Journal of North Khorasan

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.