Papillon-Lefèvre syndrome (PLS) is a rare autosomal recessive disorder, characterized by diffuse palmoplantar keratoderma and premature loss of deciduous and permanent teeth. Parental consanguinity is often found in this autosomal recessive disease. The causative gene has been mapped to the short arm of chromosome 11. The gene codes for cathepsin C, a lysosomal protease that plays an important role in cellular maintenance.


Palmoplantar keratoderma in a glove and stocking distribution (transgrediens), as in mal de Meleda in a child associated with periodonopathy and loss of teeth is characteristic. Painful fissures may form on the soles making walking difficult. The deciduous teeth develop normally, but then destruction of the periodontium, purulent gingivitis, and a fetid mouth odor accompany tooth loss which is often complete by age 4-5. After the deciduous teeth are lost, the gingiva return to normal. With the eruption of the permanent teeth, the process is repeated. Most teeth are lost by age 14. Several other findings have been associated, most notably an increased susceptibility to infection.


Oral retinoids can prevent tooth loss and improve the skin. Genetic counseling should be done.


Clin Cosmet Investig Dent. 2015 Jul 15;7:75-81


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