Basal cell nevus syndrome: A tale of ancient mummies and cyclopic sheep

Abstract

Disease has left its record upon this earth from before a written or historic record is available. The study of mummies and human remains in the last 100 years has shed light on numerous diseases.  Diseases previously prominent in society such as tuberculosis and leprosy, are well represented in the paleorecord.  Even rare diseases whose identities were unknown until recent times have been evidenced in the paleorecord.  Basal cell nevus syndrome, also known as Gorlin- Goltz syndrome, is a rare, heritable, autosomal-dominant condition with complete penetrance and variable expressivity.  Although this disease was first recognized by Jarisch and White in 1894 and its spectrum elaborated by Gorlin and Goltz in 1960, the archeological finding of Egyptian Dynastic brothers exhibits proof of this disease in the paleorecord antedates these descriptions by over 3000 years. A look at the original finding and how far our knowledge has come in understanding this disease is the focus of this review.