Congenital primary cutis verticis gyrata

Cutis verticis gyrata is an uncommon disorder. However, it is important to recognize the numerous disease processes this condition can accompany. An understanding of the classification of cutis verticis gyrata is essential to properly diagnose and treat patients who present with these unusual scalp lesions. This case of congenital primary cutis verticis gyrata, in addition to seven of the eight reported cases in the literature, occurred in an infant with a 45X karyotype, suggesting that cutis verticis gyrata may be a rare feature of Turner syndrome. Although small cutis verticis gyrata lesions are best excised and closed primarily, we recommend tissue expanders as an effective method of treating larger localized areas of primary cutis verticis gyrata.