Raynaud Phenomenon Clinical Presentation

Updated: Aug 04, 2022
  • Author: Heather Hansen-Dispenza, MD; Chief Editor: Herbert S Diamond, MD  more...
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Patients with Raynaud phenomenon report vasospastic episodes provoked by cold temperatures or emotional stress. Episodes usually affect the fingers and toes but may rarely affect the nose, ears, nipples, or lips.

Numbness and pain in the affected area or areas may be present. Affected areas also show at least two color changes: white (pallor), blue (cyanosis), and red (hyperemia). The color changes are usually in the order noted, but not always. These changes are usually reversible but may, in severe cases, lead to local ischemia and ulceration.

Any history of associated symptoms should raise suspicion of an underlying disorder. A history of other vasospastic symptoms such as migraines may be useful.

Obtain a history of injury or frostbite, as this may leave the involved limb vulnerable to vasospasm. Also obtain an occupational history, with consideration of the following:

  • Secondary Raynaud phenomenon has been associated with the frequent use of vibrating tools such as jackhammers and sanders [29, 30] ; see the image below

    Photo of a patient with Raynaud phenomenon that re Photo of a patient with Raynaud phenomenon that resulted from working with a jackhammer. Courtesy of the CDC.
  • Industrial exposure to polyvinyl chloride has been implicated

  • Raynaud phenomenon may be associated with laboratory work, with exposure to organic solvents such as xylene, toluene, acetone, or chlorinated solvents [31]

Autoimmune disorders associated with secondary Raynaud phenomenon include the following:

Infectious syndromes associated with secondary Raynaud phenomenon include the following:

  • Hepatitis B and C infections (especially associated with mixed or type 3 cryoglobulinemia)
  • Mycoplasma infections (with cold agglutinins)

Neoplastic syndromes associated with secondary Raynaud phenomenon include the following:

  • Lymphoma
  • Leukemia
  • Myeloma
  • Waldenström macroglobulinemia
  • Polycythemia
  • Monoclonal or type 1 cryoglobulinemia
  • Other paraneoplastic disorders

Environmental associations with Raynaud phenomenon include the following:

  • Vibration injury
  • Vinyl chloride exposure
  • Frostbite
  • Lead exposure
  • Arsenic exposure
  • Organic solvent exposure (eg, xylene, toluene, acetone, chlorinated solvents) [31]

Metabolic/endocrine syndromes associated with secondary Raynaud phenomenon include the following:

Hematologic syndromes associated with secondary Raynaud phenomenon include the following:

Drugs associated with Raynaud phenomenon include the following:

  • Oral contraceptives
  • Ergot alkaloids
  • Bromocriptine
  • Beta-adrenergic blocking drugs
  • Antineoplastics (eg, vinca alkaloids, bleomycin, cisplatin)
  • Cyclosporine
  • Interferon alfa
  • Methylphenidate and other central nervous system stimulants [32, 22]
  • Pembrolizumab [21]

Physical Examination

Carefully examine the digits if either primary or secondary Raynaud is suspected. Observe for sclerodactyly, calcinosis, or digital ulcers.

Nailfold capillaroscopy—examination of nailfold capillaries under magnification—can help with the diagnosis of secondary Raynaud phenomenon by identifying the scleroderma pattern characteristic of systemic sclerosis. [33] In these cases, the normally regular pattern of capillary loops is replaced with abnormally large loops, alternating with areas without any capillaries. [34] Other features of the scleroderma pattern are giant capillaries (apical diameter > 50 μm) and sometimes capillary hemorrhages. The gold standard device for performing nailfold capillaroscopy is the digital videocapillaroscope, which combines a microscope with a digital video camera, but a variety of devices may be used, including a widefield microscope, an ophthalmoscope, or even a smartphone camera. [33]

A cold challenge test can trigger Raynaud phenomenon in the office setting. However, it is not usually needed for making the diagnosis.

A sharp demarcation of the border between the affected and unaffected areas is required for diagnosis. However, many patients do not have classic triphasic color changes.

Evaluate any signs or symptoms of other syndromes associated with secondary Raynaud phenomenon, as follows:

  • Bone pain may suggest a paraneoplastic syndrome associated with a hyperviscosity syndrome
  • The presence of nephritis, malar erythema, and arthritis suggests systemic lupus erythematosus
  • Persistent cyanosis or necrotic distal tissue suggests an underlying disorder or permanent ischemia
  • Livedo reticularis suggests an autoimmune disorder or coagulation abnormality
  • Carpal tunnel syndrome has been associated with an increased frequency of Raynaud phenomenon