Dupuytren’s disease: How to recognize its early signs

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Applied Evidence

Dupuytren’s disease: How to recognize its early signs

J Fam Pract. 2017 March;66(3):E5-E10

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References

1. DiBenedetti DB, Nguyen D, Zografos L, et al. Prevalence, incidence, and treatments of Dupuytren’s disease in the United States: results from a population-based study. Hand. 2011;6:149-158.

2. Townley WA, Baker R, Sheppard N, et al. Dupuytren’s contracture unfolded. BMJ. 2006;332:397-400.

3. Rayan GM. Clinical presentation and types of Dupuytren’s disease. Hand Clin. 1999;15:87-96.

4. Hindocha S, Stanley JK, Watson S, et al. Dupuytren’s diathesis revisited: Evaluation of prognostic indicators for risk of disease recurrence. J Hand Surg Am. 2006;31:1626-1634.

5. Bayat A, McGrouther DA. Management of Dupuytren’s disease—clear advice for an elusive condition. Ann R Coll Surg Engl. 2006;88:3-8.

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7. Wilbrand S, Ekbom A, Gerdin B. The sex ratio and rate of reoperation for Dupuytren’s contracture in men and women. J Hand Surg Br. 1999;24:456-459.

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Rule out possible DD mimics

The differential diagnosis for flexion contracture of the MCP or PIP joints seen in Dupuytren’s disease includes stenosing flexor tenosynovitis, or trigger finger. Other conditions that can mimic DD are ulnar claw hand, trauma scars, intrinsic joint diseases such as degenerative or rheumatoid arthritis (RA), diabetic cheiroarthropathy, camptodactyly, and Volkmann’s contracture. Trauma scars—especially longitudinal scars—have a tendency to contract and develop keloid formation.

It's not known which patients with early Dupuytren changes will progress to severe contracture.

Stenosing flexor tenosynovitis and ulnar claw hand are distinguishable from DD by full or nearly full active or passive extension of the affected digit, whereas DD is a true contracture of the joint that does not allow full extension. A careful history can rule out previous injury to the area. Although intrinsic joint disease, such as RA, can cause finger contractures, the joints are usually enlarged, painful, and associated with characteristic radiologic findings.

Unlike DD, diabetic cheiroarthropathy often involves all of the digits except for the thumb, and is often associated with a waxy appearance of the skin. Camptodactyly is an autosomal dominant disorder that more often presents in childhood and can be caused by a number of congenital syndromes. Volkmann’s contracture can manifest as a claw-like deformity of the hand caused by undiagnosed compartment syndrome of the forearm.

Assess the severity of DD

In your evaluation, first identify palmar or digital fibromatosis presenting as a nodule or a cord. Second, estimate the degree of MCP and PIP joint contractures. A common measure of contracture is the Hueston tabletop test. Ask the patient to place the palm of the hand on a flat surface. If the patient is unable to completely flatten the hand against the surface, presume a positive result (FIGURE 2).¹⁶

An accurate measure of the degree of flexion can be accomplished with a goniometer. For a simple assessment of severity, have the patient place each affected finger along the convexity of a spoon. If adjoining surfaces are flush, assume that the contracture is at least 30 degrees (FIGURE 3). The severity of DD can also be graded according to the Tubiana classification system (TABLE¹⁷), wherein the total deformity or contracture score is the sum of the angles of all 3 digital joints of the finger.

A look at the treatment options

Once the diagnosis of DD is made, reassure the patient that the disease is not cancerous, although it may be progressive. Advise patients, too, that several treatment options (detailed below) are available, but that surgery is the mainstay of treatment. (Of note: Despite what would seem logical, stretching a cord to straighten the finger is not recommended, as it may actually worsen the condition.¹⁸)

Options for early DD. Corticosteroids such as triamcinolone may have a role as an adjunct for early DD by reducing the size and firmness of the Dupuytren’s nodules and possibly slowing the progression of the disease. However, in one study of 63 patients, half of the individuals experienced reactivation of disease between one and 3 years.¹⁹

Radiotherapy has been studied as a potential treatment for early DD and for patients unable to undergo surgery. Radiation does have a biologic effect on nodular DD, but has no effect on the cords that cause the joint contracture. In a long-term follow-up study of 135 patients treated with radiotherapy, disease remained stable in 59% of patients, improved in 10%, and progressed in 31%.²⁰ Results were better for early stage disease. This modality is not used for more advanced cases.

Surgery is the primary method by which to restore function and minimize complications. In the past, referral was recommended for MCP joint contractures >30 degrees or for any involvment of the PIP joint, because involvement of the latter carries a worse prognosis and may not be fully correctable, even with surgery.²¹ However, treatment may also be warranted in cases of functional disability—regardless of the degree of contracture (eg, for pain associated with a prominent nodule or cord when gripping objects).

Although fasciectomies have shown the best efficacy and lowest recurrence rates, complications are more likely with these procedures.Depending on disease progression and the surgeon’s preference, the most popular procedures are fasciotomies and fasciectomies. Surgical options for contractures—in increasing order of invasiveness and amounts of fascia removed—are fasciotomy (needle, open), fasciectomy (partial, radical), and dermofasciectomy.

Needle fasciotomy is an outpatient procedure performed under local block. The surgeon uses a needle bevel to transect the diseased cord at multiple puncture sites.

Open fasciotomy is also an outpatient procedure involving an incision that allows direct visualization of the cord and internal structures before dividing the fascia. Percutaneous and open fasciotomies are more successful for MCP joint contractures and less successful for PIP joint contractures.

With partial fasciectomy, only the grossly affected fascia is removed.

With radical fasciectomy, the entire palmar fascia is removed, regardless of which areas are grossly diseased.

Dermofasciectomy removes diseased fascia and the overlying skin, with a skin graft applied to heal the wound.

Another option: Collagenase injection. In February 2010, the US Food and Drug Administration approved collagenase clostridium histolyticum (Xiaflex) as the first drug specifically indicated for the treatment of Dupuytren’s contracture. Collagenase is injected directly into the fascial cord, cleaving its collagen component and progressively softening and breaking down the cord. If not properly injected, collagenase may injure neurovascular structures and may rupture tendons.