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| Research article summary (published 30 Mar 2009): |
Ehlers-Danlos syndrome--vascular type (ecchymotic variant): cutaneous and dermatopathologic features.
Full Abstract
Ehlers-Danlos syndrome - 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-old non-consanguineous male had brown-black plaques with atrophy and frequent ulceration over legs and dorsal feet and tortuous varicose veins around ankles for the past 15 years. Perivenous skin was translucent and hypopigmented. He had multiple and ecchymotic keloids and small atrophic, pityriasis versicolor-like 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 COL3A1 gene.
Author information
Author/s: Sharma, Nand L (NL); Mahajan, Vikram K (VK); Gupta, Neelam (N); Ranjan, Nitin (N); Lath, Anju (A);
Affiliation: Department of Dermatology, Venereology & Leprosy, Indira Gandhi Medical College, Shimla, Himachal Pradesh, India. nandlals(-atsign-)hotmail.com
Journal and publication information
Publication Type: Case Reports; Journal Article
Journal: Journal of cutaneous pathology (J Cutan Pathol), published in Denmark. (Language: eng)
Reference: 2009-Apr; vol 36 (issue 4) : pp 486-92
Dates: Created 2009/03/12; Completed 2009/05/26;
PMID: 19278438, status: MEDLINE (last retrieval date: 5/26/2009, IMS Date: )
Sourced from the National Library of Medicine. Abstract text and other information may be subject to copyright.
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