Raynaud Phenomenon

 

 

Symptoms & Causes

In-Depth

 

Named for the French medical student who first described it in 1862, Raynaud’s phenomenon is a rare condition in which blood vessels react in an exaggerated way to cold or emotional stress. You may also see the terms “Raynaud’s disease” and “Raynaud’s syndrome,” but they’re misleading. Raynaud’s isn’t a disease: It’s a variant of the body’s natural way of keeping its temperature under control.

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When we’re hot, we get flushed as small blood vessels under the skin widen (dilate) to bring blood close to the surface and let its warmth escape. But when we’re cold, those blood vessels narrow (constrict) to preserve warmth inside for our brain, heart and other vital organs.

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In Raynaud’s, blood vessels going to certain parts of the body—often the fingers—overreact to cold or even a brief drop in temperature, like walking into an air-conditioned room. The vessels clamp down in what’s called a vasospastic attack, blocking the blood flow and causing the skin in the affected area to:

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• turn blue (called cyanosis), as the blood’s oxygen level drops

• turn white (called pallor), as the blood flow stops

• turn red (called rubor), as the vessels reopen and flood the tissues with blood

 

Not everyone with Raynaud’s has all three colour changes, or in the same order. Which body parts are affected and how long the attacks last also varies from person to person. But all patients do fall into one of two categories of Raynaud’s.

• primary Raynaud’s: If your child has primary Raynaud’s, his blood vessels are healthy. They overreact simply because they’re more sensitive, or “twitchy,” than most people’s.

• secondary Raynaud’s: If your child has this rarer and more serious form of Raynaud’s, his blood vessels overreact because they have been damaged by an underlying disease or condition.

 

Who is at risk?

 

According to the Arthritis Foundation, 4 to 15 percent of the people in the United States have Raynaud’s phenomenon. In that group, more than 80 percent have primary Raynaud’s (largely benign), while the rest have secondary Raynaud’s (sometimes serious). And just as the severity of the two types of Raynaud’s is different, so are their risk factors.

Risk factors for Primary Raynaud’s

• gender: About 80 percent of those with primary Raynaud’s are female.

• body type: Raynaud’s is most often seen in slender girls and women.

• age: Primary Raynaud’s usually develops between the ages of 12 and 30.

• family history: About 25 percent of those with primary Raynaud’s have a family history of the condition.

• other factors: Certain foods and medications can exaggerate vasospasm, including caffeine and drugs often used for attention-deficit hyperactivity disorder (ADHD), such as Ritalin.

Risk factors for Secondary Raynaud’s

• age: Secondary Raynaud’s is more likely to occur in people older than 30.

• disease: Secondary Raynaud's is often seen in connection with scleroderma, lupus and other illnesses that damage blood vessels or the nerves that control them.

• other factors: Trauma to the hands or feet, like frostbite

 

When to see a doctor

If your child or you seem extremely sensitive to cold or emotional stress and you think he may have Raynaud's, it’s generally okay to decide to wait until his next regular check-up. Raynaud’s isn’t a disease, and for the vast majority of kids, the diagnosis just means they’ll have to take extra care to stay warm.

But you should make an appointment to bring your child in sooner if his symptoms are accompanied by warning signs of a more serious illness. These include:

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• skin sores

• substantial pain

• marked asymmetry (the fingers of one hand change color but not the other)

• rashes or other changes in the skin

• fever

• chest pain

• bad heartburn

• weakness

 

Complications

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Most people with Raynaud’s phenomenon have no complications. While they may not outgrow their condition, it likely won’t get any worse and they’ll live a normal life.

For the rare few with secondary Raynaud’s, there is a risk of tissue damage if their condition is severe and their attacks tend to last a long time. Blood flow to the affected area may become permanently diminished, causing ulcers or gangrene—ailments that can be very difficult to treat.

 

Prevention

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you may worry whether there was anything you should’ve done to head off your child’s condition. Yet primary Raynaud’s —which, by far, —can’t be prevented. Nor can secondary Raynaud’s, if it is caused by an underlying disease.

But by avoiding the cold, reduce stress and make some simple lifestyle changes, you can help cut down on the frequency and severity of his Raynaud’s attacks. 

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Causes

 

For children with primary Raynaud’s, we don’t yet know what causes their blood vessels to react in such a vigorous way to cold and emotional stress.

However, there are concrete causes for secondary Raynaud’s, and in children it’s most often an underlying autoimmune disease. Here at Children’s Hospital Boston, the illnesses we tend to see with secondary Raynaud’s are:

• scleroderma, which leads to the hardening and scarring of the skin and other body tissues (about 90 percent of those with scleroderma also have Raynaud’s)

• systemic lupus erythematosus (SLE, or simply lupus), which can cause inflammation and damage in many parts of the body, especially the heart, lungs, kidneys and brain (about a third of those with lupus also have Raynaud’s)

• mixed connective tissue disease, an “overlap” disease that has features of scleroderma, lupus, polymyositis and sometimes rheumatoid arthritis

• vasculitis, which causes inflammation of the blood vessels

 

Diseases like arteriosclerosis and hypertension can also damage the blood vessels and cause secondary Raynaud’s, though almost always in adults. Other potential causes of secondary Raynaud’s include:

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• medications: Certain kinds of drugs have been linked to Raynaud’s, such as beta blockers (used to treat high blood pressure), migraine prescriptions with ergotamine, medications with estrogen, drugs containing caffeine (such as Excedrin), medications used for ADHD (like Ritalin) and some over-the-counter decongestants.

• injury to the hands and feet: Frostbite is a notable culprit in this category, but things like repetitive trauma—seen mainly in adults who work with vibrating tools, like drills—can also trigger Raynaud’s.

 

Signs and symptoms

 

Raynaud’s phenomenon isn’t something you can spot by simply looking at your child. Symptoms occur only when the blood vessels are actually in vasospasm—clamping down—in response to cold or emotional stress. Here are the telltales of a Raynaud’s attack:

• changes in skin colour occur as the blood vessels seal off and cause the affected areas (usually the fingers and/or toes) to turn blue or white, or first one and then the other. The skin may then become red and puffy as the vessels reopen. The discoloration can be splotchy or solid, and usually affects both hands and/or both feet at the same time.
   

• coldness, numbness and/or clumsiness happens in the blue or white phase, as if the affected areas have “gone to sleep.”
   

• warmth, tingling and/or throbbing happens in the red, or “rewarming,” phase. This can feel like the pins-and-needles sensation of “waking up” a limb that’s fallen asleep. It can be uncomfortable, and occasionally painful.

It usually takes about 15 to 20 minutes for these symptoms to resolve themselves after whatever triggered the attack is gone (for example, after you’ve moved your child to a warmer area or helped him calm down).

In secondary Raynaud’s, the attacks can last longer and may have more severe symptoms, such as pain in the affected area; lingering weakness or numbness; and skin ulcers or dead tissue (gangrene).

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Questions to ask your doctor

 

You and your family are key players in your child’s medical care. It’s important that you share your observations and ideas with your child’s health care provider and that you understand your provider’s recommendations.

If your child has Raynaud’s phenomenon and you’ve set up an appointment, you probably already have some ideas and questions on your mind. But at the appointment, it can be easy to forget the questions you wanted to ask. It’s often helpful to jot them down ahead of time so that you can leave the appointment feeling that you have the information you need.

If your child is old enough, you may want to suggest that he write down what he wants to ask his health care provider, too.

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Here are some questions you may want to ask:

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• Is this primary or secondary Raynaud’s?

• What is the cause of my child’s Raynaud’s?

• What further tests does my child need?

• When might medications be required?

• What are the side effects of those medications?

• What is the outlook for my child?

• What can I do to help minimize the impact of my child’s Raynaud’s?

 

Helpful medical terms

 

Cyanosis: A bluish color of the skin caused by insufficient oxygen in the blood; one of three color changes associated with Raynaud’s phenomenon.

Digit: A finger or toe. The small blood vessels that feed the fingers and toes are called digital arteries.

Extremities: The limbs of the body, most commonly referring to the hands and feet.

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Ischemia: A lack of blood flow in a body part.

Necrosis: The death of body tissue. Gangrene is a kind of necrosis.

Pallor: A whitening of the skin caused by lack of blood flow; one of three color changes associated with Raynaud’s.

Rubor: A reddish color of the skin caused by increased blood flow; one of three color changes associated with Raynaud’s.

Triphasic: Having three parts. If the skin changes to blue, white and then red during a Raynaud’s attack, it’s called a triphasic reaction. If only two colors occur, it’s called a biphasic reaction.

Vasoconstriction: A narrowing of blood vessels caused by the tightening of muscles in their walls.

Vasodilation: A widening of blood vessels caused by the relaxation of muscles in their walls.

Vasospasm: Sudden, temporary vasoconstriction—literally, a spasm in the blood vessels.

 

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