Papillon-Lefevre Disease

**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.

**Tree numbers:** - C16.320.850.475.600 - C17.800.428.435.600 - C17.800.827.475.600

**Synonyms:** - Papillon-Lefevre Syndrome - Keratosis Palmoplantar Periodontopathy - Keratosis Palmoplantaris with Periodontopathia - Papillon Lefevre Disease