Of 32 patients (19 females and 13 males, ranging in age from 2 to 44 years), nine had ocular problems related to neutrophil dysfunction. Four patients had blepharokeratoconjunctivitis and pannus formation. In one of these, severe visual loss secondary to corneal thinning and scarring occurred. Five patients had inactive chorioretinal scars without visual loss. Although some of the other 23 patients had minor ocular abnormalities, we could not demonstrate that they were related to the neutrophil dysfunction. However, none of the control subjects (20 patients with systemic lupus erythematosus and 20 randomly selected eye clinic patients) had lesions resembling those of the patients with neutrophil dysfunction. Fisher's exact test (one-tailed) gave the following values: P less than .03 for keratitis; P less than .01 for chorioretinal scars; and P less than .001 for keratitis and chorioretinal scars. Abnormal neutrophil function probably interferes with the control of normal eyelid flora and predisposes the eye to the development of marginal keratitis. Eyelid hygiene and topical administration of antibiotics and corticosteroids during inflammatory episodes may prevent the progression of corneal vascularization. The chorioretinal scars do not appear to be progressive.
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