Severe panniculitis caused by homozygous ZZ α1‐antitrypsin deficiency treated successfully with human purified enzyme (Prolastin®)
Identifieur interne : 001B82 ( Istex/Corpus ); précédent : 001B81; suivant : 001B83Severe panniculitis caused by homozygous ZZ α1‐antitrypsin deficiency treated successfully with human purified enzyme (Prolastin®)
Auteurs : M. M. U. Chowdhury ; E. J. Williams ; J. S. Morris ; B. J. M. Ferguson ; A. D. Mcgregor ; A. R. Hedges ; J. D. Stamatakis ; F. M. PopeSource :
- British Journal of Dermatology [ 0007-0963 ] ; 2002-12.
English descriptors
- Teeft :
- A1at, Antitrypsin, Antitrypsin deficiency, Bayer, Bayer corporation, Bridgend, British association, British journal, Case report, Dermatologist, Dermatology, Functional activity, Human enzyme, Liver disease, Liver transplantation, Maintenance therapy, Panniculitis, Phenotype, Prolastin, Rapid progression, Septicaemia, Severe panniculitis, Skin necrosis, Trunk distribution, Upper thighs.
Abstract
Summary Severe panniculitis caused by α1‐antitrypsin deficiency is very rare even though the ZZ phenotype occurs in 1 : 3500 of the population of northern Europe. We describe a 33‐year‐old woman with rapidly progressing panniculitis and extensive skin necrosis with multiple life‐threatening complications. Initial treatment followed by maintenance therapy with human purified enzyme (Prolastin®, Bayer, Bridgend, U.K.) has been life‐saving.
Url:
DOI: 10.1046/j.1365-2133.2002.05095.x
Links to Exploration step
ISTEX:320A14EECB86A0B293D0B68ADBE525E814EC4D08Le document en format XML
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Chowdhury</name><affiliation><mods:affiliation>Department of Dermatology, University Hospital of Wales, Cardiff CF14 4XW, U.K.</mods:affiliation></affiliation></author><author><name sortKey="Williams, E J" sort="Williams, E J" uniqKey="Williams E" first="E. J." last="Williams">E. J. Williams</name><affiliation><mods:affiliation>Chemical Pathology,</mods:affiliation></affiliation></author><author><name sortKey="Morris, J S" sort="Morris, J S" uniqKey="Morris J" first="J. S." last="Morris">J. S. Morris</name><affiliation><mods:affiliation>Medicine,</mods:affiliation></affiliation></author><author><name sortKey="Ferguson, B J M" sort="Ferguson, B J M" uniqKey="Ferguson B" first="B. J. M." last="Ferguson">B. J. M. Ferguson</name><affiliation><mods:affiliation>Anaesthesia,</mods:affiliation></affiliation></author><author><name sortKey="Mcgregor, A D" sort="Mcgregor, A D" uniqKey="Mcgregor A" first="A. D." last="Mcgregor">A. D. Mcgregor</name><affiliation><mods:affiliation>Department of Plastic Surgery, Morriston Hospital, Swansea, U.K.</mods:affiliation></affiliation></author><author><name sortKey="Hedges, A R" sort="Hedges, A R" uniqKey="Hedges A" first="A. R." last="Hedges">A. R. Hedges</name><affiliation><mods:affiliation>Surgery and</mods:affiliation></affiliation></author><author><name sortKey="Stamatakis, J D" sort="Stamatakis, J D" uniqKey="Stamatakis J" first="J. D." last="Stamatakis">J. D. Stamatakis</name><affiliation><mods:affiliation>Surgery and</mods:affiliation></affiliation></author><author><name sortKey="Pope, F M" sort="Pope, F M" uniqKey="Pope F" first="F. M." last="Pope">F. M. Pope</name><affiliation><mods:affiliation>Dermatology, Princess of Wales Hospital, Bridgend CF31 1RQ, U.K.</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">147</biblScope><biblScope unit="issue">6</biblScope><biblScope unit="page" from="1258">1258</biblScope><biblScope unit="page" to="1261">1261</biblScope><biblScope unit="page-count">4</biblScope><publisher>Blackwell Science Ltd</publisher><pubPlace>Oxford, UK</pubPlace><date type="published" when="2002-12">2002-12</date></imprint><idno type="ISSN">0007-0963</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0007-0963</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="Teeft" xml:lang="en"><term>A1at</term><term>Antitrypsin</term><term>Antitrypsin deficiency</term><term>Bayer</term><term>Bayer corporation</term><term>Bridgend</term><term>British association</term><term>British journal</term><term>Case report</term><term>Dermatologist</term><term>Dermatology</term><term>Functional activity</term><term>Human enzyme</term><term>Liver disease</term><term>Liver transplantation</term><term>Maintenance therapy</term><term>Panniculitis</term><term>Phenotype</term><term>Prolastin</term><term>Rapid progression</term><term>Septicaemia</term><term>Severe panniculitis</term><term>Skin necrosis</term><term>Trunk distribution</term><term>Upper thighs</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Summary Severe panniculitis caused by α1‐antitrypsin deficiency is very rare even though the ZZ phenotype occurs in 1 : 3500 of the population of northern Europe. We describe a 33‐year‐old woman with rapidly progressing panniculitis and extensive skin necrosis with multiple life‐threatening complications. Initial treatment followed by maintenance therapy with human purified enzyme (Prolastin®, Bayer, Bridgend, U.K.) has been life‐saving.</div></front></TEI><istex><corpusName>wiley</corpusName><keywords><teeft><json:string>panniculitis</json:string><json:string>prolastin</json:string><json:string>a1at</json:string><json:string>dermatology</json:string><json:string>antitrypsin</json:string><json:string>bayer</json:string><json:string>phenotype</json:string><json:string>antitrypsin deficiency</json:string><json:string>british journal</json:string><json:string>bridgend</json:string><json:string>septicaemia</json:string><json:string>human enzyme</json:string><json:string>upper thighs</json:string><json:string>dermatologist</json:string><json:string>british association</json:string><json:string>severe panniculitis</json:string><json:string>skin necrosis</json:string><json:string>trunk distribution</json:string><json:string>rapid progression</json:string><json:string>liver disease</json:string><json:string>functional activity</json:string><json:string>liver transplantation</json:string><json:string>maintenance therapy</json:string><json:string>case report</json:string><json:string>bayer corporation</json:string></teeft></keywords><author><json:item><name>M.M.U. Chowdhury</name><affiliations><json:string>Department of Dermatology, University Hospital of Wales, Cardiff CF14 4XW, U.K.</json:string></affiliations></json:item><json:item><name>E.J. Williams</name><affiliations><json:string>Chemical Pathology,</json:string></affiliations></json:item><json:item><name>J.S. Morris</name><affiliations><json:string>Medicine,</json:string></affiliations></json:item><json:item><name>B.J.M. Ferguson</name><affiliations><json:string>Anaesthesia,</json:string></affiliations></json:item><json:item><name>A.D. McGregor</name><affiliations><json:string>Department of Plastic Surgery, Morriston Hospital, Swansea, U.K.</json:string></affiliations></json:item><json:item><name>A.R. Hedges</name><affiliations><json:string>Surgery and</json:string></affiliations></json:item><json:item><name>J.D. Stamatakis</name><affiliations><json:string>Surgery and</json:string></affiliations></json:item><json:item><name>F.M. Pope</name><affiliations><json:string>Dermatology, Princess of Wales Hospital, Bridgend CF31 1RQ, U.K.</json:string></affiliations></json:item></author><subject><json:item><lang><json:string>eng</json:string></lang><value>α1‐antitrypsin deficiency</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>panniculitis</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>Prolastin®</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>ZZ homozygous</value></json:item></subject><articleId><json:string>BJD5095</json:string></articleId><arkIstex>ark:/67375/WNG-LNM9NQ39-D</arkIstex><language><json:string>eng</json:string></language><originalGenre><json:string>caseStudy</json:string></originalGenre><abstract>Summary Severe panniculitis caused by α1‐antitrypsin deficiency is very rare even though the ZZ phenotype occurs in 1 : 3500 of the population of northern Europe. We describe a 33‐year‐old woman with rapidly progressing panniculitis and extensive skin necrosis with multiple life‐threatening complications. 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startDate="2002-12">December 2002</coverDate></publicationMeta><publicationMeta level="unit" type="caseStudy" position="0125800" status="forIssue"><doi origin="wiley">10.1046/j.1365-2133.2002.05095.x</doi><idGroup><id type="unit" value="BJD5095"></id></idGroup><countGroup><count type="pageTotal" number="4"></count></countGroup><titleGroup><title type="tocHeading1">Case reports</title></titleGroup><eventGroup><event type="firstOnline" date="2002-12-09"></event><event type="publishedOnlineFinalForm" date="2002-12-09"></event><event type="xmlConverted" agent="Converter:BPG_TO_WML3G version:2.3.2 mode:FullText source:FullText result:FullText" date="2010-03-08"></event><event type="xmlConverted" agent="Converter:WILEY_ML3G_TO_WILEY_ML3GV2 version:4.0.1" date="2014-03-15"></event><event type="xmlConverted" agent="Converter:WML3G_To_WML3G version:4.1.7 mode:FullText,remove_FC" date="2014-10-15"></event></eventGroup><numberingGroup><numbering type="pageFirst" number="1258">1258</numbering><numbering type="pageLast" number="1261">1261</numbering></numberingGroup><correspondenceTo> Professor F.M.Pope, Department of Dermatology, West Middlesex University Hospital, Twickenham Road, Isleworth, Middlesex TW7 6AF, U.K. E‐mail: <email normalForm="michael.pope@wmuh-tr.nthames.nhs.uk">michael.pope@wmuh‐tr.nthames.nhs.uk</email></correspondenceTo><linkGroup><link type="toTypesetVersion" href="file:BJD.BJD5095.pdf"></link></linkGroup></publicationMeta><contentMeta><unparsedEditorialHistory>Accepted for publication 5 August 2002</unparsedEditorialHistory><countGroup><count type="figureTotal" number="3"></count><count type="tableTotal" number="0"></count><count type="formulaTotal" number="0"></count><count type="referenceTotal" number="6"></count><count type="wordTotal" number="1723"></count><count type="linksPubMed" number="0"></count><count type="linksCrossRef" number="0"></count></countGroup><titleGroup><title type="main">Severe panniculitis caused by homozygous ZZ α<sub>1</sub>‐antitrypsin deficiency treated successfully with human purified enzyme (Prolastin<sup>®</sup>)</title><title type="shortAuthors">M.M.U.CHOWDHURY <i>et al</i>.</title><title type="short">PROLASTIN<sup>®</sup> FOR A1AT DEFICIENCY PANNICULITIS</title></titleGroup><creators><creator creatorRole="author" xml:id="cr1" affiliationRef="#a1"><personName><givenNames>M.M.U.</givenNames><familyName>Chowdhury</familyName></personName></creator><creator creatorRole="author" xml:id="cr2" affiliationRef="#a2"><personName><givenNames>E.J.</givenNames><familyName>Williams</familyName></personName></creator><creator creatorRole="author" xml:id="cr3" affiliationRef="#a3"><personName><givenNames>J.S.</givenNames><familyName>Morris</familyName></personName></creator><creator creatorRole="author" xml:id="cr4" affiliationRef="#a4"><personName><givenNames>B.J.M.</givenNames><familyName>Ferguson</familyName></personName></creator><creator creatorRole="author" xml:id="cr5" affiliationRef="#a5"><personName><givenNames>A.D.</givenNames><familyName>McGregor</familyName></personName></creator><creator creatorRole="author" xml:id="cr6" affiliationRef="#a6"><personName><givenNames>A.R.</givenNames><familyName>Hedges</familyName></personName></creator><creator creatorRole="author" xml:id="cr7" affiliationRef="#a6"><personName><givenNames>J.D.</givenNames><familyName>Stamatakis</familyName></personName></creator><creator creatorRole="author" xml:id="cr8" affiliationRef="#a7"><personName><givenNames>F.M.</givenNames><familyName>Pope</familyName></personName></creator></creators><affiliationGroup><affiliation xml:id="a1"><unparsedAffiliation>Department of Dermatology, University Hospital of Wales, Cardiff CF14 4XW, U.K.</unparsedAffiliation></affiliation><affiliation xml:id="a2"><unparsedAffiliation>Chemical Pathology,</unparsedAffiliation></affiliation><affiliation xml:id="a3"><unparsedAffiliation>Medicine,</unparsedAffiliation></affiliation><affiliation xml:id="a4"><unparsedAffiliation>Anaesthesia,</unparsedAffiliation></affiliation><affiliation xml:id="a6"><unparsedAffiliation>Surgery and</unparsedAffiliation></affiliation><affiliation xml:id="a7"><unparsedAffiliation>Dermatology, Princess of Wales Hospital, Bridgend CF31 1RQ, U.K.</unparsedAffiliation></affiliation><affiliation xml:id="a5"><unparsedAffiliation>Department of Plastic Surgery, Morriston Hospital, Swansea, U.K.</unparsedAffiliation></affiliation></affiliationGroup><keywordGroup xml:lang="en"><keyword xml:id="k1">α<sub>1</sub>‐antitrypsin deficiency</keyword><keyword xml:id="k2">panniculitis</keyword><keyword xml:id="k3">Prolastin<sup>®</sup></keyword><keyword xml:id="k4">ZZ homozygous</keyword></keywordGroup><abstractGroup><abstract type="main" xml:lang="en"><p><b>Summary</b> Severe panniculitis caused by α<sub>1</sub>‐antitrypsin deficiency is very rare even though the ZZ phenotype occurs in 1 : 3500 of the population of northern Europe. We describe a 33‐year‐old woman with rapidly progressing panniculitis and extensive skin necrosis with multiple life‐threatening complications. Initial treatment followed by maintenance therapy with human purified enzyme (Prolastin<sup>®</sup>, Bayer, Bridgend, U.K.) has been life‐saving.</p></abstract></abstractGroup></contentMeta></header></component></istex:document></istex:metadataXml><mods version="3.6"><titleInfo lang="en"><title>Severe panniculitis caused by homozygous ZZ α1‐antitrypsin deficiency treated successfully with human purified enzyme (Prolastin®)</title></titleInfo><titleInfo type="abbreviated" lang="en"><title>PROLASTIN® FOR A1AT DEFICIENCY PANNICULITIS</title></titleInfo><titleInfo type="alternative" contentType="CDATA" lang="en"><title>Severe panniculitis caused by homozygous ZZ α1‐antitrypsin deficiency treated successfully with human purified enzyme (Prolastin®)</title></titleInfo><name type="personal"><namePart type="given">M.M.U.</namePart><namePart type="family">Chowdhury</namePart><affiliation>Department of Dermatology, University Hospital of Wales, Cardiff CF14 4XW, U.K.</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">E.J.</namePart><namePart type="family">Williams</namePart><affiliation>Chemical Pathology,</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">J.S.</namePart><namePart type="family">Morris</namePart><affiliation>Medicine,</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">B.J.M.</namePart><namePart type="family">Ferguson</namePart><affiliation>Anaesthesia,</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">A.D.</namePart><namePart type="family">McGregor</namePart><affiliation>Department of Plastic Surgery, Morriston Hospital, Swansea, U.K.</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">A.R.</namePart><namePart type="family">Hedges</namePart><affiliation>Surgery and</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">J.D.</namePart><namePart type="family">Stamatakis</namePart><affiliation>Surgery and</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">F.M.</namePart><namePart type="family">Pope</namePart><affiliation>Dermatology, Princess of Wales Hospital, Bridgend CF31 1RQ, U.K.</affiliation><role><roleTerm type="text">author</roleTerm></role></name><typeOfResource>text</typeOfResource><genre type="case-report" displayLabel="caseStudy" authority="ISTEX" authorityURI="https://content-type.data.istex.fr" valueURI="https://content-type.data.istex.fr/ark:/67375/XTP-29919SZJ-6">case-report</genre><originInfo><publisher>Blackwell Science Ltd</publisher><place><placeTerm type="text">Oxford, UK</placeTerm></place><dateIssued encoding="w3cdtf">2002-12</dateIssued><edition>Accepted for publication 5 August 2002</edition><copyrightDate encoding="w3cdtf">2002</copyrightDate></originInfo><language><languageTerm type="code" authority="rfc3066">en</languageTerm><languageTerm type="code" authority="iso639-2b">eng</languageTerm></language><physicalDescription><extent unit="figures">3</extent><extent unit="tables">0</extent><extent unit="formulas">0</extent><extent unit="references">6</extent><extent unit="linksCrossRef">0</extent><extent unit="words">1723</extent></physicalDescription><abstract lang="en">Summary Severe panniculitis caused by α1‐antitrypsin deficiency is very rare even though the ZZ phenotype occurs in 1 : 3500 of the population of northern Europe. We describe a 33‐year‐old woman with rapidly progressing panniculitis and extensive skin necrosis with multiple life‐threatening complications. Initial treatment followed by maintenance therapy with human purified enzyme (Prolastin®, Bayer, Bridgend, U.K.) has been life‐saving.</abstract><subject lang="en"><genre>keywords</genre><topic>α1‐antitrypsin deficiency</topic><topic>panniculitis</topic><topic>Prolastin®</topic><topic>ZZ homozygous</topic></subject><relatedItem type="host"><titleInfo><title>British Journal of Dermatology</title></titleInfo><genre type="journal" authority="ISTEX" authorityURI="https://publication-type.data.istex.fr" valueURI="https://publication-type.data.istex.fr/ark:/67375/JMC-0GLKJH51-B">journal</genre><identifier type="ISSN">0007-0963</identifier><identifier type="eISSN">1365-2133</identifier><identifier type="DOI">10.1111/(ISSN)1365-2133</identifier><identifier type="PublisherID">BJD</identifier><part><date>2002</date><detail type="volume"><caption>vol.</caption><number>147</number></detail><detail type="issue"><caption>no.</caption><number>6</number></detail><extent unit="pages"><start>1258</start><end>1261</end><total>4</total></extent></part></relatedItem><relatedItem type="references" displayLabel="cit1"><titleInfo><title>Alpha‐1 antitrypsin deficiency associated panniculitis: resolution with intravenous alpha‐1 antitrypsin administration and liver transplantation</title></titleInfo><name type="personal"><namePart type="given">K</namePart><namePart type="family">O'Riordan</namePart><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">A</namePart><namePart type="family">Blei</namePart><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">MS</namePart><namePart type="family">Rao</namePart><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">M</namePart><namePart type="family">Abecassis</namePart><role><roleTerm type="text">author</roleTerm></role></name><genre>journal-article</genre><note type="citation/reference">O'Riordan K, Blei A, Rao MS, Abecassis M. 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