Dupuytren Disease

Superficial fibromatoses are fibroblastic proliferations that are genetically distinct from the deep fibromatoses. They tend to arise in the palmar or plantar soft tissues. Like their deep counterparts (e.g., desmoid fibromatosis) they are characterized by infiltrative growth, a tendency to locally recur, and no metastatic potential.

Dupuytren disease, or palmar fibromatosis, is the most common superficial fibromatosis, affecting about 1% of the population and 20% of people over 65 years of age. The lesions more commonly affect the ulanr-sided digits and are bilateral in about 50% of patients. These painless subcutaneous nodules may progress to cords or bands that pull on the underlying flexor tendons, causing Dupuytren contractures.

On MRI, there are multiple nodular or band-like soft tissue masses that arise from the proximal palmar aponeurosis parallel to the flexor tendons and terminate in a branching or nodular pattern at the level of the distal metacarpal. The signal intensity of the nodules and cords is variable. Lesions with higher cellular content (and less abundant collagen) tend to have higher signal intensity on T1- and T2-weighted images and more prominent enhancement. These lesions also tend to have higher rates of local recurrence after resection. It has been suggested that these lesions be allowed to "mature" more (i.e., become less cellular and more collagenous) before surgical resection is attempted in order to reduce rates of recurrence.

Ultrasound shows hypoechoic and hypervascular nodules superficial to the flexor tendons.

There is an association with other fibromatoses: Up to 20% have plantar fibromatosis (Ledderhose disease), while Peyronie disease and knuckle pad involvement can also be seen.

Surgery is the treatment of choice, and is usually reserved for symptomatic patients or those with flexion contracture greater than 20 degrees at the metacarpophalangeal joint and more than 30 degrees at the proximal interphalangeal joint.

References

• Goldblum JR, Fletcher JA. Superficial fibromatoses. in Pathology and Genetics of Tumours of Soft Tissue and Bone. Fletcher CDM, Unni KK, Mertens F (eds). IARCPress Lyon, 2002. pp 82-82.
• Montgomery E, Lee JH, Abraham SC, Wu TT. Superficial fibromatoses are genetically distinct from deep fibromatoses. Mod Pathol. 2001 Jul;14(7):695-701.
• Murphey MD, Ruble CM, Tyszko SM, Zbojniewicz AM, Potter BK, Miettinen M. From the archives of the AFIP: musculoskeletal fibromatoses: radiologic-pathologic correlation. Radiographics. 2009 Nov;29(7):2143-73.