Pyogenic liver abscess and Papillon-Lefèvre syndrome: not a rare association.
نویسندگان
چکیده
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.
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 کاملHyperkeratosis palmoplantaris with periodontosis. "Papillon-LeFevre syndrome". Report of three cases in the same family.
Papillon-Lefèvre syndrome is a rare autosomal recessive disorder caused by cathepsin C gene mutation leading to the deficiency of cathepsin C enzymatic activity. The disease is characterized by palmoplantar hyperkeratosis, periodontopathy and precocious loss of dentition, and increased susceptibility to infections. Pyogenic liver abscess is an increasingly recognized complication. Three cases o...
متن کاملPapillon-Lèfevre syndrome with palmoplantar keratoderma and periodontitis, a rare cause of pyrexia of unknown origin: a case report
INTRODUCTION Papillon-Lefèvre Syndrome is a rare autosomal recessive disorder characterized by diffuse, transgradient palmoplantar keratoderma, destructive periodontitis beginning in childhood, premature loss of primary teeth, and frequent cutaneous and systemic pyogenic infections. Pyogenic liver abscess is an uncommon presentation of the disease present in this case. CASE PRESENTATION A 16-...
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 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...
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ورودعنوان ژورنال:
- Pediatrics
دوره 111 1 شماره
صفحات -
تاریخ انتشار 2003