Raynaud's Phenomenon (Diabetic Vascular Disease) is a vasospastic disorder causing discoloration of the fingers, toes, and occasionally other areas. This condition can also cause nails to become brittle with longitudinal ridges. Named for French physician Maurice Raynaud (1834-1881), the phenomenon is believed to be the result of vasospasms that decrease blood supply to the respective regions. Emotional stress and cold are classic triggers of the phenomenon. It comprises both Raynaud's Disease (also known as "Primary Raynaud's phenomenon") where the phenomenon is idiopathic, and Raynaud's Syndrome (secondary Raynaud's), where it is caused by some other factor. Measurement of hand-temperature gradients is one tool used to distinguish between the primary and secondary forms.
It is possible for the primary form to progress to the secondary form. In extreme cases, the secondary form can progress to necrosis or gangrene of the fingertips.
Raynaud's phenomenon is an exaggeration of vasomotor responses to cold or emotional stress. More specifically, it is a hyperactivation of the sympathetic system causing extreme vasoconstriction of the peripheral blood vessels, leading to tissue hypoxia. Chronic, recurrent cases of Raynaud phenomenon can result in atrophy of the skin, subcutaneous tissues, and muscle. In rare cases it can cause ulceration and ischemic gangrene.
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SYMPTOMS
The condition can cause pain within the affected extremities, discoloration (paleness) and sensations of cold and/or numbness. This can often be distressing to those who are not diagnosed, and sometimes it can be obstructive. If someone with Raynaud's is placed in too cold a climate, it could potentially become dangerous.
The symptoms include several cyclic color changes:
- When exposed to cold temperatures, the blood supply to the fingers or toes, and in some cases the nose or earlobes, is markedly reduced; the skin turns pale or white (pallor), and becomes cold and numb.
- When the oxygen supply is depleted, the skin colour turns blue (called cyanosis). If the color progresses to purple, resembling a bruise that doesn't go away (or spreads in size) and is accompanied by excruciating pain, contact your doctor immediately.
- These events are episodic, and when the episode subsides or the area is warmed, the blood flow returns and the skin colour first turns red (rubor), and then back to normal, often accompanied by swelling, tingling, and a painful "pins and needles" sensation.
All three colour changes are observed in classic Raynaud's. However, not all patients see all of the aforementioned colour changes in all episodes, especially in milder cases of the condition. Symptoms are thought to be due to reactive hyperemias of the areas deprived of blood flow.
In pregnancy, this sign normally disappears due to increased surface blood flow. Raynaud's has also occurred in breastfeeding mothers, causing nipples to turn white and become extremely painful. Nifedipine, a calcium channel blocker and vasodilator was recommended to increase blood flow to the extremities and noticeably relieved pain to the breast, in an extremely small study group.
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DIAGNOSIS
A careful medical history will often reveal whether the condition is primary or secondary. Once this has been established, an examination is largely to identify or exclude possible secondary causes.
- Digital artery pressure: pressures are measured in the arteries of the fingers before and after the hands have been cooled. A decrease of at least 15 mmHg is diagnostic (positive).
- Doppler ultrasound: to assess blood flow.
- Full blood count: this can reveal a normocytic anaemia suggesting the anaemia of chronic disease or renal failure.
- Blood test for urea and electrolytes: this can reveal renal impairment.
- Thyroid function tests: this can reveal hypothyroidism.
- An autoantibody screen, tests for rheumatoid factor, Erythrocyte sedimentation rate and C-reactive protein, which may reveal specific causative illnesses or a generalised inflammatory process.
- Nail fold vasculature: this can be examined under the microscope.
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TREATMENT OPTIONS
Treatment options are dependent on the type of Raynaud's present. Raynaud's syndrome is treated primarily by addressing the underlying cause, but includes all options for Raynaud's disease as well. Treatment of primary Raynaud's focuses on avoiding triggers.
General care
- Avoid environmental triggers, e.g. cold, vibration, etc. Emotional stress is another recognized trigger; although the various sources of stress can not all be avoided, it is possible to learn healthier, more effective ways of dealing with them, which will reduce stress and its damaging physical effects.
- Keep your hands, feet and head warm-especially your fingers, toes, ears, and nose - by wearing mittens, insulated footwear, a ski mask; or using hand and foot warmers, etc.
- Quit smoking.
- Avoid caffeine and other stimulants and vasoconstrictors that have not been prescribed to you by your doctor. Read product labels. Caffeine is found not only in coffee and tea, stay-awake pills, soft drinks, and candies, but also in some cosmetics, soaps, and shampoos (shower shock caffeinated bar and alpecin shampoo).
- Make sure all your doctors know about all the medicines you take and the OTC remedies you use, especially hormones and drugs that regulate hormones, such as hormonal contraception, so that these professionals can make an assessment of your chemical regimen and make any changes that may be indicated. Contraception which is low in estrogen is preferable, and the progesterone only pill is often prescribed for women with Raynaud's.
- If you are diabetic, follow your diabetes treatment plan.
- Calcium channel blockers can be helpful for the treatment of Raynaud's phenomenon.
Emergency measures
If white finger (Raynaud's) occurs unexpectedly and a source of warm water is available, allow tepid to slightly warm water to run over the affected digits while you gently massage the area. Continue this process until the white area returns to its normal, healthy color.
If triggered by exposure in a cold environment, and no warm water is available, place the affected digits in a warm body cavity - arm pit, crotch, or even in the mouth. Keep the affected area warm at least until the whiteness returns to its normal, healthy color. Get out of the cold as soon as possible.
A useful method exists to take measures ensuring circulation is restored quickly, though this should not be attempted if other circulatory issues are present that disallow fast or vigorous movement. This method is achieved by "windmilling" the arms (swinging them in large circles) reasonably fast to force the blood to the hands and fingers. This can cause dizziness, so it should be attempted only for a short time before stopping for a minute and subsequently continuing. A less effective (but less conspicuous) variation is to swing the arms back and forth in a part of a circle to achieve the same results. This variation does take longer, though, and requires swifter movement of the arms, so it is advised to use the first variation if possible.
Drug therapy
Treatment for Raynaud's phenomenon may include prescription medicines that dilate blood vessels, such as calcium channel blockers (nifedipine) or diltiazem. It has the usual common side effects of headache, flushing, and ankle edema; but these are not typically of sufficient severity to require cessation of treatment.
There is some evidence that Angiotensin II receptor antagonists (often Losartan) reduce frequency and severity of attacks, and possibly better than nifedipine.
Alpha-1 adrenergic blockers such as prazosin can be used to control Raynaud's vasospasms under supervision of a health care provider.
In a study published in the November 8, 2005 issue of Circulation, sildenafil (Viagra) improved both microcirculation and symptoms in patients with secondary Raynaud's phenomenon resistant to vasodilatory therapy. The authors, led by Dr Roland Fries (Gotthard-Schettler-Klinik, Bad Schönborn, Germany), report: "In the present study, capillary blood flow was severely impaired and sometimes hardly detectable in patients with Raynaud's phenomenon. Sildenafil led to a more than 400% increase of flow velocity."
Fluoxetine, a selective serotonin reuptake inhibitor, and other antidepressant medications may reduce the frequency and severity of episodes if caused mainly by psychological stress.
Surgical Intervention
In severe cases, a sympathectomy procedure can be performed. Here, the nerves that signal the blood vessels of the fingertips to constrict are surgically cut. Microvascular surgery of the affected areas is another possible therapy. Infusions of prostaglandins, e.g. prostacyclin, may be tried, with amputation in exceptionally severe cases.
A more recent treatment for severe Raynaud's is the use of Botox. The 2009 article studied 19 patients ranging in age from 15 to 72 years with severe Raynaud's phenomenon of which 16 patients (84%) reported pain reduction at rest. 13 patients reported immediate pain relief, 3 more had gradual pain reduction over 1-2 months. All 13 patients with chronic finger ulcers healed within 60 days. Only 21% of the patients required repeated injections. A 2007 article describes similar improvement in a series of 11 patients. All patients had significant relief of pain.
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