{"id":4158,"date":"2020-11-11T02:58:02","date_gmt":"2020-11-11T02:58:02","guid":{"rendered":"https:\/\/dermatly.com\/sindrome-de-brooke-spiegler\/"},"modified":"2026-03-10T10:12:32","modified_gmt":"2026-03-10T10:12:32","slug":"sindrome-di-brooke-spiegler","status":"publish","type":"glossary","link":"https:\/\/dermatly.com\/it\/sindrome-de-brooke-spiegler\/","title":{"rendered":"Sindrome di Brooke-Spiegler"},"content":{"rendered":"<section class=\"textBlock\">\n<h2>\u00bfQu\u00e9 es Brooke-Spiegler? <span class=\"term\" data-term-id=\"350\">s\u00edndrome<\/span>?<\/h2>\n<p>El s\u00edndrome de Brooke-Spiegler (BRSS o BSS) es una rara <span class=\"term\" data-term-id=\"558\">gen\u00e9tico<\/span> condici\u00f3n que resulta en una variedad de tumores derivados de la piel <span class=\"term\" data-term-id=\"14\">ap\u00e9ndices<\/span> (<span class=\"term\" data-term-id=\"122\">cabello<\/span> <span class=\"term\" data-term-id=\"1300\">fol\u00edculo<\/span> tumores y sudor <span class=\"term\" data-term-id=\"2388\">gl\u00e1ndula<\/span> tumores).  El s\u00edndrome incluye las variantes limitadas, <span class=\"term\" data-term-id=\"849\">familiar<\/span> cilindromatosis y tricoepiteliomas familiares m\u00faltiples (MFT1).<\/p>\n<h2>\u00bfQu\u00e9 causa el s\u00edndrome de Brooke-Spiegler?<\/h2>\n<p>El s\u00edndrome de Brooke-Spiegler, la cilindromatosis familiar y los tricoepiteliomas familiares m\u00faltiples se deben a <span class=\"term\" data-term-id=\"776\">l\u00ednea germinal<\/span> <span class=\"term\" data-term-id=\"871\">mutaciones<\/span> en la cilindromatosis (CYLD) <span class=\"term\" data-term-id=\"774\">gene<\/span> en <span class=\"term\" data-term-id=\"1271\">cromosoma<\/span> 16q12.  Estos trastornos tienen una <span class=\"term\" data-term-id=\"828\">autos\u00f3mico<\/span> herencia dominante, lo que significa que la mitad de los hijos de una persona afectada tambi\u00e9n tendr\u00e1n la enfermedad.<\/p>\n<p>CYLD funciona como <span class=\"term\" data-term-id=\"289\">tumor<\/span> gen supresor y tiene funciones reguladoras en <span class=\"term\" data-term-id=\"2341\">desarrollo<\/span>, inmunidad y <span class=\"term\" data-term-id=\"148\">inflamaci\u00f3n<\/span>.  Hasta la fecha, se han encontrado un total de 51 mutaciones de CYLD de l\u00ednea germinal.  Se ha informado de una amplia gama de antecedentes \u00e9tnicos y raciales en los pacientes y sus familias afectados.<\/p>\n<div id=\"square-placement-country-holder\" class=\"country-dependent advert\">\n<div class=\"box-placement square-placement\">\n<div class=\"advert__frame\">\n                        <!-- \/340621630\/dermnet_mobile_afterfirstsection_300x250 --><\/p>\n<div style=\"display: none;\" id=\"hidden-div-gpt-ad-1484122037505-8\" class=\"[ js-hideable ]\"><\/div>\n<p><script>\n    if (ads_screenwidth < 780 ) {\n        var inline = document.createElement(\"div\");\n        var ad = document.createElement(\"div\");\n\n        ad.id = 'div-gpt-ad-1484122037505-8';\n        ad.style.height=\"250px\";\n        ad.style.width=\"300px;\";\n\n        inline.appendChild(ad);\n        inline.className = \"inline\";\n        var placeholder = document.getElementById(\"hidden-div-gpt-ad-1484122037505-8\");\n        placeholder.parentNode.replaceChild(inline, placeholder);\n        (function () {\n            googletag.cmd.push(function () {\n                googletag.display('div-gpt-ad-1484122037505-8');\n            });\n        })();\n    }\n<\/script>\n                    <\/div>\n<\/p><\/div>\n<\/p><\/div>\n<h2>\u00bfCu\u00e1les son las caracter\u00edsticas del s\u00edndrome de Brooke-Spiegler?<\/h2>\n<p>El s\u00edndrome de Brooke-Spiegler resulta en un <span class=\"term\" data-term-id=\"1494\">predisposici\u00f3n<\/span> a tres tipos de <span class=\"term\" data-term-id=\"22\">benigno<\/span> piel <span class=\"term\" data-term-id=\"1960\">ap\u00e9ndice<\/span> tumor.<\/p>\n<ul>\n<li>Cilindromas: tumores solitarios o m\u00faltiples en el cuero cabelludo<\/li>\n<li>Tricoepiteliomas - color piel <span class=\"term\" data-term-id=\"366\">p\u00e1pulas<\/span> sobre la cara central<\/li>\n<li>Espiradenomas - dolorosos <span class=\"term\" data-term-id=\"367\">n\u00f3dulos<\/span> en cabeza, cuello y tronco<\/li>\n<\/ul>\n<p>El s\u00edndrome de Brooke-Spiegler tambi\u00e9n se asocia con poca frecuencia con <span class=\"term\" data-term-id=\"2459\">salival<\/span> y <span class=\"term\" data-term-id=\"1778\">gl\u00e1ndula par\u00f3tida<\/span> Tumores.<\/p>\n<p>Las formas limitadas de la enfermedad son:<\/p>\n<ul>\n<li>Cilindromatosis familiar: caracterizada por cilindromas solos;  y;<\/li>\n<li>Tricoepiteliomas familiares m\u00faltiples: caracterizados por tricoepiteliomas solos.<\/li>\n<\/ul>\n<p>Los individuos afectados suelen presentarse desde la ni\u00f1ez tard\u00eda hasta la edad adulta temprana con m\u00faltiples p\u00e1pulas y n\u00f3dulos en el cuero cabelludo, la cara y el cuello.  Ellos <span class=\"term\" data-term-id=\"2343\">desarrollar<\/span> n\u00famero creciente de <span class=\"term\" data-term-id=\"2115\">lesiones<\/span> tiempo extraordinario.  Sin embargo, existe una amplia variabilidad de presentaci\u00f3n dentro y entre familias con s\u00edndrome de Brooke-Spiegler.<\/p>\n<p>Aunque los tumores generalmente se consideran inofensivos, hay informes de <span class=\"term\" data-term-id=\"176\">maligno<\/span> transformaci\u00f3n.<\/p>\n<ul>\n<li>El espiradenoma puede transformarse en espiradenocarcinoma.<\/li>\n<li>El cilindroma puede transformarse en cilindrocarcinoma.<\/li>\n<li>El tricoepitelioma puede transformarse en <span class=\"term\" data-term-id=\"831\">basal<\/span> c\u00e9lula <span class=\"term\" data-term-id=\"32\">carcinoma<\/span>.<\/li>\n<\/ul>\n<h2>\u00bfC\u00f3mo se hizo el diagn\u00f3stico?<\/h2>\n<p>Se puede sospechar el s\u00edndrome de Brooke-Spiegler si hay antecedentes familiares de m\u00faltiples tumores benignos de piel.<\/p>\n<p>Los cilindromas, tricoepiteliomas y espiradenomas son dif\u00edciles de distinguir cl\u00ednicamente.  Por lo tanto piel <span class=\"term\" data-term-id=\"24\">biopsia<\/span> es necesario para confirmar el diagn\u00f3stico.<\/p>\n<h2>\u00bfQu\u00e9 tratamiento est\u00e1 disponible para el s\u00edndrome de Brooke-Spiegler?<\/h2>\n<p>El s\u00edndrome de Brooke-Spiegler no es curable.  Las posibles opciones de tratamiento para tumores individuales incluyen:<\/p>\n<ul>\n<li><span class=\"term\" data-term-id=\"848\">Excisi\u00f3n<\/span><\/li>\n<li>Electrocirug\u00eda<\/li>\n<li>Dermoabrasi\u00f3n<\/li>\n<li>Erbio: itrio-aluminio-granate (Er: YAG) <span class=\"term\" data-term-id=\"1617\">l\u00e1ser<\/span><\/li>\n<li>Rejuvenecimiento con l\u00e1ser de di\u00f3xido de carbono<\/li>\n<li><span class=\"term\" data-term-id=\"1145\">Crioterapia<\/span><\/li>\n<li><span class=\"term\" data-term-id=\"1325\">Radioterapia<\/span>.<\/li>\n<\/ul>\n<\/section>\n","protected":false},"excerpt":{"rendered":"<p>\u00bfQu\u00e9 es Brooke-Spiegler? s\u00edndrome? El s\u00edndrome de Brooke-Spiegler (BRSS o BSS) es una rara gen\u00e9tico condici\u00f3n que resulta en una variedad de tumores derivados de la piel ap\u00e9ndices (cabello fol\u00edculo tumores y sudor gl\u00e1ndula tumores). El s\u00edndrome incluye las variantes limitadas, familiar cilindromatosis y tricoepiteliomas familiares m\u00faltiples (MFT1). \u00bfQu\u00e9 causa el s\u00edndrome de Brooke-Spiegler? El [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":0,"parent":0,"template":"","glossary-cat":[],"class_list":["post-4158","glossary","type-glossary","status-publish","hentry"],"yoast_head":"<!-- This site is optimized with the Yoast SEO Premium plugin v26.2 (Yoast SEO v27.1.1) - https:\/\/yoast.com\/product\/yoast-seo-premium-wordpress\/ -->\n<title>S\u00edndrome de Brooke-Spiegler - Dermatly.com - El sitio de tu piel<\/title>\n<meta name=\"robots\" content=\"index, follow, max-snippet:-1, max-image-preview:large, max-video-preview:-1\" \/>\n<link rel=\"canonical\" href=\"https:\/\/dermatly.com\/it\/sindrome-di-brooke-spiegler\/\" \/>\n<meta property=\"og:locale\" content=\"it_IT\" \/>\n<meta property=\"og:type\" content=\"article\" \/>\n<meta property=\"og:title\" content=\"S\u00edndrome de Brooke-Spiegler\" \/>\n<meta property=\"og:description\" content=\"\u00bfQu\u00e9 es Brooke-Spiegler? s\u00edndrome? El s\u00edndrome de Brooke-Spiegler (BRSS o BSS) es una rara gen\u00e9tico condici\u00f3n que resulta en una variedad de tumores derivados de la piel ap\u00e9ndices (cabello fol\u00edculo tumores y sudor gl\u00e1ndula tumores). El s\u00edndrome incluye las variantes limitadas, familiar cilindromatosis y tricoepiteliomas familiares m\u00faltiples (MFT1). \u00bfQu\u00e9 causa el s\u00edndrome de Brooke-Spiegler? 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