Vitamin A-responsive panocular xerophthalmia in a healthy adult

An unusual case of classical xerophthalmia occurred in an otherwise healthy, well-nourished, 25-year-old woman. She had marked conjunctival and corneal xerosis, including early stromal edema, evolving pigmentary alterations of the retinal pigment epithelium, and notable constriction of her visual fields, which paralleled the distribution of the retinal lesions. Abnormalities of the conjunctiva, cornea, and visual fields disappeared within two weeks of oral vitamin A therapy, and 1 1/2 months later, many of the retinal lesions cleared as well. This case supports the primacy of isolated vitamin A deficiency in the etiology of corneal xerosis and "fundus xerophthalmicus"; demonstrates that stromal edema is an important early component of corneal involvement; and localizes the funduscopic abnormalities to the retinal pigment epithelium.