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'''Hypertrichosis''' (also called '''Ambras syndrome''') is an abnormal amount of hair growth over the body.<ref name="PMID12582385"/><ref name="PMID12444804"/> The two distinct types of hypertrichosis are generalized hypertrichosis, which occurs over the entire body, and localized hypertrichosis, which is restricted to a certain area.<ref name="PMID12582385"/> Hypertrichosis can be either congenital (present at birth) or acquired later in life.<ref name="dermnet"/><ref name="diseasesoftheskin"/> The excess growth of hair occurs in areas of the skin with the exception of [[Androgenic hair|androgen-dependent]] hair of the pubic area, face, and axillary regions.<ref name="Andrews"/>
'''Hypertrichosis''' (also called '''Ambras syndrome''') is an abnormal amount of hair growth over the body.<ref name="PMID12582385">{{Cite journal | doi = 10.1067/mjd.2003.100 | pmid = 12582385 | title = Hypertrichosis | year = 2003 | last1 = Wendelin | first1 = D. | last2= Pope| first2= D.| last3= Mallory| first3= S.| journal = Journal of the American Academy of Dermatology | volume = 48 | issue = 2 | pages = 161–179 }}</ref><ref name="PMID12444804">{{Cite journal | doi = 10.2165/00128071-200203090-00004 | pmid = 12444804 | year = 2002 | last1 = Trüeb | first1 = RM | title = Causes and management of hypertrichosis | volume = 3 | issue = 9 | pages = 617–27 | journal = American journal of clinical dermatology}}</ref> The two distinct types of hypertrichosis are generalized hypertrichosis, which occurs over the entire body, and localized hypertrichosis, which is restricted to a certain area.<ref name="PMID12582385"/> Hypertrichosis can be either congenital (present at birth) or acquired later in life.<ref name="dermnet">{{cite web |url= http://dermnetnz.org/hair-nails-sweat/hypertrichosis.html |title= Hypertrichosis| last1= Ngan | first1= Vanessa | date= June 15, 2009 |work= DermNet NZ |publisher= New Zealand Dermatological Society Incorporated |accessdate=November 29, 2009}}</ref><ref name="diseasesoftheskin">{{cite book |title= Diseases of The Skin |last= Sutton |first= Richard L. |year= 1916 |publisher= C.V. Mosby Company |location= St. Louis |pages= 408, 705 |url= https://books.google.com/books?id=5PBY5xtLCWIC&pg=PA705&dq=Hypertrichosis#v=onepage&q=Hypertrichosis&f=false |accessdate= November 29, 2009}}</ref> The excess growth of hair occurs in areas of the skin with the exception of [[Androgenic hair|androgen-dependent]] hair of the pubic area, face, and axillary regions.<ref name="Andrews">{{Cite book | last1= James | first1= William | last2= Berger | first2= Timothy | last3= Elston | first3=Dirk | title = Andrews Diseases of the Skin: Clinical Dermatology | publisher = Saunders | year = 2005 | edition = 10 | pages = 769 | isbn = 0-7216-2921-0 | postscript= <!-- Bot inserted parameter. Either remove it; or change its value to "." for the cite to end in a ".", as necessary. -->{{inconsistent citations}}}}</ref>


Several circus sideshow performers in the 19th and early 20th centuries, such as [[Julia Pastrana]], had hypertrichosis.<ref name="juliapastrana"/> Many of them worked as [[Freak show|freaks]] and were promoted as having distinct human and animal traits.
Several circus sideshow performers in the 19th and early 20th centuries, such as [[Julia Pastrana]], had hypertrichosis.<ref name="juliapastrana">{{Cite journal | last1= Miles | first1= A. E. W. | last2= | first2= | title = Julia Pastrana: the bearded lady | journal = The Royal Society of Medicine | volume = 67 | issue = 2 | pages = 160–164 | date = February 1974 | pmid= 4595237 | pmc= 1645262 | id = 1645262 | postscript= <!-- Bot inserted parameter. Either remove it; or change its value to "." for the cite to end in a ".", as necessary. -->{{inconsistent citations}}}}</ref> Many of them worked as [[Freak show|freaks]] and were promoted as having distinct human and animal traits.


==Classification==
==Classification==
Two methods of classification are used for hypertrichosis.<ref name="diseasesoftheskin"/> One divides them into either generalized versus localized hypertrichosis, while the other divides them into congenital versus acquired.<ref name="PMID12582385"/><ref name=Book08/>
Two methods of classification are used for hypertrichosis.<ref name="diseasesoftheskin"/> One divides them into either generalized versus localized hypertrichosis, while the other divides them into congenital versus acquired.<ref name="PMID12582385"/><ref name=Book08>{{cite book |author1=Hordinsky, Maria K. |author2=Amy J. McMichael |title=Hair and Scalp Diseases: Medical, Surgical, and Cosmetic Treatments (Basic and Clinical Dermatology) |publisher=Informa Healthcare |location= |year=2008 |pages=221 |isbn=1-57444-822-6 |oclc= |doi= |accessdate=}}</ref>


===Congenital===
===Congenital===
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;Hypertrichosis lanuginosa
;Hypertrichosis lanuginosa
Congenital hypertrichosis lanuginosa is noticeable at birth, with the infant completely covered in thin [[lanugo]] hair.<ref name="dermnet"/> In normal circumstances, lanugo hair is shed before birth and replaced by [[vellus]] hair; however, in a person with congenital hypertrichosis lanuginosa, the lanugo hair remains after birth.<ref name="dermnet"/> The palms of the hands, soles of the feet, and mucous membranes are not affected.<ref name="PMID12582385"/> As the person ages, the lanugo hair may thin, leaving only limited areas of hypertrichosis.<ref name="congenitallanuginosa"/>
Congenital hypertrichosis lanuginosa is noticeable at birth, with the infant completely covered in thin [[lanugo]] hair.<ref name="dermnet"/> In normal circumstances, lanugo hair is shed before birth and replaced by [[vellus]] hair; however, in a person with congenital hypertrichosis lanuginosa, the lanugo hair remains after birth.<ref name="dermnet"/> The palms of the hands, soles of the feet, and mucous membranes are not affected.<ref name="PMID12582385"/> As the person ages, the lanugo hair may thin, leaving only limited areas of hypertrichosis.<ref name="congenitallanuginosa">{{cite web |url= http://emedicine.medscape.com/article/1072987-overview |title= Congenital Hypertrichosis Lanuginosa|last1= Taylor|first1= Sarah K |date= June 18, 2009 |work= Emedicine|publisher= Medscape |accessdate=December 4, 2009}}</ref>


;Generalized hypertrichosis
;Generalized hypertrichosis
Congenital generalized hypertrichosis causes males to exhibit excessive facial and upper body hair, whereas women exhibit less severe asymmetrical hair distribution.<ref name="PMID7663516"/> The palms, soles, and mucous membranes are not affected.<ref name="PMID12582385"/>
Congenital generalized hypertrichosis causes males to exhibit excessive facial and upper body hair, whereas women exhibit less severe asymmetrical hair distribution.<ref name="PMID7663516">{{Cite journal| last1 = Figuera | first1 = L.| last2 = Pandolfo | first2 = M.| last3 = Dunne | first3 = P.| last4 = Cantú |first4 = J.| last5 = Patel | first5 = P.| title = Mapping of the congenital generalized hypertrichosis locus to chromosome Xq24-q27.1|journal = Nature Genetics| volume = 10| issue = 2| pages = 202–207| year = 1995| pmid = 7663516| doi = 10.1038/ng0695-202}}</ref> The palms, soles, and mucous membranes are not affected.<ref name="PMID12582385"/>


;Terminal hypertrichosis
;Terminal hypertrichosis
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;Circumscribed hypertrichosis
;Circumscribed hypertrichosis
Congenital circumscribed hypertrichosis is associated with the presence of thick [[vellus hair]] on the upper extremities.<ref name="PMID8844750"/> Circumscribed signifies this type of hypertrichosis is restricted to certain parts of the body, in this case, the extensor surfaces of the upper extremities.<ref name="PMID8844750"/> Hairy elbow syndrome, a type of congenital circumscribed hypertrichosis, shows excessive growth on and around the elbows.<ref name="PMID8844750"/> This type of hypertrichosis is present at birth, becomes more prominent during aging, and regresses at [[puberty]].<ref name="PMID8844750"/>
Congenital circumscribed hypertrichosis is associated with the presence of thick [[vellus hair]] on the upper extremities.<ref name="PMID8844750">{{Cite journal| doi = 10.1111/j.1525-1470.1996.tb01245.x| pmid = 8844750| year = 1996| last1 = Escalonilla| first1 = P| last2 = Aguilar| first2 = A| last3 = Gallego| first3 = M| last4 = Piqué| first4 = E| last5 = Fariña| first5 = MC| last6 = Requena| first6 = L| title = A new case of hairy elbows syndrome (Hypertrichosis cubiti)|volume = 13| issue = 4| pages = 303–5| journal = Pediatric dermatology}}</ref> Circumscribed signifies this type of hypertrichosis is restricted to certain parts of the body, in this case, the extensor surfaces of the upper extremities.<ref name="PMID8844750"/> Hairy elbow syndrome, a type of congenital circumscribed hypertrichosis, shows excessive growth on and around the elbows.<ref name="PMID8844750"/> This type of hypertrichosis is present at birth, becomes more prominent during aging, and regresses at [[puberty]].<ref name="PMID8844750"/>


;Localized hypertrichosis
;Localized hypertrichosis
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;Nevoid hypertrichosis
;Nevoid hypertrichosis
Nevoid hypertrichosis may be present at birth or appear later in life.<ref name="dermnet"/> It features an isolated area of excessive terminal hair and is usually not related to any other diseases.<ref name="dermnet"/><ref name="PMID9198325"/>
Nevoid hypertrichosis may be present at birth or appear later in life.<ref name="dermnet"/> It features an isolated area of excessive terminal hair and is usually not related to any other diseases.<ref name="dermnet"/><ref name="PMID9198325">{{Cite journal | pmid = 9198325 | year = 1997 | last1 = Chang | first1 = SN | last2 = Hong | first2 = CE | last3 = Kim | first3 = DK | last4 = Park | first4 = WH | title = A case of multiple nevoid hypertrichosis | volume = 24 | issue = 5 | pages = 337–41 | journal = The Journal of dermatology | doi=10.1111/j.1346-8138.1997.tb02801.x}}</ref>


===Acquired===
===Acquired===
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;Hypertrichosis lanuginosa
;Hypertrichosis lanuginosa
Acquired hypertrichosis lanuginosa is characterized by rapid growth of lanugo hair, particularly on the face.<ref name="acquiredlanugo2"/><ref name="hypertrichosislanuginosaacquisita"/> Hair also appears on the trunk and armpits, while palms and soles are unaffected.<ref name="hypertrichosislanuginosaacquisita"/> The excess hair is commonly referred to as malignant down.<ref name="hypertrichosislanuginosaacquisita"/> This hair is very fine and unpigmented.<ref name="hypertrichosislanuginosaacquisita"/>
Acquired hypertrichosis lanuginosa is characterized by rapid growth of lanugo hair, particularly on the face.<ref name="acquiredlanugo2">{{cite journal |last1=Samson |first1=Michael K. |last2=Buroker |first2=Thomas R. |last3=Henderson |first3=Michael D. |last4=Baker |first4=Laurence H. |last5=Vaitkevicius |first5=Vainutis K. |year=2006 |title=Acquired hypertrichosis lanuginosa |journal=Cancer |volume=36 |pmid=1100223 |issue=4 |pages=1519–1521 |url=http://www3.interscience.wiley.com/journal/112680525/abstract?CRETRY=1&SRETRY=0 |doi= 10.1002/1097-0142(197510)36:4<1519::AID-CNCR2820360446>3.0.CO;2-H }}</ref><ref name="hypertrichosislanuginosaacquisita">{{cite web |url= http://www.dermnetnz.org/hair-nails-sweat/hypertrichosis-lanuginosa.html|title= Hypertrichosis lanuginosa acquisita| last1= Ngan | first1= Vanessa | date= June 15, 2009 |work= DermNet NZ |publisher= New Zealand Dermatological Society Incorporated |accessdate=December 6, 2009}}</ref> Hair also appears on the trunk and armpits, while palms and soles are unaffected.<ref name="hypertrichosislanuginosaacquisita"/> The excess hair is commonly referred to as malignant down.<ref name="hypertrichosislanuginosaacquisita"/> This hair is very fine and unpigmented.<ref name="hypertrichosislanuginosaacquisita"/>


;Generalized hypertrichosis
;Generalized hypertrichosis
Acquired generalized hypertrichosis commonly affects the cheeks, upper lip, and chin.<ref name="dermnet"/><ref name="diseasesoftheskin"/> This form also affects the forearms and legs, but is less common in these areas. Another deformity associated with acquired generalized hypertrichosis is multiple hairs occupying the same follicle. It may also include abnormal hair growth patterns as what happens to the eyelashes in a condition known as [[trichiasis]].<ref name="diseasesoftheskin"/> Oral [[minoxidil]] treatments for [[hypertension]] are known to cause this condition. Topical minoxidil used for [[alopecia]] causes hair growth in the areas where it is applied, however this hair disappears shortly after discontinuing the use of topical minoxidil.<ref name="minoxidil"/>
Acquired generalized hypertrichosis commonly affects the cheeks, upper lip, and chin.<ref name="dermnet"/><ref name="diseasesoftheskin"/> This form also affects the forearms and legs, but is less common in these areas. Another deformity associated with acquired generalized hypertrichosis is multiple hairs occupying the same follicle. It may also include abnormal hair growth patterns as what happens to the eyelashes in a condition known as [[trichiasis]].<ref name="diseasesoftheskin"/> Oral [[minoxidil]] treatments for [[hypertension]] are known to cause this condition. Topical minoxidil used for [[alopecia]] causes hair growth in the areas where it is applied, however this hair disappears shortly after discontinuing the use of topical minoxidil.<ref name="minoxidil">{{Cite journal | doi = 10.1111/j.1365-2230.1994.tb01147.x | last = Gonzales | first = M. | last2 = Landa | first2 = N. | last3 = Gardeazabal | first3 = J. | last4 = Calseron | first4 = M.J. | last5 = Nilbao | first5 = I. | last6 = Perez | first6 = J. L. Diaz | title = Clinical and Experimental Dermatology | journal = British Association of Dermatologists Journal | volume = 19 | issue = 2 | pages = 157–158 | date = April 27, 2006 | url = http://www3.interscience.wiley.com/journal/119275626/abstract | id = 119275626 | postscript = <!-- Bot inserted parameter. Either remove it; or change its value to "." for the cite to end in a ".", as necessary. -->{{inconsistent citations}}}}</ref>


;Patterned hypertrichosis
;Patterned hypertrichosis
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;Localized hypertrichosis
;Localized hypertrichosis
Acquired localized hypertrichosis is an increase in hair density and length often secondary to irritation or trauma.<ref name="Fitz2"/> This form is restricted to certain areas of the body.
Acquired localized hypertrichosis is an increase in hair density and length often secondary to irritation or trauma.<ref name="Fitz2">{{cite book |title= Fitzpatrick's Dermatology in General Medicine |last= Freedberg |edition= 6 |year= 2003 |publisher= McGraw-Hill |isbn= 0-07-138076-0 |page= 651 }}</ref> This form is restricted to certain areas of the body.


===Hirsutism===
===Hirsutism===


{{Main|Hirsutism}}
{{Main|Hirsutism}}
Hypertrichosis is often mistakenly classified as hirsutism.<ref name="PMID12582385"/> Hirsutism is a type of hypertrichosis exclusive to women and children, resulting from an excess of [[androgen]]-sensitive hair growth.<ref name="hirsutism"/> Patients with hirsutism exhibit patterns of adult male hair growth.<ref name="PMID12582385"/> Chest and back hair are often present on women with hirsutism.<ref name="hirsutism"/>
Hypertrichosis is often mistakenly classified as hirsutism.<ref name="PMID12582385"/> Hirsutism is a type of hypertrichosis exclusive to women and children, resulting from an excess of [[androgen]]-sensitive hair growth.<ref name="hirsutism">{{Cite web | last = Ehrlich | first = Steven| title = Hirsutism | date = August 26, 2008 | url = http://www.umm.edu/altmed/articles/hirsutism-000081.htm | accessdate = December 16, 2009 | postscript = <!-- Bot inserted parameter. Either remove it; or change its value to "." for the cite to end in a ".", as necessary. -->{{inconsistent citations}} }}</ref> Patients with hirsutism exhibit patterns of adult male hair growth.<ref name="PMID12582385"/> Chest and back hair are often present on women with hirsutism.<ref name="hirsutism"/>


Hirsutism is both congenital and acquired. It is linked to excessive male hormones in women, thus symptoms may include acne, deepening of the voice, irregular menstrual periods, and the formation of a more masculine body shape.<ref name="hirsutism"/> Increases in androgen (male hormone) levels are the primary cause of most hirsutism cases.<ref name="PMID18081798"/> If caused by increased levels of androgens, it can be treated with medications that reduce androgen levels. Some birth control pills and [[spironolactone]] reduce androgen levels.<ref name="hirsutism"/>
Hirsutism is both congenital and acquired. It is linked to excessive male hormones in women, thus symptoms may include acne, deepening of the voice, irregular menstrual periods, and the formation of a more masculine body shape.<ref name="hirsutism"/> Increases in androgen (male hormone) levels are the primary cause of most hirsutism cases.<ref name="PMID18081798">{{Cite journal | doi = 10.1111/j.1742-1241.2007.01621.x | pmid = 18081798 | title = Hirsutism | year = 2008 | last1 = Mofid | first1 = A. | last2 = Seyyed Alinaghi |first2 = S. A. | last3 = Zandieh | first3 = S. | last4 = Yazdani | first4 = T. | journal = International Journal of Clinical Practice | volume = 62 | issue = 3 |pages = 433–43}}</ref> If caused by increased levels of androgens, it can be treated with medications that reduce androgen levels. Some birth control pills and [[spironolactone]] reduce androgen levels.<ref name="hirsutism"/>


==Signs and symptoms==
==Signs and symptoms==
The primary characteristic of all forms of hypertrichosis is excessive hair. Hair in hypertrichosis is usually longer than expected<ref name="Book08"/> and may consist of any hair type ([[lanugo]], [[vellus hair|vellus]], or [[Terminal hair|terminal]]).<ref name="FP09"/> Patterned forms of hypertrichosis cause hair growth in patterns. Generalized forms of hypertrichosis result in hair growth over the entire body. Circumscribed and localized forms lead to hair growth restricted to a certain area.
The primary characteristic of all forms of hypertrichosis is excessive hair. Hair in hypertrichosis is usually longer than expected<ref name="Book08"/> and may consist of any hair type ([[lanugo]], [[vellus hair|vellus]], or [[Terminal hair|terminal]]).<ref name=FP09>{{cite book |author1=Johnson, Richard R. |author2=Wolff, Klaus Dieter |title=Fitzpatrick's Color Atlas and Synopsis of Clinical Dermatology: Sixth Edition (Fitzpatrick's Color Atlas & Synopsis of Clinical Dermatology) |publisher=McGraw-Hill Professional |location= |year=2009 |pages=Part 4 Section 32 |isbn=0-07-159975-4 |oclc= |doi= |accessdate=}}</ref> Patterned forms of hypertrichosis cause hair growth in patterns. Generalized forms of hypertrichosis result in hair growth over the entire body. Circumscribed and localized forms lead to hair growth restricted to a certain area.


==Cause==
==Cause==
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;Hypertrichosis lanuginosa
;Hypertrichosis lanuginosa


Congenital hypertrichosis lanuginosa may be caused by an paracentric inversion mutation of the q22 band of chromosome 8;<ref name="PMID12582385"/> however, it could also be possibly the result of a spontaneous genetic mutation rather than inheritance.<ref name="congenitallanuginosa"/> This form is an autosomal dominant (not located on the sex chromosomes) cutaneous disorder, that affects the skin.<ref name="lanuginosacongenita1"/>
Congenital hypertrichosis lanuginosa may be caused by an paracentric inversion mutation of the q22 band of chromosome 8;<ref name="PMID12582385"/> however, it could also be possibly the result of a spontaneous genetic mutation rather than inheritance.<ref name="congenitallanuginosa"/> This form is an autosomal dominant (not located on the sex chromosomes) cutaneous disorder, that affects the skin.<ref name="lanuginosacongenita1">{{Cite journal | last = Mendiratta | first = Vibhu | last2 = Harjai | first2 = Bhawna | last3 = Gupta | first3 = Tanvi | title = Pediatric Dermatology | place = Lady Hardinge Medical College, Associated Shrimati Sucheta Kriplani and Kalawati Saran Children's Hospital, New Delhi, India | publisher = Wiley Periodicals, Inc. | pages = 483–4 | year = 2008 | volume = 25 | journal = Pediatric Dermatology | edition = 4 | postscript = <!-- Bot inserted parameter. Either remove it; or change its value to "." for the cite to end in a ".", as necessary. -->{{inconsistent citations}} | pmid=18789097 | issue=4 | doi = 10.1111/j.1525-1470.2008.00716.x}}</ref>


;Generalized hypertrichosis
;Generalized hypertrichosis


Congenital generalized hypertrichosis has a dominant pattern of inheritance and has been linked to chromosome Xq24-27.1.<ref name="PMID12582385"/><ref name="PMID6698556"/> An affected female (carrying the hypertrichosis gene) has a 50% chance of passing it to her offspring. An affected male will pass this form of hypertrichosis to his daughters, but never the sons.
Congenital generalized hypertrichosis has a dominant pattern of inheritance and has been linked to chromosome Xq24-27.1.<ref name="PMID12582385"/><ref name="PMID6698556">{{Cite journal| doi = 10.1007/BF00275189| pmid = 6698556| year = 1984| last1 = Macías-Flores| first1 = MA| last2 = García-Cruz| first2 = D|last3 = Rivera| first3 = H|last4 = Escobar-Luján| first4 = M| last5 = Melendrez-Vega| first5 = A| last6 = Rivas-Campos| first6 = D| last7 = Rodríguez-Collazo| first7 = F| last8 = Moreno-Arellano| first8 = I| last9 = Cantú| first9 = JM| displayauthors = 8| title = A New Form of Hypertrichosis Inherited as an X-linked Dominant Trait| volume = 66| issue = 1| pages = 66–70| journal = Human Genetics}}</ref> An affected female (carrying the hypertrichosis gene) has a 50% chance of passing it to her offspring. An affected male will pass this form of hypertrichosis to his daughters, but never the sons.
;Generalized hypertrichosis terminalis
;Generalized hypertrichosis terminalis


Congenital generalized hypertrichosis terminalis is thought to be caused by genetic changes on [[chromosome 17]] resulting in the addition or removal of millions of [[nucleotide]]s.<ref name="PMID19463983"/> The gene [[MAP2K6]] may be a factor contributing to this condition. This condition may also be due to the change in the chromosome affecting the [[transcription (biology)|transcription]] of genes.<ref name="newscientist"/>
Congenital generalized hypertrichosis terminalis is thought to be caused by genetic changes on [[chromosome 17]] resulting in the addition or removal of millions of [[nucleotide]]s.<ref name="PMID19463983">{{Cite journal| doi = 10.1016/j.ajhg.2009.04.018| pmid = 19463983| year = 2009| last1 = Sun| first1 = M.| last2 = Li| first2 = N.| last3 = Dong| first3 = W.| last4 = Chen| first4 = Z.| last5 = Liu| first5 = Q.| last6 = Xu| first6 = Y.| last7 = He| first7 = G.| last8 = Shi| first8 = Y.| last9 = Li| first9 = X.| last10 = Hao| first10 = Jiajie| last11 = Luo| first11 = Yang| last12 = Shang| first12 = Dandan| last13 = Lv| first13 = Dan| last14 = Ma| first14 = Fen| last15 = Zhang| first15 = Dai| last16 = Hua| first16 = Rui| last17 = Lu| first17 = Chaoxia| last18 = Wen| first18 = Yaran| last19 = Cao| first19 = Lihua| last20 = Irvine| first20 = Alan D.| last21 = McLean| first21 = W.H. Irwin| last22 = Dong| first22 = Qi| last23 = Wang| first23 = Ming-Rong| last24 = Yu| first24 = Jun| last25 = He| first25 = Lin| last26 = Lo| first26 = Wilson H.Y.| last27 = Zhang| first27 = Xue| title = Copy-Number Mutations on Chromosome 17q24.2-q24.3 in Congenital Generalized Hypertrichosis Terminalis with or without Gingival Hyperplasia| volume = 84| issue = 6| pages = 807–813| pmc = 2694973| journal = American Journal of Human Genetics| display-authors = 8}}</ref> The gene [[MAP2K6]] may be a factor contributing to this condition. This condition may also be due to the change in the chromosome affecting the [[transcription (biology)|transcription]] of genes.<ref name="newscientist">{{cite news |title= "Wolf Man" Condition Down To Huge DNA Malfunction |author= Ewen Callaway |newspaper= NewScientist |date= May 21, 2009|url= https://www.newscientist.com/article/dn17176-wolf-man-condition-down-to-huge-dna-malfunction.html |accessdate= November 29, 2009}}</ref>


'''Other hypertrichosis patterns'''
'''Other hypertrichosis patterns'''
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===Medical conditions===
===Medical conditions===
Acquired hypertrichosis lanuginosa is commonly present with cancer.<ref name="acquiredlanugo"/> This condition is also linked to metabolic disorders, such as [[Anorexia nervosa|anorexia]], hormone imbalances, such as [[hyperthyroidism]], or as a side effect of certain drugs.<ref name="acquiredlanugo"/>
Acquired hypertrichosis lanuginosa is commonly present with cancer.<ref name="acquiredlanugo">{{cite journal |last1=Slee |first1=Peter H.Th.J. |last2=Verzijlbergen |first2=Fred J. |last3=Schagen van Leeuwen |first3=Jules H. |last4=van der Waal |first4=Rutger I.F. |year=2006 |title=CASE 2. Acquired Hypertrichosis: A Rare Paraneoplastic Syndrome in Various Cancers |journal=Journal of Clinical Oncology |volume=24 |pmid=16421430 |issue=3 |pages=523–524 |url=http://jco.ascopubs.org/cgi/content/full/24/3/523 |doi= 10.1200/JCO.2005.01.2443
}}</ref> This condition is also linked to metabolic disorders, such as [[Anorexia nervosa|anorexia]], hormone imbalances, such as [[hyperthyroidism]], or as a side effect of certain drugs.<ref name="acquiredlanugo"/>


Acquired generalized hypertrichosis may be caused by cancer. The resulting hair growth is known as malignant down. The mechanism behind cancer induced hypertrichosis is unknown.<ref name="dermnet"/> Oral and topical [[minoxidil]] treatments are also known to cause acquired generalized hypertrichosis.<ref name="minoxidil"/>
Acquired generalized hypertrichosis may be caused by cancer. The resulting hair growth is known as malignant down. The mechanism behind cancer induced hypertrichosis is unknown.<ref name="dermnet"/> Oral and topical [[minoxidil]] treatments are also known to cause acquired generalized hypertrichosis.<ref name="minoxidil"/>
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{{See also|Hair removal}}
{{See also|Hair removal}}


There is no cure for any congenital forms of hypertrichosis. The treatment for acquired hypertrichosis is based on attempting to address the underlying cause.<ref name=FP09/> 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.<ref name="hairremovaltreatments"/> Hair removal treatments are categorized into two principal subdivisions: temporary removal and permanent removal.<ref name="hairremovaltreatments"/> Treatment may have adverse effects by causing scarring, [[dermatitis]], or [[hypersensitivity]].<ref name="dermnet"/>
There is no cure for any congenital forms of hypertrichosis. The treatment for acquired hypertrichosis is based on attempting to address the underlying cause.<ref name=FP09/> 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.<ref name="hairremovaltreatments">{{cite web |url= http://dermnetnz.org/procedures/hair-removal.html |title= Hair removal techniques|last1= Ngan |first1= Vanessa |date= June 15, 2009 |work= DermNet NZ |publisher= New Zealand Dermatological Society Incorporated |accessdate=November 29, 2009}}</ref> Hair removal treatments are categorized into two principal subdivisions: temporary removal and permanent removal.<ref name="hairremovaltreatments"/> Treatment may have adverse effects by causing scarring, [[dermatitis]], or [[hypersensitivity]].<ref name="dermnet"/>


Temporary hair removal may last from several hours to several weeks, depending on the method used. These procedures are purely cosmetic.<ref name="PMID12444804"/> Depilation methods, such as trimming, [[shaving]], and [[Chemical depilatory|depilatories]], remove hair to the level of the skin and produce results that last several hours to several days.<ref name="Epilation"/> Epilation methods, such as [[Plucking (hair removal)|plucking]], [[electrology]], [[waxing]], [[Sugaring (epilation)|sugaring]], [[Threading (epilation)|threading]] remove the entire hair from the root, the results lasting several days to several weeks.<ref name="Epilation"/>
Temporary hair removal may last from several hours to several weeks, depending on the method used. These procedures are purely cosmetic.<ref name="PMID12444804"/> Depilation methods, such as trimming, [[shaving]], and [[Chemical depilatory|depilatories]], remove hair to the level of the skin and produce results that last several hours to several days.<ref name="Epilation">{{cite web |url= http://dermnetnz.org/procedures/epilation.html |title= Epilation|last1= Ngan |first1= Vanessa |date= June 15, 2009 |work= DermNet NZ |publisher= New Zealand Dermatological Society Incorporated |accessdate=November 29, 2009}}</ref> Epilation methods, such as [[Plucking (hair removal)|plucking]], [[electrology]], [[waxing]], [[Sugaring (epilation)|sugaring]], [[Threading (epilation)|threading]] remove the entire hair from the root, the results lasting several days to several weeks.<ref name="Epilation"/>


Permanent hair removal uses chemicals, energy of various types, or a combination to target the cells that cause hair growth. [[Laser hair removal]] is an effective method of hair removal on hairs that have color. Laser cannot treat white hair. The laser targets the melanin color in the lower 1/3 of the hair follicle, which is the target zone.<ref name="PMID12444804"/> [[Electrology|Electrolysis (electrology)]] uses electrical current, and/or localized heating. The U.S. [[Food and Drug Administration]] (FDA) allows only electrology to use the term "permanent hair removal" because it has been shown to be able treat all colors of hair.
Permanent hair removal uses chemicals, energy of various types, or a combination to target the cells that cause hair growth. [[Laser hair removal]] is an effective method of hair removal on hairs that have color. Laser cannot treat white hair. The laser targets the melanin color in the lower 1/3 of the hair follicle, which is the target zone.<ref name="PMID12444804"/> [[Electrology|Electrolysis (electrology)]] uses electrical current, and/or localized heating. The U.S. [[Food and Drug Administration]] (FDA) allows only electrology to use the term "permanent hair removal" because it has been shown to be able treat all colors of hair.


Medication to reduce production of hair is currently under testing. One medicinal option suppresses testosterone by increasing the sex hormone-binding globulin.<ref name="medication"/> Another controls the overproduction of hair through the regulation of a luteinizing hormone.<ref name="medication"/>
Medication to reduce production of hair is currently under testing. One medicinal option suppresses testosterone by increasing the sex hormone-binding globulin.<ref name="medication">{{Cite journal|last=Clark |first=Jennifer |title=Hirsutism May Signal Uderlying Disorder |journal=Dermatology Times |volume=27 |issue=10 |pages=60–61 |date=October 2006 |url=http://dermatologytimes.modernmedicine.com/dermatologytimes/issue/issueDetail.jsp?id=10462 |postscript={{inconsistent citations}} |deadurl=yes |archiveurl=https://web.archive.org/web/20111007072523/http://dermatologytimes.modernmedicine.com/dermatologytimes/issue/issueDetail.jsp?id=10462 |archivedate=2011-10-07 |df= }}</ref> Another controls the overproduction of hair through the regulation of a luteinizing hormone.<ref name="medication"/>


==Epidemiology==
==Epidemiology==
Congenital forms of hypertrichosis are rare. Only 50 cases of congenital hypertrichosis lanuginosa have been recorded since the Middle Ages,<ref name="dermnet"/> and fewer than 100 cases of congenital generalized hypertrichosis have been documented in scientific publications and by the media.<ref name="newscientist"/> Congenital generalized hypertrichosis is isolated to one family in Mexico.<ref name="PMID12582385"/> Acquired hypertrichosis and hirsutism are more common.<ref name="diseasesoftheskin"/><ref name="definehirsutism"/> For example, hirsutism occurs in about 10% of women between ages 18 and 45.<ref name="definehirsutism"/>
Congenital forms of hypertrichosis are rare. Only 50 cases of congenital hypertrichosis lanuginosa have been recorded since the Middle Ages,<ref name="dermnet"/> and fewer than 100 cases of congenital generalized hypertrichosis have been documented in scientific publications and by the media.<ref name="newscientist"/> Congenital generalized hypertrichosis is isolated to one family in Mexico.<ref name="PMID12582385"/> Acquired hypertrichosis and hirsutism are more common.<ref name="diseasesoftheskin"/><ref name="definehirsutism">{{cite web|url=http://www.hirsutism.info/hirsutism.html |title=Definition of Hirsutism |last1=Mueller |first1=Hans |date=June 20, 2006 |work=Hirsutism.info |publisher=Cirkuit Networks, Inc. |accessdate=December 31, 2009 |deadurl=yes |archiveurl=https://web.archive.org/web/20100311165126/http://www.hirsutism.info/hirsutism.html |archivedate=March 11, 2010 |df= }}</ref> For example, hirsutism occurs in about 10% of women between ages 18 and 45.<ref name="definehirsutism"/>


==Society and culture==
==Society and culture==
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[[File:Alice Teennager.JPG|thumb|Alice E. Doherty]]
[[File:Alice Teennager.JPG|thumb|Alice E. Doherty]]
[[File:South London Palace (Hairy Man and His Son) Wellcome L0032823.jpg|thumb|1874 London advertisement]]
[[File:South London Palace (Hairy Man and His Son) Wellcome L0032823.jpg|thumb|1874 London advertisement]]
People with hair often found jobs as circus performers, barbers, making the best of their unusual appearance. [[Fedor Jeftichew]] ("Jo-Jo the Dog-faced Man"), [[Stephan Bibrowski]] ("Lionel the Lion-faced Man"), [[Jesús Aceves|Jesús "Chuy" Aceves]] ("Wolfman"), [[Annie Jones (bearded woman)|Annie Jones]] ("the bearded woman") and [[Alice Elizabeth Doherty]] ("The Minnesota Woolly Girl") all had hypertrichosis.<ref name="congenitallanuginosa"/> Extensive hypertrichosis carries an emotional burden and can cause cosmetic embarrassment; however, some people attempt no treatments because they say it defines who they are.<ref name="PMID12444804"/><ref name="society"/>
People with hair often found jobs as circus performers, barbers, making the best of their unusual appearance. [[Fedor Jeftichew]] ("Jo-Jo the Dog-faced Man"), [[Stephan Bibrowski]] ("Lionel the Lion-faced Man"), [[Jesús Aceves|Jesús "Chuy" Aceves]] ("Wolfman"), [[Annie Jones (bearded woman)|Annie Jones]] ("the bearded woman") and [[Alice Elizabeth Doherty]] ("The Minnesota Woolly Girl") all had hypertrichosis.<ref name="congenitallanuginosa"/> Extensive hypertrichosis carries an emotional burden and can cause cosmetic embarrassment; however, some people attempt no treatments because they say it defines who they are.<ref name="PMID12444804"/><ref name="society">{{cite news |title= Hypertrichosis - Real Life Werewolves? |author1=John Quinones |author2=Laura Viddy |author3=Cecile Bouchardeau |newspaper= ABC News |date= September 12, 2007 |url= http://abcnews.go.com/Primetime/story?id=2258069&page=1 |accessdate= September 20, 2009}}</ref>


===Julia Pastrana===
===Julia Pastrana===
Line 149: Line 150:


===The hairy family of Burma===
===The hairy family of Burma===
One 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]].<ref name="The hairy family of burma"/><ref name="Hairy family of Burma High-Res Glass Slide"/> He tells of meeting a hairy man, Shwe-Maong. Shwe-Maong lived in the court of King Ava and acted as an entertainer.<ref name="The hairy family of burma"/> Shwe-Maong had four children: three unaffected children, and one child with congenital hypertrichosis, named Maphoon.<ref name="The hairy family of burma"/> On a second mission to Ava, Maphoon was described as a thirty-year-old woman with two sons, one of which had hypertrichosis.<ref name="The hairy family of burma"/> The affected son was named Maong-Phoset. He had an affected daughter named Mah-Me.<ref name="The hairy family of burma"/> Whereas all affected members of the family had dental problems, the unaffected members had perfect teeth.<ref name="The hairy family of burma"/>
One 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]].<ref name="The hairy family of burma">{{Cite journal | last1= Bondeson | first1= J | last2= Miles | first2= A | title = The hairy family of Burma: a four generation pedigree of congenital hypertrichosis lanuginosa | journal = The Royal Society of Medicine | volume = 89 | issue = 7 | pages = 403–408 | date = July 1996 | pmid= 8774541 | pmc= 1295857 | id = 1295857 | postscript= <!-- Bot inserted parameter. Either remove it; or change its value to "." for the cite to end in a ".", as necessary. -->{{inconsistent citations}}}}</ref><ref name="Hairy family of Burma High-Res Glass Slide">{{cite web |url= http://images.is.ed.ac.uk/luna/servlet/s/2y8713|title= Hi-res glass slide of Hairy Family of Burma|publisher= University of Edinburgh |date=October 31, 2014}}</ref> He tells of meeting a hairy man, Shwe-Maong. Shwe-Maong lived in the court of King Ava and acted as an entertainer.<ref name="The hairy family of burma"/> Shwe-Maong had four children: three unaffected children, and one child with congenital hypertrichosis, named Maphoon.<ref name="The hairy family of burma"/> On a second mission to Ava, Maphoon was described as a thirty-year-old woman with two sons, one of which had hypertrichosis.<ref name="The hairy family of burma"/> The affected son was named Maong-Phoset. He had an affected daughter named Mah-Me.<ref name="The hairy family of burma"/> Whereas all affected members of the family had dental problems, the unaffected members had perfect teeth.<ref name="The hairy family of burma"/>


==History==
==History==
The first recorded case of hypertrichosis was [[Petrus Gonsalvus]] of the Canary Islands.<ref name="congenitallanuginosa"/> This was documented by Altrovandus in mid 17th century and published in his text Monstrorum Historia cum Paralipomenis historiae omnium animalium published in 1642.<ref name="congenitallanuginosa"/><ref name="ambras syndrome"/> He noted two daughters, a son, and a grandchild in Gonsalvus' family all had hypertrichosis. Altrovandus dubbed them the Ambras family, after the [[Ambras Castle]] near [[Innsbruck]], where portraits of the family were found.<ref name="congenitallanuginosa"/> During the next 300 years, about 50 cases were observed. The scientist Rudolf Virchow described a form of hypertrichosis accompanied by [[Gingival enlargement|gingival hyperplasia]] in 1873.<ref name="congenitallanuginosa"/>
The first recorded case of hypertrichosis was [[Petrus Gonsalvus]] of the Canary Islands.<ref name="congenitallanuginosa"/> This was documented by Altrovandus in mid 17th century and published in his text Monstrorum Historia cum Paralipomenis historiae omnium animalium published in 1642.<ref name="congenitallanuginosa"/><ref name="ambras syndrome">{{cite journal |last1= Rashid|first1=Rashid M. |last2=White |first2=Lucile E. |year=2007 |title=A hairy development in hypertrichosis: a brief review of Ambras syndrome |journal=Dermatology Online Journal |volume=13 |issue=3 |url=http://dermatology.cdlib.org/133/reviews/ambras/rashid.html |pmid=18328202 |pages= 8}}</ref> He noted two daughters, a son, and a grandchild in Gonsalvus' family all had hypertrichosis. Altrovandus dubbed them the Ambras family, after the [[Ambras Castle]] near [[Innsbruck]], where portraits of the family were found.<ref name="congenitallanuginosa"/> During the next 300 years, about 50 cases were observed. The scientist Rudolf Virchow described a form of hypertrichosis accompanied by [[Gingival enlargement|gingival hyperplasia]] in 1873.<ref name="congenitallanuginosa"/>


==In animals==
==In animals==
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{{columns-list|2|
{{columns-list|2|
{{Reflist|refs=
{{Reflist|refs=
<ref name="acquiredlanugo"/>
<ref name="acquiredlanugo">{{cite journal |last1=Slee |first1=Peter H.Th.J. |last2=Verzijlbergen |first2=Fred J. |last3=Schagen van Leeuwen |first3=Jules H. |last4=van der Waal |first4=Rutger I.F. |year=2006 |title=CASE 2. Acquired Hypertrichosis: A Rare Paraneoplastic Syndrome in Various Cancers |journal=Journal of Clinical Oncology |volume=24 |pmid=16421430 |issue=3 |pages=523–524 |url=http://jco.ascopubs.org/cgi/content/full/24/3/523 |doi= 10.1200/JCO.2005.01.2443
<ref name="PMID19463983"/>
}}</ref>
<ref name="acquiredlanugo2"/>
<ref name="PMID19463983">{{Cite journal| doi = 10.1016/j.ajhg.2009.04.018| pmid = 19463983| year = 2009| last1 = Sun| first1 = M.| last2 = Li| first2 = N.| last3 = Dong| first3 = W.| last4 = Chen| first4 = Z.| last5 = Liu| first5 = Q.| last6 = Xu| first6 = Y.| last7 = He| first7 = G.| last8 = Shi| first8 = Y.| last9 = Li| first9 = X.| last10 = Hao| first10 = Jiajie| last11 = Luo| first11 = Yang| last12 = Shang| first12 = Dandan| last13 = Lv| first13 = Dan| last14 = Ma| first14 = Fen| last15 = Zhang| first15 = Dai| last16 = Hua| first16 = Rui| last17 = Lu| first17 = Chaoxia| last18 = Wen| first18 = Yaran| last19 = Cao| first19 = Lihua| last20 = Irvine| first20 = Alan D.| last21 = McLean| first21 = W.H. Irwin| last22 = Dong| first22 = Qi| last23 = Wang| first23 = Ming-Rong| last24 = Yu| first24 = Jun| last25 = He| first25 = Lin| last26 = Lo| first26 = Wilson H.Y.| last27 = Zhang| first27 = Xue| title = Copy-Number Mutations on Chromosome 17q24.2-q24.3 in Congenital Generalized Hypertrichosis Terminalis with or without Gingival Hyperplasia| volume = 84| issue = 6| pages = 807–813| pmc = 2694973| journal = American Journal of Human Genetics| display-authors = 8}}</ref>
<ref name="dermnet"/>
<ref name="acquiredlanugo2">{{cite journal |last1=Samson |first1=Michael K. |last2=Buroker |first2=Thomas R. |last3=Henderson |first3=Michael D. |last4=Baker |first4=Laurence H. |last5=Vaitkevicius |first5=Vainutis K. |year=2006 |title=Acquired hypertrichosis lanuginosa |journal=Cancer |volume=36 |pmid=1100223 |issue=4 |pages=1519–1521 |url=http://www3.interscience.wiley.com/journal/112680525/abstract?CRETRY=1&SRETRY=0 |doi= 10.1002/1097-0142(197510)36:4<1519::AID-CNCR2820360446>3.0.CO;2-H }}</ref>
<ref name="PMID9198325"/>
<ref name="dermnet">{{cite web |url= http://dermnetnz.org/hair-nails-sweat/hypertrichosis.html |title= Hypertrichosis| last1= Ngan | first1= Vanessa | date= June 15, 2009 |work= DermNet NZ |publisher= New Zealand Dermatological Society Incorporated |accessdate=November 29, 2009}}</ref>
<ref name="PMID9198325">{{Cite journal | pmid = 9198325 | year = 1997 | last1 = Chang | first1 = SN | last2 = Hong | first2 = CE | last3 = Kim | first3 = DK | last4 = Park | first4 = WH | title = A case of multiple nevoid hypertrichosis | volume = 24 | issue = 5 | pages = 337–41 | journal = The Journal of dermatology | doi=10.1111/j.1346-8138.1997.tb02801.x}}</ref>


<ref name="Andrews"/>
<ref name="Andrews">{{Cite book | last1= James | first1= William | last2= Berger | first2= Timothy | last3= Elston | first3=Dirk | title = Andrews Diseases of the Skin: Clinical Dermatology | publisher = Saunders | year = 2005 | edition = 10 | pages = 769 | isbn = 0-7216-2921-0 | postscript= <!-- Bot inserted parameter. Either remove it; or change its value to "." for the cite to end in a ".", as necessary. -->{{inconsistent citations}}}}</ref>


<ref name="congenitallanuginosa"/>
<ref name="congenitallanuginosa">{{cite web |url= http://emedicine.medscape.com/article/1072987-overview |title= Congenital Hypertrichosis Lanuginosa|last1= Taylor|first1= Sarah K |date= June 18, 2009 |work= Emedicine|publisher= Medscape |accessdate=December 4, 2009}}</ref>
<ref name="diseasesoftheskin"/>
<ref name="diseasesoftheskin">{{cite book |title= Diseases of The Skin |last= Sutton |first= Richard L. |year= 1916 |publisher= C.V. Mosby Company |location= St. Louis |pages= 408, 705 |url= https://books.google.com/books?id=5PBY5xtLCWIC&pg=PA705&dq=Hypertrichosis#v=onepage&q=Hypertrichosis&f=false |accessdate= November 29, 2009}}</ref>


<ref name="lanuginosacongenita1"/>
<ref name="lanuginosacongenita1">{{Cite journal | last = Mendiratta | first = Vibhu | last2 = Harjai | first2 = Bhawna | last3 = Gupta | first3 = Tanvi | title = Pediatric Dermatology | place = Lady Hardinge Medical College, Associated Shrimati Sucheta Kriplani and Kalawati Saran Children's Hospital, New Delhi, India | publisher = Wiley Periodicals, Inc. | pages = 483–4 | year = 2008 | volume = 25 | journal = Pediatric Dermatology | edition = 4 | postscript = <!-- Bot inserted parameter. Either remove it; or change its value to "." for the cite to end in a ".", as necessary. -->{{inconsistent citations}} | pmid=18789097 | issue=4 | doi = 10.1111/j.1525-1470.2008.00716.x}}</ref>


<ref name="hypertrichosislanuginosaacquisita"/>
<ref name="hypertrichosislanuginosaacquisita">{{cite web |url= http://www.dermnetnz.org/hair-nails-sweat/hypertrichosis-lanuginosa.html|title= Hypertrichosis lanuginosa acquisita| last1= Ngan | first1= Vanessa | date= June 15, 2009 |work= DermNet NZ |publisher= New Zealand Dermatological Society Incorporated |accessdate=December 6, 2009}}</ref>
<ref name="PMID850811">{{Cite journal | pmid = 850811 | year = 1977 | last1 = Earhart | first1 = RN | last2 = Ball | first2 = J | last3 = Nuss | first3 = DD | last4 = Aeling | first4 = JL | title = Minoxidil-induced hypertrichosis: treatment with calcium thioglycolate depilatory | volume = 70 | issue = 4 | pages = 442–3| journal = Southern Medical Journal | doi = 10.1097/00007611-197704000-00023}}</ref>
<ref name="PMID850811">{{Cite journal | pmid = 850811 | year = 1977 | last1 = Earhart | first1 = RN | last2 = Ball | first2 = J | last3 = Nuss | first3 = DD | last4 = Aeling | first4 = JL | title = Minoxidil-induced hypertrichosis: treatment with calcium thioglycolate depilatory | volume = 70 | issue = 4 | pages = 442–3| journal = Southern Medical Journal | doi = 10.1097/00007611-197704000-00023}}</ref>


<ref name="PMID6698556"/>
<ref name="PMID6698556">{{Cite journal| doi = 10.1007/BF00275189| pmid = 6698556| year = 1984| last1 = Macías-Flores| first1 = MA| last2 = García-Cruz| first2 = D|last3 = Rivera| first3 = H|last4 = Escobar-Luján| first4 = M| last5 = Melendrez-Vega| first5 = A| last6 = Rivas-Campos| first6 = D| last7 = Rodríguez-Collazo| first7 = F| last8 = Moreno-Arellano| first8 = I| last9 = Cantú| first9 = JM| displayauthors = 8| title = A New Form of Hypertrichosis Inherited as an X-linked Dominant Trait| volume = 66| issue = 1| pages = 66–70| journal = Human Genetics}}</ref>


<ref name="PMID7663516"/>
<ref name="PMID7663516">{{Cite journal| last1 = Figuera | first1 = L.| last2 = Pandolfo | first2 = M.| last3 = Dunne | first3 = P.| last4 = Cantú |first4 = J.| last5 = Patel | first5 = P.| title = Mapping of the congenital generalized hypertrichosis locus to chromosome Xq24-q27.1|journal = Nature Genetics| volume = 10| issue = 2| pages = 202–207| year = 1995| pmid = 7663516| doi = 10.1038/ng0695-202}}</ref>
<ref name="PMID12582385"/>
<ref name="PMID12582385">{{Cite journal | doi = 10.1067/mjd.2003.100 | pmid = 12582385 | title = Hypertrichosis | year = 2003 | last1 = Wendelin | first1 = D. | last2= Pope| first2= D.| last3= Mallory| first3= S.| journal = Journal of the American Academy of Dermatology | volume = 48 | issue = 2 | pages = 161–179 }}</ref>
<ref name="PMID8844750"/>
<ref name="PMID8844750">{{Cite journal| doi = 10.1111/j.1525-1470.1996.tb01245.x| pmid = 8844750| year = 1996| last1 = Escalonilla| first1 = P| last2 = Aguilar| first2 = A| last3 = Gallego| first3 = M| last4 = Piqué| first4 = E| last5 = Fariña| first5 = MC| last6 = Requena| first6 = L| title = A new case of hairy elbows syndrome (Hypertrichosis cubiti)|volume = 13| issue = 4| pages = 303–5| journal = Pediatric dermatology}}</ref>


<ref name="Epilation"/>
<ref name="Epilation">{{cite web |url= http://dermnetnz.org/procedures/epilation.html |title= Epilation|last1= Ngan |first1= Vanessa |date= June 15, 2009 |work= DermNet NZ |publisher= New Zealand Dermatological Society Incorporated |accessdate=November 29, 2009}}</ref>


<ref name="hairremovaltreatments"/>
<ref name="hairremovaltreatments">{{cite web |url= http://dermnetnz.org/procedures/hair-removal.html |title= Hair removal techniques|last1= Ngan |first1= Vanessa |date= June 15, 2009 |work= DermNet NZ |publisher= New Zealand Dermatological Society Incorporated |accessdate=November 29, 2009}}</ref>


<ref name="minoxidil"/>
<ref name="minoxidil">{{Cite journal | doi = 10.1111/j.1365-2230.1994.tb01147.x | last = Gonzales | first = M. | last2 = Landa | first2 = N. | last3 = Gardeazabal | first3 = J. | last4 = Calseron | first4 = M.J. | last5 = Nilbao | first5 = I. | last6 = Perez | first6 = J. L. Diaz | title = Clinical and Experimental Dermatology | journal = British Association of Dermatologists Journal | volume = 19 | issue = 2 | pages = 157–158 | date = April 27, 2006 | url = http://www3.interscience.wiley.com/journal/119275626/abstract | id = 119275626 | postscript = <!-- Bot inserted parameter. Either remove it; or change its value to "." for the cite to end in a ".", as necessary. -->{{inconsistent citations}}}}</ref>


<ref name="The hairy family of burma"/>
<ref name="The hairy family of burma">{{Cite journal | last1= Bondeson | first1= J | last2= Miles | first2= A | title = The hairy family of Burma: a four generation pedigree of congenital hypertrichosis lanuginosa | journal = The Royal Society of Medicine | volume = 89 | issue = 7 | pages = 403–408 | date = July 1996 | pmid= 8774541 | pmc= 1295857 | id = 1295857 | postscript= <!-- Bot inserted parameter. Either remove it; or change its value to "." for the cite to end in a ".", as necessary. -->{{inconsistent citations}}}}</ref>


<ref name="Hairy family of Burma High-Res Glass Slide">{{cite web |url= http://images.is.ed.ac.uk/luna/servlet/s/2y8713|title= Hi-res glass slide of Hairy Family of Burma|publisher= University of Edinburgh |date=October 31, 2014}}</ref>
<ref name="Hairy family of Burma High-Res Glass Slide"/>


<ref name="newscientist"/>
<ref name="newscientist">{{cite news |title= "Wolf Man" Condition Down To Huge DNA Malfunction |author= Ewen Callaway |newspaper= NewScientist |date= May 21, 2009|url= https://www.newscientist.com/article/dn17176-wolf-man-condition-down-to-huge-dna-malfunction.html |accessdate= November 29, 2009}}</ref>


<ref name="ambras syndrome"/>
<ref name="ambras syndrome">{{cite journal |last1= Rashid|first1=Rashid M. |last2=White |first2=Lucile E. |year=2007 |title=A hairy development in hypertrichosis: a brief review of Ambras syndrome |journal=Dermatology Online Journal |volume=13 |issue=3 |url=http://dermatology.cdlib.org/133/reviews/ambras/rashid.html |pmid=18328202 |pages= 8}}</ref>


<ref name="society"/>
<ref name="society">{{cite news |title= Hypertrichosis - Real Life Werewolves? |author1=John Quinones |author2=Laura Viddy |author3=Cecile Bouchardeau |newspaper= ABC News |date= September 12, 2007 |url= http://abcnews.go.com/Primetime/story?id=2258069&page=1 |accessdate= September 20, 2009}}</ref>


<ref name="hirsutism"/>
<ref name="hirsutism">{{Cite web | last = Ehrlich | first = Steven| title = Hirsutism | date = August 26, 2008 | url = http://www.umm.edu/altmed/articles/hirsutism-000081.htm | accessdate = December 16, 2009 | postscript = <!-- Bot inserted parameter. Either remove it; or change its value to "." for the cite to end in a ".", as necessary. -->{{inconsistent citations}} }}</ref>


<ref name="juliapastrana"/>
<ref name="juliapastrana">{{Cite journal | last1= Miles | first1= A. E. W. | last2= | first2= | title = Julia Pastrana: the bearded lady | journal = The Royal Society of Medicine | volume = 67 | issue = 2 | pages = 160–164 | date = February 1974 | pmid= 4595237 | pmc= 1645262 | id = 1645262 | postscript= <!-- Bot inserted parameter. Either remove it; or change its value to "." for the cite to end in a ".", as necessary. -->{{inconsistent citations}}}}</ref>
<ref name="Fitz2"/>
<ref name="Fitz2">{{cite book |title= Fitzpatrick's Dermatology in General Medicine |last= Freedberg |edition= 6 |year= 2003 |publisher= McGraw-Hill |isbn= 0-07-138076-0 |page= 651 }}</ref>


<ref name="PMID12444804"/>
<ref name="PMID12444804">{{Cite journal | doi = 10.2165/00128071-200203090-00004 | pmid = 12444804 | year = 2002 | last1 = Trüeb | first1 = RM | title = Causes and management of hypertrichosis | volume = 3 | issue = 9 | pages = 617–27 | journal = American journal of clinical dermatology}}</ref>
<ref name="definehirsutism"/>
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Revision as of 15:45, 14 September 2017

Hypertrichosis
SpecialtyDermatology Edit this on Wikidata

Hypertrichosis (also called Ambras syndrome) is an abnormal amount of hair growth over the body.[1][2] The two distinct types of hypertrichosis are generalized hypertrichosis, which occurs over the entire body, and localized hypertrichosis, which is restricted to a certain area.[1] Hypertrichosis can be either congenital (present at birth) or acquired later in life.[3][4] The excess growth of hair occurs in areas of the skin with the exception of androgen-dependent hair of the pubic area, face, and axillary regions.[5]

Several circus sideshow performers in the 19th and early 20th centuries, such as Julia Pastrana, had hypertrichosis.[6] Many of them worked as freaks and were promoted as having distinct human and animal traits.

Classification

Two methods of classification are used for hypertrichosis.[4] One divides them into either generalized versus localized hypertrichosis, while the other divides them into congenital versus acquired.[1][7]

Congenital

Congenital forms of hypertrichosis are caused by genetic mutations, and are extremely rare, unlike acquired forms.[4] Congenital hypertrichosis is always present at birth.[3]


Hypertrichosis lanuginosa

Congenital hypertrichosis lanuginosa is noticeable at birth, with the infant completely covered in thin lanugo hair.[3] In normal circumstances, lanugo hair is shed before birth and replaced by vellus hair; however, in a person with congenital hypertrichosis lanuginosa, the lanugo hair remains after birth.[3] The palms of the hands, soles of the feet, and mucous membranes are not affected.[1] As the person ages, the lanugo hair may thin, leaving only limited areas of hypertrichosis.[8]

Generalized hypertrichosis

Congenital generalized hypertrichosis causes males to exhibit excessive facial and upper body hair, whereas women exhibit less severe asymmetrical hair distribution.[9] The palms, soles, and mucous membranes are not affected.[1]

Terminal hypertrichosis

Congenital terminal hypertrichosis is characterized by the presence of fully pigmented terminal hair that covers the entire body.[3] This condition is usually accompanied by gingival hyperplasia.[3] This form is most responsible for the term "werewolf syndrome" because of the thick, dark hair that appears.[3] People with this condition are sometimes performers at circuses because of their unusual appearance.[3]

Circumscribed hypertrichosis

Congenital circumscribed hypertrichosis is associated with the presence of thick vellus hair on the upper extremities.[10] Circumscribed signifies this type of hypertrichosis is restricted to certain parts of the body, in this case, the extensor surfaces of the upper extremities.[10] Hairy elbow syndrome, a type of congenital circumscribed hypertrichosis, shows excessive growth on and around the elbows.[10] This type of hypertrichosis is present at birth, becomes more prominent during aging, and regresses at puberty.[10]

Localized hypertrichosis

Congenital localized hypertrichosis is a localized increase in hair density and length.[4]

Nevoid hypertrichosis

Nevoid hypertrichosis may be present at birth or appear later in life.[3] It features an isolated area of excessive terminal hair and is usually not related to any other diseases.[3][11]

Acquired

Acquired hypertrichosis appears after birth. The multiple causes include the side effects of drugs, associations with cancer, and possible links with eating disorders. Acquired forms can usually be reduced with various treatments.

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 excess hair is commonly referred to as malignant down.[13] This hair is very fine and unpigmented.[13]

Generalized hypertrichosis

Acquired generalized hypertrichosis commonly affects the cheeks, upper lip, and chin.[3][4] This form also affects the forearms and legs, but is less common in these areas. Another deformity associated with acquired generalized hypertrichosis is multiple hairs occupying the same follicle. It may also include abnormal hair growth patterns as what happens to the eyelashes in a condition known as trichiasis.[4] Oral minoxidil treatments for hypertension are known to cause this condition. Topical minoxidil used for alopecia causes hair growth in the areas where it is applied, however this hair disappears shortly after discontinuing the use of topical minoxidil.[14]

Patterned hypertrichosis

Acquired patterned hypertrichosis is an increase in hair growth in a pattern formation. It is similar to acquired generalized hypertrichosis and is a sign of internal malignancy.[5]

Localized hypertrichosis

Acquired localized hypertrichosis is an increase in hair density and length often secondary to irritation or trauma.[15] This form is restricted to certain areas of the body.

Hirsutism

Hypertrichosis is often mistakenly classified as hirsutism.[1] Hirsutism is a type of hypertrichosis exclusive to women and children, resulting from an excess of androgen-sensitive hair growth.[16] Patients with hirsutism exhibit patterns of adult male hair growth.[1] Chest and back hair are often present on women with hirsutism.[16]

Hirsutism is both congenital and acquired. It is linked to excessive male hormones in women, thus symptoms may include acne, deepening of the voice, irregular menstrual periods, and the formation of a more masculine body shape.[16] Increases in androgen (male hormone) levels are the primary cause of most hirsutism cases.[17] If caused by increased levels of androgens, it can be treated with medications that reduce androgen levels. Some birth control pills and spironolactone reduce androgen levels.[16]

Signs and symptoms

The primary characteristic of all forms of hypertrichosis is excessive hair. Hair in hypertrichosis is usually longer than expected[7] and may consist of any hair type (lanugo, vellus, or terminal).[18] Patterned forms of hypertrichosis cause hair growth in patterns. Generalized forms of hypertrichosis result in hair growth over the entire body. Circumscribed and localized forms lead to hair growth restricted to a certain area.

Cause

Genetic

X Linked female recessive traits
Patterns of inheritance of females with sex linked forms of hypertrichosis
X Linked male dominant traits
Patterns of inheritance of males with sex linked forms of hypertrichosis
Hypertrichosis lanuginosa

Congenital hypertrichosis lanuginosa may be caused by an paracentric inversion mutation of the q22 band of chromosome 8;[1] however, it could also be possibly the result of a spontaneous genetic mutation rather than inheritance.[8] This form is an autosomal dominant (not located on the sex chromosomes) cutaneous disorder, that affects the skin.[19]

Generalized hypertrichosis

Congenital generalized hypertrichosis has a dominant pattern of inheritance and has been linked to chromosome Xq24-27.1.[1][20] An affected female (carrying the hypertrichosis gene) has a 50% chance of passing it to her offspring. An affected male will pass this form of hypertrichosis to his daughters, but never the sons.

Generalized hypertrichosis terminalis

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.[21] The gene MAP2K6 may be a factor contributing to this condition. This condition may also be due to the change in the chromosome affecting the transcription of genes.[22]

Other hypertrichosis patterns

Porphyria cutanea tarda may manifest in some patients as hypertrichosis on the face (mainly on top of the cheeks).

Medical conditions

Acquired hypertrichosis lanuginosa is commonly present with cancer.[23] This condition is also linked to metabolic disorders, such as anorexia, hormone imbalances, such as hyperthyroidism, or as a side effect of certain drugs.[23]

Acquired generalized hypertrichosis may be caused by cancer. The resulting hair growth is known as malignant down. The mechanism behind cancer induced hypertrichosis is unknown.[3] Oral and topical minoxidil treatments are also known to cause acquired generalized hypertrichosis.[14]

Medications

Several medications can cause generalized or localized acquired hypertrichosifs including: Anticonvulsants: phenytoin Immunosuppressants: cyclosporine Vasodilators: diazoxide and minoxidil Antibiotics: streptomycin Diuretics: acetazolamide Photosensitizes: Psoralen.

The acquired hypertrichosis is usually reversible once these medications are discontinued.

Other

Unknown causes

The exact genetic mutation that causes congenital circumscribed, localized, and nevoid hypertrichosis is unknown.[1]

Pathophysiology

Krao Farini, 1883

A number of mechanisms can lead to hypertrichosis. One cause involves areas of the skin that are transforming from the small vellus type to the larger terminal type.[7] This change normally occurs during adolescence, when vellus hair follicles in the underarms and groin grow into terminal hair follicles.[1] Hypertrichosis involves this same type of switching, but in areas that do not normally produce terminal hair.[1] The mechanisms for this switch are poorly understood.[1]

Another mechanism involves a change in the hair cycle.[1] There are three stages of the hair cycle: the anagen phase (hair growth), the catagen phase (hair follicle death), and the telogen phase (hair shedding).[1] If the anagen phase increases beyond what is normal; that region of the body will experience excessive hair growth.[7]

Diagnosis

Hypertrichosis is diagnosed clinically by the occurrence of hair in excess of what is expected for age, sex, and ethnicity in areas that are not androgen-sensitive.[7] The excess can be in the form of excessive length or density and may consist of any hair type (lanugo, vellus, or terminal).[18]

Management

There is no cure for any congenital forms of hypertrichosis. The treatment for acquired hypertrichosis is based on attempting to address the underlying cause.[18] 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.[24] Hair removal treatments are categorized into two principal subdivisions: temporary removal and permanent removal.[24] Treatment may have adverse effects by causing scarring, dermatitis, or hypersensitivity.[3]

Temporary hair removal may last from several hours to several weeks, depending on the method used. These procedures are purely cosmetic.[2] Depilation methods, such as trimming, shaving, and depilatories, remove hair to the level of the skin and produce results that last several hours to several days.[25] Epilation methods, such as plucking, electrology, waxing, sugaring, threading remove the entire hair from the root, the results lasting several days to several weeks.[25]

Permanent hair removal uses chemicals, energy of various types, or a combination to target the cells that cause hair growth. Laser hair removal is an effective method of hair removal on hairs that have color. Laser cannot treat white hair. The laser targets the melanin color in the lower 1/3 of the hair follicle, which is the target zone.[2] Electrolysis (electrology) uses electrical current, and/or localized heating. The U.S. Food and Drug Administration (FDA) allows only electrology to use the term "permanent hair removal" because it has been shown to be able treat all colors of hair.

Medication to reduce production of hair is currently under testing. One medicinal option suppresses testosterone by increasing the sex hormone-binding globulin.[26] Another controls the overproduction of hair through the regulation of a luteinizing hormone.[26]

Epidemiology

Congenital forms of hypertrichosis are rare. Only 50 cases of congenital hypertrichosis lanuginosa have been recorded since the Middle Ages,[3] and fewer than 100 cases of congenital generalized hypertrichosis have been documented in scientific publications and by the media.[22] Congenital generalized hypertrichosis is isolated to one family in Mexico.[1] Acquired hypertrichosis and hirsutism are more common.[4][27] For example, hirsutism occurs in about 10% of women between ages 18 and 45.[27]

Society and culture

Petrus Gonsalvus (1648), the first recorded case of hypertrichosis[8]
Alice E. Doherty
1874 London advertisement

People with hair often found jobs as circus performers, barbers, making the best of their unusual appearance. Fedor Jeftichew ("Jo-Jo the Dog-faced Man"), Stephan Bibrowski ("Lionel the Lion-faced Man"), Jesús "Chuy" Aceves ("Wolfman"), Annie Jones ("the bearded woman") and Alice Elizabeth Doherty ("The Minnesota Woolly Girl") all had hypertrichosis.[8] Extensive hypertrichosis carries an emotional burden and can cause cosmetic embarrassment; however, some people attempt no treatments because they say it defines who they are.[2][28]

Julia Pastrana

Julia Pastrana (1834–1860)[6] travelled throughout the United States in a freak show as the bearded lady, capturing the attention of many artists.[6] She is portrayed as having dark extensive hairs distributed equally throughout the surface of her body, even on the palms of her hands.[6] Originally, she was believed to have congenital hypertrichosis lanuginosa; however, the generalized form of the syndrome coupled with her gingival hyperplasia indicated that her condition was congenital terminal hypertrichosis.[6][8] This was not confirmed until after her death, when it became clear that her X-linked syndrome resulted in terminal hairs.[8]

Supattra Sasupan

In 2011, Supattra Sasupan[29] (Thai: สุพัตรา สะสุพันธ์; born August 5, 2000),[29] an 11-year-old girl from Thailand with hypertrichosis was named the world's hairiest girl by the Guinness Book of World Records (as Supatra Sasuphan).[30]

The hairy family of Burma

One 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.[31][32] He tells of meeting a hairy man, Shwe-Maong. Shwe-Maong lived in the court of King Ava and acted as an entertainer.[31] Shwe-Maong had four children: three unaffected children, and one child with congenital hypertrichosis, named Maphoon.[31] On a second mission to Ava, Maphoon was described as a thirty-year-old woman with two sons, one of which had hypertrichosis.[31] The affected son was named Maong-Phoset. He had an affected daughter named Mah-Me.[31] Whereas all affected members of the family had dental problems, the unaffected members had perfect teeth.[31]

History

The first recorded case of hypertrichosis was Petrus Gonsalvus of the Canary Islands.[8] This was documented by Altrovandus in mid 17th century and published in his text Monstrorum Historia cum Paralipomenis historiae omnium animalium published in 1642.[8][33] He noted two daughters, a son, and a grandchild in Gonsalvus' family all had hypertrichosis. Altrovandus dubbed them the Ambras family, after the Ambras Castle near Innsbruck, where portraits of the family were found.[8] During the next 300 years, about 50 cases were observed. The scientist Rudolf Virchow described a form of hypertrichosis accompanied by gingival hyperplasia in 1873.[8]

In animals

The condition is also sometimes found in cats. A male Persian with the condition named Atchoum achieved a certain level of notice due to the unusual appearance his hypertrichosis gave him, and has been nicknamed "The Werewolf Cat".[34]

In 1955, a female Müller's Bornean gibbon was obtained from Sarawak that exhibited abnormal hair growth in the facial region. It has been hypothesized that this could be due to facial hypertrichosis.[35]

See also

References

2