Partial expression of the Papillon-Lefevre syndrome.

ABSTRACT

Papillon-Lefevre Syndrome (PLS) is a rare autosomal recessive genodermatosis characterized by palmar-plantar hyperkeratosis, and destructive periodontitis. It is transmitted with an estimated frequency of one to four per million individuals. The two hallmarks of the syndrome, dermatological lesions and destructive periodontitis, are known to occur as an independent diseases. We present a unique case of Papillon-Lefevre syndrome in a 28 years old woman with its pathognomonic dermatological features without oral features.