Literally, the term scleroderma means “hard skin”. It is a progressive disease of the vascular and immune systems and a severe connective tissue disorder. In scleroderma, some unknown factor triggers the over production of collagen (body protein), causing thickening, hardening and scarring of the skin and other organs. This often affects the small blood vessels that carry blood to many parts of the body.
Scleroderma is also known as systemic sclerosis.
Types of Scleroderma
There are two types of Scleroderma, Localized and Systemic.
- Linear Scleroderma
This form of scleroderma affects some areas of the skin, but does not affect the internal organs.
Morphea is the most common form of localized scleroderma and is characterized by oval patches of inflamed, often discoloured skin. The trunk, face, and extremities may be involved.
In linear scleroderma, a band or bands of skin hardens or thickens on the trunk and/or extremities.
This form of scleroderma involves not only the skin but also internal organs, most commonly the digestive, circulatory, pulmonary, and muscular systems. Systemic scleroderma is divided into three forms: limited, diffuse and sine. Sine may resemble either limited or diffuse systemic sclerosis causing damage in lungs, kidneys and/or blood vessels. However, unlike other forms of systemic sclerosis, the skin in sine is not usually involved.
Diffuse scleroderma is the most serious internally involved form of the disease. It involves many of the internal organs of the body: esophagus, the digestive tract, kidney, heart, and/or lungs. The skin involvement includes the face, neck, torso, and both hands, arms, feet and legs.
Sine may resemble either limited or diffuse systemic sclerosis causing damage in lungs, kidneys and/or blood vessels. However, unlike other forms of systemic sclerosis, the skin in sine is not usually involved.
*Limited scleroderma is often referred to as CREST. This is an acronym that stands for a combination of symptoms:
- C – Calcinosis – small white calcium lumps forming under the skin
- R – Raynaud’s Phenomenon – poor circulation in the fingers and/or toes. Small blood vessels in the fingers tend to narrow and decrease blood flow causing patients to be unduly sensitive to cool temperatures. This narrowing can also be a response to stress or emotion. Fingers often turn white, then blue
- E – Esophageal dysfunction – difficulty swallowing, heartburn, or regurgitation
- S – Sclerodactyly – skin of fingers and sometimes toes become thick and shiny. Affected digits may be difficult to move and may become fixed in a bent position
- T – Telangiectasia – small clusters of dilated blood vessels in the skin especially on the face and fingers and palms of hands
The CREST syndrome commonly manifests itself slowly over a period of ten to twenty years. Usually it involves the skin first, then the esophagus, lungs, and bowels.
Please contact us for more information.
Scleroderma Association of Saskatchewan
54 – 4901 Child Avenue,