• Alternative Names

    CREST syndrome; Limited scleroderma; Progressive systemic sclerosis; Systemic sclerosis; Localized scleroderma; Morphea, linear


    Localized scleroderma usually affects only the skin on the hands and face. Its course is very slow, and it rarely, if ever, spreads throughout the body (becomes systemic) or causes serious complications.

    Systemic scleroderma is also called systemic sclerosis. This form of the disease may affect the organs of the body, large areas of the skin, or both. This form of scleroderma has two main types: limited and diffuse scleroderma.

    Skin symptoms may include:

    • Blanching, blueness, or redness of fingers and toes in response to heat and cold ( Raynaud's phenomenon)
    • Hair loss
    • Skin hardness
    • Skin that is abnormally dark or light
    • Skin thickening, stiffness, and tightness of fingers, hands, and forearm
    • Small white lumps beneath the skin, sometimes oozing a white substance that looks like toothpaste
    • Sores (ulcerations) on fingertips or toes
    • Tight and mask-like skin on the face

    Bone and muscle symptoms may include:

    • Joint pain
    • Numbness and pain in the feet
    • Pain, stiffness, and swelling of fingers and joints
    • Wrist pain

    Breathing problems may include:

    • Dry cough
    • Shortness of breath
    • Wheezing

    Digestive tract problems may include:

    • Bloating after meals
    • Constipation
    • Diarrhea
    • Difficulty swallowing
    • Esophageal reflux or heartburn
    • Problems controlling stools (fecal incontinence)

    Signs and tests

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

    Blood tests may include:

    • Antinuclear antibody
    • Anticentromere antibody
    • Anti-SCL-70 antibody
    • ESR
    • Rheumatoid factor

    Other tests may include:

    • Chest x-ray
    • CT scan of the lungs
    • Echocardiogram
    • Urinalysis
    • Pulmonary function studies
    • Skin biopsy