Incontinentia pigmenti and Behçet´s syndrome: an unusual combination

We describe an unusual case of a child who had had incontinentia pigmenti from birth and developed the clinical picture of Beh√ßet´s syndrome at five years of age. Among the various investigations performed, chemotactic activity of the polymorphonuclear leukocyte was found to be low. We discuss the possibility that there are common immunological abnormalities in the two syndromes.