{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,11]],"date-time":"2026-05-11T11:21:04Z","timestamp":1778498464362,"version":"3.51.4"},"reference-count":23,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2016,10,3]],"date-time":"2016-10-03T00:00:00Z","timestamp":1475452800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Cutan Pathol"],"published-print":{"date-parts":[[2016,12]]},"abstract":"<jats:p>Birt\u2013Hogg\u2013Dub\u00e9 syndrome (<jats:styled-content style=\"fixed-case\">BHDS<\/jats:styled-content>), first described in 1977, is a rare autosomal dominant disorder, linked to germline mutations in the <jats:italic><jats:styled-content style=\"fixed-case\">FLCN<\/jats:styled-content><\/jats:italic> (folliculin) gene. Patients may present with different skin tumors, pulmonary cysts with recurrent spontaneous pneumothorax, and renal cancers, but it has also been estimated that about 25% of carriers older than 20 years do not show skin involvement. So far, besides the triad of skin lesions of the original description (fibrofolliculomas, trichodischomas and acrochordons), a wide range of neoplastic and non\u2010neoplastic skin conditions have been reported, i.e. melanomas, trichoblastoma, neural\u2010 and connective tissue tumors, lipomas, angiolipomas and focal cutaneous mucinosis.<\/jats:p><jats:p>We describe a patient with <jats:styled-content style=\"fixed-case\">BHDS<\/jats:styled-content> developing multiple skin angiomatous lesions with prominent signet\u2010ring features, an association never reported so far. As renal carcinomas represent the most threatening complication in <jats:styled-content style=\"fixed-case\">BHDS<\/jats:styled-content> and the identification of the patients with <jats:styled-content style=\"fixed-case\">BHDS<\/jats:styled-content> is mainly based on the clinical and histopathologic identification of the diagnostic skin lesions, the role of the dermatologist can be crucial in the prevention and early detection of a potentially aggressive renal cancer.<\/jats:p>","DOI":"10.1111\/cup.12811","type":"journal-article","created":{"date-parts":[[2016,9,6]],"date-time":"2016-09-06T07:23:02Z","timestamp":1473146582000},"page":"1197-1202","source":"Crossref","is-referenced-by-count":5,"title":["Multiple angiomatous nodules: a novel skin tumor in Birt\u2013Hogg\u2013Dub\u00e9 syndrome"],"prefix":"10.1111","volume":"43","author":[{"given":"C.","family":"Nikolaidou","sequence":"first","affiliation":[{"name":"Department of Histopathology Hippokration General Hospital  Thessaloniki 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