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The disease results from mutations in LEMD3 (MAN1), which lead to enhanced transforming growth factor\u2010\u03b2 (TGF\u2010\u03b2) signaling and resultant changes in fibroblast function. TGF\u2010\u03b2 alterations have been implicated in a number of fibrotic disorders, and it is therefore not surprising that a range of cutaneous and skeletal abnormalities have been associated with Buschke\u2013Ollendorff syndrome. Herein, we report a novel association between ossifying fibroma and Buschke\u2013Ollendorff syndrome and discuss how these conditions are likely to be mechanistically linked.<\/jats:p>","DOI":"10.1111\/cup.12365","type":"journal-article","created":{"date-parts":[[2014,6,10]],"date-time":"2014-06-10T15:04:55Z","timestamp":1402412695000},"page":"740-744","source":"Crossref","is-referenced-by-count":7,"title":["Ossifying fibroma in Buschke\u2013Ollendorff syndrome"],"prefix":"10.1111","volume":"41","author":[{"given":"Annelise L.","family":"Dawson","sequence":"first","affiliation":[{"name":"Department of Dermatology University of California  San Francisco CA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joshua M.","family":"Schulman","sequence":"additional","affiliation":[{"name":"Department of Dermatology University of California  San Francisco CA USA"},{"name":"Department of Pathology University of California  San Francisco CA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Richard C.","family":"Jordan","sequence":"additional","affiliation":[{"name":"Departments of Orofacial Sciences, Pathology &amp; Radiation Oncology University of California  San Francisco CA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jeffrey P.","family":"North","sequence":"additional","affiliation":[{"name":"Department of Dermatology University of California  San Francisco CA USA"},{"name":"Department of Pathology University of California  San Francisco CA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2014,7,4]]},"reference":[{"key":"e_1_2_4_2_1","first-page":"693","article-title":"Buschke\u2010Ollendorff syndrome in a three\u2010generation family: influence of a novel LEMD3 mutation to tropoelastin expression","volume":"20","author":"Burger B","year":"2010","journal-title":"Eur J Dermatol"},{"key":"e_1_2_4_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2007.03.031"},{"key":"e_1_2_4_4_1","first-page":"257","article-title":"Ein fall von dermatofibrosis lenticularis disseminata und osteopathia condensans disseminata","volume":"86","author":"Buschke A","year":"1928","journal-title":"Derm Wochenschr"},{"key":"e_1_2_4_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2230.1986.tb00419.x"},{"key":"e_1_2_4_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2010.12.020"},{"key":"e_1_2_4_7_1","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1001\/archderm.1982.01650130048020","article-title":"An elastic tissue defect in dermatofibrosis lenticularis disseminata. 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