Definition
Dupuytrens Disease is a disease which affects some of the normal fibrous structures of the palm and fingers causing scar-like tissue to be deposited under the skin. Other areas may also be affected, albeit uncommonly.
Incidence
Dupuytrens disease is common in those of Celtic origin, but is very uncommon in Black and Asian patients. Males are affected 5 times more commonly than females.The disease usually starts in adult life, most commonly between the ages of 45-50, although young patients have been reported. Family history is positive in up to 60 % of cases.The disease is usually affects both hands and the right hand is affected more than the left. The thumb and index are seldom affected (7%), the little finger frequently affected (51 %), and the ring finger most commonly affected (61 %).
Etiology
The exact cause is uncertain, but the release of oxygen free radicals in the tissues of the palm is theorized. There are some associated conditions such as diabetes, epilepsy, liver failure, chronic pulmonary disease and recurrent microtrauma. Up to 15 % of patients have similar problems in other areas such as:
- Knuckle pads (or Garrod nodes) are firm, usually painless, fibrous plaques adhering the skin to the extensor tendon of the hand.
- Lederhose disease is the occurrence of fibrous, occasionally painful patches fixed only to the non-weight bearing sole of the foot.
- Peyronie Disease is a round plaque of the penis which produces pain and deformity on erection.
Clinical Findings
The characteristic finding is a nodule. This is a pea-sized firmness under the skin of the palm. The disease then progresses through 3 stages.
- Early: This stage involves thickening and multiple nodules under the skin.
- Active: This stage is marked by contraction, thickened cords as well as grooves and pits of the skin.
- Advanced: Nodules have disappeared, leaving joint contracture and firm tendon-like cords.
Treatment
There is no cure for Dupuytrens Disease. Surgery is the only option for joint contracture. There are 2 indications for surgery:
- When the tabletop test is positive (ie the hand cannot be laid flat on a hard surface). This is a universally accepted criterion for surgical correction.
- Finger contractures need to be addressed early since complete correction difficult and recurrence is likely.
Operations
Although there are many described techniques available, I prefer the use of open, extensive fasciectomy with z-plasty closure. Other procedures have high recurrence rates or unacceptable cosmetic outcomes. I perform a complete removal of all abnormal tissue. The incision line is then divided into several zig-zags (z-plasty) and closed quite loosely with small dissolving sutures.
Post Operative Care
The hand is elevated for the first 48 hours. A static splint is used continuously during the first 3 weeks. It is removed for dressing and therapy. Splintage may also improve on the results gained at surgery. 3 months of intermittent splintage is usually necessary.
Complications
Overall rate is 20%. Hematoma (Blood clot under the skin) is the most common. The skin may be very thin after dissection and some of the skin may die – in this situation a skin graft may be used. Infection and nerve injury are less common. Late complications include stiffness, disease recurrence and chronic pain.
Results
Correction of the palm is the most successful area, with 80-90% excellent results. Fingers are less rewarding, with only 50% excellent results. Recurrence is the biggest long term problem, but the lowest recurrence rates are associated with radical excisions and z-plasty.