Harlequin Ichthyosis (HI) is a rare and severe genetic disorder that affects the skin of infants. This condition prevents the formation of a protective layer of the skin; instead, it produces a scale-like and hard layer in the skin.
The ears may be very poorly developed or absent entirely, as may the nose. The eyelids are severely everted and the lips, pulled by the dry skin, are fixed into a wide grimace. There can also be the development of diamond-shaped plates that are separated by deep ridges in the skin.