Pediatric Raynaud Phenomenon Clinical Presentation

Updated: Jun 23, 2022
  • Author: Suzanne C Li, MD, PhD; Chief Editor: Lawrence K Jung, MD  more...
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Presentation

History

How Raynaud phenomenon (RP) should be diagnosed is somewhat controversial. Several studies have reported that most patients with Raynaud phenomenon do not have the classic triphasic color pattern. Nigrovic et al reported that only 24% of children with primary Raynaud phenomenon and 19% of children with secondary Raynaud phenomenon had the classic 3 color changes. [6] Maricq et al reported that only 1% of adults who were cold sensitive had triphasic color changes, 37% had white or blue color only, and 6% had 2 color changes. [53]

Diagnosis requires a sharp demarcation of the border between the area affected by Raynaud phenomenon and the unaffected area, and most groups accept having only white or blue color changes with an appropriate history. [53, 18, 2, 9] However, the UK Scleroderma Study Group defined definite Raynaud phenomenon as the presence of 2 color changes in response to cold, and possible Raynaud phenomenon as the presence of uniphasic color change accompanied by numbness or paresthesia. [66] Color charts may aid in the diagnosis; patients are asked to compare their affected digits with those shown in the color chart, not all of which represent Raynaud phenomenon. [57]

Patient history should include affected sites, frequency and severity of attacks, duration of attacks, color pattern, triggers, seasonality, and associated symptoms (ie, numbness, paresthesia, pain). [29] The most commonly affected sites are the fingers, toes, ears, nose, and, rarely, nipples. [1, 18] The triggers of Raynaud phenomenon in children are similar to those described in adults. Cold and emotional stresses are the most common triggers; primary Raynaud phenomenon can also be triggered by exercise. [59, 6] Episodes are more common in the winter than in the summer, and serious ischemia is also more common in the winter. [5]

Secondary Raynaud phenomenon episodes are typically more intense, painful, asymmetric, frequent, and more likely to lead to digital ulcers and scars. [4] Thumb involvement is more suggestive of secondary Raynaud phenomenon than primary Raynaud phenomenon. [67]

Patients and their parents should be queried about changes in digits such as pits, ulcers, or poor healing, and about the presence of infection in affected digits. They should also be asked about possible associated or precipitating factors including frostbite, drug or toxin exposure, infection, vibration injury, [36] family history of Raynaud phenomenon, family history of connective tissue diseases, history of migraine, [25] weight loss or eating disorders, and cardiovascular diseases.

Patients should also be questioned for any history suggestive of connective tissue disease such as fever, weight loss, fatigue, rash (malar, vasculitic, dermatomyositis), morning stiffness, arthralgia, myalgia, dysphagia, peripheral edema, lymphadenopathy, or oral sores.

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Physical Examination

A general physical examination should be performed to evaluate for signs suggestive of rheumatic disease or other conditions associated with secondary Raynaud phenomenon. Careful examination of the nailfold capillaries, digit tips, and extremities should be performed to look for signs associated with severe Raynaud phenomenon or signs associated with an increased likelihood of developing secondary Raynaud phenomenon. Pediatric patients with primary Raynaud phenomenon generally have normal nailfold capillary findings. [32]  Nigrovic et al reported that 13% of pediatric patients with primary Raynaud phenomenon had nailfold changes compared with 54% of patients with secondary Raynaud phenomenon. [28]

Digit tips should be examined for pits, ulcers, and healing problems. Other signs potentially associated with rheumatic diseases include livedo reticularis, rash (malar, vasculitic, dermatomyositis), arthritis, skin edema or tightening, abnormal peripheral pulses, weakness, and oral ulcers.

Nailfold capillaries can be examined with an ophthalmoscope set at 10-40 diopters or with an otoscope and a drop of grade B immersion oil or lubricating jelly (eg, Surgilube, K-Y Jelly) placed over the periungual area of each finger, [2]  or with a dermoscope, widefield microscope, or digital video capillaroscope. The examiner must manually zoom in until the nailfold capillaries are clearly visible.

A normal capillary pattern consists of thin, parallel vessels. The earliest feature of a scleroderma spectrum–associated nailfold capillary pattern is symmetrically enlarged giant capillaries. Later signs include microhemorrhages, loss of capillaries (decreased density to avascular areas), and neoangiogenesis. [68]  The presence of any of these features has been found to identify adult primary Raynaud phenomenon patients at high risk for developing a scleroderma spectrum condition and secondary Raynaud phenomenon. [69]  It has therefore been recommended that nailfold capillaroscopy be performed at least once a year in patients with primary Raynaud phenomenon to identify those at risk for evolving to secondary Raynaud phenomenon. [68]

A decrease in capillary density and the presence of giant loops surrounded by avascular areas were only seen in pediatric patients with secondary Raynaud phenomenon in a prospective study of 250 patients. [49]  A sclerodermalike nailfold capillaroscopy pattern was also found in many pediatric patients with dermatomyositis or mixed connective tissue disease, but rarely in those with juvenile idiopathic arthritis, systemic lupus erythematosus, or localized scleroderma. [70]  In systemic lupus erythematosus patients, the development of a sclerodermalike nailfold capillaroscopy pattern was associated with development of lung fibrosis and pulmonary artery hypertension. [69]

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