Papillon-Lefèvre Syndrome — NDEB AFK MCQ

Instant feedback + full explanation. One question, done properly.

ModeratePeriodontologyPapillon-Lefèvre SyndromeNDEB AFK

Which of the following systemic conditions is most strongly associated with aggressive periodontal destruction in children and young adults?

Educational content. Not a substitute for clinical judgement or local policy.

Reveal the answer and explanation

Correct answer: B — Papillon-Lefèvre syndrome

Papillon-Lefèvre syndrome (PLS) is an autosomal recessive condition caused by mutations in the cathepsin C gene (CTSC). It is characterised by palmoplantar hyperkeratosis (thickened skin on palms and soles) and severe aggressive periodontitis leading to premature loss of both primary and permanent teeth. The periodontal destruction is devastating — primary teeth are typically lost by age 4-5, and permanent teeth by age 14-16. A. actinomycetemcomitans is frequently implicated. Treatment includes aggressive periodontal therapy, extraction of hopeless teeth, and prosthetic rehabilitation.

Reference: Neville – Oral and Maxillofacial Pathology, 4th ed., 2016