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Raynaud phenomenon in Child
See also in: Nail and Distal Digit
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Raynaud phenomenon in Child

See also in: Nail and Distal Digit
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Contributors: Elyse M. Love MD, Susan Burgin MD
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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 is divided into primary and secondary with varied clinical courses.

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 nailfold 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 torturous nailfold 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.

Raynaud phenomenon is considered to be rare in childhood. Raynaud phenomenon that presents in childhood should be closely worked up to exclude underlying causes. Patients should be followed closely as Raynaud phenomenon can precede other symptoms of connective tissue disease by years.

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:
Normal blood vessels with abnormal blood elements:
Normal blood vessels with abnormal vasomotion:
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

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: 11/14/2017
Last Updated: 11/14/2017
Copyright © 2019 VisualDx®. All rights reserved.
Raynaud phenomenon in Child
See also in: Nail and Distal Digit
Print 6 Images
View all Images (6)
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Raynaud phenomenon : All fingernails or distal fingers, Bilateral, Blue color, Cold exposure, Cyanosis, White color
Clinical image of Raynaud phenomenon
Copyright © 2019 VisualDx®. All rights reserved.