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Congenital
hypertrichosis terminalis with gingival hyperplasia
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 means
all over body hair growth which is long and non-pigmented. However,
there is a sight 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.
Genetics are very clearly involved in congenital hypertrichosis
terminalis with generations of families affected. Hirsutism with
gingival fibromatosis follows a dominant pattern of inheritance.
There is no specific relationship between the age of development
of the changes in the teeth and the hypertrichosis. The latter may
be present at birth but often appears later at puberty.
People described as “werewolves” or “wolf men”
for their excessive hair growth most likely have congenital hypertrichosis
lanuginosa or terminalis. There is no known treatment available
other than depilation.
Congenital
hypertrichosis terminalis with gingival hyperplasia references
- Bondeson
J, Miles AE. Julia Pastrana, the nondescript: an example of congenital,
generalized hypertrichosis terminalis with gingival hyperplasia.
Am J Med Genet. 1993 Aug 15;47(2):198-212.
- Lee IJ,
Im SB, Kim DK. Hypertrichosis universalis congenita: a separate
entity, or the same disease as gingival fibromatosis? Pediatr
Dermatol. 1993 Sep;10(3):263-6.
- Lacombe D, Bioulac-Sage P, Sibout M, Daussac
E, Lesure F, Manchart JP, Battin J. Congenital marked hypertrichosis
and Laband syndrome in a child: overlap between the gingival fibromatosis-hypertrichosis
and Laband syndromes. Genet Couns. 1994;5(3):251-6.
- Irvine AD, Dolan OM, Hadden DR, Stewart
FJ, Bingham EA, Nevin NC. An autosomal dominant syndrome of acromegaloid
facial appearance and generalised hypertrichosis terminalis. J
Med Genet. 1996 Nov;33(11):972-4.
- Anavi Y, Lerman P, Mintz S, Kiviti S. Idiopathic
familial gingival fibromatosis associated with mental retardation,
epilepsy and hypertrichosis. Dev Med Child Neurol. 1989 Aug;31(4):538-42.
- Horning GM, Fisher JG, Barker BF, Killoy
WJ, Lowe JW. Gingival fibromatosis with hypertrichosis. A case
report. J Periodontol. 1985 Jun;56(6):344-7.
- Miles AE. Julia Pastrana: the bearded lady.
Proc R Soc Med. 1974 Feb;67(2):160-4.
- Suskind R, Esterly NB. Congenital hypertrichosis
universalis. Birth Defects Orig Artic Ser. 1971 Jun;7(8):103-6.
- Bondeson J. [The bearded lady is a case
of severe hypertrichosis]. Lakartidningen. 1998 Jan 28;95(5):393.
- Moreau JL, Ndiaye R, Dupiot M, Diallo PD.
[Congenital idiopathic gingival hyperplasia observed in a 6-year-old
child]. Rev Stomatol Chir Maxillofac. 1985;86(3):192-4.
- Vogel RI. Gingival hyperplasia and folic acid
deficiency from anticonvulsive drug therapy: a theoretical relationship.
J Theor Biol. 1977 Jul 21;67(2):269-78.
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