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Hereditary Hemorrhagic Telangiectasia (HHT) - also called Rendu-Osler-Weber syndrome - is a genetic disorder of the blood vessels. It occurs in approximately 1 in 5,000 people and affects males and females from all racial and ethnic groups.
HHT causes abnormalities of the blood vessels. While most blood vessels in a person with HHT are normal, they have a small number that have a specific type of abnormality. A person with HHT has a tendency to form blood vessels that lack the capillaries between an artery and vein. A blood vessel that is abnormal in this way is called telangiectasia if it involves small blood vessels. If it involves larger blood vessels, we call it arteriovenous malformation (AVM).
The abnormality causes bleeding, typically during and after puberty and usually affects skin and mucous membranes (mouth, nose, and gastrointestinal tract). Nosebleeds (epistaxis) are one of the most common types of this bleeding. In HHT, AVMs may occur in the lungs, liver, and brain.
Antibiotic prophylaxis is recommended before dental and surgical procedures for all patients with pulmonary AVMs to prevent potential life-threatening conditions.
More information on HHT from the HHT Foundation International
Cutis marmorata telangiectatica congenita (CMTC), which literally means "marbeled skin," presents as a distinctive deep purple, net-like vascular staining pattern that is present at birth. This vascular birthmark usually involves the trunk and extremities. Ulceration of the depressed purple areas can occur, as can overgrowth of the affected limb and tissue beneath the skin. Almost all infants with this condition show steady improvement during the first year of life. In time, widening of the veins becomes more prominent and persists into adulthood, along with atrophy and staining of the skin.
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Telangiectasias
