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An unusual presentation of the Papillon-Lefevre syndrome as recurrent liver abscesses

S Anuradha1, S K Agarwal, N P Singh

  • 1Department of Medicine, Maulana Azad Medical College and Associated Lok Nayak Hospital, New Delhi.

The Journal of the Association of Physicians of India
|July 20, 2002
PubMed

Insights

Papillon-Lefevre syndrome, a rare genetic disorder, can lead to severe infections. This case highlights a unique association with recurrent pyogenic liver abscesses in an affected adolescent.

Area of Science:

  • Genetics
  • Infectious Diseases
  • Dermatology

Background:

  • Papillon-Lefevre syndrome is a rare autosomal recessive disorder.
  • Characterized by palmoplantar hyperkeratosis and periodontitis.
  • Associated with an increased susceptibility to infections.

Observation:

  • A 17-year-old male with Papillon-Lefevre syndrome was studied.
  • He presented with a 9-year history of recurrent pyogenic liver abscesses.
  • Four out of eight siblings in the family were also affected.

Findings:

  • This report details a previously undocumented association between Papillon-Lefevre syndrome and recurrent pyogenic liver abscesses.
  • The patient experienced multiple liver abscesses over nearly a decade.
  • The genetic predisposition of the syndrome likely contributes to severe infection susceptibility.

Implications:

  • This finding expands the known clinical spectrum of Papillon-Lefevre syndrome.
  • Highlights the importance of considering liver abscesses in affected individuals.
  • Suggests potential genetic links between Papillon-Lefevre syndrome and hepatic infections.

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