Papillon-Lefèvre Syndrome: Symptoms, Oral Manifestations & Key Features Explained
⚡ High-Yield Summary
Papillon-Lefèvre Syndrome is a rare autosomal recessive disorder characterized by juvenile periodontitis, palmar–plantar hyperkeratosis, gingival inflammation, and early tooth loss. Learn its features, oral findings, and clinical relevance for NEET MDS, BDS, MBBS, and INBDE.
🔎 What Is Papillon-Lefèvre Syndrome?
Papillon-Lefèvre Syndrome (PLS) is a rare autosomal recessive disorder involving both skin and periodontal tissues.
It typically appears in childhood and is strongly associated with:
Severe early-onset periodontitis
Palmar–plantar hyperkeratosis
Early exfoliation of both primary and permanent teeth
The condition is linked to mutations in the Cathepsin C (CTSC) gene, which affects immune response and epithelial integrity.
📌 Key Oral Manifestations (High-Yield for Exams)
Papillon-Lefèvre Syndrome presents with dramatic dental changes early in life:
✔ Juvenile Periodontitis
Aggressive, rapidly progressing periodontal destruction involving both dentitions.
✔ Gingival Ulcers & Inflammation
Severe gingival swelling, redness, and bleeding.
✔ Gingival Enlargement & Pocket Formation
Deep periodontal pockets appear early → mobility → tooth loss.
✔ Early Tooth Exfoliation
Primary teeth may exfoliate by age 4–5, permanent teeth soon after eruption.
These features make PLS a must-know topic in Oral Pathology & Periodontology.
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