Definition and epidemiology
Raynaud phenomenon (RP) is episodic, reversible digital vasospasm provoked by cold or emotional stress, producing the classic triphasic colour change — pallor (white, ischaemia), cyanosis (blue, deoxygenation), then rubor (red, reperfusion hyperaemia) — though not all three phases are required. It is common, affecting up to 15% of adolescents, with a female predominance and frequent onset around puberty. The central clinical task is to separate primary RP (idiopathic, benign, vasospastic) from secondary RP, where vasospasm is the herald of an underlying connective-tissue disease — most importantly systemic sclerosis — or another structural/vasculopathic cause.
Clinical features
Primary RP is symmetrical, involves the fingers (often sparing thumbs), is triggered reproducibly by cold, and leaves no tissue damage between episodes; examination, nailfolds, and serology are normal. Features pointing to secondary RP include onset before age ~12 or after adolescence with severe attacks, asymmetry, intense pain, digital pitting scars, ulcers, or gangrene, abnormal nailfold capillaries, and systemic symptoms (arthralgia, rash, dysphagia, sicca, weight loss). Toe and earlobe involvement and a positive family history of autoimmune disease add weight. Secondary causes in children are dominated by the scleroderma spectrum (JSSc, mixed connective-tissue disease, juvenile dermatomyositis, SLE) but also include drugs, hyperviscosity, and vibration/occupational injury.
Diagnosis
RP is a clinical diagnosis; the work is risk-stratification. Apply the LeRoy and Medsger criteria to classify: primary RP requires vasospastic episodes with normal nailfold capillaries, no digital tissue injury, negative (or low-titre) ANA, and a normal ESR; secondary RP is defined by the presence of abnormal capillaroscopy, SSc-specific autoantibodies, or evidence of an underlying disease. Nailfold capillaroscopy is the single most useful bedside test and the gold standard for distinguishing the two — giant capillaries, microhaemorrhages, and avascular/dropout areas define the “scleroderma pattern.” Autoantibody testing (ANA with reflex to anticentromere, anti–Scl-70, anti-RNP) further stratifies risk. The combination of an abnormal capillaroscopy and a specific autoantibody carries a high long-term risk of progression to systemic sclerosis, whereas a child with normal capillaroscopy and negative serology is very unlikely to develop secondary disease.
See the full criteria: Raynaud Phenomenon Algorithm
Red flags
- Abnormal nailfold capillaroscopy (giant capillaries, dropout) — strongest single predictor of evolving connective-tissue disease.
- Positive ANA / SSc-specific autoantibodies — anticentromere, anti–Scl-70, anti-RNP.
- Digital pitting scars, ulcers, or critical ischaemia/gangrene — secondary vasculopathy needing urgent vasodilation.
- Asymmetric, very painful, or below-age-typical onset — investigate for an underlying cause.
- Systemic features — arthralgia, rash, dysphagia, sicca, or weight loss.
Management overview
Primary RP is managed conservatively: cold avoidance, gloves/layered warming, smoking-environment avoidance, stress management, and withdrawal of vasoconstrictor drugs; most children need no pharmacotherapy. When attacks are frequent, severe, or secondary, calcium-channel blockers (dihydropyridines) are first-line vasodilators. Escalation for refractory disease or digital ulcers uses phosphodiesterase-5 inhibitors, topical nitrates, endothelin-receptor antagonists, and prostanoids for critical ischaemia. Children meeting criteria for secondary RP need referral to paediatric rheumatology, treatment of the underlying connective-tissue disease, and scheduled surveillance with serial capillaroscopy and serology to detect progression. Doses are intentionally omitted — verify against current guidance.
References
- LeRoy EC, Medsger TA Jr. Raynaud’s phenomenon: a proposal for classification. Clin Exp Rheumatol. 1992;10(5):485-488.
- Pope JE. Diagnosis and treatment of Raynaud’s phenomenon. Drugs. 2007;67(4):517-525.
- Cutolo M, et al. Nailfold capillaroscopy in connective tissue disease. Best Pract Res Clin Rheumatol. 2019.
- Nelson Textbook of Pediatrics, 21st ed.
Decision support for qualified clinicians only — verify against current primary guidelines and your clinical judgement.