When healthy people are in a cold environment, the tiny blood vessels in their skin constrict, or narrow, in an effort to conserve heat. In people with Raynaud's phenomenon (also called Raynaud's disease or Raynaud's syndrome), that natural response to cold is extreme. The tiny blood vessels go into spasm, narrowing and reducing the blood flow to the affected areas. This response, called vasospasm, is seen most often in the fingers and toes, but it also can occur in the ears, cheeks and nose. In some people with the disorder, the constriction also can occur in response to emotional stress or a rapid change of temperature from warm to cool.

The effect of this can be dramatic and frightening, but it is temporary and rarely dangerous. The color of the affected skin is very different from the color of normal skin. The affected area may be numb or tingling ("pins and needles"). Once the affected area is warmed, the blood vessels relax and expand, which allows more blood flow. The skin then turns pink or red as the blood returns, and there may be a feeling of warmth, throbbing or soreness.

Approximately 5% to 10% of people have Raynaud's phenomenon, and women are more likely to develop the disorder than men. No one knows what causes Raynaud's phenomenon. People who do not have any other symptoms or disease are said to have primary Raynaud's. People who have Raynaud's phenomenon as part of another disease or condition are said to have secondary Raynaud's.

Secondary Raynaud's phenomenon commonly is linked to connective-tissue disorders, such as scleroderma and lupus. It also can occur as a result of damage to blood vessels from injury, frostbite or use of jarring machinery, such as jackhammers or chainsaws. Smoking, some heart and migraine medications and diseases that affect circulation (such as atherosclerosis) also can cause secondary Raynaud's or can worsen pre-existing Raynaud's phenomenon.

People with Raynaud's phenomenon see and feel changes in their fingers and toes when exposed to cold. The skin blanches, or turns white, then blue. Fingers and toes can tingle or feel numb. When rewarmed, the skin flushes pink or red, and there can be throbbing or soreness as the blood surges back into the tiny blood vessels.

People with secondary Raynaud's related to a rheumatic disease also can have arthritis, rash, or a thickening or hardening of the skin.

A physician usually diagnoses Raynaud's phenomenon based on the patient's description of symptoms. If other symptoms such as rash also are present, blood tests and other procedures may be ordered to check for other diseases. More testing also is needed if symptoms are not typical, for example, if only one finger or one hand is affected or if there are color changes that seem permanent. These symptoms would be unusual for Raynaud's and could indicate a blood clot in a blood vessel or other problem with circulation. Careful examination of the nail beds (the skin near the fingernail farthest from the fingertip) may show changes in the blood vessels that may suggest an underlying rheumatic condition such as scleroderma.

Although each episode of vasospasm is temporary, Raynaud's phenomenon is considered a chronic (long-lasting) condition.

Although there is no way to prevent a person from developing the condition, the number of episodes of vasospasm can be reduced or even eliminated by avoiding situations that can trigger events. To avoid vasospasm:

• Avoid unnecessary exposure to the cold.
• At home, turn up the thermostat a few degrees.
• In very cold weather, wear a hat and mittens (which protect better than gloves) to conserve as much warmth as possible.
• Bundle up before you head out into the cold, rather than after you are in the cold, to make sure your arms and legs are not shocked by the temperature change.
• Leave your coat, mittens and hat on in the freezer or refrigerator section of the grocery store.
• At home, use pot holders, mittens or a towel to remove items from the refrigerator.
• Use a cup holder when drinking a cold beverage.
• Stop smoking.

Most people with primary Raynaud's phenomenon do not need professional care. Their symptoms can be managed by avoiding triggering events as much as possible. An episode of vasospasm often can be stopped by warming the affected areas by going indoors or soaking fingers or toes in a bowl of warm water. Biofeedback, which trains the body to warm the extremities, can help some patients to control their symptoms. This mind-body method uses a machine to help you learn how to gain voluntary control over body processes that are typically involuntary, such as blood flow.

For more severe cases (more common with secondary causes of Raynaud's phenomenon), medication may be prescribed. Calcium channel blockers, including nifedipine (Procardia, Adalat) and diltiazem (Cardizem, Dilacor), can decrease the severity and number of vasospasm episodes. Other medications that dilate (widen) blood vessels and are usually prescribed for high blood pressure, also can be effective. These medications include nitroglycerin cream or ointment, hydralazine (Apresoline), prazosin (Minipress) or losartan (Cozaar). Recent studies suggest that sildenafil (Viagra) and other medicines approved for erectile dysfunction in men may improve severe symptoms of Raynaud's (such as non-healing ulcers or gangrene) when other treatments fail. In extreme and rare cases, the sympathetic nerves that control blood vessels may be cut in a surgical procedure called a sympathectomy, or medications called prostaglandins may be given intravenously (into a vein) to open up the blood supply to the fingers or toes and prevent the affected tissues from dying. Newer approaches to treating Raynaud's include medications for erectile dysfunction, such as sildenafil (Viagra) and intravenous medications (such as iloprost). However, these medications are generally reserved for severe cases that do not respond to other treatments; additional study is required to establish the role of these drugs in the treatment of Raynaud's.

People with secondary Raynaud's may also need to be treated for their main disease.

Call your health care professional if you have symptoms of Raynaud's phenomenon to learn more about the disease and to make certain that you do not have any other disease. If you have been diagnosed with Raynaud's phenomenon, see your doctor if you have any unusual symptoms such as severe redness, inflammation or open sores. Seek emergency care if you have color changes, numbness or tingling in your arms or legs that does not go away within minutes after the area is warmed.

For most people, primary Raynaud's phenomenon can be annoying and cause discomfort, but it is rarely dangerous. Although it usually is a lifelong condition, it also usually does not get worse. In rare cases in which the vasospasm is severe, the lack of blood flow may mean that the affected areas may be prone to open sores that are slow to heal.

Raynaud's phenomenon, however, may be the first symptom of a rheumatic disease, so the condition should not be ignored. Patients may need to be examined, tested and monitored to diagnose an associated rheumatic disease at its earliest stage.

American College of Rheumatology
1800 Century Place
Suite 250
Atlanta, GA 30345-4300
Phone: 404-633-3777
Fax: 404-633-1870
http://www.rheumatology.org/

Arthritis Foundation
P.O. Box 7669
Atlanta, GA 30357-0669
Phone: 404-872-7100
Toll-Free: 1-800-283-7800
http://www.arthritis.org/

National Heart, Lung, and Blood Institute (NHLBI)
P.O. Box 30105
Bethesda, MD 20824-0105
Phone: 301-592-8573
TTY: 240-629-3255
Fax: 301-592-8563
Email: nhlbiinfo@rover.nhlbi.nih.gov
http://www.nhlbi.nih.gov/

National Institute of Arthritis and Musculoskeletal and Skin Diseases
Information Clearinghouse
National Insitutes of Health
1 AMS Circle
Bethesda, MD 20892-3675
Phone: 301-495-4484
Toll-Free: 1-877-226-4267
Fax: 301-718-6366
TTY: 301-565-2966
Email: niamsinfo@mail.nih.gov
http://www.niams.nih.gov/