Harlequin Color Change in Babies
Harlequin color change in babies requires no treatment—it is a benign, self-limited phenomenon that resolves spontaneously within minutes to hours and should be recognized to avoid unnecessary interventions. 1, 2
What This Condition Is
Harlequin color change (also called harlequin phenomenon) presents as a sudden, well-demarcated color change where half of the baby's body displays erythema (redness) while the other half shows pallor (paleness). 1, 2 This distinctive cutaneous phenomenon:
- Most commonly occurs in healthy newborns during the first days of life 2
- Typically resolves spontaneously within 20 minutes of appearance 2
- Results from relative hypothalamic immaturity affecting sympathetic control of peripheral vascular tone 1
- May be influenced by gravity, often appearing on the dependent side when the infant is lying laterally 2
Clinical Management Approach
Observation only is recommended—no diagnostic workup or treatment is needed for typical presentations in healthy neonates. 1, 2
When to Reassure Parents
The phenomenon is benign when:
- The infant is otherwise healthy and vitally stable 2
- Episodes are transient (resolving within minutes) 2
- The baby is in the neonatal period (first 28 days) 1
- No other concerning neurological signs are present 3
When to Investigate Further
Beyond the neonatal period, vigorously pursue an underlying cause, as harlequin color change may indicate sympathetic dysautonomia or central nervous system pathology. 1, 3
Search for underlying conditions if:
- The phenomenon persists beyond infancy 1
- Associated neurological symptoms are present (altered consciousness, seizures, focal deficits) 3
- Episodes are prolonged or recurrent beyond the neonatal period 3
- Consider meningitis, hypothalamic lesions, brain stem lesions, or sympathetic nervous system disorders 3
Important Clinical Pitfalls
Do not discontinue prostaglandin E1 therapy if harlequin color change occurs in neonates receiving this medication for congenital heart disease. 4 The color change may be dose-related but is benign and self-limited, and stopping the medication is not warranted. 4
Avoid ordering unnecessary investigations in typical neonatal cases—recognition of this phenomenon prevents irrelevant testing and interventions. 2 The condition requires no laboratory work, imaging, or treatment in healthy newborns. 1, 2
Variations in Presentation
The phenomenon can present in different patterns:
- Hemifacial (affecting only the face) 3
- Hemiscrotal (affecting genital area) 3
- Patchy scattered across the whole body 3
- Classic hemibody distribution 2
All variants share the same benign prognosis in healthy neonates. 3
Medications That May Enhance Expression
Certain drugs can enhance harlequin color change through their influence on capillary tone:
This drug-related enhancement is promptly reversible with medication withdrawal but does not require discontinuation unless medically indicated for other reasons. 3, 4
Note: This answer addresses harlequin color change, a benign vascular phenomenon. This is distinct from harlequin ichthyosis, a severe genetic skin disorder requiring intensive neonatal care in a specialized unit with multidisciplinary management. 5