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

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Ontology: Erythromelalgia (C0014804)

Definition (NCI) A rare disorder characterized by periodic inflammation and blockage of the vessels of the extremities, resulting in skin redness, swelling, and burning pain in the affected sites. It may manifest as a primary disorder caused by mutations of the SCN9A gene or as a secondary disorder due to hematologic disorders or medication side effects.
Definition (MSH) A peripheral arterial disease that is characterized by the triad of ERYTHEMA, burning PAIN, and increased SKIN TEMPERATURE of the extremities (or red, painful extremities). Erythromelalgia may be classified as primary or idiopathic, familial or non-familial. Secondary erythromelalgia is associated with other diseases, the most common being MYELOPROLIFERATIVE DISORDERS.
Concepts Disease or Syndrome (T047)
MSH D004916
ICD9 443.82
ICD10 I73.81
SnomedCT 238777005, 37151006
English Erythermalgia, Erythermalgias, Erythromelalgia, Erythromelalgias, ERYTHROMELALGIA, erythromelalgia (red burning extremities) (physical finding), erythromelalgia (red burning extremities), erythromelalgia (diagnosis), erythromelalgia, Erythromelalgia [Disease/Finding], weir mitchell's disease, mitchell disease, gerhardt disease, Erythralgia, Weir Mitchell's disease, Erythromelalgia (disorder), Mitchell, erythermalgia, erythralgia
French ERYTHROMELALGIE, Erythralgie, Maladie de Weir-Mitchell, Erythromélalgie, Érythromélalgie, Érythermalgie
Portuguese ERITROMELALGIA, Eritralgia, Eritromelalgia
Spanish ERITROMELALGIA, Eritralgia, enfermedad de Weir Mitchell, eritermalgia, eritralgia, eritromelalgia (trastorno), eritromelalgia, Eritromelalgia, Eritermalgia
German ERYTHROMELALGIE, Erythralgie, Erythermalgie, Erythromelalgie
Dutch erytralgie, erytromelalgia, Erythermalgie, Erytromelalgie
Italian Eritralgia, Eritermalgia, Eritromelalgia
Japanese 紅痛症, コウツウショウ, ヒフコウツウショウ, 肢端紅痛症, 先端紅痛症, 皮膚紅痛症
Swedish Erytromelalgi
Czech erytromelalgie, Erytromelalgie, Erytralgie
Finnish Erytromelalgia
Russian ERITROMELAL'GIIA, ЭРИТРОМЕЛАЛЬГИЯ
Polish Erytromelalgia
Hungarian Erythromelalgia, Erythralgia
Norwegian Erytromelalgi