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I have a confession of sorts, really more of an admission: I have a disease. That may be putting it a bit dramatically, but seriously, I have an affliction.
No, it's nothing serious, certainly not debilitating, but it does affect my quality of life -- a little. I live with cold fingers! Dreadful? Not really, and as diseases go, this is a very innocuous one. It does not limit me or prevent me from pursuing any desired activities. But it is really a nuisance.
This is a common condition, found in approximately 1 out of 10 people (depending on which study you consult). This particular disease is one many live with, never realizing they have a recognized medical diagnosis. This problem causes some area of your body -- typically the fingers or toes -- to feel numb and cold in response to even mildly cold temperatures or stress.
It's called Raynaud's disease because of Dr. Maurice Raynaud, a French doctor, who, in 1862, first described the phenomenon. It is a vasospastic phenomenon, meaning the nerves controlling certain smaller arteries over-react to certain stimuli. When it occurs, the blood supply to your skin is reduced due to the over-active stimulation of these nerves. Consequently, there a limitation of the blood circulating to the affected area.
It is an acute situation, one that develops over a short period of time, as opposed to one of gradual chronic course. Thus, it is termed an attack. But is it really a disease? Perhaps it is a syndrome, or maybe a phenomenon?
All these terms have been associated with this condition. Although great confusion abounds (especially on the internet), Raynaud's syndrome seems to be the more general term for this problem. If it is not associated with an underlying condition, like an auto-immune disease, it is called either Raynaud's disease or primary Raynaud's. If associated with some other precipitating systemic or regional disorder, it is called Raynaud's syndrome, or secondary Raynaud's. Mystery solved!
Because of the narrowing of the blood vessels, there will be reduced blood flow to the affected part. Thus, the area usually turns white, at least initially. This is predictable, since it is the presence of blood which makes skin pink. Reduced blood flow to the skin causes it to look white. Following this attack, they often turn blue and feel cold and numb. As the body warms, and circulation improves, the affected areas may turn red, throb, tingle or swell. The severity can vary tremendously. When the more serious form of Raynaud’s phenomenon is present, where the vasospasm in the vessels leads to some tissue death, there will be a subsequent increase in blood flow to remove the waste products and clear up cell debris. This increased blood flow means the digits will become a darker red, a response termed reactive hyperemia. There are other vasospastic phenomenon, but Raynaud's is by far the most common. Women are more likely than men to have Raynaud's disease. It appears to be more common in people who live in colder climates and seems to have no racial tendencies.
For most people, Raynaud's disease isn’t disabling, although, as mentioned, it can affect your quality of life. There are other symptoms associated with Raynaud’s disease beyond the aforementioned cold digits and color changes in your skin in response to cold or stress. The sufferer may experience numbness, or a prickly feeling. There can be a stinging pain upon re-warming the afflicted part. Although Raynaud’s most commonly affects your fingers and toes, it can also affect other areas of your body, such as your nose, lips, ears and even nipples. After warming, it can take 15 minutes for normal blood flow to return to the area.
As mentioned, many have Raynaud's because of some other condition or disease. The diseases most often linked with Raynaud's are autoimmune or connective tissue diseases such as Lupus (SLE), scleroderma, Sjogren syndrome or Rheumatoid arthritis. The many occlusive vascular diseases, such as the too-common atherosclerosis (aka "hardening of the arteries"), can also lead to the development of Raynaud's. Other disorders not associated with the autoimmune diseases include frostbite, vibration injury (jack-hammer operators!), and PVC exposure.
How is a person to know if they have this strange and generally benign condition? Certain criteria are considered diagnostic for Raynaud disease including attacks which are triggered by exposure to cold or stress with involvement of the same digits of both hands or feet. With Raynaud's disease, no tissue death will occur. The capillaries, the tiniest of blood vessels, will be normal when examined under a microscope. Laboratory tests for inflammation will be normal, including the tests for autoimmune disorders.
Young women who have had Raynaud phenomenon alone for more than 2 years and have not developed any additional manifestations are unlikely to develop an autoimmune disease. Unfortunately, the same cannot be said for older patients, and also male patients, with Raynaud phenomenon, as these attacks can predate the development of systemic disease by as many as 20 years. In some studies, 46-81 percent of affected patients have secondary Raynaud phenomenon.
Treatment of Raynaud's disease depends on its severity and whether you have other associated health conditions. For primary Raynaud phenomenon, the first line of therapy consists of lifestyle measures. The most obvious of these would be smoking cessation. Another beneficial measure is to avoid the cold, very difficult if one is a resident of the UP. If you can't stay away from frigid temps, protection is highly recommended, although wearing gloves after washing your hands every time is annoying.
If these prove inadequate, the patient may benefit from pharmacologic treatment, although there are other, more holistic methods. I have found some patient benefit greatly from medical magnets. Some preliminary studies say that cold laser treatments reduce the degree of the pathologic response. Patients with secondary Raynaud phenomenon are more likely to require pharmacologic therapy. A variety of drugs are used off-label for treatment; the most commonly used drug is a cardiac medication.
As a resident of the U.P., I definitely enjoy many of the myriad recreational pursuits available to us here. This includes some frigid activities like skiing and fat tire biking. But I'm lucky, in that I have a very mild form of Raynaud's: it's annoying, but not debilitating. To those with more severe primary forms, I can only wish them good luck. And recommend to them some heated gloves!
Editor's note: Dr. Conway McLean is a podiatric physician now practicing foot and ankle medicine in the Upper Peninsula, having assumed the practice of Dr. Ken Tabor. McLean has lectured internationally on surgery and wound care, and is board certified in both, with a sub-specialty in foot orthotic therapy. Dr. McLean welcomes questions, comments and suggestions at drcmclean@penmed.com.