Hypertrichosis (Werewolf Syndrome)

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Hypertrichosis is a rare disorder that causes abnormal excessive body-hair growth [7]. Hypertrichosis has informally been called Werewolf Syndrome [13]. It is categorized as either congenital (acquired at birth), or it can be acquired later in life, and can be characterized as either regional or generalized [15].

Pruthviraj Patil, an 11-year old Indian boy who was born with hypertrichosis [1] [16]

History

Petrus Gonzales, known as Wolf Boy of the Canary Islands [2] [12]

The first documented case of hypertrichosis was in 1648. Aldrovandus documented a family with hypertrichosis from the Canary Islands. Petrus Gonzales was sent to France as a curiousity to the nobles; he, a son, his two daughters, and a grandchild were affected with the disorder. Over the next 300 years, there were more than 50 similar cases documented, and 34 of those patients had alleged congenital hypertrichosis, which is very rare. Since the Middle Ages, approximately 50 individuals have been described with congenital hypertrichosis, and according to recent estimates (2009), approximately 34 cases are documented adequately in the literature [13]. There is no recollection of who it was that actually coined the term hypertrichosis.

All in all, there are many sources that have their own views on the history of the discovery of hypertrichosis. No absolute assurance is known on exactly who first discovered the disease and in what year. One rather recent claim is that just over twenty-five years ago, Frank Greenberg, an American geneticist from the Baylor College of Medicine, discovered a family from Mexico that possessed a rare mutation that led to the appearance of thick hair that completely surrounded bodies of all members of the family, which was forced to flee from their Mexican society to a town up in the mountains known as Zacatecas. Work in the circus was the only career path that this family could obtain [6].

There are many people who have been publicly recognized for being hypertrichosis patients. Jesus "Chuy" Aceves (image below) and his sister Lili were both born with the disease. "Chuy" resembled the typical wolfman seen in movies and some referred to him as "monkey man." "Chuy" shaved for the first time when he made his documentary debut back in 2005. He was featured in books such as Guinness World Records and Ripley's Believe It of Not! in 2007 and he currently works for a traveling circus [9]. A sideshow circus performer known as the Lion-Faced Man had hypertrichosis over the course of his entire body. His name is Stephen Bibrowski and his mother actually lied about how his physical characteristics came to be, claiming that he turned out the way he did because she witnessed his father being attacked by a lion before Stephen was born [5].

"Chuy" Aceves [3] [10]

Mechanisms of Hypertrichosis

The overall cause of hypertrichosis for the most part is unknown. Scientists believe that congenital generalized (CGH) hypertrichosis is a genetic disorder that occurs due to a spontaneous mutation in the DNA sequence [8]. However, some studies have shown that the mutation leading to CGH is located between Xq24-q27.1 regions of the X-chromosome [4]. But much of the research in this area is still being conducted and nothing is known for certain [8].

Acquired generalized hypertrichosis can be caused by taking certain drugs. The drugs that most often cause hypertrichosis are phenytoin, cyclosporine, and minoxidil. Phenytoin can cause hypertrichosis in as little as three months and often coincides with acne and gingival hyperplasia. In 60% of patients hypertrichosis developed when they had been treated with cyclosporine. Within six months of cyclosporine treatment thick, dark hair growths started on the face, trunk, and limbs. Minoxidil causes hypertrichosis on the face and extremities mainly in only a few short days. Some other less common drugs that may be responsible for hypertrichosis are: corticosteroids, diazoxide, interferon-α2, latanoprost, penicillamine, phenytoin, psoralens, streptomycin, and zidovudine. Drugs are not the only way one can acquire this disease. In some cases, head injuries, malnutrition, anorexia nervosa, AIDs, and some forms of cancer can also cause hypertrichosis [14].

One study suggests that with the use of the drug minoxidil and diazoxide, increased cutanous blood flow is a possible explanation for the cause of hypertrichosis, since they are both vasodialators. Also, in vitro experiments, clinical observations, and animal studies suggest that hypertrichosis caused by minoxidil is somehow related to the drugs action as a potassium channel opener [18]. The drugs causing hypertrichosis may turn fine villus hair into longer, dark terminal hair. Drugs could also extend the anagen phase of hair follicles which makes longer hairs. The drug's affect on hair follicles on a molecular level is still unknown, and is not currently understood by scientists [2].

Types of Hypertrichosis

The primary symptoms of all forms of hypertrichosis is excess hair. This excess hair is longer than regular hair growth and the hair type varies. Hypertrichosis that is regional causes hair to grow in restricted areas around the body and/or face. Hypertrichosis that is generalized results in hair growth over the entire body [13]. Several different types of hypertrichosis exist.

Congenital hypertrichosis lanuginosa is a very rare syndrome. Since the Middle Ages, only about fifty cases have been reported. In this case, children right after birth have excessive hair growth, and most of the body is covered with lanugo hair. Lanugo hair is fine, unpigmented, soft, silky hair that covers the fetus, but is later shed after the eighth month of pregnancy, and is then replaced with vellus hair and terminal scalp hair just before birth. In this case, lanugo hair continues to grow. So the person will have extremely long hair covering their body throughout their life [8].

Congenital hypertrichosis terminalis shows hair growth all over the body, but the hair is pigmented terminal hair. It is usually associated with gingival hyperplasia. People with this type of hypertrichosis are called wolf men or werewolves frequently [8].

Naevoid hypertrichosis is an unusual form where only a patch of terminal hair growth occurs. The patch is usually at the bottom of the person’s back. It can be present at birth or occur later in life. It can also be found in the form of one large, bushy eyebrow [8].

Acquired hypertrichosis develops after birth and is typically growth of unpigmented vellus or pigmented terminal hair. Sometimes the hair growth is centered on a certain area or is covers most areas of the body [8].

Current Treatments

The current treatment for hypertrichosis is through cosmetic procedures. That is, to remove the excessive hair from the body through various methods, such as bleaching, plucking, waxing, trimming, shaving, chemical depilatories, electrosurgical epilation, and laser hair removal. No single method can be chosen as a best fit for removing the hair, because it depends on the hair type, the amount, area, and the patient themselves [14]. There are many affordable and more convenient treatments for hypertrichosis. They include bleaching, trimming, shaving, plucking, and waxing. Some more expensive, but longer lasting treatments for hypertrichosis are chemical depilatories,electrosurgical epilation, tropical eflornithine, and laser treatments.

Chemical Depilatories: a method that breaks the hair at the surface of the skin. This method works by attaching to the hair, removing the oily layer on the shaft, and allowing the thioglycolates to break down the keratin in the hairs. This method is usually used weekly on small areas [14].

Electrosurgical Epilation: a more permanent method of removing the hair. It is performed by inserting a fine wire needle into the follicle of the hair and sending electrical currents through it to destroy the dermal papilla or follicle of the hair. There are two types of treatment that use this method; electrolysis and thermolysis [14]. Electrolysis kills the dermal papillae layer of the follicle through electrical currents, while thermolysis kills the follicle through heat produced from electrical currents [15]. Electrosurgical epilation will prevent most hairs from growing back. However, it is not guaranteed that the hairs will not grow back so many treatments may be necessary.

Tropical Eflornithine: a treatment used to slow the growth of hair. This is done by using the chemical eflornithine to inhibit ornithine decarboxylase (an enzyme used in maintaining hair growth). This treatment is applied as a cream [15].

Laser Treatment: this is used for long-term hair removal.in this treatment, high pulse light is absorbed by the malanosomes of the hair bulb. This light energy is then given off the bulb as heat damaging the surrounding tissues causing the hair to regrow slowly. This is less painful than electrosurgical epilation, and usually more effective [15].

There is no cure for hypertrichosis. Therefore, people with hypertrichosis must undergo countless treatments to remove the excess hair throughout their lifetime.

Current Research

There is no current research being addressed for the time being. This being said, a couple of research studies from the past on hypertrichosis have lead to intriguing results. A specific gene known as a Wnt determines anatomical diversity in different taxa. It is perceived that this gene regulates hair patterning. Research in mice embryos showed that the hypertrichosis phenotype is displayed when the protein beta-catenin is overexpressed, leading to activation of Wnt signaling to the nucleus, which ultimately induces excess hair follicles in the embryos. On the other hand, the hypotrichosis phenotype is expressed when beta-trichosis becomes inactive, leading to less thick epithelial tissue and less hair follicles [3].

Wnt does not just regulate the amount of hair, but also the spacing of hair. In a study with dogs it was found that the Wnt pathway affects hair patterning more than any other pathway, making it the main precursor to hair development. There are two critical Wnt genes in mice that are the most crucial to hair development, but nineteen in all. Humans, too, possess nineteen Wnt genes [3].

It has been found that hirsutism is a form of hypertrichosis in which women are affected. Patients have usually been found to exhibit excessive hair that is usually common among adult males, such as hair of the chest and back. Hirsutism in studied individuals was both acquired throughout life and present at birth. Women possessed other male characteristics as well, such as deeper voices, body shapes and sizes more similar to males, and a higher prevalence of acne, just to name a few. A surplus of male hormones, or androgens, in females is the main reason why hirsutism as come to be. Oddly enough, research has shown that birth control pills are capable of decreasing androgen levels, as are certain other medications used to lessen androgen production in the body [11].

Future Outlook

Although there is no current cure for hypertrichosis, there are certain treatments used that temporarily help with the removal of the hair. Hair removal using laser and light sources focused on hair removal through thermal injury. Also, photodynamic therapy involves the use of a photosensitizer (5-aminolevulinic acid, 5-ALA) and light in order for it to have therapeutic effects. Following the photochemical activation of the photosensitizer by light, the mechanism of action involves the generation of toxic reactive oxygen species. As 5-ALA accumulates in the pilosebacious unit, the destruction of selective hair follicles could potentially occur. This technique does not require a laser light source, which also makes it less expensive than laser treatment [14].

Advances in molecular technology have really opened the doors to the study of molecular genetics with the control of hair growth. In the future, further powerful strategies for hair removal will be discovered once more knowledge is gained on the important molecules that control hair growth [14].

References

1 Burton, J. L., & Marshall, A. (1979, November). Hypertrichosis Due to Minoxidil. British Journal of Dermatology, 101(5), 593-595. Retrieved from http://onlinelibrary.wiley.com/doi/10.1111/j.1365-2133.1979.tb15106.x/abstract

2 Drug Induced Hypertrichosis. (n.d.). Retrieved November 30, 2010, from http://www.keratin.com/ah/ah003.shtml

3 Held, L. I., Jr. (2010). The Evo-Devo Puzzle of Human Hair Patterning. Evol Biol, (37), 113-122. Retrieved from http://journals.ohiolink.edu/ejc/pdf.cgi/Held_Lewis_I.pdf?issn=00713260&issue=v37i2-3&article=113_tepohhp

4 Henahan, S. (1995, June). Atavistic “Werewolf” Gene Localized. Retrieved November 18, 2010, from Access Excellence website: http://www.accessexcellence.org/WN/SUA05/wolfman.php

5 Homberger, F. (2005). Stephan Bibrowski. In Carny Folk (p. 138). New York: Kensington Publishing Corp.

6 Hypertrichosis. (n.d.). Retrieved from http://werewolves.monstrous.com/hypertrichosis.htm

7 Hypertrichosis. (2005). In E. Scott, L. David, & E. Mills (Eds.), Professional Guide to Disease (8th ed., p. 1249). Philadelphia: A Wolters Kluwer Company.

8 Hypertrichosis. (2010, September 12). Retrieved November 18, 2010, from DermNet NZ website: http://dermnetnz.org/hair-nails-sweat/hypertrichosis.html

9 Jesús Aceves. (n.d.). Retrieved November 18, 2010, from Wikipedia website: http://en.wikipedia.org/wiki/Jes%C3%BAs_Aceves

10 [Jesús Aceves] [Photograph]. (n.d.). Retrieved from Indian Info database.

11 Mofid, A., Seyyed-Alinaghi, A. S., & Yazdani, T. (2008). Hirsutism. International Journal of Clinical Practice, 62, 433. Retrieved from http://onlinelibrary.wiley.com/doi/10.1111/j.1742-1241.2007.01621.x/full

12 Petrus Gonzales- Wolf Boy of the Canary Islands. (n.d.). Retrieved November 18, 2010, from The Human Marvels website: http://thehumanmarvels.com/?p=29

13 Taylor, S. K., MD., & Galeckas, K. J., MD. (2009, June 18). Congenital Hypertrichosis Lanuginosa. Retrieved November 18, 2010, from eMedicine database.

14 Trüeb, R. M. (2002, September 1). Causes and Management of Hypertrichosis. American Journal of Clinical Dermatology, 3(9), 617-627. Retrieved from http://journals.ohiolink.edu/ejc/article.cgi?issn=11750561&issue=v03i0009&article=617_camoh

15 Wendelin, D. S., MD., Pope, D. N., MD., & Mallory, S. B., MD. (2003, December). Hypertrichosis. Continuing Medical Education, (48), 161-179. Retrieved from http://journals.ohiolink.edu/ejc/pdf.cgi/Wendelin_Daniel_S.pdf?issn=01909622&issue=v48i0002&article=161_h

16 ’Werewolf boy’ Pruthviraj Patil [Photograph]. (n.d.). Retrieved from The Telegraph database.