Papillon-Lèfevre syndrome with palmoplantar keratoderma and periodontitis, a rare cause of pyrexia of unknown origin: a case report
Identifieur interne : 000E54 ( Main/Exploration ); précédent : 000E53; suivant : 000E55Papillon-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.
Url:
DOI: 10.1186/s13256-015-0773-7
PubMed: 26683691
PubMed Central: 4684603
Affiliations:
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sortKey="Iqtadar, Somia" sort="Iqtadar, Somia" uniqKey="Iqtadar S" first="Somia" last="Iqtadar">Somia Iqtadar</name><affiliation wicri:level="1"><nlm:aff id="Aff1">North Medical Ward,Mayo Hospital, King Edward Medical University, Lahore, Pakistan</nlm:aff><country xml:lang="fr">Pakistan</country><wicri:regionArea>North Medical Ward,Mayo Hospital, King Edward Medical University, Lahore</wicri:regionArea><wicri:noRegion>Lahore</wicri:noRegion></affiliation></author><author><name sortKey="Mumtaz, Sami Ullah" sort="Mumtaz, Sami Ullah" uniqKey="Mumtaz S" first="Sami Ullah" last="Mumtaz">Sami Ullah Mumtaz</name><affiliation wicri:level="1"><nlm:aff id="Aff1">North Medical Ward,Mayo Hospital, King Edward Medical University, Lahore, Pakistan</nlm:aff><country xml:lang="fr">Pakistan</country><wicri:regionArea>North Medical Ward,Mayo Hospital, King Edward Medical University, Lahore</wicri:regionArea><wicri:noRegion>Lahore</wicri:noRegion></affiliation></author><author><name sortKey="Abaidullah, Sajid" sort="Abaidullah, Sajid" uniqKey="Abaidullah S" first="Sajid" last="Abaidullah">Sajid Abaidullah</name><affiliation wicri:level="1"><nlm:aff id="Aff1">North Medical Ward,Mayo Hospital, King Edward Medical University, Lahore, Pakistan</nlm:aff><country xml:lang="fr">Pakistan</country><wicri:regionArea>North Medical Ward,Mayo Hospital, King Edward Medical University, Lahore</wicri:regionArea><wicri:noRegion>Lahore</wicri:noRegion></affiliation></author></analytic><series><title level="j">Journal of Medical Case Reports</title><idno type="eISSN">1752-1947</idno><imprint><date when="2015">2015</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en"><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></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><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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