Systemic Scleroderma

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Systemic scleroderma or systemic sclerosis involves not only the skin, but the tissues underneath. The blood vessels and many major internal organs are involved, most commonly in the digestive, circulatory, pulmonary, and muscular systems. Systemic scleroderma is often divided into three forms: limited, diffuse and sine.

Limited scleroderma typically develops gradually and affects the skin only in certain areas, usually the fingers, hands, face, lower arms and legs. It is often referred to as CREST, which is an acronym that stands for the following combination of symptoms:

C – Calcinosis – small white calcium deposits form in the connective tissues. These deposits are typically found on the fingers, hands, face and trunk and can be quite painful as they work their way to the surface of the skin causing open sores.

R – Raynaud's phenomenon – poor circulation in the fingers and/or toes are caused when small blood vessels in the digits constrict or narrow. This decreases blood flow causing patients to be unduly sensitive to cool temperatures. Sensitivity to the cold, primarily in hands and feet, are accompanied by changes in the colour of the skin. Raynaud's can also be a response to stress or emotion. Many people have Raynaud's phenomenon for years before the skin thickening begins.

E – Esopahgeal dysfunction - impaired function of the esophagus occurs when muscles lose normal movement, causing difficulty in swallowing, heartburn, or reflux.

S – Sclerodactyly – because of deposits of excess collagen within skin layers, the skin on fingers and sometimes toes become thick and shiny. As the skin thickens, the affected digits can become curled.

T – Telangiectasia – small clusters of dilated blood vessels may appear as red spots in the skin especially on the face, fingers and palms of hands.

The CREST syndrome often manifests itself slowly over a period of ten to twenty years. Usually it involves the skin first, then the esophagus, lungs, and bowels. This form of systemic scleroderma tends to have less severe organ damage than the diffuse form.

Diffuse scleroderma is the most serious form of the disease. It typically develops quickly with the skin thickening occurring over much of the body including the face, neck, torso, and both hands, arms, feet and legs . This usually occurs in a symmetrical fashion. Internally, it can damage many of the key organs: the esophagus, the digestive tract, kidney, lungs, and/or heart.

Patients with diffuse scleroderma are often tired, lose appetite and weight, and have joint swelling and/or pain. The damage that patients with diffuse scleroderma experience typically occurs in the first several years. The disease often enters a stable phase lasting for varying lengths of time. During this phase, skin thickness and appearance change very little. Damage to internal organs progresses very little, if at all. Other symptoms often subside. Less collagen is made and the body seems to get rid of the excess. Some patients' skin returns to a somewhat normal state, while others are left with thin, fragile skin, without hair or sweat glands. More serious damage to heart, lungs or kidneys is unlikely to occur unless previous damage leads to deterioration.

Usually less than one third of patients with diffuse disease develop more severe complications to the lungs, heart, kidneys or digestive system. These patients face the most serious long-term outlook.

Sine scleroderma may resemble either limited or diffuse systemic sclerosis, causing damage to the lungs, kidneys, and blood vessels. The key difference between sine and other forms of systemic sclerosis is that the skin is not involved.