منابع مشابه
Papillon Lefevre syndrome.
Papillon Lefèvre syndrome is a rare disease characterized by skin lesions caused by palmar-plantar hyperkeratosis, and severe periodontal destruction involving both the primary and permanent dentitions. It is transmitted as an autosomal recessive condition and consanguinity of parents is evident in about one-third of cases. Pyogenic liver abscess is an increasingly recognized complication. We r...
متن کامل17. Papillon-Lefevre syndrome
Papillon-Lefevre syndrome is a rare autosomal recessive disorder and is characterized by the diffuse palmoplantar hyperkeratosis with rapid destruction of the periodontal support of both the primary and secondary dentition. Patient is often completely or partially edentulous at the stage of primary (4-5 yrs) and permanent dentition (13-15 yrs). Though, exact etiopathogenesis is unknown; three m...
متن کاملPapillon-lefevre Syndrome.
Five cases of Papillon-Lefevre syndrome occurring in 2 families were seen to have variable clinical features. Two of these cases treated with etretinate showed a good response.
متن کاملManagement of Papillon Lefevre Syndrome
It is a rare, autosomal recessive disorder occurring between the f irst and fi f th years of l i fe and is characterized by palmoplantar keratoderma and periodontitis fol lowed by the premature shedding of both primary and permanent teeth. The teeth are affected in the order of their eruption, exhibiting inflammation of the periodontal tissue, bleeding of the gums, pocket formation, loosening o...
متن کاملPapillon-Lefevre Syndrome: A Case Report
PapillonLefevre syndrome (PLS) is a very rare autosomal recessive disorder characterized by palmoplantar hyperkeratosis and severe early onset of destructive periodontitis leading to premature loss of both primary and permanent dentition.PLS is transmitted as an autosomal recessive condition and consanguinity of parents is evident in about one third of cases. Here we report a case of a 13 year ...
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ژورنال
عنوان ژورنال: QJM: An International Journal of Medicine
سال: 2017
ISSN: 1460-2725,1460-2393
DOI: 10.1093/qjmed/hcx090