Soft tissue
Benign vascular lesions
Hereditary hemorrhagic telangiectasia

Author: Vijay Shankar, M.D. (see Authors page)

Revised: 22 September 2016, last major update November 2012

Copyright: (c) 2003-2016, PathologyOutlines.com, Inc.

PubMed search: hereditary hemorrhagic telangiectasia [title]

Related topics: Vascular ectasias

Cite this page: Hereditary hemorrhagic telangiectasia. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/softtissuevascularectasiashereditary.html. Accessed November 22nd, 2017.
Definition / general
  • Also known as Osler-Rendu-Weber syndrome
  • Autosomal dominant disorder in which localized capillary dilation causes arterial blood to be shunted directly into postcapillary venules from birth
  • Dilated capillaries and veins are present over skin and mucous membranes of GI, GU, lips, oral cavity, respiratory
  • May bleed into gut, nose, urine (Am Fam Physician 2010;82:785)
  • Patients have mutations in two transforming growth factor-beta binding proteins including endoglin
Case reports
Clinical images
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Fingertips, tongue, stomach

Nasal mucosa

Tongue

Microscopic (histologic) images
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Dilated capillaries lined by flat endothelial cells