Raynaud’s Phenomenon

September 26, 2011

Raynaud’s phenomenon is a condition in which exposure to cold or emotional stress causes skin discoloration and pain. This occurs most often in the fingers, but can also affect the toes, the tip of the nose, and tips of the ears. Raynaud’s phenomenon can occur itself or may be a symptom of an underlying disease.

During an attack, the skin color changes to white, blue, or red. The color changes are caused by spasm of blood vessels. On exposure to a stress, such as cold or an emotional situation, blood vessels narrow (vasospasm) causing the skin to appear pale. Because blood flow is reduced, the skin then turns blue. When the causative condition (cold or stress) is removed, after a period of about 10-15 minutes, rewarming occurs, turning the skin red. The diagnosis of Raynaud’s phenomenon is based on a description or observation of these color changes in the setting of a typical exposure.

Approximately 4-15% of the population describe symptoms of Raynaud’s. Most of these people have no other symptoms or signs of an underlying disease, toxin exposure, or occupational risk. This is defined as primary Raynaud’s phenomenon. Nearly 80% of patients diagnosed with primary Raynaud’s are female. Attacks generally begin between the ages of 15 and 25 in otherwise healthy individuals. Symptoms are mild and no damage to blood vessels or destructive skin damage occurs. Treatment includes using protective clothing and avoiding excessive exposure to conditions that cause symptoms. Medications that cause narrowing of blood vessels should be discontinued or changed if medically advisable. Any tobacco use should be avoided.

Attacks that begin at a later age are usually more severe and more likely to result from an underlying disease. Systemic sclerosis (also known as scleroderma), a rheumatological disease, is the most common underlying disease. Secondary Raynaud’s can also be caused by other rheumatological diseases (lupus, vasculitis), drugs and toxins (amphetamines), structural diseases of the blood vessels (atherosclerosis), occupational disorders (hand-arm vibration syndrome), and certain blood conditions (cryoglobulinemia).

In systemic sclerosis, nearly 90% of the patients also have Raynaud’s. In these people, Raynaud’s is more severe and can involve skin ulcerations on the fingers or toes. In the worst case, gangrene can occur and surgical amputation is necessary. This kind of Raynaud’s is caused by more than the simple vasospasm of primary Raynaud’s. Changes in the normal lining of the blood vessels lead to scarring, clot formation, and chronic damage. Because the vessels are damaged to start with, vasospasm can lead to blockage of the blood vessels. One distinguishing feature of secondary Raynaud’s that occurs with systemic sclerosis is the abnormal appearance of the small blood vessels of the finger nailbeds when viewed on close inspection.

Treatment for both primary and secondary Raynaud’s includes topical nitrate creams and a class of medications known as calcium channel blockers. Traditionally, these medications are used to treat heart disease and high blood pressure. Here, their role is to decrease vasospasm and stabilize blood vessel membranes. Unfortunately, in cases where gangrene develops, amputation may be the only treatment option.

See Also: Autoimmune disorders, Scleroderma

Suggested Reading

  • Klippel, J. H. (Ed.) (2001). Primer on the rheumatic diseases (12th ed.). Atlanta, GA: Arthritis Foundation.
  • Ruddy, S., Harris, E. D., & Sledge, C. B. (Eds.) (2001). Kelley’s textbook of rheumatology (6th ed.). Philadelphia, PA: W. B. Saunders.

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