Systemic sclerosis (scleroderma)

Alternative names:

CREST syndrome; progressive systemic sclerosis; scleroderma

Symptoms:

• blanching, blueness, or redness of fingers and toes in response to heat and cold (Raynaud's phenomenon)
• pain, stiffness, and swelling of fingers and joints
• skin thickening and shiny hands and forearm
• skin is hard
• tight and mask-like facial skin
• ulcerations on fingertips or toes
• esophageal reflux or heartburn
• difficulty swallowing
• bloating after meals
• weight loss
• diarrhea
• constipation
• shortness of breath

Additional symptoms that may be associated with this disease:

• wrist pain
• wheezing
• skin, abnormally dark or light
• joint pain
• hair loss
• eye burning, itching & discharge

Signs and tests:

Examination of the skin may show tightness, thickening, and hardening.

• The ESR is elevated.
• The rheumatoid factor is elevated.
• An antinuclear antibody test is positive.
• Urinalysis shows protein and microscopic blood.
• A chest X-ray may show fibrosis.
• Pulmonary function studies often show restrictive lung disease.
• A skin biopsy may also be performed.

This disease may also alter the results of the following tests:

• LE cell test
• febrile/cold agglutinins
• esophageal manometry
• ACE levels