Papillon–Lefèvre syndrome

The Papillon -Lefevre syndrome is a rare autosomal recessive inherited hyperkeratosis of the skin ( hyperkeratosis), mainly on the palms and soles, with severe periodontitis. The milk teeth and permanent teeth of patients go because of rapid bone loss usually early lost. The inflammatory gums ( gingiva) has the often observed in the presence of systemic systemic diseases crimson gingival margin.

The underlying mutation is in the gene for the cathepsin C on chromosome 11

Swell

• E. Hellwig, J. Klimek, T. Attin Introduction to Restorative Dentistry. Urban and Fischer 2003 ISBN 978-3437050510