Basics

Description

  • Painless, symmetric, distal bluish discoloration usually affecting hands and occasionally feet (1)
    • Peripheral parts: Ear, nose, and nipple can be affected.
    • Affected areas can be cool.
  • Often secondary to underlying cause (2)
  • Unlike Raynaud phenomenon (RP), acrocyanosis is continually persistent.
    • RP has a broad differential but is distinct from acrocyanosis and is differentiated by the waxing and waning of extremity discoloration.

Epidemiology

Incidence
Unknown, uncommon


Prevalence
  • Mostly affects persons <30 years old
  • Typically more common in colder climates, with increased outdoor exposure, and in people with lower BMIs

Etiology and Pathophysiology

Unknown, several theories

  • Vasospasm of arteries and arterioles with secondary dilatation of capillaries and veins, leading to cyanosis (2)

Genetics
Unknown

Risk Factors

  • Low BMI
  • Outdoor exposure in cold climates

General Prevention

  • Keep hands and feet warm.
  • Wear additional socks and gloves (fingerless gloves for work).

Commonly Associated Conditions

Associated conditions may occur with secondary RP and include the following:

  • Connective tissue disease (systemic lupus erythematosus [SLE], scleroderma, etc.)
  • Occlusive peripheral vascular disease. Buerger disease is an advanced case.
  • Drugs: SSRIs, TCAs, vasopressors, α-agonists (cocaine, amphetamines), ergotamines, nicotine, some chemotherapeutic agents
  • Hematologic causes: cold agglutinins, polycythemia, paraproteinemias, cryoglobulins, cryofibrinogens
  • Trauma and vibration: pneumatic tools, damage to distal arteries
  • Environmental exposure: chilblains (pernio), frostbite

Diagnosis

History

  • Painless blue fingers, hands, nose, lips, toes, or feet
  • Digits may often feel cool.
  • Symptoms worsen with cooler temperatures
  • Onset typically in 2nd or 3rd decade
  • Symptoms are long-standing but not generally progressive.
  • Can be associated with hyperhidrosis of hands (“sweaty palms”)

Physical Exam

  • Cyanosis of affected areas, usually distal hands and feet
  • Bilateral involvement
  • Peripheral pulses are strong.
  • No trophic changes (no loss of hair) nor ulcerations

Differential Diagnosis

  • RP
  • Staining of skin (Consider wiping the skin with an alcohol swab.)
  • Chilblains
  • Livedo reticularis
  • Peripheral neuropathy affecting vascular flow
  • Complex regional pain syndrome (previously reflex sympathetic dystrophy or causalgia)
  • Occlusive arterial disease (usually affects only one limb and is progressively painful)

Diagnostic Tests & Interpretation

Initial Tests (lab, imaging)
  • All tests are typically normal with acrocyanosis, but it is a diagnosis of exclusion and usually needs further evaluation to rule out secondary cause of acrocyanosis.
  • Capillary oximetry
  • Complete blood count (CBC)
  • If concerned about connective tissue diseases, CBC, antinuclear antibody (ANA), erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP) to screen
  • If concerned about hypercoagulable state: anticardiolipin antibodies, prothrombin gene mutation, proteins C and S, factor V Leiden, antithrombin III, and others
  • CMP and/or metabolic screening
  • CT, MRI, NCV, and so forth

Follow-Up Tests & Special Considerations
None

Diagnostic Procedures/Other
  • In general, none
  • Ankle–brachial index and duplex ultrasound if concerned about peripheral arterial disease (PAD)
  • Consider evaluation for asymmetric involvement (arteriography for abnormal anatomy; e.g., coarctation of aorta).

Treatment

General Measures

  • Keep extremities warm with insulated clothing.
  • Behavioral changes to avoid inciting situations: outdoor weather without warm clothes, sitting under the AC at work, smoking cessation, and so forth
  • α-Blockers or calcium channel blockers
  • Patient education and reassurance is quite helpful.

Medication

First Line
  • Generally not indicated (1,2)[C]
  • Avoid vasoactive medications.
  • Limited evidence suggests medications that block peripheral catecholamines (i.e., β-blockers) may be beneficial.

Issues For Referral

  • Only in cases where the diagnosis is uncertain
  • Consider dermatology, rheumatology, or vascular surgery as appropriate.

Ongoing Care

Follow-up Recommendations

  • Most patients do well.
  • Progression to digital or limb ulceration or pain does not typically occur with acrocyanosis. If so, reconsider diagnosis to include vasculitis syndromes.

Patient Monitoring
  • None required
  • Patients may engage in normal activity and employment.
  • Patients should modify their dress to keep distal extremities warm.

Diet

  • Nothing specific
  • If patients notice worsening with sympathomimetics (caffeine, decongestants), adjust accordingly.

Patient Education

Reassurance is the best policy.

Prognosis

Excellent; does not progress or worsen; often resolves in middle age

Additional Reading

  • Haimovici H, Mishima Y. Nonatherosclerotic diseases of small arteries. In: Ascher E, Hollier L, Strandness DE, et al, eds. Vascular Surgery. 5th ed. Malden, MA: Blackwell; 2004:475–498.  [PMID:11712060]

  • Codes

    ICD-10

    • I73.89 Other specified peripheral vascular diseases

    ICD-9

    • 443.89 Other specified peripheral vascular diseases

    SNOMED

    • 25003006 acrocyanosis (finding)

    Clinical Pearls

    • Acrocyanosis is generally benign.
    • Acrocyanosis is persistent; RP is intermittent.
    • Make sure secondary etiology is ruled out.

    Authors


    Faraz Ghoddusi, MD
    Stacey A. Ghoddusi, MPH
    Gabriel T. Harris, MD, FAAFP

    Bibliography

    1. Das S, Maiti A. Acrocyanosis: an overview. Indian J Dermatol. 2013;58(6):417–420.  [PMID:21427140]
    2. Nousari H, Kimyai-Asadi A, Anhalt G. Chronic idiopathic acrocyanosis. J Am Acad Dermatol. 2001;45(Suppl 6):S207–S208. [PMID:11712060]


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