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标题：Papillon-Lefèvre syndrome of Iranian girl: Case report and review of the literature
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作者：Jalaleddin Hamissi ; Hesameddin Hamissi
期刊名称：International Journal of Collaborative Research on Internal Medicine & Public Health
电子版ISSN：1840-4529
出版年度：2012
卷号：4
期号：12
出版社：DRUNPP Sarajevo
摘要：Papillon- Lefevre syndrome (PLS) is one of rare autosomal recessive disorder which characterized by hyperkeratosis of palms and soles and diagnosed in both sexes and it may have severe destructive periodontal disease affecting the primary and permanent teeth. The exact patho-mechanism of these clinical syndrome events mainly remains speculative. This is transmitted as a recessive autosomal condition and consanguinity of parents has apparent in about one-third of all cases .This paper describes classic clinical features and briefly reviews the relevant current literature. An 11 years old female presented with keratotic plaques over the skin of her palms and soles extending on the dorsal surface and swollen gums since the age of 4 with subsequent loss of most of his permanent dentition.
关键词：palmar-plantar keratoderma; Papillon-Lefevre syndrome (PLS); periodontitis