Harlequin discoloration P83.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Harlequin color change; Harlequin phenomenon; Harlequin Syndrome

History
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Neligan & Strang, 1952

Definition
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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.

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

Etiopathogenesis
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The pathogenesis is unknown. It is assumed to be a dysregulation of the still immature cutaneous vascular plexus (often depending on the position!). Probably, triggering by hypoxia, e.g. in the context of cynotic heart defects (e.g. pulmonary atresia; transposition of the large vessels). Possible connections with the application of prostaglandin E1 are also described sporadically.

Manifestation
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In about 10% of mature newborns, between the 3rd-5th day of life until the end of the first month of life. The harmless disorder occurs more frequently in immature babies.

Localization
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trunk, face, genital region

Clinical features
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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.

Diagnosis
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Clinical picture; reversibility by changing position.

Therapy
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Not required.

Progression/forecast
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Completely harmless and reversible.

Note(s)
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The effect is perceived as dramatic by the parents of the child and leads to emergency medical attention!

Case report(s)
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  • Case 1: Timely born girl with transposition of the large vessels: To keep the ductus arteriosus open continuous prostaglandin E1 infusions. On the 10th day of life development of circumscribed erythema on the face, neck and occasionally on one side of the trunk. No dependence of the efflorescences on position, no change in general condition. Spontaneous subsidence after several hours. The intravenous administration of Fenistil was unsuccessful. On the 11th day of life switch-operation of the large vessels and end of prostaglandin infusions. Afterwards no recurrence of skin changes.
  • Case 2: Girl born on schedule with pulmonary artery atresia: On the 3rd day of life balloon dilatation of the valve. Among others prostaglandin E1 infusions in the medication. On the 11th day of life development of spotty erythema on the face, neck and occasionally on one side of the trunk. The intravenous administration of Fenistil was unsuccessful, spontaneous subsidence of erythema after about 30 minutes. Recurrences in decreasing intensity during the following 8 days. No recurrence of erythema after discontinuation of prostaglandin.

Literature
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  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

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Last updated on: 29.10.2020