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Systemic sclerosis (scleroderma) 

Systemic sclerosis (scleroderma)
Chapter:
Systemic sclerosis (scleroderma)
Author(s):

Christopher P. Denton

, and Carol M. Black

DOI:
10.1093/med/9780198746690.003.0455
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date: 07 March 2021

The scleroderma spectrum of disorders includes several diseases that have Raynaud’s phenomenon or skin sclerosis in common, comprising (1) localized cutaneous scleroderma; (2) systemic sclerosis (SSc)—the most important form of scleroderma—limited cutaneous SSc, diffuse cutaneous SSc, and overlap syndromes (with features of another autoimmune rheumatic disease, e.g. systemic lupus erythematosus); (3) Raynaud’s phenomenon—autoimmune (with antinuclear or other SSc-associated antibodies) or primary. These conditions affect women four times as often as men, most often beginning in the fifth decade. The cause of SSc is not known: an attractive hypothesis is that the disease represents a syndrome of dysfunctional connective tissue repair with associated immunological, epithelial, and vascular pathology, triggered by some environmental factor(s) in a genetically and immunologically susceptible individual.

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