{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,27]],"date-time":"2026-07-27T22:09:04Z","timestamp":1785190144266,"version":"3.55.0"},"reference-count":24,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2024,2,22]],"date-time":"2024-02-22T00:00:00Z","timestamp":1708560000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J of Cosmetic Dermatology"],"published-print":{"date-parts":[[2024,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Klippel\u2010Trenaunay\u2010Weber syndrome (KTWS) is a rare disease with a wide range of manifestations. KTWS is characterized by a clinical triad of varicosities of the extremities, cutaneous vascular malformations, and hypertrophy of soft tissues and long bones. The diagnosis is made clinically supplemented with magnetic resonance imaging and computed tomography.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aim<\/jats:title><jats:p>Hereby we aim to highlight the significance of the possible life\u2010threatening first\u2010time presentations associated with the GI system in previously undiagnosed KTWS patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Patient<\/jats:title><jats:p>We report the case of a 47\u2010year\u2010old male with KTWS, who presented with various symptoms such as rectorrhagia since childhood, digestive problems and abnormal lateral vascular malformations of the left buttock which extended all the way to the leg, vascular malformations of the left fourth and fifth toes as well as soft tissue swelling of the left foot. There was no evidence of other clinical presentations. The patient was hospitalized with severe rectorrhagia and a hemoglobin level of 3\/9. Physical examination revealed a blood pressure of 85\/55 and pulse rate of 115. Ruptured aneurysm of the superior mesenteric artery was found on angiography and subsequently treated with embolization. Dermatologic evaluation showed pitting edema of the left leg and foot and multiple vascular lesions. Thus a diagnosis of KTWS was established. Pulsed dye laser therapy and compression bandage was performed for the patient. The patient's follow\u2010up was done 3\u2009months after discharge for which the patient was again consulted by a dermatologist and gastroenterologist. Lymphedema of the left leg had improved to a great extend so treatment with compression bandage was continued. Colonoscopy was repeated for the patient to evaluate and control possible active sources of bleeding, due to potential life\u2010threating complications.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>According to previous findings, there have been few case reports of KTWS presenting with gastrointestinal manifestations, fewer of which have covered acute life\u2010threatening bleedings associated with this system.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jocd.16247","type":"journal-article","created":{"date-parts":[[2024,2,23]],"date-time":"2024-02-23T06:15:21Z","timestamp":1708668921000},"page":"2215-2219","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["<scp>KTWS<\/scp> (<scp>Klippel\u2010Trenaunay\u2010Weber<\/scp> syndrome): A systematic presentation of a rare disease"],"prefix":"10.1111","volume":"23","author":[{"given":"Ramina","family":"Mofarrah","sequence":"first","affiliation":[{"name":"Student Research Committee Shiraz University of Medical Sciences  Shiraz Iran"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5145-792X","authenticated-orcid":false,"given":"Ramin","family":"Mofarrah","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Faculty of Medicine Islamic Azad University of Medical Sciences  Sari Iran"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peyman","family":"Gooranorimi","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology, Faculty of Medicine Islamic Azad University of Medical Sciences  Sari Iran"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sadaf","family":"Emadi","sequence":"additional","affiliation":[{"name":"Student Research Committee Islamic Azad University of Medical Sciences  Sari Iran"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sueshianth Ghobadi","family":"Aski","sequence":"additional","affiliation":[{"name":"General Physician Islamic Azad University of Medical Sciences  Sari Iran"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,2,22]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-1809.2008.00458.x"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.wneu.2020.06.012"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.juro.2006.11.099"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-5347(05)65798-9"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jvs.2010.06.166"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1308\/147870808X303182"},{"issue":"2","key":"e_1_2_9_8_1","first-page":"126","article-title":"Artroplast\u00eda total de cadera en el s\u00edndrome de Klippel\u2010Trenaunay. 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