Scleroderma is not a disease but rather a symptom of a group of diseases associated with abnormal growth of collagen in the body’s connective tissue. This excess of collagen hardens the skin causing it to become stiff and immobile. Scleroderma is more common in women than men.
There are many different types of scleroderma, but two main classes: localized and systemic. Localized scleroderma types affect the skin, connective tissue and sometimes the muscle below. Systemic scleroderma, also called systemic sclerosis, is an autoimmune disease involving the skin and connective tissues along with other major organs. This form of scleroderma is most common in African American women.
Localized scleroderma often presents in one of two forms, morphea and linear. Morphea is typically seen in those of European descent between the ages of 20 and 40. In linear scleroderma, thick bands of abnormally colored skin run down the arm, leg or forehead in a sword stroke pattern. Localized scleroderma may improve over time, but the skin changes are permanent.
Limited systemic scleroderma progresses gradually and affects skin only in certain areas of the body including the fingers, hands, face, forearms and legs. Individuals may present with some or all of the below symptoms, which dermatologists and health care professionals may refer to as CREST syndrome:
- Calcinosis – calcium deposits in connective tissues that can break through the skin and form ulcers
- Raynaud’s phenomenon – a condition, triggered by cold temperatures or anxiety, where the small blood vessels in the hands and feet constrict causing them to turn white or blue
- Esophageal dysfunction – muscle stiffness in the gastrointestinal tract that can lead to reflux or heartburn
- Sclerodactyly – tight, hardened, shiny skin on the fingers caused by excess collagen deposits
- Telangiectasis – Swelling of the small blood vessels near the surface of the skin causing red spots to appear on the hands and face
Diffuse systemic sclerosis symptoms appear suddenly and skin thickening is more widespread. Internal organs are also more likely to be affected. Careful monitoring and treatment is needed to avoid serious health consequences.
Dermatologists often diagnose scleroderma based on skin symptoms, medical history and physical exam. Blood work may be ordered to confirm the diagnosis. Since there is no treatment to stop the overproduction of collagen in scleroderma, the goal of treatment is symptom management.