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scleroderma

Overview of scleroderma

Scleroderma is an autoimmune connective tissue disease and rheumatic disease that causes inflammation of the skin and other areas of the body. When the immune response makes tissues think they are damaged, it causes inflammation and the body produces too much collagen, leading to scleroderma. Excess collagen in the skin and other tissues results in patches of tight and hard skin. Scleroderma affects many systems in your body. The following definitions will help you better understand how the disease affects each of these systems.

  • Connective tissue disease is a disease that affects tissues such as skin, tendons, and cartilage. Connective tissue supports, protects, and provides structure to other tissues and organs.
  • Autoimmune diseases occur when the immune system, which normally helps protect the body from infection and disease, attacks its own tissues.
  • Rheumatic diseases refer to a group of conditions characterized by inflammation or pain in muscles, joints or fibrous tissue.

There are two main types of scleroderma:

  • Localized scleroderma only affects the skin and structures directly under the skin.
  • Systemic scleroderma, also called systemic sclerosis, affects many body systems. This is a more serious type of scleroderma that can damage blood vessels and internal organs such as the heart, lungs, and kidneys.

There is no cure for scleroderma. The goal of treatment is to relieve symptoms and stop the progression of the disease. Early diagnosis and ongoing monitoring are essential.

What happens with scleroderma?

The cause of scleroderma is unknown. However, researchers believe that the immune system overreacts and causes inflammation and damage to the cells lining the blood vessels. This causes connective tissue cells, especially a cell type called fibroblasts, to produce too much collagen and other proteins. Fibroblasts live longer than normal, causing collagen to build up in the skin and other organs, leading to the signs and symptoms of scleroderma.

Who gets scleroderma?

Anyone can get scleroderma; however, some groups have a higher risk of developing the disease. The following factors may affect your risk.

  • Sex. Scleroderma is more common in women than in men.
  • Age. The disease usually appears between the ages of 30 and 50 and is more common in adults than in children.
  • Race. Scleroderma can affect people of all races and ethnic groups, but the disease can affect African Americans more severely. For example: 
    • The disease is more common in African Americans than in European Americans.
    • African Americans with scleroderma develop the disease earlier compared to other groups.
    • African Americans are more prone to skin lesions and lung disease compared to other groups.

Types of scleroderma

  • Localized scleroderma affects the skin and underlying tissues and usually presents with one or both of the following types:
    • Morpheus or scleroderma patches, which can be half an inch or more in diameter.
    • Linear scleroderma is when the thickening of scleroderma occurs along a line. It usually spreads down the arm or leg, but sometimes it spreads over the forehead and face.
  • Systemic scleroderma, sometimes called systemic sclerosis, affects the skin, tissues, blood vessels, and major organs. Doctors usually divide systemic scleroderma into two types:
    • Limited cutaneous scleroderma that develops gradually and affects the skin of the fingers, hands, face, forearms, and legs below the knees.
    • Diffuse cutaneous scleroderma that develops more rapidly and begins with the fingers and toes, but then spreads beyond the elbows and knees to the shoulders, trunk, and hips. This type usually has more damage to internal organs.  

scleroderma

Symptoms of scleroderma

Symptoms of scleroderma vary from person to person depending on the type of scleroderma.

Localized scleroderma usually causes patches of thick, hard skin in one of two types.

  • Morphea causes thickening of patches of skin into hard, oval-shaped patches. These areas may have a yellow, waxy appearance surrounded by a reddish or bruised edge. The spots may remain in one area or spread to other areas of the skin. The disease usually becomes inactive over time, but you may still have dark patches of skin. Some people also develop fatigue (feeling tired).
  • In linear scleroderma, lines of thickened or colored skin run down the arm, leg, and, rarely, forehead.

Systemic scleroderma, also known as systemic sclerosis, can develop quickly or gradually and can cause problems not only with the skin but also with internal organs. Many people with this type of scleroderma experience fatigue.

  • Localized cutaneous scleroderma develops gradually and usually affects the skin on the fingers, hands, face, forearms, and legs below the knees. It can also cause problems with the blood vessels and esophagus. The limited form has visceral involvement but is usually milder than the diffuse form. People with localized cutaneous scleroderma often have all or some of the symptoms, which some doctors refer to as CREST, which means the following symptoms:
    • Calcification, the formation of calcium deposits in the connective tissues, which can be detected by X-ray examination.
    • Raynaud's phenomenon, a condition in which the small blood vessels in the hands or feet contract in response to cold or anxiety, causing the fingers and toes to change color (white, blue, and/or red).
    • Esophageal dysfunction, which refers to dysfunction of the esophagus (the tube that connects the throat and stomach) that occurs when the smooth muscles of the esophagus lose their normal movement.
    • Sclerodactyly is thick and dense skin on the fingers resulting from the deposition of excess collagen in the layers of the skin.
    • Telangiectasia, a condition caused by swelling of tiny blood vessels that causes small red spots to appear on the hands and face.
  • Diffuse cutaneous scleroderma occurs suddenly, usually with thickening of the skin on the fingers or toes. The skin thickening then extends to the rest of the body above the elbows and/or knees. This type can damage your internal organs such as:
    • Anywhere in your digestive system.
    • your lungs.
    • your kidneys.
    • Your heart.

Although CREST has historically been referred to as localized scleroderma, people with diffuse scleroderma may also have signs of CREST.

Causes of scleroderma

Researchers don't know the exact cause of scleroderma, but they suspect several factors may be contributing to the condition:

  • genetic composition. Genes may increase the likelihood of certain people developing scleroderma and play a role in determining the type of scleroderma they have. You cannot inherit the disease, and it is not passed from parent to child like some genetic diseases. However, immediate family members of people with scleroderma are at a higher risk of developing scleroderma than the general population.
  • Environment. Researchers suspect that exposure to certain environmental factors, such as viruses or chemicals, may cause scleroderma.
  • Immune system changes. Abnormal immune or inflammatory activity in your body causes cellular changes that cause too much collagen to be produced.
  • Hormones. Women get most types of scleroderma more often than men. Researchers suspect that hormonal differences between women and men may play a role in the disease.