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Raynaud phenomenon in Adult
See also in: Nail and Distal Digit
Other Resources UpToDate PubMed

Raynaud phenomenon in Adult

See also in: Nail and Distal Digit
Contributors: Elyse M. Love MD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Raynaud phenomenon is a vascular disorder characterized by intermittent arteriolar vasospasm of the digits, most often due to cold temperature or stress. Symptoms present with characteristic changing of hands from white (pallor) to blue (cyanosis) to red (hyperemia), although all 3 phases may not be present in each individual. Raynaud phenomenon may be primary or secondary.

Primary Raynaud phenomenon typically follows a less severe course than secondary Raynaud phenomenon. In primary Raynaud phenomenon, also termed Raynaud disease, vasospasm is not associated with ischemic injury or an underlying associated disease. Primary Raynaud phenomenon typically presents at a younger age (< 30 years), involves all fingers symmetrically excluding the thumbs, and is not painful. Patients do not have a history of peripheral vascular disease or ischemic injury. Antinuclear antibody titers are normal or low (< 1:40 on indirect immunofluorescence) and nail fold capillaries are normal.

Secondary Raynaud phenomenon is associated with an underlying disease, usually a connective tissue disease of which systemic sclerosis is the most common. Because vasospasm is associated with concurrent ischemia, attacks are typically painful and asymmetric involvement of digits or hands is common. Digital ulcerations or necrosis, digital tuft pits, pterygium inversum, and tortuous nail fold capillaries with capillary dropout may be seen.

Additional associations include systemic lupus erythematosus, rheumatoid arthritis, pulmonary hypertension, frostbite, hematologic malignancies, polyvinyl chloride exposure, cryoglobulinemia, reflex sympathetic dystrophy, repeated trauma / vibration, arteriovenous fistula, intra-arterial drug administration, thoracic outlet syndrome, thromboangiitis obliterans, and Takayasu arteritis.

Codes

ICD10CM:
I73.00 – Raynaud's syndrome without gangrene

SNOMEDCT:
266261006 – Raynaud's Phenomenon

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Structural vasculopathies:
  • Thoracic outlet syndrome
  • Brachiocephalic trunk disease (atherosclerosis, Takayasu arteritis)
  • Buerger disease (thromboangiitis obliterans)
  • Crutch pressure
  • Scleroderma
  • Systemic lupus erythematosus
  • Dermatomyositis
  • Connective tissue overlap syndromes
  • Cold injury
  • Vibration disease (hand-arm vibration syndrome, Hypothenar hammer syndrome)
  • Chemotherapy (bleomycin, vinca alkaloids, cisplatin, carboplatin)
  • Vinyl chloride disease
  • Arsenic poisoning
Normal blood vessels with abnormal blood elements:
  • Cryoglobulinemia (monoclonal or mixed)
  • Cryofibrinogenemia
  • Cold agglutinin disease
  • Myeloproliferative disorders
Normal blood vessels with abnormal vasomotion:
  • Drug-induced Raynaud (ergot alkaloids, bromocriptine, interferon, estrogen, cyclosporine, sympathomimetic agents, clonidine, cocaine, nicotine)
  • Carpal tunnel syndrome
  • Pheochromocytoma
  • Carcinoid syndrome
  • Complex regional pain syndrome
Other:
  • Pernio – itching, burning, or painful lesions on the extremities after exposure to nonfreezing cold; lesions last for 1-3 weeks
  • Erythromelalgia – burning, pain, and warmth of extremities worsened by heat and improved by cold
  • Cold hypersensitivity – no skin color changes
  • Achenbach syndrome – paroxysmal bruising in the hand or fingers, of unknown etiology
  • Atrial myxoma

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed:11/13/2017
Last Updated:06/20/2022
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Raynaud phenomenon in Adult
See also in: Nail and Distal Digit
A medical illustration showing key findings of Raynaud phenomenon : All fingernails or distal fingers, Bilateral distribution, Blue color, Cold exposure, Cyanosis, White color
Clinical image of Raynaud phenomenon - imageId=239203. Click to open in gallery.  caption: 'Violaceous crusted plaques and papules on the toes.'
Violaceous crusted plaques and papules on the toes.
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