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April 1968

Conjunctival and Retinal Incontinentia Pigmenti

Author Affiliations

Miami, Fla
From the Department of Ophthalmology, University of Miami (Fla) School of Medicine. Doctor Mc-Crary was a special fellow of the National Institute of Neurological Diseases and Blindness: he is now at the Department of Ophthalmology, Baylor University School of Medicine, Houston.

Arch Ophthalmol. 1968;79(4):417-422. doi:10.1001/archopht.1968.03850040419010

This is, to our knowledge, the first report of conjunctival involvement in incontinentia pigmenti, or the Bloch-Sulzberger syndrome. The patient had not only unusual pigmentation of the conjunctivae and optic fundi, but right hemiatrophy as well. The histologic features of the pigment deposition in the conjunctivae are of interest.

Report of a Case  This 13-year-old left-handed girl (MD 484687) was first seen May 4, 1967. She was born March 15, 1954, in Mexico at full term, after an uncomplicated pregnancy and delivery. As an infant, the parents noted that the right side of her body was smaller than the left. She progressed more slowly than her siblings. She sat alone at 8 months, but did not walk until she was 3 years of age. A peculiar pigmentation of the skin developed early in life. In 1962, the patient had pulmonary tuberculosis, but made a good recovery after treatment with isoniazid,

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