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Hypertrichosis lanuginosa acquisita. DermNet NZ dermnetnz.org |
Hypertrichosis is a term used to describe hair growth on the body in an amount which is considered abnormal.[1][2] 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.[3][4] The excess growth of hair occurs in areas that are not androgen-dependent areas of the skin.[5] Hypertrichosis differs from Hirsutism, which is abnormal hair growth on females in places which normally have minimal or no hair (i.e. chest hair, beard).[6] 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.[4]
[edit] ClassificationThere are two method of classification for hypertrichosis. One divides them into either generalized versus localized well the other divides them into congenital versus acquired.[7] [edit] CongenitalCongenital forms of hypertrichosis are caused by genetic mutations. These forms of hypertrichosis are far more rare than acquired forms. Congenital hypertrichosis is always present at birth.[4]
Congenital hypertrichosis lanuginosa is first noticeable at birth with 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 remains after birth.[4] As the person with this type of hypertrichosis ages, the lanugo hair may thin and eventually only limited areas of hypertrichosis will be present.[8]
Congenital generalized hypertrichosis causes males to exhibit excessive facial and upper body hair, while women typically exhibit less severe asymmetrical hair distribution.[9]
Congenital terminal hypertrichosis is characterized by the presence of fully pigmented terminal hair which covers the entire body.[4] This condition is usually present along with gingival hyperplasia.[4] 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.[4]
Congenital circumscribed hypertrichosis is associated with the presence of thick vellus hair on the upper extremities.[10] 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.[10] 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.[10]
Congenital localized hypertrichosis is a localized increase in hair density and length.
Nevoid hypertrichosis may be present birth or appear later in life.[4] It features a lone area of excessive terminal hair on the body and is usually not related to any other diseases.[4][11] [edit] AcquiredAcquired 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 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]
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.[3] 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.[14]
Acquired patterned hypertrichosis is an increase in hair growth in a pattern formation, and, similar to acquired generalized hypertrichosis, is an sign of internalmalignancy.[5]
Acquired localized hypertrichosis is an increase in hair density and length often secondary to irritation or trauma.[15] This form is restricted to a certain area of the body. [edit] Hirsutism.jpg) Main article: Hirsutism Hirsutism is a type of hypertrichosis exclusive to women.[6] Women suffering hirsutism exhibit patterns of regular male hair growth. Chest and back hair are often present on women with hirsutism.[6] Hirsutism is both congenital and acquired. Hirsutism can be acquired through an excess of male hormones in women, thus resulting in symptoms such as acne, deepening of the voice, irregular menstrual periods, and the formation of a more masculine body shape.[6] Increased androgen levels account for approximately 50% of hirsutism cases. If this is the cases, hirsutism can be treated with medications that reduce androgen levels. Some birth control pills and spironolactone reduce androgen levels.[6] [edit] Signs and symptomsHair in hypertrichosis is usually longer than what is expected for that area of the body[7] and and may consist of any hair type (lanugo, vellus, or terminal).[16] [edit] Causes[edit] Genetic Patterns of inheritance of females with sex linked forms of hypertrichosis  Patterns of inheritance of males with sex linked forms of hypertrichosis
The source of congenital hypertrichosis lanuginosa is unknown, it may be caused by a spontaneous genetic mutation rather than being inherited.[8] 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.[17]
Congenital generalized hypertrichosis has a dominant pattern of inheritance and has been linked to the X chromosome.[18] 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.
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.[19] 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.[20] [edit] Medical conditions
Acquired hypertrichosis lanuginosa is commonly present with cancer.[21] 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.[21]
Acquired generalized hypertrichosis can be obtained through cancer. The hair that grows due to this condition is known as malignant down. The mechanism behind cancer causes hypertrichosis is unknown. Oral and topical minoxidil treatments are also known to cause acquired generalized hypertrichosis.[14] [edit] Medications
Minoxidil, a medication for preventing hair loss, is thought to trigger acquired localized hypertrichosis.[22] [edit] Other
The exact genetic mutation which causes congenital circumscribed, localized, and nevoid hypertrichosis is unknown.[1] [edit] PathophysiologyA number of mechanisms can lead to hypertrichosis. One involves the type of hair growing in an area switching from the small vellus type to the larger terminal type.[7] Another mechanism involves a change in the hair cycle. If the anagen phase ( or the growth phase ) increases beyond what is normal for that area of the body increased hair results.[7] [edit] DiagnosisHypertrichosis is diagnosed clinically via 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).[16] [edit] ManagementSee also: Hair removal The treatment of acquired hypertrichosis is based on finding and attempting to address the underlying cause.[16] 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.[23] Treatment may have adverse effects by causing scarring, dermatitis, or hypersensitivity.[4] Temporary hair removal lasts from several hours to several weeks. These procedures are purely cosmetic.[2] Depilation methods remove hair to the level of the skin. These techniques produce results that last several hours to several days. Trimming, shaving, and depilatories are examples of depilation methods.[24] Epilation methods remove the entire hair from the root producing a result lasting several days to several weeks. Plucking, electrology, waxing, sugaring, threading are examples of epilation methods.[24] Permanent hair removal involves several 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. Laser hair removal has proven to be the most effective method of long-term hair removal available.[2] Electrolysis (electrology), and Laser Hair Removal are two examples of permanant hair removal methods. [edit] Epidemiology
Congenital hypertrichosis lanuginosa is a rare condition. Around 50 cases have been recorded since the Middle Ages.[4]
Fewer than 100 cases of congenital generalized hypertrichosis have been documented in scientific publications and by the media.[20] [edit] History The first recorded case of hypertrichosis was Petrus Gonzales of the Canary Islands.[8] This was documented by Altrovandus in 1648.[8][25] He noted in Gonzales' family that two daughters, a son, and a grandchild all suffered hypertrichosis. Altrovandus dubbed them the Ambras family, after an Innsborough castle where portraits of the family were found.[8] For the next 300 years there were approximately 50 cases observed. The scientist Rudolf Virchow described a form of hypertrichosis accompanied by gingival hyperplasia in 1873.[8] [edit] Society and culturePeople 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 people with hypertrichosis.[8] Extensive hypertrichosis carries a heavy emotional burden and can cause cosmetic embarrassment, however some people do nothing to get rid of this hair because they say it defines who they are.[2][26] [edit] Julia PastranaMain article: Julia Pastrana A notable example of congenital terminal hypertrichosis is Julia Pastrana (1834-1860)[27]. At the age of 20, Julia traveled throughout the United States in the traveling freak show as the bearded lady, capturing the attention of many artists. She served as an interesting subject of depiction because of the intensity of her condition, which portrayed her has having dark extensive hairs distributed equally throughout the surface of her body, even on the palms of her hands[27]. Originally, she was believed to have congenital hypertrichosis lanuginosa, when in reality the generalized form of the syndrome coupled with her gingival hyperlasia indicated that her condition was congenital terminal hypertrichosis[8][27]. This was not confirmed until after her death, when it became clear that her X-linked syndrome resulted in terminal hairs.[8]. [edit] The Hairy Family of BurmaOne 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. 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.[28] [edit] See also
[edit] References
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