Papillon - lefe'vere syndrome - A report of three cases in same family

Papillon-Lefevre syndrome is a rare inherited autosomal recessive condition characterized by palmar-plantar hyperkeratosis and early onset of a severe destructive periodontitis, leading to premature loss of both primary and permanent dentitions. An early diagnosis of the condition & use of multidisciplinary treatment approach can help to preserve the dentition & control the dermatological lesions. The current case series presents three cases of Papillon-Lefevre syndrome in the same family having all of the characteristic features along with a comprehensive review of the etiology, pathology, clinical features, differential diagnosis and management of the condition.