Harlequin ichthyosis: a report of two cases

ABSTRACT

Harlequin ichthyosis is one of the most severe congenital skin cornification disorders with an incidence rate of about 1 in 300,000 births and characteristic clinical features. We report two cases of harlequin ichthyosis [HI] or harlequin fetus [HF], which were products of consanguineous union. Clinical examination showed typical features of harlequin ichthyosis, with the presence of plaques with diamond-like configuration, external ear anomaly, severe ectropion and eclabium, and rigid semi flexion of limbs