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June is Scleroderma Awareness Month

June has been designated Scleroderma Awareness Month. The name “scleroderma” is Greek in origin and translates to “hardening of the skin.” This disorder is a connective tissue disease that generally falls under the category of autoimmune rheumatic diseases. This disease is not contagious, infectious, communicable or cancerous.

The symptoms of scleroderma can range from mild to life threatening. The disease is diagnosed through blood tests and neurological consultations.  It is estimated that approximately 300,000 Americans may have the systemic form of scleroderma. Localized scleroderma is more common in children.  It is more common in females than males. The exact cause of the disease is unknown although it involves overproduction of collagen in the body.  Organ involvement increases the severity of symptoms. Genetic predisposition does not appear to be a factor as most patients do not know of family members who have had the disease prior to them.

Currently there is no cure for scleroderma, but many treatments are available to help with particular symptoms. For example, heartburn can be controlled through the use of medications that decrease the acid or improve motility of the bowel. Due to the great variation in symptoms, treatments also vary.

There are two major classifications of scleroderma; localized and systemic. Localized scleroderma is usually found on the skin or in muscles and rarely spreads anywhere else.  Generally speaking, this is the milder version of the disease. Morphea is a form of localized scleroderma characterized by waxy patches on the skin varying in size and shape. The patches may enlarge or shrink but is usually appears in people between the ages of 20 and 50 and is more common among young children. Linear scleroderma is a form of localized scleroderma which frequently starts as a line of hardened waxy skin on the arm, leg or forehead.  Sometimes it forms a long crease on the head or neck known as the en coup de saber due to its saber or sword like appearance. This type of scleroderma tends to involve the deeper layers of the skin as well as the motion of joints and usually develops during childhood.  Affected limbs may not grow as expected. Systemic Sclerosis may affect the connective tissue in all parts of the body including the skin, esophagus, gastrointestinal tract, lungs, heart and kidneys as well as other internal organs.

Blood vessels, joints and muscles may also be affected. The tissue of the organ will become hard and fibrous causing them to function less efficiently. Approximately 50 percent of patients have a slower and more benign form of illness called limited scleroderma. The skin thickening is less widespread typically limited to the fingers and hands and develops gradually. The internal organ problems that occur are less frequent and tend to be less severe in comparison to diffuse scleroderma. Despite that, patients with limited scleroderma may develop pulmonary hypotension, a condition in which the blood vessels in the lungs become narrow leading to impaired blood flow and shortness of breath. Limited scleroderma is sometimes called CREST syndrome which is symbolic for the initial letters of five common features including Calcinosis, Raynaud Syndrome, Esophageal dysfunction, Sclerodactyly, and Telangiectsia.

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