Papillon-Lefevre syndrome

Papillon-Lefèvre syndrome (PLS) is an autosomal recessive genetic disorder caused by a deficiency in cathepsin C.



Symptoms
PLS is characterized by periodontitis and palmoplantar keratoderma. The severe destruction of periodontium results in loss of most primary teeth by the age of 4 and most permanent teeth by age 14. Hyperkeratosis of palms and soles of feet appear in first few years of life.

Treatment
Retinoids and antibiotics have been used.

Eponym
It is named for M. M. Papillon and Paul Lefèvre.