Revealed: Why the duchess was so ugly

2016 02 03 09 04 57 264 2016 02 03 Ugly Duchess Light 20160203160610

Radiologists from Malta have shed new light on "The Ugly Duchess," a famous early 16th century painting by the Flemish artist Quinten Massys. The unfortunate woman suffered from a rare form of Paget's disease, or osteitis deformans.

The portrait is one of the most popular paintings in the National Gallery in London, and the old woman's looks inspired illustrations for Lewis Carroll's Alice's Adventures in Wonderland. She had an advanced form of Paget's disease that enlarged her jaw bones, pushed up her nose, and extended her upper lip. The disease also affected her forehead, chin, hands, eye sockets, and collarbones.

An Old Woman (known as 'The Ugly Duchess'), was painted in about 1513 by Quinten Massys (1465/6-1530). Oil on oak, 62.4 x 45.5 cm. Bequeathed by Miss Jenny Louisa Roberta Blaker in 1947. Copyright of National Gallery, London.An Old Woman (known as "The Ugly Duchess"), was painted in about 1513 by Quinten Massys (1465/6-1530). Oil on oak, 62.4 x 45.5 cm. Bequeathed by Miss Jenny Louisa Roberta Blaker in 1947. Copyright of National Gallery, London.

"Paget's disease commonly affects the axial skeleton," noted Dr. Christine Cannataci, from the medical imaging department at Mater Dei Hospital, an acute general teaching hospital in Malta. "The radiologist should be able to recognize the imaging manifestations of the different stages of the disease and the complications associated with this condition on different imaging modalities."

This disease owes its name to Sir James Paget, a prominent 19th century U.K. surgeon and pathologist who in 1877 described osteitis deformans in five middle-aged men. Several other reports of men who complained that their hats no longer fitted them had already been published at the time, and by the end of the century, this affliction had become known as Paget's disease of bone. According to Paget, this disease was a rare chronic inflammatory disorder that had a slow rate of progression and did not affect the individual's general health but resulted in change in shape, size, and deformity of the affected bones.

The prevalence of Paget's disease is on the decline, Cannataci and colleagues explained in an e-poster presented at RSNA 2015 in Chicago. It increases with age, and there is a slight male predominance. It is relatively common in Malta, and is also found more often in the U.K., France, Germany, Italy, and regions inhabited by migrants from these areas.

"Maltese immigrants residing in Australia were also noted to have a higher incidence of Paget's (1.8%) as compared with other patient groups," the authors stated. "This noninflammatory, metabolic disorder may affect single or multiple bones in a synchronous or metachronous fashion. Most patients are asymptomatic and cases are discovered incidentally on imaging carried out for other reasons."

The most common manifestation of this disease, if any, is pain at the site of the bone involved and nearby joints. Paget's disease shows a predilection for the axial skeleton, with the skull and vertebral column being the most commonly affected sites. The pelvis is the most commonly affected part of the appendicular skeleton. Pathological lythis disease involves excessive bone resorption followed by formation of bone that is structurally abnormal, they continued.

The osteolytic phase is characterized by geographic osteolysis with an advancing edge of resorption. In the skull, this is referred to as osteoporosis circumscripta, and it results in well-defined oval lytic areas crossing sutures, with a predilection for the frontal and occipital bones.

In the mixed lytic/sclerotic phase, endosteal new bone formation encroaches in the medullary cavity, resulting in loss of corticomedullary differentiation. Together with coarse trabecular markings, osseous expansion, and cortical thickening, these are the hallmarks of this stage and make it the most recognized aspect of Paget's disease. Most cases are diagnosed at this stage, Cannataci et al pointed out.

The sclerotic phase is the final stage of Paget's disease, and is represented by the late osteosclerotic "burnt-out" phase where bone turnover may return to an almost normal level when osteoclastic activity declines. Restoration of the marrow occurs, and the prominent mosaic pattern and thickened trabeculated bone may persist, they wrote.

The painting by Massys inspired the U.K. illustrator John Tenniel to make the Duchess (the unpredictable, Cheshire Cat-owning baby abuser) one of the most grotesque characters in his illustrations of the Lewis Carroll classic novel, according to an article published on 11 October 2008 by the Guardian newspaper.

"Curators are particularly excited about this painting because two important discoveries have been made in recent research: firstly, the portrait is truthful and she almost certainly looked like that, and secondly, a long held historical theory that the painter was copying Leonardo da Vinci is wrong," the report in the Guardian noted.

Massys reportedly painted it as part of a pair. The other, "The Old Man," has been loaned to the National Gallery and has hung alongside "The Ugly Duchess" for the first time in 150 years.

"I've always been intrigued by this painting. It's fascinating because it is so meticulously and lovingly painted," said Michael Baum, emeritus professor of surgery at University College London who, with his student Christopher Cook, investigated the portrait. "You think, why would someone go to so much trouble in order to paint such a grotesque image? I always suspected there was something more to it than just a study in grotesquery."

He told the Guardian he had never seen a full-blown case like this.

"In all probability, the condition would have happened later in life so she may even have been a beauty before the condition set in. Aside from the effect on her looks, she may have suffered no more than headaches and a damaged pituitary gland," Baum said, who is convinced the sitter was a very powerful woman and may even have been a real duchess. "You either love it or you hate it and I love it; it's part of the background of London, part of London's iconography."

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