Abstract

A 58-year-old woman was referred by her general practitioner, in December, 1999, with a 3-month history of rapidly progressing symmetrical contraction of the fingers of both hands, which began suddenly with pain and difficulty in holding objects. A trial of non-steroidal anti-inflammatory drugs and oral corticosteroids was ineffective. She was a non-smoker who had taken hormone-replacement treatment since her menopause. She had a long history of mild Raynaud's syndrome, a family history of colon cancer, and reported a recent weight loss of several kilograms. On examination she had tight, disabling flexion contracture of her fingers with marked tethering of the flexor tendons with a Dupuytren's-like process (figure). The overlying skin was normal, with no evidence of telangiectasia, digital pitting, or of systemic sclerosis elsewhere. She was unable to flex or extend her fingers. Otherwise, her joint, cardiovascular, respiratory, and abdominal examinations were unremarkable. A pelvic examination was not done. A skin biopsy specimen from the palmar aspect of the base of her thumb showed a normal epidermis with spindle-cell proliferation in the deep dermis, consistent with fibromatosis. Blood glucose, anti-nuclear antibodies, extractable nuclear antibodies (Scl-70, RNP, Sm, Ro, La), rheumatoid factor, erythrocyte sedimentation rate, Borrelia serology, and plain radiographs and magnetic resonance images of both hands were negative or normal.

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