What does HHT look like?

Signs. Nosebleeds are the most common sign of HHT, resulting from small abnormal blood vessels within the inside layer of the nose. Abnormal blood vessels in the skin can appear on the hands, fingertips, face, lips, lining of the mouth, and nose as delicate red or purplish spots that lighten briefly when touched.

How rare is Osler-Weber-Rendu syndrome?

Osler-Weber-Rendu disease, also known as hereditary hemorrhagic telangiectasia, is a rare autosomal dominant condition causing systemic fibrovascular dysplasia. It has an incidence of 1-2/100,000.

Can HHT be fatal?

HHT can be fatal if the AVM is in the patient’s brain, lungs or GI tract (stomach and intestines). AVMs in the GI tract do not cause pain or discomfort.

What is Osler Weber Rendu disease?

Osler-Weber-Rendu Disease. Medically reviewed by University of Illinois-Chicago, College of Medicine on February 11, 2016 — Written by Teresa Bergen. Osler-Weber-Rendu syndrome (OWR) is also known as hereditary hemorrhagic telangiectasia (HHT). It’s a genetic blood vessel disorder that often leads to excessive bleeding.

What are the indications for intervention in Osler-Weber-Rendu syndrome (HHT)?

Indications for intervention in Osler-Weber-Rendu syndrome, or hereditary hemorrhagic telangiectasia (HHT), vary according to site of involvement and presentation. One third of cases of are mild, one third are moderate, and one third are severe. In mild cases of HHT, no treatment is necessary.

What are telangiectasias in Osler-Weber-Rendu syndrome?

Telangiectases in the gastric mucosa of a patient with Osler-Weber-Rendu syndrome. The lesions can be seen most prominently at the 11-o’clock position proximally. The endoscopic evidence of vascular lesions in patients with bleeding confirms the role of GI telangiectasias in the bleeding episodes.

How common is Osler-Weber-Rendu syndrome?

According to the HHT Foundation International, the syndrome affects about one in 5,000 people. However, many people with the disease don’t know they have it, so this number may actually be higher. The name Osler-Weber-Rendu syndrome is named for the doctors who worked on researching this condition in the 1890s.