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Papillon-Lefevre Disease

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.

Year introduced: 1991(1975)

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Tree Number(s): C16.320.850.475.600, C17.800.428.435.600, C17.800.827.475.600

MeSH Unique ID: D010214

Entry Terms:

  • Papillon Lefevre Disease
  • Keratosis Palmoplantaris with Periodontopathia
  • Papillon-Lefevre Syndrome
  • Papillon Lefevre Syndrome
  • Keratosis Palmoplantar Periodontopathy
  • Keratosis Palmoplantar Periodontopathies
  • Palmoplantar Periodontopathies, Keratosis
  • Haim-Monk Syndrome
  • Haim Monk Syndrome

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