Incontinentia pigmenti (Bloch-Sulzberger syndrome) associated with acute granulocytic leukemia.

Incontinentia pigmenti, a syndrome of developmental defects, was found to be associated with acute granulocytic leukemia in a 4-month-old black girl. She has been responsive to antileukemic drugs, and remission has been induced without difficulty after each relapse. Although survival time was expected to be very short because of her age, race, type of leukemia, and initial peripheral leukocyte count at time of diagnosis, she is still alive 35 months after diagnosis of leukemia. One may speculate that the prolonged survival time might be related to the unknown genetic defect.