Raynaud's disease, also known as Raynaud's syndrome and Raynaud's phenomenon, vascular and nerve disorders caused by small artery spasm limb disease. Paroxysmal limb to limb (mainly the fingers) symmetry of the intermittent pale, cyanosis, and flushing of its clinical features, and often emotional or induced by the cold.
Raynaud's disease rare, mostly occurs in women, especially the nervous allergy to female ratio was 1:10. Age of onset of Raynaud's disease more than 20 to 30 years old. Heavy attack during the cold season.
Raynaud's disease is mainly limb spasm of small arteries, the cause is not entirely clear, may be related to the following factors:
1, the central nervous system dysfunction, the sympathetic hyperfunction;
2, the blood circulation of epinephrine and norepinephrine levels increased;
3, the condition often aggravated during menstruation, pregnancy reduced, and it was felt and endocrine-related;
4, limb defects in small arteries themselves, show normal physiological phenomenon caused by over-reaction;
5, also suggested that initially a small artery on the cold extremities over-reaction, then the long-term vascular spasm, so that arterial intimal hyperplasia, poor blood flow, if more are to reduce limb blood flow in all arteries Physiological factors, can act on the arterial lesions caused by seizures;
6, patients often have a family history, suggesting that hereditary;
7, immune and connective tissue diseases such as systemic lupus erythematosus, scleroderma, nodular nodosa, dermatomyositis, rheumatoid arthritis, polymyositis, mixed connective tissue disease, hepatitis B antigens induced vasculitis, drug-induced vasculitis and Sjogren syndrome;
8, obstructive arterial disease, such as arteriosclerosis obliterans, thromboembolism, vasculitis, etc.;
9, physical factors, such as vibration damage, direct arterial trauma, cold injury;
10, due to certain drugs, such as ergot, lead, thallium, arsenic poisoning, polyvinyl chloride, β-blockers, cytotoxic drugs, contraceptives, etc.;
11, the mechanism of neural factors such as vascular neck rib, anterior scalene muscle syndrome, thoracic outlet syndrome, axillary crutches improper use of compression, tumor compression of brachial plexus and subclavian vessels, cervical inflammation, or rupture of the nucleus, peripheral nerve Yan, syringomyelia, or tabes, etc.;
12, cold agglutinin in the blood increase, or cryoglobulinemia, polycythemia vera, paroxysmal hemoglobinuria, etc.;
13, some with migraine and variant angina related.
Pathology and pathogenesis
The cause of Raynaud's syndrome, currently remains unclear. Most scholars believe that with the cold stimulation, nervous excitement and endocrine disorders, connective tissue disease and other factors. Divided into three basic changes: spastic ischemic period, expansion and congestion period.
Early lesions, fingers and toes were no significant pathological changes veins visible. Arterial intimal hyperplasia can be seen later, elastic membrane rupture and myometrial thickening and other changes, the small arteries narrow, blood flow. Finally, a small number of patients may have thrombosis, luminal occlusion, accompanied by changes in local tissue nutrition, severe cases can occur (toe) end of ulcers, occasional necrosis. Artery disease refers to conditions under the sign can be divided into obstructive (62.6%) and spastic (37.4%), the two groups. Obstructive obvious palm, digital artery obstruction, and more from autoimmune diseases and are associated with atherosclerosis due to chronic arteritis. Because of severe arterial obstruction, so the room temperature, mean arterial pressure was significantly reduced. Obstruction of the normal vasoconstrictor response to cold enough to cause seizures. Spastic no palm, digital artery obstruction in normal room temperature refers to the artery, at the critical temperature (18 ~ 20 ℃) was caused by seizures. Spastic abnormal adrenergic receptor changes in platelet α2-receptor activity was significantly increased, resulting in blood increased sensitivity to cold stimuli.
During the onset of Raynaud's disease, first finger (toe) artery spasm or functional occlusion, followed by capillaries and small veins are cramps, which showed local skin pale. Small vein artery spasm spasm subsided quickly, causing capillary blood stasis, hypoxia, cyanosis appears. Vasospasm removed, the local circulation restored, and the emergence of reactive hyperemia, it is skin flushing, and then to normal color.
Clinical manifestations
A typical attack of Raynaud's symptoms can be divided into three phases:
Pale period: the early performance, the case of cold stimulation, finger (toe) side of the skin pale. This change was the process of development, that is first and foremost from the finger (toe) end of the beginning, and then to refer to parts of the development of roots and above, generally no more than wrist. Pale skin began to start with a finger, and then gradually involving the other fingers, some involving almost all the fingers, the thumbs rich blood circulation, so the only serious condition involving only when. Attack consciously fingers, palms, toes, feet are cold feeling, the local temperature decreases, the temperature lower than normal limbs. At the same time may have numbness, needle-like, heavy and stiff feeling, bend your fingers to feel your fingers hold back inflation, flexion and extension negative, it can cause movement disorders. Some patients will be sweating and so on.
Bruising on: pale after a few minutes of emergence, self-lifting of small artery spasm, and spasticity is still a small vein, impairing blood flow, venous plexus and the capillaries appear at this time of hypoxic paralysis, slow blood flow or stasis, resulting in blood oxygen reduction, so the skin appears pale purple after the phenomenon. At this time the symptoms are relatively light.
Flushing period: bruising of the fingers (toes) to get warm, the cold stimulation relieved vasospasm can ease or disappear, then arterioles, capillaries and small veins reactive hyperemia, so that the phenomenon of skin flushing. At this point the local temperature increase, may have swelling and mild pulsating pain. When the normal blood perfusion, skin color and the symptoms were back to normal.
But there are many "Raynaud's sign" in patients with disease onset, unlike the laws of the clear changes in the boundaries of performance is not clear if any bruising directly into the onset of a serious number of patients with the Sustainable months without relief, the state was sustained attack, intermittent period almost disappeared, there are changes in local tissue nutrition, such as skin atrophy or thickening of the nail deformity was buckling, the finger pad weight loss, decalcified distal phalanx, finger nails to the ulcer Under the expansion, causing separation of the nail and nail bed, accompanied by severe pain. In addition, it may cause the tip of finger gangrene. 10 ~ 12% of patients can occur after a long illness limitations (toe) skin sclerosis. Some patients are also always in the first phase, after the two less obvious; also some of the patients without flushing from the pale and (or) directly after the restoration of the normal purple. Occasionally at the tip of individual patients, the zygomatic cheek, ear and other parts of the head and face similar symptoms.
