Case #2: Raynaud disease
The woman had the classic signs and symptoms of Raynaud phenomenon. In the absence of a related systemic disease, this condition is termed Raynaud disease, or primary Raynaud phenomenon.
In 1862, French physician Maurice Raynaud first described the phenomenon that now bears his name as an exaggerated vasospastic response to cold temperatures, resulting in transient digital ischemia. These episodes occur in approximately 3% of the population.1 Affected patients report a sequence of three distinct phases when exposed to cold temperatures. First, the fingers become painful and pale. This pallor represents the initial effects of arterial and arteriolar constriction. The areas of pallor tend to be well-demarcated and readily apparent. Next, the fingers turn blue as the continued vasoconstriction leads to cyanosis. Finally, a reactive hyperemia results once the digital arteries and arterioles dilate. This phase manifests clinically as redness, paresthesias, and digital edema. The diagnosis of Raynaud phenomenon is made clinically and requires the presence of all three phases.
Once the diagnosis is made, the patient must be evaluated for evidence of an associated systemic disease. Approximately 20% of patients with Raynaud phenomenon will ultimately be found to have a concomitant disease. Causes of so-called secondary Raynaud phenomenon include connective tissue diseases, such as systemic lupus erythematosus, dermatomyositis, Sjögren syndrome, rheumatoid arthritis, and scleroderma; hypothyroidism; and thoracic outlet syndrome. Secondary Raynaud phenomenon is also a well-known component of the CREST syndrome in which patients develop Calcinosis cutis, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasias.
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An appropriate workup for the patient with newly diagnosed Raynaud phenomenon includes a thorough history, complete physical examination, and selected laboratory analysis. A history of arthralgias, dyspnea, digital ulcers, or rash suggests underlying connective tissue disease. Careful inspection of the nailfolds may reveal dilated or engorged capillary beds, also suggestive of connective tissue disease. Initial laboratory tests should include erythrocyte sedimentation rate and antinuclear antibody, rheumatoid factor, serum complement, and thyroid-stimulating hormone determinations. The diagnosis is primary Raynaud phenomenon if the following criteria are met: vasospastic episodes are symmetric and involve both hands, there is no digital necrosis, nailfold capillaries exhibit no abnormalities, serology results are within normal limits, and there is no historical or physical evidence of underlying disease.1
Avoidance of cold temperatures is the mainstay of therapy for patients with Raynaud disease. If this approach fails or is unacceptable, calcium channel blockers, such as nifedipine, may be initiated and have been shown to reduce the frequency of vasospastic events by up to 66%.1
Our patient had no evidence of underlying systemic disease, and the results of her laboratory tests were normal. Although she experienced frequent vasospastic attacks during the winter months, she continued to decline medical therapy. She was hopeful that the warmer spring weather would bring wildflowers to the Rocky Mountains and relief to her cold blue fingers.
Dr. Vandergriff is a dermatology resident at The University of Texas Southwestern Medical Center in Dallas,and Dr. Neyman is a resident in the Department of Dermatology at Emory University in Atlanta. Dr. Dwinnell is associate professor of medicine at the University of Colorado Health Science Center in Denver. He directs resident training at Presbyterian-St. Luke’s Medical Center, where the woman presented as a patient during Dr. Vandergriff’s and Dr. Neyman’s internship. The authors have no relationships to disclose relating to the content of this article.
Reference
1. Wigley FM. Clinical practice. Raynaud’s phenomenon. N Engl J Med. 2002;347:1001-1008.
