2/10/2020

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

Oral Pathology

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.


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.

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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.

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


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