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International Journal of Advances In Case Reports

Volume 11, Issue 1, 2024
Mcmed International
International Journal of Advances In Case Reports
Issn
XXX-XXXX (Print), 2349 - 8005 (Online)
Frequency
bi-annual
Email
editorijacr@mcmed.us
Journal Home page
http://mcmed.us/about/ijacr
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Abstract
Title
PAPILLON-LEFEVRE SYNDROME: A CASE REPORT AND REVIEW OF LITERATURE
Author
Faizal C.Peedikayil1* and Soni Kottayi2
Email
drfaizalcp@gmail.com
keyword
Papillon-Lefevre syndrome, Autosomal recessive disorder.
Abstract
Papillon-Lefevre syndrome (PLS) is an autosomal recessive condition characterized by dermatological manifestations and early onset periodontitis. The pathogenesis of PLS is secondary to mutation of the cathepsin C gene. Hence, the manifestations are expressed on the areas of the body covered by epithelium, such as palms, soles, knees and keratinized oral gingiva. PLS and can occur in siblings born of consanguineous marriages. This case report discusses the literature review of pathogenesis, clinical features and treatment options in a 10 year old child.
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