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Special Feature
March 7, 2011

Picture of the Month—Diagnosis

Arch Pediatr Adolesc Med. 2011;165(3):275-276. doi:10.1001/archpediatrics.2011.2-b

Telangiectasias represent a direct communication between an artery and a vein without an intervening capillary bed. They tend to be red or purple, to be elevated, and to blanch with pressure. The connection between the high-pressure artery and low-pressure vein tends to be fragile and is prone to spontaneous and recurrent bleeding.

Frequent epistaxis that also occurs in other family members is a common symptom of hereditary hemorrhagic telangiectasia (HHT), also called Rendu-Osler-Weber syndrome. This is an autosomal dominant disorder with varying penetrance and expressivity. It has an estimated incidence of 1 in 5000 persons.1Most patients with HHT have a mutation in 1 of 2 genes that are required for normal angiogenesis.2,3