Scleroderma (systemic sclerosis) - Triad of autoimmunity, noninflammatory vasculopathy, and collagen deposition with fibrosis. Commonly sclerosis of skin, manifesting as puffy, taut skin A without wrinkles, fingertip pitting B . Also sclerosis of renal, pulmonary (most common cause of death), cardiovascular, GI systems. 75% female. 2 major types:
Diffuse sclerodermaโwidespread skin involvement, rapid progression, early visceral involvement. Associated with anti-Scl-70 antibody (anti-DNA topoisomerase I antibody).
Limited sclerodermaโlimited skin involvement confined to fingers and face. Also with CREST syndrome: Calcinosis C , anti-Centromere antibody, Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia. More benign clinical course
submitted by โscoresloth(3)
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5825946/