Papillon-Lefevre Syndrome is a rare disorder that is often evident in the age group of one to five year olds. The condition is characterized by the development of dry scaly patches on the skin of the palms and the soles along with degeneration of the structures surrounding the teeth. There can be frequent skin infections as well as abnormalities of nails in the affected children.
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. Treatment may require the coordinated efforts of a team of specialists. Physicians may carefully monitor affected individuals to help prevent and ensure early identification of infection. Should gum infection occur antibiotic therapy may be prescribed. In some cases, surgery and skin grafts may be used to alleviate skin problems. Use of antiperspirants and deodorants may help with excessive perspiration. Genetic counseling may be of benefit for affected individuals and their families. Other treatment is symptomatic and supportive. Treatment with vitamin A (retinoids) has been found helpful in treating some individuals with Papillon-Lefèvre Syndrome.
DID YOU KNOW?
Though rare, this is disease can cause a lot of uneasiness.