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Hypertrichosis is a disorder characterized by excessive hair growth on the body. 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 may also be either congenital (present at birth), or acquired later in life. The excess growth of hair occurs in areas that are not androgen-dependent areas of the skin. 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.

Classification

Congenital

Congenital forms of hypertrichosis are a dominant trait which has been linked to the X chromosome.

Congenital hypertrichosis lanuginosa


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

Congenital generalized hypertrichosis


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

Congenital terminal hypertrichosis


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

Congenital circumscribed hypertrichosis


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

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 patterned hypertrichosis


Acquired patterned hypertrichosis is an increase in hair growth in a pattern formation, and, similar to acquired generalized hypertrichosis, is an ominous sign of internal malignancy.

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. 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.

Acquired localized hypertrichosis


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

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.

Causes

Genetics

The cause of all congenital hypertrichosis is a genetic mutation. Congenital hypertrichosis is always present at birth.

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

Acquired

Acquired hypertrichosis is the most common form of hypertrichosis. Causes include, metabolic disorders, anorexia, thyroid disorders, and most commonly drugs or chemicals such as oral phentynoin and ciclosporin. Minoxidil, a medication for preventing hair loss, is thought to trigger acquired localized hypertrichosis. Medication induced hypertrichosis can often be treated by using hair removal agents. 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.

Epidemiology

Occurrence

Frequency for congenital hypertrichosis lanuginosa is dismal, with less than 50 reported cases worldwide. The total occurrence of the condition has been reported in the range of 1 in a billion to 1 in 10 billion. Additionally, there is no known prediction for congenital hypertrichosis in terms of race, sex, or geographic region. Most of the cases are limited to one family. There are fewer than 100 documented cases of congenital generalized terminal hypertrichosis.

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

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:

  • Waxing (a hot or cold layer is applied and then removed with porous strips)


  • Sugaring (similar to waxing, but with a sticky paste)


  • Threading (also called fatlah or khite, in which a twisted thread catches hairs as it is rolled across the skin)


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 in 1556. Portraits of Gonzales were found in the castle of King Ferdinand II of France.

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.

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 Burmamarker 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.

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