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DUPUYTREN CONTRACTURE

Rebecca M. King, MD Karl T. Clebak, MD, FAAFP

Shawn F. Phillips, MD, MSPT

BASICS

DESCRIPTION

Palmar fibromatosis; caused by progressive fibrous proliferation and tightening of the fascia of the palms, resulting in flexion deformities and loss of function

Not the same as “trigger finger,” which is caused by thickening of the distal flexor tendon

Similar change rarely occurs in plantar fascia, usually appearing simultaneously.

System(s) affected: musculoskeletal

Dupuytren diathesis is an aggressive form that has ectopic involvement of knuckle pads, plantar fibromatosis (Ledderhose – 10%), and penile fibromatosis (Peyronie – 2%).

Synonyms: morbus Dupuytren; Dupuytren disease; “Celtic hand;” Viking’s disease; palmar fascial fibromatosis, contracture of palmar fascia

EPIDEMIOLOGY

Prevalence

Increases with age; mean prevalence in western countries: 12%, 21%, and 29% at ages 55, 65, and 75 years, respectively. Norway: 30% of males >60 years; Spain: 19% of males >60 years

More common in Caucasians of Scandinavian or Northern European ancestry

Mean age of onset is 60 years.

ETIOLOGYAND PATHOPHYSIOLOGY

Unknown; possibly oxidative stress, altered wound repair, and/or abnormal immune response. Occurs in three stages:

Proliferative phase: proliferation of myofibroblasts with nodule development on palmar surface

Involutional stage: spread along palmar fascia to fingers with cord

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development

Residual phase: spread into fingers with cord tightening and contracture formation

Genetics

Autosomal dominant with incomplete penetrance:

– Siblings with 3-fold risk

68% of male relatives of affected patients develop disease at some time.

Possible association with HLAalleles

RISK FACTORS

Smoking (mean 16 pack-years, odds ratio: 2.8)

Increasing age

Male/Caucasian; male > female (range 3.5:1 to 9:1)

Vibration exposure and manual work—risk doubles if regular (weekly) exposure

Diabetes mellitus (increases with duration of DM, usually mild; middle and ring finger involved)

Epilepsy

Chronic illness (e.g., pulmonary tuberculosis, liver disease, HIV)

Hypercholesterolemia

Excessive alcohol consumption

Northern European ethnicity

Family history

Hand trauma

Low body weight and BMI

GENERALPREVENTION

Avoid risk factors, especially if a strong family history.

COMMONLYASSOCIATED CONDITIONS

Alcoholism

Epilepsy (inconstant data)

DM

Chronic lung disease

Occupational hand trauma (vibration)

Hypercholesterolemia Carpal tunnel syndrome

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Peyronie disease

HIV

Cancer

Adhesive capsulitis of shoulder

DIAGNOSIS

HISTORY

Caucasian male aged 50 to 60 years

Family history

Mild pain early:

– Begins in palm and spreads to digits Unilateral or bilateral (50%)

Right hand more frequent

Ring finger or little finger most common, but any digit can be involved

Ulnar digits more affected than radial digits

Flexion contracture of metacarpophalangeal (MCP) before proximal interphalangeal (PIP) joint

PHYSICALEXAM

Painless plaques or nodules in palmar fascia

Cordlike band in the palmar fascia

Skin adheres to fascia and becomes puckered.

Palpable subcutaneous nodules

Reduced flexibility of MCPand PIPjoints

No sign of inflammation

Web space contractures

Knuckle pads over PIP

– Garrod nodes associated with severe disease Disease stages:

–Early: skin pits (can also be seen in nevoid basal cell cancer and palmar keratosis)

–Intermediate: nodules and cords. Nerves and vessels can be entwined in cords.

–Late: contractures

DIFFERENTIALDIAGNOSIS

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Camptodactyly: early teens; tight fascial bands on ulnar side of small finger

Diabetic cheiroarthropathy: all four fingers

Volkmann ischemic contracture

Trigger finger

Ganglion cyst

DIAGNOSTIC TESTS & INTERPRETATION

Diagnostic Procedures/Other

Diagnosis based on history and physical, testing is not routinely indicated. MRI can assess cellularity of lesions that correlate with recurrence after surgery.

Test Interpretation

Myofibroblasts predominate.

Nodules: Lumps fixed to skin hypercellular masses

Cords: organized collagen type III arranged parallel and hypocellular

First stage (proliferative): increased myofibroblasts

Second stage (residual): dense fibroblast network Third stage (involutional): Myofibroblasts disappear.

TREATMENT

GENERALMEASURES

Physical therapy alone is ineffective:

–Intermittent splinting is unlikely to be effective.

–Continuous splinting may help preand postop.

Follow isolated involvement of palmar fascia conservatively.

MCPjoint involvement can be followed conservatively if flexion contracture is <30 degrees.

MEDICATION

First Line

Clostridial collagenase injections (FDA-approved 2010):

–Degrades collagen to allow manual rupture of diseased cord

–Best for isolated cord of MCPjoint

–5-year recurrence rate of 47%; comparable with surgical recurrence rates (1)[B]

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–More rapid recovery of hand function compared to limited fasciectomy with fewer serious adverse events (2)[B]

–Complications: injection site reaction, skin tear

–Can do two cords concurrently

–Can be effective for postsurgical recurrence

Steroid injection:

–Can treat acute nodules or painful knuckle pads

–Serial triamcinolone injections improved long-term outcomes when combined with needle aponeurotomy (3)[B].

–Steroid alone associated with 50% recurrence in 1 to 3 years

Second Line

Surgery for contracture >30%

ISSUES FOR REFERRAL

Any involvement of PIPjoints

MCPjoints contracted >30 degrees

Impaired function

Progressively worsening contracture

Disabling deformity

ADDITIONALTHERAPIES

Percutaneous and needle fasciotomy:

–Best for MCPjoint; improvement of 93% versus 57% for PIPjoint

–Recurrence common; 50%

–Shown to be effective for recurrent disease

–Better for MCPjoints in patients with comorbid conditions; lower complication rate, but higher recurrence

–At 3 months and 1 year, outcomes of needle fasciotomy and collagenase injections are the same (4)[B].

SURGERY/OTHER PROCEDURES

Dermofasciectomy/limited fasciectomy/segmental aponeurectomy:

–Greater initial correction over nonincisional treatment; higher complication rates

–Percutaneous aponeurotomy and lipofilling (PALF) is a new, minimally invasive procedure that appears to have shorter convalescence, less longterm complications, similar operative contraction correction, and no

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significant difference at 1 year in results versus limited fasciectomy (5)[A].

Indications:

–Any involvement of the PIPjoints

–MCPjoints contracted at least 30 degrees

–Positive Hueston tabletop test (Patient is unable to lay palm flat on a table.)

May require skin grafts for wound closure with severe cutaneous shrinkage

80% have full range of movement with early surgery.

Amputation of 5th digit if severe and deforming

MCPjoints respond better to surgery than PIPjoints, especially if contracted >45 degrees.

ONGOING CARE

FOLLOW-UPRECOMMENDATIONS

Patient Monitoring

Regular follow-up every 6 months to 1 year

PATIENT EDUCATION

Avoid risk factors (alcohol, vibratory exposure, etc.), especially if strong family history.

Mild disease: Passively stretch digits twice a day and avoid recurrent gripping of tools.

PROGNOSIS

Unpredictable but usually slowly progressive

10% may regress spontaneously.

Dupuytren diathesis predicts aggressive course. Features include ethnicity (Nordic), family history, bilateral lesions outside of palm, age <50 years—all factors with 71% risk of recurrence compared to baseline 23% without any risk factors.

Prognosis better for MCPversus PIPjoint after surgery and collagenase injection

COMPLICATIONS

Complex regional pain syndrome Operative nerve injury

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Postoperative recurrence in 46–80%

Postoperative hand edema and skin necrosis

Digital infarction

Limited hand function

REFERENCES

1.Peimer CA, Blazar P, Coleman S, et al. Dupuytren contracture recurrence following treatment with collagenase Clostridium histolyticum (CORDLESS [Collagenase Option for Reduction of Dupuytren Long-Term Evaluation of Safety Study]): 5-year data. J Hand Surg Am. 2015;40(8):1597–1605.

2.Zhou C, Hovius SE, Slijper HP, et al. Collagenase Clostridium histolyticum versus limited fasciectomy for Dupuytren’s contracture: outcomes from a multicenter propensity score matched study. Plast Reconstr Surg.

2015;136(1):87–97.

3.McMillan C, Binhammer P. Steroid injection and needle aponeurotomy for Dupuytren disease: long-term follow-up of a randomized controlled trial. J Hand Surg Am. 2014;39(10):1942–1947.

4.Scherman P, Jenmalm P, Dahlin LB. One-year results of needle fasciotomy and collagenase injection in treatment of Dupuytren’s contracture: a twocentre prospective randomized clinical trial. J Hand Surg Eur Vol.

2016;41(6):577–582.

5.Kan HJ, Selles RW, van Nieuwenhoven CA, et al. Percutaneous aponeurotomy and lipofilling (PALF) versus limited fasciectomy in patients with primary Dupuytren’s contracture: a prospective, randomized, controlled trial. Plast Reconstr Surg. 2016;137(6):1800–1812.

ADDITIONALREADING

Ball C, Pratt AL, Nanchahal J. Optimal functional outcome measures for assessing treatment for Dupuytren’s disease: a systematic review and recommendations for future practice. BMC Musculoskelet Disord. 2013;14:131.

Collis J, Collocott S, Hing W, et al. The effect of night extension orthoses following surgical release of Dupuytren contracture: a single-center, randomized, controlled trial. J Hand Surg Am. 2013;38(7):1285.e2–1294.e2.

Eaton C. Evidence-based medicine: Dupuytren contracture. Plast Reconstr Surg. 2014;133(5):1241–1251.

Henry M. Dupuytren’s disease: current state of the art. Hand (N Y).

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2014;9(1):1–8.

Lanting R, Broekstra DC, Werker PM, et al. Asystematic review and metaanalysis on the prevalence of Dupuytren disease in the general population of Western countries. Plast Reconstr Surg. 2014;133(3):593–603.

Michou L, Lermusiaux JL, Teyssedou JP, et al. Genetics of Dupuytren’s disease. Joint Bone Spine. 2012;79(1):7–12.

Sweet S, Blackmore S. Surgical and therapy update on the management of Dupuytren’s disease. J Hand Ther. 2014;27(2):77–83.

van Rijssen AL, Werker PM. Percutaneous needle fasciotomy for recurrent Dupuytren disease. J Hand Surg Am. 2012;37(9):1820–1823.

CODES

ICD10

M72.0 Palmar fascial fibromatosis [Dupuytren]

CLINICALPEARLS

Dupuytren contracture is a fixed flexion deformity of (most commonly) the 4th and 5th digits due to palmar fibrosis. 90% of cases are progressive; not trigger finger, which is due to thickening of the distal flexor tendon

Refer patients with involvement of the PIPjoints or MCPinvolvement with contractures of >30 degrees.

Both surgical and enzymatic fasciotomy have high rate of recurrence.

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DYSHIDROSIS

Patricia Y. Chipi, MD George G.A. Pujalte, MD, FACSM

BASICS

DESCRIPTION

Acommon chronic dermatitis often involving the palms and soles. The precise definition is frequently debated, with many terms being used interchangeably. Efforts are being made to more specifically define dyshidrosis, and literature supports the presence of several different classes within the family dyshidrosis but no general consensus.

Dyshidrotic eczema

–Common, chronic, or recurrent; nonerythematous; symmetric vesicular eruption primarily of the palms, soles, and interdigital areas

–Associated with burning, itching, and pain

Pompholyx (from Greek “bubble”)

–Rare condition characterized by abrupt onset of large bullae

–Often used interchangeably with dyshidrotic eczema (small vesicles); however, may be a distinct entity

Lamellar dyshidrosis

–Fine, spreading exfoliation of the superficial epidermis in the same distribution as described above

System(s) affected: dermatologic, exocrine, immunologic

Synonym(s): cheiropompholyx, keratolysis exfoliativa, vesicular palmoplantar eczema, desquamation of interdigital spaces pompholyx, acute and recurrent vesicular hand dermatitis

EPIDEMIOLOGY

Incidence

Mean age of onset is 40 years and younger.

Male = female

Comprises 5–20% of hand eczema cases

Prevalence

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20 cases/100,000 populations

ETIOLOGYAND PATHOPHYSIOLOGY

Exact mechanism unknown; thought to be multifactorial

Dermatopathology: intraepidermal spongiosis without effect on eccrine sweat glands

Vesicles remain intact due to thickness of stratum corneum of palmar/plantar skin (1).

Exact cause not known; although the following have been implicated: allergies, genetics, and dermatophyte infection

Aggravating factors (debated)

–Hyperhidrosis (in 40% of patients with the condition)

–Detergents/solvents

–Increased water exposure

–Climate: hot/cold weather; humidity

–Contact sensitivity (in 30–67% of patients with the condition) (2)

–Nickel, cobalt, and chromate sensitivity (may include implanted orthopedic or orthodontic metals) (1)

–Stress

–Dermatophyte infection (present in 10% of patients with the condition) (2)

–Prolonged wear of occlusive gloves

–IV immunoglobulin therapy

–Smoking

–UVAradiation

Genetics

Atopy: 50% of patients with dyshidrotic eczema have atopic dermatitis (1).

Rare autosomal dominant form of pompholyx found in Chinese population maps to chromosome 18q22.1–18q22.3 (2)

RISK FACTORS

Many risk factors are disputed in the literature, with none being consistently associated.

Atopy

Other dermatologic conditions

–Atopic dermatitis (early in life)

–Contact dermatitis (later in life)

–Dermatophytosis

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