palmar fibromatosis

Dupuytren's contracture, also called Viking disease, or palmar fibromatosis, is a fibrosing condition that characteristically presents as a firm nodularity on the palmar surface of the hand with coalescing cords of soft tissue on the webs and digits.

Epidemiology

It is considered the most common of the superficial fibromatoses and is thought to affect 1-2% of the population. People of northern European descent are typically affected with the highest prevalence in countries such as northern Scotland, Iceland, Norway and Australia . Age at presentation usually peaks around 65. There may be an increased male predilection.

Associations

Additional diseases that are associated with palmar fibromatosis include:

• diabetes mellitus
• epilepsy
• alcoholism (particularly liver disease related to alcoholism)
• keloids
• plantar fibromatosis
• Peyronie disease

Clinical presentation

Patients typically present with palmar surface subcutaneous nodules on the distal crease of the hand. This may progress to cords and bands and finally, the characteristic flexion contracture secondary to fibrous attachments to the underlying tendon sheath.

Pathology

It is a progressive condition that causes shortening and thickening of the fibrous tissue of the palmar fascia.

Location

• lesions can be bilateral in about half of cases
• the 4 ray is typically involved

Radiographic features

Ultrasound

Initial findings are of nodules seen superficial to the flexor tendons in the superficial fascia of the palm. Early on these appear hypoechoic to tendons with hypervascularity while chronic nodules can appear hyperechoic without associated vascularity . These can progress to hypoechoic cords and bands adhering to the flexor tendon margins, eventually resulting in the characteristic clinically apparent contracture deformity.

MRI

The lesions typically comprise of subcutaneous nodules, usually at the level of the distal palmar crease and cords that lie parallel and superficial to the flexor tendons.

Signal characteristics of the cords include:

Typical (~80% of cases)

• T1: uniformly low signal intensity (similar to the signal intensity of tendon)
• T2: uniformly low signal intensity (similar to the signal intensity of tendon)

Atypical

• T1: low to intermediate signal intensity (a slightly higher signal intensity than that of the tendon)
• T2: low signal intensity

Treatment and prognosis

Medical or surgical intervention may be required in patients suffering from a functional impairment (significant inability to use their hands for routine activities) or due to cosmetic embarrassment. Medical intervention includes triamcinolone, lidocaine or collagenase injection. Vitamin E, gamma interferon, dimethyl sulfoxide and splinting are not considered adequate treatments for Dupuytren contracture. Surgical release of palmar fascia is usually indicated in patients with severe functional impairment and neurovascular deficit in a finger .

History and etymology

It was originally described in 1831 by the French physician Dupuytren .

Differential diagnosis

As a general differential for contracted fingers, consider other forms of contractures such as

• congenital contractures
  • neurological pathology
  • arthrogryposis: tends to be quite severe and involve the hand as well
• acquired contractures
  • Volkmann contracture

See also

• nodular fasciitis

Siehe auch:

• Kamptodaktylie
• musculoskeletal fibromatoses
• Morbus Ledderhose
• Induratio penis plastica
• Fibromatose
• pseudotumoröse Weichteilläsionen von Hand und Handgelenk

und weiter:

• Fasciitis nodularis
• fibrous lesions