One of several primary arteriospastic disorders, Raynaud’s disease is characterized by episodic vasospasm in the small peripheral arteries and arterioles, precipitated by exposure to cold or stress. This condition occurs bilaterally and usually affects the hands or, less often, the feet.
Raynaud’s disease is most prevalent in women, particularly between puberty and age 40. A benign condition, it requires no specific treatment and has no serious aftereffects.
Raynaud’s phenomenon, however, a condition often associated with several connective tissue disorders—such as scleroderma, systemic lupus erythematosus, and polymyositis—has a progressive course, leading to ischemia, gangrene, and amputation. Differentiating the two disorders is difficult because some patients who experience mild symptoms of Raynaud’s disease for several years may later develop overt connective tissue disease—most commonly, scleroderma.
Although the cause is unknown, several theories account for the reduced digital blood flow: intrinsic vascular wall hyperactivity to cold, increased vasomotor tone resulting from sympathetic stimulation, and antigen-antibody immune response (the most probable theory, because abnormal immunologic test results accompany Raynaud’s phenomenon).
Signs and symptoms
After exposure to cold or stress, the skin on the fingers typically blanches, then becomes cyanotic before changing to red and before changing from cold to normal temperature. Numbness and tingling may also occur but are usually relieved by warmth.
In long-standing disease, trophic changes such as sclerodactyly, ulcerations, or chronic paronychia may result. Although it’s extremely uncommon, minimal cutaneous gangrene necessitates amputation of one or more fingers.
Criteria that help establish a diagnosis of Raynaud’s disease include skin color changes induced by cold or stress; bilateral involvement; absence of gangrene or, if present, minimal cutaneous gangrene; normal arterial pulses; and a patient history of signs and symptoms that last for more than 2 years. Blood flow studies and a cold stimulation test may be performed. The diagnosis must also rule out secondary disease processes, such as chronic arterial occlusive or connective tissue disease.
Initially, treatment involves avoidance of cold and mechanical or chemical injury; cessation of smoking; and reassurance that symptoms are benign.
Because adverse drug effects, especially from vasodilators, may be more bothersome than the disease itself, drug therapy to relax the walls of the blood vessels is reserved for unusually severe symptoms. Treatment of the underlying condition is also important.
When conservative treatment fails to prevent ischemic ulcers, sympathectomy may be helpful; fewer than a quarter of patients require this procedure.
VN:F [1.9.20_1166]Raynaud's disease,