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Pediatric Raynaud Phenomenon Clinical Presentation

  • Author: Suzanne C Li, MD, PhD; Chief Editor: Lawrence K Jung, MD  more...
Updated: Apr 22, 2014


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.[44]

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.[44, 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.[50] 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.[48]

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).[32] 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.[36, 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.[51]

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,[52] family history of Raynaud phenomenon, family history of connective tissue diseases, history of migraine,[24] 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.



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.[53] 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.[37]

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.[54] 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.[55] 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.[54]

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.[30] 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.[56] In systemic lupus erythematosus patients, the development of a sclerodermalike nailfold capillaroscopy pattern was associated with development of lung fibrosis and pulmonary artery hypertension.[55]



Cold exposure was the reported trigger in 90% of patients with primary Raynaud phenomenon and secondary Raynaud phenomenon; 3% of patients with primary Raynaud phenomenon and 10% of patients with secondary Raynaud phenomenon reported that stress triggered the episode.[6] Jones et al did not find any associated psychosocial or social variables, such as socioeconomic status, conduct problems, or peer problems.[26] In addition, 5% of pediatric patients with primary Raynaud phenomenon and none of the patients with secondary Raynaud phenomenon reported episodes precipitated by exercise, and 14% of patients with primary Raynaud phenomenon and 7% of patients with secondary Raynaud phenomenon said no trigger was known for some of episodes.[37]

Besides outdoor cold exposure, cold stresses can include taking items out of the freezer, sitting in air conditioning, and coming out of an outdoor swimming pool. Combined cold and wet exposure, as occurs in spring and fall seasons, can also precipitate Raynaud phenomenon episodes.[32]

Factors reported to exacerbate or to be associated with Raynaud phenomenon include the following:

Contributor Information and Disclosures

Suzanne C Li, MD, PhD Associate Professor, Department of Pediatrics, Rutgers New Jersey Medical School; Senior Attending, Department of Pediatrics, Joseph M Sanzari Children’s Hospital, Hackensack University Medical Center

Suzanne C Li, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American College of Rheumatology, Childhood Arthritis and Rheumatology Research Alliance

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

David D Sherry, MD Chief, Rheumatology Section, Director, Amplified Musculoskeletal Pain Program, The Children's Hospital of Philadelphia; Professor of Pediatrics, University of Pennsylvania School of Medicine

David D Sherry, MD is a member of the following medical societies: American College of Rheumatology, American Pain Society

Disclosure: Nothing to disclose.

Chief Editor

Lawrence K Jung, MD Chief, Division of Pediatric Rheumatology, Children's National Medical Center

Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, New York Academy of Sciences

Disclosure: Nothing to disclose.

Additional Contributors

James M Oleske, MD, MPH François-Xavier Bagnoud Professor of Pediatrics, Director, Division of Pulmonary, Allergy, Immunology and Infectious Diseases, Department of Pediatrics, Rutgers New Jersey Medical School; Professor, Department of Quantitative Methods, Rutgers New Jersey Medical School

James M Oleske, MD, MPH is a member of the following medical societies: Academy of Medicine of New Jersey, American Academy of Allergy Asthma and Immunology, American Academy of Hospice and Palliative Medicine, American Association of Public Health Physicians, American College of Preventive Medicine, American Pain Society, Infectious Diseases Society of America, Infectious Diseases Society of New Jersey, Medical Society of New Jersey, Pediatric Infectious Diseases Society, Arab Board of Family Medicine, American Academy of Pain Management, National Association of Pediatric Nurse Practitioners, Association of Clinical Researchers and Educators, American Academy of HIV Medicine, American Thoracic Society, American Academy of Pediatrics, American Public Health Association, American Society for Microbiology, Infectious Diseases Society of America, Pediatric Infectious Diseases Society

Disclosure: Nothing to disclose.

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Raynaud phenomenon showing demarcation of color difference.
Nine-year-old with Raynaud phenomenon. Notice discoloration of fingers.
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