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Current Issue Volume 29 Issue 04 2020
Papillon Lefevre Syndrome: Diagnosis and Management in
Two Affected Siblings – A Case Report
Abul Khair Zalan BDS
Khadeejah Khalil Zubairy BDS
Anser Maxood BDS, FRACDS
Manahil Niazi BDS
Hira Zaman BDS, MDS
Anika Gul BDS
Miraat Anser BDS
Papillon-Lefèvre syndrome (PLS) an autosomal recessive disorder characterized by diffuse transgradient palmar-plantar hyperkeratosis, with premature loss of deciduous and permanent teeth, along with the calcification of the dura mater. This results in teeth radiographically appearing as “floating” in the soft tissue. Genetic studies of patients with PLS have mapped the major gene locus to chromosome 11q24-q21 and revealed mutation and loss of function of the cathepsin gene.
Vol. 29, Issue No. 04 – October – December 2020
Past issues of the Journal of Pakistan Dental Association are published here.