- 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.
- 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)
- Low BMI
- Outdoor exposure in cold climates
- 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
- 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”)
- Cyanosis of affected areas, usually distal hands and feet
- Bilateral involvement
- Peripheral pulses are strong.
- No trophic changes (no loss of hair) nor ulcerations
- 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)
- 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
- 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).
- 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.
Issues For Referral
- Only in cases where the diagnosis is uncertain
- Consider dermatology, rheumatology, or vascular surgery as appropriate.
- 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.
- None required
- Patients may engage in normal activity and employment.
- Patients should modify their dress to keep distal extremities warm.
- Nothing specific
- If patients notice worsening with sympathomimetics (caffeine, decongestants), adjust accordingly.
Reassurance is the best policy.
Excellent; does not progress or worsen; often resolves in middle age
- I73.89 Other specified peripheral vascular diseases
- 443.89 Other specified peripheral vascular diseases
- 25003006 acrocyanosis (finding)
- Acrocyanosis is generally benign.
- Acrocyanosis is persistent; RP is intermittent.
- Make sure secondary etiology is ruled out.
Faraz Ghoddusi, MD
Stacey A. Ghoddusi, MPH
Gabriel T. Harris, MD, FAAFP
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