Case Report: Clinical manifestation and dental management of Papillon-Lefèvre syndrome

Name	World Continuing Education Alliance
Activity Title	Case Report: Clinical manifestation and dental management of Papillon-Lefèvre syndrome
Details	Papillon-Lefèvre syndrome (PLS) is an autosomal recessive disorder that typically becomes apparent from one to five years of age, which coincides with the timing of eruption of primary dentition. The estimated prevalence of the syndrome is 1–4 cases per million individuals. The exact etiopathogenesis of the syndrome is relatively unclear and different etiological factors have been suggested, such as immunologic, genetic or bacterial, but recently it was suggested that mutations of cathepsin C gene, which results in deficiency of cathepsin C enzymatic activity, to be the possible etiological factor. This was supported by the fact that expression of the cathepsin C gene occurs mainly in epithelial regions, such as the soles, palms and keratinized oral gingiva, which are the most affected areas in patients with PLS. An important feature of the syndrome is the presence of palmoplantar hyperkeratosis; its onset usually occurs between the ages of one to four years and usually involves the pa... Objectives Course Objective Papillon-Lefèvre syndrome (PLS) is considered a rare syndrome, which is characterized by the presence of palmar-plantar hyperkeratosis and aggressively progressing periodontitis that finally leads to premature loss of both deciduous and permanent teeth.. A four-year-old Egyptian boy presented with a maternal complaint that her child suffers from early loss of many teeth, presence of loose teeth along with an asymptomatic swelling related to the upper anterior area.
Competence	General Medicine
Start Date	<span class="not-set">(not set)</span>
End Date	<span class="not-set">(not set)</span>
Event Time	11:45 AM
Location	World Continuing Education Alliance eLearning System
Cost (UGX)	0
CPD Points	1