The Harlequin infant represents the most severe form of nonbullous ich
thyosis. Although the clinical features of infants with Harlequin icht
hyosis are generally similar, histological, ultrastructural, and bioch
emical analyses have not shown consistent findings. An unexpected case
of Harlequin ichihyosis in a female infant born at 35 wks gestation i
s presented. Light microscopic and ultrastructural investigations of s
kin biopsies are detailed. The presence of extracellular lipid materia
l in the stratum corneum has not been described in the previously repo
rted cases of Harlequin ichthyosis.