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ErythralgiaAka: Erythromelalgia, Erythremomelalgia

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  1. See Also
    1. Skin reactions to temperature
  2. Pathophysiology
    1. Episodic bilateral vasodilatation with hyperemia
  3. Causes
    1. Primary (Idiopathic) form occurs in Young adult males
    2. Secondary form occurs in over age 40 years
      1. Polycythemia Rubra Vera
      2. Chronic myeloproliferative disorder
      3. Primary Thrombocythemia
  4. Symptoms
    1. Hand and feet symptoms
      1. Burning pain
      2. Prickling pain
      3. Erythema
      4. Cyanosis
    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
  5. Signs
    1. Provocative maneuvers reproduce pain
      1. Lower the limb
      2. Expose limb to heat
  6. Differential Diagnosis
    1. Atherosclerosis with Claudication
    2. Frostbite
    3. Immersion foot (Trench foot)
    4. Peripheral neuritis
    5. Disseminated sclerosis
    6. Hemiplegia
    7. Chronic heavy metal Poisoning
    8. Gout
  7. Labs: Skin biopsy
    1. Arteriolar inflammation
    2. Fibromuscular intima proliferation
    3. Vessel thrombi
  8. Management
    1. Evaluate for underlying disorder
    2. Avoid heat
    3. Aspirin 650 mg per day
  9. References
    1. Alarcon-Segovia (1966) Arch Intern Med 117(4):511

Erythromelalgia (C0014804)

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.
ConceptsDisease or Syndrome (T047)
ICD9443.82
MSHD004916
EnglishErythermalgia, Erythermalgias, Erythralgia, Erythromelalgia, Erythromelalgias, GERHARDT DISEASE, MITCHELL DISEASE, WEIR MITCHELL SYNDROME, Weir Mitchell's disease
Spanishenfermedad de Weir Mitchell, eritermalgia, eritralgia, eritromelalgia
Parent Concepts[X]Other specified peripheral vascular diseases (C0029822), Peripheral Vascular Diseases (C0085096), Symptoms (C1457887), Vascular Diseases (C0042373), Erythema (C0041834), Disorder characterized by pain (C1300028), Duplicate concept (C1274013)
SourcesDXP, ICD9CM, LCH, MSH, NCI, NDFRT, QMR, SCTSPA, SNOMEDCT
Derived from the NIH UMLS (Unified Medical Language System)



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