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Acrocyanosis
Other Resources UpToDate PubMed

Acrocyanosis

Contributors: Lisa Altieri MD, Lowell A. Goldsmith MD, MPH
Other Resources UpToDate PubMed

Synopsis

Acrocyanosis is persistent bluish discoloration of the extremities, worsened by cold temperatures. This condition is due to decreased deoxyhemoglobin in the peripheral vasculature that may be primary (ie, unknown etiology) or secondary (ie, due to underlying pathology).

Primary acrocyanosis is asymptomatic, affects digits symmetrically, and is not associated with increased morbidity or mortality. It is most often seen in patients in their 20s and 30s and completely resolves in many women after menopause. The cyanotic discoloration seen in primary acrocyanosis is due to vasospasm in the cutaneous arterioles. Cold climate, outdoor occupation, and low body mass index are risk factors for developing acrocyanosis.

Secondary acrocyanosis can be symmetrical or asymmetrical, and it is sometimes associated with pain and necrosis of the affected extremities. As opposed to primary acrocyanosis, secondary acrocyanosis results from an underlying systemic pathology. It has been associated with many etiologies, including connective tissue diseases, Buerger disease, stroke, myocardial infection, lung diseases causing hypoxia, eating disorders, hematologic disorders, neoplasms, drug exposures, genetic diseases, spinal cord injury, and infections.

Codes

ICD10CM:
I73.89 – Other specified peripheral vascular diseases

SNOMEDCT:
25003006 – Acrocyanosis

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Raynaud phenomenon – paroxysmal episodes of well-demarcated cutaneous pallor and cyanosis followed by erythema
  • Chilblains – rapid onset of itching, pain, tenderness after exposure to cold
  • Erythromelalgia – can coexist with acrocyanosis
  • Peripheral cyanosis resulting from hypoxemia or methemoglobinemia – look for cyanotic mucosae and low oxygen saturation
  • Peripheral arterial disease – would have decreased pulses
  • Ecchymosis – would have soft tissue swelling
  • Cryoglobulinemia
  • Purpura fulminans
  • Leukocytoclastic vasculitis

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Updated:12/09/2015
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Patient Information for Acrocyanosis
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Acrocyanosis
A medical illustration showing key findings of Acrocyanosis : Acral distribution, Cold extremities, Cyanosis
Clinical image of Acrocyanosis - imageId=367116. Click to open in gallery.  caption: 'A violaceous tinge to the fingers.'
A violaceous tinge to the fingers.
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