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A progressive genetic process of pathologic collagen production and deposition.
A benign fibromatosis of the hands and fingers, associated with the formation of nodules and cords and often leading to flexion contractures.
DC is a manifestation of a fibroproliferative disease in which fibrous cords form in the hand.
Prevalence in Western countries ranges from 0.6 to 31.6% with a higher prevalence among men, individuals older than 50 years, persons of northern European descent.
Smoking and occupations that involve manual labor, or forceful stretching of the fascia ARE associated with increased risk.
These cords shorten, and pull one or more fingers into a bent position and increasingly interfere with hand function.
No cure for DC exists and contractions may recur and need additional treatment anfter intervention.
Cause remains unknown.
Type of hand deformity that affects tissue under the skin of the palm connected to the tissues in the fingers.
Most common in people of northern European descent.
Type of hand deformity that affects tissue under the skin of the palm connected to the tissues in the fingers.
Onset is insidious with nodules and flexor tendon thickening along the,ulnar side of the hand.
Begins with palpable nodules in the palm that later form collagen cords that extend longitudinally become thickened and shortened and cause flexion contractures of the hand joints, limiting function (Murrell).
Pathogenesis is not fully understood.
Contractures typically affect the metcarpophalangeal joint, the proximal interphalangeal joint, or both.
The ring and little fingers are most commonly involved.
Incidence highest in individuals of northern European descent, but occurs in all racial and ethnic groups.
Prevalence between 0.2% and 56% (Hondocha S et al).
More common in men.
Patients with long-standing diabetes may develop thickening of the digits known as diabetic cheiroarthropathy and palmar flexion contraction.
By the ninth decade of life, the incidence in women is the same as men.
Estimated global prevalence is 3-6% among whites people.
Incidence increases with age.
Associated with diabetes, smoking, alcoholism, epilepsy and HIV infection.
Clustering of cases those occur in families, suggesting a genetic influence with environmental risk factors contribute to susceptibility of the disease.
In a genome wide study of 960 Dutch persons with DC nine different loci of genetic susceptibility was implicated, and six of the nine harbor genes encoding proteins in the Wnt-signaling pathway suggesting its relationship to the process of fibromatosis (Dolmans GH et al).
Surgery with an open fasciectomy is standard treatment.
Standard treatment consists of surgical excision or transection of nodules and cords.
Fasciectomy may be open, percutaneous or needle faciotomy.
Surgery is recommended when metcarpophalangeal joint contractures are 30 degrees or more.
For proximal interphalangeal joint contractures recommendations for surgery vary.
No effective drug management exists.
No cure exists, and recurrence rates range from 8-66% depending upon the treatment.
Collagenase clostridium histolyticum can lyse collagen and can disrupt contracted cords.
Collagenase clostridium histolyticum can be injected into the contraction cord, with subsequent manipulation of the cord to attempt its rupture.
Collagenasevenzyme weakens the cord by breaking down collagen.
Studies of collagenase clostridium histolyticum reduced contractures of the metcarpophalangeal and proximal interphalangeal joints to 0-5 degrees of full extension in nearly two-thirds of treated joints (Badalamente).
In a randomized study of 308 patients with joint contractures of 20 degrees or more in a double blind, placebo controlled trial patients received up to three collagenase clostridium histolyticum injections or placebo in the contracted collagen cord at 30 day intervals, followed by joint manipulation one day after injection: the range of motion of the joints was significantly improved after the injection with collagenase as compared to placebo (Collagenase Option for Reduction of Dupuytren’s (CORD) I study).
Collagenase injections associated with swelling, pain, bruising, pruritus, and transient lymph node enlargement and two tendon ruptures and one case of complex regional pain syndrome was present (Hurst).
Collagenase (Xiaflex), is injected into the hands where Dupuytren’s has formed and works to weaken the Dupuyten’s tissue.
Collagenase injection is not inferior to limited fasciectomy with respect to score on the patient evaluation measure-hand health profile at one year after treatment.