Ulerythema ophryogenes in Cornelia de Lange syndrome.

A 17-year-old woman with Cornelia de Lange syndrome had asymptomatic skin lesions since the age of 4 years. These were multiple, follicular, horny papules, present on both cheeks, and surrounded by erythematous skin. Similar lesions were present on the external aspect of the arms, but amidst skin of normal coloration. Keratosis pilaris atrophicans faciei (ulerythema ophryogenes) has been described as a cutaneous marker for several congenital syndromes. To our knowledge, its possible association with Cornelia de Lange syndrome has never been reported.