Raynaud Syndrome

Short duration vasospasms of the small arteries of the arms and legs, hands and feet. Usually diagnosed before age 40 years.

Raynaud Disease; Raynaud Phenomenon; Acrocyanosis Chronica Anesthetica; Symmetric Asphyxia.

Raynaud Disease refers to the familial (genetic) part. Raynaud Syndrome or Phenomenon refers to the acquired symptoms that are often associated with underlying diseases.

Disorders associated with Raynaud Phenomenon include the following:

• Neurovascular compression (thoracic outlet syndromes, crutch pressure carpal tunnel syndrome)
• Arterial diseases (thromboangiitis obliterans, thromboembolism, arteriosclerosis)
• Blood abnormalities (cryoglobulinemia, cryofibrinogenemia, cold hemagglutinins, monoclonal gammopathy, polycythemia)
• Occupational (percussion and vibratory tool workers, traumatic occlusive arterial disease, vinyl chloride workers)
• Drugs and toxins (ergot, methylsergide, beta-blocker drugs, chemotherapy, epinephrine)
• Connective-tissue diseases (systemic sclerosis, systemic lupus erythematosus, mixed connective-tissue disease, rheumatoid arthritis, dermatomyositis, polymyositis)
• Miscellaneous (complex regional pain syndrome—reflex sympathetic dystrophy, hypothyroidism, pheochromocytoma, neoplasm, primary pulmonary hypertension, variant angina)

First described by A.G. Maurice Raynaud, French Physician, in 1862.

Not known because of the confusion between the acquired form and familial illness; however, reported to range from 0.06 to 30 from 0.006 to 30% in the general population. Primary disease may vary from 15 to 75% in this population. Male:female ratio is 1:5. Most common in young women and 60 to 90% of reported cases are idiopathic.

No evidence of genetic inheritance.

Unknown.

Clinically evocated by characteristic attack of distal vasospasm with whitening of the extremities.

Intermittent attacks of numb and white fingers. The classic description includes three phases. First, vasospasm leads to pallor of the fingers and toes. Usually bilateral and involves all fingers. Occasionally, it may involve ears and nose. Second, capillaries dilate after a few minutes, but the digital arteries still are in spasm. The fingers get blue and the numbness and pain appear. Finally, the vessels dilate with redness of the fingers with a feeling of warmth and itching and tingling. In long-standing cases, atrophy and resorption of the fingertips with or without painful areas of necrosis may appear.

Assess the presence and the severity of the underlying diseases.

Special attention to avoid cold exposure.

Avoid methylsergide and ergot derivatives. Minimize the use of beta-blocking drugs, and, if possible, catecholamines, especially the more potent alpha-vasoconstrictive ones. If absolutely necessary, consider regional sympathetic blockade for the extremities.

CREST Syndrome: An autoimmune connective tissue disorder associated with anticentromere antibodies. A form of scleroderma associated with esophageal dysmotility.

Erythromelalgia: A disorder characterized by vasodilatation associated with paroxysmal, intense burning pain, and episodic reddening of the extremities (mainly feet). The symptoms of redness, heat, pain, and swelling, when not associated with organic disease, constitute the primary form, which has also been termed “erythermalgia” because of the significance of the heat. The secondary or acquired form is related to underlying medical conditions.