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Hypertrichosis

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Hypertrichosis
SpecialtyDermatology Edit this on Wikidata

Hypertrichosis is a disorder characterized by excessive hair growth on the body. Generalized hypertrichosis occurs over the entire body, while localized hypertrichosis is restricted to a certain area. Hypertrichosis may be either congenital (present at birth), or acquired later in life.[1][2] The excess growth of hair occurs in areas that are not androgen-dependent areas of the skin.[3] Hypertrichosis differs from hirsuitism, which is abnormal hair growth on females in places which normally have minimal or no hair (i.e. chest hair, beard).

Several circus sideshow performers in the 19th and early 20th centuries, such as "the bearded lady" and "wolf-man", suffered from hypertrichosis. They were usually displayed as "freaks" and promoted as if they had distinct human and animal traits. More extensive cases of hypertrichosis have been informally called werewolf syndrome.[2]

Classification

Congenital

Congenital forms of hypertrichosis are inherited from the parents through their genes.

File:Hyper1.jpg
Congenital hypertrichosis lanuginosa

Congenital hypertrichosis lanuginosa is a rare form of hypertrichosis first noticeable at birth, because the infant is completely covered with thin lanugo hair. Normally, lanugo hair is shed before the infant is born and is replaced by vellus hair, however in an individual suffering from congenital hypertrichosis lanuginosa the lanugo hair is never shed and instead persists throughout the life of the individual.[2] Congenital hypertrichosis languniosa is inherited through a gene, hence the name congenital.[4] Congenital hypertrichosis languniosa is a dominant autosomal cutaneous disorder, meaning it is a dominant trait, not related to the sex chromosome, that affects the skin.

Congenital generalized hypertrichosis

Congenital generalized hypertrichosis is a dominant trait which has been linked to the X chromosome.[5] In males, excessive facial and upper body hair are apparent, while women typically exhibit asymmetrical hair distribution.[6] Congenital generalized hypertrichosis is a very rare disorder, with fewer than 100 cases documented in scientific publications and by the media. It is thought that this condition is caused by genetic changes on chromosome 17 resulting in the addition or removal of millions of nucleotides. The gene MAP2K6 may play a role, but this condition may also be due to the change in the chromosome affecting the transcription of genes further away on the chromosome.[7]

Congenital hypertrichosis terminalis

Congenital hypertrichosis terminalis is characterized by the presence of terminal hair which covers the entire body. This condition is almost always presents with gingival hyperplasia. This form is most responsible for the term "Werewolf Syndrome" because of the thick dark hair that appears. Sufferers of this condition are usually performers at circuses because of their unusual appearance.[2]

Congenital circumscribed hypertrichosis

Congenital circumscribed hypertrichosis is closely associated with the presence of thick vellus hair on the upper extremities. Circumscribed signifies that this type of hypertrichosis is restricted to certain parts of the body, in this case, the upper extremities. Hairy Elbow Syndrome, a type of congenital circumscribed hypertrichosis, shows excessive growth on and around the elbows. This type of hypertrichosis is present at birth and becomes more prominent during aging, only to regress at puberty.[8]

Congenital localized hypertrichosis

The presence of undesired hair growth can be very troublesome to many people.[9] Localized congenital hypertrichosis is inherited, localized increase in hair density and length. The instances of the condition can occur over the entire skin, or be limited to only certain areas. Most treatments focus on permanent hair removal.

Acquired

File:Hyper5.jpg

Acquired hypertrichosis is gained after birth as a side effect of a drug, or from another cause.

Acquired patterned hypertrichosis

Patterned acquired hypertrichosis is an increase in hair growth in a pattern formation, and, similar to acquired generalized hypertrichosis, is an ominous sign of internal malignancy.[3]: 770–771 

Acquired generalized hypertrichosis

Acquired generalized hypertrichosis is usually gained due to an underlying cause. This type of hypertrichosis can usually be reduced with various treatments. The most common areas affected are the cheeks, upper lip, and chin. This form also affects the forearms and legs, however it is less common to occur in these areas. Another deformity associated with acquired generalized hypertrichosis are multiple hairs occupying the same follicle, or hairs going off of their normal growth pattern as what happens to the eyelashes in a condition known as trichiasis.[1] Oral minoxidil treatments for hypertension are known to cause this condition. Topical minoxidil used for alopecia causes hair growth in the areas it is applied to. This hair disappears shortly after discontinuing the use of topical minoxidil however.[10]

Acquired localized hypertrichosis

Localized acquired hypertrichosis is an increase in hair density and length often secondary to irritation or trauma.[11]: 651 

Naevoid hypertrichosis

Naevoid hypertrichosis may be congenital or acquired because it can appear at birth or later in life. It features a lone excessively hairy area on the body and is usually not related to any other diseases.[2]

Causes

Genetics

The cause of all congenital hypertrichosis is a genetic mutation. Congenital hypertrichosis is always present at birth.[2] The exact mutation of the gene which triggers hypertrichosis is unknown.

X Linked female recessive traits
Patterns of inheritance of females with hypertrichosis.
X Linked male dominant traits
Patterns of inheritance of males with hypertrichosis.

The congenital hypertrichosis condition has been linked to the X chromosome. A female carrying the hypertrichosis gene has a 50-50 chance of passing it to her offspring. A male carrying the gene will always pass it onto his daughters, but never to his sons. The gene that causes hypertrichosis is believed to be from the time when primates were evolving to men.[12]

Acquired

Acquired hypertrichosis is the most common form of hypertrichosis. Causes include, metabolic disorders, anorexia, thyroid disorders, and most commonly drugs or chemicals such as oral phentynoin and ciclosporin.[2] Minoxidil, a medication for preventing hair loss, is thought to trigger acquired localized hypertrichosis. Medication induced hypertrichosis can often be treated by using hair removal agents.[13] Acquired generalized hypertrichosis can be obtained through cancer. The hair that grows due to this condition is known as malignant down and is fine and silky. Scientists are unsure of why cancer causes hypertrichosis.


Epidemiology

Occurence

Frequency for congenital hypertrichosis lanuginosa is dismal, with less than 50 reported cases worldwide. The current incidence of the condition has been reported in the range of 1 in a billion to 1 in 10 billion. Additionally, there is no known prediction for congenital hypertrichosis in terms of race, sex, or geographic region.[14] Most of the cases are limited to one family.

Management

There is no cure for congenital hypertrichosis; however, the disease is only cosmetic, and hair removal can reduce its appearance. Hair removal treatments are divided into three principal subdivisions: temporary removal, temporary reduction, and permanent removal.[15] Temporary hair removal methods (also referred to as epilation methods) include plucking, waxing, shaving, threading, abrasives, and sugaring.[16]

  • Plucking: The use of tweezers to remove a recommended small cluster of hairs.
  • Waxing: Requires the user to equally distribute a layer of heated wax on a small section of hair, and then place a small cotton strip over the designated area. Once the wax hardens, the user rapidly rips the strip off in the opposite direction of hair growth.
  • Threading: Considered as one of the least painful methods of Epilation, threading requires the user to roll a twisted shred of cotton along the desired area of hair removal, entangling hairs gently within the cotton.[16]
  • Abrasives: This method is not suggested for those with sensitive skin because the hair is removed by rigorously rubbing it off. Some examples of abrasives are pumice scrubs, sandpaper, and exfoliaters.[16]
  • Sugaring: Much like waxing, sugaring involves the same steps but uses a different mixture. Replacing the wax is a heated sugar paste composed of sugar, lemon juice, and water.

Treatment may have adverse effects by causing scarring, dermatitis, or hypersensitivity.[2]

History

Society and culture

People with hypertrichosis often found jobs as circus performers due to their unique appearance. Fedor Jeftichew, Jo-Jo the Dog-Faced Man, Stephan Bibrowski, Lionel the Lion-Faced Man, Jesus "Chuy" Aceves, wolfman, andAnnie Jones, the bearded woman, were all notable sufferers of hypertrichosis. [17]

The Hairy Family of Burma

One interesting record in history concerning congenital hypertrichosis languinosa is the hairy family of Burma that had a four-generation pedigree of the disease.[18] A man named John Crawford met the first member of The Hairy Family of Burma in 1826 when he led a mission of the Governor-General of India, to the court of King Ava: a providence in Burma. John Crawford later in life became a notable naturalist and anthropologist. He tells of meeting a hairy man named Shwe-Maong. Shwe-Maong lived in the palace and acted as an entertainer known for acting the fool and grimacing. Shwe-Maong had four kids total: three normal children, two which died at an early age and one child with congenital hypertrichosis. The abnormal child was named Maphoon. On a second mission to Ava, Captain Henry Yule described Ava as a thirty year old woman with two sons.

See also

Wikimedia Commons has media related to Hypertrichosis.

References

  1. ^ a b Sutton, Richard L. (1916). Diseases of the Skin. C.V. Mosby Company. pp. 408, 705.
  2. ^ a b c d e f g h Baird, Elizabeth; Strack, Mathew (June 15), Hypertrichosis, retrieved September 20, 2009 {{citation}}: Check date values in: |date= and |year= / |date= mismatch (help)
  3. ^ a b James, William; Berger, Timothy; Elston (2005), Andrews Diseases of the Skin: Clinical Dermatology (10 ed.), Saunders, p. 769, ISBN 0721629210 {{citation}}: Text "first3 -Dirk" ignored (help)
  4. ^ Mendiratta, Vibhu; Harjai, Bhawna; Gupta, Tanvi (2008), Pediatric Dermatology, vol. 25 (4 ed.), Lady Hardinge Medical College, Associated Shrimati Sucheta Kriplani and Kalawati Saran Childrens Hospital, New Delhi, India: Wiley Periodicals, Inc., doi:10.1111/j.1525-1470.2008.00716.x
  5. ^ Macías-Flores, MA; García-Cruz; Rivera; Escobar-Luján; Melendrez-Vega; Rivas-Campos; Rodríguez-Collazo; Moreno-Arellano; Cantú (1984). "A New Form of Hypertrichosis Inherited as an X-linked Dominant Trait". Human genetics. 66 (1): 66–70. PMID 6698556.
  6. ^ Figuera, L.; Pandolfo, M.; Dunne, P.; Cantú, J.; Patel, P. (1995). "Mapping of the congenital generalized hypertrichosis locus to chromosome Xq24-q27.1". Nature genetics. 10 (2): 202–207. doi:10.1038/ng0695-202. PMID 7663516.
  7. ^ Callaway, Ewen (21 May 2009). "'Wolf man' condition down to huge DNA malfunction". New Scientist. Retrieved 2009-05-23.
  8. ^ Escalonilla, P; Aguilar; Gallego; Piqué; Fariña; Requena (1996). "A new case of hairy elbows syndrome (Hypertrichosis cubiti)". Pediatric dermatology. 13 (4): 303–5. PMID 8844750.
  9. ^ Blume-Peytavi, Ulrike, et al. (2008).Hair Growth and Disorders. Springer. ISBN 978-3-540-46908-7.
  10. ^ Gonzales, M. (April 27), "Clinical and Experimental Dermatology", British Association of Dermatologists Journal, 19 (2): 157–158, 119275626 {{citation}}: Check date values in: |date= and |year= / |date= mismatch (help)
  11. ^ Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). McGraw-Hill. ISBN 0071380760.
  12. ^ ABC News (August 2), Hypertrichosis - Real Life Werewolves?, retrieved September 20, 2009 {{citation}}: Check date values in: |date= and |year= / |date= mismatch (help)
  13. ^ Earhart, RN; Ball; Nuss; Aeling (1977). "Minoxidil-induced hypertrichosis: treatment with calcium thioglycolate depilatory". Southern medical journal. 70 (4): 442–3. PMID 850811.
  14. ^ Samalonis, Lisa (August), Detecting Pigmentary Hair Disorders in Children, retrieved October 17 2009 {{citation}}: Check date values in: |accessdate=, |date=, and |year= / |date= mismatch (help)
  15. ^ Ngan, Vanessa (June 15), Hair removal Techniques, retrieved October 4 2009 {{citation}}: Check |url= value (help); Check date values in: |accessdate=, |date=, and |year= / |date= mismatch (help)
  16. ^ a b c Ngan, Vanessa (June 15), Epilation, retrieved October 4 2009 {{citation}}: Check date values in: |accessdate=, |date=, and |year= / |date= mismatch (help)
  17. ^ Hall, Dave (2007), Doctor Doctor..., retrieved October 23, 2009
  18. ^ Bondeson, J; Miles, A (July), "The hairy family of Burma: a four generation pedigree of congenital hypertrichosis lanuginosa.", The Royal Society of Medicine, 89 (7): 403–408, 1295857 {{citation}}: Check date values in: |date= and |year= / |date= mismatch (help)
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