{"id":19740,"date":"2024-06-04T09:40:05","date_gmt":"2024-06-04T08:40:05","guid":{"rendered":"https:\/\/apps.nhslothian.scot\/refhelp\/?page_id=19740"},"modified":"2024-12-30T15:10:54","modified_gmt":"2024-12-30T15:10:54","slug":"paediatric-scleroderma","status":"publish","type":"page","link":"https:\/\/apps.nhslothian.scot\/refhelp\/guidelines\/paediatrics\/paediatric-rheumatology\/paediatric-scleroderma\/","title":{"rendered":"Paediatric Scleroderma"},"content":{"rendered":"\n<h4 class=\"wp-block-heading\"><strong>Localised &amp; Systemic<\/strong><strong><\/strong><\/h4>\n\n\n\n<p><strong>Information<\/strong><\/p>\n\n\n\n<p>Scleroderma is a range of autoimmune conditions that cause hard, thickened areas of skin and sometimes problems with muscles, bones, internal organs and blood vessels.&nbsp; It can be localised or systemic.&nbsp;<\/p>\n\n\n\n<p>It is rare in children, but a high index of clinical suspicion is required as prompt referral and treatment improves outcomes.&nbsp; <strong>Please refer all suspected cases urgently to Paediatric Rheumatology.<\/strong><\/p>\n\n\n\n<p>Most children (93%) will have <strong>Juvenile Localised Scleroderma<\/strong>, which has an incidence rate 3.4 per one million children per year.&nbsp; 20% of children with juvenile localised scleroderma can have extracutaneous features including arthritis, uveitis, autoimmune disease or neurological involvement.<\/p>\n\n\n\n<p>Suggested website to have a look at images to aid diagnosis: www.dermnetnz.org<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li>Localised:<ul><li>Morphoea<\/li><\/ul><ul><li>Linear scleroderma<\/li><\/ul><ul><li>En coup de sabre<\/li><\/ul>\n<ul class=\"wp-block-list\">\n<li>Progressive facial hemi-atrophy or Parry Romberg syndrome &nbsp;&nbsp;&nbsp;&nbsp;<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<p><strong>Systemic sclerosis<\/strong> is extremely rare, with an incidence of 0.27 per 1 million children\/year.&nbsp; Systemic sclerosis is associated with organ involvement and patients require a multisystem workup.<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li>Systemic:<ul><li>Limited cutaneous<\/li><\/ul><ul><li>Diffuse cutaneous<\/li><\/ul><ul><li>Undifferentiated connective tissue disease<\/li><\/ul>\n<ul class=\"wp-block-list\">\n<li>Overlap syndromes<\/li>\n<\/ul>\n<\/li>\n<\/ul>\n\n\n\n<p>Patients in both groups will often require treatment with steroids and systemic immunosuppression.&nbsp; A small number of patients can be managed with topical treatment alone in conjunction with Dermatology.<\/p>\n\n\n\n<p><strong>M.A. &amp; J.H. 04-06-24<\/strong><\/p>\n\n\n\n<div class=\"wp-block-getwid-tabs\" data-active-tab=\"0\"><ul class=\"wp-block-getwid-tabs__nav-links\"><\/ul>\n<div class=\"wp-block-getwid-tabs__nav-link\"><span class=\"wp-block-getwid-tabs__title-wrapper\"><a href=\"#\"><span class=\"wp-block-getwid-tabs__title\">Referral Guidelines<\/span><\/a><\/span><\/div><div class=\"wp-block-getwid-tabs__tab-content-wrapper\"><div class=\"wp-block-getwid-tabs__tab-content\">\n<p><strong>Please refer all suspected cases urgently to Paediatric Rheumatology.<\/strong><\/p>\n<\/div><\/div>\n\n\n\n<div class=\"wp-block-getwid-tabs__nav-link\"><span class=\"wp-block-getwid-tabs__title-wrapper\"><a href=\"#\"><span class=\"wp-block-getwid-tabs__title\">Primary Care Management<\/span><\/a><\/span><\/div><div class=\"wp-block-getwid-tabs__tab-content-wrapper\"><div class=\"wp-block-getwid-tabs__tab-content\">\n<p><\/p>\n<\/div><\/div>\n\n\n\n<div class=\"wp-block-getwid-tabs__nav-link\"><span class=\"wp-block-getwid-tabs__title-wrapper\"><a href=\"#\"><span class=\"wp-block-getwid-tabs__title\">Resources and Links<\/span><\/a><\/span><\/div><div class=\"wp-block-getwid-tabs__tab-content-wrapper\"><div class=\"wp-block-getwid-tabs__tab-content\">\n<p><a href=\"https:\/\/dermnetnz.org\/\" target=\"_blank\" rel=\"noreferrer noopener\">DermNet | Dermatology Resource (dermnetnz.org)<\/a><\/p>\n<\/div><\/div>\n<\/div>\n","protected":false},"excerpt":{"rendered":"<p>Localised &amp; Systemic Information Scleroderma is a range of autoimmune conditions that cause hard, thickened areas of skin and sometimes problems with muscles, bones, internal organs and blood vessels.&nbsp; It can be localised or systemic.&nbsp; It is rare in children, but a high index of clinical suspicion is required as prompt referral and treatment improves<\/p>\n","protected":false},"author":6,"featured_media":0,"parent":18217,"menu_order":0,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"categories":[1107],"class_list":["post-19740","page","type-page","status-publish","hentry","category-https-apps-nhslothian-scot-refhelp-paediatric-scleroderma"],"publishpress_future_workflow_manual_trigger":{"enabledWorkflows":[]},"rttpg_featured_image_url":null,"rttpg_author":{"display_name":"heatherlevy","author_link":"https:\/\/apps.nhslothian.scot\/refhelp\/author\/heatherlevy\/"},"rttpg_comment":0,"rttpg_category":" <a href=\"https:\/\/apps.nhslothian.scot\/refhelp\/category\/paediatric\/https-apps-nhslothian-scot-refhelp-paediatric-rheumatology\/https-apps-nhslothian-scot-refhelp-paediatric-scleroderma\/\" rel=\"tag\">Paediatric Scleroderma<\/a>","rttpg_excerpt":"Localised &amp; Systemic Information Scleroderma is a range of autoimmune conditions that cause hard, thickened areas of skin and sometimes problems with muscles, bones, internal organs and blood vessels.&nbsp; It can be localised or systemic.&nbsp; It is rare in children, but a high index of clinical suspicion is required as prompt referral and treatment improves","_links":{"self":[{"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/pages\/19740","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/pages"}],"about":[{"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/types\/page"}],"author":[{"embeddable":true,"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/users\/6"}],"replies":[{"embeddable":true,"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/comments?post=19740"}],"version-history":[{"count":2,"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/pages\/19740\/revisions"}],"predecessor-version":[{"id":22329,"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/pages\/19740\/revisions\/22329"}],"up":[{"embeddable":true,"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/pages\/18217"}],"wp:attachment":[{"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/media?parent=19740"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/apps.nhslothian.scot\/refhelp\/wp-json\/wp\/v2\/categories?post=19740"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}