Scleroderma literally means hard skin. It is a multi-systemic disorder not simply limited to the skin. It may also involve the heart, lungs and kidneys. It is often associated with Raynaud's Phenomenon, which is a color change in the extremities often secondary to exposure to cold, trauma or stress. Diffuse cutaneous forms of disease are referred to as systemic sclerosis. Limited cutaneous forms, overlapping syndromes, and localized forms are referred to as linear sclerosis or morphea. The limited cutaneous forms are also referred to as CREST syndrome. They have a strong association with certain antibodies, such as the anticentromere antibody and overall have a better prognosis.
What is CREST syndrome?
CREST syndrome is a limited form of scleroderma and literally CREST stands for Calcinosis, Raynaud's phenomenon, Esophageal dysmotility (often given rise to heartburn symptoms and lower esophagitis), Sclerodactyly which refers to the sclerodermatous changes of the fingers, and Telangiectasias which are dilated venules, capillaries and arterioles involving the hands, face, lips or mouth. It is often associated with an anti-centromere antibody and has a relatively good prognosis, but needs to be followed carefully for the development of pulmonary hypertension or pulmonary fibrosis. Patients with CREST syndrome can also have progressive Raynaud's Phenomenon even leading to auto-amputation of the digits. Another feature that is sometimes associated with CREST syndrome is the presence of biliary cirrhosis.
What is Raynaud's phenomenon?
Raynaud's phenomenon refers to the vasospasm that can occur in an episodic and self-limited as well as reversible manner. It affects digits, primarily toes and fingers. Occasionally it can affect the ears, nose and lips. The classic changes in color are from white to blue and then red, but not all patients experience this triad. Raynaud's may affect between 4% to 15% of the general population and is often mild and not associated with any other structural problem or related to any underlying medical disorder. Secondary Raynaud's phenomenon occurs as a manifestation of an underlying disease such as scleroderma, mixed connective tissue disorder, systemic lupus erythematosus, certain malignancies and occasionally acromegaly. It is often treated with short courses of calcium channel blockers such as nifedipine, but in severe cases other vasodilators are used. Proper local care for digital ulcers may occasionally include short courses of antibiotics to treat infections such as staphylococcal bacterial infections.
What causes scleroderma?
No one knows the exact cause of scleroderma, although at the present time there is growing evidence to suggest a genetic predisposition for this disorder. It can be seen in higher incidences in African-Americans as compared to Nigerian Blacks, and in Oklahoma Choctaw Indians as compared to Missouri Choctaw Indians. Testing for genetic markers are still considered a research tool and would not be helpful for diagnosis. Scleroderma has also been associated with exposure to silica dust and various organic solvents as well as the use of the appetite suppressants L-tryptophan, tainted rapeseed oil, vinyl chloride and bleomycin which is a chemotherapy medication,. Several retrospective studies have not found a link between silicone implants in women and systemic sclerosis. Other investigations have pointed toward a virus as a causative factor and there are several viruses, which are being investigated at this time.