It is feasible that the primary title of the record Papillon Lef vre Syndrome is not the name you expected. Kindly inspect the words listing to discover the alternating name(s) and condition subdivision(s) covered by this record.
- Palmoplantar Keratoderma with Periodontosis
- Palmar-plantar Hyperkeratosis as well as Concomitant Gum Destruction
- Keratoris Palmoplantaris with Periodontopathia
- Hyperkeratosis Palmoplantaris with Periodontosis
Papillon-Lef vre Syndrome (PLS) is an exceptionally unusual congenital disease that typically becomes apparent from approximately one to five years of age. PLS is characterized by the property development of completely dry scaly patches on the skin of the palms and also the soles (palmar-plantar hyperkeratosis) in organization with serious inflammation as well as weakening of the structures bordering and also supporting the teeth (periodontium). The primary (deciduous) teeth often become loosened as well as befall by about age five. Without treatment, a lot of the additional (permanent) teeth may also be shed by approximately age 17. Additional signs as well as findings associated with PLS might include constant pus-producing (pyogenic) skin infections, problems of the nails (nail dystrophy), as well as too much perspiration (hyperhidrosis).
Papillon-Lef vre Syndrome is sent as an autosomal recessive trait. Genetic analysis of several affected families (kindreds) recommends that the disorder might result from changes (mutations) of a gene that regulates production of an enzyme called cathespin C. The gene is located on the lengthy arm (q) of chromosome 11 (11q14).