Raynaud's disease

Raynaud’s disease is one of several primary arteriospastic disorders 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 females, particularly those between puberty and age 40. It’s a benign condition, requiring no specific treatment and causing no serious sequelae.

Raynaud’s phenomenon, however, a condition commonly associated with several connective tissue disorders — such as scleroderma, systemic lupus erythematosus (SLE), or polymyositis — has a progressive course, leading to ischemia, gangrene, and amputation. Distinguishing between 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 — especially scleroderma.

Causes and incidence

Although the cause is unknown, several theories account for the reduced digital blood flow: intrinsic vascular wall hyperactivity to cold, increased vasomotor tone due to sympathetic stimulation, and antigen-antibody immune response (the most likely theory because abnormal immunologic test results accompany Raynaud’s phenomenon). Risk factors include associated diseases (Buerger’s disease, atherosclerosis, rheumatoid arthritis, scleroderma, and SLE) and smoking.

This disorder affects females more often than males.

Signs and symptoms

After exposure to cold or stress, the skin on the fingers typically blanches and then becomes cyanotic before changing to red and before changing from cold to normal temperature. Numbness and tingling may also occur. These symptoms are 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 phalanges.

Diagnosis

Clinical criteria that establish 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 patient history of clinical symptoms of longer than 2 years’duration. Diagnosis must also rule out secondary disease processes, such as chronic arterial occlusive or connective tissue disease.

Treatment

Initially, treatment consists of avoidance of cold, 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 is reserved for unusually severe symptoms. Such therapy may include phenoxybenzamine or reserpine; low doses of nifedipine have been shown to be effective. Sympathectomy may be helpful when conservative modalities fail to prevent ischemic ulcers and becomes necessary in less than 25% of patients.

Special considerations

❑ Warn the patient against exposure to the cold. Tell him to wear mittens or gloves in cold weather or when handling cold items or defrosting the freezer.

❑ Advise the patient to avoid stressful situations and to stop smoking.

❑ Instruct the patient to inspect the skin frequently and to seek immediate care for signs of skin breakdown or infection.

❑ Teach the patient about drugs, their use, and their adverse effects.

❑ Provide psychological support and reassurance to allay the patient’s fear of amputation and disfigurement.

Copyright Details: Professional Guide to Diseases (Eighth Edition), Copyright © 2005 Lippincott Williams & Wilkins.

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More About This Book: Title: Professional Guide to Diseases (Eighth Edition) Authors: Springhouse Publisher: Lippincott Williams & Wilkins Copyright: 2005 ISBN: 1-58255-370-X