Patient education: Raynaud phenomenon (Beyond the Basics)
- Fredrick M Wigley, MD
Fredrick M Wigley, MD
- Professor of Medicine
- Johns Hopkins University School of Medicine
The Raynaud phenomenon (RP) is a condition in which some of the body’s blood vessels (most commonly those in the fingers and toes) constrict in an exaggerated way in response to cold or emotional stress.
Normally, blood vessels supplying the skin provide nutrition, allow for tissue metabolism, and provide an important way to maintain a normal body temperature. Specialized skin blood vessels constrict or narrow in response to cold temperatures. This reaction, called “vasoconstriction,” decreases blood flow to the skin, which helps to minimize heat loss and preserve a normal internal or “core” temperature. In warm temperatures, these same blood vessels dilate, allowing heat to leave the body. The blood vessels in the skin that react to temperature changes are called thermoregulatory vessels. These specialized blood vessels are controlled by the sympathetic nervous system, the same system that reacts when we are nervous or upset emotionally. This explains why both cold and emotional stress can trigger vasoconstriction of these blood vessels, causing cold fingers and toes. Thus, cold hands and feet occur normally in everybody when we are exposed to cold temperatures.
In people with RP, the mechanisms that control vasoconstriction are thought to be altered or defective. The vessels constrict in an exaggerated way in response to cold and emotional or physical stress, causing what is called Raynaud’s phenomenon or a Raynaud’s attack. There are three phases of the event represented by skin color changes. First, severe vasoconstriction reduces blood flow to the skin of the affected areas, causing the skin surface to feel cold to touch and to have a white color. The pale white color is due to virtually no blood flow to the skin. The skin then typically becomes a purplish-blue color (called acrocyanosis), as a reduced flow of blood through the skin returns. When the vessel fully recovers, it dilates, allowing blood flow to resume; the skin may blush, becoming very pink or red
RAYNAUD PHENOMENON RISK FACTORS
It is estimated that Raynaud phenomenon (RP) affects 3 to 5 percent of the general population.
Primary disease — Patients who have RP without a related disease are said to have primary RP. The underlying reason for blood vessel sensitivity to cold is uncertain. Primary RP may be a family trait, suggesting that one or more genes that regulate skin blood flow may be responsible.
Primary RP accounts for the majority of cases and is more common among women, younger age groups, and family members of patients with RP. Fortunately, most patients with primary RP are not significantly disabled by the condition and respond well to treatment. It often improves with time, and patients learn to avoid cold and emotional triggers.
Secondary disease — Individuals with an underlying disease or defined cause of RP are classified as having secondary RP. There are several known causes of secondary RP. A common cause of secondary RP is the autoimmune rheumatic diseases, such as scleroderma or systemic lupus erythematosus. These diseases cause a secondary injury that alters the blood vessels and, as a consequence, the injury affects how they react to stimuli such as cold and stress. Secondary RP can be more difficult to manage than primary RP because it is linked to an underlying condition that can physically damage the blood vessel. (See "Patient education: Systemic lupus erythematosus (SLE) (Beyond the Basics)".)
RAYNAUD PHENOMENON CAUSES
The normal control of blood vessel responses to cold and other stimuli is complex, involving the central nervous system, peripheral sensory nerves, and molecules released by circulating cells or from the inner lining of the blood vessel itself (called the endothelium). Raynaud phenomenon (RP) occurs when this complex and delicate system of control is disrupted, which alters the normal responses to the environment.
Alpha adrenergic receptors are proteins on the surface of the muscle cells of blood vessels. They help to maintain vascular “tone.” When stimulated, the receptors trigger blood vessels to constrict in response to certain stimuli such as stress or cold. Receptors that are overly sensitive to adrenalin-like chemicals, released from nerves in the skin, could be responsible for the exaggerated responses to cold that people with RP experience.
A RP attack can be triggered by exposure to cold temperature or even by a shift in temperature from warm to cool. As a result, even mildly cold exposures, such as those caused by air conditioning or the cold of the refrigerated food section in a grocery store, can cause a RP attack. Experiencing a general body chill can also trigger an attack, even if the hands and feet are kept warm. Feeling emotional stress and being startled can cause an attack of RP due to the release of nerve transmitter substances; these substances activate the alpha receptors on the muscle of the blood vessel, which signal the blood vessels to narrow.
RAYNAUD PHENOMENON SYMPTOMS
Most often, a Raynaud phenomenon (RP) attack affects the blood vessels in the fingers. In a typical RP attack, the fingers (or toes) become suddenly cold as the blood vessels constrict. The skin color changes markedly and may become pale (called a “white attack”) or a purple or blue color (called a “blue attack”). Usually, an attack of RP begins in a single finger and then spreads to other fingers in both hands. The index, middle, and ring fingers are most commonly involved, while the thumb is often not affected (figure 1).
Patients may experience discomfort, including a “pins and needles” feeling, aching, numbness, or clumsiness of the affected hand(s). The feeling of true pain occurs in secondary RP and is caused by a critical and prolonged loss of blood flow to the tissues.
Blood vessels supplying the skin of the ears, nose, face, knees, and nipples can also be affected, and the skin in these areas may become pale or bluish in color after cold exposure. Mottling (a bluish discoloration) of the skin of the arms and legs might also appear. Attacks affecting the toes are also common, although patients tend to complain of these less frequently.
Symptoms of RP resolve as the provoking factor (cold or stress) is removed. After leaving the cold area and rewarming, the discoloration resolves after 15 to 20 minutes, and, as normal blood flow resumes, the skin “blushes” or becomes pink.
Patients with severe secondary RP can sometimes experience a serious decrease in blood flow that does not resolve even after the provoking factor or cold is removed. Pain and ulceration of the skin (usually on the tips of the fingers and toes) can result. Serious deeper tissue injury can occur that can lead to finger loss in some patients with a secondary vascular disease causing RP.
RAYNAUD PHENOMENON DIAGNOSIS
Raynaud phenomenon (RP) is diagnosed based on the patient's description of a typical attack following cold exposure. By asking questions, a healthcare provider can usually tell whether RP or another more common condition is causing cold hands or feet.
RAYNAUD PHENOMENON TREATMENT
By taking some simple steps, you can reduce the frequency of Raynaud phenomenon (RP) attacks. Medicine might also be used to help control the symptoms.
●Avoid sudden cold exposure – Use strategies to keep the whole body warm and avoid rapidly shifting temperature, cold breezes, and damp cold conditions. These include dressing warmly, wearing layer clothing such as thermal underwear, donning a hat, and using mittens or gloves.
●Help end an attack – Methods include placing the hands under warm water or in a warm place (such as in the armpits) or rotating the arms in a whirling windmill pattern.
●Avoid smoking – Nicotine and other chemicals in cigarettes cause the blood vessels to constrict and can aggravate RP. (See "Patient education: Quitting smoking (Beyond the Basics)".)
●Avoid medications that cause vasoconstriction – Such medications include decongestants containing phenylephrine or pseudoephedrine, other amphetamines, diet pills, some migraine remedies containing ergotamine, herbs containing ephedra, and medications used to treat attention deficit disorder (ADD) such as methylphenidate, dextroamphetamine-amphetamine, and atomoxetine.
●Reduce stress – Reduce stress and use relaxation techniques to reduce anxiety.
Medicine — If the measures above are not sufficient, your doctor or nurse might recommend a medicine. Medicines called calcium channel blockers are the most commonly used and can reduce both the frequency and the severity of RP attacks.
Secondary Raynaud phenomenon — More aggressive treatment is sometimes needed for people with secondary Raynaud phenomenon. Most people need a medicine to reduce the frequency of attacks and prevent injury to the skin on the fingers and toes.
Several medicines have been studied for people who do not respond to calcium channel blockers. These include nitrates (topical nitroglycerin), blood pressure medications (prazosin or angiotensin receptor inhibitors like losartan), phosphodiesterase inhibitors (sildenafil, pentoxifylline, or cilostazol), antidepressant medication (fluoxetine), or prostaglandins (prostacyclin). Botulinum toxin locally injected at the base of the fingers can also be used.
Hospitalization may be required if an attack of RP does not resolve and if blood flow to a finger or toe is seriously restricted. Treatment in this situation requires additional medications to dilate the blood vessels and prevent blood clots. In some cases, surgery may be used to cut the nerves at the base of the finger that trigger vasoconstriction in the affected area.
In rare instances, treatment may be ineffective in reversing the vasoconstriction. Ulceration of the skin may result. If all other treatments have failed and if deep tissue injury has occurred due to lack of blood flow, surgical amputation of the affected finger or toe may become necessary.
WHERE TO GET MORE INFORMATION
Your healthcare provider is the best source of information for questions and concerns related to your medical problem.
This article will be updated as needed on our web site (www.uptodate.com/patients). Related topics for patients, as well as selected articles written for healthcare professionals, are also available. Some of the most relevant are listed below.
Patient level information — UpToDate offers two types of patient education materials.
The Basics — The Basics patient education pieces answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials.
Beyond the Basics — Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are best for patients who want in-depth information and are comfortable with some medical jargon.
Professional level information — Professional level articles are designed to keep doctors and other health professionals up-to-date on the latest medical findings. These articles are thorough, long, and complex, and they contain multiple references to the research on which they are based. Professional level articles are best for people who are comfortable with a lot of medical terminology and who want to read the same materials their doctors are reading.
Clinical manifestations and diagnosis of the Raynaud phenomenon
Initial treatment of the Raynaud phenomenon
Pathogenesis of the Raynaud phenomenon
Treatment of the Raynaud phenomenon resistant to initial therapy
The following organizations also provide reliable health information.
●The National Institutes of Health (NIH)
●The National Institute of Arthritis and Musculoskeletal and Skin Diseases
●The Arthritis Foundation
●The Arthritis Society
●American college of rheumatology
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- Block JA, Sequeira W. Raynaud's phenomenon. Lancet 2001; 357:2042.
- Wigley FM. Clinical practice. Raynaud's Phenomenon. N Engl J Med 2002; 347:1001.
- Flavahan NA, Flavahan S, Mitra S, Chotani MA. The vasculopathy of Raynaud's phenomenon and scleroderma. Rheum Dis Clin North Am 2003; 29:275.
- Boin F, Wigley FM. Understanding, assessing and treating Raynaud's phenomenon. Curr Opin Rheumatol 2005; 17:752.
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- Herrick AL. Contemporary management of Raynaud's phenomenon and digital ischaemic complications. Curr Opin Rheumatol 2011; 23:555.
- Pope JE. Raynaud's phenomenon (primary). BMJ Clin Evid 2011; 2011.
- Raynaud's Phenomenon: A Guide to Pathogenesis and Treatment, Wigley FM, Herrick AL, Flavahan N. (Eds), Springer, 2014.
- Herrick AL. Management of Raynaud's phenomenon and digital ischemia. Curr Rheumatol Rep 2013; 15:303.
- Herrick AL. The pathogenesis, diagnosis and treatment of Raynaud phenomenon. Nat Rev Rheumatol 2012; 8:469.
- Pope JE. Primary raynaud phenomenon. Am Fam Physician 2014; 90:403.
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