Repair of cicatricial ectropion in a harlequin baby.

[harlequin ichthyosis]

The ichthyoses are a collection of scaling skin diseases or keratinizing skin disorders giving the appearance of "fish skin ," of which harlequin ichthyosis is the most severe form . It is characterized by profound thickening of the keratin skin layer , armorlike scales that cover the body , and contraction abnormalities of the eyes , ears , and mouth . We report a case of a 6 -week -old boy with harlequin ichthyosis and severe bilateral upper and lower eyelid cicatricial ectropion who underwent surgical repair with full-thickness postauricular skin autografts . To our knowledge , this is the youngest reported case and the only case of harlequin ichthyosis in which postauricular skin grafts were used .