Papillon-Lefevre Disease

Synonyms

Haim Monk Syndrome

Haim-Monk Syndrome

Keratosis Palmoplantar Periodontopathies

Keratosis Palmoplantar Periodontopathy

Keratosis Palmoplantaris with Periodontopathia

Palmoplantar Periodontopathies, Keratosis

Papillon Lefevre Disease

Papillon Lefevre Syndrome

Papillon-Lefevre Syndrome

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.

See Also