Papillon Lefevre Syndrome: Report of Two Cases in a Family

Vani, C; Reddy, P Krishnanjaneya; Babu, DB Gandhi

doi:10.5005/jp-journals-10011-1013

Search citation statements

Order By: Relevance

Paper Sections

Select...

Papillon-lefevre Syndrome: a Novel Familial Presentation1

Citation Types

Supporting

Mentioning

Contrasting

Year Published

2010

Publication Types

Select...

Article1

Relationship

Self Cite0

Independent1

Authors

Journals

Cited by 1 publication

(1 citation statement)

References 10 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…16 The findings may worsen in winter and be associated with painful fissures. [17][18][19] The second major feature of PLS is severe periodontitis, which starts at age 3 or 4. 17 The development and eruption of the deciduous teeth proceed normally, but their eruption is associated with gingival inflammation and subsequent mobility and migration of the teeth.…”

Section: Papillon-lefevre Syndrome: a Novel Familial Presentationmentioning

confidence: 99%

Papillon-Lefevre Syndrome: A Novel Familial Presentation

Sudhakar¹,

Prabhat²,

Balachandran³

2010

World Journal of Dentistry

View full text Add to dashboard Cite

Papillon-Lefevre syndrome (PLS) is a 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. Various immune cells, including polymorphonuclear leukocytes, macrophages, and their precursors are also affected leading to functional disability. PLS is an autosomal recessive condition and can occur in siblings born of consanguineous marriages. This report highlights a rare instance of two siblings of a family affected with Papillon-Lefevre syndrome.

show abstract

Section: Papillon-lefevre Syndrome: a Novel Familial Presentationmentioning

confidence: 99%