Dupuytrens disease

Dupuytrens disease is one of those diseases which present to our Plastic Surgery OPD frequently.Most of the patients come to us, when they are unable to work.So it is the functional limitation due to this disease that forces the person to consult expert help.Let us see what this disease is about.

What is dupuytrens disease?

Dupuytren’s disease is a progressive disease of the palmar and digital fascial structures characterized by nodular thickening and subsequent contracture. Deformity of the hand occurs primarily at the metacarpophalangeal (MCP) and proximal interphalangeal (PIP) joint level.This results in functional limitations to the patient.

Dupuytrens disease is closely related to Peyronie’s disease (penile fibromatosis) and Ledderhose’s disease (plantar fibromatosis).It is  most prevalent in populations with a strong northern European ancestry. Men are six times more likely to develop Dupuytren’s disease than are women.It usually begins in 5th decade in men and 6th decade in women.It has an autosomal dominant inheritance pattern.

Associations

Repetetive trauma,chronic obstructive pulmonary disease, human immunodeficiency virus, malignancy (paraneoplastic manifestation), and epilepsy have been associated with Dupuytrens contracture.

Palmar fascia and its role in Dupuytrens disease

Dupuytren’s disease is ultimately manifest as the transformation of the normal palmar and digital fascial structures to thickened diseased cords through the deposition of type I and type III collagen and the contractile forces generated by myofibroblasts.

The palmar fascia provides a flexible yet firm framework for the soft tissues of the palm, tethering the skin to the underlying musculoskeletal structures.

Layers of Palmar fascia

The palmar fascia consists of two distinct layers: the deep fascia and the superficial fascia or palmar aponeurosis. The deep fascia covers the interosseous muscles and is not involved in Dupuytren’s disease. The superficial fascia, in contrast, is affected by the pathologic progression of Dupuytren’s disease.

Pathogenesis of Dupuytrens disease

The pathogenesis of Dupuytren’s contracture in pathologic terms is divided into proliferative, involutional, and residual phases.

Proliferative phase

The proliferative phase is characterized by nodule formation within the palmar fascia and biochemically by increased fibrinolytic activity.

Involutional phase

Marked nodular thickening and signs of early joint contracture characterize the involutional phase. Throughout the involutional phase, type III collagen is synthesized and the myofibroblasts reorient along the lines of tension within the palm.

Residual phase

Type III collagen deposition continues and is gradually replaced with type I collagen throughout the residual phase.Myofibroblasts disappear leaving predominantly Type I and Type III collagen.

Signs in Dupuytrens disease

1.  Hugh Johnson sign- Widening of the skin crease in Dupuytrens disease

2. Short–Watson sign- A soft palpable fullness immediately adjacent to the cord at the level of the MCP joint may indicate displacement of the neurovascular bundle by a pathologic spiral cord

 

 Treatment

a)Non Operative

Extension splinting to prevent prevent contracture.

Ultrasound therapy has been reported to soften palmar nodules but has not been effective in the treatment of cords or contracture.

b)Injection treatment

Steroids have demonstrated a restrictive effect on the formation of fibrous tissue and scar.Steroids are effective in treating palmar nodule.

Enzymatic fasciotomy-Injection in enzymatic fasciotomy is performed with 0.58 mg clostridial collagenase diluted in 0.25 mL (MCP joint) or 0.20 mL (PIP joint) of sterile diluent. The cord is
located by manual palpation and injection is performed at three points along the length of the cord via a single puncture with a 25-gauge needle.

Injection is done at 1 month intervals, injection is followed by manual manipulation and night splinting.

c) Surgical Treatment of Dupuytrens contracture

Surgical treatment of Dupuytren’s disease consists of both fasciotomy and fasciectomy. Fasciotomy involves division of the diseased cords without excision and may be performed
via an open technique or percutaneously. Fasciectomy involves excision of the diseased cords.

Surgical treatment of dupuytrens in itself is a vast topic, and will be delt with in the next post.

Leave a Reply

 Master Of Medicine proudly uses PHP Speedy

%d bloggers like this: