Progressive fibroproliferative disease of the palmar fascia causing flexion contractures of the digits.
Also known as: Dupuytren disease, Dupuytren contracture, palmar fibromatosis, Viking disease
Overview
A benign fibroproliferative disorder of the palmar fascia characterized by myofibroblast proliferation and abnormal collagen deposition, producing palmar nodules, longitudinal cords, and progressive metacarpophalangeal and proximal interphalangeal joint flexion contractures.
Epidemiology
Most common in Northern European descent (the 'Viking disease'). Prevalence rises with age, peaking in the sixth to seventh decade. Male-to-female ratio 5-7:1, with women presenting later and with milder disease. The ring and small fingers are most commonly affected.
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Family history (autosomal dominant with variable penetrance)
Diabetes mellitus
Alcohol use and chronic liver disease
Tobacco use
Anti-epileptic medications (historically associated with phenytoin and phenobarbital)
HIV infection
Pathophysiology
Myofibroblasts within the palmar aponeurosis proliferate and contract, depositing type III collagen and producing the characteristic nodules and cords. The disease progresses through three histologic stages: proliferative (myofibroblast-rich, hypercellular), involutional (aligned myofibroblasts producing contraction), and residual (acellular, collagen-rich cords). The pretendinous, spiral, lateral digital, and Grayson ligaments are the typical anatomic contributors to digital contracture.
Clinical presentation
Symptoms
Painless palmar nodule, typically in the line of the ring finger at the distal palmar crease
Progressive inability to fully extend the affected digit
Functional limitations: difficulty placing the hand flat, putting hand in pocket, washing the face, shaking hands
Discomfort is uncommon; pain should prompt consideration of alternative diagnoses
Signs / physical exam
Firm palmar nodule and longitudinal cord, often in line with the ring or small finger
Active and passive flexion contracture of MCP and/or PIP joints
Positive Hueston tabletop test — inability to flatten the hand on a tabletop
Skin pitting and tethering of overlying skin
Classic findings
Painless palmar nodule with cord extending into the ring or small finger producing an MCP flexion contracture in an older man of Northern European descent.
Differential diagnosis
Stenosing tenosynovitis (trigger finger) — Catching with motion, palpable nodule at A1 pulley, no longitudinal cord or fixed contracture without triggering
Flexor tendon adhesions — Following injury or surgery; both active and passive ROM limited
Camptodactyly — Congenital flexion deformity of the PIP joint of the small finger, present from childhood
Palmar fascia callus — Hyperkeratotic skin without subcutaneous nodule or cord
Diagnostic workup
Diagnostic criteria
Hueston tabletop test — the patient is asked to place the palm flat on a table. Inability to do so indicates contracture sufficient to consider intervention. Treatment is typically offered for MCP contracture of 30 degrees or more or for any PIP contracture.
Labs
None required; consider HbA1c if not previously screened
Imaging
Clinical diagnosis; imaging not routinely required
Diagnostic algorithm
Modality
Setting
Recurrence
Recovery
Needle aponeurotomy
Office
Higher (50-65% at 5 yr)
Days
Collagenase injection
Office, 2 visits
Intermediate (35-45% at 5 yr)
1-2 weeks
Limited fasciectomy
Operating room
Lower (~20% at 5 yr)
6-12 weeks
Dermofasciectomy
Operating room
Lowest
Months
Comparison of treatment modalities for Dupuytren contracture by setting, recurrence rate, and recovery.
Treatment
First-line
Observation for nodules without functional contracture
Avoidance of repetitive trauma; no proven benefit from stretching or splinting
Patient education about progressive natural history
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