Pigmentation in the Bloch-Sulzberger Syndrome (Incontinentia Pigmenti)

Some authors of recent articles on "incontinentia pigmenti" believe that Bloch-Sulzberger syndrome is a better name for this entity. The peculiar cutaneous hyper-pigmentation drew the attention of early investigators (Bardach,1Bloch,2Sulzberger,3and Naegeli4) to this disease. The first impression of a pigmentary abnormality has been expanded until "incontinentia pigmenti" is now recognized to be a developmental defect showing many lesions of ectodermal and mesodermal origin (Curth,5Epstein,6Carney,7Kitamura,8Cramer,9and Franceschetti10). Various lesions have been investigated histologically, but the origin and meaning of the pigment has never been explained. We have been fortunate in being able to investigate this disease in two sisters Report of Cases in Two Sisters Family History.—Father is Irish, English, and French; mother is Mexican. Father had two normal children by previous wife. Mother was told by