{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T06:40:05Z","timestamp":1756276805162,"version":"3.44.0"},"reference-count":21,"publisher":"BMJ","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Reports"],"accepted":{"date-parts":[[2018,6,18]]},"abstract":"<jats:p>Phakomatosis pigmentovascularis (PPV) is an uncommon dermatosis characterised by the presence of both pigmentary and vascular abnormalities.<jats:sup>1<\/jats:sup> Its pathogenesis is not elucidated, and the prognosis is mainly determined by the presence of extracutaneous manifestations, such as Klippel-Trenaunay syndrome (KTS), that is defined by the triad of a port-wine stain (PWS), anomalous veins and progressive overgrowth of the affected extremity. Herein, we report a case of an adult patient, who presented with a large PWS, nevus of Ota, ocular melanosis, and limb hypertrophy and varicosities. These findings represented a rare association of PPV type IIb and KTS.<\/jats:p>","DOI":"10.1136\/bcr-2018-225721","type":"journal-article","created":{"date-parts":[[2018,7,13]],"date-time":"2018-07-13T23:27:14Z","timestamp":1531524434000},"page":"bcr-2018-225721","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":1,"title":["Port-wine stain as a clue for two rare coexisting entities"],"prefix":"10.1136","volume":"2018","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6561-5338","authenticated-orcid":false,"given":"Filipa Tavares","family":"Almeida","sequence":"first","affiliation":[{"name":"Department of Dermatovenereology, Hospital de Braga, Braga, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Regina","family":"Caldas","sequence":"additional","affiliation":[{"name":"Department of Dermatovenereology, Hospital de Braga, Braga, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria da Luz","family":"Duarte","sequence":"additional","affiliation":[{"name":"Department of Dermatovenereology, Hospital de Braga, Braga, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Celeste","family":"Brito","sequence":"additional","affiliation":[{"name":"Department of Dermatovenereology, Hospital de Braga, Braga, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2018,7,13]]},"reference":[{"key":"2025082623232308000_2018.jul12_1.bcr-2018-225721.1","doi-asserted-by":"publisher","DOI":"10.1136\/bcr.12.2011.5432"},{"key":"2025082623232308000_2018.jul12_1.bcr-2018-225721.2","doi-asserted-by":"crossref","first-page":"e46","DOI":"10.1016\/j.jaad.2010.05.025","article-title":"Phakomatosis pigmentovascularis type IIb associated with Klippel-Tr\u00e9naunay syndrome and congenital triangular alopecia","volume":"65","author":"Turk","year":"2011","journal-title":"J Am Acad Dermatol"},{"key":"2025082623232308000_2018.jul12_1.bcr-2018-225721.3","doi-asserted-by":"crossref","DOI":"10.1097\/MD.0000000000001025","article-title":"Phakomatosis Pigmentovascularis Associated With Sturge-Weber Syndrome, Ota Nevus, and Congenital Glaucoma","volume":"94","author":"Yang","year":"2015","journal-title":"Medicine"},{"issue":"5 suppl 1","key":"2025082623232308000_2018.jul12_1.bcr-2018-225721.4","doi-asserted-by":"crossref","first-page":"54","DOI":"10.1590\/abd1806-4841.20164516","article-title":"Phacomatosis pigmentovascularis of cesioflammea type","volume":"91","author":"Villarreal","year":"2016","journal-title":"An Bras Dermatol"},{"key":"2025082623232308000_2018.jul12_1.bcr-2018-225721.5","doi-asserted-by":"crossref","first-page":"1293","DOI":"10.1016\/j.jpeds.2012.12.040","article-title":"Blotchy baby: a case of phakomatosis pigmentovascularis","volume":"162","author":"Brittain","year":"2013","journal-title":"J Pediatr"},{"key":"2025082623232308000_2018.jul12_1.bcr-2018-225721.6","doi-asserted-by":"publisher","DOI":"10.1001\/archderm.141.3.385"},{"key":"2025082623232308000_2018.jul12_1.bcr-2018-225721.7","doi-asserted-by":"publisher","DOI":"10.1038\/nature07586"},{"key":"2025082623232308000_2018.jul12_1.bcr-2018-225721.8","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1213507"},{"key":"2025082623232308000_2018.jul12_1.bcr-2018-225721.9","doi-asserted-by":"crossref","first-page":"342","DOI":"10.1046\/j.1365-2133.2003.05118.x","article-title":"Cutis marmorata telangiectatica congenita and extensive mongolian spots: type 5 phacomatosis pigmentovascularis","volume":"148","author":"Torrelo","year":"2003","journal-title":"Br J Dermatol"},{"key":"2025082623232308000_2018.jul12_1.bcr-2018-225721.10","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2007.08.012"},{"key":"2025082623232308000_2018.jul12_1.bcr-2018-225721.11","doi-asserted-by":"crossref","first-page":"e32","DOI":"10.1111\/1346-8138.13505","article-title":"Phakomatosis pigmentovascularis type IIb: A case with Klippel-Tren\u00e1unay syndrome and extensive dermal melanocytosis as nevus of Ota, nevus of Ito and ectopic Mongolian spots","volume":"44","author":"Namiki","year":"2017","journal-title":"J Dermatol"},{"key":"2025082623232308000_2018.jul12_1.bcr-2018-225721.12","doi-asserted-by":"publisher","DOI":"10.1155\/2015\/501658"},{"issue":"Pt 2","key":"2025082623232308000_2018.jul12_1.bcr-2018-225721.13","first-page":"212","article-title":"Recupero SM1, Abdolrahimzadeh S, De Dominicis M, Mollo R. Sturge-Weber syndrome associated with naevus of Ota","volume":"12","year":"1998","journal-title":"Eye"},{"key":"2025082623232308000_2018.jul12_1.bcr-2018-225721.14","doi-asserted-by":"crossref","first-page":"945","DOI":"10.1002\/ajmg.a.31970","article-title":"Phakomatosis pigmentovascularis: Implications for severity with special reference to Mongolian spots associated with Sturge-Weber and Klippel-Trenaunay syndromes","volume":"143A","author":"Hall","year":"2007","journal-title":"Am J Med Genet A"},{"key":"2025082623232308000_2018.jul12_1.bcr-2018-225721.15","doi-asserted-by":"publisher","DOI":"10.1542\/peds.2004-1607"},{"key":"2025082623232308000_2018.jul12_1.bcr-2018-225721.16","doi-asserted-by":"crossref","first-page":"303","DOI":"10.1111\/j.1525-1470.2010.01144.x","article-title":"Two reports of phacomatosis pigmentovascularis type IIb, one in association with Sturge-Weber syndrome and Klippel-Trenaunay syndrome","volume":"27","author":"Finklea","year":"2010","journal-title":"Pediatr Dermatol"},{"key":"2025082623232308000_2018.jul12_1.bcr-2018-225721.17","first-page":"e121","article-title":"[Phakomatosis pigmentovascularis cesioflammea: a case report]","volume":"116","author":"Viada Pel\u00e1ez","year":"2018","journal-title":"Arch Argent Pediatr"},{"key":"2025082623232308000_2018.jul12_1.bcr-2018-225721.18","doi-asserted-by":"crossref","unstructured":"Shields CL , Di Nicola M , Pellegrini M , et al . Choroidal melanoma in phakomatosis pigmentovascularis with klippel-trenaunay syndrome. Retina 2017:1.doi:10.1097\/IAE.0000000000001856","DOI":"10.1097\/IAE.0000000000001856"},{"issue":"6 Suppl 1","key":"2025082623232308000_2018.jul12_1.bcr-2018-225721.19","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1590\/abd1806-4841.20132248","article-title":"Phacomatosis pigmentovascularis type IIa\u2013case report","volume":"88","author":"Segatto","year":"2013","journal-title":"An Bras Dermatol"},{"key":"2025082623232308000_2018.jul12_1.bcr-2018-225721.20","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.2009.09513.x"},{"key":"2025082623232308000_2018.jul12_1.bcr-2018-225721.21","doi-asserted-by":"crossref","first-page":"51","DOI":"10.4103\/0378-6323.125507","article-title":"Sturge-Weber syndrome in association with Klippel-Trenaunay syndrome and phakomatosis pigmentovascularis type IIb","volume":"80","author":"Mandal","year":"2014","journal-title":"Indian J Dermatol Venereol Leprol"}],"container-title":["BMJ Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bcr-2018-225721","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,8,27]],"date-time":"2025-08-27T06:23:38Z","timestamp":1756275818000},"score":1,"resource":{"primary":{"URL":"https:\/\/casereports.bmj.com\/lookup\/doi\/10.1136\/bcr-2018-225721"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,7,13]]},"references-count":21,"alternative-id":["10.1136\/bcr-2018-225721"],"URL":"https:\/\/doi.org\/10.1136\/bcr-2018-225721","relation":{},"ISSN":["1757-790X"],"issn-type":[{"type":"electronic","value":"1757-790X"}],"subject":[],"published":{"date-parts":[[2018,7,13]]}}}