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September 1976

Incontinentia Pigmenti

Author Affiliations


Arch Ophthalmol. 1976;94(9):1631. doi:10.1001/archopht.1976.03910040461031

To the Editor.  —The excellent article, "Retinal Vascular Changes of Incontinentia Pigmenti" by Watzke et al, published in the May Archives (94:743-746, 1976), certainly increases our understanding of the pathogenesis of the pseudoglioma so often seen in the disease. The authors state that they found three reports in the world literature of retinal vascular anomalies associated with incontinentia pigmenti. In addition, we have described and illustrated a case with similar findings in our book, Ocular Pathology.1 The case illustrated in the book is that of a 6-month-old black girl I examined under general anesthesia some years ago. Fluorescein angiography, performed by me at that time, showed changes essentially the same as those described by Watzke et al. As the authors point out, the autosomal dominant gene generally is lethal for boys, so mostly infant girls will be affected, and my case also was that of an infant girl.