Raynaud's phenomenon

What is Raynaud's phenomenon?

Raynaud's phenomenon (RP) is a condition resulting in a particular series of discolorations of the fingers and/or the toes after exposure to changes in temperature (cold or hot) or emotional events. Skin discoloration occurs because an abnormal spasm of the blood vessels causes a diminished blood supply to the local tissues. Initially, the digit(s) involved turn white because of the diminished blood supply. The digit(s) then turn blue because of prolonged lack of oxygen. Finally, the blood vessels reopen, causing a local "flushing" phenomenon, which turns the digit(s) red. This three-phase color sequence (white to blue to red), most often upon exposure to cold temperature, is characteristic of RP.

What causes Raynaud's phenomenon?

The causes of primary and secondary RP are unknown. Both abnormal nerve control of the blood-vessel diameter and nerve sensitivity to cold exposure have been suspected as being contributing factors. The characteristic color changes of the digits are in part related to initial blood-vessel narrowing due to spasm of the tiny muscles in the wall of the vessels, followed by sudden opening (dilation), as described above. The small arteries of the digits can have microscopic thickness of their inner lining, which also leads to abnormal narrowing of the blood

What conditions have been associated with Raynaud's phenomenon?

Raynaud's phenomenon has been seen with a number of conditions, including rheumatic diseases (scleroderma, rheumatoid arthritis, systemic lupus erythematosus), hormone imbalance (hypothyroidism and carcinoid), trauma (frostbite, vibrating tools), medications (propranolol [Inderal], estrogens without additional progesterone, bleomycin [Bleoxane] used in cancer treatment, and ergotamine used for headaches), nicotine, and even rarely with cancers.

A typical Raynaud’s phenomenon attack may go something like this: You are fixing dinner and go into the freezer to get a bag of peas. They are buried in the back and you have to move things around for a moment before you find them. By the time you close the freezer door, your fingers turn white and they feel cold, numb and start to hurt. The fingers then turn blue. After 10 minutes or so they turn red, tingle, throb and get warm again.

So, the symptoms of Raynaud’s phenomenon are:

• Extreme sensitivity to cold

• Body reacts to emotional stress as if it were reacting to cold

• Skin color changes: Fingers and/or toes (and sometimes ears, lips, nose) turn white due to lack of blood flow (called pallor). The blood that’s left in the tissues loses its oxygen and the fingers turn blue (called cyanosis). Finally, the skin will turn red (called rubor) as fresh oxygenated blood returns to the fingers once the vessels open.

• Coldness, pain and numbness: A lack of oxygenated blood in the fingers triggers feelings of coldness, pain and numbness – the sensation that the hands fingers have fallen asleep.

• Warmth, tingling and throbbing: The quick return of blood to the fingers triggers feelings of warmth, tingling and throbbing, like when your hands “wake up” again.

• Skin ulcers: If your Raynaud’s phenomenon is severe and your attacks tend to last a long time, you may get painful, slow-healing sores on the tips of your fingers.

• Gangrene: In rare cases, a long-term lack of oxygen to the tissues can result in gangrene and amputation of the affected digit.

Diagnosis:

Your primary care doctor can usually determine if you have Raynaud’s phenomenon simply by listening to you describe an attack. You may even have an attack while at the doctor’s office. Determining whether the disorder is primary or secondary to an underlying disease may take some time and testing, however.

To determine whether your Raynaud’s is primary or secondary, your doctor may

• Look at the nailfold capillaries. To do this, the doctor may place a drop of oil on your skin at the base of your fingernail; you will then hold your finger under a microscope. If the capillaries are enlarged or deformed, you may have a connective tissue disease.

• Send your blood to the lab. If you have antinuclear antibodies in your blood, you may have a connective tissue disease or other autoimmune disorder. If you have an elevated erythrocyte sedimentation rate, you may have an inflammatory disorder.

• Perform tests of the blood vessels to see if there is blockage of blood flow in the arms or legs. This is often done with ultrasound, or sometimes with x-rays and dye (angiogram).

Criteria for the diagnosis of Raynaud’s phenomenon:

• Primary Raynaud’s Phenomenon
  • Periodic vasospastic attacks of pallor or cyanosis (some doctors add that the attacks should have been present for at least two years)
  • Normal nailfold capillary pattern
  • Negative antinuclear antibody test
  • Normal erythrocyte sedimentation rate
  • Absence of pitting scars or ulcers of the skin, or gangrene (tissue death) in the fingers or toes

• Secondary Raynaud’s Phenomenon
  • Periodic vasospastic attacks of pallor and cyanosis
  • Abnormal nailfold capillary pattern
  • Positive antinuclear antibody test
  • Abnormal erythrocyte sedimentation rate
  • Presence of pitting scars or ulcers of the skin, or gangrene in the fingers or toes

Treatment option:

For most people with Raynaud’s phenomenon, a conservative approach not using medicines is sufficient to control attacks. For people with more severe attacks, medications can be added.

To shorten the length of an attack once it has started, try these tips:

• Warm your hands or feet in warm (not hot) water.
• Swing your arms in large circles to increase circulation.

• Use relaxation techniques, such as deep breathing or meditation.

• Practice biofeedback methods.

Self-help treatments for preventing attacks include:

• Dress warmly: It is important not only to keep your hands and feet warm, but you also must keep your whole body warm.
  • Wear layers of loose-fitting clothing, warm socks, hats, scarves and gloves or mittens in cold weather. Mittens are warmer than gloves because they trap more air and let the fingers warm each other.
  • Keep a sweater or jacket with you at all times, even in the summer. You may need it in cold, air-conditioned buildings.

• Control your body temperature at home.

• Use flannel sheets or layers of blankets. Use an electric blanket to warm the sheets before you get into bed (make sure you follow the user instructions carefully). Consider wearing mittens and socks to bed if your hands and feet get cold when you sleep.
• Keep the rooms you use most often at a comfortable temperature.
• Start running your bath or shower water before you are ready to bathe so you don’t touch cold water. Keep the bathroom door closed so the steam will warm the room.
• Avoid cold items. Use insulated drinking glasses for your cold drinks; wear gloves when reaching in the freezer; use tepid water to rinse vegetables or hand-wash clothing.
• Use chemical warmers. Small heating pouches can be placed in your pockets, mittens or boots when you need to be outside in the cold for a while.

• Protect your skin: Poor blood flow may make your skin dry. It also may cause cuts, cracks or sores to heal more slowly than usual.
  • Use lotion with lanolin every day on your hands and feet to keep your skin from chapping or cracking.
  • Wash with a mild, creamy soap. Clean between your fingers and toes, but don’t soak them.
  • Examine your feet and hands daily to check for ulcers. If you develop one, keep it clean and covered. See your doctor right away.
  • Protect your nails. Use lotion to keep your cuticles soft. Carefully cut hangnails and file your nails in a rounded fashion to the tips of your fingers.

• Quit smoking: Chemicals in cigarette smoke constrict blood vessels – something you definitely don’t need if you have Raynaud’s phenomenon.

• Control stress: Stress and emotional upset can trigger a Raynaud’s attack, so avoiding stressful situations and learning to relax once you’re feeling anxious can lessen the number of attacks you have. Relaxation techniques and biofeedback training can be learned through a stress management program.

Medical treatments to control severe Raynaud’s phenomenon include:

• Calcium-channel blockers and alpha blockers: These drugs (such as nifedipine [Procardia] and doxazosin [Cardura]) relax smooth muscle and dilate small blood vessels. They decrease the frequency and severity of attacks and help skin ulcers heal.

• Nitroglycerine paste: This vasodilator (a drug that dilates blood vessels) can be applied to the fingers to help heal skin ulcers.

• Antibiotic ointment: In some cases needed to prevent ulcers from becoming infected.

• Analgesics: Pain-relieving drugs, sometimes containing narcotics, may be needed to control the pain of skin ulcers.

• Prostaglandin and prostacyclin: Intravenous prostaglandin and prostacyclin may be needed for some people with severely affected digits.

• Arm pump: A counterpulsation arm pump which keeps blood in the arm at higher pressures is available and may be helpful in managing ulcers of the fingers resulting from severe Raynaud’s phenomenon.

• Surgery: In severe cases, a doctor may recommend surgery to treat Raynaud’s phenomenon. Surgical procedures that are used to treat Raynaud’s phenomenon include cutting the nerves that cause narrowing of the blood vessels or performing vascular surgery to widen the blood vessels causing the Raynaud’s phenomenon. These procedures are done mainly for very severe secondary forms of the condition.

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