Dupuytren's contracture

The Dupuytren's contracture ( Dupuytren's contracture) is a benign disease of the connective tissue in the palm of the hand ( palmar fascia ). In 1832, Baron Guillaume Dupuytren presented ( 1777-1835 ) before the disease named after him in Paris. The underlying cause could not be found until today. The Dupuytren's disease belongs to the group of fibromatosis. It affects mostly the ring or little finger, but any fingers be affected.

Epidemiology

The Dupuytren's disease usually occurs in middle age, 85 % of affected patients are men whose disease typically occurs earlier than in women. Dupuytren's contracture, there are mainly in central and northern Europe and North America, less frequently in Africa and Asia. More recently, some cases from Japan and China have been reported. The prevalence is highly variable and is in the western industrial countries 2-42 %. The disease is more common with increasing age. It is associated with alcoholic liver injury and tobacco smoking and diabetes mellitus. In addition, there is a strong familial aggregation. Typically small and ring fingers are affected, contractures are found almost exclusively in the medium metacarpophalangeal joints - and. In half of the cases of M. Dupuytren found on both hands, the right hand total is affected slightly more often. A genetic component is well established; every third concerned a family member is also affected by the disease.

Symptoms and course

Characteristic of the disease is the occurrence of lumps and strings in the palm of the hand.

The natural history is variable, and progression is often slow for years instead. However, a standstill is possible as well and at any stage. It often takes years for the first palpable, nodular or cord-like induration also leads to a limitation of mobility in the metacarpophalangeal and medium - joints ( flexion contracture ). Usually is only at this late stage of treatment, particularly intended for an operation.

The thumb is the third most affected by the ring and little fingers. Studies show an involvement 3-28 %. This leads to painful nodules between the first and second metacarpal bones and the area of ​​the thenar. Especially extension and abduction (abduction ) of the thumb are restricted.

Fibromatosis

Similar and well- related diseases are Crohn's Ledderhose, that is, the corresponding condition on the inside of the feet ( plantar aponeurosis ), which is found in 5-20% of patients with M. Dupuytren, and possibly the penile curvature ( at 4 %), and Peyronie's disease or penis plastica Induratio called. Occasionally, symptomatic growths similar to the abdominal wall ( nodular fasciitis ) are associated with Dupuytren's disease, as well as the retroperitoneal Ormond's disease.

These diseases are often summed up as fibromatosis, among benign tumor-like Bindegewebswucherungen be understood, which can often grow aggressively. The growths go of myofibroblasts, and there are in addition to genetic factors, disorders of estrogen metabolism discussed as causes in 2011 were various changes in the Wnt signaling pathway are associated with the occurrence of the disease.

The WHO classified the palmar and plantar fibromatosis as the separate entity "superficial fibromatoses " (superficial fibromatosis ) in the large group of fibroblastic and myofibroblastic tumors

Differential Diagnosis

The pattern with the palmar nodules is fairly typical, so the diagnosis usually is not difficult. Rarely is a stenosing tenosynovitis in question, the camptodactyly is to be distinguished.

Therapy

Conservative measures such as ointment dressings, medicines, physiotherapy and massages have no chance of success. In early stages, the irradiation of the palm a promising therapeutic option represents a further possible treatment consists of surgical removal of the entire affected tissue (open fasciotomy ). It should in this case not be operated on early, but only when it has come already beginning to yield disabilities of the fingers ( about 45 °) or if pain persist. Another option is the Nadelfasziotomie, also called Fibrosenperforation. Their advantage is that they can be on an outpatient basis and without anesthesia, also repeatedly carried out.

A new method consists of the injection of bacterial collagenase ( enzymes I and AUX AUX II from Clostridium histolyticum ), which enzymatically destroys the scarred strands, followed by physiotherapy mobilization. In a first large clinical trial, a full extension of up to 5 ° extension deficit could be achieved in this case 64%, compared to 6.8% in the placebo group. The stretching ability has improved over the metacarpophalangeal joints by an average of 41 ° in the proximal interphalangeal joints by an average of 29 °. It often occurred bruising, sometimes even cracks in the skin on, almost all completely healed. A clinical use of general does not exist. The long-term effect of this study, however, was questioned because the observation period was too short relative to the rate of recurrence to provide meaningful results ..

As this drug Microbial collagenase (trade name Xiapex ®; manufacturer Pfizer ) was approved in May 2011 in the European Union, after a negative benefit assessment by the IQWiG presented Pfizer but in May 2012 the sales in Germany first one.

Another treatment option is the irradiation of the palms with Röntgen-/Gammastrahlen. The irradiation treatment is established particularly in the early stages in order to prevent the progression of the disease. In a study of 135 patients (208 hands) in the early stages was an irradiation of 5 x 3 Gy ( 5x/Woche ) in 2 series with an interval of 6-8 weeks. Long-term results after median 13 years (range 2-25 years) reported in 59 % to a constant finding an improvement in 10% and progression in 31 %. Late side effects were deplored by 32 % of patients. Statements about the extent of movement were not available, the documentation of findings was not quantitative, it also lacked a comparison group.