newborn infant with vesiculobullous skin lesions

Incontinentia Pigmenti Syndrome [IPS] is a rare hyperpigmentary disorder with an X-Iinked dominant inheritance. It is characterized by four phases, vesicular, verucous, pigmentary, and hypopigmentary stages that often is associated with ocular, dental and central nervous system abnormalities. We describe an eleven days old girl with multiple erythematous vesiculoboltous skin lesions were found at birth over distal part of her limbs and trunk. The family history for IPS was negative. The cause of incontinentia pigmenti has been traced to a defective gene on the X chromosome called NEMO, but genetic heterogeneity may exist. IPS may also arise as a spontaneous mutation