Harlequin fetus is the most
severe form of congenital ichthyosis with an incidence of one in
300.000 births. An autosomal recessive pattern of inheritance is
seen in this disorder. However; a new autosomal dominant mutation
may possibly be responsible. The disorder has an ominous prognosis
since the neonates usually die in the first hours or days of life.
We reported here; a case of harlequin fetus with a history of
psoriasis in his fath
Figure 1: The patient showing
ectropion, eclabium and thickened skin with deep
fissures
Figure 2: The patient with
small testes and rudimentary scrotum
Figure 3: Edematous hands and
clenched in a flexed position
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