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Diagnosis

Raynaud Phenomenon

Filippone, Lisa M. MD

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    This patient has Raynaud phenomenon. Notice the second picture displayed here. All the fingers are back to normal. Can you appreciate that during the symptomatic episode, there is a clear line of demarcation of the involved digit? While classically there are three stages of color change (pallor, cyanosis, and hyperemia), many patients may present with only two of the three, usually pallor and hyperemia.

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    Figure
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    Also different fingers may be at different stages of perfusion. In the first image, the right index finger appears cyanotic while the right middle and left index and middle fingers are pale. While many patients present to the ED complaining of cold fingers or toes, patients with Raynaud phenomenon have several distinguishing features. First, this is a reversible phenomenon usually incited by a predictable stimulus. Secondly, there must be two distinct well demarcated color changes.

    Maurice Raynaud is credited with the first well described case of the reversible condition characterized by the triphasic color change observed in the digits back in 1862. In the past, Raynaud disease referred to the reversible episodic vasospasm of peripheral arterioles in response to various stimuli in patients with no underlying disorder. The term Raynaud syndrome was applied when an underlying condition, often a rheumatologic disorder, was present. Today the terms primary Raynaud phenomenon and secondary Raynaud phenomenon are preferred to Raynaud disease and Raynaud syndrome, respectively.

    Proper vasomotor homeostasis depends on complex interactions between the vascular endothelium, the vascular smooth muscle, and the autonomic and sensory nerves that innervate the vessel. A multitude of abnormalities can result in loss of proper vascular integrity. In primary Raynaud phenomenon, it is believed there is some trigger that results in vasospasm of the small muscular arteries resulting in ischemia to the area, often a digit. The digit appears pale and then cyanotic. As reperfusion ensues, the area becomes hyperemic. In primary Raynaud phenomenon, symptoms are often mild and tissue damage is uncommon. Fingers are most commonly involved, but toes, ears, noses, and even nipples can be involved.

    While the most common rheumatologic disease associated with secondary Raynaud phenomenon is systemic sclerosis (scleroderma), others include systemic lupus erythematosus, rheumatoid arthritis, Sjögren syndrome, and dermatomyositis. Secondary Raynaud phenomenon may be seen as a result of environmental injury. Frostbite can result in a disruption in proper vasomotor tone. People who work with jackhammers are susceptible to vibration injury of arterioles, referred to as hand-arm vibration syndrome. Chemical and heavy metal exposures (lead, arsenic) may be associated with secondary Raynaud. Neoplastic syndromes in which blood viscosity is increased may result in Raynaud phenomenon. Classic examples are leukemia and myeloma. As compared with primary Raynaud, the symptoms in secondary Raynaud are often more severe, and tissue damage/ulcerations are not uncommon.

    In the ED, a goal-directed history and physical exam aimed at identifying possible causes of secondary Raynaud phenomenon should be performed. Patients who complain of fatigue, easy bruising, and bone pain may have leukemia. Patients with a malar rash and arthritis may have lupus. Environmental past and present exposures should be reviewed. In general no specific testing is needed in the primary ED evaluation unless there is suspicion of an underlying illness. Proper outpatient referral to a rheumatologist and reassurance is all that is required. If the inciting stimulus is identified, behavior modification can be implemented. Cold is the most common trigger. Instructing patients to avoid holding cold objects and to wear well insulated gloves during the winter may be all that is required. Medications that contribute to peripheral vessel vasoconstriction (beta-blockers, ergot alkaloids, decongestants) may be discontinued. Smoking cessation should be encouraged.

    If the above measures are not helpful or if symptoms are more severe, pharmacotherapy may be initiated. The most commonly used agents are calcium channel blockers. Nifedipine and amlodipine, which are dihydropyridines, are preferred because they are selective for vascular smooth muscle without affecting cardiac function. Other agents such as angiotension-converting enzyme antagonists, prostaglandins, and topical nitrates have been used with varying success. If ulcerations develop, local wound care should be initiated with particular attention to the development of bacterial infection. In severe cases, referral to a surgeon may be needed for cervical or digital sympathectomy.

    © 2004 Lippincott Williams & Wilkins, Inc.