ABSTRACT
Hypertrichosis is defined as excessive hair growth anywhere on the body beyond the normal variation compared with individuals of the same age, race, and sex and affecting areas not predominantly androgen-dependent, and affecting both males and females. Hypertrichosis can be produced by the conversion of vellus hairs into terminal hairs or due to an increase in the vellus hair density. Congenital hypertrichosis is rare and may be an isolated condition of the skin or a component feature of other disorders. The “Ambras syndrome” is an autosomal dominant trait inheritance of congenital hypertrichosis lanuginosa linked to chromosome 8q22, but also as sporadic events. Donohue syndrome is a severe and very rare autosomal recessive inheritance form of congenital insulin resistance due to a mutation in the insulin receptor gene. Schinzel Giedion Syndrome is an autosomal recessive hypertrichosis. The gene involved in this disorder is SETBP1. It presents multiple congenital craniofacial anomalie.
