– Episodic vasospasm of the small cutaneous arteries, usually in the fingers but occasionally in the toes, nose, or tongue, that results in intermittent pallor or cyanosis of the skin.
Causes and Incidence
Raynaud’s disease may be idiopathic, or it may occur secondary to other underlying conditions such as connective tissue disorders, obstructive arterial disease, neurogenic lesions, trauma, or drug intoxication. Attacks are often triggered by stress or cold or are seen in conjunction with migraines and angina. Raynaud’s disease is a rare occurrence. The idiopathic type accounts for somewhere between 70% and 90% of reported cases and is most often seen in young women.
The pathophysiology is not fully clear but involves a severe constriction of cutaneous vessels followed by vessel dilation and then a reactive hyperemia. Catecholamine release and prostaglandin metabolism are thought to play a role in the process. Vessels may thicken with advanced disease.
Intermittent attacks lasting from a few minutes to hours cause the fingers to blanch, then become cyanotic, and finally turn red and throb with pain. In long-standing disease, the skin becomes smooth, shiny, and tight with a loss of subcutaneous tissue. Small, painful ulcers may appear on the tips of the fingers.
Recurring infection and gangrene are rare complications of severe Raynaud’s disease.
The diagnosis is made from the clinical pattern and by abnormal perfusion patterns on digital plethysmography or peripheral arteriography.
Sympathectomy for individuals with progressive symptoms.
Reserpine to reduce vasoconstriction; alpha-adrenergic blocking agents and calcium antagonists to dilate peripheral vessels; use of prostaglandins is under study.
Avoidance of exposure to cold and mechanical and chemical irritants; quitting smoking; stress reduction programs, such as biofeedback; treatment of underlying causes in secondary disease.