Case Presentation
Harlequin fetus or Harlequin is a very rare and most severe form of
congenital ichthyosis characterized by a thick, heavily keratinized and
scaly skin (1,2).
A 3.3kg term male neonate with Apgar scores of 8 and 9 at one and
5minutes respectively was delivered to a 25-year-old Eritrean woman by
caesarean section. The baby was covered with thick yellowish to whitish
scales, split by extensive some deep and others shallow fissures
extending to the dermis. The scale covered almost the entire body. There
was severe ectropion. There was scalp hair, the limbs were edematous and
inflexible digits due to taut skins (Figure 1A, B and C). The pregnancy
was uneventful. Both parents were paternal first-degree cousins. The
baby was admitted to neonatal intensive care. In the first day, the baby
was suckling well and was well hydrated. On the second day, the scales
started to change in color (Figure 1D) and neonatal sepsis ensued. He
was intravenous antibiotic, but he progressively deteriorated. The
parents demanded, they wanted to take their baby home and they were
discharged eventually against medical advice.