Clinically amyopathic dermatomyositis: clinical features, response to medications and malignancy‐associated risk factors in a specific tertiary‐care‐centre cohort
Identifieur interne : 000A75 ( Istex/Corpus ); précédent : 000A74; suivant : 000A76Clinically amyopathic dermatomyositis: clinical features, response to medications and malignancy‐associated risk factors in a specific tertiary‐care‐centre cohort
Auteurs : F. Galimberti ; Y. Li ; A. P. FernandezSource :
- British Journal of Dermatology [ 0007-0963 ] ; 2016-01.
Abstract
Background: Clinically amyopathic dermatomyositis (CADM) is a subset of dermatomyositis (DM) characterized by the typical DM cutaneous manifestations but without myositis. There is a relative paucity of characterized cases of CADM in the peer‐reviewed medical literature. Objectives: To characterize the clinical features, response to medications and malignancy‐associated risk factors of patients with CADM with available baseline data seen at a single tertiary‐care centre. Methods: A retrospective review was undertaken of 44 patients with CADM with available clinical and serological data prior to onset of treatment. Results: Patients with CADM comprised 18% of all patients with DM with baseline data available at our institution. Although the majority of patients showed improvement with the first prescribed treatment, most required additional medications to control their CADM. Six of 44 patients had an associated malignancy. Photosensitivity and periungual erythema were found to be associated with absence of malignancy (P = 0·03 and P = 0·02, respectively). Patients with malignancy‐associated CADM were found to be more likely to have a cutaneous response with the first prescribed treatment than patients without malignancy (P = 0·04). Conclusions: CADM represents a significant subset of the DM population. As with classic DM, the cutaneous manifestations of CADM often represent a therapeutic challenge. A subset of patients with CADM has underlying malignancies, and these may differ from those typically associated with classic DM. Differences in serological abnormalities, cutaneous manifestations and response to first treatment among patients with CADM with and without malignancy were found, and suggest distinct pathophysiologies among CADM subsets. Characterization of this cohort expands the knowledge about this unique DM subset.
What's already known about this topic? Clinically amyopathic dermatomyositis (CADM) comprises a significant subset of patients with dermatomyositis (DM). Like classic DM, CADM presents with similar cutaneous manifestations and may be associated with lung disease or malignancy. What does this study add? Although patients with CADM may initially respond to conservative therapy, most eventually require more aggressive treatment. Malignancies and clinical features found in patients with malignancy‐associated (MA)‐CADM may differ from those in MA classic DM. Clinical, serological and treatment response differences between patients with idiopathic and MA‐CADM suggest distinct pathophysiologies.
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DOI: 10.1111/bjd.14227
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<record><TEI wicri:istexFullTextTei="biblStruct"><teiHeader><fileDesc><titleStmt><title xml:lang="en">Clinically amyopathic dermatomyositis: clinical features, response to medications and malignancy‐associated risk factors in a specific tertiary‐care‐centre cohort</title><author><name sortKey="Galimberti, F" sort="Galimberti, F" uniqKey="Galimberti F" first="F." last="Galimberti">F. Galimberti</name><affiliation><mods:affiliation>Cleveland Clinic Lerner College of Medicine, OH, Cleveland, U.S.A.</mods:affiliation></affiliation></author><author><name sortKey="Li, Y" sort="Li, Y" uniqKey="Li Y" first="Y." last="Li">Y. Li</name><affiliation><mods:affiliation>Cleveland Clinic Lerner College of Medicine, OH, Cleveland, U.S.A.</mods:affiliation></affiliation></author><author><name sortKey="Fernandez, A P" sort="Fernandez, A P" uniqKey="Fernandez A" first="A. P." last="Fernandez">A. P. Fernandez</name><affiliation><mods:affiliation>Departments of Dermatology and Pathology, Cleveland Clinic, 9500 Euclid Avenue, OH, A61, Cleveland, U.S.A.</mods:affiliation></affiliation><affiliation><mods:affiliation>.:</mods:affiliation></affiliation><affiliation><mods:affiliation>E-mail: fernana6@ccf.org</mods:affiliation></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">ISTEX</idno><idno type="RBID">ISTEX:F59145BD86FA0D2D387E684876A83B82FD32A7A6</idno><date when="2016" year="2016">2016</date><idno type="doi">10.1111/bjd.14227</idno><idno type="url">https://api.istex.fr/ark:/67375/WNG-72QTZS9Z-3/fulltext.pdf</idno><idno type="wicri:Area/Istex/Corpus">000A75</idno><idno type="wicri:explorRef" wicri:stream="Istex" wicri:step="Corpus" wicri:corpus="ISTEX">000A75</idno></publicationStmt><sourceDesc><biblStruct><analytic><title level="a" type="main">Clinically amyopathic dermatomyositis: clinical features, response to medications and malignancy‐associated risk factors in a specific tertiary‐care‐centre cohort</title><author><name sortKey="Galimberti, F" sort="Galimberti, F" uniqKey="Galimberti F" first="F." last="Galimberti">F. Galimberti</name><affiliation><mods:affiliation>Cleveland Clinic Lerner College of Medicine, OH, Cleveland, U.S.A.</mods:affiliation></affiliation></author><author><name sortKey="Li, Y" sort="Li, Y" uniqKey="Li Y" first="Y." last="Li">Y. Li</name><affiliation><mods:affiliation>Cleveland Clinic Lerner College of Medicine, OH, Cleveland, U.S.A.</mods:affiliation></affiliation></author><author><name sortKey="Fernandez, A P" sort="Fernandez, A P" uniqKey="Fernandez A" first="A. P." last="Fernandez">A. P. Fernandez</name><affiliation><mods:affiliation>Departments of Dermatology and Pathology, Cleveland Clinic, 9500 Euclid Avenue, OH, A61, Cleveland, U.S.A.</mods:affiliation></affiliation><affiliation><mods:affiliation>.:</mods:affiliation></affiliation><affiliation><mods:affiliation>E-mail: fernana6@ccf.org</mods:affiliation></affiliation></author></analytic><monogr></monogr><series><title level="j" type="main">British Journal of Dermatology</title><title level="j" type="alt">BRITISH JOURNAL OF DERMATOLOGY</title><idno type="ISSN">0007-0963</idno><idno type="eISSN">1365-2133</idno><imprint><biblScope unit="vol">174</biblScope><biblScope unit="issue">1</biblScope><biblScope unit="page" from="158">158</biblScope><biblScope unit="page" to="164">164</biblScope><biblScope unit="page-count">7</biblScope><date type="published" when="2016-01">2016-01</date></imprint><idno type="ISSN">0007-0963</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0007-0963</idno></seriesStmt></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract">Background: Clinically amyopathic dermatomyositis (CADM) is a subset of dermatomyositis (DM) characterized by the typical DM cutaneous manifestations but without myositis. There is a relative paucity of characterized cases of CADM in the peer‐reviewed medical literature. Objectives: To characterize the clinical features, response to medications and malignancy‐associated risk factors of patients with CADM with available baseline data seen at a single tertiary‐care centre. Methods: A retrospective review was undertaken of 44 patients with CADM with available clinical and serological data prior to onset of treatment. Results: Patients with CADM comprised 18% of all patients with DM with baseline data available at our institution. Although the majority of patients showed improvement with the first prescribed treatment, most required additional medications to control their CADM. Six of 44 patients had an associated malignancy. Photosensitivity and periungual erythema were found to be associated with absence of malignancy (P = 0·03 and P = 0·02, respectively). Patients with malignancy‐associated CADM were found to be more likely to have a cutaneous response with the first prescribed treatment than patients without malignancy (P = 0·04). Conclusions: CADM represents a significant subset of the DM population. As with classic DM, the cutaneous manifestations of CADM often represent a therapeutic challenge. A subset of patients with CADM has underlying malignancies, and these may differ from those typically associated with classic DM. Differences in serological abnormalities, cutaneous manifestations and response to first treatment among patients with CADM with and without malignancy were found, and suggest distinct pathophysiologies among CADM subsets. Characterization of this cohort expands the knowledge about this unique DM subset.</div><div type="abstract" xml:lang="en">What's already known about this topic? Clinically amyopathic dermatomyositis (CADM) comprises a significant subset of patients with dermatomyositis (DM). Like classic DM, CADM presents with similar cutaneous manifestations and may be associated with lung disease or malignancy. What does this study add? Although patients with CADM may initially respond to conservative therapy, most eventually require more aggressive treatment. Malignancies and clinical features found in patients with malignancy‐associated (MA)‐CADM may differ from those in MA classic DM. Clinical, serological and treatment response differences between patients with idiopathic and MA‐CADM suggest distinct pathophysiologies.</div></front></TEI><istex><corpusName>wiley</corpusName><author><json:item><name>F. Galimberti</name><affiliations><json:string>Cleveland Clinic Lerner College of Medicine, OH, Cleveland, U.S.A.</json:string></affiliations></json:item><json:item><name>Y. Li</name><affiliations><json:string>Cleveland Clinic Lerner College of Medicine, OH, Cleveland, U.S.A.</json:string></affiliations></json:item><json:item><name>A.P. Fernandez</name><affiliations><json:string>Departments of Dermatology and Pathology, Cleveland Clinic, 9500 Euclid Avenue, OH, A61, Cleveland, U.S.A.</json:string><json:string>.:</json:string><json:string>E-mail: fernana6@ccf.org</json:string></affiliations></json:item></author><articleId><json:string>BJD14227</json:string></articleId><arkIstex>ark:/67375/WNG-72QTZS9Z-3</arkIstex><language><json:string>eng</json:string></language><originalGenre><json:string>article</json:string></originalGenre><abstract>What's already known about this topic? Clinically amyopathic dermatomyositis (CADM) comprises a significant subset of patients with dermatomyositis (DM). Like classic DM, CADM presents with similar cutaneous manifestations and may be associated with lung disease or malignancy. What does this study add? Although patients with CADM may initially respond to conservative therapy, most eventually require more aggressive treatment. Malignancies and clinical features found in patients with malignancy‐associated (MA)‐CADM may differ from those in MA classic DM. 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<address><settlement>Cleveland</settlement>
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<country key="US" xml:lang="en">UNITED STATES</country></address></affiliation></author><author xml:id="author-0001"><persName><forename type="first">Y.</forename><surname>Li</surname></persName><affiliation><orgName type="institution">Cleveland Clinic Lerner College of Medicine</orgName>
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<country key="US" xml:lang="en">UNITED STATES</country></address></affiliation></author><author xml:id="author-0002" role="corresp"><persName><forename type="first">A.P.</forename><surname>Fernandez</surname></persName><affiliation><orgName type="division">Departments of Dermatology and Pathology</orgName>
<orgName type="institution">Cleveland Clinic</orgName>
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<ref target="http://www.tei-c.org/">We use TEI</ref></desc></application></appInfo></encodingDesc><profileDesc><abstract style="main" xml:id="bjd14227-abs-0001"><head>Summary</head><head>Background</head><p>Clinically amyopathic dermatomyositis (<hi rend="fc">CADM</hi>) is a subset of dermatomyositis (<hi rend="fc">DM</hi>) characterized by the typical <hi rend="fc">DM</hi> cutaneous manifestations but without myositis. There is a relative paucity of characterized cases of <hi rend="fc">CADM</hi> in the peer‐reviewed medical literature.</p><head>Objectives</head><p>To characterize the clinical features, response to medications and malignancy‐associated risk factors of patients with <hi rend="fc">CADM</hi> with available baseline data seen at a single tertiary‐care centre.</p><head>Methods</head><p>A retrospective review was undertaken of 44 patients with <hi rend="fc">CADM</hi> with available clinical and serological data prior to onset of treatment.</p><head>Results</head><p>Patients with <hi rend="fc">CADM</hi> comprised 18% of all patients with <hi rend="fc">DM</hi> with baseline data available at our institution. Although the majority of patients showed improvement with the first prescribed treatment, most required additional medications to control their <hi rend="fc">CADM</hi>. Six of 44 patients had an associated malignancy. Photosensitivity and periungual erythema were found to be associated with absence of malignancy (<hi rend="italic">P</hi> = 0·03 and <hi rend="italic">P</hi> = 0·02, respectively). Patients with malignancy‐associated <hi rend="fc">CADM</hi> were found to be more likely to have a cutaneous response with the first prescribed treatment than patients without malignancy (<hi rend="italic">P</hi> = 0·04).</p><head>Conclusions</head><p><hi rend="fc">CADM</hi> represents a significant subset of the <hi rend="fc">DM</hi> population. As with classic <hi rend="fc">DM</hi>, the cutaneous manifestations of <hi rend="fc">CADM</hi> often represent a therapeutic challenge. A subset of patients with <hi rend="fc">CADM</hi> has underlying malignancies, and these may differ from those typically associated with classic <hi rend="fc">DM</hi>. Differences in serological abnormalities, cutaneous manifestations and response to first treatment among patients with <hi rend="fc">CADM</hi> with and without malignancy were found, and suggest distinct pathophysiologies among <hi rend="fc">CADM</hi> subsets. Characterization of this cohort expands the knowledge about this unique <hi rend="fc">DM</hi> subset.</p></abstract><abstract xml:lang="en" style="short" xml:id="bjd14227-abs-0002"><p><hi rend="bold">What's already known about this topic?</hi></p><p><list xml:id="bjd14227-list-0001" style="bulleted"><item>Clinically amyopathic dermatomyositis (<hi rend="fc">CADM</hi>) comprises a significant subset of patients with dermatomyositis (<hi rend="fc">DM</hi>).</item><item>Like classic <hi rend="fc">DM</hi>,<hi rend="fc"> CADM</hi> presents with similar cutaneous manifestations and may be associated with lung disease or malignancy.</item></list></p><p><hi rend="bold">What does this study add?</hi></p><p><list xml:id="bjd14227-list-0002" style="bulleted"><item>Although patients with <hi rend="fc">CADM</hi> may initially respond to conservative therapy, most eventually require more aggressive treatment.</item><item>Malignancies and clinical features found in patients with malignancy‐associated (<hi rend="fc">MA</hi>)‐<hi rend="fc">CADM</hi> may differ from those in <hi rend="fc">MA</hi> classic <hi rend="fc">DM</hi>.</item><item>Clinical, serological and treatment response differences between patients with idiopathic and <hi rend="fc">MA</hi>‐<hi rend="fc">CADM</hi> suggest distinct pathophysiologies.</item></list></p></abstract><textClass><keywords rend="articleCategory"><term>Medical Dermatology</term></keywords><keywords rend="tocHeading1"><term>Original Articles</term></keywords><keywords rend="tocHeading2"><term>MEDICAL DERMATOLOGY</term></keywords></textClass><langUsage><language ident="en"></language></langUsage></profileDesc><revisionDesc><change when="2019-12-20" who="#istex" xml:id="pub2tei">formatting</change></revisionDesc></teiHeader></istex:fulltextTEI><json:item><extension>txt</extension><original>false</original><mimetype>text/plain</mimetype><uri>https://api.istex.fr/ark:/67375/WNG-72QTZS9Z-3/fulltext.txt</uri></json:item></fulltext><metadata><istex:metadataXml wicri:clean="Wiley, elements deleted: body"><istex:xmlDeclaration>version="1.0" encoding="UTF-8" standalone="yes"</istex:xmlDeclaration><istex:document><component type="serialArticle" version="2.0" xml:id="bjd14227" xml:lang="en"><header><publicationMeta level="product"><doi origin="wiley" registered="yes">10.1111/(ISSN)1365-2133</doi><issn type="print">0007-0963</issn><issn type="electronic">1365-2133</issn><idGroup><id type="product" value="BJD"></id></idGroup><titleGroup><title sort="BRITISH JOURNAL OF DERMATOLOGY" type="main">British Journal of Dermatology</title><title type="short">Br J Dermatol</title></titleGroup></publicationMeta><publicationMeta level="part" position="10"><doi origin="wiley">10.1111/bjd.2016.174.issue-1</doi><copyright ownership="thirdParty">Copyright © 2016 British Association of Dermatologists</copyright><numberingGroup><numbering type="journalVolume" number="174">174</numbering><numbering type="journalIssue">1</numbering></numberingGroup><coverDate startDate="2016-01">January 2016</coverDate></publicationMeta><publicationMeta level="unit" position="250" status="forIssue" type="article"><doi>10.1111/bjd.14227</doi><idGroup><id type="unit" value="BJD14227"></id></idGroup><countGroup><count number="7" type="pageTotal"></count></countGroup><titleGroup><title type="articleCategory">Medical Dermatology</title><title type="tocHeading1">Original Articles</title><title type="tocHeading2">MEDICAL DERMATOLOGY</title></titleGroup><copyright ownership="thirdParty">© 2015 British Association of Dermatologists</copyright><eventGroup><event date="2015-09-28" type="manuscriptAccepted"></event><event agent="SPS" date="2015-11-04" type="xmlCreated"></event><event type="publishedOnlineAccepted" date="2015-10-22"></event><event type="publishedOnlineEarlyUnpaginated" date="2015-12-01"></event><event type="firstOnline" date="2015-12-01"></event><event type="publishedOnlineFinalForm" date="2016-01-21"></event><event type="xmlConverted" agent="Converter:WML3G_To_WML3G version:5.0.6 mode:FullText" date="2017-02-09"></event></eventGroup><numberingGroup><numbering type="pageFirst">158</numbering><numbering type="pageLast">164</numbering></numberingGroup><correspondenceTo><lineatedText><line><b>Correspondence</b></line><line><i>Anthony P. Fernandez</i>.</line><line><i>E‐mail</i>: <email>fernana6@ccf.org</email></line></lineatedText></correspondenceTo><linkGroup><link type="toTypesetVersion" href="file:BJD.BJD14227.pdf"></link></linkGroup></publicationMeta><contentMeta><titleGroup><title type="main">Clinically amyopathic dermatomyositis: clinical features, response to medications and malignancy‐associated risk factors in a specific tertiary‐care‐centre cohort</title><title type="shortAuthors">F. Galimberti <i>et al</i>.</title></titleGroup><creators><creator affiliationRef="#bjd14227-aff-0001" creatorRole="author" xml:id="bjd14227-cr-0001"><personName><givenNames>F.</givenNames> <familyName>Galimberti</familyName></personName></creator><creator affiliationRef="#bjd14227-aff-0001" creatorRole="author" xml:id="bjd14227-cr-0002"><personName><givenNames>Y.</givenNames> <familyName>Li</familyName></personName></creator><creator affiliationRef="#bjd14227-aff-0002" corresponding="yes" creatorRole="author" xml:id="bjd14227-cr-0003"><personName><givenNames>A.P.</givenNames> <familyName>Fernandez</familyName></personName></creator></creators><affiliationGroup><affiliation countryCode="US" type="organization" xml:id="bjd14227-aff-0001"><orgName>Cleveland Clinic Lerner College of Medicine</orgName> <address><city>Cleveland</city> <countryPart>OH</countryPart> <country>U.S.A.</country></address></affiliation><affiliation countryCode="US" type="organization" xml:id="bjd14227-aff-0002"><orgDiv>Departments of Dermatology and Pathology</orgDiv> <orgName>Cleveland Clinic</orgName> <address><street>9500 Euclid Avenue</street> <postCode>A61</postCode> <city>Cleveland</city> <countryPart>OH</countryPart> <country>U.S.A.</country></address></affiliation></affiliationGroup><abstractGroup><abstract type="main" xml:id="bjd14227-abs-0001"><title type="main">Summary</title><section xml:id="bjd14227-sec-0001"><title type="main">Background</title><p>Clinically amyopathic dermatomyositis (<fc>CADM</fc>) is a subset of dermatomyositis (<fc>DM</fc>) characterized by the typical <fc>DM</fc> cutaneous manifestations but without myositis. There is a relative paucity of characterized cases of <fc>CADM</fc> in the peer‐reviewed medical literature.</p></section><section xml:id="bjd14227-sec-0002"><title type="main">Objectives</title><p>To characterize the clinical features, response to medications and malignancy‐associated risk factors of patients with <fc>CADM</fc> with available baseline data seen at a single tertiary‐care centre.</p></section><section xml:id="bjd14227-sec-0003"><title type="main">Methods</title><p>A retrospective review was undertaken of 44 patients with <fc>CADM</fc> with available clinical and serological data prior to onset of treatment.</p></section><section xml:id="bjd14227-sec-0004"><title type="main">Results</title><p>Patients with <fc>CADM</fc> comprised 18% of all patients with <fc>DM</fc> with baseline data available at our institution. Although the majority of patients showed improvement with the first prescribed treatment, most required additional medications to control their <fc>CADM</fc>. Six of 44 patients had an associated malignancy. Photosensitivity and periungual erythema were found to be associated with absence of malignancy (<i>P</i> = 0·03 and <i>P</i> = 0·02, respectively). Patients with malignancy‐associated <fc>CADM</fc> were found to be more likely to have a cutaneous response with the first prescribed treatment than patients without malignancy (<i>P</i> = 0·04).</p></section><section xml:id="bjd14227-sec-0005"><title type="main">Conclusions</title><p><fc>CADM</fc> represents a significant subset of the <fc>DM</fc> population. As with classic <fc>DM</fc>, the cutaneous manifestations of <fc>CADM</fc> often represent a therapeutic challenge. A subset of patients with <fc>CADM</fc> has underlying malignancies, and these may differ from those typically associated with classic <fc>DM</fc>. Differences in serological abnormalities, cutaneous manifestations and response to first treatment among patients with <fc>CADM</fc> with and without malignancy were found, and suggest distinct pathophysiologies among <fc>CADM</fc> subsets. Characterization of this cohort expands the knowledge about this unique <fc>DM</fc> subset.</p></section></abstract><abstract type="short" xml:id="bjd14227-abs-0002" xml:lang="en"><p><b>What's already known about this topic?</b></p><p><list formatted="paragraph" style="bulleted" xml:id="bjd14227-list-0001"><listItem>Clinically amyopathic dermatomyositis (<fc>CADM</fc>) comprises a significant subset of patients with dermatomyositis (<fc>DM</fc>).</listItem><listItem>Like classic <fc>DM</fc>,<fc> CADM</fc> presents with similar cutaneous manifestations and may be associated with lung disease or malignancy.</listItem></list></p><p><b>What does this study add?</b></p><p><list formatted="paragraph" style="bulleted" xml:id="bjd14227-list-0002"><listItem>Although patients with <fc>CADM</fc> may initially respond to conservative therapy, most eventually require more aggressive treatment.</listItem><listItem>Malignancies and clinical features found in patients with malignancy‐associated (<fc>MA</fc>)‐<fc>CADM</fc> may differ from those in <fc>MA</fc> classic <fc>DM</fc>.</listItem><listItem>Clinical, serological and treatment response differences between patients with idiopathic and <fc>MA</fc>‐<fc>CADM</fc> suggest distinct pathophysiologies.</listItem></list></p></abstract></abstractGroup></contentMeta><noteGroup xml:id="bjd14227-ntgp-0001"><note numbered="no" xml:id="bjd14227-note-0001"><b>Funding sources:</b> None.</note><note numbered="no" xml:id="bjd14227-note-0002"><b>Conflicts of interest:</b> A.P.F. is a consultant for Amgen and Castle Biosciences, and a consultant and speaker for AbbVie.</note></noteGroup></header></component></istex:document></istex:metadataXml><mods version="3.6"><titleInfo lang="en"><title>Clinically amyopathic dermatomyositis: clinical features, response to medications and malignancy‐associated risk factors in a specific tertiary‐care‐centre cohort</title></titleInfo><titleInfo type="alternative" contentType="CDATA" lang="en"><title>Clinically amyopathic dermatomyositis: clinical features, response to medications and malignancy‐associated risk factors in a specific tertiary‐care‐centre cohort</title></titleInfo><name type="personal"><namePart type="given">F.</namePart><namePart type="family">Galimberti</namePart><affiliation>Cleveland Clinic Lerner College of Medicine, OH, Cleveland, U.S.A.</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Y.</namePart><namePart type="family">Li</namePart><affiliation>Cleveland Clinic Lerner College of Medicine, OH, Cleveland, U.S.A.</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">A.P.</namePart><namePart type="family">Fernandez</namePart><affiliation>Departments of Dermatology and Pathology, Cleveland Clinic, 9500 Euclid Avenue, OH, A61, Cleveland, U.S.A.</affiliation><affiliation>.:</affiliation><affiliation>E-mail: fernana6@ccf.org</affiliation><role><roleTerm type="text">author</roleTerm></role></name><typeOfResource>text</typeOfResource><genre type="article" displayLabel="article" authority="ISTEX" authorityURI="https://content-type.data.istex.fr" valueURI="https://content-type.data.istex.fr/ark:/67375/XTP-6N5SZHKN-D">article</genre><originInfo><publisher>Blackwell Publishing Ltd</publisher><dateIssued encoding="w3cdtf">2016-01</dateIssued><dateCreated encoding="w3cdtf">2015-11-04</dateCreated><dateValid encoding="w3cdtf">2015-09-28</dateValid><copyrightDate encoding="w3cdtf">2016</copyrightDate></originInfo><language><languageTerm type="code" authority="rfc3066">en</languageTerm><languageTerm type="code" authority="iso639-2b">eng</languageTerm></language><abstract>Background: Clinically amyopathic dermatomyositis (CADM) is a subset of dermatomyositis (DM) characterized by the typical DM cutaneous manifestations but without myositis. There is a relative paucity of characterized cases of CADM in the peer‐reviewed medical literature. Objectives: To characterize the clinical features, response to medications and malignancy‐associated risk factors of patients with CADM with available baseline data seen at a single tertiary‐care centre. Methods: A retrospective review was undertaken of 44 patients with CADM with available clinical and serological data prior to onset of treatment. Results: Patients with CADM comprised 18% of all patients with DM with baseline data available at our institution. Although the majority of patients showed improvement with the first prescribed treatment, most required additional medications to control their CADM. Six of 44 patients had an associated malignancy. Photosensitivity and periungual erythema were found to be associated with absence of malignancy (P = 0·03 and P = 0·02, respectively). Patients with malignancy‐associated CADM were found to be more likely to have a cutaneous response with the first prescribed treatment than patients without malignancy (P = 0·04). Conclusions: CADM represents a significant subset of the DM population. As with classic DM, the cutaneous manifestations of CADM often represent a therapeutic challenge. A subset of patients with CADM has underlying malignancies, and these may differ from those typically associated with classic DM. Differences in serological abnormalities, cutaneous manifestations and response to first treatment among patients with CADM with and without malignancy were found, and suggest distinct pathophysiologies among CADM subsets. Characterization of this cohort expands the knowledge about this unique DM subset.</abstract><abstract type="short" lang="en">What's already known about this topic? Clinically amyopathic dermatomyositis (CADM) comprises a significant subset of patients with dermatomyositis (DM). Like classic DM, CADM presents with similar cutaneous manifestations and may be associated with lung disease or malignancy. What does this study add? Although patients with CADM may initially respond to conservative therapy, most eventually require more aggressive treatment. Malignancies and clinical features found in patients with malignancy‐associated (MA)‐CADM may differ from those in MA classic DM. 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