SclerodermaCREST syndrome; Limited scleroderma; Progressive systemic sclerosis; Systemic sclerosis; Localized scleroderma; Mixed connective disease; Morphea - linear
Scleroderma is a disease that involves the buildup of scar-like tissue in the skin. It also damages the cells that line the walls of the small arteries.
Scleroderma is a type of autoimmune disorder. In this condition, the immune system mistakenly attacks and destroys healthy body tissue.
The cause of scleroderma is unknown. A buildup of a substance called collagen in the skin and other organs leads to the symptoms of the disease.
The disease most often affects people 30 to 50 years old. Women get scleroderma more often than men do. Some people with scleroderma have a history of being around silica dust and polyvinyl chloride, but most do not.
Some types of scleroderma affect only the skin, while others affect the whole body.
- Localized scleroderma. Often affects only the skin on the hands and face. It develops slowly, and rarely spreads in the body or causes serious problems.
- Systemic scleroderma, or sclerosis. May affect large areas of skin and organs such as the heart, lungs, or kidneys. There are two main types: limited disease (CREST syndrome) and diffuse disease.
Skin symptoms of scleroderma may include:
- Fingers or toes that turn blue or white in response to cold temperatures (Raynaud's phenomenon)
- Hair loss
- Skin that is darker or lighter than normal
- Stiffness, and tightness of skin of fingers, hands, forearm, and face
- Small white lumps beneath the skin that sometimes ooze a white substance that looks like toothpaste
- Sores (ulcers) on the 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 result from scarring in the lungs and can include:
- Dry cough
- Shortness of breath
Digestive tract problems may include:
- Bloating after meals
- Difficulty swallowing
- Esophageal reflux or heartburn
- Problems controlling stools
Exams and Tests
The health care provider will do a physical exam. The exam may show tight, thick skin.
Your blood pressure will be checked. Scleroderma can cause small blood vessels in the kidneys to become narrowed. Problems with your kidneys can lead to high blood pressure and decrease function of the kidney.
Blood and urine tests may include:
- Antinuclear antibody (ANA) panel
- Antibody testing
- ESR (sed rate)
- Rheumatoid factor
- Complete blood count
- Metabolic panel including creatinine
Other tests may include:
- Chest x-ray
- CT scan of the lungs
- Tests to see how well your lungs and gastrointestinal (GI) tract are working
- Skin biopsy
There is no specific treatment for scleroderma. Your provider will assess the extent of disease in the lungs, kidneys, heart, and gastrointestinal tract.
You will be prescribed medicines and other treatments to control your symptoms and prevent complications.
Medicines used to treat scleroderma include:
- Corticosteroids such as prednisone. Doses above 10 mg per day are not recommended.
- Drugs that suppress the immune system such as methotrexate
- Nonsteroidal anti-inflammatory drugs (NSAIDs)
Other treatments for specific symptoms may include:
- Medicines for heartburn or swallowing problems
- Blood pressure medicines (such as ACE inhibitors) for high blood pressure or kidney problems
- Light therapy to relieve skin thickening
- Medicines to improve breathing (bosentan)
- Treatments to improve Raynaud phenomenon, including medicines, gloves to keep the hands warm, and avoiding smoking
Treatment often involves physical therapy as well.
Some people can benefit from attending a support group for people with scleroderma.
In some people, symptoms develop quickly for the first few years and continue to get worse. However, in most people, the disease gets worse slowly.
People who have only skin symptoms have a better outlook. Widespread (systemic) scleroderma can lead to.
- Heart failure
- Scarring of the lungs, called pulmonary fibrosis
- High blood pressure in the lungs (pulmonary hypertension)
- Kidney failure
- Problems absorbing nutrients from food
When to Contact a Medical Professional
Call your health care provider if your symptoms become worse or new symptoms develop.
Mendoza FA, Nagle SJ, Lee JB, Jimenez SA. A prospective observational study of mycophenolate mofetil treatment in progressive diffuse cutaneous systemic sclerosis of recent onset. J Rheumatol. 2012; 39:1241. PMID: 22467932 www.ncbi.nlm.nih.gov/pubmed/22467932.
Varga J. Etiology and Pathogenesis of Scleroderma. In: Firestein GS, Budd RC, Gabriel SE, et al, eds. Kelley's Textbook of Rheumatology. 9th ed. Philadelphia, PA: Elsevier Saunders; 2012:chap 83.
Varga J. Systemic Sclerosis (Scleroderma). In: Goldman L, Schafer AI, eds. Goldman's Cecil Medicine. 25th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 267.
Raynaud's phenomenon - illustration
CREST syndrome - illustration
Sclerodactyly - illustration
Telangiectasia - illustration
Review Date: 1/20/2015
Reviewed By: Gordon A. Starkebaum, MD, Professor of Medicine, Division of Rheumatology, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.