Harlequin Ichthyosis the Most Severe Form of the Congenital Ichthyosis; A Case Report Study

Abstract

Harlequin ichthyosis (HI) is a rare and the most severe form of the congenital ichthyosis with an autosomalrecessive inheritance. At birth, the HI phenotype is striking with thick hyperkeratotic plate-like scaleswith deep dermal fissures, severe ectropion, among other findings. Although HI infants have historicallysuccumbed in the perinatal period related to their profound epidermal compromise, the prognosis of HIinfants has vastly improved over the past 20 years. The disease might be lethal at birth and the affected babiesare often premature. The present study reports a new case with HI and adds to the collective knowledge ofthis rare skin disorder.