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ORAL PATHOLOGY : Report of a case of Papillon-Lefevre syndrome

Papillon-Lefevre Syndrome is a rare, autosomal recessive disorder. It is characterized by dermatological and periodontal changes that include palmar-plantar hyperkeratosis and generalized aggressive periodontitis in both primary and permanent dentition. 

Hiam-Munk syndrome and hypophosphatasia are some of its differential diagnoses. Dentists and dermatologists both should be aware of its characteristics which help to have an early diagnosis, a multidisciplinary approach and better prognosis. 

Papillon-Lefevre-syndrome

Here, we present a case of Papillon-Lefevre Syndrome in a 4-year-old girl who was brought to a private dental office with the chief complaint of tooth mobility. 


Introduction : Papillon-Lefevre Syndrome (PLS) is a rare, autosomal recessive disorder. The syndrome is believed to affect 1 person per 4 million people with no sex predilection or racial predominance. 

It is characterized by the development of dry scaly patches on the skin of palms and soles (palmar-plantar hyperkeratosis) along with generalized aggressive periodontitis in both primary and permanent dentition. 



°indjos.com 
°Indian Journal of Oral Sciencies 
°Saeedeh Mokhtari, Ali Lotfi, Sepideh Mokhtari