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A hand flexion contracture with progression

Prevalence ranges from 0.6% to 31.6% in general populations in Western countries and increases with age. 2 The disease has a strong genetic component, which had been attributed to an autosomal dominant gene of variable penetrance, although it is now thought to be a complex disorder involving many ge...

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Bibliographic Details
Published in:BMJ (Online) 2014-10, Vol.349 (oct30 7), p.g6137-g6137
Main Authors: McInerney, Niall M, O’Shaughnessy, Michael, Clover, James
Format: Article
Language:English
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Summary:Prevalence ranges from 0.6% to 31.6% in general populations in Western countries and increases with age. 2 The disease has a strong genetic component, which had been attributed to an autosomal dominant gene of variable penetrance, although it is now thought to be a complex disorder involving many genes. 3 A more aggressive form of the disease (Dupuytren's diathesis) is characterised by a positive family history, disease onset under 40 years of age, the presence of ectopic areas of fibromatosis, and bilateral disease that particularly affects the radial digits. 4 Ectopic fibromatoses may be seen on the knuckle pads of the hand (Garrod's pads), the plantar surface of the foot (Ledderhose disease), and the penis (Peyronie's disease). Patient outcome Our patient had complete correction of the contractures affecting the metacarpophalangeal and proximal interphalangeal joints (fig 3 ). Because the overlying skin was affected a dermofasciectomy was performed.
ISSN:0959-8138
1756-1833
1756-1833
DOI:10.1136/bmj.g6137