three siblings were born of a second-degree consanguineous marriage, where the parents were first cousins. All the siblings reported with mobility, eventually leading to loss of teeth and masticatory inefficiency. All showed hyperkeratosis of palms and soles, with youngest sibling showing mild to severe form of severe generalized periodontal destruction. The elder two siblings were given dentures. The youngest sibling is undergoing periodontal therapy and has received partial dentures for missing teeth. Clinical Relevance to Interdisciplinary Dentistry
  1. PLS is a rare autosomal disorder which needs to be diagnosed early.
  2. Timely intervention of periodontal therapy helps in decreasing the loss of teeth at an early stage.
  3. This facilitates growth of jaws and eventual successful placement of implants.
  4. Dermatological treatment and psychological counseling are also mandatory
  5. Helps to educate the parents and family members. Needs team approach for successful outcome.


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