Raynaud’s Phenomenon in Children and Adolescents
What is Raynaud’s phenomenon (RP)?
Raynaud’s phenomenon is a condition in which the fingers (and less often, the toes) turn different colors (white, blue and red) because of exposure to certain factors such as cold or stress. Patients may have discomfort or a “pins and needles” sensation when the fingers change color.
What are the types of Raynaud’s phenomenon (RP) in children and adolescents ?
There are two types of RP (primary and secondary):
- Primary RP occurs in patients who do not have underlying autoimmune disease. It is a normal response of our bodies to temperature. In children, it usually affects females, starting in the teenage years or the early 20s. While symptoms can be uncomfortable, there is no danger of damage to the tissue.
- Secondary RP is “secondary” to (a result of) another condition. These conditions are numerous and can include several autoimmune conditions, most commonly scleroderma, lupus, inflammatory myositis, and Sjögren’s syndrome.
Symptoms and Causes
What causes Raynaud’s phenomenon (RP) in children and adolescents?
Raynaud’s phenomenon in children and adolescents is caused by overly sensitive blood vessels in the fingers and toes that are more affected by cold and stress. These blood vessels “spasm” and constrict (narrow), which causes a decrease in blood flow and the changes in color.
The lack of blood flow often results in a pale, or white, discoloration of the fingers or toes. The digits can turn blue because less oxygen is being supplied to them, and eventually turn red when blood suddenly rushes back into the finger(s) after the episode is over.
What are the symptoms of Raynaud’s phenomenon (RP) in children and adolescents?
In most patients, the fingers (or toes) suddenly become cold as the blood vessels narrow. The skin color changes noticeably and may become pale, or purple or blue.
Usually, an attack of RP begins in a single finger and then spreads to other fingers in both hands. The index, middle and ring fingers are most frequently involved, while the thumb is often not affected. An attack can cause discomfort, including a "pins and needles" feeling, aching, numbness, or clumsiness of the affected hand(s).
The feeling of true pain occurs more often in secondary RP and is caused by a prolonged loss of blood flow to the tissues. Blood vessels supplying the skin of the ears, nose, face, knees, and nipples can also be affected, and the skin in these areas may become pale or bluish after exposure to cold. Mottling (a bluish discoloration) of the skin of the arms and legs might also appear. Attacks affecting the toes are also common, although people tend to complain of these less often.
Symptoms of RP go away as the factor that caused them (cold or stress) is removed. When the person leaves the cold area and rewarms his or her body, normal blood flow resumes and the discoloration disappears after 15 to 20 minutes.
Diagnosis and Tests
How is Raynaud’s phenomenon (RP) in children and adolescents diagnosed?
RP in children and adolescents is diagnosed based on a history and physical examination of the patient. Patients with primary Raynaud’s usually have a normal examination and blood work; those who have secondary Raynaud’s usually have an abnormal exam and/or blood work.
The doctor may use a technique called nailfold capillaroscopy to look at the blood vessels below the fingernails to help differentiate primary from secondary Raynaud’s.
Management and Treatment
How is Raynaud’s phenomenon (RP) in children and adolescents treated?
RP in children and adolescents can be managed with lifestyle modifications and medications.
Lifestyle modifications involve keeping warm and avoiding the factors that can bring on RP. Most patients can control their symptoms with lifestyle modifications alone, which include the following:
- Keep your whole body warm--specifically, the core (midsection) of the body.
- Use hand warmers and mittens/gloves to help keep your fingers warm.
- Bundle up outdoors. When it's cold, put on a hat, scarf, socks and boots, and two layers of mittens or gloves before you go outside. Wear a coat with snug cuffs to go around your mittens or gloves to prevent cold air from reaching your hands.
- Use chemical hand warmers.
- Wear earmuffs and a face mask if the tip of your nose and your earlobes are sensitive to cold.
- Warm your car. Run your car heater for a few minutes before driving in cold weather.
- Take precautions indoors. Wear socks. When taking food out of the refrigerator or freezer, wear gloves, mittens or oven mitts. Some people find it helpful to wear mittens and socks to bed during winter.
- Because air conditioning can trigger attacks, set your air conditioner to a warmer temperature.
- Use insulated drinking glasses.
- Avoid trigger factors, including cigarette smoking, caffeinated drinks (coffee, tea, soda) and medications that can cause narrowing of blood vessels, such as decongestants that contain pseudoephedrine, migraine medications that contain ergotamine, and ADHD medication such as methylphenidate (Ritalin®).
- Control or limit emotional stress, which can cause blood vessels to narrow. Stress plus cold exposure is an especially potent trigger for RP.
There are several medications for treating RP. They act by dilating (widening) blood vessels to increase blood flow to the fingers and toes.
Blood pressure medications such as the calcium channel blockers amlodipine (Norvasc®, Twynsta®, Azor®, Prestalia®) and nifedipine (Adalat CC®, Procardia®, Adalat®), and angiotensin-receptor blockers are commonly used. For patients who have more severe symptoms or who have developed complications such as ulcers on the fingertips, other medications can be used, such as topical nitrate cream or sildenafil (Revatio®, Viagra®).
What are the complications of Raynaud’s phenomenon (RP) in children and adolescents?
Complications of RP in children and adolescents include the following:
- Tissue damage occurs when blood circulation to fingers or toes is restricted for a prolonged period. Although this is not common, this can happen to patients who have secondary Raynaud's.
- A completely blocked artery can lead to sores (skin ulcers) or dead tissue (gangrene), both of which can be difficult to treat.
- Rarely, extreme untreated cases might require removing the affected part of the body with surgery (amputation).