Papillon-Lefèvre Syndrome: A Series of Six Cases in the Same Family
نویسندگان
چکیده
منابع مشابه
Papillon-Lefèvre Syndrome: A Series of Six Cases in the Same Family
Papillon-Lefèvre syndrome (PLS) is a rare, autosomal recessive heterogeneous disorder, which is characterized by palmoplantar hyperkeratosis, early loss of primary and permanent teeth, and associated calcification of the dura mater. Herein we described six cases of PLS in the same family. In this series, six cases (two females and four males) with the mean age of 15.6 ± 10.4 years were recruite...
متن کاملPapillon–Lefèvre syndrome: a series of five cases among siblings
BACKGROUND Papillon-Lefèvre syndrome is a rare autosomal recessive disorder characterized by palmoplantar hyperkeratosis and aggressively progressing periodontitis leading to premature loss of deciduous and permanent dentition. The etiopathogenesis of the syndrome is relatively obscure, and immunologic, genetic, or possible bacterial etiologies have been proposed. CASE PRESENTATION A series o...
متن کاملPapillon-Lefèvre syndrome.
Papillon- Lefèvre syndrome (PLS) is a rare autosomal recessive disorder of keratinization characterized by palmoplantar hyperkeratosis, periodontopathy and precocious loss of dentition. The exact pathomechanism of these clinical events mainly remains speculative. This paper describes two cases of PLS with classic clinical features and briefly review the relevant literature.
متن کاملPapillon-Lefèvre Syndrome: a case report
Introduction: Papillon-Lefèvre syndrome(PLS) characterized by palmoplantar hyperkeratosis is a rare autosomal recessive genetic disorder with rapidly progressive periodontitis and premature loss of both deciduous and permanent teeth. In this study, we report the clinical and radiographic features of Papillon-Lefèvre syndrome in an 11- year-old girl and we also discuss the history ...
متن کاملPapillon-Lefevre syndrome: A report of two cases in a family
Palmoplantar keratodermas are a heterogenous group of diseases, one of them is Papillon-Lefevre Syndrome (PLS). This rare disease is inherited as autosomal recessive and characterized by focal hyperkeratotic plaques on elbows and knees, severe periodontal disease resulting in premature loss of teeth. We report two brothers with PLS who did not have a history of this disease in their famil...
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ژورنال
عنوان ژورنال: ISRN Dermatology
سال: 2012
ISSN: 2090-4606
DOI: 10.5402/2012/139104