Long-term survival of a harlequin fetus.

Harlequin ichthyosis is a rare, severe form of congenital ichthyosis, which probably represents a heterogeneous group of disorders. Unfortunately very little is known regarding the pathogenesis, course, and prognosis of harlequin ichthyosis because most of these infants die within a few weeks of birth. A case of an 9-year-old girl that had been a harlequin fetus, with clinical and histologic features similar to nonbullous congenital ichthyosiform erythroderma, is described.