Symptoms Limited Scleroderma

Many patients with limited scleroderma have the five classical symptoms that comprise the CREST syndrome: calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telang-iectasia. The term CREST syndrome is derived from the first letter of each of these symptoms, but it is also often used to describe patients with limited scleroderma who have only three or four of the symptoms of CREST. Some physicians use the terms CREST syndrome and limited scleroderma interchangeably.

Skin Virtually all patients with limited sclero-derma have Raynaud's phenomenon, and this is often present for many years before skin changes develop. Severe Raynaud's leading to painful, poorly healing ulcers on the tips of the fingers, called digital ulcers, is more common in patients with limited scleroderma than in those with diffuse scleroderma. Digital ulcers are very painful and heal over several months. The skin changes of limited scleroderma often affect only the hands and face but can be severe, leading to tight, shiny, poorly mobile fingers, or very subtle with just a little skin thickening. Tightness of the skin on the fingers is called sclerodactyly. Many patients also have telangiectasia. These are small red spots, about the size of a freckle, that consist of a network of blood vessels. Calcinosis (deposits of calcium in the skin and soft tissues) is less common.

Respiratory system In addition to the mildness of the skin and internal organ involvement, a major feature that characterizes limited sclero-derma and differentiates it from diffuse disease is pulmonary hypertension. Patients with limited scleroderma have a predisposition to develop pulmonary hypertension that is virtually identical to primary pulmonary hypertension. Patients with diffuse scleroderma can also develop pulmonary hypertension, but this is usually milder and due to fibrosis of the lungs. in patients with limited scle-roderma and pulmonary hypertension, the lungs themselves are fairly normal and the problem is in the small blood vessels of the lungs. The walls of these small blood vessels become thick and fibrotic, and the channel through which blood can flow is narrowed. Pulmonary hypertension develops in about 5 percent of patients and causes symptoms of fatigue, shortness of breath, fainting, and heart failure.

Gastrointestinal system Limited scleroderma usually affects only the esophagus and causes the same symptoms as diffuse scleroderma.

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