Ichthyosis hystrix (epidermal nevus syndrome) and Coats' disease

A 4-year-old girl had the ichthyosis hystrix variant of the epidermal nevus syndrome with ocular fundus manifestations of Coats' disease. Her hearing was impaired because of serous otitis media and her visual acuity decreased because of hard yellow exudate in the macula as a result of decompensation and plasma leakage from the peripheral retinal vascular anomalies. Treatment of the vascular malformations with cryotherapy resulted in a reabsorption of the retinal edema and hard yellow exudate.