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<meta name="keywords" content="C0334082, disease or syndrome, epidermal naevus, epidermal nevi, epidermal nevus, epidermal nevus, somatic, epithelial nevus, fgfr3, hras, nevus sebaceous, nevus sebaceous or woolly hair nevus, somatic, nevus sebaceous or wooly hair nevus, somatic, nevus, epidermal, nevus, epidermal, somatic, nevus, keratinocytic, nonepidermolytic, nevus, woolly hair, nevus, wooly hair, nonepidermolytic keratinocytic nevus, nras, pik3ca, autosomal dominant, autosomal recessive, birth defects, chromosomal disease, chromosome, clinical features, clinical findings, clinical genetics, clinical recommendations, clinvar, congenital chromosomal disease, consumer genetic resources, cytogenetic location, disease characteristics, disease definitions, disease descriptions, disease ontology, disease synonyms, disease vocabulary, dysmorphology, entrez, familial disease, gene, gene-disease relationship, genereviews, genetic disease, genetic disorder, genetic terminology, genetic testing registry, genetics home reference, genomic disease, gtr, hereditary disease, heritable disease, hpo, human phenotype ontology, inherited disease, management guidelines, maternal inheritance, medgen, medical genetics, medical subject headings, mesh, mitochondrial inheritance, mode of inheritance, national center for biotechnology information, national institutes of health, national library of medicine, ncbi, nih, nlm, omim, ordo, orphanet, paternal inheritance, phenome, position statements, professional practice guidelines, rare disease, reference sequence, refseq, snomed ct, syndrome, undiagnosed diseases, x-linked recessive" /><meta name="description" content="PIK3CA-related overgrowth spectrum (PROS) encompasses a range of clinical findings in which the core features are congenital or early-childhood onset of segmental/focal overgrowth with or without cellular dysplasia. Prior to the identification of PIK3CA as the causative gene, PROS was separated into distinct clinical syndromes based on the tissues and/or organs involved (e.g., MCAP [megalencephaly-capillary malformation] syndrome and CLOVES [congenital lipomatous asymmetric overgrowth of the trunk, lymphatic, capillary, venous, and combined-type vascular malformations, epidermal nevi, skeletal and spinal anomalies] syndrome). The predominant areas of overgrowth include the brain, limbs (including fingers and toes), trunk (including abdomen and chest), and face, all usually in an asymmetric distribution. Generalized brain overgrowth may be accompanied by secondary overgrowth of specific brain structures resulting in ventriculomegaly, a markedly thick corpus callosum, and cerebellar tonsillar ectopia with crowding of the posterior fossa. Vascular malformations may include capillary, venous, and less frequently, arterial or mixed (capillary-lymphatic-venous or arteriovenous) malformations. Lymphatic malformations may be in various locations (internal and/or external) and can cause various clinical issues, including swelling, pain, and occasionally localized bleeding secondary to trauma. Lipomatous overgrowth may occur ipsilateral or contralateral to a vascular malformation, if present. The degree of intellectual disability appears to be mostly related to the presence and severity of seizures, cortical dysplasia (e.g., polymicrogyria), and hydrocephalus. Many children have feeding difficulties that are often multifactorial in nature. Endocrine issues affect a small number of individuals and most commonly include hypoglycemia (largely hypoinsulinemic hypoketotic hypoglycemia), hypothyroidism, and growth hormone deficiency." /><meta name="robots" content="index,nofollow,noarchive" />
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<!--
UID=83106
ConceptID=C0334082
-->
<!--imgCountBooks = 4--><div class="ncbi_carousel" data-ncbicarousel-config="imageWidth:'100px',numItemsVisible:2,toggler:false"><div class="nc_header"><span class="img_strip_title">Images (4)</span></div><div class="nc_content"><div class="nc_item"><a class="figpopup"><img alt="Image from GeneReviews" src="/books/NBK153722/bin/pik3ca-overgrowth-Image001.gif" src-large="/books/NBK153722/bin/pik3ca-overgrowth-Image001.jpg" /></a><br /><a href="/books/NBK153722/figure/pik3ca-overgrowth.F1/" title="GeneReviews" target="_blank">details</a></div><div class="nc_item"><a class="figpopup"><img alt="Image from GeneReviews" src="/books/NBK153722/bin/pik3ca-overgrowth-Image002.gif" src-large="/books/NBK153722/bin/pik3ca-overgrowth-Image002.jpg" /></a><br /><a href="/books/NBK153722/figure/pik3ca-overgrowth.F2/" title="GeneReviews" target="_blank">details</a></div><div class="nc_item"><a class="figpopup"><img alt="Image from GeneReviews" src="/books/NBK153722/bin/pik3ca-overgrowth-Image003.gif" src-large="/books/NBK153722/bin/pik3ca-overgrowth-Image003.jpg" /></a><br /><a href="/books/NBK153722/figure/pik3ca-overgrowth.F3/" title="GeneReviews" target="_blank">details</a></div><div class="nc_item"><a class="figpopup"><img alt="Image from GeneReviews" src="/books/NBK153722/bin/pik3ca-overgrowth-Image004.gif" src-large="/books/NBK153722/bin/pik3ca-overgrowth-Image004.jpg" /></a><br /><a href="/books/NBK153722/figure/pik3ca-overgrowth.F4/" title="GeneReviews" target="_blank">details</a></div></div></div><h1 class="medgenTitle"><div class="MedGenTitleText">Epidermal nevus</div></h1><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>83106</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C0334082</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div><table class="medgenTable"><tbody><tr><td>Synonyms:</td>
<td>Nevus, epidermal, somatic; NEVUS, KERATINOCYTIC, NONEPIDERMOLYTIC</td></tr>
<tr><td><span class="bold">SNOMED CT: </span></td>
<td>Epidermal nevus (239107007); Epidermal nevus (25201003); Epithelial nevus (25201003)</td></tr>
<tr><td colspan="2" class="small"> </td></tr><tr><td><a class="help jig-ncbi-popper" data-jig="ncbipopper" href="#target-gene-loc">Genes (locations):<img class="pulldown" src="//static.pubmed.gov/portal/portal3rc.fcgi/4223267/img/4204968" /></a><div class="display-none" id="target-gene-loc">
Gene(s) directly associated with<br />
this condition or phenotype.</div></td>
<td><a target="_blank" title="FGFR3 - ID: 2261 - NCBI Gene" href="/gene/2261" class="medgenPMinfo">FGFR3</a> (4p16.3); <a target="_blank" title="HRAS - ID: 3265 - NCBI Gene" href="/gene/3265" class="medgenPMinfo">HRAS</a> (11p15.5); <a target="_blank" title="NRAS - ID: 4893 - NCBI Gene" href="/gene/4893" class="medgenPMinfo">NRAS</a> (1p13.2); <a target="_blank" title="PIK3CA - ID: 5290 - NCBI Gene" href="/gene/5290" class="medgenPMinfo">PIK3CA</a> (3q26.32)</td></tr>
<tr><td colspan="2" class="small"> </td></tr><tr><td>HPO:</td>
<td><a target="_blank" title="Human Phenotype Ontology" href="https://hpo.jax.org/app/browse/term/HP:0010816">HP:0010816</a></td></tr>
<tr><td>Monarch Initiative:</td>
<td><a href="https://monarchinitiative.org/disease/MONDO:0008093" target="_blank">MONDO:0008093</a></td></tr>
<tr><td>OMIM<span class="superscript">®</span>:</td>
<td><a href="https://omim.org/entry/162900" target="_blank">162900</a></td></tr>
</tbody></table></div><div class="rprt-body jig-ncbiinpagenav" data-jigconfig="smoothScroll: false, gotoTopLink: true, gotoTopLinkText: '', gotoTopLinkAttrs: {'title': 'Go to the top of the page'},allHeadingLevels: ['h1'], topOfPageTOC: true, tocId: 'my-toc'"><div id="rprt-tabs-1" class="rprt-tab"><div id="tb-termsProp-1"><div class="leftCol mgCol"><div>
<div class="portlet mgSection" id="ID_101">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Disease_characteristics">Disease characteristics</h1><a sid="101" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln"><div class="excerpt">Excerpted from the <i>GeneReview: </i><a href="/books/NBK153722" target="_blank">PIK3CA-Related Overgrowth Spectrum</a></div><div>PIK3CA-related overgrowth spectrum (PROS) encompasses a range of clinical findings in which the core features are congenital or early-childhood onset of segmental/focal overgrowth with or without cellular dysplasia. Prior to the identification of PIK3CA as the causative gene, PROS was separated into distinct clinical syndromes based on the tissues and/or organs involved (e.g., MCAP [megalencephaly-capillary malformation] syndrome and CLOVES [congenital lipomatous asymmetric overgrowth of the trunk, lymphatic, capillary, venous, and combined-type vascular malformations, epidermal nevi, skeletal and spinal anomalies] syndrome). The predominant areas of overgrowth include the brain, limbs (including fingers and toes), trunk (including abdomen and chest), and face, all usually in an asymmetric distribution. Generalized brain overgrowth may be accompanied by secondary overgrowth of specific brain structures resulting in ventriculomegaly, a markedly thick corpus callosum, and cerebellar tonsillar ectopia with crowding of the posterior fossa. Vascular malformations may include capillary, venous, and less frequently, arterial or mixed (capillary-lymphatic-venous or arteriovenous) malformations. Lymphatic malformations may be in various locations (internal and/or external) and can cause various clinical issues, including swelling, pain, and occasionally localized bleeding secondary to trauma. Lipomatous overgrowth may occur ipsilateral or contralateral to a vascular malformation, if present. The degree of intellectual disability appears to be mostly related to the presence and severity of seizures, cortical dysplasia (e.g., polymicrogyria), and hydrocephalus. Many children have feeding difficulties that are often multifactorial in nature. Endocrine issues affect a small number of individuals and most commonly include hypoglycemia (largely hypoinsulinemic hypoketotic hypoglycemia), hypothyroidism, and growth hormone deficiency. [from <a title="GeneReviews" href="https://www.ncbi.nlm.nih.gov/books/NBK1116" class="defSource" target="_blank">GeneReviews</a>]</div><div class="spaceAbove"><strong>Full text of <i>GeneReview</i> (by section):</strong><br /><a class="medgenPMinfo" href="/books/NBK153722#pik3ca-overgrowth.Summary" target="NBK153722">Summary</a>  |  <a class="medgenPMinfo" href="/books/NBK153722#pik3ca-overgrowth.GeneReview_Scope" target="NBK153722">GeneReview Scope</a>  |  <a class="medgenPMinfo" href="/books/NBK153722#pik3ca-overgrowth.Diagnosis" target="NBK153722">Diagnosis</a>  |  <a class="medgenPMinfo" href="/books/NBK153722#pik3ca-overgrowth.Clinical_Characteristi" target="NBK153722">Clinical Characteristics</a>  |  <a class="medgenPMinfo" href="/books/NBK153722#pik3ca-overgrowth.Genetically_Related_Al" target="NBK153722">Genetically Related (Allelic) Disorders</a>  |  <a class="medgenPMinfo" href="/books/NBK153722#pik3ca-overgrowth.Differential_Diagnosis" target="NBK153722">Differential Diagnosis</a>  |  <a class="medgenPMinfo" href="/books/NBK153722#pik3ca-overgrowth.Management" target="NBK153722">Management</a>  |  <a class="medgenPMinfo" href="/books/NBK153722#pik3ca-overgrowth.Genetic_Counseling" target="NBK153722">Genetic Counseling</a>  |  <a class="medgenPMinfo" href="/books/NBK153722#pik3ca-overgrowth.Resources" target="NBK153722">Resources</a>  |  <a class="medgenPMinfo" href="/books/NBK153722#pik3ca-overgrowth.Molecular_Genetics" target="NBK153722">Molecular Genetics</a>  |  <a class="medgenPMinfo" href="/books/NBK153722#pik3ca-overgrowth.Chapter_Notes" target="NBK153722">Chapter Notes</a>  |  <a class="medgenPMinfo" href="/books/NBK153722#pik3ca-overgrowth.References" target="NBK153722">References</a></div><div class="spaceAbove"><strong>Authors:</strong><br />
Ghayda Mirzaa  |  John M Graham  |  Kim Keppler-Noreuil   <a href="/books/NBK153722" target="NBK153722" title="NCBI Bookshelf: PIK3CA-Related Overgrowth Spectrum">view full author information</a></div></div>
</div>
<div class="portlet mgSection" id="ID_117">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Additional_descriptions">Additional descriptions</h1><a sid="117" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln"><div class="mgSection"><strong>From OMIM</strong><br />Epidermal nevi are congenital lesions that affect about 1 in 1,000 people. They appear at or shortly after birth as localized epidermal thickening with hyperpigmentation that frequently follow the lines of Blaschko, suggesting that they result from postzygotic somatic mutation in the skin (Paller et al., 1994).&#13;
A rare subgroup of epidermal nevi is clinically indistinguishable from other epidermal nevi, but displays histopathologic features typical of epidermolytic hyperkeratosis (see EHK, 113800), and patients with this type of epidermal nevi sometimes have offspring with generalized EHK (Paller et al., 1994).&#13;
Woolly hair nevus is a rare condition characterized by the development of woolly hair in a restricted area on the scalp, either present at birth or becoming evident later in life when scalp hair begins to grow. Woolly hair nevus can be an isolated finding or can occur in association with additional ectodermal defects; epidermal nevi have been reported in association with woolly hair nevi (summary by Ramot and Zlotogorski, 2015).&#13;
Nevus sebaceous, a benign congenital skin lesion that preferentially affects the scalp and face, is characterized by hairless, yellow-orange plaques of various size and shape. Histology shows that nevus sebaceous is a hamartoma consisting of epidermal, sebaceous, and apocrine elements. About 24% of nevi develop secondary tumors, some of which may be malignant (summary by Groesser et al., 2012).&#13;
Also see giant pigmented hairy nevus (137550) and malignant melanoma (155600).  <a target="_blank" href="http://www.omim.org/entry/162900">http://www.omim.org/entry/162900</a></div><div class="mgSection"><strong>From MedlinePlus Genetics</strong><br />An epidermal nevus (plural: nevi) is an abnormal, noncancerous (benign) patch of skin caused by an overgrowth of cells in the outermost layer of skin (epidermis). Epidermal nevi are typically seen at birth or develop in early childhood. Affected individuals have one or more nevi that vary in size.<br /><br />There are several types of epidermal nevus that are defined in part by the type of epidermal cell involved. The epidermis is composed primarily of a specific cell type called a keratinocyte. One group of epidermal nevi, called keratinocytic or nonorganoid epidermal nevi, includes nevi that involve only keratinocytes. Keratinocytic epidermal nevi are typically found on the torso or limbs. They can be flat, tan or brown patches of skin or raised, velvety patches. As affected individuals age, the nevi can become thicker and darker and develop a wart-like (verrucous) appearance. Often, keratinocytic epidermal nevi follow a pattern on the skin known as the lines of Blaschko. The lines of Blaschko, which are normally invisible on skin, are thought to follow the paths along which cells migrate as the skin develops before birth. Keratinocytic epidermal nevi are also known as linear epidermal nevi or verrucous epidermal nevi, based on characteristics of their appearance.<br /><br />Other types of epidermal nevi involve additional types of epidermal cells, such as the cells that make up the hair follicles, the sweat glands, or the sebaceous glands (glands in the skin that produce a substance that protects the skin and hair). These nevi comprise a group called organoid epidermal nevi. A common type of organoid epidermal nevus is called nevus sebaceous. Nevi in this group are waxy, yellow-orange patches of skin, usually on the scalp or face. The patch is typically hairless, leaving a distinct region of baldness (alopecia). Similar to keratinocytic epidermal nevi, nevi sebaceous can become thicker and more verrucous over time. In about one-quarter of people with a nevus sebaceous, a tumor forms in the same region as the nevus. The tumor is usually benign, although rarely cancerous (malignant) tumors develop.<br /><br />Some affected individuals have only an epidermal nevus and no other abnormalities. However, sometimes people with an epidermal nevus also have problems in other body systems, such as the brain, eyes, or bones. In these cases, the affected individual has a condition called an epidermal nevus syndrome. There are several different epidermal nevus syndromes characterized by the type of epidermal nevus involved.  <a target="_blank" href="https://medlineplus.gov/genetics/condition/epidermal-nevus">https://medlineplus.gov/genetics/condition/epidermal-nevus</a></div></div>
</div>
<div class="portlet mgSection" id="ID_102">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Clinical_features">Clinical features</h1><a sid="102" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln clinfeat"><strong>From HPO</strong><br />
<div class="divPopper rprt" id="clin_14364"><div><strong>Melanocytic nevus</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>14364</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C0027962</a></dd><dt><span class="dotprefix"></span></dt><dd>Neoplastic Process</dd></dl></div></div></div>
<div class="spaceAbove">A oval and round, colored (usually medium-to dark brown, reddish brown, or flesh colored) lesion. Typically, a melanocytic nevus is less than 6 mm in diameter, but may be much smaller or larger.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/14364">Feature record</a> | <a href="/medgen?term=%22Melanocytic%20nevus%22%5BClinical%20Features%5D%20OR%2014364%5Buid%5D">Search on this feature</a></div></div>
<div class="divPopper rprt" id="clin_341508"><div><strong>Numerous nevi</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>341508</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C1849677</a></dd><dt><span class="dotprefix"></span></dt><dd>Finding</dd></dl></div></div></div>
<div class="spaceAbove nowrap">See: <a href="/medgen/341508">Feature record</a> | <a href="/medgen?term=%22Numerous%20nevi%22%5BClinical%20Features%5D%20OR%20341508%5Buid%5D">Search on this feature</a></div></div><div class="TreeLite"><ul><li><span class="TLline">Abnormality of the integument</span><ul><li class="TLline">
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_14364" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Melanocytic nevus</a></span></li><li class="TLline">
<span class="TLline"><a title="click for more information" class="jig-ncbipopper" href="#clin_341508" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Numerous nevi</a></span></li></ul></li></ul></div></div>
</div>
<div class="portlet mgSection" id="ID_118">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Term_Hierarchy">Term Hierarchy</h1><a sid="118" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln HierarchyGTR"><div class="jig-ncbitabs"><ul><li><a href="#tabGTR">GTR</a></li><li><a href="#tabMGEN">MeSH</a></li><li><a href="#tabORDO">Orphanet</a></li></ul><div id="tabGTR"><div class="search_result"><div class="rprts"><div class="chiclet_legend"><span class="chiclet_list" style="position:static;"><span title="Clinical test" class="chiclet Ccolor round">C</span><span>Clinical test,  </span><span title="Research test" class="chiclet Rcolor round">R</span><span>Research test,  </span><span title="OMIM" class="chiclet Ocolor ">O</span><span>OMIM,  </span><span title="GeneReview" class="chiclet Gcolor">G</span><span><em>GeneReviews</em>,  </span><span title="ClinVar" class="chiclet Vcolor">V</span><span>ClinVar  </span></span></div><div id="hierarchy" class="margin_t1"><div class="ds_tree"><ul><li class="matched_ds"><span class="chiclet_list"><span class="chiclet Ccolor round" title="Clinical test"><a target="_blank" href="/gtr/tests/?term=C0334082[DISCUI]&amp;test_type=Clinical" ref="ncbi_uid=83106">C</a></span><span class="chiclet unavailable round" title="Research Tests">R</span><span class="chiclet Ocolor" title="OMIM"><a ref="ncbi_uid=83106" target="_blank" href="/omim/162900">O</a></span><span class="chiclet Gcolor" title="GeneReviews"><a target="_blank" href="/books/NBK153722/" ref="ncbi_uid=83106">G</a></span><span class="chiclet Vcolor" title="ClinVar"><a target="_blank" href="/clinvar?LinkName=medgen_clinvar&amp;from_uid=83106" ref="ncbi_uid=83106">V</a></span></span><span class="TLline">Epidermal nevus</span></li></ul></div></div></div></div></div><div id="tabMGEN"><div class="ds_tree"><ul><li><span class="TLline"><a href="/medgen/867443" ref="tree=MeSH" title="MedGen record for Phenotypic abnormality">Phenotypic abnormality</a></span><ul><li><span class="TLline"><a href="/medgen/871273" ref="tree=MeSH" title="MedGen record for Abnormality of the integument">Abnormality of the integument</a></span><ul><li><span class="TLline"><a href="/medgen/1845238" ref="tree=MeSH" title="MedGen record for Abnormality of the skin">Abnormality of the skin</a></span><ul><li><span class="TLline"><a href="/medgen/869110" ref="tree=MeSH" title="MedGen record for Abnormal skin morphology">Abnormal skin morphology</a></span><ul><li><span class="TLline"><a href="/medgen/208858" ref="tree=MeSH" title="MedGen record for Localized skin lesion">Localized skin lesion</a></span><ul><li><span class="TLline"><a href="/medgen/45074" ref="tree=MeSH" title="MedGen record for Nevus">Nevus</a></span><ul><li><span class="matched_ds">Epidermal nevus</span><ul><li><span class="TLline"><a href="/medgen/75592" ref="tree=MeSH" title="MedGen record for Nevus comedonicus syndrome">Nevus comedonicus syndrome</a></span></li><li><span class="TLline"><a href="/medgen/840896" ref="tree=MeSH" title="MedGen record for Nevus sebaceous">Nevus sebaceous</a></span><ul><li><span class="TLline"><a href="/medgen/1646345" ref="tree=MeSH" title="MedGen record for Linear nevus sebaceous syndrome">Linear nevus sebaceous syndrome</a></span></li></ul></li><li><span class="TLline"><a href="/medgen/83927" ref="tree=MeSH" title="MedGen record for Verrucous nevus">Verrucous nevus</a></span></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></li></ul></div></div><div id="tabORDO">Follow <a target="_blank" href="http://www.orpha.net/consor/cgi-bin/Disease_Classif.php?lng=EN&amp;data_id=156&amp;PatId=10375&amp;search=Disease_Classif_Simple&amp;new=1" class="ital bold">this link</a> to review classifications for <span class="ital">Epidermal nevus</span> in Orphanet.</div></div></div>
</div>
<div class="portlet mgSection" id="ID_112">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Conditions_with_this_feature">Conditions with this feature</h1><a sid="112" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln clinfeat">
<div class="divPopper rprt" id="rdis_5957"><div><strong>Seborrheic keratosis</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>5957</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C0022603</a></dd><dt><span class="dotprefix"></span></dt><dd>Neoplastic Process</dd></dl></div></div></div>
<div class="spaceAbove">Seborrheic keratoses are common benign epidermal lesion that can develop on any part of the body.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/5957">Condition Record</a></div></div>
<div class="divPopper rprt" id="rdis_39008"><div><strong>Proteus syndrome</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>39008</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C0085261</a></dd><dt><span class="dotprefix"></span></dt><dd>Neoplastic Process</dd></dl></div></div></div>
<div class="spaceAbove">Proteus syndrome (PS) is characterized by progressive segmental or patchy overgrowth most commonly affecting the skeleton, skin, adipose, and central nervous systems. In most individuals PS has modest or no manifestations at birth, develops and progresses rapidly beginning in the toddler period, and relentlessly progresses through childhood, causing severe overgrowth and disfigurement. It is associated with a range of tumors, pulmonary complications, and a striking predisposition to deep vein thrombosis and pulmonary embolism.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/39008">Condition Record</a></div></div>
<div class="divPopper rprt" id="rdis_82697"><div><strong>Child syndrome</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>82697</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C0265267</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">NSDHL-related disorders include CHILD (congenital hemidysplasia with ichthyosiform nevus and limb defects) syndrome, an X-linked disorder that is usually male lethal during gestation and thus predominantly affects females; and CK syndrome, an X-linked disorder that affects males. CHILD syndrome is characterized by unilateral distribution of ichthyosiform skin lesions and ipsilateral limb defects that range from shortening of the metacarpals and phalanges to absence of the entire limb. Intellect is usually normal. The ichthyosiform skin lesions are usually present at birth or in the first weeks of life; new lesions can develop in later life. Onychodystrophy and periungual hyperkeratosis are common. Heart, lung, and kidney malformations can also occur. CK syndrome is characterized by mild-to-severe cognitive impairment and behavior problems (aggression, attention-deficit/hyperactivity disorder [ADHD], and irritability). All reported affected males have developed seizures in infancy and have cerebral cortical malformations and microcephaly. All have distinctive facial features, a thin habitus, and relatively long, thin fingers and toes. Some have scoliosis and kyphosis. Strabismus is common. Optic atrophy is also reported.</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/82697">Condition Record</a></div></div>
<div class="divPopper rprt" id="rdis_120545"><div><strong>Dermatofibrosis lenticularis disseminata</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>120545</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C0265514</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">Buschke-Ollendorff syndrome (BOS) is an autosomal dominant connective tissue disorder manifest by multiple subcutaneous nevi or nodules. They may be either elastin-rich (elastoma) or collagen-rich (dermatofibrosis lenticularis disseminata) on histologic examination. The lesions are usually nontender and firm. Affected individuals also have osteopoikilosis (OPK), literally meaning 'spotted bones,' which are osteosclerotic foci that occur in the epiphyses and metaphyses of long bones, wrist, foot, ankle, pelvis, and scapula. Some individuals have both skin and bone manifestations, whereas others may lack skin or bone manifestations. Some individuals may also have melorheostosis (155950), which is characterized by 'flowing' hyperostosis of the cortex of tubular bones. Most reported cases of BOS and OPK are benign, and the bone lesions are found incidentally, although some patients may have joint pain (reviews by Hellemans et al., 2004 and Zhang et al., 2009).</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/120545">Condition Record</a></div></div>
<div class="divPopper rprt" id="rdis_333068"><div><strong>Toriello-Lacassie-Droste syndrome</strong><div class="aux"><div class="resc"><dl class="rprtid"><dt>MedGen UID: </dt><dd>333068</dd><dt><span class="dotprefix"></span>Concept ID: </dt><dd><a href="/medgen/docs/help/#sources" target="_blank" title="Concept Unique Identifier from NLM's Unified Medical Language system (UMLS)&#10;Click for more information.">C1838329</a></dd><dt><span class="dotprefix"></span></dt><dd>Disease or Syndrome</dd></dl></div></div></div>
<div class="spaceAbove">Oculoectodermal syndrome (OES) is characterized by the association of epibulbar dermoids and aplasia cutis congenita. Affected individuals exhibit congenital scalp lesions which are atrophic, nonscarring, hairless regions that are often multiple and asymmetric in distribution, and may have associated hamartomas. Ectodermal changes include linear hyperpigmentation that may follow the lines of Blaschko and, rarely, epidermal nevus-like lesions. Epibulbar dermoids may be uni- or bilateral. Additional ocular anomalies such as skin tags of the upper eyelid and rarely optic nerve or retinal changes or microphthalmia can be present. Phenotypic expression is highly variable, and various other abnormalities have occasionally been reported, including growth failure, lymphedema, and cardiovascular defects, as well as neurodevelopmental symptoms such as developmental delay, epilepsy, learning difficulties, and behavioral abnormalities. Benign tumor-like lesions such as nonossifying fibromas of the long bones and giant cell granulomas of the jaws have repeatedly been observed and appear to be age-dependent, becoming a common manifestation in individuals aged 5 years or older (summary by Boppudi et al., 2016).</div>
<div class="spaceAbove nowrap">See: <a href="/medgen/333068">Condition Record</a></div></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_82697" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Child syndrome</a></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_120545" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Dermatofibrosis lenticularis disseminata</a></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_39008" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Proteus syndrome</a></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_5957" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Seborrheic keratosis</a></div>
<div class="hangingIndent"><a title="click for more information" class="jig-ncbipopper" href="#rdis_333068" data-jigconfig="hasArrow: true, openEvent: 'click', closeEvent: 'mouseout', openAnimation: 'fadeIn', closeAnimation: 'fadeOut', triggerPosition: 'center right', destPosition: 'center left', arrowDirection: 'left'">Toriello-Lacassie-Droste syndrome</a></div></div>
</div>
<div class="portlet mgSection" id="ID_105">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Professional_guidelines">Professional guidelines</h1><a sid="105" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln"><h3 class="subhead">PubMed<a class="help jig-ncbi-popper" data-jig="ncbipopper" href="#guidelinesHelpPM"><img class="pulldown" src="//static.pubmed.gov/portal/portal3rc.fcgi/4223267/img/4204968" /></a></h3>
<div class="nl"><a target="_blank" href="/pubmed/38360177">Inflammatory linear verrucous epidermal nevus should be genotyped to direct treatment and genetic counseling.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Polubothu S,
Riachi M,
Stadnik P,
Ogunbiyi O,
Brändli-Wälchli R,
Cullup T,
Sebire NJ,
Pittman A,
Kinsler VA</span><br />
<span class="medgenPMjournal">J Am Acad Dermatol</span>
2024 Jun;90(6):1279-1280.
Epub 2024 Feb 14
doi: 10.1016/j.jaad.2024.01.075.
<span class="bold">PMID: </span><a href="/pubmed/38360177" target="_blank">38360177</a></div>
<div class="nl"><a target="_blank" href="/pubmed/36098675">S2k guideline: Laser therapy of the skin.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Paasch U,
Zidane M,
Baron JM,
Bund T,
Cappius HJ,
Drosner M,
Feise K,
Fischer T,
Gauglitz G,
Gerber PA,
Grunewald S,
Herberger K,
Jung A,
Karsai S,
Kautz G,
Philipp C,
Schädel D,
Seitz AT,
Nast A</span><br />
<span class="medgenPMjournal">J Dtsch Dermatol Ges</span>
2022 Sep;20(9):1248-1267.
Epub 2022 Sep 13
doi: 10.1111/ddg.14879.
<span class="bold">PMID: </span><a href="/pubmed/36098675" target="_blank">36098675</a></div>
<div class="nl"><a target="_blank" href="/pubmed/26564069">Phenotype/genotype correlations in epidermal nevus syndrome as a neurocristopathy.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Flores-Sarnat L,
Sarnat HB</span><br />
<span class="medgenPMjournal">Handb Clin Neurol</span>
2015;132:9-25.
doi: 10.1016/B978-0-444-62702-5.00002-0.
<span class="bold">PMID: </span><a href="/pubmed/26564069" target="_blank">26564069</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=(%22epidermal%20nevus%22%5Btiab%3A~0%5D)%20AND%20(%22english%20and%20humans%22%5BFilter%5D)%20AND%20(%20(%22practice%20guideline%22%5BFilter%5D)%20OR%20(practice*%5Btitl%5D%20AND%20(guideline%5Btitl%5D%20OR%20parameter%5Btitl%5D%20OR%20resource%5Btitl%5D%20OR%20bulletin%5Btitl%5D%20OR%20best%5Btitl%5D))%20OR%20(genetic*%5Btitl%5D%20AND%20(evaluation%5Btitl%5D%20OR%20counseling%5Btitl%5D%20OR%20screening%5Btitl%5D%20OR%20test*%5Btitl%5D))%20OR%20(clinical%5Btitl%5D%20AND%20((expert%5Btitl%5D%20AND%20consensus%5Btitl%5D)%20OR%20utility%5Btitl%5D%20OR%20guideline*%5Btitl%5D))%20OR%20(management%5Btitl%5D%20AND%20(clinical%5Btitl%5D%20OR%20diagnos*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20pain%5Btitl%5D%20OR%20surveillance%5Btitl%5D%20OR%20emergency%5Btitl%5D%20OR%20guideline*%5Btitl%5D%20OR%20therap*))%20OR%20(treatment%5Btitl%5D%20AND%20((evaluation%5Btitl%5D%20AND%20diagnosis%5Btitl%5D)%20OR%20(assessment%5Btitl%5D%20AND%20prevention%5Btitl%5D)%20OR%20therap*))%20OR%20(Diagnos*%5Btitl%5D%20AND%20(prenatal%5Btitl%5D%20OR%20treatment%5Btitl%5D%20OR%20follow-up%5Btitl%5D%20OR%20statement%5Btitl%5D%20OR%20criteria%5Btitl%5D%20OR%20newborn%5Btitl%5D%20OR%20differential%5Btitl%5D%20OR%20neonatal%5Btitl%5D%20OR%20neonate%5Btitl%5D))%20OR%20(guideline*%5Btitl%5D%20AND%20(pharmacogenetic*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20evidence-based%5Btitl%5D%20OR%20consensus%5Btitl%5D%20OR%20(technical%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20(molecular%5Btitl%5D%20AND%20testing%5Btitl%5D)))%20OR%20(risk%5Btitl%5D%20AND%20assessment%5Btitl%5D)%20OR%20(recommendation*%5Btitl%5D%20AND%20(statement%5Btitl%5D%20OR%20Evidence-based%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(care%20AND%20((Patient%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20primary%5Btitl%5D%20OR%20psychosocial%5Btitl%5D))%20OR%20(Health%5Btitl%5D%20AND%20supervision%5Btitl%5D)%20OR%20(statement%5Btitl%5D%20AND%20(policy%5Btitl%5D%20OR%20position%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(pharmacogenetics%5Btitl%5D%20AND%20(Dosing%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20genotype*%5Btitl%5D%20OR%20drug*%5Btitl%5D))%20OR%20(Chemotherapy%5Btitl%5D%20AND%20decision*%5Btitl%5D)%20OR%20(screening%5Btitl%5D%20AND%20(newborn%5Btitl%5D%20OR%20neonat*%5Btitl%5D%20OR%20detection%5Btitl%5D%20OR%20diagnos*%5Btitl%5D))%20OR%20(criteria%5Btitl%5D%20OR%20genotype*%5Btitl%5D)%20)%20NOT%20(%22Case%20reports%22%5BPublication%20type%5D%20OR%20%22clinical%20study%22%5BPublication%20Type%5D%20OR%20%22randomized%20controlled%20trial%22%5BPublication%20Type%5D)" title="PubMed search">See all (12)</a></div></div>
</div>
<div class="display-none help-popup" id="guidelinesHelpPM">These guidelines are articles in PubMed that match specific search criteria developed by MedGen to capture the most relevant practice guidelines. This list may not be comprehensive and may include broader topics as well. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div><div class="display-none help-popup" id="guidelinesHelpCurated">These guidelines are manually curated by the MedGen team
to supplement articles available in PubMed. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div>
<div class="portlet mgSection" id="ID_103">
<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Recent_clinical_studies">Recent clinical studies</h1><a sid="103" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
<div class="portlet_content ln"><h3 class="subhead">Etiology</h3>
<div class="nl"><a target="_blank" href="/pubmed/35853659">Inflammatory linear verrucous epidermal nevus (ILVEN) encompasses a spectrum of inflammatory mosaic disorders.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Atzmony L,
Ugwu N,
Hamilton C,
Paller AS,
Zech L,
Antaya RJ,
Choate KA</span><br />
<span class="medgenPMjournal">Pediatr Dermatol</span>
2022 Nov;39(6):903-907.
Epub 2022 Jul 19
doi: 10.1111/pde.15094.
<span class="bold">PMID: </span><a href="/pubmed/35853659" target="_blank">35853659</a><a href="/pmc/articles/PMC9712156" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/26564089">Epidermal nevus syndromes.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Asch S,
Sugarman JL</span><br />
<span class="medgenPMjournal">Handb Clin Neurol</span>
2015;132:291-316.
doi: 10.1016/B978-0-444-62702-5.00022-6.
<span class="bold">PMID: </span><a href="/pubmed/26564089" target="_blank">26564089</a></div>
<div class="nl"><a target="_blank" href="/pubmed/15818440">Dermatological aspects of cerebrovascular diseases.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Al Aboud D,
Broshtilova V,
Al Aboud K,
Al Hawsawi K</span><br />
<span class="medgenPMjournal">Acta Dermatovenerol Alp Pannonica Adriat</span>
2005 Mar;14(1):9-14.
<span class="bold">PMID: </span><a href="/pubmed/15818440" target="_blank">15818440</a></div>
<div class="nl"><a target="_blank" href="/pubmed/10592398">Nevus comedonicus.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Lefkowitz A,
Schwartz RA,
Lambert WC</span><br />
<span class="medgenPMjournal">Dermatology</span>
1999;199(3):204-7.
doi: 10.1159/000018275.
<span class="bold">PMID: </span><a href="/pubmed/10592398" target="_blank">10592398</a></div>
<div class="nl"><a target="_blank" href="/pubmed/2655139">Ocular choristomas.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Mansour AM,
Barber JC,
Reinecke RD,
Wang FM</span><br />
<span class="medgenPMjournal">Surv Ophthalmol</span>
1989 Mar-Apr;33(5):339-58.
doi: 10.1016/0039-6257(89)90011-8.
<span class="bold">PMID: </span><a href="/pubmed/2655139" target="_blank">2655139</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Epidermal%20nevus%22%20AND%20Etiology%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (104)</a></div><h3 class="subhead">Diagnosis</h3>
<div class="nl"><a target="_blank" href="/pubmed/37808438">Verrucous epidermal nevus.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Rivera-Silva G,
Moreno-Treviño MG</span><br />
<span class="medgenPMjournal">Pan Afr Med J</span>
2023;45:146.
Epub 2023 Aug 2
doi: 10.11604/pamj.2023.45.146.40966.
<span class="bold">PMID: </span><a href="/pubmed/37808438" target="_blank">37808438</a><a href="/pmc/articles/PMC10559149" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/36852958">Linear verrucous epidermal nevus on penis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Qi T,
Peng C,
Cheng Y</span><br />
<span class="medgenPMjournal">Minerva Pediatr (Torino)</span>
2023 Aug;75(4):636-637.
Epub 2023 Feb 28
doi: 10.23736/S2724-5276.23.07188-4.
<span class="bold">PMID: </span><a href="/pubmed/36852958" target="_blank">36852958</a></div>
<div class="nl"><a target="_blank" href="/pubmed/36670501">What is an epidermal nevus?</a></div>
<div class="portlet_content ln"><span class="medgenPMjournal">Pediatr Dermatol</span>
2023 Jan;40(1):188-189.
doi: 10.1111/pde.15259.
<span class="bold">PMID: </span><a href="/pubmed/36670501" target="_blank">36670501</a></div>
<div class="nl"><a target="_blank" href="/pubmed/35321589">Systematized Sebaceous Nevus.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Zhang LL,
Liu ZH</span><br />
<span class="medgenPMjournal">J Cutan Med Surg</span>
2022 Nov-Dec;26(6):643.
Epub 2022 Mar 23
doi: 10.1177/12034754221088568.
<span class="bold">PMID: </span><a href="/pubmed/35321589" target="_blank">35321589</a></div>
<div class="nl"><a target="_blank" href="/pubmed/35321579">Linear Nevus Comedonicus.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Singh AK,
Devi GC</span><br />
<span class="medgenPMjournal">J Cutan Med Surg</span>
2022 Nov-Dec;26(6):642.
Epub 2022 Mar 23
doi: 10.1177/12034754221088567.
<span class="bold">PMID: </span><a href="/pubmed/35321579" target="_blank">35321579</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Epidermal%20nevus%22%20AND%20Diagnosis%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (258)</a></div><h3 class="subhead">Therapy</h3>
<div class="nl"><a target="_blank" href="/pubmed/35853659">Inflammatory linear verrucous epidermal nevus (ILVEN) encompasses a spectrum of inflammatory mosaic disorders.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Atzmony L,
Ugwu N,
Hamilton C,
Paller AS,
Zech L,
Antaya RJ,
Choate KA</span><br />
<span class="medgenPMjournal">Pediatr Dermatol</span>
2022 Nov;39(6):903-907.
Epub 2022 Jul 19
doi: 10.1111/pde.15094.
<span class="bold">PMID: </span><a href="/pubmed/35853659" target="_blank">35853659</a><a href="/pmc/articles/PMC9712156" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/34428533">Topical sirolimus therapy for nevus sebaceus and epidermal nevus: A case series.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Zhou AG,
Antaya RJ</span><br />
<span class="medgenPMjournal">J Am Acad Dermatol</span>
2022 Aug;87(2):407-409.
Epub 2021 Aug 21
doi: 10.1016/j.jaad.2021.08.029.
<span class="bold">PMID: </span><a href="/pubmed/34428533" target="_blank">34428533</a></div>
<div class="nl"><a target="_blank" href="/pubmed/32133868">Off-Label Therapeutic Potential of Crisaborole.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Makins C,
Sanghera R,
Grewal PS</span><br />
<span class="medgenPMjournal">J Cutan Med Surg</span>
2020 May/Jun;24(3):292-296.
Epub 2020 Mar 5
doi: 10.1177/1203475420909794.
<span class="bold">PMID: </span><a href="/pubmed/32133868" target="_blank">32133868</a></div>
<div class="nl"><a target="_blank" href="/pubmed/24364998">Verrucous epidermal nevus.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Kim R,
Marmon S,
Kaplan J,
Kamino H,
Pomeranz MK</span><br />
<span class="medgenPMjournal">Dermatol Online J</span>
2013 Dec 16;19(12):20707.
<span class="bold">PMID: </span><a href="/pubmed/24364998" target="_blank">24364998</a></div>
<div class="nl"><a target="_blank" href="/pubmed/18573722">Unilateral blaschkolinear psoriasis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">López N,
Cabra B,
Castillo R,
Alcaraz I,
Bosch R,
Herrera E</span><br />
<span class="medgenPMjournal">Eur J Dermatol</span>
2008 Jul-Aug;18(4):457-8.
Epub 2008 Jun 23
doi: 10.1684/ejd.2008.0442.
<span class="bold">PMID: </span><a href="/pubmed/18573722" target="_blank">18573722</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Epidermal%20nevus%22%20AND%20Therapy%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (74)</a></div><h3 class="subhead">Prognosis</h3>
<div class="nl"><a target="_blank" href="/pubmed/31729336">Linear Verrucous Epidermal Nevus.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Gupta A,
Rai R</span><br />
<span class="medgenPMjournal">Indian Pediatr</span>
2019 Nov 15;56(11):981.
<span class="bold">PMID: </span><a href="/pubmed/31729336" target="_blank">31729336</a></div>
<div class="nl"><a target="_blank" href="/pubmed/26730692">Lesions With an Epidermal Hyperplastic Pattern: Morphologic Clues in the Differential Diagnosis.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Fernandez-Flores A</span><br />
<span class="medgenPMjournal">Am J Dermatopathol</span>
2016 Jan;38(1):1-16; quiz 17-9.
doi: 10.1097/DAD.0000000000000324.
<span class="bold">PMID: </span><a href="/pubmed/26730692" target="_blank">26730692</a></div>
<div class="nl"><a target="_blank" href="/pubmed/26564069">Phenotype/genotype correlations in epidermal nevus syndrome as a neurocristopathy.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Flores-Sarnat L,
Sarnat HB</span><br />
<span class="medgenPMjournal">Handb Clin Neurol</span>
2015;132:9-25.
doi: 10.1016/B978-0-444-62702-5.00002-0.
<span class="bold">PMID: </span><a href="/pubmed/26564069" target="_blank">26564069</a></div>
<div class="nl"><a target="_blank" href="/pubmed/10651962">Inflammatory linear verrucous epidermal nevus (ILVEN) and psoriasis in a child?</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Menni S,
Restano L,
Gianotti R,
Boccardi D</span><br />
<span class="medgenPMjournal">Int J Dermatol</span>
2000 Jan;39(1):30-2.
doi: 10.1046/j.1365-4362.2000.00872.x.
<span class="bold">PMID: </span><a href="/pubmed/10651962" target="_blank">10651962</a></div>
<div class="nl"><a target="_blank" href="/pubmed/8613290">Epidermal nevus syndrome.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Umakumaran P,
Srinivas TP,
Vishwanath D,
Maiya PP</span><br />
<span class="medgenPMjournal">Indian Pediatr</span>
1995 Mar;32(3):343-5.
<span class="bold">PMID: </span><a href="/pubmed/8613290" target="_blank">8613290</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Epidermal%20nevus%22%20AND%20Prognosis%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (47)</a></div><h3 class="subhead">Clinical prediction guides</h3>
<div class="nl"><a target="_blank" href="/pubmed/27497815">Cutaneous skeletal hypophosphatemia syndrome: clinical spectrum, natural history, and treatment.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Ovejero D,
Lim YH,
Boyce AM,
Gafni RI,
McCarthy E,
Nguyen TA,
Eichenfield LF,
DeKlotz CM,
Guthrie LC,
Tosi LL,
Thornton PS,
Choate KA,
Collins MT</span><br />
<span class="medgenPMjournal">Osteoporos Int</span>
2016 Dec;27(12):3615-3626.
Epub 2016 Aug 6
doi: 10.1007/s00198-016-3702-8.
<span class="bold">PMID: </span><a href="/pubmed/27497815" target="_blank">27497815</a><a href="/pmc/articles/PMC6908308" target="_blank" class="PubMedFree">Free PMC Article</a></div>
<div class="nl"><a target="_blank" href="/pubmed/26564069">Phenotype/genotype correlations in epidermal nevus syndrome as a neurocristopathy.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Flores-Sarnat L,
Sarnat HB</span><br />
<span class="medgenPMjournal">Handb Clin Neurol</span>
2015;132:9-25.
doi: 10.1016/B978-0-444-62702-5.00002-0.
<span class="bold">PMID: </span><a href="/pubmed/26564069" target="_blank">26564069</a></div>
<div class="nl"><a target="_blank" href="/pubmed/24364998">Verrucous epidermal nevus.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Kim R,
Marmon S,
Kaplan J,
Kamino H,
Pomeranz MK</span><br />
<span class="medgenPMjournal">Dermatol Online J</span>
2013 Dec 16;19(12):20707.
<span class="bold">PMID: </span><a href="/pubmed/24364998" target="_blank">24364998</a></div>
<div class="nl"><a target="_blank" href="/pubmed/17010754">The acanthosis nigricans form of epidermal nevus.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Ersoy-Evans S,
Sahin S,
Mancini AJ,
Paller AS,
Guitart J</span><br />
<span class="medgenPMjournal">J Am Acad Dermatol</span>
2006 Oct;55(4):696-8.
doi: 10.1016/j.jaad.2006.01.026.
<span class="bold">PMID: </span><a href="/pubmed/17010754" target="_blank">17010754</a></div>
<div class="nl"><a target="_blank" href="/pubmed/11834841">Transposable elements and the lines of Blaschko: a new perspective.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Happle R</span><br />
<span class="medgenPMjournal">Dermatology</span>
2002;204(1):4-7.
doi: 10.1159/000051801.
<span class="bold">PMID: </span><a href="/pubmed/11834841" target="_blank">11834841</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Epidermal%20nevus%22%20AND%20Clinical%20prediction%20guides%2Fbroad%5Bfilter%5D%20%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (79)</a></div></div>
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<div class="portlet_head mgSectionHead ui-widget-header"><h1 class="nl" id="Recent_systematic_reviews">Recent systematic reviews</h1><a sid="104" href="#" class="portlet_shutter" title="Show/hide content"></a></div>
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<div class="nl"><a target="_blank" href="/pubmed/32645757">Rickets in association with skin diseases and conditions: A review with emphasis on screening and prevention.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Litaiem N,
Chabchoub I,
Bacha T,
Slouma M,
Zeglaoui F,
Khachemoune A</span><br />
<span class="medgenPMjournal">Photodermatol Photoimmunol Photomed</span>
2020 Sep;36(5):339-350.
Epub 2020 Jul 24
doi: 10.1111/phpp.12590.
<span class="bold">PMID: </span><a href="/pubmed/32645757" target="_blank">32645757</a></div>
<div class="nl"><a target="_blank" href="/pubmed/31336213">Ameloblastoma associated with syndromes: A systematic review.</a></div>
<div class="portlet_content ln"><span class="medgenPMauthor">Atarbashi-Moghadam S,
Atarbashi-Moghadam F,
Sijanivandi S,
Mokhtari S</span><br />
<span class="medgenPMjournal">J Stomatol Oral Maxillofac Surg</span>
2020 Apr;121(2):146-149.
Epub 2019 Jul 20
doi: 10.1016/j.jormas.2019.07.010.
<span class="bold">PMID: </span><a href="/pubmed/31336213" target="_blank">31336213</a></div>
<div><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=%22Epidermal%20nevus%22%20AND%20systematic%5Bsb%5D%20AND%20%22english%20and%20humans%22%5Bfilter%5D%20NOT%20comment%5BPTYP%5D%20NOT%20letter%5BPTYP%5D" title="PubMed search">See all (2)</a></div></div>
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<div class="portlet_content ln"><ul><li><a href="/gtr/tests?term=C0334082%5bDISCUI%5d&amp;filter=method%3A2%5F8" target="_blank">Deletion/duplication analysis (69)</a></li>
<li><a href="/gtr/tests?term=C0334082%5bDISCUI%5d&amp;filter=method%3A2%5F17" target="_blank">Mutation scanning of the entire coding region (1)</a></li>
<li><a href="/gtr/tests?term=C0334082%5bDISCUI%5d&amp;filter=method%3A2%5F9" target="_blank">Sequence analysis of select exons (1)</a></li>
<li><a href="/gtr/tests?term=C0334082%5bDISCUI%5d&amp;filter=method%3A2%5F7" target="_blank">Sequence analysis of the entire coding region (88)</a></li>
<li><a href="/gtr/tests?term=C0334082%5bDISCUI%5d&amp;filter=method%3A2%5F19" target="_blank">Targeted variant analysis (11)</a></li>
<li class="portletSeeAll portletSeeAllPad"><total><a href="/gtr/tests?term=C0334082%5bDISCUI%5d" target="_blank">See all (96)</a></total></li>
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<div class="portlet_content ln"><ul class="a_poppers"><li><a target="_blank" href="https://pubmed.ncbi.nlm.nih.gov/?term=(%22epidermal%20nevus%22%5Btiab%3A~0%5D)%20AND%20(%22english%20and%20humans%22%5BFilter%5D)%20AND%20(%20(%22practice%20guideline%22%5BFilter%5D)%20OR%20(practice*%5Btitl%5D%20AND%20(guideline%5Btitl%5D%20OR%20parameter%5Btitl%5D%20OR%20resource%5Btitl%5D%20OR%20bulletin%5Btitl%5D%20OR%20best%5Btitl%5D))%20OR%20(genetic*%5Btitl%5D%20AND%20(evaluation%5Btitl%5D%20OR%20counseling%5Btitl%5D%20OR%20screening%5Btitl%5D%20OR%20test*%5Btitl%5D))%20OR%20(clinical%5Btitl%5D%20AND%20((expert%5Btitl%5D%20AND%20consensus%5Btitl%5D)%20OR%20utility%5Btitl%5D%20OR%20guideline*%5Btitl%5D))%20OR%20(management%5Btitl%5D%20AND%20(clinical%5Btitl%5D%20OR%20diagnos*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20pain%5Btitl%5D%20OR%20surveillance%5Btitl%5D%20OR%20emergency%5Btitl%5D%20OR%20guideline*%5Btitl%5D%20OR%20therap*))%20OR%20(treatment%5Btitl%5D%20AND%20((evaluation%5Btitl%5D%20AND%20diagnosis%5Btitl%5D)%20OR%20(assessment%5Btitl%5D%20AND%20prevention%5Btitl%5D)%20OR%20therap*))%20OR%20(Diagnos*%5Btitl%5D%20AND%20(prenatal%5Btitl%5D%20OR%20treatment%5Btitl%5D%20OR%20follow-up%5Btitl%5D%20OR%20statement%5Btitl%5D%20OR%20criteria%5Btitl%5D%20OR%20newborn%5Btitl%5D%20OR%20differential%5Btitl%5D%20OR%20neonatal%5Btitl%5D%20OR%20neonate%5Btitl%5D))%20OR%20(guideline*%5Btitl%5D%20AND%20(pharmacogenetic*%5Btitl%5D%20OR%20recommendation%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20evidence-based%5Btitl%5D%20OR%20consensus%5Btitl%5D%20OR%20(technical%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20(molecular%5Btitl%5D%20AND%20testing%5Btitl%5D)))%20OR%20(risk%5Btitl%5D%20AND%20assessment%5Btitl%5D)%20OR%20(recommendation*%5Btitl%5D%20AND%20(statement%5Btitl%5D%20OR%20Evidence-based%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(care%20AND%20((Patient%5Btitl%5D%20AND%20standard*%5Btitl%5D)%20OR%20primary%5Btitl%5D%20OR%20psychosocial%5Btitl%5D))%20OR%20(Health%5Btitl%5D%20AND%20supervision%5Btitl%5D)%20OR%20(statement%5Btitl%5D%20AND%20(policy%5Btitl%5D%20OR%20position%5Btitl%5D%20OR%20Consensus%5Btitl%5D))%20OR%20(pharmacogenetics%5Btitl%5D%20AND%20(Dosing%5Btitl%5D%20OR%20therap*%5Btitl%5D%20OR%20genotype*%5Btitl%5D%20OR%20drug*%5Btitl%5D))%20OR%20(Chemotherapy%5Btitl%5D%20AND%20decision*%5Btitl%5D)%20OR%20(screening%5Btitl%5D%20AND%20(newborn%5Btitl%5D%20OR%20neonat*%5Btitl%5D%20OR%20detection%5Btitl%5D%20OR%20diagnos*%5Btitl%5D))%20OR%20(criteria%5Btitl%5D%20OR%20genotype*%5Btitl%5D)%20)%20NOT%20(%22Case%20reports%22%5BPublication%20type%5D%20OR%20%22clinical%20study%22%5BPublication%20Type%5D%20OR%20%22randomized%20controlled%20trial%22%5BPublication%20Type%5D)" title="PubMed search">PubMed</a><div class="help-popup">See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the <a href="/medgen/docs/faq/" title="Frequently asked questions" target="_blank">FAQ</a> for details.</div></li></ul></div>
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<div class="portlet_content ln"><ul><li><a href="http://www.omim.org/search?index=entry&amp;start=1&amp;limit=10&amp;sort=score%20desc&amp;field=number&amp;search=134934%20164790%20171834%20190020" target="_blank">OMIM</a></li><li><a href="/clinvar/?term=2261[geneid]" target="_blank">View FGFR3 variations in ClinVar</a></li><li><a href="/clinvar/?term=3265[geneid]" target="_blank">View HRAS variations in ClinVar</a></li><li><a href="/clinvar/?term=4893[geneid]" target="_blank">View NRAS variations in ClinVar</a></li><li><a href="/clinvar/?term=5290[geneid]" target="_blank">View PIK3CA variations in ClinVar</a></li><li><a href="/nuccore/187423895,187960074,237858742,255522834" target="_blank">RefSeqGene</a></li><li><a href="https://catalog.coriell.org/Search?q=162900" target="_blank">Coriell Institute for Medical Research</a></li></ul></div>
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<p class="address_footer text-white">National Library of Medicine<br />
<a href="https://www.google.com/maps/place/8600+Rockville+Pike,+Bethesda,+MD+20894/@38.9959508,-77.101021,17z/data=!3m1!4b1!4m5!3m4!1s0x89b7c95e25765ddb:0x19156f88b27635b8!8m2!3d38.9959508!4d-77.0988323" class="text-white" target="_blank" rel="noopener noreferrer">8600 Rockville Pike<br />
Bethesda, MD 20894</a></p>
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<p><a href="https://www.nlm.nih.gov/web_policies.html" class="text-white">Web Policies</a><br />
<a href="https://www.nih.gov/institutes-nih/nih-office-director/office-communications-public-liaison/freedom-information-act-office" class="text-white">FOIA</a><br />
<a href="https://www.hhs.gov/vulnerability-disclosure-policy/index.html" class="text-white" id="vdp">HHS Vulnerability Disclosure</a></p>
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<div class="col-lg-3 col-12 centered-lg">
<p><a class="supportLink text-white" href="https://support.nlm.nih.gov/">Help</a><br />
<a href="https://www.nlm.nih.gov/accessibility.html" class="text-white">Accessibility</a><br />
<a href="https://www.nlm.nih.gov/careers/careers.html" class="text-white">Careers</a></p>
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<div class="row">
<div class="col-lg-12 centered-lg">
<nav class="bottom-links">
<ul class="mt-3">
<li>
<a class="text-white" href="//www.nlm.nih.gov/">NLM</a>
</li>
<li>
<a class="text-white" href="https://www.nih.gov/">NIH</a>
</li>
<li>
<a class="text-white" href="https://www.hhs.gov/">HHS</a>
</li>
<li>
<a class="text-white" href="https://www.usa.gov/">USA.gov</a>
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</ul>
</nav>
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