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OVERVIEW — 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, the vessels that supply blood to the skin constrict or narrow in response to cold temperatures. This reaction, called "vasoconstriction," decreases blood flow to the skin, which helps to minimize heat loss from the warm blood and therefore preserve a normal internal or "core" temperature. In warm temperatures, these same blood vessels dilate or widen, increasing the flow of blood to the skin surface, thus allowing heat to leave the body, and keeping the core body temperature from rising to a dangerous level.
The blood vessels in the skin that react to temperature changes are called "thermoregulatory" vessels. They are primarily controlled by the sympathetic nervous system, which is the same system that reacts when we are stressed or upset emotionally. This explains why both cold and emotional stress can trigger vasoconstriction of these blood vessels, causing cold fingers and toes.
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 — People 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 females, younger age groups (ie, people under 30), and family members of people with RP. Fortunately, most people with primary RP do not have an underlying medical problem, and while their symptoms can be bothersome, they are not likely to be severe or cause long-term damage. People with primary RP are more likely than people with secondary disease to manage with simple non-drug interventions (see 'Secondary disease' below). Primary RP often improves with time, and people learn to avoid or prepare for cold and emotional triggers.
Secondary disease — People with an underlying disease or defined cause of RP are classified as having secondary RP. There are several known causes of secondary RP. The autoimmune rheumatic diseases, such as scleroderma or systemic lupus erythematosus, represent a common cause. 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. (See "Patient education: Systemic lupus erythematosus (Beyond the Basics)".)
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. People with secondary RP are more likely to need medications to manage their symptoms.
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.
A Raynaud 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 an attack. Experiencing a general body chill can 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 certain proteins in the blood vessels, which signal the vessels to narrow (constrict).
RAYNAUD PHENOMENON SYMPTOMS — Most often, a Raynaud attack affects the blood vessels in the fingers. In a typical 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") (picture 1). Usually, an attack of RP begins in a single finger and then spreads to other fingers in both hands. The color changes often affect the whole finger from tip to base. The index, middle, and ring fingers are most commonly involved, while the thumb is often not affected.
An attack can cause discomfort, including a "pins and needles" feeling, aching, numbness, or clumsiness of the affected hand(s). The feeling of true pain occurs more often in secondary RP and is caused by a 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. Rarely, the tongue can be involved. Temporary mottling (a bluish discoloration) of the skin of the arms and legs might also appear. Attacks affecting the toes are also common, although people 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. During the recovery phase, the person may experience mild discomfort.
People with severe secondary Raynaud phenomenon (RP) can sometimes experience a serious decrease in blood flow that does not resolve even after the provoking factor or cold is removed. This is due to underlying damage to the blood vessels or irreversible structural changes such as a blockage in the vessel that obstructs blood flow. Pain and ulceration of the skin (usually on the tips of the fingers and toes) can result. In severe cases, deeper tissue injury can occur that can lead to loss of the tips of the fingers in some people with a secondary vascular disease that causes RP.
RAYNAUD PHENOMENON DIAGNOSIS — Raynaud phenomenon (RP) is diagnosed based on the person's description of a typical attack following cold exposure. By asking questions, a health care provider can usually tell whether RP or another more common condition is causing cold hands or feet. It is most important to have a full evaluation by a health care provider to determine if RP is happening and the underlying cause of the RP. This requires a good history and physical examination and laboratory testing that may include blood tests and, on occasion, special studies of the blood vessels and their function.
RAYNAUD PHENOMENON TREATMENT — There are several simple measures that can reduce the frequency of Raynaud 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 Raynaud phenomenon (RP). (See "Patient education: Quitting smoking (Beyond the Basics)".)
●Avoid medications that cause vasoconstriction (narrowing of the blood vessels) – 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, medication might be recommended. The medications used to treat RP work by opening (vasodilating) the involved thermoregulatory vessels. Medicines called calcium channel blockers are the most commonly used. They are taken daily and can reduce both the frequency and the severity of Raynaud attacks.
Secondary Raynaud phenomenon — More aggressive treatment is sometimes needed for people with secondary RP. Most people need a medicine to reduce the frequency of attacks and prevent injury to the skin on the fingers and toes. The response to medication in secondary RP varies a great deal depending on the underlying cause and severity of any blood vessel damage. Treatment directed at the underlying cause or aggravating factor (eg, stopping a drug if that is what is causing vasoconstriction) is the most important goal.
Several medicines have been studied for people who do not respond to calcium channel blockers. These include nitrates (topical nitroglycerin), phosphodiesterase inhibitors (sildenafil), or prostaglandins (prostacyclin). Botulinum toxin (brand name: Botox) locally injected at the base of the fingers can also be used, but the use of botulinum toxin for RP needs more study.
Hospitalization may be required if an attack of RP does not resolve and if blood flow is seriously restricted and threatens loss of a finger or toe. Treatment in this situation requires additional medications to dilate the blood vessels, improve blood flow, and prevent tissue injury or ulcerations. In some cases, surgery may be used to cut certain nerves at the base of the finger that trigger vasoconstriction or to repair a blocked blood vessel supplying the affected area.
In rare instances, treatment may be ineffective in reversing the vasoconstriction. This can lead to ulceration (breaking down) of the skin. 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 be necessary.
WHERE TO GET MORE INFORMATION — Your health care 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 Raynaud phenomenon
Treatment of Raynaud phenomenon: Initial management
Pathogenesis and pathophysiology of Raynaud phenomenon
Treatment of Raynaud phenomenon: Refractory or progressive ischemia
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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