Systemic Sclerosis and Scleroderma Variants: Clinical Aspects
Posted Nov 18 2010 1:42am
The term scleroderma traditionally has been applied to the cutaneous changes of both systemic sclerosis and a heterogeneous group of conditions designated collectively as scleroderma variants. Systemic sclerosis is a chronic disorder of connective tissue characterized by inflammation and fibrosis and by degenerative changes in the blood vessels, skin, synovium, skeletal muscle, and certain internal organs, notably the gastrointestinal tract, lung, heart, and kidney .
Although there is an early, often clinically unappreciated, inflammatory component, the hallmark of the disease is thickening of the skin (scleroderma) and other organs caused by excessive accumulation of connective tissue. Subintimal proliferative vascular changes are prominent and lead to Raynaud’s phenomenon and obliterative arteriolar and capillary lesions.
In the scleroderma variant, dermal fibrosis is more circumscribed, both vascular and internal organ involvement are generally absent, and the characteristic serologic abnormalities of systemic sclerosis are not found.
Coexistence of these entities is rare. Although “evolution” of localized scleroderma to systemic sclerosis has been suggested by some case reports, it is more likely that these are instances in which a single patient suffers from two sequential autoimmune diseases. Many other conditions with systemic features exhibit scleroderma-like, but often distinctive, skin changes and lack the typical visceral manifestations and characteristic serologic abnormalities of systemic sclerosis, as discussed later in this chapter.