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Papillon Lefevre syndrome.

S V Dhadke1, P M Kulkarni, V N Dhadke

  • 1Department of Medicine Dr. VM Medical College Solapur.

The Journal of the Association of Physicians of India
|June 28, 2006
PubMed
Summary

Papillon–Lefèvre syndrome, a rare genetic disorder, causes severe skin and dental issues. This report details a case linking it to pyogenic liver abscesses, a known complication.

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Area of Science:

  • Genetics and rare diseases
  • Dermatology
  • Periodontology
  • Hepatology

Background:

  • Papillon–Lefèvre syndrome (PLS) is a rare autosomal recessive disorder.
  • Key features include palmoplantar hyperkeratosis and severe, aggressive periodontitis affecting both primary and permanent teeth.
  • Pyogenic liver abscess (PLA) is an emerging complication associated with PLS.

Observation:

  • This report presents a novel case of Papillon–Lefèvre syndrome associated with a pyogenic liver abscess.
  • The case highlights the clinical presentation and diagnostic considerations for this rare association.

Findings:

  • The study confirms the association between Papillon–Lefèvre syndrome and pyogenic liver abscess.
  • Review of literature indicates an increasing recognition of this complication in PLS patients.

Implications:

  • Early recognition and management of pyogenic liver abscesses are crucial in patients with Papillon–Lefèvre syndrome.
  • Further research is warranted to understand the pathophysiology linking PLS and hepatic complications.
  • This association underscores the importance of a multidisciplinary approach in managing complex rare diseases.

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