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Partial expression of the Papillon-Lefevre syndrome.

Research paper by Muhammad M Shanavas, Laxmikanth L Chatra, Prashanth P Shenai, Sreeja S Kumar, N N Bilahari, Siva S Balakrishnan

Indexed on: 19 Dec '14Published on: 19 Dec '14Published in: Journal of the College of Physicians and Surgeons--Pakistan : JCPSP



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.