Cutaneous porphyrias part II: treatment strategies
Identifieur interne : 000092 ( Istex/Corpus ); précédent : 000091; suivant : 000093Cutaneous porphyrias part II: treatment strategies
Auteurs : Suzanne Tintle ; Ali Alikhan ; Mary E. Horner ; Jennifer L. Hand ; Dawn Marie R. DavisSource :
- International Journal of Dermatology [ 0011-9059 ] ; 2014-01.
Abstract
The porphyrias are diverse in pathophysiology, clinical presentation, severity, and prognosis, presenting a diagnostic and therapeutic challenge. Although not easily curable, the dermatological manifestations of these diseases, photosensitivity and associated cutaneous pathology, can be effectively prevented and managed. Sun avoidance is essential, and patient education regarding the irreversibility of photocutaneous damage is a necessary corollary. Beyond preventative measures, the care of fragile, vulnerable skin, and pain management, each of the porphyrias has a limited number of unique additional therapeutic options. Many of the treatments have been published only in small case series or anecdotal reports and do not have well‐understood nor proven mechanisms of action. This article presents a comprehensive review of available therapeutic options and long‐term management recommendations for the cutaneous porphyrias.
Url:
DOI: 10.1111/ijd.12016
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ISTEX:A5D9A929E99FC3B0A392C790C8B83C43C2B7018FLe document en format XML
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value="IJD12016"></id></idGroup><countGroup><count number="22" type="pageTotal"></count></countGroup><titleGroup><title type="articleCategory">Review</title><title type="tocHeading1">Review</title></titleGroup><copyright ownership="thirdParty">© 2013 The International Society of Dermatology</copyright><eventGroup><event agent="SPS" date="2012-10-16" type="xmlCreated"></event><event type="xmlConverted" agent="Converter:WILEY_ML3G_TO_WILEY_ML3GV2 version:3.2.8 mode:FullText" date="2013-12-18"></event><event type="publishedOnlineEarlyUnpaginated" date="2013-10-18"></event><event type="firstOnline" date="2013-10-18"></event><event type="publishedOnlineFinalForm" date="2013-12-18"></event><event type="xmlConverted" agent="Converter:WML3G_To_WML3G version:4.6.4 mode:FullText" date="2015-10-08"></event></eventGroup><numberingGroup><numbering type="pageFirst">3</numbering><numbering type="pageLast">24</numbering></numberingGroup><correspondenceTo><lineatedText><line><b>Correspondence</b></line><line>Ali Alikhan, <sc>md</sc></line><line>Department of Dermatology, Mayo Clinic</line><line>200 First Street SW</line><line>Rochester</line><line>MN 55905</line><line>USA</line><line>E‐mail: <email>alikhan.mirza@mayo.edu</email></line></lineatedText></correspondenceTo><linkGroup><link type="toTypesetVersion" href="file:IJD.IJD12016.pdf"></link></linkGroup></publicationMeta><contentMeta><titleGroup><title type="main">Cutaneous porphyrias part <fc>II</fc>: treatment strategies</title><title type="shortAuthors"><i>Tintle</i> et al.</title></titleGroup><creators><creator affiliationRef="#ijd12016-aff-0001" creatorRole="author" xml:id="ijd12016-cr-0001"><personName><givenNames>Suzanne</givenNames> <familyName>Tintle</familyName> <degrees>MD, MPH</degrees></personName></creator><creator affiliationRef="#ijd12016-aff-0002" corresponding="yes" creatorRole="author" xml:id="ijd12016-cr-0002"><personName><givenNames>Ali</givenNames> <familyName>Alikhan</familyName> <degrees>MD</degrees></personName></creator><creator affiliationRef="#ijd12016-aff-0003" creatorRole="author" xml:id="ijd12016-cr-0003"><personName><givenNames>Mary E.</givenNames> <familyName>Horner</familyName> <degrees>MD</degrees></personName></creator><creator affiliationRef="#ijd12016-aff-0002 #ijd12016-aff-0004 #ijd12016-aff-0005" creatorRole="author" xml:id="ijd12016-cr-0004"><personName><givenNames>Jennifer L.</givenNames> <familyName>Hand</familyName> <degrees>MD</degrees></personName></creator><creator affiliationRef="#ijd12016-aff-0002 #ijd12016-aff-0004" creatorRole="author" xml:id="ijd12016-cr-0005"><personName><givenNames>Dawn Marie R.</givenNames> <familyName>Davis</familyName> <degrees>MD</degrees></personName></creator></creators><affiliationGroup><affiliation countryCode="US" type="organization" xml:id="ijd12016-aff-0001"><orgDiv>Department of Dermatology</orgDiv> <orgName>Tufts Medical Center</orgName> <address><city>Boston</city> <countryPart>MA</countryPart> <country>USA</country></address></affiliation><affiliation countryCode="US" type="organization" xml:id="ijd12016-aff-0002"><orgDiv>Department of Dermatology</orgDiv> <orgName>Mayo Clinic</orgName> <address><city>Rochester</city> <countryPart>MN</countryPart> <country>USA</country></address></affiliation><affiliation countryCode="US" type="organization" xml:id="ijd12016-aff-0003"><orgDiv>Department of Dermatology</orgDiv> <orgName>Baylor University Medical Center</orgName> <address><city>Dallas</city> <countryPart>TX</countryPart> <country>USA</country></address></affiliation><affiliation countryCode="US" type="organization" xml:id="ijd12016-aff-0004"><orgDiv>Department of Pediatrics</orgDiv> <orgName>Mayo Clinic</orgName> <address><city>Rochester</city> <countryPart>MN</countryPart> <country>USA</country></address></affiliation><affiliation countryCode="US" type="organization" xml:id="ijd12016-aff-0005"><orgDiv>Department of Medical Genetics</orgDiv> <orgName>Mayo Clinic</orgName> <address><city>Rochester</city> <countryPart>MN</countryPart> <country>USA</country></address></affiliation></affiliationGroup><abstractGroup><abstract type="main" xml:id="ijd12016-abs-0001"><title type="main">Abstract</title><p>The porphyrias are diverse in pathophysiology, clinical presentation, severity, and prognosis, presenting a diagnostic and therapeutic challenge. Although not easily curable, the dermatological manifestations of these diseases, photosensitivity and associated cutaneous pathology, can be effectively prevented and managed. Sun avoidance is essential, and patient education regarding the irreversibility of photocutaneous damage is a necessary corollary. Beyond preventative measures, the care of fragile, vulnerable skin, and pain management, each of the porphyrias has a limited number of unique additional therapeutic options. Many of the treatments have been published only in small case series or anecdotal reports and do not have well‐understood nor proven mechanisms of action. This article presents a comprehensive review of available therapeutic options and long‐term management recommendations for the cutaneous porphyrias.</p></abstract></abstractGroup></contentMeta><noteGroup xml:id="ijd12016-ntgp-0001"><note numbered="no" xml:id="ijd12016-note-0001"><p>Funding: None.</p></note><note numbered="no" xml:id="ijd12016-note-0002"><p>Conflicts of interest: None.</p></note></noteGroup></header></component></istex:document></istex:metadataXml><mods version="3.6"><titleInfo lang="en"><title>Cutaneous porphyrias part II: treatment strategies</title></titleInfo><titleInfo type="alternative" contentType="CDATA" lang="en"><title>Cutaneous porphyrias part II: treatment strategies</title></titleInfo><name type="personal"><namePart type="given">Suzanne</namePart><namePart type="family">Tintle</namePart><namePart type="termsOfAddress">MD, MPH</namePart><affiliation>Department of Dermatology, Tufts Medical Center, MA, Boston, USA</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Ali</namePart><namePart type="family">Alikhan</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Dermatology, Mayo Clinic, MN, Rochester, USA</affiliation><affiliation>Ali Alikhan, Department of Dermatology, Mayo Clinic200 First Street SWRochesterMN 55905USAE‐mail: </affiliation><affiliation>E-mail: alikhan.mirza@mayo.edu</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Mary E.</namePart><namePart type="family">Horner</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Dermatology, Baylor University Medical Center, TX, Dallas, USA</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Jennifer L.</namePart><namePart type="family">Hand</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Dermatology, Mayo Clinic, Rochester, MN, USA</affiliation><affiliation>Department of Pediatrics, Mayo Clinic, Rochester, MN, USA</affiliation><affiliation>Department of Medical Genetics, Mayo Clinic, MN, Rochester, USA</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Dawn Marie R.</namePart><namePart type="family">Davis</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Dermatology, Mayo Clinic, Rochester, MN, USA</affiliation><affiliation>Department of Pediatrics, Mayo Clinic, MN, Rochester, USA</affiliation><role><roleTerm type="text">author</roleTerm></role></name><typeOfResource>text</typeOfResource><genre type="review-article" displayLabel="reviewArticle" authority="ISTEX" authorityURI="https://content-type.data.istex.fr" valueURI="https://content-type.data.istex.fr/ark:/67375/XTP-L5L7X3NF-P">review-article</genre><originInfo><publisher>Blackwell Publishing Ltd</publisher><dateIssued encoding="w3cdtf">2014-01</dateIssued><dateCreated encoding="w3cdtf">2012-10-16</dateCreated><copyrightDate encoding="w3cdtf">2014</copyrightDate></originInfo><language><languageTerm type="code" authority="rfc3066">en</languageTerm><languageTerm type="code" authority="iso639-2b">eng</languageTerm></language><abstract>The porphyrias are diverse in pathophysiology, clinical presentation, severity, and prognosis, presenting a diagnostic and therapeutic challenge. Although not easily curable, the dermatological manifestations of these diseases, photosensitivity and associated cutaneous pathology, can be effectively prevented and managed. Sun avoidance is essential, and patient education regarding the irreversibility of photocutaneous damage is a necessary corollary. Beyond preventative measures, the care of fragile, vulnerable skin, and pain management, each of the porphyrias has a limited number of unique additional therapeutic options. Many of the treatments have been published only in small case series or anecdotal reports and do not have well‐understood nor proven mechanisms of action. 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