Worth syndrome, also known as benign form of Worth hyperostosis corticalis generalisata with torus platinus, autosomal dominant osteosclerosis, autosomal dominant endosteal hyperostosis or Worth disease,[1][2] is a rare autosomal dominant congenital disorder that is caused by a mutation in the LRP5 gene.
[3] It is characterized by increased bone density and benign bony structures on the palate.
[1][3][4][5] Worth syndrome is caused by a mutation in the LRP5 gene, located on human chromosome 11q13.4.
In 1977, two doctors, R.J. Gorlin and L. Glass, distinguished the syndrome from van Buchem disease.
In 1987 a group of Spanish doctors pointed out that the condition may not be benign, and may sometimes cause nerve damage.