Washington Manual of Medical Therapeutics
Systemic Sclerosis
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General Principles
Definition
Systemic sclerosis (scleroderma) is a systemic illness characterized by progressive fibrosis of the skin and visceral organs. The etiology is unknown, but immune dysregulation, vasculopathy, and fibrosis are implicated in disease pathogenesis.
Classification
- Scleroderma can be subdivided based on anatomic skin distribution into localized scleroderma (morphea and linear scleroderma) and systemic sclerosis (diffuse cutaneous, limited cutaneous, and systemic sclerosis sine scleroderma). The limited cutaneous form involves the extremities distal to the knees and elbows as well as the face. Diffuse cutaneous scleroderma involves the skin of the proximal extremities and the trunk. Systemic sclerosis sine scleroderma affects the internal organs without skin involvement. The CREST syndrome is calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias.
- Overlap syndrome is the term used to describe patients who have overlapping clinical or serologic features of two or more CTDs. Arguably, the most well-defined of these overlap syndromes is MCTD which encompasses features of SLE, systemic sclerosis, and inflammatory myopathies. Therefore, it is important to recognize that the features of systemic sclerosis that will be reviewed here can be present along with features of other CTDs.
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General Principles
Definition
Systemic sclerosis (scleroderma) is a systemic illness characterized by progressive fibrosis of the skin and visceral organs. The etiology is unknown, but immune dysregulation, vasculopathy, and fibrosis are implicated in disease pathogenesis.
Classification
- Scleroderma can be subdivided based on anatomic skin distribution into localized scleroderma (morphea and linear scleroderma) and systemic sclerosis (diffuse cutaneous, limited cutaneous, and systemic sclerosis sine scleroderma). The limited cutaneous form involves the extremities distal to the knees and elbows as well as the face. Diffuse cutaneous scleroderma involves the skin of the proximal extremities and the trunk. Systemic sclerosis sine scleroderma affects the internal organs without skin involvement. The CREST syndrome is calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias.
- Overlap syndrome is the term used to describe patients who have overlapping clinical or serologic features of two or more CTDs. Arguably, the most well-defined of these overlap syndromes is MCTD which encompasses features of SLE, systemic sclerosis, and inflammatory myopathies. Therefore, it is important to recognize that the features of systemic sclerosis that will be reviewed here can be present along with features of other CTDs.
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