Papillon–Lefèvre syndrome: a series of five cases among siblings
Identifieur interne : 000982 ( Pmc/Curation ); précédent : 000981; suivant : 000983Papillon–Lefèvre syndrome: a series of five cases among siblings
Auteurs : Zyad M. Aibarrak [Arabie saoudite] ; Adel S. Alqarni ; Elna P. Chalisserry [Arabie saoudite] ; Sukumaran AnilSource :
- Journal of Medical Case Reports [ 1752-1947 ] ; 2016.
Abstract
Papillon–Lefèvre syndrome is a rare autosomal recessive disorder characterized by palmoplantar hyperkeratosis and aggressively progressing periodontitis leading to premature loss of deciduous and permanent dentition. The etiopathogenesis of the syndrome is relatively obscure, and immunologic, genetic, or possible bacterial etiologies have been proposed.
A series of five cases of Papillon–Lefèvre syndrome among the siblings in a family is presented here: a 3-year-old Arab girl, a 4-year-old Arab boy, a 11-year-old Arab boy, a 12-year-old Arab boy, and a 14-year-old Arab boy. The patients presented with severe gingival inflammation and mobility of teeth. The clinical manifestations were typical of Papillon–Lefèvre syndrome and the degree of involvement of the oral and skin conditions varied among them.
This case series stresses the consanguinity in the family as an etiologic factor. All siblings in the family were affected with Papillon–Lefèvre syndrome which makes this a rare case. A multidisciplinary approach with the active participation of a dental surgeon, dermatologist, and pediatrician is essential for the management of cases of Papillon–Lefèvre syndrome.
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DOI: 10.1186/s13256-016-1051-z
PubMed: 27658951
PubMed Central: 5034476
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Adel S. Alqarni<affiliation><nlm:aff id="Aff2">Department of Preventive Dental Sciences, College of Dentistry, Prince Sattam Bin Abdulaziz University, 153, AIkharj, 11942 Riyadh Saudi Arabia</nlm:aff><wicri:noCountry code="subfield">11942 Riyadh Saudi Arabia</wicri:noCountry></affiliation><affiliation><nlm:aff id="Aff2">Department of Preventive Dental Sciences, College of Dentistry, Prince Sattam Bin Abdulaziz University, 153, AIkharj, 11942 Riyadh Saudi Arabia</nlm:aff><wicri:noCountry code="subfield">11942 Riyadh Saudi Arabia</wicri:noCountry></affiliation>Le document en format XML
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Papillon–Lefèvre syndrome: a series of five cases among siblings</title><author><name sortKey="Aibarrak, Zyad M" sort="Aibarrak, Zyad M" uniqKey="Aibarrak Z" first="Zyad M." last="Aibarrak">Zyad M. Aibarrak</name><affiliation wicri:level="1"><nlm:aff id="Aff1">King Abdulaziz Medical City, Ministry of Health, Riyadh, Saudi Arabia</nlm:aff><country xml:lang="fr">Arabie saoudite</country><wicri:regionArea>King Abdulaziz Medical City, Ministry of Health, Riyadh</wicri:regionArea></affiliation></author><author><name sortKey="Alqarni, Adel S" sort="Alqarni, Adel S" uniqKey="Alqarni A" first="Adel S." last="Alqarni">Adel S. Alqarni</name><affiliation><nlm:aff id="Aff2">Department of Preventive Dental Sciences, College of Dentistry, Prince Sattam Bin Abdulaziz University, 153, AIkharj, 11942 Riyadh Saudi Arabia</nlm:aff><wicri:noCountry code="subfield">11942 Riyadh Saudi Arabia</wicri:noCountry></affiliation></author><author><name sortKey="Chalisserry, Elna P" sort="Chalisserry, Elna P" uniqKey="Chalisserry E" first="Elna P." last="Chalisserry">Elna P. Chalisserry</name><affiliation wicri:level="1"><nlm:aff id="Aff3">Department of Maxillofacial Surgery and Diagnostic Sciences, College of Dentistry, Jazan University, Jazan, 82943 Saudi Arabia</nlm:aff><country xml:lang="fr">Arabie saoudite</country><wicri:regionArea>Department of Maxillofacial Surgery and Diagnostic Sciences, College of Dentistry, Jazan University, Jazan</wicri:regionArea></affiliation></author><author><name sortKey="Anil, Sukumaran" sort="Anil, Sukumaran" uniqKey="Anil S" first="Sukumaran" last="Anil">Sukumaran Anil</name><affiliation><nlm:aff id="Aff2">Department of Preventive Dental Sciences, College of Dentistry, Prince Sattam Bin Abdulaziz University, 153, AIkharj, 11942 Riyadh Saudi Arabia</nlm:aff><wicri:noCountry code="subfield">11942 Riyadh Saudi Arabia</wicri:noCountry></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">PMC</idno><idno type="pmid">27658951</idno><idno type="pmc">5034476</idno><idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5034476</idno><idno type="RBID">PMC:5034476</idno><idno type="doi">10.1186/s13256-016-1051-z</idno><date when="2016">2016</date><idno type="wicri:Area/Pmc/Corpus">000982</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Corpus" wicri:corpus="PMC">000982</idno><idno type="wicri:Area/Pmc/Curation">000982</idno><idno type="wicri:explorRef" wicri:stream="Pmc" wicri:step="Curation">000982</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a" type="main">Papillon–Lefèvre syndrome: a series of five cases among siblings</title><author><name sortKey="Aibarrak, Zyad M" sort="Aibarrak, Zyad M" uniqKey="Aibarrak Z" first="Zyad M." last="Aibarrak">Zyad M. Aibarrak</name><affiliation wicri:level="1"><nlm:aff id="Aff1">King Abdulaziz Medical City, Ministry of Health, Riyadh, Saudi Arabia</nlm:aff><country xml:lang="fr">Arabie saoudite</country><wicri:regionArea>King Abdulaziz Medical City, Ministry of Health, Riyadh</wicri:regionArea></affiliation></author><author><name sortKey="Alqarni, Adel S" sort="Alqarni, Adel S" uniqKey="Alqarni A" first="Adel S." last="Alqarni">Adel S. Alqarni</name><affiliation><nlm:aff id="Aff2">Department of Preventive Dental Sciences, College of Dentistry, Prince Sattam Bin Abdulaziz University, 153, AIkharj, 11942 Riyadh Saudi Arabia</nlm:aff><wicri:noCountry code="subfield">11942 Riyadh Saudi Arabia</wicri:noCountry></affiliation></author><author><name sortKey="Chalisserry, Elna P" sort="Chalisserry, Elna P" uniqKey="Chalisserry E" first="Elna P." last="Chalisserry">Elna P. Chalisserry</name><affiliation wicri:level="1"><nlm:aff id="Aff3">Department of Maxillofacial Surgery and Diagnostic Sciences, College of Dentistry, Jazan University, Jazan, 82943 Saudi Arabia</nlm:aff><country xml:lang="fr">Arabie saoudite</country><wicri:regionArea>Department of Maxillofacial Surgery and Diagnostic Sciences, College of Dentistry, Jazan University, Jazan</wicri:regionArea></affiliation></author><author><name sortKey="Anil, Sukumaran" sort="Anil, Sukumaran" uniqKey="Anil S" first="Sukumaran" last="Anil">Sukumaran Anil</name><affiliation><nlm:aff id="Aff2">Department of Preventive Dental Sciences, College of Dentistry, Prince Sattam Bin Abdulaziz University, 153, AIkharj, 11942 Riyadh Saudi Arabia</nlm:aff><wicri:noCountry code="subfield">11942 Riyadh Saudi Arabia</wicri:noCountry></affiliation></author></analytic><series><title level="j">Journal of Medical Case Reports</title><idno type="eISSN">1752-1947</idno><imprint><date when="2016">2016</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><sec><title>Background</title><p>Papillon–Lefèvre syndrome is a rare autosomal recessive disorder characterized by palmoplantar hyperkeratosis and aggressively progressing periodontitis leading to premature loss of deciduous and permanent dentition. The etiopathogenesis of the syndrome is relatively obscure, and immunologic, genetic, or possible bacterial etiologies have been proposed.</p></sec><sec><title>Case presentation</title><p>A series of five cases of Papillon–Lefèvre syndrome among the siblings in a family is presented here: a 3-year-old Arab girl, a 4-year-old Arab boy, a 11-year-old Arab boy, a 12-year-old Arab boy, and a 14-year-old Arab boy. The patients presented with severe gingival inflammation and mobility of teeth. The clinical manifestations were typical of Papillon–Lefèvre syndrome and the degree of involvement of the oral and skin conditions varied among them.</p></sec><sec><title>Conclusions</title><p>This case series stresses the consanguinity in the family as an etiologic factor. All siblings in the family were affected with Papillon–Lefèvre syndrome which makes this a rare case. 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<front><journal-meta><journal-id journal-id-type="nlm-ta">J Med Case Rep</journal-id><journal-id journal-id-type="iso-abbrev">J Med Case Rep</journal-id><journal-title-group><journal-title>Journal of Medical Case Reports</journal-title></journal-title-group><issn pub-type="epub">1752-1947</issn><publisher><publisher-name>BioMed Central</publisher-name><publisher-loc>London</publisher-loc></publisher></journal-meta><article-meta><article-id pub-id-type="pmid">27658951</article-id><article-id pub-id-type="pmc">5034476</article-id><article-id pub-id-type="publisher-id">1051</article-id><article-id pub-id-type="doi">10.1186/s13256-016-1051-z</article-id><article-categories><subj-group subj-group-type="heading"><subject>Case Report</subject></subj-group></article-categories><title-group><article-title>Papillon–Lefèvre syndrome: a series of five cases among siblings</article-title></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><name><surname>AIBarrak</surname><given-names>Zyad M.</given-names></name><address><email>ksucod@gmail.com</email></address><xref ref-type="aff" rid="Aff1">1</xref></contrib><contrib contrib-type="author"><name><surname>Alqarni</surname><given-names>Adel S.</given-names></name><address><email>codriyadh@gmail.com</email></address><xref ref-type="aff" rid="Aff2">2</xref></contrib><contrib contrib-type="author"><name><surname>Chalisserry</surname><given-names>Elna P.</given-names></name><address><email>ceena1999@gmail.com</email></address><xref ref-type="aff" rid="Aff3">3</xref></contrib><contrib contrib-type="author"><name><surname>Anil</surname><given-names>Sukumaran</given-names></name><address><email>drsanil@gmail.com</email></address><xref ref-type="aff" rid="Aff2">2</xref></contrib><aff id="Aff1"><label>1</label>King Abdulaziz Medical City, Ministry of Health, Riyadh, Saudi Arabia</aff><aff id="Aff2"><label>2</label>Department of Preventive Dental Sciences, College of Dentistry, Prince Sattam Bin Abdulaziz University, 153, AIkharj, 11942 Riyadh Saudi Arabia</aff><aff id="Aff3"><label>3</label>Department of Maxillofacial Surgery and Diagnostic Sciences, College of Dentistry, Jazan University, Jazan, 82943 Saudi Arabia</aff></contrib-group><pub-date pub-type="epub"><day>22</day><month>9</month><year>2016</year></pub-date><pub-date pub-type="pmc-release"><day>22</day><month>9</month><year>2016</year></pub-date><pub-date pub-type="collection"><year>2016</year></pub-date><volume>10</volume><elocation-id>260</elocation-id><history><date date-type="received"><day>10</day><month>4</month><year>2016</year></date><date date-type="accepted"><day>1</day><month>9</month><year>2016</year></date></history><permissions><copyright-statement>© The Author(s). 2016</copyright-statement><license license-type="OpenAccess"><license-p><bold>Open Access</bold>This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (<ext-link ext-link-type="uri" xlink:href="http://creativecommons.org/licenses/by/4.0/">http://creativecommons.org/licenses/by/4.0/</ext-link>), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (<ext-link ext-link-type="uri" xlink:href="http://creativecommons.org/publicdomain/zero/1.0/">http://creativecommons.org/publicdomain/zero/1.0/</ext-link>) applies to the data made available in this article, unless otherwise stated.</license-p></license></permissions><abstract id="Abs1"><sec><title>Background</title><p>Papillon–Lefèvre syndrome is a rare autosomal recessive disorder characterized by palmoplantar hyperkeratosis and aggressively progressing periodontitis leading to premature loss of deciduous and permanent dentition. The etiopathogenesis of the syndrome is relatively obscure, and immunologic, genetic, or possible bacterial etiologies have been proposed.</p></sec><sec><title>Case presentation</title><p>A series of five cases of Papillon–Lefèvre syndrome among the siblings in a family is presented here: a 3-year-old Arab girl, a 4-year-old Arab boy, a 11-year-old Arab boy, a 12-year-old Arab boy, and a 14-year-old Arab boy. The patients presented with severe gingival inflammation and mobility of teeth. The clinical manifestations were typical of Papillon–Lefèvre syndrome and the degree of involvement of the oral and skin conditions varied among them.</p></sec><sec><title>Conclusions</title><p>This case series stresses the consanguinity in the family as an etiologic factor. All siblings in the family were affected with Papillon–Lefèvre syndrome which makes this a rare case. A multidisciplinary approach with the active participation of a dental surgeon, dermatologist, and pediatrician is essential for the management of cases of Papillon–Lefèvre syndrome.</p></sec></abstract><kwd-group xml:lang="en"><title>Keywords</title><kwd>Papillon–Lefèvre syndrome</kwd><kwd>Consanguinity</kwd><kwd>Periodontitis</kwd><kwd>Premature tooth loss</kwd><kwd>Cathepsin C</kwd><kwd>Gene mutation</kwd><kwd>Hyperkeratosis</kwd><kwd>Palmoplantar keratosis</kwd></kwd-group><custom-meta-group><custom-meta><meta-name>issue-copyright-statement</meta-name><meta-value>© The Author(s) 2016</meta-value></custom-meta></custom-meta-group></article-meta></front></pmc></record>Pour manipuler ce document sous Unix (Dilib)
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