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<title>
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Entry
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- #224750 - SCHOPF-SCHULZ-PASSARGE SYNDROME; SSPS
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- OMIM
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<span class="h4">#224750</span>
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<br />
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<strong>Table of Contents</strong>
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<a href="#phenotypeMap"><strong>Phenotype-Gene Relationships</strong></a>
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<a href="/clinicalSynopsis/224750"><strong>Clinical Synopsis</strong></a>
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<a href="#description">Description</a>
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<a href="#clinicalFeatures">Clinical Features</a>
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<a href="#inheritance">Inheritance</a>
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<a href="#molecularGenetics">Molecular Genetics</a>
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<a href="#contributors"><strong>Contributors</strong></a>
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<a href="#creationDate"><strong>Creation Date</strong></a>
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<a href="#editHistory"><strong>Edit History</strong></a>
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<div><a href="https://clinicaltrials.gov/search?cond=(SCHOPF-SCHULZ-PASSARGE SYNDROME) OR (WNT10A)" class="mim-tip-hint" title="Clinical Trials" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'Clinical Trials', 'domain': 'clinicaltrials.gov'})">Clinical Trials</a></div>
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<div><a href="https://www.orpha.net/consor/cgi-bin/ClinicalLabs_Search_Simple.php?lng=EN&LnkId=10664&Typ=Pat" class="mim-tip-hint" title="A list of European laboratories that offer genetic testing." target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'EuroGentest', 'domain': 'orpha.net'})">EuroGentest</a></div>
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<div><a href="https://www.diseaseinfosearch.org/x/9270" class="mim-tip-hint" title="Network of disease-specific advocacy organizations, universities, private companies, government agencies, and public policy organizations." target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'Genetic Alliance', 'domain': 'diseaseinfosearch.org'})">Genetic Alliance</a></div>
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<div><a href="https://www.ncbi.nlm.nih.gov/gtr/all/tests/?term=224750[mim]" class="mim-tip-hint" title="Genetic Testing Registry." target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'GTR', 'domain': 'ncbi.nlm.nih.gov'})">GTR</a></div>
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<div><a href="https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=50944" class="mim-tip-hint" title="European reference portal for information on rare diseases and orphan drugs." target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'OrphaNet', 'domain': 'orpha.net'})">OrphaNet</a></div>
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<div><a href="https://www.possumcore.com/nuxeo/nxdoc/default/0b25d170-844a-4fd1-bdef-7dec9dfdcb7f/view_documents?source=omim" class="mim-tip-hint" title="A dysmorphology database of multiple malformations; metabolic, teratogenic, chromosomal, and skeletal syndromes; and their images." target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'POSSUM', 'domain': 'possum.net.au'})">POSSUM</a></div>
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<div style="display: table-cell;">Animal Models</div>
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<div><a href="https://www.alliancegenome.org/disease/DOID:0111647" class="mim-tip-hint" title="Search Across Species; explore model organism and human comparative genomics." target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'Alliance Genome', 'domain': 'alliancegenome.org'})">Alliance Genome</a></div>
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<div><a href="http://www.informatics.jax.org/disease/224750" class="mim-tip-hint" title="Phenotypes, alleles, and disease models from Mouse Genome Informatics." target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'MGI Mouse Phenotype', 'domain': 'informatics.jax.org'})">MGI Mouse Phenotype</a></div>
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<div><a href="https://wormbase.org/resources/disease/DOID:0111647" class="mim-tip-hint" title="Database of the biology and genome of Caenorhabditis elegans and related nematodes." target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'Wormbase Disease Ontology', 'domain': 'wormbase.org'})">Wormbase Disease Ontology</a></div>
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<span>
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<span class="mim-tip-bottom" qtip_title="<strong>Looking for this gene or this phenotype in other resources?</strong>" qtip_text="Select a related resource from the dropdown menu and click for a targeted link to information directly relevant.">
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<a id="title" class="mim-anchor"></a>
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<div>
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<a id="number" class="mim-anchor"></a>
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<div class="text-right">
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<a href="#" class="mim-tip-icd" qtip_title="<strong>ICD+</strong>" qtip_text="
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<strong>SNOMEDCT:</strong> 700062000<br />
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<strong>ORPHA:</strong> 50944<br />
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<strong>DO:</strong> 0111647<br />
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">ICD+</a>
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</div>
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<div>
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<span class="h3">
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<span class="mim-font mim-tip-hint" title="Phenotype description, molecular basis known">
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<span class="text-danger"><strong>#</strong></span>
|
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224750
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</span>
|
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</span>
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</div>
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</div>
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<div>
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<a id="preferredTitle" class="mim-anchor"></a>
|
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<h3>
|
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<span class="mim-font">
|
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SCHOPF-SCHULZ-PASSARGE SYNDROME; SSPS
|
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</span>
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</h3>
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</div>
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<div>
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<br />
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</div>
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<div>
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<a id="alternativeTitles" class="mim-anchor"></a>
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<div>
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<p>
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<span class="mim-font">
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<em>Alternative titles; symbols</em>
|
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</span>
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</p>
|
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</div>
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<div>
|
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<h4>
|
|
<span class="mim-font">
|
|
KERATOSIS PALMOPLANTARIS WITH CYSTIC EYELIDS, HYPODONTIA, AND HYPOTRICHOSIS<br />
|
|
ECCRINE TUMORS WITH ECTODERMAL DYSPLASIA
|
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</span>
|
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</h4>
|
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</div>
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</div>
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<div>
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<br />
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</div>
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</div>
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|
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<div>
|
|
<a id="phenotypeMap" class="mim-anchor"></a>
|
|
<h4>
|
|
<span class="mim-font">
|
|
<strong>Phenotype-Gene Relationships</strong>
|
|
</span>
|
|
</h4>
|
|
<div>
|
|
<table class="table table-bordered table-condensed table-hover small mim-table-padding">
|
|
<thead>
|
|
<tr class="active">
|
|
<th>
|
|
Location
|
|
</th>
|
|
<th>
|
|
Phenotype
|
|
</th>
|
|
<th>
|
|
Phenotype <br /> MIM number
|
|
</th>
|
|
<th>
|
|
Inheritance
|
|
</th>
|
|
<th>
|
|
Phenotype <br /> mapping key
|
|
</th>
|
|
<th>
|
|
Gene/Locus
|
|
</th>
|
|
<th>
|
|
Gene/Locus <br /> MIM number
|
|
</th>
|
|
</tr>
|
|
</thead>
|
|
<tbody>
|
|
|
|
<tr>
|
|
<td>
|
|
<span class="mim-font">
|
|
<a href="/geneMap/2/1021?start=-3&limit=10&highlight=1021">
|
|
2q35
|
|
</a>
|
|
</span>
|
|
</td>
|
|
<td>
|
|
<span class="mim-font">
|
|
Schopf-Schulz-Passarge syndrome
|
|
|
|
</span>
|
|
</td>
|
|
<td>
|
|
<span class="mim-font">
|
|
<a href="/entry/224750"> 224750 </a>
|
|
</span>
|
|
</td>
|
|
<td>
|
|
<span class="mim-font">
|
|
|
|
<abbr class="mim-tip-hint" title="Autosomal recessive">AR</abbr>
|
|
|
|
</span>
|
|
</td>
|
|
<td>
|
|
<span class="mim-font">
|
|
|
|
<abbr class="mim-tip-hint" title="3 - The molecular basis of the disorder is known"> 3 </abbr>
|
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|
|
</span>
|
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</td>
|
|
<td>
|
|
<span class="mim-font">
|
|
WNT10A
|
|
</span>
|
|
</td>
|
|
<td>
|
|
<span class="mim-font">
|
|
<a href="/entry/606268"> 606268 </a>
|
|
</span>
|
|
</td>
|
|
</tr>
|
|
|
|
</tbody>
|
|
</table>
|
|
</div>
|
|
</div>
|
|
|
|
|
|
|
|
|
|
<div>
|
|
|
|
|
|
<div class="btn-group ">
|
|
<a href="/clinicalSynopsis/224750" class="btn btn-warning" role="button"> Clinical Synopsis </a>
|
|
<button type="button" id="mimPhenotypicSeriesToggle" class="btn btn-warning dropdown-toggle mimSingletonFoldToggle" data-toggle="collapse" href="#mimClinicalSynopsisFold" onclick="ga('send', 'event', 'Unfurl', 'ClinicalSynopsis', 'omim.org')">
|
|
<span class="caret"></span>
|
|
<span class="sr-only">Toggle Dropdown</span>
|
|
</button>
|
|
</div>
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
<div class="btn-group">
|
|
<button type="button" class="btn btn-success dropdown-toggle" data-toggle="dropdown" aria-haspopup="true" aria-expanded="false">
|
|
PheneGene Graphics <span class="caret"></span>
|
|
</button>
|
|
<ul class="dropdown-menu" style="width: 17em;">
|
|
<li><a href="/graph/linear/224750" target="_blank" onclick="gtag('event', 'mim_graph', {'destination': 'Linear'})"> Linear </a></li>
|
|
<li><a href="/graph/radial/224750" target="_blank" onclick="gtag('event', 'mim_graph', {'destination': 'Radial'})"> Radial </a></li>
|
|
</ul>
|
|
</div>
|
|
<span class="glyphicon glyphicon-question-sign mim-tip-hint" title="OMIM PheneGene graphics depict relationships between phenotypes, groups of related phenotypes (Phenotypic Series), and genes.<br /><a href='/static/omim/pdf/OMIM_Graphics.pdf' target='_blank'>A quick reference overview and guide (PDF)</a>"></span>
|
|
|
|
|
|
|
|
<div>
|
|
<p />
|
|
</div>
|
|
|
|
|
|
<div id="mimClinicalSynopsisFold" class="well well-sm collapse mimSingletonToggleFold">
|
|
<div class="small" style="margin: 5px">
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
<div>
|
|
<div>
|
|
<span class="h5 mim-font">
|
|
<strong> INHERITANCE </strong>
|
|
</span>
|
|
</div>
|
|
<div style="margin-left: 2em;">
|
|
|
|
<div>
|
|
<span class="mim-font">
|
|
|
|
- Autosomal recessive <span class="mim-feature-ids hidden">[SNOMEDCT: <a href="https://purl.bioontology.org/ontology/SNOMEDCT/258211005" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'SNOMEDCT\', \'domain\': \'bioontology.org\'})">258211005</a>]</span> <span class="mim-feature-ids hidden">[UMLS: <a href="https://bioportal.bioontology.org/search?q=C0441748&searchproperties=true" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'UMLS\', \'domain\': \'bioontology.org\'})">C0441748</a> HPO: <a href="https://hpo.jax.org/app/browse/term/HP:0000007" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'HPO\', \'domain\': \'hpo.jax.org\'})">HP:0000007</a>]</span> <span class="mim-feature-ids hidden">[HPO: <a href="https://hpo.jax.org/app/browse/term/HP:0000007" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'HPO\', \'domain\': \'hpo.jax.org\'})">HP:0000007</a>]</span><br />
|
|
|
|
</span>
|
|
</div>
|
|
|
|
</div>
|
|
|
|
</div>
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
<div>
|
|
<div>
|
|
<span class="h5 mim-font">
|
|
<strong> HEAD & NECK </strong>
|
|
</span>
|
|
</div>
|
|
<div style="margin-left: 2em;">
|
|
|
|
|
|
|
|
<div>
|
|
<div>
|
|
<span class="h5 mim-font">
|
|
<em> Face </em>
|
|
</span>
|
|
</div>
|
|
<div style="margin-left: 2em;">
|
|
<span class="mim-font">
|
|
|
|
- Bird-like facies <span class="mim-feature-ids hidden">[UMLS: <a href="https://bioportal.bioontology.org/search?q=C1837758&searchproperties=true" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'UMLS\', \'domain\': \'bioontology.org\'})">C1837758</a> HPO: <a href="https://hpo.jax.org/app/browse/term/HP:0000320" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'HPO\', \'domain\': \'hpo.jax.org\'})">HP:0000320</a>]</span> <span class="mim-feature-ids hidden">[HPO: <a href="https://hpo.jax.org/app/browse/term/HP:0000320" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'HPO\', \'domain\': \'hpo.jax.org\'})">HP:0000320</a>]</span><br />
|
|
|
|
</span>
|
|
</div>
|
|
</div>
|
|
|
|
|
|
|
|
<div>
|
|
<div>
|
|
<span class="h5 mim-font">
|
|
<em> Eyes </em>
|
|
</span>
|
|
</div>
|
|
<div style="margin-left: 2em;">
|
|
<span class="mim-font">
|
|
|
|
- Eyelid margin cysts (hidrocystoma) <span class="mim-feature-ids hidden">[UMLS: <a href="https://bioportal.bioontology.org/search?q=C1857070&searchproperties=true" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'UMLS\', \'domain\': \'bioontology.org\'})">C1857070</a>]</span><br />
|
|
|
|
</span>
|
|
</div>
|
|
</div>
|
|
|
|
|
|
|
|
|
|
<div>
|
|
<div>
|
|
<span class="h5 mim-font">
|
|
<em> Teeth </em>
|
|
</span>
|
|
</div>
|
|
<div style="margin-left: 2em;">
|
|
<span class="mim-font">
|
|
|
|
- Hypodontia <span class="mim-feature-ids hidden">[SNOMEDCT: <a href="https://purl.bioontology.org/ontology/SNOMEDCT/64969001" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'SNOMEDCT\', \'domain\': \'bioontology.org\'})">64969001</a>]</span> <span class="mim-feature-ids hidden">[ICD10CM: <a href="https://purl.bioontology.org/ontology/ICD10CM/K00.0" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'ICD10CM\', \'domain\': \'bioontology.org\'})">K00.0</a>]</span> <span class="mim-feature-ids hidden">[UMLS: <a href="https://bioportal.bioontology.org/search?q=C0020608&searchproperties=true" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'UMLS\', \'domain\': \'bioontology.org\'})">C0020608</a> HPO: <a href="https://hpo.jax.org/app/browse/term/HP:0000668" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'HPO\', \'domain\': \'hpo.jax.org\'})">HP:0000668</a>]</span> <span class="mim-feature-ids hidden">[HPO: <a href="https://hpo.jax.org/app/browse/term/HP:0000668" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'HPO\', \'domain\': \'hpo.jax.org\'})">HP:0000668</a>]</span><br />
|
|
|
|
</span>
|
|
</div>
|
|
</div>
|
|
|
|
|
|
</div>
|
|
|
|
</div>
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
|
<div>
|
|
<div>
|
|
<span class="h5 mim-font">
|
|
<strong> SKIN, NAILS, & HAIR </strong>
|
|
</span>
|
|
</div>
|
|
<div style="margin-left: 2em;">
|
|
|
|
|
|
<div>
|
|
<div>
|
|
<span class="h5 mim-font">
|
|
<em> Skin </em>
|
|
</span>
|
|
</div>
|
|
<div style="margin-left: 2em;">
|
|
<span class="mim-font">
|
|
|
|
- Palmoplantar keratoderma <span class="mim-feature-ids hidden">[SNOMEDCT: <a href="https://purl.bioontology.org/ontology/SNOMEDCT/706885006" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'SNOMEDCT\', \'domain\': \'bioontology.org\'})">706885006</a>]</span> <span class="mim-feature-ids hidden">[UMLS: <a href="https://bioportal.bioontology.org/search?q=C4551675&searchproperties=true" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'UMLS\', \'domain\': \'bioontology.org\'})">C4551675</a> HPO: <a href="https://hpo.jax.org/app/browse/term/HP:0000982" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'HPO\', \'domain\': \'hpo.jax.org\'})">HP:0000982</a>]</span> <span class="mim-feature-ids hidden">[HPO: <a href="https://hpo.jax.org/app/browse/term/HP:0000982" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'HPO\', \'domain\': \'hpo.jax.org\'})">HP:0000982</a>]</span><br /> -
|
|
Eyelid margin cysts (apocrine hidrocystoma) <span class="mim-feature-ids hidden">[UMLS: <a href="https://bioportal.bioontology.org/search?q=C1857071&searchproperties=true" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'UMLS\', \'domain\': \'bioontology.org\'})">C1857071</a>]</span><br /> -
|
|
Eccrine poroma <span class="mim-feature-ids hidden">[SNOMEDCT: <a href="https://purl.bioontology.org/ontology/SNOMEDCT/399985004" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'SNOMEDCT\', \'domain\': \'bioontology.org\'})">399985004</a>, <a href="https://purl.bioontology.org/ontology/SNOMEDCT/128915006" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'SNOMEDCT\', \'domain\': \'bioontology.org\'})">128915006</a>]</span> <span class="mim-feature-ids hidden">[UMLS: <a href="https://bioportal.bioontology.org/search?q=C1533161&searchproperties=true" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'UMLS\', \'domain\': \'bioontology.org\'})">C1533161</a>]</span><br />
|
|
|
|
</span>
|
|
</div>
|
|
</div>
|
|
|
|
|
|
|
|
|
|
<div>
|
|
<div>
|
|
<span class="h5 mim-font">
|
|
<em> Nails </em>
|
|
</span>
|
|
</div>
|
|
<div style="margin-left: 2em;">
|
|
<span class="mim-font">
|
|
|
|
- Hypoplastic nails <span class="mim-feature-ids hidden">[SNOMEDCT: <a href="https://purl.bioontology.org/ontology/SNOMEDCT/11375002" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'SNOMEDCT\', \'domain\': \'bioontology.org\'})">11375002</a>]</span> <span class="mim-feature-ids hidden">[UMLS: <a href="https://bioportal.bioontology.org/search?q=C0263523&searchproperties=true" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'UMLS\', \'domain\': \'bioontology.org\'})">C0263523</a> HPO: <a href="https://hpo.jax.org/app/browse/term/HP:0001792" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'HPO\', \'domain\': \'hpo.jax.org\'})">HP:0001792</a>]</span> <span class="mim-feature-ids hidden">[HPO: <a href="https://hpo.jax.org/app/browse/term/HP:0001792" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'HPO\', \'domain\': \'hpo.jax.org\'})">HP:0001792</a>]</span><br /> -
|
|
Thin, narrow nails <span class="mim-feature-ids hidden">[UMLS: <a href="https://bioportal.bioontology.org/search?q=C1857072&searchproperties=true" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'UMLS\', \'domain\': \'bioontology.org\'})">C1857072</a>]</span><br /> -
|
|
Longitudinal ridging <span class="mim-feature-ids hidden">[SNOMEDCT: <a href="https://purl.bioontology.org/ontology/SNOMEDCT/271768001" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'SNOMEDCT\', \'domain\': \'bioontology.org\'})">271768001</a>]</span> <span class="mim-feature-ids hidden">[UMLS: <a href="https://bioportal.bioontology.org/search?q=C0423820&searchproperties=true" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'UMLS\', \'domain\': \'bioontology.org\'})">C0423820</a> HPO: <a href="https://hpo.jax.org/app/browse/term/HP:0001807" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'HPO\', \'domain\': \'hpo.jax.org\'})">HP:0001807</a>]</span> <span class="mim-feature-ids hidden">[HPO: <a href="https://hpo.jax.org/app/browse/term/HP:0001807" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'HPO\', \'domain\': \'hpo.jax.org\'})">HP:0001807</a>]</span><br /> -
|
|
Onycholysis <span class="mim-feature-ids hidden">[SNOMEDCT: <a href="https://purl.bioontology.org/ontology/SNOMEDCT/75789001" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'SNOMEDCT\', \'domain\': \'bioontology.org\'})">75789001</a>]</span> <span class="mim-feature-ids hidden">[ICD10CM: <a href="https://purl.bioontology.org/ontology/ICD10CM/L60.1" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'ICD10CM\', \'domain\': \'bioontology.org\'})">L60.1</a>]</span> <span class="mim-feature-ids hidden">[UMLS: <a href="https://bioportal.bioontology.org/search?q=C0085661&searchproperties=true" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'UMLS\', \'domain\': \'bioontology.org\'})">C0085661</a> HPO: <a href="https://hpo.jax.org/app/browse/term/HP:0001806" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'HPO\', \'domain\': \'hpo.jax.org\'})">HP:0001806</a>]</span> <span class="mim-feature-ids hidden">[HPO: <a href="https://hpo.jax.org/app/browse/term/HP:0001806" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'HPO\', \'domain\': \'hpo.jax.org\'})">HP:0001806</a>]</span><br />
|
|
|
|
</span>
|
|
</div>
|
|
</div>
|
|
|
|
|
|
<div>
|
|
<div>
|
|
<span class="h5 mim-font">
|
|
<em> Hair </em>
|
|
</span>
|
|
</div>
|
|
<div style="margin-left: 2em;">
|
|
<span class="mim-font">
|
|
|
|
- Hypotrichosis (scalp) <span class="mim-feature-ids hidden">[UMLS: <a href="https://bioportal.bioontology.org/search?q=C1857042&searchproperties=true" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'UMLS\', \'domain\': \'bioontology.org\'})">C1857042</a> HPO: <a href="https://hpo.jax.org/app/browse/term/HP:0002209" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'HPO\', \'domain\': \'hpo.jax.org\'})">HP:0002209</a>]</span> <span class="mim-feature-ids hidden">[SNOMEDCT: <a href="https://purl.bioontology.org/ontology/SNOMEDCT/53602002" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'SNOMEDCT\', \'domain\': \'bioontology.org\'})">53602002</a>, <a href="https://purl.bioontology.org/ontology/SNOMEDCT/56558005" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'SNOMEDCT\', \'domain\': \'bioontology.org\'})">56558005</a>, <a href="https://purl.bioontology.org/ontology/SNOMEDCT/1162675003" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'SNOMEDCT\', \'domain\': \'bioontology.org\'})">1162675003</a>]</span> <span class="mim-feature-ids hidden">[HPO: <a href="https://hpo.jax.org/app/browse/term/HP:0008070" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'HPO\', \'domain\': \'hpo.jax.org\'})">HP:0008070</a>]</span> <a href="https://elementsofmorphology.nih.gov/index.cgi?tid=25abd69e028ffccfdc206598d9b9c939" target="_blank" class="small mim-tip-eom" title="<img src="https://elementsofmorphology.nih.gov/images/terms/Eyebrow,Sparse-small.jpg"> <br/>Further Information: <a href="https://elementsofmorphology.nih.gov/index.cgi?tid=25abd69e028ffccfdc206598d9b9c939" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'EOM\', \'domain\': \'elementsofmorphology.nih.gov\'})">Elements of Morphology</a>"><span class="glyphicon glyphicon-user" aria-hidden="true"></span></a><br />
|
|
|
|
</span>
|
|
</div>
|
|
</div>
|
|
|
|
</div>
|
|
|
|
</div>
|
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<div>
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Squamous cell carcinoma <span class="mim-feature-ids hidden">[SNOMEDCT: <a href="https://purl.bioontology.org/ontology/SNOMEDCT/1162767002" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'SNOMEDCT\', \'domain\': \'bioontology.org\'})">1162767002</a>, <a href="https://purl.bioontology.org/ontology/SNOMEDCT/402815007" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'SNOMEDCT\', \'domain\': \'bioontology.org\'})">402815007</a>]</span> <span class="mim-feature-ids hidden">[UMLS: <a href="https://bioportal.bioontology.org/search?q=C0007137&searchproperties=true" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'UMLS\', \'domain\': \'bioontology.org\'})">C0007137</a> HPO: <a href="https://hpo.jax.org/app/browse/term/HP:0002860" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'HPO\', \'domain\': \'hpo.jax.org\'})">HP:0002860</a>]</span> <span class="mim-feature-ids hidden">[HPO: <a href="https://hpo.jax.org/app/browse/term/HP:0002860" target="_blank" onclick="gtag(\'event\', \'mim_outbound\', {\'name\': \'HPO\', \'domain\': \'hpo.jax.org\'})">HP:0002860</a>]</span><br />
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<strong> MOLECULAR BASIS </strong>
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- Caused by mutation in the wingless-type MMTV integration site family, member 10A gene (WNT10A, <a href="/entry/606268#0002">606268.0002</a>)<br />
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<span class="mim-tip-floating" qtip_title="<strong>Looking For More References?</strong>" qtip_text="Click the 'reference plus' icon <span class='glyphicon glyphicon-plus-sign'></span> at the end of each OMIM text paragraph to see more references related to the content of the preceding paragraph.">
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<p>A number sign (#) is used with this entry because of evidence that Schopf-Schulz-Passarge syndrome (SSPS) is caused by homozygous mutation in the WNT10A gene (<a href="/entry/606268">606268</a>) on chromosome 2q35.</p>
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<p>Schopf-Schulz-Passarge syndrome (SSPS) is an autosomal recessive disorder characterized by a constellation of multiple eyelid cysts, hypodontia, hypotrichosis, palmoplantar hyperkeratosis, and onychodystrophy (summary by <a href="#7" class="mim-tip-reference" title="Mallaiah, U., Dickinson, J. <strong>Bilateral multiple eyelid apocrine hidrocystomas and ectodermal dysplasia.</strong> Arch. Ophthal. 119: 1866-1867, 2001.[PubMed: <a href="https://pubmed.ncbi.nlm.nih.gov/11735807/" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">11735807</a>] [<a href="https://doi.org/10.1001/archopht.119.12.1866" target="_blank" onclick="gtag('event', 'mim_outbound', {'destination': 'Publisher'})">Full Text</a>]" pmid="11735807">Mallaiah and Dickinson, 2001</a>). <a href="https://pubmed.ncbi.nlm.nih.gov/?cmd=link&linkname=pubmed_pubmed&from_uid=11735807" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed Related', 'domain': 'pubmed.ncbi.nlm.nih.gov'})"><span class="glyphicon glyphicon-plus-sign mim-tip-hint" title="Click this 'reference-plus' icon to see articles related to this paragraph in PubMed."></span></a></p>
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<p><a href="#11" class="mim-tip-reference" title="Schopf, E., Schulz, H.-J., Passarge, E. <strong>Syndrome of cystic eyelids, palmo-plantar keratosis, hypodontia and hypotrichosis as a possible autosomal recessive trait.</strong> Birth Defects Orig. Art. Ser. VII(8): 219-221, 1971."None>Schopf et al. (1971)</a> described keratosis palmoplantaris with hypodontia, hypotrichosis, and cysts of the eyelids in sisters whose parents were first cousins. The deciduous teeth were lost early and the permanent dentition in 1 patient consisted only of 2 incisors and a molar. Palmoplantar keratosis and fragility of the nails began at about age 12. At age 25, head hair became sparse and body hair was lost completely. Cysts of both upper and lower eyelids were noted at age 60. The cysts were thought to be derived from the glands of Moll.</p><p><a href="#4" class="mim-tip-reference" title="Font, R. L., Stone, M. S., Schanzer, M. C., Lewis, R. A. <strong>Apocrine hidrocystomas of the lids, hypodontia, palmar-plantar hyperkeratosis, and onychodystrophy: a new variant of ectodermal dysplasia.</strong> Arch. Ophthal. 104: 1811-1813, 1986.[PubMed: <a href="https://pubmed.ncbi.nlm.nih.gov/2947556/" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">2947556</a>] [<a href="https://doi.org/10.1001/archopht.1986.01050240085045" target="_blank" onclick="gtag('event', 'mim_outbound', {'destination': 'Publisher'})">Full Text</a>]" pmid="2947556">Font et al. (1986)</a> reported a family with 3 affected brothers who showed manifestations fairly similar to those in the family reported by <a href="#11" class="mim-tip-reference" title="Schopf, E., Schulz, H.-J., Passarge, E. <strong>Syndrome of cystic eyelids, palmo-plantar keratosis, hypodontia and hypotrichosis as a possible autosomal recessive trait.</strong> Birth Defects Orig. Art. Ser. VII(8): 219-221, 1971."None>Schopf et al. (1971)</a>. However, whereas <a href="#11" class="mim-tip-reference" title="Schopf, E., Schulz, H.-J., Passarge, E. <strong>Syndrome of cystic eyelids, palmo-plantar keratosis, hypodontia and hypotrichosis as a possible autosomal recessive trait.</strong> Birth Defects Orig. Art. Ser. VII(8): 219-221, 1971."None>Schopf et al. (1971)</a> thought that their patients might have Papillon-Lefevre syndrome (<a href="/entry/245000">245000</a>), <a href="#4" class="mim-tip-reference" title="Font, R. L., Stone, M. S., Schanzer, M. C., Lewis, R. A. <strong>Apocrine hidrocystomas of the lids, hypodontia, palmar-plantar hyperkeratosis, and onychodystrophy: a new variant of ectodermal dysplasia.</strong> Arch. Ophthal. 104: 1811-1813, 1986.[PubMed: <a href="https://pubmed.ncbi.nlm.nih.gov/2947556/" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">2947556</a>] [<a href="https://doi.org/10.1001/archopht.1986.01050240085045" target="_blank" onclick="gtag('event', 'mim_outbound', {'destination': 'Publisher'})">Full Text</a>]" pmid="2947556">Font et al. (1986)</a> concluded that both families had a separate disorder, which is distinguished by the occurrence of apocrine hidrocystomas of the eyelid margins. <a href="https://pubmed.ncbi.nlm.nih.gov/?cmd=link&linkname=pubmed_pubmed&from_uid=2947556" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed Related', 'domain': 'pubmed.ncbi.nlm.nih.gov'})"><span class="glyphicon glyphicon-plus-sign mim-tip-hint" title="Click this 'reference-plus' icon to see articles related to this paragraph in PubMed."></span></a></p><p><a href="#8" class="mim-tip-reference" title="Monk, B. E., Pieris, S., Soni, V. <strong>Schopf-Schulz-Passarge syndrome.</strong> Brit. J. Derm. 127: 33-35, 1992.[PubMed: <a href="https://pubmed.ncbi.nlm.nih.gov/1637691/" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">1637691</a>] [<a href="https://doi.org/10.1111/j.1365-2133.1992.tb14822.x" target="_blank" onclick="gtag('event', 'mim_outbound', {'destination': 'Publisher'})">Full Text</a>]" pmid="1637691">Monk et al. (1992)</a> described an English family in which a 62-year-old woman and her 58-and 51-year-old brothers had what the authors referred to as the Schopf-Schulz-Passarge syndrome. They found previous reports of only 8 cases, including those of <a href="#2" class="mim-tip-reference" title="Burket, J. M., Burket, B. J., Burket, D. A. <strong>Eyelid cysts, hypodontia, and hypotrichosis.</strong> J. Am. Acad. Derm. 10: 922-925, 1984.[PubMed: <a href="https://pubmed.ncbi.nlm.nih.gov/6725680/" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">6725680</a>] [<a href="https://doi.org/10.1016/s0190-9622(84)80448-x" target="_blank" onclick="gtag('event', 'mim_outbound', {'destination': 'Publisher'})">Full Text</a>]" pmid="6725680">Burket et al. (1984)</a>, <a href="#9" class="mim-tip-reference" title="Nordin, H., Mansson, T., Svensson, A. <strong>Familial occurrence of eccrine tumours in a family with ectodermal dysplasia.</strong> Acta Derm. Venereol. 68: 523-530, 1988.[PubMed: <a href="https://pubmed.ncbi.nlm.nih.gov/2467492/" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">2467492</a>]" pmid="2467492">Nordin et al. (1988)</a>, and <a href="#10" class="mim-tip-reference" title="Perret, C. <strong>Schopf syndrome.</strong> Brit. J. Derm. 120: 131-132, 1989."None>Perret (1989)</a>. The woman reported by <a href="#8" class="mim-tip-reference" title="Monk, B. E., Pieris, S., Soni, V. <strong>Schopf-Schulz-Passarge syndrome.</strong> Brit. J. Derm. 127: 33-35, 1992.[PubMed: <a href="https://pubmed.ncbi.nlm.nih.gov/1637691/" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">1637691</a>] [<a href="https://doi.org/10.1111/j.1365-2133.1992.tb14822.x" target="_blank" onclick="gtag('event', 'mim_outbound', {'destination': 'Publisher'})">Full Text</a>]" pmid="1637691">Monk et al. (1992)</a> had thickening of the skin of her palms and soles for 15 years and had had a hypernephroma and a basal cell carcinoma of the skin. Abnormality of deciduous teeth had been noted on school examination and no secondary teeth had ever erupted. She had scanty scalp hair and the fingernails were hypoplastic. The eyelid margins of both eyes showed numerous cysts 1-2 mm in diameter, some clear and others showing a milky opacity. The brothers had essentially identical findings. One of the sibs had an unaffected son. <a href="https://pubmed.ncbi.nlm.nih.gov/?term=6725680+1637691+2467492" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed Related', 'domain': 'pubmed.ncbi.nlm.nih.gov'})"><span class="glyphicon glyphicon-plus-sign mim-tip-hint" title="Click this 'reference-plus' icon to see articles related to this paragraph in PubMed."></span></a></p><p><a href="#12" class="mim-tip-reference" title="Stevens, H. P., Kelsell, D. P., Bryant, S. P., Bishop, D. T., Spurr, N. K., Weissenbach, J., Marger, D., Marger, R. S., Leigh, I. M. <strong>Linkage of an American pedigree with palmoplantar keratoderma and malignancy (palmoplantar ectodermal dysplasia type III) to 17q24: literature survey and proposed updated classification of the keratodermas.</strong> Arch. Derm. 132: 640-651, 1996.[PubMed: <a href="https://pubmed.ncbi.nlm.nih.gov/8651714/" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">8651714</a>]" pmid="8651714">Stevens et al. (1996)</a> provided a comprehensive classification of the primary palmoplantar keratodermas. Where more than a single ectodermal structure was involved, as in this condition, they coined the term 'palmoplantar ectodermal dysplasia' to emphasize the generalized nature of the disorder. They further identified a total of 19 subtypes, the Schopf-Schultz-Passarge syndrome being type XIX. <a href="https://pubmed.ncbi.nlm.nih.gov/?cmd=link&linkname=pubmed_pubmed&from_uid=8651714" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed Related', 'domain': 'pubmed.ncbi.nlm.nih.gov'})"><span class="glyphicon glyphicon-plus-sign mim-tip-hint" title="Click this 'reference-plus' icon to see articles related to this paragraph in PubMed."></span></a></p><p><a href="#5" class="mim-tip-reference" title="Gorlin, R. J. <strong>Personal Communication.</strong> Minneapolis, Minn. 3/24/1997."None>Gorlin (1997)</a> concluded that eccrine tumors with ectodermal dysplasia as described by <a href="#9" class="mim-tip-reference" title="Nordin, H., Mansson, T., Svensson, A. <strong>Familial occurrence of eccrine tumours in a family with ectodermal dysplasia.</strong> Acta Derm. Venereol. 68: 523-530, 1988.[PubMed: <a href="https://pubmed.ncbi.nlm.nih.gov/2467492/" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">2467492</a>]" pmid="2467492">Nordin et al. (1988)</a> is in fact Schopf syndrome. Among the 8 children of normal parents, <a href="#9" class="mim-tip-reference" title="Nordin, H., Mansson, T., Svensson, A. <strong>Familial occurrence of eccrine tumours in a family with ectodermal dysplasia.</strong> Acta Derm. Venereol. 68: 523-530, 1988.[PubMed: <a href="https://pubmed.ncbi.nlm.nih.gov/2467492/" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">2467492</a>]" pmid="2467492">Nordin et al. (1988)</a> observed 2 brothers and a sister with ectodermal dysplasia manifested by hypodontia, onychodystrophy, trichodysplasia, and palmoplantar keratosis. In addition, a diffuse palmoplantar eccrine hyperplasia, as well as tumors and cysts of eccrine origin, was noted. The parents were not known to be consanguineous. All 3 affected sibs, in their 70s or 80s, had children, and 2 of them had grandchildren, all unaffected. Eccrine glands, also known as exocrine glands, include the sweat glands. Excessive sweating of the hands and feet was described. One of the patients had a cauliflower-like tumor on the heel, with secretions that stained the coverings of the heel. In later years the patients developed nodules on the bodies of the upper and lower eyelids. Eyelashes and eyebrows were sparse, but scalp and body hair was described as unremarkable. <a href="https://pubmed.ncbi.nlm.nih.gov/?cmd=link&linkname=pubmed_pubmed&from_uid=2467492" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed Related', 'domain': 'pubmed.ncbi.nlm.nih.gov'})"><span class="glyphicon glyphicon-plus-sign mim-tip-hint" title="Click this 'reference-plus' icon to see articles related to this paragraph in PubMed."></span></a></p><p><a href="#3" class="mim-tip-reference" title="Craigen, W. J., Levy, M. L., Lewis, R. A. <strong>Schopf-Schulz-Passarge syndrome with an unusual pattern of inheritance.</strong> Am. J. Med. Genet. 71: 186-188, 1997.[PubMed: <a href="https://pubmed.ncbi.nlm.nih.gov/9217219/" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">9217219</a>] [<a href="https://doi.org/10.1002/(sici)1096-8628(19970808)71:2<186::aid-ajmg12>3.0.co;2-a" target="_blank" onclick="gtag('event', 'mim_outbound', {'destination': 'Publisher'})">Full Text</a>]" pmid="9217219">Craigen et al. (1997)</a> stated that 10 cases of this syndrome had been reported; all were compatible with autosomal recessive inheritance except for the family reported by <a href="#6" class="mim-tip-reference" title="Kuster, W., Hammerstein, W. <strong>Das Schopf-Syndrom: Klinische, genetische und lipidbiochemische Untersuchungen.</strong> Hautarzt 43: 763-766, 1992.[PubMed: <a href="https://pubmed.ncbi.nlm.nih.gov/1473970/" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">1473970</a>]" pmid="1473970">Kuster and Hammerstein (1992)</a>, which exhibited dominant inheritance. They reported a family in which 3 full sibs and 1 half sib, all males, had Schopf-Schultz-Passarge syndrome. The half sibs shared a father. The 4 affected males had a total of 8 sons and 4 daughters, none of whom were affected. <a href="#3" class="mim-tip-reference" title="Craigen, W. J., Levy, M. L., Lewis, R. A. <strong>Schopf-Schulz-Passarge syndrome with an unusual pattern of inheritance.</strong> Am. J. Med. Genet. 71: 186-188, 1997.[PubMed: <a href="https://pubmed.ncbi.nlm.nih.gov/9217219/" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">9217219</a>] [<a href="https://doi.org/10.1002/(sici)1096-8628(19970808)71:2<186::aid-ajmg12>3.0.co;2-a" target="_blank" onclick="gtag('event', 'mim_outbound', {'destination': 'Publisher'})">Full Text</a>]" pmid="9217219">Craigen et al. (1997)</a> presented photographs of the eyelid cysts, nail dystrophy, and dry erythematous palm of 1 of the patients. <a href="https://pubmed.ncbi.nlm.nih.gov/?term=1473970+9217219" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed Related', 'domain': 'pubmed.ncbi.nlm.nih.gov'})"><span class="glyphicon glyphicon-plus-sign mim-tip-hint" title="Click this 'reference-plus' icon to see articles related to this paragraph in PubMed."></span></a></p><p><a href="#7" class="mim-tip-reference" title="Mallaiah, U., Dickinson, J. <strong>Bilateral multiple eyelid apocrine hidrocystomas and ectodermal dysplasia.</strong> Arch. Ophthal. 119: 1866-1867, 2001.[PubMed: <a href="https://pubmed.ncbi.nlm.nih.gov/11735807/" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">11735807</a>] [<a href="https://doi.org/10.1001/archopht.119.12.1866" target="_blank" onclick="gtag('event', 'mim_outbound', {'destination': 'Publisher'})">Full Text</a>]" pmid="11735807">Mallaiah and Dickinson (2001)</a> presented a photo essay of eyelid cysts, plantar hyperkeratosis, dysplastic toenails, and adontia in sibs in their sixth decade who had become symptomatic in their third decade. Histopathology of apocrine hidrocystomas was included. Additionally, the authors commented that simple excision of eyelid cysts was rarely adequate and that en bloc excision of the anterior lamella of the eyelids might be beneficial. <a href="https://pubmed.ncbi.nlm.nih.gov/?cmd=link&linkname=pubmed_pubmed&from_uid=11735807" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed Related', 'domain': 'pubmed.ncbi.nlm.nih.gov'})"><span class="glyphicon glyphicon-plus-sign mim-tip-hint" title="Click this 'reference-plus' icon to see articles related to this paragraph in PubMed."></span></a></p>
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<p>The transmission pattern of SPSS in the family reported by <a href="#1" class="mim-tip-reference" title="Bohring, A., Stamm, T., Spaich, C., Haase, C., Spree, K., Hehr, U., Hoffmann, M., Ledig, S., Sel, S., Wieacker, P., Ropke, A. <strong>WNT10A mutations are a frequent cause of a broad spectrum of ectodermal dysplasias with sex-biased manifestation pattern in heterozygotes.</strong> Am. J. Hum. Genet. 85: 97-105, 2009.[PubMed: <a href="https://pubmed.ncbi.nlm.nih.gov/19559398/" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">19559398</a>, <a href="https://www.ncbi.nlm.nih.gov/pmc/?term=19559398[PMID]&report=imagesdocsum" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed Image', 'domain': 'ncbi.nlm.nih.gov'})">images</a>] [<a href="https://doi.org/10.1016/j.ajhg.2009.06.001" target="_blank" onclick="gtag('event', 'mim_outbound', {'destination': 'Publisher'})">Full Text</a>]" pmid="19559398">Bohring et al. (2009)</a> was consistent with autosomal recessive inheritance. <a href="https://pubmed.ncbi.nlm.nih.gov/?cmd=link&linkname=pubmed_pubmed&from_uid=19559398" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed Related', 'domain': 'pubmed.ncbi.nlm.nih.gov'})"><span class="glyphicon glyphicon-plus-sign mim-tip-hint" title="Click this 'reference-plus' icon to see articles related to this paragraph in PubMed."></span></a></p>
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<p>In a female patient with cysts of the eyelids in addition to hypodontia, hypotrichosis, hyperhidrosis, palmoplantar hyperkeratosis, and dystrophic nails, who was negative for mutation in the ectodysplasin-A gene (EDA; <a href="/entry/300451">300451</a>), <a href="#1" class="mim-tip-reference" title="Bohring, A., Stamm, T., Spaich, C., Haase, C., Spree, K., Hehr, U., Hoffmann, M., Ledig, S., Sel, S., Wieacker, P., Ropke, A. <strong>WNT10A mutations are a frequent cause of a broad spectrum of ectodermal dysplasias with sex-biased manifestation pattern in heterozygotes.</strong> Am. J. Hum. Genet. 85: 97-105, 2009.[PubMed: <a href="https://pubmed.ncbi.nlm.nih.gov/19559398/" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">19559398</a>, <a href="https://www.ncbi.nlm.nih.gov/pmc/?term=19559398[PMID]&report=imagesdocsum" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed Image', 'domain': 'ncbi.nlm.nih.gov'})">images</a>] [<a href="https://doi.org/10.1016/j.ajhg.2009.06.001" target="_blank" onclick="gtag('event', 'mim_outbound', {'destination': 'Publisher'})">Full Text</a>]" pmid="19559398">Bohring et al. (2009)</a> identified homozygosity for a nonsense mutation in the WNT10A gene (C107X; <a href="/entry/606268#0002">606268.0002</a>). They also found homozygous or compound heterozygous WNT10A mutations in 8 patients with odontoonychodermal dysplasia (OODD; <a href="/entry/257980">257980</a>) but no eyelid cysts. The proband with SSPS had 2 brothers who were heterozygous for the mutation and exhibited dental anomalies: one had agenesis of both lower central incisors, and the other had anomalies of the upper lateral permanent incisors. The proband was also diagnosed with a porocarcinoma of the left heel. <a href="https://pubmed.ncbi.nlm.nih.gov/?cmd=link&linkname=pubmed_pubmed&from_uid=19559398" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed Related', 'domain': 'pubmed.ncbi.nlm.nih.gov'})"><span class="glyphicon glyphicon-plus-sign mim-tip-hint" title="Click this 'reference-plus' icon to see articles related to this paragraph in PubMed."></span></a></p>
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<a id="Bohring2009" class="mim-anchor"></a>
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Bohring, A., Stamm, T., Spaich, C., Haase, C., Spree, K., Hehr, U., Hoffmann, M., Ledig, S., Sel, S., Wieacker, P., Ropke, A.
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<strong>WNT10A mutations are a frequent cause of a broad spectrum of ectodermal dysplasias with sex-biased manifestation pattern in heterozygotes.</strong>
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Am. J. Hum. Genet. 85: 97-105, 2009.
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[PubMed: <a href="https://pubmed.ncbi.nlm.nih.gov/19559398/" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">19559398</a>, <a href="https://www.ncbi.nlm.nih.gov/pmc/?term=19559398[PMID]&report=imagesdocsum" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed Image', 'domain': 'ncbi.nlm.nih.gov'})">images</a>, <a href="https://pubmed.ncbi.nlm.nih.gov/?cmd=link&linkname=pubmed_pubmed&from_uid=19559398" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed Related', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">related citations</a>]
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[<a href="https://doi.org/10.1016/j.ajhg.2009.06.001" target="_blank">Full Text</a>]
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<a id="Burket1984" class="mim-anchor"></a>
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Burket, J. M., Burket, B. J., Burket, D. A.
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<strong>Eyelid cysts, hypodontia, and hypotrichosis.</strong>
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J. Am. Acad. Derm. 10: 922-925, 1984.
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[PubMed: <a href="https://pubmed.ncbi.nlm.nih.gov/6725680/" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">6725680</a>, <a href="https://pubmed.ncbi.nlm.nih.gov/?cmd=link&linkname=pubmed_pubmed&from_uid=6725680" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed Related', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">related citations</a>]
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[<a href="https://doi.org/10.1016/s0190-9622(84)80448-x" target="_blank">Full Text</a>]
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<a id="Craigen1997" class="mim-anchor"></a>
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<p class="mim-text-font">
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Craigen, W. J., Levy, M. L., Lewis, R. A.
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<strong>Schopf-Schulz-Passarge syndrome with an unusual pattern of inheritance.</strong>
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Am. J. Med. Genet. 71: 186-188, 1997.
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[PubMed: <a href="https://pubmed.ncbi.nlm.nih.gov/9217219/" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">9217219</a>, <a href="https://pubmed.ncbi.nlm.nih.gov/?cmd=link&linkname=pubmed_pubmed&from_uid=9217219" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed Related', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">related citations</a>]
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[<a href="https://doi.org/10.1002/(sici)1096-8628(19970808)71:2<186::aid-ajmg12>3.0.co;2-a" target="_blank">Full Text</a>]
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<a id="Font1986" class="mim-anchor"></a>
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Font, R. L., Stone, M. S., Schanzer, M. C., Lewis, R. A.
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<strong>Apocrine hidrocystomas of the lids, hypodontia, palmar-plantar hyperkeratosis, and onychodystrophy: a new variant of ectodermal dysplasia.</strong>
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Arch. Ophthal. 104: 1811-1813, 1986.
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[PubMed: <a href="https://pubmed.ncbi.nlm.nih.gov/2947556/" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">2947556</a>, <a href="https://pubmed.ncbi.nlm.nih.gov/?cmd=link&linkname=pubmed_pubmed&from_uid=2947556" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed Related', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">related citations</a>]
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[<a href="https://doi.org/10.1001/archopht.1986.01050240085045" target="_blank">Full Text</a>]
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Gorlin, R. J.
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<strong>Personal Communication.</strong>
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Minneapolis, Minn. 3/24/1997.
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Kuster, W., Hammerstein, W.
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<strong>Das Schopf-Syndrom: Klinische, genetische und lipidbiochemische Untersuchungen.</strong>
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Hautarzt 43: 763-766, 1992.
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[PubMed: <a href="https://pubmed.ncbi.nlm.nih.gov/1473970/" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">1473970</a>, <a href="https://pubmed.ncbi.nlm.nih.gov/?cmd=link&linkname=pubmed_pubmed&from_uid=1473970" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed Related', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">related citations</a>]
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Mallaiah, U., Dickinson, J.
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<strong>Bilateral multiple eyelid apocrine hidrocystomas and ectodermal dysplasia.</strong>
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Arch. Ophthal. 119: 1866-1867, 2001.
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[PubMed: <a href="https://pubmed.ncbi.nlm.nih.gov/11735807/" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">11735807</a>, <a href="https://pubmed.ncbi.nlm.nih.gov/?cmd=link&linkname=pubmed_pubmed&from_uid=11735807" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed Related', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">related citations</a>]
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[<a href="https://doi.org/10.1001/archopht.119.12.1866" target="_blank">Full Text</a>]
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<a id="Monk1992" class="mim-anchor"></a>
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Monk, B. E., Pieris, S., Soni, V.
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<strong>Schopf-Schulz-Passarge syndrome.</strong>
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Brit. J. Derm. 127: 33-35, 1992.
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[PubMed: <a href="https://pubmed.ncbi.nlm.nih.gov/1637691/" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">1637691</a>, <a href="https://pubmed.ncbi.nlm.nih.gov/?cmd=link&linkname=pubmed_pubmed&from_uid=1637691" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed Related', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">related citations</a>]
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[<a href="https://doi.org/10.1111/j.1365-2133.1992.tb14822.x" target="_blank">Full Text</a>]
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Nordin, H., Mansson, T., Svensson, A.
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<strong>Familial occurrence of eccrine tumours in a family with ectodermal dysplasia.</strong>
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Acta Derm. Venereol. 68: 523-530, 1988.
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[PubMed: <a href="https://pubmed.ncbi.nlm.nih.gov/2467492/" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">2467492</a>, <a href="https://pubmed.ncbi.nlm.nih.gov/?cmd=link&linkname=pubmed_pubmed&from_uid=2467492" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed Related', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">related citations</a>]
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Perret, C.
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<strong>Schopf syndrome.</strong>
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Brit. J. Derm. 120: 131-132, 1989.
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Schopf, E., Schulz, H.-J., Passarge, E.
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<strong>Syndrome of cystic eyelids, palmo-plantar keratosis, hypodontia and hypotrichosis as a possible autosomal recessive trait.</strong>
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Stevens, H. P., Kelsell, D. P., Bryant, S. P., Bishop, D. T., Spurr, N. K., Weissenbach, J., Marger, D., Marger, R. S., Leigh, I. M.
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<strong>Linkage of an American pedigree with palmoplantar keratoderma and malignancy (palmoplantar ectodermal dysplasia type III) to 17q24: literature survey and proposed updated classification of the keratodermas.</strong>
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Arch. Derm. 132: 640-651, 1996.
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[PubMed: <a href="https://pubmed.ncbi.nlm.nih.gov/8651714/" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">8651714</a>, <a href="https://pubmed.ncbi.nlm.nih.gov/?cmd=link&linkname=pubmed_pubmed&from_uid=8651714" target="_blank" onclick="gtag('event', 'mim_outbound', {'name': 'PubMed Related', 'domain': 'pubmed.ncbi.nlm.nih.gov'})">related citations</a>]
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<div class="col-lg-6 col-md-6 col-sm-6 col-xs-6">
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<span class="mim-text-font">
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Anne M. Stumpf - updated : 03/20/2020
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Marla J. F. O'Neill - updated : 7/30/2009<br>Jane Kelly - updated : 4/19/2002<br>Victor A. McKusick - updated : 8/18/1997<br>Victor A. McKusick - edited : 5/9/1997
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Victor A. McKusick : 12/22/1988
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<a href="#mimCollapseEditHistory" role="button" data-toggle="collapse"> Edit History: </a>
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carol : 11/17/2022
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alopez : 03/20/2020<br>carol : 05/08/2017<br>carol : 01/04/2017<br>alopez : 09/13/2016<br>carol : 06/08/2012<br>wwang : 8/19/2009<br>terry : 7/30/2009<br>mgross : 3/17/2004<br>mgross : 4/19/2002<br>terry : 2/2/2000<br>dholmes : 10/1/1997<br>terry : 8/21/1997<br>terry : 8/18/1997<br>jamie : 6/3/1997<br>mark : 5/9/1997<br>mark : 5/9/1997<br>mark : 5/6/1997<br>terry : 5/2/1997<br>mimadm : 4/14/1994<br>supermim : 3/16/1992<br>supermim : 3/20/1990<br>ddp : 10/26/1989<br>carol : 12/22/1988
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<strong>#</strong> 224750
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SCHOPF-SCHULZ-PASSARGE SYNDROME; SSPS
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KERATOSIS PALMOPLANTARIS WITH CYSTIC EYELIDS, HYPODONTIA, AND HYPOTRICHOSIS<br />
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ECCRINE TUMORS WITH ECTODERMAL DYSPLASIA
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<strong>SNOMEDCT:</strong> 700062000;
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<strong>ORPHA:</strong> 50944;
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<strong>DO:</strong> 0111647;
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<strong>Phenotype-Gene Relationships</strong>
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<table class="table table-bordered table-condensed small mim-table-padding">
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Location
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Phenotype
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Phenotype <br /> MIM number
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Inheritance
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Phenotype <br /> mapping key
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Gene/Locus
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Gene/Locus <br /> MIM number
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<span class="mim-font">
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2q35
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<span class="mim-font">
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Schopf-Schulz-Passarge syndrome
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<span class="mim-font">
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224750
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Autosomal recessive
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<span class="mim-font">
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3
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WNT10A
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606268
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<strong>TEXT</strong>
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<p>A number sign (#) is used with this entry because of evidence that Schopf-Schulz-Passarge syndrome (SSPS) is caused by homozygous mutation in the WNT10A gene (606268) on chromosome 2q35.</p>
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<strong>Description</strong>
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<p>Schopf-Schulz-Passarge syndrome (SSPS) is an autosomal recessive disorder characterized by a constellation of multiple eyelid cysts, hypodontia, hypotrichosis, palmoplantar hyperkeratosis, and onychodystrophy (summary by Mallaiah and Dickinson, 2001). </p>
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<strong>Clinical Features</strong>
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<p>Schopf et al. (1971) described keratosis palmoplantaris with hypodontia, hypotrichosis, and cysts of the eyelids in sisters whose parents were first cousins. The deciduous teeth were lost early and the permanent dentition in 1 patient consisted only of 2 incisors and a molar. Palmoplantar keratosis and fragility of the nails began at about age 12. At age 25, head hair became sparse and body hair was lost completely. Cysts of both upper and lower eyelids were noted at age 60. The cysts were thought to be derived from the glands of Moll.</p><p>Font et al. (1986) reported a family with 3 affected brothers who showed manifestations fairly similar to those in the family reported by Schopf et al. (1971). However, whereas Schopf et al. (1971) thought that their patients might have Papillon-Lefevre syndrome (245000), Font et al. (1986) concluded that both families had a separate disorder, which is distinguished by the occurrence of apocrine hidrocystomas of the eyelid margins. </p><p>Monk et al. (1992) described an English family in which a 62-year-old woman and her 58-and 51-year-old brothers had what the authors referred to as the Schopf-Schulz-Passarge syndrome. They found previous reports of only 8 cases, including those of Burket et al. (1984), Nordin et al. (1988), and Perret (1989). The woman reported by Monk et al. (1992) had thickening of the skin of her palms and soles for 15 years and had had a hypernephroma and a basal cell carcinoma of the skin. Abnormality of deciduous teeth had been noted on school examination and no secondary teeth had ever erupted. She had scanty scalp hair and the fingernails were hypoplastic. The eyelid margins of both eyes showed numerous cysts 1-2 mm in diameter, some clear and others showing a milky opacity. The brothers had essentially identical findings. One of the sibs had an unaffected son. </p><p>Stevens et al. (1996) provided a comprehensive classification of the primary palmoplantar keratodermas. Where more than a single ectodermal structure was involved, as in this condition, they coined the term 'palmoplantar ectodermal dysplasia' to emphasize the generalized nature of the disorder. They further identified a total of 19 subtypes, the Schopf-Schultz-Passarge syndrome being type XIX. </p><p>Gorlin (1997) concluded that eccrine tumors with ectodermal dysplasia as described by Nordin et al. (1988) is in fact Schopf syndrome. Among the 8 children of normal parents, Nordin et al. (1988) observed 2 brothers and a sister with ectodermal dysplasia manifested by hypodontia, onychodystrophy, trichodysplasia, and palmoplantar keratosis. In addition, a diffuse palmoplantar eccrine hyperplasia, as well as tumors and cysts of eccrine origin, was noted. The parents were not known to be consanguineous. All 3 affected sibs, in their 70s or 80s, had children, and 2 of them had grandchildren, all unaffected. Eccrine glands, also known as exocrine glands, include the sweat glands. Excessive sweating of the hands and feet was described. One of the patients had a cauliflower-like tumor on the heel, with secretions that stained the coverings of the heel. In later years the patients developed nodules on the bodies of the upper and lower eyelids. Eyelashes and eyebrows were sparse, but scalp and body hair was described as unremarkable. </p><p>Craigen et al. (1997) stated that 10 cases of this syndrome had been reported; all were compatible with autosomal recessive inheritance except for the family reported by Kuster and Hammerstein (1992), which exhibited dominant inheritance. They reported a family in which 3 full sibs and 1 half sib, all males, had Schopf-Schultz-Passarge syndrome. The half sibs shared a father. The 4 affected males had a total of 8 sons and 4 daughters, none of whom were affected. Craigen et al. (1997) presented photographs of the eyelid cysts, nail dystrophy, and dry erythematous palm of 1 of the patients. </p><p>Mallaiah and Dickinson (2001) presented a photo essay of eyelid cysts, plantar hyperkeratosis, dysplastic toenails, and adontia in sibs in their sixth decade who had become symptomatic in their third decade. Histopathology of apocrine hidrocystomas was included. Additionally, the authors commented that simple excision of eyelid cysts was rarely adequate and that en bloc excision of the anterior lamella of the eyelids might be beneficial. </p>
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<span class="mim-font">
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<strong>Inheritance</strong>
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<p>The transmission pattern of SPSS in the family reported by Bohring et al. (2009) was consistent with autosomal recessive inheritance. </p>
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<strong>Molecular Genetics</strong>
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<p>In a female patient with cysts of the eyelids in addition to hypodontia, hypotrichosis, hyperhidrosis, palmoplantar hyperkeratosis, and dystrophic nails, who was negative for mutation in the ectodysplasin-A gene (EDA; 300451), Bohring et al. (2009) identified homozygosity for a nonsense mutation in the WNT10A gene (C107X; 606268.0002). They also found homozygous or compound heterozygous WNT10A mutations in 8 patients with odontoonychodermal dysplasia (OODD; 257980) but no eyelid cysts. The proband with SSPS had 2 brothers who were heterozygous for the mutation and exhibited dental anomalies: one had agenesis of both lower central incisors, and the other had anomalies of the upper lateral permanent incisors. The proband was also diagnosed with a porocarcinoma of the left heel. </p>
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<span class="mim-font">
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<strong>REFERENCES</strong>
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<p class="mim-text-font">
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Bohring, A., Stamm, T., Spaich, C., Haase, C., Spree, K., Hehr, U., Hoffmann, M., Ledig, S., Sel, S., Wieacker, P., Ropke, A.
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<strong>WNT10A mutations are a frequent cause of a broad spectrum of ectodermal dysplasias with sex-biased manifestation pattern in heterozygotes.</strong>
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Am. J. Hum. Genet. 85: 97-105, 2009.
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[PubMed: 19559398]
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[Full Text: https://doi.org/10.1016/j.ajhg.2009.06.001]
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<p class="mim-text-font">
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Burket, J. M., Burket, B. J., Burket, D. A.
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<strong>Eyelid cysts, hypodontia, and hypotrichosis.</strong>
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J. Am. Acad. Derm. 10: 922-925, 1984.
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[PubMed: 6725680]
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[Full Text: https://doi.org/10.1016/s0190-9622(84)80448-x]
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<li>
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<p class="mim-text-font">
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Craigen, W. J., Levy, M. L., Lewis, R. A.
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<strong>Schopf-Schulz-Passarge syndrome with an unusual pattern of inheritance.</strong>
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Am. J. Med. Genet. 71: 186-188, 1997.
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[PubMed: 9217219]
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[Full Text: https://doi.org/10.1002/(sici)1096-8628(19970808)71:2<186::aid-ajmg12>3.0.co;2-a]
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<p class="mim-text-font">
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Font, R. L., Stone, M. S., Schanzer, M. C., Lewis, R. A.
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<strong>Apocrine hidrocystomas of the lids, hypodontia, palmar-plantar hyperkeratosis, and onychodystrophy: a new variant of ectodermal dysplasia.</strong>
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Arch. Ophthal. 104: 1811-1813, 1986.
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[PubMed: 2947556]
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[Full Text: https://doi.org/10.1001/archopht.1986.01050240085045]
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<p class="mim-text-font">
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Gorlin, R. J.
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<strong>Personal Communication.</strong>
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Minneapolis, Minn. 3/24/1997.
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<p class="mim-text-font">
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Kuster, W., Hammerstein, W.
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<strong>Das Schopf-Syndrom: Klinische, genetische und lipidbiochemische Untersuchungen.</strong>
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Hautarzt 43: 763-766, 1992.
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[PubMed: 1473970]
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<li>
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<p class="mim-text-font">
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Mallaiah, U., Dickinson, J.
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<strong>Bilateral multiple eyelid apocrine hidrocystomas and ectodermal dysplasia.</strong>
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Arch. Ophthal. 119: 1866-1867, 2001.
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[PubMed: 11735807]
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[Full Text: https://doi.org/10.1001/archopht.119.12.1866]
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<p class="mim-text-font">
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Monk, B. E., Pieris, S., Soni, V.
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<strong>Schopf-Schulz-Passarge syndrome.</strong>
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Brit. J. Derm. 127: 33-35, 1992.
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[PubMed: 1637691]
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[Full Text: https://doi.org/10.1111/j.1365-2133.1992.tb14822.x]
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<p class="mim-text-font">
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Nordin, H., Mansson, T., Svensson, A.
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<strong>Familial occurrence of eccrine tumours in a family with ectodermal dysplasia.</strong>
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Acta Derm. Venereol. 68: 523-530, 1988.
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[PubMed: 2467492]
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<p class="mim-text-font">
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Perret, C.
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<strong>Schopf syndrome.</strong>
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Brit. J. Derm. 120: 131-132, 1989.
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</p>
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</li>
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<li>
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<p class="mim-text-font">
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Schopf, E., Schulz, H.-J., Passarge, E.
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<strong>Syndrome of cystic eyelids, palmo-plantar keratosis, hypodontia and hypotrichosis as a possible autosomal recessive trait.</strong>
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Birth Defects Orig. Art. Ser. VII(8): 219-221, 1971.
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<p class="mim-text-font">
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Stevens, H. P., Kelsell, D. P., Bryant, S. P., Bishop, D. T., Spurr, N. K., Weissenbach, J., Marger, D., Marger, R. S., Leigh, I. M.
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<strong>Linkage of an American pedigree with palmoplantar keratoderma and malignancy (palmoplantar ectodermal dysplasia type III) to 17q24: literature survey and proposed updated classification of the keratodermas.</strong>
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Arch. Derm. 132: 640-651, 1996.
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[PubMed: 8651714]
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</ol>
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<div>
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<div class="row">
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<div class="col-lg-1 col-md-1 col-sm-2 col-xs-2">
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<span class="text-nowrap mim-text-font">
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Contributors:
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</span>
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</div>
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<div class="col-lg-6 col-md-6 col-sm-6 col-xs-6">
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<span class="mim-text-font">
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Anne M. Stumpf - updated : 03/20/2020<br>Marla J. F. O'Neill - updated : 7/30/2009<br>Jane Kelly - updated : 4/19/2002<br>Victor A. McKusick - updated : 8/18/1997<br>Victor A. McKusick - edited : 5/9/1997
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</span>
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Victor A. McKusick : 12/22/1988
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carol : 11/17/2022<br>alopez : 03/20/2020<br>carol : 05/08/2017<br>carol : 01/04/2017<br>alopez : 09/13/2016<br>carol : 06/08/2012<br>wwang : 8/19/2009<br>terry : 7/30/2009<br>mgross : 3/17/2004<br>mgross : 4/19/2002<br>terry : 2/2/2000<br>dholmes : 10/1/1997<br>terry : 8/21/1997<br>terry : 8/18/1997<br>jamie : 6/3/1997<br>mark : 5/9/1997<br>mark : 5/9/1997<br>mark : 5/6/1997<br>terry : 5/2/1997<br>mimadm : 4/14/1994<br>supermim : 3/16/1992<br>supermim : 3/20/1990<br>ddp : 10/26/1989<br>carol : 12/22/1988
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