Scleroderma
Scleroderma is a chronic systemic autoimmune disease primarily of the skin and characterized by fibrosis or hardening of the skin, vascular alterations, and autoantibodies.
There are two major forms:
Involves cutaneous manifestations that mainly affect the hands, arms, and face. There can be serious complications of the disease including calcinosis, Raynaud’s phenomenon, esophageal dysfunction, and pulmonary arterial hypertension.
Is rapidly progressing and affects a large area of the skin and one or more internal organs, frequently the kidneys, esophagus, heart, and lungs. This form of scleroderma can be quite disabling. There are no treatments for scleroderma itself, but individual organ system complications are treated.
There are other forms of scleroderma which are localized forms and lack skin changes. Prognosis is generally good for limited cutaneous scleroderma patients who escape pulmonary complications. The cause is unknown and it runs in families. There is no direct cure for scleroderma because the exact cause is unknown and the treatment is specific for each patient depending on the symptoms and involvement of other areas.
Refer to the Scleroderma Foundation for more information and your rheumatologist.
Author: Norman B. Gaylis, M.D., F.A.C.P., M.A.C.R.
