We dwarfs have no homeland, no parents; we allow ourselves to born of strangers, in secret . . . so that our race should not die out . . . they sell us to powerful princes that we may amuse them with our misshapen bodies and be their jesters . . . thus did my mother sell me. . . . –Pars Lagervist, The Dwarf1(p15)
The Spanish court of Philip IV (1605-1665) was exceptional for the number of jesters, dwarfs, and people with disabilities who lived under royal patronage and traveled with the king. More exceptional still were the realistic and sensitive portraits of them (cover, Figure) by court painter Diego Velázquez (1599-1660). Velázquez painted dwarfs along with their royal patrons, including young princes (Figure) and princesses,2,3 and as individuals (cover), demonstrating that they were part of the royal family. Although the modern viewer might assume that their inclusion in palace life was evidence of royal concern about the welfare of those who were less fortunate, their inclusion was primarily for the entertainment of the royal household and companionship for the royal children. Members of the royal entourage constantly flattered and vied with one another to gain the favor of the king while jesters and dwarfs were frequently allowed to bluntly speak their minds or joke about royal affairs. Because most court dwarfs were intelligent, some assumed positions of importance. One, Don Diego de Acedo (“el Primo”),2 is depicted by Velázquez with a large book.
Velázquez's Portrait of Francisco Lezcano–The “Niño de Vallecas” is a realistic depiction of a dwarf. He holds playing cards in his hands, according to a royal painting inventory,2 and is shown in hunting attire in the open air. His head is slightly tilted and his right leg faces forward. The Sierra de Guadarrama mountains are shown in the background. Possibly painted when he was accompanying the king on a hunt, this portrait was displayed in the Torre de la Parada, a hunting lodge on the outskirts of Madrid.
Another of Velázquez's portraits shows a dwarf with Prince Balthasar Carlos,2 the long-awaited heir to the throne, dressed in an embroidered ceremonial robe. The 16-month-old prince is standing on a carpeted step beneath a draped wine-red curtain (Figure), holding a baton and a dagger in his hands. He wears steel armor, the gorget, to protect his neck in anticipation of his future role as military commander. Balthasar Carlos turns toward a dwarf dressed in female clothing who holds a silver rattle and an apple, which mimic the scepter and orb of the future king. Despite the feminine dress, the dwarf is believed to be male since male dwarfs were paired
with male children. Some authorities propose this dwarf is El Niño. Others do not agree, citing records that El Niño did not come to court until 4 years after the painting was completed. Careful inspection of the skeletal dyscrasia of the dwarfs in each of these 2 paintings reveals some small differences in their physical features.
Art historians have generally assumed that El Niño (cover) has a diagnosis of intellectual disability based on his facial appearance; some have referred to him as a cretin. His diagnosis is not hypothyroidism, but he has a skeletal dysplasia, achondroplasia, or possibly the milder variant, hypochondroplasia (written communications; Judith Hall, MD, December 1, 2010; Julie Hoover Fong, MD, November 30, 2010). Unless there are birth complications, people with achondroplasia have normal intelligence despite arrested hydrocephalus in some cases and megalencephaly in others.4 El Niño's facial expression might also be that of a person reflecting on his thoughts or otherwise engaged. Dwarfs in both paintings show characteristic features of achondroplasia with shortening of the upper arms (rhizomelia) and short fingers (brachydactyly). The head is enlarged and the nasal bridge is depressed. There is an apparent shortening of the upper and lower parts of El Niño's legs. His right foot is small and the right leg is bowed.
Achondroplasia is the most common form of short-limb dwarfism, affecting more than 250 000 individuals worldwide. More than 95% of patients have the same point mutation in the gene for fibroblast growth factor receptor 3 (FGFR3), and more than 80% of these are new mutations.5 The mutation, which causes gain of FGFR3 function, affects many tissues, most strikingly the cartilaginous growth plate in the growing skeleton. Possible future treatments include chemical inhibition of receptor signaling, antibody blockade of receptor activation, and alteration of pathways that modulate the downstream propagation of FGFR3 signals.
People with dwarfism have often been stigmatized and mocked and been a source of superstition and public curiosity. Fortunately, their lives today as Little People have been transformed,6 allowing them to be active members of modern society.
1.Lagervist
P The Dwarf. Dick A, trans. New York, NY Farrar, Straus, and Giroux1973;
2.Brown
J Velázquez: Painter and Courtier. New Haven, CT Yale University1986;
4.Rogers
JGPerry
MARosenberg
LA IQ measurement in children with skeletal dysplasia.
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BM The Lives of Dwarfs: Their Journey From Public Curiosity to Social Liberation. New Brunswick, NJ Rutgers University Press2005;