Diseases Medical Encyclopedia Health Navigator Drug Interactions Drug Notes Health Capsules Smart Sites Back Pain Hip Pain Knee Pain Weight Loss Scleroderma CREST syndrome; Limited scleroderma; Progressive systemic sclerosis; Systemic sclerosis; Localized scleroderma; Mixed connective disease; Morphea - linear Definition 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. Causes Scleroderma is a type of autoimmune disorder in which 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.Widespread scleroderma can occur with other autoimmune diseases, including systemic lupus erythematosus and polymyositis.These cases are referred to as mixed connective disease. Symptoms 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 lossSkin that is darker or lighter than normalStiffness, and tightness of skin of fingers, hands, forearm, and faceSmall 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 painNumbness and pain in the feetPain, stiffness, and swelling of fingers and jointsWrist pain Breathing problems may result from scarring in the lungs and can include:Dry coughShortness of breathWheezingDigestive tract problems may include:Bloating after mealsConstipationDiarrheaDifficulty swallowingEsophageal reflux or heartburnProblems 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 inflamed. Problems with your kidneys can lead to high blood pressure.Blood and urine tests may include:Antinuclear antibody (ANA) panelAntibody testingESR (sed rate)Rheumatoid factorComplete blood countMetabolic panel including creatinineUrinalysisOther tests may include:Chest x-rayCT scan of the lungsEchocardiogramTests to see how well your lungs and gastrointestinal (GI) tract are workingSkin biopsy Treatment There is no specific treatment for scleroderma.Your doctor will prescribe medicines and other treatments to control your symptoms and prevent complications.Medicines used to treat scleroderma include:CorticosteroidsDrugs that suppress the immune system such as methotrexate and CytoxanNonsteroidal anti-inflammatory drugs (NSAIDs)Other treatments for specific symptoms may include:Medicines for heartburn or swallowing problemsBlood pressure medications (such as ACE inhibitors) for high blood pressure or kidney problemsLight therapy to relieve skin thickeningMedicines to improve breathing (bosentan)Treatments to improve Raynaud's phenomenon, including medicines, gloves to keep the hands warm, avoiding smokingTreatment often involves physical therapy as well. Support Groups Some people can benefit from attending a support group for people with scleroderma. Outlook (Prognosis) In some people, symptoms develop quickly for the first few years and continue to get worse. However, in most people, the disease slowly gets worse.People who have only skin symptoms have a better outlook. Widespread (systemic) scleroderma can lead to.CancerHeart failureScarring of the lungs, called pulmonary fibrosisHigh blood pressure in the lungs (pulmonary hypertension)Kidney failureProblems absorbing nutrients from food When to Contact a Medical Professional Call your health care provider if your symptoms become worse or new symptoms develop. Open References References Varga J. Systemic Sclerosis (Scleroderma). In: Goldman L, Schafer AI, eds. Cecil Medicine. 24th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 275.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: Saunders Elsevier; 2012:chap 83. Raynaud's phenomenon Illustration Raynaud's phenomenon Raynaud's phenomenon is characterized by fingers becoming white due to lack of blood flow, then blue due to oxygen consumption, and finally red as blood flow returns. CREST syndrome Illustration CREST syndrome The symptoms involved in CREST syndrome are associated with the generalized form of the disease Systemic sclerosis (scleroderma). CREST is an acronym for the clinical features that are seen in a patient with this disease. The “C” stands for calcinosis, where calcium deposits form under the skin on the fingers or other areas of the body. The “R”, stands for Raynaud’s phenomenon, spasm of blood vessels in the fingers or toes in response to cold or stress. The “E” represents esophageal dysmotility, which can cause difficulty in swallowing. The “S” is for sclerodactyly, tightening of the skin causing the fingers to bend. Finally, the letter “T” is for telangiectasia, dilated vessels on the skin of the fingers, face, or inside of the mouth. Usually only 2 of the five symptoms of the CREST syndrome is necessary to be diagnosed with the disease. Sclerodactyly Illustration Sclerodactyly The most classic symptom of scleroderma is a type of skin tightening called sclerodactyly. The initial stages of the disease involves swelling of the fingers. Later, as the connective tissue becomes fibrotic, skin on the fingers and toes becomes hard and shiny. The fingers can become difficult to bend and can form contractures due to the severe tightening of the skin. Telangiectasia Illustration Telangiectasia Telangiectasia is the dilation of small superficial vessels and capillaries that cause numerous flat red marks on the hands, face and tongue. Telangiectasia can be a symptom of scleroderma or other systemic diseases. Tests for Scleroderma Gallium scan Rheumatoid factor (RF) A Closer Look Scleroderma Systemic lupus erythematosus Related Information Systemic Heart Failure Overview Acute kidney failure Idiopathic pulmonary fibrosis Malabsorption Review Date: 01/22/2014 Reviewed By: Gordon A. Starkebaum, MD, Professor of Medicine, Division of Rheumatology, University of Washington School of Medicine. Also reviewed by David Zieve, MD, MHA, Bethanne Black, and the A.D.A.M. Editorial team.