Ocular changes in the Bloch-Sulzberger syndrome (Incontinentia pigmenti)

THE Bloch-Sulzberger syndrome is a familial condition consisting chiefly of ectodermal defects, of which changes in the skin, nails, hair, teeth, central nervous system, and eyes are the most common. The final dermal phase "incontinentia pigmenti" has received the most attention. This disease can be described as a rare and peculiar abnormality of development found almost exclusively in females at birth or shoitly afterwards; it usually begins as an inflammatory eruption of the skin tending to assume a linear or racemose pattern. This phase is succeeded or accompanied by the characteristic pigmentary disturbance (Fig. 1), which may, however, occur without the preceding inflammation.