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<title xml:lang="en">High-frequency (20–50 MHz) ultrasonography of pseudoxanthoma elasticum skin lesions</title>
<author>
<name sortKey="Guerin Moreau, M" sort="Guerin Moreau, M" uniqKey="Guerin Moreau M" first="M." last="Guérin-Moreau">M. Guérin-Moreau</name>
<affiliation>
<nlm:aff id="A1">L'UNAM University, Dept. of Dermatology, Angers Hospital, Angers, France</nlm:aff>
</affiliation>
<affiliation>
<nlm:aff id="A2">L'UNAM University, PXE Consultation Centre, Angers Hospital, Angers, France</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Leftheriotis, G" sort="Leftheriotis, G" uniqKey="Leftheriotis G" first="G." last="Leftheriotis">G. Leftheriotis</name>
<affiliation>
<nlm:aff id="A2">L'UNAM University, PXE Consultation Centre, Angers Hospital, Angers, France</nlm:aff>
</affiliation>
<affiliation>
<nlm:aff id="A3">L'UNAM University, Dept. of Vascular Medicine, Angers Hospital, Angers, France</nlm:aff>
</affiliation>
<affiliation>
<nlm:aff id="A4">L'UNAM University, Integrated Neurovascular and Mitochondrial Biology, INSERM 1083 / CNRS 6214, Angers School of Medicine, Angers, France</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Le Corre, Y" sort="Le Corre, Y" uniqKey="Le Corre Y" first="Y." last="Le Corre">Y. Le Corre</name>
<affiliation>
<nlm:aff id="A1">L'UNAM University, Dept. of Dermatology, Angers Hospital, Angers, France</nlm:aff>
</affiliation>
<affiliation>
<nlm:aff id="A2">L'UNAM University, PXE Consultation Centre, Angers Hospital, Angers, France</nlm:aff>
</affiliation>
<affiliation>
<nlm:aff id="A4">L'UNAM University, Integrated Neurovascular and Mitochondrial Biology, INSERM 1083 / CNRS 6214, Angers School of Medicine, Angers, France</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Etienne, M" sort="Etienne, M" uniqKey="Etienne M" first="M." last="Etienne">M. Etienne</name>
<affiliation>
<nlm:aff id="A1">L'UNAM University, Dept. of Dermatology, Angers Hospital, Angers, France</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Amode, R" sort="Amode, R" uniqKey="Amode R" first="R." last="Amode">R. Amode</name>
<affiliation>
<nlm:aff id="A1">L'UNAM University, Dept. of Dermatology, Angers Hospital, Angers, France</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Hamel, J F" sort="Hamel, J F" uniqKey="Hamel J" first="J. F." last="Hamel">J. F. Hamel</name>
<affiliation>
<nlm:aff id="A5">L'UNAM University, Clinical Research Centre, Angers Hospital, Angers, France</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Croue, A" sort="Croue, A" uniqKey="Croue A" first="A." last="Croué">A. Croué</name>
<affiliation>
<nlm:aff id="A2">L'UNAM University, PXE Consultation Centre, Angers Hospital, Angers, France</nlm:aff>
</affiliation>
<affiliation>
<nlm:aff id="A6">L'UNAM University, Dept. of Pathology, Angers Hospital, Angers, France</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Le Saux, O" sort="Le Saux, O" uniqKey="Le Saux O" first="O." last="Le Saux">O. Le Saux</name>
<affiliation>
<nlm:aff id="A7">John A. Burns School of Medicine, University of Hawai'i, Honolulu, HI, USA</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Machet, L" sort="Machet, L" uniqKey="Machet L" first="L." last="Machet">L. Machet</name>
<affiliation>
<nlm:aff id="A8">Inserm U930, François-Rabelais University, Tours, France</nlm:aff>
</affiliation>
<affiliation>
<nlm:aff id="A9">Dept. of Dermatology, Tours University Hospital, Tours, France</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Martin, L" sort="Martin, L" uniqKey="Martin L" first="L." last="Martin">L. Martin</name>
<affiliation>
<nlm:aff id="A1">L'UNAM University, Dept. of Dermatology, Angers Hospital, Angers, France</nlm:aff>
</affiliation>
<affiliation>
<nlm:aff id="A2">L'UNAM University, PXE Consultation Centre, Angers Hospital, Angers, France</nlm:aff>
</affiliation>
<affiliation>
<nlm:aff id="A4">L'UNAM University, Integrated Neurovascular and Mitochondrial Biology, INSERM 1083 / CNRS 6214, Angers School of Medicine, Angers, France</nlm:aff>
</affiliation>
</author>
</titleStmt>
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<idno type="pmid">23909384</idno>
<idno type="pmc">3855177</idno>
<idno type="url">http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3855177</idno>
<idno type="RBID">PMC:3855177</idno>
<idno type="doi">10.1111/bjd.12545</idno>
<date when="2013">2013</date>
<idno type="wicri:Area/Pmc/Corpus">003228</idno>
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<title xml:lang="en" level="a" type="main">High-frequency (20–50 MHz) ultrasonography of pseudoxanthoma elasticum skin lesions</title>
<author>
<name sortKey="Guerin Moreau, M" sort="Guerin Moreau, M" uniqKey="Guerin Moreau M" first="M." last="Guérin-Moreau">M. Guérin-Moreau</name>
<affiliation>
<nlm:aff id="A1">L'UNAM University, Dept. of Dermatology, Angers Hospital, Angers, France</nlm:aff>
</affiliation>
<affiliation>
<nlm:aff id="A2">L'UNAM University, PXE Consultation Centre, Angers Hospital, Angers, France</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Leftheriotis, G" sort="Leftheriotis, G" uniqKey="Leftheriotis G" first="G." last="Leftheriotis">G. Leftheriotis</name>
<affiliation>
<nlm:aff id="A2">L'UNAM University, PXE Consultation Centre, Angers Hospital, Angers, France</nlm:aff>
</affiliation>
<affiliation>
<nlm:aff id="A3">L'UNAM University, Dept. of Vascular Medicine, Angers Hospital, Angers, France</nlm:aff>
</affiliation>
<affiliation>
<nlm:aff id="A4">L'UNAM University, Integrated Neurovascular and Mitochondrial Biology, INSERM 1083 / CNRS 6214, Angers School of Medicine, Angers, France</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Le Corre, Y" sort="Le Corre, Y" uniqKey="Le Corre Y" first="Y." last="Le Corre">Y. Le Corre</name>
<affiliation>
<nlm:aff id="A1">L'UNAM University, Dept. of Dermatology, Angers Hospital, Angers, France</nlm:aff>
</affiliation>
<affiliation>
<nlm:aff id="A2">L'UNAM University, PXE Consultation Centre, Angers Hospital, Angers, France</nlm:aff>
</affiliation>
<affiliation>
<nlm:aff id="A4">L'UNAM University, Integrated Neurovascular and Mitochondrial Biology, INSERM 1083 / CNRS 6214, Angers School of Medicine, Angers, France</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Etienne, M" sort="Etienne, M" uniqKey="Etienne M" first="M." last="Etienne">M. Etienne</name>
<affiliation>
<nlm:aff id="A1">L'UNAM University, Dept. of Dermatology, Angers Hospital, Angers, France</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Amode, R" sort="Amode, R" uniqKey="Amode R" first="R." last="Amode">R. Amode</name>
<affiliation>
<nlm:aff id="A1">L'UNAM University, Dept. of Dermatology, Angers Hospital, Angers, France</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Hamel, J F" sort="Hamel, J F" uniqKey="Hamel J" first="J. F." last="Hamel">J. F. Hamel</name>
<affiliation>
<nlm:aff id="A5">L'UNAM University, Clinical Research Centre, Angers Hospital, Angers, France</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Croue, A" sort="Croue, A" uniqKey="Croue A" first="A." last="Croué">A. Croué</name>
<affiliation>
<nlm:aff id="A2">L'UNAM University, PXE Consultation Centre, Angers Hospital, Angers, France</nlm:aff>
</affiliation>
<affiliation>
<nlm:aff id="A6">L'UNAM University, Dept. of Pathology, Angers Hospital, Angers, France</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Le Saux, O" sort="Le Saux, O" uniqKey="Le Saux O" first="O." last="Le Saux">O. Le Saux</name>
<affiliation>
<nlm:aff id="A7">John A. Burns School of Medicine, University of Hawai'i, Honolulu, HI, USA</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Machet, L" sort="Machet, L" uniqKey="Machet L" first="L." last="Machet">L. Machet</name>
<affiliation>
<nlm:aff id="A8">Inserm U930, François-Rabelais University, Tours, France</nlm:aff>
</affiliation>
<affiliation>
<nlm:aff id="A9">Dept. of Dermatology, Tours University Hospital, Tours, France</nlm:aff>
</affiliation>
</author>
<author>
<name sortKey="Martin, L" sort="Martin, L" uniqKey="Martin L" first="L." last="Martin">L. Martin</name>
<affiliation>
<nlm:aff id="A1">L'UNAM University, Dept. of Dermatology, Angers Hospital, Angers, France</nlm:aff>
</affiliation>
<affiliation>
<nlm:aff id="A2">L'UNAM University, PXE Consultation Centre, Angers Hospital, Angers, France</nlm:aff>
</affiliation>
<affiliation>
<nlm:aff id="A4">L'UNAM University, Integrated Neurovascular and Mitochondrial Biology, INSERM 1083 / CNRS 6214, Angers School of Medicine, Angers, France</nlm:aff>
</affiliation>
</author>
</analytic>
<series>
<title level="j">The British journal of dermatology</title>
<idno type="ISSN">0007-0963</idno>
<idno type="eISSN">1365-2133</idno>
<imprint>
<date when="2013">2013</date>
</imprint>
</series>
</biblStruct>
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<textClass></textClass>
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</teiHeader>
<front>
<div type="abstract" xml:lang="en">
<title>Summary</title>
<sec id="S1">
<title>Background</title>
<p id="P1">In most patients pseudoxanthoma elasticum (PXE) manifests with yellowish cutaneous papules and dermal elastorrhexis on skin biopsy. However, in a small number of cases there are no skin manifestations on clinical examination. To establish a diagnosis of PXE in such patients with limited manifestations such as angioid streaks and/or premature cardiovascular disease is challenging. It would therefore be valuable to predict the skin areas that would yield a biopsy specimen positive for elastorrhexis with a non-invasive procedure. High frequency ultrasonography (HFUS) should be evaluated in this respect.</p>
</sec>
<sec id="S2">
<title>Objectives</title>
<p id="P2">Prior to achieve the goal mentioned above we aimed at describing the characteristics of clinically visible PXE skin using HFUS, and to evaluate its relevance for diagnosis.</p>
</sec>
<sec id="S3">
<title>Methods</title>
<p id="P3">HFUS was performed in a cohort of PXE patients and controls at a referral centre. HFUS images of PXE skin were compared to those of other conditions. Five operators were tasked with the blind scoring of multiple HFUS images of photoprotected or photoexposed skin from patients with PXE and controls. The diagnostic relevance indices (sensitivity, specificity, likelihood ratios, inter-observer agreement) were calculated.</p>
</sec>
<sec id="S4">
<title>Results</title>
<p id="P4">The HFUS changes considered as diagnostic for PXE were primarily oval homogeneous hypoechogenic areas in the middermis. The size of these areas closely matched the extent of the histological changes. The sensitivity and specificity of the diagnostic items and inter-observer agreement were high, particularly in photoprotected skin. Dermal hypoechogenicity in PXE could be related to high hydration of connective tissue due to the presence of glycosaminoglycans despite elastic fibre mineralization.</p>
</sec>
<sec id="S5">
<title>Conclusions</title>
<p id="P5">HFUS provides suggestive images of PXE skin lesions. HFUS should be now studied to determine if it is a potentially valuable technique for the non-invasive identification of elastorrhexis in PXE patients in whom skin involvement is clinically minimal or absent.</p>
</sec>
</div>
</front>
</TEI>
<pmc article-type="research-article">
<pmc-comment>The publisher of this article does not allow downloading of the full text in XML form.</pmc-comment>
<pmc-dir>properties manuscript</pmc-dir>
<front>
<journal-meta>
<journal-id journal-id-type="nlm-journal-id">0004041</journal-id>
<journal-id journal-id-type="pubmed-jr-id">1793</journal-id>
<journal-id journal-id-type="nlm-ta">Br J Dermatol</journal-id>
<journal-id journal-id-type="iso-abbrev">Br. J. Dermatol.</journal-id>
<journal-title-group>
<journal-title>The British journal of dermatology</journal-title>
</journal-title-group>
<issn pub-type="ppub">0007-0963</issn>
<issn pub-type="epub">1365-2133</issn>
</journal-meta>
<article-meta>
<article-id pub-id-type="pmid">23909384</article-id>
<article-id pub-id-type="pmc">3855177</article-id>
<article-id pub-id-type="doi">10.1111/bjd.12545</article-id>
<article-id pub-id-type="manuscript">NIHMS515260</article-id>
<article-categories>
<subj-group subj-group-type="heading">
<subject>Article</subject>
</subj-group>
</article-categories>
<title-group>
<article-title>High-frequency (20–50 MHz) ultrasonography of pseudoxanthoma elasticum skin lesions</article-title>
</title-group>
<contrib-group>
<contrib contrib-type="author">
<name>
<surname>Guérin-Moreau</surname>
<given-names>M.</given-names>
</name>
<xref ref-type="aff" rid="A1">1</xref>
<xref ref-type="aff" rid="A2">2</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Leftheriotis</surname>
<given-names>G.</given-names>
</name>
<xref ref-type="aff" rid="A2">2</xref>
<xref ref-type="aff" rid="A3">3</xref>
<xref ref-type="aff" rid="A4">4</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Le Corre</surname>
<given-names>Y.</given-names>
</name>
<xref ref-type="aff" rid="A1">1</xref>
<xref ref-type="aff" rid="A2">2</xref>
<xref ref-type="aff" rid="A4">4</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Etienne</surname>
<given-names>M.</given-names>
</name>
<xref ref-type="aff" rid="A1">1</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Amode</surname>
<given-names>R.</given-names>
</name>
<xref ref-type="aff" rid="A1">1</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Hamel</surname>
<given-names>J.F.</given-names>
</name>
<xref ref-type="aff" rid="A5">5</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Croué</surname>
<given-names>A.</given-names>
</name>
<xref ref-type="aff" rid="A2">2</xref>
<xref ref-type="aff" rid="A6">6</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Le Saux</surname>
<given-names>O.</given-names>
</name>
<xref ref-type="aff" rid="A7">7</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Machet</surname>
<given-names>L.</given-names>
</name>
<xref ref-type="aff" rid="A8">8</xref>
<xref ref-type="aff" rid="A9">9</xref>
</contrib>
<contrib contrib-type="author">
<name>
<surname>Martin</surname>
<given-names>L.</given-names>
</name>
<xref ref-type="aff" rid="A1">1</xref>
<xref ref-type="aff" rid="A2">2</xref>
<xref ref-type="aff" rid="A4">4</xref>
</contrib>
</contrib-group>
<aff id="A1">
<label>1</label>
L'UNAM University, Dept. of Dermatology, Angers Hospital, Angers, France</aff>
<aff id="A2">
<label>2</label>
L'UNAM University, PXE Consultation Centre, Angers Hospital, Angers, France</aff>
<aff id="A3">
<label>3</label>
L'UNAM University, Dept. of Vascular Medicine, Angers Hospital, Angers, France</aff>
<aff id="A4">
<label>4</label>
L'UNAM University, Integrated Neurovascular and Mitochondrial Biology, INSERM 1083 / CNRS 6214, Angers School of Medicine, Angers, France</aff>
<aff id="A5">
<label>5</label>
L'UNAM University, Clinical Research Centre, Angers Hospital, Angers, France</aff>
<aff id="A6">
<label>6</label>
L'UNAM University, Dept. of Pathology, Angers Hospital, Angers, France</aff>
<aff id="A7">
<label>7</label>
John A. Burns School of Medicine, University of Hawai'i, Honolulu, HI, USA</aff>
<aff id="A8">
<label>8</label>
Inserm U930, François-Rabelais University, Tours, France</aff>
<aff id="A9">
<label>9</label>
Dept. of Dermatology, Tours University Hospital, Tours, France</aff>
<author-notes>
<corresp id="CR1">
<bold>
<italic>Correspondence</italic>
</bold>
Prof. Ludovic Martin, MD, PhD Service de Dermatologie CHU d'Angers 4 rue Larrey F – 49933 Angers Cedex 9 France Tel. +33 2 41 35 34 19 Fax +33 2 41 35 54 54
<email>LuMartin@chu-angers.fr</email>
</corresp>
</author-notes>
<pub-date pub-type="nihms-submitted">
<day>18</day>
<month>9</month>
<year>2013</year>
</pub-date>
<pub-date pub-type="ppub">
<month>12</month>
<year>2013</year>
</pub-date>
<pub-date pub-type="pmc-release">
<day>01</day>
<month>12</month>
<year>2014</year>
</pub-date>
<volume>169</volume>
<issue>6</issue>
<elocation-id>10.1111/bjd.12545</elocation-id>
<abstract>
<title>Summary</title>
<sec id="S1">
<title>Background</title>
<p id="P1">In most patients pseudoxanthoma elasticum (PXE) manifests with yellowish cutaneous papules and dermal elastorrhexis on skin biopsy. However, in a small number of cases there are no skin manifestations on clinical examination. To establish a diagnosis of PXE in such patients with limited manifestations such as angioid streaks and/or premature cardiovascular disease is challenging. It would therefore be valuable to predict the skin areas that would yield a biopsy specimen positive for elastorrhexis with a non-invasive procedure. High frequency ultrasonography (HFUS) should be evaluated in this respect.</p>
</sec>
<sec id="S2">
<title>Objectives</title>
<p id="P2">Prior to achieve the goal mentioned above we aimed at describing the characteristics of clinically visible PXE skin using HFUS, and to evaluate its relevance for diagnosis.</p>
</sec>
<sec id="S3">
<title>Methods</title>
<p id="P3">HFUS was performed in a cohort of PXE patients and controls at a referral centre. HFUS images of PXE skin were compared to those of other conditions. Five operators were tasked with the blind scoring of multiple HFUS images of photoprotected or photoexposed skin from patients with PXE and controls. The diagnostic relevance indices (sensitivity, specificity, likelihood ratios, inter-observer agreement) were calculated.</p>
</sec>
<sec id="S4">
<title>Results</title>
<p id="P4">The HFUS changes considered as diagnostic for PXE were primarily oval homogeneous hypoechogenic areas in the middermis. The size of these areas closely matched the extent of the histological changes. The sensitivity and specificity of the diagnostic items and inter-observer agreement were high, particularly in photoprotected skin. Dermal hypoechogenicity in PXE could be related to high hydration of connective tissue due to the presence of glycosaminoglycans despite elastic fibre mineralization.</p>
</sec>
<sec id="S5">
<title>Conclusions</title>
<p id="P5">HFUS provides suggestive images of PXE skin lesions. HFUS should be now studied to determine if it is a potentially valuable technique for the non-invasive identification of elastorrhexis in PXE patients in whom skin involvement is clinically minimal or absent.</p>
</sec>
</abstract>
<kwd-group>
<kwd>Pseudoxanthoma elasticum</kwd>
<kwd>skin</kwd>
<kwd>ultrasonography</kwd>
<kwd>diagnostic test</kwd>
<kwd>elastic fibresl glycosaminoglycans</kwd>
</kwd-group>
<funding-group>
<award-group>
<funding-source country="United States">National Heart, Lung, and Blood Institute : NHLBI</funding-source>
<award-id>R01 HL108249 || HL</award-id>
</award-group>
</funding-group>
</article-meta>
</front>
</pmc>
</record>

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