Noonan's syndrome with extensive verrucae.
Identifieur interne : 00D995 ( Main/Exploration ); précédent : 00D994; suivant : 00D996Noonan's syndrome with extensive verrucae.
Auteurs : J. Bernier-Buzzanga [États-Unis] ; W P SuSource :
- Cutis [ 0011-4162 ] ; 1990.
Descripteurs français
- KwdFr :
- MESH :
- anatomopathologie : Syndrome de Noonan, Verrues.
- Adulte, Humains, Lymphoedème, Mâle.
English descriptors
- KwdEn :
- MESH :
- complications : Lymphedema.
- congenital : Lymphedema.
- pathology : Noonan Syndrome, Warts.
- Adult, Humans, Male.
Abstract
We report the case of a patient with multiple findings characteristic of Noonan's syndrome, including a severe lymphedema present since early infancy, hypertelorism, low-set and prominent ears, broad facies, low posterior hairline, high arched palate, broad short neck, slightly short stature, and moderate dental malocclusion. In addition, extensive verrucae planae, verrucae vulgares, and condylomata acuminata occurred and were refractory to conventional therapy. To our knowledge, this is the first report of an association of Noonan's syndrome with extensive verrucae. We speculate that it is related to immunodeficiency as a consequence of loss of immunoglobulins and lymphocytes from intestinal lymphangiectasia. Cutaneous findings and other physical anomalies reported in patients with Noonan's syndrome are reviewed.
PubMed: 2225932
Affiliations:
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Le document en format XML
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Bernier-Buzzanga</name><affiliation wicri:level="2"><nlm:affiliation>Department of Dermatology, Mayo Clinic, Rochester, Minnesota 55905.</nlm:affiliation><country xml:lang="fr">États-Unis</country><placeName><region type="state">Minnesota</region></placeName><wicri:cityArea>Department of Dermatology, Mayo Clinic, Rochester</wicri:cityArea></affiliation></author><author><name sortKey="Su, W P" sort="Su, W P" uniqKey="Su W" first="W P" last="Su">W P Su</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="1990">1990</date><idno type="RBID">pubmed:2225932</idno><idno type="pmid">2225932</idno><idno type="wicri:Area/PubMed/Corpus">005D13</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">005D13</idno><idno type="wicri:Area/PubMed/Curation">005D13</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Curation">005D13</idno><idno type="wicri:Area/PubMed/Checkpoint">005D13</idno><idno type="wicri:explorRef" wicri:stream="Checkpoint" wicri:step="PubMed">005D13</idno><idno type="wicri:Area/Ncbi/Merge">004991</idno><idno type="wicri:Area/Ncbi/Curation">004991</idno><idno type="wicri:Area/Ncbi/Checkpoint">004991</idno><idno type="wicri:doubleKey">0011-4162:1990:Bernier Buzzanga J:noonan:s:syndrome</idno><idno type="wicri:Area/Main/Merge">00E579</idno><idno type="wicri:Area/Main/Curation">00D995</idno><idno type="wicri:Area/Main/Exploration">00D995</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Noonan's syndrome with extensive verrucae.</title><author><name sortKey="Bernier Buzzanga, J" sort="Bernier Buzzanga, J" uniqKey="Bernier Buzzanga J" first="J" last="Bernier-Buzzanga">J. Bernier-Buzzanga</name><affiliation wicri:level="2"><nlm:affiliation>Department of Dermatology, Mayo Clinic, Rochester, Minnesota 55905.</nlm:affiliation><country xml:lang="fr">États-Unis</country><placeName><region type="state">Minnesota</region></placeName><wicri:cityArea>Department of Dermatology, Mayo Clinic, Rochester</wicri:cityArea></affiliation></author><author><name sortKey="Su, W P" sort="Su, W P" uniqKey="Su W" first="W P" last="Su">W P Su</name></author></analytic><series><title level="j">Cutis</title><idno type="ISSN">0011-4162</idno><imprint><date when="1990" type="published">1990</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term><term>Humans</term><term>Lymphedema (complications)</term><term>Lymphedema (congenital)</term><term>Male</term><term>Noonan Syndrome (pathology)</term><term>Warts (pathology)</term></keywords><keywords scheme="KwdFr" xml:lang="fr"><term>Adulte</term><term>Humains</term><term>Lymphoedème ()</term><term>Mâle</term><term>Syndrome de Noonan (anatomopathologie)</term><term>Verrues (anatomopathologie)</term></keywords><keywords scheme="MESH" qualifier="anatomopathologie" xml:lang="fr"><term>Syndrome de Noonan</term><term>Verrues</term></keywords><keywords scheme="MESH" qualifier="complications" xml:lang="en"><term>Lymphedema</term></keywords><keywords scheme="MESH" qualifier="congenital" xml:lang="en"><term>Lymphedema</term></keywords><keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Noonan Syndrome</term><term>Warts</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Humans</term><term>Male</term></keywords><keywords scheme="MESH" xml:lang="fr"><term>Adulte</term><term>Humains</term><term>Lymphoedème</term><term>Mâle</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">We report the case of a patient with multiple findings characteristic of Noonan's syndrome, including a severe lymphedema present since early infancy, hypertelorism, low-set and prominent ears, broad facies, low posterior hairline, high arched palate, broad short neck, slightly short stature, and moderate dental malocclusion. In addition, extensive verrucae planae, verrucae vulgares, and condylomata acuminata occurred and were refractory to conventional therapy. To our knowledge, this is the first report of an association of Noonan's syndrome with extensive verrucae. We speculate that it is related to immunodeficiency as a consequence of loss of immunoglobulins and lymphocytes from intestinal lymphangiectasia. Cutaneous findings and other physical anomalies reported in patients with Noonan's syndrome are reviewed.</div></front></TEI><affiliations><list><country><li>États-Unis</li></country><region><li>Minnesota</li></region></list><tree><noCountry><name sortKey="Su, W P" sort="Su, W P" uniqKey="Su W" first="W P" last="Su">W P Su</name></noCountry><country name="États-Unis"><region name="Minnesota"><name sortKey="Bernier Buzzanga, J" sort="Bernier Buzzanga, J" uniqKey="Bernier Buzzanga J" first="J" last="Bernier-Buzzanga">J. 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