Scleroderma or Progressive Systemic Sclerosis


- See: vascular malformations of the upper extremity

- Clinical Presentation:
    - usually begins incidiously
    - vague weakness, wt loss, diffuse stiffness, aching, burning polyarticular arthritis;
    - diffuse edema of hands;
    - skin and GI manifestations are common;
    - hand:
          - skin is taut, thickened, or edematous & is bound tightly to subQ tissues;
          - normal skin folds at the knuckles disappear;
          - chronic recurrent painful ulcerations appear at ends of digits;
          - Raynaud's phenomenon is common;
          - hyperhidrosis, finger stiffness of fingers;
          - ulcerations fingertip amputation;
          - ref: Images in Clinical Medicine. Cold Hands Associated with Scleroderma
- Radiographs:
    - diffuse subQ calcifications (calcinosis circumscripta) are seen;
    - see:  Images in Clinical Medicine: Calcinosis Cutis in Systemic Sclerosis

- Labs: sed rate is elevated;


- Treatment:
    - sympathectomy:
    - prednisone, intravenous immune globulin, and penicillamine


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Images in Clinical Medicine. Cold Hands Associated with Scleroderma




Original Text by Clifford R. Wheeless, III, MD.

Last updated by Data Trace Staff on Thursday, December 1, 2016 10:12 am