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class="mw-page-title-main">Netherton syndrome</span></h1> <div id="p-lang-btn" class="vector-dropdown mw-portlet mw-portlet-lang" > <input type="checkbox" id="p-lang-btn-checkbox" role="button" aria-haspopup="true" data-event-name="ui.dropdown-p-lang-btn" class="vector-dropdown-checkbox mw-interlanguage-selector" aria-label="Go to an article in another language. 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.infobox-subbox{padding:0;border:none;margin:-3px;width:auto;min-width:100%;font-size:100%;clear:none;float:none;background-color:transparent}.mw-parser-output .infobox-3cols-child{margin:auto}.mw-parser-output .infobox .navbar{font-size:100%}@media screen{html.skin-theme-clientpref-night .mw-parser-output .infobox-full-data:not(.notheme)>div:not(.notheme)[style]{background:#1f1f23!important;color:#f8f9fa}}@media screen and (prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .infobox-full-data:not(.notheme) div:not(.notheme){background:#1f1f23!important;color:#f8f9fa}}@media(min-width:640px){body.skin--responsive .mw-parser-output .infobox-table{display:table!important}body.skin--responsive .mw-parser-output .infobox-table>caption{display:table-caption!important}body.skin--responsive .mw-parser-output .infobox-table>tbody{display:table-row-group}body.skin--responsive .mw-parser-output .infobox-table tr{display:table-row!important}body.skin--responsive .mw-parser-output .infobox-table th,body.skin--responsive .mw-parser-output .infobox-table td{padding-left:inherit;padding-right:inherit}}</style><table class="infobox ib-medical-condition"><tbody><tr><th colspan="2" class="infobox-above" style="background:#ccc">Netherton syndrome</th></tr><tr><th scope="row" class="infobox-label">Other names</th><td class="infobox-data">Comèl-Netherton syndrome</td></tr><tr style="background-color: #f8f9fa;"><td colspan="2" class="infobox-full-data"><span class="mw-default-size" typeof="mw:File/Frameless"><a href="/wiki/File:Autosomal_recessive_-_en.svg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/f/f1/Autosomal_recessive_-_en.svg/220px-Autosomal_recessive_-_en.svg.png" decoding="async" width="220" height="382" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/f/f1/Autosomal_recessive_-_en.svg/330px-Autosomal_recessive_-_en.svg.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/f/f1/Autosomal_recessive_-_en.svg/440px-Autosomal_recessive_-_en.svg.png 2x" data-file-width="738" data-file-height="1283" /></a></span></td></tr><tr><td colspan="2" class="infobox-full-data">Netherton syndrome has an autosomal recessive pattern of <a href="/wiki/Heredity" title="Heredity">inheritance</a>.</td></tr><tr><th scope="row" class="infobox-label"><a href="/wiki/Medical_specialty" title="Medical specialty">Specialty</a></th><td class="infobox-data"><a href="/wiki/Medical_genetics" title="Medical genetics">Medical genetics</a> <span class="penicon autoconfirmed-show"><span class="mw-valign-text-top" typeof="mw:File/Frameless"><a href="https://www.wikidata.org/wiki/Q9390284?uselang=en#P1995" title="Edit this on Wikidata"><img alt="Edit this on Wikidata" src="//upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/10px-OOjs_UI_icon_edit-ltr-progressive.svg.png" decoding="async" width="10" height="10" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/15px-OOjs_UI_icon_edit-ltr-progressive.svg.png 1.5x, //upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/20px-OOjs_UI_icon_edit-ltr-progressive.svg.png 2x" data-file-width="20" data-file-height="20" /></a></span></span></td></tr></tbody></table> <p><b>Netherton syndrome</b> is a severe, <a href="/wiki/Autosome" title="Autosome">autosomal</a> <a href="/wiki/Dominance_(genetics)" title="Dominance (genetics)">recessive</a><sup id="cite_ref-ns05_1-0" class="reference"><a href="#cite_note-ns05-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup> form of <a href="/wiki/Ichthyosis" title="Ichthyosis">ichthyosis</a> associated with mutations in the <i><a href="/wiki/SPINK5" class="mw-redirect" title="SPINK5">SPINK5</a></i> gene.<sup id="cite_ref-pmid10835624_2-0" class="reference"><a href="#cite_note-pmid10835624-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-Fitz2_3-0" class="reference"><a href="#cite_note-Fitz2-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup> It is named after Earl W. Netherton (1910–1985), an American dermatologist who discovered it in 1958.<sup id="cite_ref-4" class="reference"><a href="#cite_note-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup> </p> <meta property="mw:PageProp/toc" /> <div class="mw-heading mw-heading2"><h2 id="Signs_and_symptoms">Signs and symptoms</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Netherton_syndrome&action=edit&section=1" title="Edit section: Signs and symptoms"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Netherton syndrome is characterized by chronic skin inflammation, universal pruritus (itch), severe dehydration, and stunted growth. Patients with this disorder tend to have a hair shaft defect (<a href="/wiki/Trichorrhexis_invaginata" title="Trichorrhexis invaginata">trichorrhexis invaginata</a>), also known as "bamboo hair". The disrupted skin barrier function in affected individuals also presents a high susceptibility to <a href="/wiki/Infection" title="Infection">infection</a> and <a href="/wiki/Allergy" title="Allergy">allergy</a>, leading to the development of scaly, reddish skin similar to <a href="/wiki/Atopic_dermatitis" title="Atopic dermatitis">atopic dermatitis</a>.<sup id="cite_ref-descargues_5-0" class="reference"><a href="#cite_note-descargues-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> In severe cases, these atopic manifestations persist throughout the individual's life, and consequently post-natal mortality rates are high. In less severe cases, this develops into the milder <a href="/wiki/Ichthyosis_linearis_circumflexa" title="Ichthyosis linearis circumflexa">ichthyosis linearis circumflexa</a>.<sup id="cite_ref-Fitz2_3-1" class="reference"><a href="#cite_note-Fitz2-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup> </p><p>Netherton syndrome has recently been characterised as a primary immunodeficiency, which straddles the innate and acquired immune system, much as does <a href="/wiki/Wiskott%E2%80%93Aldrich_syndrome" title="Wiskott–Aldrich syndrome">Wiskott–Aldrich syndrome</a>. A group of Netherton patients have been demonstrated to have altered immunoglobulin levels (typically high IgE and low to normal IgG) and immature <a href="/wiki/Natural_killer_cells" class="mw-redirect" title="Natural killer cells">natural killer cells</a>. These natural killer cells have a reduced lytic function; which can be improved with regular infusions of immunoglobulin (see 'Treatment'); although the mechanism for this is not clear.<sup id="cite_ref-Renner_6-0" class="reference"><a href="#cite_note-Renner-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> </p><p>Patients are more prone than healthy people to infections of all types, especially recurrent skin infections with staphylococcus. They may have more severe infections; but are not as vulnerable to opportunistic pathogens as patients with true natural killer cell deficiency-type SCID.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (October 2021)">citation needed</span></a></i>]</sup> </p> <div class="mw-heading mw-heading2"><h2 id="Cause">Cause</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Netherton_syndrome&action=edit&section=2" title="Edit section: Cause"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Netherton syndrome is an autosomal <a href="/wiki/Recessive" class="mw-redirect" title="Recessive">recessive</a> disorder associated with mutations in the <i>SPINK5</i> gene, which encodes the <a href="/wiki/Serine_protease" title="Serine protease">serine protease</a> inhibitor lympho-epithelial Kazal-type-related inhibitor (<a href="/wiki/LEKTI" title="LEKTI">LEKTI</a>).<sup id="cite_ref-pmid10835624_2-1" class="reference"><a href="#cite_note-pmid10835624-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup> These mutations result in a dysfunctional protein that has a reduced capacity to inhibit serine proteases expressed in the skin. Potential endogenous targets of LEKTI include <a href="/wiki/KLK5" class="mw-redirect" title="KLK5">KLK5</a>, <a href="/wiki/KLK7" title="KLK7">KLK7</a> and <a href="/wiki/KLK14" title="KLK14">KLK14</a>.<sup id="cite_ref-Ovaere2009_7-0" class="reference"><a href="#cite_note-Ovaere2009-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> These enzymes are involved in various aspects of epidermal remodelling, including <a href="/wiki/Desquamation" title="Desquamation">desquamation</a>, <a href="/wiki/PAR-2" class="mw-redirect" title="PAR-2">PAR-2</a> activation and degradation of lipid hydrolases, suggesting a potential mechanism for the development of atopic manifestations characteristic of Netherton syndrome.<sup id="cite_ref-pmid16601670_8-0" class="reference"><a href="#cite_note-pmid16601670-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup> </p><p>Disease severity is determined by the level of LEKTI expression and, consequently, serine protease activity. Complete SPINK5 gene deletions have been linked to severe cases, while mutations which induce <a href="/wiki/Alternate_splicing" class="mw-redirect" title="Alternate splicing">alternate splicing</a> or create <a href="/wiki/Premature_stop_codon" class="mw-redirect" title="Premature stop codon">premature stop codons</a> may lead to varying levels of severity.<sup id="cite_ref-pmid16601670_8-1" class="reference"><a href="#cite_note-pmid16601670-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup> Furthermore, LEKTI-knockout mice exhibit a phenotype similar to Netherton syndrome in humans.<sup id="cite_ref-descargues_5-1" class="reference"><a href="#cite_note-descargues-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Diagnosis">Diagnosis</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Netherton_syndrome&action=edit&section=3" title="Edit section: Diagnosis"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <style data-mw-deduplicate="TemplateStyles:r1251242444">.mw-parser-output .ambox{border:1px solid #a2a9b1;border-left:10px solid #36c;background-color:#fbfbfb;box-sizing:border-box}.mw-parser-output .ambox+link+.ambox,.mw-parser-output .ambox+link+style+.ambox,.mw-parser-output .ambox+link+link+.ambox,.mw-parser-output .ambox+.mw-empty-elt+link+.ambox,.mw-parser-output .ambox+.mw-empty-elt+link+style+.ambox,.mw-parser-output .ambox+.mw-empty-elt+link+link+.ambox{margin-top:-1px}html body.mediawiki .mw-parser-output .ambox.mbox-small-left{margin:4px 1em 4px 0;overflow:hidden;width:238px;border-collapse:collapse;font-size:88%;line-height:1.25em}.mw-parser-output .ambox-speedy{border-left:10px solid #b32424;background-color:#fee7e6}.mw-parser-output .ambox-delete{border-left:10px solid #b32424}.mw-parser-output .ambox-content{border-left:10px solid #f28500}.mw-parser-output .ambox-style{border-left:10px solid #fc3}.mw-parser-output .ambox-move{border-left:10px solid #9932cc}.mw-parser-output .ambox-protection{border-left:10px solid #a2a9b1}.mw-parser-output .ambox .mbox-text{border:none;padding:0.25em 0.5em;width:100%}.mw-parser-output .ambox .mbox-image{border:none;padding:2px 0 2px 0.5em;text-align:center}.mw-parser-output .ambox .mbox-imageright{border:none;padding:2px 0.5em 2px 0;text-align:center}.mw-parser-output .ambox .mbox-empty-cell{border:none;padding:0;width:1px}.mw-parser-output .ambox .mbox-image-div{width:52px}@media(min-width:720px){.mw-parser-output .ambox{margin:0 10%}}@media print{body.ns-0 .mw-parser-output .ambox{display:none!important}}</style><table class="box-Empty_section plainlinks metadata ambox mbox-small-left ambox-content" role="presentation"><tbody><tr><td class="mbox-image"><span typeof="mw:File"><a href="/wiki/File:Wiki_letter_w_cropped.svg" class="mw-file-description"><img alt="[icon]" src="//upload.wikimedia.org/wikipedia/commons/thumb/1/1c/Wiki_letter_w_cropped.svg/20px-Wiki_letter_w_cropped.svg.png" decoding="async" width="20" height="14" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/1/1c/Wiki_letter_w_cropped.svg/30px-Wiki_letter_w_cropped.svg.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/1/1c/Wiki_letter_w_cropped.svg/40px-Wiki_letter_w_cropped.svg.png 2x" data-file-width="44" data-file-height="31" /></a></span></td><td class="mbox-text"><div class="mbox-text-span"><b>This section is empty.</b> You can help by <a class="external text" href="https://en.wikipedia.org/w/index.php?title=Netherton_syndrome&action=edit&section=">adding to it</a>. <span class="date-container"><i>(<span class="date">May 2017</span>)</i></span></div></td></tr></tbody></table> <div class="mw-heading mw-heading2"><h2 id="Treatment">Treatment</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Netherton_syndrome&action=edit&section=4" title="Edit section: Treatment"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>There is no known cure at the moment but there are several things that can be done to relieve the symptoms. Moisturising products are very helpful to minimize the scaling/cracking, and anti-infective treatments are useful when appropriate because the skin is very susceptible to infection. Extra protein in the diet during childhood is also beneficial, to replace that which is lost through the previously mentioned "leaky" skin.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (September 2020)">citation needed</span></a></i>]</sup> </p><p>Steroid and retinoid products have been proven ineffective against Netherton syndrome, and may in fact make things worse for the affected individual.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (June 2010)">citation needed</span></a></i>]</sup> </p><p>Intravenous immunoglobulin has become established as the treatment of choice in Netherton syndrome.<sup id="cite_ref-Renner_6-1" class="reference"><a href="#cite_note-Renner-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup> This therapy reduces infection; enables improvement and even resolution of the skin and hair abnormalities, and dramatically improves quality of life of the patients; although exactly how it achieves this is not known. Given this; it is possible that the reason Netherton syndrome usually is not very severe at or shortly after birth is due to a protective effect of maternal antibodies; which cross the placenta but wane by four to six months.<sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Citation_needed" title="Wikipedia:Citation needed"><span title="This claim needs references to reliable sources. (October 2021)">citation needed</span></a></i>]</sup> </p> <div class="mw-heading mw-heading2"><h2 id="See_also">See also</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Netherton_syndrome&action=edit&section=5" title="Edit section: See also"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <ul><li><a href="/wiki/List_of_skin_conditions" title="List of skin conditions">List of skin conditions</a></li></ul> <div class="mw-heading mw-heading2"><h2 id="References">References</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Netherton_syndrome&action=edit&section=6" title="Edit section: References"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <style data-mw-deduplicate="TemplateStyles:r1239543626">.mw-parser-output .reflist{margin-bottom:0.5em;list-style-type:decimal}@media screen{.mw-parser-output .reflist{font-size:90%}}.mw-parser-output .reflist .references{font-size:100%;margin-bottom:0;list-style-type:inherit}.mw-parser-output .reflist-columns-2{column-width:30em}.mw-parser-output .reflist-columns-3{column-width:25em}.mw-parser-output .reflist-columns{margin-top:0.3em}.mw-parser-output .reflist-columns ol{margin-top:0}.mw-parser-output .reflist-columns li{page-break-inside:avoid;break-inside:avoid-column}.mw-parser-output .reflist-upper-alpha{list-style-type:upper-alpha}.mw-parser-output .reflist-upper-roman{list-style-type:upper-roman}.mw-parser-output .reflist-lower-alpha{list-style-type:lower-alpha}.mw-parser-output .reflist-lower-greek{list-style-type:lower-greek}.mw-parser-output .reflist-lower-roman{list-style-type:lower-roman}</style><div class="reflist"> <div class="mw-references-wrap"><ol class="references"> <li id="cite_note-ns05-1"><span class="mw-cite-backlink"><b><a href="#cite_ref-ns05_1-0">^</a></b></span> <span class="reference-text"><style data-mw-deduplicate="TemplateStyles:r1238218222">.mw-parser-output cite.citation{font-style:inherit;word-wrap:break-word}.mw-parser-output .citation q{quotes:"\"""\"""'""'"}.mw-parser-output .citation:target{background-color:rgba(0,127,255,0.133)}.mw-parser-output .id-lock-free.id-lock-free a{background:url("//upload.wikimedia.org/wikipedia/commons/6/65/Lock-green.svg")right 0.1em center/9px no-repeat}.mw-parser-output .id-lock-limited.id-lock-limited a,.mw-parser-output .id-lock-registration.id-lock-registration a{background:url("//upload.wikimedia.org/wikipedia/commons/d/d6/Lock-gray-alt-2.svg")right 0.1em center/9px no-repeat}.mw-parser-output .id-lock-subscription.id-lock-subscription a{background:url("//upload.wikimedia.org/wikipedia/commons/a/aa/Lock-red-alt-2.svg")right 0.1em center/9px no-repeat}.mw-parser-output .cs1-ws-icon a{background:url("//upload.wikimedia.org/wikipedia/commons/4/4c/Wikisource-logo.svg")right 0.1em center/12px no-repeat}body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-free a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-limited a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-registration a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-subscription a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .cs1-ws-icon a{background-size:contain;padding:0 1em 0 0}.mw-parser-output .cs1-code{color:inherit;background:inherit;border:none;padding:inherit}.mw-parser-output .cs1-hidden-error{display:none;color:var(--color-error,#d33)}.mw-parser-output .cs1-visible-error{color:var(--color-error,#d33)}.mw-parser-output .cs1-maint{display:none;color:#085;margin-left:0.3em}.mw-parser-output .cs1-kern-left{padding-left:0.2em}.mw-parser-output .cs1-kern-right{padding-right:0.2em}.mw-parser-output .citation .mw-selflink{font-weight:inherit}@media screen{.mw-parser-output .cs1-format{font-size:95%}html.skin-theme-clientpref-night .mw-parser-output .cs1-maint{color:#18911f}}@media screen and (prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .cs1-maint{color:#18911f}}</style><cite id="CITEREFChaoRichardLee2005" class="citation journal cs1">Chao SC, Richard G, Lee JY (2005). <a rel="nofollow" class="external text" href="https://doi.org/10.1111%2Fj.1365-2133.2005.06337.x">"Netherton syndrome: report of two Taiwanese siblings with staphylococcal scalded skin syndrome and mutation of SPINK5"</a>. <i>Br J Dermatol</i>. <b>152</b> (1): 159–165. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<span class="id-lock-free" title="Freely accessible"><a rel="nofollow" class="external text" href="https://doi.org/10.1111%2Fj.1365-2133.2005.06337.x">10.1111/j.1365-2133.2005.06337.x</a></span>. <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a> <a rel="nofollow" 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title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=Nat+Genet&rft.atitle=Spink5-deficient+mice+mimic+Netherton+syndrome+through+degradation+of+desmoglein+1+by+epidermal+protease+hyperactivity.&rft.volume=37&rft.issue=1&rft.pages=56-65&rft.date=2005-01&rft_id=https%3A%2F%2Fapi.semanticscholar.org%2FCorpusID%3A11404025%23id-name%3DS2CID&rft_id=info%3Apmid%2F15619623&rft_id=info%3Adoi%2F10.1038%2Fng1493&rft.aulast=Descargues&rft.aufirst=P&rft.au=Deraison%2C+C&rft.au=Bonnart%2C+C&rft.au=Kreft%2C+M&rft.au=Kishibe%2C+M&rft.au=Ishida-Yamamoto%2C+A&rft.au=Elias%2C+P&rft.au=Barrandon%2C+Y&rft.au=Zambruno%2C+G&rft.au=Sonnenberg%2C+A&rft.au=Hovnanian%2C+A&rft_id=https%3A%2F%2Fdoi.org%2F10.1038%252Fng1493&rfr_id=info%3Asid%2Fen.wikipedia.org%3ANetherton+syndrome" class="Z3988"></span></span> </li> <li id="cite_note-Renner-6"><span class="mw-cite-backlink">^ <a href="#cite_ref-Renner_6-0"><sup><i><b>a</b></i></sup></a> <a href="#cite_ref-Renner_6-1"><sup><i><b>b</b></i></sup></a></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFRennerHartleRylaarsdamYoung2009" class="citation journal cs1">Renner E, Hartle D, Rylaarsdam S, Young M, Monaco-Shawver L, Kleiner G, Markert ML, Stiehm ER, Belohradsky B, Upton M, Torgerson T, Orange J, Ochs H (August 2009). <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3685174">"Comel-Netherton syndrome defined as primary immunodeficiency"</a>. <i>The Journal of Allergy and Clinical Immunology</i>. <b>124</b> (3): 536–543. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<a rel="nofollow" class="external text" href="https://doi.org/10.1016%2Fj.jaci.2009.06.009">10.1016/j.jaci.2009.06.009</a>. <a href="/wiki/PMC_(identifier)" class="mw-redirect" title="PMC (identifier)">PMC</a> <span class="id-lock-free" title="Freely accessible"><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3685174">3685174</a></span>. <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a> <a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/19683336">19683336</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=The+Journal+of+Allergy+and+Clinical+Immunology&rft.atitle=Comel-Netherton+syndrome+defined+as+primary+immunodeficiency.&rft.volume=124&rft.issue=3&rft.pages=536-543&rft.date=2009-08&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC3685174%23id-name%3DPMC&rft_id=info%3Apmid%2F19683336&rft_id=info%3Adoi%2F10.1016%2Fj.jaci.2009.06.009&rft.aulast=Renner&rft.aufirst=E&rft.au=Hartle%2C+D&rft.au=Rylaarsdam%2C+S&rft.au=Young%2C+M&rft.au=Monaco-Shawver%2C+L&rft.au=Kleiner%2C+G&rft.au=Markert%2C+ML&rft.au=Stiehm%2C+ER&rft.au=Belohradsky%2C+B&rft.au=Upton%2C+M&rft.au=Torgerson%2C+T&rft.au=Orange%2C+J&rft.au=Ochs%2C+H&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC3685174&rfr_id=info%3Asid%2Fen.wikipedia.org%3ANetherton+syndrome" class="Z3988"></span></span> </li> <li id="cite_note-Ovaere2009-7"><span class="mw-cite-backlink"><b><a href="#cite_ref-Ovaere2009_7-0">^</a></b></span> <span class="reference-text"> <link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFOvaereLippensVandenabeeleDeclercq2009" class="citation journal cs1">Ovaere P, Lippens S, Vandenabeele P, Declercq W (August 2009). "The emerging roles of serine protease cascades in the epidermis". <i>Trends Biochem Sci</i>. <b>34</b> (9): 453–63. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<a rel="nofollow" class="external text" href="https://doi.org/10.1016%2Fj.tibs.2009.08.001">10.1016/j.tibs.2009.08.001</a>. <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a> <a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/19726197">19726197</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=Trends+Biochem+Sci&rft.atitle=The+emerging+roles+of+serine+protease+cascades+in+the+epidermis&rft.volume=34&rft.issue=9&rft.pages=453-63&rft.date=2009-08&rft_id=info%3Adoi%2F10.1016%2Fj.tibs.2009.08.001&rft_id=info%3Apmid%2F19726197&rft.aulast=Ovaere&rft.aufirst=P&rft.au=Lippens%2C+S&rft.au=Vandenabeele%2C+P&rft.au=Declercq%2C+W&rfr_id=info%3Asid%2Fen.wikipedia.org%3ANetherton+syndrome" class="Z3988"></span></span> </li> <li id="cite_note-pmid16601670-8"><span class="mw-cite-backlink">^ <a href="#cite_ref-pmid16601670_8-0"><sup><i><b>a</b></i></sup></a> <a href="#cite_ref-pmid16601670_8-1"><sup><i><b>b</b></i></sup></a></span> <span class="reference-text"> <link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFHachemWagbergSchmuthCrumrine2006" class="citation journal cs1">Hachem JP, Wagberg F, Schmuth M, Crumrine D, Lissens W, Jayakumar A, Houben E, Mauro TM, Leonardsson G, Brattsand M, Egelrud T, Roseeuw D, Clayman GL, Feingold KR, Williams ML, Elias PM (April 2006). <a rel="nofollow" class="external text" href="https://doi.org/10.1038%2Fsj.jid.5700288">"Serine protease activity and residual LEKTI expression determine phenotype in Netherton syndrome"</a>. <i>J Invest Dermatol</i>. <b>126</b> (7): 1609–21. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<span class="id-lock-free" title="Freely accessible"><a rel="nofollow" class="external text" href="https://doi.org/10.1038%2Fsj.jid.5700288">10.1038/sj.jid.5700288</a></span>. <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a> <a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/16601670">16601670</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=J+Invest+Dermatol&rft.atitle=Serine+protease+activity+and+residual+LEKTI+expression+determine+phenotype+in+Netherton+syndrome&rft.volume=126&rft.issue=7&rft.pages=1609-21&rft.date=2006-04&rft_id=info%3Adoi%2F10.1038%2Fsj.jid.5700288&rft_id=info%3Apmid%2F16601670&rft.aulast=Hachem&rft.aufirst=JP&rft.au=Wagberg%2C+F&rft.au=Schmuth%2C+M&rft.au=Crumrine%2C+D&rft.au=Lissens%2C+W&rft.au=Jayakumar%2C+A&rft.au=Houben%2C+E&rft.au=Mauro%2C+TM&rft.au=Leonardsson%2C+G&rft.au=Brattsand%2C+M&rft.au=Egelrud%2C+T&rft.au=Roseeuw%2C+D&rft.au=Clayman%2C+GL&rft.au=Feingold%2C+KR&rft.au=Williams%2C+ML&rft.au=Elias%2C+PM&rft_id=https%3A%2F%2Fdoi.org%2F10.1038%252Fsj.jid.5700288&rfr_id=info%3Asid%2Fen.wikipedia.org%3ANetherton+syndrome" class="Z3988"></span></span> </li> </ol></div></div> <ul><li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFYang_T,_Liang_D,_Koch_PJ,_Hohl_D,_Kheradmand_F,_Overbeek_PA2004" class="citation journal cs1">Yang T, Liang D, Koch PJ, Hohl D, Kheradmand F, Overbeek PA (October 2004). <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC522985">"Epidermal detachment, desmosomal dissociation, and destabilization of corneodesmosin in Spink5-/- mice"</a>. <i>Genes Dev</i>. <b>18</b> (19): 2354–8. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<a rel="nofollow" class="external text" href="https://doi.org/10.1101%2Fgad.1232104">10.1101/gad.1232104</a>. <a href="/wiki/PMC_(identifier)" class="mw-redirect" title="PMC (identifier)">PMC</a> <span class="id-lock-free" title="Freely accessible"><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC522985">522985</a></span>. <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a> <a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/15466487">15466487</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=Genes+Dev.&rft.atitle=Epidermal+detachment%2C+desmosomal+dissociation%2C+and+destabilization+of+corneodesmosin+in+Spink5-%2F-+mice&rft.volume=18&rft.issue=19&rft.pages=2354-8&rft.date=2004-10&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC522985%23id-name%3DPMC&rft_id=info%3Apmid%2F15466487&rft_id=info%3Adoi%2F10.1101%2Fgad.1232104&rft.au=Yang+T%2C+Liang+D%2C+Koch+PJ%2C+Hohl+D%2C+Kheradmand+F%2C+Overbeek+PA&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC522985&rfr_id=info%3Asid%2Fen.wikipedia.org%3ANetherton+syndrome" class="Z3988"></span><span class="cs1-maint citation-comment"><code class="cs1-code">{{<a href="/wiki/Template:Cite_journal" title="Template:Cite journal">cite journal</a>}}</code>: CS1 maint: multiple names: authors list (<a 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ol{counter-reset:listitem}.mw-parser-output .hlist ol>li{counter-increment:listitem}.mw-parser-output .hlist ol>li::before{content:" "counter(listitem)"\a0 "}.mw-parser-output .hlist dd ol>li:first-child::before,.mw-parser-output .hlist dt ol>li:first-child::before,.mw-parser-output .hlist li ol>li:first-child::before{content:" ("counter(listitem)"\a0 "}</style><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"></div><div role="navigation" class="navbox" aria-label="Navbox" style="width:100%; margin:0.5em 0 0.5em 0;;padding:3px"><table class="nowraplinks navbox-inner" style="border-spacing:0;background:transparent;color:inherit"><tbody><tr><th scope="row" class="navbox-group" style="width:1%;background: #EAECF0;color:black;">Classification</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"><div style="position:relative; float:right; font-size:0.8em;"><a href="https://www.wikidata.org/wiki/Q9390284" class="extiw" title="d:Q9390284">D</a></div><div class="hlist" style="text-align:left;"><ul><li><b><a href="/wiki/International_Statistical_Classification_of_Diseases_and_Related_Health_Problems" class="mw-redirect" title="International Statistical Classification of Diseases and Related Health Problems">ICD</a>-<a href="/wiki/ICD-10" title="ICD-10">10</a></b>: <a rel="nofollow" class="external text" href="https://icd.who.int/browse10/2019/en#/Q80.8">Q80.8</a> (<a href="/wiki/ILDS" class="mw-redirect" title="ILDS">ILDS</a> Q80.870)</li><li><b><a href="/wiki/International_Statistical_Classification_of_Diseases_and_Related_Health_Problems" class="mw-redirect" title="International Statistical Classification of Diseases and Related Health Problems">ICD</a>-<a href="/wiki/List_of_ICD-9_codes" title="List of ICD-9 codes">9-CM</a></b>: <a rel="nofollow" class="external text" href="http://www.icd9data.com/getICD9Code.ashx?icd9=757.1">757.1</a></li><li><b><a href="/wiki/Online_Mendelian_Inheritance_in_Man" title="Online Mendelian Inheritance in Man">OMIM</a></b>: <a rel="nofollow" class="external text" href="https://omim.org/entry/256500">256500</a></li><li><b><a href="/wiki/Medical_Subject_Headings" title="Medical Subject Headings">MeSH</a></b>: <a rel="nofollow" class="external text" href="https://meshb.nlm.nih.gov/record/ui?ui=D056770">D056770</a></li><li><b><a href="/wiki/Diseases_Database" title="Diseases Database">DiseasesDB</a></b>: <a rel="nofollow" class="external text" href="http://www.diseasesdatabase.com/ddb30791.htm">30791</a></li><li><b><a href="/wiki/SNOMED_CT" title="SNOMED CT">SNOMED CT</a></b>: <a rel="nofollow" class="external text" href="http://snomed.info/id/312514006">312514006</a></li></ul></div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%;background: #EAECF0;color:black;">External resources</th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"><div class="hlist" style="text-align:left;"><ul><li><b><a href="/wiki/EMedicine" title="EMedicine">eMedicine</a></b>: <a rel="nofollow" class="external text" href="https://emedicine.medscape.com/derm/431-overview">derm/431</a></li><li><b><a href="/wiki/Orphanet" title="Orphanet">Orphanet</a></b>: <a rel="nofollow" class="external text" href="https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=634">634</a></li></ul></div></div></td></tr></tbody></table></div> <div class="navbox-styles"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1236075235"></div><div role="navigation" class="navbox" aria-labelledby="Congenital_malformations_and_deformations_of_integument_/_skin_disease" style="padding:3px"><table class="nowraplinks mw-collapsible autocollapse navbox-inner" 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href="/wiki/Special:EditPage/Template:Congenital_malformations_and_deformations_of_integument" title="Special:EditPage/Template:Congenital malformations and deformations of integument"><abbr title="Edit this template">e</abbr></a></li></ul></div><div id="Congenital_malformations_and_deformations_of_integument_/_skin_disease" style="font-size:114%;margin:0 4em"><a href="/wiki/Congenital_disorder" class="mw-redirect" title="Congenital disorder">Congenital</a> malformations and deformations of <a href="/wiki/Integument" title="Integument">integument</a> / <a href="/wiki/Skin_disease" class="mw-redirect" title="Skin disease">skin disease</a></div></th></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Genodermatosis" title="Genodermatosis">Genodermatosis</a></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th id="Congenital_ichthyosis/erythrokeratodermia" scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Congenital_ichthyosis" class="mw-redirect" title="Congenital ichthyosis">Congenital ichthyosis</a>/<br /><a href="/wiki/Erythrokeratodermia" title="Erythrokeratodermia">erythrokeratodermia</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Autosomal_dominant" class="mw-redirect" title="Autosomal dominant">AD</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Ichthyosis_vulgaris" title="Ichthyosis vulgaris">Ichthyosis vulgaris</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Autosomal_recessive" class="mw-redirect" title="Autosomal recessive">AR</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Congenital_ichthyosiform_erythroderma" title="Congenital ichthyosiform erythroderma">Congenital ichthyosiform erythroderma</a>: <a href="/wiki/Epidermolytic_hyperkeratosis" title="Epidermolytic hyperkeratosis">Epidermolytic hyperkeratosis</a></li> <li><a href="/wiki/Lamellar_ichthyosis" title="Lamellar ichthyosis">Lamellar ichthyosis</a> <ul><li><a href="/wiki/Harlequin-type_ichthyosis" title="Harlequin-type ichthyosis">Harlequin-type ichthyosis</a></li></ul></li></ul> <ul><li><a class="mw-selflink selflink">Netherton syndrome</a></li> <li><a href="/wiki/CHIME_syndrome" title="CHIME syndrome">CHIME syndrome</a></li> <li><a href="/wiki/Sj%C3%B6gren%E2%80%93Larsson_syndrome" title="Sjögren–Larsson syndrome">Sjögren–Larsson syndrome</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/X-linked_recessive" class="mw-redirect" title="X-linked recessive">XR</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/X-linked_ichthyosis" title="X-linked ichthyosis">X-linked ichthyosis</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Ungrouped</th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Ichthyosis_bullosa_of_Siemens" title="Ichthyosis bullosa of Siemens">Ichthyosis bullosa of Siemens</a></li> <li><a href="/wiki/Ichthyosis_follicularis" class="mw-redirect" title="Ichthyosis follicularis">Ichthyosis follicularis</a></li> <li><a href="/wiki/Ichthyosis_prematurity_syndrome" title="Ichthyosis prematurity syndrome">Ichthyosis prematurity syndrome</a></li> <li><a href="/wiki/Ichthyosis%E2%80%93sclerosing_cholangitis_syndrome" class="mw-redirect" title="Ichthyosis–sclerosing cholangitis syndrome">Ichthyosis–sclerosing cholangitis syndrome</a></li> <li><a href="/wiki/Nonbullous_congenital_ichthyosiform_erythroderma" class="mw-redirect" title="Nonbullous congenital ichthyosiform erythroderma">Nonbullous congenital ichthyosiform erythroderma</a></li> <li><a href="/wiki/Ichthyosis_linearis_circumflexa" title="Ichthyosis linearis circumflexa">Ichthyosis linearis circumflexa</a></li> <li><a href="/wiki/Ichthyosis_hystrix" title="Ichthyosis hystrix">Ichthyosis hystrix</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Epidermolysis_bullosa" title="Epidermolysis bullosa">EB</a><br />and related</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Epidermolysis_bullosa_simplex" title="Epidermolysis bullosa simplex">EBS</a> <ul><li><a href="/wiki/Generalized_epidermolysis_bullosa_simplex" class="mw-redirect" title="Generalized epidermolysis bullosa simplex">EBS-K</a></li> <li><a href="/wiki/Localized_epidermolysis_bullosa_simplex" class="mw-redirect" title="Localized epidermolysis bullosa simplex">EBS-WC</a></li> <li><a href="/wiki/Epidermolysis_bullosa_herpetiformis" class="mw-redirect" title="Epidermolysis bullosa herpetiformis">EBS-DM</a></li> <li><a href="/wiki/Epidermolysis_bullosa_simplex_of_Ogna" class="mw-redirect" title="Epidermolysis bullosa simplex of Ogna">EBS-OG</a></li> <li><a href="/wiki/Epidermolysis_bullosa_simplex_with_muscular_dystrophy" class="mw-redirect" title="Epidermolysis bullosa simplex with muscular dystrophy">EBS-MD</a></li> <li><a href="/wiki/Epidermolysis_bullosa_simplex_with_mottled_pigmentation" class="mw-redirect" title="Epidermolysis bullosa simplex with mottled pigmentation">EBS-MP</a></li></ul></li></ul> <ul><li><a href="/wiki/Junctional_epidermolysis_bullosa_(medicine)" title="Junctional epidermolysis bullosa (medicine)">JEB</a> <ul><li><a href="/wiki/Junctional_epidermolysis_bullosa_gravis" class="mw-redirect" title="Junctional epidermolysis bullosa gravis">JEB-H</a></li> <li><a href="/wiki/Junctional_epidermolysis_bullosa_(medicine)" title="Junctional epidermolysis bullosa (medicine)">Mitis</a></li> <li><a href="/wiki/Junctional_epidermolysis_bullosa_(medicine)" title="Junctional epidermolysis bullosa (medicine)">Generalized atrophic</a></li> <li><a href="/wiki/Junctional_epidermolysis_bullosa_with_pyloric_atresia" class="mw-redirect" title="Junctional epidermolysis bullosa with pyloric atresia">JEB-PA</a></li></ul></li></ul> <ul><li><a href="/wiki/Epidermolysis_bullosa_dystrophica" title="Epidermolysis bullosa dystrophica">DEB</a> <ul><li><a href="/wiki/Dominant_dystrophic_epidermolysis_bullosa" class="mw-redirect" title="Dominant dystrophic epidermolysis bullosa">DDEB</a></li> <li><a href="/wiki/Recessive_dystrophic_epidermolysis_bullosa" class="mw-redirect" title="Recessive dystrophic epidermolysis bullosa">RDEB</a></li></ul></li></ul> <ul><li>related: <a href="/wiki/Costello_syndrome" title="Costello syndrome">Costello syndrome</a></li> <li><a href="/wiki/Kindler_syndrome" title="Kindler syndrome">Kindler syndrome</a></li> <li><a href="/wiki/Laryngoonychocutaneous_syndrome" class="mw-redirect" title="Laryngoonychocutaneous syndrome">Laryngoonychocutaneous syndrome</a></li> <li><a href="/wiki/Skin_fragility_syndrome" title="Skin fragility syndrome">Skin fragility syndrome</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Ectodermal_dysplasia" title="Ectodermal dysplasia">Ectodermal dysplasia</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Naegeli_syndrome" class="mw-redirect" title="Naegeli syndrome">Naegeli syndrome</a>/<a href="/wiki/Dermatopathia_pigmentosa_reticularis" title="Dermatopathia pigmentosa reticularis">Dermatopathia pigmentosa reticularis</a></li> <li><a href="/wiki/Hay%E2%80%93Wells_syndrome" title="Hay–Wells syndrome">Hay–Wells syndrome</a></li> <li><a href="/wiki/Hypohidrotic_ectodermal_dysplasia" title="Hypohidrotic ectodermal dysplasia">Hypohidrotic ectodermal dysplasia</a></li> <li><a href="/wiki/Focal_dermal_hypoplasia" title="Focal dermal hypoplasia">Focal dermal hypoplasia</a></li> <li><a href="/wiki/Ellis%E2%80%93van_Creveld_syndrome" class="mw-redirect" title="Ellis–van Creveld syndrome">Ellis–van Creveld syndrome</a></li> <li><a href="/wiki/Rapp%E2%80%93Hodgkin_syndrome" title="Rapp–Hodgkin syndrome">Rapp–Hodgkin syndrome</a>/<a href="/wiki/Hay%E2%80%93Wells_syndrome" title="Hay–Wells syndrome">Hay–Wells syndrome</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Elastic_fiber" title="Elastic fiber">Elastic</a>/<a href="/wiki/Connective_tissue_disease" title="Connective tissue disease">Connective</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Ehlers%E2%80%93Danlos_syndrome" title="Ehlers–Danlos syndrome">Ehlers–Danlos syndrome</a></li> <li><a href="/wiki/Cutis_laxa" title="Cutis laxa">Cutis laxa</a> (<a href="/wiki/Gerodermia_osteodysplastica" title="Gerodermia osteodysplastica">Gerodermia osteodysplastica</a>)</li> <li><a href="/wiki/Popliteal_pterygium_syndrome" title="Popliteal pterygium syndrome">Popliteal pterygium syndrome</a></li> <li><a href="/wiki/Pseudoxanthoma_elasticum" title="Pseudoxanthoma elasticum">Pseudoxanthoma elasticum</a></li> <li><a href="/wiki/Van_der_Woude_syndrome" title="Van der Woude syndrome">Van der Woude syndrome</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Hyperkeratosis" title="Hyperkeratosis">Hyperkeratosis</a>/<br /><a href="/wiki/Keratin_disease" title="Keratin disease">keratinopathy</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Palmoplantar_keratoderma" title="Palmoplantar keratoderma">PPK</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Palmoplantar_keratoderma" title="Palmoplantar keratoderma">diffuse</a>: <a href="/wiki/Palmoplantar_keratoderma" title="Palmoplantar keratoderma">Diffuse epidermolytic palmoplantar keratoderma</a></li> <li><a href="/wiki/Palmoplantar_keratoderma" title="Palmoplantar keratoderma">Diffuse nonepidermolytic palmoplantar keratoderma</a></li> <li><a href="/wiki/Palmoplantar_keratoderma" title="Palmoplantar keratoderma">Palmoplantar keratoderma of Sybert</a></li> <li><a href="/wiki/Meleda_disease" title="Meleda disease">Meleda disease</a></li></ul> <ul><li>syndromic <ul><li><i><a href="/wiki/Connexin" title="Connexin">connexin</a></i> <ul><li><a href="/wiki/Bart%E2%80%93Pumphrey_syndrome" title="Bart–Pumphrey syndrome">Bart–Pumphrey syndrome</a></li> <li><a href="/wiki/Clouston%27s_hidrotic_ectodermal_dysplasia" title="Clouston's hidrotic ectodermal dysplasia">Clouston's hidrotic ectodermal dysplasia</a></li> <li><a href="/wiki/Vohwinkel_syndrome" class="mw-redirect" title="Vohwinkel syndrome">Vohwinkel syndrome</a></li></ul></li> <li><a href="/wiki/Corneodermatoosseous_syndrome" title="Corneodermatoosseous syndrome">Corneodermatoosseous syndrome</a></li> <li><i><a href="/wiki/Plakoglobin" title="Plakoglobin">plakoglobin</a></i> <ul><li><a href="/wiki/Naxos_syndrome" title="Naxos syndrome">Naxos syndrome</a></li></ul></li> <li><a href="/wiki/Scleroatrophic_syndrome_of_Huriez" class="mw-redirect" title="Scleroatrophic syndrome of Huriez">Scleroatrophic syndrome of Huriez</a></li> <li><a href="/wiki/Olmsted_syndrome" class="mw-redirect" title="Olmsted syndrome">Olmsted syndrome</a></li> <li><i><a href="/wiki/Cathepsin_C" title="Cathepsin C">Cathepsin C</a></i> <ul><li><a href="/wiki/Papillon%E2%80%93Lef%C3%A8vre_syndrome" title="Papillon–Lefèvre syndrome">Papillon–Lefèvre syndrome</a></li> <li><a href="/wiki/Haim%E2%80%93Munk_syndrome" title="Haim–Munk syndrome">Haim–Munk syndrome</a></li></ul></li> <li><a href="/wiki/Camisa_disease" title="Camisa disease">Camisa disease</a></li> <li><a href="/wiki/Palmoplantar_keratoderma" title="Palmoplantar keratoderma">focal</a>: <a href="/wiki/Palmoplantar_keratoderma" title="Palmoplantar keratoderma">Focal palmoplantar keratoderma with oral mucosal hyperkeratosis</a></li> <li><a href="/wiki/Focal_palmoplantar_and_gingival_keratosis" title="Focal palmoplantar and gingival keratosis">Focal palmoplantar and gingival keratosis</a></li> <li><a href="/wiki/Howel%E2%80%93Evans_syndrome" title="Howel–Evans syndrome">Howel–Evans syndrome</a></li> <li><a href="/wiki/Pachyonychia_congenita" title="Pachyonychia congenita">Pachyonychia congenita</a> <ul><li><a href="/wiki/Pachyonychia_congenita_type_I" class="mw-redirect" title="Pachyonychia congenita type I">Pachyonychia congenita type I</a></li> <li><a href="/wiki/Pachyonychia_congenita_type_II" class="mw-redirect" title="Pachyonychia congenita type II">Pachyonychia congenita type II</a></li></ul></li> <li><a href="/wiki/Palmoplantar_keratoderma" title="Palmoplantar keratoderma">Striate palmoplantar keratoderma</a></li> <li><a href="/wiki/Tyrosinemia_type_II" title="Tyrosinemia type II">Tyrosinemia type II</a></li></ul></li></ul> <ul><li><a href="/wiki/Palmoplantar_keratoderma" title="Palmoplantar keratoderma">punctate</a>: <a href="/wiki/Acrokeratoelastoidosis_of_Costa" title="Acrokeratoelastoidosis of Costa">Acrokeratoelastoidosis of Costa</a></li> <li><a href="/wiki/Focal_acral_hyperkeratosis" class="mw-redirect" title="Focal acral hyperkeratosis">Focal acral hyperkeratosis</a></li> <li><a href="/wiki/Keratosis_punctata_palmaris_et_plantaris" class="mw-redirect" title="Keratosis punctata palmaris et plantaris">Keratosis punctata palmaris et plantaris</a></li> <li><a href="/wiki/Keratosis_punctata_of_the_palmar_creases" title="Keratosis punctata of the palmar creases">Keratosis punctata of the palmar creases</a></li> <li><a href="/wiki/Sch%C3%B6pf%E2%80%93Schulz%E2%80%93Passarge_syndrome" title="Schöpf–Schulz–Passarge syndrome">Schöpf–Schulz–Passarge syndrome</a></li> <li><a href="/wiki/Porokeratosis_plantaris_discreta" class="mw-redirect" title="Porokeratosis plantaris discreta">Porokeratosis plantaris discreta</a></li> <li><a href="/wiki/Spiny_keratoderma" class="mw-redirect" title="Spiny keratoderma">Spiny keratoderma</a></li></ul> <ul><li>ungrouped: <a href="/wiki/Palmoplantar_keratoderma" title="Palmoplantar keratoderma">Palmoplantar keratoderma and spastic paraplegia</a></li> <li><i><a href="/wiki/Desmoplakin" title="Desmoplakin">desmoplakin</a></i> <ul><li><a href="/wiki/Palmoplantar_keratoderma" title="Palmoplantar keratoderma">Carvajal syndrome</a></li></ul></li> <li><i><a href="/wiki/Connexin" title="Connexin">connexin</a></i> <ul><li><a href="/wiki/Erythrokeratodermia_variabilis" title="Erythrokeratodermia variabilis">Erythrokeratodermia variabilis</a></li> <li><a href="/wiki/Hystrix-like_ichthyosis%E2%80%93deafness_syndrome" title="Hystrix-like ichthyosis–deafness syndrome">HID</a>/<a href="/wiki/Keratitis%E2%80%93ichthyosis%E2%80%93deafness_syndrome" title="Keratitis–ichthyosis–deafness syndrome">KID</a></li></ul></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Other</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Meleda_disease" title="Meleda disease">Meleda disease</a></li> <li><a href="/wiki/Keratosis_pilaris" title="Keratosis pilaris">Keratosis pilaris</a></li> <li><i><a href="/wiki/ATP2A2" title="ATP2A2">ATP2A2</a></i> <ul><li><a href="/wiki/Darier%27s_disease" title="Darier's disease">Darier's disease</a></li></ul></li> <li><a href="/wiki/Dyskeratosis_congenita" title="Dyskeratosis congenita">Dyskeratosis congenita</a></li> <li><a href="/wiki/Lelis_syndrome" title="Lelis syndrome">Lelis syndrome</a></li></ul> <ul><li><a href="/wiki/Dyskeratosis_congenita" title="Dyskeratosis congenita">Dyskeratosis congenita</a></li> <li><a href="/wiki/Keratolytic_winter_erythema" title="Keratolytic winter erythema">Keratolytic winter erythema</a></li> <li><a href="/wiki/Keratosis_follicularis_spinulosa_decalvans" title="Keratosis follicularis spinulosa decalvans">Keratosis follicularis spinulosa decalvans</a></li> <li><a href="/wiki/Keratosis_linearis_with_ichthyosis_congenita_and_sclerosing_keratoderma_syndrome" title="Keratosis linearis with ichthyosis congenita and sclerosing keratoderma syndrome">Keratosis linearis with ichthyosis congenita and sclerosing keratoderma syndrome</a></li> <li><a href="/wiki/Keratosis_pilaris_atrophicans_faciei" title="Keratosis pilaris atrophicans faciei">Keratosis pilaris atrophicans faciei</a></li> <li><a href="/wiki/Keratosis_pilaris" title="Keratosis pilaris">Keratosis pilaris</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Other</th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><i><a href="/wiki/Cadherin" title="Cadherin">cadherin</a></i> <ul><li><a href="/wiki/EEM_syndrome" title="EEM syndrome">EEM syndrome</a></li></ul></li> <li><i><a href="/wiki/Immune_system" title="Immune system">immune system</a></i> <ul><li><a href="/wiki/Lymphedema" title="Lymphedema">Hereditary lymphedema</a></li> <li><a href="/wiki/Mastocytosis" title="Mastocytosis">Mastocytosis</a>/<a href="/wiki/Urticaria_pigmentosa" title="Urticaria pigmentosa">Urticaria pigmentosa</a></li></ul></li> <li><a href="/wiki/Hailey%E2%80%93Hailey_disease" title="Hailey–Hailey disease">Hailey–Hailey</a></li></ul> <p><i>see also <a href="/wiki/Template:Congenital_malformations_and_deformations_of_skin_appendages" title="Template:Congenital malformations and deformations of skin appendages">Template:Congenital malformations and deformations of skin appendages</a>, <a href="/wiki/Template:Phakomatoses" title="Template:Phakomatoses">Template:Phakomatoses</a></i>, <i><a href="/wiki/Template:Pigmentation_disorders" title="Template:Pigmentation disorders">Template:Pigmentation disorders</a></i>, <i><a href="/wiki/Template:DNA_replication_and_repair-deficiency_disorder" title="Template:DNA replication and repair-deficiency disorder">Template:DNA replication and repair-deficiency disorder</a></i> </p> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Developmental<br />anomalies</th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%">Midline</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Dermoid_cyst" title="Dermoid cyst">Dermoid cyst</a></li> <li><a href="/wiki/Encephalocele" title="Encephalocele">Encephalocele</a></li> <li><a href="/wiki/Nasal_glioma" class="mw-redirect" title="Nasal glioma">Nasal glioma</a></li> <li><a href="/wiki/PHACE_association" class="mw-redirect" title="PHACE association">PHACE association</a></li> <li><a href="/wiki/Sinus_pericranii" title="Sinus pericranii">Sinus pericranii</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Nevus" title="Nevus">Nevus</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Capillary_hemangioma" class="mw-redirect" title="Capillary hemangioma">Capillary hemangioma</a></li> <li><a href="/wiki/Port-wine_stain" title="Port-wine stain">Port-wine stain</a> <ul><li><a href="/wiki/Nevus_flammeus_nuchae" title="Nevus flammeus nuchae">Nevus flammeus nuchae</a></li></ul></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Other/ungrouped</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Aplasia_cutis_congenita" title="Aplasia cutis congenita">Aplasia cutis congenita</a></li> <li><a href="/wiki/Amniotic_band_syndrome" class="mw-redirect" title="Amniotic band syndrome">Amniotic band syndrome</a></li> <li><a href="/wiki/Branchial_cyst" class="mw-redirect" title="Branchial cyst">Branchial cyst</a></li> <li><a href="/wiki/Cavernous_venous_malformation" class="mw-redirect" title="Cavernous venous malformation">Cavernous venous malformation</a></li></ul> <ul><li><a href="/wiki/Accessory_nail_of_the_fifth_toe" title="Accessory nail of the fifth toe">Accessory nail of the fifth toe</a></li> <li><a href="/wiki/Bronchogenic_cyst" title="Bronchogenic cyst">Bronchogenic cyst</a></li> <li><a href="/wiki/Congenital_cartilaginous_rest_of_the_neck" title="Congenital cartilaginous rest of the neck">Congenital cartilaginous rest of the neck</a></li> <li><a href="/wiki/Congenital_hypertrophy_of_the_lateral_fold_of_the_hallux" title="Congenital hypertrophy of the lateral fold of the hallux">Congenital hypertrophy of the lateral fold of the hallux</a></li> <li><a href="/wiki/Congenital_lip_pit" title="Congenital lip pit">Congenital lip pit</a></li> <li><a href="/wiki/Congenital_malformations_of_the_dermatoglyphs" title="Congenital malformations of the dermatoglyphs">Congenital malformations of the dermatoglyphs</a></li> <li><a href="/wiki/Congenital_preauricular_fistula" class="mw-redirect" title="Congenital preauricular fistula">Congenital preauricular fistula</a></li> <li><a href="/wiki/Congenital_smooth_muscle_hamartoma" title="Congenital smooth muscle hamartoma">Congenital smooth muscle hamartoma</a></li> <li><a href="/wiki/Cystic_lymphatic_malformation" class="mw-redirect" title="Cystic lymphatic malformation">Cystic lymphatic malformation</a></li> <li><a href="/wiki/Median_raphe_cyst" title="Median raphe cyst">Median raphe cyst</a></li> <li><a href="/wiki/Melanotic_neuroectodermal_tumor_of_infancy" title="Melanotic neuroectodermal tumor of infancy">Melanotic neuroectodermal tumor of infancy</a></li> <li><a href="/wiki/Mongolian_spot" title="Mongolian spot">Mongolian spot</a></li> <li><a href="/wiki/Nasolacrimal_duct_cyst" title="Nasolacrimal duct cyst">Nasolacrimal duct cyst</a></li> <li><a href="/wiki/Omphalomesenteric_duct_cyst" title="Omphalomesenteric duct cyst">Omphalomesenteric duct cyst</a></li> <li><a href="/wiki/Poland_anomaly" class="mw-redirect" title="Poland anomaly">Poland anomaly</a></li> <li><a href="/wiki/Rapidly_involuting_congenital_hemangioma" class="mw-redirect" title="Rapidly involuting congenital hemangioma">Rapidly involuting congenital hemangioma</a></li> <li><a href="/wiki/Rosenthal%E2%80%93Kloepfer_syndrome" title="Rosenthal–Kloepfer syndrome">Rosenthal–Kloepfer syndrome</a></li> <li><a href="/wiki/Skin_dimple" title="Skin dimple">Skin dimple</a></li> <li><a href="/wiki/Superficial_lymphatic_malformation" class="mw-redirect" title="Superficial lymphatic malformation">Superficial lymphatic malformation</a></li> <li><a href="/wiki/Thyroglossal_duct_cyst" class="mw-redirect" title="Thyroglossal duct cyst">Thyroglossal duct cyst</a></li> <li><a href="/wiki/Verrucous_vascular_malformation" class="mw-redirect" title="Verrucous vascular malformation">Verrucous vascular malformation</a></li> <li><a href="/wiki/Birthmark" title="Birthmark">Birthmark</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr></tbody></table></div>    </div><noscript><img src="https://login.wikimedia.org/wiki/Special:CentralAutoLogin/start?type=1x1" alt="" width="1" height="1" style="border: none; position: absolute;"></noscript> <div class="printfooter" data-nosnippet="">Retrieved from "<a dir="ltr" href="https://en.wikipedia.org/w/index.php?title=Netherton_syndrome&oldid=1253559601">https://en.wikipedia.org/w/index.php?title=Netherton_syndrome&oldid=1253559601</a>"</div></div> <div id="catlinks" class="catlinks" data-mw="interface"><div id="mw-normal-catlinks" class="mw-normal-catlinks"><a href="/wiki/Help:Category" title="Help:Category">Categories</a>: <ul><li><a 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