Congenital generalized hypertrichosis terminalis (CGHT) is a heterogenous group 
of diseases with continuing excessive growth of terminal hair. "Ambras syndrome" 
was first coined by Baumeister in 1993 to describe a case of nonsyndromic CGHT 
which was erroneously analogized to the portrait paintings of Petrus Gonzales 
and his children, exhibited in Ambras Castle near Innsbruck, Austria. This 
family probably, a syndromic type with abnormal dentition, inherited as an 
autosomal dominant trait. CGHT associated with gingival hyperplasia is probably 
a particular entity typified by the historical cases of Julia Pastrana and her 
son. An X-linked type of CGHT has likewise been categorized as "Ambras 
syndrome". Moreover, some reports have mistakenly classified "Ambras syndrome" 
as an example of hypertrichosis lanuginosa. Potential gene loci identified so 
far may include 8q22, 17q24.2-q24.3 and Xq24-q27.1. The designation "Ambras 
syndrome" has thus been applied to various types of congenital hypertrichosis 
that differ to such degree that the name "Ambras" has no specific meaning, 
neither in the past nor in the future. Hence, this misleading term should now be 
jettisoned.