Congenital hypertrichosis lanuginosa "Werewolf Syndrome"

Congenital hypertrichosis lanuginosa (CHL) is a very rare syndrome characterized by excessive hair growth. Hypertrichosis can accompany certain genetic syndromes, or it can be induced secondarily by exogenous medications. Fewer than 40 cases are documented worldwide and the incidence of CHL ranges from 1 in a billion to 1 in 10 billion. Patients with CHL have growth of the lanugo hair (which normally grows on fetuses as a normal part of gestation, but is usually shed and replaced by vellus hair at about 40 weeks of gestational age) that increases in length and extent of involvement from birth to approximately age 2 years. Occasionally, the lanugo hair may be totally lost by the time the patient becomes an adult. A variant in which patients do not lose their lanugo hair over time is called congenital hypertrichosis universalis or persistent hypertrichosis universalis. No abnormalities of other organ systems are associated with CHL, although solitary case reports note abnormalities such as delayed tooth eruption, diffuse hamartoma of the arrector muscles, supernumerary teeth, glaucoma, aortic and cardiac valve abnormalities, and macromastia.

CHL is believed to be inherited in an autosomal dominant manner; most cases involve a familial component. The specific genetic abnormality in CHL has not been defined.

Treatment includes the use of hair-removal creams and shaving. Laser hair removal has been proposed as a treatment option.

The following is a preview for a discovery channel special on patients with CHL and Ambras syndrome.

Images from: http://www.keratin.com/ah/ah007.shtml
http://hubpages.com/hub/Ten-Unusual-And-Bizarre-Diseases-And-How-To-Avoid-Them