Dermatology Book

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Erythralgia

Aka: Erythralgia, 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): 511-5

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 Weir-Mitchell disease, Erythermalgia, Erythermalgias, Erythromelalgia, Erythromelalgias, ERYTHERMALGIA, ERYTHROMELALGIA, GERHARDT DISEASE, MITCHELL DISEASE, WEIR MITCHELL SYNDROME, 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, Erythromélalgie, Érythromélalgie, Érythermalgie
Portuguese ERITROMELALGIA, Eritralgia, Eritromelalgia
Spanish ERITROMELALGIA, Eritralgia, Erythromelalgia, enfermedad de Weir Mitchell, eritermalgia, eritralgia, eritromelalgia (trastorno), eritromelalgia, Eritromelalgia, Eritermalgia
German ERYTHROMELALGIE, Erythralgie, Erythermalgie, Erythromelalgie
Dutch erytralgie, erythromelalgia, 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
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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