{"id":899,"date":"2023-01-05T21:37:23","date_gmt":"2023-01-06T02:37:23","guid":{"rendered":"https:\/\/pressbooks.bccampus.ca\/pedsdermprimer\/?post_type=chapter&p=899"},"modified":"2023-01-05T22:07:43","modified_gmt":"2023-01-06T03:07:43","slug":"genodermatoses-tuberous-sclerosis","status":"publish","type":"chapter","link":"https:\/\/pressbooks.bccampus.ca\/pedsdermprimer\/chapter\/genodermatoses-tuberous-sclerosis\/","title":{"raw":"Genodermatoses: Tuberous Sclerosis","rendered":"Genodermatoses: Tuberous Sclerosis"},"content":{"raw":"
\r\n
\r\n

Tuberous Sclerosis<\/h1>\r\n<\/div>\r\n
\r\n\r\nSimilar to NF-1, tuberous sclerosis presents with skin findings in addition to a constellation of other changes.\u00a0 It is caused by mutation in the TSC gene.\r\n
\r\n
\r\n
\r\n\r\nSelected criteria for TSC1 include:\u00a0<\/strong>\r\n\r\n<\/div>\r\n<\/div>\r\n<\/header>\r\n
\r\n
\r\n
\r\n\r\nMajor criteria\r\n\r\n<\/div>\r\n
\r\n
    \r\n \t
  • Hypomelanotic macules at least 5 mm in diameter<\/li>\r\n \t
  • Facial angiofibromas or fibrous cephalic plaque<\/li>\r\n \t
  • Periungual fibromas<\/li>\r\n \t
  • Shagreen patch (connective tissue nevus)<\/li>\r\n \t
  • Multiple retinal hamartomas<\/li>\r\n \t
  • Cortical dysplasias<\/li>\r\n \t
  • Subependymal nodules<\/li>\r\n \t
  • Subependymal giant cell astrocytoma<\/li>\r\n \t
  • Cardiac rhabdomyosma<\/li>\r\n \t
  • Lymphangioleiomyomatosis<\/li>\r\n \t
  • Angiomyolipomas<\/li>\r\n<\/ul>\r\n<\/div>\r\n<\/div>\r\n
    \r\n
    <\/div>\r\n<\/div>\r\n
    \r\n
    <\/div>\r\n<\/div>\r\n
    \r\n
    \r\n\r\nMinor features\r\n\r\n<\/div>\r\n
    \r\n
      \r\n \t
    • Hypopigmented macules (\u201cconfetti\u201d macules)<\/li>\r\n \t
    • Dental enamel pits<\/li>\r\n<\/ul>\r\n<\/div>\r\n<\/div>\r\n
      \r\n
      <\/div>\r\n<\/div>\r\n<\/div>\r\n<\/div>\r\n \r\n\r\n<\/div>\r\n<\/div>\r\n\r\n[caption id=\"attachment_922\" align=\"aligncenter\" width=\"300\"]\"\" Image 9.11: Tuberous sclerosis: Periungual fibroma[\/caption]","rendered":"
      \n
      \n

      Tuberous Sclerosis<\/h1>\n<\/div>\n
      \n

      Similar to NF-1, tuberous sclerosis presents with skin findings in addition to a constellation of other changes.\u00a0 It is caused by mutation in the TSC gene.<\/p>\n

      \n
      \n
      \n
      \n

      Selected criteria for TSC1 include:\u00a0<\/strong><\/p>\n<\/div>\n<\/div>\n<\/header>\n

      \n
      \n
      \n

      Major criteria<\/p>\n<\/div>\n

      \n
        \n
      • Hypomelanotic macules at least 5 mm in diameter<\/li>\n
      • Facial angiofibromas or fibrous cephalic plaque<\/li>\n
      • Periungual fibromas<\/li>\n
      • Shagreen patch (connective tissue nevus)<\/li>\n
      • Multiple retinal hamartomas<\/li>\n
      • Cortical dysplasias<\/li>\n
      • Subependymal nodules<\/li>\n
      • Subependymal giant cell astrocytoma<\/li>\n
      • Cardiac rhabdomyosma<\/li>\n
      • Lymphangioleiomyomatosis<\/li>\n
      • Angiomyolipomas<\/li>\n<\/ul>\n<\/div>\n<\/div>\n
        \n
        <\/div>\n<\/div>\n
        \n
        <\/div>\n<\/div>\n
        \n
        \n

        Minor features<\/p>\n<\/div>\n

        \n
          \n
        • Hypopigmented macules (\u201cconfetti\u201d macules)<\/li>\n
        • Dental enamel pits<\/li>\n<\/ul>\n<\/div>\n<\/div>\n
          \n
          <\/div>\n<\/div>\n<\/div>\n<\/div>\n

           <\/p>\n<\/div>\n<\/div>\n

          \"\"
          Image 9.11: Tuberous sclerosis: Periungual fibroma<\/figcaption><\/figure>\n","protected":false},"author":1682,"menu_order":7,"template":"","meta":{"pb_show_title":"on","pb_short_title":"","pb_subtitle":"","pb_authors":[],"pb_section_license":""},"chapter-type":[],"contributor":[],"license":[],"class_list":["post-899","chapter","type-chapter","status-publish","hentry"],"part":885,"_links":{"self":[{"href":"https:\/\/pressbooks.bccampus.ca\/pedsdermprimer\/wp-json\/pressbooks\/v2\/chapters\/899","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/pressbooks.bccampus.ca\/pedsdermprimer\/wp-json\/pressbooks\/v2\/chapters"}],"about":[{"href":"https:\/\/pressbooks.bccampus.ca\/pedsdermprimer\/wp-json\/wp\/v2\/types\/chapter"}],"author":[{"embeddable":true,"href":"https:\/\/pressbooks.bccampus.ca\/pedsdermprimer\/wp-json\/wp\/v2\/users\/1682"}],"version-history":[{"count":2,"href":"https:\/\/pressbooks.bccampus.ca\/pedsdermprimer\/wp-json\/pressbooks\/v2\/chapters\/899\/revisions"}],"predecessor-version":[{"id":930,"href":"https:\/\/pressbooks.bccampus.ca\/pedsdermprimer\/wp-json\/pressbooks\/v2\/chapters\/899\/revisions\/930"}],"part":[{"href":"https:\/\/pressbooks.bccampus.ca\/pedsdermprimer\/wp-json\/pressbooks\/v2\/parts\/885"}],"metadata":[{"href":"https:\/\/pressbooks.bccampus.ca\/pedsdermprimer\/wp-json\/pressbooks\/v2\/chapters\/899\/metadata\/"}],"wp:attachment":[{"href":"https:\/\/pressbooks.bccampus.ca\/pedsdermprimer\/wp-json\/wp\/v2\/media?parent=899"}],"wp:term":[{"taxonomy":"chapter-type","embeddable":true,"href":"https:\/\/pressbooks.bccampus.ca\/pedsdermprimer\/wp-json\/pressbooks\/v2\/chapter-type?post=899"},{"taxonomy":"contributor","embeddable":true,"href":"https:\/\/pressbooks.bccampus.ca\/pedsdermprimer\/wp-json\/wp\/v2\/contributor?post=899"},{"taxonomy":"license","embeddable":true,"href":"https:\/\/pressbooks.bccampus.ca\/pedsdermprimer\/wp-json\/wp\/v2\/license?post=899"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}