Erythralgia

Aka: Erythralgia, Erythromelalgia, Erythremomelalgia

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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

VI. Differential Diagnosis

  1. Peripheral Arterial Disease (Claudication or Critical Limb Ischemia)
  2. Venous Insufficiency
  3. Myeloproliferative disorders (Polycythemia Vera, Thrombocythemia)
  4. Raynaud Phenomenon
  5. Frostbite
  6. Immersion Foot (Trench Foot)
  7. Peripheral neuritis
  8. Disseminated sclerosis
  9. Hemiplegia
  10. Chronic Heavy Metal Poisoning
  11. HIV Infection
  12. Diabetes Mellitus
  13. Rheumatologic Conditions
    1. Gouty Arthritis
    2. Systemic Lupus Erythematosus (SLE)
    3. Rheumatoid Arthritis
  14. Medications
    1. Bromocriptine
    2. Isopropanol
    3. Calcium Channel Blockers (Nifedipine, Felodipine, and Nicardipine)

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

  1. Saliba (2015) Erythromelalgia, Emedicine, accessed 12/15/2015
    1. http://emedicine.medscape.com/article/200071-overview
  2. Alarcon-Segovia (1966) Arch Intern Med 117(4): 511-5 [PubMed]

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