Pyogenic Liver Abscess and Papillon-Lefevre Syndrome: Not a Rare Association

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Pyogenic liver abscess and Papillon-Lefèvre syndrome: not a rare association.

Papillon-Lefèvre syndrome is a rare, autosomal recessive disease comprising palmoplantar keratoderma and periodontitis. Pyogenic liver abscess is an increasingly recognized complication. We report a new case of this association and review the current literature.

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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...

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17. 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...

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Papillon-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.

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Management 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...

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ژورنال

عنوان ژورنال: PEDIATRICS

سال: 2003

ISSN: 0031-4005,1098-4275

DOI: 10.1542/peds.111.1.e85