The Royal History of Porphyria

The word “Porphyria” originates from the Greek “porphyros”, meaning purple. During the Phoenician Era, wearing a purple garment meant having to harvest mollusks to obtain the dye. The process itself proved to be costly and laborious as about 250,000 mollusks were needed to produce just one ounce of the purple dye.1 As a result, purple apparel could only be afforded by the wealthy, and in effect began to symbolize royalty. However, the word “porphyria” may invoke the thought of royalty for another reason being that King George III of Great Britain was thought to have had porphyria.

The Porphyrias comprise a group of metabolic disorders that result from an enzyme deficiency in the heme synthesis pathway. A deficient enzyme in a step of this pathway leads to the buildup of a porphyrin intermediate, resulting in the manifestation of clinical symptoms. The presentation of porphyria may include cutaneous, neurological, and gastrointestinal symptoms, and is dependent on the type of porphyrin that is elevated.

It has been speculated that King George III of Great Britain may have had a type of hereditary porphyria, variegate porphyria.2 He had suffered episodes of mental instability along with cutaneous, gastrointestinal, and neurologic symptoms. During his episodes, he experienced rashes, abdominal pain, constipation, and pain in his limbs. 3, 4 His personal physician also felt that he was borderline delirious. In his diary, he wrote that the king brazenly talked about secrets of the parliament, and he had even reached a point where he attempted to jump out of a window.5

King George’s past history of mental instability gained more attention upon the release of the popular play “The Madness of King George III” in 1991, followed by a film adaptation shortly afterward. However, the reputation of King George’s mental condition associated with the diagnosis of porphyria has been met with skepticism. In recent years, critics have argued that porphyria does not seem to be a likely diagnosis to explain his clinical presentation. In 2010, the History of Psychiatry journal published a study that analyzed the writing features of King George’s letters by a computer analysis. From the results, researchers speculated that he may have instead been experiencing manic episodes instead of a disorder of porphyrin metabolism.

Porphyria is not a foreign term in the royal family tree. Princess Charlotte of Wales, the granddaughter of King George III, was also suspected to have had porphyria.  A later descendant of King George III, Princess Charlotte of Prussia, wrote letters to her physician that described episodes of skin rashes, abdominal pain, and dark red urine.6 DNA testing performed on her remains also demonstrated a mutation in the gene encoding protoporphyrinogen oxidase.7 Moreover Prince William of Gloucester, a recent royal blood, had seen a physician for a blistering rash on his face and the back of his hands, and was diagnosed as having variegate porphyria.7 He was later seen by a hematologist in Cambridge and a professor in Tokyo, and was also diagnosed by them with variegate porphyria.

While King George’s diagnosis of porphyria has been debated, his struggle with mental health does not seem to be a point of contention. It therefore raises the interesting question if his condition impaired his judgment during his reign as king, as it was also known to be a period marked by political instability. Under his leadership, there was a fast turnover in the parliament, and laws were imposed on the American Colonies as quickly as they were repealed.8 King George was also quick to seek retribution with any signs of resistance by the colonies.

Tensions between the American Colonies and Great Britain had finally reached its breaking point when the Tea Act – supported by the king – was imposed on the colonies, resulting in an outbreak of conflict that spurred the American Revolution.

It is interesting to conjecture if the American rebellion would have occurred had the King George been in a healthier state of mind; however, his leadership was often perceived as unstable by those around him. His decisions were often perceived as impulsive and may have been due, in part, to his mental instability.

King George III
Image from: Royal.uk
Artist: Allan Ramsay

References

  1. Andrews, E. (2015, July 15).Why is purple considered the color of royalty? Retrieved from https//www.history.com/news/why-is-purple-considered-the-color-of-royalty
  2. Cox, T. M., Jack, N., Lofthouse, S., Watling, J., Haines, J., & Warren, M. J. (2005). King George III and porphyria: An elemental hypothesis and investigation. The Lancet, 366(9482), 332-335. doi:10.1016/s0140-6736(05)66991-7
  3. Macalpine, I., & Hunter, R. (1966). The “insanity” of King George 3d: A classic case of porphyria. Bmj, 1(5479), 65-71. doi:10.1136/bmj.1.5479.65
  4. Crews, E. (2010). The Poisoning of King George III. Retrieved from https//www.history.org/
  5. Ray, I. (1855). Insanity of King George III: Read before the Association of Superintendents of Insane Hospitals, May 22, 1855. Utica, NY: Printed at the Asylum.
  6. Ensminger, P. A. (2001). Life under the sun. New Haven: Yale University Press.
  7. Rohl, J., Warren, M., & Hunt, D. (1999). Purple Secret: Genes, ‘Madness’ and the Royal Houses of Europe. London: Corgi Books.
  8. King George III. (n.d.). Retrieved from https//www.pbs.org/wgbh/americanexperience/features/adams-king-george-III/

 

Author: Christopher Dallo, B.S., University of Texas Medical Branch at Galveston

 

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