Papillon-Lefèvre syndrome in a father and son

This paper describes Papillon-Lefevre syndrome (PLS) in a father and son.The son came to our hospital on May 27, 1995 because of loose front teeth in the upper and lower jaws with dental separation. The patient had coronations on his palms and soles, first noticed at 2 months of age, and palmoplantar keratoderma was diagnosed when he was 3 years old.General physical examination showed coronations on the palms and soles as well as dental mobility with separation, gingival flare, and swelling in the oral cavity. His father also showed cornification on the palms and soles and had edentulous jaws.X-ray examination showed horizontal bone resorption in both patients. These results led to a diagnosis of PLS.As PLS is a genetic disease, chromosomal and immunological examinations were performed. Immunological examination showed the following abnormalities in the son: CD11b+CD8+, CD11b+CD8-, and CD11b+CD8++.