Scleroderma may be associated with severe joint contracture or cold intolerance, digital ulceration/ischemia and Raynaud's phenomenon. Diffuse and limited cutaneous variants, and CREST sydrome--are different subtypes. Typically Systemic sclerosis with diffuse cutaneous involvement is associated with joint stiffness while the limited cutaneous involvment/CREST are associated with vascular problems.
When I began my career at the University of Pittsburgh I was blessed to work with Dr Thomas Medsger--a rheumotologist and world authority on Sceroderma. I had the opportunity to treat patients from all over the country for digital ulceration and pain, as well as joint contracture.
When severe flexion contractures exist at the PIP joints, ulcerations often develop. If the position of the finger is not altered--in these cases by fusion in a more exteded position--amputation may ultimately be required to treat infection and soft tissue loss. .
This procedure has been very helpful for this problem--and truly reflects that "an ounce of prevention is worth a pound of cure."
