Raynaud Phenomenon

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

Complications