Dupuytren Disease

For 3 years, a linear plaque had been slowly developing on the left palm of a 47-year-old woman who had difficulty in opening and closing her hand. There was no contributory family history. Dr Charles E. Crutchfield III of Eagan, Minn, diagnosed Dupuytren disease, a hand disorder of unknown origin. Also called palmar fasciitis or palmar fibromatosis, the condition is characterized by progressive fibrosis of the palmar aponeurosis and its extensions into the digits. The inherited disorder most commonly occurs in persons of northern European ancestry, particularly Celtic lineage. It may be associated with epilepsy, alcoholism, type 1 diabetes mellitus, or trauma. This patient’s disease affected her left hand; however, manifestations can include bilateral involvement of the upper extremities and knuckle pads. In advanced fibrosis, overlying skin and underlying flexor tendons are affected and cause contracture and deformity. Most often, contracture is noted in the ring finger; the thumb is seldom involved. Initially, reassurance and observation are appropriate management strategies if the patient has no contractures and no skin, hygiene, or pain problems associated with fibrosis. When the patient is unable to simultaneously place the entire palm and palmar surface of each finger flat on a surface, fasciectomy-the mainstay of treatment-is warranted. The timing of surgical intervention is crucial. Premature fasciectomy may be associated with early disease recurrence and progression of fibrosis; whereas complications, such as skin deficiency that requires grafts; joint stiffness; prolonged edema; pain; and wound dehiscence, can result when surgery is delayed. Hematoma formation after surgery or other trauma may result in the loss of palmar skin. This can lead to a recurrence of the fibrosis and/or the deformity and irreparable contracture that may require amputation.

References:

FOR MORE INFORMATION:

• Ackerman AB, Kerl H, Sanchez J. A Clinical Atlas of 101 Common Skin Diseases:With Histopathologic Correlation. New York: Ardor Scribendi; 2000.
• Dover JS, Jackson BA. Pocket Guide to Cutaneous Medicine and Surgery. Philadelphia:WB Saunders Company; 1996.