Main

 
What Is Scleroderma?
WHAT IS SCLERODERMA ?

 
Scleroderma is a disease that can affect the skin, joints, blood vessels, and internal organs. The word scleroderma means "hard skin." Most people with scleroderma have problems with their skin and other parts of their bodies.


Scleroderma is a chronic disease. This means it may last for months, years, or for a lifetime. There is no cure for it, but it can be treated. With early detection and proper management, many people with scleroderma can lead full, productive lives.

There are two forms of scleroderma: Localized and Generalized (also called systemic sclerosis).
 

LOCALIZED SCLERODERMA

 
Localized scleroderma affects mainly the skin. It can affect skin in different areas of the body. It may also affect muscles and bone, but it does not affect internal organs. This form is usually not as severe as generalized scleroderma. People who develop localized scleroderma usually do not develop generalized scleroderma. There are two types of localized scleroderma: Morphea and Linear.
 

MORPHEA

 
Morphea happens when hard, oval-shape patches form on the skin. The patches are usually whitish with a purplish ring around them. They usually occur on the trunk, but can also occur on the face, arms, legs, and other parts of the body. Morphea often improves by itself, over time.
 

LINEAR

 
Linear is a line of thickened skin that occurs in areas such as the arms, legs, or forehead. It can occur in more than one area. The line can extend deep into the skin and affect the bones and muscles underneath it. This can affect the motion of joints and muscles, as well as the growth of the affected area.


When the line forms a long crease on the head or neck, it is sometimes called en coup de sabre (the strike of the sword).
Linear scleroderma usually occurs in childhood.

 

GENERALIZED

 
Generalized scleroderma affects many parts of the body. It can affect the skin as well as internal body parts, such as blood vessels, the digestive system (esophagus, stomach, and bowel), the heart, lungs, kidneys, muscles, and joints. The severity of this form depends on the organs affected and how much they are affected. In rare cases, scleroderma may affect only some internal system, leaving the skin and joints untouched.
There are two types of generalized scleroderma: Limited (also called the CREST Syndrome) and Diffuse.
 
CREST stands for a combination of symptoms: Calcinosis, Raynaud's phenomenon, Esophageal dysfunction, Sclerodactyly, and Telangiectasia. This type usually has a slow onset, with the first symptoms appearing 10 to 20 years before the full syndrome occurs. It usually affects the skin on the face, fingers, and hands. Later on, it may affect internal organs, such as the esophagus (the tube leading from the mouth to the stomach), the lungs and bowels.
 

DIFFUSE

 
Diffuse occurs throughout the body. It usually affects the skin as well as other body parts, such as the lungs, kidneys, heart, bowels, blood vessels and joints. Depending on the areas affected, this type can cause problems such as high blood pressure, muscle weakness, trouble swallowing, or shortness of breath.
 
Diffuse scleroderma may progress slowly in some people and more rapidly in others. However, with proper management, it can be controlled.