Papillon-Lefèvre syndrome.
نویسندگان
چکیده
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.
منابع مشابه
Papillon-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 کاملسندروم پاپیلون لفور در یک کودک 4 ساله: گزارش یک مورد بیماری نادر و پیگیری سه ساله آن
Papillon-Lefèvre syndrome is an extremely rare autosomal recessive condition. It is characterized by severe destruction of the periodontium and hyperkeratotic skin lesions on palms, soles, knees, elbows and in some cases calcification of the Dura. The features usually appear together between the ages of 2 and 4 years. Severe periodontal destruction leads to the early shedding of primary and per...
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: Report of a case and its management
Papillon-Lefèvre Syndrome (PLS) is a rare autosomal recessive disorder first described by two French physicians, Papillon and Lefèvre in 1924. The disorder is characterized by diffuse palmoplantar keratoderma and precocious aggressively progressing periodontitis, leading to the premature loss of deciduous and permanent teeth at a very young age. The cutaneous lesions are usually manifested simu...
متن کاملPyogenic 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.
متن کاملPapillon-Lefèvre syndrome and squamous cell carcinoma: a case report
Papillon-Lefèvre syndrome is a rare autosomal recessive genodermatosis characterised by palmoplantar hyperkeratosis and severe early-onset periodontitis. The development of malignant cutaneous neoplasms within the hyperkeratotic lesions of the syndrome is very rare. Here, we report on a 67-year-old German Caucasian male with Papillon-Lefèvre syndrome associated with recurrent squamous cell carc...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Indian pediatrics
دوره 40 12 شماره
صفحات -
تاریخ انتشار 1988