Dupuytren's Disease
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ONLINE EXCLUSIVE Thomas Auld, MD; Joanne R. Werntz, MD Dupuytren’s disease: Department of Orthopedic Surgery, Rutgers New Jersey Medical School, Newark (Dr. How to recognize its early signs Auld); University of Central Florida School of Medicine, Palmar skin dimpling and nodule or cord formation Orlando (Dr. Werntz) signal advancing disease. Treatments vary in [email protected] The authors reported no potential conflict of interest effectiveness—in part, depending on condition severity. relevant to this article. CASE u A 52-year-old right-hand-dominant white man arrived PRACTICE at our clinic complaining that he was unable to straighten RECOMMENDATIONS his right ring finger. He had no associated pain or numbness, ❯ Evaluate patients with and had not injured his hand. The patient had type 2 diabetes suspected Dupuytren’s disease that was controlled with metformin. He had no history of sur- (DD) for coexisting conditions gery or drug allergies, did not smoke, and said he drank 2 to such as Ledderhose disease and Peyronie’s disease. C 3 alcoholic beverages per day. He was a car salesman and was self-conscious when shaking hands with customers. On physi- ❯ Use the Hueston cal examination, we noted that he held his right ring finger tabletop test to assess at roughly 45 degrees of flexion at the metacarpophalangeal severity of DD. C joint; a painless cord-like structure was palpable on the palmar ❯ Do not recommend surface of that joint. His left hand had no abnormalities. stretching exercises If this were your patient, how would you proceed? for DD; they can hasten disease progression. C upuytren’s disease (DD) is a disabling fibroprolif- Strength of recommendation (SOR) erative disorder of the hand for which there is no A Good-quality patient-oriented cure. While the exact cause of DD is unknown, it has evidence D been linked to a number of risk factors, including smoking, B Inconsistent or limited-quality patient-oriented evidence alcohol consumption, and diabetes. It affects about 5% of the C Consensus, usual practice, US population, and up to 70% of affected individuals may opinion, disease-oriented 1 evidence, case series initially seek treatment from a primary care physician. The disease is also referred to as Dupuytren’s contracture, which describes the flexion contractures of fingers at the end stage of the disease. Palmar fibromatosis is yet another name for the disorder. DD refers to a spectrum of presentations ranging from nodules to cords to discernible contractures, and it is not known which patients with early Dupuytren changes will prog- ress to severe contracture. With recognition of early changes, nonsurgical intervention is possible, such as collagenase injec- tion or percutaneous fasciotomy, and can slow the progression of DD, restore function, and avoid or delay surgical interven- tion. DD is a clinically challenging disorder. Treatment for an affected area may resolve symptoms, only to have them recur in that location or another. CONTINUED JFPONLINE.COM VOL 66, NO 3 | MARCH 2017 | THE JOURNAL OF FAMILY PRACTICE E5 FIGURE 1 The nodule, cord, and contracture seen in Dupuytren’s disease A B C PHOTOS COURTESY OF: JOANNE R. WERNTZ, MD PHOTOS COURTESY Skin dimpling and a palpable nodule are evident near the base of a patient’s ring finger (A). A well-defined cord associated with the little finger is causing minimal misalignment (B). Severe contracture at the proximal interphalangeal joint (C) can interfere with routine daily activities. How underlying pathology proximal interphalangeal (PIP) joints develop correlates with clinical findings flexion contractures. The distal interpha- DD affects the palmar fascia, a thick trian- langeal (DIP) joint, rarely involved, instead gular-shaped sheet of dense fibrous collag- exhibits a hyperextension contracture. Digi- enous connective tissue that lies deep to the tal flexion contractures are often disabling, dermis and superficial to the flexor tendons interfering with daily activities such as pick- of the hand with fibers extending both into ing up a glass, shaking hands, or putting the skin and into the deep tissue. The palmar one's hand in a pants pocket. Many patients fascia secures the skin during gripping and seek medical attention only after a palpable twisting motions, and it bifurcates into distal nodule, cord, or flexion contracture becomes extensions, called pretendinous bands, that apparent (FIGURE 1). overlay and mimic the flexor tendons. In a study of 326 patients who reported Clinical findings reflect the progression Dupuytren’s symptoms, the most common of underlying pathology. The earliest mani- symptoms that led them to seek treatment festation of DD is painless dimpling of the were, in descending order, a hard bump skin on the palmar surface of the hand.2 Over (48%), a ropelike growth (12%), dimpling time, the underlying fibrosis with increased (11%), and finger contractures (10%). Only collagen deposition can progress, leading 9% of patients seeking treatment for hand to development of nodules and eventually, symptoms associated with DD had received cords, which are sometimes mistaken for a diagnosis of DD from their initial medical flexor tendons. Dupuytren-like fibrotic tissue encounter, causing an unnecessary delay in can occur on the sole of the foot (Ledderhose treatment.1 disease) and penis (Peyronie’s disease).2,3 In patients with these coexisting condi- tions, prognosis is generally worse.4 With Who is at increased risk for DD? the hands, bilateral involvement is common, DD is most often seen in elderly white men although it is usually more severe in one of European descent.5 In the United States, hand. The ring finger is the digit most fre- the prevalence of the disease is roughly 5%,1 quently involved, followed by the little finger, compared with 4% to 39% in northwestern middle finger, and index finger. The thumb is Europe (eg, Iceland).6 The male-to-female rarely affected.3 ratio of DD ranges from 6:1 to 15:1.7 The prev- As the disease progresses and cords con- alence of DD appears to increase with age. tract, the metacarpophalangeal (MCP) and The prevalence in men and women is simi- E6 THE JOURNAL OF FAMILY PRACTICE | MARCH 2017 | VOL 66, NO 3 DUPUYTREN'S DISEASE lar up to age 45 years, after which the rate is FIGURE 2 much greater in males.7 Patient can’t flatten hand DD is associated with many risk factors including smoking, alcohol consumption, vascular insufficiency, epilepsy, hyperlip- idemia, manual labor, occupations with exposure to vibration, hand trauma, and even hand surgery such as carpal tunnel release or trigger finger release.8-11 It is also associated with diabetes; particularly type 1 insulin- dependent diabetes.12 There may also be an association with frozen shoulder.12,13 The need for surgical treatment of DD Presume Dupuytren’s disease when a patient with becomes more likely with a history of ciga- a hand nodule or cord is unable to flatten the palm rette smoking and heavy alcohol consump- of the hand against a table surface. tion. There seems to be an association with epilepsy, most likely from anti-epileptic drugs.14 Rheumatoid arthritis is the only con- dition that has been associated with a lower thumb, and is often associated with a waxy incidence of DD, possibly because of the appearance of the skin. Camptodactyly is It's not known use of anti-inflammatory drugs.15 There are an autosomal dominant disorder that more which patients genetic differences between patients with often presents in childhood and can be with early and without DD, although a “Dupuytren’s caused by a number of congenital syndromes. Dupuytren gene” has not been identified. Volkmann’s contracture can manifest as a changes will claw-like deformity of the hand caused by progress undiagnosed compartment syndrome of the to severe Rule out possible DD mimics forearm. contracture. The differential diagnosis for flexion con- tracture of the MCP or PIP joints seen in Dupuytren’s disease includes stenosing Assess the severity of DD flexor tenosynovitis, or trigger finger. Other In your evaluation, first identify palmar or conditions that can mimic DD are ulnar claw digital fibromatosis presenting as a nodule hand, trauma scars, intrinsic joint diseases or a cord. Second, estimate the degree of such as degenerative or rheumatoid arthritis MCP and PIP joint contractures. A common (RA), diabetic cheiroarthropathy, campto- measure of contracture is the Hueston table- dactyly, and Volkmann’s contracture. Trauma top test. Ask the patient to place the palm of scars—especially longitudinal scars—have the hand on a flat surface. If the patient is a tendency to contract and develop keloid unable to completely flatten the hand formation. against the surface, presume a positive result Stenosing flexor tenosynovitis and (FIGURE 2).16 ulnar claw hand are distinguishable from DD An accurate measure of the degree of by full or nearly full active or passive exten- flexion can be accomplished with a goniom- sion of the affected digit, whereas DD is a true eter. For a simple assessment of severity, have contracture of the joint that does not allow the patient place each affected finger along full extension. A careful history can rule out the convexity of a spoon. If adjoining sur- previous injury to the area. Although intrin- faces are flush, assume that the contracture sic joint disease, such as RA, can cause finger is at least 30 degrees (FIGURE 3). The severity contractures, the joints are usually enlarged, of DD can also be graded according to the painful, and associated with characteristic Tubiana classification system (TABLE17), radiologic findings. wherein the total deformity or contracture Unlike DD, diabetic cheiroarthropathy score is the sum of the angles of all 3 digital often involves all of the digits except for the joints of the finger.