Incontinentia Pigmenti. Presented by Dr. David V. Omens, and (by invitation) Dr. Harold D. Omens and Dr. R. Bernstein.
The patient, a 2-year-old Negro girl, was found to have numerous vesicular and bullous lesions at birth. They cleared with wet dressings, and now there remains on the body dark chocolate-brown pigmented macular lesions which assume many different irregular forms and gyrations; some are guttate, some streaked, and others form irregular whorls. They have no particular distribution. Examination of the teeth shows pegging. Skull, teeth, and bone surveys are negative. Family history reveals that a sister has the same condition and was shown before the Society two months ago. There are many twins, but only those not twins have been affected with this condition.Microscopic section shows moderate hyperkeratosis. The basal layer varied in pigment content, in some areas being lightly pigmented while other areas were heavily pigmented. The underlying