Papillon-Lèfevre syndrome with palmoplantar keratoderma and periodontitis, a rare cause of pyrexia of unknown origin: a case report

J Med Case Rep. 2015 Dec 18:9:288. doi: 10.1186/s13256-015-0773-7.


Introduction: Papillon-Lefèvre Syndrome is a rare autosomal recessive disorder characterized by diffuse, transgradient palmoplantar keratoderma, destructive periodontitis beginning in childhood, premature loss of primary teeth, and frequent cutaneous and systemic pyogenic infections. Pyogenic liver abscess is an uncommon presentation of the disease present in this case.

Case presentation: A 16-year-old Punjabi, Pakistani boy presented to the outpatient department of a tertiary-care hospital of Lahore with high-grade fever of 2 months duration. He had been treated for a pyogenic liver abscess 2 years back with antibiotics followed by incision and drainage. He had poor orodental hygiene, palmoplantar keratoderma and periodontitis. His parents had history of consanguinity. His brother and two cousins had similar skin lesions and were edentulous. An orthopentogram showed atrophy of the alveolar bone. He was treated with broad-spectrum antibiotics, and antipyretics for systemic infection. Multivitamins, topical steroids, topical keratolytics and emollients were used for his dermatological issues.

Conclusions: Our patient was successfully treated. His fever settled and his skin lesions improved with antibiotics, topical steroids and keratolytics. He was sent home and was asked to return for follow-up on a monthly basis.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Anti-Bacterial Agents / administration & dosage*
  • Fever / drug therapy
  • Fever / etiology
  • Fever / pathology*
  • Humans
  • Keratoderma, Palmoplantar / etiology
  • Keratoderma, Palmoplantar / pathology*
  • Male
  • Papillon-Lefevre Disease / complications
  • Papillon-Lefevre Disease / diagnosis*
  • Papillon-Lefevre Disease / drug therapy
  • Papillon-Lefevre Disease / pathology
  • Periodontitis / drug therapy
  • Periodontitis / pathology*
  • Treatment Outcome


  • Anti-Bacterial Agents