Pyogenic liver abscess and peritonitis due to Rhizopus oryzae in a child with Papillon-Lefevre syndrome

Eur J Pediatr. 2011 Jun;170(6):803-5. doi: 10.1007/s00431-010-1368-3. Epub 2010 Dec 17.


Papillon-Lefevre syndrome (PLS) is an autosomal recessive disease that is characterized by symmetric palmoplantar keratodermatitis and severe periodontal destruction. Mutations in the cathepsin C gene (CTSC) have recently been detected in PLS. Immune dysregulation, due to a mutation in CTSC, increases the risk of pyogenic infections in PLS patients. A child with PLS is presented here with liver abscesses and peritonitis caused by Rhizopus oryzae. His liver abscess and peritonitis were cured with amphotericin B without surgical care. This is the first case in the literature liver abscess due to Rhizopus oryzae in a child with PLS.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Amphotericin B / therapeutic use
  • Humans
  • Liver Abscess, Pyogenic / drug therapy
  • Liver Abscess, Pyogenic / microbiology*
  • Male
  • Mucormycosis / complications*
  • Mucormycosis / diagnosis*
  • Mucormycosis / drug therapy
  • Papillon-Lefevre Disease / diagnosis*
  • Periodontitis / drug therapy
  • Periodontitis / microbiology*
  • Rhizopus / isolation & purification*
  • Treatment Outcome


  • Amphotericin B