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The site is secure. NCBI Bookshelf. Systemic sclerosis or scleroderma is a rare connective tissue disorder with an unknown and complex pathogenesis. Scleroderma can be divided into 2 primary forms—localized scleroderma including morphea, linear scleroderma, and scleroderma en coup de sabre and systemic sclerosis. Systemic sclerosis can be further classified as limited systemic sclerosis formerly known as CREST syndrome, characterized by calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia or diffuse systemic sclerosis based on clinical and serological criteria.
Localized scleroderma primarily affects the skin and subcutaneous tissue, whereas systemic sclerosis is associated with systemic manifestations and internal organ involvement, leading to increased mortality.
The manifestations of scleroderma may overlap extensively with those of other rheumatological or immunological diseases. The severity of the presentation may also vary depending on the timing of the systemic sclerosis diagnosis.
Systemic sclerosis affects multiple organ systems, necessitating a collaborative healthcare team involving primary care clinicians, rheumatologists, gastroenterologists, cardiologists, pulmonologists, nephrologists, and dermatologists to address the condition. Effective management of systemic sclerosis requires early diagnosis and ongoing disease progression monitoring.
