— Michelle, Texas What you describe is suggestive of Raynaud’s phenomenon, a condition in which the arteries of the hands and feet react abnormally to cold exposure by narrowing and causing color changes, pain, and tingling. Women are more frequently affected than men. What is unusual about your description is that your daughter’s condition seems to have started in the toes. Raynaud’s usually starts in the fingers and hands and may affect the toes later. The usual presentation of Raynaud’s phenomenon is a mottled bluish or white color of fingers — or even only one finger — accompanied by pain and tingling, after the person’s hands or body is exposed to cold temperatures. This happens because the arteries go into vasospasm, become narrower, and the blood supply to the fingers is reduced. When the hands warm up and blood circulation is restored, there is a dusky red color to the fingers. Eventually the same reaction may be seen in the toes and at times can be triggered by emotional stress. Raynaud’s phenomenon can exist by itself (primary or Raynaud’s disease) or as a part of a rheumatic disease like scleroderma, systemic lupus, or pulmonary hypertension. At times Raynaud’s may precede scleroderma by several years. Raynaud’s-like symptoms can also appear when the thoracic outlet is narrow. The thoracic outlet is the upper opening of the bones of the chest (thorax). The boundaries of the outlet are the first rib, the clavicle (collar bone), and the first vertebra of the thoracic (dorsal) spine. The neurovascular bundle, a very important structure of nerves and blood vessels for the arm and hand, goes through this outlet. The neurovascular bundle includes the brachial plexus, made of the nerve roots that eventually form the nerves of the upper arm and the subclavian artery and vein that provide its blood supply. When the thoracic outlet structure is such that raising or stretching the arms backward causes narrowing of the outlet and pressure on the neurovascular bundle, certain symptoms occur, known as thoracic outlet syndrome. A cervical rib (an extra rib from the cervical spine) can also cause thoracic outlet syndrome. Thoracic outlet syndrome causes Raynaud’s-like symptoms from artery pressure. Brachial plexus pressure causes numbness, tingling, and weakness of arm muscles. The treatment is usually surgical removal of part of the first rib, or the cervical rib. At times, carpal-tunnel syndrome, the compression of the median nerve at the wrist, may cause Raynaud’s-like symptoms. Carpal-tunnel syndrome is treated with splints, steroid injection, and, more definitively, by surgically freeing the median nerve. Exposure to certain chemicals, like polyvinyl chloride (PVC), can cause Raynaud’s. Because Raynaud’s phenomenon may have several different causes, the patient needs a comprehensive evaluation with detailed history and physical examination by a rheumatologist. Laboratory tests may be needed. Examination of the very small (capillary) blood vessels at the nailfold (the skin at the base of the nail) should be repeated every six months, so that any possible transition to a rheumatic disease can be detected early. Treatment will address the Raynaud’s phenomenon and the disease causing it, if one is found (lupus, scleroderma, other). It is best to prevent Raynaud’s phenomenon. A person with this problem should keep their hands, feet, and body warm, avoid immersion of the hands in cold water or handling frozen items, avoid air conditioning at low temperatures (below 72°F or so) and avoid stress. Keep a pair of gloves and a shawl or sweater handy for cool summer evenings, and dress warmly in the winter, with lined boots or warm, thick socks. The following aggravating factors narrow the blood vessels and thus should be avoided:
Smoking: Nicotine is responsible.CaffeineUse of vibrating toolsOver-the-counter drugs for colds containing pseudoephedrine (i.e., Actifed, Chlor-Trimeton, Sudafed)Certain drugs used to treat high blood pressure and heart disease (beta blockers like metoprolol and others)The pill (oral contraceptives), in certain patients
Moderate exercise helps blood circulation in general, and biofeedback can be used to increase blood flow to the hands and feet. If an attack is starting, it is best to go to a warmer area and warm up the hands and toes. One might rub the hands together under warm water (not hot, it will hurt), or place them in the armpits. You may also rub the toes, and put a warm towel or heated gel pack over them. If preventive measures alone fail to eliminate all attacks of Raynaud’s phenomenon, or if it is severe and frequent, further treatment includes medicines that help dilate blood vessels. These are:
Calcium channel blockers reduce the frequency and severity of Raynaud’s in about two-thirds of patients, and can help heal ulcers on fingers and toes. The main drugs of this class are nifedipine and diltiazem, but others are available too.Alpha blockers help dilate blood vessels by suppressing the action of norepinephrine (a hormone from the adrenal glands) that narrows them. Prazosin and doxazosin are such drugs.Vasodilators, as the name implies, are drugs that dilate blood vessels by relaxing their wall. Nitroglycerin cream or gel is primarily used to help heal finger ulcers by applying it on the skin.Sympathetic ganglia block by injection or surgical sympathectomy is an option that has been used, but I am not in favor of this for Raynaud’s, because it may not work.
To sum up, Raynaud’s phenomenon can exist by itself, be part of important systemic illnesses, or may be brought on by a condition that can be treated. Prevention, avoidance of aggravating factors, and a thorough evaluation and follow-up by a rheumatologist are the best guidelines for patient care. Q2. I’m 23 and recently developed a bizarre symptom. When I drink a beverage from a cold can or I get really upset, my fingers turn ghostly white then blue and go numb on me. Then, after about ten or 15 minutes, they start tingling, and after a while they return to normal. A friend of mine saw it happen the other day, and she said that it was a sign that I was coming down with a bad immune disease like MS or something. Is she right? Should I be worried? Should I see a doctor about it? — Anonymous You give an excellent description of what we call Raynaud’s phenomenon, which is the result of abnormal blood vessel reactivity (spasm of small arteries) to cold exposure or emotional upset. Raynaud’s phenomenon can exist on its own (idiopathic) or can be associated with other problems, but not with multiple sclerosis (MS). The major problems it can be associated with are either thoracic outlet syndrome or an immune-system related rheumatic disease, such as systemic scleroderma or systemic lupus erythematosus (SLE). Chances are you do not have any of these illnesses, but it’s important to understand what they are just the same. Thoracic outlet syndrome is an anatomic condition where the opening between the first rib and the neck bones is narrower than normal. Through this opening (the thoracic outlet) go the subclavian artery and vein, which supply blood to the arm and hand, and the brachial plexus, the bundle of nerve roots coming off the spinal cord that form the three nerves of the arm and hand. If certain anatomic variations are present, such as an extra rib (cervical rib) or if the collar bone (clavicle) is too close to the first rib, the blood vessels and the nerves are compressed (pinched) and can cause Raynaud’s phenomenon. There are several body positions that can bring on the symptoms of thoracic outlet syndrome; the raised arms position is one of them. A rheumatologist or vascular surgeon often uses positioning tests to diagnose the condition. If thoracic outlet syndrome is determined to be the problem, corrective surgery is typically done (removal of the extra rib or the first rib, depending on which causes the narrowing). As to the rheumatic causes of Raynaud’s phenomenon, I should clarify that both scleroderma and lupus can exist as localized illnesses and under those circumstances would not be the cause of Raynaud’s. Scleroderma is less common than lupus, but Raynaud’s occurs much more frequently in people with scleroderma (over 90 percent), while only about 18–22 percent of patients with lupus have it. Raynaud’s phenomenon can precede scleroderma by months or years, so it is important to have a rheumatologist perform an initial examination and then follow up. Other organs that scleroderma can affect are the skin, with tightness and thickening of the face, hands, and other areas; the gastrointestinal tract, starting with the esophagus (difficulty swallowing); the lungs (shortness of breath in later stages); heart; joints; and at times, the kidneys. Scleroderma can be insidious and gradual, or rarely, very progressive, so a very thorough physical examination coupled with specific lab tests should be done. There are new treatments available and others being evaluated. With systemic lupus, there is usually mild arthritis, hair loss, rashes (the typical butterfly rash is on the face, but occurs elsewhere too), and pleurisy and/or pericarditis with chest pain (inflammation and fluid around the lungs or heart). The disease can also involve the kidneys and blood vessels, with inflammation (vasculitis), low red blood cells counts (anemia), low white cell and platelet counts (the clotting cells), and autoantibodies in the blood (antibodies against one’s own cells and tissues). SLE can be insidious also, with new systemic involvement appearing later. Once again, a rheumatologist is the physician to see for consultation and treatment, which is typically done with steroids, antimalarial drugs, and, sometimes, certain types of chemotherapy. Treatment of Raynaud’s phenomenon per se includes prevention (dressing warmly, having a sweater available even on summer evenings; wearing warm, lined gloves for winter and cotton gloves in summer). You can also prevent attacks by avoiding difficult emotional situations, as much as possible, and using medications called calcium channel blockers (nifedipine, diltiazem) that relax the blood vessels. If Raynaud’s is not prevented and is left untreated, especially in patients who also have scleroderma, it could worsen and lead to finger ulcers that are very slow to heal. Bottom line: Prevent your Raynaud’s flare-ups and get to a good rheumatologist. Learn more in the Everyday Health Lupus Center.
