Isn’t it worrisome if a neonate’s tiny body gets
divided into two colors?
Even the imagination of a baby’s body turning half red and half pale triggers a series of worrisome thoughts. The good news is that a temporary change in color is not troublesome; it’s just a Harlequin phenomenon!
A male neonate who was born at 35 weeks of gestation had well-demarcated unilateral erythema of the body on the third day of birth. He had been antenatally diagnosed with Ebstein’s anomaly.
At birth, he had respiratory distress with low oxygen saturation, not responding to the initial measures; therefore, he was intubated. Infusions of alprostadil (prostaglandin E1) and dobutamine were initiated.
A computed tomographic scan showed pulmonary hypoplasia and bronchial obstruction secondary to cardiomegaly. Echocardiography had revealed right atrial dilatation, severe tricuspid dysplasia, and pulmonary atresia.
On the third day, during the episode of unilateral erythema of the body, the vitals, and the physical examination were normal.
A diagnosis of Harlequin color change was made.
Harlequin phenomenon is a rare, benign, and transient change in the color of one side of the body or body part, displaying erythema on one side and pallor on the other side. The change in color is only short-lived, persisting for 30 seconds to a maximum of 20 minutes. This change in color usually occurs between two to five days of age. A few cases have been reported to occur as late as three weeks of birth.
Harlequin color change (HCC) is only seen in 10% of the healthy neonates.
The exact mechanism behind HCC remains unknown. However, some theories have suggested the role of sympathetic autonomic dysfunction. Anesthetic agents, alprostadil, prematurity, hypoxia, and low birth weight have been reported to be associated with HCC.
The transient, positional color demarcation with erythematous dependant side can be vertical with a sharp line of demarcation, as seen in the patient in the discussion here. However, the color demarcation can be horizontal or even patchy. When its patchy, even then, the margins are sharply defined.
HCC is an entirely harmless and benign phenomenon. It is not associated with permanent long term complications; therefore, no treatment is necessary. However, it is imperative to counsel the parents and the family members as this change in color, though transient, may be worrisome for the naïve individuals.
Gert van den Berg, M. P. (2020, January 30). Harlequin Color Change in a Neonate. Retrieved from The New England Journal of Medicine: https://www.nejm.org/doi/full/10.1056/NEJMicm1907713
Velayuthan S, Sankararaman S. Visual diagnosis: newborn who has unilateral color change. Diagnosis: Harlequin color change. Pediatr Rev. 2013;34(7):e25–e26
Valerio, E., Barlotta, A., Lorenzon, E., Antonazzo, L., & Cutrone, M. (2015). Harlequin Color Change: Neonatal Case Series and Brief Literature Review. AJP reports, 5(1), e73–e76. https://doi.org/10.1055/s-0035-1545671
Tang, J., Bergman, J., & Lam, J. M. (2010). Harlequin color change: unilateral erythema in a newborn. CMAJ: Canadian Medical Association journal = journal de l’Association medicale canadienne,182(17), E801. https://doi.org/10.1503/cmaj.092038