Scleroderma is an autoimmune rheumatic disease that affects the connective tissues. It is similar to diseases that fall in the category of Systemic Lupus Erythematous (SLE). The term ‘sclero’ means hard and ‘derma’ means skin in Greek. Hardening of the skin is the main characteristics of this disease.
Scleroderma is a chronic condition that turns into a debilitating condition as the disease progresses. It can affect the lungs, kidneys and the gastrointestinal tract, leading to fatal consequences. When muscles are affected, it causes disability to move. In some cases, people need amputation of digits or limbs, due to peripheral damage by scleroderma.
This disease is very dangerous as it can affect various systems of the body. For example, it can affect the connective tissues, leading to hardening of the skin, tendons and bones. It can affect the vascular system and lead to damage of blood vessels. And as it is an autoimmune disorder, it causes the body to make antibodies against healthy cells of the body, leading to various other conditions.
There is no known cause for scleroderma. It is an autoimmune disease where the fibroblasts produce too much collagen leading to hardening of the skin. It is said to be hereditary, but the exact genes that cause this condition has not yet been identified. Also, the immune system of the body turns against the body and leads to inflammation.
Types of Scleroderma
Scleroderma has been classified as two major types. These include:
This condition affects the skin and soft tissues of the body. It affects the internal organs to some extent and is further divided into two types. These include:
- Morphea: Skin develops thick bands of hard skin that is initially red in color and progresses to form whitish bands. In this condition, the internal organs are not affected.
- Linear scleroderma: In this type, the skin as well as muscles, bones and tissues are affective. It usually appears during childhood and causes headaches, muscle weakness, etc.
In this condition, not just the skin and tissues are affected, but it progresses towards many major organs and tissues of the body. It manifests itself in two types, that includes:
- Limited cutaneous systemic sclerosis
This condition causes deposits of calcium in the skin, Raynaud’s phenomenon, problems with esophageal functioning, thickening of the skin and tendons of the fingers and toes as well as tiny red spots on hands, face, and mouth.
- Diffuse cutaneous systemic sclerosis
In this condition, the disease progresses from the skin to a disease of the internal organs.
The symptoms include:
- Thickened and hardened skin of the hands
- Facial skin becomes thick
- Fingers and toes become blue, especially in cold temperature due to Raynaud’s phenomenon
- Dilated capillaries that cause the appearance of small red spots on face, hands and arm
- Calcium deposits under the sin causing appearance of soft or hard lumps
- Pain and stiffness in joints
- Esophageal dysfunction causes problems swallowing and heartburn
There is no particular test that will help diagnose scleroderma. The doctor may conduct many tests on different organs to determine which ones are affected along with observation of hardened skin.
There is no particular treatment for scleroderma. This is because one does not know the exact cause of this autoimmune disease. The doctor will treat the patient for other complications arising due to the disease. These include:
- Medications to increase blood flow to body extremities
- Medications like cyclosporine, colchicine, etc. to treat skin fibrosis
- Immunosuppressive drugs that help keep the immune system in control like methotrexate, azathioprine, etc.
- NSAIDs for treating joint pain
- Medications for high blood pressure and kidney problems
- Antacids for heartburn
People who have scleroderma affecting just the skin and tissues, have a better prognosis. Those who have systemic sclerosis, are more likely to have poor prognosis. Most patients have 20 to 50 years life span after diagnosis. However, patients who have a rapidly progressive scleroderma, have just 50% survival rate for 5 years after diagnosis.
Scleroderma is a challenging disease as there is no known cause or cure. However, early diagnosis and following the treatment regiment advised helps people live a longer, active life.
Date last updated: February 25, 2015