{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,18]],"date-time":"2026-10-18T11:03:04Z","timestamp":1792321384271,"version":"4.4.0"},"reference-count":19,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2008,10,14]],"date-time":"2008-10-14T00:00:00Z","timestamp":1223942400000},"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":[[2009,4]]},"abstract":"<jats:p>Ehlers\u2010Danlos syndrome \u2013 vascular type, the only lethal form, is rarely reported in dermatology literature. It is characterized by translucent, atrophic skin, easy bruising, arterial, intestinal and\/or uterine fragility manifesting as varicose veins, aneurysms and vascular\/visceral\/uterine rupture. As its dermatopathologic features are not well elucidated, diagnosis is often made after a catastrophic complication or at autopsy. This 36 year\u2010old non\u2010consanguineous male had brown\u2010black plaques with atrophy and frequent ulceration over legs and dorsal feet and tortuous varicose veins around ankles for the past 15\u2003years. Perivenous skin was translucent and hypopigmented. He had multiple and ecchymotic keloids and small atrophic, pityriasis versicolor\u2010like lesions over trunk. He did not have hypermobile\/hyperextensible skin and joints and showed no systemic or investigative abnormality. Histopathologic features of atrophic lesion included blood extravasation in atrophic epidermis\/dermis, focal clustering and dilatation of blood vessels, malformed vessel walls, abundant hemosiderin in the dermis and homogenously stained\/whorled patterned collagen especially around blood vessels. Pathology of keloidal lesion showed new collagen and vascular fragility. These histopathologic features appear of diagnostic value especially in patients who have compatible clinical findings but cannot afford confirmation by biochemical testing for abnormal synthesis of type III procollagen or identification of mutations in the <jats:italic>COL3A1<\/jats:italic> gene.<\/jats:p>","DOI":"10.1111\/j.1600-0560.2008.01065.x","type":"journal-article","created":{"date-parts":[[2008,10,15]],"date-time":"2008-10-15T00:48:24Z","timestamp":1224031704000},"page":"486-492","source":"Crossref","is-referenced-by-count":16,"title":["Ehlers\u2010Danlos syndrome \u2013 vascular type (ecchymotic variant): cutaneous and dermatopathologic features"],"prefix":"10.1111","volume":"36","author":[{"given":"Nand L.","family":"Sharma","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vikram K.","family":"Mahajan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Neelam","family":"Gupta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nitin","family":"Ranjan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anju","family":"Lath","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2009,3,2]]},"reference":[{"key":"e_1_2_4_2_2","first-page":"330","article-title":"Oral manifestations of Ehlers\u2010Danlos syndrome","volume":"67","author":"L\u00e9tourneau Y","year":"2001","journal-title":"J Can Dent Assoc"},{"key":"e_1_2_4_3_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00201664"},{"key":"e_1_2_4_4_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa002939"},{"key":"e_1_2_4_5_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1525-1470.2001.018002156.x"},{"key":"e_1_2_4_6_2","doi-asserted-by":"publisher","DOI":"10.1111\/1523-1747.ep12319926"},{"key":"e_1_2_4_7_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.1996.tb01143.x"},{"key":"e_1_2_4_8_2","doi-asserted-by":"publisher","DOI":"10.1086\/324123"},{"key":"e_1_2_4_9_2","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM200003093421001"},{"key":"e_1_2_4_10_2","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-2-32"},{"key":"e_1_2_4_11_2","doi-asserted-by":"publisher","DOI":"10.1002\/9780470750520.ch46"},{"key":"e_1_2_4_12_2","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320290316"},{"key":"e_1_2_4_13_2","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-8628(19980428)77:1<31::AID-AJMG8>3.0.CO;2-O"},{"key":"e_1_2_4_14_2","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.72.4.1314"},{"key":"e_1_2_4_15_2","doi-asserted-by":"publisher","DOI":"10.1016\/0002-8703(92)90833-H"},{"key":"e_1_2_4_16_2","doi-asserted-by":"publisher","DOI":"10.1002\/art.20488"},{"key":"e_1_2_4_17_2","first-page":"160","article-title":"A clinical and cardiovascular survey of Ehlers\u2010Danlos syndrome patients with complete deficiency of tenascin\u2010X","volume":"62","author":"Peeters ACTM","year":"2004","journal-title":"Neth J Med"},{"key":"e_1_2_4_18_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00273196"},{"key":"e_1_2_4_19_2","doi-asserted-by":"publisher","DOI":"10.1001\/archsurg.1986.01400090091016"},{"key":"e_1_2_4_20_2","unstructured":"PepinMG ByersPH.Ehlers\u2010Danlos syndrome vascular type.Gene Reviews. 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