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See also: Raynaud phenomenon.
Patients have intermittent vasospastic attacks of varying severity and frequency that affect the digits of the hands bilaterally; the toes are less commonly involved. Color changes occur in sequence, first white (pallor), then blue (cyanosis), and then red (hyperemia as blood flow returns). Initially, there is numbness and sensation of cold; during the red phase patients may have throbbing and paresthesia. Normal skin color returns after the attack. Patients with long-term disease may develop atrophy of the skin and subcutaneous tissues, brittle nails, and, occasionally, skin ulcerations or gangrene.
People with Reynaud disease should maintain warmth in their extremities by wearing warm mittens or gloves and socks. They should avoid contact with cold materials and prolonged exposure to cold environments; they should also avoid emotional stress. Use of tobacco is contraindicated because of the vasoconstrictive effects of nicotine. Other aggravating factors include alcohol, caffeine, and medications such as beta blockers, adrenergic receptor agonists, sympathomimetic agents, ergotamine drugs, antineoplastic agents, estrogens, immunosuppressants, biologic response modifiers, and stimulants such as amphetamines and cocaine. Increasing hydrostatic pressure, and therefore circulation, by vigorous exercise of the arms may be useful. Exercise increases circulation, warms the body, and can prevent or limit vasoconstriction. If attacks are prolonged and frequent, vasodilator drugs, including calcium channel blockers and sympatholytic agents (alpha-adrenergic receptor blockers) may be helpful. Direct vasodilators may be effective in primary Raynaud phenomenon, but not in secondary Raynaud phenomenon. Transdermal nitroglycerin or a long-acting oral nitrate reduces the severity and frequency of attacks and provides symptomatic relief in both conditions. Applying nitroglycerin cream to the fingers may help heal skin ulcerations, which progress to necrosis if left untreated. Investigational drugs (angiotensin II-receptor blockers such as oral losartin, intravenous prostaglandins, topical nitric acid gel, and cilostazol) are also used. A sympathectomy to prevent vasoconstriction may be tried but is not always successful.
Nonpharmacologic management include massaging the affected digits; placing hands under the armpits or placing hands and feet in warm (never hot) water; climate control (avoiding winter air and air-conditioned rooms); dressing warmly in winter (coat with snug cuffs, hat, scarf, waterproof and insulated footwear, thermal underwear); clothing made of wool, silk, down, or polypropylene synthetics that retain warmth; running the car heater for a few minutes before beginning to drive and insulating the steering wheel; wearing socks and shoes or slippers indoors and keeping indoor temperatures above 70°F; handling cold drinks and frozen foods only with barrier hand protection (insulated glasses or sleeves, mittens, pot holders). Relaxation techniques such as structured relaxation exercises with concurrent biofeedback should be encouraged because they can decrease the frequency and severity of attacks. Feet and hands must be protected from injury, examined daily for skin changes, and lotion used to prevent drying. Pavlovian conditioning takes time to master but may be beneficial: the hands are immersed in 110°F water for 45 min while the rest of the body is exposed to cold or freezing temperatures in order to condition peripheral vessels to dilate in response to cold rather than constricting. Complementary and alternative therapies include acupuncture, ginkgo biloba to increase blood flow in primary RP, omega-3 and omega-6 fatty acids (in flaxseed oil) to reduce red blood cell aggregation and improve blood flow, and niacin to dilate blood vessels and increase circulation to the skin.