Definition

Dupuytren disease is a nonmalignant fibroproliferative disorder causing progressive and permanent contracture of the palmar fascia; subsequent flexion contracture usually begins with the fourth and fifth digits on the ulnar side of the hand (Fig. 29.1). The eponym Cooper contracture has been suggested for Astley Cooper, who first described and lectured on the entity in 1822. A nodule in the palm is the primary lesion in Dupuytren contracture. It is a firm, soft tissue mass fixed to both the skin and the deeper fascia. It is characterized histologically by dense, noninflammatory, chaotic cellular tissue and appears on the anterior aspect of the palmar aponeurosis.

The key cell response for tissue contraction in Dupuytren disease is thought to be the fibroblast and its differentiation into a myofibroblast [1]. This idiopathic activation happens in response to the fibrogenic cytokines interleukin-1, prostaglandin F₂, prostaglandin E₂, platelet-derived growth factor, connective tissue–derived growth factor, and, most important, transforming growth factor-β and fibroblast growth factor 2. In addition, microRNAs (miRNAs) identified in Dupuytren contracture samples, including miR-29c, miR-130b, miR-101, miR-30b, and miR-140-3p, were found to regulate important genes related to the β-catenin pathway: WNT5A, ZIC1, and TGFB1 [2]. As the nodule extends slowly, it induces shortening and tension on the longitudinal fascial bands of the palmar aponeurosis, resulting in cords of hypertrophied tissue. It is unique among ailments of the hand, and one could conceive of it as a focal autoimmune collagen vascular phenomenon. Dupuytren disease is thought to begin in the overlying dermis. Unlike the nodule, the cord is strikingly different histologically; it contains few or no myofibroblasts and few fibroblasts in a dense collagen matrix with less vascularity. Skin changes are the earliest signs of Dupuytren disease, including thickening of the palmar skin and underlying subcutaneous tissue. Rippling of the skin can occur before the development of a digital flexion deformity [3].

A controversy exists as to whether there is a relationship between Dupuytren disease and repetitive microtrauma [4], but more recent meta-analysis does seem to suggest some degree of occupational correlation with manual work and vibration exposure [5]. It is now thought that microruptures of the palmar fascia are related to the contracture rather than a primary cause [6]. Cessation of manual labor and immobilization can lead to acceleration of the disease, which has been noted in laborers after retirement [7]. One study seems to suggest that there may be an association with rock climbing in men [8].

A genetic predisposition is thought to be inherited as an autosomal dominant trait with variable penetrance [9]. Family history is often unreliable as many individuals are unaware they have family members with the disease. Dupuytren disease has been termed Viking disease [10] because it has a high prevalence in areas that were populated by the Vikings and where the Vikings migrated. Global prevalence is 3% to 6% of the white population, and it is rare in nonwhite populations. Dupuytren disease occurs more commonly in the elderly but tends to be associated with greater functional compromise in younger patients. Women are affected half as often as men [11]. There is no relationship to handedness; however, affected individuals tend to complain more frequently about the dominant hand. Other associations with the condition include diabetes mellitus [12] (specifically with an increased risk from diet-controlled diabetes to sulfonylureas to metformin to insulin requiring), alcohol consumption [13], cigarette smoking [13,14], human immunodeficiency virus infection [15], and antecedent Colles fracture. Conflicting reports exist of an association with epilepsy, but antiepileptic drugs do not present an increased risk [12,16].

There are potential secondary findings in Dupuytren disease that are rarely seen but when present suggest a strong Dupuytren diathesis (genetic penetrance of the disease). These findings include knuckle pads (Garrod nodes), plantar fascial disease, and Peyronie disease. The contractile tissue in all of these conditions resembles the pathologic findings of Dupuytren disease in the palm [17], and alterations in the expression of certain gene families, fibroblast to myofibroblast differentiation among others, are similar [18]. However, these associated conditions are found in only an estimated 1% or less of patients with Dupuytren disease [19]. All patients with the disease have a diathesis. The association with these conditions as well as onset at an early age and family history suggests that the diathesis is strong. Recognition of a strong diathesis is important for planning an appropriate rehabilitation protocol, including long-term follow-up and awareness of possible poor prognosis and likelihood of recurrence with surgical treatment.

Symptoms

Dupuytren disease typically has a painless onset and progression. Decreased range of motion, loss of dexterity, and getting the hand “caught” when trying to place it in one’s pant-pocket are common presenting symptoms. Pain can be a result of concomitant injuries to the hand and fingers that can precede the development or worsening of Dupuytren disease. Abrasions or ecchymosis to the distal interphalangeal and proximal interphalangeal joints of the affected digits may be seen and may be the reason for the initial consultation. With use of the relatively new Dupuytren disease scale of subjective well-being of patients questionnaire, which covers four areas of the quality of life, there were no differences in quality of life in patients affected in the left or right hand regardless of hand dominance of the patients [20].

The progression of the condition is generally considered to be a result of immobility after an injury in a predisposed individual rather than of the injury itself. Pure sensory symptoms in digits four and five may arise from palmar digital nerves against the relatively inelastic deep transverse metacarpal ligament.

Physical Examination

The most common first sign of Dupuytren disease is a lump in the palm close to the distal palmar crease and in the axis of the ray of the fourth digit (ring finger) (Fig. 29.2). It can also be manifested in the digit, generally over the proximal phalanx. The thumb and index finger are the least affected of the five digits. The nodule can be tender to palpation. In most cases, the skin is closely adherent to the nodule, and movement with tendon excursion often suggests other conditions, such as stenosing tenosynovitis. The condition is more readily apparent when it is manifested in a more advanced stage with palmar nodule, cord, and digital flexion contracture. Conditions associated with this disease include fat pads at the knuckles and evidence of the disease in the plantar fascia. “Swan-neck” deformity as a dorsal variant of Dupuytren disease has been suggested [21].

The examination should evaluate the range of motion and kinetic chain of the entire upper limb, including associated adhesive capsulitis, epicondylitis, and other tenosynovitis. Sensory, manual motor, and muscle stretch reflex components of the neurologic examination should be normal.

Functional Limitations

The majority of individuals with this condition have little functional limitation early on. With more advanced contracture, properly opening the palm and grasping can become difficult, making gripping activities such as activities of daily living, opening cans, buttoning shirts, and placing keys in automotive ignitions troublesome. In many cases, the insidious onset allows gradual compensation, and outside observers may notice irregularity during a simple hand shake.

Diagnostic Studies

The diagnosis of Dupuytren disease is generally made on a clinical basis. Biopsy is considered when a palmar soft tissue mass cannot be reliably differentiated from sarcoma [22