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Concept information

Preferred term

Papillon-Lefevre Disease  

Type

  • mesh:Descriptor

Definition

  • Rare, autosomal recessive disorder occurring between the first and fifth years of life. It is characterized by palmoplantar keratoderma with periodontitis followed by the premature shedding of both deciduous and permanent teeth. Mutations in the gene for CATHEPSIN C have been associated with this disease.

Entry terms

  • Keratosis Palmoplantaris with Periodontopathia
  • Keratosis Palmoplantar Periodontopathy
  • Papillon Lefevre Disease
  • Papillon-Lefevre Syndrome

In other languages

URI

http://data.loterre.fr/ark:/67375/JVR-R4NMZL48-2

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