The disease
Dupuytren’s disease, Morbus Dupuytren or palmar fibromatosis, sometimes also called DD, Dupuytren’s contracture, or Viking disease, is a chronic disease that produces a benign thickening of the connective tissue, or fascia, of the palm and fingers. It usually begins with a small lump, called a nodule, in the palm or fingers that is often mistaken for a blister or callus. Eventually, string-like cords may develop beneath the skin and, if the condition progresses, the affected fingers are pulled towards the palm and cannot be straightened anymore. This condition is then known as Dupuytren’s contracture.
Dupuytren’s disease is one of a group of diseases which result in fibrosis or abnormal scar tissue. Dupuytren’s may run in families and is also associated with other related conditions that you can read about here. Although treatments and management strategies are available for this disease, there is currently no cure. The causes are still unknown.
Dupuytren’s is named after a French doctor, Baron Guillaume Dupuytren, who studied the condition in 1832.
Ledderhose disease (Morbus Ledderhose, plantar fibromatosis) is similar to Dupuytren’s disease, but with nodules typically developing in the arch of the foot. These nodules can become painful or grow to a size where they affect walking. Therapies for Ledderhose disease are generally similar to those for Dupuytren’s disease but differ in detail due to the different mechanics of hands and feet.
Ledderhose is named after a German surgeon, Dr. Georg Ledderhose, who described the condition in 1894.
Progression of Dupuytren’s disease
After their initial growth, the small nodules or cords typically keep their size for about five or six years. Then, the contraction process starts to bend the fingers. It is usually only at this late stage that patients start to seriously consider an efficient treatment, generally surgery.
It is less well known that radiotherapy is highly efficient in the early stages of the disease.
Based on this total angle of contracture, the development stages of Dupuytren’s disease are usually classified as follows.
| STAGE | CONTRACTURE | COMMENTS |
| 0 | 0 | Healty |
| N | 0 | Feel nodules/cords |
| N/1 | 0-5 degrees | Beginning contracture |
| 1 | 6-45 degrees | |
| 2 | 46-90 degrees | |
| 3 | 91-135 degrees | |
| 4 | >135 degrees |
Statistical prevalence
DD is the most common hereditary disease of the connective tissue which largely affects Caucasians originating from Northern Europe. The genetic mode of inheritance, however, is not well understood.
France and UK have prevalence rates from 6 to 10 percent of the total population. Canada’s history, with its massive colonization from northern France and England, and waves of immigration from Ireland, tends to suggest a high prevalence in Canada, although this is still insufficiently documented.
Some environmental factors are associated with DD, namely alcohol consumption, tobacco exposure and manual activities.
Ledderhose Disease, Diabetes and epilepsy are also the most frequently reported DD-associated diseases.
Association with other conditions
LEDDERHOSE DISEASE
Ledderhose disease, sometimes called plantar fibromatosis or “Dupuytren’s of the foot,” is characterized by lumps called nodules on the underside of the foot. These are caused by a thickening in the deep connective tissue (the fascia) in the foot and can cause discomfort or pain when walking. The overlying skin is freely movable. Ledderhose occurs in both feet (bilateral) in about 25% of patients.
FROZEN SHOULDER OR ADHESIVE CAPSULITIS
Frozen shoulder or adhesive capsulitis, causes pain and loss of motion in the shoulder joint. Although it is more of a condition than a definite disease, it is often associated with Dupuytren's disease.
Adhesive Capsulitis : Diagnosis and Management
PEYRONIE’S DISEASE
Peyronie's disease, also known as Morbus Peyronie or Induratio Penis Plastica (IPP), results in a curved penis due to the accumulation of collagen and tissue contraction. This condition is possibly due to a genetic predisposition.