Papillon-Lefevre Syndrome – Symptoms, Causes, Treatment
Write short note on Papillon-Lefevre syndrome.
Answer. Papillon-Leférve syndrome is an autosomal recessive and inherited disorder.
- Papillon-Leférve syndrome is characterized by mutation in cathapsin C gene located on chromosome 11.
- Patients with Papillon–Lefévre syndrome has little or no cathepsin C activity.
- This syndrome was first described by papillon and Lefevre in 1924.
Papillon-Lefèvre syndrome
“Role of CTSC gene mutation in Papillon-Lefèvre Syndrome”
Triad
Papillon-Leférve syndrome is a triad of:
- Calcification of dura
- Hyperkeratosis of palms of hand, soles of foot
- Extensive pre-pubertal destruction of periodontal bone supporting the dentition
“Clinical examination for Papillon-Lefèvre Syndrome”
“Genetic factors leading to Papillon-Lefèvre Syndrome”
Papillon-Lefevre Syndrome Clinical Features
Papillon-Leférve syndrome Oral Manifestations
- Gingival swelling and severe halitosis.
- Horizontal PDL bone destruction, PDL pocket
- Loss of primary dentition by the age of 5 years
- Loss of secondary dentition by the age of 20 years.
“Impact of enzyme deficiency in Papillon-Lefèvre Syndrome”
Papillon-Leférve syndrome Skin Manifestations
- Dirty colored skin
- Reddened, scaly, rough palms and soles.
Papillon-Lefevre Syndrome Treatment
No specific treatment.
Papillon-Lefèvre syndrome treatment
“Role of genetic testing in identifying Papillon-Lefèvre Syndrome”
Dental Management
- Systemic administration of retinoids, when combined with meticulous plaque control, debridement, topical antimicrobials along with systemic antibiotic therapy may give the best chance for preventing the progression of periodontitis.
- Severely involved teeth must be extracted.
- The loss of teeth is almost inevitable, even with a high degree of patient compliance
- A more realistic aim is to maintain alveolar bone height to eventually support removable Implant-retained prosthesis.
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