{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,10]],"date-time":"2026-03-10T11:44:43Z","timestamp":1773143083855,"version":"3.50.1"},"reference-count":15,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2014,9,18]],"date-time":"2014-09-18T00:00:00Z","timestamp":1410998400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Dermatology"],"published-print":{"date-parts":[[2014,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Ataxia\u2010telangiectasia (<jats:styled-content style=\"fixed-case\">AT<\/jats:styled-content>) is a rare autosomal recessive disorder characterized by faulty <jats:styled-content style=\"fixed-case\">DNA<\/jats:styled-content> damage repair. The disease affects multiple systems and is noted to be particularly difficult to diagnose in children because of the wide spectrum of clinical presentations. We present an unusual case of a child in whom the primary cutaneous manifestation of <jats:styled-content style=\"fixed-case\">AT<\/jats:styled-content> was noninfectious cutaneous caseating granulomas. A 3\u2010year\u2010old girl presented to the emergency department with ataxia, poor growth, and multiple ulcerated plaques on both upper extremities that had been present for 2\u00a0years. She had two prolonged hospitalizations and underwent extensive examination to identify an etiology for the skin lesions. She was diagnosed with <jats:styled-content style=\"fixed-case\">AT<\/jats:styled-content> after immunology examinaton and genetic testing. Outpatient intravenous immunoglobulin (<jats:styled-content style=\"fixed-case\">IVIG<\/jats:styled-content>) therapy was initiated and she was prescribed twice\u2010daily mometasone 0.01% ointment under occlusion. After 6\u00a0weeks on this regimen her lesions had completely healed. Twenty\u2010two cases of <jats:styled-content style=\"fixed-case\">AT<\/jats:styled-content> have been reported in which patients presented with cutaneous granulomas. This report demonstrates the first reported case in which the granulomatous skin lesions of <jats:styled-content style=\"fixed-case\">AT<\/jats:styled-content> healed after aggressive application of topical steroids with concurrent <jats:styled-content style=\"fixed-case\">IVIG<\/jats:styled-content> therapy, without oral steroids. A brief review of cutaneous granulomas in the setting of immunodeficiency is also presented.<\/jats:p>","DOI":"10.1111\/pde.12411","type":"journal-article","created":{"date-parts":[[2014,9,19]],"date-time":"2014-09-19T03:25:06Z","timestamp":1411097106000},"page":"703-707","source":"Crossref","is-referenced-by-count":16,"title":["Healing of Granulomatous Skin Changes in Ataxia\u2010Telangiectasia After Treatment with Intravenous Immunoglobulin and Topical Mometasone 0.1% Ointment"],"prefix":"10.1111","volume":"31","author":[{"given":"Emily D.","family":"Privette","sequence":"first","affiliation":[{"name":"Divisions of Dermatology Children's Hospital of Philadelphia Philadelphia Pennsylvania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gita","family":"Ram","sequence":"additional","affiliation":[{"name":"Allergy and Immunology Children's Hospital of Philadelphia Philadelphia Pennsylvania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"James R.","family":"Treat","sequence":"additional","affiliation":[{"name":"Divisions of Dermatology Children's Hospital of Philadelphia Philadelphia Pennsylvania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Albert C.","family":"Yan","sequence":"additional","affiliation":[{"name":"Divisions of Dermatology Children's Hospital of Philadelphia Philadelphia Pennsylvania"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jennifer R.","family":"Heimall","sequence":"additional","affiliation":[{"name":"Allergy and Immunology Children's Hospital of Philadelphia Philadelphia Pennsylvania"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2014,9,18]]},"reference":[{"key":"e_1_2_3_2_1","doi-asserted-by":"publisher","DOI":"10.2165\/11530080-000000000-00000"},{"key":"e_1_2_3_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1525-1470.2011.01409.x"},{"key":"e_1_2_3_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12016-013-8377-8"},{"key":"e_1_2_3_5_1","doi-asserted-by":"publisher","DOI":"10.1159\/000330335"},{"key":"e_1_2_3_6_1","volume-title":"Weedon's skin pathology","author":"Weedon D","year":"2002"},{"key":"e_1_2_3_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0190-9622(84)80089-4"},{"key":"e_1_2_3_8_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-0032-1313915"},{"key":"e_1_2_3_9_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddi333"},{"key":"e_1_2_3_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.2005.06619.x"},{"key":"e_1_2_3_11_1","first-page":"347","volume-title":"Hereditary neuropathies and spinocerebellar atrophies. 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