Abstract
Incontinentia pigmenti in female monozygotic twins are reported. They were born following cesarean section at 34 weeks of gestation. Case 1: the first baby showed edematous erythema with blisters and crusts on arms, legs and trunk after birth. Ophtalmological examination revealed retinal hemorrage. Case 2: the second baby were more severe eruption than the first baby. Because of typical eruption and course they were diagnosed as incontinentia pigmenti. This is the fourth twin-case described in Japan.
