Harlequin discoloration P83.8

Seizure-like, one-sided, sharply defined, often position-dependent reddening of the skin in newborns, especially premature babies, lasting from a few seconds to about 30 minutes on the 2nd to 4th day of life as a result of vasomotor immaturity. S.a.u. Erythema neonatorum. A sharp midline border of the erythema can be particularly noticeable.

Incidence is reported to be 10% in immature newborns. Due to the volatility of the eythema, the phenomenon is probably underdiagnosed (Höger P 2018).

The pathogenesis is unclear. A dysregulation of the immature cutaneous vascular plexus is assumed (often position-dependent!).

Probably a triggering by hypoxia takes place, e.g. in the context of cynotic heart defects (e.g. pulmonary atresia; transposition of the great vessels). Possible connections with the application of prostaglandin E1 are also described in isolated cases.

Characteristically, there are strictly hemiplegic flat erythema with sharp midline demarcation on the trunk, face and genital region. The erythema can also affect symmetrically individual body segments. They are often gravity-dependent (flat redness at the bottom; normal skin at the top) and persist from a few seconds to about 30 minutes. The harlequin colour change can occur in healthy newborns as well as in those with other diseases.

Livedo reticularis: Completely harmless, not unilateral

Cutis marmorata teleangiectatica congenita: Complex immaturity syndrome of skin and subcutis. No one-sidedness; no color change on rearrangement.

Case 1: Term-born girl with transposition of the great vessels: continuous prostaglandin E1 infusions to keep the ductus arteriosus open. On the 10th day of life development of circumscribed erythema on the face, neck and occasionally half-sided on the trunk. The erythema did not depend on the position of the patient and there was no change in the general condition. Spontaneous resolution after several hours. Intravenous administration of Fenistil was unsuccessful. On the 11th day of life switch operation of the great vessels and end of prostaglandin infusions. No recurrence of skin lesions thereafter.

Case 2: Term born girl with pulmonary artery atresia: On the 3rd day of life balloon dilatation of the valve. Among other things prostaglandin E1 infusions in the medication. On the 11th day of life, development of patchy erythema on the face, neck, and isolated hemifacial erythema on the trunk. Intravenous administration of Fenistil was unsuccessful, spontaneous resolution of erythema after about 30 minutes. Recurrences in decreasing intensity during the following 8 days. After discontinuation of the prostaglandin no recurrence of the erythema.

1. Januário G et al (2011) The Harlequin phenomenon. J Eur Acad Dermatol Venereol 25:1381-1384
2. Höger P (2018) Neonatal Dermatology. In: G. Plewig et al. (ed.), Braun-Falco`s Dermatology, Venerology and Allergology, Springer Reference Medicine. S. 1538
3. Neligan GA, strand LB (1952) A "harlequin" colour change in the newborn. Lancet 2: 1005-1007
4. Rao J, Campbell ME, Krol A (2004) The harlequin color change and association with prostaglandin E1. Pediatric Dermatol 21: 573-576
5. Rao J, Krol A (2003) Images in clinical medicine. The harlequin color change. N Engl J Med 349: 968