Scleroderma is a serious autoimmune disorder in which the body produces too much collagen. This excess collagen builds up in the skin and internal organs, causing harm.
Limited scleroderma is a subtype of scleroderma in which skin thickening is less widespread; typically it occurs in the fingers, hands, lower arms and legs and, sometimes, the face. Limited scleroderma is also sometimes referred to as CREST syndrome. The acronym stands for:
- C – calcinosis (calcium deposits under the skin and in tissues)
- R – Raynaud’s phenomenon (usually associated with cold intolerance and stress)
- E – esophageal dysfunction (which causes heartburn)
- S – sclerodactyly (thick skin on the fingers)
- T – telangiectasias (enlarged blood vessels that appear as red spots on the skin)
Limited scleroderma can be milder than systemic scleroderma and can develop at a slower pace. But it can also be serious, and affect internal organs such as the heart or lungs, as well as the digestive tract. Difficulties in swallowing are common in people with limited scleroderma, as it often affects the muscles of the esophagus.
Like all forms of scleroderma, limited scleroderma is marked by hardened and thickened skin and can, as noted, show symptoms of Raynaud’s phenomenon, brief episodes triggered by cold or stress that cause small arteries of the hands and feet to narrow (vasospasm) and cut the flow of blood.
Limited scleroderma affects about half of all people with scleroderma. It is more common among Caucasians, and up to four times more common among women than men. Genetic factors or exposure to toxins may be risk factors; so is a family history of autoimmune diseases like lupus or rheumatoid arthritis.
Although no cure for any type of scleroderma exists, treatment options are available to help manage symptoms and prevent complications.
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