Raynaud’s disease and Raynaud’s syndrome

  In 1862, Raynaud first described a disease in which intermittent changes in the skin color of the extremities, first pale, then cyanotic, then flushed, and finally normal, can occur with cold or emotional stress, and pointed out that the pathogenesis is a vascular nerve disorder. The disease can be seen in patients with no organic lesions in the arteries of the extremities, but can also appear when there are occlusive lesions in the arteries of the extremities, while the extremities can be complicated by symmetrical ulcerative gangrene. In 1901, Hutchinson pointed out that the finger skin color change described by Raynaud’s was not an independent disease, but a clinical manifestation common to a variety of diseases of different etiologies. , into Raynaud’s disease and Raynaud’s phenomenon. Both showed similar vasospastic symptoms, but the former had no underlying disease and was milder, whereas the latter had one or more other diseases and was often more severe and could develop gangrene of the extremities.  In 1957, Giffora et al. reported a group of typical Raynaud’s syndrome with associated diseases found long after the onset of the disease. 1965, Tohnston et al. reported a follow-up of 75 patients thought to have Raynaud’s disease and found that 19% had generalized scleroderma. 1981 Porter reported the results of his follow-up of 219 patients with Raynaud’s syndrome treated over a 10-year period and found that 79% (155 cases) had primary disease such as connective tissue disease and arterial occlusive disease. Only 29% (64 cases) were found to have no primary disease. After long-term studies, many scholars have concluded that patients with Raynaud’s disease can develop significant connective tissue disease after years of skin color changes at the extremities. Therefore, it is considered unnecessary to subdivide Raynaud’s disease into Raynaud’s disease and Raynaud’s phenomenon, and to unify the name as Raynaud’s syndrome.  Raynaud’s syndrome is most common in young women, accounting for about 60% to 90% of cases, mostly secondary to other conditions, such as connective tissue disease (systemic scleroderma, rheumatoid arthritis, systemic lupus erythematosus, etc.), occlusive arterial disease (arteriosclerosis occlusive disease, thrombotic occlusive arteritis, thoracic outlet stenosis syndrome), neurogenic lesions, drug intoxication (ergot, dimethyl ergonovine, plasma protein abnormalities mucinosis, mucinous edema, primary pulmonary hypertension and trauma).  Symptoms include intermittent pallor and cyanosis at the ends of the fingers (and also the toes), which is often triggered by cold or emotional stress. The typical skin color change can be triphasic (pale – cyanotic – reddish) or biphasic (cyanotic, reddish). The skin color change is more common in the fourth and fifth fingers, rarely involving the thumb and not occurring in the palm of the hand. However, in severe cases, it can occur in both hands and both feet. Pain is uncommon, but the attacks are often associated with abnormal sensation, and prolonged and severe attacks can be associated with significant pain at the end of the fingers and even ischemic ulceration and necrosis. In some cases, it can also be seen on the tongue, nose, and ears.  Clinically, the diagnosis is not difficult based on the typical skin color changes. However, sometimes Raynaud’s phenomenon needs to be differentiated from the following: (1) mechanical obstruction of arteries, such as thoracic outlet syndrome, where the cervical ribs or crutches compress the subclavian artery causing ischemic symptoms in the distal tissues of the upper extremities, or even pulseless; (2) abnormal blood flow components, such as cold globulinemia, hyperglobulinemia, and true erythrocytosis causing increased blood viscosity and impaired circulation; (3) vascular due to drugs, tumors (pheochromocytoma, carcinoid syndrome) and diastolic disorders.  These conditions can have a localized deficiency in blood supply, but the terminal tissues do not necessarily have the typical sequential color changes. This indicates the importance of a detailed history and subtle observation.  In the treatment, the first step is to clarify the diagnosis, identify the primary cause, and use targeted therapeutic measures. For the improvement of symptoms, the use of evidence-based treatment mainly with Chinese herbal medicine, together with simple symptomatic treatment with western medicine to dilate blood vessels and improve circulation, mostly has obvious near-term efficacy. Long-term effects require long-term consolidation treatment. In severe cases, surgical treatment can be used.