Papillon-Lefèvre Syndrome (PLS) is an extremely rare genetic disorder that typically becomes apparent from approximately one to five years of age. PLS is characterized by the development of dry scaly patches on the skin of the palms and the soles in association with severe inflammation and degeneration of the structures surrounding and supporting the teeth. Additional symptoms and findings associated with PLS may include frequent pus-producing skin infections, abnormalities of the nails (nail dystrophy), and excessive perspiration
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?
Topical lubricants may also be used for temporary relief.