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:: Volume 3, Issue 1 (Spring 2011 2011) ::
JNKUMS 2011, 3(1): 75-78 Back to browse issues page
Report of 2 cases of Papillon Syndrome
HR Kianifar , A Khakshour , A Jahanbin , SA Jafari , S.H Hosseini
Abstract:   (798 Views)

Abstract Papillon Lefevre Syndrome (PLS) is a rare autosomal recessive disorder, which is characterized by palmar-plantar hyperkeratosis, periodontitis, and premature loss of dentition leading to loss of teeth by age 4–5 yr if untreated. This syndrome is recoginized by progressive pyogenic liver abscess Pyogenic liver abscess is an increasingly recognized complication and operation. Rapid diagnosis and immediate intervention are essential because any delay in diagnosis could increase mortality and irreversible complications. In this study we report two patients )two brothers aged 12 and 14) who presented with fever of unknown origin in whom pyogenic liver abscess was diagnosed. They had to undergo surgery and were shown to have PLS. immediately following diagnosis, suitable treatment procedures resulted in the patients' recovery and subsequent discharge. The aim of this report is to highlight the importance of paying attention to patients with fever of unknown origin as well as dental and skin lesions. Early diagnosis and suitable treatment procedures can prevent subsequent complications.

Keywords: Keywords: Hyperkeratosis, Papillon Lefevre Syndrome, Periodontitis, Pyogenic Abscesis
Full-Text [PDF 408 kb]   (435 Downloads)    
Type of Study: Case Report | Subject: Basic Sciences
Received: 2015/01/12 | Accepted: 2015/01/12 | Published: 2015/01/12
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Kianifar H, Khakshour A, Jahanbin A, Jafari S, Hosseini S. Report of 2 cases of Papillon Syndrome. JNKUMS. 2011; 3 (1) :75-78
URL: http://journal.nkums.ac.ir/article-1-243-en.html


Volume 3, Issue 1 (Spring 2011 2011) Back to browse issues page
مجله دانشگاه علوم پزشکی خراسان شمالی Journal of North Khorasan University of Medical Sciences
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