Adenoma Sebaceum of Nose and Face in Tuberous Sclerosis
Adenoma sebaceum of the nose and face in tuberous sclerosis (Bourneville's syndrome)
What is adenoma sebaceum?
Adenoma sebaceum is a hamartoma on the face, composed of fibrovascular tissue and appearing as an aggregation of red or yellow papules that may be associated with tuberous sclerosis, also called Bourneville 's syndrome or Bourneville-Pringle's disease. (http://www.whonamedit.com/doctor.cfm/2408.html)
Bourneville's syndrome consists of epilepsy, severe mental retardation associated with adenoma sebaceum (overgrowth of the sebaceous glands) of the face, cerebral cortical tubers (hence the name "tuberous sclerosis") and hamartomatous tumours of the heart and kidney. It is heredofamilial and usually manifests itself early in life. Females are more often affected. Incomplete forms of the syndrome occur. Transmitted as an autosomal dominant trait with variable expressivity.