Raynaud’s disease is a disease characterized by paroxysmal ischemic changes in the extremities caused by strong contraction of small arteries in the extremities after exposure to cold or emotional stress, also known as vasospasm of the extremities. During the attack, the skin of the extremity changes from pale to cyanotic, and then turns flushed. The disease is called Raynaud’s disease when there is no other related disease and a clear cause (primary); related to certain diseases (secondary) is called Raynaud’s phenomenon. Raynaud’s disease is more common in female patients, the ratio of men to women is 1:10, the age of onset is mostly in the 20 to 30 years. Winter is more common.
Etiology
The etiology of Raynaud’s disease is still not completely clear, and is related to genetic and environmental factors. Cold stimulation, emotional excitement or nervousness are the main triggering factors. Other triggers include infection, fatigue, etc. To diagnose Raynaud’s disease, we must exclude the related diseases that cause Raynaud’s phenomenon and clarify the etiology.
1. Immune diseases and connective tissue diseases
Almost all connective tissue diseases can be accompanied by Raynaud’s phenomenon and can appear before other manifestations of connective tissue diseases. Examples include scleroderma, mixed connective tissue disease, systemic lupus erythematosus, rheumatoid arthritis, dermatomyositis, and dry syndrome. The vascular lesions in these diseases are predominantly spastic in the early stages, and after repeated episodes they cause inflammation of the arterial wall, which leads to thrombosis and lumen occlusion, and eventually to tissue necrosis and ulceration.
2. Chronic occlusive arterial disease
Occlusive arteriosclerosis, thrombo-occlusive vasculitis, arterial embolism.
3. Neurological diseases
Including central and peripheral nervous system diseases, such as subthalamic tumor, spinal cord tumor, myelitis and nerve injury.
4. Drug-related factors
Ergot and other antispasmodics, beta-blockers, contraceptives, cyclosporine, heavy metal salts and discontinuation of nitroglycerin, etc.
5. Occupational factors
Such as repeated vibratory damage, small fish interferential hammer syndrome (ulcerative arterial thrombosis). Commonly found in cast iron workers, mechanics, stone workers, typists, pianists, etc. It also occurs due to direct damage to arteries, cold injury and exposure to vinyl chloride at work.
6. Blood diseases
Such as cold agglutininemia and cryoglobulinemia, etc.
7. Endocrine diseases
Low thyroid function.
8. Other
Such as chronic renal failure, malignant tumor and pulmonary hypertension, etc.
Clinical manifestations
The typical course of Raynaud’s disease is when cold stimulation or emotional excitement and nervousness, the skin of fingers appears pale and cyanotic, numbness, coldness and tingling at the end of the fingers, after warmth, the skin color becomes flushed, then there is warmth and swelling, then the skin color returns to normal and the symptoms disappear. In the early stage of the disease, the above changes occur frequently in the cold season with obvious symptoms and long duration, while the opposite is true in the warm season. If the disease is severe, frequent attacks can occur throughout the year.
The skin color changes are often regular, the affected fingers are often symmetrical, the skin color changes mostly develop in the order of the 4th, 5th, 3rd and 2nd fingers, the thumb is rarely involved because of more muscles and richer blood supply, the skin color changes first start from the end of the knuckle and gradually develop upward, but rarely exceed the wrist, all occur in both hands. The occurrence of Raynaud’s disease in the toes is less common, and pale or cyanotic skin of the ears, nose, and lips is occasionally seen.
Some patients lack the typical intermittent skin color changes, especially in the late stages, and have only pale or cyanotic episodes. In severe cases, the skin of the fingertips is nutritionally impaired, such as dry skin, muscle atrophy, brittle nails, and perinail infection. When the finger arteries are narrowed or occluded, superficial ulcers and small gangrenous areas with severe pain appear on the fingertips, leaving punctate skin scars after the ulcers heal.
Patients with Raynaud’s disease mostly have symptoms of phytodysfunction, such as easy excitement, emotional impulsiveness, paranoia, depression, insomnia and dreaminess. Raynaud’s disease does not have other systemic symptoms, Raynaud’s phenomenon can be accompanied by the clinical manifestations of the primary disease.
Raynaud’s disease can cause occlusion of small blood vessels, resulting in ischemic necrosis at the end of the finger. In severe cases, flattening and gangrene of the end of the finger may occur, and the end phalanges may be necrotic, resorbed and dissolved due to ischemia, resulting in shortening or amputation of the finger. In some patients with low resistance, ulceration due to ischemia at the end of the finger may lead to osteomyelitis and sepsis, which are the most serious complications of the disease, and the correct and timely application of anti-infective drugs can help prevent these complications.
Examination
In patients lacking typical manifestations, it is difficult to determine the diagnosis based only on their complaints. Therefore, ancillary tests and induced arterial spasm tests are needed to clarify the diagnosis and to understand the peripheral circulation. There are many methods of ancillary examinations, and the following methods are commonly used.
1. Cold water test
According to the principle of vascular response to cold stimulation, the patient’s hands are immersed in lower temperature water and the response is observed. Generally with a water temperature of about 4 ℃, soaked for 1 minute, the skin color change induced rate of 75%. This test is simple and easy to perform, but some patients may experience symptoms such as finger pain. Patients with hypertension and heart disease need to be used with caution.
2. Local cooling test
When the room temperature is 20℃, first measure the skin temperature of the fingers, and then immerse both hands in 4℃ water for 2 minutes. Then observe the change in finger skin temperature, and count the time to restore the skin temperature before the test, more than 30 minutes is positive. Can be combined with the cold water test.
3. Arm restraint test
The cuff of the sphygmomanometer is tied to the upper arm and the skin color change of the finger is observed after the release of the blood pressure measurement. This method uses pressure stimulation to induce vasospasm, which is simple and easy to perform, but the rate of induction is low.
4. Fist clenching test
Both hands are clenched for 1.5 minutes, then the upper limbs are bent at the elbows and flattened at the waist to release the hands. This test can induce skin color changes and delay the time for the skin color to return to normal from pale.
5. nail wrinkle microcirculation examination
In normal people, capillary loops are clear, neatly arranged and of uniform diameter, with a reddish-yellow base color and smooth blood flow. In contrast, the capillary collaterals of Raynaud’s disease patients are obviously reduced, the tube diameter is very thin, the tube loops are short, most of the tube collaterals are broken or dotted, and the blood flow is slow or even stagnant.
6. Arteriography
Spasm of peripheral arteries, especially the metacarpophalangeal artery, is most obvious. The arteriogram shows a small lumen and a serpentine bend in the artery; the late changes are coarse intima and luminal narrowing or obstruction of the finger artery. These changes are not usually seen in the proximal aspect of the palmar arch artery.
Diagnosis
Diagnosis is based on the clinical appearance of paroxysmal extremity skin pallor, cyanosis and flushing with tingling and numbness after cold or mood swings, and a return to normal after warmth. Diagnosis of Raynaud’s disease is based on.
(1) Intermittent color changes in the skin of the extremities during episodes.
(2) Prevalence in females, generally between 20 and 40 years of age.
(3) Both hands are usually involved, symmetrically.
(4) Cold stimulation can trigger the onset of symptoms.
(5) A few advanced cases may have occlusion of the finger arteries and/or sclerosis of the skin of the fingers, superficial ulceration or gangrene of the finger ends.
(6) Exclude Raynaud’s phenomenon and other similar diseases.
Differential diagnosis
In the diagnosis of Raynaud’s disease, care should be taken to differentiate it from cyanosis of the hands and feet, reticular cyanosis, erythema limbosum, etc.
1. Cyanosis of hands and feet
The cause of this disease is unknown, and it is mostly seen in female adolescents. It is characterized by persistent cyanosis of the skin of the hands and feet, which is widespread, glove and garter shaped, uniformly discolored, with fine skin and significantly lower skin temperature. Symptoms are heavier in the hands than in the feet, and cyanosis increases in low temperatures and when the upper limbs are down, and decreases in warm environments, or when the upper limbs are lifted. Continuous massage can make the skin cyanosis fade or return to normal, and the above skin color changes are different from Raynaud’s disease. Therefore, it is easy to distinguish the two.
2. Reticulocytosis
Under the influence of cold stimulation and other factors, spasm of small arteries occurs in the skin, accompanied by secondary dilatation of small veins and blood stagnation, so that reticular cyanosis appears on the skin surface. This kind of skin change can appear on the extremities, head and neck and trunk, which is more extensive, mostly on the lower extremities, and in severe cases, it can invade the whole extremities, but rarely appears on the hands and feet alone. Primary reticulocutaneous cyanosis has no other symptoms except chilliness and unpleasant feeling due to cyanosis; secondary cases have clinical wheals of each primary disease. According to the above characteristics, it is easy to distinguish from Raynaud’s disease.
3. Erythema limb pain
This is a disease characterized by dilatation of the peripheral arteries and sensitivity to warmth, the cause of which is unknown. The clinical presentation is characterized by four major symptoms of paroxysmal redness, swelling, pain and heat in the hands and feet. It can occur in both hands and feet, but is more common and pronounced in both feet. It is mostly symmetrical. When the temperature of the foot rises, it often feels unbearable burning pain. Therefore, patients are afraid of heat and prefer to be cool, preferring to go barefoot and immerse their feet in cold water to relieve symptoms. The symptoms of this disease and Raynaud’s disease are very different, so it is easy to distinguish.
4. Frostbite
Frostbite is a cold seasonal disease, mostly seen in children and women. The sensitivity of peripheral blood vessels to cold is the main factor. It usually occurs on both hands, feet, ears and nose, especially on the back of the hands and ear drums. Frostbite is initially locally pale, followed by redness and swelling, with small red, purple or fuchsia bordered lumps that fade when pressed, especially on the lateral dorsum of the hand. When exposed to heat, they often become congested and have a mild burning sensation. In severe cases, blisters appear and ulcers can form, which heal slowly and often leave atrophic scars. When the temperature warms up, frostbite gradually improves and can recur. For those who have had recurrence for many years, the skin of both hands may be purplish red, resembling cyanosis of the hands and feet.
Treatment
1. Local treatment
Topical medication can be 2% nitroglycerin ointment, 1%-2% nicotinic acid ointment, mucopolysaccharide polysulfate cream or compound heparin gel, 2-3 times a day.
2. Systemic treatment
(1) Vascular smooth muscle relaxants can act directly on vascular smooth muscle to relax peripheral blood vessels. Commonly used drugs include niacin and nifedipine.
(2) Anti-hypertensives and peripheral vasodilators general drugs include tolazoline hydrochloride, phenibut, prazosin, hydrogenated ergometrine and reserpine.
(3) 5-hydroxytryptamine antagonist ketanserin can antagonize the vasoconstrictive and platelet agglutinating effects of 5-hydroxytryptamine.
3. Surgical treatment
Surgical treatment, such as sympathectomy, should be performed in severe cases where drug therapy is ineffective and skin tissue nutrition is impaired.
4. Chinese medicine treatment
Chinese medicine identifies the disease as a deficiency of spleen and kidney yang and cold blockage, so the treatment is to warm the spleen and kidney, invigorate the blood and open the ligaments, which can be prescribed with the addition and subtraction of Angelica Sinensis Si-wei Tang and Yang He Tang.
Prognosis
The prognosis of Raynaud’s disease is generally good after avoiding cold stimulation, emotional excitement, avoiding smoking and drug and surgical treatment. Raynaud’s phenomenon depends on the treatment effect and prognosis of the primary disease. Raynaud’s phenomenon caused by autoimmune diseases generally has a poor prognosis.
Prevention
Avoid cold stimulation and emotional excitement as much as possible; avoid smoking and drinking; avoid staying in cold, wet, windy environment, strengthen the warmth measures, add clothes, wear thick cotton or sheepskin gloves; wearing cotton shoes, can be enough to keep warm; avoid trauma or mental stimulation, eliminate mental concerns, stable emotions, appropriate application of tranquilizers or anti-anxiety drugs; obvious occupational causes (long-term use of vibrating tools under low temperature If possible, change the work status or environment; if conditions permit, move to a warm and dry climate.