{"id":352405,"date":"2023-02-09T08:00:00","date_gmt":"2023-02-09T07:00:00","guid":{"rendered":"https:\/\/medizinonline.com\/melanocytose-dermique-atypique\/"},"modified":"2023-04-21T09:41:56","modified_gmt":"2023-04-21T07:41:56","slug":"melanocytose-dermique-atypique","status":"publish","type":"post","link":"https:\/\/medizinonline.com\/fr\/melanocytose-dermique-atypique\/","title":{"rendered":"M\u00e9lanocytose dermique atypique"},"content":{"rendered":"\n<p><strong>Chez un patient p\u00e9diatrique, une m\u00e9lanocytose dermique atypique a conduit au diagnostic de mucopolysaccharidose de type II, \u00e9galement connue sous le nom de &#8220;maladie de Hunter&#8221;.<\/strong><\/p>\n\n<!--more-->\n\n<p><em>(red)<\/em>  La plupart du temps, la m\u00e9lanocytose dermique atypique est une affection isol\u00e9e et b\u00e9nigne, mais elle peut aussi cacher une maladie m\u00e9tabolique ou un syndrome rare.<\/p>\n\n<p>Un gar\u00e7on de 16 mois a \u00e9t\u00e9 adress\u00e9 au service de dermatologie de l&#8217;h\u00f4pital pour enfants de Zurich pour l&#8217;\u00e9valuation d&#8217;une m\u00e9lanocytose dermique extensive et persistante. Les ant\u00e9c\u00e9dents \u00e9taient sans particularit\u00e9, \u00e0 l&#8217;exception d&#8217;infections respiratoires r\u00e9currentes et d&#8217;une respiration sifflante occasionnelle. Les \u00e9tapes de d\u00e9veloppement n&#8217;ont \u00e9t\u00e9 que l\u00e9g\u00e8rement retard\u00e9es. L&#8217;inspection a r\u00e9v\u00e9l\u00e9 des zones d&#8217;hyperpigmentation bleu-gris au niveau des \u00e9paules, du dos, des fesses et des extr\u00e9mit\u00e9s. Le visage et l&#8217;arri\u00e8re de la t\u00eate \u00e9taient l\u00e9g\u00e8rement aplatis, l&#8217;abdomen l\u00e9g\u00e8rement dilat\u00e9 avec un foie palpable \u00e0 environ trois centim\u00e8tres sous le thorax.<\/p>\n\n<p>Pour d\u00e9tecter une \u00e9ventuelle maladie de stockage lysosomale, les glycosaminoglycanes et les oligosaccharides ont \u00e9t\u00e9 mesur\u00e9s dans l&#8217;urine et le sang. Les taux de glycosaminoglycanes (h\u00e9parane et dermatane sulfate) \u00e9taient \u00e9lev\u00e9s et l&#8217;activit\u00e9 enzymatique de l&#8217;iduronate-2-sulfatase dans les leucocytes \u00e9tait absente, ce qui a conduit au diagnostic de mucopolysaccharidose de type II.<\/p>\n\n<p><em>Source : Alvarez E, et al. : A diagnosis out of the blue &#8211; Atypical dermal melanocytosis associated with mucopolysaccharidosis type II (Hunter&#8217;s disease), Poster 32, Abstractband, SGDV Jahresversammlung, 9-11.11.2022.<\/em><\/p>\n\n<p><\/p>\n\n<p><em>DERMATOLOGIE PRATIQUE 2023 ; 33(1) : 37<\/em><\/p>\n","protected":false},"excerpt":{"rendered":"<p>Chez un patient p\u00e9diatrique, une m\u00e9lanocytose dermique atypique a conduit au diagnostic de mucopolysaccharidose de type II, \u00e9galement connue sous le nom de &#8220;maladie de Hunter&#8221;.<\/p>\n","protected":false},"author":7,"featured_media":349206,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_acf_changed":false,"pmpro_default_level":"","cat_1_feature_home_top":false,"cat_2_editor_pick":false,"csco_eyebrow_text":"\u00c9tude de cas en clinique","footnotes":""},"category":[11541,11362,11531,11417,11315,11458,11535,11549],"tags":[27248,22715,66062,66051,66059,66047,66055,36680],"powerkit_post_featured":[],"class_list":["post-352405","post","type-post","status-publish","format-standard","has-post-thumbnail","category-cases-fr","category-dermatologie-et-venerologie","category-formation-continue","category-genetique","category-medecine-interne-generale","category-pediatrie","category-rapports-de-congres","category-rx-fr","tag-clinique","tag-etude-de-cas","tag-hopital-pour-enfants","tag-melanocytose","tag-melanocytose-dermique-atypique","tag-morbus-hunter-fr","tag-mucopolysaccharidose-de-type-ii","tag-sgdv-fr","pmpro-has-access"],"acf":[],"publishpress_future_action":{"enabled":false,"date":"2026-04-27 18:58:08","action":"change-status","newStatus":"draft","terms":[],"taxonomy":"category","extraData":[]},"publishpress_future_workflow_manual_trigger":{"enabledWorkflows":[]},"wpml_current_locale":"fr_FR","wpml_translations":{"it_IT":{"locale":"it_IT","id":352348,"slug":"melanocitosi-cutanea-atipica","post_title":"Melanocitosi cutanea atipica","href":"https:\/\/medizinonline.com\/it\/melanocitosi-cutanea-atipica\/"},"pt_PT":{"locale":"pt_PT","id":352364,"slug":"melanocitose-dermica-atipica","post_title":"Melanocitose d\u00e9rmica at\u00edpica","href":"https:\/\/medizinonline.com\/pt-pt\/melanocitose-dermica-atipica\/"},"es_ES":{"locale":"es_ES","id":352380,"slug":"melanocitosis-dermica-atipica","post_title":"Melanocitosis d\u00e9rmica at\u00edpica","href":"https:\/\/medizinonline.com\/es\/melanocitosis-dermica-atipica\/"}},"_links":{"self":[{"href":"https:\/\/medizinonline.com\/fr\/wp-json\/wp\/v2\/posts\/352405","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/medizinonline.com\/fr\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/medizinonline.com\/fr\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/medizinonline.com\/fr\/wp-json\/wp\/v2\/users\/7"}],"replies":[{"embeddable":true,"href":"https:\/\/medizinonline.com\/fr\/wp-json\/wp\/v2\/comments?post=352405"}],"version-history":[{"count":1,"href":"https:\/\/medizinonline.com\/fr\/wp-json\/wp\/v2\/posts\/352405\/revisions"}],"predecessor-version":[{"id":352410,"href":"https:\/\/medizinonline.com\/fr\/wp-json\/wp\/v2\/posts\/352405\/revisions\/352410"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/medizinonline.com\/fr\/wp-json\/wp\/v2\/media\/349206"}],"wp:attachment":[{"href":"https:\/\/medizinonline.com\/fr\/wp-json\/wp\/v2\/media?parent=352405"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/medizinonline.com\/fr\/wp-json\/wp\/v2\/category?post=352405"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/medizinonline.com\/fr\/wp-json\/wp\/v2\/tags?post=352405"},{"taxonomy":"powerkit_post_featured","embeddable":true,"href":"https:\/\/medizinonline.com\/fr\/wp-json\/wp\/v2\/powerkit_post_featured?post=352405"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}