Scalp skin lesion in Turner syndrome: more than lymphoedema?

We present a case of a newborn with Turner syndrome (TS) and a scalp skin lesion resembling cutis verticis gyrata (CVG). Several reports on CVG in TS describe the lesions and correlate it with lymphoedema frequently seen in foetuses and infants with Turner syndrome. Histological examination in this case, however, shows a congenital mucinous nevus. Possibly an abnormal amount of proteoglycans in the skin of patients with TS can clarify the occurrence of this type of skin lesion.