Acral pallor, hair group, and flushing reactions

Introduction The Raynaud's phenomenon (RP) is also called Raynaud's syndrome. It is characterized by the appearance of pale, hair and flushing three-phase reactions in the extremities, mostly in the upper limbs, bilaterally symmetric, but also involving the lower limbs, or both upper and lower limbs, occasionally in the ears, nose, cheeks or collar. Often induced by cold or emotional agitation. When the attack occurs, the fingers are cold, the skin is obviously pale, stiff, and even the fingers are difficult to move. At the same time, there is a feeling of numbness and acupuncture, and then the color is deepened, which is dark red or blue-purple. When severe, some nails are also grouped, and then the skin color Lightening, diffuse flushing, increased beating sensation, and finally returned to normal. Repeated Raynaud's phenomenon can cause local ulceration, atrophy, hardening and even gangrene. But more is the variety of nutrients in the fingers (toes), often pointed or pointed, or nails can be distorted. The Raynaud phenomenon can be divided into primary and secondary. The former cause is unknown, is a benign acromegaly, also known as Raynaud's disease, more common in women. The latter is secondary to other diseases, namely the manifestation of Raynaud's phenomenon in other diseases. Recent studies have shown that the Raynaud phenomenon not only affects the extremities, but also the Reina elephant in the internal organs of patients with connective tissue diseases, mainly involving the lungs, heart, brain and kidneys. As for whether the Raynaud's phenomenon can cause damage to internal organs, there are different reports. .

Cause

Raynaud's phenomenon can occur in many diseases, the main cause:

Rheumatoid arthritis (RA), Sjogren's syndrome (SS).

Arterial disease: occlusive arteriosclerosis, thromboangiitis obliterans.

Nodular polyarteritis, hematologic abnormal cryoglobulinemia, cold agglutininemia, paraproteinemia, red blood cells and thrombocytosis.

Neurovascular: thoracic hemorrhagic syndrome, shoulder compression syndrome, subarachnoid aneurysm, subclavian artery ulcer.

Occupational diseases: steam hammer disease, invasive occlusive arterial disease and vasospasm, tremor pale (professional occupations such as typing and piano playing), working lesions in long-term wet and cold environment, and vinyl chloride disease.

Drug-induced lesions: ergotamine, blockers, cytotoxic drugs, sympathomimetic drugs, oral contraceptives, heavy metals (lead, Ming) and arsenic, cytotoxic drugs.

Neurological diseases: fluidic edema, malignant tumors, biliary cirrhosis, pulmonary hypertension, chronic renal failure.

mechanism:

First, the mechanism of the occurrence of the limbic Raynaud phenomenon

The mechanism of the occurrence of the phenomenon of Raynaud is not yet fully understood. The following various theories are considered to be the following factors.

1 vasculitis lesions: under the influence of various factors, certain antigens bind to phospholipids on vascular endothelial cells, under the action of complement, damage vascular endothelial cells, form vasculitis, thicken the blood vessel wall or narrow the lumen. Under the action of sympathetic nerves, blood vessels are prone to sputum and occlusion.

2 immune dysfunction: Raynaud's phenomenon is more common in connective tissue disease, systemic lupus erythematosus, polymyositis, etc., in these patients, abnormal humoral and cellular immune indicators are more than those without Renault phenomenon, such as blood RNP, Sm antibody. -globulin, circulating immune complex, and cold agglutinin are higher than those without Raynaud.

3 platelet aggregation enhancement: thromboxane A2 (TxA2) produced by platelet activation is a powerful vasoconstrictor and platelet agglutinating agent, arachidonic acid produced by vascular endothelial cells and the main product prostacyclin (PGI2) is a vasodilator And agglutination inhibitors, patients with Raynaud's phenomenon have higher levels of TxA2/PGI2 in the plasma than those without Renault, and treatment with thromboxane synthesis inhibitors can alleviate the disease, but the ratio of TxA2/PGI2 is normal, which is supposed to be a kind of human body. Compensation response. Due to the extreme instability of TxA2 and PGI2, the metabolites TXB2 and 6-keto-PGFI2 of TxA2 and PGI2 can also be determined. Increased platelet aggregation can increase platelet aggregation in the circulation, affect limb blood perfusion, and accelerate the pathological process of terminal arterial ischemia.

Second, the mechanism and inspection of visceral Raynaud phenomenon

(1) Mechanism of the phenomenon of lung Raynaud

Patients with primary Raynaud's phenomenon are not limited to peripheral vasospasm, but also associated with pulmonary vasospasm. Pulmonary capillary vasospasm leads to a decrease in the number of capillary beds and a decrease in diffuse function of the lungs. Although there was no change in lung carbon monoxide diffusion (DLCO) in patients with secondary Raynaud's phenomenon, there was also an abnormal change in nailfold microcirculation after cold stimulation. It is suggested that patients with secondary Raynaud's phenomenon have damage to the pulmonary interstitial or vascular bed, and pulmonary fibrosis or pulmonary hypertension limits the reactivity of the pulmonary circulation.

(B) the mechanism of cardiac Renault phenomenon

Using 201TI () myocardial imaging method to measure myocardial damage in patients with Raynaud's phenomenon before and after cold stimulation, is a non-invasive examination of myocardial ischemia, the number of muscle uptake and local myocardial blood flow and cell function Just proportional. Some people used this method to examine patients with progressive systemic sclerosis with myocardial damage. It was found that 10 of 13 patients induced Raynaud's phenomenon after cold stimulation, and myocardial perfusion was reduced. Domestic Li Ming and other studies also found that 6 In 5 cases, this phenomenon occurs.

However, this myocardial ischemia is transient, and the vasospasm of the heart is relieved when the Limouson phenomenon is relieved. Bulkley et al analyzed the autopsy data of 52 patients with progressive systemic sclerosis, 23 of whom were on the right, with focal constriction necrosis and secondary myocardial fibrosis. In these patients, the visceral visceral organs of the ventricular wall are smooth and unobstructed. Contraction banded necrosis is a special pathological change after myocardial cell injury. It is different from coagulative necrosis of myocardial cells caused by continuous infarction of coronary artery. It is caused by myocardial ischemia and then blood reperfusion, which is caused by cardiac Reynolds phenomenon. This myocardial damage is cumulative.

(3) Mechanism of brain Raynaud phenomenon

It has been found in foreign countries that patients with recurrent stroke have no history of migratory headaches and Raynaud's phenomenon, and no other history of stroke may occur. The cerebral arterial blood flow map and EEG are normal. It is speculated that the recurrent stroke is related to the brain Raynaud phenomenon. Domestic Li Ming et al. used 99mTc-HM-PAO cerebral blood perfusion tomography to examine cerebral blood perfusion in patients with connective tissue disease after cold stimulation and at rest. 12 patients in the Raynaud group were cold. When stimulating the limbic Raynaud's phenomenon, 8 cases of the cerebral cortex also showed multiple areas of focal blood perfusion reduction. In the resting state without the phenomenon of Raynaud, these reduced areas have different degrees of recovery. This phenomenon was not observed in patients with systemic lupus erythematosus without Raynaud's phenomenon in the control group. This temporary reduction in transient cerebral blood flow associated with the Limouson phenomenon may be due to the Raynaud phenomenon of the brain. It has also been reported that the incidence of Raynaud's phenomenon in patients with migraine headaches is higher than that of the control group.

(4) Mechanism of renal Renault phenomenon

Blood flow in the renal cortex of patients was determined by 133Xe renal perfusion imaging. In 4 patients with progressive systemic sclerosis who induced Raynaud's phenomenon under cold stimulation, the renal cortical blood flow decreased by an average of 32% compared with the Raynaud phenomenon, while the 4 normal people only reduced by 10%. After the human vasodilator drug aminophenin, cortical blood flow increased rapidly in 3 of the patients, suggesting that the decrease in blood flow is caused by renal vasospasm.

an examination

Related inspection

Skin color skin disease physical examination skin elasticity check

First, medical history

The diagnosis of the limbic Raynaud phenomenon is not difficult. By asking, the patient can describe the symptoms of a typical episode in detail. Some people think that the phenomenon of Raynaud's phenomenon alone for more than two years is an important condition for the diagnosis of the primary Raynaud's phenomenon. However, clinical studies in recent years have shown that the Raynaud's phenomenon has existed for more than 10 years alone, and some of them have developed into connective tissue diseases. The age of onset is mostly between 20-30 years old. Some patients have a family history, and the incidence ratio of female to male is about 10:1. Patients with longer course of disease should be differentiated from acromegaly, and the course of the disease is as long as 24 years.

Second, physical examination

(1) Cold water stimulation test

After sitting in a warm room for 20-30 minutes, the patient or the foot was immersed in 4 °C ice water. The local skin color was observed to be pale for about 1 to 2 minutes. After leaving the water for 2-5 minutes, the skin color became purple and flushed. With local cold, hemp, acupuncture-like pain, the seizure lasted for a few minutes. This method can estimate the extent of the condition and the therapeutic effect. More domestic applications.

(B) nail fold capillary angioscopy

It can be seen that the patient's capillary is abnormal, the blood is dark, the blood is dark, the blood is bleeding, the blood flow is slow, and the fluid state is abnormal. Normal people do not have this phenomenon. This method is commonly used in China.

(C) laser Doppler blood flow meter

The laser is emitted into the skin through the optical fiber (about 1mm below the skin surface, the human photon encounters the red blood cells moving in the microcirculation, the frequency changes, the emitted light is collected through the skin, converted into a signal is displayed, the blood flow rate can be calculated And the flow, China has not seen the report detected by this method.

(4) Determination of radionuclide

The radionuclide can be measured by radionuclide 131I sodium.

(5) Microcirculation microscope

Foreign countries have been able to display intradermal capillaries on the TV screen, directly record the flow rate and flow rate, and can also insert the cannula into the capillary network, and rub the person to measure the pressure change.

(6) Volume and photoplethysmograph

The increase and decrease values of the annular surface of the affected finger are measured, and the pulse of the finger artery is detected to estimate the change of blood flow. The blood flow of the affected finger is reduced, and the amplitude of the arterial beat is decreased.

(7) Upper extremity angiography

After the first angiography, the hand was immersed in ice water for 20 seconds, dried and then re-contrasted. In addition to the visible arterial spasm, the larger palm artery and even the forearm artery were involved.

Third, laboratory inspection

(a) cold agglutinin antibody

Some patients may have a cold agglutinin in the serum. This antibody binds to the glycoprotein I/i antigen on the erythrocyte membrane at 15-20 °C to agglomerate it, so that the red blood cells dissolve, and can be dissociated at 37 °C, mostly IgM, normal. The titer of condensed concentration in human serum is 1:32 or less, and the concentration of condensed agglutinin in Raynaud's disease can reach 1:128, but this test is not specific, and many other diseases can also detect condensed agglutinin. Such as viral pneumonia, infectious mononucleosis, rubella, lymphoma, systemic lupus erythematosus and so on.

(two) cryoglobulinemia

The plasma can be precipitated at 4 ° C and dissolved above 37 ° C. Normal human plasma fibrinogen is 2-4 g/L, which can be exceeded in Raynaud's disease. This symptom can also be seen in patients with cyanosis of the extremities, reticular leukoplakia, cold and other measles, and paroxysmal cold hemoglobinuria. A large amount of cryoglobulinemia is found only in multiple myeloma.

(3) Other antibodies

Due to the disorder of immune function, RNP antibody and anti-Sm antibody (a nucleoprotein polypeptide antibody linked to RNA) in serum of patients with Raynaud's phenomenon, blood -globulin, IgG level, and circulating immune complexes are less Reynolds The phenomenon is high.

Differential diagnosis

First, systemic lupus erythematosus

Also known as systemic lupus erythematosus, 80% can occur skin lesions, especially facial butterfly erythema. Skin lesions can be the earliest or even the only clinical symptoms, more common in young and middle-aged women, erythema appears before systemic symptoms , can also appear at the same time or after. These erythema are in different stages of the disease and are colored reddish. Bright red to purple. When the inflammation subsides, there may be scaling or pigmentation, sometimes telangiectasia or mild skin atrophy. The erythema of the palm, the sole of the foot, the side of the palm of the finger, and the side of the toe is important for the diagnosis of the disease. 90% of patients may be accompanied by fever, heat type is variable, can range from low fever to 40 °C, 90% of patients may be associated with joint pain, pain is uncertain.

Renault can occur in 18%-45% of patients, with typical three-phase response after cold, and 50%-80% of patients can find lupus cells, especially in the acute phase. However, 17% of patients with rheumatoid arthritis and patients with dermatomyositis and systemic scleroderma can also find lupus cells, which should be identified. Organ damage is most common in organ damage, with an incidence of 50%-70%, and multiple antinuclear antibodies are positive. High titer anti-ds-DNA antibody is a marker for systemic lupus erythematosus, followed by anti-ss-DNA antibody (70%), anti-histone antibody (70%), anti-Sm antibody (30%-40%) ), anti-RNP antibody (30%-50%), anti-Ma antibody (20%), anti-La/SSB antibody (15%), anti-K1 antibody (10%).

Second, progressive systemic sclerosis (PSS)

The disease is characterized by extensive or systemic skin hardening, multiple system involvement, and ultimately skin atrophy. The disease is slow onset, the earliest symptoms are often Raynaud's phenomenon, the incidence rate is as high as 80%-90%, it is more likely to occur after cold, there are typical skin three-phase changes. Therefore, the Raynaud's phenomenon has important diagnostic significance for progressive systemic sclerosis. At the beginning of the hand, the skin and the skin first appeared non-finger concave hard swelling, the surface is smooth, and the normal wrinkles are lost. It is pale yellow or pale and gradually spreads to the neck, shoulders, limbs and trunk. It can also start from the torso or limbs, and the skin gradually hardens during the development of the swelling. Because the facial skin is tightened, it is difficult to open the mouth, no expression, and the tongue strap is shortened, which makes the tongue move obstacles. The fingers are semi-curved due to hardening, the activity is difficult, and ulcers and atrophy can form.

Progressive systemic sclerosis can have multiple systemic lesions. The incidence of bone, joint, and muscle lesions is 50%-100%. The digestive tract can be affected from the mouth to the rectum. The small intestine is often accompanied by diarrhea and constipation. Pulmonary fibrosis can make breathing difficult and the chest has a tight feeling. Kidney involvement can occur with hypertension and acute renal failure, and myocardial fibrosis can cause cardiac insufficiency. In severe cases, the prognosis is poor. Laboratory examination: there may be accelerated erythrocyte sedimentation rate, increased gamma globulin and so on. More women than men (about 3-4:1), mostly adult onset, should pay attention to the identification of extremity sclerosis, adult scleredema, dermatomyositis.

Third, acral sclerosis

It is characterized by skin hardening that only occurs on the extremities and can affect the face and neck. Before the skin hardens, the hand and foot parts first appear intermittent convulsions, with typical three-phase performance. After a long-term episode, the hardening starts from the finger (toe) end, upwards the finger (or the back of the foot), and even affects the forearm or calf, which can cause finger (toe) ulceration, finger dystrophy, atrophy or shedding. Generally do not extend to the whole body, the internal organs are generally not affected, and the prognosis is good. However, some studies have shown that individual patients with acral sclerosis can eventually evolve into progressive systemic sclerosis.

Fourth, polymyositis / dermatomyositis

It is an autoimmune disease characterized by striated muscle degeneration and necrotic inflammation. There are acute, subacute and chronic points, and the common ones are chronic processes. There is no difference between men and women, and both adults and children can develop the disease. Skin lesions account for 60%, may have edema, erythema, pigmentation and telangiectasia at the same time, the diagnostic value of the lesion is called "purple eyelids", that is, the location of edema in the upper eyelid magenta skin lesions Dermatomyositis is unique. The bulging papule plaque at the bulging bulge of the knuckle, elbow, knee or ankle has a higher diagnostic value. 10%-20% of patients have Raynaud's phenomenon and the muscles are swollen. Tenderness and limited joint activity.

When you go upstairs, you can feel the pain in your legs. When you raise your arms, you can feel pain in the back of both sides. Muscle symptoms can occur at the same time as skin lesions, but can occur earlier or later. The degree of rash and muscle degeneration varies in different patients. The systemic symptoms are sweating, fever, and anemia. Laboratory examination: CPR, ALT, AST and LDH increased, 24 h uric acid creatine >200 rug, muscle biopsy is important, visible muscle fibrosis or rupture, 20% of adult patients with malignant tumors.

V. Mixed connective tissue disease (MCTD)

Both lupus erythematosus, progressive systemic sclerosis and polymyositis/dermatomyositis, such as facial erythema with systemic lupus erythematosus, telangiectasia; purple-red spots and swelling with dermatomyositis, joint extension Atrophic erythema on the side, pigmentation with cutaneous sclerosis and hardening of the skin. 95% of patients have high titers of anti-ribonucleoprotein antibodies in the blood (anti-RNP human fluorescent antibodies are examined as high titer spots. Raynaud's phenomenon is a valuable early symptom of this disease. Recurrent Renault phenomenon can lead to fingers ( Toe) ulcer. This disease is good for adrenocortical hormone.

6. Rheumatoid arthritis (RA)

It is a kind of chronic disease with chronic, symmetrical and polyarthritis. The cause is unknown and is generally considered to be an autoimmune response caused by infection. Typical skin symptoms are rheumatoid nodules, which occur in 20% to 30% of patients. The nodules occur on the extension side of the joint, mainly the elbow. The nodules are spherical, hard and have no obvious tenderness. Rheumatoid arthritis can present a variety of vascular lesions, including arteritis, capillary vasculitis, and phlebitis. The incidence of the Raynaud phenomenon was reported to be 18.9%. There are more people with gangrenous pyoderma, and the skin of the hand changes with palm erythema. 25%-30% of juvenile rheumatoid arthritis has a characteristic rash. The rash is mostly located in the trunk and limbs. The affected joints have the most joints, wrists, knees and foot joints. The joints are swollen. Tenderness and stiffness, joint stiffness is most obvious in the morning, called morning stiffness, and relieved after activity. Can affect the heart; visceral disease, echocardiography about 40% -50% of cases have pericarditis. The pleural and pleural effusions are the most common.

Seven, Sjogren's Syndrome (SS)

It is an autoimmune disease, with my membrane drying as a prominent symptom, mainly invading the lacrimal gland and salivary glands. As the lacrimal gland shrinks, tear secretion decreases. Salivary glands shrink, dry stomatitis occurs, dry mouth, dry lips, thirst, dry mouth. The tongue film is dry and red. 50% of patients have rheumatoid arthritis. Some patients with polymyositis, systemic lupus erythematosus, hardness disease. The parotid gland and thyroid gland can be swollen, and some have hepatosplenomegaly. Non-thrombocytopenic purpura can occur on the skin. There are often telangiectasia in the fingers and lips. 25% of patients have Raynaud's phenomenon. Hair tends to dry and become brittle. The pubic hair and the hair are scarce or falling off. Most of the patients are women. Laboratory tests: rheumatoid factor titers and antinuclear antibody titers were >1:160. The total number of white blood cells is reduced and eosinophilia is increased. Hypergammaglobulinemia is often present. A few patients may have condensed globulin and lupus cells.

Eight, occlusive arteriosclerosis

A disease caused by atherosclerotic lesions in the peripheral arteries, resulting in chronic narrowing or occlusion of the arteries, mainly found in large and medium-sized arteries of the lower extremities. The affected limbs are cold, numb, painful, intermittently broken, etc. due to ischemia. Symptoms. In the later stage, due to dystrophy of the limb tissue, ulcers and gangrene of the toes, feet, and legs may occur. Most of the patients are male, with more than 50 years old, 20% with diabetes, and 35% with hypertension.

Nine, thrombotic occlusive vasculitis

Arteries and veins are chronically inflamed and occluded, causing severe pain. Local tissues often cause gangrene due to ischemia, which can cause limbs to be destroyed. 75% of patients had "intermittent breaks". In severe cases, due to local tissue and nerve ending ischemia, there were severe paroxysmal pain in the lower limbs and toes during rest. There are beating burning pains in ulcers and gangrene, and the heaviest in the evening. The dorsal artery pulsation can disappear. More common in men aged 25-50, smoking is an important incentive.

Ten, nodular polyarteritis

It is a systemic disease. The skin lesions are characterized by subcutaneous nodules and erythema nodules distributed along the blood vessels, and can lead to necrosis and ulceration, resulting in epilepsy. The incidence of men and women is 7:4. Early manifestations include fever, peripheral neuritis, muscle and joint pain, loss of appetite, and weight loss. Skin damage occurs in 1/3-1/4 of patients. It is common to have a single or clustered subcutaneous nodule along the blood vessels of the calf, which has tenderness. The nodules are adhered or non-adherent to the epidermis and are as large as soybeans to red dates. The skin surface can be normal skin tone, bright red or dark red. Sometimes edema, and soon nodules can occur necrosis, forming a difficult to heal ulcer. After several months or more, the ulcer heals, leaving significant signs of seizures and pigmentation. Red spots, wheal, purple epilepsy, and ulcers of the extremities can be seen on the skin, and Raynaud's phenomenon can occur.

XI, cryoglobulinemia

It is characterized by the occurrence of extremities, partial cyanosis, purpura, Raynaud's phenomenon, reticular bluish or cold measles in the exposed parts of the hands, feet, auricles and tip of the nose. There are even skin necrosis or ulcers. The symptoms are numbness and tingling, which occurs in young people or children.

The disease is divided into idiopathic and secondary, secondary to lupus erythematosus, nodular polyarteritis, systemic sclerosis, dry syndrome, allergic vasculitis, acromegaly, Reticular leukoplakia, xanthoma, acne, hives dermatitis and phyllos. There are also some malignant tumors such as multiple myeloma, lymphosarcoma, liver cancer, chronic lymphocytic leukemia, chronic infections such as syphilis, kala-azar, lepromatous leprosy, etc. What can not be found is called idiopathic.

The serum chillin in the patient is >250mg/L, which can be confirmed by the following method: 10mm of venous blood is collected, serum is separated at 37 °C, and then precipitated and gelatinized in a refrigerator at 4 °C, and put back at 37 °C to dissolve. The ice water test tube is placed on the patient's skin. The purpura may appear on the lh, and the ice water test tube is placed on the eyelid. The red blood cells in the orbital blood vessels are arranged like a paper money or arranged in a string by a living microscope, and the blood flow is slow. Laboratory tests: rheumatoid factor, antinuclear antibody, syphilis serum reaction may be positive, red blood cells and protein in the urine, abnormal renal function, cold globulin in the cerebrospinal fluid.

Twelve, condensed agglutinin

When the body's condensed agglutinin is increased, it can cause Raynaud's phenomenon in the skin under cold stimulation. There is a typical three-phase reaction, necrosis and ulceration can occur in the extremities, and high titer of cold agglutinin in the serum.

Thirteen, thoracic outlet syndrome

In the stenosis of the clavicle and the first rib, the first rib of the anterior scalene muscle may compress the brachial plexus group and the subclavian artery to produce two major symptoms of nerve damage and vascular dysfunction. The upper ulnar nerve disorder appears due to compression of the lower brachial plexus. At the same time, the subclavian artery is compressed and the symptoms of insufficient blood supply to the upper limbs are Raynaud's phenomenon. This abnormal condition can be seen in the neck rib (the rib protruding from the cervical vertebra is called neck assist, which is a congenital anomaly, the upper part of the neck and the supraclavicular fossa can reach the hard block. It is usually found only on X-ray examination), the anterior scalene muscle. Syndrome (when sputum occurs due to inflammation, such as the patient's head to the opposite side of the rotation, the pain can be aggravated and the pulsation of the sacral artery disappears), can also be seen in the subclavian aneurysm and rib-clavicular syndrome.

Fourteen, water soaked feet

It is actually a type of frostbite that can occur in a crew member who has been immersed in sea water for a long time. At first, the feet were pale and unconscious. After leaving the water, the feet became congested with redness and fever, with ecchymoses and water cancer, accompanied by severe pain and paresthesia. Later, Raynaud's vasospasm symptoms may occur.

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