Carcinomas basocelulares múltiples sobre facomatosis pigmentoqueratótica

We present a patient with phacomatosis pigmentokeratotica (PPK) who developed several basal cell carcinomas on epidermal nevus lesions in adult life. PPK shows an elevated incidence of development of malignant lesions both on the sebaceous or epidermal nevus component as well as on the nevus spilus one.