Diagnosis of Scleroderma

Signs and Symptoms of Scleroderma

Initial signs and symptoms vary with the different types of scleroderma. For localized scleroderma, the first symptom usually appears spontaneously and is most often a red, swollen area on the skin characterized by hair loss, and anhidrosis (reduced sweat response). There is no pitting of the skin in the affected area.

The onset of systemic scleroderma is more pronounced and the symptoms are more diffuse. These may include:

• CREST syndrome

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

  • Raynaud's phenomenon - numbness, pain or color changes in the extremities brought on by cold temperatures or emotional stress. This is 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 systemic 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 systemic 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.

• Progressive thickening, hardening, and tightening of the skin (fibrosis) especially on the arms or hands, resulting in loss of flexibility. In limited systemic scleroderma, skin thickening is usually limited to the hands and face.

• Swelling and/or itching of the skin of the hands, feet, chest or face.

• Curling (flexion) and stiffening of the fingers

• Stiffness or pain in muscles and/or joints especially in the morning. This pain is usually worse than what would be otherwise expected from a generic inflammation. This is often an early symptom of systemic scleroderma.

• Sores over the joints such as the elbows and knuckles

• Digestive difficulties from both impaired absorption of nutrients and muscular activity in the intestine

• Shortness of breath

• Chest pain

• Dizziness

• Syncope (fainting)

• Constipation or diarrhea

• Fatigue and weight loss

• Depression (studies show up to 50% of patients with systemic scleroderma develop moderate to severe depression)

• Sjogren's syndrome - an autoimmune disorder characterized by dry eyes, dry mouth, dry skin, joint stiffness and fatigue. Approximately 1% of patients with systemic scleroderma develop Sjogren's syndrome.