Systemic Sclerosis
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In the limited form of systemic sclerosis, the cutaneous manifestations predominate and there is absence of involvement of internal viscera. In the initial stages, the skin lesions are usually limited to hands but may involve the face, forearms, feet, and legs.
The manifestations begin with nonpitting, bilateral, and symmetrical edema. The fingers may have sausage-like appearance. The skin becomes atrophic and tight with a shiny, smooth and waxy appearance as it gets stretched over the subcutaneous tissues. There is loss of the usual skin folds.
In the later stages, ischemia leads to loss of finger pulp and absorption of terminal phalanges. The fingers appear tapered and the fingertips do not protrude beyond the free margin of the nails. Flexion contractures may develop that severely limit the mobility and function of the fingers as well as hands and feet. Raynaud's phenomenon, peripheral gangrene, and auto-amputation are additional vascular complications seen in systemic sclerosis.
The limited peripheral cutaneous sclerosis is associated with an anti-centromere antibody. In the diffuse form, anti-Scl-70 (anti-DNA topoisomerase) and RNA polymerase III antibodies are often present.
The manifestations begin with nonpitting, bilateral, and symmetrical edema. The fingers may have sausage-like appearance. The skin becomes atrophic and tight with a shiny, smooth and waxy appearance as it gets stretched over the subcutaneous tissues. There is loss of the usual skin folds.
In the later stages, ischemia leads to loss of finger pulp and absorption of terminal phalanges. The fingers appear tapered and the fingertips do not protrude beyond the free margin of the nails. Flexion contractures may develop that severely limit the mobility and function of the fingers as well as hands and feet. Raynaud's phenomenon, peripheral gangrene, and auto-amputation are additional vascular complications seen in systemic sclerosis.
The limited peripheral cutaneous sclerosis is associated with an anti-centromere antibody. In the diffuse form, anti-Scl-70 (anti-DNA topoisomerase) and RNA polymerase III antibodies are often present.