Dupuytren contracture results from contracture of the palmar fascia within the hand, possibly resulting in a fixed flexion deformity of the metacarpophalangeal (MCP) joints and the proximal interphalangeal (PIP) joints. This condition usually affects the fourth and fifth digits (the ring and small fingers). (See the images below.)
Arrow denotes the cord often present in Dupuytren contracture. Metacarpophalangeal joint and proximal interphalangeal joint contractures are also present. This photo shows a patient with an inability to extend the fourth and fifth digits. The differential diagnosis includes Dupuytren contracture, which is a flexion contracture most commonly involving digits 4 and/or 5. The condition is a fibrosing disorder that results in slowly progressive thickening and shortening of the palmar fascia, leading to the debilitating digital contractures. Dupuytren contracture belongs to the group of fibromatoses that include plantar fibromatosis (Ledderhose disease), penile fibromatosis (Peyronie disease), and fibromatosis of the dorsal proximal interphalangeal (PIP) joints (Garrod nodes or knuckle pads).[1] Although many cases appear to be idiopathic and without coexisting conditions, a variety of associated diseases have been reported. (See Etiology.)
Dupuytren contracture is most commonly observed in persons of Northern European descent and affects 4-6% of whites worldwide.[2] Many individuals have bilateral disease (45%); in unilateral cases, the right side is more often affected.[3] The ring finger is most commonly involved, followed by the fifth digit and then the middle finger. The index finger and the thumb are typically spared. (See Epidemiology and Presentation.)
Although the cause of Dupuytren disease is unknown, a family history is often present. Males are 3 times as likely to develop disease and are more likely to have higher disease severity.[4, 5] Male predominance may be related to expression of androgen receptors in Dupuytren fascia. (See Etiology and Epidemiology.)[6]
Additional risk factors include manual labor with vibration exposure, prior hand trauma, alcoholism, smoking, diabetes mellitus, hyperlipidemia, Peyronie disease, and complex regional pain syndrome.[7] Rheumatoid arthritis seems to protect against the development of Dupuytren disease. (See Etiology.)
Therapies include conservative medical and surgical modalities. Although the condition is not fatal, significant morbidity can occur if patients remain untreated. (See Prognosis, Treatment, and Medication.)
Arrow denotes the cord often present in Dupuytren contracture. Metacarpophalangeal joint and proximal interphalangeal joint contractures are also present. This photo shows a patient with an inability to extend the fourth and fifth digits. The differential diagnosis includes Dupuytren contracture, which is a flexion contracture most commonly involving digits 4 and/or 5. The condition is a fibrosing disorder that results in slowly progressive thickening and shortening of the palmar fascia, leading to the debilitating digital contractures. Dupuytren contracture belongs to the group of fibromatoses that include plantar fibromatosis (Ledderhose disease), penile fibromatosis (Peyronie disease), and fibromatosis of the dorsal proximal interphalangeal (PIP) joints (Garrod nodes or knuckle pads).[1] Although many cases appear to be idiopathic and without coexisting conditions, a variety of associated diseases have been reported. (See Etiology.)
Dupuytren contracture is most commonly observed in persons of Northern European descent and affects 4-6% of whites worldwide.[2] Many individuals have bilateral disease (45%); in unilateral cases, the right side is more often affected.[3] The ring finger is most commonly involved, followed by the fifth digit and then the middle finger. The index finger and the thumb are typically spared. (See Epidemiology and Presentation.)
Although the cause of Dupuytren disease is unknown, a family history is often present. Males are 3 times as likely to develop disease and are more likely to have higher disease severity.[4, 5] Male predominance may be related to expression of androgen receptors in Dupuytren fascia. (See Etiology and Epidemiology.)[6]
Additional risk factors include manual labor with vibration exposure, prior hand trauma, alcoholism, smoking, diabetes mellitus, hyperlipidemia, Peyronie disease, and complex regional pain syndrome.[7] Rheumatoid arthritis seems to protect against the development of Dupuytren disease. (See Etiology.)
Therapies include conservative medical and surgical modalities. Although the condition is not fatal, significant morbidity can occur if patients remain untreated. (See Prognosis, Treatment, and Medication.)
Stages of Dupuytren disease
Dupuytren disease occurs in the following 3 stages:- Proliferative phase - Local fascial fibroplasia and development of a nodule, in which myofibroblasts proliferate, occur (see the image below), with palmar skin blanching on finger extension; in early disease, some patients may report tenderness and discomfort associated with the nodules
- Involutional phase - Contracture develops, with associated nodular thickening of the palmar fascia; myofibroblasts are predominant during this phase and align themselves along tension lines within the nodule
- Residual phase - The nodular tissue disappears, leaving acellular tissue and thick bands of collagen; the ratio of type III collagen to type I collagen increases, which is the reverse of the normal pattern in the palmar fascia[8]
Grades of severity
The grading system for Dupuytren disease severity is as follows (see the images below)[9] :- Grade 1 - Thickened nodule and band in the palmar aponeurosis; may have associated skin abnormalities
- Grade 2 - Development of pretendinous and digital cords with limitation of finger extension
- Grade 3 - Presence of flexion contractureArrow denotes the cord often present in Dupuytren contracture. Metacarpophalangeal joint and proximal interphalangeal joint contractures are also present. Arrow denotes the typical cords of Dupuytren contracture. These cords are usually painless. Note the metacarpophalangeal joint contracture. This photo demonstrates the presence of a nodule as well as skin blanching with extension of the affected digits. Three clinical grades of Dupuytren disease.
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