Raynaud phenomenon in Child
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Synopsis
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.
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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Differential Diagnosis & Pitfalls
Structural vasculopathies:- Thoracic outlet syndrome
- Brachiocephalic trunk disease (atherosclerosis, Takayasu arteritis)
- Buerger disease (thromboangiitis obliterans)
- Crutch pressure
- Systemic sclerosis
- 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
- Cryoglobulinemia (monoclonal or mixed)
- Cryofibrinogenemia
- Cold agglutinin disease
- Myeloproliferative disorders
- Drug-induced Raynaud (ergot alkaloids, bromocriptine, interferon, estrogen, cyclosporine, sympathomimetic agents, clonidine, cocaine, nicotine)
- Carpal tunnel syndrome
- Pheochromocytoma
- Carcinoid syndrome
- Reflex sympathetic dystrophy
- 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
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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.Subscription Required
References
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Last Reviewed:11/13/2017
Last Updated:06/20/2022
Last Updated:06/20/2022
Patient Information for Raynaud phenomenon in Child
Overview
Raynaud's phenomenon is a disorder in which the blood vessels to the fingers and toes (digits) become abnormally closed off (constricted). The fingers and toes of individuals with Raynaud's phenomenon change color from white to blue to red, often causing them to feel numb. Raynaud's phenomenon is sometimes seen with autoimmune diseases, such as rheumatoid arthritis, scleroderma, and lupus, where the body's immune system turns against itself, causing various symptoms. Other causes of Raynaud's phenomenon include repeated trauma/vibration, abnormalities in the structure of blood vessels, and drug injection into one type of blood vessel (arteries).Who’s At Risk
Raynaud's phenomenon usually affects younger to middle-aged women. When it affects men, it is usually seen in middle age or later in life. Raynaud's phenomenon is seen in 20% of people with lupus.Signs & Symptoms
- Raynaud's phenomenon affects the fingers and toes (digits). The digits feel cool and their color changes from white to blue to red.
- Severe Raynaud's phenomenon can result in skin ulcers. This complication is more common in people who have both scleroderma and Raynaud's phenomenon.
- People with Raynaud's phenomenon may have more noticeable small blood vessels (capillaries) into their fingers and toes where the nail plate meets the skin of the finger (proximal nail fold), particularly in people who also have an autoimmune disease.
Self-Care Guidelines
Avoid exposure to cold as much as possible, and do not smoke, as this contributes to blood vessel narrowing.When to Seek Medical Care
See your doctor if you notice symptoms suspicious for Raynaud's phenomenon. Further testing may be needed to see if you might have another medical condition that is causing your symptoms.Treatments
- Oral medications, including nifedipine (a blood pressure medication), epoprostenol (a prostaglandin), or a drug that affects the nerves (selective serotonin reuptake inhibitor), such as fluoxetine.
- Topical nitroglycerin paste, which helps to dilate blood vessels, may be used as needed to treat or prevent active Raynaud's phenomenon. Apply the paste carefully as it can lead to low blood pressure (hypotension) if used on large areas.
- Biofeedback, which is a treatment technique in which people are trained to improve their health by using signals from their own bodies, is another therapy for Raynaud's phenomenon.
References
Bolognia, Jean L., ed. Dermatology, pp.262, 626. New York: Mosby, 2003.
Freedberg, Irwin M., ed. Fitzpatrick's Dermatology in General Medicine. 6th ed. pp.1031, 1326, 1637. New York: McGraw-Hill, 2003.
Raynaud phenomenon in Child
See also in: Nail and Distal Digit