Congenital hypertrichosis lanuginosa (CHL) is an extremely rare skin disease that has fascinated the public since the Middle Ages. Often referred to as “werewolf syndrome,” the affliction causes excessive hairiness usually present at birth that covers almost the entire body, with the exception of the mucous membranes, palms, and soles of the feet. The hair is characterized as lanugo, which is unpigmented, fine, and soft, and is typically 3 to 5 cm in length. Otherwise, patients with CHL are typically asymptomatic.
CHL has an unknown pathogenesis. It is thought to be inherited in an autosomal dominant manner; however, the specific genetic abnormality has not been discovered. Cases of this disorder are very rare, and it’s estimated that there have only been 50 or so cases described, with only 34 patients positively identified.
Wolf-like people appear in the legends of many cultures in all regions of the world. In the past, sufferers of the disease entertained in traveling circuses and freak shows. Even large circuses like Barnum & Bailey often had headliners that included the bearded lady or dog-faced man.
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Consistent with an autosomal dominant mode of inheritance, 4 generations of a Burmese family, also known as the Sacred Family of Burma, suffered with CHL. Their history begins in 1826 when they lived at the court of Ava, a province in Burma. Escaping the Third Burmese War in 1885, the family began to tour, exhibiting themselves for profit. In 1888, they appeared with the P.T. Barnum Circus in the US, after which their whereabouts became unknown. While with P.T. Barnum, the Sacred Family of Burma appeared with JoJo the Dog Faced Man, who also had CHL. In 1904, while performing with P.T. Barnum in Greece, JoJo caught pneumonia and died at the age of 35. He never had any children. The earliest fully documented case of CHL is that of Petrus Gonzales, born in the Canary Islands in 1556. As a curiosity, he went on to become an entertainer in the court of the French King Henry II. He married and had several children, all of whom inherited CHL.
CHL has no connection to the phases of the moon, and there is definitely no genetic connection to wolves. The majority of sufferers present only with excessive hairiness; however, some exhibit symptoms that include delayed eruption of teeth, hearing impairment, glaucoma, pyloric stenosis, skeletal abnormalities, and abnormal skin pigmentation. No other abnormalities of human organ systems are typically associated with CHL.
The severity of CHL may decrease or increase with age. Treatment usually consists of cosmetic intervention, such as electrolysis, laser hair removal, and depilation, as there is no cure for this affliction.
Reference
- Beighton P. Congenital hypertrichosis lanuginosa. Arch Dermatol. 1970;101(6):669-672. http://archderm.jamanetwork.com/article.aspx?articleid=531637.
- Bondeson J, Miles AE. The hairy family of Burma: a four generation pedigree of congenital hypertrichosis lanuginose. J R Soc Med. 1996;89(7):403-408. http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1295857/.
- Hypertrichosis information. Hypertrichosis website. http://www.hypertrichosis.com/.
- Hypertrichosis lanuginosa congenital. Genetic and Rare Diseases Information Center (GARD). National Institutes of Health website. Updated October 23, 2014. http://rarediseases.info.nih.gov/gard/2865/hypertrichosis-lanuginosa-congenita/resources/1.
- Martel T. JoJo the Dog Faced Man and the sacred hairy family of Burmah. Prezi website. October 11, 2012. https://prezi.com/fslibgooyl1x/jojo-the-dog-faced-man-and-the-sacred-hairy-family-of-burmah/.
- Taylor SK, James WD, Galeckas KJ. Congenital hypertrichosis lanuginosa. Medscape website. May 2, 2014. http://emedicine.medscape.com/article/1072987-overview.
