{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T03:42:48Z","timestamp":1785555768406,"version":"3.56.0"},"reference-count":30,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2015,11,6]],"date-time":"2015-11-06T00:00:00Z","timestamp":1446768000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"REUNI (Program for the Restructuring and Expansion of Federal Universities), Brazil"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Int J Dermatology"],"published-print":{"date-parts":[[2016,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Incontinentia pigmenti (<jats:styled-content style=\"fixed-case\">IP<\/jats:styled-content>) is a rare genodermatosis with early prenatal lethality in affected males. Clinical manifestations are usually more exuberant in sporadic than in familial cases. Cutaneous manifestations occur in all sporadic cases and about 96% of familial cases. As well as the skin, other tissues arising from the neuroectoderm may be affected.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objectives<\/jats:title><jats:p>This study was designed to evaluate dermatologic, dental, neurologic, and ophthalmologic manifestations in patients with <jats:styled-content style=\"fixed-case\">IP<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Findings in <jats:styled-content style=\"fixed-case\">IP<\/jats:styled-content> patients and family members also diagnosed with <jats:styled-content style=\"fixed-case\">IP<\/jats:styled-content> in Porto Alegre, Brazil, during 2003\u20132012, were analyzed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Thirteen children and seven relatives were diagnosed with <jats:styled-content style=\"fixed-case\">IP<\/jats:styled-content>; 38.4% of cases were familial, and 61.5% were sporadic. Mean \u00b1 standard deviation follow\u2010up was 46.08 \u00b1 39.47 months. Frequencies of 100% and 85.7% for dermatologic manifestations, 23.0% and 0% for neurologic manifestations, 62.5% and 71.4% for dental manifestations, and 11.1% and 42.8% for ophthalmologic manifestations were found in affected children and relatives, respectively. Associated diseases include Wilms\u2019 tumor, myasthenia gravis, Still's syndrome, and congenital hypothyroidism.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>These findings reinforce the heterogeneity of dermatologic findings and the numerous extracutaneous manifestations requiring a multidisciplinary approach. The follow\u2010up of patients with <jats:styled-content style=\"fixed-case\">IP<\/jats:styled-content> is important in the detection of serious associated diseases. The relationships between these disorders and <jats:styled-content style=\"fixed-case\">IP<\/jats:styled-content> raise the need for additional longitudinal studies with longterm monitoring of these patients. The management of <jats:styled-content style=\"fixed-case\">IP<\/jats:styled-content> in clinical practice may benefit from early efforts to detect associated diseases.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ijd.13060","type":"journal-article","created":{"date-parts":[[2015,11,8]],"date-time":"2015-11-08T18:54:20Z","timestamp":1447008860000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":23,"title":["Clinical study of 20 patients with incontinentia pigmenti"],"prefix":"10.1111","volume":"55","author":[{"given":"Claudia S.","family":"Poziomczyk","sequence":"first","affiliation":[{"name":"Section of Dermatology Federal University of Health Sciences of Porto Alegre Porto Alegre Brazil"},{"name":"Department of Pathology Postgraduate Program Federal University of Health Sciences of Porto Alegre Porto Alegre Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Renan R.","family":"Bonamigo","sequence":"additional","affiliation":[{"name":"Section of Dermatology Federal University of Health Sciences of Porto Alegre Porto Alegre Brazil"},{"name":"Department of Pathology Postgraduate Program Federal University of Health Sciences of Porto Alegre Porto Alegre Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fernanda D.","family":"Santa Maria","sequence":"additional","affiliation":[{"name":"Department of Pathology Postgraduate Program Federal University of Health Sciences of Porto Alegre Porto Alegre Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paulo R. 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