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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.
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Vol. 29, Issue No. 04 – October – December 2020

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