Management of New Born with Harlequin Ichthyosis: A Rare Disease Condition

Abstract

Harlequin ichthyosis is the most severe form of non-bullous ichthyosis, which is unusual in newborns and is usually marked by deadly excessive keratinization of the skin. Ichthyosis is a family of genetic skin disorders, characterized by dry, thickened, scaly skin with severe morbidity and mortality. The term “harlequin” derives from the facial appearance and the triangular and diamond-shaped pattern of the scaly skin. It occurs in about 1 in 300,000 births and has no known sex predilection. The disorder affects the skin in utero causing thick, horny, armory-like plates that cover the skin with contraction abnormalities of the eyes, ears, mouth and appendages. There is no specific guideline to manage the newborn of harlequin ichthyosis, in that nursing care is most important in the initial phase. There are required multi-disciplinary teams to take care of harlequin ichthyosis.