Papillon-Lèfevre syndrome with palmoplantar keratoderma and periodontitis, a rare cause of pyrexia of unknown origin: a case report
Identifieur interne : 006240 ( Ncbi/Merge ); précédent : 006239; suivant : 006241Papillon-Lèfevre syndrome with palmoplantar keratoderma and periodontitis, a rare cause of pyrexia of unknown origin: a case report
Auteurs : Somia Iqtadar [Pakistan] ; Sami Ullah Mumtaz [Pakistan] ; Sajid Abaidullah [Pakistan]Source :
- Journal of Medical Case Reports [ 1752-1947 ] ; 2015.
Abstract
Papillon-Lefèvre Syndrome is a rare autosomal recessive disorder characterized by diffuse, transgradient palmoplantar keratoderma, destructive periodontitis beginning in childhood, premature loss of primary teeth, and frequent cutaneous and systemic pyogenic infections. Pyogenic liver abscess is an uncommon presentation of the disease present in this case.
A 16-year-old Punjabi, Pakistani boy presented to the outpatient department of a tertiary-care hospital of Lahore with high-grade fever of 2 months duration. He had been treated for a pyogenic liver abscess 2 years back with antibiotics followed by incision and drainage. He had poor orodental hygiene, palmoplantar keratoderma and periodontitis. His parents had history of consanguinity. His brother and two cousins had similar skin lesions and were edentulous. An orthopentogram showed atrophy of the alveolar bone. He was treated with broad-spectrum antibiotics, and antipyretics for systemic infection. Multivitamins, topical steroids, topical keratolytics and emollients were used for his dermatological issues.
Our patient was successfully treated. His fever settled and his skin lesions improved with antibiotics, topical steroids and keratolytics. He was sent home and was asked to return for follow-up on a monthly basis.
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DOI: 10.1186/s13256-015-0773-7
PubMed: 26683691
PubMed Central: 4684603
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Pyogenic liver abscess is an uncommon presentation of the disease present in this case.</p></sec><sec><title>Case presentation</title><p>A 16-year-old Punjabi, Pakistani boy presented to the outpatient department of a tertiary-care hospital of Lahore with high-grade fever of 2 months duration. He had been treated for a pyogenic liver abscess 2 years back with antibiotics followed by incision and drainage. He had poor orodental hygiene, palmoplantar keratoderma and periodontitis. His parents had history of consanguinity. His brother and two cousins had similar skin lesions and were edentulous. An orthopentogram showed atrophy of the alveolar bone. He was treated with broad-spectrum antibiotics, and antipyretics for systemic infection. Multivitamins, topical steroids, topical keratolytics and emollients were used for his dermatological issues.</p></sec><sec><title>Conclusions</title><p>Our patient was successfully treated. His fever settled and his skin lesions improved with antibiotics, topical steroids and keratolytics. He was sent home and was asked to return for follow-up on a monthly basis.</p></sec></div></front><back><div1 type="bibliography"><listBibl><biblStruct><analytic><author><name sortKey="Krol, Al" uniqKey="Krol A">AL Krol</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Dhadke, S" uniqKey="Dhadke S">S Dhadke</name></author><author><name sortKey="Kulkarni, P" uniqKey="Kulkarni P">P Kulkarni</name></author><author><name sortKey="Dhadke, V" uniqKey="Dhadke V">V Dhadke</name></author><author><name sortKey="Deshpande, N" uniqKey="Deshpande N">N Deshpande</name></author><author><name sortKey="Wattamwar, P" uniqKey="Wattamwar P">P Wattamwar</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Hattab, Fn" uniqKey="Hattab F">FN Hattab</name></author><author><name sortKey="Rawashdeh, Ma" uniqKey="Rawashdeh M">MA Rawashdeh</name></author><author><name sortKey="Yassin, Om" uniqKey="Yassin O">OM Yassin</name></author><author><name sortKey="Al Momani, As" uniqKey="Al Momani A">AS Al-Momani</name></author><author><name sortKey="Al Ubosi, Mm" uniqKey="Al Ubosi M">MM Al-Ubosi</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Van Dyke, Te" uniqKey="Van Dyke T">TE Van Dyke</name></author><author><name sortKey="Taubman, Ma" uniqKey="Taubman M">MA Taubman</name></author><author><name sortKey="Ebersole, Jl" uniqKey="Ebersole J">JL Ebersole</name></author><author><name sortKey="Haffajee, Ad" uniqKey="Haffajee A">AD Haffajee</name></author><author><name sortKey="Socransky, Ss" uniqKey="Socransky S">SS Socransky</name></author><author><name sortKey="Smith, Dj" uniqKey="Smith D">DJ Smith</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Bergman, R" uniqKey="Bergman R">R Bergman</name></author><author><name sortKey="Friedman Irnbaum, R" uniqKey="Friedman Irnbaum R">R Friedman‐Birnbaum</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Khandpur, S" uniqKey="Khandpur S">S Khandpur</name></author><author><name sortKey="Reddy, Bsn" uniqKey="Reddy B">BSN Reddy</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Balci, Dd" uniqKey="Balci D">DD Balci</name></author><author><name sortKey="Serarslan, G" uniqKey="Serarslan G">G Serarslan</name></author><author><name sortKey="Sangun, O" uniqKey="Sangun O">O Sangun</name></author><author><name sortKey="Homan, S" uniqKey="Homan S">S Homan</name></author></analytic></biblStruct><biblStruct><analytic><author><name sortKey="Pilger, U" uniqKey="Pilger U">U Pilger</name></author><author><name sortKey="Hennies, Hc" uniqKey="Hennies H">HC Hennies</name></author><author><name sortKey="Truschnegg, A" uniqKey="Truschnegg A">A Truschnegg</name></author><author><name sortKey="Aberer, E" uniqKey="Aberer E">E Aberer</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 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">26683691</article-id><article-id pub-id-type="pmc">4684603</article-id><article-id pub-id-type="publisher-id">773</article-id><article-id pub-id-type="doi">10.1186/s13256-015-0773-7</article-id><article-categories><subj-group subj-group-type="heading"><subject>Case Report</subject></subj-group></article-categories><title-group><article-title>Papillon-Lèfevre syndrome with palmoplantar keratoderma and periodontitis, a rare cause of pyrexia of unknown origin: a case report</article-title></title-group><contrib-group><contrib contrib-type="author" corresp="yes"><name><surname>Iqtadar</surname><given-names>Somia</given-names></name><address><email>somia.iqtadar@gmail.com</email></address><xref ref-type="aff" rid="Aff1"></xref></contrib><contrib contrib-type="author"><name><surname>Mumtaz</surname><given-names>Sami Ullah</given-names></name><address><email>drsamiullah210@gmail.com</email></address><xref ref-type="aff" rid="Aff1"></xref></contrib><contrib contrib-type="author"><name><surname>Abaidullah</surname><given-names>Sajid</given-names></name><address><email>sajidabaidullahemw@hotmail.com</email></address><xref ref-type="aff" rid="Aff1"></xref></contrib><aff id="Aff1">North Medical Ward,Mayo Hospital, King Edward Medical University, Lahore, Pakistan</aff></contrib-group><pub-date pub-type="epub"><day>18</day><month>12</month><year>2015</year></pub-date><pub-date pub-type="pmc-release"><day>18</day><month>12</month><year>2015</year></pub-date><pub-date pub-type="collection"><year>2015</year></pub-date><volume>9</volume><elocation-id>288</elocation-id><history><date date-type="received"><day>15</day><month>5</month><year>2015</year></date><date date-type="accepted"><day>22</day><month>11</month><year>2015</year></date></history><permissions><copyright-statement>© Iqtadar et al. 2015</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>Introduction</title><p>Papillon-Lefèvre Syndrome is a rare autosomal recessive disorder characterized by diffuse, transgradient palmoplantar keratoderma, destructive periodontitis beginning in childhood, premature loss of primary teeth, and frequent cutaneous and systemic pyogenic infections. Pyogenic liver abscess is an uncommon presentation of the disease present in this case.</p></sec><sec><title>Case presentation</title><p>A 16-year-old Punjabi, Pakistani boy presented to the outpatient department of a tertiary-care hospital of Lahore with high-grade fever of 2 months duration. He had been treated for a pyogenic liver abscess 2 years back with antibiotics followed by incision and drainage. He had poor orodental hygiene, palmoplantar keratoderma and periodontitis. His parents had history of consanguinity. His brother and two cousins had similar skin lesions and were edentulous. An orthopentogram showed atrophy of the alveolar bone. He was treated with broad-spectrum antibiotics, and antipyretics for systemic infection. Multivitamins, topical steroids, topical keratolytics and emollients were used for his dermatological issues.</p></sec><sec><title>Conclusions</title><p>Our patient was successfully treated. His fever settled and his skin lesions improved with antibiotics, topical steroids and keratolytics. He was sent home and was asked to return for follow-up on a monthly basis.</p></sec></abstract><kwd-group xml:lang="en"><title>Keywords</title><kwd>Papillon-Lefèvre syndrome</kwd><kwd>Palmoplantar keratoderma</kwd><kwd>Periodontitis</kwd><kwd>Pyogenic liver abscess</kwd></kwd-group><custom-meta-group><custom-meta><meta-name>issue-copyright-statement</meta-name><meta-value>© The Author(s) 2015</meta-value></custom-meta></custom-meta-group></article-meta></front></pmc><affiliations><list><country><li>Pakistan</li></country></list><tree><country name="Pakistan"><noRegion><name sortKey="Iqtadar, Somia" sort="Iqtadar, Somia" uniqKey="Iqtadar S" first="Somia" last="Iqtadar">Somia Iqtadar</name></noRegion><name sortKey="Abaidullah, Sajid" sort="Abaidullah, Sajid" uniqKey="Abaidullah S" first="Sajid" last="Abaidullah">Sajid Abaidullah</name><name sortKey="Mumtaz, Sami Ullah" sort="Mumtaz, Sami Ullah" uniqKey="Mumtaz S" first="Sami Ullah" last="Mumtaz">Sami Ullah Mumtaz</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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