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Dermatologic Marvels—Hypertrichosis

Dermatologic Marvels—Hypertrichosis Carnivals and circuses have always attracted spectators to witness the spectacular, unusual, and intriguing. These events would expose people with genetic abnormalities, displaying a phenotype that could easily entice a crowd. The most famous, Fedor Jefitchew, also known as “Jojo the Dog-Faced Boy,” was exhibited in the late 1800s. Most recently in 2011, 11-year-old Supatra Sasuphan from Thailand was named the “World’s Hairiest Girl” by the Guinness Book of World Records. Both of these alluring humans suffered from a rare dermatologic condition known as generalized hypertrichosis.1 Hypertrichosis is a disturbance in villous hair development. Villous hair is often shorter, lightly pigmented, and medullated, and is uniformly distributed over the forehead, eyelids, nose, cheeks, and preauricular regions.2 Because hypertrichosis often presents with varied abnormalities of the teeth and broadened facial features, it has been given the characteristic laymen description of a “dog face” or even a humanoid canine “werewolf.” Hypertrichosis can be classified as generalized hypertrichosis, which occurs over the entire body, or localized hypertrichosis, which is restricted to a certain area. It is postulated that the abnormal hair growth is associated with an abnormal telogen phase of the hair growth cycle.2 In contrast to hirsutism, hypertrichosis is not associated with abnormal androgen secretion or other endocrine abnormalities but has been linked to alterations in chromosome 8q22, suggesting that genes involved with hair growth and distribution are localized to this chromosomal region.3 The first documented case of hypertrichosis was Petrus Gonzales, who was born in the Canary Islands in 1556.3 He was presented as a gift to French nobles and subsequently put on display as a rare enigma. Since his time, others with similar genetic abnormalities have been exploited for their phenotypic anomalies, often exhibited in sideshows and circuses. The history of this rare medical anomaly is fraught with turmoil and sadness, as those affected were scorned, ridiculed, and mocked when displayed as “side show freaks.” Many people with hypertrichosis were thought of as werewolves, frequently presumed to be dangerous. These prejudices, owing to lack of information of the underlying pathophysiology of hypertrichosis, were unwarranted. There have been more than 20 documented cases of hypertrichosis, some of which have been featured in Ripley’s Believe it or Not and The Guinness Book of World Records.3 With a better understanding of the underlying mechanisms of hypertrichosis, the perception of this rare genetic abnormality can be changed, and an accepting public response should be promoted. People with hypertrichosis should be celebrated in our society because they have persevered through prejudice while having contributed drastically to the current pool of knowledge about this rare dermatologic condition. Back to top Article Information Corresponding Author: Eric L. Maranda, BS, University of Miami, Miller School of Medicine, Department of Dermatology and Cutaneous Surgery, 1475 NW 12th Ave, Miami, FL 33136 (emaranda@med.miami.edu). References 1. Hypertrichosis. Extraordinary People, A My Multiple Sclerosis site. http://mymultiplesclerosis.co.uk/ep/chuy-the-wolf-man-jesus-fajardo-manuel-aceves/. Accessed August 16, 2015. 2. Rashid RM, White LE. A hairy development in hypertrichosis: a brief review of Ambras syndrome. Dermatol Online J. 2007;13(3):8.PubMedGoogle Scholar 3. Taylor S, James W. Congenital hypertrichosis lanuginosa. http://emedicine.medscape.com/article/1072987-overview. Accessed August 16, 2015. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png JAMA Dermatology American Medical Association

Dermatologic Marvels—Hypertrichosis

Abstract

Carnivals and circuses have always attracted spectators to witness the spectacular, unusual, and intriguing. These events would expose people with genetic abnormalities, displaying a phenotype that could easily entice a crowd. The most famous, Fedor Jefitchew, also known as “Jojo the Dog-Faced Boy,” was exhibited in the late 1800s. Most recently in 2011, 11-year-old Supatra Sasuphan from Thailand was named the “World’s Hairiest Girl” by the Guinness Book of World...
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Publisher
American Medical Association
Copyright
Copyright © 2016 American Medical Association. All Rights Reserved.
ISSN
2168-6068
eISSN
2168-6084
DOI
10.1001/jamadermatol.2015.4846
pmid
27168211
Publisher site
See Article on Publisher Site

Abstract

Carnivals and circuses have always attracted spectators to witness the spectacular, unusual, and intriguing. These events would expose people with genetic abnormalities, displaying a phenotype that could easily entice a crowd. The most famous, Fedor Jefitchew, also known as “Jojo the Dog-Faced Boy,” was exhibited in the late 1800s. Most recently in 2011, 11-year-old Supatra Sasuphan from Thailand was named the “World’s Hairiest Girl” by the Guinness Book of World Records. Both of these alluring humans suffered from a rare dermatologic condition known as generalized hypertrichosis.1 Hypertrichosis is a disturbance in villous hair development. Villous hair is often shorter, lightly pigmented, and medullated, and is uniformly distributed over the forehead, eyelids, nose, cheeks, and preauricular regions.2 Because hypertrichosis often presents with varied abnormalities of the teeth and broadened facial features, it has been given the characteristic laymen description of a “dog face” or even a humanoid canine “werewolf.” Hypertrichosis can be classified as generalized hypertrichosis, which occurs over the entire body, or localized hypertrichosis, which is restricted to a certain area. It is postulated that the abnormal hair growth is associated with an abnormal telogen phase of the hair growth cycle.2 In contrast to hirsutism, hypertrichosis is not associated with abnormal androgen secretion or other endocrine abnormalities but has been linked to alterations in chromosome 8q22, suggesting that genes involved with hair growth and distribution are localized to this chromosomal region.3 The first documented case of hypertrichosis was Petrus Gonzales, who was born in the Canary Islands in 1556.3 He was presented as a gift to French nobles and subsequently put on display as a rare enigma. Since his time, others with similar genetic abnormalities have been exploited for their phenotypic anomalies, often exhibited in sideshows and circuses. The history of this rare medical anomaly is fraught with turmoil and sadness, as those affected were scorned, ridiculed, and mocked when displayed as “side show freaks.” Many people with hypertrichosis were thought of as werewolves, frequently presumed to be dangerous. These prejudices, owing to lack of information of the underlying pathophysiology of hypertrichosis, were unwarranted. There have been more than 20 documented cases of hypertrichosis, some of which have been featured in Ripley’s Believe it or Not and The Guinness Book of World Records.3 With a better understanding of the underlying mechanisms of hypertrichosis, the perception of this rare genetic abnormality can be changed, and an accepting public response should be promoted. People with hypertrichosis should be celebrated in our society because they have persevered through prejudice while having contributed drastically to the current pool of knowledge about this rare dermatologic condition. Back to top Article Information Corresponding Author: Eric L. Maranda, BS, University of Miami, Miller School of Medicine, Department of Dermatology and Cutaneous Surgery, 1475 NW 12th Ave, Miami, FL 33136 (emaranda@med.miami.edu). References 1. Hypertrichosis. Extraordinary People, A My Multiple Sclerosis site. http://mymultiplesclerosis.co.uk/ep/chuy-the-wolf-man-jesus-fajardo-manuel-aceves/. Accessed August 16, 2015. 2. Rashid RM, White LE. A hairy development in hypertrichosis: a brief review of Ambras syndrome. Dermatol Online J. 2007;13(3):8.PubMedGoogle Scholar 3. Taylor S, James W. Congenital hypertrichosis lanuginosa. http://emedicine.medscape.com/article/1072987-overview. Accessed August 16, 2015.

Journal

JAMA DermatologyAmerican Medical Association

Published: May 1, 2016

References

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