Acrocyanosis
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
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
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
Authors
Faraz Ghoddusi, MD
Stacey A. Ghoddusi, MPH
Gabriel T. Harris, MD, FAAFP
Bibliography
- Das S, Maiti A. Acrocyanosis: an overview. Indian J Dermatol. 2013;58(6):417–420. [PMID:24249890]
- Nousari H, Kimyai-Asadi A, Anhalt G. Chronic idiopathic acrocyanosis. J Am Acad Dermatol. 2001;45(Suppl 6):S207–S208. [PMID:11712060]
© Wolters Kluwer Health Lippincott Williams & Wilkins
Citation
Domino, Frank J., et al., editors. "Acrocyanosis." 5-Minute Clinical Consult, 33rd ed., Wolters Kluwer, 2025. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816852/1.2/Acrocyanosis.
Acrocyanosis. In: Domino FJF, Baldor RAR, Golding JJ, et al, eds. 5-Minute Clinical Consult. Wolters Kluwer; 2025. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816852/1.2/Acrocyanosis. Accessed December 10, 2024.
Acrocyanosis. (2025). In Domino, F. J., Baldor, R. A., Golding, J., & Stephens, M. B. (Eds.), 5-Minute Clinical Consult (33rd ed.). Wolters Kluwer. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816852/1.2/Acrocyanosis
Acrocyanosis [Internet]. In: Domino FJF, Baldor RAR, Golding JJ, Stephens MBM, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2025. [cited 2024 December 10]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816852/1.2/Acrocyanosis.
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