Papillon- Lefèvre Syndrome: Report of a case and its management
Identifieur interne : 001D43 ( Pmc/Checkpoint ); précédent : 001D42; suivant : 001D44Papillon- Lefèvre Syndrome: Report of a case and its management
Auteurs : Shabina Sachdeva ; Namita Kalra ; Pranav KapoorSource :
- Journal of Clinical and Experimental Dentistry [ 1989-5488 ] ; 2012.
Abstract
Papillon-Lefèvre Syndrome (PLS) is a rare autosomal recessive disorder first described by two French physicians, Papillon and Lefèvre in 1924. The disorder is characterized by diffuse palmoplantar keratoderma and precocious aggressively progressing periodontitis, leading to the premature loss of deciduous and permanent teeth at a very young age. The cutaneous lesions are usually manifested simultaneously with the intra-oral presentations and include keratotic plaques on the palms and soles varying from mild psoriasiform scaly skin to overt hyperkeratosis. The etiopathogenesis of the syndrome is relatively obscure and immunologic, genetic or possible bacterial etiologies have been proposed. Due to the vast degree of periodontal breakdown involved at such an early age, the dental surgeon is often the first to diagnose the syndrome. This paper presents a clinical presentation a 15 year old male diagnosed with Papillon- Lefèvre Syndrome.
Url:
DOI: 10.4317/jced.50594
PubMed: 24558530
PubMed Central: 3908815
Affiliations:
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<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Papillon- Lefèvre Syndrome:
Report of a case and its management
</title><author><name sortKey="Sachdeva, Shabina" sort="Sachdeva, Shabina" uniqKey="Sachdeva S" first="Shabina" last="Sachdeva">Shabina Sachdeva</name><affiliation><nlm:aff id="A1">MDS. Formerly: Senior Resident, Department of Dentistry, University College of Medical Sciences, New Delhi -110095. Presently: Assistant Professor, Faculty of Dentistry, Jamia Millia Islamia, New Delhi -110025</nlm:aff><wicri:noCountry code="subfield">New Delhi -110025</wicri:noCountry></affiliation></author><author><name sortKey="Kalra, Namita" sort="Kalra, Namita" uniqKey="Kalra N" first="Namita" last="Kalra">Namita Kalra</name><affiliation><nlm:aff id="A2">MDS. Professor & Head, Department of Dentistry, University College of Medical Sciences, New Delhi-110095</nlm:aff><wicri:noCountry code="subfield">New Delhi-110095</wicri:noCountry></affiliation></author><author><name sortKey="Kapoor, Pranav" sort="Kapoor, Pranav" uniqKey="Kapoor P" first="Pranav" last="Kapoor">Pranav Kapoor</name><affiliation><nlm:aff id="A3">MDS. Formerly: Lecturer, Department of Dentistry, University College of Medical Sciences, New Delhi -110095. Presently: Assistant Professor, Faculty of Dentistry, Jamia Millia Islamia, New Delhi -110025</nlm:aff><wicri:noCountry code="subfield">New Delhi -110025</wicri:noCountry></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">PMC</idno><idno type="pmid">24558530</idno><idno type="pmc">3908815</idno><idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3908815</idno><idno type="RBID">PMC:3908815</idno><idno type="doi">10.4317/jced.50594</idno><date when="2012">2012</date><idno type="wicri:Area/Pmc/Corpus">001D66</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">001D66</idno><idno type="wicri:Area/Pmc/Curation">001D66</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Curation">001D66</idno><idno type="wicri:Area/Pmc/Checkpoint">001D43</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Checkpoint">001D43</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Papillon- Lefèvre Syndrome:
Report of a case and its management
</title><author><name sortKey="Sachdeva, Shabina" sort="Sachdeva, Shabina" uniqKey="Sachdeva S" first="Shabina" last="Sachdeva">Shabina Sachdeva</name><affiliation><nlm:aff id="A1">MDS. Formerly: Senior Resident, Department of Dentistry, University College of Medical Sciences, New Delhi -110095. Presently: Assistant Professor, Faculty of Dentistry, Jamia Millia Islamia, New Delhi -110025</nlm:aff><wicri:noCountry code="subfield">New Delhi -110025</wicri:noCountry></affiliation></author><author><name sortKey="Kalra, Namita" sort="Kalra, Namita" uniqKey="Kalra N" first="Namita" last="Kalra">Namita Kalra</name><affiliation><nlm:aff id="A2">MDS. Professor & Head, Department of Dentistry, University College of Medical Sciences, New Delhi-110095</nlm:aff><wicri:noCountry code="subfield">New Delhi-110095</wicri:noCountry></affiliation></author><author><name sortKey="Kapoor, Pranav" sort="Kapoor, Pranav" uniqKey="Kapoor P" first="Pranav" last="Kapoor">Pranav Kapoor</name><affiliation><nlm:aff id="A3">MDS. Formerly: Lecturer, Department of Dentistry, University College of Medical Sciences, New Delhi -110095. Presently: Assistant Professor, Faculty of Dentistry, Jamia Millia Islamia, New Delhi -110025</nlm:aff><wicri:noCountry code="subfield">New Delhi -110025</wicri:noCountry></affiliation></author></analytic><series><title level="j">Journal of Clinical and Experimental Dentistry</title><idno type="eISSN">1989-5488</idno><imprint><date when="2012">2012</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><p>Papillon-Lefèvre Syndrome (PLS) is a rare autosomal recessive disorder first described by two French physicians, Papillon and Lefèvre in 1924. The disorder is characterized by diffuse palmoplantar keratoderma and precocious aggressively progressing periodontitis, leading to the premature loss of deciduous and permanent teeth at a very young age. The cutaneous lesions are usually manifested simultaneously with the intra-oral presentations and include keratotic plaques on the palms and soles varying from mild psoriasiform scaly skin to overt hyperkeratosis. The etiopathogenesis of the syndrome is relatively obscure and immunologic, genetic or possible bacterial etiologies have been proposed. Due to the vast degree of periodontal breakdown involved at such an early age, the dental surgeon is often the first to diagnose the syndrome. This paper presents a clinical presentation a 15 year old male diagnosed with Papillon- Lefèvre Syndrome.
</p><p><bold> Key words:</bold>Papillon-Lefèvre Syndrome, palmoplantar keratoderma, rapidly progressing periodontitis.</p></div></front><back><div1 type="bibliography"><listBibl><biblStruct><analytic><author><name sortKey="Haneke, E" uniqKey="Haneke E">E Haneke</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Gorlin, Rj" uniqKey="Gorlin R">RJ Gorlin</name></author><author><name sortKey="Sedano, H" uniqKey="Sedano H">H Sedano</name></author><author><name sortKey="Anderson, Ve" uniqKey="Anderson V">VE Anderson</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Almuneef, M" uniqKey="Almuneef M">M Almuneef</name></author><author><name sortKey="Al Khenaizan, S" uniqKey="Al Khenaizan S">S Al Khenaizan</name></author><author><name sortKey="Al Ajaji, S" uniqKey="Al Ajaji S">S Al Ajaji</name></author><author><name sortKey="Al Anazi, A" uniqKey="Al Anazi A">A Al-Anazi</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Hattab, Fn" uniqKey="Hattab F">FN Hattab</name></author><author><name sortKey="Amin, Wm" uniqKey="Amin W">WM Amin</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Subramaniam, P" uniqKey="Subramaniam P">P Subramaniam</name></author><author><name sortKey="Mathew, S" uniqKey="Mathew S">S Mathew</name></author><author><name sortKey="Gupta, Kk" uniqKey="Gupta K">KK Gupta</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Firatli, E" uniqKey="Firatli E">E Firatli</name></author><author><name sortKey="Gurel, N" uniqKey="Gurel N">N Gürel</name></author><author><name sortKey="Efeoglu, A" uniqKey="Efeoglu A">A Efeoglu</name></author><author><name sortKey="Badur, S" uniqKey="Badur S">S Badur</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Toomes, C" uniqKey="Toomes C">C 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uniqKey="Hart T">TC Hart</name></author><author><name sortKey="Hart, Ps" uniqKey="Hart P">PS Hart</name></author><author><name sortKey="Bowden, Dw" uniqKey="Bowden D">DW Bowden</name></author><author><name sortKey="Michalec, Md" uniqKey="Michalec M">MD Michalec</name></author><author><name sortKey="Callison, Sa" uniqKey="Callison S">SA Callison</name></author><author><name sortKey="Walker, Sj" uniqKey="Walker S">SJ Walker</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Ryu, Oh" uniqKey="Ryu O">OH Ryu</name></author><author><name sortKey="Choi, Sj" uniqKey="Choi S">SJ Choi</name></author><author><name sortKey="Firatli, E" uniqKey="Firatli E">E Firatli</name></author><author><name sortKey="Choi, Sw" uniqKey="Choi S">SW Choi</name></author><author><name sortKey="Hart, Ps" uniqKey="Hart P">PS Hart</name></author><author><name sortKey="Shen, Rf" uniqKey="Shen R">RF Shen</name></author></analytic></biblStruct><biblStruct><analytic><author><name 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sortKey="Kugel, B" uniqKey="Kugel B">B Kugel</name></author><author><name sortKey="Pohl, S" uniqKey="Pohl S">S Pohl</name></author><author><name sortKey="N Her, H" uniqKey="N Her H">H Näher</name></author><author><name sortKey="Staehle, Hj" uniqKey="Staehle H">HJ Staehle</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Al Khenaizan, S" uniqKey="Al Khenaizan S">S Al-Khenaizan</name></author></analytic></biblStruct></listBibl></div1></back></TEI><pmc article-type="case-report"><pmc-dir>properties open_access</pmc-dir>
<front><journal-meta><journal-id journal-id-type="nlm-ta">J Clin Exp Dent</journal-id><journal-id journal-id-type="iso-abbrev">J Clin Exp Dent</journal-id><journal-id journal-id-type="publisher-id">Medicina Oral S.L.</journal-id><journal-title-group><journal-title>Journal of Clinical and Experimental Dentistry</journal-title></journal-title-group><issn pub-type="epub">1989-5488</issn><publisher><publisher-name>Medicina Oral S.L.</publisher-name></publisher></journal-meta><article-meta><article-id pub-id-type="pmid">24558530</article-id><article-id pub-id-type="pmc">3908815</article-id><article-id pub-id-type="publisher-id">50594</article-id><article-id pub-id-type="doi">10.4317/jced.50594</article-id><article-categories><subj-group subj-group-type="heading"><subject>Case Report</subject><subj-group><subject>Odontostomatology for the Disabled or Special Patients</subject></subj-group></subj-group></article-categories><title-group><article-title>Papillon- Lefèvre Syndrome:
Report of a case and its management
</article-title></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><name><surname>Sachdeva</surname><given-names>Shabina</given-names></name><xref ref-type="aff" rid="A1">1</xref></contrib><contrib contrib-type="author"><name><surname>Kalra</surname><given-names>Namita</given-names></name><xref ref-type="aff" rid="A2">2</xref></contrib><contrib contrib-type="author"><name><surname>Kapoor</surname><given-names>Pranav</given-names></name><xref ref-type="aff" rid="A3">3</xref></contrib></contrib-group><aff id="A1"><label>1</label>MDS. Formerly: Senior Resident, Department of Dentistry, University College of Medical Sciences, New Delhi -110095. Presently: Assistant Professor, Faculty of Dentistry, Jamia Millia Islamia, New Delhi -110025</aff><aff id="A2"><label>2</label>MDS. Professor & Head, Department of Dentistry, University College of Medical Sciences, New Delhi-110095</aff><aff id="A3"><label>3</label>MDS. Formerly: Lecturer, Department of Dentistry, University College of Medical Sciences, New Delhi -110095. Presently: Assistant Professor, Faculty of Dentistry, Jamia Millia Islamia, New Delhi -110025</aff><author-notes><corresp> B-4/33, Azad Apartments
Aurobindo Marg
New Delhi-16
, E-mail: <email>drshabinasachdeva@yahoo.co.in</email></corresp></author-notes><pub-date pub-type="epub"><day>1</day><month>2</month><year>2012</year></pub-date><pub-date pub-type="collection"><month>2</month><year>2012</year></pub-date><volume>4</volume><issue>1</issue><fpage>e77</fpage><lpage>e81</lpage><history><date date-type="accepted"><day>17</day><month>11</month><year>2011</year></date><date date-type="received"><day>8</day><month>6</month><year>2011</year></date></history><permissions><copyright-statement>Copyright: © 2012 Medicina Oral S.L.</copyright-statement><copyright-year>2012</copyright-year><license license-type="open-access" xlink:href="http://creativecommons.org/licenses/by/2.5/"><license-p>This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.</license-p></license></permissions><abstract><p>Papillon-Lefèvre Syndrome (PLS) is a rare autosomal recessive disorder first described by two French physicians, Papillon and Lefèvre in 1924. The disorder is characterized by diffuse palmoplantar keratoderma and precocious aggressively progressing periodontitis, leading to the premature loss of deciduous and permanent teeth at a very young age. The cutaneous lesions are usually manifested simultaneously with the intra-oral presentations and include keratotic plaques on the palms and soles varying from mild psoriasiform scaly skin to overt hyperkeratosis. The etiopathogenesis of the syndrome is relatively obscure and immunologic, genetic or possible bacterial etiologies have been proposed. Due to the vast degree of periodontal breakdown involved at such an early age, the dental surgeon is often the first to diagnose the syndrome. This paper presents a clinical presentation a 15 year old male diagnosed with Papillon- Lefèvre Syndrome.
</p><p><bold> Key words:</bold>Papillon-Lefèvre Syndrome, palmoplantar keratoderma, rapidly progressing periodontitis.</p></abstract></article-meta></front></pmc><affiliations><list></list><tree><noCountry><name sortKey="Kalra, Namita" sort="Kalra, Namita" uniqKey="Kalra N" first="Namita" last="Kalra">Namita Kalra</name><name sortKey="Kapoor, Pranav" sort="Kapoor, Pranav" uniqKey="Kapoor P" first="Pranav" last="Kapoor">Pranav Kapoor</name><name sortKey="Sachdeva, Shabina" sort="Sachdeva, Shabina" uniqKey="Sachdeva S" first="Shabina" last="Sachdeva">Shabina Sachdeva</name></noCountry></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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