Wiler, Jennifer L. MD, MBA
Dr. Wiler is an assistant professor of emergency medicine and the vice chair of clinical quality, patient safety, and process improvement at the University of Colorado Denver School of Medicine and an adjunct assistant professor of emergency medicine at the Washington University School of Medicine in St. Louis.
A 31-year-old woman complains of white toes after skiing. She has no known medical problems. She denies palpitations, chest pain, fever, shortness of breath, and claudication symptoms.
What is the diagnosis? See next page.
Diagnosis: Raynaud's Phenomenon
Raynaud's phenomenon is a vasospastic condition that results in distal extremity discoloration, and when severe, gangrene secondary to impaired vascular perfusion. Named after Maurice Raynaud, a French physician, this condition is typically induced by emotional stress or a cold local environment. The body, when exposed to cold, attempts to conserve heat by shunting blood from the extremities to the core, resulting in a relative decrease in distal microperfusion (thermoregulation response). Patients with Raynaud's phenomenon are thought to have a hyperactive physiologic response to cold.
Raynaud's can be either primary (idiopathic or hereditary) or secondary. Primary Raynaud's is thought to be related to a hyper alpha-2 adrenergic response in the cutaneous vessels. (Curr Opin Rheumatol 2005;17:752.) The etiology of secondary Raynaud's phenomenon includes autoimmune conditions including scleroderma, systemic lupus erythematosus and rheumatoid arthritis. Raynaud's phenomenon is one of the clinical features associated with the limited cutaneous form of systemic scleroderma (CREST syndrome: calcinosis, Raynaud's, esophageal dysmotility, sclerodactyly, and telangiectasia).
The exact incidence of Raynaud's phenomenon is unknown but reported to be as high as 20 percent in women and 14 percent in men. (J Rheumatol 1993;20:70.) It has also been noted to occur in children. (Arthritis Rheum 2003;48:3518.)
Risk factors for Raynaud's phenomenon include history of smoking, family history, personal history of cardiovascular disease, older age, a low body mass index, use of vibrating tools, personal history of rheumatoid arthritis or hypothyroidism, smoking, and daily environmental exposures. The role of climate and environmental exposure appears to be multifactorial and complex in predisposing development of Raynaud's phenomenon. (J Rheumatol 1997;24:879.)
Patients with Raynaud's phenomenon typically develop symptoms in the hands or toes. Symptoms may include sharply demarcated white (ischemic attack), blue, or purplish (cyanotic attack) distal digits, and typically last for 20 minutes. Patients may complain of pain, paresthesias, and limb clumsiness. The skin becomes hyperemic and slightly swollen upon rewarming and reperfusion. Severe cases of Raynaud's phenomenon can result in critical ischemia resulting in skin ulcerations or dry gangrene. Patients may also present with livedo reticularis, a lacy, mottled blue/purple skin discoloration of the limb.
The diagnosis of Raynaud's phenomenon is typically clinical, but some unique diagnostic tools, including measurement of finger systolic pressure response to cold (Scand J Clin Lab Invest 1978;38:765) and capillaroscopy (Rheum Dis Clin North Am 2008;34:89), have been described in small studies. Efforts should be made to identify secondary causes of the condition. Most cases are associated with autoimmune vasculitides, so the diagnosis will rarely be definitively made in the ED.
The differential diagnosis of Raynaud's phenomenon includes thromboembolic disease, arterial disease, vasculitis and connective tissue disorders, atherosclerosis, infection, neuropathy, complex regional pain syndrome (formerly reflex sympathetic dystrophy), drug-induced vasospasm (methamphetamine, ephedra), frostbite and chilblains, and hyperviscosity syndromes.
The treatment of Raynaud's phenomenon depends on the etiology and severity of symptoms. Patients should be counseled to stop smoking, reduce or eliminate exposures to sympathomimetic drugs (decongestants), and limit caffeine exposure. Initial management includes cold avoidance. Calcium channel blockers, topical nitrates, and phosphodiesterase 5 inhibitors (sildenafil) may be used if initial supportive care is ineffective and inhibits quality of life or function.
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