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Multidisciplinary management of Papillon-Lefevre syndrome as a result of consanguineous marriage
  1. Narayane Ramkumar1 and
  2. Hanumanth Sankar2
  1. 1Periodontology, Indira Gandhi Institute of Dental Sciences, Sri Balaji Vidyapeeth University, Puducherry, India
  2. 2Orthodontics and Dentofacial Orthopedics, Indira Gandhi Institute of Dental Sciences, Sri Balaji Vidyapeeth University, Puducherry, India
  1. Correspondence to Dr Narayane Ramkumar; narayane{at}igids.ac.in

Abstract

Papillon-Lefevre syndrome (PLS) is a rare autosomal recessive syndrome, and consanguinity has been reported in 20%–40% of cases. It is characterised by palmoplantar hyperkeratosis associated with severe early-onset periodontitis and premature loss of primary and permanent teeth. This report describes a case of PLS in a female patient with consanguineously married parents. The patient reported mobile upper front teeth. Clinical examination revealed presence of marked palmoplantar hyperkeratosis.

Symmetric, well-demarcated, yellowish, keratotic and confluent plaques were seen on the skin of her palms and soles. Intraoral periodontal examination revealed erythematous gingiva with generalised periodontal pockets. Generalised mobility of teeth was present with clinically missing lower anterior teeth. Based on clinical and radiographic feature and the patient’s medical, dental and family history, a diagnosis of PLS was made.

  • Dentistry and oral medicine
  • Dermatology

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Footnotes

  • Contributors NR, the corresponding author, treated the patient under her observation. The detailed case history and treatment planning were done accordingly, and the case was treated with multidisciplinary management. The coauthor, HS, helped the corresponding author with all the necessary documentation required for the case.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.