Papillon-Lefèvre syndrome with pyogenic liver abscess : case report focusing on radiological findings and review of the literature

We present the results of CT and US in the rare case of Papillon-Lefèvre syndrome (PLS) with multiple pyogenic liver abscesses. A 26-year-old woman presented with fever and right hypochondrial pain. At 3 years of age, she had been diagnosed as having PLS at another hospital. She had a history of abdominal, liver, and renal abscess. Again, a liver abscess was suspected, and Staphylococcus aureus was found in the drainage fluid specimen. She was trans- ferred to our hospital. Upon admission, her temperature was 37.2 °C and her pulse was 94 per minute. She had mild keratosis of the soles. Although she had a history of periodontosis, her permanent teeth were still in place probably because of continual treatment for periodon- tosis. The laboratory results showed a total white blood cell count of 12,500/µL and a C-reactive protein of 6.13 mg/dL. The nitroblue tetrazolium (NBT) reduction test showed a reduced response to latex stimulation (12%). Contrast-enhanced CT and US of the abdomen revealed multiple ill-defined hypodense and inhomoge- neous masses with peripheral enhancement in the liver (Fig. 1). Our diagnosis was pyogenic liver abscess asso- ciated with PLS, as no other causes of liver abscess could be demonstrated. After treatment, the patient recovered and was discharged four months after admission. Further CTs performed 16 months later showed that the lesions were well defined and reduced, suggesting absorption of the liver abscess (Fig. 2). The patient is now symptom- free, and periodic observation in the outpatient depart- ment is maintained.