Dupuytren's disease

Dupuytren's disease or palmar fibromatosis is a pathological thickening of the palmar aponeurosis and the palmar subcutaneous tissue. As disease progresses this results in flexion contractures of the hand, particularly of the 3rd, 4th and 5th digits.

Epidemiology

While only about 7% percent of the general population is affected, about 15-20% of patients with diabetes will get Dupuytren's disease. In diabetes, men and women are equally affected, whereas in the general population the ratio of men to women is 6 to 1. Risk factors for the disease include Caucasian descent, high age and smoking. Although diabetes clearly is a risk factor, the duration of diabetes and glycaemic control do not seem to influence the risk. Interestingly, in diabetes the disease seems to have a more radial presentation and it also seems to follow a milder course.

Pathogenesis

Two factors seem to play a role. In line with the common pathogenesis of connective tissue disorders in diabetes advanced glycation of the connective tissue is found, with a relative increase in the non-elastic type III collagen fibers. However, there are also indications that Dupuytren's disease is a T-cell mediated auto-immune disorder.

Diagnosis and treatment

The diagnosis is made by inspection an palpation of the palmar aponeurosis, which thickens already before the occurence of the characteristic contractures. While the disease is painless, these contractures can be very invalidating. Surgical intervention will often result in improvement, but unfortunately recurrence is frequent.

Plantar fibromatosis (Morbus Ledderhose)

This is the equivalence of Dupuytren in the feet. however, it occurs far less frequently. Interestingly, in type 1 diabetes the thickness of the plantar aponeurosis seems to be predictive for the risk of subsequent microvascular complications.

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