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Telangiectasia

Telangiectasias are small enlarged blood vessels near the surface of the skin, usually they measure only a few millimetres. They can develop anywhere on the body but commonly on the face around the nose, cheeks and chin. more...

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These are actually developmental abnormalities but can closely mimic the behavior of benign vascular neoplasms. They may be composed of abnormal aggregations of arterioles, capillaries, or venules.

Features

Telangiectasias can result in nevus flammeus (port-wine stain), which is a flat birthmark on the head or neck that spontaneously regresses. A port-wine stain, if present, will grow proportionately with the child. There is a high association with Sturge-Weber syndrome, a nevus formation in the skin supplied by the trigeminal nerve and associated with glaucoma, meningeal angiomas, and mental retardation. Finally, spider telangiectasias are a radial array of tiny arterioles that commonly occur in pregnant women and in patients with hepatic cirrhosis. In men, they are related to high estrogen levels secondary to liver disease.

Associated conditions

Telangiectasias may occur in a number of diseases, including (ICD-10 codes are provided):

  • (M34.1) CREST syndrome (a variant of scleroderma)
  • (I78.0) hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome)
  • (G11.3) Ataxia telangiectasia

Treatment

Telangiectasias are often treated with laser or IPL therapy.

External Links

  • Information about Hereditary Hemorrhagic Telangiectasia from Children's Hospital, Seattle.

Read more at Wikipedia.org


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Hereditary hemorrhagic telangiectasia
From Gale Encyclopedia of Medicine, 4/6/01 by Dorothy Elinor Stonely

Definition

Hereditary hemorrhagic telangiectasia is an inherited condition characterized by abnormal blood vessels which are delicate and prone to bleeding. Hereditary hemorrhagic telangiectasia is also known as Rendu-Osler-Weber disease.

Description

The term telangiectasia refers to a spot formed, usually on the skin, by a dilated capillary or terminal artery. In hereditary hemorrhagic telangiectasia these spots occur because the blood vessel is fragile and bleeds easily. The bleeding may appear as small, red or reddish-violet spots on the face, lips, inside the mouth and nose or the tips of the fingers and toes. Other small telangiectasias may occur in the digestive tract.

Unlike hemophilia, where bleeding is caused by an ineffective clotting mechanism in the blood, bleeding in hereditary hemorrhagic telangiectasia is caused by fragile blood vessels. However, like hemophilia, bleeding may be extensive and can occur without warning.

Causes & symptoms

Hereditary hemorrhagic telangiectasia, an autosomal dominant inherited disorder, occurs 1 in 50,000 people.

Recurrent nosebleeds are a nearly universal symptom in this condition. Usually the nosebleeds begin in childhood and become worse with age. The skin changes begin at puberty, and the condition becomes progressively worse until about 40 years of age, when it stabilizes.

Diagnosis

The physician will look for red spots on all areas of the skin, but especially on the upper half of the body, and in the mouth and nose and under the tongue.

Treatment

There is no specific treatment for hereditary hemorrhagic telangiectasia. The bleeding resulting from the condition can be stopped by applying compresses or direct pressure to the area. If necessary, a laser can be used to destroy the vessel. In severe cases, the leaking artery can be plugged or covered with a graft from normal tissue.

Prognosis

In most people, recurrent bleeding results in an iron deficiency. It is usually necessary to take iron supplements.

Prevention

Hereditary hemorrhagic telangiectasia is an inherited disorder and cannot be prevented.

Key Terms

Autosomal dominant
A pattern of inheritance in which the dominant gene on any non-sex chromosome carries the defect.
Chromosome
A threadlike structure in the cell which transmits genetic information.

Further Reading

For Your Information

    Organizations

  • The American Medical Association, 515 North State Street, Chicago, IL, 60610, 312/464-5000.
  • Association of Birth Defect Children, 3526 Emerywood Lane, Orlando, FL, 32806,305/859-2821.

Gale Encyclopedia of Medicine. Gale Research, 1999.

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