“The Curse of the MacCrimmons”

A history of Dupuytren’s Contracture.

Long ago, in the sixteenth century in Scotland, the chieftains of Clan MacLeod were chieftains of the Island of Skye. Second only to the MacLeods were their pipers, the MacCrimmons.

There are many stories as to how the curse arose. Some say that the MacCrimmons were cursed by a widow whose only son was taken by the press gangs. Others tell of a MacCrimmon woman by the name of Annag who was punished for giving piping secrets to her MacPherson lover, by the removal of several fingers.

Whoever performed the curse, all are agreed that the angry woman foretold that the MacCrimmons would cease to be the official pipers to the MacLeods of Dunvegan and would leave the Isle of Skye forever.

And so it came to pass. The fingers of the MacCrimmon men bent so far into their palms that they became unable to play the bagpipes any more.

And from that day to this, any piper who suffers from Dupuytren’s Contracture will tell you that he has been affected by the Curse of the MacCrimmons.

It’s a great story, and it’s also one of many ways in which we can track Dupuytren’s back through history. We can’t trace the condition directly through written records because few survive, and also because Dupuytren’s wasn’t formally described in medical literature until the 17th century, but we find its footprints (or should that be hand prints) in many places.

Where did the MacCrimmons’ Curse originally come from? Well, it’s generally accepted that the surname MacCrimmon may be of Norse origin, and in Icelandic, Orcadian and Danish sagas, we find several descriptions of ‘fingers clenched in the hands’. Here’s one from the Longer Saga of Magnus: Another man hight Sigurd, from the north of Shetland; he had cramped hands, so that all the fingers lay in the palms. He sought the halidom of the saint earl Magnus, and there he got his cure with straight and lissom fingers for all his needs.

It’s probably no coincidence that these Viking peoples settled in large numbers in Scotland, carrying their genetic inheritance with them.

This 13th century altar cloth from Iceland shows bishops with their little finger and ring finger bent into the palm. Of course, it’s always possible that the Icelandic bishops (or the Icelandic embroiders) were simply using a gesture that was already established somewhere else. If we go to Rome and look at the statue in St Peter’s Basilica, we see the same gesture.

The statue of St Peter is very old. Some scholars have attributed it to Arnolfo di Cambio (1245-1302 AD), but others believe that it is a fifth century casting.

St Peter shows the ‘hand of benediction’ that has been used by many Popes and it can be seen in other holy images such as this Venetian coin.

There is a general problem with such images. They may represent someone with Dupuytren’s, or copying a gesture popularised by someone with Dupuytren’s. Or they may have nothing to do with Dupuytren’s at all...


 

Imagine someone in the future digging up this statue:

 

Sir Winston Churchill did NOT have Dupuytren’s, but a future archaeologist, not knowing his “V for Victory” sign, might draw a totally incorrect conclusion.

It really helps if you can find more concrete evidence and there’s nothing quite so convincing as an actual body:

In 2010 a group of archaeologists examined 18 Egyptian mummies from around 1000 BC , and diagnosed Dupuytren’s contracture in the left hand of mummy number two. Sadly, we can’t find a photo of the hand.

In a post-medieval graveyard in St Ilija, Serbia (Serbia is another Dupuytren’s hot-spot) the skeleton of a man age 40-50 years old was found with the bones of the little finger fused into a right angle. The most likely cause for this is Dupuytren’s (when the finger can no longer be straightened, the lack of movement allows the bones to fuse together). The full article is available for download.

 

The ‘Viking Disease’

The frequency of Dupuytren’s is high in many northern European countries where the Vikings settled and thus it is often nicknamed ‘The Viking Disease’. However, there are nine or ten different genes associated with Dupuytren’s and this suggests that it may have evolved on several different occasions. Add to this the known prehistoric cases (like the Egyptian mummy) that are clearly pre-Viking and the fact that the Japanese with no Viking ancestry at all have a high incidence of Dupuytren’s, and it can be seen that the story isn’t that simple.

It does seem appear that Dupuytren’s is more frequent in populations originating in northern latitudes, like the Japanese and the Vikings, but we don’t currently know why. Dupuytren’s is rare in Indian and African populations.

 

Doctor’s History

Dupuytren’s first appears in the written medical records in Basel, Switzerland in 1614 when doctor Felix Platter (sometimes spelled Plater) examined a stonemason whose left ring finger and little finger were contracted into his palm. From Platter’s description, it seems likely that he was also able to dissect the hand after the man’s death.

In his book ‘Observationes in hominis affectibus plerisque corpori et animo functionum laesione, dolore, aliave, molestia et vitio insensis libri tres’ (observationes 3) , Platter described what he called ‘ crispatura tendineum’ (meaning ‘tendon-like crispness’).

Platter’s description and a translation can be found on here.

In 1777 AD a British doctor and anatomist Mr Henry Cline Sr., after dissecting two hands with finger contractures, correctly described the contraction as being caused by the fascia rather than the tendons.

In 1822 Cline’s student, Astley Cooper, wrote ‘A treatise on the dislocations and fractures of joints’, which included a detailed description of the what we now call Dupuytren’s contracture, and described a surgical treatment that would have been a bit like today’s needle fasciotomy , “when the aponeurosis is the cause of the contraction, and the contracted band is narrow, it may be with advantage divided by a pointed bistory, introducect through a very small wound in the integument. The finger is then extended, and a splint is applied to preserve it in the straight position.” (There are people who argue that the disease could justifiably be called ‘Cline-Cooper’s Contracture’).

Like Henry Cline, Astley Cooper practised and lectured in St Guy’s and St Thomas’s hospital in London.

In 1831 a Frenchman called Baron Guillaume Dupuytren, an anatomist and surgeon, was head physician at a famous hospital called the Hotel Dieu in Paris.

He dissected the hand of a patient who had been suffering from finger contractures, and diagnosed the cause as coming from the fascia. In his lectures at the Hotel Dieu in 1832, he described this dissection, and also a case he treated on 12th June. He operated on a man with contracted fingers (without any anaesthetic, as anaesthesia didn’t exist then) and performed an open fasciotomy (cutting through the skin and the fascia). By 2nd July the post-operative infection and inflammation had gone and the patient could use his hand again. Dupuytren didn’t suture any of the wounds, but left them open to heal. He carried out another demonstration by operating on a patient in front of the audience while he was giving the lecture.

Dupuytren claimed to have been the first to recognise the cause of the disease and to suggest a way of treating it. He repeatedly misquoted Cooper and said that Cooper believed that the disease to be incurable. Reference.

Hotel Dieu 1830, where Guillaume Dupuytren was head physician.

 

Peyronie’s

A century before Dupuytren, in 1743, Francois Gigot de la Peyronie, personal physician to King Louis XIV, who also lectured at the Hotel Dieu, examined three patients and described changes in the penis that were to become known as 'Peyronie's disease’: "rosary beads of scar tissue to cause an upward curvature of the penis during erection." Peyronie considered the condition to be caused by trauma. It’s likely that Peyronie’s name became attached to the disease because he was so well known.

The condition was known to exist before Peyronie’s day. Guilio Cesare Aranzi, a famed Italian anatomist from Bologna, wrote in 1587 in his book “Tumores Praeter Naturam”, that it was “palpable like a bean in the flaccid penis, causing a deformity similar to a ram horn during erection”.

There are earlier reports, some as early as the thirteenth century of conditions that might be Peyronie’s, but the descriptions aren’t accurate enough for certainty.

Some people claim this statue of Silenus in the National Archaeological Museum in Athens is proof of early Peyronie’s.

However, many people have some curvature of the penis without having Peyronie’s. Moreover Silenus is one of a company of phallic or half-animal tutors of the gods, a group that includes Priapus - and thus would be expected to have an exaggerated phallus. It’s not Peyronie’s if the penis does not have lumps as curvature alone is not enough. Also given the mythological nature of Silenus, it’s quite likely that his penis was modelled on that of a horse. (Note his horses hooves and tail.)

 

Garrod’s Pads

Michelangelo’s Moses, carved in the early 16th century, has visible knuckle pads on his fingers.

In 1893 a French doctor Mr A E Garrod gave a detailed description of 'knuckle pads' found at the PIP joints mainly on the 2nd, 3rd and 4th finger and suggested a relation to Dupuytren's disease, as some of the nodules showed a Dupuytren's like appearance on histology and many of his patients who had knuckle pads also had Dupuytren’s disease.

Recently there has been a suggestion that in fact maybe there are two separate conditions, Dupuytren's related 'dorsal nodules' and skin related fibromatous knuckle pads (calluses).

 

Ledderhose disease

Dupuytren mentioned in his lectures in 1832 that the condition he had described in the hand could also occur in the foot, which was presumably a reference to the condition that we now know as Ledderhose.

The first detailed reference we have is in 1894, when a German doctor, Georg Ledderhose described plantar fibromatosis, a condition affecting the plantar fascia of the feet. You can read a scan of his 1894 paper here (in German). Ledderhose examined over fifty patients with the disease and concluded it was probably caused by trauma because he observed it mainly in patients who had been wearing a splint to treat other foot problems.

 

Frozen shoulder

Frozen shoulder (where the lining around the shoulder joint becomes inflamed and stiffens up) was a condition well known to physicians by the early 1900’s, but the possible link to Dupuytren’s was only made in 1936 by H. Schaer.

 

All Conditions are Related

Initially, conditions like Dupuytren’s, Ledderhose, Peyronie’s and Garrod’s Pads were all viewed as separate diseases. However, by 1902, Janssen was writing about what he called ‘fibromatous diathesis’, with the understanding that all of those conditions were due to the same underlying cause, often now called Dupuytren’s diathesis. (diathesis means ‘disposition’)

 

Moving to the Present

Treatment has come a long way since Dupuytren’s first operation where the patients had to be restrained during surgery. Anaesthetics, introduced in1846, make surgery more comfortable for the patient and easier for the surgeon and students.The realisation of the need for cleanliness and hygiene in hospitals from 1867 onwards made a great difference to survival rates. By the late 19th/early 20th century the discovery of antibiotics made further progress possible in surgery and reduced post-operative infections.

Surgical techniques have evolved as well, from simple dermo-fasciotomy (cutting through skin and cord) to a much wider ranger of options: closed fasciotomy (cutting the cords with a needle, without cutting the skin), open fasciotomy , partial fasciectomy (removing the affected part of fascia), dermo-fasciectomy (removing the affected fascia and the skin attached to it, which usually results in a need for skin grafts) and many choices in-between.

Radiotherapy has been used since the 1980’s especially in Germany, where the incidence of Dupuytren’s is high. It is normally used in the early stages, when the patient has growing nodules and contractures are less than 10%.

In 2001 the first randomised trial was done, to find the best radiotherapy treatment protocol.

In 2010 a follow-up study was done which showed lumps shrinking in most patients (and no cancers). In those patients who needed surgery, the previous radiotherapy caused no problems. The main side-effect of radiotherapy was thin and dry flaky skin, found in 32% of patients.

In the 1980’s New York academic surgeon Dr L C Hurst was researching the role of prostaglandins (a type of tissue hormone that plays a role in inflammation) in Dupuytren’s Contracture, when a colleague Mr L T Wegman asked his help in using collagenase (an enzyme that breaks down collagen) to prevent scar tissue formation in nerve repairs. Dr Hurst realised collagenase could be useful in Dupuytren’s disease (because the lumps and cords are mainly made of collagen), and thus started the research that finally led to commercially available Collagenase injections. Reference.

Meanwhile the research into the causes of the disease was taking off as well.

In 1980, myofibroblasts (fibroblasts are fibre producing cells and myofibroblasts are a type of fibroblast that are associated with contraction) were identified in Dupuytren’s tissue by electron microscope.

In 1991 research showed that when skin adheres to the nodules/cords it is best to remove that skin as well, as it contains myofibroblasts that can cause recurrence. The number of myofibroblasts found in Dupuytren’s tissue can help to predict the chance of recurrence after surgery.

In 1995 research began on Dupuytren’s tissue for growth factors that were known to affect myofibroblasts and scar tissue. TGF-ß 1-3 (transforming growth factors beta 1, 2 and 3) are found and produced in nodules. TGF-ß 2 was later found to be the most important one.

Last, but not least, geneticists are trying to find the genetic causes of Dupuytren’s and are making some progress. There are at least nine genes involved. Some are inherited via the mother and some can come from either parent.

We’re much closer to understanding the causes of Dupuytren’s than our ancestors were, and we have much better treatments available, but for for the present at least, we still have no cure.

Who knows what the future may bring?