II. Pathophysiology

  1. Episodic bilateral vasodilatation with hyperemia

III. Causes

  1. Primary (Idiopathic) form occurs in Young adult males
  2. Secondary form due to chronic myeloproliferative disorder (esp. over age 40 years old)
    1. Polycythemia Rubra Vera
    2. Primary Thrombocythemia

IV. Symptoms

  1. Hand and feet symptoms
    1. Burning pain
    2. Prickling pain
    3. Erythema
    4. Cyanosis
    5. Edema or congestion
  2. Provocative factors
    1. Heat exposure
    2. Exercise
    3. Standing
  3. Palliative factors
    1. Elevating or cooling extremities
      1. Patient walks in snow or cold floors
      2. Sleeps with feet outside bed covers

V. Signs

  1. Normal peripheral pulses
    1. Critical to exclude ischemia
  2. Provocative maneuvers reproduce pain
    1. Lower the limb
    2. Expose limb to heat

VII. Labs: Skin biopsy

  1. Arteriolar inflammation
  2. Fibromuscular intima proliferation
  3. Microvascular thrombi

VIII. Management

  1. Evaluate for underlying disorder (esp. serious conditions)
    1. Peripheral Arterial Disease (Claudication or Critical Limb Ischemia)
    2. Myeloproliferative disorders (Polycythemia Vera, Thrombocythemia)
  2. Symptomatic measures
    1. Avoid provocative activities (e.g. heat)
    2. Aspirin 650 mg per day

IX. References

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