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Congenital
hypertrichosis
Congenital hypertrichosis is present from birth and usually persists
throughout life. There are several different names used by dermatologists
to describe this disease. "Congenital hypertrichosis lanuginosa",
and "congenital hypertrichosis universalis" are the most
common names and they mean exactly the same thing, all over body
hair growth which is long and non-pigmented. However, there is a
variation called "congenital hypertrichosis terminalis".
This condition involves all over body hair growth, but the hair
is fully pigmented terminal hair and the condition is almost always
associated with gingival hyperplasia (teeth defects). This is not
the same as congenital hypertrichosis lanuginosa and there is a
separate page describing this condition.
 In addition to congenital hypertrichosis lanuginosa where hair
develops all over the body, there are other case reports of individuals
with hypertrichosis lanuginosa limited only to the face with the
torso, arms, and legs being mostly unaffected. Some research reports
have tried to collect these case reports together and have called
the condition "ambras syndrome"
Lanugo hair is the very first hair to be made by an embryo’s
hair follicles when still inside the womb. This hair can grow quite
long but it is usually very fine and unpigmented. This first wave
of hair growth is normally shed by the embryo at around 8 months
gestation and replaced by fine vellus hair and terminal scalp hair
in preparation for birth. Normally a few of the long lanugo hairs
might survive through birth but are shed shortly after. However,
sometimes a child can be born with most or all of the lanugo hairs
still growing. This gives the appearance of the child being covered
in a light colored fur. This excessively long hair all over the
body persists throughout life. Congenital hypertrichosis lanuginosa
is a very rare form of hypertrichosis affecting less than 1 in 1000
million children. It seems to have a genetic component with family
histories of hypertrichosis being reported in relations of those
affected. People described as "werewolves" or "wolfmen"
for their excessive hair growth most likely have congenital hypertrichosis
lanuginosa or terminalis. There is no known treatment available
other than depilation.
Congenital
hypertrichosis lanuginosa references
- Bondeson
J, Miles AE. The hairy family of Burma: a four generation pedigree
of congenital hypertrichosis lanuginosa. J R Soc Med. 1996 Jul;89(7):403-8.
- Balducci
R, Toscano V, Tedeschi B, Mangiantini A, Toscano R, Galasso C,
Cianfarani S, Boscherini B. A new case of Ambras syndrome associated
with a paracentric inversion (8). Clin Genet. 1998 Jun;53(6):466-8.
- Baumeister
FA, Egger J, Schildhauer MT, Stengel-Rutkowski S. Ambras syndrome:
delineation of a unique hypertrichosis universalis congenita and
association with a balanced pericentric inversion (8). Clin Genet.
1993 Sep;44(3):121-8.
- Baumeister FA. Differentiation of Ambras
syndrome from Hypertrichosis Universalis. Clin Genet. 2000 Feb;57(2):157-8.
- Partridge JW. Congenital hypertrichosis
lanuginosa: neonatal shaving. Arch Dis Child. 1987 Jun;62(6):623-5.
- Kint AH, Vermander FR, Decroix JM. [Congenital
hypertrichosis lanuginosa]. Hautarzt. 1985 Jul;36(7):423-4.
- Freire-Maia N, Felizali J, de Figueiredo
AC, Opitz JM, Parreira M, Maia NA. Hypertrichosis lanuginosa in
a mother and son. Clin Genet. 1976 Nov;10(5):303-6.
- Suskind R, Esterly NB. Congenital hypertrichosis
universalis. Birth Defects Orig Artic Ser. 1971 Jun;7(8):103-6.
- Beighton P.Congenital hypertrichosis lanuginosa.
Arch Dermatol. 1970 Jun;101(6):669-72.
- Felgenhauer WR. [Hypertrichosis languinosa
universalis]. J Genet Hum. 1969 May;17(1):1-44.
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