Papillon–Lefèvre syndrome is also referred to as PLS and palmoplantar keratoderma and it is an autosomal recessive genetic disorder. The cause of this problem is deficiency in cathepsin C. The patient here would experience extreme signs of weakness in gums and destructions of periodontium. This usually commences after the 4th molar tooth comes out and teeth are lost before the age of 4.
HOW IS PAPILLON-LEFÈVRE SYNDROME DIAGNOSED?
PLS is diagnosed by a clinical evaluation including a detailed patient history and identification of characteristic physical findings. In some cases, skin abnormalities may be apparent at birth or during infancy including characteristic skin abnormalities on the palms of the hands and the soles of the feet. However, in most cases, the diagnosis of the disorder may not be confirmed until inflammation and degeneration of the tissues surrounding and supporting the teeth become apparent.
HOW IS PAPILLON-LEFÈVRE SYNDROME TREATED?
The treatment of Papillon-Lefèvre Syndrome is directed toward the specific symptoms that are apparent in each individual. Different specialists would need to work together to treat the various unrelated symptoms. In some cases, surgery and skin grafts may be used to alleviate skin problems.
DID YOU KNOW?
Skin abnormalities may sometimes be apparent at birth or during infancy.