The prevalences of hypopigmented maculae and cafe-au-lait spots were investigated in 210 children with idiopathic epilepsy, between 2 and 17 years of age, and 2754 health controls children, at the Departments of Pediatrics and Dermatology, Hacettepe University and Inonu University Medical Schools, Turkey. In epileptic children, hypopigmented maculae and cafe-au-lait spots occurred in 14.3% and 30%, respectively, compared to 1.6% and 2.8%, in healthy children (P<.0001). Hypopigmented maculae were polygonal, ash leaf, and fingerprint in shape. Cafe-au-lait spots were discrete, round or oval, and uniformly hyperpigmented. These skin lesions should be considered a concomitant risk factor for epilepsy. [1]

COMMENT. The diagnostic criteria for type 1 neurofibromatosis include 6 or more cafe-au-lait spots greater than 5 mm in prepubertal and 15 mm in postpubertal children. They may be localized in any region except the palms and soles. Hypopigmented maculae are found in tuberous sclerosis and also in albinism, Waardenburg’s syndrome, and vitiligo. The incidence is higher when examined with a Wood lamp. The figure 3 is sometimes regarded as a significant number of cafe-au-lait spots in patients failing to meet criteria for the diagnosis but sufficient to indicate a partial penetrance of NF-1 [2]. In the above study, 3 spots were counted in 10% of 78 healthy children with spots and in 17% of 63 with epilepsy and cafe-au-lait spots. This number may indicate a trend toward increased risk of idiopathic epilepsy.