Basics
Description
- Painless, symmetric, distal bluish discoloration usually affecting hands and feet (1)
- Affected areas can be cool.
- Does not come and go like Raynaud phenomenon (RP)
- RP has a broad differential but is distinct from acrocyanosis.
Epidemiology
Incidence
Unknown, uncommon
Prevalence
- Affects younger women
- Perhaps more common in colder climates
Etiology and Pathophysiology
Unknown, several theories
- Vasospasm of arteries and arterioles with secondary dilatation of capillaries and veins, leading to cyanosis
Genetics
Unknown
Risk Factors
None specific for acrocyanosis
General Prevention
- Keep hands and feet warm as able.
- Some patients wear two sets of socks and will wear fingerless gloves at 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: alpha agonists (cocaine, amphetamines), ergotamines, nicotine, some chemotherapeutic agents, others
- Hematologic causes: cold agglutinins, polycythemia, paraproteinemias, cryoglobulins, cryofibrinogens
- Trauma and vibration: pneumatic tools, damage to distal arteries
- Environmental: chilblains (pernio), frostbite
Diagnosis
History
- Painless blue fingers, hands, toes, or feet
- Digits may often feel cool.
- Symptoms worse with cooler temperatures
- Onset 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)
- 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)
- None necessary if the clinical diagnosis is sure. All tests are typically normal.
- If concerned about connective tissue diseases, complete blood count (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
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, for example, coarctation of aorta).
Treatment
General Measures
- Keep extremities warm.
- Avoid inciting situations (outdoor weather without warm clothes, sitting under the AC at work, etc.).
- Patient education and reassurance is quite helpful.
Medication
First Line
- Generally not indicated (1,2,3)[C]
- Avoidance of vasoactive medications
- Scant evidence suggests medications that block peripheral catecholamines (i.e., beta-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
- The vast majority of patients do well.
- Progression to digital or limb ulceration does not happen in acrocyanosis. If so, reconsider diagnosis to include vasculitis.
Patient Monitoring
- None required
- Patients may engage in normal activity and employment. They will naturally 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
References
1.Kurklinsky AK, Miller VM, Rooke TW. Acrocyanosis: the Flying Dutchman. Vasc Med. 2011;16(4):288.
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2.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; 2004:475-498.3.Nousari H, Kimyai-Asadi A, Anhalt G. Chronic idiopathic acrocyanosis. J Am Acad Dermatol. 2001;45(Suppl 6):S207-S208.
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Codes
ICD09
- 443.89 Other specified peripheral vascular diseases
ICD10
- I73.89 Other specified peripheral vascular diseases
SNOMED
- 25003006 acrocyanosis (finding)
Clinical Pearls
- Acrocyanosis is persistent; RP is intermittent.
- Acrocyanosis does not respond to calcium channel blockers (1); RP does.
- Acrocyanosis is generally benign.