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What Syndrome Is This

A 2-month-old boy was sent for evaluation of congenital hypotrichosis and skin lesions. He was the first son of nonconsanguineous parents, and was the product of an uneventful pregnancy and labor. His height and weight at birth had been normal, and Apgar test scores were 9 and 10. After the first shedding of scalp hairs, no new hairs appeared except for a few, scattered, twisted black hairs. Furthermore, numerous white lesions progressively developed during the first weeks of life, mainly on his scalp and face, and less profusely on his trunk and limbs. On examination, the child had very sparse scalp hair (Fig. 1) and eyebrows and body hair were absent. Widespread white milialike papules were seen over the entire body, but were more profuse over his head and face (Fig. 2). He did not have dysmorphic features, and sweated normally. He was otherwise healthy.