Phacomatosis pigmentokeratotica: Speckled-lentiginous nevus in associationwith nevus sebaceus

We report a case of a congenital nevus sebaceus with numerous brown macules and papules in the left auricular region. In the same lesion, pigmented ba sal cell carcinomas developed later in life. Additionally, on the left side of the neck, both shoulders and the left arm, features of nevus spilus wit h melanocytic and blue nevi were present. Such a combination of nevus sebac eus with melanocytic lesions may be explained by the concept of twin spotti ng and represent another case of phacomatosis pigmentokeratotica.