Harlequin fetus

The harlequin fetus has a severe ichthyosis that is usually fatal in the first few weeks of life.
The name is derived from the thick diamond shaped plates of ichthyotic skin present over most of the body surface.
It is inherited as an autosomal recessive.
There appears to be at least three different keratin disorders that can result in a harlequin fetus.
These infants have ectropion, eclabium, and deformed ears.

Treatment:

• Clinical improvement can be induced by oral retinoids, but the prognosis remains grave.
• Harlequin fetus can be diagnosed prenatally by fetal skin biopsy.

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