9- Scleroderma

Question 1

What is the general info on Scleroderma?
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1071 txt

Answer 1

• Systemic inflammatory connective tissue disease of unknown skin involving skin, lungs, gastrointestinal tract, heart and kidneys, and musculoskeletal system.
• Radiographic signs appear well after the disease is well established
• skeletal involvement is primarily appendicular

Question 2

What are the clinical features of scleroderma

Answer 2

• onset 30-50 yrs, F:M, 3:1
• skin changes: edema, induration (becoming hardened), atrophy
• skin becomes thick, taut, and hidebound, involving hands and face. Involvement of oesophagus with dysphagia is common

Question 3

What re the radiographic features seen in the hand/wrist?

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Answer 3

• earliest sign is swelling in the fingertips
• resorption(remodelling) of the soft tissue
• tapered fingers with retraction (pg 1073 pic)
• 20% see soft tissue calcification, 75% of these in the hands
• tuft resorption in 80%
• osteoporosis from disuse