Introduction:
Harlequin ichthyosis (HI) is the most dangerous form of the autosomal
recessive congenital ichthyosis characterized with thickening of keratin
part of the baby’s skin and a gross thick scaly appearance, which is
triangular or diamond pattern.1 The name takes origin
from its characteristic facial appearance as the face is pulled wide
open in manner of a clowns smile. Marked ectropion and eclabium with
absent or poorly developed ears and nose, mobility limitation of joints
are some of the clinical features of HI.1 As the skin
barrier is severally compromised there is excessive water loss and
electrolyte abnormalities followed by temperature dysregulation and
increase risk of infections. Because of these reason HI is usually fatal
albeit aggressive management.