🔎 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.