Papillon-Lefèvre Syndrome: A Rare Case Report of Two Brothers and Review of the Literature
January 2018
in “
Jaypee's international journal of clinical pediatric dentistry
”
TLDR Papillon-Lefèvre Syndrome causes early tooth loss and skin issues, needing early dental diagnosis.
Papillon–Lefèvre Syndrome (PLS) was a rare autosomal recessive disorder marked by palmoplantar keratoderma and severe periodontitis, leading to early tooth loss. This case report detailed two brothers with PLS, highlighting their clinical symptoms, diagnostic process, and management strategies. The literature review noted that PLS was caused by mutations in the cathepsin C gene. Early diagnosis and multidisciplinary management were crucial for improving outcomes, with genetic counseling and regular dental care being essential components of treatment.