Updated: 10/17/2018

Raynaud Phenomenon

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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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