Differential Diagnosis

This disorder refers to a recurrent response to cold stimuli in which the digits of the hands progress from turning white and numb to cyanotic and eventually to red (pallor, cyanosis, then erythema). Symptoms may progress to include gangrene and ulceration in severe cases. The anatomy of the vascular system is such that the peripheral parts of the body (fingers, knees, soles, toes, etc.) are densely populated with capillaries and arteriovenous shunts. When exposed to cold stimuli, these shunts close and blood flow is mostly prevented from entering the cutaneous vasculature, thus conserving body heat. When this response to cold stimuli is excessive, afflicted persons suffer from Raynauds phenomenon. Arterial closure manifests as the initial pallor. This is followed by cyanosis, which reflects pooling of deoxygenated blood during vasospasm, and the symptoms eventually progress to erythema as a result of vasodilatation with excessive reflow of blood to the area. This disorder usually occurs in a bilateral and symmetrical pattern. Familial clustering is common, and women are affected much more commonly than men.

Primary Raynauds phenomenon occurs in the absence of any underlying disease process. Young women are most commonly afflicted with symptoms beginning just after menarche or anytime after the second decade of life. As described, the episodes are symmetric and involve the hands and feet. Even though one digit may be more severely affected than the others, this disorder usually includes all of the fingers from the tip to the metacarpophalangeal joint. Severe disease characterized by necrosis and tissue damage is uncommon with primary Raynauds although it may occur.

Secondary Raynauds phenomenon occurs in the presence of an underlying disease process or secondary to certain medications. Rheumatologic diseases are frequently associated with this disorder. In patients afflicted with scleroderma, approximately 90% suffer from Raynauds phenomenon. Less commonly, patients afflicted with systemic lupus erythematosus (10-40%), dermatomyositis (30%), and Sjogrens syndrome (20%) suffer from secondary Raynauds phenomenon. Secondary Raynauds phenomenon may also occur in persons with normal vasculature who suffer with a hyperviscous state (polycythemia vera, essential thrombocytosis, macroglobulinemia, cryoglobulinemia). Criteria that indicate secondary Raynauds phenomenon as the diagnosis are: late age of the initial attack, the ingestion of medications known to be associated with Raynauds phenomenon, severe painful attacks, the presence of skin necrosis and ulceration, or if the patient manifests symptoms and signs of an underlying disease that is known to be associated with Raynauds phenomenon.

The physical exam of patients afflicted with this disorder should include a careful examination of the nail-fold capillaries. A drop of oil should be placed on the skin at the base of the fingernail and then the area should be inspected using either a wide-field microscope or an ophthalmoscope. If dilated, enlarged capillary loops are noted, secondary Raynauds phenomenon should be suspected. The changes in the nail bed capillaries occur early in the course of the underlying disease.

Therapy for Raynauds phenomenon ally includes avoidance of initiating factors (cold stimuli, stress, etc.). As primary disease is usually not as severe and is generally not associated with gangrene or ulceration, pharmacologic therapy is usually not warranted. When severe attacks occur, the calcium channel blockers nifedipine or diltiazem are effective. The application of topical 2% nitroglycerin ointment may also be beneficial.