Acrocyanosis is a vasospastic disorder affecting the arteries supplying blood to the skin of the hands and feet. Vasospasm refers to the arteries going into spasm and blocking the flow of blood. These small arteries carry oxygen and nutrients through the blood to the skin of the hands and feet. When the blood cannot flow through, the skin will lack the necessary oxygen required, and turn a dark blue to purple color. This characteristic color is called cyanosis, hence the name acrocyanosis. It is not a common condition. It is seen more frequently in woman than in men.
Unlike the vasospasm seen in Raynaud’s disease which may last several minutes to several hours, the vasospasm in acrocyanosis is more persistent. In addition, the vasospasm in Raynaud’s disease affects the small arteries supplying blood to the fingers and toes. In acrocyanosis the vasospasm affects the arteries supplying blood flow to the skin of the hands and/or feet. Therefore the skin damage and ulcerations seen in Raynaud’s disease are not present in acrocyanosis. Lastly, Raynaud’s disease goes through a typical triphasic or biphasic color change. In contrast, acrocyanosis maintains its characteristic blue skin coloration.
Typical symptoms and signs of acrocyanosis of the hands or feet, are a persistently cold temperature and blue discoloration. They often feel sweaty or moist, and swelling may be present. The blue cyanosis usually appears worse upon exposure to cold, and improves upon warming. Rarely is there any pain associated. Normal arterial pulses are always present in the hands and feet since there is no blockage of the larger arteries of the arms or legs.
Generally the treatment is a common sense approach to preventing cold exposure and keeping the feet dry. This may involve the use of insulated boots, thin polypropylene liner socks to wick the moisture away from the skin, and an insulated sock to maintain normal skin temperature. Generally no other treatment is necessary. Vasodilators have been tried with limited success. In extreme cases a surgical procedure called a sympathectomy has been performed to relax the persistent vasospasm. This surgery is rarely necessary, and seldom recommended.
There are two sets of veins which carry blood from the feet back to the heart and lungs. The superficial veinsare located just beneath the skin, and are often visible as enlarged or varicose veins. The second network of veins are the deep veins which are located close to the bone of the leg and thigh, and are not visible. Each of these sets of veins has the potential to develop a blood clot.
A blood clot is referred to as a thrombosis. When a blood clot forms there is usually an inflammatory reaction. An inflamed vein is called phlebitis. The inflammation causes swelling, pain, redness, and warmth along the course of the vein. Because these two events (blood clot and inflammation) almost always occur together, the terms venous thrombosis and thrombophlebitis are both used to refer to a blood clot in a vein.
Under normal circumstances blood does not form a clot in the vein. There are certain things, which will make a clot more likely to occur. For example, some families have an unusual trait, which causes their blood to clot more than normal. This is a hereditary abnormality. More common causes of blood clots would include a direct injury to the vein, oral birth control pills, long hours of sitting (for example truck drivers), a recent surgery, prolonged periods of bed rest, a recent pregnancy, or the presence of some types of cancers. Each individual with a blood clot should be evaluated for the underlying cause.
When a thrombosis (blood clot) forms in a superficial vein in the foot or leg it is recognizable as a linear, firm cord. That is because these veins are inflamed and swollen, and because they are located just beneath the skin. They may appear red and feel warm from the inflammation. They are usually painful and very tender to pressure. Classically in the legs, these occur along the course of the greater or lesser saphenous veins. A blood test to evaluate white blood cells may be necessary to differentiate superficial phlebitis from cellulitus (an infection under the skin which can also lead to pain, swelling, and redness of the foot or leg).
Superficial phlebitis, although painful, is not a serious condition. This condition should be evaluated by your doctor to rule out other more serious problems. Treatment usually involves the use of anti-inflammatory medication, elevation of the foot and leg, and warm compresses applied over the area of inflammation. Diabetic patients should not use heating pads or warm compresses unless they are supervised by their treating doctor.
Deep Vein Thrombosis
Although occasionally asympotomatic, most people with deep vein clots complain of pain, swelling, and warmth of the leg. There may be swollen superficial veins as well. The leg pain and soreness is worse with standing or walking, and feels better with rest and elevation. When the area is examined there is often severe tenderness with deep pressure, although this could also be found with muscular problems in the same location.
Confirmation of a suspected deep vein thrombosis can be made by ultrasound testing or by venogram. These tests are important because it is sometimes difficult to establish the diagnosis without them. A proper diagnosis is essential with deep vein clots because failure to properly treat these can result in chronic venous insufficiency or a life threatening pulmonary embolus.
Most patients with deep vein thrombosis require hospitalization. In this setting the patient will be given a blood thinner to prevent blood clots from spreading in the leg veins, and to prevent pieces of the clot (emboli) from traveling up to the lungs. Traditionally, heparin has been the blood thinner used in this situation. Other clot dissolving medications are now sometimes added to this treatment to prevent long term damage to the veins, thereby helping to preventing chronic venous insufficiency. Care must be taken with all these medication because they can result in serious undesirable bleeding. An additional treatment, which may be recommended, is a filter, which is surgically placed into the large vein returning blood to the heart. This filter is to prevent pieces of clots from traveling from the leg veins up to the heart and lungs. Clots in the lungs can cause death.
Once the patient has been stabilized, the heparin is discontinued and an oral blood thinner called warfarin(Coumadin) is used. This is usually continued for several months depending upon the severity of the episode, and the patient. Periodic blood tests are required to monitor the bleeding and clotting ability of the patient. The dose of the Coumadin is then adjusted as necessary.
Following an episode of deep vein thrombosis it is wise to wear a firm below knee elastic stocking (30 – 40 mm compression) to control swelling. Failure to wear an elastic stocking can lead to chronic venous insufficiencyand it’s associated problems including pain, swelling, dermatitis, skin discoloration, and ulcerations.
Erythromelalgia is a fairly rare disorder manifested by vasodilatation of the blood vessels in the feet. Normally blood flow through the feet and hands is regulated by nerves and muscles in the walls of the blood vessels that either tighten and shrink the vessel’s diameter so as to restrict flow or to open the blood vessels diameter to allow for more flow. This is all controlled automatically by the body and is necessary to preserve or give up body heat so that we maintain a constant body temperature.
For instance, when we are subjected to cold temperatures the blood vessels will constrict and shunt blood back to the heart and body cavity. This is an example of how our body responds to cold and is a survival mechanism to keep us alive if we where exposed to prolonged or severely cold temperatures. The heat of the blood is not allowed to escape in the fingers and toes into the air or water around us and is sent back to the heart to keep the core body temperature warm so your heart will continue to beat. Certainly you have heard “Cold hands, warm heart”. When we are hot just the opposite happens and the blood vessels enlarge or dilate allowing for more blood to go to the fingers and toes thereby ‘giving up heat’ to the surrounding air. The ‘cooler blood then goes back to the heart where it helps lower core temperature.
The vasoconstriction (tightening of the blood vessels) and the vasodilatation (opening of the blood vessels) is always changing and adjusting to maintain blood pressure, control body heat, regulate heart rate, among other functions you don’t even think about. Part of the controls for this are partially understood and maintained by the primitive part of our nervous system called the autonomic nervous system. It is this part of our nervous system that is responsible for out heart to beat and for us to breath regularly without having to think about it.
When these controls fail to operate normally we see the pathologic disease patterns of erythromelalgia or Raynauds disease or phenomenon. Raynauds disease or phenomenon is a vasoconstriction of the blood vessels in the feet and hands. We notice it when our fingers and toes get icy cold and turn blue or even white. This can be a very painful condition depending on how long we are subjected to the cold and the vasoconstriction since the tissues of the hands or feet are deprived of blood and therefore oxygen. In many individuals it may be very mild and not be a problem. All of us experience vasoconstriction to some degree when we are in cold weather. In the disease state however the vasoconstriction does not entirely reverse when subjected to warmer temperatures and a chronic painful situation ensues.
Erythromelalgia on the other hand is just the opposite. The blood vessels are open or dilated and the oxygen and heat of the blood is discharged into the tissues making them turn red and feel hot all the time. Likewise this can be a very painful condition. It is a more rare disorder and less understood than Raynauds.
Before treatment, the diagnosis should be confirmed. This can be accomplished by a variety of different medical specialties. Internal medicine or an internist is a good place to start to make sure there are no disease factors or other medications causing the Raynauds or erythromelalgia. In many instances the internist will treat the disorder so he may help you monitor medications that have undeliverable side effects or react with other medications.
These disease states can be treated with varying degrees of success using drugs that induce vasodilatation or vasoconstriction. Unfortunately, the side effects of vasodilators or vasoconstrictors are often times worse than the disease. Obviously, avoiding temperatures or situations that can trigger the responses are also useful. For instance, people with vasoconstrictive problems should wear socks and well insulated shoes to maintain heat. Patients with vasodilatation problems may be more comfortable in sandals, going barefoot, or certainly using a light shoe that can ‘breath’ to allow heat to escape. In severe conditions pain medications can be a useful adjunct. Hypnosis and biofeedback may have some degree of success in certain individuals if administered by appropriately trained individuals.
Gangrene of the skin is associated with the loss of blood supply of a particular area. In some instances, it is caused by bacterial infection of an open sore or ulceration. The most common form of gangrene develops in the feet of people with diabetes who also have associated loss of circulation in the feet and toes. Any person with poor circulation can develop gangrene. A sudden onset of pain in the feet or legs associated with a decrease in skin temperature, and color changes to the skin of the feet is a strong indication that there has been a sudden blockage of blood flow to the legs. This condition needs immediate medical attention. People who have diabetes may not experience pain associated with such an event because of a condition called diabetic neuropathy. Diabetic neuropathy affects the nerves of the feet and legs causing a diminished ability to perceive pain, excessive heat, cold, vibration, or excessive pressure. This condition places people who have diabetes at greater risk of injury from any source without their being aware of it. For instance, a patient with diabetes can develop an ingrown toenail, and if they also have diabetic neuropathy, they may not experience the same level of pain as someone without the neuropathy. As a consequence the ingrown toenail can worsen, and become infected without providing the warning signs of pain. If the person with diabetes also has poor circulation, the infection can lead to gangrene of the toe. This situation can ultimately lead to the amputation of the toe, foot, or leg, depending upon how bad the circulation is in the leg.
Treatment consists of surgical removal of the gangrene, surgery to improve the circulation (by-pass surgery), hyperbaric oxygen treatment and IV antibiotics.
Severe infections can also cause gangrene. The flesh-eating bacterium called Hemolytic Streptococcus is a rapidly spreading infection. Intense local heat, redness, swelling, fever, and weakness characterize this rapidly developing infection. The infection can start with a small abrasion or injury. This condition requires immediate medical treatment. It can result in amputation and/or death. Treatment consists of surgical removal of the infected tissue and IV antibiotics and supportive care as needed for any failing body functions.
Peripheral Vascular Disease
Circulation disorders includes a large number of different problems with one thing in common, they result in poor blood flow. Specifically, the term peripheral vascular disease refers to blood flow impairment into the feet and legs (although it could include the arms and hands as well).
Blood is circulated throughout the human body by the strong, muscular pump called the heart. With each heartbeat, blood is pushed along through blood vessels called arteries that carry the oxygen and nutrient rich blood to all parts of the body including the legs and the feet. The individual cells in the body take up the oxygen and nutrients. Then a second set of blood vessels known as veins carry the oxygen depleted blood back to the heart and lungs to get more oxygen, and again be pumped throughout the body. Peripheral vascular disease may refer to arterial inflow disorders, (arterial insufficiency) or venous outflow disorders (venous insufficiency).
Arterial inflow disorders are categorized by the size of the artery involved. If a large artery in the thigh or behind the knee becomes blocked by cholesterol deposits this is referred to as large vessel disease or atherosclerosis. The result may be a painful ischemic foot, which means there is a severe lack of arterial blood flow from the heart into the foot. If smaller arteries like those in the lower leg or foot is blocked, this is referred to as small vessel disease, or arteriosclerosis. This too can result in ischemia of the foot. Small vessel disease is seen more often in diabetics, but can affect non-diabetics as well. If the skin of the feet or legs lacks adequate blood flow a sore will develop which may be difficult to heal. These sores are known as ischemic ulcers. Any blockage to arterial inflow will result in a circulation problem to the body tissues down stream. Occasionally a small blockage will occur in the small arteries that supply blood to a toe. This is known as a “Blue Toe Syndrome.” Another arterial inflow problem may result when the smooth muscles that control the size of the arteries go into spasm. The arterial muscle spasm can block the blood from circulating into the foot. One common vasospastic disorder is called Raynaud’s Syndrome. A second vasospastic disorder is called acrocyanosis.
Venous outflow disorders refer to problems getting blood from the foot back to the heart. There are two sets of veins in the feet and legs to help bring the blood back toward the heart. The superficial venous network refers to veins located just beneath the skin. The deep venous networks are veins located closer to the bones and are not visible when looking at the foot or legs.
Varicose veins refer to an enlargement of the veins and a loss in the ability of the vein to properly maintain blood flow back toward the heart. When this occurs blood can collect in the feet and legs. Superficial varicose veins may appear as unsightly cords or a small bunch of grapes, which usually appear on the tops of the feet, around the ankles and may extend upward to the knees and thighs. Deep varicose veins while usually not visible will result in chronic swelling of the feet, ankles and legs. When the blood is not circulated from the feet back to the heart gravity will cause the fluid to collect in the feet and ankles. This results in swelling, called edema. Chronic edema over a long period of time may cause a discoloration of the skin around the ankles. The skin can become inflamed, and is know as venous stasis dermatitis. If left untreated the skin will become weakened and a weeping sore will develop, usually on the inside of the ankle called a venous stasis ulcer.
A potentially serious consequence of blood collecting in the feet and legs is the formation of blood clots in the veins. A superficial vein blood clot will result in a painful, inflamed superficial vein called superficial phlebitis. When a blood clot forms in a deep vein, it is called deep venous thrombosis, or deep phlebitis. This is a serious condition that causes painful swelling of the leg and may result in part of the clot breaking free. If the clot should travel back up to the heart and get caught in the lungs, it is referred to as a pulmonary emboluswhich can be life threatening and requires emergency treatment.
Raynaud’s Disease is a vasospastic disorder most commonly affecting the hands and feet. A vasospasm occurs when the smooth muscles controlling the small arteries supplying circulation into the hands and feet contract. This smooth muscle contraction, or spasm, makes the arteries so small that blood has difficulty passing through. The most common event causing the vasospasm is exposure to cold temperature. Raynaud’s disease must be differentiated from Raynaud’s phenomenon. Raynaud’s disease is a disease that is not associated with any other specific disease entity. Raynaud’s phenomenon has the same findings, as Raynaud’s disease except there is an underlying disease associated with the vasospasm. Raynaud’s phenomenon may be seen with rheumatoid arthritis, scleroderma, lupus, and other diseases. The condition is aggravated by smoking. A single cigarette may decrease the circulation by one half for more than an hour. Raynaud’s disease is most common in young women, and has no known cause. It typically is found in both feet and both hands. It appears that the threshold for vasospasm is lowered in these individuals, and may occur following exposure to cold, or during times of stress.
The diagnosis of Raynaud’s disease is a clinical diagnosis based upon the patients’ symptoms and the findings on examination. Patients with Raynaud’s disease will often hear the expression, “”Cold hands, warm heart”" when shaking hands with others. This is because the arterial vasospasm decreases blood flow into the hands and feet. In addition to the cold skin temperature, there is a pattern of color changes seen in the fingers and toes. Initially after exposure to cold, the toes and fingers will turn white, then as the oxygen is depleted they become cyanotic which is a blue color, finally the spasm relaxes and there is an excess of blood flow which turns the toes red and warm (reactive hyperemia). This pattern of triphasic color change, white to blue to red, is a hallmark of Raynaud’s disease. However, not all individuals will have the triphasic (three-color) change. Some will have a biphasic (two color) change going directly from white to red.
The length of vasospasm may last from several minutes to several hours. This may be accompanied by pain, or more commonly by numbness. In severe cases there may damage to the skin on the tips of the toes and fingers with an appearance similar to frostbite. These areas may develop into ulcers (sores) which can be very painful.
In mild forms of Raynaud’s disease the simplest treatment is to protect the hands, and feet from cold exposure. Since cold is the cause of the vasospasm, preventing exposure to cold will prevent the spasm from occurring. This may require insulated boots and thick warm socks in colder climates. Dressing in layers, with new polyester liners and new insulating materials may be helpful. Most sporting goods stores that stock hiking or camping equipment will be able to help in this regard. Cigarette smoking contains nicotine, which is known to cause vasospasm. Discontinuation of cigarette smoking will be a definite benefit for the individual with Raynaud’s disease. In more severe cases, or in those with recurrent episodes, medications are available to help relax the arterial smooth muscle. Medications that have been used successfully in some individuals include phenoxybenzamine, prazosin, nifidipine, pentoxyphylline, and guanethidine. These medications are only available by prescription, and patients must be monitored for potential side effects.
The term “ischemic foot” refers to a lack of adequate arterial blood flow from the heart to the foot. There are a wide variety of possible causes for poor arterial circulation into the foot including arterial blockage from cholesterol deposits, arterial blood clots, arterial spasm, or arterial injury. The ischemic foot is also referred to as having arterial insufficiency, meaning there is not enough blood reaching the foot to provide the oxygen and nutrient needs required for the cells to continue to function.
The result of insufficient blood supply to the foot can manifest itself in a variety of ways depending upon how severe the impairment to circulation. Early symptoms may include cold feet, purple or red discoloration of the toes, or muscle cramping after walking short distances (intermittent claudication). Later findings may include a sore that won’t heal (ischemic ulcer), pain at night while resting in bed, or tissue death to part of the foot (gangrene).
The diagnosis of ischemia is made by reviewing the patient’s symptoms, examination of the foot, and special testing to evaluate the circulation. The examination should reveal cold skin temperature, and skin atrophy that causes the skin to appear shiny or paper thin with loss of normal hair on tops of the toes and on the lower leg. There is often a color change associated with ischemic feet. This may show as a purple discoloration of the toes, white blanching of the toes when the foot is elevated, and red discoloration when the foot is hanging down. Additionally the two arterial pulses in the foot will not be as strong as normal, or may be entirely absent. Certainly the presence of a pale looking ulcer, or black gangrenous toes would be an ominous sign of poor circulation.
When these findings are present further testing is usually required. This will often include arterial Doppler testing. This test uses sound waves to listen to the blood flow through the arteries and is able to record the quality of the blood flow and also the blood pressure. If the quality of blood flow is poor and the pressure is greatly diminished, this would indicate a lack of adequate blood flow. A second test may be required to further determine where the arterial blockage is located and how much blood is able to squeeze past the blockage. This test is known as an arteriogram. The arteriogram requires the injection of a special dye into the artery so that the artery will be visible when an x-ray is taken. This x-ray can then show where the artery is blocked and how much blood can flow past the blockage.
In the early stages of ischemia of the foot, the doctor will often recommend a program of walking exercises to increase blood flow, protective shoes and insoles if necessary, to protect the skin from rubbing producing irritations which may lead to ulcerations. Medications are also available to help improve the blood flow into the feet.
In more advanced stages of ischemia, a referral to a vascular specialist is appropriate for further evaluation. Oftentimes, if the patient is in otherwise good general health, a surgery may be recommended to bypass the blocked artery or to clean out the area of blockage. This can be major surgery, however in these cases, failure to improve the circulation into the foot may result in gangrene, which may ultimately require amputation of part of the foot or leg. The surgery is an attempt to save the foot and leg from the impending amputation. The surgery has improved over the years and the chances for success are now greater than ever before. However, each individual needs to be evaluated as to the potential risks and possible benefits from this type of surgery.
Venous stasis refers to loss of proper function of the veins in the legs that would normally carry blood back toward the heart. This may occur following injury to the veins, which can result in blood clots in the superficial veins known as superficial phlebitis, or following blood clots in the deep veins known as deep venous thrombosis. Swelling in the lower legs and ankle can also occur as a result of heart disease called Chronic Congestive Heart Failure and due to kidney disease. In some instances the cause of the swelling may not be easily identified.
Individuals with this condition usually exhibit edema, which means swelling, of the legs and ankles. The superficial veins in the legs may be varicosed, causing the veins to be enlarged and appear as a cord or a bunch of grapes. Patients often complain of a feeling of fullness, aching, or tiredness in their legs. These symptoms are worse with standing, and are relieved when the legs are elevated.
As the condition progresses the blood continues to collect in the feet, ankles, and legs. The pigmentation from the red blood cells stains the skin from the inside, and a reddish-brown discoloration develops on the skin. This is called venous stasis dermatitis.
In severe cases of long-standing venous stasis, the skin begins to lose its elasticity, and a sore may develop on the inside of the ankle. This is known as venous stasis ulceration. This ulcer often will drain large amount of fluid and will have a red base. Secondary infection can complicate the ulcer and will require antibiotic treatment.
Further testing may be requested by your doctor to further evaluate the condition of your veins. This may includevenous Doppler testing, which uses sound waves to listen to the blood flow through the veins. If there is a suspicion of an acute thrombosis (blood clots), a venogram may be requested. This enables the veins to be visible on x-rays, and the blood clot can be identified with greater certainty. Identification of deep vein thrombosis is important, because failure to properly treat may result in a blood clot breaking loose in the leg and traveling to the lungs called pulmonary embolus, which can be fatal.
The most common treatments for venous stasis are rest, elevation, and compression stockings. When elevating your feet the ideal position is to have your feet above the level of your heart. This permits greater return of blood back toward the heart. This usually means you are lying down with your legs raised with pillows.
The compressive stockings come in different lengths. A knee high stocking may be sufficient if the swelling is confined to the lower legs and ankles. However if the swelling extends up to the knee, then a thigh high or panty hose style elastic stocking may be required. The compression stockings are also available in a variety of compression strengths. The greater the compression the more squeeze the stocking will apply to the leg. Generally, over-the-counter elastic stockings are available (without prescription) in most pharmacies or surgical supply stores. These have a compression range of 10 to 20 mm compression. If these do not provide enough compression to control the edema, then a prescription compression stocking may be necessary. These begin at 30 to 40 mm compression, and are often referred to as T.E.D. stockings. In more severe cases a higher level of compression may be necessary. These stockings often need to be custom sized to each individual leg, otherwise they are difficult to put on and may not provide even compression throughout the extremity. Your doctor may also use medications to reduce the swelling called Diuretics. Diuretics increase the output of urine and your doctor should closely monitor the use of this medication. If the cause of the swelling is due to heart problems or kidney problems your doctor will evaluate the need to adequately treat these conditions.