Dupuytren’s Disease

By: Zombieland

Although many factors may contribute to the predisposition of Dupuytren’s Disease, there has been conflicting evidence of correlation and causation found in relation to alcoholism or hard-labor work. Many studies over history have failed to establish a definitive link between alcoholism, hard-labor work and Dupuytren’s Disease, but rather the only proven correlating link is solely heredity.

Dupuytren’s Disease, also known as “Viking’s Disease”, is a condition causing debilitating contractures (flexing, rigidity, or clawing) of the fingers and can progress to limited or full loss of hand function. The disease occurs most often in men of Northern European decent that are over the age of 60 (women can also develop the disease, but are less likely than men). Dupuytren’s is estimated to affect about 10% of the population of men aged over 65 currently living in Northern Europe 4. Dupuytren’s Disease has a well-established history, whose transmission is believed to stem from the 10th century invasions by Scandinavian Vikings. Once colonization of Scotland, Ireland, England, France, Holland and Belgium was established by the Vikings, the disease began to be passed on through intercultural relationships1.

Dupuytren’s Disease was first described by Swiss physician Felix Plater in 1614 2, but its namesake is derived from Baron Guillaume Dupuytren who, later in 1831, recorded multiple lectures regarding the disease and its origin and also performed the first surgery on a hand contracture of this type. Dupuytren, known as the greatest French surgeon of the 19th century, described seeing an increase of male patients of Irish descent presenting with permanent flexed fingers. Through research of his own, he concluded that the disorder was caused by an increase or thickening in the palmar fascia (a tough, fibrous layer of tissue in the palm of the hand5). Dupuytren also concluded that there were strong indicating factors such as Irish descent, alcoholism, and manual labor contributing to the cause of the disease, as most of his presenting patients were immigrant Irish manual laborers who drank excessively. This foundation of knowledge also brought to light “The Curse of the Mac Crimmons”, which was a belief that the Mac Crimmon family, who ran The College of Bagpiping in Scotland from the 15th century to the 18th century, had a curse laid upon the men in the family that inflicted bent fingers in the adult male bagpipers and prevented them from playing later in life1.

These bent fingers were described by Dupuytren in his lectures as a bend of the finger (contracture). Contractures can develop in a variety of number of digits, but most occur in the ring and small fingers. The degree of contracture can vary greatly depending on each individual’s disease process. Along with these contractures, nodules (small lumps) were described to be present on the palms of the hands. These nodules are usually firm and round and may cause the skin of the palm to pucker or pit. Although these hallmark characteristics observed in someone with Dupuytren’s Disease is often debilitating, it is usually not painful. The rate of development of contractures and nodules is different with each individual. Some patients may have nodules but never develop contractures and some patients may have both that progressively and rapidly worsen. Variabilities exist between the least severe case and the worst severe case. Patients often seek medical advice when the bent finger(s) impedes normal, everyday functions. Patients often describe that the contracted finger will snag on the pocket of their pants or that the finger will poke them in the face as they are attempting to wash. Many seek what treatments are available from an Orthopedic Hand Surgeon, who has specialized training in the diagnosis and treatment of Dupuytren’s Disease. Treatments include injections to dissolve collagen and multiple degrees of surgery, but there is no cure. Dupuytren’s Disease does recur and the rate of recurrence is dependent upon the individuals’ disease process 3.

Guillaume Dupuytren may have monumentally described the basic origin and characteristics of the disease, but many discoveries have been made regarding the actual process of development. It is widely agreed upon that the formation of contractures results from a tight cord that forms from an increase in collagen growth, promoted by cells called myofibroblasts (a cell that shares characteristics of both smooth muscle cells and fibroblast cells). This tight cord can be likened to a rope and is comprised mostly of collagen type III; normal palmar fascia is predominantly made up of collagen type II. The nodules that form, likened to a knot, are dense, highly cellular (many cells) masses of tissue. Cords and nodules can be exclusive from each other but nodules may also present on or within the cord, like knots in a rope 3.

The evolution of the discovery of Dupuytren’s Disease and its cause has been controversial for hundreds of years. Many researchers believe that the previously stated predicting factor of alcohol use was simply an oversight on behalf of Guillaume Dupuytren, who assumed that alcohol was a causative agent in the development of the disease, and failed to recognize a factor of culture in Irish and Scottish descent where heavy alcohol use was widely accepted and practiced. Other researchers, more recently in the last century, believe they have found a link that does relate to alcohol use being a contributing factor, by means of exciting myofibroblast cells and in turn increasing collagen growth in the hand 3. Controversy also surrounds the original idea that manual labor contributes to Dupuytren’s contractures. Some researchers have documented that the correlation that Guillaume Dupuytren made based on his Irish immigrant patients was, again, an oversight of coincidence; most Irish immigrants in France during the 19th century were manual laborers. On the other hand, some recent research indicates that trauma to the hand may trigger a repair process to begin where cytokines (small proteins that aid in cell signaling) are released and tell the myofibroblast cells to increase their number to repair the injury. This subject is loosely discussed in relationship with the original idea that manual labor contributes to the disease process, but more-over believed to be a possibility that repetitive, hard labor of the hand causes small injuries to occur, which in-turn trigger this process of repair 3.

There may be controversy and argument surrounding the causation of Dupuytren’s Disease but three things are widely agreed upon: (1) the cause of the disease is multifactorial in nature 4, (2) onset and prevention of progression has yet to be discovered 4 and (3) congenital predisposition is the only confirmed correlation of transmission, causation and recurrence although no specific gene has yet to be isolated 3.

Citations:

1 Flatt, A. E., MD. (2001). The Vikings and Baron Dupuytren’s disease. Baylor University Medical Center Proceedings, 14(4), 378-384. Retrieved February 10, 2016, from www.ncbi.nlm.nih.gov/pmc/articles/PMC1305903

2 Desai, S. S., MD. (2011). The Treatment of Dupuytren Disease. Journal of Hand Surgery, 36(A), 936-942. Retrieved February 10, 2016 from www.jhandsurg.org

3 Black, E. M., MD, & Blazar, P. E., MD. (2011). Dupuytren Disease: An Evolving Understanding of an Age-old Disease. Journal of the American Academy of Orthopaedic Surgeons, 19,746-757. Retrieved February 10, 2016 from www.aaos.org/jaaos

4 Frey, M., MD. (1997). Risks and prevention of Dupuytren’s Contracture. The Lancet, 350, 1568. Retrieved February 10, 2016

5 Krames. (2014). Dupuytren’s Contracture- Restoring Movement in Your Hand. www.kramesstaywell.com