Widespread nevus sebaceus with multiple secondary tumors as presenting feature of Schimmelpenning-Feuerstein-Mims syndrome

A women presented with a systematized sebaceous nevus of the head and neck. She had developed multiple basal cell carcinomas and sebaceomas in the nev us over the past 3 years after a period of about 55 years without any chang es. Detailed medical history and physical examination revealed a constellat ion of findings suggesting a syndrome. Besides ptosis and a congenital, vas cular corneal clouding at the right,there was hypoplasia of the right maxil lary sinus, a right labyrinthine deafness and recurrent inguinal herniae on the right. The Schimmel-penning-feuerstein-Mims-Syndrome was diagnosed.