Hypertrichosis: Difference between revisions
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===The Hairy Family of Burma=== |
===The Hairy Family of Burma=== |
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One interesting record in history concerning congenital hypertrichosis lanuginosa is the hairy family of Burma, a four-generational pedigree of the disease. In 1826, John Crawford was leading a mission for the Governor-General of India through [[Burma]] when he met a member of the family of Burma. He tells of meeting a hairy man, Shwe-Maong. Shwe-Maong lived in the court of King Ava and acted as an entertainer. Shwe-Maong had four children: three normal children, and one child with congenital hypertrichosis, Maphoon. On a second mission to Ava, Maphoon was described as a thirty year old woman with two sons, one of which had hypertrichosis. The affected son was named Maong-Phoset. He was said to be a well mannered man and had an affected daughter named Mah-Me. Where as all affected members of the family had dental problems, the unaffected members had perfect teeth. |
One interesting record in history concerning congenital hypertrichosis lanuginosa is the hairy family of Burma, a four-generational pedigree of the disease. In 1826, John Crawford was leading a mission for the Governor-General of India through [[Burma]] when he met a member of the family of Burma. He tells of meeting a hairy man, Shwe-Maong. Shwe-Maong lived in the court of King Ava and acted as an entertainer. Shwe-Maong had four children: three normal children, and one child with congenital hypertrichosis, Maphoon. On a second mission to Ava, Maphoon was described as a thirty year old woman with two sons, one of which had hypertrichosis. The affected son was named Maong-Phoset. He was said to be a well mannered man and had an affected daughter named Mah-Me. Where as all affected members of the family had dental problems, the unaffected members had perfect teeth.<ref name="the hairy family of burma"/> |
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==See also== |
==See also== |
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Revision as of 16:52, 6 December 2009
| Hypertrichosis | |
|---|---|
| Specialty | Dermatology  |
Hypertrichosis is a disorder characterized by excessive hair growth on the body.[1] There are two distinct types of hypertrichosis: generalized hypertrichosis, which occurs over the entire body, and localized hypertrichosis, which is restricted to a certain area. Hypertrichosis is either congenital (present at birth), or acquired later in life.[2][3] The excess growth of hair occurs in areas that are not androgen-dependent areas of the skin.[4] 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.[3]
Classification
Congenital
Congenital forms of hypertrichosis are caused by genetic mutations. Congenital hypertrichosis is always present at birth.[3]
Congenital hypertrichosis lanuginosa
Congenital hypertrichosis lanuginosa is 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 replaced by vellus hair, however in an individual suffering from congenital hypertrichosis lanuginosa the lanugo hair is remains after birth.[3] As the person with this type of hypertrichosis ages the lanugo hair may thin and eventually only limited areas of hypertrichosis will be present.[5]
- Cause
The source of congenital hypertrichosis lanuginosa is unknown, it may be caused by a spontaneous genetic mutation rather than being inherited.[5]This form is also a dominant autosomal cutaneous disorder, meaning it is a dominant trait, not related to the sex chromosome, that affects the skin.[6]
- Occurrence
Congenital hypertrichosis lanuginosa is a rare condition. Around 50 cases have been recorded since the middle ages.[3]
Congenital generalized hypertrichosis
Congenital generalized hypertrichosis causes males to exhibit excessive facial and upper body hair, while women typically exhibit less severe asymmetrical hair distribution.[7]
- Cause
Congenital generalized hypertrichosis has a dominant pattern of inheritance and has been linked to the X chromosome.[8] A female carrying the hypertrichosis gene has a 50-50 chance of passing it to her offspring. A male carrier will pass this form of hypertrichosis on to his daughters, but not his sons.
- Occurrence
Fewer than 100 cases of congenital generalized hypertrichosis have been documented in scientific publications and by the media.[9]
Congenital generalized hypertrichosis terminalis
Congenital generalized hypertrichosis terminalis is characterized by the excessive growth of terminal hair over the entire body.[10] Gingival hyperplasia is often present with this condition.[10]
- Cause
Congenital generalized hypertrichosis terminalis is thought to be caused by genetic changes on chromosome 17 resulting in the addition or removal of millions of nucleotides.[10] The gene MAP2K6 may play a role, or this condition may also be due to the change in the chromosome affecting the transcription of genes further away on the chromosome.[9]
Congenital terminal hypertrichosis
Congenital terminal hypertrichosis is characterized by the presence of fully pigmented terminal hair which covers the entire body.[3] This condition is usually present along with gingival hyperplasia.[3] 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.[3]
- Cause
The exact genetic mutation which causes congenital terminal hypertrichosis is unknown.
Congenital circumscribed hypertrichosis
Congenital circumscribed hypertrichosis is associated with the presence of thick vellus hair on the upper extremities.[11] Circumscribed signifies that this type of hypertrichosis is restricted to certain parts of the body, in this case, the extrasensory surfaces of the upper extremities.[11] 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.[11]
- Cause
The exact genetic mutation which causes congenital circumscribed hypertrichosis is unknown.
Congenital localized hypertrichosis
Congenital localized hypertrichosis is a localized increase in hair density and length.
Acquired
Acquired hypertrichosis is gained after birth as a side effect of a drug, or from another cause. Acquired forms of hypertrichosis can usually be reduced with various treatments.
Acquired hypertrichosis lanuginosa
Acquired hypertrichosis lanuginosa is characterized by rapid growth of lanugo hair particularly on the face.[12][13] Hair also appears on the trunk, and armpits, while palms and soles are unaffected.[13] The hair that grows due to this condition os commonly referred to as malignant down. This hair is very fine and unpigmented.[13]
- Cause
Acquired hypertrichosis lanuginosa is commonly present with cancer.[14] This condition is also known to be caused by metabolic disorders, such as anorexia, hormone imbalances, such as hyperthyroidism, or as a side effect of a drug.[14]
Acquired patterned hypertrichosis
Acquired patterned hypertrichosis is an increase in hair growth in a pattern formation, and, similar to acquired generalized hypertrichosis, is an sign of internalmalignancy.[4]
Acquired generalized hypertrichosis
Acquired generalized hypertrichosis commonly affects 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 is 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.[2] 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, however this hair disappears shortly after discontinuing the use of topical minoxidil.[15]
- Cause
Acquired generalized hypertrichosis can be obtained through cancer. The hair that grows due to this condition is known as malignant down. Scientists are unsure of why cancer causes hypertrichosis. Oral and topical minoxidil treatments are also known to cause acquired generalized hypertrichosis. [15]
Acquired localized hypertrichosis
Acquired localized hypertrichosis is an increase in hair density and length often secondary to irritation or trauma.[16]: 651 This form is restricted to a certain area of the body.
- Cause
Minoxidil, a medication for preventing hair loss, is thought to trigger acquired localized hypertrichosis.[17]
Nevoid hypertrichosis
Nevoid hypertrichosis may be congenital or acquired because it can appear at birth or later in life. It features a lone area of excessive terminal hair on the body and is usually not related to any other diseases.[3]
Epidemiology
Management
There are no cures for congenital forms of hypertrichosis. Acquired forms of hypertrichosis have a variety of sources, and are usually treated by removing the factor causing hypertrichosis, e.g. a medication with undesired side-effects. All hypertrichosis, congenital or acquired, can be reduced through hair removal. Hair removal treatments are categorized into two principal subdivisions: temporary removal and permanent removal.[18] Treatment may have adverse effects by causing scarring,dermatitis, or hypersensitivity.[3]
- Temporary Removal
"Depilation", or removal of hair to the level of the skin, lasts several hours to several days and can be achieved by:
- Shaving or trimming (manually or with electric shavers)
- Depilatories (creams or "shaving powders" which chemically dissolve hair)
- Friction (rough surfaces used to buff away hair)
"Epilation", or removal of the entire hair from the root, lasts several days to several weeks and may be achieved by:
- Threading (also called fatlah or khite, in which a twisted thread catches hairs as it is rolled across the skin) [19]
- Permanent Removal
Permanent hair removal involves several imperfect options. A number of methods have been developed that use chemicals, energy of varying types, or a combination to target the areas that regulate hair growth. Permanently destroying these areas while sparing surrounding tissue is a difficult challenge. Methods include:
History
The first recorded case of hypertrichosis was a man by the name of Petrus Gonzales that was born in the Canary Islands and was documented by Altrovandus in 1648. He observed Gonzales and his family and noted that two daughters, a son and a grandchild were all effected with hypertrichosis. Altrovandus dubbed them the Ambras family, after an Innsborough castle where portraits of the family were found. For the next 300 years there were approximately 50 cases observed. The German scientist Rudolf Virchow described a form of hypertrichosis accompanying teeth defects in 1873. Three years later, Bartles added the term "universalis" to the disease. In 1890 Chiari coined the term hypertrichosis when he called the disease "hypertrichosis of the dog-men."[20]
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, and Annie Jones, the bearded woman, were all notable sufferers of hypertrichosis.[21]
The Hairy Family of Burma
One interesting record in history concerning congenital hypertrichosis lanuginosa is the hairy family of Burma, a four-generational pedigree of the disease. In 1826, John Crawford was leading a mission for the Governor-General of India through Burma when he met a member of the family of Burma. He tells of meeting a hairy man, Shwe-Maong. Shwe-Maong lived in the court of King Ava and acted as an entertainer. Shwe-Maong had four children: three normal children, and one child with congenital hypertrichosis, Maphoon. On a second mission to Ava, Maphoon was described as a thirty year old woman with two sons, one of which had hypertrichosis. The affected son was named Maong-Phoset. He was said to be a well mannered man and had an affected daughter named Mah-Me. Where as all affected members of the family had dental problems, the unaffected members had perfect teeth.[22]
See also
- Fur: An Imaginary Portrait of Diane Arbus, a film in which one of the main characters suffers from hypertrichosis.
References
- ^ Wendelin, D.; Pope, D.; Mallory, S. (2003). "Hypertrichosis". Journal of the American Academy of Dermatology. 48: 161–179. doi:10.1067/mjd.2003.100.
- ^ a b Sutton, Richard L. (1916). Diseases of The Skin. St. Louis: C.V. Mosby Company. pp. 408, 705. Retrieved November 29, 2009.
- ^ a b c d e f g h i j Ngan, Vanessa (June 15, 2009). "Hypertrichosis". DermNet NZ. New Zealand Dermatological Society Incorporated. Retrieved November 29, 2009.
- ^ a b James, William; Berger, Timothy; Elston, Dirk (2005), Andrews Diseases of the Skin: Clinical Dermatology (10 ed.), Saunders, p. 769, ISBN 0721629210
- ^ a b Taylor, Sarah K (June 18, 2009). "Congenital Hypertrichosis Lanuginosa". Emedicine. Medscape. Retrieved December 4, 2009.
- ^ 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
- ^ 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.
- ^ 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.
- ^ a b Ewen Callaway (May 21, 2009). ""Wolf Man" Condition Down To Huge DNA Malfunction". NewScientist. Retrieved November 29, 2009.
- ^ a b c Sun, M.; Li, N.; Dong, W.; Chen, Z.; Liu, Q.; Xu, Y.; He, G.; Shi, Y.; Li, X. (2009). "Copy-number mutations on chromosome 17q24.2-q24.3 in congenital generalized hypertrichosis terminalis with or without gingival hyperplasia". American journal of human genetics. 84 (6): 807–813. doi:10.1016/j.ajhg.2009.04.018. PMC 2694973. PMID 19463983.
- ^ a b c 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.
- ^ Samson, Michael K.; Buroker, Thomas R.; Henderson, Michael D.; Baker, Laurence H.; Vaitkevicius, Vainutis K. (2006). "Acquired hypertrichosis lanuginosa". Cancer. 36 (4): 1519–1521. doi:10.1002/1097-0142(197510)36:4<1519::AID-CNCR2820360446>3.0.CO;2-H.
- ^ a b c Ngan, Vanessa (June 15, 2009). "Hypertrichosis lanuginosa acquisita". DermNet NZ. New Zealand Dermatological Society Incorporated. Retrieved December 6, 2009.
- ^ a b Slee, Peter H.Th.J.; Verzijlbergen, Fred J.; Schagen van Leeuwen, Jules H.; van der Waal, Rutger I.F. (2006). "CASE 2. Acquired Hypertrichosis: A Rare Paraneoplastic Syndrome in Various Cancers". Journal of Clinical Oncology. 24 (3): 523–524. doi:10.1200/JCO.2005.01.2443.
- ^ a b 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) - ^ Freedberg (2003). Fitzpatrick's Dermatology in General Medicine (6 ed.). McGraw-Hill. p. 651. ISBN 0071380760.
- ^ Earhart, RN; Ball; Nuss; Aeling (1977). "Minoxidil-induced hypertrichosis: treatment with calcium thioglycolate depilatory". Southern medical journal. 70 (4): 442–3. PMID 850811.
- ^ Ngan, Vanessa (June 15, 2009). "Hair removal techniques". DermNet NZ. New Zealand Dermatological Society Incorporated. Retrieved November 29, 2009.
- ^ a b c Ngan, Vanessa (June 15, 2009). "Epilation". DermNet NZ. New Zealand Dermatological Society Incorporated. Retrieved November 29, 2009.
- ^ Rashid; White (2007), A hairy development in hypertrichosis: a brief review of Ambras syndrome, retrieved November 8, 2009
- ^ Taylor, Sarah; Galeckas, Kenneth (June 18), Congenital Hypertrichosis Lanuginosa, retrieved December 6, 2009
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