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CRST syndrome and “neuropathic” arthropathy

✍ Scribed by Dr. Irving Karten


Publisher
John Wiley and Sons
Year
1969
Tongue
English
Weight
255 KB
Volume
12
Category
Article
ISSN
0004-3591

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✦ Synopsis


A 48-year-old woman had features of the CRST variant of systemic sclerosis as well as a severe "neuropathic" arthropathy. No underlying neurologic disorder could be found with certainty. She de-N 1964, Winterbauerl coined the term I CRST to describe those cases of systemic sclerosis that exhibited calcinosis, Raynaudk phenomenon, sclerodactyly, and telangiectasia. This case report describes a patient who, in addition to this syndrome, had a severe mutilating "neuropathic" arthropathy, which to our knowledge has not been previously associated with CRST syndrome or systemic sclerosis.

CASE HISTORY

A 48-year-old unmarried woman was transferred to Goldwater Memorial Hospital on October 3, 1968, because of scleroderma and inability to walk or to care for herself. She first developed Raynaud's phenomenon at age 18 and telangiectasia at age 32. Two years later, polyarthritis began insidiously, but she was able to perform clerical work until 1967. Because of the absence of joint pain and despite the development of obvious joint deformities, she had never consulted a physician nor did she take analgesics or corticosteroids.

The patient was a well-developed, poorly nourished woman with impaired hearing. There was intense telangiectasia of the lips and skin of the face, limbs, and trunk (Fig. 1 ). Some tightening and hyperpigmentation were present in the skin of the extremities. Rhinitis sicca and a large septal


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