Snapshot A 20-year-old woman presents to her primary care physician’s office for discolored fingers when in the cold. She reports that this is not painful, but the sight of her blue fingers bothers her. She reports that it becomes white first, blue, and finally red when she rewarms her hands. Although she has tried wearing gloves, she still reports having this discoloration. She has no significant past medical history. Her physician prescribes her nifedipine. Introduction Clinical definition discoloration in extremities due to an exaggerated physiologic response to cold temperatures or emotional stress classification Raynaud disease primary (idiopathic) Raynaud phenomenon is not associated with an underlying disease Raynaud syndrome secondary Raynaud phenomenon is associated with an underlying disease Epidemiology demographics Raynaud disease majority of patients with Raynaud phenomenon female > male most commonly < 30 years of age Raynaud syndrome most commonly > 30 years of age location fingers > toes risk factors family history autoimmune disease Etiology cold temperatures emotional stress drugs beta blockers Pathogenesis ↓ blood flow to skin due to exaggerated physiologic response to cold temperatures or emotional stress small vessel vasospasm with impaired vasodilation and increased vasoconstriction phases initially, extremities turn white, indicating ischemia then, extremities turn blue, indicating hypoxia and cyanosis finally, extremities turn pink with re-warming, indicating reperfusion Associated conditions systemic sclerosis or limited scleroderma systemic lupus erythematosus mixed connective tissue disease cryoglobulinemia thromboangiitis obliterans (Buerger disease) Presentation Symptoms attacks of triphasic color changes in extremities with cold temperatures or emotional stress white, blue, then pink or red white and blue phase may be associated with a feeling of tightness or pain in severe disease Physical exam may not present with all 3 phases usually in fingers or toes, but can also affect nose, cheeks, or ears may have faint peripheral pulses may have digital ischemia, but this is uncommon ulcers gangrene Studies Labs only indicated if an underlying disease is suspected Making the diagnosis most cases are clinically diagnosed can use cold stimulation test to trigger an attack digits rewarm in > 20 minutes if the patient has Raynaud phenomenon Differential Thromboangiitis obliterans (Buerger disease) distinguishing factor a vasculitis that results in digital necrosis and ischemia Frostebite distinguishing factors the disease does not resolve with rewarming when thawed, patients may have bullae with clear or hemorrhagic fluid Treatment Conservative avoid cold temperatures indication for all patients modalities use of mittens or gloves smoking cessation indication for all patients Medical dihydropyridine-type calcium channel blockers indication for patients refractory to conservative methods drugs nifedipine, nimodipine, nicardipine, or amlodipine dihydropyridines act on vascular smooth muscle phosphodiesterase inhibitors indication for patients refractory to calcium channel blockers drugs sildenafil, vardenafil, or tadalafil Complications Digital ulceration more common with secondary Raynaud syndrome
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