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• Calcinosis - deposits of calcium crystals under the skin around the joints and organs. Skin ulcers may form over these areas.

• Raynaud 's phenomenon - caused by changes in the small arteries and capillaries resulting in constriction and a temporary disruption of circulation, usually in the extremities (fingers, toes, nose and ears). This is often the first symptom of scleroderma.

• Esophageal motility dysfunction (dysphagia) - muscles in the esophagus are unable to contract normally due to scarring. This can cause heartburn, or a sensation of food being stuck in the throat or chest. It is estimated that up to 90% of patients with scleroderma have esophageal involvement.

• Sclerodactyly (acrosclerosis) - stiffness and tightening of the skin of the fingers. Bone loss may also occur in the fingers and toes. This symptom is usually found distal to the elbows and knees and may or may not involve the face.

• Telangiectasia - dilation of the small vessels and capillaries near the skin surface, causing flat red marks on the palms of the hands, face and tongue.

Progression of limited systemic scleroderma is usually slow and the skin involvement is most often confined to the face and fingers or distal extremities but not the arms, chest or abdomen, back or thighs. The severe forms of limited systemic scleroderma can cause organ damage as well as functional and cosmetic disfigurement. Some patients slowly progress over many years, even decades, to develop serious pulmonary (lung) and gastrointestinal complications.

Diffuse Systemic Scleroderma

Diffuse systemic scleroderma is a chronic condition and is much more severe than limited systemic scleroderma. It is characterized by rapid, extensive skin fibrosis that often begins with swelling of the hand and face and then extends to proximal parts of the body, usually between the knees/elbows and the trunk, and is marked by organ involvement. Although there is variability from person to person in the progression of the disease, involvement of the lungs, gastrointestinal tract, and kidneys is usually seen early in the course of the disease. Long- standing diffuse systemic scleroderma can lead to physical deformities such as constricture of the fingers and toes and tightening of the mouth.

Systemic sclerosis sine scleroderma is a subtype of systemic scleroderma that affects the organs but not the skin. Systemic sclerosis sine scleroderma represents only about 10% of cases of diffuse systemic scleroderma.

The term "scleroderma" in this Medifocus Guidebook will be used to refer to "systemic scleroderma" unless otherwise noted.