Raynaud Phenomenon
Raynaud Phenomenon: Excerpt from In A Page: Pediatric Signs and Symptoms
Raynaud phenomenon is a periodic vasoconstrictor response to cold and physical or emotional stress. It was first described in 1862 by Raynaud, a French medical student. He attributed the color changes to exaggerated CNS response. Exact pathophysiology is still not understood, but several players, such as altered skin blood flow, platelet activation, locally secreted mediators of smooth muscle tone, and endothelial injury may be involved to various degrees depending on the form of Raynaud. The population prevalence is 5–10%.
Differential Diagnosis
- Raynaud phenomenon is triphasic
–Pallor (white): Decreased cutaneous blood flow
–Cyanosis (blue): Venous stasis
–Erythema (red): Reflex vasodilation
–Fingers are affected more than toes; earlobes, tip of nose, lips, and tongue may also be affected
–The presence of intense, painful episodes with ischemic skin lesions and clinical features (arthritis, rash) of a connective tissue disease suggests a secondary cause
–Idiopathic Raynaud is uncommon in children apart from familial benign type; however, if there are no clinical or laboratory signs in the 2 years after Raynaud develops, an underlying disease is unlikely
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Familial benign type
–Median age at onset is around 14 years
–Positive family history in a first-degree relative in 25%
–ESR is normal and ANA is negative
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Connective tissue disease
–Scleroderma: Almost 90% have Raynaud; it is the initial symptom in most cases
–May also be found in SLE and CREST syndrome
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Vascular diseases
–Takayasu arteritis
–Giant cell arteritis
–Arteriosclerosis
–Thromboangitis obliterans
–Thoracic outlet syndrome
-
Environmental causes
–Frostbite
–Polyvinyl chloride disease
–Vibration disease
–Hypothenar hammer syndrome
-
Neuropathy (e.g., carpal tunnel syndrome)
-
Primary endocrine abnormalities
–Hypothyroidism
–GH excess/acromegaly
-
Hormone-secreting tumors
–Pheochromocytoma
–Carcinoid syndrome
Workup and Diagnosis
- History
–Sensitivity to cold with color changes (white or blue)
–Medication and environmental/cold injury, toxins, digital ulcers, long-lasting wounds
–Signs and symptoms of a possible systemic disease (myalgias, arthralgias/arthritis, weakness, weight loss, fever, rash; respiratory, CV, and GI symptoms)
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Physical exam
–Vital signs (fever, tachypnea, tachycardia, absent pulses, asymmetric blood pressure)
–Skin exam for rashes and ulcers
–Nail fold capillary exam
–Arthritis
–Lymphadenopathy
–Organomegaly
-
Labs
–Indicated for patients with signs and symptoms
compatible with a systemic disease
–CBC with differential, ESR/CRP, chemistry, urinalysis
–ANA and disease-specific autoantibodies, C3, C4
–TFTs, serum protein electrophoresis, cryoglobulins
- Vascular studies
–Indicated for patients with history of asymmetric attacks, physical exam findings of absent pulses, blood pressure asymmetry, evidence of ischemia
–Doppler ultrasonography
–Plethysmography
–Angiography
Treatment
-
Avoidance of cold temperature exposure
–Wear warm hats, socks, and gloves
-
Stress management
–Biofeedback techniques
–Both temperature-related and other relaxation
techniques
-
Calcium-channel blockers (such as nifedipine)
–Clinical trials have shown significant benefit, thus they are widely used
-
Other agents (frequently used but not well studied) Prazosin, losartan, pentoxifylline, fluoxetine, nitroglycerine, hydralazine, papavarine
-
Promising experimental agents: Iloprost (prostacyclin analog); cilostazol and sildenafil (phosphodiesterase inhibitors); bosentan (endothelin-receptor inhibitor)
-
Sympathectomy: Limited benefit, should be reserved for patients with severe ischemia unresponsive to medical treatment
Book Source Details
- Book Title: In A Page: Pediatric Signs and Symptoms
- Author(s): Jonathan E. Teitelbaum, Kathleen O. Deantonis, Scott Kahan
- Year of Publication: 2007
- Copyright Details: In A Page: Pediatric Signs and Symptoms, Copyright © 2007 Lippincott Williams & Wilkins.
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: In A Page: Pediatric Signs and Symptoms
Authors: Jonathan E. Teitelbaum, Kathleen O. Deantonis, Scott Kahan
Publisher: Lippincott Williams & Wilkins
Copyright: 2007
ISBN: 1-4051-0427-9
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» Next page: Raynaud's Phenomenon (A Pocket Manual of Differential Diagnosis)
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