• Limited*
• Diffuse
• Sine

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.