Raynaud phenomenon

Last updated: September 11, 2023

Summarytoggle arrow icon

Raynaud phenomenon (RP) is an exaggerated vasoconstrictive response of distal arteries and arterioles (most commonly in the fingers and toes) to cold or emotional stress. Primary RP is idiopathic, whereas secondary RP is caused by underlying systemic diseases (e.g., mixed connective tissue disease, vasculitides, medications). Both types typically manifest with the sequential discoloration of fingers and/or toes from white (ischemia) to purple-blue (hypoxia) to red (reactive hyperemia). Episodes of vasoconstriction usually last for 15–20 minutes after removal of the trigger. Ischemic episodes in secondary RP can be more prolonged, leading to tissue loss (ischemic ulcers) and rarely, gangrene. Trigger avoidance is the main aspect of managing RP. In patients with secondary RP, the underlying etiology should be identified and treated. Pharmacotherapy (preferably with calcium channel blockers) may be considered for RP refractory to trigger avoidance. Intravenous prostaglandins or interventional therapy (e.g., selective digital sympathectomy) may be required for patients with severe or refractory disease.

Etiologytoggle arrow icon

Overview [1][2][3]

  • Vasospastic attack triggered by cold or emotional stress
  • More common in female individuals

Primary Raynaud phenomenon (previously called Raynaud disease)

  • Idiopathic (no identifiable vascular changes); possible genetic susceptibility [1]
  • Vasospasms of the digital arteries and arterioles
  • Onset usually < 30 years of age

Secondary Raynaud phenomenon (previously called Raynaud syndrome)

Clinical featurestoggle arrow icon

Classic presentation [1][4][5]

An episode does not always involve all three phases; often, blood flow is restored without causing reactive hyperemia. [1]

Duration [1][5]

  • Vasospastic episodes are usually reversible.
  • Symptoms normally subside within an hour (typically 15–20 minutes after cessation of trigger exposure). [1]
  • Can potentially last for several hours

Associated findings [1][5]

Trophic changes are uncommon in primary RP. Irreversible ischemia with tissue damage indicates secondary RP and requires further investigation to identify the underlying cause. [1]

Diagnosticstoggle arrow icon

General principles [1][4][6][7]

Diagnostic studies [1][6][8]

Findings are described in the table below.

Differentiation between primary and secondary RP

Primary vs. secondary Raynaud phenomenon [4][6]
Primary RP Secondary RP

Characteristic features

  • Onset < 30 years of age
  • Episodes entirely reversible
  • Thumb is typically spared
  • No tissue loss
  • No signs of CTD

Laboratory studies

Nailfold capillaroscopy

  • Normal pattern
    • Uniform and regularly distributed
    • Hairpin capillary morphology
  • Abnormal (scleroderma pattern)
    • Decreased capillary density (i.e., dropout)
    • Dilated “giant” capillaries (diameter ≥ 50 μm)
    • Abnormal architecture
    • Hemorrhages

It is critical to evaluate for secondary RP because missing early CTD or other associated causes can have serious consequences.

Differential diagnosestoggle arrow icon

Perniosis (chilblains) [11][12][13]

  • Erythrocyanotic papules or nodules, predominantly on the hands, fingers, toes, legs, and face on exposure to cold
  • Lesions appear 12–24 hours after exposure to cold and last for 1–3 weeks.
  • Typically seasonal (colder months)
  • See “Nonfreezing cold injuries” for details.

Acrocyanosis [14][15]

  • No triphasic color response like in RP, nonparoxysmal manifestation (often persistent)
  • Recurrent or frequently persistent bluish/cyanotic discoloration of the peripheral extremities
  • Most commonly affects the hands; more rarely the feet, ears, nose, lips, and nipples
  • Caused by decreased oxyhemoglobin due to vasospasm
  • Usually triggered and/or aggravated by exposure to cold temperatures, most cases improve in summer.
  • Trophic changes and ulceration are extremely rare.
  • May be primary (idiopathic) or secondary to neurologic, autoimmune, infective, metabolic, or other causes

Erythromelalgia [16][17]

  • Rare condition characterized by episodes of hyperperfusion that affect the feet and less commonly the hands
  • Symptoms include intense burning pain, erythema, warmth, and swelling of the affected areas.
  • Triggers include warmth and physical activity.
  • May be primary (genetically determined) or secondary to myeloproliferative disorders, CTD, cancer, infections, or poisoning.

Peripheral artery disease (PAD)

  • Exercise-induced pain (may also be persistent)
  • Cold and pale extremities
  • Diminished or absent radial or ulnar artery pulse
  • Can be a cause of secondary RP (functional vasospasm), but must be considered as an independent differential diagnosis.

Acute arterial occlusion of an extremity

Thoracic outlet syndrome [9][18]

The differential diagnoses listed here are not exhaustive.

Treatmenttoggle arrow icon

Approach [1][4]

  • First line management in all patients: trigger avoidance and lifestyle measures to minimize vasospastic attacks
  • Persistent symptoms
    • Consider pharmacotherapy.
    • Consider interventional therapies in patients with refractory disease and surgical treatment when indicated.
  • Secondary RP
    • Identify and treat the underlying cause.
    • Specialist referral (e.g., immunology, rheumatology) for further management.

Trigger avoidance [1][4]

  • Avoidance of cold (e.g., wearing warm gloves or multiple layers of clothing)
  • Stress management (e.g., anxiety attacks, episodes of irritability/anger)
  • Minimize vibration exposure (e.g., occupational use of power hand tools such as jackhammers)
  • Smoking cessation (smoking promotes vasoconstriction)
  • Discontinuation of medications that may trigger attacks (e.g., β-blockers, ergotamine, oral contraceptives)

Cold avoidance and stress management are key aspects of managing RP. [4][7]

Pharmacotherapy [1][4][6][7]

There is a paucity of evidence on the efficacy of pharmacotherapy in RP and currently, no drugs have received FDA approval for treatment of the condition. [1][7][19][20]

Pharmacotherapy for RP often causes significant side effects (e.g., hypotension). Start all medications at a low dose and gradually increase as tolerated. [1]

Interventional therapies and surgery [1][4][6]

Referencestoggle arrow icon

  1. Belch J, Carlizza A, Carpentier PH, et al. ESVM guidelines – the diagnosis and management of Raynaud’s phenomenon. Vasa. 2017; 46 (6): p.413-423.doi: 10.1024/0301-1526/a000661 . | Open in Read by QxMD
  2. Kasper DL, Fauci AS, Hauser S, Longo D, Jameson LJ, Loscalzo J . Harrisons Principles of Internal Medicine . McGraw-Hill Medical Publishing Division ; 2016
  3. Pathophysiology and clinical consequences of Raynaud's phenomenon related to systemic sclerosis. Updated: October 1, 2006. Accessed: April 4, 2017.
  4. Wigley FM, Flavahan NA. Raynaud’s Phenomenon. N Engl J Med. 2016; 375 (6): p.556-565.doi: 10.1056/nejmra1507638 . | Open in Read by QxMD
  5. Maverakis E, Patel F, Kronenberg DG, et al. International consensus criteria for the diagnosis of Raynaud's phenomenon. J Autoimmun. 2014; 48-49: p.60-65.doi: 10.1016/j.jaut.2014.01.020 . | Open in Read by QxMD
  6. Haque A, Hughes M. Raynaud's phenomenon. Clin Med (Northfield Il). 2020; 20 (6): p.580-587.doi: 10.7861/clinmed.2020-0754 . | Open in Read by QxMD
  7. Gregory J. Landry. Current medical and surgical management of Raynaud's syndrome. Journal of Vascular Surgery. 2013; 57 (6): p.1710-1716.doi: 10.1016/j.jvs.2013.03.012 . | Open in Read by QxMD
  8. Herrick AL. Evidence-based management of Raynaud’s phenomenon. Ther Adv Musculoskelet Dis. 2017; 9 (12): p.317-329.doi: 10.1177/1759720x17740074 . | Open in Read by QxMD
  9. Jones MR, Prabhakar A, Viswanath O, et al. Thoracic Outlet Syndrome: A Comprehensive Review of Pathophysiology, Diagnosis, and Treatment. Pain and therapy. 2019; 8 (1): p.5-18.doi: 10.1007/s40122-019-0124-2 . | Open in Read by QxMD
  10. Fayyaz A, Igoe A, Kurien BT, et al. Haematological manifestations of lupus. Lupus Sci Med. 2015; 2 (1): p.e000078-e000078.doi: 10.1136/lupus-2014-000078 . | Open in Read by QxMD
  11. Vano-Galvan S, Martorell A. Chilblains. CMAJ. 2012; 184 (1): p.67.doi: 10.1503/cmaj.110100 . | Open in Read by QxMD
  12. Gordon R, Arikian AM, Pakula AS. Chilblains in Southern California: two case reports and a review of the literature. J Med Case Reports. 2014; 8: p.381.doi: 10.1186/1752-1947-8-381 . | Open in Read by QxMD
  13. Nyssen A, Benhadou F, Magnée M, André J, Koopmansch C, Wautrecht JC. Chilblains. Vasa. 2020; 49 (2): p.133-140.doi: 10.1024/0301-1526/a000838 . | Open in Read by QxMD
  14. Das S, Maiti A. Acrocyanosis: An overview. Indian J Dermatol. 2013; 58 (6): p.417-420.doi: 10.4103/0019-5154.119946 . | Open in Read by QxMD
  15. Wollina U, Koch A, Langner D, et al. Acrocyanosis - A Symptom with Many Facettes. Open Access Maced J Med Sci. 2018; 6 (1): p.208-212.doi: 10.3889/oamjms.2018.035 . | Open in Read by QxMD
  16. Klein-Weigel PF, Volz TS, Richter JG. Erythromelalgia. Vasa. 2018; 47 (2): p.91-97.doi: 10.1024/0301-1526/a000675 . | Open in Read by QxMD
  17. Tang Z, Chen Z, Tang B, Jiang H. Primary erythromelalgia: a review. Orphanet J Rare Dis. 2015; 10 (1).doi: 10.1186/s13023-015-0347-1 . | Open in Read by QxMD
  18. Urschel HC, Patel A. Thoracic outlet syndromes. Curr Treat Options Cardiovasc Med. 2003; 5 (2): p.163-168.doi: 10.1007/s11936-003-0024-x . | Open in Read by QxMD
  19. Stewart M, Morling JR. Oral vasodilators for primary Raynaud's phenomenon. Cochrane Database Syst Rev. 2012: p.CD006687.doi: 10.1002/14651858.CD006687.pub3 . | Open in Read by QxMD
  20. Holly Ennis, Michael Hughes, Marina E Anderson, Jack Wilkinson, Ariane L Herrick. Calcium channel blockers for primary Raynaud's phenomenon. Cochrane Database Syst Rev. 2016.doi: 10.1002/14651858.cd002069.pub5 . | Open in Read by QxMD
  21. Chung L, Shapiro L, Fiorentino D, et al. MQX-503, a novel formulation of nitroglycerin, improves the severity of Raynaud's phenomenon: a randomized, controlled trial. Arthritis Rheum. 2009; 60 (3): p.870-7.doi: 10.1002/art.24351 . | Open in Read by QxMD
  22. Dhaliwal K, Griffin MF, Salinas S, Howell K, Denton CP, Butler PEM. Optimisation of botulinum toxin type a treatment for the management of Raynaud’s phenomenon using a dorsal approach: a prospective case series. Clin Rheumatol. 2019; 38 (12): p.3669-3676.doi: 10.1007/s10067-019-04762-4 . | Open in Read by QxMD

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